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ISSN 1311-8641

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 íéå 7 / Åêéâ 5 ÑÖäÖåÇêà, 2007

VOLUME 7 / NUMBER 5 DECEMBER, 2007

ëöÑöêÜÄçàÖ

CONTENTS

éÅáéêà

REVIEWS

äéãàóÖëíÇÖç ÄçÄãàá çÄ ÖÖÉ à îÄêåÄäé-ÖÖÉ àáëãÖÑÇÄçàü èêà Åéãçà ë ÖèàãÖèëàü ä.ÅÂηÂÓ‚‡, Ä.ÄÎÂÍÒË‚ . . . . . . . . . . . . . . . . . . . . . . . . .209

QUANTITATIVE EEG ANALYSIS AND PHARMACOEEG STUDIES IN PATIENTS WITH EPILEPSY K.Belberova, A.Alexiev . . . . . . . . . . . . . . . . . . . . . . . . . .209

ÑÖÉÖçÖêÄíàÇçÄ ãìåÅÄãçÄ ëèéçÑàãéãàëíÖáÄ è. ñ‚ÂÚ‡ÌÓ‚, à. Ç˙ÎÍÓ‚, Å. ëÚ‡ÏÂÌÓ‚, Ç. êÓÒχÌÓ‚, ã. âÓ‚˜Â‚‡, ë. çfl„ÓÎÓ‚‡ , ç. íӈ‚ . . . . . . . . . . . . . . . . . .216

DEGENERATIVE LUMBAR SPONDYLOLISTHESIS P.Tzvetanov, I. Volkov, B. Stamenov, V. Rossmanov, L. Yovcheva, S. Niagulova, N. Tocev . . . . . . . . . . . . . . . .216

çÖîÄêåÄäéãéÉàóçÄ çÖÇêéèêéíÖäñàü èêà àëïÖåàóÖç åéáöóÖç àçëìãí Ñ. å‡Ò·Ó‚, Ç. ä‡Î‡È‰ÊË‚ . . . . . . . . . . . . . . . . . . . . .219

NON-PHARMACOLOGICAL NEURéPROTECTION IN ACUTE STROKE D. Maslarov, V. Kalaidjiev . . . . . . . . . . . . . . . . . . . . . . . .219

éêàÉàçÄãçà ëíÄíàà

ORIGINAL PAPERS

çÖÇêéçÄãçà ñÖêéàÑãàèéîìëñàçéáà Ç ÑÖíëäÄ ÇöáêÄëí: çÖ íéãäéÇÄ êÖÑäà, çé íêìÑçé êÄáèéáçÄÇÄçà Ç ÅöãÉÄêàü è. ÑËÏÓ‚‡,Ç. ÅÓÊËÌÓ‚‡, á. ãÛ͇˜ . . . . . . . . . . . . . . . . . .225

NEURONAL CEROID LIPOFUSCINOSES IN CHILDHOOD: NOT SO RARE, BUT DIFFICULT DIAGNOSIS IN BULGARI P. Dimova, V. Bojinova, Z. Lukacs . . . . . . . . . . . . . . . . . .225

ïêéçàóçÄ óÖêçéÑêéÅçÄ ÖçñÖîÄãéèÄíàü èêà èÄñàÖçíà ë óÖêçéÑêéÅçÄ ñàêéáÄ å. чÒ͇ÎÓ‚, ç. ñÓÎÓ‚‡, å. ꇯ‚‡, ë. ëÚÓÈÌÓ‚, Ä. ÄÎÂÍ҇̉Ó‚ . . . . . . . . . . . . . . . . . . . . . .231

CHRONIC HEPATIC ENCEPHALOPATHY IN PATIENTS WITH LIVER CIRRHOSIS – CLINICAL, NEUROPSYCHOLOGICAL AND EEG STUDY M. Daskalov, N. Tzolova, M. Rasheva, S. Stoinov, A. Alexandrov . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .231

ÖÑàçàóçà à åçéÉéäêÄíçà Ä-Çöãçà èêà èÄñàÖçíà ë ÑàÄÅÖíçÄ çÖÇêéèÄíàü Ä. ë‚. ÄÎÂÍ҇̉Ó‚, ç. åÛ‡‰flÌ, å. чÒ͇ÎÓ‚, Å. à¯ÔÂÍÓ‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .237

SINGLE AND MULTIPLE A-WAVES IN PATIENTS WITH DIABETIC NEUROPATHY IN THE LOW BACK PATHOLOGY AS Alexandrov, N Muradyan, M Daskalov, B Ishpekova . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .237

äéçÉÖçàíÄãÖç åàÄëíÖçÖç ëàçÑêéå íàè IÄ (îÄåàãçÄ àçîÄçíàãçÄ åàÄëíÖçàü) èêà ÅöãÉÄêëäàíÖ êéåà Ç. ÉÂ„ÂΘ‚‡, à. ãËÚ‚ËÌÂÌÍÓ, ë. óÂÌËÌÍÓ‚‡, Ç. ÅÓÊËÌÓ‚‡, Ç. åËı‡ÈÎÓ‚‡, Å. à¯ÔÂÍÓ‚‡, ã. ÄÌ„ÂÎÓ‚‡, à. à‚‡ÌÓ‚, è. ÑËÏÓ‚‡, Ä. éÒ͇, Ä. âÓ‰‡ÌÓ‚‡, Å. Öχ, Ä. ÖÌ„ÂÎ, ï. ãÓÍÏ˛ÎÂ, ã. ä‡Î‡È‰ÊË‚‡, à. í˙Ì‚ . . . . . . . . . . . . . . . . . . . . . . .242

CONGENITAL MYASTHENIC SYNDROME TYPE IA (FAMILIAL INFANTILE MYASTHENIA) IN BULGARIAN ROMA V. Guergueltcheva, I. Litvinenko, S. Cherninkova, V. Bojinova, V. Mihaylova, B. Ishpekova, L. Angelova, I. Ivanov, P. Dimova, A. Oscar, A. Jordanova, B. Eymard, A. Engel, H. Lochmuller, L. Kalaydjieva, I. Tournev . . . . . . . . . . . . . . . . . . . . . . . .242


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

Publisher Color Studio®

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

EDITORS

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. à‚‡ÌÓ‚‡ ã. à¯ÔÂÍÓ‚‡ Å. äÓ΂ é. å‡Ì˜Â‚ à. å‡Ò·Ó‚ Ñ. åË·ÌÓ‚ à. åË·ÌÓ‚‡ å. åË̘‚ Ñ. çËÍÓ‚ÒÍË ç. èÂÚÓ‚ à. èÂÚÓ‚‡ û. ê‡È˜Â‚ à. ꇯ‚‡ å. ëÚ‡ÏÂÌÓ‚ Å. íËÚflÌÓ‚‡ Ö. í‡ÈÍÓ‚ ã. í˙Ì‚ à. ÂÁÓ‚‡ ã. ÊË‚ Ñ ï‡‰ÊËÔÂÚÓ‚‡ Ö. ï‡‡Î‡ÌÓ‚ ã. ñ‡ÌÍÓ‚‡ ñ. ó‡Î˙ÍÓ‚‡ ç. óÂÌËÌÍÓ‚‡ ë. òÓÚÂÍÓ‚ è. ü̘‚‡ ë.

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. Ivanova L. Ishpekova B. Kolev O. Manchev I. Maslarov D. Milanov I. Milanova M. Minchev D. Nikoevsky N. Petrov I. Petrova U. Raychev I. Rasheva M. Stamenov B. Titianova E. Traykov L. Tarnev I. Havezova L. Hadjiev D. Hadjipetrova E. Haralanov L. Tzankova Tz. Chalakova N. Charninkova S. Shotekov P. Yancheva S.

