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Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

Publisher Color Studio®

àÁ‰‡ÌË ̇ Å˙΄‡ÒÍÓÚÓ ‰ÛÊÂÒÚ‚Ó ÔÓ Ì‚ÓÎÓ„Ëfl Official Journal of The Bulgarian Society of Neurology êÖÑÄäñàéççÄ äéãÖÉàü

EDITORS

ÄÎÂÍÒË‚ Ä. ŇÈÍۯ‚ ë. ÅÂÎÓÔËÚÓ‚‡ ã. ÅÓÊËÌÓ‚ ëÚ. LJÒË΂‡ í. ÇÂ΂‡ ëÚ. ɇÌ‚‡ É. ÉÂÓ„Ë‚ Ñ. ÉÂ‡ÒËÏÓ‚ Å. ÉË„ÓÓ‚‡ é. ÑÂ΂‡ ç. ÑÂÌÒÍË Ç á‡ı‡Ë‚ á. à‚‡ÌÓ‚‡ ã. à¯ÔÂÍÓ‚‡ Å. âÓÚÓ‚‡ ê. äÓ΂ é. ä˛˜ÛÍÓ‚ å. å‡Ì˜Â‚ à. åË·ÌÓ‚ à. åË̘‚ Ñ. çËÍÓ‚ÒÍË ç. èÂÚÓ‚ à. èÓÔÓ‚‡ å. ꇯ‚‡ å. ÂÁÓ‚‡ ã. ÊË‚ Ñ. ÊËÔÂÚÓ‚‡ Ö. ñ‡ÌÍÓ‚‡ ñ. ñ‚ÂÚ‡ÌÓ‚‡ Ö. 󇂉‡Ó‚ Ñ. ó‡ÎχÌÓ‚ Ç. ó‡Î˙ÍÓ‚‡ ç. òÓÚÂÍÓ‚ è. ü̘‚‡ ë.

Alexiev A. Baykushev S. Belopitova L. Bojinov St. Vassileva T. Veleva St. Ganeva G. Georgiev D. Gerassimov B Grigorova O. Deleva N. Drensky V. Zahariev Z. Ivanova L. Ishpekova B. Iotova R. Kolev O. Kyuchukov M. Manchev I. Milanov I. Minchev D. Nikoevsky N. Petrov I. Popova M. Rasheva M. Havezova L. Hadjiev D. Hadjipetrova E. Tzankova Tz. Tzvetanova E. Chavdarov D. Chalmanov D. Chalakova N. Shotekov P. Yancheva S.

ëÓÙËfl èÎÓ‚‰Ë‚ ëÓÙËfl è΂ÂÌ èÎÓ‚‰Ë‚ ëÓÙËfl ëÓÙËfl ëÓÙËfl ëÓÙËfl ëÓÙËfl LJ̇ LJ̇ èÎÓ‚‰Ë‚ ëÓÙËfl ëÓÙËfl ëÓÙËfl ëÓÙËfl ëÓÙËfl ëÚ.ᇄÓ‡ ëÓÙËfl LJ̇ ëÓÙËfl ëÓÙËfl è΂ÂÌ ëÓÙËfl LJ̇ ëÓÙËfl èÎÓ‚‰Ë‚ ëÓÙËfl ëÓÙËfl ëÓÙËfl ëÓÙËfl èÎÓ‚‰Ë‚ ëÓÙËfl ëÓÙËfl

Sofia Plovdiv Sofia Pleven Plovdiv Sofia Sofia Sofia Sofia Sofia Varna Varna Plovdiv Sofia Sofia Sofia Sofia Sofia St. Zagora Sofia Varna Sofia Sofia PlÂven Sofia Varna Sofia Plovdiv Sofia Sofia Sofia Sofia Plovdiv Sofia Sofia

ÉãÄÇçà êÖÑÄäíéêà Ñ.ó‡Í˙Ó‚, è. ëÚ‡ÏÂÌÓ‚‡, à. ÇÂΘ‚‡

EDITORS IN CHIEF D. Chakarov, P. Stamenova, I. Velcheva

ëÖäêÖíÄê å. чÒ͇ÎÓ‚

SECRETARY M. Daskalov

íÓÁË ·ÓÈ Ì‡ ÒÔËÒ‡ÌË Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl ËÁÎËÁ‡ Ò ÔÓ‰ÍÂÔ‡Ú‡ ̇


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology ìäÄáÄçàÖ áÄ ÄÇíéêàíÖ

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl

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INSTRUCTION FOR AUTHORS

Bulgarian Neurology

is the official journal of the Bulgarian Society of Neurology. It will consider for publication papers in neurology and related areas in the following categories: -Editorials, consisting of up to 3 pages, when approved by the Editorial Board -Original papers - up to 8 pages, including tables, figures and references. An abstract in Bulgarian and English up to 40 rows on a separate sheet is required. The abstract should contain title, authors, objective, background, methods, results and conclusions plus up to 6 keywords. The original papers include short introduction, material, methods results, discussion and references. The title page should carry the full title, the short running title, the authors with their initials, academic degrees, institutional affiliations, address for correspondence, with telephone, fax and e-mail -Short communications and case reports up to 3 pages -Review articles up to 10 pages -Book reviews and information. It includes information for new books, congresses and conferences, new drugs, future events in neurology -Letters to the Editor with comments on previously published papers, short case reports and discussion on current problems -The manuscripts should be submitted on diskette / 3.5 inch/ using Word 6/Windows 96 or Word7/Windows 98 with a printed copy. The tables should be presented on separate sheets with a short heading The illustrations /figures, diagrams, formulas/ should be ready for reproduction. Explanatory legends should be provided on a separate sheet of paper . References should be presented in alphabetic order on a separate sheet with all authors` names and full title of papers . In the text the authors should be indicated by the number from the reference list . The reference should be presented as follows: Journal paper : (1) author(s), (2)title, (3) journal name ( as abbreviated in Index Medicus ). (4) volume, (5) year of publication, (6) journal number , (7) inclusive pages. Example: Andersen, G., Vestergaard, K., Lauritzen, L. Effective treatment of poststroke depression with the selective serotonin reuptake inhibitor citalopram. Stroke, 25, 1994, 6, 10991104. Book : (1) author(s), (2) title, (3) city of publication, (4) publisher, (5) year of publication , (6) pages. Example : Calligaro,K., DeLaurentis, D., Baker, W. Management of Extracranial Cerebrovascular Disease.Philadelphia-New York, Lippincott - Raven Publishers, 1997, 217. References to books : (1) authors(s), (2) chapter title, (3) title of book, (4) editor(s), (5) city of publication, (6) publisher, (7) year of publication, (8) specific pages. Example : Binnie, C. , Jeavsons , .M. Photosensitive epilepsies. In : Epileptic syndromes in infancy, childhood and adolescence , eds. J.Roger, M. Bureau, Ch.Dravet, F.E. Dreifuss, A.Perret , P.Wolf. London, John Libbey & Company, Ltd, 1992, 299-305. The articles are considered for publication on the understanding that the presented material has not been published or is being submitted elsewhere before appearing in BULGARIAN NEUROLOGY. Authors should indicate that ethical approval of the study was granted and that informed consent was given. All authors should approve and sign the final manuscript. Manuscript with one copy (or a diskette and a copy) should be sent to the following address: Sofia 1504, Bulgaria, 8, Bialo more str. Department of Neurology University Hospital ◊ Queene Jovanna“: Prof. P. Stamenova


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology ëöÑöêÜÄçàÖ

CONTENTS

éÅáéêà

REVIEWS

ÉÂÌÂÚË͇ Á‡ ÏÓÁ˙˜ÌËfl ËÌÒÛÎÚ. åÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl ‡ÒÓˆËË‡ÌË Ò ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ è. òÓÚÂÍÓ‚, à. í˙Ì‚, Ç. åËı‡ÈÎÓ‚‡ . . . . . . . . 114

Genetics of Stroke. Single gene disorders associated with stroke P. Shotekov, I. Tournev, V. Mihaylova . . . . . . . . . . .114

ÉÂÌÂÚË͇ ̇ ÔÓÎË„ÂÌÌËfl ËÌÒÛÎÚ è. òÓÚÂÍÓ‚, à. í˙Ì‚, Ç. åËı‡ÈÎÓ‚‡ . . . . . . . . 119

Genetics of Polygenic Stroke P. Shotekov, I. Tournev, V. Mihaylova . . . . . . . . . . .119

ë˙‚ÂÏÂÌÌË ‡ÒÔÂÍÚË Ì‡ ̇ÒΉÒÚ‚Â̇ڇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl. åÓÎÂÍÛÎÌË ÏÂı‡ÌËÁÏË ç.à‚‡ÌÓ‚‡, Ä.âÓ‰‡ÌÓ‚‡, Ç.åËÚ‚, Ä.Ä̉‚‡, à.í˙Ì‚, à.ãËÚ‚ËÌÂÌÍÓ, à.äÂÏÂÌÒÍË . . . . . . . 124

New aspects in hereditary spastic paraplegia. Molecular mechanisms N. Ivanova, A. Iordanova, V. Mitev, A. Andreeva, I. Tournev, I. Litvinenko, I. Kremenska . . . . . . . . . . . .124

ë˙‚ÂÏÂÌÌË ‡ÒÔÂÍÚË Ì‡ ̇ÒΉÒÚ‚Â̇ڇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl. äÎËÌË͇, ‰Ë‡„ÌÓÒÚË͇ Ë Ì‚ÓÔ‡ÚÓÎÓ„Ëfl Ä. Ä̉‚‡, à. í˙Ì‚, à. ãËÚ‚ËÌÂÌÍÓ, ç. à‚‡ÌÓ‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .130

New aspects in hereditary spastic paraplegia. Clinical features, diagnostic criteria and neuropathology A. Andreeva, I. Turnev,I. Litvinenko, N. Ivanova . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .130

ÅÓÎÂÒÚ Ì‡ Wilson – ÍÎËÌËÍÓ-„ÂÌÂÚ˘ÌË ‡ÒÔÂÍÚË Ç.åËı‡ÈÎÓ‚‡, à.í˙Ì‚ . . . . . . . . . . . . . . . . . . . .135

Clinical and Genetic Aspects of Wilson’s Disease V. Mihaylova, I. Tournev . . . . . . . . . . . . . . . . . . . . .135

ïËÛ„˘ÌÓ Î˜ÂÌË ̇ ‡ÛÚËÒÚËÒÚ˘ÌÓÂÔËÎÂÔÚËÙÓÏÂ̇ „ÂÒËfl Ë Landau-Kleffner ÒË̉ÓÏ â. è‡ÌÓ‚, ã. è‡ÌÓ‚‡ . . . . . . . . . . . . . . . . . . . . . . .140

Surgical treatment of autistic epileptiform regression and Landau- Kleffner syndrome

éêàÉàçÄãçà ëíÄíàà

ORIGINAL PAPERS

äÓÌ„ÂÌËÚ‡Î̇ ͇ڇ‡ÍÚ‡, Îˈ‚ ‰ËÁÏÓÙËÁ˙Ï, Ì‚ÓÔ‡ÚËfl /ääãÑç/ ÒË̉ÓÏ ÔË ˆË„‡ÌË – ˜ËÒÚ ‰ÂÙÂÍÚ Ì‡ êçä ÔÓÎËÏÂ‡Á‡ II – ωËË‡Ì‡ Ú‡ÌÒÍË·ˆËfl à. í˙Ì‚, ê. LJÓÌ, Ñ. ÄÌ„Â΢‚‡, Ç. ÉÂ„ÂΘ‚‡, ê. ÉÛ‰ËÌ„, Å. âÓÎ, Å. à¯ÔÂÍÓ‚‡, ê. äËÌ„, å. ê‡È˜Â‚‡, á. ä‡ÏÂÌÓ‚, ë. óÂÌËÌÍÓ‚‡, Ä. íÓÏÓ‚, ê. èÂÚÍÓ‚, Ç. ÅÓÊËÌÓ‚‡, å. 䇈‡Ó‚‡, Ä. òχÓ‚, à. ãËÚ‚ËÌÂÌÍÓ, ê. íË̘‚‡, ï. ñÂÍÓ‚, è. ÅÓËÒÓ‚‡, Å. ÅÛÊÓ‚, û. èÂÚÓ‚‡, è.ä. íÓχÒ, ã. ä‡Î‡È‰ÊË‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . .142

Congenital cataracts facial dysmorphism neuropathy syndrome in gypsies – pure defect in polymerase II – mediated transcription

äÎËÌËÍÓ-„ÂÌÂÚ˘ÌÓ ÔÓÛ˜‚‡Ì ̇ ıÂ‰ËÚ‡̇ڇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ ãéå /ïåëçã; MIM 601455/ ‚ Å˙΄‡Ëfl à. í˙Ì‚, Å. à¯ÔÂÍÓ‚‡, ê. äËÌ„, Ç. ÉÂ„ÂΘ‚‡, û. èÂÚÓ‚‡, Ä. òχÓ‚, Ä. çËÍÓÎÓ‚‡, Ä. 뇂ӂ, å. 䇈‡Ò͇, Ç. ÅÓÊËÌÓ‚‡, à. ãËÚ‚ËÌÂÌÍÓ, ï. ñÂÍÓ‚, à. èÂÚÍÓ‚‡, Ä. ïËÒÚÓ‚‡, ç. èÓÔÓ‚‡, ã. ÄÌ‚‡, Ñ. ÄÌ„Â΢‚‡, Ä. âÓ‰‡ÌÓ‚, à. äÂÏÂÌÒÍË, è.ä. íÓχÒ, ã. ä‡Î‡È‰ÊË‚‡ . . . .148

Clinical and genetic investigation of hereditary mÓtor and sensory neuropathy type lom (hmsnl; mim 601455) in Bulgaria I. Tournev, B. Ishpekova, R. King, V. Guergueltcheva, J. Petrova, A. Shmarov, A. Nikolova, A. Savov, M. Katzarova, V. Bojinova, I. Litvinenko, H. Tzekov, I. Petkova, A. Hristova, N. Popova, L. Aneva, D. Angelicheva, A. Jordanova, I. Kremenski, P.K. Thomas, L. Kalaydjieva . . . . . . .148

ïÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ êÛÒ /ïåëçê/ - ÍÎËÌ˘ÌË Ë „ÂÌÂÚ˘ÌË ‡ÒÔÂÍÚË à. í˙Ì‚, í. êÓ‰Ê˙Ò, Ç. ÉÂ„ÂΘ‚‡, Å. âÓÎ, ê. äËÌ„, Å. à¯ÔÂÍÓ‚‡, Ç. ÅÓÊËÌÓ‚‡, Ä. òχÓ‚, å. 䇈‡Ó‚‡, ï. ñÂÍÓ‚, è.ä. íÓχÒ, ã. ä‡Î‡È‰ÊË‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . . .151

Hereditary motor and sensory neuropathy type Russe – clinical and genetic aspects

ä‡ÓÚˉÌË Ô·ÍË Ë ÒÂÛÏÌË ÎËÔË‰Ë Ö. LJÒË΂‡, É. ÉÂ̘‚ . . . . . . . . . . . . . . . . . . . . . .154

Carotid artery plaques and blood lipids E. Vassileva, G. Genchev . . . . . . . . . . . . . . . . . . . . .154

ÄÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ ÔË ‰Âˆ‡ Ò ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ç. ÅÓÊËÌÓ‚‡, è. ÑËÏÓ‚‡, Ç. ÉÂ„ÂΘ‚‡, Ç. íÓÏÓ‚, í. üÌÍÓ‚ . . . . . . . . . . . . . . . . . . . . . . . .158

Antiphospholipid syndrome in children with stroke V. Bojionova ,P. Dimova.,V. Gergueltcheva , V. Tomov, T Yankov . . . . . . . . . . . . . . . . . . . . . . . . .158

Y. Panov, L. Panova . . . . . . . . . . . . . . . . . . . . . . . .140

I. Tournev , R. Varon, D. Angelicheva, V. Guergueltcheva, R. Gooding, B. Youl, B. Ishpekova, R. King, M. Raycheva, Z. Kamenov, S. Cherninkova, A. Tomov, R. Petkov, V. Bojinova, M. Katzarova, A. Shmarov, I. Litvinenko, R. Tincheva, H. Tzekov, P. Borisova, B. Buzhov, J. Petrova, P.K.Thomas L. Kalaydjieva . . . . . . . . . . . . . . . . . . . . . . . . . . . . .142

I. Tournev, T Rogers, V. Guergueltcheva, B. Youl, R. King, B. Ishpekova, V. Bojinova, A. Shmarov, M. Katzarova, P.K. Thomas L. Kalaydjieva . . . . . . . . . . . . . . . . . . . . . . . . . . . .151


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology Clopidogrel – ‰ÌӄӉ˯̇ ÔÓÙË·ÍÚË͇ ̇ ·ÓÎÌË Ò ËÒıÂÏ˘ÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË ÔË Ô‡ÚÓÎÓ„Ëfl ̇ χ„ËÒÚ‡ÎÌË ÏÓÁ˙˜ÌË ‡ÚÂËË Ö. LJÒË΂‡, å. äÎËÒÛÒÍË . . . . . . . . . . . . . . . . . .163

Clopidogrel - one year follow up of patients with transient ischemic attack or stroke and large artery diseases

ëÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË ÓÚ n.tibialisq n.medianus Ë ÎÛÏ·ÓÒ‡Í‡ÎÌËÚ ‰ÂχÚÓÏË – ÌÓχÚË‚ÌË ‰‡ÌÌË ä. äÓÒÚÓ‚, à‚. èÂÚÓ‚, à‚. à‚‡ÌÓ‚‡ . . . . . . . . .165

Somatosensory evoked potentials of n. tibialis, n. medianus and lumbosacral dermatoma – normative data Kostov K., Iv. Petrov, Iv. Ivanova . . . . . . . . . . . . . .165

ë˙ı‡ÌÂÌË ̇ ÒÎÛı‡ ÔË ÓÔÂ‡ÚË‚ÌÓ Î˜ÂÌË ̇ „ÓÎÂÏË ‚ÂÒÚË·Û·ÌË ¯‚‡ÌÓÏË (Ì‚ËÌÓÏË Ì‡ n.vestibulocochlearis) Ç. ÉÂ„‡ÌÓ‚, A. ÅÛÒ‡ÒÍË, K. êÓχÌÒÍË, ä. ê‡Ì„ÂÎÓ‚, ã. çۯ‚ , à.àÎË‚ . . . . . . . . . . . . .172

Preservation of hearing in surgery of large vestibular schwannomas (acoustic neuromas)

éèàëÄçàÖ çÄ äãàçàóçà ëãìóÄà

CASE REPORT

í‡‚χÚ˘̇ ‰ËÒÂ͇ˆËfl ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl à.ëÚ‡ÈÍÓ‚, ï.å‡ÚΠ. . . . . . . . . . . . . . . . . . . . . .175

Traumatic common carotid artery dessection I. Staikov, H. Mattle . . . . . . . . . . . . . . . . . . . . . . . .175

Ñˇ„ÌÓÒÚ˘̇ ÒÚÓÈÌÓÒÚ Ì‡ Ú‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ÔË ÌÂflÒÌË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl Ö.LJÒË΂‡, å.чÒ͇ÎÓ‚ . . . . . . . . . . . . . . . . . . . .178

Diagnostic value of the transcranial Doppler sonography in unclear cerebravascular diseases

Locked-in ÒË̉ÓÏ ÔË Ô‡ˆËÂÌÚ Ò ‰ÓÎËıÓÂÍÚ‡ÁËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl à. ëÚ‡ÈÍÓ‚, Ö. LJ‚ÂÍ, ñ. ñ‡ÌÍÓ‚‡, è. ëÚ‡ÏÂÌÓ‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . . . .181

Locked-in syndrome in patient with a dolichoectasia of the basilar artery I. Staikov, E. Vavrek , Tz. Tzankoka, P. Stamenova . . . . . . . . . . . . . . . . . . . . . . . . . . . . .181

ëÛ·ÍÎËÌ˘ÌË ËÚÏ˘ÌË ÂÎÂÍÚÓ„‡Ù˘ÌË ‡Áfl‰Ë ‚ Áfl· ‚˙Á‡ÒÚ ( SREDA) – ÓÔËÒ‡ÌË ̇ ‰‚‡ ÒÎÛ˜‡fl å. ꇯ‚‡, Ö. LJÒË΂‡ . . . . . . . . . . . . . . . . . . . .184

Subclinical rhythmic electrographic discharge of adults (SREDA) in two patients

E. Vassileva, M. Klisurski . . . . . . . . . . . . . . . . . . . .163

V. Gerganov , A. Bussarsky , K. Romansky, C. Ranguelov, L. Noutchev, I.Iliev . . . . . . . . . . . . .172

E. Vassileva, M. Daskalov . . . . . . . . . . . . . . . . . . . .178

M. Rascheva, E. Vasileva . . . . . . . . . . . . . . . . . . . . .184


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

114

é·ÁÓ ÉÖçÖíàäÄ çÄ åéáöóçàü àçëìãí. I. åéçéÉÖççà áÄÅéãüÇÄçàü ÄëéñààêÄçà ë åéáöóÖç àçîÄêäí è. òÓÚÂÍÓ‚, à. í˙Ì‚, Ç. åËı‡ÈÎÓ‚‡ äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, åÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇” SUMMARY GENETICS OF STROKE. I. SINGLE GENE DISORDERS ASSOCIATED WITH STROKE P.Shotekov, I.Tournev, V.Mihaylova Stroke is not a single disease. A variety of pathophysiological processes result in disruption of cerebral blood flow followed by neuronal death and neurologic deficit. Most strokes are sporadic with no obvious patterns of Mendelian inheritance. A minority can be ascribed to a multi-system single gene disorders in which stroke is a prominent feature. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy /CADASIL/ is the most common single gene disorder with stroke as a core sign. A number of monogenic diseases due to mutations in different genes coding for proteins with diverse functions may lead to stroke by a wide range of mechanisms. Key words: single gene disorder, CADASIL, MELAS, MTHFR, homocysteine. êÖáûåÖ åÓÁ˙˜ÌËflÚ ËÌÙ‡ÍÚ Ì  ıÓÏÓ„ÂÌÌÓ Á‡·ÓÎfl‚‡ÌÂ. ê‡Á΢ÌË Ô‡ÚÓÙËÁËÓÎӄ˘ÌË ÏÂı‡ÌËÁÏË ‚Ó‰flÚ ‰Ó ̇Û¯ÂÌË ‚ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ, ÒΉ ÍÓÂÚÓ ÔÓ Ó·˘Ë ·ËÓıËÏ˘ÌË Ô˙Úˢ‡ ÒΉ‚‡ ÌÂÍÓÁ‡ ̇ ÏÓÁ˙˜ÌËÚ ÍÎÂÚÍË Ë ÔÓfl‚‡ ̇ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ. èӂ˜ÂÚÓ ËÌÒÛÎÚË Ò‡ ÒÔÓ‡‰Ë˜ÌË, ·ÂÁ ̇·Î˛‰‡‚‡Ì åẨÂÎÓ‚ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. ։̇ ÏÌÓ„Ó Ï‡Î͇ ˜‡ÒÚ ÓÚ ËÌÒÛÎÚËÚ ҇ ı‡‡ÍÚÂ̇ ÍÎËÌ˘̇ ËÁfl‚‡ ̇ ÏÛÎÚËÒËÒÚÂÏÌË ÏÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl. ñÂ·‡Î̇ڇ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ‡ÚÂËÓÔ‡ÚËfl Ò˙Ò ÒÛ·ÍÓÚË͇ÎÌË ËÌÙ‡ÍÚË Ë Î‚ÍÓÂ̈ÂÙ‡ÎÓÔ‡ÚËfl /CADASIL/  ̇È-˜ÂÒÚÓÚÓ ÏÓÌÓ„ÂÌÌÓ Á‡·ÓÎfl‚‡ÌÂ Ò ËÌÒÛÎÚË Í‡ÚÓ ÓÒÌӂ̇ ÍÎËÌ˘̇ ÔÓfl‚‡. ë˙˘ÂÒÚ‚Û‚‡Ú Ë ‰Û„Ë ÏÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl Ò‚˙Á‡ÌË Ò ÏÛÚ‡ˆËË ‚ ‡Á΢ÌË „ÂÌË, ÍÓ‰Ë‡˘Ë ·ÂÎÚ˙ˆË Ò ‡ÁÌÓÓ·‡ÁÌË ÙÛÌ͈ËË, ÍÓËÚÓ ÔÓ ‡Á΢ÌË Ô‡ÚÓ„ÂÌÂÚ˘ÌË ÏÂı‡ÌËÁÏË Ò˙˘Ó ‚Ó‰flÚ ‰Ó ÔÓfl‚‡ ̇ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ. äβ˜Ó‚Ë ‰ÛÏË: ÏÓÌÓ„ÂÌÌÓ Á‡·ÓÎfl‚‡ÌÂ, CADASIL, MELAS, MTHFR, ıÓÏÓˆËÒÚÂËÌ. àχ ÌflÍÓÎÍÓ Á‡·ÓÎfl‚‡ÌËfl Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ, ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ, ï-Ò‚˙Á‡ÌÓ Û̇ÒΉfl‚‡ÌÂ, ͇ÍÚÓ Ë ·˙ÁÓ Û̇ÒΉfl‚‡Ì /ÏËÚÓıÓ̉ˇÎÌÓ/, ÔË ÍÓËÚÓ ËÌÒÛÎÚ˙Ú Â ı‡‡ÍÚÂ̇ ÔÓfl‚‡ ̇ ·ÓÎÂÒÚÚ‡. íÂÁË Á‡·ÓÎfl‚‡ÌËfl Úfl·‚‡ ‰‡ Ò ËÏ‡Ú Ô‰‚ˉ ‚ ‰ËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡ ̇ ËÌÒÛÎÚËÚ ÔË Ï·‰Ë ıÓ‡ Ò ÓÚÒ˙ÒÚ‚Ë ̇ ӷ˘‡ÈÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Ë ÓÒÓ·ÂÌÓ ÔË Ì‡Î˘ËÂÚÓ Ì‡ Ù‡ÏËÎ̇ ‡Ì‡ÏÌÂÁ‡ Á‡ ‡ÌÌÓ Ì‡˜‡ÎÓ Ì‡ ËÌÒÛÎÚË. Ç ÚÂÁË ÒÎÛ˜‡Ë ‰Ë‡„ÌÓÁ‡Ú‡  ÛÎÂÒÌÂ̇ ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ ‰ÓÔ˙ÎÌËÚÂÎÌËÚ ÔÓfl‚Ë Ì‡ ‰̇ ÒËÒÚÂÏ̇ ·ÓÎÂÒÚ. àÌÒÛÎÚ˙Ú Â ÂÁÛÎÚ‡Ú ÓÚ ‡Á΢ÌË Ô‡ÚÓÙËÁËÓÎӄ˘ÌË ÏÂı‡ÌËÁÏË ÔÓ‰ ‚ÎËflÌË ̇ ÏÛÚ‡ˆËË Á‡Òfl„‡˘Ë ‰‡‰ÂÌ „ÂÌ /‚Ê. 퇷Îˈ‡Ú‡/. íÓ‚‡ Ò‡ ͇‰ËÓÂÏ·ÓÎËÁ˙Ï, Á‡Òfl„‡Ì ̇ „ÓÎÂÏËÚÂ Ë Ï‡ÎÍËÚ ÏÓÁ˙˜ÌË Ò˙‰Ó‚Â, ıÂχÚÓÎӄ˘ÌË

ÓÚÍÎÓÌÂÌËfl, ÏÂÚ‡·ÓÎËÚÌË ‡ÁÒÚÓÈÒÚ‚‡, Á‡·ÓÎfl‚‡ÌËfl ̇ Ò˙‰ËÌËÚÂÎ̇ڇ Ú˙͇Ì, ‚Ó‰Â˘Ë ‰Ó ‰ËÒÂ͇ˆËfl ̇ ‡ÚÂˇÎÌËÚ Ò˙‰Ó‚Â Ë ‰. çflÍÓË Ì‡ÒΉÒÚ‚ÂÌË Á‡·ÓÎfl‚‡ÌËfl Ô‰‡ÁÔÓ·„‡Ú Í˙Ï ËÌÒÛÎÚ ÔÓ ÌflÍÓÎÍÓ ÏÂı‡ÌËÁχ (1,2) (퇷Îˈ‡1) ç‡È-˜ÂÒÚÓÚÓ ÏÓÌÓ„ÂÌÌÓ Á‡·ÓÎfl‚‡Ì ‚Ó‰Â˘Ó ‰Ó ËÌÒÛÎÚ Â CADASIL – ˆÂ·‡Î̇ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ‡ÚÂËÓÔ‡ÚËfl Ò˙Ò ÒÛ·ÍÓÚË͇ÎÌË ËÌÙ‡ÍÚË Ë Î‚ÍÓÂ̈ÂÙ‡ÎÓÔ‡ÚËfl /Tournier-Lasserve et al, 1993/. ᇷÓÎfl‚‡ÌÂÚÓ Ò ı‡‡ÍÚÂËÁË‡ Ò ËÌÚÂ- Ë ËÌÚ‡Ù‡ÏËÎ̇ ‚‡ˇ·ËÎÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ ÒËÏÔÚÓÏËÚÂ. Ç Ì‡È-χ˘‡·ÌËÚ ÔÓÛ˜‚‡ÌËfl Úfl  ÏÂÊ‰Û 45 Ë 50 „Ó‰ËÌË /Chabriat et al., 1995c; Dichgans et al., 1998/. èËÒÚ˙ÔË Ì‡ ÏË„Â̇ Ò ‡Û‡ Ò‡ Ô˙‚ËÚ ÍÎËÌ˘ÌË ËÁfl‚Ë Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔË ÓÍÓÎÓ 40% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ͇ÚÓ Ì‡˜‡ÎÓÚÓ Â Ì‡ 30 „Ӊ˯̇ ‚˙Á‡ÒÚ. óÂÒÚÓÚ‡Ú‡ ̇ ÏË„ÂÌÓÁÌËÚ ÔËÒÚ˙ÔË Â ‡Á΢̇ ÔË ‡Á΢ÌËÚ Ë̉˂ˉË, ‡ ̇È-˜ÂÒÚËÚ ÒËÏÔÚÓÏË Ì‡ ‡Û‡Ú‡ Ò‡ ÁËÚÂÎÌË, ÒÂÚË‚ÌË Ë ‡Ù‡Ú˘ÌË. àÌÒÛÎÚËÚ ҇ ̇È-˜ÂÒÚ‡Ú‡ ÔÓfl‚‡ ̇ ·ÓÎÂÒÚÚ‡. 燷≇‚‡Ú Ò ÏÂÊ‰Û 27 Ë 65 „Ó‰ËÌË. éÍÓÎÓ 85% ÓÚ ÒËÏÔÚÓχÚ˘ÌËÚ ԇˆËÂÌÚË Ò‡ ÔÂÊË‚ÂÎË Ú‡ÌÁËÚÓÌË ËÒıÂÏ˘ÌË ‡Ú‡ÍË ËÎË ËÌÒÛÎÚË, ͇ÚÓ 2/3 ÓÚ Úflı Ò‡ ·ÍÛ̇ÌË. çflÍÓË ÂÔËÁÓ‰Ë Ì‡ ÙÓ͇ÎÂÌ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ Ì‡ÒÚ˙Ô‚‡Ú Á‡Â‰ÌÓ Ò „·‚Ó·ÓÎËÂ, ÍÓÂÚÓ Úfl·‚‡ ‰‡ Ò ÓÚ‰ËÙÂÂ̈Ë‡ ÓÚ ÏË„ÂÌÓÁÌËÚ ÔËÒÚ˙ÔË. ç‡È-˜ÂÒÚÓ ËÌÒÛÎÚËÚ ÔË CADASIL ̇ÒÚ˙Ô‚‡Ú ÔË ÎËÔÒ‡ ̇ ËÒÍÓ‚Ë Ù‡ÍÚÓË. èË 20-30% ÓÚ Ô‡ˆËÂÌÚËÚ Á‡·ÓÎfl‚‡ÌÂÚÓ ÏÓÊ ‰‡ Ò χÌËÙÂÒÚË‡ Ò ÔÒËı˘ÂÒÍË Ì‡Û¯ÂÌËfl – ‰ÂÔÂÒËfl, ÔÓÌflÍÓ„‡ ‰ۂ‡˘‡ ÒÂ Ò Ï‡ÌËfl. Ç Ú‡ÍË‚‡ ÒÎÛ˜‡Ë Ò ÔÓÒÚ‡‚fl ‰Ë‡„ÌÓÁ‡Ú‡ ·ËÔÓÎflÌÓ ‡ÙÂÍÚË‚ÌÓ ‡ÁÒÚÓÈÒÚ‚Ó. ä˙ÒÌÓÚÓ Ì‡˜‡ÎÓ Ì‡ ‰ÂÔÂÒË‚ÌË ÂÔËÁÓ‰Ë ËÎË Ì‡ ·ËÔÓÎflÌÓ ‡ÁÒÚÓÈÒÚ‚Ó, ÂÙ‡ÍÚÂÌÓÒÚÚ‡ ËÏ Ì‡ ΘÂÌËÂ, ‡ÒÓˆËË‡ÌÂÚÓ ËÏ Ò ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ë ‰ÓË Ò‡ÏÓ Ì‡Î˘ËÂÚÓ Ì‡ Ù‡ÏËÎ̇ ‡Ì‡ÏÌÂÁ‡ Á‡ ËÌÒÛÎÚË, ÏË„Â̇ Ò ‡Û‡ ËÎË ‰ÂÏÂ̈Ëfl, ̇·„‡Ú ÔÓ‚Âʉ‡ÌÂÚÓ Ì‡ åêí ̇ „·‚‡ ÔË ÚÂÁË Ô‡ˆËÂÌÚË. ÑÂÏÂ̈ËflÚ‡  ‚ÚÓ‡Ú‡ ÔÓ ˜ÂÒÚÓÚ‡ ÍÎËÌ˘̇ ÔÓfl‚‡. 燷≇‚‡ Ò ÔË 1/3 ÓÚ ÒËÏÔÚÓχÚ˘ÌËÚ ԇˆËÂÌÚË. Ç 90% ÓÚ ÒÎÛ˜‡ËÚ Úfl ÔÓ„ÂÒË‡ Ò ˆˉ˂Ë‡ÌÂÚÓ Ì‡ ËÌÒÛÎÚËÚÂ. ífl  ÒÛ·ÍÓÚË͇Î̇ Ò Ô‰ËÏÌÓ ÒËÏÔÚÓÏË ÓÚ ÒÚ‡Ì‡ ̇ ˜ÂÎÌËfl ‰flÎ Ë Ô‡ÏÂÚÓ‚Ë Ì‡Û¯ÂÌËfl. ÄÙ‡ÁËfl, ‡Ô‡ÍÒËfl, ‡„ÌÓÁËfl fl‰ÍÓ Ò ̇·Î˛‰‡‚‡Ú ËÎË Ò ̇·Î˛‰‡‚‡Ú Ò‡ÏÓ ‚ ̇Ô‰̇ÎËÚ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ(3). ÑÂÏÂ̈Ëfl ËÏ‡Ú Ì‡‰ 85% ÓÚ Ô‡ˆËÂÌÚËÚ ̇ ‚˙Á‡ÒÚ Ì‡‰ 65 „Ó‰ËÌË. /Dichgans et al 1998/. ëÔÓ‰ Amberta et al /2004/ ̇Û¯ÂÌËflÚ‡ ‚ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË Á‡ÔÓ˜‚‡Ú ÔÓÒÚÂÔÂÌÌÓ ÔÂ‰Ë Ì‡˜‡ÎÓÚÓ Ì‡ Ò˙‰Ó‚ËÚ Ë̈ˉÂÌÚË, Ú. Â. ÔÂËÌÒÛÎÚÌÓ. è‡ˆË‡ÎÌË Ë „ÂÌÂ‡ÎËÁË‡ÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË Ò ̇·Î˛‰‡‚‡Ú ÔË 6 ‰Ó 10% ÓÚ ÒÎÛ˜‡ËÚ /Dichgans et al 1998/. ãËÔÒ‡Ú‡ ̇ Á‡Òfl„‡Ì ̇ óåç Ë Ì‡ „˙·Ì‡˜ÌËfl ÏÓÁ˙Í /Ò ËÁÍÎ. Ò‡ÏÓ Ì‡ ‰ËÌ ÒÎÛ˜‡È/  ‚‡ÊÌÓ Ò Ó„Î‰ ̇ ‰ËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡. åêí  ÏÌÓ„Ó ‚‡ÊÂÌ ÏÂÚÓ‰ Á‡ ‰Ë‡„ÌÓÁ‡Ú‡. åêí ÔÓÏÂÌË Ò‡ ̇·Î˛‰‡‚‡ÌË ‰ÓË Ì‡ 20 „Ӊ˯̇ ‚˙Á‡ÒÚ. ëΉ 35 „Ó‰ËÌË ‚Ò˘ÍË Ô‡ˆËÂÌÚË ÌÓÒËÚÂÎË Ì‡ Ò˙ÓÚ‚ÂÚ̇ڇ ÏÛ-


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

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퇷Î.1. åÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl ‡ÒÓˆËË‡ÌË Ò ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ á‡·ÓÎfl‚‡Ì CADASIL HERNS – ıÂ‰ËÚ‡̇ ẨÓÚÂÎËÓÔ‡ÚËfl Ò ÂÚËÌÓÔ‡ÚËfl, ÌÂÙÓÔ‡ÚËfl Ë ËÌÒÛÎÚ ÅÓÎÂÒÚ MoyaMoya

ÉÂÌ/ıÓÏÓÁÓÏÂÌ ÎÓÍÛÒ, Û̇ÒΉfl‚‡Ì ĂÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Notch3 „ÂÌ 3p21.1-21.3 ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ

3p24.2-26 17q25 8q23 ëË̉ÓÏ Ì‡ Ehlers-Danlos äÓ·„ÂÌ ÚËÔ III „ÂÌ; ÚËÔ IV ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ

ëË̉ÓÏ Ì‡ Marfan

îË·ËÎËÌ 1 „ÂÌ ‡‚ÚÓÁÓÏÌÓ‰ÓÏË̇ÌÚÌÓ

èÒ‚‰ÓÍÒ‡ÌÚÓχ ·ÒÚËÍÛÏ

ABCC6/MRP6 „ÂÌ Ä‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ

ÑËÒÎËÔˉÂÏËË ê‡Á΢ÌË „ÂÌË ç‚ÓÙË·ÓχÚÓÁ‡ ÚËÔ I NFI „ÂÌ Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ

ë˙ÔӂˉÌÓ-ÍÎÂÚ˙˜Ì‡ ‡ÌÂÏËfl

ïÂÏÓ„ÎÓ·ËÌ S Ä‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ

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ÄÎÙ‡-„‡Î‡ÍÚÓÁˉ‡Á‡ Ä „ÂÌ ï-ˆÂÒË‚ÌÓ

ïÓÏÓˆËÒÚÂÌÛËfl

ñËÒÚ‡ÚËÓÌ b ÒËÌÚ‡Á‡ „ÂÌ; MTHFR ê‡Á΢ÌË

ä‡‰ËÓÏËÓÔ‡ÚËË – Ô˙‚˘ÌË Ë ‚ÚÓ˘ÌË î‡ÏËÎÌË ‰ËÒËÚÏËË èÓÚÂËÌ ë, S ‰ÂÙˈËÚ

ÑÂÙˈËÚ Ì‡ ‡ÌÚËÚÓÏ·ËÌ III î‡ÏËÎÂÌ ‡ÌÚË͇‰ËÓÎËÔËÌÓ‚ ÒË̉ÓÏ êÂÁËÒÚÂÌÚÌÓÒÚ ÒÂ˘Û ‡ÍÚË‚Ë‡Ì ÔÓÚÂËÌ ë MELAS

è‡ÚÓ„ÂÌÂÚ˘ÂÌ ÏÂı‡ÌËÁ˙Ï ç‡ÚÂÓÒÍÎÂÓÚ˘̇, ̇ÏËÎÓˉ̇ ‡Ì„ËÓÔ‡ÚËfl åËÍӇ̄ËÓÔ‡ÚËfl ̇ ÏÓÁ˙͇ ‚ ÍÓÏ·Ë̇ˆËfl Ò˙Ò Ò˙‰Ó‚‡ ÂÚËÌÓÔ‡ÚËfl è˙‚˘̇ ẨÓÚÂÎ̇ ‰ËÒÙÛÌ͈Ëfl ëÔÓÌÚ‡ÌÌË ÓÍÎÛÁËË Ì‡ ·‡Á‡ÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË Ò ÌÂflÒ̇ Ô˘Ë̇

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íÓÏ·ÓÁË ÔÓ ‚ÂÏ ̇ ÍËÁË; ÒÚÂÌÓÁË Ì‡ „ÓÎÂÏËÚ ÂÍÒÚ‡- Ë ËÌÚ‡-Í‡ÌˇÎÌË Ò˙‰Ó‚ / ‚ÚÓ˘ÌÓ Ì‡ ÙË·ÓÁ̇ ËÌÚËχÎ̇ ÔÓÎËÙÂ‡ˆËfl/ ÑÂÙˈËÚ Ì‡ ÂÌÁËÏ Ì‡ ÎËÁÓÁÓÏËÚÂ, ‚Ӊ¢ ‰Ó ̇ÚÛÔ‚‡Ì ̇ ˆÂ‡ÏˉÚËıÂÍÒÓÁˉ ‚ ËÌÚËχڇ Ë Ï‰ˇ, ‚Ó‰Â˘Ó ‰Ó ÒÚÂÒÌfl‚‡Ì ̇ ÎÛÏÂ̇ Ë ‡ÁÛ¯‡‚‡Ì ̇ ẨÓÚÂÎÌËÚ ÍÎÂÚÍË èӂ˯ÂÌË ̇ ıÓÏÓˆËÒÚÂË̇ ‚ Í˙‚Ú‡ Ë ÛË̇ڇ, ‚Ó‰Â˘Ó ‰Ó ẨÓÚÂÎ̇ ‰ËÒÙÛÌ͈Ëfl ä‡‰ËÓÂÏ·ÓÎËÁ˙Ï

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åÛÚ‡ˆËfl ‚˙‚ Ù‡ÍÚÓ èÓÚÓÏ·ÓÚ˘ÌÓ Ò˙ÒÚÓflÌË V Leiden ËÌÒÛÎÚË ‚ Ï·‰‡ ‚˙Á‡ÒÚ åÛÚ‡ˆËË ‚ ÏËÚÓıÓ̉ˇÎ̇ڇ åËÚÓıÓ̉ˇÎ̇ ‰ËÒÙÛÌ͈Ëfl Ñçä, ÏËÚÓıÓ̉ˇÎÌÓ Û̇ÒΉfl‚‡Ì ÓÔ‰ÂÎÂÌ Ò˙‰Ó‚ /Ú. ÓÚ Ï‡È͇ڇ/ ç‡ÒΉÒÚ‚Â̇ Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ ç‚ÓÎӄ˘ÌËÚ ÛÒÎÓÊÌÂÌËfl Ò‡ Ó·ËÍÌÓ‚ÂÌÓ ıÂÏÓ‡„˘̇ Ò ‚ËÒÓ͇ ÔÂÌÂÚ‡ÌÚÌÓÒÚ ‡ÒÓˆËË‡ÌË Ò ÔÛÎÏÓ̇Î̇ ‡ÚÂËÓ-‚ÂÌÓÁ̇ Ú·̄ËÂÍÚ‡ÁËfl ÙËÒÚÛ·, ̇È-˜ÂÒÚÓ Ò ̇·Î˛‰‡‚‡Ú ıÂÏÓ‡„ËË, (·ÓÎÂÒÚ Ì‡ Rendu- Osler) ÂÁÛÎÚ‡Ú Ì‡ ÄÇå ‚ ÏÓÁ˙͇, fl‰ÍÓ Ëχ ËÒıÂÏ˘ÌË ËÌÒÛÎÚË. çÂflÒ̇ Ô‡ÚÓ„ÂÌÂÁ‡ ̇ Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ Ò˙‰Ó‚ËÚ χÎÙÓχˆËË

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ÉÓÎÂÏË Ë Ï‡ÎÍË Ò˙‰Ó‚ ÉÓÎÂÏË Ë Ï‡ÎÍË Ò˙‰Ó‚ ÉÓÎÂÏË Ë Ï‡ÎÍË Ò˙‰Ó‚ ÇÂÌÓÁÌË ÚÓÏ·ÓÁË, ÓÎfl ̇ ÔÂÒËÒÚË‡˘ FO Ë ‰flÒÌÓ-Îfl‚ ¯˙ÌÚ ÔË ËÌÒÛÎÚ ÇÂÌÓÁÌË ÚÓÏ·ÓÁË ÇÂÌÓÁÌË ÚÓÏ·ÓÁË; àÌÒÛÎÚËÚ Ì ҇ ÎÓ͇ÎËÁË‡ÌË ‚ ·‡ÒÂÈÌ àÒıÂÏ˘ÌËÚ ËÌÒÛÎÚË Ò ‰˙ÎÊ‡Ú Ì‡ ÔÓÒΉˈËÚ ÓÚ Ôӂ˯ÂÌËfl Í˙‚ÂÌ ‚ËÒÍÓÁËÚÂÚ, ‚˙Á‰Û¯ÂÌ ËÎË Ô‡‡‰ÓÍÒ‡ÎÂÌ ÂÏ·ÓÎËÁ˙Ï, ÔÓ‡‰Ë ÔÛÎÏÓ̇Î̇ڇ ÙËÒÚÛ·


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

Ú‡ˆËfl ËÏ‡Ú ‡·ÌÓÏÌÓ åêí ËÁÒΉ‚‡ÌÂ. ç‡ í1-Ó·‡ÁËÚ Ò ÓÚÍË‚‡Ú ÔÛÌÍÚËÙÓÏÂÌË Ë ÌÓ‰Û·ÌË ıËÔÓ‰ÂÌÁÌË ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË Ë ·flÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚¯ÂÒÚ‚Ó. èË í2-ËÁÏÂ‚‡ÌÂÚÓ ‚ Ò˙˘ËÚ ӷ·ÒÚË ÎÂÁËËÚ ҇ ıËÔÂËÌÚÂÌÁÌË. èË Ô‡ˆËÂÌÚË ÔÓ‰ 40 „Ó‰ËÌË Ú ҇ ÒËÏÂÚ˘ÌË ‡ÁÔÓÎÓÊÂÌË Ô‰ËÏÌÓ ÔÂË‚ÂÌÚËÍÛ·ÌÓ Ë ‚ ˆÂÌÚÛÏ ÒÂÏËÓ‚‡ÎÂ. èÓ-Í˙ÒÌÓ ÎÂÁËËÚ ‚ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó Ò‡ ‰ËÙÛÁÌË /΂ÍÓ‡ÂÈÓÁ‡/ Ë ÏÓ„‡Ú ‰‡ Á‡ÒÂ„Ì‡Ú ˆflÎÓÚÓ ·flÎÓ ‚¢ÂÒÚ‚Ó, ‚ÍÎ. Ë ÒÛ·ÍÓÚË͇ÎÌËÚ U ‚·Í̇. ãÂÁËËÚ Ò ۂÂ΢‡‚‡Ú Ò ‚˙Á‡ÒÚÚ‡ Ì ҇ÏÓ ‚ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó, ÌÓ Ë ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË Ë ÒÚ‚Ó· (3). óÂÎÌËflÚ ‰flΠ ̇È-ÒËÎÌÓ Á‡Ò„̇Ú, ÒΉ‚‡Ì ÓÚ ÚÂÏÔÓ‡ÎÌËfl Ë Ô‡ËÂÚ‡ÎÌËfl ‰flÎÓ‚Â. é͈ËÔËÚ‡ÎÌËflÚ ‰flÎ Â Á̇˜ËÚÂÎÌÓ ÔÓÒ··Ó Á‡Ò„̇Ú(4). Ç˙ÁÏÓÊÌÓ Â Á‡Òfl„‡ÌÂÚÓ Ë Ì‡ corpus callosum, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÔÓÒÚ‡‚flÌ ̇ ‰Ë‡„ÌÓÁ‡Ú‡ ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡ (5). íËÔ˘ÌÓ Â Á‡Òfl„‡ÌÂÚÓ Ì‡ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó Ì‡ ‚˙̯ÌËÚ ͇ÔÒÛÎË Ë Ô‰̇ڇ ˜‡ÒÚ Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (6). ãÂÁËËÚ ‚ ÒÚ‚Ó· Ò‡ Ô‰ËÏÌÓ ‚ ÏÓÒÚ‡. é·ËÍÌÓ‚ÂÌÓ Ï‰Û·ڇ Ë ÏÂÁÂ̈ÂÙ‡ÎÓ̇ Ì ҇ Á‡Ò„̇ÚË. ìÎÚ‡ÒÓÌÓ„‡ÙËflÚ‡ ̇ ÏÓÁ˙˜ÌËÚ Ò˙‰Ó‚Â, ÂıÓ͇‰ËÓ„‡ÙËflÚ‡ Ë ÏÓÁ˙˜Ì‡Ú‡ ‡Ì„ËÓ„‡ÙËfl Ò‡ Ó·ËÍÌÓ‚ÂÌÓ ÌÓχÎÌË. ãËÍ‚ÓÌÓÚÓ ËÁÒΉ‚‡Ì Ò˙˘Ó, ÌÓ Ò‡ ÓÔËÒ‡ÌË ÓÎË„ÓÍÎÓ̇ÎÌÓÒÚ Ë ÔÎÂÈÓˆËÚÓÁ‡ (3). è‡ÚÓÎӄӇ̇ÚÓÏ˘ÌÓ Â Ì‡Îˈ Ì‡ÚÂÓÒÍÎÂÓÚ˘̇, ̇ÏËÎÓˉ̇ ‡Ì„ËÓÔ‡ÚËfl, Á‡Òfl„‡˘‡ ωˇڇ ̇ χÎÍËÚ ÏÓÁ˙˜ÌË Ò˙‰Ó‚Â, Ò „‡ÌÛ·ÂÌ ÂÓÁËÌÓÙËÎÂÌ Ï‡ÚÂˇΠ‚ ωˇڇ, ‡ÒÓˆËË‡Ì Ò ÙÓ͇ÎÌÓ ‡ÁÛ¯‡‚‡Ì ̇ „·‰ÍÓÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË. í‡ÍË‚‡ ÔÓÏÂÌË Ò ̇·Î˛‰‡‚‡Ú Ë ‚˙‚ vasa nervorum, ‡ÚÂËÓÎËÚ ̇ ÍÓʇڇ, ÏÛÒÍÛÎËÚÂ Ë ‚ËÒˆÂ‡ÎÌËÚ Ó„‡ÌË Ú.Â, ̇Îˈ  ÒËÒÚÂÏ̇ ‡ÚÂËÓÔ‡ÚËfl. ÇÒÂ Ô‡Í ‰ÂÒÚÛ͈ËflÚ‡ ̇ „·‰ÍÓÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË ‚ ÔÂËÙÂÌËÚ Ò˙‰Ó‚  ÔÓ-χÎÍÓ ËÁ‡ÁÂ̇ (1). ᇠ‰Ë‡„ÌÓÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ÏÓÊ ‰‡ ÔÓÏÓ„ÌÂ Ë ÂÎÂÍÚÓÌÌÓÏËÍÓÒÍÓÔÒÍÓÚÓ ËÁÒΉ‚‡Ì ̇ ‡ÚÂËÓÎË ÔË ·ËÓÔÒËfl ̇ ÍÓʇ (7,8). Ç Ï‰ˇڇ ̇·Î˛‰‡‚‡ÌËflÚ „‡ÌÛÓ·ÂÌ ÓÒÏËÓÙËÎÂÌ Ï‡ÚÂˇΠ ÏÌÓ„Ó ÒÔˆËÙ˘ÂÌ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÌÓ Ì ‚Ë̇„Ë Ò ÓÚÍË‚‡ ËÎË Â ÌÂÓ·ıÓ‰ËÏÓ ÏÌÓ„Ó ÔÓ‰Ó·ÌÓ ËÁÒΉ‚‡Ì (9). Ç ÔÓÒΉÌËÚ „Ó‰ËÌË Ò ‚˙‚‰ ËÏÛÌÓıËÒÚÓıËÏ˘ÂÒÍÓ ËÁÒΉ‚‡Ì Á‡ Notch3 ‚ ÍÓÊ̇ ·ËÓÔÒËfl. í‡ÁË ÚÂıÌË͇  ‚ËÒÓÍÓ ÒÔˆËÙ˘̇ Ë ÔÓ-ÚӘ̇ ‚ Ò‡‚ÌÂÌËÂ Ò ÂÎÂÍÚÓÌ̇ڇ ÏËÍÓÒÍÓÔËfl (10,11). ᇷÓÎfl‚‡ÌÂÚÓ Â „ÂÌÂÚ˘ÌÓ ıÓÏÓ„ÂÌÌÓ - ÔË ‚Ò˘ÍË Ù‡ÏËÎËË Ò ÓÚÍË‚‡Ú ÏÛÚ‡ˆËË ‚ Notch3 „ÂÌ. ÉÂÌÌËflÚ ÎÓÍÛÒ Â 19p13.2-p13.1. çÓχÎÌËflÚ ÔÓ‰ÛÍÚ Ì‡ „Â̇  Ú‡ÌÒÏÂÏ·‡ÌÂÌ ˆÂÔÚÓ Ò‚˙Á‡Ì Ò ÍÎÂÚ˙˜Ì‡Ú‡ ‰ËÙÂÂ̈ˇˆËfl ÔÓ ‚ÂÏ ̇ ÂÏ·ËÓ̇ÎÌÓÚÓ ‡Á‚ËÚËÂ. íÓÈ Ëχ ÂÍÒÚ‡ˆÂÎÛ·ÂÌ ÎË„‡Ì‰-Ò‚˙Á‚‡˘ Û˜‡ÒÚ˙Í ÓÚ 34 Öêî/ÂÔˉÂχÎÂÌ ‡ÒÚÂÊÂÌ Ù‡ÍÚÓ/-ÔÓ‰Ó·ÌË ÔÓ‚ÚÓË, ÔÂÍÓÒfl‚‡ ‰ÌÓÍ‡ÚÌÓ ÏÂÏ·‡Ì‡Ú‡ Ë Ëχ ËÌÚ‡ˆÂÎÛ·ÂÌ ‰ÓÏÂÌ ÌÂÓ·ıÓ‰ËÏ Á‡ ÍÎÂÚ˙˜Ì‡Ú‡ Ò˄̇ÎËÁ‡ˆËfl (12). ÇÂÓflÚÌÓ Notch3 Ò˄̇ÎËÁ‡ˆËflÚ‡  ‚‡Ê̇ Á‡ ÔÓ‰‰˙ʇÌ ̇ Ò˙‰Ó‚ËÚ „·‰ÍÓÏÛÒÍÛÎÌË ÍÎÂÚÍË ‚ ‰ËÙÂÂ̈Ë‡ÌÓ Ò˙ÒÚÓflÌË (1). èË CADASIL Ëχ ÒËÒÚÂÏ̇ ‡ÚÂËÓÔ‡ÚËfl, ÍÓflÚÓ ÒÔˆËÙ˘ÌÓ Á‡Òfl„‡ χÎÍËÚ ÏÓÁ˙˜ÌË Ò˙‰Ó‚Â. íÂÊÍÓ Á‡Ò„̇ÚË Ò‡ „·‰ÍÓÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË Ì‡ ωËflÚ‡, ‚˙ÔÂÍË ˜Â Ò‡ ÓÔËÒ‡ÌË Ë ÔÓÏÂÌË ‚ ẨÓÚ·. íÂÁË ‰ËÙÛÁÌË ÔÓÏÂÌË ‚Ó‰flÚ ‰Ó ̇χÎÂÌË ̇ ÏÓÁ˙˜Ì‡Ú‡ ÔÂÙÛÁËfl Ë Ì‡Û¯Â-

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ÌË ‚ ‡‚ÚÓ„Û·ˆËflÚ‡ ̇ ÏÓÁ˙˜ÌËÚ Ò˙‰Ó‚Â. íÓ‚‡ Ò ÔÓ‰ÍÂÔfl Ë ÓÚ Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ÏÂÚÓ‰Ë, ÍÓËÚÓ ÔÓ͇Á‚‡Ú ̇χÎÂ̇ ÔÂÙÛÁËfl Ë Ì‡Û¯ÂÌ ‚‡ÁӉ˷ڇÚÓÂÌ ÓÚ„Ó‚Ó Í˙Ï ‚˙„ÎÂÓ‰ÌËfl ‰ËÓÍÒˉ ËÎË ‡ˆÂÚ‡ÁÓ·Ïˉ. (13) ÖÍÁÓÌ 4 ̇ Notch3 „Â̇  Ô‰ËÎÂ͈ËÓÌÌÓÚÓ ÏflÒÚÓ Á‡ ÏÛÚ‡ˆËË /70% ÓÚ ÒÎÛ˜‡ËÚÂ/. (14) ÖÚÓ Á‡˘Ó ÔË ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì Ò Ú˙ÒflÚ ÏÛÚ‡ˆËË Ì‡È̇Ô‰ ‚ ÚÓÁË ÂÍÁÓÌ /Markus et al/ Ë ‡ÍÓ Ì Ò ÓÚÍËflÚ Ú‡ÍË‚‡, ÒΉ‚‡ ‡Ì‡ÎËÁ ̇ ÓÒڇ̇ÎËÚ ÂÍÁÓÌË, ÍÓËÚÓ ÍÓ‰Ë‡Ú ‚Ò˘ÍË Öêî-ÔÓ‰Ó·ÌË ÔÓ‚ÚÓË. èÓ ÚÓÁË Ì‡˜ËÌ Ò ÓÚÍË‚‡Ú ÏÛÚ‡ˆËË ‚ ̇‰ 95% ÓÚ ÒÎÛ˜‡ËÚÂ. éÍÓÎÓ 95% ÓÚ ÏÛÚ‡ˆËËÚ ҇ ÏËÒ˙ÌÒ, ‚Ó‰Â˘Ë ‰Ó Á‡„Û·‡ ËÎË ‰Ó·‡‚flÌ ̇ ˆËÒÚÂËÌÓ‚ ÓÒÚ‡Ú˙Í ‚ ‰ËÌ ÓÚ 34Ú Öêî-ÔÓ‰Ó·ÌË Û˜‡ÒÚ˙ˆË ̇ ·ÂÎÚ˙͇ (15). ÇÒÂÍË Öêî-ÔÓ‰Ó·ÂÌ ‰ÓÏÂÌ Ò˙‰˙ʇ ÚË ˜ËÙÚ‡ ‰ËÒÛÎÙˉÌË /ˆËÒÚÂË̈ËÒÚÂËÌ/ ‚˙ÁÍË. åÛÚ‡ˆËËÚ (ÏËÒ˙ÌÒ, ÌÓ Ë ÓÔËÒ‡ÌËÚ ÏÛÚ‡ˆËË, ÍÓËÚÓ Ì‡Û¯‡‚‡Ú ÒÔ·ÈÒËÌ„‡ Ë ‰ÂΈËËÚÂ, ÌÂ̇Û¯‡‚‡˘Ë ‡Ï͇ڇ ̇ ˜ÂÚÂÌÂ, Joutel et al. 2000, Dichgans et al. 2000) ‚Ó‰flÚ ‰Ó ̘ËÙÚÂÌ ·ÓÈ Ì‡ ˆËÒÚÂËÌÓ‚ËÚ ÓÒÚ‡Ú˙ˆË ‚ ‰‡‰ÂÌ Öêî-ÔÓ‰Ó·ÂÌ Û˜‡ÒÚ˙Í, ÔÓ ÚÓÁË Ì‡˜ËÌ Ì‡È-‚ÂÓflÚÌÓ Ò ÔÓÏÂÌfl ÚÂÚ˘̇ڇ ÒÚÛÍÚÛ‡ ̇ ·ÂÎÚ˙͇ ÍÓ‰Ë‡Ì ÓÚ Notch3 „Â̇ (16). Ç˙ÔÂÍË Ú‡ÁË ÒÚÂÂÓÚËÔÌÓÒÚ Ì‡ ÏÛÚ‡ˆËËÚÂ, ͇ÍÚÓ ÔÓ-„Ó ·Â¯Â ÓÚ·ÂÎflÁ‡ÌÓ, Ò ̇·Î˛‰‡‚‡ ËÁ‡ÁÂ̇ ‚‡ˇ·ËÎÌÓÒÚ ‚˙‚ ÙÂÌÓÚËÔ‡. çflÍÓË Ô‡ˆËÂÌÚË ‡Á‚Ë‚‡Ú ËÌÒÛÎÚ ‚ Ï·‰‡ ‚˙Á‡ÒÚ /‚ÚÓ‡Ú‡ ‰Â͇‰‡/, ‰Ó͇ÚÓ ‰Û„Ë ‚ ¯ÂÒÚ‡Ú‡ ‰Â͇‰‡. Ç˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ ‰ÂÏÂ̈ËflÚ‡ Ò˙˘Ó  ‡Á΢̇. åË„Â̇ڇ, ÍÓflÚÓ Â ˜ÂÒÚ‡ ÔÓfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, Ò˙˘Ó ÏÓÊ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË ‰‡ ÎËÔÒ‚‡. ê‰ˈ‡ ÔÓÛ˜‚‡ÌËfl ‚˙ıÛ „ÂÌÓÚËÔ-ÙÂÌÓÚËÔ ÍÓ·ˆËË ÔË CADASIL, ÔÓ͇Á‚‡Ú ˜Â ÏflÒÚÓÚÓ Ì‡ ÏÛÚ‡ˆËflÚ‡ ‚ Notch3 Ëχ ÌÂÁ̇˜ËÚÂÎÌÓ ‚ÎËflÌË ‚˙ıÛ ÙÂÌÓÚËÔ‡ (ËÁÍβ˜ÂÌË Ô‡‚Ë C455R ÏÛÚ‡ˆËflÚ‡ ‚ ÎË„‡Ì‰-Ò‚˙Á‚‡˘Ëfl Û˜‡ÒÚ˙Í, ÍÓflÚÓ Â ‡ÒÓˆËË‡Ì‡ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ì‡ ËÌÒÛÎÚ Arboleda-Velasquez et al., 2002). íÓ‚‡ Ô‰ÔÓ·„‡ ̇΢ˠ̇ ‰Û„Ë ÏÓ‰ÛÎË‡˘Ë ·ÓÎÂÒÚÚ‡ Ù‡ÍÚÓË, ͇ÍÚÓ „ÂÌÂÚ˘ÌË, ڇ͇ Ë Ù‡ÍÚÓË Ì‡ ÓÍÓÎ̇ڇ Ò‰‡. ã‡ÍÛ̇ÌËÚ ËÌÒÛÎÚË Ò‡ ÂÁÛÎÚ‡Ú ÓÚ ÓÒÚÓ Ì‡Û¯ÂÌË ̇ ÏÓÁ˙˜Ì‡Ú‡ ÔÂÙÛÁËfl Ë/ËÎË ÚÓÏ·ÓÁ‡ ̇ Í‡ÈÌËÚ ۘ‡ÒÚ˙ˆË ̇ ÔÂÙÓ‡ÌÚÌËÚ ‡ÚÂËË. ëΉӂ‡ÚÂÎÌÓ, ÏÓÊ ‰‡ Ò Ô‰ÔÓÎÓÊË, ˜Â ‚ÒÂÍË Ù‡ÍÚÓ Á‡ÒË΂‡˘ ‰ËÙÛÁ̇ڇ ‡ÚÂËÓÔ‡ÚËfl ËÎË ‚Ӊ¢ ‰Ó Û‚Â΢Â̇ ÒÍÎÓÌÌÓÒÚ Í˙Ï ÚÓÏ·ÓÁ‡ ÏÓÊ ‰‡ Ò ‡ÒÓˆËË‡ Ò ÔÓ-‡ÌÌÓ Ì‡˜‡ÎÓ Ì‡ ËÌÒÛÎÚË ÔË Ô‡ˆËÂÌÚË Ò CADASIL. ÑËÙÛÁÌËÚ ËÒıÂÏ˘ÌË Û‚Â‰Ë /΂ÍÓ‡ÂÈÓÁ‡/ ÏÓÊ ‰‡ Ò‡ Ò‚˙Á‡ÌË Ò ‡ÁÌÓÓ·‡ÁÌË Ù‡ÍÚÓË, ÍÓËÚÓ Û‚Â΢‡‚‡Ú ÒÍÎÓÌÌÓÒÚÚ‡ Í˙Ï Ì‚Ó̇Î̇ Û‚‰‡ /̇Ô. apoE „ÂÌÓÚËÔ˙Ú/. Ö‰ÌÓ ÓÚ ÔÓÒΉÌËÚ ÔÓÛ˜‚‡ÌËfl(13) ‚˙ıÛ ‚ÎËflÌËÂÚÓ Ì‡ „ÂÌÂÚ˘ÌË Ë Í‡‰ËÓ‚‡ÒÍÛ·ÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË ‚˙ıÛ ÙÂÌÓÚËÔ‡ ÔË 127 Ô‡ˆËÂÌÚË Ò CADASIL ÓÚ 65 Ù‡ÏËÎËË Ò 17 ‡Á΢ÌË ÏÛÚ‡ˆËË ÔÓ͇Á‚‡ ÒΉÌËÚ ÂÁÛÎÚ‡ÚË: ÎËÔÒ‡ ̇ ‡ÒӈˇˆËfl ÏÂÊ‰Û ÍÓÌ‚Â̈ËÓ̇ÎÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË, ͇ÚÓ ıËÔÂÚÓÌËfl, ıÓÎÂÒÚÂÓÎ Ë Ú˛Ú˛ÌÓÔÛ¯ÂÌ ‚˙ıÛ ÚÂÊÂÒÚÚ‡ ̇ ΂ÍÓ‡ÂÈÓÁ‡Ú‡ ÔË åêí ËÁÒΉ‚‡ÌÂÚÓ. ç‡Îˈ  ӷ‡˜Â Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û Ú˛Ú˛ÌÓÔÛ¯ÂÌÂÚÓ Ë ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ ·ÍÛ̇ÌËfl ËÌÒÛÎÚ, ͇ÚÓ ÔË ÔÛ¯‡˜ËÚ ̇˜‡ÎÓÚÓ Â ‚ ÔÓ-‡Ì̇ ‚˙Á‡ÒÚ. êÂÁÛÎÚ‡ÚËÚ ÓÚ ÚÓ‚‡ ÔÓÛ˜‚‡Ì ÔÓ‰˜ÂÚ‡‚‡Ú ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ÓÚ ÔÂÍ-

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ıÓÏÓˆËÒÚÂËÌ (ÏÂÚËÎÂÌ ÚÂÚ‡ÙˉÓÙÓ·Ú, Ë ÏÂÚËÎÍÓ·‡Î‡ÏËÌ) îË„.1 ëıÂχ ̇ ÏÂÚ‡·ÓÎËÁχ ̇ ÏÂÚËÓÌË̇

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˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

‡Úfl‚‡Ì ̇ Ú˛Ú˛ÌÓÔÛ¯ÂÌÂÚÓ ÔË ÚÂÁË Ô‡ˆËÂÌÚË. (13) CADASIL  ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Á‡·ÓÎfl‚‡ÌÂ Ò 100% ÔÂÌÂÚ‡ÌÚÌÓÒÚ Ë ‚‡ˇ·ËÎ̇ ÂÍÒÔÂÒË‚ÌÓÒÚ. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË ËÏ‡Ú ·ÓÎÂÌ Ó‰ËÚÂÎ. ãËÔÒ‡Ú‡ ̇ Ù‡ÏËÎÌÓÒÚ ÏÓÊ ‰‡ Ò ‰˙ÎÊË Ì‡ ‡Ì̇ ÒÏ˙Ú ËÎË ÌÂÔ‡‚ËÎ̇ ‰Ë‡„ÌÓÁ‡ ÔË Ó‰ËÚÂÎËÚÂ Ë ÏÌÓ„Ó fl‰ÍÓ Ì‡ ‰Â ÌÓ‚Ó ÏÛÚ‡ˆËfl /Ëχ ÓÔËÒ‡Ì Â‰ËÌ Ú‡Í˙‚ ÒÎÛ˜‡È Joutel et al 2000/. Ç ÚÂÁË ÒÎÛ˜‡Ë  ÌÂÓ·ıÓ‰ËÏÓ Ì‚ÓËÁÓ·‡Áfl‚‡˘Ó ËÁÒΉ‚‡Ì ÔË Ó‰ÒÚ‚ÂÌˈËÚÂ, ͇ÍÚÓ Ë Ú˙ÒÂÌ ̇ ÛÒÚ‡ÌÓ‚Â̇ڇ ÔË ÔÓ·‡Ì‰‡ ÏÛÚ‡ˆËfl. ÇÒflÍÓ ‰ÂÚ ̇ Ô‡ˆËÂÌÚËÚÂ Â Ò 50% ËÒÍ Á‡ Û̇ÒΉfl‚‡Ì ̇ Notch3 ÏÛÚ‡ˆËflÚ‡. èÓ ÓÚÌÓ¯ÂÌË ̇ „ÂÌÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ÔË ‡ÒËÏÔÚÓÏÌËÚ ˜ÎÂÌӂ ̇ Ù‡ÏËÎËË Ò ‰Ó͇Á‡Ì CADASIL, ÒΉ‚‡ ‰‡ Ò ÒÔ‡Á‚‡Ú Ò˙˘ËÚ Ô‡‚Ë· ͇ÚÓ ÔË ï˙ÌÚËÌ„Ú˙ÌÓ‚‡Ú‡ ıÓÂfl. èÂ̇ڇÎ̇ڇ ‰Ë‡„ÌÓÁ‡  ‚˙ÁÏÓÊ̇ ÒΉ ͇ÚÓ Â ÛÒÚ‡ÌÓ‚Â̇ ÏÛÚ‡ˆËflÚ‡ ÔË Á‡Ò„̇ÚËÚ ˜ÎÂÌӂ ̇ Ù‡ÏËÎËflÚ‡. (17) àÒıÂÏ˘ÌËflÚ ËÌÒÛÎÚ Â ˜‡ÒÚ ÓÚ ÙÂÌÓÚËÔ‡ ̇ ÏËÚÓıÓ̉ˇÎÌÓÚÓ Á‡·ÓÎfl‚‡Ì MELAS /ÏËÚÓıÓ̉ˇÎ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl, ·ÍÚ‡Ú ‡ˆË‰ÓÁ‡ Ë ËÌÒÛÎÚÓÔÓ‰Ó·ÌË ÂÔËÁÓ‰Ë/. ᇷÓÎfl‚‡ÌÂÚÓ Â ÏÛÎÚËÒËÒÚÂÏÌÓ, ͇ÚÓ Ì‡È-ı‡‡ÍÚÂÌÓ Â Ì‡Î˘ËÂÚÓ Ì‡ ËÌÒÛÎÚ ËÎË ËÌÒÛÎÚÓÔÓ‰Ó·ÌË ÂÔËÁÓ‰Ë Ó˘Â ‚ ˛ÌÓ¯ÂÒÚ‚ÓÚÓ Ò ÔÂıӉ̇ ËÎË ÔÓÒÚÓflÌ̇ ıÂÏˇÌÓÔÒËfl, ÍÓÓ‚‡ ÒÎÂÔÓÚ‡, ‡Ù‡ÁËfl ËÎË ıÂÏËÔ‡ÂÁ‡. àÌÙÂ͈ËËÚÂ Ë Ù·ËÎËÚÂÚ˙Ú Ò‡ ‚˙ÁÏÓÊÌË ÚË„ÂË‡˘Ë Ù‡ÍÚÓË. óÂÒÚÓ Ì‡·Î˛‰‡‚‡ÌË Ò‡ ÏË„ÂÌÓÔÓ‰Ó·ÌÓ „·‚Ó·ÓÎËÂ, ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË, ÔÓ‚˙˘‡ÌÂ, ÔÓ„ÂÒ˂̇ ‚˙̯̇ ÓÙÚ‡ÎÏÓÔ΄Ëfl, ÔÚÓÁ‡, ÒÂÌÁÓÌ‚‡Î̇ „ÎÛıÓÚ‡, ‡Ú‡ÍÒËfl, ‰ÂÏÂ̈Ëfl.(1,20) èË ÏÛÒÍÛÎ̇ ·ËÓÔÒËfl Ò ÓÚÍË‚‡ ÏËÚÓıÓ̉ˇÎ̇ ÏËÓÔ‡ÚËfl Ò ragged red fibres20. ì‚Â΢ÂÌË Ò‡ ÌË‚‡Ú‡ ̇ ·ÍÚ‡Ú‡ ‚ Í˙‚Ú‡, ÍÓÂÚÓ Â ÂÁÛÎÚ‡Ú Ì‡ ̇Û¯ÂÌË ‚˙‚ ÙÛÌ͈ËflÚ‡ ̇ ‰Ëı‡ÚÂÎ̇ڇ ‚ÂË„‡ Ë ËÌıË·Ë‡Ì ̇ ˆËÍ˙· ̇ ä·Ò, ÒΉÒÚ‚Ë ̇ ÍÓÂÚÓ Ò ̇ÚÛÔ‚‡Ú ÔËÛ‚‡Ú Ë Î‡ÍÚ‡Ú.(20) åÓÁ˙˜ÌËÚ ÎÂÁËË Ì‡È-˜ÂÒÚÓ Á‡Òfl„‡Ú Ó͈ËÔËÚÓ-Ô‡ËÂÚ‡ÎÌËÚ ‡ÈÓÌË, ‚Ó‰Â˘Ë ‰Ó ‰ÂÙÂÍÚË ‚ ÁËÚÂÎÌËÚ ÔÓÎÂÚ‡ Ë ˜ÂÒÚÓ Ì ÔÓ͇Á‚‡Ú ‚˙Á͇ Ò ÓÔ‰ÂÎÂÌ Ò˙‰Ó‚ ·‡ÒÂÈÌ. í ÏÓ„‡Ú ‰‡ ÔÂÚ˙ÔflÚ Ó·‡ÚÌÓ ‡Á‚ËÚË ÔË ÔÓÒΉ‚‡˘ËÚ åêí ËÁÒΉ‚‡ÌËfl (1). è˘Ë̇ڇ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò‡ ÏÛÚ‡ˆËË ‚ ÏËÚÓıÓ̉ˇÎ̇ڇ Ñçä, ÍÓËÚÓ ËÏ‡Ú Ï‡È˜ËÌÓ Û̇ÒΉfl‚‡ÌÂ. èË Ú‡ÁË „ÛÔ‡ Á‡·ÓÎfl‚‡ÌËfl Ëχ „ÓÎflχ ÙÂÌÓÚËÔ̇ ‚‡ˇ·ËÎÌÓÒÚ ‰ÓË ÔË Â‰Ì‡ Ë Ò˙˘‡ ÏÛÚ‡ˆËfl, ÔÓ‡‰Ë Ú.̇. ıÂÚÂÓÔ·ÁÏËfl /̇΢ËÂÚÓ Ì‡ ‡ÁÎË͇ ‚ ÍÓ΢ÂÒÚ‚ÓÚÓ ÏÛÚ‡ÌÚÌË Ë ÌÓχÎÌË ÏËÚÓıÓ̉ˇÎÌË Ñçä Ë ‚ ‡Á΢ÌËÚ ÍÎÂÚÍË Ë Ú˙͇ÌË ‚ ‰ËÌ Ë Ò˙˘ Ë̉˂ˉ (1,20). ç‡È-˜ÂÒÚË Ò‡ ÏËÒ˙ÌÒ ÏÛÚ‡ˆËËÚ ‚ „Â̇ ÍÓ‰Ë‡˘ Úêçä Á‡ ‡ÏËÌÓÍËÒÂÎË̇ڇ ΂ˆËÌ. ç‡È˜ÂÒÚ‡  Ú‡ÌÁˈËflÚ‡ A?G ̇ ÔÓÁˈËfl 3243 (1,20). àÌÒÛÎÚ Â Ì‡·Î˛‰‡‚‡Ì Ë ÔË ‰Û„Ë ÏÛÚ‡ˆËË ‚ ÏËÚÓıÓ̉ˇÎ̇ڇ Ñçä. åÌÓ„Ó ˜ÂÒÚÓ Ò ÔÓÎÛ˜‡‚‡Ú ÍÓÏ·Ë̇ˆËfl ÓÚ ÒËÏÔÚÓÏË ı‡‡ÍÚÂÌË Á‡ ‰‚‡ ËÎË Ôӂ˜ ÒË̉Óχ, Ú.̇. overlap ÒË̉ÓÏË. ç‡ÔËÏÂ ÍÓÏ·Ë̇ˆËfl ÓÚ MELAS Ë MERRF, MELAS Ë Kearns-Sayre Syndrome.(20) åËÚÓıÓ̉ˇÎ̇ڇ Ñçä ÍÓ‰Ë‡ ˜‡ÒÚ ÓÚ ·ÂÎÚ˙ˆËÚ ̇ ‰Ëı‡ÚÂÎ̇ڇ ‚ÂË„‡. ç‡È-‚ÂÓflÚÌÓ ËÌÒÛÎÚËÚ ÔË ÏËÚÓıÓ̉ˇÎÌËÚ Á‡·ÓÎfl‚‡ÌËfl Ò‡ ÂÁÛÎÚ‡Ú Ì‡ ‚ÌÂÁ‡ÔÌÓ Ì‡Û¯ÂÌË ̇ ÏÂÚ‡·ÓÎËÁχ ‚ ÌÂ‚̇ڇ Ú˙Í‡Ì Ò „ÓÎflÏÓ ÍÓ΢ÂÒÚ‚Ó ÏÛÚ‡ÌÚÌË ÏËÚÓıÓ̉ˇÎÌË ÑçäË, ÔÓÒΉ‚‡ÌÓ ÓÚ Á‡„Û·‡ ̇ ÙÛÌ͈Ëfl Ë ÔÂıӉ̇ ËÎË ÔÓÒÚÓflÌ̇ ÍÎÂÚ˙˜Ì‡ Û‚‰‡, ‚ÂÓflÚÌÓ ÚË„ÂË‡ÌÓ ÓÚ Ù·ËÎËÚÂÚ ËÎË ËÌÙÂ͈Ëfl. íÓ‚‡ Ó·flÒÌfl‚‡ Á‡˘Ó ÎÂÁËËÚ Ì ҇ Ó„‡Ì˘ÂÌË ‚ ‰ËÌ Ò˙‰Ó‚ ·‡ÒÂÈÌ. éÒ‚ÂÌ ÚÓ‚‡ ÏËÚÓıÓ̉ˇÎ̇ڇ ‰ËÒÙÛÌ͈Ëfl ÏÓÊ ‰‡ Á‡Òfl„‡ ÔÓˆÂÒËÚ ̇ ͇ΈË‚ÓÚÓ Ò˄̇ÎËÁË‡ÌÂ, ͇ÍÚÓ Ë ‡ÍÚË‚Ë‡ÌÂÚÓ Ì‡ ‡ÔÓÔÚÓÁ‡Ú‡ (20). MELAS Ò Û̇ÒΉfl‚‡ ÓÚ Ï‡È͇ڇ, Ú. ·ÓÎÂÌ ·‡˘‡ Ì ÏÓÊ ‰‡ Ô‰‡‰Â ÏÛÚ‡ˆËflÚ‡ ̇ Ò‚ÓÂÚÓ ÔÓÚÓÏÒÚ‚Ó. åÛÚ‡ˆËfl Ó·ËÍÌÓ‚ÂÌÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË Ï‡È͇ڇ ̇ ÔÓ·‡Ì‰‡, ÍÓflÚÓ ÏÓÊ ‰‡ Ëχ ËÎË Ìflχ ÒËÏÔÚÓÏË.èË Á‡·ÓÎfl‚‡-

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ÌÂÚÓ Ì  ‚˙ÁÏÓÊ̇ ÔÂ̇ڇÎ̇ ‰Ë‡„ÌÓÁ‡, ÔÓ‡‰Ë ÌflÍÓË ÓÒÓ·ÂÌÓÒÚË ÔË ÏËÚÓıÓ̉ˇÎÌËÚ Á‡·ÓÎfl‚‡ÌËfl (21). àχ ÌflÍÓÎÍÓ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌË Ë ˆÂÒË‚ÌË Á‡·flÎfl‚‡ÌËfl Ò ‡Á΢ÌË ÂÌÁËÏÌË ‰ÂÙˈËÚË, ÍÓËÚÓ ‚Ó‰flÚ ‰Ó Ôӂ˯ÂÌË ÌË‚‡ ̇ ıÓÏÓˆËÒÚÂËÌ ‚ Ô·Áχڇ Ë ÛË̇ڇ, Ú.̇. ıÓÏÓˆËÒÚÂËÌÛËfl. íÓ‚‡ Úfl·‚‡ ‰‡ Ò ÓÚ‰ËÙÂÂ̈Ë‡ ÓÚ ÎÂ͇ ıËÔÂıÓÏÓˆËÒÚÂËÌÂÏËfl ·ÂÁ ıÓÏÓˆËÒÚÂËÌÛËfl, ÍÓÂÚÓ Â ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ÒÔÓ‡‰Ë˜ÌËfl ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ (1). ïÓÏÓˆËÒÚÂËÌ˙Ú Â Òfl‡Ò˙‰˙ʇ˘‡ ‡ÏËÌÓÍËÒÂÎË̇, ÔÓ‰ÛÍÚ Ì‡ ÏÂÚ‡·ÓÎËÁχ ̇ ÏÂÚËÓÌË̇. àχ ‰‚‡ Ô˙Úfl ̇ ËÌÚ‡ˆÂÎÛ·ÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï Ì‡ ıÓÏÓˆËÒÚÂË̇ – Ú‡ÌÒÛÎÙÛË‡Ì ‰Ó ˆËÒÚÂËÌ Ë ÂÏÂÚËÎË‡Ì ‰Ó ÏÂÚËÓÌËÌ (‚Ê. ÒıÂχڇ). èÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ ıÓÏÓˆËÒÚÂË̇ Ò ÔÂÓ·‡ÁÛ‚‡ ‚ ˆËÒÚÂËÌ Ò Û˜‡ÒÚËÂÚÓ Ì‡ ÂÌÁËχ ˆËÒÚ‡ÚËÓÌËÌ ·ÂÚ‡ ÒËÌÚÂÚ‡Á‡, ÍÓÈÚÓ Â ‚ËÚ‡ÏËÌ Ç6 Á‡‚ËÒËÏ. ÑÛ„ËflÚ ÏÂÚ‡·ÓÎËÚÂÌ Ô˙Ú /ÂÏÂÚËÎË‡Ì ‰Ó ÏÂÚËÓÌËÌ/ Ò ÓÒ˙˘ÂÒÚ‚fl‚‡ ÓÚ ÏÂÚËÓÌËÌ ÒËÌÚ‡Á‡, ÍÓÈÚÓ ËÁÔÓÎÁ‚‡ ÏÂÚËÎÂÌÚÂÚ‡ıˉÓÙÓÎ‡Ú Ë ÏÂÚËÎÍÓ·‡Î‡ÏËÌ Í‡ÚÓ ÍÓÙ‡ÍÚÓË. ê‡Á΢ÌË ÂÌÁËÏÌË ‰ÂÙˈËÚË, ̉ÓËÏ˙˜ÌË Ò˙ÒÚÓflÌËfl, ÚÓÍÒËÌË Ë ÎÂ͇ÒÚ‚‡ /̇Ô. ÚÂÓÙËÎËÌ, ÙÂÌËÚÓËÌ/ ÏÓ„‡Ú ‰‡ Ô‰ËÁ‚ËÍ‡Ú ıËÔÂıÓÏÓˆËÒÚÂËÌÂÏËfl. ÑÂÙˈËÚ Ì‡ ÚË ÂÌÁËχ - ˆËÒÚ‡ÚËÓÌËÌ ·ÂÚ‡ ÒËÌÚÂÚ‡Á‡, ÏÂÚËÎÂÌ ÚÂÚ‡ÙˉÓÙÓÎ‡Ú ‰ÛÍÚ‡Á‡ /MTHFR/ Ë ÏÂÚËÓÌËÌ ÒËÌÚ‡Á‡ ‚Ó‰flÚ ‰Ó ıËÔÂıÓÏÓˆËÒÚÂËÌÂÏËfl, ͇ÍÚÓ Ë Ì‰ÓËÏ˙Í Ì‡ ÍÓ·‡Î‡ÏËÌ /‚ËÚ. Ç12/, ÔËˉÓÍÒËÌ /‚ËÚ. Ç6/ Ë ÙÓÎË‚‡ ÍËÒÂÎË̇ (18). ç‡È-˜ÂÒÚËflÚ ÂÌÁËÏÂÌ ‰ÂÙˈËÚ Â Ì‡ ˆËÒÚ‡ÚËÓÌËÌ ·ÂÚ‡ ÒËÌÚÂÚ‡Á‡, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó Ôӂ˯ÂÌÓ ÌË‚Ó Ì‡ ıÓÏÓˆËÒÚÂËÌ, ÏÂÚËÓÌËÌ Ë ıÓÏÓˆËÒÚÂËÌ-ˆËÒÚÂËÌ ‰ËÒÛÎÙˉ. ïÓÏÓˆËÒÚÂËÌ˙Ú Â ÚÓÍÒ˘ÂÌ Á‡ ÂÌÚÓÚÂÎÌËÚ ÍÎÂÚÍË Ë ‚Ó‰Ë ‰Ó ÔÓÚÓÏ·ÓÚ˘ÌÓ Ò˙ÒÚÓflÌËÂ, ÓÒ‚ÂÌ ÚÓ‚‡  ‡ÒÓˆËË‡Ì Ò ÔÂʉ‚ÂÏÂÌ̇ ‡ÚÂÓÒÍÎÂÓÁ‡. àÁ‚ÂÒÚÌË Ò‡ ‰‚‡ ÙÂÌÓÚËÔ‡ ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÚÂ‡Ô‚Ú˘ÌËfl ÓÚ„Ó‚Ó ÒÔflÏÓ ÔËˉÓÍÒËÌ – ‚ËÚ. Ç6 ÓÚ„Ó‚‡fl˘‡ Ë ‚ËÚ. Ç6 ÌÂÓÚ„Ó‚‡fl˘‡ ıÓÏÓˆËÒÚÂËÌÂÏËfl. èË ÔÓÒΉÌËÚ ÍÎËÌ˘̇ڇ ͇ÚË̇  ÔÓ-ÚÂÊ͇ (1). ᇷÓÎfl‚‡ÌÂÚÓ Ò ı‡‡ÍÚÂËÁË‡ Ò ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl, ‰ËÒÎÓ͇ˆËfl ̇ ΢ËÚ Ë/ËÎË ÚÂÊ͇ ÏËÓÔËfl, ÒÍÂÎÂÚÌË ‡·ÌÓÏÌÓÒÚË /χÙ‡ÌÓÔÓ‰Ó·ÌË ÔÓfl‚Ë/ Ë ÚÓÏ·ÓÂÏ·ÓÎËÁ˙Ï, ÍÓÈÚÓ Â Ô˘Ë̇ Á‡ ‡Ì̇ ÒÏ˙Ú Ë ·ÓÎÂÒÚÌÓÒÚ. àÌÒÛÎÚËÚ Ò ÔÓfl‚fl‚‡Ú ‚ Ï·‰‡ ‚˙Á‡ÒÚ, ‡ ·ÂÏÂÌÌÓÒÚÚ‡ Û‚Â΢‡‚‡ ËÒ͇ Ë ÓÒÓ·ÂÌÓ ‚ ÔÛÂÔÂËÛχ. ᇷÓÎfl‚‡ÌÂÚÓ Ò Û̇ÒΉfl‚‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ. ÉÂÌ˙Ú ÍÓ‰Ë‡˘ ˆËÒÚ‡ÚËÓÌËÌ ·ÂÚ‡ ÒËÌÚÂÚ‡Á‡Ú‡  ͇ÚË‡Ì ̇ 21q22.3. àÁ‚ÂÒÚÌË Ò‡ ̇‰ 100 ÏÛÚ‡ˆËË, ÍÓËÚÓ Ô‰ËÁ‚ËÍ‚‡Ú Á‡·ÓÎfl‚‡ÌÂÚÓ. èӂ˜ÂÚÓ Á‡Òfl„‡Ú ‡ÍÚË‚ÌËfl ˆÂÌÚ˙ ̇ ÂÌÁËχ (19). çflÍÓË ÏÛÚ‡ˆËË Ò‡ ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-˜ÂÒÚË. åÛÚ‡ˆËËÚ I278T Ë A114V Ò Ò¢‡Ú ˜ÂÒÚÓ ÔË ‚ËÚ. Ç6 ÓÚ„Ó‚‡fl˘ËÚÂ, ‰Ó͇ÚÓ ÏÛÚ‡ˆËflÚ‡ G307S Ë A1224-2C, ˜ÂÒÚÓ Ò¢‡ÌË ‚ Ò‚ÂÌÓ‚ÓÔÂÈÒÍËÚ ÔÓÔÛ·ˆËË, Ò‡ ‡ÒÓˆËË‡ÌË Ò ÂÁËÒÚÂÌÚÌÓÒÚ Í˙Ï Î˜ÂÌËÂ Ò ‚ËÚ. Ç6 (1). Ç ÌflÍÓË ÒÚ‡ÌË Â ‚˙‚‰ÂÌ ÌÂÓ̇ڇÎÂÌ ÒÍËÌËÌ„ Á‡ ıËÔÂÏÂÚËÓÌËÌÂÏËfl /ÔÓ‰Ó·ÂÌ Ì‡ ÔÓÁ̇ÚËfl ÌË ÚÂÒÚ Ì‡ Guthrie/, ˜ÂÁ ÍÓÂÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ıÓÏÓˆËÒÚÂËÌÂÏËfl, ‰˙Îʇ˘‡ Ò ҇ÏÓ Ì‡ ‰ÂÙˈËÚ Ì‡ ˆËÒÚ‡ÚËÓÌËÌ ·ÂÚ‡ ÒËÌÚÂÚ‡Á‡Ú‡, ÌÓ ÌÂ Ë Ì‡ ‰ÂÙÂÍÚË ‚ ÂÏÂÚËÎË‡ÌÂÚÓ, Á‡˘ÓÚÓ ÚÓ„‡‚‡ ÏÂÚËÓÌËÌ˙Ú Ì  Ôӂ˯ÂÌ /‚Ê. ëıÂχڇ/. Ç˙ÁÏÓÊ̇  ÔÂ̇ڇÎ̇ ‰Ë‡„ÌÓÁ‡ ˜ÂÁ ËÁÏÂ‚‡Ì ̇ ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ ÂÌÁËχ ‚ ÍÛÎÚË‚Ë‡ÌË ‡ÏÌËÓˆËÚË ËÎË ËÁÏÂ‚‡Ì ̇ ıÓÏÓˆËÒÚÂË̇ ‚ ‡ÏÌËÓÚ˘̇ڇ Ú˜ÌÓÒÚ; Ò˙˘Ó  ‚˙ÁÏÓÊÂÌ Ë Ñçä ‡Ì‡ÎËÁ, ‡ÍÓ Ò‡ ËÁ‚ÂÒÚÌË Ë ‰‚‡Ú‡ ÏÛÚ‡ÌÚÌË ‡Î· ̇ ·ÓÎÌËfl Ë̉˂ˉ (19). ëïÖåÄ çÄ åÖíÄÅéãàáåÄ çÄ åÖíàéçàçÄ ïÂÏÓ„ÎÓ·ËÌÓÔ‡ÚËËÚÂ, ‚ÍÎ. Ò˙ÔӂˉÌÓÍÎÂÚ˙˜Ì‡Ú‡ ‡ÌÂÏËfl Ë ‡Á΢ÌËÚ ̇ÒΉÒÚ‚ÂÌË ÍÓ‡„ÛÎÓÔ‡ÚËË Ò ‡ÒÓˆËË‡Ú Ò ÚÓÏ·ÓÚ˘ÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË. ÑÂÙˈËÚ Ì‡ ÂÒÚÂ-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÒÚ‚ÂÌËÚ ‡ÌÚËÍÓ‡„Û·ÌÚË ÔÓÚÂËÌ ë Ë ÔÓÚÂËÌ S  Ô˘Ë̇ Á‡ Ù‡ÏËÎ̇ ‚ÂÌÓÁ̇ ÚÓÏ·ÓÁ‡. ÄÒӈˇˆËflÚ‡ Ò ‡ÚÂˇÎÂÌ ËÌÒÛÎÚ Â ÔÓ-Ò··‡. ÑÓ͇Á‚‡ÌÂÚÓ Ì‡ ڇ͇‚‡ Ò ÛÒÎÓÊÌfl‚‡ ÓÚ Ù‡ÍÚ‡, ˜Â ̇χÎÂÌË ÌË‚‡ ̇ ÔÓÚÂËÌ ë Ë ÔÓÚÂËÌ S Ò ÛÒÚ‡Ìfl‚fl‚‡Ú ÔÂıÓ‰ÌÓ ÒΉ ËÌÒÛÎÚ, ÌËÒÍË ÌË‚‡ Ò ÛÒÚÓÌÓ‚fl‚‡Ú ÔË ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl ͇ÚÓ ˜ÂÌÓ‰Ó·ÌË, Ñàä ÒË̉ÓÏ, ·˙·˜ÌË Á‡·ÓÎfl‚‡ÌËfl ËÎË ÔË ÚÂ‡ÔËfl Ò ‚‡Ù‡ËÌ. ëΉӂ‡ÚÂÎÌÓ Â ÌÂÓ·ıÓ‰ËÏÓ ÔÓÚ‚˙ʉ‡‚‡Ì ̇ „ÂÌÂÚ˘ÌËfl ‰ÂÙÂÍÚ ˜ÂÁ ÌÂÍÓÎÍÓÍ‡ÚÌÓ ÔÓÒΉfl‚‡Ì ̇ ÒÂÛÏÌËÚ ÌË‚‡ Ë ˜ÂÁ ËÁÒΉ‚‡Ì ̇ ÓÒڇ̇ÎËÚ Ó‰ÒÚ‚ÂÌˈË. èÓÚÓÏ·ÓÚ˘ÌËÚ Ò˙ÒÚÓflÌËfl Ò‡ ÔÂ‰Ë ‚Ò˘ÍÓ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ‡ÚÂˇÎÂÌ ËÌÒÛÎÚ ÔË Ï·‰Ë ıÓ‡, ÔË ‚˙Á‡ÒÚÌË ‚˙Á͇ڇ ÏÂÊ‰Û ÌË‚ÓÚÓ Ì‡ ÂÒÚÂÒÚ‚ÂÌËÚ ‡ÌÚËÍÓ‡„Û·ÌÚË Ë ËÌÒÛÎÚ‡  Ò··‡. éÒ‚ÂÌ ÚÓ‚‡ ËÒÍ˙Ú ÓÚ ‚ÂÌÓÁ̇ ÚÓÏ·ÓÁ‡  ÏÌÓ„Ó ÔÓ-‚ËÒÓÍ ÓÚÍÓÎÍÓÚÓ ÓÚ ‡ÚÂˇÎ̇ ÚÓÏ·ÓÁ‡ Ë ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ ÔÂÒËÒÚË‡˘ foramen ovale Ò ‰flÒÌÓ-Îfl‚ ¯˙ÌÚ Úfl·‚‡ ‰‡ Ò Ëχ Ô‰‚ˉ ÔË Ú‡ÍË‚‡ Ô‡ˆËÂÌÚË. èӂ˯ÂÌ Â Ò˙˘Ó ËÒÍ˙Ú Ë Á‡ ÏÓÁ˙˜Ì‡ ‚ÂÌÓÁ̇ ÚÓÏ·ÓÁ‡. ÑÂÙˈËÚ Ì‡ ‡ÌÚËÚÓÏ·ËÌ III, Û̇ÒΉÂÌ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ, Ò˙˘Ó ÔÂ‰Ë ‚Ò˘ÍÓ Ò ‡ÒÓˆËË‡ Ò ‚ÂÌÓÁÌË ÚÓÏ·ÓÁË, ‚˙ÔÂÍË ˜Â Ò‡ ÓÔËÒ‡ÌË Ë ÒÎÛ˜‡Ë ̇ ‡ÚÂˇÎÌË ËÌÒÛÎÚË (1). èÂÁ 1993  ÓÚÍËÚ‡ ÌÓ‚ ÚËÔ Ì‡ Ù‡ÏËÎ̇ ÚÓÏ·ÓÙËÎËfl - ÂÁËÒÚÂÌÚÌÓÒÚ Í˙Ï ‡ÍÚË‚Ë‡Ì ÔÓÚÂËÌ ë (Dahlback et al.). ç‡È-˜ÂÒÚÓ ÚÓ‚‡  ÂÁÛÎÚ‡Ú Ì‡ Ú‡ÌÁˈËflÚ‡ A➞G ̇ ÔÓÁˈËfl 1691 (Q506 Leiden) ‚ „Â̇ ÍÓ‰Ë‡˘ Ù‡ÍÚÓ V (Bertina et al. 1994). íÓ‚‡  ̇È-˜ÂÒÚ‡Ú‡ „ÂÌÂÚ˘̇ Ô˘Ë̇ Á‡ ‚ÂÌÓÁ̇ ÚÓÏ·ÓÁ‡ Ë Â ‚‡ÊÂÌ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ÏÓÁ˙˜Ì‡ ‚ÂÌÓÁ̇ ÚÓÏ·ÓÁ‡. éÔËÒ‡ÌË Ò‡ ÌflÍÓÎÍÓ Ù‡ÏËÎËË Ò Û̇ÒΉÂ̇ ÂÁËÒÚÂÌÚÌÓÒÚ Í˙Ï ‡ÍÚË‚Ë‡Ì ÔÓÚÂËÌ ë Ë ËÌÒÛÎÚ ‚ ÏÎa‰‡ ‚˙Á‡ÒÚ. åÛÚ‡ˆËfl ‚ ÌÂÍÓ‰Ë‡˘Ëfl 3'- Í‡È ̇ ÔÓÚÓÏ·ËÌÓ‚Ëfl „ÂÌ Â ‡ÒÓˆËË‡Ì‡ Ò Û‚Â΢Â̇ ÂÍÒÔÂÒËfl ̇ ·ÂÎÚ˙͇ (G20210A), ÍÓÂÚÓ Â Ò‚˙Á‡ÌÓ Ò ‚ÂÌÓÁÌË ÚÓÏ·ÓÁË (Poort et al., 1996), ÌÓ Ôӂ˜ÂÚÓ ‰‡ÌÌË Ì ÔÓ͇Á‚‡Ú ÓÎfl ̇ ÚÓÁË ‚‡ˇÌÚ ÔË ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ. çflÍÓÎÍÓ ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë ‰ÓÔÛÒ͇Ú, ˜Â ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ëfl ÏÂÊ‰Û „ÂÌËÚ ÏÓ„‡Ú ÒËÌÂ„˘ÌÓ ‰‡ Û‚Â΢‡Ú ËÒ͇ ÓÚ ‡ÚÂˇÎÌË ÚÓÏ·ÓÁË ÔË ËÌ‰Ë‚Ë‰Ë ÌÓÒËÚÂÎË Ì‡ ÔÓÚÓÏ·ÓÚ˘ÌË ÏÛÚ‡ˆËË Ë/ËÎË ıËÔÂıÓÏÓˆËÒÚÂËÌÂÏËfl (1). Ç˙ÔÂÍË ˜Â Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë Ò ‡ÌÚË͇‰ËÓÎËÔËÌÓ‚Ë ‡ÌÚËÚ· Ë ÎÛÔÛÒÌË ‡ÌÚËÍÓ‡„Û·ÌÚË Ò‡ ÒÔÓ‡‰Ë˜ÌË, ÓÔËÒ‡ÌË Ò‡ ÌflÍÓÎÍÓ Ù‡ÏËÎÌË ÒÎÛ˜‡Ë (Mackie et al., 1987). éÔËÒ‡Ì Â Ë Ù‡ÏËÎÂÌ ÒË̉ÓÏ Ì‡ ë̉˙Ì Ò ‚ÂÓflÚÌÓ ‡‚ÚÓÁÓÏÌÓ‰ÓÏË̇ÌÚÌÓ Û̇ÒΉfl‚‡Ì (Pettee et al., 1994). ç‡ÒΉÒÚ‚ÂÌËÚ ‰ËÒÎËÔˉÂÏËË Ò‡ ‡ÒÓˆËË‡ÌË Ò ÔÂʉ‚ÂÏÂÌ̇ ‡ÚÂÓÒÍÎÂÓÁ‡, ÍÓflÚÓ ‚Ó‰Ë ‰Ó ‡ÌÂÌ ËÌÒÛÎÚ. Ç˙Á͇ڇ ÏÂÊ‰Û ÚÂÁË Á‡·ÓÎfl‚‡ÌËfl Ë ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ Â ÔÓ-ÌÂflÒÌÓ ÓÔ‰ÂÎÂ̇ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÓ̇̇ڇ ·ÓÎÂÒÚ, ÌÓ Â ÛÒÚ‡ÌÓ‚Â̇ ÔË ÌflÍÓË Á‡·ÓÎfl‚‡ÌËfl, ͇ÚÓ Ù‡ÏËÎ̇ڇ ıËÔÓ‡ÎÙ‡ÎËÔÓÔÓÚÂËÌÂÏËfl, ÚËÔ II Ë ÚËÔ IV ıËÔÂÎËÔˉÂÏËfl Ë ·ÓÎÂÒÚ Ì‡ Tangier.(1) ë˙‰Â˜ÌËÚ Á‡·ÓÎfl‚‡ÌËfl ˜ÂÒÚÓ ‚Ó‰flÚ ‰Ó ÂÏ·Ó΢ÂÌ ËÌÒÛÎÚ. åÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl Ò˙Ò Á‡Òfl„‡Ì ̇ Ò˙ˆÂÚÓ Ò‡ Ù‡ÏËÎÌËÚ ‡ÚˇÎÌË ÏËÍÒÓÏË, ̇ÒΉÒÚ‚ÂÌËÚ Ò˙‰Â˜ÌË ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËfl Ë Ì‡ÒΉÒÚ‚ÂÌËÚ ͇‰ËÓÏËÓÔ‡ÚËË. èÓÒΉÌËÚ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú Ô˙‚˘ÌË Ò˙‰Â˜ÌË Á‡·ÓÎfl‚‡ÌËfl /̇Ô. ıËÔÂÚÓÙ˘̇ڇ ͇‰ËÓÏËÓÔ‡ÚËfl/ ËÎË ‚ÚÓ˘ÌË - ÔË Ì‚ÓÏÛÒÍÛÎÌË Á‡·ÓÎfl‚‡ÌËfl /̇Ô. ÔË ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ, ÖÏÂË-Ñ‡ÈÙÛÒ ÏÛÒÍÛÎ̇ڇ ‰ËÒÚÓÙËfl Ë ‰./ ËÎË ÔË ‚Ó‰ÂÌË ‰ÂÙÂÍÚË ‚ ÏÂÚ‡·ÓÎËÁχ /̇Ô. ·ÓÎÂÒÚ Ì‡ Menkes/. êËÒÍ˙Ú ‚‡Ë‡ ¯ËÓÍÓ ÒÔÓ‰ Á‡·ÓÎfl‚‡ÌÂÚÓ. ç‡ÔËÏÂ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌËflÚ ÔÓ··Ò ̇ ÏËÚ‡Î̇ڇ Í·ԇ Ì  ҂˙Á‡Ì Ò˙Ò Á̇˜ËÏ ËÒÍ ÓÚ Í‡‰ËÓÂÏ·Ó΢ÂÌ ËÌÒÛÎÚ, ‰Ó͇ÚÓ Ô‡ˆËÂÌÚË Ò ÙÓÏË Ì‡ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌË ËÎË ˆÂÒË‚ÌË ‰ËÒËÚÏËË Ë Ù‡ÏËÎÌË ‡ÚˇÎÌË ÏËÍÒÓÏË ËÏ‡Ú ÏÌÓ„Ó ‚ËÒÓÍ ËÒÍ (1).

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àÒıÂÏ˘ÌËflÚ ËÌÒÛÎÚ ÏÓÊ ÔÓÌflÍÓ„‡ ‰‡  ÔÓÒΉˈ‡ ̇ ‡ÚÂˇÎ̇ ‰ËÒÂ͇ˆËfl Ë fl‰ÍÓ ÚÓ‚‡ ÏÓÊ ‰‡  ÛÒÎÓÊÌÂÌË ÔË Ì‡ÒΉÒÚ‚ÂÌË Á‡·ÓÎfl‚‡ÌËfl ̇ Ò˙‰ËÌËÚÂÎ̇ڇ Ú˙Í‡Ì /̇Ô. ÔË Ehlers-Danlos type IV Ë ÒË̉ÓÏ Ì‡ Marfan, ‚Ê. Ú‡·Îˈ‡Ú‡/. èË ‰Û„Ë Ì‡ÒΉÒÚ‚ÂÌË Á‡·ÓÎfl‚‡ÌËfl Ò ̇·Î˛‰‡‚‡ ÒÚÂÌÓÁ‡ ̇ Ò˙‰Ó‚ÂÚÂ, ‰˙Îʇ˘‡ Ò ̇ ÙË·ÓËÌÚËχÎ̇ ÔÓÎËÙÂ‡ˆËfl Ò ÔÓÒΉ‚‡˘Ë ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË /Ì‚ÓÙË·ÓχÚÓÁ‡ I ÚËÔ, pseudoxanthoma elasticum/ (1). Ç˙ÔÂÍË ˜Â ÏÓÌÓ„ÂÌÌËÚ Á‡·ÓÎfl‚‡ÌËfl, ˜ËflÚÓ ÙÂÌÓÚËÔ̇ ËÁfl‚‡ ‚Íβ˜‚‡ Ë ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ, Ò‡ ‰ÍË Â ÌÂÓ·ıÓ‰ËÏÓ ÚflıÌÓÚÓ ÔÓÁ̇‚‡ÌÂ. Ç̉fl‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë ‰Ë‡„ÌÓÒÚ˘ÌË Ë ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌË ÏÂÚÓ‰ËÍË ÔÓÁ‚ÓÎfl‚‡ ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ Ô‡‚ËÎ̇ ‰Ë‡„ÌÓÁ‡, ‡ ÔË ÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ‚ˉ‡ ̇ ÏÛÚ‡ˆËflÚ‡ Ëχ ‚˙ÁÏÓÊÌÓÒÚ Ë Á‡ ÔÂ̇ڇÎ̇ ‰Ë‡„ÌÓÒÚË͇ ÔË ÌflÍÓË ÓÚ Á‡·ÓÎfl‚‡ÌËflÚ‡. Ñˇ„ÌÓÒÚˈË‡ÌÂÚÓ Ì‡ ÔÓ·‡Ì‰‡, ÔÓÒΉ‚‡ÌÓ ÓÚ ËÁÒΉ‚‡Ì ̇ Ì„ӂËÚ Ó‰ÒÚ‚ÂÌˈËÚÂ, ‚Ó‰Ë ‰Ó ÔÂÒËÏÔÚÓχÚ˘ÌÓ ÔÓÒÚ‡‚flÌ ̇ ‰Ë‡„ÌÓÁ‡Ú‡ ÔË ÌflÍÓË ÓÚ Úflı /ÌÓÒËÚÂÎË Ì‡ Ò˙ÓÚ‚ÂÚÌËfl „ÂÌÂÚ˘ÂÌ ‰ÂÙÂÍÚ/, ÍÓÂÚÓ ‰‡‚‡ ‚˙ÁÏÓÊÌÓÒÚ Á‡ Á‡ÔÓ˜‚‡Ì ̇ ωË͇ÏÂÌÚÓÁ̇ ÔÓÙË·ÍÚË͇. éÒ‚ÂÌ ÚÓ‚‡ ËÁÛ˜‡‚‡ÌÂÚÓ Ì‡ Ú‡ÁË „ÛÔ‡ Á‡·ÓÎfl‚‡ÌËfl ÒÔÓχ„‡ Á‡ ËÁflÒÌfl‚‡ÌÂÚÓ Ì‡ ÒÎÓÊ̇ڇ Ô‡ÚÓ„ÂÌÂÁ‡ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ. ãàíÖêÄíìêÄ 1. Hassan A, Markus H, Genetics and Ischaemic Stroke, Brain 2000,123,17841812; 2. Markus H Genes for stroke J Neurol Neurosurg Psychiatry 2004; 75:12291231 3. Bogousslavsky J., Caplan L., Uncommon Causes of Stroke, Cambridge University Press 2001, pages 266-271 4. Yousry TA, Seelos K, Mayer M, Bruning R, Uttner I, Dichgans M, Mammi S, Straube A, Mai N, Filippi M (1999) Characteristic MR lesion pattern and correlation of T1 and T2 lesion volume with neurologic and neuropsychological findings in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). AJNR Am J Neuroradiol 20:91-100 5. Coulthard A, Blank SC et al Distribution of cranial MRI abnormallities in patients with symptomatic and subclinical CADASIL Br J Radiol 2000; 73:256-65 6. O'Sullivan M, Jarosz JM, Martin RJ, Deasy N, Powell JF, Markus HS (2001) MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL. Neurology 56:628-34 7. Ruchoux MM, Chabriat H, Bousser MG, Baudrimont M, Tournier-Lasserve E (1994) Presence of ultrastructural arterial lesions in muscle and skin vessels of patients with CADASIL. [letter] Stroke 25:2291-2 8. Goebel HH, Meyermann R, Rosin R, Schlote W (1997) Characteristic morphologic manifestation of CADASIL, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, in skeletal muscle and skin. Muscle Nerve 20:625-7 9. Schultz A, Santoianni R, Hewan-Lowe K (1999) Vasculopathic changes of CADASIL can be focal in skin biopsies. Ultrastruct Pathol 23:241-7 10. Joutel A, Favrole P, Labauge P, Chabriat H, Lescoat C, Andreux F, Domenga V, Cecillon M, Vahedi K, Ducros A, Cave-Riant F, Bousser MG, Tournier-Lasserve E (2001) Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis. Lancet 358:2049-51 11. Oberstein SAJ (2003) Diagnostic strategies in CADASIL. Neurology 60:2020 12. Weinmaster G (1997) The ins and outs of notch signaling. Mol Cell Neurosci 9:91-102; 13. Singhal S., Bevan S., Barrick T., Rich P., Markus H., The influence of genetic and cardiovascular risk factors on the CADASIL phenotype; Brain (2004), 127, 2031-2038 14. Joutel A, Vahedi K, Corpechot C, Troesch A, Chabriat H, Vayssiere C, Cruaud C, Maciazek J, Weissenbach J, Bousser MG, Bach JF, Tournier-Lasserve E (1997) Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients. Lancet 350:1511-5 15. Dichgans M, Herzog J, Gasser T (2001) Notch3 mutation involving three cysteine residues in a family with typical CADASIL. Neurology 57:1714-7. 16. Joutel A, Vahedi K, Corpechot C, Troesch A, Chabriat H, Vayssiere C, Cruaud C, Maciazek J, Weissenbach J, Bousser MG, Bach JF, Tournier-Lasserve E (1997) Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients. Lancet 350:1511-5. 17. Saskia AJ Lesnik Oberstein Martij Breuning, J Haan Gene reviews www. genetests.org CADASIL 18. Bogousslavsky J., Caplan L., Uncommon Causes of Stroke, Cambridge University Press 2001, pages 283-284 19. Jonathan D Picker, MBChB, PhD Harvey L Levy, MD Homocystinuria Caused by Cystathionine Beta-Synthase Deficiency, Gene reviews www. genetests.org 20. Bogousslavsky J., Caplan L., Uncommon Causes of Stroke, Cambridge University Press 2001, pages 324-327 21. MELAS, Salvatore DiMauro, MD Gene reviews www. genetests.org

ĉÂÒ Á‡ ÍÓÂÔÓ̉Â̈Ëfl: èÓÙ. è. òÓÚÂÍÓ‚ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl ìÅ ◊ÄÎÂÍ҇̉Ó‚Ò͇“ ÅÛÎ. ◊ë‚. ÉÂÓ„Ë ëÓÙËÈÒÍË“1, ëÓÙËfl 1431


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

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é·ÁÓ II. ÉÖçÖíàäÄ çÄ èéãàÉÖççàü àçëìãí è. òÓÚÂÍÓ‚, à. í˙Ì‚, Ç. åËı‡ÈÎÓ‚‡ äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, åÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇” SUMMARY II. GENETICS OF POLYGENIC STROKE P.Shotekov, I.Tournev, V.Mihaylova Stroke is not a single disease, but rather is a syndrome of different pathological processes all resulting in focal cerebral damage due to disruption of cerebral blood flow. Most strokes appear to be sporadic without obvious Mendelian mode of inheritance. They are thought to arise as a consequence of polygenic and multifactorial influences where multiple genes exert an influence together with the environmental factors.The genetics of multifactorial stroke is problematic because of the genetic and phenotypic heterogeneity, the variable penetrance, the presence of phenocopies and coexistent risk factors. The vast majority of genetic studies of stroke are based on candidate gene approach. A number of genetic variants in the different components of the coagulation cascade and fibrinolytic system, different polymorphisms in the ACE gene, as well as in the methylene tetrahydrofolate reductase and in the endothelial NO synthase gene have been shown to confer stroke risk. Some of the genes could act by modulating the effect of conventional risk factors. It is important for the clinical practice that certain genes may affect both the stroke outcome and response to therapy. Key words: polygenic disorder, gene polymorphisms, intermediate phenotypes, homocysteine, eNOS, ALOX5AP. êÖáûåÖ åÓÁ˙˜ÌËflÚ ËÌÒÛÎÚ Ì  e‰ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ‡ ÔÓ-ÒÍÓÓ Â ÒË̉ÓÏ, ÂÁÛÎÚ‡Ú ÓÚ ‡Á΢ÌË Ô‡ÚÓÎӄ˘ÌË ÔÓˆÂÒË, ÍÓËÚÓ ‚Ó‰flÚ ‰Ó ÎÓ͇Î̇ ÏÓÁ˙˜Ì‡ Û‚‰‡, ÔÓ‡‰Ë ̇Û¯ÂÌ ÏÓÁ˙˜ÂÌ Í˙‚ÓÚÓÍ. èӂ˜ÂÚÓ ËÌÒÛÎÚË Ò‡ ÒÔÓ‡‰Ë˜ÌË, ·ÂÁ ̇·Î˛‰‡‚‡Ì åẨÂÎÓ‚ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. í ҇ ÂÁÛÎÚ‡Ú Ì‡ ÔÓÎË„ÂÌÌË Ë ÏÛÎÚËÙ‡ÍÚÓÌË ‚ÎËflÌËfl, ÔË ÍÓÂÚÓ ÏÌÓÊÂÒÚ‚Ó „ÂÌË ÛÔ‡ÊÌfl‚‡Ú ‚ÎËflÌËÂÚÓ ÒË ‚ ÍÓÏ·Ë̇ˆËfl Ò Ù‡ÍÚÓËÚ ̇ ÓÍÓÎ̇ڇ Ò‰‡. ÉÂÌÂÚË͇ڇ ̇ ÒÔÓ‡‰Ë˜ÌËfl ËÌÒÛÎÚ Â ÔÓ·ÎÂχÚ˘̇, ÔÓ‡‰Ë ÙÂÌÓÚËÔ̇ڇ Ë „ÂÌÂÚ˘̇ ıÂÚÂÓ„ÂÌÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ‚‡ˇ·ËÎ̇ڇ ÔÂÌÂÚ‡ÌÚÌÓÒÚ, ̇΢ËÂÚÓ Ì‡ ÙÂÌÓÍÓÔËfl, ͇ÍÚÓ Ë Ì‡ Ò˙Ô˙ÚÒÚ‚‡˘Ë ËÒÍÓ‚Ë Ù‡ÍÚÓË. èÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡ ‚˙ıÛ „ÂÌÂÚË͇ ̇ ËÌÒÛÎÚ‡ ËÁÔÓÎÁ‚‡Ú Ú. ̇. ͇̉ˉ‡Ú-„ÂÌ ÔÓ‰ıÓ‰. ìÒÚ‡ÌÓ‚ÂÌË Ò‡ ‡Á΢ÌË ÔÓÎËÏÓÙËÁÏË ‚ „ÂÌË ÓÚ ÍÓ‡„Û·ˆËÓÌ̇ڇ ͇Ò͇‰‡ Ë ÙË·ËÌÓÎËÚ˘̇ڇ ÒËÒÚÂχ, ‚ „Â̇ ÄëÖ, ‚ ÏÂÚËÎÂÌÚÂÚ‡ıˉÓÙÓÎ‡Ú ‰ÛÍÚ‡Á‡ „Â̇, ‚ „Â̇ Á‡ ẨÓÚÂÎ̇ڇ ‡ÁÓÚÂÌ ÓÍÒˉ ÒËÌÚ‡Á‡, ÍÓËÚÓ Û‚Â΢‡‚‡Ú ËÒ͇ Á‡ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ. çflÍÓË „ÂÌË ÔÓ‚ÎËfl‚‡Ú ÍÓÌ‚Â̈ËÓ̇ÎÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Ë ˜ÂÁ Úflı ÏÓ‰ËÙˈË‡Ú ËÒ͇ Á‡ ËÌÒÛÎÚ. LJÊÌÓ Á‡ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇  ̇΢ËÂÚÓ Ì‡ „ÂÌË, ÍÓËÚÓ ÔÓ‚ÎËfl‚‡Ú ËÁıÓ‰‡ ̇ ÏÓÁ˙˜ÌËfl ËÌÒÛÎÚ, ͇ÍÚÓ Ë ÓÚ„Ó‚Ó‡ Í˙Ï ÚÂ‡ÔËflÚ‡. äβ˜Ó‚Ë ‰ÛÏË: ÔÓÎË„ÂÌÌÓ Á‡·ÓÎfl‚‡ÌÂ, „ÂÌÌË ÔÓÎËÏÓÙËÁÏË, ÏÂʉËÌÌË ÙÂÌÓÚËÔÓ‚Â, ıÓÏÓˆËÒÚÂËÌ, eNOS, ALOX5AP. àÒıÂÏ˘ÌËflÚ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ Â ÒË̉ÓÏ, ÂÁÛÎÚ‡Ú ÓÚ ‡Á΢ÌË Ô‡ÚÓÎӄ˘ÌË ÔÓˆÂÒË, ÍÓËÚÓ ‚Ó‰flÚ ‰Ó ÎÓ͇Î̇ ÏÓÁ˙˜Ì‡ Û‚‰‡, ÔÓ‡‰Ë ̇Û¯ÂÌ ÏÓÁ˙˜ÂÌ Í˙‚Ó-

ÚÓÍ. ÉÓÎflÏ ·ÓÈ ÏÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl ‚Íβ˜‚‡Ú ‚ ÍÓÏÔÎÂÍÒ̇ڇ ÒË ÍÎËÌ˘̇ ͇ÚË̇ Ë ËÌÒÛÎÚ, ÌÓ ÔÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ Úflı Ò‡ ËÁÍβ˜ËÚÂÎÌÓ ‰ÍË. Ç Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë ËÌÒÛÎÚ˙Ú Â ÒÔÓ‡‰Ë˜ÂÌ Ë Ô‰ÒÚ‡‚Îfl‚‡ ÏÛÎÚËÙ‡ÍÚÓÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÔË ÍÓÂÚÓ Ì Ò ̇·Î˛‰‡‚‡ åẨÂÎÓ‚ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. É·‚ÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË /ıËÔÂÚÓÌËfl, ‰Ë‡·ÂÚ, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ/ Ó·flÒÌfl‚‡Ú ‰Ó 50% ÓÚ ËÒ͇ Á‡ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‚ ÔÓÔÛ·ˆËflÚ‡, ‡ „ÂÌÂÚ˘̇ Ô‰ËÒÔÓÁˈËfl Ò ‰ÓÔÛÒ͇, ˜Â  ÓÚ„Ó‚Ó̇ Á‡ ˜‡ÒÚ ÓÚ ÌÂÓ·flÒÌËÏËfl ËÒÍ (3). èË ÚÓ‚‡ „ÂÌÂÚ˘ÌËÚ هÍÚÓË Ò‡ ̇È-ÒËÎÌÓ ËÁ‡ÁÂÌË ÔË Ô‡ˆËÂÌÚË Ò ËÌÒÛÎÚ ÔÂ‰Ë 65 „Ó‰ËÌË (5). àÒıÂÏ˘ÌËflÚ ËÌÒÛÎÚ Â ÔÓÎË„ÂÌÌÓ Á‡·ÓÎfl‚‡ÌÂ, Ú. ÂÁÛÎÚ‡Ú ÓÚ ‚ÎËflÌËÂÚÓ Ì‡ ÏÌÓ„Ó ‡Á΢ÌË „ÂÌË, ÍÓËÚÓ ÔÓ‚ÎËfl‚‡Ú ‡Á΢ÌË Ô‡ÚÓÙËÁËÓÎӄ˘ÌË ÔÓˆÂÒË. ÇÒÂÍË „ÂÌ Ò‡ÏÓÒÚÓflÚÂÎÌÓ Ò ‡ÒÓˆËË‡ Ò ÏËÌËχÎÂÌ ËÒÍ. ç‡Î˘ËÂÚÓ Ì‡ ÌflÍÓÎÍÓ „Â̇ ÏÓÊ ‰‡ Û‚ÂÎË˜Ë ËÒ͇ Á‡ ·ÓÎÂÒÚÚ‡ ˜ÂÁ Ú.̇. „ÂÌ-‰ÓÁË‡Ì ÂÙÂÍÚ ËÎË „ÂÌ˙Ú ÏÓÊ ‰‡ ‚Á‡ËÏÓ‰ÂÈÒÚ‚‡ Ò ÍÓÌ‚Â̈ËÓ̇ÎÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Ë ‰‡ ÏÓ‰ÛÎË‡ ÚÂıÌËÚ ÂÙÂÍÚË ËÎË Ò ‰Û„Ë „ÂÌË. ç‡ÔËÏÂ ÔË ÌflÍÓË ıËÔÂÚÓÌËˆË Ò ÛÒÚ‡ÌÓ‚fl‚‡ Á‡Òfl„‡Ì ̇ χÎÍËÚ ÏÓÁ˙˜ÌË Ò˙‰Ó‚Â, ·ÂÁ ‰‡ÌÌË Á‡ ‡ÚÂÓÒÍÎÂÓÁ‡ ‚ „ÓÎÂÏËÚ Ò˙‰Ó‚Â, ‰Ó͇ÚÓ ÔË ‰Û„Ë Â ÚÓ˜ÌÓ Ó·‡ÚÌÓÚÓ ËÎË Â Ì‡Îˈ ÍÓÏ·Ë̇ˆËfl ÓÚ ‰‚‡Ú‡ ÚËÔ‡ (2). à‰ÂÌÚËÙË͇ˆËflÚ‡ ̇ „ÂÌË Ô‰‡ÁÔÓ·„‡˘Ë Í˙Ï ÏÛÎÚËÙ‡ÍÚÓÌËfl ËÌÒÛÎÚ Â Úۉ̇ Á‡‰‡˜‡. á‡ÚÛ‰ÌÂÌËflÚ‡ Ò‡ Ò‚˙Á‡ÌË Ò˙Ò ÒΉÌËÚ Ô˘ËÌË: Í˙ÒÌÓÚÓ Ì‡˜‡ÎÓ Ì‡ ËÌÒÛÎÚ‡, ÙÂÌÓÚËÔ̇ڇ ıÂÚÂÓ„ÂÌÌÓÒÚ /‡Á΢ÌËÚ ÚËÔÓ‚Â ËÌÒÛÎÚ ‚ÂÓflÚÌÓ Ò‡ Ò‚˙Á‡ÌË Ò ‡Á΢ÌË ÂÚËÓÎӄ˘ÌË Ù‡ÍÚÓË/, „ÂÌÂÚ˘̇ڇ ıÂÚÂÓ„ÂÌÌÓÒÚ /ÏÛÚ‡ˆËË ‚ ‡Á΢ÌË „ÂÌË ‚Ó‰flÚ ‰Ó ‰̇Í˙‚ ÙÂÌÓÚËÔ/, ̇΢ËÂÚÓ Ì‡ ÙÂÌÓÍÓÔËfl /ÌflÍÓË Ô‡ˆËÂÌÚË Ì Û̇ÒΉfl‚‡Ú Ô‰‡ÁÔÓ·„‡˘ ‡ÎÂÎ, ÌÓ Ò˙˘Ó ËÏ‡Ú ËÌÒÛÎÚ/, ‚‡ˇ·ËÎ̇ڇ ÔÂÌÂÚ‡ÌÚÌÓÒÚ /ÌflÍÓË ıÓ‡ Ò‡ Û̇ÒΉËÎË Ô‰‡ÁÔÓ·„‡˘ ‡ÎÂÎ, ÌÓ ÌflÏ‡Ú ËÌÒÛÎÚ/, ÓÒ‚ÂÌ ÚÓ‚‡ ̇΢ËÂÚÓ Ì‡ Ò˙Ô˙ÚÒÚ‚‡˘Ë ËÒÍÓ‚Ë Ù‡ÍÚÓË, ͇ÚÓ ıËÔÂÚÓÌËfl Ë ‰Ë‡·ÂÚ, Ò˙˘Ó Á‡ÚÛ‰Ìfl‚‡Ú ÓˆÂÌfl‚‡ÌÂÚÓ Ì‡ ÂÙÂÍÚ‡ ̇ ‰‡‰ÂÌ „ÂÌ ÔË Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë (2). èÓ˜ÚË ‚Ò˘ÍË ÔÓÛ˜‚‡ÌËfl ËÁÔÓÎÁ‚‡Ú ͇̉ˉ‡Ú-„ÂÌ ÔÓ‰ıÓ‰‡ - ËÁÛ˜‡‚‡Ú Ò ‡Á΢ÌË ÔÓÎËÏÓÙËÁÏË /Ñçä ÔÓÒΉӂ‡ÚÂÎÌÓÒÚË/ ̇ ‰ˈ‡ ͇̉ˉ‡Ú „ÂÌË /Ú. Â. „ÂÌË, Á‡ ˜ËËÚÓ ÔÓ‰ÛÍÚË Ò ‰ÓÔÛÒ͇, ˜Â Ë„‡flÚ ÓÎfl ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ËÌÒÛÎÚ‡, ̇Ô. „ÂÌË Ò‚˙Á‡ÌË Ò ıÂÏÓÒÚ‡Á‡Ú‡, ıÓÏÓˆËÒÚÂËÌÓ‚Ëfl ÏÂÚ‡·ÓÎËÁ˙Ï, ‡Ì„ËÓÚÂÌÁËÌ-ÍÓÌ‚ÂÚË‡˘Ëfl ÂÌÁËÏ, „ÂÌ˙Ú ÍÓ‰Ë‡˘ ẨÓÚÂÎ̇ ‡ÁÓÚÂÌ ÓÍÒˉ ÒËÌÚ‡Á‡ Ë ‰/ ÔË Ô‡ˆËÂÌÚË Ë Á‰‡‚Ë ÍÓÌÚÓÎË, ÓˆÂÌfl‚‡ Ò Ëχ ÎË ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ‡ÁÎË͇ ‚ Úflı̇ڇ ˜ÂÒÚÓÚ‡ ‚ ‰‚ÂÚ „ÛÔË (1,3). ÑÓÒ„‡ Ò‡ ËÁÛ˜ÂÌË „ÓÎflÏ ·ÓÈ Í‡Ì‰Ë‰‡Ú „ÂÌË, ÌÓ Ì  ÛÒÚ‡ÌÓ‚Â̇ flÒ̇ ‡ÒӈˇˆËfl, ÍÓÂÚÓ Â ÔÓÒΉˈ‡ ̇ ̉ÓÒÚ‡Ú˙˜ÌËfl ·ÓÈ Ô‡ˆËÂÌÚË, ÔÓÔÛ·ˆËÓÌ̇ڇ ÒÚ‡ÚËÙË͇ˆËfl, ̉ӷÓÚÓ ÙÂÌÓÚËÔËÁË‡Ì ̇ ÒÎÛ˜‡ËÚÂ, ̉ÓÓˆÂÌfl‚‡ÌÂÚÓ Ì‡ ıÂÚÂÓ„ÂÌÌÓÒÚÚ‡ ̇ ËÌÒÛÎÚ‡. ç‡ÔÓÒΉ˙Í ÚÂÁË ÏÂÚÓ‰ÓÎӄ˘ÌË Ó„‡Ì˘ÂÌËfl Ò ‚ÁÂÏ‡Ú Ô‰‚ˉ ÔË ËÁ„ÓÚ‚flÌÂÚÓ Ì‡ ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÔÓÂÍÚË, ÍÓËÚÓ ‚Íβ˜‚‡Ú Ë Ò˙ÚÛ‰Ì˘ÂÒÚ‚Ó Ì‡ ̇ˆËÓ̇ÎÌÓ Ë ÏÂʉÛ̇Ó‰ÌÓ ÌË‚Ó (1,3). àÒıÂÏ˘ÌËflÚ ËÌÒÛÎÚ Â ÒË̉ÓÏ, ‡ Ì ‰ÌÓӉ̇ ·ÓÎÂÒÚ. ç‡Ô‰˙Í˙Ú ‚ Ì‚ÓËÁÓ·‡Áfl‚‡ÌÂÚÓ ÔÓÁ‚ÓÎË ÓÔ‰ÂÎflÌÂÚÓ Ì‡ Ô‡ÚÓ„ÂÌÂÚ˘ÌËfl ÏÂı‡ÌËÁ˙Ï Ì‡ ËÌÒÛÎÚ‡ ÔË


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÏÌÓ„Ó ÒÎÛ˜‡Ë. ÇÂÓflÚÌÓ ÓÔ‰ÂÎÂÌË „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË Ô‰‡ÁÔÓ·„‡Ú Ë̉˂ˉ‡ Í˙Ï ÒÔˆËÙ˘ÂÌ ÚËÔ Ì‡ ËÌÒÛÎÚ‡. îÂÌÓÚËÔÌÓ ËÌÒÛÎÚËÚ Ò ‡Á‰ÂÎflÚ Ì‡ Ú‡ÍË‚‡ ‰˙Îʇ˘Ë Ò ̇ ͇‰ËÓÂÏ·ÓÎËÁ˙Ï, ̇ Á‡Òfl„‡Ì ̇ ËÌÚ‡Í‡ÌˇÎÌË Ë ÂÍÒÚ‡Í‡ÌˇÎÌË „ÓÎÂÏË ‡ÚÂˇÎÌË Ò˙‰Ó‚Â, ̇ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚ - Á‡ÔÛ¯‚‡Ì ̇ ‰˙ηÓÍËÚ ÔÂÙÓ‡ÌÚÌË ‡ÚÂËÓÎË. ᇠ‚ÒÂÍË ÓÚ Úflı „ÂÌÂÚ˘ÌËÚ هÍÚÓË ‚ÂÓflÚÌÓ ËÎË Ô‰‡ÁÔÓ·„‡Ú Í˙Ï ÍÓÌ‚Â̈ËÓ̇ÎÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË, ËÎË ÏÓ‰ÛÎË‡Ú ÚÂıÌËÚ ÂÙÂÍÚË ‚˙ıÛ ÔˈÂÎÌËÚ Ó„‡ÌË, ËÎË ‰ÂÈÒÚ‚‡Ú ‰ËÂÍÚÌÓ ‚˙ıÛ ËÒ͇ ÓÚ ËÌÒÛÎÚ. ë‡ÏËÚ ÍÓÌ‚Â̈ËÓ̇ÎÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË Í‡ÚÓ ‰Ë‡·ÂÚ, ıËÔÂÚÓÌËfl Ë ıËÔÂÎËÔˉÂÏËfl Ò˙˘Ó Ò‡ ÔÓ‰ „ÂÌÂÚ˘ÂÌ ÍÓÌÚÓÎ (3). äÓÏÔÎÂÍÒÌËflÚ ı‡‡ÍÚÂ ̇ ËÌÒÛÎÚ‡ ‰Ó‚‰ ‰Ó ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ Ú.̇. ÏÂʉËÌÌË ÙÂÌÓÚËÔÓ‚Â (2,3). àÌÒÛÎÚ˙Ú Â Í‡ÂÌ ÂÁÛÎÚ‡Ú ÓÚ ‰ˈ‡ Ô‡ÚÓÎӄ˘ÌË ÔÓˆÂÒË, ‚ÒÂÍË ÓÚ ÍÓËÚÓ Â ÔÓ‰ÎÓÊÂÌ Ì‡ „ÂÌÂÚ˘ÌË ‚ÎËflÌËfl. åÂʉËÌÌËÚ ÙÂÌÓÚËÔÓ‚Â Ô‰ÒÚ‡‚Îfl‚‡Ú ÓÚ‰ÂÎÌË ÂÚ‡ÔË ÓÚ ‡Á‚ËÚËÂÚÓ Ì‡ ËÌÒÛÎÚ‡ Ë ÒΉӂ‡ÚÂÎÌÓ ·ÓflÚ Ì‡ „ÂÌËÚ ‚Íβ˜ÂÌË ‚ Úflı̇ڇ Ô‡ÚÓ„ÂÌÂÁ‡  ÔÓ-χÎ˙Í ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË ‚˙‚ΘÂÌË ‚ ̇Ô˙ÎÌÓ Á‡‚˙¯ÂÌËfl ËÌÒÛÎÚ. íÓ‚‡ Ô‰ÓÒÚ‡‚fl ‚˙ÁÏÓÊÌÓÒÚ Á‡ Úflı̇ڇ ÛÒÔ¯̇ ˉÂÌÚËÙË͇ˆËfl. éÒ‚ÂÌ ÚÓ‚‡ ÏÂʉËÌÌËÚ ÙÂÌÓÚËÔÓ‚Â Ô‰ÓÒÚ‡‚flÚ ‚˙ÁÏÓÊÌÓÒÚ Á‡ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏË ÂÁÛÎÚ‡ÚË, ÔÓ‡‰Ë ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÔÓÏÂÌÎË‚Ë, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ Ò ËÁÏÂ‚‡Ú ‚ ˆfl·ڇ ÔÓÔÛ·ˆËfl, ͇ÍÚÓ Ë ÒΉ ÓÔ‰ÂÎÂÌ ÔÂËÓ‰ Á‡ ‰‡ Ò ÓÔ‰ÂÎË ‚˙Á͇ Ò ÔÓ„ÂÒËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. òËÓÍÓ ËÁÔÓÎÁ‚‡ÌË ÏÂʉËÌÌË ÙÂÌÓÚËÔÓ‚Â Á‡ ËÌÒÛÎÚ ÔË Á‡·ÓÎfl‚‡Ì ̇ „ÓÎÂÏËÚ Ò˙‰Ó‚ ҇ ͇ÓÚˉ̇ڇ IMT (intima-media thickness) Ë ‡ÁÏÂ˙Ú Ì‡ ͇ÓÚˉÌËÚ Ô·ÍË ÔË ÛÎÚ‡ÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ. í ÏÓ„‡Ú ‰‡ Ò ËÁÔÓÎÁ‚‡Ú Á‡ ÓÔ‰ÂÎflÌ ÓÎflÚ‡ ̇ ‰ˈ‡ ÍÓÌ‚Â̈ËÓ̇ÎÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË, ͇ÍÚÓ Ë ÌÓ‚Ë ËÒÍÓ‚Ë Ù‡ÍÚÓË Í‡ÚÓ ıÓÌ˘̇ ËÌÙÂ͈Ëfl, ‚˙ÁÔ‡ÎÂÌËÂ Ë „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ͇ÓÚˉ̇ڇ ‡ÚÂÓÒÍÎÂÓÁ‡. èÓÎÂÁÂÌ ÏÂʉËÌÂÌ ÙÂÌÓÚËÔ ÔË ËÁÛ˜‡‚‡Ì ̇ ËÌÒÛÎÚ ‰˙Îʇ˘ Ò ̇ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚ ҇ ıËÔÂËÌÚÂÌÁÌËÚ ÎÂÁËË ‚ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó ÔË í2-ËÁÏÂ‚‡ÌÂÚÓ Ì‡ åêí (2,3). éÒ‚ÂÌ ËÁÛ˜‡‚‡ÌÂÚÓ Ì‡ ͇̉ˉ‡Ú „ÂÌË, ÍÓËÚÓ ÔÓ‚ÎËfl‚‡Ú ËÒ͇ Á‡ ËÌÒÛÎÚ, Ëχ ÔÓÛ˜‚‡ÌËfl ̇ÒÓ˜ÂÌË Í˙Ï „ÂÌË ÓÔ‰ÂÎfl˘Ë „ÓÎÂÏË̇ڇ ̇ ËÌÙ‡ÍÚ‡ ÒΉ Á‡ÔÛ¯‚‡ÌÂÚÓ Ì‡ Í˙‚ÓÌÓÒÂÌ Ò˙‰ ˜ÂÁ ÔÓ‚ÎËfl‚‡Ì ̇ Ò˙‰Ó‚‡Ú‡ ‡ÍÚË‚ÌÓÒÚ, ÍÓ·ÚÂ‡ÎÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ Ë ÚÓÎÂ‡ÌÚÌÓÒÚÚ‡ ̇ Ì‚ÓÌËÚ Í˙Ï ËÒıÂÏËfl. çflÍÓË „ÂÌË ÏÓÊ ‰‡ Ô‰‡ÁÔÓ·„‡Ú Í˙Ï ËÌÒÛÎÚ Ë ‰‡ ÔÓ‚ÎËfl‚‡Ú ËÁıÓ‰‡ ÓÚ ËÒıÂÏËflÚ‡. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÌflÍÓË Í‡Ì‰Ë‰‡Ú „ÂÌË ËÏ‡Ú ‚‡ÊÌÓ Á̇˜ÂÌË ҇ÏÓ ÔË Ï·‰Ë Ô‡ˆËÂÌÚË. ᇠÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ‚˙Á͇ ÏÂÊ‰Û Ò˙ÓÚ‚ÂÚÌËÚ „ÂÌË Ë ËÌÒÛÎÚ‡  ÌÂÓ·ıÓ‰ËÏÓ ‡Ì‡ÎËÁ ̇ ‰‡ÌÌËÚ ÒÔÓ‰ ÚËÔ‡ ̇ ËÌÒÛÎÚ‡ Ë ÓÚ‰ÂÎÌË ÔÓÛ˜‚‡ÌËfl ‚˙ıÛ Ï·‰Ë ËÌ‰Ë‚Ë‰Ë (2). т ̇ÒÍÓÓ Ôӂ‰ÂÌË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú ‚˙Á͇ ÏÂÊ‰Û Ì‡Î˘ËÂÚÓ Ì‡ Ù‡ÏËÎÌÓÒÚ Ë ËÌÒÛÎÚ ‰˙Îʇ˘ Ò ̇ Á‡Òfl„‡Ì ̇ „ÓÎÂÏËÚÂ Ë Ï‡ÎÍËÚ Ò˙‰Ó‚Â, ‰Ó͇ÚÓ Ú‡Í‡‚‡ Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË Í‡‰ËÓÂÏ·Ó΢ÌËfl ËÌÒÛÎÚ (5,6). ëΉӂ‡ÚÂÎÌÓ ËÁÛ˜‡‚‡ÌÂÚÓ Ì‡ „ÂÌÂÚ˘ÌËÚ ÓÒÌÓ‚Ë Ì‡ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ ·Ë ·ËÎÓ ÔÓ-ÂÙÂÍÚË‚ÌÓ ‡ÍÓ Ò ӷı‚‡Ì‡Ú Ò˙ÓÚ‚ÂÚÌËÚ ÚËÔÓ‚Â ËÌÒÛÎÚ Ë Ï·‰ËÚ ‚˙Á‡ÒÚÓ‚Ë „ÛÔË, ÔË ÍÓËÚÓ ÓÎflÚ‡ ̇ „ÂÌÂÚ˘ÌËfl ÍÓÏÔÓÌÂÌÚ Â „ÓÎflχ (5). èӂ˜ÂÚÓ ÔÓÛ˜‚‡ÌËfl Ì ҇ ÛÒÚ‡ÌÓ‚ËÎË ‡ÒӈˇˆËfl ÏÂÊ‰Û ÔÓÚÓÏ·ÓÚ˘ÌËÚ Ò˙ÒÚÓflÌËfl /̇Ô. ÂÁËÒÚÂÌÚÌÓÒÚ Í˙Ï ‡ÍÚË‚Ë‡Ì ÔÓÚÂËÌ ë Ë ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ ÔË ‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË. í‡ÍË‚‡ „ÂÌÂÚ˘ÌË ‰ÂÙÂÍÚË ÏÓ„‡Ú ‰‡ Ò‡ Ô˘Ë̇ Á‡ ËÌÒÛÎÚ ÔË ÔÓ-Ï·‰Ë Ë̉˂ˉË, ÌÓ Í‡ÚÓ ˆflÎÓ Ú ̇È-‚ÂÓflÚÌÓ Ì ҇ ‚‡Ê̇ Ô˘Ë̇ Á‡ ÏÛÎÚËÙ‡ÍÚÓÌËfl ËÌÒÛÎÚ ÔË ‚˙Á‡ÒÚÌËÚ (3). ÉÂÌÂÚ˘ÌË ‚‡ˇÌÚË ‚ ‰Û„Ë ÍÓÏÔÓÌÂÌÚË Ì‡ ÍÓ‡„Û·-

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ˆËÓÌ̇ڇ ͇Ò͇‰‡, ͇ÚÓ Ù‡ÍÚÓ VII Ë ÙË·ËÌÓ„ÂÌ ËÏ‡Ú ‰Ó͇Á‡Ì‡ ÓÎfl ‚ ‡ÚÂˇÎÌËÚ Á‡·ÓÎfl‚‡ÌËfl (3). ïÓÏÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌË Á‡ Ú‡ÌÁˈËflÚ‡ G➞A ̇ 455 ÔÓÁˈËfl ‚ b-ÙË·ËÌÓ„ÂÌÓ‚Ëfl „ÂÌ (G455A) Ò ‡ÒÓˆËË‡ Ò Ôӂ˯ÂÌË ÌË‚‡ ‚˙‚ ÙË·ËÌÓ„Â̇ Ë ËÌÒÛÎÚ, ‰˙Îʇ¯ Ò ̇ „ÓÎÂÏËÚ Ò˙‰Ó‚ (Kessler et al., 1997). 臈ËÂÌÚË Ò ËÌÒÛÎÚ ÌÓÒËÚÂÎË Ì‡ Ä ‡Î· ̇ ÔÓÎËÏÓÙËÁχ G455A ̇ b-ÙË·ËÌÓ„ÂÌÓ‚Ëfl „ÂÌ ˜ÂÒÚÓ ËÏ‡Ú Ë ÏÌÓÊÂÒÚ‚Ó Î‡ÍÛ̇ÌË ËÌÙ‡ÍÚË. ëΉӂ‡ÚÂÎÌÓ ÚÓÁË ‡ÎÂÎ Ô‰‡ÁÔÓ·„‡ Í˙Ï ‡ÚÂÓÚÓÏ·ÓÁ‡ ‚ ÏÓÁ˙˜Ì‡Ú‡ ˆËÍÛ·ˆËfl.(7) èÓÛ˜‚‡ÌËfl ÔË ÏËÓ͇‰ÌËfl ËÌÙ‡ÍÚ ÔÓ͇Á‚‡Ú, ˜Â Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ‡·ÌÓÏÌË ÙË·ËÌÓ‚Ë ÔÓÎËÏÂË Â ‚‡ÊÌÓ ÔË ‡ÚÂˇÎ̇ڇ ÚÓÏ·ÓÁ‡ (Fatah et al., 1996). çÓχÎÌÓ ÚÓ‚‡ Á‡‚ËÒË ÓÚ ÙÛÌ͈ËÓÌË‡ÌÂÚÓ Ì‡ Ù‡ÍÚÓ XIII Ë ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ ÙË·ËÌÓÎËÚ˘̇ڇ ÒËÒÚÂχ. èÓÎËÏÓÙËÁÏ˙Ú ‚ „Â̇ ÍÓ‰Ë‡˘ Ù‡ÍÚÓ XIII Val34Leu ÔÓ-‡ÌÓ ·Â¯Â ‡ÒÓˆËË‡Ì Ò Ì‡Ï‡ÎÂÌ ËÒÍ ÓÚ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Ë Ôӂ˯ÂÌ ËÒÍ ÓÚ Ô‡ÂÌıËÏÂÌ ÏÓÁ˙˜ÂÌ Í˙‚ÓËÁÎË‚, ÌÓ Â‰ÌÓ ÌÓ‚Ó ÔÓ-„ÓÎflÏÓ ÔÓÛ˜‚‡Ì Ì ÔÓÚ‚˙‰Ë ڇ͇‚‡ ‡ÒӈˇˆËfl Ë ÒΉӂ‡ÚÂÎÌÓ ËÁÒΉ‚‡ÌÂÚÓ Ì‡ ÚÓÁË ÔÓÎËÏÓÙËÁ˙Ï Ì ‰ÓÔË̇Òfl Á‡ ÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ËÒÍ Á‡ ËÌÒÛÎÚ (8). Ö̉ӄÂÌÌËflÚ Ú˙͇ÌÂÌ Ô·ÁÏËÌÓ„ÂÌÓ‚ ‡ÍÚË‚‡ÚÓ /íèÄ/  ÓÒÌÓ‚ÂÌ Ï‰ˇÚÓ ̇ ËÌÚ‡‚‡ÒÍÛ·̇ڇ ÙË·ËÌÓÎËÁ‡ Ë Ò ÓÚ‰ÂÎfl Ô‰ËÏÌÓ ÓÚ ẨÓÚ· ̇ χÎÍËÚ Ò˙‰Ó‚Â. éÒÚÓÚÓ ÓÒ‚Ó·Óʉ‡‚‡Ì ̇ íèÄ Á‡‚ËÒË ÓÚ ÔÓÎËÏÓÙËÁχ íèÄ C7351í. ÑÓ͇Á‡ÌÓ Â, ˜Â ÚÓÁË ÔÓÎËÏÓÙËÁ˙Ï Â ÌÂÁ‡‚ËÒËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ·ÍÛ̇ÌËfl ËÌÒÛÎÚ, ÒΉӂ‡ÚÂÎÌÓ Ì‡Û¯ÂÌË ‚˙‚ ÙË·ËÌÓÎËÁ‡Ú‡ Ë„‡flÚ ‚‡Ê̇ ÓÎfl ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÚÓÁË ÚËÔ ËÌÒÛÎÚ.(9) êÓÎflÚ‡ ̇ ÔÓÎËÏÓÙËÁχ ̇ ÚÓÏ·ÓˆËÚÌËÚ „ÎËÍÓÔÓÚÂËÌÓ‚Ë ˆÂÔÚÓË (Gp)  ӷÒÚÓÈÌÓ ËÁÒΉ‚‡Ì‡ ÔË Ô‡ˆËÂÌÚË Ò ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ. íÂÁË ÏÓÎÂÍÛÎË Ò‡ ˜ÎÂÌӂ ̇ Ù‡ÏËÎËflÚ‡ ̇ ËÌÚ„ËÌËÚÂ Ë ÍÓ„‡ÚÓ Ò ‡ÍÚË‚Ë‡Ú Ò‚˙Á‚‡Ú ÙË·ËÌÓ„ÂÌ, Ù‡ÍÚÓ‡ ̇ von Willebrand Ë ÍÓ·„ÂÌ, ÒÚËÏÛÎË‡Ú ÚÓÏ·ÓˆËÚ̇ڇ ‡„„‡ˆËfl Ë ÚÓÏ·ÓÁ‡. àχ ‰‚‡ „·‚ÌË ‚‡ˇÌÚ‡ ̇ „Â̇, ÍÓ‰Ë‡˘ IIIa Ò۷‰ËÌˈ‡Ú‡ ̇ ÏÂÏ·‡ÌÌËfl „ÎËÍÓÔÓÚÂËÌÓ‚ ˆÂÔÚÓ IIb/IIIa, ̇˜ÂÌË PLA1 (A1) Ë PLA2 (A2). ÑÓ͇Á‡ÌÓ Â, ˜Â Ä2 ‡ÎÂÎ˙Ú Â ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ËÌÒÛÎÚ ‰˙Îʇ˘ Ò ̇ Á‡Òfl„‡Ì ̇ „ÓÎÂÏËÚ Ò˙‰Ó‚Â, ͇ÚÓ ÔË Â‰ÌÓ ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡ Ú‡ÁË ‡ÒӈˇˆËfl  ÛÒÚ‡ÌÓ‚Â̇ Ò‡ÏÓ ÔË Ï˙ÊÂÚ (10,11). èÓÎËÏÓÙËÁÏ˙Ú GpIa ë807í ‚ „ÎËÍÓÔÓÚÂËÌÓ‚Ëfl ˆÂÔÚÓ Ia/III ÍÓÂÎË‡ Ò Ôӂ˯Â̇ ÂÍÒÔÂÒËfl ̇ ÍÓ·„ÂÌÓ‚Ëfl ˆÂÔÚÓ ËÌ ‚ËÚÓ Ë Â ÌÂÁ‡‚ËÒËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ËÌÒÛÎÚ ÔË Ï·‰Ë Ô‡ˆËÂÌÚË (Carlsson et al., 1999). íÓÏ·ÓˆËÚÌËflÚ „ÎËÍÓÔÓÚÂËÌ Ibalpha ‚Á‡ËÏÓ‰ÂÈÒÚ‚‡ Ò Ù‡ÍÚÓ‡ ̇ von Willebrand ͇ÚÓ ÔÓ ÚÓÁË Ì‡˜ËÌ Ò ËÌˈËË‡ ÚÓÏ·ÓÁ‡. éÚ ËÁ‚ÂÒÚÌËÚ ÔÓÎËÏÓÙËÁÏË Ì‡ Gp Ibalpha, Kozak T/C ÔÓÎËÏÓÙËÁÏ˙Ú Â ‡ÒÓˆËË‡Ì Ò Û‚Â΢ÂÌË ̇ ÂÍÒÔÂÒËflÚ‡ ̇ Gp Ibalpha ‚˙ıÛ ÔÓ‚˙ıÌÓÒÚÚ‡ ̇ ÚÓÏ·ÓˆËÚËÚÂ Ë Â ÌÂÁ‡‚ËÒËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ (12). Ö‰ËÌ ÓÚ Ì‡È-ËÁÒΉ‚‡ÌËÚ ͇̉ˉ‡Ú „ÂÌË Á‡ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Â „ÂÌ˙Ú ÍÓ‰Ë‡˘ ÄëÖ /‡Ì„ËÓÚÂÌÁËÌÍÓ‚ÂÚË‡˘ ÂÌÁËÏ/. èÓ‰Û͈ËflÚ‡ ̇ ‡Ì„ËÓÚÂÌÁËÌ II Ë Í‡Ú‡·ÓÎËÁÏ˙Ú Ì‡ ·‡‰ËÍËÌË̇ Ò‡ ÂÁÛÎÚ‡Ú Ì‡ ‰ÂÈÒÚ‚ËÂÚÓ Ì‡ ÄëÖ. íÂÁË ÔÂÔÚË‰Ë ËÏ‡Ú ‚‡ÊÌË ÙÛÌ͈ËË Ò‚˙Á‡ÌË Ò „Û·ˆËflÚ‡ ̇ Ò˙‰Ó‚Ëfl ÚÓÌÛÒ, ẨÓÚÂÎ̇ڇ ÙÛÌ͈Ëfl, „·‰ÍÓÏÛÒÍÛÎ̇ڇ ÔÓÎËÙÂ‡ˆËfl ‚ Ò˙‰Ó‚ËÚ ÒÚÂÌË. åÌÓ„Ó·ÓÈÌËÚ ÔÓÛ˜‚‡ÌËfl Ò‡ ̇ÒÓ˜ÂÌË Í˙Ï ÛÒÚ‡ÌÓ‚fl‚‡Ì ÓÎflÚ‡ ̇ ËÌÒÂˆËfl/‰ÂΈËfl ÔÓÎËÏÓÙËÁχ ̇ 16 ËÌÚÓÌ Ì‡ ÄëÖ „Â̇ Ë ËÒ͇ Á‡ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ. êÂÁÛÎÚ‡ÚËÚ ҇ ÔÓÚË‚Ó˜˂Ë, ÍÓÂÚÓ Â ÂÁÛÎÚ‡Ú Ì‡ ÏÂÚÓ‰ÓÎӄ˘ÌË Ó„‡Ì˘ÂÌËfl (2). èË Â‰ÌÓ Ì‡ÒÍÓÓ Ôӂ‰ÂÌÓ ÔÓÛ˜‚‡Ì ‚ ÉÂχÌËfl (U. Schminke, J. Luedemann, A. Kilian et al., 2003)  ÔÓÛ˜Â̇ ÓÎflÚ‡ ̇ ËÌÒÂˆËfl/‰ÂΈËfl (I/D) ÔÓÎËÏÓÙËÁχ ̇ 16


