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Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology ë‰ÛÊÂÌË ◊Å˙΄‡ÒÍÓ ‰ÛÊÂÒÚ‚Ó ÔÓ Ì‚ÓÎÓ„Ëfl“ Official Journal of The Bulgarian Society of Neurology êÖÑÄäñàéççÄ äéãÖÉàü

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ÄÎÂÍÒË‚ Ä. ŇΉ‡‡ÌÓ‚ Ñ. ÅÓÊËÌÓ‚ ëÚ. ÅÓÊËÌÓ‚‡ Ç Ç‡ÒË΂‡ Ö. LJÒË΂‡ í. ɇÌ‚‡ É. ÉÂÓ„Ë‚ Ñ. ÉÂ‡ÒËÏÓ‚ Å. ÉÓÁχÌÓ‚ É. ÉË„ÓÓ‚‡ é. ÑÂ΂‡ ç. á‡ı‡Ë‚ á. äÓ΂ é. äÓ΂ è. å‡Ì˜Â‚ à. å‡Ò·Ó‚ Ñ. åË·ÌÓ‚ à. åË·ÌÓ‚‡ å. åË̘‚ Ñ. èÂÚÓ‚ à. èÂÚÓ‚‡ û. ê‡È˜Â‚ à. ê‡È˜Â‚‡ å. ëÚ‡ÏÂÌÓ‚ Å. íËÚflÌÓ‚‡ Ö. í‡ÈÍÓ‚ ã. í˙Ì‚ à. ÂÁÓ‚‡ ã. ï‡‡Î‡ÌÓ‚ ã. ó‡Î˙ÍÓ‚‡ ç. óÂÌËÌÍÓ‚‡ ë. òÓÚÂÍÓ‚ è. ü̘‚‡ ë.

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

EDITORS Alexiev A. Baldaranov D. Bojinov St. Bojinoba V. Vassileva E. Vassileva T. Ganeva G. Georgiev D. Gerassimov B. Gozmanov G. Grigorova O. Deleva N. Zahariev Z. Kolev O. Kolev P. Manchev I. Maslarov D. Milanov I. Milanova M. Minchev D. Petrov I. Petrova U. Raychev I. Raycheva M. Stamenov B. Titianova E. Traykov L. Tarnev I. Havezova L. Haralanov L. Chalakova N. Charninkova S. Shotekov P. Yancheva S.

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

ÉãÄÇçà êÖÑÄäíéêà: à. åË·ÌÓ‚, è. ëÚ‡ÏÂÌÓ‚‡, à. ÇÂΘ‚‡

EDITOR IN CHIEF: I. Milanov, P. Stamenova, I. Velcheva

ëÖäêÖíÄê: å. чÒ͇ÎÓ‚

SECRETARY: M. Daskalov


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

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl

 ÓÙˈˇÎÂÌ Ó„‡Ì ̇ ë‰ÛÊÂÌË ◊Å˙΄‡ÒÍÓ ‰ÛÊÂÒÚ‚Ó ÔÓ Ì‚ÓÎÓ„Ëfl“ Ë ÔÛ·ÎËÍÛ‚‡ ÒÚ‡ÚËË ÓÚ ‚Ò˘ÍË Ó·Î‡ÒÚË Ì‡ Ì‚ÓÎÓ„ËflÚ‡. ëÔËÒ‡ÌËÂÚÓ Ò˙‰˙ʇ ÒΉÌËÚ Û·ËÍË: ● ꉇ͈ËÓÌ̇ ÒÚ‡ÚËfl Ò ÚÂÍÒÚ ‰Ó3 ÒÚ‡ÌˈË. Ç˙Á·„‡ Ò ÓÚ ê‰ÍÓ΄ËflÚ‡. ● éË„Ë̇ÎÌË ÒÚ‡ÚËË - ‰Ó 8 ÒÚ‡ÌˈË, ‚Íβ˜ËÚÂÎÌÓ Ú‡·ÎˈË, ÙË„ÛË, ÍÌË„ÓÔËÒ. ä˙Ï ÚÂÁË ÒÚ‡ÚËË Ò ËÁËÒÍ‚‡ ÂÁ˛Ï ̇ ·˙΄‡ÒÍË Ë ‡Ì„ÎËÈÒÍË ÂÁËÍ Ó·˘Ó ‰Ó 40 ‰‡, ÓÚÔ˜‡Ú‡ÌË Ì‡ ÓÚ‰ÂÎÌË ÒÚ‡ÌˈË. ● êÂÁ˛ÏÂÚÓ Úfl·‚‡ ‰‡ Ò˙‰˙ʇ Á‡„·‚ËÂ, ËÏÂ̇ڇ ̇ ‡‚ÚÓËÚÂ, ËÌÙÓχˆËfl Á‡ ˆÂÎÚ‡ Ë Ó·ÂÍÚ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ, ÏÂÚÓ‰ËÍËÚÂ, ÔÓÎÛ˜ÂÌËÚ ÂÁÛÎÚ‡ÚË. èÓÒÓ˜‚‡Ú Ò ‰Ó 6 Íβ˜Ó‚Ë ‰ÛÏË. ● éË„Ë̇ÎÌËÚ ÒÚ‡ÚËË ‚Íβ˜‚‡Ú Í‡Ú˙Í Û‚Ó‰, ÍÓÌÚËÌ„ÂÌÚ, ÏÂÚÓ‰Ë, ÂÁÛÎÚ‡ÚË, Ó·Ò˙ʉ‡ÌÂ Ë ÍÌË„ÓÔËÒ. ● ᇄ·‚̇ڇ ÒÚ‡Ìˈ‡ Ò˙‰˙ʇ Ô˙ÎÌÓ Ë Ò˙Í‡ÚÂÌÓ Á‡„·‚ËÂ, ËÏÂ̇ڇ ̇ ‡‚ÚÓËÚÂ Ò ËÌˈˇÎËÚ ËÏ, ÚÂıÌËÚ ‡Í‡‰ÂÏ˘ÌË ÒÚÂÔÂÌË Ë ÏÂÒÚÓ‡·ÓÚ‡, ‡‰ÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl Ò ÚÂÎÂÙÓÌ, Ù‡ÍÒ Ë Â-mail. ● ä‡ÚÍË Ì‡Û˜ÌË Ò˙Ó·˘ÂÌËfl ‰Ó 3 ÒÚ‡ÌËˆË ● é·ÁÓÌË ÒÚ‡ÚËË ‰Ó 10 ÒÚ‡ÌËˆË ● àÌÙÓχˆËË Ë ˆÂÌÁËË Ì‡ ÍÌË„Ë . ë˙‰˙ʇ ËÌÙÓχˆËfl Á‡ ÍÓÌ„ÂÒË Ë ÍÓÌÙÂÂ̈ËË, ÌÓ‚Ë ÍÌË„Ë, ‡ÍÚÛ‡Î̇ ÎÂ͇ÒÚ‚Â̇ ËÌÙÓχˆËfl, Ô‰ÒÚÓfl˘Ë Ò˙·ËÚËfl. ● ëÚ‡ÌËˆË Ì‡ ˜ËÚ‡ÚÂÎfl. èÓÏÂÒÚ‚‡Ú Ò ÔËÒχ ‰Ó ‰‡ÍÚÓ‡, ‚ ÍÓËÚÓ Ò ÍÓÏÂÌÚË‡Ú ÔÛ·ÎËÍÛ‚‡ÌË Ï‡ÚÂˇÎË, Í‡ÚÍË ÓÔËÒ‡ÌËfl ̇ ÒÎÛ˜‡Ë Ë ‰ËÒÍÛÒËË ÔÓ ‡ÍÚÛ‡ÎÌË ÔÓ·ÎÂÏË . ● ëÚ‡ÚËËÚ Úfl·‚‡ ‰‡ ·˙‰‡Ú Ô‰ÒÚ‡‚ÂÌË Ì‡ CD/USB, Á‡ÔËÒ‡ÌË Ì‡ ‰‡ÍÚÓÒ͇ ÔÓ„‡Ï‡ Word 6/Windows 96 ËÎË Windows Microsoft Word 97/2000XP, Ò Â‰ËÌ ÂÍÁÂÏÔÎfl ‡ÁÔ˜‡Ú͇. ● 퇷ÎˈËÚ Ò Ô‰ÒÚ‡‚flÚ Ì‡ ÓÚ‰ÂÎÂÌ ÎËÒÚ, ÌÓÏÂË‡ÌË, ͇ÚÓ ‚ ÚÂÍÒÚ‡ Ò ÓÚ·ÂÎÂÊË ÏflÒÚÓÚÓ ËÏ. 퇷ÎˈËÚ ‰‡ ËÏ‡Ú Í‡ÚÍÓ Á‡„·‚ËÂ. ● àβÒÚ‡ˆËËÚ (ÙË„ÛË, ‰Ë‡„‡ÏË, ÙÓÏÛÎË) Úfl·‚‡ ‰‡ Ò‡ „ÓÚÓ‚Ë Á‡ ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ ÔÓÎË„‡Ù˘ÌÓ ‚˙ÁÔÓËÁ‚Âʉ‡ÌÂ. íÂÍÒÚÓ‚ÂÚ ÔÓ‰ ÙË„ÛËÚ Ò Ô‰ÒÚ‡‚flÚ Ì‡ ÓÚ‰ÂÎÂÌ ÎËÒÚ ● äÌË„ÓÔËÒ˙Ú ‰‡ Ò ÓÚÔ˜‡Ú‡ ̇ ÓÚ‰ÂÎÂÌ ÎËÒÚ. Ä‚ÚÓËÚ ‰‡ Ò ÔÓ‰Âʉ‡Ú ÔÓ ‡Á·Û˜ÂÌ ‰, ͇ÚÓ ‚ ̇˜‡ÎÓÚÓ Ò ËÁ·Ófl‚‡Ú ËÁÚÓ˜ÌˈËÚ ̇ ÍËËÎˈ‡, ‡ ÒΉ Úflı ̇ ·ÚËÌˈ‡. ᇄ·‚ËflÚ‡ ‰‡ Ò Ô‰ÒÚ‡‚flÚ ËÁˆflÎÓ. Ç ÚÂÍÒÚ‡ ˆËÚË‡ÌËÚ ‡‚ÚÓË ‰‡ Ò Ô‰ÒÚ‡‚flÚ Ò ÔÓ‰ÂÌ ÌÓÏÂ ÓÚ ÍÌË„ÓÔËÒ‡. чÌÌËÚ ‚ ÍÌË„ÓÔËÒ‡ Ò Ô‰ÒÚ‡‚flÚ ÔÓ ÒΉÌËfl ̇˜ËÌ: ● ëÚ‡ÚËfl ÓÚ ÒÔËÒ‡ÌË : Ä‚ÚÓ /Ë/. ᇄ·‚Ë ̇ ÒÚ‡ÚËflÚ‡. ᇄ·‚Ë ̇ ÒÔËÒ‡ÌËÂÚÓ / Ò˙Í‡ÚÂÌÓ ÔÓ Index medicus/, ÚÓÏ, „Ó‰Ë̇ ̇ ËÁ‰‡‚‡ÌÂ, ÌÓÏÂ ̇ ÍÌËÊ͇ڇ, ÒÚ‡Ìˈ‡ / ÓÚ-‰Ó/. èËÏÂ: Andersen, G., Vestergaard, K., Lauritzen, L. Effective treatment of poststroke depression with the selective serotonin reuptake inhibitor citalopram. Stroke, 25, 1994, 6, 1099-1104. ● äÌË„‡: Ä‚ÚÓ /Ë/ . ᇄ·‚ËÂ. èÓ‰Á‡„·‚ÌË ‰‡ÌÌË. åÂÒÚÓËÁ‰‡‚‡ÌÂ, ËÁ‰‡ÚÂÒÚ‚Ó, „Ó‰Ë̇ ̇ ËÁ‰‡‚‡ÌÂ, ÒÚ‡Ìˈ‡ /ÓÚ-‰Ó/.èËÏÂ : Calligaro,K., DeLaurentis, D., Baker, W. Management of Extracranial Cerebrovascular Disease.Philadelphia-New York, Lippincott - Raven Publishers, 1997, 217. ● èÛ·ÎË͇ˆËË ÓÚ Ò·ÓÌËÍ : Ä‚ÚÓ /Ë/. ᇄ·‚ËÂ: - Ç: ᇄ·‚Ë ̇ Ò·ÓÌË͇. àÁ‰‡ÚÂÎ /Ë/. åÂÒÚÓËÁ‰‡‚‡ÌÂ, „Ó‰Ë̇ ̇ ËÁ‰‡‚‡ÌÂ, ÒÚ‡Ìˈ‡ / ÓÚ-‰Ó/. èËÏÂ : 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. ● Ä‚ÚÓËÚ ÔÓ‰ÔËÒ‚‡Ú ‰ÂÍ·‡ˆËfl, ˜Â Ô‰ÓÒÚ‡‚ÂÌËfl χÚÂˇΠÌ  Ô‰ÎÓÊÂÌ Á‡ Ô˜‡Ú ̇ ‰Û„Ó ÏflÒÚÓ. ● è‰ÓÒÚ‡‚ÂÌËÚ χÚÂˇÎË Ë ÓÔËÒ‡ÌËÚ ‚ Úflı ËÁÒΉ‚‡ÌËfl Úfl·‚‡ ‰‡ ÓÚ„Ó‚‡flÚ Ì‡ ÂÚ˘ÌËÚ Òڇ̉‡ÚË ● èË Ò˙‡‚ÚÓÒÚ‚Ó ÛÚ‚˙‰Â̇ڇ Á‡ Ô˜‡Ú ÒÚ‡ÚËfl Úfl·‚‡ ‰‡ ·˙‰Â ÔÓ‰ÔË҇̇ ÓÚ ‚Ò˘ÍË ‡‚ÚÓË. ê˙ÍÓÔËÒËÚ ‚ ‰‚‡ ÂÍÁÂÏÔÎfl‡ (ËÎË Â‰ËÌ ÂÍÁÂÏÔÎfl Ë CD/USB) ËÁÔ‡˘‡ÈÚ ̇ ‡‰ÂÒ:

ëÓÙËfl 1504, ìÎ. 4 ÅflÎÓ ÏÓ " ‹ 8 åÅÄã " ñ‡ˈ‡ âÓ‡Ì̇" - ÖÄÑ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl èÓÙ. è.ëÚ‡ÏÂÌÓ‚‡

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 CD/USB, using Word 6/Windows 96 or Windows Microsoft Word 97/2000XP 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, 1099-1104. ● 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 CD/USB 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

ëíÄíàçàíÖ - ÑöãÉéëêéóçÄ ëíêÄíÖÉàü Ç èêÖÇÖçñàüíÄçÄ åéáöóçéëöÑéÇÄíÄ ÅéãÖëí Ö‚„ÂÌËfl LJÒË΂‡ . . . . . . . . . . . . . . . . . . . . . . . . . . . .143

STATINS AND PREVENTION OF CEREBROVASULAR DISORDERS Ö. Vassileva . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .143

ÇÄêàÄÅàãçéëí çÄ ëöêÑÖóçÄíÄ óÖëíéíÄ: åÖíéÑ áÄ éñÖçäÄ çÄ ëöêÑÖóçÄíÄ ÄÇíéçéåçÄ êÖÉìãÄñàü èêà çÖÇêéãéÉàóçà áÄÅéãüÇÄçàü ë. å‡ÌÚ‡Ó‚‡, ç. ÑÓÒ. ÑËÏËÚÓ‚, à. ÇÂΘ‚‡ . . . .148

HEART RATE VARIABILITY: A METHOD FOR ASSESSMENT OF CARDIAC AUTONOMIC REGULATION IN NEUROLOGICAL DISEASES S. Mantarova, N. Dos. Dimitrov, I. Velcheva . . . . . . . .148

ÑàëíêéîàçéèÄíàà- ÉÖçéíàè-îÖçéíàèçà äéêÖãÄñàà, äéÉçàíàÇçà çÄêìòÖçàü à ÑàÄÉçéëíàäÄ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡, å ê‡È˜Â‚‡, à. í˙Ì‚‡ . . . . . . . . . . . . . .153

DYSTROPHINOPATHIES- GENOTYPE- PHENOTYPE CORRELATIONS AND COGNITIVE DISORDERS IN PATIENTS WITH DUCHENNE AND BECKER MUSCULAR DYSTROPHIES T Chamova, M Raytcheva, I Tournev . . . . . . . . . . . . . .153

ÑàëíêéîàçéèÄíàà- ëöÇêÖåÖççé ãÖóÖçàÖ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡, à. í˙Ì‚ . . . . . . . . . . . . . . . . . . . . . . . . .160

DYSTROPHINOPATHIES- MODERN TREATMENT OF DUCHENNE AND BECKER MUSCULAR DYSTROPHIES T. Chamova, I. Tournev . . . . . . . . . . . . . . . . . . . . . . . .160

ÄÇíéáéåçé-ÑéåàçÄçíçà îÄåàãçà èÄêñàÄãçà ÖèàãÖèíàóçà ëàçÑêéåàäãàçàóçÄ, ÖãÖäíêéîàáàéãéÉàóçÄ à ÉÖçÖíàóçÄ ïÄêÄäíÖêàëíàäÄ ë. ÜÂÎflÁÍÓ‚‡ . à. í˙Ì‚ . . . . . . . . . . . . . . . . . . . . .164

AUTOSOMAL-DOMINANT FAMILIAL PARTIAL EPILEPSIES-CLINICAL, ELECTROPHYSIOLOGICAL AND GENETIC CHARACTERISTICS. S. Zhelyazkova . I. Tournev . . . . . . . . . . . . . . . . . . . . .164

êÄççà äéÉçàíàÇçà çÄêìòÖçàü èêà ÅéãÖëííÄ çÄ èÄêäàçëéç èÂÚÓ‚‡ å, ê‡È˜Â‚‡ å,, í‡ÈÍÓ‚ ã. . . . . . . . . . . . . .170

EARLY COGNITIVE CHANGES IN PARKINSON’S DISEASE Petrova M., Raycheva M., Traykov L. . . . . . . . . . . . . . .170

éêàÉàçÄãçà ëíÄíàà

ORIGINAL PAPERS

ÖîàäÄëçéëí à èéçéëàåéëí çÄ LEVETIRACETAM èêà ãÖóÖçàÖ çÄ èÄñàÖçíà ë ÖèàãÖèëàü á. á‡ı‡Ë‚, Ö. ÇËÚ‚‡ . . . . . . . . . . . . . . . . . . . . . . .174

EFFICACY AND TOLERABILITY OF LEVETIRACETAM IN PATIENTS WITH EPILEPSY Z. Zahariev, E. Viteva . . . . . . . . . . . . . . . . . . . . . . . . .174

çÄóÄãçàüí ÑÇàÉÄíÖãÖç ëàåèíéå ÇãàüÖ Çöêïì äéÉçàíàÇçàü ëèÄÑ èêà ãéçÉàíìÑàçÄãçé èêéëãÖÑÖçà èÄñàÖçíà ë èÄêäàçëéçéÇÄ ÅéãÖëí ü. ÜÂ΂, å. ê‡È˜Â‚‡, å. èÂÚÓ‚‡, ã. í‡ÈÍÓ‚ . . . .183

INITIAL MOTOR SYMPTOM INFLUENCES COGNITIVE DECLINE IN A LONGITUDINALLY FOLLOWED PATIENTS WITH PARKINSON'S DISEASE Y.Zhelev, M. Raycheva, M. Petrova, L. Traykov . . . . . .183

äéÉçàíàÇçà çÄêìòÖçàü èêà åàéíéçàóçà Ñàëíêéîàà íàè STEINERT à PROMM. ÉÖçéíàè-îÖçéíàè äéêÖãÄñàà í. ó‡ÏÓ‚‡, å ê‡È˜Â‚‡, à. í˙Ì‚ . . . . . . . . . . . . . . .187

COGNITIVE DISORDERS IN MYOTONIC DYSTROPHIES TYPE STEINERT AND PROMM. GENOTYPE CORRELATIONS T. Chamova, M. Raycheva, I. Tarnev . . . . . . . . . . . . . .187


ÑÂÍÂÏ‚Ë 2009

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

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é·ÁÓ ëíÄíàçàíÖ - ÑöãÉéëêéóçÄ ëíêÄíÖÉàü Ç èêÖÇÖçñàüíÄ çÄ åéáöóçéëöÑéÇÄíÄ ÅéãÖëí Ö‚„ÂÌËfl LJÒË΂‡ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇”, ëÓÙËfl SUMMARY STATINS AND PREVENTION OF CEREBROVASULAR DISORDERS Ö. Vassileva Lowering of cholesterol concentration with statins reduces the risk of stroke in high-risk populations and in patients with non-cardioembolic stroke or transient ischaemic attacks. In patients with coronary artery disease, statin treatment has been associated with reduction of risk of stroke. Evidence is now available that statin therapy also reduce stroke risk in patients without coronary artery disease but at high cardiovascvular risk or with diabetes mellitus. Intensive statin therapy started within 6 months after a cerebrovascular event significantly reduces stroke risk. They also have essential biological effects on stability of atherosclerotic plaque, chronic inflammation, thrombogenesis, immune regulation Statin therapy is the most important advance in stroke prevention scince the introduction of aspirin and antihypertensive treatments. KEY WORDS: statins, cerebrovascular disorder, prevention. êÖáûåÖ èÓÌËʇ‚‡ÌÂÚÓ Ì‡ ÍÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ ıÓÎÂÒÚÂÓ· ÓÚ ÒÚ‡ÚËÌÓ‚ÓÚÓ Î˜ÂÌË ̇χÎfl‚‡ ·ÚË‚ÌËfl ËÒÍ ÓÚ ËÒıÂÏ˘ÌË ËÌÒÛÎÚË ‚˙‚ ‚ËÒÓÍÓËÒÍÓ‚‡ ÔÓÔÛ·ˆËfl ͇ÍÚÓ Ë ÔË ·ÓÎÌË Ò ÌÂ͇‰ËÓÂÏ·Ó΢ÂÌ ËÌÒÛÎÚ ËÎË ÔÂıÓ‰ÌË Ì‡Û¯ÂÌËfl ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÒÚ‡ÚËÌËÚ ̇χÎfl‚‡Ú ËÒ͇ ÓÚ Ô˙‚Ë ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ÔË ·ÓÎÌË Ò ÍÓÓ̇̇ Ô‡ÚÓÎÓ„Ëfl, ͇ÍÚÓ Ë ÔË Ú‡ÍË‚‡ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. àÌÚÂÌÁË‚ÌÓÚÓ ÎËÔˉÓÔÓÌËʇ‚‡˘Ó ΘÂÌËÂ, Á‡ÔӘ̇ÚÓ ‰Ó 6 ÏÂÒˆ‡ ÒΉ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ ÒË„ÌËÙË͇ÌÚÌÓ Ì‡Ï‡Îfl‚‡ ËÒ͇ ÓÚ ˆˉ˂. ëÚ‡ÚËÌËÚ ÔËÚÂʇ‚‡Ú ‚‡ÊÌË ·ËÓÎӄ˘ÌË ÂÙÂÍÚË ‚˙ıÛ ÒÚ‡·ËÎËÚÂÚ‡ ̇ ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ô·͇, ÔÓˆÂÒËÚ ̇ ËÏÛÌ̇ „Û·ˆËfl, ıÓÌ˘ÌÓÚÓ ‚˙ÁÔ‡ÎÂÌËÂ, ÚÓÏ·Ó„ÂÌÂÁ‡Ú‡. ëÚ‡ÚËÌÓ‚ÓÚÓ Î˜ÂÌË  ̇È-‚‡ÊÌËfl ̇Ô‰˙Í ‚ Ô‚Â̈ËflÚ‡ ̇ ÏÓÁ˙˜ÌËfl ËÌÒÛÎÚ ÒΉ ‚˙‚Âʉ‡ÌÂÚÓ Ì‡ ‡ÌÚˇ„„‡ÌÚ̇ڇ ÔÓÙË·ÍÚË͇ Ë ‡ÌÚËıËÔÂÚÂÌÁË‚ÌÓÚÓ Î˜ÂÌËÂ. äãûóéÇà Ñìåà: ÒÚ‡ÚËÌË, ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl, Ô‚Â̈Ëfl. àÒıÂÏ˘ÌËÚ ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ò‡ ̇È-˜ÂÒÚËÚ ÓÒÚË Ì‡Û¯ÂÌËfl ̇ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌË - 80% ÓÚ ‚Ò˘ÍË ËÌÒÛÎÚË. ÄÚÂÓÒÍÎÂÓÁ‡Ú‡ Ë ‡ÚÂÓÚÓÏ·ÓÁ‡Ú‡ Ò‡ ÓÒÌÓ‚ÌËÚ ԇÚÓÎӄ˘ÌË ÔÓˆÂÒË, Ô˘ËÌfl‚‡˘Ë ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (àåà). ÄÚÂÓÒÍÎÂÓÁ‡Ú‡ ‚Ó‰Ë ‰Ó ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ ÔÓ ‰‚‡ ÓÒÌÓ‚ÌË ÏÂı‡ÌËÁχ: ˜ÂÁ Ó·‡ÁÛ‚‡Ì ̇ Ô·ÍË ÔÓ ‡ÓÚ̇ڇ ‰˙„‡ Ë ÒÚÂÌÓÁË‡Ì ̇ ÂÍÒÚ‡Í‡ÌˇÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË Ë ˜ÂÁ ‡Á‚ËÚË ̇ ËÒıÂÏ˘̇ ·ÓÎÂÒÚ Ì‡ Ò˙ˆÂÚÓ Ò ÔÓÒΉ‚‡˘ ÏËÓ͇‰ÂÌ ËÌÙ‡ÍÚ Ë Ô‰Ò˙‰ÌÓ Ï˙ʉÂÌÂ. éÍÓÎÓ 30% ÓÚ ÏÓÁ˙˜ÌËÚ ËÌÙ‡ÍÚË Ò‡ ÂÁÛÎÚ‡Ú ÓÚ ‡ÚÂÓÒÍÎÂÓÁ‡ Ë ‡ÚÂÓÚÓÏ·ÓÁ‡ ̇ ‡ÓÚ̇ڇ ‰˙„‡ Ë Ì‡ ÂÍÒÚ‡Í‡ÌˇÎÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË.

ëíÄíàçà à àëïÖåàóÖç àçëìãí ÑËÂÍÚ̇ڇ ‚˙Á͇ ÏÂÊ‰Û ÒÂÛÏÌÓÚÓ ÌË‚Ó Ì‡ ıÓÎÂÒÚÂÓ· Ë ÏÓÁ˙˜ÌËfl ËÌÒÛÎÚ Â ·Ë· Ó·ÂÍÚ Ì‡ ÔÓÛ˜‚‡Ì ÓÚ ÏÌÓÊÂÒÚ‚Ó ÏÂÚ‡‡Ì‡ÎËÁË, ̇Ô‡‚ÂÌË ÔÂÁ ÔÓÒΉÌËÚ ‰ÂÒÂÚ „Ó‰ËÌË. êÂÁÛÎÚ‡ÚËÚ ÓÚ Ô˙‚ËÚ ÓÚ Úflı Ì ‰Ó͇Á‚‡Ú ÍÓ·ˆËfl ÏÂÊ‰Û ÒÂÛÏÌÓÚÓ ÌË‚Ó Ì‡ ıÓÎÂÒÚÂÓ· Ë ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (1). è˘ËÌËÚ Á‡ ÚÓ‚‡ Ò‡ ‡Á΢ÌË. í‡Í‡ ̇ÔËÏÂ ÌflÍÓË ÓÚ ÏÂÚ‡‡Ì‡ÎËÁËÚÂ, ‚˙ÔÂÍË ˜Â Ó·ı‚‡˘‡Ú ÏÌÓ„Ó·ÓÈÌË ÔÓÛ˜‚‡ÌËfl Ò ÏÌÓ„Ó „ÓÎflÏ ·ÓÈ ·ÓÎÌË, ËÁÒΉ‚‡Ú ‚˙Á͇ڇ ̇ ıÓÎÂÒÚÂÓ· Ò ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ò‡ÏÓ Ì‡ Ù‡Ú‡ÎÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË (1,2). éÚÌÓÒËÚÂÎÌËflÚ ‰flΠ̇ Ù‡Ú‡ÎÌËÚ ËÌÒÛÎÚË Ó·‡˜Â  ҇ÏÓ 20% ÓÚ ‚Ò˘ÍË ËÌÒÛÎÚË. éÒ‚ÂÌ ÚÓ‚‡ ‚ ÚÂÁË ‡Ì‡ÎËÁË Ò ‚Íβ˜‚‡Ú ‚Ò˘ÍË ÓÒÚË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Ë̈ˉÂÌÚË, ‚Íβ˜ËÚÂÎÌÓ Ë ÏÓÁ˙˜ÌËÚ Í˙‚ÓËÁÎË‚Ë. ç Ò ‚ÁÂχ ÔÓ‰ ‚ÌËχÌË ̇΢ËÂÚÓ Ì‡ ıÂÚÂÓ„ÂÌÌÓÒÚ Ì‡ àåà ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÂÚËÓÔ‡ÚÓ„ÂÌÂÁ‡Ú‡. ᇠ‰‡ ÔÓ͇Ê ÓÎflÚ‡ ̇ ıÓÎÂÒÚÂÓ· ‚ Ô‡ÚÓÙËÁËÓÎÓ„ËflÚ‡ ̇ ÏÓÁ˙˜ÌËfl ËÌÒÛÎÚ, ÔÂÁ 2009 „Ó‰Ë̇ Amarenco Ë Labreuche (3) Ô‡‚flÚ ÏÂÚ‡‡Ì‡ÎËÁ ̇ 24 ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËfl, ÔÛ·ÎËÍÛ‚‡ÌË ÏÂÊ‰Û 1994 Ë 2009 „. Ò ÓÒÌÓ‚ÌÓ ËÁÔÓÎÁ‚‡ÌËÚ ÒÚ‡ÚËÌË. ä‡Ò‡Â Ò Á‡ ÓË„Ë̇ÎÌË ÔÓÛ˜‚‡ÌËfl, ‚ÒflÍÓ ÓÚ ÍÓËÚÓ Ò ÏËÌËÏÛÏ 1000 Ô‡ˆËÂÌÚË. åÂÚ‡‡Ì‡ÎËÁ˙Ú ËÁÒΉ‚‡ ÂÙÂÍÚ‡ ̇ ÒÚ‡ÚËÌËÚ Á‡ Ô‚Â̈Ëfl ̇ ËÌÒÛÎÚ Ó·˘Ó ÔË 165 000 ‚ËÒÓÍÓËÒÍÓ‚Ë Îˈ‡. èË Ô‡ˆËÂÌÚËÚÂ, ‡Ì‰ÓÏËÁË‡ÌË ‰‡ ÔÓÎÛ˜‡‚‡Ú ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËfl, ÏÂÚ‡-‡Ì‡ÎËÁ˙Ú ‰ÂÏÓÌÒÚË‡ ‰Û͈Ëfl ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‚Ò˘ÍË ËÌÒÛÎÚË Ò 18% (ÙË„.1). ìÒÚ‡ÌÓ‚fl‚‡ Ò Ò˙˘Ó, ˜Â ‚ÒÂÍË 1 mmol/l (39 mg/dL) ÔÓÌËʇ‚‡Ì ̇ ÍÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ LDL-ıÓÎÂÒÚÂÓ· Ò ‡‚Ìfl‚‡ ̇ 21% ‰Û͈Ëfl ̇ ·ÚË‚ÌËfl ËÒÍ Á‡ ËÌÒÛÎÚ (95% CI 6 3–33 5, p=0 009). Ç˙‚ ‚ÚÓ˘̇ڇ ÔÓÙË·ÍÚË͇ ̇ ÌÂ͇‰ËÓÂÏ·Ó΢ÌËfl ËÌÒÛÎÚ, ËÌÚÂÌÁ˂̇ڇ ‰Û͈Ëfl ̇ LDL-ıÓÎÂÒÚÂÓ· ÔÓÒ‰ÒÚ‚ÓÏ ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËfl Ò˙˘Ó ‚Ó‰Ë ‰Ó ÒË„ÌËÙË͇ÌÚ̇ ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ ˆˉ˂ (ÓÚÌÓÒËÚÂÎÂÌ ËÒÍ 0 84, 0 71–0 99, p=0 03) Ë Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Ë̈ˉÂÌÚË (0 80, 0 69–0 92, p=0 002). Ç˙ÔÂÍË ˜Â  ÚÛ‰ÌÓ ‰‡ ·˙‰Â ÛÒÚ‡ÌÓ‚Â̇ ‰ËÂÍÚ̇ڇ

îË„.1 äÓ·ˆËfl ÏÂÊ‰Û ‰Û͈Ëfl ̇ LDL-ıÓÎÂÒÚÂÓÎ Ë ˜ÂÒÚÓÚ‡ ̇ ÏÓÁ˙˜ÌË ËÌÒÛÎÚË


ÑÂÍÂÏ‚Ë 2009

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

‚˙Á͇ ÏÂÊ‰Û ıËÔÂıÓÎÂÒÚÂÓÎÂÏËflÚ‡ Ë àåà, ÂÁÛÎÚ‡ÚËÚ ÓÚ Ôӂ˜ÂÚÓ ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ÔÓÌËʇ‚‡ÌÂÚÓ Ì‡ ıÓÎÂÒÚÂÓ· ÔÓÌËʇ‚‡ Ë ËÒ͇ ÓÚ ÏÓÁ˙˜ÌË ËÌÒÛÎÚË ÔË ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË. ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà èêà èÄñàÖçíà ë äéêéçÄêçÄ èÄíéãéÉàü êÓÎflÚ‡ ̇ ÒÚ‡ÚËÌËÚ ‚ Ô‚Â̈ËflÚ‡ ̇ ÏËÓ͇‰ÌËfl ËÌÙ‡ÍÚ ÔË ·ÓÎÌË Ò ÍÓÓ̇̇ Ô‡ÚÓÎÓ„Ëfl  ÓÚ‰‡‚̇ ÛÒÚ‡ÌÓ‚Â̇ Ë ·ÂÁÒÔÓ̇, ÍÓÂÚÓ Ó·ÛÒ·‚fl „ÓÎflÏÓÚÓ ËÏ Á̇˜ÂÌË Á‡ ÔÓÙË·ÍÚË͇ڇ ̇ ÚÂÁË Á‡·ÓÎfl‚‡ÌËfl. Ç ÏÌÓ„Ó ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡, ËÁÒΉ‚‡˘Ë Á̇˜ÂÌËÂÚÓ Ì‡ ÒÚ‡ÚËÌËÚ Á‡ Ô‚Â̈Ëfl ̇ ÏËÓ͇‰ÂÌ ËÌÙ‡ÍÚ ÔË ·ÓÎÌË Ò ËÁ‚ÂÒÚ̇ ÍÓÓ̇̇ ·ÓÎÂÒÚ, Ò ÓÚ˜ËÚ‡ ÚflıÌÓÚÓ Á̇˜ÂÌËÂ Ë ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ô˙‚˘̇ڇ ÔÓÙË·ÍÚË͇ ̇ àåà. ìÒÚ‡ÌÓ‚ÂÌÓ ·Â, ˜Â ÚÓ‚‡ ΘÂÌË ‰ÓÔË̇Òfl ÓÒ‚ÂÌ Á‡ ̇χÎfl‚‡Ì ˜ÂÒÚÓÚ‡Ú‡ ̇ ÏËÓ͇‰ÌËfl ËÌÙ‡ÍÚ, Ó˘Â Ë Á‡ ÔÓÌËʇ‚‡Ì Á‡·Ó΂‡ÂÏÓÒÚÚ‡ ÓÚ ËÌÒÛÎÚË ‚ ÚÓÁË ÍÓÌÚËÌ„ÂÌÚ ·ÓÎÌË. í‡Í‡ ̇ÔËÏÂ, ‚ ÔÓÛ˜‚‡ÌÂÚÓ Heart Protecton Study (HPS) Ò‡ ‡Ì‰ÓÏËÁË‡ÌË Ó·˘Ó 20 536 ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË, 65% (13 386) ÓÚ ÍÓËÚÓ Ò ‰Ë‡„ÌÓÒÚˈË‡Ì‡ ÍÓÓ̇̇ Ô‡ÚÓÎÓ„Ëfl. äÓ„‡ÚÓ ÒΉ 4,8 „Ó‰ËÌË Ò‰ÌÓ ÔÓÒΉfl‚‡Ì  ̇Ô‡‚ÂÌ ‡Ì‡ÎËÁ ̇ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÒÚ‡ÚËÌÓ‚‡Ú‡ ÔÓÙË·ÍÚË͇, ‚ ÔÓÒΉ̇ڇ „ÛÔ‡  ÛÒÚ‡ÌÓ‚Â̇ 25% ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ Ô˙‚Ë àåà (4). Ç ‰Û„Ó ÔÓ‰Ó·ÌÓ ÔÓÛ˜‚‡Ì – Scandinavian Simvastatin Survival Study (4S) Ò‡ ‚Íβ˜ÂÌË 4444 ·ÓÎÌË Ò ËÒıÂÏ˘̇ ·ÓÎÂÒÚ Ì‡ Ò˙ˆÂÚÓ (àÅë), ÍÓËÚÓ Ì ҇ ÔÂÊË‚flÎË àåà. ᇠÔÂËÓ‰ ÓÚ 5,4 „Ó‰ËÌË Ò‰ÌÓÚÓ ÌË‚Ó Ì‡ LDL-ıÓÎÂÒÚÂÓÎ ‚ „ÛÔ‡Ú‡, ÔÓÎÛ˜‡‚‡Î‡ ÒÚ‡ÚËÌÓ‚Ó Î˜ÂÌËÂ,  ̇χÎÂÌÓ Ò 35% ÔÓ ÓÚÌÓ¯ÂÌË ̇ ËÁıÓ‰ÌËÚ ÒÚÓÈÌÓÒÚË Ë Â ÓÚ˜ÂÚÂ̇ 30% ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ Ù‡Ú‡ÎÌË Ë ÌÂÙ‡Ú‡ÎÌË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Ë̈ˉÂÌÚË (5). ëË„ÌËÙË͇ÌÚÌÓÚÓ Ì‡Ï‡Îfl‚‡Ì ̇ ËÒ͇ ÓÚ àåà ÔË ·ÓÎÌË Ò àÅë ‚ ÂÁÛÎÚ‡Ú Ì‡ ÒÚ‡ÚËÌÓ‚‡ ÔÓÙË·ÍÚË͇ Ò˙˘Ó  ÔÓÚ‚˙‰ÂÌÓ Ë ÓÚ ‰‚ ‰Û„Ë ÔÓÛ˜‚‡ÌËfl – Cholesterol and Recurrent Events (CARE) Ë Long-term Intervention with Pravastatin in Ischemic Diseasese (LIPID) (6, 7). í Ò‡‚Ìfl‚‡Ú 40 mg Ô‡‚‡ÒÚ‡ÚËÌ ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ô·ˆÂ·Ó ÔË Ô‡ˆËÂÌÚË Ò àÅë Ë ÓÚ˜ËÚ‡Ú 22% ‰Û͈Ëfl ̇ ·ÚË‚ÌËfl ËÒÍ ÔË ·ÓÎÌËÚ Ò˙Ò ÒÚ‡ÚËÌÓ‚Ó Î˜ÂÌËÂ. èÓÛ˜‚‡ÌÂÚÓ Treating to New Targets (íNí) (8) ËÁÒΉ‚‡ ÂÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ËÌÚÂÌÁ˂̇ڇ ÎËÔˉÓÔÓÌËʇ‚‡˘‡ ÚÂ‡ÔËfl, ÍÓflÚÓ ‚Ó‰Ë ‰Ó ̇χÎfl‚‡Ì ̇ LDL- ıÓÎÂÒÚÂÓ· ÔÓ‰ 2,6 mmol/L (100 mg/dL). ᇠڇÁË ˆÂÎ 10 001 Ô‡ˆËÂÌÚË Ò ‰ÓÍÛÏÂÌÚË‡Ì‡ àÅë Ò‡ ÔÓÎÛ˜‡‚‡ÎË Òڇ̉‡ÚÌÓ Î˜ÂÌË (Ò 10 mg ‡ÚÓ‚‡ÒÚ‡ÚËÌ) ËÎË ËÌÚÂÌÁË‚ÌÓ ÎËÔˉÓÔÓÌËʇ‚‡˘Ó ΘÂÌË (Ò 80 mg ‡ÚÓ‚‡ÒÚ‡ÚËÌ). èË Ò‡‚ÌÂÌË ̇ ‰‚ÂÚ „ÛÔË Â ÓÚ˜ÂÚÂ̇ 23% ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Ë̈ˉÂÌÚË Ë 25% ̇ ËÒ͇ ÓÚ Ù‡Ú‡ÎÌË Ë ÌÂÙ‡Ú‡ÎÌË ËÌÒÛÎÚË ‚ ÔÓÎÁ‡ ̇ ËÌÚÂÌÁË‚ÌÓÚÓ ÎËÔˉÓÔÓÌËʇ‚‡˘Ó ΘÂÌËÂ. чÌÌËÚ ÓÚ Ó·˘Ó 26 ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËfl Ò‡ Ó·Ó·˘ÂÌË ÓÚ Amarenco et al. ‚ ÏÂÚ‡‡Ì‡ÎËÁ, ÍÓÈÚÓ ‚Íβ˜‚‡ Ó·˘Ó 90 000 Ô‡ˆËÂÌÚ‡ Ò àÅë Ë ‰Ó͇Á‚‡, ˜Â ÒÚ‡ÚËÌËÚ ̇χÎfl‚‡Ú ËÒ͇ ÓÚ Ô˙‚Ë àåà ÔË ·ÓÎÌË Ò àÅë Ò 21% ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ô·ˆÂ·Ó (9). Ç Á‡Íβ˜ÂÌË - ÓÎflÚ‡ ̇ ÒÚ‡ÚËÌËÚ Á‡ Ô˙‚˘̇ ÔÓÙË·ÍÚË͇ ̇ àåà ÔË ·ÓÎÌË Ò ÍÓÓ̇̇ Ô‡ÚÓÎÓ„Ëfl  ·ÂÁÒÔÓ̇. ÇÒ˘ÍË „ÓÎÂÏË Ô·ˆÂ·Ó-ÍÓÌÚÓÎË‡ÌË, ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËfl (4S, CARE, LIPID, HPS, TNT) (4-8) ‰Ó͇Á‚‡Ú, ˜Â ΘÂÌËÂÚÓ Ò˙Ò ÒÚ‡ÚËÌË ÔÓÌËʇ‚‡ ËÒ͇ ÓÚ Ô˙‚Ë àåà ÔË Ô‡ˆËÂÌÚË Ò ÍÓÓ̇̇ ·ÓÎÂÒÚ Ò‰ÌÓ Ò

144

25%-35%. ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà èêà èÄñàÖçíà ëöë áÄïÄêÖç ÑàÄÅÖí êÓÎflÚ‡ ̇ ÎËÔˉÓÔÓÌËʇ‚‡˘‡Ú‡ ÚÂ‡ÔËfl Á‡ Ô˙‚˘̇ ÔÓÙË·ÍÚË͇ ̇ Ò˙‰Â˜ÌÓ-Ò˙‰Ó‚Ë Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl ÔË ·ÓÎÌË Ò ‚ÚÓË ÚËÔ Á‡ı‡ÂÌ ‰Ë‡·ÂÚ Â ÛÒÚ‡ÌÓ‚Â̇ Ӣ ÔÂÁ 2004 „Ó‰. èÓÛ˜‚‡ÌÂÚÓ Collaborative Atorvastatin Diabetes Study (CARDS) (10) ‡Ì‰ÓÏËÁË‡ 2838 Ô‡ˆËÂÌÚ‡ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ Ë ÔÓÌ Ӣ ‰ËÌ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ åëÅ, ÌÓ ·ÂÁ ÔÂ͇‡ÌË ÓÒÚË Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Ë̈ˉÂÌÚË. éÒ˙˘ÂÒÚ‚ÂÌÓ Â ÚflıÌÓÚÓ ÔÓÒΉfl‚‡Ì ‰Ó ÏÓÏÂÌÚ‡ ̇ ‚˙ÁÌËÍ‚‡Ì ̇ Ú‡Í˙‚ Ë̈ˉÂÌÚ. èÓÛ˜‚‡ÌÂÚÓ Ó·‡˜Â  ÔÂÍ‡ÚÂÌÓ ‰‚ „Ó‰ËÌË ÔÓ-‡ÌÓ ÓÚ Ô‰‚ˉÂÌËfl ÒÓÍ ÔÓ‡‰Ë ‰ÓÒÚË„‡ÌÂÚÓ Ì‡ ۷‰ËÚÂÎÌË ÂÁÛÎÚ‡ÚË. ÑÓ͇Á‡Ì‡  48% ‰Û͈Ëfl ‚ ·ÚË‚ÌËfl ËÒÍ ÓÚ àåà (95% CI,11% to 69%; p=0,016), 31% ‰Û͈Ëfl - Á‡ ÓÒÚË ÍÓÌÓ‡ÌË Ë̈ˉÂÌÚË Ë 31% - Á‡ ÍÓÓ̇̇ ‚‡ÒÍÛ·ËÁ‡ˆËfl. èÓ‡‰Ë ÌÂÒ˙ÏÌÂÌËÚ ÔÓÎÁË Á‡ Ô‡ˆËÂÌÚËÚ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, ÄÏÂË͇ÌÒ͇ڇ ÄÒӈˇˆËfl ÔÓ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë, ͇ÍÚÓ Ë Ú‡ÁË ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl ÔÂÁ 2006 „Ó‰ ÔËÂχÚ, ˜Â ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰Ë‡·ÂÚ, ÓÒÓ·ÂÌÓ ÔË Ì‡Î˘ËÂ Ë Ì‡ ‰Û„Ë ËÒÍÓ‚Ë Ù‡ÍÚÓË,  ÔÂÔÓ˙˜ËÚÂÎÌÓ ‰Ó·‡‚flÌ ̇ ÒÚ‡ÚËÌË ‚ ‰ÓÔ˙ÎÌÂÌË ̇ ÒÚËÍÚÌËfl ÍÓÌÚÓΠ̇ Í˙‚ÌÓÚÓ Ì‡Îfl„‡ÌÂ, Ò ˆÂÎ ÔÓÙË·ÍÚË͇ ̇ àåà (11). èÓÙË·ÍÚË͇ڇ Ò˙Ò ÒÚ‡ÚËÌË Ì‡Ï‡Îfl‚‡ Ò Â‰Ì‡ ˜ÂÚ‚˙Ú ˜ÂÒÚÓÚ‡Ú‡ ̇ ÏËÓ͇‰ÌËÚ ËÌÙ‡ÍÚË, ËÒıÂÏ˘ÌËÚ ËÌÒÛÎÚË Ë ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡ ÔË ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË. ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà èêà èÄñàÖçíà ÅÖá ëöÑéÇÄ èÄíéãéÉàü éÒÓ·ÂÌ ËÌÚÂÂÒ Ô‰ÒÚ‡‚Îfl‚‡ ‚˙ÔÓÒ˙Ú, ËÏ‡Ú ÎË ÏflÒÚÓ ÒÚ‡ÚËÌËÚ ‚ Ô˙‚˘̇ڇ ÔÓÙË·ÍÚË͇ ̇ àåà ÔË Ô‡ˆËÂÌÚË ·ÂÁ ‰Ë‡„ÌÓÒÚˈË‡Ì‡ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl ËÎË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. è˙‚ÓÚÓ ÔÓÛ˜‚‡ÌÂ, ÍÓÂÚÓ ÔÓ͇Á‚‡ ÒË„ÌËÙË͇ÌÚÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ àåà ÔË Ô‡ˆËÂÌÚËÚ ·ÂÁ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl  Anglo-Scandinavian Cardiac Outcome Trial – Lipid Lowering Arm (ASCOT-LLA) (12). 臈ËÂÌÚËÚÂ, Ó·ÂÍÚ Ì‡ ËÁÒΉ‚‡ÌÂ, Ò‡ ‡ÒËÏÔÚÓχÚ˘ÌË, ÌÓ ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË - Ò ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl Ë ÔÓÌ Ӣ ÚË ËÒÍÓ‚Ë Ù‡ÍÚÓ‡ Á‡ åëÅ. êÂÁÛÎÚ‡ÚËÚ ÔÓ͇Á‚‡Ú 27% ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ Ô˙‚Ë àåà ‚ ÂÁÛÎÚ‡Ú Ì‡ ΘÂÌË Ò˙Ò ÒÚ‡ÚËÌË ÔË ÚÂÁË Ô‡ˆËÂÌÚË. éÚ ÓÒÓ·ÂÌÓ Á̇˜ÂÌË Á‡ Ó·Ó„‡Úfl‚‡Ì Á̇ÌËflÚ‡ Á‡ ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl Ë Ô‚Â̈ËflÚ‡ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡Ú‡ ·ÓÎÂÒÚ Â Ì‡ÒÍÓÓ ÔÛ·ÎËÍÛ‚‡ÌÓÚÓ ÔÓÛ˜‚‡Ì JUPITER (The Justification for the Use of Statins in Primary Prevention: an Intervention Trial Evaluating Rosuvastatin) (13). íÓ ‚Íβ˜‚‡ 89 890 Ô‡ˆËÂÌÚ‡, ÍÓËÚÓ ÌflÏ‡Ú Ë̉Ë͇ˆËË Á‡ ΘÂÌË Ò˙Ò ÒÚ‡ÚËÌË. JUPITER  ‡Ì‰ÓÏËÁË‡ÌÓ, ‰‚ÓÈÌÓ-ÒÎflÔÓ, Ô·ˆÂ·Ó-ÍÓÌÚÓÎË‡ÌÓ, ÏÌÓ„ÓˆÂÌÚÓ‚Ó ÔÓÛ˜‚‡ÌÂ, ËÁÒΉ‚‡˘Ó ÓÎflÚ‡ ̇ ÚÂ‡ÔËflÚ‡ Ò˙Ò ÒÚ‡ÚËÌË ÔË Îˈ‡ Ò ÌÓχÎÌË ÌË‚‡ ̇ LDL-ıÓÎÂÒÚÂÓÎ, ÌÓ Ò Ôӂ˯ÂÌ Í‡‰ËÓ‚‡ÒÍÛ·ÂÌ ËÒÍ, ˉÂÌÚËÙˈË‡Ì ÓÚ ÒÚÓÈÌÓÒÚË Ì‡ ‚ËÒÓÍÓ-ÒÂÌÁËÚË‚ÌËfl C-‡ÍÚË‚ÂÌ ÔÓÚÂËÌ (hsCRP _>2 mg/l). è˙‚˘ÂÌ ÔÓÒΉfl‚‡Ì ÔÓ͇Á‡ÚÂÎ Â ‚ÂÏÂÚÓ ‰Ó ÔÓfl‚‡ ̇ Ô˙‚Ó Ò˙·ËÚË (ËÌÙ‡ÍÚ Ì‡ ÏËÓ͇‰‡, àåà, ıÓÒÔËÚ‡ÎËÁ‡ˆËfl ÔÓ‡‰Ë ÌÂÒÚ‡·ËÎ̇ ÒÚÂÌÓ͇‰Ëfl, ‡ÚÂˇÎ̇ ‚‡ÒÍÛ·ËÁ‡ˆËfl ËÎË Í‡‰ËÓ‚‡ÒÍÛ·̇ ÒÏ˙Ú). èÂÁ ÔÂËÓ‰‡ ̇ ÔÓÒΉfl‚‡Ì (Ò‰ÌÓ 1,9 „Ó‰ËÌË, χÍÒËÏÛÏ 5 „Ó‰ËÌË), rosuvastatin ‚Ó‰Ë ‰Ó ÒË„ÌËÙË͇ÌÚ̇ ‰Û͈Ëfl ̇ ÒÂËÓÁÌËÚ ͇‰ËÓ‚‡ÒÍÛ·ÌË Ë̈ˉÂÌÚË (142


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

ÑÂÍÂÏ‚Ë 2009

ÒÔflÏÓ 251 ‚ „ÛÔ‡Ú‡ ̇ Ô·ˆÂ·Ó), ÌÂÁ‡‚ËÒËÏÓ ÓÚ Ù‡ÍÚ‡, ˜Â ÔÓ˜ÚË ‚Ò˘ÍË Û˜‡ÒÚÌËˆË ËÏ‡Ú Ì‡˜‡ÎÌË ÌË‚‡ ̇ ÎËÔˉËÚÂ, ÌÂËÁËÒÍ‚‡˘Ë ÚÂ‡ÔËfl Ò˙„·ÒÌÓ Ì‡ÒÚÓfl˘ËÚ ˙ÍÓ‚Ó‰ÒÚ‚‡ Á‡ Ô‚Â̈Ëfl (퇷Î.1). ÅÓflÚ Ì‡ Ô‡ˆËÂÌÚËÚ ÓÚ ËÁÒΉ‚‡ÌËfl ÍÓÌÚËÌ„ÂÌÚ, ÍÓËÚÓ Â ÌÂÓ·ıÓ‰ËÏÓ ‰‡ Ò ÎÂÍÛ‚‡Ú Ò rosuvastatin ‚ ÔÓ‰˙ÎÊÂÌË ̇ 2 „Ó‰ËÌË, Á‡ ‰‡ Ò Ô‰ÓÚ‚‡ÚË ÔÓfl‚‡Ú‡ ̇ ‰ËÌ í‡·Î.1 êÂÁÛÎÚ‡ÚË

Ò˙‰Ó‚ Ë̈ˉÂÌÚ,  95. ᇠÔÂڄӉ˯ÂÌ ÔÂËÓ‰  ÌÂÓ·ıÓ‰ËÏÓ ‰‡ Ò ÎÂÍÛ‚‡Ú 25 Ô‡ˆËÂÌÚË, Á‡ ‰‡ Ò Ô‰ÓÚ‚‡ÚË ÔÓfl‚‡Ú‡ ̇ ‰ÌÓ Ò˙·ËÚËÂ. éÚ ÓÒÓ·ÂÌÓ Á̇˜ÂÌË  هÍÚ˙Ú, ˜Â Ó·˘ËflÚ ·ÓÈ Ì‡ Ò˙Ó·˘ÂÌË ÒÂËÓÁÌË ÌÂÊ·ÌË Ò˙·ËÚËfl  ÒıÓ‰ÂÌ ‚ „ÛÔ‡Ú‡ ̇ rosuvastatin Ë Ú‡ÁË Ò Ô·ˆÂ·Ó (Ò˙ÓÚ‚ÂÚÌÓ 1352 Ë 1377; P = 0.60). è‰˯ÌË ÔÓÛ˜‚‡ÌËfl Ò˙Ò ÒÚ‡ÚËÌË (Ôӂ˜ÂÚÓ ÓÚ ÍÓËÚÓ ËÁÔÓÎÁ‚‡Ú ÌË‚ÓÚÓ Ì‡ LDL-ıÓÎÂÒÚÂÓΠ͇ÚÓ ÍËÚÂËÈ Á‡ ‚Íβ˜‚‡ÌÂ) Ò˙Ó·˘‡‚‡Ú Á‡ 20% ‰Û͈Ëfl ̇ Ò˙‰Ó‚Ëfl ËÒÍ Á‡ ‚ÒÂÍË 1 mmol/l ‡·ÒÓβÚ̇ ‰Û͈Ëfl ̇ ÌË‚ÓÚÓ Ì‡ LDL-ıÓÎÂÒÚÂÓ·, ÍÓÂÚÓ Ô‰ÔÓ·„‡ ÔÓÔÓˆËÓ̇Î̇ ‰Û͈Ëfl ̇ ·Ófl ̇ Ò˙·ËÚËflÚ‡ ‚ JUPITER ÓÚ ÓÍÓÎÓ 25%. é·‡˜Â, ‰Û͈ËflÚ‡ ̇ ËÒ͇ ‚ ÚÓ‚‡ ÔÓÛ˜‚‡ÌÂ, ÔË ÍÓÂÚÓ ÍËÚÂËÈ Á‡ ‚Íβ˜‚‡Ì  Ôӂ˯ÂÌÓÚÓ ÌË‚Ó Ì‡ ‚ËÒÓÍÓ-˜Û‚ÒÚ‚ËÚÂÎÌËfl ë-‡ÍÚË‚ÂÌ ÔÓÚÂËÌ, ‡ Ì Ôӂ˯ÂÌÓÚÓ ÌË‚Ó Ì‡ LDL-ıÓÎÂÒÚÂÓ·,  ·ÎËÁÓ ‰‚‡ Ô˙ÚË ÔÓ-„ÓÎflχ Ë ÔÓ͇Á‚‡ ÔÓ-‚ËÒÓ͇ ·Ú˂̇ ÔÓÎÁ‡, ÓÚÍÓÎÍÓÚÓ Ú‡ÁË, ÛÒÚ‡ÌÓ‚Â̇ ‚ Ôӂ˜ÂÚÓ Ô‰˯ÌË ÔÓÛ˜‚‡ÌËfl Ò˙Ò ÒÚ‡ÚËÌË. Ç Ó·Ó˘ÂÌËÂ, ΘÂÌËÂÚÓ Ò rosuvastatin 20 mg ‚Ó‰Ë ‰Ó 44% ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ ÒÂËÓÁÌË Í‡‰ËÓ‚‡ÒÍÛ·ÌË Ò˙·ËÚËfl (<0.00001), 54% ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ Ù‡Ú‡ÎÂÌ Ë ÌÂÙ‡Ú‡ÎÂÌ ËÌÙ‡ÍÚ Ì‡ ÏËÓ͇‰‡, 47% ‰Û͈Ëfl ̇ ‡ÚÂˇÎ̇ڇ ‚‡ÒÍÛ·ËÁ‡ˆËfl ËÎË ıÓÒÔËÚ‡ÎËÁ‡ˆËfl ÔÓ‡‰Ë ÌÂÒÚ‡·ËÎ̇ ÒÚÂÌÓ͇‰Ëfl, 47% ‰Û͈Ëfl Á‡ ÍÓÏ·ËÌË‡ÌËfl ÔÓ͇Á‡ÚÂÎ, ‚Íβ˜‚‡˘ ͇‰ËÓ‚‡ÒÍÛ·̇ ÒÏ˙Ú, ÌÂÙ‡Ú‡ÎÂÌ ËÌÙ‡ÍÚ Ì‡ ÏËÓ͇‰‡ Ë ÌÂÙ‡Ú‡ÎÂÌ ËÌÒÛÎÚ. ã˜ÂÌËÂÚÓ Ò rosuvastatin 20 mg ‚Ó‰Ë ‰Ó ‰Û͈Ëfl ̇ Ó·˘‡Ú‡ ÒÏ˙ÚÌÓÒÚ Ò 20% ‚ Ò‡‚ÌÂÌËÂ Ò Ô·ˆÂ·Ó (=0.02), ‚ ÔÓÔÛ·ˆËfl ·ÂÁ ÛÒÚ‡ÌÓ‚Â̇ ÍÓÓ̇̇ ·ÓÎÂÒÚ Ì‡ Ò˙ˆÂÚÓ. àÁ‚ÂÒÚÌÓ Â, ˜Â ÔÓ-ÒÚ‡ËÚ ÔÓÛ˜‚‡ÌËfl (WOSCOPS, AFCAPS/TexCAPS, PROSPER) (14-16) Ì ÛÒÔfl‚‡Ú ‰‡ ‰ÓÍ‡Ê‡Ú Ì‡Ï‡Îfl‚‡Ì ̇ ËÒ͇ ÓÚ Ô˙‚Ë àåà ÔË Ô‡ˆËÂÌÚËÚ ·ÂÁ ÍÓÓ̇̇ Ô‡ÚÓÎÓ„Ëfl. ëíÄíàçà à ÇíéêàóçÄ èêéîàãÄäíàäÄ çÄ àåà èÓÛ˜‚‡ÌÂÚÓ SPARCL (Stroke Prevention by Aggressive Reduction in Cholesterol Levels) (17)  Ô˙‚ÓÚÓ ÔÓÛ˜‚‡ÌÂ,

145

ˆÂÎÚ‡ ̇ ÍÓÂÚÓ Â ‰‡ ̇Ô‡‚Ë ÔÓÒÔÂÍÚ˂̇ ÓˆÂÌ͇ ̇ ΘÂÌËÂÚÓ Ò˙Ò ÒÚ‡ÚËÌË ÔË Ô‡ˆËÂÌÚË, ÔÂ͇‡ÎË àåà. ÇÍβ˜ÂÌË Ò‡ 4 731 ·ÓÎÌË ÔÂÊË‚ÂÎË àåà ËÎË íàÄ ‚ ‡ÏÍËÚ ̇ 6 ÏÂÒˆ‡ ÓÚ Á‡ÔÓ˜‚‡Ì ̇ ÒÚ‡ÚËÌÓ‚ÓÚÓ Î˜ÂÌËÂ. 臈ËÂÌÚËÚ ҇ ·ÂÁ ÍÓÓ̇̇ Ô‡ÚÓÎÓ„Ëfl Ë Ò ÌÓχÎÌÓ ıÓÎÂÒÚÂÓÎÓ‚Ó ÌË‚Ó. ç‡Ô‡‚ÂÌÓ Â ÔÓÒΉfl‚‡Ì ‰Ó ‚˙ÁÌËÍ‚‡Ì ̇ ÒΉ‚‡˘ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ Ë̈ˉÂÌÚ. êÂÁÛÎÚ‡ÚËÚ ÔÓ͇Á‚‡Ú, ˜Â ËÌÚÂÌÁ˂̇ڇ ÚÂ‡ÔËfl Ò˙Ò ÒÚ‡ÚËÌË (atorvastatin 80 mg), ÔËÎÓÊÂ̇ ‚‰̇„‡ ÒΉ ËÒıÂÏ˘ÌËfl ÌÂ͇‰ËÓÂÏ·Ó΢ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ, ̇χÎfl‚‡ ·ÚË‚ÌËfl ËÒÍ ÓÚ ‚ÚÓË àåà Ò 16%. íÂÁË ‰‡ÌÌË ÔÓÚ‚˙ʉ‡‚‡Ú ıËÔÓÚÂÁ‡Ú‡, ˜Â ÒÚ‡ÚËÌËÚ ÒÚ‡·ËÎËÁË‡Ú ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ô·͇ ‚ ‡ÌÌËfl ÒΉËÌÒÛÎÚÂÌ ÔÂËÓ‰, ͇ÍÚÓ Ë ˜Â ËÏ‡Ú Ì‚ÓÔÓÚÂÍÚË‚ÂÌ ÂÙÂÍÚ. éÒÌÓ‚‡‚‡ÈÍË Ò ̇ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ SPARCL, ÄÏÂË͇ÌÒ͇ڇ ÄÒӈˇˆËfl ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl ÔÂÔÓ˙˜‚‡ ÔË·„‡Ì ̇ ËÌÚÂÌÁ˂̇ ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËfl ÔË ·ÓÎÌËÚÂ Ò ‡ÚÂÓÒÍÎÂÓÚ˘̇ „ÂÌÂÁ‡ ̇ àåà ËÎË íàÄ, Á‡ ̇χÎfl‚‡Ì ËÒ͇ ÓÚ ËÌÒÛÎÚ ËÎË Ò˙‰Â˜ÌÓ-Ò˙‰Ó‚Ë Ë̈ˉÂÌÚË (ÍÎ‡Ò 1, ÌË‚Ó Ç), ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÚÓ‚‡, ˜Â ÌflÏ‡Ú ‰Ó͇Á‡Ì‡ àÅë (18). Ö‚ÓÔÂÈÒ͇ڇ ÄÒӈˇˆËfl ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl Ò˙˘Ó ÔÂÔÓ˙˜‚‡ ÒÚ‡ÚËÌÓ‚Ó Î˜ÂÌË Á‡ ‚ÚÓ˘̇ ÔÓÙË·ÍÚË͇ ÔË ‚Ò˘ÍË ·ÓÎÌË Ò ÌÂ͇‰ËÓÂÏ·Ó΢ÂÌ àåà (19). äÄäÇà ëÄ èêàñÖãçàíÖ çàÇÄ áÄ LDL-ïéãÖëíÖêéã äÓÎÍÓÚÓ ÔÓ-ÌËÒ͇  ÍÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ LDL-ıÓÎÂÒÚÂÓ·, ÚÓÎÍÓ‚‡ ÔÓ-Á̇˜ËÚÂÎ̇  ‰Û͈ËflÚ‡ ̇ ËÒ͇ ÔÓ ÓÚÌÓ¯ÂÌË ͇ÍÚÓ Ì‡ Ô˙‚˘̇ڇ, ڇ͇ Ë Ì‡ ‚ÚÓ˘̇ڇ Ô‚Â̈Ëfl ̇ ËÌÒÛÎÚ‡. åÂÚ‡‡Ì‡ÎËÁ˙Ú, Ôӂ‰ÂÌ ÓÚ Amarenco Ë Labreuche (3), ‚Íβ˜‚‡˘ 165 000 ‚ËÒÓÍÓËÒÍÓ‚Ë Îˈ‡ ‰ÂÏÓÌÒÚË‡, ˜Â ‚ÒÂÍË 1 mmol/l ÔÓÌËʇ‚‡Ì ̇ LDL-ıÓÎÂÒÚÂÓ· Ò ‡‚Ìfl‚‡ ̇ 21% ‰Û͈Ëfl ̇ ·ÚË‚ÌËfl ËÒÍ Á‡ ËÌÒÛÎÚ. êÂÁÛÎÚ‡ÚËÚ ÓÚ ‰Û„ ÏÂÚ‡‡Ì‡ÎËÁ (PROVE-IT, TNT, IDEAL, ALLIANCE, SEARCH) (8,20-22), ‚Íβ˜‚‡˘ 42 054 Ô‡ˆËÂÌÚË Ò àÅë ÒÓ˜‡Ú, ˜Â ‚ Ò‡‚ÌÂÌË Ò˙Ò Òڇ̉‡Ú̇ڇ ÚÂ‡ÔËfl, ËÌÚÂÌÁ˂̇ڇ ÎËÔˉÓÔÓÌËʇ‚‡˘‡ ÚÂ‡ÔËfl ̇χÎfl‚‡ ËÒ͇ ÓÚ ËÌÒÛÎÚ Ò 13%. Ç˙‚ ‚ÚÓ˘̇ڇ ÔÓÙË·ÍÚË͇ ̇ ÌÂ͇‰ËÓÂÏ·Ó΢ÌËfl ËÌÒÛÎÚ ËÌÚÂÌÁ˂̇ڇ ‰Û͈Ëfl ̇ LDL-ıÓÎÂÒÚÂÓ· ÔÓÒ‰ÒÚ‚ÓÏ ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËfl Ò˙˘Ó ‚Ó‰Ë ‰Ó ÒË„ÌËÙË͇ÌÚ̇ ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ ˆˉ˂. ÇÒflÍÓ Û‚Â΢‡‚‡Ì ̇ LDL-ıÓÎÂÒÚÂÓ· Ò 1 mmol/l Û‚Â΢‡‚‡ ËÒ͇ ÓÚ Ò˙‰Ó‚‡ ÒÏ˙Ú Ò 0,3 % (23). ÄÏÂË͇ÌÒ͇ڇ ‡ÒӈˇˆËfl ÔÓ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë, ͇ÍÚÓ Ë Ú‡ÁË ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl ÔÂÔÓ˙˜‚‡Ú ωË͇ÏÂÌÚË ÓÚ „ÛÔ‡Ú‡ ̇ ÒÚ‡ÚËÌËÚ Á‡ Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚Â̇ àÅë ËÎË ÒËÏÔÚÓχÚ˘̇ ‡ÚÂÓÒÍÎÂÓÚ˘̇ ·ÓÎÂÒÚ, ͇ÚÓ Úfl·‚‡ ‰‡ Ò ÔÓÒÚË„Ì‡Ú ÔˈÂÎÌË ÌË‚‡ Á‡ LDL-ıÓÎÂÒÚÂÓÎ ÔÓ‰ 2,6 mmol/l. ᇠԇˆËÂÌÚË Ò ÏÌÓ„Ó ‚ËÒÓÍ ËÒÍ Ë ÏÌÓÊÂÒÚ‚Ó ËÒÍÓ‚Ë Ù‡ÍÚÓË Ò ÔÂÔÓ˙˜‚‡Ú ÌË‚‡ ̇ LDL-ıÓÎÂÒÚÂÓÎ ÔÓ‰ 1,8 mmol/l. (ÍÎ‡Ò 1, ÌË‚Ó Ä) (11). éÚ ‚‡ÊÌÓ Á̇˜ÂÌË Á‡ ÔÓÙË·ÍÚË͇ڇ ̇ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ËÚ Ë̈ˉÂÌÚË Â ‰ÓÒÚË„‡ÌÂÚÓ Ì‡ ÔˈÂÎÌË ÌË‚‡ ̇ LDL-ıÓÎÂÒÚÂÓ·, ÍÓÂÚÓ Ò ÔÓÒÚË„‡ Ò ‡Á΢̇ ‰ÓÁËӂ͇ ̇ ËÁÔÓÎÁ‚‡ÌËÚ ‚ Ô‡ÍÚË͇ڇ ÒÚ‡ÚËÌË. чÌÌËÚ ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ STELLAR ‰ÂÏÓÌÒÚË‡Ú, ˜Â ÔˈÂÎÌÓÚÓ ÌË‚Ó Ì‡ LDL-ıÓÎÂÒÚÂÓÎ (<2,6 mmol/L) Ò ÔÓÒÚË„‡ ÔË Ì‡È-‚ËÒÓÍ ÔÓˆÂÌÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÍÓËÚÓ ÔÓÎÛ˜‡‚‡Ú rosuvastatin ‚ Ò‡‚ÌÂÌËÂ Ò atorvastatin, simvastatin Ë pravastatin (ÙË„.2) (24). ëíÄíàçà à äÄêéíàÑçÄ ÄíÖêéëäãÖêéáÄ ÑÓ· ËÁ‚ÂÒÚÌÓ Â, ˜Â ÒÚ‡ÚËÌËÚ ÓÒ‚ÂÌ ÎËÔˉÓÔÓÌËʇ‚‡˘Ëfl ÒË ÂÙÂÍÚ – ÓÒÌÓ‚ÌÓ ÔÓ ÓÚÌÓ¯ÂÌË ̇ LDL-ıÓ-


ÑÂÍÂÏ‚Ë 2009

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

îË„. 2. ÑflÎ Ô‡ˆËÂÌÚË (%), ÔÓÒÚ˄̇ÎË ÔÓˆÂÎÌË ÒÚÓÈÌÓÒÚË Ì‡ ıÓÎÂÒÚÂÓ·, Ò˙„·ÒÌÓ NCEP Adult Treatment Panel 3 ÎÂÒÚÂÓ·, ËÏ‡Ú Ë ‰Û„Ë ÏÌÓ„Ó ‚‡ÊÌË – ÔÎÂÈÓÚÓÔÌË Ò‚ÓÈÒÚ‚‡. ëÚ‡ÚËÌËÚ ÒÚ‡·ËÎËÁË‡Ú ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ô·͇ ÔÓ ÏÌÓ„Ó Ì‡˜ËÌË: ÔÓ‰Ó·fl‚‡Ú ẨÓÚÂÎ̇ڇ ‰ËÒÙÛÌ͈Ëfl, ËÏ‡Ú ‡ÌÚËËÌÙ·χÚÓÌÓ ‰ÂÈÒÚ‚ËÂ Ë ‡ÌÚËÔÓÎËÙÂ‡ÚË‚ÌÓ ‰ÂÈÒÚ‚ËÂ, ̇χÎfl‚‡Ú ÚÓÏ·Ó„ÂÌÌËfl ÔÓÚÂ̈ˇΠ̇ ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ô·͇. íÓ‚‡ Ó·ÛÒ·‚fl „ÓÎflχڇ ÔÓÎÁ‡ ÓÚ Úflı Á‡ ÔÓÙË·ÍÚË͇ڇ ̇ Ò˙‰Ó‚Ë Ë̈ˉÂÌÚË ÔË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â Ì‡Îˈ ‡ÚÂÓÒÍÎÂÓÚ˘ÂÌ ÔÓˆÂÒ. ÅÂÁÒÔÓÌÓ Â, ˜Â ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡  ·‡‚ÂÌ ÔÓˆÂÒ, ‡Á‚Ë‚‡˘ Ò ‚ ÔÓ‰˙ÎÊÂÌË ̇ ‰ÂÒÂÚÍË „Ó‰ËÌË, ÔÓ‡‰Ë ÍÓÂÚÓ, ‡ÍÓ Úfl·‚‡ ‰‡ Ò ӈÂÌË ‚˙Á‰ÂÈÒÚ‚ËÂÚÓ Ì‡ ‡Á΢ÌË Ù‡ÍÚÓË ‚˙ıÛ Ì„ӂ‡Ú‡ ÔÓ„ÂÒËfl,  ÌÂÓ·ıÓ‰ËÏÓ ÔÓÒΉfl‚‡Ì ̇ „ÓÎflÏ ·ÓÈ Ô‡ˆËÂÌÚË ‚ ÔÓ‰˙ÎÊÂÌË ̇ ÏÌÓ„Ó „Ó‰ËÌË. íÓ‚‡ ̇·„‡ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÔˆËÙ˘ÌË Ï‡ÍÂË Á‡ ÒÚÂÔÂÌÚ‡ ̇ ‡ÚÂÓÒÍÎÂÓÁ‡ Ë ÌÂÈ̇ڇ ÔÓ„ÂÒËfl. åÂÊ‰Û ÏÌÓ„ÓÚÓ ËÁÔÓÎÁ‚���ÌË Ú‡ÍË‚‡ χÍÂË, ̇È-‰Ó·ËflÚ ÏÂÚÓ‰ Ò ·‡ÁË‡ ̇ ËÁÏÂ‚‡ÌÂÚÓ Ì‡ ͇ÓÚˉÌËfl ËÌÚËχ-ωËfl ÍÓÏÔÎÂÍÒ (intima-media thickness, IMT). íÓÁË Ï‡ÍÂ Ò ËÁÏÂ‚‡ ÔË ‰ÛÔÎÂÍÒ ÒÍÂÌË‡ÌÂ, Í˙‰ÂÚÓ ÒÚÂ̇ڇ ̇ Ó·˘‡Ú‡ Ò˙Ì̇ ‡ÚÂËfl Ëχ ı‡‡ÍÚÂÂÌ Ó·‡Á – Ò ‰‚ ÂıÓ„ÂÌÌË ÔÓ‚˙ıÌÓÒÚË. Ç˙Ú¯̇ڇ ÔÓ‚˙ıÌÓÒÚ ÓÚ„Ó‚‡fl ̇ „‡Ìˈ‡Ú‡ Í˙‚ - ËÌÚËχ, ‡ ‚˙̯̇ڇ - ̇ „‡Ìˈ‡Ú‡ ωËfl – ‡‰‚ÂÌÚˈËfl. IMT Ò ۂÂ΢‡‚‡ ÔË Ô‡ˆËÂÌÚË Ò ˆÂ·Ó‚‡ÒÍÛ·ÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË: ıËÔÂıÓÎÂÒÚÂÓÎÂÏËfl, Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ, ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl. ì‚Â΢‡‚‡ÌÂÚÓ Ì‡ IMT  ·Â΄ Á‡ ‡Ì̇ ‡ÚÂÓÒÍÎÂÓÁ‡, Á‡ ÒÚÂÔÂÌÚ‡ ̇ ÌÂÈÌÓÚÓ ‡Á‚ËÚËÂ Ë ÔÓ„ÂÒËfl Ë Â ÌÂÁ‡‚ËÒËÏ Ô‰ËÍÚÓ ̇ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl (25-30). åÌÓÊÂÒÚ‚Ó ÔÓÛ˜‚‡ÌËfl (31-40) Ò‡ ÔÓÒ‚ÂÚÂÌË Ì‡ ËÁÒΉ‚‡Ì ‚˙Á͇ڇ ÏÂÊ‰Û ‰Â·ÂÎË̇ڇ ̇ IMT Ë ÒÚ‡ÚËÌÓ‚ÓÚÓ Î˜ÂÌËÂ. èÓ˜ÚË ‚Ò˘ÍË ÔÓ͇Á‚‡Ú Á‡·‡‚flÌ ÔÓ„ÂÒËflÚ‡ ̇ IMT Ò‰ ÎÂÍÛ‚‡ÌËÚ ·ÓÎÌË ‚ Ò‡‚ÌÂÌËÂ Ò Ô·ˆÂ·Ó. åÂÚ‡‡Ì‡ÎËÁ (41), Ó·Ó·˘‡‚‡˘ ÂÁÛÎÚ‡ÚËÚ ÓÚ ‰Â‚ÂÚ ÓÚ Úflı, ÔÓ͇Á‚‡ ̇΢ËÂÚÓ Ì‡ ÒÚÓ„‡ ÍÓ·ˆËfl ÏÂÊ‰Û ÒÚÂÔÂÌÚ‡ ̇ ‰Û͈Ëfl ̇ LDL- ıÓÎÂÒÚÂÓ· Ë Ú‡ÁË Ì‡ IMT (r=0.65; P=0.004). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ‚ÒflÍÓ ‰ÂÒÂÚÔÓˆÂÌÚÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ LDL-ıÓÎÂÒÚÂÓ· ̇χÎfl‚‡ IMT Ò 0,73% ̇ „Ó‰Ë̇. èӂ˜ÂÚÓ ÓÚ ÚÂÁË ÔÓÛ˜‚‡ÌËfl ‚Íβ˜‚‡Ú ‚ËÒÓÍÓËÒÍÓ‚Ë Ô‡ˆËÂÌÚË Ò Ôӂ˯ÂÌ LDL-ıÓ-

146

ÎÂÒÚÂÓÎ ËÎË Ú‡ÍË‚‡, ÔÂ͇‡ÎË àåà. Ç ÔÓÛ˜‚‡ÌÂÚÓ METEOR (42) Ò ËÁÔÓÎÁ‚‡ B-mode ÛÎÚ‡Á‚ÛÍ Á‡ ‰‡ Ò ÓÔ‰ÂÎË ‰‡ÎË Î˜ÂÌËÂÚÓ Ò rosuvastatin ÏÓÊ ‰‡ Á‡·‡‚Ë ÔÓ„ÂÒËflÚ‡ ËÎË ‰‡ Ô‰ËÁ‚Ë͇ „ÂÒËfl ̇ ͇ÓÚˉ̇ڇ IMT ÔË ‡ÒËÏÔÚÓχÚ˘ÌË Ô‡ˆËÂÌÚË Ò ÌËÒ˙Í Ò˙‰Ó‚ ËÒÍ. ã˜ÂÌËÂÚÓ Ò rosuvastatin 40 mg Á‡ 2-„Ӊ˯ÂÌ ÔÂËÓ‰ Á̇˜ËÏÓ Á‡·‡‚fl ÒÚÂÔÂÌÚ‡ ̇ ‡Á‚ËÚË ̇ IMT ‚ Ò‡‚ÌÂÌËÂ Ò Ô·ˆÂ·Ó. èÓÛ˜‚‡ÌÂÚÓ METEOR  ‚‡ÊÌÓ, Ú.Í.  ‰ËÌÒÚ‚ÂÌÓÚÓ Ô·ˆÂ·Ó ÍÓÌÚÓÎË‡ÌÓ, ÍÓÂÚÓ ÔÓ͇Á‚‡ ÔÓÁËÚË‚ÂÌ ÂÙÂÍÚ ‚˙ıÛ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡ ÔË Ô‡ˆËÂÌÚË Ò ÚÓÎÍÓ‚‡ ‡ÌÌË ·ÂÎÂÁË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë Ò ÌËÒ˙Í ËÒÍ ÓÚ ÍÓÓ̇ÌË Ë̈ˉÂÌÚË (Framingham 10 „Ӊ˯ÂÌ ËÒÍ < 10%). Rosuvastatin ÔÓÌËʇ‚‡ LDL-ıÓÎÂÒÚÂÓ· Ò 48,8% Ë ÔÓ͇˜‚‡ HDL–ıÓÎÂÒÚÂÓ· Ò 8,0% ÔÓ ÓÚÌÓ¯ÂÌË ̇ ËÁıÓ‰ÌËÚ ÒÚÓÈÌÓÒÚË. íÓ Ì ÓÚ˜ËÚ‡ „ÂÒËfl ̇ ‡ÚÂÓÒÍÎÂÓÚ˘ÌËfl ÔÓˆÂÒ ‚ ͇ÓÚˉÌËÚ ‡ÚÂËË, Á‡ ‡ÁÎË͇ ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ ASTEROID, ÍÓÂÚÓ ÔÓ͇Á‚‡ „ÂÒËfl ̇ ÍÓÓ̇̇ڇ ‡ÚÂÓÒÍÎÂÓÁ‡ ÔË ÔËÎÓÊÂÌË ̇ rosuvastatin 40 mg, ÓÚ˜ÂÚÂ̇ ˜ÂÁ ËÌÚ‡‚‡Á‡ÎÌÓ ÛÎÚ‡Á‚ÛÍÓ‚Ó ËÁÒΉ‚‡Ì (43). 臈ËÂÌÚËÚ ÓÚ ASTEROID Ò‡ Ò ‡ÚÂÓÒÍÎÂÓÚ˘ÂÌ ÔÓˆÂÒ ‚ ̇Ô‰̇· Ù‡Á‡, ÍÓÂÚÓ ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ÓÚ˜ÂÚÂ Ë „ÂÒËflÚ‡, ‰Ó͇ÚÓ Ú‡Í˙‚ ÎËÔÒ‚‡ ‚ METEOR. óÂÁ ÌÂËÌ‚‡ÁË‚ÌË ËÁÒΉ‚‡ÌËfl ̇ ͇ÓÚˉÌËÚ ‡ÚÂËË (‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl, fl‰ÂÌÓχ„ÌËÚÂÌ ÂÁÓ̇ÌÒ) Ò ÔÓÎÛ˜‡‚‡ ËÌÙÓχˆËfl Á‡ ̇΢ËÂÚÓ, „ÓÎÂÏË̇ڇ, ÏÂÒÚÓÔÓÎÓÊÂÌËÂÚÓ Ë ÒÚÛÍÚÛ‡Ú‡ ̇ ‡ÚÂÓÒÍÎÂÓÚ˘ÌËÚ Ô·ÍË. èÓÒΉÌËÚ ËÏ‡Ú ‡Á΢ÂÌ Ò˙ÒÚ‡‚, ÔÓ‡‰Ë ÍÓÂÚÓ ˜ÂÁ Ç-mode Ò ‚ËÁÛ‡ÎËÁË‡Ú ͇ÚÓ ıËÔÂÂıÓ„ÂÌÌË, ıËÔÓÂıÓ„ÂÌÌË ËÎË ıÂÚÂÓ„ÂÌÌË. ë˜ËÚ‡ ÒÂ, ˜Â ÓÒÌÓ‚ÌËflÚ Ù‡ÍÚÓ ÍÓÈÚÓ ÓÔ‰ÂÎfl Ô·ÍÓ‚‡Ú‡ ÒÚ‡·ËÎÌÓÒÚ,  Ô·ÍÓ‚‡Ú‡ ÒÚÛÍÚÛ‡, ‡ Ì ÚÓÎÍÓ‚‡ Ô·ÍÓ‚‡Ú‡ „ÓÎÂÏË̇ (44). ïËÔÂÂıÓ„ÂÌÌËÚ Ô·ÍË Ò‡ ·Ó„‡ÚË Ì‡ ÙË·ËÌ Ë Í‡ÎˆËÂ‚Ë ÓÚ·„‡ÌËfl. ïËÔÓÂıÓ„ÂÌÌËÚ Ô·ÍË ËÏ‡Ú ·Ó„‡ÚÓ ÎËÔˉÌÓ Ò˙‰˙ʇÌËÂ Ë Ì‡Î˘Ë ̇ ÌÂÍÓÚ˘ÌË Û˜‡ÒÚ˙ˆË. í ҇ ÔÓ-‡ÌËÏË, Ò ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ÛÔÚÛ‡ Ë ÎÂÒÌÓ Ò Ô‚˙˘‡Ú ‚ ËÁÚÓ˜ÌËÍ Ì‡ ÏÓÁ˙˜ÂÌ ÂÏ·ÓÎËÁ˙Ï. 臈ËÂÌÚËÚÂ Ò ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË ËÏ‡Ú ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ àåà, ÔÓ-‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ ‡ÒËÏÔÚÓÏÌË ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË Ë ÍÓÓ̇ÌË Ë̈ˉÂÌÚË. ëÌËʇ‚‡ÌÂÚÓ Ì‡ HDL- ıÓÎÂÒÚÂÓ· Û‚Â΢‡‚‡ ËÒ͇ ÓÚ Ó·‡ÁÛ‚‡Ì ̇ ıËÔÓÂıÓ„ÂÌÌË Ô·ÍË Ì‡‰ 8 Ô˙ÚË (45-48). çflÍÓÎÍÓ Ï‡ÎÍË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ΘÂÌËÂÚÓ Ò˙Ò ÒÚ‡ÚËÌË ‚Ó‰Ë ‰Ó ÔÓÏfl̇ ‚ Ô·ÍÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëfl (48-51). ç‡Ï‡Îfl‚‡ÌÂÚÓ Ì‡ Ô·ÁÏÂÌËÚ ÌË‚‡ ̇ ıÓÎÂÒÚÂÓ· Ô‰ËÁ‚ËÍ‚‡ ÔÓÏÂÌË „·‚ÌÓ ‚ ÍÓÏÔÓÌÂÌÚËÚ ̇ Ô·ÍËÚ Ò˙Ò Á̇˜ËÚÂÎÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ ÎËÔˉÌÓÚÓ ËÏ Ò˙‰˙ʇÌËÂ Ë ÔÓ-χÎÍÓ ‚ÎËflÌË ‚˙ıÛ ÙË·ÓÁ̇ڇ ËÏ Ó·‚˂͇. í‡Í‡ ̇ÔËÏÂ ÔÓÒ‰ÒÚ‚ÓÏ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl  ÓÚ˜ÂÚÂÌÓ Û‚Â΢‡‚‡Ì ̇ Ô·ÍÓ‚‡Ú‡ ÂıÓ„ÂÌÌÓÒÚ ÒΉ ÔÓ‰˙ÎÊËÚÂÎÌÓ Î˜ÂÌË (50). ÑÛ„Ó ÌÓ‚Ó Ï‡ÎÍÓ ÔÓÛ˜‚‡Ì ‰ÂÏÓÌÒÚË‡, ˜Â ËÌÚÂÌÁ˂̇ڇ ÎËÔˉÓÔÓÌËʇ‚‡˘‡ ÚÂ‡ÔËfl Á‡ 6-ÏÂÒ˜ÂÌ ÔÂËÓ‰ ÓÚ ‚ÂÏ ÔË ·ÓÎÌË Ò Ì‡‰ 40% ÒÚÂÌÓÁË ·ÂÁ Ë̉Ë͇ˆËË Á‡ ËÌÚÂ‚Â̈Ëfl, ÔÓ‰Ó·fl‚‡ Ô·ÍÓ‚‡Ú‡ ÂıÓ„ÂÌÌÓÒÚ, ÓÚ˜ÂÚÂ̇ ˜ÂÁ GSM (GrayScale Median) (51). àÁÔÓÎÁ‚‡ÈÍË fl‰ÂÌÓχ„ÌËÚÂÌ ÂÁÓ̇ÌÒ R. Hunter et al. (52) ÔÓ͇Á‚‡Ú, ˜Â 24 ÏÂÒ˜ÌÓÚÓ Î˜ÂÌËÂ Ò rosuvastatin ‚Ó‰Ë ‰Ó ̇χÎfl‚‡Ì ̇ ‚˙ÚÂÔ·ÍÓ‚ËÚ ÎËÔˉÌË ÌÂÍÓÚ˘ÌË fl‰‡. èË ËÏÛÌÓıËÒÚÓıËÏ˘ÂÒÍÓ ËÁÒΉ‚‡Ì ̇ ÓÚ‰ÂÎÂÌËÚ ÔË Í‡ÓÚˉ̇ Ẩ‡ÚÂÂÍÚÓÏËfl Ô·ÍË Â ÛÒÚ‡ÌÓ‚Â̇ Á̇˜Ëχ ‡ÁÎË͇ ÔÓ ÓÚÌÓ¯ÂÌË ËÌÙËÎÚ‡ˆËflÚ‡ ÓÚ Ï‡ÍÓÙ‡„Ë Ë ÂÍÒÔÂÒËflÚ‡ ̇ χÚËÍÒ̇ڇ ÏÂÚ‡ÎÓÔÓÚÂË̇Á‡ (53, 54). 臈ËÂÌÚËÚ ÔÓ‚Âʉ‡ÎË ÒÚ‡ÚËÌÓ‚Ó Î˜ÂÌËÂ, ËÏ‡Ú ÒË„ÌËÙË͇ÌÚÌÓ ÔÓ-χÎ˙Í ÔÓˆÂÌÚ Ì‡ ËÌÙËÎÚ‡ˆËfl ÓÚ Ï‡ÍÓÙ‡„Ë Ë ÂÍÒÔÂÒËfl ̇ χÚËÍÒ̇ڇ ÏÂÚ‡ÎÓÔÓÚÂË̇Á‡ ‚ Ò‡‚ÌÂÌËÂ Ò ÌÂÎÂÍÛ‚‡ÌË ·ÓÎÌË. íÂÁË ‰‚‡