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

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

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

EDITOR IN CHIEF: I. Milanov, P. Stamenova, I. Velcheva SECRETARY: M. Daskalov


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

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl

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INSTRUCTION FOR AUTHORS

Bulgarian Neurology

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


Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology ëöÑöêÜÄçàÖ éÅáéêà äéãàóÖëíÇÖç ÄçÄãàá çÄ ÖÖÉ à îÄêåÄäéÖÖÉ àáëãÖÑÇÄçàü èêà Åéãçà ë ÖèàãÖèëàü ä.ÅÂηÂÓ‚‡, Ä.ÄÎÂÍÒË‚ . . . . . . . . . . . . . . . . . . . .209 ÑÖÉÖçÖêÄíàÇçÄ ãìåÅÄãçÄ ëèéçÑàãéãàëíÖáÄ è. ñ‚ÂÚ‡ÌÓ‚, à. Ç˙ÎÍÓ‚, Å. ëÚ‡ÏÂÌÓ‚, Ç. êÓÒχÌÓ‚, ã. âÓ‚˜Â‚‡, ë. çfl„ÓÎÓ‚‡ , ç. íӈ‚ . . . . . . . . . . .216 çÖîÄêåÄäéãéÉàóçÄ çÖÇêéèêéíÖäñàü èêà àëïÖåàóÖç åéáöóÖç àçëìãí Ñ. å‡Ò·Ó‚, Ç. ä‡Î‡È‰ÊË‚ . . . . . . . . . . . . . . . .219 éêàÉàçÄãçà ëíÄíàà çÖÇêéçÄãçà ñÖêéàÑãàèéîìëñàçéáà Ç ÑÖíëäÄ ÇöáêÄëí: çÖ íéãäéÇÄ êÖÑäà, çé íêìÑçé êÄáèéáçÄÇÄçà Ç ÅöãÉÄêàü è. ÑËÏÓ‚‡,Ç. ÅÓÊËÌÓ‚‡, á. ãÛ͇˜ . . . . . . . . . . . . .225 ïêéçàóçÄ óÖêçéÑêéÅçÄ ÖçñÖîÄãéèÄíàü èêà èÄñàÖçíà ë óÖêçéÑêéÅçÄ ñàêéáÄ å. чÒ͇ÎÓ‚, ç. ñÓÎÓ‚‡, å. ꇯ‚‡, ë. ëÚÓÈÌÓ‚, Ä. ÄÎÂÍ҇̉Ó‚ . . . . . . . . . . . . . . . . .231 ÖÑàçàóçà à åçéÉéäêÄíçà Ä-Çöãçà èêà èÄñàÖçíà ë ÑàÄÅÖíçÄ çÖÇêéèÄíàü Ä. ë‚. ÄÎÂÍ҇̉Ó‚, ç. åÛ‡‰flÌ, å. чÒ͇ÎÓ‚, Å. à¯ÔÂÍÓ‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . . . . .237 äéçÉÖçàíÄãÖç åàÄëíÖçÖç ëàçÑêéå íàè IÄ (îÄåàãçÄ àçîÄçíàãçÄ åàÄëíÖçàü) èêà ÅöãÉÄêëäàíÖ êéåà Ç. ÉÂ„ÂΘ‚‡, à. ãËÚ‚ËÌÂÌÍÓ, ë. óÂÌËÌÍÓ‚‡, Ç. ÅÓÊËÌÓ‚‡, Ç. åËı‡ÈÎÓ‚‡, Å. à¯ÔÂÍÓ‚‡, ã. ÄÌ„ÂÎÓ‚‡, à. à‚‡ÌÓ‚, è. ÑËÏÓ‚‡, Ä. éÒ͇, Ä. âÓ‰‡ÌÓ‚‡, Å. Öχ, Ä. ÖÌ„ÂÎ, ï. ãÓÍÏ˛ÎÂ, ã. ä‡Î‡È‰ÊË‚‡, à. í˙Ì‚ . . . . . . . . . . . . . . . . . .242 óÖëíéíÄ çÄ ε4 ÄãÖã çÄ ÄèéãàèéèêéíÖàç Ö èêà ÅöãÉÄêëäà èÄñàÖçíà ë ÅéãÖëí çÄ ÄãñïÄâåÖê ã. í‡ÈÍÓ‚, ò. åÂı‡·Ë‡Ì, Ä. âÓ‰‡ÌÓ‚‡, å. ê‡È˜Â‚‡, å. èÂÚÓ‚‡, ü. ÜÂ΂, à. âÓ‰‡ÌÓ‚‡, à. äÂÏÂÌÒÍË . . . . . . . . . . . . . . . .245 àçíÖêåÖÑàÖêçÄ îéêåÄ çÄ ÅéãÖëí çÄ NIEMANN-PICK èêà ÅöãÉÄêëäà êéåà Ç. åËı‡ÈÎÓ‚‡, à. í˙Ì‚, ü. ï‡ÌÚÍÂ, à. ëËÌË„ÂÒ͇, ë.óÂÌËÌÍÓ‚‡, å. ꇉËÓÌÓ‚‡, å. ê‡È˜Â‚‡ ,ë. ä˙ÒÚ‚, à. í‡ÈÍÓ‚‡, Ä. òχÓ‚, Ç. ɇ‚ËÍÓ‚‡, Å. åË΂, ê. íË̘‚‡, Ñ. äÛ˛Ï‰ÊË‚, ã.ä‡Î‡È‰ÊË‚‡ . . . . . . . . . . . . . .249 êÄÅÑéåàéãàáÄ ë èêàçéë çÄ ÑÇÄ ëãìóÄü – êÄáãàóçÄ ÖíàéãéÉàü, èÄíéÉÖçÖáÄ à ãÖóÖçàÖ, ëÇöêáÄçà ë êÄáãàóÖç àáïéÑ å. äÎËÒÛÒÍË, Ö. LJÒË΂‡, Å. ÑÂÎËÈÒ͇, ü. ïËÒÚÓ‚, Ñ. Äڇ̇ÒÓ‚‡, ñ. ŇΉ‚, ñ. ÇÂÎÍÓ‚ÒÍË, å. åË·ÌÓ‚‡ . . . . . . . . . . . . . . . . .255 äêöÇéëçÄÅÑüÇÄçÖ çÄ åéáöóçà íêÄçëèãÄçíÄíà â. è‡ÌÓ‚ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .261 éèàëÄçàÖ çÄ äãàçàóÖç ëãìóÄâ çÖÇêéÑÖÉÖçÖêÄñàü ë éíãÄÉÄçÖ çÄ ÜÖãüáé Ç åéáöäÄ (ëàçÑêéå çÄ HALLERVORDEN-SPATZ) ã. í‡ÈÍÓ‚, ò. åÂı‡·Ë‡Ì, å. ê‡È˜Â‚‡, å. èÂÚÓ‚‡, ü. ÜÂ΂, é. ä˙΂ . . . . . . . . . . . . . . . . . . . . . . . .265

CONTENTS REVIEWS QUANTITATIVE EEG ANALYSIS AND PHARMACOEEG STUDIES IN PATIENTS WITH EPILEPSY K.Belberova, A.Alexiev . . . . . . . . . . . . . . . . . . . . .209 DEGENERATIVE LUMBAR SPONDYLOLISTHESIS P.Tzvetanov, I. Volkov, B. Stamenov, V. Rossmanov, L. Yovcheva, S. Niagulova, N. Tocev . . . . . . . . . . . .216 NON-PHARMACOLOGICAL NEURéPROTECTION IN ACUTE STROKE D. Maslarov, V. Kalaidjiev . . . . . . . . . . . . . . . . . . .219 ORIGINAL PAPERS NEURONAL CEROID LIPOFUSCINOSES IN CHILDHOOD: NOT SO RARE, BUT DIFFICULT DIAGNOSIS IN BULGARI P. Dimova, V. Bojinova, Z. Lukacs . . . . . . . . . . . . .225 CHRONIC HEPATIC ENCEPHALOPATHY IN PATIENTS WITH LIVER CIRRHOSIS – CLINICAL, NEUROPSYCHOLOGICAL AND EEG STUDY M. Daskalov, N. Tzolova, M. Rasheva, S. Stoinov, A. Alexandrov . . . . . . . . . . . . . . . . . . . . . . . . . . . . .231 SINGLE AND MULTIPLE A-WAVES IN PATIENTS WITH DIABETIC NEUROPATHY IN THE LOW BACK PATHOLOGY AS Alexandrov, N Muradyan, M Daskalov, B Ishpekova . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .237 CONGENITAL MYASTHENIC SYNDROME TYPE IA (FAMILIAL INFANTILE MYASTHENIA) IN BULGARIAN ROMA V. Guergueltcheva, I. Litvinenko, S. Cherninkova, V. Bojinova, V. Mihaylova, B. Ishpekova, L. Angelova, I. Ivanov, P. Dimova, A. Oscar, A. Jordanova, B. Eymard, A. Engel, H. Lochmuller, L. Kalaydjieva, I. Tournev . . . . . . . . . . . . . . . . . . .242 APOLIPOPROTEIN E ε4 ALLELE FREQUENCY IN BULGARIAN PATIENTS WITH ALZHEIMER’S DISEASE. L. Traykov, S. Mehrabian, A. Jordanova, M. Raycheva, M. Petrova, Y. Jelev, I. Jordanova,I.Kremensky . . . . . . . . . . . . . . . . . . . . . .245 INTERMEDIATE TYPE OF NIEMANN-PICK DISEASE IN BULGARIAN ROMA V. Mihaylova, I .Tournev, J .Hantke, I. Sinigerska, S. Cherninkova, M .Radionova M. Raicheva, S. Krastev, I. Traikova, A. Shmarov, V .Gavrikova, B.Milev ,R Tincheva, D .Khuyomdziev, L .Kalaydjieva . . . . . . . . . . . . . .249 TWO CASES OF RHABDOMYOLYSIS WITH DIFFERENT ETIOLOGY, PATHOGENESIS, TREATMENT AND FINAL OUTCOME M. Klissurski, Ö. Vassileva, B. Deliiska, Y. Hristov, D. Atanasova, Tz. Baldev, Tz. Velkovski, M.Milanova . . . . . . . . . . . . . . . . . . . .255 NEOVASCULARIZATION OF THE BRAIN’S GRAFTS Y. Panov . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .261 CASE REPORT NEURODEGENERATION WITH BRAIN IRON ACCUMULATION (HALLERVORDEN-SPATZ SYNDROME) L. Traykov, S. Mehrabian, M. Raycheva, M. Petrova, Y. Zhelev, O. Kalev . . . . . . . . . . . . . . . . . . . . . . . . .265


‰ÂÍÂÏ‚Ë, 2007

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

209

é·ÁÓ äéãàóÖëíÇÖç ÄçÄãàá çÄ ÖÖÉ à îÄêåÄäé-ÖÖÉ àáëãÖÑÇÄçàü èêà Åéãçà ë ÖèàãÖèëàü ä. ÅÂηÂÓ‚‡, Ä. ÄÎÂÍÒË‚ ìåÅÄã ◊ÄÎÂÍ҇̉Ó‚Ò͇” ,äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ –ëÓÙËfl

SUMMARY QUANTITATIVE EEG ANALYSIS AND PHARMACOEEG STUDIES IN PATIENTS WITH EPILEPSY K.Belberova, A.Alexiev Digital EEG represents significant, technologically new advance in the development of electroencephalography and provides not only more accurate and convenient way for registration, storage, analysis and visual EEG interpretation, but moreover further computer-assisted signal processing enables obtaining of additional otherwise inaccessible information. Published literature indicates that quantitative EEG analysis (QEEG) is still underutilized in clinical setting. Nevertheless existing data support its promising potential application in the field of epileptology as objective and sensitive tool for identification and evaluation of background abnormalities, monitoring CNS effects of antiepileptic drugs, early detection and prediction of neurotoxicity as well as an additional tool in the assessment of cerebral and cognitive dysfunctions associated with other neurological disorders. KEY WORDS: EEG, quantitative analysis, background abnormalities, epilepsy, antiepileptic drugs. êÖáûåÖ ÑË„ËÚ‡Î̇ڇ ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡Ï‡ /ÖÖÉ/  Á̇˜ËÚÂÎÂÌ, ÚÂıÌÓÎӄ˘ÌÓ ÌÓ‚ ÂÚ‡Ô ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ÏÂÚÓ‰Ë͇ڇ, ÍÓÈÚÓ ÔÓÁ‚ÓÎfl‚‡ Ì ҇ÏÓ ÛÒ˙‚˙¯ÂÌÒÚ‚‡ÌÂ Ë ÛÎÂÒÌfl‚‡Ì ̇ „ËÒÚ‡ˆËflÚ‡, Ò˙ı‡Ìfl‚‡ÌÂÚÓ, ‡Ì‡ÎËÁ‡ Ë ‚ËÁÛ‡Î̇ڇ ËÌÚÂÔÂÚ‡ˆËfl, ÌÓ Ë ÔÓ-̇ڇÚ˙¯Ì‡Ú‡ ÍÓÏÔ˛Ú˙̇ Ó·‡·ÓÚ͇ ̇ Ò˄̇· Ë ÔÓÁ‚ÓÎfl‚‡ ÔÓÎÛ˜‡‚‡ÌÂÚÓ Ì‡ ‰ÓÔ˙ÎÌËÚÂÎ̇, ̉ÓÒÚËÊËχ ÔÓ ‰Û„ ̇˜ËÌ ËÌÙÓχˆËfl. чÌÌËÚ ÓÚ ÎËÚÂ‡ÚÛ‡Ú‡ ÔÓ͇Á‚‡Ú, ˜Â ÍÓ΢ÂÒÚ‚ÂÌËflÚ ÖÖÉ ‡Ì‡ÎËÁ ‚Ò Ӣ Ì Ò  ÛÚ‚˙‰ËΠ̇ ÔÓ·„‡˘ÓÚÓ ÏÛ Ò ÏflÒÚÓ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇, ÌÓ ‚˙ÔÂÍË ÚÓ‚‡ ۷‰ËÚÂÎÌÓ Ì‡ÒÓ˜‚‡Ú Í˙Ï ÔÓÚÂ̈ˇÎÌÓÚÓ ÏÛ ÛÒÔ¯ÌÓ ÔËÎÓÊÂÌË ‚ ӷ·ÒÚÚ‡ ̇ ÂÔËÎÂÔÚÓÎÓ„ËflÚ‡ Á‡ ÛÒÚ‡ÌÓ‚fl‚‡ÌÂ Ë ÔÓÒΉfl‚‡Ì ̇ ÔÓÏÂÌËÚ ‚ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÔË ·ÓÎÌËÚÂ Ò ÂÔËÎÂÔÒËfl, Á‡ ÏÓÌËÚÓË‡Ì ̇ ˆÂÌÚ‡ÎÌËÚ ÂÙÂÍÚË ÓÚ ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓÚÓ Î˜ÂÌËÂ, ‡Ì̇ ‰ÂÚÂ͈Ëfl Ë ÔÓ„ÌÓÁË‡Ì ̇ Ì‚ÓÚÓÍÒ˘ÌË ÔÓfl‚Ë, ‡ ڇ͇ Ò˙˘Ó Ë Í‡ÚÓ ‰ÓÔ˙ÎÌËÚÂÎÂÌ ÏÂÚÓ‰ ‚ ÓˆÂÌ͇ڇ ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË ÔË ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl ̇ ñçë. äãûóéÇà Ñìåà: ÖÖÉ, ÍÓ΢ÂÒÚ‚ÂÌ ‡Ì‡ÎËÁ, ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ, ÂÔËÎÂÔÒËfl, ‡ÌÚËÂÔËÎÂÔÚ˘ÌË ÎÂ͇ÒÚ‚‡. èÂ‰Ë Ôӂ˜ ÓÚ 80„. Hans Berger ‰ÂÏÓÌÒÚË‡, ˜Â ˜Ó‚¯ÍËflÚ ÏÓÁ˙Í, „ÂÌÂË‡ ÂÎÂÍÚ˘ÂÒÍË Ò˄̇ÎË, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú Á‡ÔËÒ‡ÌË ÓÚ Ò͇ÎÔ‡, Ë Ì‡˘‡ „ËÒÚ‡ˆËflÚ‡ Â̈ÂÙ‡ÎÓ„‡Ï‡. í‡ÁË Ô˙‚‡ ÚÂıÌË͇, ÒÔÓÒӷ̇ ‰‡ ÓÚ·ÂÎflÁ‚‡ ÔÓÏÂÌËÚ ‚˙‚ ÙÛÌ͈ËflÚ‡ ̇ ˜Ó‚¯ÍËfl ÏÓÁ˙Í, ·Ë‚‡ ÛÒ˙‚˙¯ÂÌÒÚ‚‡Ì‡ Ë ÒΉ ÇÚÓ‡Ú‡ Ò‚ÂÚӂ̇ ‚ÓÈ̇ Ò ÛÚ‚˙ʉ‡‚‡ ͇ÚÓ Â‰ËÌ ÓÚ ÓÒÌÓ‚ÌËÚ ËÌÒÚÛÏÂÌÚ‡ÎÌË ÏÂÚÓ‰Ë Ì‡ ËÁÒΉ‚‡Ì ‚ Ì‚ÓÎӄ˘̇ڇ ‰Ë‡„ÌÓÒÚË͇. èÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË ÒÏ ҂ˉÂÚÂÎË Ì‡ Á̇˜ËÚÂÎÂÌ Ì‡Ô‰˙Í ‚ ‰Ë‡„ÌÓÒÚË͇ڇ ‚ ÂÁÛÎÚ‡Ú Ì‡ ÒÍÓÍÓÓ·‡ÁÌÓ