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ËÌÚÓÌ Ì‡ ÄëÖ „Â̇ Ë ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡ ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl, ÓˆÂÌÂ̇ ÛÎÚ‡ÒÓÌÓ„‡ÙÒÍË. èÓÛ˜‚‡ÌÂÚÓ Ó·ı‚‡˘‡ 811 Ï˙Ê ÌÂÔÛ¯‡˜Ë Ë ·ÂÁ Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. êÂÁÛÎÚ‡ÚËÚ ÔÓ͇Á‚‡Ú, ˜Â ˜ÂÒÚÓÚ‡Ú‡ ̇ „ÂÌÓÚËÔ‡ ‰ÂΈËfl/‰ÂΈËfl (D/D, Ú. ıÓÏÓÁË„ÓÚË ÔÓ ‰ÂΈËfl ̇ Ñçä ÔÓÒΉӂ‡ÚÂÎÌÓÒÚ Ì‡ 16 ËÌÚÓÌ)  ÔÓ-„ÓÎflχ Ò‰ Ë̉˂ˉËÚÂ Ò Í‡ÓÚˉ̇ ÒÚÂÌÓÁ‡ (>50%). ãÓ„ËÒÚ˘ÌËflÚ „ÂÒËÓÌÂÌ ‡Ì‡ÎËÁ (͇ÓÚˉ̇ ÒÚÂÌÓÁ‡ - ËÌÒÂˆËfl/‰ÂΈËfl ÔÓÎËÏÓÙËÁ˙Ï Ì‡ ÄëÖ, ‚˙Á‡ÒÚ, ıËÔÂÚÓÌËfl, HDL/LDL) ÓÚÍË‚‡, ˜Â ‰ÂΈËfl/‰ÂΈËfl „ÂÌÓÚËÔ˙Ú Ì‡ ÄëÖ Â ÌÂÁ‡‚ËÒËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ͇ÓÚˉ̇ ÒÚÂÌÓÁ‡ ÔË Ï˙Ê ÌÂÔÛ¯‡˜Ë Ë ·ÂÁ ‰Ë‡·ÂÚ, Ó·‡˜Â Ì ÛÒÚ‡ÌÓ‚fl‚‡ ‚˙Á͇ Ò IMT ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl. Ç˙ÔÂÍË ÚÓ‚‡ ÔË Â‰ËÌ ÏÂÚ‡‡Ì‡ÎËÁ  ÛÒÚ‡ÌÓ‚Â̇ Á̇˜ËÚÂÎ̇ ÔÓÎÓÊËÚÂÎ̇ ‡ÒӈˇˆËfl ̇ D ‡Î· ̇ ÄëÖ „Â̇ Ò IMT ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl, ͇ÍÚÓ ÔË ·ÂÎË, ڇ͇ Ë ÔË ‡ÁË‡ÚˆË (13). è‡ÚӇ̇ÚÓÏ˘ÌËÚÂ Ë ÍÎËÌ˘ÌË ‰‡ÌÌË ÔÓ͇Á‚‡Ú, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò ËÒıÂÏ˘ÌË Î‡ÍÛ̇ÌË ÒË̉ÓÏË Ò‡ ıÂÚÂÓ„ÂÌ̇ „ÛÔ‡. 臈ËÂÌÚËÚÂ Ò ËÁÓÎË‡Ì ·ÍÛ̇ÂÌ ËÌÙ‡ÍÚ ËÏ‡Ú ÎÓ͇ÎËÁË‡Ì‡ ÏËÍÓ‡ÚÂÓχ ‚ ÔÓÍÒËχÎÌËfl Û˜‡ÒÚ˙Í Ì‡ ÔÂÙÓ‡ÌÚÌËÚ ‡ÚÂËË, ‰Ó͇ÚÓ ÚÂÁË Ò ÔˉÛʇ‚‡˘‡ ΂ÍÓ‡ÂÈÓÁ‡ ËÏ‡Ú ‰ËÙÛÁÌÓ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚ /ÎËÔÓıˇÎËÌÓÁ‡/. èË ‚ÚÓ‡Ú‡ „ÛÔ‡ Ô‡ˆËÂÌÚË ÏÌÓ„Ó ÔÓ-˜ÂÒÚÓ Ò ̇·Î˛‰‡‚‡ ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl Ë ÏÌÓÊÂÒÚ‚Ó Î‡ÍÛÌË. ÇÎËflÌËÂÚÓ Ì‡ ÄëÖ „Â̇  ‡Á΢ÌÓ ÏÂÊ‰Û ‰‚ÂÚ „ÛÔË, ÍÓÂÚÓ ÓÚ‡Áfl‚‡ ıÂÚÂÓ„ÂÌÌÓÒÚÚ‡ ̇ ÙÂÌÓÚËÔ‡ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡Ú‡ ·ÓÎÂÒÚ, ‰˙Îʇ˘‡ Ò ̇ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚Â. èË Ô‡ˆËÂÌÚË Ò Î‚ÍÓ‡ÂÈÓÁ‡ Ò ̇·Î˛‰‡‚‡ ÏÌÓ„Ó ÔÓ-‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ „ÂÌÓÚËÔ‡ D/D (‰ÂΈËfl/‰ÂΈËfl ̇ 16 ËÌÚÓÌ Ì‡ ÄëÖ „ÂÌ), ‰Ó͇ÚÓ ÚÓ‚‡ Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË Ô‡ˆËÂÌÚË Ò ËÁÓÎË‡ÌË Î‡ÍÛ̇ÌË ËÌÙ‡ÍÚË. ÖÙÂÍÚËÚ ̇ ÚÓÁË „ÂÌÓÚËÔ Ò‡ ÌÂÁ‡‚ËÒËÏË ÓÚ ıËÔÂÚÓÌËflÚ‡ Ë ‚ÂÓflÚÌÓ Ò‡ Ò‚˙Á‡ÌË Ò ẨÓÚÂÎ̇ ‰ËÒÙÛÌ͈Ëfl (14). ÉÂÌ˙Ú ÍÓ‰Ë‡˘ ‡Ì„ËÓÚÂÌÁËÌÓ„Â̇ /ÒÛ·ÒÚ‡Ú ̇ ÂÌË̇/ Ò˙˘Ó  ËÁÛ˜‡‚‡Ì ÔË Ò˙‰Ó‚ËÚ Á‡·ÓÎfl‚‡ÌËfl. éÔËÒ‡ÌË Ò‡ 5 ı‡ÔÎÓÚËÔ‡ ̇ ÔÓÏÓÚÓ‡ ̇ „Â̇, ÍÓÈÚÓ ÍÓ‰Ë‡ ‡Ì„ËÓÚÂÌÁËÌÓ„Â̇. ìÒÚ‡ÌÓ‚Â̇  Á̇˜Ëχ ‡ÒӈˇˆËfl ÏÂÊ‰Û Ç-ı‡ÔÎÓÚËÔ‡ Ë åêí ÔÓÏÂÌË Í‡ÚÓ ÔË Á‡Òfl„‡Ì ̇ χÎÍËÚ ÏÓÁ˙˜ÌË Ò˙‰Ó‚Â. í‡ÁË ‡ÒӈˇˆËfl  ÌÂÁ‡‚ËÒËχ ÓÚ ‡ÚÂˇÎ̇ڇ ıËÔÂÚÓÌËfl, ÍÓÂÚÓ Ô‰ÔÓ·„‡ ÏÓÁ˙˜ÌÓÒÔˆËÙ˘ÂÌ ÂÙÂÍÚ Ì‡ ÚÓÁË ı‡ÔÎÓÚËÔ. É·‚ÌËflÚ ËÁÚÓ˜ÌËÍ Ì‡ ‡Ì„ËÓÚÂÌÁËÌÓ„ÂÌ ‚ ÏÓÁ˙͇ Ò‡ ‡ÒÚÓˆËÚËÚÂ. ç‡Î˘ËÂÚÓ Ì‡ Ç-ı‡ÔÎÓÚËÔ Û‚Â΢‡‚‡ Ú‡ÌÒÍËÔˆËÓÌ̇ڇ ‡ÍÚË‚ÌÓÒÚ ‚ ‡ÒÚÓˆËÚËÚÂ, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó Ôӂ˯Â̇ ÂÍÒÔÂÒËfl ̇ ‡Ì„ËÓÚÂÌÁËÌÓ„ÂÌ ‚ ÏÓÁ˙͇, ‚ ÂÁÛÎÚ‡Ú Ì‡ ÚÓ‚‡ Ò ·Î‡„ÓÔËflÚÒÚ‚‡ ‡Á‚ËÚËÂÚÓ Ì‡ ·ÓÎÂÒÚ Ì‡ χÎÍËÚ Ò˙‰Ó‚ ‚ ÏÓÁ˙͇ (15). Ö̉ÓÚÂÎ̇ڇ ‰ËÒÙÛÌ͈Ëfl Ëχ ̇È-‚‡Ê̇ ÓÎfl ‚ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡Ú‡ ·ÓÎÂÒÚ ‰˙Îʇ˘‡ Ò ̇ Á‡Òfl„‡ÌÂÚÓ Ì‡ χÎÍËÚ Ò˙‰Ó‚ (16). íÓ‚‡ ‚ÂÓflÚÌÓ Â Ò‚˙Á‡ÌÓ Ò Ì‡Û¯ÂÌË ̇ Í˙‚ÌÓ-ÏÓÁ˙˜Ì‡ ·‡ËÂ‡ (Tomimoto et al., 1996; Lin et al., 2000) Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡Ú‡ ‡‚ÚÓ„Û·ˆËfl (Bakker et al., 1999; Terborg et al.,2000), ÍÓËÚÓ Ò‡ ̇·Î˛‰‡‚‡ÌË ÔË ÚÂÁË Ô‡ˆËÂÌÚË. ÑÓ͇Á‡ÌÓ Â, ËÌ ‚ËÚÓ (Wall et al.1980; Stamler et al., 1993) Ë ËÌ ‚Ë‚Ó (Tawakol et al.,1997; Woo et al.,1997), ˜Â ıÓÏÓˆËÒÚÂËÌ˙Ú Ëχ ‚‡ÊÌË ÂÙÂÍÚË ‚˙ıÛ ÙÛÌ͈ËflÚ‡ ̇ ẨÓÚ·. èӂ˯ÂÌËÚ ÌË‚‡ ̇ ıÓÏÓˆËÒÚÂËÌ ‡ÁÛ¯‡‚‡Ú ẨÓÚÂÎÌËÚ ÍÎÂÚÍË Ë Ì‡Û¯‡‚‡Ú ÚÂıÌËÚ ÍÓ‡„Û·ˆËÓÌÌË „Û·ÚÓÌË ÙÛÌ͈ËË Ë Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ‡ÁÓÚÂÌ ÓÍÒˉ. éÒ‚ÂÌ ÚÓ‚‡ Ò Ë̉ۈË‡ ÔÓÎËÙÂ‡ˆËflÚ‡ ̇ „·‰ÍÓÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË (4). ìÏÂÂ̇ڇ ıËÔÂıÓÏÓˆËÒÚÂËÌÂÏËfl (>15 mmol/l) ÏÓÊ ‰‡ Ò ‰˙ÎÊË Ì‡ „ÂÌÂÚ˘ÌË ‰ÂÙÂÍÚË ËÎË Ù‡ÍÚÓË Ì‡ ÓÍÓÎ̇ڇ Ò‰‡ /ÔËÂÏ Ì‡ ÙÓ·ÚË Ò ı‡Ì‡Ú‡, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ Ë ‰./ ËÎË ÍÓÏ·Ë̇ˆËfl ÓÚ ‰‚ÂÚÂ. ç‡È-˜ÂÒÚËflÚ „ÂÌÂÚ˘ÂÌ ‰ÂÙÂÍÚ Á‡Òfl„‡ „Â̇ ÍÓ‰Ë‡˘ ÂÌÁËχ ÏÂÚËÎÂÌ ÚÂÚ‡ıˉÓÙÓ·Ú

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‰ÛÍÚ‡Á‡. íÓ‚‡  ˜ÂÒÚÓ Ì‡·Î˛‰‡‚‡Ì ÔÓÎËÏÓÙËÁ˙Ï, ÔË ÍÓÈÚÓ Ì‡ ÔÓÁˈËfl 677 ˆËÚÓÁËÌ Â Á‡ÏÂÌÂÌ Ò ÚËÏËÌ, Ú. ë677í. íÓÁË ÔÓÎËÏÓÙËÁ˙Ï Ò ‡ÒÓˆËË‡ Ò ıËÔÂıÓÏÓˆËÒÚÂËÌÂÏËfl /ÒΉÒÚ‚Ë ̇χÎÂ̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÏÂÚËÎÂÌ ÚÂÚ‡ıˉÓÙÓÎ‡Ú ‰ÛÍÚ‡Á‡Ú‡/, ͇ÚÓ Ì‡È-‚ËÒÓÍË ÌË‚‡ ̇ ıÓÏÓˆËÒÚÂËÌ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË ıÓÏÓÁË„ÓÚËÚ ÔÓ ÚÓÁË ‡ÎÂÎ, Ú. ÔË íí „ÂÌÓÚËÔ (Fross et al., 1995). çflÍÓË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ıÓÏÓˆËÒÚÂËÌ˙Ú Â ÔÓÁ̇˜ËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡Ú‡ ·ÓÎÂÒÚ ‰˙Îʇ˘‡ Ò ̇ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚ ‚ Ò‡‚ÌÂÌËÂ Ò ‰Û„ËÚ ÚËÔÓ‚Â ËÌÒÛÎÚ (Evers et al.,1997; Fassbender et al., 1999). Ö‰ÌÓ Ì‡ÒÍÓÓ ÔÛ·ÎËÍÛ‚‡ÌÓ ÔÓÛ˜‚‡Ì ÔÓÚ‚˙ʉ‡‚‡ ÓÎflÚ‡ ̇ ıÓÏÓˆËÒÚÂË̇ ÔË ÚÓÁË ÚËÔ Ì‡ åëÅ, ÌÓ ÔÓ͇Á‚‡, ˜Â  ÏÌÓ„Ó ÔÓ-Á̇˜ËÏ ËÒÍÓ‚ Ù‡ÍÚÓ ÔË ËÒıÂÏ˘̇ڇ ΂ÍÓ‡ÂÈÓÁ‡, ÓÚÍÓÎÍÓÚÓ ÔË ËÁÓÎË‡Ì ·ÍÛ̇ÂÌ ËÌÙ‡ÍÚ. éÒ‚ÂÌ ÚÓ‚‡  ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â ÍÓÎÍÓÚÓ ÔÓ-‚ËÒÓ͇  ÍÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ ıÓÏÓˆËÒÚÂË̇, ÚÓÎÍÓ‚‡ ÔÓ-‚ËÒÓÍ Â ËÒÍ˙Ú Á‡ ‡Á‚ËÚË ̇ åëÅ ‰˙Îʇ˘‡ Ò ̇ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚Â. èÓÎËÏÓÙËÁÏ˙Ú ë677í  ¯ËÓÍÓ Á‡ÒÚ˙ÔÂÌ ‚ „ÛÔ‡Ú‡ Ò ËÒıÂÏ˘̇ ΂ÍÓ‡ÂÈÓÁ‡, ÌÓ ÌÂ Ë ‚ „ÛÔ‡Ú‡ Ò ËÁÓÎË‡Ì ·ÍÛ̇ÂÌ ËÌÙ‡ÍÚ, ÍÓÂÚÓ ÓÚ‡Áfl‚‡ ÛÒÚ‡ÌÓ‚ÂÌËÚ ÔÓ-‚ËÒÓÍË ÌË‚‡ ̇ ıÓÏÓˆËÒÚÂËÌ ÔË „ÛÔ‡Ú‡ Ô‡ˆËÂÌÚË Ò Î‚ÍÓ‡ÂÈÓÁ‡. íÂÁË ÂÁÛÎÚ‡ÚË ËÏ‡Ú „ÓÎflÏÓ Á̇˜ÂÌË Á‡ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇. çË‚‡Ú‡ ̇ ıÓÏÓˆËÒÚÂË̇ ÏÓ„‡Ú ‰‡ Ò ̇χÎflÚ ˜ÂÁ ÙÓÎ‡Ú Ë ‚ËÚ. Ç12. ç‡È-ÒËÎÌÓ ÌË‚ÓÚÓ Ì‡ ıÓÏÓˆËÒÚÂË̇ Ò ‚ÎËfl ÓÚ ÌË‚ÓÚÓ Ì‡ ÙÓÎË‚‡Ú‡ ÍËÒÂÎË̇ ÔË 677 íí „ÂÌÓÚËÔ / ͇ÍÚÓ Ò ÛÚÓ˜ÌË ÚÓÈ Â ¯ËÓÍÓ Á‡ÒÚ˙ÔÂÌ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ËÒıÂÏ˘̇ ΂ÍÓ‡ÂÈÓÁ‡/. ç‡Ï‡Îfl‚‡ÌÂÚÓ Ì‡ ıÓÏÓˆËÒÚÂËÌÓ‚ËÚ ÌË‚‡ Ò ÙÓÎË‚‡ ÍËÒÂÎË̇ Ëχ ·Î‡„ÓÔËflÚÌË ÂÙÂÍÚË ‚˙ıÛ ẨÓÚÂÎ̇ڇ ÙÛÌ͈Ëfl. ëΉӂ‡ÚÂÎÌÓ ‡ÍÓ ·˙‰Â ‰Ó͇Á‡Ì ÂÙÂÍÚ ÓÚ Î˜ÂÌËÂ Ò ‚ËÚ‡ÏËÌË Ë ÙÓÎË‚‡ ÍËÒÂÎË̇, ÚÓ ÚÓÈ ˘Â ·˙‰Â ‡Á΢ÂÌ ÔË ‡Á΢ÌËÚ ËÌÒÛÎÚË, ͇ÚÓ ·Ë ·ËÎ ÓÒÓ·ÂÌÓ ‰Ó·˙ ÔË Ô‡ˆËÂÌÚË Ò Î‚ÍÓ‡ÂÈÓÁ‡ (6). ÄÁÓÚÌËflÚ ÓÍÒˉ, ÒËÌÚÂÁË‡Ì ÓÚ ÂÌÚÓ‰ÂÎ̇ڇ ‡ÁÓÚÂÌ ÓÍÒˉ ÒËÌÚÂÚ‡Á‡ (eNOS),  ‚‡ÊÂÌ Ï‰ˇÚÓ ̇ ẨÓÚÂÎ̇ڇ ÙÛÌ͈Ëfl. íÓÈ ÔÓ‚ÎËfl‚‡ ÚÓÌÛÒ‡, ËÌÚ„ËÚÂÚ‡, ÚÓÏ·Ó„ÂÌÌËÚ ͇˜ÂÒÚ‚‡ ̇ Ò˙‰Ó‚‡Ú‡ ÒÚÂ̇. èÓÛ˜‚‡ÌËfl ÔË ıÓ‡ Ë ÔË ÊË‚ÓÚÌË ÔÓ͇Á‚‡Ú, ˜Â ÒËÌÚÂÁ‡Ú‡ ̇ ‡ÁÓÚÂÌ ÓÍÒˉ Ò ‚ÎËfl ÓÚ „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË (2). àχ ÌflÍÓÎÍÓ ÔÓÎËÏÓÙËÁχ ̇ „Â̇, ÍÓÈÚÓ ÍÓ‰Ë‡ eNOS. àÌÚÓÌ 4‡ ‚‡ˇÌÚ˙Ú Ô‰ԇÁ‚‡ ÓÚ Î‡ÍÛ̇ÂÌ ËÌÙ‡ÍÚ ˜ÂÁ ÔÓÏÂÌË ‚ ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ ÔÓÏÓÚÓ‡ ̇ „Â̇ Ë Û‚Â΢ÂÌË ÌË‚‡ ̇ ‡ÁÓÚÂÌ ÓÍÒˉ. àÌÚÓÌ 4‡b ËÌÒÂˆËfl/‰ÂΈËfl „ÂÌÓÚËÔ Ò ‡ÒÓˆËË‡ Ò ËÁÓÎË‡Ì ·ÍÛ̇ÂÌ ËÌÙ‡ÍÚ, ÌÓ ÌÂ Ë Ò ËÒıÂÏ˘̇ ΂ÍÓ‡ÂÈÓÁ‡. íÓ‚‡ ÓÚ‡Áfl‚‡ ‡Á΢̇ڇ Ô‡ÚÓ„ÂÌÂÁ‡ ̇ ‰‚‡Ú‡ ÔÓ‰ÚËÔ‡ ̇ åëÅ ÓÚ Á‡Òfl„‡Ì ̇ χÎÍËÚ Ò˙‰Ó‚Â. ãËÔÒ‡Ú‡ ̇ ‡ÁÓÚÂÌ ÓÍÒˉ Ô‰‡ÁÔÓ·„‡ Í˙Ï Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ÏËÍÓ‡ÚÂÓχ ‚ ÔÓÍÒËχÎÌËfl Û˜‡ÒÚ˙Í Ì‡ ‡ÚÂËÓÎËÚÂ, ‡ Ì Í˙Ï ÚflıÌÓÚÓ ‰ËÙÛÁÌÓ ‰ËÒÚ‡ÎÌÓ Á‡Òfl„‡Ì /͇ÍÚÓ Â ÔË ËÒıÂÏ˘̇ڇ ΂ÍÓ‡ÂÈÓÁ‡ (17). чÌÌËÚ ÔÓÒÓ˜ÂÌË ÔÓ-„Ó Ӣ ‚‰Ì˙Ê ÔÓÚ‚˙ʉ‡‚‡Ú, ˜Â Ëχ ‡Á΢ÌË „ÂÌÂÚ˘ÌË ‚ÎËflÌËfl ÔË ‡Á΢ÌËÚ ԇÚÓ„ÂÌÂÚ˘ÌË ÏÂı‡ÌËÁÏË, ÍÓËÚÓ ‚Ó‰flÚ ‰Ó ËÌÒÛÎÚ. íÓ‚‡ ÓÚÌÓ‚Ó ÔÓ‰˜ÂÚ‡‚‡ ÓÎflÚ‡ ̇ ÔˆËÁÌÓÚÓ ÙÂÌÓÚËÔËÁË‡Ì ÔË ËÁÒΉ‚‡ÌÂÚÓ Ì‡ „ÂÌÂÚË͇ڇ ̇ ËÌÒÛÎÚ‡. ÄÚÂÓÒÍÎÂÓÁ‡Ú‡  ‚ ÓÒÌÓ‚‡Ú‡ ̇ ͇‰ËÓÂÏ·Ó΢ÌËfl ËÌÒÛÎÚ Ë ËÌÒÛÎÚ‡, ‰˙Îʇ˘ Ò ̇ Á‡Òfl„‡Ì ̇ „ÓÎÂÏËÚ ÂÍÒÚ‡- Ë ËÌÚ‡- Í‡ÌˇÎÌË ÏÓÁ˙˜ÌË Ò˙‰Ó‚Â. ÄÚÂÓÒÍÎÂÓÁ‡Ú‡  ÒÎÓÊÂÌ ıÓÌ˘ÂÌ ‚˙ÁÔ‡ÎËÚÂÎÂÌ ÔÓˆÂÒ, ‚ ÍÓÈÚÓ Û˜‡ÒÚ‚‡Ú ÏÌÓ„Ó ÍÎÂÚÍË /ẨÓÚÂÎÌË, „·‰ÍÓÏÛÒÍÛÎÌË, íÎËÏÙÓˆËÚË, ÏÓÌÓˆËÚË, χÍÓÙ‡„Ë, ÌÂÛÚÓÙËÎË, ÚÓÏ·ÓˆËÚË/, ˆËÚÓÍËÌË, ‡ÒÚÂÊÌË Ù‡ÍÚÓË, ıÂÏÓÚ‡ÍÒ˘ÌË Ù‡ÍÚÓË, ÓÍÒˉË‡ÌË LDL, Ò‚Ó·Ó‰ÌË ‡‰Ë͇ÎË (18). åÌÓÊÂÒÚ‚Ó „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË ÔÓ‚ÎËfl‚‡Ú ‡ÁÌÓÓ·‡ÁÌË Á‚Â̇ ÓÚ ÚÓÁË ‰˙Î˙„ Ô‡ÚÓÎӄ˘ÂÌ ÔÓˆÂÒ, ͇ÚÓ ÌflÍÓË ÓÚ


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

Úflı „Ó ÒÚËÏÛÎË‡Ú, Ú. ҇ ÔÓ‡ÚÂÓ„ÂÌÌË, ‡ ‰Û„Ë „Ó ÔÓÚËÒÍ‡Ú /ÔÓÚÂÍÚË‚ÌË/. LJˇÌÚË Ì‡ „Â̇ ALOX5AP, ÍÓÈÚÓ ÍÓ‰Ë‡ 5-ÎËÔÓÓÍÒË„Â̇Á‡ ‡ÍÚË‚Ë‡˘ ÔÓÚÂËÌ Ò‡ ‡ÒÓˆËË‡ÌË Ò Ôӂ˯ÂÌ ËÒÍ /‰‚‡ Ô˙ÚË ÔÓ-„ÓÎflÏ ‚ ËÁÒΉ‚‡Ì‡Ú‡ ÍÓıÓÚ‡ ‚ àÒ·̉Ëfl/ ÓÚ ÏËÓ͇‰ÂÌ ËÌÙ‡ÍÚ Ë ËÌÒÛÎÚ. 5-ÎËÔÓÓÍÒË„Â̇Á‡ ‡ÍÚË‚Ë‡˘ËflÚ ÔÓÚÂËÌ Ëχ ‚‡Ê̇ ÓÎfl ‚ ̇˜‡ÎÌËÚ ÂÚ‡ÔË ‚ ·ËÓÒËÌÚÂÁ‡Ú‡ ̇ ΂ÍÓÚËÂÌËÚÂ, ÍÓËÚÓ Ò‡ Ò˙Ò‰ÓÚÓ˜ÂÌË ‚ ΂ÍÓˆËÚËÚÂ Ë Ò Á‡ÔÛÒÍ‡Ú ÓÚ ‡ÁÌÓÓ·‡ÁÌË ÒÚËÏÛÎË. èË ÚÓÁË ·ËÓÒËÌÚÂÚ˘ÂÌ Ô˙Ú ÓÚ ÌÂÂÒÚÂËÙˈË‡Ì‡ ‡‡ıˉÓÌÓ‚‡ ÍËÒÂÎË̇ Ò ÔÓÎÛ˜‡‚‡ ΂ÍÓÚËÂÌ Ä4 ÔÓ‰ ‰ÂÈÒÚ‚ËÂÚÓ Ì‡ 5-ÎËÔÓÓÍÒË„Â̇Á‡Ú‡ Ë ÌÂÈÌËfl ‡ÍÚË‚Ë‡˘ ÔÓÚÂËÌ. ã‚ÍÓÚËÂÌ Ä4  ÌÂÒÚ‡·ËÎÂÌ ÂÔÓÍÒˉ, ÍÓÈÚÓ Ò ÏÂÚ‡·ÓÎËÁË‡ ‰Ó ΂ÍÓÚËÂÌ Ç4 Ë Î‚ÍÓÚËÂÌ ë4. ã‚ÍÓÚËÂÌËÚ ËÏ‡Ú ‡ÁÌÓÓ·‡ÁÌË ÔÓËÌÙ·χÚÓÌË ÂÙÂÍÚË. ã‚ÍÓÚËÂÌ Ç4 ‡ÍÚË‚Ë‡ ΂ÍÓˆËÚËÚÂ Ë ÒÚËÏÛÎË‡ Úflı̇ڇ ‡‰ıÂÁËfl Í˙Ï ‡Ì‰ÓÚ·, ÓÒ‚Ó·Óʉ‡‚‡ÌÂÚÓ Ì‡ ÎËÁÓÁÓχÎÌË ÂÌÁËÏË /̇Ô. ÏËÂÎÓÔÂÓÍÒˉ‡Á‡/ Ë ÔÓ‰Û͈ËflÚ‡ ̇ ÒÛÔÂÓÍÒˉÌË ‡ÌËÓÌË. êÓÎflÚ‡ ̇ ΂ÍÓÚËÂÌËÚ ‚ „ÂÌÂÁ‡Ú‡ ̇ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡  ‰Ó͇Á‡Ì‡ ÔË ÊË‚ÓÚÌË Ë ıÓ‡. ì‚Â΢Â̇ ÂÍÒÔÂÒËfl ̇ 5-ÎËÔÓÓÍÒË„Â̇Á‡Ú‡ Ë ÌÂÈÌËfl ‡ÍÚË‚Ë‡˘ ÔÓÚÂËÌ Â ÛÒÚ‡ÌÓ‚Â̇ ‚ ‡ÚÂÓÒÍÎÂÓÚ˘ÌËÚ Ô·ÍË ‚ ‡Á΢ÂÌ ÒÚ‡‰ËÈ Ì‡ ‡Á‚ËÚËÂ. ã‚ÍÓˆËÚË ·Ó„‡ÚË Ì‡ 5-ÎËÔÓÓÍÒË„Â̇Á‡ Ò ̇ÚÛÔ‚‡Ú ‚ ÏÂÒÚ‡Ú‡ ̇ Ô·͇ڇ, ÍÓËÚÓ Ì‡È-ÎÂÒÌÓ ÛÔÚÛË‡Ú. Ö‰ËÌ ‚‡ˇÌÚ Ì‡ ÔÓÏÓÚÓ‡ ̇ „Â̇ ÍÓ‰Ë‡˘ 5-ÎËÔÓÓÍÒË„Â̇Á‡Ú‡  ‡ÒÓˆËË‡Ì Ò ‡·ÌÓÏ̇ IMT ̇ Ò˙Ì̇ڇ ‡ÚÂËfl Ë Û‚Â΢ÂÌË Ï‡ÍÂË Ì‡ ‚˙ÁÔ‡ÎÂÌËÂÚÓ. HapA /ı‡ÔÎÓÚËÔ˙Ú Ì‡ ALOX5AP Ò‚˙Á‡Ì Ò Ôӂ˯ÂÌ ËÒÍ ÓÚ ËÌÒÛÎÚ/ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ò ‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ÔË ËÒıÂÏ˘ÂÌ Ë ıÂÏÓ‡„˘ÂÌ ËÌÒÛÎÚ, ͇ÍÚÓ Ë ÔË Ú‡ÌÁËÚÓÌË ËÒıÂÏ˘ÌË ‡Ú‡ÍË ÔË ËÒ·̉ˆËÚÂ. èË ‰Û„Ë ÔÓÔÛ·ˆËË /·Ëڇ̈Ë/ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‰Û„ ÔÓ‡ÚÂÓ„ÂÌÂÌ ‚‡ˇÌÚ Ì‡ Ò˙˘Ëfl „ÂÌ. èË ˜ÂÒÚÓ Ò¢‡ÌË Á‡·ÓÎfl‚‡ÌËfl, ͇ÚÓ ÏËÓ͇‰ÂÌ ËÌÙ‡ÍÚ Ë ËÌÒÛÎÚ, Ëχ ̇È-‡Á΢ÌË ÏÛÚ‡ˆËË ‚ ‰ËÌ Ë Ò˙˘Ë „ÂÌ, ͇ÚÓ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‡Á΢ÌËÚ ‡ÎÂÎË Ò‚˙Á‡ÌË Ò˙Ò Á‡·ÓÎfl‚‡ÌÂÚÓ ÏÓÊ ‰‡  ‡Á΢̇ ‚ ‡Á΢ÌËÚ ÔÓÔÛ·ˆËË (19). íÓ‚‡ ÔÓÛ˜‚‡Ì  ËÁÔÓÎÁ‚‡ÎÓ ‰Û„ ÔÓ‰ıÓ‰ /Ú. Ì ͇̉ˉ‡Ú-„ÂÌ ÏÂÚÓ‰‡/ - Ú.̇. ÎËÌÍË‰Ê ‡Ì‡ÎËÁ. óÂÁ ÎËÌÍË‰Ê ‡Ì‡ÎËÁ, ÔËÎÓÊÂÌ ÓÚÌÓ‚Ó ‚˙ıÛ ËÒ·̉Ò͇ڇ ÔÓÔÛ·ˆËfl,  ÛÒÚ‡ÌÓ‚ÂÌ ÎÓÍÛÒ, ‡ÒÓˆËË‡Ì Ò ËÌÒÛÎÚ ‚˙ıÛ 5q. Ç ÚÓÁË ÎÓÍÛÒ ÔÂ‰Ë „Ó‰Ë̇  ÓÚÍËÚ ÓÚ„Ó‚ÓÌËflÚ „ÂÌ - „ÂÌ˙Ú ÍÓ‰Ë‡˘ ÙÓÒÙÓ‰ËÂÒÚÂ‡Á‡ 4D (PDE4D) (20). íÓÈ Ò ‡ÒÓˆËË‡ Ò ÔÓ‰ÚËÔÓ‚ÂÚ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‰˙Îʇ˘Ë Ò ̇ ‡ÚÂÓÒÍÎÂÓÁ‡ /Ú. ͇‰ËÓÂÏ·Ó΢ÌËfl Ë ÚÓÁË ‰˙Îʇ˘ Ò ̇ Á‡Òfl„‡Ì ̇ „ÓÎÂÏËÚ Ò˙‰Ó‚Â/, Ú‡ÁË ‡ÒӈˇˆËfl  ÌÂÁ‡‚ËÒËχ ÓÚ ÍÓÌ‚Â̈ËÓ̇ÎÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË. ç  ÛÒÚ‡ÌÓ‚Â̇ ‚˙Á͇ Ò Î‡ÍÛ̇ÌËfl ËÌÒÛÎÚ Ë Î‚ÍÓ‡ÂÈÓÁ‡Ú‡, ÍÓËÚÓ ËÏ‡Ú ‰Û„‡ Ô‡ÚÓ„ÂÌÂÁ‡. ï‡ÔÎÓÚËÔÓ‚ÂÚ ̇ „Â̇ Á‡Òfl„‡˘Ë Ì„ӂËfl Ô˙‚Ë ÂÍÁÓÌ ÏÓ„‡Ú ‰‡ Ò Í·ÒËÙˈË‡Ú ‚ ÚË „ÛÔË – ÔÓÚÂÍÚË‚ÂÌ, ÌÓχÎÂÌ Ë ËÒÍÓ‚. éÍÓÎÓ 16% ÓÚ ÔÓÔÛ·ˆËflÚ‡ Ò‡ ÌÓÒËÚÂÎË Ì‡ ËÒÍÓ‚ ı‡ÔÎÓÚËÔ Ë ËÏ‡Ú 1,8 Ô˙ÚË ÔÓ-‚ËÒÓÍ ËÒÍ ÒÔflÏÓ Ó·˘‡Ú‡ ÔÓÔÛ·ˆËfl. éÍÓÎÓ 0,8% ÓÚ ÔÓÔÛ·ˆËflÚ‡ Ò‡ ıÓÏÓÁË„ÓÚË Ë ÔË Úflı ËÒ͇  3,8 Ô˙ÚË ÔÓ-„ÓÎflÏ. é·‡˜Â Ì ҇ ÛÒÚ‡ÌÓ‚ÂÌË ÏÛÚ‡ˆËË ‚ ËÁ‚ÂÒÚÌËÚ ÍÓ‰Ë‡˘Ë ÔÓÒΉӂ‡ÚÂÎÌÓÒÚË Ì‡ „Â̇ Ë ÒΉӂ‡ÚÂÎÌÓ Ìflχ ‡Á΢ÌË ÙÛÌ͈ËÓ̇ÎÌË ‚‡ˇÌÚË Ì‡ „Â̇, ÍÓËÚÓ ‰‡ Ó·flÒÌflÚ Ì‡·Î˛‰‡‚‡ÌËÚ ‡Á΢Ëfl ‚ „ÛÔËÚÂ Ò ‡Á΢ÌË ı‡ÔÎÓÚËÔÓ‚Â. óÂÁ ‡ÎÚÂ̇ÚË‚ÂÌ ÒÔ·ÈÒËÌ„ Ë ‡ÍÚË‚Ë‡Ì ̇ ‡Á΢ÌË ÔÓÏÓÚÓË PDE4D Ó·‡ÁÛ‚‡ ÔÓÌ ÓÒÂÏ ‡Á΢ÌË ËÁÓÙÓÏË, ÓÚ ÍÓËÚÓ ÔÓËÁıÓʉ‡Ú ÔÓÚÂËÌË ‡Á΢‡‚‡˘Ë Ò ‚ ÚÂıÌËfl N-Í‡È. èË Ô‡ˆËÂÌÚËÚÂ Ò ËÌÒÛÎÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ̇χÎÂ̇ ÂÍÒÔÂÒËfl ̇ ËÁÓÙÓÏËÚ PDE4D1, PDE4D2,

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PDE4D5. êËÒÍÓ‚ËflÚ ı‡ÔÎÓÚËÔ Ó·ı‚‡˘‡ Ë 5?-Í‡fl Ë Ô‰ÔÓ·„‡ÂÏËfl ÔÓÏÓÚÓ ̇ ÌÓ‚‡Ú‡ ˉÂÌÚËÙˈË‡Ì‡ ËÁÓÙÓχ PDE4D7, ÍÓÂÚÓ Ô‰ÔÓ·„‡ ‚˙ÁÏÓÊ̇ ÙÛÌ͈ËÓ̇Î̇ ‚‡ˇˆËfl Ò‚˙Á‡Ì‡ Ò „Û·ˆËflÚ‡ ̇ Ú‡ÌÒÍËÔˆËflÚ‡. íÓ‚‡ Ò ÔÓ‰ÍÂÔfl ÓÚ ‡Ì‡ÎËÁ‡ ̇ ÂÍÒÔÂÒËflÚ‡ ̇ PDE4D, ÍÓÈÚÓ ÔÓ͇Á‚‡ Á̇˜ËÚÂÎ̇ ÍÓ·ˆËfl ÏÂÊ‰Û ËÒÍÓ‚Ëfl ı‡ÔÎÓÚËÔ Ë ÌË‚ÓÚÓ Ì‡ PDE4D7. PDE4D ÔË̇‰ÎÂÊË Í˙Ï „ÓÎflχڇ „ÛÔ‡ ̇ ÙÓÒÙÓ‰ËÂÒÚÂ‡ÁËÚÂ. íÓÁË ÂÌÁËÏ ‡Á„‡Ê‰‡ ˆÄåî, ÍÓÈÚÓ Â ‚ÚÓ˘ÂÌ ÔÓÒ‰ÌËÍ Ë Ë„‡Â ‚‡Ê̇ ÓÎfl ‚ ÍÎÂÚ˙˜Ì‡Ú‡ Ò˄̇ÎËÁ‡ˆËfl Ë „Û·ˆËflÚ‡ ̇ ‰ˈ‡ ÙËÁËÓÎӄ˘ÌË ÔÓˆÂÒË. ÖÌÁËÏ˙Ú Ò ÂÍÒÔÂÒË‡ ‚ Ò˙‰Ó‚ËÚ „·‰ÍÓÏÛÒÍÛÎÌË ÍÎÂÚÍË, ẨÓÚÂÎÌËÚ ÍÎÂÚÍË, í-ÎËÏÙÓˆËÚËÚÂ, χÍÓÙ‡„ËÚÂ, ÏÓÌÓˆËÚËÚ – ÍÎÂÚÍË, ÍÓËÚÓ Ò‡ ‚‡ÊÌË Á‡ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡. Ç Ò˙‰Ó‚ËÚ „·‰ÍÓÏÛÒÍÛÎÌË ÍÎÂÚÍË ÌËÒÍËÚ ÌË‚‡ ̇ ˆÄåî ‚Ó‰flÚ ‰Ó Û‚Â΢Â̇ ÔÓÎËÙÂ‡ˆËfl Ë ÏË„‡ˆËfl, ÍÓÂÚÓ Â ÔÓÌ ÓÚ˜‡ÒÚË Ï‰ËË‡ÌÓ ÓÚ PDE4D. èË ÊË‚ÓÚÌË Â ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â Ôӂ˯ÂÌËÚ ÌË‚‡ ̇ ˆÄåî ̇χÎfl‚‡Ú ÙÓχˆËflÚ‡ ̇ ÌÓ‚Ë ËÌÚËχÎÌË ÎÂÁËË Ë ËÌıË·Ë‡Ú ÔÓÎËÙÂ‡ˆËflÚ‡ ̇ „·‰ÍÓÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË ÒΉ Û‚‰‡ ̇ ‡ÚÂˇÎ̇ڇ ÒÚÂ̇. Ç ÏÓÌÓˆËÚËÚÂ Ë í-ÎËÏÙÓˆËÚËÚ ̇ÚÛÔ‚‡ÌÂÚÓ Ì‡ ˆÄåî Ò ‡ÒÓˆËË‡ Ò ËÌıË·Ë‡Ì ̇ Úflı̇ڇ ÔÓÎËÙÂ‡ˆËfl Ë ÒÂÍˆËflÚ‡ ̇ ˆËÚÓÍËÌË, ÍÓËÚÓ Í‡ÍÚÓ ·Â¯Â ÔÓÒÓ˜ÂÌÓ, Ò˙˘Ó Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡. éÚ ÚÓ‚‡ ·Ë ÒΉ‚‡ÎÓ, ˜Â ÔË Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë ˘Â Ëχ Ôӂ˯Â̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ PDE4D, ÌËÒÍË ÌË‚‡ ̇ ˆÄåî Ë ‡Á‚ËÚË ̇ ‡ÚÂÓÒÍÎÂÓÁ‡. èÓÚË‚ÌÓ Ì‡ Ó˜‡Í‚‡ÌÓÚÓ, ͇ÍÚÓ ‚˜ Ò ÒÔÓÏÂ̇, ÔË ÚÓ‚‡ ÔÓÛ˜‚‡Ì  ÛÒÚ‡ÌÓ‚Â̇ ÔÓÌËÊÂ̇ ÂÍÒÔÂÒËfl ̇ ÌflÍÓË ÓÚ ËÁÓÙÓÏËÚ ̇ PDE4D ÔË Á‡Ò„̇ÚËÚ Ë̉˂ˉË. ÖÍÒÔÂÒËflÚ‡ ̇ ÚÂÁË ËÁÓÙÓÏË Ò „ÛÎË‡ ÓÚ ˆÄåî, ÒΉӂ‡ÚÂÎÌÓ ÔË Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Ëχ ‰ËÒ„Û·ˆËfl ̇ ÌË‚Ó ˆÄåî. Ç˙ÁÏÓÊÌÓ Â Ôӂ˯Â̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ‰ËÌ ËÎË ÌflÍÓÎÍÓ ÓÚ ÒÔ·ÈÒËÌ„‚‡ˇÌÚËÚ ‰‡ ÔÓÏÂÌfl ÂÌÁËÏ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì‡ PDE4D ‚ ÍÎÂÚ͇ڇ, ‰‡ ̇χÎfl‚‡ ÌË‚ÓÚÓ Ì‡ ˆÄåî Ë Ú‡Í‡ ‰‡ ÔÓÏÂÌfl ÂÍÒÔÂÒËflÚ‡ ̇ ˆÄåî-„ÛÎË‡ÌËÚ ËÁÓÙÓÏË, ͇ÍÚÓ Â Ì‡·Î˛‰‡‚‡ÌÓ ÔË ÚÓ‚‡ ÔÓÛ˜‚‡ÌÂ.(1,3,20) ÉÓÎflÏÓ Â Á̇˜ÂÌËÂÚÓ Ì‡ ÚÂÁË ÌÓ‚Ë ÓÚÍËÚËfl, ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡, ‡ ÓÚ ‰Û„‡ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ΘÂÌËÂÚÓ Ì‡ ËÌÒÛÎÚ‡ – Ò˙Á‰‡‚‡ÌÂÚÓ Ì‡ ÎÂ͇ÒÚ‚‡ ËÌıË·Ë‡˘Ë Ò˙ÓÚ‚. ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ 5-ÎËÔÓÓÍÒË„Â̇Á‡ ‡ÍÚË‚Ë‡˘Ëfl ÔÓÚÂËÌ Ë Ì‡ PDE4D, Á‡ Ô‰ԇÁ‚‡Ì ÓÚ ËÌÒÛÎÚ.(19,20) ê‡Á΢ÌË ÍÓÏ·Ë̇ˆËË ÓÚ ÔÓÎËÏÓÙËÁÏË Ì‡ „ÂÌËÚ ÍÓ‰Ë‡˘Ë ‰ˈ‡ ËÌÙ·χÚÓÌË ÏÓÎÂÍÛÎË Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË Ô‡ˆËÂÌÚË Ò ÔÂÊË‚flÌ ËÌÒÛÎÚ, ͇ÚÓ ÂÙÂÍÚËÚ ËÏ Ò‡ ÒËÌÂ„˘ÌË, Ú. ËÒ͇ ̇‡ÒÚ‚‡ Ò Û‚Â΢‡‚‡Ì ̇ ·Ófl ̇ ÔÓËÌÙ·χÚÓÌËÚ „ÂÌÌË ‚‡ˇÌÚË. íÓ‚‡ Ò‡ ÌflÍÓË ÔÓÎËÏÓÙËÁÏË Ì‡ „ÂÌË ÍÓ‰Ë‡˘Ë: ËÌÚÂ΂ÍËÌ 6, åëê-1 /ÏÓÌÓˆËÚ ıÂÏÓÚ‡ÍÒ˘ÂÌ ÔÓÚÂËÌ 1/, ICAM-1 /ËÌÚÂˆÂÎÛ·̇ ‡‰ıÂÁËÓÌ̇ ÏÓÎÂÍÛ· –1/, E-sel /Ö-ÒÂÎÂÍÚËÌ/, ååê3 /χÚËÍÒ̇ ÏÂÚ‡ÎÓÔÓÚÂË̇Á‡ 3/. ëΉÌËÚ „ÂÌÓÚËÔÓ‚Â Ò ̇·Î˛‰‡‚‡Ú ÔË Ô‡ˆËÂÌÚË Ò ÔÂÊË‚flÌ ËÌÒÛÎÚ: IL-6 GG, IL-6 GC, MCP-1 GG, ICAM-1 EE, E-sel AA, MMP-3 5A5A (23). éÒ‚ÂÌ Ò‚˙Á‡ÌË Ò Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ËÌÒÛÎÚ‡, ÌflÍÓË ÔÓËÌÙ·χÚÓÌË ˆËÚÓÍËÌË Ë„‡flÚ ÓÎfl Ë ÔË Ì„ӂËfl ËÁıÓ‰. èË ÚÛÏÓÌÂÍÓÚËÁË‡˘Ëfl Ù‡ÍÚÓ ‡ÎÙ‡ (TNFalpha) Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË „ÓÎÂÏË ‡Á΢Ëfl ‚ Ì„ӂËÚ ÌË‚‡ ÔË ‡Á΢ÌËÚ Ë̉˂ˉË. Ç ÓÒÌÓ‚‡Ú‡ ̇ ÚÓ‚‡ Ò‡ ‡Á΢ÌËÚ „ÂÌÂÚ˘ÌË ‚‡ˇÌÚË Ì‡ TNF-alpha „Â̇. í‡ÌÁˈËflÚ‡ G➞A ̇ 308 ÔÓÁˈËfl ‚ ÔÓÏÓÚÓ‡ ̇ „Â̇ Ëχ ‰ËÂÍÚÂÌ ÂÙÂÍÚ ‚˙ıÛ „Û·ˆËflÚ‡ Ë ‚Ó‰Ë ‰Ó Ôӂ˯ÂÌË ÌË‚‡ ̇ TNF-alpha. èË Â‰ÌÓ ÔÓÛ˜‚‡Ì Ôӂ‰ÂÌÓ ÔË Ô‡ˆËÂÌÚË Ò ËÌÒÛÎÚ ‚ èÓί‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â ÌÓÒËÚÂÎÒÚ‚ÓÚÓ Ì‡ ‰ËÌ Ú‡Í˙‚ ‡ÎÂÎ, Ú. „ÂÌÓÚËÔ G/A, Ò ‡ÒÓˆËË‡ Ò˙Ò Á̇-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˜ËÚÂÎÌÓ ËÁ‡ÁÂÌ Ì··„ÓÔËflÚÂÌ ËÁıÓ‰ ÓÚ ËÌÒÛÎÚ‡, ËÁÏÂÂÌÓ ˜ÂÁ Scandinavian Stroke Scale, Barthel Stroke Ë Scale Rankin Scale ‚ Ò‡‚ÌÂÌËÂ Ò ıÓÏÓÁË„ÓÚËÚ ÔÓ ÌÓχÎÌËfl ‡ÎÂÎ /Ú. „ÂÌÓÚËÔ G/G/. ëΉӂ‡ÚÂÎÌÓ „ÂÌÂÚ˘ÌËflÚ ÔÓÎËÏÓÙËÁ˙Ï Ì‡ TNF-alpha „Â̇ ÔÓ‚ÎËfl‚‡ ËÁıÓ‰‡ ÓÚ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ (G. Gromadska, M. Baranska et al., 2003). ÉÂÌÂÚ˘ÌËÚ هÍÚÓË ÏÓ„‡Ú ‰‡ ‚Á‡ËÏÓ‰ÂÈÒÚ‚‡Ú Ò Ù‡ÍÚÓËÚ ̇ ÓÍÓÎ̇ڇ Ò‰‡ Ë Ú‡Í‡ ‰‡ ÔÓÏÂÌflÚ ËÒ͇ Á‡ ËÌÒÛÎÚ. èÛ¯ÂÌÂÚÓ Â ÛÚ‚˙‰ÂÌ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ‡Á‚ËÚË ̇ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡ Ë ËÌÒÛÎÚ‡. íÓ Ë„‡Â ÓÎfl ̇ ÔÓËÌÙ·χÚÓÂÌ ÒÚËÏÛÎ, ‚ÂÓflÚÌÓ ‚˙ÁÔ‡ÎÂÌËÂÚÓ Ë ÓÚ‰ÂÎflÌÂÚÓ Ì‡ ‡Á΢ÌË ˆËÚÓÍËÌË, Ò‡ ÏÂı‡ÌËÁÏËÚ ˜ÂÁ ÍÓËÚÓ ÔÛ¯ÂÌÂÚÓ ·Î‡„ÓÔËflÚÒÚ‚‡ ‡Á‚ËÚËÂÚÓ Ì‡ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡. ëΉӂ‡ÚÂÎÌÓ Ë̉˂ˉۇÎÌËÚ ‡Á΢Ëfl ‚˙‚ ‚˙ÁÔ‡ÎËÚÂÎÌËfl ÓÚ„Ó‚Ó, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÔÓÎËÏÓÙËÁχ ̇ „ÂÌËÚ ÍÓ‰Ë‡˘Ë ‡Á΢ÌË ˆËÚÓÍËÌË, ·Ëı‡ ÔÓ‚ÎËflÎË ÚÂÊÂÒÚÚ‡ ̇ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡ ÔË ‡Á΢ÌËÚ ÔÛ¯‡˜Ë. èË Â‰ÌÓ ÔÓÛ˜‚‡Ì  ËÁÒΉ‚‡Ì‡ ‚ÎËflÌËÂÚÓ Ì‡ ÔÓÎËÏÓÙËÁÏËÚ ̇ 4 “ËÌÙ·χÚÓÌË” „ÂÌË ‚˙ıÛ IMT ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl /Ïfl͇ Á‡ ‡Ì̇ ‡ÚÂÓÒÍÎÂÓÁ‡/ ÔË ÔÛ¯‡˜Ë. àÁÒΉ‚‡ÌË Ò‡ 6 ÔÓÎËÏÓÙËÁχ ̇ 4 „ÂÌË: IL-6174, IL-6-572, IL-1-b-31, IL-1 receptor antagonist VNTR, Ë CD14-159. ÉÂÌÓÚËÔÓ‚ÂÚ ‡ÒÓˆËË‡ÌË Ò Ôӂ˯ÂÌË ÌË‚‡ ̇ Il-6 /ËÌÚÂ΂ÍËÌ 6/ Ò‡ “ËÌÙ·χÚÓÌË”. äÓÎÍÓÚÓ Ôӂ˜ “ËÌÙ·χÚÓÌË” ‡Î· Ëχ ‰ËÌ ÔÛ¯‡˜, ÚÓÎÍÓ‚‡ ÔÓ-‚ËÒÓÍË Ò‡ ÒÂÛÏÌËÚ ÌË‚‡ ̇ IL-6 Ë ÔÓ-„ÓÎflχ ‚Â΢Ë̇ڇ ̇ IMT (21). èË Ï·‰Ë ÔÛ¯‡˜Ë, ÔÓÎËÏÓÙËÁÏ˙Ú Ì‡ APOE Ò˙˘Ó ÔÓ‚ÎËfl‚‡ ËÒ͇. çÓÒËÚÂÎÒÚ‚ÓÚÓ Ì‡ e4 ‡Î· ‰ÂÈÒÚ‚‡ ÒËÌÂ„˘ÌÓ Ò ÔÛ¯ÂÌÂÚÓ Ë Û‚Â΢‡‚‡ ËÒ͇ ÓÚ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ (22). ÑÛ„ ÏÓ‰ÛÎË‡˘ ÂÙÂÍÚ‡ ̇ ÔÛ¯ÂÌÂÚÓ „ÂÌÓÚËÔ Â ÔÓÎËÏÓÙËÁÏ˙Ú Ì‡ „Â̇ ÍÓ‰Ë‡˘ ẨÓÚÂÎ̇ڇ ‡ÁÓÚÂÌ ÓÍÒˉ ÒËÌÚÂÚ‡Á‡ (eNOS) T786C /Ú. Á‡Ïfl̇ ̇ ÚËÏËÌÓ‚ ÌÛÍÎÂÓÚˉ Ò ˆËÚÓÁËÌÓ‚ ̇ ÔÓÁˈËfl 786/. èÛ¯‡˜ËÚ ıÓÏÓÁË„ÓÚË ÔÓ ÚÓÁË ‡ÎÂÎ ÔÓ͇Á‚‡Ú Á̇˜ËÚÂÎÌÓ Ì‡Ï‡ÎÂÌ ÏÓÁ˙˜ÂÌ Í˙‚ÓÚÓÍ Ë Ôӂ˯Â̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡ ÂÁËÒÚÂÌÚÌÓÒÚ ‚ Ò‡‚ÌÂÌËÂ Ò ÔÛ¯‡˜ËÚ ıÂÚÂÓÁË„ÓÚË ËÎË ıÓÏÓÁË„ÓÚË ÔÓ ÌÓχÎÌËfl ‡ÎÂÎ (24). Ç Á‡Íβ˜ÂÌË ÏÓÊÂÏ ‰‡ ÔÓ‰˜ÂÚ‡ÂÏ, ˜Â „ÂÌÂÚË͇ڇ ̇ ÏÛÎÚËÙ‡ÍÚÓÌËÚ Á‡·ÓÎfl‚‡ÌËfl ÓÒÚ‡‚‡ ÔÓ·ÎÂχÚ˘̇. Å·„Ó‰‡ÂÌË ̇ ̇Ô‰˙͇ ̇ ̇Û͇ڇ Ó·‡˜Â ÔÓÒÚÂÔÂÌÌÓ ˘Â Ò ËÁflÒÌË Ì‡Ô˙ÎÌÓ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ‰ˈ‡ ÒӈˇÎÌÓÁ̇˜ËÏË Á‡·ÓÎfl‚‡ÌËfl, ‚ÍÎ. ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ. ìÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë „ÂÌË Ë ÚÂıÌËÚ ÔÓ‰ÛÍÚË, ÚflıÌÓÚÓ ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂ Ò Ù‡ÍÚÓËÚ ̇ Ò‰‡Ú‡, ˘Â ‰‡‰‡Ú Ú·Ò˙Í Á‡ Ò˙Á‰‡‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë ÚÂ‡Ô‚Ú˘ÌË ÒÚ‡Ú„ËË ÔË ËÌÒÛÎÚËÚÂ. ãàíÖêÄíìêÄ 1. Bevan S, Markus H., The Genetics of Stroke, ACNR, volume 4, number 4, september/october 2004. 2. Hassan A, Markus H, Genetics and Ischaemic Stroke, Brain 2000,123,17841812; 3. Markus H Genes for stroke J Neurol Neurosurg Psychiatry 2004; 75:12291231 4. Bogousslavsky J., Caplan L., Uncommon Causes of Stroke, Cambridge University Press 2001, pages 283-284 5. Dunne P., Cloud G., Hassan A., Markus H. Evaluating the Genetic Component of Ischemic Stroke Subtypes, A Family History Study, Stroke 2003; 34:1364-1369

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6. Polychronopoulos P, Gioldasis G., Ellul J et al., Family History of Stroke in Stroke Types and Subtypes, J Neurol Sci 2002; 195:117-22 7. Martiskainen M., Pohjasvaara T., Mikkelsson J., Mantyla R., et al., Fibrinogen Gene Promoter –455A Allele as a Risk Factor for Lacunar Stroke, Stroke 2003, Apr; 34(4):886-91 8. Endler G, Funk M, Haering D, Lalouschek W, Lang W et al, Is the Factor XIII 34Val/Leu Polymorphism a Protective Factor for Cerebrovascular Disease?, Br J Haematol. 2003 Jan; 120(2):310-4 9. Jannes J, Hamilton-Bruce M, Pilotto Louis, Smith B et al., Tissue Plasminogen Activator 735C/T Enhancer Polymorphism Is a Risk Factor for Lacunar Stroke, Stroke 2004; 35:1090 10. Szolnoki Z, Somogyvari F, Kondacs A, Szabo M, Bene J, Havasi V, Komlosi K, Melegh B. Increased prevalence of platelet glycoprotein IIb/IIIa PLA2 allele in ischaemic stroke associated with large vessel pathology., Thromb res. 2003 Mar 15; 109 (5-6):265-9 11. Slowick A, Dziedzic T, Pera J, Turaj W et al., A2 Allel of GpIIIa Gene Is a Risk Factor for Stroke Caused by Large Vessel Disease in Males 12. Baker RI, Eikelboom J, Lofthouse E, Staples N, Afshar-Kharghan V, Lopez JA, Shen Y, Berndt MC, Hankey G. Platelet glycoprotein Ibalpha Kozak polymorphism is associated with an increased risk of ischemic stroke, Blood. 2001 Jul 1;98(1):36-40. 13. Fakhredin A. Sayed-Tabatabaei, MSc, MD; Jeanine J. Houwing-Duistermaat, PhD; Cornelia M. van Duijn, PhD; Jacqueline C.M. Witteman, PhD; AngiotensinConverting Enzyme Gene Polymorphism and Carotid Artery Wall Thickness, A Meta-Analysis; Stroke. 2003;34:1634. 14. A Hassan, A Lansbury, A J Catto, A Guthrie, J Spencer, C Craven, P J Grant and J M Bamford ; Angiotensin converting enzyme insertion/deletion genotype is associated with leukoaraiosis in lacunar syndromes Journal of Neurology Neurosurgery and Psychiatry 2002;72:343-346 15. Helena Schmidt MD, MSc; Yurii S. Aulchenko PhD; Natascha Schweighofer MSc; Reinhold Schmidt MD; Sasa Frank PhD; Gerhard M. Kostner PhD; Erwin Ott MD; and Cornelia van Duijn MD; Angiotensinogen Promoter B-Haplotype Associated With Cerebral Small Vessel Disease Enhances Basal Transcriptional Activity; Stroke 2004, doi:10.1161/01.STR.0000144646.96121.d2 16. A Hassan, B. Hunt, M. O Sullivan, R. Bell. R. D Souza, S. Jeffery, J. Bamford, H. Markus, Homocystein Is a Risk Factor for Cerebral Small Vessel Disease, Acting via Endothelial Dysfunction; Brain (2004), 127, 212-219; 17. A Hassan, K. Gormley, M. O Sullivan, J. Knight, P. Sham, P. Vallance, J. Bamford, H. Markus; Endothelial Nitric Oxide Gene Haplotypes and Risk of Cerebral Small Vessel Disease, Stroke 2004; 35:654 18. RB Singh, SA Mengi, Y-J Xu, AS Arneja, NS Dhalla; Pathogenesis of Atherosclerosis – a Multifactorial Process; Spring 2002, Vol 7 Number 1:40-53, Experimental&Clinical Cardiology 19. A. Helgadottir, A. Manolescu, G. Thorleifsson, S. Gretarsdottir, H. Jonsdottir, U. Thorsteinsdottir et al., The Gene Encoding 5-Lipoxygenase Activating Protein Confers Risk of Myocardial Infarction and Stroke; Nature Genetics Volume 36, Number 3, March 2004 20. S. Gretarsdottir, G. Thorleifsson, S. Th. Reynisdottir, A. Manolescu, Sif Jonsdottir, T. Jonsdottir et al., The Gene Encoding Phosphodiesterase 4D Confers Risk of Ischemic Stroke; Nature Genetics Volume 35, Number 2, October 2003 21. P. Jerrard-Dunne, M. Sitzer, P. Risley, A. Buehler, S. von Kegler, H. Markus; Inflammatory Gene Load Is associated with Enhanced Inflammation and Early Carotid Atherosclerosis in Smokers; Stroke 2004, doi:10.1161/01.STR.0000144681.46696.b3 22. Alessandro Pezzini, MD; Mario Grassi, PhD; Elisabetta Del Zotto, MD; Elena Bazzoli, MD; Silvana Archetti, PhD; Deodato Assanelli, MD; Nabil Maalikjy Akkawi, MD; Alberto Albertini, MD; Alessandro Padovani, MD, PhD; Synergistic Effect of Apolipoprotein E Polymorphisms and Cigarette Smoking on Risk of Ischemic Stroke in Young Adults; Stroke. 2004;35:438 23. Andrea Flex, MD; Eleonora Gaetani, MD; Pierangelo Papaleo, MD; Giuseppe Straface, MD; Anna S. Proia, MD; Giovanni Pecorini, MD; Paolo Tondi, MD; Paolo Pola, MD; Roberto Pola, MD PhD; Proinflammatory Genetic Profiles in Subjects With History of Ischemic Stroke; Stroke. 2004;35:2270 24. Nasreen S, Nabika T, Shibata H, Moriyama H, Yamashita K, Masuda J, Kobayashi S.; T-786C polymorphism in endothelial NO synthase gene affects cerebral circulation in smokers: possible gene-environmental interaction.; Arterioscler Thromb Vasc Biol. 2002 Apr 1;22(4):605-10.

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ÔÓ ÏÓÎÂÍÛÎ̇ Ô‡ÚÓÎÓ„Ëfl, ëÅÄãÄÉ”å‡È˜ËÌ ‰ÓÏ” Ë ·ËÓıËÏËfl”, è‰ÍÎËÌ˘ÂÌ ÛÌË‚ÂÒËÚÂÚÒÍË ˆÂÌÚ˙, åì-ëÓÙËfl 3äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, ìåÅÄã”ÄÎÂÍ҇̉Ó‚Ò͇” 4ëÅÄãÑÅ SUMMARY Ӣ  Ò··Ó ÔÓÛ˜ÂÌÓ Á‡·ÓÎfl‚‡ÌÂ. èÂÁ 1981„. ï‡‰ËÌ„ (15), ̇ ·‡Á‡Ú‡ ̇ ‰ÂÚ‡ÈÎÌË ÍÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl, Ô‡‚Ë NEW ASPECTS IN HEREDITARY SPASTIC PARAPLEÔ˙‚‡Ú‡ Í·ÒËÙË͇ˆËfl ̇ çëè Ë Ô‰·„‡ ÍËÚÂËË Á‡ GIA MOLECULAR MECHANISMS ‡Á„‡Ì˘‡‚‡ÌÂÚÓ Ì‡ ‰‚ÂÚ ÓÒÌÓ‚ÌË ÍÎËÌ˘ÌË ÙÓÏË N. Ivanova, A. Yordanova, W. Mitev, A. Andreeva “˜ËÒÚ‡” Ë “ÍÓÏÔÎÂÍÒ̇”. íÂÁË ÍËÚÂËË Ò ËÁÔÓÎÁ‚‡Ú Ë I. Tarnev, I. Litvinenko, I. Kremenski Hereditary spastic paraplegia (HSP) is one of the most ‰Ó ‰ÌÂÒ Ë Ò‡ Á‡Î„̇ÎË ‚ Ò˙‚ÂÏÂÌÌËÚ ÔÂÔÓ˙˜ËÚÂÎÌË quickly developed trends of modern neurogenetics and molec- ‰Ë‡„ÌÓÒÚ˘ÌË ÔÓÚÓÍÓÎË Á‡ çëè (21). 燂ÎËÁ‡ÌÂÚÓ Ì‡ ÏÓÎÂÍÛÎfl̇ڇ ·ËÓÎÓ„Ëfl ‚ ÔÓÛ˜‚‡ÌËular pathology. During the last 20 years, the application of molecular biology methods in neuromuscular disorders stud- flÚ‡ ̇ Ì‚ÓÏÛÒÍÛÎÌËÚ Á‡·ÓÎfl‚‡ÌËfl ÔÂÁ ÔÓÒΉÌËÚ 20 ies revealed part of the molecular mechanisms underlining „Ó‰ËÌË ‰Ó‚‰ ‰Ó ËÁflÒÌfl‚‡Ì ̇ „ÓÎflχ ˜‡ÒÚ ÓÚ „ÂÌÂÚ˘HSP pathology. The aim of this review is to summarize the ÌËÚ Ô˘ËÌË Ë ÏÓÎÂÍÛÎÌËÚ ÏÂı‡ÌËÁÏË ‚Ó‰Â˘Ë ‰Ó ‡Árecent advances in HSP according to the molecular biology ‚ËÚËÂÚÓ Ì‡ çëè. ä‡Í‚Ó Á̇ÂÏ ‰ÌÂÒ Á‡ ÚÓ‚‡ ÓÚ‰‡‚̇ ÓÔËÒ‡ÌÓ Á‡·ÓÎfl‚‡ÌÂ? point of view. Key words: hereditary spastic paraplegia, molecular ÑÖîàçàñàü mechanisms. ë˙‚ÂÏÂÌ̇ڇ Ì‚Ó„ÂÌÂÚË͇ Ë Ì‚ÓÔ‡ÚÓÎÓ„Ëfl ÓÔÂêÖáûåÖ ‰ÂÎflÚ Ì‡ÒΉÒÚ‚ÂÌËÚ ÒÔ‡ÒÚ˘ÌË Ô‡‡Ô΄ËË Í‡ÚÓ ÏÓç‡ÒΉÒÚ‚Â̇ڇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl (çëè)  ‰ÌÓ ÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl, ÔË ÍÓËÚÓ ‡ÍÒÓÌËÚ ̇ ÍÓÚËÍÓÒÓÚ Ì‡È-·˙ÁÓ ‡Á‚Ë‚‡˘ËÚ Ò ̇Ô‡‚ÎÂÌËfl ̇ Ò˙‚ÂÏÂÌ- ÔË̇ÎÌËfl Ú‡ÍÚ Ì Ò ‡Á‚Ë‚‡Ú ÌÓχÎÌÓ ËÎË ÔÂÚ˙Ôfl̇ڇ Ì‚Ó„ÂÌÂÚË͇ Ë ÏÓÎÂÍÛÎ̇ Ô‡ÚÓÎÓ„Ëfl. 燂ÎËÁ‡ÌÂ- ‚‡Ú ÔÓ„ÂÒË‡˘‡ ‰Â„ÂÌÂ‡ˆËfl ÒΉ Ô˙‚Ó̇˜‡ÎÌÓ ÌÓÚÓ Ì‡ ÏÓÎÂÍÛÎfl̇ڇ ·ËÓÎÓ„Ëfl ‚ ÔÓÛ˜‚‡ÌËflÚ‡ ̇ Ì‚Ó- χÎÌÓ ‡Á‚ËÚË (27). ÏÛÒÍÛÎÌËÚ Á‡·ÓÎfl‚‡ÌËfl ÔÂÁ ÔÓÒΉÌËÚ 20 „Ó‰ËÌË ‰Ó‚ÂÖèàÑÖåàéãéÉàü ‰Â ‰Ó ËÁflÒÌfl‚‡Ì ̇ „ÓÎflχ ˜‡ÒÚ ÓÚ „ÂÌÂÚ˘ÌËÚ Ô˘ËÌË Ë Ô‡ÚÓÎӄ˘ÌËÚ ÏÂı‡ÌËÁÏË ‚Ó‰Â˘Ë ‰Ó ‡Á‚ËÚËÂÚÓ óÂÒÚÓÚ‡Ú‡ ̇ Á‡·Ó΂‡ÂÏÓÒÚ Á‡ ‚Ò˘ÍË ÙÓÏË Ì‡ ̇ ÚÓ‚‡ Á‡·ÓÎfl‚‡ÌÂ. ñÂÎÚ‡ ̇ ̇ÒÚÓfl˘Ëfl Ó·ÁÓ  ‰‡ çëè ‚‡Ë‡ ÔË ‡Á΢ÌËÚ ÔÓÛ˜‚‡ÌËfl ÓÚ 1.5 ‰Ó 2.7 Ó˜Âڇ Ò˙‚ÂÏÂÌÌÓÚÓ ÌË‚Ó Ì‡ ÔÓÁ̇ÌË ‚ Ú‡ÁË Ó·Î‡ÒÚ /100 000 (12). ç‡È-˜ÂÒÚËÚ ÙÓÏË Ì‡ çëè ‚ ‚ÓÔÂÈÒÍËÓÚ „Ή̇ ÚӘ͇ ̇ ÏÓÎÂÍÛÎfl̇ڇ ·ËÓÎÓ„Ëfl. Ú ÒÚ‡ÌË Ò‡ ÚÂÁË Ò ÄÑ ÏÓ‰ÂΠ̇ Û̇ÒΉfl‚‡ÌÂ Ë ÍÎËÌ˘äβ˜Ó‚Ë ‰ÛÏË: ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ̇ ͇ÚË̇ Ò˙ÓÚ‚ÂÚÒÚ‚‡˘‡ ̇ “˜ËÒÚ‡” ÙÓχ ̇ Á‡·ÓÏÓÎÂÍÛÎÌË ÏÂı‡ÌËÁÏË. Îfl‚‡ÌÂÚÓ. Ä‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌËflÚ ÏÓ‰ÂΠ̇ Û̇ÒΉfl‚‡Ì ‚ Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë Ò ‡ÒÓˆËË‡ Ò “ÍÓÏÔÎÂÍÒ̇” ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ç‡ÒΉÒÚ‚Â̇ڇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl (çëè)  ‰ÌÓ ÓÚ Ì‡È-·˙ÁÓ ‡Á‚Ë‚‡˘ËÚ Ò ̇Ô‡‚ÎÂÌËfl ̇ Ò˙‚ÂÏÂÌäãÄëàîàäÄñàü ̇ڇ Ì‚Ó„ÂÌÂÚË͇ Ë ÏÓÎÂÍÛÎ̇ Ô‡ÚÓÎÓ„Ëfl. íÂÏËÌ˙Ú ë˙‚ÂÏÂÌ̇ڇ Í·ÒËÙË͇ˆËflÚ‡ ̇ çëè ‚Íβ˜‚‡ ÓÚ‚Íβ˜‚‡ ÍÎËÌ˘ÌÓ Ë „ÂÌÂÚ˘ÌÓ ıÂÚÂÓ„ÂÌ̇ „ÛÔ‡ ÓÚ Ì‡ÒflÌÂÚÓ Ì‡ ÍÓÌÍÂÚ̇ڇ ÍÎËÌ˘̇ ÙÓχ Í˙Ï ÓÔ‰Â̇ÒΉÒÚ‚ÂÌË ‰Â„ÂÌÂ‡ÚË‚ÌË Á‡·ÓÎfl‚‡ÌËfl ̇ ñçë Ò Ó·˘‡ ÎÂÌ „ÂÌÂÚ˘ÂÌ ÎÓÍÛÒ. ÑÓ ÚÓÁË ÏÓÏÂÌÚ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË 23 ÍÎËÌ˘̇ ı‡‡ÍÚÂËÒÚË͇ ÔÓ„ÂÒË‡˘‡ ÒÔ‡ÒÚ˘ÌÓÒÚ Ë „ÂÌÂÚ˘ÌË ÎÓÍÛÒ‡ ÓÚ„Ó‚ÓÌË Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ çëè ÙÂÔÓ-ÎÂÍÓ ËÁ‡ÁÂ̇ ËÎË ÎËÔÒ‚‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‚ ‰ÓÎÌË- ÌÓÚËÔ, ÓÁ̇˜ÂÌË Í‡ÚÓ SPG ÎÓÍÛÒË Ú Í‡ÈÌËˆË (21). Ç Á‡‚ËÒËÏÓÒÚ ÓÚ ÚÓ‚‡ ‰‡ÎË ÒÔ‡ÒÚ˘- (www.ncbi.nlm.nih.gov/OMIM, 퇷Î.1). Ç 11 ÓÚ Úflı Ò‡ ̇ڇ Ô‡‡Ô‡ÂÁ‡ Ô‰ÒÚ‡‚Îfl‚‡ ËÁÓÎË‡Ì ÒËÏÔÚÓÏ ËÎË Â Ë‰ÂÌÚËÙˈË‡ÌË „ÂÌËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ ÍÎËÌ˘̇ڇ ËÁfl‚‡ ‡ÒÓˆË‡Ì‡ Ò ‰ÓÔ˙ÎÌËÚÂÎ̇ Ì‚ÓÎӄ˘̇ ËÎË ÂÍÒÚ‡Ì‚- ̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl. ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇, çëè Ò ÓÔ‰ÂÎfl ͇ÚÓ “˜ËÒÚ‡” ËÎË “ÍÓÏÔÎÂÍÒ̇” ÙÓχ. Ç „ÂÌÂÚ˘ÂÌ ‡ÒÔÂÍÚ Ò‡ äãàçàóçÄ äÄêíàçÄ Ô‰ÒÚ‡‚ÂÌË ‚Ò˘ÍË ÏÓ‰ÂÎË Ì‡ Û̇ÒΉfl‚‡Ì – ‡‚ÚÓÁÓÏéÒÌӂ̇ڇ ÒËÏÔÚÓχÚË͇ ÔË Ô‡ˆËÂÌÚË Ò “˜ËÒÚ‡” ÌÓ-‰ÓÏË̇ÌÚÂÌ (ÄÑçëè), ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÙÓχ ̇ çëè ‚Íβ˜‚‡: ̇Û¯ÂÌËfl ‚ ÔÓıӉ͇ڇ, ÒÔ‡ÒÚ˘(Äêçëè) Ë ï-Ò‚˙Á‡Ì. ᇷÓÎfl‚‡ÌÂÚÓ Â ÓÔËÒ‡ÌÓ Á‡ Ô˙‚ Ô˙Ú ÓÚ Seeligmuller ÌÓÒÚ Ì‡ ‰ÓÎÌË Í‡ÈÌˈË, ıËÔÂÂÙÎÂÍÒËfl, ̇Û¯ÂÌ ÓÚ„ÓÔÂÁ 1876„. (31), ÌÓ ÒÚ‡‚‡ ËÁ‚ÂÒÚÌÓ Í‡ÚÓ ÅÓÎÂÒÚ Ì‡ ‚Ó ̇ ÂÍÒÚÂÌÁÓ Ô·ÌÚ‡̇ڇ „ÛÔ‡ ÏÛÒÍÛÎË Ë Ò··ÓÒÚ ‚ Strumpell – Lorrain, ‚ ˜ÂÒÚ Ì‡ ‰‚‡Ï‡Ú‡ Ì‚ÓÎÓÁË, ÍÓËÚÓ ÔË‡ÏˉÌËÚ Ô˙Úˢ‡ ̇ ‰ÓÎÌËÚ Í‡ÈÌˈË. èË ÍÓÏÔÎÂÌflÍÓÎÍÓ „Ó‰ËÌË ÔÓ-Í˙ÒÌÓ ÔÛ·ÎËÍÛ‚‡Ú ÌÓ‚Ë ÒÎÛ˜‡Ë ̇ çëè ÍÒÌËÚ ÙÓÏË ÒÔ‡ÒÚ˘̇ڇ Ô‡‡Ô‡ÂÁ‡  ‡ÒÓˆË‡Ì‡ Ò ‡ÏËË ‰‡‚‡Ú Ô˙‚ËÚ ÚÓ˜ÌË ÍÎËÌ˘ÌË ı‡‡ÍÚÂËÒÚËÍË Ì‡ ÓÚÓÙËfl, Ò˙‰Â˜ÌË ‰ÂÙÂÍÚË, ˆÂ·Â·̇ ËÎË ÂÍÒÚ‡ÔË‡Ïˉ̇ ÒËÏÔÚÓχÚË͇, „ÎÛıÓÚ‡, ‰ÂÏÂ̈Ëfl, ÂÔËÎÂÔÒËfl, Ëı“˜ËÒÚ‡” ÙÓχ ̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl (19,33,34). Ç˙ÔÂÍË Ì‡ÚÛÔ‡ÌËÚ ‰‡ÌÌË, ‚ÂÍ ÔÓ-Í˙ÒÌÓ çëè ‚Ò ÚËÓÁ‡, ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl, ÂÚËÌÓÔ‡ÚËfl Ë ‰. 2ä‡Ú‰‡”ïËÏËfl


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

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퇷Î.1. ÉÂÌÂÚ˘ÌË ÎÓÍÛÒË Ë ÙÓÏË Ì‡ çëè. SPG ÎÓÍÛÒ SPG3Ä SPG4 SPG6 SPG8 SPG9 SPG10 SPG12 SPG13 SPG17 SPG19 SPG5Ä SPG7 SPG11 SPG14 SPG15 SPG20 SPG21 SPG23 SPG24 SPG27 SPG1 SPG2 SPG16

ïÓÏÓÁÓÏ̇ ÎÓ͇ÎËÁ‡ˆËfl ÉÂÌ/ ÅÂÎÚ˙˜ÂÌ ÔÓ‰ÛÍÚ Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌË ÙÓÏË Ì‡ çëè 14q11-q21 ÄÚ·ÒÚËÌ 2p21-p24 ëÔ‡ÒÚËÌ 15q11-q12 NIPA1 8q24 ÌÂËÁ‚ÂÒÚÌË 10q23-q24 ÌÂËÁ‚ÂÒÚÌË 12q13 KIF5A 19q13 ÌÂËÁ‚ÂÒÚÌË 2q24-q34 HSP60 11q12-q14 ëÂËÔËÌ 9q33-q34 ÌÂËÁ‚ÂÒÚÌË Ä‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌË ÙÓÏË Ì‡ çëè 8q ÌÂËÁ‚ÂÒÚÌË 16q24 è‡‡Ô΄ËÌ 15q ÌÂËÁ‚ÂÒÚÌË 3q27-q28 ÌÂËÁ‚ÂÒÚÌË 14q ÌÂËÁ‚ÂÒÚÌË 13q12 ëÔ‡ÚËÌ 15q21-q22 Äëê33/å‡ÒÔ‡‰ËÌ 1q24-q32 ÌÂËÁ‚ÂÒÚÌË 13q14 ÌÂËÁ‚ÂÒÚÌË 12p11.1-q14 ÌÂËÁ‚ÂÒÚÌË ï-Ò‚˙Á‡ÌË ÙÓÏË Ì‡ çëè ïq28 L1CAM Xq21 PLP Xq11.2 ÌÂËÁ‚ÂÒÚÌË

äÎËÌ˘̇ ÙÓχ óËÒÚ‡ óËÒÚ‡/ ÍÓÏÔÎÂÍÒ̇ óËÒÚ‡ óËÒÚ‡ äÓÏÔÎÂÍÒ̇ óËÒÚ‡ óËÒÚ‡ óËÒÚ‡ ëË̉ÓÏ Ì‡ Silver óËÒÚ‡ óËÒÚ‡ äÓÏÔÎÂÍÒ̇/ ˜ËÒÚ‡ óËÒÚ‡ äÓÏÔÎÂÍÒ̇ äÓÏÔÎÂÍÒ̇ ëË̉ÓÏ Ì‡ Troyer å‡ÒÚ ÒË̉ÓÏ ëË̉ÓÏ Ì‡ Lison óËÒÚ‡ äÓÏÔÎÂÍÒ̇ äÓÏÔÎÂÍÒ̇ äÓÏÔÎÂÍÒ̇/ ˜ËÒÚ‡ äÓÏÔÎÂÍÒ̇

çÖÇêéèÄíéãéÉàü éÒÌӂ̇ڇ Ì‚ÓÔ‡ÚÓÎӄ˘̇ ̇ıӉ͇ ÔË “˜ËÒÚ‡” ÙÓχ ̇ çëè  ‡ÍÒÓ̇Î̇ڇ ‰Â„ÂÌÂ‡ˆËfl, ÍÓflÚÓ Á‡ÔÓ˜‚‡ ‰ËÒÚ‡ÎÌÓ Ë Ì‡Ô‰‚‡ ‚ ÔÓÒÓ͇ Í˙Ï ÚflÎÓÚÓ Ì‡ Ì‚Ó̇, Ú.̇. “dying back” ‡ÍÒÓÌÓÔ‡ÚËfl (1, 30).

Í˙Ò̇ ‚˙Á‡ÒÚ, ÓÚ Ò˙Ô˙ÚÒÚ‚‡˘Ë Á‡·ÓÎfl‚‡ÌËfl. ëÚÂÔÂÌÚ‡ ̇ Á‡Òfl„‡Ì ‚‡Ë‡ ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚ هÏËÎËË Ë ‚ ÔÓχÎ͇ ÒÚÂÔÂÌ ‚˙Ú ‚˙‚ Ù‡ÏËÎËËÚÂ. éÍÓÎÓ 30% ÓÚ Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Ì ‡Á‚Ë‚‡Ú ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇ (12).

ÑàÄÉçéëíàäÄ èÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡ “ç‡ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl” Ò ÓÒÌÓ‚‡‚‡ ͇ÍÚÓ Ì‡ ÍÎËÌ˘ÌË ÍËÚÂËË, ڇ͇ Ë Ì‡ ‰‡ÌÌËÚ ÓÚ Ù‡ÏËÎ̇ڇ ËÒÚÓËfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. éÔ‰ÂÎflÌÂÚÓ Ì‡ „ÂÌÂÚ˘̇ڇ ÙÓχ ̇ çëè ËÁËÒÍ‚‡ Ô‰‚‡ËÚÂÎÂ̇ χÍÒËχÎÌÓ ÔˆËÁË‡Ì‡ ÍÎËÌ˘̇ ‰Ë‡„ÌÓÁ‡ Ë ÔÓ‰Ó·ÂÌ „Â̇Îӄ˘ÂÌ ‡Ì‡ÎËÁ. ÉÂÌÂÚ˘ÌË ÚÂÒÚÓ‚Â, ÔË·„‡ÌË ‚ ÍÎËÌ˘̇ڇ ‰Ë‡„ÌÓÒÚ˘̇ Ô‡ÍÚË͇ Ò‡ ‡Á‡·ÓÚÂÌË Á‡ ‰ÂÚÂ͈Ëfl ̇ ÏÛÚ‡ˆËË ‚ „ÂÌËÚ Á‡ SPG1, SPG2, SPG3A, SPG4, SPG6, SPG7, SPG13, SPG20, SPG21. åÛÚ‡ˆËÓÌÂÌ ÒÍËÌËÌ„ ̇ „ÂÌËÚ Á‡ SPG10 Ë SPG21 Á‡ Ò„‡ Ò ËÁ‚˙¯‚‡ Ò‡ÏÓ ‚ ËÁÒΉӂ‡ÚÂÎÒÍË Î‡·Ó‡ÚÓËË.

ÉÖçÖíàóçé äéçëìãíàêÄçÖ ÉÂÌÂÚ˘ÌÓÚÓ ÍÓÌÒÛÎÚË‡Ì  ‚˙ÁÏÓÊÌÓ Ò‡ÏÓ ÔË Ù‡ÏËÎËËÚÂ, ‚ ÍÓËÚÓ „ÂÌÂÚ˘ÌËfl ‰ÂÙÂÍÚ Â ‚ ËÁ‚ÂÒÚÂÌ „ÂÌ, ‰Ó͇Á‡Ì‡  ÏÛÚ‡ˆËfl ÔË ÒË„ÛÌÓ ·ÓÎÂÌ ˜ÎÂÌ ÓÚ ÒÂÏÂÈÒÚ‚ÓÚÓ Ë Ëχ ‚˙ÁÏÓÊÌÓÒÚ Á‡ ËÁÒΉ‚‡Ì ̇ ‡Á¯ËÂÌÓ Ó‰ÓÒÎÓ‚ËÂ. èË ÔÓ-„ÓÎflχ ˜‡ÒÚ ÓÚ Ë̉˂ˉËÚÂ Ò ÄÑçëè  ̇Îˈ هÏËÎ̇ ËÒÚÓËfl, ÌÓ Ò Ò¢‡Ú Ë ÒÎÛ˜‡Ë ̇ de novo ÏÛÚ‡ˆËË. èË Ì‡Î˘Ë ̇ Á‡ÒÂ„Ì‡Ú Ó‰ËÚÂÎ, ËÒ͇ Á‡ ÒË·ÎËÌ„ËÚ ̇ ÔÓ·‡Ì‰‡ ‰‡ ÌÓÒflÚ ÏÛÚ‡ÌÚÌËfl ‡ÎÂÎ Â 50%. êËÒÍ˙Ú Á‡ Ô‰‡‚‡Ì ̇ ÏÛÚ‡ˆËflÚ‡ ‚ ÔÓÍÓÎÂÌËÂÚÓ, ÔË ËÌ‰Ë‚Ë‰Ë Ò ÄÑçëè  50% Á‡ ‚Òfl͇ ÓÚ‰ÂÎ̇ ·ÂÏÂÌÌÓÒÚ. èÂÌÂÚ‡ÌÚÌÓÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÓÍÓÎÓ 70% (7). óÂÒÚÓÚ‡Ú‡ ̇ de novo ÏÛÚ‡ˆËËÚ  ÌÂËÁ‚ÂÒÚ̇ (11). Ç ÒÎÛ˜‡ËÚ ̇ Äêçëè, Ó‰ËÚÂÎËÚ ̇ Ë̉ÂÍÒÌËfl Ô‡ˆËÂÌÚ Ò‡ Ó·ÎË„‡ÚÌË ıÂÚÂÓÁË„ÓÚË Ë ÌÓÒflÚ ÔÓ Â‰ËÌ ÏÛÚ‡ÌÚÂÌ ‡ÎÂÎ. ïÂÚÂÓÁË„ÓÚËÚ (ÌÓÒËÚÂÎËÚÂ) Ò‡ ‡ÒËÏÔÚÓχÚ˘ÌË. êËÒÍ˙Ú Á‡ ÒË·ÎËÌ„ËÚ ̇ Ë̉ÂÍÒÌËfl Ô‡ˆËÂÌÚ ‰‡ Ò‡ Á‡Ò„̇ÚË Â 25%, ‰‡ Ò‡ ÌÂÁ‡Ò„̇ÚË ÍÎËÌ˘ÌÓ ÌÓÒËÚÂÎË Â 50% Ë 25%  ¯‡ÌÒ˙Ú ‰‡ ·˙‰‡Ú Á‰‡‚Ë Ë ‰‡ Ì ÌÓÒflÚ ÏÛÚ‡ÌÚÌËfl ‡ÎÂÎ. èÓÍÓÎÂÌËflÚ‡ ̇ ÔÓ·‡Ì‰‡ Ò‡ Ó·ÎË„‡ÚÌË ıÂÚÂÓÁË„ÓÚË. èË ï-Ò‚˙Á‡ÌËÚ ˆÂÒË‚ÌË ÙÓÏË ‚Ò˘ÍË ÊÂÌË, ÍÓËÚÓ ËÏ‡Ú Á‡ÒÂ„Ì‡Ú ÒËÌ Ë ‰Û„ Á‡ÒÂ„Ì‡Ú Ó‰ÌË̇ ÓÚ Ï˙ÊÍË ÔÓÎ, Ò‡ Ó·ÎË„‡ÚÌË ıÂÚÂÓÁË„ÓÚË. Ç ÒÎÛ˜‡È, ˜Â Á‡Ò„̇ÚËfl Ë̉˂ˉ ÓÚ Ï˙ÊÍË ÔÓÎ Ô‰ÒÚ‡‚Îfl‚‡ ÒÔÓ‡‰Ë˜ÂÌ ÒÎÛ˜‡È ̇ ï-Ò‚˙Á‡Ì‡ ÙÓχ ̇ çëè, Ò˙˘ÂÒÚ‚Û‚‡Ú Ìfl-

ÑàîÖêÖçñàÄãçÄ ÑàÄÉçéáÄ Ñˇ„ÌÓÒÚË͇ڇ ̇ çëè ‚ „ÓÎflχ ÒÚÂÔÂÌ Ò ÓÒÌÓ‚‡‚‡ ̇ ËÁÍβ˜‚‡Ì ̇ ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl, ˜ËÂÚÓ ÍÎËÌ˘ÌÓ ÔÓÚ˘‡Ì ̇ÔÓ‰Ó·fl‚‡ ÚÓ‚‡ ̇ ÒÔ‡ÒÚ˘̇ڇ Ô‡‡Ô΄Ëfl. í‡ÍË‚‡ Ò‡ ÒÚÛÍÚÛÌË ‡ÌÓχÎËË Ì‡ ñçë, Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌË Á‡·ÓÎfl‚‡ÌËfl Ò ÙÂÌÓÚËÔ ·ÎËÁ˙Í ‰Ó ÚÓÁË Ì‡ ÍÓÏÔÎÂÍÒÌËÚ ÙÓÏË Ì‡ çëè, ͇ÍÚÓ Ë ÌflÍÓÈ ÏÂÚ‡·ÓÎËÚÌË Á‡·ÓÎfl‚‡ÌËfl, Á‡ ÍÓËÚÓ Ëχ ΘÂÌËÂ. èêéÉçéáÄ çëè  Á‡·ÓÎfl‚‡ÌÂ, Ò˙‚ÏÂÒÚËÏÓ Ò ÌÓχÎ̇ڇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ ÊË‚ÓÚ‡. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË ÛÏË‡Ú ‚


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÍÓÎÍÓ ‚˙ÁÏÓÊÌÓÒÚË Á‡ ÌÓÒËÚÂÎÒÍËfl ÒÚ‡ÚÛÒ Ì‡ Ì„ӂ‡Ú‡ χÈ͇ – Ô‡ˆËÂÌÚ‡ Ëχ de novo ÏÛÚ‡ˆËfl Ë Ì„ӂ‡Ú‡ χÈ͇ Ì  ÌÓÒËÚÂÎ, χÈ͇ڇ ËÎË ·‡·‡Ú‡ ÔÓ Ï‡È˜Ë̇ ÎËÌËfl Ëχ de novo ÏÛÚ‡ˆËfl. êËÒÍ˙Ú Á‡ ÒË·ÎËÌ„ËÚ ̇ Ô‡ˆËÂÌÚ‡ Ò ï-Ò‚˙Á‡Ì‡ ÙÓχ Á‡‚ËÒË ÓÚ ÌÓÒËÚÂÎÒÍËfl ÒÚ‡ÚÛÒ Ì‡ χÈ͇ڇ. ÄÍÓ Ï‡È͇ڇ  ÌÓÒËÚÂÎ, Ò˙˘ÂÒÚ‚Û‚‡ 50% ËÒÍ Á‡ Ô‰‡‚‡Ì ̇ ÏÛÚ‡ˆËflÚ‡ ÔË ‚Òfl͇ ·ÂÏÂÌÌÓÒÚ. ÇÒ˘ÍË ÏÓϘÂÚ‡, ÍÓËÚÓ Ò‡ Û̇ÒΉËÎË ÏÛÚ‡ˆËflÚ‡ ˘Â ·˙‰‡Ú Á‡Ò„̇ÚË, ‡ ÏÓÏ˘ÂÚ‡Ú‡ ˘Â ·˙‰‡Ú Á‰‡‚Ë ÌÓÒËÚÂÎË. ÇÒ˘ÍË ‰˙˘ÂË Ì‡ Á‡ÒÂ„Ì‡Ú Ë̉˂ˉ ÓÚ Ï˙ÊÍË ÔÓÎ ˘Â ·˙‰‡Ú ÌÓÒËÚÂÎË Ì‡ ÏÛÚ‡ÌÚÌËfl ‡ÎÂÎ, ‡ ‚Ò˘ÍË ÒËÌÓ‚Â ˘Â ·˙‰‡Ú Á‡Ò„̇ÚË. åéãÖäìãçÄ èÄíéãéÉàü åÂı‡ÌËÁÏËÚ ‚Ó‰Â˘Ë ‰Ó ËÁfl‚‡ ̇ çëè Ë Ò‚˙Á‡ÌËÚÂ Ò Úflı „ÂÌË (퇷Îˈ‡ 2), ÏÓ„‡Ú ‰‡ Ò ‡Á‰ÂÎflÚ Ì‡ 2 ÓÒÌÓ‚ÌË „ÛÔË: ‡ÍÒÓ̇Î̇ ‰Â„ÂÌÂ‡ˆËfl ‚ ÒΉÒÚ‚Ë ̇ ‰ÂÙÂÍÚË ‚ „ÂÌË Ò‚˙Á‡ÌË Ò ‡Á‚ËÚËÂÚÓ Ë ÒÚÛÍÚÛ‡Ú‡ ̇ ÍÓÚËÍÓÒÔË̇ÎËfl Ú‡ÍÚ; ‡ÍÒÓ̇Î̇ ‰Â„ÂÌÂ‡ˆËfl ‚ ÂÁÛÎÚ‡Ú Ì‡ ÙÛÌ͈ËÓ̇ÎÌË Ì‡Û¯ÂÌËfl Ò‚˙Á‡ÌË Ò ÏÛÚ‡ˆËË ‚ „ÂÌË, ÍÓËÚÓ ÍÓ‰Ë‡Ú ·ÂÎÚ˙ˆË Û˜‡ÒÚ‚‡˘Ë ‚ ÏÓÎÂÍÛÎÌËfl ÔÂÌÓÒ, Ú‡ÌÒÔÓÚ Ë ÂÌÂ„ËÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï. ä˙Ï Ô˙‚‡Ú‡ „ÛÔ‡ ÒÔ‡‰‡Ú ‰‚‡ „Â̇ ‡ÁÔÓÎÓÊÂÌË ‚˙ıÛ ï-ıÓÏÓÁÓχڇ, ÍÓ‰Ë‡˘Ë ÔÓ‚˙ıÌÓÒÚ̇ ‡‰ıÂÁËÓÌ̇ ÏÓÎÂÍÛ· L1 (L1CAM) Ë ÒÚÛÍÚÛÂÌ ÏËÂÎËÌÓ‚ ·ÂÎÚ˙Í PLP1 (ÔÓÚÂÓÎËÔˉÂÌ ÔÓÚÂËÌ 1). L1CAM  Ì‚ÓÌ-ÒÔˆËÙ˘ÂÌ, Ú‡ÌÒÏÂÏ·‡ÌÂÌ „ÎËÍÓÔÓÚÂËÌ, ωËË‡˘ ÍÎÂÚ˙˜Ì‡Ú‡ ‡‰ıÂÁËfl Ë Ì‡‡ÒÚ‚‡ÌÂÚÓ Ì‡ ÌÂ‚ÌËÚ ÓÍÓ̘‡ÌËfl. ÖÍÒÔÂÒË‡ Ò ÔÓ ÔÓ‚˙ıÌÓÒÚÚ‡ ̇ ‰˙΄ËÚ ‡ÍÒÓÌË Ë ÚÂıÌËÚ ÍÓÌÛÒË Ì‡ ̇‡ÒÚ‚‡Ì ÔÓ ‚ÂÏ ̇ ÂÏ·ËÓ̇ÎÌÓÚÓ ‡Á‚ËÚË ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ Ë ‚ ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË ‚ ÔÂËÙÂ̇ڇ ÌÂ‚̇ ÒËÒÚÂχ. L1CAM Û˜‡ÒÚ‚‡ ‚ ÒÎÓÊÌË ÏÓÎÂÍÛÎÌË ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ëfl, ÍÓÌÚÓÎË‡˘Ë ̇ÒÓ˜ÂÌËfl ‡ÍÒÓ̇ÎÂÌ ‡ÒÚÂÊ Ë Ù‡ÒˆËÍÛ·ˆËflÚ‡. äÓÏÔÓÌÂÌÚ Â Ì‡ ˆÂÔÚÓÂÌ ÍÓÏÔÎÂÍÒ, ˜ËËÚÓ ÎË„‡Ì‰  ıÂÏÓÂÔÂÎÂÌÚ̇ڇ ÏÓÎÂÍÛ· Sema3A, ÒÂÍÂÚË‡Ì‡ ÓÚ ‚ÂÌÚ‡Î̇ڇ ˜‡ÒÚ Ì‡ „˙·Ì‡˜ÌËfl ÏÓÁ˙Í. ë‚˙Á‚‡ÌÂÚÓ Ì‡ Sema3A-ˆÂÔÚÓ‡ Ò˙Ò Sema3A ËÁÚ·ÒÍ‚‡ ÍÓÚËÍÓÒÔË̇ÎÌËfl ‡ÍÒÓÌ ÍÓÌÚ‡Î‡ÚÂ‡ÎÌÓ Ë ‰ÓÁ‡ÎÌÓ ‚ ωÛ·ڇ ÔË ‰ÓÒÚË„‡Ì ̇ ÒÔËÌÓ-ωÛ·ÌËÚ ‚˙ÁÍË (4). åÛÚ‡ˆËË ‚ „Â̇ ÍÓ‰Ë‡˘ L1CAM, Ò‡ Ô˘Ë̇ Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ¯ËÓÍ ÒÔÂÍÚ˙ ÓÚ ÍÎËÌ˘ÌË ÙÂÌÓÚËÔÓ‚Â - ÍÓÏÔÎÂÍÒ̇ ÙÓχ ̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl ‡ÒÓˆË퇷Î. 2. åÂı‡ÌËÁÏË Ì‡ ‚˙ÁÌËÍ‚‡Ì ̇ ‡ÍÒÓ̇Î̇ ‰Â„ÂÌÂ‡ˆËfl ÔË çëè Ë Ò‚˙Á‡ÌËÚÂ Ò Úflı „ÂÌË Ë „ÂÌÂÚ˘ÌË ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. åÂı‡ÌËÁÏË Ì‡ ‡ÍÒÓ̇Î̇ ‰Â„ÂÌÂ‡ˆËfl ÉÂÌ SPG ÚËÔ I. ç‡Û¯ÂÌËfl ‚ ‡Á‚ËÚËÂÚÓ Ë ÒÚÛÍÚÛ‡Ú‡ ̇ ÍÓÚËÍÓÒÔË̇ÎÌËfl Ú‡ÍÚ. 1. ç‡Û¯Â̇ ÍÎÂÚ˙˜Ì‡ Ò˄̇ÎËÁ‡ˆËfl L1CAM SPG1 2. èÂÍ˙Ò̇ÚÓ ‡ÍÒÓÌ-„ΡÎÌÓÚÓ PLP1 SPG2 ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ë II. îÛÌ͈ËÓ̇ÎÌË Ì‡Û¯ÂÌËfl 1. ç‡Û¯ÂÌËfl ‚ ÏËÍÓÚÛ·ÛÎÌËfl Ú‡ÌÒÔÓÚ ‡) ̇Û¯Â̇ ÏËÍÓÚÛ·ÛÎ̇ ‰Ë̇ÏË͇ ëÔ‡ÒÚËÌ SPG4 ·) ‰ÂÙÂÍÂÌ ÍËÌÂÁËÌÓ‚ ÏÓÚÓÂÌ ÍÓÏÔÎÂÍÒ KIF5A SPG10 2. ç‡Û¯ÂÌ ‚ÂÁËÍÛ·ÌÓ-ωËË‡Ì ‡Ú·ÒÚËÌ SPG3Ä ẨÓÁÓÏÂÌ Ú‡ÙËÍ ÒÔ‡ÚËÌ SPG20 Äëê33 SPG21 3. ç‡Û¯ÂÌËfl ‚ ÂÌÂ„ËËÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï (¯‡ÔÂÓÌÓÔ‡ÚËË) ‡) ‰ÂÙÂÍÚÂÌ ¯‡ÔÂÓÌÂÌ ÍÓÏÔÎÂÍÒ çSP60 SPG13 ·) ̇Û¯Â̇ ÔÓÚÂËÌÓ‚‡ ‰Â„‡‰‡ˆËfl Ô‡‡Ô΄ËÌ SPG7 4. çÂËÁ‚ÂÒÚÂÌ Ô‡ÚÓ„ÂÌÂÚ˘ÂÌ NIPA1 SPG6 ÏÂı‡ÌËÁ˙Ï BSCL2 SPG17

126

‡Ì‡ Ò ÎËÔÒ‡ ̇ extensor pollicis longus ÏÛÒÍÛÎ, SPG1, ïÒ‚˙Á‡Ì ıˉÓˆÂÙ‡ÎÛÒ Ë MASA ÒË̉ÓÏ. èÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ ÚÂÁË ÏÛÚ‡ˆËË ‚Ó‰flÚ ‰Ó ÒËÌÚÂÁ‡Ú‡ ̇ ÒÍ˙ÒÂÌ ·ÂÎÚ˙˜ÂÌ ÔÓ‰ÛÍÚ. ç‚ÓÔ‡ÚÓÎӄ˘ÌËÚ ̇ıÓ‰ÍË ÔË Ô‡ˆËÂÌÚË Ò SPG1 ÔÓ͇Á‚‡Ú, ˜Â ωÛ·ÌËÚ ÔË‡ÏˉÌË Ó·‡ÁÛ‚‡ÌËfl ÎËÔÒ‚‡Ú ËÎË Ò‡ ÒËÎÌÓ Ì‡Ï‡ÎÂÌË ÔÓ ‡ÁÏÂ. àÁÒΉ‚‡ÌËflÚ‡ ̇ ÏË¯Ë ÏÓ‰ÂÎË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔË ÍÓËÚÓ L1CAM „Â̇  Ë̇ÍÚË‚Ë‡Ì (L1CAM knock-out Ï˯ÍË) ÔÓ͇Á‚‡Ú, ˜Â Ô˘Ë̇ڇ Á‡ ÚÓ‚‡ ‡·ÌÓχÎÌÓ ‡Á‚ËÚË ̇ ÍÓÚËÍÓÒÔË̇ÎÌËfl Ú‡ÍÚ Â Ì‡Û¯Â̇ڇ Sema3A ÍÎÂÚ˙˜Ì‡Ú‡ Ò˄̇ÎËÁ‡ˆËfl. èÓ‡‰Ë ÌÂÔ‡‚ËÎÌÓ Ì‡ÒÓ˜ÂÌËfl ‡ÒÚÂÊ, „ÓÎflχ ˜‡ÒÚ ÓÚ ‡ÍÒÓÌËÚ ̇ ÍÓÚËÍÓÒÔË̇ÎÌËfl Ú‡ÍÚ Ì ÏÓ„‡Ú ‰‡ ÔÂÏËÌ‡Ú ‚ ωÛ·ڇ Ë ‰ÂÒˆẨË‡Ú ËÔÒË·ÚÂ‡ÎÌÓ. Ç ÂÁÛÎÚ‡Ú Ì‡ ÚÓ‚‡ ÔÓÂ͈ËflÚ‡ ̇ ‡ÍÒÓÌËÚ ÔÓ‰ ˆÂ‚Ë͇ÎÌËÚ ÌË‚‡  ÒËÎÌÓ ‰ۈË‡Ì‡. PLP1 Ë Ì„ӂËfl ÒÍ˙ÒÂÌ ‚‡ˇÌÚ DM20 Ò‡ ÒÚÛÍÚÛÌË ÍÓÏÔÓÌÂÌÚË Ì‡ ÏËÂÎËÌÓ‚‡Ú‡ Ó·‚˂͇ Ë ËÏ‡Ú ÓÒÌӂ̇ ÓÎfl ‚ ÁÂÂÌÂÚÓ Ì‡ ÓÎË„Ó‰ẨÓˆËÚËÚÂ. ÑÂÙÂÍÚË ‚ PLP1 „Â̇ Ò‡ ÓÚ„Ó‚ÓÌË Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÍÓÏÔÎÂÍÒÌË Ë ÔÓfl‰ÍÓ ˜ËÒÚË ÙÓÏË Ì‡ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, SPG2, ͇ÍÚÓ Ë Á‡ ·ÓÎÂÒÚÚ‡ ̇ Pelizaeus-Merzbacher (PMD) (29). åÛÚ‡ˆËÓÌÌËflÚ ÒÔÂÍÚ˙ ÔÓ͇Á‚‡ Í·ÒÚÂË‡Ì ̇ ‰‚ÂÚ Á‡·ÓÎfl‚‡ÌËfl. è˙ÎÌË ‰ÂΈËË ËÎË ‰ÛÔÎË͇ˆËË Ì‡ „Â̇ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔË ÚÂÊÍË ÒÎÛ˜‡Ë ̇ PMD. åÛÚ‡ˆËËÚÂ, ‚Ó‰Â˘Ë ‰Ó çëè Ò‡ ÔÓ„¯ÌÓÒÏËÒÎÂÌË (missense) Ë ÌËÍÓ„‡ Ì Ò ̇·Î˛‰‡‚‡Ú ÔË Ô‡ˆËÂÌÚË Ò PMD. íÓ˜ÌËflÚ ÏÂı‡ÌËÁ˙Ï, ÔÓ ÍÓÈÚÓ ÓÚ‰ÂÎÌËÚ PLP1 ÏÛÚ‡ˆËË Ô‰ËÁ‚ËÍ‚‡Ú ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl Ì  ËÁflÒÌÂÌ, ÌÓ Â ÛÒÚ‡ÌÓ‚Â̇ ‚˙Á͇ ÏÂÊ‰Û Ì‡·Î˛‰‡‚‡ÌËfl ÙÂÌÓÚËÔ Ë Ì‡Û¯Â̇ڇ ÒÔÓÒÓ·ÌÓÒÚ Á‡ ÔÂÌÓÒ Ì‡ ÏÛÚË‡ÎËfl PLP1. èӂ˯ÂÌË ÌË‚‡ ̇ ÂÍÒÔÂÒËfl ̇ PLP1 ‚ ÒΉÒÚ‚Ë ̇ Ô˙Î̇ „ÂÌ̇ ‰ÛÔÎË͇ˆËfl Ә‚ˉÌÓ Á‡ÚÛ‰Ìfl‚‡Ú Ô‡ÍÂÚË‡ÌÂÚÓ, ÔÂÌÓÒ‡ Ë ËÁ‚Âʉ‡ÌÂÚÓ Ì‡ ·ÂÎÚ˙˜ÌËfl ÔÓ‰ÛÍÚ ÓÚ ÍÎÂÚ͇ڇ Ë ‚Ó‰flÚ ‰Ó ̇ÚÛÔ‚‡ÌÂÚÓ ÏÛ ‚ ÒÂÍÂÚÓÌËÚ Ô˙Úˢ‡. ë˙˘ËflÚ ÂÙÂÍÚ Ò ̇·Î˛‰‡‚‡ Ë ÔË ˜‡ÒÚ ÓÚ missense ÏÛÚ‡ˆËËÚÂ, ÍÓËÚÓ ‚ÂÓflÚÌÓ Á‡Òfl„‡Ú ‚‡ÊÌË Á‡ ÓÒ˙˘ÂÒÚ‚fl‚‡ÌÂÚÓ Ì‡ ÚÂÁË ÔÓˆÂÒË Û˜‡ÒÚ˙ˆË ÓÚ PLP1. ë˙˘Â‚ÂÏÂÌÌÓ, ÏÛÚ‡ˆËË ÔÓÁ‚ÓÎfl‚‡˘Ë ̇ ·ÂÎÚ˙͇ ‰‡ ‰ÓÒÚË„Ì ÍÎÂÚ˙˜Ì‡Ú‡ ÔÓ‚˙ıÌÓÒÚ Ò‡ ‡ÒÓˆË‡ÌË Ò ÔÓ-ÎÂ͇ ÍÎËÌ˘̇ ËÁfl‚‡ ̇ çëè. 凄ÌËÚÌËfl ÂÁÓ̇ÌÒ ÔË Ô‡ˆËÂÌÚË Ò SPG2 ÔÓ͇Á‚‡ ËÁÏÂÌÂÌËfl ‚ ·flÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó, ÔÓ‰Ó·ÌË Ì‡ ÚÂÁË ÔË ‰ÂÏËÂÎËÌËÁË‡˘ËÚ Ò˙ÒÚÓflÌËfl. PLP1 knock-out Ï˯ÍË ÙÓÏË‡Ú ÍÓÏÔ‡ÍÚ̇ ÏËÂÎËÌÓ‚‡ Ó·‚˂͇, ÌÓ ‚ ÔÓÒΉÒÚ‚Ë ‡Á‚Ë‚‡Ú ¯ËÓÍÓ Á‡ÒÚ˙ÔÂÌË ‡ÍÒÓ̇ÎÌË Á‡‰Â·ÂÎfl‚‡ÌËfl Ë ‰Â„ÂÌÂ‡ˆËfl (37). íÂÁË ‰‡ÌÌË ÔÓ͇Á‚‡Ú, ˜Â ‚ ÒΉÒÚ‚Ë ̇ ̇Û¯Â̇ڇ ÒË Ï‡ÚÛ‡ˆËfl, ÓÎË„Ó‰ẨÓˆËÚËÚ Ì ҇ ‚ Ò˙ÒÚÓflÌË ‰‡ ÓÒ˙˘ÂÒÚ‚flÚ ÎÓ͇Î̇ڇ ÔÓ‰‰˙Ê͇ ̇ ‚˜ ÏËÂÎËÌËÁË‡ÌËfl ‡ÍÒÓÌ. ëΉӂ‡ÚÂÎÌÓ, ‚ ÓÒÌÓ‚‡Ú‡ ̇ ‡ÍÒÓ̇Î̇ڇ ‰Â„ÂÌÂ‡ˆËfl ÔË SPG2/PMD ÎÂÊË ÔÂÍ˙Ò‚‡ÌÂÚÓ Ì‡ ‡ÍÒÓÌ-„ΡÎÌËÚ ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ëfl. ÇÚÓ‡Ú‡ ÓÒÌӂ̇ „ÛÔ‡ „ÂÌË, ˜ËËÚÓ ‰ÂÙÂÍÚË ‚Ó‰flÚ ‰Ó ‡Á‚ËÚËÂÚÓ Ì‡ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl Ò‡ Ò‚˙Á‡ÌË Ò ẨÓÁÓÏÌËfl ÔÂÌÓÒ, ÏËÍÓÚÛ·ÛÎ̇ڇ ‰Ë̇ÏË͇ Ë ÂÌÂ„ËËÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï. èÓ‡‰Ë „ÓÎflχڇ ÒË ‰˙ÎÊË̇ Ë ÒËÎÌÓ ÔÓÎflËÁË‡Ì‡ ‡ıËÚÂÍÚÛ‡, ÏÓÚÓÌËÚ Ì‚ÓÌË Ò‡ ÒËÎÌÓ Á‡‚ËÒËÏË ÓÚ ÒÎÓÊ̇ڇ Ú‡ÌÒÔÓÚ̇ ÒËÒÚÂχ, ÒÚÛÍÚÛÌÓ ·‡ÁË‡Ì‡ ‚˙ıÛ ˆËÚÓÒÍÂÎÂÚ‡ Ë Ó„‡ÌËÁË‡Ì‡ ڇ͇ ˜Â ‰‡ ÓÒË„ÛË Ô‡‚ËÎÌÓÚÓ Ôˉ‚ËÊ‚‡ÌÂ Ë ‰ÓÒÚ‡‚͇ ̇ ÍÎÂÚ˙˜ÌË ÍÓÏÔÓÌÂÌÚË ‰Ó ̇È-ÓÚ‰‡Î˜ÂÌËÚ Í‡Ë˘‡ ̇ ‡ÍÒÓ̇. éÒÌÓ‚ÂÌ ÍÓÏÔÓÌÂÌÚ Ì‡ Ú‡ÁË ÒËÒÚÂχ Ò‡ ÏËÍÓÚÛ·ÛÎËÚÂ, ÔÓ ÍÓËÚÓ ‡Á΢ÌË ÏÓÚÓÌË ·ÂÎÚ˙ˆË Ôˉ‚ËÊ‚‡Ú ÚÓ‚‡Ë Ò ‡Á΢̇ ÒÍÓÓÒÚ (·‡‚ÂÌ Ë ·˙Á Ú‡ÌÒÔÓÚ) Ë ‚ ‡Á΢̇ ÔÓÒÓ͇ (‡ÌÚÂÓ„‡‰ÂÌ Ë ÂÚÓ„‡‰ÂÌ Ú‡ÌÒÔÓÚ) ̇ „ÓÎÂÏË ‡ÁÒÚÓflÌËfl ÔÓ ‰˙ÎÊË̇ڇ ̇ ÌÂ‚ÌÓÚÓ ‚·ÍÌÓ. Ñ‚‡ ÓÒÌÓ‚ÌË ·ÂÎÚ˙͇ ̇ ÏËÍÓÚÛ·ÛÎÌËfl Ô˙Ú – ÒÔ‡ÒÚËÌ Ë KIF5A Ò‡ Ò‚˙Á‡ÌË Ò ÍÎËÌ˘̇ڇ ËÁfl‚‡ ̇ çëè.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

åÛÚ‡ˆËË ‚ ÒÔ‡ÒÚËÌÓ‚Ëfl „ÂÌ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‚ 40% ÓÚ ÒÎÛ˜‡ËÚ ̇ ÄÑçëè, ÍÓÂÚÓ Ô‡‚Ë SPG4 ̇È-˜ÂÒÚÓ Ò¢‡Ì‡Ú‡ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (16). åÛÚ‡ˆËÓÌÌËflÚ ‡Ì‡ÎËÁ ÔÓ͇Á‚‡ ¯ËÓÍ ÒÔÂÍÚ˙ ÏÓÎÂÍÛÎÌÓ-„ÂÌÂÚ˘ÌË ÔÓÏÂÌË – ‰ÂΈËË, ËÌÒÂˆËË, frameshift, missense, nonsense Ë splice-site ÏÛÚ‡ˆËË. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË Ò SPG4 ‡Á‚Ë‚‡Ú “˜ËÒÚ‡” ÙÓχ ̇ çëè, ‡ 25% ÓÚ Úflı ÓÒÚ‡‚‡Ú ‡ÒËÏÔÚÓχÚ˘ÌË ÔÂÁ ˆÂÎËflÚ ÒË ÊË‚ÓÚ. ï‡‡ÍÚÂÌÓ Á‡ SPG4 Â, ˜Â ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ ‚‡Ë‡Ú ͇ÍÚÓ ÔË ÓÚ‰ÂÎÌËÚ هÏËÎËË Ú‡Í‡ Ë ÏÂÊ‰Û ·ÓÎÌËÚ ÓÚ Â‰Ì‡ Ù‡ÏËÎËfl. ։̇ ÓÚ Ô˘ËÌËÚ Á‡ ÚÓ‚‡  ̇΢ËÂÚÓ Ì‡ ‚˙Ú„ÂÌÌË ÏÓ‰ËÙË͇ÚÓË ‚ Ò‡ÏËfl ÒÔ‡ÒÚËÌÓ‚ „ÂÌ. ë˙Ó·˘ÂÌË Ò‡ ‰‚‡ ÔÓÎËÏÓÙËÁχ ‚ ÍÓ‰Ë‡˘‡Ú‡ ˜‡ÒÚ Ì‡ „Â̇, ÍÓËÚÓ ‚ ÍÓÏ·Ë̇ˆËfl Ò ‰Ó͇Á‡ÌÓ Ô‡ÚÓ„ÂÌÌË ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË ‚Ó‰flÚ ‰Ó Á̇˜ËÚÂÎÌÓ ÔÓÌËʇ‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (35). ëÔ‡ÒÚË̇  ˜ÎÂÌ Ì‡ „ÛÔ‡Ú‡ ̇ Äíî‡ÁÌË ·ÂÎÚ˙ˆË ‡ÒÓˆË‡ÌË Ò ‡Á΢ÌË ÍÎÂÚ˙˜ÌË ‡ÍÚË‚ÌÓÒÚË (ÄÄÄ). èËÚÂʇ‚‡ ‰‚‡ ÙÛÌ͈ËÓ̇ÎÌÓ ‚‡ÊÌË ‰ÓÏÂ̇ – Äíî-Ò‚˙Á‚‡˘/Äíî‡ÁÂÌ Ë MIT (microtubule-interacting and trafficking molecules) ‰ÓÏÂÌ Ô‰ÒÚ‡‚ÂÌ ‚ ẨÓÁÓÏÌÓ- ‡ÒÓˆËË‡ÌËÚ ÏÓÎÂÍÛÎË (5). à„‡Â ‚‡Ê̇ ÓÎfl ‚ ÔÓ‰‰˙ʇÌÂÚÓ Ì‡ ÏËÍÓÚÛ·ÛÎ̇ ÏÂʇ. àÁÒΉ‚‡ÌËflÚ‡ in vitro ÔÓ͇Á‚‡Ú, ˜Â ÒÔ‡ÒÚË̇ Ò ҂˙Á‚‡ ÔÂıÓ‰ÌÓ Í˙Ï ÔÓÎËÏÂËÁË‡ÎËÚ ÏËÍÓÚÛ·ÛÎË ÔÓ Äíî-Á‡‚ËÒËÏ Ì‡˜ËÌ, ˜ÂÁ MIT ‰ÓÏÂ̇ Ë, ˜Â Äíî-‡Á̇ڇ ÏÛ ‡ÍÚË‚ÌÓÒÚ Â ÌÂÓ·ıÓ‰Ëχ Á‡ ‰‡ Ò ÓÚ‰ÂÎË ÓÚ ÏËÍÓÚÛ·ÛÎËÚÂ, ͇ÚÓ „Ë ‰ÂÁËÌÚ„Ë‡ (8, 20), Ú.Â., ÌÓχÎÌÓ ÒÔ‡ÒÚË̇  ‰ËÒÓˆË‡˘ ÏËÍÓÚÛ·ÛÎËÚ ‡„ÂÌÚ. åÛÚ‡ÌÚÌËfl ÒÔ‡ÒÚËÌ ÍÓÎÓ͇ÎËÁË‡ Ò ÏËÍÓÚÛ·ÛÎËÚÂ, ÌÓ Ì Ò ‰ËÒÓˆË‡ ÓÚ Úflı, ÍÓÂÚÓ Â Ò‚˙Á‡ÌÓ Ò Ì‡Î˘ËÂÚÓ Ì‡ ÒÌÓÔ˜ÂÚ‡ ÓÚ ÏËÍÓÚÛ·ÛÎË Ë ÔÂËÌÛÍ·ÌË Í·ÒÚÂË ÓÚ ÏËÚÓıÓ̉ËË Ë ÔÂÓÍÒËÁÓÏË. ëΉӂ‡ÚÂÎÌÓ, ÓÒÌÓ‚ÌËfl Ô‡ÚÓÎӄ˘ÂÌ ÏÂı‡ÌËÁ˙Ï ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË Â Ì‡Û¯ÂÌ Ó„‡ÌÂÎÂÌ Ú‡ÌÒÔÓÚ ÔÓ ÏËÍÓÚÛ·ÛÎËÚ ‚ ÂÁÛÎÚ‡Ú Ì‡ ‡·ÌÓχÎÌÓÚÓ ËÏ ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂ Ò ÏÛÚ‡ÌÚÌËfl ÒÔ‡ÒÚËÌ. àÁÒΉ‚‡ÌÂÚÓ Ì‡ ÔÓÏÂÌËÚ ‚ ÏÓÎÂÍÛÎ̇ڇ ‰Ë̇ÏË͇ ‚ ÏÓÙÓÎӄ˘ÌÓ ÌÓχÎ̇ ÏÛÒÍÛÎ̇ Ú˙Í‡Ì ÔË SPG4 Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡ ÒËÎÌÓ ÔÓÌËÊÂ̇ ÂÍÒÔÂÒËfl ̇ „ÂÌË ‡ÒÓˆË‡ÌË Ò ÏËÚÓıÓ̉ˇÎÌËfl, ·ÂÎÚ˙˜ÌËfl Ë ‚ÂÁËÍÛ·ÂÌ ÔÂÌÓÒ (22). ÇÂÓflÚÌÓ ÂÍÒÔÂÒËflÚ‡ ̇ ÚÂÁË Ô˙Úˢ‡ ͇ÚÓ ˆflÎÓ Â ÒËÎÌÓ „ÛÎË‡Ì‡ ̇ Ú‡ÌÒÍËÔˆËÓÌÌÓ ÌË‚Ó Ò ÔÓÏÓ˘Ú‡ ̇ ÒÔ‡ÒÚË̇, ÍÓÈÚÓ “ÒΉ˔ Á‡ ÒÔ‡Á‚‡Ì ̇ ÍÓÂÍÚÌËÚ ÒÚÂıËÓÏÂÚ˘ÌËÚ Ò˙ÓÚÌÓ¯ÂÌËfl ̇ ‚Ò˘ÍË ÍÓÏÔÓÌÂÌÚË. ëÔ‡ÒÚËÌÓ‚Ëfl ·ÂÎÚ˙Í Ò ÂÍÒÔÂÒË‡ ÔÓ‚ÒÂÏÂÒÚÌÓ ‚ ˜Ó‚¯ÍËfl Ó„‡ÌËÁ˙Ï Ë ÒΉӂ‡ÚÂÎÌÓ Ôӂ˜ÂÚÓ ÍÎÂÚÍË ‚ ˜Ó‚¯ÍÓÚÓ ÚflÎÓ ËÏ‡Ú Ò˙˘ËÚ ̇Û¯ÂÌËfl ‚ ÏËÍÓÚÛ·ÛÎÌËfl Ú‡ÌÒÔÓÚ, ÌÓ Ì ÔÓ͇Á‚‡Ú ‰‡ÌÌË Á‡ ÍÎÂÚ˙˜Ì‡ ‰ËÒÙÛÌ͈Ëfl. èÓÏÂÌÂÌËfl ÏËÍÓÚÛ·ÛÎÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï ÔË SPG4 ‚Ó‰Ë ‰Ó Ô‡ÚÓÎӄ˘ÌË ËÁÏÂÌÂÌËfl ‚ ‰˙΄ËÚ ÌËÁıÓ‰fl˘Ë Ú‡ÍÚӂ ̇ ÏÓÚÓÌËÚ Ì‚ÓÌË, ÍÓËÚÓ Ò ‚ÎËflflÚ ÔÓ-ÒËÎÌÓ ÓÚ ÂÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ÏËÍÓÚÛ·ÛÎÂÌ Ú‡ÌÒÔÓÚ. ÄÎÚÂ̇ÚË‚ÌËfl ÏÓ‰ÂÎ Â, ˜Â ÔÓÏÂÌË ‚ ÂÍÒÔÂÒËflÚ‡ ̇ ÚÛ·ÛÎËÌÓ‚Ëfl Ô˙Ú ‚ ÏÛÒÍÛÎËÚÂ, ÂÙÂÍÚË‚ÌÓ ÍÓÏÔÂÌÒË‡ Ô˙‚˘ÌËfl ÒÔ‡ÒÚËÌÓ‚ ‰ÂÙˈËÚ. Ç˙ÁÏÓÊÌÓ Â ÏÓÚÓÌËÚ Ì‚ÓÌË ‰‡ Ì ҇ ‚ Ò˙ÒÚÓflÌË ‰‡ ÓÒ˙˘ÂÒÚ‚flÚ ÔÓ‰Ó·ÌË ÍÓÏÔÂÌÒ‡ÚÓÌË ÔÓÏÂÌË, ÍÓÂÚÓ ‰‡ ‰Ó‚‰ ‰Ó Ô‡ÚÓÎÓ„Ëfl. KIF5A  Ì‚ÓÌ-ÒÔˆËÙ˘̇ ·ÂÎÚ˙˜Ì‡ ÏÓÎÂÍÛ·, Û˜‡ÒÚ‚‡˘‡ ‰ËÂÍÚÌÓ ‚˙‚ ‚˙ÚÂÍÎÂÚ˙˜ÌËfl ÏÂÏ·‡ÌÂÌ Ú‡ÙËÍ Í‡ÚÓ ÓÒÌÓ‚ÂÌ ÍÓÏÔÓÌÂÌÚ Ì‡ ÍËÌÂÁËÌ I (13). íÓÈ Ë„‡Â ÓÎflÚ‡ ̇ ÏËÍÓÚÛ·ÛÎÂÌ ÏÓÚÓ, ͇ÚÓ ÓÒ˙˘ÂÒÚ‚fl‚‡ ·˙ÁËfl ‡ÌÚÂÓ„‡‰ÂÌ Ú‡ÌÒÔÓÚ ‚ ‡ÍÒÓ̇, ËÁÔÓÎÁ‚‡Ì Á‡ ÔÂÌÓÒ Ì‡ ‚ÂÁËÍÛÎË, ÏÂÏ·‡ÌË Ë ÏÂÏ·‡ÌÌË Ó„‡ÌÂÎË Í˙Ï ÍÎÂÚ˙˜Ì‡Ú‡ ÔÂËÙÂËfl (17). Ö‰ËÌÒÚ‚Â̇ڇ Á‡ Ò„‡

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ÏÛÚ‡ˆËfl ‚ „Â̇ Á‡ KIF5A Á‡Òfl„‡ ‚ËÒÓÍÓÍÓÌÒÂ‚‡ÚË‚ÂÌ ÏÓÚË‚ ‚ ÏÓÚÓÌËfl ‰ÓÏÂÌ Ì‡ ÏÓÎÂÍÛ·ڇ Ë ‚Ó‰Ë ‰Ó SPG10 (28). ÑÛ„ ‚‡ÊÂÌ ÍÓÏÔÓÌÂÌÚ Ì‡ ÍÎÂÚ˙˜ÌËfl Ú‡ÌÒÔÓÚ Ò‡ ẨÓÁÓÏËÚÂ, Ô‰ÒÚ‡‚Îfl‚‡˘Ë ˆÂÌÚ‡ÎËÁË‡Ì‡ ÒËÒÚÂχ Á‡ Ô‡ÍÂÚË‡ÌÂ, ÔÂÌÓÒ Ë ÒÓÚË‡Ì ̇ ÔÓ‰ÛÍÚËÚ ̇ ·ËÓÒËÌÚÂÁ‡ Ë ·ËӉ„‡‰‡ˆËfl ‚ ÍÎÂÚ͇ڇ. åÛÚ‡ˆËË ‚ ÚË „Â̇ - ‡Ú·ÒÚËÌ, Äëê33 Ë ÒÔ‡ÚËÌ, ˜ËËÚÓ ·ÂÎÚ˙˜ÌË ÔÓ‰ÛÍÚË Ò‡ Ò‚˙Á‡ÌË Ò ẨÓÁÓÏ̇ڇ ‰Ë̇ÏË͇ ‚Ó‰flÚ ‰Ó çëè. ÄÚ·ÒÚËÌ˙Ú Â ÏÛÎÚËÏÂÂÌ ËÌÚ„‡ÎÂÌ ÏÂÏ·‡ÌÂÌ ÔÓÚÂËÌ (39), ˜ÎÂÌ Ì‡ ‰Ë̇ÏËÌÓ‚‡Ú‡ ÒÛÔÂÙ‡ÏËÎËfl „ÓÎÂÏË Éíî‡ÁË (38). ãÓ͇ÎËÁË‡Ì  ‚ ÏÂÏ·‡ÌËÚ ̇ ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË. ÖÍÒÔÂÒË‡ Ò Ô‰ËÏÌÓ ‚ „·‚ÌËfl ÏÓÁ˙Í, ̇È-‚˜ ‚ ÔË‡ÏˉÌËÚ Ì‚ÓÌË Ì‡ ÏÓÁ˙˜Ì‡Ú‡ ÍÓ‡ Ë ‚ ıËÔÓ͇ÏÔÛÒ‡. íӘ̇ڇ ÏÛ ÙÛÌ͈Ëfl  ‚Ò Ӣ ÌÂËflÁÂÒÌÂ̇, ÌÓ Ì„ӂËÚ ıÓÏÓÎÓÁË Û˜‡ÒÚ‚‡Ú ‚˙‚ ‚ÂÁËÍÛ·ÌËfl Ú‡ÌÒÔÓÚ, ÏÓ„‡Ú ‰‡ Ò ҂˙Á‚‡Ú Ò ÏËÍÓÚÛ·ÛÎËÚÂ Ë Ò‡ ÓÚ„Ó‚ÓÌË Á‡ ·˙ÁÓÚÓ Ë ÂÙÂÍÚË‚ÌÓ ˆËÍÎË‡Ì ̇ ÒË̇ÔÚ˘ÌËÚ ‚ÂÁËÍÛÎË Ë Á‡ ÔÓ‰‰˙ʇÌ ÏÓÙÓÎÓ„ËflÚ‡ ̇ ÒË̇ÔÚ˘̇ڇ ÏÂÏ·‡Ì‡, ÍÓÂÚÓ Â ÓÚ ÓÒÌÓ‚ÌÓ Á̇˜ÂÌË Á‡ Ì‚ÓÚ‡ÌÒÏËÒËflÚ‡ Ë ÚÓÙË͇ڇ ̇ ÌÂ‚̇ڇ ÍÎÂÚ͇. åÛÚ‡ˆËË ‚ ‡Ú·ÒÚËÌÓ‚Ëfl „ÂÌ ‚Ó‰flÚ ‰Ó ˜ËÒÚ‡ ÙÓχ ̇ çëè Ò Ì‡˜‡ÎÓ ‚ ‡Ì̇ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ Ë ÚÂÊÍÓ ÔÓÚ˘‡Ì ̇ ·ÓÎÂÒÚÚ‡, SPG3Ä. í‡ÁË ÙÓχ Ò Ò¢‡ ÔË 10% ÓÚ ÒÎÛ˜‡ËÚ ̇ ÄÑçëè. åÂı‡ÌËÁÏ˙Ú Ì‡ ‰ÂÈÒÚ‚Ë ̇ ‡Ú·ÒÚËÌÓ‚ËÚ ÏÛÚ‡ˆËË Â ÌÂËÁflÒÌÂÌ, ÌÓ Ò Ô‰ÔÓ·„‡, ˜Â ‚Ó‰flÚ ‰Ó ̇Û¯ÂÌËfl ‚ ÏÂÏ·‡ÌÌËÚ ÒÚÛÍÚÛË Ì‡ ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË, ÍÓÂÚÓ ‚˙ÁÔÂÔflÚÒÚ‚‡ ‡ÒÚÂʇ ̇ ‡ÍÒÓ̇. åÛÚ‡ˆËË ‚ „ÂÌ ÒÔ‡ÚËÌ ‚Ó‰flÚ ‰Ó ‡Á‚ËÚËÂÚÓ Ì‡ Troyer ÒË̉ÓÏ, SPG20 (24), ÍÓÏÔÎÂÍÒ̇ ÙÓχ ̇ çëè ‡ÒÓˆË‡Ì‡ Ò ‰ËÒÚ‡Î̇ ‡ÏËÓÚÓÙËfl, ‰ËÁ‡ÚËfl, ÎÂÍÓ ËÁÓÒÚ‡‚‡Ì ‚ ‡Á‚ËÚËÂÚÓ Ë ÌËÒ˙Í ˙ÒÚ. ÅÂÎÚ˙˜Ì‡Ú‡ ÏÓÎÂÍÛ· ̇ ÒÔ‡ÚË̇ ÔÓ͇Á‚‡ ıÓÏÓÎÓ„Ëfl Ò ÔÓÚÂËÌË Ò‚˙Á‡ÌË Ò ẨÓÁÓÏ̇ڇ ÏÓÙÓÎÓ„Ëfl Ë Û˜‡ÒÚ‚‡˘Ë ‚ ÔÂÌÓÒ‡ ̇ ·ÂÎÚ˙ˆË ‚ Í˙ÒÌËÚ ẨÓÁÓÏÌË ÍÓÏÔÓÌÂÌÚË. èÓ‰Ó·ÌÓ Ì‡ ÒÔ‡ÒÚË̇, ÔËÚÂʇ‚‡ ÙÛÌ͈ËÓ̇ÎÂÌ ‰ÓÏÂÌ Ô‰ÒÚ‡‚ÂÌ ‚ ẨÓÁÓÏÌÓ ‡ÒÓˆË‡˘ËÚ Ò ÏÓÎÂÍÛÎË, MIT-‰ÓÏÂÌ. Ö‰ËÌÒÚ‚Â̇ڇ Á‡ Ò„‡ ÏÛÚ‡ˆËfl ‚ „ÂÌ Äëê33 (32)  ÛÒÚ‡ÌÓ‚Â̇ ÔË ÍÓÏÔÎÂÍÒ̇ ÙÓχ ̇ çëè ‡ÒÓˆËË‡Ì‡ Ò ‰ÂÏÂ̈Ëfl, Mast ÒË̉ÓÏ, SPG21 (6). íÓÁË „ÂÌ ÍÓ‰Ë‡ ÔÓÚÂËÌ, ̇˜ÂÌ “χÒÔ‡‰ËÌ”. ëÛ·ÍÎÂÚ˙˜Ì‡Ú‡ ÏÛ ÎÓ͇ÎËÁ‡ˆËfl ‚ ‡ÌÌËÚ ẨÓÁÓÏÌË ÒÚÛÍÚÛË Ì‡ ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË ÔÓ‰Ò͇Á‚‡ ‚ÂÓflÚ̇ ÏÛ ÓÎfl ‚˙‚ ‚ÂÁËÍÛ·ÌÓωËË‡ÌËfl ÔÂÌÓÒ Ë ÒÓÚË‡Ì ̇ ÍÎÂÚ˙˜ÌËÚ ÔÓÚÂËÌË. åÛÚ‡ˆËË ‚ ‰‚‡ fl‰ÂÌÓÍÓ‰Ë‡ÌË ÏËÚÓıÓ̉ˇÎÌË ·ÂÎÚ˙͇, HSP60 Ë Ô‡‡Ô΄ËÌ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔË ‰‚ ÓÚ ÙÓÏËÚ ̇ çëè, ÍÓÂÚÓ ÔÓ͇Á‚‡, ˜Â ÚÂÏË̇ÎÌËÚ ˜‡ÒÚË Ì‡ ‰˙΄ËÚ ‡ÍÒÓÌË ‚ ñçë Ò‡ ÓÒÓ·ÂÌÓ ˜Û‚ÒÚ‚ËÚÂÎÌË Í˙Ï ‰ÂÙÂÍÚË ‚ ÂÌÂ„ËÈÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï. HSP60 ÔË̇‰ÎÂÊË Í˙Ï ÚËÔ1 ¯‡ÔÂÓÌËÌÓ‚Ë (ÔÓÏÓ˘ÌË) ·ÂÎÚ˙ˆË Ò ı‡‡ÍÚÂ̇ ˆËÎË̉˘̇ ÙÓχ. á‡Â‰ÌÓ Ò˙Ò Ò‚Ófl ÍÓ-¯‡ÔÂÓÌËÌ HSP10, ͇ÚÓ ÍÓÏÔÓÌÂÌÚ Ì‡ ÏÛÎÚËÏÂÂÌ ÍÓÏÔÎÂÍÒ, Û˜‡ÒÚ‚‡ ‚ Ô‡‚ËÎÌÓÚÓ Ì‡„˙‚‡Ì ̇ ÔÓÚÂËÌËÚ ‚ ÏËÚÓıÓ̉ËËÚÂ. ë‰ÂÏ Ò۷‰ËÌËˆË ÓÚ HSP60 Ó·‡ÁÛ‚‡Ú ‰ËÌ˘ÂÌ ˆËÎË̉˘ÂÌ Ô˙ÒÚÂÌ, ‚ ÍÓËÚÓ Ò ‡ÁÔÓ·„‡ ÌÓ‚ÓÒËÌÚÂÁË‡ÌËfl ÌÂ̇„˙Ì‡Ú ÔÓÎËÔÂÔÚˉ. íÓÁË Ô˙ÒÚÂÌ ‚Á‡ËÏÓ‰ÂÈÒÚ‚‡ Ò Â‰ËÌ˘ÌËfl Ô˙ÒÚÂÌ Ó·‡ÁÛ‚‡Ì ÓÚ Ò‰ÂÏ ÒÛ·ÂÌËˆË Ì‡ HSP10, ÍÓÈÚÓ ÒÎÛÊË Í‡ÚÓ Í‡Ô‡˜Â ̇ HSP60 ˆËÎË̉˙‡. í‡Í‡ ÌÓ‚Ëfl ·ÂÎÚ˙Í ÏÓÊ ‰‡ ÙÓÏË‡ Ò‚ÓflÚ‡ ‚ÚÓ˘̇ Ë ÚÂÚ˘̇ڇ ÒÚÛÍÚÛ‡ ÔË ÔÓ‰ıÓ‰fl˘Ë ÛÒÎÓ‚Ëfl (23). åÛÚ‡ˆËfl ‚ HSP60  ÛÒÚ‡ÌÓ‚Â̇ ÔË ˜ËÒÚ‡ ÙÓχ ̇ çëè, Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Û̇ÒΉfl‚‡ÌÂ, SPG13 (14). è‡ÚÓÎӄ˘ÌËflÚ ÏÂı‡ÌËÁ˙Ï Ì‡


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

‰ÂÈÒÚ‚Ë ̇ ÏÛÚ‡ˆËflÚ‡ Ì  ËÁflÒÌÂÌ, ÌÓ ‚ÂÓflÚÌÓ Ò ͇҇ Á‡ Á‡„Û·‡ ̇ ÙÛÌ͈Ëfl ËÎË ‰ÓÏË̇ÌÚÌÓ Ì„‡ÚË‚ÂÌ ÂÙÂÍÚ ‚˙ıÛ ÏÛÎÚËÏÂÌËfl ¯‡ÔÂÓÌÂÌ ÍÓÏÔÎÂÍÒ, ‚ ÍÓÈÚÓ Û˜‡ÒÚ‚‡ ‚ HSP60. è‡‡Ô΄ËÌÓ‚ËflÚ ·ÂÎÚ˙Í Â Zn-Ò˙‰˙ʇ˘‡ ÏÂÚ‡ÎÓÔÓÚ‡Á‡ ÓÚ „ÛÔ‡Ú‡ ̇ ÏËÚÓıÌÓ‰ˇÎÌËÚ ÄÄÄ ÔÓÚÂËÌË Ò ÔÓÚÂÓÎËÚ˘̇ Ë ¯‡ÔÂÓÌ̇ ÙÛÌ͈Ëfl (2). í ‡ÍÚË‚Ë‡Ú ‡ÒÂÏ·ÎË‡ÌÂÚÓ Ì‡ ÍÓÏÔÎÂÍÒËÚ ‚ ‰Ëı‡ÚÂÎÌËÚ ‚ÂË„Ë Ë Û˜‡ÒÚ‚‡Ú ‚ “͇˜ÂÒÚ‚ÂÌËfl ÍÓÌÚÓΔ ̇ ÏËÚÓıÓ̉ˇÎÌËÚ ·ÂÎÚ˙ˆË, ͇ÚÓ Ò‚˙Á‚‡Ú ÔÓ„¯ÌÓ Ú‡ÌÒÎË‡ÌËÚ ËÎË ÌÂÔ‡‚ËÎÌÓ ÍÓÌÙË„ÛË‡ÌË ÔÓÚÂËÌË Ë ÓÒË„Ûfl‚‡Ú Úflı̇ڇ ÒÔˆËÙ˘̇ ÔÓÚÂÓÎËÁ‡ (18). á‡Â‰ÌÓ Ò ‰Û„‡ ÏËÚÓıÓ̉ˇÎ̇ ÏÂÚ‡ÎÓÔÓÚ‡Á‡, Ô‡‡Ô΄Ë̇ Û˜‡ÒÚ‚‡ ‚ Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ̇‰ÏÓÎÂÍÛÎÂÌ ÍÓÏÔÎÂÍÒ ‚˙‚ ‚˙Ú¯̇ڇ ÏËÚÓıÓ̉ˇÎ̇ ÏÂÏ·‡Ì‡. íÓÁË ÍÓÏÔÎÂÍÒ ÎËÔÒ‚‡ ÔË ÂÍÒÔÂÒËflÚ‡ ̇ ÏÛÚ‡ÌÚÂÌ Ô‡‡Ô΄ËÌÓ‚ ‡ÎÂÎ ‚ ÍÎÂÚ˙˜ÌË ÎËÌËË. åÛÚ‡ˆËË ‚ Ô‡‡Ô΄ËÌÓ‚Ëfl „ÂÌ ÏÓ„‡Ú ‰‡ ‰Ó‚‰‡Ú ‰Ó ˜ËÒÚ‡ ËÎË ÍÓÏÔÎÂÍÒ̇ ÙÓχ ̇ çëè ‡ÒÓˆË‡Ì‡ Ò ˆÂ·Â·̇, ÍÓÚË͇Î̇ Ë ÓÔÚ˘̇ ‡ÚÓÙËfl, SPG7. åÛÒÍÛÎÌË ·ËÓÔÒËË ÔË SPG7 Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú ÚËÔ˘ÌË ÔËÁ̇ˆË ̇ ‰ÂÙÂÍÚË ‚ ÓÍËÒÎËÚÂÎÌÓÚÓ ÙÓÒÙÓËÎË‡ÌÂ, ͇ÚÓ ÌË‚ÓÚÓ Ì‡ ÏËÚÓıÓ̉ˇÎ̇ ‰ËÒÙÛÌ͈Ëfl ÍÓÂÎË‡ Ò˙Ò ÒÚÂÔÂÌÚ‡ ̇ ÍÎËÌ˘ÌÓ Á‡Òfl„‡ÌÂ. ÖÎÂÍÚÓÌÌÓÏËÍÓÒÍÓÔÒÍÓÚÓ ÔÓÛ˜‚‡Ì ̇ Ínock-out Ï˯ÍË ÔÓ Ô‡‡Ô΄ËÌÓ‚ „ÂÌ ‡ÁÍË‚‡ „ÓÎflÏ ·ÓÈ ‡·ÌÓχÎÌË ÏËÚÓıÓ̉ËË ‚ ‡ÍÒÓ̇, ÏÌÓ„Ó ÔÂ‰Ë ‡Á‚ËÚËÂÚÓ Ì‡ ‡ÍÒÓ̇Î̇ ‰Â„ÂÌÂ‡ˆËfl. á̇˜ËÚÂÎÌÓ ÔÓÍ˙ÒÌÓ Ò ̇·Î˛‰‡‚‡ Á‡‰Â·ÂÎfl‚‡Ì ̇ ‡ÍÒÓ̇, Ò˙‰˙ʇ˘ ‡ÍÛÏÛÎË‡ÌË Ó„‡ÌÂÎË Ë Ì‚ÓÙË·ÏÂÌÚË (9). íÂÁË ‰‡ÌÌË, ͇ÍÚÓ Ë ÓÚÌÓÒËÚÂÎÌÓ Í˙ÒÌÓÚÓ Ì‡˜‡ÎÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔË SPG7 Ò˙ÓÚ‚ÂÚÒÚ‚‡Ú ̇ ıËÔÓÚÂÁ‡Ú‡ Á‡ ̇Û¯ÂÌ “͇˜ÂÒÚ‚ÂÌ ÍÓÌÚÓΔ ̇ ·ÂÎÚ˙˜Ì‡Ú‡ ÒËÌÚÂÁ‡. åÛÚ‡ÌÚÌËflÚ Ô‡‡Ô΄ËÌ Ì  ‚ Ò˙ÒÚÓflÌË ‰‡ ÂÎËÏËÌË‡ ÔÓ„¯ÌÓ Ì‡„˙̇ÚËÚ ·ÂÎÚ˙˜ÌË ÏÓÎÂÍÛÎË. íÓ‚‡ ‚Ó‰Ë ‰Ó ̇ÚÛÔ‚‡Ì ̇ ‡·ÌÓχÎÌË ÔÓÚÂËÌË ‚ ÏËÚÓıÓ̉ˇÎÌËfl χÚËÍÒ Ë ÔÓÒΉ‚‡˘‡ ÏËÚÓıÓ̉ˇÎ̇ ‰ËÒÙÛÌ͈Ëfl, ÍÓÂÚÓ ‚ÂÓflÚÌÓ Â Ô˘Ë̇ڇ Á‡ ‡ÍÒÓ̇Î̇ڇ ‰Â„ÂÌÂ‡ˆËfl ÔË SPG7 (3). åÛÚ‡ˆËfl ‚ ·ÂÎÚ˙˜Ì‡ ÏÓÎÂÍÛ· Ò ‚ËÒÓÍË ÌË‚‡ ̇ ÂÍÒÔÂÒËfl ‚ ÌÂ‚̇ڇ Ú˙Í‡Ì Ë ÌÂËÁ‚ÂÒÚ̇ ‰Ó ÚÓÁË ÏÓÏÂÌÚ ÙÛÌ͈Ëfl Ë ÒÛ·ÍÎÂÚ˙˜Ì‡ ÎÓ͇ÎËÁ‡ˆËfl, NIPA1 (26)  ÛÒÚ‡ÌÓ‚Â̇ ÔË SPG6 (10). ÅÂÎÚ˙˜Ì‡Ú‡ ÏÓÎÂÍÛ· ̇ NIPA1 Ì ÔÓ͇Á‚‡ ıÓÏÓÎÓ„Ëfl Ò ÌËÚÓ Â‰ËÌ ÓÚ ÓÒڇ̇ÎËÚ ÔÓÚÂËÌË ‚ çëè Ô‡ÚÓÎÓ„ËflÚ‡, ÌÓ ÍÎËÌ˘̇ڇ ͇ÚË̇ ÔË Ô‡ˆËÂÌÚË Ò SPG6  ÏÌÓ„Ó ÔӉӷ̇ ̇ Ú‡ÁË Ì‡·Î˛‰‡‚‡Ì‡ ÔË SPG4. äÓÏÔ˛Ú˙ÌËfl ‡Ì‡ÎËÁ ̇ Ô˙‚˘̇ڇ ÒÚÛÍÚÛ‡ Ô‰ÔÓ·„‡, ˜Â NIPA1  ËÌÚ„‡ÎÂÌ Ú‡ÌÒÏÂÏ·‡ÌÂÌ ·ÂÎÚ˙Í, ‚ÂÓflÚÂÌ ˆÂÔÚÓ ËÎË Ú‡ÌÒÔÓÚÂ. Ç˙ÔÂÍË ÌÂflÒ̇ڇ ÒË ÙÛÌ͈Ëfl, ËÁ‚ÂÒÚ̇ Ò‚ÂÚÎË̇ ‚˙ıÛ Ô‡ÚÓÎӄ˘ÌËÚ ÏÂı‡ÌËÁÏË ı‚˙Îfl Ù‡ÍÚ‡, ˜Â SPG6 ÙÓχڇ  ‡ÎÂÎÌÓ Á‡·ÓÎfl‚‡Ì ̇ PWS (Prader-Willi syndrome) Ë AS (Angelman syndrome). Ç „ÓÎflχ ˜‡ÒÚ ÓÚ ÒÎÛ˜‡ËÚ ̇ ÚÂÁË ‰‚‡ ÒË̉Óχ  ÛÒÚ‡ÌÓ‚Â̇ ıÓÏÓÁÓÏ̇ ‰ÂΈËfl 15q ÍÎ‡Ò I, ‚Íβ˜‚‡˘‡ NIPA1 „Â̇, ÌÓ Ô‡ˆËÂÌÚËÚ Ì ‡Á‚Ë‚‡Ú ÒËÏÔÚÓÏË Ì‡ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô‡ÂÁ‡, ÍÓÂÚÓ ÔÓ͇Á‚‡, ˜Â 50% ‰ÂÙˈËÚ Ì‡ NIPA1 ÔÓÚÂË̇ Ì  ÍËÚ˘ÌÓ ÌË‚Ó, Á‡ ‡Á‚ËÚË ̇ çëè ÒËÏÔÚÓχÚË͇. ÇÂÓflÚÌÓ ÏÛÚ‡ˆËËÚ ‚ NIPA1 ÛÔ‡ÊÌfl‚‡Ú Ò‚Ófl ÂÙÂÍÚ ÔÓ ‰ÓÏË̇ÌÚÌÓ Ì„‡ÚË‚ÂÌ ÏÂı‡ÌËÁ˙Ï. ç‡ÒÍÓÓ ÏÛÚ‡ˆËË ‚ „ÂÌ BSCL2 (36) ·flı‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚ‡Î̇ ÏÓÚÓÌÓÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl Ë ÍÓÏÔÎÂÍÒ̇ ÙÓχ ̇ çëè ‡ÒÓˆË‡Ì‡ Ò ‡ÏËÓÚÓÙËfl ̇ ˙ˆÂÚÂ Ë ÒÚ˙ԇ·ڇ, Silver syndrome (SPG17) (25). ÉÂÌ˙Ú BSCL2 ÍÓ‰Ë‡ ÒÂËÔËÌ, ËÌÚ„‡ÎÂÌ Ú‡ÌÒÏÂÏ·‡ÌÂÌ ·ÂÎÚ˙Í, ÎÓ͇ÎËÁË‡Ì ‚ ÏÂÏ·‡ÌËÚ ̇ Öèê. ÖÍÒÔÂÒË‡ Ò ‚ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ Ë Ï‡ÒÚ̇ڇ Ú˙͇Ì,