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

ÑÂÍÂÏ‚Ë 2009

ÔÓ͇Á‡ÚÂÎfl Ò‡ χÍÂË Á‡ ‚ËÒÓÍÓËÒÍÓ‚Ë ‡ÚÂÓÒÍÎÂÓÚ˘ÌË Ô·ÍË Ë Ô·ÍÓ‚‡ ÌÂÒÚ‡·ËÎÌÓÒÚ. êÂÁÛÎÚ‡ÚËÚ ÔÓÚ‚˙ʉ‡‚‡Ú ÚÓ‚‡, ˜Â ÒÚ‡ÚËÌËÚ ‚ÎËflflÚ Ì ҇ÏÓ ‚˙ıÛ ÎËÔˉÌÓÚÓ Ò˙‰˙ÊËÏÓ, ÌÓ ÔÓ‚ÎËfl‚‡Ú Ë ËÌÙ·χÚÓÌËÚÂ Ë „·‰ÍÓÏÛÒÍÛÎÌË ÍÎÂÚÍË, ÏÂÚ‡ÎÓÔÓÚÂË̇Á̇ڇ ‡ÍÚË‚ÌÓÒÚ Ë ÓÍÒˉ‡ÚË‚ÌËfl ÒÚÂÒ. èÓÛ˜‚‡ÌÂÚÓ SPARCLE ÔÓ͇Á‚‡ ÔÓ-„ÓÎflχ ÔÓÎÁ‡ ÔÓ ÓÚÌÓ¯ÂÌË ‰Û͈Ëfl ̇ ËÒ͇ ÓÚ àåà ÔË ·ÓÎÌËÚÂ Ò Í‡ÓÚˉÌË ÒÚÂÌÓÁË, ͇ÚÓ ÒÚ‡ÚËÌÓ‚ÓÚÓ Î˜ÂÌË ̇χÎfl‚‡ ·ÚË‚ÌËfl ËÒÍ Ò 33%. áÄäãûóÖçàÖ ëÚ‡ÚËÌËÚ ËÏ‡Ú ‡Á΢ÌË ÂÙÂÍÚË ‚˙ıÛ ‡ÚÂÓÒÍÎÂÓÚ˘̇ڇ Ô·͇, ͇ÚÓ ÔÓÏÂÌËÚ ‚ ẨÓÚÂÎ̇ڇ ‰ËÒÙÛÌ͈Ëfl Ë Ô·ÍÓ‚‡Ú‡ ÒÚÛÍÚÛ‡ ‰ÓÔË̇ÒflÚ Á‡ Ô‚‡ÌÚË‚ÌÓÚÓ ËÏ ‰ÂÈÒÚ‚Ë ÔË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ËÚ Á‡·ÓÎfl‚‡ÌËfl. èÓÙË·ÍÚË͇ڇ Ò˙Ò ÒÚ‡ÚËÌË Ëχ ¯ËÓÍÓ ÔËÎÓÊÂÌË ͇ÍÚÓ ‚ Ô˙‚˘̇ڇ, ڇ͇ Ë ‚˙‚ ‚ÚÓ˘̇ڇ ÔÓÙË·ÍÚË͇ ̇ åëÅ. ífl  ̇È-‚‡ÊÌÓÚÓ ÔÓÒÚËÊÂÌË ‚ Ô‚Â̈ËflÚ‡ ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ ÓÚ ‚˙‚Âʉ‡ÌÂÚÓ Ì‡ ‡ÒÔËËÌ Ë ‡ÌÚËıËÔÂÚÂÌÁË‚ÌÓÚÓ Î˜ÂÌËÂ. ãàíÖêÄíìêÄ 1. 2.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ‰Óˆ. ‰- Ö‚„ÂÌËfl LJÒË΂‡, ‰.Ï. äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇”, ëÓÙËfl i-mail: iruschev@yahoo.com


ÑÂÍÂÏ‚Ë 2009

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

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é·ÁÓ ÇÄêàÄÅàãçéëí çÄ ëöêÑÖóçÄíÄ óÖëíéíÄ: åÖíéÑ áÄ éñÖçäÄ çÄ ëöêÑÖóçÄíÄ ÄÇíéçéåçÄ êÖÉìãÄñàü èêà çÖÇêéãéÉàóçà áÄÅéãüÇÄçàü ë. å‡ÌÚ‡Ó‚‡, ç. ÑËÏËÚÓ‚, à. ÇÂΘ‚‡ ç‚ÓÎӄ˘̇ ÍÎËÌË͇, ëÔˆˇÎËÁË‡Ì‡ ·ÓÎÌˈ‡ ÔÓ Ì‚ÓÎÓ„Ëfl Ë ÔÒËıˇÚËfl ◊ë‚. ç‡ÛÏ”, å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ - ëÓÙËfl SUMMARY HEART RATE VARIABILITY: A METHOD FOR ASSESSMENT OF CARDIAC AUTONOMIC REGULATION IN NEUROLOGICAL DISEASES S. Mantarova, N. Dos. Dimitrov, I. Velcheva Dept of Neurology, University Hospital of Neurology and Psychiatry “St. Naum”, Medical University- Sofia The heart rate variability (HRV) is a noninvasive and reliable method with high reproducibility, by which, through measuring the variations of the heart intervals in short-term or 24-hour records the sympathovagal balance at sinoatrial level is estimated. The standard clinical parameters of the method are measurements in the frequency and time domain. Recently HRV is examined not only at rest but also after applying of different stimuli: deep breathing, body tilting, mental tasks, ect). A number of studies have shown certain diagnostic value of HRV in patients with diabetic neuropathy, Parkinson disease, stroke and multiple sclerosis. Its evaluation contributes for detecting early asymptomatic cardiac autonomic dysfunctions and correlates with basic clinical symptoms of the diseases: the hemoglobin A1C in diabets mellitus, the severity of the movement disorders in Parkinson`s disease, the degree of fatigue in multiple sclerosis, the type of the seizures in epilepsy. The impairment of the HRV is associated with increased risk of cardiac rhythm disturbances and of sudden cardiac death. The changes in HRV are associated with impairment of the cerebral autonomic structures and disturbance of their descending projections and neurotransmitter systems. The elucidation of their diagnostic value in different neurological diseases needs further studies. KEY WORDS: cardiac autonomic regulation, heart rate variability, neurological diseases. êÖáûåÖ Ç‡ˇ·ËÎÌÓÒÚÚ‡ ̇ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ (Çëó)  ÌÂËÌ‚‡ÁË‚ÂÌ Ë Ì‡‰ÂʉÂÌ ÏÂÚÓ‰ Ò ‚ËÒÓ͇ ÂÔÓ‰ÛÍÚË‚ÌÓÒÚ, ÔË ÍÓÈÚÓ ˜ÂÁ ËÁÏÂ‚‡Ì ̇ ‚‡ˇˆËËÚ ̇ Ò˙‰Â˜ÌËÚ ËÌÚÂ‚‡ÎË ÔË Í‡ÚÍÓÚ‡ÈÌË ËÎË ÔÓ‰˙ÎÊËÚÂÎÌË Á‡ÔËÒË Ò ÓÔ‰ÂÎfl ÒËÏÔ‡ÚËÍÓ‚‡„‡ÎÌËfl ·‡Î‡ÌÒ Ì‡ ÒËÌÓ‡ÚˇÎÌÓ ÌË‚Ó. ëڇ̉‡ÚÌËÚ ÍÎËÌ˘ÌË Ô‡‡ÏÂÚË Ì‡ ÏÂÚÓ‰‡ Ò‡ ËÁÏÂ‚‡ÌËfl ‚ ˜ÂÒÚÓÚ̇ڇ Ë ‚˙‚ ‚ÂÏ‚‡Ú‡ ӷ·ÒÚ. ç‡ÔÓÒΉ˙Í Çëó Ò ËÁÒΉ‚‡ Ì ҇ÏÓ ‚ ÔÓÍÓÈ, ÌÓ Ë ÒΉ ÔË·„‡Ì ̇ ‡Á΢ÌË ÒÚËÏÛÎË (‰˙ηÓÍÓ ‰Ë¯‡ÌÂ, ÔÓ‚‰Ë„‡Ì ̇ ÚflÎÓÚÓ ÔÓ‰ ÓÔ‰ÂÎÂÌ ˙„˙Î Ë ‰Û„Ë). ÉÓÎflÏ ·ÓÈ ÔÓÛ˜‚‡ÌËfl Ò‡ ÔÓ͇Á‡ÎË ‰Ë‡„ÌÓÒÚ˘̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ Çëó ÔË ·ÓÎÌË Ò ‰Ë‡·ÂÚ̇ Ì‚ÓÔ‡ÚËfl, Ô‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ë ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡. çÂÈÌÓÚÓ ËÁÒΉ‚‡Ì ‰ÓÔË̇Òfl Á‡ ÓÚÍË‚‡Ì ̇

‡Ì̇ ‡ÒËÏÔÚÓÏ̇ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl Ë ÍÓÂÎË‡ Ò ÓÒÌÓ‚ÌË ÍÎËÌ˘ÌË ÔÓfl‚Ë Ì‡ Á‡·ÓÎfl‚‡ÌËflÚ‡: ÌË‚Ó Ì‡ ıÂÏÓ„ÎÓ·ËÌ A1C ÔË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, ÚÂÊÂÒÚ Ì‡ ‰‚Ë„‡ÚÂÎÌËÚ ̇Û¯ÂÌËfl ÔË Ô‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ‚ˉ ̇ ÔËÒÚ˙ÔËÚ ÔË ÂÔËÎÂÔÒËfl, ÒË̉ÓÏ Ì‡ ÛÏÓ‡ ÔË ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡. ç‡Î˘ËÂÚÓ ËÏ Â Ò‚˙Á‡ÌÓ Ò Ôӂ˯ÂÌ ËÒÍ ÓÚ Ò˙‰Â˜ÌË ËÚ˙ÏÌË Ì‡Û¯ÂÌËfl Ë ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. èÓÏÂÌËÚ ‚˙‚ Çëó Ò‡ Ò‚˙Á‡Ì‡ Ò Û‚Âʉ‡Ì ̇ ÏÓÁ˙˜ÌËÚ ‡‚ÚÓÌÓÏÌË ÒÚÛÍÚÛË Ë Ì‡Û¯‡‚‡Ì ̇ ÚÂıÌËÚ ‰ÂÒˆẨÂÌÚÌË ÔÓÂ͈ËË Ë ÙÛÌ͈ËÓ̇ÎÌË ‚˙ÁÍË. Ç „ÂÌÂÁ‡Ú‡ ËÏ Û˜‡ÒÚ‚‡Ú Ë Ì‡Û¯ÂÌËfl ‚ ËÏÛÌ̇ڇ Ë Ì‚ÓÚ‡ÌÒÏËÚÂÌËÚ ÒËÒÚÂÏË. àÁflÒÌfl‚‡ÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÒÚ˘̇ڇ ËÏ ÒÚÓÈÌÓÒÚ ÔË ÓÚ‰ÂÎÌËÚ Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl ̇·„‡ ÔÓ‚Âʉ‡Ì ̇ ÔÓ-̇ڇÚ˙¯ÌË ËÁÒΉ‚‡ÌËfl. äãûóéÇà Ñìåà: ‚‡ˇ·ËÎÌÓÒÚ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡, Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl, Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏ̇ „Û·ˆËfl. ë˙‰Â˜ÌËflÚ ËÚ˙Ï ÔÓ‰ÎÂÊË Ì‡ ÒËÏÔ‡ÚËÍÓ‚ Ë Ô‡‡ÒËÏÔ‡ÚËÍÓ‚ ÍÓÌÚÓÎ ÏÓ‰ÛÎË‡Ì ÓÚ ˆÂÌÚ‡ÎÌË Ë ÔÂËÙÂÌË ‡‚ÚÓÌÓÏÌË ÒÚÛÍÚÛË (1). èÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË Á‡ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ Ò˙‰Â˜ÌËÚ ‡‚ÚÓÌÓÏÌË ‰ËÒÙÛÌ͈ËË Ò ÔË·„‡Ú ÌÂËÌ‚‡ÁË‚ÌË ËÁÒΉ‚‡ÌËfl, ·‡ÁË‡ÌË Ì‡ ‡Ì‡ÎËÁ‡ ̇ ÓÒÌÓ‚ÌË ÙËÁËÓÎӄ˘ÌË Ô‡‡ÏÂÚË. í‡ÍË‚‡ Ò‡ ‚‡ˇ·ËÎÌÓÒÚ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ (Çëó), ·‡ÓÂÙÎÂÍÒ̇ ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ, ‰˙ÎÊË̇ ̇ QT ËÌÚÂ‚‡Î‡, ÍÓÒÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ Ë ‰. éˆÂÌ͇ڇ ̇ Çëó  ·˙Á, ÌÂËÌ‚‡ÁË‚ÂÌ, ÎÂÒÌÓ ‰ÓÒÚ˙ÔÂÌ Ë Ì‡‰ÂʉÂÌ ÏÂÚÓ‰ Ò ‚ËÒÓ͇ ÂÔÓ‰ÛÍÚË‚ÌÓÒÚ, ˜ÂÁ ÍÓÈÚÓ ÏÓÊ ‰‡ Ò ÓÔ‰ÂÎË ÒËÏÔ‡ÚËÍÓ‚‡„‡ÎÌËfl ·‡Î‡ÌÒ Ì‡ ÒËÌÓ‡ÚˇÎÌÓ ÌË‚Ó (60). íÓÈ Ò ËÁÔÓÎÁ‚‡ ÛÚËÌÌÓ Í‡ÍÚÓ ‚ ͇‰ËÓÎӄ˘̇ڇ Ô‡ÍÚË͇, ڇ͇ Ë ÔË ‰ˈ‡ Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl ÔË ÍÓËÚÓ Ò ̇·Î˛‰‡‚‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl – ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ÂÔËÎÂÔÒËfl, Ô‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl Ë ‰. óÂÒÚÓ ÛÒÚ‡ÌÓ‚ÂÌËÚ ÌÛ¯ÂÌËfl ÍÓÂÎË‡Ú Ò ÚÂÊÂÒÚÚ‡ Ë ÔÓ„ÌÓÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (1). Ä̇ÎËÁ˙Ú Ì‡ Çëó Ò ÓÒÌÓ‚‡‚‡ ̇ ÒÂËfl ÓÚ ËÁÏÂ‚‡ÌËfl ̇ ‚‡ˇˆËËÚ ‚ Ò˙‰Â˜ÌËÚ ËÌÚÂ‚‡ÎË Ò˙Ò ÒËÌÛÒÓ‚ ÔÓËÁıÓ‰, ÍÓËÚÓ Ò ӷÓÁ̇˜‡‚‡Ú ͇ÚÓ NN ËÌÚÂ‚‡ÎË. åÓ„‡Ú ‰‡ Ò ËÁÔÓÎÁ‚‡Ú ÔÓ‰˙ÎÊËÚÂÎÌË 24 ˜‡ÒÓ‚Ë ıÓÎÚÂ Á‡ÔËÒË ËÎË ÔÓ – Í˙ÒË ÂÎÂÍÚÓ͇‰ËÓ„‡ÙÒÍË Á‡ÔËÒË ‚ ‡ÏÍËÚ ÓÚ 0.5 ‰Ó 5 ÏËÌÛÚË, ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ˆÂÎËÚ ̇ ËÁÒΉ‚‡ÌÂÚÓ (60). ê‡Á΢ÌË ÙËÁËÓÎӄ˘ÌË Ù‡ÍÚÓË Í‡ÚÓ ÔÓÎ, ‚˙Á‡ÒÚ, ÚÂÎÂÒÌÓ Ú„ÎÓ, ÂÚÌÓÒ, ‰Ë¯‡ÌÂ, ÙËÁ˘ÂÒ͇ ‡ÍÚË‚ÌÓÒÚ, ‚ÂÏ ÓÚ ‰ÂÌÓÌÓ˘ËÂÚÓ, ÔÓÎÓÊÂÌË ̇ ÚflÎÓÚÓ, ÔËÂÏ Ì‡ ωË͇ÏÂÌÚË, ÂÏÓˆËË Ë ‰Û„Ë ÏÓ„‡Ú ‰‡ Ó͇Á‚‡Ú ‚ÎËflÌË ‚˙ıÛ ËÁÒΉ‚‡ÌËÚ ԇ‡ÏÂÚË(1,10). Çëó  ÔÓ- ÌËÒ͇ ÔË ÊÂÌË, ÔË Îˈ‡ Ò Ì‡‰ÌÓÏÂÌÓ Ú„ÎÓ ËÎË ‚ ̇Ô‰Ì���· ‚˙Á‡ÒÚ (10, 57). èÂÁ 1996 í‡ÒÍ Force of European Society of Cardiology Ë North American Society of Pacing and Electrophysiology


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

ÑÂÍÂÏ‚Ë 2009

ÔËÂı‡ Òڇ̉‡ÚË Á‡ ËÁÏÂ‚‡ÌÂ, ËÌÚÂÔÂÚ‡ˆËfl Ë ÍÎËÌ˘ÌÓ ÔËÎÓÊÂÌË ̇ Çëó. ëڇ̉‡ÚÌËÚ ÍÎËÌ˘ÌË Ô‡‡ÏÂÚË Ò‡ ËÁÏÂ‚‡ÌËfl ‚ ˜ÂÒÚÓÚ̇ڇ Ë ‚˙‚ ‚ÂÏ‚‡Ú‡ ӷ·ÒÚ, ͇ÍÚÓ Ë „ÂÓÏÂÚ˘ÌË ÔÓ͇Á‡ÚÂÎË (61). ç‡ Ú‡·Îˈ‡ 1. Ò‡ Ô‰ÒÚ‡‚ÂÌË Ì‡È – ˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚ ‚ÂÏÂ‚Ë Ë ˜ÂÒÚÓÚÌË Ô‡‡ÏÂÚË Ë Úflı̇ڇ ı‡‡ÍÚÂËÒÚË͇. ëÔÂÍÚ‡ÎÌËflÚ ‡Ì‡ÎËÁ ̇ ˜ÂÒÚÓÚÌËÚ ԇ‡ÏÂÚË Ò ÓÒ˙˘ÂÒÚ‚fl‚‡ ˜ÂÁ ÌÂÔ‡‡ÏÂÚ˘ÌË – ·˙Á‡ Ú‡ÌÒÙÓχˆËfl ̇ îÛËÂ Ë Ô‡‡ÏÂÚ˘ÌË ÏÂÚÓ‰Ë - ‡‚ÚÓ„ÂÒË‚ÂÌ ‡Ì‡ÎËÁ. è‡‡ÏÂÚËÚ ÓÚ ˜ÂÒÚÓÚ̇ڇ ӷ·ÒÚ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú Í·ÒËÙˈË‡ÌË ‚ ˜ÂÚËË Í‡Ú„ÓËË ÛÎÚ‡ÌËÒÍË (UVL), ÏÌÓ„Ó ÌËÒÍË (VLF), ÌËÒÍË (LF) Ë ‚ËÒÓÍË (HF) ˜ÂÒÚÓÚË. (퇷Îˈ‡ 1). èË Í‡ÚÍÓÚ‡ÈÌËÚ ËÁÒΉ‚‡ÌËfl Ò ËÁÔÓÎÁ‚‡Ú VLF, LF Ë HF, ‰Ó͇ÚÓ ÔË ‰˙΄ÓÚ‡ÈÌËÚ Ò ‚Íβ˜‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ UVL. àÁ˜ËÒÎfl‚‡Ú ÒÂ Ò ÔÓÏÓ˘Ú‡ ̇ ‡Á΢ÌË ÍÓÏÔ˛Ú˙ÌË ÔÓ„‡ÏË (îË„Û‡ 1). LF Ë HF ÏÓ„‡Ú ‰‡ Ò Ô‰ÒÚ‡‚flÚ ‚ ‡·ÒÓβÚÌË ËÎË ‚ ÌÓχÎËÁË‡ÌË Â‰ËÌˈË. èÓÒΉÌËÚ ҇ ÔÓÎÂÁÌË ÔË ÓˆÂÌ͇ ‚ÎËflÌËÂÚÓ Ì‡ ‡Á΢ÌË ÒÚËÏÛÎË ËÎË ÔË Ò‡‚ÌËÚÂÎÂÌ ‡Ì‡ÎËÁ ̇ ‰‡ÌÌË ÓÚ Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Ò ̇·Î˛‰‡‚‡ „ÓÎflχ ‡ÁÎË͇ ‚ Ó·˘ËÚ ÏÓ˘ÌÓÒÚË Ì‡ ÒÔÂÍÚ˙‡. ÇÒ ÔÓ – ˜ÂÒÚÓ Ò ËÁÒΉ‚‡ Çëó Ì ҇ÏÓ ‚ ÔÓÍÓÈ, ÌÓ Ë ÒΉ ÔË·„‡Ì ̇ ‡Á΢ÌË ÒÚËÏÛÎË – ‰˙ηÓÍÓ ‰Ë¯‡ÌÂ, ÔÓ‚‰Ë„‡Ì ̇ ÚflÎÓÚÓ ÔÓ‰ ÓÔ‰ÂÎÂÌ ˙„˙Î, ÔË ÙËÁ˘ÂÒÍÓ ÛÒËÎËÂ, ÒÎÛıÓ‚Ë Ë ÁËÚÂÎÌË ÒÚËÏÛÎË, ÏÂÌÚ‡ÎÌË Á‡‰‡˜Ë, ÔË Ù‡χÍÓÎӄ˘ÌÓ ‚˙Á‰ÂÈÒÚ‚Ë ËÎË ‚ ÍÓÏ·Ë̇ˆËfl Ò˙Ò ÒˆËÌÚË„‡ÙÒÍË ÏÂÚÓ‰Ë (60). ëÔÂÍÚ‡ÎÌËflÚ ‡Ì‡ÎËÁ ̇ Çëó  ̇‰ÂʉÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ‡‚ÚÓÌÓÏ̇ڇ ‰Ë‡·ÂÚ̇ ‰ËÒÙÛÌ͈Ëfl (49). ë ÔË·„‡ÌÂÚÓ ÏÛ ÔË ‰Âˆ‡ ·ÓÎÌË ÓÚ ‰Ë‡·ÂÚ ÏÓÊ ‰‡ ÓÚÍËflÚ ‡ÌÌË ‡ÒËÏÔÚÓÏÌË ‡‚ÚÓÌÓÏÌË Ì‡Û¯ÂÌËfl, ÍÓËÚÓ Ò‡ Ô‰ËÏÌÓ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë Ë Á‡‚ËÒflÚ ÓÚ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ‡‰ÂÍ‚‡ÚÌËfl ÏÂÚ‡·ÓÎËÚÂÌ ÍÓÌÚÓÎ (9). ëÌËʇ‚‡ÌÂÚÓ Ì‡ Ô‡‡ÏÂÚËÚ ̇ Çëó ÏÓ-

150

Ê ‰‡ Ô‰¯ÂÒÚ‚‡ ÍÎËÌ˘̇ڇ ËÁfl‚‡ ̇ Ò˙‰Â˜Ì‡Ú‡ ‡‚ÚÓÌÓÏ̇ Ì‚ÓÔ‡ÚËfl, ÍÓflÚÓ Â ÚflÒÌÓ Ò‚˙Á‡Ì‡ Ò ËÒÍ ÓÚ ÏËÓ͇‰Ì‡ ËÒıÂÏËfl, ‡ËÚÏËË Ë ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú (32, 43). èË ·ÓÎÌË Ò ‰Ë‡·eÚ ÚËÔ 1 Ë ÓÒÓ·ÂÌÓ ÔË ÚÂÁË Ò˙Ò Ò˙Ô˙ÚÒÚ‚‡˘‡ ÌÂÙÓÔ‡ÚËfl  ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â ̇Û¯ÂÌËÂÚÓ ‚˙‚ Çëó  ÌÂÁ‡‚ËÒËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎfl‚‡ÌËfl Ë ÒÏ˙ÚÌÓÒÚ ÓÚ Úflı (5, 41). èÓÒÔÂÍÚË‚ÌÓ ÔÓÛ˜‚‡Ì  ÔÓ͇Á‡ÎÓ Ò˙˘Ó ˜ÂÒÚ‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl (ëÄÑ) ÔË ·ÓÎÌË Ò ‰Ë‡·ÂÚ 2 ÚËÔ, ÍÓËÚÓ Ò‡ ÔÓ – ‚˙Á‡ÒÚÌË, Ò ÔÓ – „ÓÎflχ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ËÏ‡Ú ıËÔÂÚÓÌËfl, ÂÚËÌÓÔ‡ÚËfl Ë ÏËÍӇηÛÏËÌÛËfl. èË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ 2 ÚËÔ ëÄÑ ÍÓÂÎË‡ Ò ÌË‚ÓÚÓ Ì‡ ıÂÏÓ„ÎÓ·ËÌ A1C (33). èË Ò˙˘Ëfl ÍÓÌÚËÌ„ÂÌÚ ·ÓÎÌË Ì‡ÏÂÂ̇ڇ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ÒÌËÊÂÌË ÒÚÓÈÌÓÒÚË Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡ Ë Á‡‰Â·ÂÎfl‚‡ÌÂÚÓ Ì‡ ËÌÚËχ-ωËfl ÍÓÏÔÎÂÍÒ‡ ‚ ӷ·ÒÚÚ‡ ̇ ͇ÓÚˉ̇ڇ ·ËÙÛ͇ˆËfl ÔÓ‰Ò͇Á‚‡ ‚˙Á͇ڇ ̇ ̇Û¯Â̇ڇ Çëó Ò Ì‡Î˘ËÂÚÓ Ë ÔÓ„ÂÒËflÚ‡ ̇ ͇ÓÚˉ̇ ‡ÚÂÓÒÍÎÂÓÁ‡ (20). èË ·ÓÎÌË Ò èÅ ˜ÂÒÚÓ Ò ̇·Î˛‰‡‚‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl. Mihci E. et al. (47) ÔË 24 ˜‡ÒÓ‚Ó ËÁÒΉ‚‡Ì ̇ ‚‡ˇ·ËÎÌÓÒÚÚ‡ ̇ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ „ËÒÚË‡Ú ÔÓ – ÌËÒÍË ÒÚÓÈÌÓÒÚË, ͇ÍÚÓ Á‡ ‚ÂÏ‚ËÚÂ, ڇ͇ Ë Á‡ ˜ÂÒÚÓÚÌËÚ ԇ‡ÏÂÚË. Ç ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇ ͇‰ËÓ‚‡ÒÍÛ·ÌËÚ ÚÂÒÚÓ‚Â ÏÓ„‡Ú ‰‡ ÔÓ‰ÔÓÏÓ„Ì‡Ú ‰ËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡ ̇ Ô‡ÍËÌÒÓÌÓ‚ËÚ ÒË̉ÓÏË. ç‡Ï‡ÎÂ̇ Çëó Ò ̇·Î˛‰‡‚‡ ÔË ·ÓÎÌË Ò ÏÌÓÊÂÒÚ‚Â̇ ÒËÒÚÂÏ̇ ‡ÚÓÙËfl, ÌÂÁ‡‚ËÒËÏÓ ÓÚ Úflı̇ڇ ‚˙Á‡ÒÚ Ë ÓÚ ‰‡‚ÌÓÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. èË èÅ ÔӉӷ̇ ÍÓÌÒÚ·ˆËfl Ò Ò¢‡ Ò‡ÏÓ ‚ ̇Ô‰̇· ‚˙Á‡ÒÚ Ë ÔË „ÓÎflχ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (29). èË ÌÓ‚ÓÓÚÍËÚË ÌÂÎÂÍÛ‚‡ÌË ·ÓÎÌË Ò èŠ ̇Îˈ ·ÚÂÌÚ̇ Ò˙‰Â˜Ì‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‰ËÒÙÛÌ͈Ëfl, ÍÓflÚÓ Ì‡È – ‚ÂÓflÚÌÓ Ò ‰˙ÎÊË Ì‡ Û‚Âʉ‡Ì ̇ ÒËÌÛÒÓ‚Ëfl ‚˙ÁÂÎ. è‡-

퇷Îˈ‡ 1. è‡‡ÏÂÚË ÓÚ ‚ÂÏ‚‡Ú‡ Ë ˜ÂÒÚÓÚ̇ڇ ӷ·ÒÚ è‡‡ÏÂÚË ÓÚ ‚ÂÏ‚‡Ú‡ ӷ·ÒÚ SDNN SDANN

Ö‰ËÌˈË

ï‡‡ÍÚÂËÒÚË͇

ms ms

SD(ËÎËSDSD)

ms

RMSSD

ms

Pnn50

%

è‡‡ÏÂÚË ÓÚ ˜ÂÒÚÓÚ̇ڇ ӷ·ÒÚ é·˘‡ ÏÓ˘ÌÓÒÚ

Ö‰ËÌˈË

ëڇ̉‡ÚÌÓ ÓÚÍÎÓÌÂÌË ̇ ‚Ò˘ÍË NN ËÌÚÂ‚‡ÎË,Ó·˘ ÔÓ͇Á‡ÚÂÎ, ‚ÎËfl Ò ÓÚ ˆË͇‰ÌËÚ ËÚÏË. ëڇ̉‡ÚÌÓ ÓÚÍÎÓÌÂÌË ̇ Ò‰ÌËÚ NN ËÌÚÂ‚‡ÎË ÓÚ ‚ÒÂÍË 5 ÏËÌÛÚÂÌ Ò„ÏÂÌÚ ÓÚ Á‡ÔËÒ‡, ˜Û‚ÒÚ‚ËÚÂÎÂÌ ÔÓ͇Á‡ÚÂÎ Á‡ ÔÓ – ÌËÒÍËÚ ˜ÂÒÚÓÚË, Á‡‚ËÒ¢ ÓÚ ÙËÁ˘ÂÒ͇ڇ ‡ÍÚË‚ÌÓÒÚ, ˆË͇‰ÌËÚ ËÚÏË, ÔÓÏfl̇ ‚ ÔÓÁˈËflÚ‡ ̇ ÚflÎÓÚÓ. ëڇ̉‡ÚÌÓ ÓÚÍÎÓÌÂÌË ̇ ‡ÁÎËÍËÚ ÏÂÊ‰Û Ò˙Ò‰ÌË NN ËÌÚÂ‚‡ÎË, ÓÚ‡Áfl‚‡˘ ‰ÂÌÓÌÓ˘ÌËÚ ÔÓÏÂÌËÚ ‚ HRV. ë‰ÌÓ Í‚‡‰‡Ú˘ÌÓ Ì‡ ‡ÁÎËÍËÚ ÏÂÊ‰Û ‚ÒÂÍË ‰‚‡ ÔÓÒΉӂ‡ÚÂÎÌË NN ËÌÚÂ‚‡ÎË, Á‡ Í‡ÚÍÓ‚ÂÏÂÌÌË ÔÓÏÂÌË ‚ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Ë ÌÂÁ‡‚ËÒËÏ ÓÚ ˆËÍ˙· ‰ÂÌ/ÌÓ˘. èÓˆÂÌÚ‡ ̇ ÔÓÒΉӂ‡ÚÂÎÌËÚ ËÌÚÂ‚‡ÎË, ÍÓËÚÓ Ò ‡Á΢‡‚‡Ú Ò Ôӂ˜ ÓÚ 50 ms, Í‡ÚÍÓ‚ÂÏÂÌÌË ÔÓÏÂÌË ‚ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Ë ÌÂÁ‡‚ËÒËÏ ÓÚ ˆËÍ˙· ‰ÂÌ/ÌÓ˘. èÓ – ÌÂÒÚ‡·ËÎÂÌ ÔÓ͇Á‡ÚÂÎ ÓÚ RMSSD. ï‡‡ÍÚÂËÒÚË͇ óÂÒÚÓÚÂÌ ‰Ë‡Ô‡ÁÓÌ

ms2

UVL

ms2

VLF

ms2

LF

ms2

HF

ms2

LF/HF Ò˙ÓÚÌÓ¯ÂÌËÂ

LJˇˆËfl ̇ ‚Ò˘ÍË NN ËÌÚÂ‚‡ÎË, Ó·˘‡ ‚‡ˇ·ËÎÌÓÒÚ, ÍÓÂÒÔÓ̉Ë‡ Ò˙Ò ÒÛχڇ ÓÚ ‚Ò˘ÍË ÒÔÂÍÚ‡ÎÌË ÍÓÏÔÓÌÂÌÚË. ìÎÚ‡ÌËÒÍË ˜ÂÒÚÓÚË, ÓÚ‡Áfl‚‡ ‚ÎËflÌËÂÚÓ Ì‡ ˆË͇‰ÌËÚÂ Ë Ì‚ÓẨÓÍËÌÌË ËÚÏË. åÌÓ„Ó ÌËÒÍË ˜ÂÒÚÓÚË., Ò‚˙Á‡Ì Ò ‰˙΄ÓÚ‡ÈÌË ÔÓÏÂÌË, Á‡‚ËÒË ÓÚ ÙËÁ˘ÂÒ͇ڇ ‡ÍÚË‚ÌÓÒÚ, χÍÂ Á‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ. çËÒÍË ˜ÂÒÚÓÚË, ËÁ‡Á ͇ÍÚÓ Ì‡ ÒËÏÔ‡ÚËÍÓ‚Ë, ڇ͇ Ë Ì‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë ‚ÎËflÌËfl, ͇ÚÓ ‚ ÌÓχÎËÁË‡ÌË Â‰ËÌËˆË ÓÚ‡Áfl‚‡ ÒËÏÔ‡ÚËÍÓ‚‡Ú‡ ‡ÍÚË‚ÌÓÒÚ. ÇËÒÓÍË ˜ÂÒÚÓÚË, ÂÒÔË‡ÚÓÌÓ Á‡‚ËÒËÏ Ï‡ÍÂ Á‡ ‚‡„ÛÒÓ‚ËÚ ‚ÎËflÌËfl, ÓÔ‰ÂÎfl Ò ÓÚ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‰Ë¯‡ÌÂÚÓ. ë˙ÓÚÌÓ¯ÂÌË ̇ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚ Í˙Ï ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡, ÓÚ‡Áfl‚‡ ÒËÏÔ‡ÚËÍÓ‚‡„‡ÎÌËfl ·‡Î‡ÌÒ.

< 0.4 Hz <0.003 Hz <0.003-0.4 Hz 0.04 – 0.15 Hz 0.15 – 0.4 Hz


ÑÂÍÂÏ‚Ë 2009

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

151

îË„. 1 èÓ„‡Ï‡ Á‡ ËÁ˜ËÒÎfl‚‡Ì ̇ ÒÔÂÍÚ‡ÎÌËÚ ‰‡ÌÌË Ì‡ Çëó ‡ÒËÏÔ‡ÚËÍÓ‚‡Ú‡ ͇‰ËÓ‚‡ÒÍÛ·̇ „Û·ˆËfl Ò˙˘Ó Ì  ÔÓ˘‡‰Â̇ ÔË ÚÓÁË ÍÓÌÚËÌ„ÂÌÚ ·ÓÎÌË; ÔÓÏÂÌËÚ ҇ Ì‡È – ÒËÎÌÓ ËÁ‡ÁÂÌË ÔÂÁ ÌÓ˘ÌËÚ ˜‡ÒÓ‚Â (55). èË Ô‡‡ÎÂÎÌÓ ÔÓ‚Âʉ‡Ì ̇ Ò˙‰Â˜Ì‡Ú‡ ÒˆËÌÚË„‡ÙËfl Ò I123 MIBG Ò‚˙Á‚‡ÌÂÚÓ Ì‡ ËÁÓÚÓÔ‡ ÔÓ„ÂÒË‚ÌÓ Ì‡Ï‡Îfl‚‡ Ò Ì‡‡ÒÚ‚‡Ì ̇ ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (48). è‡‡ÏÂÚËÚ ̇ Çëó ÍÓÂÎË‡Ú Ò ÚÂÊÂÒÚÚ‡ ̇ ‰‚Ë„‡ÚÂÎÌËÚ ̇Û¯ÂÌËfl ÔË èÅ , ÌÓ Ì ÔÓ͇Á‚‡Ú Á‡‚ËÒËÏÓÒÚ ÓÚ ÔÓfl‚ËÚ ̇ ÚÂÏÓ Ë ˄ˉÌÓÒÚ. èË ·ÓÎÌËÚÂ Ò ÚÂÊ˙Í ‰‚Ë„‡ÚÂÎÂÌ ‰ÂÙˈËÚ Ò ̇·Î˛‰‡‚‡ ÒÌËÊÂÌË ̇ ÒÚÓÈÌÓÒÚËÚ ̇ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡, ‡ ÔÓ- ÚÂÊÍÓ ËÁ‡ÁÂ̇ ıËÔÓÍËÌÂÁËfl  ҂˙Á‡Ì‡ Ò ÔÓ- ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËÚ ÍÓÏÔÓÌÂÌÚË (22). éÔ‰ÂÎflÌÂÚÓ Ì‡ ‰ÓÏËÌË‡˘‡Ú‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚‡ ËÎË ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ ÔÂÁ ‡Á΢ÌË Ù‡ÁË Ì‡ Ò˙Ìfl  ˜Û‚ÒÚ‚ËÚÂÎÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ÌÓ˘ÌË ëÄÑ ÔË èÅ. ê‡ÍÚË‚ÌÓÒÚÚ‡ ̇ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ ÔË ‰‚ËÊÂÌË ̇ ÚflÎÓÚÓ ÔË ÚÂÁË ·ÓÎÌË Â ÒÌËÊÂ̇ ÒÔflÏÓ Á‰‡‚ËË Îˈ‡, ÓÒÓ·ÂÌÓ ÔÂÁ êÖå ÒÚ‡‰Ëfl ̇ Ò˙Ìfl. èË Úflı Ò‡ ̇χÎÂÌË ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËÚ ÍÓÏÔÓÌÂÌÚË Í‡ÍÚÓ ÔË Ò˙·Ûʉ‡ÌÂ, ڇ͇ Ë ÔÂÁ ÌÂ- êÖå Ò˙Ìfl; ̇Û¯ÂÌ Â Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ëfl ͇‰ËÓ‚‡ÒÍÛ·ÂÌ ÍÓÌÚÓÎ (31). èËÂÏ˙Ú Ì‡ ΂ӉÓÔ‡ ωË͇ÏÂÌÚË ÔË èÅ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ‚ÎÓ¯‡‚‡ ̇Û¯ÂÌËfl ‡‚ÚÓÌÓÏÂÌ ÍÓÌÚÓΠ̇ ‡ÚÂˇÎÌÓÚÓ Ì‡Îfl„‡ÌÂ Ë Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ (8). Ç Ò‡‚ÌÂÌËÂ Ò ÌÂÎÂÍÛ‚‡ÌË ·ÓÎÌË Ò˙Ò Ò··Ó ËÁ‡ÁÂÌ ‰‚Ë„‡ÚÂÎÂÌ ‰ÂÙˈËÚ, LF Ë Ò˙ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF, ËÁÏÂÂÌË ÔÂÁ ‰Ì‚ÌËÚ ˜‡ÒÓ‚Â, Ò‡ ÒÌËÊÂÌË ÔË ·ÓÎÌË Ò˙Ò Ò‰̇ ÚÂÊÂÒÚ Ì‡ èÅ Ë ÔËÂÏ Ì‡ ΂ӉÓÔ‡, ͇ÍÚÓ Ë ÔË ÚÂÁË Ò ÛÒÎÓÊÌÂÌËfl ÓÚ Î˜ÂÌËÂÚÓ. LJ„ÛÒÓ‚ËÚ χÍÂË - HF Ë pNN50 Ò‡ ̇χÎÂÌË ÔÂÁ ÌÓ˘Ú‡ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ËÒÍËÌÂÁËË Ë ÍÓÂÎË‡Ú Ò Ï‰Ë͇ÏÂÌÚÓÁÌÓ Ò‚˙Á‡ÌËÚ “off” ÔÂËÓ‰Ë, ÌÓ ÌÂ

Ë Ò “on” ÔÂËÓ‰ËÚ (12). ᇠ‡ÁÎË͇ ÓÚ Î‚ӉÓÔ‡ ωË͇ÏÂÌÚËÚÂ, COåí – ËÌıË·ËÚÓËÚ (tolcapone) Ì ÔÓ‚ÎËfl‚‡Ú ͇‰ËÓ‚‡ÒÍÛ·ÌËÚ ÙÛÌ͈ËË ÔË Ô‡ˆËÂÌÚË Ò èÅ (44). àÁÒΉ‚‡ÌÂÚÓ Ì‡ Äçë Ë Çëó ÔË ·ÓÎÌË Ò ÂÔËÎÂÔÒËfl ‰ÓÔË̇Òfl Á‡ ÓÚÍË‚‡Ì ̇ ‡Á΢ÌË ‡‚ÚÓÌÓÏÌË ÔÓfl‚Ë Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, Á‡ ÔÓ‰Ó·fl‚‡Ì ̇ ÌÂÈ̇ڇ ‰Ë‡„ÌÓÒÚË͇ Ë Á‡ ËÁ·Ó‡ ̇ ÔÓ – ÂÙÂÍÚ˂̇ ÚÂ‡ÔËfl. (18). ç‡Û¯ÂÌËflÚ‡ ‚ ‡‚ÚÓÌÓÏÌËfl Ò˙‰Â˜ÌÓÒ˙‰Ó‚ ÍÓÌÚÓÎ Ë„‡flÚ ‚‡Ê̇ ÓÎfl Á‡ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú ÔË ·ÓÎÌË ÓÚ ÂÔËÎÂÔÒËfl. ífl ̇ÒÚ˙Ô‚‡ Ì‡È – ˜ÂÒÚÓ ÌÓ˘ÂÏ, ÍÓ„‡ÚÓ ÔÓÏÂÌË ‚˙‚ Çëó Ò‡ ÔÓ – ÒËÎÌÓ ËÁ‡ÁÂÌË (51). èÓÛ˜‚‡ÌËfl ̇ ‡Á΢ÌË ‡‚ÚÓË ÔÓ͇Á‚‡Ú Á‡‚ËÒËÏÓÒÚ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ‡‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl Ë Ì‡ Çëó ÓÚ ÎÓ͇ÎËÁ‡ˆËflÚ‡ ̇ ÂÔËÎÂÔÚ˘ÌÓÚÓ Ó„ÌË˘Â Ì‡ ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡Ï‡Ú‡ Ë ÓÚ ‚ˉ‡ ̇ ÔËÒÚ˙ÔËÚÂ. í‡Í‡ ÔË Ô‡ˆËÂÌÚË Ò ÙÓÌÚ‡Î̇ ÂÔËÎÂÔÒËfl Ò ̇·Î˛‰‡‚‡ ̇χÎÂ̇ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ (26), ‰Ó͇ÚÓ ÔË ÚÂÁË Ò „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌ˘ÌÓ – ÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË Ë Ò ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ôӂ˯Â̇ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ (15). ëÌËÊÂ̇ Çëó, ÓÒÓ·ÂÌÓ ‚ ÌÓ˘ÌËÚ ˜‡ÒÓ‚Â, Ò ÓÚÍË‚‡ ÔË Ô‡ˆËÂÌÚË Ò ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl. èÓÏÂÌËÚ ÔÂӷ·‰‡‚‡Ú ‚ ÒÎÛ˜‡ËÚÂ Ò ‰ÂÒÂÌ ÖÖÉ ÙÓÍÛÒ, Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ Ò˙‰Â˜Ì‡ ‡ËÚÏËfl Ë Ò‡ Ò‚˙Á‡ÌË Ò ‚ËÒÓÍ ËÒÍ ÓÚ ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. ç‡Û¯ÂÌËflÚ‡ ‚˙‚ Çëó ÔË ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl Ì Á‡‚ËÒflÚ ÓÚ ÂÙÂÍÚ‡ ̇ ÔÓ‚Âʉ‡ÌÓÚÓ Î˜ÂÌË (59). èË ÂÁËÒÚÂÌÚ̇ ̇ ωË͇ÏÂÌÚÓÁÌÓ Î˜ÂÌË ÂÔËÎÂÔÒËfl ̇΢ËÂÚÓ ËÏ Â Ò‚˙Á‡ÌÓ Ë Ò Ì··„ÓÔËflÚ̇ ÔÓ„ÌÓÁ‡ ÔË Â‚ÂÌÚÛ‡Î̇ ıËÛ„˘̇ ̇ÏÂÒ‡ (52). èË ‰Âˆ‡ Ò ÂÔËÎÂÔÒËfl Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Á‡‚ËÒËÏÓÒÚË ÏÂÊ‰Û ı‡‡ÍÚÂÌËÚ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÖÖÉ ÔÓÏÂÌË Ë Çëó (4). èË ÚÂÁË Ò ÂÙ‡ÍÚÂ̇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔ-


ÑÂÍÂÏ‚Ë 2009

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

ÒËfl Ò ̇·Î˛‰‡‚‡ ‰Û͈Ëfl ̇ Çëó Ô‰ËÏÌÓ ‚˙‚ ‚ËÒÓÍÓ˜ÂÒÚÓÚ̇ڇ ӷ·ÒÚ (25). èÓ‰Ó·ÌÓ Ì‡ ‚˙Á‡ÒÚÌËÚÂ, ÔË ‰Âˆ‡Ú‡ Ò Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl ÔÓ ‚ÂÏ ̇ Ò˙Ì Ò ÔÓfl‚fl‚‡ ÚẨÂ̈Ëfl Á‡ ÒÌËÊÂÌË ̇ ÔÓ͇Á‡ÚÂÎËÚ ̇ Çëó ͇ÍÚÓ ‚˙‚ ‚ÂÏ‚‡Ú‡, ڇ͇ Ë ‚ ˜ÂÒÚÓÚ̇ڇ ӷ·ÒÚ. íÓ Â Ì‡È- ‰Ó· ËÁ‡ÁÂÌÓ ‚ ÒÚ‡‰ËÈ 3 Ë 4 ̇ êÖå Ò˙Ìfl (17). ëÌËÊÂÌËÂÚÓ Ì Á‡‚ËÒË ÓÚ Ì‡Î˘ËÂÚÓ ËÎË ÓÚÒ˙ÒÚ‚ËÂÚÓ Ì‡ ωË͇ÏÂÌÚÓÁÌÓ Î˜ÂÌËÂ. èË ‰Âˆ‡Ú‡ ·ÂÁ ÔÓ‚Âʉ‡ÌÓ ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓ Î˜ÂÌË ÔÂӷ·‰‡‚‡ Ò˙˘Ó ÒÌËÊÂÌË ̇ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚ËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ Çëó (24). ÑÓÔÛÒ͇ÌÂÚÓ Ì‡ O. Hallioglu et al. (24), ˜Â ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓÚÓ Î˜ÂÌË ÏÓÊ ‰‡ ‰Ó‚‰ ‰Ó ÔÓ‰Ó·fl‚‡Ì ̇ ‡‚ÚÓÌÓÏÌËÚ Ò˙‰Â˜ÌË ÙÛÌ͈ËË, Ò ÔÓÚ‚˙ʉ‡‚‡ ÓÚ ‰‡ÌÌËÚ Á‡ ·Î‡„ÓÔËflÚÂÌ ÂÙÂÍÚ ‚˙ıÛ Ì‡Û¯Â̇ڇ Çëó ÓÚ Î˜ÂÌËÂÚÓ Ò Ääíï ÔË ÒË̉Óχ ̇ West (27) Ë ÓÚ ÒÚËÏÛ·ˆËflÚ‡ ̇ n. vagus ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒË̉ÓÏ Ì‡ Lennox-Gastaut (35). éÚ ‰Û„‡ ÒÚ‡Ì‡, ÔÓ‰˙ÎÊËÚÂÎÌÓÚÓ Î˜ÂÌËÂ Ò ‡ÌÚËÂÔËÎÂÔÚ˘ÌË Ï‰Ë͇ÏÂÌÚË, ÓÒÓ·ÂÌÓ Í‡·‡Ï‡ÁÂÔËÌ Ò ҂˙Á‚‡ Ò ÔÓÌËÊÂ̇ Çëó ‚ Ò‡‚ÌÂÌË Ò˙Ò ÒÚÓÈÌÓÒÚËÚÂ È ÔË ÌӂӉˇ„ÌÓÒÚˈË‡ÌË ÌÂÎÂÍÛ‚‡ÌË Ô‡ˆËÂÌÚË (50). èÓÒÚÂÔÂÌÌÓÚÓ ÓÚÌÂχÌ ̇ ÚÓÁË Ï‰Ë͇ÏÂÌÚ Ó͇Á‚‡ ·Î‡„ÓÔËflÚÂÌ ÂÙÂÍÚ Í‡ÍÚÓ ‚˙ıÛ ÒËÏÔ‡ÚËÍÓ‚ËÚÂ, ڇ͇ Ë ‚˙ıÛ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚ËÚ ÙÛÌ͈ËË (40). ÇÌÂÁ‡ÔÌÓÚÓ ÒÔË‡Ì ̇ ÔËÎÓÊÂÌËÂÚÓ ÏÛ Ó·‡˜Â ‚Ó‰Ë ‰Ó Ôӂ˯Â̇ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ ÔÓ ‚ÂÏ ̇ Ò˙Ì, ÍÓÂÚÓ Ô‰‡ÁÔÓ·„‡ Í˙Ï ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú (28). èË ·ÓÎÌËÚÂ Ò ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ ÔÓÏÂÌË ‚˙‚ Çëó Ò ̇·Î˛‰‡‚‡Ú ̇È-˜ÂÒÚÓ ÔË ÎÓ͇ÎËÁ‡ˆËfl ̇ ËÌÙ‡ÍÚ‡ ‚ ‰flÒ̇ڇ ËÌÒÛ·. í Ò ÔÓfl‚fl‚flÚ Ò˙Ò Á̇˜ËÏÓ ÒÌËÊÂÌË ÒÚÓÈÌÓÒÚË Ì‡ SDNN Ë Ì‡ rMSSD Ë ÔÓ‚ËÒÓÍË ÒÚÓÈÌÓÒÚË Ì‡ ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF. èË ÚÂÁË ·ÓÎÌË ÔÓ-˜ÂÒÚÓ Ì‡ÒÚ˙Ô‚‡Ú ËÚ˙ÏÌË Ì‡Û¯ÂÌËfl (11). èËÂχ ÒÂ, ˜Â ÔÓÏÂÌËÚ ̇ Çëó, ÓÒÓ·ÂÌÓ Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚÂ È ÍÓÏÔÓÌÂÚË, Ò‡ ÌÂÁ‡‚ËÒËÏ ÔÓ„ÌÓÒÚ˘ÂÌ Ù‡ÍÚÓ Á‡ ËÁıÓ‰‡ ÓÚ ÓÒÚ˙ ÚÂÊ˙Í ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ (21). éˆÂÌ͇ڇ ËÏ ÔÂ‰Ë Á‡ÔÓ˜‚‡Ì ̇ Âı‡·ËÎËÚ‡ˆËflÚ‡ ̇ ÚÂÁË ·ÓÎÌË ÏÓÊ ‰‡ ‰‡‰Â ËÌÙÓχˆËfl Á‡ Ó˜‡Í‚‡Ì‡Ú‡ ÒÚÂÔÂÌ Ì‡ ÙÛÌ͈ËÓ̇ÎÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡ÌÂ. ífl  Ì··„ÓÔËflÚ̇ ÔË ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ SDNN (6). èÓ͇Á‡ÌÓ Â, ˜Â Çëó ÓÒÚ‡‚‡ Á̇˜ËÏÓ ÔÓ-ÌËÒ͇ ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‰‡‚Ë Îˈ‡ Ë ÒΉ ÔÂÏË̇‚‡Ì ̇ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ (38). ᇠÔÓÏÂÌËÚÂ È ‰ÓÔË̇Òfl Ë Ì‡Î˘ËÂÚÓ Ì‡ ‰ÂÔÂÒËfl ÒΉ ÔÂÊË‚ÂÌËfl ÏÓÁ˙˜ÂÌ Ò˙‰Ó‚ Ë̈ˉÂÌÚ (58). ëÌËÊÂ̇ Çëó Ò ̇·Î˛‰‡‚‡ ÔË ÔˉÛʇ‚‡˘‡ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ Í‡ÓÚˉ̇ ‡ÚÂÓÒÍÎÂÓÁ‡ (37). Ç ÂÁÛÎÚ‡Ú ÓÚ Ò‚Ó ÔÓÛ˜‚‡Ì ‚˙ıÛ Çëó ÔË ·ÓÎÌË Ò ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡ J.Diamond et al. (13) ÔËÂχÚ, ˜Â ÂÁÛÎÚ‡ÚËÚÂ, ÔÓÎÛ˜ÂÌË Ò ÚÓÁË ÏÂÚÓ‰ Ò‡ ËÁÚÓ˜ÌËÍ Ì‡ ÔÓÎÂÁ̇ ËÌÙÓχˆËfl Á‡ ÓˆÂÌ͇ ̇ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ëfl ÍÓÌÚÓΠ̇ Ò˙ˆÂÚÓ, ͇ÍÚÓ Ë Á‡ ËÁ·Ó‡ ̇ ÔÓ – ÂÙÂÍÚË‚ÌÓ Î˜ÂÌËÂ Ë eı‡·ËÎËÚ‡ˆËÓÌ̇ ÒÚ‡Ú„Ëfl. LJˇ·ËÎÌÓÒÚÚ‡ ̇ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ ̇χÎfl‚‡ Ò Û‚Â΢‡‚‡Ì ̇ ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë Ì‡ Ì„ӂ‡Ú‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ (42). èË ·ÓÎÌË Ò ‚ÚÓ˘ÌÓ ÔÓ„ÂÒË‡˘‡ ÙÓχ ̇ ÏÌÓÊÂÒÚ‚Â̇ ÒÍÎÂÓÁ‡ Merico et al. (45) ̇ÏË‡Ú ÒÚ‡ÚËÒÚ˘ÌÓ Á̇˜ËÏË Á‡‚ËÒËÏÓÒÚË ÏÂÊ‰Û Ô‡‡ÏÂÚËÚ ̇ Çëó Ë ÂÁÛÎÚ‡ÚËÚ ÓÚ Ò͇·ڇ Á‡ ÓˆÂÌ͇ ̇ ÛÏÓ‡; ÔË Ò˙˘ËÚ ·ÓÎÌË ÓÚÒ˙ÒÚ‚‡ Á‡‚ËÒËÏÓÒÚ Ì‡ Çëó Ò ‚˙Á‡ÒÚÚ‡ Ë EDSS Ò͇·ڇ. 燷≇‚‡ÌËÚ Á̇˜ËÏË ‡ÁÎËÍË ‚ ÒÚÂÔÂÌÚ‡ ̇ ÛÏÓ‡ ÏÂÊ‰Û Ô‡ˆËÂÌÚËÚÂ Ò Ë ·ÂÁ ‡‚ÚÓÌÓÏÌË Ì‡Û¯ÂÌËfl ÔÓ‰Ò͇Á‚‡Ú ‰ÓÔ˙ÎÌËÚÂÎÌÓÚÓ È ÔÓ‚ÎËfl‚‡Ì ÓÚ ‡‚ÚÓÌÓÏ̇ ‰ËÒÙÛÌ͈Ëfl (46). àÁÒΉ‚‡ÌÂÚÓ Ì‡ ÔÓÏÂÌËÚ ‚˙‚ Çëó Ì ÍÓÂÎË‡ Ò ÂÁÛÎÚ‡ÚËÚ ÓÚ I-123 MIBG ÒˆËÌÚË„‡ÙËfl ̇ ÏËÓ͇‰‡. ë˙˜ÂÚ‡ÌÓÚÓ ËÁÔÓÎÁ‚‡Ì ̇ ÚÂÁË ÏÂÚÓ‰Ë ÔË ·ÓÎÌË Ò ‚ÚÓ˘ÌÓ ÔÓ„ÂÒË‡˘‡ ÏÌÓ-

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ÊÂÒÚ‚ÂÌ̇ ÒÍÎÂÓÁ‡ ÏÓÊ ‰‡ ‰ÓÔËÌÂÒ Á‡ ÓˆÂÌfl‚‡Ì ̇ ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (39). èË ‰ˈ‡ ‰Û„Ë Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl Ò‡ Ô‡‚ÂÌË Â‰ËÌ˘ÌË ËÁÒΉ‚‡ÌËfl ̇ ‚‡ˇ·ËÎÌÓÒÚÚ‡ ̇ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡, ‰Ó͇Á‚‡˘Ë ̇΢ˠ̇ ‡‚ÚÓÌÓÏÌË Ì‡Û¯ÂÌËfl. ÑÓ͇ÚÓ Allan L. et al. (2) Ì ̇ÏË‡Ú ÔÓÏÂÌË ‚˙‚ Çëó ÔË ·ÓÎÂÒÚÚ‡ ̇ ÄΈı‡ÈÏÂ, ‰Û„Ë ‡‚ÚÓË (19) ÓÚÍË‚‡Ú Á̇˜ËÏÓ ÒÌËÊÂÌË ̇ ‚Ò˘ÍË ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡, ÓÒÓ·ÂÌÌÓ Ì‡ LF Ë LF/HF ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‰‡‚Ë Îˈ‡. í ‰ÓÔÛÒ͇Ú, ˜Â ̇Û¯ÂÌËÂÚÓ Ì‡ Ò˙‰Â˜ÌËÚ ‡‚ÚÓÌÓÏÌË ÙÛÌ͈ËË Ò ‰˙ÎÊË Ì‡ ıÓÎËÌÂ„˘ÂÌ ‰ÂÙˈËÚ, ÍÓÈÚÓ ‚ ÌflÍÓË ÒÎÛ˜‡Ë ÏÓÊ ‰‡ Ò ÍÓË„Ë‡ ˜ÂÁ ΘÂÌËÂ Ò ıÓÎËÌÂÒÚÂ‡ÁÌË ËÌıË·ËÚÓË. Çëó  ÔÓÎÂÁÂÌ Ë ˆÂÌÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ÙÛÌ͈ËÓ̇ÎÌÓÚÓ Ò˙ÒÚÓflÌË ̇ Äçë, ÓÒÓ·ÂÌÓ ‚ ‡ÌÌËÚ ÒÚ‡‰ËË Ì‡ ÓÎË‚ÓÔÓÌÚÓˆÂ·Â·̇ ‰Â„ÂÌÂ‡ˆËfl (36). èË ÒÔËÌÓˆÂ·Â·̇ ‰Â„ÂÌÂ‡ˆËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ô‰ËÏÌÓ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë Ì‡Û¯ÂÌËfl Ë ÚÂÊÂÒÚÚ‡ ËÏ ÍÓÂÎË‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (54). èË Ô‡ˆËÂÌÚË Ò Î‡ÚÂ‡Î̇ ‡ÏËÓÚÓÙ˘̇ ÒÍÎÂÓÁ‡ Ò ̇·Î˛‰‡‚‡ Ôӂ˯Â̇ Ò˙‰Â˜Ì‡ ˜ÂÒÚÓÚ‡, ̇χÎÂÌÓ SDNN Ë Û‚Â΢‡‚‡Ì ̇ ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF, ÍÓËÚÓ Ì ÍÓÂÎË‡Ú Ò ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË Ë ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ê„ËÒÚË‡ÌÓÚÓ ÒÛ·ÍÎËÌ˘ÌÓ ‡‚ÚÓÌÓÏÌÓ Ì‡Û¯ÂÌË Á‡Òfl„‡ Ô‰ËÏÌÓ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ëfl Ò˙‰Â˜ÂÌ ÍÓÌÚÓÎ (53). èË ÚÂÁË ·ÓÎÌË Ëχ ‰‡ÌÌË Á‡ ÔÓÒÚ„‡Ì„ÎËÓ̇̇ ÒËÏÔ‡ÚËÍÓ‚‡ ‡‰ÂÌÂ„˘̇ ‰ÂÌÂ‚‡ˆËfl, ‰Ó͇Á‡Ì‡ ˜ÂÁ I123 MIBG ÒˆËÌÚË„‡ÙËfl Ë Á‡ ͇‰ËÓ‚‡„‡Î̇ ‰ÂÌÂ‚‡ˆËfl, ÛÒÚ‡ÌÓ‚Â̇ ˜ÂÁ ËÁÒΉ‚‡Ì ̇ Çëó (14). èË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ ̇ ï˙ÌÚËÌ„ÚÓÌ, ÍÓËÚÓ Ò‡ ‡ÒËÏÔÚÓÏÌË „ÂÌÌË ÌÓÒËÚÂÎË ËÎË Ò‡ Ò ÛÏÂÂÌÓ ËÁ‡ÁÂÌË ÍÎËÌ˘ÌË ÔÓfl‚Ë, Ò ̇·Î˛‰‡‚‡ Ôӂ˯Â̇ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ, ÍÓflÚÓ Â ‚ ÍÓÌÚ‡ÒÚ Ò˙Ò ÒÌËÊÂ̇ڇ ‡‚ÚÓÌÓÏ̇ „Û·ˆËfl ‚ ̇Ô‰̇ÎËfl ÒÚ‡‰ËÈ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (34). ÅÓÎÌËÚÂ Ò ÛÏÂÂÌÓ ‰Ó ÚÂÊÍÓ ËÁ‡ÁÂ̇ ·ÓÎÂÒÚ Ì‡ ï˙ÌÚËÌ„ÚÓÌ ËÏ‡Ú Í‡ÍÚÓ ÒËÏÔ‡ÚËÍÓ‚Ë, ڇ͇ Ë Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë ‰ËÒÙÛÌ͈ËË (3). ãÓÌ„ËÚÛ‰Ë̇ÎÂÌ ‚ÂÏÂ‚Ë Ë ˜ÂÒÚÓÚÂÌ ‡Ì‡ÎËÁ ̇ Çëó ÔË ·ÓÎÌË Ò ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl ÚËÔ Ñ˛¯ÂÌ Â ÔÓ͇Á‡Î ÔÓ„ÂÒË‚ÌÓ ÒÌËÊÂÌË ̇ Ô‡‡ÏÂÚËÚ ÓÚ ˜ÂÒÚÓÚ̇ڇ ӷ·ÒÚ, ÓÒÓ·ÂÌÌÓ ‚ ÌÓ˘ÌËÚ ˜‡ÒÓ‚Â, ‰Ó͇ÚÓ ‚ÂÏ‚ËÚ ԇ‡ÏÂÚË Ò‡ ÔÓ͇Á‡ÎË ÒÌËÊÂÌË ÔÂÁ ˆflÎÓÚÓ ‰ÂÌÓÌӢˠ(62). èË ÚÂÁË ·ÓÎÌË Ò ̇·Î˛‰‡‚‡ ̇Û¯Â̇ ‡‚ÚÓÌÓÏ̇ ÙÛÌ͈Ëfl, ÍÓflÚÓ Á‡Òfl„‡ ÓÒÌÓ‚ÌÓ Ó·˘‡Ú‡ ‚‡ˇ·ËÎÌÓÒÚ ÔË ÓÚÒ˙ÒÚ‚Ë ̇ ‡·ÌÓÏÌË ÔÓÏÂÌË ‚ ÍÓÌ‚Â̈ËÓ̇ÎÌÓÚÓ ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡Ì ̇ Ò˙‰Â˜Ì‡Ú‡ ÙÛÌ͈Ëfl. Ä‚ÚÓËÚ Ô‰·„‡Ú ËÁÏÂ‚‡Ì ̇ Ò‰̇ڇ Ò˙‰Â˜Ìa ˜ÂÒÚÓÚ‡ ÔÂÁ ÌÓ˘Ú‡ ͇ÚÓ Ì‡È-ÎÂÒÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ‡‚ÚÓÌÓÏ̇ڇ ÙÛÌ͈Ëfl ÔË Ú‡ÍË‚‡ Ô‡ˆËÂÌÚË (30). èË ·ÓÎÌË Ò ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl 1 ÚËÔ ÔË 24 – ˜‡ÒÓ‚Ë Á‡ÔËÒË SDNN Ë Ó·˘‡Ú‡ ‚‡ˇ·ËÎÌÓÒÚ Ò‡ Á̇˜ËÏÓ ÔÓ – ÌËÒÍË ÒÔflÏÓ Á‰‡‚ËÚÂ. éÒڇ̇ÎËÚ ԇ‡ÏÂÚË Ò˙˘Ó ÔÓ͇Á‚‡Ú ÚẨÂ̈Ëfl Í˙Ï ÒÌËÊÂÌËÂ. чÌÌËÚ ÔÓ‰Ò͇Á‚‡Ú ̇΢ˠ̇ ÒËÏÔ‡ÚËÍÓ‚‡ ‰ËÒÙÛÌ͈Ëfl Ë Ô‚‡ÎË‡Ì ̇ ‚‡„ÛÒÓ‡Ú‡ ‡ÍÚË‚ÌÓÒÚ ÔË ÚÂÁË ·ÓÎÌË. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡ Á‡‚ËÒËÏÓÒÚ Ì‡ ëÄÑ ÓÚ ‚˙Á‡ÒÚÚ‡ ̇ ·ÓÎÌËÚÂ Ë ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ(56). àÁÒΉ‚‡ÌÂÚÓ Ì‡ Çëó ÒΉ ÔÂ͇‡ÌË ÚÂÊÍË ˜ÂÂÔÌÓÏÓÁ˙˜ÌË Ú‡‚ÏË (óåí)  ҂˙Á‡ÌÓ Ò ‰Ó͇Á‡Ì‡ ‰Û͈Ëfl ̇ TP Ë LF/HF Ë Ì··„ÓÔËflÚ̇ ÔÓ„ÌÓÁ‡ Á‡ ·ÓÎÌËÚ (23). èË ‰Âˆ‡ Ò óåí ‡Ì‡ÎËÁ˙Ú Ì‡ Çëó ÒΉ Ô‡ÒË‚ÌÓ ËÁÔ‡‚flÌ ‰ÓÔË̇Òfl Á‡ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ Ì‚ÓωËË‡ÌË ÒËÌÍÓÔË. ìÒÚ‡ÌÓ‚ÂÌËflÚ ıËÔÂ‡ÍÚË‚ÂÌ ÓÚ„Ó‚Ó Ë„‡Â ÓÎfl ‚ ‡Á‚ËÚËÂÚÓ Ì‡ Ô‡ÚÓÎӄ˘ÌË ‡‚ÚÓÌÓÏÌË ÂÙÎÂÍÒË (16). ÉÓÎÂÏËflÚ ·ÓÈ ÔÓÛ˜‚‡ÌËfl Ë Ì‡ÚÛÔ‡ÌËflÚ ÓÔËÚ ÓÚ


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

ÑÂÍÂÏ‚Ë 2009

ËÁÒΉ‚‡ÌÂÚÓ Ì‡ Çëó ÔË ‡Á΢ÌË Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl ÌË ÔÓÁ‚ÓÎfl‚‡ ‰‡ ÔËÂÏÂÏ, ˜Â ÚÓÁË ÌÂËÌ‚‡ÁË‚ÂÌ, ÎÂÒÂÌ Á‡ ËÁÔ˙ÎÌÂÌËÂ Ë ËÌÚÂÔÂÚ‡ˆËfl ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ Ò˙‰Â˜Ì‡Ú‡ ‡‚ÚÓÌÓÏ̇ ÙÛÌ͈Ëfl Ëχ ÓÔ‰ÂÎÂ̇ ‰Ë‡„ÌÓÒÚ˘̇ ÒÚÓÈÌÓÒÚ ÔË ÚÂÁË Á‡·ÓÎfl‚‡ÌËfl. ç‡È-˜ÂÒÚÓ ÔË·„‡ÌÂÚÓ ÏÛ Â Ò‚˙Á‡ÌÓ Ò˙Ò ÒÌËÊÂÌË ̇ Ó·˘‡Ú‡ ‚‡ˇ·ËÎÌÓÒÚ, ̇ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡ Ë Ì‡ ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF, ÍÓËÚÓ ÔÓ͇Á‚‡Ú ̇Û¯Â̇ ÒËÏÔ‡ÚËÍÓ‚‡ ÙÛÌ͈Ëfl. èÓfl‚‡Ú‡ ËÏ Â Ò‚˙Á‡Ì‡ Ò Û‚Âʉ‡Ì ̇ ÏÓÁ˙˜ÌË ‡‚ÚÓÌÓÏÌË ÒÚÛÍÚÛË (ÎËϷ˘ÌË, ËÌÒÛ·̇ ÍÓ‡, ıËÔÓڇ·ÏÛÒ, ÒÚ‚ÓÎÓ‚Ë Ë „˙·Ì‡˜ÌÓÏÓÁ˙˜ÌË ‡‚ÚÓÌÓÏÌË fl‰‡), ͇ÍÚÓ Ë Ò Ì‡Û¯‡‚‡Ì ̇ ÚÂıÌËÚ ‰ÂÒˆẨÂÌÚÌË ÔÓÂ͈ËË Ë ÙÛÌ͈ËÓ̇ÎÌË ‚˙ÁÍË. Ç „ÂÌÂÁ‡Ú‡ ̇ ÔÓÏÂÌËÚ ̇ Çëó Û˜‡ÒÚ‚‡Ú Ë Ì‡Û¯ÂÌËfl ‚ ËÏÛÌ̇ڇ Ë Ì‚ÓÚ‡ÌÒÏËÚÂÌËÚ ÒËÒÚÂÏË. èˆËÁË‡ÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÒÚ˘̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ Çëó ÔË ÓÚ‰ÂÎÌËÚ Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl ̇·„‡ ÔÓ‚Âʉ‡Ì ̇ ÔÓ-̇ڇÚ˙¯ÌË ËÁÒΉ‚‡ÌËfl Ò ÚÓÁË ÏÂÚÓ‰. ãàíÖêÄíìêÄ 1.

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Pol Merkur Lekarski., 19, 2005, 548552. 44. Meco G, Vanacore N, Locuratolo N, Bonifati V, Vella C, Giovani A, Tubani L, Baratta L, Mastrocola C. Heart rate variability in Parkinson's disease patients treated with tolcapone. Parkinsonism Relat Disord., 6, 2000, 223227. 45. Merico A, Piccione F, Levedianos G, VescovoG, Tonin P. Autonomic and Cardiac Testing in Multiple Sclerosis Patients Complaining Fatigue During Rehabilitative Treatment. Basic Appl Myol. 15,, 2005, 2, 87-92. 46. Merkelbach S, Dillmann U, Kolmel C, Holz I, Muller M. Cardiovascular autonomic dysregulation and fatigue in multiple sclerosis. Mult Scler., 7, 2001, 320-326. 47. Mihci E, Kardelen F, Dora B, Balkan S. Orthostatic heart rate variability analysis in idiopathic Parkinson's disease. Acta Neurol Scand., 113, 2006, 288-293. 48. Oka H, Mochio S, Onouchi K, Morita M, Yoshioka M, Inoue K. Cardiovascular dysautonomia in de novo Parkinson's disease. J Neurol Sci., 241, 2006, 59-65. 49. Pagani M. Heart rate variability and autonomic diabetic neuropathy. Diabetes Nutr Metab., 13, 2000, 341-346. 50. Persson H, Ericson M, Tomson T. Heart rate variability in patients with untreated epilepsy. Seizure, 16, 2007, 504-508. 51. Persson H, Kumlien E, Ericson M, Tomson T. Circadian variation in heartrate variability in localization-related epilepsy. Epilepsia, 48, 2007, 917-922. 52. Persson H, Kumlien E, Ericson M, Tomson T. Preoperative heart rate variability in relation to surgery outcome in refractory epilepsy. Epilepsia, 65, 2005, 1021-1025. 53. Pisano F, Miscio G, Mazzuero G, Lanfranchi P, Colombo R, Pinelli P. Decreased heart rate variability in amyotrophic lateral sclerosis. Muscle Nerve., 1995, 18, 1225-1231. 54. Pradhan C, Yashavantha BS, Pal PK, Sathyaprabha TN. Spinocerebellar ataxias type 1, 2 and 3: a study of heart rate variability. Acta Neurol Scand., 117, 2008, 337-342. 55. Pursiainen V, Haapaniemi TH, Korpelainen JT, Huikuri HV, Sotaniemi KA, Myllyla VV. 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myotonic dystrophy type 1. Acta Myol., 26, 2007, 112-114. 57. Reardon M, Malik M. Changes in heart rate variability with age. Pacing Clin Electrophysiol., 19, 1996, 1863-1866. 58. Robinson RG, Spalletta G, Jorge RE, Bassi A, Colivicchi F, Ripa A, Caltagirone C. Decreased heart rate variability is associated with poststroke depression. Am J Geriatr Psychiatry., 16, 2008, 867-873. 59. Ronkainen E, Ansakorpi H, Huikuri HV, Myllyla VV, Isojarvi JI, Korpelainen JT. Suppressed circadian heart rate dynamics in temporal lobe epilepsy. J Neurol Neurosurg Psychiatry., 76, 2005, 1382-1386. 60. Sztajzel J. Heart rate variability: a noinvasive electrocardiographic method to measure the autonomic nervous system. Swiss med., 134, 2004, 514–522. 61. Task Force of the European Society of Cardiology and the North American Society of Pacing and Electrophysiology. Heart ratevariability. Standards of measurement, physiological interpretation,and clinical use. Circulation, 93, 1996, 1043–1065. 62. Yotsukura M, Fujii K, Katayama A, Tomono Y, Ando H, Sakata K, Ishihara T, Ishikawa K.Nine-year follow-up study of heart rate variability in patients with Duchenne-type progressive muscular dystrophy. Am Heart J., 136, 1998, 289-296.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- ëÚÂÙ͇ å‡ÌÚ‡Ó‚‡, ÇÚÓ‡ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇ ëÅÄãçè ◊ë‚. ç‡ÛÏ” ëÓÙËfl ÛÎ. ◊ã. êÛÒ‚” ‹ 1, 1113 ëÓÙËfl, ÚÂÎ. 9702 214 e-mail: steffmant@abv.bg

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ÑÂÍÂÏ‚Ë 2009

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é·ÁÓ ÑàëíêéîàçéèÄíàà- ÉÖçéíàè-îÖçéíàèçà äéêÖãÄñàà, äéÉçàíàÇçà çÄêìòÖçàü à ÑàÄÉçéëíàäÄ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡1, å ê‡È˜Â‚‡1, à. í˙Ì‚1,2 ç‚ÓÎӄ˘̇ ÍÎËÌË͇, ìåÅÄã ◊ÄÎÂÍ҇̉Ó‚Ò͇”, åî –ëÓÙËfl 2çÓ‚ ·˙΄‡ÒÍË ÛÌË‚ÂÒËÚÂÚ, ä‡Ú‰‡Ú‡ ÔÓ äÓ„ÌËÚ˂̇ ̇Û͇ Ë ÔÒËıÓÎÓ„Ëfl 1

SUMMARY DYSTROPHINOPATHIES- GENOTYPE- PHENOTYPE CORRELATIONS AND COGNITIVE DISORDERS IN PATIENTS WITH DUCHENNE AND BECKER MUSCULAR DYSTROPHIES T Chamova1, M Raytcheva1, I Tournev1,2 1 Neurology clinic, University hospital “Alexandrovska”, Medical Faculty 2 New Bulgarian University, Chair of cognitive sciences and psychology Duchenne and Becker progressive muscular dystrophies (DMD and BMD) are allelic X-linked genetic myopathies, caused by mutations in the dystrophin gene. They are clinically characterized by progressive muscle wasting, loss of walking ability leading to complete wheelchair dependence, cardiomyopathy, and in some of the cases by mental retardation and nanismus. These two forms of dystrophinopathy differ from one another by the age of onset, severity of the clinical features and the progession of the disorder. The non-progressive cognitive disorders with lower scores for verbal skills and short-term memory are specific clinical features for some of the patients with DMD , whereas the patients with BMD have a more heterogeneous cognitive profile, which encompass learning difficulties as well as autism, behavioral and attention problems. The pathogenesis of this disorders is complex, mainly due to mutations in the second part of the gene, that disturb the regulation of the gene expression of the dystrophin isoforms, localized in the central nervous system (Dp140, Dp71, Dp116) and subsequently cause disruption of the neuronal maturation and development, the synaptogenesis and the intracellular homeostasis of the neurons and glial cells.The diagnostic algorithm of dystrophinopathies is complex as the genetic tests are an important diagnostic procedure, used to identify the mutations in the dystrophin gene in patients and in female carriers in order to perform prenatal diagnosis. KEY WORDS: Dystrophinopathies; dystrophin isoforms; cognitive disorders. êÖáûåÖ èÓ„ÂÒË‚ÌËÚ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ Ò‡ ï-Ò‚˙Á‡ÌË Ì‡ÒΉÒÚ‚ÂÌË ÏËÓÔ‡ÚËË, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÏÛÚ‡ˆËË, Á‡Òfl„‡˘Ë ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ. äÎËÌ˘ÌÓ Ò ÔÓfl‚fl‚‡Ú Ò ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‰Ó ÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl, ͇‰ËÓÏËÓÔ‡ÚËfl, ‡ ‚ ÌflÍÓË ÓÚ ÒÎÛ˜‡ËÚÂ Ë Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë Ì‡ÌËÁ˙Ï. ê‡Á΢‡‚‡Ú Ò ÔÓÏÂÊ‰Û ÒË ÔÓ Ì‡˜‡ÎÓÚÓ, ÚÂÊÂÒÚÚ‡ ̇ ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ Ë ÒÍÓÓÒÚÚ‡ ̇ ÔÓ„ÂÒËfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. äÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ Ò ÔÓ-ÚÂÊÍÓ Á‡Òfl„‡Ì ̇ ‚Â·‡ÎÌËÚ ÛÏÂÌËfl Ë Í‡ÚÍÓÒӘ̇ڇ Ô‡ÏÂÚ Ò‡ ÒÔˆËÙ˘̇ ÍÎËÌ˘̇ ÓÒÓ·ÂÌÓÒÚ ÔË ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ, ‰Ó-

͇ÚÓ Á‡ Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ ÍÓ„ÌËÚË‚ÌËflÚ ÔÓÙËÎ Â ÔÓ-ıÂÚÂÓ„ÂÌÂÌ Ë ‚Íβ˜‚‡ Á‡ÚÛ‰ÌÂÌËfl ‚ Ó·Û˜ÂÌËÂÚÓ, ͇ÍÚÓ Ë ‡ÛÚËÁ˙Ï, Ôӂ‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl, ıËÔÂ‡ÍÚË‚ÌÓ Ôӂ‰ÂÌËÂ Ë ‰ÂÙˈËÚ Ì‡ ‚ÌËχÌËÂÚÓ. íÂÁË ÒÏÛ˘ÂÌËfl ̇È-‚ÂÓflÚÌÓ Ò‡ Ò ÍÓÏÔÎÂÍÒ̇ „ÂÌÂÁ‡, ͇ÚÓ ÓÒÌÓ‚ÌÓ Á̇˜ÂÌË ËÏ‡Ú ÏÛÚ‡ˆËË, ‚˙‚ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚Ë̇ „Â̇, ÍÓËÚÓ Ì‡Û¯‡‚‡Ú „Û·ˆËflÚ‡ ̇ „ÂÌ̇ڇ ÂÍÒÔÂÒËfl ̇ ‰ËÒÚÓÙËÌÓ‚ËÚ ËÁÓÙÓÏË, ÎÓ͇ÎËÁË‡ÌË ‚ ñçë (Dp140, Dp71, Dp116) Ë ÔÓ ÚÓÁË Ì‡˜ËÌ Ò˙Áfl‚‡ÌÂÚÓ Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÌÂ‚ÌËÚ ÍÎÂÚÍË, ÒË̇ÔÚÓ„ÂÌÂÁ‡Ú‡ Ë ‚˙ÚÂÍÎÂÚ˙˜Ì‡Ú‡ ıÓÏÂÓÒÚ‡Á‡ ̇ Ì‚ÓÌËÚÂ Ë „ÎËflÚ‡. ᇠ‰Ë‡„ÌÓÒÚˈË‡ÌÂÚÓ Ì‡ èåÑ ÓÒ‚ÂÌ ·ËÓıËÏ˘ÌËÚ ËÁÒΉ‚‡ÌËfl, ÖåÉ Ë ËÏÛÌÓıËÒÚÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl ÓÚ Ò˙˘ÂÒÚ‚ÂÌÓ Á̇˜ÂÌË ҇ Ò˙‚ÂÏÂÌÌËÚ „ÂÌÂÚ˘ÌË ÏÂÚÓ‰Ë, ‡ÁÍË‚‡˘Ë „ÂÌÂÌ ‰ÂÙÂÍÚ Û ·ÓÎÌË, ÌÓÒËÚÂÎÍË Ë ‚ ÛÒÎÓ‚ËflÚ‡ ̇ ÔÂ̇ڇÎ̇ ‰Ë‡ÌÓÁ‡. äãûóéÇà Ñìåà:ÑËÒÚÓÙËÌÓÔ‡ÚËË; ‰ËÒÚÓÙËÌÓ‚Ë ËÁÓÙÓÏË; ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl. èÓ„ÂÒË‚ÌËÚ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË (èåÑ) ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ Ò‡ ̇ÒΉÒÚ‚ÂÌË ÏËÓÔ‡ÚËË, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÏÛÚ‡ˆËË, Á‡Òfl„‡˘Ë ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ, ÎÓ͇ÎËÁË‡Ì ‚ ï ıÓÏÓÁÓχڇ, ÍÎËÌ˘ÌÓ ÔÓfl‚fl‚‡˘Ë ÒÂ Ò ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‰Ó ÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl, ͇‰ËÓÏËÓÔ‡ÚËfl, ‡ ‚ ÌflÍÓË ÓÚ ÒÎÛ˜‡ËÚÂ Ë Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë Ì‡ÌËÁ˙Ï. ê‡Á΢‡‚‡Ú Ò ÔÓÏÂÊ‰Û ÒË ÔÓ Ì‡˜‡ÎÓÚÓ, ÚÂÊÂÒÚÚ‡ ̇ ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ Ë ÒÍÓÓÒÚÚ‡ ̇ ÔÓ„ÂÒËfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. è˙‚ÓÚÓ ÔÓ‰Ó·ÌÓ ÍÎËÌ˘ÌÓ Ë ·ËÓÔÒ˘ÌÓ Ô‡ÚӇ̇ÚÓÏ˘ÌÓ ÓÔËÒ‡ÌË ̇ ÔÓ„ÂÒ˂̇ڇ ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl ͇ÚÓ ◊èÒ‚‰ÓıËÔÂÚÓÙ˘̇ ÏÛÒÍÛÎ̇ Ô‡‡ÎËÁ‡” e ̇ ÙÂÌÒÍËfl Ì‚ÓÎÓ„ Guillaume Benjamin Amand Duchenne (1868) „. Gowers ÓÔËÒ‚‡ ÙÓχڇ Ò ÔÓ-·‡‚ÌÓ ‡Á‚ËÚË ÓÍÓÎÓ Ë ÒΉ ÔÛ·ÂÚÂÚ‡, ÍÓflÚÓ Â ÔÓ‰Ó·ÌÓ ı‡‡ÍÚÂËÁË‡Ì‡ ÓÚ E.Becker (1955). éÚÍËÚËÂÚÓ Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ (Monaco Ë Ò˙‡‚Ú. 1986)  ÓÒÌÓ‚‡Ú‡ Á‡ Ò˙‚ÂÏÂÌÌÓÚÓ ‡Á·Ë‡Ì ̇ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ë ‚˙ÁÏÓÊÌÓÒÚËÚ Á‡ ÚÂ‡ÔËfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÖèàÑÖåàéãéÉàü èåÑ ÚËÔ Ñ˛¯ÂÌ Ò Ò¢‡ Ò ˜ÂÒÚÓÚ‡ 1 ̇ 3500 ÊË‚ÓÓ‰ÂÌË ÏÓϘÂÚ‡. èË 30% ÓÚ Úflı ÏÛÚ‡ˆËflÚ‡ ‚˙ÁÌËÍ‚‡ de novo. èåÑ ÚËÔ ÅÂÍÂ Ò ÔÓfl‚fl‚‡ Ò ˜ÂÒÚÓÚ‡ 1 ̇ 30 000 ÊË‚ÓÓ‰ÂÌË ÏÓϘÂÚ‡. èË 50% ÏÛÚ‡ˆËËÚ ҇ de novo. ÖíàéãéÉàü ÉÂÌ˙Ú ÓÚ„Ó‚ÓÂÌ Á‡ èåÑ ÚËÔ Ñ˛¯ÂÌ Ë ÅÂÍÂ  ÍÎÓÌË‡Ì Ë Í‡ÚË‡Ì ÔÂÁ 1986 „. (60) Ë ÔÓ‰ÛÍÚ˙Ú ÏÛ- ÔÓÚÂËÌ˙Ú ‰ËÒÚÓÙËÌ Â ÓÔËÒ‡Ì ÓÚ ÂÍËÔ‡ ̇ Kunkel ÔÂÁ 1987 (44). ÑËÒÚÓÙËÌÓ‚ËflÚ „ÂÌ Ò ̇ÏË‡ ̇ ï-ıÓÏÓÁÓχڇ ‚ ÎÓÍÛÒ ï21. ë˙ÒÚÓË Ò 2,4 Ï„‡·‡ÁË (mb) ÓÚ Ñçä, Ô‰Ò-