‡Á‚ËÚË ‚ Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ÏÂÚÓ‰ËÍË. åRI, SPECT, PET, fMRI Ô‰·„‡Ú ËÁÍβ˜ËÚÂÎÌÓ ‚ËÒÓ͇ ÒÔÓÒÓ·ÌÓÒÚ Á‡ ÎÓ͇ÎËÁ‡ˆËfl. ç‡ Ô˙‚ ÔӄΉ ËÁ„ÎÂʉ‡, ˜Â ‡ÍˆÂÌÚ˙Ú ‚ Ì‚ÓÎӄ˘̇ڇ ‰Ë‡„ÌÓÒÚË͇ Ò  ËÁÏÂÒÚËÎ Í˙Ï Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ÏÂÚÓ‰Ë, ÌÓ Ô‡‡ÎÂÎÌÓ Ò ‚˙ÁÏÓÊÌÓÒÚËÚ ̇ ÚÂÁË ÚÂıÌËÍË Á‡ ‡ÎËÒÚ˘ÌÓ Ô‰ÒÚ‡‚flÌ ̇ ÒÚÛÍÚÛ‡Ú‡ Ë ÙÛÌ͈ËflÚ‡ ̇ ˜Ó‚¯ÍËfl ÏÓÁ˙Í, Ò ‡Á¯Ëfl‚‡Ú Á̇˜ËÚÂÎÌÓ Ë ‚˙ÁÏÓÊÌÓÒÚËÚ ̇ ÍÎËÌ˘̇ڇ Ì‚ÓÙËÁËÓÎÓ„Ëfl. 燂ÎËÁ‡ÌÂÚÓ ‚ Ô‡ÍÚË͇ڇ ̇ ‰Ë„ËÚ‡Î̇ڇ ÖÖÉ ÓÒË„Ûfl‚‡ ‚˙ÁÏÓÊÌÓÒÚ ‰‡ Ò ӷ‡·ÓÚ‚‡Ú ÖÖÉ Á‡ÔËÒËÚ ÔÓ ‡Á΢ÌË Ì‡˜ËÌË, ÍÓÂÚÓ ÔÓÁ‚ÓÎfl‚‡ ÂÍÒÚ‡ÍˆËflÚ‡ ̇ ̉ÓÒÚËÊËχ ÔÓ ‰Û„ ̇˜ËÌ ËÌÙÓχˆËfl. çÂ˘Ó Ôӂ˜Â, Ò ÔÓÏÓ˘Ú‡ ̇ Ò˙‚ÂÏÂÌÌËÚ ÍÓÏÔ˛Ú˙ÌË ÚÂıÌÓÎÓ„ËË Â ‚˙ÁÏÓÊ̇ ‰ÌÓ‚ÂÏÂÌ̇ڇ „ËÒÚ‡ˆËfl ̇ ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡Ï‡ Ò åRI, SPECT Ë PET, ÍÓÂÚÓ ÔÓÁ‚ÓÎfl‚‡ ÏÛÎÚËÏÓ‰‡ÎÌÓ ËÁÓ·‡Áfl‚‡ÌÂ, ÒÔÓÒÓ·ÌÓ ‰‡ ‡ÁÍË Ôӂ˜ ÔÓ‰Ó·ÌÓÒÚË ÓÚ ‚Òfl͇ ‰̇ ÏÓ‰‡ÎÌÓÒÚ Ò‡ÏÓÒÚÓflÚÂÎÌÓ. ÖÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙËflÚ‡ Ô‰ÒÚ‡‚Îfl‚‡ ÙÛÌ͈ËÓ̇ÎÂÌ ÏÂÚÓ‰ Á‡ „ËÒÚ‡ˆËfl ̇ ÏÓÁ˙˜Ì‡Ú‡ ÂÎÂÍÚ˘̇ ‡ÍÚË‚ÌÓÒÚ, ÓÒ˙˘ÂÒÚ‚Â̇ ˜ÂÁ ÌÂÔÂÍ˙ÒÌ‡Ú Á‡ÔËÒ Ì‡ ÔÓÚÂ̈ˇÎÌËÚ ‡ÁÎËÍË Ì‡ ÂÎÂÍÚ˘ÂÒÍËÚ ÔÓÎÂÚ‡, „ËÒÚË‡ÌË ÔÓÒ‰ÒÚ‚ÓÏ ÂÎÂÍÚÓ‰Ë, ̇È-˜ÂÒÚÓ ÓÚ ÔÓ‚˙ıÌÓÒÚÚ‡ ̇ „·‚‡Ú‡. ÑË„ËÚ‡Î̇ڇ ÖÖÉ Ô‰ÒÚ‡‚Îfl‚‡ Á̇˜ËÚÂÎÂÌ, ÚÂıÌÓÎӄ˘ÌÓ ÌÓ‚ ÂÚ‡Ô ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ÖÖÉ Ò ÏÌÓ„Ó·ÓÈÌËÚ Ô‰ËÏÒÚ‚‡, ‚ Ò‡‚ÌÂÌËÂ Ò ‡Ì‡ÎÓ„Ó‚‡Ú‡ ÖÖÉ, ÔË „ËÒÚË‡ÌÂÚÓ, Ò˙ı‡Ìfl‚‡ÌÂÚÓ, ‡Ì‡ÎËÁ‡ Ë Ô‰ÒÚ‡‚flÌÂÚÓ Ì‡ ÂÁÛÎÚ‡ÚËÚÂ, ‚Íβ˜ËÚÂÎÌÓ Ë ÓÚ ÓÚ‰‡Î˜Â̇ ÚӘ͇ (ÔÂÁ ËÌÚÂÌÂÚ). ÑË„ËÚ‡Î̇ڇ ÖÖÉ Â ËÍÓÌÓÏ˘̇ Ë Ò˙ı‡Ìfl‚‡˘‡ ‚ÂÏÂÚÓ ‚ ‰ÓÔ˙ÎÌÂÌË ̇ Ô‰·„‡ÌËÚ ‚˙ÁÏÓÊÌÓÒÚË Á‡ ÍÎËÌ˘̇ ‰Ë‡„ÌÓÁ‡ Ë Ì‡Û˜ÌÓËÁÒΉӂ‡ÚÂÎÒ͇ ‰ÂÈÌÓÒÚ. ë ÛÒ˙‚˙¯ÂÌÒÚ‚‡ÌÂÚÓ Ë ‚Ò ÔÓ-¯ËÓÍÓÚÓ Ì‡‚ÎËÁ‡Ì ̇ ÍÓÏÔ˛Ú˙̇ڇ ÚÂıÌÓÎÓ„Ëfl, ÏÓÊ ‰‡ Ò Ә‡Í‚‡ ÔÓÎÛ˜‡‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë ‰‡ÌÌË, ÍÓËÚÓ ˘Â ‡Á¯ËflÚ ‡Á·Ë‡ÌËflÚ‡ Á‡ Á‰‡‚Ëfl Ë Á‡·ÓÎÂÎËfl ˜Ó‚¯ÍË ÏÓÁ˙Í Ë ˘Â ‡Á¯ËflÚ ‰Ë‡„ÌÓÒÚ˘ÌÓÚÓ ÔËÎÓÊÂÌË ̇ ÖÖÉ (1,2,14,19,68). éÒÌÓ‚ÌËflÚ ÏÂÚÓ‰ Á‡ ‡Á˜ËÚ‡ÌÂ Ë ËÌÚÂÔÂÚ‡ˆËfl ̇ ÖÖÉ Â ‚ËÁÛ‡ÎÌËflÚ ‡Ì‡ÎËÁ. èË ÌÂ„Ó Ó·‡˜Â Ò ·Ó‡‚Ë Ò ÓÔËÒ‡ÚÂÎ̇ Ë ÒÚÂÔÂÌÛ‚‡˘‡ ÚÂÏËÌÓÎÓ„Ëfl. éˆÂÌ͇ڇ  ÒÛ·ÂÍÚ˂̇ Ë Á‡‚ËÒË ‚ „ÓÎflχ ÒÚÂÔÂÌ ÓÚ Ó·Û˜ÂÌËÂÚÓ Ë ÓÔËÚ‡ ̇ Â̈ÂÙ‡ÎÓ„‡ÙËÒÚ‡ Ë ÚӘ̇ ÍÓ΢ÂÒÚ‚Â̇ ÓˆÂÌ͇ ̇ ‚˙ÎÌËÚÂ, ̇ ÚflıÌÓÚÓ Ò˙ÓÚÌÓ¯ÂÌË ËÎË ÔÓÒÚ‡ÌÒÚ‚ÂÌÓ ‡ÁÔ‰ÂÎÂÌË  Ì‚˙ÁÏÓÊ̇. íÓ‚‡  ӷÓÒÌÓ‚‡ÎÓ ‡Á‡·ÓÚ‚‡ÌÂÚÓ Ì‡ ‡Á΢ÌË ÏÂÚÓ‰Ë Á‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ó·ÂÍÚË‚ËÁË‡Ì ̇ ‡Ì‡ÎËÁ‡ ̇ Â̈ÂÙ‡ÎÓ„‡Ï‡Ú‡. LJÊ̇ Ô‰ÔÓÒÚ‡‚͇ Á‡ ÚÓ‚‡  ‚˙ÁÏÓÊÌÓÒÚÚ‡, ÍÓflÚÓ Ô‰ÓÒÚ‡‚flÚ ÍÓÏÔ˛Ú˙ÌËÚ ÚÂıÌÓÎÓ„ËË Á‡ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÎÓÊÌË Ï‡ÚÂχÚ˘ÂÒÍË ‡Î„ÓËÚÏË, ÍÓËÚÓ Á̇˜ËÚÂÎÌÓ ‡Á¯Ëfl‚‡Ú Í˙„ÓÁÓ‡ Ë ÔËÎÓÊÂÌËÂÚÓ Ì‡ ÏÂÚÓ‰Ë͇ڇ. çflÍÓË ÓÚ Úflı ‚˜ ҇ Á‡ÂÎË Ú‚˙‰Ó ÏflÒÚÓ ‚ ‰Ë‡„ÌÓÒÚË͇ڇ, ‰Û„Ë ¯ËÓÍÓ Ò ËÁÔÓÎÁ‚‡Ú ‚ ̇ۘÌËÚ ËÁÒΉ‚‡ÌËfl, ÌÓ ‚ÒÂ Ó˘Â Ó˜‡Í‚‡Ú Ò‚ÓÂÚÓ ÏflÒÚÓ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇. çÂÔÂÍ˙Ò̇ÚÓ Ò ‡Á‡·ÓÚ‚‡Ú ‚Ò ÔÓ-ÒÎÓÊÌË ÏÂÚÓ‰Ë, ËÁÔÓÎÁ‚‡˘Ë ÔÓÚÂ̈ˇ· ̇ ÍÓÏÔ˛Ú˙ÌËÚ ÚÂıÌÓÎÓ„ËË, ÍÓËÚÓ Ó˜‡Í‚‡Ú Ò‚ÓÂÚÓ ·˙‰Â˘Ó ÔÓÚ‚˙ʉÂÌË (1,14,19,68).