128

ÍÓÂÚÓ Ó·flÒÌfl‚‡ Ù‡ÍÚ‡, ˜Â ÏÛÚ‡ˆËË ‚ ÚÓÁË „ÂÌ ·flı‡ ÓÚÍËÚË Ô˙‚Ó̇˜‡ÎÌÓ ÔË Ô‡ˆËÂÌÚË Ò ÍÓÌ„ÂÌËÚ‡Î̇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÎËÔÓ‰ËÒÚÓÙËfl. éÅéÅôÖçàÖ ë˙‚ÂÏÂÌÌÓÚÓ ÌË‚Ó Ì‡ ÔÓÁ̇‚‡Ì ̇ çëè ÔÓ͇Á‚‡, ˜Â ÚÓ‚‡  ‰ÌÓ ËÁÍβ˜ËÚÂÎÌÓ ıÂÚÂÓ„ÂÌÌÓ Á‡·ÓÎfl‚‡Ì ‚˙‚ ‚ÒÂÍË Â‰ËÌ ‡ÒÔÂÍÚ ÓÚ Ô‡ÚÓÎÓ„ËflÚ‡ ÒË. 燷≇‚‡ÌÓÚÓ ‡ÁÌÓÓ·‡ÁË ‚ ÍÎËÌ˘ÌËÚ ÙÂÌÓÚËÔÓ‚Â Ó·‡˜Â, Ì ̇ÏË‡ ‰ÌÓÁ̇˜ÌÓ Ò˙ÓÚ‚ÂÚÒÚ‚Ë ‚ ÛÒÚ‡ÌÓ‚ÂÌËÚ „ÂÌÂÚ˘ÌË ÙÓÏË. ë˙‚ÂÏÂÌÌËÚ ÔÓÛ˜‚‡ÌËfl ̇ ÏÂı‡ÌËÁÏËÚ ÎÂʇ˘Ë ‚ ÓÒÌÓ‚‡Ú‡ ̇ Ô‡ÚÓÎӄ˘ÌËfl ÔÓˆÂÒ ÔË çëè Ó˜ÂÚ‡‚‡Ú ÌflÍÓÎÍÓ ËÌÚÂÂÒÌË ‚ ÚÓ‚‡ ÓÚÌÓ¯ÂÌË ÚẨÂ̈ËË. 1. ÑÂÙÂÍÚË ‚ ‡Á΢ÌË „ÂÌË ‚Ó‰flÚ ‰Ó ËÁfl‚‡ ̇ ÒıÓ‰ÂÌ ÍÎËÌ˘ÂÌ ÙÂÌÓÚËÔ (̇Ô. ‰ÂÙÂÍÚË ‚ „ÂÌ ÒÔ‡ÒÚËÌ ÔË SPG4 Ë ‚ „ÂÌ NIPA1 ÔË SPG6 ÙÓÏËÚÂ). ÇÂÓflÚÌÓ „ÂÌËÚ ҂˙Á‡ÌË Ò ËÁfl‚‡Ú‡ ̇ ÚÂÁË ÙÓÏË ÍÓ‰Ë‡Ú ·ÂÎÚ˙˜ÌË ÔÓ‰ÛÍÚË Ì‡ ‰̇ Ë Ò˙˘‡ ÍÎÂÚ˙˜Ì‡ ÙÛÌ͈Ëfl. 2. àÁfl‚‡ ̇ ‡Á΢ÌË ÍÎËÌ˘ÌË ÙÓÏË (˜ËÒÚË Ë ÍÓÏÔÎÂÍÒÌË) ÔË ‰ÂÙÂÍÚË ‚ ‰ËÌ Ë Ò˙˘ „ÂÌ (̇Ô. ÔË SPG4, SPG7, SPG2); ̇΢ˠ̇ ÏÂʉÛÙ‡ÏËÎ̇ Ë ËÌÚ‡Ù‡ÏËÎ̇ ‚‡ˇ·ËÎÌÓÒÚ ‚ ÍÎËÌ˘ÌÓÚÓ ÔÓÚ˘‡Ì ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÇÂÓflÚÌÓ Ò˙˘ÂÒÚ‚Û‚‡Ú ÏÓ‰ËÙˈË‡˘Ë Ù‡ÍÚÓË, ‚Íβ˜ËÚÂÎÌÓ Ë „ÂÌÂÚ˘ÌË (SPG4). 3. åÛÚ‡ˆËË ‚ ‰ËÌ Ë Ò˙˘ „ÂÌ Ò‡ ÓÚ„Ó‚ÓÌË Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ Ôӂ˜ ÓÚ Â‰ÌÓ Á‡·ÓÎfl‚‡ÌÂ. ç‡Î˘ËÂÚÓ Ì‡ ‡ÎÂÎÌË Ì‡ çëè Á‡·ÓÎfl‚‡ÌËfl (ï-Ò‚˙Á‡Ì ıˉÓˆÂÙ‡ÎÛÒ Ë MASA ÒË̉ÓÏ, PMD, PWS Ë AS, dHMN Ë ÍÓÌ„ÂÌËÚ‡Î̇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÎËÔÓ‰ËÒÚÓÙËfl)  ‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ ËÁÍβ˜ËÚÂÎÌÓÚÓ ÏÌÓ„ÓÓ·‡ÁË ‚˙‚ Ù‡ÍÚÓËÚÂ Ë ÏÂı‡ÌËÁÏËÚÂ, ÍÓËÚÓ Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ‚ÒflÍÓ Â‰ÌÓ Á‡·ÓÎfl‚‡ÌÂ. 4. ê‡Á΢ÌË ÏÓÎÂÍÛÎÌË ÏÂı‡ÌËÁÏË ‚Ó‰flÚ ‰Ó ‰ËÌ Ë Ò˙˘ Ô‡ÚÓÎӄ˘ÂÌ ÂÙÂÍÚ Ì‡ ‰Â„ÂÌÂ‡ˆËfl ̇ ÍÓÚËÍÓÒÔË̇ÎÌËÚ Ô˙Úˢ‡. èÓ‡‰Ë ËÁÍβ˜ËÚÂÎÌÓ „ÓÎflχڇ ÒË ‰˙ÎÊË̇ (̇‰ 1Ï), ÍÓÚËÍÓÒÔË̇ÎÌËÚÂ Ë ÒÂÌÁÓÌËÚ Ú‡ÍÚÓ‚Â Ò Ó͇Á‚‡Ú ÓÒÓ·ÂÌÓ ˜Û‚ÒÚ‚ËÚÂÎÌË Í˙Ï Ì‡Û¯ÂÌËfl ‚ ÏÓÎÂÍÛÎÌËfl ÔÂÌÓÒ, Ú‡ÌÒÔÓÚ Ë ÂÌÂ„ËÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï. ÇÂÓflÚÌÓ Ì‡ ÚÓ‚‡ Ò ‰˙ÎÊË Ù‡ÍÚ‡, ˜Â „ÓÎflχ ˜‡ÒÚ ÓÚ Ë‰ÂÌÚËÙˈË‡ÌËÚ ‰Ó ÚÓÁË ÏÓÏÂÌÚ çëè ÓÚ„Ó‚ÓÌË „ÂÌË ÍÓ‰Ë‡Ú ·ÂÎÚ˙˜ÌË ÏÓÎÂÍÛÎË Ëχ˘Ë Íβ˜Ó‚‡ ÓÎfl ‚ ÚÂÁË ÔÓˆÂÒË. ÉÂÌÂÚ˘ÌËÚ ‰ÂÙÂÍÚË ÔË SPG4 Ë SPG10 Á‡Òfl„‡Ú ÏËÍÓÚÛ·ÛÎÌËfl Ú‡ÌÒÔÓÚ, ‰Ó͇ÚÓ ÚÂÁË ÔË SPG3Ä, SPG20 Ë SPG21 Ò‡ Ô˘Ë̇ Á‡ ̇Û¯ÂÌËfl ‚ ẨÓÁÓÏÌËfl Ú‡ÙËÍ. èË SPG7 Ë SPG13 ÏÛÚË‡Ú „ÂÌË ÍÓ‰Ë‡˘Ë ÏËÚÓıÓ̉ˇÎÌË ·ÂÎÚ˙ˆË Ò ¯‡ÔÂÓÌ̇ ÙÛÌ͈Ëfl, ÍÓÂÚÓ ÔÓ͇Á‚‡, ˜Â ˜‡ÒÚ ÓÚ çëè ÙÓÏËÚ ҇ “¯‡ÔÂÓÌÓÔ‡ÚËË”. ÑÛ„‡Ú‡ ÓÒÌӂ̇ „ÛÔ‡ ÏÓÎÂÍÛÎÌË ‰ÂÙÂÍÚË Á‡Òfl„‡Ú „ÂÌË Ëχ˘Ë Íβ˜Ó‚‡ ÓÎfl ‚ ‡Á‚ËÚËÂÚÓ (L1CAM) Ë ÒÚÛÍÚÛ‡Ú‡ (PLP1) ̇ ÍÓÚËÍÓÒÔË̇ÎÌËÚ Ú‡ÍÚÓ‚Â. Ñ‚‡ ÓÚ Ò‚˙Á‡ÌËÚÂ Ò çëè Ô‡ÚÓÎÓ„ËflÚ‡ „ÂÌË, NIPA1 Ë BSCL2, Ò‡ Ò ÌÂËÁ‚ÂÒÚ̇ Á‡ ÏÓÏÂÌÚ‡ ÙÛÌ͈Ëfl. чÎË Ú ˘Â ·˙‰‡Ú Ô˘ËÒÎÂÌË Í˙Ï ÌflÍÓfl ÓÚ ‰ÓÒ„‡ ËÁ‚ÂÒÚÌËÚ ͇Ú„ÓËË ËÎË ˘Â Ô‰ÒÚ‡‚flÚ ÌÓ‚ Ô‡ÚÓ„ÂÌÂÚ˘ÂÌ ÏÂı‡ÌËÁ˙Ï? ä‡Í‚Ó ÌË Ô‰ÒÚÓË ‰‡ ̇ۘËÏ ‡ÁÌˢ‚‡ÈÍË Ô‡ÚÓÎÓ„ËflÚ‡ ̇ çëè? èÖêëèÖäíàÇà Ç˙ÔÂÍË ÌËÒ͇ڇ ÒË ˜ÂÒÚÓÚ‡ Ë Â‰ÌÓÚËÔ̇ ÓÒÌӂ̇ ÍÎËÌ˘̇ ı‡‡ÍÚÂËÒÚË͇ ̇ ‰ÓÎ̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô‡ÂÁ‡, ‰ÌÂÒ Â flÒÌÓ, ˜Â çëè Ô‰ÒÚ‡‚Îfl‚‡ Ô‡‡‰Ë„χ ‚ Ò˙‚ÂÏÂÌ̇ڇ Ì‚Ó̇Û͇. èÓÛ˜‚‡ÌÂÚÓ Ì‡ ÏÂı‡ÌËÁÏËÚ ̇ çëè ˘Â ËÁflÒÌË Ì ҇ÏÓ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ÏÛ, ÌÓ ˘Â ‰‡‰Â ËÌÙÓχˆËfl Á‡ ÌÓχÎ̇ڇ ÙÛÌ͈Ëfl ̇ ÏÌÓ„Ó ÓÚ “Û˜‡ÒÚÌˈËÚ” ‚ Ô‡ÚÓÎӄ˘ÌËfl ÔÓˆÂÒ Ë ˘Â ÔÓÒÎÛÊË Í‡ÚÓ ÏÓ‰ÂÎ Á‡ ËÁÛ˜‡‚‡ÌÂÚÓ Ì‡ ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ãàíÖêÄíìêÄ 1. Behan W, Maia M. Strumpell’s familial spastic paraplegia: genetics and neutopahy. J Neurol Neurosurg Psychiatry 37, 1974, 8-20. 2. Casari G, DeFusco M, Ciarmatori S et al. Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear encoded mitochondrial metalloprotease. Cell 93, 1998, 973-983. 3. Casari, G, Rugarli, E. Molecular basis of inherited spastic paraplegias. Cur Op Genet Dev 11, 2001, 336-342. 4. Castellani V, Chedotal A, Schachner M, Faivre Sarailh C, Rougon G. Analysis of the L1-deficient mouse phenotype reveals cross-talk between Sema3A and L1 signaling pathways in axonal guidance. Neuron 27, 2000, 237-249. 5. Ciccarelli F, Proukakis C, Patel H, Cross H, Azam S, PattonM, Bork P, Crosby A. The identification of a conserved domain in both spartin and spastin, mutated in hereditary spastic paraplegia. Genomics 81, 2003, 437-441. 6. Cross HE, McKusick VA. The Mast syndrome: a recessively inherited form of presenile dementia with motor disturbances. Arch Neurol 16, 1967, 1-13. 7. Durr A, Brice A. Genetics of movement disorders. Review. Curr Opin Neurol. Aug 9, 1996, 4, 290-7. 8. Errico A, Ballabio A, Rugarli EI. Spastin, the protein mutated in autosomal dominant hereditary spastic paraplegia, is involved in microtubule dynamics. Hum Mol Genet 11, 2002, 2, 153-163. 9. Ferreirinha N, Quattrini A, Valsecchi V, Errico A, Balabio A, Rugarli EI. Mice lacking paraplegin, a mitochondrial protease involved in hereditary spastic paraplegia, show axonal degeneration and abnormal mitochondria. Mitochondrion 1, 2001, S21-S22. 10. Fink JK, Wu C, Jones SM et al. Autosomal dominant familial spastic paraplegia: tight linkage to chromosome 15q. Am J Hum Genet 56, 1995, 183-7. 11. Fink JK, Rainier S. Hereditary spastic paraplegia: spastin phenotype and function. Review. Arch Neurol Jun 61, 2004, 6, 830-3. 12. Fortini D, Cricchi F, DiFabio R, Damiano M, Commanducci G, Benedetti L, Valoppi M, Grieco GS, D’Eugenio O, Celato A, Santorelli F, Casali C, Amabile GA, Pierelli F. Current insights into familial spastic paraparesis: new advances in an old disease. Functional Neurol 18, 2003, 1, 43-49. 13. Goldstein LSB. Kinesin molecular motors: transport pathways, receptors and human disease. Procl Natl Acad Sci USA 98, 2001, 6999-7003. 14. Hansen JJ, Durr A, Cournu-Rebeix I, Georgopoulos C, Ang D, Nielsen MN, Davoine CS, Brice A, Fontaine B, Gregersen N, Bross P. Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60. Am J Hum Genet 70, 2002, 1328-32. 15. Harding AE. Hereditary pure spastic paraplegia: a clinical and genetic study of 22 families. J Neurol Neurosurg psychiatry 44, 1981, 871-83. 16. Hazan J, Fonknechten N, Mavel D, et al. Spastin, a novel AAA protein, is altered in the most frequent form of autosomal dominant spastic paraplegia. Nat Genet 23, 1999, 296-303. 17. Kanai Y, Okada Y, Tanaka Y, Harada A, Terada S, Hirokawa N. KIF5C, a novel neuronal kinesin enriched in motor neurons. J Neurosci 20, 2000, 6374-84. 18. Leonhard K, Hermann JM, Stuart RA, Mannhaupt G, Neupert W, Langer, T. AAA proteases with catalytic sites on opposite membrane surface comprise a proteolytic system for the ATP-dependent degradation of inner membrane proteins in mitochondria. EMBO J 15, 1996, 4218-29. 19. Lorrain M. Contribution a l’etude de la paraplegie spasmodique familiale. Paris: Steinheil, 1898. 20. McDermott C, Grierson A, Wood JD, Bingley M, Wharton S, Bushby K, Shaw PJ. Hereditary spastic paraplegias: Disrupted intracellular transport associated with spastin mutation. Ann Neurol 54, 2003, 748-59. 21. McDermott, C, White, K, Bushby, K, Shaw, P. Hereditary spastic paraparesis: a review of new developments. J Neurol Neurosurg psychiatry 69, 2000, 150160. 22. Molon A, DiGiovanni S, Chen YW, Clarkson PM, Angelini C, Pegoraro E, Hoffman EP. Large-scale disruption of microtubule pathways in morphologically normal human spastin muscle. Neurol 62, 2004, 1097-1104. 23. Nielsen KL, Cowan NJ. A single ring is sufficient for productive chaperoninmediated folding in vivo. Mol Cell 2, 1998, 93-99. 24. Patel H, Cross H, Proukakis C, Hershberger R, Bork P, Ciccarelli FD, Patton

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MA, McKusick VA, Crosby AH. SPG20 is mutated in Troyer syndrome, a hereditary spastic paraplegia. Nat Genet 31, 2002, 347-8. 25. Patel H, Hart PE, Warner TT, Houlston RS, Patton MA, Jeffery S, Crosby AH. The Silver syndrome variant of HSP maps to chromosome 11q12-q14, with evidence for genetic heterogeneity with this subtype. Am J Hum Genet 69, 2001, 209-15. 26. Rainier S, Chai J-H, Tokarz D, Nichols RD, Fink J. NIPA1 gene mutations cause autosomal dominant hereditary spastic paraplegia (SPG6). Am J Hum Genet 73, 2003, 967-971. 27. Reid E. Science in motion: common molecular pathological themes emerge in the hereditary spastic paraplegias. J Med Genet 40, 2003, 81-86. 28. Reid E, Kloos M, Ashley-Koch A, Hughes L, Bevan S, Svenson IK,Lennon Graham F, Gaskell PC, Dearlove A, Pericak-Vance MA, Rubinsztein DC, Marchuk DA. A kinesin heavy chain (KIF5A) mutation in hereditary spastic paraplegia (SPG10). Am J Hum Genet 71, 2002, 1189-94. 29. Saugier-Veber P, Munnich A, Bonneau D, Rozet JM, LeMerrer M, Gil R, Boespflug-Tanguy O. X-linked spastic paraplegia and PMD disease are allelic disorders at the proteolipid protein locus. Nat Genet 6, 1994, 257-262. 30. Schwartz GA, Liu C-N. Hereditary (familial) spastic paraplegia: further clinical and pathological observations. Arch Neurol Psychiatry 75, 1956, 144-162. 31. Seeligmuller A. Sklerose der Seitenstrange des Ruckenmarks bei vier Kinderen derselben Familie. Dtsch Med Wschr 2, 1876, 185 - 186. 32. Simpson MA, Cross H, Proukakis C, Pryde A, Hershberger R, Chatonnet A, Patton MA, Crosby AH. Maspardin is mutated in Mast syndrome, a complicated form of hereditary spastic paraplegia associated with dementia. Am J Hum Genet 73, 2003, 1147-1156. 33. Strumpell A. Beitrage zur pathologie des ruckenmarks. Archiv fur Psychiatrie und Nervenkrankheiten 10, 1880, 676 - 717. 34. Strumpell A. Uber die hereditare spastiche spinalparalyse. Deutsche Zeitschrift fur Nervenfeilkunde 4, 1893, 173 – 88. 35. Svenson I, Kloos M, Gaskel P, Nance M, Garbern J, Hisanaga S, PericakVance M, Ashley-Koch A, Marchuk D. Intragenic modifiers of hereditary spastic paraplegia due to spastin gene mutations. Neurogenetics 5, 2004, 157-164. 36. Windpassinger C, Auer-Grumbach M, Irobi J, Patel H, Petek E, Horl G, Malli R, Reed JA, Dierick I, Verpoorten N, Warner TT, Proukakis C, Van den Bergh P, Verellen C, Van Maldergem L, Merlini L, De Jonghe P, Timmerman V, Crosby AH, Wagner K. Heterozygous missense mutations in BSCL2 are associated with distal hereditary motor neuropathy and Silver syndrome. Nature Genet 36, 2004, 271-276. 37. Yool DA, Edgar JM, Montague P, Malcolm S. The proteolipid protein gene and myelin disorders in man and animal models. Hum Mol Genet 9, 2000, 987992. 38. Zhao X, Alvarado D, Rainer S, Lemons R, Hedera P, Weber CH, Tukel T, Apak M, Heiman-Patterson T, Ming L, Bui M, Fink JK. Mutations in newly identified GTP-ase gene cause autosomal dominant hereditary spastic paraplegia. Nat Genet 29, 2001, 326-331. 39. Zhu PP, Patterson A, Lavoie B, Stadler J, Shoeb M, Patel R, Blackstone C. Cellular localization, oligomerization, and membrane association of the SPG3A protein Atlastin. J Biol Chem 278, 2003, 49, 49063-71.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ç‚fl̇ à‚‡ÌÓ‚‡ ㇷÓ‡ÚÓËfl ÔÓ åÓÎÂÍÛÎ̇ Ô‡ÚÓÎÓ„Ëfl ëÅÄãÄÉ”å‡È˜ËÌ ‰ÓÏ” ÛÎ.”á‰‡‚” 2 1431 ëÓÙËfl ÚÂÎ.: 9172 473 Â-mail: nevianai@hotmail.com


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é·ÁÓ ëöÇêÖåÖççà ÄëèÖäíà çÄ çÄëãÖÑëíÇÖçÄíÄ ëèÄëíàóçÄ èÄêÄèãÖÉàü II. äãàçàäÄ, ÑàÄÉçéëíàäÄ à çÖÇêéèÄíéãéÉàü Ä. Ä̉‚‡1, à. í˙Ì‚2, à. ãËÚ‚ËÌÂÌÍÓ3 Ë ç. à‚‡ÌÓ‚‡4,5

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ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, ëÓÙËfl 3 ëÅÄãÑÅ 4 ㇷÓ‡ÚÓËfl ÔÓ ÏÓÎÂÍÛÎfl̇ Ô‡ÚÓÎÓ„Ëfl, ëÅÄãÄÉ “å‡È˜ËÌ ‰ÓÏ” 5 ä‡Ú‰‡ ÔÓ ıËÏËfl Ë ·ËÓıËÏËfl, è‰ÍÎËÌ˘ÂÌ ÛÌË‚ÂÒËÚÂÚÒÍË ˆÂÌÚ˙, åì- ëÓÙËfl SUMMARY NEW ASPECTS IN HEREDITARY SPASTIC PARAPLEGIA. II. CLINICAL FEATURES, DIAGNOSTIC CRITERIA AND NEUROPATHOLOGY A. Andreeva, I. Turnev, I. Litvinenko, N. Ivanova Hereditary spastic paraplegia (HSP) is a group of disorders characterized by a great clinical and genetical heterogenity. HSP is classified into two forms: “pure” and “complicated”. The age of symptom onset may be as early as infancy or as late as eighth decade of life. The main clinical features of “pure” forms are slowly progressive spastic weakness in lower limbs, urinary bladder disturbances and mild diminution of vibration and position sensation. HSP is classified as “complicated” if the impearment present in pure forms is accompanied by other neurological findings such as dementia, amyotrophy, peripheral neuropathy, optic atrophy, seizures or extrapyramidal disturbance. HSP may be transmitted in an autosomal dominant, an autosomal recessive, or an X-linked manner. About 23 forms have been described till now – 10 autosomal dominant, 10 autosomal recessive and 3 X-linked. The aim of this review is to summarize the advances in understanding of the clinical features, diagnostics and neuropathology in HSP. Key words: Hereditary spastic paraplegia, pure forms, complicated forms, clinical features, diagnostic criteria, neuropathology. êÖáûåÖ ç‡ÒΉÒÚ‚ÂÌËÚ ÒÔ‡ÒÚ˘ÌË Ô‡‡Ô΄ËË (çëè) Ò‡ „ÛÔ‡ Á‡·ÓÎfl‚‡ÌËfl, ı‡‡ÍÚÂËÁË‡˘Ë ÒÂ Ò „ÓÎflχ „ÂÌÂÚ˘̇ Ë ÍÎËÌ˘̇ ıÂÚÂÓ„ÂÌÌÓÒÚ. ä·ÒËÙˈË‡Ú Ò ͇ÚÓ ˜ËÒÚË Ë ÍÓÏÔÎˈË‡ÌË ÙÓÏË. è˙‚ËÚ ÒËÏÔÚÓÏË ÏÓ„‡Ú ‰‡ Ò ÔÓfl‚flÚ ‚˙‚ ‚Òfl͇ ‚˙Á‡ÒÚ – ÓÚ ‰ÂÚÒ͇ ‰Ó 80 „Ó‰ËÌË. éÒÌÓ‚ÌË ·ÂÎÂÁË Ì‡ ˜ËÒÚËÚ ÙÓÏË Ò‡ ÔÓÒÚÂÔÂÌÌÓ ÔÓ„ÂÒË‡˘‡Ú‡ ÒÔ‡ÒÚ˘ÌÓÒÚ ‚ ‰ÓÎÌËÚ Í‡ÈÌˈË, Ú‡ÁÓ‚ÓÂÁÂ‚Ó‡ÌËÚ ‡ÁÒÚÓÈÒÚ‚‡ Ë ‰ËÒÍÂÚÌËÚ ÒÏÛ˘ÂÌËfl ‚˙‚ ‚Ë·‡ˆËÓÌÌËfl Ë ÔÓÁˈËÓÌÂÌ ÛÒÂÚ. ç‡ÒΉÒÚ‚ÂÌËÚ ÒÔ‡ÒÚ˘ÌË Ô‡‡Ô΄ËË Ò ÓÔ‰ÂÎflÚ Í‡ÚÓ ÍÓÏÔÎˈË‡ÌË ÔË Ì‡Î˘Ë ̇ ‰ÓÔ˙ÎÌËÚÂÎÌË Ì‚ÓÎӄ˘ÌË ·ÂÎÂÁË Í‡ÚÓ ‰ÂÏÂ̈Ëfl, ‡ÏËÓÚÓÙËfl, ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl, ÓÔÚ˘̇ ‡ÚÓÙËfl, ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË ËÎË ÂÍÒÚ‡ÔË‡ÏˉÌË ÒÏÛ˘ÂÌËfl. 燘ËÌ˙Ú Ì‡ Û̇ÒΉfl‚‡Ì ÏÓÊ ‰‡ ·˙‰Â ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚÂÌ, ‡‚ÚÓÁÓÏÌÓ ˆÂÒË‚ÂÌ Ë ï-Ò‚˙Á‡Ì. ÑÓ ÏÓÏÂÌÚ‡ Ò‡ ÓÔËÒ‡ÌË ÓÍÓÎÓ 23 ‡Á΢ÌË ÙÓÏË – 10 ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚÌË, 10 ‡‚ÚÓÁÓÏÌÓ ˆÂÒË‚ÌË Ë 3 ïÒ‚˙Á‡ÌËÚ çëè. ñÂÎÚ‡ ̇ ÚÓÁË Ó·ÁÓ  ‰‡ Ó˜Âڇ ÌÓ‚ÓÒÚËÚ ‚ ‡Á·Ë‡ÌÂÚÓ Ì‡ ÍÎËÌË͇ڇ, ‰Ë‡„ÌÓÒÚË͇ڇ Ë ÌÂÓÔ‡ÚÓÎÓ„ËflÚ‡ ÔË çëè. äβ˜Ó‚Ë ‰ÛÏË: ̇ÒΉÒÚ‚ÂÌË ÒÔ‡ÒÚ˘ÌË Ô‡‡Ô΄ËË, ˜ËÒÚË ÙÓÏË, ÍÓÏÔÎˈË‡ÌË ÙÓÏË, ÍÎËÌË͇, ‰Ë‡„ÌÓÒÚË͇, Ì‚ÓÔ‡ÚÓÎÓ„Ëfl.

àëíéêàóÖëäà èêÖÉãÖÑ èÂÁ 1880 „. Strumpell ÓÔËÒ‚‡ Á‡ Ô˙‚Ë Ô˙Ú Á‡·ÓÎfl‚‡ÌÂÚÓ. êratt ÓÔ‰ÂÎfl ÒÎÛ˜‡ËÚÂ Ò ‰ÓÔ˙ÎÌËÚÂÎÌË Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË, ËÁÎËÁ‡˘Ë ËÁ‚˙Ì ‡ÏÍËÚ ̇ ÓË„Ë̇ÎÌÓÚÓ ÓÔËÒ‡ÌË ̇ Strumpell, ͇ÚÓ çëè (+) ÒË̉ÓÏË. Harding Ô‰·„‡ Í·ÒËÙˈË‡ÌÂÚÓ Ì‡ çëè ̇ ˜ËÒÚË Ë ÍÓÏÔÎˈË‡ÌË ÙÓÏË, ͇ÚÓ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ÔÓ‰‡Á‰ÂÎfl ˜ËÒÚËÚ ÙÓÏË ÒÔÓ‰ ‚˙Á‡ÒÚÚ‡ ̇ ‰Â·˛Ú‡ ̇ çëè Ò Ì‡˜‡ÎÓ Ì‡ ÓÔ·͂‡ÌËflÚ‡ ÔÂ‰Ë 35 „. (I ÚËÔ) Ë Ì‡ ïëè ÒΉ 35 „. (II ÚËÔ). í‡ÁË Í·ÒËÙË͇ˆËfl Ì  Ò˙‚˙¯Â̇ Ë ÏÌÓ„Ó ÓÚ Ù‡ÏËÎËËÚ Ì ÏÓ„‡Ú ‰‡ Ò ÓÚÌÂÒ‡Ú Í˙Ï ÌËÚÓ Â‰Ì‡ ÓÚ ÔÓÒÓ˜ÂÌËÚ „ÛÔË. Ç˙‚Âʉ‡ÌÂÚÓ Ì‡ ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌËÚ ËÁÒΉ‚‡ÌËfl ÔË ËÁÛ˜‡‚‡ÌÂÚÓ Ì‡ çëè ‰‡‚‡ ‚˙ÁÏÓÊÌÓÒÚ ‰‡ Ò ‚Íβ˜‡Ú Ì ҇ÏÓ ÍÎËÌ˘ÌË, ÌÓ Ë „ÂÌÂÚ˘ÌË ‰‡ÌÌË ‚ Ò˙‚ÂÏÂÌ̇ڇ Í·ÒËÙË͇ˆËfl ̇ çëè. ÑàÄÉçéëíàóçà äêàíÖêàà Ä/ óËÒÚË ÙÓÏË Ì‡ çëè éÒÌÓ‚ÂÌ ÔËÁÌ‡Í Ì‡ ˜ËÒÚËÚ ÙÓÏË Ì‡ çëè  ‡Á‚ËÚËÂÚÓ Ì‡ ÒÔ‡ÒÚ˘ÌÓÒÚ ‚ ‰ÓÎÌËÚ Í‡ÈÌˈË. ë ̇Ô‰‚‡Ì ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Í˙Ï ÔÓÒÚÂÔÂÌÌÓ ÔÓ„ÂÒË‡˘‡Ú‡ ‰ÓÎ̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô‡ÂÁ‡ Ò ‰Ó·‡‚flÚ Ú‡ÁÓ‚ÓÂÁÂ‚Ó‡ÌË ÒÏÛ˘ÂÌËfl Ë Ì‡Ï‡ÎÂÌË ‚Ë·‡ˆËÓÌÂÌ Ë ÔÓÁˈËÓÌÂÌ ÛÒÂÚ. ï‡‡ÍÚÂÌÓ Â Ì‡Î˘ËÂÚÓ Ì‡ ÒËÏÂÚËfl - ‰‚‡Ú‡ ‰ÓÎÌË Í‡ÈÌË͇ Ò Á‡Òfl„‡Ú ÔÓ˜ÚË Â‰ÌÓ‚ÂÏÂÌÌÓ Ë ‚ ‰̇͂‡ ÒÚÂÔÂÌ. ÑÛ„‡ ÚËÔ˘̇ ÓÒÓ·ÂÌÓÒÚ Â ÌÂÒ˙ÓÚ‚ÂÚÒÚ‚ËÂÚÓ ÏÂÊ‰Û ‰Ó· ËÁfl‚Â̇ڇ ÒÔ‡ÒÚ˘ÌÓÒÚ Ë ÎÂÍÓ ÔÓfl‚Â̇ڇ ËÎË ‰ÓË ÎËÔÒ‚‡˘‡ Ò··ÓÒÚ ‚ ÏÛÒÍÛÎËÚ ̇ ‰ÓÎÌË Í‡ÈÌËˆË (10). äËÚÂËËÚÂ, Ô‰ÎÓÊÂÌË Á‡ ‰Ë‡„ÌÓÒÚË͇ ̇ ˜ËÒÚËÚ ÙÓÏË Ò‡: I. ᇉ˙ÎÊËÚÂÎÌË: î‡ÏËÎÌÓÒÚ, ÔÓ„ÂÒË‚ÌÓ ÒÏÛ˘ÂÌË ̇ ÔÓıӉ͇ڇ, ÒÔ‡ÒÚ˘ÌÓÒÚ ‚ ‰ÓÎÌË Í‡ÈÌˈË, ıËÔÂÂÙÎÂÍÒËfl ‚ ‰ÓÎÌË Í‡ÈÌˈË, ÂÍÒÚÂÌÁÓÌË Ô·ÌÚ‡ÌË ÓÚ„Ó‚ÓË; II. óÂÒÚË: è‡ÂÁ‡ ‚ ‰ÓÎÌË Í‡ÈÌˈË, íê ‡ÁÒÚÓÈÒÚ‚‡, ÎÂ͇ Á‡‰ÌÓÒÚ˙ηˆÓ‚‡ ÒËÏÔÚÓχÚË͇, ÔÂÒ Í‡‚ÛÒ, ıËÔÂÂÙÎÂÍÒËfl ‚ „ÓÌË Í‡ÈÌˈË, ÎÂ͇ ‰ËÒÏÂÚËfl, Á‡„Û·‡ ̇ ÄıËÎÓ‚ËÚ ÂÙÎÂÍÒË; III. ê‰ÍË: è‡ÂÁ‡ ‚ „ÓÌË Í‡ÈÌˈË, ‰ËÒÚ‡Î̇ ‡ÏËÓÚÓÙËfl (10); Å/äÓÏÔÎˈË‡ÌË ÙÓÏË Ì‡ çëè èË ÍÓÏÔÎˈË‡ÌËÚ ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÒÔ‡ÒÚ˘̇ڇ Ô‡‡Ô‡ÂÁ‡  ҇ÏÓ Â‰ËÌ ÓÚ ÍÓÏÔÓÌÂÌÚËÚ ̇ ‰ËÌ ÏÌÓ„Ó ÔÓ-‡ÁÌÓÓ·‡ÁÂÌ ÙÂÌÓÚËÔ. ÄÒÓˆËË‡ÌË ÔËÁ̇ˆË ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÂÔËÎÂÔÒËflÚ‡ Ò ÔÓÒÚË Ë ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, ÏËÓÍÎÓÌ˘ÌË, „ÂÌÂ‡ÎËÁË‡ÌË Ë ‡·Ò‡ÌÒÌË ÔËÒÚ˙ÔË. ífl Ò Ò¢‡ ÔË çëè Ò ÄÑ Ë Äê ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. ÄÏËÓÚÓÙËflÚ‡  ı‡‡ÍÚÂ̇ ˜ÂÚ‡ Á‡ ÒË̉Óχ ̇ Troyer (ÒÔ‡ÒÚ˘̇ Í‚‡‰ËÔ‡ÂÁ‡, ÔÒ‚‰Ó·Ûη‡̇ Ô‡‡ÎËÁ‡, ıÓÂÓ‡ÚÂÚÓÁ‡ Ë ‰ËÒÚ‡Î̇ ‡ÏËÓÚÓÙËfl) Ë Ì‡ ‡‚ÚÓÁÓÏÌÓ ˆÂÒ˂̇ڇ ÒÔ‡ÒÚ˘̇ ‡Ú‡ÍÒËfl ̇ Charlevoix-Saguenay (ARSACS), ̇·Î˛‰‡‚‡Ì‡ ÔË ÙÂÌÒ-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÍÓ-͇̇‰ÒÍË Ô‡ˆËÂÌÚË ÓÚ Ò‚ÂÓËÁÚÓ˜ÂÌ ä‚·ÂÍ. äÎËÌ˘ÌÓ ARSACS Ò ı‡‡ÍÚÂËÁË‡ Ò˙Ò ÒÔ‡ÒÚ˘̇ ‡Ú‡ÍÒËfl, ‰ËÁ‡ÚËfl, ÌËÒÚ‡„˙Ï, ‡ÍÒÓ̇Î̇ Ì‚ÓÔ‡ÚËfl Ë ıËÔÂÏËÂÎËÌËÁ‡ˆËfl ̇ ‚·Í̇ڇ ̇ ÂÚË̇ÎÌËfl ÌÂ‚. êÂÁÛÎÚ‡Ú Â ÓÚ ÏÛÚ‡ˆËfl ‚ ·ÂÎÚ˙Í, ̇˜ÂÌ Ò‡ÍÒËÌ. ÄÏËÓÚÓÙËfl ̇ χÎÍËÚ ÏÛÒÍÛÎË Ì‡ ˙ˆÂÚÂ Ë Á̇˜ËÚÂÎÌÓ ÔÓ-fl‰ÍÓ Ì‡ ÒÚ˙ԇ·ڇ ‚ Ò˙˜ÂÚ‡ÌË Ò˙Ò ÒÔ‡ÒÚ˘̇ Ô‡‡Ô‡ÂÁ‡ Ò ̇·Î˛‰‡‚‡ Ë ÔË ÒË̉Óχ ̇ Silver. ëË̉ÓÏ˙Ú Ò ı‡‡ÍÚÂËÁË‡ Ò „ÓÎflχ ÙÂÌÓÚËÔ̇ ‚‡ˇ·ËÎÌÓÒÚ. çÂÁ‡‚ËÒËÏÓ, ˜Â ̇È-ÓÚ΢ËÚÂÎ̇ڇ ˜ÂÚ‡  ÓÚÒ··‚‡ÌÂÚÓ Ì‡ χÎÍËÚ ÏÛÒÍÛÎË Ì‡ ˙ˆÂÚÂ, ÔË ÌflÍÓË ËÌ‰Ë‚Ë‰Ë ÏÓÊ ‰‡ Ëχ Ò‡ÏÓ ÒÏÛ˘ÂÌËfl ‚ ÔÓıӉ͇ڇ, ‰ÂÙÓχˆËË Ì‡ ÒÚ˙ԇ·ڇ ËÎË ‰‡ Ò‡ ‡ÒËÏÔÚÓÏÌË ÌÓÒËÚÂÎË. 燘‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ‚ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ ËÎË ÔÂÁ Ô˙‚ËÚ „Ó‰ËÌË Ì‡ ‚ÚÓ‡Ú‡ ‰Â͇‰‡ (18). éÔËÒ‡ÌË Ò‡ ÍÓÏÔÎˈË‡ÌË ÙÓÏË Ì‡ çëè ‚ ‡ÒӈˇˆËfl Ò ÂÍÒÚ‡ÔË‡ÏˉÌË ·ÂÎÂÁË Í‡ÚÓ ıÓÂÓ‡ÚÂÚÓÁ‡, ‰ËÒÚÓÌËfl Ë ˄ˉÌÓÒÚ. ëÂÌÁÓ̇ڇ Ì‚ÓÔ‡ÚËfl ÏÓÊ ‰‡ ·˙‰Â ‡ÒËÏÔÚÓÏ̇, ‰‡ Ò ÔÓfl‚Ë ‚ ‰ÂÚÒ͇ ËÎË Áfl· ‚˙Á‡ÒÚ. íÂÊÍËÚ ÙÓÏË Ò ı‡‡ÍÚÂËÁË‡Ú Ò ÚÓÙ˘ÌË ÍÓÊÌË ÔÓÏÂÌË Ë flÁ‚Ë ÔÓ ÒÚ˙ԇ·ڇ. ë˜ËÚ‡ ÒÂ, ˜Â Á‡ ÙÂÌÓÚËÔ‡ ̇ ÍÓÏÔÎˈË‡ÌËÚ ÙÓÏË Â ı‡‡ÍÚÂ̇ ‰ÂÏÂ̈Ëfl Ò ·ÂÎÂÁË Ì‡ ÒÛ·ÍÓÚË͇Î̇ (10). 燷≇‚‡ÌË Ò‡ Ë Ò˙˜ÂÚ‡ÌËfl Ò˙Ò Ò˙‰Â˜ÌË ‰ÂÙÂÍÚË, „ÎÛıÓÚ‡, ËıÚËÓÁ‡ Ë ˆÂ·Â·ÌË ·ÂÎÂÁË. çÖÇêéèÄíéãéÉàü éÒÌӂ̇ ̇ıӉ͇ ÔË ‚Ò˘ÍË ÙÓÏË Ì‡ Hëè  ‡ÍÒÓ̇Î̇ڇ ‰Â„ÂÌÂ‡ˆËfl ̇ ‰ÂÒˆẨË‡˘ËÚ ÍÓÚËÍÓÒÔË̇ÎÌË Ô˙Úˢ‡, χÍÒËχÎÌÓ ËÁfl‚Â̇ ‚ ÚÂÏË̇ÎÌËÚ ËÏ ˜‡ÒÚË. éÚ Ò˙˘Ëfl ÔÓˆÂÒ Ò‡ Á‡Ò„̇ÚË Ë ‚·Í̇ڇ ̇ Ù‡ÒˆËÍÛÎÛÒ „‡ˆËÎËÒ ÓÚ ‰ÓÎÌËÚ Í‡ÈÌˈË. ÄÍÒÓ̇ÎÌËÚ Á‡„Û·Ë ÏÓ„‡Ú ‰‡ Ò Ò˙Ô˙ÚÒÚ‚‡Ú ÓÚ ‰ÂÏËÂÎËÌËÁ‡ˆËfl Ë „ÎËÓÁ‡. Ç 50% ÓÚ ÒÎÛ˜‡ËÚ Ò ÓÔËÒ‚‡ Ë ‚˙‚΢‡Ì ̇ ÒÔËÌÓˆÂ·Â·ÌËÚ Ô˙Úˢ‡. ë‰ flÔÓÌÒÍË, ‡ÏÂË͇ÌÒÍË Ë ËڇΡÌÒÍË Ù‡ÏËÎËË Ò‡ ÓÔËÒ‡ÌË ˆÂÒË‚ÌË ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò ËÁÚ˙Ìfl‚‡Ì ̇ ÍÓÔÛÒ Í‡ÎÓÁÛÏ (3,12). Ç 83% ÓÚ ÚÂÁË ·ÓÎÌË Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔÂË‚ÂÌÚËÍÛ·ÌË ÎÂÁËË ‚ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó, ‡ ÙÓÌÚ‡Î̇ڇ ÍÓÓ‚‡ ‡ÚÓÙËfl Ò ̇·Î˛‰‡‚‡ ‚ ̇Ô‰̇ÎËÚ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÑÛ„‡ ̇ıӉ͇  ̇χÎfl‚‡Ì ·Ófl ̇ ÍÎÂÚÍËÚ ̇ ň ‚ 5-ÚË ÒÎÓÈ Ì‡ ÏÓÚÓ̇ڇ ÍÓ‡ (10). àÌÚ„ËÚÂÚ˙Ú Ì‡ ÏÓÚÓ̇ڇ ÍÓ‡  ËÁÒΉ‚‡Ì ˜ÂÁ Ú‡ÌÒÍ‡ÌˇÎ̇ χ„ÌËÚ̇ ÒÚËÏÛ·ˆËfl. ìÒÚ‡ÌÓ‚fl‚‡ Ò ۉ˙ÎÊÂÌÓ ˆÂÌÚ‡ÎÌÓ ÔÓ‚Ó‰ÌÓ ‚ÂÏÂ Ë ‡Á΢ÌË ‰Û„Ë ‡·ÌÓÏÌÓÒÚË Í‡ÚÓ Ì‡Ï‡ÎÂÌË ‡ÏÔÎËÚÛ‰‡ Ë ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ, ͇ÍÚÓ Ë ÔÓÎËÙ‡ÁÌÓÒÚ Ì‡ ÔÓÚÂ̈ˇÎÚÂ. èÓ-fl‰ÍÓ Ò ̇·Î˛‰‡‚‡ Ì‚Ó̇Î̇ Á‡„Û·‡ ‚ ÍÓÎÓ̇ڇ ̇ ä·Í. èË Í‡˜ÂÒÚ‚ÂÌË ËÁÒΉ‚‡ÌËfl  ÛÒÚ‡ÌÓ‚ÂÌÓ Á̇˜ËÚÂÎÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ Ó·Âχ ̇ ÔË‡ÏˉËÚÂ Ë ‰Û͈Ëfl ̇ Ó·˘Ëfl ·ÓÈ ÏËÂÎËÌËÁË‡ÌË ‚·Í̇ (10). çÂÁ‡‚ËÒËÏÓ ÓÚ Ó·Ë˜‡ÈÌÓ ÌÓχÎ̇ڇ åê ̇ıӉ͇ ÔË ËÁÒΉ‚‡Ì ̇ „·‚ÂÌ Ë „˙·Ì‡˜ÂÌ ÏÓÁ˙Í ÔË Ô‡ˆËÂÌÚË Ò ˜ËÒÚ‡ ÙÓχ, ÚÓ Ò˙˘ÂÒÚ‚Û‚‡Ú Ò˙Ó·˘ÂÌËfl Á‡ ‡ÚÓÙËfl ̇ ÚÓ‡Í‡ÎÌËfl „˙·Ì‡Í, ÍÓÈÚÓ Â Ò Ì‡Ï‡ÎÂÌ Ì‡Ô˜ÂÌ ‰Ë‡ÏÂÚ˙ ÔË ÌflÍÓË ·ÓÎÌË. ç‡ ·‡Á‡Ú‡ ̇ ‚Ò˘ÍË ÚÂÁË ‰‡ÌÌË Ò ҘËÚ‡, ˜Â çëè Ô‰ÒÚ‡‚Îfl‚‡ “dying back” ‡ÍÒÓÌÓÔ‡ÚËfl, ͇ÚÓ ‰Â„ÂÌÂ‡ˆËflÚ‡ Á‡ÔÓ˜‚‡ ‰ËÒÚ‡ÎÌÓ Ë ÔÓ„ÂÒË‡ Í˙Ï ÍÎÂÚ˙˜ÌÓÚÓ ÚflÎÓ (1,16). í·ڇ ̇ ÏÓÚÓÌ‚ÓÌËÚ ҇ Ò˙ı‡ÌÂÌË Ë ÎËÔÒ‚‡Ú ‰‡ÌÌË Á‡ Ô˙‚˘̇ ‰ÂÏËÂÎËÌËÁ‡ˆËfl. èÄíéÉÖçÖáÄ ç‡ÚÛÔ‡ÌËflÚ ÓÔËÚ ÔÓ͇Á‚‡, ˜Â Ôӂ˜ÂÚÓ ‰ÓÏË̇ÌÚÌÓ Û̇ÒΉfl‚‡ÌË Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl Ò‡ ÂÁÛÎÚ‡Ú Ì‡ “ÚÓÍÒ˘ÌÓÒÚ” ÓÚ ‡·ÌÓÏÌË ÔÓÚÂËÌË, ÔÓËÁ‚Âʉ‡ÌË ÓÚ ÏÛÚ‡ÌÚÌË „ÂÌË. ë˜ËÚ‡ ÒÂ, ˜Â ‰ÂÙÂÍÚÌËÚ Ú‡ÌÒÔÓÚÌË ÒËÒÚÂÏË ÏÓ„‡Ú ‰‡ Ò ÓÍ‡Ê‡Ú Ó·˘ ÏÂı‡ÌËÁ˙Ï Á‡ ‡Á‚ËÚË-

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ÂÚÓ Ì‡ ÌflÍÓÎÍÓ ÙÓÏË Ì‡ çëè. éÒÓ·ÂÌ ËÌÚÂÂÒ Ô‰ÒÚ‡‚Îfl‚‡ Ù‡ÍÚ˙Ú, ˜Â ‡ÍÒÓ̇Î̇ڇ ‰Â„ÂÌÂ‡ˆËfl  χÍÒËχÎÌÓ ËÁfl‚Â̇ ‚ ‰˙΄ËÚ Ô˙Úˢ‡. èÓ‡‰Ë ‰˙ÎÊË̇ڇ ̇ Ò‚ÓËÚ ‡ÍÒÓÌË Ì‚ÓÌËÚ ҇ Á‡‚ËÒËÏË ÓÚ Â‰Ì‡ ÔÂÙÂÍÚÌÓ ÙÛÌ͈ËÓÌË‡˘‡ Ú‡ÌÒÔÓÚ̇ ÒËÒÚÂχ, ÓÒË„Ûfl‚‡˘‡ Ô‡‚ËÎÌÓ ‡ÁÔ‰ÂÎÂÌË ̇ ÔÂ̇ÒflÌËÚ ‚¢ÂÒÚ‚‡ ‰Ó ÍÎÂÚ˙˜ÌËÚ ÍÓÏÔÓÌÂÌÚË. LJÊ̇ Ò˙ÒÚ‡‚͇ ̇ ˆËÚÓÒÍÂÎÂÚ‡, ‚ ÍÓÈÚÓ ‡Á΢ÌË ÔÓÚÂËÌË ÔÂ̇ÒflÚ Ò‚Ófl ÚÓ‚‡ Ò‡ ÔÓÎflËÁË‡ÌËÚ ÏËÍÓÚÛ·ÛÎË, ÓÒË„Ûfl‚‡˘Ë ‰‡Î˜ÌËfl Ú‡ÌÒÔÓÚ Ë ‡ÍÚËÌ˙Ú, ÓÚ„Ó‚ÓÂÌ Á‡ ·ÎËÁÍËfl Ú‡ÌÒÔÓÚ (5,11). ëÏÛÚÂÌË Ú‡ÌÒÔÓÚÌË ÏÂı‡ÌËÁÏË Ò Ô‰ÔÓ·„‡Ú ÔË ÌflÍÓË ÓÚ ÙÓÏËÚÂ, Ò‚˙Á‡ÌË Ò ÏÛÚ‡ˆËË L1CAM (L1 cell adhesion molecule), PLP1 (proteolipid protein) Ë KIF5A „ÂÌË, ̇ „ÂÌËÚÂ, ÍÓ‰Ë‡˘Ë ÒÔ‡ÒÚËÌ, ÒÔ‡ÚËÌ Ë ‡Ú·ÒÚËÌ (6,11). èË ÌflÍÓË ÙÓÏË ÏÛÚ‡ÌÚÌËÚ ÔÓÚÂËÌË Ò ҂˙Á‚‡Ú ÍÓÏÔÂÚËÚË‚ÌÓ Ò ÏËÍÓÚÛ·ÛÎËÚÂ, ÍÓÂÚÓ ÔÓ‡Ê‰‡ ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ‚˙ÁÌËÍ‚‡Ì ̇ ‰ÓÏË̇ÌÚÌÓ Ì„‡ÚË‚ÂÌ ÂÙÂÍÚ. ëÂËÓÁÂÌ ‡„ÛÏÂÌÚ ÔÓÚË‚ Ú‡ÁË ıËÔÓÚÂÁ‡ Ó·‡˜Â  Á̇˜ËÚÂÎ̇ڇ ÌÂÒÚ‡·ËÎÌÓÒÚ Ì‡ Ïêçä ‚ ÏÛÚ‡ÌÚÌËÚ ÔÓÚÂËÌË. è‡‡Ô΄ËÌ˙Ú Ô‰ÒÚ‡‚Îfl‚‡ ÏËÚÓıÓ̉ˇÎ̇ ÏÂÚ‡ÎÓÔÓÚ‡Á‡. éÚÍË‚‡ÌÂÚÓ Ì‡ ÏÛÚ‡ˆËË ‚ ÍÓ‰Ë‡˘Ëfl „Ó „ÂÌ ‰‡‚‡ ‚˙ÁÏÓÊÌÓÒÚ ‰‡ Ò Ô‰ÔÓÎÓÊË, ˜Â ‚ ÓÒÌÓ‚‡Ú‡ ̇ ÌflÍÓË ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÎÂÊË ‰ÂÙÂÍÚ ‚ ÓÍÒˉ‡ÚË‚ÌÓÚÓ ÙÓÒÙÓËÎË‡Ì (OXPHOS) (5,6,13). ëÏÛ˘ÂÌËflÚ‡ ‚ OXPHOS ËÏ‡Ú ‡ÁÌÓÓ·‡Á̇ ÍÎËÌ˘̇ ÔÂÁÂÌÚ‡ˆËfl Ë Í‡ÚÓ ˆflÎÓ Ò‡ ËÁ‚ÂÒÚÌË Í‡ÚÓ ÏËÚÓıÓ̉ˇÎÌË Â̈ÂÙ‡ÎÓÏËÓÔ‡ÚËË. í ҇ ÓÚÌÓÒËÚÂÎÌÓ ˜ÂÒÚË ÔË Á‡·ÓÎfl‚‡ÌËfl ͇ÚÓ è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ıÓÂfl ̇ ï˙ÌÚËÌ„ÚÓÌ, îˉ‡ÈıÓ‚‡ ‡Ú‡ÍÒËfl Ë Äãë. ÑÂÙÂÍÚËÚ ‚ OXPHOS ÏÓ„‡Ú ‰‡ Ó͇Á‚‡Ú Ò‚ÓËÚ ÂÙÂÍÚË ˜ÂÁ ‡ÍÛÏÛÎË‡Ì ̇ Û‚‰‡ ‚ ÏÑçä Ë ÔÓ-̇ڇÚ˙¯ÌË ÓÍÒˉ‡ÚË‚ÌË ÒÏÛ˘ÂÌËfl ÔÓÒ‰ÒÚ‚ÓÏ ÔÓËÁ‚Ó‰ÒÚ‚Ó Ì‡ Ò‚Ó·Ó‰ÌË ‡‰Ë͇ÎË. çÂÁ‡‚ËÒËÏÓ ÓÚ ‚Ò˘ÍË Ì‡ÚÛÔ‡ÌË ‰Ó ÏÓÏÂÌÚ‡ ‰‡ÌÌË ÚÓ˜ÌËÚ ԇÚÓ„ÂÌÂÚ˘ÌË ÏÂı‡ÌËÁÏË ÔË Hëè ‚Ò Ӣ ÓÒÚ‡‚‡Ú ÌÂÔ˙ÎÌÓ ËÁflÒÌÂÌË Í‡ÚÓ ·˙‰Â˘ËÚ ËÁÒΉ‚‡ÌËfl ‚ Ú‡ÁË Ì‡ÒÓ͇ ·Ëı‡ ‚ÌÂÒÎË ÔÓ-„ÓÎflχ flÒÌÓÚ‡ ‚ ‡Á·Ë‡Ì ̇ ÔËÓ‰‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. äãàçàóçà ïÄêÄäíÖêàëíàäà çÄ ÄÇíéáéåçéÑéåàçÄçíçàíÖ îéêåà Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ ÒÔ‡ÒÚËÌÓ‚Ëfl „ÂÌ åÛÚ‡ˆËËÚ ‚ SPG4 „Â̇, ÍÓ‰Ë‡˘ ÒÔ‡ÒÚËÌÓ‚Ëfl ÔÓÚÂËÌ Ô‰ÒÚ‡‚Îfl‚‡Ú ÓÍÓÎÓ 40% ÓÚ ÒÎÛ˜‡ËÚÂ Ò ÄÑ HSP (7,9). íÓ‚‡  ̇È-˜ÂÒÚ‡Ú‡ ÙÓχ ̇ ÄÑ HSP, ‡ ÏÓÊ ·Ë Ë Ì‡È-˜ÂÒÚ‡Ú‡ ÙÓχ ËÁÓ·˘Ó. ÑÓ ÏÓÏÂÌÚ‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ì‡‰ 50 ıÂÚÂÓÁË„ÓÚÌË ÏÛÚ‡ˆËË ‚ ÒÔ‡ÒÚËÌÓ‚Ëfl „ÂÌ. èÓÒΉÌËÚ ËÏÛÌÓÙÎÛÓÂÒˆÂÌÚÌË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ÒÔ‡ÒÚËÌ˙Ú Â fl‰ÂÌ ÔÓÚÂËÌ. éÚÍË‚‡ Ò ‚ Ì‚ÓÌËÚ ̇ „·‚ÌËfl ÏÓÁ˙Í Ë ‚ Ô‰ÌÓÓ„Ó‚ËÚ ÍÎÂÚÍË, ÌÓ ÌÂ Ë ‚ „ÎËflÚ‡. èӂ˜ÂÚÓ SPG4 ÒÂÏÂÈÒÚ‚‡ Ò‡ Ò ˜ËÒÚ‡ ÙÓχ, ÌÓ Ò‡ ÓÔËÒ‡ÌË Ë ÌflÍÓÎÍÓ Ù‡ÏËÎËË Ò ÍÓÏÔÎˈË‡Ì ÙÂÌÓÚËÔ. èË ÒÂÏÂÈÒÚ‚‡Ú‡ Ò ˜ËÒÚ ÙÂÌÓÚËÔ Ò ̇·Î˛‰‡‚‡ ËÁ‡ÁÂ̇ ËÌÚÂ- Ë ËÌÚ‡Ù‡ÏËÎ̇ ‚‡ˇ·ËÎÌÓÒÚ Ì‡ ÚÂÊÂÒÚÚ‡ ̇ ÒËÏÔÚÓÏËÚÂ Ë Ì‡ ̇΢ËÂÚÓ ËÎË ÎËÔÒ‡Ú‡ ̇ ‰ÓÔ˙ÎÌËÚÂÎÌË ÒËÏÔÚÓÏË Í‡ÚÓ Ú‡ÁÓ‚ÓÂÁÂ‚Ó‡ÌË ÒÏÛ˘ÂÌËfl Ë ÒÏÛÚÂÌ ‚Ë·‡ˆËÓÌÂÌ ÛÒÂÚ. àÁÒΉ‚‡ÌÂÚÓ Ì‡ „ÓÎÂÏË ÒÂËË ÓÚ Ô‡ˆËÂÌÚË Ò˙Ò ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ̇Ô‡‚flÚ ÌflÍÓË Ó·Ó·˘ÂÌËfl ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÎËÌ˘̇ڇ ÔÂÁÂÌÚ‡ˆËfl ̇ Ú‡ÁË ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. 1. éÍÓÎÓ 25% ÓÚ ÌÓÒËÚÂÎËÚ ̇ „Â̇ Ò‡ ÍÎËÌ˘ÌÓ ‡ÒËÏÔÚÓÏÌË ËÎË ËÏ‡Ú ·ÂÎÂÁË, ÍÓËÚÓ Ë„ÌÓË‡Ú Ë ÍÓËÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ò‡ÏÓ ÔË ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡ÌÂ. çflÍÓË ÓÒÚ‡‚‡Ú ‡ÒËÏÔÚÓÏÌË ‰ÓË ‰Ó ÓÒχڇ ÒË ‰Â͇‰‡;


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

2. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡  29 „Ó‰ËÌË, ÌÓ Ò „ÓÎÂÏË ‚‡ˇˆËË ÓÚ 1 ‰Ó 74 „Ó‰ËÌË; 3. èÓ„ÂÒËflÚ‡  ‚‡ˇ·ËÎ̇, ÌÓ Ò‰ÌÓ 31% ÓÚ Ô‡ˆËÂÌÚËÚ Ì ÏÓ„‡Ú ‰‡ Ò ‰‚ËÊ‡Ú Ò‡ÏÓÒÚÓflÚÂÎÌÓ Ì‡ ‚˙Á‡ÒÚ 48 „Ó‰ËÌË. èÓ„ÂÒËflÚ‡  Á̇˜ËÚÂÎÌÓ ÔÓ-·˙Á‡ ÔË Í˙ÒÌÓ Ì‡˜‡ÎÓ (ÒΉ 35 „.), ͇ÍÚÓ Â ·ËÎÓ Ô‰ÔÓÎÓÊÂÌÓ Ô˙‚Ó̇˜‡ÎÌÓ ÓÚ Harding (17),. çflÍÓË ËÁÒΉ‚‡ÌËfl Ò‡‚Ìfl‚‡Ìfl‚‡Ú ÚÂÊÂÒÚÚ‡ ̇ ÏÓÚÓÌËÚ ‰ËÒÙÛÌ͈ËË ÔË Ô‡ˆËÂÌÚËÚ Ò˙Ò ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÍÓÌÍÂÚÌËfl ÚËÔ ÏÛÚ‡ˆËfl. èÓ ÚÓÁË Ì‡˜ËÌ Ò Ô‡‚Ë ÓÔËÚ Á‡ ÓÒ˙˘ÂÒÚ‚fl‚‡Ì ̇ ÚӘ̇ ÍÓ·ˆËfl ÏÂÊ‰Û ÓÔ‰ÂÎÂÌ ÏÛÚ‡ˆËÓÌÂÌ „ÂÌÓÚËÔ Ë ÒÔˆËÙ˘ÂÌ ÍÎËÌ˘ÂÌ ÙÂÌÓÚËÔ. éÔËÒ‡ÌË Ò‡ ‰‚ ÒÂÏÂÈÒÚ‚‡ Ò ‡Á΢ÌË ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË, ÍÓËÚÓ ÔÓ͇Á‚‡Ú ÏÌÓ„Ó „ÓÎflχ ÍÎËÌ˘̇ ·ÎËÁÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‚˙Á‡ÒÚÚ‡ ̇ ‰Â·˛Ú‡, ÔÓ„ÂÒËflÚ‡ Ë ÚÂÊÂÒÚÚ‡ ̇ ÒËÏÔÚÓÏËÚÂ. åÓÚÓ̇ڇ ÒËÒÚÂχ  ËÁÒΉ‚‡Ì‡ ˜ÂÁ Ú‡ÌÒÍ‡ÌˇÎ̇ χ„ÌËÚ̇ ÒÚËÏÛ·ˆËfl (íåë) Ò ˆÂÎ ‰‡ Ò ÛÒÚ‡ÌÓ‚Ë ‰‡ÎË ‡Á΢ÌËÚ ÏÛÚ‡ˆËË ‚ ÒÔ‡ÒÚËÌÓ‚Ëfl „ÂÌ ‚Ó‰flÚ ‰Ó ËÁfl‚‡ ̇ ÒÔˆËÙ˘ÌË ÍÎËÌ˘ÌË ·ÂÎÂÁË Ë Í‡Í ÔÓ‰Ó·ÌË ÏÓÎÂÍÛÎÌË ÔÓÏÂÌË Ò ÔÂ̇ÒflÚ ‚ ‡Á΢ÂÌ ÍÎËÌ˘ÂÌ ÙÂÌÓÚËÔ. àÁÒΉ‚‡ÌË Ò‡ ˆÂÌÚ‡ÎÌÓÚÓ Ë ÔÂËÙÂÌÓ ÏÓÚÓÌÓ ÔÓ‚Ó‰ÌÓ ‚ÂÏÂ Ë ‡ÏÔÎËÚÛ‰ËÚ ̇ ÏÓÚÓÌËÚ ‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË ÒΉ íåë. ë‡‚ÌÂÌËÂÚÓ ÏÂÊ‰Û ‰‚ÂÚ ÒÂÏÂÈÒÚ‚‡ ÔÓ͇Á‡ÎÓ ‡Á΢Ëfl ‚ ˆÂÌÚ‡ÎÌÓÚÓ ÏÓÚÓÌÓ ÔÓ‚Ó‰ÌÓ ‚ÂÏ ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÚËÔ‡ ̇ ÏÛÚ‡ˆËflÚ‡. çÂ˘Ó Ôӂ˜Â, ‚ Ô˙‚ÓÚÓ ÒÂÏÂÈÒÚ‚Ó Ò‡ ̇·Î˛‰‡‚‡ÌË åÖè, ÌÂ‡Á΢ËÏË ÓÚ ÚÂÁË ÔË Á‰‡‚ËÚ ÍÓÌÚÓÎË, ‰Ó͇ÚÓ ÔË ‚ÚÓÓÚÓ ÒÂÏÂÈÒÚ‚Ó åÖè ÔÓ͇Á‚‡Ú Á̇˜ËÚÂÎÌË ‡·ÌÓÏÌÓÒÚË. íÓ‚‡ ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ӷӷ˘Ë, ˜Â ‚ÚÓ‡Ú‡ ÏÛÚ‡ˆËfl Ó·ÛÒ·‚fl ÚÂÊÍË ÔÓÏÂÌË ‚ åÖè Ë ˜Â íåë ÏÓÊ ‰‡ Ò ËÁÔÓÎÁ‚‡ Á‡ ÙÂÌÓÚËÔ̇ ÓˆÂÌ͇ ÔË Ô‡ˆËÂÌÚË Ò çëè ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÙÛÌ͈ËÓ̇ÎÌÓÚÓ Á̇˜ÂÌË ̇ ‰‡‰Â̇ ÒÔ‡ÒÚËÌÓ‚‡ ÏÛÚ‡ˆËfl. àÌÚÂÂÒ Ô‰ÒÚ‡‚Îfl‚‡ Ë Ù‡ÍÚ˙Ú, ˜Â ÒÔ‡ÒÚËÌÓ‚ËÚ ÏÛÚ‡ˆËË Ò ‡ÒÓˆËË‡Ú Ò ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËfl. ãÂÍË Ë Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËfl Ò Ò¢‡Ú Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚÓ ÓÚ Ó˜‡Í‚‡ÌÓÚÓ Á‡ ÔÓÔÛ·ˆËflÚ‡ ÓÚ çëè ÒÂÏÂÈÒÚ‚‡ Ò “˜ËÒÚ” ÙÂÌÓÚËÔ. ê‡Á‚Ë‚‡Ú Ò ÒΉ 40 „Ó‰ËÌË Í‡ÚÓ ÎÂÍËflÚ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ ‰ÓË ÏÓÊ ‰‡ ·˙‰Â Ô˙‚‡ ËÁfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. Ç à·̉Ëfl Ë î‡ÌˆËfl Ò‡ ÓÔËÒ‡ÌË ‰‚ هÏËÎËË Ò ˜ËÒÚ‡ ÙÓχ ̇ çëè, ‡ÒÓˆËË‡Ì‡ Ò ‰ÂÏÂ̈Ëfl Ë ÂÔËÎÂÔÚ˘ÌË ÔËÔ‡‰˙ˆË. Ç ÄÌ„ÎËfl ‚ ÒÂÏÂÈÒÚ‚Ó Ò ˜ËÒÚ‡ ÙÓχ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Â ÓÔËÒ‡Ì Ô‡ˆËÂÌÚ Ò ‰ÂÏÂ̈Ëfl Ò Í˙ÒÂÌ ‰Â·˛Ú ‚ ıÓ‰‡ ̇ ÊË‚ÓÚ‡ ÏÛ. àÁÒΉ‚‡ÌËflÚ‡ ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â Ë ‰‚‡Ï‡ Ô‰ÒÚ‡‚ËÚÂÎË Ì‡ Ô‰ıӉ̇ڇ „ÂÌÂ‡ˆËfl ‚ ÚÓ‚‡ ÒÂÏÂÈÒÚ‚Ó Ò˙˘Ó ËχÎË ËÌÚÂÎÂÍÚÛ‡ÎÌË ‰ËÒÙÛÌ͈ËË. è‡ÚÓÎӄӇ̇ÚÓÏ˘̇ڇ ̇ıӉ͇ ÔË ËÁÒΉ‚‡ÌËfl Ô‡ˆËÂÌÚ ÔÓ͇Á‡Î‡ ̇΢ËÂÚÓ Ì‡ ÒÔˆËÙ˘ÌË ÍÓÚË͇ÎÌË ÔÓÏÂÌË. ç‡Îˈ ·Ë· Ì‚Ó̇Î̇ Á‡„Û·‡ Ë Ú‡Û-ËÏÛÌÓ‡ÍÚË‚ÌË Ì‚ÓÙË·ËÎÂÌË ÒÔÎËÚ‡ÌËfl ‚ ıËÔÓ͇ÏÔ‡. í‡Û-ËÏÛÌÓ‡ÍÚË‚ÌË ·‡ÎÓÌË‡ÌË ÍÎÂÚÍË Ò‡ ÓÚÍËÚË Ë ‚ ÎËϷ˘̇ڇ ÍÓ‡ Ë ‚ ÌÂÓÍÓÚÂÍÒ‡. Ç ÒÛ·Òڇ̈Ëfl ÌË„‡ Ò‡ ÓÚÍËÚË ÚÂΈ‡ ̇ ã‚Ë. í‡ÁË Ô‡ÚÓÎӄ˘̇ ̇ıӉ͇ Ì  ı‡‡ÍÚÂ̇ Á‡ ÌËÚÓ Â‰Ì‡ ÓÚ ËÁ‚ÂÒÚÌËÚ ‰Ó ÏÓÏÂÌÚ‡ Ú‡Û-Ô‡ÚËË. Ç˙Á ÓÒÌÓ‚‡ ̇ ÚÂÁË Ì‡·Î˛‰ÂÌËfl Ò Ô‰ÔÓ·„‡, ˜Â ˜ËÒÚ‡Ú‡ çëè Ò Ò˙˜ÂÚ‡‚‡ ‰ÓÒÚ‡ ˜ÂÒÚÓ Ò ‰ÂÏÂ̈Ëfl. ë˙˘Â‚ÂÏÂÌÌÓ ‰ÂÏÂ̈ËflÚ‡ Ì  ÌÂÓÚÏÂÌÂÌ ÒËÏÔÚÓÏ Ì‡ çëè. åÓ‰ÂÎ˙Ú Ì‡ ‰ÂÏÂÌÚ̇ڇ ·ÓÎÂÒÚ ‚ ÚÓ‚‡ ÒÂÏÂÈÒÚ‚Ó Ô‰ÔÓ·„‡ ̇΢ËÂÚÓ Ì‡ ÚÂÊ͇ ÒÏÂÒÂ̇ ‰ÂÏÂ̈Ëfl, fl‚fl‚‡˘‡ Ò Í˙ÒÌÓ ‚ ıÓ‰‡ ̇ ·ÓÎÂÒÚÚ‡. è‰ÒÚÓË ‰‡ Ò ÔÓÚ‚˙‰Ë ‰‡ÎË ÚÂÁË ÔÓÏÂÌË Ò‡ ÂÁÛÎÚ‡Ú ÓÚ ‰ÂÈÒÚ‚ËÂÚÓ Ì‡ ‡·ÌÓÏÌËfl ÒÔ‡ÒÚËÌ (17). èË Ù‡ÏËÎËËÚ Ò˙Ò ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË Â ÛÒÚ‡ÌÓ‚Â̇ Ë ‚‡ˇ·ËÎÌÓ ÔÓfl‚Â̇ ‡ÌÚˈËÔ‡ˆËfl (ÔÓ-‡Ì̇ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡ ËÎË ÔÓ-„ÓÎflχ ÚÂÊÂÒÚ Ì‡ ÒËÏÔÚÓÏËÚ ‚ ÒΉ‚‡˘ËÚ ÔÓÍÓÎÂÌËfl). ç‡Îˈ ҇ ͇ÍÚÓ ËÌÚÂ-, ڇ͇ Ë ËÌÚ‡Ù‡ÏËÎÌË ‡Á΢Ëfl ‚˙‚ ‚˙Á‡ÒÚÚ‡ ̇ ‰Â·˛Ú‡. ÄÌÚˈËÔ‡-

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ˆËflÚ‡ Ò Ò¢‡ ÔË ‰ˈ‡ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌË Á‡·ÓÎfl‚‡ÌËfl Ë Â Ò‚˙Á‡Ì‡ Ò ‡·ÌÓÏÌË ÚËÌÛÍÎÂÓÚˉÌË ÔÓ‚ÚÓÂÌËfl. ëÔ‡ÒÚËÌÓ‚ËflÚ „ÂÌ Ì Ò˙‰˙ʇ ÔÓ‰Ó·ÌË ‡·ÌÓÏÌË ÔÓ‚ÚÓÂÌËfl. è‰ÒÚÓË ‰‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÚӘ̇ڇ Ô˘Ë̇ Á‡ ‡ÌÚˈËÔ‡ˆËflÚ‡. ë˙˘ÂÒÚ‚Û‚‡ Ë ‚˙ÁÏÓÊÌÓÒÚÚ‡ “‡ÌÚˈËÔ‡ˆËflÚ‡” ‰‡  ÂÁÛÎÚ‡Ú ÓÚ ÔÓ-‡ÌÌÓ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔË ‰Âˆ‡ ̇ Á‡Ò„̇ÚË Ó‰ËÚÂÎË (2,10,17). è‡‚ÂÌË Ò‡ ÓÔËÚË Ë Á‡ ÓÒ˙˘ÂÒÚ‚fl‚‡Ì ̇ ÙÂÌÓÚËÔÌË ÍÓ·ˆËË ÏÂÊ‰Û ·ÓÎÌË Ò ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚ̇ çëè Ò ÏÛÚ‡ˆËË ‚ ÒÔ‡ÒÚËÌÓ‚Ëfl „ÂÌ ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ Ë ·ÂÁ ÔÓ‰·ÌË ÏÛÚ‡ˆËË ÓÚ ‰Û„‡. é·Ó·˘ÂÌÓ ÏÓÊ ‰‡ Ò ͇ÊÂ, ˜Â ëèÄëí (+) ÒÂÏÂÈÒÚ‚‡ ÔÓ͇Á‚‡Ú ÔÓ-„ÓÎflχ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡, ÔÓÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl, ÔÓ-·˙Á‡ ÔÓ„ÂÒËfl ̇ Ô‡‡Ô‡ÂÁ‡Ú‡ Ë ÔÓ-ÚÂÊÍË ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËfl. çÂÁ‡‚ËÒËÏÓ ÓÚ ‡Á΢ÌËÚ ÏÛÚ‡ˆËË ÚÂÁË ÒÂÏÂÈÒÚ‚‡ ËÏ‡Ú ·ÎËÁ˙Í ÙÂÌÓÚËÔ, ÍÓÈÚÓ ÏÓÊ ‰‡ ·˙‰Â ‡Á„‡Ì˘ÂÌ ÓÚ ‰Û„ËÚ „ÂÌÂÚ˘ÌË „ÛÔË. Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ ‡Ú·ÒÚËÌÓ‚Ëfl „ÂÌ åÛÚ‡ˆËËÚ ‚ SPG3A „Â̇, ÍÓ‰Ë‡˘ ·ÂÎÚ˙͇ ‡Ú·ÒÚËÌ ‚Ó‰flÚ ‰Ó ËÁfl‚‡ ̇ ÔË·ÎËÁËÚÂÎÌÓ 10%-15% ÓÚ ‰ÓÏË̇ÌÚÌÓ Û̇ÒΉfl‚‡ÌËÚ ÒÎÛ˜‡È ̇ çëè. SPG3A Ò ÂÍÒÔÂÒË‡ Ô‰ËÏÌÓ ‚ ñçë-ÔË‡ÏˉÌËÚ Ì‚ÓÌË Ë ıËÔÓ͇ÏÔ‡. è˙‚‡Ú‡ ËÁfl‚‡ ̇ ÒËÏÔÚÓÏË Â ‚ ‰ÂÚÒÚ‚ÓÚÓ – ÏÂÊ‰Û Ô˙‚‡ Ë ‚ÚÓ‡ ‰Â͇‰‡. èË Ú‡ÁË ÙÓχ ÎËÔÒ‚‡Ú ‰‡ÌÌË Á‡ ̇΢ËÂÚÓ Ì‡ ‡ÌÚˈËÔ‡ˆËfl. è˙‚Ó̇˜‡ÎÌÓ ËÁ„ÎÂʉ‡ÎÓ, ˜Â ÙÂÌÓÚËÔ˙Ú Â Á̇˜ËÚÂÎÌÓ ÔÓ-ÚÂÊ˙Í ÓÚ ÚÓÁË ÔË ÓÒڇ̇ÎËÚ ÒÛ·ÚËÔӂ ̇ çëè. èÓÒΉ‚‡ÎËÚ ̇·Î˛‰ÂÌËfl ‚˙ıÛ ÔÓ-Ó·¯ËÌË „ÛÔË ÓÚ Ô‡ˆËÂÌÚË ÔÓ͇Á‡ÎË, ˜Â ÚÂÊÂÒÚÚ‡ ÔË Ú‡ÁË ÙÓχ Ì Ò ÓÚ΢‡‚‡ Á̇˜ËÏÓ ÓÚ ÒÂÏÂÈÒÚ‚‡Ú‡ Ò˙Ò ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË. ï‡‡ÍÚÂ̇ Á‡ ÙÂÌÓÚËÔ‡ ̇ ‡Ú·ÒÚËÌÓ‚ËÚ ÏÛÚ‡ˆËË Â ÎËÔÒ‡Ú‡ ̇ Ú‡ÁÓ‚ÓÂÁÂ‚Ó‡ÌË Ë ÒÂÚË‚ÌË ÒÏÛ˘ÂÌËfl. èÓ„ÂÒËflÚ‡ ̇ ÒÏÛ˘ÂÌËflÚ‡ ‚ ÔÓıӉ͇ڇ  ·‡‚̇ Ë ÏÌÓ„Ó ÓÚ ÔÓ-‚˙Á‡ÒÚÌËÚ ·ÓÎÌË Ò ӷÒÎÛÊ‚‡Ú Ò‡ÏÓÒÚÓflÚÂÎÌÓ. èË Ì‡˜‡ÎÓ Ì‡ çëè ‚ ‰ÂÚÒÚ‚ÓÚÓ ‰Ë‡„ÌÓÁ‡Ú‡ ˜ÂÒÚÓ Ò „Â¯Ë Ò Ññè. èÓ„ÂÒËflÚ‡ ̇ ÒËÏÔÚÓÏËÚ ӷËÍÌÓ‚ÂÌÓ ‡Á„‡Ì˘‡‚‡ ‰‚ÂÚ Á‡·ÓÎfl‚‡ÌËfl. íÓ‚‡ ˜ÂÒÚÓ Ì ‚‡ÊË Á‡ ÒÎÛ˜‡ËÚÂ Ò ‡Ú·ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË, Í˙‰ÂÚÓ ÔÓ‡‰Ë ·‡‚̇ڇ ÔÓ„ÂÒËfl ̇ ÒËÏÔÚÓÏËÚ ‰ËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡  ‰ÓÒÚ‡ Úۉ̇ Ë Ò ̇·„‡ ÔÓ‚Âʉ‡Ì ̇ „ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì (7,20). àÌÚÂÂÒ Ô‰ÒÚ‡‚Îfl‚‡ Ù‡ÍÚ˙Ú, ˜Â Á‡ Ô˙‚Ë Ô˙Ú Â ÓÔËÒ‡ÌÓ ‡ÙÓ‡ÏÂË͇ÌÒÍÓ ÒÂÏÂÈÒÚ‚Ó Ò ÏÛÚ‡ˆËfl ‚ ‡Ú·ÒÚËÌÓ‚Ëfl „ÂÌ. 燘‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ‚ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ Ë Ò‡ Á‡Ò„̇ÚË ÚË ÔÓÒΉӂ‡ÚÂÎÌË „ÂÌÂ‡ˆËË ‚ ‰̇ Ù‡ÏËÎËfl. çËÚÓ Â‰ËÌ ÓÚ Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Ì‡ Ô‡ÍÚË͇ ÌËÍÓ„‡ Ì  ËχΠÌÓχÎ̇ ÔÓıӉ͇. èË ‚Ò˘ÍË Â Ì‡Îˈ ·‡‚̇ ÔÓ„ÂÒËfl ̇ ÚÛ‰ÌÓÒÚËÚ ÔË ıÓ‰ÂÌÂ Ë pes cavus. èË ÌËÍÓÈ ÓÚ ·ÓÎÌËÚ Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÒÂÚË‚ÌË Ë Ú‡ÁÓ‚ÓÂÁÂ‚Ó‡ÌË ÒÏÛ˘ÂÌËfl. ÑÓ ÏÓÏÂÌÚ‡ Ò‡ ÓÔËÒ‡ÌË 6 ÏÛÚ‡ˆËË ‚ ‡Ú·ÒÚËÌÓ‚Ëfl „ÂÌ. èÂÚ ÓÚ ÚÂÁË ÏÛÚ‡ˆËË Ò‡ ÔÓ„¯ÌÓ ÒÏËÒÎÂÌË. Ö‰ËÌÒÚ‚Â̇ڇ ‰Û„‡ ÏÛÚ‡ˆËfl  ËÌÒÂˆËfl. á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë ÔË ÔÓÒΉ̇ڇ ËÏ‡Ú ‚‡ˇ·ËÎ̇ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡ – ÓÚ 5 ‰Ó 39 „Ó‰ËÌË. Ñ‚‡Ï‡ Ô‡ˆËÂÌÚË ‰ÓË Ò‡ ‡ÒËÏÔÚÓÏÌË, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÌflÍÓË ‡·ÌÓÏÌÓÒÚË ‚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ. í‡ÁË ÙÂÌÓÚËÔ̇ ËÁfl‚‡  ‰ÓÒÚ‡ ÔÓ-‡Á΢̇ ÓÚ Ì‡·Î˛‰‡‚‡Ì‡Ú‡ ÔË ÔÓ„¯ÌÓ ÒÏËÒÎÂÌËÚ ÏÛÚ‡ˆËË, ÔË ÍÓËÚÓ Â Ì‡Îˈ ڂ˙‰Â ÒÚÂÂÓÚËÔ̇ ÍÎËÌ˘̇ χÌËÙÂÒÚ‡ˆËfl ̇ ÒËÏÔÚÓÏËÚÂ Ò ÌÂÓÚÏÂÌÌÓ ‡ÌÌÓ Ì‡˜‡ÎÓ, ӷ˘‡ÈÌÓ ÔÂ‰Ë 10 „Ó‰ËÌË. Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ „Â̇, ÍÓ‰Ë‡˘ ÏËÚÓıÓ̉ˇÎÌËfl ÔÓÚÂËÌ heat shock protein 60 (chaperonin 60) åÛÚ‡ˆËflÚ‡ ‚ ÏËÚÓıÓ̉ˇÎÌËfl ÔÓÚÂËÌ heat shock


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

protein 60 (chaperonin 60) Ô‰ËÁ‚ËÍ‚‡ ˜ËÒÚ‡ ÙÓχ ̇ ÄÑ HSP, Ò‚˙Á‡Ì‡ Ò ÎÓÍÛÒ SPG13. ÑÓ ÏÓÏÂÌÚ‡  ÓÔË҇̇ Ò‡ÏÓ Â‰Ì‡ Ù‡ÏËÎËfl Ò ‰Â‚ÂÚ Á‡Ò„̇ÚË Ë̉˂ˉ‡. Ñ·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â Ì‡ ‚˙Á‡ÒÚ ÏÂÊ‰Û 17 Ë 68 „Ó‰ËÌË (6,8). ÑÓÏË̇ÌÚÌÓÚÓ Û̇ÒΉfl‚‡Ì ÔË Ú‡ÁË ÙÓχ ÏÓÊ ‰‡  ÂÁÛÎÚ‡Ú ÓÚ ı‡ÔÎÓËÌÒÛÙˈËÂÌÚÌÓÒÚ, ‰˙Îʇ˘‡ Ò ̇ ̇χÎfl‚‡Ì ̇ Ó·˘‡Ú‡ ¯‡ÔÂÓÌËÌÓ‚‡ ‡ÍÚË‚ÌÓÒÚ. èÓ‡‰Ë ÓÚÍË‚‡ÌÂÚÓ Ì‡ ÏÛÚ‡ˆËË ‚ „ÂÌËÚÂ, ÍÓ‰Ë‡˘Ë ¯‡ÔÂÓÌËÌËÚÂ Ë Úflı̇ڇ ÓÎfl ͇ÚÓ ÏÓ‰Û·ÚÓË Ì‡ ÙÂÌÓÚËÔ‡, ‚Ò Ôӂ˜ Ò ‡Á¯Ëfl‚‡ ‰Ë‡Ô‡ÁÓ̇ ̇ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌËÚ Á‡·ÓÎfl‚‡ÌËfl, ÔË ÍÓËÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Íβ˜Ó‚‡Ú‡ ËÏ ÓÎfl, ‚Íβ˜ËÚÂÎÌÓ AR ÒÔ‡ÒÚ˘̇ ‡Ú‡ÍÒËfl ̇ CharlevoixSaguenay Ë ALS, Ëχ˘‡ ÌflÍÓË Ó·˘Ë ˜ÂÚË Ò çëè. Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ Kinesin heavy chain (KIF5A) „Â̇ åÛÚ‡ˆËËÚ ‚ KIF5A „Â̇ ‚Ó‰flÚ ‰Ó ËÁfl‚‡ ̇ ‰ÓÏË̇ÌÚ̇ çëè, Ò‚˙Á‡Ì‡ Ò ÎÓÍÛÒ SPG10. ífl ‚Ó‰Ë ‰Ó ËÁfl‚‡Ú‡ ̇ ˜ËÒÚ‡ ËÎË ÍÓÏÔÎˈË‡Ì‡ Ò ‰ËÒÚ‡Î̇ ‡ÏËÓÚÓÙËfl ÙÓÏË. éÔËÒ‡ÌÓ Â Â‰ÌÓ ·ËÚ‡ÌÒÍÓ ÒÂÏÂÈÒÚ‚Ó Ò ˜ËÒÚ‡ ÙÓχ, ÔË ÍÓÂÚÓ ‚˙Á‡ÒÚÚ‡ ̇ ‰Â·˛Ú‡  ÔÓ-‡Ì̇ ÓÚ Ú‡ÁË ÔË Ù‡ÏËÎËËÚ Ò˙Ò ÒÔ‡ÒÚËÌÓ‚Ë ÏÛÚ‡ˆËË – 10-11 „Ó‰ËÌË. îÂÌÓÚËÔ˙Ú Ò ı‡‡ÍÚÂËÁË‡ Ò „ÓÎflχ ‚‡ˇ·ËÎÌÓÒÚ Ì‡ ÚÂÊÂÒÚÚ‡ ̇ ÒËÏÔÚÓÏËÚ ͇ÚÓ ÌflÍÓË Ô‡ˆËÂÌÚË Ò ÌÛʉ‡flÚ ÓÚ ËÌ‚‡ÎˉÂÌ ÒÚÓÎ, ‡ ‰Û„Ë ÓÒÚ‡‚‡Ú ‡ÒËÏÔÚÓÏÌË Ì‡ ‚˙Á‡ÒÚ 40 „Ó‰ËÌË (6,7). Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ NIPA1 (neuronal membrane protein of unknown function) „Â̇ NIPA1 „ÂÌÌËÚ ÏÛÚ‡ˆËË ‚Ó‰flÚ ‰Ó ‡Á‚ËÚËÂÚÓ Ì‡ ÄÑ çëè Ò ‰Â·˛Ú ‚ ˛ÌÓ¯ÂÒÚ‚ÓÚÓ ËÎË ‚ Áfl· ‚˙Á‡ÒÚ, ·‡‚̇ ÔÓ„ÂÒËfl Ë ˜ËÒÚ ÙÂÌÓÚËÔ. ÑÓ ÏÓÏÂÌÚ‡ Ò‡ ÓÔËÒ‡ÌË ‰‚ هÏËÎËË Ò ÏÛÚ‡ˆËË ‚ NIPA1 (6,7,15). íÓ‚‡  ‰̇ ÓÚ Ì‡ÈÚÂÊÍËÚ ÙÓÏË Ì‡ ‰ÓÏË̇ÌÚÌÓ Û̇ÒΉfl‚‡Ì‡, ˜ËÒÚ‡ çëè. NIPA1 ¯ËÓÍÓ Ò ÂÍÒÔÂÒË‡ ‚ ñçë. éÔËÒ‡ÌÓ Â Â‰ÌÓ „ÓÎflÏÓ Ò‚ÂÌÓ ‡ÏÂË͇ÌÒÍÓ ÒÂÏÂÈÒÚ‚Ó, Ò‚˙Á‡ÌÓ Ò ÏÛÚ‡ˆËfl ‚ NIPA1. 燘‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÏÂÊ‰Û 12 Ë 35 „Ӊ˯̇ ‚˙Á‡ÒÚ. Ç˙ÔÂÍË ˜Â ÙÂÌÓÚËÔ˙Ú Â ˜ËÒÚ, ÚÓÈ Ò ÓÚ΢‡‚‡ Ò ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-„ÓÎflχ ÚÂÊÂÒÚ. èÓ˜ÚË 1/3 ÓÚ Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Ò ÌÛʉ‡flÚ ÓÚ ËÌ‚‡ÎˉÂÌ ÒÚÓΠ̇ Ò‰̇ ‚˙Á‡ÒÚ 40 „Ó‰ËÌË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Ì‡Îˈ pes cavus . ÇÚÓÓÚÓ ÓÔËÒ‡ÌÓ ÒÂÏÂÈÒÚ‚Ó Â Ò ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡ ÏÂÊ‰Û 5 Ë 22 „Ó‰ËÌË (10). äãàçàóçà ïÄêÄäíÖêàëíàäà çÄ ÄÇíéáéåçéêÖñÖëàÇçàíÖ îéêåà çÄ çëè Ä‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ ıÂ‰ËÚ‡̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ Ô‡‡Ô΄ËÌÓ‚Ëfl „ÂÌ SPG7 ÍÓ‰Ë‡ ÔÓÚÂËÌ, ̇˜ÂÌ Ô‡‡Ô΄ËÌ, ÓÚÍËÚ ÔÂÁ 1998 „ ÓÚ De Michele. íÓ‚‡  Ô˙‚ËflÚ ‡‚ÚÓÁÓÏÂÌ „ÂÌ, ÛÒÚ‡ÌÓ‚ÂÌ ÔË çëè. è˙‚Ó̇˜‡ÎÌÓ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË 4, ‡ ̇ÔÓÒΉ˙Í Ó˘Â 6 ÏÛÚ‡ˆËË ‚ Ì„Ó. íÓÈ ‚Ó‰Ë ‰Ó ËÁfl‚‡Ú‡ ̇ ÍÎËÌ˘ÌÓ ˜ËÒÚ ÙÂÌÓÚËÔ ËÎË Ì‡ ÍÓÏÔÎˈË‡Ì Ò ‰ËÁ‡ÚËfl, ‰ËÒÙ‡„Ëfl, ÓÔÚ˘̇ ‡ÚÓÙËfl, ‡ÍÒÓ̇Î̇ Ì‚ÓÔ‡ÚËfl Ë Ì‡Î˘Ë ̇ “Ò˙‰Ó‚Ë” ÎÂÁËË, ˆÂ·‡Î̇ ËÎË ˆÂ·Â·̇ ‡ÚÓÙËfl ̇ åêí. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â 25 „Ó‰ËÌË (6,7,10). ᇠÔ˙‚ÓÚÓ ÓÔËÒ‡ÌÓ ÒÂÏÂÈÒÚ‚Ó Ò Ú‡ÁË ÏÛÚ‡ˆËfl  ÔˆÂÌÂÌÓ, ˜Â Â Ò ˜ËÒÚ ÙÂÌÓÚËÔ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ ‰ËÁ‡ÚËfl, ÍÓflÚÓ ÌÓχÎÌÓ Ì Ò Ò¢‡ ÔË ˜ËÒÚËÚ ÙÓÏË. èË ËÁÒΉ‚‡Ì ̇ ÔÓ-Ó·¯ËÌË „ÛÔË ÓÚ Ô‡ˆËÂÌÚË Ò ÌÓ‚ÓÓÔËÒ‡ÌËÚ 6 ÏÛÚ‡ˆËË ‚ Ô‡‡Ô΄ËÌÓ‚Ëfl „ÂÌ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ò‰̇ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú‡ ÏÂÊ‰Û 11 Ë 19 „. Ë ·‡‚̇ ÔÓ„ÂÒËfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ç‡Îˈ  ‚‡ˇ·ËÎÌÓ ÍÓÏÔÎˈË‡Ì ÙÂÌÓÚËÔ Í‡ÚÓ Ì‡‰ Ò˙Ò ÒÔ‡ÒÚ˘ÌÓÒÚÚ‡ ‚

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‰ÓÎÌË Í‡ÈÌËˆË Ò ̇·Î˛‰‡‚‡Ú ˆÂ·Â·ÌË ·ÂÎÂÁË Ò ‡Ú‡ÍÒËfl ‚ Í‡ÈÌˈËÚÂ, ‰ËÁ‡ÚËfl Ë ÌËÒÚ‡„˙Ï. çËÍÓÈ ÓÚ Ô‡ˆËÂÌÚËÚ Ìflχ ÓÔÚ˘̇ ‡ÚÓÙËfl. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í ÔË ·ÓÎÌËÚÂ Ò ‡Ú‡ÍÒËfl ÔÓ͇Á‚‡ ̇΢ˠ̇ ˆÂ·Â·̇ ‡ÚÓÙËfl. óÂÒÚÓ Ô‡ˆËÂÌÚËÚ ҇ ‰Âˆ‡ ̇ ·ÎËÁÍÓ Ó‰ÒÚ‚ÂÌË ·‡ÍÓ‚Â. ïËÒÚÓÎӄ˘ÌËÚÂ Ë ıËÒÚÓ·ËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl ‡ÁÍË‚‡Ú ̇΢ËÂÚÓ Ì‡ ‰ÂÙÂÍÚË ‚ ÏËÚÓıÓ̉ˇÎÌËÚ ÂÒÔË‡ÚÓÌÓ ‚ÂËÊÌË ‡ÍÚË‚ÌÓÒÚË. Ä‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ ̇ÒΉÒÚ‚Â̇‡ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ ÒÔ‡ÚËÌÓ‚Ëfl „ÂÌ ëÔ‡ÚËÌÓ‚ËÚ ÏÛÚ‡ˆËË ‚Ó‰flÚ ËÁfl‚‡Ú‡ ̇ çëè, ‡ÒÓˆËË‡Ì‡ Ò ‰ËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ (SPG20 Troyer syndrome). ëË̉ÓÏ˙Ú Ì‡ Troyer  ÓÔËÒ‡Ì ÔÂÁ 1967 „. ÔË Â‚ÂÈÒ͇ ÔÓÔÛ·ˆËfl Ô‡ˆËÂÌÚË, ÔÓÒΉӂ‡ÚÂÎË Ì‡ ëÚ‡Ëfl Á‡‚ÂÚ ‚ ëÄô, ˘‡Ú‡ éı‡ÈÓ. í Ô‡ÍÚËÍÛ‚‡Ú ÒÚÓ„‡ Ẩӄ‡ÏËfl Ë ËÏ‡Ú „ÓÎÂÏË ÒÂÏÂÈÒÚ‚‡ ͇ÚÓ ÔÓ ÚÓÁË Ì‡˜ËÌ Ô‰ÒÚ‡‚Îfl‚‡Ú ËÁÍβ˜ËÚÂÎÌÓ ÔÓ‰ıÓ‰fl˘‡ „ÛÔ‡ Á‡ ËÁÒΉ‚‡Ì ̇ ˆÂÒË‚ÌÓ Û̇ÒΉfl‚‡ÌËÚ Á‡·ÓÎfl‚‡ÌËfl. ëË̉ÓÏ˙Ú Ô‰ÒÚ‡‚Îfl‚‡ ÍÓÏÔÎˈË‡Ì‡ ÙÓχ ̇ ˆÂÒ˂̇ çëè Ò ·‡‚̇ ÔÓ„ÂÒËfl. ä‡‰Ë̇ÎÌËÚ ÏÛ ·ÂÎÂÁË Ò‡ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô‡ÂÁ‡, ÔÒ‚‰Ó·Ûη‡̇ Ô‡‡ÎËÁ‡ Ë ‰ËÒÚ‡Î̇ ‡ÏËÓÚÓÙËfl ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ÎÂ͇ ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl Ë ÒÍÂÎÂÚÌË ‡ÌÓχÎËË. ÇÒ˘ÍË Ô‡ˆËÂÌÚË ÔÓıÓʉ‡Ú Ë ÔÓ„Ó‚‡flÚ Ì‡ χÎÍÓ ÔÓ-Í˙Ò̇ ‚˙Á‡ÒÚ ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‰‡‚ËÚ ÒË Ó‰ÌËÌË. ÉÓ‚Ó˙Ú Ë ÔÓıӉ͇ڇ Ò‡ ‡·ÌÓÏÌË Ó˘Â ÓÚ Ò‡ÏÓÚÓ Ì‡˜‡ÎÓ. ë‰ ·ÓÎÌËÚ ˜ÂÒÚÓ Ò ÓÚÍË‚‡Ú ÂÏÓˆËÓ̇Î̇ ··ËÎÌÓÒÚ Ë ‡ÙÂÍÚË‚ÌË ÒÏÛ˘ÂÌËfl. ÖÔËÎÂÔÒËflÚ‡ Ì  ı‡‡ÍÚÂ̇ Á‡ ÚÓÁË ÒË̉ÓÏ. èË ‚Ò˘ÍË, ‚Íβ˜ËÚÂÎÌÓ Ë ÔË ‰Âˆ‡Ú‡ Ò Ò¢‡ ÒÔ‡ÒÚ˘̇ ‰ËÁ‡ÚËfl, ÍÓflÚÓ Ò ‚ÎÓ¯‡‚‡ Ò ‚˙Á‡ÒÚÚ‡. ÅÂÁ ËÁÍβ˜ÂÌË Ò Ò¢‡ Ë ‰ËÒÚ‡Î̇ڇ ‡ÏËÓÚÓÙËfl ̇ ˙ˆÂÚÂ Ë Í‡Í‡Ú‡ Ò ÔÓ„ÂÒË‡˘Ó ‚ÎÓ¯‡‚‡ÌÂ Ò ‚˙Á‡ÒÚÚ‡. ñÂ·Â·ÌËÚ ÒÏÛ˘ÂÌËfl Ò‡ Ò˙˘Ó Ó·˘‡ ˜ÂÚ‡ Á‡ ‚Ò˘ÍË Ô‡ˆËÂÌÚË. èË ÔÓ-̇Ô‰̇ÎËÚ ÒÎÛ˜‡Ë Ò Ò¢‡Ú Ë ‰ËÒÙ‡„Ëfl, ‰ËÒÚÓÌ˘ÌË Ë ıÓÂÓ‡ÚÂÚÓÁÌË ‰‚ËÊÂÌËfl ‚ Í‡ÈÌˈËÚÂ. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í ÔÓ͇Á‚‡ ̇΢ËÂÚÓ Ì‡ ÔÓÏÂÌË ‚ ÔÂË‚ÂÌÚËÍÛ·ÌÓÚÓ ·flÎÓ ‚¢ÂÒÚ‚Ó ÙÓÌÚÓÔ‡ËÂÚ‡ÎÌÓ Á‡ ‡ÁÎË͇ ÓÚ Ó·Ë˜‡ÈÌÓ ÌÓχÎ̇ڇ ̇ıӉ͇ ÔË Ôӂ˜ÂÚÓ ÓÒڇ̇ÎË ÙÓÏË Ì‡ çëè (14). äãàçàóçà ïÄêÄäíÖêàëíàäà çÄ ï-ëÇöêáÄçàíÖ îéêåà çÄ çëè ï-Ò‚˙Á‡Ì‡ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ „Â̇, ÍÓ‰Ë‡˘ ÔÓÚÂÓÎËÔˉÌËfl ÔÓÚÂËÌ ÉÂÌ˙Ú Ì‡ SPG2 ÎÓÍÛÒ Â ÚÓÁË Ì‡ ÔÓÚÂÓÎËÔˉÌËfl ÔÓÚÂËÌ. PLP1  ÓÒÌÓ‚ÂÌ ÏËÂÎËÌÓ‚ ÔÓÚÂËÌ, ˜ËËÚÓ ÏÛÚ‡ˆËË ‚Ó‰flÚ ‰Ó ÍÓÏÔÎˈË‡ÌË Ë Á̇˜ËÚÂÎÌÓ ÔÓ-fl‰ÍÓ ‰Ó ˜ËÒÚË ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ᇠÍÓÏÔÎˈË‡ÌËfl ÙÂÌÓÚËÔ Â ı‡‡ÍÚÂÌÓ Ì‡Î˘ËÂÚÓ Ì‡ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, Ò˙˜Âڇ̇ Ò ˆÂ·Â·ÌË ·ÂÎÂÁË Ë ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl. ï‡‡ÍÚÂÌË Ò‡ ËÌÚ‡- Ë ËÌÚÂÙ‡ÏËÎÌË ‚‡ˇˆËË ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÂÊÂÒÚÚ‡ ̇ ÒËÏÔÚÓÏËÚÂ Ë Ì‡Î˘ËÂÚÓ ËÎË ÎËÔÒ‡Ú‡ ̇ ‰ÓÔ˙ÎÌËÚÂÎÌË ·ÂÎÂÁË Í‡ÚÓ ÓÔÚ˘̇ ‡ÚÓÙËfl. óËÒÚËflÚ ÙÂÌÓÚËÔ Â ËÁÍβ˜ËÚÂÎÌÓ fl‰˙Í Ë ‰Ó ÏÓÏÂÌÚ‡  ÓÔËÒ‡Ì Ò‡ÏÓ ÔË ‰‚ ÒÂÏÂÈÒÚ‚‡. éÔ‰ÂÎÂÌ ‚ˉ ÏÛÚ‡ˆËË (̇È-˜ÂÒÚÓ ‰ÛÔÎË͇ˆËË) ‚ PLP1 „Â̇ ‚Ó‰flÚ ‰Ó ‡Á‚ËÚË ̇ ·ÓÎÂÒÚÚ‡ ̇ Pelizaeus-Marzbacher (PMD). ífl Ô‰ÒÚ‡‚Îfl‚‡ ‰ËÒÏËÂÎËÌËÁË‡˘Ó Á‡·ÓÎfl‚‡Ì ̇ ñçë Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ·˙Á‡ ÔÓ„ÂÒËfl ̇ ˆÂ·Â·ÌË Ë ÔË‡ÏˉÌË ·ÂÎÂÁË Ë Ù‡Ú‡ÎÂÌ Í‡È ‚ ‰ÂÚÒÚ‚ÓÚÓ. ᇠ·ÓÎÌËÚÂ Ò ÍÓÏÔÎˈË‡Ì‡ ÙÓχ ̇ çëè, Ò‚˙Á‡Ì‡ Ò SPG2 Ò‡ ı‡‡ÍÚÂÌË ‰Û„ ‚ˉ ÏÛÚ‡ˆËË. í‡Í‡ ‡Á΢ÌËÚ ÏÛÚ‡ˆËË ‚ ÚÓÁË „ÂÌ ÏÓ„‡Ú ‰‡ ‰Ó‚‰‡Ú ‰Ó ‡Á‚ËÚË ̇ ·ÓÎÂÒÚÚ‡ ̇ PMD ËÎË Ì‡ çëè. PLP Ëχ χÎ͇ ËÁÓÙÓχ, ̇˜Â̇ DM-20. à ÔË SPG2, Ë ÔË PMD Â


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÛÒÚ‡ÌÓ‚ÂÌÓ Ì‡Ï‡ÎÂÌË ̇ PLP, ÌÓ Ò‡ÏÓ ÔË PMD Ëχ ̇χÎÂÌË ̇ DM-20. ífl·‚‡ ‰‡ Ò ÓÚ·ÂÎÂÊË Ó·‡˜Â, ˜Â  ÓÔËÒ‡ÌÓ ÒÂÏÂÈÒÚ‚Ó ‚ ÍÓÂÚÓ Ò‡ ‚˙ÁÌËÍ̇ÎË Ë ‰‚‡Ú‡ ÒË̉Óχ, ÔË ÛÒÎÓ‚ËÂ, ˜Â ‚Ò˘ÍËÚ ÏÛ ˜ÎÂÌÓ‚Â ËÏ‡Ú Â‰Ì‡ Ë Ò˙˘‡ PLP ÏÛÚ‡ˆËfl. íÓ‚‡ ̇·Î˛‰ÂÌË ÔÓ͇Á‚‡, ˜Â ÔÓÌflÍÓ„‡ Ì‚ÓÎӄ˘ÌËÚ ÔÓÒÎÂ‰ËˆË ÓÚ PLP ÏÛÚ‡ˆËËÚ Á‡‚ËÒflÚ Ë ÓÚ ÏÓ‰ËÙˈË‡˘Ë Ù‡ÍÚÓË, ÍÓËÚÓ ‚ÂÓflÚÌÓ ‚Íβ˜‚‡Ú ‰ÓÔ˙ÎÌËÚÂÎÌË „ÂÌË Ë Ù‡ÍÚÓË Ì‡ Ò‰‡Ú‡ (6,7,10,17). ï-Ò‚˙Á‡Ì‡ ̇ÒΉÒÚ‚Â̇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl, ‰˙Îʇ˘‡ Ò ÏÛÚ‡ˆËË ‚ „Â̇, ÍÓ‰Ë‡˘ L1CAM (L1 cell adhesion molecule) L1CAM  Ú‡ÌÒÏÂÏ·‡ÌÂÌ „ÎËÍÓÔÓÚÂËÌ, ÍÓÈÚÓ Ò ÂÍÒÔÂÒË‡ ÓÒÌÓ‚ÌÓ ‚ Ì‚ÓÌËÚÂ Ë ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË. à„‡Â Íβ˜Ó‚‡ ÓÎfl ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ñçë ͇ÚÓ ÙÛÌ͈ËËÚ ÏÛ ‚Íβ˜‚‡Ú ÛÔ‡‚ÎÂÌË ̇ Ì‚ËÚÌËfl ‡ÒÚÂÊ ÔÓ ‚ÂÏ ̇ ÂÏ·ËÓ̇ÎÌÓÚÓ ‡Á‚ËÚËÂ Ë Ì‡ Ì‚Ó̇Î̇ڇ ÍÎÂÚ˙˜Ì‡ ÏË„‡ˆËfl. éÔËÒ‡ÌÓ Â ÒÂÏÂÈÒÚ‚Ó Ò ÍÓÏÔÎÂÍÒÂÌ ÙÂÌÓÚËÔ Ò ÚÂÊ͇ ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl Ë ÍÓÌ„ÂÌËÚ‡ÎÌË ÏÛÒÍÛÎÌÓ-ÒÍÂÎÂÚÌË ‡ÌÓχÎËË (ÎËÔÒ‡ ̇ Ï. ÂÍÒÚÂÌÁÓ ı‡ÎÛˆËÒ ÎÓÌ„ÛÒ). ê‡Á΢ÌËÚ ÏÛÚ‡ˆËË Ì‡ L1CAM ‚Ó‰flÚ ‰Ó ‡Á‚ËÚËÂÚÓ ‡ÁÌÓÓ·‡ÁÌË ÒË̉ÓÏË Í‡ÚÓ MASA (ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl, ‡Ù‡ÁËfl, ÒÏÛÚÂ̇ ÔÓıӉ͇ Ë ‡‰‰ÛˆË‡ÌË Ô‡ÎˆË) ÒË̉ÓÏ, ̇ ï-Ò‚˙Á‡Ì‡ ıˉÓˆÂÙ‡ÎËfl Ë Ì‡ ïÒ‚˙Á‡Ì‡ ‡„ÂÌÂÁËfl ̇ ÍÓÔÛÒ Í‡ÎÓÁÛÏ. ᇠÏÛÚ‡ˆËËÚ ‚ ÚÓÁË „ÂÌ Â ı‡‡ÍÚÂ̇ „ÓÎflχ ËÌÚÂ- Ë ËÌÚ‡Ù‡ÏËÎ̇ ‚‡ˇ·ËÎÌÓÒÚ. Ç˙ÁÏÓÊÌÓ Â ‰ÓË ‚ ‡ÏÍËÚ ̇ ‰ÌÓ ÒÂÏÂÈÒÚ‚Ó ‰‡ Ëχ Ô‡ˆËÂÌÚË Ò ‡Á΢ÂÌ ÙÂÌÓÚËÔ. íÂÁË Á‡·ÓÎfl‚‡ÌËfl Ò„‡ Ò ÓÚ̇ÒflÚ Í˙Ï ÍÎËÌ˘ÌËfl ÒË̉ÓÏ CRASH (ÍÓÔÛÒ Í‡ÎÓÁÛÏ ıËÔÓÔ·ÁËfl, ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl, ‡‰‰ÛˆË‡ÌË Ô‡ÎˆË, ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl Ë ıˉÓˆÂÙ‡ÎËfl) (10,17). ÑàîÖêÖçñàÄãçÄ ÑàÄÉçéáÄ ÑÓ „ÓÎflχ ÒÚÂÔÂÌ çëè Ô‰ÒÚ‡‚Îfl‚‡ ‰Ë‡„ÌÓÁ‡ ̇ ËÁÍβ˜‚‡ÌÂÚÓ. àÁÍβ˜ËÚÂÎÌÓ ‚‡ÊÌÓ Â ‰‡ Ò ËÁÍβ˜‡Ú Î˜ËÏËÚ Á‡·ÓÎfl‚‡ÌËfl, ͇ÍÚÓ Ë ÚÂÁË, ˜ËflÚÓ ÔÓ„ÌÓÁ‡ Ò˙˘ÂÒÚ‚ÂÌÓ Ò‡ ÓÚ΢‡‚‡ ÓÚ ïëè. Ç ‰ËÙÂÂ̈ˇÎÌÓ ‰Ë‡„ÌÓÒÚ˘ÂÌ ‡ÒÔÂÍÚ Úfl·‚‡ ‰‡ Ò ËÏ‡Ú Ô‰‚ˉ ÒΉÌËÚ Á‡·ÓÎfl‚‡ÌËfl: ● ëÚÛÍÚÛÌË ‡·ÌÓÏÌÓÒÚË Ì‡ „·‚ÌËfl Ë „˙·Ì‡˜ÂÌ ÏÓÁ˙Í; ● ã‚ÍÓ‰ËÒÚÓÙËË Í‡ÚÓ ‡‰ÂÌÓÏËÂÎÓÌ‚ÓÔ‡ÚËflÚ‡, ·ÓÎÂÒÚÚ‡ ̇ Krabbe Ë ÏÂÚ‡ıÓχÚ˘̇ڇ ΂ÍÓ‰ËÒÚÓÙËfl; ● ÑÂÙˈËÚ Ì‡ ‚ËÚ‡ÏËÌ Ç12; ● åÛÎÚËÔÎÂ̇ ÒÍÎÂÓÁ‡; ● íÓÔ˘ÂÒ͇ ÒÔ‡ÒÚ˘̇ Ô‡‡Ô΄Ëfl; ● L-Dopa ÔÓ‚ÎËfl‚‡˘‡ Ò ‰ËÒÚÓÌËfl; ● ÄÏËÓÚÓÙ˘̇ ·ÚÂ‡Î̇ ÒÍÎÂÓÁ‡; ● è˙‚˘̇ ·ÚÂ‡Î̇ ÒÍÎÂÓÁ‡ (7). áÄäãûóÖçàÖ ïÂÚÂÓ„ÂÌÌÓÒÚÚ‡, „ÂÌÂÚ˘̇ Ë ÙÂÌÓÚËÔ̇,  Íβ˜Ó‚ ·Â΄ ̇ çëè. 燷≇‚‡Ú Ò ‡Á΢Ëfl ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚ هÏËÎËË, ÏÂÊ‰Û Ù‡ÏËÎËËÚÂ, Ò‚˙Á‡ÌË Ò Â‰ËÌ Ë Ò˙˘ ÎÓÍÛÒ, ÏÂÊ‰Û ÒÂÏÂÈÒÚ‚‡Ú‡, Ò‚˙Á‡ÌË Ò ‡Á΢ÌË ÎÓÍÛÒË Ë ‰ÓË ÏÂÊ‰Û ·ÓÎÌËÚ ÓÚ Â‰Ì‡ „ÂÌÂ‡ˆËfl ̇ ‰‡‰Â̇ Ù‡ÏËÎËfl. íÓ‚‡ ÔÓ͇Á‚‡, ˜Â ÓÔ‰ÂÎÂ̇ ÓÎfl Ë„‡flÚ Ë ‰ÓÔ˙ÎÌËÚÂÎÌË ÏÓ‰ËÙˈË‡˘Ë Ù‡ÍÚÓË – „ÂÌÂÚ˘ÌË ËÎË Ù‡ÍÚÓË Ì‡ Ò‰‡Ú‡. ÄÍÓ ‡Á΢ËflÚ‡ ‚˙‚ ‚˙Á‡ÒÚÚ‡ ̇ ‰Â·˛Ú‡ Ì ҇ ÂÁÛÎÚ‡Ú ÓÚ ‡ÌÚˈËÔ‡ˆËfl, ÚÓ ÚÓ„‡‚‡ Ú ̇È-‚ÂÓflÚÌÓ Ò ‰˙ÎÊ‡Ú Ì‡ ‚‡ˇˆËË ‚ ÂÍÒÔÂÒËflÚ‡ ̇ ÏÛÚ‡ÌÚÌËÚ ÔÓÚÂËÌË. éÚÍË‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë „ÂÌË, ‡Ì„‡ÊË‡ÌË Ò ‡Á‚ËÚËÂÚÓ Ì‡ çëè, ·˙ÁÓ ‚Ó‰Ë ‰Ó ÓÙÓÏflÌ ̇ ÌÓ‚‡ ÍÓ̈ÂÔˆËfl Á‡ Ô‡ÚÓÙËÁËÓÎӄ˘ÌËÚ ÏÂı‡ÌËÁÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ëıÓ‰ÒÚ‚ÓÚÓ ‚ ÍÎËÌ˘ÌÓÚÓ ÔÓfl‚ÎÂÌË ̇ ˜ËÒÚËÚ çëè

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Ô˙‚Ó̇˜‡ÎÌÓ Ì‡‚Âʉ‡ ̇ Ô‰ÔÓÎÓÊÂÌËÂÚÓ, ˜Â ‚ ÓÒÌÓ‚‡Ú‡ ËÏ ÎÂÊ‡Ú ·ÎËÁÍË ·ËÓıËÏ˘ÌË ÏÂı‡ÌËÁÏË. á‡Òfl„‡ÌÂÚÓ Ì‡ ‰˙΄ËÚ ÔÓ‚Ó‰ÌËˆË ‚ ñçë Ó·‡˜Â Ò ‰˙ÎÊË Ì‡ ‡Á΢ÌË ·ËÓıËÏ˘ÌË ÒÏÛ˘ÂÌËfl Ë ·ÎËÁÍËÚÚ ÍÎËÌ˘ÌË ˜ÂÚË Ì‡ „ÂÌÂÚ˘ÌÓ ‡Á΢ÌËÚ ˜ËÒÚË ÙÓÏË Ò‡ ÂÁÛÎÚ‡Ú Ì‡ ‰ËÌ Ò‡‚ÌËÚÂÎÌÓ Ó„‡Ì˘ÂÌ ÂÔÂÚÓ‡ ÓÚ ÒËÏÔÚÓÏË ÓÚ ÒÚ‡Ì‡ ̇ ÍÓÚËÍÓÒÔË̇ÎÌËfl Ú‡ÍÚ Ë Á‡‰ÌËÚ ÒÚ˙ηˆË. éÚ ËÁÍβ˜ËÚÂÎ̇ ‚‡ÊÌÓÒÚ Â Ú˙ÒÂÌÂÚÓ Ì‡ ÏÛÚ‡ˆËË ‚ ÒÔ‡ÒÚËÌÓ‚Ëfl „ÂÌ, ÓÒÓ·ÂÌÓ ÔË ‡ÒËÏÔÚÓÏÌË Ô‡ˆËÂÌÚË Ë ÔÂ̇ڇÎ̇ڇ ‰Ë‡„ÌÓÒÚË͇ ÔË PLP-Ò‚˙Á‡ÌËÚ çëè, ÔË SPG1 Ë SPG7. åËÚÓıÓ̉ˇÎÌËÚ ‰ËÒÙÛÌ͈ËË, ÎÂʇ˘Ë ‚ ÓÒÌÓ‚‡Ú‡ ̇ SPG7, „Ë Ô‡‚Ë ÔÓÚÂ̈ˇÎÌÓ ÔÓ-ÔÓ‰‡ÚÎË‚Ë Ì‡ ΘÂÌË ‚ Ò‡‚ÌÂÌËÂ Ò ÓÒڇ̇ÎËÚ ÙÓÏË. éÚÍË‚‡ÌÂÚÓ Ì‡ ‚ÒÂÍË ÌÓ‚ „ÂÌ, ÎÂʇ˘ ‚ ÓÒÌÓ‚‡Ú‡ ̇ ‰‡‰ÂÌ ÚËÔ çëè ÔÓÁ‚ÓÎfl‚‡ ‰ËÂÍÚÌÓ ËÁÒΉ‚‡Ì ̇ ÏÓÎÂÍÛÎflÌËÚ ÏÂı‡ÌËÁÏË Ë ‚Ò˘ÍÓ ÚÓ‚‡ ÔÓÒÚÂÔÂÌÌÓ ÌË ‰Ó·ÎËʇ‚‡ ‰Ó ‡Á·Ë‡Ì ̇ ÔËÓ‰‡Ú‡ ̇ ÚÂÁË Á‡·ÓÎfl‚‡ÌËfl Ë ‚˙‚Âʉ‡ÌÂÚÓ Ì‡ ÔÓÚÂ̈ˇÎÌÓ ÏÓ‰ËÙˈË‡˘Ó ıÓ‰‡ ËÏ Î˜ÂÌËÂ. ãàíÖêÄíìêÄ 1. Behan W, Maia M. Strumpell’s familial spastic paraplegia: genetics and neuropathology. J Neurol Neurosurg Psychiatry 37, 1974, 8-20 2. Burger J, Metzke H, Paternotte C, Schilling F, Hazan J, Reis A. Autosomal dominant spastic parapegia with anticipation maps to a 4-cM interval on chromosome 2p21-p24 in a large German family, Hum Genet, 98, 2004, 371-375 3. Casali C, Valente E, Bertini E, Montagna G, Crisculo C, et al. Clinical and genetical studies in hereditary spastic paraplegia with thin corpus callosum Neurology 62, 2004, 262-268 4. Casari G, DeFusco M, Ciarmatori S et al. Spastic paraplegia and OXPHOS impearment caused by mutations in paraplegin, a nuclear encoded mitohondrial metalloprotease. Cell 93, 1998, 973-983 5. Crosby A, Proukakis Ch. Is the transportation highway the right road for hereditary spastic paraplegia? Am. J. Hum. Genet. 71, 2002, 1009-1016 6. Fink, JK The hereditary spastic paraplegia. Arch Neurol. 60, 2003, 10451049 7. Fink, JK Hereditary spastic paraplegia overview, Gene reviews, 2004, 1-20 8. Hansen J, Durr A, Cournu-Rebeix I, Georgopoulos C, Ang D, Niesen MN, Davoine CS, Brice A, Fontaine B, Gregersen N, Bross P. Hereditari spastic paraplegia SPG13 is associated with mutation in a gene encoding the mitochondrial chaperonin Hsp60. Am J Hum Genet 70, 2002, 1328-32 9. Hazan J, Fonknechten N, Mavel D, et al. Spastin, a novel AAA protein, is altered in the most frequent form of autosomal dominant spastic paraplegia. Nature genetics 23, 1999, 296-303 10. McDermott C, White K, Bushby K, Shaw P. Hereditary spastic paraplegia: a review of new developments. J Neurol Neurosurg Psychiatry 69, 2000, 150-160 11. Molon A, Di Giovanni S, Chen YW, Clarkson PM, Angelini C, Pegoraro E, Hoffman EP Large-scale disruption of microtubule pathways in morphologically normal human spastin muscule. Neurol 62, 2004, 1097-1104 12. Orlacchio A, Kawarai T et al. Hereditary spastic paraplegia. Arch Neurol 61, 2004, 849-855 13. Piemonte F, Casali C, Carrozzo R, Schagger H, Tessa A, Tozzi G, et al. Respiratory chain defects in hereditary spastic paraplegias. Neuromuscular Disorders 11, 2001, 565-569 14. Proukakis Ch, Cross H, Patel H, Patton MA, Valentine A, Crosby AH Troyer syndrome revisited – A clinical and radiological study of a complicated spastic paraplegia. J Neurol 9, 2004, 1105-1110 15. Rainer S, Chai JH, Tokarz D, Nichols RD, Fink J. NIPA1 gene mutations cause autosomal dominant hereditary spastic paraplegia (SPG6). Am J Hum Genet 73, 2003, 967-971 16. Schwartz GA, Liu CN. Hereditary (familial) spastic paraplegia: further clinical and pathological observations. Arch Neurol Psychiatry 75, 1956, 144-162 17. Tallaksen CM, Durr A, Brice A. Recent advancas in hereditary spastic paraplegia. Curr Opin Neurol 14, 2001, 457-463 18. Warner TT, Patel H, Proukakis Ch, Reed JA, McKie L, Wills A, Patton MA, Crosby AH. A clinical, genetic and candidate gene study of Silver syndrome, a complicated form of hereditary spastic paraplegia. J Neurol 9, 1068-1074 19. Yool DA, Edgar JM, Montague P, Malcolm S. The proteolipid protein gene and myelin disorders in man and animal models. Hum Mol Genet 9, 2000, 987992 20. Zhao X, Alvarado D, Rainer S, Lemons R, Hereda P et al. Mutations in newly Identified GTP-ase gene cause autosomal dominant hereditary spastic paraplegia. Nat Genet 29, 2001, 326-331

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÄηÂ̇ Ä̉‚‡ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇” ÛÎ. “ÉÂÓ„Ë ëÓÙËÈÒÍË” ‹1 ÚÂÎ. 9230 462 e-mail: albena_andreeva@abv.bg


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

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é·ÁÓ ÅéãÖëí çÄ WILSON – äãàçàäé-ÉÖçÖíàóçà ÄëèÖäíà Ç. åËı‡ÈÎÓ‚‡, à.í˙Ì‚ ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl

SUMMARY CLINICAL AND GENETIC ASPECTS OF WILSON’S DISEASE V.Mihaylova, I.Tournev Wilson’s Disease is a disorder of copper metabolism with autosomal recessive mode of inheritance resulting in accumulation of copper in the liver and subsequently in other organs, mainly in the central nervous system, kidneys and the cornea. Over 200 different mutations in the responsible gene ATP7B have been reported.The H1069Q mutation is the most common mutation in Central and Eastern Europe. It is considered that the establishment of genotype-phenotype correlations is hampered by the large number of compound heterozygous . On the other hand the patients sharing the same mutations have diverse clinical features suggesting that additional genetic factors may influence the phenotypic presentation of Wilson’s disease. More often than not patients present with neurologic, hepatic or psychiatric symptoms, or some combination of these. Wilson’s disease is unique among genetic diseases in that it can be very effectively treated. The prevention of severe permanent damage depends on early diagnosis and treatment. Key words: ATP7B, genotype-phenotype correlations, genetic modifiers. êÖáûåÖ ÅÓÎÂÒÚÚ‡ ̇ Wilson  ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ Á‡·ÓÎfl‚‡Ì ̇ ωÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï, ‚ ÂÁÛÎÚ‡Ú Ì‡ ÍÓÂÚÓ Ò ̇ÚÛÔ‚‡ ω ‚ ˜ÂÌËfl ‰Ó·, ÒΉ ÚÓ‚‡ Ë ‚ ‰Û„ËÚ Ó„‡ÌË, ÌÓ Ì‡È-‚˜ ‚ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ, ·˙·ˆËÚÂ Ë ÍÓ̇ڇ. éÔËÒ‡ÌË Ò‡ ̇‰ 200 ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ „Â̇ Äíê7Ç, ÍÓËÚÓ Ô˘ËÌfl‚‡Ú Á‡·ÓÎfl‚‡ÌÂÚÓ. åÛÚ‡ˆËflÚ‡ H1069Q  ̇È-˜ÂÒÚ‡ ‚ ñÂÌÚ‡Î̇ Ë àÁÚӘ̇ Ö‚ÓÔ‡. ìÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ „ÂÌÓÚËÔ-ÙÂÌÓÚËÔ ÍÓ·ˆËË Â ÚÛ‰ÌÓ ÔÓ‡‰Ë „ÓÎÂÏËfl ·ÓÈ ÒÎÓÊÌË ıÂÚÂÓÁË„ÓÚË. éÚ ‰Û„‡ ÒÚ‡Ì‡ „ÓÎflÏÓÚÓ ÙÂÌÓÚËÔÌÓ ‡ÁÌÓÓ·‡ÁËÂ, ‰ÓË Ë ÔË Ô‡ˆËÂÌÚË Ò Â‰Ì‡Í‚Ë ÏÛÚ‡ˆËË, Ô‰ÔÓ·„‡ ̇΢ˠ̇ ‰ÓÔ˙ÎÌËÚÂÎÌË „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË, ÍÓËÚÓ ‚ÎËflflÚ ‚˙ıÛ ÙÂÌÓÚËÔ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ç‡È-˜ÂÒÚÓ Ô‡ˆËÂÌÚËÚ Ò Ô‰ÒÚ‡‚flÚ Ò Ì‚ÓÎӄ˘ÌË, ˜ÂÌÓ‰Ó·ÌË Ë ÔÒËı˘ÌË ÒËÏÔÚÓÏË ËÎË ÍÓÏ·Ë̇ˆËfl ÓÚ Úflı. ÅÓÎÂÒÚÚ‡ ̇ Wilson  ÛÌË͇Î̇ Ò‰ „ÂÌÂÚ˘ÌËÚ Á‡·ÓÎfl‚‡ÌËfl ÔÓ ÚÓ‚‡, ˜Â ÏÓÊ ÏÌÓ„Ó ÛÒÔ¯ÌÓ ‰‡ Ò ÎÂÍÛ‚‡. è‰ÓÚ‚‡Úfl‚‡ÌÂÚÓ Ì‡ ÚÂÊÍË ÌÂÓ·‡ÚËÏË Û‚Â‰Ë Á‡‚ËÒË ÓÚ ‡Ì̇ڇ ‰Ë‡„ÌÓÁ‡ Ë Î˜ÂÌËÂ. äβ˜Ó‚Ë ‰ÛÏË: Äíê7Ç, „ÂÌÓÚËÔ-ÙÂÌÓÚËÔ ÍÓ·ˆËË, ÏÓ‰ËÙˈË‡˘Ë „ÂÌË. ᇷÓÎfl‚‡ÌÂÚÓ Â ÓÔËÒ‡ÌÓ Á‡ Ô˙‚Ë Ô˙Ú ÔÂÁ 1912„. ÓÚ S.A.Kinnear Wilson ͇ÚÓ “ÔÓ„ÂÒ˂̇ ÎÂÌÚËÍÛ·̇ ‰Â„ÂÌÂ‡ˆËfl”, Ù‡ÏËÎÌÓ Ì‚ÓÎӄ˘ÌÓ Á‡·ÓÎfl‚‡Ì ÔˉÛÊÂÌÓ ÓÚ ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ (23). èÓ-Í˙ÒÌÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÓÎflÚ‡ ̇ ωڇ ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ͇ÍÚÓ Ë Ì‡˜ËÌ˙Ú Ì‡ Û̇ÒΉfl‚‡ÌÂ. ÅÓÎÂÒÚÚ‡ ̇ Wilson  ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÍÓÂÚÓ Ò ı‡‡ÍÚÂËÁË‡ Ò Û‚Â΢ÂÌÓ Ò˙‰˙ʇÌË ̇

ω ‚ Ó„‡ÌËÁχ, ÍÓflÚÓ Ò ̇ÚÛÔ‚‡ ‚ ‡Á΢ÌËÚ Ó„‡ÌË, ÌÓ Ì‡È-‚˜ ‚ ˜ÂÌËfl ‰Ó·, ÏÓÁ˙͇, ·˙·ˆËÚÂ Ë ӄӂˈ‡Ú‡. èÓÚ˘‡ ̇È-˜ÂÒÚÓ Ò ˜ÂÌÓ‰Ó·ÌË, Ì‚ÓÎӄ˘ÌË Ë ÔÒËıˇÚ˘ÌË ÔÓfl‚Ë. ÅÓÎÂÒÚÚ‡ ̇ Wilson Ò ‰˙ÎÊË Ì‡ ÏÛÚ‡ˆËË ‚ „Â̇, ÍÓÈÚÓ ÍÓ‰Ë‡ ω Ú‡ÌÒÔÓÚË‡˘‡ Äíî‡Á‡ /ATP7B/. íÓÁË ·ÂÎÚ˙Í Ò ÂÍÒÔÂÒË‡ ̇È-‚˜ ‚ ıÂÔ‡ÚÓˆËÚËÚÂ, ‡ ‚ ÔÓχÎ͇ ÒÚÂÔÂÌ Ë ‚ ·˙·ˆËÚÂ, ÏÓÁ˙͇ Ë Ô·ˆÂÌÚ‡Ú‡. ÉÂÌÌËflÚ ÎÓÍÛÒ Â 13q14.3-q21.1. å‰ڇ  ÂÒÂ̈ˇÎÂÌ ÂÎÂÏÂÌÚ Á‡ Ó‡„‡ÌËÁχ, Ú˙È Í‡ÚÓ Â Ò˙ÒÚ‡‚̇ ˜‡ÒÚ Ì‡ ‰ˈ‡ ÂÌÁËÏË: ˆËÚÓıÓÏ ÓÍÒˉ‡Á‡ /ÓÚ ‰Ëı‡ÚÂÎ̇ڇ ‚ÂË„‡/, ÒÛÔÂÓÍÒˉ‰ËÒÏÛÚ‡Á‡ /Û˜‡ÒÚ‚‡ ‚ Ó·ÂÁ‚Âʉ‡ÌÂÚÓ Ì‡ Ò‚Ó·Ó‰ÌËÚ ‡‰Ë͇ÎË/, ÚËÓÁË̇Á‡ /ÓÚ Ï·ÌËÌÓ‚‡Ú‡ ·ËÓÒËÌÚÂÁ‡/, ÎËÁËÎ ÓÍÒˉ‡Á‡ /ÓÚ ÍÓ·„ÂÌÓ‚‡Ú‡ ÒËÌÚÂÁ‡/, ÙÂÓÍÒˉ‡Á‡ /ÌÂÓ·ıÓ‰ËÏ Á‡ ıÓÏÂÓÒÚ‡Á‡Ú‡ ̇ ÊÂÎflÁÓÚÓ/, ‰ÓÔ‡ÏËÌ ·ÂÚ‡-ıˉÓÍÒË·Á‡ /͇ڇÎËÁË‡ Ô‚˙˘‡ÌÂÚÓ Ì‡ ‰ÓÔ‡ÏËÌ ‚ ÌÓ‡‰Â̇ÎËÌ/ Ë ‰. ëÔˆËÙ˘ÌË ·ÂÎÚ˙ˆË ÓÒË„Ûfl‚‡Ú ‚˙ÚÂÍÎÂÚ˙˜ÌËfl Ú‡ÌÒÔÓÚ Ì‡ ωڇ Ë ËÌÍÓÔÓË‡ÌÂÚÓ ÏÛ ‚ ω-Á‡‚ËÒËÏËÚ ÂÌÁËÏË. Ö‰ËÌÒÚ‚ÂÌËflÚ ÏÂı‡ÌËÁ˙Ï Á‡ ωÌÓÚÓ ÂÎËÏËÌË‡Ì  ÂÍÒÍˆËflÚ‡ ‚ ÊÎ˙˜Í‡Ú‡. å‰ڇ ÓÚ ı‡Ì‡Ú‡ Ò ‡·ÒÓ·Ë‡ ‚ Ú˙ÌÍÓÚÓ ˜Â‚Ó. óÂÌËflÚ ‰Ó·  ÓÒÌÓ‚ÌËflÚ Ó„‡Ì, ÍÓÈÚÓ ÒÍ·‰Ë‡ ω. å‰ڇ ̇‚ÎËÁ‡ ‚ ıÂÔ‡ÚÓˆËÚ‡ ˜ÂÁ ÏÂÏ·‡ÌÂÌ Ú‡ÌÒÔÓÚÂ /CTR1/ (27). èË ÙËÁËÓÎӄ˘ÌË ÛÒÎÓ‚Ëfl Ò˙‰˙ʇÌËÂÚÓ Ì‡ ҂ӷӉ̇ڇ ω ËÌÚ‡ˆÂÎÛ·ÌÓ Â Ó„‡Ì˘ÂÌÓ. ëΉ ̇‚ÎËÁ‡Ì ‚ ıÂÔ‡ÚÓˆËÚ‡ ωڇ Ò ҂˙Á‚‡ Ò „ÛÔ‡ ·ÂÎÚ˙ˆË ̇˜ÂÌË ÏÂÚ‡ÎÓ˜ÂÔÂÓÌË, ÍÓËÚÓ fl ÔÂı‚˙ÎflÚ ÏÂÊ‰Û ÍÎÂÚ˙˜ÌËÚ ÍÓÏÔ‡ÚÏÂÌÚË (2,27). Atox1 ˜ÂÔÂÓÌ ‰ËÂÍÚÌÓ ‚Á‡ËÏÓ‰ÂÈÒÚ‚‡ Ò, ATP7B (Wilson ATPase). Atox1 Ò˙‰˙ʇ ‰ÌÓ ÍÓÔË ÓÚ Ï‰-Ò‚˙Á‚‡˘Ëfl ÏÓÚË‚ åïëïïë, ÍÓÈÚÓ Ò ̇ÏË‡ Ò˙˘Ó Ë ‚ ‡ÏËÌÓÚÂÏË̇ÎÌËfl Û˜‡ÒÚ˙Í Ì‡ Äíê7Ç. Ç ˆËÚÓÔ·Áχڇ ̇ ıÂÔ‡ÚÓˆËÚ‡ ωڇ  ҂˙Á‡Ì‡ Ò˙˘Ó Ë Ò ÔÓÚÂËÌË ·Ó„‡ÚË Ì‡ ˆËÒÚÂËÌ - ÏÂÚ‡ÎÓÚËÓÌÂËÌË. í Ô‰ԇÁ‚‡Ú ÍÎÂÚ͇ڇ ÓÚ Ï‰̇ ÚÓÍÒ˘ÌÓÒÚ ÔË Ò‚˙ı̇ÚÓ‚‡‚‡ÌÂ Ò Ï‰. Äíê7Ç Â Ï‰ Ú‡ÒÔÓÚË‡˘‡ ê-ÚËÔ Äíî‡Á‡ ÎÓ͇ÎËÁË‡Ì‡ ‚ ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË, ÍÓflÚÓ Ú‡ÌÒÔÓÚË‡ ω ͇ÍÚÓ Á‡ ËÌÍÓÔÓË‡Ì ‚ ‡ÔÓˆÂÛÎÓÔ·ÁÏËÌ, ڇ͇ Ë Á‡ ÂÍÒÍÂÚË‡Ì ‚ ÊÎ˙˜Í‡Ú‡ (2). äÓ„‡ÚÓ ÍÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ ωڇ ‚ ıÂÔ‡ÚÓˆËÚ‡ Ò Ôӂ˯Ë, ATP7B ‰ËÒÓˆËË‡ ÓÚ ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË Ë ÔÓÔ‡‰‡ ‚ ˆËÚÓÁÓ· ·ÎËÁÓ ‰Ó ͇̇ÎËÍÛ·̇ڇ ÏÂÏ·‡Ì‡, Í˙‰ÂÚÓ Ï‰ڇ Ò ̇ÚÛÔ‚‡ ‚˙‚ ‚ÂÁËÍÛÎË ÔÂ‰Ë ‰‡ Ò ÂÍÒÍÂÚË‡ ‚ ÊÎ˙˜Í‡Ú‡ (2). ëΉ ·ËΡ̇ڇ ÂÍÒÍˆËfl ÌË‚ÓÚÓ Ì‡ ωڇ ÒÔ‡‰‡ Ë Äíê7Ç Ò ‚˙˘‡ Ó·‡ÚÌÓ ‚ ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË. èË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚ Ì‡ Wilson ÎËÔÒ‡Ú‡ ̇ ÙÛÌ͈ËÓÌË‡˘ ·ÂÎÚ˙Í Äíê7Ç ‚Ó‰Ë ‰Ó ÎËÔÒ‡ ̇ Ú‡ÌÒÔÓÚË‡Ì ̇ ω Í˙Ï ‡Ô‡‡Ú‡ ̇ ÉÓΉÊË Ë ËÌÍÓÔÓË‡Ì ‚ ‡ÔÓˆÂÛÎÓÔ·ÁÏË̇ (2,27). Ç ÚÓÁË ÒÎÛ˜‡È ÔÓ‰˙Îʇ‚‡ ‰‡ Ò ÒËÌÚÂÁË‡ Ë ÒÂÍÂÚË‡ ‡ÔÓˆÂÛÎÓÔ·ÁÏËÌ, ÍÓÈÚÓ Ó·‡˜Â ÔÓ‡‰Ë ÎËÔÒ‡Ú‡ ̇ ω  Î˯ÂÌ ÓÚ ÙÂÓÍÒˉ‡Á̇ ‡ÍÚË‚ÌÓÒÚ Ë Ëχ ÔÓÎÛÊË‚ÓÚ 4-5 ˜‡Ò‡. ᇠÒ‡‚ÌÂÌˠω-Ò˙‰˙ʇ˘ËflÚ ‡ÍÚË‚ÂÌ ÔÓÚÂËÌ /ıÓÎÓˆÂÛÎÓÔ·ÁÏËÌ/ Ëχ ÔÓÎÛÊË‚ÓÚ 4-5 ‰ÌË (27). ÖÚÓ Á‡˘Ó ÌËÒÍÓÚÓ ÌË‚Ó Ì‡ ÒÂÛÏÌËfl ˆÂÛÎÓÔ·ÁÏËÌ Â Ò ‰Ë‡„ÌÓÒÚ˘̇ ÒÚÓÈÌÓÒÚ ÔË Á‡·ÓÎfl‚‡ÌÂÚÓ (2,27). ÉÂÌ˙Ú MURR1, ÏÛÚ‡ˆËË ‚ ÍÓÈÚÓ Ô‰ËÁ‚ËÍ‚‡Ú ω̇ ÚÓÍÒËÍÓÁ‡ ÔË Å‰ÎËÌ„ÚÓÌÓ‚ËÚ ÚÂËÂË, ÍÓ‰Ë‡ ‰ËÌ Ï‡Î˙Í ˆËÚÓÁÓÎÂÌ ·ÂÎÚ˙Í ‚Á‡ËÏÓ‰ÂÈÒÚ‚‡˘ ËÌ ‚ËÚÓ Ò


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

Äíê7Ç, ÍÓÈÚÓ Â ÌÂÓ·ıÓ‰ËÏ Á‡ Í‡ÈÌËfl ÂÚ‡Ô Ì‡ ‚ÂÁËÍÛ·ÂÌ Ï‰ÂÌ Ú‡ÌÒÔÓÚ Ë ·ËΡ̇ ÂÍÒÍˆËfl (2,28). Äíê7Ç Ëχ ‰‚‡ „·‚ÌË ÙÛÌ͈ËÓ̇ÎÌË ‰ÓÏÂ̇: ω҂˙Á‚‡˘ Ë Äíî-Ò‚˙Á‚‡˘. SEPHL ÏÓÚË‚ ÓÚ ÄíîÒ‚˙Á‚‡˘Ëfl ‰ÓÏÂÌ Â ÏflÒÚÓÚÓ Ì‡ ËÁfl‚‡ ̇ ̇È-˜ÂÒÚÓ Ò¢‡Ì‡Ú‡ Ò‰ ‚ÓÔÂȈËÚ missense ÏÛÚ‡ˆËfl H1069Q, ÔË ÍÓÂÚÓ ÒÚ‡‚‡ Á‡Ïfl̇ ̇ ıËÒÚˉË̇ Ò „ÎÛÚ‡ÏËÌ, ‚Ó‰Â˘Ó ‰Ó ̇Û¯ÂÌÓ ÙÛÌ͈ËÓÌË‡Ì ̇ Äíê7Ç (1). ᇷÓÎfl‚‡ÌÂÚÓ Ò Ò¢‡ ̇‚ÒflÍ˙‰Â ÔÓ Ò‚ÂÚ‡ Ò ˜ÂÒÚÓÚ‡ 1 ̇ 30 000 Ë̉˂ˉË, ͇ÚÓ ‚ ÌflÍÓË ÔÓÔÛ·ˆËË Á‡·ÓÎfl‚‡ÌÂÚÓ Ò Ò¢‡ ÔÓ-˜ÂÒÚÓ. Ç ë‡‰ËÌËfl ˜ÂÒÚÓÚ‡Ú‡  1:7000 (2,4). óÂÒÚÓÚ‡Ú‡ ̇ ıÂÚÂÓÁË„ÓÚÌËÚ ÌÓÒËÚÂÎË Â 1:100 (2). Ç Á‡Ú‚ÓÂÌË ÔÓÔÛ·ˆËË Ò Í˙‚ÌÓÓ‰ÒÚ‚ÂÌË ·‡ÍÓ‚Â ˜ÂÒÚÓÚ‡Ú‡ ̇ ÌÓÒËÚÂÎËÚ  ÔÓ-„ÓÎflχ – ̇Ô. ‚ Å˙΄‡Ëfl Ò‰ ÓÏÒÍÓÚÓ Ì‡ÒÂÎÂÌË ÙÂ‰ʇÎËË (í˙Ì‚ Ë Ò˙Ú.). àÁ‚ÂÒÚÌË Ò‡ ̇‰ 200 ÏÛÚ‡ˆËË ‚ „Â̇ Äíê7Ç. Ç Ò‚Â̇, ˆÂÌÚ‡Î̇ Ë ËÁÚӘ̇ Ö‚ÓÔ‡ ̇È-˜ÂÒÚËÚ ҇: H1069Q /˜ÂÒÚÓÚ‡ ̇ ‡Î· 43,5%/, ÏÛÚ‡ˆËË Ì‡ ÂÍÁÓÌ 8 /6,8%/, 3400delC /3%/ Ë P969Q /1,6%/ (1). Ç ‰Û„Ë Ó·Î‡ÒÚË Ò ÔÓ-‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ Ò Ò¢‡Ú ‰Û„Ë ÏÛÚ‡ˆËË, ̇ÔËÏÂ Á‡ äËÚ‡È Ë äÓÂfl ÏÛÚ‡ˆËflÚ‡ R778L Â Ò ˜ÂÒÚÓÚ‡ Ò˙ÓÚ‚ÂÚÌÓ 37,7% Ë 37,9% (24,25). éÍÓÎÓ ÔÓÎÓ‚Ë̇ڇ ÓÚ ÚÂÁË ÏÛÚ‡ˆËË Ò‡ missense ÏÛÚ‡ˆËË, ‚ ÂÁÛÎÚ‡Ú Â‰Ì‡ ‡ÏËÌÓÍËÒÂÎË̇ Ò Á‡ÏÂÌfl Ò ‰Û„‡, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÔÓÏfl̇ ‚˙‚ ÙÛÌ͈ËflÚ‡ ̇ ·ÂÎÚ˙͇ ATP7B. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË Ò‡ ÒÎÓÊÌË ıÂÚÂÓÁË„ÓÚË, Ú. ËÏ‡Ú ‡Á΢ÌË ÏÛÚ‡ˆËË Ì‡ ‰‚‡Ú‡ ‡Î·. íÓ‚‡ Á‡ÚÛ‰Ìfl‚‡ „ÂÌÓÚËÔ-ÙÂÌÓÚËÔ ÍÓ·ˆËËÚ (2). ç‡ÒÍÓÓ Ôӂ‰ÂÌ ÏÂÚ‡‡Ì‡ÎËÁ ̇ 577 Ô‡ˆËÂÌÚË ÔÓ͇Á‡, ˜Â ıÓÏÓÁË„ÓÚËÚ ËÎË ıÂÚÂÓÁË„ÓÚËÚ ÔÓ H1069Q ËÏ‡Ú ÔÓ-˜ÂÒÚÓ Ì‚ÓÎӄ˘ÌË ÔÓfl‚Ë Ë ÔÓ-Í˙Ò̇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚ ·ÂÁ ç1069Q ÏÛÚ‡ˆËË.(30) Ç˙ÔÂÍË ÚÓ‚‡ ‰ÓË Ë Ô‡ˆËÂÌÚË ıÓÏÓÁË„ÓÚË ÔÓ Â‰Ì‡ Ë Ò˙˘‡ ÏÛÚ‡ˆËfl Ò ‡Á΢‡‚‡Ú ÔÓ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ, ÍÎËÌ˘ÌË ÔÓfl‚Ë, ·ËÓıËÏ˘ÌË ÔÓ͇Á‡ÚÂÎË Ë ÚÂÊÂÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (2). ëΉӂ‡ÚÂÎÌÓ ‰ÓÔ˙ÎÌËÚÂÎÌË „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË /ÏÓ‰ËÙˈË‡˘Ë „ÂÌË/ Ë Ù‡ÍÚÓË Ì‡ ÓÍÓÎ̇ڇ Ò‰‡ Ó·ÛÒ·‚flÚ „ÓÎflÏÓÚÓ ÙÂÌÓÚËÔÌÓ ‡ÁÌÓÓ·‡ÁË ̇ ·ÓÎÂÒÚÚ‡. ÉÂÌÓÚËÔ˙Ú ÄÓÖ ÔÓ‚ÎËfl‚‡ ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ Ì‚ÓÎӄ˘ÌË Ë ˜ÂÌÓ‰Ó·ÌË ÒËÏÔÚÓÏË ÔË ıÓÏÓÁË„ÓÚËÚ ÔÓ H1069Q – Ô‡ˆËÂÌÚË Ò ÄÓ Ö ?3/3 ËÏ‡Ú Á̇˜ËÚÂÎÌÓ ÔÓ-Í˙ÒÌÓ Ì‡˜‡ÎÓ (ÓÚ 19 ‰Ó 31 „Ó‰ËÌË) ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË Ò ÄÓÖ ?3/4 (ÓÚ 17 ‰Ó 23 „Ó‰ËÌË) (29). ç‡ÒÍÓÓ ÔÛ·ÎËÍÛ‚‡ÌÓ ÔÓÛ˜‚‡Ì ÔÓ͇Á‚‡, ˜Â ıÂÚÂÓÁË„ÓÚÌÓÚÓ Ò˙ÒÚÓflÌË GAT/GAC ̇ ÍÓ‰ÓÌ Asn-164 ̇ „Â̇ MURR1 /„ÂÌ, ÏÛÚ‡ˆËË ‚ ÍÓÈÚÓ Ô‰ËÁ‚ËÍ‚‡Ú ωÌÓ Ì‡ÚÛÔ‚‡Ì ‚ ˜ÂÌËfl ‰Ó· ÔË Å‰ÎËÌ„Ú˙ÌÓ‚Ë ÚÂËÂË/ ‚Ó‰Ë ‰Ó ÔÓ-‡ÌÌÓ Ï‡ÌËÙÂÒÚË‡Ì ̇ ·ÓÎÂÒÚÚ‡ ̇ Wilson, ÍÓÂÚÓ Â ÓÒÓ·ÂÌÓ ËÁ‡ÁÂÌÓ ÔË ıÓÏÓÁË„ÓÚËÚ ÔÓ H1069Q Ò Ô˙‚Ó̇˜‡Î̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ /13 „Ó‰ËÌË ÔÓ-‡ÌÌÓ Ì‡˜‡ÎÓ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚ ıÓÏÓÁË„ÓÚË ÔÓ GAT/GAT Asn-164/ (28). ᇄ۷‡Ú‡ ̇ ÙÛÌ͈Ëfl ̇ Äíê7Ç Ì‡Û¯‡‚‡ ÒËÌÚÂÁ‡Ú‡ ̇ ıÓÎÓˆÂÛÎÓÔ·ÁÏËÌ Ë ·ËΡ̇ڇ ω̇ ÂÍÒÍˆËfl Ò ÔÓÒΉ‚‡˘Ó ̇ÚÛÔ‚‡Ì ̇ ω ‚ ıÂÔ‡ÚÓˆËÚËÚÂ, ωωËË‡Ì‡ ÓÍÒˉ‡Ú˂̇ Û‚‰‡, ÍÎÂÚ˙˜Ì‡ ÒÏ˙Ú, ÓÒ‚Ó·Óʉ‡‚‡Ì ̇ ω ‚ Ô·Áχڇ Ë ÓÚ·„‡Ì ‚ Ôӂ˜ÂÚÓ Ú˙͇ÌË (2). äÎËÌ˘ÂÒÍË Á‡·ÓÎfl‚‡ÌÂÚÓ ÏÓÊ ‰‡ Ò ÔÓfl‚Ë Ò ‡ÁÌÓÓ·‡ÁÌË ÒËÏÔÚÓÏË, ÌÓ Ì‡È-˜ÂÒÚÓ Ò¢‡ÌË Ò‡ ˜ÂÌÓ‰Ó·ÌËÚÂ, Ì‚ÓÎӄ˘ÌË Ë ÔÒËıˇÚ˘ÌË ÔÓfl‚Ë. éÍÓÎÓ 45% ÓÚ ·ÓÎÌËÚ ËÏ‡Ú Ô˙‚Ó̇˜‡ÎÌÓ ˜ÂÌÓ‰Ó·ÌÓ Á‡Òfl„‡ÌÂ, 35% - Ì‚ÓÎӄ˘ÌÓ, ‡ 10% - ÔÒËı˘ÌË ÔÓfl‚Ë. èË ÓÒڇ̇ÎËÚ 10% ̇˜‡ÎÓÚÓ ‚Íβ˜‚‡ ıÂÏÓÎËÚ˘̇ ‡ÌÂÏËfl, ËÍÚÂ, ͇‰ËÓÏËÓÔ‡ÚËfl Ë ‰Û„Ë ÔÓ-‰ÍË ÒËÏÔÚÓÏË (2). çËÚÓ Â‰ËÌ ÒËÏÔÚÓÏ Ì  ԇÚÓ„ÌÓÏÓÌ˘ÂÌ.