ÑÂÍÂÏ‚Ë 2009

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

Ú‡‚Îfl‚‡ 1% ÓÚ ï ıÓÏÓÁÓχڇ Ë Â Â‰ËÌ ÓÚ Ì‡È-„ÓÎÂÏËÚ „ÂÌË ‚ ˜Ó‚¯ÍËfl „ÂÌÓÏ. äÓ‰Ë‡˘‡Ú‡ ˜‡ÒÚ Á‡ ÒËÌÚÂÁ‡ ̇ ‰ËÒÚÓÙËÌ Â ÓÚ ÓÍÓÎÓ 15 ÍËÎÓ·‡ÁË ËÎË 79 ÂÍÁÓ̇ Ë Ò˙ÒÚ‡‚Îfl‚‡ Ò‡ÏÓ 0,6 % ÓÚ „Â̇ (38, 64). ÖÍÁÓÌËÚ ҇ ÓÚ„‡Ì˘ÂÌË Â‰ËÌ ÓÚ ‰Û„ ˜ÂÁ ËÌÚÓÌË, ÍÓËÚÓ Ò‡ Ò‚˙Á‡ÌË Ò „Û·ˆËflÚ‡ ̇ „ÂÌÂÚ˘̇ڇ ÂÍÒÔÂÒËfl Ë Â‚ÓβˆËflÚ‡ ̇ „ÂÌÂÚ˘ÌËfl ÍÓ‰. ÉÂÌ˙Ú Ò ı‡‡ÍÚÂËÁË‡ Ò˙Ò ÒÎÓÊ̇ „Û·ˆËfl ̇ Ú‡ÌÒÍË·ˆËflÚ‡, ÔÓ‡‰Ë ̇΢ËÂÚÓ Ì‡ ÓÒÂÏ ‡Á΢ÌË ÔÓÏÓÚÓ‡ ÔÓ ˆfl·ڇ ÏÛ ‰˙ÎÊË̇, „ÛÎË‡˘Ë ÒËÌÚÂÁ‡Ú‡ ̇ ‡Á΢ÌË ÔÓ ‰˙ÎÊË̇ ‰ËÒÚÓÙËÌÓ‚Ë ËÁÓÙÓÏË. åÛÚ‡ˆËËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ èåÑ ÚËÔ Ñ˛¯ÂÌ (31;63) Ò‡ „·‚ÌÓ ‰ÂΈËË (ÓÍÓÎÓ 60%), Ô‰ËÏÌÓ ÓÍÓÎÓ ËÌÚÓÌ 7 Ë ‰ÛÔÎË͇ˆËË (ÓÍÓÎÓ 8%); ÓÍÓÎÓ 30% Ò‡ ÚÓ˜ÍÓ‚Ë ÏÛÚ‡ˆËË, ‰ÌÓÌÛÍÎÂÓÚˉÌË Á‡ÏÂÌË, ËÌÒÂˆËË, χÎÍË ‰ÂΈËË Ë ‰ÛÔÎË͇ˆË. èË èåÑ ÚËÔ ÅÂÍÂ 80% ÓÚ ÏÛÚ‡ˆËËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ ·ÓÎÂÒÚÚ‡ Ò‡ ‰ÂΈËË ‚ ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ. ç‡È-˜ÂÒÚÓ ÏÛÚ‡ˆËËÚ Ò ÎÓ͇ÎËÁË‡Ú ‚ ӷ·ÒÚÚ‡ ̇ Ô˙‚ËÚ 20 ÂÍÁÓ̇ (ÓÒÌÓ‚ÌÓ ÓÍÓÎÓ 7 ÂÍÁÓÌ) Ë ÏÂÊ‰Û 44 Ë 45 ÂÍÁÓÌ, Í˙‰ÂÚÓ ËÌÚÓÌËÚ ҇ Ò ÔÓ-„ÓÎflχ ÌÛÍÎÂÓÚˉ̇ ÔÓÒΉӂ‡ÚÂÎÌÓÒÚ. Ç ËÌÚÓÌ 50 Ò‡ „ËÒÚË‡ÌË Ì‡È-ÏÌÓ„Ó ÚÓ˜ÍË Ì‡ ‡ÁÍ˙Ò‚‡Ì ‚ ÔÓÔÛ·ˆËflÚ‡ ̇ ·‡Î͇ÌÒÍËfl ÔÓÎÛÓÒÚÓ‚ (3). Ñ‚Â ÓÒÌÓ‚ÌË ÂÍÓÏ·Ë̇ˆËÓÌÌË Ò˙·ËÚËfl ‚Ó‰flÚ ‰Ó „ÂÌÂË‡Ì ̇ ‰ÂΈËË Ë ËÁfl‚‡ ̇ „ÂÌÂÚ˘ÌË Á‡·ÓÎfl‚‡ÌËfl: ıÓÏÓÎÓÊ̇ ÌÂˆËÔӘ̇ ÂÍÓÏ·Ë̇ˆËfl, ÔÓÓ‰Â̇ ÓÚ ÒıÓ‰ÌË „ÂÌÌË ÔÓÒΉӂ‡ÚÂÎÌÓÒÚË ËÎË ÓÚ ÔÓ‚ÚÓÂÌË ÂÎÂÏÂÌÚË Ë ÌÂıÓÏÓÎÓÊ̇ ÂÍÓÏ·Ë̇ˆËfl, ‚Íβ˜‚‡˘‡ Û˜‡ÒÚ˙ˆË Ò ÏËÌËχÎ̇ ÒÂÍ‚Â̈ËÓÌ̇ ıÓÏÓÎÓ„Ëfl. ÉÓÎÂÏË̇ڇ ̇ ‰ÂΈËflÚ‡ Ì ‚Ë̇„Ë ÍÓÂÎË‡ Ò ÔÓÚÂÊ͇ ÍÎËÌ˘̇ ËÁfl‚‡. ïËÔÓÚÂÁ‡Ú‡ ̇ Monaco Ë Ò˙‡‚Ú. (1986) Ô‡‚Ë ÓÔËÚ ‰‡ Ó·flÒÌË ‡ÁÎËÍËÚ ‚˙‚ ÙÂÌÓÚËÔ̇ڇ ËÁfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. äÓ„‡ÚÓ ‰ÂΈËflÚ‡ ̇Û¯Ë ‡Ï͇ڇ ̇ ˜ÂÚÂÌÂ Ò ÔÓfl‚‡ ̇ ÒÚÓÔ ÍÓ‰ÓÌ, Ò ÒËÌÚÂÁË‡ ‰ÂÙÂÍÚÂÌ, ÔÓ-Í˙Ò ‰ËÒÚÓÙËÌ Ò ÎËÔÒ‡ ̇ ë-ÚÂÏË̇Î, ÍÓÈÚÓ ·˙ÁÓ Ò ‡ÁÛ¯‡‚‡. îÂÌÓÚËÔ̇ڇ ËÁfl‚‡ ̇ Ú‡Í˙‚ ÚËÔ ÏÛÚ‡ˆËË Â èåÑ ÚËÔ Ñ˛¯ÂÌ. äÓ„‡ÚÓ Ì Ò ̇Û¯Ë ‡Ï͇ڇ ̇ ˜ÂÚÂÌ ÒΉ ÏÛÚ‡ˆËflÚ‡, Ò ÒËÌÚÂÁË‡ ‰ËÒÚÓÙËÌ Ò ‡Á΢̇ ÔÓ ‰˙ÎÊË̇ Á‡„Û·‡ ̇ ‚˙Ú¯ÌËfl Ò„ÏÂÌÚ, ÌÓ Á‡Ô‡ÁÂÌË ë Ë NÚÂÏË̇ÎË, ÍÓÂÚÓ Ì ÒÏÛ˘‡‚‡ ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂÚÓ Ò ‰Û„ËÚ ·ÂÎÚ˙ˆË ÓÚ „ÎËÍÓÔÓÚÂËÌ-‡ÒÓˆËË‡ÌËfl ÍÓÏÔÎÂÍÒ Ë Á‡Ô‡Á‚‡ ÓÚÌÓÒËÚÂÎ̇ڇ ÙÛÌ͈ËÓ̇ÎÌÓÒÚ Ì‡ ‰ËÒÚÓÙË̇. íÓ‚‡ Ó·ÛÒ·‚fl ÔÓ-ÎÂ͇ڇ ÙÂÌÓÚËÔ̇ ËÁfl‚‡ ̇ èåÑ ÚËÔ ÅÂÍÂ (32; 65). àÁÍβ˜ÂÌËfl ÓÚ Ô‡‚ËÎÓÚÓ Ò Ì‡Û¯‡‚‡Ì ËÎË Á‡Ô‡Á‚‡Ì ̇ ‡Ï͇ڇ ̇ ˜ÂÚÂÌ Ô‡‚flÚ ÒÎÛ˜‡ËÚÂ, ÔË ÍÓËÚÓ ‰ÂΈËflÚ‡ Á‡Òfl„‡ ÔÓÚÂËÌ-Ò‚˙Á‚‡˘ËÚ ‰ÓÏÂÌË (14). í‡ÍË‚‡ ÏÛÚ‡ˆËË, ÌÂÁ‡‚ËÒËÏÓ ÓÚ Á‡Ô‡Á‚‡ÌÂÚÓ Ì‡ ‡Ï͇ڇ ̇ ˜ÂÚÂÌÂ, ‰Ó‚Âʉ‡Ú ‰Ó ÚÂÊÍÓ Á‡Òfl„‡Ì ̇ ÙÛÌ͈ËflÚ‡ ̇ ‰ËÒÚÓÙË̇, ÂÒÔ. ËÁfl‚‡ ̇ èåÑ ÚËÔ Ñ˛¯ÂÌ (45). èË ÂÍÁÓÌ-ÔÂÒ͇˜‡˘ËÚ ÏÛÚ‡ˆËË ‰ÂΈËËÚÂ, ̇Û¯‡‚‡˘Ë ‡Ï͇ڇ ̇ ˜ÂÚÂÌ ÏÓ„‡Ú ‰‡ Ò ÔÓfl‚fl‚‡Ú ÙÂÌÓÚËÔÌÓ, ͇ÚÓ Ú‡ÍË‚‡ Ò˙Ò Á‡Ô‡ÁÂ̇ ‡Ï͇ ̇ ˜ÂÚÂÌÂ Ë Ó·‡ÚÌÓ (22). èË èåÑ ÏÛÚ‡ˆËËÚ Ò Û̇ÒΉfl‚‡Ú ï-ˆÂÒË‚ÌÓ Ë ·ÓΉۂ‡Ú ÓÒÌÓ‚ÌÓ ÏÓϘÂÚ‡, Á‡˘ÓÚÓ Ò‡ ıÂÏËÁË„ÓÚË ÔÓ ï-ıÓÏÓÁÓχڇ. ÜÂÌËÚ ҇ ÌÓÒËÚÂÎÍË Ë Ô‰‡‚‡Ú Á‡·ÓÎfl‚‡ÌÂÚÓ Ì‡ Ò‚ÓËÚ ÒËÌÓ‚Â Ò ËÒÍ ÓÚ 50% ÓÚ ‡Ê‰‡Ì ̇ ·ÓÎÌÓ ÏÓϘÂ, ‡ 50% ÓÚ ‰˙˘ÂËÚ ̇ ڇ͇‚‡ ÊÂ̇ Ò˙˘Ó Ò‡ ÌÓÒËÚÂÎÍË. Ç ÏÌÓ„Ó ‰ÍË ÒÎÛ˜‡Ë ÊÂÌË ÏÓ„‡Ú ‰‡ ·˙‰‡Ú Á‡Ò„̇ÚË, ÌÓ Ò ÔÓ-ÎÂ͇ ÍÎËÌ˘̇ ËÁfl‚‡ ÔË ÒË̉ÓÏ Ì‡ í˙Ì˙ 45ï0, ‡ÒËÏÂÚ˘̇ Ë̇ÍÚË‚‡ˆËfl ̇ Á‰‡‚‡Ú‡ ï-ıÓÏÓÁÓχ Ë Â‰ÌÓÓ‰ËÚÂÎÒ͇ ‰ËÁÓÏËfl (2, 4, 30) èÄíéÉÖçÖáÄ çÄ åìëäìãçÄíÄ ìÇêÖÑÄ à äéÉçàíàÇçàíÖ çÄêìòÖçàü èêà èåÑ ÑËÒÚÓÙËÌ˙Ú Â ÔÓÚÂËÌ Ò ÏÓÎÂÍÛÎ̇ χ҇ ÓÚ 427 kDa (ÍËÎÓ‰‡ÎÚÓ̇). Ç ‡Á΢ÌËÚ ÒË ËÁÓÙÓÏË Ò ÓÚÍË‚‡ ‚ ÏÛÒÍÛÎËÚÂ, ÂÚË̇ڇ, Ì‚ÓÌËÚÂ Ë „ΡÎÌËÚ ÍÎÂÚÍË ‚

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ñçë, ‚ ÍÎÂÚÍËÚ ̇ ·˙·ˆËÚÂ, ˜ÂÌËfl ‰Ó·, ÂÚË̇ڇ, Ô‡ÌÍ‡҇, ·ÂÎËÚ ‰Ó·Ó‚Â Ë ÚÂÒÚËÒËÚ (61). èӂ˜ÂÚÓ ËÁÓÙÓÏË Ò‡ ÚËÔ˘ÌË Á‡ ‰‡‰ÂÌ ÍÎÂÚ˙˜ÂÌ ÚËÔ, Dp71 Ò ÓÚÍË‚‡ ‚ „ÓÎflχ ˜‡ÒÚ ÓÚ ËÁ·ÓÂÌËÚ Ú˙͇ÌË, ÌÓ ÌÂ Ë ‚ ̇Ô˜ÌÓ̇·‡Á‰ÂÌËÚ ÏÛÒÍÛÎÌË ‚·Í̇. ÑÛ„ËÚ ËÁÓÙÓÏË (Dp71, Dp116, Dp140 Ë Dp260) ËÏ‡Ú ÔÓÏÓÚÓË ‡Á΢ÌË ÓÚ ÚÂÁË Ì‡ ‰ËÒÚÓÙË̇ ̇ ̇Ô˜ÌÓ̇·‡Á‰ÂÌËÚ ÏÛÒÍÛÎÌË ‚·Í̇. ÑËÒÚÓÙËÌ˙Ú Â ˆËÚÓÒÍÂÎÂÚÂÌ ÔÓÚÂËÌ, Ò ‚ËÒÓ͇ ÂÍÒÔÂÒËfl ‚ ÌÂ‚ÌÓÏÛÒÍÛÎÌËÚ ÒË̇ÔÒË Ë ÏÛÒÍÛÎÌÓ-ÒÛıÓÊËÎÌËÚ ‚˙ÁÍË (20). ãÓ͇ÎËÁË‡Ì  ÔÓ ‚˙Ú¯̇ڇ ÔÓ‚˙ıÌÓÒÚ Ì‡ Ò‡ÍÓÎÂχڇ. ä‡ÚÓ ıÓÏÓÚÂÚ‡ÏÂ (25) Ò Ò˙ÒÚÓË ÓÚ: ● ÉÎÓ·Û·ÂÌ ‡ÏËÌÓ-Í‡È, ˜ÂÁ ÍÓÈÚÓ Ò ҂˙Á‚‡ Ò˙Ò Ò‡ÍÓÏÂÌËfl ·ÂÎÚ˙Í ‡ÍÚËÌ; ● ñÂÌÚ‡ÎÂÌ ‰ÓÏÂÌ Ò 24 ÔÓ‚ÚÓ‡, ÔÓ‰Ó·ÂÌ Ì‡ ˆËÚÓÒÍÂÎÂÚÌËfl ÔÓÚÂËÌ ÒÔÂÍÚËÌ. èÓ‚ÚÓËÚ Ò ÔÂÍ˙Ò‚‡Ú ÓÚ ˜ÂÚËË, ·Ó„‡ÚË Ì‡ ÔÓÎËÌ ÌÂÔÓ‚Ú‡fl˘Ë Ò „ËÓÌË, ̇˜ÂÌË Ò‚˙Á‚‡˘Ë; ● ÑÓÏÂÌ, ·Ó„‡Ú ̇ ˆËÒÚÂËÌ; ● ë-ÚÂÏË̇ÎÂÌ ‰ÓÏÂÌ, Ó·‡ÁÛ‚‡˘ ‰ËÒÚÓÙËÌ-‡ÒÓˆËË‡Ì ÍÓÏÔÎÂÍÒ ˜ÂÁ Ò‚˙Á‚‡Ì ̇ ‰ËÒÚÓÙË̇ Ò β-‰ËÒÚÓ„ÎË͇̇. ÑËÒÚÓÙËÌ-‡ÒÓˆËË‡ÌËfl „ÎËÍÓÔÓÚÂËÌÓ‚ ÍÓÏÔÎÂÍÒ Â ÒÎÓÊ̇ ̇‰ÏÓÎÂÍÛÎ̇ Ó„‡ÌËÁ‡ˆËfl, ‚Íβ˜‚‡˘‡ ‰ËÒÚÓÙËÌ, ‰ËÒÚÓ„ÎË͇ÌÓ‚, Ò‡ÍÓ„ÎË͇ÌÓ‚ Ë ÒËÌÚÓÙËÌÓ‚ ÒÛ·ÍÓÏÔÎÂÍÒË. íÓÁË ÍÓÏÔÎÂÍÒ Ë„‡Â ÓÎflÚ‡ ̇ ÏÓÒÚ ÏÂÊ‰Û ˆËÚÓÒÍÂÎÂÚ‡, Ò‡ÍÓÎÂχڇ Ë ·‡Á‡Î̇ڇ ÏÂÏ·‡Ì‡ Ë Ì‡ ÒÚ‡·ËÎËÁ‡ÚÓ ̇ Ò‡ÍÓÎÂχڇ ÔË ÏÛÒÍÛÎ̇ ÍÓÌÚ‡ÍˆËfl, ͇ÚÓ Ô‰ԇÁ‚‡ ÏËÓÙË·ËÎËÚ ÓÚ ÌÂÍÓÁ‡. èË èåÑ ÚËÔ Ñ˛¯ÂÌ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔÓ˜ÚË Ô˙Î̇ ÎËÔÒ‡ ̇ ‰ËÒÚÓÙËÌ. èË èåÑ ÚËÔ ÅÂÍÂ ÚÓÈ Â ‡·ÌÓÏÂÌ ËÎË Ì‡Ï‡ÎÂÌ. íÂÁË Ù‡ÍÚÓË Ó·ÛÒ·‚flÚ Ì‡Û¯Â̇ڇ ÒÚ‡·ËÎÌÓÒÚ Ì‡ ‰ËÒÚÓÙËÌ „ÎËÍÓÔÓÚÂËÌ-‡ÒÓˆËË‡ÌËfl ÍÓÏÔÎÂÍÒ. è‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÔÓÏÂÌËÚ ‚ ̇Ô˜ÌÓ̇·‡Á‰Â̇ڇ ÏÛÒÍÛ·ÚÛ‡ ÔË èåÑ ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÎËÔÒ‡Ú‡ ËÎË ‰ÂÙˈËÚ‡ ̇ ‰ËÒÚÓÙËÌ Ò ӷflÒÌfl‚‡Ú Ò ‰‚ ÓÒÌÓ‚ÌË ıËÔÓÚÂÁË. ● ä‡ÎˆË‚‡ ıËÔÓÚÂÁ‡ (15): ÑËÒÚÓÙËÌÓ‚ËflÚ ‰ÂÙˈËÚ ‚Ó‰Ë ‰Ó ÒÏÛ˘ÂÌË ‚ ͇ΈË‚‡Ú‡ ıÓÏÂÓÒÚ‡Á‡, ÔÓ‡‰Ë ̇Û¯‡‚‡Ì ̇ ÙÛÌ͈ËflÚ‡ ̇ ÏÂÏ·‡ÌÌËÚ ÈÓÌÌËÚ ͇̇ÎË. ìÒÚ‡ÌÓ‚fl‚‡ Ò Ôӂ˯ÂÌ Í‡ÎˆË‚ ËÌÙÎÛÍÒ ‚ Á‡Ò„̇ÚËÚ ÏÛÒÍÛÎÌË ‚·Í̇, ÍÓÈÚÓ ‚ÂÓflÚÌÓ Ô˘ËÌfl‚‡ Úflı̇ڇ ÌÂÍÓÁ‡, ‚ÒΉÒÚ‚Ë ËÌÚÂÌÁËÙˈË‡Ì ̇ ÔÓˆÂÒËÚ ̇ ÔÓ„‡ÏË‡Ì‡ ÍÎÂÚ˙˜Ì‡ ÒÏ˙Ú. è‰ÔÓ·„‡ ÒÂ, ˜Â ͇ΈËflÚ ‡ÍÚË‚Ë‡ ë‡-Á‡‚ËÒËÏË ÔÓÚ‡ÁË Ë ÓÚÍβ˜‚‡ ‚˙ÁÔ‡ÎËÚÂÎÂÌ ÔÓˆÂÒ. ÑËÒÚÓÙËÌ˙Ú Ë„‡Â ÒÚÛÍÚÛ̇ ÓÎfl Ë ÓÒË„Ûfl‚‡ ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂÚÓ ÏÂÊ‰Û Ò‡ÍÓÎÂχڇ Ë ÂÍÒÚ‡ˆÂÎÛ·ÌËfl χÚËÍÒ (14). ãËÔÒ‡Ú‡ ÏÛ ‚Ó‰Ë ‰Ó ÓÒÏÓÚ˘̇ ÌÂÒÚ‡·ËÎÌÓÒÚ ÔË ÏÛÒÍÛÎÌÓ Ò˙Í‡˘ÂÌËÂ Ë ÔÓÒΉ‚‡˘‡ ÌÂÍÓÁ‡. ÑËÒÚÓÙËÌ˙Ú Ò ÓÚÍË‚‡ Ë ‚ Ò˙‰Â˜ÌËÚÂ Ë „·‰ÍËÚ ÏÛÒÍÛÎÌË ÍÎÂÚÍË (62). ë ‰ÂÙˈËÚ‡ ̇ ‰ËÒÚÓÙËÌ Ò ӷflÒÌfl‚‡ ͇‰ËÓÏËÓÔ‡ÚËflÚ‡ ÔË 50% ‰Ó 80% ÓÚ èåÑ ÚËÔ Ñ˛¯ÂÌ (73) Ë ‰ËÒÙÛÌ͈ËflÚ‡ ̇ „·‰Í‡Ú‡ ÏÛÒÍÛ·ÚÛ‡ ‚ ÂÁÛÎÚ‡Ú ÓÚ ÌÂÍÓÁ‡ ̇ ÏÛÒÍÛÎÌËÚ ‚·Í̇, ‰Â„ÂÌÂ‡ˆËfl ̇ ÍÎÂÚ˙˜ÌËÚ ËÏ fl‰‡ Ë Ï‡ÒÚ̇ ËÌÙËÎÚ‡ˆËfl. è‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl ÔË ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚ Ò˙˘Ó  ҂˙Á‡Ì‡ ÓÒÌÓ‚ÌÓ Ò ‰ËÒÚÓÙËÌÓ‚Ëfl ‰ÂÙˈËÚ. Ç ñçë Ò‡ ËÁÓÎË‡ÌË ‡Á΢ÌË ËÁÓÙÓÏË Ì‡ ‰ËÒÚÓÙË�� ÓÚ ÍÓÚË͇ÎÌË Ì‚ÓÌË (10; 16), ÍÎÂÚÍËÚ ̇ Purkinje, (40), „ΡÎÌË ÍÎÂÚÍË (40), ¯‚‡ÌÓ‚Ë ÍÎÂÚÍË (20) (퇷Îˈ‡ 1). 퇷Îˈ‡ 1. ãÓ͇ÎËÁ‡ˆËfl ̇ ËÁÓÙÓÏËÚ ̇ ‰ËÒÚÓÙË̇ ‚ ñçë (26,39) Ç ñçë Ò ÂÍÒÔÂÒË‡Ú ‰‚ ‡ÎÚÂ̇ÚË‚ÌË ËÁÓÙÓÏË Ì‡ ‰ËÒÚÓÙË̇ Ò Ô˙Î̇ ‰˙ÎÊË̇ Ë ÚË- ÍÓ‰Ë‡ÌË ÓÚ


ÑÂÍÂÏ‚Ë 2009

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

‰ËÒÚ‡Î̇ڇ ˜‡ÒÚ Ì‡ „Â̇ (Dp140, Dp71, Dp116). åÓÁ˙˜ÌËflÚ ‰ËÒÚÓÙËÌ Ëχ ‚‡ÊÌÓ Á̇˜ÂÌË Á‡ Ò˙Áfl‚‡ÌÂÚÓ Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÌÂ‚ÌËÚ ÍÎÂÚÍË. ÇÂÓflÚÌÓ Û˜‡ÒÚ‚‡ ‚ ÒË̇ÔÚÓ„ÂÌÂÁ‡Ú‡ Ë ÍÎÂÚ˙˜Ì‡Ú‡ ÏË„‡ˆËfl ÔÓ ‚ÂÏ ̇ ‡Á‚ËÚËÂÚÓ Ì‡ ÌÂ‚̇ڇ ÒËÒÚÂχ (35). àχ ÚÂÓËË, ˜Â ÔË Á‡‚˙¯ÂÌË ÔÓˆÂÒË Ì‡ ‡Á‚ËÚËÂ Ë Ò˙Áfl‚‡ÌÂ, ‰ËÒÚÓÙËÌ˙Ú Û˜‡ÒÚ‚‡ ‚ ‰Ë̇Ï˘̇ڇ ÒË̇ÔÚ˘̇ ÏÓ‰Û·ˆËfl (56; 57) Ë ÔÓ ÚÓÁË Ì‡˜ËÌ ‚ÎËfl ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË. ë˙˘ÂÒÚ‚Û‚‡Ú ‰Ó͇Á‡ÚÂÎÒÚ‚‡, ˜Â ÎËÔÒ‡Ú‡ ËÎË ‰ÂÙˈËÚ˙Ú Ì‡ ‰ËÒÚÓÙËÌ ‚Ó‰flÚ ‰Ó ÒÂËÓÁÌË Ì‡Û¯ÂÌËfl ̇ ‚˙ÚÂÍÎÂÚ˙˜Ì‡Ú‡ ͇ΈË‚‡ ıÓÏÂÓÒÚ‡Á‡. íÓ‚‡ ÏÓÊ ‰‡ ÒÏÛÚË „Û·ˆËflÚ‡ ̇ ÍÎÂÚ˙˜ÌËfl ˆËÍ˙Î, ÊËÁÌÂÒÔÓÒÓ·ÌÓÒÚÚ‡ ̇ ÍÎÂÚÍËÚÂ, Ì‚ӷ·ÒÚ̇ڇ ÏË„‡ˆËfl ‚ ÔÂËÓ‰‡ ̇ ‡Á‚ËÚËÂ Ë ÔÓÒΉ‚‡˘‡Ú‡ ÒË̇ÔÚ˘̇ Ò˄̇ÎËÁ‡ˆËfl ‚ ÔÂËÓ‰‡ ̇ ÁflÎÓÒÚ Ì‡ çë (36). KÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ë ÛÏÒÚ‚ÂÌ ‰ÂÙˈËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ò ‚‡ˇ·ËÎ̇ ˜ÂÒÚÓÚ‡ ÔË ·ÓÎÌËÚÂ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËË (26, 74) Ë Ò‡ ÔÓ-˜ÂÒÚË ÔË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ, ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ (8). äÓ„ÌËÚË‚ÌËÚ ÒÏÛ˘ÂÌËfl Á‡Òfl„‡Ú ÏÂÊ‰Û 20% Ë 50% ÓÚ ·ÓÎÌËÚÂ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò 9% Ô‡ˆËÂÌÚË Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì ÓÚ Ó·˘‡Ú‡ ÔÓÔÛ·ˆËfl (35; 68). èË ·ÓÎÌË Ò èåÑ ÚËÔ ÅÂÍÂ Ò˙˘Ó Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë ÔÒËı˘ÂÒÍË ÓÚÍÎÓÌÂÌËfl (26, 74). äÓ„ÌËÚË‚ÌËflÚ ‰ÂÙˈËÚ Ì ÍÓÂÎË‡ Ò ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë Ì ÔÓ„ÂÒË‡ (16; 40; 58). ᇠԇÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÚÓÁË ÚËÔ ÒÏÛ˘ÂÌËfl ÔË ÚÂÁË Ô‡ˆËÂÌÚË Ò˙˘ÂÒÚ‚Û‚‡Ú ‡Á΢ÌË ıËÔÓÚÂÁË, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ Ò „ÛÔË‡Ú ‚ 3 ÓÒÌÓ‚ÌË Ì‡Ô‡‚ÎÂÌËfl: ‰ÂÙˈËÚ, ÎËÔÒ‡ ËÎË ‡·ÌÓÏÂÌ ‰ËÒÚÓÙËÌ ‚ Ì‚ÓÌËÚ ̇ ñçë, Á‡ ÍÓÂÚÓ Ò‡ ÔÓ‚Âʉ‡ÌË Ì‡È-ÏÌÓ„Ó ËÁÒΉ‚‡ÌËfl; ÏÛÚ‡ˆËË ‚ „ÂÌË, ÎÓ͇ÎËÁË‡ÌË ‚ Ò˙Ò‰ÒÚ‚Ó Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ ‚ ï ıÓÏÓÁÓχڇ; ÏÓÁ˙˜Ì‡ ıËÔÓÍÒËfl ‚ÒΉÒÚ‚Ë ̇ ‰Ëı‡ÚÂÎ̇ڇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. ‡) ÑÂÙˈËÚ, ÎËÔÒ‡ ËÎË ‡·ÌÓÏÂÌ ‰ËÒÚÓÙËÌ ‚ Ì‚ÓÌËÚ ̇ ñçë ● åÛÚ‡ˆËËÚ (‰ÂΈËË Ë ‰ÛÔÎË͇ˆËË) ‚˙‚ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚Ë̇ ̇ „Â̇ ÔÓ-˜ÂÒÚÓ ‚Ó‰flÚ ‰Ó ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËfl (18; 58), χ͇ ˜Â Ë ÔË Ô‡ˆËÂÌÚË Ò ÏÛÚ‡ˆËË ‚ ÔÓÍÒËχÎ̇ڇ ˜‡ÒÚ Ì‡ „Â̇ Ò˙˘Ó ÏÓÊ ‰‡ Ò ÛÒÚ‡ÌÓ‚Ë ‡Á΢ÂÌ ÔÓ ÒÚÂÔÂÌ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ. ● ãËÔÒ‡Ú‡ ̇ Ô˙ÎÌÓ‚ÂËÊÂÌ ‰ËÒÚÓÙËÌ ÏÓÊ ‰‡ ‰Ó‚‰ ‰Ó ÛÏÂÂÌÓ Ì‡Û¯ÂÌË ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË(21; 33; 53; 58). ● à‰ÂÌÚËÙˈË‡ÌË Ò‡ ÚË ‰ËÒÚ‡ÎÌË ‰ËÒÚÓÙËÌÓ‚Ë ËÁÓÙÓÏË: Dp140, Dp71, Dp116. - Dp140  ÏÓÁ˙˜Ì‡ ‰ËÒÚ‡Î̇ ËÁÓÙÓχ, ÂÍÒÔÂÒË‡˘‡ Ò ÔÓ ‚ÂÏ ̇ ÂÏ·ËÓ̇ÎÌËfl ÔÂËÓ‰ ̇ ‡Á‚ËÚË (8; 55; 58). éÚÍË‚‡ ÒÂ Ë ‚ ·˙·ˆËÚÂ. ë˙ÒÚÓË Ò ÓÚ ‰ËÒÚ‡Î̇ڇ ˜‡ÒÚ Ì‡ ˆÂÌÚ‡ÎÌËfl ‰ÓÏÂÌ Ë ÓÚ ë-ÚÂÏË̇ÎÌËfl ‰ÓÏÂÌ, ‚Íβ˜‚‡ Ú. ̇. Ò‚˙Á‚‡˘ „ËÓÌ, ÍÓÈÚÓ ÏÓÊ ‰‡ Û˜‡ÒÚ‚‡ ‚ ÍÎÂÚ˙˜Ì‡Ú‡ Ò˄̇ÎËÁ‡ˆËfl ËÎË ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂÚÓ Ò ‰Û„Ë ÔÓÚÂËÌË. èË Dp140 ̇˜‡ÎÌËflÚ „ËÓÌ Ì‡ Ú‡ÌÒ·ˆËfl Ò ̇ÏË‡ ‚ ÂÍÁÓÌ 51, ÌÓ Ì„ӂËflÚ ÔÓÏÓÚÓ Ë Ô˙‚ËflÚ ÂÍÁÓÌ Ò ̇ÏË‡Ú ‚ „ÓÎÂÏËfl ËÌÚÓÌ ÏÂÊ‰Û 44 Ë 45 ÂÍÁÓÌË. íÓ‚‡  „ËÓÌ, ‚ ÍÓÈÚÓ ˜ÂÒÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰ÂΈËË ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl (33). - Dp71  ÓÒÌӂ̇ڇ ‰ËÒÚÓÙËÌÓ‚‡ ËÁÓÙÓχ, ÂÍÒÔÂÒË‡Ì‡ ‚ ÏÓÁ˙͇ (19; 69; 71). èË Dp71 ÔÓÏÓÚÓ˙Ú Â ÎÓ͇ÎËÁË‡Ì ‚ ËÌÚÓÌ 62, ‡ ̇˜‡ÎÌËflÚ „ËÓÌ Ì‡ Ú‡ÌÒ·ˆËfl Á‡ÔÓ˜‚‡ ÓÚ ÂÍÁÓÌ 63 (13). Dp71  ÚflÒÌÓ Ò‚˙Á‡Ì Ò˙Ò ÒË̇ÔÚ˘̇ڇ Ô·ÁÏÂ̇ ÏÂÏ·‡Ì‡ Ë Ú‡Í‡ ‚ÂÓflÚÌÓ Û˜‡ÒÚ‚‡ ‚ Ô‰‡‚‡ÌÂÚÓ Ì‡ ÌÂ‚ÌËfl ËÏÔÛÎÒ (58). è‰ÔÓ·„‡ ÒÂ, ˜Â ÔÓ‡‰Ë ‡Ì̇ڇ ÏÛ ÂÍÒÔÂÒËfl ‚ Ì‚ÓÌËÚ ÔÓ ‚ÂÏ ̇ ÂÏ·ËÓ̇ÎÌÓÚÓ ‡Á‚ËÚËÂ, ÚÓÈ Ëχ ÓÒÌÓ‚ÓÔÓ·„‡˘‡ ÓÎfl ‚ ÏÓÁ˙˜ÌÓ-

157

퇷Îˈ‡ 1. ãÓ͇ÎËÁ‡ˆËfl ̇ ËÁÓÙÓÏËÚ ̇ ‰ËÒÚÓÙË̇ ‚ ñçë (26,39) àÁÓÙÓÏË äÎÂÚ˙˜Ì‡ ÎÓ͇ÎËÁ‡ˆËfl (ÓÎfl) ̇ ‰ËÒÚÓÙË̇ Dp427M ÉΡÎÌË ÍÎÂÚÍË Ë Ò˙‰Ó‚Ë ẨÓÚÂÎÌË ÍÎÂÚÍË Dp427C äÓÚË͇ÎÌË Ë ıËÔÓ͇ÏÔ‡ÎÌË ÔË‡ÏˉÌË Ì‚ÓÌË Dp427P îÂÚ‡ÎÂÌ ˆÂ·‡ÎÂÌ ÍÓÚÂÍÒ ÍÎÂÚÍË Ì‡ Purkinje ‚ ˆÂ·ÂÎÛχ Dp260 êÂÚË̇ Dp140 ● ÄÒÚӄΡÎÌË ÔÓˆÂÒË ÔÓ ‚ÂÏ ̇ ‡Á‚ËÚËÂÚÓ ● èÓ‚˙ıÌÓÒÚ Ì‡ ÎÂÔÚÓÏÂÌËÌ„ËÚ ● èÓ ıÓ‰‡ ̇ ÔÓÌËÍ‚‡˘ËÚ Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ● ç‚ÓıËÔÓÙËÁ‡ Dp116 ● ŇÁ‡ÎÌË „‡Ì„ÎËË ● åËÂÎËÌÓ‚ËÚ ӷ‚Ë‚ÍË Ì‡ ÔÂËÙÂÌËÚ ÌÂ‚Ë Dp71 ● ê‡ÌÌË ÂÏ·ËÓ̇ÎÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ● åÂÁÂÌıËÏÌÓ-ÂÔËÚÂÎÌË ‚˙ÁÍË ● ÖÏ·ËÓ̇ÎÂÌ ÏÓÁ˙˜ÂÌ ÒÚ‚ÓÎ ● ÑËÙÂÂ̈ˇˆËfl ̇ ÏÓÁ˙͇ ‚ ÔÂË̇ڇÎÌËfl ÔÂËÓ‰ ● êÂÚË̇ ● ÖÏ·ËÓ̇ÎÂÌ Ô‰ÂÌ ÏÓÁ˙Í ● åÓÁ˙˜Ì‡ ÍÓ‡ ● á˙ÌÂÒÚË ÍÎÂÚÍË ‚ ıËÔÓ͇ÏÔ‡ Ë gyrus dentatus ● ïËÔÓÙËÁ‡ ÚÓ Ò˙Áfl‚‡Ì (47; 50). éÒÌӂ̇ڇ ÎÓ͇ÎËÁ‡ˆËfl ̇ Dp71  ‚ ıËÔÓ͇ÏÔ‡ÎÌËfl gyrus dentatus, ÍÓÂÚÓ Ì‡Î‡„‡ Òı‚‡˘‡ÌÂÚÓ, ˜Â Ú‡ÁË ‰ËÒÚÓÙËÌÓ‚‡ ËÁÓÙÓχ  ҂˙Á‡Ì‡ Ò Ô‡ÏÂÚÓ‚ËÚ ÙÛÌ͈ËË (41). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÏÛÚ‡ˆËË, Á‡Òfl„‡˘Ë ÔÓÏÓÚÓÌËflÚ Û˜‡ÒÚ˙Í Ì‡ Dp71„Â̇, ÔÓ„¯ÌÓÒÏËÒÎÂÌË ÏÛÚ‡ˆËË Ë Ú‡ÍË‚‡, ‚Ó‰Â¯Ë ‰Ó ÚÂÏË̇ˆËfl ̇ Ú‡ÌÒ·ˆËflÚ‡ Ò‡ Ò‚˙Á‡ÌË Ò ÛÏÂÂÌÓ ËÎË ÚÂÊÍÓ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì ̇ Ô‡ˆËÂÌÚËÚÂ. - äÓ‰Ë‡˘‡Ú‡ ÒÂÍ‚Â̈Ëfl Á‡ Dp116 Á‡ÔÓ˜‚‡ ÓÚ ÂÍÁÓÌ 57. Dp116 ‰ËÒÚÓÙËÌ˙Ú ‚Íβ˜‚‡ ‰ËÒÚ‡Î̇ڇ ˜‡ÒÚ ÓÚ ˆÂÌÚ‡ÎÌËfl ‰ÓÏÂÌ (‰‚‡ Ô˙ÎÌË ÔÓ‚ÚÓ‡), ·Ó„‡ÚËfl ̇ ˆËÒÚÂËÌ „ËÓÌ Ë Í‡·ÓÍÒË-ÚÂÏË̇·. ãËÔÒ‡Ú‡ ̇ ‡ÍÚËÌ-Ò‚˙Á‚‡˘ ‰ÓÏÂÌ Ô‰ÔÓ·„‡, ˜Â ÓÒÌӂ̇ڇ ÙÛÌ͈Ëfl ̇ Ú‡ÁË ËÁÓÙÓχ Ò˄̇Î̇ڇ Ú‡ÌÒ‰Û͈Ëfl Ë Ìflχ Á̇˜Ëχ ÓÎfl Á‡ ÏÂı‡Ì˘̇ڇ ÒÚ‡·ËÎËÁ‡ˆËfl ̇ Ò‡ÍÓÎÂχڇ ̇ ÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË. éÚÍË‚‡ Ò ‚ ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË ÔÓ ‚ÂÏ ̇ Í˙ÒÌËÚ ÂÚ‡ÔË Ì‡ ÂÏ·ËÓ̇ÎÌÓÚÓ ‡Á‚ËÚË ̇ ÏÓÁ˙͇. (57; 59). àÁÒΉ‚‡ÌËfl, ̇Ô‡‚ÂÌË ‚ ÔÓÒΉÌËÚ „Ó‰ËÌË ÛÒÚ‡ÌÓ‚fl‚‡Ú ÍÓ·ˆËfl ÏÂÊ‰Û ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ Ë ÏÛÚ‡ˆËËÚ ‚ Dp71, Dp116 Ë Dp140 ËÁÓÙÓÏËÚÂ. ç fl‰ÍÓ ‰ÂΈËË ‚ ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ Á‡Òfl„‡Ú „Û·ÚÓÌËfl „ËÓÌ Ì‡ Dp71 Ë ÍÓ‰Ë‡˘ËÚ ÒÂÍ‚Â̈ËË Ì‡ Dp116 Ë Dp140, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, ÔÓ‡‰Ë ÎËÔÒ‡Ú‡ ̇ ÏÓÁ˙˜ÌËÚ ËÁÓÙÓÏË ‚ Ì‚ÓÌËÚ (35). çflÍÓË ‡‚ÚÓË ÔÓ‰˜ÂÚ‡‚‡Ú ÚflÒ̇ڇ ‚˙Á͇ ÏÂÊ‰Û ÏÛÚ‡ˆËËÚ ‚ „Û·ÚÓÌËfl „ËÓÌ Ì‡ Dp140 Ë ÛÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡Ì ÔË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ ÅÂÍÂ (9). ÑÂÙˈËÚ˙Ú ËÎË ÎËÔÒ‡Ú‡ ̇ ‰ËÒÚÓÙËÌ ‚ÂÓflÚÌÓ Ò‡ ‚ ÓÒÌÓ‚‡Ú‡ ̇ ÏÓÁ˙˜ÌËÚ ÒÚÛÍÚÛÌË ‡·ÌÓÏÌÓÒÚË ÔË Ú‡ÍË‚‡ Ô‡ˆËÂÌÚË: Á‡„Û·‡ ̇ Ì‚ÓÌË, ıÂÚÂÓÚÓÔËË, „ÎËÓÁ‡ Ì‚ÓÙË·ËÎÂÌË ‚Íβ˜‚‡ÌËfl, Á‡„Û·‡ ̇ ÍÎÂÚÍË Ì‡ Purkinje, ‰ẨËÚÌË ‡·ÌÓÏÌÓÒÚË, ̇Û¯Â̇ ˆËÚÓ‡ıËÚÂÍÚÓÌË͇, ÔÂËÌÛÍ·̇ ‚‡ÍÛÓÎËÁ‡ˆËfl (6; 46). ● è‰ÔÓ·„‡ ÒÂ, ˜Â ‰ËÒÚÓÙËÌÓÔÓ‰Ó·ÌËÚÂ Ë ‰ËÒÚÓÙËÌ-‡ÒÓˆËË‡ÌËÚ ÔÓÚÂËÌË Ò˙˘Ó ËÏ‡Ú ÓÚÌÓ¯ÂÌË Í˙Ï ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË. íÓ‚‡ Ò‡ ·ÏËÌËÌ, ÏÂÓÁËÌ, ÔÂÎÂ͇Ì, ‡„ËÌ, ·Ë„ÎËÍ‡Ì Ë ÌÓ‚ÓÓÚÍËÚËflÚ Ì‚ÂÍÒËÌ, ÍÓËÚÓ ËÏ‡Ú ÓÚÌÓ¯ÂÌË Í˙Ï ÏÂʉÛÍÎÂÚ˙˜Ì‡Ú‡ Ò˄̇ÎËÁ‡ˆËfl (42; 66; 69). ÑËÒÚÓÙË̇ÒÓˆËË‡ÌËÚ ÔÓÚÂËÌË α Ë β-‰ËÒÚÓ·‚ËÌ Ò‡ ‡ÁÔÓÒÚ‡ÌÂÌË ‚ ÏÓÁ˙͇. α-ÑËÒÚÓ·‚ËÌ˙Ú ÒÂ


ÑÂÍÂÏ‚Ë 2009

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

ËÁÓÎË‡ ÓÚ „ΡÎÌËÚË ÍÎÂÚÍË, ‰Ó͇ÚÓ β-‰ËÒÚÓ·‚ËÌ˙Ú- ÓÚ Ì‚ÓÌË Ë Ëχ ‚ÂÓflÚ̇ Ò˄̇Î̇ ÙÛÌ͈Ëfl. ÑËҷˉËÌ˙Ú Â Ò‚˙Á‡Ì Ò β-‰ËÒÚÓ·‚Ë̇ ‚ ÏÓÁ˙͇ Ë Ò α-‰ËÒÚÓ·‚Ë̇ ‚ ̇Ô˜ÌÓÌÓ·‡Á‰Â̇ڇ ÏÛÒÍÛ·ÚÛ‡. ● ê‰ۈË‡Ì  „βÍÓÁÌËflÚ ÏÂÚ‡·ÓÎËÁ˙Ï ‚ ӷ·ÒÚË Ì‡ ñçë Ò ‚ËÒÓ͇ ÂÍÒÔÂÒËfl ̇ ‰ËÒÚÓÙËÌ Í‡ÚÓ Ï‰ˇÎÌËÚ ÚÂÏÔÓ‡ÎÌË ÒÚÛÍÚÛË, χÎÍËfl ÏÓÁ˙Í, ÒÂÌÁÓÏÓÚÓÌËfl Ë Î‡ÚÂ‡ÎÌËfl ÚÂÏÔÓ‡ÎÂÌ ÍÓÚÂÍÒ ‚ ‰flÒ̇ڇ ıÂÏËÒÙÂ‡. êÓÎflÚ‡ ̇ ‰ËÒÚÓÙË̇ ÔÓ ÓÚÌÓ¯ÂÌË ̇ „βÍÓÁÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï ÓÒÚ‡‚‡ ÌÂflÒ̇. ë˙˘ÂÒÚ‚Û‚‡ ÚÂÓËfl, ÒÔÓ‰ ÍÓflÚÓ ‰ËÒÚÓÙËÌ˙Ú ÏÓÊ ‰‡ Û˜‡ÒÚ‚‡ ‚ Ò˄̇Î̇ڇ Ú‡ÌÒ‰Û͈Ëfl ÓÚ ÂÍÒÚ‡ˆÂÎÛ·ÌÓÚÓ ÔÓÒÚ‡ÌÒÚ‚Ó Í˙Ï ÍÎÂÚ˙˜ÌÓÚÓ fl‰Ó. ● èӂ˯ÂÌÓ Â ÌË‚ÓÚÓ Ì‡ ÌÂÓ„‡Ì˘ÌËÚ ÙÓÒÙ‡ÚË Ë Â Ì‡Ï‡ÎÂÌÓ ÍÓ΢ÂÒÚ‚ÓÚÓ Ì‡ Äíî ‚ ÌÂ‚ÌËÚ ÍÎÂÚÍË. ìÒÚ‡ÌÓ‚fl‚‡Ú Ò ‚ËÒÓÍË ÌË‚‡ ̇ ıÓÎËÌ-Ò˙‰˙ʇ˘Ë ÒÛ·Òڇ̈ËË ‚˙‚ ÙÓÌÚ‡ÎÌËfl ÍÓÚÂÍÒ (7). ● è‰ÔÓ·„‡ ÒÂ, ˜Â Ò˙˘ÂÒÚ‚Û‚‡ ‚˙Á͇ ÏÂÊ‰Û ÙÛÌ͈ËÓÌË‡ÌÂÚÓ Ì‡ ÉÄåäÄ ˆÂÔÚÓËÚ („‡Ï‡-‡ÏËÌÓχÒÎÂ̇ ÍËÒÂÎË̇) Ë ÏÓÁ˙˜ÌËÚ ‰ËÒÚÓÙËÌË, ÛÒÚ‡ÌÓ‚Â̇ ÔË ÓÔËÚÌË Ï˯ÍË-ÏÓ‰ÂÎË. íÂÁË ˆÂÔÚÓË ËÏ‡Ú Á̇˜ÂÌË Á‡ ÔÓ‰˙ʇÌÂÚÓ Ì‡ ÓÚ‰ÂÎÌËÚ هÁË Ì‡ Ò˙Ìfl, ‚˙Á·Û‰ËÏÓÒÚÚ‡ Ë ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ Ì‚ÓÌËÚÂ. ëΉӂ‡ÚÂÎÌÓ ‰ËÒÙÛÌ͈ËflÚ‡ ̇ ÉÄåäÄ ˆÂÔÚÓËÚ ÏÓÊ ‰‡  Ô˘Ë̇ Á‡ ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËË (49). ·) åÛÚ‡ˆËË ‚ „ÂÌË, ÎÓ͇ÎËÁË‡ÌË ‚ Ò˙Ò‰ÒÚ‚Ó Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ ‚ ï ıÓÏÓÁÓχڇ, ÍÓËÚÓ ËÏ‡Ú Á̇˜ÂÌË Á‡ ÛÏÒÚ‚ÂÌÓÚÓ ‡Á‚ËÚË ( XLRM, ACSL4, NXT2, GUCY2F, KCNE1L)( 12). ‚) åÓÁ˙˜Ì‡ ıËÔÓÍÒËfl, ‚ÒΉÒÚ‚Ë ÔÓ„ÂÒË‡˘‡Ú‡ Ò··ÓÒÚ Ì‡ ‰Ëı‡ÚÂÎ̇ڇ ÏÛÒÍÛ·ÚÛ‡ (37) Ëχ ‰ÓÔ˙ÎÌËÚÂÎÌÓ Á̇˜ÂÌË ̇ ÔÓ-Í˙ÒÌËÚ ÂÚ‡ÔË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÒΉ Á‡„Û·‡Ú‡ ̇ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓıӉ͇. äãàçàóçÄ ïÄêÄäíÖêàëíàäÄ çÄ ÑàëíêéîàçéèÄíààíÖ èåÑ íàè ÑûòÖç èÓ˜ÚË Ô˙Î̇ڇ ÎËÔÒ‡ ̇ ‰ËÒÚÓÙËÌ ‚ ̇Ô˜ÌÓ̇·‡Á‰Â̇ڇ ÏÛÒÍÛ·ÚÛ‡ ÓÔ‰ÂÎfl ı‡‡ÍÚÂ̇ڇ Ë ‰Ó· ËÁ‚ÂÒÚ̇ ÍÎËÌË͇ ̇ èåÑ ÚËÔ Ñ˛¯ÂÌ Ò ‡ÌÂÌ Ë ·˙ÁÓ ÔÓ„ÂÒË‡˘ ÒËÏÂÚ˘ÂÌ ÏËÓÔ‡ÚÂÌ ÒË̉ÓÏ Ò ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ò Ì‡˜‡ÎÓ ÔÂ‰Ë 5-„Ӊ˯̇ ‚˙Á‡ÒÚ Ë Á‡„Û·‡ ̇ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ ıÓ‰flÚ Ò‡ÏÓÒÚÓflÚÂÎÌÓ ÔÂ‰Ë 13 „.‚˙Á‡ÒÚ. ÄÌ„‡ÊË‡ Ò Ô‰ËÏÌÓ ÔÓÍÒËχÎ̇ڇ ÏÛÒÍÛ·ÚÛ‡ Ô˙‚Ó Ì‡ ‰ÓÎÌËÚ Í‡ÈÌˈË, ‡ ‚ ÔÓÒΉÒÚ‚ËÂ Ë Ì‡ „ÓÌËÚ Í‡ÈÌˈË, Ô‡‡‚ÂÚ·‡Î̇ڇ Ë ¯ËÈ̇ ÏÛÒÍÛ·ÚÛ‡. ï‡Ô‡ÍÚÂÌË Ò‡ ÔÒ‚‰ÓıËÔÂÚÓÙËfl ̇ m. triceps surae, ‡ ̇ ÔÓ-Í˙ÒÌËÚ ÂÚ‡ÔË ‡ÚÓÙËËÚ ̇ ‰ËÒÚ‡ÎÌËÚ ÏÛÒÍÛÎË Ì‡ ‰ÓÎÌËÚÂ, „ÓÌËÚ Í‡ÈÌËˆË Ë ÙÎÂÍÒÓËÚ ̇ ¯ËflÚ‡, ÍÓÌÚ‡ÍÚÛËÚ ̇ „ÎÂÁÂÌÌËÚ ÒÚ‡‚Ë Ë ‡ıËÎÂÒÓ‚ÓÚÓ ÒÛıÓÊËÎËÂ, ‡ ‚ ÔÓÒΉÒÚ‚ËÂ Ë ÍÓÎÂÌÌË, ·Í˙ÚÌË, ‡ÏÂÌÌË, Ú‡Áӷ‰ÂÌË Ë ËÌÚÂ‚ÂÚ·‡ÎÌË ÒÚ‡‚Ë (1). äËÙÓÒÍÓÎËÓÁ‡Ú‡ Ë Ò··ÓÒÚÚ‡ ̇ ËÌÚÂÍÓÒÚ‡ÎÌËÚ ÏÛÒÍÛÎË Ë ‰Ë‡Ù‡„χڇ ̇Û¯‡‚‡Ú ‚˙̯ÌÓÚÓ ‰Ë¯‡ÌÂ. ê‡Á‚Ë‚‡ Ò ÂÒÚËÍÚË‚ÂÌ ÚËÔ Ì‡Û¯ÂÌË ̇ ‰Ë¯‡ÌÂÚÓ, ÔÓ„ÂÒË‡˘‡ ıËÔÓÍÒÂÏËfl Ë ıËÔÂ͇ÔÌËfl Ò ÎÂÒ̇ ÛÏÓflÂÏÓÒÚ, ̇Û¯ÂÌËfl ̇ Ò˙Ìfl Ë „·‚Ó·ÓÎËÂ. ç‡‡ÒÚ‚‡ ËÒÍ˙Ú ÓÚ ÒÏ˙Ú, ÔÓ‡‰Ë ÓÒÚ‡ ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÔÓ‚ÓÍË‡Ì‡ ÓÚ ËÌÚÂÍÛÂÌÚÌË ËÌÙÂ͈ËË. 臈ËÂÌÚËÚ ҇ Ò˙Ò ÒÌËÊÂÌ ‚ËÚ‡ÎÂÌ Í‡Ô‡ˆËÚÂÚ, ˜ÂÒÚË ÔÌ‚ÏÓÌËË Ë ‡Á‚Ë‚‡Ú ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÍÓÂÚÓ Ì‡Î‡„‡ ‰Ëı‡ÚÂÎ̇

158

‡ÌËχˆËfl ‚ Í‡ÈÌËÚ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡. ä‡‰ËÓÏËÓÔ‡ÚËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 50% ‰Ó 80% ÓÚ Ô‡ˆËÂÌÚËÚ ‚ ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò Í‡‰ËÓÏ„‡ÎËfl, ÔÂÒËÒÚË‡˘‡ Ú‡ıË͇‰Ëfl, ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËfl Ë ÓÒÚË ÏËÓ͇‰ÌË ËÌÙ‡ÍÚË (73). ëËÏÔÚÓÏËÚ ÓÚ ‰ËÒÙÛÌ͈Ëfl ̇ „·‰Í‡Ú‡ ÏÛÒÍÛ·ÚÛ‡ Ò ËÁfl‚fl‚‡Ú Ò ıËÔÓÏÓÚËÎËÚÂÚ Ì‡ ÒÚÓχı‡ Ë/ËÎË ı‡ÌÓÔÓ‚Ó‰‡, χ··ÒÓ·ˆËÓÌÂÌ ÒË̉ÓÏ, Ï„‡ÍÓÎÓÌ Ë ÍÓÌÒÚËÔ‡ˆËfl. èË „ÓÎflχ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ. ë‰ÌËflÚ ÍÓÂÙˈËÂÌÚ Ì‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ (äà)  85, Ò ‚‡ˇˆËË ÏÂÊ‰Û 40 Ë 130 (23, 59). èË·ÎËÁËÚÂÎÌÓ 25% Ò‡ Ò äà ÔÓ‰ 70. èÓ-ÚÂÊÍÓ Ò‡ Á‡Ò„̇ÚË ‚Â·‡ÎÌËÚ ÛÏÂÌËfl, ‚ Ò‡‚ÌÂÌËÂ Ò Ì‚Â·‡ÎÌËÚ (58). íÓÁË ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ Ò ҂˙Á‚‡ Ò Ì‡Û¯ÂÌËfl ‚ ÌÓχÎ̇ڇ ‡ıËÚÂÍÚÓÌË͇ ̇ ÍÓÚË͇ÎÌËÚ Ì‚ÓÌË Ë ÚÂıÌËÚ ‰ẨËÚË, ÍÓËÚÓ ÌÓχÎÌÓ ÂÍÒÔÂÒË‡Ú ‰ËÒÚÓÙËÌ. ç‡ Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl. íÂÊÂÒÚÚ‡ ̇ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì Ì ÍÓÂÎË‡ Ò ÚÂÊÂÒÚÚ‡ ̇ ÏÛÒÍÛÎÌÓ Á‡Òfl„‡ÌÂ Ë Á‡ ‡ÁÎË͇ ÓÚ ÏÛÒÍÛÎ̇ڇ Ò··ÓÒÚ Ì ÔÓ„ÂÒË‡ Ò ‚ÂÏÂÚÓ (72). Hinton Ë Ò˙‡‚Ú. (2000) ËÁ͇Á‚‡Ú ÚÂÓËflÚ‡, ˜Â ÔÓ-ÚÂÊÍÓÚÓ Ì‡Û¯ÂÌË ̇ ‚Â·‡ÎÌËÚ ÛÏÂÌËfl  ÂÁÛÎÚ‡Ú ÓÚ Á‡Òfl„‡Ì ̇ Í‡ÚÍÓÒӘ̇ڇ Ô‡ÏÂÚ. Ñ˙΄ÓÒӘ̇ڇ Ô‡ÏÂÚ Ì  ÚÂÊÍÓ Á‡Ò„̇ڇ. Wicksell Ë Ò˙‡‚Ú.(2004) ÓÚÍË‚‡Ú ‰ÂÙˈËÚ ‚ ‡ÍÚ˂̇ڇ ‡·ÓÚ̇ Ô‡ÏÂÚ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË. í ËÁ͇Á‚‡Ú ÚÂÓËfl Á‡ Â‰Ì‡Í‚Ó ÚÂÊÍÓ Á‡Òfl„‡Ì ̇ ÍÓÌÒÚÛÍÚË‚ÌËÚÂ Ë ‚Â·‡ÎÌËÚ ÛÏÂÌËfl. 臈ËÂÌÚËÚ ËÁÓÒÚ‡‚‡Ú ‚ ÔÓˆÂÒ‡ ̇ ̇ۘ‡‚‡Ì ‰‡ ˜ÂÚ‡Ú, Ô˯‡Ú Ë ‚ χÚÂχÚ˘ÂÒÍËÚ ÒË ÛÏÂÌËfl (48; 52). íÛ‰ÌÓÒÚËÚ ÔË ˜ÂÚÂÌ Ò ‡ÒÓˆËË‡Ú Ò ‡·ÌÓÏÌÓÒÚË ‚ ‰ÂÒÌËfl ˆÂ·ÂÎÛÏ (28). ÑËÒÎÂÍÒËflÚ‡, ÍÓflÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ, ‚ÂÓflÚÌÓ Â Ò‚˙Á‡Ì‡ Ò˙Ò ÒÏÛ˘ÂÌËfl ‚ ÒË̇ÔÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÌË‚Ó ˆÂ·ÂÎÛÏ ÔË ‡·ÌÓÏ̇ ÂÍÒÔÂÒËfl ̇ ‡-‰ËÒÚÓ„ÎË͇̇ (66). Ç ‰ÍË ÒÎÛ˜‡Ë Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ÏÓ„‡Ú ‰‡ ËÏ‡Ú Ë ‰Û„Ë ï-Ò‚˙Á‡ÌË Á‡·ÓÎfl‚‡ÌËfl, ͇ÚÓ ÔË„ÏÂÌÚÂÌ ÂÚËÌËÚ, ıÓÌ˘̇ „‡ÌÛÎÓχÚÓÁ̇ ·ÓÎÂÒÚ Ë ·ÓÎÂÒÚ Ì‡ McLeod (ÒË̉ÓÏ Ì‡ Ì‚Ӈ͇ÌÚÓˆËÚÓÁ‡), „ÎˈÂÓÎ-ÍË̇ÁÂÌ ‰ÂÙˈËÚ ËÎË ‡‰Â̇Î̇ ıËÔÓÔ·ÁËfl, ‚ ÂÁÛÎÚ‡Ú Ì‡ ‡Á΢ÌË ‰ÂΈËË. èÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ÊË‚ÓÚ‡ ÔË ÏÓϘÂÚ‡ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ Â Ò‰ÌÓ 20-25 „Ó‰ËÌË. ç‡È-˜ÂÒÚÓ Ô˘ËÌË Á‡ ÎÂÚ‡ÎÌËfl ËÁıÓ‰ Ò‡ ·ÂÎÓ‰Ó·ÌË ËÌÙÂ͈ËË Ë Ò˙‰Â˜Ì‡ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. èåÑ íàè ÅÖäÖê ï‡‡ÍÚÂËÁË‡ ÒÂ Ò ÔÓ-Í˙ÒÌÓÚÓ Ì‡˜‡ÎÓ Ë ÔÓ-ÎÂ͇ڇ ÍÎËÌ˘̇ ͇ÚË̇ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ÔÓ‡‰Ë ̇΢ËÂÚÓ Ì‡ ‰ËÒÚÓÙËÌ ‚ ‰ۈË‡ÌË ÍÓ΢ÂÒÚ‚‡ ËÎË ‡·ÌÓÏÂÌ ÔÓ ÒÚÛÍÚÛ‡. 燘‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÒΉ 7-„Ӊ˯̇ ‚˙Á‡ÒÚ. èÓÒÚÂÔÂÌÌÓ Ò ‡Á‚Ë‚‡ ÔÓ„ÂÒ˂̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Í‡ÚÓ ÔÓÍÒËχÎÌËÚ ÏÛÒÍÛÎË Ò‡ ÔÓ-ÚÂÊÍÓ Á‡Ò„̇ÚË ÓÚ ‰ËÒÚ‡ÎÌËÚÂ, ‡ ‰ÓÎÌËÚ Í‡ÈÌˈË- ÔÓ-‡ÌÓ ÓÚ „ÓÌËÚÂ. óÂÒÚ ÒËÏÔÚÓÏ Â ÔÒ‚‰ÓıËÔÂÚÓÙËflÚ‡ ̇ ÔÓ‰·Â‰ˈËÚÂ. ë··ÓÒÚÚ‡ ‚ m. quadriceps femoris ÏÓÊ ‰˙Î„Ó ‚ÂÏ ‰‡ ·˙‰Â ‰ËÌÒÚ‚Â̇ڇ ËÁfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. Ç ÒÎÛ˜‡ËÚÂ, ÔË ÍÓËÚÓ „ÂÌÌËÚ ÏÛÚ‡ˆËË ‚Ó‰flÚ ‰Ó ‰ÂÙÂÍÚË ‚ ‡ÏËÌÓ-Í‡fl ̇ ‰ËÒÚÓÙËÌÓ‚‡Ú‡ ÏÓÎÂÍÛ·, Á‡·ÓÎfl‚‡ÌÂÚÓ ÏÓÊ ‰‡ Ò ÔÓfl‚Ë Ò ÏÛÒÍÛÎÌË Í‡ÏÔË, ÔÓ-ÎÂÒ̇ ÛÏÓflÂÏÓÒÚ ÔË ÙËÁ˘ÂÒÍË ÛÒËÎËfl, ÏËÓ„ÎÓ·ËÌÛËfl Ë ‡ÒËÏÔÚÓÏÌÓ Û‚Â΢Â̇ äîä (Í‡ÚËÌÙÓÒÙÓÍË̇Á‡). ëÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓıӉ͇ Ò „Û·Ë ÒΉ 16-„Ӊ˯̇ ‚˙Á‡ÒÚ, ‚ ÌflÍÓË ÒÎÛ˜‡Ë ÒΉ 30-35 „Ӊ˯̇ ‚˙Á‡ÒÚ. èË ÚÂÁË Ô‡ˆËÂÌÚË Í‡‰ËÓÏËÓÔ‡ÚËËÚÂ Ë ÛÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡Ì ҇ ÔÓ-


ÑÂÍÂÏ‚Ë 2009

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

‰ÍË Ë ÔÓ-ÎÂÍË. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ ËÏ‡Ú ÔÓ-χÎÍÓ ıÓÏÓ„ÂÌÂÌ ÍÓ„ÌËÚË‚ÂÌ ÙÂÌÓÚËÔ ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ. Ç Ò‡‚ÌÂÌËÂ Ò Ó·˘‡Ú‡ ÔÓÔÛ·ˆËfl, Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ, Ò‡ Ò ÔÓ-‚ËÒÓ͇ ˜ÂÒÚÓÚ‡ ̇ Á‡ÚÛ‰ÌÂÌËfl ‚ Ó·Û˜ÂÌËÚÓ, ‡ÛÚËÁ˙Ï, Ôӂ‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl, ıËÔÂ‡ÍÚË‚ÌÓ Ôӂ‰ÂÌËÂ Ë ‰ÂÙˈËÚ Ì‡ ‚ÌËχÌËÂÚÓ. èË ÔÓ‚Âʉ‡ÌÂÚÓ Ì‡ Ì‚ÓÔÒËıÓÎӄ˘ÂÒÍË ËÁÒΉ‚‡ÌËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔÓ-Ò··Ó Ô‰ÒÚ‡‚flÌ ̇ Á‡‰‡˜Ë, Ò‚˙Á‡ÌË Ò ‚Â·‡Î̇ Ô‡ÏÂÚ Ë ÏÓÚÓÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌË ÛÏÂÌËfl (9; 74). àÌÚÂωËÂÌËflÚ ÚËÔ ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl Ò ı‡‡ÍÚÂËÁË‡ Ò Ì‡˜‡ÎÓ Ì‡ ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ ÏÂÊ‰Û 5 Ë 7-„Ӊ˯̇ ‚˙Á‡ÒÚ Ë Á‡„Û·‡ ̇ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓıӉ͇ ÏÂÊ‰Û 13 Ë 16-„Ӊ˯̇ ‚˙Á‡ÒÚ. ÑàÄÉçéëíàñàêÄçÖ à èêéëãÖÑüÇÄçÖ çÄ èêéÉêÖëàÇçàíÖ åìëäìãçà Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê Ñˇ„ÌÓÁ‡Ú‡ Ò ÔÓÒÚ‡‚fl ‚˙Á ÓÒÌÓ‚‡ ̇ ı‡‡ÍÚÂ̇ڇ ÍÎËÌË͇ Ë Ô‡‡ÍÎËÌ˘ÌË ËÁÒΉ‚‡ÌËfl. ● ÅËÓıËÏ˘ÌËÚ ËÁÒΉ‚‡ÌËfl Ò‡ Ò ‰‡ÌÌË Á‡ Ôӂ˯ÂÌË ÒÚÓÈÌÓÒÚË Ì‡ äîä, ÄëÄí, ÄãÄí, Ë ãÑï. èË Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ äîä Ò Ôӂ˯‡‚‡ ̇ÈχÎÍÓ 10 Ô˙ÚË Ì‡‰ ÌÓχڇ (̇È-˜ÂÒÚÓ ÏÂÊ‰Û 25 Ë 200 Ô˙ÚË), ‡ ÔË èåÑ ÚËÔ ÅÂÍÂ- ̇È-χÎÍÓ 5 Ô˙ÚË. èӂ˯ÂÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ äîä, ÍÓËÚÓ Ò‡ ÒΉÒÚ‚Ë ̇ ÌÂÍÓÁ‡ ̇ ÏÛÒÍÛÎÌËÚ ‚·Í̇, Ò˙ÓÚ‚ÂÚÒÚ‚‡Ú ̇ ‚˙Á‡ÒÚÚ‡ Ë Ò˙ÒÚÓflÌËÂÚÓ Ì‡ Ô‡ˆËÂÌÚ‡ (5). ● ÖåÉ (ÂÎÂÍÚÓÏËÓ„‡ÙËfl) ÛÒÚ‡ÌÓ‚fl‚‡ ı‡‡ÍÚÂ̇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÏËÓ„ÂÌ̇ Û‚‰‡ ‡ ‚ Í‡ÈÌËÚ ÒÚ‡‰ËË Ì‡ èåÑ Ó·˘ÓÚÓ ˜ËÒÎÓ Ì‡ ‡ÍˆËÓÌÌËÚ ÔÓÚÂ̈ˇÎË Ò ‰ۈË‡ Ò ÓÍÓÎÓ 35%. ● óÂÁ ÖäÉ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡Á΢ÌË ÙÓÏË Ì‡ ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËfl (Ô˙ÎÂÌ ‰ÂÒÂÌ ·Â‰ÂÌ ·ÎÓÍ, ÒËÌÛÒÓ‚‡ Ú‡ıË͇‰Ëfl, Ô‰Ò˙‰ÌË Ë Í‡ÏÂÌË ÂÍÒÚ‡ÒËÒÚÓÎË). ÖıÓ͇‰ËÓ„‡ÙËflÚ‡ Ò ÔË·„‡ Á‡ ÔÓÒΉfl‚‡Ì ̇ Ò˙‰Â˜ÌËfl ÒÚ‡ÚÛÒ. ÇËÁÛ‡ÎËÁË‡Ú ÏËÓ͇‰Ì‡ ÙË·ÓÁ‡, ıËÔÓÍËÌÂÁËfl ̇ Ò˙ˆÂÚÓ, ‰Ë·ڇÚ˂̇ ͇‰ËÓÏËÓÔ‡ÚËfl, ̇χÎÂ̇ Ù‡ÍˆËfl ̇ ËÁÚ·ÒÍ‚‡ÌÂ. ÑÓ ‰ÂÒÂÚ „Ӊ˯̇ ‚˙Á‡ÒÚ Ò ÔÂÔÓ˙˜‚‡ ÖäÉ Ë ÂıÓ͇‰ËÓ„‡ÙËfl ‰‡ Ò ÔÓ‚Âʉ‡Ú ‚‰Ì˙Ê Ì‡ ‰‚ „Ó‰ËÌË, ‡ ÒΉ ÚÓ‚‡ ‚‰Ì˙Ê „Ӊ˯ÌÓ. ● îÛÌ͈ËÓ̇ÎÌÓ ËÁÒΉ‚‡Ì ̇ ‰Ë¯‡ÌÂÚÓ. èË Ô‡ˆËÂÌÚËÚ Ò ËÁÒΉ‚‡Ú îÓÒË‡Ì ÇËÚ‡ÎÂÌ ä‡Ô‡ˆËÚÂÚ (îÇä) ÔÓ‡‰Ë ÂÒÚËÍÚË‚ÂÌ ÚËÔ Ì‡Û¯ÂÌË ̇ ‰Ë¯‡ÌÂÚÓ Ò˙Ò ÒÌËÊÂÌË ÒÚÓÈÌÓÒÚË Ì‡ îÖé1 Ë îÇä Ë ÌÓχÎÂÌ ÍÓÂÙˈËÂÌÚ Ì‡ íËÙÌÓ îÇä/îÖé1 (67). éÔ‰ÂÎflÚ Ò ÍËÒÎÓӉ̇ڇ Ò‡ÚÛ‡ˆËfl Ë Ô‡ˆË‡ÎÌËÚ ̇Îfl„‡ÌËfl ̇ ÍËÒÎÓÓ‰‡ Ë ‚˙„ÎÂÓ‰ÌËfl ‰ËÓÍÒˉ ‚ Í˙‚Ú‡, ˜ËËÚÓ ÔÓÏÂÌË Ò‡ ‚ ÚflÒ̇ ÍÓ·ˆËfl Ò ÔÓÏÂÌËÚ ̇ ÙÛÌ͈ËÓ̇ÎÌËÚ ÔÓ͇Á‡ÚÂÎË. ● åÛÒÍÛÎ̇ ·ËÓÔÒËfl. ïËÒÚÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ‚ ‡ÌÌËÚ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‡ÁÍË‚‡ ‰Â„ÂÌÂ‡ˆËfl Ë Ù‡„ÓˆËÚÓÁ‡ ̇ ‰ËÌ˘ÌË ËÎË „ÛÔË ÏÛÒÍÛÎÌË ‚·Í̇, ÌÂÍÓÚ˘ÌË Ë „ÂÌÂ‡ÚÓÌË ÔÓÏÂÌË. Ç Í˙ÒÌËÚ ÒÚ‡‰ËË Ì‡ ÏÛÒÍÛÎÌËÚ ‰ËÒÚÓÙËË Ò ÓÚÍË‚‡Ú: Á‡„Û·‡ ̇ ÏÛÒÍÛÎÌË ‚·Í̇, ÏËÓˆËÚË Ò ÔÓχÎÍË ËÎË ÔÓ-„ÓÎÂÏË ‡ÁÏÂË ÓÚ ÌÓχÎÌËÚÂ Ë ·ÂÁ‡Á·ÓÌÓ ‡ÁÔÓÎÓÊÂÌËÂ, ‡Á‡ÒÚ‚‡Ì ẨÓÏËÁˇÎÌÓ Ì‡ χÒÚ̇ Ë Ò˙‰ËÌËÚÂÎ̇ Ú˙͇Ì. àÏÛÌÓıËÒÚÓıËÏ˘ÌÓÚÓ ËÁÒΉ‚‡ÌÂ Ë Western blot ÔÓ͇Á‚‡Ú ÎËÔÒ‡ ̇ ‰ËÒÚÓÙËÌ ÔË èåÑ ÚËÔ Ñ˛¯ÂÌ, ‰ۈË‡Ì ËÎË ‡·ÌÓÏÂÌ- ÔË ÚËÔ ÅÂÍÂ. ● ç‚ÓÔÒËıÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl ‚Íβ˜‚‡Ú Ó·˘Ó

159

ËÁÒΉ‚‡Ì ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË, ÚÂÒÚÓ‚ÂÚ Á‡ Ô‡ÏÂÚÓ‚Ë ÙÛÌ͈ËË, ÂÍÁÂÍÛÚË‚ÌË ÙÛÌ͈ËË, Ôӂ‰Â̘ÂÒÍËÚ ̇Û¯ÂÌËfl (54). ● é·‡ÁÌË ËÁÒΉ‚‡ÌËfl ̇ ñçë (äÄí Ë åêí), ͇ÍÚÓ Ë ÙÛÌ͈ËÓ̇ÎÌË ËÁÒΉ‚‡ÌËfl ̇ ÏÓÁ˙˜ÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï (SPECT Ë PET). èË ÌflÍÓË Ô‡ˆËÂÌÚË ÛÒÚ‡ÌÓ‚fl‚‡Ú ̇χÎÂÌ Ó·ÂÏ Ì‡ ÏÓÁ˙˜ÌÓÚÓ ‚¢ÂÒÚ‚Ó Ë ‡Á¯ËÂÌË ‚ÂÌÚËÍÛÎË (17; 68) èË êÖí Ò ‚ËÁÛ‡ÎËÁË‡Ú ̇χÎÂÌ „βÍÓÁÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï ‚ ‰ÂÒÌËÚ gyrus postcentralis Ë gyrus temporalis medius, uncus, ΂Ëfl gyrus hyppocampalis Ë Ï‡ÎÍËfl ÏÓÁ˙Í (17; 51), ÍÓÂÚÓ ‚ÂÓflÚÌÓ ÓÚ‡Áfl‚‡ ÒÏÛÚÂ̇ Ì‚Ó̇Î̇ ÏË„‡ˆËfl Ë ÍÎÂÚ˙˜Ì‡ ‰ËÙÂÂ̈ˇˆËfl, ÍÓËÚÓ Ò „ÛÎË‡Ú ÓÚ ‰ËÒÚÓÙËÌ. ● ÉÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì ̇ ·ÓÎÌË ˜ÂÁ ‰ËÂÍÚÂÌ Ë Ë̉ËÂÍÚÂÌ Ñçä-‡Ì‡ÎËÁ ÄçÄãàá çÄ ÑÖãÖñàà à ÑìèãàäÄñàà åÛÎÚËÔÎÂÍÒ̇ڇ PCR, Southern blot Ë FISH Ò ËÁÔÓÎÁ‚‡Ú Á‡ ÓÚÍË‚‡Ì ̇ ‰ÂΈËË ‚ ‰ËÒÚÓÙËÌÓ‚Ëfl „ÂÌ. èË·ÎËÁËÚÂÎÌÓ 98% ÓÚ ‰ÂΈËËÚ Ò ÓÚÍË‚‡Ú Ò ÚÂÁË ÏÂÚÓ‰ËÍË. Southern blot Ë ÍÓ΢ÂÒÚ‚Â̇ PCR Ò ÔË·„‡Ú Á‡ ÓÚÍË‚‡Ì ̇ ‰ÛÔÎË͇ˆËË. MLPA (Multiple ligation probe amplification) Ò ËÁÔÓÎÁ‚‡ Á‡ ‡Ì‡ÎËÁ ̇ ‰ÂΈËË Ë ‰ÛÔÎË͇ˆËË ÔË ·ÓÎÌË Ë ÊÂÌË-ÌÓÒËÚÂÎÍË (70). - ë͇ÌË‡ÌÂÚÓ Ì‡ ÏÛÚ‡ˆËËÚÂ Ë ÒÂÍ‚ÂÌË‡ÌÂÚÓ ÓÚÍË‚‡Ú χÎÍË ‰ÂΈËË, ËÌÒÂˆËË, ÚÓ˜ÍÓ‚Ë Ë ÒÔ·ÈÒËÌ„ ÏÛÚ‡ˆËË (11, 29, 70). àÁÔÓÎÁ‚‡Ú Ò ‡Ì‡ÎËÁ ̇ ÔÓÎËÏÓÙËÁÏË ‚ ÍÓÌ��ÓχˆËfl ̇ ‰ÌÓ‚ÂËÊÌËÚ (SSCP) Ë ‰‚ÓÈÌÓ‚ÂËÊÌËÚ (DSCP) Ñçä Ù‡„ÏÂÌÚË, ıÂÚÂÓ‰ÛÔÎÂÍÒÂÌ ‡Ì‡ÎËÁ, ÒÂÍ‚ÂÌË‡ÌÂ, ÔÂʉ‚ÂÏÂÌÌÓ ÔÂÍ‡Úfl‚‡Ì ̇ ·ÂÎÚ˙˜Ì‡Ú‡ Ú‡ÌÒ·ˆËfl (Protein truncation test). çÓ‚Ë ÏÂÚÓ‰Ë Í‡ÚÓ SCAIP (single-condition amplification internal primer sequencing) Ë DGGE ‰Â̇ÚÛË‡˘ „ÂÎ ÂÎÂÍÚÓÙÓÂÁ‡- ·‡ÁË‡ÌÓ Ò͇ÌË‡Ì ̇ „Â̇ ËÏ‡Ú Á‡ ˆÂÎ ‰‡ ÓÚÍËflÚ ÓÒڇ̇ÎËÚ 30-35% ÓÚ ÏÛÚ‡ˆËËÚ ÔË èåÑ ÚËÔ Ñ˛¯ÂÌ. àÁÒΉ‚‡ÌÂÚÓ Ì‡ ·ËÓÔÒ˘ÂÌ Ï‡ÚÂˇΠÓÚ ÏÛÒÍÛÎ ˜ÂÁ ÔÓÚÂËÌÂÌ Ë êçä-·‡ÁË‡Ì ‡Ì‡ÎËÁ ‰ÓÔ˙΂‡ ‰ËÂÍÚÌÓÚÓ Ñçä-ÒÂÍ‚ÂÌË‡Ì (27). ● ÉÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì Á‡ ÌÓÒËÚÂÎÒÚ‚Ó àÁÒΉ‚‡Ú Ò ÊÂÌË, ‚ ÒÂÏÂÈÒÚ‚‡Ú‡ ̇ ÍÓËÚÓ Ëχ ·ÓÎÂÌ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl. äÓ„‡ÚÓ ÏÛÚ‡ˆËflÚ‡ ̇ Ô‡ˆËÂÌÚ‡ Ò ÑåÑ Â ËÁ‚ÂÒÚ̇, ËÁÒΉ‚‡ÌÂÚÓ Á‡ ÌÓÒËÚÂÎÒÚ‚Ó Ì‡ ÏÛÚ‡ˆËfl ÓÚ ÚËÔ‡ ‰ÂΈËfl ËÎË ‰ÛÔÎË͇ˆËfl ÏÓÊ ‰‡ ·˙‰Â ËÁ‚˙¯ÂÌÓ ˜ÂÁ ÍÓ΢ÂÒÚ‚ÂÌ ‡Ì‡ÎËÁ ̇ „ÂÌÌËfl ‰ÓÁ‡Ê. íÓÁË ÏÂÚÓ‰ ÏÓÊ ‰‡  ÚÛ‰ÂÌ Á‡ ÓÒ˙˘ÂÒÚ‚fl‚‡ÌÂ Ë ËÌÚÂÔÂÚ‡ˆËfl. àÁÔÓÎÁ‚‡Ú Ò Ò˙˘Ó ڇ͇ PCR (Á‡ ‰ÂΈËË Ë ‰ÛÔÎË͇ˆËË), FISH (Á‡ ‰ÂΈËË) Ë ÒÂÍ‚ÂÌË‡Ì (Á‡ ÚÓ˜ÍÓ‚Ë ÏÛÚ‡ˆËË). äÓ„‡ÚÓ ÏÛÚ‡ˆËflÚ‡ ̇ Ô‡ˆËÂÌÚ‡ Ò ÑåÑ Ì  ËÁ‚ÂÒÚ̇ ÏÓÊ ‰‡ Ò ËÁÔÓÎÁ‚‡ Linkage analysis, ÍÓ„‡ÚÓ ‚ ÒÂÏÂÈÒÚ‚ÓÚÓ Ì‡ ËÁÒΉ‚‡ÌËÚ ÊÂÌË Ëχ Ôӂ˜ ÓÚ Â‰ËÌ Á‡ÒÂ„Ì‡Ú Ò˙Ò ÒË„Û̇ ‰Ë‡„ÌÓÁ‡ èåÑ ÚËÔ Ñ˛¯ÂÌ/ ÅÂÍÂ ËÎË Ñ˛¯ÂÌ-‡ÒÓˆËË‡Ì‡ ͇‰ËÓÏËÓÔ‡ÚËfl. ● à‰ÂÌÚËÙˈË‡Ì ̇ de novo ÏÛÚ‡ˆËË. Ç ÒÂÏÂÈÒÚ‚‡ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl ÔË Ò‡ÏÓ Â‰ËÌ ˜ÎÂÌ Ì‡ ÒÂÏÂÈÒÚ‚ÓÚÓ, ÔÓËÁıÓ‰˙Ú Ì‡ de novo ÏÛÚ‡ˆËflÚ‡ ÏÓÊ ‰‡ ·˙‰Â ˉÂÌÚËÙˈË‡Ì ˜ÂÁ ‰ÂΈËÓÌÂÌ/ ‰ÛÔÎË͇ˆËÓÌÂÌ ‡Ì‡ÎËÁ ËÎË ÒÂÍ‚ÂÌË‡Ì ˜ÂÁ linkage analysis. ï‡ÔÎÓÚËÔ˙Ú, ‡ÒÓˆËË‡Ì Ò ÏÛÚË‡ÎËfl ‡ÎÂÎ ÔË Á‡Ò„̇ÚËfl Ë̉˂ˉ ÏÓÊ ‰‡ ·˙‰Â ÔÓÒΉÂÌ ÔË Ï‡È͇ڇ Ë ‡ÍÓ Â ÌÂÓ·ıÓ‰ËÏÓ ÔË ÌÂÈÌËÚ Ó‰ËÚÂÎË (34). ● èÂ̇ڇÎ̇ڇ Ë ÔÂÍÓ̈ÂÔˆËÓÌ̇ڇ ‰Ë‡„ÌÓÒÚË͇ ‚ Á‡Ò„̇ÚËÚ ÒÂÏÂÈÒÚ‚‡ Ò‡ ÓÒÌÓ‚ÂÌ ÂÎÂ-


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

ÑÂÍÂÏ‚Ë 2009

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SUMMARY DYSTROPHINOPATHIES- MODERN TREATMENT OF DUCHENNE AND BECKER MUSCULAR DYSTROPHIES Teodora Chamova1, Ivailo Tournev1,2 1 Neurology clinic, University hospital “Alexandrovska”, Medical Faculty 2New Bulgarian University, Chair of cognitive science and psychology Duchenne and Becker muscular dystrophies are genetic disorders, characterized by progressive muscle wasting, loss of walking ability leading to complete wheelchair dependence, cognitive disorders, as well as cardiomyopathy and respiratory insufficiency, which are the main causes of death. The therapy of these diseases relies on multidisciplinary management and aims to slow the progression of the disorder and to improve the life quality of these patients. The key areas of the symptomatic treatment are physiotherapy, respiratory management, management of cardiomyopathy and orthopedic interventions. The glucocorticosteroids are the gold standard of treatment for muscle weakness in ambulant children with DMD, although the specific cellular events responsible for their beneficial effects are not known. The eagerly awaited pathogenic approaches based on gene therapy are at a stage of in vitro clinical trials and clinical trials on animal models. These advanced technologies encompass influencing the gene expression by gene replacement therapy with stem cells and delivery of dystrophin minigenes; DNA/RNA manipulation; pharmacological reagents (drugs to turn on the urotrophin gene expression, drugs to cause read-through of premature stop codon, viral delivery of growth factors, protease inhibitors, antiinflamatory reagents). The gene modification approaches such as DNA/RNA manipulation with antisense oligonucleotides resulting in exon skipping and short forms of dystrophin, which attempts to turn a Duchenne mutation into a less severe Becker form are the most promising techniques. KEY WORDS: Dystrophinopathies, Duchenne muscular dystrophy, Becker muscular dystrophy, physiotherapy, respiratory management, drug therapy of cardiomyopathy, orthopedic interventions, corticosteroids, gene replacement therapy with stem cells, DNA/RNA manipulation; pharmacologic agents. êÖáûåÖ èÓ„ÂÒË‚ÌËÚ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË ÚËÔ Ñ˛¯ÂÌ Ë ÅÂÍÂ Ò‡ „ÂÌÂÚ˘ÌË Á‡·ÓÎfl‚‡ÌËfl, ˜ËflÚÓ ÍÎËÌ˘̇ ͇ÚË̇ ‚Íβ˜‚‡ ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ ‰Ó ÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl, ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl, ͇ÍÚÓ Ë Í‡‰ËÓÏËÓÔ‡ÚËfl Ë ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÍÓËÚÓ Ò‡ ̇ȘÂÒÚËÚ ÌÂÔÓÒ‰ÒÚ‚ÂÌË Ô˘ËÌË Á‡ ÎÂÚ‡ÎÂÌ ËÁıÓ‰. ìÚ‚˙‰Â̇ڇ ̇ ÚÓÁË ÂÚ‡Ô ÚÂ‡ÔËfl ̇ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚ  ÍÓÏÔÎÂÍÒ̇ Ë Ëχ Á‡ ˆÂÎ ‰‡ Á‡·‡‚Ë ÔÓ„ÂÒËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÔÓ‰Ó·Ë Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ ·ÓÎÌËÚÂ. éÒÌÓ‚ÌË ÂÎÂÏÂÌÚË Ì‡ ÒËÏÔÚÓχÚ˘ÌÓÚÓ Î˜ÂÌËÂ

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ÑÂÍÂÏ‚Ë 2009

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

● ã˜ÂÌË ̇ ͇‰ËÓÏËÓÔ‡ÚËflÚ‡ Ç Ì‡ÒÚÓfl˘Ëfl ÏÓÏÂÌÚ ÓÒÌÓ‚ÌÓ ÔËÎÓÊÂÌË ̇ÏË‡ ÚÂ‡ÔËflÚ‡ Ò ÍÓÚËÍÓÒÚÂÓˉË, ÄëÖ-ËÌıË·ËÚÓË, ‚·ÎÓÍÂË, ÓÚÓÔ‰˘̇ڇ ıËÛ„Ëfl Ë ÙËÁËÓÚÂ‡ÔËflÚ‡, ˜ÂÁ ÍÓËÚÓ Ò ˆÂÎË ÔÓ‰Ó·fl‚‡Ì ̇ ͇˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ.