‰ÂÍÂÏ‚Ë, 2007

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

äéãàóÖëíÇÖç ÄçÄãàá çÄ ÖÖÉ (äéãàóÖëíÇÖçÄ ÖÖÉ) - èêàçñàèà ÑË„ËÚ‡Î̇ڇ ÖÖÉ ÔÓÁ‚ÓÎfl‚‡ Ì ҇ÏÓ ÛÒ˙‚˙¯ÂÌÒÚ‚‡ÌÂ Ë ÛÎÂÒÌfl‚‡Ì ̇ ‚ËÁÛ‡Î̇ڇ ËÌÚÂÔÂÚ‡ˆËfl ̇ ÖÖÉ, ÌÓ Ë ËÁ‡ÁÂ̇ڇ ‚ ˆËÙÓ‚ ‚ˉ ËÌÙÓχˆËfl Á‡ Ò˄̇· ÏÓÊ ‰‡ ·˙‰Â Ó·ÂÍÚ Ì‡ ÔÓ-̇ڇÚ˙¯Ì‡ Ó·‡·ÓÚ͇, ÍÓÂÚÓ ÔÓÁ‚ÓÎfl‚‡ ÔÓÎÛ˜‡‚‡ÌÂÚÓ Ì‡ ‰ÓÔ˙ÎÌËÚÂÎ̇, ̉ÓÒÚËÊËχ ÔÓ ‰Û„ ̇˜ËÌ ËÌÙÓχˆËfl. äÓ΢ÂÒÚ‚ÂÌËflÚ ÖÖÉ ‡Ì‡ÎËÁ ÔÓ‰˙Îʇ‚‡ ‰‡ ·˙‰Â ÔÓÛ˜‚‡Ì ͇ÚÓ ÏÂÚÓ‰ Á‡ ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÍÎËÌ˘ÌÓ Á̇˜ËÏË ÔÓÏÂÌË, ÍÓËÚÓ Ì ҇ ‡ÁÍËÚË ˜ÂÁ ‚ËÁÛ‡ÎÂÌ ‡Ì‡ÎËÁ. èÓ̇ÒÚÓfl˘ÂÏ ÍÓ΢ÂÒÚ‚Â̇ڇ ÖÖÉ Ò ÔËÂχ ͇ÚÓ ‰ÓÔ˙ÎÌÂÌË ̇ ÛÚËÌ̇ڇ ÖÖÉ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇. äÓ΢ÂÒÚ‚ÂÌËflÚ ÖÖÉ ‡Ì‡ÎËÁ Ì ÏÓÊ ‰‡ Ò ËÁÔÓÎÁ‚‡ Ò‡ÏÓÒÚÓflÚÂÎÌÓ. ãÂ͇ËÚÂ, ÔË·„‡˘Ë ÚÓÁË ÏÂÚÓ‰ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇, Úfl·‚‡ Á‡‰˙ÎÊËÚÂÎÌÓ ‰‡ ·˙‰‡Ú ÓÔËÚÌË Â̈ÂÙ‡ÎÓ„‡ÙËÒÚË, ÍÓËÚÓ ËÏ‡Ú Á‡‰˙ηӘÂÌÓ ÔÓÁ̇ÌËÂ Ë ‡Á·Ë‡Ì ̇ ÔË·„‡ÌËÚ ÏÂÚÓ‰Ë Ì‡ ‡Ì‡ÎËÁ (1,43,46,68). äÓ΢ÂÒÚ‚Â̇ڇ ÖÖÉ Â Ï‡ÚÂχÚ˘ÂÒ͇ Ó·‡·ÓÚ͇ ̇ ‰Ë„ËÚ‡ÎÌÓ Á‡ÔË҇̇ڇ ÖÖÉ Ò ˆÂÎ ‰‡ Ò ÔÓ‰˜ÂÚ‡flÚ ÒÔˆËÙ˘ÌË ÍÓÏÔÓÌÂÌÚË Ì‡ ‚˙ÎÌËÚÂ, ‰‡ Ò Ú‡ÌÒÙÓÏË‡ ÖÖÉ ‚ ‰Û„ ÙÓÏ‡Ú ËÎË ‰ÓÏÂÈÌ, ͇ÚÓ ‰Ó·‡‚fl Ô‡ÍÚ˘ÂÒ͇ ËÌÙÓχˆËfl ËÎË ‡ÒÓˆËË‡ ˆËÙÓ‚ËÚ ÂÁÛÎÚ‡ÚË Ò ÖÖÉ-‰‡ÌÌËÚ Á‡ ÔÓÒΉ‚‡˘ ‡Ì‡ÎËÁ ËÎË Ò‡‚ÌÂÌË (19,43,68). äÓ΢ÂÒÚ‚ÂÌËflÚ ‡Ì‡ÎËÁ ̇ ÖÖÉ (ÍÓ΢ÂÒÚ‚Â̇ ÖÖÉ) ‚Íβ˜‚‡ ÒΉÌËÚ ÍÓÏÔÓÌÂÌÚË: 1. ë˄̇ÎÂÌ ‡Ì‡ÎËÁ - ‚Í₇ ˜ÂÒÚÓÚÂÌ ‡Ì‡ÎËÁ; ‡‚ÚÓχÚ˘ÂÌ ‡Ì‡ÎËÁ ̇ Ò˙·ËÚËfl; ÏÓÌËÚÓË‡ÌÂ Ë ÚẨÂ̈Ëfl; ‡Ì‡ÎËÁ ̇ ËÁÚÓ˜ÌË͇. 2. íÓÔÓ„‡ÙÒÍË ‡Ì‡ÎËÁ (Brain Mapping) 3. ëÚ‡ÚËÒÚ˘ÂÒÍË ‡Ì‡ÎËÁ – ‚Íβ˜‚‡ Ò‡‚ÌÂÌËÂ Ò ÌÓχÚË‚ÌË ÒÚÓÈÌÓÒÚË; ‰Ë‡„ÌÓÒÚ˘ÂÌ ‰ËÒÍËÏË̇ˆËÓÌÂÌ ‡Ì‡ÎËÁ (43). óÖëíéíÖç (ëèÖäíêÄãÖç) ÄçÄãàá çÄ ÖÖÉ ÖÖÉ ÓÚ‡Áfl‚‡ ÌÂÔÂÍ˙Ò̇ÚËÚ ÍÓη‡ÌËfl ̇ ÏÓÁ˙˜Ì‡Ú‡ ÂÎÂÍÚ˘̇ ‡ÍÚË‚ÌÓÒÚ, ÍÓËÚÓ Ò ËÁÓ·‡Áfl‚‡Ú ̇ ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡Ï‡Ú‡ ͇ÚÓ ÌÂÔÂÍ˙ÒÌ‡Ú ‚˙ÎÌÓÓ·‡ÁÂÌ Ò˄̇Î, Ô‰ÒÚ‡‚fl˘ ÔÓÏÂÌËÚ ‚ „ÓÎÂÏË̇ڇ ̇ Ò˄̇· ‚˙‚ ‚ÂÏÂÚÓ /‚˙‚ ‚ÂÏ‚Ëfl ‰ÓÏÂÈÌ/. éÚ Ï‡ÚÂχÚ˘ÂÒ͇ „Ή̇ ÚӘ͇, ‚ÒÂÍË Ò˄̇ΠÏÓÊ ‰‡ ·˙‰Â Ô‰ÒÚ‡‚ÂÌ Í‡ÚÓ ÙÛÌ͈Ëfl. óÂÒÚÓÚÌËflÚ ‡Ì‡ÎËÁ ÓÔËÒ‚‡ ÙÛÌ͈ËflÚ‡ ‚ ˜ÂÒÚÓÚÌËfl ‰ÓÏÂÈÌ, Ú. ÔÓ ÓÚÌÓ¯ÂÌË ̇ ˜ÂÒÚÓÚ‡Ú‡. è‰ÒÚ‡‚flÌÂÚÓ ‚˙‚ ‚ÂÏ‚Ëfl ‰ÓÏÂÈÌ ÔÓ͇Á‚‡ Í‡Í Ò˄̇Î˙Ú Ò ÔÓÏÂÌfl Ò ‚ÂÏÂÚÓ, ‡ ‚ ˜ÂÒÚÓÚÌËflÚ ‰ÓÏÂÈÌ Í‡Í‚‡ ˜‡ÒÚ ÓÚ Ò˄̇· ÎÂÊË ‚ ‰‡‰ÂÌ ˜ÂÒÚÓÚÂÌ ‰Ë‡Ô‡ÁÓÌ (1,14,19,68). éÒÌÓ‚ÌËflÚ Ë Ì‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡Ì ÏÂÚÓ‰ Á‡ ˜ÂÒÚÓÚÂÌ ‡Ì‡ÎËÁ  Ú‡ÌÒÙÓχˆËflÚ‡ ̇ Fourier. ífl Ò ÓÒÌÓ‚‡‚‡ ̇ ÍÓ̈ÂÔˆËflÚ‡, ˜Â ‚Òfl͇ ÔÓËÁ‚ÓÎ̇ ‚˙Î̇ ÏÓÊ ‰‡ ·˙‰Â Ô‰ÒÚ‡‚Â̇ ͇ÚÓ ÒÛχ ÓÚ ı‡ÏÓÌ˘ÌË, ÒËÌÛÒÓˉÌË/ÍÓÒËÌÛÒÓˉÌË ‚˙ÎÌË, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÓÔËÒ‡ÌË ÔÓÒ‰ÒÚ‚ÓÏ ÍÓÂÙˈËÂÌÚË, ‰‡‚‡˘Ë ËÌÙÓχˆËfl Á‡ Úflı̇ڇ ‡ÏÔÎËÚÛ‰‡, Ù‡Á‡ Ë ˜ÂÒÚÓÚ‡. LJÊÌÓ Â ‰‡ Ò ÔÓ‰˜ÂÚ‡Â, ˜Â Ú‡ÌÒÙÓχˆËflÚ‡ ̇ Fourier  ӷ‡ÚËχ, ÚÓÂÒÚ ÓÚ ÔÓÎÛ˜ÂÌËfl ÒÔÂÍÚ˙ ÏÓÊ ‰‡ Ò ÂÍÓÌÒÚÛË‡ ÓË„Ë̇ÎÌËflÚ Ò˄̇Î, Ú˙È Í‡ÚÓ Úfl Ò˙‰˙ʇ ËÌÙÓχˆËfl Ì ҇ÏÓ Á‡ „ÓÎÂÏË̇ڇ ̇ ‚ÒÂÍË ˜ÂÒÚÓÚÂÌ ÍÓÏÔÓÌÂÌÚ, ÌÓ Ë Á‡ Ì„ӂ‡Ú‡ Ù‡Á‡. èÓÎÛ˜Â̇ڇ ËÌÙÓχˆËfl ÏÓÊ ‰‡ ·˙‰Â Ô‰ÒÚ‡‚Â̇ ͇ÚÓ ‚ÂÍÚÓ, ÍÓÏÔÎÂÍÒÌÓ ˜ËÒÎÓ ËÎË Ò „ÓÎÂÏË̇ Ë Ù‡Á‡. Fast Furier Transform (FFT)  χÚÂχÚ˘ÂÒÍË ‡Î„ÓËÚ˙Ï Á‡ ·˙ÁÓ ËÁ˜ËÒÎfl‚‡Ì ̇ Ú‡ÌÒÙÓχˆËflÚ‡ ̇ Fourier Ë Â ÎÂÒÌÓ ÔËÎÓÊËÏ ‚ ÛÒÎÓ‚ËflÚ‡ ̇ Ò˙‚ÂÏÂÌÌËÚ ÍÓÏÔ˛Ú˙ÌË ÚÂıÌÓÎÓ„ËË, ÍÓÂÚÓ „Ó Ô‡‚Ë ÓÒÓ·ÂÌÓ ÔÓ‰ıÓ‰fl˘ Á‡ ¯ËÓÍ Í˙„ ÔËÎÓÊÂÌËfl. è˙‚ËflÚ Ô‰ÎÓÊÂÌ Ë