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èË ‰Âˆ‡Ú‡ ̇È-˜ÂÒÚÓ Ì‡˜‡Î̇ ËÁfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò‡ ˜ÂÌÓ‰Ó·ÌËÚ ÔÓfl‚Ë: Ó·ËÍÌÓ‚ÂÌÓ Ì‡ 8-16 „Ӊ˯̇ ‚˙Á‡ÒÚ, Ì‚ÓÎӄ˘ÌËÚÂ Ë ÔÒËı˘ÌË ÒËÏÔÚÓÏË Í‡ÚÓ Ì‡˜‡Î̇ ËÁfl‚‡ Ó·ËÍÌÓ‚ÂÌÓ Ì Ò Ò¢‡Ú ÔÂ‰Ë 12 „Ó‰ËÌË (5). Ç˙ÔÂÍË ˜Â ̇È-˜ÂÒÚÓ ‚˙Á‡ÒÚÚ‡ ̇ ËÁfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÏÂÊ‰Û 8 Ë 50 „Ó‰ËÌË, ÓÔËÒ‡ÌË Ò‡ ÒÎÛ˜‡Ë ̇ 55-62 „Ӊ˯̇ ‚˙Á‡ÒÚ ÔÓ ‚ÂÏ ̇ ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡ (6,7), ‡ ̇È-χÎÍËfl Ô‡ˆËÂÚ Ò ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ ‰˙Îʇ˘‡ Ò ̇ ÚÓ‚‡ Á‡·ÓÎfl‚‡Ì  3 „Ӊ˯ÂÌ (8). íÓ‚‡ ÔÓ͇Á‚‡, ˜Â ‚˙Á‡ÒÚÚ‡ Ì ·Ë‚‡ ‰‡ ËÁÍβ˜‚‡ ÚÓ‚‡ Á‡·ÓÎfl‚‡Ì ÔË Ì‡Î˘ËÂÚÓ Ì‡ Ò˙ÓÚ‚ÂÚÌËÚ ÍÎËÌ˘ÌË ÒËÏÔÚÓÏË Ë ·ËÓıËÏ˘ÌË ÍÓÌÒÚ·ˆËË. óÂÌÓ‰Ó·ÌËÚ ÔÓfl‚Ë Ò‡ ‡ÁÌÓÓ·‡ÁÌË: ÓÚ ‡ÒËÏÔÚÓÏ̇ ıÂÔ‡ÚÓÏ„‡ÎËfl ËÎË ‡·ÌÓÏÌË Ú‡ÌÒ‡ÏË̇ÁË ‰Ó ÙÛÎÏË̇ÌÚ̇ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (8). éÒÚËflÚ ıÂÔ‡ÚËÚ ÔË ·ÓÎÂÒÚÚ‡ ̇ Wilson  ÌÂ‡Á΢ËÏ ÓÚ ÓÒÚËfl ‚ËÛÒÂÌ, ÚÓÍÒ˘ÂÌ Ë ‡‚ÚÓËÏÛÌÂÌ ıÂÔ‡ÚËÚ. çflÍÓË Ô‡ˆËÂÌÚË ËÏ‡Ú Ò‡ÏÓ ·ËÓıËÏ˘ÌË ‡·ÌÓÏÌÓÒÚË ËÎË ÒÚ‡ÚÓÁ̇ Ú‡ÌÒÙÓχˆËfl ÔË ˜ÂÌÓ‰ӷ̇ ·ËÓÔÒËfl (8). åÌÓ„Ó Ô‡ˆËÂÌÚË ËÏ‡Ú ÒËÏÔÚÓÏË Ì‡ ıÓÌ˘ÂÌ ıÂÔ‡ÚËÚ Ë ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ /ÍÓÏÔÂÌÒË‡Ì‡ ËÎË ‰ÂÍÓÏÔÂÌÒË‡Ì‡/ ËÎË ËÁÓÎË‡Ì‡ ÒÔÎÂÌÓÏ„‡ÎËfl Ò ÔÓÚ‡Î̇ ıËÔÂÚÓÌËfl (8). óÂÌÓ‰ӷ̇ڇ ·ËÓÔÒËfl ÔÓ͇Á‚‡ ıÓÌ˘ÂÌ ıÂÔ‡ÚËÚ ËÎË Ì‡Ô‰̇· ˆËÓÁ‡, ÌÓ ‰Ë‡„ÌÓÁ‡Ú‡ ÏÓÊ ‰‡ Ì Ò ÔÓÒÚ‡‚Ë, ‡ÍÓ Ì Ò ËÁÏÂË Ò˙‰˙ʇÌËÂÚÓ Ì‡ ωڇ (1). ÅÓÎÂÒÚÚ‡ ̇ Wilson ÏÓÊ ‰‡ Ò Ô‰ÒÚ‡‚Ë Í‡ÚÓ ÙÛÎÏË̇ÌÚ̇ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. ÉÓÎÂÏË ÍÓ΢ÂÒÚ‚‡ ÒÍ·‰Ë‡Ì‡ ω Ò ÓÒ‚Ó·Óʉ‡‚‡Ú ÓÚ ÌÂÍÓÚËÁË‡ÎËÚ ıÂÔ‡ÚÓˆËÚË Ë Ë̉ۈË‡Ú ÚÂÊ͇ ıÂÏÓÎËÚ˘̇ ‡ÌÂÏËfl, ÍÓflÚÓ ÛÒÎÓÊÌfl‚‡ ÓÒÚÓÚÓ ˜ÂÌÓ‰Ó·ÌÓ Û‚Âʉ‡ÌÂ. Ç˙ÔÂÍË ˜Â ·ÓÎÂÒÚÚ‡ ̇ Wilson  fl‰Í‡, Úfl ÓÚ„Ó‚‡fl Á‡ 6-12% ÓÚ ÒÎÛ˜‡ËÚ ËÁËÒÍ‚‡˘Ë ÒÔ¯̇ ˜ÂÌÓ‰ӷ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl (1). 燂ÂÏÂÌ̇ڇ ‰Ë‡„ÌÓÁ‡ ÏÓÊ ‰‡  Úۉ̇. ì‚Â΢ÂÌËÂÚÓ Ì‡ ÒÂÛÏÌËÚ ‡ÏËÌÓÚ‡ÌÒÙÂ‡ÁË Â ÔÓ-χÎÍÓ ÓÚ 10 Ô˙ÚË - ÔÓ-ÌËÒÍÓ ÓÚ ÚÓ‚‡ ÔË ÙÛÎÏË̇ÌÚÂÌ ıÂÔ‡ÚËÚ Ò ‰Û„‡ ÂÚËÓÎÓ„Ëfl. äÓÏ·Ë̇ˆËflÚ‡ ÓÚ ‡ÌÂÏËfl, ËÁ‡ÁÂ̇ Ê˙ÎÚÂÌˈ‡ Ë ÓÚÌÓÒËÚÂÎÌÓ ÌËÒÍË Ú‡ÌÒ‡ÏË̇ÁË ÔË Ï·‰ Ô‡ˆËÂÌÚ ‚Ë̇„Ë Úfl·‚‡ ‰‡ ̇ÒÓ˜Ë Í˙Ï ·ÓÎÂÒÚ Ì‡ Wilson. ç‡È-‰Ó·ËflÚ ‰Ë‡„ÌÓÒÚ˘ÂÌ ÚÂÒÚ Â ÓÔ‰ÂÎflÌÂÚÓ Ì‡ ÍÓ΢ÂÒÚ‚ÓÚÓ Ì‡ ωڇ ‚ ˜ÂÌËfl ‰Ó· (8). ç‚ÓÎӄ˘ÌËÚ ÒËÏÔÚÓÏË Ò‡ Ô‰ËÏÌÓ Ï‡ÎÍÓÏÓÁ˙˜ÌË Ë ÂÍÒÚ‡ÔË‡ÏˉÌË. í Ò ‡Á‚Ë‚‡Ú ̇È-˜ÂÒÚÓ ÏÂÊ‰Û 15 Ë 20 „Ӊ˯̇ ‚˙Á‡ÒÚ, ‚˙ÔÂÍË ˜Â Ëχ ÓÔËÒ‡ÌË ÒÎÛ˜‡Ë Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ 45-55 „Ó‰ËÌË (1). ç‡È-˜ÂÒÚ‡Ú‡ Ì‚ÓÎӄ˘̇ ÔÓfl‚‡  ÚÂÏÓ˙Ú – ÔË ÓÍÓÎÓ 50% ÓÚ Ô‡ˆËÂÌÚËÚÂ. íÓÈ ÏÓÊ ‰‡ ·˙‰Â ̇ ÔÓÍÓÈ, ÍËÌÂÚ˘ÂÌ ËÎË ÔÓÒÚÛ‡ÎÂÌ (9). åÌÓ„Ó ı‡‡ÍÚÂÂÌ Â ÔÓÒÚÛ‡ÎÌËflÚ ÚÂÏÓ ‚ „ÓÌËÚ Í‡ÈÌËˆË Í‡ÚÓ “‡Áχı‚‡Ì ̇ ÍË· ̇ ÔÚˈ‡”. 燷≇‚‡Ú Ò ԇÍËÌÒÓÌÓ‚Ë ÔÓfl‚Ë – ˄ˉÌÓÒÚ, ·‡‰ËÍËÌÂÁËfl Ë Ï‡ÒÍӂˉÂÌ Ù‡ˆËÂÒ. óÂÒÚÓ Ò¢‡ÌË Ò‡ ‰ËÒÙ‡„ËflÚ‡, ‰ËÁ‡ÚËflÚ‡ Ë ËÁÚ˘‡ÌÂÚÓ Ì‡ ÒβÌ͇ ÓÚ ÛÒÚ‡Ú‡ (8,9). ÑÛ„Ë ÔÓfl‚Ë Ò‡ ‰ËÒÚÓÌËflÚ‡, ÓÒÓ·ÂÌÓ Ì‡ Îˈ‚‡Ú‡ Ë Ù‡Ë̄‡Î̇ ÏÛÒÍÛ·ÚÛ‡ Ë ıÓÂflÚ‡ (5,9). èÓÌflÍÓ„‡ ÔÂӷ·‰‡‚‡Ú χÎÍÓÏÓÁ˙˜ÌËÚ ÔÓfl‚Ë: ‡Ú‡ÍÒËfl, Ò͇̉Ë‡Ì „Ó‚Ó, ËÌÚÂ̈ËÓÌÂÌ ÚÂÏÓ, ‰Ë҉ˇ‰ÓıÓÍËÌÂÁËfl – Ú.̇. “ÔÒ‚‰ÓÒÍÎÂÓÚ˘ÂÌ ÚËÔ” (Walsh 1984), ̇ÔÓ‰Ó·fl‚‡˘ ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡. èË‡ÏˉÌË ·ÂÎÂÁË Ò Ò¢‡Ú fl‰ÍÓ. ÉÂÌÂ‡ÎËÁË‡ÌË Ë Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË fl‰ÍÓ ÏÓ„‡Ú ‰‡ Ò ̇·Î˛‰‡‚‡Ú ͇ÚÓ ‡Ì̇ Ë Í˙Ò̇ ËÁfl‚‡ ̇ ·ÓÎÂÒÚÚ‡ (10,11). éÔËÒ‡ÌË Ò‡ ̇Û¯ÂÌËfl ‚ ÓÍÛÎÓÏÓÚÓË͇ڇ – Á‡·‡‚ÂÌË Ò‡Í‡‰Ë˜ÌË ‰‚ËÊÂÌËfl; ÏË„ÂÌÓÁÌÓ „·‚Ó·ÓÎËÂ Ë ËÌÒÓÏÌËfl (8). 臈ËÂÌÚË Ò Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË Ò˙˘Ó ÏÓ„‡Ú ‰‡ ËÏ‡Ú Á̇˜ËÚÂÎÌÓ ˜ÂÌÓ‰Ó·ÌÓ Û‚Âʉ‡ÌÂ. ÄÍÓ Ò ÓÒ˙˘ÂÒÚ‚Ë ˜ÂÌÓ‰ӷ̇ ·ËÓÔÒËfl ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò Ì‚Ó-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

Îӄ˘̇ ÒËÏÔÚÓχÚË͇, ÔË 38,7% Ò ÓÚÍË‚‡ ˆËÓÁ‡, ‡ ÔË 50% - Ò‡ÏÓ ÏËÌËχÎÌË ˜ÂÌÓ‰Ó·ÌË ÔÓÏÂÌË (1). èÒËı˘̇ڇ ÒËÏÔÚÓχÚË͇ ‚Íβ˜‚‡ ‡Á΢ÌË Î˘ÌÓÒÚÓ‚Ë ÔÓÏÂÌË, ‡Á‰‡ÁÌËÚÂÎÌÓÒÚ, ÂÏÓˆËÓ̇Î̇ ··ËÎÌÓÒÚ, Úۉ̇ ÍÓ̈ÂÌÚ‡ˆËfl, ÔÒËıÓÁË, χÌËfl, ‰ÂÔÂÒËfl, ¯ËÁÓÙÂÌËfl (5). ç‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ÓÚÍË‚‡ ̇Û¯ÂÌË ‚ ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË Ë ‡·ÓÚ̇ڇ Ô‡ÏÂÚ ÔË Ô‡ˆËÂÌÚË Ò Ì‚ÓÎӄ˘̇ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ è‡ˆËÂÌÚË Ì‚ÓÎӄ˘ÌÓ ‡ÒËÏÔÚÓÏÌË ÌflÏ‡Ú ÔÓÏÂÌË ‚ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË, ÍÓÂÚÓ ÔÓ͇Á‚‡ Á̇˜ÂÌËÂÚÓ ÔÓ-ÒÍÓÓ Ì‡ ‰ËÂÍÚ̇ڇ Û‚‰‡ ̇ ÏÓÁ˙͇ ÓÚ Ï‰ÌËfl ‰ÂÔÓÁËÚ, ÓÚÍÓÎÍÓÚÓ Ì‡ ıÂÔ‡Ú‡Î̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (16). ç‚ÓÓÙÚ‡ÎÏÓÎӄ˘ÌË ÒËÏÔÚÓÏË: ÔÓ˜ÚË ‚Ë̇„Ë /‚ 95%/ ÔË ÒÎÛ˜‡ËÚÂ Ò Ì‚ÓÎӄ˘ÌË ÔÓfl‚Ë Ò ÓÚÍË‚‡ Ô˙ÒÚÂÌ Ì‡ Kayser-Fliesher (1). Ç 50-60% ÚÓÈ Ò ÓÚÍË‚‡ Ë ÔË ÒÎÛ˜‡Ë Ò‡ÏÓ Ò ˜ÂÌÓ‰Ó·ÌË ÔÓfl‚Ë (1,5,8,9), ‡ ‚ 10% ÔË ‡ÒËÏÔÚÓÏÌË ÒË·ÒË (1). Ñ˙ÎÊË Ò ̇ ÓÚ·„‡ÌÂÚÓ Ì‡ ω ‚ ÑÂÒˆÂÏÂÚÓ‚‡Ú‡ ÏÂÏ·‡Ì‡. è˙ÒÚÂÌËÚ ̇ KayserFliesher Ì ҇ ̇Ô˙ÎÌÓ ÒÔˆËÙ˘ÌË, Ú˙È Í‡ÚÓ Ò ̇·Î˛‰‡‚‡Ú ÔË ‰Û„Ë ˜ÂÌÓ‰Ó·ÌË Á‡·ÓÎfl‚‡ÌËfl, ͇ÚÓ Ô˙‚˘̇ ·ËΡ̇ ˆËÓÁ‡ ËÎË Ô˙‚˘ÂÌ ÒÍÎÂÓÁË‡˘ ıÓ·̄ËÚ (8). èË ˜ÛÊ‰Ë Ú· ‚ ÓÍÓÚÓ Ò˙‰˙ʇ˘Ë ω, ͇ÍÚÓ Ë ÔË Ô‡ˆËÂÌÚË Ò ÏÛÎÚËÔÎÂÌ ÏËÂÎÓÏ Ë ·ÂÎÓ‰Ó·ÂÌ Í‡ˆËÌÓÏ Ò˙˘Ó ÏÓÊ ‰‡ Ò ‚ËÁÛ‡ÎËÁË‡Ú Ú‡ÍË‚‡ Ô˙ÒÚÂÌË. èÓfl‰ÍÓ ÏÓÊ ‰‡ Ò ̇·Î˛‰‡‚‡Ú ͇ڇ‡ÍÚË ÚËÔ “ÒÎ˙̘ӄΉ”, ‰˙Îʇ˘Ë Ò ̇ ωÂÌ ‰ÂÔÓÁËÚ ‚ ΢‡Ú‡ (8). íÂ, ͇ÍÚÓ Ë Ô˙ÒÚÂÌËÚ ̇ Kayser-Fliesher, ÔÓÒÚÂÔÂÌÌÓ ËÁ˜ÂÁ‚‡Ú ÔË ÂÙÂÍÚË‚ÌÓ Ï‰Ë͇ÏÂÌÚÓÁÌÓ Î˜ÂÌË ËÎË ÒΉ ˜ÂÌÓ‰ӷ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl, ‚˙ÔÂÍË ˜Â ÚÂÏÔ˙Ú Ì ÍÓÂÎË‡ Ò Ì‡Ï‡Îfl‚‡ÌÂÚÓ Ì‡ ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË (8). ë˜ËÚ‡ ÒÂ, ˜Â ̇ ωÌÓ ÓÚ·„‡Ì Ò ‰˙ÎÊË ÒËÌ͇‚ÓÚÓ Óˆ‚ÂÚfl‚‡ÌÂÚÓ Ì‡ ÎÛÌÛÎËÚ ̇ ÌÓÍÚËÚ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË. èË 20-50% ÓÚ Ô‡ˆËÂÌÚËÚ ÏÓ„‡Ú ‰‡ Ò ̇·Î˛‰‡‚‡Ú ̇Û¯ÂÌËfl ‚ ÓÔÓÌÓ-‰‚Ë„‡ÚÂÎ̇ڇ ÒËÒÚÂχ, ͇ÚÓ: ÓÒÚÂÓÔÓÓÁ‡, ÓÒÚÂÓχ·ˆËfl, ıÓ̉Ó͇ΈËÌÓÁ‡, ÓÒÚÂÓ‡ÚËÚ, ÒÚ‡‚ÂÌ ıËÔÂÏÓ·ËÎËÚÂÚ. (5,8,9). èË 10-15% ÓÚ ·ÓÎÌËÚ Ò ÓÚÍË‚‡ Coombs (-) ıÂÏÓÎËÚ˘̇ ‡ÌÂÏËfl (5,8), ÍÓflÚÓ ÏÓÊ ‰‡ Ô‰ıÓʉ‡ ÓÒڇ̇ÎËÚ ÍÎËÌ˘ÌË ËÁfl‚Ë (12). Å˙·ˆËÚ ÏÓ„‡Ú ‰‡ Ò‡ Á‡Ò„̇ÚË Ô˙‚˘ÌÓ ËÎË ‚ÚÓ˘ÌÓ. êÂ̇Î̇ڇ ÚÛ·ÛÎ̇ ‰ËÒÙÛÌ͈Ëfl /ÒË̉ÓÏ Ì‡ î‡ÌÍÓÌË/ ‚Ó‰Ë ‰Ó ‡ÏËÌÓ‡ˆË‰ÛËfl, „βÍÓÁÛËfl, ÙÛÍÚÓÁÛËfl, ͇ΈËÛËfl, ÙÓÒÙ‡ÚÛËfl, Ôӂ˯Â̇ ÂÍÒÍˆËfl ̇ ÔËÍӘ̇ ÍËÒÂÎË̇ (8,9,13). èË ÓÍÓÎÓ 16% ÓÚ ·ÓÎÌËÚ ÏÓÊ ‰‡ Ëχ ÌÂÙÓÎËÚˇÁ‡ (9,14). éÔËÒ‡ÌË Ò‡ Ӣ ıËÔÓÔ‡‡ÚËÂÓˉËÁ˙Ï, ÏÂÌÒÚÛ‡ÎÌË Ì‡Û¯ÂÌËfl, ÒÔÓÌÚ‡ÌÌË ‡·ÓÚË, Ô‡ÌÍ‡ÚËÚ (8). ÖıÓ͇‰ËÓ„‡ÙËflÚ‡ Ë 24˜ ïÓÎÚÂ-ÏÓÌËÚÓËÌ„ ÓÚÍË‚‡Ú Û‚Â΢Â̇ ‰Â·ÂÎË̇ ̇ ËÌÚÂ‚ÂÌÚËÍÛ·ÌËfl ÒÂÔÚÛÏ Ë Î‚Ó͇ÏÂ̇ڇ ÒÚÂ̇, ÂÔËÁÓ‰Ë Ì‡ ÒÛÔ‡‚ÂÌÚËÍÛ·̇ Ú‡ıË͇‰Ëfl (15). èË Ô‡ˆËÂÌÚË Ò Ì‚ÓÎӄ˘ÌË ÔÓfl‚Ë Ò ̇·Î˛‰‡‚‡Ú ÔÓÏÂÌË ‚ ‚ÓÍË‡ÌËÚ ÔÓÚÂ̈ˇÎË (Öè), Û‰˙ÎÊÂÌË Î‡ÚÂ̈ËË Ò ̇·Î˛‰‡‚‡Ú ‚ 58-78% ÔË ÒÓχÚÓÒÂÌÁÓÌËÚ Öè, ‚ 26-53% ÔË ÁËÚÂÎÌËÚ Öè Ë ‚ 40-100% ÔË ÏÓÁ˙˜ÌÓÒÚ‚ÓÎÓ‚ËÚ ÒÎÛıÓ‚Ë Öè. èË Â‰ÌÓ ÔÓÛ˜‚‡Ì Öè Ò‡ Ôӂ‰ÂÌË Ì‡ ·‡ÚflÚ‡, ÒÂÒÚËÚÂ Ë Ó‰ËÚÂÎËÚ ̇ 13 ÌÓ‚ÓÓÚÍËÚË ‰Âˆ‡ Ò ·ÓÎÂÒÚ Ì‡ Wilson, ÔË ÍÓÂÚÓ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Û‰˙ÎÊÂÌË Î‡ÚÂ̈ËË ÔË 42.9% ÓÚ ÔÂÒËÏÔÚÓχÚ˘ÌËÚÂ Ë 21.4% ÓÚ ‡ÒËÏÔÚÓχÚ˘ÌËÚ ‰Âˆ‡, ÔË ÎËÔÒ‡ ̇ Ô˙ÒÚÂÌ Ì‡ Kayser-Fleischer Ë ÎÂÁËË ÔË åêí ËÁÒΉ‚‡ÌÂÚÓ Ì‡ „·‚ÌËfl ÏÓÁ˙Í. ëΉӂ‡ÚÂÎÌÓ Ì‡Î˘Ë ̇ ÒÛ·ÍÎËÌ˘ÌÓ Á‡Òfl„‡Ì ̇ ñçë  ̇Îˈ ‚ ÏÌÓ„Ó ‡ÌÌËÚ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡, ‡ Öè ÏÓ„‡Ú ‰‡ ‰ÓÔ˙ÎÌflÚ Ú.̇. “Ù‡ÏËÎÂÌ ÒÍËÌËÌ„” (31).

137

ç‚ÓËÁÓ·‡ÁËÚÂÎÌËÚ ÏÂÚÓ‰Ë Ì ÔÓ͇Á‚‡Ú ÒÔˆËÙ˘ÌË Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔÓÏÂÌË. ë äí Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ıËÔÓ‰ÂÌÁÌË ÁÓÌË ‚ putamen, nucleus ruber, nucleus dentatus, ÍÓÓ‚‡ ‡ÚÓÙËfl Ë ıˉÓˆÂÙ‡ÎËfl. èË åêí Ò ÓÚÍË‚‡Ú ıËÔÂËÌÚÂÌÁÌË ‚ í2WI ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË, ڇ·ÏÛÒ‡, nucleus dentatus, ÒÚ‚Ó·, ÔË‡ÏˉÌËfl Ë ÂÍÒÚ‡ÔË‡ÏˉÌËÚ Ô˙Úˢ‡ (17). Ç˙ÁÏÓÊÌÓ Â ÚÂÁË ÔÓÏÂÌË ‰‡ Ò ÓÚÍËflÚ Ó˘Â ÔÂ‰Ë Ì‡˜‡ÎÓÚÓ Ì‡ ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË (8). ÇËÒÓÍÓÒ˄̇ÎÌËÚ ÔÓÏÂÌË ‚ ÒË‚ÓÚÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó ÔË í2 ËÁÏÂ‚‡ÌÂÚÓ ÏÓÊ ‰‡ Ò ‰˙ÎÊ‡Ú Ì‡ ‰ÂÏ, „ÎËÓÁ‡, ÌÂÍÓÁ‡, ÍËÒÚ˘̇ ‰Â„ÂÌÂ‡ˆËfl, ‡ ÚÂÁË ‚ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó – ̇ ‰ÂÏËÂÎËÌËÁ‡ˆËfl, ÒÔÓÌ„ËÙÓÏÂ̇ ‰Â„ÂÌÂ‡ˆËfl ËÎË Í‡‚ËÚ‡ÌÓ ‡ÁÔ‡‰‡ÌÂ. çflÍÓË ÓÚ åêí ÔÓÏÂÌËÚ ҇ Ó·‡ÚËÏË ÒΉ ÔÓ‚Âʉ‡Ì ̇ ΘÂÌË – ‚ËÒÓÍÓÒ˄̇ÎÌËÚ ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË, χÎÍËÚ ıËÔÂËÌÚÂÌÁÌË ÌÓ‰ÛÎË, ÍÓËÚÓ ÔÓ-‡ÌÓ Ò‡ ·ËÎË ÒÏflÚ‡ÌË Á‡ ÌÂÍÓÚ˘ÌË. Ç˙ÁÏÓÊ̇ åêí ̇ıӉ͇ Â Ë ÔÓÌÚËÌ̇ڇ ÏËÂÎËÌÓÁ‡. èÓÒΉ̇ڇ Ò˙˘Ó ËÁ˜ÂÁ‚‡ Ò Ì‡Ï‡Îfl‚‡ÌÂÚÓ Ì‡ ÒËÏÔÚÓÏËÚ ÔË ‡‰ÂÍ‚‡ÚÌÓ Î˜ÂÌËÂ. ëΉӂ‡ÚÂÎÌÓ åêí ËÁÒΉ‚‡ÌÂÚÓ ÏÓÊ ‰‡ ·˙‰Â ÔÓÎÂÁÌÓ ÔË ÔÓÒΉfl‚‡Ì ̇ ÎÂÍÛ‚‡ÌËÚ ԇˆËÂÌÚË (26). àÁÒΉ‚‡ÌÂÚÓ Ì‡ ω̇ڇ Ó·Ïfl̇ ‡ÁÍË‚‡ ÒΉÌËÚ ÔÓÏÂÌË: ● çËÒÍÓ ÌË‚Ó Ì‡ Ó·˘‡Ú‡ ω ‚ ÒÂÛχ /ÌÓχ 12-24 m mol/l/, ÍÓÂÚÓ Ò ‰˙ÎÊË Ì‡ ÔÓÌËÊÂÌËfl ˆÂÛÎÓÔ·ÁÏËÌ ● çËÒÍÓ ÌË‚Ó Ì‡ ˆÂÛÎÓÔ·ÁÏË̇ /ÌÓχ 0,2-0,6 g/l/. èÓÌËÊÂÌ Â Ó·‡˜Â Ë ÔË Ï‡Î‡·ÒÓ·ˆËÓÌÂÌ ÒË̉ÓÏ, ÌÂÙÓÚ˘ÂÌ ÒË̉ÓÏ, ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ‡ˆÂÛÎÓÔ·ÁÏËÌÂÏËfl, ÔË ıÂÚÂÓÁË„ÓÚËÚ (1,8). çÓχÎÌÓÚÓ ÌË‚Ó Ì ËÁÍβ˜‚‡ ‰Ë‡„ÌÓÁ‡Ú‡, Á‡˘ÓÚÓ ÚÓ‚‡  ÓÒÚÓÙ‡ÁÓ‚ ·ÂÎÚ˙Í Ë ÔË Ô‡ˆËÂÌÚË Ò˙Ò Ò˙Ô˙ÚÒÚ‚‡˘Ë ‚˙ÁÔ‡ÎËÚÂÎÌË ÔÓˆÂÒË ÒÂÛÏ̇ڇ ÍÓ̈ÂÌÚ‡ˆËfl ÏÓÊ ‰‡  ÌÓχÎ̇ (2). ● ì‚Â΢ÂÌÓ ÒÂÛÏÌÓ ÌË‚Ó Ì‡ ÌÂÒ‚˙Á‡Ì‡Ú‡ Ò ˆÂÛÎÓÔ·ÁÏËÌ Ï‰ /ÌÓχ 10-15 m g/dl / - ‰Ë‡„ÌÓÒÚ˘ÂÌ ÚÂÒÚ ÔË Á‡·ÓÎfl‚‡ÌÂÚÓ, ‚‡ÊÂÌ ÔË ÏÓÌËÚÓË‡ÌÂÚÓ Ì‡ Ù‡χÍÓÚÂ‡ÔËflÚ‡. í‚˙‰Â Á‡‚ËÒËÏ Â ÓÚ Î‡·Ó‡ÚÓ̇ڇ ÏÂÚÓ‰Ë͇. ● ì‚Â΢Â̇ ÂÍÒÍˆËfl ̇ ω ‚ ÛË̇ڇ / ÌÓχ 0,441,1 m mol/24h / - ‰Ë‡„ÌÓÒÚ˘ÂÌ ÚÂÒÚ ÔË Á‡·ÓÎfl‚‡ÌÂÚÓ, ‚‡ÊÂÌ ÔË ÏÓÌËÚÓË‡ÌÂÚÓ Ì‡ Ù‡χÍÓÚÂ‡ÔËflÚ‡. åÓÊ ӷ‡˜Â ‰‡ Ò ̇·Î˛‰‡‚‡ Ë ÔË ‰Û„Ë ˜ÂÌÓ‰Ó·ÌË Á‡·ÓÎfl‚‡ÌËfl. ● èË ‰Âˆ‡Ú‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ò ÔÓ‚Âʉ‡ ÚÂÒÚ Ò DPenicillamine – Û‚Â΢‡‚‡Ì ̇ ω̇ڇ ÂÍÒÍˆËflÚ‡ /̇‰ 25m mol/ ‚ 24˜ ÛË̇ /ÒΉ ‰‚ÛÍ‡ÚÂÌ ÔËÂÏ Ì‡ 500 m g DPenicillamine/, ÍÓÂÚÓ ËÁÍβ˜‚‡ ‰Û„Ë ˜ÂÌÓ‰Ó·ÌË Á‡·ÓÎfl‚‡ÌËfl ● ì‚Â΢Â̇ ÍÓ̈ÂÌÚ‡ˆËfl ̇ ω ‚ ˜ÂÌËfl ‰Ó· /̇‰ 250 m g/g ÒÛıÓ Ú„ÎÓ/ ÔË ˜ÂÌÓ‰ӷ̇ ·ËÓÔÒËfl. íÓ‚‡  ̇È-‰Ó·ÓÚÓ ‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. çÓχÎ̇ڇ ÍÓ̈ÂÌÚ‡ˆËfl fl‰ÍÓ Ì‡‰ı‚˙Îfl 50m g/g ÒÛıÓ Ú„ÎÓ, ‡ ÔË ıÂÚÂÓÁË„ÓÚËÚÂ, ‚˙ÔÂÍË ˜Â ˜ÂÒÚÓ Â Ì‡‰ ÌÓχڇ, Ì ̇‰ı‚˙Îfl 250 m g/g ÒÛıÓ Ú„ÎÓ (8) ● ç‡Ï‡ÎÂÌÓ ‚Íβ˜‚‡Ì ̇ ‡‰ËÓ‡ÍÚË‚ÂÌ Ï‰ /64Cu/ ‚ ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌËfl ˆÂÛÎÓÔ·ÁÏËÌ – ÚÓÁË ÚÂÒÚ ‰ÌÂÒ fl‰ÍÓ Ò ËÁÔÓÎÁ‚‡, ÔÓ‡‰Ë ÚÛ‰ÌÓÒÚË ÔË Ì‡ÏË‡ÌÂÚÓ Ì‡ ËÁÓÚÓÔ (8). ïËÒÚÓÎӄ˘ÌËÚ ÔÓÏÂÌË ‚ ˜ÂÌËfl ‰Ó· ‚ ̇˜‡ÎÓÚÓ Ò‡ ÎÂ͇ ÒÚ‡ÚÓÁ‡ Ë ÙÓ͇Î̇ ıÂÔ‡ÚÓˆÂÎÛ·̇ ÌÂÍÓÁ‡. åÓÊ ‰‡ Ò ̇·Î˛‰‡‚‡Ú Í·Ò˘ÂÒÍË ıËÒÚÓÎӄ˘ÌË ·ÂÎÂÁË Ì‡ ‡‚ÚÓËÏÛÌÂÌ ıÂÔ‡ÚËÚ. ë ÔÓ„ÂÒË‡Ì ̇ Ô‡ÂÌıËÏÌÓÚÓ Û‚Âʉ‡Ì Ò ‡Á‚Ë‚‡ ÙË·ÓÁ‡, ‡ ‚ÔÓÒΉÒÚ‚ËÂ Ë ˆËÓÁ‡. é·ËÍÌÓ‚ÂÌÓ Úfl  χÍÓÌÓ‰Û·̇, ÌÓ ÔÓÌflÍÓ͇  ÏËÍÓÌÓ‰Û·̇. Ç ‡ÌÌËÚ ÒÚ‡‰ËË Ï‰ڇ  ÌÂ‡Á΢Ëχ ıËÒÚÓıËÏ˘ÂÒÍË, ÔÓ‡‰Ë Ò‚˙Á‚‡ÌÂÚÓ È Ò ÏÂÚ‡ÎÓÚËÓÌÂËÌËÚÂ. ìÎÚ‡ÒÚÛÍÚÛÌËÚ ËÁÒΉ‚‡ÌËfl Ӣ ‚ ̇˜‡ÎÓÚÓ


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÓÚÍË‚‡Ú ÒÔˆËÙ˘ÌË ÏËÚÓıÓ̉ˇÎÌË ‡·ÌÓÏÌÓÒÚË: ‚‡ˇ·ËÎÌÓÒÚ ‚ ‡ÁÏÂ‡ Ë ÙÓχڇ, Û‚Â΢Â̇ ÔÎ˙ÚÌÓÒÚ Ì‡ χÚËÍÒ‡, ÏÌÓ„Ó·ÓÈÌË ‚Íβ˜‚‡ÌËfl ÓÚ ÎËÔË‰Ë Ë ÌÂÊÌË „‡ÌÛ·ÌË Ï‡ÚÂËË, ÍÓËÚÓ ÏÓÊ ‰‡ Ò‡ ωÌË. ç‡È‚Ô˜‡ÚÎfl‚‡˘‡Ú‡ ÔÓÏfl̇  ۂÂ΢ÂÌÓÚÓ ÔÓÒÚ‡ÌÒÚ‚Ó ÏÂÊ‰Û ÍËÒÚËÚÂ Ò ‡Á¯ËÂÌË ̇ Ò˙˘ËÚÂ, Ò˙Á‰‡‚‡˘Ó ÍËÒÚ˘ÂÌ ËÁ„Ή. èË ÓÚÒ˙ÒÚ‚Ë ̇ ıÓÎÂÒÚ‡Á‡ ÚÂÁË ÔÓÏÂÌË Ò‡ Ô‡ÚÓ„ÌÓÏÓÌ˘ÌË Á‡ ·ÓÎÂÒÚ Ì‡ Wilson. èË ÔÓÍ˙ÒÌËÚ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡ Ò ̇·Î˛‰‡‚‡Ú ÔÎ˙ÚÌË ‰ÂÔÓÁËÚË ‚ ÎËÁÓÁÓÏËÚÂ. ìÎÚ‡ÒÚÛÍÚÛÌËÚ ËÁÒΉ‚‡ÌËfl ÏÓÊ ‰‡ ÔÓÏÓ„Ì‡Ú ÔË ‡Á„‡Ì˘‡‚‡ÌÂÚÓ Ì‡ ·ÓÎÌËÚÂ Ë ıÂÚÂÓÁË„ÓÚÌËÚ ÌÓÒËÚÂÎË (8). ÉÂÌÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl – ‰ËÂÍÚÌËflÚ ‡Ì‡ÎËÁ ̇ ÏÛÚ‡ˆËËÚ  ӄ‡Ì˘ÂÌ, Ú˙È Í‡ÚÓ Ôӂ˜ÂÚÓ Ô‡ˆËÂÌÚË Ò‡ ÌÓÒËÚÂÎË Ì‡ ‡Á΢ÌË ÏÛÚ‡ˆËË ‚˙‚ ‚ÒÂÍË ‡ÎÂÎ, ‡ ÔÓ̇ÒÚÓfl˘ÂÏ Ò‡ ËÁ‚ÂÒÚÌË Ì‡‰ 200 ÏÛÚ‡ˆËË. èË ÌflÍÓË ÔÓÔÛ·ˆËË /äÓÂfl, ë‡‰ËÌËfl, üÔÓÌËfl, ËÁÚӘ̇ Ö‚ÓÔ‡/ Ò ‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ ÓÔ‰ÂÎÂ̇ ÏÛÚ‡ˆËfl ‰ËÂÍÚÌËflÚ ‡Ì‡ÎËÁ ÔÓχ„‡ Á‡ ‰Ë‡„ÌÓÁ‡Ú‡ (1,8). ÑËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡ Ô‰‚ˉ „ÓÎflÏÓÚÓ ‡ÁÌÓÓ·‡ÁË ÓÚ ÍÎËÌ˘ÌË ÔÓfl‚Ë Â ¯ËÓ͇. èË Ô‡ˆËÂÌÚË Ò ÌÂÓ·flÒÌËχ ˜ÂÌÓ‰ӷ̇ ‰ËÒÙÛÌ͈Ëfl, Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇, ıÂÏÓÎËÚ˘̇ ‡ÌÂÏËfl ËÎË ÔÒËı˘ÌË ÔÓfl‚Ë Úfl·‚‡ ‰‡ Ò ÏËÒÎË Ë Á‡ ÚÓ‚‡ Á‡·ÓÎfl‚‡ÌÂ. èÓ ÓÚÌÓ¯ÂÌË ̇ Ì‚ÓÎӄ˘ÌËÚ ÒËÏÔÚÓÏË, ̇È-˜ÂÒÚÓ Í‡ÚÓ ‰ËÙÂÂ̈ˇÎ̇ ‰Ë‡„ÌÓÁ‡ Úfl·‚‡ ‰‡ Ò ËÏ‡Ú Ô‰‚ˉ ÒΉÌËÚ Á‡·ÓÎfl‚‡ÌËfl: è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, Ô‡ÍËÌÒÓÌËÁ˙Ï ÔÎ˛Ò ÒË̉ÓÏË, ωË͇ÏÂÌÚÓÁÌÓ, ÚÓÍÒ˘ÌÓ Ë ÏÂÚ‡·ÓÎËÚÌÓ Ë̉ۈË‡Ì Ô‡ÍËÌÒÓÌËÁ˙Ï, ÔÓÒÚÂ̈ÂÙ‡ÎËÚÂÌ, ÔÓÒÚÚ‡‚χÚ˘ÂÌ Ô‡ÍËÌÒÓÌËÁ˙Ï, ÂÒÂ̈ˇÎÂÌ ÚÂÏÓ, ˉËÓÔ‡Ú˘̇ ‰ËÒÚÓÌËfl, ÍÓÚËÍÓ·‡Á‡Î̇ ‰Â„ÂÌÂ‡ˆËfl, ÏÛÎÚËÒËÒÚÂÏ̇ ‡ÚÓÙËfl, ·ÓÎÂÒÚ Ì‡ Hallervorden-Spatz, ÓÎË‚ÓÔÓÌÚÓˆÂ·Â·̇ ‡ÚÓÙËfl, ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡ (5). Ç ÒÂÏÂÈÒÚ‚ÓÚÓ Ì‡ ‚ÒÂÍË ‰Ë‡„ÌÓÒÚˈË‡Ì ·ÓÎÂÌ Úfl·‚‡ ‰‡ Ò ÓÒ˙˘ÂÒÚ‚Ë “Ù‡ÏËÎÂÌ ÒÍËÌËÌ„”: ÓÒ‚ÂÌ ‡Ì‡ÏÌÂÁ‡, ÒÓχÚ˘ÂÌ Ë Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ, ·ËÓÏËÍÓÒÍÓÔËfl, Úfl·‚‡ ‰‡ Ò ËÁÒΉ‚‡ Ô˙Î̇ڇ Í˙‚̇ ͇ÚË̇, ·ËÓıËÏËflÚ‡ Ò ÔÓ͇Á‡ÚÂÎËÚ Á‡ ˜ÂÌÓ‰ӷ̇ ÙÛÌ͈Ëfl, ˆÂÛÎÓÔ·ÁÏËÌ˙Ú, ωڇ ‚ 24˜ ÛË̇ Ë ÏÓÊ ‰‡ Ò Ôӂ‰ Ñçä ‡Ì‡ÎËÁ ÔË ÛÒÚ‡ÌÓ‚Â̇ ÏÛÚ‡ˆËfl ̇ ÔÓ·‡Ì‰‡. ãÖóÖçàÖ î‡χÍÓÎӄ˘ÌÓÚÓ Î˜ÂÌË ÔÓ‰˙Îʇ‚‡ ‰Ó Í‡fl ̇ ÊË‚ÓÚ‡. àÁÔÓÎÁ‚‡Ú ÒÂ: ‰ËÂÚ‡, Ó„‡Ì˘‡‚‡˘‡ ‰Ì‚ÌËfl ÔËÂÏ Ì‡ ω /‰Ó 1Ï„ ‰Ì./, ı·ÚÓË Ë ˆËÌÍÓ‚Ë ÒÓÎË. èË ÓÒÚ‡ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ò ÔË·„‡ ˜ÂÌÓ‰ӷ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl. ï·ÚÓËÚ Ò ÔÂÔÓ˙˜‚‡Ú ͇ÚÓ Ì‡˜‡Î̇ ÚÂ‡ÔËfl ÔË ÒËÏÔÚÓχÚ˘ÌË ·ÓÎÌË (8). ç‡È„ÓÎflÏ Â Ò‚ÂÚÓ‚ÌËflÚ ÓÔËÚ Ò ı·ÚÓ‡ D-Penicillamine, ÌÓ ÔÓ‡‰Ë Ô˙‚Ó̇˜‡ÎÌÓ ‚ÎÓ¯‡‚‡Ì ̇ Ì‚ÓÎӄ˘̇ڇ ÒËÏÔÚÓχÚË͇ Ë ÒÂËÓÁÌËÚ ÒÚ‡Ì˘ÌË ÂÙÂÍÚË, ̇ÔÓÒΉ˙Í Ò ÔÂÔÓ˙˜‚‡Ú Trientine (8) Ë Tetrathiomolybdate (18) ͇ÚÓ Ì‡˜‡Î̇ ÚÂ‡ÔËfl. ëΉ ÒÚ‡·ËÎËÁË‡Ì ̇ ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË Ë ·ËÓıËÏ˘ÌËÚ ÔÓ͇Á‡ÚÂÎË /Ó·ËÍÌÓ‚ÂÌÓ 2-6 ÏÂÒˆ‡ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ÚÂ‡ÔËflÚ‡/ Ò ÔÂÏË̇‚‡ ̇ ÔÓ‰‰˙ʇ˘Ó ΘÂÌËÂ Ò ı·ÚÓ ËÎË ˆËÌÍÓ‚Ë ÒÓÎË - Zn acetate (8). èË ÔÂÒËÏÔÚÓÏÌË Ô‡ˆËÂÌÚË Ò ËÁÔÓÎÁ‚‡Ú ˆËÌÍÓ‚Ë ÒÓÎË, ÍÓËÚÓ Ò‡ Ò ‚ËÒÓ͇ ÂÙÂÍÚË‚ÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË Ô‰ÓÚ‚‡Úfl‚‡Ì ÔÓfl‚‡Ú‡ ̇ ÒËÏÔÚÓÏË Ì‡ ·ÓÎÂÒÚÚ‡ (19,20), ÍÓÂÚÓ Ó˘Â Ôӂ˜ ÔÓÚ‚˙ʉ‡‚‡ ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ÓÚ Ù‡ÏËÎÂÌ ÒÍËÌËÌ„ Á‡ ÓÚÍË‚‡ÌÂÚÓ Ì‡ Ú‡ÍË‚‡ Ô‡ˆËÂÌÚË. D-PENICILLAMINE ë˙‰˙ʇ Ò‚Ó·Ó‰ÌË ÒÛÎÙıˉËÎÌË „ÛÔË, ÍÓËÚÓ Ò‚˙Á‚‡Ú ω. éÒÌÓ‚ÌËflÚ ÏÛ ÂÙÂÍÚ ÔË ·ÓÎÂÒÚÚ‡ ̇

138

Wilson  Ôӂ˯‡‚‡Ì ̇ ω̇ڇ ÛËÌ̇ ÂÍÒÍˆËfl. àχ Ë ËÏÛÌÓÒÛÔÂÒË‚ÌÓ ‰ÂÈÒÚ‚ËÂ. ÖÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ΘÂÌËÂÚÓ Ò ÚÓÁË Ï‰Ë͇ÏÂÌÚ Â ‰Ó͇Á‡ÌÓ Ò ÏÌÓ„Ó·ÓÈÌË ÔÓÛ˜‚‡ÌËfl (8). èӂ˜ÂÚÓ ÒËÏÔÚÓχÚ˘ÌË Ô‡ˆËÂÌÚË /˜ÂÌÓ‰Ó·ÌË, Ì‚ÓÎӄ˘ÌË ËÎË ÔÒËıˇÚ˘ÌË/ ÓÚ„Ó‚‡flÚ ‚ ‡ÏÍËÚ ̇ ÏÂÒÂˆË ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ÚÂ‡ÔËflÚ‡ (1). ÇÎÓ¯‡‚‡Ì ̇ Ì‚ÓÎӄ˘ÌËÚ ÒËÏÔÚÓÏË ÔÂÁ ̇˜‡Î̇ڇ Ù‡Á‡ ̇ ΘÂÌËÂÚÓ Ò Ò¢‡ ‚ 10% ‰Ó 50%. íÂÏÓ˙Ú Ë Ï‡ÎÍÓÏÓÁ˙˜ÌËÚ ÔÓfl‚Ë Ò ÔÓ‚ÎËfl‚‡Ú ÔÓ-‰Ó· ÓÚ ‰ËÒÚÓÌËflÚ‡. ã˜ÂÌËÂÚÓ Â Ò‚˙Á‡ÌÓ Ò ÏÌÓ„Ó·ÓÈÌË ÒÚ‡Ì˘ÌË ÂÙÂÍÚË, ÍÓËÚÓ ÔË 20%-30% ÓÚ Ô‡ˆËÂÌÚËÚ ̇·„‡Ú ÔÂÍ‡Úfl‚‡ÌÂÚÓ ÏÛ. í ҇ ‰‚ „ÛÔË – ‰ËÂÍÚÌË, ‰ÓÁÓÁ‡‚ËÒËÏË Ë ËÏÛÌÓÎӄ˘ÌÓ Ë̉ۈË‡ÌË. ÑËÂÍÚÌË Ò‡ ̉ÓÒÚË„˙Ú Ì‡ ‚ËÚ‡ÏËÌ Ç6 Ë Ì‡Û¯ÂÌËÂÚÓ Ì‡ Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ÍÓ·„ÂÌ Ë Â·ÒÚËÌ, ÍÓÂÚÓ Ô˙Í ‚Ó‰Ë ‰Ó ÍÓÊÌË ÎÂÁËË /cutis laxa, elastosis perforans serpingiosa/ (1). àÏÛÌÓÎӄ˘ÌÓ Ï‰ËË‡ÌËÚ ÒÚ‡Ì˘ÌË ÂÙÂÍÚË ‚Íβ˜‚‡Ú ΂ÍÓÔÂÌËfl Ë ÚÓÏ·ÓˆËÚÓÔÂÌËfl, ÒËÒÚÂÏÂÌ ÎÛÔÛÒ ÂËÚÂχÚÓ‰ÂÒ, ËÏÛÌÌÓÍÓÏÔÎÂÍÒÂÌ ÌÂÙËÚ, ÔÂÏÙË„ÛÒ, ·Û͇ÎÌË ÛΈÂ‡ˆËË, ÏˇÒÚÂÌËfl „‡‚ËÒ, ÓÔÚ˘ÂÌ Ì‚ËÚ, ÒË̉ÓÏ Ì‡ Goodpasture. é·Ë˜‡È̇ڇ ‰ÓÁ‡ D-Penicillamine  1-2g/‰Ì ‚ ÚË ÔËÂχ (2). ëΉ ͇ÚÓ Ò ÛÒÚ‡ÌÓ‚Ë ÚÂ‡Ô‚Ú˘ÂÌ ÂÙÂÍÚ, ‰ÓÁ‡Ú‡ Ò ̇χÎfl‚‡ ̇ 0,5-1,0g/‰Ì. TRIENTINE /TRIETHYLENE TETRAMINE DIHYDROCHLORIDE/ ê‡Á΢‡‚‡ Ò ÔÓ ıËÏ˘̇ ÒÚÛÍÚÛ‡ ÓÚ DPenicillamine, ÌÓ Ò˙˘Ó  ı·ÚÓ. ÖÙÂÍÚË‚ÂÌ Â ÔË Î˜ÂÌËÂÚÓ Ì‡ ·ÓÎÂÒÚÚ‡ ̇ Wilson Ë Â ÓÒÓ·ÂÌÓ ÔÂÔÓ˙˜‚‡Ì ÔË Ô‡ˆËÂÌÚË, ÍÓËÚÓ Ì ÔÓ̇ÒflÚ D-Penicillamine (8). ÇÎÓ¯‡‚‡Ì ̇ Ì‚ÓÎӄ˘ÌËÚ ÒËÏÔÚÓÏË ‚ ̇˜‡ÎÓÚÓ Ì‡ ΘÂÌËÂÚ  ÏÌÓ„Ó ÔÓ-fl‰ÍÓ. å‡ÎÍÓ Ò‡ Ë ÒÚ‡Ì˘ÌËÚ ÂÙÂÍÚË. é·Ë˜‡È̇ڇ ‰ÓÁ‡  ÓÚ 1-2g/‰Ì ‚ 3 ÔËÂχ (2). TETRATHIOMOLYBDATE ÑÛ„ ıÂÎË‡˘ ‡„ÂÌÚ, ÍÓÈÚÓ Ò ÔÓÛ˜‚‡ Á‡ ̇˜‡ÎÌÓ Î˜ÂÌË ̇ Ô‡ˆËÂÌÚË Ò Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇. è˙‚ËÚ ÂÁÛÎÚ‡ÚË ÔÓ͇Á‚‡Ú ·˙ÁÓ Ì‡Ï‡ÎÂÌË ̇ ҂ӷӉ̇ڇ ÒÂÛÏ̇ ω ÔÂÁ Ô˙‚ËÚ 8 Ò‰ÏËˆË ÓÚ Î˜ÂÌËÂÚÓ, ·ÂÁ ‚ÎÓ¯‡‚‡Ì ̇ ÒËÏÔÚÓÏËÚ (18). ñàçä ÑÂÈÒÚ‚‡ ÔÓ ‰‚‡ ̇˜Ë̇. å‰ڇ Ë ˆËÌÍ˙Ú ËÏ‡Ú Â‰ËÌ Ë Ò˙˘ ÔÂÌÓÒËÚÂÎ ‚ ÂÌÚÂÓˆËÚËÚÂ, ‡ ΘÂÌËÂÚÓ Ò ˆËÌÍ ‚Ó‰Ë ‰Ó Ì„ӂÓÚÓ ·ÎÓÍË‡Ì (1). éÒ‚ÂÌ ÚÓ‚‡ Ë̉ۈË‡ ÏÂÚ‡ÎÓÚËÓÌÂËÌËÚ ‚ ÂÌÚÂÓˆËÚËÚÂ, ÍÓËÚÓ ËÏ‡Ú ÔÓ‚ËÒÓÍ ‡ÙËÌËÚÂÚ Í˙Ï Ï‰ڇ ÓÚÍÓÎÍÓÚÓ Í˙Ï ˆËÌ͇, ÔÓ‡‰Ë ÍÓÂÚÓ Ò‚˙Á‚‡Ú ω ‚ ÂÌÚÂÓˆËÚ‡ Ë ËÌıË·Ë‡Ú ̇‚ÎËÁ‡ÌÂÚÓ È ‚ ÔÓÚ‡Î̇ڇ ÒËÒÚÂχ (1,8). ë ‰ÂÒÍ‚‡Ï‡ˆËflÚ‡ ̇ ÂÌÚÂÓˆËÚËڠωڇ Ò ÓÚ‰ÂÎfl ‚˙‚ ÙÂ͇ÎËËÚÂ. ñËÌÍ˙Ú Ë̉ۈË‡ ÏÂÚ‡ÎÓÚËÓÌÂËÌËÚÂ Ë ‚ ˜ÂÌËfl ‰Ó·, ڇ͇ Ô‰ԇÁ‚‡ ıÂÔ‡ÚÓˆËÚËÚ ÓÚ Ï‰̇ڇ ÚÓÍÒ˘ÌÓÒÚ (1,8). Ç˙ÔÂÍË ˜Â Ò ÔÂÔÓ˙˜‚‡ ͇ÚÓ ÔÓ‰‰˙ʇ˘‡ ÚÂ‡ÔËfl Ë ÔË ‡ÒËÏÔÚÓÏÌË ËÎË ÔÂÒËÏÔÚÓÏÌË Ô‡ˆËÂÌÚË, ˆËÌÍ˙Ú Â ËÁÔÓÎÁ‚‡Ì Ë Í‡ÚÓ Ì‡˜‡Î̇ ÚÂ‡ÔËfl ÔË ÒËÏÔÚÓÏÌË /Ì‚ÓÎӄ˘ÌÓ Ë ˜ÂÌÓ‰Ó·ÌÓ/ ·ÓÎÌË. èÓÛ˜‚‡ÌËflÚ‡ ÒÓ˜‡Ú, ˜Â ÂÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ˆËÌ͇  ÔӉӷ̇ ̇ DPenicillamine, ÌÓ ˆËÌÍ˙Ú Ò ÔÓ̇Òfl ÔÓ-‰Ó· (1,8). é·ËÍÌÓ‚ÂÌÓ Ò ÔË·„‡ ÚË Ô˙ÚË ÔÓ 50mg ‰Ì (2). ÄçíàéäëàÑÄçíà ÄÌÚËÓÍÒˉ‡ÌÚË, „·‚ÌÓ ‚ËÚ‡ÏËÌ Ö ‰ÓÔ˙΂‡Ú ΘÂÌËÂÚÓ. ëÂÛÏÌÓÚÓ ÌË‚Ó Ë ÌË‚ÓÚÓ ‚ ˜ÂÌËfl ‰Ó· ̇ ‚ËÚ‡ÏËÌ Ö Â Ì‡Ï‡ÎÂÌÓ ÔË Á‡·ÓÎfl‚‡ÌÂÚÓ. àχ Ò˙Ó·˘ÂÌËfl, ˜Â ‰Ó·‡-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

‚flÌÂÚÓ ÏÛ Í˙Ï ÚÂ‡ÔËflÚ‡ ‚Ó‰Ë ‰Ó ÒËÏÔÚÓχÚ˘ÌÓ ÔÓ‰Ó·ÂÌËÂ, ÌÓ Ì ҇ ÔÓ‚Âʉ‡ÌË ÒÚËÍÚÌË ÔÓÛ˜‚‡ÌËfl (1,8) ëÔÓ‰ George Brewer ÚÂ‡ÔËflÚ‡ Úfl·‚‡ ‰‡ ·˙‰Â ‡Á΢̇ ÔË ‡Á΢ÌËÚ „ÛÔË Ô‡ˆËÂÌÚË è‡ˆËÂÌÚË ç‚ÓÎӄ˘ÌÓ Ì‡˜‡ÎÓ èÒËıˇÚ˘ÌÓ Ì‡˜‡ÎÓ óÂÌÓ‰Ó·ÌÓ Ì‡˜‡ÎÓ èÓ‰‰˙ʇ˘Ó ΘÂÌË ëΉ Ô˙‚Ó̇˜‡Î̇ ÚÂ‡ÔËfl èÂÒËÏÔÚÓχÚ˘ÌË ·ÓÎÌË ÅÂÏÂÌÌË Ô‡ˆËÂÌÚÍË Ñˆ‡

ã˜ÂÌË Tetrathiomolybdate Tetrathiomolybdate Trientine Ë Zinc Zinc Zinc Zinc Zinc

íÖêÄèÖÇíàóçé åéçàíéêàêÄçÖ ÄÍÓ Ò ËÁÔÓÎÁ‚‡ ıÂÎË‡˘ ‡„ÂÌÚ Â ÌÂÓ·ıÓ‰ËÏÓ ‰‡ Ò ËÁÒΉ‚‡ ÌË‚ÓÚÓ Ì‡ ωڇ ‚ 24˜ ÛË̇. ÄÍÓ Ô‡ˆËÂÌÚ˙Ú ÓÚ„Ó‚‡fl ̇ ΘÂÌËÂÚÓ Ë Ò ‚ÂÏÂÚÓ Ï‰̇ڇ ÛËÌ̇ ÂÍÒÍˆËfl ̇χÎfl‚‡ Ë Ò ÒÚ‡·ËÎËÁË‡ ÔÓ‰ 500mg, ‰ÓÁ‡Ú‡ ̇ D-Penicillamine ÏÓÊ ‰‡ Ò ̇χÎË. ÖÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ΘÂÌËÂÚÓ ÏÓÊ ‰‡ Ò ÏÓÌËÚÓË‡ ˜ÂÁ ÓÔ‰ÂÎflÌÂÚÓ Ì‡ ҂ӷӉ̇ڇ ω ‚ ÒÂÛχ. èË ˜ÂÌÓ‰Ó·ÌË ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÏÓÊ ‰‡ Ò ËÁÒΉ‚‡Ú ÔÓ͇Á‡ÚÂÎËÚ Á‡ ˜ÂÌÓ‰ӷ̇ ÙÛÌ͈Ëfl. èÓ‰Ó·ÂÌËÂÚÓ Ì‡ Ì‚ÓÎӄ˘ÌËÚ ÒËÏÔÚÓÏË Ò ӈÂÌfl‚‡ Ò ÍÎËÌ˘ÌËfl Ô„Ή. ä‡ÚÓ ‰ÓÔ˙ÎÌÂÌË ÌflÍÓË åêí ÔÓÏÂÌË Ò‡ ̇Ô˙ÎÌÓ Ó·‡ÚËÏË ÔË Î˜ÂÌËÂÚÓ. åÓÁ˙˜ÌÓÒÚ‚ÓÎÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË Ò˙˘Ó ÏÓÊ ‰‡ Ò‡ ÔÓÎÂÁÌË Á‡ ÓÚ˜Ëڇ̠ÔÓ‰Ó·ÂÌËÂÚÓ ÓÚ ÚÂ‡ÔËflÚ‡ (1,31). óÖêçéÑêéÅçÄ íêÄçëèãÄçíÄñàü óÂÌÓ‰ӷ̇ڇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl  Ò‰ÒÚ‚Ó Ì‡ ËÁ·Ó ÔË Ô‡ˆËÂÌÚË Ò ‰ÂÍÓÏÔÂÌÒË‡ÌÓ ˜ÂÌÓ‰Ó·ÌÓ Û‚Âʉ‡ÌÂ, ÌÂÔÓ‚ÎËfl‚‡˘Ó Ò ÓÚ ı·ÚÓÌÓ Î˜ÂÌËÂ Ë ÙÛÎÏË̇ÌÚ̇ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (1,8). Ç˙ÔÂÍË ˜Â ÚÓ‚‡ ÍÓË„Ë‡ ·ËÓıËÏ˘ÌËflÚ ‰ÂÙÂÍÚ, ÍÓÈÚÓ Â ‚ ÓÒÌÓ‚‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÓÎflÚ‡ ̇ ˜ÂÌÓ‰ӷ̇ڇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ÔË Ì‚ÓÎӄ˘ÌË ÙÓÏË Ì‡ ·ÓÎÂÒÚÚ‡ ‚ ÓÚÒ˙ÒÚ‚Ë ̇ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ ‚Ò Ӣ Ì  ÛÒÚ‡ÌÓ‚Â̇. ã˜ÂÌËÂÚÓ ÔÓ ‚ÂÏ ̇ ·ÂÏÂÌÌÓÒÚ Úfl·‚‡ ‰‡ ÔÓ‰˙ÎÊË. Ö‰ËÌÒÚ‚ÂÌËflÚ ‰Ó͇Á‡ÌÓ ·ÂÁÓÔ‡ÒÂÌ Ï‰Ë͇ÏÂÌÚ Â ˆËÌÍ˙Ú. D-Penicillamine  ÚÂ‡ÚÓ„ÂÌÂÌ ÔË ÊË‚ÓÚÌË Ë ıÓ‡. Trientine  ÚÂ‡ÚÓ„ÂÌÂÌ ÔË ÊË‚ÓÚÌË, ‚˙ÔÂÍË ˜Â ·ÂÁÓÔ‡ÒÌÓÒÚÚ‡ ÏÛ ÔË ıÓ‡ Ì  ‰Ó͇Á‡Ì‡, Devesa Ë ‰. /1995„/ Ò˙Ó·˘‡‚‡Ú Á‡ ΘÂÌËÂ Ò Trientine ÔÓ ‚ÂÏ ̇ ·ÂÏÂÌÌÓÒÚ, ÍÓflÚÓ Á‡‚˙¯Ë· Ò ‡Ê‰‡Ì ̇ Á‰‡‚Ó ‰ÂÚÂ. ã˜ÂÌËÂÚÓ Ò ˆËÌÍÓ‚Ë ÒÓÎË ÔÓ ‚ÂÏ ̇ ·ÂÏÂÌÌÓÒÚÚ‡  ·ÂÁÓÔ‡ÒÌÓ Ë ÂÙÂÍÚË‚ÌÓ (8,20,21), ‡ ÒÔÓ‰ ÌflÍÓË ‡‚ÚÓË Î˜ÂÌËÂÚÓ ÏÓÊ ‰‡ ÔÓ‰˙ÎÊË Ò ı·ÚÓË Ò 20-50% ‰Û͈Ëfl ̇ Ô˙‚Ó̇˜‡Î̇ڇ ‰ÓÁ‡ (8). Ç ·˙‰Â˘Â ‚ÂÓflÚÌÓ ˘Â ÏÓÊ ‰‡ Ò ÔË·„‡ „ÂÌ̇ ÚÂ‡ÔËfl Á‡ ‰ÂÙËÌËÚË‚ÌÓ Î˜ÂÌËÂ. èË LEC ÔÎ˙ıÓ‚Â /ÂÍÒÔÂËÏÂÌÚ‡ÎÂÌ ÊË‚ÓÚËÌÒÍË ÏÓ‰ÂΠ̇ ·ÓÎÂÒÚ Ì‡ Wilson/  ÓÒ˙˘ÂÒÚ‚ÂÌ ÛÒÔ¯ÂÌ, ÌÓ ‚ÂÏÂÌÂÌ Ú‡ÌÒÙÂ ̇ „Â̇ ATP7B ˜ÂÁ ‡‰ÂÌÓ‚ËÛÒÂÌ ‚ÂÍÚÓ, ÍÓÂÚÓ Â ÔÓÒΉ‚‡ÌÓ ÓÚ Ôӂ˯‡‚‡Ì ̇ ˆÂÛÎÓÔ·ÁÏË̇ ‚ ÒÂÛχ Ë Ì‡ ωڇ ‚ ËÁÔ‡ÊÌÂÌËflÚ‡ (22).

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ãàíÖêÄíìêÄ 1. Ferenci P. Review article: diagnosis and current therapy of Wilson`s disease. Aliment Pharmacol Ther 2003; 18:1-9 2. Gitlin Jonathan, Wilson Disease, Gastroenterology 2003; 125:1868-1877 3. Britton RS. Metal-induced hepatotoxicity. Semin Liver Dis 1996;16:3-12 4. Loudianos G, Dessi V, Lovicu M, Angius A, Figus A, Lilliu F, De Virgiliis S, Nurchi AM, Deplano A, Moi P, Pirastu M, Cao A. Hum Mutat. 1999; 14(4): 294-303 5. Assistant Professor Celia H. Chamg, MD Emedecine, March 27, 2002; 6. Gow PJ, Smallwood RA, Angus PW, Smith AL, Wall AJ Diagnosis of Wilson’s disease an experience over three decades, Gut 2000, 46:415-419 7. Danks DM, Mets G, Sewell R Wilson Disease in adults with cirrhosis but no neurological abnormalities BrMedS 1990; 301:331-332 8. Roberts E., Schilsky M A Practice Guideline on Wilson Disease Hepa tology, Vol.37, ‹6, 2003 9. Rahil Shah, MD,Emedecine april 28, 2003 10. Smith CK, Mattsou RH Seizures in Wilson disease, Neurology 1967; 17:1121-13 11. Denning TR, Berrious GE, Walshe JM, Wilson’s disease and epilepsy, Brain 1988; 111:1139-55 12. Baban NK; Hubbs DT; and Roy TM; Wilson’s Disease, South Med J. 1997 May; 90(5):535-8 13. Hypercalciuria in hepatolenticular degeneration (Wilson's disease). Am. J. Med. Sci. 238: 614-620, 1959.; PubMed ID : 14417517 14. Wiebers, D. O.; Wilson, D. M.; McLeod, R. A.; Goldstein, N. P.: Renal stones in Wilson's disease. Am. J. Med. 67: 249-254, 1979. PubMed ID : 463930 15. Hlubocka, Z.; Marecek, Z.; Linhart, A.; Kejrova, E.; Pospisilova, L.; Martasek, P.; Aschermann, M. : Cardiac involvement in Wilson disease. J. Inherit. Metab. Dis. 25: 269-277, 2002.; PubMed ID : 12227457 16. Seniow, J., BAK, T., Gajda, J., Poniatowska, R., Czlonkowska, A.Title : Cognitive Functioning in neurologically symptomatic and asymptomatic forms of Wilson's Disease, Reference: Movement Disorders Year : 2001 17. van Wassenaer-van Hall, H. N.; van den Heuvel, A. G.; Jansen, G. H.; Hoogenraad, T. U.; Mali, W. P. T. M. Cranial MR in Wilson disease: abnormal white matter in extrapyramidal and pyramidal tracts. Am. J. Neuroradiol. 16: 20212027, 1995. PubMed ID : 8585490 18. Brewer, G. J.; Hedera, P.; Kluin, K. J.; Carlson, M.; Askari, F.; Dick, R. B.; Sitterly, J.; Fink, J. K. : Treatment of Wilson disease with ammonium tetrathiomolybdate: III. Initial therapy in a total of 55 neurologically affected patients and follow-up with zinc therapy. Arch. Neurol. 60: 379-385, 2003. PubMed ID 12633149 19. Wu, Z.-Y.; Lin, M.-T.; Murong, S.-X.; Wang, N. : Molecular diagnosis and prophylactic therapy for presymptomatic Chinese patients with Wilson disease. Arch. Neurol. 60: 737-741, 2003. PubMed ID : 12756138 20. Brewer, G. J.; Dick, R. D.; Johnson, V. D.; Brunberg, J. A.; Kluin, K. J.; Fink, J. K. : Treatment of Wilson's disease with zinc. XV. Long-term follow-up studies. J. Lab. Clin. Med. 132: 264-278, 1998. PubMed ID : 9794697 21. Treatment of Wilson's disease with zinc. XVII: treatment during pregnancy.Brewer GJ, Johnson VD, Dick RD, Hedera P, Fink JK, Kluin KJ. Hepatology. 2000 Feb;31(2):364-70. 22. Ha-Hao D, Merle U, Hofmann C, Wesch H, Doll J, Auburger G, Tuma S, Strauss M, Stremmel W. Z Chances and shortcomins of adenovirus mediated ATP7B gene transfer in Wilson disease: proof of principle demonstrated in a pilot study with LEC rats. Gastroenterol. 2002 Apr; 40(4): 209-16. 23. Wilson SAK. Progressive lenticular degeneration& a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34:295-507 24. Wu ZY, Wang N, Lin MT, Fang L, Murong SX, Yu L. Mutation analysis and the correlation between genotype and phenotype of Arg778Leu mutation in chinese patients with Wilson disease. Arch Neurol. 2001 Jun;58(6):971-6. 25. Yoo, H. W. : Identification of novel mutations and the 3 most common mutations in the human ATP7B gene of Korean patients with Wilson disease. Genet. Med. 4: 43S-48S, 2002. PubMed ID : 12544487 26. 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Topcu M, Topcuoglu M, Kose G, Nurlu G, Turanli G; Evoked Potentials in Children with Wilson;s Disease; Brain&Development 24 (2002) 276-280

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Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

140

é·ÁÓ ïàêìêÉàóçé ãÖóÖçàÖ çÄ ÄìíàëíàëíàóçéÖèàãÖèíàîéêåÖçÄ êÖÉêÖëàü à LANDAU-KLEFFNER ëàçÑêéå â. è‡ÌÓ‚1, ã. è‡ÌÓ‚‡2 ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡ “ãÓÁÂ̈” – ÒÂÍÚÓ “Ì‚ÓıËÛ„Ëfl” -ëÓÙËfl, 2Ñäñ ïï- ëÓÙËfl

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SUMMARY SURGICAL TREATMENT OF AUTISTIC EPILEPTIFORM REGRESSION AND LANDAU- KLEFFNER SYNDROME Y. Panov, L. Panova This article presents a summary of clinical studies and discusses traditional medical treatments, patient selection and outcome of children who underwent neurosurgical intervention for treatment of autistic epileptiform regression and LandauKleffner syndrome (LKS). Diagnostic studies included videoelectroencephalogram (EEG) monitoring, magnetic resonance (MR) imaging, positron emission tomography (PET), magnetoencephalography (MEG), intraoperative electrocorticography (EcoG). The indications for surgical intervention are brain lesion or epilepsy refractory to antiepileptic treatment medication. Surgical treatment includ lesionectomy or focal cortical resections, lobectomy, multiple subpial transections(MSTs). The results demonstrate that epilepsy surgery can be performed with acceptable morbidity. Verbal aphasia is an frequent sequela when the focus is on the speech-dominant side. Despite the multifocal nature of the epileptiform activity, neurosurgical intervention aimed at control has lead to a reduction of autistic features and improvement in language skills Seizure outcome after surgical treatment is generally satisfactory, with 80% significantly improvement. Key words: autistic epileptiform regression, LandauKleffner syndrome, neurosurgical treatment. êÖáûåÖ è‰ÒÚ‡‚ÂÌË Ò‡ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÍÎËÌ˘ÌË ËÁÒΉ‚‡ÌËfl Ë Ò ‰ËÒÍÛÚË‡ ωË͇ÏÂÌÚÓÁÌÓÚÓ Î˜ÂÌËÂ, ÒÂÎÂ͈ËflÚ‡ Ë ËÁıÓ‰‡ ÓÚ ÓÔÂ‡ÚË‚ÌÓ Î˜ÂÌË ̇ ‰Âˆ‡ Ò ‡ÛÚËÒÚ˘ÌÓ-ÂÔËÎÂÔÚËÙÓÏÂ̇ „ÒËfl Ë Landau-Kleffner ÒË̉ÓÏ. èÓÒÓ˜ÂÌË Ò‡ ÌÂÓ·ıÓ‰ËÏËÚ Ô‰ÓÔÂ‡ÚË‚ÌË ËÁÒΉ‚‡ÌËfl: üåê, ÙÛÌ͈ËÓ̇ÎÂÌ üåê, èÖí, ‚ˉÂÓ ÖÖÉ, χ„ÌËÚÓÂ̈ÂÙ‡ÎÓ„‡ÙËfl Ë ÂÚ‡ÔËÚ ̇ ΘÂÌË ‰Ó ÔÓÒÚ‡‚flÌ ̇ Ë̉Ë͇ˆËË Á‡ ÓÔÂ‡ˆËfl- ̇΢ˠ̇ ÏÓÁ˙˜Ì‡ ÎÂÁËfl ËÎË ÂÁËÒÚÂÌÚÌÓ Ì‡ ‡ÌÚËÍÓÌ‚ÛÎÒË‚ÌÓ Î˜ÂÌË ÂÔËÎÂÔÚÓ„ÂÌÌÓ Ó„ÌˢÂ. é·Ò˙ʉ‡Ú Ò ÔÓ͇Á‡ÌËflÚ‡ Á‡ ‡Á΢ÌË Ì‚ÓıËÛ„˘ÌË Ì‡ÏÂÒË: ÎÓ·ÂÍÚÓÏËfl, ÂÍÒˆËÁËfl ̇ ÎÂÁËflÚ‡, ÏÛÎÚËÔÎÂ̇ ÒÛ·ÔˇÎ̇ Ú‡ÌÒÂ͈Ëfl, ÏËÌËχÎ̇ ÍÓÚË͇Î̇ ÂÁÂ͈Ëfl. éÚ˜ËÚ‡ Ò ÔÓ‰Ó·ÂÌË ÔÓ ÓÚÌÓ¯ÂÌË ̇ „Ó‚Ó‡ Ë ‡ÛÚËÒÚ˘̇ڇ „ÂÒËfl, ÔË ‰ÓÔÛÒÚËÏË ÓÔÂ‡ÚË‚ÌË ÛÒÎÓÊÌÂÌËfl. à̉Ë͇ˆËËÚ Úfl·‚‡ ‰‡ ·˙‰‡Ú ÔÓÒÚ‡‚ÂÌË Ò ‚ÌËχÌËÂ, ÓÒÓ·ÂÌÌÓ ‚ ÒÎÛ˜‡ËÚ ̇ ‡Ì„‡ÊË‡Ì ̇ ÏÓÚÓÌË ÁÓÌË, ˜ËÂÚÓ ‚ÚÓ˘ÌÓ Û‚Âʉ‡Ì  ̇È- ˜ÂÒÚÓÚÓ ÛÒÎÓÊÌÂÌË ÒΉ ËÌÚÂ‚Â̈ËflÚ‡. ç‡Ô‡‚ÂÌÓ Â Á‡Íβ˜ÂÌËÂ, ˜Â Ì‚ÓıËÛ„ËflÚ‡ ‰Ó͇Á‚‡ Ò‚ÓflÚ‡ ÂÙË͇ÒÌÓÒÚ ÔË Ú‡ÁË ÚÂÊ͇ Ô‡ÚÓÎÓ„Ëfl ‚ 80% ÓÚ ÒÎÛ˜‡ËÚÂ. äβ˜Ó‚Ë ‰ÛÏË: ‡ÛÚËÒÚ˘ÌÓ-ÂÔËÎÂÔÚËÙÓÏÂ̇ „ÒËfl, Landau-Kleffner ÒË̉ÓÏ, Ì‚ÓıËÛ„˘ÌÓ Î˜ÂÌËÂ. Ç ÔÓÒΉÌËÚ „Ó‰ËÌË ‚ Ö‚ÓÔ‡ Ë ë‚Â̇ ÄÏÂË͇, ‰ÓË ‚ ÒÚ‡ÌË, ÍÓËÚÓ Ò ӈÂÌfl‚‡Ú ͇ÚÓ ‡Á‚Ë‚‡˘Ë ÒÂ, ‡Á‡·ÓÚ‚‡Ú ÔÓ„‡ÏË Á‡ ‡Á‚ËÚË ̇ ıËÛ„Ëfl ̇ ÂÔËÎÂÔÒËflÚ‡ Ë ‚ ˜‡ÒÚÌÓÒÚ ÔË ‡ÛÚËÒÚ˘ÌÓ-ÂÔÂÎÂÔÚËÙÓÏÂ̇ „ÒËfl /ÄÖê/ Ë Landau-Kleffner ÒË̉ÓÏ /ãäë/. Ç˙‚ î‡ÌˆËfl ‰ÌÓ-

„Ӊ˯ÌÓ Ì‡·Î˛‰ÂÌËÂ, ÔÓ‰„ÓÚӂ͇, ÓÔÂ‡ˆËfl Ë ÔÓÒÚÓÔÂ‡ÚË‚ÌË „ËÊË ÒÚÛ‚‡Ú 100000 ‚Ó Á‡ Ô‡ˆËÂÌÚ. Ç óËÎË5000 ‰Ó·‡ ÔÓ ‰‡ÌÌË Ì‡ (3), ‚ à̉Ëfl (16) ˆflÎÓÒÚÌÓÚÓ Î˜ÂÌË ̇ Ú‡ÍË‚‡ ·ÓÎÌË Â ÓˆÂÌÂÌÓ Ì‡ 1200 ‰Ó·‡. ü‚ÌÓ Ì‡‚ÒflÍ˙‰Â ÔÓ Ò‚ÂÚ‡ ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÙË̇ÌÒÓ‚ËÚÂ Ë Ó„‡ÌËÁ‡ˆËÓÌÌË ÔÓ·ÎÂÏË ÔˆÂÌ͇ڇ Â, ˜Â ωˈË̇ڇ  ‰Î˙ÊÌËÍ Ì‡ ÚÂÁË ·ÓÎÌË Ë ÛÒËÎËflÚ‡ ‚ Ú‡ÁË Ì‡ÒÓ͇ ÒË ÒÚÛ‚‡Ú. ë‰ ÔÓÔÛ·ˆËflÚ‡ ̇ ‰Âˆ‡– ‡ÛÚËÒÚË Ì‡·Î˛‰‡‚‡ÌËÚ ÂÔËÎÂÔÚ˘ÌË ÍËÁË ËÎË „ËÒÚ‡ˆËfl ̇ Ô‡ÚÓÎӄ˘Ìa ÖÖÉ „Ó‚ÓflÚ Á‡ ÏÓÁ˙˜Ì‡ ‰ËÒÙÛÌ͈Ëfl ËÎË ÏÓÁ˙˜Ì‡ ÎÂÁËfl Ò ‡Á΢̇ ÂÚËÓÎÓ„Ëfl, ÍÓflÚÓ ËÁËÒÍ‚‡ ‡ÌÚËÍÓÌ‚ÛÎÒË‚ÌÓ Î˜ÂÌË ËÎË Ì‚ÓıËÛ„˘ÌÓ Î˜ÂÌËÂ, ÔÓÌflÍÓ„‡ ‚ ÒÔ¯ÂÌ ÔÓfl‰˙Í. (14) Ô‰ÒÚ‡‚flÚ ‰ÂÚÂ Ò ‡ÛÚËÒÚ˘ÌÓ-ÂÔËÎÂÔÚËÙÓÏÂ̇ „ÂÒËfl, ‡·ÌÓÏÌÓ ÖÖÉ– ·‡‚ÌË ÔËÍ-‚˙Î̇ ÍÓÏÔÎÂÍÒË, Ò ÛÒÚ‡ÌÓ‚Â̇ ıËÔÓڇ·Ï˘̇ ı‡Ï‡ÚÓχ- ÒΉ ÓÚÒ‡Ìfl‚‡ÌÂÚÓ Ì‡ ÚÛÏÓ‡ Ò ÓÚ˜ËÚ‡ ËÁ˜ÂÁ‚‡Ì ̇ ÔËÒÚ˙ÔÌËÚ ÂÔËÁÓ‰Ë Ì‡ ËÒÚÂ˘ÂÌ ÒÏflı, ÒÚÂÂÓÚËÔ˘ÌÓÚÓ Ôӂ‰ÂÌËÂ, ÂÔËÎÂÔÚ˘ÌËÚ ÔËÒÚ˙ÔË. ëË̉ÓÏ˙Ú ÄÖê  ÓÔËÒ‡Ì ÓÚ (20). ë˙˘ÂÒÚ‚Û‚‡ ÔËÎË͇ ̇ ÒË̉Óχ Ò ãäë Ë ‰ÓË ÏÓÊ ‰‡ Ò Ò¢Ì ‚ ÎËÚÂ‡ÚÛ‡Ú‡ ͇ÚÓ ãäë ‚‡ˇÌÚ. ëÔÓ‰ (13), ãäë Ò ‡Á‚Ë‚‡ ÒΉ 3 „Ӊ˯̇ ‚˙Á‡ÒÚ, ÄÖê ÔÂ‰Ë 3, ̇Û¯ÂÌËflÚ‡ ‚ Ôӂ‰ÂÌËÂÚÓ ÔË ãäë  ‚‡ˇ·ËÎÌÓ, Á‡‰˙ÎÊËÚÂÎ̇  ÏÓÚÓ̇ڇ ‡Ù‡ÁËfl, ÔË ÄÖê ̇Û¯ÂÌËflÚ‡ ‚ ÒӈˇÎËÁ‡ˆËflÚ‡ Ò‡ ÚÂÊÍË Ë ‰ÓÏËÌË‡Ú. ÖÔËÎÂÔÚÓ„ÂÌÌÓÚÓ Ó„Ìˢ ÔË ãäë  ‚ „Ó‚Ó̇ ÏÓÚÓ̇ ÁÓ̇ ̇ ÍÓ‡Ú‡ ‰ÌÓÒÚ‡ÌÌÓ, ÔË ÄÖê Ò‡ ‰‚ÛÒÚ‡ÌÌË– ÏÛÎÚËÙÓ͇ÎÌË Ë ÏÛÎÚËÎÓ·‡ÌË. ñflÎÓÒÚÌÓÚÓ ‰Ë‡„ÌÓÒÚ˘ÌÓ ÛÚÓ˜Ìfl‚‡ÌÂ Ë Î˜ÂÌË ̇ ‰Âˆ‡ Ò ‡ÛÚËÁ˙Ï Ò ‚Ó‰Ë ÓÚ Ô‰ÓÔÒËıˇÚ˙ Ë ÓÚÓÙÓÌËÒÚ, ËÁÒΉ‚‡˘ ‚ËÒ¯Ë ÍÓÓ‚Ë ÙÛÌ͈ËË Ë ÔÓ‚Âʉ‡˘ ÚÂÒÚÓ‚Ó Ó·Û˜ÂÌË ÔË Ô‡ÚÓÎӄ˘̇ڇ ËÏ ÔÓÏfl̇. è‰ÓÔÒËıˇÚ˘̇ڇ ‰Ë‡„ÌÓÁ‡  ÒÔÓ‰ Í·ÒËÙË͇ˆËËÚ DSM IV, DSM III R Ë Ò͇· CARS. è‰ÓÔÂ‡ÚË‚ÌÓÚÓ ËÁÒΉ‚‡Ì ‚Íβ˜‚‡: ● üåê- Á‡ ÛÚÓ˜Ìfl‚‡Ì ̇ ‡Ì‡ÚÓÏ˘ÌË ÎÂÁËË ● ÙÛÌ͈ËÓ̇ÎÂÌ üåê– ÔÓÁ‚ÓÎfl‚‡˘ ͇ÚÓ„‡ÙË‡Ì ̇ ÏÓÚÓÌË ÁÓÌË Ë ÓÔ‰ÂÎflÌ ·ÚÂ‡ÎËÁ‡ˆËflÚ‡ ̇ „Ó‚Ó̇ ÏÓÚÓ̇ ÁÓ̇ ● ÔÓÁËÚÓÌ̇ ÂÏËÒËÓÌ̇ ÚÓÏÓ„‡ÙËfl– ËÁÔÓÎÁ‚‡Ú Ò ·ËÓÎӄ˘ÌË ÏÓÎÂÍÛÎË Ï‡ÍË‡ÌË Ò ‡‰ËÓËÁÓÚÓÔ, ˜ÂÁ ÍÓËÚÓ Ò ÓÚ˜ËÚ‡ ÂÔËÎÂÔÚÓ„ÂÌÌÓÚÓ Ó„Ìˢ ͇ÚÓ ÁÓ̇ ̇ ıËÔÓÏÂÚ‡·ÓÎËÁ˙Ï ● χ„ÌËÚÓÂ̈ÂÙ‡ÎÓ„‡ÙËfl- ÌÂËÌ‚‡ÁË‚ÂÌ ÏÂÚÓ‰ Á‡ ÓÚÍË‚‡Ì ̇ Ô‡ÚÓÎӄ˘̇ ÂÎÂÍÚÓÙËÁËÓÎӄ˘̇ ÏÓÁ˙˜Ì‡ ‡ÍÚË‚ÌÓÒÚ ÔÓ ‚ÂÏ ̇ Ò˙Ì ● ‚ˉÂÓ ÖÖÉ–24 ˜‡ÒÓ‚ ÏÓÌËÚÓËÌ„ ë˙˘ÂÒÚ‚Û‚‡Ú ‰‚ „ÛÔË ·ÓÎÌË Ò ÄÖê ËÎË ãäë ͇̉ˉ‡ÚË Á‡ Ì‚ÓıËÛ„˘ÌÓ Î˜ÂÌËÂ. Ç Â‰ËÌËfl ÒÎÛ˜‡È  ̇Îˈ ˆÂ·‡Î̇ ‡Ì‡ÚÓÏ˘̇ ÎÂÁËfl, ‚ ˜‡ÒÚÌÓÒÚ Ò ÍÓÏÔÂÒËfl ‚˙ıÛ ÏÓÁ˙˜ÌËfl Ô‡ÂÌıËÏ, ‡ ‚ ‰Û„Ëfl ÎËÔÒ‚‡Ú Ô‡ÚӇ̇ÚÓÏ˘ÌË ÔÓÏÂÌË, ÌÓ ÂÔË- ÔËÒÚ˙ÔËÚ ËÎË Ô‡ÚÓÎӄ˘̇ڇ ·ËÓÂÎÂÍÚ˘̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÏÓÁ˙͇ Ò‡ ÂÁËÒÚÂÌÚË Ì‡ ‡ÌÚËÍÓÌ‚ÛÎÒË‚ÌÓ Î˜ÂÌËÂ. ëÔÓ‰ ÒÂËflÚ‡ ̇ (19) et al, 1999, Ô‡ÚÓÎӄ˘ÌËÚ ÎÂÁËË Ì‡ „·‚ÌËfl ÏÓÁ˙Í ‰Ó‚ÂÎË ‰Ó ‡Á‚ËÚËÂÚÓ Ì‡ ÄÖê Ò ÔÓ‰Âʉ‡Ú ÔÓ ˜ÂÒÚÓÚ‡ ÔÓ ÒΉÌËfl ̇˜ËÌ: ● Ì‚ÓÂÔËÚÂΡÎÌË ‰ËÒÂÏ·Ëӷ·ÒÚ˘ÌË ÚÛÏÓË


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

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ÍÓÚË͇Î̇ ‰ËÒÔ·ÁËfl ÚÛ·ÂÓÁ̇ ÒÍÎÂÓÁ‡ ıÂÏËÍÓÚË͇ÎÌË ÎÂÁËË ÚÂÏÔÓ‡Î̇ ÒÍÎÂÓÁ‡ ÔÓÒÚÂ̈ÂÙ‡ÎËÚÌË ÎÂÁËË íÂ‡Ô‚Ú˘̇ڇ ÒıÂχ  Ô‰ÒÚ‡‚Â̇ ‚ ÚË ÂÚ‡Ô‡ (9) êÂÁÛÎÚ‡ÚËÚ ÓÚ Ï‰Ë͇ÏÂÌÚÓÁÌÓÚÓ Î˜ÂÌË ÔË ÚÓÁË ÚËÔ ·ÓÎÌË Â Á‡‰Ó‚ÓÎËÚÂÎÂÌ ‚ ÓÍÓÎÓ 50% ÓÚ ÒÎÛ˜‡ËÚÂ. Ç ÓÒڇ̇ÎËÚ Ò ӷÒ˙ʉ‡ ıËÛ„˘ÌÓ Î˜ÂÌËÂ. (4) ‰ÓÍ·‰‚‡Ú ÒÎÛ˜‡È ̇ ‰ÂÚÂ Ò ÚÛ·ÂÓÁ̇ ÒÍÎÂÓÁ‡, Ò ‡ÛÚËÒ퇷Îˈ‡ 1: ÖÚ‡ÔË Ì‡ ΘÂÌË ÔË ÄÖê Ë ãäë ë ‡Ì‡ÚÓÏ˘̇ ÎÂÁËfl I ÂÚ‡Ô Depakine, Depacote, Sabrile, Urbanil Lamictal

II ÂÚ‡Ô ïËÛ„˘ÌÓ Î˜ÂÌËÂ

ÅÂÁ ‡Ì‡ÚÓÏ˘̇ ÎÂÁËfl I ÂÚ‡Ô Depakine, Depacote II ÂÚ‡Ô ACTH, ÒÚÂÓË‰Ë III ÂÚ‡Ô ïËÛ„˘ÌÓ Î˜ÂÌËÂ

Ú˘̇ „ÂÒËfl, Ò flÁÍÓ ÔÓ‰Ó·fl‚‡Ì ̇ ÍÓ„ÌËÚË‚ÌËÚ ÒÔÓÒÓ·ÌÓÒÚË ÒΉ ÓÔÂ‡ˆËflÚ‡. (8) Ô‰ÒÚ‡‚flÚ ÔÓ‰Ó·ÂÌ ÒÎÛ˜‡È ̇ ‰ÂÚÂ Ò ÂÔËÎÂÔÒËfl Ë ‡ÛÚËÒÚ˘̇ „ÂÒËfl, Ò ÌÂÒÚ‡·ËÎÌË ÂÁÛÎÚ‡ÚË ‚˙‚ ‚ÂÏÂÚÓ ÒΉ ËÌÚÂ‚Â̈ËflÚ‡ ÔÓ‡‰Ë ÏÌÓÊÂÒÚ‚ÂÌÌÓÒÚÚ‡ ̇ ÎÓ͇ÎËÁ‡ˆËflÚ‡. ÄÍÓ ÎÂÁËflÚ‡  ‚˙‚ ÙÛÌ͈ËÓ̇ÎÌÓ Ì‡ÍÚ˂̇ ӷ·ÒÚ Ò ËÁ‚˙¯‚‡ ÎÓ·ÂÍÚÓÏËfl ËÎË ÓÚÒÚ‡Ìfl‚‡Ì ҇ÏÓ Ì‡ Ô‡ÚÓÎӄ˘ÌÓÚÓ fl‰Ó. èË Ì‡Î˘Ë ̇ ÂÔËÎÂÔÚÓ„ÂÌÌÓ Ó„Ìˢ ‚˙‚ ÙÛÌ͈Ë̇ÎÌÓ Á̇˜Ëχ ÁÓ̇ Ò ÔË·„‡Ú: ● ÏÛÎÚËÔÎÂ̇ ÒÛ·ÔˇÎ̇ Ú‡ÌÒÂ͈Ëfl -åëí ● ÏËÌËχÎ̇ ÍÓÚË͇Î̇ ÂÁÂ͈Ëfl /„ËÂÍÚÓÏËfl/- åäê èË åëí (2), (15) Ò ËÁ‚˙¯‚‡ ÒÂ͈Ëfl ̇ ÍÓÚËÍÓÍÓÚË͇ÎÌËÚ ıÓËÁÓÌÚ‡ÎÌË Ì‚Ó̇ÎÌË ‚˙ÁÍË. èÓ ÚÓÁË Ì‡˜ËÌ Ò ÔÂÍ˙Ò‚‡ ‡ÁÔÓÒÚ‡ÌÂÌËÂÚÓ Ì‡ Ô‡ÚÓÎӄ˘̇ڇ ÂÎÂÍÚÓÙËÁËÓÎӄ˘̇ ‡ÍÚË‚ÌÓÒÚ ÔË Ò˙ı‡ÌÂÌË ̇ ÔÓ‰ÎÂʇ˘ËÚ ‚ÂÚË͇ÎÌË ‡ÍÒÓÌË. àÌÚ‡ÓÔÂ‡ÚË‚ÂÌ ÍÓÌÚÓÎ Ò ÓÒ˙˘ÂÒÚ‚fl‚‡ Ò ÂÎÂÍÚÓÍÓÚËÍÓ„‡ÙËfl. Ç ÒÎÛ˜‡È ̇ ÔÂÒËÒÚË‡Ì ̇ Ô‡ÚÓÎӄ˘ÌÓ ÖÖÉ ‚ ÁÓ̇ڇ ̇ ËÌÚÂÂÒ, ÓÔÂ‡ˆËflÚ‡ Ò ÔÓ‰˙Îʇ‚‡ Ò åäê ‚˙ıÛ 1- 2 ÁÓÌË ÓÚ 0,5 ‰Ó 1 ÒÏ ‚ ‰Ë‡ÏÂÚ˙. åÂÚÓ‰˙Ú Â ËÁ‚ÂÒÚÂÌ Ë Ò ÔË·„‡ Ë ÔË ‚˙Á‡ÒÚÌË Ò ÂÙ‡ÍÚÂ̇ ÂÔËÎÂÔÒËfl. (12) ‰ÓÍ·‰‚‡Ú Ô˙ÎÌÓ ËÁÎÂÍÛ‚‡Ì ̇ 50% ÓÚ ·ÓÎÌËÚ ÒΉ ÓÔÂ‡ˆËflÚ‡, ÔË 32% ÓÚ˜ËÚ‡Ú ÔÓ‰Ó·ÂÌË ËÁ‡Áfl‚‡˘Ó Ò ‚ ‰ۈË‡Ì ̇ ‡ÌÚËÍÓÌ‚ÛÎÒË‚ÌÓÚÓ Î˜ÂÌËÂ Ë ÔËÔ‡‰˙ˆËÚÂ, ÔË 18% ÓÚ ·ÓÎÌËÚ Ò ÓÚ˜ËÚ‡ ‚ÎÓ¯‡‚‡Ì ÓÚ 2 ‰Ó 7 „Ó‰ËÌË ÒΉ ÓÔÂ‡ˆËflÚ‡. èË ‰Âˆ‡ Ò ÄÖê ·ÂÁ ‡Ì‡ÚÓÏ˘̇ ÎÂÁËfl, ÌÂÔÓ‚ÎËfl‚‡˘Ë Ò ÓÚ ‡ÌÚËÍÓÌ‚ÛÎÒË‚ÌÓ Î˜ÂÌËÂ, ‡ÌÌÓÚÓ ÔÓÒÚ‡‚flÌ ̇ Ë̉Ë͇ˆËË Á‡ ÓÔÂ‡ÚË‚ÌÓ Î˜ÂÌË ÏÓÊ ‰‡ Ó„‡ÌË˜Ë Í‡Ú‡ÒÚÓÙ‡ÎÌÓÚÓ ‡Á‚ËÚË ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. (9) ‰̇ „Ó‰Ë̇ ÒΉ ÓÔÂ‡ˆËfl ÓÚ˜ËÚ‡ ÔÓ‰Ó·fl‚‡Ì ‚ CARS ÓÚ 1 ̇ 9 ÚÓ˜ÍË. ëΉ ËÌÚÂ‚Â̈Ëfl ÓÚ ÚËÔ‡ åëê Ë åäê, (13) ÓÚ·ÂÎflÁ‚‡Ú ‰Û͈Ëfl ̇ ÂÔËÎÂÔÚ˘ÌËÚ ÍËÁË Ôӂ˜ ÓÚ 90%, ‰Û͈Ëfl ̇ ÒÚÂÂÓÚËÔÌËÚ ‰‚ËÊÂÌËfl, ̇ ıÂÚÂÓ Ë ‡‚ÚÓ‡„ÂÒË‚ÌÓÒÚÚ‡, ÔÓ-‰Ó·‡ ÒӈˇÎ̇ ‡‰‡ÔÚ‡ˆËfl Ë ÛÔÓÚ·‡ ̇ ÂÁË͇. (10) ÔÛ·ÎËÍÛ‚‡ ÒÂËfl ÓÚ 7 ‰Âˆ‡ Ò ‡ÛÚËÁ˙Ï, ÔÓÌÂÒÎË MëT ‚ Îfl‚‡ ÙÓÌÚ‡Î̇, Ô‡ËÂÚ‡Î̇ Ë ÚÂÏÔÓ‡Î̇ ӷ·ÒÚ ‚Íβ˜‚‡˘‡ ˜ÂÒÚÓ Ë ÏÓÚÓ̇ „Ó‚Ó̇ ÁÓ̇, ÔË Â‰ÌÓ ‰ÂÚ  ËÁ‚˙¯Â̇ Ë ÚÂÏÔÓ‡Î̇ ÎÓ·ÂÍÚÓÏËfl, ͇ÚÓ ÔË ‚Ò˘ÍË Â ÓÚ˜ÂÚÂÌÓ ÔÓ‰Ó·ÂÌËÂ. (1) Ô‰ÒÚ‡‚flÚ 30 ‰Âˆ‡ Ò ÂÔËÎÂÔÒËfl, ÔË ÍÓËÚÓ Â ËÁ‚˙¯Â̇ åëí. èË 12 ‰Âˆ‡  ÓÚ˜ÂÚÂÌ ÎÂÍ ‰Ó ÛÏÂÂÌ ÏÓÚÓÂÌ ‰ÂÙˈËÚ ÒΉ ËÌÚÂ‚Â̈Ëfl ‚ ÏÓÚÓ̇ ÁÓ̇. ç‚ÓÎӄ˘ÌËÚ ÛÒÎÓÊÌÂÌËfl Ò‡ Ò ÔÂıÓ‰ÂÌ ı‡‡ÍÚÂ- ‰Ó 6 Ò‰ÏËˆË ÒΉ ÓÔÂ‡ˆËflÚ‡. Ä‚ÚÓËÚ ÓÚ·ÂÎflÁ‚‡Ú 40% Ô˙ÎÌÓ ËÁÎÂÍÛ‚‡ÌÂ. (7) ÔÛ·ÎËÍÛ‚‡Ú ÏÌÓ„Ó ‰Ó·Ë ÂÁÛÎÚ‡ÚË ÔË 5 ‰Âˆ‡ Ò ãäë ÒΉ

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ÓÔÂ‡Ú˂̇ ËÌÚÂ‚Â̈Ëfl –åëí, Ò ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ „Ó‚Ó‡, ÌÓχÎËÁË‡Ì ̇ ÖÖÉ, ·ÂÁ Ú‡ÈÌË Ì‚ÓÎӄ˘ÌË ÛÒÎÓÊÌÂÌËfl. (6) Ô‰ÒÚ‡‚flÚ 14 ‰Âˆ‡ Ò ãäë ÓÔÂË‡ÌË ˜ÂÁ åëí, Ò ‰‡Ï‡Ú˘ÌÓ ÔÓ‚ÎËfl‚‡ÌÂ. (17), (11) ÓÚ˜ËÚ‡Ú ÔÓÎÓÊËÚÂÎÌË ÂÁÛÎÚ‡ÚË ÓÚ ÓÔÂ‡ÚË‚ÌÓ Î˜ÂÌË ˜ÂÁ ÔÓÒÓ˜ÂÌËÚ ÏÂÚÓ‰Ë ‚ 80% ÓÚ ÒÎÛ˜‡ËÚ ÔË ÂÁËÒÚÂÌÚ̇ ̇ ωË͇ÏÂÌÚÓÁÌÓ Î˜ÂÌË ÂÔËÎÂÔÒËfl. (5) ÔË ‡Ì‡ÎËÁ ̇ ÒÂËfl Ô‡ˆËÂÌÚË ÔËÂχ,˜Â ÂÁÛÎÚ‡ÚËÚ ҇ ÔÓ- ‰Ó·Ë ÔË ÍÓÏ·ËÌË‡Ì ̇ ‰‚‡Ú‡ ÏÂÚÓ‰‡ åëí Ë åäê. (18) ÓÒ˙˘ÂÒÚ‚fl‚‡Ú Ô˙‚Ëfl ÏÂʉÛ̇Ó‰ÂÌ ‡Ì‡ÎËÁ ̇ 211 Ô‡ˆËÂÌÚ‡ ÓÔÂË‡ÌË ‚ ¯ÂÒÚ ‡Á΢ÌË ˆÂÌÚ˙‡ ˜ÂÁ åëí Ë åäê ËÎË Ò‡ÏÓ Ò åëí ͇ÚÓ ÓÚ˜ËÚ‡Ú ‰Û͈Ëfl ̇ ÔËÔ‡‰˙ˆËÚ ‚ 95% ÓÚ ÒÎÛ˜‡ËÚ ·ÂÁ ÒÚ‡ÚËÒÚ˘ÂÒ͇ ‡ÁÎË͇ ÏÂÊ‰Û ÓÔÂ‡ÚË‚ÌËÚ ÏÂÚÓ‰Ë. Ç Á‡Íβ˜ÂÌË ÏÓÊ ‰‡ Ò ͇ÊÂ, ˜Â Ì‚ÓıËÛ„ËflÚ‡ ‰Ó͇Á‚‡ Ò‚ÓflÚ‡ ÂÙË͇ÒÌÓÒÚ ÔË Ú‡ÁË ÚÂÊ͇ Ô‡ÚÓÎÓ„Ëfl. à̉Ë͇ˆËËÚ Úfl·‚‡ ‰‡ ·˙‰‡Ú ÔÓÒÚ‡‚ÂÌË Ò ‚ÌËχÌËÂ, ÓÒÓ·ÂÌÌÓ ‚ ÒÎÛ˜‡ËÚ ̇ ‡Ì„‡ÊË‡Ì ̇ ÏÓÚÓÌË ÁÓÌË, ˜ËÂÚÓ ‚ÚÓ˘ÌÓ Û‚Âʉ‡Ì  ̇È- ˜ÂÒÚÓÚÓ ÛÒÎÓÊÌÂÌË ÒΉ ËÌÚÂ‚Â̈ËflÚ‡. ãàíÖêÄíìêÄ 1. Blount JP, Langburt W, Otsubo H, Chitoku S, Ochi A, Weiss S, Snead OC, Rutka JT. Multiple subpial transections in the treatment of pediatric epilepsy. J Neurosurg Spine. 2004 Feb;100(2):118-24 2. Buelow JM, Aydelott P, Pierz DM, Heck B.Multiple subpial transection for Landau-Kleffner syndrome. AORN J. 1996 Apr;63(4):727-9, 732-5, 737-9; quiz 741-4. 3. Campos MG, Godoy J, Mesa MT, Torrealba G, Gejman R, Huete I. Temporal lobe epilepsy surgery with limited resources: results and economic considerations. Epilepsia. 2000;41 Suppl 4:S18-21 4. Deonna T, Ziegler AL, Mourra-Serra J, Innocenti G. Autistic regression in relation to limbic pathology and epilepsy. Developmental Medicine and Child Neurology. 1993; 35:158-176 5. Devinsky O, Romanelli P, Orbach D, Pacia S, Doyle W Surgical treatment of multifocal epilepsy involving eloquent cortex. Epilepsia. 2003 May; 44(5):718-23. 6.Grote CL, Van Slyke P, Hoeppner JA. Language outcome following multiple subpial transection for Landau-Kleffner syndrome. Brain. 1999 Mar; 122 ( Pt 3):561-6. 7. Irwin K, Birch V, Lees J, Polkey C, Alarcon G, Binnie C, Smedley M, Baird G, Robinson RO. Multiple subpial transection in Landau-Kleffner syndrome. Dev Med Child Neurol. 2001 Apr; 43(4):248-52. 8. Jullien A, Bouley JC. Maladie de Bourneville, epilepsie et troubles envahissants du development. Neuropsychiatrie Enfance Adolescence 2001; 49: 124-130 9. Lewine JD, Andrews R, Chez M, Patil A, Devinsky O, Smith M, Kanner A, Davis J, Funke M, Jones J, Chong B, Provensal S, Weisend M, Lee R, Orrison W. Magnetoencephalographic Patterns of Epileptiform activity in children with regressive autism spectrum disorders. Pediatrics. 1999; vol 104; 3: 405-418 10. Nass R, Gross A, Wisoff J, Devinsky O. Outcome of multiple subpial transections for autistic epileptiform regression. Pediatr Neurol. 1999 Jul; 21(1):464-70. 11. Neville BGR, Harkness WFJ, Cross JH, Cass HC, Burch VC, Lees JA, Taylor DC. Surgical treatment of severe autistic regression in childhood epilepsy. Pediatric Neurology. 1997; vol 16; 2:137-140 12. Orbach D, Romanelli P, Devinsky O, Doyle W. Late seizure recurrence after multiple subpial transections. Epilepsia. 2001 Sep; 42(9):1130-3. 13. Patil AA, Andrews R, Torkelson R. Isolation of dominant seizure foci by multiple subpial transections. Stereotact Funct Neurosurg. 1997;69(1-4 Pt 2):210-5 14. Perez-Jimenez A, Villarejo FJ, Fournier Del Castillo MC, Garcia-Penas JJ, Carreno M. Continuous giggling and autistic disorder associated with hypothalamic hamartoma. Epileptic Disord. 2003 Mar; 5(1):31-7. 15. Polkey CE. Multiple subpial transection. Adv Tech Stand Neurosurg. 2000;26:3-23. 16. Rao MB, Radhakrishnan K. Is epilepsy surgery possible in countries with limited resources? Epilepsia. 2000; 41 Suppl 4:S31-4. 17. Shimizu H. Surgical treatment for intractable epilepsy. Rinsho Shinkeigaku. 2001 Dec; 41(12):1094-6. 18. Spencer SS, Schramm J, Wyler A, O'Connor M, Orbach D, Krauss G, Sperling M, Devinsky O, Elger C, Lesser R, Mulligan L, Westerveld M. Multiple subpial transection for intractable partial epilepsy: an international meta-analysis. Epilepsia. 2002 Feb; 43(2):141-5. 19. Taylor DC, Neville BG, Cross JH.Autistic spectrum disorders in childhood epilepsy surgery candidates.Eur Child Adolesc Psychiatry. 1999 Sep; 8(3):189-92. 20. Tuchman R. Acquired epileptiform aphasia. Semin Pediatric Neurol. 1997; 4: 93-101

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- âÓ‡Ì è‡ÌÓ‚ – Á‡‚. ÒÂÍÚÓ ◊Ì‚ÓıËÛ„Ëfl” ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡ ◊ãÓÁÂ̈” ‹ 1, äÓÁflÍ 1407 ëÓÙËfl íÂÎ. 9607389 E- mail: ts_panov@yahoo.com


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

142

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à. í˙Ì‚1,6, ê. LJÓÌ2, Ñ. ÄÌ„Â΢‚‡3, Ç. ÉÂ„ÂΘ‚‡1,6, ê. ÉÛ‰ËÌ„3, Å. âÓÎ4, Å. à¯ÔÂÍÓ‚‡1, ê. äËÌ„5, å. ê‡È˜Â‚‡1, á. ä‡ÏÂÌÓ‚1, ë. óÂÌËÌÍÓ‚‡1, Ä. íÓÏÓ‚1, ê. èÂÚÍÓ‚1, Ç. ÅÓÊËÌÓ‚‡1, å. 䇈‡Ó‚‡1, Ä. òχÓ‚1, à. ãËÚ‚ËÌÂÌÍÓ1, ê. íË̘‚‡1, ï. ñÂÍÓ‚1, è. ÅÓËÒÓ‚‡7, Å. ÅÛÊÓ‚1, û. èÂÚÓ‚‡1, è.ä. íÓχÒ5, ã. ä‡Î‡È‰ÊË‚‡3 1å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ – ëÓÙËfl; àÌÒÚËÚÛÚ ÔÓ „ÂÌÂÚË͇ ̇ ˜Ó‚Â͇, ïÛÏ·ÓÎÚÓ‚ ÛÌË‚ÂÒËÚÂÚ, ÅÂÎËÌ, ÉÂχÌËfl; 3 ㇷÓ‡ÚÓËfl ÔÓ ÏÓÎÂÍÛÎfl̇ „ÂÌÂÚË͇ ̇ ˜Ó‚Â͇, á‡Ô‡‰ÌÓ-Ä‚ÒÚ‡ÎËÈÒÍË ËÌÒÚËÚÛÚ Á‡ ωˈËÌÒÍË ËÁÒΉ‚‡ÌËfl, ìÌË‚ÂÒËÚÂÚ Ì‡ á‡Ô‡‰Ì‡ Ä‚ÒÚ‡ÎËfl, è˙Ú; 4 ä‡Ú‰‡ ÔÓ ÍÎËÌ˘̇ Ì‚ÓÙËÁËÓÎÓ„Ëfl, ìÌË‚ÂÒËÚÂÚ Ì‡ ãÓ̉ÓÌ, ÇÂÎËÍÓ·ËÚ‡ÌËfl; 5 ä‡Ú‰‡ ÔÓ ÍÎËÌ˘ÌË Ì‚Ó̇ÛÍË, ìÌË‚ÂÒËÚÂÚ Ì‡ ãÓ̉ÓÌ, ÇÂÎËÍÓ·ËÚ‡ÌËfl; 6 îÓ̉‡ˆËfl “á‰‡‚ÌË ÔÓ·ÎÂÏË Ì‡ χΈËÌÒÚ‚‡Ú‡” - ëÓÙËfl; 7 àÌÒÚËÚÛÚ ÔÓ ‡ÌÚÓÔÓÎÓ„Ëfl, ÅÄç, ëÓÙËfl 2

SUMMARY CONGENITAL CATARACTS FACIAL DYSMORPHISM NEUROPATHY SYNDROME IN GYPSIES – PURE DEFECT IN POLYMERASE II – MEDIATED TRANSCRIPTION Tournev I., R. Varon, D. Angelicheva, V. Guergueltcheva, R. Gooding, B. Youl, B. Ishpekova, R. King, M. Raycheva, Z. Kamenov, S. Cherninkova, A. Tomov, R. Petkov, V. Bojinova, M. Katzarova, A. Shmarov, I. Litvinenko, R. Tincheva, H. Tzekov, P. Borisova, B. Buzhov, J. Petrova, P.K.Thomas and L. Kalaydjieva During the extensive study on hereditary neurological disorders in Gypsies in Bulgaria, a previously unrecognised neurological disorder was encountered, mainly in Wallachian Gypsies. The disorder has been termed the congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome to emphasize its salient features. CCFDN is an autosomal recessive developmental disorder. It is characterized by a complex clinical phenotype with seemingly unrelated features involving multiple organs and systems. Seventhy-three individuals from 29 extended pedigrees were identified and examined clinically, electrophysiologically and genetically. MRI of the brain and spinal cord was performed in 21 patients. Neuropathological studies of sural nerves were performed in 5 patients. The disorder is recognised in infancy by the presence of congenital cataracts and microcorneae. Motor development is delayed. A predominantly motor neuropathy develops during childhood and leads to severe disability by the third decade. Motor nerve conduction velocities are reduced into the demyelinating range. Neuropathological studies show primary hypomyelination with superimposed demyelination and axonal degeneration. Brain and spinal cord MRI demonstrate abnormalities in most patients investigated. Associated neurological features include moderate non-progressive cognitive deficit and pyramidal signs, mild chorea and mild ataxia in some. Accompanying non-neurological features include short stature, characteristic facial dysmorphism and hypogonadotropic hypogonadism. CCFDN is caused by a single-nucleotide substitution in an antisense Alu element in intron 6 of C-terminal-domain phosphatase (CTDP1) of RNA polymerase II gene. CCFDN thus joins the group of transcription syndromes and is the first purely transcriptional disorder identified with affected polymerase II-mediated expression. Key words: congenital cataracts facial dysmorphism neuropathy syndrome; peripheral neuropathy; congenital cataract; Marinesco-Sjogren syndrome.

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˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

„Ó̇‰ËÚ (21). éÒÌÓ‚ÌËÚ ÍÎËÌ˘ÌË ÔËÁ̇ˆË ̇ ·ÓÎÂÒÚÚ‡ Ò‡ ‰‚ÛÒÚ‡Ì̇ ‚Ó‰Â̇ ͇ڇ‡ÍÚ‡ Ë ÏËÍÓÍÓ̇, Îˈ‚ ‰ËÁÏÓÙËÁ˙Ï Ë ÌËÒ˙Í ˙ÒÚ, ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl, ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl Ë ıËÔÓ„Ó̇‰ËÁ˙Ï. ᇷÓÎfl‚‡ÌÂÚÓ Â ÓÔËÒ‡ÌÓ Á‡ Ô˙‚Ë Ô˙Ú ÔË ·˙΄‡ÒÍËÚ ÓÏË (21). äéçíàÉÖçí à åÖíéÑà çÄ àáëãÖÑÇÄçÖ à‰ÂÌÚËÙˈË‡ÌË Ò‡ 73 Ô‡ˆËÂÌÚË Ò ääãÑç ÒË̉ÓÏ ÓÚ 29 Ó‰ÓÒÎÓ‚Ëfl, ͇ÚÓ Ôӂ˜ ÓÚ ÔÓÎÓ‚Ë̇ڇ Ò‡ ÓÚ „ÛÔ‡Ú‡ ̇ ‚·¯ÍËÚ ˆË„‡ÌË (ÍÓԇ̇Ë). äÓԇ̇ËÚ ҇ Ò ‚·¯ÍÓ ÔÂÙÂË‡ÌÓ Ò‡ÏÓÒ˙Á̇ÌËÂ. ç „Ó‚ÓflÚ ˆË„‡ÌÒÍË ÂÁËÍ, ‡ ÒÚ‡ËÌÂÌ ‚·¯ÍË ‰Ë‡ÎÂÍÚ. ë‚ÓflÚ‡ ÂÚÌ˘ÂÒ͇ ÔË̇‰ÎÂÊÌÓÒÚ Ú ÓÔ‰ÂÎflÚ Í‡ÚÓ ‚·ÒË. ç‡ ‚Òfl͇ Ù‡ÏËÎËfl Ò˙Ò Á‡Ò„̇ÚË ËÌ‰Ë‚Ë‰Ë Â Ôӂ‰ÂÌÓ ÔÓ‰Ó·ÌÓ „Â̇Îӄ˘ÌÓ ÔÓÛ˜‚‡ÌÂ. ç‡ ‚Ò˘ÍË ·ÓÎÌË Ò‡ Ôӂ‰ÂÌË Ì‚ÓÎӄ˘ÌË, ÓÙÚ‡ÎÏÓÎӄ˘ÌË Ë ÓÚÓÔ‰˘ÌË ËÁÒΉ‚‡ÌËfl. èÓÌ ‰ËÌ ·ÓÎÂÌ ÓÚ ‚ÒflÍÓ ÒÂÏÂÈÒÚ‚Ó Â ıÓÒÔËÚ‡ÎËÁË‡Ì Á‡ Ô˙ÎÌË ËÁÒΉ‚‡ÌËfl. ÖÎÂÍÚÓÌ‚Ó„‡ÙÒÍÓ ËÁÒΉ‚‡Ì (ÖçÉ)  Ôӂ‰ÂÌÓ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË ÔÓ Òڇ̉‡Ú̇ ÏÂÚÓ‰Ë͇ ‚ åìëÓÙËfl Ò ‡Ô‡‡Ú Phasis II (Esaotebiomedica, Florence, Italy) ËÎË ‚ ‰ÓÏÓ‚ÂÚ ̇ ·ÓÎÌËÚÂ Ò ÔÓÚ‡ÚË‚ÂÌ Medelec Saphire (Woking, UK) ÂÎÂÍÚÓÏËÓ„‡Ù. å.Ò.ëÖè Ò‡ ËÁÒΉ‚‡ÌË ÔË 20 Ô‡ˆËÂÌÚË ˜ÂÁ ÍÎËÍ ÒÚËÏÛÎË Ì‡ 80 dB Ò Ô‡„ Ò ÛÒË΂‡ÚÂÎ̇ ˜ÂÒÚÓÚ̇ ÎÂÌÚ‡ 200-2000 Hz. èË 25 Ô‡ˆËÂÌÚË Â Ì‡Ô‡‚ÂÌÓ ÓÚÓÌ‚ÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ. èË 15 Ô‡ˆËÂÌÚË Ò‡ Ôӂ‰ÂÌË Òڇ̉‡ÚÌË ÖÖÉ Á‡ÔËÒË Ë ÏÓÁ˙˜Ì‡ ͇ÚÓ„‡ÙËfl Ò Nicolet Pathfinder MEGA. èË 18 Ô‡ˆËÂÌÚË Â Ôӂ‰Â̇ χ„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl (åêí) ̇ „·‚ÌËfl Ë „˙·Ì‡˜ÌËfl ÏÓ˝˙Í. èË 20 Ô‡ˆËÂÌÚË (11 ‚˙Á‡ÒÚÌË Ë 9 ‰Âˆ‡)  Ôӂ‰Â̇ ÓˆÂÌ͇ ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË. èË·„‡ÌË Ò‡ èÓ„ÂÒË‚ÌËÚ χÚËˆË Ì‡ êÂÈ‚˙Ì Ë Ò͇·ڇ ̇ ÇÂÍÒÎÂ Á‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ ÔË ‚˙Á‡ÒÚÌË (WAIS, ·˙΄‡Ò͇ ‡‰‡ÔÚ‡ˆËfl, 1976). èÂÚ ÓÚ ‰Âˆ‡Ú‡ Ò‡ ÚÂÒÚÛ‚‡ÌË ÔÓ Ò͇ÎËÚ ̇ ÅËÌÂ-íÂÏ‡Ì Ë å‡ÌÓ‚‡-íÓÏÓ‚‡ (·˙΄‡Ò͇ Òڇ̉‡ÚËÁ‡ˆËfl Á‡ ‰Âˆ‡ ‰Ó 3-„Ӊ˯̇ ‚˙Á‡ÒÚ, 1980). èË 20 Ô‡ˆËÂÌÚË Ò‡ ËÁÒΉ‚‡ÌË ẨÓÍËÌÌËÚ ÙÛÌ͈ËË. éÔ‰ÂÎflÌË Ò‡ ·‡Á‡ÎÌËÚÂ Ë ÒΉ ÒÚËÏÛ·ˆËfl ÌË‚‡ ̇ ‡ÒÚÂÊÌËfl ıÓÏÓÌ, í3, í4, ííï, Ô‡‡ÚıÓÏÓ̇, îëï, ãëï, ÂÒÚ‡‰ËÓ· ÔË ÊÂÌË Ë ÚÂÒÚÓÒÚÂÓ̇ ÔË Ï˙ÊÂ. çË‚‡Ú‡ ̇ ıÓÏÓÌËÚ ҇ ÓÔ‰ÂÎflÌË ˜ÂÁ RIA-mat ËÎË IRMA-mat (Byk-Sangtec Diagnostica, Germany) ̇ ‡‚ÚÓχÚ˘̇ „‡Ï‡ ͇ÏÂ‡ (1470 Wizard TM, Wallac). äÓÒÚÌÓ-ÏËÌÂ‡Î̇ڇ ÔÎ˙ÚÌÓÒÚ Â ÓÔ‰ÂÎÂ̇ ÔË 10 Ô‡ˆËÂÌÚË ‚ Ô‰Ï˯Ìˈ‡Ú‡ ˜ÂÁ Osteometer DTX100 (Osteometer A/S, Denmark), ͇ÍÚÓ Ë ÌË‚‡Ú‡ ̇ ÒÂÛÏÌËfl ͇ΈËÈ Ë ÙÓÒÙÓ, Ë ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ ‡Î͇Î̇ڇ ÙÓÒÙ‡Ú‡Á‡. çË‚‡Ú‡ ̇ ·ÍÚ‡Ú‡ ‚ ÒÂÛχ Ò‡ ËÁÒΉ‚‡ÌË ·ÂÁ ÔÓÚÂËÌËÁ‡ˆËfl ˜ÂÁ ÏÓ‰ËÙË͇ˆËfl ̇ ÏÂÚÓ‰‡ ̇ Noll (Boehringer Mannheim, Germany). èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Ôӂ‰ÂÌÓ Ë ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡ÌÂ. ÄçÄåçÖëíàóçà ÑÄççà êÓ‰ËÚÂÎËÚ ̇ ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò˙Ó·˘‡‚‡Ú Á‡ ÌÓχÎÌÓ ÔÓÚÂÍ· ·ÂÏÂÌÌÓÒÚ Ë ‡Ê‰‡Ì ̇ Á‡Ò„̇ÚËÚ Ë̉˂ˉË. ÇÓ‰ÂÌËÚ ͇ڇ‡ÍÚË Ò‡ Ô˙‚Ëfl ÛÒÚ‡ÌÓ‚fl‚‡Ì ÔËÁÌ‡Í Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Á‡·‡‚ÂÌÓ ‰‚Ë„‡ÚÂÎÌÓÚÓ ‡Á‚ËÚËÂ. èӂ˜ÂÚÓ ÓÚ Úflı Ò‡ ÔÓıÓ‰ËÎË ÏÂÊ‰Û ‰‚Â Ë ÚË „Ӊ˯̇ ‚˙Á‡ÒÚ, ‡ 15% - ÏÂÊ‰Û ÚË Ë ÔÂÚ „Ӊ˯̇ ‚˙Á‡ÒÚ. á‡Òfl„‡ÌÂÚÓ Ì‡ „ÓÌËÚ Í‡ÈÌËˆË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÏÂÊ‰Û 3 Ë 7 „. èÓÒÚÂÔÂÌÌÓ Ò ‡Á‚Ë‚‡Ú ‰ËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Ë ‰ÂÙÓÏËÚÂÚË Ì‡ ıӉ˷ڇ. èË 19 Ô‡ˆËÂÌÚË Ò Ò˙Ó·˘‡‚‡ Á‡ Á‡·‡‚ÂÌÓ ÂÁËÍÓ‚Ó ‡Á‚ËÚË ͇ÚÓ ÚÂÁË Ô‡ˆËÂÌÚË Ò‡ ÔÓ„Ó‚ÓËÎË ÒΉ 3 „Ӊ˯̇ ‚˙Á‡ÒÚ. ÇÒ˘ÍË Á‡Ò„-

143

̇ÚË ËÌ‰Ë‚Ë‰Ë Ò‡ ÔÓÒ¢‡‚‡ÎË ÒÔˆˇÎÌË Û˜ËÎˢ‡ Á‡ Ò··ÓÁfl˘Ë ‰Âˆ‡ Ë Ò‡ ·ËÎË Ò ÎÓ¯‡ ÛÒÔ‚‡ÂÏÓÒÚ ‚ Û˜ËÎˢÂ. èӂ˜ÂÚÓ ÓÚ Úflı Ò‡ ̇ÔÛÒ̇ÎË Û˜ËÎˢ Ӣ ÔÂÁ Ô˙‚‡Ú‡ „Ó‰Ë̇ ÓÚ Ì‡˜‡ÎÌÓÚÓ Ó·Û˜ÂÌËÂ Ë Ò‡ÏÓ ‰‚‡Ï‡ Ô‡ˆËÂÌÚË Ò‡ Á‡‚˙¯ËÎË ÓÒÌÓ‚ÌÓ Ó·‡ÁÓ‚‡ÌËÂ. ÇÒ˘ÍË ÊÂÌË Ò˙Ó·˘‡‚‡Ú Á‡ ÌÂ‰ӂ̇ ÏÂÌÒÚÛ‡ˆËfl, ‡ ÔÓ-‚˙Á‡ÒÚÌËÚ ÓÚ Úflı - Á‡ ‚ÚÓ˘̇ ‡ÏÂÌÓ‡, ̇ÒÚ˙Ô‚‡˘‡ ÏÂÊ‰Û 25 Ë 35 „. óÂÚËË ÓÚ Á‡Ò„̇ÚËÚ ÊÂÌË Ò‡ Ó‰ËÎË ÌÓχÎÌË ‰Âˆ‡, ÌÓ ÌËÚÓ Â‰ËÌ ÓÚ Ï˙ÊÂÚ Ì  Ò˙Á‰‡Î ÔÓÍÓÎÂÌËÂ. îàáàäÄãçé àáëãÖÑÇÄçÖ ÇÒ˘ÍË Ô‡ˆËÂÌÚË Ò‡ Ò ÌËÒ˙Í ˙ÒÚ, ‡ Ôӂ˜ÂÚÓ ÓÚ Úflı Ë Ò ÌËÒÍÓ Ú„ÎÓ. ê˙ÒÚ˙Ú Ì‡ ‚˙Á‡ÒÚÌËÚ Ï˙Ê (N=22)  147.3 ± 5.11 ÒÏ, ‡ ̇ ‚˙Á‡ÒÚÌËÚ ÊÂÌË (N=21) 141.4 ± 8.3 ÒÏ Í‡ÚÓ Ò˙ÓÚ‚ÂÚÒÚ‚‡˘ËÚ ÌÓÏË Á‡ ·˙΄‡Ò͇ڇ ÔÓÔÛ·ˆËfl Ò‡ 173.3 ± 6.8 ÒÏ Á‡ Ï˙ÊÂÚÂ Ë 160.3 ± 6.4 Á‡ ÊÂÌËÚ (1). íÂÎÂÒÌÓÚÓ Ú„ÎÓ Â 46.5 ± 9.7 Í„ Á‡ Ï˙ÊÂÚÂ Ë 44.3 ± 7.6 Í„ Á‡ ÊÂÌËÚ (ÔË ÌÓÏË Ò˙ÓÚ‚ÂÚÌÓ 73.9 ± 10.4 Í„ Ë 63.0 ± 10.7 Í„). é·ËÍÓÎ͇ڇ ̇ „·‚‡Ú‡  ̇χÎÂ̇, 50.8 ± 2.2 ÒÏ Á‡ Ï˙ÊÂÚÂ Ë 50.4 ± 3.2 ÒÏ Á‡ ÊÂÌËÚ (ÔË ÌÓÏË Ò˙ÓÚ‚ÂÚÌÓ 57.2 ± 2.1 ÒÏ Ë 54.8 ± 1.9 ÒÏ). éÚÌÓ¯ÂÌËÂÚÓ ÙÓÌÚÓ-Ó͈ËÔËÚ‡Î̇ Ó·ËÍÓÎ͇/˙ÒÚ Â ÌÓχÎÌÓ, 0.34 Á‡ Ï˙ÊÂÚÂ Ë 0.35 Á‡ ÊÂÌËÚ (ÔË ÌÓÏË Ò˙ÓÚ‚ÂÚÌÓ 0.33 Á‡ Ï˙ÊÂÚÂ Ë 0.34 Á‡ ÊÂÌËÚÂ). íÂÎÂÒÌËÚ ÔÓÔÓˆËË ÔË Ï˙ÊÂÚ ҇ ÓÚ ÊÂÌÒÍË ÚËÔ. ãˈ‚ ‰ËÁÏÓÙËÁ˙Ï Â ÛÒÚ‡ÌÓ‚ÂÌ ÔË 53 ÓÚ 73 Ô‡ˆËÂÌÚË. ÑËÁÏÓÙ˘ÌËÚ ÎËˆÂ‚Ë ˜ÂÚË Ò‡ ÒıÓ‰ÌË ÔË Ô‡ˆËÂÌÚËÚ ÓÚ ‡Á΢ÌË ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË Ë ‡Ì„‡ÊË‡Ú „·‚ÌÓ ‰ÓÎÌËfl Îˈ‚ Ò„ÏÂÌÚ ÔÓ‰ ÙÓχڇ ̇ Ôӄ̇ÚËfl ËÎË ÏËÍӄ̇ÚËfl, Ô‰̇ ‰ÂÌÚˈËfl Ë Á‡‰Â·ÂÎfl‚‡Ì ̇ ÔÂËÓ‡ÎÌËÚ Ú˙͇ÌË. ãˈ‚ËflÚ ‰ËÁÏÓÙËÁ˙Ï Â Ò··Ó ÔÓfl‚ÂÌ ËÎË ÎËÔÒ‚‡ ‚ ‡Ì̇ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ Ë Ò Á‡ÒË΂‡ Ò Ì‡Ô‰‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡. àÁÒΉ‚‡Ì  ÓÚÓÔ‰˘ÌËflÚ ÒÚ‡ÚÛÒ Ì‡ ‚Ò˘ÍË Ô‡ˆËÂÌÚË Í‡ÚÓ ÔË 52 ÓÚ Úflı Ò ÓÚÍË‚‡Ú ÓÚÓÔ‰˘ÌË Ì‡Û¯ÂÌËfl, Ò‚˙Á‡ÌË Ò ıӉ˷ڇ, ‰Î‡ÌËÚÂ Ë „˙·Ì‡˜ÌËfl ÒÚ˙η. éÚÓÔ‰˘ÌËÚ ‰ÂÙÓχˆËË Ò ÔÓfl‚fl‚‡Ú ÒΉ 4 – 5 „Ӊ˯̇ ‚˙Á‡ÒÚ. ïӉ˷ڇ Ò‡ ‰ÂÙÓÏË‡ÌË ‚ ÔÓÁˈËË: pes excavatus, pes equinovarus Ë pes equinovarus excavatus. èË 33 Ô‡ˆËÂÌÚË Ò ̇·Î˛‰‡‚‡ main en griffe, ËÁ‡Áfl‚‡˘‡ Ò ‚ ıËÔÂÂÍÒÚÂÌÁËfl ̇ ÓÒÌÓ‚ÌËÚ ه·̄Ë, ‡ ‰ËÒÚ‡ÎÌËÚ ‰‚ ҇ ÙÎÂÍÚË‡ÌË. ÑÂÙÓχˆËË Ì‡ „˙·Ì‡˜ÌËfl ÒÚ˙η Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔË 21 Ô‡ˆËÂÌÚË. äËÙÓÒÍÓÎËÓÁ‡, ËÁÍË‚fl‚‡Ì ‚˙‚ ÙÓÌÚ‡Î̇ Ë Ò‡„ËÚ‡Î̇ ‡‚ÌË̇ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 6 ÊÂÌË Ë 9 Ï˙ÊÂ. äËÙÓÁ‡, ËÁÍË‚fl‚‡Ì ‚ Ò‡„ËÚ‡Î̇ڇ ‡‚ÌË̇, Ò ̇·Î˛‰‡‚‡ ÔË 4 ÊÂÌË Ë 2 Ï˙ÊÂ. êÂÒÔË‡ÚÓÌËflÚ ‚ËÚ‡ÎÂÌ Í‡Ô‡ˆËÚÂÚ Â ‰ۈË‡Ì ‚ÚÓ˘ÌÓ ‚ ÒΉÒÚ‚Ë ̇ ÍËÙÓÒÍÓÎËÓÁ‡Ú‡ ÔË ÚÂÁË Ô‡ˆËÂÌÚË (42 - 75% ÓÚ ÌÓχڇ). ÇÚÓ˘ÌËÚ ÔÓÎÓ‚Ë ·ÂÎÂÁË, ‚Íβ˜‚‡˘Ë ‡Á‚ËÚË ̇ ÏΘÌËÚ ÊÎÂÁË Ë ÚÂÒÚËÒËÚÂ Ë ÔÛ·ËÒÌÓÚÓ ÓÍÓÒÏfl‚‡ÌÂ, Ò‡ ÌÓχÎÌË ÔË ‚Ò˘ÍË Á‡Ò„̇ÚË Ë̉˂ˉË. çÖÇêéãéÉàóçé àáëãÖÑÇÄçÖ ç‡È-ı‡‡ÍÚÂ̇ڇ ÍÎËÌ˘̇ ÔÓfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ‰ËÒÚ‡Î̇ڇ ÒËÏÂÚ˘̇ ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl. åÓÚÓÌÓÚÓ Á‡Òfl„‡Ì  ÔÓ-ÚÂÊÍÓ, ÓÚÍÓÎÍÓÚÓ ÒÂÌÁÓÌÓÚÓ Ë Â Ì‡Îˈ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ‚Íβ˜ËÚÂÎÌÓ ÔË Ì‡ÈÏ·‰Ëfl, ÍÓÈÚÓ Â Ì‡ 4 ÏÂÒˆ‡. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔÓ‰ÚËÒ͇Ì ËÎË ÎËÔÒ‡ ̇ ÒÛıÓÊËÎÌËÚÂ Ë Ì‡‰ÍÓÒÚÌËÚ ÂÙÎÂÍÒË. è˙Î̇ ‡ÂÙÎÂÍÒËfl ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Ò ̇·Î˛‰‡‚‡ ÔË 63 Ô‡ˆËÂÌÚË, ‡ ÔË ÓÒڇ̇ÎËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‡ıËÎÓ‚‡ ‡ÂÙÎÂÍÒËfl Ò˙Ò Á‡Ô‡ÁÂÌË ÍÓÎÂÌÌË ÂÙÎÂÍÒË. Ç „ÓÌËÚ Í‡ÈÌËˆË ÒÛıÓÊËÎÌËÚ ÂÙÎÂÍÒË Ò‡ ÌÓχÎÌË ÔË 17 Ô‡ˆËÂÌÚË, ‡ ÔË ÓÒڇ̇ÎËÚ – ÎËÔÒ‚‡˘Ë


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ËÎË ÒÌËÊÂÌË. èÓ‚Âʉ‡ÌÂÚÓ Ì‡ ÒÂÚË‚ÌÓÚÓ ËÁÒΉ‚‡Ì Ì ·Â¯Â ‚˙ÁÏÓÊÌÓ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ÔÓ‡‰Ë Úflı̇ڇ ‚˙Á‡ÒÚ ËÎË ÔÓ‡‰Ë Ó„‡Ì˘ÂÌËÚ ËÏ ÍÓ„ÌËÚË‚ÌË ÒÔÓÒÓ·ÌÓÒÚË. èË ÚÂÁË, Á‡ ÍÓËÚÓ Ò‡ ÔÓÎÛ˜ÂÌË ‰‡ÌÌË, Ò ÓÚÍË‚‡Ú ‰ËÒÚ‡ÎÌË Ì‡Û¯ÂÌËfl, ͇ÍÚÓ Ì‡ ÛÒÂÚ‡ Á‡ ‰ÓÔË Ë ·ÓÎ͇, ڇ͇ Ë Ì‡ ÔÓÔËÓˆÂÔÚË‚ÌËfl ÛÒÂÚ. èË ÌËÚÓ Â‰ËÌ Ì  ÛÒÚ‡ÌÓ‚ÂÌÓ Á‡‰Â·ÂÎfl‚‡Ì ̇ ÔÂËÙÂÌËÚ ÌÂ‚Ë. ÑÛ„ËÚ ÓÚÍËÚË Ì‚ÓÎӄ˘ÌË ÔËÁ̇ˆË Ò‡ ÔÓ-ÌÂÔÓÒÚÓflÌÌË. á‡Òfl„‡Ì ̇ ÍÓÚËÍÓÒÔË̇ÎÌËÚ Ô˙Úˢ‡, ‰ÂÏÓÌÒÚË‡ÌÓ ˜ÂÁ Ô‡ÚÓÎӄ˘ÌËÚ ÂÙÎÂÍÒË Ì‡ Ň·ËÌÒÍË Â Ì‡·Î˛‰‡‚‡ÌÓ ÔË 28 ÓÚ 73 Ô‡ˆËÂÌÚË. èË ÌËÚÓ Â‰ËÌ ÓÚ Úflı Ì  ÛÒÚ‡ÌÓ‚Â̇ ÒÔ‡ÒÚ˘ÌÓÒÚ ‚ Í‡ÈÌˈËÚÂ. ïÓÂËÙÓÏÂÌË ‰‚ËÊÂÌËfl Ò‡ ̇·Î˛‰‡‚‡ÌË ÔË 30 Ô‡ˆËÂÌÚË. í Ò ËÁ‡Áfl‚‡Ú ‚ ÎËˆÂ‚Ë „ËχÒË Ë Ì‚ÓÎÂ‚Ë ÍÎÓÌ˘ÌË ‰‚ËÊÂÌËfl ̇ Í‡ÈÌˈËÚÂ Ë ÚÛÎӂˢÂÚÓ, ̇È-ËÁ‡ÁÂÌË ‚ ‰ÓÎÌËÚ Í‡ÈÌˈË. èÓÒÚÛ‡ÎÂÌ ÚÂÏÓ ‚ „ÓÌËÚ Í‡ÈÌËˆË Â Ì‡·Î˛‰‡‚‡Ì ÔË 11 Ô‡ˆËÂÌÚË; 16 Ô‡ˆËÂÌÚË Ò‡ ÎÂÍÓ ‡Ú‡ÍÚ˘ÌË. éîíÄãåéãéÉàóçé àáëãÖÑÇÄçÖ Ñ‚ÛÒÚ‡Ì̇ ÁÓÌÛ·̇ ͇ڇ‡ÍÚ‡ Ë ÏËÍÓÍÓ̇ Ò ÓÚÍË‚‡Ú ÔË ‚Ò˘ÍËÚ ԇˆËÂÌÚË. ë‰ÌËflÚ ıÓËÁÓÌÚ‡ÎÂÌ ‰Ë‡ÏÂÚ˙ ̇ ÍÓ̇ڇ Á‡ Îfl‚ÓÚÓ ÓÍÓ Â ‡‚̇ ̇ 7.3 ± 0.96 ÏÏ Ë Á‡ ‰flÒÌÓÚÓ ÓÍÓ - 7.6 ± 1.6 ÏÏ. èË 55 Ô‡ˆËÂÌÚË Â Ôӂ‰ÂÌÓ ıËÛ„˘ÌÓ ÓÚÒÚ‡Ìfl‚‡Ì ̇ ͇ڇ‡ÍÚËÚ ÔÓ ‚ÂÏ ̇ Ô˙‚‡Ú‡ ÔÓÎÓ‚Ë̇ ÓÚ ÊË‚ÓÚ‡. ëÚ‡·ËÁ˙Ï Â Ì‡Îˈ ÔË ‚Ò˘ÍË Á‡Ò„̇ÚË Ë̉˂ˉË. Ñ‚ÛÒÚ‡Ì̇ ÔÚÓÁ‡  ̇·Î˛‰‡‚‡Ì‡ ÔË 7 ‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË. èẨÛ·ÂÌ ÌËÒÚ‡„˙Ï Â Ì‡·Î˛‰‡‚‡Ì ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ËÁÍβ˜ÂÌË ̇ 1 Ï˙Ê. é҈˷ˆËËÚ ҇ Ò ‡‚̇ ‡ÏÔÎËÚÛ‰‡ Ë ÒÍÓÓÒÚ ‚ ‰‚ÂÚ ÔÓÒÓÍË Ë Ò‡ ̇Îˈ ÔË ‚Ò˘ÍË ÔӄΉÌË ‰‚ËÊÂÌËfl. åËÍÓÙÚ‡ÎÏ Â ÛÒÚ‡ÌÓ‚ÂÌ ÔË 86% ÓÚ Ô‡ˆËÂÌÚËÚÂ. éíéçÖÇêéãéÉàóçé àáëãÖÑÇÄçÖ à àáëãÖÑÇÄçÖ çÄ å.ë.ëÖè ëڇ̉‡Ú̇ ‡Û‰ËÓÏÂÚËfl  Ôӂ‰Â̇ ÔË 25 Ô‡ˆËÂÌÚË. 22 ÓÚ Úflı ËÏ‡Ú ÌÓχÎÌË ÒÎÛıÓ‚Ë ÙÛÌ͈ËË, ‡ ÚËχ ÔÓ͇Á‚‡Ú ‰‚ÛÒÚ‡ÌÌÓ Á‚ÛÍÓÔËÂÏÌÓ ÒÎÛıÓ‚Ó Ì‡Û¯ÂÌËÂ Ò ‡Á΢̇ ÒÚÂÔÂÌ Ì‡ ‰ÂÙˈËÚ. à̉ۈË‡Ì ÌËÒÚ‡„˙Ï ˜ÂÁ ·ËÚÂχÎÂÌ Í‡ÎÓ˘ÂÌ ÚÂÒÚ Ë ÂÎÂÍÚÓÌËÒÚ‡„ÏÓ„‡ÙÒÍË Á‡ÔËÒË ÔÓ͇Á‚‡Ú ÚẨÂ̈Ëfl Í˙Ï ıËÔÂÂÙÎÂÍÒËfl Ò˙Ò Á‡·‡‚ÂÌË ÓÚ„Ó‚ÓË ·ÂÁ „Û·Ë ÔÓÏÂÌË Ì‡ Ô‡‡ÏÂÚËÚ ̇ Ë̉ۈË‡ÌËfl ÌËÒÚ‡„˙Ï. å.Ò.ëÖè Ò‡ Á‡ÔËÒ‡ÌË ÔË 20 Ô‡ˆËÂÌÚË. ãÂÍË Ì‡Û¯ÂÌËfl Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ò‡ÏÓ ÔË ÚËχڇ Ô‡ˆËÂÌÚË Ò Ì‡Û¯ÂÌ ÒÎÛı. éñÖçäÄ çÄ äéÉçàíàÇçàíÖ ëèéëéÅçéëíà Ç˙Á ÓÒÌÓ‚‡ ̇ ÂÁÛÎÚ‡ÚËÚ ÓÚ Ôӂ‰ÂÌËÚ ÔÒËıÓÏÂÚ˘ÌË ÚÂÒÚÓ‚Â, 18 ÓÚ 20 ËÁÒΉ‚‡ÌË ËÌ‰Ë‚Ë‰Ë Ò‡ Í·ÒËÙˈË‡ÌË Ò ÎÂÍÓ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë ‰‚‡Ï‡ Ò‡ ‚ „‡Ì˘ÌËfl ‰Ë‡Ô‡ÁÓÌ. ÇÒ˘ÍË Ô‡ˆËÂÌÚË ËÏ‡Ú ÏÌÓ„Ó ÌËÒÍË ÔÓ͇Á‡ÚÂÎË Ì‡ ÄËÚÏÂÚ˘ÌËfl ÒÛ·ÚÂÒÚ Ë Digit Span, ÌÓ ËÁÔ˙ÎÌfl‚‡Ú ÓÚÌÓÒËÚÂÎÌÓ ‰Ó· ‚Â·‡ÎÌËÚ Á‡‰‡˜Ë. ÖÁËÍÓ‚ËÚ ÒÔÓÒÓ·ÌÓÒÚË Ë ÍÓ̈ÂÔÚÛ‡ÎËÁ‡ˆËflÚ‡ Ò‡ ‡Á‚ËÚË Ì‡ ÍÓÌÍÂÚÌÓ Ô‡„χÚ˘ÌÓ ‡‚ÌˢÂ. ë‡‚Ìfl‚‡ÌÂÚÓ ÏÂÊ‰Û ÂÁÛÎÚ‡ÚËÚÂ, ÔÓÎÛ˜ÂÌË ˜ÂÁ ËÁÔÓÎÁ‚‡Ì ̇ ‡Á΢ÌË Ò͇ÎË Ë ÏÂÊ‰Û ‚Â·‡ÎÌËÚÂ Ë Ì‚Â·‡ÎÌËÚ ÒÛ·ÚÂÒÚÓ‚Â, Ì ÓÚÍË‚‡ Á̇˜ËÏË ‡Á΢Ëfl. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡Á΢Ëfl ‚ ËÌÚÂÎÂÍÚÛ‡ÎÌÓÚÓ ÌË‚Ó Ë ÔË Ô‡ˆËÂÌÚË Ì‡ ‡Á΢̇ ‚˙Á‡ÒÚ ÔË ¯ËÓÍ ‚˙Á‡ÒÚÓ‚ ‰Ë‡Ô‡ÁÓÌ (3 - 44 „.). ãËÔÒ‡Ú‡ ̇ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ÒÚÂÔÂÌÚ‡ ̇ ËÌÚÂÎÂÍÚÛ‡ÎÌËÚ ̇Û¯ÂÌËfl Ë ‚˙Á‡ÒÚÚ‡ ÏÓÊ ‰‡ Ò ËÌÚÂÔÂÚË‡ ͇ÚÓ ‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ ÌÂÔÓ„ÂÒË‡˘Ëfl ı‡‡ÍÚÂ ̇ ÍÓ„ÌËÚË‚ÌËÚ ‰ÂÙˈËÚË.