1. äéêíàäéëíÖêéàÑà (äë). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â Prednisolone Ë Deflazacort Ò‡ Á·ÚÂÌ Òڇ̉‡Ú ‚ ΘÂÌËÂÚÓ Ì‡ ÏÛÒÍÛÎ̇ڇ Ò··ÓÒÚ ÔË ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚ (17,24). Öʉ̂ÌÓÚÓ ËÏ ÔËÎÓÊÂÌË ËÎË ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‡ÎÚÂÌË‡˘Ë ÒıÂÏË ‚ÂÏÂÌÌÓ Á‡·‡‚fl ÔÓ„ÂÒËflÚ‡ ̇ ÏÛÒÍÛÎ̇ڇ ‰ËÒÚÓÙËfl, ÒÚ‡·ËÎËÁË‡ ÏÛÒÍÛÎ̇ڇ ÒË· Ë ÙÛÌ͈Ëfl, ̇χÎfl‚‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÛÒÎÓÊÌÂÌËflÚ‡ ͇ÚÓ ÒÍÓÎËÓÁ‡, ÔÓ‰Ó·fl‚‡ ‰Ëı‡ÚÂÎ̇ڇ ÙÛÌ͈Ëfl, ËÁÏÂÂ̇ ˜ÂÁ ÙÓÒË‡ÌËfl ‚ËÚ‡ÎÂÌ Í‡Ô‡ˆËÚÂÚ (îÇä) Ë Ì‡Ï‡Îfl‚‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ Ò˙‰Â˜ÌËÚ ÛÒÎÓÊÌÂÌËfl. í˙È Í‡ÚÓ ÔÓ‰˙ÎÊËÚÂÎ̇ڇ ËÏ ÛÔÓÚ·‡ ÏÓÊ ‰‡ ‰Ó‚‰ ‰Ó ÒÂËÓÁÌË ÌÂÊ·ÌË ÎÂ͇ÒÚ‚ÂÌË ‡͈ËË (çãê) ͇ÚÓ: ‡ÚÂˇÎ̇ ıËÔÂÚÓÌËfl, Á‡ÒÚÓÈ̇ Ò˙‰Â˜Ì‡ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, flÁ‚Â̇ ·ÓÎÂÒÚ, ˜ÂÌÓ‰ӷ̇ ÒÚ‡ÚÓÁ‡, Ô‡ÌÍ‡ÚËÚ, flÚÓ„ÂÌÂÌ ıËÔÂÍÓÚˈËÁ˙Ï Ò ˆÂÌÚËÔÂÚ‡ÎÌÓ Á‡ÚÎ˙ÒÚfl‚‡ÌÂ, facies lunata, ‡ÍÌÂ, ıËÁÛÚËÁ˙Ï, ÒÚÂÓˉÂÌ ‰Ë‡·ÂÚ, ‰ËÒÎËÔˉÂÏËfl, ıËÔÓ͇ÎËÂÏËfl, ÏÂÚ‡·ÓÎËÚ̇ ‡Î͇ÎÓÁ‡, ÓÒÚÂÓÔÓÓÁ‡ Ò ÍÓÏÔÂÒËÓÌÌË Ù‡ÍÚÛË (8), ÍÓÌ‚ÛÎÒËË, ·ÂÌË„ÌÂ̇ ËÌÚ‡Í‡ÌˇÎ̇ ıËÔÂÚÂÌÁËfl, ÂÏÓˆËÓ̇Î̇ ··ËÎÌÓÒÚ Ë ÔÒËıÓÁË, ͇ڇ‡ÍÚ‡ Ë ËÏÛÌÓÒÛÔÂÒËfl Ò ˆˉ˂Ë‡˘Ë ËÌÙÂ͈ËË, ÔËÎÓÊÂÌËÂÚÓ ËÏ ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl, ÒΉ‚‡ ‰‡ ·˙‰Â ‚ÌËχÚÂÎÌÓ ÔˆÂÌÂÌÓ. åÂı‡ÌËÁÏËÚ ̇ ‰ÂÈÒÚ‚Ë ̇ äë ̇ ÍÎÂÚ˙˜ÌÓ ÌË‚Ó, ÍÓËÚÓ ‚Ó‰flÚ ‰Ó Á‡·‡‚flÌ ̇ ÔÓ„ÂÒËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÓÒÚ‡‚‡Ú ÌÂflÒÌË. ë˙˘ÂÒÚ‚Û‚‡Ú ‡Á΢ÌË ÚÂÓËË, ÔÓÛ˜ÂÌË ÔË ÂÍÒÔÂËÏÂÌÚ‡ÎÌË ÏÓ‰ÂÎË Ì‡ Ï˯ÍË (14): ● èÓÏfl̇ ‚ ÍÓ΢ÂÒÚ‚ÓÚÓ Ì‡ Ëêçä (ËÌÙÓχˆËÓÌ̇ êçä) Á‡ ÒÚÛÍÚÛÌË, Ò˄̇ÎÌË Ë „ÂÌË, Û˜‡ÒÚ‚‡˘Ë ‚ ËÏÛÌÌËfl ÓÚ„Ó‚Ó ● ç‡Ï‡Îfl‚‡Ì ·Ófl ̇ ˆËÚÓÚÓÍÒ˘ÌËÚ í-ÍÎÂÚÍË ● é„‡Ì˘‡‚‡Ì ̇ ͇ΈË‚Ëfl ËÌÙÎÛÍÒ Ë ‚˙ÚÂÍÎÂÚ˙˜Ì‡Ú‡ ͇ΈË‚‡ ÍÓ̈ÂÌÚ‡ˆËfl ● èӂ˯‡‚‡Ì ̇ ÂÍÒÔÂÒËflÚ‡ ̇ ·ÏËÌËÌ Ë ÒÚËÏÛÎË‡Ì ̇ ÏÛÒÍÛÎÌÓÚÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ● éÚÒÓ˜‚‡Ì ‚˙‚ ‚ÂÏÂÚÓ Ì‡ ‡ÔÓÔÚÓÁ‡Ú‡ ̇ ÏÛÒÍÛÎÌËÚ ‚·Í̇ Ë ÍÎÂÚ˙˜Ì‡Ú‡ ËÌÙËÎÚ‡ˆËfl ● èӂ˯‡‚‡Ì ̇ ÂÍÒÔÂÒËflÚ‡ ̇ ‰ËÒÚÓÙËÌ ● èÓ‚ÎËfl‚‡Ì ÌÂ‚ÌÓÏÛÒÍÛÎÌÓÚÓ Ô‰‡‚‡Ì ● è‰ԇÁ‚‡Ì ̇ ÏÛÒÍÛÎÌËÚ ‚·Í̇ ÓÚ ÏÂı‡Ì˘̇ Û‚‰‡ ● ç‡Ï‡Îfl‚‡Ì ÌÂÍÓÁ‡Ú‡ ̇ ÏËÓˆËÚËÚ ● èӂ˯‡‚‡Ì ̇ ÌË‚‡Ú‡ ̇ ÏÛÒÍÛÎÌËfl Ú‡ÛËÌ Ë Í‡ÚËÌ ë˙˘ÂÒÚ‚Û‚‡Ú ‡Á΢ÌË ÒıÂÏË Ì‡ ÔËÎÓÊÂÌË ̇ äë, ÔË ÍÓËÚÓ Ú ÏÓ„‡Ú ‰‡ Ò ÔË·„‡Ú ‚ÒÂÍË ‰ÂÌ ËÎË ‡ÎÚÂÌË‡˘Ó. Prednisolone Ò ‰ÓÁË‡ ÔÓ 0,75 Ï„/Í„/‰Ì‚ÌÓ, Deflazacort ÔÓ 0,9 Ï„/Í„/‰Ì‚ÌÓ (25). äÎËÌ˘ÌËÚ ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ÚÂÁË Ì‡˜ËÌË Ì‡ ÔËÎÓÊÂÌË ËÏ‡Ú ‰Ó·˙ ÂÙÂÍÚ ‚˙ıÛ ÏÛÒÍÛÎ̇ڇ ÒË· Ë ‰Ëı‡ÚÂÎ̇ڇ ÙÛÌ͈Ëfl ‚ Í‡ÚÍÓÒÓ˜ÂÌ ÔÎ‡Ì (‰˙΄ÓÒÓ˜ÌËÚ ÂÙÂÍÚË ÒΉ‚‡ ‰‡ Ò ÓÚ˜ËÚ‡Ú ‚ ·˙‰Â˘Â). ÑÛ„Ë ÒıÂÏË, ÓÔËÒ‡ÌË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ò‡: Prednisolone 0,3-1,5 Ï„/Í„/‰Ì‚ÌÓ. èË ÔÓ-ÌËÒÍËÚ ‰ÓÁËÓ‚ÍË 0,3-0,5 Ï„/Í„ Ëχ ÔÓ-χÎ˙Í ËÒÍ ÓÚ çãê, ÌÓ Ë ÔÓ-Ò·· ÚÂ‡Ô‚Ú˘ÂÌ ÂÙÂÍÚ. ÄÎÚÂÌË‡˘Ë ÒıÂÏË: Prednisolone 0,75 Ï„/Í„/‰Ì‚ÌÓ Ò ‰ۂ‡Ì ̇ ÔÂËÓ‰Ë ÓÚ 10 ‰Â̇ ÔËÂÏ Ë 10 ‰Â̇ ÔӘ˂͇. Prednisolone 0,65 Ï„/Í„/‰Ì‚ÌÓ 20 ‰Â̇ ÔËÂÏ 10 ‰Â̇ ÔӘ˂͇. èÂÔÓ˙˜‚‡ Ò ÚÂ‡ÔËflÚ‡ ‰‡ Ò Á‡ÔÓ˜ÌÂ, ‰Ó͇ÚÓ

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ÏÓϘÂÚÓ Ì  Á‡„Û·ËÎÓ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ÒË ‰‡ ıÓ‰Ë (̇ȘÂÒÚÓ ÒΉ 4-„Ӊ˯̇ ‚˙Á‡ÒÚ). Prednisolone Ë Deflazacort ÔÓ͇Á‚‡Ú ÒıӉ̇ ÂÙÂÍÚË‚ÌÓÒÚ, ÌÓ Ò ‡Á΢‡‚‡Ú ÔÓ ˜ÂÒÚÓÚ‡Ú‡ ̇ çêã, ÍÓËÚÓ Ô˘ËÌfl‚‡Ú. Deflazacort ÔÓ-fl‰ÍÓ ‚Ó‰Ë ‰Ó Ôӂ˯‡‚‡Ì ̇ ÚÂÎÂÒ̇ڇ χ҇, ÌÓ ˜ÂÒÚÓ Ô˘ËÌfl‚‡ ‡ÒËÏÔÚÓÏ̇ ͇ڇ‡ÍÚ‡ (9,14). èÓfl‚‡Ú‡ ̇ ÒÂËÓÁÌË çãê, ͇ÚÓ „ÓÂËÁ·ÓÂÌËÚ  Ë̉Ë͇ˆËfl Á‡ ÔÂÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÚÂ‡ÔËflÚ‡ Ò ÍÓÚËÍÓÒÚÂÓˉË. Ç ÒÎÛ˜‡È, ˜Â Ú‡ÍË‚‡ Ì ҇ ̇ÎˈÂ, ÚÂ‡ÔËflÚ‡ ÔÓ‰˙Îʇ‚‡ ‰Ó͇ÚÓ Ô‡ˆËÂÌÚËÚ Á‡„Û·flÚ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ Ò Ôˉ‚ËÊ‚‡Ú Ò‡ÏÓÒÚÓflÚÂÎÌÓ. ÉÓÎÂÏËflÚ ·ÓÈ çãê, ‰˙Îʇ˘Ë Ò ̇ ÔÓ‰˙ÎÊËÚÂÎ̇ ÚÂ‡ÔËfl Ò äë, ̇·„‡ Ô‡ˆËÂÌÚËÚ ‰‡ ·˙‰‡Ú ‰ӂÌÓ ÔÓÒΉfl‚‡ÌË, ͇ÚÓ Ò ÒΉflÚ: ‚ËÒÓ˜Ë̇, ÚÂÎÂÒ̇ χ҇, ÄÚÂˇÎÌÓ Ì‡Îfl„‡ÌÂ, „βÍÓÁ‡Ú‡ ‚ ÛË̇ڇ, Ôӂ‰ÂÌËÂÚÓ Á‡ Ò˙ÓÚ‚ÂÚÌË ÓÚÍÎÓÌÂÌËfl, Ó˜ÂÌ ÒÚ‡ÚÛÒ, ı‡ÌËÚÂÎÂÌ ÂÊËÏ, ‰ÓÒÚ‡Ú˙˜ÂÌ ÔËÂÏ Ì‡ ͇ΈËÈ Ë Vitamin D, DEXA (Dual Energy X-Ray Absorbtiometry). åÌÓ„Ó ˜ÂÒÚÓ Á‡„Û·‡Ú‡ ̇ ÒÔÓÒÓ·ÌÓÒÚ‡ Á‡ Ò‡ÏÓÒÚÓflÚÂÎÌÓ ıÓ‰ÂÌ ҇ fl‚fl‚‡ ‰ËÌ ÓÚ Ù‡ÍÚÓËÚÂ, Ó·ÛÒ·‚fl˘Ë ÌËÒ͇ڇ ÍÓÒÚ̇ ÔÎ˙ÚÌÓÒÚ Ë ÔÓÒΉ‚‡˘Ë Ù‡ÍÚÛË, ̇È-˜ÂÒÚÓ Ô¯ÎÂÌÌË. äÓÚËÍÓÒÚÂÓˉËÚ Ò˙˘Ó ËÏ‡Ú Ì··„ÓÔËflÚÂÌ ÂÙÂÍÚ ‚˙ıÛ ÍÓÒÚ̇ڇ ÒËÒÚÂχ, ͇ÚÓ ÔÓÛ˜‚‡ÌËfl ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â ‰Û͈ËflÚ‡ ̇ ÍÓÒÚ̇ڇ ÔÎ˙ÚÌÓÒÚ, Ó·ÛÒÎÓ‚Â̇ ÓÚ Ú‡ÁË ÚÂ‡ÔËfl,  ̇È-ÒËÎÌÓ ËÁ‡ÁÂ̇ ‚ ‰ËÒÚ‡ÎÌËÚ ˜‡ÒÚË Ì‡ ÏÂÚ‡ÙËÁËÚ ̇ ÍÓÒÚËÚÂ. ᇠÔÓÙË·ÍÚË͇ ̇ ÍÓÚËÍÓÒÚÂÓˉ-Ë̉ۈË‡Ì‡Ú‡ ÓÒÚÂÓÔÓÓÁ‡ Ë ÍÓÏÔÂÒËÓÌÌË Ù‡ÍÚÛË ‚ Ò˙Ó·‡ÊÂÌË ‚ÎËÁ‡Ú ÒÎ˙̘‚‡ ÂÍÒÔÓÁˈËfl, ‰ËÂÚ‡, ·Ó„‡Ú‡ ̇ ͇ΈËÈ, ÚÂ‡ÔËflÚ‡ Ò Vitamin D (‰Ó 400Ö/‰ÂÌ) + ä‡ÎˆËÈ (1000Ï„/‰ÂÌ), ËÎË ·ËÙÓÒÙÓ̇ÚË. àÏÛÌÓÒÛÔÂÒË‚ÌËflÚ ÂÙÂÍÚ Ì‡ ÍÓÚËÍÓÒÚÂÓˉËÚ ̇·„‡ ÔÂ‰Ë Ì‡˜‡ÎÓÚÓ Ì‡ ÚÂ‡ÔËflÚ‡ ‰‡ Ò ËÁÍβ˜Ë ‡ÍÚ˂̇ ÚÛ·ÂÍÛÎÓÁ̇ ËÌÙÂ͈Ëfl, ‡ ‚˙‚ ÇÂÎËÍÓ·ËÚ‡ÌËfl Ò ÔË·„‡ Ë ‚‡ÍÒË̇ˆËfl ÒÂ˘Û ‚‡ˈ·. èÓ ‚ÂÏ ̇ ÍÓÚËÍÓÒÚÂÓˉ̇ڇ ÚÂ‡ÔËfl Ò ÔÓÒΉfl‚‡ Ó·ÂÍÚË‚ÌÓ ÏÛÒÍÛÎ̇ڇ ÒË·, ‰Ëı‡ÚÂÎ̇ڇ, Ò˙‰Â˜ÌÓÒ˙‰Ó‚ËÚ ÙÛÌ͈ËË Ë ÔÒËı˘ÂÒÍÓÚÓ Ò˙ÒÚÓflÌËÂ, ÔÓfl‚‡Ú‡ ̇ çêã. èË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ ÅÂÍÂ ÂÙÂÍÚ˙Ú Ì‡ ÚÂ‡ÔËfl Ò ÍÓÚËÍÓÒÚÂÓË‰Ë Ì  ÔÓÛ˜ÂÌ, ÔÓ‡‰Ë ÔÓ-·‡‚̇ڇ ÔÓ„ÂÒËfl Ë ÔÓ-Í˙Ò̇ڇ ËÌ‚‡ÎˉËÁ‡ˆËfl. 2. îàáàéíÖêÄèàüíÄ C˙˘Ó  ‚‡ÊÂÌ ÂÎÂÏÂÌÚ ÓÚ ÍÓÏÔÎÂÍÒÌÓÚÓ Î˜ÂÌË ̇ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ. èË·„‡ Ò ‚˙‚ ‚ÒÂÍË Â‰ËÌ ÏÓÏÂÌÚ ÓÚ ÔÓ„ÂÒËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Í‡ÍÚÓ ÔË ÔÓ‰‚ËÊÌË Ú‡Í‡ Ë ÔË Á‡„Û·ËÎË ÒÔÓÒÓ·ÌÓÒÚÚ‡ ÒË ‰‡ ıÓ‰flÚ Ô‡ˆËÂÌÚË. éÒÌÓ‚ËÚÂ È ˆÂÎË Ò‡ ‰‡ Á‡·‡‚Ë Ì‡Ï‡ÎÂÌËÂÚÓ Ì‡ ÏÛÒÍÛÎ̇ڇ ÒË· Ë Ï‡Ò‡, ‰‡ ÔÓÙË·ÍÚË‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÒÚ‡‚ÌËÚ ÍÓÌÚ‡ÍÚÛË Ë ‰‡ ÔÓ‰Ó·Ë Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ ·ÓÎÌËÚÂ. îËÁËÓÚÂ‡ÔËfl ÒΉ‚‡ ‰‡ Ò Á‡ÔӘ̠ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ ÒΉ ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡. è˙‚Ó̇˜‡ÎÂÌ ÔËÓËÚÂÚ Â Ô‚Â̈ËflÚ‡ ̇ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ‡ÒËÏÂÚ˘ÌË ÍÓÌÚ‡ÍÚÛË Ì‡ ‡ıËÎÂÒÓ‚ËÚ ÒÛıÓÊËÎËfl, ÍÓÎÂÌÌËÚÂ Ë Ú‡Áӷ‰ÂÌËÚ ÒÚ‡‚Ë, ͇ÚÓ ÔÓ ÚÓÁË Ì‡˜ËÌ Ò ̇χÎfl‚‡ ËÒ͇ ÓÚ ÔÓÒΉ‚‡˘‡ ÒÍÓÎËÓÁ‡. è‡ÒË‚ÌËÚ ‡ÁÚfl„‡ÌËfl Ë ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ „ÎÂÁÂÌÌË ÓÚÂÁË Ò‡ ÓÒÌÓ‚ÂÌ ÏÓÏÂÌÚ ÓÚ Î˜ÂÌËÂÚÓ ‚ ÂÚ‡Ô, ÍÓ„‡ÚÓ Ô‡ˆËÂÌÚËÚ ҇ ‚Ò Ӣ ÔÓ‰‚ËÊÌË. ÉÎÂÁÂÌÌËÚ ÓÚÂÁË Ò ÔÓÒÚ‡‚flÚ ÌÓ˘ÂÏ, Á‡˘ÓÚÓ Á‡ÚÛ‰Ìfl‚‡Ú ıÓ‰ÂÌÂÚÓ ÔÂÁ ‰ÂÌfl. åÓϘÂÚ‡Ú‡ ÒΉ‚‡ ‰‡ Ò ÒÚËÏÛÎË‡Ú ‰‡ Ô‡ÍÚËÍÛ‚‡Ú ÔÓ‰ıÓ‰fl˘Ë ÙËÁ˘ÂÒÍË ÛÔ‡ÊÌÂÌËfl Ë ıˉÓÚÂ‡ÔËfl. àÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÍÓÎflÌÌÓ-„ÎÂÁÂÌÌË ÓÚÂÁË ÏÓÊ ‰‡ Û‰˙ÎÊË ‚˙‚ ‚ÂÏÂÚÓ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚflÚÂÎÌÓ ıÓ‰ÂÌÂ Ë ÒÚÓÂÌ Ô‡‚. ç fl‰ÍÓ Úflı̇ڇ ÛÔÓÚ·‡ Ò Ô‰¯ÂÒÚ‚‡ ÓÚ ÚẨÓÂÎÓÌ„‡ˆËË Ì‡ ‡ıËÎÂÒÓ‚ËÚ ÒÛıÓÊËÎËfl.


ÑÂÍÂÏ‚Ë 2009

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

3. éêíéèÖÑàóçÄ ïàêìêÉàü éÍÓÎÓ 90% ÓÚ ÏÓϘÂÚ‡Ú‡ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl ‡Á‚Ë‚‡Ú ÍÎËÌ˘ÌÓ Á̇˜Ëχ ÒÍÓÎËÓÁ‡. èÓ‰ıÓ‰fl˘‡Ú‡ ÔÓÙË·ÍÚË͇ ÒÂ˘Û „˙·Ì‡˜ÌËÚ ËÁÍË‚fl‚‡ÌËfl ÔË ÚÂÁË Ô‡ˆËÂÌÚË Úfl·‚‡ ‰‡ Á‡ÔӘ̠ÔÂ‰Ë ‰‡ Ò‡ ËÁ„Û·ËÎË ÒÔÓÒÓ·ÌÓÒÚÚ‡ ÒË ‰‡ Ò ‰‚ËÊ‡Ú Ë ‚Íβ˜‚‡ ÙËÁËÓÚÂ‡Ô‚Ú˘ÌË Ôӈ‰ÛË Ë ÔÓ‰ıÓ‰fl˘‡ ÔÓÁ‡, ËÁ·fl„‚‡˘‡ Ú‡ÁÓ‚‡Ú‡ ‡ÒËÏÂÚËfl. ljÌ˙Ê ÔÓfl‚Ë· ÒÂ, ÒÍÓÎËÓÁ‡Ú‡ ÏÓÊ ‰‡ ·˙‰Â ÍÓË„Ë‡Ì‡ ıËÛ„˘ÌÓ. ë ̇È-‰Ó·˙ ÂÙÂÍÚ Ò‡ ÚÂÁË ËÌÚÂ‚Â̈ËË, ËÁ‚˙¯ÂÌË Ì‡ ÂÚ‡Ô, ÍÓ„‡ÚÓ „˙·Ì‡˜ÌËflÚ ÒÚ˙η  ‚Ò Ӣ ÔÓ‰‚ËÊÂÌ Ë ˙„˙Î˙Ú Ì‡ äÓ·  20-401 (15). Ç ÚÂÁË ÔÓ‡ÌÌË ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‰Ëı‡ÚÂÎ̇ڇ Ë Ò˙‰Â˜Ì‡Ú‡ ÙÛÌ͈ËË Ì‡ ÚÂÁË ·ÓÎÌË Ò‡ ‰ÓÒÚ‡Ú˙˜ÌÓ Ò˙ı‡ÌÂÌË, Á‡ ‰‡ ÏÓÊ Ó„‡ÌËÁÏ˙Ú ËÏ ‰‡ ÔÓÌÂÒ ÓÔÂ‡Ú˂̇ ËÌÚÂ‚Â̈Ëfl. èË Ô‡ˆËÂÌÚË, ÍÓÌÚ‡Ë̉ˈË‡ÌË Á‡ ÓÔÂ‡ˆËË Ò ËÁÔÓÎÁ‚‡Ú Ò˙ÓÚ‚ÂÚÌË ÒÚ‡·ËÎËÁË‡˘Ë ÍÓ·ÌË Ë ÍÓÒÂÚË. íẨÓÂÎÓÌ„‡ˆËËÚ ̇ ÄıËÎÂÒÓ‚ËÚ ÒÛıÓÊËÎËfl Ò˙˘Ó ËÏ‡Ú Á̇˜ÂÌË Á‡ Û‰˙Îʇ‚‡Ì ‚˙‚ ‚ÂÏÂÚÓ Ì‡ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ̇ ÚÂÁË ·ÓÎÌË ‰‡ ıÓ‰flÚ Ò‡ÏÓÒÚÓflÚÂÎÌÓ. 4. åÖÑàäÄåÖçíéáçÄ íÖêÄèàü çÄ äÄêÑàéåàéèÄíàüíÄ ä‡‰ËÓÏËÓÔ‡ÚËflÚ‡  ˜ÂÒÚÓ ÛÒÎÓÊÌËÂÌË ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËË. èÓ-Í˙Ò̇ڇ È ËÁfl‚‡ ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‡Òfl„‡ÌÂÚÓ Ì‡ ÒÍÂÎÂÚÌËÚ ÏÛÒÍÛÎË Ò ӷÛÒ·‚fl ÓÚ Ì‡Ï‡ÎÂ̇ڇ ÙËÁ˘ÂÒ͇ ‡ÍÚË‚ÌÓÒÚ ÔË Ô‡ˆËÂÌÚËÚÂ, ‚ÒΉÒÚ‚Ë Á‡„Û·‡Ú‡ ̇ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓıӉ͇. ë˙‰Â˜ÌÓÚÓ Á‡Òfl„‡Ì  ÌÂÔÓÒ‰ÒÚ‚Â̇ Ô˘Ë̇ Á‡ ÒÏ˙ÚÚ‡ ÔË ÓÍÓÎÓ 10% ÓÚ ÚÂÁË Ô‡ˆËÂÌÚË (12). èÂÔÓ˙˜‚‡ÌËڠωË͇ÏÂÌÚË Á‡ ÚÂ‡ÔËfl ͇‰ËÓÏËÓÔ‡ÚËflÚ‡ ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl Ò‡: ÄëÖ-ËÌıË·ËÚÓË ËÎË ÄÌ„ËÓÚÂÌÁËÌ-ˆÂÔÚÓÌË ·ÎÓÍÂË, ·ËÏÍÓ‚Ë ‰ËÛÂÚˈË, ÒÔËÓÌÓ·ÍÚÓÌ Ë ‚ ‰ÓÔ˙ÎÌÂÌË ÌÂÒÂÎÂÍÚË‚ÌË ‚-·ÎÓÍÂË ‚ ÔÓ-̇Ô‰̇ÎËÚ ÒÚ‡‰ËË Ì‡ Ò˙‰Â˜ÌÓ Á‡Òfl„‡ÌÂ. ífl Ò ÔË·„‡ Á‡ Á‡·‡‚flÌ ̇ ÔÓ„ÂÒËflÚ‡ ̇ Ò˙‰Â˜ÌÓÚÓ Á‡Òfl„‡ÌÂ Ë Á‡ ÔÓ‰Ó·fl‚‡Ì ÔÓ„ÌÓÁ‡Ú‡ ̇ Ô‡ˆËÂÌÚË Ò ·ÂÁÒËÏÔÚÓÏ̇ ΂Ó͇ÏÂ̇ ıËÔÂÚÓÙËfl (12). ÄëÖ-ËÌıË·ËÚÓËÚ ҇ ‰Ó͇Á‡ÎË Ò‚ÓflÚ‡ ÂÙÂÍÚË‚ÌÓÒÚ ‚ Ô‚Â̈ËflÚ‡ ̇ ÂÏÓ‰ÂÎË‡ÌÂÚÓ Ì‡ ÒÚÂ̇ڇ ̇ Îfl‚‡ ͇ÏÂ‡, ‡Á‡ÒÚ‚‡Ì ̇ ÙË·ÓÁ‡ Ë Ó„‡Ì˘‡‚‡ÌÂÚÓ Ì‡ ΂ÓÍÓÏÂ̇ڇ ıËÔÂÚÓÙËfl, ÍÓÂÚÓ Ì‡Ï‡Îfl‚‡ ËÒ͇ ÓÚ Í‡ÏÂÌË ‡ËÚÏËË Ë ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. äÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl ÔÓÚ‚˙ʉ‡‚‡Ú ÂÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ÍÓÏ·ËÌË‡Ì‡ ÚÂ‡ÔËfl ÓÚ ÄëÖ-ËÌıË·ËÚÓËÚÂ Ë ‚-·ÎÓÍÂË Á‡ Û‚Â΢‡‚‡Ì ̇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ÊË‚ÓÚ‡ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Î‚ÓÍÓÏÂ̇ ‰ËÒÙÛÌ͈Ëfl (16). 5. èéÑöêÜÄçÖ çÄ ÑàïÄíÖãçàíÖ îìçäñàà óÂÒÚËÚ ‰Ëı‡ÚÂÎÌË ËÌÙÂ͈ËË Ë ÔÓ„ÂÒË‡˘‡Ú‡ ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ ‚ ÔÓ-̇Ô‰̇ÎËÚ ÒÚ‡‰ËË Ì‡ èåÑ ÚËÔ Ñ˛¯ÂÌ Ì‡Î‡„‡Ú ÔÂËӉ˘ÌÓ ÏÓÌËÚÓË‡Ì ̇ ‰Ëı‡ÚÂÎÌËÚ ÙÛÌ͈ËË ˜ÂÁ ËÁÏÂ‚‡Ì ̇ îÇä Ë Í˙‚ÌËÚ „‡ÁÓ‚Â, ͇ÍÚÓ Ë Ò˙ÓÚ‚ÂÚ̇ ÚÂ‡ÔËfl. é·ÂÁÔÓÍÓfl‚‡˘Ë ÒËÏÔÚÓÏË, ̇·„‡˘Ë ÚÂ‡ÔËfl Ò‡: ·ÂÎÂÁË Ì‡ ÌÓ˘Ì‡ ıËÔÓ‚ÂÌÚË·ˆËfl (̇Û¯ÂÌËfl ‚ Ò˙Ìfl, ‰Ì‚̇ ÛÏÓ‡, ̇Û¯Â̇ ÍÓ̈ÂÌÚ‡ˆËfl), ̇χÎÂÌ ‡ÔÂÚËÚ, Ò‡ÚÛ‡ˆËfl ̇ O2 <92%, îÇä<60% ÓÚ Ó˜‡Í‚‡ÌËfl, ˜ÂÒÚË ‰Ëı‡ÚÂÎÌË ËÌÙÂ͈ËË. Ç ÔÓ-‡ÌÌËÚ ÒÚ‡‰ËË ‰Ó·˙ ËÏ‡Ú ‰Ëı‡ÚÂÎ̇ڇ „ËÏ̇ÒÚË͇ Ë ÔÓÒÚÛ‡ÎÌËflÚ ‰Â̇Ê. óÂÒÚËÚ ‰Ëı‡ÚÂÎÌË ËÌÙÂ͈ËË ËÁËÒÍ‚‡Ú ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÔÓÚË‚Ó„ËÔÌË Ë ÔÌ‚ÏÓÍÓÍÓ‚Ë ‚‡ÍÒËÌË Í‡ÚÓ ÔÓÙË·ÍÚË͇, ͇ÍÚÓ Ë ‡‰ÂÍ‚‡Ú̇ ‡ÌÚË·ËÓÚ˘̇ ÚÂ‡ÔËfl Á‡ ΘÂÌË (4). àÁÍÛÒÚ‚Â̇ڇ ‚ÂÌÚË·ˆËfl Ò ÔÓÁËÚË‚ÌÓ Ì‡Îfl„‡Ì ̇È-˜ÂÒÚÓ Á‡ÔÓ˜‚‡ ‰‡ Ò ÔË·„‡ ÓÍÓÎÓ 17-„Ӊ˯̇ ‚˙Á‡ÒÚ. éÒÌӂ̇ Ë̉Ë͇ˆËfl Á‡ ÔËÎÓÊÂÌËÂÚÓ Ì‡ ÌÂËÌ‚‡Á˂̇ ÌÓ˘Ì‡ ‚ÂÌÚË·ˆËfl  ÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ÍÎËÌ˘ÌÓ ËÁfl‚Â̇ ÌÓ˘Ì‡ ıËÔÓ‚ÂÌÚË·-

164

ˆËfl. ÖÏÔË˘ÌÓ Â ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â ÌÓ˘Ì‡Ú‡ ‚ÂÌÚË·ˆËfl (ÌÂËÌ‚‡Á˂̇ ËÎË ËÌ‚‡Á˂̇ ˜ÂÁ Ú‡ıÂÓÒÚÓÏËfl) ‚Ó‰Ë ‰Ó ‰˙΄ÓÒӘ̇ ÒÚ‡·ËÎËÁ‡ˆËfl ̇ ‰Ëı‡ÚÂÎÌËÚ ÙÛÌ͈ËË, ‰Û͈Ëfl ̇ ‰Ëı‡ÚÂÎÌËÚ ËÌÙÂ͈ËË Ë Û‚Â΢ÂÌË ̇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ÊË‚ÓÚ‡ (18). ë ̇Ô‰‚‡Ì ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔÂËÓ‰ËÚ ̇ ËÁÍÛÒÚ‚Â̇ ‚ÂÌÚË·ˆËfl ‚ ‡ÏÍËÚ ̇ ‰ÂÌÓÌÓ˘ËÂÚÓ Ò ۉ˙Îʇ‚‡Ú. II. ÉÖççÄ íÖêÄèàü 1. íÖêÄèàü, èéÇãàüÇÄôÄ ÉÖççÄíÄ ÖäëèêÖëàü çÄ Ñàëíêéîàç èÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË Ò ‡Á‚Ë‚‡Ú ‡Á΢ÌË ÔÓ‰ıÓ‰Ë Á‡ Í��Â͈Ëfl ̇ „ÂÌÂÚ˘ÌËfl ‰ÂÙÂÍÚ Ë ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ ‰ËÒÚÓÙËÌÓ‚‡Ú‡ ÂÍÒÔÂÒËfl, ÍÓËÚÓ ·Ëı‡ Á‡·‡‚ËÎË ÔÓ„ÂÒËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ·Ëı‡ ÔÓ‰Ó·ËÎË Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ ·ÓÎÌËÚ (29, 30, 21). í ҇ ‚Ò Ӣ ÂÍÒÔÂËÏÂÌÚ‡ÎÌË. íÂÁË ÔÓ‰ıÓ‰Ë Ò ͇Ú„ÓËÁË‡Ú ‚ ÚË ÓÒÌÓ‚ÌË Í·҇: „ÂÌÂÚ˘ÌË, ÍÎÂÚ˙˜ÌÓ-·‡ÁË‡ÌË Ë Ù‡χÍÓÎӄ˘ÌË (퇷Îˈ‡ 1). 퇷Îˈ‡ 1. éÒÌÓ‚ÌË ÚÂ‡Ô‚Ú˘ÌË ÒÚ‡Ú„ËË Á‡ „ÂÌ̇ڇ ÚÂ‡ÔËfl (32) ÉÂÌ-Á‡ÏÂÒ- ëÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË åÛÒÍÛÎÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË, ÚËÚÂÎ̇ ̇˜ÂÌË Ó˘Â Ò‡ÚÂÎËÚÌË ÚÂ‡ÔËfl åÛÒÍÛÎÌË Ë ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË äÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚÓχÎÌË ÍÎÂÚÍË Ç̇ÒflÌ ‚ Ó„‡ÌËÁχ ̇ ĉÂÌÓ‡ÒÓˆËË‡ÌË ‚ËÛÒË ‰ËÒÚÓÙËÌÓ‚Ë ÏËÌË„ÂÌË ˜ÂÁ: ÇËÛÒË ÓÚ Ó‰ Lentivirus Ñçä Ô·ÁÏË‰Ë Ñçä/êçä ÄÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚˉË, χÌËÔÛ- ÍÓËÚÓ ‚Ó‰flÚ ‰Ó ÔÂÒ͇˜‡Ì ÎË‡Ì ̇ ÂÍÁÓÌ Ë ÒËÌÚÂÁ ̇ Í˙ÒË ‰ËÒÚÓÙËÌÓ‚Ë ÙÓÏË. Ñçä ıËÏÂ‡Ô·ÒÚË Á‡ ÍÓÂ͈Ëfl ̇ ÚÓ˜ÍÓ‚Ë ÏÛÚ‡ˆËË Ë Ï‡ÎÍË ‰ÂΈËË ÇËÛÒÌÓ-̇ÒÓ˜ÂÌÓ ÔÂÒ͇˜‡Ì ̇ ÂÍÁÓÌ î‡χÍÓ- å‰Ë͇ÏÂÌÚË, ÒÚËÏÛÎË‡˘Ë Heregulin Ë ‰Û„Ë Ò˙‰ËÌÂÌËfl ‚ Îӄ˘ÌË „ÂÌ̇ڇ ÂÍÒÔÂÒËfl ÂÚ‡Ô Ì‡ ÔÓÛ˜‚‡Ì ‡„ÂÌÚË Ì‡ ÛÓÚÓÙËÌ å‰Ë͇ÏÂÌÚË, ÓÒË„Ûfl‚‡˘Ë ÄÏËÌÓ„ÎËÍÓÁË‰Ë Ú‡ÌÒÍ‡ÔˆËfl Ë ÒΉ ÔÂʉ‚- êíë124 ÂÏÂÌÌÓ ÔÓfl‚ËÎ Ò ÒÚÓÔ ÍÓ‰ÓÌ ÑÛ„Ë Ò˙‰ËÌÂÌËfl ‚ ÂÚ‡Ô Ì‡ ÔÓÛ˜‚‡Ì å‰Ë͇ÏÂÌÚË Ë Ù‡ÍÚÓË, ÍÓËÚÓ ÓÒË„Ûfl‚‡Ú ÏÛÒÍÛÎÌÓÚÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ËÎË Ì‡Ï‡Îfl‚‡Ú ÏÛÒÍÛÎ̇ڇ Û‚‰‡ Ç̇ÒflÌ ˜ÂÁ ‚ËÛÒÌË ‡„ÂÌÚË IGF 1 ̇ ‡ÒÚÂÊÌË Ù‡ÍÚÓË åËÓÒÚ‡ÚËÌ ËÌı˷ˈËfl èÓÚ‡ÁÌË ËÌıË·ËÚÓË, Leupeptin-ËÌıË·ËÚÓ ̇ Á‡·‡‚fl˘Ë ÏÛÒÍÛÎ̇ڇ Û‚‰‡ ͇ÎÔ‡Ë̇ BBIC-ÒÂËÌ ÔÓÚ‡ÁÂÌ ËÌıË·ËÚÓ MG-132-ÔÓÚÂÓÁÓÏÂÌ ËÌıË·ËÚÓ ꇄÂÌÚË, ÔÓ‰ÚËÒ͇˘Ë Cyclosporine A ‚˙ÁÔ‡ÎÂÌËÂÚÓ Enbrel Remicade NFkB nNOS „Û·ˆËfl ̇„Ó Pentoxifillin


ÑÂÍÂÏ‚Ë 2009

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

ÉÖçÖç íêÄçëîÖê îÛÌ͈ËÓ̇ÎÂÌ ‰ËÒÚÓÙËÌÓ‚ „ÂÌ ÏÓÊ ‰‡ ·˙‰Â ‚˙‚‰ÂÌ ‚ ÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË Ì‡ Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËfl, Á‡ ‰‡ Á‡ÏÂÒÚË ‰ÂÙÂÍÚÌÓÚÓ ÍÓÔËÂ. êÂÍÓÏ·Ë̇ÌÚÌËÚ ‡‰ÂÌÓ‡ÒÓˆËË‡ÌË ‚ËÛÒË ËÏ‡Ú ÌflÍÓË Ô‰ËÏÒÚ‚‡ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌ‚Â̈ËÓ̇ÎÌËÚ ‡‰ÂÌÓ‚ËÛÒÌË Ë ÂÚÓ‚ËÛÒÌË ‚ÂÍÚÓË Ë Ò ËÁÔÓÎÁ‚‡Ú ‚Ò ÔÓ-˜ÂÒÚÓ Á‡ Ú‡ÌÒÙÂ ̇ ÏËÌË Ë ÏËÍÓ‰ËÒÚÓÙËÌÓ‚Ë Ë ÛÓÚÓÙËÌÓ‚Ë „ÂÌË ‚ ̇Ô˜ÌÓ̇·‡Á‰ÂÌË Ë Ò˙‰Â˜ÌË ÏËÓˆËÚË Ì‡ Ï˯ÍË- ÏÓ‰ÂÎË. çÂÒÔˆËÙ˘ÌË CMV Ë ÒÔˆËÙ˘ÌË ÏÛÒÍÛÎÌË ÔÓÏÓÚÂË Ò ËÁÔÓÎÁ‚‡Ú Á‡ ÍÓÌÚÓΠ̇ ÂÍÒÔÂÒËflÚ‡ ̇ ÚÂÁË „ÂÌË. ÑÛ„ ÏÂÚÓ‰  ËÌÊÂÍÚË‡ÌÂÚÓ Ì‡ „ÓÎË Ñçä Ô·ÁÏˉË, ÌÓÒÂ˘Ë „Â̇, ‚ ÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË (23). ᇠˆÂÎÚ‡ Ò ËÁÔÓÎÁ‚‡Ú ÂÎÂÍÚÓÚ‡ÌÒÙÂ Ë ıˇÎÛÓÌˉ‡Á‡. ÉÖççÄ åéÑàîàäÄñàü ñÂÎÚ‡ ̇ „ÂÌ̇ڇ ÏÓ‰ËÙË͇ˆËfl  ‰‡ Á‡ÏÂÌË ËÎË ‚˙ÁÒÚ‡ÌÓ‚Ë „ÂÌÌËÚ ÏÛÚ‡ˆËË. Ö‰ËÌ ÓÚ Ì‡È-ӷ¢‡‚‡˘ËÚ ÏÂÚÓ‰Ë Â ÔÂÒ͇˜‡ÌÂÚÓ Ì‡ ÂÍÁÓÌ, ˆÂÎÚ‡, ̇ ÍÓÈÚÓ Â ‰‡ ÏÓ‰ËÙˈË‡ ‰̇ ÔÓ-ÚÂÊ͇ ÏÛÚ‡ˆËfl, ‚Ӊ¢‡ ‰Ó ÍÎËÌË͇ ̇ Ñ˛¯ÂÌ ‚ ÔÓ-ÎÂ͇ Ò ÙÂÌÓÚËÔ Ì‡ èåÑ ÚËÔ ÅÂÍÂ (3). ÖÍËÔË ÓÚ ÏÌÓ„Ó ÒÚ‡ÌË ËÁ‚˙¯‚‡Ú ÍÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl ‚ Ú‡ÁË Ì‡ÒÓ͇, ͇ÚÓ Ò ËÁÔÓÎÁ‚‡Ú ÍÓÏ·Ë̇ˆËË ÓÚ ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚˉË. àÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚˉË, ÍÓËÚÓ ÓÒË„Ûfl‚‡Ú ÔÂÒ͇˜‡Ì ̇ „ËÓÌ ÏÂÊ‰Û 45 Ë 51 ÂÍÁÓÌË (ÏÓÎÂÍÛÎÌË Í˙ÔÍË), ÏÓÊ ‰‡  ÔÓ‰ıÓ‰fl˘Ó ΘÂÌË Á‡ „ÓÎflχ ˜‡ÒÚ (ÓÍÓÎÓ 80%) ÓÚ Ô‡ˆËÂÌÚËÚ (7,19). ÑÛ„ ÚËÔ „ÂÌ̇ ÚÂ‡ÔËfl Ò‡ ıËÏÂ‡Ô·ÒÚËÚÂ, ÔË ÍÓËÚÓ Ò ËÁÔÓÎÁ‚‡Ú Ñçä ÔÓÒΉӂ‡ÚÂÎÌÓÒÚË, Ò‚˙Á‡ÌË Ò êçä, ÍÓËÚÓ Ò ҂˙Á‚‡Ú Ò˙Ò ÒÓ·ÒÚ‚Â̇ڇ Ñçä ̇ Ô‡ˆËÂÌÚ‡ Ë ‡ÍÚË‚Ë‡Ú ÏÂı‡ÌËÁÏËÚ ̇ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡ÌÂ. êÂÁÛÎÚ‡ÚËÚ ÓÚ ÚÓÁË ÏÂÚÓ‰ Ì ҇ ÏÌÓ„Ó ‰Ó·Ë (22). ÄÌÚË·ËÓÚËˆË Í‡ÚÓ ‡ÏËÌÓ„ÎËÍÓÁˉËÚ ÏÓ„‡Ú ‰‡ ÔÓ‚ÎËflflÚ ÔÓˆÂÒËÚ ̇ Ú‡ÌÒ·ˆËfl, ͇ÚÓ Ò ˄ÌÓË‡ ÔÂʉ‚ÂÏÂÌÌÓ ‚˙ÁÌËÍ̇ÎËfl ÒÚÓÔ ÍÓ‰ÓÌ Ë Ò ÒËÌÚÂÁË‡ ÌÓχÎÂÌ ‰ËÒÚÓÙËÌ (6, 26). è‡‚flÚ Ò ÓÔËÚË Ò Gentamicin, Negamicin Ë PTC124 ÔË Ï˯ÍË- ÏÓ‰ÂÎË Ë ÔË ıÓ‡ Ò Ó·Â˘‡‚‡˘Ë ̇ ÚÓÁË ÂÚ‡Ô ÂÁÛÎÚ‡ÚË (5). äãÖíöóçÄ íÖêÄèàü çÓχÎ̇ڇ ÏÛÒÍÛÎ̇ ÙÛÌ͈Ëfl Á‡‚ËÒË ÓÚ ËÁ‰˙ÊÎË‚ÓÒÚÚ‡ Ë „ÂÌÂ‡ˆËflÚ‡ ̇ ÏËÓÙË·ËÚÂ. íÓ‚‡  ‰ËÌ ÒÎÓÊÌÓ „ÛÎË‡Ì ÔÓˆÂÒ, Á‡ÔÓ˜‚‡˘ Ò ‡ÍÚË‚‡ˆËflÚ‡ ̇ ÔÂÍÛÒÓÌË ÍÎÂÚÍË, ÍÓËÚÓ ‚ÔÓÒΉÒÚ‚Ë ÙÓÏË‡Ú ÔÓ„ÂÌËÚÓÌË ÍÎÂÚÍË, ÍÓËÚÓ ÒΉ ÒÎË‚‡ÌÂÚÓ ÒË, Ó·‡ÁÛ‚‡Ú ‰ËÙÂÂ̈Ë‡ÌË ÏËÓÙË·Ë. í‡ÌÒÔ·ÌÚ‡ˆËflÚ‡ ̇ ‡Á΢ÌË ‚ˉӂ ÏÛÒÍÛÎÌË ÔÂÍÛÒÓÌË ÍÎÂÚÍË Ëχ Á‡ ˆÂÎ ‰‡ Û‚ÂÎË˜Ë ÒË·ڇ ̇ ‰ËÒÚÓÙ˘ÌËÚ ÏÛÒÍÛÎË. èÓÒÚ˄̇ÚËÚ ÂÁÛÎÚ‡ÚË Ò‡ ‡ÁÓ˜‡Ó‚‡˘Ë, ÔÓ‡‰Ë ‡Á‚Ë‚‡˘‡Ú‡ Ò ‚˙ÁÔ‡ÎËÚÂÎ̇ ‡͈Ëfl Ë Ò··‡Ú‡ ÏË„‡ˆËÓÌ̇ ÒÔÓÒÓ·ÌÓÒÚ Ì‡ ÏËӷ·ÒÚËÚ (20,28). ë‡ÚÂÎËÚÌËÚ ÍÎÂÚÍË, ‡ÁÔÓÎÓÊÂÌË ÏÂÊ‰Û Ô·ÁÏÂ̇ڇ ÏÂÏ·‡Ì‡ ̇ ÏÛÒÍÛÎÌËÚ ‚·Í̇ Ë ·‡Á‡Î̇ڇ ÏÂÏ·‡Ì‡, Ò ҘËÚ‡Ú Á‡ ÏÛÒÍÛÎÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÍÚË. í ÂÍÒÔÂÒË‡Ú CD34, ‡ ÒΉ ‡ÍÚË‚‡ˆËfl Ë MyoD, Myf-5, M-Cadherin. ÇÒflÍÓ Ô˜ËÒÚÂÌÓ Â‰ËÌ˘ÌÓ ËÌÚ‡ÍÚÌÓ ÏÛÒÍÛÎÌÓ ‚·ÍÌÓ Â ‡ÒÓˆËË‡ÌÓ Ò ÓÍÓÎÓ 7-22 Ò‡ÚÂÎËÚÌË ÍÎÂÚÍË Ë Ú‡ÌÒÔ·ÌÚË‡ÌË ‚ ËÏÛÌÓÒÛÔÂÒË‡ÌË Ï˯ÍË-ÏÓ‰ÂÎË, ‰‡‚‡Ú ̇˜‡ÎÓÚÓ Ì‡ ÓÍÓÎÓ 100 ÌÓ‚Ë ÏÛÒÍÛÎÌË ‚·Í̇. ë‡ÚÂÎËÚÌËÚ ÍÎÂÚÍË Ò‡ ‚ Ò˙ÒÚÓflÌË ‰‡ ÓÒË„ÛflÚ „ÂÌÂ‡ˆËfl ̇ Û‚‰ÂÌË ÏÛÒÍÛÎÌË ‚·Í̇ in vivo. Ç Ò‡‚ÌÂÌËÂ Ò ÚÂÁË ÍÎÂÚÍË, ‰Û„ËÚ ËÁÔÓÎÁ‚‡ÌË ÏÛÒÍÛÎÌË ÒÚ‚ÓÎÓ‚Ë Ë ÔÓ„ÂÌËÚÓÌË ÍÎÂÚÍË ËÏ‡Ú Ï‡Î˙Í ÏËÓ„ÂÌÂÌ ÔÓÚÂ̈ˇΠ(31). è‡‚flÚ Ò ÓÔËÚË Ò ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ıÂÏÓÔÓÂÚ˘ÌË Ë ÒÚ¸ÓχÎÌË ÍÎÂÚÍË, Ëχ˘Ë ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡

165

ÔÂÏË̇‚‡Ú ÓÚ Í˙‚ÓÌÓÒÌËÚ Ò˙‰Ó‚ Í˙Ï ÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË, Ò ‚Ò Ӣ ÌÂÒË„ÛÂÌ ÂÁÛÎÚ‡Ú. éÒÌÓ‚ÂÌ ÔÓ·ÎÂÏ, Ò ÍÓÈÚÓ Ò ҷÎ˙ÒÍ‚‡ ÍÎÂÚ˙˜Ì‡Ú‡ ÚÂ‡ÔËfl  ‡͈ËflÚ‡ ̇ ÓÚı‚˙ÎflÌÂ. àÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÓ·ÒÚ‚ÂÌË ÍÎÂÚÍË Ì‡ Ô‡ˆËÂÌÚ‡  ‰ÌÓ ÓÚ ‚˙ÁÏÓÊÌËÚ ¯ÂÌËfl. ìêéíêéîàç ìÓÚÓÙËÌ˙Ú Â ÔÓÚÂËÌ Ò ·ÎËÁ͇ ‰Ó ‰ËÒÚÓÙË̇ ÒÚÛÍÚÛ‡ Ë Ò ÓÚÍË‚‡ ‚˙‚ ‚ËÒÓÍË ÍÓ̈ÂÌÚ‡ˆËË ‚ ÏÛÒÍÛÎÌËÚ ÍÎÂÚÍË ÔÓ ‚ÂÏ ̇ ÂÏ·ËÓ̇ÎÌÓÚÓ ‡Á‚ËÚËÂ. ëΉ ‡Ê‰‡ÌÂÚÓ Ò ÂÍÒÔÂÒË‡ ÓÒÌÓ‚ÌÓ ‚ Ì‚ÓÏÛÒÍÛÎÌËÚ Ò˙˜ÎÂÌÂÌËfl. èË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ÂÍÒÔÂÒËflÚ‡ ̇ ÛÓÚÓÙË̇  Ôӂ˯Â̇ ͇ÚÓ ÍÓÏÔÂÌÒ‡ÚÓ̇ ‡͈Ëfl ÒÔflÏÓ Ì‡Ï‡ÎÂÌËfl ‰ËÒÚÓÙËÌ. èÓ‰ıÓ‰Ë ‚ ÚÂ‡ÔËflÚ‡ ̇ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚ ҇ ‚˙‚Âʉ‡Ì ̇ ÔÓÏÓÚÓ ËÎË ‚¢ÂÒÚ‚Ó, ÍÓËÚÓ Ôӂ˯‡‚‡Ú ÂÍÒÔÂÒËflÚ‡ ̇ ÛÓÚÓÙËÌÓ‚Ëfl „ÂÌ (10, 27). îÄêåÄäéãéÉàóçà èéÑïéÑà àÁÔÓÎÁ‚‡Ú ҠωË͇ÏÂÌÚË, ÛÚ‚˙‰ÂÌË Á‡ ΘÂÌËÂÚÓ Ì‡ ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl (‚Ê. ÍÓÚËÍÓÒÚÂÓˉ̇ ÚÂ‡ÔËfl). ñÂÎÚ‡ ËÏ Â ÔÓ‰‰˙Ê‡Ú Í‡ÎˆË‚‡Ú‡ ıÓÏÂÓÒÚ‡Á‡, ‰‡ ÔÓ‰Ó·flÚ ÏÛÒÍÛÎ̇ڇ ÒË·, ‰‡ ÒÛÔÂÒË‡Ú ÒÚÓÔ ÍÓ‰ÓÌËÚÂ, ‰‡ ÒÚËÏÛÎË‡Ú ÛÓÚÓÙËÌÓ‚‡Ú‡ ÂÍÒÔÂÒËfl. áÄäãûóÖçàÖ ã˜ÂÌËÂÚÓ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Â Ò ÍÓÚËÍÓÒÚÂÓË‰Ë (Prednisolone Ë Deflazacort)  Á·ÚÂÌ Òڇ̉‡Ú, Ú˙È Í‡ÚÓ Ú ÔÓ Ì ̇Ô˙ÎÌÓ ËÁflÒÌÂÌË ÍÎÂÚ˙˜ÌË ÏÂı‡ÌËÁÏË ‚Ó‰flÚ ‰Ó Á‡·‡‚flÌ ̇ ÔÓ„ÂÒËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. íÓÁË ÚËÔ Î˜ÂÌË Ì  ÔË·„‡ÌÓ Ï‡ÒÓ‚Ó ‚ Å˙΄‡Ëfl, ‡  ÌÂÓ·ıÓ‰ËÏÓ ‰‡ Ò ËÁÔÓÎÁ‚‡ ÔÓ‡‰Ë ‰ÓÒÚ˙ÔÌÓÒÚÚ‡ Ë ÔÂÒÔÂÍÚË‚‡Ú‡ Á‡ Û‰˙Îʇ‚‡Ì ÔÂËÓ‰‡ ̇ ‰ÓÒÚË„‡Ì ‰Ó ÚÂÊ͇ ËÌ‚‡ÎˉËÁ‡ˆËfl. ã˜ÂÌËÂÚÓ Ò ‡‚‡Ì„‡‰ÌË ÚÂıÌÓÎÓ„ËË, ‰ÂÈÒÚ‚‡˘Ë ̇ Ô‡ÚÓ„ÂÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò ÔÓ‚ÎËfl‚‡Ì „ÂÌ̇ڇ ÂÍÒÔÂÒËfl ̇ ‰ËÒÚÓÙËÌ ‚Íβ˜‚‡: „ÂÌ-Á‡ÏÂÒÚËÚÂÎ̇ ÚÂ‡ÔËfl Ò˙Ò ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË (ÏÛÒÍÛÎÌË Ë ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË, ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚÓχÎÌË ÍÎÂÚÍË); Ñçä/êçä χÌËÔÛÎË‡ÌÂ Ë Ù‡χÍÓÎӄ˘ÌË ‡„ÂÌÚË (ωË͇ÏÂÌÚË, ÒÚËÏÛÎË‡˘Ë „ÂÌ̇ڇ ÂÍÒÔÂÒËfl ̇ ÛÓÚÓÙËÌ; ωË͇ÏÂÌÚË, ÓÒË„Ûfl‚‡˘Ë Ú‡ÌÒÍ‡ÔˆËfl Ë ÒΉ ÔÂʉ‚ÂÏÂÌÌÓ ÔÓfl‚ËÎ Ò ÒÚÓÔ ÍÓ‰ÓÌ; ‚̇ÒflÌ ˜ÂÁ ‚ËÛÒÌË ‡„ÂÌÚË Ì‡ ‡ÒÚÂÊÌË Ù‡ÍÚÓË; ÔÓÚ‡ÁÌË ËÌıË·ËÚÓË, Á‡·‡‚fl˘Ë ÏÛÒÍÛÎ̇ڇ Û‚‰‡; ‡„ÂÌÚË, ÔÓ‰ÚËÒ͇˘Ë ‚˙ÁÔ‡ÎÂÌËÂÚÓ). ç‡È-ӷ¢‡‚‡˘Ó Á‡ Ò„‡  Ñçä/êçä χÌËÔÛÎË‡ÌÂÌÓ Ò ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚˉË, ÔË ÍÓÂÚÓ Ò ÒËÌÚÂÁË‡Ú Ò Í˙ÒË ‰ËÒÚÓÙËÌÓ‚Ë ÙÓÏË Ë Ò ÔÂÏË̇‚‡ ÓÚ ÚÂÊ͇ ÏÛÚ‡ˆËfl Ò ÍÎËÌË͇ ̇ Ñ˛¯ÂÌ ‚ ÔÓ-ÎÂ͇ Ò ÙÂÌÓÚËÔ Ì‡ èåÑ ÚËÔ ÅÂÍÂ. èË ÔÓÁËÚË‚ÌË ÂÁÛÎÚ‡ÚË ÓÚ ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ËÁÒΉ‚‡ÌËfl Ë ÍÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl, ÌflÍÓË ÓÚ Úflı ·Ëı‡ ÏÓ„ÎË ‰‡ Ò ËÁÔÓÎÁ‚‡Ú Ë ÔË ·˙΄‡ÒÍË Ô‡ˆËÂÌÚË. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7.

燈ËÓ̇ÎÂÌ ÍÓÌÒÂÌÒÛÒ Á‡ ‰Ë‡„ÌÓÒÚË͇, ΘÂÌËÂ Ë ÔÓÙË·ÍÚË͇ ̇ Ì‚Ó-ÏÛÒÍÛÎÌËÚ Á‡·ÓÎfl‚‡ÌËfl. Å˙΄‡Ò͇ Ì‚ÓÎÓ„Ëfl 2005, ‹1. í˙Ì‚ à. ç‚ÓÎÓ„Ëfl, äÎËÌ˘̇ Ì‚ÓÎÓ„Ëfl, ëÓÙËfl: å‰ˈË̇ Ë ÙËÁÍÛÎÚÛ‡ ÖééÑ, 2007, 462-470. Aartsma-Rus A, Van Ommen G. Antisense-mediated exon skipping: A versatile tool with therapeutic and research applications. RNA 2007, 13:1609–1624. American Thoracic Society Documents: Respiratory Care of the Patient with Duchenne Muscular Dystrophy; ATS Consensus Statement 2004. Arakawa M, Shiozuka M, Nakayama Y, Hara T, Hamada M, Kondo S, et al. Negamycin restores dystrophin expression in skeletal and cardiac muscles of mdx mice. J Biochem 2003; 134: 751-758. Barton-Davis ER, Cordier L, Shoturma DI, Leland SE, Sweeney HL. Aminoglycoside antibiotics restore dystrophin function to sketetal muscles of mdx mice. J Clin Invest 1999; 104: 375-381. Beroud C, Tuffery-Giraud S, Matsuo M, Hamroun D, Humbertclaude V,


ÑÂÍÂÏ‚Ë 2009

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19. 20.

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

Monnier N, Moizard M, Voelckel M, Calemard L, Boisseau P, Blayau M, Philippe C, Cossee M, Multiexon Skipping Leading to an Artificial DMD Protein Lacking Amino Acids from Exons 45 Through 55 Could Rescue Up to 63% of Patients With Duchenne Muscular Dystrophy. Human mutation 2007; 28(2):196-202, Bianchi ML, Mazzanti A, et al. Bone mineral density in Duchenne muscular dystrophy. Osteoporosis Int 2003;14: 761–7. WD, Politano L, et al. Deflazacort in Duchenne muscular dystrophy: a comparison of two different protocols. Neuromuscul Disord 2004;14(89):476–82. Bogdanovich S, Perkins KJ, Krag TO, Khurana TS. Therapeutics for Duchenne muscular dystrophy: current approaches and future directions. J Mol Med 2004; 82: 102-115. Brooke MH, Fenichel GM, Griggs RC. Duchene muscular dystrophy: patterns of clinical progression and effects of supportive therapy. Neurology 1989;39:475–481. Bushby KMD, Muntoni F, Bourke JP. The management of cardiac complications in muscular dystrophy and myotonic dystrophy. Proceedings of 107th ENMC Workshop. Neuromuscul Disord 2003;13:166–172. Bushby K, Bourke J, Bullock R et al. The multidisciplinary management of Duchenne muscular dystrophy .Current Paediatrics (2005) 15, 292–300. Bushby K, Griggs K. 145th ENMC International Workshop: Planning for an International Trial of Steroid Dosage Regimes in DMD (FOR DMD) 22–24th October 2006, Naarden, The Netherlands .Neuromuscular Disorders. 2007;17: 423–428. Cervellati S, Bettini N, Moscato M, Gusella A, Dema E, Maresi R. Surgical treatment of spinal deformities in Duchenne muscular dystrophy: a long term follow-up study. Eur Spine J 2004;13(5):441–448. Duboc, et al. Effect of perindropril on the onset and progression of LV dysfunction in DMD. Journal of the American Cardiology Association 2005;45:855–857. Dubowitz V. Special Centennial Workshop—101st ENMC International Workshop: therapeutic possibilities in Duchenne muscular dystrophy. Neuromuscul Disord 2002;12:421–431. Eagle M, Baudouin SV, Chandler C, Giddings DR, Bullock R, Bushby K. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromusc Disord 2002; 12(10):926–929. Gurvich O, Tuohy T, Howard M et al. DMD Pseudoexon Mutations: Splicing Efficiency, Phenotype, and Potential Therapy. Annals of Neurology. 2008; 63: 81-89. Huard J, Cao B, Qu-Petersen Z. Muscle-derived stem cells: potential for muscle regeneration. Birth Defects Res C Embryo Today 2003; 69: 230-237.

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21. Kapsa R, Kornberg AJ, Byrne E. Novel therapies for Duchenne muscular dystrophy. Lancet Neurol 2003; 2: 299-310. 22. Kmiec EB. Targeted gene repair -in the arena. J Clin Invest 2003; 112: 632636. 23. Liang KW, Nishikawa M, Liu F, Sun B, Ye Q, Huang L. Restoration of dystrophin expression in mdx mice by intravascular injection of naked DNA containing full-length dystrophin cDNA. Gene Ther 2004; 11: 901-908. 24. Manzur AY, Kuntzer T, Pike M, et al; Glucocorticoid corticosteroids for Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2004;(2):CD003725. 25. Moxley, R. T. III, S. Ashwal, S. Pandya et al. Practice Parameter: Corticosteroid treatment of Duchenne dystrophy: Report of the Practice Committee of the Child Neurology Societythe Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2005;64;13-20. 26. Politano L, Nigro G, Nigro V, Piluso G, Papparella S, Paciello O, et al. Gentamicin administration in Duchenne patients with premature stop codon. Preliminary results. Acta Myol 2003; 22:15-21. 27. M, Brownback K, Werle MJ. Okadaic acid augments utrophin in myogenic cells. Neurosci Lett 2004;363: 163-167. 28. JP, Vilquin JT. Transplantation of normal or genetically modified myoblasts for the treatment of hereditary or acquired diseases. J Soc Biol 2001; 195: 29-37. 29. Van Deutekom JC, van Ommen GJ. Advances in Duchenne muscular dystrophy gene therapy. Nat Rev Genet 2003;4:774-783. 30. Voisin V, de la Porte S. Therapeutic strategies for Duchenne and Becker dystrophies. Int Rev Cytol 2004; 240: 1-30. 31. Wagers AJ, Conboy IM. Cellular and molecular signatures of muscle regeneration: current concepts and controversies in adult myogenesis. Cell 2005;122: 659-667. 32. Zhou G, Xie H, Zhang S, Yang Z. Current understanding of dystrophin-related muscular dystrophy and therapeutic challenges ahead. Chinese Medical Journal 2006; 119 (16):1381-1391.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: äÎËÌË͇ ÔÓ ÌÂ‚ÌË ·ÓÎÂÒÚË, ìåÅÄã ◊ÄÎÂÍ҇̉Ó‚Ò͇”, ·ÛÎ. ÉÂÓ„Ë ëÓÙËÈÒÍË ‹1. E-mail: teodoratch@abv.bg


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é·ÁÓ ÄÇíéáéåçé-ÑéåàçÄçíçà îÄåàãçà èÄêñàÄãçà ÖèàãÖèíàóçà ëàçÑêéåà-äãàçàóçÄ, ÖãÖäíêéîàáàéãéÉàóçÄ à ÉÖçÖíàóçÄ ïÄêÄäíÖêàëíàäÄ ë.ÜÂÎflÁÍÓ‚‡1. à.í˙Ì‚1,2 1ìåÅÄã 2çÓ‚

◊ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, åî –ëÓÙËfl ·˙΄‡ÒÍË ÛÌË‚ÂÒËÚÂÚ, ä‡Ú‰‡ ÔÓ äÓ„ÌËÚ˂̇ ̇Û͇ Ë ÔÒËıÓÎÓ„Ëfl

SUMMARY AUTOSOMAL-DOMINANT FAMILIAL PARTIAL EPILEPSIES-CLINICAL, ELECTROPHYSIOLOGICAL AND GENETIC CHARACTERISTICS. S.Zhelyazkova1. I.Tournev1,2 1 University Hospital “Alexandrovska”, Neurological Clinic, Medical faculty-Sofia 2New Bulgarian University, Department of Cognitive Science and Psychology Genetic factors are thought to play a role in the pathogenesis of approximately 40% of the epilepsies. While the significance of these factors for the generalized epilepsies is wellknown, the concept of genetic focal epilepsies is relatively new. In the last decade, the progress, achieved in the field of molecular genetics and phenotype defining contributed to the identification of several new familial partial epilepsies with autosomal-dominant inheritance. They are characterized by incomplete penetrance, variable expressivity in terms of age of onset, type and frequency of seizures, and duration of the disorder. In some of these syndromes, the responsible genes have been identified while in others, new genes are expected to be discovered. The fact that the clinical presentation in all familial partial epilepsy syndromes is similar to that in sporadic patients, necessitates the taking of detailed family history by the physicians for determining the exact diagnosis. The aim of this review is to present the main clinical, electrophysiological and genetic features of some of the newly discovered in the last decade familial partial epilepsy syndromes with autosomal-dominant inheritance. KEY WORDS: genetic, autosomal-dominant inheritance, familial focal epilepsies. êÖáûåÖ ÉÂÌÂÚ˘ÌËÚ هÍÚÓË Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÓÍÓÎÓ 40% ÓÚ ÂÔËÎÂÔÒËËÚÂ. ÑÓ͇ÚÓ ÚflıÌÓÚÓ Á̇˜ÂÌË ÔË „ÂÌÂ‡ÎËÁË‡ÌËÚ ÂÔËÎÂÔÒËË Â ÓÚ‰‡‚̇ ËÁ‚ÂÒÚÌÓ, ÚÓ Ë‰ÂflÚ‡ Á‡ Ò˙˘ÂÒÚ‚Û‚‡Ì ̇ „ÂÌÂÚ˘ÌË ÙÓÏË Ì‡ ÙÓ͇ÎÌË ÂÔËÎÂÔÒËË Â Ò‡‚ÌËÚÂÎÌÓ ÌÓ‚‡. Ç ÔÓÒΉÌËÚ 10 „Ó‰ËÌË ·Î‡„Ó‰‡ÂÌË ̇ ÔÓÒÚËÊÂÌËflÚ‡ ‚ ӷ·ÒÚÚ‡ ̇ ÏÓÎÂÍÛÎfl̇ڇ „ÂÌÂÚË͇ Ë ÔÓ-ÔˆËÁÌÓÚÓ ÙÂÌÓÚËÔÌÓ ‰ÂÙËÌË‡ÌÂ, ·flı‡ ˉÂÌÚËÙˈË‡ÌË ÌflÍÓÎÍÓ ÌÓ‚Ë Ô‡ˆË‡ÎÌË, Ù‡ÏËÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉Óχ Ò ‡‚ÚÓÁÓÏÌÓ - ‰ÓÏË̇ÌÚÓ Û̇ÒΉfl‚‡ÌÂ. í Ò ı‡‡ÍÚÂËÁË‡Ú Ò ÌÂÔ˙Î̇ ÔÂÌÂÚ‡ÌÌÓÒÚ, ‚‡ˇ·ËÎ̇ ÂÍÒÔÂÒË‚ÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ, ÚËÔ Ë ˜ÂÒÚÓÚ‡ ̇ ÂÔËÎÂÔÚ˘ÌËÚ ÔËÒÚ˙ÔË, Ë ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. èË ÌflÍÓË ÓÚ Úflı ‚˜ ҇ ˉÂÌÚËÙˈË‡ÌË ÓÚ„Ó‚ÓÌË Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ „ÂÌË, ‡ ÔË ‰Û„Ë ÚÓ‚‡ ÚÂÔ˙‚‡ Ô‰ÒÚÓË. äÎËÌ˘̇ڇ ͇ÚË̇ ̇ ‚Ò˘ÍË Ù‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË Â ÒıӉ̇ Ò Ú‡ÁË Ì‡ ÒÔÓ‡‰Ë˜ÌËÚ ÙÓÏË, ÂÚÓ Á‡˘Ó Ò‡ÏÓ Ì‡ÒÓ˜ÂÌÓÚÓ Ú˙ÒÂÌ ̇ Ù‡ÏËÎ̇ ‡Ì‡ÏÌÂÁ‡ ÓÚ ÒÚ‡Ì‡ ̇ ÍÎËÌˈËÒÚËÚÂ, ·Ë ÔÓÏӄ̇ÎÓ Á‡ ÔÓÒÚ‡‚flÌ ̇ ÚӘ̇ ‰Ë‡„ÌÓÁ‡.

ñÂÎÚ‡ ̇ ÚÓÁË Ó·ÁÓ  ‰‡ Ò Ô‰ÒÚ‡‚flÚ ÓÒÌÓ‚ÌËÚ ÍÎËÌ˘ÌË, ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌË Ë „ÂÌÂÚ˘ÌË ı‡‡ÍÚÂËÒÚËÍË Ì‡ ÌflÍÓË ÓÚ ÌÓ‚ÓÓÚÍËÚËÚ ‚ ÔÓÒΉÌÓÚÓ ‰ÂÒÂÚËÎÂÚË هÏËÎÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË Ò ‡‚ÚÓÁÓÏÌÓ – ‰ÓÏË̇ÌÚÓ Û̇ÒΉfl‚‡ÌÂ. äãûóéÇà Ñìåà: „ÂÌÂÚË͇, ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚÓ Û̇ÒΉfl‚‡ÌÂ, Ù‡ÏËÎÌË ÙÓ͇ÎÌË ÂÔËÎÂÔÒËË. ÖÔËÎÂÔÒËflÚ‡  ‰ÌÓ ÓÚ Ì‡È-˜ÂÒÚËÚ ıÓÌ˘ÌË Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl, ÍÓÂÚÓ Ò ı‡‡ÍÚÂËÁË‡ Ò Ë̉˂ˉۇÎ̇ Ô‰‡ÁÔÓÎÓÊÂÌÓÒÚ Í˙Ï ÔÂËӉ˘ÌÓ ÔÓ‚Ú‡fl˘Ë Ò ÔÓfl‚Ë, ̇˜ÂÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË. ÖÔËÎÂÔÒËflÚ‡  ‡ÁÔÓÒÚ‡ÌÂ̇ ‚˙‚ ‚Ò˘ÍË ‡ÒË Ë Á‡Òfl„‡ ÔÓ˜ÚË Â‰Ì‡Í‚Ó ‰‚‡Ú‡ ÔÓ·. ᇷÓ΂‡ÂÏÓÒÚÚ‡ ÓÚ ÂÔËÎÂÔÒËfl  2070/100 000, ͇ÚÓ Â Ì‡È-‚ËÒÓ͇ ‚ ‰ÂÚÒ͇ڇ (120-240/100 000) Ë ‚ ÒÚ‡˜ÂÒ͇ڇ ‚˙Á‡ÒÚ (140-150/100 000 ÒΉ 70 „.) (1). ífl Ô‰ÒÚ‡‚Îfl‚‡ ÏÛÎÚËÙ‡ÍÚÓÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÍÓÂÚÓ Ò ‡Á‚Ë‚‡ ÔË ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ë ̇ ÌflÍÓÎÍÓ Ù‡ÍÚÓ‡, Ëχ˘Ë ‡Á΢̇ ÚÂÊÂÒÚ ÔË ÓÚ‰ÂÎÌËÚ ÙÓÏË Ë ‚Ó‰Â˘Ë ‰Ó ÙÓÏË‡Ì ̇ ÂÔËÎÂÔÚÓ„ÂÌÌÓ Ó„ÌˢÂ, ͇ÚÓ ËÁ‡Á ̇ ÏËÍÓ- ËÎË Ï‡ÍÓÒÍÓÔÒÍË ÏÓÁ˙˜ÌË ÎÂÁËÁË. ᇠۘ‡ÒÚËÂÚÓ Ì‡ „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË ‚ ÂÚËÓÎÓ„ËflÚ‡ ̇ ÂÔËÎÂÔÒËËÚ Ò  Á̇ÂÎÓ Ó˘Â ÓÚ ‰‚ÌÓÒÚÚ‡ Ë ÚÓ‚‡  ‰ÓÔËÌÂÒÎÓ Á‡ ÒÚ˄χÚËÁ‡ˆËflÚ‡ ̇ ıÓ‡Ú‡ Ò ÂÔËÎÂÔÒËfl (33). ç‡È–„ÓÎÂÏËflÚ Ì‡Ô‰˙Í Á‡ ‡ÁÍË‚‡Ì „ÂÌÂÚË͇ڇ ̇ ÂÔËÎÂÔÒËËÚ Á‡ÔÓ˜‚‡ ÓÚ 1930„. Ò ‚˙‚Âʉ‡ÌÂÚÓ ÓÚ ìËÎflÏ ãÂÌÓÍÒ Ì‡ ̇ۘÌËflÚ ÏÂÚÓ‰ Á‡ ËÁÛ˜‡‚‡ÌÂÚÓ È. éÚ ÚÓ„‡‚‡ ‰Ó ‰ÌÂÒ ‚ ÂÁÛÎÚ‡Ú Ì‡ ÔÓÛ˜‚‡ÌËfl ‚˙ıÛ Á‡Ò„̇ÚË Ù‡ÏËÎËË Ë ÒÔÓÌÚ‡ÌÌË ËÎË Ë̉ۈË‡ÌË ÊË‚ÓÚËÌÒÍË ÏÓ‰ÂÎË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ì‡‰ 70 „ÂÌË Û˜‡ÒÚ‚‡˘Ë ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÂÔËÎÂÔÒËflÚ‡. ç‡È-„ÓÎflÏ ·ÓÈ ÓÚ Ë‰ÂÌÚËÙˈË‡ÌËÚ „ÂÌË ÍÓ‰Ë‡Ú Ò۷‰ËÌËˆË Ì‡ ‚ÓÎÚ‡Ê Á‡‚ËÒËÏËÚÂ Ë ÎË„‡Ì‰ Á‡‚ËÒËÏËÚ ÈÓÌÌË Í‡Ì‡ÎË. çflÍÓÎÍÓ ÏÛÚ‡ˆËË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‚˙‚ ‚ÒÂÍË ÓÚ ÚÂÁË „ÂÌË, ÍÓÂÚÓ ÔÓ͇Á‚‡ ‡ÎÂÎ̇ ıÂÚÂÓ„ÂÌÌÓÒÚ. èÓ Ì‡ÒÚÓfl˘ÂÏ Ò ÔËÂχ, ˜Â „ÂÌÂÚ˘ÌËÚ هÍÚÓË Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ÓÍÓÎÓ 40% ÓÚ ÂÔËÎÂÔÒËËÚ (59). ç‡È-˜ÂÒÚËÚ هÏËÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË ÔÓ͇Á‚‡Ú ÍÓÏÔÎÂÍÒÌÓ Û̇ÒΉfl‚‡ÌÂ. å‡Î˙Í Â ·Ófl ̇ ◊ ˜ËÒÚËÚ” ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË, ÍÓËÚÓ Ò Û̇ÒΉfl‚‡Ú ÏÓÌÓ„ÂÌÌÓ Ë ÏÓ„‡Ú ‰‡ ·˙‰‡Ú „ÂÌÂ‡ÎËÁË‡ÌË ( ̇Ô. ·ÂÌË„ÌÂÌËÚ هÏËÎÌË ÌÂÓ̇ڇÎÌË ÍÓÌ‚ÛÎÒËË) ËÎË Ô‡ˆË‡ÎÌË (̇Ô. ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚ̇ ÌÓ˘Ì‡ ÂÔËÎÂÔÒËfl ̇ ÙÓÌÚ‡ÎÌËfl ‰flÎ). í ҇ ‡‚ÌËÚÂÎÌÓ ‰ÍË Ë Ò ÓÚÍË‚‡Ú ‚ ÓÍÓÎÓ 1% ÓÚ ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl (59). äÓ̈ÂÔˆËflÚ‡ Á‡ ̇΢ˠ̇ Ù‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÒË̉ÓÏË Â Ò‡‚ÌËÚÂÎÌÓ ÌÓ‚‡. Ñ˙Î„Ó ‚ÂÏ Ò  ҘËÚ‡ÎÓ, ˜Â Ô‡ˆË‡ÎÌËÚ ÂÔËÎÂÔÒËË Ò ‰˙ÎÊ‡Ú Ì‡ Ù‡ÍÚÓË Ì‡ ÓÍÓÎ̇ڇ Ò‰‡ ͇ÚÓ Ó‰Ó‚Ë Ë ÔÓÒÚ‡ÌÚ‡ÎÌË Ú‡‚ÏË, ËÌÙÂ͈ËË Ë ÏÓÁ˙˜ÌË ÎÂÁËË Ì‡Ô. ÚÛÏÓË Ë Ò˙‰Ó‚Ë ËÌÒÛÎÚË. Ç˙ÔÂÍË, ˜Â Ò‡ ÓÚÍË‚‡ÌË Ù‡ÏËÎËË Ò Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl, Ò  ҘËÚ‡ÎÓ, ˜Â Ú ҇ Ò ÍÓÏÔÎÂÍÒÌÓ Û̇ÒΉfl‚‡ÌÂ. ᇠËÁÒΉ‚‡Ì ̇ Úflı̇ڇ „ÂÌÂÚË͇ Ò‡ ËÁÔÓÎÁ‚‡ÌË ‡Á΢ÌË ÔÓ‰ıÓ‰Ë: ÂÔˉÂÏËÓÎӄ˘ÌËfl ÔÓ‰ıÓ‰ „Ë ‡Á„ÎÂʉ‡ ͇ÚÓ ıÓÏÓ„ÂÌ̇ „ÛÔ‡; ÎÓ·‡ÌËflÚ ÔÓ‰ıÓ‰ Í·ÒËÙˈË‡ Ô‡ˆËÂÌÚËÚÂ


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ÒÔÓ‰ Ô‰ÔÓ·„‡Âχ ËÎË ‰Ó͇Á‡Ì‡ ‡Ì‡ÚÓÏ˘̇ ÎÓ͇ÎËÁ‡ˆËfl; EEG – ÔÓ‰ıÓ‰‡ ӷ‰ËÌfl‚‡ ‰‡ÌÌËÚ ÓÚ Ôӂ‰ÂÌËÚ ÖÖÉ ËÁÒΉ‚‡ÌËfl; ÔË ÔÓ‰ıÓ‰‡ Ò ËÁÔÓÎÁ‚‡Ì ̇ „ÓÎÂÏË Ù‡ÏËÎËË Ò ÔÓ‚Âʉ‡Ú ‡Ì‡ÎËÁË Á‡ Ò͇˜ÂÌÓÒÚ Ë Ú˙ÒÂÌ ̇ „ÂÌËÚ ÓÚ„Ó‚ÓÌË Á‡ ‡Á‚ËÚË ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ(33). Ç ÔÓÒΉÌÓÚÓ ‰ÂÒÂÚËÎÂÚË ҇ ˉÂÌÚËÙˈË‡ÌË ÌflÍÓÎÍÓ Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉Óχ Û̇ÒΉfl‚‡˘Ë Ò ‡‚ÚÓÁÓÏÌÓ - ‰ÓÏË̇ÌÚÌÓ Ë Ú ҇ ‚Íβ˜ÂÌË ‚ Ô‰ÎÓÊÂÌËÂÚÓ Á‡ ÌÓ‚‡ Í·ÒËÙË͇ˆËfl ̇ ÂÔËÎÂÔÚ˘ÌËÚ ÒË̉ÓÏË Ì‡ ILAE (International League Against Epilepsy) (28). íÓ‚‡ Ò‡: ÄÑ ÌÓ˘Ì‡ ÂÔËÎÂÔÒËfl ̇ ÙÓÌÚ‡ÎÌËfl ÎÓ·, ÄÑ Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË, Ù‡ÏËÎ̇ڇ ÏÂÁˇÎ̇ ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl, Ù‡ÏËÎ̇ڇ Ó͈ËÔËÚÓÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl, Ù‡ÏËÎ̇ڇ Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò ÔÓÏÂÌÎË‚Ë Ó„Ìˢ‡ Ë Ô‡ˆË‡Î̇ڇ ÂÔËÎÂÔÒËfl Ò ÔÂˈÂÌÚ‡ÎÌË ÒÔ‡ÈÍÓ‚Â (퇷Î. 1).