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̇È-χÒÓ‚Ó ËÁÔÓÎÁ‚‡Ì FFT ‡Î„ÓËÚ˙Ï Â ‚˙‚‰ÂÌ ÓÚ Cooley Ë Tukey ÔÂÁ 1965„. Ä̇ÎËÁ˙Ú Ì‡ Fourier ̇ÏË‡ ËÁÍβ˜ËÚÂÎÌÓ ¯ËÓÍÓ Ì‡Û˜ÌÓ Ë Ô‡ÍÚ˘ÂÒÍÓ ÔËÎÓÊÂÌË – ÚÂÎÂÍÓÏÛÌË͇ˆËË, ÙËÁË͇, ÚÂÓËfl ̇ ˜ËÒ·ڇ, ‡Ì‡ÎËÁ ̇ Ò˄̇ÎË, ÒÚ‡ÚËÒÚË͇, ÚÂÓËfl ̇ ‚ÂÓflÚÌÓÒÚËÚÂ, ‡ÍÛÒÚË͇, ÓÔÚË͇, Ó͇ÌÓ„‡ÙËfl, ÍËÔÚÓ„‡ÙËfl, „ÂÓÏÂÚËfl, ωˈË̇. Ç ‰ˈ‡ ÔËÎÓÊÂÌËfl ËÌÙÓχˆËflÚ‡ Á‡ Ù‡Á‡Ú‡ ̇ Ò˄̇· Ìflχ Ò˙˘ÂÒÚ‚ÂÌÓ Á̇˜ÂÌËÂ. èÓ Ú‡Í˙‚ ̇˜ËÌ ÏÓÊ ‰‡ Ò ‡Ì‡ÎËÁË‡ Ò‡ÏÓ Á‡‚ËÒËÏÓÒÚÚ‡ ̇ ‡ÏÔÎËÚÛ‰‡Ú‡ ÒÔflÏÓ ˜ÂÒÚÓÚ‡Ú‡ – „ÂÌÂË‡ Ò ‡ÏÔÎËÚÛ‰ÂÌ ÒÔÂÍÚ˙ (14,19,24,68). ëΉ ÔÓÎÛ˜‡‚‡ÌÂÚÓ Ì‡ ‡ÏÔÎËÚÛ‰ÌËfl ÒÔÂÍÚ˙ ËÎË ÒÔÂÍÚ˙‡ ̇ ÏÓ˘ÌÓÒÚÚ‡, ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÍÓ΢ÂÒÚ‚ÂÌÓ ÓˆÂÌÂÌË ‰ˈ‡ ÒÔÂÍÚ‡ÎÌË ı‡‡ÍÚÂËÒÚËÍË Ì‡ ÖÖÉ Ò˄̇·. í‡ÍË‚‡ Ò‡: ‡·ÒÓβÚ̇ Ë ÓÚÌÓÒËÚÂÎ̇ ‡ÏÔÎËÚÛ‰‡ ËÎË ÏÓ˘ÌÓÒÚ, ˜ÂÒÚÓÚÌË Ô‡‡ÏÂÚË Í‡ÚÓ Edge frequency („‡Ì˘̇ ˜ÂÒÚÓÚ‡), Mean/AvÂr‡ge frequency (Ò‰̇ ˜ÂÒÚÓÚ‡), Peak frequency (ÔËÍÓ‚‡ ˜ÂÒÚÓÚ‡), Median frequency (Ò‰ËÌ̇ ˜ÂÒÚÓÚ‡). ÇÒÂÍË Â‰ËÌ ÓÚ ÚÂÁË Ô‡‡ÏÂÚË ÏÓÊ ‰‡ ·˙‰Â ‡Ì‡ÎËÁË‡Ì Á‡ ˆÂÎËfl ËÁÒΉ‚‡Ì ˜ÂÒÚÓÚÂÌ ‰Ë‡Ô‡ÁÓÌ ËÎË Á‡ ‰‡‰Â̇ ˜‡ÒÚ ÓÚ ÌÂ„Ó /̇Ô. ‡ÎÙ‡ ‰Ë‡Ô‡ÁÓÌ/, ͇ÚÓ ÏÓÊ ‰‡ ·˙‰Â ‡Ì‡ÎËÁË‡Ì Ë Ò˙ÔÓÒÚ‡‚flÌ Ò˙˘Ó ڇ͇ ÔÓ „ËÓÌË ËÎË ÔÓ ÓÚ‚Âʉ‡ÌËfl (19). É·‚ÌÓ Ó„‡Ì˘ÂÌË ̇ ÒÔÂÍÚ‡ÎÌËfl ‡Ì‡ÎËÁ  Á‡„Û·‡Ú‡ ̇ ËÌÙÓχˆËfl Á‡ ÏÓÙÓÎÓ„ËflÚ‡ ̇ ‚˙ÎÌËÚ ÒΉ Ú‡ÌÒÙÓχˆËflÚ‡ ËÏ ‚ ˜ÂÒÚÓÚÌËfl ‰ÓÏÂÈÌ. ч‰Â̇ ÒÚÓÈÌÓÒÚ Ì‡ ˜ÂÒÚÓÚ̇ ı‡‡ÍÚÂËÒÚË͇ Ì ÔÓ͇Á‚‡ ‚˙ÎÌÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëfl, ÌËÚÓ ÍÓÎÍÓ Ô˙ÚË ‰‡‰Â̇ ‚˙Î̇ Ò  ÔÓfl‚Ë·. èÓ Ú‡Í˙‚ ̇˜ËÌ Â‰Ì‡ Ë Ò˙˘‡ ÒÚÓÈÌÓÒÚ Ì‡ ÒÔÂÍÚ‡Î̇ ı‡‡ÍÚÂËÒÚË͇ ÏÓÊ ‰‡ ·˙‰Â ÂÁÛÎÚ‡Ú ËÎË ÓÚ ÌÂÔÂÍ˙Ò̇ÚË ÌËÒÍÓ‡ÏÔÎËÚÛ‰ÌË ‚˙ÎÌË ËÎË ÓÚ ËÌÚÂÏËÚÂÌÚÌË ‚ËÒÓÍÓ‡ÏÔÎËÚÛ‰ÌË ‚˙ÎÌË ‚ Ò˙˘Ëfl ˜ÂÒÚÓÚÂÌ ‰Ë‡Ô‡ÁÓÌ. ëΉӂ‡ÚÂÎÌÓ ÍÎËÌ˘ÌÓ Á̇˜Ëχ ËÌÙÓχˆËfl (͇ÚÓ Ì‡ÔËÏÂ ̇΢ËÂÚÓ Ì‡ ÂÔËÎÂÔÚËÙÓÏÂÌË Ú‡ÌÁËÂÌÚË) ÏÓÊ ‰‡ ·˙‰Â Á‡„Û·Â̇, ÔÓ‡‰Ë ÍÓÂÚÓ Â ËÁÍβ˜ËÚÂÎÌÓ ‚‡ÊÌÓ ËÁıӉ̇ڇ ÖÖÉ ‰‡ ·˙‰Â Ô‰‚‡ËÚÂÎÌÓ ‚ËÁÛ‡ÎÌÓ ‡Ì‡ÎËÁË‡Ì‡. ë˙˘Ó ڇ͇  ‚‡ÊÌÓ ÓÔËÚÂÌ Â̈ÂÙ‡ÎÓ„‡ÙËÒÚ ‰‡ ËÁ·Â Ò„ÏÂÌÚËÚ ÓÚ ÖÖÉ Á‡ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ – Ò Ó„Î‰ ‰‡ Ò ‡Ì‡ÎËÁË‡Ú Ò„ÏÂÌÚË ·ÂÁ ‡ÚÂÙ‡ÍÚË ËÎË Ú‡ÌÁËÂÌÚË Ë ‰‡ Ò ÛÎÂÒÌË ËÌÚÂÔÂÚ‡ˆËflÚ‡ ̇ ÂÁÛÎÚ‡ÚËÚ (19,43). èË·„‡Ú ÒÂ Ë ‰Û„Ë ÏÂÚÓ‰Ë, ÍÓËÚÓ Ò‡‚Ìfl‚‡Ú Ò˄̇· ÔÓ ˜ÂÒÚÓÚ‡, ‡ÏÔÎËÚÛ‰‡ Ë Ù‡Á‡. èӂ˜ÂÚÓ ÓÚ Úflı Ò ÓÒÌÓ‚‡‚‡Ú ̇, ËÎË ËÁÔÓÎÁ‚‡Ú ÍÓÒ-ÍÓ·ˆËÓÌÂÌ ‡Ì‡ÎËÁ Ë ÍÓıÂÂÌÚÌË ÙÛÌ͈ËË. ÇÒ Ӣ ‚ ÂÍÒÔÂËÏÂÌÚ‡ÎÌË ÛÒÎÓ‚Ëfl Ë ÓÚ ÏÛÎÚˉËÒˆËÔÎË̇ÌË ÂÍËÔË Ò ‡Á‡·ÓÚ‚‡Ú ÌÓ‚Ë Ë ‚Ò ÔÓ-ÒÎÓÊÌË ÏÂÚÓ‰Ë Á‡ ÖÖÉ ‡Ì‡ÎËÁ - Ì‚ÓÌÌË ÏÂÊË, ÚÂÓËfl ̇ ı‡ÓÒ‡ Ë ‰. (14,19,43). èêàãéÜÖçàü çÄ äéãàóÖëíÇÖçàíÖ åÖíéÑà áÄ ÄçÄãàá çÄ ÖÖÉ éÒÌÓ‚ÌÓÚÓ ÔËÎÓÊÂÌË ̇ ÖÖÉ Â ‚ ‰Ë‡„ÌÓÒÚË͇ڇ Ë ı‡‡ÍÚÂËÁË‡ÌÂÚÓ Ì‡ ̇Û¯ÂÌËflÚ‡ ̇ ÏÓÁ˙˜Ì‡Ú‡ ‡ÍÚË‚ÌÓÒÚ. åÌÓ„Ó Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl ÏÓ„‡Ú ‰‡ ̇Û¯‡Ú ÙÛÌ͈ËflÚ‡ ̇ ÏÓÁ˙͇, ·ÂÁ ÌÂÔÂÏÂÌÌÓ ‰‡ Á‡Òfl„‡Ú Ì„ӂ‡Ú‡ ÒÚÛÍÚÛ‡. ÖÖÉ ÏÓÊ ‰‡ ÓÒË„ÛË ‚‡Ê̇ ‰Ë‡„ÌÓÒÚ˘̇ ËÌÙÓχˆËfl ‚ ÒÎÛ˜‡ËÚÂ, ÍÓ„‡ÚÓ äí ËÎË åêí, ÍÓËÚÓ ÓÒÌÓ‚ÌÓ ËÁÒΉ‚‡Ú ‡Ì‡ÚÓÏ˘̇ڇ ÒÚÛÍÚÛ‡ ̇ ÏÓÁ˙͇, Ò‡ ÌÓχÎÌË ËÎË Ì‰ÓÒÚ‡Ú˙˜ÌÓ ËÌÙÓχÚË‚ÌË. èË Ì‡Î˘ËÂÚÓ Ì‡ ÒÚÛÍÚÛÌË ÔÓÏÂÌË, ˉÂÌÚËÙˈË‡ÌË ˜ÂÁ Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ÚÂıÌËÍË, ÖÖÉ ÏÓÊ ‰‡ ‰‡‰Â ‰ÓÔ˙ÎÌËÚÂÎ̇ ËÌÙÓχˆËfl ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚËÔ‡ Ë ÒÚÂÔÂÌÚ‡ ̇ ÔˉÛʇ‚‡˘ËÚ ÙÛÌ͈ËÓ̇ÎÌË Ì‡Û¯ÂÌËfl (5,43,19,68). äÓ΢ÂÒÚ‚ÂÌËÚ ÖÖÉ ÏÂÚÓ‰Ë Ò‡ Á̇˜ËÏË, ͇ÍÚÓ ‚ ̇ۘÂÌ, ڇ͇ Ë ‚ ÍÎËÌ˘ÂÌ ‡ÒÔÂÍÚ ÔË ‰Ë‡„ÌÓÁ‡Ú‡ Ë ‰ËÙÂÂ̈ˇÎ̇ڇ ‰Ë‡„ÌÓÁ‡, ÍÓ΢ÂÒÚ‚Â̇ڇ ÓˆÂÌ͇ ̇ ÍÓ„ÌË-