144

àáëãÖÑÇÄçàü çÄ çÖêÇçÄíÄ èêéÇéÑàåéëí (ÖçÉ) êÂÁÛÎÚ‡ÚËÚ ÓÚ Á‡ÔËÒËÚ ̇ ÏÓÚÓ̇ڇ Ë ÒÂÚ˂̇ ÌÂ‚̇ ÔÓ‚Ó‰ËÏÓÒÚ Ò‡ Ô‰ÒÚ‡‚ÂÌË Ì‡ Ú‡·Îˈ‡ 1. ëÍÓÓÒÚÚ‡ ̇ ÔÓ‚Âʉ‡Ì (ëè) ÔÓ ÏÓÚÓÌËÚ ÌÂ‚ÌË ‚·Í̇  ÒÌËÊÂ̇ ‚ ‰ÂÏËÂÎËÌËÁË‡˘Ëfl ‰Ë‡Ô‡ÁÓÌ, ‡ ‰ËÒÚ‡ÎÌÌÓÚÓ Î‡ÚÂÌÚÌÓ ‚ÂÏ  ۉ˙ÎÊÂÌÓ, ͇ÍÚÓ Á‡ ÔÓÍÒË퇷Îˈ‡ 1. ÖÎÂÍÚÓÌ‚Ó„‡ÙËfl ÔË Ô‡ˆËÂÌÚË Ò ääãÑç ÒË̉ÓÏ. èÓ‚Âʉ‡Ì ÔÓ ÏÓÚÓÌË ëè (Ï/ÒÂÍ) ÑãÇ (ÏÒÂÍ) ‚·Í̇ Ç˙Á‡ÒÚ 4 ÏÂÒˆ‡ ‰Ó 7 „Ó‰ËÌË n. ulnaris 30.7 ± 1.6 3.8 ± 1.5 n. peroneus 30.7 ± 3.1 3.7 ± 1.6 Ç˙Á‡ÒÚ 10 – 44 „Ó‰ËÌË n. ulnaris 25.9 ± 3.9 4.7 ± 1.7 n. peroneus 21.6 ± 2.8 7.8 ± 2.0 n. facialis 8.1 ± 2.0 n. axillaris 9.2 ± 2.3 n. musculocutaneous 12.5 ± 2.3 èÓ‚Âʉ‡Ì ÔÓ ÒÂÚË‚ÌË ÄÏÔÎËÚÛ‰‡ (m V) ëè (Ï/ÒÂÍ) ‚·Í̇ n. medianus ÓÚÓ‰ÓÏÌÓ 13.0 ± 2.4 30.3 ± 3.1 ‡ÌÚˉÓÏÌÓ 29.2 ± 3.1 33.7 ± 3.2 n. suralis 10.0 ± 2.2 28.0 ± 3.0 ëè, ÒÍÓÓÒÚ Ì‡ ÔÓ‚Âʉ‡ÌÂ; ÑãÇ, ‰ËÒÚ‡ÎÌÓ Î‡ÚÂÌÚÌÓ ‚ÂÏ χÎÌËÚÂ, ڇ͇ Ë Á‡ ‰ËÒÚ‡ÎÌËÚ ۘ‡ÒÚ˙ˆË ̇ ÔÂËÙÂÌËÚ ÌÂ‚Ë. ᇷ‡‚flÌÂÚÓ Ì‡ ÏÓÚÓ̇ڇ ëè  ÔÓ-ËÁ‡ÁÂÌÓ ÔË ÔÓ-‚˙Á‡ÒÚÌËÚ ԇˆËÂÌÚË (̇‰ 10 „.), ÓÚÍÓÎÍÓÚÓ ÔË ÔÓ-Ï·‰ËÚ ËÌ‰Ë‚Ë‰Ë (ÔÓ‰ 10 „.). ëÌËÊÂÌËÂÚÓ Ì‡ ÏÓÚÓÌËÚ ëè Ò ‚˙Á‡ÒÚÚ‡  Á̇˜ËÏÓ (p<0.05) ‚ ÛÎ̇ÌËfl ÌÂ‚ Ò˙Ò ÒÍÓÓÒÚ ÓÚ 0.2 Ï/ÒÂÍ/„Ó‰., ÌÓ Â ÌÂÁ̇˜ËÏÓ ‚ ÔÂÓ̇ÎÌËfl ÌÂ‚ (p>0.05) Ò˙Ò ÒÍÓÓÒÚ 0.1 Ï/ÒÂÍ/„Ó‰. ë‰ÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ ÒÂÚË‚ÌËÚ ‡ÍˆËÓÌÌË ÔÓÚÂ̈ˇÎË Ò‡ Ò ÌÓχÎ̇ ‡ÏÔÎËÚÛ‰‡ ‚ „ÓÌËÚÂ Ë ‚ ‰ÓÎÌËÚ Í‡ÈÌˈË, ÌÓ ÒÍÓÓÒÚÚ‡ ̇ ÔÓ‚Âʉ‡ÌÂ Ë ÔË Úflı  ÒÌËÊÂ̇ ‚ ‰ÂÏËÂÎËÌËÁË‡˘Ëfl ‰Ë‡Ô‡ÁÓÌ. ëÌËÊÂÌË ÏÓÚÓÌË ëè Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ̇ ‚˙Á‡ÒÚ ÓÍÓÎÓ 1.5 „. ͇ÚÓ ‰Ó Ú‡ÁË ‚˙Á‡ÒÚ ÒÚÓÈÌÓÒÚËÚ ҇ ‚ „‡Ìˈ‡Ú‡ ̇ ÌÓχڇ. ÖãÖäíêéÖçñÖîÄãéÉêÄîëäà, ÖãÖäíêéäÄêÑàéÉêÄîëäà à ÖïéäÄêÑàéÉêÄîëäà àáëãÖÑÇÄçàü Ä·ÌÓÏÌË ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙÒÍË Á‡ÔËÒË Ò‡ ÔÓÎÛ˜ÂÌË ÔË ‚Ò˘ÍËÚ 15 ËÁÒΉ‚‡ÌË Ô‡ˆËÂÌÚË. èË 13 ÓÚ Úflı Ò ÓÚÍË‚‡ ‰ËÙÛÁ̇ ·‡‚ÌÓ‚˙ÎÌÓ‚‡ ‡ÍÚË‚ÌÓÒÚ (ÚÂÚ‡ Ë ‰ÂÎÚ‡). Ñ‚‡Ï‡ Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú ÂÔËÁӉ˘̇ ·‡‚ÌÓ‚˙ÎÌÓ‚‡ ‡ÍÚË‚ÌÓÒÚ, ÔË Â‰ËÌËfl - Ò ÔÂӷ·‰‡‚‡Ì ‚ Îfl‚‡Ú‡, ‡ ÔË ‰Û„Ëfl - ‚ ‰flÒ̇ڇ ÏÓÁ˙˜Ì‡ ıÂÏËÒÙÂ‡. èË ‰‚‡Ï‡ Ô‡ˆËÂÌÚË Ò ̇ÏË‡ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ, Ò˙ÒÚÓfl˘‡ Ò ÓÚ Â‰ËÌ˘ÌË Á‡ÎÔÓ‚Â ÓÚ ·‡‚ÌË ‚˙ÎÌË. ÖäÉ Ë ÂıÓ͇‰ËÓ„‡ÙÒÍËÚ ËÁÒΉ‚‡ÌËfl, Ôӂ‰ÂÌË ÔË 17 Ô‡ˆËÂÌÚË, ÔÓ͇Á‚‡Ú ÌÓχÎ̇ ̇ıӉ͇ Ò ËÁÍβ˜ÂÌË ̇ 1 Ô‡ˆËÂÌÚ, ÔË ÍÓÈÚÓ Ò ÓÚÍË‚‡ ‰Ë·ڇÚ˂̇ ͇‰ËÓÏËÓÔ‡ÚËfl. ÖçÑéäêàççà à ÉàçÖäéãéÉàóçà àáëãÖÑÇÄçàü äÓÚËÁÓÎÓ‚ËflÚ ËÚ˙Ï, ËÁÒΉ‚‡Ì ÔË 20 Ô‡ˆËÂÌÚË, ÔÓ͇Á‚‡ ÌÓχÎÌË ÒÚÓÈÌÓÒÚË. çÓχÎÂÌ Â Ë ÓÚ„Ó‚Ó˙Ú Ì‡ ËÌÒÛÎËÌ-Ë̉ۈË‡Ì‡Ú‡ ıËÔÓ„ÎËÍÂÏËfl, ÓÒ˙˘ÂÒÚ‚Â̇ ÔË 7 Ô‡ˆËÂÌÚË. T3, T4 Ë TTï Ò‡ ËÁÒΉ‚‡ÌË ÔË 20 Ô‡ˆËÂÌÚË Ë ÔÓ͇Á‚‡Ú ÌÓχÎÌË ÒÚÓÈÌÓÒÚË. èË 7 Ô‡ˆËÂÌÚË


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

 Ôӂ‰Â̇ ÒÚËÏÛ·ˆËfl Ò íêï Ë ÓÚ„Ó‚Ó˙Ú Ì‡ ÒÚËÏÛ·ˆËflÚ‡  ÌÓχÎÂÌ. ëÓχÚÓÚÓÔÌËflÚ ıÓÏÓÌ (ëíï)  ËÁÒΉ‚‡Ì ÔË 20 Ô‡ˆËÂÌÚË ‚˙‚ ‚˙Á‡ÒÚÓ‚Ëfl ‰Ë‡Ô‡ÁÓÌ ÏÂÊ‰Û 18 Ë 36 „. ÇÒ˘ÍË Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú ·‡Á‡ÎÌË ÒÚÓÈÌÓÒÚË Ì‡ ëíï ‚ ÌËÒÍÓ-ÌÓχÎÌËfl ‰Ë‡Ô‡ÁÓÌ – 0.24 ± 0.09 ng/ml. èË 7 Ô‡ˆËÂÌÚË Â Ôӂ‰ÂÌÓ ËÁÒΉ‚‡Ì ̇ ëíï ÒΉ ËÌÒÛÎËÌ-Ë̉ۈË‡Ì‡ ıËÔÓ„ÎËÍÂÏËfl. 燷≇‚‡ Ò ËÁ‡ÁÂÌÓ Ôӂ˯‡‚‡Ì ̇ ÒÚÓÈÌÓÒÚËÚ ̇ ëíï ÒΉ ÒÚËÏÛ·ˆËfl, ÍÓÂÚÓ ÔÓ͇Á‚‡ „Û·ÚÓÂÌ ‰ÂÙˈËÚ Ì‡ ÒÓχÚÓÚÓÔÌËfl ıÓÏÓÌ. ëÚÓÈÌÓÒÚËÚ ̇ ·‡Á‡ÎÌËfl ÔÓ·ÍÚËÌ Ò‡ ‚ ÌÓχ Ë ÔË Ï˙ÊÂÚÂ Ë ÔË ÊÂÌËÚÂ. è‡‡ÚıÓÏÓÌ˙Ú (èíï), ËÁÒΉ‚‡Ì ÔË 12 Ô‡ˆËÂÌÚË, Ò˙˘Ó  ‚ ÌÓχ. ëÚÓÈÌÓÒÚËÚ ̇ ÚÂÒÚÓÒÚÂÓ̇, ËÁÒΉ‚‡ÌË ÔË 10 Ï˙Ê ҇ ÌËÒÍË: 6.29 ± 1.94 nmol/l (ÔË ÌÓχ 9 – 30 nmol/l). ãÛÚÂËÌËÁË‡˘ËflÚ ıÓÏÓÌ (ãï)  ̇ ‰ÓÎ̇ „‡Ìˈ‡ ̇ ÌÓχڇ: 2.05 ± 1.24 mIU/ml, ‡ ÙÓÎËÍÛÎÓÒÚËÏÛÎË‡˘ËflÚ ıÓÏÓÌ (îëï) ÔÓ͇Á‚‡ ÔÓ-ÌËÒÍË ÓÚ ÌÓχÎÌËÚ ÒÚÓÈÌÓÒÚË: 1.54 ± 0.86 mIU/ml (ÔË ÌÓχ 2 – 10 mIU/ml). ëÚÓÈÌÓÒÚËÚ ̇ ÂÒÚ‡‰ËÓ·, ËÁÒΉ‚‡ÌË ÔË 10 ÊÂÌË, Ò‡ ÔÓÌËÊÂÌË ‚ Ò‡‚ÌÂÌËÂ Ò ÌÓχڇ: 19.21 ± 6.46 pg/ml (ÔË ÌÓχ 30 – 90 pg/ml). ÜÂÌËÚ ÔÓ͇Á‚‡Ú Ë ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ ÎÛÚÂËÌËÁË‡˘Ëfl ıÓÏÓÌ: 3.75 ± 0.25 mIU/ml (ÔË ÌÓχ ÓÚ 4 - 12 mIU/ml), ‰Ó͇ÚÓ ÒÚÓÈÌÓÒÚËÚ ̇ ÙÓÎËÍÛÎÓÒÚËÏÛÎË‡˘Ëfl ıÓÏÓÌ Ò‡ ‚ „‡ÌˈËÚ ̇ ÌÓχڇ: 7.60 ± 0.59 mIU/ml (ÌÓχ 4 - 12 mIU/ml). ÜÂÌËÚÂ Ò ‚ÚÓ˘̇ ‡ÏÂÌÓ‡ Ò‡ ËÁÍβ˜ÂÌË ÓÚ ÒÚ‡ÚËÒÚ˘ÂÒÍËfl ‡Ì‡ÎËÁ. ç  ÛÒÚ‡ÌÓ‚ÂÌ Ô‡‡ÎÂÎËÁ˙Ï ÏÂÊ‰Û ÒÚÓÈÌÓÒÚËÚ ̇ ÂÒÚ‡‰ËÓ·, ãï, îëï, ÛÎÚ‡Á‚ÛÍÓ‚ËÚ ԇ‡ÏÂÚË Ì‡ χÚ͇ڇ Ë flȘÌˈËÚÂ Ë ÏÂÌÒÚÛ‡ÎÌËÚ ̇Û¯ÂÌËfl (ÓÎË„ÓÏÂÌÓ‡, ÔÓÎËÏÂÌÓ‡). íÂÁË ÂÁÛÎÚ‡ÚË ÔÓ͇Á‚‡Ú ÎÂÍ ıËÔÓ„Ó̇‰ËÁ˙Ï, ͇ÍÚÓ ÔË Ï˙ÊÂÚÂ, ڇ͇ Ë ÔË ÊÂÌËÚÂ, ‰ÂÏÓÌÒÚË‡Ì Ò ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ ÚÂÒÚÓÒÚÂÓÌ Ë ÂÒÚÓ„ÂÌË. ïËÔÓ„Ó̇‰ËÁÏ˙Ú Ì‡È-‚ÂÓflÚÌÓ Â ÓÚ ıËÔÓ„Ó̇‰ÓÚÓÔÂÌ ÚËÔ. äÓÒÚ̇ڇ ÏËÌÂ‡Î̇ ÔÎ˙ÚÌÓÒÚ Â ÔÓÌËÊÂ̇ ‚ ÓÒÚÂÓÔÂÌ˘ÌËfl/ÓÒÚÂÓÔÓÓÚ˘ÂÌ ‰Ë‡Ô‡ÁÓÌ, ÌÂÁ‡‚ËÒËÏÓ ˜Â Ô‡ˆËÂÌÚËÚ ҇ ̇ ‚˙Á‡ÒÚ, ÍÓ„‡ÚÓ Ò ÔÓÒÚË„‡ ÔËÍÓ‚‡ ÍÓÒÚ̇ χ҇. èË ‚Ò˘ÍË ËÁÒΉ‚‡ÌË ËÌ‰Ë‚Ë‰Ë Ëχ ‰Û͈Ëfl ̇ ÍÓÒÚ̇ڇ ÏËÌÂ‡Î̇ ÔÎ˙ÚÌÓÒÚ ‚ ÍÓÏÔ‡ÍÚÌËÚÂ Ë Ú‡·ÂÍÛ·ÌËÚ ÍÓÒÚË, ÌÓ ‚ ‡Á΢̇ ÒÚÂÔÂÌ. ç‰ÓÒÚË„‡ÌÂÚÓ Ì‡ ÓÔÚËχÎ̇ ÍÓÒÚ̇ χ҇ ÔÂÁ ÚÂÚ‡Ú‡ ‰Â͇‰‡ ÓÚ ÊË‚ÓÚ‡ ÏÓÊ ‰‡ Ò ӷÛÒ·‚fl ÓÚ ‡Á΢ÌË Ù‡ÍÚÓË: ‰ÂÙˈËÚ Ì‡ ÔÓÎÓ‚ËÚÂ Ë ÒÓχÚÓÚÓÔÌËfl ıÓÏÓÌË, ̇χÎÂÌÓ Í‡ÎˆËÂ‚Ó ÛÒ‚Ófl‚‡ÌÂ, ıËÔÓÍËÌÂÁËfl ËÎË ‰‡  Ò˙Ô˙ÚÒÚ‚‡˘ ÔÂËÙÂ̇ڇ Ì‚ÓÔ‡ÚËfl ÔËÁ̇Í. ìÎÚ‡Á‚ÛÍÓ‚Ó ËÁÒΉ‚‡Ì ̇ χÚ͇ Ë flȘÌËˆË Â ÓÒ˙˘ÂÒÚ‚ÂÌÓ ÔË 10 ÊÂÌË. ë‰̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ Ô‰ÌÓ-Á‡‰ÌËfl ‰Ë‡ÏÂÚ˙ ̇ χÚ͇ڇ  27.3 ± 2.1 ÏÏ; ‰Â·ÂÎË̇ ̇ ẨÓÏÂÚËÛÏ: 3.5 ± 1.3 ÏÏ; ‡ÁÏÂË Ì‡ ‰ÂÒÂÌ flȘÌËÍ: 35 ± 2.3 / 15 ± 2.6 / 14 ± 3.0 ÏÏ; ‡ÁÏÂË Ì‡ Îfl‚ flȘÌËÍ: 36.5 ± 1.5 / 16 ± 2.0 / 14 ± 2.5 ÏÏ. ìÒÚ‡ÌÓ‚fl‚‡ Ò ÎÂ͇ ıËÔÓÔ·ÁËfl ̇ χÚ͇ڇ Ë flȘÌˈËÚ ÔË ‚Ò˘ÍË ËÁÒΉ‚‡ÌË ÊÂÌË. êÂÁÛÎÚ‡ÚËÚ ÓÚ Ôӂ‰ÂÌËÚ ËÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â Ì‚Ó-ẨÓÍËÌ̇ڇ ‰ËÒ„Û·ˆËfl  ‰˙ηÓÍÓ ‡Ì„‡ÊË‡Ì‡ ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÌÓ Úfl Ì Á‡Òfl„‡ ‚Ò˘ÍË ıËÔÓÙËÁ‡ÌË ÓÒË. ìÒÚ‡ÌÓ‚ÂÌÓ Â ÌÓχÎÌÓ ÙÛÌ͈ËÓÌË‡ÌÂ, ͇ÍÚÓ Ì‡ ıËÔÓڇ·ÏÛÒ-ıËÔÓÙËÁ‡-̇‰·˙·˜̇ڇ ÓÒ, ڇ͇ Ë Ì‡ ıËÔÓڇ·ÏÛÒ-ıËÔÓÙËÁ‡-ÚËÂÓˉ̇ڇ ÓÒ. ÇÚÓ˘ÌËÚ ÔÓÎÓ‚Ë ·ÂÎÂÁË ÔÓ͇Á‚‡Ú ÌÓχÎ̇ ÔÓÎÓ‚‡ ‰ËÙÂÂ̈ˇˆËfl Ë Ì‡˜‡ÎÓ Ì‡ ÔÛ·ÂÚÂÚ‡. èÓ˜ÚË ‚Ò˘ÍË ÊÂÌË Ó·‡˜Â Ò˙Ó·˘‡‚‡Ú Á‡ ÏÂÌÒÚÛ‡ÎÌË Ì‡Û¯ÂÌËfl ÓÚ ‡Á΢ÂÌ ‚ˉ: ÓÎË„Ó-, ÔÓÎË- Ë ‡ÏÂÌÓ‡. ïÓÏÓ̇ÎÌËÚ ÂÁÛÎÚ‡ÚË ÔÓÚ‚˙ʉ‡‚‡Ú ̇΢ËÂÚÓ Ì‡ ıËÔÓ„Ó̇‰ËÁ˙Ï Ë ÔË ‰‚‡Ú‡ ÔÓ·. ìÒÚ‡ÌÓ‚fl‚‡Ú Ò ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ ãï

145

ÔË ÊÂÌËÚÂ Ë Ì‡ îëï ÔË Ï˙ÊÂÚÂ. ç‡È-‚ÂÓflÚÌÓ Â Ì‡Û¯Â̇ ıËÔÓڇ·Ï˘̇ڇ ‰ËÒ„Û·ˆËfl ̇ „Ó̇‰Ì‡Ú‡ ÓÒ, ÍÓflÚÓ ÔÓ„ÂÒË‡ Ë Ò ÔÓfl‚fl‚‡ ÍÎËÌ˘ÂÒÍË ÒΉ ̇˜‡ÎÓÚÓ Ì‡ ÔÛ·ÂÚÂÚ‡. Ö‰ËÌ ÓÚ Ì‡È-flÍËÚ ÍÎËÌ˘ÌË ÔËÁ̇ˆË  ÌËÒÍËflÚ ˙ÒÚ, ͇ÍÚÓ Á‡ Ï˙ÊÂÚ (-3.8 SD), ڇ͇ Ë Á‡ ÊÂÌËÚ (2.95 SD). ŇÁ‡ÎÌËÚ ÌË‚‡ ̇ ëíï ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ ÌËÒÍÓ ÌÓχÎÌË, ‡ ËÌÒÛÎËÌ-Ë̉ۈË‡Ì‡Ú‡ ıËÔÓ„ÎËÍÂÏËfl Ô‰ËÁ‚ËÍ‚‡ Ôӂ˯ÂÌË ̇ ëíï. íÂÁË ÂÁÛÎÚ‡ÚË ÌË ÔÓÁ‚ÓÎfl‚‡Ú ‰‡ ÔËÂÏÂÏ, ˜Â  ̇Îˈ Ì‚Ó-„Û·ÚÓÂÌ ‰ÂÙˈËÚ Ì‡ ëíï – ̇Û¯Â̇ ÒÔÓÌÚ‡Ì̇ ÒÂÍˆËfl ̇ ëíï (ÌËÒÍË Ë ‰ÍË ÔËÍӂ ̇ ÒÂÍˆËfl), ÌÓ Á‡Ô‡ÁÂ̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÒÚËÏÛ·ˆËË. çÖÇêéàáéÅêÄáàíÖãçà àáëãÖÑÇÄçàü ä‡ÌËÓ„‡ÙËËÚ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú: 1/ ç‡Û¯ÂÌÓ Ò˙ÓÚÌÓ¯ÂÌË ÏÂÊ‰Û flÏÍËÚ ̇ ˜ÂÂÔ̇ڇ ÓÒÌÓ‚‡: ‡) Û‚Â΢Â̇ Ô‰̇ ˜ÂÂÔ̇ flÏ͇; ·) Û‚Â΢ÂÌ Ì‡ÍÎÓÌ Ì‡ Ô·ÌÛÏ ÒÙÂÌÓˉ‡ÎÂ; ‚) ‰˙ηÓÍÓ ‡ÁÔÓÎÓÊÂÌË ̇ Ò· ÚÛˆË͇; 2/ ç‡Û¯ÂÌÓ Ò˙ÓÚ‚ÂÚÒÚ‚ËÂ Ë „ÓÎÂÏË̇ ̇ „Ó̇ڇ Ë ‰ÓÎ̇ڇ ˜ÂβÒÚ: ‡) ‚ ‰‚‡ ÒÎÛ˜‡fl  ÛÒÚ‡ÌÓ‚Â̇ ÏËÍӄ̇ÚËfl Ë ÏËÍÓ„ÂÌËfl; ·) ‚ ÓÒڇ̇ÎËÚ ÒÎÛ˜‡Ë – Ôӄ̇ÚËfl Ò˙˜Âڇ̇ Ò ÔÓ„ÂÌËfl; 3/ èÓÏÂÌË ‚ ÚÂÏÔÓÓ-χ̉˷Û·ÌËÚ ÒÚ‡‚Ë Ë ‚ ‰ÓÎ̇ڇ ˜ÂβÒÚ: ‡) “ÔÎËÚÍË” ÍÓ̉ËÎÓˉÌË flÏÍË; ·) ıËÔÓÔ·ÁËfl ̇ ‡ÚËÍÛÎË‡˘ËÚ ËÁ‡ÒÚ˙ˆË Ò Ì‡Ï‡ÎÂÌ ÍÓÌ‚ÂÍÒËÚÂÚ Ì‡ ÒÚ‡‚ÌËÚ ËÏ ÔÓ‚˙ıÌËÌË; ‚) Û‚Â΢ÂÌ ˙„˙Î ÏÂÊ‰Û ıÓËÁÓÌÚ‡ÎÌËfl Ë ‚ÂÚË͇ÎÌËfl ÍÎÓÌ Ì‡ ‰ÓÎ̇ڇ ˜ÂβÒÚ; 4/ èÓÏÂÌË ‚ „Ó̇ڇ ˜ÂβÒÚ: ‡) ıËÔÂÔÌ‚χÚËÁ‡ˆËfl ̇ χÍÒË·ÌËÚ ÒËÌÛÒË; ·) ԇ·ÚÛÏ ÙËÒÛÏ. åêí ̇ „·‚ÌËfl Ë „˙·Ì‡˜ÌËfl ÏÓÁ˙Í ÔÓ͇Á‚‡Ú Û‚Âʉ‡ÌËfl ÔË 17 ÓÚ 18 ËÁÒΉ‚‡ÌË Ô‡ˆËÂÌÚË. àÁÍβ˜ÂÌË Ô‡‚Ë 10 „Ӊ˯ÌÓ ÏÓϘÂ, ÍÓÂÚÓ Â Ò ÌÓχÎ̇ ̇ıӉ͇, ‚˙ÔÂÍË ˜Â ÔË 6-„Ӊ˯ÌÓ ÏÓÏ˘ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Á̇˜ËÚÂÎ̇ ‡ÚÓÙËfl ̇ „·‚ÌËfl Ë „˙·Ì‡˜ÌËfl ÏÓÁ˙Í. åÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò ‡Á¯ËÂÌË Î‡ÚÂ‡ÎÌË ‚ÂÌÚËÍÛÎË Â ÔÓÒÚÓflÌ̇ ̇ıӉ͇ ÔË Á‡Ò„̇ÚËÚ Ë̉˂ˉË. ÄÚÓÙËflÚ‡  ‰ËÙÛÁ̇ ÔË 15 Ô‡ˆËÂÌÚË, ‡ ÔË ‰Û„Ë ‰‚‡Ï‡ ̇ ‚˙Á‡ÒÚ 18 Ë 21 „. ÔÂӷ·‰‡‚‡˘Ó Á‡Òfl„‡ ÙÓÌÚ‡ÎÌËÚ ÎÓ·Ó‚Â. èË Â‰ËÌ Ô‡ˆËÂÌÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÒËÎÌÓ ËÁÚ˙ÌflÎ ÍÓÔÛÒ Í‡ÎÓÁÛÏ, ‡ ÔË ‰Û„ Ò ‚Ëʉ‡, ˜Â ıˇÁχ ÓÔÚËÍÛÏ Â ‰ۈË‡Ì‡ ‚ Á̇˜ËÚÂÎ̇ ÒÚÂÔÂÌ. ÄÚÓÙËfl ̇ „˙·Ì‡˜ÌËfl ÏÓÁ˙Í Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 13 Ë̉˂ˉË, ‡ ÔË ‰‚‡Ï‡ ÓÚ Úflı ‡ÚÓÙËflÚ‡ Á‡Òfl„‡ Ë ÔÓ‰˙΄ӂ‡ÚËfl ÏÓÁ˙Í. ëËÏÂÚ˘ÌË ÔÓ‡ÊÂÌËfl ‚ ÔÂË‚ÂÌÚËÍÛ·ÌÓÚÓ ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó Ò ̇·Î˛‰‡‚‡Ú ÔË 7 Ô‡ˆËÂÌÚË Ì‡ ‚˙Á‡ÒÚ Ì‡‰ 30 „., ‡ ÔË ‰‚‡Ï‡ ÓÚ Úflı Ò ÓÚÍË‚‡Ú Ë ÔÓ‡ÊÂÌËfl ‚ „˙·Ì‡˜ÌËfl ÏÓÁ˙Í. Ö‰ËÌ˘ÌË ÔÓ-„ÓÎÂÏË ÔÓ‡ÊÂÌËfl Ò ÌÂflÒÂÌ ı‡‡ÍÚÂ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË 3 Ô‡ˆËÂÌÚË: ‚ „ÎÓ·ÛÒ Ô‡ÎˉÛÒ – ÔË 6 „Ӊ˯ÌÓ ÏÓÏ˘Â, ̇ ÔÓÌÚÓωÛ·ÌËfl ÔÂıÓ‰ – ÔË 33 „Ӊ˯̇ ÊÂ̇ Ë ‚ ӷ·ÒÚÚ‡ ̇ ‰ÂÒÌËfl ÔÛÚ‡ÏÂÌ ÔË 40 „Ӊ˯̇ Ô‡ˆËÂÌÚ͇. çÖÇêéèÄíéãéÉàóçà àáëãÖÑÇÄçàü àÁÒΉ‚‡ÌË Ò‡ ÒÛ‡ÎÌË ÌÂ‚ÌË ÒÔÂÒËÏÂÌË ÓÚ 4 Ô‡ˆËÂÌÚË Ì‡ ‚˙Á‡ÒÚ 4, 10, 28 Ë 32 „Ó‰ËÌË. èÎ˙ÚÌÓÒÚÚ‡ ̇ ÏËÂÎËÌËÁË‡ÌËÚ ÌÂ‚ÌË ‚·Í̇ ‚ ÒÛ‡ÎÌËfl ÌÂ‚  ‚ „‡ÌˈËÚ ̇ ÌÓχڇ Ë ÔË ˜ÂÚËËÚ ËÁÒΉ‚‡ÌË Ë̉˂ˉË, ÌÓ ÔË ‚ÒÂÍË Â‰ËÌ ÓÚ Úflı  ̇Îˈ ıËÔÓÏËÂÎËÌËÁ‡ˆËfl. ífl Á‡Òfl„‡, ͇ÍÚÓ ÔÓÔÛ·ˆËËÚ ̇ „ÓÎÂÏËÚÂ, ڇ͇ Ë ÔÓÔÛ·ˆËËÚ ̇ χÎÍËÚ ‚·Í̇. íÓ‚‡ ÒÔÓχ„‡ Á‡ Ó·flÒÌÂÌË ̇ Ó·˘ÓÚÓ Á‡·‡‚flÌ ̇ ÔÓ‚Ó‰ËÏÓÒÚÚ‡, Á‡Òfl„fl˘Ó ‚Ò˘ÍË ‚·Í̇ ‚ ÔË·ÎËÁËÚÂÎÌÓ Â‰Ì‡Í‚‡ ÒÚÂÔÂÌ. Ç˙ıÛ ıËÔÓÏËÂÎËÌËÁ‡ˆËflÚ‡ Ò ̇Ò·„‚‡ Ë ‰ÂÏËÂÎËÌËÁ‡ˆËfl/ÂÏËÂÎËÌËÁ‡-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ˆËfl, ÍÓflÚÓ ÔÓ„ÂÒË‡ Ò Û‚Â΢‡‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡. èË Ì‡È-‚˙Á‡ÒÚÌËfl ËÁÒΉ‚‡Ì Ë̉˂ˉ Ò‡ ̇Îˈ ‰‡ÌÌË Ë Á‡ ‡ÍÒÓ̇Î̇ ‰Â„ÂÌÂ‡ˆËfl/„ÂÌÂ‡ˆËfl. íÂÁË ÔÓÏÂÌË Ó·flÒÌfl‚‡Ú Á‡„Û·‡Ú‡ ̇ ÒÂÚË‚ÌË ‡ÍˆËÓÌÌË ÔÓÚÂ̈ˇÎË ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË ÔË ÌflÍÓË Ô‡ˆËÂÌÚË. ãËÔÒ‡Ú‡ ̇ ËÁ‡ÁÂÌË ıËÔÂÚÓÙ˘ÌË ÔÓÏÂÌË ÔÓ͇Á‚‡, ˜Â Ì Ò ÓÒ˙˘ÂÒÚ‚fl‚‡Ú ÔÓ‚Úflfl˘Ë Ò ˆËÍÎË Ì‡ ‰ÂÏËÂÎËÌËÁ‡ˆËfl Ë ÂÏËÂÎËÌËÁ‡ˆËfl, ÔÓÌ ‚ ‡ÌÌËÚ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡. ÉÖçÖíàóçé àáëãÖÑÇÄçÖ èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‰ËÌ˘̇ ÌÛÍÎÂÓÚˉ̇ Á‡Ïfl̇ (ë➞í) ‚ ‡ÌÚËÒÂÌÒ Alu ÂÎÂÏÂÌÚ Ì‡ ËÌÚÓÌ 6 ̇ CTDP1 (ÍÓ‰Ë‡˘ ÔÓÚÂË̇ ÙÓÒÙ‡Ú‡Á‡ FCP1, ÓÒÌÓ‚ÂÌ ÍÓÏÔÓÌÂÌÚ Ì‡ ÂÛ͇ËÓÚÌËfl Ú‡ÌÒÍË·ˆËÓÌÂÌ ÏÂı‡ÌËÁ˙Ï). åÛÚ‡ˆËflÚ‡ Ô˘ËÌfl‚‡ ‡·Â‡ÌÚÂÌ ÒÔ·ÈÒËÌ„ Ë Alu ËÌÒÂˆËfl ‚ mRNA. í‡Í‡ ääãÑç ÒË̉ÓÏ Ò ÔËÒ˙‰ËÌfl‚‡ Í˙Ï „ÛÔ‡Ú‡ ̇ “Ú‡ÌÒÍË·ˆËÓÌÌËÚ ÒË̉ÓÏË” Ë Â Ô˙‚Ëfl “˜ËÒÚ” Ú‡ÌÒÍË·ˆËÓÌÂÌ ‰ÂÙÂÍÚ, ÍÓÈÚÓ Á‡Òfl„‡ ÔÓÎËÏÂ‡Á‡ II –ωËË‡Ì‡Ú‡ „ÂÌ̇ ÂÍÒÔÂÒËfl (22). ëÍËÌË‡ÌÂÚÓ Á‡ ÏÛÚ‡ˆËflÚ‡ ̇ 887 ÌÂÁ‡Ò„̇ÚË ÍÓÌÚÓÎË ÛÒÚ‡ÌÓ‚Ë 6.9% ÌÓÒËÚÂÎÒÚ‚Ó Ò‰ ÍÓԇ̇ËÚÂ, 0.6% ÌÓÒËÚÂÎÒÚ‚Ó ÔË ÓÒڇ̇ÎËÚ ˆË„‡ÌÒÍË „ÛÔË Ë 0% ÔË ÌÂ-ÓÏËÚÂ. íÂÁË ‰‡ÌÌË ÔÓ͇Á‚‡Ú Ì ҇ÏÓ, ˜Â ÏÛÚ‡ˆËflÚ‡  ÒÔˆËÙ˘̇ Á‡ ÓÏÒÍËfl ÂÚÌÓÒ, ÌÓ Ò˙˘Ó, ˜Â Úfl ̇ȂÂÓflÚÌÓ Â ‚˙ÁÌËÍ̇· ÒΉ ‰Ë‚Â„Â̈ËflÚ‡ ̇ ÓÏËÚÂ Ë Á‡ ÚÓ‚‡ Ò ̇·Î˛‰‡‚‡ ÔÂӷ·‰‡‚‡˘Ó ÔË ‚·¯ÍËÚ ˆË„‡ÌË, ÍÓԇ̇ËÚÂ. íÂÁË ÂÁÛÎÚ‡ÚË ÒÔÓχ„‡Ú Á‡ Ô·ÌË‡Ì ̇ ·˙‰Â˘‡ ÒÂÎÂÍÚ˂̇ „ÂÌÂÚ˘̇ ÔÓÙË·ÍÚ˘̇ ÔÓ„‡Ï‡ Á‡ ÚÓ‚‡ Á‡·ÓÎfl‚‡ÌÂ. éÅëöÜÑÄçÖ à‰ÂÌÚËÙˈË‡ÌÓ Â Á‡ Ô˙‚Ë Ô˙Ú ÒÎÓÊÌÓ Ì‡ÒΉÒÚ‚ÂÌÓ ÏÛÎÚËÒËÒÚÂÏÌÓ Á‡·ÓÎfl‚‡ÌÂ. èË ‚Ò˘ÍËÚ 73 Ô‡ˆËÂÌÚË Ò ̇·Î˛‰‡‚‡ ÍÓÌÒÚ·ˆËfl ÓÚ ÍÎËÌ˘ÌË ÔËÁ̇ˆË, ‚Íβ˜‚‡˘‡ ‰‚ÛÒÚ‡Ì̇ ÍÓÌ„ÂÌËÚ‡Î̇ ͇ڇ‡ÍÚ‡ Ë ÏËÍÓÍÓÌÂfl, ı‡‡ÍÚÂÂÌ Îˈ‚ ‰ËÁÏÓÙËÁ˙Ï, ıËÔÓ„Ó̇‰ÓÚÓÔÂÌ ıËÔ„Ó̇‰ËÁ˙Ï Ë ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl. ç‡È-ı‡‡ÍÚÂ̇ڇ ÔÓfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â Ì‚ÓÔ‡ÚËflÚ‡, ÍÓflÚÓ Â Ô‰ËÏÌÓ ÏÓÚÓ̇ Ë ıËÔÓÏËÂÎËÌËÁË‡˘‡/‰ÂÏËÂÎËÌËÁË‡˘‡. á‡Òfl„‡ÌÂÚÓ Ì‡ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ Ò ÔÓfl‚fl‚‡ Ò Ì‡Î˘ËÂÚÓ Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ÔË Ôӂ˜ÂÚÓ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ë ‰‡ÌÌË Á‡ Á‡Òfl„‡Ì ̇ ÍÓÚËÍÓÒÔË̇ÎÌËÚ Ô˙Úˢ‡ Ë ÎÂ͇ ıÓÂfl ÛÒÚ‡ÌÓ‚ÂÌË ÔË ÓÍÓÎÓ 1/3 ÓÚ Ô‡ˆËÂÌÚËÚ . íÓ‚‡ Ò ÔÓÚ‚˙ʉ‡‚‡ ÓÚ ‡·ÌÓÏÌËÚ Ì‚ÓËÁÓ·‡ÁËÚÂÎÌË Ë ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙÒÍË Ì‡ıÓ‰ÍË. ç‡Î˘ËÂÚÓ Ì‡ „˙·Ì‡˜ÌÓ-ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ô‰ÔÓ·„‡ Ë ËÁÚ˙Ìfl‚‡Ì ̇ „˙·Ì‡˜ÌÓ-ÏÓÁ˙˜ÌËÚ Ú‡ÍÚÓ‚Â. ãˈ‚ËflÚ ‰ËÁÏÓÙËÁ˙Ï ÒÚ‡‚‡ ÔÓ-ËÁ‡ÁÂÌ ‚ ÔÓ-Í˙ÒÌÓÚÓ ‰ÂÚÒÚ‚Ó Ë Â ÔÓ-‰ÂÏÓÌÒÚ‡ÚË‚ÂÌ ÔË Ï˙ÊÂÚÂ, ÓÚÍÓÎÍÓÚÓ ÔË ÊÂÌËÚÂ. ãËÔÒ‚‡Ú ÔÓÎÓ‚Ë ‡Á΢Ëfl ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ì‚ÓÎӄ˘ÌËÚ ÔÓfl‚Ë. èË ÌflÍÓË ÓÚ Ô‡ˆËÂÌÚËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÎÂ͇ ‡Ú‡ÍÒËfl, ÌÂÒ‚˙Á‡Ì‡ Ò Ì‡Û¯ÂÌËÂÚÓ Ì‡ ÔÓÔËÓˆÂÔÚ˂̇ڇ ÒÂÚË‚ÌÓÒÚ. ç‡Î˘ËÂÚÓ Ì‡ ÎÂÍÓ Ï‡ÎÍÓÏÓÁ˙˜ÌÓ Á‡Òfl„‡Ì Ò ÔÓ‰ÍÂÔfl Ë ÓÚ ÂÎÂÍÚÓÌËÒÚ‡„ÏÓ„‡ÙÒÍËÚ ËÁÒΉ‚‡ÌËfl Ë ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ Í‚‡‰‡ÌÚÌÓ‚˙ÎÌÓ‚Ë ÔÓÚÂÔ‚‡ÌËfl ̇ Ó˜ÌËÚ fl·˙ÎÍË ÔÓ ‚ÂÏ ̇ ÔÓÒΉfl‚‡˘ËÚ ÔӄΉÌË ‰‚ËÊÂÌËfl ÔË ÓÚ‰ÂÎÌË Ô‡ˆËÂÌÚË. å‡ÎÍÓÏÓÁ˙˜ÌËÚ ÔÓfl‚Ë Ì ҇ Á̇˜ËÏË Ë Ì Ò ÓÚÍË‚‡Ú ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË. 燷≇‚‡ÌËflÚ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË ÔÓÒÚÛ‡ÎÂÌ ÚÂÏÓ ‚ „ÓÌËÚ Í‡ÈÌËˆË ‚ÂÓflÚÌÓ Ô‰ÒÚ‡‚Îfl‚‡ “Ì‚ÓÔ‡ÚÂÌ ÚÂÏÓ”, Ò‚˙Á‡Ì Ò Ì‡Ï‡ÎÂ̇ڇ ëè ÔÓ ÌÂ‚ÌËÚ ‚·Í̇. èË ËÌÚÂÔÂÚË‡Ì ̇ ̇Û¯ÂÌËÚ ÍÓ„ÌËÚË‚ÌË ÙÛÌ͈ËË Â ‚‡ÊÌÓ ‰‡ Ò ÓÚ·ÂÎÂÊË, ˜Â Ôӂ˜ÂÚÓ Ô‡ˆËÂÌÚË ÔÓËÁıÓʉ‡Ú ÓÚ ËÁÓÎË‡Ì‡ ÂÚÌ˘ÂÒ͇ „ÛÔ‡ Ò ÌËÒ˙Í ÒÓ-

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ˆË‡ÎÂÌ ÒÚ‡ÚÛÒ Ë ˜Â ÔÓ‡‰Ë ÍÓÌ„ÂÌËÚ‡ÎÌËÚ ͇ڇ‡ÍÚË ‚Ò˘ÍË ‰Âˆ‡  Úfl·‚‡ÎÓ ‰‡ ÔÓÒ¢‡‚‡Ú ÒÔˆˇÎÌË Û˜ËÎˢ‡. çËÒÍËÚ ÂÁÛÎÚ‡ÚË ÓÚ Ôӂ‰ÂÌËÚ ÔÒËıÓÏÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl Ò ÔÓÚ‚˙ʉ‡‚‡Ú ÓÚ Á‡·‡‚ÂÌÓÚÓ ÂÁËÍÓ‚Ó ‡Á‚ËÚË ÔË Ô‡ˆËÂÌÚËÚÂ Ò ääãÑç, ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂıÌËÚ ÌÂÁ‡Ò„̇ÚË ·‡Úfl Ë ÒÂÒÚË, ÍÓÂÚÓ Ò Ò˙Ó·˘‡‚‡ ÓÚ ÚÂıÌËÚ Ó‰ËÚÂÎË Ë ÓÚ Ì‡·Î˛‰ÂÌËÂÚÓ, ˜Â Úflı̇ڇ ÛÒÔ‚‡ÂÏÓÒÚ ‚ Û˜ËÎˢ  ÏÌÓ„Ó ÔÓ-ÌËÒ͇, ‚ Ò‡‚ÌÂÌËÂ Ò ‰Û„Ë ‰Âˆ‡ ÓÏË, ÌÂÁ‡Ò„̇ÚË ÓÚ ääãÑç Ë Ò ‰Û„Ë ‰Âˆ‡, ÔÓÒ¢‡‚‡˘Ë Ò˙˘ËÚ ۘËÎˢ‡. çÂÁ‡‚ËÒËÏÓ ÓÚ åêí ‰‡ÌÌËÚÂ, ˜Â ÏÓÁ˙˜Ì‡Ú‡ ‡ÚÓÙËfl Ò ۂÂ΢‡‚‡ Ò ‚˙Á‡ÒÚÚ‡, Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰‡ÌÌË Á‡ ÔÓ„ÂÒË‡Ì ̇ ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ. àÁÒΉ‚‡ÌËflÚ‡ ̇ ÌÂ‚̇ڇ ÔÓ‚Ó‰ËÏÓÒÚ ÔÓ͇Á‚‡Ú Á‡·‡‚flÌ ‚ ‰ÂÏËÂÎËÌËÁË‡˘Ëfl ‰Ë‡Ô‡ÁÓÌ. àÌÚÂÂÒÂÌ Ù‡ÍÚ Â, ˜Â ‚˙ÔÂÍË Á̇˜ËÚÂÎÌÓÚÓ Á‡·‡‚flÌ ̇ ÔÓ‚Âʉ‡ÌÂÚÓ ÔÓ ÒÂÚË‚ÌËÚ ‚·Í̇, ‡ÏÔÎËÚÛ‰‡Ú‡ ̇ ÒÂÚË‚ÌËÚ ÌÂ‚ÌË ‡ÍˆËÓÌÌË ÔÓÚÂ̈ˇÎË ˜ÂÒÚÓ Â ‰Ó· Á‡Ô‡ÁÂ̇ Ë ÚÓ‚‡ Ì Ò ‰˙ÎÊË Ì‡ ÂÙÂÍÚ Ì‡ Óı·ʉ‡ÌÂ. ìÒÚ‡ÌÓ‚Ë Ò ÓÚÌÓÒËÚÂÎÌÓ Â‰Ì‡Í‚‡ ÒÚÂÔÂÌ Ì‡ Á‡·‡‚flÌ ̇ ÔÓ‚Ó‰ËÏÓÒÚÚ‡ ÔÓ ‚Ò˘ÍË ÌÂ‚ÌË ‚·Í̇, ÍÓÂÚÓ „Ó‚ÓË Á‡ Ò‚˙Á‡Ì‡ Ò ‡Á‚ËÚËÂÚÓ ıËÔÓÏËÂÎËÌËÁ‡ˆËfl. èÓ‰Ó·ÌË Ì‡ıÓ‰ÍË Ò‡ ̇·Î˛‰‡‚‡ÌË ÔË ·ÓÎÂÒÚÚ‡ ̇ Leigh (9). èÂËÙÂ̇ڇ Ì‚ÓÔ‡ÚËfl ÔÓ„ÂÒË‡, ÍÓÂÚÓ ÎË˜Ë ÓÚ ÔÓ-ËÁ‡ÁÂ̇ڇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl ̇ Í‡ÈÌËˆË Ì‡ ÔÓ-‚˙Á‡ÒÚÌËÚ ԇˆËÂÌÚË. ìÒÚ‡ÌÓ‚Ë Ò ҇ÏÓ ÎÂÍÓ ÒÌËÊÂÌËÂÚÓ Ì‡ ÔÓ‚Ó‰ËÏÓÒÚÚ‡ ÔÓ ÏÓÚÓÌËÚ ‚·Í̇ Ò ‚˙Á‡ÒÚÚ‡, ÍÓÂÚÓ Ì  Á̇˜ËÚÂÎÌÓ ‚ ÔÂÓ̇ÎÌËÚ ÌÂ‚Ë Ë ÍÓÂÚÓ ÔÓ͇Á‚‡, ˜Â ‡ÍÒÓ̇Î̇ڇ Û‚‰‡ Ì  Ô˘ËÌÂ̇ ÓÚ Ì‡Ô‰‚‡˘‡ ‰ÂÏËÂÎËÌËÁ‡ˆËfl. 凄ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ڇ ÚÓÏÓ„‡ÙËfl ̇ ÏÓÁ˙͇ ÛÒÚ‡ÌÓ‚Ë, ÓÒ‚ÂÌ ÒËÒÚÂÏ̇ ÏÓÁ˙˜Ì‡ Ë „˙·Ì‡˜ÌÓ-ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl, Ë ÙÓ͇ÎÌË ÔÓ‡ÊÂÌËfl, ‚Íβ˜ËÚÂÎÌÓ ‚ ÔÂË‚ÂÌÚËÍÛ·ÌÓÚÓ ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó ÔË ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ. íflıÌÓÚÓ Ó·flÒÌÂÌË Á‡Ò„‡  ÌÂÒË„ÛÌÓ, ÌÓ ÔÓÏÂÌËÚ Ì Ò˙ÓÚ‚ÂÚÒÚ‚‡Ú ̇ ÚÂÁË ÔË Î‚ÍÓ‰ËÒÚÓÙËfl. ãÂÍ ıËÔÓ„Ó̇‰ËÁ˙Ï Â ÛÒÚ‡ÌÓ‚ÂÌ Ë ÔË ‰‚‡Ú‡ ÔÓ·. çÂÁ‡‚ËÒËÏÓ ÓÚ ÚÓ‚‡, ‚˙̯ÌËÚ ‚ÚÓ˘ÌË ÔÓÎÓ‚Ë ı‡‡ÍÚÂËÒÚËÍË ËÁ„ÎÂʉ‡Ú ÌÓχÎÌË, ‚˙ÔÂÍË ˜Â ÔË ÛÎÚ‡Á‚ÛÍÓ‚Ó ËÁÒΉ‚‡Ì Ò ÓÚÍË‚‡Ú ̇χÎÂÌË ‡ÁÏÂË Ì‡ flȘÌˈËÚÂ Ë Ï‡Ú͇ڇ, ‡ Ò˙˘Ó ڇ͇ ÔÓ˜ÚË ‚Ò˘ÍË ÊÂÌË Ò˙Ó·˘‡‚‡Ú Á‡ ÌÂ‰ӂÌË ÏÂÌÒÚÛ‡ÎÌË ˆËÍÎË, ÔÓ„ÂÒË‡˘Ë ‰Ó ‚ÚÓ˘̇ ‡ÏÂÌÓ‡. ëÚÓÈÌÓÒÚËÚ ̇ ÒÓχÚÓÚÓÔÌËfl ıÓÏÓÌ Ò‡ ÌËÒÍÓ ÌÓχÎÌË, Ë Ò˙ÓÚ‚ÂÚÒÚ‚‡Ú ̇ ̇χÎÂÌËfl ˙ÒÚ. 燷≇‚‡ Ò ËÁ‡ÁÂÌÓ Ôӂ˯ÂÌË ̇ ıÓÏÓ̇ ÒΉ ËÌÒÛÎËÌ – Ë̉ۈË‡Ì‡ ıËÔÓ„ÎËÍÂÏËfl. íÓ‚‡ „Ó‚ÓË Á‡ „Û·ÚÓ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ì‡ ‡ÒÚÂÊÌËfl ıÓÏÓÌ. ç ҇ ÛÒÚ‡ÌÓ‚ÂÌË ‰‡ÌÌË Á‡ „ÂÌÂ‡ÎËÁË‡Ì ıËÔÓÔËÚÛËÚ‡ËÁ˙Ï. ÇÒ˘ÍË ËÁÒΉ‚‡ÌË Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú ‰ۈË‡Ì‡ ÍÓÒÚ̇ ÔÎ˙ÚÌÓÒÚ Â‰ÌÓ‚ÂÏÂÌÌÓ Ë ‚ ÍÓÏÔ‡ÍÚÌËÚÂ Ë ‚ Ú‡·ÂÍÛ·ÌËÚ ÍÓÒÚË Ë ÚÓ ‚˙‚ ‚˙Á‡ÒÚ, ÔË ÍÓflÚÓ Â Úfl·‚‡ÎÓ ‰‡ Ò ÔÓÒÚË„Ì ÔËÍÓ‚‡Ú‡ ÍÓÒÚ̇ χ҇. ë˙˘ÂÒÚ‚Û‚‡Ú ÌflÍÓÎÍÓ ‚˙ÁÏÓÊÌË Ó·flÒÌÂÌËfl, ͇ÚÓ ‰ÂÙˈËÚ Ì‡ ÔÓÎÓ‚ËÚ ıÓÏÓÌË Ë ‡ÒÚÂÊÌËfl ıÓÏÓÌ, ̇χÎÂÌ ı‡ÌËÚÂÎÂÌ ÔËÂÏ Ì‡ ͇ΈËÈ, Ó„‡Ì˘Â̇ ÔÓ‰‚ËÊÌÓÒÚ ÔÓ‡‰Ë ‡ÌÌÓ Ì‡ÒÚ˙ÔË· ËÌ‚‡ÎˉËÁ‡ˆËfl. ä‡ÎˆËflÚ, ‡Î͇Î̇ڇ ÙÓÒÙ‡Ú‡Á‡ Ë Ô‡‡‰ ıÓÏÓÌ˙Ú Ò‡ ‚ ÌÓχ, ‚˙ÔÂÍË ˜Â ÒÚÓÈÌÓÒÚËÚ ̇ ÒÂÛÏÌËfl ÙÓÒÙÓ Ò‡ ÎÂÍÓ ÔÓÌËÊÂÌË. èÓÛ˜‚‡ÌËflÚ‡ ̇ ÎËÚÂ‡ÚÛ‡Ú‡ Ë Ì‡ OMIM ‰‡ÌÌËÚ Ì ÛÒÔflı‡ ‰‡ ÛÒÚ‡ÌÓ‚flÚ Ô‰˯ÌË ÓÔËÒ‡ÌËfl ̇ ˉÂÌÚËÙˈË‡ÌËfl ÒË̉ÓÏ. ëË̉ÓÏ˙Ú Ì‡ å‡ËÌÂÒÍÓ-ë¸Ó„ÂÌ (åëë) Ò˙‰˙ʇ ÌflÍÓË, ÌÓ ÌÂ Ë ‚Ò˘ÍË ÔÓfl‚Ë Ì‡ ääãÑç – ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, ÍÓÌ„ÂÌËÚ‡ÎÌË Í‡Ú‡‡ÍÚË Ë ‰ÂÙˈËÚ Ì‡ ‡ÒÚÂʇ (13, 17). èË ääãÑç ÍÓÌ„ÂÌËÚ‡ÎÌËÚ ͇ڇ‡ÍÚË Ò‡ ‚Ë̇„Ë Ò‚˙Á‡ÌË Ò ÏËÍÓÍÓÌÂË. ãˈ‚ËflÚ ‰ËÁÏÓÙËÁ˙Ï Â Â‰Ì‡ ÓÚ ı‡‡ÍÚÂÌËÚ ÔÓfl‚Ë ÔË ääãÑç Ë ‚Ò Ӣ Ì  Ò˙Ó·˘ÂÌ ÔË åëë (15). ÇÒ˘ÍË Ô‡-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ˆËÂÌÚË Ò ääãÑç ËÏ‡Ú ıËÔÓÏËÂÎËÌËÁË‡˘‡ ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl, ÍÓflÚÓ Â fl‰Í‡ ÔÓfl‚‡ ̇ åëë (2, 8, 10, 23). èӂ˜ÂÚÓ ÒÎÛ˜‡Ë Ò åëë ËÏ‡Ú ÏËÓÔ‡ÚÌË ÔÓfl‚Ë, ÛÒÚ‡ÌÓ‚ÂÌÓ ÍÎËÌ˘ÌÓ, Ò ÖåÉ Ë ÏÛÒÍÛÎ̇ ·ËÓÔÒËfl, ͇ÍÚÓ Ë Ôӂ˯ÂÌË ÌË‚‡ ̇ Í‡ÚËÌÙÓÒÙÓÍË̇Á‡Ú‡ ÔË ÌflÍÓË ÓÚ Úflı (3, 6, 7, 11, 14, 16, 19, 20). èË Â‰ËÌ Ô‡ˆËÂÌÚ Ò ääãÑç ÎËÔÒ‚‡Ú ÏËÓÔ‡ÚÌË ÔÓÏÂÌË Ì‡ ÏÛÒÍÛÎ̇ ·ËÓÔÒËfl, ‡ Í‡ÚËÌÙÓÒÙÓÍË̇Á‡Ú‡  ‚ ÌÓχÎÌË ÒÚÓÈÌÓÒÚË ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË ‚ ̇¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ. ñÂ·Â·ÌËflÚ ÒË̉ÓÏ Â ‰Ó· ËÁ‡ÁÂ̇ ÔÓfl‚‡ ̇ åëë, ‰Ó͇ÚÓ ÔË ääãÑç Ò‡ÏÓ 12 Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú ÎÂ͇ ‡Ú‡ÍÒËfl ·ÂÁ ‰ËÁ‡ÚËfl Ë ‰ËÁÏÂÚËfl. ëÔ‡ÒÚˈËÚÂÚ Ì‡ Í‡ÈÌˈËÚÂ Ë ÍÎÓÌÛÒ Ì‡ ÒÚ˙ԇ·ڇ Ò Ò˙Ó·˘‡‚‡Ú ÔË ÏÌÓ„Ó åëë ÒÎÛ˜‡Ë Ë ÎËÔÒ‚‡Ú ÔË ääãÑç. ÇÒ ԇÍ, ÔË ääãÑç ÔÓÎÓÊËÚÂÎÂÌ ÂÙÎÂÍÒ Ì‡ Ň·ËÌÒÍË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 28/73 Ô‡ˆËÂÌÚË. ìÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡Ì  ̇ÎËˆÂ Ë ÔË ‰‚‡Ú‡ ÒË̉Óχ, ÌÓ ÔË ääãÑç  ÎÂÍÓ (IQ 55-70), ‰Ó͇ÚÓ ÔË åëë Ôӂ˜ÂÚÓ ÓÚ ÒÎÛ˜‡ËÚ ҇ Ò ÛÏÂÂ̇ ËÎË ÚÂÊ͇ ÒÚÂÔÂÌ Ì‡ ËÁÓÒÚ‡‚‡ÌÂ. ê‡ÒÚÂÊÌËflÚ ‰ÂÙˈËÚ Â Ì‡ÎËˆÂ Ë ÔË ‰‚‡Ú‡ ÒË̉Óχ ͇ÚÓ ÔË ääãÑç  ‚ÂÓflÚÌÓ Ò‚˙Á‡Ì Ò „Û·ÚÓÂÌ ‰ÂÙˈËÚ Ì‡ ‡ÒÚÂÊÌËfl ıÓÏÓÌ. èË ääãÑç Ò ÛÒÚ‡ÌÓ‚fl‚‡ ıËÔÓ„Ó̇‰ÓÚÓÔÂÌ ıËÔÓ„Ó̇‰ËÁ˙Ï, ‡ ÔË ÌflÍÓË ÒÎÛ˜‡Ë Ò åëë – ıËÔÂ„Ó̇‰ÓÚÓÔÂÌ (11, 18). Ç˙ÔÂÍË, ˜Â ˆÂ·Â·̇ڇ ‡ÚÓÙËfl ÔË åêí ËÁÒΉ‚‡Ì  ‰̇ ÓÚ Ì‡È-˜ÂÒÚËÚ ÔÓfl‚Ë åëë, Ò˙Ó·˘‡‚‡Ú Ò Ò˙˘Ó ڇ͇ Ë ‰ËÙÛÁ̇ ÏÓÁ˙˜Ì‡, ÒÚ‚ÓÎÓ‚‡ Ë „˙·Ì‡˜ÌÓ-ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl (4). èӂ˜ÂÚÓ ÓÚ ÒÎÛ˜‡ËÚÂ Ò ääãÑç ÔÓ͇Á‚‡Ú ÔÓ‰Ó·ÌË ÔÓÏÂÌË ‚ ‡Á΢̇ ÒÚÂÔÂÌ. Ç ÌflÍÓË ÓÚ ÒÎÛ˜‡ËÚ ÔÂӷ·‰‡‚‡ ÍÓÓ‚‡Ú‡ ‡ÚÓÙËfl, ‰Ó͇ÚÓ ÔË ‰Û„Ë – ÒÚ‚ÓÎÓ‚‡Ú‡ ËÎË „˙·Ì‡˜ÌÓ-ÏÓÁ˙˜Ì‡Ú‡. ÅÓÎÂÒÚÚ‡ Á‡Òfl„‡ ÔÂӷ·‰‡‚‡˘Ó ÓÏÒÍË ÒÛ·„ÛÔË Ë ÏÂÚ‡„ÛÔË, ÔË̇‰ÎÂʇ˘Ë ̇ Ç·¯Í‡Ú‡ „ÛÔ‡ ˆË„‡ÌË, ‡ ËÏÂÌÌÓ ÚÂÁË Ì‡ ÍÓԇ̇ËÚÂ. åËÚÓıÓ̉ˇÎÌÓÚÓ Ñçä ÒÂÍ‚ÂÌË‡ÌÂ Ë ‡Ì‡ÎËÁ‡ ̇ Y ıÓÏÓÁÓχڇ ÔÓ͇Á‡ „ÂÌÂÚ˘̇ ıÓÏÓ„ÂÌÌÓÒÚ ‚ „ÛÔ‡Ú‡ (5). íÓ‚‡ ‰‡‰Â ÓÒÌÓ‚‡ÌË ‰‡ Ò Ә‡Í‚‡, ˜Â Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë ËÏ‡Ú Â‰Ì‡ Ë Ò˙˘‡ ÏÛÚ‡ˆËfl ̇ Ô‡Ó‰ËÚÂÎfl. à‰ÂÌÚËÙˈË‡Ì  „ÂÌÂÚ˘ÌËflÚ ‰ÂÙÂÍÚ, ‚Ӊ¢ ‰Ó ˜‡ÒÚ˘ÂÌ ‰ÂÙˈËÚ Ì‡ ë-ÚÂÏË̇ÎÌËfl ‰ÓÏÂÌ Ì‡ ÙÓÒÙ‡Ú‡Á‡Ú‡ ̇ êçä ÔÓÎËÏÂ‡Á‡ ¨¨. ᇷÓÎfl‚‡ÌÂÚÓ Ò ÓÚ̇Òfl Í˙Ï „ÛÔ‡Ú‡ ̇ “Ú‡ÌÒÍË·ˆËÓÌÌËÚ ÒË̉ÓÏË” Ë Â Ô˙‚Ëfl “˜ËÒÚ” ‰ÂÙÂÍÚ Ì‡ ÔÓÎËÏÂ‡Á‡ ¨¨ –ωËË‡Ì‡Ú‡ Ú‡ÌÒÍË·ˆËfl. Ç Ú‡ÁË „ÛÔ‡ Á‡·ÓÎfl‚‡ÌËfl Ò ̇ÏË‡ Ë åëë, ͇ÚË‡Ì ̇ÒÍÓÓ ‚˙ıÛ 5q32 „ËÓÌ (12), Ò˙‰˙ʇ˘ ÌflÍÓÎÍÓ „ÂÌË, ÍÓ‰Ë‡˘Ë Ú‡ÌÒÍË·ˆËÓÌÌË Ù‡ÍÚÓË. å‡Í‡ ˜Â ääãÑç ÒË̉ÓÏ Ë åëë ËÏ‡Ú ÌflÍÓË Ó·˘Ë ÔËÁ̇ˆË, ͇ÚÓ Ì‡Û¯ÂÌÓ ÙËÁË͇ÎÌÓ Ë ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ‡Á‚ËÚËÂ Ë ËÌÙÂÚËÎËÚÂÚ, Ú ËÏ‡Ú Ë Ú˙͇ÌÌÓ-ÒÔˆËÙ˘ÌË ÔÓfl‚Ë, ÍÓËÚÓ ˘Â ÒÔÓÏÓ„Ì‡Ú Á‡ ÔÓ-‰Ó·ÓÚÓ ‡Á·Ë‡Ì ̇ Ú‡ÌÒÍË·ˆËÓÌ̇ڇ „Û·ˆËfl. ãàíÖêÄíìêÄ 1. ëÎ˙̘‚ è. îËÁ˘ÂÒÍÓ ‡Á‚ËÚË ̇ ·˙΄‡Ò͇ڇ ÔÓÔÛ·ˆËfl. ëÓÙËfl: Å˙΄‡Ò͇ ÒÔÓÚ̇ ‡ÒӈˇˆËfl 1992, 34-86. 2. Alexianu M., Christodorescu D., Vasilescu C. et al.: Sensorimotor neuropa-

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thy in a patient with Marinesco-Sjogren syndrome. European Neurology 1983, 22, 222-226. 3. Borud O., Aasly J., Lindal S.: Mitochondrial myopathy in six patients wirh Marinesco-Sjogren syndrome. Progress in Clinical and Biological Research 1989, 306, 135-139. 4. Bromberg M., Junck L., Gebarski S. et al.: The Marinesco-Sjogren syndrome examined by computed tomography, magnetic resonance, and 18F-2-fluoro-2deoxy-d-glucose and positron emission tomography. Arch. Neurol.1990, 47, 12391242. 5. Chaix R, Austerlitz F, Morar B, Kalaydjieva L, Heyer E. Vlax Roma history: what do coalescent-based methods tell us? Eur J Hum Genet. 2004, 12, 285-92. 6. Chako J.: Marinesco-Sjogren syndrome with myopathy. Confinia Neurologica 1969, 31, 349-351. 7. Goto Y., Komiyama A., Tanabe Y. et al.: Myopathy in Marinesco-Sjogren syndrome: an ultrastructural study. Acta Neuropathologica 1990, 80, 123-128. 8. Hakamada S., Sobue G., Watanabe K. et al.: Peripheral neuropathy in Marinesco-Sjogren syndrome. Brain and Development 1981, 3, 403-406. 9. Jacobs JM, Harding BN, Lake BD, Payan J, Wilson J. Peripheral neuropathy in Leigh's disease. Brain. 1990, 113, 447-62. 10. Katafuchi Y., Kosai K., Ohtaki E. et al.: Cerebral cortex and brainstem involvement in Marinesco-Sjogren syndrome. Annals of Neurology 1990, 27, 448-449. 11. Komiyama A., Nonaka I., Hirayama K.: Muscle pathology in MarinescoSjogren syndrome. J. Neurol. Sci.1989, 89, 103-113. 12. Lagier-Tourenne C, Tranebaerg L, Chaigne D, Gribaa M, Dollfus H, Silvestri G, Betard C, Warter JM, Koenig M. Homozygosity mapping of Marinesco-Sjogren syndrome to 5q31. Eur J Hum Genet. 2003, 11, 770-8. 13. Marinescu G., Draganescu S., Vasiliu D.: Nouvelle maladie familiale caracterisee par une cataracte congenitale et un arret du development somato-neuro-psychique. Encephale 1931, 26, 97-109. 14. Marks H., Kelleher F., DeLeon G. et al.: Muscle mitochondrial abnormalitie in the Marinesko-Sjogren syndrome. Annals of Neurology 1980, 8, 122 (abstact). 15.McLaughlin J., Pagon R., Weinberg E. et al.: Developmental Medicine and Child Neurology 1996, 38, 632-644. 16. Sewry C., Voit T., Dubowitz V.: Myopathy with unique ultrastructural feature in Marinesco-Sjogren syndrome. Annals of Neurology 1988, 24, 576-578. 17. Sjogren T.: Hereditary congenital spinocerebellar atxia accompanied by congenital cataract and oligophrenia. Confin. Neurol.1950, 10, 293 -308. 18. Skre H., Berg K.: Linkage studies on the Marinesco-Sjogren syndrome and hypergonadotropic hypogonadism. Clin. Genet. 1977, 11, 57-66. 19. Superneau D., Wertelecki W., Zellweger H. et al.: Myopathy in MarinescoSjogren syndrome. Europ. Neurol.1987, 26, 8-16. 20. Torbergsen T., Aasly J., Borud A. et al.: Mitochondrial myopathy in Marinesco-Sjogren syndrome. Journal of Mental Deficiency Research 1991, 35, 154-159. 21. Tournev I., L. Kalaydjieva, B.Youl, B. Ishpekova, V. Guergueltcheva, O. Kamenov, M. Katzarova, Z. Kamenov, M. Raicheva-Terzieva, R.H.M. King, K. Romanski, R. Petkov, A. Schmarov, G.Dimitrova, N.Popova, M.Uzunova, S.Milanov, J. Petrova, I.Petkov, G.Kolarov, L.Aneva, O.Radeva & P.K.Thomas. The Congenital Cataracts Facial Dysmorphism Neuropathy (CCFDN) syndrome: a novel complex genetic disease in Balkan Gypsies. Annals of Neurology, 1999, 45, 742-750. 22. Varon R, Gooding R, Steglich C, Marns L, Tang H, Angelicheva D, Yong KK, Ambrugger P, Reinhold A, Morar B, Baas F, Kwa M, Tournev I, Guerguelcheva V, Kremensky I, Lochmuller H, Mullner-Eidenbock A, Merlini L, Neumann L, Burger J, Walter M, Swoboda K, Thomas PK, von Moers A, Risch N, Kalaydjieva L. Partial deficiency of the C-terminal-domain phosphatase of RNA polymerase II is associated with congenital cataracts facial dysmorphism neuropathy syndrome. Nat Genet. 2003, 35, 185-9. 23. Zimmer C., Gostonyi G., Cervos-Navarro J. et al.: Neuropathy with lysosomal changes in Marinesco-Sjogren syndrome: fine structural findings in skeletal muscle and conjunctiva. Neuropediatrics 1992, 23, 329-335.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÑÓˆ. à‚‡ÈÎÓ í˙Ì‚ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡ “ÄÎÂÍ҇̉Ó‚Ò͇” ÅÛÎ. “ë‚. É. ëÓÙËÈÒÍË” ‹1, ëÓÙËfl 1431 íÂÎ. (02) 9230 752 E-mail: emhpf@techno-link.com


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

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éË„Ë̇ÎÌË ÒÚ‡ÚËË äãàçàäé-ÉÖçÖíàóçé èêéìóÇÄçÖ çÄ ïÖêÖÑàíÄêçÄíÄ åéíéêçÄ à ëÖçáéêçÄ çÖÇêéèÄíàü íàè ãéå (ïåëçã; åIå 601455) Ç ÅöãÉÄêàü

à.í˙Ì‚1,3, Å. à¯ÔÂÍÓ‚‡1, ê. äËÌ„2, Ç. ÉÂ„ÂΘ‚‡1,3, û. èÂÚÓ‚‡1, Ä. òχÓ‚1, Ä. çËÍÓÎÓ‚‡1, Ä. 뇂ӂ1, å. 䇈‡Ó‚‡1,3, Ç. ÅÓÊËÌÓ‚‡1, à. ãËÚ‚ËÌÂÌÍÓ1, ï. ñÂÍÓ‚1, à. èÂÚÍÓ‚‡1, Ä.ïËÒÚÓ‚‡1, ç. èÓÔÓ‚‡1, ã. ÄÌ‚‡3,4, Ñ. ÄÌ„Â΢‚‡5, Ä. âÓ‰‡ÌÓ‚‡1, à. äÂÏÂÌÒÍË1, è.ä. íÓχÒ2, ã. ä‡Î‡È‰ÊË‚‡1,5 1å‰ˈËÌÒÍË

ÛÌË‚ÂÒËÚÂÚ – ëÓÙËfl; ÔÓ ÍÎËÌ˘ÌË Ì‚Ó̇ÛÍË, ìÌË‚ÂÒËÚÂÚ Ì‡ ãÓ̉ÓÌ, ÇÂÎËÍÓ·ËÚ‡ÌËfl; 3îÓ̉‡ˆËfl “á‰‡‚ÌË ÔÓ·ÎÂÏË Ì‡ χΈËÌÒÚ‚‡Ú‡”- ëÓÙËfl; 4åÅÄã – Å·„Ó‚„‡‰; 5ㇷÓ‡ÚÓËfl ÔÓ ÏÓÎÂÍÛÎfl̇ „ÂÌÂÚË͇ ̇ ˜Ó‚Â͇, á‡Ô‡‰ÌÓ-Ä‚ÒÚ‡ÎËÈÒÍË ËÌÒÚËÚÛÚ Á‡ ωˈËÌÒÍË ËÁÒΉ‚‡ÌËfl, ìÌË‚ÂÒËÚÂÚ Ì‡ á‡Ô‡‰Ì‡ Ä‚ÒÚ‡ÎËfl, è˙Ú, Ä‚ÒÚ‡ÎËfl 2ä‡Ú‰‡

SUMMARY CLINICAL AND GENETIC INVESTIGATION OF HEREDITARY MéTOR AND SENSORY NEUROPATHY TYPE LOM (HMSNL; MIM 601455) IN BULGARIA I. Tournev, B. Ishpekova, R. King, V. Guergueltcheva, J. Petrova, A. Shmarov, A. Nikolova, A. Savov, M. Katzarova, V. Bojinova, I. Litvinenko, H. Tzekov, I. Petkova, A. Hristova, N. Popova, L. Aneva, D. Angelicheva, A. Jordanova, I. Kremenski, P.K. Thomas & L. Kalaydjieva A new autosomal-recessive neuropathy with deafness was identified for the first time by M. Abadjiev and I. Georgiev in the 70-ies among the Roma community in the town of Lom. B. Ishpekova examined Roma families with the disease originating from Pleven and Gabrovo regions. In 1993 Luba Kalaydjieva initiated the clinical and genetic investigation of families with Hereditary motor and sensory neuropathy type Lom (HMSNL), identified by V. Georgieva and B. Ishpekova. Kalaydjieva et al. (1996) mapped the genetic mutation responsible for the disease on 8q24. The following refined genetic mapping led to the identification of the “private” for the Roma people mutation R148X in exon 7 of the NDRG1 gene (N-myc-downstream-regulated gene 1). Tournev et al. (1994-2004) performed an extensive clinical and epidemiological investigation of the disease in 1300 towns and villages in the country. Phenotype characterization was performed of 110 HMSNL cases from 39 pedigrees. The onset of the disease is in the first decade of life when distal muscle weakness in the legs and gait disturbance appear. The distal muscles of the hands are affected in the second decade. Sensory-neural deafness is established in the third decade. Bulbar muscle weakness appears later in life along with the progression of the disease. HMSNL is the most frequent autosomal-recessive peripheral neuropathy among the Roma people in Bulgaria and is established in almost all Roma groups in 41 towns and villages in the country. Key words: hereditary motor and sensory neuropathy type Lom. êÖáûåÖ Ä‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ Ì‚ÓÔ‡ÚËfl, ÔˉÛÊÂ̇ Ò „ÎÛıÓÚ‡  ˉÂÌÚËÙˈË‡Ì‡ Á‡ Ô˙‚Ë Ô˙Ú ÔÂÁ 70-Ú „Ó‰ËÌË ÓÚ å. Ä·‡‰ÊË‚ Ë à. ÉÂÓ„Ë‚ Ò‰ ÓÏÒ͇ڇ Ó·˘ÌÓÒÚ ‚ „‡‰ ãÓÏ. Å. à¯ÔÂÍÓ‚‡ ËÁÒΉ‚‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò‰ ÓÏÒÍË Ù‡ÏËÎËË ÓÚ è΂ÂÌÒÍË Ë É‡·Ó‚ÒÍË „ËÓÌË. ã. ä‡Î‡È‰ÊË‚‡ (1993) ËÌˈËË‡ ÍÎËÌËÍÓ-„ÂÌÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ̇ Ù‡ÏËÎËË Ò ïÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ ãÓÏ (ïåëçã), ÔÓ‰‡‰ÂÌË ÓÚ Ç. ÉÂÓ„Ë‚‡ Ë Å. à¯ÔÂÍÓ‚‡.

ä‡Î‡È‰ÊË‚‡ Ë Ò˙Ú. (1996) ͇ÚË‡Ú „ÂÌ̇ڇ ÏÛÚ‡ˆËfl, ÓÚ„Ó‚Ó̇ Á‡ ·ÓÎÂÒÚÚ‡ ‚˙ıÛ 8q24. èÓÒΉ‚‡˘ËÚ ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl ‰Ó‚Âʉ‡Ú ‰Ó ˉÂÌÚËÙˈË‡Ì ̇ “˜‡ÒÚ̇ڇ” Á‡ ÓÏËÚ ÏÛÚ‡ˆËfl R148X ‚ ÂÍÁÓÌ 7 ̇ NDRG1 (N-myc downstream-regulated gene 1) „Â̇. í˙Ì‚ Ë Ò˙Ú. (1994 – 2004) ÔÓ‚Âʉ‡Ú χ˘‡·ÌË ÍÎËÌËÍÓ-ÂÔˉÂÏËÓÎӄ˘ÌË ÔÓÛ˜‚‡ÌËfl Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‚ 1300 ̇ÒÂÎÂÌË ÏÂÒÚ‡ ‚ ˆfl·ڇ ÒÚ‡Ì‡. èӂ‰ÂÌÓ Â ÙÂÌÓÚËÔËÁË‡Ì ̇ 110 ÒÎÛ˜‡Ë Ò ïåëçã ÓÚ 39 Ó‰ÓÒÎÓ‚Ëfl. 燘‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÔÂÁ Ô˙‚ÓÚÓ ‰ÂÒÂÚËÎÂÚË ÓÚ ÊË‚ÓÚ‡ Ò ÔÓfl‚‡ ̇ ‰ËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‚ Í‡Í‡Ú‡ Ë Ì‡Û¯ÂÌË ‚ ÔÓıӉ͇ڇ. èÂÁ ‚ÚÓÓÚÓ ‰ÂÒÂÚËÎÂÚË Ò Á‡Òfl„‡Ú Ë ‰ËÒÚ‡ÎÌËÚ ÏÛÒÍÛÎË Ì‡ ˙ˆÂÚÂ. á‚ÛÍÓÔËÂÏÌÓ Ì‡Û¯ÂÌË ̇ ÒÎÛı‡ ‚˙ÁÌËÍ‚‡ ̇È-˜ÂÒÚÓ ÔÂÁ ÚÂÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ. ÅÛη‡̇ ÒËÏÔÚÓχÚË͇ Ò ÔÓfl‚fl‚‡ ‚ ÔÓ-Í˙Ò̇ ‚˙Á‡ÒÚ ÔË Ì‡Ô‰‚‡Ì ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ïåëçã  ̇È-˜ÂÒÚ‡Ú‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl Ò‰ ÓÏËÚ ‚ Å˙΄‡Ëfl Ë Â ÛÒÚ‡ÌÓ‚Â̇ Ò‰ ÔÓ˜ÚË ‚Ò˘ÍË ÓÏÒÍË „ÛÔË ‚ 41 ̇ÒÂÎÂÌË ÏÂÒÚ‡ ‚ ÒÚ‡Ì‡Ú‡. äβ˜Ó‚Ë ‰ÛÏË: ıÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ ãÓÏ. ÇöÇÖÑÖçàÖ á‡·ÓÎfl‚‡ÌÂÚÓ Â Ë‰ÂÌÚËÙˈË‡ÌÓ Á‡ Ô˙‚Ë Ô˙Ú ÔÂÁ 70-Ú ÓÚ å. Ä·‡‰ÊË‚ Ë à. ÉÂÓ„Ë‚ Ò‰ ÓÏÒ͇ڇ Ó·˘ÌÓÒÚ ‚ „‡‰ ãÓÏ. Ä·‡‰ÊË‚ Ë ÉÂÓ„Ë‚ (1) Ô˙‚Ë ÔÓ‚Âʉ‡Ú „Â̇Îӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ Ë ÛÚÓ˜Ìfl‚‡Ú ÚËÔ‡ ̇ Û̇ÒΉfl‚‡ÌÂ. Ä·‡‰ÊË‚ å. Ë Ò˙Ú. (1) Ò˙Ó·˘‡‚‡Ú, ˜Â  ̇Îˈ fl‰ÍÓÚÓ Ò˙˜ÂÚ‡ÌË ̇ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ Ì‚‡Î̇ ÏÛÒÍÛÎ̇ ‡ÚÓÙËfl Ò Ì‡Ô‰‚‡˘‡ ‰‚ÛÒÚ‡Ì̇ „ÎÛıÓÚ‡ ÔË 22 ·ÓÎÌË ÓÚ Â‰ÌÓ Ó‰ÓÒÎÓ‚ËÂ. Å. à¯ÔÂÍÓ‚‡ (2) ËÁÒΉ‚‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò‰ ÓÏÒÍË Ù‡ÏËÎËË ÓÚ è΂ÂÌÒÍË Ë É‡·Ó‚ÒÍË „ËÓÌË, Í·ÒËÙˈË‡ÈÍË „Ë Í‡ÚÓ ïåëç ¨¨¨ ÚËÔ. ã. ä‡Î‡È‰ÊË‚‡ (1993) ËÌˈËË‡ ÍÎËÌËÍÓ-„ÂÌÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ̇ Ù‡ÏËÎËË Ò ïåëçã, ÔÓ‰‡‰ÂÌË ÓÚ Ç. ÉÂÓ„Ë‚‡ Ë Å. à¯ÔÂÍÓ‚‡. Ç ÔÂËÓ‰‡ 1993-1997 „. ÍÎËÌ˘ÂÌ ÂÍËÔ Ò Û˜‡ÒÚËÂÚÓ Ì‡ à¯ÔÂÍÓ‚‡, çËÍÓÎÓ‚‡, í˙Ì‚, èÂÚÓ‚‡ Ë òχÓ‚ ÔÓ‚Âʉ‡Ú ÍÎËÌËÍÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì ̇ 35 Á‡Ò„̇ÚË ËÌ‰Ë‚Ë‰Ë ÓÚ ËÁ‚ÂÒÚÌËÚ ‰Ó ÚÓÁË ÏÓÏÂÌÚ Ù‡ÏËÎËË. èÂÁ ÓÍÚÓÏ‚Ë 1997 ‚ Å˙΄‡Ëfl „ÓÒÚÛ‚‡Ú ÔÓÙ. íÓÏ‡Ò Ë ‰- âÓÎ, ÍÓËÚÓ Ò˙˘Ó ËÁÒΉ‚‡Ú ·ÓÎÌË Ò ïåëçã. èÂÁ ÔÂËÓ‰‡ 1997-2004 „. í˙Ì‚ ÔÓ‰˙Îʇ‚‡ Á‡ÔӘ̇ÚÓÚÓ ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡ÌÂ, ‡ Ò˙˘Ó ÔÓ‚Âʉ‡ ÂÔˉÂÏËÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ‚ Å˙΄‡Ëfl. à‰ÂÌÚËÙˈË‡ÌË Ò‡ Ó·˘Ó 110 ·ÓÎÌË ‚ 41 ̇ÒÂÎÂÌË ÏÂÒÚ‡. äÎËÌ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ̇ Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Â ÔÓ‚Âʉ‡ÌÓ ÓÒÌÓ‚ÌÓ ‚ è˙‚‡ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇ ̇ ìå-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, Í˙‰ÂÚÓ Â‰Ì‡ „ÓÎflχ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚ ҇ ıÓÒÔËÚ‡ÎËÁË‡ÌË, ‡ ‰Û„Ë Ò‡ ËÁÒΉ‚‡ÌË ‡Ï·Û·ÚÓÌÓ. 臈ËÂÌÚËÚ ‚ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ Ò‡ ıÓÒÔËÚ‡ÎËÁË‡ÌË Ë ËÁÒΉ‚‡ÌË ‚ ÑÂÚÒ͇ڇ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇ ̇ ìëÅÄãçè “ë‚. ç‡ÛÏ” – IV ÍÏ. ÖÎÂÍÚÓÙËÁËÓÎӄ˘ÌËÚ ËÁÒΉ‚‡ÌËfl Ò‡ ÔÓ‚Âʉ‡ÌË ËÁÍβ˜ËÚÂÎÌÓ ÓÚ ÔÓÙ. à¯ÔÂÍÓ‚‡ ‚ ㇷÓ‡ÚÓËflÚ‡ ÔÓ ÂÎÂÍÚÓÏËÓ„‡ÙËfl, ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇”, ÌÓ Ò˙˘Ó Ë ‚ ÌflÍÓË ÖåÉ Í‡·ËÌÂÚË Ì‡ Ù‡ÍÛÎÚÂÚÒÍË ·ÓÎÌËˆË ‚ ÔÓ‚Ë̈ËflÚ‡. ïËÒÚÓÎӄ˘ÌËÚ ËÁÒΉ‚‡ÌËfl Ò‡ ÔÓ‚Âʉ‡ÌË ÓÚ äËÌ„ Ë è.K. íÓÏ‡Ò ‚ ä‡Ú‰‡Ú‡ ÔÓ ÍÎËÌ˘ÌË Ì‚Ó̇ÛÍË Ì‡ ìÌË‚ÂÒËÚÂÚ‡ ‚ ãÓ̉ÓÌ. åÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌËÚ ËÁÒΉ‚‡ÌËfl Ò‡ ÔÓ‚Âʉ‡ÌË ÓÚ ÂÍËÔ‡ ̇ ã. ä‡Î‡È‰ÊË‚‡ ‚ ñÂÌÚ˙‡ ÔÓ „ÂÌÂÚË͇ ̇ ˜Ó‚Â͇, Ö‰ËÚ äÓÛ˙Ì ìÌË‚ÂÒËÚÂÚ‡, á‡Ô‡‰Ì‡ Ä‚ÒÚ‡ÎËfl Ë ‚ ㇷÓ‡ÚÓËflÚ‡ ÔÓ ÏÓÎÂÍÛÎfl̇ Ô‡ÚÓÎÓ„Ëfl, ìëÅÄãÄÉ “å‡È˜ËÌ ‰ÓÏ”. èÓ-Í˙ÒÌÓ Á‡·ÓÎfl‚‡ÌÂÚÓ Â Ë‰ÂÌÚËÙˈË‡ÌÓ Ò‰ ÓÏËÚÂ Ë ‚ ‰ˈ‡ ‰Û„Ë Â‚ÓÔÂÈÒÍË ‰˙ʇ‚Ë: àÚ‡ÎËfl (16), ëÎÓ‚ÂÌËfl (5), ÉÂχÌËfl (3), àÒÔ‡ÌËfl (6), î‡ÌˆËfl Ë êÛÏ˙ÌËfl (ä‡Î‡È‰ÊË‚‡ ã., ÌÂÔÛ·ÎËÍÛ‚‡ÌË ‰‡ÌÌË). åÄíÖêàÄã à åÖíéÑà à‰ÂÌÚËÙˈË‡ÌË Ò‡ 110 Ô‡ˆËÂÌÚË ÓÚ 39 Ù‡ÏËÎËË Ò ïåëçã – 53 Ï˙ÊÂ Ë 57 ÊÂÌË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â ÔÓ‚Âʉ‡ÌÓ ÔÓ‰Ó·ÌÓ ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡ÌÂ, ‚Íβ˜‚‡˘Ó: ‡/ ‡Ì‡ÎËÁ ̇ ‡Ì‡ÏÌÂÒÚ˘ÌËÚ ‰‡ÌÌË Í‡ÚÓ Ò‡ ÓÚ˜ËÚ‡ÌË Ù‡ÍÚÓËÚÂ: ‚˙Á‡ÒÚ ÔÓ ‚ÂÏ ̇ ËÁÒΉ‚‡ÌÂÚÓ; ̇˜‡Î̇ ‚˙Á‡ÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ; ̇˜‡ÎÌË ÒËÏÔÚÓÏË; ‚ÓβˆËfl ‰Ó Ô˙ÎÌÓ ‡Á‚ËÚË ̇ ÒËÏÔÚÓχÚË͇ڇ; ‚˙Á‡ÒÚ Ì‡ ËÌ‚‡ÎˉËÁ‡ˆËfl Ë ÒÏ˙ÚÌÓÒÚ; ·/ ÔÓ‰Ó·ÌÓ ËÁÒΉ‚‡Ì ̇ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ; ‚/ ÓÙÚ‡ÎÏÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ; „/ ÓÚÓÌ‚ÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ; ‰/ ËÁÒΉ‚‡Ì ̇ Ó·ÓÌflÌËÂÚÓ Ë ‚ÍÛÒ‡ ˜ÂÁ ÍÓ΢ÂÒÚ‚ÂÌË Ë Í‡˜ÂÒÚ‚ÂÌË ÚÂÒÚÓ‚Â ‡Á‡·ÓÚÂÌË ‚ äÎËÌË͇ڇ Á‡ ËÁÒΉ‚‡Ì ̇ Ó·ÓÌflÌËÂ Ë ‚ÍÛÒ Ì‡ ïÂÏÓÒÂÌÁÓÌËfl ÍÎËÌËÍÓËÁÒΉӂ‡ÚÂÎÒÍË ˆÂÌÚ˙, ä˙ÌÂÍÚËÍ˙Ú (CCCRC); Â/ ËÁÒΉ‚‡Ì ̇ ÓÚÓÔ‰˘ÂÌ ÒÚ‡ÚÛÒ. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ ÔÓ‚Âʉ‡ÌË Ì‚ÓÙËÁËÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl Í·Ò˘ÂÒ͇ Ë„ÎÂ̇ ÂÎÂÍÚÓÏËÓ„‡ÙËfl, ͇ÍÚÓ Ë ÒÚËÏÛ·ˆËÓÌ̇ ÂÎÂÍÚÓÏËÓ„‡ÙËfl Ë ÂÎÂÍÚÓÌ‚Ó„‡ÙËfl. àÁÒΉ‚‡ÌËflÚ‡ Ò‡ ËÁ‚˙¯ÂÌË Ò ‡Ô‡‡Ú “Phasis II” ̇ ÙËχڇ Esaote Biomedica (Florence, Italy) ‚ ··Ó‡ÚÓËËÚ ̇ ëÂ͈ËflÚ‡ ÔÓ äÎËÌ˘̇ Ì‚ÓÙËÁËÓÎÓ„Ëfl Í˙Ï ä‡Ú‰‡Ú‡ ÔÓ ç‚ÓÎÓ„Ëfl ̇ åì – ëÓÙËfl ËÎË Ò ÔÓÚ‡ÚË‚ÂÌ ÂÎÂÍÚÓÏËÓ„‡Ù Medelec Saphire (Medelec, Wolking, U.K.) ÔÓ ‰ÓÏÓ‚ÂÚ ̇ ·ÓÎÌËÚÂ. èË 38 Ô‡ˆËÂÌÚË Ò‡ ÔÓ‚Âʉ‡ÌË Ë ËÁÒΉ‚‡ÌËfl ̇ ÏÓÁ˙˜ÌÓ-ÒÚ‚ÓÎÓ‚Ë ÒÎÛıÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË. àÁÔÓÎÁ‚‡ÌË Ò‡ ÍÎËÍ ÒÚËÏÛÎË Ì‡ 80 dB ÔË ˜ÂÒÚÓÚ‡ ̇ ÙËÎÚËÚ 200–2000 Hz Ë ÛÒË΂‡Ì 5 ÏÍ‚ ̇ ‰ÂÎÂÌËÂ. èË 5 Ô‡ˆËÂÌÚË Ò‡ Ôӂ‰ÂÌË ıËÒÚÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl ̇ ÒÛ‡ÎÌË ÌÂ‚Ë. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Ôӂ‰ÂÌÓ ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì Á‡ ̇΢ËÂÚÓ Ì‡ ÏÛÚ‡ˆËflÚ‡ R148X, ÓÚ„Ó‚Ó̇ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÄçÄåçÖëíàóçà ÑÄççà ÅÂÏÂÌÌÓÒÚÚ‡ Ë ‡Ê‰‡ÌÂÚÓ Ò‡ ÌÓχÎÌË ÔË ‚Ò˘ÍË Á‡Ò„̇ÚË Ô‡ˆËÂÌÚË. 燘‡ÎÌËflÚ ÒËÏÔÚÓÏ ÔË ‚Ò˘ÍË Â Ì‡Û¯‡‚‡Ì ̇ ÔÓıӉ͇ڇ. 94 Ô‡ˆËÂÌÚË Ò‡ ÔÓıÓ‰ËÎË Ì‡ ‚˙Á‡ÒÚ ÏÂÊ‰Û 12 Ë 15 ÏÂÒˆ‡, 12 - ̇ 18 ÏÂÒˆ‡ Ë ˜ÂÚËËχ ̇ 24 ÏÂÒˆ‡. ç‡Û¯ÂÌË ‚ ÔÓıӉ͇ڇ Ò ÔÓfl‚fl‚‡ ̇ ‚˙Á‡ÒÚ ÏÂÊ‰Û 5 Ë 10 „., Ò‰ÌÓ 5.8 ± 1.12 „. ÉÓÌËÚ Í‡ÈÌËˆË Ò Á‡Òfl„‡Ú ÔÓ-Í˙ÒÌÓ, ÏÂÊ‰Û 5 Ë 15 „., Ò‰ÌÓ 12.9 ± 1.41 „. éÔ·͂‡ÌËfl ÓÚ Ì‡Û¯ÂÌËfl ̇ ÒÎÛı‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË 89 Ô‡ˆËÂÌÚË, ÔÓfl‚fl‚‡˘Ë Ò ÏÂÊ‰Û 13 Ë 26 „., Ò‰ÌÓ 21.8 ± 1.9 „. 21 Ô‡ˆËÂÌÚË ·ÂÁ ÒÎÛıÓ‚Ë Ì‡Û¯ÂÌËfl

149

Ò‡ ̇ ‚˙Á‡ÒÚ ÓÚ 5 ‰Ó 48 „., Ò‰ÌÓ 23.4 ± 1.95 „. èÂÁ ÔÂÚ‡Ú‡ Ë ¯ÂÒÚ‡Ú‡ ‰Â͇‰‡ ÓÚ ÊË‚ÓÚ‡ ̇ Ô‡ˆËÂÌÚËÚ Ò ÔÓfl‚fl‚‡Ú Ë ÓÔ·͂‡ÌËfl ÓÚ Ì‡Û¯ÂÌ „Ó‚Ó, „˙ÎÚ‡ÌÂ Ë ÙÓ̇ˆËfl. çÖÇêéãéÉà˜çé àáëãÖÑÇÄçÖ àÁÒΉ‚‡ÌÂÚÓ Ì‡ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ ÔÓ͇Á‡, ˜Â Ô˙‚ËÚ ¯ÂÒÚ ˜ÂÂÔÌÓ-ÏÓÁ˙˜ÌË ÌÂ‚Ë Ì ҇ Á‡Ò„̇ÚË ÔË ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚÂ. ç‡È-‡ÌÓ, ÔÂÁ ‚ÚÓ‡Ú‡ ËÎË ÚÂÚ‡Ú‡ ‰Â͇‰‡ ̇ ÊË‚ÓÚ‡ Ò Á‡Òfl„‡ ÒÎÛıÓ‚Ó-‡‚ÌÓ‚ÂÒÌËflÚ ÌÂ‚. á‡Òfl„‡Ì ̇ ÔÓ‰ÂÁ˘ÌËfl ÌÂ‚ Ò Ì‡Î˘Ë ̇ ‡ÚÓÙËfl ̇ ÂÁË͇ Ò ÓÚÍË‚‡ ÔË 9 ·ÓÎÌË ‚ ÚÂÚ‡Ú‡ ‰Â͇‰‡. èÓ-Í˙ÒÌÓ Ò ÔÓfl‚fl‚‡ Ë Îˈ‚‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ. ê‡Á„˙Ì‡Ú ·Ûη‡ÂÌ ÒË̉ÓÏ Ò ËÁ‡ÁÂ̇ ‰ËÒÙ‡„Ëfl, ‰ËÒÙÓÌËfl Ë ‰ËÁ‡ÚËfl ‚˙ÁÌËÍ‚‡ ÔË Ôӂ˜ÂÚÓ Ô‡ˆËÂÌÚË ‚ ÔÂÚ‡Ú‡ Ë ¯ÂÒÚ‡Ú‡ ‰Â͇‰‡. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÚÂÊ͇ ‰ËÒÚ‡Î̇ ÒËÏÂÚ˘̇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Ò ËÁÍβ˜ÂÌË ̇ ÔÂÚËχڇ ̇È-Ï·‰Ë Ô‡ˆËÂÌÚË. èÓ-ÎÂ͇ ‰ËÒÚ‡Î̇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl  ̇Îˈ ‚ „ÓÌËÚ Í‡ÈÌˈË. 燷≇‚‡ Ò Ô˙Î̇ ‡ÂÙÎÂÍÒËfl ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ËÁÍβ˜ÂÌË ̇ ÚËχڇ ̇È-Ï·‰Ë, ÔË ÍÓËÚÓ ÎËÔÒ‚‡Ú ÄıËÎÓ‚ËÚ ÂÙÎÂÍÒË, ÌÓ Ò‡ Á‡Ô‡ÁÂÌË ÍÓÎÂÌÌËÚ ÂÙÎÂÍÒË. ëÛıÓÊËÎÌËÚÂ Ë Ì‡‰ÍÓÒÚÌË ÂÙÎÂÍÒË ‚ „ÓÌËÚ Í‡ÈÌËˆË Ò‡ ÎÂÍÓ ÔÓÌËÊÂÌË ÔË 14 Ô‡ˆËÂÌÚË, ‡ ÔË ÓÒڇ̇ÎËÚ ҇ ÒËÎÌÓ ÔÓ‰ÚËÒ̇ÚË ËÎË ÎËÔÒ‚‡Ú. ëÂÚË‚ÌÓÒÚÚ‡  ̇Û¯Â̇ ÓÚ ‰ËÒÚ‡ÎÂÌ ÚËÔ ‚ ˜ÂÚËËÚ Í‡ÈÌË͇, Ò ÔÓ-ÚÂÊÍÓ Á‡Òfl„‡Ì ̇ ‰ÓÎÌËÚ Í‡ÈÌˈË. èÓÔËÓˆÂÔÚË‚ÌËflÚ ÛÒÂÚ Â Ì‡Û¯ÂÌ ÔË ÔÓ˜ÚË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ËÁÍβ˜ÂÌË ̇ 9 ÓÚ Úflı ̇ ‚˙Á‡ÒÚ ÓÚ 5 ‰Ó 9 „. èÓıӉ͇ڇ  ̇Û¯Â̇ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ͇ÚÓ Úfl  ÒÚÂÔ‡Ê̇ ÔË ÔÂÚ ÓÚ Úflı, ‡ ÔË ÓÒڇ̇ÎËÚ  ÒÚÂÔ‡ÊÌÓ-‡Ú‡ÍÚ˘̇, ‰˙Îʇ˘‡ Ò ÓÒ‚ÂÌ Ì‡ ‰ËÒÚ‡Î̇ڇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ë Ì‡ ÒÏÛÚÂÌËfl ÔÓÔËÓˆÂÔÚË‚ÂÌ ÛÒÂÚ. èË ‰‚‡Ï‡ Ô‡ˆËÂÌÚË Ò ̇·Î˛‰‡‚‡Ú ÚÂÊÍË ‰ËÒÚ‡ÎÌË ÚÓÙ˘ÌË ÔÓÏÂÌË Ò˙Ò Ò‡ÏÓ‡ÏÔÛÚ‡ˆËfl ̇ ‰ËÒÚ‡ÎÌËÚÂ Ù‡Î‡Ì„Ë Ì‡ Ô˙ÒÚËÚ ̇ ‰ÓÎÌËÚ Í‡ÈÌˈË. ëÍÂÎÂÚÌËÚ ‰ÂÙÓÏËÚÂÚË Ò ˜ÂÒÚË. èË 86 ÓÚ 110 Ô‡ˆËÂÌÚË Ò ̇·Î˛‰‡‚‡ ‰‚ÛÒÚ‡ÌÂÌ pes equinovarus, ‡ ÔË 59 Ë ÙÎÂÍÒËÓÌÌË ÍÓÌÚ‡ÍÚÛË Ì‡ Ô˙ÒÚËÚ ̇ ˙ˆÂÚÂ. ëÍÓÎËÓÁ‡  ÛÒÚ‡ÌÓ‚Â̇ ÔË 21 Ô‡ˆËÂÌÚË. çÖÇêéîàáàéãéÉàóçà àáëãÖÑÇÄçàü ëÍÓÓÒÚËÚ ̇ ÔÓ‚Âʉ‡Ì (ëè) ÔÓ ÏÓÚÓÌËÚ ‚·Í̇ Ò‡ ÒËÎÌÓ ‰ËÙÛÁÌÓ ÒÌËÊÂÌË. Ç ÛÎ̇ÌËfl ÌÂ‚ Ò‰̇ڇ ÒÍÓÓÒÚ Â 9.8 ±1.43 m/s Ò Û‰˙ÎÊÂ̇ ‰ËÒÚ‡Î̇ ÏÓÚÓ̇ ·ÚÂÌÚÌÓÒÚ (24.2±1.92 ms). ë˙ÓÚ‚ÂÚÌËÚ ÒÚÓÈÌÓÒÚË Á‡ ωˇÌÌËfl ÌÂ‚ Ò‡ 12.5±1.86 m/s Ë 14.4±1.68 ms. ã‡ÚÂÌÚÌÓÒÚÚ‡ ̇ Ô‰ËÁ‚Ë͇ÌËfl ÓÚ„Ó‚Ó ‚ Îˈ‚Ëfl ÌÂ‚ Ò˙˘Ó  Ôӂ˯Â̇ Ò˙Ò Ò‰̇ ÒÚÓÈÌÓÒÚ 14.8±0.78 ms (ÌÓχ < 4 ms). ë˙˘ÓÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ë Á‡ ‡ÍÒË·ÌËfl ÌÂ‚ – 14.6 ±0.82 ms Ë Á‡ n.musculocutaneus – 17.6±0.76 ms (ÌÓχ <3.5 ms Á‡ ‰‚‡Ú‡ ÌÂ‚‡). ç Ò ÓÚ‚Âʉ‡Ú ÒÂÚË‚ÌË ÔÓÚÂ̈ˇÎË ÓÚ Ï‰ˇÌÌËfl, ÛÎ̇ÌËfl Ë ÒÛ‡ÎÌËfl ÌÂ‚. àáëãÖÑÇÄçÖ çÄ ëãìïéÇàíÖ îìçäñàà ëÎÛıÓ‚ËÚ ÙÛÌ͈ËË Ò‡ ̇Û¯ÂÌË ÔË 78 ÓÚ 110 ËÁÒΉ‚‡ÌË Ô‡ˆËÂÌÚË. ÄÛ‰ËÓÏÂÚËflÚ‡ ÔÓ͇Á‚‡, ˜Â ÒÎÛıÓ‚ËflÚ ‰ÂÙˈËÚ Â ÔÓ-„ÓÎflÏ ÔË 1500 – 3000 Hz Ë ÔË > 4000 Hz, ÓÚÍÓÎÍÓÚÓ ÔË <1000 Hz. ìÒÚ‡ÌÓ‚fl‚‡ Ò ÒÎÛıÓ‚Ó Ì‡Û¯ÂÌË ÓÚ ÒÂÌÁÓÌ‚‡ÎÂÌ ÚËÔ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ËÁÍβ˜ÂÌË ̇ ‰ËÌ, ÔË ÍÓÈÚÓ Â Ì‡Îˈ Á‚ÛÍÓÔÓ‚Ó‰ÂÌ ‰ÂÙÂÍÚ. àÁÒΉ‚‡ÌËfl ̇ ÏÓÁ˙˜ÌÓÒÚ‚ÓÎÓ‚ËÚ ÒÎÛıÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË (Ï.Ò.ëÖè) Ò‡ Ôӂ‰ÂÌË ÔË 38 Ô‡ˆËÂÌÚË Ë Ò‡ÏÓ ÔË 8 Ô‡ˆËÂÌÚË Ò‡ ÔÓÎÛ˜ÂÌË Á‡ÔËÒË. ë‰ÌËÚ ÒÚÓÈ-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÌÓÒÚË ±SEM Á‡ Ô‡ˆËÂÌÚËÚÂ, ÔË ÍÓËÚÓ Ò‡ ÔÓÎÛ˜ÂÌË Á‡ÔËÒË, ͇ÚÓ Ò ÍÓÏ·ËÌË‡Ú ‰flÒ̇ Ë Îfl‚‡ ÒÚ‡Ì‡, Ò‡ ÒΉÌËÚÂ: ·ÚÂÌÚÌÓÒÚ Ì‡ ‚˙Î̇ I – 2.18 ± 0.90 ms (ÌÓχ 1.56 ms); ·ÚÂÌÚÌÓÒÚ Ì‡ ‚˙Î̇ III – 4.88 ± 1.26 ms (ÌÓχ 3.46 ms); ·ÚÂÌÚÌÓÒÚ Ì‡ ‚˙Î̇ V – 7.75 ± 1.50 ms (ÌÓχ 4.94 ms); I – V ËÌÚÂÔËÍÓ‚‡ ·ÚÂÌÚÌÓÒÚ – 5.59 ± 0.48 ms (ÌÓχ 4.15 ms). ÇÒ˘ÍË ÚÂÁË ÒÚÓÈÌÓÒÚË Ò‡ Ôӂ˯ÂÌË ÔË Ô‡ˆËÂÌÚËÚÂ. çÖÇêéèÄíéãéÉàóçà àáëãÖÑÇÄçàü àÁÒΉ‚‡ÌË Ò‡ ÒÛ‡ÎÌË Ì‚‡ÎÌË ÒÔÂÒËÏÂÌË ÓÚ 5 Ô‡ˆËÂÌÚË Ò ïåëçã ̇ ‚˙Á‡ÒÚ 6, 17, 28, 32 Ë 34 „. ÇÒ˘ÍËÚ 5 ÒÔÂÒËÏÂ̇ ÔÓ͇Á‚‡Ú ÚÂÊ͇ ‰ÂÔΈËfl ‚ ÏËÎËÌËÁË‡ÌËÚ ÌÂ‚ÌË ‚·Í̇, ‰ÓË Ë ÔË Ì‡È-Ï·‰Ëfl Ô‡ˆËÂÌÚ. èÎ˙ÚÌÓÒÚÚ‡ ̇ ‚·Í̇ڇ ̇χÎfl‚‡ Ò Ôӂ˯‡‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡ ̇ Ô‡ˆËÂÌÚ‡. á‡Ô‡ÁÂÌËÚ ÌÂ‚ÌË ‚·Í̇ Ò‡ Ò Ï‡Î˙Í ‡ÁÏÂ. éÚÌÓÒËÚÂÎ̇ڇ ‰Â·ÂÎË̇ ̇ ÏËÂÎË̇  ӈÂÌÂ̇ ˜ÂÁ g ÓÚÌÓ¯ÂÌËÂÚÓ – ‰Ë‡ÏÂÚ˙ ̇ ‡ÍÒÓ̇/Ó·˘ ‰Ë‡ÏÂÚ˙ ̇ ‚·ÍÌÓÚÓ. ó‡ÒÚÚ‡ ̇ ‚·Í̇ڇ Ò g ÓÚÌÓ¯ÂÌË ÔÓ-„ÓÎflÏÓ ÓÚ 0.7  Ôӂ˯Â̇ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ÍÓÂÚÓ Ò ‰˙ÎÊË Ì‡ Á‡„Û·‡ ̇ ‰Â·ÂÎËÚ ÏËÂÎËÌËÁË‡ÌË ‚·Í̇. ó‡ÒÚÚ‡ Ò g ÓÚÌÓ¯ÂÌË ÔÓ-χÎÍÓ ÓÚ 0.4 Ò˙˘Ó  ÔÓÌËÊÂ̇. ëΉӂ‡ÚÂÎÌÓ ÎËÔÒ‚‡Ú ‰‡ÌÌË Á‡ Á̇˜ËÚÂÎ̇ ıËÔÂÏËÂÎËÌËÁ‡ˆËfl. ç  ÛÒÚ‡ÌÓ‚Â̇ ‡ÍÚ˂̇ ‰Â„ÂÌÂ‡ˆËfl ËÎË ‰ÂÏËÂÎËÌËÁ‡ˆËfl, ÌÓ ÔÓÌflÍÓ„‡ Ò ̇·Î˛‰‡‚‡Ú ÏËÂÎËÌÓ‚Ë ÓÒÚ‡Ú˙ˆË, ÍÓÂÚÓ ÔÓ͇Á‚‡ ÒÍÓӯ̇ ‰ÂÏËÂÎËÌËÁ‡ˆËfl Ë ‰Â„ÂÌÂ‡ˆËfl. ïËÔÂÚÓÙ˘ÌË ÔÓÏÂÌË Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‚ Ì‚‡ÎÌËÚ ·ËÓÔÒËË ÓÚ ÔÓÏ·‰ËÚ ԇˆËÂÌÚË. èË Úflı Ò ÓÚÍË‚‡Ú ÏÌÓÊÂÒÚ‚ÂÌË “ÎÛÍӂˈ˔, ÍÓËÚÓ Ò‡ Ò··Ó ‡Á‚ËÚË. ë˙ÒÚÓflÚ Ò ÓÚ ÍÓ̈ÂÌÚ˘ÌÓ ÔÓÎËÙÂË‡ÎË ò‚‡ÌÓ‚Ë ÍÎÂÚÍË ÓÍÓÎÓ ˆÂÌÚ‡ÎÌËÚ ÏËÂÎËÌËÁË‡ÌË ‡ÍÒÓÌË ËÎË ò‚‡ÌÓ‚Ë ÍÎÂÚÍË, ‡ÒÓˆËË‡ÌË Ò ÌÂÏËÂÎËÌËÁË‡ÌË ‡ÍÒÓÌË. ã‡ÏËÌËÚ ̇ ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË Ì‡ “ÎÛÍӂˈËÚ” ˜ÂÒÚÓ Ò ÔˉÛʇ‚‡Ú ÓÚ ÌÂÏËÂÎËÌËÁË‡ÌË ‡ÍÒÓÌË. ï‡‡ÍÚÂ̇ ̇ıӉ͇ ‚ ·ËÓÔÒËflÚ‡ ÓÚ 28 „. Ô‡ˆËÂÌÚ Â, ˜Â “ÎÛÍӂˈËÚ” „ÂÒË‡Ú. ç Ò ÓÚÍË‚‡ ˆÂÌÚ‡ÎÌÓ ‡ÁÔÓÎÓÊÂÌ ‡ÍÒÓÌ Ë Â Ì‡Îˈ ҇ÏÓ Ó„‡Ì˘ÂÌ ·ÓÈ ÍÓ̈ÂÌÚ˘ÌÓ ÔÓ‰‰ÂÌË ÒÎÓ‚ ÓÚ ò‚‡ÌÓ‚Ë ÍÎÂÚÍË. èË Ì‡È-‚˙Á‡ÒÚÌËÚ ԇˆËÂÌÚË Ì Ò ÓÚÍË‚‡Ú ÌËÍ‡Í‚Ë ÎÛÍӂˈË. ÉÛÔË ÓÚ ò‚‡ÌÓ‚Ë ÍÎÂÚÍË Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ·ÂÁ fl‚ÌÓ ÍÓ̈ÂÌÚ˘ÌÓ ÔÓ‰Âʉ‡ÌÂ. í Ò ÔˉÛʇ‚‡Ú ÓÚ ÌÂÏËÂÎËÌËÁË‡ÌË ‡ÍÒÓÌË ËÎË ÔÓ-fl‰ÍÓ ÓÚ Ï‡ÎÍË ÏËÂÎËÌËÁË‡ÌË ‡ÍÒÓÌË (14). ÇÒ˘ÍË Ì‚‡ÎÌË ÒÔÂÒËÏÂÌË ÔÓ͇Á‚‡Ú ÂÍÒÚÂÌÁ˂̇ ẨÓÌ‚‡Î̇ ÍÓ·„ÂÌËÁ‡ˆËfl. èË ÔÓ‚˙Á‡ÒÚÌËÚ ԇˆËÂÌÚË ÏÌÓ„Ó ÓÚ ẨÓÌ‚‡ÎÌËÚ Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ҇ Á‡Ó·ËÍÓÎÂÌË ÓÚ ÏÌÓÊÂÒÚ‚ÂÌË ÒÎÓ‚ ÓÚ ‰ÛÔÎË͇ÚË‚ÌË ·‡Á‡ÎÌË Î‡ÏËÌË. èÂËÌ‚ËÛÏ˙Ú Ì ÔÓ͇Á‚‡ Á̇˜ËÏË ‰ÂÙÂÍÚË. ï‡‡ÍÚÂ̇ ˜ÂÚ‡  ̇΢ËÂÚÓ Ì‡ ÔÎÂÓÏÓÙÌË ÔÓÏÂÌË ‚ ˆËÚÓÔ·Áχڇ ̇ ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË, ˜ÂÒÚÓ Ò˙ÒÚÓfl˘Ë Ò ÓÚ ‡ÏÓÙÂÌ „‡ÌÛ·ÂÌ Ï‡ÚÂˇÎ, ‚Íβ˜ÂÌ ‚ ˜‡ÒÚË ÓÚ ÏÂÏ·‡Ì‡Ú‡ ËÎË ‡ÁÔÓÒÚË‡˘ Ò ÔÓ ÌÂÔÂÍ˙Ò̇ڇ ‰˙ÎÊË̇ ̇ ËÌÚÂÌÓ‰‡ (14). åéãÖäìãüêçé-ÉÖçÖíàóçé àáëãÖÑÇÄçÖ á‡·ÓÎfl‚‡ÌÂÚÓ Ô˙‚Ó̇˜‡ÎÌÓ Â Í‡ÚË‡ÌÓ ‚˙ıÛ 8q24 ıÓÏÓÁÓχ (11). èÓ-Í˙ÒÌÓ Â Ë‰ÂÌÚËÙˈË‡Ì‡ Ë ÏÛÚ‡ˆËflÚ‡ ë➞í Ú‡ÌÁˈËfl ‚ ÂÍÁÓÌ 7 Ë ÌÛÍÎÂÓÚˉ̇ ÔÓÁˈËfl 564 ̇ NDRG1 (N-myc downstream-regulated gene 1) „Â̇, ÓÚ„Ó‚Ó̇ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (10). íÓ‚‡ Á‡ÏÂÒÚ‚‡Ì Ô˘ËÌfl‚‡ Á‡Ïfl̇ ̇ ‡ÏËÌÓÍËÒÂÎË̇ڇ ‡„ËÌËÌ Ì‡ 148 ÔÓÁˈËfl (R148X). èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÏÛÚ‡ˆËflÚ‡ R148X ‚ ıÓÏÓÁË„ÓÚÌÓ, ‡ ÔË ÚÂıÌËÚ Ó‰ËÚÂÎË – ‚ ıÂÚÂÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌËÂ.

150

éÅëöÜÑÄçÖ äÎËÌ˘̇ڇ ͇ÚË̇ ̇ ïåëçã Ò ı‡‡ÍÚÂËÁË‡ Ò Ì‚ÓÔ‡ÚËfl, ÍÓflÚÓ Á‡ÔÓ˜‚‡ ÔÓÒÚÂÔÂÌÌÓ Ò Ì‡Û¯ÂÌË ̇ ÔÓıӉ͇ڇ ‚ Ô˙‚‡Ú‡ ‰Â͇‰‡ ̇ ÊË‚ÓÚ‡, ÔÓÒΉ‚‡Ì‡ ÓÚ Á‡Òfl„‡Ì ̇ „ÓÌËÚ Í‡ÈÌËˆË ‚˙‚ ‚ÚÓ‡Ú‡ ‰Â͇‰‡. åÓÚÓÌÓÚÓ Á‡Òfl„‡Ì  ÔÓ-ÚÂÊÍÓ ÓÚ ÒÂÌÁÓÌÓÚÓ Ë ÔÂӷ·‰‡‚‡ ‰ËÒÚ‡ÎÌÓ ‚ „ÓÌËÚÂ Ë ‰ÓÎÌËÚ Í‡ÈÌˈË. ÇÒ˘ÍË ÒÂÚË‚ÌË ÙÛÌ͈ËË Ò‡ ̇Û¯ÂÌË. ÑÂÙÓÏËÚÂÚË Ì‡ ıӉ˷ڇ ‚˙ÁÌËÍ‚‡Ú ÔË ÓÍÓÎÓ 77% ÓÚ Á‡Ò„̇ÚËÚ Ë̉˂ˉË, ÒÍÓÎËÓÁ‡  ̇Îˈ ÔË 19%. ëÂÌÁÓÌ‚‡Î̇ „ÎÛıÓÚ‡  ̇Îˈ ÔË Ôӂ˜ÂÚÓ ‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË Ë ‚˙ÁÌËÍ‚‡ ̇È-˜ÂÒÚÓ ‚ ÚÂÚ‡Ú‡ ‰Â͇‰‡, ‡ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË Ó˘Â ‚˙‚ ‚ÚÓ‡Ú‡ ‰Â͇‰‡. Ä‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl Ì Ò ̇·Î˛‰‡‚‡ Ò ËÁÍβ˜ÂÌË ̇ Ïۉ̇ ÁÂÌ˘̇ ‡͈Ëfl ̇ Ò‚ÂÚÎË̇ ÔË ‰‚‡Ï‡ Ô‡ˆËÂÌÚË (12). ëÎÛıÓ‚Ë Ì‡Û¯ÂÌËfl Ò‡ ÓÔËÒ‚‡ÌË, ͇ÍÚÓ ÔË ïåëç ÚËÔ I, ڇ͇ Ë ÔË ïåëç ÚËÔ II Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡Ì (17, 20). Perez H. Ë Ò˙Ú. (17) ÒÏflÚ‡Ú, ˜Â ̇Û¯ÂÌË ̇ ÒÎÛı‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË·ÎËÁËÚÂÎÌÓ ÔË 30% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ïåëç I Ë ïåëç II. éÚÓÌ‚ÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ÔÓ͇Á‚‡, ˜Â „ÎÛıÓÚ‡Ú‡  ÂÁÛÎÚ‡Ú ÓÚ Á‡Òfl„‡Ì ̇ ÓÒÏË ˜ÂÂÔÌÓÏÓÁ˙˜ÂÌ ÌÂ‚. ÉÎÛıÓÚ‡  Ò˙Ó·˘‡‚‡Ì‡ Ò˙˘Ó ÔË Ù‡ÏËÎËË Ò ïåëç Ò Ô‰ÔÓ·„‡ÂÏ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡Ì (4, 13). éÒÓ·ÂÌ ËÌÚÂÂÒ Ô‰ÒÚ‡‚Îfl‚‡ ÓÔË҇̇ڇ Ë̉ËÈÒ͇ Ù‡ÏËÎËfl ‚ ûÊ̇ ÄÙË͇ Ò ‰ÂÏËÂÎËÌËÁË‡˘‡ Ì‚ÓÔ‡ÚËfl Ò ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ, ÔˉÛÊÂ̇ Ò „ÎÛıÓÚ‡ (7). èË Ô‡ˆËÂÌÚËÚ ÓÚ Ú‡ÁË Ù‡ÏËÎËfl Ó·‡˜Â „ÎÛıÓÚ‡Ú‡ Ò ‡Á‚Ë‚‡ ‚ ‡ÌÌÓ ‰ÂÚÒÚ‚Ó, ÔÓ‡‰Ë ÍÓÂÚÓ ÔË Úflı Ì Ò  ‡Á‚Ë· ˜. ÉÎÛıÓÚ‡  Ò˙Ó·˘‡‚‡Ì‡ Ë ÔË ÔÓ-ÒÎÓÊÌË Á‡·ÓÎfl‚‡ÌËfl Ò ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ, ‚ ÍÓÏ·Ë̇ˆËfl Ò ÓÔÚ˘̇ ‡ÚÓÙËfl Ë ‰ËÒÚ‡Î̇ ÒÂÌÁÓ-ÏÓÚÓ̇ Ì‚ÓÔ‡ÚËfl (19) ËÎË Ò ÓÔÚ˘̇ ‡ÚÓÙËfl Ë ‰ËÒÚ‡Î̇ ‡ÏËÓÚÓÙËfl (9). ç‡Û¯ÂÌË ̇ ÒÎÛı‡ ‚˙ÁÌËÍ‚‡ Ò˙˘Ó ÔË ï – Ò‚˙Á‡Ì‡Ú‡ Ì‚ÓÔ‡ÚËfl, ÔˉÛÊÂ̇ Ò ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl, ͇ÚË‡Ì‡ ‚ ÔÂˈÂÌÚÓÏÂÌËfl ‡ÈÓÌ Ì‡ ıÓÏÓÁÓχڇ. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ïåëçã ÒÎÛıÓ‚ÓÚÓ Ì‡Û¯ÂÌË  ÓÚ ÒÂÌÁÓÌ‚‡ÎÂÌ ÚËÔ Ë Ò‡ÏÓ ÔË Â‰ËÌ Ô‡ˆËÂÌÚ ÚÓ Â ÍÓÏ·ËÌË‡ÌÓ Ò˙Ò Á‚ÛÍÓÔÓ‚Ó‰ÌÓ Ì‡Û¯ÂÌËÂ. éÒÓ·ÂÌÓ ı‡‡ÍÚÂÌÓ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò‡ ÒËÎÌÓ ‡·ÌÓÏÌËÚ Ï.Ò.ëÖè. è‰ÔÓ·„‡ ÒÂ, ˜Â „ÂÌÂ‡ÚÓËÚ Á‡ ‡Á΢ÌË ÍÓÏÔÓÌÂÌÚË Ì‡ Ï.Ò.ëÖè Ò‡ ‚ ‰ËÒÚ‡Î̇ڇ ˜‡ÒÚ Ì‡ ÓÒÏË ˜ÂÂÔÌÓÏÓÁ˙˜ÂÌ ÌÂ‚ Á‡ ‚˙Î̇ ¨, ÔÓÍÒËχÎ̇ڇ ˜‡ÒÚ Ì‡ ÓÒÏË ˜ÂÂÔÌÓÏÓÁ˙˜ÂÌ ÌÂ‚ Á‡ ‚˙Î̇ ¨¨, ‰ÓÎ̇ڇ ˜‡ÒÚ Ì‡ ÔÓÌÒ‡ Á‡ ‚˙Î̇ III, Ò‰̇ڇ Ë „Ó̇ ˜‡ÒÚ Ì‡ ÔÓÌÒ‡ Á‡ ‚˙Î̇ IV Ë „Ó̇ڇ ˜‡ÒÚ Ì‡ ÔÓÌÒ‡ ËÎË colliculus inferior Á‡ ‚˙Î̇ V. á‡ÔËÒË Ì‡ Ï.Ò.ëÖè Ò‡ ÓÒ˙˘ÂÒÚ‚ÂÌË ÔË ‰ˈ‡ ËÁÒΉ‚‡ÌËfl. Satya-Murti Ë Ò˙Ú. (20) Ò˙Ó·˘‡‚‡Ú Á‡ ÂÁÛÎÚ‡ÚË ÓÚ Ï.Ò.ëÖè ÔË ‰‚‡Ï‡ ·‡Úfl Ò ïåëç ·ÂÁ „ÎÛıÓÚ‡ ËÎË Ì‡Û¯ÂÌË ̇ ÍÓÌ‚Â̈ËÓ̇ÎÌÓÚÓ ‡Û‰ËÓÏÂÚ˘ÌÓ ÚÂÒÚÛ‚‡ÌÂ, ÔÓ͇Á‚‡˘Ë Ôӂ˯Â̇ I-III ËÌÚÂÔËÍÓ‚‡ ·ÚÂÌÚÌÓÒÚ. èËÍÓ‚Â IV –V Ì ҇ flÒÌÓ Ë‰ÂÌÚËÙˈË‡ÌË. ëè ÔÓ ÏÓÚÓÌËÚ ÌÂ‚ÌË ‚·Í̇  ÛÏÂÂÌÓ ÔÓÌËÊÂ̇ ÔË Â‰ËÌËfl ·‡Ú Ë ‚ ÌÓχÎÌË „‡ÌËˆË ÔË ‰Û„Ëfl. Scaioli Ë Ò˙Ú. (21) ËÁÒΉ‚‡Ú 31 Ô‡ˆËÂÌÚË Ò ïåëç ÚËÔ I Ë 11 Ô‡ˆËÂÌÚË Ò ïåëç ÚËÔ II. 臈ËÂÌÚËÚÂ Ò ïåëç ÚËÔ ¨¨ Ì ÔÓ͇Á‚‡Ú ÔÓÒÚÓflÌÌÓ Ôӂ˯ÂÌË ̇ ‡·ÒÓβÚÌËÚ ·ÚÂÌÚÌÓÒÚË, ‡ Ò‡ÏÓ ÎÂÍË ‡·ÌÓÏÌÓÒÚË Ì‡ I-III ËÌÚÂÔËÍÓ‚‡ ·ÚÂÌÚÌÓÒÚ. èË Ô‡ˆËÂÌÚËÚÂ Ò ïåëç ÚËÔ ¨ ·ÚÂÌÚÌÓÒÚÚ‡ ̇ ‚˙Î̇ I  Ôӂ˯Â̇ ‰ÌÓÒÚ‡ÌÌÓ ËÎË ‰‚ÛÒÚ‡ÌÌÓ ÔË 9 Ô‡ˆËÂÌÚË, ‡ I-III ËÌÚÂÔËÍÓ‚‡ ·ÚÂÌÚÌÓÒÚ Â Ôӂ˯Â̇ ÔË Â‰ËÌ. Ñ‚‡Ï‡ Ô‡ˆËÂÌÚË ËÏ‡Ú „‡Ì˘ÌË III–V ËÌÚÂÔËÍÓ‚Ë Î‡ÚÂÌÚÌÓÒÚË. ëËÎÌÓÚÓ ÒÌËÊÂÌË ̇ ëè ÔÓ ÏÓÚÓÌËÚ ÌÂ‚ÌË ‚·Í̇ ÔË ïåëçã ÔÓ͇Á‚‡, ˜Â Ì‚ÓÔ‡ÚËflÚ‡  ‰ÂÏËÂÎËÌË-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÁË‡˘‡ ÔÓ ı‡‡ÍÚÂ. ÑÂÏËÂÎËÌËÁ‡ˆËflÚ‡ Ò ÔÓÚ‚˙ʉ‡‚‡ ÓÚ Ì‡ıÓ‰ÍËÚ ÓÚ ÌÂ‚ÌËÚ ·ËÓÔÒËË. ífl Ò ÔˉÛʇ‚‡ ÓÚ Á̇˜ËÚÂÎ̇ Á‡„Û·‡ ̇ ‡ÍÒÓÌË ‰ÓË Ë ÔË Ì‡È-Ï·‰Ëfl Ô‡ˆËÂÌÚ, ÓÚ ÍÓÈÚÓ Â ‚ÁÂÚ‡ Ì‚‡Î̇ ·ËÓÔÒËfl. ÑÂÏËÂÎËÌËÁ‡ˆËflÚ‡ ÏÓÊ ‰‡ ·˙‰Â Ô˙‚˘̇, Ò‚˙Á‡Ì‡ Ò Ì‡Û¯ÂÌËÂ, Á‡Òfl„‡˘Ó ÙÛÌ͈ËflÚ‡ ̇ ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË, ËÎË ‚ÚÓ˘̇, ‰˙Îʇ˘‡ Ò ̇ ‡ÍÒÓ̇Î̇ ‡ÚÓÙËfl. Kalaydjieva L. Ë Ò˙Ú. (10) ˉÂÌÚËÙˈË‡Ú ÏÛÚ‡ˆËflÚ‡ R148X, ÓÚ„Ó‚Ó̇ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ‚ NDRG1 (N-myc downstream-regulated gene 1) „Â̇. ÇÒ˘ÍË Ô‡ˆËÂÌÚË Ò ïåëçã Ò‡ ÓÚ ÓÏÒÍË ÔÓËÁıÓ‰. ê‡ÁÔÓÒÚ‡ÌÂÌËÂÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔÓ͇Á‚‡, ˜Â ÚÓ‚‡  ̇È-˜ÂÒÚ‡Ú‡ ıÂ‰ËÚÂ̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl Ò‰ ÓÏËÚ ‚ Å˙΄‡Ëfl. ãàíÖêÄíìêÄ 1. Ä·‡‰ÊË‚ å, à. ÉÂÓ„Ë‚, å. LJÒË΂, è. å·‰ÂÌÓ‚, à. âÓÒËÙÓ‚, ë. ëÂÏÍÓ‚. èÓÔÛ·ˆËÓÌÌÓ-„ÂÌÂÚ˘ÌË ÔÓÛ˜‚‡ÌËfl ̇ ‰Â„ÂÌÂ‡ÚË‚ÌË Ë Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl ‚ åËı‡ÈÎÓ‚„‡‰ÒÍË ÓÍ˙„. ç‚ÓÎÓ„Ëfl, èÒËıˇÚËfl Ë ç‚ÓıËÛ„Ëfl 1977, 16, 153-160. 2. à¯ÔÂÍÓ‚‡ Å. ÖÎÂÍÚÓÙËÁËÓÎӄ˘ÌË ı‡‡ÍÚÂËÒÚËÍË Ë ‰Ë‡„ÌÓÒÚ˘ÌË ÍËÚÂËË ÔË ÌflÍÓË Ì‡ÒΉÒÚ‚ÂÌË Ì‚Ó-ÏÛÒÍÛÎÌË Á‡·ÓÎfl‚‡ÌËfl. ÑÓÍÚÓÒ͇ ‰ËÒÂÚ‡ˆËfl, ëÓÙËfl, 1995. 3. Baethmann M., Gohlich-Ratmann G., Schroder J., Kalaydjieva L., Voit T. HMSNL in a 13-year-old Bulgarian girl. Neuromusc. Disord., 1998, 8, 90-94. 4. Bouldin T.W., Riley E., Hall C.D., Swift M.: Clinical and pathological features of an autosomal recessive neuropathy. J. Neurol. Sci. 1980, 46, 315-23. 5. Butinar D., Zidar J. Leonardis L., et al. Hereditary, auditory, vestibular, motor and sensory neuropathy in a Slovenian Roma kindred. Ann. Neurol., 1999, 46, 36-44. 6. Colomer J., Iturriaga C., Kalaydjieva L., Angelicheva D., King R., Thomas P.K. Hereditary motor and sensory neuropathy-Lom in a Spanish family: clinical, electrophysiological, pathological and genetic studies. Neuromusc. Disord., 2000, 10, 578-583. 7. Cornell J., Sellars S., Beighton P.: Autosomal recessive inheritance of Charcot-Marie-Tooth disease associated with sensorineural deafness. Clin. Genet. 1984;25:163-165. Friedman H.C., Jelsma T.N., Bray G.M., Aguayo A.J.: A distinct pattern of trophic factor expression in myelin-deficient nerves of trembler mice: implications for trophic support by Schwann cells. J. Neurosci. 1996, 16, 5344-50. 8. Harding A.E., Thomas P.K.: Autosomal recessive forms of hereditary motor and sensory neuropathy. J. Neurol. Neurosurg. Psychiatry 1980, 43, 669-678. 9. Iwashita H., Inoue N., Araki S., Kuroiwa Y.: Optic atrophy, neural deafness and distal neurogenic amyotrophy. Arch. Neurol. 1970, 22, 357-364. 10. Kalaydjieva L., Gresham D., Gooding R., Heather L., Baas F., De Jonge R.,

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÑÓˆ. ‰- à‚‡ÈÎÓ í˙Ì‚ ‰.Ï.Ì. äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡ “ÄÎÂÍ҇̉Ó‚Ò͇” ÅÛÎ. “ë‚. É. ëÓÙËÈÒÍË” ‹1, ëÓÙËfl 1431 íÂÎ. (02) 9230 752 E-mail: emhpf@techno-link.com

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1å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ – ëÓÙËfl; ÔÓ ÏÓÎÂÍÛÎfl̇ „ÂÌÂÚË͇ ̇ ˜Ó‚Â͇, á‡Ô‡‰ÌÓ-Ä‚ÒÚ‡ÎËÈÒÍË ËÌÒÚËÚÛÚ Á‡ ωˈËÌÒÍË ËÁÒΉ‚‡ÌËfl, ìÌË‚ÂÒËÚÂÚ Ì‡ á‡Ô‡‰Ì‡ Ä‚ÒÚ‡ÎËfl, è˙Ú; 3ä‡Ú‰‡ ÔÓ ÍÎËÌ˘̇ Ì‚ÓÙËÁËÓÎÓ„Ëfl, ìÌË‚ÂÒËÚÂÚ Ì‡ ãÓ̉ÓÌ, ÇÂÎËÍÓ·ËÚ‡ÌËfl; 4ä‡Ú‰‡ ÔÓ ÍÎËÌ˘ÌË Ì‚Ó̇ÛÍË ìÌË‚ÂÒËÚÂÚ Ì‡ ãÓ̉ÓÌ, ÇÂÎËÍÓ·ËÚ‡ÌËfl; 5îÓ̉‡ˆËfl “á‰‡‚ÌË ÔÓ·ÎÂÏË Ì‡ χΈËÌÒÚ‚‡Ú‡” - ëÓÙËfl;

SUMMARY HEREDITARY MOTOR AND SENSORY NEUROPATHY TYPE RUSSE – CLINICAL AND GENETIC ASPECTS I. Tournev, T Rogers, V. Guergueltcheva, B. Youl, R. King, B. Ishpekova, V. Bojinova, A. Shmarov, M. Katzarova, P.K. Thomas, L. Kalaydjieva Hereditary motor and sensory neuropathy (HMSN) type Russe was initially identified as a novel type of severe autosomal recessive HMSN in Bulgarian Gypsies and subsequently diagnosed in Gypsy patients from Spain, Romania and France. We investigated 19 affected patients from 11 families. Distal muscle weakness of the lower limbs is established from ages 8-16 years, and of the upper limbs – from ages 10-43 years. The progressive disorder leads to severe muscle weak-

ness of the lower limbs and marked distal muscle upper limb weakness. Prominent distal sensory loss involves all modalities. All patients show foot deformities, and most show hand deformities. Motor nerve conduction velocities are moderately reduced in the upper limbs and unobtainable in the lower limbs. Sensory nerve action potentials are absent. There is loss of larger myelinated nerve fibers and profuse regenerative activity on the sural nerve biopsy. The disease was mapped to chromosome 10q23 distal to the EGR2 gene. Key words: hereditary motor and sensory neuropathy type Russe, conduction velocities, sural nerve biopsy. êÖáûåÖ ïÂ‰ËÚ‡̇ڇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl (ïåëç) ÚËÔ êÛÒ  ˉÂÌÚËÙˈË‡Ì‡ Ô˙‚Ó̇˜‡ÎÌÓ Í‡ÚÓ


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÌÓ‚ ÚËÔ ÚÂÊ͇ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ ïåëç ÔË ·˙΄‡ÒÍË ˆË„‡ÌË, ‡ ‚ ÔÓÒΉÒÚ‚Ë  ‰Ë‡„ÌÓÒÚˈË‡Ì‡ Ë Ò‰ ˆË„‡ÌË ÓÚ àÒÔ‡ÌËfl, êÛÏ˙ÌËfl Ë î‡ÌˆËfl. àÁÒΉ‚‡ıÏ 19 Ô‡ˆËÂÌÚË Ò ïåëç ÚËÔ êÛÒ ÓÚ 11 Ù‡ÏËÎËË. Ç˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ì‡ ‰ËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‚ ‰ÓÎÌË Í‡ÈÌËˆË Â 8-16 „., ‡ ‚ „ÓÌË Í‡ÈÌËˆË – 10-43 „. ᇷÓÎfl‚‡ÌÂÚÓ Â Ò ÔÓ„ÂÒË‡˘ ıÓ‰ Ë ‚Ó‰Ë ‰Ó ÚÂÊ͇ Ò··ÓÒÚ ‚ ‰ÓÎÌË Í‡ÈÌËˆË Ë ËÁ‡ÁÂ̇ ‰ËÒÚ‡Î̇ Ò··ÓÒÚ ‚ „ÓÌË Í‡ÈÌˈË. ìÒÚ‡ÌÓ‚fl‚‡Ú Ò Á̇˜ËÚÂÎÌË ‰ËÒÚ‡ÎÌË ÒÂÚË‚ÌË Ì‡Û¯ÂÌËfl, ÍÓËÚÓ Á‡Òfl„‡Ú ‚Ò˘ÍË ÏÓ‰‡ÎÌÓÒÚË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰ÂÙÓÏËÚÂÚË Ì‡ ÒÚ˙ԇ·ڇ, ‡ ÔË Ôӂ˜ÂÚÓ – Ë Ì‡ ˙ˆÂÚÂ. åÓÚÓÌËÚ ÒÍÓÓÒÚË Ì‡ ÔÓ‚Âʉ‡Ì ҇ ÛÏÂÂÌÓ ÒÌËÊÂÌË ‚ „ÓÌË Í‡ÈÌËˆË Ë Ì Ò ÔÓÎÛ˜‡‚‡Ú ‚ ‰ÓÎÌË Í‡ÈÌˈË. ëÂÚË‚ÌËÚ ÌÂ‚ÌË ‡ÍˆËÓÌÌË ÔÓÚÂ̈ˇÎË Ò‡ ÎËÔÒ‚‡˘Ë. èË ·ËÓÔÒËfl ̇ ÒÛ‡ÎÂÌ ÌÂ‚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Á‡„Û·‡ ̇ „ÓÎÂÏË ÏËÂÎËÌËÁË‡ÌË ÌÂ‚ÌË ‚·Í̇ Ë ËÁ‡ÁÂ̇ „ÂÌÂ‡Ú˂̇ ‡ÍÚË‚ÌÓÒÚ. ᇷÓÎfl‚‡ÌÂÚÓ Â Í‡ÚË‡ÌÓ ‚˙ıÛ ıÓÏÓÁÓχ 10q23 ‰ËÒÚ‡ÎÌÓ ÓÚ EGR2 „Â̇. äβ˜Ó‚Ë ‰ÛÏË: ıÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ êÛÒÂ, ÒÍÓÓÒÚË Ì‡ ÔÓ‚Âʉ‡ÌÂ, ·ËÓÔÒËfl ̇ ÒÛ‡ÎÂÌ ÌÂ‚. ÇöÇÖÑÖçàÖ èÓ ‚ÂÏ ̇ ÍÎËÌËÍÓ-ÂÔˉÂÏËÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ̇ ̇ÒΉÒÚ‚ÂÌËÚ Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl Ò‰ ÓÏËÚ ‚ Å˙΄‡Ëfl í˙Ì‚ Ë ä‡Î‡È‰ÊË‚‡ (1997-1998 „.) ˉÂÌÚËÙË‡Ú ÚÂÚ‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ ıÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl Ò ÔÓ-Í˙ÒÌÓ Ì‡˜‡ÎÓ Ë ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-·ÂÌË„ÌÂÌ ÍÎËÌ˘ÂÌ ıÓ‰ ‚ Ò‡‚ÌÂÌËÂ Ò ıÂ‰ËÚ‡̇ڇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ ãÓÏ (ïåëçã). èÂÁ 1996 „. ä‡Î‡È‰ÊË‚‡ ÓÚÍË‚‡ „ÓÎflÏÓ Ó‰ÓÒÎÓ‚ËÂ Ò ïåëçã ‚ „. êÛÒÂ. íË ÒÂÒÚË Ò ıÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÓÚ ÚÓ‚‡ Ó‰ÓÒÎÓ‚Ë Ì ҇ Ò͇˜ÂÌË Ò ÎÓÍÛÒ‡ ̇ ïåëçã. èÂÁ ÒΉ‚‡˘‡Ú‡ „Ó‰Ë̇ í˙Ì‚ Ë ë‡‚Ó‚ ËÁÒΉ‚‡Ú Ë ‡Á¯Ëfl‚‡Ú êÛÒÂÌÒÍÓÚÓ Ó‰ÓÒÎÓ‚ËÂ Ë ‚ ıÓ‰‡ ̇ ÚÓ‚‡ ËÁÒΉ‚‡Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â ‚ ÌÂ„Ó Ëχ 7 Ô‡ˆËÂÌÚË ÌÂÒ͇˜ÂÌË Ò 8q24. èÂÁ Ò˙˘‡Ú‡ „Ó‰Ë̇ í˙Ì‚ ˉÂÌÚËÙˈË‡ ÌÓ‚Ë Ó‰ÓÒÎÓ‚Ëfl ‚ „. óÂ‚ÂÌ ·fl„, Ò. ï˙Έ Ç‡˜‡ÌÒÍÓ, èÂÌËÍ, è‡Á‡‰ÊËÍ, ÄÍÒ‡ÍÓ‚Ó Ç‡ÌÂÌÒÍÓ Ò Ó·˘Ó 12 Á‡Ò„̇ÚË ÓÚ Á‡·ÓÎfl‚‡ÌÂÚÓ ËÌ‰Ë‚Ë‰Ë Ë ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â ‚Ò˘ÍË Ô‡ˆËÂÌÚË ÔË̇‰ÎÂÊ‡Ú Ì‡ ‰̇ Ë Ò˙˘‡ „ÛÔ‡, Ú‡ÁË Ì‡ ͇‰‡‡¯ËÚÂ. èÂÁ 1997 „. í˙Ì‚ Á‡ÔÓ˜‚‡ ÔÓÛ˜‚‡Ì ̇ ÍÎËÌ˘̇ڇ ı‡‡ÍÚÂËÒÚË͇ ̇ ·ÓÎÂÒÚÚ‡ ‚ è˙‚‡ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇ ̇ åÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”. è˙‚ËflÚ ËÁÒΉ‚‡Ì Ô‡ˆËÂÌÚ Ò “ÚÂÚ‡Ú‡ Ì‚ÓÔ‡ÚËfl” Â Ë Ì‡È-‚˙Á‡ÒÚÌËfl ËÁ‚ÂÒÚÂÌ, ̇ 61 „., Ò Í˙ÒÂÌ ‰Â·˛Ú ‚ Ò‡‚ÌÂÌËÂ Ò ïåëçã – ̇ 14 „., ‚Ò Ӣ Á‡Ô‡ÁÂ̇ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓıӉ͇ Ë ÌÓχÎÌË ÏÓÁ˙˜ÌÓ-ÒÚ‚ÓÎÓ‚Ë ÒÎÛıÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË – ÙÂÌÓÚËÔ, Ò˙˘ÂÒÚ‚ÂÌÓ ‡Á΢‡‚‡˘ Ò ÓÚ ÚÓÁË Ì‡ ïåëçã. èÂÁ 1998 „. ä‡Î‡È‰ÊË‚‡ ÔÓ‚Âʉ‡ „ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì ̇ ˉÂÌÚËÙˈË‡ÌËÚ Ó‰ÓÒÎÓ‚Ëfl Ò˙Ò Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÓÚÍË‚‡, ˜Â ÌËÚÓ Â‰ÌÓ ÓÚ Úflı Ì  Ò͇˜ÂÌÓ Ò ËÁ‚ÂÒÚÌËÚ Á‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌËÚ Ì‚ÓÔ‡ÚËË ÎÓÍÛÒË (5q23-33, 8q24, 8q13-21, 11q23.1 Ë 18qter). èӂ‰ÂÌËÚ ÍÎËÌ˘ÌË Ë „ÂÌÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl ‰‡‚‡Ú ÓÒÌÓ‚‡ÌË ‰‡ Ò ÔËÂÏÂ, ˜Â  ˉÂÌÚËÙˈË‡Ì‡ ÌÂËÁ‚ÂÒÚ̇ ‰Ó ÏÓÏÂÌÚ‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ ıÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl. í‡ÁË ÌÓ‚ÓÓÚÍËÚ‡ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ ò‡ÍÓ-å‡Ë-íÛÚ Â ÓÁ̇˜Â̇ ͇ÚÓ ıÂ‰ËÚ‡̇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ êÛÒ (ïåëçê), ̇ ËÏÂÚÓ Ì‡ „‡‰‡ Ò Ì‡È-ÏÌÓ„Ó Á‡Ò„̇ÚË Ë̉˂ˉË. èÂÁ 1998 „. ·˙΄‡ÒÍËflÚ ÍÎËÌ˘ÂÌ ÂÍËÔ Ò˙‚ÏÂÒÚÌÓ Ò ÔÓÙ. íÓÏ‡Ò Ë ‰- âÓÎ ÓÚ ä‡Ú‰‡Ú‡ ÔÓ ÍÎËÌ˘ÌË Ì‚Ó̇ÛÍË Ì‡ ãÓ̉ÓÌÒÍËfl ìÌË‚Â-

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ÒËÚÂÚ, ÍÓËÚÓ Ë‰‚‡Ú ‚ Å˙΄‡Ëfl Ò ÔÓÚ‡ÚË‚ÂÌ ÂÎÂÍÚÓÏËÓ„‡Ù, ÔÓÒ¢‡‚‡ „ÓÎflχ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚ ÔÓ ÚÂıÌËÚ ‰ÓÏÓ‚Â Ë ÔÓ‚Âʉ‡ ÍÎËÌ˘ÌË Ë ÂÎÂÍÚÓÏËÓ„‡ÙÒÍË ËÁÒΉ‚‡ÌËfl. äÎËÌ˘ÌÓÚÓ ÙÂÌÓÚËÔËÁË‡Ì ̇ ïåëçê Á‡‚˙¯‚‡ ÔÂÁ Ï‡È 1999 „., ÒΉ ͇ÚÓ Ò‡ ÓÒ˙˘ÂÒÚ‚ÂÌË ‰ÓÔ˙ÎÌËÚÂÎÌË ËÁÒΉ‚‡ÌËfl Ë Ò‡ ‚ÁÂÚË 2 Ì‚‡ÎÌË ·ËÓÔÒËË ÓÚ Ô‡ˆËÂÌÚË Ò˙Ò Á‡·ÓÎfl‚‡ÌÂÚÓ ‚ ÑìÅ “ÄÎÂÍ҇̉Ó‚Ò͇”. é·‡·ÓÚÂÌËÚÂ Ë Á‡Ï‡ÁÂÌËÚ Ì‚‡ÎÌË ÒÔÂÒËÏÂÌË Ò‡ Ú‡ÌÒÔÓÚË‡ÌË Ò˙Ò ÒÛı Ή ÓÚ åì - ëÓÙËfl ‰Ó ãÓ̉ÓÌ. ç‚ÓÔ‡ÚÓÎӄ˘ÌËÚ ËÁÒΉ‚‡ÌËfl Ò‡ ÓÒ˙˘ÂÒÚ‚ÂÌË ÓÚ ‰- ê. äËÌ„ Ë ÔÓÙ. íÓχÒ. åÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌËflÚ ‡Ì‡ÎËÁ  ÓÒ˙˘ÂÒÚ‚ÂÌ ÓÚ ÂÍËÔ‡ ̇ ‰- ã. ä‡Î‡È‰ÊË‚‡ ‚ ñÂÌÚ˙‡ ÔÓ „ÂÌÂÚË͇ ̇ ˜Ó‚Â͇, Ö‰ËÚ äÓÛ˙Ì ìÌË‚ÂÒËÚÂÚ‡, è˙Ú. ÑàáÄâç çÄ àáëãÖÑÇÄçÖíé àÁÒΉ‚‡ÌË Ò‡ Ó·˘Ó 19 Ô‡ˆËÂÌÚË, ÔË̇‰ÎÂʇ˘Ë ̇ 11 Ù‡ÏËÎËË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Ôӂ‰ÂÌÓ ËÁÒΉ‚‡Ì ̇ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ, ͇ÍÚÓ Ë ÂÎÂÍÚÓÏËÓ„‡ÙÒÍË ËÁÒΉ‚‡ÌËfl. èË 5 Ô‡ˆËÂÌÚË Â ÓÒ˙˘ÂÒÚ‚ÂÌÓ ÓÚÓÌ‚ÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ Ë ËÁÒΉ‚‡Ì ̇ ÏÓÁ˙˜ÌÓ-ÒÚ‚ÓÎÓ‚ËÚ ÒÎÛıÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË. ëÛ‡ÎÌË Ì‚‡ÎÌË ·ËÓÔÒËË Ò‡ ‚ÁÂÚË ÓÚ 2-χ Ô‡ˆËÂÌÚË Ò˙Ò Á‡·ÓÎfl‚‡ÌÂÚÓ. èӂ‰ÂÌË Ò‡ ÏÓÙÓÏÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl ̇ Ì‚‡ÎÌËÚ ÒÔÂÒËÏÂÌË. èӂ‰ÂÌÓ Â ÍÎËÌËÍÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡ÌÂ, ÍÓÂÚÓ ÔÓ͇Á‚‡ ‡‚ÚÓÁÓÏÌÓ – ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ, ͇ÍÚÓ Ë „ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì Á‡ Ò͇˜ÂÌÓÒÚ. ÄçÄåçÖëíàóçà à äãàçàóçà ÑÄççà ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ËÁÒΉ‚‡ÌËÚ ËÌ‰Ë‚Ë‰Ë Â 36.8 „., ‚‡Ë‡˘‡ ÏÂÊ‰Û 6 Ë 61 „Ó‰ËÌË. ë˙ÓÚÌÓ¯ÂÌËÂÚÓ Ï˙Ê – ÊÂÌË Â 14:5. Ñ·˛Ú˙Ú Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚ ̇˜‡ÎÓÚÓ Ì‡ ‚ÚÓ‡Ú‡ ‰Â͇‰‡ ÓÚ ÊË‚ÓÚ‡ Ò ÔÓfl‚‡ ̇ ‰ËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‚ ‰ÓÎÌËÚ Í‡ÈÌˈË. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓÚÓ Â 11.1 ± 5.6, ‚‡Ë‡˘‡ ÓÚ 8 ‰Ó 16 „Ó‰ËÌË. 燘‡ÎÓÚÓ Ì‡ Á‡Òfl„‡ÌÂÚÓ Ì‡ „ÓÌËÚ Í‡ÈÌËˆË ‚‡Ë‡ ‚ ÏÌÓ„Ó ÔÓ-„ÓÎflχ ÒÚÂÔÂÌ (ÏÂÊ‰Û 10 Ë 43„.) Ë Ì‡ÒÚ˙Ô‚‡ Ò‰ÌÓ Ì‡ 23 ± 7.0 „Ó‰ËÌË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰ËÒÚ‡ÎÌË ÚÓÙ˘ÌË Ì‡Û¯ÂÌËfl Ò ÔÓfl‚‡ ̇ ‰ÂÙÓÏËÚÂÚË ‚ ıӉ˷ڇ ÔÓ‰ ÙÓχڇ ̇ pes equinovarus Ë ÙÎÂÍÒËÓÌÌË ÍÓÌÚ‡ÍÚÛË ‚ ˙ˆÂÚ (ÔË ‚Ò˘ÍË, Ò ËÁÍβ˜ÂÌË ̇ ‰ËÌ). ëÍÓÎËÓÁ‡ Ì  ̇·Î˛‰‡‚‡Ì‡ ÔË ÌËÚÓ Â‰ËÌ Ô‡ˆËÂÌÚ. ç  ̇·Î˛‰‡‚‡ÌÓ Ë Á‡‰Â·ÂÎfl‚‡Ì ̇ ÔÂËÙÂÌËÚ ÌÂ‚Ë. èË Ì‚ÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì Ò ÓÚÍË‚‡ ËÁ‡ÁÂ̇ ‰ËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl ‚ ˜ÂÚËËÚ Í‡ÈÌˈË. Ç ‰ÓÎÌËÚ Í‡ÈÌËˆË Ò‡ Á‡Ò„̇ÚË ÚÂÊÍÓ, ͇ÍÚÓ ‡ÌÚÂÓ-·ÚÂ‡ÎÌËÚÂ, ڇ͇ Ë Á‡‰ÌËÚ ÏÛÒÍÛÎÌË „ÛÔË, ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ËÁÍβ˜ÂÌË ̇ 3-χ ÔÓ-Ï·‰Ë, ÔË ÍÓËÚÓ Â Á‡Ò„̇ڇ ÔÂӷ·‰‡‚‡˘Ó ‡ÌÚÂÓ-·ÚÂ‡Î̇ڇ ÏÛÒÍÛÎ̇ „ÛÔ‡. ë··ÓÒÚÚ‡ Ò Á‡ÒË΂‡ Ò ‚˙Á‡ÒÚÚ‡ Ë ÔË Ì‡È-‚˙Á‡ÒÚÌËfl Ô‡ˆËÂÌÚ Ì‡ 61 „. Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÚÓÚ‡Î̇ Ô‡‡ÎËÁ‡ ̇ ıӉ˷ڇ Ò ËÁÍβ˜ÂÌË ̇ ÎÂÍÓ Á‡Ô‡Á‚‡Ì ̇ ÙÎÂÍÒËflÚ‡ ̇ ·Â‰ÓÚÓ. ÑËÒÚ‡Î̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‚ „ÓÌËÚ Í‡ÈÌËˆË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË ‚Ò˘ÍËÚ 19 Ô‡ˆËÂÌÚË, Á‡Òfl„‡˘‡, ͇ÍÚÓ Ï‡ÎÍËÚ ÏÛÒÍÛÎË Ì‡ ‰Î‡ÌËÚÂ, ڇ͇ Ë ÙÎÂÍÒÓËÚÂ Ë ÂÍÒÚÂÌÁÓËÚ ̇ Ô‰Ï˯ÌˈËÚÂ. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ô˙Î̇ ÒÛıÓÊËÎ̇ Ë Ì‡‰ÍÓÒÚ̇ ‡ÂÙÎÂÍÒËfl, ‡ Ô·ÌÚ‡ÌËÚ ÂÙÎÂÍÒË Ì Ò ÔÓÎÛ˜‡‚‡Ú. ÑËÒÚ‡ÎÌË ÒÂÚË‚ÌË Ì‡Û¯ÂÌËfl, ͇ÍÚÓ Ì‡ ÛÒÂÚ‡ Á‡ ·ÓÎ͇ Ë Á‡ ‰ÓÔË, ڇ͇ Ë Ì‡ ÒÚ‡‚ÌÓ-ÏÛÒÍÛÎÌËfl Ë ‚Ë·‡ˆËÓÌÌËfl ÛÒÂÚ, Ò ̇·Î˛‰‡‚‡Ú Ò˙˘Ó ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Í‡ÚÓ Ú ҇ ÔÓ-ÚÂÊÍË ÔË ÔÓ-‚˙Á‡ÒÚÌËÚ ÓÚ Úflı. èË Â‰ËÌ Ô‡ˆËÂÌÚ Â Ì‡·Î˛‰‡‚‡Ì‡ ‰‚ÛÒÚ‡Ì̇ Ì‚ÓÔ‡Ú̇ ‰Â„ÂÌÂ‡ˆËfl ‚ „ÎÂÁÂÌÌËÚ ÒÚ‡‚Ë. Ä̇ÏÌÂÁ‡ Á‡ ÏÌÓÊÂÒÚ‚Â-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÌË ·ÂÁ·ÓÎÍÓ‚Ë Ì‡‡Ìfl‚‡ÌËfl ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Â Ì‡Îˈ ÔË ‰‚‡Ï‡ Ô‡ˆËÂÌÚË, ͇ÚÓ ÔË Â‰ËÌ ÓÚ Úflı Ò  ‡Á‚ËÎ Ë ÓÒÚÂÓÏËÂÎËÚ Ì‡ ‰̇ڇ ÔÓ‰·Â‰ˈ‡. ãÂÍ ÔÓÒÚÛ‡ÎÂÌ ÚÂÏÓ ‚ „ÓÌËÚ Í‡ÈÌËˆË Â ÛÒÚ‡ÌÓ‚ÂÌ Ò‡ÏÓ ÔË Â‰ËÌ ÒÎÛ˜‡È. á‡Òfl„‡Ì ̇ ˜ÂÂÔÌÓ-ÏÓÁ˙˜ÌË ÌÂ‚Ë (ÔÚÓÁ‡, ‰‚ÛÒÚ‡Ì̇ Îˈ‚‡ Ò··ÓÒÚ, ‰ËÒÙÓÌËfl)  ̇Îˈ ÔË ÚË ÒÎÛ˜‡fl. ÖãÖäíêéîàáàéãéÉàóçà àáëãÖÑÇÄçàü èË ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚ Ì ҇ Óڂ‰ÂÌË ÒÍÓÓÒÚË Ì‡ ÔÓ‚Âʉ‡Ì (ëè) ÔÓ ÏÓÚÓÌËÚ ‚·Í̇ ÓÚ Ï‡ÎÍËÚ ÏÛÒÍÛÎË Ì‡ ıÓ‰ËÎÓÚÓ ËÎË ÓÚ m.tibialis anterior. Ç „ÓÌËÚ Í‡ÈÌËˆË ëè ÔÓ ÏÓÚÓÌËÚ ‚·Í̇ Ë ÓÚ‚Âʉ‡Ì ÓÚ Ï‡ÎÍËÚ ÏÛÒÍÛÎË Ì‡ ‰Î‡ÌËÚ Ò ÔÓÎÛ˜‡‚‡Ú ÔË Â‰ËÌ Ô‡ˆËÂÌÚ Ì‡ 27 „Ó‰ËÌË, ÔË ÍÓÈÚÓ ÒÍÓÓÒÚÚ‡ ‚ n. ulnaris, Á‡ÔË҇̇ ÓÚ mm.abductor digiti minimi  ÒÌËÊÂ̇ ‰Ó 33 m/s Ò ‰ËÒÚ‡Î̇ ·ÚÂÌÚÌÓÒÚ Ì‡ „Ó̇ „‡Ìˈ‡ ̇ ÌÓχڇ – 3.4 ms. èË ÓÒڇ̇ÎËÚ ԇˆËÂÌÚË ëè ÔÓ ÏÓÚÓÌËÚ ‚·Í̇ Ò‡ Óڂ‰ÂÌË Á‡ n. ulnaris Ë n. medianus ‚ „ÓÌËÚ Í‡ÈÌˈË, Á‡ÔËÒ‡ÌË ÂÒÔÂÍÚË‚ÌÓ ÓÚ m. flexor carpi ulnaris Ë m. flexor digitorum profundus. í ҇ ÛÏÂÂÌÓ ÒÌËÊÂÌË: 31.9 ± 7.05 m/s Ë 32.0 ± 6.8 m/s, Ò ‰ËÒÚ‡ÎÌË Î‡ÚÂÌÚÌÓÒÚË 4.4 ± 3.8 Ë 4.2 ± 3.5 ms. èË ‰‚‡Ï‡Ú‡ ̇È-‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË Ì‡ 57 Ë Ì‡ 61 „Ӊ˯̇ ‚˙Á‡ÒÚ Ì ҇ Óڂ‰ÂÌË ëè ‚ „ÓÌËÚ Í‡ÈÌËˆË ‰ÓË ÓÚ m. biceps Ë m. deltoideus ÔË ÒÚËÏÛÎË‡Ì ‚ ÚӘ͇ڇ ̇ Ö·. á‡ÔËÒË Ò‡ ̇Ô‡‚ÂÌË Ë ÓÚ m. nasalis ÔË ÒÚËÏÛ·ˆËfl ̇ Îˈ‚Ëfl ÌÂ‚. ìÒÚ‡ÌÓ‚Â̇  Ò‰̇ ÒÚÓÈÌÓÒÚ 3.6 ± 3.5 ms. (ÌÓχ < 3.0 ms). èË Â‰ËÌ Ô‡ˆËÂÌÚ ·ÂÁ ÍÎËÌ˘ÌÓ ÔÓfl‚Â̇ Îˈ‚‡ Ò··ÓÒÚ Ì  ÔÓÎÛ˜ÂÌ ÓÚ„Ó‚Ó ‰ÓË ÔË ÒÚËÏÛ·ˆËfl Ò „ÓÎflÏ ËÌÚÂÌÁËÚÂÚ. ìÒÚ‡ÌÓ‚fl‚‡ Ò Ò˙˘Ó, ˜Â ÔË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Ò Á‡ÔËÒ‚‡Ú ÓÚ„Ó‚ÓË, Ò ËÁËÒÍ‚‡Ú ÒÚËÏÛ·ˆËË Ò ‚ËÒÓÍ ËÌÚÂÌÁËÚÂÚ. ëÂÚË‚ÌË ‡ÍˆËÓÌÌË ÔÓÚÂ̈ˇÎË Ì ҇ Óڂ‰ÂÌË ÌËÚÓ ÓÚ „ÓÌËÚ (n.medianus ËÎË n. ulnaris), ÌËÚÓ ÓÚ ‰ÓÎÌËÚ Í‡ÈÌËˆË (n. suralis). å.Ò.ëÖè ËÁÒΉ‚‡ÌË ÔË 5 Ô‡ˆËÂÌÚË Ò ïåëçê, Ò‡ ‚ ÌÓχ. çÖÇêéèÄíéãéÉàóçà àáëãÖÑÇÄçàü ëÛ‡ÎÌË Ì‚‡ÎÌË ·ËÓÔÒËË Ò‡ ÔÓÎÛ˜ÂÌË ÓÚ 2-χ Ô‡ˆËÂÌÚË – Ï˙Ê Ì‡ 31 „. Ë ÊÂ̇ ̇ 43 „. ç Ò ̇·Î˛‰‡‚‡ ‡ÍÚ˂̇ ‰Â„ÂÌÂ‡ˆËfl Ë ‰ÂÏËÂÎËÌËÁ‡ˆËfl ̇ ÌÂ‚ÌËÚ ‚·Í̇. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ıËÔÂÚÓÙ˘ÌË ÔÓÏÂÌË, ÌÓ Ë ‚ ‰‚‡Ú‡ ÒÎÛ˜‡fl Ò ÓÚÍË‚‡Ú „ÓÎflÏ ·ÓÈ „ÂÌÂ‡ÚË‚ÌË Í·ÒÚÂË. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡·ÌÓÏÌË ‚Íβ˜‚‡ÌËfl ‚ ‡ÍÒÓÌËÚ ËÎË ‚ ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË. èÎ˙ÚÌÓÒÚÚ‡ ̇ ÏËÂÎËÌÓ‚ËÚ ‚·Í̇  ‚ ÌÓχÎÌË „‡ÌËˆË ÔË ÔÓ-Ï·‰Ëfl Ô‡ˆËÂÌÚ, ÌÓ Â ÔÓÌËÊÂ̇ ÔË ÔÓ-‚˙Á‡ÒÚÌËfl. ç‡Îˈ  ÔÂӷ·‰‡‚‡˘‡ ‰ÂÔΈËfl ̇ „ÓÎÂÏËÚ ‚·Í̇ Ë ÔË ‰‚‡Ú‡ ÒÎÛ˜‡fl Ò ÓÚÌÓÒËÚÂÎÂÌ ËÁÎ˯˙Í Ì‡ ÔÓ-χÎÍËÚ ‚·Í̇, ÍÓÂÚÓ ˜‡ÒÚ˘ÌÓ ÏÓÊ ‰‡ Ò ҂˙ÊÂ Ò Ì‡Î˘ËÂÚÓ Ì‡ „ÂÌÂË‡˘Ë ‚·Í̇ Ò Ï‡Î˙Í Í‡ÎË·˙. íflıÌÓÚÓ Ì‡Î˘Ë ‚ÂÓflÚÌÓ ‰ÓÔË̇Òfl Á‡ ÔÓ‰‰˙ʇÌÂÚÓ Ì‡ ÔÎ˙ÚÌÓÒÚÚ‡ ̇ ‚·Í̇ڇ. Ñ·ÂÎË̇ڇ ̇ ÏËÂÎËÌÓ‚‡Ú‡ Ó·‚˂͇  ‡·ÌÓÏ̇ Á‡ ‰Ë‡ÏÂÚ˙‡ ̇ ‡ÍÒÓ̇. ç‡Î˘ËÂÚÓ Ì‡ ‰‡ÌÌË Á‡ ıËÔÓÏËÂÎËÌËÁ‡ˆËfl Ò ÔÓÚ‚˙ʉ‡‚‡ ÓÚ ËÁÏÂ‚‡ÌÂÚÓ Ì‡ g ÓÚÌÓ¯ÂÌËÂÚÓ (‰Ë‡ÏÂÚ˙ ̇ ‡ÍÒÓ̇/Ó·˘ ‰Ë‡ÏÂÚ˙ ̇ ‚·ÍÌÓÚÓ). ìÒÚ‡ÌÓ‚fl‚‡ ÒÂ, ˜Â ˜‡ÒÚÚ‡ ÓÚ ‚·Í̇ڇ Ò ‚ËÒÓÍÓ g ÓÚÌÓ¯ÂÌË (ÔÓ-„ÓÎflÏÓ ÓÚ 0.7)  Ôӂ˯Â̇ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎÌËÚ ËÌ‰Ë‚Ë‰Ë ‚ Ò˙˘Ëfl ‚˙Á‡ÒÚÓ‚ ‰Ë‡Ô‡ÁÓÌ. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰‡ÌÌË Á‡ ‡ÍÒÓ̇Î̇ ‡ÚÓÙËfl: ˜‡ÒÚÚ‡ ̇ ‚·Í̇ڇ Ò ÌËÒÍÓ g ÓÚÌÓ¯ÂÌËÂ, ÔÓ͇Á‚‡˘‡ ÓÚÌÓÒËÚÂÎ̇ڇ ‰Â·ÂÎË̇ ̇ ÏËÂÎËÌÓ‚‡Ú‡ Ó·‚˂͇, Ì  Ôӂ˯Â̇ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎÌËÚ ÌÂ‚Ë. èÎ˙ÚÌÓÒÚÚ‡ ̇ ÌÂÏËÂÎËÁË‡ÌËÚ ‡ÍÒÓÌË Â ÎÂÍÓ Ôӂ˯Â̇ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎËÚÂ, ÍÓÂÚÓ ‚ÂÓflÚÌÓ Â Ò‚˙-