ÔÓ-fl‰ÍÓ ‰Û„Ë ÒÂÚË‚ÌË (ÒÎÛıÓ‚Ë, ‚ÂÚË„ËÌÓÁÌË, ÁËÚÂÎÌË, Ó·ÓÌflÚÂÎÌË, ‚ÍÛÒÓ‚Ë) Ë ÔÒËı˘ÌË (ÒÚ‡ı, deja vu) ÙÂÌÓÏÂÌË (60, 61). èË ‰‚ ÚÂÚË ÓÚ Ô‡ˆËÂÌÚËÚ Ò ‡Á‚Ë‚‡Ú ‚ÚÓ˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌ˘ÌÓ – ÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË. Ç˙ÁÏÓÊÌË Ò‡ Ë ‰Ì‚ÌË ÔËÒÚ˙ÔË ÓÒÓ·ÂÌÓ ÔË Ô‡ˆËÂÌÚË Ò Ì‰ӷ˙ ÍÓÌÚÓΠ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÖÖÉ ËÁ‚˙Ì ÔËÒÚ˙Ô Ó·ËÍÌÓ‚ÂÌÓ Â ÌÓχÎ̇, ÌÓ ‚ 1265% ÓÚ ÒÎÛ˜‡ËÚ Ò „ËÒÚË‡Ú ÙÓÌÚ‡ÎÌË ÂÔËÎÂÔÚËÙÓÏÂÌË ‡·ÌÓÏÌÓÒÚË, „·‚ÌÓ ÔÓ ‚ÂÏ ̇ Ò˙Ì. àÍÚ‡Î̇ڇ ÖÖÉ Ò˙˘Ó ˜ÂÒÚÓ Â ÌÓχÎ̇, ÌÓ ‚ 44%-88% Ò „ËÒÚË‡Ú ÙÓÌÚ‡ÎÌË ÓÒÚËÂÚ‡, ÓÒÚËÂ-·‡‚̇ ‚˙Î̇ ËÎË ËÚÏ˘ÌË ÚÂÚ‡ ‚˙ÎÌË Â‰ÌÓÒÚ‡ÌÌÓ ËÎË ‰‚ÛÒÚ‡ÌÌÓ (53). éÚ Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ÏÂÚÓ‰Ë åêí Ó·ËÍÌÓ‚ÂÌÓ Ì ÔÓ͇Á‚‡ ÓÚÍÎÓÌÂÌËfl ÓÚ ÌÓχڇ, ÌÓ ÔË PET/ SPECT ËÁÒΉ‚‡Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÛÌË·ÚÂ‡ÎÌË ‡·ÌÓÏÌÓÒÚË ‚˙‚ ÙÓÌÚ‡ÎÌËfl ‰flÎ (30). 燷≇‚‡Ú Ò ËÁ‡ÁÂÌË ËÌÚ‡Ù‡ÏËÎÌË ‡Á΢Ëfl ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ, ÒÂÏËÓÎÓ„Ëfl ̇ ÔËÒÚ˙ÄÇíéáéåçé ÑéåàçÄçíçÄ çéôçÄ ÖèàãÖèëàü ÔËÚÂ Ë ÚÂÊÂÒÚ Ì‡ ÔÓÚ˘‡ÌÂ. èÓ‡‰Ë ÎÂ͇ڇ ÒÚÂÔÂÌ Ì‡ çÄ îêéçíÄãçàü Ñüã (ADNFLE) ADNFLE  Ô˙‚‡Ú‡ ÓÔË҇̇ Ù‡ÏËÎ̇ Ô‡ˆË‡Î̇ ÂÔË- Á‡Òfl„‡Ì ̇ Ó‰ÒÚ‚ÂÌˈËÚÂ, Ù‡ÏËÎÌËfl ı‡‡ÍÚÂ ̇ Ò˙ÒÎÂÔÒËfl, ÔË ÍÓflÚÓ Â ÛÒÚ‡ÌÓ‚ÂÌ Â‰ËÌÒÚ‚ÂÌ „ÂÌ Í‡ÚÓ ÔË- ÚÓflÌËÂÚÓ ˜ÂÒÚÓ Ì Ò ‡ÁÔÓÁ̇‚‡. èËÒÚ˙ÔËÚ ÏÓ„‡Ú ˜Ë̇ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (60, 61). ë‡ÏÓ ‰ÂÒÂÚËÎÂÚË ÔÓ-Í˙Ò- ‰‡ Ò ҷ˙Í‡Ú Ò Ô‡‡ÒÓÏÌËË, ÌÓ ÔÓÒΉÌËÚ ҇ ÔÓ-‰ÍË, ÌÓ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ (21) Ò‡ Ò˙Ó·˘ÂÌË Ì‡‰ 109 Ó‰ÓÒÎÓ‚Ëfl Ì Ò „ÛÔË‡Ú Ë ËÏ‡Ú ÒÍÎÓÌÌÓÒÚ ‰‡ ÓÚÁ‚Û˜‡‚‡Ú ‰Ó 18 Ò ADNFLE Ë ‚˙Á ÓÒÌÓ‚‡ ̇ Úflı  ÛÒÚ‡ÌÓ‚ÂÌÓ ‡‚ÚÓÁÓÏÌÓ „Ӊ˯̇ ‚˙Á‡ÒÚ. äÎËÌ˘̇ڇ ͇ÚË̇ ̇ ADNFLE  Ì– ‰ÓÏË̇ÌÚÌÓ Û̇ÒΉfl‚‡ÌÂ Ò 84% ÔÂÌÂÚ‡ÌÌÓÒÚ Ë Â‰- ‡Á΢Ëχ ÓÚ ÒÔÓ‡‰Ë˜ÌËÚ ÙÓÏË Ì‡ ÌÓ˘Ì‡Ú‡ ÂÔËÎÂÔÒËfl ̇ ˜ÂÎÌËfl ‰flÎ, Í˙‰ÂÚÓ ËÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ ˜ÂÒÚÓ Â Ì‡Í‚‡ ˜ÂÒÚÓÚ‡ ̇ Á‡Òfl„‡Ì ̇ ‰‚‡Ú‡ ÔÓ·. 燘‡ÎÓÚÓ Â ‚ 1-2 ‰ÂÒÂÚËÎÂÚË (Ò‰ÌÓ 8-12 „Ó‰. ‚˙Á- ÌÓχÎ̇, ‡ ËÍÚ‡Î̇ڇ ÖÖÉ Â Ï‡ÒÍË‡Ì‡ ÓÚ ÏÌÓÊÂÒÚ‚Ó ‡ÒÚ), ͇ÚÓ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÔËÒÚ˙ÔËÚ ̇χÎfl‚‡ Ò ‚˙Á- ‡ÚÂÙ‡ÍÚË. èÓ„ÌÓÁ‡Ú‡  ‰Ó·‡. Ç˙ÁÏÓÊÌË Ò‡ ÒÔÓÌÚ‡ÌÌË ÂÏËÒËË. ‡ÒÚÚ‡. äÎËÌ˘ÌËÚ ÔÓfl‚Ë Ò‡ Í‡ÚÍË (20-50 ÒÂÍ.) Ë „Ûé·ËÍÌÓ‚ÂÌÓ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÔËÒÚ˙ÔËÚ ̇χÎfl‚‡ Í˙Ï ÔË‡ÌË ÏÓÚÓÌË ÔËÒÚ˙ÔË ÓÚ ıËÔÂÍËÌÂÚ˘ÂÌ ËÎË ÚÓÔÂÚÓÚÓ – ¯ÂÒÚÓÚÓ ‰ÂÒÂÚËÎÂÚË ÓÚ ÊË‚ÓÚ‡. ë‡ÏÓ ÔË Ì˘ÂÌ ÚËÔ, ‚˙ÁÌËÍ‚‡˘Ë ÔÓ ‚ÂÏ ̇ çêÖå Ò˙Ìfl (ÓÒÓ·ÂÌÓ 2 ÒÚ‡‰ËÈ). éÔËÒ‡ÌË Ò‡ ‡Á΢ÌË ËÚÏ˘ÌË Ë ÔÓ‚Ú‡fl˘Ë ÓÚ‰ÂÎÌË Ô‡ˆËÂÌÚË Ò ÓÔËÒ‚‡Ú ˜ÂÒÚË, ÂÁËÒÚÂÌÚÌË Ì‡ ΘÂÌË ÔËÒÚ˙ÔË. 퇷Îˈ‡ 1. î‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË ë‰̇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ ÖÔËÎÂÔÚ˘ÂÌ ÙÂÌÓÚËÔ ãÓ͇ÎËÁ‡ˆËfl ̇ „Â̇ Ä‚ÚÓÁÓÏÌÓ –‰ÓÏË̇ÌÚ̇ ÌÓ˘Ì‡ ÂÔËÎÂÔÒËfl 1-2 ‰ÂÒÂÚËÎÂÚË çÓ˘ÌË ÏÓÚÓÌË ÔËÒÚ˙ÔË CHRNA 4 ̇ ÙÓÌÚ‡ÎÌËfl ‰flÎ ÓÚ ıËÔÂÍËÌÂÚ˘ÂÌ ÚËÔ, CHRNB2 ˜ÂÒÚÓ ÔË Á‡Ô‡ÁÂÌÓ Ò˙Á̇ÌË CHRNA2 Ä‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚ̇ Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl 2 - 4 ÚÓ ‰ÂÒÂÚËÎÂÚË ÄÛ‡ Ò˙Ò ÒÎÛıÓ‚Ë, ÁËÚÂÎÌË, LGI1 Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË ‰ËÒÙ‡Á˘ÌË ÒËÏÔÚÓÏË î‡ÏËÎÌË ÏÂÁˇÎÌË ÂÔËÎÂÔÒËË Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ 2 – 4ÚÓ ‰ÂÒÂÚËÎÂÚË Deja vu, ‡‚ÚÓÌÓÏ̇ Ë ‡ÙÂÍÚ˂̇ 4q* - ·ÂÁ HS, ·ÂÁ Ù·ËÎÌË ÔËÒÚ˙ÔË ‡Û‡ Ë ‰Ó·Ó͇˜ÂÒÚ‚ÂÌ ıÓ‰ - Ò HS, +/- Ù·ËÎÌË ÔËÒÚ˙ÔË 1 – 3 ÚÓ ‰ÂÒÂÚËÎÂÚË Deja vu, ‡‚ÚÓÌÓÏ̇ Ë ‡ÙÂÍÚ˂̇ ÌÂËÁ‚ÂÒÚÂÌ ‡Û‡, ˜ÂÒÚÓ ÂÙ‡ÍÚÓ̇ - ·ÂÁ HS, Ò Ù·ËÎÌË ÔËÒÚ˙ÔË 1 – 2 Ó ‰ÂÒÂÚËÎÂÚË Deja vu, ‡‚ÚÓÌÓÏ̇ ‡Û‡ 18q*, 1q* î‡ÏËÎ̇ Ó͈ËÔËÚÓÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl 1 - 4 ÚÓ ‰ÂÒÂÚËÎÂÚË ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, 9q* ÏË„Â̇, ‡ÁÌÓÓ·‡ÁÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË î‡ÏËÎ̇ Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò ÔÓÏÂÌÎË‚Ë Ó„Ìˢ‡ 1 – 3 ÚÓ ‰ÂÒÂÚËÎÂÚË ÙÓÌÚ‡ÎÌË, ÚÂÏÔÓ‡ÎÌË, Ó͈ËÔËÚ‡ÎÌË, ˆÂÌÚÓÚÂÏÔÓ‡ÎÌË ÔËÒÚ˙ÔË 22q12 è‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò ÔÂˈÂÌÚ‡ÎÌË ÒÔ‡ÈÍÓ‚Â 1 – 2 Ó ‰ÂÒÂÚËÎÂÚË ïÂÏËÍÓÌ‚ÛÎÒËË ˜ÂÒÚÓ ÌÓ˘ÂÏ, ‰Û„Ë 415* Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË *ë͇˜ÂÌÓÒÚ Â ÛÒÚ‡ÌÓ‚Â̇ ÔË Â‰Ì‡ Ù‡ÏËÎËfl, HS – ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡ *åÓ‰ËÙË͇ˆËfl ÔÓ Helbig et al. (2008)3 Ò ÔÓÚÂÔ‚‡ÌËfl ̇ ˙ˆÂÚÂ Ë Í‡Í‡Ú‡, ·˙ÁË ÌÂÍÓÓ‰ËÌË‡ÌË ‰‚ËÊÂÌËfl Ò ‰ËÒÚÓÌ˘ÂÌ ËÎË ‰ËÒÍËÌÂÚ˘ÂÌ ÍÓÏÔÓÌÂÌÚ, ÒÎÓÊÌË ÏÓÚÓÌË ‰ÂÈÌÓÒÚË, ‚ÌÂÁ‡ÔÌË ÔÓ‚‰Ë„‡ÌËfl ̇ ÚflÎÓÚÓ Ë „·‚‡Ú‡ Ò˙ÔÓ‚Ó‰ÂÌË ÓÚ ˜Û‚ÒÚ‚Ó Ì‡ ÒÚ‡ı Ë ‚Ó͇ÎËÁ‡ˆËfl. ë˙ÌflÚ flÁÍÓ Ò ÔÂÍ˙Ò‚‡, ‡ ÒΉ ÔËÒÚ˙Ô‡ Ò ‚˙Á‚˙˘‡ ÓÚÌÓ‚Ó. 臈ËÂÌÚËÚ ˜ÂÒÚÓ Ò˙Ó·˘‡‚‡Ú Á‡ ‡Û‡, ‡ Ò˙Á̇ÌËÂÚÓ ‚ ÏÌÓ„Ó ÓÚ ÒÎÛ˜‡ËÚ  Á‡Ô‡ÁÂÌÓ. ÄÛ‡Ú‡  ÌÂÒÔˆËÙ˘̇ – „ÂÌÂ‡ÎËÁË‡ÌÓ ËÁÚ˙Ô‚‡ÌÂ Ë ÚÂÔÂÂÌÂ, ˆÂه΄˘ÌË, ÚÓ‡Í‡ÎÌË Ë ÂÔË„‡ÒÚ‡ÎÌË ÛÒ¢‡ÌËfl,

èÓ̇ÒÚÓfl˘ÂÏ Ò‡ ˉÂÌÚËÙˈË‡ÌË ÚË ÎÓÍÛÒ‡ Á‡ ADNFLE. í ҇ ͇ÚË‡ÌË Ì‡ ıÓÏÓÁÓÏË 20q13 (ENFL1), 15q24 ( ENFL2), 1q21 (ENFL3). ë‡ÏÓ ÔË ‰‚‡ ÓÚ Úflı (ENFL1 Ë ENFL3)  ˉÂÌÚËÙˈË‡Ì „ÂÌ ÓÚ„Ó‚ÓÂÌ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. éÚ ÓÚÍË‚‡ÌÂÚÓ Ì‡ Ô˙‚Ëfl ÎÓÍÛÒ ENFL1 ‰Ó Ò„‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ˜ÂÚËË ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ CHRNA4 „Â̇ ÍÓ‰Ë‡˘ α4 Ò۷‰ËÌˈ‡Ú‡ ̇ Ì‚Ó̇ÎÌËfl ÌËÍÓÚËÌÓ‚ ‡ˆÂÚËÎıÓÎËÌÓ‚ ˆÂÔÚÓ (nAChR). íË Ò‡ ÏËÒÒÂÌÒ ÏÛÚ‡ˆËË (S252F, S256L Ë T265I), ‡ ‰̇  ËÌÒÂˆËfl ̇


ÑÂÍÂÏ‚Ë 2009

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

΂ˆËÌ (263InsL). í ҇ Ò˙Ó·˘ÂÌË Ò‡ÏÓ ‚ 10 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË (21, 35, 46, 47) Ë Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË ÓÒڇ̇ÎËÚ ADNFE Ù‡ÏËÎËË (ÓÍÓÎÓ 90% ) (22, 25, 50, 54, 55), ÍÓÂÚÓ Ô‰ÔÓ·„‡, ˜Â ÏÛÚ‡ˆËËÚ ‚ CHRNA4 „Â̇ ËÏ‡Ú Ï‡Î˙Í ÔËÌÓÒ ÔË ADNFE. Ç˙‚ ‚Ò˘ÍËÚ 10 Ù‡ÏËÎËË ÏÛÚ‡ˆËËÚ ҇ ·ËÎË ‚ ıÂÚÂÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌËÂ, ÍÓÂÚÓ Â ‚ Ò˙ÓÚ‚ÂÚÒÚ‚ËÂ Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌËfl ÏÓ‰ÂΠ̇ Û̇ÒΉfl‚‡ÌÂ. èÓ ÓÚÌÓ¯ÂÌË ̇ ENFL3 ÎÓÍÛÒ‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‰‚ ‡Á΢ÌË ÏÛÚ‡ˆËË (V287M and V287L) ‚ CHRNB2 „Â̇, ÍÓ‰Ë‡˘ β2 Ò۷‰ËÌˈ‡Ú‡ ̇ ÌËÍÓÚËÌÓ‚Ëfl ‡ˆÂÚËÎıÓÎËÌÓ‚ ˆÂÔÚÓ ÓÚÍËÚË ‚ ÚË Ö‚ÓÔÂÈÒÍË Ù‡ÏËÎËË (22, 25, 54). àÁ‚ÂÒÚÌËÚ ÏÛÚ‡ˆËË ËÏ‡Ú ‡Á΢ÂÌ ÂÙÂÍÚ ‚˙ıÛ Ò‚ÓÈÒÚ‚‡Ú‡ ̇ Ì‚Ó̇ÎÌËfl ÌËÍÓÚËÌÓ‚ ‡ˆÂÚËÎıÓÎËÌÓ‚ ˆÂÔÚÓ, ÌÓ Í‡ÈÌËflÚ ÂÁÛÎÚ‡Ú Â Ì‡Ï‡ÎÂÌ ÔÂχ·ËÎËÚÂÚ Á‡ ë‡++, Û‚Â΢ÂÌ ÔÂËÓ‰ ̇ Ë̇ÍÚË‚ËÚÂÚ, ̇χÎÂÌÓ ‚ÂÏ ̇ ÓÚ‚‡flÌ ̇ ͇̇· Ë Ôӂ˯Â̇ ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ Í˙Ï ‡„ÓÌËÒÚË (56). é·˘Ó ÏÛÚ‡ˆËËÚ ‚ CHRNA4 Ë CHRNB2 „ÂÌËÚ Ò ÓÚÍË‚‡Ú Ò‡ÏÓ ‚ 12% ÓÚ Ù‡ÏËÎËËÚÂ Ò ADNFE, ‰Ó͇ÚÓ ÔË ÓÒڇ̇ÎËÚ 88% ÓÚ ÒÎÛ˜‡ËÚ „ÂÌÂÚ˘ÌËflÚ ‰ÂÙÂÍÚ ÓÒÚ‡‚‡ ÌÂËÁ‚ÂÒÚÂÌ. è‰ÔÓ·„‡ Ò Ò˙˘ÂÒÚ‚Û‚‡ÌÂ Ë Ì‡ ˜ÂÚ‚˙ÚË ÎÓÍÛÒ Ò „ÂÌ ‡Á΢ÂÌ ÓÚ ÚÂÁË ÍÓ‰Ë‡˘Ë Ò۷‰ËÌËˆË Ì‡ Ì‚Ó̇ÎÌËfl ÌËÍÓÚËÌÓ‚ ‡ˆÂÚËÎıÓÎËÌÓ‚ ˆÂÔÚÓ (13). ë˙˘ÂÒÚ‚Û‚‡ÌÂÚÓ Ì‡ ‡Á΢ÌË ÎÓÍÛÒË, ͇ÍÚÓ Ë ‚‡ˇ·ËÎ̇ڇ ÂÍÒÔÂÒË‚ÌÓÒÚ Ì‡ ÒËÏÔÚÓÏËÚ ̇·Î˛‰‡‚‡Ì‡ Ò‰ ˜ÎÂÌÓ‚ÂÚ ̇ ‰̇ Ë Ò˙˘‡ Ù‡ÏËÎËfl Ò Â‰ËÌ Ë Ò˙˘ „ÂÌÂÚ˘ÂÌ ‰ÂÙÂÍÚ Ô‰ÔÓ·„‡ ̇΢ËÂÚÓ Ì‡ ÏÓ‰ËÙˈË‡˘Ë „ÂÌË Ë Ì„ÂÌÂÚ˘ÌË Ù‡ÍÚÓË ÏÓ‰ÛÎË‡˘Ë ÙÂÌÓÚËÔ‡ ̇ ‚ÒÂÍË Ë̉˂ˉ (21). îÄåàãçà ÖèàãÖèëàà çÄ íÖåèéêÄãçàü Ñüã ë˙˘ÂÒÚ‚Û‚‡Ú ‰‚‡ ÓÒÌÓ‚ÌË ÚËÔ‡ Ù‡ÏËÎÌË ÂÔËÎÂÔÒËË Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÒÂÏËÓÎÓ„ËflÚ‡ ̇ ÔËÒÚ˙ÔËÚÂ, „ÂÌÂÚ˘ÌËÚÂ Ë åêí ı‡‡ÍÚÂËÒÚËÍË: ·ÚÂ‡Î̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (FLTLE, ̇˜Â̇ Ӣ ÄÑ Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË) Ë ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (FMTLE). ãÄíÖêÄãçÄ ÖèàãÖèëàü çÄ íÖåèéêÄãçàü Ñüã (ÄÑ èÄêñàÄãçÄ ÖèàãÖèëàü ëöë ëãìïéÇà ëàåèíéåà) íÓ‚‡  ·ÂÌË„ÌÂÌ ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ ÓÔËÒ‡Ì Á‡ Ô˙‚Ë Ô˙Ú ‚ ÎËÚÂ‡ÚÛ‡Ú‡ ÔÂÁ 1995„. ÓÚ Ottman et al. (51). ì̇ÒΉfl‚‡ Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Ò 50-80% ÔÂÌÂÚ‡ÌÌÓÒÚ (58). Ç˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚˙‚ ‚ÚÓÓÚÓ Ë ÚÂÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ. Ñ‚‡Ú‡ ÔÓ· Ò Á‡Òfl„‡Ú Ò Â‰Ì‡Í‚‡ ˜ÂÒÚÓÚ‡. èÓfl‚fl‚‡ ÒÂ Ò ÔÓÒÚË ÒÎÛıÓ‚Ë ı‡Î˛ˆË̇ˆËË ÔÓ‰ ÙÓχڇ ̇ ·˙ϘÂÌÂ, Á‚˙ÌÂÌÂ, ˘‡Í‡ÌÂ Ë ÌÂı‡‡ÍÚÂÂÌ ¯ÛÏ. éÔËÒ‡ÌË Ò‡ Ë ‰Û„Ë ÔÒËı˘ÌË, ÒÂÚË‚ÌË (ÁËÚÂÎÌË, Ó·ÓÌflÚÂÎÌË, ‚ÂÚË„ËÌÓÁÌË ) Ë ÏÓÚÓÌË ÙÂÌÓÏÂÌË, ÌÓ ‡Û‡Ú‡ Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË Â Ì‡È- ı‡‡ÍÚÂ̇ڇ ÔÓfl‚‡ ̇ ÚÓÁË ÒË̉ÓÏ. ë˙Ó·˘ÂÌË Ò‡ Ù‡ÏËÎËË ÔË ÍÓËÚÓ ÔÂӷ·‰‡‚‡˘‡ ÍÎËÌ˘̇ ËÁfl‚‡ ̇ ÒË̉Óχ  ËÍÚ‡Î̇ ÒÂÌÁÓ̇ ‡Ù‡ÁËfl (15, 16, 39), Ú‡ÍË‚‡ ÔË ÍÓËÚÓ ÔËÒÚ˙ÔËÚ Ò ÔÓ‚ÓÍË‡Ú ÓÚ ÚÂÎÂÙÓÌÂÌ Á‚˙Ì (48, 66), ͇ÍÚÓ Ë Ú‡ÍË‚‡ Ò ˜ÂÒÚË ‚ÚÓ˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌ˘ÌÓ-ÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË (15, 48, 66). ç‚ÓÔÒËı˘ÌÓÚÓ ‡Á‚ËÚË  ÌÓχÎÌÓ. ÖÖÉ Ì‡È-˜ÂÒÚÓ Â ÌÓχÎ̇ ËÎË Ò ÂÔËÎÂÔÚËÙÓÏÂÌË ‡Áfl‰Ë Á‡‰ÌÓ ÚÂÏÔÓ‡ÎÌÓ. åêí ËÁÒΉ‚‡ÌÂÚÓ Â ÌÓχÎÌÓ ËÎË ÔÓ͇Á‚‡ χÎÙÓχˆËË ‚ ·ÚÂ‡ÎÌËÚ ӷ·ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ ÔË ÓÍÓÎÓ 45% ÓÚ Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë ( ‚ÍÎ. Ë ÔË ‡ÒËÏÔÚÓÏÂÌ ÌÓÒËÚÂΠ̇ ÏÛÚ‡ˆËfl) (42). ã‚ËflÚ ÚÂÏÔÓ‡ÎÂÌ ‰flΠ̇ ÚÂÁË Ô‡ˆËÂÌÚË ËÁ„ÎÂʉ‡ Û„ÓÎÂÏÂÌ Ë ÔÓÌflÍÓ„‡ Ò ̇·Î˛‰‡‚‡ ÔÓÚÛÁËfl ̇ ÏÓÁ˙˜ÌËfl Ô‡ÂÌıËÏ Î‡ÚÂ‡ÎÌÓ (Â̈ÂÙ‡ÎÓˆÂΠÔÓ‰Ó·ÂÌ ‚ˉ).

169

á̇˜ÂÌËÂÚÓ Ì‡ ÚÂÁË ÒÚÛÍÚÛÌË ‡ÌÓχÎËË Á‡ ÂÔËÎÂÔÚÓ„ÂÌÂÁ‡Ú‡  ‚Ò Ӣ ÌÂËÁ‚ÂÒÚÌÓ. ÑËÙÂÂ̈ˇÎ̇ ‰Ë‡„ÌÓÁ‡ Ò Ô‡‚Ë Ò˙Ò ÒËÏÔÚÓχÚ˘̇ ·ÚÂ‡Î̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ, ͇ÍÚÓ Ë Ù‡ÏËÎ̇ ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ. èÓ„ÌÓÁ‡Ú‡  ‰Ó·‡, ÔËÒÚ˙ÔËÚ ҇ ÎÂÍË, ‰ÍË Ë Ò ÍÓÌÚÓÎË‡Ú ‰Ó·Â Ò ‡ÌÚËÂÔËÎÂÔÚ˘ÌË Ï‰Ë͇ÏÂÌÚË. èË ÓÍÓÎÓ 50% ÓÚ Ù‡ÏËÎËËÚÂ Ò Î‡ÚÂ‡Î̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÏÛÚ‡ˆËfl ‚ leucin-rich, glioma inactivated 1 „Â̇ ( LGI1 ) ÎÓ͇ÎËÁË‡Ì ̇ ‰˙΄ÓÚÓ ‡ÏÓ Ì‡ 10 ıÓÏÓÁÓχ-10q (10, 38, 48, 51). 燉 20 ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ ÚÓÁË „ÂÌ Ò‡ Ò˙Ó·˘ÂÌË ‰Ó Ò„‡, Ôӂ˜ÂÚÓ ÓÚ ÍÓËÚÓ Ò‡ ◊˜‡ÒÚÌË” Ú. ÒÔˆËÙ˘ÌË Á‡ ÓÚ‰ÂÎÌËÚ هÏËÎËË (52). LGI1 „Â̇ ÍÓ‰Ë‡ ÏÂÏ·‡ÌÌÓ Ò‚˙Á‡Ì ÔÓÚÂËÌ Ò ÌÂËÁ‚ÂÒÚ̇ ÙÛÌ͈Ëfl. ï‡‡ÍÚÂËÁË‡ ÒÂ Ò ˆÂÌÚ‡ÎÂÌ ·Ó„‡Ú ̇ ΂ˆËÌ „ËÓÌ, ÍÓÈÚÓ ‚ÂÓflÚÌÓ Û˜‡ÒÚ‚‡ ‚ „Û·ˆËflÚ‡ ̇ ÍÎÂÚ˙˜ÌËfl ‡ÒÚÂÊ, ‡‰ıÂÁËfl Ë ÏË„‡ˆËfl (36). äÎÓÌË‡Ì  ÓÚ „ÎËӷ·ÒÚÓÏ̇ ÍÎÂÚ˙˜Ì‡ ÎËÌËfl Ë ‚ÂÓflÚÌÓ Ô‰ÒÚ‡‚Îfl‚‡ ÚÛÏÓ-ÒÛÔÂÒÓÂÌ „ÂÌ (19). ÄÒÓˆËË‡ÌÂÚÓ ÏÛ Ò Ï‡ÎÙÓχˆËË Ì‡ ·ÚÂ‡ÎÌËÚ ӷ·ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ ÔË ÌflÍÓË Ù‡ÏËÎËË Ô‰ÔÓ·„‡ ‚ÂÓflÚÌÓÚÓ ÏÛ Á̇˜ÂÌË Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (42). åÛÚ‡ˆËËÚ ‚ LGI1 „Â̇ ËÁ„ÎÂʉ‡ Ò‡ ÒÔˆËÙ˘ÌË Á‡ ÒË̉Óχ, ÌÓ Ë‰ÂÌÚËÙˈË‡ÌÂÚÓ ËÏ Ò‡ÏÓ ÔË ÔÓÎÓ‚Ë̇ڇ ÓÚ Ù‡ÏËÎËËÚÂ Ò ÚËÔ˘ÂÌ ÙÂÌÓÚËÔ ÔÓ͇Á‚‡ „ÂÌÂÚ˘̇ڇ ıÂÚÂÓ„ÂÌÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ (10). Bisulli et al. ( 2004 )(12) ÓÔËÒ‚‡Ú 53 ÒÔÓ‡‰Ë˜ÌË ÒÎÛ˜‡fl ̇ ·ÚÂ‡��̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ. ëË̉ÓÏ˙Ú Â Ì‡˜ÂÌ ÓÚ ‡‚ÚÓËÚ ˉËÓÔ‡Ú˘̇ Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË Ë Ì Ò ‡Á΢‡‚‡ ÍÎËÌ˘ÌÓ ÓÚ Ù‡ÏËÎ̇ڇ ÙÓχ. èË ÌÂ„Ó Ó·‡˜Â ÎËÔÒ‚‡ ÏÛÚ‡ˆËfl ‚ LGI1 „Â̇. îÄåàãçÄ åÖáàÄãçÄ ÖèàãÖèëàü çÄ íÖåèéêÄãçàü Ñüã (FMTLE) éÔË҇̇  Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Bercovic et al. (7, 8) ÔÂÁ 1994„. ͇ÚÓ ·ÂÌË„ÌÂÌ ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ. Ç ÔÓÒΉÒÚ‚Ë Ò ÓÚÍË‚‡Ú Ù‡ÏËÎËË Ò Ì ÚÓÎÍÓ‚‡ ‰Ó·Ó͇˜ÂÒÚ‚ÂÌÓ ÔÓÚ˘‡Ì ̇ ÂÔËÎÂÔÒËflÚ‡ Ë Ô‰¯ÂÒÚ‚‡˘Ë ÛÒÎÓÊÌÂÌË Ù·ËÎÌË ÔËÒÚ˙ÔË, ͇ÍÚÓ Ë ËÁ‡ÁÂ̇ ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡. íÓ‚‡ ‰Ó‚Âʉ‡ ‰Ó ‡Á‰ÂÎflÌÂÚÓ Ì‡ ÒË̉Óχ ̇ ÚË ÓÒÌÓ‚ÌË „ÛÔË: ● ·ÂÌË„ÌÂ̇ Ù‡ÏËÎ̇ ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ: ë˙ÓÚ‚ÂÚÒÚ‚‡ ̇ ÓË„Ë̇ÎÌÓÚÓ ÓÔËÒ‡ÌË ̇ Bercovic et al.(1994, 1996) (7, 8). ì̇ÒΉfl‚‡ Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Ò ÌÂÔ˙Î̇ ÔÂÌÂÚ‡ÌÌÓÒÚ. ÜÂÌËÚ Ò Á‡Òfl„‡Ú ÔÓ˜ÂÒÚÓ ÓÚ Ï˙ÊÂÚÂ. 燘‡ÎÓÚÓ Â ÏÂÊ‰Û ‚ÚÓÓÚÓ Ë ˜ÂÚ‚˙ÚÓÚÓ ‰ÂÒÂÚËÎÂÚË ÓÚ ÊË‚ÓÚ‡ (Ì ÔÓ-‡ÌÓ ÓÚ 10 „Ó‰. ‚˙Á‡ÒÚ, Ò‰ÌÓ 25 „Ó‰ ). ãËÔÒ‚‡ ‡Ì‡ÏÌÂÁ‡ Á‡ Ô‰¯ÂÒÚ‚‡˘Ë Ù·ËÎÌË ÔËÒÚ˙ÔË, ‡ „·‚ÌËÚ ÂÔËÎÂÔÚ˘ÌË ÔÓfl‚Ë ‚ Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Ò‡ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò ÔÒËı˘ÌË Ë ‡‚ÚÓÌÓÏÌË ÒËÏÔÚÓÏË, Ô‰ÔÓ·„‡˘Ë ÔÓËÁıÓ‰ ÓÚ ÏÂÁˇÎÌËfl ‰flΠ̇ ÚÂÏÔÓ‡ÎÌËfl ÎÓ·. ç‡È-˜ÂÒÚËÚ ÒËÏÔÚÓÏË Ì‡ ÂÔËÎÂÔÚ˘̇ڇ ‡Û‡ Ò‡ ÙÂÌÓÏÂÌË Ì‡ deja-vu Ë jamais vu Ë ÔÓ-fl‰ÍÓ „‡‰ÂÌÂ, Ú‡ıË͇‰Ëfl, ÒÚ‡ı, ˜Û‚ÒÚ‚Ó Á‡ Á‡·‡‚ÂÌÓÒÚ Ì‡ ‰‚ËÊÂÌËflÚ‡ Ë ÒÍÓ‚‡ÌÓÒÚ, ‰ÂÙÓÏË‡ÌÓ ‚˙ÁÔËÂχÌ ̇ Ó·ÂÍÚËÚÂ Ë Á‚ÛˆËÚÂ, ‰ËÙÛÁÌË Ô‡ÂÒÚÂÁË. ãËÔÒ‚‡ ÂÔË„‡ÒÚ‡Î̇ ‡Û‡. èË Ôӂ˜ÂÚÓ Á‡Ò„̇ÚË ËÌ‰Ë‚Ë‰Ë ÂÔËÎÂÔÒËflÚ‡ ÔÓÚ˘‡ Ò‡ÏÓ Ò ÔÓ‚Ú‡fl˘Ë Ò ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò „ÓÂÓÔË҇̇ڇ ı‡‡ÍÚÂËÒÚË͇, ÍÓËÚÓ ˜ÂÒÚÓ Ì Ò ‡ÁÔÓÁ̇‚‡Ú ͇ÚÓ ÂÔËÎÂÔÚ˘ÌË. ë˙Ó·˘‡‚‡Ú ÒÂ Ë ‰ÍË, ÎÂÍË ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ë ‚ÚÓ˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌ˘ÌÓ – ÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË ÎÂÒÌÓ ÍÓÌÚÓÎË‡˘Ë ÒÂ Ò ‡ÌÚËÂÔËÎÂÔÚ˘ÌË Ï‰Ë͇ÏÂÌÚË. ç‚ÓÎӄ˘ÌÓÚÓ Ë


ÑÂÍÂÏ‚Ë 2009

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

ÔÒËı˘ÌÓÚÓ ‡Á‚ËÚË ҇ ÌÓχÎÌË. àÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ Â Ó·ËÍÌÓ‚ÂÌÓ ÌÓχÎ̇, ËÎË Ò ÙÓ͇ÎÌË ·‡‚ÌË ‚˙ÎÌË, ËÎË ‰ÍË ÍÓÏÔÎÂÍÒË ÓÒÚËÂ-·‡‚̇ ‚˙Î̇ ÎÓ͇ÎËÁË‡ÌË Â‰ÌÓÒÚ‡ÌÌÓ ÚÂÏÔÓ‡ÎÌÓ. åêí ËÁÒΉ‚‡ÌÂÚÓ Â ÌÓχÎÌÓ. èÓ„ÌÓÁ‡Ú‡  ÓÚÌÓÒËÚÂÎÌÓ ·Î‡„ÓÔËflÚ̇. èÓ-Í˙ÒÌÓ ÔÓ‰Ó·ÌË Ù‡ÏËÎËË Ò‡ Ò˙Ó·˘ÂÌË Ë ÓÚ ‰Û„Ë ‡‚ÚÓË (29, 57, 65), ͇ÚÓ ‚ ‰ËÌ˘̇ „ÓÎflχ Ù‡ÏËÎËfl  ÛÒÚ‡ÌÓ‚Â̇ Ò͇˜ÂÌÓÒÚ Ò ıÓÏÓÁÓχ 4q13.2-q21.3 (31). ● Ù‡ÏËÎ̇ ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ ‡ÒÓˆËË‡Ì‡ Ò ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡: 燘‡ÎÓÚÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚ Ô˙‚ËÚ ÚË ‰ÂÒÂÚËÎÂÚËfl ÓÚ ÊË‚ÓÚ‡ (42). ëÂÏËÓÎÓ„ËflÚ‡ ̇ ÔËÒÚ˙ÔËÚ  ı‡‡ÍÚÂ̇ Á‡ ÏÂÁˇÎÌËÚ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ - ÂÔË„‡ÒÚ‡ÎÌË ÛÒ¢‡ÌËfl ËÁ‰Ë„‡˘Ë Ò Í˙Ï „˙ÎÓÚÓ Ë ÔÒËı˘ÌË ÙÂÌÓÏÂÌË. óÂÒÚË Ò‡ ÍÓÏÔÎÂÍÒÌËÚ ԇˆË‡ÎÌË ÔËÒÚ˙ÔË Ò Ó‡ÎÌË ËÎË ÊÂÒÚÓ‚Ë ‡‚ÚÓχÚËÁÏË Ë ËÁ‡ÁÂÌÓ ÔÓÒÚËÍÚ‡ÎÌÓ Ó·˙Í‚‡Ì ( ÔÓ‰Ó·ÌÓ Ì‡ ÚÓ‚‡ ̇·Î˛‰‡‚‡ÌÓ ÔË Ô‡ˆËÂÌÚË Ò ÌÂÙ‡ÏËÎ̇ ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ). ÇÚÓ˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌ˘ÌÓ-ÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË Ò ̇·Î˛‰‡‚‡Ú Ó·ËÍÌÓ‚ÂÌÓ ‚ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÔÂ‰Ë Á‡ÔÓ˜‚‡Ì ̇ ΘÂÌËÂÚÓ. Ä̇ÏÌÂÁ‡ Á‡ Ù·ËÎÌË ÔËÒÚ˙ÔË ‚ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó Ò ÓÚÍË‚‡ ÔÓfl‰ÍÓ ( ÓÍÓÎÓ 11% ) (42) ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚË Ò FMTLE ÔÓÒΉfl‚‡ÌË ‚ ıËÛ„˘ÌË ÓÚ‰ÂÎÂÌËfl ( 30% ) (43) Ë Ó˘Â ÔÓ-fl‰ÍÓ ÔË Ú‡ÍË‚‡ Ò ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl ÌÂÒÂÎÂÍÚË‡ÌË Á‡ Ù‡ÏËÎ̇ ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl ( 40% ) (2). àÌÚÂËÍÚ‡Î̇ڇ Ë ËÍÚ‡Î̇ ÖÖÉ ÔÓ͇Á‚‡Ú ‰ÌÓÒÚ‡ÌÌË ËÎË ‰‚ÛÒÚ‡ÌÌË ÂÔËÎÂÔÚËÙÓÏÂÌË flÁfl‰Ë ‚ ÏÂÁˇÎÌËÚ ÚÂÏÔÓ‡ÎÌË Ó·Î‡ÒÚË, ‚˙ÔÂÍË, ˜Â ÔË ÌflÍÓË Ô‡ˆËÂÌÚË ÖÖÉ Ì‡ıӉ͇ڇ ÏÓÊ ‰‡  ÌÓχÎ̇. åêí ËÁÒΉ‚‡ÌÂÚÓ ‡ÁÍË‚‡ ‡Á΢̇ ÔÓ ÒÚÂÔÂÌ ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡ ‰ÓË Ë ÔË ‡ÒËÏÔÚÓÏÌË ˜ÎÂÌӂ ̇ Á‡Ò„̇ÚËÚ هÏËÎËË (44). ífl Ó·‡˜Â  ̇È-˜ÂÒÚ‡ Ë Ì‡È-ÚÂÊÍÓ ËÁ‡ÁÂ̇ ÔË Ô‡ˆËÂÌÚË Ò ÂÙ‡ÍÚÂÌË Ì‡ ΘÂÌË ÔËÒÚ˙ÔË (45). ç‡Î˘ËÂÚÓ Ì‡ åêí ‰‡ÌÌË Á‡ ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡ ͇ÍÚÓ ÔË ‡ÒËÏÔÚÓÏÌË, ڇ͇ Ë ÔË ·ÓÎÌË ˜ÎÂÌӂ ̇ Ù‡ÏËÎËflÚ‡ Ô‰ÔÓ·„‡ ‚˙ÁÏÓÊÌÓÒÚÚ‡ ÚÂÁË ‡·ÌÓÏÌÓÒÚË ‰‡ Ò Û̇ÒΉfl‚‡Ú ·ÂÁ Á‡‰˙ÎÊËÚÂÎÌÓ ‰‡ ‚Ó‰flÚ ‰Ó ‡Á‚ËÚË ̇ ÂÔËÎÂÔÒËfl (44). ïÓ‰˙Ú Ì‡ ÔÓÚ˘‡Ì  ÓÚÌÓÒËÚÂÎÌÓ ·Î‡„ÓÔËflÚÂÌ Ò˙Ò ÒÔÓÌÚ‡ÌÌË ÂÏËÒËË. êÂÙ‡ÍÚÓÌË ÔËÒÚ˙ÔË Ò ÓÔËÒ‚‡Ú ÔË ÓÍÓÎÓ 29% ÓÚ Ô‡ˆËÂÌÚËÚ (45). ÑÓ ÏÓÏÂÌÚ‡ Ì  ˉÂÌÚËÙˈË‡Ì ÓÚ„Ó‚ÓÂÌ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ „ÂÌ. Ç˙ÔÂÍË, ˜Â Ò‡ ÓÔËÒ‚‡ÌË ÔÓÎËÏÓÙËÁÏË ÔË Ô‡ˆËÂÌÚË Ò ÏÂÁˇÎ̇ ÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl ( prodynorphin „ÂÌ, Apoε „ÂÌ, GABAÇ „ÂÌ, prion protein „ÂÌ ) Ú‡ÍË‚‡ Ì ҇ ÛÒÚ‡ÌÓ‚ÂÌË Ò‰ Ó‰ÓÒÎÓ‚Ëfl Ò Ù‡ÏËÎ̇ ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (18, 63). ● Ù‡ÏËÎ̇ ÏÂÁˇÎ̇ ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ ‡ÒÓˆËË‡Ì‡ Ò Ù·ËÎÌË ÔËÒÚ˙ÔË, ·ÂÁ åêí ‰‡ÌÌË Á‡ ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡: Baulac et al. (2001) (6) ÓÔËÒ‚‡Ú ÙÂÌÒ͇ Ù‡ÏËÎËfl ÔË ÍÓflÚÓ Ò ̇·Î˛‰‡‚‡Ú Ù·ËÎÌË ÔËÒÚ˙ÔË ‡ÒÓˆËË‡ÌË Ò ÂÔËÎÂÔÒËfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ, ·ÂÁ åêí ‰‡ÌÌË Á‡ ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡. Depondt et al. (2002„.) (23) ÓÔËÒ‚‡Ú „ÓÎflχ ·Â΄ËÈÒ͇ Ù‡ÏËÎËfl Ò ÔÓ‰Ó·ÂÌ ÙÂÌÓÚËÔ. ì̇ÒΉfl‚‡ÌÂÚÓ Â ‡‚ÚÓÁÓÏÌÓ ‰ÓÏË̇ÌÚÓ Ò 80 % ÔÂÌÂÚ‡ÌÌÓÒÚ, ͇ÚÓ Â Ì‡ÎËˆÂ Ë ËÁ‡ÁÂ̇ ÙËÌÓÚËÔ̇ ıÂÚÂÓ„ÂÌÌÓÒÚ. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ Ì‡ ‡Ù·ËÎÌËÚ ÔËÒÚ˙ÔË Â 8 „Ó‰ËÌË. ëÂÏËÓÎÓ„ËflÚ‡ ̇ ÔËÒÚ˙ÔËÚ  ı‡‡ÍÚÂ̇ Á‡ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ, ͇ÚÓ Ì‡È-˜ÂÒÚËÚ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò‡ ‚ËÒˆÂÓÒÂÌÁÓÌË, ÔÒËı˘ÌË Ë ÓÚ ÒÚ‡Ì‡ ̇ ñçë. ç Ò Ò˙Ó·˘‡‚‡Ú deja vu ÙÂÌÓÏÂÌË, ÒÎÛıÓ‚Ë ËÎË ÁËÚÂÎÌË ı‡Î˛ˆË̇ˆËË. äÓÏÔÎÂÍÒÌËÚ ԇˆË‡ÎÌË ÔËÒÚ˙ÔË ÔÓÚ˘‡Ú Ò Ì‡Û¯ÂÌÓ Ò˙Á̇ÌËÂ, ‚ÚÂ̘‚‡ÌÂ, ÓÚÍÎÓÌfl‚‡Ì ̇ „·‚‡Ú‡, ıËÔÂ‚ÂÌÚË·ˆËfl, ‚Ó͇ÎËÁ‡ˆËfl, ‰ËÒÚÓÌ˘ÌË ÔÓÁË, ‡‚ÚÓχÚËÁÏË Ë ÛÌË·ÚÂ‡ÎÌË ÍÓÌ‚ÛÎÒËË. èËÒÚ˙ÔËÚ ҇ Ô‰ËÏÌÓ ÔÂÁ ‰ÂÌfl. óÂÒÚÓÚ‡Ú‡ ̇ Ù·-

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ËÎÌËÚ ÔËÒÚ˙ÔË Â ‚ËÒÓ͇ ÓÚ 50% (23) ‰Ó 100% (6), ͇ÚÓ Ú‡ÍË‚‡ Ì Ò ÓÔËÒ‚‡Ú ÒΉ 6 „Ӊ˯̇ ‚˙Á‡ÒÚ. àÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ Â ËÎË ÌÓχÎ̇ ËÎË Ò ÂÔËÎÂÔÚËÙÓÏÂÌË ‡·ÌÓÏÌÓÒÚË ‚ ÚÂÏÔÓ‡ÎÌËÚ ÓÚ‚Âʉ‡ÌËfl. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Â ·ÂÁ ‰‡ÌÌË Á‡ ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡. èÓ„ÌÓÁ‡Ú‡ Ó·ËÍÌÓ‚ÂÌÓ Â ‰Ó·‡ (20). èӂ‰ÂÌËflÚ „ÂÌÓÏÂÌ ÒÍ‡Ì Ì‡ ÙÂÌÒ͇ڇ Ù‡ÏËÎËfl ˉÂÌÚËÙˈË‡ ‰‚‡ ‡Á΢ÌË ÎÓÍÛÒ‡ ̇ ıÓÏÓÁÓÏË 18qter Ë 1q25-q31, ·ÂÁ ‰‡  ÛÒÚ‡ÌÓ‚ÂÌ ÓÚ„Ó‚ÓÂÌ „ÂÌ. Depondt et al. (2002„) (23) ÛÒÚ‡ÌÓ‚fl‚‡Ú Ò͇˜ÂÌÓÒÚ Ò ıÓÏÓÁÓχ 12q2223.3. îÄåàãçÄ èÄêñàÄãçÄ ÖèàãÖèëàü ë èêéåÖçãàÇà éÉçàôÄ (FPEVF) íÓ‚‡  ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ ı‡‡ÍÚÂËÁË‡˘ ÒÂ Ò ‡Á΢ÂÌ ÚËÔ Ë ÍÓÓ‚‡ ÎÓ͇ÎËÁ‡ˆËfl ̇ Ô‡ˆË‡ÎÌËÚ ÔËÒÚ˙ÔË ÔË ÓÚ‰ÂÎÌËÚ هÏËÎËË, ÔË ÓÚÒ˙ÒÚ‚ËÂÚÓ Ì‡ ͇͂ËÚÓ Ë ‰‡ ·ËÎÓ ÒÚÛÍÚÛÌË ÎÂÁËË ‚ ÏÓÁ˙˜ÌËfl Ô‡ÂÌıËÏ (3). èÓ̇ÒÚÓfl˘ÂÏ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ò‡ ÓÔËÒ‡ÌË ÓÍÓÎÓ 10 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË. èËÒÚ˙ÔËÚÂ Ë ÖÖÉ ÔÓÏÂÌËÚ ҇ ÔÓÒÚÓflÌÌË ‚˙‚ ‚ÂÏÂÚÓ ÔË ‚ÒÂÍË ÓÚ Á‡Ò„̇ÚËÚ ˜ÎÂÌӂ ̇ Ù‡ÏËÎËËÚÂ Ë ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÙÓÌÚ‡ÎÌË, ÚÂÏÔÓ‡ÎÌË ËÎË Ó͈ËÔËÚ‡ÎÌË. éÒ‚ÂÌ ËÁ‡ÁÂÌËÚ ËÌÚÂÙ‡ÏËÎÌË ‚‡ˇˆËË ‚ ÍÎËÌ˘̇ڇ ͇ÚË̇ Ò‡ ̇ÎËˆÂ Ë ËÌÚ‡Ù‡ÏËÎÌË ‚‡ˇˆËË, ÍÓÂÚÓ ˜ÂÒÚÓ ‚Ó‰Ë ‰Ó ÔÓ„¯̇ ‰Ë‡„ÌÓÁ‡ Ò ‰Û„Ë Ù‡ÏËÎÌË ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË. ì̇ÒΉfl‚‡ÌÂÚÓ Â ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Ò 70% ÔÂÌÂÚ‡ÌÌÓÒÚ, ͇ÚÓ ‰‚‡Ú‡ ÔÓ· Ò Á‡Òfl„‡Ú Ò Â‰Ì‡Í‚‡ ˜ÂÒÚÓÚ‡. 燘‡ÎÓ Ì‡ ÔËÒÚ˙ÔËÚ ‚‡Ë‡ Á̇˜ËÚÂÎÌÓ, ÌÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚ Ô˙‚ËÚ ÚË ‰ÂÒÂÚËÎÂÚËfl ÓÚ ÊË‚ÓÚ‡, ͇ÚÓ Ò ÓÙÓÏflÚ ‰‚‡ ÔË͇ ̇ 5 Ë Ì‡ 25 „Ӊ˯̇ ‚˙Á‡ÒÚ (67). èËÒÚ˙ÔËÚ ÔÓËÁÚ˘‡˘Ë ÓÚ Ó„Ìˢ ‚˙‚ ÙÓÌÚ‡ÎÌËfl ‰flÎ Ò‡ ̇È-˜ÂÒÚÓ ÓÔËÒ‚‡ÌËÚ ÔË ÚÓÁË ÒË̉ÓÏ. í Ò ‡Á΢‡‚‡Ú ÓÚ ÚÂÁË ÔË ADNFLE ÔÓ ÚÓ‚‡, ˜Â ÔÓ-fl‰ÍÓ Ò „ÛÔË‡Ú, ˜ÂÒÚÓÚ‡Ú‡ ËÏ Â ÔÓ-ÌËÒ͇, ‚˙ÁÏÓÊÌË Ò‡ ͇ÍÚÓ ÌÓ˘ÌË Ú‡Í‡ Ë ‰Ì‚ÌË ÔËÒÚ˙ÔË, ͇ÚÓ ÔÓÒΉÌËÚ ÏÓ„‡Ú ‰‡ ÔÂӷ·‰‡‚‡Ú, fl‰ÍÓ Ëχ ‡Û‡, ˜ÂÒÚ‡  ‚ÚÓ˘̇ڇ „ÂÌÂ‡ÎËÁ‡ˆËfl (11, 67). ÇÚÓË ÔÓ ˜ÂÒÚÓÚ‡ Ò‡ ÔËÒÚ˙ÔËÚ ÔÓËÁÚ˘‡˘Ë ÓÚ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ, ‰Ó͇ÚÓ ÚÂÁË Ò ˆÂÌÚÓÔ‡ËÂÚ‡ÎÂÌ Ë Ó͈ËÔËÚ‡ÎÂÌ ÔÓËÁıÓ‰ Á‡ ‰ÍË. àÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ ÔÓ͇Á‚‡ Ó„ÌˢÌË ÂÔËÎÂÔÚËÙÓÏÂÌË ‡·ÌÓÏÌÓÒÚË ÔË Ôӂ˜ÂÚÓ Ô‡ˆËÂÌÚË, ÎÓ͇ÎËÁË‡ÌË ‚ ÚÂÏÔÓ‡ÎÌËÚÂ, ÙÓÌÚ‡ÎÌËÚÂ, ˆÂÌÚÓÚÂÏÔÓ‡ÎÌËÚ ËÎË Ó͈ËÔËÚ‡ÎÌËÚ „ËÓÌË. èË Á‰‡‚Ë ˜ÎÂÌӂ ̇ Ù‡ÏËÎËËÚ Ò˙˘Ó ÏÓ„‡Ú ‰‡ Ò „ËÒÚË‡Ú ÖÖÉ ÔÓÏÂÌË, ÍÓÂÚÓ ‚ÂÓflÚÌÓ Â ı‡‡ÍÚÂÂÌ Á‡ ÒË̉Óχ ·Â΄ (53). íÂÊÂÒÚÚ‡ ̇ ÖÖÉ ÔÓÏÂÌËÚ ‚‡Ë‡ ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚ ËÌ‰Ë‚Ë‰Ë Ë Ì ÍÓÂÎË‡ Ò ˜ÂÒÚÓÚ‡Ú‡ ̇ ÔËÒÚ˙ÔËÚÂ. àÍÚ‡Î̇ڇ ÖÖÉ ÔÓ͇Á‚‡ Ó„ÌˢÌË ÔÓÏÂÌË. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ó·ËÍÌÓ‚ÂÌÓ Â ÌÓχÎ̇. àχ ËÁ‡ÁÂÌË ËÌÚ‡Ù‡ÏËÎÌË ‚‡ˇˆËË Ë ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÔÓ„ÌÓÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. èË Ô˙‚ÓÚÓ ÓÔËÒ‡ÌÓ Ó‰ÓÒÎÓ‚ËÂ Ò ÚÓÁË ÒË̉ÓÏ (‡‚ÒÚ‡ÎËÈÒ͇ Ù‡ÏËÎËfl)  ˉÂÌÚËÙˈË‡Ì ÎÓÍÛÒ Ì‡ ıÓÏÓÁÓχ 2q (64), ÌÓ ÔÓ‡‰Ë χÎÍËfl ÏÛ ‡ÁÏÂ ÔÓ-̇ڇÚ˙¯ÌË ËÁÒΉ‚‡ÌËfl Ì ҇ Ô‡‚ÂÌË. ë͇˜ÂÌÓÒÚ Ò ıÓÏÓÁÓχ 22q  ÛÒÚ‡ÌÓ‚Â̇ ‚ ÔÂÚ Ù‡ÏËÎËË (11,17, 67). íË ÓÚ Ù‡ÏËÎËËÚ ҇ ÓÚ ÙÂÌÒÍÓ-͇̇‰ÒÍË ÔÓËÁıÓ‰ (11, 67) Ë ËÏ‡Ú Â‰ËÌ Ë Ò˙˘ ı‡ÔÎÓÚËÔ, ÍÓÂÚÓ Ô‰ÔÓ·„‡ ◊ÂÙÂÍÚ Ì‡ Ô‡Ó‰ËÚÂÎfl”. Ç ÔÓÒΉÒÚ‚Ë ҇ ÓÔËÒ‡ÌË Â‰Ì‡ ËÒÔ‡ÌÒ͇ (11) Ë Â‰Ì‡ ‰‡ÚÒ͇ (62) Ù‡ÏËÎËfl Ë Ò͇˜ÂÌÓÒÚ Ò˙Ò Ò˙˘Ëfl ıÓÏÓÁÓÏÂÌ ÎÓÍÛÒ 22q, ÌÓ Ò ‡Á΢ÂÌ ı‡ÔÎÓÚËÔ. îÄåàãçÄ éäñàèàíéíÖåèéêÄãçÄ ÖèàãÖèëàü Deprez et al. (2007) (24) ÓÔËÒ‚‡Ú „ÓÎflχ ·Â΄ËÈÒ͇ Ù‡ÏËÎËfl Ò Ó͈ËÔËÚÓÚÂÏÔÓ‡Î̇ ÂÔËÎÂÔÒËfl ‡ÒÓˆËË‡Ì‡ Ò ÏË„Â̇ Ò˙Ò ÁËÚÂÎ̇ ‡Û‡. ì̇ÒΉfl‚‡ÌÂÚÓ Â ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË-


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

ÑÂÍÂÏ‚Ë 2009

̇ÌÚÓ Ò 75% ÔÂÌÂÚ‡ÌÌÓÒÚ. Ç˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ ‚‡Ë‡ ÏÂÊ‰Û 7 ÏÂÒˆ‡ Ë 63 „Ó‰ËÌË (Ò‰ÌÓ 21 „Ӊ˯̇ ‚˙Á‡ÒÚ). èËÒÚ˙ÔËÚ ҇ Ô‰ËÏÌÓ ‰Ì‚ÌË. éÔËÒ‡ÌË Ò‡ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË - ̇È-˜ÂÒÚÓ Ò˙Ò ÁËÚÂÎÌË ÒËÏÔÚÓÏË (60%), ÍÓ„ÌËÚË‚ÌË ÒËÏÔÚÓÏË (50%), ‡‚ÚÓÌÓÏÌË ÒËÏÔÚÓÏË, ÒÚ‡ı, ÏÓÚÓÌË, ‚ÂÚË„ËÌÓÁÌË Ë ÒÓχÚÓÒÂÌÁÓÌË ÒËÏÔÚÓÏË Ë Ó·ÓÌflÚÂÎÌË ËÎË ÒÎÛıÓ‚Ë ı‡Î˛ˆË̇ˆËË; ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË Ë ‚ÚÓ˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË. ãËÔÒ‚‡ ‡Ì‡ÏÌÂÁ‡ Á‡ Ù·ËÎÌË ÔËÒÚ˙ÔË. èË 50% ÓÚ ˜ÎÂÌÓ‚ÂÚ ̇ Ù‡ÏËÎËflÚ‡ Ó͈ËÔËÚÓÚÂÏÔÓ‡Î̇ڇ ÂÔËÎÂÔÒËfl Ò ‡ÒÓˆËË‡ Ò ÏË„Â̇ Ò ‡Û‡. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ Ì‡ ÏË„ÂÌÓÁÌËÚ ÔËÒÚ˙ÔË Â 42 „Ó‰ËÌË ( ÏÂÊ‰Û 30 ‰Ó 65 „Ӊ˯̇ ‚˙Á‡ÒÚ ). ç‡È-˜ÂÒÚ‡Ú‡ ÁËÚÂÎ̇ ‡Û‡ Ò ËÁ‡Áfl‚‡ ‚ ÔÓfl‚‡ ̇ ‰‚ÓÈÌÓ ‚Ëʉ‡ÌÂ, Ò‚ÂÚ͇‚ËˆË Ë ÒÍÓÚÓÏ. ë‰̇ڇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ ‡Û‡Ú‡  20 ÏËÌÛÚË. ÖÔËÎÂÔÚ˘ÌËÚÂ Ë ÏË„ÂÌÓÁÌËÚ ÔËÒÚ˙ÔË ÔË Ôӂ˜ÂÚÓ Á‡Ò„̇ÚË ËÌ‰Ë‚Ë‰Ë ÔÓÚ˘‡Ú ÌÂÁ‡‚ËÒËÏÓ Â‰ÌË ÓÚ ‰Û„Ë ‚˙‚ ‚ÂÏÂÚÓ. ç‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ Ó·ËÍÌÓ‚ÂÌÓ Â ÌÓχÎÂÌ. àÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ Ì‡È-˜ÂÒÚÓ Â ÌÓχÎ̇. åêí ËÁÒΉ‚‡ÌÂÚÓ Ò˙˘Ó  ÌÓχÎÌÓ. èË Ôӂ‰ÂÌËfl ‡Ì‡ÎËÁ Á‡ Ò͇˜ÂÌÓÒÚ Â Ë‰ÂÌÚËÙˈË‡Ì ÎÓÍÛÒ Ì‡ ıÓÏÓÁÓχ 9q21-q22, ÓÚ ÍÓÂÚÓ ÏÓÊ ‰‡ Ò Ô‰ÔÓÎÓÊË, ˜Â ÏÛÚ‡ˆËfl ‚ ‰ËÌÒÚ‚ÂÌ Ó·˘ „ÂÌ Â ÓÚ„Ó‚ÓÂÌ Â‰ÌÓ‚ÂÏÂÌÌÓ Á‡ ‰‚ÂÚ Á‡·ÓÎfl‚‡ÌËfl ( ÂÔËÎÂÔÒËfl Ë ÏË„Â̇ ). èÄêñàÄãçÄ ÖèàãÖèëàü ë èÖêàñÖçíêÄãçà ëèÄâäéÇÖ ëË̉ÓÏ˙Ú Â ÓÔËÒ‡Ì Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Kinton Ât al. 2002„. (40) ÔË ·‡ÁËÎÒ͇ Ù‡ÏËÎËfl. 燘‡ÎÓÚÓ Â ‚ Ô˙‚ËÚ ‰‚ ‰ÂÒÂÚËÎÂÚËfl ÓÚ ÊË‚ÓÚ‡, ͇ÚÓ ÔËÒÚ˙ÔËÚ ӷËÍÌÓ‚ÂÌÓ ÔÂÒËÒÚË‡Ú ‰Ó 70 „Ó‰. ‚˙Á‡ÒÚ, ‡ ÖÖÉ ÔÓÏÂÌËÚ ‰Ó 30 „Ó‰. ‚˙Á‡ÒÚ. èË Á‡Ò„̇ÚËÚ ˜ÎÂÌÓ‚Â ÓÚ Ù‡ÏËÎËflÚ‡ Ò ̇·Î˛‰‡‚‡Ú ‡Á΢ÌË ‚ˉӂ ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË – ıÂÏËÍÎÓÌ˘ÌË, ıÂÏËÚÓÌ˘ÌË, Éíäè, ÔÓÒÚË ÙÓ͇ÎÌË ( ÒÚÂÂÓÚËÔÌË ÂÔËÁÓ‰Ë ÓÚ ÂÔË„‡ÒÚ‡Î̇ ·ÓÎ͇) Ë ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò˙Ò ÒÂÏËÓÎÓ„Ëfl Ò˙ÓÚ‚ÂÚÒÚ‚‡˘‡ ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ. ï‡‡ÍÚÂÂÌ ·Â΄ ̇ ÚÓÁË ÒË̉ÓÏ Ò‡ ÖÖÉ ÔÓÏÂÌËÚ ÓÚ ÓÒÚËÂÚ‡ ËÎË ·‡‚ÌË ‚˙ÎÌË ‚ ÔÂˈÂÌÚ‡ÎÌËÚ „ËÓÌË (ˆÂÌÚÓÔ‡ËÂÚ‡ÎÌÓ, ˆÂÌÚÓÙÓÌÚ‡ÎÌÓ ËÎË ˆÂÌÚ‡ÚÂÏÔÓ‡ÎÌÓ). Ñˇ„ÌÓÁ‡Ú‡ ˜ÂÒÚÓ Ò Á‡ÚÛ‰Ìfl‚‡ ÓÚ ‚‡ˇ·ËÎ̇ڇ ÔÂÌÂÚ‡ÌÌÓÒÚ Ë ÍÎËÌ˘̇ ͇ÚË̇ Ò‰ Á‡Ò„̇ÚËÚ ˜ÎÂÌӂ ̇ Ù‡ÏËÎËflÚ‡. ïÓ‰˙Ú Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ·Î‡„ÓÔËflÚÂÌ. ìÒÚ‡ÌÓ‚Â̇  Ò͇˜ÂÌÓÒÚ Ò ıÓÏÓÁÓχ 415.

5. 6. 7.

8. 9. 10. 11. 12. 13.

14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29.

áÄäãûóÖçàÖ Ç˙ÔÂÍË, ˜Â „ÓÂÓÔËÒ‡ÌËÚ ÌÓ‚Ë ÒË̉ÓÏË Ò Ò¢‡Ú Ò‡‚ÌËÚÂÎÌÓ fl‰ÍÓ, ÔÓÁ̇‚‡ÌÂÚÓ ËÏ Ë Ì‡ÒÓ˜ÂÌÓÚÓ Ú˙ÒÂÌ ̇ Ù‡ÏËÎ̇ ‡Ì‡ÏÌÂÁ‡  ÓÚ „ÓÎflÏÓ Á̇˜ÂÌË Á‡ ÍÎËÌˈËÒÚËÚÂ. íÓ‚‡ ˘Â ÛÎÂÒÌË ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ÚӘ̇ ‰Ë‡„ÌÓÁ‡ Ë ˘Â ‰Ó‚‰ ‰Ó ÔÓ‚Âʉ‡Ì ̇ ÔÓ‰ıÓ‰fl˘Ó ΘÂÌËÂ, ÓÒË„Ûfl‚‡Ì ̇ ÔÓ-‰Ó·Ë „ËÊË Á‡ Ô‡ˆËÂÌÚËÚÂ Ë ÔÓ-ÚӘ̇ ÔÓ„ÌÓÁ‡ Á‡ ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. à‰ÂÌÚËÙˈË‡ÌÂÚÓ Ì‡ „ÂÌËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ ‡Á΢ÌËÚ ÂÔËÎÂÔÚ˘ÌË ÒË̉ÓÏË ˘Â ‰ÓÔËÌÂÒ Á‡ ÔÓ-‰Ó·Ó ‡Á·Ë‡Ì ̇ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ë ‰Ó ÔÓ-ÂÙÂÍÚË‚ÌÓ, „ÂÌÂÚ˘ÌÓ Ó·ÓÒÌÓ‚‡ÌÓ Î˜ÂÌË ̇ ÂÔËÎÂÔÒËËÚÂ, ‡ Ò˙˘Ó Ë Á‡ ÔÓ-̇ڇÚ˙¯ÌÓÚÓ ËÁflÒÌfl‚‡Ì ̇ ÌÓχÎÌÓÚÓ ÙÛÌ͈ËÓÌË‡Ì ̇ ÏÓÁ˙͇.

30. 31. 32. 33. 34. 35. 36. 37.

ãàíÖêÄíìêÄ 1. 2. 3. 4.

á‡ı‡Ë‚, á., ɇÌ‚‡, É ÖÔËÎÂÔÒËfl.- ‚ ç‚ÓÎÓ„Ëfl ÔÓ‰ ‰‡ÍˆËflÚ‡ ̇ ÔÓÙ.åË·ÌÓ‚ Ë ÔÓÙ.ü̘‚‡. ëÓÙËfl: å‰ˈË̇ Ë ÙËÁÍÛÎÚÛ‡ 2007; 545-546 Abou-Khalil B, Andermann E, Andermann F, et al. Temporal lobe epilepsy after prolonged febrile convulsions: excellent outcome after surgical treatment. Epilepsia 1993;34:878–883. Andermann F, Kobayashi E, Andermann E. Genetic Focal Epilepsies: State of the Art and Paths to the Future. Epilepsia 2005; 46 (Suppl. 10):61–67 Annegers JF, Hauser WA, Anderson VE, Kurland LT. The risks of seizure disorders among relatives of patients with childhood onset epilepsy.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- ë. ÜÂÎflÁÍÓ‚‡ ìåÅÄã ◊ÄÎÂÍ҇̉Ó‚Ò͇” äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ·ÛÎ. ◊ ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËfl 1431 ÚÂÎ. 02/ 92 30 388 e-mail: doc_sashka@abv.bg


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é·ÁÓ êÄççà äéÉçàíàÇçà çÄêìòÖçàü èêà ÅéãÖëííÄ çÄ èÄêäàçëéç å. èÂÚÓ‚‡, å. ê‡È˜Â‚‡, ã. í‡ÈÍÓ‚ ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËfl SUMMARY EARLY COGNITIVE CHANGES IN PARKINSON’S DISEASE M. Petrova, M. Raycheva, L. Traykov Parkinson’s disease (PD) is a slowly progressive neurodegenerative disorder mainly characterized by degeneration of dopaminergic neurons in the substantia nigra and the ventral tegmental area, in combination with a varying loss of central noradrenergic, cholinergic and serotonergic integrity, leading to a multitude of motor and non-motor disturbances (such as cognitive impairment and dementia). Studies describing cognitive dysfunction in PD tend to focus on the dementia, although it is important to establish the milder degrees of cognitive impairment, which also impact significantly on quality of life and increase significant the risk of dementia development. Cognitive impairments are often associated with PD, although deficits may be relatively subtle and not clinically apparent, or not overtly affect daily functioning. However, when compared to controls, subtle to prominent cognitive impairment is almost always found in PD patients. A wide variety of cognitive deficits has been described in nondemented PD patients, the most prominent of which is a deficit in executive function. Besides executive dysfunction, there is considerable evidence of deficits in visuospatial functions, constructional abilities, language and memory in nondemented PD. In this article, we review profoundly changes of different cognitive domains in non-demented PD in order to establish the specific profile of these early cognitive changes in PD. KEY WORDS: early cognitive changes, Parkinson’s disease. êÖáûåÖ ÅÓÎÂÒÚÚ‡ ̇ è‡ÍËÌÒÓÌ (Åè)  ·‡‚ÌÓ ÔÓ„ÂÒË‡˘Ó Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÍÓÂÚÓ Ò ı‡‡ÍÚÂËÁË‡ ÓÒÌÓ‚ÌÓ Ò ‰Â„ÂÌÂ‡ˆËfl ̇ ‰ÓÔ‡ÏËÌÂ„˘ÌËÚ Ì‚ÓÌË ‚ ÒÛ·Òڇ̈Ëfl ÌË„‡ Ë ‚ÂÌÚ‡Î̇ڇ Ú„ÏÂÌÚ‡Î̇ ӷ·ÒÚ. ÑÓÔ‡ÏËÌÂ„˘ÌËflÚ ‰ÂÙˈËÚ Ò ÍÓÏ·ËÌË‡ Ë Ò˙Ò Á‡„Û·‡ ̇ ÌÓ‡‰ÂÌÂ„˘ÌË, ıÓÎËÌÂ„˘ÌË Ë ÒÂÓÚÓÌËÌÂ„˘ÌË ÔÓÂ͈ËË, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÏÌÓÊÂÒÚ‚Ó ÏÓÚÓÌË Ë ÌÂÏÓÚÓÌË Ì‡Û¯ÂÌËfl (͇ÚÓ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ë ‰ÂÏÂ̈Ëfl). íẨÂ̈ËflÚ‡ ÔË ÔÓÛ˜‚‡ÌËflÚ‡, ÓÔËÒ‚‡˘Ë ÍÓ„ÌËÚË‚ÌËÚ ‰ÂÙˈËÚË ÔË Åè,  ‰‡ Ò ÙÓÍÛÒË‡Ú Ô‰ËÏÌÓ ‚˙ıÛ ‰ÂÏÂ̈ËflÚ‡. ë˙˘Ó ڇ͇ Ó·‡˜Â  ‚‡ÊÌÓ ÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ÔÓ-ÎÂÍËÚ ÒÚÂÔÂÌË Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ÔË Åè, ÍÓËÚÓ Ò˙˘Ó Ó͇Á‚‡Ú Á̇˜ËÏÓ ‚ÎËflÌË ‚˙ıÛ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ë Ôӂ˯‡‚‡Ú Á̇˜ËÚÂÎÌÓ ËÒ͇ ÓÚ ÔÓÒΉ‚‡˘‡ ‰ÂÏÂ̈Ëfl. äÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl Ò‡ ˜ÂÒÚÓ ‡ÒÓˆËË‡ÌË Ò Åè, ‚˙ÔÂÍË ˜Â ‰ÂÙˈËÚËÚ ÏÓ„‡Ú ‰‡ Ò‡ ÓÚÌÓÒËÚÂÎÌÓ ÎÂÍË, ‰‡ Ì ҇ ÍÎËÌ˘ÌÓ ËÁfl‚ÂÌË Ë ‰‡ Ì Á‡Òfl„‡Ú ÓÚ˜ÂÚÎË‚Ó ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ Âʉ̂ÌËÚ ÙÛÌ͈ËË Ì‡ Ô‡ˆËÂÌÚËÚÂ. èË ‚Ò ÚÓ‚‡ ÍÓ„‡ÚÓ Ò Ò‡‚Ìfl‚‡Ú Ò ÍÓÌÚÓÎÌË Îˈ‡, Ô‡ˆËÂÌÚËÚÂ Ò Åè ÔÓ͇Á‚‡Ú ÔÓ˜ÚË ‚Ë̇„Ë ÓÚ Â‰‚‡ ‰ÓÎÓ‚ËÏË ‰Ó ËÁ‡ÁÂÌË ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl. òËÓÍ ‰Ë‡Ô‡ÁÓÌ

ÓÚ ÍÓ„ÌËÚË‚ÌË ‰ÂÙˈËÚË Ò‡ ÓÔËÒ‡ÌË ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè, ͇ÚÓ Ì‡È-ËÁ‡ÁÂÌË ÓÚ Úflı Ò‡ ÚÂÁË ‚ ӷ·ÒÚÚ‡ ̇ ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË. ç‡‰ Ò Ì‡Û¯ÂÌËflÚ‡ ‚ ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË, ̇Îˈ ҇ Ò˙˘Ó Ë ÒÂËÓÁÌË ‰Ó͇Á‡ÚÂÎÒÚ‚‡ Á‡ ‰ÂÙˈËÚË ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÙÛÌ͈ËË, ÍÓÌÒÚÛÍÚË‚ÌËÚ ÛÏÂÌËfl, ÂÁË͇ Ë Ô‡ÏÂÚÚ‡ ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè. Ç Ì‡ÒÚÓfl˘‡Ú‡ ÒÚ‡ÚËfl ˘Â ̇Ô‡‚ËÏ Á‡‰˙ηӘÂÌ Ô„Ή ̇ ÔÓÏÂÌËÚ ‚ ÓÚ‰ÂÎÌËÚ ÍÓ„ÌËÚË‚ÌË Ó·Î‡ÒÚË ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè Ò Ó„Î‰ Ó˜ÂÚ‡‚‡ÌÂÚÓ Ì‡ ÒÔˆËÙ˘ÌËfl ÔÓÙËΠ̇ ÚÂÁË ‡ÌÌË ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ÔË Åè. äãûóéÇà Ñìåà: ‡ÌÌË ÍÓ„ÌËÚË‚Ì Ì‡Û¯ÂÌËfl, ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ. ÅÓÎÂÒÚÚ‡ ̇ è‡ÍËÌÒÓÌ (Åè)  ·‡‚ÌÓ ÔÓ„ÂÒË‡˘Ó Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÍÓÂÚÓ Ò ı‡‡ÍÚÂËÁË‡ ÓÒÌÓ‚ÌÓ Ò ‰Â„ÂÌÂ‡ˆËfl ̇ ‰ÓÔ‡ÏËÌÂ„˘ÌËÚ Ì‚ÓÌË ‚ ÒÛ·Òڇ̈Ëfl ÌË„‡ Ë ‚ÂÌÚ‡Î̇ڇ Ú„ÏÂÌÚ‡Î̇ ӷ·ÒÚ. ÑÓÔ‡ÏËÌÂ„˘ÌËflÚ ‰ÂÙˈËÚ Ò ÍÓÏ·ËÌË‡ Ë Ò˙Ò Á‡„Û·‡ ̇ ÌÓ‡‰ÂÌÂ„˘ÌË (locus coeruleus), ıÓÎËÌÂ„˘ÌË (nucleus basalis of Meynert) Ë ÒÂÓÚÓÌËÌÂ„˘ÌË (dorsal raphe nuclei) ÔÓÂ͈ËË (35), ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÏÌÓÊÂÒÚ‚Ó ÏÓÚÓÌË Ë ÌÂÏÓÚÓÌË Ì‡Û¯ÂÌËfl (͇ÚÓ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ë ‰ÂÏÂ̈Ëfl). èÓÛ˜‚‡ÌËflÚ‡, ÍÓËÚÓ ËÁÒΉ‚‡Ú ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË ÔË Åè ËÏ‡Ú ÚẨÂ̈Ëfl ‰‡ Ò ÙÓÍÛÒË‡Ú ‚˙ıÛ ‡Á‚ËÚËÂÚÓ Ì‡ ‰ÂÏÂ̈Ëfl, ‚˙ÔÂÍË ˜Â  ‚‡ÊÌÓ ‰‡ Ò ÛÒÚ‡ÌÓ‚flÚ ÔÓ-ÎÂÍËÚ ÒÚÂÔÂÌË Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl, ÍÓËÚÓ ÓÒ‚ÂÌ ˜Â Ôӂ˯‡‚‡Ú ËÒ͇ ÓÚ ÔÓÒΉ‚‡˘‡ ‰ÂÏÂ̈Ëfl (34), Ò˙˘Ó ‚ÎËflflÚ Á̇˜ËÚÂÎÌÓ ‚˙ıÛ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ (36). äÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl Ò‡ ˜ÂÒÚÓ ‡ÒÓˆËË‡ÌË Ò Åè, ‚˙ÔÂÍË ˜Â ‰ÂÙˈËÚËÚ ÏÓ„‡Ú ‰‡ Ò‡ ÓÚÌÓÒËÚÂÎÌÓ ÎÂÍË, ‰‡ Ì ҇ ÍÎËÌ˘ÌÓ ËÁfl‚ÂÌË Ë ‰‡ Ì Á‡Òfl„‡Ú ÓÚ˜ÂÚÎË‚Ó ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ Âʉ̂ÌËÚ ÙÛÌ͈ËË Ì‡ Ô‡ˆËÂÌÚËÚÂ. äÓ„‡ÚÓ Ò Ò‡‚Ìfl‚‡Ú Ò ÍÓÌÚÓÎÌË Îˈ‡, Ô‡ˆËÂÌÚËÚÂ Ò Åè ÔÓ͇Á‚‡Ú ˜ÂÒÚÓ ÓÚ Â‰‚‡ ‰ÓÎÓ‚ËÏ ‰Ó ËÁ‡ÁÂÌ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ (14). ç‡Û¯ÂÌËflÚ‡ ‚ ‡Á΢ÌËÚ ÍÓ„ÌËÚË‚ÌË Ó·Î‡ÒÚË Í‡ÚÓ ‚ÌËχÌËÂ, ÂÍÁÂÍÛÚË‚ÌË ÙÛÌ͈ËË, Ô‡ÏÂÚ, ˜ Ë ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌË ÛÏÂÌËfl, Ò‡ Ó·ÂÍÚ Ì‡ ‰ˈ‡ ÔÓÛ˜‚‡ÌËfl ÔË Ô‡ˆËÂÌÚË Ò Åè, ‰ÓË Ë ‚ ̇˜‡ÎÌËÚ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÌÓ ÂÁÛÎÚ‡ÚËÚ ˜ÂÒÚÓ Ò‡ ÔÓÚË‚ÓÂ˜Ë‚Ë (37, 38, 56, 26, 28, 44, 47). Ç Ì‡ÒÚÓfl˘‡Ú‡ ÒÚ‡ÚËfl ˘Â ·˙‰‡Ú ‡Á„Ή‡ÌË ÔÓÓÚ‰ÂÎÌÓ Ì‡Û¯ÂÌËflÚ‡ ‚ ÓÚ‰ÂÎÌËÚ ÍÓ„ÌËÚË‚ÌË ÒÙÂË ÔË Ì‰ÂÏÂÌÚÌËÚ ԇˆËÂÌÚË Ò Åè. 1. çÄêìòÖçàü çÄ ÇçàåÄçàÖíé îÂÌÓÏÂÌËÚÂ, Ò‚˙Á‡ÌË Ò ‚ÌËχÌËÂÚÓ, ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ‡Á‰ÂÎÂÌË Ì‡ ‰‚ „·‚ÌË „ÛÔË. è˙‚‡Ú‡ „ÛÔ‡ ÓÚ„Ó‚‡fl ̇ Ú. ̇. vigilance Ë Ò ËÁÒΉ‚‡ Ò ÚÂÒÚÓ‚Â, ‚Íβ˜‚‡˘Ë ËÁÏÂ‚‡Ì ̇ ‚ÂÏÂÚÓ, ÌÂÓ·ıÓ‰ËÏÓ Á‡ Ó·ËÍÌÓ‚ÂÌËÚ ‡͈ËË. ÇÚÓ‡Ú‡ Ô‰ÒÚ‡‚Îfl‚‡ ÒÂÎÂÍÚË‚ÌÓÚÓ ‚ÌËχÌËÂ, ÍÓÂÚÓ Ëχ ‰‚‡ ‡ÒÔÂÍÚ‡ – ̇ÒÓ˜ÂÌÓ Ë ‡ÁÔ‰ÂÎÂÌÓ ‚ÌËχÌË (52). ç‡ÒÓ˜ÂÌÓÚÓ ‚ÌËχÌË  ÓÚ„Ó‚ÓÌÓ Á‡ ËÁ·Ó‡ ̇ ‰ËÌ ËÁÚÓ˜ÌËÍ Ì‡ ËÌÙÓχˆËfl Ë Ë„ÌÓË‡ÌÂÚÓ Ì‡ ‰Û„ËÚÂ. ê‡ÁÔ‰ÂÎÂÌÓÚÓ ‚ÌËχÌË ÔÓχ„‡ Á‡ ÛÒÔ¯̇ڇ Ó·‡-


ÑÂÍÂÏ‚Ë 2009

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

·ÓÚ͇ ̇ ËÌÙÓχˆËflÚ‡, ˉ‚‡˘‡ ÓÚ Ôӂ˜ ÓÚ Â‰ËÌ ËÁÚÓ˜ÌËÍ. ë˙˘ÂÒÚ‚Û‚‡ Ë ÚÂÚË ÚËÔ – ÔÓ‰‰˙ʇ˘Ó ‚ÌËχÌË (ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ‰‡ Ò ‡„Ë‡ ‚ÒÂÍË Ô˙Ú Ì‡ fl‰ÍÓ ÒÎÛ˜‚‡˘Ë Ò Ò˙·ËÚËfl). ê‡ÁÔ‰ÂÎÂÌÓÚÓ ‚ÌËχÌËÂ Ë ‡·ÓÚ̇ڇ Ô‡ÏÂÚ Ì ·Ë Úfl·‚‡ÎÓ ‰‡ ·˙‰‡Ú ‚˙ÁÔËÂχÌË Í‡ÚÓ Â‰ËÌÌË ÒËÒÚÂÏË Ò ÙËÍÒË‡Ì ͇ԇˆËÚÂÚ. Ö‰ËÌ ÏÓ‰ÂÎ Á‡ ÍÓ̈ÂÔÚÛ‡ÎËÁ‡ˆËfl ̇ ‡·ÓÚ̇ڇ Ô‡ÏÂÚ Â ÚËÍÓÏÔÓÌÂÌÚ̇ڇ ÒËÒÚÂχ (9). ífl Ó·ı‚‡˘‡˘‡ ÙÓÌÓÎӄ˘ÌËfl Í˙„ Á‡ ÔÓ‰‰˙ʇÌ ̇ ‚Â·‡Î̇ڇ ËÌÙÓχˆËfl, (sketchpad) ÔÓ‰ÒËÒÚÂχڇ Á‡ ÔÓ‰‰˙ʇÌ ̇ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚Â̇ڇ ËÌÙÓχˆËfl (7) Ë ˆÂÌÚ‡Î̇ڇ ÂÍÁÂÍÛÚ˂̇ ÒËÒÚÂχ ̇ ‚ÌËχÌËÂÚÓ (central executive attentional system), ÍÓflÚÓ ÍÓÓ‰ËÌË‡ ‰‚ÂÚ ÔÓ‰‰˙ʇ˘Ë ÒËÒÚÂÏË ˜ÂÁ Ó·ÌÓ‚fl‚‡Ì ̇ ÒÍ·‰Ë‡˘Ëfl ·ÛÙÂ (storage buffer), ÍÓ„‡ÚÓ Â Ì‡Îˈ ÌÓ‚‡ Ò˙ÓÚ‚ÂÚÒÚ‚‡˘‡ ËÌÙÓχˆËfl (8, 50). ç‡ÒÍÓÓ ·Â¯Â ‰Ó·‡‚ÂÌ ˜ÂÚ‚˙ÚË ÍÓÏÔÓÌÂÌÚ, ÂÔËÁӉ˘ÂÌ ·ÛÙÂ, ÍÓÈÚÓ ËÌÚ„Ë‡ ËÌÙÓχˆËflÚ‡ ÓÚ ‡Á΢ÌË ÏÓ‰‡ÎÌÓÒÚË Ë ÓÒË„Ûfl‚‡ ‚˙Á͇ ÏÂÊ‰Û ÔÓ‰ÒËÒÚÂÏËÚÂ Ë ‰˙΄ÓÒӘ̇ڇ Ô‡ÏÂÚ (6, 8). èË Ô‡ˆËÂÌÚË Ò Åè, ËÁÔÓÎÁ‚‡ÈÍË ÚÓÁË ˜ÂÚËËÍÓÏÔÓÌÂÌÚÂÌ ÏÓ‰ÂΠ̇ ‡·ÓÚ̇ڇ Ô‡ÏÂÚ, Ò Ò˙Ó·˘‡‚‡ Á‡ ‰ۈË‡Ì ̇ ÂÙÂÍÚË‚ÌÓÒÚÚ‡ ̇ ˆÂÌÚ‡ÎÌËfl ÂÍÁÂÍÛÚË‚ÂÌ Ë ÂÔËÁӉ˘ÂÌ ·ÛÙÂ ÔË Ò˙ı‡ÌÂÌÓ ÙÛÌ͈ËÓÌË‡Ì ̇ ‚Â·‡ÎÌËfl Ë ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËfl ·ÛÙÂ (2). åÓÁ˙˜ÌË ÒÚÛÍÚÛË, ÍÓËÚÓ ‚ÁËÏ‡Ú Û˜‡ÒÚË ‚ ÔÓˆÂÒËÚ ̇ ‚ÌËχÌËÂ, Ò‡ ˜ÂÎÌËÚÂ Ë Ô‡ËÂÚ‡ÎÌËÚ ÍÓÓ‚Ë ÁÓÌË, ËÌÚ‡Î‡ÏË̇ÌËÚÂ Ë ÂÚËÍÛ·ÌË fl‰‡ ̇ ڇ·ÏÛÒ‡ Ë ÏÓÁ˙˜ÌËfl ÒÚ‚ÓÎ (‡ÒˆẨÂÌÚ̇ڇ ‡ÍÚË‚Ë‡˘‡ ÂÚËÍÛ·̇ ÙÓχˆËfl Ë ÔËÎÂʇ˘ËÚ fl‰‡) (46). ç‡Û¯ÂÌËflÚ‡ ‚˙‚ ‚ÌËχÌËÂÚÓ Ò‡ ˜ÂÒÚÓ Ò¢‡ÌË ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè ‚Íβ˜ËÚÂÎÌÓ Ë ‚ ̇È-‡ÌÌËÚ ÒÚ‡‰ËË Ì‡ ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ (41). Ç Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎÌË Á‰‡‚Ë Îˈ‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔÓ-„ÓÎÂÏË ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ë ÌÂÔÓÒÚÓflÌÒÚ‚Ó ‚ ‡͈ËÓÌÌËÚ ‚ÂÏÂ̇ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè (18, 19, 24, 48). 燷≇‚‡ÌÓ Â Ò˙˘Ó, ˜Â Ô‡ˆËÂÌÚËÚ ‚ ̇Ô‰̇ÎËÚ ÒÚ‡‰ËË Ì‡ Åè Ò‡ ÔÓ-·‡‚ÌË Ë Ò ÔÓ„ÓÎflχ ‚‡ˇ·ËÎÌÓÒÚ ‚ ËÁÔ˙ÎÌÂÌËflÚ‡ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚ ‚ ÎÂÍËÚ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡ (24). èË ËÁÒΉ‚‡Ì ̇ ÌÂÎÂÍÛ‚‡ÌË Ô‡ˆËÂÌÚË Ò Åè Ì Ò ÓÚÍË‚‡Ú ÍÓ·ˆËË ÏÂÊ‰Û ËÁÏÂÂÌËÚ ‡͈ËÓÌÌË ‚ÂÏÂ̇ Ë ÂÁÛÎÚ‡ÚËÚ ÓÚ ËÁÒΉ‚‡ÌËflÚ‡ ̇ „ÎÓ·‡ÎÌËÚ ÏÓÚÓÌË Ë ÍÓ„ÌËÚË‚ÌË ÙÛÌ͈ËË, Ô‰ÔÓ·„‡ÈÍË, ˜Â ÚÂÁË Ì‡Û¯ÂÌËfl ·Ëı‡ ÏÓ„ÎË ‰‡ Ò‡ ÓÚ‰ÂÎÂÌ Ï‡ÍÂ ÔË Åè (19). ÑÂÙˈËÚËÚ ‚˙‚ ‚ÌËχÌËÂÚÓ Ò‡ Ò‚˙Á‡ÌË ËÁ„ÎÂʉ‡ Ò Ì‡Û¯ÂÌËflÚ‡ ‚ ÔÓıӉ͇ڇ Ë ‡‚ÌÓ‚ÂÒËÂÚÓ. èË Óڂ΢‡Ì ̇ ‚ÌËχÌËÂÚÓ Ì‡ Åè Ô‡ˆËÂÌÚË Ò Ô‡‰‡ÌËfl  ÛÒÚ‡ÌÓ‚ÂÌÓ Á‡ÒË΂‡Ì ̇ ÔÓÒÚÛ‡Î̇ڇ ËÏ ÌÂÒÚ‡·ËÎÌÓÒÚ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚË ·ÂÁ Ô‡‰‡ÌËfl (3), ͇ÍÚÓ Ë Á‡ÒË΂‡Ì ̇ ÙËÈÁËÌ„ ÙÂÌÓÏÂ̇ Ë Á‡„Û·‡ ̇ ‡‚ÌÓ‚ÂÒË ÔË ıÓ‰ÂÌ (42). ë˙Ó·˘‡‚‡ ÒÂ, ˜Â Åè Ô‡ˆËÂÌÚËÚÂ Ò ËÁ‡ÁÂ̇ ÔÓÒÚÛ‡Î̇ ÌÂÒÚ‡·ËÎÌÓÒÚ Ë Ì‡Û¯ÂÌËfl ‚˙‚ ‚ÌËχÌËÂÚÓ Ò‡ Ò Ôӂ˯Â̇ ˜ÂÒÚÓÚ‡ ̇ Ô‡‰‡ÌËflÚ‡ (1). 2. çÄêìòÖçàü çÄ ÖäáÖäìíàÇçàíÖ îìçäñàà ÖÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË Ó·ı‚‡˘‡Ú Ò˙‚ÍÛÔÌÓ��Ú ÓÚ ÍÓ„ÌËÚË‚ÌË ÔÓˆÂÒË, ÌÂÓ·ıÓ‰ËÏË Á‡ ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ ÒΉÌËÚ Á‡‰‡˜Ë: ËÁ‡·ÓÚ‚‡Ì ̇ Ô·Ì, ‚ÁËχÌ ̇ ¯ÂÌËÂ, ÔˆÂÌ͇ Ë ‡‚ÚÓÔÂˆÂÔˆËfl (ËÎË feedback) (17, 57). èËÂχ ÒÂ, ˜Â ̇Û¯ÂÌËflÚ‡ ‚ ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË Ò‡ ‚ ÓÒÌÓ‚‡Ú‡ ̇ ÔÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ ÍÓ„ÌËÚË‚ÌËÚ ÔÓÏÂÌË ÔË Ô‡ˆËÂÌÚË Ò Åè. ç‰ÂÏÂÌÚÌËÚ ԇˆËÂÌÚË Ò Åè ËÁÔ˙ÎÌfl‚‡Ú ÔÓ-ÎÓ¯Ó Á‡‰‡˜Ë, ÍÓËÚÓ ËÁËÒÍ‚‡Ú „ÂÌÂË‡ÌÂÚÓ Ì‡ Ô‡ÏÂÚÓ‚ ÒÂÚ, ‡‰ÂÍ‚‡ÚÌÓ Ô·ÌË‡ÌÂ Ë ÍÓ„ÌËÚË‚ÌÓ ÔÓÒΉfl‚‡Ì (16, 26). Ö‰ËÌ ˜ÂÒÚÓ Ò˙Ó·˘‡‚‡Ì ‰ÂÙˈËÚ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ Â ‚ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ô‚Íβ˜‚‡ÌÂ