‰ÂÍÂÏ‚Ë, 2007

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

ÚË‚ÌËÚ ̇Û¯ÂÌËfl, ÔÓÒΉfl‚‡ÌÂÚÓ Ë Î˜ÂÌËÂÚÓ Ì‡ ‰ÂÏÂ̈ËflÚ‡. èӂ˜ÂÚÓ ÔÓÏÂÌË ‚ ÍÓ΢ÂÒÚ‚ÂÌËÚ ԇ‡ÏÂÚË Ì‡ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÔË ‰ÂψËfl ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÛÒÚ‡ÌÓ‚ÂÌË Ë Ì‡ ÛÚËÌ̇ڇ ÖÖÉ, ÌÓ ÍÓ΢ÂÒÚ‚ÂÌËflÚ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ÛÒÚ‡ÌÓ‚Ë ÔÓ Í‡Ú„Ó˘ÂÌ Ì‡˜ËÌ Ì‡ ÔÓ-‡ÌÂÌ ÂÚ‡Ô Ì‡Î˘ËÂÚÓ Ì‡ ‡·ÌÓÏÌÓ Á‡·‡‚flÌ ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ, ÍÓÂÚÓ ‰‡ ·˙‰Â ÍÓ΢ÂÒÚ‚ÂÌÓ ÓˆÂÌÂÌÓ, Ë ÚÓ‚‡ ÌÓÒË ‰ÓÔ˙ÎÌËÚÂÎ̇ ËÌÙÓχÚË‚ÌÓÒÚ Í˙Ï Òڇ̉‡Ú̇ڇ ÖÖÉ. äÓ΢ÂÒÚ‚Â̇ڇ ËÁ‡ÁÂÌÓÒÚ Ì‡ ÔÓÏÂÌËÚ ÍÓÂÒÔÓ̉Ë‡ Ò ÚÂÊÂÒÚÚ‡ ̇ ÍÓ„ÌËÚË‚ÌËfl ‰ÂÙˈËÚ Ë ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ ÔÓ„ÂÒËfl (43). ê‡Á΢ÌË ÍÓÎÂÍÚË‚Ë ËÁÛ˜‡‚‡Ú ÔÓÏÂÌËÚ ‚ ÍÓ΢ÂÒÚ‚ÂÌËÚ ԇ‡ÏÂÚË Ë ‚˙ÁÏÓÊÌÓÒÚËÚ ̇ ÍÓ΢ÂÒÚ‚ÂÌËÚ ÖÖÉ ÏÂÚÓ‰Ë ‚ ‡Á΢ÌË ‡ÒÔÂÍÚË: 1. ᇠӈÂÌ͇ ̇ ̇΢ËÂÚÓ Ë ÒÚÂÔÂÌÚ‡ ̇ ÍÓ„ÌËÚË‚ÌË Ë Ô‡ÏÂÚÓ‚Ë Ì‡Û¯ÂÌËfl (7,34,35). 2. ᇠÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ Ó„‡Ì˘̇ ÂÚËÓÎÓ„Ëfl ̇ ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl Ë ‰ËÙËÂ̈Ë‡Ì ÓÚ ÙÛÌ͈ËÓ̇ÎÌË Ò˙ÒÚÓflÌËfl ͇ÚÓ Ú‚ÓÊÌÓÒÚ, ‰ÂÔÂÒËfl Ë ‰., (46). 3. ᇠ‰ËÙÂÂ̈Ë‡Ì ̇ ÅÓÎÂÒÚÚ‡ ̇ ÄΈı‡ÈÏÂ /ÅÄ/ Ë Ò˙‰Ó‚‡Ú‡ ‰ÂÏÂ̈Ëfl ÓÚ ÍÓ„ÌËÚË‚ÌËÚ ÔÓÏÂÌË ÔË ÌÓχÎÌÓÚÓ ÒÚ‡ÂÂÌ (4,10,28,35,38,48,49). 4.ᇠ‡Á„‡Ì˘‡‚‡Ì ̇ ÅÄ ÓÚ ‰Û„Ë ‰ÂÏÂ̈ËË, ‡ ڇ͇ Ò˙˘Ó ÏÂÊ‰Û ÙÓÏËÚ ̇ ÅÄ Ò ‡Á΢̇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓ (51,52). 5. äÓ·ˆËfl ÏÂÊ‰Û ‰‡ÌÌËÚ ÓÚ ÍÓ΢ÂÒÚ‚ÂÌËfl ÖÖÉ ‡Ì‡ÎËÁ Ë êÖí ÔË Á‰‡‚Ë ËÌ‰Ë‚Ë‰Ë Ë Á‡ ‰ÂÚ��͈ËflÚ‡ ̇ Ò˙‰Ó‚‡ Ë ‰Â„ÂÌÂ‡Ú˂̇ ‰ÂÏÂ̈Ëfl (4,62,63,64). 6. ᇠӈÂÌ͇ ̇ ÂÙÂÍÚËÚ ̇ ‡Á΢ÌË ÎÂ͇ÒÚ‚‡ ‚˙ıÛ ÒÔÂÍÚ‡ÎÌËÚ ԇ‡ÏÂÚË, ͇ÍÚÓ ÔË Á‰‡‚Ë, ڇ͇ Ë ÔË ‡Á΢ÌË ÙÓÏË Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl. (3,40,54). 7. ᇠӈÂÌ͇ ̇ ÚÂÊÂÒÚÚ‡ Ë ÔÓ„ÌÓÁ‡Ú‡ ̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (6). Ç˙ÔÂÍË, ˜Â äí Ë åêí Ò‡ ÓÒÌÓ‚ÂÌ ÏÂÚÓ‰ ̇ ËÁ·Ó Á‡ ÎÓ͇ÎËÁ‡ˆËfl Ë ‰ËÙÂÂ̈Ë‡Ì ̇ ËÌÙ‡ÍÚ ÓÚ ıÂÏÓ‡„Ëfl, ÚÛÏÓ ËÎË ‰Û„Ë ÙÓ͇ÎÌË ÏÓÁ˙˜ÌË ÎÂÁËË (59), ÔÓÛ˜‚‡ÌËfl ÔË ÏÓÁ˙˜ÌÓ-Ò˙‰Ó‚‡Ú‡ ·ÓÎÂÒÚ Ò‡ ÔÓ͇Á‡ÎË, ˜Â ‰ˈ‡ ÒÔÂÍÚ‡ÎÌË ÖÖÉ Ô‡‡ÏÂÚË ËÏ‡Ú ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ Ì‡‰ 80% Ë ‰Ó·‡ ÒÔˆËÙ˘ÌÓÒÚ, ͇ÍÚÓ Ë Á̇˜Ëχ ÍÓ·ˆËfl ÏÂÊ‰Û ÖÖÉ ÔÓÏÂÌËÚÂ Ë „ËÓ̇ÎÌËfl ÏÓÁ˙˜ÂÌ Í˙‚ÓÚÓÍ ËÎË ÏÂÚ‡·ÓÎËÁ˙Ï ‚ ËÒıÂÏ˘ÌË Ë ÌÂËÒıÂÏ˘ÌË Ó·Î‡ÒÚË. (42). åÌÓ„Ó ÓÚ Úflı Ò‡ ‰ÂÏÓÌÒÚË‡ÎË, ˜Â ÍÓ΢ÂÒÚ‚ÂÌËflÚ ‡Ì‡ÎËÁ ̇ ÖÖÉ ÏÓÊ ‰‡ ÛÒÚ‡ÌÓ‚Ë Ò ‚ËÒÓ͇ ÒÚÂÔÂÌ Ì‡ ‰ÓÒÚÓ‚ÂÌÓÒÚ ÙÓ͇ÎÌË ÔÓÏÂÌË, ÍÓËÚÓ Ò‡ ‰ËÒÍÂÚÌË Ë ·Ëı‡ ÏÓ„ÎË ‰‡ ·˙‰‡Ú ÔÓÔÛÒ̇ÚË ÔË Ô˙‚Ó̇˜‡ÎÌËfl ‚ËÁÛ‡ÎÂÌ ‡Ì‡ÎËÁ ̇ ÖÖÉ (44), ÓÒÓ·ÂÌÓ ‚ ̇˜‡ÎÓÚÓ, ÍÓ„‡ÚÓ äí  ‚Ò Ӣ ÌÓχÎ̇ ËÎË ÍÓ„‡ÚÓ ËÒıÂÏËflÚ‡  ÎÂ͇ Ë Ô‰ËÁ‚ËÍ‚‡ ÏÓÁ˙˜Ì‡ ‰ËÒÙÛÌ͈Ëfl ·ÂÁ ÏÓÙÓÎӄ˘ÌË ËÁÏÂÌÂÌÂÌËfl (30). ë˙˜ÂÚ‡ÌËÂÚÓ Ì‡ ÌÂÔÂÍ˙Ò̇ÚÓ ÖÖÉ ÏÓÌËÚÓË‡ÌÂ Ò ‡‚ÚÓχÚ˘̇ ‰ÂÚÂ͈Ëfl ̇ ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË Ë ÍÓÏÔÂÒË‡Ì ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ÛÒÚ‡ÌÓ‚flÚ ‡ÌÓ ÓÒÚË ËÌÚ‡Í‡ÌˇÎÌË ÛÒÎÓÊÌÂÌËfl Ë ‰‡ Ò ÔÓ‚Âʉ‡ ÒÍËÈÌËÌ„ Á‡ ‚˙ÁÏÓÊÌË ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË ÔË ‚ËÒÓÍÓ-ËÒÍÓ‚Ë Ô‡ˆËÂÌÚË ‚ ËÌÚÂÌÁË‚ÌÓ ÓÚ‰ÂÎÂÌË ËÎË ÔÓ ‚ÂÏ ̇ ÓÔÂ‡ˆËË Í‡ÚÓ Í‡ÓÚˉ̇ Ẩ‡ÚÂÂÍÚÓÏËfl (43,53,67), ‡ ڇ͇ Ò˙˘Ó ÔÓÁ‚ÓÎfl‚‡ ÍÓÌÚÓΠ̇ ÚÂ‡ÔËflÚ‡ (26,27). ë˙˘ÂÒÚ‚Û‚‡Ú „ÓÎflÏ ·ÓÈ ÔÛ·ÎË͇ˆË, ÔË·„‡˘Ë ‡Á΢ÌË ÏÂÚÓ‰Ë Ì‡ ÍÓ΢ÂÒÚ‚ÂÌ ‡Ì‡ÎËÁ Á‡ ÓˆÂÌ͇ ̇ Ô‡ˆËÂÌÚË Ò ÚÛÏÓË, ‡ÏËÓÚÓÙ˘̇ ·ÚÂ‡Î̇ ÒÍÎÂÓÁ‡, ÅÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ, Gilles de la Tourette ÒË̉ÓÏ, óåí, ÏË„Â̇, ıÓÌ˘̇ ·ÓÎ͇, ¯ËÁÓÙÂÌËfl, ‰ÂÔÂÒËfl, ‡ÎÍÓıÓÎËÁ˙Ï, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ, ËÌÚÓÍÒË͇ˆËË, Ì‚ÓÙËÁËÓÎӄ˘ÌË ÔÓÛ˜‚‡ÌËfl ÔË ‰Âˆ‡ Ò Ì‡Û¯ÂÌËfl ̇ ‚ÌËχÌËÂÚÓ Ë Ó·Û˜ÂÌËÂÚÓ (39,43,46). îÄêåÄäé ÖÖÉ äÓ΢ÂÒÚ‚ÂÌËflÚ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÏÓÁ˙͇  ÛÚ‚˙‰Â̇ ÏÂÚÓ‰Ë͇ Á‡ Ó·ÂÍÚË‚ÌÓ