153

Á‡ÌÓ Ò˙Ò Á‡„Û·‡Ú‡ ̇ „ÓÎÂÏËÚ ÏËÂÎËÌËÁË‡ÌË ‚·Í̇ Ë ‚Íβ˜‚‡ÌÂÚÓ Ì‡ ÌÂÏËÂÎËÌËÁË‡ÌËÚ „ÂÌÂË‡ÎË ËÁ‡ÒÚ˙ˆË ̇ ‡ÍÒÓ̇. èÓ‰ ÂÎÂÍÚÓÌÂÌ ÏËÍÓÒÍÓÔ ÏÓÙÓÎÓ„ËflÚ‡ ̇ ‚·Í̇ڇ ̇ Remak  ÌÓχÎ̇. êÖáìãíÄíà éí ÉÖçÖíàóçéíé àáëãÖÑÇÄçÖ çÄ ïåëçê èӂ‰ÂÌÓÚÓ „Â̇Îӄ˘ÌÓ ËÁÒΉ‚‡Ì ̇ ˉÂÌÚËÙˈË‡ÌËÚ هÏËÎËË Ò ïåëçê ÔÓ͇Á‚‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. êÓ‰ËÚÂÎËÚ ̇ ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ Á‰‡‚Ë. Ç˙‚ ‚Ò˘ÍË Á‡Ò„‡ÌË Ù‡ÏËÎËË Ò ÛÒÚ‡ÌÓ‚Ë ‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ Í˙‚ÌÓÓ‰ÒÚ‚ÂÌË ·‡ÍÓ‚Â. éÚ Á‡Ò„̇ÚËÚ Ë̉˂ˉË, ÚÂıÌËÚ Ó‰ËÚÂÎË, ·‡Úfl Ë ÒÂÒÚË Â ‚ÁÂÚ‡ Í˙‚ Á‡ ÂÍÒÚ‡ÍˆËfl ̇ Ñçä Ç ‡Ì‡ÎËÁ‡ Á‡ Ò͇˜ÂÌÓÒÚ Ò‡ ‚Íβ˜ÂÌË 15 Ë̉˂ˉË, ÓÚ ÍÓËÚÓ 11 ·ÓÎÌË Ò ïåëçê. ëΉ ˉÂÌÚËÙˈË‡Ì ̇ ͇̉ˉ‡Ú-‡ÈÓ̇, ‚ „ÂÌÂÚ˘ÌÓÚÓ Í‡ÚË‡Ì ҇ ‚Íβ˜ÂÌË ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ó˘Â 4 Á‡Ò„̇ÚË Ë̉˂ˉË. ÉÂÌ˙Ú Á‡ ïåëçê  ͇ÚË‡Ì ‚ χÎ˙Í ËÌÚÂ‚‡Î ‚ 10q23.2 ıÓÏÓÁÓχ, Ù·ÌÍË‡Ì ÏÂÊ‰Û Ï‡ÍÂËÚ D10S581 Ë D10S1742, ÚÂÎÓÏÂÌÓ Ì‡ EGR2 „Â̇ (6). èÂÁ 2003 „Ó‰. ÍËÚ˘ÌËflÚ ‡ÈÓÌ ÓÚ ıÓÏÓÁÓχ 10q23.2  ̇χÎÂÌ Ì‡ 70 kb (3). Ç ÚÓÁË ‡ÈÓÌ ‡‚ÚÓËÚ ËÁÍβ˜‚‡Ú ÏÛÚ‡ˆËË ‚ „ÂÌË, ˜ËflÚÓ ÙÛÌ͈Ëfl ÒÔÂÍÛ·ÚË‚ÌÓ Ò ÒÏflÚ‡, ˜Â  ҂˙Á‡Ì‡ Ò ÔÂËÙÂ̇ڇ ÌÂ‚̇ ÒËÒÚÂχ. éÒÚ‡‚‡ ‰‡ Ò Ô‰ÔÓ·„‡, ˜Â ÏÛÚ‡ˆËflÚ‡ Ò ̇ÏË‡ ËÎË ‚ ÌÂËÁ‚ÂÒÚÂÌ Ú‡ÌÒÍËÔÚ Ì‡ ÌflÍÓÈ ÓÚ ËÁ‚ÂÒÚÌËÚ „ÂÌË ËÎË ‚ ÌÓ‚, ÌÂˉÂÌÚËÙˈË‡Ì „ÂÌ. ÉÂÌÓÚËÔÌËflÚ ‡Ì‡ÎËÁ ÔÓ͇Á‚‡ „ÂÌÂÚ˘̇ ıÓÏÓ„ÂÌÌÓÒÚ Ë Ô‰ÔÓ·„‡ Ó·˘‡ ÏÛÚ‡ˆËfl ̇ Ô‡Ó‰ËÚÂÎfl. àÁÒΉ‚‡ÌËflÚ‡ ÔÓ ÍÎÓÌË‡ÌÂÚÓ Ì‡ „Â̇ Ë Ë‰ÂÌÚËÙˈË‡ÌÂÚÓ Ì‡ ÏÛÚ‡ˆËflÚ‡, ‚Ӊ¢‡ ‰Ó ·ÓÎÂÒÚÚ‡, ÔÓ‰˙Îʇ‚‡Ú. éÅëöÜÑÄçÖ Ä‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌËÚ ıÂ‰ËÚ‡ÌË ÏÓÚÓÌË Ë ÒÂÌÁÓÌË Ì‚ÓÔ‡ÚËË Ò‡ ÍÎËÌ˘ÌÓ, Ì‚ÓÔ‡ÚÓÎӄ˘ÌÓ Ë „ÂÌÂÚ˘ÌÓ ıÂÚÂÓ„ÂÌ̇ „ÛÔ‡ ÓÚ ‰ÍË Á‡·ÓÎfl‚‡ÌËfl. Ñ·˛Ú˙Ú ËÏ Ó·ËÍÌÓ‚ÂÌÓ Â ‚ ‡Ì̇ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ, ‚ÓβˆËflÚ‡ ËÏ Â ·˙Á‡ Ë ‚Ó‰flÚ ‰Ó ÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl. í Ò ˉÂÌÚËÙˈË‡Ú ̇È-˜ÂÒÚÓ ‚ Á‡Ú‚ÓÂÌË Ë Í˙‚ÌÓÓ‰ÒÚ‚ÂÌË ËÁÓ·ÚÌË „ÛÔË. ÑÓÒ„‡ Ò‡ ÓÔËÒ‡ÌË 8 ‡Á΢ÌË ‰ÂÏËÂÎËÌËÁË‡˘Ë/ıËÔÓÏËÂÎËÌËÁË‡˘Ë ÙÓÏË Ì‡ ò‡ÍÓå‡Ë-íÛÚ Ò ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ, ‚Òfl͇ ‰̇ Ò˙Ò ÒÔˆËÙ˘ÂÌ ÙÂÌÓÚËÔ Ë Í‡ÚË‡Ì‡ ‚ ÓÔ‰ÂÎÂÌ „ÂÌÂÚ˘ÂÌ ÎÓÍÛÒ. ïÂ‰ËÚ‡̇ڇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ êÛÒ  ÚÂÚ‡, ÌÓ‚ÓÓÚÍËÚ‡, Ò‰ ·˙΄‡ÒÍËÚ ÓÏË, ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl Ò ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. Ç Ò‡‚ÌÂÌËÂ Ò ‰Û„ËÚ ‰‚ Ì‚ÓÔ‡ÚËË (ïÂ‰ËÚ‡̇ڇ ÏÓÚÓ̇ Ë ÒÂÌÁÓ̇ Ì‚ÓÔ‡ÚËfl ÚËÔ ãÓÏ Ë ÒË̉Óχ ̇ äÓÌ„ÂÌËÚ‡Î̇ ͇ڇ‡ÍÚ‡, Îˈ‚ ‰ËÁÏÓÙËÁ˙Ï Ë Ì‚ÓÔ‡ÚËfl) Úfl Ëχ ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-Í˙ÒÂÌ ‰Â·˛Ú, Ì  ÔÓÒÚÓflÌÌÓ ‡ÒÓˆËË‡Ì‡ Ò ‰Û„Ë ÍÎËÌ˘ÌË ÔËÁ̇ˆË Ë Ëχ ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-‰Ó·Ó͇˜ÂÒÚ‚ÂÌ ÍÎËÌ˘ÂÌ ıÓ‰. ëΉ ˉÂÌÚËÙˈË‡ÌÂÚÓ Ì‡ ïåëçê ‚ Å˙΄‡Ëfl ‚ ÔÓÒΉÒÚ‚Ë Ò ‰Ë‡„ÌÓÒÚˈË‡Ú Ô‡ˆËÂÌÚË Ò˙Ò Ò˙˘ÓÚÓ Á‡·ÓÎfl‚‡Ì ÓÚ êÛÏ˙ÌËfl, àÒÔ‡ÌËfl Ë î‡ÌˆËfl (1, 3, 7). ïåëçê Ô‰ÒÚ‡‚Îfl‚‡ ‰ËÒÚ‡Î̇ ÒÂÌÁÓ-ÏÓÚÓ̇ Ì‚ÓÔ‡ÚËfl Ò Ì‡˜‡ÎÓ ‚ Í‡fl ̇ Ô˙‚‡Ú‡ ËÎË ‚ ̇˜‡ÎÓÚÓ Ì‡ ‚ÚÓ‡Ú‡ ‰Â͇‰‡ ̇ ÊË‚ÓÚ‡. çflχ ‰Û„‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒ˂̇ Ì‚ÓÔ‡ÚËfl, ÍÓflÚÓ ‰‡ Á‡ÔÓ˜‚‡ ÚÓÎÍÓ‚‡ Í˙ÒÌÓ. èË ‚Ò˘ÍË ÒÎÛ˜‡Ë Úfl Ò ÔˉÛʇ‚‡ ÓÚ ‰ÂÙÓÏËÚÂÚË ‚ ıӉ˷ڇ, ÔÓ„ÂÒË‡˘Ë Ò Û‚Â΢‡‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡ ̇ Ô‡ˆËÂÌÚËÚÂ. äÎËÌ˘ÌËflÚ ıÓ‰ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÛÏÂÂÌÓ ÔÓ„ÂÒË‡˘, Ò ÔÓ-Í˙ÒÌÓ Á‡Òfl„‡Ì ̇ „ÓÌËÚ Í‡ÈÌˈË. Ç ˜ÂÚ‚˙Ú‡Ú‡ ËÎË ÔÂÚ‡Ú‡ ‰Â͇‰‡ ‚˙ÁÌËÍ‚‡ ÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl, Ô˘ËÌÂ̇ ÓÚ ÚÓÚ‡Î̇ڇ Ô‡‡ÎËÁ‡ ̇


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

ÏÛÒÍÛÎËÚ ÔÓ‰ ÍÓÎÂ̇ڇ, ‡ ‚ ÏÌÓ„Ó ÒÎÛ˜‡Ë Ë ÔÓ‰ ·ÍÚËÚÂ. Ö‰ÌÓ‚ÂÏÂÌÌÓÚÓ Á‡Òfl„‡Ì ̇ ‡ÌÚÂÓ-·ÚÂ‡ÎÌËÚÂ Ë Á‡‰ÌËÚ ÏÛÒÍÛÎË Ì‡ ÔÓ‰·Â‰ˈËÚ ̇ÔÓÏÌfl Ôӂ˜ Á‡ ÚËÔ‡ Á‡Òfl„‡Ì ÔË ïåëç II, ÓÚÍÓÎÍÓÚÓ ïåëç I. ëÂÚË‚ÌËÚ ̇Û¯ÂÌËfl Ò‡ ÒËÎÌÓ ËÁ‡ÁÂÌË Ë ÔË 2 ÒÎÛ˜‡fl Ò ‡Á‚Ë‚‡ Ì‚ÓÔ‡Ú̇ ‰Â„ÂÌÂ‡ˆËfl ̇ „ÎÂÁÂÌÌËÚ ÒÚ‡‚Ë. íËÔ˘ÌË ‡ÍÓ‰ËÒÚÓÙ˘ÌË ÔÓÏÂÌË Ì Ò ̇·Î˛‰‡‚‡Ú, ‚˙ÔÂÍË ˜Â ÔË Â‰ËÌ Ô‡ˆËÂÌÚ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‰‡ÌÌË Á‡ ÔÓÒÚÓflÌÌË ÏÌÓÊÂÒÚ‚ÂÌË ·ÂÁ·ÓÎÍÓ‚Ë Ì‡‡Ìfl‚‡ÌËfl ‚ ‰ÓÎÌËÚ Í‡ÈÌˈË, ‡ ÔË ‰Û„ – ‰‡ÌÌË Á‡ ıÓÌ˘ÂÌ ÓÒÚÂÓÏËÂÎËÚ Ì‡ ‰̇ڇ ÔÓ‰·Â‰ˈ‡. ãÂÍÓ Á‡Òfl„‡Ì ̇ ˜ÂÂÔÌÓ-ÏÓÁ˙˜ÌË ÌÂ‚Ë Â Ì‡Îˈ ÔË 3 ÒÎÛ˜‡Ë. ç ҇ ̇·Î˛‰‡‚‡ÌË Ì‡Û¯ÂÌËfl ̇ ‡‚ÚÓÌÓÏÌËÚ ÙÛÌ͈ËË. ᇷÓÎfl‚‡ÌÂÚÓ Â Ò ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. Dyck Ë Lambert (1968), ͇ÍÚÓ Ë Harding Ë Thomas (1980) ËÁÒΉ‚‡Ú ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌË ÙÓÏË Ì‡ ïåëç I Ë II Ë Á‡Íβ˜‡‚‡Ú, ˜Â Ú ҇ Ò˙Ò Á̇˜ËÚÂÎÌÓ ÔÓ-ÚÂÊÍÓ ÔÓÚ˘‡ÌÂ, ÓÚÍÓÎÍÓÚÓ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌËÚ ÙÓÏË (2, 4). êÂÁÛÎÚ‡ÚËÚ ÓÚ ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌËÚ ËÁÒΉ‚‡ÌËfl ÔË ïåëçê Ò‡ ÌÂÓ·ËÍÌÓ‚ÂÌË. ëÌËÊÂÌËÂÚÓ Ì‡ ëè ÔÓ ÏÓÚÓÌËÚ ‚·Í̇  ÔÓ-„ÓÎflÏÓ ÓÚ Ó˜‡Í‚‡ÌÓÚÓ. íÓ‚‡ ÏÓÊ ‰‡ Ò ‰˙ÎÊË Ì‡ ‰ËÙÛÁ̇ ıËÔÓÏËÂÎËÌËÁ‡ˆËfl, ÍÓflÚÓ Ò ̇·Î˛‰‡‚‡ ‚ Ì‚‡ÎÌËÚ ·ËÓÔÒËË, χ͇ ˜Â Úfl  ҂˙Á‡Ì‡ Ò „ÂÌÂ‡ˆËfl ̇ ÌÂ‚ÌËÚ ‚·Í̇. ÇËÒÓÍËflÚ Ô‡„ Á‡ ÂÎÂÍÚ˘̇ ÒÚËÏÛ·ˆËfl ̇ ÔÂËÙÂÌËÚ ÌÂ‚Ë Â ı‡‡ÍÚÂ̇ ÂÎÂÍÚÓÙËÁËÓÎӄ˘̇ ÔÓfl‚‡ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ífl ÏÓÊ ‰‡ Á‡‚ËÒË ÓÚ ÏÛÒÍÛÎ̇ڇ ÂËÌÂ‚‡ˆËfl ÓÚ ıËÔÓÏËÂÎËÌËÁË‡ÌËÚ ‚·Í̇ Ò Ï‡Î˙Í Í‡ÎË·˙, ÌÓ ÏÓÙÓÎӄ˘ÌËÚ ı‡‡ÍÚÂËÒÚËÍË ‚ ÔÓ-ÔÓÍÒËχÎÌËÚ ÌÂ‚ÌË ÒÚ‚ÓÎӂ ̇ ÚÓÁË ÂÚ‡Ô Ò‡ ÌÂËÁ‚ÂÒÚÌË. ÑËÙÛÁ̇ ıËÔÓÏËÂÎËÌËÁ‡ˆËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‚ ÒÎÛ˜‡ËÚ ̇ ·ÓÎÂÒÚ Ì‡ Leigh, ÓÔË҇̇ ÓÚ Jacobs Ë Ò˙Ú. (1990) (5), ͇ÍÚÓ Ë ÔË ÒË̉Óχ ̇ ÍÓÌ„ÂÌËÚ‡Î̇ ͇ڇ‡ÍÚ‡ Îˈ‚ ‰ËÁÏÓÙËÁ˙Ï Ì‚ÓÔ‡ÚËfl (8), ÔË ÍÓËÚÓ ëè ÔÓ ÏÓÚÓÌËÚ ‚·Í̇ Ò‡ ÔÓ ÔÓ‰Ó·ÂÌ Ì‡˜ËÌ ÒÌËÊÂÌË. ïåëçê Â Ò ÍÎËÌ˘̇ ͇ÚË̇ ̇ ÚÂÊ͇ ÔÓ„ÂÒË‡˘‡ ÒÂÌÁÓÏÓÚÓ̇ Ì‚ÓÔ‡ÚËfl Ò ÔÓ‰˜ÂÚ‡ÌË ÒÂÚË‚ÌË Ì‡Û¯ÂÌËfl, ‡ÒÓˆËË‡ÌË Ò “ÏÂʉËÌÌÓ“ ÒÌËÊÂÌË ̇ ëè. í‡Í‡-

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‚‡ ÍÎËÌ˘̇ ͇ÚË̇ Ì  ÓÔËÒ‚‡Ì‡ ‰ÓÒ„‡ Ë fl ÓÚ΢‡‚‡ ÓÚ ‰Û„ËÚ ÙÓÏË Ì‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌË ïåëç. ä‡ÍÚÓ ÔË ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌËÚ ÙÓÏË Ì‡ ïåëç, ڇ͇ Ë ÔË ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌËÚ ÌÂÔÂÍ˙Ò̇ÚÓ Ò ‡Á¯Ëfl‚‡ ÍÎËÌ˘ÌËfl ÒÔÂÍÚ˙. ê‡Á¯ËÂÌËflÚ ÒÔÂÍÚ˙ ̇ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌËÚ ïåëç ÓÚ‡Áfl‚‡ Ò˙˘ÂÒÚ‚Û‚‡ÌÂÚÓ Ì‡ ‡Á΢ÌË ÔÓ‰ÎÂʇ˘Ë Ô‡ÚÓ„ÂÌÂÚ˘ÌË ÏÂı‡ÌËÁÏË. ãàíÖêÄíìêÄ 1. Colomer J, Iturriaga C, Kalaydjieva L, et al: HMSN-Russe in two Spanish patients: distinctive features of the disease and current genetic findings. Acta Myologica 2001, 20, 202-210. 2. Dyck PJ, Lambert EH. Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. I. Neurologic, genetic, and electrophysiologic findings in hereditary polyneuropathies. Arch Neurol. 1968, 18, 603-18. 3. Hantke J, Rogers T, French L, Tournev I, Guergelcheva V, Urtizberea JA, Colomer J, Corches A, Lupu C, Merlini L, Thomas PK, Kalaydjieva L. Refined mapping of the HMSNR critical gene region – construction of a high-density integrated genetic and physical map. Neuromusc Disord 2003, 13, 729-736. 4. Harding AE, Thomas PK. The clinical features of hereditary motor and sensory neuropathy types I and II. Brain. 1980, 103, 259-280. 5. Jacobs JM, Harding BN, Lake BD, Payan J, Wilson J. Peripheral neuropathy in Leigh's disease. Brain. 1990, 113, 447-62. 6. Rogers T., D. Chandler, D. Angelicheva, P. Thomas, B. Youl, I. Tournev, V. Guergueltcheva, L. Kalaydjieva. A novel locus for autosomal recessive peripheral neuropathy in the EGR2 region on 10q23. Am J Hum Genet 2000, 67, 664-671. 7. Thomas P., Kalaydjieva L., Youl B., Rogers T., Angelicheva D., King R., Guergueltcheva V., Colomer J., Lupu C., Corches A., Popa G., Merlini L., Shmarov A., Muddle J., Nourallah M., Tournev I. Hereditary motor and sensory neuropathy-Russe: new autosomal recessive neuropathy in Balkan Gypsies. Ann Neurol 2001, 50, 452-457. 8. Tournev I., L. Kalaydjieva, B.Youl, B. Ishpekova, V. Guergueltcheva, O. Kamenov, M. Katzarova, Z. Kamenov, M. Raicheva-Terzieva, R.H.M. King, K. Romanski, R. Petkov, A. Schmarov, G.Dimitrova, N.Popova, M.Uzunova, S.Milanov, J. Petrova, I.Petkov, G.Kolarov, L.Aneva, O.Radeva & P.K.Thomas. The Congenital Cataracts Facial Dysmorphism Neuropathy (CCFDN) syndrome: a novel complex genetic disease in Balkan Gypsies. Annals of Neurology, 1999, 45, 742-750.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÑÓˆ. ‰- à‚‡ÈÎÓ í˙Ì‚, ‰.Ï.Ì. äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡ “ÄÎÂÍ҇̉Ó‚Ò͇” ÅÛÎ. “ë‚. É. ëÓÙËÈÒÍË” ‹1, ëÓÙËfl 1431 íÂÎ. (02) 9230 752 E-mail: emhpf@techno-link.com

éË„Ë̇ÎÌË ÒÚ‡ÚËË äÄêéíàÑçà èãÄäà à ëÖêìåçà ãàèàÑà Ö. LJÒË΂‡ , É. ÉÂ̘‚1 ç‚ÓÎӄ˘̇ ÍÎËÌË͇ åÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇” 1ëӈˇÎ̇ ωˈË̇

SUMMARY CAROTID ARTERY PLAQUES AND BLOOD LIPIDS E.Vassileva, G. Genchev Ultrasound-assessed plaque morphology is an independent predictor of ischemic stroke. The relationship between atherosclerotic carotid artery plaque echogenicity and the level of the blood lipids (total cholesterol, low density (LDL), high density (HDL) lipoprotein, triglycerides) as well as the most common cerebrovascular risk factors was investigated. 150 consecutive patients with carotid artery atherosclerosis were enrolled in the study. All patients underwent color coded duplex sonography of the carotid and vertebral arteries. The internal carotid artery plaques were classified as echogenic (n=100) and echolucent (n=40). The aging, gender, the presence of arterial hypertension, diabetes mellitus, cigarette smoking and history of ischemic stroke or transient ischemic attacks were recorded. Logistic regression was used to evaluate relationship between the type of the plaque (echogenic or echolucent) and the level of total cholesterol, LDL cholesterol,

HDL cholesterol, triglycerides and the other cerebrovascular risk factors. Low level of HDL cholesterol is significantly associated with echolucent atherosclerotic plaques. Low levels of HDL cholesterol predict the appearance of echolucent rupture-prone plaques. LDL cholesterol and triglycerides were not related to plaque echogenicity. The other risk factors diabetes, arterial hypertension, smoking were not related to plaque morphologies. Key words: color coded duplex sonography, echogenic and echolucent plaque, cerebrovascular risk factors. êÖáûåÖ åÓÙÓÎÓ„ËflÚ‡ ̇ ͇ÓÚˉÌËÚ ‡ÚÂÓÒÍÎÂÓÚ˘ÌË Ô·ÍË, ÓˆÂÌÂ̇ ˜ÂÁ ˆ‚ÂÚ̇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl  ҂˙Á‡Ì‡ Ò ËÒ͇ ÓÚ ‚˙ÁÌËÍ‚‡Ì ̇ ËÒıÂÏ˘ÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË. ñÂΠ̇ ÔÓÛ˜‚‡ÌÂÚÓ Â ËÁÒΉ‚‡Ì ‚ÎËflÌËÂÚÓ Ì‡ ̇È‡ÁÔÓÒÚ‡ÌÂÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl (åëÅ) (Ó·˘ ıÓÎÂÒÚÂÓÎ, ıÓÎÂÒÚÂÓÎ ‚ ÎËÔÓÔÓÚÂËÌË Ò ‚ËÒÓ͇ ÔÎ˙ÚÌÓÒÚ (ãÇè ıÓÎÂÒÚÂÓÎ), ıÓ-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÎÂÒÚÂÓÎ ‚ ÎËÔÓÔÓÚÂËÌË Ò ÌËÒ͇ ÔÎ˙ÚÌÓÒÚ (ãçè ıÓÎÂÒÚÂÓÎ), ÚË„ÎˈÂˉË) ‚˙ıÛ Ô·ÍÓ‚‡Ú‡ ÂıÓ„ÂÌÌÓÒÚ. Ç ÔÓÛ˜‚‡ÌÂÚÓ Ò‡ ‚Íβ˜ÂÌË 150 Ô‡ˆËÂÌÚ‡ Ò Í‡ÓÚˉ̇ ‡ÚÂÓÒÍÎÂÓÁ‡, ÔË ÍÓËÚÓ Â ÓÒ˙˘ÂÒÚ‚Â̇ ˆ‚ÂÚ̇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ̇ ͇ÓÚˉÌË Ë ‚ÂÚ·‡ÎÌË ‡ÚÂËË. ÄÚÂÓÒÍÎÂÓÚ˘ÌËÚ Ô·ÍË Ì‡ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl Ò‡ Í·ÒË‡ÌË Í‡ÚÓ ıËÔÂÂıÓ„ÂÌÌË (n=110) Ë ıËÔÓÂıÓ„ÂÌÌË (n=40). ÑÓÍÛÏÂÌÚË‡ÌË Ò‡ ÒΉÌËÚ ̇ȘÂÒÚÓ Ò¢‡ÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ ‚ ËÁÒΉ‚‡Ì‡Ú‡ „ÛÔ‡ – ‚˙Á‡ÒÚ, ÔÓÎ, ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl, ‰Ë‡·ÂÚ, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ, ‰ËÁÎËÔˉÂÏËfl, ͇ÍÚÓ Ë ‡Ì‡ÏÌÂÒÚ˘ÌËÚ ‰‡ÌÌË Á‡ ÔÂ͇‡Ì ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ËÎË ÔÂıÓ‰ÌÓ Ì‡Û¯ÂÌË ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ. ãÓ„ËÒÚ˘̇ „ÂÒËfl  ËÁÔÓÎÁ‚‡Ì‡ Á‡ ÍÓ·ˆËfl ÏÂÊ‰Û ‚ˉ‡ ̇ Ô·͇ڇ (ıËÔÂÂıÓ„ÂÌ̇ ËÎË ıËÔÓÂıÓ„ÂÌ̇) Ë ÒÂÛÏÌÓÚÓ ÌË‚Ó Ì‡ Ó·˘Ëfl ıÓÎÂÒÚÂÓÎ, ãÇè ıÓÎÂÒÚÂÓÎ, ãçè ıÓÎÂÒÚÂÓÎ, ÚË„ÎˈÂË‰Ë Ë ‰Û„ËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ. çËÒÍÓÚÓ ÌË‚Ó Ì‡ ãÇè ıÓÎÂÒÚÂÓ·  ҂˙Á‡ÌÓ ÒË„ÌËÙË͇ÌÚÌÓ Ò ‚˙ÁÌËÍ‚‡Ì ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË. çËÒÍÓÚÓ ÌË‚Ó Ì‡ çÑã ıÓÎÂÒÚÂÓ· ‰ÓÔË̇Òfl Á‡ ÙÓÏË‡Ì ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË. çË‚ÓÚÓ Ì‡ ãÑã Ë ÚË„ÎˈÂˉËÚ Ì ‚ÎËfl ‚˙ıÛ ÔÎ˙ÚÌÓÒÚÚ‡ ̇ Ô·͇ڇ. ÑÛ„ËÚ ËÁÒΉ‚‡ÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË – ‚˙Á‡ÒÚ, ÔÓÎ, ‰Ë‡·ÂÚ, ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl, Ú˛Ú˛ÌÓÔÛ¯ÂÌ ÌflÏ‡Ú ‚˙Á͇ Ò Ô·ÍÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëfl. äβ˜Ó‚Ë ‰ÛÏË: ˆ‚ÂÚ̇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl, ıËÔÂÂıÓ„ÂÌÌË Ë ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË, ËÒÍÓ‚Ë Ù‡ÍÚÓË. ։̇ ÓÚ „·‚ÌËÚ Ô˘ËÌË Á‡ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ Â Ó·ÒÚÛÍÚË‚ÌËflÚ ‡ÚÂˇÎÂÌ ÔÓˆÂÒ Ì‡ χ„ËÒÚ‡ÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË Ò ‡Á‚ËÚË ̇ ‡ÚÂˇÎÌË ÒÚÂÌÓÁË, ÚÓÏ·ÓÁË Ë ÏÓÁ˙˜ÂÌ ÂÏ·ÓÎËÁ˙Ï. ÄÚÂÓÒÍÎÂÓÁ‡Ú‡  ÒÎÓÊÂÌ ÔÓˆÂÒ, ‚ ÓÒÌÓ‚‡Ú‡ ̇ ÍÓÈÚÓ ÎÂÊË ẨÓÚÂÎ̇ڇ Û‚‰‡. ífl ÏÓÊ ‰‡ ·˙‰Â Ô‰ËÁ‚Ë͇̇ ÓÚ ‡Á΢ÌË Ù‡ÍÚÓË Ò ÔÓÒΉ‚‡˘Ë ÔÓÏÂÌË, ‚Ó‰Â˘Ë ‰Ó ̇Û¯Â̇ ÔÓ‰Û͈Ëfl ̇ ˆËÚÓÍËÌË, ‡‰ıÂÁËÓÌÌË ÏÓÎÂÍÛÎË, ‡ÒÚÂÊÌË Ù‡ÍÚÓË. Ö̉ÓÚÂÎ̇ڇ Û‚‰‡ Á‡ÔÓ˜‚‡ Ò Û‚Â΢ÂÌ ÔÂχ·ËÎËÚÂÚ Ì‡ Ò˙‰Ó‚‡Ú‡ ·‡ËÂ‡, ‚Ӊ¢ ‰Ó ÓÒ‚Ó·Óʉ‡‚‡Ì ̇ Ò‚Ó·Ó‰ÌË ‡‰Ë͇ÎË Ë ÓÍËÒÎfl‚‡Ì ̇ Ô·ÁÏÂÌËÚ ÎËÔÓÔÓÚÂËÌË Ò ÔÓÒΉ‚‡˘‡ Ú‡ÌÒÙÓχˆËfl ̇ χÍÓÙ‡„ËÚ ‚ ÔÂÌÂÒÚË ÍÎÂÚÍË Ë ÙÓÏË‡Ì ̇ ÎËÔˉÌË ÔÂÚ̇ (1). éÙÓÏflÚ Ò ‡ÚÂÓχÚÓÁÌË Ô·ÍË, ÍÓËÚÓ ËÏ‡Ú ‡Á΢ÌÓ ÎËÔˉÌÓ Ò˙‰˙ÊËÏÓ, ‡Á΢ÌÓ Ò˙‰˙ʇÌË ̇ ÙË·ÓÁ̇ Ú˙͇Ì, ‚˙ÚÂÔ·ÍÓ‚Ë ıÂÏÓ‡„ËË Ë Í‡ÎˆËÂ‚Ë ÓÚ·„‡ÌËfl. Ç Á‡‚ËÒËÏÓÒÚ ÓÚ ÚÓ‚‡ ÔË ‰ÓÔÎÂÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì ̇ ÏÓÁ˙˜ÌËÚ ‡ÚÂËË, ‡ÚÂÓÒÍÎÂÓÚ˘ÌËÚ Ô·ÍË Ò ‚ËÁÛ‡ÎËÁË‡Ú ͇ÚÓ ıËÔÂÂıÓ„ÂÌÌË Ë ıËÔÓÂıÓ„ÂÌÌË. ïËÔÂıÓÎÂÒÚÂÓÎÂÏËflÚ‡  ‚‡ÊÂÌ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ‡ÚÂÓÒÍÎÂÓÁ‡. ÄÍÛÏÛ·ˆËflÚ‡ ̇ ÎËÔÓÔÓÚÂËÌË ‚ ËÌÚËχڇ Á‡‚ËÒË ÓÚ ·‡Î‡ÌÒ‡ ÏÂÊ‰Û ‚ıÓ‰ÌÓÚÓ ËÁıÓ‰ÌÓÚÓ ÌË‚Ó Ë ÒÚÂÔÂÌÚ‡ ̇ ‰Â„‡‰‡ˆËfl ̇ ÎËÔÓÔÓÚÂËÌÓ‚ËÚ Ù‡ÍˆËË. ç‡ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì Ëχ Á‡ ˆÂÎ ËÁÒΉ‚‡Ì ‚ÎËflÌËÂÚÓ Ì‡ ̇È-˜ÂÒÚÓ Ò¢‡ÌËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl, ‚˙ıÛ Ô·ÍÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëfl – Ó·‡ÁÛ‚‡Ì ̇ ıËÔÂÂıÓ„ÂÌÌË ËÎË ıËÔÓÂıÓ„ÂÌÌË Í‡ÓÚˉÌË Ô·ÍË. äéçíàçÉÖçí àÁÒΉ‚‡ÌËflÚ ÍÓÌÚËÌ„ÂÌÚ Ò Ò˙ÒÚÓË ÓÚ 150 Ô‡ˆËÂÌÚ‡ – 50 (33,33%) Ï˙ÊÂ Ë 100 (66,67%) ÊÂÌË (ÙË„. 1) Ò˙Ò Ò‰̇ ‚˙Á‡ÒÚ 65,67±8,22 „Ó‰ËÌË, ‚ ‰Ë‡Ô‡ÁÓ̇ 45 - 80 „Ó‰ËÌË. ÇÒ˘ÍË ·ÓÎÌË Ò‡ ËÁÒΉ‚‡ÌË ˜ÂÁ ÂÍÒÚ‡Í‡ÌˇÎ̇ ÔÓÒÚÓflÌÌÓ‚˙ÎÌÓ‚‡ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl Ë ˆ‚ÂÚ̇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl. äËÚÂËË Á‡ ‚Íβ˜‚‡Ì ҇ ̇΢Ë-

155

îË„.1 ê‡ÁÔ‰ÂÎÂÌË ̇ Û˜‡ÒÚÌˈËÚ ‚ ÔÓÛ˜‚‡ÌÂÚÓ ÒÔÓ‰ ÔÓÎÓ‚‡Ú‡ ÔË̇‰ÎÂÊÌÓÒÚ. ÂÚÓ Ì‡ Ô·ÍË ‚ Ó·˘ËÚÂ, ‚˙Ú¯ÌËÚ ËÎË ‚˙̯ÌËÚ Ò˙ÌÌË ‡ÚÂËË Ë ÎËÔÒ‡ ̇ ‡ÌÚËÎËÔÂÏ˘ÌÓ Î˜ÂÌËÂ. åÖíéÑà ä‡ÓÚˉÌËÚ ‡ÚÂËË Ò‡ ËÁÒΉ‚‡ÌË Ò ˆ‚ÂÚÂÌ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚ ‡Ô‡‡Ú Image Point Agilent Technology Ò 10 MHz Ú‡ÌÒ‰˛ÒÂ. ÇÒ˘ÍË Ô·ÍË Ò‡ ‚ËÁÛ‡ÎËÁË‡ÌË ‚ Çmode ‚ Ô‰̇, ·ÚÂ‡Î̇ Ë Ì‡Ô˜̇ ‡‚ÌË̇. ëΉ ÚÓ‚‡  ËÁÔÓÎÁ‚‡ÌÓ Ë ˆ‚ÂÚÌÓ ÍÓ‰Ë‡Ì ̇ Í˙‚ÓÚÓ͇. Ç Á‡‚ËÒËÏÓÒÚ ÓÚ ÂıÓ„‡ÙÒÍËfl Ó·‡Á, Ô·ÍËÚ ҇ Í·ÒË‡ÌË Í‡ÚÓ ıËÔÓÂıÓ„ÂÌÌË (echolucent) Ë ıËÔÂÂıÓ„ÂÌÌË (echogenic) (2). ïËÔÓÂıÓ„ÂÌÌËÚ Ô·ÍË ËÏ‡Ú ÌËÒ͇ ÔÎ˙ÚÌÓÒÚ, ‰Ó͇ÚÓ ıËÔÂÂıÓ„ÂÌÌËÚ Ò˙‰˙Ê‡Ú Í‡ÎˆËÂ‚Ë ÓÚ·„‡ÚËfl Ë ÙË·ÓÁ̇ Ú˙͇Ì. ê„ËÒÚË‡ÌË Ò‡ ̇È-˜ÂÒÚËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ – ÔÓÎ, ‚˙Á‡ÒÚ, ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl, Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ, Á‡ÚÎ˙ÒÚfl‚‡ÌÂ. àÁÒΉ‚‡ÌË Ò‡ ÒΉÌËÚ Ù‡ÍˆËË Ì‡ ÎËÔˉ̇ڇ Ó·Ïfl̇ – ıÓÎÂÒÚÂÓÎ, ÎËÔÓÔÓÚÂËÌË Ò ‚ËÒÓ͇ ÔÎ˙ÚÌÓÒÚ (ãÇè ıÓÎÂÒÚÂÓÎ, ïéã-ãÇè), ÎËÔÓÔÓÚÂËÌË Ò ÌËÒ͇ Ô˙ÚÌÓÒÚ (ãçè ıÓÎÂÒÚÂÓÎ, ïéã – ãçè), ÚË„ÎˈÂˉË. ëíÄíàëíàóÖëäà åÖíéÑà чÌÌËÚ ·flı‡ ‚˙‚‰ÂÌË Ë Ó·‡·ÓÚÂÌË Ò˙Ò ÒÚ‡ÚËÒÚ˘ÂÒÍËfl Ô‡ÍÂÚ SPSS 12.0.1. ᇠÌË‚Ó Ì‡ Á̇˜ËÏÓÒÚ, ÔË ÍÓÂÚÓ Ò ÓÚı‚˙Îfl ÌÛ΂‡Ú‡ ıËÔÓÚÂÁ‡ ·Â ËÁ·‡ÌÓ p < 0,05. Åflı‡ ÔËÎÓÊÂÌË ÒΉÌËÚ ÏÂÚÓ‰Ë: 1. ÑËÒÍËÔÚË‚ÂÌ ‡Ì‡ÎËÁ – ‚ Ú‡·Î˘ÂÌ ‚ˉ  Ô‰ÒÚ‡‚ÂÌÓ ˜ÂÒÚÓÚÌÓÚÓ ‡ÁÔ‰ÂÎÂÌË ̇ ‡Á„ÎÂʉ‡ÌËÚ ÔËÁ̇ˆË, ‡Á·ËÚË ÔÓ „ÛÔË. 2. LJˇˆËÓÌÂÌ ‡Ì‡ÎËÁ – ËÁ˜ËÒÎfl‚‡Ì ӈÂÌÍËÚ ̇ ˆÂÌÚ‡Î̇ڇ ÚẨÂ̈Ëfl Ë ‡ÁÒÂÈ‚‡ÌÂ. 3. íÂÒÚ c2 Á‡ ÔÓ‚Â͇ ̇ ıËÔÓÚÂÁË Á‡ ̇΢ˠ̇ ‚˙Á͇ ÏÂÊ‰Û Í‡Ú„ÓËÈÌË ÔÓÏÂÌÎË‚Ë. 4. ÖÍÁ‡ÍÚÂÌ ÚÂÒÚ Ì‡ î˯Â Á‡ ÔÓ‚Â͇ ̇ ıËÔÓÚÂÁË Á‡ ̇΢ˠ̇ ‚˙Á͇ ÏÂÊ‰Û Í‡Ú„ÓËÈÌË ÔÓÏÂÌÎË‚Ë. 5. Ö‰ÌÓËÁ‚‡‰ÍÓ‚ ÏÂÚÓ‰ ̇ äÓÎÏÓ„ÓÓ‚-ëÏËÌÓ‚ – Á‡ ÓÔ‰ÂÎflÌ ‚ˉ‡ ̇ ‡ÁÔ‰ÂÎÂÌËÂÚÓ. 6. çÂÔ‡‡ÏÂÚ˘ÂÌ ÚÂÒÚ Ì‡ Mann-Whitney Á‡ ÔÓ‚Â͇ ̇ ıËÔÓÚÂÁË Á‡ ‡Á΢ˠÏÂÊ‰Û ‰‚ ÌÂÁ‡‚ËÒËÏË ËÁ‚‡‰ÍË. 7. ãÓ„ËÒÚ˘ÂÌ „ÂÒËÓÌÂÌ ‡Ì‡ÎËÁ Á‡ ÍÓ΢ÂÒÚ‚Â̇ ÓˆÂÌ͇ ̇ Ù‡ÍÚÓËÚÂ, ‚ÎËflÂ˘Ë ‚˙ıÛ Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË. êÖáìãíÄíà ïËÔÓÂıÓ„ÂÌÌË Ô·ÍË Ò ̇·Î˛‰‡‚‡Ú ÔË 40 (26,67%) ÓÚ Û˜‡ÒÚÌˈËÚÂ, ‡ ıËÔÂÂıÓ„ÂÌÌË - ÔË 110 (73,33%) ÓÚ Úflı (ÙË„. 2).


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

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Ò‡ Á‡ ıËÔÓÂıÓ„ÂÌ̇ Ô·͇ Ò ÓÍÓÎÓ 6%, Ú.Â. ‚˙Á‡ÒÚÚ‡ Ò fl‚fl‚‡ ÔÓÚÂÍÚË‚ÂÌ Ù‡ÍÚÓ Á‡ ‚˙ÁÌËÍ‚‡Ì ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË.

îË„.2 ê‡ÁÔ‰ÂÎÂÌË ̇ Û˜‡ÒÚÌˈËÚ ‚ ÔÓÛ˜‚‡ÌÂÚÓ ÒÔÓ‰ ‚ˉ‡ ̇ Ô·ÍËÚÂ. ç‡ Ú‡·Î. 1 Ò‡ Ô‰ÒÚ‡‚ÂÌË ÂÁÛÎÚ‡ÚËÚ ÓÚ Ò‡‚ÌËÚÂÎÌËfl ‡Ì‡ÎËÁ ̇ ‚˙Á‡ÒÚÚ‡ ÔË ‰‚ÂÚ „ÛÔË - Ò ıËÔÓ- Ë ıËÔÂÂıÓ„ÂÌÌË Ô·ÍË. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ò ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË Ò Ó͇Á‡ ÔÓ-ÌËÒ͇, ÌÓ ‡ÁÎË͇ڇ Ìflχ ÒÚ‡ÚËÒÚ˘ÂÒ͇ ÒË„ÌËÙË͇ÌÚÌÓÒÚ, ÍÓÂÚÓ ÓÁ̇˜‡‚‡, ˜Â ÔÓ ÚÓÁË ÔÓ͇Á‡ÚÂÎ ‰‚ÂÚ „ÛÔË Ò‡ Û‰̇͂ÂÌË. íÓ‚‡ ÓÚ Ò‚Ófl ÒÚ‡Ì‡  ‰Ó·‡ ·‡Á‡ Á‡ ÓˆÂÌ͇ ̇ ‚ÎËflÌËÂÚÓ Ì‡ ÓÒڇ̇ÎËÚ ËÁÒΉ‚‡ÌË Ù‡ÍÚÓË. êÂÁÛÎÚ‡ÚËÚ ÓÚ Ò‡‚ÌËÚÂÎÌËfl ‡Ì‡ÎËÁ ̇ ͇Ú„ÓËÈÌËÚ ÔËÁ̇ˆË  ÔÓ͇Á‡Ì ̇ Ú‡·Î. 2. éÚ ÌÂfl Ò ‚Ëʉ‡, ˜Â ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË Ò ̇·Î˛‰‡‚‡Ú ÔÓ-˜ÂÒÚÓ ÔË ÊÂÌËÚ - 67,5% ÒÔflÏÓ 66,4%, ÔË ÌÓχÎ̇ ıÓÎÂÒÚÂÓÎÂÏËfl – 40,0% ÒÂ˘Û 31,8%, ÔË Û‚Â΢ÂÌ ïéã – ãçè – 53,8%

éÅëöÜÑÄçÖ ë ӄΉ ̇‡ÒÚ‚‡˘Ëfl ·ÓÈ Ô‡ˆËÂÌÚË Ò ‚ËÒÓÍ ËÒÍ ÓÚ Ò˙‰Ó‚Ë ÛÒÎÓÊÌÂÌËfl, ‚ Âʉ̂̇ڇ ÍÎËÌ˘̇ Ô‡ÍÚË͇ Á‡ ÔÓÙË·ÍÚË͇ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ËÚ Á‡·ÓÎfl‚‡ÌËfl ‚Ò ÔÓ¯ËÓÍÓ Ò ËÁÔÓÎÁ‚‡Ú ÌÂËÌ‚‡ÁË‚ÌË ÏÂÚÓ‰Ë Á‡ ÚflıÌÓÚÓ Ë‰ÂÌÚËÙˈË‡ÌÂ. Ö‰ËÌ ÓÚ ÚÂÁË ÏÂÚÓ‰Ë Â ‰ÓÔÎÂÒÓÌÓ„‡ÙÒ͇ڇ ‰Ë‡„ÌÓÒÚË͇ ̇ ͇ÓÚˉ̇ڇ ‡ÚÂÓÒÍÎÂÓÁ‡, ÍÓflÚÓ ‚Íβ˜‚‡ ÓÔ‰ÂÎflÌ ÔÓˆÂÌÚ‡ ̇ ͇ÓÚˉ̇ڇ ÒÚÂÌÓÁ‡, ı‡‡ÍÚÂËÁË‡Ì ÔÎ˙ÚÌÓÒÚÚ‡ ̇ ͇ÓÚˉ̇ڇ Ô·͇, ËÁÏÂ‚‡Ì ‰Â·ÂÎË̇ڇ ̇ ËÌÚËχ-ωËfl ÍÓÏÔÎÂÍÒ‡ Ë ‰. Spence et al ÔÓ͇Á‚‡Ú ÌÓ‚ ÏÂÚÓ‰ Á‡ ˉÂÌÚËÙˈË‡Ì ̇ ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË - ËÁÏÂ‚‡Ì „ÓÎÂÏË̇ڇ ̇ ͇ÓÚˉ̇ڇ ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ô·͇ Ë ÓÔ‰ÂÎflÌ ÌÂÈ̇ڇ ÔÓ„ÂÒËfl (1). ÅÓÎÌËÚÂ Ò Ô·ÍÓ‚‡ ÔÓ„ÂÒËfl ‡Á‚Ë‚‡Ú 2,1 Ô˙ÚË ÔÓ-˜ÂÒÚÓ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ÏËÓ͇‰ÂÌ ËÌÙ‡ÍÚ ËÎË Ò˙‰Ó‚‡ ÒÏ˙Ú ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË, ÔË ÍÓËÚÓ Ì Ò ̇·Î˛‰‡‚‡ ̇‡ÒÚ‚‡Ì ̇ Ô·͇ڇ. ÑÛ„Ë ‰ÓÔ˙ÎÌËÚÂÎÌË ÌÂËÌ‚‡ÁË‚ÌË ÏÂÚÓ‰Ë Á‡ ÓÚÍË‚‡Ì ̇ ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË Ò‡ ËÁÏÂ‚‡Ì ‰Â·ÂÎË̇ڇ ̇ ËÌÚËχ-ωËfl ÍÓÏÔÎÂÍÒ‡ /intima-media ticknes/ ͇ÍÚÓ Ë ÓÔ‰ÂÎflÌ ÏÓÙÓÎÓ„ËflÚ‡ ̇ Ô·͇ڇ. ä‡ÍÚÓ Â ËÁ‚ÂÒÚÌÓ, Ì ҇ÏÓ ÒÚÂÔÂÌÚ‡ ̇ ͇ÓÚˉ̇ڇ ÒÚÂÌÓÁ‡, ÌÓ Ë Ô·ÍÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëfl  ÓÚ Á̇˜ÂÌË Á‡ ‚˙ÁÌËÍ‚‡Ì ̇ ËÒıÂÏ˘ÌË ËÌÒÛÎÚË (3-8). ç‡Î˘ËÂÚÓ Ì‡ Ô·ÍÓ‚Ë ÛΈÂ‡ˆËË Ë ÔËÒÚÂÌÌË Ô·ÍÓ‚Ë ÚÓÏ·Ë Ò‡ ÓÒÌÓ‚ÂÌ ËÁÚÓ˜ÌËÍ Ì‡ ÏÓÁ˙˜ÂÌ ÏËÍÓÂÏ·ÓÎËÁ˙Ï (9). çÂËÌ‚‡ÁË‚ÌËÚ ÏÂÚÓ‰Ë Ó·‡˜Â ‚ ÚÓ‚‡ ˜ËÒÎÓ ‰ÓÔÎÂÓ‚‡Ú‡ ÒÓÌÓ„‡ÙËfl ÌflÏ‡Ú ‰ÓÒÚ‡Ú˙˜ÌË ‰Ë‡„ÌÓÒ-

퇷Îˈ‡ 1: ë‡‚ÌËÚÂÎÂÌ ‡Ì‡ÎËÁ ̇ ‚˙Á‡ÒÚÚ‡ ÔË ‰‚ÂÚ ËÁÒΉ‚‡ÌË „ÛÔË ÖıÓ„ÂÌÌË Ô·ÍË èËÁÌ‡Í Ç˙Á‡ÒÚ („Ó‰ËÌË)

N 40

X 63,10

ïËÔÓ SD 9,04

Min 45,00

Max 77,00

ÒÂ˘Û 38,3%, ÔË Ì‡Ï‡ÎÂÌ ïéã - ãÇè – 67,5% ÒÂ˘Û 20,0%, ÔË Û‚Â΢ÂÌË ÚË„ÎˈÂË‰Ë – 40,0% ÒÂ˘Û 36,4%, ÔË ·ÓÎÌËÚÂ Ò ‰Ë‡·ÂÚ – 15,0% ÒÂ˘Û 13,6%, ÔË ·ÓÎÌËÚÂ Ò ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl – 65,0% ÒÂ˘Û 53,6%, ÔË ÌÂÔÂ͇‡ÎËÚ ËÌÒÛÎÚ– 67,5% ÒÂ˘Û 64,2%, ÔË ÔÛ¯‡˜ËÚ – 7,5% ÒÂ˘Û 4,5%. éÚ ÒÚ‡ÚËÒÚ˘ÂÒ͇ „Ή̇ ÚӘ͇ ‰ËÌÒÚ‚ÂÌËflÚ ÒË„ÌËÙË͇ÌÚÂÌ Ù‡ÍÚÓ Ò Ó͇Á‡ ̇χÎÂÌËflÚ ïéã-ãÇè (p<0.001). ãéÉàëíàóÖç êÖÉêÖëàéçÖç ÄçÄãàá óÂÁ ̇Ô‡‚ÂÌËÚ ‰Ó ÚÛÍ ‡Ì‡ÎËÁË ·Â¯Â ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â ÓÚ ËÁÒΉ‚‡ÌËÚ ÔËÁ̇ˆË ‰ËÌÒÚ‚ÂÌÓ Ì‡Ï‡ÎÂÌËflÚ ãÇè ıÓÎÂÒÚÂÓÎ ÏÓÊ ‰‡ Ò ҘËÚ‡ Á‡ ÒË„ÌËÙË͇ÌÚÂÌ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË. ᇠ‰‡ Ò ̇Ô‡‚Ë ÍÓ΢ÂÒÚ‚Â̇ ÓˆÂÌ͇ ̇ ‚ÎËflÌËÂÚÓ Ì‡ ÚÓÁË Ù‡ÍÚÓ ·Â¯Â ÔËÎÓÊÂÌ ·Ë̇ÂÌ ÎÓ„ËÒÚ˘ÂÌ „ÂÒËÓÌÂÌ ‡Ì‡ÎËÁ. éÚ Ú‡·Î. 3 Ò ‚Ëʉ‡, ˜Â ̇΢ËÂÚÓ Ì‡ ̇χÎÂÌ ïéããÇè Û‚Â΢‡‚‡ ËÒ͇ Á‡ Ó·‡ÁÛ‚‡Ì ̇ ıËÔÓÂıÓ„ÂÌ̇ Ô·͇ ̇‰ 8 Ô˙ÚË ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË, ÍÓËÚÓ ËÏ‡Ú ÌÓχÎÂÌ ãÇè ıÓÎÂÒÚÂÓÎ. ëΉ ÔÓÒÚ‡‚flÌ ‚ „ÂÒËÓÌÌÓÚÓ Û‡‚ÌÂÌË ̇ ËÁ‚ÂÒÚÌËÚ Á‡Ï˙„Îfl‚‡˘Ë Ù‡ÍÚÓË ‚˙Á‡ÒÚ Ë ÔÓÎ, Á‡ ‰‡ ·˙‰Â ÓÚ˜ÂÚÂÌÓ ÚflıÌÓÚÓ ‚ÎËflÌËÂ, ËÒÍ˙Ú Ò ۂÂÎË˜Ë ‰Ó 9,279 Ô˙ÚË. à ‚ ‰‚‡Ú‡ ÒÎÛ˜‡fl ÓÚÌÓ¯ÂÌËÂÚÓ Ì‡ ËÒÍÓ‚ÂÚ Ëχ ÒË„ÌËÙË͇ÌÚÂÌ ı‡‡ÍÚÂ. ëÔÓ‰ „ÂÒËÓÌÌÓÚÓ Û‡‚ÌÂÌËÂ, ‚Íβ˜‚‡˘Ó ‚˙Á‡ÒÚÚ‡ Ë ÔÓ·, Û‚Â΢ÂÌËÂÚÓ Ì‡ ‚˙Á‡ÒÚÚ‡ Ò Â‰Ì‡ „Ó‰Ë̇ ̇χÎfl‚‡ ¯‡Ì-

N 110

X 66,60

ïËÔÂ SD 7,73

 Min 46,00

Max 80,00

n.s.

Ú˘ÌË ‚˙ÁÏÓÊÌÓÒÚË Á‡ ‰Ë‡„ÌÓÒÚË͇ ̇ Ô·ÍÓ‚Ë ÛΈÂ‡ˆËË. ᇠ‡ÁÎË͇ ÓÚ ı‡‡ÍÚÂËÁË‡Ì Ô·ÍÓ‚‡Ú‡ ÔÓ‚˙ıÌÓÒÚ, ̇΢ËÂÚÓ Ì‡ ÎËÔˉÌÓ Ò˙‰˙ÊËÏÓ Ë Í‡ÎˆËÂ‚Ë Ì‡ÚÛÔ‚‡ÌËfl ÏÓÊ ‰‡ ·˙‰Â ÓÔ‰ÂÎÂÌÓ ‰ÓÒÚÓ‚ÂÌÓ ˜ÂÁ ÛÎÚ‡Á‚ÛÍÓ‚Ó Í·ÒË‡Ì ̇ Ô·ÍËÚ ÔÓ ÓÚÌÓ¯ÂÌË Úflı̇ڇ ÔÎ˙ÚÌÓÒÚ /ÂıÓ„ÂÌÌÓÒÚ/. íÓ‚‡ Ò ÓÒ˙˘ÂÒÚ‚fl‚‡ ‰ËÂÍÚÌÓ ‚ËÁÛ‡ÎÌÓ ÓÚ ËÁÒΉ‚‡˘Ëfl. ë ˆÂÎ ÔÓ-ÚӘ̇ ı‡‡ÍÚÂËÒÚË͇ Ò‡ ‡Á‡·ÓÚÂÌË ÍÓÏÔ˛Ú˙ÌË ÔÓ„‡ÏË, ˜ËÂÚÓ ÔËÎÓÊÂÌË ‚ ÏÓÏÂÌÚ‡ Ò ÚÂÒÚ‚‡ (10). ïËÔÓÂıÓ„ÂÌÌËÚ Ô·ÍË ËÏ‡Ú ·Ó„‡ÚÓ ÎËÔˉÌÓ Ò˙‰˙ÊËÏÓ Ë ‚˙ÚÂÔ·ÍÓ‚Ë ıÂÏÓ‡„ËË, ‡ ıËÔÂÂıÓ„ÂÌÌËÚ Ô·ÍË Ò˙‰˙Ê‡Ú Ôӂ˜ ÙË·ÓÁ̇ Ú˙Í‡Ì Ë Í‡ÎˆËÂ‚Ë ÓÚ·„‡ÌËfl /2,10,11/. èÓ‡‰Ë ÚÓ‚‡ ıËÔÓÂıÓ„ÂÌÌËÚ Ô·ÍË ÌÓÒflÚ Ôӂ˯ÂÌ ËÒÍ Á‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë ÛÒÎÓÊÌÂÌËfl, Ú˙È Í‡ÚÓ Ò‡ Á̇˜ËÚÂÎÌÓ ÔÓ-‡ÌËÏË Ë ÎÂÒÌÓ Ò Ô‚˙˘‡Ú ‚ ËÁÚÓ˜ÌËÍ Ì‡ ÏÓÁ˙˜ÂÌ ÂÏ·ÓÎËÁ˙Ï /5-7,12/. éÒ‚ÂÌ ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ËÒıÂÏ˘ÌË ËÌÒÛÎÚË /8,13/, ÔË ·ÓÎÌËÚÂ Ò ıËÔÓÂıÓ„ÂÌÌË ‡ÚÂÓÒÍÎÂÓÚ˘ÌË Í‡ÓÚˉÌË Ô·ÍË, Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔÓ‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ ‡ÒËÏÔÚÓÏÌË ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË (12) Ë ÔÓ-„ÓÎflχ ˜ÂÒÚÓÚ‡ ̇ ÍÓÓ̇ÌË Ë̈ˉÂÌÚË (14). êÂÁÛÎÚ‡ÚËÚ ÓÚ Ì‡ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì ÔÓ͇Á‚‡Ú, ˜Â ÓÚ ‚Ò˘ÍË Ì‡È-˜ÂÒÚÓ ÔÓÛ˜‚‡ÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ, ÔÓÌËÊÂÌÓÚÓ ÌË‚Ó Ì‡ ãÇè ıÓÎÂÒÚÂÓÎ Â Ò ÓÒÌÓ‚ÌÓ Á̇˜ÂÌË Á‡ Ó·‡ÁÛ‚‡Ì ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË. ê‰ˈ‡ ÔÓÔÛ·ˆËÓÌÌË ËÁÒΉ‚‡ÌËfl ‰Ó͇Á‚‡Ú ˜Â ÔË Ì‡Ï‡Îfl‚‡Ì ÌË‚ÓÚÓ Ì‡ ãÇè ıÓÎÂÒÚÂÓÎ Ò˙˘ÂÒÚ‚Û‚‡ Ôӂ˯ÂÌ ËÒÍ Á‡


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

퇷Îˈ‡ 2: ë‡‚ÌËÚÂÎÂÌ ‡Ì‡ÎËÁ ̇ ËÁÒΉ‚‡ÌËÚ ÌÂÏÂÚË‡ÌË ÔËÁ̇ˆË èÓ͇Á‡ÚÂÎË èÓÎ Ï˙Ê ÊÂÌË ïÓÎÂÒÚÂÓÎÂÏËfl ıËÔÂ çÓχ ãçè ıÓÎÂÒÚÂÓÎ Û‚Â΢ÂÌ çÓχÎÂÌ ãÇè ıÓÎÂÒÚÂÓΠ̇χÎÂÌ çÓχÎÂÌ íË„ÎˈÂË‰Ë Û‚Â΢ÂÌË çÓχÎÌË Ñˇ·ÂÚ ‰‡ Ì ÄÚÂˇÎ̇ ıËÔÂÚÓÌËfl ‰‡ Ì èÂÊË‚flÌ ËÌÒÛÎÚ ‰‡ Ì í˛Ú˛ÌÓÔÛ¯ÂÌ ‰‡ ÌÂ

óÂÒÚÓÚ‡ (%) ïËÔÓÂıÓ„ÂÌÌË ïËÔÂÂıÓ„ÂÌÌË Ô·ÍË Ô·ÍË

ê

13 (32,5) 27 (67,5)

37 (33,6) 73 (66,4)

n.s.

24 (60,0) 16 (40,0)

75 (68,2) 35 (31,8)

n.s.

21 (53,8) 18 (46,2)

41 (38,3) 66 (61,7)

n.s.

27 (67,5) 13 (32,5)

22 (20,0) 88 (80,0)

<0.001

16 (40,0) 24 (60,0)

40 (36.4) 70 (63,6)

n.s.

6 (15,0) 34 (85,0)

15 (13,6) 95 (86,4)

n.s.

26 (65,0) 14 (35,0)

59 (53,6) 51 (46,4)

n.s.

13 (32,5) 27 (67,5)

39 (35,8) 70 (64,2)

n.s.

3 (7,5) 37 (92,5)

5 (4,5) 105 (95,5)

n.s.

157

‡ÁÎË͇ ‚ ÔÓˆÂÌÚ‡ ̇ ·ÓÎÌËÚÂ Ò ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË ‚ „ÛÔ‡Ú‡ ÔÂÊË‚fl· ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ë ÔË Á‰‡‚ËÚ Ë̉˂ˉË. èÓ‰Ó·ÌË ÂÁÛÎÚ‡ÚË Ò‡ ÔÓÎÛ˜ÂÌË Ë ‚ ‰Û„Ë ÔÓÛ˜‚‡ÌËfl /13,19,20/, ‰Ó͇ÚÓ ÒÔÓ‰ Gronholdt å et al ÔÓ-‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË Â Ò‚˙Á‡Ì‡ Ò Ôӂ˯ÂÌÓ ÌË‚Ó Ì‡ ÚË„ÎˈÂˉËÚ (10,21). 燷≇‚‡ Ò ÚẨÂ̈Ëfl Á‡ ‡Á‚ËÚË ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË ‚ Ï·‰‡ ‚˙Á‡ÒÚ, ‚˙ÔÂÍË ÎËÔÒ‡Ú‡ ̇ ÒÚ‡ÚËÒÚ˘ÂÒ͇ Á̇˜ËÏoÒÚ. Ç˙Á‡ÒÚÚ‡ Ò fl‚fl‚‡ ÔÓÚÂÍÚË‚ÂÌ Ù‡ÍÚÓ ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË. íÓÁË Ù‡ÍÚ Ì‡ÒÓ˜‚‡ Í˙Ï ıËÔÓÚÂÁ‡Ú‡ ˜Â ıËÔÓÂıÓ„ÂÌÌËÚ Ô·ÍË Ò‡ ÂÚ‡Ô ÓÚ Í‡ÓÚˉ̇ڇ ‡ÚÂÓÒÍÎÂÓÁ‡, Ú‡ÌÒÙÓÏË‡ÈÍË Ò ‚ÔÓÒΉÒÚ‚Ë ‚ ıËÔÂÂıÓ„ÂÌÌË Ô·ÍË. ᇠ‰ÓÒÚÓ‚ÂÌÓÒÚ Ì‡ Ú‡ÁË ıËÔÓÚÂÁ‡ Ò‡ ÌÂÓ·ıÓ‰ËÏË ÔÓ-̇ڇÚ˙˜ÌË ‰Ó͇Á‡ÚÂÎÒÚ‚‡. Ç Á‡Íβ˜ÂÌË ̇΢ËÂÚÓ Ì‡ ̇χÎÂÌ ãÇè ıÓÎÂÒÚÂÓÎ Û‚Â΢‡‚‡ ËÒ͇ Á‡ Ó·‡ÁÛ‚‡Ì ̇ ıËÔÓÂıÓ„ÂÌ̇ Ô·͇ ̇‰ 8 Ô˙ÚË ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË, ÍÓËÚÓ ËÏ‡Ú ÌÓχÎÂÌ ãÇè ıÓÎÂÒÚÂÓÎ. 燷≇‚‡ Ò ÚẨÂ̈Ëfl Á‡ ÔÓ-„ÓÎflχ ˜ÂÒÚÓÚ‡ ̇ Ó·‡ÁÛ‚‡Ì ‚ Ï·‰‡ ‚˙Á‡ÒÚ. ÑÛ„ËÚ ̇È-˜ÂÒÚÓ Ò¢‡ÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ – ÔÓÎ, ‰Ë‡·ÂÚ, ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl Ë Ú˛Ú˛ÌÓÔÛ¯ÂÌ ÌflÏ‡Ú ‚˙Á͇ Ò ÏÓÙÓÎÓ„ËflÚ‡ ̇ Ô·͇ڇ. ãàíÖêÄíìêÄ

‡Á‚ËÚË ̇ ËÒıÂÏ˘ÌË ÍÓÓ̇ÌË Ë̈ˉÂÌÚË Ì‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl, ̇ ͇ÓÚˉ̇ ‡ÚÂÓÒÍÎÂÓÁ‡ (1417). àÁ‚ÂÒÚÌË Ò‡ ÏÌÓ„Ó ‰Ó͇Á‡ÚÂÎÒÚ‚‡ Á‡ ·Î‡„ÓÔËflÚÂÌ ‰ËÂÍÚÂÌ ÂÙÂÍÚ Ì‡ ïéã-ãÇè ‚˙ıÛ ‡ÚÂˇÎ̇ڇ ÒÚÂ̇, ÓÚ ÍÓËÚÓ Ò Ì‡È-„ÓÎflÏÓ Á̇˜ÂÌË  ۘ‡ÒÚËÂÚÓ ÏÛ ‚ ËÁ‚΢‡Ì ̇ ËÁÎ˯ÌËfl ıÓÎÂÒÚÂÓÎ ÓÚ ÔÂËÙÂÌËÚ ÍÎÂÚÍË Ë ‡ÚÂÓÒÍÎÂÓÚ˘ÌË Ô·ÍË Ë ÒΉ Ì„ӂ‡Ú‡ ÂÒÚÂËÙË͇ˆËfl – Ú‡ÌÒÔÓÚË‡Ì ‰Ó ˜ÂÌËfl ‰Ó·, Í˙‰ÂÚÓ Ò ËÁÔÓÎÁ‚‡ Á‡ ÒËÌÚÂÁ ̇ ÊÎ˙˜ÌË ÍËÒÂÎËÌË, ÎËÔÓÔÓÚÂËÌË, ‚ËÚ‡ÏËÌ D Ë ÒÚÂÓˉÌË ıÓÏÓÌË (18). ÑÛ„ËÚ ‡ÚÂÓ„ÂÌÌË Ò‚ÓÈÒÚ‚‡ Ò‡ Ò‚˙Á‡ÌË Ò ËÌıË·Ë‡Ì ̇ ÓÍËÒÎÂÌËÂÚÓ Ì‡ ÎËÔÓÔÓÚÂËÌËÚÂ Ò ÌËÒ͇ ÔÎ˙ÚÌÓÒÚ Ë ‡ÚÂÓ„ÂÌÌËÚ ÂÙÂÍÚË Ì‡ ÓÍËÒÎÂÌËÚ ãçè, ËÌıË·Ë‡Ì ‡ÍÚË‚Ë‡ÌÂÚÓ Ì‡ ÚÓÏ·ÓˆËÚËÚÂ Ë ÂÍÒÔÂÒËflÚ‡ ̇ ẨÓÚÂÎÌËÚ ÍÎÂÚÍË Ì‡ ‡‰ıÂÁËÓÌÌËÚ ÏÓÎÂÍÛÎË Ë ÒÂÎÂÍÚË̇ (18). êÂÁÛÎÚ‡ÚËÚ ÓÚ Ì‡ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì ÔÓ͇Á‚‡Ú ˜Â ‰Û„ËÚ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ – ÔÓÎ, ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl, Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, Ú˛Ú˛ÌÓÔÛ¯ÂÌ Ì Ó͇Á‚‡Ú ‚ÎËflÌË ‚˙ıÛ Ô·ÍÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëfl. ç Ò Á‡·ÂÎflÁ‚‡

1. Spence J, Eliasziw M, DiCicco M, Hackam D et al. Carotid plaque area. Stroke, 2002,33,2916-20. 2. Öuropean Carotid Plaque Study Group. Carotid artery plaque composition: relationship to clinical presentation and ultrasound B-mode imaging. Eur J Vasc Endovasc Surg,1995,10,23-30. 3. Langsfield M, Gray-Weale A, Lusby R. The role of plaque morphology and diameter reduction in the development of new symptoms in asymptomatic carotid arteries. J Vasc Surg.1989,9,548-557. 4. Johnson J, Kennelly M, Decesare D et al. Natural history of asymptomatic carotid plaque. Arch Surg 1985.120,1010-12. 5. Sterpetii A, Schultz R, Feldhaus R et al. Ultrasonographic features of carotid plaque and the risk of subsequent neurologic deficits. Surgery.1988,104,652-60. 6. Groulakos G, Ramaswami G, Nicolaides A et al. Characterization of symptomatic and asymptomatic carotid plaques using high-resolution real-time ultrasonography. Br J Surg,1993.80,1274-77. 7. Feeley T, Leen E, Colgan M et al. Histologic characteristics of carotid artery plaque. J Vasc Surg.1991,13,719-24. 8. Mathiesen E, Bonaa K, Joakimsen O. Echolucent plaques are associated with high risk of ischemic cerebrovascular events in carotid stenosis: the Tromso Study. Circulation.2001,103,2171-75. 9. Sitzer M, Muller W, Siebler M et al. Plaque Ulceration and Lumen Thrombus Are the Main Sources of Cerebral Microemboli in High-Grade Internal Carotid Artery Stenosis. Stroke 1993.26,1231-33. 10. Gronholdt M, Nordestgaard B, Wiebe B. Echo lucency of computerized ultrasound images of carotid atherosclerotic plaque are associated with increased levels of triglyceride-rich lipoproteins as well as increased plaque lipid content. Circulation.1998,97,34-40. 11. Goncalves I, Moses J, Pedro L et al. Echolucency of carotid plaques correlates with plaque ellularity. Eur J Endovasc Surg, 2003.26,32-8. 12. Iannuzzi A, Wilcosky T, Mercuri M et al. Ultasonic correlates of carotid atherosclerosis in transient ischemic attack and stroke. Stroke.1995,26,614-9. 13. Zaletel M, Zvan B. Blood lipids and carotid plaques. Acta clin Croat. 2002,41,5-10. 14. Honda O, Sugiyama S, Kugiyama K et al. Echolucent carotid plaques predict future coronary events in patients with coronary artery disease. J Am Coll Cardiol. 2004,43,1177-84. 15. Gordon T, Castelli W, Hjortland M et al. Hihh density lipoprotein as a protective factor against coronary heart disease: the Framingame Study. Am J Med. 1977,62,707-714. 16. Miller N, Thelle D, Forde O et al. The Tromso heart-Study: high-density lipoprotein an coronary heart disease: a prospective case-control study. Lancet. 1977,1,965-968. 17. Tell G, Crouse J, Furberg C. Relation between blood lipids. Lipoproteins and cerebrovascular atherosclerosis: a review. Stroke.1988,19,423-430. 18. äËflÍÓ‚ ã, 䇂˙ÍÓ‚‡ û, ÑËÏÓ‚‡ ü. ãËÔÓÔÓÚÂËÌË Ò ‚ËÒÓ͇ ÔÎ˙ÚÌÓÒÚ /ãÇè/ Ë ‡ÚÂÓ„ÂÌÂÌ ËÒÍ. ÄÍÚÛ‡Î̇ ÎËÔˉÓÎÓ„Ëfl. 2003,1,4-12.

퇷Îˈ‡ 3: éÚÌÓ¯ÂÌË ̇ ËÒÍÓ‚ÂÚÂ Ë 95% ‰Ó‚ÂËÚÂÎÂÌ ËÌÚÂ‚‡Î ÔË Ù‡ÍÚÓ‡ ãÇè ıÓÎÂÒÚÂÓÎ

ãÇè ıÓÎÂÒÚÂÓÎ çÓχÎÂÌ ç‡Ï‡ÎÂÌ * - p<0.001

ÅÂÁ ÓÚ˜Ëڇ̠‚ÎËflÌËÂÚÓ Ì‡ ‚˙Á‡ÒÚÚ‡ Ë ÔÓ· éÚÌÓ¯ÂÌË ̇ 95% ‰Ó‚ÂËÚÂÎÂÌ ËÒÍÓ‚ÂÚ ËÌÚÂ‚‡Î 1 8,308* 3,696 – 18,674

ë ÓÚ˜Ëڇ̠‚ÎËflÌËÂÚÓ Ì‡ ‚˙Á‡ÒÚÚ‡ Ë ÔÓ· éÚÌÓ¯ÂÌË ̇ 95% ‰Ó‚ÂËÚÂÎÂÌ ËÒÍÓ‚ÂÚ ËÌÚÂ‚‡Î 1 9,279* 3,960 – 21,741


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Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

19. Mathiesen E, Bonaa K, Joakimsen O. Low levels of high-density lipoprotein cholesterol are associated with echolucent carotid artery plaque: the Tromso study. Stroke.2001,32,1960-5. 20. Bradley W, Gianturco S. Trigliceride rich lipoproteins and atherosclerosis: pathophysiological considerations. J Intern Med. 1994, (Suppl 736)33-9. 21. Gronholdt M, Nordestgaard B, Nielsen T et al. Echolucent Carotid Artery Plaques are associated with elevated levels of fasting and postprandial triglycerideRich Lipoproteins. Stroke. 1996,27,1266-72.

158

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- Ö. LJÒË΂‡ ‰.Ï. äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl åÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇“ 1527 ëÓÙËfl , ÛÎ. ◊ÅflÎÓ ÏÓ“8 íÂÎ. 9432/597

éË„Ë̇ÎÌË ÒÚ‡ÚËË ÄçíàîéëîéãàèàÑÖç ëàçÑêéå èêà ÑÖñÄ ë åéáöóçà àçëìãíà Ç.ÅÓÊËÌÓ‚‡, è.ÑËÏÓ‚‡, Ç.ÉÂ„ÂΘ‚‡, Ç.íÓÏÓ‚, í.üÌÍÓ‚ ÑÂÚÒ͇ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇, ìëÅÄãçè “ë‚.ç‡ÛÏ”-ëÓÙËfl SUMMARY ANTIPHOSPHOLIPID SYNDROME IN CHILDREN WITH STROKE V. Bojionova, P. Dimova, V. Gergueltcheva, V. Tomov, T. Yankov Screening for antiphospholipid syndrome (ALP) is a routine procedure for evaluation of the etiology in children with cerebral infarcts or TIAs. Objective: To analyze the frequency of antiphospholipid syndrome in children with “idiopatic” cerebral infarcts or transitory ischemic attacks (TIAs) as well as the clinical characteristics, prognosis and pathology in the neuroimaging examinations in these cases. Clinical contingent and methods: Nine out of 13 children with idiopathic cerebral infarcts or TIAs, being aCL of IgG or IgM class positive, were neurologically examined and followed-up from 1 to 6 years. CT and/or MRI were done for each of them. Results: APL was diagnosed in 9/13 or 69.2% of the children with “idiopathic” stroke. The stroke onset was between 9 months and 13 years of age. Eight children were with acute course and one- with chronic progressive (pseudotumour) course. Recurrent stroke have 5 children (55.6 %). Focal epileptic seizures (n=4) or “migraine–like” headache (n=2) preceded 1 month to 4 years the stroke. In 6 children multiple ischemic lesions, localized in the branches of internal carotid artery and in the posterior cerebral arteries were found. One child had “moya-moya”- like pathology on angiography. During the follow-up in two of the children the neurological deficit and the multiple MRI lesions were fully recovered, in one the lesions were in stationary stage; 6 out of 9 children (66.7%) with recurrent stroke (n=5) or with chronic progressive course (n=1) have new CT lesions and in 3 of them progressive cerebral atrophy was found. Increased aCL IgG titers were found in 7 children and aCL IgM titers- in 4 children. In 3 children anti-smooth muscle and anti-mitochondrial antibodies were established, in 2 - antinuclear antibodies and LE cells, but the constellation was insufficient for diagnosis “lupus erythematodes”. Conclusions: In children with cerebral infarcts or TIAs due to ALP recurrent stroke or progressive neurological deficiency, multiple ischemic lesions and progressive cerebral atrophy were found. äey words: antiphospholipid syndrome, anticardiolipin antibodies, stroke, transitory ischemic attacks, children, recurrent clinical course. êÖáûåÖ ëÍËÌËÌ„Ó‚ÓÚÓ ËÁÒΉ‚‡Ì Á‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò ÔËÂχ Á‡ ÛÚËÌÌÓ ÔË ËÁflÒÌfl‚‡Ì ̇ ÂÚËÓÎÓ„ËflÚ‡ ̇ ÏÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË Ë èàçåä Û ‰Âˆ‡.

àÁÒΉ‚‡ÌÂÚÓ ˆÂÎË ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ ÔË ‰Âˆ‡ Ò Ë‰ËÓÔ‡Ú˘ÌË ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË Ë èàçåä Ë ‡Ì‡ÎËÁË‡Ì ̇ ÍÎËÌ˘̇ڇ ı‡‡ÍÚÂËÒÚË͇, Ô‡ÚÓÎÓ„ËflÚ‡ ÔË Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl Ë ÔÓ„ÌÓÁ‡Ú‡ ‚ ÚÂÁË ÒÎÛ˜‡Ë. ÄÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò Ì‡Î˘Ë ̇ ÒÂÛÏÌË ‡ÌÚË͇‰ËÓÎËÔËÌÓ‚Ë ‡ÌÚËÚ· (aCL) ÓÚ ÍÎ‡Ò IgG ËÎË IgM  ÛÒÚ‡ÌÓ‚ÂÌ ÔË ÔË 9 ÓÚ 13 ‰Âˆ‡ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ËÎË èàçåä (69,2%). 臈ËÂÌÚËÚ ҇ ÔÓÒΉÂÌË Ì‚ÓÎӄ˘ÌÓ, Ò äí Ë/ËÎË åêí ËÁÒΉ‚‡Ì ÓÚ 1 ‰Ó 6 „. åÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË Ë èàçåä Ò‡ Ò Ì‡˜‡ÎÓ ÏÂÊ‰Û 9 ÏÂÒˆ‡ Ë 13 „. èË 8 ‰Âˆ‡ Ò‡ Ò ÓÒÚÓ ‡Á‚ËÚËÂ, ‡ ÔË 1- Ò ıÓÌ˘ÌÓ-ÔÓ„ÂÒË‡˘Ó (ÔÒ‚‰ÓÚÛÏÓÌÓ) ÔÓÚ˘‡ÌÂ, ÔË 5 Ò‡ Ò ˆˉ˂Ë‡˘ ıÓ‰. è‰¯ÂÒÚ‚‡Ú Ò ÓÚ 1 ÏÂÒˆ ‰Ó 4 „. ÓÚ ÙÓ͇ÎÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË (2 ‰Âˆ‡) ËÎË “ÏË„ÂÌÓÔÓ‰Ó·ÌÓ” „·‚Ó·ÓÎË (2 ‰Âˆ‡). èË 6 ‰Âˆ‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÏÌÓÊÂÒÚ‚ÂÌË ËÒıÂÏ˘ÌË ÎÂÁËË, ÎÓ͇ÎËÁË‡ÌË ‚ ·‡ÒÂÈ̇ ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl Ë ‚ Á‡‰ÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË. èË Â‰ÌÓ ÓÚ ‰Âˆ‡Ú‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ë ‡Ì„ËÓ„‡ÙÒÍË ·ÂÎÂÁË Á‡ moya-moya. èË ÔÓÒΉfl‚‡ÌÂÚÓ ÔË ‰‚ ‰Âˆ‡ Ì‚ÓÎӄ˘ÌËflÚ ‰ÂÙˈËÚ Ë ÏÌÓÊÂÒÚ‚ÂÌËÚ ÎÂÁËË ‚ åêí ÔÂÚ˙Ôfl‚‡Ú Ô˙ÎÌÓ Ó·‡ÚÌÓ ‡Á‚ËÚËÂ, ÔË Â‰ÌÓ ‰ÂÚ ÎÂÁËËÚ ҇ ÒÚ‡ˆËÓÌË‡ÌË; ÔË 6 ‰Âˆ‡ (66,7%), 5 ÓÚ ÍÓËÚÓ Ò ˆˉ˂Ë‡˘ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ Ë Â‰ÌÓ Ò ÔÒ‚‰ÓÚÛÏÓÌÓ ÔÓÚ˘‡ÌÂ, Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÌÓ‚Ë Ó„ÌˢÌË ÎÂÁËË ÔË ëí, ‡ ÔË 3 Ë ÔÓ„ÂÒË‡˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl. èË 7 ‰Âˆ‡ aCL Ò‡ ÓÚ IgG ÚËÔ, ‡ ÔË 4 –ÓÚ IgM ÚËÔ. èË 3 ÓÚ ‰Âˆ‡Ú‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ë ‡ÌÚ˄·‰ÍÓÏÛÒÍÛÎÌË, ‡ÌÚËÏËÚÓıÓ̉ˇÎÌË ‡ÌÚËÚ·, ‡ ÔË 2- Ë ‡ÌÚËÌÛÍ·ÌË ‡ÌÚËÚ· Ë LE ÍÎÂÚÍË, ÌÓ ·ÂÁ Ô˙Î̇ ÍÓÌÒÚ·ˆËfl Á‡ ÔÓÒÚ‡‚flÌ ̇ ‰Ë‡„ÌÓÁ‡Ú‡ Lupus erythematodes. èË ‰Âˆ‡Ú‡ Ò ÏÓÁ˙˜ÌË ËÒıÂÏËË Ë ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò‡ ı‡‡ÍÚÂÌË ˆˉ˂Ë‡˘ ıÓ‰ ËÎË ÔÓ„ÂÒË‡˘Ó ‡Á‚ËÚË ̇ ÌÓ‚ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ, ÏÌÓÊÂÒÚ‚ÂÌË ËÒıÂÏ˘ÌË ÎÂÁËË Ë ÔÓ„ÂÒË‡˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl. äβ˜Ó‚Ë ‰ÛÏË: ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ, ‡ÌÚË͇‰ËÓÎËÔËÌÓ‚Ë ‡ÌÚËÚ·, ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË Ë ÔÂıÓ‰ÌË ËÒıÂÏ˘ÌË Ì‡Û¯ÂÌËfl ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ, ‰Âˆ‡, ˆˉ˂Ë‡˘ ıÓ‰. ÇöÇÖÑÖçàÖ ÖÚËÓÎÓ„ËflÚ‡ ̇ ÏÓÁ˙˜ÌËÚ ËÒıÂÏËË ‚ ‰ÂÚÒ͇ڇ ‚˙Á‡ÒÚ Â ËÁÍβ˜ËÚÂÎÌÓ ‡ÁÌÓÓ·‡Á̇. Ç ÔÓÒΉÌËÚ „Ó‰ËÌË Ò ӷ˙˘‡ ‚ÌËχÌË ̇ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ Í‡ÚÓ Ô˘Ë̇ Á‡ ÔÂıÓ‰ÌË ËÒıÂÏ˘ÌË Ì‡Û¯ÂÌËfl ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌË (èàçåä), ËÒıÂÏ˘ÌË ËÌÒÛÎÚË (3-16) Ë ÂÔËÎÂÔÒËfl ‚ ‰ÂÚÒ͇ڇ ‚˙Á‡ÒÚ (6). ÄÌÚËÙÓÒÙÓÎËÔˉÌËflÚ ÒË̉ÓÏ Â ËÏÛÌÓωËË‡Ì ÚÓÏ·ÓÚ˘ÂÌ ÔÓˆÂÒ, Ò‚˙Á‡Ì Ò ıËÔÂÍÓ‡„ÛÎÓ·ËÎËÚÂÚ, χÍÓ- Ë ÏËÍÓÚÓÏ·ÓÁË Ì‡ ‡ÚÂˇÎÌËÚÂ Ë ‚ÂÌÓÁÌË Ò˙‰Ó‚Â, ÔË Ì‡Î˘Ë ̇ ‡ÌÚËÙÓÒÙÓÎËÔË‰Ë ‡ÌÚËÚ· (ÒÂÛÏÌË ÔÓÎËÍÎÓ̇ÎÌË


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

IgG, IgA, IgM). éÔËÒ‚‡Ú Ò ‰‚‡ ‚‡ˇÌÚ‡- Ô˙‚˘ÂÌ Ë ‚ÚÓ˘ÂÌ ÔË Lupus erythematodes, ‰Û„Ë ÍÓ·„ÂÌÓÁË Ë ‡‚ÚÓËÏÛÌÌË Á‡·ÓÎfl‚‡ÌËfl (1,2). èË ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ Ò‡ ̇·Î˛‰‡‚‡ÌË èàçåä, ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË, ÏË„Â̇, ÂÔËÎÂÔÒËfl, Ú‡ÌÁ‚ÂÁ‡Î̇ ÏËÂÎÓÔ‡ÚËfl, Ò˙‰Â˜ÌË Í·ÔÌË Á‡·ÓÎfl‚‡ÌËfl, ÒÔÓÌÚ‡ÌÌË ‡·ÓÚË, ‚ÂÌÓÁÌË ÚÓÏ·ÓÁË (1). ᇠ‰Ë‡„ÌÓÒÚˈË‡ÌÂÚÓ Ì‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ Â ÌÂÓ·ıÓ‰ËÏÓ ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ‡ÌÚË͇‰ËÓÎËÔËÌÓ‚Ë ‡ÌÚËÚ· (‡ëL) Ë ÎÛÔÛÒÂÌ ‡ÌÚËÍÓ‡„Û·ÌÚ (LA) ‰‚ÛÍ‡ÚÌÓ ‚ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ‚ „‡ÌˈËÚ ̇ 6 Ò‰ÏËˆË (18) ‰Ó 3 ÏÂÒeˆ‡ (1), ÔË ËÁÍβ˜‚‡Ì ̇ ‰Û„Ë Ô˘ËÌË Á‡ Ò˙‰Ó‚Ë Ë̈ˉÂÌÚË (ÔÓÚÂËÌ ë Ë ÔÓÚÂËÌ S ‰ÂÙˈËÚ, ÒËÒÚÂÏÌË ‚‡ÒÍÛÎËÚË Ë Ú.Ì.). ëÍËÌËÌ„Ó‚ÓÚÓ ËÁÒΉ‚‡Ì Á‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò ÔËÂχ Á‡ ÛÚËÌÌÓ ÔË ËÁflÒÌfl‚‡Ì ̇ ÂÚËÓÎÓ„ËflÚ‡ ̇ ÏÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË Ë èàçåä Û ‰Âˆ‡ (7). ÑËÒÍÛÚË‡ Ò ÔÓ„ÌÓÒÚ˘ÌÓÚÓ Á̇˜ÂÌË ̇ ̇΢ËÂÚÓ Ì‡ aCL ÓÚ ÍÎ‡Ò IgG Á‡ ˆˉ˂Ë‡˘ËÚ ÏÓÁ˙˜ÌË ËÒıÂÏËË ‚ ‰ÂÚÒ͇ڇ ‚˙Á‡ÒÚ (11). è‡‚ËÎÌÓ ÔÓÒÚ‡‚Â̇ڇ ‰Ë‡„ÌÓÁ‡ ÓÔ‰ÂÎfl ÚÂ‡Ô‚Ú˘ÌËfl ÔÓ‰ıÓ‰. ÄÌÚˇ„„‡ÌÚÌÓ Î˜ÂÌËÂ Â Ò ÔÓ‰Ó·˙ ÂÙÂÍÚ, ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÚËÍÓÒÚÂÓˉ̇ڇ Ë ËÏÛÌÓÒÛÔÂÒ‡ÌÚ̇ ÚÂ‡ÔËfl. àÁÒΉ‚‡ÌÂÚÓ ˆÂÎË ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ ÔË ‰Âˆ‡ Ò “ˉËÓÔ‡Ú˘ÌË” ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË Ë èàçåä Ë ‡Ì‡ÎËÁË‡Ì ̇ ÍÎËÌ˘̇ڇ ı‡‡ÍÚÂËÒÚË͇, Ô‡ÚÓÎÓ„ËflÚ‡ ÔË Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl Ë ÔÓ„ÌÓÁ‡Ú‡ ‚ ÚÂÁË ÒÎÛ˜‡Ë. äãàçàóÖç äéçíàçÉÖçí à åÖíéÑà 燷≇‚‡ÌË Ò‡ 13 ‰Âˆ‡ Ò “ˉËÓÔ‡Ú˘ÌË” ÏÓÁ˙˜ÌË ËÒıÂÏËË, ÔÂÏË̇ÎË ÔÂÁ ÑÂÚÒ͇ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇ ̇ ìëÅÄãçè “ë‚.ç‡ÛÏ”-ëÓÙËfl Á‡ 5 „Ӊ˯ÂÌ ÔÂËÓ‰. èË 9 ÓÚ Úflı (5 ÏÓϘÂÚ‡ Ë 4 ÏÓÏ˘ÂÚ‡ ̇ ‚˙Á‡ÒÚ ÓÚ 2 ‰Ó 13 „.) Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‰‚ÛÍ‡ÚÌÓ ÒÂÛÏÌË ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ· ÔÓ ‚ÂÏ ̇ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (‰Ó 6 Ò‰ÏˈË). 臈ËÂÌÚËÚ ҇ ÔÓÒΉÂÌË ‚ ‰Ë̇ÏË͇ ÓÚ 1 ‰Ó 6 „. Ì‚ÓÎӄ˘ÌÓ, ˜ÂÁ ËÁÒΉ‚‡ÌËfl Á‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ·, ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl (äí) Ë/ËÎË Ï‡„ÌËÚÌÓÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl (åêí). 凄ÌËÚÌÓÂÁÓ̇ÌÒ̇ ‡Ì„ËÓ„‡ÙËfl (åê-ÄÉ)  ÓÒ˙˘ÂÒÚ‚Â̇ ÔË 2 ‰Âˆ‡, ÏÓÁ˙˜Ì‡ ‡Ì„ËÓ„‡ÙËfl- ÔË 2 ‰Âˆ‡.

퇷Îˈ‡ 2. íËÔ Ì‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËÚ ‡ÌÚËÚ· Ë ıÓ‰ ̇ ÏÓÁ˙˜ÌËÚ ËÒıÂÏËË (n=9) 燷β‰ÂÌË ‹ 1. 2. 3. 4. 5. 6. 7. 8. 9.

é·˘Ó

ë‡ÏÓ IgG

ë‡ÏÓ IgM

+ + +

IgG+IgM èÂÒËÒÚË‡˘Ë aCL IgG + IgG + +

+ + + + +

5/9 2/9 2/9 (55,6) (22,2%) (22,2%)

Igå +

ïÓ‰ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ Ë/ËÎË èàçåä êˆˉ˂Ë‡˘ êˆˉ˂Ë‡˘ Ö‰ÌÓÍ‡ÚÂÌ êˆˉ˂Ë‡˘ êˆˉ˂Ë‡˘ Ö‰ÌÓÍ‡ÚÂÌ êˆˉ˂Ë‡˘ Ö‰ÌÓÍ‡ÚÂÌ ïÓÌ˘ÌÓ ÔÓ„ÂÒË‡˘ (ÔÒ‚‰ÓÚÛÏÓÂÌ)

3/9 (33,3%)

ÎË ÔË 4 ‰Âˆ‡. è‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò ÔÓÒÚË, ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË Ë ‚ÚÓ˘ÌÓ-„ÂÌÂ‡ÎËÁË‡ÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË Â ‰Ë‡„ÌÓÒÚˈË‡Ì‡ 1 „. ÔÂ‰Ë ÏÓÁ˙˜ÌËfl ËÌÒÛÎÚ ÔË 2 ‰Âˆ‡. åË„ÂÌÓÔÓ‰Ó·ÌÓ „·‚Ó·ÓÎËÂ, ̇ÔÓ‰Ó·fl‚‡˘Ó “ÏË„Â̇ Ò ‡Û‡”, Ò Ì‡˜‡ÌË ıÂÏËÔ‡ÂÒÚÂÁËË Ë ‡Ù‡ÁËfl, Ë Ò˙Ô˙ÚÒÚ‚‡˘Ë ÙÓÚÓ- Ë ÙÓÌÓÙÓ·Ëfl  ̇Îˈ ÓÚ 1 ÏÂÒˆ ‰Ó 4 „. ÔÂ‰Ë ËÌÒÛÎÚ‡. Ñ‚Â ÓÚ 3-Ú ‰Âˆ‡ Ò ıÂÏËÔ‡ÂÚ˘ÂÌ ÒË̉ÓÏ ÔË Ô˙‚Ëfl ËÌÒÛÎÚ, ÔË ˆˉ˂‡ ‡Á‚Ë‚‡Ú ÍÓÌÚ‡Î‡ÚÂ‡Î̇ ıÂÏËÔ‡ÂÁ‡. ÖÍÒÚ‡ÔË‡ÏˉÌËÚ ‰‚ÛÒÚ‡ÌÌË ıÓÂÓ-‡ÚÂÚÓÁÌË ıËÂÍËÌÂÁË Ò ÓÒÚÓ Ì‡˜‡ÎÓ ÔË 2 ‰Âˆ‡ ÍÓÂÒÔÓ̉Ë‡Ú Ò ‰‚ÛÒÚ‡ÌÌË äí ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ fl‰‡. è‡ÎÂÓ- Ë ÌÂÓˆÂ·Â·ÂÌ ÒË̉ÓÏ Ò ̇·Î˛‰‡‚‡ ÔË Â‰ÌÓ ‰ÂÚÂ. êˆˉ˂Ë‡˘Ë ÏÓÁ˙˜ÌË ËÒıÂÏËË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔË 5 ÓÚ ‰Âˆ‡Ú‡ (55,6%) ÔË ÔÓÒΉfl‚‡Ì ÓÚ 1 ‰Ó 5 „. èË 2 ÓÚ Úflı Ë ÔË ‰ÂÚÂÚÓ Ò ÔÒ‚‰ÓÚÛÏÓÌÓ ‡Á‚ËÚË ÔÓÒÚÂÔÂÌÌÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ë ÌÓ‚‡ Ó„ÌË˘Ì‡ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ (è‡ÍËÌÒÓÌÓ‚ ÒË̉ÓÏ, ÍÓÌÚ‡Î‡ÚÂ‡ÎÌË ÔË‡ÏˉÌË ·ÂÎÂÁË). ë ‰ÌÓÍ‡ÚÂÌ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ Ò‡ 3 ‰Âˆ‡, ÔÓÒΉÂÌË Ò˙ÓÚ‚ÂÚÌÓ Â‰Ì‡ Ë 4 „.

n (%)

ç‚ÓËÁÓ·‡Áfl‚‡˘Ë ËÁÒΉ‚‡ÌËfl äí Ë åêí Ò‡ Ò ‰‡ÌÌË Á‡ ‰ËÌ˘ÌË ËÒıÂÏ˘ÌË ÎÂÁËË ‚ ̇˜‡ÎÓÚÓ Ò‡ÏÓ ÔË 3 ÓÚ ‰Âˆ‡Ú‡, ‡ ÔË 6 ÔÓÏÂÌËÚ ҇ ÏÌÓÊÂÒÚ‚ÂÌË. èÓ-̇ڇÚ˙¯ÌÓÚÓ ÔÓÒΉfl‚‡Ì ÔË 6 ‰Âˆ‡ (5 Ò ˆˉ˂Ë‡˘Ë ËÌÒÛÎÚË Ë Â‰ËÌ Ò ÔÒ‚‰ÓÚÛÏÓÌÓ ÔÓÚ˘‡ÌÂ) ‡ÁÍË‚‡ ÏÌÓÊÂÒÚ‚ÂÌË ÎÂÁËË Ò ‡Á΢̇ ‰‡‚ÌÓÒÚ ‚ ‡Á΢ÌË Ò˙‰Ó‚Ë ·‡ÒÂÈÌË. ë ‰‚ÛÒÚ‡ÌÌË ÏÓÁ˙˜ÌËÚ ËÒıÂÏËË ‚ ‡Á΢ÌË ÍÎÓÌӂ ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl Ò‡ 8 ‰Âˆ‡, 2 ÓÚ ÍÓËÚÓ Ò ÎÓ͇ÎËÁ‡ˆËfl ‚ ·‡Á‡ÎÌËÚ fl‰‡ ‰‚ÛÒÚ‡ÌÌÓ; ‰ÌÓÒÚ‡ÌÌË ËÒıÂÏËË ‚ ·‡ÒÂÈ̇ ̇ Á‡‰ÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË ËÏ‡Ú 3 ‰Âˆ‡, ‡ Ò ‰‚ÛÒÚ‡ÌÌË Â Â‰ÌÓ ‰ÂÚÂ. ë äí ‰‡ÌÌË Á‡ ÔÓ„ÂÒË‡˘ ÏÓÁ˙˜ÂÌ ‡ÚÓÙ˘ÂÌ ÔÓˆÂÒ Ò‡ 3 ‰Âˆ‡, ÍÓËÚÓ Ò‡ Ò ÔÓÒÚÂÔÂÌÌÓ ‡Á‚ËÚË ̇ ÌÓ‚‡ Ó„ÌË˘Ì‡ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇. è˙ÎÌÓ Ó·‡ÚÌÓ ‡Á‚ËÚË ̇ ÎÂÁËËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ò‡ÏÓ ÔË 2 ÓÚ ‰Âˆ‡Ú‡ Ò Â‰ÌÓÍ‡ÚÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË, ÔË Â‰ÌÓ ‰ÂÚ ·ÂÁ ÌÓ‚ ˆˉ˂ ÎÂÁËËÚ ҇ ÒÚ‡ˆËÓÌË‡ÌË. åÓÁ˙˜Ì‡Ú‡ ‡Ì„ËÓ„‡ÙËfl ÔË Â‰ÌÓ ÓÚ ‰Âˆ‡Ú‡ Â Ò ·ÂÎÂÁË Á‡ moya-moya (ÙË„. 4 ·).

4 2 3 2 1

àÏÛÌÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl Ç ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ· aCL ÔË ‚Ò˘ÍË 9 ‰Âˆ‡. èË 7 ÓÚ 9-Ú ‰Âˆ‡ (77,8%) aCL Ò‡ ÓÚ IgG ÚËÔ, ‡ ÔË 4 (44,4%)ÓÚ IgM ÚËÔ: Ò‡ÏÓ Ò aCL Ò‡ ÓÚ IgG ÚËÔ Ò‡ 5 ‰Âˆ‡, Ò aCL Ò‡ ÓÚ Igå ÚËÔ- 2 ‰Âˆ‡, ÔË 2 ‰Âˆ‡ ‰ÌÓ‚ÂÏÂÌÌÓ Ò‡ ÛÒÚ‡-

êÖáìãíÄíà ÄÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Â ÛÒÚ‡ÌÓ‚ÂÌ ÔË 9 ÓÚ 13Ú ‰Âˆ‡ (69,2%) Ò “ˉËÓÔ‡Ú˘ÌË” ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË Ò Ì‡˜‡ÎÓ ÏÂÊ‰Û 9- ÏÂÒ˜̇ Ë 13 „Ӊ˯̇ ‚˙Á‡ÒÚ. äÎËÌ˘̇ ı‡‡ÍÚÂËÒÚË͇ åÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË ÔË ‰Âˆ‡Ú‡ Ò ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò ‡Á‚Ë‚‡Ú ÓÒÚÓ (n=8) ËÎË ıÓÌ˘ÌÓ-ÔÓ„ÂÒË‡˘Ó (ÔÒ‚‰ÓÚÛÏÓÌÓ) (n=1). 燘‡ÎÌËÚ ÍÎËÌ˘ÌË ÔÓfl‚Ë Ò‡ Ô‰ÒÚ‡‚ÂÌË Ì‡ 퇷Î.1 ê‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ Ò Ô‰ıÓʉ‡ ÓÚ ÙÓ͇ÎÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË Ë ÏË„ÂÌÓÔÓ‰Ó·ÌÓ „·‚Ó·Ó퇷Î. 1. 燘‡ÎÌË ÒË̉ÓÏË Ë ÒËÏÔÚÓÏË ÔË ‰Âˆ‡Ú‡ Ò ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ë ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ëË̉ÓÏË Ë ÒËÏÔÚÓÏË ‚ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ îÓ͇ÎÂÌ ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ êˆˉ˂Ë‡˘Ó “ÏË„ÂÌÓÔÓ‰Ó·ÌÓ” „·‚Ó·ÓÎË ïÂÏËÔ‡ÂÚ˘ÂÌ ÒË̉ÓÏ ÖÍÒÚ‡ÔË‡ÏˉÂÌ ıÓÂÓ‡ÚÂÚÓÁÂÌ ÒË̉ÓÏ ñÂ·Â·ÂÌ ÒË̉ÓÏ

159

(44,4%) (22,2%) (33,3%) (22,2%) (10,1%)


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„ 1. äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ ê.á. 2 „. ÒΉ ‚ÚÓËfl ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ Ò ‰‡ÌÌË Á‡ ÏÌÓÊÂÒÚ‚ÂÌË ıËÔÓ‰ÂÌÁÌË ÎÂÁËË ‚ Îfl‚Ó ÚÂÏÔÓ‡ÎÌÓ Ë Ó͈ËÔËÚ‡ÎÌÓ Ë ‚ ‰flÒÌÓ ÙÓÌÚ‡ÎÌÓ Ë Ó͈ËÔËÚ‡ÎÌÓ Ë ‰ËÙÛÁ̇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl. ÌÓ‚ÂÌË aCL ÓÚ IgG Ë IgM (퇷Îˈ‡ 2). èË ÔÓÒΉfl‚‡ÌÂ, ÔÂÒËÒÚË‡˘Ë aCL Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË 3 ‰Âˆ‡ ÒΉ 3 ‰Ó 4 „.: ÓÚ IgG ÚËÔ ÔË 2 ‰Âˆ‡, ÛÒÔÓ‰ÌÓ Ò ÔÓ„ÂÒË‡˘ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ (n=1) Ë äí ‰‡ÌÌË Á‡ ÔÓ„ÂÒË‡˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl (n=2) Ë ÓÚ IgM ÚËÔ ÔË ‰ÂÚÂÚÓ Ò ıÓÌ˘ÌÓ-ÔÓ„ÂÒË‡˘Ó, ÔÒ‚‰ÓÚÛÏÓÌÓ ‡Á‚ËÚËÂ. çÂÁ‡‚ËÒËÏÓ ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ ‡ÌÚ˄·‰ÍÓÏÛÒÍÛÎÌË Ë ‡ÌÚËÏËÚÓıÓ̉ˇÎÌË ‡ÌÚËÚ· (n=3), ‡ÌÚËÌÛÍ·ÌË ‡ÌÚËÚ· Ë LE ÍÎÂÚÍË (n=2), ÔË Ô‡ˆËÂÌÚËÚ ÎËÔÒ‚‡ Ô˙Î̇ڇ ÍÓÌÒÚ·ˆËfl Á‡ ‰Ë‡„ÌÓÁ‡Ú‡ “Lupus erthematodes disseminatus”. êÂÁÛÎÚ‡ÚËÚ ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ aCL ÓÚ IgG ÚËÔ Ë IgM ÚËÔ Ë Ò˙˜ÂÚ‡ÌËÂÚÓ ËÏ Ë ıÓ‰‡ ̇ ÏÓÁ˙˜Ì‡Ú‡ ËÒıÂÏËfl (‰ÌÓÍ‡ÚÌË, ˆˉ˂Ë‡˘Ë èàçåä Ë/ËÎË ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ËÎË ÔÒ‚‰ÓÚÛÏÓÌÓ ÔÓÚ˘‡ÌÂ) ÔË 9-Ú ‰Âˆ‡ Ò‡ Ô‰ÒÚ‡‚ÂÌË Ì‡ 퇷Îˈ‡ 2. 燷β‰ÂÌË 1. ê.á. è˙‚Ë ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‡Á‚Ë‚‡ ̇ 9 ÏÂÒˆ‡, Ò ÙÓ͇ÎÂÌ ÂÔËÎÂÔÚ˘ÂÌ ÒÚ‡ÚÛÒ ‚ ÎÂ‚Ë Í‡ÈÌˈË, ÔÓÒΉ‚‡˘‡ ΂ÓÒÚ‡Ì̇ ˆÂÌÚ‡Î̇ ıÂÏËÔ‡ÂÁ‡ Ë äí ‰‡ÌÌË Á‡ ËÒıÂÏ˘̇ ÎÂÁËfl ‚ ‰flÒÌÓ ÙÓÌÚ‡ÎÌÓ Ë Ô‡ËÂÚÓÓ͈ËÔËÚ‡ÎÌÓ. èË ‚ÚÓËfl ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Ì‡ 1 „.11 ÏÂÒˆ‡ ÓÒÚÓ Ò ‡Á‚Ë‚‡ ‰ÂÒÌÓÒÚ‡Ì̇ ˆÂÌÚ‡Î̇ ıÂÏËÔ‡ÂÁ‡ Ë ÒÂÌÁÓ-ÏÓÚÓ̇ ‡Ù‡ÁËfl. íÓ„‡‚‡ åêí Â Ò ‰‡ÌÌË Á‡ ÏÌÓÊÂÒÚ‚ÂÌË ıËÔÂËÌÚÂÌÁÌË ÎÂÁËË ÔË í2 Ò ‡Á΢̇ ‰‡‚ÌÓÒÚ- ÌÓ‚Ë ‚ Îfl‚Ó ÚÂÏÔÓ‡ÎÌÓ Ë ÒÚ‡Ë ‚ ‰flÒÌÓ ÙÓÌÚ‡ÎÌÓ Ë Ó͈ËÔËÚ‡ÎÌÓ, ‡ åê-ÄÉ- Ò ÒËÎÌÓ ‰ۈË‡Ì Í˙‚ÂÌ ÚÓÍ ‚ ·‡ÒÂÈ̇ ̇ ‰‚ÂÚ ͇ÓÚˉÌË ‡ÚÂËË. ÑÓ 5 „Ӊ˯̇ ‚˙Á‡ÒÚ Ò ̇·Î˛‰‡‚‡ ÔÓ„ÂÒË‡˘‡ Í‚‡‰ËÔ‡ÂÁ‡, ÍÓflÚÓ ‚ÔÓÒΉÒÚ‚Ë Ò ÒÚ‡ˆËÓÌË‡. èË äí Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔÓ„ÂÒË‡˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl (îË„.1). ç‚ÓÎӄ˘ÌÓ Ò ÍÓÌÒÚ‡ÚË‡ ÏËÍÓˆÂÙ‡ÎËfl, Í‚‡‰ËÔ‡ÂÚ˘ÂÌ ÒË̉ÓÏ, ÁËÚÂÎ̇ ‡„ÌÓÁËfl (ÔÓÁÓÔ‡„ÌÓÁËfl) Ë ‰ÍË ÙÓ͇ÎÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË Ì‡ ÙÓ̇ ̇ ΘÂÌËÂ Ò ‚‡ÎÔÓ‡Ú Ë Î‡ÏÓÚËÊËÌ. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì – ̇΢ˠ̇ aCL ÓÚ IgG ÚËÔ, ÍÓËÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú 3 Ë 4 „. ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. 燷β‰ÂÌË 2. à. å. è˙‚Ë ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ Ì‡ 6 „. Ò Ì‡˜‡ÎÂÌ ÂÔËÎÂÔÚ˘ÂÌ ÒÚ‡ÚÛÒ, ‰‚ÛÒÚ‡ÌÌË ıÓÂÓ‡ÚÂÚÓÁÌË ıËÔÂÍËÌÂÁË, ‰ÂÒÌÓÒÚ‡Ì̇ ˆÂÌÚ‡Î̇ ıÂÏËÔ‡ÂÁ‡ Ë ÒÂÌÁÓÏÓÚÓ̇ ‡Ù‡ÁËfl. èË äí Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÎÂÁËË ‚

160

îË„.2. äí ̇ „΂ÂÌ ÏÓÁ˙Í Ì‡ à.å. Ò ‰‚ÛÒÚ‡ÌÌË ıËÔÓ‰ÂÌÁÌË ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ fl‰‡ Ë Îfl‚‡ capsula interna ÔË í2. ·‡Á‡ÎÌË fl‰‡ ‰‚ÛÒÚ‡ÌÌÓ Ë ‚ Îfl‚‡ capsula interna; äÎËÌ˘ÂÌ ˆˉ˂ Ò ̇·Î˛‰‡‚‡ ÒΉ 3 ÏÂÒˆ‡ Ò ‡Á‚ËÚË ̇ ÔË‡ÏˉÌË ·ÂÎÂÁË Ë ‚ ÎÂ‚Ë Í‡ÈÌˈË, ‰ÂÏÂ̈Ëfl; è‡ÍËÌÒÓÌÓ‚ ÒË̉ÓÏ Ò ‡Á‚Ë‚‡ ÒΉ 2 „. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ- aCL ÓÚ IgG ÚËÔ, ÛÒÚ‡ÌÓ‚ÂÌË ‚ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÒΉ 4 „., ÛÒÔÓ‰ÌÓ Ò ÍÎËÌ˘ÌË ‰‡ÌÌË Á‡ ÔÓ„ÂÒË‡˘ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ Ë äí ‰‡ÌÌË Á‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl (îË„. 2). 燷β‰ÂÌË 3. Ä.Ç. ÖÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ ÓÚ 9 „Ӊ˯̇ ‚˙Á‡ÒÚ. ç‡ 10 „. ÓÒÚÓ Ò ‡Á‚Ë‚‡ ıÓÂÓ‡ÚÂÚÓÁÂÌ ÒË̉ÓÏ Ë ÓÍÛÎÓ„ËÌË ÍËÁË ÔË äí Ë åêí ‰‡ÌÌË Á‡ ‰‚ÛÒÚ‡ÌÌË ËÒıÂÏ˘ÌË ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ fl‰‡ (îË„. 3). àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ- aCL ÓÚ IgG ÚËÔ, ‡ÌÚËÏËÚÓıÓ̉ˇÎÌË, ‡ÌÚ˄·‰ÍÓÏÛÒÍÛÎÌË ‡ÌÚËÚ· Ë Â‰ÌÓÍ‡ÚÌÓ LE ÍÎÂÚÍË. èË ÔÓÒΉfl‚‡Ì ‚ ÔÓ‰˙ÎÊÂÌË ̇ 4 „.

îË„.3. äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ Ä. Ç. Ò ‰‚ÛÒÚ‡ÌÌË ıËÔÓ‰ÂÌÁÌË ÎÂÁËË ‚ ·‡Á‡ÎÌËÚ fl‰‡.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„ 4 ‡. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ ë.ä., ÒΉ ‚ÚÓËfl ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ Ò ÌÓ‚‡ ÎÂÁËfl ‚ Îfl‚Ó Ó͈ËÔËÚÓ-Ô‡ËÂÚ‡ÎÌÓ Ë ÒÚ‡‡ ÎÂÁËfl ‚ ‰flÒÌÓ Ô‡‡Í‡ÔÒÛ·ÌÓ, ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl.

îË„ 4 ·. ä‡ÓÚˉ̇ ‡Ì„ËÓ„‡ÙËfl ̇ ë.ä.Ò Ì˯ÍӂˉÂÌ ÎÛÏÂÌ Ë ÔÓ„ÂÒË‚ÌÓ ÒÚÂÒÌfl‚‡Ì ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl, ÎËÔÒ‡ ̇ ‰ËÒÚ‡Î̇ ‚ËÁÛ‡ÎËÁ‡ˆËfl Ë ÍÓ·ÚÂ‡Î̇ ÏÂʇ ÔÂÁ ‚˙̯̇ڇ ͇ÓÚˉ̇ ‡ÚÂËË Í‡ÍÚÓ ÔË moya-moya. Ìflχ ÌÓ‚ ˆˉ˂. 燷β‰ÂÌË 4. ë.ä. îÓ͇ÎÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË ‚ Îfl‚‡ ˙͇ ÓÚ 8 „. ç‡ 10 „. – ÓÒÚÓ ‡Á‚ËÚË ̇ ‡ÏÌÂÒÚ˘̇ ‡Ù‡ÁËfl Ò ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì Á‡ ÓÍÓÎÓ 15 ‰ÌË; äí ̇ „·‚ÂÌ ÏÓÁ˙Í ÚÓ„‡‚‡ Â Ò ‰‡ÌÌË Á‡ ÒÚ‡‡ ıËÔÓ‰ÂÌÁ̇ ÎÂÁËfl ‚ ‰flÒÌÓ Ô‡‡Í‡ÔÒÛ·ÌÓ. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í ÒΉ 4 ÏÂÒˆ‡ Â Ò ÔÓ„ÂÒËfl ̇ ̇ıӉ͇ڇ Ò ÔÓfl‚‡ ̇ ÌÓ‚‡ ÎÂÁËfl ‚ Îfl‚Ó Ó͈ËÔËÚÓ-Ô‡ËÂÚ‡ÎÌÓ. ç‡ 11 „. ÓÒÚÓ ‡Á‚ËÚË ̇ ˜ÂÒÚË ÙÓ͇ÎÌË ÏÓÚÓÌË ÔËÒÚ˙ÔË ‚ ÎÂ‚Ë Í‡ÈÌËˆË ‰Ó ÂÔËÎÂÔÚ˘ÂÌ ÒÚ‡ÚÛÒ, ΂ÓÒÚ‡Ì̇ ˆÂÌÚ‡Î̇ ıÂÏËÔ΄Ëfl, ΂ÓÒÚ‡Ì̇ ıÂÏˇÌÓÔÒËfl. äí Ë åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ò‡ Ò ÔÓ„ÂÒËfl ̇ Ô‡ÂÌıËÏ̇ڇ ‡ÚÓÙËfl, ÌÓ‚Ë ÎÂÁËË ‚ ‰flÒÌÓ Ô‡ËÂÚ‡ÎÌÓ, Ô‡‡ˆÂÌÚ‡ÎÌÓ Ë ÓÔÂÍÛ·ÌÓ Ë ‡Á¯Ëfl‚‡Ì ̇ ÎÂÁËËÚ ‚ ‚ Îfl‚Ó Ó͈ËÔËÚÓÔ‡ËÂÚ‡ÎÌÓ (îË„. 4. ‡.); åê‡Ì„ËÓ„‡ÙËfl – ÓÍÎÛÁËË Ì‡ ‰‚ÂÚ ‚˙Ú¯ÌË Ò˙ÌÌË ‡ÚÂËË, ÔÓıÓ‰Ëχ ‡.basilaris ‰Ó ·ËÙÛ͇ˆËflÚ‡. åÓÁ˙˜Ì‡ ԇ̇̄ËÓ„‡ÙËfl– ‰‚ÂÚ ‚˙Ú¯ÌË Ò˙ÌÌË ‡ÚÂËË Ò‡ Ò Ì˯ÍӂˉÂÌ ÎÛÏÂÌ, ÔÓ„ÂÒË‚ÌÓ ÒÚÂÒÌfl‚‡ÌÂ Ë Ì Ò ‚ËÁÛ‡ÎËÁË‡Ú ‰ËÒÚ‡ÎÌÓ, ÍÓ·ÚÂ‡Î̇ ÏÂʇ ÔÂÁ ‚˙̯ÌËÚ ͇ÓÚˉÌË ‡ÚÂËË (îË„. 4 ·); ‚ÂÚ·‡Î̇ ‡Ì„ËÓ„‡ÙËflÌ˯ÍӂˉÌË Á‡‰ÌË ÏÓÁ˙˜ÌË ‡ÚÂËË Ò ·Ó„‡Ú‡ ÍÓ·ÚÂ‡Î̇ ÏÂʇ, ͇ÍÚÓ ÔË moya-moya. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ- aCL ÓÚ Igå Ë IgG ÚËÔ ÔÓ ‚ÂÏ ̇ ÓÒÚ‡Ú‡ Ù‡Á‡ ‰Ó 2 ÏÂÒˆ‡ ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. 燷β‰ÂÌË 5. à.Ç. ç‡ 8 „.- ÔÂıӉ̇ ‡Ù‡ÁËfl, ÒΉ 1 ÏÂÒˆ- ÓÒÚÓ ‡Á‚ËÚË ̇ ‡Ï·ÎËÓÔËfl Ë ıÂÏËÔ‡ÂÁ‡ Ò ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì Á‡ ÓÍÓÎÓ 1 ÏÂÒˆ; íÓ„‡‚‡ åêí Ò ÏÌÓÊÂÒÚ‚ÂÌË ÎÂÁËË. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì - ̇΢ˠ̇ aCL

161

ÓÚ IgG ÚËÔ. ëΉ 6 ÏÂÒˆ‡- ˆÂ·Â·ÂÌ ÒË̉ÓÏ ÔË ÔÓfl‚‡ ̇ ÌÓ‚Ë ıËÔÂËÌÚÂÌÁÌË ÎÂÁËË Ó͈ËÔËÚ‡ÎÌÓ Ë Ô‡‡Í‡ÔÒÛ·ÌÓ. 燷β‰ÂÌË 6: è.ä. ç‡ 6 „. ‰ÍË ÏË„ÂÌÓÔÓ‰Ó·ÌË ÔËÒÚ˙ÔË Ò Ì‡˜‡ÎÌË Ô‡ÂÒÚÂÁËË ‚ ÎÂ‚Ë ËÎË ‰ÂÒÌË Í‡ÈÌˈË; ̇ 10 „.-ÚÂÊÍË Ë ÔÓ‰˙Îʇ‚‡˘Ë ÔÓ 4-5 ˜‡Ò‡ «ÏË„ÂÌÓÔÓ‰Ó·ÌË» ÙÓÌÚ‡ÎÌË „·‚Ó·ÓÎËfl Ò ÔÓ‚˙˘‡ÌÂ, ÙÓÚÓ- Ë ÙÓÌÓÙÓ·Ëfl ‚ 7 ÔÓÒΉӂ‡ÚÂÎÌË ‰ÌË; ÒΉ 10 ‰ÌË- ÓÒÚÓ ‡Á‚ËÚË ̇ ‡Ï‡‚ÓÁ‡ Ë ÔË‡ÏˉÌË ÂÙÎÂÍÒÌË ÔÓÏÂÌË ‚ ‰ÂÒÌË Í‡ÈÌˈË, Ò ÔÓÒΉ‚‡˘Ó ÔÓÒÚÂÔÂÌÌÓ Ô˙ÎÌÓ Ó·‡ÚÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì Á‡ ÓÍÓÎÓ 2 ÏÂÒˆ‡ ‚ ıÓ‰‡ ̇ ÍÓÚËÍÓÒÚÂÓˉÌÓ Î˜ÂÌËÂ; äí Ë åêí ‰‡ÌÌË Ó·¯ËÌË ÎÂÁËË ‚ ‰flÒÌÓ Ó͈ËÔËÚ‡ÎÌÓ Ë Îfl‚Ó Ó͈ËÔËÚÓ-Ô‡ËÂÚ‡ÎÌÓ ÔÓ ‚ÂÏ ̇ ‡Ï‡‚ÓÁ‡Ú‡ (îË„ 5). äÓÌÚÓÎ̇ åêí ÒΉ 3 ÏÂÒˆ‡Á̇˜ËÚÂÎ̇ ‰Û͈Ëfl ̇ ÎÂÁËËÚÂ. ãËÍ‚ÓÌÓ ËÁÒΉ‚‡ÌÂÌÓχÎÌÓ, ‚Íβ˜ËÚÂÎÌÓ ÎËÍ‚Ó̇ ÂÎÂÍÚÓÙÓÂÁ‡. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì - ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ· ÓÚ aCL ÓÚ Igå ÚËÔ ÔÓ ‚ÂÏ ̇ ÓÒÚ‡Ú‡ Ù‡Á‡ ‰‚ÛÍ‡ÚÌÓ, Ì„‡ÚË‚ÂÌ ÂÁÛÎÚ‡Ú ÒΉ 4 ÏÂÒˆ‡. 燷β‰ÂÌË 7: É.à. ç‡ 13 „. ˜ÂÚËÍ‡ÚÌË èàçåä ‚ ·‡ÒÂÈ̇ ̇ Îfl‚‡ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl; åêí ‰‡ÌÌË Á‡ ÏÌÓÊÂÒÚ‚ÂÌË ıËÔÂËÌÚÂÌÁÌË ÔË í2 ÎÂÁËË ‚ Îfl‚Ó ÔÂË‚ÂÌÚËÍÛ·ÌÓ Ë ‚ ‰flÒÌÓ ‰˙ηÓÍÓ Ó͈ËÔËÚ‡ÎÌÓ; àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì – aCL- 10 Í‡ÚÌÓ Ôӂ˯ÂÌË ̇ IgM, „‡Ì˘̇ ÒÚÓÈÌÓÒÚ Ì‡ IgG. 燷β‰ÂÌË 8: å.û. ç‡ 8 „. ÓÒÚÓ ‡Á‚ËÚËÂ̇ ˆÂ·Â·ÂÌ ÒË̉ÓÏ, ÔË‡ÏˉÌË ·ÂÎÂÁË ‚ Îfl‚Ó, ‰‚ÛÒÚ‡Ì̇ ÎÂÁËfl ̇ n.ocolomotorius Ë Ì‡ n. abducens ‚ Îfl‚Ó Ò Ó·‡ÚÌÓ ‡Á‚ËÚË Á‡ ÓÍÓÎÓ 30 ‰ÌË. åêí ‰‡ÌÌË Á‡ ıËÔÂËÌÚÂÌÁÌË ÔË T2 ËÁÏÂ‚‡Ì ÎÂÁËË ‚ corpus callosum, Îfl‚ thalamus, Îfl‚Ó Í‡˜Â ̇ ÏÂÁÂ̈ÂÙ‡ÎÓ̇, ‰‚ÂÚ χÎÍÓÏÓÁ˙˜ÌË ıÂÏËÒÙÂË, vermis cerebelli Ë Îfl‚Ó Ó͈ËÔËÚ‡ÎÌÓ. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì – Ôӂ˯ÂÌË aCL ÓÚ IgG ÚËÔ. ᇠ1 „. ÎËÔÒ‚‡ ÌÓ‚‡ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇. 燷β‰ÂÌË 9: Ñ.å ç‡ 13 „. ‚˙Á‡ÒÚ- ΂ÓÒÚ‡Ì̇ ˆÂÌÚ‡Î̇ ıÂÏËÔ‡ÂÁ‡ Ò ËÁ‚ÂÒÚÌÓ Ó·‡ÚÌÓ ‡Á‚ËÚËÂ Ë ÔÓÒΉ‚‡˘Ó ÒÚ‡ˆËÓÌË‡ÌÂ. åêí- ÎÂÁËfl ‚ ‰flÒ̇ capsula interna. ç‡ 14 „.- ÔÓfl‚‡ ̇ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË ‚ ÎÂ‚Ë Í‡ÈÌˈË, ÔÓ„ÂÒËfl ̇ ΂ÓÒÚ‡Ì̇ڇ ıÂÏËÔ‡ÂÁ‡, ÔË·‡‚flÌ ̇ ÔË‡ÏˉÌË ·ÂÎÂÁË ‚ ‰ÂÒÌË Í‡ÈÌˈË, ÎÂÁËfl ̇ ‰ÂÒÌËfl n.ocolomotorius. åêí- ÔÓ„ÂÒËfl Ò ıËÔÓËÌÚÂÌÁÌË ÔË í1 Ë ıËÔÂËÌÚÂÌÁÌË ÔË í2 ÎÂÁËË ‚ ‰ÂÒÌËfl Ô‡ËÂÚ‡ÎÂÌ ÎÓ·, ‰ÂÒÌËfl ڇ·ÏÛÒ, ‰ÓÁ‡ÎÌÓÚÓ Í‡˜Â ̇ ‰flÒ̇ capsula interna Ë ÒÛÔ‡ÍÓÚË͇ÎÌÓ ‚ ӷ·ÒÚÚ‡ ̇ gyrus cinguli. éÚı‚˙ÎÂÌË ÚÛÏÓÂÌ ÔÓˆÂÒ Ò ÏÓÁ˙˜Ì‡ ·ËÓÔÒËfl, ‰ÂÏËÂÎËÌËÁË‡˘ ÔÓˆÂÒ (ÌÓχÎÌË ÎËÍ‚Ó̇ ˆËÚÓÎÓ„Ëfl, ÔÓÚÂËÌË, ÂÎÂÍÚÓÙÓÂÁ‡, ËÏÛÌÓ„ÎÓ·ÛÎËÌË), MELAS ÔÓ‡‰Ë ÎËÔÒ‡ ̇ ËÁ‚ÂÒÚÌËÚ Á‡ MELAS ÏËÚÓıÓ̉ˇÎ̇ Ñçä ÏÛÚ‡ˆËË. àÏÛÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ- ÔÂÒËÒÚË‡˘Ë Ôӂ˯ÂÌË ÒÚÓÈÌÓÒÚË Ì‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ· ÓÚ IgM ÚËÔ, ‡ÌÚË-„·‰ÍÓÏÛÒÍÛÎÌË, ‡ÌÚË-ÏËÚÓıÓ̉ˇÎÌË Ë ‡ÌÚË-̇Ô˜ÌÓ̇·‡Á‰ÂÌÓÏÛÒÍÛÎÌË ‡ÌÚËÚ·. éÅëöÜÑÄçÖ èàçåä Ë ÏÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË ‚ ‰ÂÚÒ͇ڇ ‚˙Á‡ÒÚ Ò‡ ‰ÍË (‰Ó 3,1/100000). Ç ‰ËÙÂÂ̈ˇÎÌÓ-‰Ë‡„ÌÓÒÚ˘ÂÌ ÔÎ‡Ì Ò ÔÓ‚Âʉ‡Ú ̇ÒÓ˜ÂÌË ËÁÒΉ‚‡ÌËfl Á‡ ‰Ó͇Á‚‡Ì ̇ ‚Ó‰ÂÌË ÏÓÁ˙˜ÌÓ-Ò˙‰Ó‚Ë ‡ÌÓχÎËË, ÂÏ·ÓÎËË Ò Í‡‰ËÓ„ÂÌÂÌ ÔÓËÁıÓ‰, ıÂχÚÓÎӄ˘ÌË Ë ·˙·˜ÌË Á‡·ÓÎfl‚‡ÌËfl, ‚ÚÓ˘ÌË ËÎË Ô˙‚˘ÌË ÏÓÁ˙˜ÌË ‚‡ÒÍÛÎËÚË, ıÓÏÓˆËÒÚÂËÌÂÏËfl, ‰ÂÙˈËÚ Ì‡ ÔÓÚÂËÌ S Ë ÔÓÚÂËÌ C, åELAS, moya-moya. ÄÌÚË͇‰ËÓÎËÔËÌÓ‚Ë ‡ÌÚËÚ· ÓÚ IgG ÍÎ‡Ò Ò ÓÚÍË‚‡Ú ÔË 3% ‰Ó 55% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ËÎË èàçåä (3,4,11,12,17). èË Ô‡ˆËÂÌÚËÚÂ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ‚ Ï·‰‡ ‚˙Á‡ÒÚ (2,5) ÔË 60% (15/25) Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË aCL, ‡ ÔË 75% (18/24) - LA (2). èÂÁ ÔÓÒ-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„.5. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ è.ä. Ò ‰‡ÌÌË Á‡ Ó·¯ËÌË ıËÔÂËÌÚÂÁÌË ÔË í2 ÎÂÁËË ‚ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó ‰‚ÛÒÚ‡ÌÌÓ ÙÓÌÚÓÔ‡ËÂÚÓ-ÚÂÏÔÓ‡ÎÌÓ Ôӂ˜ ‚ ‰flÒÌÓ. ΉÌËÚ „Ó‰ËÌË Ò ‰Ó͇Á‡ ‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ· ÔË ‰Âˆ‡ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ‚ ‰‚ ÂÚÓÒÔÂÍÚË‚ÌË (16, 17) Ë ÚË ÔÓÒÔÂÍÚË‚ÌË ÔÓÛ˜‚‡ÌËfl (3,4,11). óÂÒÚÓÚ‡Ú‡ ̇ aCL ‚ ÔÓ-„ÓÎÂÏËÚ ÔÓÛ˜‚‡ÌËfl ̇ ‰Âˆ‡ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË, èàçåä Ë ‚ÂÌÓÁÌË ÚÓÏ·ÓÁË Ò ÍÓη‡Â ÏÂÊ‰Û 18,3% (32/185 ÓÚ Ì‡·Î˛‰ÂÌËflÚ‡ ̇ Lanthier Ë Ò˙‡‚Ú.) (11) Ë 33% (30/92 ÓÚ ÒÎÛ˜‡ËÚ ̇ De Veber Ë Ò˙‡‚Ú.) (17). èË ÔÓ-χÎÍË ÍÎËÌ˘ÌË ÍÓÌÚËÌ„ÂÌÚË aCL Ë ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Â ÛÒÚ‡ÌÓ‚ÂÌ ÏÂÊ‰Û 70% (7/10 ÓÚ Ô‡ˆËÂÌÚËÚ ̇ Baca Ë Ò˙‡‚Ú.) (4) Ë 76 % (10/13 ÓÚ Ô‡ˆËÂÌÚËÚ ̇ Angelini Ë Ò˙‡‚Ú.) (3). èӉӷ̇  ˜ÂÒÚÓÚ‡Ú‡ ̇ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ ÔË ‰Âˆ‡Ú‡ Ò ÏÓÁ˙˜ÌË ËÒıÂÏËË Ë ‚ ̇¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ- 69,2% (9 ÓÚ 13 ÒÎÛ˜‡fl). èÂӷ·‰‡‚‡Ú ÒÎÛ˜‡ËÚ ÌË Ò Ì‡Î˘Ë ̇ aCL ÓÚ IgG ÚËÔ – ÔË Ó·˘Ó 77,8% (7/9), ‚ Ò‡‚ÌÂÌËÂ Ò aCL ÓÚ IgM ÚËÔ (44,4% ËÎË 4/9); ‰ÌÓ‚ÂÏÂÌÌÓ aCL ÓÚ IgG ÚËÔ Ë IgM ÚËÔ ËÏ‡Ú 22,2% (2/9). ÄÌÚË͇‰ËÓÎËÔËÌÓ‚Ë ‡ÌÚËÚ· ÓÚ IgG ÚËÔ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔÓ-˜ÂÒÚÓ ÔË ‰Âˆ‡ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ÔÓ ‚ÂÏ ̇ ÓÒÚ‡Ú‡ Ù‡Á‡ Ë Ì‡Î˘ËÂÚÓ ËÏ Â Ò‚˙Á‡ÌÓ Ò Ôӂ˯ÂÌÓÚÓ ÚÓÏ·ÓÓ·‡ÁÛ‚‡Ì (8). Angelini Ë Ò˙‡‚Ú (3) ̇ÏË‡Ú aCL ÓÚ IgG ÚËÔ ÔË 62% (8/13), ÔÓ‰Ó·ÌË Ò‡ ÂÁÛÎÚ‡ÚËÚÂ Ë Ì‡ Baca Ë Ò˙‡‚Ú (4). Schoning Ë Ò˙‡‚Ú. (16) ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡‚ÂÌ ·ÓÈ (ÔÓ 4) ‰Âˆ‡ Ò aCL ‡ÌÚËÚ· ÓÚ IgG Ë IgM ÚËÔ. Ç ıÓ‰‡ ̇ ÔÓÒΉfl‚‡ÌÂÚÓ Ì‡ ‰Âˆ‡Ú‡ ÍÓ΢ÂÒÚ‚ÓÚÓ Ì‡ aCL ÔÓÒÚÂÔÂÌÌÓ Ì‡Ï‡Îfl‚‡ Ë Ò Ì„‡ÚË‚Ë‡. èÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË Ò ‰ËÒÍÛÚË‡ ÓÎflÚ‡ ̇ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ Í‡ÚÓ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ ÂˆË‰Ë‚Ë Ì‡ ÏÓÁ˙˜ÌËÚ ËÒıÂÏËË ‚ ‰ÂÚÒ͇ڇ Ë Ï·‰‡Ú‡ ‚˙Á‡ÒÚ (4,5,7,11). èË Ï·‰Ë Ô‡ˆËÂÌÚË Ò ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ ËÒÍ˙Ú Á‡ ˆˉ˂ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ Â 8 Ô˙ÚË ÔÓ-‚ËÒÓÍ (5), ‡ 50% (5/10) ÓÚ ‰Âˆ‡Ú‡ ‚ ÔÓÛ˜‚‡ÌÂÚÓ Ì‡ Angelini Ë Ò˙‡‚Ú. (3) Ò‡ Ò ˆˉ˂Ë‡˘Ë ÏÓÁ˙˜ÌË ËÌÙ‡Í-

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ÚË. Lanthier Ë Ò˙‡‚Ú. (11) ÒÏflÚ‡Ú, ˜Â Ôӂ˯ÂÌËÚ aCL IgG Ì Ô‰‡ÁÔÓ·„‡Ú Í˙Ï ˆˉ˂Ë‡˘Ë ÏÓÁ˙˜ÌË ËÌÒÛÎÚË ËÎË èàçåä, Ú˙È Í‡ÚÓ ‚ ÔÓÒÔÂÍÚË‚ÌÓÚÓ ÏÛÎÚˈÂÌÚÓ‚Ó ËÁÒΉ‚‡Ì ÔË 185 ‰Âˆ‡ Ò ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË ÔË 3 „Ӊ˯ÌÓ ÔÓÒΉfl‚‡Ì Ìflχ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û ˆˉ˂ËÚ ‚ „ÛÔ‡Ú‡ ̇ aCL-ÔÓÁËÚË‚ÌËÚ (26%) Ë aCL-Ì„‡ÚË‚ÌËÚ ԇˆËÂÌÚË (38%). 燯ËÚ ÂÁÛÎÚ‡ÚË Ò˙ÓÚ‚ÂÚÒÚ‚‡Ú ̇ ‰‡ÌÌËÚ Á‡ ÔÓ-‚ËÒÓÍ ËÒÍ Á‡ ˆˉ˂ ̇ ËÒıÂÏ˘ÌËÚ ËÌÒÛÎÚË ÔË ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ (66,7%) ÔË Ì‡Î˘Ë ̇ aCL ÓÚ IgG ÚËÔ, ‚˙ÔÂÍË ˜Â χÎÍËfl ÌË ·ÓÈ Ì‡·Î˛‰ÂÌËfl Ì ÔÓÁ‚ÓÎfl‚‡ ͇Ú„Ó˘ÌË ËÁ‚Ó‰Ë Á‡ ÓÎflÚ‡ ̇ ÓÚ‰ÂÎÌËÚ ÚËÔÓ‚Â aCL Á‡ ıÓ‰‡ ̇ ÏÓÁ˙˜ÌËÚ ËÒıÂÏËË. ë ÔÓ„ÂÒË‡˘‡ ‚˙‚ ‚ÂÏÂÚÓ Ó„ÌË˘Ì‡ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ Ò‡ 66,7% ÓÚ Ô‡ˆËÂÌÚËÚ ÌË (6/9 ‰Âˆ‡), ÓÚ ÍÓËÚÓ Ò ˆˉ˂Ë‡˘Ë ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ò‡ 55,6% (5/9) Ë Â‰ËÌ ·ÓÎÂÌ Â Ò ıÓÌ˘ÌÓ-ÔÓ„ÂÒË‡˘ ıÓ‰. êˆˉ˂Ë‡˘ËflÚ ËÎË ÔÓ„ÂÒË‡˘ ıÓ‰  ÔÓ˜ÂÒÚ ÔË ‰Âˆ‡Ú‡ Ò ÛÒÚ‡ÌÓ‚ÂÌË aCL ÓÚ IgG ÚËÔ (83% ËÎË 5 ÓÚ 6 Ô‡ˆËÂÌÚË, 3 ÓÚ ÍÓËÚÓ Ò‡ÏÓ Ò IgG ÚËÔ Ë 2 Ò IgG Ë Igå ÚËÔ), ‚ Ò‡‚ÌÂÌËÂ Ò ‰Âˆ‡Ú‡ Ò aCL ÓÚ Igå ÚËÔ (50% ËÎË 3 ÓÚ 6 Ô‡ˆËÂÌÚË, ÓÚ ÍÓËÚÓ Â‰ËÌ Ò‡ÏÓ Ò Igå ÚËÔ Ë 2Ò Igå Ë IgG ÚËÔ). êˆˉ˂Ë‡˘ËflÚ ËÎË ÔÓ„ÂÒË‡˘ ıÓ‰ ÔË ÚÂÁË 6 ̇·Î˛‰ÂÌËfl ÍÓÂÎË‡ Ò ÛÒÚ‡ÌÓ‚ÂÌËÚ ÔË Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl ÏÌÓÊÂÒÚ‚ÂÌË ËÒıÂÏ˘ÌË ÎÂÁËË Ò ‡Á΢̇ ‰‡‚ÌÓÒÚ ‚ ‡Á΢ÌË Ò˙‰Ó‚Ë ·‡ÒÂÈÌË Ë Ò ÔÓ„ÂÒË‡˘‡Ú‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl ÔË 3 ÓÚ Úflı (50%). àÌÚÂÂÒÂÌ Ù‡ÍÚ Â, ˜Â ‚ ÒÎÛ˜‡ËÚ ÌË Ò ÔÓ„ÂÒË‡˘ ÏÓÁ˙˜ÂÌ ‡ÚÓÙ˘ÂÌ ÔÓˆÂÒ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ë ÔÂÒËÒÚË‡˘Ë aCL ÓÚ IgG ÚËÔ (n=2) Ë IgM ÚËÔ (n=1) ÔË ÔÓÒΉfl‚‡Ì ‚ ÔÓ‰˙ÎÊÂÌË ̇ 3 „. Ç˙ÁÏÓÊÌË Ô‡ÚÓ„ÂÌÂÚ˘ÌË ÏÂı‡ÌËÁÏË Á‡ ÔÓ„ÂÒËflÚ‡ ‚ ÚÂÁË ÒÎÛ˜‡Ë Ò‡ ˜ÂÒÚËÚ ËÏÛÌÓωËË‡ÌË ÏËÍÓÚÓÏ·ÓÁË Ë ıÓÌ˘̇ ÏÓÁ˙˜Ì‡ ËÒıÂÏËfl. èÓ-˜ÂÒÚ‡Ú‡ “ÏË„Â̇” Ë ÂÔËÎÂÔÒËfl ÔË Ï·‰Ë ıÓ‡ Ë ‰Âˆ‡ Ò ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò˙˘Ó Ò ‰ËÒÍÛÚË‡ ‚ ÎËÚÂ‡ÚÛ‡Ú‡. “åË„ÂÌÓÔÓ‰Ó·ÌÓ „·‚Ó·ÓÎË” ÛÒÔÓ‰ÌÓ Ò ÔÓfl‚Ë Ì‡ «‡Û‡» ÔÓ ıÂÏËÚËÔ ÔË 2 ÓÚ Ì‡¯ËÚ ԇˆËÂÌÚË Â Ô‰¯ÂÒÚ‚‡ÎÓ ÏÂÒÂˆË ‰Ó 4 „Ó‰ËÌË ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ. Ç ÔÓ‰Ó·ÌË ÒÎÛ˜‡Ë  ‚‡ÊÌÓ ÍÓÏÔÎÂÍÒÌÓÚÓ ‡ÌÌÓ ËÁÒΉ‚‡Ì ̇ ·ÓÎÌËÚ ‰Âˆ‡ ˜ÂÁ åêí, åê-‡Ì„ËÓ„‡ÙËfl, Ú‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl Ë ËÁÒΉ‚‡Ì Á‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÌË ‡ÌÚËÚ· Á‡ ËÁflÒÌfl‚‡Ì ̇ ı‡‡ÍÚÂ‡ ̇ „·‚Ó·ÓÎËÂÚÓ- Ô˙‚˘ÌÓ ËÎË ‚ÚÓ˘ÌÓ. èË 2 ÓÚ Ì‡¯ËÚ ԇˆËÂÌÚË ÂÔËÎÂÔÚ˘ÌËfl ÒË̉ÓÏ Ô‰ıÓʉ‡ 1 „. ÔÓfl‚‡Ú‡ ̇ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ. èË 28,8% ÓÚ ‰Âˆ‡Ú‡ Ò ÂÔËÎÂÔÒËfl 10 „. ‚˙Á‡ÒÚ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‡CL, anti- b 2 glicoprotein I, and anti-protrombin ‡ÌÚËÚ·, ÍÓÂÚÓ Ô‡‚Ë ‚˙ÁÏÓÊ̇ Ô‡ÚÓ„ÂÌÂÚ˘̇ڇ ËÏ ÓÎfl Á‡ „ÂÌÂË‡ÌÂÚÓ Ì‡ ÂÔËÎÂÔÒËflÚ‡ (6). àáÇéÑà åÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË Ë èàçåä ÔË ‰Âˆ‡ Ò ‡ÌÚËÙÓÒÙÓÎËÔˉÂÌ ÒË̉ÓÏ Ò ı‡‡ÍÚÂËÁË‡Ú Ò ÏÌÓÊÂÒÚ‚ÂÌË ËÒıÂÏ˘ÌË ÎÂÁËË, ˆˉ˂Ë‡˘ÓÚÓ ÔÓÚ˘‡Ì ËÎË ÔÓ„ÂÒË‡˘Ó ‡Á‚ËÚË ̇ ÌÓ‚Ë Ì‚ÓÎӄ˘ÌË ÒË̉ÓÏË, ÛÒÔÓ‰ÌÓ Ò ÔÓ„ÂÒË‡˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl ÔË Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl. ãàíÖêÄíìêÄ 1. ÅÓ„‰‡ÌÓ‚‡ Ñ, åË·ÌÓ‚ à‚. ç‚ÓÎӄ˘ÌË ÔÓfl‚Ë Ì‡ ‡ÌÚËÙÓÒÙÓÎËÔˉÌËfl ÒË̉ÓÏ (Ó·ÁÓ). åÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl 1999; 7 (1): 40-43. 2. ä‡Î‡¯ÌËÍÓ‚‡ ã Ä, ç‡ÒÓÌÓ‚ Ö ã, ÄÎÂÍ҇̉ӂ‡ Ö ç Ë Ò˙‡‚Ú. ÄÌÚËÚ· Í ‡ÌÚËÙÓÒÙÓÎËÔˉ‡Ï Ë Ë¯ÂÏ˘ÂÒÍË ̇Û¯ÂÌËfl ÏÓÁ„Ó‚Ó„Ó ÍÓ‚ÓÓ·‡˘ÂÌËfl ‚ ÏÓÎÓ‰ÓÏ ‚ÓÁ‡ÒÚÂ. Ü. ç‚ÓÎ. èÒËıˇÚ. 1997; 97(6):59-65. 3. Angelini L, Ravelli A, Caporali R et al. High prevalence of antiphospholipid antibodies in children with idiopatic cerebral ischemia. Pediatrics 1994; 94: 500503. 4. Baca V, Garcia-Ramirez R, Ramirez - Lacayo M et al. Cerebral infarction and antiphospholipid syndrome in children. J.Rheumatol 1996; 23: 1428-1431. 5. Bray RL, Harta RG, Sherman DG et al. Antiphospholipid antibodies and cerebral ischemia in young people. Neurology 1990; 40: 1190-1196. 6. Cimaz R, Romero A, Scarano A et al. Prevalence of anti-cardiolipin, anti- ?


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2 glicoprotein I, and anti-protrombin antibodies in young patients with epilepsy. Epilepsia 2002; 43 (1): 52-59. 7. Fullerton H.J., E.von Scheven. Back to school on APL: Rethinking the risk of recurrent stroke in children. Neurology 2004; 62: 172-173. 8. Harris EN, Chan JKH, Asherson RA et al. Thrombosis, recurrent fetus loss and thrombocytopenia. Predictive value of IgG. Arch Intern Med 1986; 146: 21532156. 9. Kelly RE, Berger JR. Ischemic stroke in a girl with lupus anticoagulant. Pediatr. Neurol 1987; 3: 58-61. 10. Kenet G, Sadetzki S, Murat K et al. Factor V Leiden and phospholipids antibodies are significant risk factors for ischemic stroke in children. Stroke 2000; 31: 1283-1288. 11. Lanthier S, Kirkham FJ, Mitchell LG et al. Increased anticadiolipin antibody IgG titres do not predict recurrent stroke or TIA in children. Neurology 2004; 62: 194-200. 12. McColl MD, Chalmers EA, Thomas A et al. Factor V Leiden, prothrombin 20210G 223 A and MTHFR C677T mutations in childhood stroke. Thromb Haemost 1999; 81:690-694. 13. Riikonen R, Santavuori P. Hereditary and acquired risk factors for childhood stroke. Neuropediatrics 1994: 25:227-233. 14. Takahashi JI, Sugita K, Miyazato S et al. Antiphospholipid antibody syn-

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drome in childhood stroke. Pediatr.Neurol. 1995; 13: 323-326. 15. Roddy SM, Giang DW. Antiphosphplipid antibodies and stroke in an infant, Pediatrics 1991; 87: 933-935. 16. Schoning M, Klein R, Krageloh –Mann I et al. Antiphospholipid antibodies in cerebrovascular ischemia and stroke in childhood. Neuropediatrics 1994; 25: 814. 17. de Veber G, Montage P, Chan A et al. Prothrombotic disorders in infants and children whith cerebral thromboembolism. Arch Neurol 1998; 55:1539-1543. 18. Wilson WA, Gharavi AE, Koike T et al. International consensus statement of preliminary classification criteria for definite antiphospholipid syndrome: report of an international wokshop. Arthritis Rheum 1999; 42: 1309-1311.

A‰ÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÑÓˆ.Ç.ÅÓÊËÌÓ‚‡, ÑÂÚÒ͇ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇, ìëÅÄãçè “ë‚.ç‡ÛÏ”, ÛÎ “ã.êÛÒ‚”‹ 1, ëÓÙËfl 1113, e-mail: tchamov@bulinfo.net

éË„Ë̇ÎÌË ÒÚ‡ÚËË CLOPIDOGREL – ÖÑçéÉéÑàòçÄ èêéîàãÄäíàäÄ çÄ Åéãçà ë àëïÖåàóçà åéáöóçà àçëìãíà èêà èÄíéãéÉàü çÄ åÄÉàëíêÄãçà åéáöóçà ÄêíÖêàà Ö. LJÒË΂‡, å. äÎËÒÛÒÍË äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl åÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” SUMMARY CLOPIDOGREL - ONE YEAR FOLLOW UP OF PATIENTS WITH TRANSIENT ISCHEMIC ATTACK OR STROKE AND LARGE ARTERY DISEASES E. Vassileva, M. Klisurski 140 patients with stroke or transient ischemic attacks and large artery diseases were studied. Secondary antiplatelet prevention by Clopidogrel was carried out in one year. Arterial diseases included: 17 high grade stenoses of internal carotid artery, 94 moderate grade carotid stenoses, 18 carotid artery thromboses, 24 stenoses and 8 thromboses of the vertebral artery, 3 subclavian steal phenomenon, 18 stenoses of the basal cerebral arteries. In one year period of time no recurrent strokes and no hematological toxicity were registered. The treatment was stopped in 5% of cases due to side effects. Key words: antiplatelet therapy, Doppler sonography, ClopidÓgrel, stroke. êÖáûåÖ èÓÛ˜‚‡ÌÂÚÓ ‚Íβ˜‚‡ 140 ·ÓÎÌË Ò Ú‡ÌÁËÚÓÌË ËÒıÂÏ˘ÌË ‡Ú‡ÍË ËÎË ËÒıÂÏ˘ÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ò Ó·ÒÚÛÍÚ˂̇ Ô‡ÚÓÎÓ„Ëfl ̇ χ„ËÒÚ‡ÎÌË ÏÓÁ˙˜ÌË ‡ÚÂËË, ÔË ÍÓËÚÓ Â Ôӂ‰Â̇ ‚ÚÓ˘̇ ‡ÌÚˇ„„‡ÌÚ̇ ÔÓÙË·ÍÚË͇ Ò Clopidogrel ‚ ÔÓ‰˙ÎÊÂÌË ̇ ‰̇ „Ó‰Ë̇. ÄÚÂˇÎ̇ڇ Ô‡ÚÓÎÓ„Ëfl ‚Íβ˜‚‡ 94 ÛÏÂÂÌË ÒÚÂÌÓÁË Ì‡ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl, 17 ‚ËÒÓÍÓÒÚÂÔÂÌÌË, 18 ͇ÓÚˉÌË ÚÓÏ·ÓÁË, 24 ÒÚÂÌÓÁË Ì‡ ‚ÂÚ·‡Î̇ڇ ‡ÚÂËfl Ë 8 ÚÓÏ·ÓÁË, 3 ÔÓ‰Íβ˜Ë˜ÌË ÒÚËÈÎ ÙÂÌÓÏÂ̇, 18 ÒÚÂÌÓÁË Ì‡ ‡ÚÂËË ÓÚ ÇËÎËÁË‚Ëfl Í˙„. ᇠ‰ÌӄӉ˯ÌËfl ÔeËÓ‰ ̇ ̇·Î˛‰ÂÌË Ì ҇ „ËÒÚË‡ÌË ÂˆË‰Ë‚Ë Ì‡ ËÒıÂÏ˘ÌËfl ÏÓÁ˙˜ÂÌ Ë̈ˉÂÌÚ. ãËÔÒ‚‡ ÔÓÏfl̇ ‚ ÔÓÒΉÂÌËÚ ԇ‡ÍÎËÌ˘ÌË ÔÓ͇Á‡ÚÂÎË. èÂÍ‡Úfl‚‡Ì ̇ ΘÂÌËÂÚÓ Â Ì‡ÎÓÊÂÌÓ ‚ 5% ÓÚ ÒÎÛ˜‡ËÚ /7/140/. äβ˜Ó‚Ë ‰ÛÏË: ‡ÌÚˇ„„‡ÌÚ̇ ÚÂ‡ÔËfl, ÑÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl, Clopidogrel, ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ. ìÇéÑ ÄÌˇ„„‡ÌÚ̇ڇ ÔÓÙË·ÍÚË͇, ̇ÒÓ˜Â̇ Í˙Ï ‚ËÒÓÍÓËÒÍÓ‚Ë Ô‡ˆËÂÌÚË,  ‚‡Ê̇ ˜‡ÒÚ ÓÚ ‚ÚÓ˘̇ڇ ÔÓÙË·ÍÚË͇ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ËÚ Á‡·ÓÎfl‚‡ÌËfl. ç‡ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì Ëχ Á‡ ˆÂΠ‰ÌӄӉ˯ÌÓ ÔÓÒ-

Ήfl‚‡Ì Ìa ·ÓÎÌË Ò ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ ËÎË Ú‡ÌÁËÚÓ̇ ËÒıÂÏ˘̇ ‡Ú‡Í‡, ÂÁÛÎÚ‡Ú Ì‡ Ó·ÒÚÛÍÚ˂̇ Ô‡ÚÓÎÓ„Ëfl ̇ „ÓÎÂÏË ÏÓÁ˙˜ÌË ‡ÚÂËË, ÔË ÍÓËÚÓ Â Ôӂ‰Â̇ ‡ÌÚˇ„„‡ÌÚ̇ ÔÓÙË·ÍÚË͇ Ò Clopidogrel. éÚ˜ÂÚÂÌË Ò‡ Ì„ӂ‡Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ, Ë̉˂ˉۇÎ̇ ÔÓÌÓÒËÏÓÒÚ, ÒÚ‡Ì˘ÌË fl‚ÎÂÌËfl, ‚ÎËflÌË ‚˙ıÛ ÍÓ‡„Û·ˆËÓÌÂÌ ÒÚ‡ÚÛÒ. äãàçàóÖç äéçíàçÉÖçí à åÖíéÑàäà é·ÂÍÚ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ Ò‡ 140 ·ÓÎÌË ‚˙‚ ‚˙Á‡ÒÚÓ‚Ëfl ‰Ë‡Ô‡ÁÓÌ 46-84 „Ó‰., Ò‰̇ ‚˙Á‡ÒÚ 63 „Ó‰., ÓÚ ÍÓËÚÓ 100 Ï˙ÊÂ Ë 40 ÊÂÌË. 13 ÓÚ Úflı Ò‡ ÔÂ͇‡ÎË Ú‡ÌÁËÚÓÌË ËÒıÂÏ˘ÌË ‡Ú‡ÍË, 100 ıÂÏËÒÙÂˇÎÌË ËÒıÂÏ˘ÌË ËÌÒÛÎÚË Ë 27 ÒÚ‚ÓÎÓ‚Ë ÏÓÁ˙˜ÌË ËÌÒÛÎÚË. èË ‚Ò˘ÍË ·ÓÎÌË Â Ôӂ‰Â̇ ÔÓÒÚÓflÌÌÓ‚˙ÎÌÓ‚‡ ÂÍÒÚ‡Í‡ÌˇÎ̇ Ë Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl. ÇÒ˘ÍË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â ÓÚÍËÚ‡ ÂÍÒÚ‡Í‡ÌˇÎ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl Ò‡ ËÁÒΉ‚‡ÌË Ò ˆ‚ÂÚ̇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl (n=122). ëÚÂÔÂÌÚ‡ ̇ ͇ÓÚˉÌËÚ ÒÚÂÌÓÁË Â ÓÔ‰ÂÎÂ̇ Ò˙„·ÒÌÓ ÛÒÚ‡ÌÓ‚ÂÌË ÍËÚÂËË (1): 1) χÍÒËχÎ̇ ÒËÒÚÓÎ̇ ÒÍÓÓÒÚ; 2) ÓÚÌÓ¯ÂÌË ÔËÍ-ÒËÒÚÓÎ̇ ÒÍÓÓÒÚ Ì‡ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl /ÇëÄ/ Í˙Ï ÔËÍ-ÒËÒÚÓÎ̇ ÒÍÓÓÒÚ Ì‡ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl; 3) ÔÓˆÂÌÚ̇ ‰Û͈Ëfl ̇ ÎÛÏÂ̇, ͇ÍÚÓ Ë ÒÔÂÍÚ‡ÎÌËÚ ÔÓÏÂÌË (ÔÓ Arbeille) (2). äÓÌÚÓÎÌÓ ‰ÓÔÎÂÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì  ËÁ‚˙¯ÂÌÓ ‰‚ÛÍ‡ÚÌÓ ÔÂÁ 6 ÏÂÒˆ‡. èÓÒΉÂÌË Ò‡ ÒΉÌËÚ ԇ‡ÍÎËÌ˘ÌË ÔÓ͇Á‡ÚÂÎË: ÚÓÏ·ÓˆËÚË, ‚ÂÏ ̇ Í˙‚ÂÌÂ, ‚ÂÏ ̇ Ò˙ÒË‚‡ÌÂ, ÔÓÚÓÏ·ËÌÓ‚Ó ‚ÂÏÂ, ‡êíí, ÄSÄí, ÄLÄí - ÔÂÁ ¯ÂÒÚ ÏÂÒˆ‡. ê„ËÒÚË‡ÌË Ò‡ ‚Ò˘ÍË ÒÚ‡Ì˘ÌË ÂÙÂÍÚË, ͇ÍÚÓ Ë ÌÓ‚ÓÔÓfl‚ËÎË Ò Ú‡ÌÁËÚÓÌË ËÒıÂÏ˘ÌË ‡Ú‡ÍË ËÎË ËÒıÂÏ˘ÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË. êÖáìãíÄíà èË 102 ·ÓÎÌË Â Ì‡Îˈ ‰ÌÓÒÚ‡Ì̇, ‡ ÔË 40 ÍÓÏ·ËÌË‡Ì‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl. ìÒÚ‡ÌÓ‚ÂÌË Ò‡ Ó·˘Ó 111 ÒÚÂÌÓÁË Ì‡ ‚˙Ú¯ÌËÚ Ò˙ÌÌË ‡ÚÂËË, ÓÚ ÍÓËÚÓ 94 ÛÏÂÂÌË (50-75%), 17 ‚ËÒÓÍÓÒÚÂÔÂÌÌË (75-99%), ͇ÍÚÓ Ë 18 ͇ÓÚˉÌË ÚÓÏ·ÓÁË. ëÚÂÌÓÁË Ì‡ ‚ÂÚ·‡Î̇ڇ ‡ÚÂËfl Ò‡ „ËÒÚË‡ÌË ÔË 24 ·ÓÎÌË, ÚÓÏ·ÓÁË – ÔË


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

8 ·ÓÎÌË, ÔÓ‰Íβ˜Ë˜ÂÌ ÒÚËÈÎ ÙÂÌÓÏÂÌ – ‚ ÚË ÒÎÛ˜‡fl. èË 18 ÓÚ ·ÓÎÌËÚ ҇ ÓÚÍËÚË ÒÚÂÌÓÁË Ì‡ ‡ÚÂËË ÓÚ ÇËÎËÁË‚Ëfl Í˙„ (Ú‡·Î.1) 퇷Î.1 ÄÚÂˇÎ̇ Ô‡ÚÓÎÓ„Ëfl ‚ ËÁÒΉ‚‡ÌËfl ÍÓÌÚËÌ„ÂÌÚ ÄÚÂ- Ç˙Ú. Ò˙Ì̇ ‡ÚÂËfl Ëfl

ÄÚÂËfl ‚ÂÚ·‡ÎËÒ

è‡ÚÓ- ìÏÂÂÌË ÇËÒÓÍÓÒÚ. íÓÏ·. ÎÓ„Ëfl ëÚÂÌÓÁË ëÚÂÌÓÁË (50-75%) (75-99%) N= 94 17 18

ëÚÂÌÓÁË íÓÏ·.