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ÓÚ Â‰ËÌ ÔÎ‡Ì Í˙Ï ‰Û„, Ú.Â. ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ Ò ÔÓÏÂÌfl Ôӂ‰ÂÌËÂÚÓ Ò˙Ó·‡ÁÌÓ ÔÓÏfl̇ڇ ‚ Ò˙ÓÚ‚ÂÚÌËfl ÒÚËÏÛÎ. é·‡˜Â Ô˘ËÌËÚ Á‡ ÚÂÁË Ì‡Û¯ÂÌËfl ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ Ì ҇ ̇Ô˙ÎÌÓ ËÁflÒÌÂÌË. Ö‰ÌË ‡‚ÚÓË Ì‡ÏË‡Ú, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò Åè ËÏ‡Ú Ì‡Û¯ÂÌË ‚ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ô‚Íβ˜‚‡ÌÂ, ÌÓ Ò‡ÏÓ ÍÓ„‡ÚÓ Ò‡ ÌÂÓ·ıÓ‰ËÏË ‚˙Ú¯ÌÓ „ÂÌÂË‡Ì ËÎË ‚˙Ú¯ÂÌ ÍÓÌÚÓÎ, Á‡„‡Ú‚‡ÈÍË Á‡‚ËÒËÏÓÒÚÚ‡ ÓÚ ÏÂı‡ÌËÁÏËÚ ̇ ‡·ÓÚ̇ڇ Ô‡ÏÂÚ (20, 54). ÑÛ„Ë ‡‚ÚÓË ÓÚ Ò‚Ófl ÒÚ‡Ì‡ ÔÓ͇Á‚‡Ú, ˜Â ‰ÂÙˈËÚËÚ ‚ Ô‚Íβ˜‚‡ÌÂÚÓ Ò˙˘Ó ÏÓ„‡Ú ‰‡ Ò ̇·Î˛‰‡‚‡Ú Ë ÔË ‚˙̯ÂÌ ÍÓÌÚÓΠ̇ Ô‚Íβ˜‚‡ÌÂÚÓ, ÍÓÂÚÓ ‰‡ÒÚ˘ÌÓ Ì‡Ï‡Îfl‚‡ ‚ÎËflÌËÂÚÓ Ì‡ ‡·ÓÚ̇ڇ Ô‡ÏÂÚ (21). í ÓÚÍË‚‡Ú Ò˙˘Ó ڇ͇, ˜Â ‰ÂÙˈËÚ˙Ú ‚ Ô‚Íβ˜‚‡ÌÂÚÓ ÔË Ô‡ˆËÂÌÚËÚ Ò ̇·Î˛‰‡‚‡ Ò‡ÏÓ ‚ ÚÂÁË ÔÓ‰Á‡‰‡˜Ë, ÔË ÍÓËÚÓ Ò ËÁËÒÍ‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ì‡Î˘ËÂÚÓ Ì‡ ÒÂÎÂÍÚË‚ÌË ÏÂı‡ÌËÁÏË, ÌÂÓ·ıÓ‰ËÏË Á‡ Ô‚Íβ˜‚‡ÌÂÚÓ ‚ ÛÒÎÓ‚ËflÚ‡ ̇ ‡ÁÒÂÈ‚‡ÌÂ. ê‰ˈ‡ ÔÓÛ˜‚‡ÌËfl Ò˙˘Ó Ò˙Ó·˘‡‚‡Ú Á‡ ËÁ‡ÁÂÌË Ì‡Û¯ÂÌËfl ‚ ÚÂÒÚÓ‚ÂÚÂ, ÓˆÂÌfl‚‡˘Ë ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ ËÌıË·Ë‡Ì ̇ ÛÚËÌÌËÚÂ Ë Ì‡‰ÂÍ‚‡ÚÌË Á‡ ÍÓÌÍÂÚ̇ ÒËÚÛ‡ˆËfl ‡ÁÒÂÈ‚‡˘Ë ÒÚËÏÛ·ˆËË Ë Ò˙ÓÚ‚ÂÚÌËÚ ‡‚ÚÓχÚËÁË‡ÌË ÓÚ„Ó‚ÓË ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè (60, 61). çflÍÓË ‡‚ÚÓË Ò˙Ó·˘‡‚‡Ú Ò˙˘Ó, ˜Â ‰ÂÙˈËÚËÚ ‚ ÚÂÁË ÚÂÒÚÓ‚Â Ò‡ Ó·‚˙Á‡ÌË Ò ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ÔÓÒΉ‚‡˘ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ Ë ‰ÂÏÂ̈Ëfl (40, 62) ç‡Û¯ÂÌËflÚ‡ ‚˙‚ ‚Â·‡Î̇ڇ ÙÎÛˉÌÓÒÚ, ÍÓËÚÓ ÓÚ˜‡ÒÚË ÓÚ‡Áfl‚‡Ú ‰ÂÙˈËÚËÚ ‚ ÂÍÁÂÍÛÚË‚ÌÓÚÓ Ú˙ÒÂÌÂ Ë ÔÓˆÂÒËÚ ̇ ËÁ‚΢‡ÌÂ, Ò‡ Ò˙˘Ó ‰Ó· ÓÔËÒ‡ÌË ÔË Åè. èÓ-ÒÍÓÓ ÎËÚÂ‡Î̇ڇ ÓÚÍÓÎÍÓÚÓ ÒÂχÌÚ˘̇ڇ ÙÎÛˉÌÓÒÚ Â ÚËÔ˘ÌÓ Ì‡Û¯Â̇ ÔË Åè (5, 21, 27, 40), χ͇ Ë ÚÂÁË ‰‡ÌÌË ‰‡ Ì ҇ ÒËÒÚÂÏÌÓ Ì‡·Î˛‰‡‚‡ÌË (30, 43). ç‡Îˈ  ËÁ‚ÂÒÚÌÓ ÔËÔÓÍË‚‡Ì ÓÚ „Ή̇ ÚӘ͇ ̇ ÔÓ‰ÎÂʇ˘‡Ú‡ Ì‚Ó·ËÓÎӄ˘̇ ·‡Á‡ ̇ ‰ÂÙˈËÚËÚ ‚ ÎËÚÂ‡Î̇ Ë ÒÂχÌÚ˘̇ ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ, ËχÈÍË Ô‰‚ˉ, ˜Â Ë ‰‚‡Ú‡ ÚËÔ‡ ÙÎÛˉÌÓÒÚË ‡Á˜ËÚ‡Ú Ì‡ ÙÓÌÚ‡ÎÌÓ ·‡ÁË‡ÌË ÂÍÁÂÍÛÚË‚ÌË ÒÚ‡Ú„ËË. 3. çÄêìòÖçàü çÄ èÄåÖííÄ Ö‰ÌÓ ÓÚ Ì‡È-ÒÚ‡ËÚ ‡Á‰ÂÎÂÌËfl ̇ Ô‡ÏÂÚÚ‡  ÚÓ‚‡ ̇ Ô˙‚˘̇ ËÎË Í‡ÚÍÓÒӘ̇ Ô‡ÏÂÚ (äëè) Ë ‚ÚÓ˘̇ ËÎË ‰˙΄ÓÒӘ̇ Ô‡ÏÂÚ (Ñëè). äëè  ÒËÒÚÂχ Ò˙Ò Ò‡ÏÓÒÚÓflÚÂÎÂÌ ‡Ì‡ÚÓÏ˘ÂÌ ÒÛ·ÒÚ‡Ú, ÍÓflÚÓ Ò˙Á̇ÚÂÎÌÓ Ò˙ı‡Ìfl‚‡ ËÌÙÓχˆËflÚ‡ Á‡ Ó„‡Ì˘ÂÌÓ ‚ÂÏ (9). Ñëè Ô‰ÒÚ‡‚Îfl‚‡ ÒËÒÚÂχ ÓÚ ÔÓˆÂÒË, ÔÓÁ‚ÓÎfl‚‡˘Ë ÔÓ-‰˙΄ÓÚ‡ÈÌÓÚÓ ÒÍ·‰Ë‡Ì ̇ ËÌÙÓχˆËfl, ͇ÍÚÓ Ë ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ Ô‰˯ÌË Á̇ÌËfl (58). Ö‰ÌÓ ÓÚ Ô˙‚ËÚ ÙÛ̉‡ÏÂÌÚ‡ÎÌË ‡Á‰ÂÎÂÌËfl ̇ Ñëè ÔÓÚË‚ÓÔÓÒÚ‡‚fl ÂÔËÁӉ˘̇ڇ ̇ ÒÂχÌÚ˘̇ Ô‡ÏÂÚ. ÖÔËÁӉ˘̇ڇ Ô‡ÏÂÚ Í‡Ò‡Â Ô‡ÏÂÚÚ‡ Á‡ Ò˙·ËÚËflÚ‡, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÒËÚÛË‡ÌË ‚ ÒÚÓ„Ó ÓÔ‰ÂÎÂÌ ÔÓÒÚ‡ÌÒÚ‚ÂÌ Ë ‚ÂÏÂ‚Ë ÍÓÌÚÂÍÒÚ. ëÂχÌÚ˘̇ڇ Ô‡ÏÂÚ Â Ô‡ÏÂÚÚ‡ Á‡ Ù‡ÍÚËÚÂ, Ô‡‚Ë·ڇ Ë ÍÓ̈ÂÔˆËËÚÂ. Ç ÎËÚÂ‡ÚÛ‡Ú‡ Ò Ò˙Ó·˘‡‚‡Ú ‰ˈ‡ ‰‡ÌÌË, ˜Â ̇Û¯ÂÌËflÚ‡ ‚ ÂÔËÁӉ˘̇ڇ Ô‡ÏÂÚ, ‚Íβ˜‚‡˘Ë ‰ÂÙˈËÚË ‚ ËÁ‚΢‡ÌÂÚÓ, Ò‡ ÚÓÎÍÓ‚‡ ˜ÂÒÚË, ÍÓÎÍÓÚÓ Ë ÚÂÁË ‚˙‚ ‚ÌËχÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË Ó˘Â ‚ ̇˜‡ÎÌËfl ÒÚ‡‰ËÈ Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ÔË Ô‡ˆËÂÌÚË Ò Åè (41). èË Ì‰ÂÏÂÌÚÌËÚ ԇˆËÂÌÚË Ó·Ë˜‡ÈÌÓ Ò ̇·Î˛‰‡‚‡ ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-Ò··Ó Á‡Òfl„‡Ì ̇ Á‡Û˜‡‚‡ÌÂÚÓ Ì‡ ÌÓ‚‡ ËÌÙÓχˆËfl Ë ‡ÁÔÓÁ̇‚‡ÌÂÚÓ Ì‡ ‚˜ Á‡Û˜Â̇ ڇ͇‚‡, ÍÓÂÚÓ ÔÓ‰Ò͇Á‚‡ ‰ÂÙˈËÚ ‚ „ÂÌÂË‡ÌÂÚÓ Ì‡ ÍÓ‰Ë‡˘Ë Ë ËÁ‚΢‡˘Ë ÒÚ‡Ú„ËË Ò ÓÚÌÓÒËÚÂÎÌÓ Á‡Ô‡Á‚‡Ì ̇ ÒÍ·‰Ë‡Ì‡Ú‡ ËÌÙÓχˆËfl, ı‡‡ÍÚÂÌÓ Á‡ ÔÓ‰ÍÓÓ‚Ëfl ÚËÔ Ô‡ÏÂÚÓ‚Ë Ì‡Û¯ÂÌËfl (54, 55). ç‡Û¯ÂÌËfl ‚˙‚ ‚Â·‡Î̇ڇ ÙÎÛˉÌÓÒÚ, ÍÓËÚÓ Ò‡ Ò˙˘Ó ·Â΄ Á‡ ÒÂχÌÚ˘ÌÓ Á‡Òfl„‡Ì ҇ ‰Ó· ÓÔËÒ‡ÌË ÔË Åè (30) Ë ‰ˈ‡ ‡‚ÚÓË Ë‰ÂÌ-


ÑÂÍÂÏ‚Ë 2009

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

ÚËÙˈË‡Ú ‰ÂÙˈËÚËÚ ‚˙‚ ‚Â·‡Î̇ڇ ÙÎÛˉÌÓÒÚ Í‡ÚÓ Ô‰ËÍÚÓË Á‡ ÔÓÒΉ‚‡˘‡ ÔÓfl‚‡ ̇ ‰ÂÏÂ̈Ëfl (32, 39, 40). ç‡ÒÍÓÓ Ôӂ‰ÂÌ ÏÂÚ‡-‡Ì‡ÎËÁ ‰ÂÏÓÌÒÚË‡, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò Åè ÔÓ͇Á‚‡Ú ÏÌÓ„Ó ÔÓ-„ÓÎÂÏË Ì‡Û¯ÂÌËfl ‚ ÚÂÒÚÓ‚ÂÚ Á‡ ÒÂχÌÚ˘̇, ÓÚÍÓÎÍÓÚÓ Á‡ ÎËÚÂ‡Î̇ ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ, ÍÓÂÚÓ Ô‰ÔÓ·„‡, ˜Â Ô‡ÚÓÎÓ„ËflÚ‡ ‚ ÚÂÏÔÓ‡ÎÌËfl ÎÓ· ÏÓÊ ‰‡ ‰ÓÔË̇Òfl Á‡ ̇·Î˛‰‡‚‡ÌËÚ ̇Û¯ÂÌËfl ‚˙‚ ÙÎÛˉÌÓÒÚÚ‡ (30). Ç ÔÓ‰ÍÂÔ‡ ̇ ‰‡ÌÌËÚ Á‡ ‡ÌÌË Ô‡ÏÂÚÓ‚Ë ‰ÂÙˈËÚË ÔË Åè, Ò‡ ÂÁÛÎÚ‡ÚËÚ ÓÚ Ôӂ‰ÂÌË ÔÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë Ë Ì‚ÓÔ‡ÚÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl. ë˙Ó·˘‡‚‡ Ò Á‡ ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ıÓÎËÌÂ„˘ÌË ‰ÂÙˈËÚË ÔË Åè (12), ͇ÍÚÓ Ë Á‡ ̇΢ËÂÚÓ Ì‡ ÛÒÚ‡ÌÓ‚ÂÌË Ô‡ÚÓÎӄ˘ÌË ÔÓÏÂÌË ‚˙‚ ÙÓÌÚ‡Î̇ڇ ·‡Á‡Î̇ ÍÓ‡ Ë ‚ ıËÔÓ͇ÏÔ‡Î̇ڇ ÙÓχˆËfl ÓÚÌÓÒËÚÂÎÌÓ ‡ÌÓ ‚ ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (15). 4. çÄêìòÖçàü çÄ êÖóíÄ à ÖáàäÄ èӂ˜ÂÚÓ ÓÚ ‡‚ÚÓËÚ ҇ ‰ËÌÓ‰Û¯ÌË, ˜Â ̇Û¯ÂÌËflÚ‡ ‚ ˜ڇ Ì ҇ ÓÒÓ·ÂÌÓ ı‡‡ÍÚÂÌË Á‡ Ô‰ÂÏÂÌÚÌËfl ÒÚ‡‰ËÈ Ì‡ Åè (11, 23, 29). èË ‚Ò ÚÓ‚‡ Ó·‡˜Â ̇Û¯ÂÌËfl ‚ ÒÂχÌÚ˘̇ڇ ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ (4), ÙÎÛˉÌÓÒÚÚ‡ Á‡ ̇ÁÓ‚‡‚‡Ì ̇ „·„ÓÎË (45) Ë ‡ÎÚÂ̇Ú˂̇ڇ ÙÎÛˉÌÓÒÚ (22, 25) Ò‡ Ò˙Ó·˘‡‚‡ÌË ÔË Ô‡ˆËÂÌÚË Ò Åè ‚ ÌflÍÓË ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡. Lee and Smith (37) ̇ÏË‡Ú, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò ‡Ì̇ Åè ‰‡‚‡Ú ̇ Á‡‰‡˜‡Ú‡ Á‡ ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ ÔÓ-„ÓÎflÏ ·ÓÈ ÔÓ‚Ú‡fl˘Ë Ò ËÌÚÛÁËË (ËÁÎ˯ÌË ‰ÛÏË) ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎ̇ „ÛÔ‡ Á‰‡‚Ë Îˈ‡. Ä‚ÚÓËÚ Ô‰ÔÓ·„‡Ú, ˜Â ÚÂÁË ÂÁÛÎÚ‡ÚË Ò ‰˙ÎÊ‡Ú Ì‡ ÚÛ‰ÌÓÒÚËÚ ̇ Ô‡ˆËÂÌÚËÚÂ Ò Åè ‚ Ô‚Íβ˜‚‡ÌÂÚÓ ÏÂÊ‰Û Í‡Ú„ÓËËÚ ÔË Ì‡Î˘Ë ̇ ‚ÂÏÂ‚Ó Ó„‡Ì˘ÂÌËÂ. éÚ Ò‚Ófl ÒÚ‡Ì‡ Cooper Ë Ò˙Ú. (22), ËÁÔÓÎÁ‚‡ÈÍË ÚÂÒÚÓ‚Â Á‡ ̇ÁÓ‚‡‚‡ÌÂ, ‡Á·Ë‡ÌÂ, ÂÁËÍÓ‚‡ ÂÍÒÔÂÒËfl Ë ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ (ÒÂχÌÚ˘̇ Ë ‡ÎÚÂ̇Ú˂̇), ÛÒÚ‡ÌÓ‚fl‚‡Ú ̇΢ËÂÚÓ Ò‡ÏÓ Ì‡ ÎÂÍË Ì‡Û¯ÂÌËfl ‚ ÂÁËÍÓ‚‡Ú‡ ÂÍÒÔÂÒËfl Ë ‚ ÌflÍÓË ÓÚ Á‡‰‡˜ËÚ Á‡ ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ ÔË ËÁÒΉ‚‡ÌËÚ ÓÚ Úflı ̉ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè. Cummings Ë Ò˙Ú. (23) Ò‡‚Ìfl‚‡Ú „Ó‚ÓÌËÚÂ Ë ÂÁËÍÓ‚Ë Ì‡Û¯ÂÌËfl ÔË Ô‡ˆËÂÌÚË Ò Åè Ò Ë ·ÂÁ ‰ÂÏÂ̈Ëfl ˜ÂÁ ÔÓ‰ӷ̇ ·‡ÚÂËfl ÓÚ 37 ÔÓ‰Ò͇ÎË Á‡ ÓˆÂÌ͇. 臈ËÂÌÚËÚÂ Ò Åè ·ÂÁ ‰ÂÏÂ̈Ëfl Ò‡ ÔÓ͇Á‚‡ÎË Ì‡Ï‡ÎÂÌË ̇ ËÌÙÓχˆËÓÌÌÓÚÓ Ò˙‰˙ʇÌË ̇ ÒÔÓÌÚ‡Ì̇ڇ ˜, ͇ÍÚÓ Ë ‚ ‡Á·Ë‡ÌÂÚÓ Ì‡ ÍÓÏÔÎÂÍÒÌË ÍÓχ̉Ë, ÌÓ Ì‡Û¯ÂÌËflÚ‡ Ì ҇ ·ËÎË ÚÓÎÍÓ‚‡ ÔÓ‰˜ÂÚ‡ÌË, ÍÓÎÍÓÚÓ ÚÂÁË ‚ ˜ڇ Ë ÏÂı‡ÌË͇ڇ ̇ ÔËÒ‡ÌÂ. éÚ ‰Û„‡ ÒÚ‡Ì‡ Ú‡ÁË „ÛÔ‡ Ô‡ˆËÂÌÚË Ì ҇ ÔÓ͇Á‡ÎË Ì‡Û¯ÂÌËfl ‚ ‡Á·Ë‡ÌÂÚÓ, ÔÓ‚ÚÓÂÌËÂÚÓ, ̇ÁÓ‚‡‚‡ÌÂÚÓ, ˜ÂÚÂÌÂÚÓ ËÎË ‡‚ÚÓχÚËÁË‡Ì‡Ú‡ ˜. ÑÛ„Ë ‡‚ÚÓË, ËÁÒΉ‚‡˘Ë ÂÁËÍÓ‚ËÚ ÙÛÌ͈ËË ÔË Ô‡ˆËÂÌÚË Ò Åè ·ÂÁ ‰ÂÏÂ̈Ëfl, Ò˙Ó·˘‡‚‡Ú Á‡ ‰ÂÙˈËÚË ‚ ̇ÏË‡ÌÂÚÓ Ì‡ ‰ÛÏË ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè ‚ Á‡‰‡˜ËÚ Á‡ ÁËÚÂÎÌÓ ÍÓÌÙÓÌÚ‡ˆËÓÌÌÓ Ì‡ÁÓ‚‡‚‡ÌÂ, ÍÓÂÚÓ Ó·‡˜Â Ò ÛÎÂÒÌfl‚‡ ˜ÂÁ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÂχÌÚ˘ÌÓ ËÎË ÙÓÌÂÚ˘ÌÓ ÔÓ‰ÔÓχ„‡Ì (43). íÂÁË ‡‚ÚÓË Ò˙˘Ó ̇·Î˛‰‡‚‡Ú ‰ËÒӈˇˆËfl ÏÂÊ‰Û Ì‡Û¯ÂÌËflÚ‡ ‚ ÎËÚÂ‡Î̇ Ë ÒÂχÌÚ˘̇ ‚Â·‡Î̇ ÙÎÛˉÌÓÒÚ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Ì‡Û¯ÂÌËfl ‚ ÒÂχÌÚ˘̇ڇ (ËÁ‚΢‡Ì ̇ ‰ÛÏË ÓÚ ÓÔ‰ÂÎÂ̇ ÒÂχÌÚ˘̇ ͇Ú„ÓËfl), ÌÓ ÌÂ Ë ‚ ÎËÚÂ‡Î̇ڇ ‚Â·‡Î̇ ÙÎÛÂÌÚÌÓÒÚ (̇ÁÓ‚‡‚‡Ì ̇ ‰ÛÏË, Á‡ÔÓ˜‚‡˘Ë Ò ÓÔ‰ÂÎÂ̇ ·ÛÍ‚‡). 5. áêàíÖãçé-èêéëíêÄçëíÇÖçà à äéçëíêìäíàÇçà çÄêìòÖçà ê‰ˈ‡ ‡‚ÚÓË Ò˙Ó·˘‡‚‡Ú Á‡ ̇΢ˠ̇ ‰ÂÙˈËÚË ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÙÛÌ͈ËË ÔË Ì‰ÂÏÂÌÚÌË

175

Ô‡ˆËÂÌÚË Ò Åè, ‰ÓË ÍÓ„‡ÚÓ ËÁÔÓÎÁ‚‡ÌËÚ ÚÂÒÚÓ‚Â ËÏ‡Ú Ï‡ÎÍÓ ÏÓÚÓÌË ÍÓÏÔÓÌÂÌÚË (13, 31). ê‡ÌÌËÚ ̇Û¯ÂÌËfl ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚Â̇ڇ Ó„‡ÌËÁ‡ˆËfl ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè Ò Ò˙Ó·˘‡‚‡Ú ÓÚ ÌflÍÓË ‡‚ÚÓË Í‡ÚÓ ‚˙ÁÏÓÊÌË Ô‰ËÍÚÓË Ì‡ ÔÓ-Í˙ÒÌÓÚÓ ‡Á‚ËÚË ̇ ‰ÂÏÂ̈Ëfl (33, 39). Clock Drawing Test (CDT)  ‰ËÌ ÓÚ ˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚ ÚÂÒÚÓ‚Â Á‡ ÓˆÂÌ͇ ̇ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÒÔÓÒÓ·ÌÓÒÚË Ì‡ ÚÂÁË Ô‡ˆËÂÌÚË, ÌÓ ‰Ó͇ÚÓ Â‰ÌË ‡‚ÚÓË Ò˙Ó·˘‡‚‡Ú Á‡ Á̇˜ËÏË Ì‡Û¯ÂÌËfl ÔË Ì‰ÂÏÂÌÚÌËÚ ԇˆËÂÌÚË Ò Åè (51), ÚÓ ‰Û„Ë Ì ÓÚÍË‚‡Ú ÔÓ‰Ó·ÌË ‰ÂÙˈËÚË (44). Clock Drawing Test, Ô˙‚Ó̇˜‡ÎÌÓ Á‡ÏËÒÎÂÌ ‰‡ ‚ÂËÙˈË‡ ÓÔ‰ÂÎÂÌË ÍÓÚË͇ÎÌË ÙÛÌ͈ËË ÔË Ô‡ˆËÂÌÚËÚÂ Ò ·ÓÎÂÒÚ Ì‡ ÄΈı‡ÈÏÂ (53), ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ë‰ÂÌÚËÙˈË‡ ÌflÍÓÎÍÓ Ô‡ÏÂÚÓ‚Ë ÔÓˆÂÒ‡, Ò‚˙Á‡ÌË Ò ÔÂÙÓÌÚ‡ÎÌËÚÂ Ë ÒÛ·ÍÓÚË͇ÎÌËÚ ÏÂı‡ÌËÁÏË. íÂÁË ÔÓˆÂÒË ‚Íβ˜‚‡Ú Ôӂ‰Â̘ÂÒ͇ڇ Ó„‡ÌËÁ‡ˆËfl, Ô‡ÏÂÚÓ‚‡Ú‡ ÙÎÂÍÒË·ËÎÌÓÒÚ, ÁËÚÂÎÌÓ-ÍÓÌÒÚÛÍÚË‚ÌÓÚÓ ËÁÔ˙ÎÌÂÌËÂ, ÍÓ„ÌËÚË‚ÌÓÚÓ ÔÓ„‡ÏË‡Ì ̇ ÔÒËıÓÏÓÚÓ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ë ËÌÚ„‡ˆËflÚ‡ ̇ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÙÛÌ͈ËË (10, 49, 59). Ç Á‡Íβ˜ÂÌË ¯ËÓÍ ‰Ë‡Ô‡ÁÓÌ ÓÚ ÍÓ„ÌËÚË‚ÌË ‰ÂÙˈËÚË Ò‡ ÓÔËÒ‡ÌË ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè, ͇ÚÓ Ì‡ÈËÁ‡ÁÂÌË ÓÚ Úflı Ò‡ ÚÂÁË ‚ ӷ·ÒÚÚ‡ ̇ ‚ÌËχÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË. ç‡‰ Ò Ì‡Û¯ÂÌËflÚ‡ ‚˙‚ ‚ÌËχÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË, ̇Îˈ ҇ Ò˙˘Ó Ë ÒÂËÓÁÌË ‰Ó͇Á‡ÚÂÎÒÚ‚��� Á‡ ‰ÂÙˈËÚË ‚ Ô‡ÏÂÚÚ‡, ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÙÛÌ͈ËË, ÍÓÌÒÚÛÍÚË‚ÌËÚ ÛÏÂÌËfl Ë ˜ڇ ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- å. èÂÚÓ‚‡, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ·ÛÎ. “ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËfl 1431 ÚÂÎ: 02/92 30 544 Â-mail: dr.mpetrova@mail.bg


ÑÂÍÂÏ‚Ë 2009

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

177

éË„Ë̇ÎÌË ÒÚ‡ÚËË ÖîàäÄëçéëí à èéçéëàåéëí çÄ LEVETIRACETAM èêà ãÖóÖçàÖ çÄ èÄñàÖçíà ë ÖèàãÖèëàü á. á‡ı‡Ë‚, Ö. ÇËÚ‚‡ å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ - èÎÓ‚‰Ë‚, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl SUMMARY EFFICACY AND TOLERABILITY OF LEVETIRACETAM IN PATIENTS WITH EPILEPSY Z. Zahariev, E. Viteva AIMS: To evaluate long-term effectiveness of Levetiracetam (LEV) as polytherapy in patients with epilepsy – refractory or not. PATIENTS AND METHODS: A prospective study of 65 patients (36,92% men and 63,08% women) of median age 32,68±14,09 years and with median disease duration 18,37±11,62 years for a period of 1 year. 71,43% of them were with partial, 28,57% - with generalized epilepsy, 20% had idiopathic, 55% - symptomatic and 25% - cryptogenic epilepsy. The mean frequency of seizures before starting LEV was 4,1±6,4 per week for the partial seizures and 3,7±9,4 for the generalized ones. According to the applied dose of LEV, 4 groups were made out: 1000 mg/d, 1500 mg/d, 2000 mg/d and 3000 mg/d. LEV was given as polytherapy most often in combination with Valproate – in 16,15% and VPA+OCBZ – in 13,85%. The treatment was terminated in 3 patients due to adverse events or increase in seizure frequency. RESULTS: Reduction of seizure frequency _>50% was recorded in 57,89% of cases. The most frequently used dosage was 1500 mg/d – in 52,31%, the most effective - 1000mg/d – it resulted in seizure reduction _>50% in all patients. There was a minimal difference of the total effectiveness of the dosages 1500 and 2000 mg/d – the reduction of seizure frequency _>50% was in 53,33% and 52,63% of cases respectively. In 18,42% there was no change in the frequency of clinical manifestations, while in 15,79% there was worsening. The clinical effect was most marked in the 6-th month for the partial seizures, when the mean month frequency showed more than double frequency reduction and at the end of the first year for the generalized seizures – reduction of 59%. It was proven the most rapid and highest reduction for partial seizures and a gradual frequency reduction for generalized seizures. At the study end 2 of 4 patients with myoclonic seizures were with seizure frequency reduction higher than 50%. CONCLUSION: LEV is highly effective as polytherapy in patients with partial and generalized epilepsies with all types of etiology. KEY WORDS: Levetiraceram, effectiveness, side effects, polytherapy. êÖáûåÖ ñÖãà: ч Ò ӈÂÌË ‰˙΄ÓÒӘ̇ڇ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ Levetiracetam (LÖV) ‚ ÔÓÎËÚÂ‡ÔËfl ÔË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl – ÂÙ‡ÍÚÂ̇ ËÎË ÌÂ. èÄñàÖçíà à åÖíéÑà: èÓÒÔÂÍÚË‚ÌÓ ÔÓÛ˜‚‡Ì ̇ 65 Ô‡ˆËÂÌÚ‡ (36,92% Ï˙ÊÂ Ë 63,08% ÊÂÌË) ̇ Ò‰̇ ‚˙Á‡ÒÚ 32,68 ± 14,09 „., Ò˙Ò Ò‰̇ ‰‡‚ÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ 18,37 ± 11,62 „. Á‡ ÔÂËÓ‰ ÓÚ 1 „Ó‰Ë̇. éÚ Úflı 71,43% Ò‡ Ò Ô‡ˆË‡Î̇, 28,57% - Ò „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËfl, 20% - Ò Ë‰ËÓÔ‡Ú˘̇, 55% - Ò˙Ò ÒËÏÔÚÓχÚ˘̇ Ë 25% - Ò ÍËÔÚÓ„ÂÌ̇ ÂÔËÎÂÔÒËfl. éÚ˜ÂÚÂ̇  Ò‰̇ ˜ÂÒÚÓÚ‡ ̇ ÔËÒÚ˙ÔËÚ ÔÂ‰Ë ‚Íβ˜‚‡Ì ̇ LEV 4,1 ± 6,4 Ò‰Ï˘ÌÓ Á‡ Ô‡ˆË‡ÎÌËÚÂ Ë 3,7 ± 9,4 ÏÂÒ˜ÌÓ Á‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ.

éÙÓÏÂÌË Ò‡ 4 „ÛÔË ÒÔÓ‰ ÔË·„‡Ì‡Ú‡ ‰ÓÁ‡ LEV: 1000 mg/d, 1500 mg/d, 2000 mg/d Ë 3000 mg/d. èÓ‚Âʉ‡Ì‡  ÔÓÎËÚÂ‡ÔËfl ̇È-˜ÂÒÚÓ Ò Valproate – ÔË 16,15% Ë VPA + OCBZ – ÔË 13,85%. èË 3-χ ÓÚ ·ÓÎÌËڠΘÂÌËÂÚÓ Ò LEV e ÔÂÍ‡ÚÂÌÓ ÔÓ‡‰Ë ÓÚ˜ÂÚÂÌËÚ ÌÂÊ·ÌË ÒÚ‡Ì˘ÌË fl‚ÎÂÌËfl ËÎË ‚ÎÓ¯‡‚‡Ì ̇ ÍÎËÌ˘ÌËÚ ÔÓfl‚Ë. êÖáìãíÄíà: éÚ˜ÂÚ Ò ӷ˘‡ ‰Û͈Ëfl ̇ ÔËÒÚ˙ÔËÚ _>50% ÔË 57,89% ÓÚ ÒÎÛ˜‡ËÚÂ. ç‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡Ì‡Ú‡ ‰ÓÁ‡  1500mg/d – ÔË 52,31%, ‡ ̇È-ÂÙÂÍÚ˂̇ �� 1000mg/d – „ËÒÚË‡ Ò ÔËÒÚ˙Ô̇ ‰Û͈Ëfl _>50% - ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â ÔË·„‡Ì‡. åËÌËχÎ̇  ‡ÁÎË͇ڇ Á‡ Ó·˘‡Ú‡ Í‡È̇ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ ‰ÓÁËÚ 1500 Ë 2000 mg/d - ‰Û͈Ëfl ̇ ÔËÒÚ˙ÔËÚ _>50% Ò˙ÓÚ‚ÂÚÌÓ ÔË 53,33% Ë 52,63%. èË 18,42% ÓÚ Ô‡ˆËÂÌÚËÚ Ì Ò ̇·Î˛‰‡‚‡ ÔÓÏfl̇ ‚ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÍÎËÌ˘ÌËÚ ÔÓfl‚Ë, ‡ ÔË 15,79% Ò ÓÚ˜ËÚ‡ ‚ÎÓ¯‡‚‡ÌÂ. äÎËÌ˘ÌËflÚ ÂÙÂÍÚ Â Ì‡ÈËÁ‡ÁÂÌ Ì‡ ¯ÂÒÚËfl ÏÂÒˆ Á‡ Ô‡ˆËÂÌÚËÚÂ Ò Ô‡ˆË‡ÎÌËÚ ÔËÒÚ˙ÔË, ÍÓ„‡ÚÓ Ò‰̇ڇ ÏÂÒ˜̇ ˜ÂÒÚÓÚ‡ Â Ò ‰Û͈Ëfl Ôӂ˜ ÓÚ 2 Ô˙ÚË Ë ‚ Í‡fl ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇ Á‡ ÚÂÁË Ò „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË - ‰Û͈Ëfl Ò 59%. ÑÓ͇Á‡Ì‡  ̇È-·˙Á‡ Ë ‚ËÒÓ͇ Í‡È̇ ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ÔË Ô‡ˆË‡ÎÌËÚ ÔËÒÚ˙ÔË Ë ÔÓ-ÔÓÒÚÂÔÂÌÌÓ Ì‡‡ÒÚ‚‡˘‡ ‰Û͈Ëfl ÔË „ÂÌÂ‡ÎËÁË‡ÌËÚ ÔËÒÚ˙ÔË. éÚ 4Ú ԇˆËÂÌÚ‡ Ò ÏËÓÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË 2-χ Ò‡ Ò ÔËÒÚ˙Ô̇ ‰Û͈Ëfl >50% ‚ Í‡fl ̇ ÔÓÛ˜‚‡ÌÂÚÓ. áÄäãûóÖçàÖ: LEV  ‚ËÒÓÍÓ eÙÂÍÚË‚ÂÌ Ï‰Ë͇ÏÂÌÚ ‚ ÔÓÎËÚÂ‡ÔËfl ÔË Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡ÎÌË Ë „ÂÌÂ‡ÎËÁË‡ÌË ÂÔËÎÂÔÒËË, ÌÂÁ‡‚ËÒËÏÓ ÓÚ Úflı̇ڇ ÂÚËÓÎÓ„Ëfl. äãûóéÇà Ñìåà: Levetiracetam, ÂÙÂÍÚË‚ÌÓÒÚ, ÒÚ‡Ì˘ÌË ÂÙÂÍÚË, ÔÓÎËÚÂ‡ÔËfl. ÇöÇÖÑÖçàÖ Levetiracetam (Keppra) ÔË̇‰ÎÂÊË Í˙Ï „ÛÔ‡Ú‡ ̇ ÔÓÌÓ‚ËÚ ‡ÌÚËÂÔËÎÂÔÚ˘ÌË Ï‰Ë͇ÏÂÌÚË (ÄÖå) Ë Ì‡ÏË‡ ‚Ò ÔÓ-¯ËÓÍÓ ÔËÎÓÊÂÌË ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇. è‰ÒÚ‡‚Îfl‚‡ S-Â̇ÌÚËÓÏÂ ̇ ÔË‡ˆÂڇχ, ÍÓÈÚÓ ÔËÚÂʇ‚‡ ÏÌÓ„Ó ÓÚ Ò‚ÓÈÒÚ‚‡Ú‡ ̇ ˉ‡ÎÌËfl ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚ. éÒÌÓ‚ÌËÚ ÏÛ Ô‰ËÏÒÚ‚‡ Ò‡ ·˙Á‡ ‡·ÒÓ·ˆËfl, ÎËÌÂÈ̇ Ù‡χÍÓÍËÌÂÚË͇, ÌÂÁ̇˜ËÚÂÎÌË ÎÂ͇ÒÚ‚ÂÌË ‚Á‡ËÏÓ-‰ÂÈÒÚ‚Ëfl, ÌÂÔÓ‚ÎËfl‚‡Ì ̇ ˜ÂÌÓ‰Ó·ÌËÚ ÂÌÁËÏË Ë ·˙·˜ÌÓ ÂÎËÏËÌË‡Ì (20). çÂÁ‡‰˙ÎÊËÚÂÎÌÓÚÓ ËÎË ·˙ÁÓ ÚËÚË‡ÌÂ Ë ‰‚ÛÍ‡ÚÌËflÚ ÔËÂÏ Ò˙˘Ó ÛÎÂÒÌfl‚‡Ú Ì„ӂÓÚÓ ÔË·„‡ÌÂ. ÄÌÚËÍÓÌ‚ÛÎÒË‚ÌËflÚ ÂÙÂÍÚ Ì‡ LEV Ò ‰˙ÎÊË Ì‡ ÌflÍÓÎÍÓ ÒÔˆËÙ˘ÌË ÏÂı‡ÌËÁχ: 1. ë‚˙Á‚‡Ì Ò˙Ò ÒË̇ÔÚ˘ÌËÚ ‚ÂÁËÍÛÎÌË ÔÓÚÂËÌË SV2A; 2. ê‰Û͈Ëfl ̇ ‚ËÒÓÍÓ‚ÓÎÚ‡ÊÌÓ ‡ÍÚË‚Ë‡˘ËÚ Ò N-ÚËÔ Ë ˜‡ÒÚ˘ÌÓ - P/Q-ÚËÔ Í‡ÎˆËÂ‚Ë ÔÓÚÓˆË; 3. ç‡Ï‡ÎÂÌÓ ÓÒ‚Ó·Óʉ‡‚‡Ì ̇ ͇ΈËÈ ÓÚ ‚˙ÚÂÍÎÂÚ˙˜ÌËÚ Ì‚ÓÌÌË ‰ÂÔ‡; 4. ê‰Û͈Ëfl ̇ ËÌıË·ËÚÓÌËfl ÂÙÂÍÚ Ì‡ ˆËÌÍ Ë β-͇·ÓÎËÌËÚ ‚˙ıÛ ıÎÓˉÌËfl ËÌÙÎÛÍÒ ˜ÂÁ ÔÓ‚ÎËfl‚‡Ì ̇ ÉÄåä- Ë „ÎˈËÌÓ‚ËÚ ˆÂÔÚÓË (4). ÉÓÎflÏ ·ÓÈ ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú ÂÙË͇ÒÌÓÒÚÚ‡ ̇ LEV ͇ÚÓ ÏÓÌÓ- Ë ÔÓÎËÚÂ‡ÔËfl ÔË Ô‡ˆË‡ÎÌË, ÏËÓÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË, Ô˙‚˘ÌÓ ËÎË ‚ÚÓ˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌ˘ÌÓ-ÍÎÓÌ˘ÌË (Éíäè), ÙÓÚÓÒÂÌÁËÚË‚ÌË ÔËÒÚ˙ÔË Ë ‡·Ò‡ÌÒË (7, 18, 19, 27, 33, 34, 35). èÓÚ‚˙‰Â̇ Â Ë Ì„ӂ‡-


ÑÂÍÂÏ‚Ë 2009

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

Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ Í‡ÚÓ Ï‰Ë͇ÏÂÌÚ ÓÚ Ô˙‚Ë ‰ ÔË ÌӂӉˇ„ÌÓÒÚˈË‡ÌË Ô‡ˆËÂÌÚË Ò Ô˙‚˘ÌÓ Éíäè ËÎË ÔËÒÚ˙ÔË Ò Ô‡ˆË‡ÎÌÓ Ì‡˜‡ÎÓ, ÍÓflÚÓ Â ÒıӉ̇ Ò Ú‡ÁË Ì‡ CBZ ÔË ÔÓ-‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ (14, 15). èÓ‰ıÓ‰fl˘ Â Ë ÔË ÁÎÓ͇˜ÂÒÚ‚ÂÌËÚ ‰ÂÚÒÍË ÂÔËÎÂÔÒËË – ÒË̉ÓÏ Ì‡ West, Lennox-Gastaut Ë ÔÓ„ÂÒ˂̇ ÏËÓÍÎÓÌÛÒ ÂÔËÎÂÔÒËfl, ÂÔËÎÂÔÒËfl Ò ÂÎÂÍÚ˘ÂÌ ÒÚ‡ÚÛÒ ÂÔËÎÂÔÚËÍÛÒ ÔÓ ‚ÂÏ ̇ ·‡‚ÌÓ‚˙ÎÌÓ‚Ëfl Ò˙Ì, ÔË ÍÓËÚÓ Ò˙˘Ó Ò ÓÚ˜ËÚ‡ ËÁ‚ÂÒÚÂÌ ·Î‡„ÓÔËflÚÂÌ ÍÎËÌ˘ÂÌ Ë ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÂÌ ÂÙÂÍÚ (2, 3, 7, 8, 16). 쉇˜ÌÓ Â ÔË·„‡ÌÂÚÓ ÏÛ ‚ ÏÓÌÓ- ËÎË ÔÓÎËÚÂ‡ÔËfl ÔË ‰Âˆ‡ Ë ÔÓ-‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË, Ô‰‚ˉ ‰Ó·‡Ú‡ ÔÓÌÓÒËÏÓÒÚ Ë ÔÓÁËÚË‚ÌËfl ÂÙÂÍÚ ‚˙ıÛ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ (10, 13). èÓ-ı‡‡ÍÚÂÌË ÌÂÊ·ÌË ÒÚ‡Ì˘ÌË ‰ÂÈÒÚ‚Ëfl Ò‡ ‰ÓÁÓÓ„‡Ì˘‡‚‡˘ËÚ ‚ ÌflÍÓË ÒÎÛ˜‡Ë ÛÏÓflÂÏÓÒÚ Ë Ò˙ÌÎË‚ÓÒÚ, ͇ÍÚÓ Ë Ôӂ‰Â̘ÂÒÍË ÓÚÍÎÓÌÂÌËfl, ÓÒÓ·ÂÌÓ ËÁ‡ÁÂÌË ÔË ·ÓÎÌË Ò ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ë/ËÎË Ô‰¯ÂÒÚ‚‡˘‡ ÔÒËıˇÚ˘̇ ÒËÏÔÚÓχÚË͇. ñÂÎÚ‡ ̇ ̇ÒÚÓfl˘ÂÚÓ ÔÓÛ˜‚‡Ì  ‰‡ Ò ÓÔ‰ÂÎË ÓÔÚËχÎ̇ڇ ‰ÓÁ‡ Ë ‰‡ Ò ÓÚ˜ÂÚ ÂÙË͇ÒÌÓÒÚÚ‡ Ë ÔÓÌÓÒËÏÓÒÚÚ‡ ̇ Levetiracetam (LEV) Á‡ ÒÓÍ ÓÚ 12 ÏÂÒˆ‡ ÔË ÔÓÎËÚÂ‡ÔËfl ̇ Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡Î̇ Ë „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËfl – ÂÁËÒÚÂÌÚ̇ ËÎË ÌÂ. èÄñàÖçíà à åÖíéÑà éÒ˙˘ÂÒÚ‚ËıÏ ÔÓÒÔÂÍÚË‚ÌÓ Ì‡·Î˛‰ÂÌËÂ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ ÓÚ 6 ÏÂÒˆ‡ ‰Ó 1 „. ̇ 65 Ô‡ˆËÂÌÚ‡ Ò ÂÔËÎÂÔÒËfl (ÛÚ‚˙‰Â̇ ËÎË Ì ͇ÚÓ ÂÁËÒÚÂÌÚ̇/ ̇ ÔÓÎËÚÂ‡ÔËfl Ò LEV. ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ Ô‡ˆËÂÌÚËÚ  32,68 ± 14,09 „. (ÓÚ 14 ‰Ó 57 „.), ‡ Ò‰̇ڇ ‰‡‚ÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ - 18,37 ± 11,62 „. (ÓÚ 22 ‰ÌË ‰Ó 40 „.). ëËÒÚÂχÚËÁË‡ıÏ ۘ‡ÒÚÌˈËÚ Ò˙Ó·‡ÁÌÓ: ÔÓÎ, ‚˙Á‡ÒÚ, ‰‡‚ÌÓÒÚ, ÂÚËÓÎÓ„Ëfl, ‚ˉ ̇ ÂÔËÎÂÔÒËflÚ‡, ÚËÔ Ë ˜ÂÒÚÓÚ‡ ̇ ÔËÒÚ˙ÔËÚÂ, Ô‰¯ÂÒÚ‚‡˘‡ Ë Ò˙Ô˙ÚÒÚ‚‡˘‡ ÚÂ‡ÔËfl Ë „Ë ÔÓÒΉËıÏ ̇ 6 ÏÂÒˆ‡ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÒÓχÚ˘ÂÌ, Ì‚ÓÎӄ˘ÂÌ, ÔÒËı˘ÂÌ ÒÚ‡ÚÛÒ, ÖÖÉ. èË ÌÂÓ·ıÓ‰ËÏÓÒÚ ÓÒ˙˘ÂÒÚ‚ËıÏ äÄí Ë üåê (퇷Îˈ‡ 1). èÓÒΉËıÏ ‚ ‰Ë̇ÏË͇ ÂÙÂÍÚ‡ ÓÚ ÔËÎÓÊÂÌËÂÚÓ Ì‡ LEV, ÓÚ˜ËÚ‡ÈÍË ˜ÂÒÚÓÚ‡Ú‡ Ë ÚÂÊÂÒÚÚ‡ ̇ ÔËÒÚ˙ÔËÚÂ, ͇ÚÓ ËÁÔÓÎÁ‚‡ıÏ LEV ͇ÚÓ ÔÓÎËÚÂ‡ÔËfl ‚ ‰ÓÁ‡ ÓÚ 1000 ‰Ó 3000 Ï„, Ò‰̇ ‰ÓÁ‡ 1646 ± 359 mg/d. ê‡ÁÔ‰ÂÎËıÏ ԇˆËÂÌÚËÚ ‚ 4 „ÛÔË ÒÔÓ‰ ‰Ì‚̇ڇ ‰ÓÁ‡ – 1000 mg/d – 7 (10,77%) 1500 mg/d – 34 (52,31%), 2000 mg/d – 23 (35,38%) Ë 3000 mg/d – 1 (1,54%). ëÔÓ‰ ÔÓ‚ÎËfl‚‡ÌÂÚÓ Ì‡ ÚÂÊÂÒÚÚ‡ Ë ˜ÂÒÚÓÚ‡Ú‡ ̇ ÂÔËÎÂÔÚ˘ÌËÚ ÔËÒÚ˙ÔË ‚ Í‡fl ̇ ̇·Î˛‰ÂÌËÂÚÓ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‰ÓÁ‡Ú‡ ̇ LEV Ë ÚËÔ‡ ÔËÒÚ˙ÔË, ‡ÁÔ‰ÂÎËıÏ ԇˆËÂÌÚËÚ ‚ 6 „ÛÔË – Ò ‚ÎÓ¯‡‚‡ÌÂ, ·ÂÁ ÔÓÏfl̇, ‰Û͈Ëfl <50%, ‰Û͈Ëfl 50%, ‰Û͈Ëfl 51-99%, ‰Û͈Ëfl 100%. ᇠÓÚ„Ó‚ÓËÎË Ì‡ ΘÂÌËÂÚÓ /ËÎË ÂÒÔÓ̉ÂË/ ÔËÂıÏ ԇˆËÂÌÚËÚÂ Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ _>50%, ‡ Á‡ ÌÂÓÚ„Ó‚ÓËÎË – Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ÔÓ‰ 50%. éÚ˜ÂÚÓıÏÂ Ë ÌÂÊ·ÌËÚ ÒÚ‡Ì˘ÌË ÂÙÂÍÚË. èËÎÓÊËıÏ ‚‡ˇˆËÓÌÂÌ Ë „‡Ù˘ÂÌ ‡Ì‡ÎËÁ Á‡ ÒÚ‡ÚËÒÚ˘ÂÒ͇ Ó·‡·ÓÚ͇. ë‰̇ڇ ˜ÂÒÚÓÚ‡ ̇ ÔËÒÚ˙ÔËÚ ÔÂ‰Ë LEV  4,1 ± 6,4 Ò‰Ï˘ÌÓ /ÓÚ 1 „Ó‰. ‰Ó 35 Ò‰Ï˘ÌÓ (Á‡ Ô‡ˆË‡ÎÌËÚÂ Ë 3,7 ± 9,4 ÏÂÒ˜ÌÓ) ÓÚ 1 ̇ 4 ÏÂÒ. ‰Ó 40 ÏÂÒ˜ÌÓ Á‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ. ìÒÚ‡ÌÓ‚ËıÏ ÓÚÍÎÓÌÂÌËfl ‚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ ÔË 10 (15,38%) Ô‡ˆËÂÌÚ‡, ‡ ‚ ÔÒËı˘ÌËfl ÒÚ‡ÚÛÒ - ÔË 21 (32,31%) - ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, ÍÓ„ÌËÚË‚ÌË Ë Ôӂ‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl. ÇÒ˘ÍË Û˜‡ÒÚÌËˆË Ò‡ ÔËÂχÎË ÔÂ‰Ë ÚÓ‚‡ ÄÖå. éÒÂÏ (12,31%) ÓÚ Ô‡ˆËÂÌÚËÚ ҇ Ò ÎÂÍÛ‚‡ÎË Ò‡ÏÓ Ò 1 ÄÖå, ÓÒڇ̇ÎËÚ 57 (87,69%) - Ò ÂÁËÒÚÂÌÚ̇ ÂÔËÎÂÔÒËfl - Ò‡ ·ËÎË Ì‡ ÔÓÎËÚÂ‡ÔËfl Ò ‰Ó 5 ωË͇ÏÂÌÚ‡ (VPA, CBZ, PB, CZP, ESM, Diazepam, PHT, OCBZ, TPM, LTG,

178

퇷Îˈ‡ 1: ï‡‡ÍÚÂËÒÚË͇ ̇ Ô‡ˆËÂÌÚËÚ èÓ͇Á‡ÚÂÎ èÓÎ - Ï˙Ê - ÊÂÌË Ñ‡‚ÌÓÒÚ Ì‡ ÂÔËÎÂÔÒËflÚ‡ /Á‡ 63 ·ÓÎÌË/ - ‰Ó 1 „. |- ÓÚ 1 ‰Ó 3 „. - ̇‰ 3 „. ÖÚËÓÎÓ„Ëfl ̇ ÂÔËÎÂÔÒËflÚ‡ /ÛÚÓ˜ÌÂ̇ ÔË 60 ·ÓÎÌË/ à‰ËÓÔ‡Ú˘̇ äËÔÚÓ„ÂÌ̇ ëËÏÔÚÓχÚ˘̇ /óåí, ‚˙ÁÔ‡ÎËÚÂÎ̇, Ó·ÂÏÂÌ ÔÓˆÂÒ, ÔÂË̇ڇÎ̇ ‡ÒÙËÍÒËfl, Â̈ÂÙ‡ÎÓÔ‡ÚËfl, ÚÓÍÒ˘̇ /‡ÎÍÓıÓÎ̇/, óåí + ‚˙ÁÔ‡ÎËÚÂÎ̇, Ò˙‰Ó‚‡, óåí + ÏÂÁˇÎ̇ „ÎËÓÁ‡, óåí + ‚˙ÁÔ‡ÎËÚÂÎ̇, ÚÛ·ÂÓÁ̇ ÒÍÎÂÓÁ‡, ıËÔÓ͇ÏÔ‡Î̇ ÒÍÎÂÓÁ‡, ÎÓ͇Î̇ ÏÓÁ˙˜Ì‡ ‡„ÂÌÂÁËfl, ÒË̉ÓÏ Ì‡ êÂÈ/ Çˉ ̇ ÂÔËÎÂÔÒËflÚ‡ /ÛÚÓ˜ÌÂ̇ ÔË 63 Ô‡ˆËÂÌÚ‡/ - Ô‡ˆË‡Î̇ /ÖÔËÎÂÔÒËfl Ô‡ˆË‡ÎËÒ ÍÓÌÚËÚÛ‡ ̇ äÓÊ‚ÌËÍÓ‚ – ÔË 1/ - „ÂÌÂ‡ÎËÁË‡Ì‡ /ûåÖ – ÔË 1, èÓ„ÂÒ˂̇ ÏËÓÍÎÓÌÛÒ ÂÔËÎÂÔÒËfl – ÔË 1/ Çˉ ̇ ÔËÒÚ˙ÔËÚ /Á‡ 63 Ô‡ˆËÂÌÚ‡/ 1. è‡ˆË‡ÎÌË Ç ÚÓ‚‡ ˜ËÒÎÓ: - èèè - äèè - è‡ˆË‡ÎÌË Ò ÇÉ 2. ÉÂÌÂ‡ÎËÁË‡ÌË - Éíäè 3. äÓÏ·ËÌË‡ÌË

n

%

Sp

24 41

36,92 63,08

6,13 6,13

1 3 59

1,75 5,25 93

1,67 2,83 3,24

12 15 33

20 25 55

5,21 5,64 6,48

45

71,43

5,74

18

28,57

5,74

28

44,45

6,31

2 7 19

3,17 11,12 30,16

2,23 3,99 5,83

13 22

20,63 34,92

5,14 6,05

GBP, TGB, ÍÓËÚÓ Ò‡ Ò Ó͇Á‡ÎË Ò Ì‰ÓÒÚ‡Ú˙˜Ì‡ ËÎË ‚ÂÏÂÌ̇ ÂÙÂÍÚË‚ÌÓÒÚ. èË ‚Ò˘ÍË ·ÓÎÌË LEV  ‚Íβ˜ÂÌ Í‡ÚÓ ‰Ó·‡‚˙˜Ì‡ ÚÂ‡ÔËfl, ͇ÚÓ ÔË 5 ·ÓÎÌË Â Á‡ÏÂÌËÎ ÔËÂχÌË ‰Ó ÚÓÁË ÏÓÏÂÌÚ ÄÖå. ç e ÔË·„‡Ì ͇ÚÓ ÏÓÌÓÚÂ‡ÔËfl. àÁÔÓÎÁ‚‡Ì  ‚ ÍÓÏ·Ë̇ˆËfl Ò VPA – ÔË 17 (16,15%), Ò VPA Ë OCBZ – ÔË 9 (13,85%), CBZ – ÔË 5 (7,69%) Ë Ò OCBZ – ÔË 5 (7,69%). èË 6 (9,23%) (Ô‡ˆËÂÌÚË Ò ‚ËÒÓÍÓ ÂÁËÒÚÂÌÚ̇ ÂÔËÎÂÔÒËfl) LEV  ‚ ˜ÂÚ‚Ó̇ ÍÓÏ·Ë̇ˆËfl ÓÚ ÄÖå – îË-

„Û‡ 1. êÖáìãíÄíà êÖÑìäñàü çÄ ÖèàãÖèíàóçàíÖ èêàëíöèà ê‡ÁÔÓ·„‡ÏÂ Ò ËÌÙÓχˆËfl Á‡ 38 ÓÚ Ô‡ˆËÂÌÚËÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÎËÌ˘ÌËfl ÂÙÂÍÚ Ë ÒÚ‡Ì˘ÌËÚ ‰ÂÈÒÚ‚Ëfl. ìÚÂÊÌfl‚‡Ì ̇ ÍÎËÌ˘ÌËÚ ËÁfl‚Ë ÓÚ˜ËڇϠÔË 6 (15,79%) Û˜‡ÒÚÌË͇, ÔÓÏfl̇ Ì ̇ÒÚ˙Ô‚‡ ÔË 7 (18,42%), Ò··Ó ÔÓ‚ÎËflÌË – Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ÔÓ‰ 50% Ò‡ 3 (7,89%) ËÎË ÌÂÓÚ„Ó‚ÓËÎË Ì‡ ΘÂÌËÂÚÓ Ò LEV Ò‡ Ó·˘Ó 16 (42,11%) ·ÓÎÌË. ê‰Û͈Ëfl ̇


ÑÂÍÂÏ‚Ë 2009

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

ÔËÒÚ˙ÔËÚÂ Ò 50% ̇·Î˛‰‡‚‡Ï ÔË 8 (21,05%), ÔË 11 (28,96%) „ËÒÚË‡Ï ‰Û͈Ëfl ÔÓ-„ÓÎflχ ÓÚ 50% Ë ÔÓ‰ 100%, 3 (7,89%) ÓÚ Ô‡ˆËÂÌÚËÚ ҇ Ò‚Ó·Ó‰ÌË ÓÚ ÔËÒÚ˙ÔË ‚ Í‡fl ̇ ÔÓÛ˜‚‡ÌÂÚÓ, Ú.Â. 22 (57,89%) ÓÚ Ô‡ˆËÂÌÚËÚ ҇ ÓÚ„Ó‚ÓËÎË Ì‡ ΘÂÌËÂÚÓ Ò LEV – îË„. 2.

èéÇãàüÇÄçÖ çÄ ÖèàãÖèíàóçàíÖ èêàëíöèà ëèéêÖÑ ÑéáÄíÄ LEV ç‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚ ÓÚ Ì‡Ò ‰ÓÁË Á‡ ÔÓÎËÚÂ‡ÔËfl Ò‡ 2000 Ë 1500 mg/d, ‡ ̇È-ÂÙÂÍÚ˂̇  1000 mg/d – ÔÓÒÚ˄̇ڇ  ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ _>50% ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â ÔË·„‡Ì‡ (Ò Ô‡ˆË‡ÎÌË Ë

„ÂÌÂ‡ÎËÁË‡ÌË, ÌÓ ÌÂ Ë Ò ÍÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË) – îË„Û‡ 3. èË ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‰ÓÁ‡Ú‡ 1500 mg/d ÂÒÔÓ̉ÂË Ò‡ 50% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, 50% - Ò „ÂÌÂ‡ÎËÁË‡ÌË Ë 71,43% - Ò ÍÓÏ·ËÌË‡ÌË. ᇠ‰ÓÁ‡Ú‡ ÓÚ 2000 mg/d Ò˙ÓÚ‚ÂÚÌÓÚÓ ÔÓ‚ÎËfl‚‡Ì  50% Á‡ Û˜‡ÒÚÌˈËÚÂ Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ë 71,43% ÓÚ ÚÂÁË Ò ÍÓÏ·ËÌË‡ÌË. ÅÓÎÌËÚÂ Ò „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË Ì‡ Ú‡ÁË ‰ÓÁ‡ Ò‡ ·ÂÁ ÔÓÏfl̇ ‚ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ËÎË Ò ‚ÎÓ¯‡‚‡ÌÂ. ÑàçÄåàóçé èêéëãÖÑüÇÄçÖ çÄ ÖîÖäíÄ éí èéãàíÖêÄèàüíÄ ë LEV ëèéêÖÑ íàèÄ ÖèàãÖèíàóçà èêàëíöèà èÄêñàÄãçà èêàëíöèà ë‡ÏÓ Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò ̇·Î˛‰‡‚‡Ú ÔË 28 (44,45%) ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÌÓ ÔË 16  ÓÚ˜ÂÚÂÌ ÂÙÂÍÚ‡ ̇ 6-ÚËfl Ë ÔË 14 – ̇ 12-ÚËfl ÏÂÒˆ. éÚ˜ËڇϠ‰Ó·Ó ÔÓ‚ÎËfl‚‡Ì ̇ ˜ÂÒÚÓÚ‡Ú‡ Ë ÚÂÊÂÒÚÚ‡ ̇ ÔËÒÚ˙ÔËÚÂ. èË 3-χ ÓÚ Ô‡ˆËÂÌÚËÚ ��‡ 6-ÚËfl ÏÂÒˆ Ë ÔË 2-χ ̇ 1 „Ó‰Ë̇ Ëχ Ôӂ˯‡‚‡Ì ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡. ç‡ 6ÚËfl ÏÂÒˆ Ë 1 „. ÓÚ Î˜ÂÌËÂÚÓ ÔË 5 Ô‡ˆËÂÌÚ‡ Ì ÓÚ˜ËڇϠÔÓÏfl̇ ‚ ÍÎËÌ˘ÌËÚ ÔÓfl‚Ë. Ç Í‡fl ̇ ÔÓÛ˜‚‡ÌÂÚÓ ÌÂÓÚ„Ó‚ÓËÎË Ò‡ 8 (57,15%) ÓÚ 14 Ô‡ˆËÂÌÚ‡. éÚÌÓ-

179

ÒËÚÂÎÌËflÚ ‰flΠ̇ Û˜‡ÒÚÌˈËÚÂ Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙ÔËÚ _>50%  50% ̇ 6-ÚËfl ÏÂÒˆ, ÒΉ ÍÓÂÚÓ Ì‡Ï‡Îfl‚‡ ‰Ó 42,85% ‚ Í‡fl ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇. ç‡ Ô˙‚‡Ú‡ ‚ËÁËÚ‡ ÓÚ ‚Íβ˜‚‡ÌÂÚÓ Ì‡ LEV Ò‚Ó·Ó‰ÌË ÓÚ ÔËÒÚ˙ÔË Ò‡ 12,5% ÓÚ Û˜‡ÒÚÌˈËÚÂ, ‚ÔÓÒΉÒÚ‚Ë ÓÚÌÓÒËÚÂÎÌËflÚ ËÏ ‰flΠ̇χÎfl‚‡ ‰Ó 7,14% ̇ 1 „. (îË„. 4).

ÉÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË. ÉÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË Ò ̇·Î˛‰‡‚‡Ú ÔË 13 (20,63%) ÓÚ Û˜‡ÒÚÌˈËÚ ‚ ÔÓÛ˜‚‡ÌÂÚÓ. ë‰ÂÏ ÓÚ Úflı Ò ÔÓÒΉfl‚‡Ú Á‡ ÔÂËÓ‰ ÓÚ 6 ÏÂÒˆ‡ ‰Ó 1 „Ó‰Ë̇. ÖÙÂÍÚ˙Ú ÓÚ Î˜ÂÌËÂÚÓ Ì‡ 6-ÚËfl ÏÂÒˆ Ò ҂Âʉ‡ ‰Ó 57,15% ÌÂÓÚ„Ó‚ÓËÎË, ÓÚ ÍÓËÚÓ 28,57% Ò‡ Ò ‚ÎÓ¯‡‚‡Ì ̇ ÍÎËÌ˘ÌËÚ ËÁfl‚Ë, ÔË 14,29% ÎËÔÒ‚‡

ÍÎËÌ˘ÂÌ ÂÙÂÍÚ, ‡ 14,29% Ò‡ Ò˙Ò Ò·· ÓÚ„Ó‚Ó – ÔÓ‰ 50% ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡. éÒڇ̇ÎËÚ 42,85% Ò‡ Ò _>50% ‰Û͈Ëfl ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÔËÒÚ˙ÔËÚÂ. Ç ÔÓˆÂÒ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ ÓÚÌÓÒËÚÂÎÌËflÚ ‰flΠ̇ ÓÚ„Ó‚ÓËÎËÚ ̇‡ÒÚ‚‡ ̇ 57,13% ‚ Í‡fl ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇. éÔËÒ‡ÌËÚ ۘ‡ÒÚÌËˆË Ò ÛÚÂÊÌfl‚‡Ì ̇ ÍÎËÌ˘̇ڇ ͇ÚË̇ ̇ 6-ÚËfl ÏÂÒˆ ̇χÎfl‚‡Ú ̇ ÒΉ‚‡˘‡Ú‡ ‚ËÁËÚ‡ Ò 50%, ‡ ÚÂÁË ·ÂÁ ÔÓ‚ÎËfl‚‡Ì ÓÒÚ‡‚‡Ú Ò˙˘ËflÚ ·ÓÈ. àÌÚÂÂÒ̇ Â Ë ‰Ë̇ÏË͇ڇ ‚ ÓÚÌÓÒËÚÂÎÌËfl ·ÓÈ Ì‡ Ô‡ˆËÂÌÚËÚ ҂ӷӉÌË ÓÚ ÔËÒÚ˙ÔË - ̇ 6-ÚËfl ÏÂÒˆ  28,57%, ̇ 1 „. – ̇‡ÒÚ‚‡ ‰Ó 42,84 (îË„. 5).


ÑÂÍÂÏ‚Ë 2009

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

äÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË. ë ÍÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË Ò‡ 22 (34,92%) ÓÚ Û˜‡ÒÚÌˈËÚ ‚ ÔÓÛ˜‚‡ÌÂÚÓ. èË 15 ÓÚ Úflı ÔÓÒΉËıÏ ÚÂÊÂÒÚÚ‡ Ë ˜ÂÒÚÓÚ‡Ú‡ ̇ ÔËÒÚ˙ÔËÚ ‚ ‰Ë̇ÏË͇. ç‡ 6-ÚËfl ÏÂÒˆ ÓÚ ÔÓÒΉfl‚‡ÌÂÚÓ ‰Û͈ËflÚ‡ ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ Ò > _50%  ̇΢̇ ÔË 60% ÓÚ Ô‡ˆËÂÌÚËÚÂ. òÂÒÚ ÏÂÒˆ‡ ÔÓ-Í˙ÒÌÓ ÓÚÌÓÒËÚÂÎÌËflÚ ‰flΠ̇ Û˜‡ÒÚÌˈËÚÂ Ò Ú‡Í‡‚‡ ÔËÒÚ˙Ô̇ ‰Û͈Ëfl ̇‡ÒÚ‚‡ ̇ 66,67%. Ä̇Îӄ˘̇  ‰Ë̇ÏË͇ڇ ‚ ÓÚÌÓÒËÚÂÎÌËfl ‰flΠ̇ ÌÂÓÚ„Ó‚ÓËÎËÚ ̇ ΘÂÌËÂÚÓ. ç‡ 6-ÚËfl ÏÂÒˆ ÚÓÈ Â 40%, ÓÚ ÍÓËÚÓ 13,33% Ò‡ Ò ÛÚÂÊÌfl‚‡Ì ̇ ÍÎËÌ˘̇ڇ ͇ÚË̇, 20% Ò‡ ·ÂÁ ÓÚ˜ÂÚÂÌ ÍÎËÌ˘ÂÌ ÂÙÂÍÚ Ë 6,67% Ò˙Ò Ò··Ó ÔÓ‚ÎËfl‚‡Ì – ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ Ò ÔÓ-χÎÍÓ ÓÚ 50%. Ç ÔÓˆÂÒ‡ ̇ ̇·Î˛‰ÂÌË ÛÚÂÊÌfl‚‡Ì ̇ ÍÎËÌ˘ÌËÚ ÔÓfl‚Ë Ò „ËÒÚË‡ ÔË 13,33% ‚ Í‡fl ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇. ãËÔÒ‚‡Ú

Ô‡ˆËÂÌÚË Ò Ô˙ÎÌÓ ÍÛÔË‡Ì ̇ ÔËÒÚ˙ÔËÚ ̇ 6-ÚËfl ÏÂÒˆ ÔÓ ‚ÂÏ ̇ ˆÂÎËfl ÔÂËÓ‰ ̇ ̇·Î˛‰ÂÌË (îË„. 6). éÚ 6 Îˈ‡ (9,52%) Ò ÏËÓÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË ÔÓÒΉËıÏ ‚ ‰Ë̇ÏË͇ ÂÙÂÍÚ‡ ÓÚ Î˜ÂÌËÂÚÓ ÔË 4 ·ÓÎÌË. ç‡ 6-ÚËfl ÏÂÒˆ ÓÚ Ì‡·Î˛‰ÂÌËÂÚÓ 3-χ Ô‡ˆËÂÌÚË Ò‡ ·ÂÁ ÔÓ‚ÎËfl‚‡ÌÂ, ‡ ÔË 1 – ÔËÒÚ˙ÔËÚ ҇ ÍÛÔË‡ÌË Ì‡Ô˙ÎÌÓ. Ç ÒΉ‚‡˘ËÚ 6 ÏÂÒˆ‡ ÔÓ‚ÎËfl‚‡Ì Ì Ò ÓÚ˜ËÚ‡ ÔË 2-χ. èË 1 Ô‡ˆËÂÌÚ ‰Û͈ËflÚ‡ ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡  ̇‰ 50%, ‡ 1 ·ÓÎÂÌ ÓÒÚ‡‚‡ ·ÂÁ ÔËÒÚ˙ÔË. Ç Ò‡‚ÌËÚÂÎÂÌ ÔÎ‡Ì ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÔÓ‚ÎËfl‚‡ÌÂÚÓ Ì‡ ‡Á΢ÌËÚ ÚËÔÓ‚Â ÔËÒÚ˙ÔË ‚ ‰Ë̇ÏË͇, ̇ 6-ÚËfl ÏÂÒˆ Ô‡‚Ë ‚Ô˜‡ÚÎÂÌË ̇È-Á̇˜ËÚÂÎÌÓ ÔÓ‚ÎËfl‚‡Ì ̇ ÍÓÏ·ËÌË‡ÌËÚ ÔËÒÚ˙ÔË – ‚ 60% ÓÚ ÒÎÛ˜‡ËÚÂ, ̇ ‚ÚÓÓ ÏflÒÚÓ Ò‡ Ô‡ˆË‡ÎÌËÚ – ‚ 50%, ‡ „ÂÌÂ‡ÎËÁË‡ÌËÚ ҇ ÔÓ‚ÎËflÌË ‚ 42,86% ÓÚ ÒÎÛ˜‡ËÚÂ. Ç Í‡fl ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇ ÓÚÌÓÒËÚÂÎÌËflÚ ‰flΠ̇ ÂÒÔÓ̉ÂËÚ ̇‡ÒÚ‚‡ Ò˙ÓÚ-

‚ÂÚÌÓ ‰Ó 66,67% Á‡ ÍÓÏ·ËÌË‡ÌËÚÂ Ë 57,13% Á‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ Ë Ì‡Ï‡Îfl‚‡ ‰Ó 42,85% Á‡ Ô‡ˆË‡ÎÌËÚÂ. (îË„. 7). èÓÒΉfl‚‡Ï ‚ ‰Ë̇ÏË͇ Ë ÔÓÏfl̇ڇ ‚ Ò‰̇ڇ ÏÂÒ˜̇ ˜ÂÒÚÓÚ‡ ̇ ÔËÒÚ˙ÔËÚÂ. ç‡È-Ò˙˘ÂÒÚ‚Â̇ ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ Á‡ Ô‡ˆË‡ÎÌËÚ ÔËÒÚ˙ÔË (Ôӂ˜ ÓÚ 2 Ô˙ÚË) ÓÚ˜ËڇϠ̇ 6-ÚËfl ÏÂÒˆ ÓÚ Î˜ÂÌËÂÚÓ – Ò‰ÌÓ 7,3 ÔËÒÚ˙Ô‡ ÏÂÒ˜ÌÓ. Ç Í‡fl ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇ Ò‰̇ڇ ÏÂÒ˜̇ ˜ÂÒÚÓÚ‡ ̇ ÔËÒÚ˙ÔËÚ  6,6, Ú. ÓÚ˜ËڇϠӷ˘‡ ‰Û͈Ëfl 2,48 Ô˙ÚË. á‡

180

„ÂÌÂ‡ÎËÁË‡ÌËÚ ÔËÒÚ˙ÔË ‰Û͈ËflÚ‡ ÔÂÁ Ô˙‚ËÚ 6 ÏÂÒˆ‡  ÏËÌËχÎ̇ (Ò ÓÍÓÎÓ 3 %) - 3,6 ÔËÒÚ˙Ô‡ ÏÂÒ˜ÌÓ Ë ÔÓ-Á̇˜ËÚÂÎ̇ ‚˙‚ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚Ë̇ ̇ Ô˙‚‡Ú‡ „Ó‰Ë8

̇ ÓÚ Î˜ÂÌËÂÚÓ – (Ò 39%) - 2,2 ÔËÒÚ˙Ô‡ ÏÂÒ˜ÌÓ - îË„Û‡ 8. é·˘Ó ÔË 6 (15,78%) ÓÚ Ì‡·Î˛‰‡‚‡ÌËÚ ԇˆËÂÌÚË ÓÚ˜ËڇϠÌÂÊ·ÌË ÒÚ‡Ì˘ÌË ‰ÂÈÒÚ‚Ëfl - „·‚Ó·ÓÎËÂ Ë ÓÚÔ‡‰Ì‡ÎÓÒÚ – ÔË 2-χ , Á‡ÔÂÍ – 1, ÒÚÓχ¯ÌÓ ‰‡ÁÌÂÌ - 1, „‡‰ÂÌÂ Ë Ò‚ÂÚÓ‚˙ÚÂÊ – 1, ‡„ÂÒË‚ÌÓÒÚ – 1 (̇ÎÓÊËÎÓ Ò  ‚Íβ˜‚‡Ì ̇ ıÎÓÔÓÚËÍÒÂÌ). í ҇ ‰Ó‚ÂÎË ‰Ó ËÁÍβ˜‚‡Ì ̇ ωË͇ÏÂÌÚ‡ ÔË 2-χ ÓÚ ·ÓÎÌËÚÂ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‰Ó·Ëfl ÍÎËÌ˘ÂÌ ÂÙÂÍÚ, ‡ ÔË 1 Û˜‡ÒÚÌËÍ LEV  Á‡ÏÂÌÂÌ Ò ‰Û„ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚ ÔÓ‡‰Ë Á̇˜ËÚÂÎÌÓ Û‚Â΢‡‚‡Ì ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡. éÅëöÜÑÄçÖ èӂ‰ÂÌÓÚÓ ÓÚ Ì‡Ò ÔÓÛ˜‚‡Ì ‚Íβ˜‚‡ 65 Ô‡ˆËÂÌÚ‡ ̇ ‚˙Á‡ÒÚ ÏÂÊ‰Û 14 Ë 57 „. Ò˙Ò Ò‰̇ ‰‡‚ÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÓÍÓÎÓ 18 „., ÍÓËÚÓ ÔÓÎÛ˜‡‚‡Ú Ô‡ˆË‡ÎÌË, „ÂÌÂ‡ÎËÁË‡ÌË Ë ÍÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË ÔË ÔÓ-‚ËÒÓÍ ÓÚÌÓÒËÚÂÎÂÌ ‰flΠ̇ ·ÓÎÌËÚ Ò˙Ò ÒËÏÔÚÓχÚ˘̇ ÂÔËÎÂÔÒËfl (55%) Ë ÍËÔÚÓ„ÂÌ̇ ÂÔËÎÂÔÒËfl (25%). í ҇ ÎÂÍÛ‚‡ÌË ·ÂÁ Á‡‰Ó‚ÓÎËÚÂÎÂÌ ÂÙÂÍÚ Ò 1 ‰Ó 6 ÄÖå, ̇ ÙÓ̇ ̇ ÍÓËÚÓ Ò‰̇ڇ ÔËÒÚ˙Ô̇ ˜ÂÒÚÓÚ‡ ÔÂ‰Ë ‚Íβ˜‚‡Ì ̇ LEV  4,1 Ô‡ˆË‡ÎÌË ÔËÒÚ˙Ô‡ Ò‰Ï˘ÌÓ Ë 3,7 „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙Ô‡ ÏÂÒ˜ÌÓ. 87,69% ÓÚ Û˜‡ÒÚÌˈËÚ ҇ Ò ÂÁËÒÚÂÌÚ̇ ÙÓχ ̇ ÂÔËÎÂÔÒËfl. ç‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡Ì‡Ú‡ ‰ÓÁ‡ LEV  1500 mg/d, ÒΉ‚‡Ì‡ ÓÚ 2000 mg/d, ‡ ̇È-ÂÙÂÍÚ˂̇ - 1000 mg/d – ÔÓÒÚ˄̇ڇ  ‰Û͈Ëfl ̇ ÔËÒÚ˙ÔËÚ >_50% ÔË 100% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÔË ÍÓËÚÓ Â ÔË·„‡Ì‡. åËÌËχÎ̇  ‡ÁÎË͇ڇ Á‡ Ó·˘‡Ú‡ Í‡È̇ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ ‰ÓÁËÚ 1500 Ë 2000 mg/d - ‰Û͈Ëfl ̇ ÔËÒÚ˙ÔËÚ >_50% Ò˙ÓÚ‚ÂÚÌÓ ÔË 53,33% Ë 52,63%. è‡‚Ë ‚Ô˜‡ÚÎÂÌË ÔÓ-‰Ó·ÓÚÓ ÔÓ‚ÎËfl‚‡Ì ̇ „ÂÌÂ‡ÎËÁË‡ÌËÚ ÔËÒÚ˙ÔË ÓÚ ‰ÓÁ‡ 1500 mg/d ‚ Ò‡‚ÌÂÌËÂ Ò 2000mg/d. ë‰̇ڇ ‰ÓÁ‡ Á‡ ÔÓÛ˜‚‡ÌÂÚÓ Â 1646mg/d ÔË ‰Ë‡Ô‡ÁÓÌ 1000-3000mg/d. Ç ‰Û„Ë ÔÓÛ˜‚‡ÌËfl ÔË·„‡Ì‡Ú‡ Ò‰̇ ‰ÓÁ‡ Á‡ ÔÓÎËÚÂ‡ÔËfl ‚‡Ë‡ ÓÚ 1100 ‰Ó 2281 mg/d (9, 17, 22, 39) . èË ÔÓÛ˜‚‡Ì ̇ Shorvon Ë Ò˙Ú. 22,8% ÓÚ ·ÓÎÌËÚ ̇ 1000 mg/d Ò‡ ÓÚ„Ó‚ÓËÎË Ì‡ ΘÂÌËÂÚÓ, ‡ ÔË ‰ÓÁ‡ 2000 mg/d – 31,6% (1). Verdru Ë Ò˙Ú. ÔË·„‡Ú ‰ÓÁ‡ ÓÚ 3000 mg/d ÔË Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡Ú˘̇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËfl Ë ÏËÓÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË Ë ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â 58,3% ÓÚ Û˜‡ÒÚÌˈËÚ ҇ Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ÔÓÌ 50% ÔË ‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ (42). íÂÁË ‰‡ÌÌË ÔÓÚ‚˙ʉ‡‚‡Ú ‰ÓÁÓÁ‡‚ËÒËÏËfl ÂÙÂÍÚ Ì‡ ωË͇ÏÂÌÚ‡. èË ·ÓÎÌËÚÂ Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË ÂÒÔÓ̉ÂË Ò‡ 42,85%, Ò „ÂÌÂ‡ÎËÁË‡ÌË – 57,13%, ‡ Ò ÍÓÏ·ËÌË‡ÌË – 66,67%. éÚ Úflı Ò˙ÓÚ‚ÂÚÌÓ 7,14% Ë 42,84% Ò‡ Ò Ô˙ÎÌÓ


ÑÂÍÂÏ‚Ë 2009

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

ÍÛÔË‡Ì ̇ ÔËÒÚ˙ÔÌËÚ ÔÓfl‚Ë. 燯ËÚ ÂÁÛÎÚ‡ÚË Ò ‰Ó·ÎËʇ‚‡Ú ‰Ó ÚÂÁË Ì‡ ‰Û„Ë ‡‚ÚÓË (9, 31, 39, 40) . ÄÎÂÍÒË‚ Ë Ò˙Ú., ͇ÍÚÓ Ë Cuba Ë Ò˙Ú. ÛÒÚ‡ÌÓ‚fl‚‡Ú ÒıÓ‰ÌË ÂÁÛÎÚ‡ÚË Á‡ ÔÓÒÚËÚÂ Ë ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, ͇ÚÓ ‰ÌËÚ ·ÂÁ ÔËÒÚ˙ÔË Ì‡‡ÒÚ‚‡Ú Ò 6,8 ̇ ÚÂÚËfl ÏÂÒˆ ÓÚ Î˜ÂÌËÂÚÓ Ò LEV (1, 26). á‡Â‰ÌÓ Ò ÚÓ‚‡ ̇χÎfl‚‡ ‚ÚÓ˘̇ڇ „ÂÌÂ‡ÎËÁ‡ˆËfl ̇ ÔËÒÚ˙ÔËÚÂ. Ö‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ÏËÓÍÎÓÌ˘ÌË ÔËÒÚ˙ÔË ‚ ̇¯ÂÚÓ ÔÓÛ˜‚‡Ì ÓÒÚ‡‚‡ ·ÂÁ ÔËÒÚ˙ÔË, ‡ 1 Â Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ̇‰ 50%. Jongsma Ë Ò˙Ú., Resor Ë Ò˙Ú., Czapinski Ë Ò˙Ú., Krauss Ë Ò˙Ú., Greenhil Ë Ò˙Ú., Gilland Ë Ò˙Ú., Fattouch Ë Ò˙Ú., Hirsch Ë Ò˙Ú., Husain Ë Ò˙Ú, Meencke Ë Ò˙Ú., Mohanraj ÔÓÒÚË„‡Ú Ò‚Ó·Ó‰‡ ÓÚ ÔËÒÚ˙ÔË ÔË Ì‡‰ 60% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ˛‚ÂÌËÎ̇ ÏËÓÍÎÓÌÛÒ ÂÔËÎÂÔÒËfl (5, 24, 25), ÔË Ì‡·Î˛‰ÂÌË ̇ ꇯ‚‡ Ë Ò˙Ú. 84% ÓÚ ·ÓÎÌËÚÂ Ò Ú‡Í‡‚‡ ÙÓχ ̇ ÂÔËÎÂÔÒËfl Ò‡ Ò ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ > _ 50% (7, 8). Ç Í‡fl ̇ ̇¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‰ÓÁ‡Ú‡ Ë ÚËÔ‡ ̇ ÂÔËÎÂÔÚ˘ÌËÚ ÔËÒÚ˙ÔË, ÓÚÌÓÒËÚÂÎÌËflÚ ‰flΠ̇ ÓÚ„Ó‚ÓËÎËÚ  57,89%. íÂÁË ÂÁÛÎÚ‡ÚË Ò ‰Ó·ÎËʇ‚‡Ú ‰Ó ÓÚ˜ÂÚÂ̇ڇ ÂÙÂÍÚË‚ÌÓÒÚ ÔË ÔÓÛ˜‚‡ÌËfl ̇ ÄÎÂÍÒË‚ Ë Ò˙Ú., ÅÓÊËÌÓ‚‡ Ë Ò˙Ú., ɇÌ‚‡ Ë Ò˙Ú., ꇯ‚‡ Ë Ò˙Ú., Allder Ë Ò˙Ú, Pellock Ë Ò˙Ú., Sake Ë Ò˙Ú., Meencke Ë Ò˙Ú., Ben-Menachen Ë Ò˙Ú., ÔË ÍÓËÚÓ ÓÚÌÓÒËÚÂÎÌËflÚ ‰flΠ̇ ÂÒÔÓ̉ÂËÚ ‚‡Ë‡ ÏÂÊ‰Û 29,8 Ë 95,7% (1, 3, 4, 5, 6, 7, 9, 11, 12, 32, 36, 39). ê‰Û͈ËflÚ‡ ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ Ò Ò˙ÔÓ‚Óʉ‡ Ë Ò˙Ò Á̇˜ËÚÂÎÌÓ ÔÓ‚ÎËfl‚‡Ì ̇ ÂÔËÎÂÔÚ˘̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÖÖÉ (2, 3, 5). ä‡ÚÓ ÂÁÛÎÚ‡Ú ÓÚ Ì‡¯ÂÚÓ ÔÓÛ˜‚‡Ì ÓÚ˜ËڇϠ̇ȷ˙Á‡ Ë ‚ËÒÓ͇ Í‡È̇ ‰Û͈Ëfl ̇ ÔËÒÚ˙Ô̇ڇ ˜ÂÒÚÓÚ‡ ÔË Ô‡ˆË‡ÎÌËÚ ÔËÒÚ˙ÔË Ë ÔÓ-ÔÓÒÚÂÔÂÌÌÓ Ì‡‡ÒÚ‚‡˘‡ ‰Û͈Ëfl ÔË „ÂÌÂ‡ÎËÁË‡ÌËÚ ÔËÒÚ˙ÔË. LJÊÂÌ ÔÓ͇Á‡ÚÂÎ Á‡ ÂÙË͇ÒÌÓÒÚ Ë ÔÓÌÓÒËÏÓÒÚ Ì‡ ‰ËÌ Ï‰Ë͇ÏÂÌÚ Â ÔÓˆÂÌÚ˙Ú Ô‡ˆËÂÌÚË ÔÓ‰˙ÎÊËÎË Î˜ÂÌËÂÚÓ Ò Ì„Ó. Ç ıÓ‰‡ ̇ ̇¯ÂÚÓ Ì‡·Î˛‰ÂÌË ҇ÏÓ ÔË 3-χ ·ÓÎÌË (7,89%) Ò ÔÂÍ˙Ò‚‡ ÔË·„‡ÌÂÚÓ Ì‡ LEV. èË 1 ÓÚ Úflı ÚÓ‚‡ Ò ‰˙ÎÊË Ì‡ Û‚Â΢Â̇ڇ ÔËÒÚ˙Ô̇ ˜ÂÒÚÓÚ‡. Ç Ò‚Ó ÔÓÛ˜‚‡Ì Soni Ë Ò˙Ú. ÛÒÚ‡ÌÓ‚fl‚‡Ú ÒÚÓÈÌÓÒÚË Ì‡ ÚÓÁË ÔÓ͇Á‡ÚÂÎ ÓÚ 70%, ‡ ÔË Ì‡·Î˛‰ÂÌËfl ̇ ‰Û„Ë ‡‚ÚÓË ÚÓÈ ‚‡Ë‡ ÓÚ 84 ‰Ó 50% ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ËÁÏË̇ÎÓÚÓ ‚ÂÏ ÓÚ ‚Íβ˜‚‡ÌÂÚÓ (41). Ç ÔÓÛ˜‚‡Ì ̇ ꇯ‚‡ Ë Ò˙Ú. – 88,9% ÓÚ Ô‡ˆËÂÌÚËÚ ÓÒÚ‡‚‡Ú ̇ ΘÂÌËÂ Ò LEV, ÍÓÂÚÓ Â ‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ Ì„ӂ‡Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ Ë ‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ (6, 26, 40). Marson Ë Ò˙Ú. ‰Ó͇Á‚‡Ú ÓÚÌÓÒËÚÂÎÂÌ ËÒÍ Á‡ ÔÂÍ‡Úfl‚‡Ì ̇ ΘÂÌËÂÚÓ Ò LEV 1,21 Ò Ì‡È-‰Ó·Ó Ò˙ÓÚÌÓ¯ÂÌË ÂÒÔÓ̉ÂË ÔÂÍ˙Ò̇ÎË Î˜ÂÌËÂÚÓ ‚ Ò‡‚ÌÂÌËÂ Ò OCBZ, Remacemide Ë Zonizamide ÔË Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl (29). èË 6 (15,78%) ÓÚ Ì‡¯ËÚ ԇˆËÂÌÚË ÓÚ˜ËڇϠÒÚ‡Ì˘ÌË ÂÙÂÍÚË („·‚Ó·ÓÎËÂ Ë ÓÚÔ‡‰Ì‡ÎÓÒÚ, ÒÏÛ˘ÂÌËfl ÓÚ ÒÚ‡Ì‡ ̇ ÒÚÓχ¯ÌÓ-˜‚ÌËfl Ú‡ÍÚ, Ò‚ÂÚÓ‚˙ÚÂÊ, ‡„ÂÒË‚ÌÓÒÚ). í ҇ ‰Ó‚ÂÎË ‰Ó ÒÔË‡Ì ̇ ωË͇ÏÂÌÚ‡ ÔË 2-χ ÓÚ Úflı. ç‡È-˜ÂÒÚËÚ ÌÂÊ·ÌË ÒÚ‡Ì˘ÌË ‰ÂÈÒÚ‚Ëfl ÓÔËÒ‡ÌË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ (ÔË ‰Ó 70% ÓÚ Ô‡ˆËÂÌÚËÚÂ) Ò‡: ÛÏÓ‡, Á‡Ï‡È‚‡ÌÂ, ÒÓÏÌÓÎÂÌÚÌÓÒÚ, ‡Ú‡ÍÒËfl, ‡ÎÂ„˘̇ ‡͈Ëfl, Á‡„Û·‡ ̇ ‡ÔÂÚËÚ, Ôӂ˯‡‚‡Ì ̇ Ú„ÎÓÚÓ, ‰Ë‡Ëfl, ·ÂÁÒ˙ÌËÂ, ËÌÓÙ‡ËÌ„ËÚ, ̇Û¯ÂÌËfl ̇ ̇ÒÚÓÂÌËÂÚÓ Ë Ôӂ‰ÂÌËÂÚÓ, ‡Á‰‡ÁÌËÚÂÎÌÓÒÚ, ‡„ÂÒË‚ÌÓÒÚ, ‰ÂÔÂÒËfl, Ú‚ÓÊÌÓÒÚ, ÔÒËıÓÚ˘ÌË ÔÓfl‚Ë (1, 2, 9, 10, 12, 14, 17, 21, 22, 23, 28, 31, 37, 38, 40). ç‡È˜ÂÒÚÓ Ú ҇ ÎÂÍÓ ËÁ‡ÁÂÌË Ë ·˙ÁÓ ÔÂıÓ‰ÌË (1, 36). é·ËÍÌÓ‚ÂÌÓ Ò‡ Ò‚˙Á‡ÌË Ò ‚ËÒÓ͇ ̇˜‡Î̇ ‰ÓÁ‡ (23). í ÒÚ‡‚‡Ú Ô˘Ë̇ Á‡ ÔÂÍ‡Úfl‚‡Ì ̇ ΘÂÌËÂÚÓ ‚ ‰Ó 14,4% ÓÚ ÒÎÛ˜‡ËÚÂ Ë Ò‡ ÔÓ-Ò··Ó ÔÓfl‚ÂÌË ÔË ÔÓ-·‡‚ÌÓ ‚˙‚Âʉ‡Ì ̇ ωË͇ÏÂÌÚ‡ (14). Ç Á‡Íβ˜ÂÌË LEV  Ï‰Ë͇ÏÂÌÚ Ò ‚ËÒÓ͇ Ë ‰˙΄ÓÒӘ̇ ÒÚ‡·ËÎ̇ ÂÙË͇ÒÌÓÒÚ ÔË Ô‡ˆË‡ÎÌË Ë „ÂÌÂ‡ÎËÁË-

181

‡ÌË ÂÔËÎÂÔÒËË. íÓÈ Â ‰Ó· ÔÓÌÓÒËÏ, Ò ÎÂÍË Ë ‚ÂÏÂÌÌË ÌÂÊ·ÌË ÒÚ‡Ì˘ÌË ‰ÂÈÒÚ‚Ëfl, fl‰ÍÓ ‰Ó‚Âʉ‡˘Ë ‰Ó ÔÂÍ˙Ò‚‡Ì ̇ ΘÂÌËÂÚÓ. ãàíÖêÄíìêÄ 1.