ëÚËÎ ÙÂÌÓÏÂÌ

24

3

8

ÄÚ. ÇËÎËÁ. ä˙„ ëÚÂÌÓÁË

18

ê„ËÒÚË‡ÌÂÚÓ Ì‡ ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl Ë ‰Û„Ë ‡ÚÂˇÎÌË Á‡·ÓÎfl‚‡ÌËfl (Ú‡·Î. 2) ÔÓ͇Á‚‡ Ô‚‡ÎË‡Ì ̇ ‡ÚÂˇÎ̇ڇ ıËÔÂÚÓÌËfl, ‰ËÁÎËÔˉÂÏËflÚ‡, ͇ÍÚÓ Ë ÍÓÏ·ËÌË‡ÌÂ Ò ‰Û„Ë ÔÓfl‚Ë Ì‡ ‡ÚÂÓÒÍÎÂÓÚ˘ÌËfl ÔÓˆÂÒ – àÅë, ïÄçä. 퇷Î.2 êËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ åëÅ Ë ‰Û„Ë ‡ÚÂˇÎÌË Á‡·ÓÎfl‚‡ÌËfl êËÒÍÓ‚Ë Ù‡ÍÚÓË Ë ÑÛ„Ë Ò˙‰Ó‚Ë Á‡·ÓÎ. ïËÔÂÚÓÌËfl ÑËÁÎËÔˉÂÏËfl Ñˇ·ÂÚ í˛Ú˛ÌÓÔÛ¯ÂÌ àÅë Ë åà ïÄçä ÄÌ‚. ̇ ÍÓ. ‡ÓÚ‡

n= 110/140 (78%) 66/140 (47%) 20/140 (14%) 32/140(22 %) 50/140 (36%) 8/140 (6%) 5/140 (4%)

ç  ̇·Î˛‰‡‚‡ÌÓ Á̇˜ËÏÓ ÓÚÍÎÓÌÂÌË ÓÚ ÂÙÂÂÌÚÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ ËÁÒΉ‚‡ÌËÚ ԇ‡ÍÎËÌ˘ÌË ÔÓ͇Á‡ÚÂÎË: ÚÓÏ·ÓˆËÚË, ÔÓÚÓÏ·ËÌÓ‚Ó ‚ÂÏÂ, ‡PTT, ‚ÂÏ ̇ Í˙‚ÂÌÂ, ‚ÂÏ ̇ Ò˙ÒË‚‡ÌÂ, ÄSÄí, ÄLÄí ÔË ÌËÚÓ Â‰ËÌ ÓÚ ÔÓÒΉÂÌËÚ ·ÓÎÌË. éÚ ‚Ò˘ÍË 140 ·ÓÎÌË, ÔÂÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ΘÂÌËÂÚÓ Ò Clopidogrel  ̇ÎÓÊÂÌÓ ‚ 5% ÓÚ ÒÎÛ˜‡ËÚ (7/140) ÔÓ ÒΉÌËÚ Ô˘ËÌË: ÒÚÓχ¯ÂÌ Í˙‚ÓËÁÎË‚ - 1 ·ÓÎÂÌ, ‡ÎÂ„Ëfl 1 ·ÓÎ̇, ÌÂÔÓÌÓÒËÏÓÒÚ Í˙Ï Ï‰Ë͇ÏÂÌÚ‡ - 3 ·ÓÎÌË, ıÂχÚÛËfl - 1 ·ÓÎÂÌ, ÏÌÓÊÂÒÚ‚ÂÌË ÔÓ‰ÍÓÊÌË ıÂχÚÓÏË - 1 ·ÓÎ̇. 臈ËÂÌÚ͇ڇ, ÔË ÍÓflÚÓ Â Ì‡·Î˛‰‡‚‡Ì‡ ‡ÎÂ„Ëfl Í˙Ï Clopidogrel Â Ò ‡Ì‡ÏÌÂÒÚ˘ÌË Ë ÍÎËÌ˘ÌË ‰‡ÌÌË Á‡ ÔÓÎËωË͇ÏÂÌÚÓÁ̇ ‡ÎÂ„Ëfl. Clopidogrel  ÔËÎÓÊÂÌ ÔË 11 ·ÓÎÌË Ò flÁ‚‡ ̇ ‰ÛÓ‰ÂÌÛχ ‚ ıÓÌ˘ÂÌ ÒÚ‡‰ËÈ. Ö‰ËÌ ÓÚ ·ÓÎÌËÚÂ Ò ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ,  ÔÓÎÛ˜ËÎ ÒÚÓχ¯ÂÌ Í˙‚ÓËÁÎË‚ (1/11–9%). ÇÍβ˜‚‡ÌÂÚÓ Ì‡ ‡ÌÚˇ„„‡ÌÚ̇ ÚÂ‡ÔËfl ÔË ÚÓÁË ·ÓÎÂÌ ·Â¯Â ÌÂËÁ·ÂÊÌÓ, ÔÓ‡‰Ë ‚ËÒÓÍËfl ËÒÍ ÓÚ ˆˉ˂ – ÌÓ‚ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ÔË ‚ËÒÓÍÓÒÚÂÔÂÌ̇ ÒÚÂÌÓÁ‡ ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl, ÍÓflÚÓ ÓÚ‰ÂÎfl ÏËÍÓÂÏ·ÓÎË (ÛÒÚ‡ÌÓ‚ÂÌË Ò Ú‡ÌÒÍ‡ÌˇÎÌÓ ‰ÓÔÎÂÓ‚Ó ÏÓÌËÚÓË‡ÌÂ). ç‡ ÔÓ-Í˙ÒÂÌ ÂÚ‡Ô ÔË Ò˙˘Ëfl ·ÓÎÂÌ ·Â¯Â Ôӂ‰Â̇ ͇ÓÚˉ̇ Ẩ‡ÚÂÂÍÚÓÏËfl. ÇÒ˘ÍË ÓÒڇ̇ÎË Ô‡ˆËÂÌÚË Ò ‰ÛÓ‰Â̇Î̇ flÁ‚‡ ÔÓ͇Á‡ı‡ ÏÌÓ„Ó ‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ, ·ÂÁ Á‡ÒË΂‡Ì ËÎË ÔÓfl‚‡ ̇ ·ÓÎ͇ ËÎË ‰Û„Ë ÓÔ·͂‡ÌËfl ÓÚ ÒÚ‡Ì‡ ̇ „‡ÒÚÓËÌÚÂÒÚË̇ÎÌËfl Ú‡ÍÚ. Ç Â‰ËÌ ÓÚ ÒÎÛ˜‡ËÚÂ, ÔÓ‡‰Ë ËÁÍβ˜ËÚÂÎÌÓ ‚ËÒÓÍ ‡ÚÂÓÚÓÏ·ÓÚ˘ÂÌ ËÒÍ Â Ôӂ‰ÂÌÓ ÍÓÏ·ËÌË‡ÌÓ ‡ÌÚˇ„„‡ÌÚÌÓ (Clopidogrel) Ë ‡ÌÚËÍÓ‡„Û·ÌÚÌÓ (Warfarin) ΘÂÌËÂ. íÓ‚‡  ԇˆËÂÌÚ Ò ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ÚÓÏ·ÓÁ‡ ̇ ‰̇ڇ ‚˙Ú¯̇ Ò˙Ì̇ ‡ÚÂËfl Ë 60% ÒÚÂÌÓÁ‡ ̇ ‰Û„‡Ú‡, Ò ˆˉ˂Ë‡˘‡ ÚÓÏ·ÓÁ‡ ̇ ‡ÚÂËfl Ú˷ˇÎËÒ ÒΉ ‡ÚÂˇÎ̇ ÂÍÓÌÒÚÛ͈Ëfl. äÓÏ·ËÌË‡ÌÓÚÓ Î˜Â-

164

ÌË - ‡ÌÚˇ„„‡ÌÚÂÌ Ë ‡ÌÚËÍÓ‡„Û·ÌÚÂÌ ÔÂÔ‡‡Ú ·Â ÔÓÌÂÒÂÌÓ ÏÌÓ„Ó ‰Ó· ÔÂÁ ‰ÌӄӉ˯ÌËfl ÔÂËÓ‰ ̇ ÔÓÒΉfl‚‡ÌÂ. èË 4 ·ÓÎÌË Â ÓÒ˙˘ÂÒÚ‚Â̇ ͇ÓÚˉ̇ Ẩ‡ÚÂÂÍÚÓÏËfl ̇ ÛÒÚ‡ÌÓ‚Â̇ڇ ‚ËÒÓÍÓÒÚÂÔÂÌ̇ ÒÚÂÌÓÁ‡. á̇˜Ëχ ÔÓ„ÂÒËfl ‚ ÔÓˆÂÌÚ‡ ̇ ͇ÓÚˉÌËÚ ÒÚÂÌÓÁË Á‡ ‰ÌӄӉ˯ÌËfl ÔÂËÓ‰  ̇·Î˛‰‡‚‡Ì‡ ‚ 2 ÒÎÛ˜‡fl, ÔË ÍÓËÚÓ Ò ͇҇ Á‡ ÔÂÏË̇‚‡Ì ̇ ‚ËÒÓÍÓÒÚÂÔÂÌÌË ÒÚÂÌÓÁË Ì‡ ÇëÄ ‚ ͇ÓÚˉÌË ÚÓÏ·ÓÁË. èË ÌËÚÓ Â‰ËÌ ÓÚ ËÁÒΉ‚‡ÌËÚ ·ÓÎÌË Ì  „ËÒÚË‡Ì ˆˉ˂ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ëfl Ë̈ˉÂÌÚ ‚ ‡ÏÍËÚ ̇ ‰̇ „Ó‰Ë̇. éÅëöÜÑÄçÖ ÇÚÓ˘̇ڇ ‡ÌÚˇ„„‡ÌÚ̇ ÔÓÙË·ÍÚË͇ ̇χÎfl‚‡ ËÒ͇ ÓÚ ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ ÔË ·ÓÎÌËÚÂ Ò ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ËÎË ÔÂıÓ‰ÌÓ Ì‡Û¯ÂÌË ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌË ÓÚ ÌÂ͇‰ËÓ„ÂÌÂÌ ÔÓËÁıÓ‰ Ò 11-15%, ‡ ÚÓÁË ÓÚ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ÏËÓ͇‰ÂÌ ËÌÙ‡ÍÚ Ë Ò˙‰Â˜ÌÓÒ˙‰Ó‚‡ ÒÏ˙Ú Ò 15-22% (3-7). äÓ„‡ÚÓ Ò ‚ÁÂχ ¯ÂÌË Á‡ ‚ÚÓ˘̇ ‡ÌÚˇ„„‡ÌÚ̇ ÔÓÙË·ÍÚË͇, ÓÚ Á̇˜ÂÌË  ÔË Í‡Í˙‚ ÍÓÌÚËÌ„ÂÌÚ ÓÚ ·ÓÎÌË Ô‰ÒÚÓË ‰‡ ·˙‰Â Ôӂ‰Â̇. í‡Í‡ ̇ÔËÏÂ ‰Ó͇ÚÓ ÍÓÏ·Ë̇ˆËflÚ‡ Clopidogrel Ë Äspirin  ÔÓ-ÂÙÂÍÚ˂̇ ÓÚ Äspirin ÔË ·ÓÎÌË Ò ÌÂÒÚ‡·ËÎ̇ ÒÚÂÌÓ͇‰Ëfl Ë Ú‡ÍË‚‡ ÒΉ ÍÓÓ̇ÌÓ ÒÚÂÌÚË‡Ì (8, 9), ÚÓ ÔÓ ‰‡ÌÌË ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ åÄíCH Úfl Ìflχ Ô‰ËÏÒÚ‚Ó Ô‰ Clopidogrel Á‡ ‚ÚÓ˘̇ ÔÓÙË·ÍÚË͇ ÔË ·ÓÎÌË Ò ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (10). Ç ÔÓÛ˜‚‡ÌÂÚÓ åÄíCH Ó·‡˜Â Ò‡ ‚Íβ˜ÂÌË Ô‡ˆËÂÌÚË Ò ‡Á΢̇ ÂÚËÓÎÓ„Ëfl ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ, Ò˙Ò Á̇˜ËÚÂÎÌÓ Û˜‡ÒÚË ̇ ·ÓÎÌË Ò Î‡ÍÛ̇ÌË ËÌÒÛÎÚË (11), ÔË ÍÓËÚÓ Í‡ÍÚÓ Â ËÁ‚ÂÒÚÌÓ Ô‡ÚÓ„ÂÌÂÚ˘ÌËflÚ ÏÂı‡ÌËÁ˙Ï Ì  ÂÏ·Ó΢ÂÌ Ë ÍÓËÚÓ ËÏ‡Ú ÌËÒ˙Í ËÒÍ ÓÚ ‡ÌÂÌ ˆˉ˂. çÓ ‡ÍÓ Ò ‡Á„Ή‡Ú Ò‡ÏÓ ·ÓÎÌËÚÂ Ò ‡ÚÂˇÎ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl, ÂÁÛÎÚ‡ÚËÚ ·Ëı‡ ÏÓ„ÎË ‰‡ ·˙‰‡Ú Ë Ò ‰Û„ ‡ÒÔÂÍÚ. èÓÛ˜‚‡ÌÂÚÓ CARESS Ò‡‚Ìfl‚‡ ÂÙÂÍÚ‡ ̇ ÍÓÏ·Ë̇ˆËflÚ‡ Clopidogrel Ë Äspirin ‚˙ıÛ ‡ÒËÏÔÚÓÏÌËfl ÏÓÁ˙˜ÂÌ ÏËÍÓÂÏ·ÓÎËÁ˙Ï ÔË ·ÓÎÌË Ò˙Ò ÒËÏÔÚÓχÚ˘ÌË Í‡ÓÚˉÌË ÒÚÂÌÓÁË, ÔÂÊË‚flÎË ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‚ Ô‰ıÓʉ‡˘ËÚ ÚË ÏÂÒˆ‡. ìÒÚ‡ÌÓ‚Â̇  ÒË„ÌËÙË͇ÌÚ̇ ‰Û͈Ëfl ̇ ÏËÍÓÂÏ·Ó΢ÌËÚ Ò˄̇ÎË, „ËÒÚË‡ÌË ˜ÂÁ Ú‡ÌÒÍ‡ÌˇÎÌÓ ‰ÓÔÎÂÓ‚Ó ÏÓÌËÚÓË‡Ì ‚ ‡ÏÍËÚ ̇ 7 ‰Ì‚ÌÓ ÔËÎÓÊÂÌË ̇ ‰‚‡Ú‡ ωË͇ÏÂÌÚ‡, ÍÓÂÚÓ ÔÓ͇Á‚‡ Á̇˜ÂÌËÂÚÓ Ì‡ ÚÓ‚‡ ΘÂÌË Á‡ ÔÓÙË·ÍÚË͇ ̇ ‡ÌÂÌ ˆˉ˂ ÔË ·ÓÎÌË Ò Ô‡ÚÓÎÓ„Ëfl ̇ „ÓÎÂÏËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË ‚ ÓÒÚ‡Ú‡ Ù‡Á‡ (11). Ä Í‡ÍÚÓ Â ËÁ‚ÂÒÚÌÓ ÓÍÓÎÓ 30% ÓÚ ÏÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË Ò ‰˙ÎÊ‡Ú Ì‡ ‡ÚÂÓÚÓÏ·ÓÁ‡ ̇ ‡ÓÚ̇ڇ ‰˙„‡ Ë ÂÍÒÚ‡Í‡ÌˇÎÌËÚ ‡ÚÂËË (12,13) Ë ·ÓÎÌËÚÂ Ò Í‡ÓÚˉÌË ÒÚÂÌÓÁË ËÏ‡Ú ‚ËÒÓÍ ËÒÍ ÓÚ ˆˉ˂ (14). 燷β‰ÂÌËÂÚÓ ‚˙ıÛ ÍÓÌÚËÌ„ÂÌÚ ÓÚ 140 ·ÓÎÌË Ò ‡ÚÂˇÎ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl, ÔÂ͇‡ÎË ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ËÎË ÔÂıÓ‰ÌÓ Ì‡Û¯ÂÌË ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ, ÔË ÍÓËÚÓ Â Ôӂ‰Â̇ ‰ÌӄӉ˯̇ ÔÓÙË·ÍÚË͇ Ò Clopidogrel ÔÓ͇Á‚‡ ‰Ó·‡ ÔÓÌÓÒÌËÏÓÒÚ Í˙Ï Ï‰Ë͇ÏÂÌÚ‡ Ò „ËÒÚË‡ÌË ‰ÍË ÒÚ‡Ì˘ÌË fl‚ÎÂÌËfl, ‰Ó‚ÂÎË ‰Ó Ì„ӂÓÚÓ ÔÂÍ‡Úfl‚‡Ì (5%). éÚ ÓÒÓ·ÂÌÓ Á̇˜ÂÌË ҇ ̇·Î˛‰ÂÌËflÚ‡ Á‡ ‚˙ÁÏÓÊÌÓÒÚ Á‡ ÔËÎÓÊÂÌË ̇ ÚÓÁË Ï‰Ë͇ÏÂÌÚ ÔË ·ÓÎÌË ‰ÓË Ò˙Ò ÒÚÓχ¯ÌÓ-˜‚ÌË Á‡·ÓÎfl‚‡ÌËfl, ÍÓËÚÓ Ò‡ ‚ ̇ÍÚ˂̇ Ù‡Á‡, ÌÓ ÔË ÍÓËÚÓ Â Ì‡Îˈ ÓÒÓ·ÂÌÓ ‚ËÒÓÍ ËÒÍ ÓÚ ˆˉ˂ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ëfl Ë̈ˉÂÌÚ. çÂÓ·ıÓ‰Ëχ  ‚ÌËχÚÂÎ̇ ÔˆËÁ̇ Ë̉˂ˉۇÎ̇ ÔˆÂÌ͇ ‚˙‚ ‚ÒÂÍË ÔÓ‰Ó·ÂÌ ÒÎÛ˜‡È. êÂÁÛÎÚ‡ÚËÚ ÓÚ Ì‡ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì ÔÓ͇Á‚‡Ú ˜Â Clopidogrel Ì ÔÓ‚ÎËfl‚‡ ÍÓ‡„Û·ˆËÓÌÌËfl ÒÚ‡ÚÛÒ Ë ˜ÂÌÓ‰Ó·ÌËÚ ÔÓ͇Á‡ÚÂÎË, ÔÓ‡‰Ë ÍÓÂÚÓ Ì  Á‡‰˙ÎÊËÚÂÎÂÌ ÒÚËÍÚÂÌ ÍÓÌÚÓÎ. Ç˙ÔÂÍË ˜Â Á‡ ÔÂËÓ‰‡ ̇ ̇·Î˛‰ÂÌË Ì ҇ „ËÒÚË‡ÌË Â-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

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8. The Clopidogrel in Unstable Angina to prevent Recurrent Events Trial Investigators. Effects of clopidogrel in addition to aspirin in patients with acute coronary syndromes without ST- segment elevation. N Engl J Med. 2001, 345, 494-502. 9. Steinhubl S., P. Berger J. Mann et al. CREDO Investigators. Clopidogrel for the Reduction of Events During Observation. Early and sustained dual oral antiplatelet therapy following percutaneous coronary intervention: a randomized controlled trial. JAMA. 2002, 288, 2411-2420. 10. Diener H, J. Bogousslavsky, L. Brass et al. MATCH investigators. Aspirin and clopidogrel compared with clopidogrel alone after recent ischaemic stroke or transient ischaemic attack in high risk patients (MATCH): randomized, doubleblind, placebo-controlled trial. Lancet, 364, 331-337. 11. Markus H, D. Droste, M. Kaps et al. Dual Antiplatelet Therapy With Clopidogrel and Aspirin in Symptomatic Carotid Stenosis Evaluated Using Doppler Embolic Signal Detection. Circulation. 2005, 111, 2233-2240 12. Adams H., B. Bendixen, L. Kappelle et al. Classification of Subtype of Acute Ischemic Stroke. Definition for Use in a Multicenter Clinical Trial. Stroke. 1993, 24, 35-41. 13. Bogousslavsky J., G. Van Melle, F. Regli. The Lausanne Stroke Registry: Analysis of 1000 consecutive patients with first stroke. Stroke. 1988, 19, 10831092. 14. Lovett J, A. Coull, P. Rothwell. Early risk of recurrence by subtype of ischemic stroke in population-based incidence studies. Neurology. 2004, 62, 326-328. 15. CAPRIE Steering Committee. A randomized, blinded trial of Clopidogrel versus Aspirin in Patients at risk of Ishaemic Events (CAPRIE) . Lancet. 1996, 348, 1329-39.

ĉÂÒ Á‡ ÍÓÂÔÓ̉Â̈Ëfl: Ñ- Ö. LJÒË΂‡ ‰.Ï. äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl åÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇“ 1527 ëÓÙËfl , ÛÎ. ◊ÅflÎÓ ÏÓ“8 íÂÎ. 9432/597

éË„Ë̇ÎÌË ÒÚ‡ÚËË ëéåÄíéëÖçáéêçà ÖÇéäàêÄçà èéíÖçñàÄãà éí N.TIBIALIS, N.MEDIANUS à ãìåÅé-ëÄäêÄãçàíÖ ÑÖêåÄíéåà – çéêåÄíàÇçà ÑÄççà ä. äÓÒÚÓ‚, à‚. èÂÚÓ‚, à‚. à‚‡ÌÓ‚‡ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, å‰ˈËÌÒÍË ËÌÒÚËÚÛÚ - åÇê SUMMARY SOMATOSENSORY EVOKED POTENTIALS OF N. TIBIALIS, N. MEDIANUS AND LUMBOSACRAL DERMATOMA – NORMATIVE DATA Kostov K., Iv. Petrov, Iv. Ivanova The paper presents the results from the examination of somatosensory evoked potentials (SEP) of n. tibialis, n. medianus and the most frequently used in clinical practice dermatomal SEP – S1, L5, L4 and L3 – in 34 healthy persons (68 limbs). Descriptive statistics was elaborated for absolute latencies (AL), interpeak latencies (IPL), amplitudes, asymmetry left/right indices variation coefficients, upper limits of the norms for the particular components. AL had normal distribution and correlated significantly with the individual’s height. Their regression formulae vs. the height were determined by regression analysis. The inter-component IPL after stimulation of n. medianus did not correlate with height. Moderate IPL correlation was found between the lumbal potential and the first positive peak of the cortex response after stimulation of n. tibialis and the height. No correlation between AL and individual’s age and gender was found. The values of the cortex response amplitudes had not normal distribution and varied in a wide range between the individuals and the left/right shifting in a particular person. No correlation of the amplitudes was found with height, age and gender. Criteria for abnormality were defined providing for 95% CI. Key words: somatosensory evoked potentials; dermatomal somatosensory evoked potentials; normative data; criteria for abnormality

êÖáûåÖ àÁÒΉ‚‡ÌË Ò‡ ÒÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË (ëÖè) ÓÚ Ì.Ú˷ˇÎËÒ, Ì. ωˇÌÛÒ Ë Ì‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇ S1, L5, L4 Ë L3 ‰ÂχÚÓÏÌË ëÖè ÔË 34 Á‰‡‚Ë Îˈ‡ (68 Í‡ÈÌË͇). éÒ˙˘ÂÒÚ‚Â̇  ‰ÂÒÍËÔÚ˂̇ ÒÚ‡ÚËÒÚË͇ ̇ ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË (Äã), ËÌÚÂÔËÍÓ‚ËÚ ·ÚÂ̈ËË (àèã), ‡ÏÔÎËÚÛ‰ËÚÂ, Ë̉ÂÍÒË Ì‡ ‡ÒËÏÂÚËfl Îfl‚Ó/‰flÒÌÓ, ÍÓÂÙˈËÂÌÚË Ì‡ ‚‡ˇˆËfl, „ÓÌË „‡ÌËˆË Ì‡ ÌÓÏËÚ Á‡ ÓÚ‰ÂÎÌËÚ ÍÓÏÔÓÌÂÌÚË. Äã Ëχı‡ ÌÓχÎ̇ ‰ËÒÚË·ÛˆËfl Ë ÍÓÂÎË‡ı‡ Á̇˜ËÏÓ Ò ‚ËÒÓ˜Ë̇ڇ ̇ Ë̉˂ˉ‡. óÂÁ „ÂÒË‚ÂÌ ‡Ì‡ÎËÁ Ò‡ ÓÔ‰ÂÎÂÌË ÙÓÏÛÎËÚ ËÏ Á‡ „ÂÒËfl ÒÔflÏÓ ‚ËÒÓ˜Ë̇ڇ. àèã ÏÂÊ‰Û ÍÓÏÔÓÌÂÌÚËÚ ÒΉ ÒÚËÏÛ·ˆËfl ̇ Ì. ωˇÌÛÒ Ì ÍÓÂÎË‡ı‡ Ò ‚ËÒÓ˜Ë̇ڇ. ìÒÚ‡ÌÓ‚Â̇  ÛÏÂÂ̇ ÍÓ·ˆËfl ̇ àèã ÏÂÊ‰Û ÎÛÏ·‡ÎÌËflÚ ÔÓÚÂÌˆË‡Î Ë Ô˙‚ËflÚ ÔÓÁËÚË‚ÂÌ ÔËÍ Ì‡ ÍÓÓ‚Ëfl ÓÚ„Ó‚Ó, ÒΉ ÒÚËÏÛ·ˆËfl ̇ Ì. Ú˷ˇÎËÒ, Ë ‚ËÒÓ˜Ë̇ڇ. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÍÓ·ˆËfl ̇ Äã Ò ‚˙Á‡ÒÚÚ‡ Ë ÔÓÎ˙Ú Ì‡ Ë̉˂ˉËÚÂ. ëÚÓÈÌÓÒÚËÚ ̇ ‡ÏÔÎËÚÛ‰ËÚ ̇ ÍÓÓ‚ËÚ ÓÚ„Ó‚ÓË Ìflχı‡ ÌÓχÎ̇ ‰ËÒÚË·ÛˆËfl Ë ‚‡Ë‡ı‡ ‚ ¯ËÓÍ ‰Ë‡Ô‡ÁÓÌ, ͇ÍÚÓ ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚ ËÌ‰Ë‚Ë‰Ë Ú‡Í‡ Ë ‚ ÓÚ‚Âʉ‡ÌËflÚ‡ Îfl‚Ó/‰flÒÌÓ ‚ ÓÚ‰ÂÎÂÌ Ë̉˂ˉ. ç  ÛÒÚ‡ÌÓ‚Â̇ ÍÓ·ˆËfl ̇ ‡ÏÔÎËÚÛ‰ËÚÂ Ò ‚ËÒÓ˜Ë̇ڇ, ‚˙Á‡ÒÚÚ‡ Ë ÔÓ·. éÔ‰ÂÎÂÌË Ò‡ ÍËÚÂËËÚ Á‡ ‡·ÌÓÏÌÓÒÚ ‰‡‚‡˘Ë 95% „‡Ìˈ‡ ̇ ‰ÓÒÚÓ‚ÂÌÓÒÚ. äβ˜Ó‚Ë ‰ÛÏË: ÒÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË, ‰ÂχÚÓÏÌË ÒÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË, ÌÓχÚË‚ÌË ‰‡ÌÌË, ÍËÚÂËË Á‡ ‡·ÌÓÏÌÓÒÚ


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

ÖÎÂÍÚÓÏËÓ„‡ÙËflÚ‡ (ÖåÉ)  ̇ÎÓÊËÎ Ò ÏÂÚÓ‰, ÍÓÈÚÓ ÓÒË„Ûfl‚‡ ÍÎËÌ˘ÌÓ ÔÓÎÂÁ̇ Ë Ò„ÏÂÌÚÌÓ ÒÔˆËÙ˘̇ ËÌÙÓχˆËfl ÔË ‡‰ËÍÛÎÂÌË Á‡·ÓÎfl‚‡ÌËfl Á‡Òfl„‡˘Ë ˙ˆÂÚÂ Ë Í‡Í‡Ú‡. É·‚ÌËflÚ Ó„‡Ì˘ËÚÂÎÂÌ Ù‡ÍÚÓ Á‡ ÖåÉ Ó·‡˜Â Â, ˜Â Ò ËÁÒΉ‚‡ ÓÒÌÓ‚ÌÓ ÏÓÚÓÌËfl ÍÓÏÔÓÌÂÌÚ Ì‡ ‡‰ËÍÛÎÂ̇ڇ ÙÛÌ͈Ëfl, ‡ ÏÌÓ„Ó Ô‡ˆËÂÌÚË Ò ‡‰ËÍÛÎÓÔ‡ÚËË ‰‡‚‡Ú ‰ËÌÒÚ‚ÂÌÓ ÒÂÚË‚ÌË ÓÔ·͂‡ÌËfl (3). óÂÁ ÒÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË (ëÖè) ÒΉ ÒÚËÏÛ·ˆËfl ̇ ÒÏÂÒÂÌ ÌÂ‚ (ωˇÌÂÌ, ÛÎ̇ÂÌ, ‡‰Ë‡ÎÂÌ, Ú˷ˇÎÂÌ Ë ÔÂÓÌˇÎÂÌ) Ò ÚÂÒÚ‚‡Ú Ô‰ËÏÌÓ ˆÂÌÚ‡ÎÌËÚ ÒÓχÚÓÒÂÌÁÓÌË Ô˙Úˢ‡. èËÎÓÊÂÌËÂÚÓ ËÏ ÔË ‡‰ËÍÛÎÂÌË Á‡·ÓÎfl‚‡ÌËfl  ӄ‡Ì˘ÂÌÓ ÔÓ‡‰Ë χÎÍËfl ·ÓÈ ÚÓ˜ÍË Á‡ ÒÚËÏÛ·ˆËfl Ë ÔÓ‡‰Ë ÚÓ‚‡, ˜Â ÒÚËÏÛ· Ò ÔÓ‚Âʉ‡ ÔÂÁ ÌflÍÓÎÍÓ ÒÂÚË‚ÌË ÍÓÂ̘ÂÚ‡ Û˜‡ÒÚ‚‡˘Ë ‚ ËÁ„‡Ê‰‡ÌÂÚÓ Ì‡ ÌÂ‚‡ Ë Û‚‰‡Ú‡ ‚ ‰ÌÓ ÓÚ Úflı Ó·ËÍÌÓ‚ÂÌÓ Ì ‚Ó‰Ë ‰Ó ‡·ÌÓÏÌÓÒÚ (1, 2, 3, 15). ÑÂχÚÓÏÌËÚ ëÖè (ÑëÖè) ‚Íβ˜‚‡Ú Á‡ÔËÒ‚‡ÌÂÚÓ Ì‡ ‚ÓÍË‡ÌË ˆÂ·‡ÎÌË ÓÚ„Ó‚ÓË ÒΉ ÒÚËÏÛ·ˆËfl ̇ ‰ÂχÚÓÏÌÓ ËÌÂ‚Ë‡ÌË ÔÓ‚˙ıÌÓÒÚË, ÓÒË„Ûfl‚‡ÈÍË ˜ËÒÚ ÒÂÚË‚ÂÌ ‚ıÓ‰ ÓÚ ‚ÒflÍÓ ÌË‚Ó Ì‡ „˙·Ì‡˜ÌËfl ÏÓÁ˙Í. (20) ñÂÎÚ‡ ̇ ̇ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì ·Â ‰‡ Ò ÓÔ‰ÂÎflÚ ÌÓχÚË‚ÌËÚ ‰‡ÌÌË, Á‡ ̇¯‡Ú‡ ··Ó‡ÚÓËfl, ̇ ̇È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇ ëÖè ÓÚ ÒÏÂÒÂÌ ÌÂ‚ Ë ÑëÖè, Ò˙„·ÒÌÓ Òڇ̉‡ÚËÚ ̇ American Electroencephalographic Society (4).

166

åÄíÖêàÄã à åÖíéÑ àÁÒΉ‚‡ıÏ 34 Á‰‡‚Ë ‰Ó·Ó‚ÓÎˆË (68 Í‡ÈÌË͇), 19 Ï˙ÊÂ Ë 15 ÊÂÌË, ̇ ‚˙Á‡ÒÚ ÓÚ 19 ‰Ó 65 „., Ò‰̇ ‚˙Á‡ÒÚ 43.21 „. (SD – 12.44). Ä̇ÏÌÂÒÚ˘ÌÓ ·flı‡ ËÁÍβ˜ÂÌË ‰‡ÌÌË Á‡ Ù‡ÏËÎ̇ Ó·ÂÏÂÌÂÌÓÒÚ Á‡ Ì‚ÓÏÛÒÍÛÎÌË Á‡·ÓÎfl‚‡ÌËfl, ‡‰ËÍÛÎÂ̇ ·ÓÎ͇ ËÎË ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl, Á‡ı‡ÂÌ ‰Ë‡·ÂÚ ËÎË ‰Û„Ë ẨÓÍËÌÓÔ‡ÚËË, ÒË̉ÓÏ Ì‡ ÉËÎÂÌ-ŇÂ Ë ‡ÎÍÓıÓÎËÁ˙Ï. ç‡ ‚Ò˘ÍË Â ÒÌÂÚ Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ Ë ÚÓÈ Â ÌÓχÎÂÌ. ê‡ÁÔ‰ÂÎÂÌËÂÚÓ Ì‡ ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ ÔÓ ‚˙Á‡ÒÚ Â ÔÓ͇Á‡ÌÓ Ì‡ îË„.1‡. Ë îË„.1·. ç‡ í‡·Îˈ‡ 1 Ò‡ ÔÓ͇Á‡ÌË Ò‰ÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ ‚˙Á‡ÒÚÚ‡ Ë ˙ÒÚ˙Ú Ì‡ ËÁÒΉ‚‡ÌËÚ Îˈ‡ Á‡ ÓÚ‰ÂÎÌËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ ëÖè Ë ÑëÖè. àÁÔÓÎÁ‚‡Ì  ‡Ô‡‡Ú Nicolet CA-2000. á‡ÔËÒËÚ ҇ ÓÒ˙˘ÂÒÚ‚fl‚‡ÌË ‚ ·Û‰ÌÓ Ò˙ÒÚÓflÌË ̇ Ô‡ˆËÂÌÚ‡, χÍÒËχÎÌÓ ÓÚÔÛÒÌ‡Ú Ò ÓÚ‚ÓÂÌË Ó˜Ë. íÂÏÔÂ‡ÚÛ‡Ú‡ ̇ Í‡ÈÌˈËÚ  ËÁÏÂ‚‡Ì‡ Ë Â ·Ë· ‚ ‡ÏÍËÚ ̇ 29 – 33 Óë ÔÓ ‚ÂÏ ̇ ËÁÒΉ‚‡ÌÂÚÓ. ëÚËÏÛ·ˆËflÚ‡  ÓÒ˙˘ÂÒÚ‚fl‚‡Ì‡ Ò Bar ÂÎÂÍÚÓ‰ Ò ‰Ë‡ÏÂÚ˙ ̇ ‰ËÒÍÓ‚ÂÚ 9 ÏÏ. Ë ‡ÁÒÚÓflÌË ÏÂÊ‰Û Úflı 30 ÏÏ. ÄÌÓ‰‡  ÔÓÒÚ‡‚flÌ ‰ËÒÚ‡ÎÌÓ ÓÚ Í‡ÚÓ‰‡. èË·„‡ÌË Ò‡ ÔÓÒÚÓflÌÌÓÚÓÍÓ‚Ë Ô‡‚Ó˙„˙ÎÌË ËÏÔÛÎÒË Ò ÔÓ‰˙Î ÊËÚÂÎÌÓÒÚ 0.1 ms. Ë ˜ÂÒÚÓÚ‡ 2.7 Hz Á‡ n.tibialis Ë ÑëÖè, Ë 5.1 Hz Á‡ n.medianus. àÌÚÂÌÁËÚÂÚ‡ ̇ ÒÚËÏÛ·  2.5 Ô˙ÚË Ì‡‰ ÒÂÚË‚ÌËfl Ô‡„, ÌÓ Ì Ôӂ˜ ÓÚ

퇷Îˈ‡ 1. ë‰ÌË ÒÚÓÈÌÓÒÚË Ì‡ ‚˙Á‡ÒÚÚ‡ Ë ˙ÒÚ˙Ú Ì‡ ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ äÓÏÔÓÌÂÌÚ

n

„Ó‰ËÌË SD min max (Ò.ÒÚ.) Erb(Fpz)* 16 43.6 12.6 20 64 N13(Fpz) 16 43.6 12.6 20 64 N13(AC) 11 43.3 10.3 24 62 P14(AC) 11 43.3 10.3 24 62 N20(Fpz) 16 43.6 12.6 20 64 LP 21 39.3 13.8 19 65 P38 22 39.2 13.5 19 65 S1 18 39.6 14.4 19 65 L5 18 41.1 13.9 19 65 L4 16 43.9 12.4 19 65 L3 16 43.9 12.4 19 65 é·˘Ó 34 43.2 12.4 19 65 * Ç ÒÍÓ·Ë Â ÔÓÒÓ˜ÂÌÓ ÏflÒÚÓÚÓ Ì‡ ÂÙÂÂÌÚÌËflÚ ÂÎÂÍÚÓ‰ ÔË ÓÚ‚Âʉ‡ÌÂÚÓ

îË„.1a.

ê˙ÒÚ (ÒÏ) 171.1 171.1 172.8 172.8 171.1 170.7 170.9 171.9 171.9 172.8 172.8 172.2

SD

min

max

12.4 12.4 14.3 14.3 12.4 9.3 9.1 7.6 7.6 8.4 8.4 10.4

152 152 152 152 152 152 152 159 159 159 159 152

187 187 187 187 187 190 190 190 190 190 190 190

îË„.1·.


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

퇷Îˈ‡ 2. åÂÒÚ‡ ̇ ‡ÁÔÓ·„‡Ì ̇ ÒÚËÏÛÎË‡˘ËÚ ÂÎÂÍÚÓ‰Ë Á‡ ÑëÖè ÑÂχ- ä‡ÚÓ‰ ÚÓÏ L3 ç‡ 45Ó Î‡ÚÂ‡ÎÌÓ Ë ÔÓÍÒËχÎÌÓ ÓÚ ‡ÌÓ‰‡ L4 ç‡ 6 ÒÏ. ‰ËÒÚ‡ÎÌÓ ÓÚ ‰ÓÎÌËfl ˙· ̇ Ô‡Ú·ڇ ‚˙ıÛ Ï‰ˇÎ̇ڇ ÔÓ‚˙ıÌÓÒÚ Ì‡ ÚË·ËflÚ‡ L5 ç‡ 3.5 ÒÏ. ̇Á‡‰ ÓÚ „˙Ì͇ڇ ÏÂÊ‰Û 1 Ë 2 Ô˙ÒÚ S1 ç‡ 3.5 ÒÏ. ̇Á‡‰ ÓÚ ÏÂÚÓÚ‡Á‡Î̇ڇ ÒÚ‡‚‡ ̇ 5 Ô˙ÒÚ ‚˙ıÛ ‚˙̯ÌËflÚ ˙· ̇ ıÓ‰ËÎÓÚÓ

167

30 mA Á‡ ÑëÖè Ë ‰ÓÒÚ‡Ú˙˜ÂÌ ‰‡ Ô‰ËÁ‚Ë͇ ‚ˉËχ, ÏËÌËχÎ̇ ÏÛÒÍÛÎ̇ ÍÓÌÚ‡ÍˆËfl Á‡ n.medianus Ë n.tibialis. åÂÒÚ‡Ú‡ Á‡ ̇̇ÒflÌ ̇ ÒÚËÏÛ· Á‡ ÓÚ‰ÂÎÌËÚ ‰ÂχÚÓÏË Ò‡ ‰‡‰ÂÌË ‚ 퇷Îˈ‡ 2. í˷ˇÎÌËflÚ ÌÂ‚  ÒÚËÏÛÎË‡Ì ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ Á‡‰ ωˇÎÌËfl χÎÂÓÎ, ‡ ωˇÌÌËflÚ ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ Ì‡‰ „Ë‚ÌÂ̇ڇ ÒÚ‡‚‡. Ñ‚‡ Ô˙ÚË ÔÓ 500 ÒÚËÏÛ· Ò‡ ÓÒ‰Ìfl‚‡ÌË ÓÚ ‚Òfl͇ ÒÚËÏÛÎË‡Ì‡ ÏÓ‰‡ÎÌÓÒÚ Á‡ ÂÔÎË͇ˆËfl ̇ ‚˙ÎÌÓ‚ËÚ ÙÓÏË Ò ˆÂÎ ‚ÂËÙˈË‡Ì ̇ Úflı̇ڇ ÔÓÒÚÓflÌÌÓÒÚ. ê„ËÒÚ‡ˆËflÚ‡  ÓÒ˙˘ÂÒÚ‚fl‚‡Ì‡ Ò˙Ò Ò·˙ÌË, ˜‡¯ÍӂˉÌË ÖÖÉ ÂÎÂÍÚÓ‰Ë Ò ‰Ë‡ÏÂÚ˙ 10 ÏÏ., ÙËÍÒË‡ÌË Ò ÂÎÂÍÚӉ̇ Ô‡ÒÚ‡ Í˙Ï ÔÓ˜ËÒÚÂ̇ڇ ÍÓʇ. á‡ÔËÒ‚‡˘ËÚ ÂÎÂÍÚÓ‰Ë (-) ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.medianus Ò‡ ‡ÁÔÓ·„‡ÌË ‚ ÚÓ͇ڇ ̇ Erb, ‚˙ıÛ ÒÔË̇ÎÌËfl ËÁ‡ÒÚ˙Í Ì‡ 7 ¯ËÂÌ Ô¯ÎÂÌ (ë7), Ë ëÒ’ (̇ 2 ÒÏ. ̇Á‡‰ ÓÚ ë3 Ò˙ÓÚ‚ÂÚÌÓ ë4, ÍÓÌÚ‡Î‡ÚÂ‡ÎÌÓ Ì‡ ÒÚËÏÛÎË‡Ì‡Ú‡ ÒÚ‡Ì‡, ÔÓ 10-20 ÏÂʉÛ̇Ӊ̇ڇ

ÄÌÓ‰ èÓ Ò‰ËÌ̇ڇ ÎËÌËfl ̇ 10 ÒÏ. ̇‰ „ÓÌËfl ˙· ̇ Ô‡Ú·ڇ çÂÔÓÒ‰ÒÚ‚ÂÌÓ ‰ËÒÚ‡ÎÌÓ

çÂÔÓÒ‰ÒÚ‚ÂÌÓ ‰ËÒÚ‡ÎÌÓ çÂÔÓÒ‰ÒÚ‚ÂÌÓ ‰ËÒÚ‡ÎÌÓ

퇷Îˈ‡ 3. éÒÌÓ‚ÌË ÒÚ‡ÚËÒÚ˘ÂÒÍË ‰‡ÌÌË Á‡ ÍÓÏÔÓÌÂÌÚËÚ ̇ ëÖè Ë ÑëÖè ‚ Îˈ‡Ú‡ ÓÚ ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ ã‡ÚÂ̈Ëfl (ms) äÓÏÔÓÌÂÌÚ n

Mean Std.

ÄÏÔÎËÚÛ‰‡ (m V)

99% Confidence Interval Deviation of the Difference 8.83 9.94 12.39 13.64 11.89 13.71 13.23 15.59 18.32 19.52

IA

CV

ALN

Mean

Std. IA Deviation 1.2 1.8 0.23 0.8 0.13 0.9 0.61 -0.1 0.5 0.73

CV

DSEP

n.tibialis

n.medianus

Erb(Fpz)* 16 9.38 0.75 0.20 8.03% 11.26 3.1 38.71% N13(Fpz) 16 13.02 0.85 0.30 6.51% 15.13 1.39 16.55% N13(AC) 11 12.80 0.95 0.20 7.43% 15.18 1.32 9.85% P14(AC) 11 14.41 1.23 -0.50 8.57% 17.49 2.1 23.46% N20(Fpz) 16 18.92 0.82 -0.80 4.31% 20.95 2.62 19.08% IPL Erb-N13 16 3.64 0.50 3.27 4.00 0.30 13.72% 4.88 N13-N20 16 5.90 0.36 5.64 6.16 0.40 6.03% 6.79 Erb-N20 16 9.54 0.52 9.15 9.92 -0.40 5.48% 10.84 LP 21 21.38 1.38 20.53 22.24 -0.11 6.45% 24.83 0.24 0.12 -0.60 50.00% P38 22 39.21 2.23 37.87 40.56 -0.13 5.68% 44.78 3.64 2.04 8.75 56.07% IPL LP-P38 21 17.82 1.47 16.91 18.73 0.40 8.25% 21.49 P1 (S1) 18 48.92 2.41 47.28 50.57 0.60 4.92% 54.94 3.20 1.04 5.78 32.39% P1 (L5) 18 47.66 2.33 46.07 49.24 0.20 4.88% 53.47 2.91 1.55 6.79 53.31% P1 (L4) 16 41.13 2.61 39.20 43.05 0.10 6.35% 47.66 2.45 1.91 7.21 77.88% P1 (L3) 16 34.93 2.99 32.72 37.13 -0.80 8.32% 42.40 1.73 0.58 3.17 33.35% * Ç ÒÍÓ·Ë Â ÔÓÒÓ˜ÂÌÓ ÏflÒÚÓÚÓ Ì‡ ÂÙÂÂÌÚÌËflÚ ÂÎÂÍÚÓ‰ ÔË ÓÚ‚Âʉ‡ÌÂÚÓ IA: Ë̉ÂÍÒ Ì‡ ‡ÒËÏÂÚËfl ÒÚ‡Ì‡/ÒÚ‡Ì‡; CV: ÍÓÂÙˈËÂÌÚ Ì‡ ‚‡ˇˆËfl (SD/mean x 100); ALN; „Ó̇ „‡Ìˈ‡ ̇ ÌÓχڇ; IPL: ËÌÚÂÔËÍÓ‚‡ ·ÚÂÌÚÌÓÒÚ

îË„.2‡ Ë îË„.2·


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„.3‡. çÓχÎÌË ‚˙ÎÌÓ‚Ë ÙÓÏË ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.tibialis dex. ÔË Ï˙Ê Ì‡ 42„. Ë ˙ÒÚ 177 ÒÏ. ç‡ ‰ÓÎ̇ڇ „‡ÙË͇ Ò ‚ËÁÛ‡ÎËÁË‡ ÌÓχÎÂÌ ÎÛÏ·‡ÎÂÌ ÔÓÚÂ̈ˇΠÓڂ‰ÂÌ Ò˙Ò Á‡ÔËÒ‚‡˘ ÂÎÂÍÚÓ‰ ̇‰ ÒÔË̇ÎÌËfl ËÁ‡ÒÚ˙Í Ì‡ 12 ÚÓ‡Í‡ÎÂÌ Ô¯ÎÂÌ Ë ÂÙÂÂÌÚÂÌ ÂÎÂÍÚÓ‰ ‚/Û ÍÓÂÏ̇ڇ ÒÚÂ̇. ç‡ ‰Û„‡Ú‡ „‡ÙË͇ Ò ‚Ëʉ‡ ÌÓχÎ̇ WÓ·‡Á̇ ÙÓχ ̇ ÍÓÓ‚Ëfl ÔÓÚÂ̈ˇΠÁ‡ÔËÒ‡Ì ÓÚ Cz’ Ò ÂÙÂÂ̈Ëfl Fpz.

îË„.3· çÓχÎ̇ ‰ËÒÚË·ÛˆËfl ̇ ÍÓÓ‚ËÚ ÓÚ„Ó‚ÓË ÒΉ ‰ÂχÚÓÏ̇ ÒÚËÏÛ·ˆËfl ‚ ‰flÒÌÓ ÔË Ò˙˘ËflÚ Ë̉˂ˉ. èÓÒÓ˜ÂÌË Ò‡ ÏÂÒÚ‡Ú‡ ̇ ÒÚËÏÛÎË‡˘ËflÚ, Á‡ÔËÒ‚‡˘ËflÚ Ë ÂÙÂÂÌÚÌËflÚ ÂÎÂÍÚÓ‰ ÖÖÉ ÒËÒÚÂχ) ͇ÚÓ ÔË ·ËÔÓÎflÌËÚ ÏÓÌÚ‡ÊË Â ËÁÔÓÎÁ‚‡Ì Ô‰ÂÌ ˜ÂÎÂÌ ÂÎÂÍÚÓ‰ (Fpz), ‡ Á‡ ÏÓÌÓÔÓÎflÌËÚ Ô‰ÂÌ ˆÂ‚Ë͇ÎÂÌ ÂÙÂÂÌÚÂÌ ÂÎÂÍÚÓ‰ (Äë) ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ ‚˙ıÛ ÚËÓˉÌËflÚ ıÛ˘flÎ. èË ÒÚËÏÛ·ˆËfl ̇ n.tibialis Á‡ÔËÒ‚‡˘Ë ÂÎÂÍÚÓ‰Ë Ò ÔÓÒÚ‡‚flı‡ ‚˙ıÛ ÒÔË-

168

̇ÎÌËfl ËÁ‡ÒÚ˙Í Ì‡ 12 ÚÓ‡Í‡ÎÂÌ Ô¯ÎÂÌ (í12) Ë ëz’ (̇ 2 ÒÏ. Á‡‰ ëz) Ò ÂÙÂÂÌÚÌË ÂÎÂÍÚÓ‰Ë Ò˙ÓÚ‚ÂÚÌÓ Ì‡ 2 ÒÏ. ̇‰ Ô˙Ô‡ (Abd), Ë Fpz. ᇠÑëÖè  ËÁÔÓÎÁ‚‡ÌÓ Â‰ÌÓ͇̇ÎÌÓ ·ËÔÓÎflÌÓ ÓÚ‚Âʉ‡Ì ÓÚ ëz’-Fpz. ᇠÁ‡ÔËÒËÚ ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.tibialis Ë ÑëÖè  ËÁÔÓÎÁ‚‡Ì‡ ¯ËÓ˜Ë̇ ̇ ÎÂÌÚ‡Ú‡ ÓÚ 1 ‰Ó 250 Hz ÔË ÂÔÓı‡ 150 ms, ‡ ÔË Ï‰ˇÌÌËÚ ëÖè Ò˙ÓÚ‚ÂÚÌÓ ÓÚ 5 ‰Ó 1500 Hz Ë ÂÔÓı‡ 40 ms. àÁÏÂ‚‡ÌË Ò‡ ‡·ÒÓβÚÌËÚ ÔËÍÓ‚Ë Î‡ÚÂ̈ËË Ì‡ ÔÓÚÂ̈ˇÎËÚÂ, ͇ÚÓ Á‡ ‚˙ÎÌÓ‚ËÚ ÍÓÏÔÎÂÍÒË Ò W-Ó·‡Á̇ ÙÓχ ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.tibialis Ë ‰ÂχÚÓÏËÚÂ,  ËÁ˜ËÒÎfl‚‡Ì‡ ·ÚÂ̈ËflÚ‡ ̇ Ô˙‚ËflÚ ÔÓÁËÚË‚ÂÌ ÔËÍ (P38 Ò˙ÓÚ‚ÂÚÌÓ ê1). ÄÏÔÎËÚÛ‰ËÚ ̇ ÍÓÏÔÓÌÂÌÚËÚ ̇ ëÖè ÓÚ n.medianus Ë ‡ÏÔÎËÚÛ‰‡Ú‡ ̇ ãè Ò‡ ËÁÏÂ‚‡ÌË ÓÚ ËÁÓÂÎÂÍÚ˘̇ڇ ÎËÌËfl ËÎË Ô‰¯ÂÒÚ‚‡˘ËflÚ ÔÓÁËÚË‚ËÚÂÚ, Ò˙ÓÚ‚ÂÚÌÓ Ì„‡ÚË‚ËÚÂÚ, ‡ÍÓ Ò‡ ̇΢ÌË, Ë ÓÚ Ì‡È-ÌËÒ͇ڇ ÚӘ͇ ̇ Ô˙‚ËflÚ ÔÓÁËÚË‚ÂÌ ÔËÍ ‰Ó ÔË͇ ̇ N2 Á‡ ê38, Ò˙ÓÚ‚ÂÚÌÓ ê1 ÒΉ ÒÚËÏÛ·ˆËfl ̇ n. tibialis Ë ‰ÂχÚÓÏËÚÂ. èË ‚Ò˘ÍË ËÌ‰Ë‚Ë‰Ë ÒÚËÏÛ·ˆËËÚ ҇ ÓÒ˙˘ÂÒÚ‚ÂÌË ÒËÏÂÚ˘ÌÓ ‰‚ÛÒÚ‡ÌÌÓ. чÌÌËÚ ҇ Ó·‡·ÓÚÂÌË ÒÚ‡ÚËÒÚ˘ÂÒÍË Ò ÔÓ„‡ÏÂÌ ÔÓ‰ÛÍÚ SPSS 12.01 Á‡ Windows, Ô˙‚Ó̇˜‡ÎÌÓ Á‡ Îfl‚‡ Ë ‰flÒ̇ ÒÚ‡Ì‡, ÒΉ ÍÓÂÚÓ ·flı‡ ӷ‰ËÌÂÌË Á‡ ‚ÒÂÍË ËÁÒΉ‚‡Ì ÌÂ‚ ËÎË ‰ÂχÚÓÏ. àÁ˜ËÒÎÂÌË Ò‡ Ò‰ÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË Ë ‡ÏÔÎËÚÛ‰Ë Ì‡ ÍÓÏÔÓÌÂÌÚËÚÂ, Òڇ̉‡ÚÌËÚ ÓÚÍÎÓÌÂÌËfl (SD), ÓÒÌÓ‚ÌËÚ ËÌÚÂÔËÍÓ‚Ë Î‡ÚÂ̈ËË (àèã), Ë̉ÂÍÒ Ì‡ ‡ÒËÏÂÚËfl, ÍÓÂÙˈËÂÌÚ Ì‡ ‚‡ˇˆËfl (CV), „Ó̇ „‡Ìˈ‡ ̇ ÌÓχڇ (ALN) Ë ÍÓ·ˆËË Ò ‚˙Á‡ÒÚÚ‡, ˙ÒÚ‡ Ë ÔÓ·. êÖáìãíÄíà èË 22 ÓÚ ‰Ó·Ó‚ÓΈËÚ  ÓÒ˙˘ÂÒÚ‚Â̇ ÒÚËÏÛ·ˆËfl ̇ n.tibialis, ÔË 18 ÓÚ Úflı Ò‡ ÒÚËÏÛÎË‡ÌË Ë S1 Ë L5 ‰ÂχÚÓÏË, ÔË 16 - L4 Ë L3 ‰ÂχÚÓÏË, ÔË 4  ÒÚËÏÛÎË‡Ì Ë n.medianus. å‰ˇÌÌË ëÖè Ò‡ ËÁÒΉ‚‡ÌË Ó·˘Ó ÔË 16 Ô‡ˆËÂÌÚ‡, ͇ÚÓ ÔË 11 ÓÚ Úflı  ÓÒ˙˘ÂÒÚ‚ÂÌÓ Á‡ÔËÒ‚‡Ì ̇ ˆÂ‚Ë͇ÎÂÌ ÔÓÚÂ̈ˇΠ(N13) Ò ËÁÔÓÎÁ‚‡Ì ̇ ‰‚ ‡Á΢ÌË ÂÙÂÂ̈ËË – Ô‰ÂÌ ˜ÂÎÂÌ Ë Ô‰ÂÌ ˆÂ‚Ë͇ÎÂÌ ÂÎÂÍÚÓ‰. èË Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚ Ì ·Â ÔÓÎÛ˜ÂÌ ÓÚ˜ÂÚÎË‚ ãè Ë Ì ·Â ‚˙ÁÏÓÊÌÓ ÓÔ‰ÂÎflÌÂÚÓ Ì‡ ˆÂÌÚ‡Î-

îË„.3‚. çÓχÎÌË ëÖè ÒΉ ÒÚËÏÛ·ˆËfl ̇ n. medianus sin. ÔË ÊÂ̇ ̇ 46 „. ˙ÒÚ 168 ÒÏ.


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ãÄíÖçñàà ëÚÓÈÌÓÒÚËÚ ̇ ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË Ì‡ ÓÚ‰ÂÎÌËÚ ÍÓÏÔÓÌÂÌÚË Ëχı‡ Ô‡‚ËÎÌÓ ‡ÁÔ‰ÂÎÂÌËÂ. SD ̇ ·ÚÂ̈ËËÚ ̇ ÍÓÏÔÓÌÂÌÚËÚ Ò ۂÂ΢‡‚‡ı‡ Ò Û‚Â΢‡‚‡Ì ̇ ‡ÁÒÚÓflÌËÂÚÓ ÓÚ ÒÚËÏÛ· ‰Ó Á‡ÔËÒ‚‡˘ËÚ ÂÎÂÍÚÓ‰Ë. ᇠÍÓÏÔÓÌÂÌÚËÚ ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.medianus SD  Ò‰ÌÓ 1 ms, ‰Ó͇ÚÓ Á‡ ÚÂÁË ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.tibialis Ë ‰ÂχÚÓÏËÚ  2.5 ms. äÓÂÙˈËÂÌÚ˙Ú Ì‡ ‚‡ˇˆËfl Ó·‡˜Â ÓÒڇ̇ ÓÚ˜ÂÚÎË‚Ó ÔÓÒÚÓflÌÂÌ. éÚÌÓÒËÚÂÎÌÓ ‚ËÒÓÍ ëV  ̇ÏÂÂÌ Á‡ àèã Erb-N13 (13.72%) Ë ÓÚÌÓÒËÚÂÎÌÓ ÌËÒ˙Í Á‡ N20 Ë ê1 ÓÚ S1 Ë L5 ‰ÂχÚÓÏË (퇷Î.3). ç  ÛÒÚ‡ÌÓ‚Â̇ ÒË„ÌËÙË͇ÌÚ̇ ‡ÁÎË͇ ÏÂÊ‰Û ÒÚËÏÛ·ˆËËÚ ÒÚ‡Ì‡/ÒÚ‡Ì‡ ÔË ÓÚ‰ÂÎÌËÚ ÍÓÏÔÓÌÂÌÚË ( > 0.2-0.9), ͇ÚÓ Ë̉ÂÍÒ‡ ̇ ‡ÒËÏÂÚËfl  ‚ „‡ÌˈËÚ ÓÚ –1.0 ‰Ó 1.0. ë‰ÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ N13 ÔÓÚÂ̈ˇ· ÔË ËÁÔÓÎÁ‚‡Ì ̇ Äë ÂÙÂÂ̈Ëfl Ò‡ Ò 0.22 ms ÔÓ-χÎÍË ÓÚ ÚÂÁË Ì‡ Óڂ‰ÂÌËflÚ Ò Fpz ÂÙÂ퇷Îˈ‡ 4. äÓ·ˆËfl Ï/Û N13 ÔÓÚÂ̈ˇÎ˙Ú Óڂ‰ÂÌ Ò Fpz Ë AC ÂÙÂÂ̈Ëfl Pearson Correlation (c2) Sig. (2-tailed) N N13(AC) Pearson Correlation (c2) Sig. (2-tailed) N ** p<0,001(2-tailed) äÓ·ˆËflÚ‡  ÒË„ÌËÙË͇ÌÚ̇ N13(Fpz)

N13(Fpz) 1 16 0.995 0,000** 11

N13(AC) 0.995 0,000** 11 1

169

퇷Îˈ‡ 6. äÓ·ˆËfl ÏÂÊ‰Û Î‡ÚÂ̈ËËÚÂ Ë ‡ÏÔÎËÚÛ‰ËÚ ̇ ÍÓÏÔÓÌÂÌÚËÚÂ Ë ˙ÒÚ‡ äÓÏÔÓÌÂÌÚ N

n. medianus

Erb(Fpz) 16 N13(Fpz) 16 N13(AC) 11 P14(AC) 11 N20(Fpz) 16 Erb-N13 16 N13-N20 16 Erb-N20 16 LP 21 P38 22 LP_P38 21 S1 18 L5 18 L4 16 L3 16 * p < 0,05 Ë p > 0,001 ** p < 0,001

n.tibialis

ÌÓÚÓ ÔÓ‚Ó‰ÌÓ ‚ÂÏÂ. 퇷Îˈ‡ 3 ‰‡‚‡ ÓÒÌӂ̇ڇ ÒÚ‡ÚËÒÚ˘ÂÒ͇ ËÌÙÓχˆËfl Á‡ ·ÚÂ̈ËËÚÂ Ë ‡ÏÔÎËÚÛ‰ËÚ ̇ ‡Á΢ÌËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ ëÖè Ë ÑëÖè ÓÚ ÍÓÌÚÓÎ̇ڇ „ÛÔ‡. îË„. 2‡ Ë îË„. 2· Ô‰ÒÚ‡‚flÚ ·ÓÍÒÔÎÓÚÂËÚ (Ò‰̇ ÒÚÓÈÌÓÒÚ, 25 Ë 75 ÔÂÒÂÌÚËÎ, ÏËÌËχÎ̇ Ë Ï‡ÍÒËχÎ̇ ÒÚÓÈÌÓÒÚ) ̇ ÍÓÏÔÓÌÂÌÚËÚ ̇ ëÖè ÒΉ ÒÚËÏÛ·ˆËfl Ò˙ÓÚ‚ÂÚÌÓ Ì‡ n.tibialis, S1, L5, L4, L3 ‰ÂχÚÓÏË Ë n.medianus. ç‡ îË„.3‡, îË„.3· Ë îË„.3‚ Ò‡ ‰ÂÏÓÌÒÚË‡ÌË ÌÓχÎÌË ëÖè ÒΉ ÒÚËÏÛ·ˆËfl Ò˙ÓÚ‚ÂÚÌÓ Ì‡ n.tibialis, S1, L5, L4, L3 ‰ÂχÚÓÏË Ë Ì‡ n.medianus Ò ËÁÔÓÎÁ‚‡Ì ̇ Fpz Ë AC ÏÓÌÚ‡Ê.

DSEP

˛ÌË, 2005

ã‡ÚÂ̈Ëfl Correlation (r) 0.733 0.815 0.858 0.833 0.764 0.448 0.030 0.448 0.733 0.867 0.615 0.746 0.688 0.809 0.520

Sig. (p) 0,000** 0,001* 0,000** 0,001* 0,000** 0.082 0.911 0.082 0,000** 0,000** 0,02* 0,000** 0,000** 0,001* 0.039

ÄÏÔÎËÚÛ‰‡ CorrelaSig. (p) tion (r) -0.325 0.220 -0.172 0.712 -0.191 0.633 -0.346 0.258 -0.103 0.846

0.216 -0.099

0.642 0.676

0.278 0.522 -0.215 -0.271

0.264 0.026 0.423 0.311

LP: 0.108 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +2.894 P38: 0.21 x ‚ËÒÓ˜Ë̇ (ÒÏ) +3.163 LP-P38: 0.104 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +0.018 S1(P1): 0.236 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +8.378 L5(P1): 0.21 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +11.548 L4(P1): 0.264 ı ‚ËÒÓ˜Ë̇ (ÒÏ) –4.289 L3(P1): 0.194 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +2.508 Erb: 0.044 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +1.788 N13: 0.062 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +2.352 N20: 0.01 ı ‚ËÒÓ˜Ë̇ (ÒÏ) +5.751 ç‡ îË„.4‡ Ë îË„.4·  ÔÓ͇Á‡ÌÓ ‡ÁÔ‰ÂÎÂÌËÂÚÓ Ì‡ ÒÚÓÈÌÓÒÚËÚ ̇ ·ÚÂ̈ËËÚ ̇ N13 Ë Ò˙ÓÚ‚ÂÚÌÓ Ì‡

11

퇷Îˈ‡ 5. ê‡ÁÎË͇ Ï/Û N13 ÔÓÚÂ̈ˇÎ˙Ú Óڂ‰ÂÌ Ò Fpz Ë AC ÂÙÂÂ̈Ëfl ANOVA Sum df Mean Square F Sig. N13(Fpz):N13(AC) of Squares Between Groups 0.31 1 0.306 0.386 0,53* Within Groups 19.82 25 0.793 Total 20.12 26 *p>0,05 ãËÔÒ‚‡ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ‡ÁÎË͇ Ï/Û ‰‚ÂÚ „ÛÔË Â̈Ëfl, ÌÓ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û ‰‚‡Ú‡ ÏÓÌڇʇ Ì  ÛÒÚ‡ÌÓ‚Â̇ (c2=0.995; >0.05). (퇷Îˈ‡ 4 Ë í‡·Îˈ‡ 5) 퇷Îˈ‡ 6 ÔÓ͇Á‚‡, ˜Â ˙ÒÚ˙Ú ËÁ‡ÁÂÌÓ ÍÓÂÎË‡ Ò ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË Ì‡ ÓÚ‰ÂÎÌËÚ ëÖè ÍÓÏÔÓÌÂÌÚËÚÂ. ᇠ‚Ò˘ÍË (Ò ËÁÍβ˜ÂÌË ̇ ê1 ÒΉ ÒÚËÏÛ·ˆËfl ̇ L3 ‰ÂχÚÓÏ),  < 0.001 Ë ÍÓÂÙˈËÂÌÚ‡ ̇ ÍÓ·ˆËfl (r)  ÏÂÊ‰Û 0.68 Ë 0.85. àÁÔÓÎÁ‚‡ÈÍË „ÂÒË‚ÂÌ ‡Ì‡ÎËÁ ÛÒÚ‡ÌÓ‚ËıÏ ÒΉÌËÚ ÙÓÏÛÎË Á‡ ÍÓÂ͈Ëfl ̇ ÒÚÓÈÌÓÒÚËÚ ̇ ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË, ̇ ÓÚ‰ÂÎÌËÚ ÍÓÏÔÓÌÂÌÚË ÒÔflÏÓ ˙ÒÚ‡, ͇ÍÚÓ ÒΉ‚‡:

îË„.4‡. ÑËÒÚË·ÛˆËfl ̇ ·ÚÂ̈ËËÚ ̇ N13 ÔÓÚÂ̈ˇ· ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ‚ËÒÓ˜Ë̇ڇ ̇ Ë̉˂ˉ‡ àèã N13-N20 ÒÔflÏÓ ˙ÒÚ‡. îË„.4‡ ‰ÂÏÓÌÒÚË‡ ËÁ‡ÁÂ̇ ÎËÌÂÈ̇ Á‡‚ËÒËÏÓÒÚ, ‰Ó͇ÚÓ Ì‡ îË„.4· ڇ͇‚‡ Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡. ç ÛÒÚ‡ÌÓ‚ËıÏ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ÍÓ·ˆËfl ÏÂÊ‰Û ˙ÒÚ‡ Ë ÒÚÓÈÌÓÒÚËÚ ̇ àèã Ò ËÁÍβ˜ÂÌË ̇ àèã LP-P38 (r=0.615; p=0.02). èË ÌËÚÓ Â‰ËÌ ÓÚ ÍÓÏÔÓÌÂÌÚËÚ Ì ÛÒÚ‡ÌÓ‚ËıÏ ÒË„-


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

îË„.4·. ÑËÒÚË·ÛˆËfl ̇ ÒÚÓÈÌÓÒÚËÚ ̇ àèã N13_N20 ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ‚ËÒÓ˜Ë̇ڇ ̇ Ë̉˂ˉ‡ 퇷Îˈ‡ 7. äÓ·ˆËfl ÏÂÊ‰Û Î‡ÚÂ̈ËËÚÂ Ë ‡ÏÔÎËÚÛ‰ËÚ ̇ ÍÓÏÔÓÌÂÌÚËÚÂ Ë ‚˙Á‡ÒÚÚ‡ äÓÏÔÓÌÂÌÚ N

DSEP

n.tibialis

n. medianus

Erb(Fpz) 16 N13(Fpz) 16 N13(AC) 11 P14(AC) 11 N20(Fpz) 16 Erb-N13 16 N13-N20 16 Erb-N20 16 LP 21 P38 22 LP_P38 21 S1 18 L5 18 L4 16 L3 16 * p < 0,05 Ë p > 0,001

ã‡ÚÂ̈Ëfl Correlation (r) -0.004 -0.028 -0.116 -0.098 -0.127 -0.043 -0.222 -0.192 0.361 0.184 -0.050 -0.062 0.120 0.049 -0.328

Sig. (p) 0.989 0.917 0.733 0.774 0.641 0.875 0.408 0.476 0.108 0.412 0.829 0.808 0.636 0.857 0.214

ÄÏÔÎËÚÛ‰‡ CorrelaSig. (p) tion (r) 0.408 0.117 0.216 0.642 0.231 0.589 0.313 0.484 0.458 0.361

0.424 -0.502

0.371 0,024*

-0.359 -0.322 0.011 -0.237

0.144 0.193 0.968 0.377

ÌËÙË͇ÌÚ̇ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ÒÚÓÈÌÓÒÚËÚ ̇ ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË Ë ‚˙Á‡ÒÚÚ‡ (>0.1-0.98) (퇷Îˈ‡ 7). ÑËÒÚË·ÛˆËËÚ ̇ ·ÚÂ̈ËËÚ ̇ ‰‡‰ÂÌ ‰ÂχÚÓÏ ÒÔflÏÓ ÓÒڇ̇ÎËÚ ·flı‡ Ô‡‚ËÎÌÓ ‡ÁÔ‰ÂÎÂÌË ‚ ÓÚ‰ÂÎÌËÚ Ë̉˂ˉË, ͇ÚÓ Ò‡ÏÓ ÔË ‰‚‡Ï‡ ÓÚ ËÁÒΉ‚‡ÌËÚ ÛÒÚ‡ÌÓ‚ËıÏ ÌÂÁ̇˜ËÚÂÎÌÓ ÔÓ-ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ ·ÚÂ̈ËËÚ Á‡ P1 ÓÚ S1, ÒÔflÏÓ ê1 ÓÚ L5 ‰ÂχÚÓÏ, Ò˙ÓÚ‚ÂÚÌÓ Ò 0.92 Ë 0.64 ms. ë‰ÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ ‡ÁÎËÍËÚ ‚ ·ÚÂ̈ËËÚ ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚ ‰ÂχÚÓÏË Ò‡: S1-L5 1.6 ms, SD 1.1 ms; L5-L4 6.9 ms SD 1.8 ms; L4-L3 5.2 ms SD 2.1 ms. ëΉ ÔÂÓ·‡ÁÛ‚‡Ì ̇ ‰‡ÌÌËÚÂ Ò ÙÓÏÛÎËÚ Á‡ „ÂÒËfl ÒÔÓ‰ ‚ËÒÓ˜Ë̇ڇ Í˙Ï Ò‰̇ ÒÚÓÈÌÓÒÚ, Ì Ò ÛÒÚ‡ÌÓ‚Ë ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û ÒÚÓÈÌÓÒÚËÚ ̇ ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË Ë ÔÓ· ̇ Ë̉˂ˉËÚ (=0.141-0.890; r=0.251-0.862). ÄåèãàíìÑà ëÚÓÈÌÓÒÚËÚ ̇ ‡ÏÔÎËÚÛ‰ËÚ ̇ ÓÚ‰ÂÎÌËÚ ÍÓÏÔÓ-

170

ÌÂÌÚË Ëχı‡ ÌÂÔ‡‚ËÎÌÓ É‡ÛÒ-ã‡Ô·ÒÓ‚Ó ‡ÁÔ‰ÂÎÂÌËÂ, ÍÓÂÚÓ Ì‡ÎÓÊË ÎÓ„‡ËÚÏ˘̇ Ú‡ÌÒÙÓχˆËfl ̇ ‰‡ÌÌËÚÂ. ÄÏÔÎËÚÛ‰ËÚ ‚‡Ë‡ı‡ ‚ ¯ËÓÍ ‰Ë‡Ô‡ÁÓÌ, ͇ÍÚÓ ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚ ËÌ‰Ë‚Ë‰Ë Ú‡Í‡ Ë ‚ ÓÚ‚Âʉ‡ÌËflÚ‡ Îfl‚Ó/‰flÒÌÓ ‚ ÓÚ‰ÂÎÂÌ Ë̉˂ˉ. äÓÂÙˈËÂÌÚ‡ ̇ ‚‡ˇˆËfl Á‡ ÍÓÏÔÓÌÂÌÚËÚ Óڂ‰ÂÌË ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.tibialis Ë ‰ÂχÚÓÏËÚ  ÏÂÊ‰Û 32 Ë 77%, ‡ Á‡ ÚÂÁË ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.medianus ÏÂÊ‰Û 9 Ë 38%. (퇷Î.3) à̉ÂÍÒ‡ ̇ ‡ÒËÏÂÚËfl ÒÚ‡Ì‡/ÒÚ‡Ì‡  Ò˙ÓÚ‚ÂÚÌÓ ÏÂÊ‰Û 3.17 Ë 7.21 ÔË ÒÚËÏÛ·ˆËfl ̇ Í‡Í‡Ú‡ Ë ÏÂÊ‰Û –1 Ë 1.8 ÒΉ ÒÚËÏÛ·ˆËfl ̇ ˙ˆÂÚÂ. èË Â‰ËÌ ÓÚ ËÁÒΉ‚‡ÌËÚ ÛÒÚ‡ÌÓ‚ËıÏ ‡ÁÎË͇ ÒÚ‡Ì‡/ÒÚ‡Ì‡ ‚ ‡ÏÔÎËÚÛ‰‡Ú‡ ̇ ê1 ÒΉ ÒÚËÏÛ·ˆËfl ̇ ã5 ‰ÂχÚÓÏ ‡‚̇ ̇ 3:1. ç  ÛÒÚ‡ÌÓ‚Â̇ ÒË„ÌËÙË͇ÌÚ̇ ÍÓ·ˆËfl ÏÂÊ‰Û ÒÚÓÈÌÓÒÚËÚ ̇ ‡ÏÔÎËÚÛ‰ËÚÂ, ‚ËÒÓ˜Ë̇ Ë ‚˙Á‡ÒÚ (>0.17-0.96). àÁÍβ˜ÂÌË  ÍÓ·ˆËflÚ‡ ÏÂÊ‰Û ‡ÏÔÎËÚÛ‰‡Ú‡ ̇ ê38 Ë ‚˙Á‡ÒÚÚ‡ (=0.024; r = -0.502). (퇷Îˈ‡ 7) í˙È Í‡ÚÓ r Â Ò ÓÚˈ‡ÚÂÎÂÌ ÁÌ‡Í Â Ì‡Îˈ ӷ‡Ú̇ ‚˙Á͇ Ú.Â. Ò Û‚Â΢‡‚‡ÌÂÚÓ Ì‡ ‚˙Á‡ÒÚÚ‡ ‡ÏÔÎËÚÛ‰‡Ú‡ ̇ ÔÓÚÂ̈ˇ· ̇χÎfl‚‡. éÅëöÜÑÄçÖ èË ‚Ò˘ÍË Îˈ‡ ÓÚ ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ ÒÏ ÔÓÎÛ˜ËÎË ÓÚ˜ÂÚÎË‚Ë, ÔÓÁ‚ÓÎfl‚‡˘Ë ËÁÏÂ‚‡Ì ÍÓÏÔÓÌÂÌÚË Ì‡ ëÖè ÒΉ ÒÚËÏÛ·ˆËfl ̇ n.medianus Ë n.tibialis, Ë ÎÛÏ·ÓÒ‡Í‡ÎÌËÚ ‰ÂχÚÓÏË (ÔË Â‰ËÌ Ì ·Â Óڂ‰ÂÌ ãè). àÁÔÓÎÁ‚‡ÎË ÒÏ χÍÒËχÎÌÓ ÓÔÓÒÚÂÌË ÏÓÌÚ‡ÊË Ò Ó„Î‰ Á‡ÔËÒ‚‡Ì ̇ ̇È-ÔÓÒÚÓflÌÌËÚ ‚˙ÎÌÓ‚Ë ÙÓÏË Ë ÒÍ˙Òfl‚‡Ì ̇ ‚ÂÏÂÚÓ Á‡ ËÁÒΉ‚‡ÌÂ. èÓÎÛ˜ÂÌËÚ ÓÚ Ì‡Ò ÂÁÛÎÚ‡ÚË Ò‡ Ò˙ÔÓÒÚ‡‚ËÏË Ò ÚÂÁË ÔÛ·ÎËÍÛ‚‡ÌË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ (1-5, 7, 9, 12-14, 16, 17, 19, 20, 21, 23). çÓχÎÌÓÚÓ ‡ÁÔ‰ÂÎÂÌËÂ, χÎÍËflÚ Ë̉ÂÍÒ Ì‡ ‡ÒËÏÂÚËfl Îfl‚Ó/‰flÒÌÓ Ë ÌËÒÍËflÚ ÍÓÂÙˈËÂÌÚ Ì‡ ‚‡ˇˆËfl ̇ ëÑ, Ô‡‚flÚ ‡·ÒÓβÚÌËÚÂ Ë ËÌÚÂÔËÍÓ‚ËÚ ·ÚÂ̈ËË ÒË„ÛÂÌ ‰Ë‡„ÌÓÒÚ˘ÂÌ ÍËÚÂËË Á‡ ‡·ÌÓÏÌÓÒÚ, ‡ ÒËÏÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ÏÓÊ ‰‡ Û‚ÂÎË˜Ë ÒÂÌÁËÚË‚ÌÓÒÚÚ‡ ̇ ÏÂÚÓ‰Ë͇ڇ. 燯ËÚ ÂÁÛÎÚ‡ÚË ÔÓÚ‚˙ʉ‡‚‡Ú ‰‡ÌÌËÚ ÓÚ ÎËÚÂ‡ÚÛ‡Ú‡, ˜Â Á̇˜Ëχڇ ÍÓ·ˆËfl ÏÂÊ‰Û ˙ÒÚ˙Ú Ë ‡·ÒÓβÚÌËÚ ·ÚÂ̈ËË ËÁËÒÍ‚‡ ÔË·„‡Ì ̇ „ÂÒË‚ÌË ÙÓÏÛÎË + 2SD Á‡ ‰ÓÒÚÓ‚ÂÌÓ Ë‰ÂÌÚËÙˈË‡Ì ̇ ‡·ÌÓÏÌËÚ ·ÚÂ̈ËË Ì‡ ÓÚ„Ó‚ÓËÚ (6, 7, 12, 19, 20, 23). Ç ÒËÌıÓÌ Ò‡ Ë ‰‡ÌÌËÚÂ, ˜Â àèã ̇ ωˇÌÌËÚ ëÖè Ì ÍÓÂÎË‡Ú ‰Ó·Â Ò ‚ËÒÓ˜Ë̇ڇ ̇ Ë̉˂ˉ‡ (8, 10, 15, 20, 23). Lastimosa (1982) Ë Tsuji (1984) Ì ÛÒÚ‡ÌÓ‚fl‚‡Ú ڇ͇‚‡ ÍÓ·ˆËfl ‚ àèã Ë ÔË ÒÚËÏÛ·ˆËfl ̇ n.tibialis. Ä‚ÚÓËÚ ӷflÒÌfl‚‡Ú Ù‡ÍÚ‡, ˜Â Ì ‚Ë̇„Ë Â Ì‡Îˈ ‰ËÂÍÚ̇ ‡Ì‡ÚÓÏ˘̇ ÍÓ·ˆËfl ÏÂÊ‰Û ‡ÁÏÂËÚ ̇ ÚflÎÓÚÓ Ë ‰˙ÎÊË̇ڇ ̇ „. ÏÓÁ˙Í (14, 23). èË Ì‡¯ËÚ ËÁÒΉ‚‡ÌËfl ÌË ÛÒÚ‡ÌÓ‚fl‚‡Ï χ͇ Ë Ì ÚÓÎÍÓ‚‡ ËÁ‡ÁÂ̇, ͇ÍÚÓ ÔË ÓÚ‰ÂÎÌËÚ ‚˙ÎÌÓ‚Ë ÍÓÏÔÓÌÂÌÚË, ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ÍÓ·ˆËfl ÏÂÊ‰Û LP-P38 àèã Ë ‚ËÒÓ˜Ë̇ڇ. é·‡·ÓÚ͇ڇ ̇ ‰‡ÌÌËÚ ÓÚ ÓÒÚ‡ÎÌÓ-͇ۉ‡ÎÌËfl ÏÓ‰ÂΠ̇ ‰ÂχÚÓÏÌËÚ ÓÚ„Ó‚ÓË, ˜ÂÁ Ò‡‚ÌÂÌË ̇ ÌË‚Ó ÒÔflÏÓ ÌË‚Ó, ÔÓ͇Á‡ ‚ËÒÓÍ ÍÓÂÙˈËÂÌÚ Ì‡ ÍÓÌÍÓ‰‡ÌÚÌÓÒÚ, ÍÓÂÚÓ ÓÁ̇˜‡‚‡ ˜Â Ò˙˘ÂÒÚ‚Û‚‡ ÔÓÒÚÓflÌÂÌ ‰ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‰ËÒÚË·ÛˆËËÚ ̇ ·ÚÂ̈ËËÚ ̇ ÓÚ„Ó‚ÓËÚ ÓÚ Â‰ÌÓ ‰ÂχÚÓÏÌÓ ÌË‚Ó Í˙Ï ‰Û„Ó. íÂÁË ‰‡ÌÌË Ò‡ ÔÓÚ‚˙‰ÂÌË Ë ÓÚ ‰Û„Ë ‡‚ÚÓË (20, 22). íÓ‚‡ ÓÁ̇˜‡‚‡, ˜Â  ÛÏÂÒÚÌÓ ‰‡ Ò Ô‡‚Ë Ò‡‚ÌÂÌË ÌË‚Ó/ÌË‚Ó, Ú˙È Í‡ÚÓ ÒÚÓÈÌÓÒÚÚ‡ ̇ ÓÚ„Ó‚Ó‡ ÓÚ ‰‡‰ÂÌÓ ÌË‚Ó ÏÓÊ ‰‡ ·˙‰Â ËÁÔÓÎÁ‚‡Ì‡ ‰‡ Ò ÔÓ„ÌÓÁË‡ Ó˜‡Í‚‡Ì‡Ú‡ ÒÚÓÈÌÓÒÚ Ì‡ ÓÚ„Ó‚Ó‡ Á‡ ‰Û„ËÚ ÌË‚‡. ìÒÚ‡ÌÓ‚ÂÌÓÚÓ Ë ‚ ‰Û„Ë ÔÛ·ÎË͇ˆËË, ˜Â ÔËÍÓ‚ËÚ ·ÚÂ̈ËË Ì Ò ÔÓÏÂÌflÚ Á̇˜ËÏÓ ÓÚ ‚˙Á‡ÒÚÚ‡ ‚ „‡ÌˈËÚ ÓÚ 10 ‰Ó 60 „. Ë ÓÚ ÔÓ· (7, 10, 14-16, 19, 20, 23) ÒÂ


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÔÓÚ‚˙ʉ‡‚‡ Ë ÓÚ Ì‡¯ËÚ ‰‡ÌÌË. ÄÏÔÎËÚÛ‰ËÚ ̇ ÍÓÏÔÓÌÂÌÚËÚ ‚ ̇¯ÂÚÓ ËÁÒΉ‚‡Ì Ìflχı‡ ÌÓχÎÌÓ É‡ÛÒ-ã‡Ô·ÒÓ‚Ó ‡ÁÔ‰ÂÎÂÌËÂ Ë Ëχı‡ ¯ËÓÍË ‚‡ˇˆËË ‚Íβ˜ËÚÂÎÌÓ ÒÚ‡Ì‡/ÒÚ‡Ì‡. éÒ‚ÂÌ ÚÓ‚‡ Ú ͇ÍÚÓ Ë ÏÓÙÓÎÓ„ËflÚ‡ ̇ ‚˙ÎÌÓ‚ËÚ ÙÓÏË Ò‡ ÚÛ‰ÌÓ ÓÔ‰ÂÎflÂÏË ÍÓ΢ÂÒÚ‚ÂÌÓ, ÍÓÂÚÓ Á̇˜ËÚÂÎÌÓ Ó„‡Ì˘‡‚‡ ÍÎËÌ˘ÌÓÚÓ ËÏ ÔËÎÓÊÂÌËÂ. Ç˙ÔÓÒ‡ Á‡ ÍÎËÌ˘̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ ‡ÏÔÎËÚÛ‰ËÚ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ìflχ ‰ÌÓÁ̇˜ÂÌ ÓÚ„Ó‚Ó Ë ÒÚË„‡ ‰Ó ‰‚ÂÚ Í‡ÈÌÓÒÚË. Tsuji Ë Ò˙Ú. (1984) Ò˜ËÚ‡Ú „‡ÌˈËÚ ̇ ÌÓχÎÌÓÒÚ Ì‡ ‡ÏÔÎËÚÛ‰ËÚ Á‡ ÚÓÎÍÓ‚‡ ¯ËÓÍË, ˜Â Ò‡ÏÓ ÎËÔÒ‡Ú‡ ̇ ‰‡‰Â̇ ‚˙Î̇  ÔËÂχ̇ Á‡ ‡·ÌÓÏ̇. ÑÛ„Ë ‡‚ÚÓË Ò˙˘Ó ÔËÂχÚ, ˜Â ‡ÏÔÎËÚÛ‰ËÚ ҇ ÌÂ̇‰ÂʉÌË Á‡ ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ‡·ÌÓÏÌÓÒÚË (5, 9, 14, 19). Scarff Ë Ò˙Ú. (1981) ÔËÂÏ‡Ú ‡ÁÎË͇ڇ ÓÚ 75% Á‡ ÒË„ÌËÙË͇ÌÚ̇ ÔË ÔÓÎÓÊÂÌËÂ, ˜Â ÔÓÚÂ̈ˇΠÓÚ 0.8 ?V  ÔÓÎÛ˜ÂÌ ÓÚ ‡ÒËÏÔÚÓχÚ˘̇ڇ ÒÚ‡Ì‡. ÅÓίËÌÒÚ‚ÓÚÓ ÓÚ ‡‚ÚÓËÚ ÔËÂÏ‡Ú Ò˙ÓÚÌÓ¯ÂÌËÂÚÓ ÒÚ‡Ì‡/ÒÚ‡Ì‡ 4:1 Á‡ ‡·ÌÓÏÌÓ (7, 11, 12, 18, 20). àÁıÓʉ‡ÈÍË ÓÚ Ì‡¯ËÚ ÂÁÛÎÚ‡ÚË Ò˜ËÚ‡ÏÂ, ˜Â ÔË ÎËÔÒ‡Ú‡ ̇ ‡·ÌÓÏÌÓÒÚ ‚ ·ÚÂ̈ËflÚ‡ ̇ ‰‡‰ÂÌ ÍÓÏÔÓÌÂÌÚ, ‡·ÌÓÏÌÓÒÚÚ‡ ‚ ‡ÏÔÎËÚÛ‰‡Ú‡ ÏÛ Úfl·‚‡ ‰‡ Ò ËÌÚÂÔÂÚË‡ Ò ‚ÌËχÌËÂ. èÓÎÛ˜ÂÌËÚ ÂÁÛÎÚ‡ÚË, ÒÚ‡ÚËÒ˘ÂÒ͇ڇ ËÏ Ó·‡·ÓÚ͇ Ë ‰‡ÌÌËÚ ÓÚ ÎËÚÂ‡ÚÛ‡Ú‡ ÌË ‰‡‰Óı‡ ‚˙ÁÏÓÊÌÓÒÚ ‰‡ ÓÔ‰ÂÎËÏ ÍÓË Ì‡ıÓ‰ÍË Úfl·‚‡ ‰‡ Ò ҘËÚ‡Ú Á‡ ‡·ÌÓÏÌË Ë Ú ҇: 1/ ÎËÔÒ‚‡˘ ÔÓÚÂ̈ˇÎ; 2/ ‡·ÒÓβÚ̇ڇ ·ÚÂ̈Ëfl Ô‚˯‡‚‡˘‡ ÒÚÓÈÌÓÒÚÚ‡ ÔÓÎÛ˜Â̇ ÒΉ ÔË·„‡Ì ̇ ÙÓÏÛ·ڇ Á‡ „ÂÒËfl + 2 ëÑ, ÍÓÂÚÓ ‰‡‚‡ 95% „‡Ìˈ‡ ̇ ‰ÓÒÚÓ‚ÂÌÓÒÚ; 3/ ÒÚ‡Ì‡/ÒÚ‡Ì‡ ‡ÁÎËÍËÚ ‰‡ ̇‰‚˯‡‚‡Ú 1.5 SD ̇ ‡·ÒÓβÚ̇ڇ ·ÚÂ̈Ëfl; 4/ ÒÚÓÈÌÓÒÚË Ì‡ ·ÚÂ̈ËËÚ ÓÚ ‰‡‰ÂÌ ‰ÂχÚÓÏ ËÁÎËÁ‡˘Ë ËÁ‚˙Ì ‡ÏÍËÚ ̇ ÒÚÓÈÌÓÒÚËÚ ̇ ÔÓ‰ÎÂʇ˘Ëfl Ë Ì‡‰ÎÂʇ˘Ëfl ÏÛ ‰ÂχÚÓÏ (ËÁÍβ˜ÂÌË ҇ ‰ÂχÚÓÏËÚ ã5 Ë ÖÒ1); 5/ ‡ÏÔÎËÚÛ‰ÌËÚ ‡ÁÎËÍË ÒÚ‡Ì‡/ÒÚ‡Ì‡ ËÁ‡ÁÂÌË Í‡ÚÓ ÓÚÌÓ¯ÂÌË > ÓÚ 4:1. èÓÒΉÌËflÚ ÍËÚÂËÈ Úfl·‚‡ ‰‡ Ò ËÌÚÂÔÂÚË‡ ‚ÌËχÚÂÎÌÓ Ë ·Ë ·ËÎÓ ÛÏÂÒÚÌÓ ‰‡ Ò Ò‡‚ÌË ÓÚ„Ó‚Ó‡ ÓÚ ‰Û„ ‰ÂχÚÓÏ ËÎË ÔÂËÙÂÂÌ ÌÂ‚ ÓÚ Ò˙˘Ëfl Í‡ÈÌËÍ. Ä·ÌÓÏÌÓÒÚËÚ Ì ̇·„‡Ú ͇Ú„Ó˘ÌÓ Á‡Íβ˜ÂÌË Á‡ ÒÔˆËÙ˘ÂÌ ÚËÔ Ë ÏflÒÚÓ Ì‡ Ô‡ÚÓÎÓ„Ëfl Ë Úfl·‚‡ ‰‡ Ò ËÌÚÂÔÂÚË‡Ú ‚ Ò‚ÂÚÎË̇ڇ ̇ ‰Û„ËÚ ÍÎËÌ˘ÌË Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ÏÂÚÓ‰Ë.

electrophysiological tests in patients with sciatica. Acta Neurol Scand 2000; 101: 249-254. 4. American Electroencephalographic Society: Guideline nine: guidelines on evoked potentials. J Clin Neurophysiol. 1994 Jan;11(1):40-73. 5. Aminoff, M.J.: Segmentally specific somatosensory evoked potentials. Neurol Clin 1991;9:663. 6. Chabot, R.J., Donald, G.D., Glastein, C.D., Merkin, H., Hession, E.F.: The diagnostic utility of dermatomal evoked potentials. J Spinal Disord, 1996; 8(4):296-303. 7. Date, E.S., Ortega, H.R., Hail, K.: Somatosensory evoked responses to dermatomal stimulation in cervical spinal cord injuries and normal subjects. Clin. Electroenceph., 1988, 19: 144-154. 8. Desmedt, J.E., Cheron, G.: Prevertebral (esophageal) recording of subcortical somatosensory evoked potentials in man: The spinal PI3 component and the dual nature of the spinal generators. Electroencephalogr. Clin. Neurophysiol., 1981; 52:257-275. 9. Eisen, A., Hoirch, M., Moll, A.: Evaluation of radiculopathies by segmental stimulation and somatosensory evoked potentials. Can. J. Neurol. Sci. 1983; 10: 178-182. 10. Ganes, T.: A study of peripheral, cervical and cortical evoked potentials and afferent conduction times in the somatosensory pathway. Electroencephalogr Clin Neurophysiol., 1980; 49: 446-451. 11. Insola, A., Valeriani, M., Santilli, V., Bedini, L., Le_Pera, D., Barba, C., Denaro, F., Tonali, P.: Somatosensory evoked potentials after multisegmental lower limb stimulation in focal lesions of the lumbosacral spinal cord. J Neurol Neurosurg Psychiatry 2000; 69 (1): 91-5. 12. Katifi, H.A., Sedgwick, E.M.: Somatosensory evoked potentials from posterior tibial nerve and lumbosacral dermatomes. Electroencephalogr Clin Neurophysiol 1986; 65:249-259. 13. Kimura, J., Mitsudome, A., Beck, D. O., Yamada, T., and Dickins, Q. S.: Field distribution of antidromically activated digital nerve potentials: Model for far-field recording. Neurology, 1983; 33:1164-1169. 14. Lastimosa, A.C.B., Bass, N.H., Stanback, K.: Lumbar spinal cord and early cortical potentials after tibial nerve stimulation: effect of stature on normative data. Electroencephalogr. Clin. Neurophysiol., 1982, 54: 499-507. 15. Lueders, H., Lesser, R., Hahn J, Dinner DS, Klem G. Cortical somatosensory evoked potentials in response to hand stimulation. J Neurosurg 1983a;58:885-94. 16. Ozaki, I., Takada, H., Shimamura, H., Baba, M.: Central conduction in somatosensory evoked potentials: comparison of ulnar and median data and evaluation of onset versus peak methods. Neurology, 1996; 47: 1299-1304. 17. Restuccia, D., Di Lazzaro, V., Valeriani, M., Conti, G., Tonali, P., Mauguierre, F.: Origin and distribution of PI3 and P14 far-field poten tials after median nerve stimulation. Scalp, nasopharyngeal and neck recording in healthy subjects and in patients with cervical and cervico-medullary lesions. Electroencephalogr Clin Neuro physiol 1995; 96:371-84. 18. Scarff, T.B., Dallman, D.E., Toleikis, J.R., Bunch, W.H.: Dermatomal somatosensory evoked potentials in the diagnosis of lumbar root entrapment. Surgical Forum 1981;32:489-491. 19. Seyal, M., Palma, G. A., Sandhu, L. S., Mack, Y. P., and Hannam, J. M. (1988): Spinal somatosensory evoked potentials following segmental sensory stimulation. A direct measure of dorsal foot function. Electroencephalogr. Clin. Neurophysiol., 69: 390-393. 20. Slimp, J.C., Rubner, D.E., Snowden, M.D., Stolov, W.C.: Dermatomal somatosensory evoked potentials: cervical, thoracic, and lumbosacral. Electroencephalogr Clin Neurophysiol 1992; 84:55-70. 21. Synek, V.M.: Somatosensory evoked potentials after stimulation of digital nerves in upper limbs: normative data. Electroenceph. Clin. Neurophysiol., 1986; 65: 460-463. 22. Toleikis, J.R., Slan, T.B., Schrader, S., Koht, A.: Scalp distribution of dermatomal evoked potentials. In: J. Schramm and S.J. Jones (Eds.), Spinal Cord Monitoring. Springer, Berlin, 1985; 59-63. 23. Tsuji, S., Luders, H., Lesser, R.P., Dinner, D.S., Klem, G.: Subcortical and cortical somatosensory potentials evoked by posterior tibial nerve stimulation: normative values. Electroencephalogr. Clin. Neurophysiol., 1984; 59: 214-228.

ãàíÖêÄíìêÄ 1. åË·ÌÓ‚, à.: ëÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË. Ç: äÎËÌ˘̇ ÂÎÂÍÚÓÏËÓ„‡ÙËfl. Å.à¯ÔÂÍÓ‚‡, à. åË·ÌÓ‚, ìÌËÒÓÌ ÄÚ ÖééÑ, ëÓÙËfl, 2003, 515-525. 2. ꇯ‚‡, å.: ëÓχÚÓÒÂÌÁÓÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË ‚ ÒÔ¯̇ڇ Ì‚ÓÎÓ„Ëfl. Ç: Ñˇ„ÌÓÒÚË͇ Ë Ôӂ‰ÂÌË ÔË ÒÔ¯ÌËÚ Ò˙ÒÚÓflÌËfl ‚ Ì‚ÓÎÓ„ËflÚ‡. Å. ÉÂ‡ÒËÏÓ‚, è. ëÚ‡ÏÂÌÓ‚‡, ‰. ìà “ë‚. äÎËÏÂÌÚ éıˉÒÍË”, ëÓÙËfl, 1997, 160 – 167. 3. Albeck, M.J., Taher, G., Lauritzen, M., Trojaborg, W.: Diagnostic value of

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˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

172

éË„Ë̇ÎÌË ÒÚ‡ÚËË ëöïêÄçÖçàÖ çÄ ëãìïÄ èêà éèÖêÄíàÇçéíé ãÖóÖçàÖ çÄ ÉéãÖåà ÇÖëíàÅìãÄêçà òÇÄçéåà (çÖÇêàçéåà çÄ N. VESTIBULOCOCHLEARIS) Ç. ÉÂ„‡ÌÓ‚ , Ç. ÅÛÒ‡ÒÍË, ä. êÓχÌÒÍË, ï. ê‡Ì„ÂÎÓ‚, ã. çۘ‚ , à. àÎË‚ ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓıËÛ„Ëfl

SUMMARY PRESERVATION OF HEARING IN SURGERY OF LARGE VESTIBULAR SCHWANNOMAS (ACOUSTIC NEUROMAS) V. Gerganov, V. Bussarsky , K. Romansky, C. Ranguelov, Noutchev LT, Iliev IN Aims: to analyze hearing function in patients after surgery of vestibular schwannomas. Patients and methods: retrospective analysis of the patients operated consecutively at the Department of Neurosurgery, University Hospital Alexandrovska - Sofia, during the period 1991- 2003. Results: during that period 168 patients have been operated. In 94% of the cases the tumors were larger than 3 cm. The retrosigmoid suboccipital approach was used in 94%. Hearing was preserved in 4 patients (8,3% of the patients with functional hearing preoperatively). Discussion: main predictors of hearing preservation are tumor size and preoperative hearing level. The prevalence of large schwannomas and of patients with severe hearing loss in our series determine the low rate of its preservation. Nevertheless, the goal of surgery in each patient should be anatomic and functional preservation of the cochlear nerve. Key words: vestibular schwannoma, surgical treatment, hearing, quality of life, retrosigmoid approach. êÖáûåÖ ñÂÎ: ‰‡ Ò ÔÓÛ˜Ë Ò˙ÒÚÓflÌËÂÚÓ Ì‡ ÒÎÛı‡ ÔË ·ÓÎÌËÚÂ Ò ‚ÂÒÚË·Û·ÌË ¯‚‡ÌÓÏË, ÓÔÂË‡ÌË ˜ÂÁ ÂÚÓÒË„ÏÓˉÂÌ ‰ÓÒÚ˙Ô. å‡ÚÂˇÎË Ë ÏÂÚÓ‰Ë: ÂÚÓÒÔÂÍÚË‚ÂÌ ‡Ì‡ÎËÁ ̇ ·ÓÎÌËÚÂ, ÓÔÂË‡ÌË ÔÓÒΉӂ‡ÚÂÎÌÓ ‚ äÎËÌË͇ڇ ÔÓ ç‚ÓıËÛ„Ëfl ̇ ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇” Á‡ ÔÂËÓ‰‡ 19912003 „. êÂÁÛÎÚ‡ÚË: é·˘ËflÚ ·ÓÈ Ì‡ ÓÔÂË‡ÌËÚ  168, ͇ÚÓ ‚ 94% ¯‚‡ÌÓÏËÚ ҇ ·ËÎË Ò ‡ÁÏÂ ̇‰ 3 ÒÏ. êÂÚÓÒË„ÏÓˉÂÌ ÒÛ·Ó͈ËÔËÚ‡ÎÂÌ ‰ÓÒÚ˙Ô Â Ô‰ÔÓ˜ÂÚÂÌ ÔË 94%. ë˙ı‡ÌÂÌ ÒÎÛı ÒΉ ÓÔÂ‡ˆËflÚ‡  ÛÒÚ‡ÌÓ‚ÂÌ ÔË 4 ·ÓÎÌË (8,3% ÓÚ ·ÓÎÌËÚÂ Ò ÌÓχÎÂÌ ËÎË Ò˙ı‡ÌÂÌ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı Ô‰ÓÔÂ‡ÚË‚ÌÓ). ÑËÒÍÛÒËfl: éÒÌÓ‚ÌËÚ هÍÚÓË, ÓÔ‰ÂÎfl˘Ë ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛı‡ Ò‡ ‡ÁÏÂ ̇ ¯‚‡ÌÓχ Ë ÌË‚Ó Ì‡ ÒÎÛı‡ Ô‰ÓÔÂ‡ÚË‚ÌÓ. ÑÓÏËÌË‡ÌÂÚÓ Ì‡ ¯‚‡ÌÓÏË Ò „ÓÎÂÏË ‡ÁÏÂË ‚ ÒÂËflÚ‡ Ë ÎËÔÒ‡Ú‡ ̇ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı Ô‰ÓÔÂ‡ÚË‚ÌÓ, Ô‰ÓÔ‰ÂÎflÚ ÌËÒ͇ڇ ˜ÂÒÚÓÚ‡ ̇ Ò˙ı‡Ìfl‚‡ÌÂÚÓ ÏÛ. Ç˙ÔÂÍË ÚÓ‚‡, ˆÂÎÚ‡ ÔË ‚ÒÂÍË ·ÓÎÂÌ Úfl·‚‡ ‰‡ ·˙‰Â ‡Ì‡ÚÓÏ˘ÌÓÚÓ Ë ÙÛÌ͈ËÓ̇ÎÌÓ Ò˙ı‡Ìfl‚‡Ì ̇ n. Òochlearis. äβ˜Ó‚Ë ‰ÛÏË: ‚ÂÒÚË·Û·ÂÌ ¯‚‡ÌÓÏ, ıËÛ„˘ÌÓ Î˜ÂÌËÂ, ÒÎÛı, ͇˜ÂÒÚ‚Ó Ì‡ ÊË‚ÓÚ‡, ÂÚÓÒË„ÏÓˉÂÌ ‰ÓÒÚ˙Ô è˙‚ËflÚ ÒÎÛ˜‡È ̇ Ò˙ı‡ÌÂÌ ÒÎÛı ÒΉ ÓÔÂ‡ÚË‚ÌÓ ÓÚÒÚ‡Ìfl‚‡Ì ̇ ‚ÂÒÚË·Û·ÂÌ ¯‚‡ÌÓÏ (Ì‚ËÌÓÏ Ì‡ n. vestibulocochlearis)  ÓÔËÒ‡Ì ÔÂÁ 1954 „. ÓÚ Elliot Ë McKissock (10). èÂÁ ÔÓÒΉ‚‡˘ËÚ ‰ÂÒÂÚËÎÂÚËfl Á‡Ô‡Á‚‡ÌÂÚÓ Ì‡ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı Ë ÌÓχÎ̇ ÙÛÌ͈Ëfl ̇ ÎË-

ˆÂ‚Ëfl ÌÂ‚ Ò Ô‚˙̇ı‡ ‚ ÓÒÌӂ̇ ˆÂÎ ÔË Î˜ÂÌËÂÚÓ Ì‡ ÚÂÁË ÚÛÏÓË. Ç ‡Á΢ÌË ÔÛ·ÎËÍÛ‚‡ÌË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ ÒÂËË Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛı‡ Ò ÔÓÒÚË„‡ ‚ 22- 87% (5, 6, 12, 17,31). éÒÌÓ‚ÌËÚ هÍÚÓËÚÂ, ÓÔ‰ÂÎfl˘Ë ‚ÂÓflÚÌÓÒÚÚ‡ ÓÚ ÔÓÒÚË„‡ÌÂÚÓ Ì‡ Ú‡ÁË ˆÂÎ, Ò‡ ‡ÁÏÂ‡ ̇ Çò Ë ÌË‚ÓÚÓ Ì‡ ÒÎÛı‡ Ô‰ÓÔÂ‡ÚË‚ÌÓ. ëÔÓ‰ ÌflÍÓË ıËÛÁË Ô‰ÂÎÌËfl ‡ÁÏÂ  2 ÒÏ. ÑÛ„Ë ‡‚ÚÓË ÔÓÒÓ˜‚‡Ú, ˜Â ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‡ÁÏÂ‡ ̇ ÚÛÏÓ‡, ÔË ‚Ò˘ÍË ·ÓÎÌË Ò ËÁÏÂËÏ ÒÎÛı Ô‰ÓÔÂ‡ÚË‚ÌÓ Úfl·‚‡ ‰‡ Ò ̇Ô‡‚Ë ÓÔËÚ Á‡ Ò˙ı‡Ìfl‚‡ÌÂÚÓ ÏÛ (11, 15, 22). Ç ÓÔËÚ ‰‡ ÓÚ„Ó‚ÓËÏ Ì‡ ‚˙ÔÓÒ‡ ‚˙ÁÏÓÊÌÓ ÎË Â Á‡Ô‡Á‚‡Ì ̇ ÒÎÛı‡ ÔË „ÓÎÂÏË Çò Ë Ì ‚Ó‰Ë ÎË ÚÓÁË ÓÔËÚ ‰Ó ÔÓ- ‚ËÒÓÍ ËÒÍ ÓÚ ÏÓ·Ë‰ËÚÂÚ ËÎË ÏÓÚ‡ÎËÚÂÚ, ÌË Ôӂ‰ÓıÏ ̇ÒÚÓfl˘ÂÚÓ ÔÓÛ˜‚‡ÌÂ. äéçíàçÉÖçí à åÖíéÑà Ç ÂÚÓÒÔÂÍÚË‚ÌÓÚÓ ËÁÒΉ‚‡Ì ҇ ‚Íβ˜ÂÌË ·ÓÎÌËÚÂ Ò Çò ÓÔÂË‡ÌË ÔÓÒΉӂ‡ÚÂÎÌÓ ‚ äÎËÌË͇ڇ ÔÓ ç‚ÓıËÛ„Ëfl ̇ ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇” ÔÂÁ ÔÂËÓ‰‡ 1991- 2003 „. èÓÛ˜ÂÌË Ò‡ ÍÎËÌ˘ÌËÚ ËÒÚÓËË, ÓÔÂ‡ÚË‚ÌËÚ ÔÓÚÓÍÓÎË, ÂÁÛÎÚ‡ÚËÚ ÓÚ Ó·‡ÁÌËÚ ËÁÒΉ‚‡ÌËfl Ë ÍÓÌÚÓÎÌËÚ Ô„ΉË. îÛÌ͈ËÓÌË‡ÌÂÚÓ Ì‡ n. vestibulocochlearis ·Â ÓÔ‰ÂÎflÌÓ Ò ‰ÂÚ‡ÈÎÌÓ Ô‰ÓÔÂ‡ÚË‚ÌÓ Ë ÔÓÒÚÓÔÂ‡ÚË‚ÌÓ Ì‚ÓÓÚÓÎӄ˘ÌÓ Ë ‡Û‰ËÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ. 䇘ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ‡ ̇ ÓÔÂË‡ÌËÚ ·ÓÎÌË ·Â ÓÔ‰ÂÎÂÌÓ Ò ÔÓÏÓ˘Ú‡ ̇ ‡ÌÍÂÚ‡Ú‡ ̇ Schwartz MS et al., 1998, ÍÓflÚÓ ‚Íβ˜‚‡ ͇ÚÓ Ò‡ÏÓÒÚÓflÚÂÎÂÌ Ô‡‡ÏÂÚ˙ Ë Ò˙ÒÚÓflÌËÂÚÓ Ì‡ ÒÎÛı‡ ÒΉ ÓÔÂ‡ˆËflÚ‡ (24). êÖáìãíÄíà é·˘ËflÚ ·ÓÈ Ì‡ ÓÔÂË‡ÌËÚ  168, ͇ÚÓ ÔË 94% ¯‚‡ÌÓÏËÚ ҇ Ò ‡ÁÏÂ ̇‰ 3 ÒÏ. ë˙ÓÚÌÓ¯ÂÌËÂÚÓ ÊÂÌË:

îË„Û‡ 1Ä. èÓÒÚÓÔÂ‡Ú˂̇ ‡Û‰ËÓ„‡Ï‡ ÔË ·ÓÎÌËfl ëâÑ. éÒÚ‡Ú˙˜ÌËflÚ ÒÎÛı ÓÚ ÓÔÂË‡Ì‡Ú‡ ÒÚ‡Ì‡ ÏÓÊ ‰‡ Ò ‰ÂÙËÌË‡ ͇ÚÓ “ÙÛÌ͈ËÓ̇ÎÂÌ”- Ô‡„˙Ú Ì‡ ‚˙ÁÔËÂχÌ  >50 dB Ò‡ÏÓ ‚˙‚ ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËfl ‰Ë‡Ô‡ÁÓÌ.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„Û‡ 1Ç: èÓÒÚÓÔÂ‡Ú˂̇ ‡Û‰ËÓ„‡Ï‡ ÔË ·ÓÎÌËfl ûéä. ëÎÛı˙Ú Â Ò˙ı‡ÌÂÌ, ÌÓ Ì  ÙÛÌ͈ËÓ̇ÎÂÌ. (Ó-----Ó – ÍÓÒÚ̇ ÔÓ‚Ó‰ËÏÓÒÚ; ı-----ı – ‚˙Á‰Û¯Ì‡ ÔÓ‚Ó‰ËÏÓÒÚ). Ï˙Ê  1,5 : 1. ë‰̇ڇ ‚˙Á‡ÒÚ Â 49,4„. ë‰ÌËflÚ ‡ÁÏÂ ̇ ¯‚‡ÌÓÏËÚ  3,8 ÒÏ, ‡ ‰Ë‡Ô‡ÁÓ̇- ÓÚ 1,5 ‰Ó 6 ÒÏ. ë ‡Ì‡ÍÛÁËÒ Ô‰ÓÔÂ‡ÚË‚ÌÓ Ò‡ ·ËÎË 76 (45%). çÓχÎÂÌ Â ·ËÎ ÒÎÛı‡ ÔË 10 ·ÓÎÌË (6%). Ö‰‚‡ 38 ÓÚ ·ÓÎÌËÚÂ Ò ıËÔ‡ÍÛÁËÒ Ò‡ ËχÎË Ò˙ı‡ÌÂÌ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı. êÂÚÓÒË„ÏÓˉÂÌ ÒÛ·Ó͈ËÔËÚ‡ÎÂÌ ‰ÓÒÚ˙Ô ·Â Ô‰ÔÓ˜ÂÚÂÌ ÔË 94%, ‡ ÒÛ·ÚÂÏÔÓ‡ÎÂÌ Ú‡ÌÒÚÂÌÚÓˇÎÂÌÔË ÓÒڇ̇ÎËÚ ·ÓÎÌË. èË 18 ·ÓÎÌË ·Â ÔËÎÓÊÂ̇ ẨÓÒÍÓÔ- ‡ÒËÒÚË‡Ì‡ ÏËÍÓıËÛ„˘̇ ÚÂıÌË͇.

îË„Û‡ 2Ä. è‰ÓÔÂÚË‚ÌÓ åêí ËÁÒΉ‚‡Ì ̇ ·ÓÎÌËfl àíå. Çò Â Ò Ï‡ÍÒËχÎÂÌ ‰Ë‡ÏÂÚ˙ 4,5 ÒÏ. îË„Û‡ 2Ç. èÓÒÚÓÔÂ‡Ú˂̇ åêí ÔË Ò˙˘Ëfl ·ÓÎÂÌ. éÒ˙˘ÂÒÚ‚Â̇  ÒÛ·ÚÓÚ‡Î̇ (̇‰ 90%) ÂÍÒÚËÔ‡ˆËfl ̇ ÚÛÏÓ‡. ë˙ı‡ÌÂÌ ÒÎÛı ÒΉ ÓÔÂ‡ˆËflÚ‡ (îË„. 1) ·Â ÛÒÚ‡ÌÓ‚ÂÌ Ò ÔÓÏÓ˘Ú‡ ̇ ‡Û‰ËÓ„‡Ï‡ ÔË 4-χ ·ÓÎÌË (8,3% ÓÚ ·ÓÎÌËÚÂ Ò ÌÓχÎÂÌ ËÎË Ò˙ı‡ÌÂÌ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı Ô‰ÓÔÂ‡ÚË‚ÌÓ). èË 1 ÓÚ Úflı (åãä) Çò  ÔÓ‰ 2 ÒÏ, ÔË 1 (ëâÑ)  ÔÓ‰ 3ÒÏ, ‡ ÔË 2 (àíå,ûéä)  ̇‰ 3 ÒÏ. ò‚‡ÌÓÏ˙Ú Â ÂÍÒÚËÔË‡Ì ÒÛ·ÚÓÚ‡ÎÌÓ ÔË ‰‚‡Ï‡ (îË„. 2) Ë ÚÓÚ‡ÎÌÓ ÔË ‰Û„ËÚ ‰‚‡Ï‡ ·ÓÎÌË. åÓ·Ë‰ËÚÂÚ˙Ú ÔË ÚÂÁË ·ÓÎÌË Ì ̇‰‚˯‡‚‡ ̇·Î˛‰‡‚‡ÌËfl ‚ ˆfl·ڇ „ÛÔ‡.

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éÅëöÜÑÄçÖ ë˙‚ÂÏÂÌÌÓÚÓ Î˜ÂÌË ̇ Çò  ÙÓÍÛÒË‡ÌÓ ‚˙ıÛ Ò˙ı‡Ìfl‚‡ÌÂÚÓ Ì‡ ÌÓχÎÂÌ ÒÎÛı Ë ÙÛÌ͈Ëfl ̇ Îˈ‚Ëfl ÌÂ‚. òËÓÍËflÚ ‰Ë‡Ô‡ÁÓÌ Ì‡ ÔÛ·ÎËÍÛ‚‡ÌËÚ ÂÁÛÎÚ‡ÚË- Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛı‡ ÓÚ 0 ‰Ó 87%, Ò ‰˙ÎÊË Ì‡ ıÂÚÂÓ„ÂÌ̇ڇ ÒÚÛÍÚÛ‡ ̇ ‡Ì‡ÎËÁË‡ÌËÚ „ÛÔË Ë Ì‡ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‡Á΢ÌË Í·ÒËÙË͇ˆËË Á‡ ÓˆÂÌ͇ ̇ ÒÎÛı‡ (23). ë˙˘ÂÒÚ‚Û‚‡Ú Ò˙˘ÂÒÚ‚ÂÌË ‡Á΢Ëfl ‚ ‰ÂÙËÌˈËflÚ‡ ̇ “ÙÛÌ͈ËÓ̇ÎÂÌ”, “ÔÓÎÂÁÂÌ” ËÎË “ÒӈˇÎÌÓ Á̇˜ËÏ” ÒÎÛı (6, 8, 12, 21, 28). èӂ˜ÂÚÓ ‡‚ÚÓË ÔËÂÏ‡Ú Í‡ÚÓ Ú‡Í˙‚ ÒÎÛı‡ ÔË Ô‡ˆËÂÌÚË Ò Ô‡„ ̇ ‚˙ÁÔËÂχÌ <50 dB Ë „Ó‚Ó̇ ‰ËÒÍËÏË̇ˆËfl >50 %. Ä‚ÚÓËÚÂ Ò „ÓÎflÏ ÓÔËÚ ÔÓÒÚË„‡Ú Ò˙ı‡Ìfl‚‡Ì ̇ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı ‚ 17,6% (31), 27% (30), 29.3% (5), 33% (8), 35% (6), 36,5% (7), 39,5% (22), 39,9% (27), 50% (20), 67,5% (19), 68% (25), 87% (12). íËÚ ÓÒÌÓ‚ÌË ıËÛ„˘ÌË ‰ÓÒÚ˙Ô‡, ËÁÔÓÎÁ‚‡ÌË ÔË Î˜ÂÌËÂÚÓ Ì‡ Çò, Ò‡ Ú‡ÌÒ··ËËÌÚÌËfl, ÂÚÓÒË„ÏÓˉÌËfl Ë ‰ÓÒÚ˙Ô‡ ÔÂÁ Ò‰̇ ˜ÂÂÔ̇ flÏ͇. éÚ Úflı ÔÓÒΉÌËÚ ‰‚‡ ÔÓÁ‚ÓÎfl‚‡Ú Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛı‡, ÌÓ Â‰ËÌÒÚ‚ÂÌÓ ÂÚÓÒË„ÏÓˉÌËflÚ Â ÔËÎÓÊËÏ ÔË ÚÛÏÓË Ò „ÓÎflχ ÔÓÔ‡„‡ˆËfl ‚ ÔÓÌÚÓˆÂ·Â·ÌËfl ˙„˙Î (1, 26). ê‰ˈ‡ ‡‚ÚÓË, ͇ÚÓ Brackmann et al., 1992 (2), Slattery et al., 1997 (25), Gormley et al., 1997 (7), Lanmann et al., 1999 (13), Briggs et al., 2000 (3), Yates et al., 2003 (31), Ú‚˙‰flÚ ˜Â ÔË „ÓÎÂÏË Çò Ì  ‚˙ÁÏÓÊÌÓ Ò˙ı‡Ìfl‚‡ÌÂÚÓ Ì‡ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı ëÔÓ‰ Úflı ÔË ‡ÁÏÂ ̇‰ 2 ËÎË 2,5 ÒÏ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ Ò˙ı‡ÌÂÌ ÒÎÛı, ËÁ·Ó˙Ú Ì‡ ÓÔÂ‡ÚË‚ÂÌ ‰ÓÒÚ˙Ô Ì Úfl·‚‡ ‰‡ Ò ‚ÎËfl ÓÚ ÚÓ‚‡ Ò˙Ó·‡ÊÂÌËÂ. èË Ú‡ÍË‚‡ ·ÓÎÌË Ú ÓÚ‰‡‚‡Ú Ô‰ÔÓ˜ËÚ‡ÌË ̇ Ú‡ÌÒ··ËËÌÚÌËfl ‰ÓÒÚ˙Ô. ÄÎÚÂ̇ÚË‚ÌÓÚÓ Òı‚‡˘‡ÌÂ, Í˙Ï ÍÓÂÚÓ Ò Ôˉ˙Ê‡Ú Ôӂ˜ÂÚÓ Ì‚ÓıËÛÁË (9, 12, 19, 15, 17, 22),  ˜Â ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‡ÁÏÂ‡ ̇ ÚÛÏÓ‡, ‡Ì‡ÚÓÏ˘ÌÓÚÓ Ò˙ı‡Ìfl‚‡Ì ̇ n. cochlearis Úfl·‚‡ ‰‡  ˆÂÎ ÔË ‚Òfl͇ ÓÔÂ‡Ú˂̇ ËÌÚÂ‚Â̈Ëfl. ë ӄΉ Ô‰ÓÚ‚‡Úfl‚‡Ì ̇ ˆˉ˂Ë, ÍÓËÚÓ Ì‡È- ˜ÂÒÚÓ ÔÓËÁÎËÁ‡Ú ÓÚ ÂÁˉۇÎ̇ ÚÛÏÓ̇ ˜‡ÒÚ ‚ ӷ·ÒÚÚ‡ ̇ ÙÛ̉ÛÒ‡ ̇ ‚˙Ú¯ÌËfl ÒÎÛıÓ‚ ÔÓıÓ‰, Ò ÔÂÔÓ˙˜‚‡ ¯ËÓÍÓÚÓ ÏÛ ÓÚ‚‡flÌÂ. í‡ÁË Ï‡ÌËÔÛ·ˆËfl  ҂˙Á‡Ì‡ Ò ËÒÍ ÓÚ ‡ÁÛ¯‡‚‡Ì ̇ ÒÚÛÍÚÛËÚ ̇ ‚˙Ú¯ÌÓÚÓ ÛıÓ, „·‚ÌÓ Ì‡ canalis semicircularis posterior Ë crus communis, Ë Ò˙ÓÚ‚ÂÚÌÓ-Á‡„Û·‡ ̇ ÒÎÛı‡. èËÎÓÊÂÌËÂÚÓ Ì‡ ẨÓÒÍÓÔ ÔÓÁ‚ÓÎfl‚‡ ÙÛ̉ÛÒ‡ ‰‡ Ò ËÌÒÔÂÍÚË‡ ÔË ÔÓ- Ó„‡Ì˘ÂÌÓ ËÁÔË΂‡Ì ̇ Á‡‰Ì‡Ú‡ ÒÚÂ̇ ̇ ÔË‡Ïˉ‡Ú‡ (29). ë˙ÓÚÌÓ¯ÂÌËÂÚÓ Ì‡ ˜ÂÂÔÌÓ- ÏÓÁ˙˜ÌËÚ ÌÂ‚Ë ÒÔflÏÓ ÚÛÏÓ‡ ÏÓÊ ‰‡ Ò ӈÂÌË Ì‡ ÔÓ- ‡ÌÂÌ ÂÚ‡Ô. 燯ËflÚ ÓÔËÚ ÓÚ ÔËÎÓÊÂÌË ̇ ẨÓÒÍÓÔ- ‡ÒËÒÚË‡Ì‡ ÏËÍÓıËÛ„˘̇ ÚÂıÌË͇ ÔÓÚ‚˙ʉ‡‚‡ ÚÂÁË Ô‰ËÏÒÚ‚‡ ̇ ÏÂÚÓ‰Ë͇ڇ. î‡ÍÚÓËÚÂ, ÓÔ‰ÂÎfl˘Ë ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛı‡, Ò‡ ‡ÁÏÂ ̇ ¯‚‡ÌÓχ, ÌË‚Ó Ì‡ ÒÎÛı‡ Ô‰ÓÔÂ‡ÚË‚ÌÓ, ÓÔËÚ Ì‡ ıËÛ„‡, Ò˙ÓÚÌÓ¯ÂÌË ÏÂÊ‰Û ËÌÚ‡- Ë ÂÍÒÚ‡Í‡Ì‡ÎËÍÛ·̇ڇ ÚÛÏÓ̇ ˜‡ÒÚ, ÒÚÂÔÂÌ Ì‡ ‰Ë·ڇˆËfl ̇ ‚˙Ú¯ÌËfl ÒÎÛıÓ‚ ÔÓıÓ‰, ÍÓÌÒËÒÚÂ̈Ëfl Ë ‚‡ÒÍÛ·ËÁ‡ˆËfl ̇ ÚÛÏÓ‡. èÂӷ·‰‡‚‡ ÏÌÂÌËÂÚÓ, ˜Â ÓÒÌÓ‚ÌËÚ هÍÚÓË Ò‡ Ô‰ÓÔÂ‡ÚË‚ÌÓÚÓ ÌË‚Ó Ì‡ ÒÎÛı‡ Ë ‡ÁÏÂ‡ ̇ Çò. Ç ÒÂËflÚ‡ ̇ Gormley et al., 1997 (7), Ò‡ÏÓ ÔË 11 % ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ÚÛÏÓ ̇‰ 4 ÒÏ Â Ò˙ı‡ÌÂÌ ÒÎÛı‡. èË ÚÛÏÓË ‚ 4-Ë ÒÚ‡‰ËË Matthies et Samii, 2002 (15), ÔÓÒÓ˜‚‡Ú Ò˙ı‡ÌÂÌ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı ‰‚‡ ÔË 20%. Ä̇ÎËÁ˙Ú Ì‡ ·ÓÎÌËÚ ÓÚ ÒÂËflÚ‡ ÔÓ͇Á‡, ˜Â Ò‰ÌÓÚÓ «Á‡Í˙ÒÌÂÌË», Ú.Â. ÔÂËÓ‰˙Ú ÓÚ ÍÎËÌ˘̇ڇ ËÁfl‚‡ ̇ ÚÛÏÓ‡ ‰Ó ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡,  51 ÏÂÒˆ‡, Ò ‰Ë‡Ô‡ÁÓÌ ÓÚ 3 ‰. ‰Ó 20 „., ÍÓÂÚÓ Ó·flÒÌfl‚‡ ‰ÓÏËÌË‡ÌÂÚÓ Ì‡ „ÓÎÂÏË ¯‚‡ÌÓÏË. ê‡Ì̇ڇ ‰Ë‡„ÌÓÁ‡  Ô‰ÔÓÒÚ‡‚͇ Á‡ ÔÓ‰Ó·ÂÌË ̇ ÓÔÂ‡ÚË‚ÌËÚ ÂÁÛÎÚ‡ÚË.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

è‰ÓÔÂ‡ÚË‚ÌÓÚÓ ÌË‚Ó Ì‡ ÒÎÛı‡  ‰Û„Ëfl ÓÒÌÓ‚ÂÌ Ù‡ÍÚÓ. Samii et al., 1997 (21) Ò˙ı‡Ìfl‚‡Ú ÒÎÛı‡ ÔË 39,5% ÓÚ ‚Ò˘ÍË ÓÔÂË‡ÌË. ÄÍÓ Ò ‡Ì‡ÎËÁË‡Ú Ò‡ÏÓ ·ÓÎÌËÚ Ò˙Ò Ò˙ı‡ÌÂÌ ÒÎÛı Ô‰ÓÔÂ‡ÚË‚ÌÓ ‰ÂÎ˙Ú Ì‡ ·ÓÎÌËÚ Ò˙Ò Ò˙ı‡ÌÂÌ ÒÎÛı ̇‡ÒÚ‚‡ ‰Ó 78%. ëÔÓ‰ Wiet et al., 2001 (30), ‰Û„ Ì ÔÓ- χÎÍÓ ‚‡ÊÂÌ Ù‡ÍÚÓ  ÓÔËÚ‡ ̇ ıËÛ„‡. äË‚‡Ú‡ ̇ ̇ۘ‡‚‡Ì ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÓÁË ÔÓ͇Á‡ÚÂÎ Â ÏÂÊ‰Û 20 Ë 200 ÒÎÛ˜‡fl (4). ÅËÓÎӄ˘ÌËÚ ÓÒÓ·ÂÌÓÒÚË Ì‡ ÚÛÏÓ‡ ÓÔ‰ÂÎflÚ ÚÛ‰ÌÓÒÚÚ‡ ̇ ‰ËÒÂ͈ËflÚ‡ ̇ ͇ÔÒÛ·ڇ ÏÛ ÓÚ óåç. èË ÔÂӷ·‰‡‚‡˘Ó ËÌÚ‡Í‡Ì‡ÎËÍÛ·ÌÓ ÔÓ‡ÒÚ‚‡ÌÂ Ë ÎËÔÒ‡ ̇ ‰Ë·ڇˆËfl ̇ ‚˙Ú¯ÌËfl ÒÎÛıÓ‚ ÔÓıÓ‰, ÍÓÏÔÂÒËflÚ‡ ̇ ÍÓı·ÌËfl ÌÂ‚ Ë Ì‡Û¯ÂÌËflÚ‡ ̇ ÒÎÛı‡ ̇ÒÚ˙Ô‚‡Ú ‚ ÔÓ- ‡ÌÂÌ ÂÚ‡Ô. Samii et al., 1997 (21,22), ÔÓÒÓ˜‚‡Ú ÓÎflÚ‡ ̇ ÔÓÒÓ͇ڇ ̇ ‡ÒÚÂÊ Ì‡ ¯‚‡ÌÓχ- Ô‰ËÏÌÓ Í‡Û‰‡ÎÌÓÚÓ ÔÓ‡ÒÚ‚‡Ì ‚Ó‰Ë ‰Ó ÔÓ- ÒËÎÌÓ ÓÔ˙‚‡Ì ̇ ÍÓı·ÌËfl ÌÂ‚ Ë ÂÒÔÂÍÚË‚ÌÓ ‰Ó ÔÓ- ÎÓ¯Ë ÂÁÛÎÚ‡ÚË. Glasscock et al., 1993 (6), Matthies et Samii, 1997 (14) Ë Tonn et al., 2000 (27), ËÁÚ˙Í‚‡Ú ÓÎflÚ‡ ̇ ËÌÚ‡ÓÔÂ‡ÚË‚ÌÓÚÓ ÏÓÌËÚÓË‡Ì ̇ ÒÎÛıÓ‚ËÚ ‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË- ËÌÚ‡ÍÚÌËÚ ÔÓ ÙÓχ ‚˙ÎÌË Ë ÌÓχÎ̇ڇ ËÎË Á‡·‡‚Â̇ ·ÚÂÌÚÌÓÒÚ Ò‡ Ù‡ÍÚÓË Ô‰Ò͇Á‚‡˘Ë ÔÓ‚ËÒÓ͇ ‚ÂÓflÚÌÓÒÚ Á‡ Ò˙ı‡ÌÂÌË ̇ ÒÎÛı‡. ᇄ۷‡Ú‡ ̇ ‚˙Î̇ V ÍÓÂÎË‡ Ò˙Ò Á‡„Û·‡Ú‡ ̇ ÒÎÛı‡, ÌÓ Ì ÏÓÊ ‰‡ Ô‰Ò͇Ê ÚÓ˜ÌÓ ‰‡ÎË ‰ÂÙˈËÚ‡ ˘Â  ÔÂχÌÂÌÚÂÌ ËÎË ÔÂıÓ‰ÂÌ. èÓÏÂÌËÚ ‚ Ú‡ÁË ‚˙Î̇ Ò Ô‰¯ÂÒÚ‚‡Ú ÓÚ ÔÓ- ‡ÌÌË Ô‰ÛÔ‰ËÚÂÎÌË ÔÓÏÂÌË, ͇ÚÓ ËÁ˜ÂÁ‚‡Ì ̇ ‚˙ÎÌËÚ III Ë I. ë˙ı‡Ìfl‚‡ÌÂÚÓ Ì‡ ‚˙Î̇ V, ÔË ÛÒÎÓ‚Ë ˜Â Û‰˙Îʇ‚‡ÌÂÚÓ Ì‡ ·ÚÂÌÚÌÓÚÓ ‚ÂÏ Ì Ô‚˯‡‚‡ 3,00 ms,  ̇‰Âʉ̇ Ë̉Ë͇ˆËfl Á‡ Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛıÓ‚ËÚ ÙÛÌ͈ËË (14). ÑÛ„Ë Ô‰ÔÓÒÚ‡‚ÍË Ò‡ Í‡Ú͇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ ̇Û¯ÂÌËflÚ‡ ̇ ÒÎÛı‡ (<1,5 „.) Ë ‚ÂÒÚË·Û·̇ڇ ‰ËÒÙÛÌ͈Ëfl (22), ͇ÍÚÓ Ë ÔÓËÁıÓ‰ ̇ Çò ÓÚ „ÓÌËfl ‚ÂÒÚË·Û·ÂÌ ÌÂ‚ (19). èË ·ÓÎÌË ‚ ̇Ô‰̇· ‚˙Á‡ÒÚ Ú‡ÁË ‚ÂÓflÚÌÓÒÚ Â ÔÓ- ÌËÒ͇ (18, 30). ëÔÓ‰ Mohr G et al., 2005 (16), ÌÂÔ˙ÎÌÓÚÓ ‡Ì„‡ÊË‡Ì ̇ ‚˙Ú¯ÌËfl ÒÎÛıÓ‚ ÔÓıÓ‰ Ë ‡ÁÏÂ ̇ ÚÛÏÓ‡ ÔÓ‰ 1,5 ÒÏ Ò‡ ‰‚‡ ÌÂÁ‡‚ËÒËÏË ·Î‡„ÓÔËflÚÌË Ù‡ÍÚÓ‡. å‡ÎÍËflÚ ·ÓÈ Ì‡ ·ÓÎÌËÚ Ò˙Ò Ò˙ı‡ÌÂÌ ÒÎÛı ‚ ̇¯‡Ú‡ ÒÂËfl Ì ÌË ÔÓÁ‚ÓÎfl‚‡ ‰‡ ̇Ô‡‚ËÏ ÒÚ‡ÚËÒÚ˘ÂÒÍË Û·Â‰ËÚÂÎÂÌ ‡Ì‡ÎËÁ. ÑÓÏËÌË‡ÌÂÚÓ Ì‡ ¯‚‡ÌÓÏË Ò „ÓÎÂÏË ‡ÁÏÂË (̇‰ 94%) Ë ÎËÔÒ‡Ú‡ ̇ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı Ô‰ÓÔÂ‡ÚË‚ÌÓ (72% ÓÚ ·ÓÎÌËÚÂ) Ô‰ÓÔ‰ÂÎflÚ ÌËÒ͇ڇ ˜ÂÒÚÓÚ‡ ̇ Ò˙ı‡Ìfl‚‡Ì ̇ ÒÎÛı‡ (8,3%). Ä̇ÎËÁ˙Ú Ì‡ ͇˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ‡ ̇ ·ÓÎÌËÚ ÓÚ ÒÂËflÚ‡ ÔÓ͇Á‡, ˜Â ÎËÔÒ‡Ú‡ ̇ ÒÎÛı  Ò‰ ÓÒÌÓ‚ÌËÚ ‰ÂÚÂÏËÌË‡˘Ë Ù‡ÍÚÓË. ÅÓÎÌËÚ Ò˙Ò Ò˙ı‡ÌÂÌ ÒÎÛı Ò‡ Ò ÔÓ- ‚ËÒÓÍÓ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ‡. éÔËÚËÚ Á‡ ÒÎÛıÓÒ˙ı‡Ìfl‚‡˘‡ ıËÛ„Ëfl Ì Ôӂ˯‡‚‡Ú ÏÓ·Ë‰ËÚÂÚ‡. Ç˙ÔÂÍË ˜Â ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ Ò˙ı‡Ìfl‚‡Ì ̇ ‰Ó·Ó ÌË‚Ó Ì‡ ÒÎÛı‡ ÔË „ÓÎÂÏË Çò  ÌËÒ͇, ‰ÓË ÏËÌËχÎÌËfl ÒÎÛı ÏÓÊ ‰‡ Ò Ô‚˙Ì ‚˙‚ ÙÛÌ͈ËÓ̇ÎÂÌ ‚ ·˙‰Â˘Â Ò ÔÓÏÓ˘Ú‡ ̇ ÒÎÛıÓ‚Ë ÔÓÚÂÁË ËÎË ËÏÔ·ÌÚ‡ÌÚË (5). íÓ‚‡ ÌË ‰‡‚‡ ÓÒÌÓ‚‡ÌË ‰‡ ÔËÂÏÂÏ Ò˙ı‡Ìfl‚‡ÌÂÚÓ Ì‡ ÒÎÛı‡ Á‡ ˆÂΠ̇ ‚Òfl͇ ÓÔÂ‡Ú˂̇ ËÌÚÂ‚Â̈Ëfl ̇ Çò, ÔË ÛÒÎÓ‚ËÂ, ˜Â Ô‰ÓÔÂ‡ÚË‚ÌÓ Â Ì‡Îˈ ÙÛÌ͈ËÓ̇ÎÂÌ ÒÎÛı. íÓ‚‡  ÓÚ Ó„ÓÏÌÓ Á̇˜ÂÌËÂ Ë Á‡ Ó·Ó„‡Úfl‚‡Ì ̇ ÓÔËÚ‡ Ë ÛÒ˙‚˙¯ÂÌÒÚ‚‡Ì ̇ Ë̉˂ˉۇÎ̇ڇ ÏËÍÓıËÛ„˘̇ ÚÂıÌË͇.

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ãàíÖêÄíìêÄ 1. ä‡‡„¸ÓÁÓ‚ ã, à‚‡ÌÓ‚ à: ç‚ËÌÓÏË Ì‡ ÒÎÛıÓ‚Ó- ‚ÂÒÚË·Û·ÌËfl ÌÂ‚. ëÓÙËfl, å‰ˈË̇ Ë ÙËÁÍÛÎÚÛ‡, 1988 2. Brackmann DE, Green JD: Translabyrinthine approach for acoustic tumor removal. Otolaryngol Clin North Am 25: 311-329, 1992 3. Briggs RJ, Fabinyi G, Kaye AH: Current management of acoustic neuromas: review of surgical approaches and outcomes. J Clin Neurosc 7: 521- 526, 2000 4. Buchman CA, Chen DA, Flannagan P, Wilberger JE, Maroon JC: The learning curve for acoustic tumor surgery Laryngoscope 106: 1406- 1411, 1996 5. Jaisinghani V, Levine SC, Nussbaum E, Haines S, Lindgren B: Hearing Preservation after Acoustic Neuroma Surgery. Skull Base Surgery 10: 141- 147, 2000 6. Fischer G, Fischer C, Remond J: Hearing preservation in acoustic neuroma surgery. J Neurosurg 76: 910-917, 1992 7. Glasscock ME III, Hays JW, Minor LB, et al. Preservation of hearing in surgery for acoustic neuroma. J Neurosurg 78: 864-870, 1993 8. Gormley WB, Sekhar LN, Wright DC, et al.: Acoustic neuroma: results of current surgical management. Neurosurgery 41:50-60, 1997 9. Hecht CS, Honrubia VF, Wiet RJ, Sims HS: Hearing preservation after acoustic neuroma resection with tumor size used as a clinical prognosticator. Laryngoscope 107: 1122-1126, 1997 10. Irving RM, Jackler KR, Pitts LH: Hearing preservation in patients undergoing vestibular schwannoma surgery: comparison of middle fossa and retrosigmoid approaches. J.Neurosurg 88: 840-845, 1998 11. Jannetta ê: Vestibular neurilemmomas. Clin Neurosurg 44: 529- 549, 1996 12. Lanmann TH, Brackmann DE, Hitselberger WE, et al.: Report of 190 consecutive cases of large acoustic tumors /vestibular schwannoma/ removed via the translabyrinthine approach. J Neurosurg 90 : 617- 623, 1999 13. Matthies C, Samii M: Management of vestibular schwannomas (acoustic neuromas): the value of neurophysiology for evaluation and prediction of auditory function in 420 cases. Neurosurgery 40: 919- 929, 1997 14. Matthies C, Samii M: Vestibular schwannomas and auditory function: options in large T3 and T4 tumors? Neurochirurgie, 48: 461- 470, 2002 15. Mohr G, Sade L, Dufour J-J, Rappaport JM: Preservation of hearing in patients undergoing microsurgery for vestibular schwannoma: degree of meatal filling J Neurosurg 102: 1-5, 2005 16. Ojemann RG: Management of acoustic neuroma (Vestibular schwannoma): Clin Neurosurg 40: 498-533, 1993 17. Koos WT, Diaz Day J, Matula C, et al.: Neurotopographic considerations in the microsurgical treatment of small acoustic neurinomas. J Neurosurg 88: 506512, 1998 18. Oghalai JS, Buxbaum JL, Pitts LH, Jackler RK: The effect of age on acoustic neuroma surgery outcomes Otol Neurotol 24: 473-477, 2003 19. Post KD, Eisenberg MB, Catalano PJ: Hearing preservation in vestibular schwannoma surgery: what factors influence outcome? J Neurosurg 83: 191-196, 1995 20. Rowed DW, Nedzelski JM: Hearing preservation in the removal of intracanalicular acoustic neuromas via the retrosigmoid approach. J Neurosurg 86: 456461, 1997 21. Samii M, Matthies C: Management of 1 000 vestibular schwannomas (acoustic neuromas): surgical management and results with an emphasis on complications and how to avoid them. Neurosurgery 40: 11- 21, 1997 22. Samii M, Matthies C: Management of 1 000 vestibular schwannomas (acoustic neuromas) : hearing function in 1 000 tumor resections. Neurosurgery 40 : 248-262, 1997 23. Sanna M, Khrais T, Russo A, Piccirillo E, Augurio A: Hearing preservation surgery in vestibular schwannoma: the hidden truth. Ann Otol Rhinol Laringol 113: 156- 163, 2004 24. Schwartz MS, Riddle SA: Quality of life following acoustic neuroma surgery. Neurosurg Focus 5 (3): Article 3, 1998 25. Slattery WH, Brackmann DE, Hitselberger WE: Middle fossa approach for hearing preservation with acoustic neuromas Am J Otol 18: 596- 601, 1997 26. Staecker H, Nadol JB, Ojeman R, Ronner S, McKenna MJ: Hearing preservation in acoustic neuroma surgery: middle fossa versus retrosigmoid approach. Am J Otol 21: 399- 404, 2000 27. Tonn JC, Schlake HP, Goldbrunner R, et al.: Acoustic neuroma surgery as an interdisciplinary approach: a neurosurgical series of 508 patients. J Neurol Neurosurg Psychiatry 69: 161-166, 2000 28. Torrens M, Maw R et al.: Facial and acoustic nerve preservation during excision of extracanalicular acoustic neuromas using the suboccipital approach. Br J Neurosurg 8: 655- 665, 1994 29. Wackym PA, King WA, Meyer GA, Poe DS. Endoscopy in neuro-otologic surgery. Otolaryngol Clin North Am: 35: 297-323, 2002 30. Wiet RJ, Mamikoglu B, Odom L, Hoistad DL: Long-term results of the first 500 cases of acoustic neuroma surgery Otolaryngol Head Neck Surg 124: 645651, 2001 31. Yates PD, Jackler RK, Satar B: Is it worthwhile to attempt hearing preservation in larger acoustic neuromas? Otol Neurotol 24: 460- 464, 2003

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ëÓÙËfl 1431, ÛÎ. “ë‚. ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓıËÛ„Ëfl íÂÎ: 9230/ 516, 310 E-mail: venelingerganov@hotmail.com


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

˛ÌË, 2005

175

éÔËÒ‡ÌË ̇ ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë íêÄÇåÄíàóçÄ ÑàëÖäÄñàü çÄ éÅôÄíÄ ëöççÄ ÄêíÖêàü à. ëÚ‡ÈÍÓ‚1 , ï. å‡ÚÎÂ2 äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” , ëÓÙËfl, 2äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, ìÌË‚ÂÒËÚÂÚÒ͇ ÅÓÎÌˈ‡, ÅÂÌ, ò‚ÂȈ‡Ëfl 1

SUMMARY TRAUMATIC COMMON CAROTID ARTERY DISSECTION I. Staikov, H. Mattle Dissections of extracranial carotid arteries are developed usually in the internal carotid artery. In contrast, dissections of the common carotid artery are rare and almost always are associated with aortic dissection. Isolated dissection of the common carotid artery, without involving the aorta is especially rare. The classic clinical presentations of the common carotid artery dissection include cerebral or retinal ischemic symptoms, headache, monocular blindness and neck pain. Doppler color-flow imaging with duplex scanning, magnetic resonance imaging, magnetic resonance angiography or computed tomographic angiography are reliable, noninvasive methods for the diagnosis and follow-up of dissection of the common carotid artery. The therapeutic management involves surgical intervention or conservative therapy such as antiplatelet or anticoagulative treatment. The prognosis of common carotid artery dissection is relatively good. We report a rare case of isolated traumatic common carotid artery dissection. A 21-year-old healthy man was hospitalized after car accident with multiple bone fractures. Some days after the admission, the patient revealed amaurosis fugax in his right eye. Doppler color-flow imaging and duplex scanning showed a moderate stenosis in the proximal part of the right common carotid artery, intramural hematoma and double lumen. This results revealed a traumatic common carotid artery dissection and anticoagulative treatment was begun. Eight months later, the patient had no neurological deficits, however the follow-up magnetic resonance angiography still showed a false lumen in the initial part of the right common carotid artery. Because of the rarity of the isolated common carotid artery dissection, we report this case and discuss the diagnostic and therapeutic management of the disease. Key Words: common carotid artery, internal carotid artery, dissection, duplex ultrasonography. êÖáûåÖ ÖÍÒÚ‡Í‡ÌˇÎÌËÚ ͇ÓÚˉÌË ‰ËÒÂ͇ˆËË Ì‡ÒÚ˙Ô‚‡Ú Ó·ËÍÌÓ‚ÂÌÓ ‚ ӷ·ÒÚÚ‡ ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl. ÑËÒÂ͇ˆËËÚ ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl Ò‡ Á̇˜ËÚÂÎÌÓ ÔÓ-‰ÍË Ë ÔÓ˜ÚË ‚Ë̇„Ë Òe ‡ÒÓˆËË‡Ú Ò ‰ËÒÂ͇ˆËfl ̇ ‡ÓÚ‡Ú‡. àÁÓÎË‡ÌËÚ ‰ËÒÂ͇ˆËË Ì‡ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl, ·ÂÁ Á‡Òfl„‡Ì ̇ ‡ÓÚ‡Ú‡ Ò Ò¢‡Ú ÓÒÓ·ÂÌÓ fl‰ÍÓ. ä·Ò˘ÂÒ͇ڇ ÍÎËÌ˘̇ ı‡‡ÍÚÂËÒÚË͇ ̇ ‰ËÒÂ͇ˆËflÚ‡ ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl ‚Íβ˜‚‡ ÂÚË̇ÎÌË ËÎË ˆÂ·‡ÎÌË ËÒıÂÏ˘ÌË ÒËÏÔÚÓÏË, ÔÓÚ˘‡˘Ë Ò íàÄ ËÎË àåà, „·‚Ó·ÓÎËÂ, ‡Ï‡‚ÓÁ‡ Ë ·ÓÎÍË ‚˙‚ ‚‡Ú‡. ÑÓÔÎÂÓ‚‡Ú‡- Ë ˆ‚ÂÚÌÓ-ÍÓ‰Ë‡Ì‡Ú‡ ‰ÛÔÎÂÍÒ-ÒÓÌÓ„‡ÙËfl, χ„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl, χ„ÌËÚÌÓ ÂÁÓ̇ÌÒ̇ ‡Ì„ËÓ„‡ÙËfl ËÎË ÍÓÏÔ˛ÚÌÓ-ÚÓÏÓ„‡ÙÒ͇ ‡Ì„ËÓ„‡ÙËfl Ò‡ ̇‰ÂʉÌË ÌÂËÌ‚‡ÁË‚ÌË ÏÂÚÓ‰Ë Á‡ ‰Ë‡„ÌÓÒÚˈË‡ÌÂ Ë ÔÓÒΉfl‚‡Ì ̇ ‰ËÒÂ͇ˆËËÚ ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl. ã˜ÂÌËÂÚÓ ‚Íβ˜‚‡ ıËÛ„˘ÌË ËÌÚÂ‚Â̈ËË Ë Ï‰Ë͇-

ÏÂÌÚÓÁ̇ ÚÂ‡ÔËfl Ò ‡ÌÚˇ„„‡ÌÚË ËÎË ‡ÌÚËÍÓ‡„Û·ÌÚË. èÓ„ÌÓÁ‡Ú‡ ̇ ‰ËÒÂ͇ˆËËÚ ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl  ÓÚÌÓÒËÚÂÎÌÓ ‰Ó·‡. è‰ÒÚ‡‚flÏ fl‰˙Í ÒÎÛ˜‡È ̇ ËÁÓÎË‡Ì‡ Ú‡‚χÚ˘̇ ‰ËÒÂ͇ˆËfl ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl. Ñ‚‡‰ÂÒÂÚ Ë Â‰Ì‡ „Ӊ˯ÂÌ, ·ÂÁ ‡Ì‡ÏÌÂÁ‡ Á‡ Ô‰‚‡ËÚÂÎÌË Á‡·ÓÎfl‚‡ÌËfl Ï˙Ê,  ıÓÒÔËÚ‡ÎËÁË‡Ì ÒΉ ͇ڇÒÚÓÙ‡ Ò ÏÌÓÊÂÒÚ‚Ó ÍÓÒÚÌË Ù‡ÍÚÛË. çflÍÓÎÍÓ ‰ÌË ÒΉ ÔËÂχÌÂÚÓ, Ô‡ˆËÂÌÚ˙Ú ÔÂÊË‚fl‚‡ ‡Ï‡‚ÓÁËÒ ÙÛ„‡ÍÒ ‚ ‰flÒÌÓÚÓ ÓÍÓ. ÑÓÔÎÂÓ‚‡Ú‡- Ë ˆ‚ÂÚÌÓ-ÍÓ‰Ë‡Ì‡Ú‡ ‰ÛÔÎÂÍÒ-ÒÓÌÓ„‡ÙËfl ÔÓ͇Á‚‡Ú Ò‰ÌÓÒÚÂÔÂÌ̇ ÒÚÂÌÓÁ‡ ‚ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ ‰flÒ̇ڇ Ó·˘‡ Ò˙Ì̇ ‡ÚÂËfl, ËÌÚ‡ÏÛ‡ÎÂÌ ıÂχÚÓÏ Ë ‰‚ÓÂÌ ÎÛÏÂÌ. ë ÔÓÏÓ˘Ú‡ ̇ Ú‡ÁË ÛÎÚ‡ÒÓÌÓ„‡ÙÒ͇ ı‡‡ÍÚÂËÒÚË͇  ‰Ë‡„ÌÓÒÚˈË‡Ì‡ Ú‡‚χÚ˘̇ ‰ËÒÂ͇ˆËfl ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl Ë Â Á‡ÔӘ̇ڇ ‡ÌÚËÍÓ‡„Û·ÌÚ̇ ÚÂ‡ÔËfl. éÒÂÏ ÏÂÒˆ‡ ÔÓ-Í˙ÒÌÓ Ô‡ˆËÂÌÚ˙Ú Â ·ÂÁ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ. èÓÒΉfl‚‡˘‡Ú‡ χ„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ‡Ì„ËÓ„‡ÙËfl ‚Ò Ӣ ÔÓ͇Á‚‡ ̇΢ËÂÚÓ Ì‡ هί˂ ÎÛÏÂÌ, Ó·ı‚‡˘‡˘ ̇˜‡ÎÌËfl Ò„ÏÂÌÚ Ì‡ ‰flÒ̇ڇ Ó·˘‡ Ò˙Ì̇ ‡ÚÂËfl. è‰ÒÚ‡‚flÏ ÚÓÁË ÒÎÛ˜‡È ÔÓ‡‰Ë Ì„ӂ‡Ú‡ fl‰ÍÓÒÚ Ë ‰ËÒÍÛÚË‡Ï ‰Ë‡„ÌÓÒÚ˘ÌÓÚÓ Ë ÚÂ‡Ô‚Ú˘ÌÓ Ôӂ‰ÂÌË ÔË ÚÓ‚‡ Á‡·ÓÎfl‚‡ÌÂ. äβ˜Ó‚Ë ‰ÛÏË: Ó·˘‡ Ò˙Ì̇ ‡ÚÂËfl, ‚˙Ú¯̇ Ò˙Ì̇ ‡ÚÂËfl, ‰ËÒÂ͇ˆËfl, ‰ÛÔÎÂÍÒ ÒÓÌÓ„‡ÙËfl. ÖÍÒÚ‡Í‡ÌˇÎÌËÚ ͇ÓÚˉÌË ‰ËÒÂ͇ˆËË Ì‡ÒÚ˙Ô‚‡Ú Ó·ËÍÌÓ‚ÂÌÓ ‚ ӷ·ÒÚÚ‡ ̇ ‚˙Ú¯̇ڇ Ò˙Ì̇ ‡ÚÂËfl (ÇëÄ) Ë Ò‡ ‰̇ ÓÚ Ì‡È-˜ÂÒÚËÚ Ô˘ËÌË Á‡ ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (àåà) ÔË Ô‡ˆËÂÌÚË ‚ Ï·‰‡ ‚˙Á‡ÒÚ (2,3). ÑËÒÂ͇ˆËËÚ ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl (éëÄ) Ò‡ Á̇˜ËÚÂÎÌÓ ÔÓ-‰ÍË Ë Òe ‡ÒÓˆËË‡Ú Ó·ËÍÌÓ‚ÂÌÓ Ò ‰ËÒÂ͇ˆËfl ̇ ‡ÓÚ‡Ú‡ (5). àÁÓÎË‡Ì‡Ú‡ ‰ËÒÂ͇ˆËfl ̇ éëÄ, ·ÂÁ Á‡Òfl„‡Ì ̇ ‡ÓÚ̇ڇ ‰˙„‡, ̇ÒÚ˙Ô‚‡ Ú‚˙‰Â fl‰ÍÓ, ͇ÚÓ ‚ ‡Ì„ÎËÈÒ͇ڇ ÎËÚÂ‡ÚÛ‡ Ò˙˘ÂÒÚ‚Û‚‡Ú ‰ËÌ˘ÌË ÓÔËÒ‡ÌËfl ̇ ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë (5,6). éÔËÒ‚‡Ï ÒÎÛ˜‡È ̇ Ï·‰ Ï˙Ê Ò ËÁÓÎË‡Ì‡ ÔÓÒÚÚ‡‚χÚ˘̇ ‰ËÒÂ͇ˆËfl ̇ éëÄ. äãàçàóÖç ëãìóÄâ 21-„Ӊ˯ÂÌ Ï˙Ê, ·ÂÁ ‡Ì‡ÏÌÂÁ‡ Á‡ ÏË̇ÎË Á‡·ÓÎfl‚‡ÌËfl  ıÓÒÔËÚ‡ÎËÁË‡Ì ‚ ìÌË‚ÂÒËÚÂÚÒ͇ڇ ·ÓÎÌˈ‡ “àÌÁÂίÔËڇΔ ‚ „. ÅÂÌ, ÒΉ ‡‚ÚÓÏÓ·ËÎ̇ ͇ڇÒÚÓÙ‡ ÔÓ‡‰Ë ˜ÂÂÔÌÓ-ÏÓÁ˙˜Ì‡ Ú‡‚χ - Ò˙ÚÂÒÂÌË ̇ ÏÓÁ˙͇, ÏÌÓÊÂÒÚ‚Ó Ù‡ÍÚÛË, Ó·ı‚‡˘‡˘Ë „Ó̇ڇ Ë ‰ÓÎ̇ ˜ÂβÒÚ, Í·‚ËÍÛ·ڇ Ë ‰ÓÎÌËfl Îfl‚ Í‡ÈÌËÍ, ͇ÍÚÓ Ë ÔÓ‡‰Ë ‡Á‚ËÚË ̇ ıÂÏÓÔÌ‚ÏÓÚÓ‡ÍÒ. èË ÔËÂχÌÂÚÓ Í˙‚ÌÓÚÓ Ì‡Îfl„‡Ì  145/100, ÔÛÎÒ‡  90/ÏËÌ. ÅÓÎÌËflÚ Â Ú‡ıÂÓÒÚÓÏË‡Ì, ÔÓÒÚ‡‚ÂÌ Â ÔÂËÚÓ̇ÎÂÌ ‰ÂÌ‡Ê Ë Â ËÁ‚˙¯Â̇ ÂÔÓÁˈËfl ̇ Ù‡ÍÚÛËÚ ̇ ‰ÓÎÌËfl Í‡ÈÌËÍ. çflÍÓÎÍÓ ‰Â̇ ÔÓ-Í˙ÒÌÓ Ô‡ˆËÂÌÚ˙Ú ÔÂÊË‚fl‚‡ Ú‡ÌÁËÚÓ̇ ËÒıÂÏ˘̇ ‡Ú‡Í‡ (íàÄ), ÔÓ‰ ÙÓχڇ ̇ ‡Ï‡‚ÓÁËÁ ÙÛ„‡ÍÒ ‚ ‰flÒÌÓÚÓ ÓÍÓ. éÚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ Ò ÓÚ·ÂÎflÁ‚‡ ıËÔÂÒÚÂÁËfl ‚ ӷ·ÒÚÚ‡ ̇ ‰ÓÎ̇ڇ ÛÒÚ̇, ͇ÍÚÓ Ë ‚ ӷ·ÒÚÚ‡ ̇ ԇΈ‡ ̇ „ÓÌËfl Í‡ÈÌËÍ ‚ Îfl‚Ó. èÓ‡‰Ë Ù‡ÍÚÛ‡Ú‡ ̇ ΂Ëfl Í‡Í ÔÓıӉ͇ڇ Ì ÏÓÊ ‰‡ Ò ËÁÒΉ‚‡, ͇ÚÓ ÓÒڇ̇ÎËflÚ Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ Ì ÔÓ͇Á‚‡ ‰Û„‡ ÙÓ͇Î̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

èӂ‰ÂÌÓÚÓ ÑÓÔÎÂ-‰ÛÔÎÂÍÒ ÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì ÔÓ͇Á‚‡ ‰ËÒÂ͇ˆËfl ‚ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ ‚ ‰flÒÌÓ Ò ı‡‡ÍÚÂÌËÚ Á‡ ‰ËÒÂ͇ˆËflÚ‡ ·ÂÎÂÁË: Á‡‰Â·ÂÎfl‚‡Ì ÒÚÂ̇ڇ ̇ ‡ÚÂËflÚ‡, ÔÂÒÂÌ ıËÔÓÂıÓ„ÂÌÂÌ ËÌÚ‡ÏÛ‡ÎÂÌ ıÂχÚÓÏ, ÏÂÏ·‡Ì‡ ̇ ‰ËÒÂ͇ˆËflÚ‡, ‡Á‰ÂÎfl˘‡ ‡ÚÂËflÚ‡ ̇ هί˂ Ë ËÒÚËÌÒÍË ÎÛÏÂÌ. éÚ·ÂÎflÁ‚‡ Ò Ò‰ÌÓÒÚÂÔÂÌ̇ ÌÂ„ÛÎfl̇ ÒÚÂÌÓÁ‡ ‚ ӷ·ÒÚÚ‡ ̇ ‰ËÒÂ͇ˆËflÚ‡ ̇ éëÄ Ò˙Ò ÒËÒÚÓ΢̇ ‚˙Î̇ ÓÚ 237 ÒÏ./ÒÂÍ. Ë ‰Ë‡ÒÚÓ΢̇ ‚˙Î̇ ÓÚ 30 ÒÏ./ÒÂÍ. ä˙‚ÌËflÚ ÚÓÍ ‚ ÔÓÒÚÒÚÂÌÓÚ˘ÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ Â ÌÓχÎÂÌ. äÓÏÔ˛Ú˙ÌÓ-ÚÓÏÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡Ì ̇ ÚÓ‡ÍÒ‡ Ì ÔÓ͇Á‚‡ Ô‡ÚÓÎӄ˘ÌË ËÁÏÂÌÂÌËfl. èӂ‰Â̇ڇ χ„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ‡Ì„ËÓ„‡ÙËfl ÔÓ͇Á‚‡ ËÁÓÎË‡Ì‡ ‰ËÒÂ͇ˆËfl ‚ ÓÒÌÓ‚‡Ú‡ ̇ éëÄ ‚ ‰flÒÌÓ. åÂÒˆ Ë ÔÓÎÓ‚Ë̇ ÔÓ-Í˙ÒÌÓ ÔË Á‡Ú‚ÓÂÌÓ Ú‡ıÂÓÒÚÓÏÌÓ ÓÚ‚ÂÒÚË ·Â¯Â Ôӂ‰ÂÌÓ ÍÓÌÚÓÎÌÓ ÑÓÔÎÂ‰ÛÔÎÂÍÒ ÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ. éÚ·ÂÎflÁ‡ Ò ̇΢ˠ̇ ‰ËÒÂ͇ˆËfl ‚˙‚ ‚ˉËÏËfl ‚˜ ‰ËÒÚ‡ÎÂÌ Ò„ÏÂÌÚ Ì‡ ÚÛÌÍÛÒ ·‡ıËÓˆÂÙ‡ÎËÍÛÒ, ÔÓÒÚË‡˘‡ Ò ‚ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ. Ç éëÄ ÓÚÌÓ‚Ó Ò ËÌÒÓÌË‡ ̇΢ˠ̇ ÏÂÏ·‡Ì‡ ̇ ‰ËÒÂ͇ˆËfl, ‡Á‰ÂÎfl˘‡ ‡ÚÂËflÚ‡ ̇ هί˂ Ë ËÒÚËÌÒÍË ÎÛÏÂÌ, ͇ÍÚÓ Ë ·Ë‰ËÂ͈ËÓ̇ÎÂÌ Í˙‚ÂÌ ÚÓÍ Ò Ôӂ˯ÂÌÓ Ò˙‰Ó‚Ó Ò˙ÔÓÚË‚ÎÂÌË ‚ ӷ·ÒÚÚ‡ ̇ هί˂Ëfl ÎÛÏÂÌ Ì‡ ‡ÚÂËflÚ‡. éÚ·ÂÎflÁ‡ Ò „ÂÒË‡Ì ̇ ÒÚÂÌÓÁ‡Ú‡ ‚ ӷ·ÒÚÚ‡ ̇ éëÄ- ÎÂÍÓ ‰Ó Ò‰ÌÓÒÚÂÔÂÌÌÓ ÒÚÂÌÓÁË‡Ì Ò˙Ò ÒËÒÚÓ΢̇ ‚˙Î̇ ÓÚ 178 ÒÏ./ÒÂÍ. Ë ‰Ë‡ÒÚÓ΢̇ ‚˙Î̇ ÓÚ 31 ÒÏ./ÒÂÍ. ä˙‚ÌËflÚ ÚÓÍ ‚ ÔÓÒÚÒÚÂÌÓÚ˘ÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ Â ÌÓχÎÂÌ (îË„Û‡ 1). äÓÌÚÓÎÌÓÚÓ ÛÎÚ‡ÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ, Ôӂ‰ÂÌÓ 8 ÏÂÒˆ‡ ÔÓ-Í˙ÒÌÓ ÔÓ͇Á‡ ÎÂÍÓ ÔÓ‰Ó·ÂÌË ̇ ÒÚÂÌÓÁ‡Ú‡ ‚ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ Ò˙Ò ÒËÒÚÓ΢̇ ‚˙Î̇ ÓÚ 158 ÒÏ./ÒÂÍ. Ë ‰Ë‡ÒÚÓ΢̇ ‚˙Î̇ ÓÚ 18 ÒÏ./ÒÂÍ (ÎÂÍÓ- ‰Ó Ò‰ÌÓÒÚÂÔÂÌ̇ ÒÚÂÌÓÁ‡). ÑÛÔÎÂÍÒÒÓÌÓ„‡ÙÒÍË Ò ÓÚ˜ÂÚÂ Ë ÔÓ‰˙Îʇ‚‡˘Ó ̇΢ˠ̇ هί˂ ÎÛÏÂÌ Ò Ôӂ˯ÂÌÓ Ò˙‰Ó‚Ó Ò˙ÔÓÚË‚ÎÂÌËÂ Ë ·Ë‰ËÂ͈ËÓ̇ÎÂÌ Í˙‚ÂÌ ÚÓÍ (îË„Û‡ 2). äÓÌÚÓÎ̇ڇ χ„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ‡Ì„ËÓ„‡ÙËfl, ̇Ô‡‚Â̇ 8 ÏÂÒˆ‡ ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Ë̈ˉÂÌÚ‡, ÔÓ͇Á‡ ËÁÓÎË‡Ì‡ ‰ËÒÂ͇ˆËfl ‚ ÓÒÌÓ‚‡Ú‡ ̇ éëÄ ‚ ‰flÒÌÓ Ò˙Ò ÒÎflÔ ÎÛÏÂÌ Ò ‰˙ÎÊË̇ 1 ÒÏ. ·ÚÂ‡ÎÌÓ ÓÚ ÓÒÌÓ‚‡Ú‡ ̇ éëÄ. çÓχÎÂÌ ‰ËÒÚ‡ÎÂÌ Ò„ÏÂÌÚ Ì‡ éëÄ (îË„Û‡ 3 Ë 4). ÅÓÎÌËflÚ ·Â¯Â ÎÂÍÛ‚‡Ì Ò Ë̉ËÂÍÚÌË ‡ÌÚËÍÓ‡„Û·ÌÚË - χÍÓχ. í‡ÌÁËÚÓ̇ڇ ËÒıÂÏ˘̇ ‡Ú‡Í‡ Ì Ò ÔÓ‚ÚÓË ÔÓ‚Â˜Â Ë ÔË ÚÓ‚‡ ΘÂÌË 8 ÏÂÒˆ‡ ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Ë̈ˉÂÌÚ‡ ·ÓÎÌËflÚ ·Â¯Â ·ÂÁ ÙÓ͇Î̇ ËÎË Ó·˘ÓÏÓÁ˙˜Ì‡ Ì‚ÓÎӄ˘ÂÌ ÒËÏÔÚÓχÚË͇.