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6. 7. 8. 9. 10.

11. 12. 13. 14. 15.

16. 17. 18.

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20. 21. 22. 23. 24. 25. 26. 27. 28. 29.

30. 31.

ÄÎÂÍÒË‚, Ä., Ä. ä‡ÔÂÎflÌ, Ñ. åË̘‚, ã. ÂÁÓ‚‡, å. ꇯ‚‡, ç. ÑÂ΂‡, è. âÓ‰‡ÌÓ‚‡, è. òÓÚÂÍÓ‚, è. ëÚ‡ÏÂÌÓ‚‡, ê. ä‡ÎÔ‡˜ÍË, ë. ñÂÍÓ‚, ë. ɇÌ‚‡, Ç. âÓÎÓ‚‡, èÓÛ˜‚‡Ì ̇ ‰˙΄ÓÚ‡ÈÌËfl ÂÙÂÍÚ Ì‡ Levetiracetam ͇ÚÓ ‰Ó·‡‚˙˜Ì‡ ÚÂ‡ÔËfl ÔË ÂÙ‡ÍÚÂÌË Ô‡ˆË‡ÎÌË ÂÔËÂÔÒËË, Å˙΄‡Ò͇ Ì‚ÓÎÓ„Ëfl, 2004, ÚÓÏ 4, ·. 3, 131-135. ÅÓÊËÌÓ‚‡, Ç., è. ÑËÏÓ‚‡, Levetiracetam (Keppra) – ÌÓ‚ ¯ËÓÍÓÒÔÂÍÚ˙ÂÌ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚ ‚ ‰ÂÚÒ͇ڇ ‚˙Á‡ÒÚ – ÂÙÂÍÚË‚ÌÓÒÚ, ÔÓÌÓÒËÏÓÒÚ, Ë̉Ë͇ˆËË, 燈ËÓ̇Î̇ ÍÓÌÙÂÂ̈Ëfl ÔÓ ‰ÂÚÒ͇ Ì‚ÓÎÓ„Ëfl, ÔÒËıˇÚËfl Ë ÔÒËıÓÎÓ„Ëfl ̇ ‡Á‚ËÚËÂÚÓ, 26-28.10.2006, 23-25. ÅÓÊËÌÓ‚‡, Ç., è. ÑËÏÓ‚‡, Levetiracetam (Keppra) ÔË ÚÂ‡Ô‚Ú˘ÌÓ-ÂÁËÒÚÂÌÚÌË ÂÔËÂÔÒËË ‚ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ – ÍÎËÌ˘ÂÌ ÂÙÂÍÚ, ÔÓÌÓÒËÏÓÒÚ, ‰˙΄ÓÚ‡ÈÌÓ ÔÓÒΉfl‚‡ÌÂ, ÖÔËÎÂÔÒËflÚ‡ ‰ÌÂÒ, 2007, ·. 1, 23-33. ɇÌ‚‡, É., Levetiracetam (Keppra) – çÓ‚Ë ‚˙ÁÏÓÊÌÓÒÚË ‚ ΘÂÌËÂÚÓ Ì‡ ÂÔËÎÂÔÒËflÚ‡, ÌÓ‚Ë Ì‡‰ÂʉË, ÌÓ‚Ë ÛÒÔÂıË, ÖÔËÎÂÔÒËflÚ‡ ‰ÌÂÒ, 2007, ·. 1, 4-14. ɇÌ‚‡, É., Ñ. ÑËÏÓ‚‡, ê. äÛÁχÌÓ‚‡, å. ꇯ‚‡, è. ëÚ‡ÏÂÌÓ‚‡, äÎËÌ˘ÌË Ë ÖÖÉ ÂÁÛÎÚ‡ÚË ÔË ‰˙΄ӂÂÏÂÌ̇ ‰ÓÔ˙΂‡˘‡ ÚÂ‡ÔËfl Ò Levetiracetam (Keppra) ̇ Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡Î̇ ÂÔËÎÂÔÒËfl, ÖÔËÎÂÔÒËflÚ‡ ‰ÌÂÒ, 2007, ·. 1, 34-41. ꇯ‚‡, å., å. åË·ÌÓ‚‡, å. ꇉ‚‡, Ñ. Äڇ̇ÒÓ‚‡, éˆÂÌ͇ ̇ ÌÓ‚ËÚ ÔÓÚË‚ÓÂÔËÎÂÔÚ˘ÌË Ò‰ÒÚ‚‡ ͇ÚÓ ‰Ó·‡‚˙˜Ì‡ ÚÂ‡ÔËfl ÔË ÂÁËÒÚÂÌÚÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÒËË, Å˙΄‡Ò͇ Ì‚ÓÎÓ„Ëfl, ÚÓÏ 4, ·. 4, 173-177. ꇯ‚‡, å., åflÒÚÓÚÓ Ì‡ Levetiracetam (Keppra) ‚ ΘÂÌËÂÚÓ Ì‡ ÏËÓÍÎÓÌ˘ÌËÚ ÂÔËÎÂÔÒËË, ÖÔËÎÂÔÒËflÚ‡ ‰ÌÂÒ, 2007, ·. 1, 15-22. ꇯ‚‡, å. åflÒÚÓÚÓ Ì‡ ã‚ÂÚË‡ˆÂڇχ ‚ ΘÂÌËÂÚÓ Ì‡ ÂÔËÎÂÔÒËflÚ‡ – ÌÓ‚ÓÒÚË ‚ ÍÎËÌ˘ÌÓÚÓ ÔËÎÓÊÂÌË ̇ ωË͇ÏÂÌÚ‡, Å˙΄‡Ò͇ Ì‚ÓÎÓ„Ëfl, 2004, ÚÓÏ 4, ·. 1, 17-20. Allder, S., S. Mulcrone, S. Kuc, C. Mack, R. Grunewald, Initial experience of levetiracetam in patients with refractory epilepsy in routine clinical practice, Epilepsia, 2002, 43 (8), 117. Andermann, E., F. Andermann, P. Meyvisch, A. Vandenriessche, J. Schiemann Delgado, S. Noachtar, N160 Study Group, Seizure control with Levetiracetam in Juvenile Myoclonic Epilepsy, Presented at the Annual Meeting of the American Epilepsy Society, Dec 2-6-th, 2005. Ben-Menachem, E., Preliminary Efficacy of Levetiracetam in Monotherapy, Epileptic Disorders, 2003, 5, 551-555. Ben-Menachem, E., P. Edrich, B. Van Vleymen, J. Sander, B. Schmidt, Evidence for Sustained Efficacy of levetiracetam as add-on epilepsy therapy, Epilepsy Research, 2003, 53, 57-64. Berkovic, S., R. Knowlton, R. Leroy, J. Schiemann, U. Falter, Placebo-controlled study of levetiracetam in idiopathic generalized epilepsy, Neurology, 2007, 69 (18), 1751-60. Brodie, M., E. Perucca, P. Ryvlin, E. Ben-Menachem, H.-J Meencke, Comparison of levetiracetam and controlled-release carbamazepine in newly diagnosed epilepsy, Neurology, 2007, 68, 402-408. M. Brodie, Ben-Menachem, E., E. Perucca, Efficacy of Levetiracetam Monotherapy; Randomized Double-blind Head-to-Head Comparison with Carbamazepine CR in Newly Diagnosed Epilepsy Patients with Partial Onset of Generalized Tonic-Clonic Seizures, Neurology, 2006, 66 (2), 73. Buyse, G., B. Ceulemans, L Lagae, Add-on levetiracetam in refractory generalized childhood epilepsy syndromes, Epilepsia, 2002, 43 (8), 154. Candlish, S, L. Nashef, Audit of levetiracetam prescribing, Epilepsia, 2002, 43 (8), 118-119. Di Bonaventura, C., J. Fattouch, F. Mari, G Egeo, A. Vandano, M. Prencipe, M. Monfredi, A. Gia Monardo, Clinical Experience with Levetiracetam in Idiopathic Generalized Epilepsy according to Different Syndrome Subtypes, Epilepsy Disorders, 2005, 7 (3), 231-5. French, J., S. Di Nicola, S. Arrigo, Å˙Á‡ Ë ÛÒÚÓȘ˂‡ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ levetiracetam ‚ ̇˜‡ÎÌËfl ÔÂËÓ‰ Ë ÔÂÁ Ô˙‚ËÚ 3 ÏÂÒˆ‡ ÓÚ Î˜ÂÌËÂÚÓ Ì‡ Ô‡ˆËÂÌÚË Ò ÂÁËÒÚÂÌÚ̇ ÂÔËÎÂÔÒËfl, Epilepsia, 2005, 46 (8), 13041307. 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ÑÂÍÂÏ‚Ë 2009

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

32. Meencke, H., C. Dehnicke, M. Meischhemke, D. Meincken-Jaeggi, Initial Monotherapy with Levetiracetam, Epilepsia, 2004, 45 (7), 134-135. 33. Mohanraj, R., P. Parker, L. Stephen, M. Brodie, Levetiracetam ÔË ÂÁËÒÚÂÌÚ̇ ÂÔËÎÂÔÒËfl: ÔÓÒÔÂÍÚË‚ÌÓ ÍÎËÌ˘ÌÓ Ì‡·Î˛‰ÂÌËÂ, Seizure, 2005, 14, 23-27. 34. Morrow, J., N01057 PGTC Study Group, Efficacy and Safety of Levetiracetam as Adjunctive Treatment in Adults and Paediatric Patients Suffering from Primary Generalized Tonic-Clonic Seizures, presented at the 7-th European Congress on Epileptology in Helsinki, 2-6 July, 2000, p. 702. 35. Panayiotopoulos, C., Juvenile Myoclonic Epilepsy, The Epileptic Seizures, Syndromes and Management, 2005, Chipping Norton, Bladon Medical Publishing, 1-24. 36. Pellock, J., Idiopathic Generalized Epilepsy: Addressing Juvenile Myoclonic Epilepsy treatment, Physician Perspective, 2005. 37. Rosenfeld, W., S. Bercovic, Efficacy and Safety of Levetiracetam as Adjunctive Treatment in Adults and Paediatric Patients Suffering from Idiopathic Generalized Epilepsy with Primary Generalized Tonic-Clonic Seizures, presented at The 58-th Annual Meeting of The American Academy of Neurology

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38. Sadek, A., A. Fix, J. French, Levetiracetam-related behavioural adverse events: a post-marketing study, Epilepsia, 2002, 43 (8), 151. 39. Sake, J., M. De Decker, G. Janssen, Efficacy of levetiracetam: an open label assessment, Epilepsia, 2002, 43 (8), 117. 40. Smith, D., A. Nicolson, Prospective analysis of the outcome of levetiracetam treatment, Epilepsia, 2002, 43 (8), 117. 41. Soni, S., M. O’Rourke, Levetiracetam in learning disability adults with tonicclonic seizures, Epilepsia, 2002, 43 (8), 181. 42. Verdru, P., A. Wajgt, J. Schiemann Delgado, S. Noachtar, Efficacy and Safety of Levetiracetam 3000 mg/d as Adjunctive Treatment in adolescents and Adults Suffering from Idiopathic Generalized Epilepsy with Myoclonic seizures, presented in Paris, 26-th ICG Proceedings.


ÑÂÍÂÏ‚Ë 2009

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

183

éË„Ë̇ÎÌË ÒÚ‡ÚËË çÄóÄãçàüí ÑÇàÉÄíÖãÖç ëàåèíéå ÇãàüÖ Çöêïì äéÉçàíàÇçàü ëèÄÑ èêà ãéçÉàíìÑàçÄãçé èêéëãÖÑÖçà èÄñàÖçíà ë èÄêäàçëéçéÇÄ ÅéãÖëí ü. ÜÂ΂, å. ê‡È˜Â‚‡, å. èÂÚÓ‚‡, ã. í‡ÈÍÓ‚ ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËfl SUMMARY INITIAL MOTOR SYMPTOM INFLUENCES COGNITIVE DECLINE IN A LONGITUDINALLY FOLLOWED PATIENTS WITH PARKINSON'S DISEASE Y.Zhelev, M. Raycheva, M. Petrova, L. Traykov University Hospital 'Alexandrovska', Neurology, Sofia, Bulgaria Parkinson's disease (PD) is often associated with cognitive impairment. Some patients exhibit severe mental deterioration over time while others show subtle cognitive decrease. Research has shown that several clinical disease parameters may correlate with cognitive deterioration. The present study examined the association between type of motor symptom at disease onset and progression of cognitive decline in a longitudinally followed group of patients with idiopathic Parkinson's disease. We investigated 27 nondemented patients with PD. All subjects underwent a comprehensive neuropsychological assessment including tests of episodic memory, language, constructional praxis, attention and executive functions. Information concerning type of initial motor symptom was obtained from subject report during clinical interview and from medical documentation. Subjects were divided into two groups - having tremor (n=15) and having bradykinesia/rigidity (n=12) as initial motor presentation of disease. We reassessed PD subjects 20±9 months later. Bradykinesia/rigidity group demonstrated significant deterioration in conceptualization subscale of Mattis Dementia Rating Scale and Digit Span Forward. Tremor group do not exhibit significant decries in performance of neuropsychological tests. In this group initial motor symptom determines progression of cognitive deterioration. A decrease in attention/executive tests performance was found in the bradykinesia/rigidity group. Tremor group showed cognitive stability over the follow-up period. The type of motor symptom at disease onset might predetermine cognitive decline in PD. êÖáûåÖ è‡ÍËÌÒÓÌÓ‚‡Ú‡ ·ÓÎÂÒÚ (èÅ) ˜ÂÒÚÓ Â Ò˙ÔÓ‚Ó‰Â̇ ÓÚ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl. çflÍÓË ÓÚ Ô‡ˆËÂÌÚËÚ ÔÓ͇Á‚‡Ú ÚÂÊ˙Í ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ ‚˙‚ ‚ÂÏÂÚÓ ‰Ó͇ÚÓ ‰Û„Ë ÔÓfl‚‡‚‡Ú Ò‡ÏÓ ÎÂÍË ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËfl Á‡ Ò˙˘Ëfl ÔÂËÓ‰ ÓÚ ‚ÂÏÂ. ç‡ÚÛÔ‡ÌËÚ ‰Ó ÏÓÏÂÌÚ‡ ËÁÒΉӂ‡ÚÂÎÒÍË ‰‡ÌÌË ÔÓ͇Á‚‡Ú ̇΢ËÂÚÓ Ì‡ ÍÓ·ˆËfl ÏÂÊ‰Û ÌflÍÓË ÓÚ ÍÎËÌ˘ÌËÚ ԇ‡ÏÂÚË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰. ç‡ÒÚÓfl˘ÂÚÓ ÔÓÛ˜‚‡Ì ËÁÒΉ‚‡ ‚˙Á͇ڇ ÏÂÊ‰Û ÚËÔ‡ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÔÓ„ÂÒËflÚ‡ ̇ ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰ ‚ ÎÓÌ„ËÚÛ‰Ë̇ÎÌÓ ÔÓÒΉÂ̇ „ÛÔ‡ Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡Ú˘̇ è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ. àÁÒΉ‚‡ÌË Ò‡ 27 ̉ÂÏÂÌÚÌË Îˈ‡ Ò èÅ. àÁÔÓÎÁ‚‡Ì‡  ÔÓ‰ӷ̇ Ì‚ÓÔÒËıÓÎӄ˘̇ ·‡ÚÂËfl ‚Íβ˜‚‡˘‡ ÚÂÒÚÓ‚Â Á‡ Ô‡ÏÂÚ, ÂÁËÍ, ÍÓÌÒÚÛÍÚË‚ÂÌ Ô‡ÍÒËÒ, ‚ÌËχÌË Ë

ÂÍÁÂÍÛÚË‚ÌË ÙÛÌ͈ËË. àÌÙÓχˆËflÚ‡ ÓÚÌÓÒÌÓ ÚËÔ‡ ‰Â·˛ÚÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Â ÔÓÎÛ˜Â̇ ÓÚ ÍÎËÌ˘ÌÓ ËÌÚÂ‚˛ Ò Ô‡ˆËÂÌÚ‡ Ë Â ÔÓÚ‚˙‰Â̇ ÓÚ Ì‡Î˘̇ڇ ωˈËÌÒ͇ ‰ÓÍÛÏÂÌÚ‡ˆËfl. ãˈ‡Ú‡ Ò‡ ‡Á‰ÂÎÂÌË Ì‡ ‰‚ „ÛÔË – Ëχ˘Ë ÚÂÏÓ Ë (n=15) Ë Ëχ˘Ë ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ (n=12) ͇ÚÓ Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. 臈ËÂÌÚËÚ ҇ ÔÓ‰ÎÓÊÂÌË Ì‡ ÔÓ‚ÚÓÌÓ ÚÂÒÚÛ‚‡Ì Ò˙Ò Ò˙˘‡Ú‡ Ì‚ÓÔÒËıÓÎӄ˘̇ ·‡ÚÂËfl ÒΉ 20±9 ÏÂÒˆ‡. ÉÛÔ‡Ú‡ Ò ‰Â·˛Ú ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ ÔÓ͇Á‡ Á̇˜ËÏ ÒÔ‡‰ ‚ ÔÓ‰Ò͇·ڇ Á‡ ÍÓ̈ÂÔÚÛ‡ÎËÁ‡ˆËfl ÓÚ Mattis Dementia Rating Scale ͇ÍÚÓ Ë Digit Span Forward. ÉÛÔ‡Ú‡ Ò ‰Â·˛Ú ÚÂÏÓ Ì ÔÓ͇Á‡ Á̇˜ËÏ ÒÔ‡‰ ̇ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÔËÎÓÊÂÌËÚ ÚÂÒÚÓ‚Â. Ç ËÁÒΉ���‡Ì‡Ú‡ „ÛÔ‡ Ô‡ˆËÂÌÚË Ì‡˜‡ÎÌËfl ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÓÔ‰ÂÎfl ÔÓ„ÂÒËflÚ‡ ̇ ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰. ìÒÚ‡ÌÓ‚fl‚‡ Ò ÒÔ‡‰ ‚ ÚÂÒÚÓ‚ÂÚ ӈÂÌfl‚‡˘Ë ‚ÌËχÌËÂ/ÂÍÁÂÍÛÚË‚ÌË ÙÛÌ͈ËË Á‡ „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÓ ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ. ÉÛÔ‡Ú‡ Ò Ì‡˜‡ÎÓ ÚÂÏÓ ÔÓ͇Á‡ ÒÚ‡·ËÎÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÓ„ÌˈËflÚ‡ Á‡ ËÁÒΉ‚‡ÌËfl ÔÂËÓ‰ ÓÚ ‚ÂÏÂ. íËÔ˙Ú ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ‚ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‚ÂÓflÚÌÓ ÏÓÊ ‰‡ Ô‰ÓÔ‰ÂÎË ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰ ÔË èÅ. ÇöÇÖÑÖçàÖ äÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl Ò˙Ô˙ÚÒÚ‚‡Ú è‡ÍËÌÒÓÌÓ‚‡Ú‡ ·ÓÎÂÒÚ (èÅ). èˆËÁÌÓÚÓ Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ÛÒÚ‡ÌÓ‚fl‚‡ ÒÏÛ˘ÂÌËfl ‚ ÍÓ„ÌˈËflÚ‡ ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ó˘Â ‚ ‡ÌÌËÚ ÂÚ‡ÔË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. çflÍÓÈ ·ÓÎÌË ·˙ÁÓ ‡Á‚Ë‚‡Ú ËÎË Á‡‰˙ηӘ‡‚‡Ú ̇΢ÌËfl ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ, ‰Ó͇ÚÓ ‰Û„Ë ÔÓ͇Á‚‡Ú ÓÚÌÓÒËÚÂÎ̇ ÒÚ‡·ËÎÌÓÒÚ ‚˙‚ ‚ÂÏÂÚÓ. ÇÎËflÌËÂÚÓ Ì‡ ÌflÍÓË ÍÎËÌ˘ÌË Ô‡‡ÏÂÚË ‚˙ıÛ ıÓ‰‡, ÔÓ„ÂÒËflÚ‡ Ë ÔÓ„ÌÓÁ‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ËÁÛ˜‡‚‡ÌÓ ÓÚ ‰ˈ‡ ‡‚ÚÓË. èÓ‰Ó·ÂÌ Â ÔÓ‰ıÓ‰˙Ú Ì‡ÒÓ˜ÂÌ Í˙Ï Ú˙ÒÂÌ ̇ ‚˙Á͇ ÏÂÊ‰Û ‰ÓÏËÌË‡˘ËÚ ÏÓÚÓÌË ÒËÏÔÚÓÏË Ë ÚÂÊÂÒÚÚ‡ ̇ ÏÌÂÒÚ˘ÌËÚ ̇Û¯ÂÌËfl. Ç ÔÓ-„ÓÎflχڇ ÒË ˜‡ÒÚ ÔÓÛ˜‚‡ÌËfl ‚ Ú‡ÁË Ì‡ÒÓ͇ ÔÓÒÓ˜‚‡Ú ̇΢ËÂÚÓ Ì‡ ÍÓ·ˆËfl ÏÂÊ‰Û ·‡‰ËÍËÌÂÁËflÚ‡ Ë/ËÎË ˄ˉÌÓÒÚÚ‡ Ë ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl, ‰Ó͇ÚÓ Á‡ ÚÂÏÓ˙Ú Ú‡Í‡‚‡ ‡ÒӈˇˆËfl ËÎË Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡ ËÎË Ì„ӂÓÚÓ ‰ÓÏËÌË‡Ì ̇ÒÓ˜‚‡ Í˙Ï ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-ÌËÒ˙Í ËÒÍ Á‡ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ (1), (2), (3), (4), (5), (6), (7), (8). Ç ÔÓ-„ÓÎflχڇ ÒË ˜‡ÒÚ ÔÓÛ˜‚‡ÌËflÚ‡ ‡ÍˆÂÌÚË‡Ú ‚˙ıÛ Ú˙ÒÂÌÂÚÓ Ì‡ ‚˙Á͇ ÏÂÊ‰Û ‡ÍÚÛ‡ÎÌËÚ ÍÎËÌ˘ÌË ÏÓÚÓÌË ÒËÏÔÚÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÓÒÓ·ÂÌÓÒÚËÚÂ Ë ÔÓ„ÂÒËflÚ‡ ̇ Ì‚ÓÔÒËıÓÎӄ˘ÌËÚ ̇Û¯ÂÌËfl. íÓÁË ÔÓ‰ıÓ‰ Ëχ ÌflÍÓË Ì‰ÓÒÚ‡Ú˙ˆË. í‡Í‡ ̇ÔËÏÂ, Ò ÔÓ„ÂÒËfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ÒÚ‡‚‡ ‚Ò ÔÓ-ÚÛ‰ÌÓ ‰‡ Ò ÓÔ‰ÂÎË ÍÓÈ ÒËÏÔÚÓÏ Â ‰ÓÏËÌË‡˘. ëΉ Á‡ÔÓ˜‚‡Ì ̇ ΘÂÌËÚÓ Ú‡ÁË ÔˆÂÌ͇ Ò Á‡ÚÛ‰Ìfl‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ÔÓ‡‰Ë ÌÂÔÓÔÓˆËÓ̇ÎÌÓÚÓ ÚÂ‡Ô‚Ú˘ÌÓ ÔÓ‚ÎËfl‚‡Ì ̇ ÓÚ‰ÂÎÌËÚ ÒËÏÔÚÓÏË. ë˙˘Ó ڇ͇ Úfl·‚‡ ‰‡ Ò Ëχ ‚ Ô‰‚ˉ, ˜Â ÒÛχ̇ ÓˆÂÌ͇, ÍÓflÚÓ ÒÂ


ÑÂÍÂÏ‚Ë 2009

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

ÔÓÎÛ˜‡‚‡ ÔË ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ Ò˙ÓÚ‚ÂÚÌËÚ Ò͇ÎË Â ÌÂËÌÙÓχÚ˂̇ Á‡ ÚÂÊÂÒÚÚ‡ ̇ ÓÚ‰ÂÎÌËfl ÒËÏÔÚÓÏ, àÁÛ˜‡‚‡ÌÂÚÓ Ì‡ ‚˙Á͇ڇ ÏÂÊ‰Û ÍÓ„ÌˈËflÚ‡ Ë Ì‡˜‡ÎÌËfl ÏÓÚÓÂÌ ÒËÏÔÚÓÏ, ‡ Ì ‡ÍÚÛ‡ÎÌËÚ ÏÓÚÓÌË ÒËÏÔÚÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ‚ÂÓflÚÌÓ Â Ì‡‰ÂʉÂÌ ÔÓ‰ıÓ‰ ÔÓ‡‰Ë Á‡Ó·Ë͇ÎflÌ ̇ ËÁ·ÓÂÌËÚ ‚˙ÁÏÓÊÌÓÒÚË Á‡ ÌÂÍÓÂÍÚ̇ ÓˆÂÌ͇ ̇ ÏÓÚÓ̇ڇ ÒËÏÔÚÓχÚË͇. åÓÊ ‰‡ Ò Ô‰ÔÓÎÓÊË, ˜Â ̇˜‡ÎÌËflÚ ‰‚Ë„‡ÚÂÎÂÌ ÒËÏÔÚÓÏ ·Ë ÏÓ„˙Î ‰‡ Ëχ Á̇˜Ëχ ÔÓ„ÌÓÒÚ˘̇ ÒÚÓÈÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰, ÓÒÓ·ÂÌÓ ‚ Ô˙‚ËÚ ÌflÍÓÎÍÓ „Ó‰ËÌË ÒΉ ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡. ñÖã ñÂÎÚ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ Â ‰‡ ËÁÒΉ‚‡ ‚˙Á͇ڇ ÏÂÊ‰Û ÚËÔ‡ ̇ ÏÓÚÓÌËfl ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÔÓÒΉ‚‡˘Ëfl ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ ‚ ÎÓÌ„ËÚÛ‰Ë̇ÎÌÓ ÔÓÒΉÂ̇ „ÛÔ‡ ̉ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡Ú˘̇ èÅ. äéçíàçÉÖçí à åÖíéÑàäÄ èÓÛ˜‚‡ÌÂÚÓ Â Ôӂ‰ÂÌÓ ‚ ÍÎËÌË͇ڇ ÔÓ Ì‚ÓÎÓ„Ëfl ̇ ìåÅÄã "ÄÎÂÍ҇̉Ó‚Ò͇" ëÓÙËfl ÔË 27 ·ÓÎÌË Ò èÅ. èÓ‰·‡ÌË Ò‡ Îˈ‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ì Ôӂ˜ ÓÚ 5 „Ó‰ËÌË. ÇÒ˘ÍË Û˜‡ÒÚÌËˆË Ò‡ ÔÓ‰Ó·ÌÓ ÍÎËÌ˘ÌÓ Ë Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌË ÔË ‚Íβ˜‚‡Ì ‚ ÔÓÛ˜‚‡ÌÂÚÓ Ë ÔÓ‚ÚÓÌÓ Ò‡ ÚÂÒÚÛ‚‡ÌË ÒΉ Ò‰ÌÓ 20,3 ± 9,4 ÏÂÒˆ‡. Ç˙Á ÓÒÌÓ‚‡ ̇ ÏÓÚÓÌËfl ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò‡ ÙÓÏË‡ÌË ‰‚ „ÛÔË – Ô‡ˆËÂÌÚË Ëχ˘Ë ·‡‡‰ËÍËÌÂÁËfl Ë/ËÎË ˄ˉÌÓÒÚ Ë „ÛÔ‡ Ò Â‰ËÌÒÚ‚ÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ. Ñˇ„ÌÓÁ‡Ú‡  ÔÓÒÚ‡‚fl̇ ‚˙Á ÓÒÌÓ‚‡ ̇ ÔÓ‰ӷ̇ ‡Ì‡ÏÌÂÁ‡, ÒÓχÚ˘ÂÌ Ë Ì‚ÓÎӄ˘ÂÌ Ô„Ή, ··Ó‡ÚÓÌË Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ËÁÒΉ‚‡ÌËfl (ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl ËÎË Ï‡„ÌËÚÌÓ ÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl ̇ ÏÓÁ˙͇), ͇ÍÚÓ Ë ÔÓ‰ӷ̇ Ì‚ÓÔÒËıÓÎӄ˘̇ ÓˆÂÌ͇. ç‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ‚Íβ˜‚‡ ÒÍËÌËÌ„Ó‚Ë Ò͇ÎË Ë ÔÓ‰ӷ̇ Ì‚ÓÔÒËıÓÎӄ˘̇ ·‡ÚÂËfl ÓÚ ÚÂÒÚÓ‚Â Ë Ò˙Í‡ÚÂÌË ‚ÂÒËË Ì‡ Ì‚ÓÔÒËıÓÎӄ˘ÌË ÏÂÚÓ‰ËÍË. é·˘ËflÚ ÍÓ„ÌËÚË‚ÂÌ Í‡Ô‡ˆËÚÂÚ Â ËÁÒΉ‚‡Ì Ò: Mini Mental State (MMS) Ë Dementia Rating Scale (DRS). è‡ÏÂÚÚ‡  ËÁÒΉ‚‡Ì‡ Ò: Digit Span (forward) Á‡ ÓˆÂÌ͇ ̇ Í‡ÚÍÓÒӘ̇ ‚Â·‡Î̇ Ô‡ÏÂÚ, ͇ÍÚÓ Ë Ò Buschke Free and Cued Selective Reminding Test (FCSRT) Á‡ ÓˆÂÌ͇ ̇ ‚Â·‡Î̇ ÂÔËÁӉ˘̇ Ô‡ÏÂÚ ˜ÂÁ ËÁÒΉ‚‡Ì ̇ Ò‚Ó·Ó‰ÌÓÚÓ ÔËÔÓÏÌflÌÂ, Ó·˘ÓÚÓ (Ò‚Ó·Ó‰ÌÓ Ë ÛÎÂÒÌÂÌÓ) ÔËÔÓÏÌflÌÂ Ë ‡ÁÔÓÁ̇‚‡ÌÂÚÓ. ÇÌËχÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË Ò‡ ËÁÒΉ‚‡ÌË Ò: Trail Making Test - A (TMT-A) Ë Ç (TMT-B); Modified Card Sorting Test (MCST); ÇÂ·‡Î̇ ÙÎÛÂÌÚÌÓÒÚ (ÙÓÌÂÏ̇), Ë Stroop Test. ê˜ڇ  ËÁÒΉ‚‡Ì‡ Ò Í‡Ú͇ ‚ÂÒËfl ̇ Boston Naming Test (BNT) Ë c ÇÂ·‡Î̇ ÙÎÛˉÌÓÒÚ (͇Ú„ÓˇÎ̇). ê‡ÁÎËÍËÚ ÏÂÊ‰Û „ÛÔËÚ ‚˙‚ ‚˙Á‡ÒÚÚ‡, Ó·‡ÁÓ‚‡ÌËÂÚÓ, ÂÁÛÎÚ‡ÚËÚ ÓÚ MMS, DRS Ë Ì‚ÓÔÒËıÓÎӄ˘ÌËÚ ÚÂÒÚÓ‚Â Ò‡ ÓˆÂÌfl‚‡ÌË Ò ÔÓÏÓ˘ Ú‡ ̇ non-paired ttest. ᇠӈÂÌ͇ ÒÚÂÔÂÌÚ‡ ̇ ÔÓÏfl̇ ‚ ÂÁÛÎÚ‡ÚËÚ ÓÚ Ì‚ÓÔÒËıÓÎӄ˘ÌËÚ ËÁÒΉ‚‡ÌËfl  ËÁÔÓÎÁ‚‡Ì paired ttest, ‚ÁÂχ˘ ‚ Ô‰‚ˉ ÂÁÛÎÚ‡ÚËÚ ÓÚ Ô˙‚ÓÚÓ Ë ‚ÚÓÓÚÓ ËÁÒΉ‚‡ÌÂ. êÖáìãíÄíà ãËÔÒ‚‡Ú ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏË ‡ÁÎËÍË ÏÂÊ‰Û „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÓÚÓÏ ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ (èÅÅ/ê) Ë „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ (èÅ-í) ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÔÓÏÂÌÎË‚ËÚ ‚˙Á‡ÒÚ (èÅ-Å/ê - 66,9 (9,3), èÅ-í - 66,5 (6,3)), Ó·‡ÁÓ‚‡ÌË (èÅ-Å/ê - 14,3 (2,9), èÅ-í - 12,9 (3,0)), ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ

184

(èÅ-Å/ê - 2,6 (1,6), èÅ-í - 2,5 (1,3)) ͇ÍÚÓ Ë ·‡ÁËÒÌËÚ ÂÁÛÎÚ‡ÚË ÓÚ Ò͇ÎËÚ Á‡ „ÎÓ·‡ÎÌÓ ÍÓ„ÌËÚË‚ÌÓ ÙÛÌ͈ËÓÌË‡Ì MMSE (èÅ-Å/ê - 27,9 (1,6), èÅ-í - 26,6 (2,0)), DRS (èÅ-Å/ê - 135,7 (4,3), èÅ-í - 131,9 (7,6)). ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏË ‡ÁÎËÍË Ë ‚ ·‡ÁËÒÌËÚ ÂÁÛÎÚ‡ÚË ÓÚ Ì‚ÓÔÒËıÓÎӄ˘ÌËÚ ÚÂÒÚÓ‚Â ÏÂÊ‰Û ‰‚ÂÚ „ÛÔË. êÂÁÛÎÚ‡ÚËÚ ÓÚ ÓˆÂÌfl‚‡˘Ëfl ‡ÍÚÛ‡ÎÌËfl Ô‡ÏÂÚÓ‚ ͇ԇˆËÚÂÚ ÚÂÒÚ (FCSRT) Ò‡ ËÁÓ·‡ÁÂÌË Í‡ÚÓ ÔÓˆÂÌÚ Ì‡ ÒÔ‡‰ ÒÔflÏÓ ·‡ÁËÒÌÓÚÓ ËÁÒΉ‚‡Ì (ÙË„. 1). чÌÌËÚ ÓÚ ‰Û„ËÚ Ì‚ÓÔÒËıÓÎӄ˘ÌË ÚÂÒÚÓ‚Â Ò‡ Ô‰ÒÚ‡‚ÂÌË Ì‡ Ú‡·Îˈ‡ 1. Ç ‰‚ÂÚ „ÛÔË Ì Ò ̇·Î˛‰‡‚‡ Á̇˜ËÏ ÒÔ‡‰ ‚ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÚÂÒÚÓ‚ÂÚ Á‡ Ó·˘Ó ÍÓ„ÌËÚË‚ÌÓ ÙÛÌ͈ËÓÌË‡Ì ͇ÍÚÓ Ë ‚ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÚÂÒÚ‡ Á‡ ‚Â·‡Î̇ ÂÔËÁӉ˘̇ Ô‡ÏÂÚ (FCSRT). èÓ ÓÚÌÓ¯ÂÌË ̇ ÚÂÒÚÓ‚ÂÚÂ, ÍÓËÚÓ ËÁÒΉ‚‡Ú ‡Á΢ÌË ‡ÒÔÂÍÚË Ì‡ ‚ÌËχÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË, Ò ̇·Î˛‰‡‚‡Ú ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏË ‡ÁÎËÍË ÔË Digit Span Forward Ë ÂÍÁÂÍÛÚ˂̇ڇ ÔÓ‰Ò͇· ̇ DRS (ÒÔÓÒÓ·ÌÓÒÚ Á‡ ÙÓÏË‡Ì ̇ ÍÓ̈ÂÔˆËË) ‰ËÌÒÚ‚ÂÌÓ ‚ „ÛÔ‡Ú‡ Ëχ˘‡ ‰Â·˛ÚÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ·‡‰ËÍËÌÂÁËfl Ë/ËÎË ˄ˉÌÓÒÚ. ÉÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ ÔÓ͇Á‚‡ ÒÚ‡·ËÎÌÓÒÚ ‚˙‚ ‚ÂÏÓÚÓ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÔËÎÓÊÂÌËÚ ÍÓ„ÌËÚË‚ÌË Ò͇ÎË. éÅëöÜÑÄçÖ Ä̇ÎËÁ˙Ú Ì‡ ÂÁÛÎÚ‡ÚËÚ ÓÚ ËÁÒΉ‚‡ÌËÚ „ÛÔË ÔÓ͇Á‚‡Ú, ˜Â ÏÓÚÓÌËflÚ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ‚ÂÓflÚÌÓ Ëχ ÔÓ„ÌÓÒÚ˘ÌÓ Á̇˜ÂÌË ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÔÓÒΉ‚‡˘Ëfl ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰. 臈ËÂÌÚË, ˜ËËÚÓ Ô˙‚Ë ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ·‡‰ËÍËÌÂÁËfl Ë/ËÎË ˄ˉÌÓÒÚ, ËÏ‡Ú ÚẨÂ̈Ëfl Á‡ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏ ÒÔ‡‰ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÌflÍÓË ÒÙÂË Ì‡ Íӄ퇷Îˈ‡ 1. êÂÁÛÎÚ‡ÚË ÓÚ Ì‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ÚÂÒÚÛ‚‡Ì ÔË Ô˙‚ÓÚÓ (1) Ë ÔË ÔÓ‚ÚÓÌÓÚÓ (2) ËÁÒΉ‚‡Ì ̇ Ô‡ˆËÂÌÚËÚÂ Ò è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ Ò ‰Â·˛Ú ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ (èÅ-Å/ê) Ë ÚÂÏÓ (èÅ-í) íÂÒÚÓ‚Â

èÅ-Å/ê 1 93,3 (59,1) 172,5 (81,6) 29,3 (13,8 ) 4,8 (2,0) 37,3 (0,7) 9,6 (3,8)

(n=12) 2 97,6 (53,0) 211,5 (92,5) 36,1 (12,2) 4,9 (1,9) 34,5 (3,2)* 9,3 (4,6)

èÅ-í (n=15) 1 2 77,6 (37,4) 85,9 (42,6) 201,9 (86,8) 223,6 (96,4) 28,8 (11,8) 30,1 (12,5) 4,9 (1,6) 4,6 (2,2) 36,2 (3,3) 36,4 (2,3) 7,7 (2,5) 7,8 (4,2)

TMT Ä (ÒÂÍ.) TMT B (ÒÂÍ.) Stroop Test MCST (͇Ú.) CONCEPT ÇÂ·‡Î̇ (å) ÙÎÛˉÌÓÒÚ Digit span 4,0 (0,7) 3,7 (0,8) 3,5 (0,8) 3,8 (0,9) (backward) Digit Span 5,7 (0,9) 5,1 (0,7)* 5,2 (1,1) 5,3 (0,9) (forward) ÇÂ·‡Î̇ 18,8 (5,2) 16,8 (5,3) 17,1 (4,4) 16,4 (5,0) ÙÎÛˉÌÓÒÚ (ÊË‚ÓÚÌË) BNT 14,3 (1,0) 14,8 (0,6) 14,5 (1,3) 14,4 (1,0) чÌÌËÚ ҇ Ò‰ÌÓ‡ËÚÏÂÚ˘ÌË (SD); TMT Ä = Trail Making Test Ä; TMT B = Trail Making Test B; MCST = Modified Card Sorting Test (͇Ú„ÓËË); CONCEPT = DRS-Conceptualization; BNT = Boston Naming Test; *-P<0.05 ÌËÚË‚ÌÓÚÓ ÙÛÌ͈ËÓÌË‡ÌÂ. äÓÌÍÂÚÌÓ ÚÓ‚‡ Ò‡ ‚˙ÁÏÓÊÌÓÒÚËÚ Á‡ ÙÓÏË‡Ì ̇ ÍÓ̈ÂÔˆËË (‚ ̇¯ÂÚÓ ÔÓÛ˜‚‡Ì ËÁÒΉ‚‡Ì‡ Ò ÔÓ‰Ò͇· ̇ DRS) Ë ‚ÌËχÌËÂ/Í‡ÚÍÓÒӘ̇ Ô‡ÏÂÚ. éÚ ‰Û„‡ ÒÚ‡Ì‡, ‚ÁËχÈÍË Ô‰‚ˉ ÎËÔÒ‡Ú‡ ̇ ÒÔ‡‰ ‚ „ÛÔ‡Ú‡ ̇ Îˈ‡Ú‡ Ëχ˘Ë ÏÓÚÓÂÌ ‰Â·˛Ú


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

ÑÂÍÂÏ‚Ë 2009

ë‚è = Ò‚Ó·Ó‰ÌÓ ÔËÔÓÏÌflÌÂ, é·è= Ó·˘Ó ÔËÔÓÏÌflÌ (Ò‚Ó·Ó‰ÌÓ Ë ÔÓ‰ÔÓÏӄ̇ÚÓ), ë‚éÚ‰ = Ò‚Ó·Ó‰ÌÓ ÓÚ‰‡Î˜ÂÌÓ ÔËÔÓÏÌflÌÂ, é·éÚ‰ = Ó·˘Ó ÓÚ‰‡Î˜ÂÌÓ ÔËÔÓÏflÌÂ, ê‡Á = ‡ÁÔÓÁ̇‚‡Ì îË„.1 ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ÚÂÏÓ, ·ËıÏ ÏÓ„ÎË ‰‡ Á‡Íβ˜ËÏ, ˜Â ÚÓÁË ‚ˉ ÒËÏÔÚÓÏ ‚ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â Ò‚˙Á‡Ì Ò ÔÓÒΉ‚‡˘‡ ÓÚÌÓÒËÚÂÎ̇ ÍÓ„ÌËÚ˂̇ Ò˙ı‡ÌÂÌÓÒÚ. éÚ˜ËÚ‡ÈÍË ÔÓ‰·Ó‡ ̇ Îˈ‡Ú‡ ‚Íβ˜ÂÌË ‚ ̇ÒÚÓfl˘Ëfl ‡Ì‡ÎËÁ, ‡ ËÏÂÌÌÓ ‚ÂÏÂ‚Ó ÓÚÒÚÓflÌË ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‰Ó Ì‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì Ì Ôӂ˜ ÓÚ 5 „Ó‰ËÌË, ÚÓ Ú‡ÁË ËÌÚÂÔÂÚ‡ˆËfl Ëχ ÔÓ„ÌÓÒÚ˘̇ ‚ÂÏ‚‡ ‰ÓÒÚÓ‚ÂÌÓÒÚ ‚ Ô˙‚ËÚ ÌflÍÓÎÍÓ „Ó‰ËÌË ÒΉ Ô˙‚ËÚ ÍÎËÌ˘ÌË ÔÓfl‚Ë Ì‡ ·ÓÎÂÒÚÚ‡. èÓÎÛ˜ÂÌËÚ ÂÁÛÎÚ‡ÚË Ò‡ ‚ ÛÌËÒÓÌ Ò Ôӂ˜ÂÚÓ ÔÓÛ˜‚‡ÌËfl ‰ÂÏÓÌÒÚË‡˘Ë ̇΢ËÂÚÓ Ì‡ ‚˙Á͇ ÏÂÊ‰Û ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ Ë ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl ͇ÍÚÓ Ë ‡ÒӈˇˆËfl ̇ ÚÂÏÓ‡ Ò ÓÚÌÓÒËÚÂÎ̇ Ò˙ı‡ÌÂÌÓÒÚ Ì‡ ÍÓ„ÌˈËflÚ‡ (9), (2), (10). åÓÊ ‰‡ Ò Ô‰ÔÓÎÓÊË, ˜Â ÍÓ„ÌËÚË���ÌËflÚ ‰ÂÙˈËÚ ÔË èÅ Ò ÓÔ‰ÂÎfl ‰Ó „ÓÎflχ ÒÚÂÔÂÌ ÓÚ Ò˙˘ËÚ ԇÚÓÙËÁËÓÎӄ˘ÌË ÏÂı‡ÌËÁÏË, ÍÓËÚÓ Ò ‡ÒÓˆËË‡Ú Ò ·‡‰ËÍËÌÂÁËflÚ‡ Ë ˄ˉÌÓÒÚÚ‡. ÑÓÔ‡ÏËÌÓ‚ËflÚ ‰ÂÙˈËÚ ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË Ì‡Û¯‡‚‡ ÙÛÌ͈ËËÚ ̇ ÌflÍÓÎÍÓ Ì‚Ӈ̇ÚÓÏ˘ÌË Í˙„‡ Ò‚˙Á‚‡˘Ë ÒÚˇÚÛχ Ò ÔÓ‰ÍÓÓ‚Ë fl‰‡ Ë ÍÓÚË͇ÎÌËÚ ӷ·ÒÚË. è‰ÔÓ·„‡ ÒÂ, ˜Â ‚ÒÂÍË ÓÚ Í‡‰Ë̇ÎÌËÚ ÒËÏÔÚÓÏË Ì‡ èÅ Ò ‡Á‚Ë‚‡ ÌÂÁ‡‚ËÒËÏÓ, ͇ÚÓ ÒΉÒÚ‚Ë ̇ ÙÛÌ͈ËÓ̇Î̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ì‡ ÓÔ‰ÂÎÂÌË Ì‚ÓӇ̇ÚÓÏ˘ÌË ÔÓ‰Í˙„Ó‚Â (11), (12). Ç˙ÁÏÓÊÌÓ Â Ô‡ÚÓÙËÁËÓÎÓ„ËflÚ‡ ̇ ÚÂÏÓ‡ ‰‡ ‡Ì„‡ÊË‡ Ô˙Úˢ‡, ÍÓËÚÓ Ì ҇ ‰ËÂÍÚÌÓ Ò‚˙Á‡ÌË Ò ÍÓ„ÌËÚË‚ÌÓÚÓ ÙÛÌ͈ËÓÌË‡ÌÂ, ‰Ó͇ÚÓ ·‡‰ËÍËÌÂÁËflÚ‡ Ë ˄ˉÌÓÒÚÚ‡ ‰‡ Ò‡ Ì‚Ӈ̇ÚÓÏ˘ÌÓ Ë Ì‚ÓωˇÚÓÌÓ Ò‚˙Á‡ÌË Ò ÔÓÂ͈ËË Í˙Ï ‰ÓÁÓ·ÚÂ‡ÎÌËfl ÔÂÙÓÌÚ‡ÎÂÌ Í˙„ Û˜‡ÒÚ‚‡˘ ‚ ÍÓ„ÌËÚË‚ÌËÚ ÔÓˆÂÒË (13), (14). ïËÔÓÚÂÁ‡Ú‡ Á‡ ‰ÓÔ‡ÏËÌÂ„˘ÌËfl ‰ÂÙˈËÚ Í‡ÚÓ Ì‡˜ËÌ ‰‡ Ò ӷflÒÌflÚ ÒÔˆËÙ˘ÌËÚ ◊ÙÓÌÚ‡ÎÌË” ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ÔË Ì‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË ‚ ̇˜‡ÎÓÚÓ Ì‡ èÅ, ÍÓ„‡ÚÓ ÔÓˆÂÒ˙Ú Ì‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl Ò ÒÏflÚ‡ Á‡ Ô‰ËÏÌÓ Ó„‡Ì˘ÂÌ ‚ ‰ÓÔ‡ÏÂÌÂ„˘ÌËÚ Ì‚ÓÌË, ËÁ„ÎÂʉ‡ Îӄ˘̇. Ç ÎÓÌ„ËÚÛ‰Ë̇ÎÌÓ ÔÓÛ˜‚‡Ì ‚˙ıÛ 20 Ô‡ˆËÂÌÚË Ò èÅ Ò ÓÚÌÓÒËÚÂÎÌÓ ÒÍÓÓ ÔÓÒÚ‡‚Â̇ ‰Ë‡„ÌÓÁ‡  ÓÚ˜ÂÚÂÌÓ ÌÂÔ˙ÎÌÓ ÔÓ‰Ó·ÂÌË ‚ ÂÁÛÎÚ‡ÚËÚ ÓÚ Ôӂ˜ÂÚÓ Ì‚ÓÔÒËıÓÎӄ˘ÌË ÚÂÒÚÓ‚Â, ͇ÍÚÓ Ë Ì‡ ÏÓÚÓÌËÚ ÒËÏÔÚÓÏË, ÒΉ ̇˜‡ÎÓÚÓ Ì‡ ÚÂ‡ÔËfl Ò Î‚ӉÓÔ‡ Ë ÔÂ„ÓÎˉ (15). èÓ‰Ó·ÂÌËÂÚÓ Â ÓÚ˜ÂÚÂÌÓ Ô‰ËÏÌÓ Á‡ ◊ÙÓÌÚ‡ÎÌËÚ” ÚÂÒÚÓ‚Â, ÌÓ Ò˙˘Ó Ë Á‡ ÚÂÒÚÓ‚Â ÓˆÂÌfl‚‡˘Ë ‰˙΄ÓÒӘ̇ڇ ‚Â·‡Î̇ Ë ÁËÚÂÎ̇ Ô‡ÏÂÚ, Ë ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÒÔÓÒÓ·ÌÓÒÚË. î‡ÍÚ˙Ú, ˜Â ‚˙ÔÂÍË ÔÓ‰Ó·ÂÌËÚ ÍÓ„ÌËÚË‚ÌË ÒÔÓÒÓ·ÌÓÒÚË Ô‡ˆËÂÌÚËÚ Ì ‰ÓÒÚË„‡Ú ÌÓχÎÌËÚ ԇ‡ÏÂÚË, ͇ÍÚÓ Ë ÎËÔÒ‡Ú‡ ̇ ÔÓÏfl̇ ‚ ÌflÍÓË ÓÚ ÚÂÒÚÓ‚ÂÚ ‚˙ÔÂÍË Î˜ÂÌËÂÚÓ, Ò‡ Ô˘Ë̇ ‰‡ Ò Ô‰ÔÓÎÓÊË ˜Â ÍÓ„ÌËÚË‚ÌËflÚ ‰ÂÙˈËÚ ÔË èÅ Ì  ËÁˆflÎÓ ÒΉÒÚ‚Ë ÓÚ ‰ÓÔ‡ÏËÌÂ„˘ÌÓÚÓ ËÁ˜ÂÔ‚‡ÌÂ, ‡ ‚Â-

185

ÓflÚÌÓ Ò ̇ÏÂÒ‚‡Ú Ë Ì‰ÓÔ‡ÏËÌÂ„˘ÌË Ù‡ÍÚÓË (15). чÌÌËÚ ÓÚ ÚÓ‚‡ ÔÓÛ˜‚‡Ì ÓÚ ˜‡ÒÚË Ò‡ ‚ ÔÓÚË‚Ó‚ÂÒ Ò ‰‡ÌÌËÚ ÓÚ ÔÓÛ˜‚‡ÌÂ Ò ÂÚÓÒÔÂÍÚË‚ÂÌ ‰ËÁ‡ÈÌ Ôӂ‰ÂÌÓ ‚˙ıÛ Ô‡ˆËÂÌÚË ‚ ÔÓ-̇Ô‰̇· Ù‡Á‡ ̇ èÅ. Ç ÌÂ„Ó ËÁÒΉӂ‡ÚÂÎËÚ Ò˙Ó·˘‡‚‡Ú Á‡ Á̇˜Ëχ Ô‰ËÍÚ˂̇ ÓÎfl ̇ ‚˙Á‡ÒÚÚ‡ Ë ÚÂÏÓ˙Ú Í‡ÚÓ Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ Á‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl (16). ëÔÓ‰ ÚÂıÌËÚ ÂÁÛÎÚ‡ÚË, Ô‡ˆËÂÌÚËÚÂ Ò ÚÂÏÓ ÔË ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÔÓ-‚ÂÓflÚÌÓ ‰‡ ‡Á‚ËflÚ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ÓÚÍÓÎÍÓÚÓ Ô‡ˆËÂÌÚË Ëχ˘Ë ‡ÍËÌÂÁËfl ËÎË ˄ˉÌÓÒÚ ‚ ̇˜‡ÎÓÚÓ Ì‡ ÒÚ‡‰‡ÌËÂÚÓ. íÂÁË Ì‡Û¯ÂÌËfl Ò‡ ·ËÎË Ó„‡Ì˘ÂÌË ‚ ÒÙÂËÚ ̇ Ô‡ÏÂÚ/‚ÌËχÌËÂ Ò ÏËÌËχÎÌË ËÎË ÎËÔÒ‚‡˘Ë ÒÏÛ¯ÂÌËfl ‚ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÒÔÓÒÓ·ÌÓÒÚË Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË Ò˙ÓÚ‚ÂÚÌÓ. ë‰̇ڇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔË Îˈ‡Ú‡ ‚ÁÂÎË Û˜‡ÒÚË ‚ ÔÓÛ˜‚‡ÌÂÚÓ Â 12,7 „Ó‰ËÌË. ç‚ÓÔ‡ÚÓÎӄ˘ÌË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú ‰Â„ÂÌÂ‡ˆËfl ‚ ÏÌÓÊÂÒÚ‚Ó ÔÓ‰ÍÓÓ‚Ë Ë ÍÓÓ‚Ë Ì‚Ó̇ÎÌË ÒËÒÚÂÏË ‚ ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (17). Ç˙ÁÏÓÊÌÓ Â Ô˙‚˘̇ڇ ‰Â„ÂÌÂ‡ˆËfl ̇ Ì‚Ӈ̇ÚÓÏ˘ÌËÚ ÒÚÛÍÚÛË Ì‡ ‰ÓÔ‡ÏËÌÂ„˘̇ڇ Ë Ì‰ÓÔ‡ÏËÌÂ„˘̇ڇ ÒËÒÚÂχ ‰‡ Ò‡ ‰‚‡ ÌÂÁ‡‚ËÒËÏË ÔÓˆÂÒ‡, ͇ÚÓ ‚ÚÓËflÚ ‰‡ Ò ‡Á‚Ë‚‡ Ò ÔÓ-ÌËÒ͇ ÒÍÓÓÒÚ ÔË Ë‰ËÓÔ‡Ú˘̇ڇ èÅ. ÄÍÓ ÔËÎÓÊËÏ ÚÓ‚‡ ‰ÓÔÛÒ͇Ì Í˙Ï Ì‡¯ËÚ ÂÁÛÎÚ‡ÚË, ÚÓ ÛÒÚ‡ÌÓ‚ÂÌËflÚ ÒÔ‡‰ ‚ „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ ·Ë ÏÓ„˙Î ‰‡ Ò ‰˙ÎÊË ÓÒÌÓ‚ÌÓ Ì‡ ÔÓÏÂÌË ‚ ‰ÓÔ‡ÏËÌÂ„˘̇ڇ Ì‚ÓωˇˆËfl, ‰Ó͇ÚÓ ÂÁÛÎÚ‡ÚËÚ ̇ Vingerhoets Ë Ò˙‡‚ÚÓË (16) ‰‡ Ò‡ ÂÁÛÎÚ‡Ú Ì‡ ·‡‚ÌÓ ‡Á‚Ë‚‡˘ Ò ÔÓˆÂÒ, ˜ËËÚÓ ÍÎËÌ˘ÂÌ ÂÙÂÍÚ Ò ËÁfl‚‡‚fl‚‡ ‚ ÔÓ-Í˙ÒÌËÚ ÂÚ‡ÔË ÓÚ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë Â ‚ÂÓflÚÌÓ ÒΉÒÚ‚Ë Ô‰ËÏÌÓ Ì‡ ÒÏÛ˘ÂÌËfl ‚ ̉ÓÔ‡ÏËÌÂ„˘ÌËÚ ÒËÒÚÂÏË. ëÔÓ‰ ÌflÍÓË ‡‚ÚÓË, Á‡ ‚˙Á͇ڇ ÏÂÊ‰Û ÏÓÚÓÌËfl ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ Ëχ Á̇˜ÂÌË ·ÚÂ‡ÎËÁ‡ˆËflÚ‡ ̇ ÒËÏÔÚÓÏËÚ ÔË Ô˙‚ËÚ ÔÓfl‚Ë Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. Katzen Ë Ò˙‡‚ÚÓË ÛÒÚÓÌÓ‚fl‚‡Ú ˜Â Ô‡ˆËÂÌÚË Ò ·‡‰ËÍËÌÂÁËfl ËÎË ˄ˉÌÓÒÚ Í‡ÚÓ Ô˙‚Ë ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÔË ÚÂÒÚÛ‚‡Ì ËÏ‡Ú ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÒÚ‡Ì‡Ú‡ (Îfl‚Ó ËÎË ‰flÒÌÓ) ̇ Úflı̇ڇ ÔÓfl‚‡, ‰Ó͇ÚÓ Ô‡ˆËÂÌÚË Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ - ÔÓ͇Á‚‡Ú Ì‚ÓÔÒËıÓÎӄ˘ÂÌ ‰ÂÙˈËÚ Ò‡ÏÓ ‚ ÒÎÛ˜‡ËÚÂ Ò Î‚ÓÒÚ‡ÌÌÓ Á‡Òfl„‡Ì (18). Ç Ò˙˘ÓÚÓ ÔÓÛ˜‚‡Ì ‰ËÌÒÚ‚ÂÌÓ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ÂÒÌÓÒÚ‡ÌÌÓ Ì‡˜‡ÎÓ Ì‡ ÚÂÏÓ‡ ÎËÔÒ‚‡ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰. èÓ‡‰Ë χÎÍËfl ·ÓÈ Ì‡ Ë̉˂ˉËÚ ‚ „ÛÔËÚ ÓÚ Ì‡ÒÚÓfl˘ÂÚÓ ÔÓÒÔÂÍÚË‚ÌÓ Ì‡·Î˛‰ÂÌË  Ì‚˙ÁÏÓÊÌÓ ‰‡ Ò ̇Ô‡‚Ë ÒÚ‡ÚËÒÚ˘ÂÒÍË ‰ÓÒÚÓ‚ÂÂÌ ‡Ì‡ÎËÁ ÔÓ·Ë‡˘ ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ‚ÎËflÌË ̇ ·ÚÂ‡ÎËÁ‡ˆËflÚ‡ ̇ ÚÂÏÓ‡ ÔË Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‚˙ıÛ ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰. äÓ„‡ÚÓ Ó·Ò˙ʉ‡Ï Á̇˜ÂÌËÂÚÓ Ì‡ ̇˜‡ÎÌËfl ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Í‡ÚÓ ‚˙ÁÏÓÊÂÌ Ô‰ËÍÚÓ ‚ ÔÓ-ÓÚ‰‡Î˜ÂÌ ‚ÂÏÂ‚Ë Ô·Ì, Úfl·‚‡ ‰‡ Ó·˙ÌÂÏ ‚ÌËχÌËÂ Ë Ì‡ Ù‡ÍÚ‡, ˜Â ‰‚Ë„‡ÚÂÎÌËÚ ÒËÏÔÚÓÏË ‚ ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ú˙ÔflÚ ‰Ë̇ÏË͇ ÔË ÌflÍÓË ·ÓÎÌË. í‡Í‡ ̇ÔËÏÂ Hershey Ë Ò˙‡‚ÚÓË ÛÒÚ‡ÌÓ‚fl‚‡Ú ˜Â ÓÚ 27 ·ÓÎÌË Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ, ÔË ÔÓÒΉfl‚‡Ì Á‡ 7 „Ó‰ËÌË Ò‡ÏÓ 16 ÔÓ‰˙Îʇ‚‡Ú ‰‡ ËÏ‡Ú ‰ÓÏËÌË‡Ì‡ ÓÚ ÚÂÏÓ‡ ÒËÏÔÚÓχÚË͇ (19). Ç Ò˙˘‡Ú‡ ‡Á‡·ÓÚ͇ ËÁÒΉӂ‡ÚÂÎËÚ Ô‡‚flÚ Á‡Íβ˜ÂÌËÂ, ˜Â Ì ҇ÏÓ ÚÂÏÓ˙Ú ÔË Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ‡ Ë Ì„ӂÓÚÓ ‰ÓÏËÌË‡Ì ‚ ÒΉ‚‡˘ËÚ ÌflÍÓÎÍÓ „Ó‰ËÌË Ëχ Á̇˜ÂÌË ‰Ó ËÁ‚ÂÒÚ̇ ÒÚÂÔÂÌ Á‡ ‰Ó·Ó͇˜ÂÒÚ‚ÂÌÓÚÓ ÍÓ„ÌËÚË‚ÌÓ ÔÓÚ˘‡ÌÂ. Ç Ì‡¯ÂÚÓ ÔÓÛ˜‚‡Ì ÚÓ‚‡  Ò˙Ó·‡ÁÂÌÓ ˜ÂÁ ÔËÎÓÊÂ̇ ÒÂÎÂ͈Ëfl ̇ ·ÓÎÌËÚÂ. äÓÌÍÂÚÌÓ ÌË ËÁÍβ˜‚‡Ï ÓÚ ‡Ì‡ÎËÁ‡ Îˈ‡, Á‡ ÍÓËÚÓ Ëχ ‰‡ÌÌË, ˜Â ÚÂÏÓ˙Ú Â ·ËÎ ÔÓÒΉ‚‡Ì ÓÚ ˄ˉÌÓÒÚ ËÎË Ì‡Û¯ÂÌËfl ‚ ÔÓıӉ͇ڇ ‚ ‡ÏÍËÚ ̇ Ô˙‚‡Ú‡ „Ó‰Ë̇ ÓÚ Ì„ӂÓÚÓ Ì‡˜‡ÎÓ. èÓ ÚÓÁË Ì‡˜ËÌ Â Ì‡Ô‡‚ÂÌ ÓÔËÚ ‰‡ Ò ̇χÎË ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ ÍÓÌ‚ÂÁËfl ̇ ÚÂ-


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

ÑÂÍÂÏ‚Ë 2009

ÏÓ ‰Â·˛ÚË‡˘‡Ú‡, ‚ ‰Û„ ÚËÔ ‰ÓÏËÌË‡˘‡ ÒËÏÔÚÓχÚË͇ ‚ ÒΉ‚‡˘ËÚ ÌflÍÓÎÍÓ „Ó‰ËÌË. ։̇ ÓÚ Ì‡È-˜ÂÒÚÓ Ò˙Ó·˘‡‚‡ÌËÚ ÓÒÓ·ÂÌÓÒÚË Ì‡ ÍÓ„ÌËÚË‚ÌËfl ÒÔ‡‰ ÔË ‡Ì̇ èÅ Â ÂÍÁÂÍÛÚË‚ÌËfl ‰ÂÙˈËÚ (20). Ç Ì‡ÒÚÓfl˘ÂÚÓ ÔÓÛ˜‚‡Ì ̇΢ËÂÚÓ Ì‡ ÒÔ‡‰ ‚ Ú‡ÁË ÍÓ„ÌËÚ˂̇ ӷ·ÒÚ Â Ó·‚˙Á‡ÌÓ Ò‡ÏÓ Ò „ÛÔ‡Ú‡ ̇ Ô‡ˆËÂÌÚËÚÂ, ˜ËËÚÓ Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Â ·‡‰ËÍËÌÂÁËfl/˄ˉÌÓÒÚ. çÂÓ·ıÓ‰ËÏÓ Â ‰‡ Ò ÛÚÓ˜ÌË, ˜Â Ôӂ‰ÂÌËfl ‡Ì‡ÎËÁ ÓÚ˜ËÚ‡ ‰ËÌÒÚ‚ÂÌÓ Ì‡Î˘ËÂÚÓ ËÎË ÎËÔÒ‡Ú‡ ̇ Á̇˜ËÏ ÒÔ‡‰ ‚ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÓÚ‰ÂÎÌËÚ Ì‚ÓÔÒËıÓÎӄ˘ÌË ÚÂÒÚÓ‚Â, Ú.Â. Ì Ò ËÁÍβ˜‚‡ ̇΢ËÂÚÓ Ì‡ ÂÍÁÂÍÛÚ˂̇ ‰ËÒÙÛÌ͈Ëfl ÔË Ô‡ˆËÂÌÚËÚÂ Ò Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ, ÌÓ ÚÓÁË ‰ÂÙˈËÚ Â ÓÚÌÓÒËÚÂÎÌÓ ÒÚ‡ˆËÓÌË‡Ì ‚˙‚ ‚ÂÏ‚Ëfl ÓÚflÁ˙Í, ‚˙ıÛ ÍÓÈÚÓ Â ËÁ‚˙¯ÂÌÓ Ì‡·Î˛‰ÂÌËÂÚÓ. ê‡Á„ÎÂʉ‡ÈÍË ÔÓ ÚÓÁË Ì‡˜ËÌ ÂÁÛÎÚ‡ÚËÚ ·ËıÏ ÏÓ„ÎË ‰‡ Ô‰ÔÓÎÓÊËÏ, ˜Â ‚ ‰˙΄ÓÒÓ˜ÂÌ ÔÎ‡Ì „ÛÔ‡Ú‡ ̇ èÅ-Åê ˘Â ‰ÂÏÓÌÒÚË‡ ÔÓ-„ÓÎflχ ÒÚÂÔÂÌ Ì‡ ÍÓ„ÌËÚ˂̇ ‰Â„‡‰‡ˆËfl ÓÚÍÓÎÍÓÚÓ „ÛÔ‡Ú‡ Ëχ˘‡ ̇˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ. Ç Á‡Íβ˜ÂÌËÂ, ̇¯ËÚ ÂÁÛÎÚ‡ÚË ÔÓ͇Á‚‡Ú χÎ˙Í, ÌÓ Á̇˜ËÏ ÒÔ‡‰ ‚ ‰‚‡ ÓÚ ÍÓ„ÌËÚË‚ÌËÚ ÚÂÒÚÓ‚Â Ò‡ÏÓ Á‡ „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ˄ˉÌÓÒÚ Ë/ËÎË ·‡‰ËÍËÌÂÁËfl. íÓÁË ÒÔ‡‰  ‚ÂÓflÚ̇ Ó·‚˙Á‡Ì Ô‰ËÏÌÓ Ò ‰ÂÙˈËÚ ‚ ‰ÓÔ‡ÏËÌÂ„˘̇ڇ ωˇˆËfl. 臈ËÂÌÚË Ò ‰Â·˛ÚÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ ËÏ‡Ú ÔÓ-‰Ó·‡ ÔÓ„ÌÓÁ‡ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÓ„ÌËÚË‚ÌÓÚÓ ÙÛÌ͈ËÓÌË‡Ì ÔÓÌ ‚ Ô˙‚ËÚ ÌflÍÓÎÍÓ „Ó‰ËÌË ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ê‡Á‰ÂÎflÌÂÚÓ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ò èṦ ÔÓ‰„ÛÔË ÒÔÓ‰ ‰Â·˛ÚÌËfl ÒËÏÔÚÓÏ Â Û‰Ó·ÂÌ ‚ Ô‡ÍÚË͇ڇ ÔÓ‰ıÓ‰ Ò Ì‡‰ÂʉÌÓ Ô‰ËÍÚË‚ÌÓ Á̇˜ÂÌË ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÓ„ÌËÚË‚ÌÓÚÓ ÙÛÌ͈ËÓÌË‡ÌÂ. ãàíÖêÄíìêÄ 1. 2. 3. 4.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- ü‚Ó ÜÂ΂, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ·ÛÎ. “ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËfl 1431 ÚÂÎ: 02/92 30 544 Â-mail: yavor_zhelev@mail.bg


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187

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ÍÎËÌË͇, ìåÅÄã ◊ÄÎÂÍ҇̉Ó‚Ò͇”, åî –ëÓÙËfl Å˙΄‡ÒÍË ìÌË‚ÂÒËÚÂÚ ◊ ä‡Ú‰‡ ÔÓ ÍÓ„ÌËÚË‚ÌË Ì‡ÛÍË Ë ÔÒËıÓÎÓ„Ëfl”

SUMMARY The myotonic dystrophies (myotonic dystrophy type 1, DM-1 and myotonic dystrophy type 2, PROMM/DM-2) are highly variable autosomal dominant, multisystem disorders characterised by myotonic phenomenons, involvement of the neuromuscular, cardiovascular and the endocrine systems as well as by abnormalities in cognition and personality. Indeed, CNS involvement in DM1 had been observed since the first descriptions of the disease, ranging from a condition of mental retardation, characteristic of congenital cases, to some behavioral and cognitive changes frequently seen in adult classical forms. The occurrence of different patterns of cognitive impairment in patients with DM1 is probably due to: altered expression of genes regulating later stages of brain development in congenital forms and affected correct splicing of tau proteins in neurons of frontotemporal cortex in classical forms. The visual-spatial abilities are affected in patients with PROMM in a milder extend compared to those with Steinert disease. KEY WORDS: myotonic dystrophies, cognitive and behavioral disorders, tau-proteins, frontotemporal cortex. êÖáûåÖ åËÓÚÓÌ˘ÌËÚ ‰ËÒÚÓÙËË ÚËÔ I (·ÓÎÂÒÚ Ì‡ Steinert) Ë ÚËÔ II (PROMM) Ò‡ „ÂÌÂÚ˘ÌË ÏÛÎÚËÒËÒÚÂÏÌË Á‡·ÓÎfl‚‡ÌËfl Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ Ë ÌÂÔ˙Î̇ ÔÂÌÂÚ‡ÌÚÌÓÒÚ, ÍÓËÚÓ Ò ı‡‡ÍÚÂËÁË‡Ú ÍÎËÌ˘ÌÓ Ò ÏËÓÚÓÌ˘ÌË ÙÂÌÓÏÂÌË (Á‡ÚÛ‰ÌÂ̇ ·ÍÒ‡ˆËfl ÒΉ ÒËÎÌÓ ÏÛÒÍÛÎÌÓ Ò˙Í‡˘ÂÌËÂ), ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl, ͇ڇ‡ÍÚ‡, ͇‰ËÓÏËÓÔ‡ÚËfl, „Ó̇‰Ì‡ ‡ÚÓÙËfl Ë ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl. èÓfl‚Ë ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÔË ÚÂÁË Ô‡ˆËÂÌÚË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ó˘Â ÔË Ô˙‚ËÚ ÓÔËÒ‡ÌËfl ̇ ÏËÓÚÓÌ˘ÌË ‰ËÒÚÓÙËË, ͇ÚÓ Ú ÏÓ„‡Ú ‰‡ ‚‡Ë‡Ú ÓÚ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì ÔË ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ ‰Ó ÒÔˆËÙ˘ÌË ÍÓ„ÌËÚË‚ÌË Ë Ôӂ‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl, ˜ÂÒÚË ÔË Í·Ò˘ÂÒÍËÚ ÙÓÏË. LJˇˆËËÚ ‚ ÍÓ„ÌËÚË‚ÌËfl ÔÓÙËÎ ÔË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ ̇ Steinert ̇È-‚ÂÓflÚÌÓ Ò‡ ÒΉÒÚ‚Ë ÓÚ ÒÏÛ˘ÂÌË ‚ ÂÍÒÔÂÒËflÚ‡ ̇ „ÂÌË, „ÛÎË‡˘Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÌËÚ ÒÚÛÍÚÛË ÔË ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ Ë Ì‡Û¯ÂÌ ÒÔ·ÈÒËÌ„ ̇ Ú‡Û-ÔÓÚÂËÌËÚ ‚ Ì‚ÓÌËÚ ̇ ÙÓÌÚÓÚÂÏÔÓ‡ÎÌËfl ÍÓÚÂÍÒ ÔË Í·Ò˘ÂÒ͇ڇ ÙÓχ. èË PROMM Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ì‡Û¯ÂÌËfl ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÛÏÂÌËfl ‚ ÔÓ-ÎÂ͇ ÒÚÂÔÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò ÚËÔ I. äãûóéÇà Ñìåà: ÏËÓÚÓÌ˘ÌË ‰ËÒÚÓÙËË, ÍÓ„ÌËÚË‚ÌË Ë Ôӂ‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl, Ú‡Û-ÔÓÚÂËÌË, ÙÓÌÚÓÚÂÏÔÓ‡ÎÂÌ ÍÓÚÂÍÒ. åËÓÚÓÌ˘ÌËÚ ‰ËÒÚÓÙËË ÚËÔ I (·ÓÎÂÒÚ Ì‡ Steinert) Ë ÚËÔ II (PROMM) Ò‡ „ÂÌÂÚ˘ÌË ÏÛÎÚËÒËÒÚÂÏÌË Á‡·ÓÎfl‚‡ÌËfl Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ Ë ÌÂÔ˙Î̇ ÔÂÌÂÚ‡ÌÚÌÓÒÚ, ÍÓËÚÓ Ò ı‡‡ÍÚÂËÁË‡Ú ÍÎË-

Ì˘ÌÓ Ò ÏËÓÚÓÌ˘ÌË ÙÂÌÓÏÂÌË (Á‡ÚÛ‰ÌÂ̇ ·ÍÒ‡ˆËfl ÒΉ ÒËÎÌÓ ÏÛÒÍÛÎÌÓ Ò˙Í‡˘ÂÌËÂ), ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl, ͇ڇ‡ÍÚ‡, ͇‰ËÓÏËÓÔ‡ÚËfl, „Ó̇‰Ì‡ ‡ÚÓÙËfl Ë ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl. èË ·ÓÎÂÒÚÚ‡ ̇ Steinert (åÑ 1) ÔÓ-ÚËÔ˘̇  Ò··ÓÒÚÚ‡ ̇ ‰ËÒÚ‡ÎÌËÚ ÏÛÒÍÛÎË, ‰Ó͇ÚÓ ÔË PROMM (åÑ 2) ÍÎËÌ˘̇ڇ ͇ÚË̇  ÔÓ-ÎÂ͇ Ë Ò Á‡Òfl„‡Ú ÔÂӷ·‰‡‚‡˘Ó ÔÓÍÒËχÎÌËÚ ÏÛÒÍÛÎË. èÓ ÓÚÌÓ¯ÂÌË ̇ ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ Ë ÚÂÊÂÒÚÚ‡ ̇ ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË Ò ̇·Î˛‰‡‚‡Ú ËÁ‡ÁÂÌË ËÌÚÂ- Ë ËÌÚ‡Ù‡ÏËÎÌË ‚‡ˇˆËË. àëíéêàü åËÓÚÓÌ˘̇ڇ ‰ËÒÚÓÙËfl ÚËÔ I  ÓÔË҇̇ Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Steinert ÔÂÁ 1909 (64) ͇ÚÓ Á‡·ÓÎfl‚‡ÌÂ Ò Ì‡˜‡ÎÓ ÔÂÁ ‚ÚÓÓÚÓ ËÎË ÚÂÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ. Vanier Ë Ò˙‡‚Ú. ÔÂÁ 1960 „. ÓÔËÒ‚‡Ú ÍÓÌ„ÂÌËÚ‡Î̇ ÙÓχ, ÍÓflÚÓ Ò Ô‰‡‚‡ ÓÚ ·ÓÎÌË Ï‡ÈÍË Ò ÚÓ‚‡ Á‡·ÓÎfl‚‡Ì (72). èÂÁ 1992 Ò ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â Á‡·ÓÎfl‚‡ÌÂÚÓ Â Ó·ÛÒÎÓ‚ÂÌÓ ÓÚ ÚËÌÛÍÎÂÓÚˉ̇ ÂÍÒÔ‡ÌÁËfl ‚ ÌÂÚ‡ÌÒÎË‡Ì „ËÓÌ Ì‡ DMPK-„Â̇ (40). äÎËÌ˘ÌËÚ ı‡‡ÍÚÂËÒÚËÍË Ì‡ PROMM Ò‡ ÓÔËÒ‡ÌË ÔÂÁ 1994 „, ‡ ÚӘ̇ڇ ÏÛÚ‡ˆËfl, Á‡Òfl„‡˘a ZNF9-„Â̇,  ÓÚÍËÚ‡ ÔÂÁ 2001 „ (39). ÖèàÑÖåàéãéÉàü ÅÓÎÂÒÚÚ‡ ̇ Steinert  ̇È-˜ÂÒÚÓ Ò¢‡Ì‡Ú‡ ÙÓχ ̇ ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl ÔË ‚˙Á‡ÒÚÌË. óÂÒÚÓÚ‡Ú‡ È ‚ Ó·˘‡Ú‡ ÔÓÔÛ·ˆËfl ‚‡Ë‡ ÓÚ 1/100 000 ‚ ÌflÍÓË Ó·Î‡ÒÚË Ì‡ üÔÓÌËfl ‰Ó 1/10 000 ‚ à·̉Ëfl, ‡ ÔÓ‡‰Ë ÂÙÂÍÚ‡ ̇ Ó‰Ó̇˜‡ÎÌË͇ ‚ ä‡Ì‡‰‡ (ä‚·ÂÍ) ˜ÂÒÚÓÚ‡Ú‡ È ‰ÓÒÚË„‡ ‰Ó 1/530. ë‰̇ڇ ˜ÂÒÚÓÚ‡  1/20 000. åÑ 2 Ò Ò¢‡ ÔÂӷ·‰‡‚‡˘Ó ‚ ÒÚ‡ÌËÚ ÓÚ Ò‚Â̇ Ö‚ÓÔ‡ Ë ÉÂχÌËfl, Í˙‰ÂÚÓ ·ÓÎÂÒÚÌÓÒÚÚ‡  ·ÎËÁ͇ ‰Ó Ú‡ÁË ÔË åÑ 1 (40). äãÄëàîàäÄñàü ë˙˘ÂÒÚ‚Û‚‡Ú ˜ÂÚËË ÙÓÏË Ì‡ ·ÓÎÂÒÚÚ‡ ̇ Steinert, ‡Á„‡Ì˘ÂÌË ÒÔÓ‰ ̇˜‡ÎÓÚÓ Ë ÚÂÊÂÒÚÚ‡ ̇ ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ ( 28): ● äÓÌ„ÂÌËÚ‡Î̇- ÓÍÓÎÓ 14% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò åÑ ÚËÔ 1 (29) ● ÑÂÚÒ͇ ● ä·Ò˘ÂÒ͇ ● ãÂ͇ éÒÌӂ̇ ÓÒÓ·ÂÌÓÒÚ, ÓÚ΢‡‚‡˘‡ PROMM ÓÚ åÑ ÚËÔ 1  ÎËÔÒ‡Ú‡ ̇ ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ. ÖíàéãéÉàü åÑ 1 Ò ӷÛÒ·‚fl ÓÚ ‰Ë̇Ï˘ÌË ÏÛÚ‡ˆËË, Á‡Òfl„‡˘Ë DMPK-„Â̇, ÎÓ͇ÎËÁË‡Ì ‚˙ıÛ 19q13.3 ıÓÏÓÁÓχ. ìÒÚ‡ÌÓ‚fl‚‡ Ò ‡ÏÔÎËÙË͇ˆËfl ̇ ÚËÌÛÍÎÂÓÚˉÌË CTGÔÓ‚ÚÓÂÌËfl ‚ 3’ ÌÂÚ‡ÌÒÎËÛÂÏ „ËÓÌ Ì‡ DMPK-„Â̇ (12). çÓχÎÌËflÚ ·ÓÈ Ì‡ ÚÂÁË ÔÓ‚ÚÓË ‚‡Ë‡ ÏÂÊ‰Û 5 Ë 37 (57). äÎËÌ˘̇ ËÁfl‚‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡,