îË„Û‡ 1. ë‰ÌÓÒÚÂÔÂÌ̇ ÒÚÂÌÓÁ‡ ‚ ӷ·ÒÚÚ‡ ̇ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ Ò ‰‡ÌÌË Á‡ هί˂ ÎÛÏÂÌ.

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îË„Û‡ 2. éÒÂÏ ÏÂÒˆ‡ ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ - ÎÂÍÓ- ‰Ó Ò‰ÌÓÒÚÂÔÂÌ̇ ÒÚÂÌÓÁ‡ ‚ ӷ·ÒÚÚ‡ ̇ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ. éÅëöÜÑÄçÖ ÉÓÎflχ ˜‡ÒÚ ÓÚ ‰ËÒÂ͇ˆËËÚ ̇ ÂÍÒÚ‡Í‡ÌˇÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË Ì‡ÒÚ˙Ô‚‡Ú ‚ ӷ·ÒÚÚ‡ ̇ ÇëÄ ËÎË ‚ ӷ·ÒÚÚ‡ ̇ ‚ÂÚ·‡ÎÌËÚ ‡ÚÂËË (5). äÎËÌ˘ÌÓ ‰ËÒÂ͇ˆËËÚ ̇ ÇëÄ ÔÓÚ˘‡Ú Ò ı‡‡ÍÚÂ̇ڇ Úˇ‰‡ ÓÚ Â‰ÌÓÒÚ‡ÌÌÓ „·‚Ó·ÓÎËÂ, ÒË̉ÓÏ Ì‡ Claude-BernardHorner Ë ˆÂ·‡ÎÌË ËÎË ÂÚË̇ÎÌË ËÒıÂÏ˘ÌË ÒËÏÔÚÓÏË, ÔÓÚ˘‡˘Ë ͇ÚÓ íàÄ ËÎË àåà (1). äÓÏ·Ë̇ˆËflÚ‡ ÓÚ ÚÂÁË ÒËÏÔÚÓÏË Ò Ò¢‡ Ò‡ÏÓ ‚ ÓÍÓÎÓ 20% ÓÚ ÒÎÛ˜‡ËÚÂ Ò ‰ËÒÂ͇ˆËfl ̇ ÇëÄ (14). ÑËÒÂ͇ˆËËÚ ̇ éëÄ Ò‡ ‰ÍË Ë ‚ „ÓÎflχ ˜‡ÒÚ ÓÚ ÒÎÛ˜‡ËÚ ҇ Ò‚˙Á‡ÌË Ò ‡ÓÚ̇ ‰ËÒÂ͇ˆËfl (5,6). ç‡È-˜ÂÒÚËÚ ÍÎËÌ˘ÌË ÔÓfl‚Ë Ò‡ ÂÚË̇ÎÌË ËÎË ˆÂ·‡ÎÌË ËÒıÂÏ˘ÌË ÒËÏÔÚÓÏË, ÔÓÚ˘‡˘Ë Ò íàÄ ËÎË àåà, „·‚Ó·ÓÎËÂ Ë ÔÓ-fl‰ÍÓ ‡Ï‡‚ÓÁ‡ ËÎË ·ÓÎÍË ‚˙‚ ‚‡Ú‡ (5,7,11,12). ᇠ‡ÁÎË͇ ÓÚ ‰ËÒÂ͇ˆËËÚ ̇ ÇëÄ ÔË ‰ËÒÂ͇ˆËËÚ ̇ éëÄ Ì Ò Ò¢‡ ÒË̉ÓÏ Ì‡ Claude-Bernard-Horner (5). êfl‰ÍÓ ÔË ‚˙‚΢‡Ì ̇ ͇ÓÚˉÌËfl ÒËÌÛÒ Ò ÓÚ·ÂÎflÁ‚‡ ·‡‰Ë͇‰Ëfl ËÎË ıËÔÓÚÓÌËfl (5). àÁÓÎË‡ÌË ‰ËÒÂ͇ˆËË Ì‡ éëÄ, ·ÂÁ Á‡Òfl„‡Ì ̇ ‡ÓÚ‡Ú‡, ̇ÒÚ˙Ô‚‡Ú Ӣ ÔÓ-fl‰ÍÓ Ë Ó·ËÍÌÓ‚ÂÌÓ Ò‡ ‡ÒÓˆËË‡ÌË Ò ¯ËÈ̇ Ú‡‚χ ËÎË Ì‚ÓÂÌÚ„ÂÌÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl, ͇ÚÓ ˆÂ·‡Î̇ ‡Ì„ËÓ„‡ÙËfl ËÎË Ẩӂ‡ÒÍÛ·ÌË Ôӈ‰ÛË (5). Ç ÓÔËÒ‡ÌËfl ÓÚ Ì‡Ò ÒÎÛ˜‡È, ‰ËÒÂ͇ˆËflÚ‡ Ò ‰˙ÎÊË Ì‡ ¯ËÈ̇ Ú‡‚χ ‚ÒΉÒÚ‚Ë ‡‚ÚÓÏÓ·ËÎ̇ ͇ڇÒÚÓÙ‡. ífl Á‡ÔÓ˜‚‡ ÓÚ ·‡ıËÓˆÂÙ‡ÎÌËfl ÚÛÌÍÛÒ Ë ÔÓ‰˙Îʇ‚‡ ‚ éëÄ, ͇ÚÓ Ì ӷı‚‡˘‡ ‡ÓÚ‡Ú‡. èË ÚÓÁË Ô‡ˆËÂÌÚ Ò ̇·Î˛‰‡‚‡ ‰ÌÓÍ‡ÚÌÓ ÂÚË̇Î̇ íàÄ, ÍÓflÚÓ Â Â‰ËÌ ÓÚ ˜ÂÒÚÓ Ò¢‡ÌËÚ ÒËÏÔÚÓÏË ÔË ‰ËÒÂ͇ˆËË Ì‡ éëÄ (5).

îË„Û‡ 3 Ë îË„Û‡ 4. åêÄ – ‰‡ÌÌË Á‡ ËÁÓÎË‡Ì‡ ‰ËÒÂ͇ˆËfl ‚ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ ‚ ‰flÒÌÓ Ò˙Ò ÒÎflÔ ÎÛÏÂÌ Ë ‰˙ÎÊË̇ ‰ËÌ Ò‡ÌÚËÏÂÚ˙ ·ÚÂ‡ÎÌÓ ÓÚ ÓÒÌÓ‚‡Ú‡ ̇ éëÄ. çÓχÎÂÌ ‰ËÒÚ‡ÎÂÌ Ò„ÏÂÌÚ Ì‡ éëÄ


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

àÁ‚ÂÒÚÌÓ Â, ˜Â ‡Ì„ËÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡Ì  Á·ÚÌËfl Òڇ̉‡Ú ÔË ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ ͇ÓÚˉÌË ‰ËÒÂ͇ˆËË. éÒÌÓ‚ÌËÚ ÂÌÚ„ÂÌÓÎӄ˘ÌË ı‡‡ÍÚÂËÒÚËÍË Ì‡ ‰ËÒÂ͇ˆËËÚ ̇ éëÄ Ò‡ ËÌÚ‡ÏÛ‡ÎÂÌ ıÂχÚÓÏ, ͇ÍÚÓ Ë Ì‡Î˘ËÂÚÓ Ì‡ ‰‚ÓÂÌ Ë Ù‡Î¯Ë‚ ÎÛÏÂÌ. Ç Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë ÚÂÁË ·ÂÎÂÁË Ì‡ ‰ËÒÂ͇ˆËflÚ‡ Ò ‚ËÁÛ‡ÎËÁË‡Ú ÔÓ-‰Ó· ÔË Ï‡„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl (åêí), χ„ÌËÚÌÓ ÂÁÓ̇ÌÒ̇ ‡Ì„ËÓ„‡ÙËfl (åêÄ) ËÎË ÍÓÏÔ˛ÚÌÓ-ÚÓÏÓ„‡ÙÒ͇ ‡Ì„ËÓ„‡ÙËfl (ëíÄ) (5,6). ëÔÓ‰ ÌflÍÓË ‡‚ÚÓË ÓÎflÚ‡ ̇ ÍÓÌ‚Â̈ËÓ̇Î̇ڇ ‡Ì„ËÓ„‡ÙËfl ÔË ‰Ë‡„ÌÓÒÚˈË‡ÌÂÚÓ Ì‡ ͇ÓÚˉÌËÚ ‰ËÒÂ͇ˆËË Ì‡Ï‡Îfl‚‡ Ë Úfl ·Ë Ïӄ· ‰‡ ·˙‰Â ̇‰ÂʉÌÓ Á‡ÏÂÒÚÂ̇ ÓÚ åêí, åêÄ ËÎË ëíÄ (5,6,13). åêí Ë åêÄ Ò ËÁÔÓÎÁ‚‡Ú Ì ҇ÏÓ ÔË ‰Ë‡„ÌÓÒÚˈË‡ÌÂÚÓ, ÌÓ Ò˙˘Ó Ë ÔË ‰Ë̇Ï˘ÌÓÚÓ ÔÓÒΉfl‚‡ÌÂÚÓ Ì‡ ͇ÓÚˉÌËÚ ‰ËÒÂ͇ˆËË (6). Ö‰ÌÓ ÓÚ ÓÒÌÓ‚ÌËÚ ÌÂËÌ‚‡ÁË‚ÌË ‰Ë‡„ÌÓÒÚ˘ÌË ËÁÒΉ‚‡ÌËfl ÔË Í‡ÓÚˉÌË ‰ËÒÂ͇ˆËË Â ÑÓÔÎÂÓ‚‡Ú‡- Ë ˆ‚ÂÚÌÓ-ÍÓ‰Ë‡Ì‡Ú‡ ‰ÛÔÎÂÍÒ-ÒÓÌÓ„‡ÙËfl. ï‡‡ÍÚÂÌË Ò‡ ıËÔÓÂıÓ„ÂÌÌËfl ËÌÚ‡ÏÛ‡ÎÂÌ ıÂχÚÓÏ, ‰‚ÓÈÌËflÚ ÎÛÏÂÌ, ‰ËÒÂÍË‡˘‡Ú‡ ÏÂÏ·‡Ì‡, ͇ÍÚÓ Ë Ì‡Î˘ËÂÚÓ Ì‡ ÒÚÂÌÓÚ˘ÌË ÔÓÏÂÌË (4,5). ìÎÚ‡ÒÓÌÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡ÌÂ Â Ë ÓÒÌÓ‚ÂÌ ÌÂËÌ‚‡ÁË‚ÂÌ ÏÂÚÓ‰ Á‡ ÏÓÌËÚÓË‡Ì ̇ ÂÙË͇ÒÌÓÒÚÚ‡ ̇ ΘÂÌËÂÚÓ. èË ÓÔËÒ‡ÌËflÚ ÓÚ Ì‡Ò Ô‡ˆËÂÌÚ ÛÎÚ‡ÒÓÌÓ„‡ÙÒÍË Ò ËÌÒÓÌË‡Ú ÚËÔ˘ÌË Á‡ ‰ËÒÂ͇ˆËfl ̇ éëÄ ÔÓÏÂÌË: Á‡‰Â·ÂÎfl‚‡Ì ÒÚÂ̇ڇ ̇ ‡ÚÂËflÚ‡ Ò Ì‡Î˘Ë ̇ ıËÔÓÂıÓ„ÂÌÂÌ ËÌÚ‡ÏÛ‡ÎÂÌ ıÂχÚÓÏ, ÏÂÏ·‡Ì‡ ̇ ‰ËÒÂ͇ˆËflÚ‡, ‡Á‰ÂÎfl˘‡ ÎÛÏÂ̇ ̇ ‡ÚÂËflÚ‡ ̇ هί˂ Ë ËÒÚËÌÒÍË. Ñˇ„ÌÓÒÚˈË‡ Ò Ò˙˘Ó Ë Ò‰ÌÓÒÚÂÔÂÌ̇ ÌÂ„ÛÎfl̇ ÒÚÂÌÓÁ‡ ‚ ӷ·ÒÚÚ‡ ̇ ‰ËÒÂ͇ˆËflÚ‡ ̇ éëÄ. èӂ‰Â̇ڇ åêÄ ÔÓ͇Á‚‡ هί˂ ÎÛÏÂÌ Ò ‰˙ÎÊË̇ 1 ÒÏ. ·ÚÂ‡ÎÌÓ ÓÚ ÓÒÌÓ‚‡Ú‡ ̇ éëÄ, ÍÓÂÚÓ Ò˙˘Ó  ı‡‡ÍÚÂ̇ åê ̇ıӉ͇ ÔË ‰ËÒÂ͇ˆËË Ì‡ éëÄ. èË ÓÔËÒ‡ÌËfl ÓÚ Ì‡Ò Ô‡ˆËÂÌÚ Ì Ò  ̇ÎÓÊËÎÓ ÔÓ‚Âʉ‡Ì ̇ ‡Ì„ËÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ. åêÄ, ͇ÍÚÓ Ë ÏÌÓ„ÓÍ‡ÚÌÓ Ôӂ‰ÂÌÓÚÓ ÛÎÚ‡ÒÓÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì ҇ ‰ÓÒÚ‡Ú˙˜ÌË Á‡ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ ‰ËÒÂ͇ˆËflÚ‡ ̇ éëÄ, ͇ÍÚÓ Ë Á‡ ÔÓÒΉfl‚‡Ì ËÁıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. èÓ‡‰Ë „ÓÎflχڇ fl‰ÍÓÒÚ Ì‡ ÚÓ‚‡ Á‡·ÓÎfl‚‡ÌÂ, ‚ ˆËÚË‡Ì‡Ú‡ ÓÚ Medline ÎËÚÂ‡ÚÛ‡, Ìflχ ‰‡ÌÌË Á‡ ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËfl, ÍÓËÚÓ ‰‡ ÔÓÒÓ˜‚‡Ú ÓÔÚËχÎ̇ڇ ÚÂ‡ÔËfl, ͇ÍÚÓ Ë ˜ÂÒÚÓÚ‡Ú‡ ̇ ÒÔÓÌÚ‡Ì̇ Â͇̇ÎËÁ‡ˆËfl ̇ ‰ËÒÂ͇ˆËËÚ ̇ éëÄ. Ç ÓÔËÒ‡ÌËÚ ‰ËÌ˘ÌË ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë ΘÂÌËÂÚÓ Â Ï‰Ë͇ÏÂÌÚÓÁÌÓ ËÎË ıËÛ„˘ÌÓ (5,6,8,9,10,12). äÓÌÒÂ‚‡Ú˂̇ڇ ÚÂ‡ÔËfl ‚Íβ˜‚‡ ÔÓ‰˙ÎÊËÚÂÎÌÓ Î˜ÂÌËÂ Ò ‡ÌÚˇ„„‡ÌÚË ËÎË ‡ÌÚËÍÓ‡„Û·ÌÚË ‰Ó ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÌÓχÎ̇ڇ ıÂÏÓ‰Ë̇ÏË͇ (5,6,9,12). ïËÛ„˘̇ڇ ÚÂ‡ÔËfl ‚Íβ˜‚‡ ÔÂÍÛÚ‡Ì̇ Ú‡ÌÒÎÛÏË̇Î̇ ‡Ì„ËÓÔ·ÒÚË͇, ÂÁÂ͈Ëfl ̇ ‰ËÒÂÍË‡˘Ëfl Ò„ÏÂÌÚ ÓÚ ‡ÚÂËflÚ‡, ͇ÍÚÓ Ë ÚÓÏ·ÂÍÚÓÏËfl (5,8,10). èÓ„ÌÓÁ‡Ú‡ ̇ ‰ËÒÂ͇ˆËËÚ ̇ éëÄ Â ÓÚÌÓÒËÚÂÎÌÓ ‰Ó·‡, ͇ÚÓ ÔÓ˜ÚË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ú ÍÎËÌ˘ÌÓ, ÌÓ ÔË ÌflÍÓË ÓÒÚ‡‚‡ ÔÂÒËÒÚË‡˘ ‰‚Ó-

177

ÂÌ ÎÛÏÂÌ (5). éÔËÒ‡ÌËflÚ ÓÚ Ì‡Ò Ô‡ˆËÂÌÚ Ò˙˘Ó Ì Ô‡‚Ë ËÁÍβ˜ÂÌËÂ. íÓÈ Â ÎÂÍÛ‚‡Ì ÔÓ‰˙ÎÊËÚÂÎÌÓ ‚ÂÏÂ Ò ‡ÌÚËÍÓ‡„Û·ÌÚ̇ ÚÂ‡ÔËfl, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó Ì„ӂÓÚÓ Ô˙ÎÌÓ ÍÎËÌ˘ÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡ÌÂ, ͇ÚÓ ÓÒÚ‡‚‡ ‰‡ ÔÂÒËÒÚË‡ هί˂ ÎÛÏÂÌ ‚ ӷ·ÒÚÚ‡ ̇ ÔÓÍÒËχÎÌËfl Ò„ÏÂÌÚ Ì‡ éëÄ. àÁÓÎË‡Ì‡Ú‡ ‰ËÒÂ͇ˆËfl ̇ éëÄ Â Ú‚˙‰Â fl‰ÍÓ Á‡·ÓÎfl‚‡ÌÂ. ëÔÓÌÚ‡Ì̇ ËÎË Ú‡‚χÚ˘ÌÓ Ì‡ÒÚ˙ÔË· Úfl Ó·ËÍÌÓ‚ÂÌÌÓ ‚Ó‰Ë ‰Ó ˆÂ·‡ÎÌË ËÎË ÂÚË̇ÎÌË íàÄ, àåà, „·‚Ó·ÓÎËÂ, ‡Ï‡‚ÓÁ‡, ·ÓÎÍË ‚˙‚ ‚‡Ú‡. èÓ-˜ÂÒÚÓ Â ‡ÒÓˆËË‡Ì‡ Ò ‡ÓÚ̇ ‰ËÒÂ͇ˆËfl. ìÎÚ‡ÒÓÌÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡Ì ‚ ÍÓÏ·Ë̇ˆËfl Ò åêÄ ËÎË Ò˙Ò ëíÄ, ‚ Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë Ò‡ ‰ÓÒÚ‡Ú˙˜ÌË Á‡ ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡. ÑËÒÂ͇ˆËËÚ ̇ éëÄ Ò‡ Ò ÓÚÌÓÒËÚÂÎÌÓ ‰Ó·‡ ÔÓ„ÌÓÁ‡ ÔË Ò‚Ó‚ÂÏÂÌÌÓ Ôӂ‰Â̇ ıËÛ„˘̇ ËÎË ‡ÌÚËÍÓ‡„Û·ÌÚ̇/‡ÌÚˇ„„‡ÌÚ̇ ÚÂ‡ÔËfl. ãàíÖêÄíìêÄ 1. ëÚ‡ÈÍÓ‚ à, ëڇ̂‡ å, èÂÚÓ‚ à, èÂÚÓ‚‡ û, ëÚ‡ÏÂÌÓ‚‡ è. ÑËÒÂ͇ˆËË Ì‡ ͇ÓÚˉÌËÚ ‡ÚÂËË. Å˙΄‡Ò͇ Ì‚ÓÎÓ„Ëfl 2005; 5:65-68. 2. Biousse V, D'Anglejan-Chatillon J, Touboul PJ, et al. Time course of symptoms in extracranial carotid artery dissections. a series of 80 patients. Stroke 1995; 26:235-239. 3. Bogousslavsky J, Pierre P. Ischemic stroke in patients under age 45. Neurol Clin 1992; 10: 113-124. 4. Bonnin P, Giannesini C, Amah G, et al. Doppler sonograpy with dynamic testing in a case of aortic dissection extending to the innominate and right common carotid arteries. Neuroradiology 2003; 45:472 – 475. 5. Chen YC, Lee TH, Chen CJ, et al. Spontaneous common carotidartery dissection. A Case Report and Review of the Literature. Eur Neurol 2003; 50:58-60. 6.de Recondo A, Woimant F, Ille O, et al. Postraumatic common carotid artery dissection. Stroke 1995; 26:705-706. 7. Hirth K, Sander S, Hormann K. Common carotid artery dissection: a rare cause for cervical pain. J Laryngol Otol. 2002; 116:309-311. 8. Humphrey PW, Keller MP, Spadone DP, et al. Spontaneous common carotid artery dissection. J Vasc Surg. 1993 18:95-99. 9. Inoue A, Fukumoto SY, Kumon Y, et al. A case of common carotid artery dissection showing remission in short-term. No Shinkei Geka. 2005; 33:81-85. 10. Kubota T, Niwa J, Chiba M, et al. Common carotid artery dissection propagated from acute aortic dissection: a case successfully treated by PTA. No Shinkei Geka 2000; 28:1015-1021. 11. Lubin J, Capparella , Vecchione M. Acute monocular blindness associated with spontaneous common carotid artery dissection. Ann Emerg Med. 2001 38:332-335. 12. Neudecker S, Bau V, Behrmann C, et al. Unilateral amaurosis as the only focal symptom caused by dissection of the common carotid artery. Klin Monatsbl Augenheilkd. 2004; 221:509-512. 13. Stringaris K, Liberopoulos K, Giaka E, et al. Three-dimensional time-offlight MR angiography and MR imaging versus conventional angiography in carotid artery dissections. Int Angiol 1996; 15:20-25. 14. Sturzenegger M, Steinke W. Dissection of cervicocerebral arteries. Therapeutische Umschau 1996; 53:544-551.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ‰- à‚‡Ì ëÚ‡ÈÍÓ‚, ‰.Ï. É·‚ÂÌ ‡ÒËÒÚÂÌÚ Í˙Ï äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” ÛÎ. ÅflÎÓ ÏÓ 8 1527 ëÓÙËfl íÂÎ: 9432 571 Ö-mail: ivanstaikov@hotmail.com


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

178

éÔËÒ‡ÌË ̇ ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë ÑàÄÉçéëíàóçÄ ëíéâçéëí çÄ íêÄçëäêÄçàÄãçÄíÄ ÑéèãÖêéÇÄ ëéçéÉêÄîàü èêà çÖüëçà åéáöóçéëöÑéÇà áÄÅéãüÇÄçàü Ö. LJÒË΂‡, å. чÒ͇ÎÓ‚ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl åÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” ëÓÙËfl SUMMARY DIAGNOSTIC VALUE OF THE TRANSCRANIAL DOPPLER SONOGRAPHY IN UNCLEAR CEREBRAVASCULAR DISEASES E. Vassileva, M. Daskalov The study presents three cases, revealing the role and the place of transcranial Doppler sonography in diagnosing unclear cerbovascular diseases. The first two cases relate to vasospasm after atypical cerebral hemorrhages (subarachnoid in the first case and intracerebral in the second case) – with non-acute development lacking neck stiffness in spite of the existence of blood in the ventricular system and subarachnoid spaces with atypical clinic symptomatic and initial negative computer tomography finding. The third case concerns ischemic stroke after post traumatical vasospasm. The role of the transcranial Doppler sonography in the study of the brain hemodynamics and detection of vasospasm with different etiology (after subarachnoid hemorrhage or posttraumatic), has been highlighted. Key words: vasospasm, transcranial Doppler sonography, subarachnoid hemorrhage. êÖáûåÖ è‰ÒÚ‡‚flÚ Ò ÚË ÒÎÛ˜‡fl, ÍÓËÚÓ ÔÓ͇Á‚‡Ú ÓÎflÚ‡ Ë ÏflÒÚÓÚÓ Ì‡ Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ‚ ‰Ë‡„ÌÓÒÚË͇ڇ ̇ ÌÂflÒÌË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl. Ç Ô˙‚ËÚ ‰‚‡ ÒÎÛ˜‡fl Ò ͇҇ Á‡ ‚‡ÁÓÒÔ‡Á˙Ï ÒΉ ÌÂÚËÔ˘ÌÓ ÔÓÚ˘‡˘Ë ÏÓÁ˙˜ÌË Í˙‚ÓËÁÎË‚Ë (ÒÛ·‡‡ıÌÓˉÌÓÔ‡ÂÌıËχÚÓÁÂÌ ‚ Ô˙‚Ëfl ÒÎÛ˜‡È Ë ËÌÚ‡ˆÂ·‡ÎÂÌ Ò ÔÓ·Ë‚ ‚˙‚ ‚ÂÌÚËÍÛÎËÚ ‚˙‚ ‚ÚÓËfl) – Ò ÔÓ‰ÓÒÚÓ ‡Á‚ËÚËÂ, ·ÂÁ ̇΢ˠ̇ ÒË̉ÓÏ Ì‡ ÏÂÌËÌ„Ó‡‰ËÍÛÎÂÌÓ ‰‡ÁÌÂÌ ‚˙ÔÂÍË Ì‡Î˘ËÂÚÓ Ì‡ Í˙‚ ‚˙‚ ‚ÂÌÚËÍÛ·̇ڇ ÒËÒÚÂχ Ë ÒÛ·‡‡ıÌÓˉÌËÚ ÔÓÒÚ‡ÌÒÚ‚‡, Ò ÌÂı‡‡ÍÚÂ̇ ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇ Ë Ì„‡Ú˂̇ Ô˙‚Ó̇˜‡Î̇ ÍÓÏÔ˛Ú˙ÚÓÏÓ„‡ÙÒ͇ ̇ıӉ͇. Ç ÚÂÚËfl ÒÎÛ˜‡È Ò ͇҇ Á‡ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ‚˙ÁÌËÍ̇ΠÒΉÒÚ‚Ë ÔÓÒÚÚ‡‚χÚ˘ÂÌ ‚‡ÁÓÒÔ‡Á˙Ï. èÓ‰˜ÂÚ‡‚‡ Ò ÓÎflÚ‡ ̇ Ú‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ‚ ËÁÒΉ‚‡Ì ̇ ÏÓÁ˙˜Ì‡Ú‡ ıÂÏÓ‰Ë̇ÏË͇ Ë ‰ÂÚÂ͈Ëfl ̇ Ò˙‰Ó‚ ÒÔ‡Á˙Ï Ò ‡Á΢̇ ÂÚËÓÎÓ„Ëfl (ÔÓÒÚıÂÏÓ‡„˘ÂÌ Ë Ú‡‚χÚ˘ÂÌ). äβ˜Ó‚Ë ‰ÛÏË: ‚‡ÁÓÒÔ‡Á˙Ï, Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl, ÒÛ·‡‡ıÌÓˉÂÌ Í˙‚ÓËÁÎË‚. äÓÌ‚Â̈ËÓ̇Î̇ڇ Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl  ÌÂËÌ‚‡ÁË‚ÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ Ë ıÂÏÓ‰Ë̇ÏË͇ ‚ ÛÒÎÓ‚ËflÚ‡ ̇ ‡Á΢ÌË Ô‡ÚÓÎӄ˘ÌË Ò˙ÒÚÓflÌËfl, Ò ‰Ó͇Á‡Ì‡ ÓÎfl Ë ‰Ë‡„ÌÓÒÚ˘̇ ÒÚÓÈÌÓÒÚ ÔË ÒÚÂÌÓÁË Ë ÓÍÎÛÁËË Ì‡ ‡ÚÂËËÚ ÓÚ ÇËÎËÁË‚Ëfl Í˙„, ÒÔ‡Á˙Ï Ì‡ ·‡Á‡ÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË, ‰ÂÚÂ͈Ëfl ̇ ÏÓÁ˙˜ÂÌ ÏËÍÓÂÏ·ÓÎËÁ˙Ï, ‰Ë‡„ÌÓÒÚË͇ ̇ ÔÂÒËÒÚË‡˘ ÏÂʉÛÔ‰Ò˙‰ÂÌ ‰ÂÙÂÍÚ Ë ‰. éÚ ÓÒÓ·ÂÌÓ Ô‡ÍÚ˘ÂÒÍÓ Á̇˜ÂÌË  ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ÓÚÍË‚‡Ì ̇ Ò˙‰Ó‚ ÒÔ‡Á˙Ï, ÍÓÈÚÓ ÏÓÊ ‰‡ Ò ‡Á‚Ë ÔË ‡Á΢ÌË Ô‡ÚÓÎӄ˘ÌË Ò˙ÒÚÓflÌËfl Ë ‰‡ ‰Ó‚‰ ‰Ó ÔÓfl‚‡ ̇ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ (1-3). ë˙‰Ó‚ËflÚ ÒÔ‡Á˙Ï Â ÔÂıÓ‰ÂÌ ÔÓˆÂÒ,

ÍÓÈÚÓ ÏÓÊ ‰‡ ·˙‰Â „ÂÌÂ‡ÎËÁË‡Ì ËÎË Ò Ô‰ËÎÂ͈Ëfl ‚ ÓÔ‰ÂÎÂÌ Ò˙‰Ó‚ ·‡ÒÂÈÌ. ç‡È-˜ÂÒÚÓ ‚˙ÁÌËÍ‚‡ ÒΉ ÒÛ·‡‡ıÌÓˉ̇ ıÂÏÓ‡„Ëfl, ÌÓ ÏÓÊ ‰‡ Ëχ Ë ÔÓÒÚÚ‡‚χÚ˘̇ (‚Íβ˜ËÚÂÎÌÓ ÔÓÒÚÓÔÂ‡Ú˂̇), ËÌÙÂ͈ËÓÁ̇ (ÔË ·‡ÍÚÂˇÎÌË ÏÂÌËÌ„ËÚË) „ÂÌÂÁ‡ ͇ÍÚÓ Ë ‰‡ ·˙‰Â Ë̉ۈË‡Ì ÓÚ ÔËÂÏ Ì‡ ÓÔ‰ÂÎÂÌË ÒÛ·Òڇ̈ËË (ÍÓ͇ËÌ Ë ‰.) (410). è‰ÒÚ‡‚flÚ Ò ÚË ÒÎÛ˜‡fl Ò ‰ÓÔÎÂÒÓÌÓ„‡ÙÒ͇ ‰Ë‡„ÌÓÒÚË͇ ̇ „ÂÌÂ‡ÎËÁË‡Ì ËÎË ÎÓ͇ÎÂÌ ‚‡ÁÓÒÔ‡Á˙Ï Ò ‡Á΢̇ ÂÚËÓÎÓ„Ëfl. ëãìóÄâ 1 46 „Ӊ˯̇ ·ÓÎ̇ Ò ‡Ì‡ÏÌÂÒÚ˘ÌË ‰‡ÌÌË Á‡ ÌÂÎÂÍÛ‚‡Ì‡ ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl, ıÓÒÔËÚ‡ÎËÁË‡Ì‡ ÔÓ ÔÓ‚Ó‰ ËÁÚ˙Ô‚‡ÌÂ Ë Ò··ÓÒÚ Ì‡ ÎÂ‚Ë Í‡ÈÌËˆË Ë Îfl‚‡ Îˈ‚‡ ÔÓÎÓ‚Ë̇ Ò ‰‡‚ÌÓÒÚ ÓÚ 7-10 ‰ÌË. èË ÔËÂχÌÂÚÓ Ò ÛÒÚ‡ÌÓ‚Ë ˆÂÌÚ‡Î̇ ΂ÓÒÚ‡Ì̇ ıÂÏËÔ‡ÂÁ‡ Ë ÎÂÁËfl ̇ 7 Ë 12 ˜ÂÂÔÌÓÏÓÁ˙˜ÌË ÌÂ‚Ë. äÓÏÔ˛Ú˙̇ڇ ÚÓÏÓ„‡ÙËfl ̇ „·‚ÂÌ ÏÓÁ˙Í Ì ÔÓ͇Á‡ Ô‡ÚÓÎӄ˘ÌË ÔÓÏÂÌË (ÙË„.1). ÖÍÒÚ‡Í‡ÌˇÎ̇ڇ Ë ˆ‚ÂÚ̇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ËÁÍβ˜Ëı‡ ÒÚÂÌÓÚ˘ÂÌ ÔÓˆÂÒ Ì‡ ͇ÓÚˉÌË Ë ‚ÂÚ·‡ÎÌË ‡ÚÂËË. í‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ‡ÁÍË ÒËÎÌÓ Ôӂ˯ÂÌË ÒÍÓÓÒÚÌË Ô‡‡ÏÂÚË, ÍÓËÚÓ Ì‡ÒÓ˜Ëı‡ Í˙Ï ÚÂÊÍÓÒÚÂÔÂÌÂÌ ÒÔ‡Á˙Ï Ì‡ ‰flÒ̇ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl (Vs 340 cm/s Vmean 295 cm/s Ë̉ÂÍÒ Ì‡ Lindegard – 6) (ÙË„.2,3) Ë Á̇˜ËÚÂÎÌÓ ÔÓ-ÎÂÍ Á‡ Îfl‚‡ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl ‚‡ÁÓÒÔ‡Á˙Ï. èӂ‰ÂÌÓ ·Â ΘÂÌËÂ Ë Ú‡ÌÒÍ‡ÌˇÎÌÓ ‰ÓÔÎÂÓ‚Ó ÏÓÌËÚÓË‡Ì ̇ Ò˙‰Ó‚Ëfl ÒÔ‡Á˙Ï, ͇ÚÓ Ò „ËÒÚË‡ ÔÓÒÚÂÔÂÌÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ ÒÍÓÓÒÚÌËÚ ÔÓ͇Á‡ÚÂÎÌË Ë Ô˙ÎÌÓ ÓÚÁ‚Û˜‡‚‡Ì ̇ ÒÔ‡Á˙χ ‚ ‡ÏÍËÚ ̇ ÓÒÂÏ ‰ÌË (ÙË„.4, 5). ᇠÂÚËÓÎӄ˘ÌÓ ËÁflÒÌfl‚‡Ì ̇ ‚‡ÁÓÒÔ‡Á˙χ ·flı‡ Ôӂ‰ÂÌË ÎËÍ‚ÓÌÓ ËÁÒΉ‚‡Ì (34mg% ·ÂÎÚ˙Í, 63/3 ÏÓÌÓÌÛÍ·ÌË ÍÎÂÚÍË) Ò ‚ËÛÒÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ, ͇ÍÚÓ Ë ÒÂÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì Á‡ ÎÛÂÒ, å‡ÌÚÛ Ë ÍÓÌÚÓÎ̇ ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl ̇ „·‚ÂÌ ÏÓÁ˙Í (ÚË ‰ÌË ÒΉ ıÓÒÔËÚ‡ÎËÁ‡ˆËflÚ‡), ÍÓËÚÓ Ì ÔÓ͇Á‡ı‡ Ô‡ÚÓÎӄ˘ÌË ÔÓÏÂÌË (ÙË„.6). 凄ÌËÚÌÓÂÁÓ̇ÌÒÌÓÚÓ ËÁÒΉ‚‡Ì ̇ „·‚ÂÌ ÏÓÁ˙Í ‡ÁÍË ËÌÚ‡ˆÂ·‡Î̇ Í˙‚̇ ÍÓÎÂ͈Ëfl ‚ ‰flÒ̇ Ô‡ËÂÚ‡Î̇ ӷ·ÒÚ ‚ ÒÚ‡‰ËÈ Ì‡ ÂÁÓ·ˆËfl (ÙË„.7). Ç˙ÔÂÍË ‰Ó·ÓÚÓ ÍÎËÌ˘ÌÓ ‡Á‚ËÚËÂ Ë ÓÚÁ‚Û˜‡‚‡Ì ̇ ‚‡ÁÓÒÔ‡Á˙χ, ̇ ‰ÂÒÂÚËfl ‰ÂÌ ·Â ̇·Î˛‰‡‚‡Ì ˆˉ˂ ̇ ÒÛ·‡‡ıÌÓˉ̇ ıÂÏÓ‡„Ëfl. ÄÌ„ËÓ„‡ÙÒÍË Ò ÛÒÚ‡ÌÓ‚Ë ‡Ì‚ËÁχ ̇ ÒËÙÓ̇ ̇ ‡ÚÂËfl ͇ÓÚËÒ ËÌÚÂ̇ ‚ ‰flÒÌÓ (ÙË„.8). ëãìóÄâ 2 48 „Ó‰. Ï˙Ê Ò ‡Ì‡ÏÌÂÒÚ˘ÌË ‰‡ÌÌË Á‡ ÔÂ͇‡Ì ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ ‚ ·‡ÒÂÈ̇ ̇ Îfl‚‡ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl, ‰̇ „Ó‰Ë̇ ÔÂ‰Ë ıÓÒÔËÚ‡ÎËÁ‡ˆËflÚ‡, ÔËÂÚ ÔÓ ÒÔ¯ÌÓÒÚ ÔÓ‡‰Ë ‚ÌÂÁ‡ÔÌÓ ‚˙ÁÌËÍ̇· Ò··ÓÒÚ Ì‡ ‰ÂÒÌË Í‡ÈÌˈË. ìÒÚ‡ÌÓ‚Â̇ ·Â ˆÂÌÚ‡Î̇ ‰ÂÒÌÓÒÚ‡Ì̇ ıÂÏËÔ‡ÂÁ‡ Ë ıÂÏËıËÔÂÒÚÂÁËfl. äÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl ̇ „·‚ÂÌ ÏÓÁ˙Í ÔÓ͇Á‡ ÒÚ‡‡ ËÒıÂÏ˘̇ ÁÓ̇ ‚ Îfl‚‡ ÚÂÏÔÓ‡Î̇ ӷ·ÒÚ. ÖÍÒÚ‡Í‡ÌˇÎ̇ڇ Ë ˆ‚ÂÚ̇ڇ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ËÁÍβ˜Ëı‡ ÂÍÒÚ‡Í‡ÌˇÎÌË ÒÚÂÌÓÁË. í‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ‰ÂÏÓÌ-


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„. 1 KT ̇ „·‚ÂÌ ÏÓÁ˙Í (1 ‰ÂÌ)

îË„. 6 äí ̇ „·‚ÂÌ ÏÓÁ˙Í (5 ‰ÂÌ)

îË„. 2 TCD a. cerebri media dex (1 ‰ÂÌ).

îË„. 3 TCD a. cerebri media sin (1 ‰ÂÌ).

îË„. 7 üåê ̇ „·‚ÂÌ ÏÓÁ˙Í

îË„. 4 TCD a cerebri media dex (8 ‰ÂÌ).

îË„. 5 TCD a. cerebri media sin (8 ‰ÂÌ).

îË„. 8 åÓÁ˙˜Ì‡ ‡Ì„ËÓ„‡ÙËfl

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˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

ÒÚË‡ ‰‚ÛÒÚ‡ÌÂÌ Ò˙‰Ó‚ ÒÔ‡Á˙Ï Ì‡ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl (Vs 260, Vmean 200, Ë̉ÂÍÒ Ì‡ Lindegard 4), ÍÓÂÚÓ Ì‡ÒÓ˜Ë Í˙Ï ÔÓ‚Âʉ‡Ì ̇ ÎËÍ‚ÓÌÓ ËÁÒΉ‚‡Ì – Í˙‚‡‚ ÎËÍ‚Ó, ÒΉ ˆÂÌÚÓÙÛ„Ë‡Ì ÍÒ‡ÌÚÓıÓÏÂÌ. äÓÌÚÓÎ̇ ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl ̇ „·‚ÂÌ ÏÓÁ˙Í (̇ ÚÂÚËfl ‰ÂÌ ÒΉ ıÓÒÔËÚ‡ÎËÁ‡ˆËflÚ‡) ‚ËÁÛ‡ÎËÁË‡ ËÌÚ‡ˆÂ·‡ÎÂÌ Í˙‚ÓËÁÎË‚ Ò ÔÓ·Ë‚ ‚˙‚ ‚ÂÌÚËÍÛÎ̇ڇ ÒËÒÚÂχ. ç Ò ÛÒÚ‡ÌÓ‚Ëı‡ ‡Ì„ËÓ„‡ÙÒÍË ‰‡ÌÌË Á‡ ‡Ì‚ËÁÏË Ë Ò˙‰Ó‚Ë Ï‡ÎÙÓχˆËË. ëãìóÄâ 3 56 „Ó‰ ÊÂ̇ ÒΉ ÓÔÂ‡ˆËfl ÔÓ ÔÓ‚Ó‰ ‡‡ıÌÓˉ̇ ÍËÒÚ‡ ‚ Îfl‚‡ ÚÂÏÔÓ‡Î̇ ӷ·ÒÚ. Ç ‡ÌÌËfl ÒΉÓÔÂ‡ÚË‚ÂÌ ÔÂËÓ‰ ·Â ̇·Î˛‰‡‚‡Ì‡ ÔÓfl‚‡ ̇ ÒÂÌÁÓÏÓÚÓ̇ ‡Ù‡ÁËfl. äÓÏÔ˛Ú˙̇ڇ ÚÓÏÓ„‡ÙËfl ̇ „·‚ÂÌ ÏÓÁ˙Í Ì ÔÓ͇Á‡ ‰‡ÌÌË Á‡ ÏÓÁ˙˜Ì‡ ËÒıÂÏËfl. í‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ‡ÁÍË ÒÔ‡Á˙Ï Ì‡ ‡. ˆÂ·Ë Ï‰Ëfl ÒËÌËÒÚ‡. (Vs 220 Vmean 170 Ë̉ÂÍÒ Ì‡ Lindegard - 3). èӂ‰ÂÌÓ ·Â ÍÓÌÒÂ‚‡ÚË‚ÌÓ Î˜ÂÌËÂ Ë ÏÓÌËÚÓË‡Ì ̇ Ò˙‰Ó‚Ëfl ÒÔ‡Á˙Ï, ÍÓÈÚÓ ÔÓ͇Á‡ ÔÓÒΉ‚‡˘Ó ÓÚÁ‚Û˜‡‚‡Ì ‚ ‡ÏÍËÚ ̇ 7 ‰ÌË. äÎËÌ˘ÌËÚ ‰‡ÌÌË Ì‡ÒÓ˜Ëı‡ Í˙Ï ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ Ò Ô˙ÎÌÓ Ó·‡ÚÌÓ ‡Á‚ËÚËÂ. éÅëöÜÑÄçÖ è˙‚ËÚ ‰‚‡Ï‡ ·ÓÎÌË Ò‡ ‚ ÓÚÌÓÒËÚÂÎÌÓ Ï·‰‡ ‚˙Á‡ÒÚ – 46-48 „., Ò Â‰ËÌ ËÒÍÓ‚ Ù‡ÍÚÓ (‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl). à ‚ ‰‚‡Ú‡ ÒÎÛ˜‡fl ÎËÔÒ‚‡ ı‡‡ÍÚÂ̇ Á‡ ÏÓÁ˙˜Ì‡ ıÂÏÓ‡„Ëfl ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇. äÓÏÔ˛Ú˙̇ڇ ÚÓÏÓ„‡ÙËfl, ÓÒ˙˘ÂÒÚ‚Â̇ ÔË ÔËÂχÌ ̇ ·ÓÎÌËÚ ÓÚı‚˙ÎË Ì‡Î˘ËÂÚÓ Ì‡ Ô‡ÂÌıËÏÂÌ ËÎË ÒÛ·‡‡ıÌÓˉÂÌ Í˙‚ÓËÁÎË‚ ͇ÚÓ ‚ËÁÛ‡ÎËÁË‡ ÒÚ‡‡ ËÒıÂÏ˘̇ ÁÓ̇ ÔË ‚ÚÓËfl ·ÓÎÂÌ. äÎËÌ˘ÌÓÚÓ ÏËÒÎÂÌ Ò ̇ÒÓ˜Ë ‚ ‰Û„‡ ÔÓÒÓ͇ ÒΉ ÔÓ‚Âʉ‡Ì ̇ ÍÓÌ‚Â̈ËÓ̇Î̇ Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl. ç‡Î˘ËÂÚÓ Ì‡ Ò˙‰Ó‚ ÒÔ‡Á˙Ï Ì‡ a. cerebri media Òڇ̇ ÓÒÌÓ‚‡ÌË Á‡ ÔÓ‚Âʉ‡Ì ̇ ÎËÍ‚ÓÌÓ ËÁÒΉ‚‡ÌÂ Ë ÔÓ‚ÚÓ̇ ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl ͇ÍÚÓ Ë Ï‡„ÌËÚÌÓfl‰ÂÌÓ ËÁÒΉ‚‡Ì ‚˙‚ ‚ÚÓËfl ÒÎÛ˜‡È. ãËÍ‚Ó̇ڇ ̇ıӉ͇ Ë ÍÓÌÚÓÎ̇ڇ ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl ‡ÁÍËı‡ ̇΢ËÂÚÓ Ì‡ Ô‡ÂÌıËÏÌÓ-ÒÛ·‡‡ıÌÓˉ̇ ıÂÏÓ‡„Ëfl. èË Ô˙‚‡Ú‡ ·ÓÎ̇ Ò ÔÓÎÛ˜Ëı‡ Ì„‡ÚË‚ÌË ÒÍÂÌË„‡ÙÒÍË Ë ÎËÍ‚ÓÌË ÂÁÛÎÚ‡ÚË. èӂ‰ÂÌÓÚÓ ‚ÔÓÒΉÒÚ‚Ë χ„ÌËÚÌÓÂÁÓ̇ÌÒÌÓ ËÁÒΉ‚‡Ì ‚ËÁÛ‡ÎËÁË‡ ̇΢ËÂÚÓ Ì‡ ËÌÚ‡Ô‡ÂÌıËÏ̇ ıÂÏÓ‡„Ëfl ‚ ÒÚ‡‰ËÈ Ì‡ ÂÁÓ·ˆËfl. Ç ÓÔËÒ‡ÌËÚ ÒÎÛ˜‡Ë Ò ͇҇ Á‡ ÌÂÚËÔ˘ÌÓ ÔÓÚ˘‡˘Ë ÏÓÁ˙˜ÌË Í˙‚ÓËÁÎË‚Ë (ÒÛ·‡‡ıÌÓˉÌÓ-Ô‡ÂÌıËχÚÓÁÂÌ Ò ˆˉ˂ ̇ ÒÛ·‡‡ıÌÓˉÌËfl Í˙‚ÓËÁÎË‚ ‚ Ô˙‚Ëfl Ë ËÌÚ‡ˆÂ·‡ÎÂÌ Ò ÔÓ·Ë‚ ‚˙‚ ‚ÂÌÚËÍÛÎËÚ ‚˙‚ ‚ÚÓËfl) – ·ÂÁ ̇΢ˠ̇ ÒË̉ÓÏ Ì‡ ÏÂÌËÌ„Ó‡‰ËÍÛÎÂÌÓ ‰‡ÁÌÂÌ ‚˙ÔÂÍË Ì‡Î˘ËÂÚÓ Ì‡ Í˙‚ ‚˙‚ ‚ÂÌÚËÍÛ·̇ڇ ÒËÒÚÂχ Ë ÒÛ·‡‡ıÌÓˉÌËÚ ÔÓÒÚ‡ÌÒÚ‚‡, Ú.Â. Ò ÌÂı‡‡ÍÚÂ̇ ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇. è˙‚‡Ú‡ ·ÓÎ̇  ıÓÒÔËÚ‡ÎËÁË‡Ì‡ ‚ ÒÚ‡‰Ëfl ̇ ‚‡ÁÓÒÔ‡Á˙Ï ÒΉ ÒÛ·‡‡ıÌÓˉÌÓ-Ô‡ÂÌıËχÚÓÁÂÌ Í˙‚ÓËÁÎË‚, ‰Ó‚ÂÎ ‰Ó ‚˙ÁÌËÍ‚‡Ì ̇ Ó„ÌË˘Ì‡ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇. LJÁÓÒÔ‡Á˙Ï˙Ú ÔË ÒÛ·‡‡ıÌÓˉÂÌ Í˙‚ÓËÁÎË‚ Ëχ ÚËÔ˘ÂÌ ıÓ‰ Ë Â‚ÓβˆËfl ‚˙‚ ‚ÂÏÂÚÓ ÔÂÁ Ô˙‚ËÚ ˜ÂÚËË Ò‰ÏˈË. é·ËÍÌÓ‚ÂÌÓ Á‡ÔÓ˜‚‡ ÓÚ ÚÂÚËfl ‰ÂÌ, Ò Ï‡ÍÒËχÎ̇ ËÁfl‚‡ ̇ 6-8 ‰ÂÌ, ÒΉ ÍÓÂÚÓ Ò Á‡‰˙ʇ ̇ ‰ÌÓ Ú‡ÈÌÓ ÌË‚Ó Ë ÓÚ 21-25 ‰ÂÌ Á‡ÔÓ˜‚‡ ÔÓÒÚÂÔÂÌÌÓ ‰‡ ÓÚÁ‚Û˜‡‚‡ (11-13). Ç Ï‡Î˙Í ÔÓˆÂÌÚ ÓÚ ÒÎÛ˜‡ËÚ ÏÓÊ ‰‡ Ò ‡Á‚Ë ‡ÌÂÌ ‚‡ÁÓÒÔ‡Á˙Ï, ͇Í˙‚ÚÓ Â Ì‡·Î˛‰‡‚‡Ì ÔË ‚ÚÓËfl Ô‡ˆËÂÌÚ (14). ê‡ÌÌËflÚ ‚‡ÁÓÒÔ‡Á˙Ï Ì‡ÒÚ˙Ô‚‡ ‚ Ô˙‚ËÚ 48 ˜‡Ò‡ ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Í˙‚ÓËÁÎË‚‡, Ò‚˙Á‡Ì Â Ò Ôӂ˯ÂÌ ËÒÍ ÓÚ ËÒıÂÏ˘ÂÌ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ Ë Ò ÔÓ-ÎÓ¯‡ ÔÓ„ÌÓÁ‡. Qureshi A. et al ÓÚÍË‚‡Ú ‡Ì„ËÓ„‡ÙÒÍË ‰‡ÌÌË Á‡ ‡ÌÂÌ ‚‡-

180

ÁÓÒÔ‡Á˙Ï ‚ 13% ÓÚ 296 ·ÓÎÌË Ò˙Ò ÒÛ·‡‡ıÌÓˉ̇ ıÂÏÓ‡„Ëfl (14). íÂÚ‡Ú‡ ·ÓÎ̇ Â Ò ÔÓÒÚÓÔÂ‡ÚË‚ÂÌ (ÔÓÒÚÚ‡‚χÚ˘ÂÌ) ÒÔ‡Á˙Ï Ì‡ ‡. cerebri media. àÁıӉ̇ڇ ˜‡ÒÚ Ì‡ Ò‰̇ڇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl Ë ÌÂÈÌËÚ ÚÂÏÔÓ‡ÎÌË ÍÎÓ̘ÂÚ‡ Ò‡ ÓÒÓ·ÂÌÓ ‡ÌËÏË ‚ ·‡Á‡ÎÌÓÚÓ ÒÛ·‡‡ıÌÓˉÌÓ ÔÓÒÚ‡ÌÒÚ‚Ó ÔÓ‡‰Ë ·ÎËÁÓÒÚÚ‡ ÒË Ò˙Ò ÒÙÂÌÓˉ̇ڇ ÍÓÒÚ. åÂı‡Ì˘ÌËÚ ‰‡ÁÌËÚÂÎË ÏÓÊ ‰‡ ÔÓ‚ÓÍË‡Ú ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ Í‡ÚÍÓÚ‡ÂÌ ‚‡ÁÓÒÔ‡Á˙Ï ‚ ‡ÌÌËfl ÔÓÒÚÓÔÂ‡ÚË‚ÂÌ ËÎË ÔÓÒÚÚ‡‚χÚ˘ÂÌ ÔÂËÓ‰, ‰ÓË ÍÓ„‡ÚÓ Ìflχ ÒÛ·‡‡ıÌÓˉ̇ ıÂÏÓ‡„Ëfl. íÓÈ Ò ÔÓfl‚fl‚‡ ÓÚ 12 ˜‡Ò ‰Ó 4 ‰ÂÌ ÒΉ Ú‡‚χÚ˘ÌËfl ÏÓÏÂÌÚ Ë ÔÓ‰˙Îʇ‚‡ ÓÚ 12 ˜‡Ò‡ ‰Ó 14 ‰ÌË (5, 15-17). Ç Ì‡¯Ëfl ÒÎÛ˜‡È ̇˜‡ÎÓÚÓ Ì‡ ÔÓÒÚÓÔÂ‡ÚË‚ÌËfl ‚‡ÁÓÒÔ‡Á˙Ï ·Â 3 ‰ÌË ÒΉ ËÌÚÂ‚Â̈ËflÚ‡, ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ÏÛ - 7 ‰ÌË Ë ÔÓ„ÌÓÁ‡Ú‡ - ·Î‡„ÓÔËflÚ̇. íÓÈ Ô‰ËÁ‚Ë͇ ÒËÏÔÚÓχÚË͇ ̇ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Ò Ô˙ÎÌÓ Ó·‡ÚÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÒÂÌÁÓÏÓÚÓ̇ڇ ‡Ù‡ÁËfl ‚ ‡ÏÍËÚ ̇ 20 ‰ÌË. LJÁÓÒÔ‡Á˙Ï˙Ú ‡Á‚Ë‚‡˘ Ò ÒΉ ÒÛ·‡‡ıÌÓˉÂÌ Í˙‚ÓËÁÎË‚ Ë ÔÓÒÚÚ‡‚χÚ˘ÌËflÚ ‚‡ÁÓÒÔ‡Á˙Ï ‚Ó‰flÚ ‰Ó ÒıÓ‰ÌË Ò˙‰Ó‚Ë ÏÓÙÓÎӄ˘ÌË ÔÓÏÂÌË, ‚˙ÔÂÍË ‡Á΢̇ڇ ËÏ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ - ‡ÔÓÔÚÓÁ‡ Ë ‰ÂÒÍ‚‡Ï‡ˆËfl ̇ ẨÓÚÂÎÌË ÍÎÂÚÍË, Á‡‰Â·ÂÎfl‚‡Ì ̇ ÒÛ·ËÌÚËχÎÌËfl ÒÎÓÈ, ÙË·ÓÁË‡Ì ̇ ÏÛÒÍÛÎÌËfl ÒÎÓÈ (4,5). Ç˙ÔÂÍË ˜Â ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ Ò˙‰Ó‚ ÒÔ‡Á˙Ï ÏÓÊ ‰‡ ·˙‰Â ÔÓÚ‚˙‰ÂÌÓ ‰ÂÙËÌËÚË‚ÌÓ Ò‡ÏÓ ˜ÂÁ ÏÓÁ˙˜Ì‡ ‡Ì„ËÓ„‡ÙËfl, ÍÓÏÔÎÂÍÒ̇ڇ ÓˆÂÌ͇ ÓÚ ËÁÏÂ‚‡ÌÂÚÓ Ì‡ ÒÍÓÓÒÚÌËÚ ÔÓ͇Á‡ÚÂÎË Ì‡ ·‡Á‡ÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ‰‡ÌÌËÚ ÓÚ ‰Û„Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ËÁÒΉ‚‡ÌËfl Ë ÍÎËÌ˘̇ڇ ͇ÚË̇ ‰‡‚‡ ËÌÙÓχˆËfl ͇ÍÚÓ Á‡ Ì„ӂ‡Ú‡ ÔÓfl‚‡Ú‡ ڇ͇ Ë Á‡ Ì„ӂ‡Ú‡ ÔÓÒΉ‚‡˘‡ ‰Ë̇ÏË͇. àÁÓÎË‡ÌÓÚÓ Ú˙ÎÍÛ‚‡Ì ҇ÏÓ Ì‡ ÒÚÓÈÌÓÒÚËÚ ̇ ÒÍÓÓÒÚÌËÚ ÔÓ͇Á‡ÚÂÎË Ì ÏÓÊ ‰‡ Ò ËÁÔÓÎÁ‚‡ Á‡ ̇‰ÂʉÌÓ ÍÎËÌ˘ÌÓ ÔÓ„ÌÓÁË‡Ì ̇ Í˙ÒÂÌ ËÒıÂÏ˘ÂÌ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ. ëÂÌÁËÚË‚ÌÓÒÚÚ‡ ̇ Ú‡ÌÒÍ‡ÌˇÎ̇ڇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl ‚ ‰ÂÚÂ͈Ëfl ̇ ‡Ì„ËÓ„‡ÙÒÍË ‚ËÁÛ‡ÎËÁË‡Ì ÒÔ‡Á˙Ï Â 8485%, ‡ ÌÂÈ̇ڇ ÒÔˆËÙ˘ÌÓÒÚ – 89-98% (18). äÓÌ‚Â̈ËÓ̇Î̇ڇ Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl  ÓÚ Á̇˜ÂÌË Á‡ ‰ËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡ ̇ ÛÒÎÓÊÌÂÌËflÚ‡ ÔË ÒÛ·‡‡ıÌÓˉÂÌ Í˙‚ÓËÁÎË‚ Ë Ò ‰Ó͇Á‡Ì‡ ÓÎfl ‚ ÏÓÌËÚÓË‡ÌÂÚÓ Ì‡ ÒÔ‡Á˙χ. ãàíÖêÄíìêÄ 1. Lindegaard K,Bakke S, Sorteberg W et al. A non-invasive Doppler ultrasound method for the evaluation of patients with subarachnoid hemorrhage. Acta Radiol. 1986, suppl 369, 96-98. 2. Sloan M, Burch C, Wozniak M et al. Transcranial Doppler detection of vertebrobasilar vasospasm following subarachnoid hemorrhage. Stroke. 1994, 25, 218797. 3. Sloan M, Alexandrov A, Tegeler C et al. Assessment: Transcranial Doppler ultrasonography. Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2004, 62,14681481. 4. Zubkov A, Lewis A, Raila F, Zhang J, Parent A. Risk factors for the development of post-traumatic cerebral vasospasm. Surg Neurol. 2000, 56, 260-266. 5. Zubkov Apilkington A, Bernanke D, Parent A, Zhang J. Posttraumatic cerebral vasospasm: clinical and morphological presentations. J Neurotrauma. 1999, 16, 763-770 6. Yamashima T, Kashihara K, Ikeda K et al. Three phases of cerebral arteriopathy in meningitis: vasospasm and vasodilatation followed by organic stenosis. Neurosurgery. 1985, 16, 546-53. 7. Pfister H, Borasio G, Dirnagl U et al. Cerebrovascular complications of bacterial meningitis in adults. Neurology. 1992, 42, 1497-1504. 8. Muller M, Merkelbach S, Hermes M et al. Relationship between short-term outcome and occurrence of cerebral artery stenosis in survivors of bacterial meningitis. J Neurol. 1998, 245, 87-92. 9. Hading H, Rotzer H, Reindl H et al. Time course of cerebral blood flow velocity in central nervous system infections. A transcranial Doppler sonography study. Archives of Neurology. 1993, 50, 1399-1403. 10. Siebler M. Bilateral symmetrical basal ganglia infarction after intravenous use of cocaine and heroine. Clin Imaging. 2001, 25, 12-4. 11. Aaslid R. Transcranial Doppler assessment of cerebral vasospasm. European Journal of Ultrasound. 2002, 16, 3-10. 12. Aaslid R. Hemodynamics of cerebrovascular spasm. Acta Neurochir (Wien). 1999, 72 (Supl), 47-57. 13. Sieler R, Newell D. Subarachnoid Hemorrhage and vasospasm. In Transcranial Doppler Newell D, Aaslid R, Raven Press 1992, p.101-107. 14. Qureshi A, Surng G, Suri M et al. Prognostic value and determinants of ultra early angiographic vasospasm after aneurysmal subarachnoid hemorrhage.


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Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

Neurosurgery.1999, 44(5), 967-73. 15. Arutiunov A, Baron M, Majorova N. The role of mechanical factors in the pathogenesis of short-term and prolonged spasm of the cerebral arteries. J Neurosurg. 1974, 40, 459-472. 16. Compton J, Teddy P. Cerebral arterial vasospasm following severe head injury: a transcranial Doppler study. Br J Neurosurg. 1987, 1, 435-439. 17. Steiger H. Pathophysiology of development and rupture of cerebral aneurysms. Acta Neurochir Suppl. 1990, 48, 1-57. 18. Mast H, Mohr J, Thompson J et al. Transcranial Doppler Ultrasonography in Cerebral Arteriovenous Malformations. Diagnostic Sensitivity and Association of Flow Velocity With Spontaneous Hemorrhage and Focal Neurological Deficit. Stroke. 1995, 26, 1024-27.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- Ö. LJÒË΂‡ ‰.Ï. äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl åÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇“ 1527 ëÓÙËfl , ÛÎ. ◊ÅflÎÓ ÏÓ“8 íÂÎ. 9432/597

éÔËÒ‡ÌË ̇ ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë LOCKED-IN ëàçÑêéå èêà èÄñàÖçí ë ÑéãàïéÖäíÄáàü çÄ ÅÄáàãÄêçÄíÄ ÄêíÖêàü à. ëÚ‡ÈÍÓ‚, E. LJ‚ÂÍ, ñ. ñ‡ÌÍÓ‚‡, è. ëÚ‡ÏÂÌÓ‚‡, äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” , „.ëÓÙËfl SUMMARY LOCKED-IN SYNDROME IN PATIENT WITH A DOLICHOECTASIA OF THE BASILAR ARTERY I.Staikov, E.Vavrek, Tz.Tzankova, P.Stamenova The vertebrobasilar dolichoectasia is a rare vascular anomaly, represented as elongation, widening and pathological tortousity of the vertebral and basilar arteries. The locked-in syndrome develops mostly after a thrombosis of the basilar artery which affects mainly the ventral part of the pons. The compression of n.facialis and n.trigeminus is one of the most common clinical manifestations of the basilar dolichoectasia. The combination of the basilar dolichoectasia and the thrombosis of basilar artery is very rare. We present a case of a 66 old man with a trigeminal neuralgia, a peripheral lesion of n.facialis and development of a locked-in syndrome after a thrombosis of the dolichoectatic basilar artery. Recanalisation of the basilar artery and essential improvement of the clinical symptomatic was achieved after a treatment with 24 000 E Heparin i.v. Key words: megadolichobasilaris, thrombosis, trigeminal neuralgia, locked-in syndrome. êÖáûåÖ ÇÂÚ·Ó·‡ÁË·̇ڇ ‰ÓÎËıÓÂÍÚ‡ÁËfl  fl‰Í‡ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl, Ô‰ÒÚ‡‚Îfl‚‡˘‡ Û‰˙Îʇ‚‡ÌÂ, ‡Á¯Ëfl‚‡ÌÂ Ë Ô‡ÚÓÎӄ˘̇ ̇„˙̇ÚÓÒÚ Ì‡ ‚ÂÚ·‡ÎÌËÚÂ Ë ·‡ÁË·̇ ‡ÚÂËË. Locked-in ÒË̉ÓÏ ‚˙ÁÌËÍ‚‡ ̇È-˜ÂÒÚÓ ÔË ÚÓÏ·ÓÁ‡ ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl Ò˙Ò Á‡Òfl„‡Ì Ô‰ËÏÌÓ Ì‡ ‚ÂÌÚ‡Î̇ڇ ˜‡ÒÚ Ì‡ ÏÓÒÚ‡. ։̇ ÓÚ Ì‡È-˜ÂÒÚËÚ ÍÎËÌ˘ÌË ËÁfl‚Ë ÔË ‰ÓÎËıÓÂÍÚ‡ÁËfl ̇ ‡ÚÂËfl ·‡ÁË·ËÒ Â ÍÓÏÔÂÒËfl ̇ V Ë VII óåç. ë˙˜ÂÚ‡ÌËÂÚÓ Ì‡ ‰ÓÎËıÓÂÍÚ‡ÁËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl Ë ÚÓÏ·ÓÁ‡ ̇ Ò˙˘‡Ú‡  ڂ˙‰Â fl‰ÍÓ. è‰ÒÚ‡‚flÏ ÒÎÛ˜‡È ̇ 66 „Ӊ˯ÂÌ Ï˙Ê Ò ÚË„ÂÏË̇Î̇ Ì‚‡Î„Ëfl, ÎÂÁËfl ̇ VII óåç ÓÚ ÔÂËÙÂÂÌ ÚËÔ Ë ‡Á‚ËÚË ̇ locked-in ÒË̉ÓÏ ÒΉ ÚÓÏ·ÓÁË‡Ì ̇ ‰ÓÎËıÓÂÍÚ‡Ú˘̇ ·‡ÁË·̇ ‡ÚÂËfl. ëΉ ΘÂÌËÂ Ò 24 000 E Heparin ËÌÚ‡‚ÂÌÓÁÌÓ Ò ÔÓÒÚ˄̇ Â͇̇ÎËÁ‡ˆËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl Ë Ò˙˘ÂÒÚ‚ÂÌÓ ÔÓ‰Ó·ÂÌË ‚ ÒËÏÔÚÓχÚË͇ڇ ̇ Ô‡ˆËÂÌÚ‡. äβ˜Ó‚Ë ‰ÛÏË: Ï„‡‰ÓÎËıÓ·‡ÁË·ËÒ, ÚÓÏ·ÓÁ‡, ÚË„ÂÏË̇Î̇ Ì‚‡Î„Ëfl, locked-in ÒË̉ÓÏ. ìÇéÑ ÇÂÚ·Ó·‡ÁË·̇ڇ ‰ÓÎËıÓÂÍÚ‡ÁËfl  fl‰Í‡ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl, Ô‰ÒÚ‡‚Îfl‚‡˘‡ Û‰˙Îʇ‚‡ÌÂ, ‡Á¯Ëfl‚‡Ì Ë

Ô‡ÚÓÎӄ˘̇ ̇„˙̇ÚÓÒÚ Ì‡ ‚ÂÚ·‡ÎÌËÚÂ Ë ·‡ÁË·̇ ‡ÚÂËË (3, 14). ÑÓÎËıÓÂÍÚ‡ÁËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl (ÅÄ) ËÎË Ï„‡‰ÓÎËıÓ·‡ÁË·ËÒ (åÑÅ) Ò ‰Ë‡„ÌÓÒÚˈË‡, ÍÓ„‡ÚÓ ÌÂÈÌËflÚ ‰Ë‡ÏÂÚ˙  ̇‰ 4.5 ÏÏ (ÂÍÚ‡ÁËfl) Ë ÍÓ„‡ÚÓ ·‡ÁË·̇ڇ ‡ÚÂËfl ÎÂÊË Î‡ÚÂ‡ÎÌÓ ÓÚ ÒÚ‡Ì˘ÌËfl Í‡È ̇ clivus ËÎË dorsum sellae ËÎË Ì‡‰ ÌË‚ÓÚÓ Ì‡ ÒÛÔ‡Ò·̇ڇ ˆËÒÚÂ̇ (ÂÎÓÌ„‡ˆËfl) (17,18). Locked-in ÒË̉ÓÏ ‚˙ÁÌËÍ‚‡ ̇È-˜ÂÒÚÓ ÔË ÚÓÏ·ÓÁ‡ ̇ ÅÄ Ò˙Ò Á‡Òfl„‡Ì Ô‰ËÏÌÓ Ì‡ ‚ÂÌÚ‡Î̇ڇ ˜‡ÒÚ Ì‡ ÏÓÒÚ‡. èË ÚÓÏ·ÓÁË‡ÌÂÚÓ Ë ÒÏ˙ÚÌÓÒÚÚ‡ ‰ÓÒÚË„‡ 90% ÔË ÎËÔÒ‡ ̇ Â͇̇ÎËÁ‡ˆËfl Ë 50% ÔË Â͇̇ÎËÁ‡ˆËfl (7). äãàçàóÖç ëãìóÄâ 66 „Ӊ˯ÂÌ Ô‡ˆËÂÌÚ Ò ‡Ì‡ÏÌÂÁ‡ Á‡ ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl (Äï)  ÔËÂÚ ‚ ÍÎËÌË͇ڇ Ò ‰Ë‡„ÌÓÁ‡ ÚË„ÂÏË̇Î̇ Ì‚‡Î„Ëfl ‚ Îfl‚Ó. éÔ·͂‡ÌËflÚ‡ ̇ Ô‡ˆËÂÌÚ‡ Á‡ÔÓ˜‚‡Ú ÔÂ‰Ë 4 „Ó‰ËÌË Ë 10 ÏÂÒˆ‡ Ò Ì‚‡Î„Ëfl ̇ V óåç ‚Îfl‚Ó, ÎÂÍÛ‚‡Ì Ò ÔÓÏÂÌÎË‚ ÛÒÔÂı Ò carbamazepin Ë baclofen. íË ÏÂÒˆ‡ ÔÓÍ˙ÒÌÓ Ò ‡Á‚Ë‚‡ ÔÂËÙÂ̇ ÎÂÁËfl ̇ n.facialis ‚ ‰flÒÌÓ Ò ‰Ó·Ó ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ÒΉ ΘÂÌËÂ. óÂÚËË „Ó‰ËÌË ÔÓÍ˙ÒÌÓ ÎÂÁËflÚ‡ ˆˉ˂Ë‡ Ë ÔÂÒËÒÚË‡ ÔË ÔËÂχÌÂÚÓ. 臈ËÂÌÚ˙Ú ·Â¯Â ıÓÒÔËÚ‡ÎËÁË‡Ì ‚ ÍÎËÌË͇ڇ ÔÓ ÔÓ‚Ó‰ Á‡˜ÂÒÚfl‚‡ÌÂ Ë ÛÚÂÊÌfl‚‡Ì ̇ Ì‚‡Î„˘ÌËÚ ÓÔ·͂‡ÌËfl ‰Ó Ì‚˙ÁÏÓÊÌÓÒÚ ‰‡ ÔÓÂχ ı‡Ì‡ Ë Ú˜ÌÓÒÚË ÔÂÁ ÛÒÚ‡Ú‡. ç‡ ‚ÚÓËfl ‰ÂÌ ÓÚ ÔÓÒÚ˙Ô‚‡ÌÂÚÓ ÔË ·ÓÎÌËfl Ò ‡Á‚Ë ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (àåà) ‚˙‚ ‚ÂÚ·Ó·‡ÁË·̇ڇ ÒËÒÚÂχ (ÇÅë) Ò ˆÂÌÚ‡Î̇ Í‚‡‰ËÔ΄Ëfl, ·˙ÁÓ Á‡‰˙ηӘ‡‚‡˘Ó Ò ÍÓ΢ÂÒÚ‚ÂÌÓ Ì‡Û¯ÂÌË ̇ Ò˙Á̇ÌËÂÚÓ, ÓÒÚÓ ‡Á‚ËÚË ̇ ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. ÅÓÎÌËflÚ ·Â¯Â ËÌÚÛ·Ë‡Ì ÓÓÚ‡ı‡ÎÌÓ, ÔÓÒÚ‡‚ÂÌ Ì‡ ËÁÍÛÒÚ‚Â̇ ·ÂÎÓ‰ӷ̇ ‚ÂÌÚË·ˆËfl – ÂÊËÏ IPPV, ‡ ‚ ÔÓÒΉÒÚ‚ËÂ Ë Ú‡ıÂÓÚÓÏË‡Ì. èÓÒÚ‡‚Ë Ò ̇ÁÓ„‡ÒÚ‡Î̇ ÒÓ̉‡. á‡ÔӘ̇ ҠΘÂÌËÂ Ò Heparin – ‚ Ô˙‚ËÚ ‰ÌË 24 000 Ö ‰Ì‚ÌÓ Ì‡ ÔÂÙÛÁÓ (1000 Ö/ ˜‡Ò). ëΉ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ Ò˙Á̇ÌËÂÚÓ ÍÓÌÚ‡ÍÚ˙Ú Ò ·ÓÎÌËfl ·Â¯Â ‚˙ÁÏÓÊÂÌ Â‰ËÌÒÚ‚ÂÌÓ ÔÓÒ‰ÒÚ‚ÓÏ ‚ÂÚË͇ÎÌË Ó˜ÌË ‰‚ËÊÂÌËfl. Ñˇ„ÌÓÒÚˈË‡ Ò locked-in ÒË̉ÓÏ. ë˙ÒÚÓflÌËÂÚÓ Ì‡ ·ÓÎÌËfl ÔÓÒÚÂÔÂÌÌÓ Ò ÔÓ‰Ó·Ë. 臈ËÂÌÚ˙Ú ‡Á‰‚ËÊË Î‚ËÚ Í‡ÈÌˈË, ‚ ‰ÂÒÌËÚ Í‡ÈÌËˆË ÓÒڇ̇ ÛÏÂÂÌÓ ËÁ‡ÁÂ̇ Ô‡ÂÁ‡, ̇Ô˙ÎÌÓ ‚˙ÁÒÚ‡ÌÓ‚Ë ‰Ë¯‡ÌÂÚÓ Ë ·Â¯Â ÓÒÚ‡‚ÂÌ Ì‡ ÒÔÓÌÚ‡ÌÌÓ ‰Ë¯‡ÌÂ. ÅÓÎÌËflÚ Ì ‚˙ÁÒÚ‡ÌÓ‚Ë Ì‡Ô˙ÎÌÓ „˙ÎÚ‡ÌÂÚÓ ÒË Ë ÓÒڇ̇ Ò Ì‡ÁÓ„‡ÒÚ‡Î̇ ÒÓ̉‡.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

îË„.1 è‡Ì‡Ì„ËÓ„‡ÙËfl ÔÓ Seldinger. ÇËÁÛ‡ÎËÁË‡Ú Ò ͇ÓÚˉÌËÚ ‡ÚÂËË, ‰flÒ̇ ‚ÂÚ·‡Î̇ ‡ÚÂËfl; Ì Ò ‚ËÁÛ‡ÎËÁË‡ Îfl‚‡ ‚ÂÚ·‡Î̇ ‡ÚÂËfl.

182

îË„.3‡

îË„. 2. äíÄ. Ö‰ËÌÒÚ‚Â̇ڇ ‰flÒ̇ ‚ÂÚ·‡Î̇ ‡ÚÂËfl ÔÂÏË̇‚‡ ‚ ‰ÓÎËıÓÂÍÚ‡Ú˘̇ ·‡ÁË·̇ ‡ÚÂËfl (2‡), ÍÓflÚÓ ‚ ÔÓÒΉÒÚ‚Ë ÔÂÏË̇‚‡ ‚ Îfl‚Ó (2·). îË„.2‡. - ÇËÁÛÎËÁË‡ Ò ‰ËÌÒÚ‚ÂÌ Ò˙‰ ‚ ‰flÒÌÓ ÓÚ Ò‰ËÌ̇ڇ ÎËÌËfl. îË„.3·. üåÄ. ÇËÁÛ‡ÎËÁË‡Ú Ò ËÁÏÂÌÂÌËflÚ ÇËÎËÁË‚ Í˙„. ç Ò ÓÚÍË‚‡ Îfl‚‡ ‚ÂÚ·‡Î̇ ‡ÚÂËfl. èӂ‰Óı‡ Ò ÒΉÌËÚ ËÁÒΉ‚‡ÌËfl: ● äÓÏÔ˛Ú˙̇ ‡ÍÒˇÎ̇ ÚÓÏÓ„‡ÙËfl (äÄí) ̇ „·‚‡ ‰‡ÌÌË Á‡ ‚˙Ú¯̇ Ë ‚˙̯̇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ë ÏÛÎÚËËÌÙ‡ÍÚ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ● è‡Ì‡Ì„ËÓ„‡ÙËfl ÔÓ Seldinger - ÔÓ‡‰Ë ËÁ‡ÁÂ̇ڇ ̇„˙̇ÚÓÒÚ Ì‡ ‚ÂÚ·‡ÎÌËÚ ‡ÚÂËË Ò ‚ËÁÛ‡ÎËÁË‡ Ò‡ÏÓ Í‡ÓÚˉ̇ڇ ÒËÒÚÂχ, ͇ÚÓ ‰ÓÒÚ˙Ô˙Ú ‰Ó ÇÅë Ì ·Â¯Â ‚˙ÁÏÓÊÂÌ ● äí ‡Ì„ËÓ„‡ÙËfl - åÑÅ ÔÓÂÍÚË‡˘‡ Ò Ô˙‚Ó ‚ ‰flÒÌÓ, ‡ ‚ Í‡ÌˇÎ̇ ÔÓÒÓ͇ ÔÂÏË̇‚‡˘‡ ̇ Îfl‚Ó ● ÑÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl (ÑëÉ) – ‰‡ÌÌË Á‡ Â͇̇ÎËÁ‡ˆËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl ● ü‰ÂÌÓ-χ„ÌËÚ̇ ‡Ì„ËÓ„‡ÙËfl – ‰‡ÌÌË Á‡ ‡Ô·ÁËfl ̇ Îfl‚‡Ú‡ ‚ÂÚ·‡Î̇ ‡ÚÂËfl, ÒËÎÌÓ Ì‡„˙̇ڇ ‰flÒ̇ ‚ÂÚ·‡Î̇ ‡ÚÂËfl, ͇ÍÚÓ Ë ‰‡ÌÌË Á‡ ‰ÓÎËıÓÂÍÚ‡ÁËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl. îË„.2·. - ÇËÁÛ‡ÎËÁË‡ ÔÓÒÓ˜Â̇ Ò˙Ò ÒÚÂÎ͇ ÂÎËÔÒӂˉ̇ ÔÓÂ͈Ëfl ̇ ‡ÚÂËfl ·‡ÁË·ËÒ ‚ Îfl‚Ó ÓÚ Ò‰ËÌ̇ڇ ÎËÌËfl, ·ÚÂ‡ÎÌÓ ÓÚ ÍÎË‚ÛÒ‡, Ò ÏËÌËχÎÂÌ ‰Ë‡ÏÂÚ˙ ̇ Ú‡ÁË ÔÓÂ͈Ëfl 5 ÏÏ


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

éÅëöÜÑÄçÖ ÑÓÎËıÓÂÍÚ‡ÁËflÚ‡ ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl Ò Ò¢‡ fl‰ÍÓ. èÓÒÔÂÍÚË‚ÌÓ ÔÓÛ˜‚‡ÌÂ Ò fl‰ÂÌÓχ„ÌËÚÂÌ ÂÁÓ̇ÌÒ ÔÓ͇Á‚‡, ˜Â Ò‡ÏÓ ‚ 0.91% ÓÚ ‚Ò˘ÍË ËÁÒΉ‚‡ÌË Ò ̇ÏË‡Ú ‰ÓÎËıÓÂÍÚ‡Ú˘ÌË ËÌÚ‡Í‡ÌˇÎÌË ‡ÚÂËË. Ñ‚‡Ï‡ ÓÚ ‚ÒÂÍË ÚËχ Ô‡ˆËÂÌÚË ÔÓ͇Á‚‡Ú ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇ (15). è‡ÚÓÎÓ„ËflÚ‡  ÔÓ-˜ÂÒÚ‡ ÔË Ï˙ÊÂ, ͇ÚÓ Ò‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ÍÎËÌ˘ÌËÚ ÔÓfl‚Ë Â ÓÍÓÎÓ 55 „Ó‰ËÌË (15). Ç ÓÍÓÎÓ ? ÓÚ ÒÎÛ˜‡ËÚ Ò ÓÚÍË‚‡ ‡Ì‡ÏÌÂÁ‡ Á‡ Äï Ë ‡ÚÂÓÒÍÎÂÓÚ˘ÌË ÔÓÏÂÌË (14,15). 燷≇‚‡ Ò Ôӂ˯Â̇ ˜ÂÒÚÓÚ‡ ̇ ÙÛÁËÙÓÏÂÌË ‰Ë·ڇˆËË Ì‡ ‰Û„Ë Í‡ÌˇÎÌË ‡ÚÂËË, Ôӂ˯Â̇ ˜ÂÒÚÓÚ‡ ̇ ‡ÚÂˇÎÌË ‡Ì‚ËÁÏË (1,14). ë˙Ó·˘‡‚‡ Ò Á‡ ‡ÒӈˇˆËfl Ò ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚ̇ ·˙·˜̇ ÔÓÎËÍËÒÚÓÁ‡, ͇ÚÓ ÚÂÁË Ì‡·Î˛‰ÂÌËfl ̇ÒÓ˜‚‡Ú Í˙Ï ‚˙ÁÏÓÊÌÓ „ÂÌÂÚ˘ÌÓ Ì‡Û¯ÂÌË ‚ ÍÓ·„ÂÌÓ‚Ëfl ÒËÌÚÂÁ (8,14,16). Ç ÔÓ‰ÍÂÔ‡ ̇ Ú‡ÁË ıËÔÓÚÂÁ‡ Ò‡ Ë ÓÔËÒ‡ÌËÚ هÏËÎÌË ÒÎÛ˜‡Ë, ͇ÍÚÓ Ë ‡Á‚ËÚËÂÚÓ Ì‡ Ô‡ÚÓÎÓ„ËflÚ‡ ‚˙‚ ‚Ò˘ÍË ‚˙Á‡ÒÚË (4,5,10,13,22). ïËÒÚÓÎӄ˘ÌÓ Ò ̇·Î˛‰‡‚‡ Ô‡ÚÓÎÓ„Ëfl ̇ membrana elastica interna, ÍÓflÚÓ Ò ÔÓÏÂÌfl Ò˙˘Ó Ë ÔË ‰ËÒÂÍË‡˘ËÚÂ Ë ÙÛÁËÙÓÏÂÌË ‡Ì‚ËÁÏË (1,12,13). åÂı‡ÌËÁÏËÚÂ, ÔÓ ÍÓËÚÓ ‚˙ÁÌËÍ‚‡Ú ÍÎËÌ˘ÌËÚ ÓÔ·͂‡ÌËfl Ò‡ ‰‚‡ ÚËÔ‡ – ÔËÚËÒ͇Ì ̇ ÒÚÛÍÚÛË ÓÚ Û‰˙ÎÊÂ̇ڇ, ‡Á¯ËÂ̇ Ë Ì‡„˙̇ڇ ‡ÚÂËfl Ë ÒÚ‚ÓÎÓ‚Ë àåà ͇ÚÓ ÒΉÒÚ‚Ë ÓÚ Á‡·‡‚flÌÂÚÓ Ë ÚÛ·ÛÎÂÌÚÌÓÒÚÚ‡ ̇ Í˙‚ÓÚÓ͇ ‚ ÔÓÏÂÌÂ̇ڇ ‡ÚÂËfl (13). äÎËÌ˘̇ڇ ͇ÚË̇  ڂ˙‰Â ‡ÁÌÓÓ·‡Á̇: ● äÓÏÔÂÒËfl ̇ ˜ÂÂÔÌÓÏÓÁ˙˜ÌËÚ ÌÂ‚Ë (óåç), ͇ÚÓ Ì‡È-˜ÂÒÚÓ Ò Á‡Òfl„‡Ú VII Ë V óåç. ● äÓÏÔÂÒËfl ̇ ÏÓÁ˙˜ÌËfl ÒÚ‚ÓÎ Ò ÔË‡Ïˉ̇ ËÎË Ï‡ÎÍÓÏÓÁ˙˜Ì‡ ÒËÏÔÚÓχÚË͇ ● ïˉÓˆÂÙ‡ÎËfl ÔÓ‡‰Ë ÔËÚËÒ͇Ì ̇ ÔÓ‰‡ ̇ III ÏÓÁ˙˜ÂÌ ‚ÂÌÚËÍÛÎ Ë ÙÓ‡ÏÂÌ åÓÌÓ (1,2,18, 21,22) ● àåà ‡Á‚ËÎË Ò ÔË ÎÓ͇Î̇ ÂÏ·ÓÎËÁ‡ˆËfl, ‡ÚÂÓÒÍÎÂÓÚ˘ÌË ÔÓÏÂÌË, Ó·ÒÚÛ͈Ëfl ̇ Ô‡‡Ï‰ˇÌÌËÚÂ Ë ÔÓÌÚËÌÌËÚ ‡ÚÂËË, ÚÓÏ·ÓÁË (1). íÓÏ·ÓÁ‡ Á‡ a.basilaris Ò ‡Á‚ËÚË ̇ locked in ÒË̉ÓÏ Â Ú‚˙‰Â fl‰Í‡ Ô‡ÚÓÎÓ„Ëfl ÔË åÑÅ (11). èË Ôӂ‰ÂÌÓÚÓ ÓÚ Passero et al. ËÁÒΉ‚‡Ì ̇ 40 Ô‡ˆËÂÌÚË Ò àåà ÔË åÑÅ Ì Ò ÓÚÍË‚‡ ÚÓÏ·ÓÁË‡Ì ̇ ÅÄ. ìÒÚ‡ÌÓ‚fl‚‡ ÒÂ, ˜Â ‡Á‚ËÚËÂÚÓ Ì‡ àåà ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ÓÎËıÓÂÍÚ‡ÁËfl ̇ ÅÄ ÍÓÂÎË‡ Ò ‡ÚÂÓÒÍÎÂÓÚ˘ÌËÚ ËÁÏÂÌÂÌËfl ‚˙‚ ÇÅë. ë˙˘Ó ڇ͇ ‚ËÒÓÍÓÒÚÂÔÂÌ̇ڇ ÂÍÚ‡ÁËfl Ë Û‰˙Îʇ‚‡Ì ̇ ÅÄ Ò ҂˙Á‚‡Ú ÔÓ-˜ÂÒÚÓ Ò àåà ‚ Á‡‰Ì‡ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl. (13) ● êfl‰ÍÓ Ò ÓÔËÒ‚‡Ú Ò˙·‡‡ıÌÓˉ‡ÎÌË Í˙‚ÓËÁÎË‚Ë, ̇ÒÚ˙ÔËÎË ‚ÒΉÒÚ‚Ë ̇ Ò˙Ô˙ÚÒÚ‚‡˘Ë ÏÓÁ˙˜ÌË ‡Ì‚ËÁÏË, ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl, ıËÔÂÚÓÌËfl, ÏË„ÂÌÓ-ÔÓ‰Ó·ÌÓ „·‚Ó·ÓÎË ËÎË „·‚Ó·ÓÎË ÔË Ì‡ÔÂÊÂÌË (1,9,20,22,23). Ñˇ„ÌÓÁ‡Ú‡ Ò ÔÓÒÚ‡‚fl ‚˙Á ÓÒÌÓ‚‡ ̇ ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ Ë ˜ÂÁ ÍÓÌ‚Â̈ËÓ̇Î̇- , äí- ËÎË fl‰ÂÌÓχ„ÌËÚ̇ ‡Ì„ËÓ„‡ÙËfl. ã˜ÂÌËÂÚÓ ‚Íβ˜‚‡ ÏËÍÓ‚‡ÒÍÛ·̇ ‰ÂÍÓÏÔÂÒËfl ÔË ÔËÚËÒ͇Ì ̇È-˜ÂÒÚÓ Ì‡ V óåç, ͇ÍÚÓ Ë ÒËÏÔÚÓχÚ˘ÌÓ ÍÓÌÒÂ‚‡ÚË‚ÌÓ Î˜ÂÌËÂ. (6,19). èË Ì‡¯Ëfl Ô‡ˆËÂÌÚ Ò ̇·Î˛‰‡‚‡ Ò˙˜ÂÚ‡ÌË ÏÂÊ‰Û Â‰Ì‡ ÓÚ Ì‡È-˜ÂÒÚËÚ ËÁfl‚Ë Ì‡ åÑÅ – ÚË„ÂÏË̇Î̇ڇ Ì‚‡Î„Ëfl - Ë Â‰ÌÓ ÓÚ ‰ÍËÚ ÛÒÎÓÊÌÂÌËfl ̇ ‰ÓÎËıÓÂÍÚ‡ÁËflÚ‡ – ÚÓÏ·ÓÁ‡ ̇ ÅÄ (5,13). Ç˙ÁÏÓÊÌÓ Â Â‰Ì‡ ıÂÏÓÍÓ̈ÂÌÚ‡ˆËfl ÔË Ì‡Î˘̇ڇ ‰ÓÎËıÓÂÍÚ‡ÁËfl ̇ ·‡ÁË·̇ڇ ‡ÚÂËfl (ÅÄ) ‰‡  ÛÎÂÒÌË· ‡Á‚ËÚËÂÚÓ Ì‡ ÚÓÏ·ÓÁ‡Ú‡. Å˙ÁÓÚÓ ‡Á‚ËÚË ̇ ÚÂÊ˙Í ÒÚ‚ÓÎÓ‚ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ Ì ‰‡‰Â ‚˙ÁÏÓÊÌÓÒÚ Á‡ Ô‰‚‡ËÚÂÎÌÓÚÓ ÔÓÚ‚˙ʉ‡‚‡Ì ̇ åÑÅ Ò ‡Ì„ËÓ„‡ÙÍÓ ËÁÒΉ‚‡ÌÂ. èӂ‰Â̇ڇ äÄí Ò ÍÓÌÚ‡ÒÚ ‰Ó͇Á‡ ̇΢ËÂÚÓ Ì‡ ‚ÂÚ·Ó·‡ÁË-

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·̇ ‰ÓÎËıÓÂÍÚ‡ÁËfl. ÑëÉ ÓÚ˜ÂÚ ÔÓÏÂÌËÚ ‚ Ò˙‰Ó‚Ëfl ÒÚ‡ÚÛÒ, ‚Íβ˜ËÚÂÎÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ Í˙‚ÓÚÓ͇ ‚ ÅÄ, ÍÓËÚÓ ÍÓÂÎË‡ı‡ Ò Â‚ÓβˆËflÚ‡ ̇ Ì‚ÓÎӄ˘̇ڇ Ô‡ÚÓÎÓ„Ëfl. èË Ì‡¯Ëfl Ô‡ˆËÂÌÚ Ì ÛÒÚ‡ÌÓ‚ËıÏ ‰‡ÌÌË Á‡ Ù‡ÏËÎÌÓÒÚ. ä‡ÚÓ ËÒÍÓ‚ Ù‡ÍÚÓ ÏÓÊ ‰‡ Ò ÓÚ·ÂÎÂÊË Äï. èӂ‰ÂÌËÚ ‡Ì„ËÓ„‡ÙÒÍË ËÁÒΉ‚‡ÌËfl ÓÚı‚˙ÎËı‡ ÓÔËÒ‚‡ÌËÚ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ò˙˜ÂÚ‡ÌËfl Ò ‡Ì‚ËÁÏË Ì‡ ÏÓÁ˙˜ÌËÚ ‡ÚÂËË. ãàíÖêÄíìêÄ 1. ëÚ‡ÈÍÓ‚, à., å‡ÚÎÂ, ï. ë˙‚ÂÏÂÌÌË ‡ÒÔÂÍÚË Ì‡ ‚ÂÚ·Ó·‡ÁË·̇ڇ ‰ÓÎËıÓÂÍÚ‡ÁËfl: ‡Ì‡ÚÓÏ˘ÌË ÓÒÓ·ÂÌÓÒÚË, Ô‡ÚÓ„ÂÌÂÁ‡, ÍÎËÌË͇, ‰Ë‡„ÌÓÁ‡. åÓÁ˙˜ÌÓ Ò˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl, 1, 1995, 30-32. 2. Aiba,T., Nakazawa, T. Non-communicating hydrocephalus due to megadolichobasilar artery-case report. Neurol.Med.Chir, 35(2), 1995, 104-106. 3. Aichner F., Felber, S., Birbamer, G., Posch, A. Magnetic resonance imaging and magnetic resonance angiography of vertebrobasilar dolichoectasia. Cerebrovascular Diseases, 3, 1993, 280-284. 4. Barbosa , ER., da Costa Mdo, D., Staut, CC., Bacheschi, LA., Bittar, MS. Familial hemifacial spasm: report of 2 cases. Arq.Neuropsiquiatr, 56(1), 1998, 111-115. 5. Besson, G., Bogouslavsky, J., Moulin, T., Hommel, M. Vertebrobasilar infarcts in patients with dolichoectatic basilar artery. Acta Neurol Scand, 91(1), 1995, 37-42. 6. Cavallari, F., Feres, M., Isac, M., Olivera, J. Tinnitus in a patient with dolichomegabasilar: a case report. RBORL, 70(2), 2004 250-253. 7. Erfahrungen, E. CT angiography in basilar artery thrombosis. Initial experiences. Radiologe, 36(11), 1996, 855-858. 8. Graf, S., Schischma, A., Eberhardt, K., Istel, R., Stiasny, B, Schulze B. Intracranial aneurysm and dolichoectasia in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant, 17, 2002, 819-823. 9. Ishikawa, T., Nagayama, M., Iida, M., Shinohara, Y. Bulbovascular compression by megadolichobasilar artery manifested as a neurogenic and refractory hypertension. Rinsho Shinkeigaku, 44(6), 2004, 359-364. 10. Johnsen, SD., Okamoto, G., Kooiker, J. Fusiform basilar artery aneurism in a child. Neurology, 27(4), 1977, 334-336. 11. Krasnianski, M., Gaul, C., Neudeker, S., Behmann, C., Schluter, Winterholler, M. Yawning despite trismus in a patient with locked-in syndrome caused by a thrombosed megadolichobasilar artery. Clin Neurol Neurosurg, 106(1), 2003, 44-46. 12. Mizutani, T., Kojima, H. Clinicopathological features of non-atherosclerotic cerebral arterial trunk aneurism. Neuropathology, 20(1), 2000, 91. 13. Passero, S., Filosomi, G. Posterior Circulation Infarcts in Patients With Vertebrobasilar Dolichoectasia. Stroke, 29, 1998, 653-659. 14. Salbeck, R., Busse, O., Reinbold, W-D. MRI findings in mega-dolicho-basilar artery. Cerebrovascular Diseases, 3, 1993, 309-312. 15. Schwartz, A., Rautenberg, W., Hennerici, M. Dolichoectatic intracranial arteries: Review of selected aspects. Cerebrovascular diseases, 3, 1993, 273-279. 16. Sessa, A. Autosomal Dominant Polycystic Kidney Disease. Karger, S.Ink, 1995, 182-184. 17. Smoker, W., Price, M., Keyes, W., Corbett, J., Gentry, L. High-resolution computed tomography of the basilar artery: 1. Normal Size and Position. AJNR, 7, 1986, 55-60. 18. Smoker, W., Corbett, J., Gentry, L., Keyes, W., Price, M., Mc Kusker, S. High-resolution computed tomography of the basilar artery: 2. Vertebrobasilar dolichoectasia: clinical pathologic correlation and review. AJNR, 7, 1986, 61-72. 19. Sola, R., Bag.., M. Microvascular decompression of trigeminal neuralgia caused by vertebrobasilar dolichoectasia. Rev Neurol, 32(8), 2001 20. Staikov, I., Mattle, H. Vertebrobasilar dolichoectasia and exertional headache. J.Neurol.Neurosurg. Psych., 57, 1994, 1554. 21. Weber, MA., Steiner, T., Fiebach, J. Unusual cause of acute impairment of consciousness. Decompensated hydrocephalus caused by blockage of the foramini Monroi due to enlarged basilar artery. Radiologe, 42(10), 2002, 833-836 22. Yu, Y., Moseley, I., Pollicino, P., McDonald, W. The clinical picture of ectasia of the intracerebral arteries. J.Neurol.Neusurg.Psych., 45,1982, 29-36. 23. Zambrino, CA., Berardinelli, A., Martelli, A., Vercelli, P., Termine, C., Lanzi, G. Dolicho-Vertebrobasilar Abnormality and Migraine-Like Attacks. European Neurology, 41(1), 1999, 10-14.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ‰- à‚‡Ì ëÚ‡ÈÍÓ‚, ‰.Ï. É·‚ÂÌ ‡ÒËÒÚÂÌÚ Í˙Ï äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” ÛÎ. ÅflÎÓ ÏÓ 8 1527 ëÓÙËfl íÂÎ: 9432 571 Ö-mail: ivanstaikov@hotmail.com


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

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éÔËÒ‡ÌË ̇ ÍÎËÌ˘ÌË ÒÎÛ˜‡Ë ëìÅäãàçàóçà êàíåàóçà ÖãÖäíéÉêÄîàóçà êÄáêüÑà Ç áêüãÄ ÇöáêÄëí (SREDA) - éèàëÄçàÖ çÄ ÑÇÄ ëãìóÄü å. ꇯ‚‡, Ö. LJÒË΂‡ ç‚ÓÎӄ˘̇ ÍÎËÌË͇ - åÅÄã "ñ‡ˈ‡ âÓ‡Ì̇" ä‡Ú‰‡ ÔÓ ç‚ÓÎÓ„Ëfl - ëÓÙËfl SUMMARY SUBCLINICAL RHYTHMIC ELECTROGRAPHIC DISCHARGE OF ADULTS (SREDA) IN TWO PATIENTS M. Rascheva, E. Vasileva Subclinical rhythmic electrographic discharge of adults (SREDA) was first described by Westmoreland in 1981 as a pseudoepileptogenic pattern. It consists of mono- or bi-phasic sharp waves followed by rhythmic theta waves occurring in a widespread manner, maximal over the parietal and posterior temporal regions, and lasting for a few seconds to a minute without clinical manifestations or symptoms. This article reports this EEG pattern in two patients with follow-up period of 2 years. Two patients tried to be treated with antiepileptic drugs on the basis of their EEG, despite they have never had unprovoked epileptic seizures. In one of the patients Doppler sonography discovered microembolic signals with cardiac origin in a.carotis interna and a. cerebri media. SREDA cannot be considered as an epileptic pattern and may be related in some cases to transient hypoxic/ischemic mechanisms. Key words: SREDA, pseudoepileptogenic pattern, EEG, nonepileptic paroxismal activity, Doppler sonography, microembolic signals, brain ischemia. êÖáûåÖ ëÛ·ÍÎËÌ˘ÌËÚ ËÚÏ˘ÌË ÂÎÍÚÓ„‡Ù˘ÌË ‡Áfl‰Ë (SREDA) Ò‡ ÓÔËÒ‡ÌË Á‡ Ô˙‚Ë Ô˙Ú ÔÂÁ 1981 „. ÓÚ Westmoreland ͇ÚÓ ÔÒ‚‰ÓÂÔËÎÂÔÚ˘̇ ̇ıӉ͇. íÂÁË ‡Áfl‰Ë Ò Ò˙ÒÚÓflÚ ÓÚ ÏÓÌÓÙ‡ÁÌË ËÎË ‰‚ÛÙ‡ÁÌË ÓÒÚË ‚˙ÎÌË, ÔÓÒΉ‚‡ÌË ÓÚ ËÚÏ˘‡ ÚÂÚ‡ ‡ÍÚË‚ÌÓÒÚ, ÔÓ‰˙Îʇ‚‡˘Ë ÓÚ ÌflÍÓÎÍÓ ÒÂÍÛÌ‰Ë ‰Ó ÏËÌÛÚ‡, ·ÂÁ Ò˙Ô˙ÚÒÚ‚‡˘Ë ÍÎËÌ˘ÌË ÔÓfl‚Ë ËÎË ÒËÏÔÚÓÏË. Ç Ò˙Ó·˘ÂÌËÂÚÓ Ò ÓÔËÒ‚‡Ú ‰‚‡ ÒÎÛ˜‡fl Ò ÚÓÁË ÖÖÉ Ô‡ÚÂÌ Ò ÔÓÒΉfl‚‡Ì Á‡ ÔÂËÓ‰ ÓÚ 2 „. à ÔË ‰‚‡Ï‡Ú‡ Ô‡ˆËÂÌÚË Â ÔÓ·‚‡ÌÓ ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓ Î˜ÂÌË ̇ ·‡Á‡Ú‡ ̇ ̇·Î˛‰‡‚‡ÌËÚ ÖÖÉ ÔÓÏÂÌË, ‚˙ÔÂÍË ˜Â Ú Ì ҇ ËχÎË ÌÂÔÓ‚ÓÍË‡ÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË. èË Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚ ÑÓÔÎÂÓ‚‡Ú‡ ÒÓÌÓ„‡ÙËfl ‰ÂÏÓÌÒÚË‡ ÏËÍÓÂÏ·Ó΢ÌË Ò˄̇ÎË ÓÚ Ò˙‰Â˜ÂÌ ÔÓËÁıÓ‰ ‚ a.carotis interna Ë ‚ a.cerebri media. SREDA Ì ‰‡ Ò ‡Á„ÎÂʉ‡ ͇ÚÓ ÂÔËÎÂÔÚ˘ÂÌ ÖÖÉ Ô‡ÚÂÌ Ë ÏÓÊ ‰‡ Ò ҂˙Á‚‡ ‚ ÌflÍÓË ÒÎÛ˜‡Ë Ò Ú‡ÌÁËÚÓÌË ıËÔÓÍÒ˘ÌÓ-ËÒıÂÏ˘ÌË ÏÂı‡ÌËÁÏË. äβ˜Ó‚Ë ‰ÛÏË: SREDA, ÔÒ‚‰ÓÂÔËÎÂÔÚ˘ÌË Ô‡ÚÂÌË, ÖÖÉ, ÌÂÂÔËÎÂÔÚ˘̇ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ, ÑÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl, ÏËÍÓÂÏ·Ó΢ÌË Ò˄̇ÎË, ÏÓÁ˙˜Ì‡ ËÒıÂÏËfl. ÖÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙËflÚ‡ (ÖÖÉ)  ÏÂÚÓ‰Ë͇ Ò ‚ËÒÓ͇ ̇‰ÂʉÌÓÒÚ, ‚‡ÎˉÌÓÒÚ Ë ‰Ó „ÓÎflχ ÒÚÂÔÂÌ Ë ÒÔˆËÙ˘ÌÓÒÚ ‚ ‰Ë‡„ÌÓÒÚË͇ڇ ̇ ÂÔËÎÂÔÒËflÚ‡. ë˙˘ÂÒÚ‚Û‚‡Ú, Ó·‡˜Â Ë ÖÖÉ Ô‡ÓÍÒËÁχÎÌË Ì‡ıÓ‰ÍË, ‰ÂÙËÌË‡ÌË Í‡ÚÓ "ÔÒ‚‰ÓÂÔËÎÂÔÚÓ„ÂÌÌË" (2), Ò˙Á‰‡‚‡˘Ë Á̇˜ËÚÂÎÌË ‰ËÙÂÂ̈ˇÎÌÓ-‰Ë‡„ÌÓÒÚ˘ÌË ÔÓ·ÎÂÏË Ò ËÌÚÂËÍÚ‡Î̇ڇ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ. í Ò Ô‰ÒÚ‡‚flÚ ÓÚ ‡ÍÚË‚ÌÓÒÚ, ‚˙ÁÌËÍ‚‡˘‡ ‚ÌÂÁ‡ÔÌÓ, Ò Í‡Ú͇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ, ‡Á΢̇ ÓÚ ÙÓ̇, ÌÓ Ì ‡ÒÓˆËË‡Ú Ò ÍÎËÌ˘ÌË ÔÓfl‚Ë Í‡ÚÓ ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË ËÎË ‰Û„Ë Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË (2, 5, 10).

èÂÁ 1981 „. Á‡ Ô˙‚Ë Ô˙Ú Westmoreland Ë Klass (10) ÓÔËÒ‡ı‡ „ÛÔ‡ ÓÚ Ô‡ˆËÂÌÚË Ò˙Ò ÒÛ·ÍÎËÌ˘ÌË ËÚÏ˘ÌË ÂÎÂÍÚÓ„‡ÙÒÍË ‡Áfl‰Ë ‚ Áfl· ‚˙Á‡ÒÚ (SREDA - subclinical rhithmic electrographic discharge of adults), ͇ÚÓ ÔÓÒÚÂÔÂÌÌÓ ·ÓflÚ Ì‡ Ò˙·‡ÌËÚ ÓÚ ‡‚ÚÓËÚ ÒÎÛ˜‡Ë ‰ÓÒÚ˄̇ 108 (4, 11). äËÚÂËË Á‡ SREDA Ò‡: ● îÓχ ̇ ‚˙ÎÌËÚÂ: ÓÒÚË, ÏÓÌÓÙ‡ÁÌË ËÎË ‰‚ÛÙ‡ÁÌË, ‚ ̇˜‡ÎÓÚÓ Ì‡ ‡Áfl‰‡ ÏÓ„‡Ú ‰‡ Ò‡ ‰ËÌ˘ÌË, ÔÂÁ 1-2 ÒÂÍ, ‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ËÏ ‚‡Ë‡ ÓÚ 150 ‰Ó 300 ÏÒÂÍ. èÓÒΉ‚‡ÌË Ò‡ ÓÚ ËÚÏ˘ÌË ÚÂÚ‡ ‚˙ÎÌË 4 ‰Ó 7 ıˆ (̇ȘÂÒÚÓ 5 ıˆ); ● èÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ‰ËÌ ‡Áfl‰  ÓÚ ÌflÍÓÎÍÓ ÒÂÍ ‰Ó 5 ÏËÌ, Ò‰ÌÓ Ì‡È-˜ÂÒÚÓ ÏÂÊ‰Û 40 Ë 80 ÒÂÍ; ● á‡ÔÓ˜‚‡Ú Ë Á‡‚˙¯‚‡Ú ‚ÌÂÁ‡ÔÌÓ, ÔÓ-fl‰ÍÓ Á‡ ÔÂËÓ‰ ÓÚ ÌflÍÓÎÍÓ ÒÂÍ; ● é·Î‡ÒÚÌÓÚÓ ‡ÁÔ‰ÂÎÂÌË ÔÓ͇Á‚‡ ÒËÏÂÚËfl ‚ ÓÍÓÎÓ 2/3 ÓÚ ÒÎÛ˜‡ËÚÂ, Ò Ï‡ÍÒËχÎ̇ ËÁ‡ÁÂÌÓÒÚ ‚ Á‡‰ÌËÚ ÚÂÏÔÓ‡ÎÌË Ë Ô‡ËÂÚ‡ÎÌËÚ ‡ÈÓÌË, ͇ÚÓ ÔÓ-fl‰ÍÓ Ò ̇·Î˛‰‡‚‡Ú ‡ÒËÏÂÚËfl ËÎË Â‰ÌÓÒÚ‡Ì̇ ÔÓfl‚‡; ● 燷≇‚‡Ú Ò ‚ Áfl· ‚˙Á‡ÒÚ, ÌÓ Ì‡ÔÓÒΉ˙Í Ò‡ ÓÔËÒ‡ÌË Ë ÔË ‰Âˆ‡ (6) ● ê„ËÒÚË‡Ú Ò ÔË ·Û‰ÌÓÒÚ, ÔÓÌflÍÓ„‡ Ò‡ÏÓ ÔË ıËÔÂ‚ÂÌÚË·ˆËfl, ÌÓ Ò˙˘Ó Ë ÔÓ ‚ÂÏ ̇ Ò˙Ì (9), ‚Íβ˜ËÚÂÎÌÓ Ë ‚ REM ÒÚ‡‰ËËÚ ̇ Ò˙Ìfl (3). Ç Ô˙‚ËÚ ÒÚ‡‰ËË Ì‡ ·‡‚ÌÓ‚˙ÎÌÓ‚Ëfl Ò˙Ì ÏÓ„‡Ú ‰‡ ‡ÒÓˆËË‡Ú Ò ‚ÂÚÂÍÒÌË ‚˙ÎÌË Ë Ò˙ÌÌË ‚ÂÚÂ̇ (2); ● ç Ò Ò˙Ô˙ÚÒÚ‚‡Ú ÓÚ ÍÎËÌ˘ÌË ËÍÚ‡ÎÌË ÒËÏÔÚÓÏË; ● ë˙Á̇ÌËÂÚÓ Ë ÍÓ„ÌËÚË‚ÌËÚ ÔÓˆÂÒË ÔÓ ‚ÂÏ ̇ SREDA Ò‡ Á‡Ô‡ÁÂÌË, ÍÓÂÚÓ Ò ‰Ó͇Á‚‡ Ò Ì‚ÓÔÒËıÓÎӄ˘ÌË ÚÂÒÚÓ‚Â ‚ ıÓ‰‡ ̇ ÖÖÉ; ● èÓ ‚ÂÏ ̇ ‰ËÌ ÛÚËÌÂÌ Á‡ÔËÒ ÓÚ ÓÍÓÎÓ 20 ÏËÌ Ò „ËÒÚË‡Ú ÌÂÍÓÎÍÓÍ‡ÚÌÓ (‰Ó 15 Ô˙ÚË); ● èÓ‚Ú‡flÚ ÒÂ Ë ‚ ÔÓÒΉ‚‡˘ËÚ ÖÖÉ ËÁÒΉ‚‡ÌËfl ‚ ÔÓ‰˙ÎÊÂÌË ̇ ÏÌÓ„Ó „Ó‰ËÌË; ● äÓ΢ÂÒÚ‚ÂÌËflÚ ÖÖÉ ‡Ì‡ÎËÁ ÔÓ͇Á‚‡, ˜Â Ò Ò˙ÒÚÓflÚ ÓÚ ÒÎÓÊ̇ ÒÏÂÒ ÓÚ ‡Á΢ÌË ·˙ÁË ˜ÂÒÚÓÚË Ò˙Ò Ò··‡ ÔÓÒÚ‡ÌÒÚ‚Â̇ Ë ‚ÂÏ‚‡ ÍÓ·ˆËfl, Á‡ ‡ÁÎË͇ ÓÚ Ô‡ÓÍÒËÁχÎÌËÚ ÂÔËÎÂÔÚ˘ÌË ‡Áfl‰Ë, ÔË ÍÓËÚÓ Ëχ ‰̇ ‰ÓÏË̇ÌÚ̇, ‰Ó· Ó„‡ÌËÁË‡Ì‡ Ë ËÚÏ˘̇ ˜ÂÒÚÓÚ‡, ÒÚ‡·ËÎ̇ ‚ ÔÓÒÚ‡ÌÒÚ‚ÂÌÓ Ë ‚ÂÏÂ‚Ó ÓÚÌÓ¯ÂÌË (7). êÖáìãíÄíà è‰ÒÚ‡‚flÏ ‰‚‡Ï‡ Ô‡ˆËÂÌÚË, ËÁÒΉ‚‡ÌË ÌÂÍÓÎÍÓÍ‡ÚÌÓ ‡Ï·Û·ÚÓÌÓ, ‡ ‰ËÌflÚ ÓÚ Úflı Ë ·ÓÎÌ˘ÌÓ ‚ ç‚ÓÎӄ˘̇ ÍÎËÌË͇ ̇ åÅÄã "ñ‡ˈ‡ âÓ‡Ì̇", ÔË ÍÓËÚÓ ·Â¯Â „ËÒÚË‡Ì‡ ‡ÍÚË‚ÌÓÒÚ ‚ ÖÖÉ ÓÚ ÚËÔ‡ ̇ SREDA. èöêÇÄ èÄñàÖçíäÄ å·‰‡ ÊÂ̇, Ö.ê.â., 39 „., ËÁÒΉ‚‡Ì‡ ‡Ï·Û·ÚÓÌÓ Ì‡ 18.06.2004 „., ̇ÒÓ˜Â̇ Á‡ ÍÓÌÒÛÎÚ‡ÚË‚ÂÌ Ô„Ή Ë ÖÖÉ ËÁÒΉ‚‡ÌÂ Ò ÍÎËÌ˘̇ ‰Ë‡„ÌÓÁ‡ "åË„Â̇". àχ ‰‡ÌÌË Á‡ ÔÂ͇‡Ì‡ ˜ÂÂÔÌÓÏÓÁ˙˜Ì‡ Ú‡‚χ ÔÂ‰Ë 10 „. Ò˙Ò Á‡„Û·‡ ̇ Ò˙Á̇ÌË Á‡ ÒÂÍÛ̉Ë(?). éÚ ‰ÂÚÂ Â Ò ÂÏÓˆËÓ̇Î̇ ÌÂÒÚ‡·ËÎÌÓÒÚ, ÌÂÓ·ÓÒÌÓ‚‡ÌË ÒÚ‡ıÓ‚Â (ÓÚ Ú˙ÏÌË̇, ÓÚ ÒÂËÓÁÌË ·ÓÎÂÒÚË), ̇Û¯ÂÌËfl ‚ Ò˙Ìfl, ÛÏÓ‡, ˜ÂÒÚË ËÁÔÓÚfl‚‡ÌËfl Ë ·ÂÁÒ˙ÌËÂ. ç  Ëχ· ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË. ëÓχÚ˘ÌËfl ÒÚ‡ÚÛÒ Â ·ÂÁ Á̇˜ËÏË ÓÚÍÎÓÌÂÌËfl Ò ËÁÍβ-


˛ÌË, 2005

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˜ÂÌË ̇ ‡ÚÂˇÎÌÓ Ì‡Îfl„‡Ì 105/70, -92/ÏËÌ, ·Î‰‡ ÍÓʇ, ‚·ÊÌË Ë ÒÚÛ‰ÂÌË ‰Î‡ÌË. Ç Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ Ò˙˘Ó Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÓÚÍÎÓÌÂÌËfl. ëí ̇ ÏÓÁ˙͇  Ì„‡ÚË‚ÂÌ. ä˙‚̇ڇ ͇ÚË̇ Ò˙˘Ó. Ç ÖÖÉ Ò „ËÒÚË‡ ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ ÓÚ „ÛÎflÌË ‡ÎÙ‡ ‚˙ÎÌË 10 ıˆ Ò ‡ÏÔÎËÚÛ‰‡ ‰Ó 40 uV, ͇ÚÓ ÔÓ ‚ÂÏ ̇ 20 ÏËÌ Á‡ÔËÒ Ò‡ ̇·Î˛‰‡‚‡ÌË ÌÂÍÓÎÍÓÍ‡ÚÌÓ Ë̈ˉÂÌÚË ÓÚ SREDA, Á‡ÔÓ˜‚‡˘Ë ‚ÌÂÁ‡ÔÌÓ Ò ÏÓÌÓËÚÏ˘ÌË ‰‚ÛÙ‡ÁÌË ÓÒÚË ‚˙ÎÌË, Ò ‡ÏÔÎËÚÛ‰ÂÌ Ï‡ÍÒËÏÛÏ ‚ Á‡‰ÌÓÚÂÏÔÓ‡ÎÌËÚ ‡ÈÓÌË, Ò ÔÓÒÚÂÔÂÌ̇ ‚ÓβˆËfl ‰Ó ËÚÏ˘̇ ÚÂÚ‡ ‡ÍÚË‚ÌÓÒÚ 5 ıˆ. í‡ÁË ‡ÍÚË‚ÌÓÒÚ Ò ÔÓ‚Ú‡fl 4Í‡ÚÌÓ ÔÓ ‚ÂÏ ̇ Á‡ÔËÒ‡, ͇ÚÓ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ë Â ÓÚ 18 ‰Ó 27 ÒÂÍ Á‡ ‚Òfl͇ ÒÂËfl. èÓ ‚ÂÏ ̇ „ËÒÚ‡ˆËflÚ‡ Ë, Ô‡ˆËÂÌÚ͇ڇ  ‡‰ÂÍ‚‡Ú̇, ÓÚ„Ó‚‡fl ̇ Á‡‰‡‰ÂÌËÚ ‚˙ÔÓÒË, ËÁ‚˙¯‚‡ ‡ËÚÏÂÚ˘ÌË ‰ÂÈÒÚ‚Ëfl ÔÓ ‚ÂÏ ̇ ‡Áfl‰ËÚ (îË„. 1, 2 Ë 3), ͇ÚÓ ÛÏÒÚ‚Â̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì ÔÂÍ‡Úfl‚‡ ÚÓÁË ÂÎÂÍÚ˘ÂÒÍË ÙÂÌÓÏÂÌ. 臈ËÂÌÚ͇ڇ Ì Ò˙Ó·˘‡‚‡ Ë Á‡ ‡ÁÎËÍË ‚˙‚ ‚˙ÁÔËflÚ̇ڇ ÒÙÂ‡ ÔÓ ‚ÂÏ ̇ SREDA ËÎË ÏÂÊ‰Û ‡Áfl‰ËÚÂ. 臈ËÂÌÚ͇ڇ Ì  ÔÓ‚Âʉ‡Î‡ ‡ÌÚËÂÔËÎÂÔÚ˘̇ ÚÂ‡ÔËfl, ‚˙ÔÂÍË ˜Â ÔÂ‰Ë ÌflÍÓÎÍÓ „Ó‰ËÌË Ë Â Ô‰·„‡Ì‡ ̇ÒÚÓflÚÂÎÌÓ Ú‡Í‡‚‡ Ò ‡„ÛÏÂÌÚË, ˜Â Á‡ÔËÒ˙Ú Ë Â Ò ÂÔËÎÂÔÚ˘ÂÌ ı‡‡ÍÚÂ Ë Â ÏÌÓ„Ó ‚ÂÓflÚÌÓ ‚ ·ÎËÁÍËÚ ÏÂÒÂˆË ‰‡ Ò ÔÓfl‚flÚ ÂÔËÎÂÔÚ˘ÌË „˙˜Ó‚Â. Çíéêà èÄñàÖçí å˙Ê Ì‡ 64 „., í.ë.É., Ò ÚËÍ‡ÚÌË Ì‚ÓÎӄ˘ÌË ÍÓÌÒÛÎÚ‡ˆËË Ë ÖÖÉ ËÁÒΉ‚‡ÌËfl - ̇ 22.03.2002 „., ̇ 20.10.2003 „. Ë Ì‡ 01.12.2004 „. Ñˇ„ÌÓÁ‡Ú‡  "ê‚χÚËÁ˙Ï- Ò˙‰Â˜ÌÓ-ÒÚ‡‚̇ ÙÓχ - ÒÚÂÌÓÁ‡ Ë ËÌÒÛÙˈËÂ̈Ëfl ̇ ÏËÚ‡Î̇ڇ Ë ‡ÓÚ̇ Í·ԇ. ÅËÓÔÓÚÂÁ‡ AVR ÔÂÁ 1980 „., ÂÓÔÂ‡ˆËfl (ReAVR) ÔÂÁ 1987 „. Ë by pass Ò˙˘Ó ÔÂÁ 1987 „. è‰Ò˙‰ÌÓ Ï˙ʉÂÌÂ. ÄÚÂˇÎ̇ ıËÔÂÚÓÌËfl. èÂ͇‡Ì ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‚ ·‡ÒÂÈ̇ ̇ ‰flÒ̇ڇ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl. åÛÎÚËËÌÙ‡ÍÚ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl". ç‡ 24.02.2002 „. Ô‡ˆËÂÌÚ˙Ú Â ËχΠÓÒÚÓ Ì‡Û¯ÂÌË ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ, ÔˆÂÌÂÌÓ Í‡ÚÓ ÚÓÏ·ÓÂÏ·Ó΢ÂÌ ËÌÒÛÎÚ, ÔÓ ‚ÂÏ ̇ ÍÓÈÚÓ Â Ì‡Ô‡‚ËÎ

îË„.1. ëÛ·ÍÎËÌ˘ÂÌ ËÚÏ˘ÂÌ ‡Áfl‰ Ò ‡ÏÔÎËÚÛ‰ÂÌ Ï‡ÍÒËÏÛÏ ‚ Á‡‰ÌÓÚÂÏÔÓ‡ÎÌËÚ ÓÚ‚Âʉ‡ÌËfl Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ 21 ÒÂÍ. ÔË Ô˙‚‡Ú‡ Ô‡ˆËÂÌÚ͇. ‰ÌÓÍ‡ÚÂÌ Í‡ÚÍÓÚ‡ÂÌ „ÂÌÂ‡ÎËÁË‡Ì ÚÓÌ˘ÌÓ-ÍÎÓÌ˘ÂÌ „˙˜. 臈ËÂÌÚ˙Ú ÓÚ ‰˙Î„Ë „Ó‰ËÌË Â Ì‡ ‡ÌÚËÍÓ‡„Û·ÌÚ̇ ÚÂ‡ÔËfl Ò˙Ò Sintrom, ͇ÚÓ ‚˙ÔÂÍË ÚÓ‚‡ Á‡ 1 ÏÂÒˆ ÒΉ ËÌÒÛÎÚ‡  ÔÓÎÛ˜‡‚‡Î Ë Carbamazepine ‚ ‰ÓÁË 200 Ï„/‚˜Â. àχΠ ‚ÔÓÒΉÒÚ‚Ë ÌflÍÓÎÍÓ Ú‡ÌÁËÚÓÌË Ë̈ˉÂÌÚ‡ ̇ ‰ËÔÎÓÔËfl, ‡Ú‡ÍÒËfl, ÛÒÚ‡ÌÓ‚fl‚‡Ì‡ Í‚‡‰ËÔË‡Ïˉ̇ ÒËÏÔÚÓχÚË͇, ÓˆÂÌfl‚‡ÌË Í‡ÚÓ ÔÂıÓ‰ÌË Ì‡Û¯ÂÌËfl ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌË ‚˙‚ ‚ÂÚ·Ó·‡ÁË·̇ڇ ÒËÒÚÂχ. ëÓχÚ˘ÌËflÚ ÒÚ‡ÚÛÒ ÔÓ͇Á‚‡ ‡·ÒÓβÚ̇ ‡ËÚÏËfl ÔË Ô‰Ò˙‰ÌÓ Ï˙ʉÂÌÂ, ·ÂÁ ÔÛÎÒÓ‚ ‰ÂÙˈËÚ, Äç 145/90, ÌÂÛ‚Â΢ÂÌ ˜ÂÂÌ ‰Ó·, ÎËÔÒ‡ ̇ ÓÚÓ-

îË„.2. èÓ‚ÚÓÂÌ ÂÎÂÍÚ˘ÂÒÍË ‡Áfl‰ Ò ‚ÌÂÁ‡ÔÌÓ Ì‡˜‡ÎÓ, Ò ‡ÍӂˉÂÌ ı‡‡ÍÚÂ ̇ ÚÂÚ‡ ‚˙ÎÌËÚÂ, Ò ‡ÏÔÎËÚÛ‰ÂÌ Ï‡ÍÒËÏÛÏ ·ËÚÂÏÔÓ‡ÎÌÓ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ SREDA 14 ÒÂÍ.


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îË„.3. SREDA Ò ÔӉӷ̇ ı‡‡ÍÚÂËÒÚË͇ ̇ Ô‰ıÓ‰ÌËfl Ë̈ˉÂÌÚ, ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ 18 ÒÂÍ. Ë ÚÛÍ, ͇ÍÚÓ Ë ‚ ÓÒڇ̇ÎËÚ ÒÎÛ˜‡Ë Ô‡ˆËÂÌÚ͇ڇ Â Ò Ô˙ÎÂÌ ÍÓÌÚ‡ÍÚ. ˆË, ‡ Ì‚ÓÎӄ˘ÌËflÚ - ΂ÓÒÚ‡Ì̇ ÔË‡Ïˉ̇ ÒËÏÔÚÓχÚË͇. äí ̇ ÏÓÁ˙͇ - ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl, ÔÓ-ËÁ‡ÁÂ̇ ‚ ‰flÒ̇ڇ í-ê ӷ·ÒÚ. ÖäÉ Ë ÍÓÌÒÛÎÚ‡ˆËfl Ò Í‡‰ËÓÎÓ„ ‰‡‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌË ‰‡ÌÌË Á‡ Îfl‚ ·Â‰ÂÌ ·ÎÓÍ, ÔÂËÓ‰Ë Ì‡ ‚ÎËÁ‡Ì ‚ ËÚ˙Ï, ̇΢ˠ̇ ͇ÏÂÌË ÂÍÒÚ‡ÒËÒÚÓÎË. Ç Ô˙‚‡Ú‡ ÖÖÉ, ‰ËÌ ÏÂÒˆ ÒΉ ÔÂ͇‡ÌËfl ËÌÒÛÎÚ, Á‡ Ô˙‚Ë Ô˙Ú Ò‡ ̇·Î˛‰‡‚‡ÌË SREDA. í ҇ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ ÓÚ 20 ‰Ó 35 ÒÂÍ ÔË Á‡Ô‡ÁÂ̇ ÙÓÌÓ‚‡ ‡ÎÙ‡ ‡ÍÚË‚ÌÓÒÚ. Ç ÍÓÌÚÓÎÌÓÚÓ ÖÖÉ ËÁÒΉ‚‡Ì ÓÚ 2003 (îË„. 4 Ë

5) Ë 2004 „. ÖÖÉ Ò‡ Ò˙Ò Ò˙˘‡Ú‡ ı‡‡ÍÚÂËÒÚË͇. ê„ËÒÚË‡Ú Ò 4 Ë̈ˉÂÌÚ‡ ÔÓ ‚ÂÏ ̇ 20 ÏËÌ ‚ÚÓË Á‡ÔËÒ, Ë 5 ÔÓ ‚ÂÏ ̇ ÚÂÚËfl, ͇ÚÓ Ô‡ˆËÂÌÚ˙Ú Â Ò Ô˙ÎÂÌ ÍÓÌÚ‡ÍÚ ÔÓ ‚ÂÏ ̇ ‡Áfl‰ËÚÂ Ë ËÁ‚˙¯‚‡ Ì‚ÓÔÒËıÓÎӄ˘ÌËÚ ÚÂÒÚÓ‚Â. Ç ÔÓÒΉÌÓÚÓ ÖÖÉ ËÁÒΉ‚‡Ì SREDA Ò ÔÓ‚Ú‡flÚ Ú‚˙‰Â „ÛÎflÌÓ ÔÂÁ 2,5 - 3 ÏËÌ.(îË„.6). óÂÒÚÓÚ‡Ú‡ ̇ ÏÓÌÓËÚÏ˘ÌËÚÂ, ‡ÁÔÓÒÚ‡Ìfl‚‡˘Ë Ò ÓÚ Á‡‰ÌËÚ ÚÂÏÔÓ‡ÎÌË Ó·Î‡ÒÚË ·‡‚ÌË ‚˙ÎÌË Â Ò‡‚ÌËÚÂÎÌÓ ÌËÒ͇ - 3-4 ıˆ, ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ËÏ Â ÔÓ-„ÓÎfl-

îË„.4. èË ‚ÚÓËfl Ô‡ˆËÂÌÚ - SREDA Ò Ì‡˜‡ÎÓ ÓÚ ÓÒÚË ‚˙ÎÌË ·ËÚÂÏÔÓ‡ÎÌÓ, ÔÓÒΉ‚‡ÌË ÓÚ ÏÓÌÓËÚÏ˘ÌË, „ÛÎflÌË, ‚ËÒÓÍÓ‡ÏÔÎËÚÛ‰ÌË ·‡‚ÌË ‚˙ÎÌË 3,5-4 ıˆ, Ò ÔÓ-‚ËÒÓ͇ ‡ÏÔÎËÚÛ‰‡ ‚ Îfl‚‡Ú‡ ë-í ӷ·ÒÚ, Ò ‡ÁÔÓÒÚ‡ÌÂÌËÂ Ë ‚˙‚ F ÓÚ‚Âʉ‡ÌËfl, ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ - 20 ÒÂÍ.


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îË„.5. SREDA ÔÓ‰Ó·ÂÌ Ì‡ Ô‰¯ÂÒÚ‚‡˘Ëfl, Ô‡ˆËÂÌÚ˙Ú ÓÚ„Ó‚‡fl ̇ ‚˙ÔÓÒËÚÂ, ÓÚ‚‡fl Ó˜Ë, ¯‡‚‡ Á‡‰‡˜Ë. χ Ë ‚‡Ë‡ ÓÚ 40 ÒÂÍ ‰Ó 2 ÏËÌ, ‡ÏÔÎËÚÛ‰‡Ú‡  ÔÓ-‚ËÒÓ͇ ‚ Îfl‚‡Ú‡ ÚÂÏÔÓ‡Î̇ ӷ·ÒÚ. çÂÔÓÒ‰ÒÚ‚ÂÌÓ ÒΉ ÖÖÉ Â ËÁ‚˙¯Â̇ ÑÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl - ÂÍÒÚ‡ Ë Ú‡ÌÒÍ‡ÌˇÎ̇ Ë Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÏËÍÓÂÏ·Ó΢ÌË Ò˄̇ÎË Ò ÔË·ÎËÁËÚÂÎÌÓ Ò˙˘‡Ú‡ ˜ÂÒÚÓÚ‡, ÔÂÁ 2-3 ÏËÌ, ÔÓ-˜ÂÒÚË ‚ Îfl‚‡Ú‡ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl Ë ‚ Îfl‚‡Ú‡ ‚˙Ú¯̇ Ò˙Ì̇ ‡ÚÂËfl (îË„.7). 臈ËÂÌÚ˙Ú Ì  ËχΠÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË ‚ ÔÓ‰˙ÎÊÂÌË ̇ Ôӂ˜ ÓÚ 2 „Ó‰ËÌË, ÓÒ‚ÂÌ Â‰ÌÓÍ‡ÚÌËfl ÔÓ ‚ÂÏ ̇ ËÌÒÛÎÚ‡. ꇷÓÚË ‡ÍÚË‚ÌÓ, ͇ÚÓ ÔÂÔÓ‰‡‚‡ ‚˙‚ Çìá. éÅëöÜÑÄçÖ èË ÓÔËÒ‡ÌËÚ ÓÚ Ì‡Ò Ô‡ˆËÂÌÚË ÌÂÒ˙ÏÌÂÌÓ Ò ͇҇ Á‡

îË„.6. SREDA Ò Ì‡·ÂÎflÁ‡Ì‡ ·ÚÂ‡ÎËÁ‡ˆËfl ‚ Îfl‚Ó Ò ‡ÁÔÓÒÚ‡ÌÂÌË ·ËÙÓÌÚ‡ÎÌÓ Ë ˆÂÌÚ‡ÎÌÓ, Ò „ÓÎflχ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ, ͇ÚÓ Ô‡ˆËÂÌÚ˙Ú Â Ò Ô˙ÎÂÌ ÍÓÌÚ‡ÍÚ Ë ·ÂÁ ÍÎËÌ˘ÌË ÒËÏÔÚÓÏË.

ÒÎÛ˜‡Ë Ò ÌÂÂÔËÎÂÔÚ˘̇ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ Ò ı‡‡ÍÚÂËÒÚË͇ ̇ SREDA - ÔË Ô˙‚‡Ú‡ Ô‡ˆËÂÌÚ͇ Ò ÚËÔ˘ÌËfl Ô‡ÚÂÌ Ì‡ ÚÓÁË ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙÒÍË ÒË̉ÓÏ, ‡ ÔË ‚ÚÓËfl Ô‡ˆËÂÌÚ Ò ˜ÂÚË Ì‡ ‰ËÌ ÓÚ ‚‡ˇÌÚËÚ ÏÛ - Ò ÔÓ-ÌËÒÍË ˜ÂÒÚÓÚË, Ò Ì‡·ÂÎflÁ‡Ì‡ ·ÚÂ‡ÎËÁ‡ˆËfl Ë Ò ÔÓ-„ÓÎflχ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ (8, 11). çÂÚËÔ˘ÌË SREDA ÒÔÓ‰ Westmoreland Ë Ò˙‡‚Ú. (5, 11) Ò „ËÒÚË‡Ú ÔË 1/5 ÓÚ ÒÎÛ˜‡ËÚÂ. íÂÁË ÖÖÉ Ô‡ÚÂÌË Ò˙Á‰‡‚‡Ú ‰ËÙÂÂ̈ˇÎÌӉˇ„ÌÓÒÚ˘ÌË ÔÓ·ÎÂÏË Ò ËÌÚÂËÍÚ‡Î̇ ÂÔËÎÂÔÚ˘̇ ‡ÍÚË‚ÌÓÒÚ. à ÔË ‰‚‡Ï‡Ú‡ Ô‡ˆËÂÌÚË Â ·Ë· Ó·Ò˙ʉ‡Ì‡ ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ÂÔËÎÂÔÒËfl Ë ÔË Â‰Ì‡Ú‡ Ô‡ˆËÂÌÚ͇  Ô‰·„‡ÌÓ, ‡ ÔË ‚ÚÓËfl Ô‡ˆËÂÌÚ Ë Ì‡Á̇˜‡‚‡ÌÓ ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓ Î˜ÂÌËÂ. Ç ÎËÚÂ‡ÚÛ‡Ú‡ Ú‡ÁË ÖÖÉ Ì‡ıӉ͇  ҂˙Á‚‡Ì‡ Ò ÔÓ-˜ÂÒÚË Ì‡Û¯ÂÌËfl ̇ ‚„ÂÚ‡Ú˂̇ڇ ÌÂ‚̇ ÒËÒÚÂχ, Ò ˜ÂÒÚË ÒËÌÍÓÔË, „·‚Ó·ÓÎËÂ, Ò‚ÂÚÓ‚˙ÚÂÊ, Ó·ÒÚÛÍÚ˂̇ Ò˙Ì̇ ‡ÔÌÂfl, ΢ÌÓÒÚÓ‚Ó-ı‡‡ÍÚÂÓ‚Ë ÔÓÏÂÌË Ë ÔÓ-ÒÂËÓÁ̇ ÔÒËıÓÔ‡ÚÓÎÓ„Ëfl (1, 2, 3, 9). åÂı‡ÌËÁÏËÚ ̇ SREDA Ò‡ ÌÂËÁflÒÌÂÌË, ÓÚ̇Òfl Ò Í˙Ï ‰Ó·Ó͇˜ÂÒÚ‚ÂÌËÚ ÖÖÉ ÙÂÌÓÏÂÌË Ò Ó„‡Ì˘ÂÌÓ ‰Ë‡„ÌÓÒÚ˘ÌÓ Á̇˜ÂÌËÂ Ë Úfl·‚‡ ‰‡ Ò ‡Á΢‡‚‡ ÓÚ ÂÔËÎÂÔÚ˘ÌËÚ ‡Áfl‰Ë (11). èӂ‰Â̇ SPECT ÔÓ ‚ÂÏ ̇ SREDA, Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ Ë ÔÓ‰‡‚‡Ì ̇ äÎÓ̇ÁÂÔ‡Ï ËÌÚ‡‚ÂÌÓÁÌÓ, ‰Ó͇Á‚‡Ú ˜Â Ú‡ÁË Ì‡ıӉ͇  ‡Á΢̇ ÓÚ Ô‡ÓÍÒËÁχÎ̇ڇ ÂÔËÎÂÔÚ˘̇ ‡ÍÚË‚ÌÓÒÚ Ë ·Ë Ïӄ· ‰‡ Ò ҂˙ÊÂ Ò ıÓÌ˘ÂÌ ıËÔÓÍÒ˘ÌÓ-ËÒıÂÏ˘ÂÌ ÏÂı‡ÌËÁ˙Ï (8). êÂÒÔË‡ÚÓ̇ ÌÂÒÚ‡·ËÎÌÓÒÚ Ò ˆËÍ΢ÂÌ ‡ÎÚÂÌË‡˘ ÚËÔ Ì‡ Ó·ÒÚÛÍÚ˂̇ Ò˙Ì̇ ‡ÔÌÂfl , ‡ÒÓˆËË‡˘ Ò ÚÓÁË ÚËÔ ÖÖÉ, Ò˙˘Ó  ‚ ÔÓ‰ÍÂÔ‡ ̇ ıËÔÓÍÒ˘ÌËfl ÏÂı‡ÌËÁ˙Ï ‚ ÌflÍÓË ÓÚ ÒÎÛ˜‡ËÚ (9). èË Ì‡·Î˛‰‡‚‡ÌËfl Ë ÏÌÓ„ÓÍ‡ÚÌÓ ËÁÒΉ‚‡Ì ÓÚ Ì‡Ò Ô‡ˆËÂÌÚ SREDA ÏÓ„‡Ú ‰‡ Ò ӷflÒÌflÚ Ò ÚÓÁË ÏÂı‡ÌËÁ˙Ï, ͇ÚÓ ÔÓÒΉÒÚ‚Ë ÓÚ ÏËÍÓÂÏ·Ó΢ÌËÚ ÒÛ·ÍÎËÌ˘ÌË Ë̈ˉÂÌÚË „ËÒÚË‡ÌË ÑÓÔÎÂÓÒÓÌÓ„‡ÙÒÍË. èË Ô‡ˆËÂÌÚ͇ڇ Ò ÏË„Â̇ ÚÓÁË ÏÂı‡ÌËÁ˙Ï Ò˙˘Ó  ‚˙ÁÏÓÊÂÌ - ÔÓ ËÁÍβ˜ÂÌË ҇ÏÓ Ô‡ˆËÂÌÚË Ò Í·Ò˘ÂÒ͇ ÏË„Â̇ ÌflÏ‡Ú Ì‡Û¯ÂÌËfl ‚ ÏÓÁ˙˜ÌËfl Í˙‚ÓÚÓÍ, ‡ „ËÓ̇Î̇ڇ ËÒıÂÏËfl  Ô˘Ë̇ Á‡ ÙÓ͇ÎÌËÚ ÒËÏÔÚÓÏË ‚ ÚÂÁË ÒÎÛ˜‡Ë.


˛ÌË, 2005

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology

188

îË„.7. ÑÓÔÎÂÓ‚‡ ëÉ - flÒÌÓ Ò ‚Ëʉ‡Ú ÏËÍÓÂÏ·Ó΢ÌËÚ Ò˄̇ÎË ‚ Îfl‚‡Ú‡ Ò‰̇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl. ãàíÖêÄíìêÄ 1. Drury,I., Klass D W, Westmoreland B F, Sharbrough F W.An acute syndrome with psychotic symptoms and EEG abnormalities. Arch Neurol, 35, 1985, 911-914. 2. Fisch B I. Epileptiform patterns without proven relation to seizures. In: Spehlmanns EEG primer,Second edition, 1991, Elsevier, Amsterdam, New York, Oxford, 384-402. 3. Fleming W E, Avidan A, Malow B A. SREDA in REM sleep. Sleep Med., 5, 2004, 77-81. 4. Klass D W, Westmoreland B F. Nonepileptogenic EEG activity. Ann Neurol, 18, 1985, 627-635. 5. Miller C R, Westmoreland B F, Klass D W. SREDA: further observations. Am J EEG Technol, 25, 1985, 217-224. 6. Nagarajan L, Gregory P B, Hewitt I K, Gubbay S S, Parry T S. SREDA in two children. Pediatr Neurol.,24, 2001, 313-316. 7. O Brien T J, Sharbrough F W, Westmoreland B F, Busacker N E. SREDA revisited: a study using digital EEG analysis. J Clin Neurophysiol.,15, 1998, 493501. 8. Thomas P, Migneco O, Darcourt J., Chatel M. SPECT study of SREDA. Electroencephalogr Clin Neurophysiol.,83, 1992, 223-227. 9. Thomas R J, Terzano M G, Parrino L, Weiss J W. Obstructive sleep-disordered breathing with a dominant cyclic alternating pattern - a recognizable polysomnographic variant with practical clinical implications. Sleep., 27, 2004,

229-234. 10. Westmoreland B F, Klass D W. A distinctive rhithmic EEG discharge of adults. EEG clin Neurophisiol.,51, 1981, 186-191. 11. Westmoreland B F, Klass D W. Unusual variants of SREDA. Electroenceph Clin Neurophisiol., 102, 1997, 1-4.

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