ÑÂÍÂÏ‚Ë 2009

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

ÍÓ„‡ÚÓ ·ÓflÚ Ì‡ ÔÓ‚ÚÓËÚ ̇‰ı‚˙ÎË 50. èË ËÌ‰Ë‚Ë‰Ë Ò CTG-ÔÓ‚ÚÓÂÌËfl ÏÂÊ‰Û 35 Ë 49 Ìflχ ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇, ÌÓ ÚÂıÌËÚ ‰Âˆ‡ Ò‡ Ò Ôӂ˯ÂÌ ËÒÍ ÓÚ Û̇ÒΉfl‚‡Ì ̇ ÔÓ-„ÓÎflÏ ·ÓÈ ÔÓ‚ÚÓË Ë Ò˙ÓÚ‚ÂÚÌÓ ËÁfl‚fl‚‡Ì ̇ ·ÓÎÂÒÚÚ‡ (33). 燘‡ÎÓÚÓ Ë ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ÍÓÂÎË‡Ú Ò ·Ófl ̇ ‡ÏÔÎËÙˈË‡ÌËÚ ÔÓ‚ÚÓË Ë ÔÓËÁıÓ‰‡ ̇ ÏÛÚ‡ÌÚÌËfl „ÂÌ- χȘËÌ ËÎË ·‡˘ËÌ. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÍÓÎÍÓÚÓ Â ÔÓ-„ÓÎflÏ Â ÚÓÁË ·ÓÈ, ÚÓÎÍÓ‚‡ ÔÓ-‡ÌÓ Á‡ÔÓ˜‚‡ Ë ÔÓÚÂÊÍÓ ÔÓÚ˘‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. í˙È Í‡ÚÓ ÚËÌÛÍÎÂÓÚˉÌËÚ CTG ÔÓ‚ÚÓË ‚ DMPK „Â̇ Ò‡ ÌÂÒÚ‡·ËÎÌË ÔÓ ‚ÂÏ ̇ ÏËÚÓÁ‡Ú‡, ˜ÂÒÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÒÓχÚ˘ÂÌ ÏÓÁ‡ËˆËÁ˙Ï ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‡ÁÏÂ‡ ̇ CTG ÂÍÒÔ‡ÌÁËflÚ‡ (28). ᇠÚÓ‚‡ Ì ‚Ë̇„Ë Â ‚˙ÁÏÓÊ̇ ÍÓ·ˆËfl ÏÂÊ‰Û ‡ÁÏÂ‡ ̇ ÔÓ‚ÚÓËÚÂ, ̇·Î˛‰‡‚‡ÌË ‚ ‰̇ Ú˙͇Ì, Ë ÚÂÊÂÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. åÌÓ„Ó ÚËÔ˘ÌÓ fl‚ÎÂÌË  ‡ÌÚˈËÔ‡ˆËflÚ‡- ÔÓ-‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÔÓ-ÚÂÊ͇ ÍÎËÌ˘̇ ËÁfl‚‡ ‚˙‚ ‚ÒflÍÓ ÒΉ‚‡˘Ó ÔÓÍÓÎÂÌËÂ. ífl Ò ‰˙ÎÊË Ì‡ Û‚Â΢‡‚‡ÌÂÚÓ Ì‡ CTG-ÔÓ‚ÚÓËÚ ‚ ÔÓÍÓÎÂÌËflÚ‡ (28; 48). ìÒÚ‡ÌÓ‚fl‚‡ Ò ÔÓÎÓ‚Ó-‰ËÙÂÂ̈Ë‡Ì‡ ÂÍÒÔÂÒËfl ̇ „Â̇ Ò ÔÓÚÂÊ͇ ÍÎËÌ˘̇ ËÁfl‚‡ ‡ÍÓ Ò Û̇ÒÎÂ‰Ë ÓÚ Ï‡È͇ڇ. åÑ 2 Ò ӷÛÒ·‚fl ÓÚ Ú‡ÌÒÍË·Ë‡Ì‡, ÌÓ ÌÂÚ‡ÌÒÎË‡Ì‡ ÂÍÒÔ‡ÌÁËfl ̇ CCTG- ÌÛÍÎÂÓÚˉÌË ÔÓ‚ÚÓË ‚ ËÌÚÓÌ 1 ̇ ZNF9 (Zinc Finger protein 9) „Â̇ (39; 48). Ç˙ÔÂÍË ˜Â ÍÎËÌ˘̇ڇ ͇ÚË̇ ÔË PROMM  ÔÓ-ÎÂ͇, ·ÓflÚ Ì‡ ‡ÏÔÎËÙˈË‡ÌËÚ CCTG- ÔÓ‚ÚÓË ÏÓÊ ‰‡ ‰ÓÒÚË„Ì ÓÚ 75 ‰Ó 11 000 (56). èÄíéÉÖçÖáÄ ÉÂÌ˙Ú, ‚ ÍÓÈÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‰Ë̇Ï˘̇ ÏÛÚ‡ˆËfl ÔË åÑ 1, ÍÓ‰Ë‡ ÒÂËÌ-ÚÂÓÌËÌ ÔÓÚÂËÌ ÍË̇Á‡ (DMPK –dystrophy myotonic protein kinase), ÎÓ͇ÎËÁË‡Ì‡ ‚ ÒÔˆËÙ˘ÌË ÍÎÂÚ˙˜ÌË ÒÚÛÍÚÛË Ì‡ ÒÍÂÎÂÚ̇ڇ, Ò˙‰Â˜Ì‡Ú‡ ÏÛÒÍÛ·ÚÛ‡ Ë Ì‚ÓÌËÚÂ, ‡ÒÓˆËË‡ÌË Ò ÏÂʉÛÍÎÂÚ˙˜Ì‡Ú‡ ÔÓ‚Ó‰ËÏÓÒÚ Ë Ô‰‡‚‡ÌÂÚÓ Ì‡ ËÏÔÛÎÒËÚ (6; 12;). ÉÂÌ˙Ú, ‚ ÍÓÈÚÓ Ò ÓÚÍË‚‡ ‰Ë̇Ï˘̇ ÏÛÚ‡ˆËfl ÔË åÑ 2  ZNF9. íÓÈ ÍÓ‰Ë‡ ÔÓÚÂËÌ, Ò˙ÒÚ‡‚ÂÌ ÓÚ Ò‰ÂÏ ÂÎÂÏÂÌÚ‡, ËÁ‚ÂÒÚÌË Í‡ÚÓ zinc finger ‰ÓÏÂÌË, ˜ËflÚÓ ÙÛÌ͈Ëfl  ‰‡ Ò ҂˙Á‚‡Ú Ò ÚÓ˜ÌÓ ÓÔ‰ÂÎÂÌË Ó·Î‡ÒÚË Ì‡ Ëêçä. Zinc finger ÔÓÚÂËÌ 9 Û˜‡ÒÚ‚‡ ‚ „Û·ˆËflÚ‡ ̇ „ÂÌËÚ ÓÚ„Ó‚ÓÌË Á‡ ÒËÌÚÂÁ‡Ú‡ Ë ÏÂÚ‡·ÓÎËÁχ ̇ ıÓÎÂÒÚÂÓ·. íÓÁË ÔÓÚÂËÌ Ò ÓÚÍË‚‡ ‚ ÏÌÓ„Ó Ú˙͇ÌË, ÌÓ ÓÒÌÓ‚ÌÓ ‚ Ò˙‰Â˜Ì‡Ú‡ Ë ÒÍÂÎÂÚ̇ڇ ÏÛÒÍÛ·ÚÛ‡. ÖÙÂÍÚ˙Ú Ì‡ CTG, ÂÒÔ. ̇ CëTG - ÔÓ‚ÚÓËÚ  ÍÓÏÔÎÂÍÒÂÌ Ë ÌÂ̇Ô˙ÎÌÓ ËÁflÒÌÂÌ. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ‡ÏÔÎËÙË͇ˆËflÚ‡ Ì Á‡Òfl„‡ ÍÓ‰Ë‡˘Ëfl Ò„ÏÂÌÚ Ì‡ Ò˙ÓÚ‚ÂÚÌËfl „ÂÌ, Ú˙È Í‡ÚÓ Úfl Ò Ú‡ÌÒÍË·Ë‡ ‚ êçä, ÌÓ Ì Ò Ú‡ÌÒÎË‡. åËÓÚÓÌ˘ÌËÚ ‰ËÒÚÓÙËË Ò‡ Ô˙‚ËflÚ ÔËÏÂ Á‡ êçä ωËË‡ÌÓ Á‡·ÓÎfl‚‡ÌÂ. ÖÍÒÔÂÒËflÚ‡ ̇ ÏÛÚ‡ÌÚÌËfl „ÂÌ Ó·ÛÒ·‚fl ÔÓfl‚‡Ú‡ ̇ ÏÛÚ‡ÌÚ̇ Ëêçä Ò ‰ËÂÍÚÂÌ ÚÓÍÒ˘ÂÌ ÂÙÂÍÚ ‚˙ıÛ ÍÎÂÚÍËÚ ( 51). ífl Ò ÓÚ·„‡ ‚ ÍÎÂÚ˙˜ÌËÚ fl‰‡ Ë ÙÓÏË‡ Ë·ÓÌÛÍ·ÌË ‚Íβ˜‚‡ÌËfl ‚ Á‡Ò„̇ÚËÚ Ú˙͇ÌË- ÓÒÌÓ‚ÌÓ ‚ ÏÛÒÍÛÎËÚÂ Ë ÏÓÁ˙͇, ÛÒÚ‡ÌÓ‚ÂÌË ˜ÂÁ FISH (Fluorescence in situ hybridization). ä‡ÚÓ Ô˙‚‡ ÔÓÒΉˈ‡ ̇ êçä ÚÓÍÒ˘ÌÓÒÚÚ‡ ÔË ·ÓÎÂÒÚÚ‡ ̇ Steinert  ‰ËÒÙÛÌ͈Ëfl ̇ ‰‚‡ Í·҇ êçä Ò‚˙Á‚‡˘Ë ÔÓÚÂËÌË, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ‡·ÌÓÏ̇ „Û·ˆËfl ̇ ‡ÎÚÂ̇ÚË‚ÌËfl ÒÔ·ÈÒËÌ„ ( ÔË Ú‡ÌÒÍË·ˆËflÚ‡ Ë ÒËÌÚÂÁ‡Ú‡ ̇ Ò˙‰Â˜ÌËfl ÚÓÔÓÌËÌ í, ËÌÒÛÎËÌÓ‚Ëfl ˆÂÔÚÓ, ıÎÓˉÌËfl ͇̇Π1- ÓÒÌÓ‚ÂÌ ‚ ÏÛÒÍÛÎËÚÂ) Ë ÒÔ·ÈÒÂÓÔ‡ÚËfl, ÂÁÛÎÚ‡Ú, ÓÚ ÍÓÂÚÓ Ò‡ ÏËÓÚÓÌËflÚ‡ Ë ËÌÒÛÎËÌÓ‚‡Ú‡ ÂÁËÒÚÂÌÚÌÓÒÚ. è‰ÔÓ·„‡ ÒÂ, ˜Â ÚÂÁË Ë·ÓÌÛÍ·ÌË ‚Íβ˜‚‡ÌËfl ÏÓ„‡Ú ‰‡ ̇Û¯‡Ú Ë ‰Û„Ë ÍÎÂÚ˙˜ÌË ÙÛÌ͈ËË. DMPK Ò ÔÓ‰ÛˆË‡ ‚ „ÓÎÂÏË ÍÓ΢ÂÒÚ‚‡ ‚ ÒÍÂÎÂÚÌËÚ ÏÛÒÍÛÎÌË ÍÎÂÚÍË Ë Í‡‰ËÓÏËÓˆËÚËÚÂ, ‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ ÍÓÂÚÓ Â ÏÛÒÍÛÎÌÓÚÓ Ë Ò˙‰Â˜ÌÓÚÓ Á‡Òfl„‡ÌÂ

188

ÔË Ô‡ˆËÂÌÚËÚÂ Ò ·ÓÎÂÒÚÚ‡ ̇ Steinert. Ç ÏÛÒÍÛÎËÚ ÚÓÁË ÔÓÚÂËÌ Ò ÓÚÍË‚‡ ÔÓÒÚÒË̇ÔÚ˘ÌÓ ‚ Ì‚ÓÏÛÒÍÛÎÌËÚ ÒË̇ÔÒË, ‡ ‚ ͇‰ËÓÏËÓˆËÚËÚ ‚ ӷ·ÒÚÚ‡ ̇ ËÌÚÂ͇·ÚÌËÚ ‰ËÒÍÓ‚Â (18). Ç ñçë ÏËÓÚÓÌËÌ ÔÓÚÂËÌ ÍË̇Á‡Ú‡ Ò ÎÓ͇ÎËÁË‡ ‚ ӷ·ÒÚÚ‡ ̇ ÒË̇ÔÒËÚ ‚ χÎÍËfl ÏÓÁ˙Í, Ò‰ÌËfl ÏÓÁ˙Í, ıËÔÓÍ���ÏÔ‡, ÔÓ‰˙΄ӂ‡ÚËfl ÏÓÁ˙Í Ë Ó·Î‡ÒÚÚ‡ ̇ ‡ÔË͇Î̇ڇ ÏÂÏ·‡Ì‡ ̇ ÂÔẨËÏÌËÚ ÍÎÂÚÍË Ë ÍÎÂÚÍËÚ ̇ ıÓËÓˉÌËfl ÔÎÂÍÒÛÒ. ÇÂÓflÚÌÓ Ò˙˘ÂÒÚ‚Û‚‡Ú ‰‚‡ ÓÒÌÓ‚ÌË Ô‡ÚÓ„ÂÌÂÚ˘ÌË ÏÂı‡ÌËÁ˙χ, ÍÓËÚÓ Ó·ÛÒ·‚flÚ ÔÓfl‚ËÚ ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÔË Ô‡ˆËÂÌÚË Ò åÑ ÚËÔ I. ● ç‡Î˘ËÂÚÓ Ì‡ ̇ ‡ÎÂÎË Ò „ÓÎflÏ ·ÓÈ CTG-ÔÓ‚ÚÓË Ë Ò˙ÓÚ‚ÂÚÒ‚‡˘ËÚ ËÏ CUG Ëêçä ÏÓÊ ‰‡ ̇Û¯Ë ÂÍÒÔÂÒËflÚ‡ ̇ „ÂÌË, „ÛÎË‡˘Ë ‡ÌÌËÚ ÂÚ‡ÔË ÓÚ ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙͇, ÍÓÂÚÓ Ò ÓÚÍË‚‡ ÔË Ï˯ÍË-ÏÓ‰ÂÎË (18; 48; 74) . íÓ‚‡ ÏÓÊ ‰‡ ·˙‰Â Ô˘Ë̇ڇ ÔË Ô‡ˆËÂÌÚË Ò „ÓÎflχ ÚËÔÎÂÚ̇ ÂÍÒÔ‡ÌÁËfl (>1000), ÍÓÌ„ÂÌËÚ‡ÎÌË ÙÓÏË Ë ÒÎÛ˜‡Ë Ò ÚÂÊ˙Í ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ, Á‡Òfl„‡˘ ‚Ò˘ÍË ÂÎÂÏÂÌÚË Ì‡ Ó·˘ÓÚÓ ÍÓ„ÌËÚË‚ÌÓ ÙÛÌ͈ËÓÌË‡ÌÂ, ‚Â·‡ÎÌËÚÂ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË (51). ● ÇËÒÓÍËÚ ÍÓ΢ÂÒÚ‚‡ ̇ DMPK êçä Ò CUG-ÂÍÒÔ‡ÌÁËfl, ÂÍÒÔÂÒË‡Ì‡ ‚ ÍÓÚË͇ÎÌËÚÂ Ë ÔÓ‰ÍÓÓ‚ËÚ Ì‚ÓÌË, ÏÓÊ ‰‡ ̇Û¯Ë Ô‡‚ËÎÌËfl ÒÔ·ÈÒËÌ„ ̇ Ú‡Û ÔÓÚÂËÌËÚ ‚˙‚ ÙÓÌÚÓÚÂÏÔÓ‡ÎÌËfl ÍÓÚÂÍÒ, ̇ N-ÏÂÚËÎ-D-‡ÒÔ‡Ú‡Ú ˆÂÔÚÓ 1 (NMDAR 1) Ë ‡ÏËÎÓˉ ÔÂÍÛÒÓÌËfl ÔÓÚÂËÌ (APP). íÓ‚‡ ÏÓÊ ‰‡ ‰Ó‚‰ ‰Ó ÔÓ„ÂÒË‡˘ Ò ‚˙Á‡ÒÚÚ‡ ÛÔ‡‰˙Í Ì‡ ÙÓÌÚ‡ÎÌËÚÂ Ë ÚÂÏÔÓ‡ÎÌËÚ ÍÓ„ÌËÚË‚ÌË ÙÛÌ͈ËË (‰˙΄ÓÒӘ̇ Ë ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚Â̇ Ô‡ÏÂÚ) ÔË Ô‡ˆËÂÌÚË Ò Ï‡Î͇ CTG-ÂÍÒÔ‡ÌÁËfl. íÂÁË ‰‡ÌÌË Ó·ÛÒ·‚flÚ ‚Íβ˜‚‡ÌÂÚÓ Ì‡ åÑ ÚËÔ 1 Í˙Ï „ÂÌÂÚ˘ÌÓ ‰ÂÚÂÏËÌË‡ÌËÚ ڇÛÔ‡ÚËË (13; 38; 61). ● èË ÏË¯Ë ÏÓ‰ÂÎË DMK Ò ÎÓ͇ÎËÁË‡ ‚ ÒË̇ÔÒËÚ ̇ χÎÍËfl ÏÓÁ˙Í, ıËÔÓ͇ÏÔ‡ Ë ÏÓÁ˙˜ÌËfl ÒÚ‚ÓÎ, ÍÓÂÚÓ ‚ÂÓflÚÌÓ Ò˙˘Ó Ëχ Á̇˜ÂÌË Á‡ ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl (19; 62). Ç˙ÔÂÍË ˜Â ‡Á΢ÌË „ÂÌÌË ÎÓÍÛÒË Ò‡ ÓÚ„Ó‚ÓÌË Á‡ ‰‚ÂÚ ÙÓÏË Ì‡ ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl, Ú Ò ӷÛÒ·‚flÚ ÓÚ ÒıÓ‰ÌË Ô‡ÚÓÙËÁËÓÎӄ˘ÌË ÏÂı‡ÌËÁÏË Ë ËÏ‡Ú ÒıÓ‰ÂÌ ÙÂÌÓÚËÔ, Ú.Â. ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ PROMM Ò Ô‰ÔÓ·„‡Ú ‡Ì‡Îӄ˘ÌË Ì‡ åÑ 1 ÏÂı‡ÌËÁÏË. èÄíéãéÉéÄçÄíéåàü ë‚ÂÚÎËÌÌÓÏËÍÓÒÍÓÔÒÍÓÚÓ ËÁÒΉ‚‡Ì ̇ ÏÛÒÍÛÎË ÔË ÚÂÁË Ô‡ˆËÂÌÚË ÓÚÍË‚‡ ÌÂÒÔˆËÙ˘ÌË ÔÓÏÂÌË, ÒıÓ‰ÌË Ò ÚÂÁË ÔË ‰Û„Ë ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË Í‡ÚÓ ÌÂÍÓÁ‡ ̇ ÏÛÒÍÛÎÌË ‚·Í̇. èË ÚÂÁË Ô‡ˆËÂÌÚË Ì  ÚËÔ˘̇ ËÎË Â Ò··Ó ËÁ‡ÁÂÌÓ ‡Á‡ÒÚ‚‡ÌÂÚÓ Ì‡ Ò˙‰ËÌËÚÂÎ̇ Ú˙͇Ì. ᇠåÑ 1  ı‡‡ÍÚÂ̇ ‡ÚÓÙËflÚ‡ ̇ ̇ ÚËÔ 1 ÏÛÒÍÛÎÌË ‚·Í̇, ÍÓÂÚÓ Ò Ò¢‡ Ë ÔË Emery-Dreifuss ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl. èË ÌÂÓ̇ڇÎ̇ڇ ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl Ò‡ ‚ Ò˙ÒÚÓflÌË ̇ ◊χÚÛ‡ˆËÓÌÂÌ ‡ÂÒÚ” ̇ ‡Á΢ÌË ÂÚ‡ÔË ÓÚ ‡Á‚ËÚËÂÚÓ: ÏËӷ·ÒÚË, ÏËÓÚÛ·ÛÎË, ÌÂÔ˙Î̇ ıËÒÚÓıËÏ˘̇ ‰ËÙÂÂ̈ˇˆËfl ̇ ÚËÔ 1 Ë ÚËÔ 2 ‚·Í̇, ÂÍÒÔÂÒËfl ̇ ËÌÚÂωËÂÌË ÙÂÚ‡ÎÌË ÙË·ÏÂÌÚË Í‡ÚÓ ‰ÂÁÏËÌ Ë ‚ËÏÂÌÚËÌ (59). èË ÌflÍÓË ÒÎÛ˜‡Ë ̇ ÍÓÌ„ÂÌËÚ‡Î̇ ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl Ò ÓÚÍË‚‡ ‰ËÒÔÓÔÓˆËfl ̇ ÏËÓÙË·ËÎËÚÂ, ÚËÔ˘̇ Á‡ ÌflÍÓË ÍÓÌ„ÂÌËÚ‡ÎÌË ÏËÓÔ‡ÚËË.èË åÑ 2 ‡ÚÓÙ˘ÌËÚ ËÁÏÂÌÂÌËfl Á‡Òfl„‡Ú Ô‰ËÏÌÓ ÏÛÒÍÛÎÌË ‚·Í̇ ÚËÔ 2. ëÔˆËÙ˘ÌËÚ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl Ò‡ Ò‚˙Á‡ÌË Ò ‡ÁÌÓÓ·‡ÁÌË ÏÓÙÓÎӄ˘ÌË ‡·ÌÓÏÌÓÒÚË. ç‡È-ÚËÔ˘ÌË Ò‡ Ì‚ÓÙË·ËÎÂÌËÚ ‰Â„ÂÌÂ‡ˆËË, ‚˙ÚÂÍÎÂÚ˙˜ÌËÚ ˆËÚÓÔ·ÁÏÂÌË ‚Íβ˜‚‡ÌËfl, χÒÚÌËÚ „‡ÌÛÎË‡ÌË ÍÎÂÚÍË, ÎÂÁËËÚ ̇ ÔÓ‰ÍÓÓ‚ÓÚÓ ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó, Ò Û‚Âʉ‡Ì ̇ ÏËÂÎËÌÓ‚ËÚ ӷ‚Ë‚ÍË ÔË ÓÚÌÓÒËÚÂÎÌÓ Á‡Ô‡ÁÂÌË ‡ÍÒÓÌË, ͇ÍÚÓ Ë ÍÓÓ‚‡Ú‡ ‡ÚÓÙËfl (16; 17; 23; 52; 76 ). ᇠԇˆËÂÌÚË Ò ÍÓÌ„ÂÌËÚ‡Î̇ ÙÓχ ̇ åÑ 1


ÑÂÍÂÏ‚Ë 2009

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

ÔÓ-ÚËÔ˘ÌË Ò‡ ÍÓÓ‚‡Ú‡ ‡ÚÓÙËfl, ıËÔÓÔ·ÁËfl ̇ corpus callosum Ë ‚ÂÌÚËÍÛÎÓÏ„‡ÎËflÚ‡ (16).íẨÂ̈ËflÚ‡ Á‡ ÒËÏÂÚ˘ÌÓ ÒÎË‚‡Ì ̇ ÎÂÁËËÚ ̇ ·flÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó Ë ÔÓ„ÂÒË‡˘‡ ‚ÂÌÚËÍÛÎÓÏ„‡ÎËfl, ÍÓflÚÓ ÔÓ Ò‚ÓflÚ‡ ÚÂÊÂÒÚ ÍÓÂÎË‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, Ô‰ÔÓ·„‡Ú ·‡‚ÌÓ ÔÓ„ÂÒË‡˘ ÔÓˆÂÒ Ì‡ ‰ÂÏËÂÎËÌËÁ‡ˆËfl (16; 18; 35). íÓÈ Â ‚˙ÁÏÓÊÌÓ ‰‡  ‚ÒΉÒÚ‚Ë ̇ ̇Û¯ÂÌ ÔÓˆÂÒ Ì‡ Ó·‡ÁÛ‚‡Ì ̇ ÏËÂÎËÌÓ‚ËÚ ӷ‚Ë‚ÍË ÔÓ ‚ÂÏ ̇ ÙÂÚ‡ÎÌÓÚÓ ‡Á‚ËÚËÂ. Ç˙ÚÂÌ‚ÓÌÌËÚ ‡„„‡ÚË Ì‡ ıËÔÂÙÓÒÙÓËÎË‡Ì Ô‡ÚÓÎӄ˘ÂÌ Ú‡Û-ÔÓÚÂËÌ Ò ÓÚÍË‚‡Ú ‚ ÏÓÁ˙ˆËÚ ̇ Ô‡ˆËÂÌÚË Ò ‡Á΢ÌË Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌË Á‡·ÓÎfl‚‡ÌËfl, ‚ÍÎ. Ë åËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl I ÚËÔ (61; 62). ç‚ÓÙË·ËÎÂÌËÚ ‰Â„ÂÌÂ‡ˆËË Ò ÓÚÍË‚‡Ú ‚ ÎËϷ˘̇ڇ ÒËÒÚÂχ, ÏÓÁ˙˜ÌËfl ÒÚ‚ÓÎ, ıËÔÓ͇ÏÔ‡, ÂÌÚÓË̇ÎÌËfl ÍÓÚÂÍÒ Ë Ë ÚÂÏÔÓ‡ÎÌËÚ ÍÓÓ‚Ë ÁÓÌË (49; 73). ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÒÂÌËÎÌË Ô·ÍË Ë ‡ÏËÎÓˉÌË ‰ÂÔÓÁËÚË. ëÔˆËÙ˘ÌËÚ Ì‚ÓÔ‡ÚÓÎӄ˘ÌË ·ÂÎÂÁË ÔË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ ̇ Steinert Ò‡ Ò‚˙Á‡ÌË Ò ÛÔ‡‰˙Í Ì‡ ÂÁËÍÓ‚ËÚÂ Ë ÂÍÁÂÍÛÚË‚ÌËÚ ÙÛÌ͈ËË, ÒıÓ‰ÂÌ Ò ÚÓÁË ÔË ÙÓÌÚÓ-ÚÂÏÔÓ‡Î̇ ‰ÂÏÂ̈Ëfl. äãàçàóçÄ äÄêíàçÄ èêà êÄáãàóçàíÖ îéêåà çÄ ÅéãÖëííÄ çÄ STEINERT ãÂ͇ڇ ÙÓχ Ò ı‡‡ÍÚÂËÁË‡ ÍÎËÌ˘ÂÒÍË Ò Í‡Ú‡‡ÍÚ‡, ÓÔί˂fl‚‡ÌÂ, ÏËÌËχÎÂÌ ‰Ó ÎËÔÒ‚‡˘ ÏÛÒÍÛÎÂÌ ‰ÂÙˈËÚ ËÎË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. ÅÓflÚ Ì‡ CTG ÔÓ‚ÚÓËÚ  ÏÂÊ‰Û 50 Ë 150 (40) . èË Í·Ò˘ÂÒ͇ڇ ÙÓχ ̇˜‡ÎÓÚÓ Â Í˙Ï 20-30, ÔÓfl‰ÍÓ ÒΉ 40 „Ӊ˯̇ ‚˙Á‡ÒÚ. ÅÓflÚ Ì‡ ÚËÌÛÍÎÂÓÚˉÌËÚ ÔÓ‚ÚÓË Â 300-1000. èÓ„ÂÒËflÚ‡ È Â Ò‡‚ÌËÚÂÎÌÓ ·‡‚̇. ï‡‡ÍÚÂËÁË‡ ÒÂ Ò ÏËÓÚÓÌ˘ÌË ÔÓfl‚Ë, Ô‰¯ÂÒÚ‚‡˘Ë ËÎË ‡Á‚Ë‚‡˘Ë Ò ‰ÌÓ‚ÂÏÂÌÌÓ Ò ÏÛÒÍÛÎ̇ڇ Ò··ÓÒÚ Ë ‡ÚÓÙËfl ‚ ‰ËÒÚ‡ÎÌËÚ ÏÛÒÍÛÎË Ì‡ Í‡ÈÌˈËÚ (1). á‡Òfl„‡Ú ÒÂ Ë m. sternocleidomastoideus, ÏÛÒÍÛÎËÚ ̇ ¯ËflÚ‡ ËÁÚ˙Ìfl‚‡Ú, ¯ËÈ̇ڇ ÎÓ‰ÓÁ‡ Ò ۂÂ΢‡‚‡, ÔÓfl‚fl‚‡ Ò Ú. ̇. ◊η‰ӂ‡ ¯Ëfl”. ìÒÚ‡ÌÓ‚fl‚‡Ú Ò ıËÔÓÚÓÙËË Ì‡ ‰˙‚͇ÚÂÎÌËÚ ÏÛÒÍÛÎË (ıÎ˙Ú‚‡Ì ̇ ·ÛÁËÚÂ Ë ÒÎÂÔÓÓ˜ËflÚ‡), ΂‡ÚÓËÚ ̇ ÍÎÂÔ‡˜ËÚ (ÔÚÓÁ‡) Ë Îˈ‚ËÚ ÏÛÒÍÛÎË. íÂÁË ·ÂÎÂÁË, Á‡Â‰ÌÓ Ò ÙÓÌÚ‡ÎÌÓÚÓ ÓÔί˂fl‚‡ÌÂ Ë „ÓÎÂÏËÚ ÓÚÎÂÔÂÌË Û¯Ë Ôˉ‡‚‡Ú ı‡‡ÍÚÂÂÌ ‚ˉ ̇ ·ÓÎÌËÚÂ. ê‡Á‚Ë‚‡ Ò ‡ÚÓÙËfl ̇ ÔÓ‰·Â‰ˈËÚ Ò˙Ò ÒÚÂÔ‡Ê̇ ÔÓıӉ͇, ‡ÚÓÙËfl ̇ Ô‰Ï˯ÌˈËÚÂ Ë ÍËÚ͇ڇ. ÄÚÓÙËËÚ ҇ ‰‚ÛÒÚ‡ÌÌË, ÒËÏÂÚ˘ÌË (1). èÓÍÒËχÎÌËÚ ÏÛÒÍÛÎË Ò‡ Ò‡‚ÌËÚÂÎÌÓ Ò··Ó Á‡Ò„̇ÚË. ë ̇Ô‰‚‡Ì ̇ ·ÓÎÂÒÚÚ‡ ÏËÓÚÓÌ˘ÌËÚ ÙÂÌÓÏÂÌË ËÁ˜ÂÁ‚‡Ú Ë ÔÂӷ·‰‡‚‡˘Ë Ò‡ ÏÛÒÍÛÎ̇ڇ Ò··ÓÒÚ Ë ‡ÚÓÙËflÚ‡. èË ÌflÍÓË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔÓÎËÌ‚ÓÔ‡ÚËfl ÓÚ ‰ÂÏËÂÎËÌËÁË‡˘ ÚËÔ Ò ÔÂӷ·‰‡‚‡˘‡ Á‡„Û·‡ ̇ ‰Â·ÂÎÓÏËÂÎËÌËÁË‡ÌË ‚·Í̇. èÓfl‚Ë ÓÚ ÒÚ‡Ì‡ ̇ Ò˙‰Â˜ÌÓÒ˙‰Ó‚‡Ú‡ ÒËÒÚÂχ Ò‡ ÏÌÓ„Ó ÚËÔ˘ÌË Ë ‚Íβ˜‚‡Ú ‡ÒËÏÔÚÓÏÌË ÖäÉ ÔÓÏÂÌË ÔË 90% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÔÓ‚Ó‰ÌËÚ (Û‰˙Îʇ‚‡Ì ̇ PR- ËÌÚÂ‚‡Î‡, AV·ÎÓÍ) Ë ËÚ˙ÏÌËÚ (͇ÏÂÌË Ë Ì‡‰Í‡ÏÂÌË ‡ËÚÏËË) ̇Û¯ÂÌËfl, ͇‰ËÓÏËÓÔ‡ÚËfl (ÔÓ-fl‰ÍÓ ‚ Ò‡‚ÌÂÌËÂ Ò ‰Û„Ë ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË). íÂÁË Ô‡ˆËÂÌÚË Ò‡ Ò ‚ËÒÓÍ ËÒÍ ÓÚ ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. (29) á‡Òfl„‡ÌÂÚÓ Ì‡ ‰Ë‡Ù‡„χڇ Ë ‰Ëı‡ÚÂÎ̇ڇ ÏÛÒÍÛ·ÚÛ‡ ‚Ó‰Ë ‰Ó ıËÔÓ‚ÂÌÚË·ˆËfl, ÍÓÂÚÓ Ì‡Î‡„‡ ÔÂËӉ˘ÌÓ ÔÓ‚Âʉ‡Ì ̇ îàÑ Ë Í˙‚ÌÓ-„‡ÁÓ‚ ‡Ì‡ÎËÁ. èË ÓÍÓÎÓ 70% ÓÚ Ï˙ÊÂÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ıËÔÂ„Ó̇‰ÓÚÓÔÂÌ ıËÔÓ„Ó̇‰ËÁ˙Ï (‚ËÒÓÍË ÌË‚‡ ̇ ãï Ë îëï ÔË ÌÓχÎÂÌ ËÎË ÔÓÌËÊÂÌ ÚÂÒÚÓÒÚÂÓÌ) Ò ‡ÚÓÙËfl ̇ ÚÂÒÚËÒËÚÂ Ë ‡ÁÓÓÒÔÂÏËfl. èË 15-20% ÓÚ ÊÂÌËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‰ËÒÏÂÌÓÂfl Ë ‡Ì̇ ÏÂÌÓÔ‡ÛÁ‡. èӂ˯ÂÌ Â ËÒÍ˙Ú ÓÚ ÒÔÓÌÚ‡ÌÌË ‡·ÓÚË. óÂÒÚÓ Â Ì‡Îˈ ËÌÒÛÎËÌÓ‚‡ ÂÁËÒÚÂÌÚÌÓÒÚ, ͇ÍÚÓ Ë Ì‡Û¯ÂÌ ÎËÔˉÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï (29). åÌÓ„Ó ÚËÔ˘ÌË Ó˜ÌË ÔÓfl‚Ë Ò‡ ‰‚ÛÒÚ-

189

‡ÌÌËÚ Á‡‰ÌÓÍÓÚË͇ÎÌË Í‡Ú‡‡ÍÚË Ë ÚÂÁË ÚËÔ Christmas tree. èË„ÏÂÌÚ̇ڇ ‰Â„ÂÌÂ‡ˆËfl ̇ ÂÚË̇ڇ, ÛÒÚ‡ÌÓ‚Â̇ ˜ÂÁ ÂÎÂÍÚÓÂÚËÌÓ„‡ÙËfl, ÏÓÊ ‰‡ Ô‰¯ÂÒÚ‚‡ ÏÛÒÍÛÎÌËÚ ÔÓfl‚Ë. éÚ ÒÚ‡Ì‡ ̇ ı‡ÌÓÒÏË·ÚÂÎ̇ڇ ÒËÒÚÂχ Ò ÓÚÍË‚‡Ú ‰ËÒÙ‡„Ëfl ÔÓ‡‰Ë Ò··ÓÒÚ Ì‡ Ù‡Ë̄‡Î̇ڇ ÏÛÒÍÛ·ÚÛ‡, „‡ÒÚÓÂÁÓÙ‡„‡ÎÂÌ ÂÙÎÛÍÒ, ̇χÎÂÌ ˜‚ÂÌ ÏÓÚËÎËÚÂÚ, ÍÓÌÒÚËÔ‡ˆËfl, ıÓÎÂÎËÚˇÁ‡. èË ÓÍÓÎÓ 50% ÓÚ Ô‡ˆËÂÌÚËÚ ҇ Ôӂ˯ÂÌË Äî Ë ÉÉí ÔÓ ÌÂflÒÂÌ ÏÂı‡ÌËÁ˙Ï ÔË ÎËÔÒ‡ ̇ ˜ÂÌÓ‰Ó·ÌÓ Û‚Âʉ‡Ì (30). ÇÒΉÒÚ‚Ë ̇ ÛÒÍÓÂÌ IgG- ÏÂÚ‡·ÓÎËÁ˙Ï ÔË ÓÍÓÎÓ 1/3 ÓÚ Ô‡ˆËÂÌÚËÚ ÌË‚‡Ú‡ ÏÛ Ò‡ ÔÓÌËÊÂÌË. èË ÏËÓÚÓÌ˘̇̇ ‰ËÒÚÓÙËfl ̇ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó Ì‡˜‡ÎÓÚÓ Â ÏÂÊ‰Û 1 Ë 10 „Ó‰ËÌË. ç‡È- ˜ÂÒÚÓ ÚÓ‚‡ Ò‡ ‰Âˆ‡ Ò ÌÓχÎÌÓ ÔÂ- Ë ÔÓÒÚ̇ڇÎÌÓ ‡Á‚ËÚË ÔÂÁ Ô˙‚‡Ú‡ „Ó‰Ë̇ ÓÚ ÊË‚ÓÚ‡, ˜ËËÚÓ Ô‡ÚÓÎӄ˘ÌË ÓÚÍÎÓÌÂÌËfl („ÂÌÂ‡ÎËÁË‡Ì‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ, Á‡Òfl„‡˘‡ ÓÒÌÓ‚ÌÓ ÎˈÂÚÓ Ë ‰ËÒÚ‡ÎÌËÚ ˜‡ÒÚË Ì‡ Í‡ÈÌˈËÚÂ) Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÓÍÓÎÓ ÔÓıÓʉ‡ÌÂÚÓ (21). èÓÌflÍÓ„‡ ÏÛÒÍÛÎ̇ڇ Ò··ÓÒÚ ÏÓÊ ‰‡ ·˙‰Â ‰ËÒÍÂÚÌÓ ÔÓfl‚Â̇. ÇӉ¢  ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ Ò ÚÛ‰ÌÓÒÚË ‚ Á‡Û˜‡‚‡ÌÂÚÓ, ‰ËÁ‡ÚËfl, ËÁÓÒÚ‡‚‡Ì ‚ Û˜ËÎË˘Â Ë Á‡ÚÛ‰ÌÂ̇ ÒӈˇÎ̇ ËÌÚ„‡ˆËfl. ç‡Îˈ  ԇÚÓÎӄ˘̇ Ò˙ÌÎË‚ÓÒÚ. óÂÒÚË Ò‡ ÔÓfl‚ËÚ ÓÚ ÒÚ‡Ì‡ ̇ ëëë Ò ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËfl, ÍÓÂÚÓ Ó·ÛÒ·‚fl Ôӂ˯ÂÌËfl ËÒÍ ÓÚ ‚ÌÂÁ‡Ô̇ Ò˙‰Â˜Ì‡ ÒÏ˙Ú (40). äÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ Ò ̇·Î˛‰‡‚‡ ÔÓ-˜ÂÒÚÓ ÔË ÌÓ‚ÓÓ‰ÂÌËÚ ̇ χÈÍË Ò ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl. èË ÚÂÁË ‰Âˆ‡ ·ÓflÚ Ì‡ CTG- ÔÓ‚ÚÓËÚ ̇‰ı‚˙Îfl 1500 (7). ífl  ÎÂÚ‡Î̇ ‚ ÓÍÓÎÓ 16% ÓÚ ÒÎÛ˜‡ËÚÂ. èÂ̇ڇÎÌÓ Ò ÔÓfl‚fl‚‡ Ò ıˉ‡ÏÌËÓÌ, Ò ÓÚÒ··ÂÌË ‰‚ËÊÂÌËfl ̇ ÔÎÓ‰‡, ËÒÍ ÓÚ ‡ÚÓ„ËÔÓÁ‡ (29). èË ‡Ê‰‡ÌÂÚÓ ÓÒÌÓ‚ÌËÚ ÒËÏÔÚÓÏË Ò‡ Îˈ‚‡ ‰ËÔ΄Ëfl (tented upper lip), „ÂÌÂ‡ÎËÁË‡Ì‡ Ò··ÓÒÚ, ıËÔÓÚÓÌËfl Ë ÂÒÔË‡ÚÓÂÌ ‰ËÒÚÂÒ (3; 29). äÎËÌ˘ÌÓ ÎËÔÒ‚‡ ÏËÓÚÓÌËfl, ÍÓflÚÓ ÏÓÊ ‰‡ Ò ӷÂÍÚË‚ËÁË‡ ˜ÂÁ ÖåÉ (67). çË‚‡Ú‡ ̇ Í‡ÚËÌ ÍË̇Á‡ ‚ Í˙‚Ú‡ Ò‡ ÌÓχÎÌË, ÎËÔÒ‚‡Ú ÒÔˆËÙ˘ÌË ÔÓÏÂÌË ‚ ÏÛÒÍÛÎÌËÚ ·ËÓÔÒËË (68). åÓÚÓÌÓÚÓ ‡Á‚ËÚË ̇ ÚÂÁË ‰Âˆ‡  Á‡·‡‚ÂÌÓ, ÌÓ Ú Ò ̇ۘ‡‚‡Ú ‰‡ ıÓ‰flÚ. åÛÒÍÛÎ̇ڇ ıËÔÓÚÓÌËfl Ë ‰‚Ë„‡ÚÂÎ̇ڇ Ò··ÓÒÚ ÏÓ„‡Ú ‰‡ Ò ÔÓ‰Ó·flÚ Ò „Ó‰ËÌËÚÂ, ÌÓ ÔÓ-Í˙ÒÌÓ Ò ‡Á‚Ë‚‡Ú ÒËÏÔÚÓÏËÚ ̇ Í·Ò˘ÂÒ͇ڇ ÙÓχ. ÑÓ 11-„Ӊ˯̇ ‚˙Á‡ÒÚ Ò ÔÓfl‚fl‚‡Ú ÍÎËÌ˘ÂÒÍË Ë ÏËÓÚÓÌ˘ÌËÚ ÙÂÌÓÏÂÌË. 50-60% ÓÚ ‰Âˆ‡Ú‡ Ò‡ Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ. è˘Ë̇ڇ Á‡ ÚÓ‚‡ ‚Ò Ӣ Ì  flÒ̇, ‚˙ÔÂÍË ˜Â ÔË ‡Ê‰‡ÌÂÚÓ ˜ÂÒÚÓ Ò‡ ̇Îˈ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl, ıËÔÓÚÓÙËfl ̇ corpus callosum Ë ‚ÂÌÚËÍÛÎ̇ ‰Ë·ڇˆËfl. é·Ò˙ʉ‡ Ò ÍÓÏÔÎÂÍÒÌËfl ÂÙÂÍÚ Ì‡ DMPK-ÏÛÚ‡ˆËflÚ‡ Ë ‡Ì̇ڇ ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. äãàçàóçÄ äÄêíàçÄ èêà PROMM ífl  ÒıӉ̇, ÌÓ ÔÓ- ÎÂ͇ ÓÚ Ú‡ÁË ÔË Í·Ò˘ÂÒ͇ڇ ÙÓχ ̇ åÑ 1, Ò ÏËÓÚÓÌËfl, ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl, ͇ڇ‡ÍÚ‡, ‰Ë‡·ÂÚ, ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËfl. ÑËÒÚÓÙËflÚ‡ Á‡Òfl„‡ ÓÒÌÓ‚ÌÓ ÔÓÍÒËχÎÌËÚ ÏÛÒÍÛÎË (ÙÎÂÍÒÓËÚÂ Ë ÂÍÒÚÂÌÁÓËÚ ̇ ·Â‰‡Ú‡, ÙÎÂÍÒÓËÚ ̇ ¯ËflÚ‡, ÂÍÒÚÂÌÁÓËÚ ̇ Ï˯Ìˈ‡Ú‡), ·ÂÁ ‰‡ Ò ‡ÒÓˆËË‡ Ò ÚÂÊÍË ‡ÚÓÙËË Ì‡ ÏÛÒÍÛÎËÚ ̇ ÎˈÂÚÓ Ë Ô‰Ï˯ÌˈËÚÂ. ÄÌÚˈËÔ‡ˆËflÚ‡  ÏÌÓ„Ó ÔÓ-Ò··Ó ËÁ‡ÁÂ̇ ‚ Ò‡‚ÌÂÌËÂ Ò ·ÓÎÂÒÚÚ‡ ̇ Steinert (40). äéÉçàíàÇçà çÄêìòÖçàü, ëèÖñàîàóçà èêà êÄáãàóçàíÖ îéêåà çÄ åàéíéçàóçÄ Ñàëíêéîàü é˘Â ÔË Ô˙‚ËÚ ÓÔËÒ‡ÌËfl ̇ Ô‡ˆËÂÌÚË Ò ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl Ò ÓÚÍË‚‡Ú Ë ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl, ÍÓËÚÓ ‚‡Ë‡Ú ÓÚ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì ÔË ÍÓÌ„ÂÌËÚ‡Î-


ÑÂÍÂÏ‚Ë 2009

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

̇ڇ ÙÓχ (8) ‰Ó ÒÔˆËÙ˘ÌË ÍÓ„ÌËÚË‚ÌË, ÂÍÁÂÍÛÚË‚ÌË, ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌË, Ôӂ‰Â̘ÂÒÍË Ë ÔÒËı˘ÂÒÍË Ì‡Û¯ÂÌËfl ÔË Í·Ò˘ÂÒ͇ڇ ÙÓχ (4; 48). ë˙˘ÂÒÚ‚Û‚‡Ú ‡Á΢ÌË ÚÂÓËË ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÓ‚‡ ‰‡ÎË ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl, ˜Â Ò‡ Ò ÔÓ„ÂÒË‡˘ ËÎË ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ. Ç Á‡˘ËÚ‡ ̇ ÒÚ‡ÌӂˢÂÚÓ Á‡ ÎËÔÒ‡Ú‡ ̇ ÔÓ„ÂÒËfl  ÚÂÓËflÚ‡, ˜Â ÍÓ„ÌËÚË‚ÌËfl ÛÔ‡‰˙Í ÔÓ-ÒÍÓÓ Â ÂÁÛÎÚ‡Ú ÓÚ Ì‡Û¯ÂÌË ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ñçë, ‡ Ì ÒΉÒÚ‚Ë ÓÚ ‰Â„ÂÌÂ‡ÚË‚ÂÌ ÔÓˆÂÒ (25; 37; 41). è‰ÔÓ·„‡ ÒÂ, ˜Â Û̇ÒΉfl‚‡ÌÂÚÓ ÓÚ Ï‡È͇ڇ, ÍÓÂÚÓ ‚ Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë ÍÓÂÎË‡ Ò ÔÓ-„ÓÎflÏ ·ÓÈ Ì‡ ‡ÏÔÎËÙˈË‡ÌËÚ ÚËÌÛÍÎÂÓÚˉÌË ÔÓ‚ÚÓË, Â Ò ÏÌÓ„Ó ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ ‚ Ò‡‚ÌÂÌËÂ Ò Û̇ÒΉfl‚‡ÌÂÚÓ ÓÚ ·‡˘‡Ú‡. éÔËÒ‡ÌË Ò‡ Ë ÒÎÛ˜‡Ë Ò „ÓÎflÏ ·ÓÈ Ì‡ ‡ÏÔÎËÙˈË‡ÌËÚ ÔÓ‚ÚÓË, Ò ÚÂÊÍÓ ÒËÒÚÂÏÌÓ Á‡Òfl„‡ÌÂ, ÌÓ ·ÂÁ Ò˙˘ÂÒÚ‚ÂÌ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ (11; 53). íÓÁË Ù‡ÍÚ ÏÓÊ ‰‡ ·˙‰Â Ó·flÒÌÂÌ Ò˙Ò ÒÓχÚ˘ÂÌ ÏÓÁ‡ËˆËÁ˙Ï. ÉÓÎÂÏËflÚ ·ÓÈ Ì‡ ÔÓ‚ÚÓËÚÂ, ËÁÓÎË‡Ì ÓÚ Î‚ÍÓˆËÚËÚ ÍÓÂÎË‡ Ò ·Ófl ̇ ‡ÏÔÎËÙˈË‡ÌËÚ ÔÓ‚ÚÓË ‚ ÏËÓˆËÚËÚÂ, ÔÓ‡‰Ë Ó·˘Ëfl ËÏ ÂÏ·ËÓ̇ÎÂÌ ÔÓËÁıÓ‰ ÓÚ ÏÂÁÓ‰Âχڇ, ÌÓ Ì ‚Ë̇„Ë Ò˙ÓÚ‚ÂÚÒÚ‚‡ ̇ ÂÍÒÔ‡ÌÁËflÚ‡ ̇ ÔÓ‚ÚÓËÚ ‚ Ì‚ÓÌËÚÂ. èÓÚË‚ÓÂ˜Ë‚Ë Ò‡ ‰‡ÌÌËÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ù‡ÍÚ‡ ‰‡ÎË ÍÓ„ÌËÚË‚ÌËflÚ ‰ÂÙˈËÚ ÔË Í·Ò˘ÂÒ͇ڇ ÙÓχ  ÂÁÛÎÚ‡Ú Ì‡ ‰Â„ÂÌÂ‡ÚË‚ÂÌ ÔÓˆÂÒ (5; 8). ìÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡ÌÂ Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ  ÏÌÓ„Ó ı‡‡ÍÚÂÌÓ Á‡ ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ. á‡Òfl„‡Ú Ò ӷ˘ÓÚÓ ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ÙÛÌ͈ËÓÌË‡Ì (Ò ËÁfl‚‡ ̇ ÎÂ͇ ‰Ó ÛÏÂÂÌÓ ÚÂÊ͇ ÒÚÂÔÂÌ Ì‡ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ), ‚Â·‡ÎÌËÚ ÒÔÓÒÓ·ÌÓÒÚË, ÙÓÌÚ‡ÎÌËÚÂ Ë ÂÍÁÂÍÛÚË‚ÌË ÙÛÌ͈ËË ( 31; 48; 71). Harper Ë Ò˙‡‚Ú. ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â Ò‰ÌËflÚ IQ (Intelligence Quotient- ÍÓÂÙˈËÂÌÚ Ì‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ) ÔË ÚÂÁË Ô‡ˆËÂÌÚË Â ÓÍÓÎÓ 66 (29; 57). àÌÚÂÂÒÌÓ Â, ˜Â ÔË ÌflÍÓË ·ÓÎÌË ÛÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡Ì ÏÓÊ ‰‡ ÓÒڇ̠‰ËÌÒÚ‚Â̇ ÍÎËÌ˘̇ ËÁfl‚‡ Á‡ ÌflÍÓÎÍÓ „Ó‰ËÌË ÒΉ ‡Ê‰‡ÌÂÚÓ (64). èË ‰Âˆ‡ Ò Ú‡ÁË ÙÓχ Ò ÓÚÍË‚‡Ú ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl, ıËÔÓÔ·ÁËfl ̇ corpus callosum Ë ‚ÂÌÚËÍÛÎ̇ ‰Ë·ڇˆËfl Ӣ ÒΉ ‡Ê‰‡ÌÂÚÓ, ÍÓËÚÓ ÍÓÂÎË‡Ú Ò ÚÂÊÂÒÚÚ‡ ̇ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì (37). ë˙‚ÍÛÔÌÓÒÚÚ‡ ÓÚ ÏÛÒÍÛÎÌÓ Á‡Òfl„‡ÌÂ, ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl Ë åêí-̇ıӉ͇ڇ Ó·ÛÒ·‚flÚ ‚˙ÁÏÓÊÌÓÒÚÚ‡ ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ ̇ åÑ 1 ‰‡ Ò ÓÚÌÂÒ Í˙Ï ÏËÓÂ̈ÂÙ‡ÎÓÔ‡ÚËËÚ (41). èË ÏËÓÚÓÌ˘̇ڇ ‰ËÒÚÓÙËfl ̇ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó ÚËÔ˘̇ ÓÒÓ·ÂÌÓÒÚ Ò‡ Ó·Û˜ËÚÂÎÌËÚ Á‡ÚÛ‰ÌÂÌËfl, ÍÓËÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË 69-86,7% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ͇ÚÓ ÓÒÌÓ‚ÌËÚ ÚÛ‰ÌÓÒÚË Ò‡ ÔË ˜ÂÚÂÌÂÚÓ (15; 26; 22). ç‚ÓÔÒËıÓÎӄ˘ÂÒÍËÚ ËÁÒΉ‚‡ÌËfl ̇ Ó·˘ÓÚÓ ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ÙÛÌ͈ËÓÌË‡Ì ÔÓ͇Á‚‡Ú IQ ÔÓ-ÌËÒ˙Í ÓÚ Â‰ÌÓ Òڇ̉‡ÚÌÓ ÓÚÍÎÓÌÂÌË ÔÓ‰ ÌÓχڇ, ͇ÚÓ ÏÂÊ‰Û 20% Ë 80% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÒÔÓ‰ ‡Á΢ÌË ËÁÒΉ‚‡ÌËfl, Ò‡ Ò ‡Á΢̇ ÒÚÂÔÂÌ Ì‡ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, Ú.Â. IQ ÔÓ‰ 70% (4; 43; 44 65; 66). íÂÁË ‰Âˆ‡ Ò‡ Ò ÔÓ- ‚ËÒÓÍÓ IQ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚÂ Ò ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ (65). èË ÔÓ‚Âʉ‡Ì ̇ Ì‚ÓÔÒËıÓÎӄ˘ÂÒÍË ÚÂÒÚÓ‚Â Ò ˉÂÌÚËÙˈË‡Ú ÌflÍÓË ÒÔˆËÙ˘ÌË ‰ÂÙˈËÚË: ‚˙‚ ‚ÌËχÌËÂÚÓ, ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ, ÍÓÌÒÚÛÍÚË‚ÌËÚ ÛÏÂÌËfl Ë ‚Â·‡Î̇ڇ Ô‡ÏÂÚ. íÂÁË ‰Âˆ‡ Ò‡ Ò ÔÓÚÂ̈ˇÎÂÌ ‰ÂÙˈËÚ Ì‡ ÂÍÁÂÍÛÚË‚ÌËÚÂ Ë ÔÂˆÂÔÚË‚ÌÓ-ÏÓÚÓÌËÚ ÙÛÌ͈ËË ‚ ÏÂÊ‰Û 50 Ë 90%. ëÔÓ‰ ÏӉ· ̇ Baddeley ‡·ÓÚ̇ڇ Ô‡ÏÂÚ Ò Ò˙ÒÚÓË ÓÚ ˆÂÌÚ‡Î̇ ÂÍÁÂÍÛÚ˂̇ ÒËÒÚÂχ, ‚Íβ˜Â̇ ‚ ËÁ·Ó‡ Ë ÛÔ‡‚ÎÂÌËÂÚÓ Ì‡ ‰ÂÈÌÓÒÚËÚ ÔÓ ‡ÁÔ‰ÂÎÂÌË ̇ ‚ÌËχÌËÂÚÓ (10). Ç˙ÁÏÓÊÌÓ Â ËÌÚÂÎÂÍÚÛ‡ÎÌÓÚÓ Á‡Òfl„‡Ì ÔË ‰ÂÚÒ͇ڇ ÙÓχ ̇ DM1 ‰‡ Ò ‰˙ÎÊË Í‡ÍÚÓ Ì‡ ÙÓÌÓÎӄ˘̇ Ô˘Ë̇, ‚ ÒÏËÒ˙Π̇ Ò˙ı‡ÌÂÌË ̇ ËÌÙÓχˆËfl Ë ‰ÂÙˈËÚ Ì‡ ‚ÌËχÌËÂÚÓ, ڇ͇ Ë Ì‡ ̇Û¯ÂÌË ‚ Ô·ÌË‡ÌÂÚÓ Ë Ó„‡ÌËÁ‡ˆËflÚ‡ ̇ ‰ÂÈÌÓÒÚËÚÂ. àÁ‚ÂÒÚÌÓ Â, ˜Â ÚÂÁË ÍÓ„ÌË-

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ÚË‚ÌË ÛÏÂÌËfl Ò‡ ÙÛÌ͈Ëfl ̇ ÍÓÚË͇ÎÌË ÏÂÊË ‚˙‚ ÙÓÌÚÓ-ÚÂÏÔÓ‡ÎÌËÚ „ËÓÌË, ÓÒÌÓ‚ÌÓ ‰ÓÁÓ·ÚÂ‡ÎÌËfl ÔÂÙÓÌÚ‡ÎÂÌ ÍÓÚÂÍÒ, ÍÓÈÚÓ ÔÂÚ˙Ôfl‚‡ Á̇˜ËÚÂÎÌÓ ‡Á‚ËÚË ÔÂÁ ÔÂËÓ‰‡ ̇ ‰ÂÚÒÚ‚ÓÚÓ (4). èË ÓÍÓÎÓ 26% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò åÑ Ì‡ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó Ò ÓÚÍË‚‡Ú ıËÔÂ‡ÍÚË‚ÌÓ Ôӂ‰ÂÌËÂ Ò ‰ÂÙˈËÚ Ì‡ ‚ÌËχÌËÂÚÓ, ‡ ÔË 40 %- Ôӂ‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl (4). ê‡Á΢ÌË ÙÓÏË Ì‡ Ú‚ÓÊÌÓÒÚ (Ó·˘‡, ÓÚ ‡Á‰fl· Ë Ú.Ì.) Ë ÙÓ·ËË Ò ÓÔËÒ‚‡Ú ÔË ÚÂÁË Ô‡ˆËÂÌÚË (26; 65). èË Í·Ò˘ÂÒ͇ڇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ Steinert ÔÓÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ IQ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË Ô‡ˆËÂÌÚË Ò ÔÓ-„ÓÎflχ CTG-ÂÍÒÔ‡ÌÁËfl Ë Û̇ÒΉfl‚‡Ì ÓÚ Ï‡È͇ڇ. ÑÓË Ô‡ˆËÂÌÚË Ò IQ ·ÎËÁ˙Í ‰Ó ÌÓχÎÌËfl ÔÓ͇Á‚‡Ú ̇Û¯ÂÌË ‚ ÂÍÁÂÍÛÚË‚ÌËÚÂ Ë ÂÁËÍÓ‚ËÚ ÙÛÌ͈ËË, ‚ÌËχÌËÂÚÓ, ÁËÚÂÎ̇ڇ ÔÂˆÂÔˆËfl (14) Ë Ô‡ÏÂÚ, ͇ÍÚÓ Ë ‚ ÍÓÌÒÚÛÍÚË‚ÌËÚ ÒÔÓÒÓ·ÌÓÒÚË (44; 49, 58). èÓÍÓÌÍÂÚÌÓ ‚ ÚÂÊ͇ ÒÚÂÔÂÌ Â Á‡Ò„̇ÚÓ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌÓÚÓ ÔËÔÓÏÌflÌÂ Ë ‚ ÔÓ-ÎÂ͇ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚Â̇ڇ ÍÓÌÒÚÛÍÚË‚ÌÓÒÚ, ‚Â·‡Î̇ڇ Í‡ÚÍÓÒӘ̇ Ë ‰˙΄ÓÒӘ̇ Ô‡ÏÂÚ ( 43). ç‡Û¯ÂÌËflÚ‡ ‚ ÔÓÒÚ‡ÌÒÚ‚Â̇ڇ ÓËÂÌÚ‡ˆËfl Ë ÁËÚÂÎ̇ڇ Ô‡ÏÂÚ ÍÓÂÎË‡Ú Ò ÛÒÚ‡ÌÓ‚Â̇ڇ ıËÔÓÔÂÙÛÁËfl ‚ Á‡‰ÌËÚ ÍÓÚË͇ÎÌË „ËÓÌË Í‡ÚÓ cuneus Ë ÂÚÓÒÔÎÂÌˇÎÌËfl ÍÓÚÂÍÒ, ͇ÍÚÓ Ë ‰ÂÒÌËfl Ô‡ËÂÚ‡ÎÂÌ ÎÓ· (43). IQ Ì ÍÓÂÎË‡ Ò ÚÂÊÂÒÚÚ‡ ̇ ÏÛÒÍÛÎÌÓÚÓ Á‡Òfl„‡Ì (49). Rubinstein Ë Ò˙‡‚Ú. 1997 Ô‰ÔÓ·„‡Ú, ˜Â Ô‡ÏÂÚÓ‚ËÚ ÙÛÌ͈ËË ÏÓ„‡Ú ‰‡ Ò‡ ÔÓ-˜Û‚ÒÚ‚ËÚÂÎÌË Ì‡ χÎ˙Í ·ÓÈ Ì‡ CTG-ÔÓ‚ÚÓËÚ ‚ Ò‡‚ÌÂÌËÂ Ò Ó·˘ÓÚÓ ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ÙÛÌ͈ËÓÌË‡Ì ÔË ÎÂÍËÚ ÙÓÏË Ì‡ ÏËÓÚÓÌ˘ÌËÚ ‰ËÒÚÓÙËË (43). èË Ô‡ˆËÂÌÚË Ò Ï‡Î˙Í ·ÓÈ Ì‡ ‡ÏÔÎËÙˈË‡ÌËÚ ÔÓ‚ÚÓË Ì‡‰ ÌÓχڇ, ÍÓËÚÓ ÏÓÊ ‰‡ Ò‡ ÓÎË„Ó- ËÎË ‡ÒËÏÔÚÓÏÌË ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÏÛÒÍÛÎÌÓÚÓ Á‡Òfl„‡ÌÂ, Ò Ì‡Ô‰‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Á̇˜ËÏÓ Á‡Òfl„‡Ì ̇ ‰˙΄ÓÒӘ̇ڇ Ë ÁËÚÂÎ̇ڇ Ô‡ÏÂÚ, ÍÓÂÚÓ Ò‚Ë‰ÂÚÂÎÒÚ‚‡ Á‡ ‰ËÒÙÛÌ͈Ëfl ̇ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (48). èË „ÓÎflχ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚ ҇ Á‡Ò„̇ÚË ÙÛÌ͈ËËÚ ̇ ÙÓÌÚ‡ÎÌËfl ‰flÎ, ͇ÚÓ Ô·ÌË‡ÌÂ, ÍÓÌÚÓΠ̇ ‚ÌËχÌËÂÚÓ, ÍÓÂÚÓ ÏÓÊ ‰‡ ‚ÎËfl ‚˙ıÛ ÍÓ„ÌˈËflÚ‡. çflÍÓË ÔÓÛ˜‚‡ÌËfl Ô‰ÔÓ·„‡Ú Ò˙˘ÂÒÚ‚Û‚‡ÌÂÚÓ Ì‡ ÚÂÒÌË ‚˙ÁÍË Á‡‰‡˜ËÚ Á‡ ¯‡‚‡Ì ̇ ÔÓ·ÎÂÏË, ·ÚÂ‡ÎÌËfl ÔÂÙÓÌÚ‡ÎÂÌ ÍÓÚÂÍÒ Ë Ó·˘‡Ú‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ. è‰ÔÓ·„‡ ÒÂ, ˜Â ÍÓ„ÌËÚË‚ÌËflÚ ÛÔ‡‰˙Í, ‚ ˜‡ÒÚÌÓÒÚ ÒÏÛ˘ÂÌËÂÚÓ Ì‡ ÂÍÁÂÍÛÚË‚ÌËÚÂ Ë ÂÁËÍÓ‚ËÚ ÙÛÌ͈ËË ÏÓÊ ÔÓ„ÂÒË‡ Ò ‚˙Á‡ÒÚÚ‡, ÌÓ Ì  ÛÒÚ‡ÌÓ‚Â̇ ÔÓ-„ÓÎflχ ÚÂÊÂÒÚ Ë ÔÓ-·˙Á‡ ÔÓ„ÂÒËfl ÔË Ô‡ˆËÂÌÚË Ò ÔÓ-„ÓÎflχ CTG-ÂÍÒÔ‡ÌÁËfl (49; 58). èÓ„ÂÒËflÚ‡ Ò ӷflÒÌfl‚‡ Ò Ì‡ÚÛÔ‚‡ÌÂÚÓ Ì‡ ÏÛÚ‡ÌÚÌË DMPK-Ú‡ÌÒÍËÔÚË ‚ fl‰‡Ú‡ ̇ Ì‚ÓÌËÚÂ, ÍÓËÚÓ ÔÓ ÚÓÁË Ì‡˜ËÌ Ì‡Û¯‡‚‡Ú ‡ÎÚÂ̇ÚË‚ÌËfl ÒÔ·ÈÒËÌ„ Ë „Û·ˆËflÚ‡ ̇ Ú‡ÌÒÍËÔˆËflÚ‡. èË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚ Ì‡ Steinert Ò‡ ÓÔËÒ‡ÌË Ì‡Ï‡ÎÂ̇ ÒÔÓÒÓ·ÌÓÒÚ Á‡ ‡ÁÔÓÁ̇‚‡Ì ̇ Îˈ‚‡Ú‡ ÂÍÒÔÂÒËfl Ë ÂÏÓˆËÓ̇ÎÌÓ ÙÛÌ͈ËÓÌË‡Ì (69;75), ÍÓÂÚÓ ‚ÂÓflÚÌÓ Â Ò‚˙Á‡ÌÓ Ò˙Ò Á‡Òfl„‡Ì ̇ ÎËϷ˘ÌËÚ ÒÚÛÍÚÛË- ‰‚ÛÒÚ‡ÌÌË ÒÛ·ÍÓÚË͇ÎÌË ÎÂÁËË ‚ Ô‰ÌËÚ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËÚ ‰flÎÓ‚Â, ‚ÍÎ. ‡ÏË„‰‡Î‡Ú‡ Ë ÂÌÚÓË̇ÎÌËfl ÍÓÚÂÍÒ ËÎË Ó·‡·ÓÚ͇ڇ ̇ ÔÓÎÛ˜Â̇ڇ ËÌÙÓχˆËfl ÓÚ ÚÂÏÓ‡ÎÌËÚ ËÎË ÙÓÌÚ‡ÎÌËÚ ‰flÎÓ‚Â. ÄÏË„‰‡Î‡Ú‡ Ò ‡ÒÓˆËË‡ Ò ‡ÁÔÓÁ̇‚‡Ì ̇ Îˈ‚‡Ú‡ ÂÍÒÔÂÒËfl ̇ ÓÚˈ‡ÚÂÎÌË ÂÏÓˆËË Í‡ÚÓ ÒÚ‡ı Ë „Ìfl‚. ÇÂÓflÚÌÓ ËÌÒÛ·ڇ Ëχ ÔÓ‰Ó·ÌË ÙÛÌ͈ËË (2). ç‡Ï‡ÎÂ̇ڇ ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ Í˙Ï Ì„‡ÚË‚ÌË ÂÏÓˆËË ÏÓÊ ‰‡  Ô˘Ë̇ ÚÂÁË Ô‡ˆËÂÌÚË ‰‡ Ò‡ ÔÓ-Ò··Ó ÍÓÓÔÂ‡ÚË‚ÌË Ë ÒÍÎÓÌÌË Í˙Ï ÂÏÔ‡ÚËfl (75). ã˘ÌÓÒÚÓ‚Ë Ë Ôӂ‰Â̘ÂÒÍË (͇ÚÓ ÔÓ‰ÓÁËÚÂÎÌÓ Ôӂ‰ÂÌËÂ, „ӈÂÌÚ˘ÌÓÒÚ Ë ·ÂÁ‡Á΢ËÂ) ̇Û¯ÂÌËfl ÔË ÚÂÁË ·ÓÎÌË Ò‡ ÓÔËÒ‡ÌË Ë ÓÚ Ò‡ÏËfl Steinert. ÅÓÎÌËÚ ҇ Ò ıÓÏÓ„ÂÌÂÌ Ôӂ‰Â̘ÂÒÍË ÔÓÙËÎ Ò˙Ò ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏË ‡Á΢Ëfl Á‡ ÓÚ·fl„‚‡ÌÂ, Ó·ÒÂÒË‚ÌÓ-ÍÓÏÔÛÎÒË‚ÌË, Ô‡-


ÑÂÍÂÏ‚Ë 2009

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

ÒË‚ÌÓ-‡„ÂÒË‚ÌË Ë ¯ËÁÓˉÌË ˜ÂÚË (20) èӂ‰ÂÌËÂÚÓ Ì‡ ÓÚ·fl„‚‡Ì Ò ı‡‡ÍÚÂËÁË‡ Ò˙Ò ÒÚ‡ı ÓÚ ÚÓ‚‡ ‰‡ ·˙‰‡Ú ÓˆÂÌfl‚‡ÌË, ÍËÚËÍÛ‚‡ÌË ËÎË ÓÚıÎ˙ÎÂÌË ÓÚ ‰Û„ËÚÂ. íÂÁË Ô‡ˆËÂÌÚË ÚÛ‰ÌÓ Ò ÒÔËflÚÂÎfl‚‡Ú ËÎË Á‡ÔÓ˜‚‡Ú ÌÓ‚‡ ‡·ÓÚ‡. ÑÂÔÂÒËflÚ‡ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ˜ÂÒÚÓ, ÌÓ Ì  flÒÌÓ ‰‡ÎË Â Ô˙‚˘̇ ËÎË ‚ÚÓ˘̇, ÔÓ‡‰Ë ÙËÁ˘ÂÒÍËÚ ӄ‡Ì˘ÂÌËfl, ‰Ó ÍÓËÚÓ ‚Ó‰Ë Á‡·ÓÎfl‚‡ÌÂÚÓ. ÇÒ˙˘ÌÓÒÚ Ô‡ˆËÂÌÚËÚ ÌflÏ‡Ú ÚÂÊÍË ÒËÏÔÚÓÏË Ì‡ Ú‚ÓÊÌÓÒÚ ËÎË ‰ÂÔÂÒËfl, ‡ ÔÓ-ÒÍÓÓ- ÂÏÓˆËÓ̇ÎÂÌ ‰ÂÙˈËÚ (43). èË Ô‡ˆËÂÌÚË Ò åÑ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ë Ôӂ˯Â̇ ‰Ì‚̇ Ò˙ÌÎË‚ÓÒÚ, ÍÓflÚÓ ‚ÂÓflÚÌÓ Â Ò ÍÓÏÔÎÂÍÒÂÌ ı‡‡ÍÚÂ, Ó·ÛÒÎÓ‚Â̇  ÓÚ ÔÓ„ÂÒË‡˘‡Ú‡ ÂÒÔË‡ÚÓ̇ ‰ËÒÙÛÌ͈Ëfl Ë ˜ÂÒÚËÚ ÔÓfl‚Ë Ì‡ ‰ÂÔÂÒËfl (54; 55). äÓ„ÌËÚ‚ÌË Ì‡Û¯ÂÌËfl ÔË Ô‡ˆËÂÌÚË Ò PROMM ÇÒ Ӣ Ì  flÒÌÓ ‰‡ÎË ÒÔÂÍÚ˙˙Ú Ì‡ ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl ÔË PROMM e ÒıÓ‰eÌ Ò ÚÂÁË ÔË ·ÓÎÂÒÚÚ‡ ̇ Steinert (43). ìÒÚ‡ÌÓ‚ÂÌË Ò‡ ̇Û¯ÂÌËfl ‚ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚ ÛÏÂÌËfl ‚ ÔÓ-ÎÂ͇ ÒÚÂÔÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò ÚËÔ I. äÓ„ÌËÚË‚ÌËÚ ‡·ÌÓÏÌÓÒÚË Ì ÍÓÂÎË‡Ú Ò ÔÓÏÂÌËÚ ‚ åêí ̇ıӉ͇ڇ (18). Ñˇ„ÌÓÒÚ˘ÌËflÚ ‡Î„ÓËÚ˙Ï ‚Íβ˜‚‡: Ä̇ÏÌÂÁ‡, ‚ÍÎ. Ù‡ÏËÎ̇; ç‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ; ÅËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl (ëêä  ÌÓχÎ̇; ïÓÏÓ̇ÎÌË ËÁÒΉ‚‡ÌËfl- Á‡ ÓÔ‰ÂÎflÌ ̇ ÙÛÌ͈ËflÚ‡ ̇ Ô‡ÌÍ‡҇ Ë „Ó̇‰Ì‡Ú‡ ÓÒ); ÖåÉ Ò ‰‡ÌÌË Á‡ ÏËÓÚÓÌ˘ÌË Á‡ÎÔÓ‚Â Ë ÏËÓ„ÂÌ̇ Û‚‰‡; ÖäÉ, ïÓÎÚÂ-ÖäÉ Ë ÖıÓäÉ ● Ö‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË á‡Òfl„‡ÌÂÚÓ Ì‡ ñçë ÔË Ô‡ˆËÂÌÚË Ò ÏËÓÚÓÌ˘̇ ‰ËÒÚÓÙËfl Ò ӷÂÍÚË‚ËÁË‡ Ë ˜ÂÁ ËÁÒΉ‚‡ÌÂÚÓ Ì‡ ÒÎÛıÓ‚ËÚ (70) Ë ÁËÚÂÎÌËÚ (24) ‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË. çflÍÓË ËÁÒΉ‚‡ÌËfl ÛÒÚ‡ÌÓ‚fl‚‡Ú Û‰˙ÎÊÂÌË ˆÂÌÚ‡ÎÌË ‚ÂÏÂ̇ ̇ ÔÓ‚Âʉ‡Ì ÔÓ ÒÓχÚÓÒÂÌÁÓÌËÚ Ô˙Úˢ‡ (24; 27;60; 72) Ë Ôӂ˯Â̇ ÌÂ‚̇ ‚˙Á·Û‰ËÏÓÒÚ Ì‡ Ô˙‚˘ÌËfl ÒÂÚË‚ÂÌ ÍÓÚÂÍÒ (47), ÍÓÂÚÓ Ô‰ÔÓ·„‡ ÒÛ·ÍÎËÌ˘ÌÓ Á‡Òfl„‡Ì ̇ ÒÓχÚÓÒÂÌÁÓÌËÚ Ô˙Úˢ‡ Ë Ô˙‚˘̇ڇ ÒÂÚ˂̇ ÍÓ‡. ë‡‚ÌËÚÂÎÌÓ Ï‡ÎÍÓ Ò‡ Ôӂ‰ÂÌËÚ ÔÓÛ˜‚‡ÌËfl, ËÁÛ˜‡‚‡˘Ë ÙÛÌ͈ËÓÌË‡ÌÂÚÓ Ì‡ ˆÂÌÚ‡Î̇ڇ ÒËÒÚÂχ ̇ ÏÓÚÓÂÌ ÍÓÌÚÓÎ. Oliveri Ë Ò˙‡‚Ú. ÔÂÁ 1997 ˜ÂÁ Ú‡ÌÒÍ‡ÌˇÎ̇ χ„ÌËÚ̇ ÒÚËÏÛ·ˆËfl ̇ ÏÓÚÓ̇ڇ ÍÓ‡ ÛÒÚ‡ÌÓ‚fl‚‡Ú Á̇˜ËÚÂÎÌÓ Û‰˙ÎÊÂÌÓ ˆÂÌÚ‡ÎÌÓ ÏÓÚÓÌÓ ‚ÂÏ ̇ ÔÓ‚Âʉ‡Ì (50). ë‚˙Á‡ÌËÚÂ Ò ‰‚ËÊÂÌË ÍÓÓ‚Ë ÔÓÚÂ̈ˇÎË Ò‡ ÂÁÛÎÚ‡Ú ÓÚ ËÁ‚˙¯‚‡Ì ̇ ‚ÓÎÂ‚Ë ‰‚ËÊÂÌËfl Ë Ò‡ ‚‡ÊÌË Á‡ ÓˆÂÌ͇ڇ ̇ ÔÓ‰„ÓÚӂ͇ڇ Ë ËÁ‚˙¯‚‡ÌÂÚÓ Ì‡ ‰‚ËÊÂÌËflÚ‡ ̇ ˆÂÌÚ‡ÎÌÓÌÂ‚ÌÓ ÌË‚Ó (63). ÄÍÓ Ò Ò‡‚ÌflÚ NS’ (negative slope) Ë BP (Bereitshaftpotential) ÔË Ô‡ˆËÂÌÚËÚÂ Ò åÑ, NS’  Á̇˜ËÏÓ ÒÌËÊÂÌ Í‡ÚÓ ‡ÏÔÎËÚÛ‰‡ ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË Ë Ô‡ˆËÂÌÚË Ò ‰Û„Ë Ì‚ÓÏÛÒÍÛÎÌË Á‡·ÓÎfl‚‡ÌËfl ͇ÚÓ ëÔË̇Î̇ ÏÛÒÍÛÎ̇ ‡ÚÓÙËfl, ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl ÚËÔ ÔÓflÒ-Í‡ÈÌËÍ Ë Ù‡ˆËÓÒ͇ÔÛÎÓıÛÏÂ‡Î̇ ÏÛÒÍÛÎ̇ ‰ËÒÚÓÙËfl. íÂÁË ÓÚÍËÚËfl Ô‰ÔÓ„‡Ú ‰ËÒÙÛÌ͈Ëfl ̇ ÒËÒÚÂχڇ ̇ ˆÂÌÚ‡ÎÂÌ ÏÓÚÓÂÌ ÍÓÌÚÓÎ, Ò‚˙Á‡Ì‡ Ò ÔÓ‰„ÓÚӂ͇ڇ Ë ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ ‰‚ËÊÂÌËflÚ‡, Ó·ÛÒ·‚fl˘ËÚ ÏÂı‡ÌËÁÏË, Á‡ ÍÓÂÚÓ ‚Ò Ӣ Ì ҇ ËÁ‚ÂÒÚÌË (46). ● ç‚ÓÔÒËıÓÎӄ˘ÂÒÍË ËÁÒΉ‚‡ÌËfl óÂÁ Úflı Ò ËÁÛ˜‡‚‡Ú ‡Á΢ÌËÚ ‡ÒÔÂÍÚË Ì‡ ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ ÔË ÚÂÁË Ô‡ˆËÂÌÚË. ● é·‡ÁÌË ËÁÒΉ‚‡ÌËfl ̇ ñçë: åêí ÔË Ô‡ˆËÂÌÚË Ò åÑ ÚËÔ I ÔÓ͇Á‚‡Ú ÍÓÓ‚‡ ‡ÚÓÙËfl, ÔÓÏÂÌË ‚ ÔÓ‰ÍÓÓ‚ÓÚÓ ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó ÔÓ ÚËÔ‡ ̇ ‰ÂÏËÂÎËÌËÁ‡ˆËË, ÓÒÌÓ‚ÌÓ ‚˙‚ ÙÓÌÚ‡ÎÌËÚ ‰flÎÓ‚Â Ë Ô‰ÌËÚ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËÚ ‰flÎÓ‚Â, ͇ΈËÙË͇ÚË ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË (16; 34). Ç˙ÔÂÍË ˜Â ÚÂÊÂÒÚÚ‡ ̇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ì ÍÓÂÎË‡ ̇Ô˙ÎÌÓ Ò ÚÂÊÂÒÚÚ‡ ̇ ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ, ÎÂÁËËÚ ‚ ·flÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó, ‡·ÌÓÏÌÓÒÚËÚ ‚ Ô‰ÌËÚ ÚÂÏÔÓ‡ÎÌË ‰flÎÓ‚Â Ë ‚ÂÌÚËÍÛÎÓÏ„‡ÎËflÚ‡ Ò‡ ÏÌÓ„Ó ÔÓ-ÚËÔ˘ÌË ÔË Ô‡ˆËÂÌÚË Ò ÚÂÊÍÓ Á‡ÒÂ„Ì‡Ú ËÌ-

àÁÒΉ‚‡ÌË ÍÓ„ÌËÚË‚ÌË ÙÛÌ͈ËË é·˘Ó ËÁÒΉ‚‡Ì ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË è‡ÏÂÚ ÇÂ·‡Î̇-ÒÎÛıÓ‚‡ áËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚Â̇ Rey complex figure test EÁËÍÓ‚‡ ÙÛÌ͈ËË Controlled association letters test ÖÍÁÂÍÛÚË‚ÌË ÙÛÌ͈ËË Ë ‚ÌËχÌËÂ

ÇÂ·‡Î̇ ÙÎÛÂÌÚÌÓÒÚ óÂÚÂÌÂ

191

ç‚ÓÔÒËıÓÎÓ-„˘ÂÌ ÚÂÒÚ Wechsler intelligence scale for children Stanford-Binet scale Raven progressive matrices Digit span Rey auditory verbal learning Story recall Block span Rey-Ostereich figure recall Spatial learning test Token test Controlled association category test Stroop test Trial making test Wisconsin card sorting test Tower of London Alertness without auditory warning signal Alertness with auditory warning signal Divided attention Verbal fluency test (semantic and phonetic) National adult reading test

ÚÂÎÂÍÚ (32; 45). ᇠԇˆËÂÌÚË Ò ÍÓÌ„ÂÌËÚ‡Î̇ ÙÓχ ̇ åÑ 1 ÔÓ-ÚËÔ˘ÌË Ò‡ ÍÓÓ‚‡Ú‡ ‡ÚÓÙËfl, ıËÔÓÔ·ÁËfl ̇ corpus callosum Ë ‚ÂÌÚËÍÛÎÓÏ„‡ÎËflÚ‡ (16). ãÂÁËË ‚ ÔÓ‰ÍÓÓ‚ÓÚÓ ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó ÔË ÚÂÁË Ô‡ˆËÂÌÚË Ò ÓÚÍË‚‡Ú ‚ ÔÓ-̇Ô‰̇· ‚˙Á‡ÒÚ. ëÔˆËÙ˘̇ ÓÒÓ·ÂÌÓÒÚ Ò‡ ıËÔÂËÌÚÂÌÁÌËÚ ÎÂÁËË ‚ „ÓÌËfl Á‡‰ÂÌ ÚË˙„˙ÎÌËÍ – trigone (37). èË Í·Ò˘ÂÒ͇ڇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ Steinert ÔÓı‡‡ÍÚÂÌË Ò‡ ıËÔÂËÌÚÂÌÁÌËÚ ̇ í2 ÁÓÌË, ‡ÁÔÓÎÓÊÂÌË ÔÓ‰ÍÓÓ‚Ó ‚ ÚÂÏÔÓ‡ÎÌËÚÂ, ÙÓÌÚ‡ÎÌËÚ ‰flÎÓ‚Â Ë ÔÂË‚ÂÌÚËÍÛ·ÌÓ ( 9; 36). ç‡ 3-D åêí ËÁÒΉ‚‡ÌËfl, ÔË ÍÓËÚÓ ÏÓÁ˙˜ÌÓ-Ô‡ÂÌıËÏ̇ڇ Ù‡ÍˆËfl ÔË åÑ 1 Ë åÑ 2 Ò Ò‡‚Ìfl‚‡ Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË, ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â ÔË ·ÓÎÂÒÚÚ‡ ̇ Steinert ÏÓÁ˙˜Ì‡Ú‡ ‡ÚÓÙËfl  ÏÌÓ„Ó ÔÓ- ÚÂÊÍÓ ËÁ‡ÁÂ̇ ‚ Ò‡‚ÌÂÌË Ò˙Ò Ò˙ÓÚ‚ÂÚÌË ÔÓ ‚˙Á‡ÒÚ Ë̉˂ˉË. èË ·ÓÎÌË Ò PROMM ÚÂÁË ËÁÏÂÌÂÌËfl Ò‡ ÔÓ- Ò··Ó ËÁ‡ÁÂÌË Ë Ò˙˘ÂÒÚ‚Û‚‡ ÚflÒ̇ ÍÓ·ˆËfl Ò ‚˙Á‡ÒÚÚ‡ ̇ Ô‡ˆËÂÌÚËÚÂ, Ú. Â. ‡ÚÓÙËflÚ‡ ÔÓ„ÂÒË‡ Ò ‚˙Á‡ÒÚÚ‡, ÍÓÂÚÓ Â ÚËÔ˘ÌÓ Ë Á‡ Á‰‡‚ËÚ ÍÓÌÚÓÎË. îÛÌ͈ËÓ̇ÎÌË Ó·‡ÁÌË ËÁÒΉ‚‡ÌËfl: ç‡ SPECT (Single Photon Emission Computed Tomography) Ë PET (Positron Emission Tomography) Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ·Ë·ÚÂ‡ÎÌÓ Ì‡Ï‡ÎÂ̇ ÔÂÙÛÁËfl Ë ‰ۈË‡Ì „βÍÓÁÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï ‚ „ÓÌËfl ÏÂÁˇÎÂÌ ÙÓÌÚ‡ÎÂÌ ÍÓÚÂÍÒ, Ô‰ÌËfl ˆËÌ„Û·ÂÌ, Ó·ËÚÓÙÓÌÚ‡ÎÌËfl, ÂÚÓÒÔÎÂÌˇÎÌËfl ÍÓÚÂÍÒ, ÓÔ‡¯‡ÚÓÚÓ fl‰Ó, ڇ·ÏÛÒ‡ Ë ÚÂÏÔÓÓÔ‡ËÂÚ‡ÌËÚ ‰flÎÓ‚Â (42). íËÔÎÂÚ̇ڇ ÂÍÒÔ‡ÌÁËfl ̇Û¯‡‚‡ ̇‚ÎËÁ‡ÌÂÚÓ Ì‡ „βÍÓÁ‡ ‚ ÍÎÂÚÍËÚÂ. ● ÉÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡ÌÂ: ÅÓflÚ Ì‡ ‡ÏÔÎËÙˈË‡ÌËÚ ÔÓ‚ÚÓË CTG ‚ DMPK-„Â̇ ÔË ·ÓÎÂÒÚÚ‡ ̇ Steinert Ë CCTG ‚ ZNF9-„Â̇ ÔË PROMM Ò ÓÔ‰ÂÎfl ˜ÂÁ Southern blot ‡Ì‡ÎËÁ Ë PCR. áÄäãûóÖçàÖ åËÓÚÓÌ˘ÌËÚ ‰ËÒÚÓÙËË ÚËÔ I (·ÓÎÂÒÚ Ì‡ Steinert) Ë ÚËÔ II (PROMM) Ò‡ „ÂÌÂÚ˘ÌË ÏÛÎÚËÒËÒÚÂÏÌË Á‡·ÓÎfl‚‡ÌËfl, ÍÓËÚÓ ÍÎËÌ˘ÌÓ Ò ı‡‡ÍÚÂËÁË‡Ú Ò ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ë ÏËÓÚÓÌËfl. èÓfl‚ËÚ ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÔË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ ̇ Steinert ÏÓ„‡Ú ‰‡ ‚‡Ë‡Ú ÓÚ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡Ì ÔË ÍÓÌ„ÂÌËÚ‡Î̇ڇ ÙÓχ ‰Ó ÒÔˆËÙ˘ÌË ÍÓ„ÌËÚË‚ÌË (ÓÒÌÓ‚ÌÓ Ì‡Û¯ÂÌËfl ‚


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

ÑÂÍÂÏ‚Ë 2009

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

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