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ÓÔËÒ‡ÌË ̇ ‚ÎËflÌËÂÚÓ ‚˙ıÛ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ ̇ ‡Á΢ÌË ÎÂ͇ÒÚ‚‡, ÔË·„‡ÌË ÔË ıÓ‡Ú‡ (24). é˘Â H. Berger ‚ ‰ËÒÂÚ‡ˆËflÚ‡ ÒË “Uber das Electroencephalogram des Menschen” (1931) Ò˙Ó·˘‡‚‡ Á‡ ÖÖÉ ÔÓÏÂÌË ‚ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÒΉ ÔËÂÏ Ì‡ ÔÒËıÓÚÓÔÌË ÎÂ͇ÒÚ‚‡ ͇ÚÓ ·‡·ËÚÛ‡ÚË, ÍÓ͇ËÌ, ÏÓÙËÌ Ë ÒÍÓÔÓ·ÏËÌ. èÂÁ ÒΉ‚‡˘ËÚ „Ó‰ËÌË Ò ÔÓfl‚fl‚‡Ú „ÓÎflÏ ·ÓÈ ÔÛ·ÎË͇ˆËË, ÓÔËÒ‚‡˘Ë ÂÙÂÍÚËÚ ̇ ‡Á΢ÌË ÎÂ͇ÒÚ‚‡ ‚˙ıÛ ˜Ó‚¯͇ڇ ÖÖÉ. èÂÁ 80-Ú „Ó‰ËÌË Ì‡‚ÎËÁ‡ÌÂÚÓ Ì‡ ÍÓÏÔ˛Ú˙ÌËÚ ÚÂıÌÓÎÓ„ËË Ë ÍÓ΢ÂÒÚ‚ÂÌËÚ ÖÖÉ ÏÂÚÓ‰Ë Ô‰ÓÒÚ‡‚flÚ Á̇˜ËÚÂÎÌË ‚˙ÁÏÓÊÌÓÒÚË ‰‡ Ò ‡Ì‡ÎËÁË‡Ú Ë ‰ËÙÂÂ̈Ë‡Ú ÂÙÂÍÚËÚ ̇ ‡Á΢ÌË ÎÂ͇ÒÚ‚‡ Ë ÓÚÍË‚‡Ú ¯ËÓÍÓ ÔÓΠ̇ ÔËÎÓÊÂÌË ‚ ÎÂ͇ÒÚ‚ÂÌËÚ ÔÓÛ˜‚‡ÌËfl Ë ÍÎËÌ˘̇ڇ Ù‡χÍÓÎÓ„Ëfl. ë˙Á‰‡‚‡ Ò ÌÓ‚Ó Ì‡Ô‡‚ÎÂÌË – Ù‡χÍÓ-ÖÖÉ (24). ÑÌÂÒ ÔÓÛ˜‚‡ÌËflÚ‡ ̇ ÂÙÂÍÚËÚ ̇ ÔÒËıÓÚÓÔÌËÚ ÎÂ͇ÒÚ‚‡ ‚˙ıÛ ÏÓÁ˙˜Ì‡Ú‡ ‡ÍÚË‚ÌÓÒÚ Ò˙˘ÂÒÚ‚ÂÌÓ Ò‡ Ò ‡Á‚ËÎË ÓÚ Í‡˜ÂÒÚ‚Â̇ ÓˆÂÌ͇ Í˙Ï ÔÓ-‰ÓÒÚÓ‚ÂÌË ÍÓ΢ÂÒÚ‚ÂÌË ÏÂÚÓ‰Ë Ò ˆÂÎ ‰‡ Ò ÛÒÚ‡ÌÓ‚flÚ Ó·ÂÍÚË‚ÌÓ ÂÙÂÍÚËÚ ̇ ÔÒËıÓÚÓÔÌËÚ ÎÂ͇ÒÚ‚‡ ‚˙ıÛ ÙÛÌ͈ËflÚ‡ ̇ ˜Ó‚¯ÍËfl ÏÓÁ˙Í (33). î‡χÍÓ-ÖÖÉ ÏÂÚÓ‰Ë͇ڇ ÔÓ Ò‚ÓflÚ‡ Ò˙˘ÌÓÒÚ Ô‰ÒÚ‡‚Îfl‚‡ ÔË·„‡Ì ̇ ÍÓÏÔ˛Ú˙ÌÓ ·‡ÁË‡ÌË ÍÓ΢ÂÒÚ‚ÂÌË ÏÂÚÓ‰Ë Á‡ ‡Ì‡ÎËÁ ̇ ˜Ó‚¯͇ڇ Â̈ÂÙ‡ÎÓ„‡Ï‡, Óڂ‰Â̇ ÓÚ Ò͇ÎÔ‡, ÍÓËÚÓ ‚ ÍÓÏ·Ë̇ˆËfl Ò ÓÔ‰ÂÎÂÌË ÒÚ‡ÚËÒÚ˘ÂÒÍË ‡Ì‡ÎËÁË Ë ËÁÓ·‡Áfl‚‡˘Ë ÚÂıÌËÍË, ÔÓÁ‚ÓÎfl‚‡Ú ‰‡ Ò ̇Ô‡‚Ë ÓˆÂÌ͇ ̇ ÂÙÂÍÚËÚ ̇ ÔÒËıÓÚÓÔÌËÚ ÎÂ͇ÒÚ‚‡, ‚˙Á ÓÒÌÓ‚‡ ̇ ÍÓflÚÓ Ú ÏÓ„‡Ú ‰‡ Ò ÍÎËÒËÙˈË‡Ú Ë ‰‡ Ò ӈÂÌË Ó·ÂÍÚË‚ÌÓ Úflı̇ڇ ·ËÓ̇΢ÌÓÒÚ ‚ Ú‡„ÂÚÌËfl Ó„‡Ì – ˜Ó‚¯ÍËfl ÏÓÁ˙Í. åÂÚÓ‰˙Ú ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ÓÔ‰ÂÎË Ó˘Â ‚ ‡ÌÂÌ ÒÚ‡‰ËÈ ‰‡ÎË Â‰ÌÓ ÎÂ͇ÒÚ‚Ó Ëχ ÂÙÂÍÚË ‚˙ıÛ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ, ͇͂‡ ·Ë Ïӄ· ‰‡ ·˙‰Â ÍÎËÌ˘̇ڇ ÂÙË͇ÒÌÓÒÚ, ÔË Í‡Í‚Ë ‰ÓÁË Ó͇Á‚‡ ÂÙÂÍÚ, ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ÂÙÂÍÚ‡, ÂÍ‚ËÔÓÚÂ̈ˇÎÌËÚ ‰ÓÁË Ì‡ ‡Á΢ÌË ÎÂ͇ÒÚ‚ÂÌË ÙÓÏË. á‡Â‰ÌÓ Ò ÔÒËıÓÙ‡χÍÓÎӄ˘ÌËÚ Ôӈ‰ÛË, Ù‡χÍÓ-ÖÖÉ Â Ì‡È-˜Û‚ÒÚ‚ËÚÂÎÌËflÚ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ÎÂ͇ÒÚ‚ÂÌÓ Ë̉ۈË‡ÌËÚ ÔÓÏÂÌË ‚˙‚ ÙÛÌ͈ËËÚ ̇ ˜Ó‚¯ÍËfl ÏÓÁ˙Í, ÍÓÂÚÓ ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ÓˆÂÌ͇ ̇ ÎÂ͇ÒÚ‚ÂÌËÚ ÂÙÂÍÚË ÔÓ Â‰ËÌ ÌÂËÌ‚‡ÁË‚ÂÌ, ·ÂÁÓÔ‡ÒÂÌ Ë ÂÔÓ‰ÛˆËÛÂÏ Ì‡˜ËÌ, Ò‡ Ô‚˙̇ÎË Ú‡ÁË ÏÂÚÓ‰Ë͇ ‚ ‡·ÒÓβÚ̇ ÌÂÓ·ıÓ‰ËÏÓÒÚ Á‡ Ô˙ÎÌÓ ÍÓ΢ÂÒÚ‚ÂÌÓ ÓÔËÒ‡ÌË ̇ ÂÙÂÍÚËÚ ̇ ÎÂ͇ÒÚ‚‡Ú‡ ‚˙ıÛ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ (25). éÒÌÓ‚ÌËÚ Á‡‰‡˜Ë ̇ Ù‡χÍÓ-ÖÖÉ Ò‡ ‰‡ ÓÔ‰ÂÎË ‰‡ÎË Â‰ÌÓ ÎÂ͇ÒÚ‚Ó Ó͇Á‚‡ ÂÙÂÍÚ ‚˙ıÛ ñçë, ÍÓÂÚÓ Ò ‰Ó͇Á‚‡ ˜ÂÁ ÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏË ‡ÁÎËÍË ÏÂÊ‰Û ÒÔÂÍÚ‡ÎÌËÚ ÔÓÙËÎË ÔË Ô·ˆÂ·Ó Ë ËÁÒΉ‚‡ÌÓÚÓ ÎÂ͇ÒÚ‚Ó; ‰‡ ÓÔ‰ÂÎË ÏËÌËχÎ̇ڇ ÂÙÂÍÚ˂̇ ‰ÓÁ‡ ̇ ÎÂ͇ÒÚ‚ÓÚÓ, ÍÓÂÚÓ Ò ‰Ó͇Á‚‡ ˜ÂÁ ̇ÈχÎ͇ڇ ‰ÓÁ‡, Ô‰ËÁ‚ËÍ‚‡˘‡ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ÔÓÏfl̇ ‚ ÒÔÂÍÚ‡ÎÌËfl ÔÓÙËÎ ÒÔflÏÓ Ô·ˆÂ·Ó; ‰‡ ÓÔ‰ÂÎË ‚ÂÏÂÚÓ Ì‡ ̇ÒÚ˙Ô‚‡ÌÂ Ë ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ÂÙÂÍÚ‡ ‚˙ıÛ ñçë, ÍÓÂÚÓ Ò ‰Ó͇Á‚‡ ˜ÂÁ ̇˜‡ÎÓÚÓ Ë Í‡fl ̇ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜ËÏËÚ ÔÓÏÂÌË ‚ ÒÔÂÍÚ‡ÎÌËfl ÔÓÙËÎ ÒÔflÏÓ Ô·ˆÂ·Ó. ëÔˆËÙ˘ÌÓÚÓ ÔËÎÓÊÂÌË ̇ ÍÓ΢ÂÒÚ‚Â̇ڇ ÖÖÉ ‚‡Ë‡ ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÚËÔ‡ ̇ ËÁÒΉ‚‡ÌÓÚÓ ÎÂ͇ÒÚ‚Ó (̇Ô. ÔÒËıÓÚÓÔÌË ÒÔflÏÓ ÌÂÔÒËıÓÚÓÔÌË ÎÂ͇ÒÚ‚‡, ÌÓ Ò ÔÓÚÂ̈ˇÎÌË ÌÂÊ·ÌË ÂÙÂÍÚË ÓÚ ÒÚ‡Ì‡ ̇ ñçë) Ë ı‡‡ÍÚÂËÒÚËÍËÚ ̇ Ú‡„ÂÚ̇ڇ ÔÓÔÛ·ˆËfl (̇Ô. Á‰‡‚Ë Ë̉˂ˉË, ËÎË ‚ ̇Ô‰̇· ‚˙Á‡ÒÚ, Ô‡ˆËÂÌÚË Ò Ì‚ÓÎӄ˘ÌË ËÎË ÔÒËıˇÚ˘ÌË Á‡·ÓÎfl‚‡ÌËfl) (33). äÓ΢ÂÒÚ‚ÂÌËÚ ÏÂÚÓ‰Ë ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ËÁÔÓÎÁ‚‡ÌË Ë Á‡ Ô‰‚Ëʉ‡Ì ̇ ÚÂ‡Ô‚Ú˘ÌËÚ ͇˜ÂÒÚ‚‡ ̇ ÌÓ‚Ë ÎÂ͇ÒÚ‚‡, Á‡ Ò˙ÔÓÒÚ‡‚͇ ÏÂÊ‰Û Ù‡χÍÓ‰Ë̇ÏË͇ڇ Ë Ù‡χÍÓÍËÌÂÚË͇ڇ ̇ ‰‡‰ÂÌÓ ÎÂ͇ÒÚ‚Ó, ̇ÔËÏÂ Á‡


‰ÂÍÂÏ‚Ë, 2007

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

ÓˆÂÌ͇ ̇ ˆÂÌÚ‡ÎÌËÚ ÂÙÂÍÚË ÒÔÓ‰ ‰ÓÁ‡Ú‡ Ë ‚ÂÏÂÚÓ, ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ËÁÔÓÎÁ‚‡ÌË Á‡ Ò˙Á‰‡‚‡Ì ̇ Ë̉ÂÍÒË Á‡ ˆÂÌÚ‡Î̇ ·ËÓ̇΢ÌÓÒÚ Ë Á‡ ÓÔ‰ÂÎflÌ ̇ ÚÂ‡Ô‚Ú˘ÌËfl ÔÓÁÓˆ. ë˙˘ÂÒÚ‚Û‚‡Ú ‚˙ÁÏÓÊÌÓÒÚË, ÔË ÍÓËÚÓ ÏÂÚÓ‰Ë͇ڇ ÏÓÊ ‰‡ ÓÒË„ÛË Ì‡ ÍÎËÌˈËÒÚ‡ Ó·ÂÍÚ˂̇ ·‡Á‡ ÔË ¯ÂÌËÂÚÓ Á‡ ËÁ·Ó Ë ‰ÓÁË‡Ì ̇ ‰‡‰ÂÌÓ ÎÂ͇ÒÚ‚Ó (29). èÓÒ‰ÒÚ‚ÓÏ Ù‡χÍÓ-ÖÖÉ ÏÂÚÓ‰ÓÎÓ„ËflÚ‡  Ò˙Á‰‡‰Â̇ Ë Í·ÒËÙË͇ˆËfl ̇ ÔÒËıÓÚÓÔÌËÚ ÎÂ͇ÒÚ‚‡. Ç˙Á ÓÒÌÓ‚‡ ̇ ÎÂ͇ÒÚ‚ÂÌÓ Ë̉ۈË‡ÌËÚ ÔÓÏÂÌË ‚ ÖÖÉ ÔÓÙËÎËÚ ҇ ÓÔ‰ÂÎÂÌË 8 ÔÒËıÓÙ‡χÍÓÎӄ˘ÌË Í·҇ (퇷Î.1) (33,55). ᇠÒ˙ʇÎÂÌË ‚Ò Ӣ Ìflχ ÔӉӷ̇, ÓÒÌÓ‚‡‚‡˘‡ Ò ̇ ÍÓ΢ÂÒÚ‚ÂÌËfl ÖÖÉ ‡Ì‡ÎËÁ, Í·ÒËÙË͇ˆËfl ̇ ÓÚ‰ÂÎÌËÚ ÚËÔÓ‚Â ‡ÌÚËÂÔËÎÂÔÚ˘ÌË ÎÂ͇ÒÚ‚‡. çflχ ‰ÓÒÚ‡Ú˙˜ÌÓ ÒËÒÚÂχÚ˘ÌË ‰‡ÌÌË ÓÚ ÍÓ΢ÂÒÚ‚Â̇ڇ ÓˆÂÌ͇ ̇ ÂÙÂÍÚËÚ ̇ ‡ÌÚËÂÔËÎÂÔÚ˘ÌËÚ ÎÂ͇ÒÚ‚‡ ‚˙ıÛ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ, ͇ÚÓ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÏÌÓ„Ó·ÓÈÌËÚ ËÁÒΉ‚‡ÌËfl ̇ ÂÙÂÍÚËÚ ̇ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚËÚ ‚˙ıÛ ÖÖÉ Ò‡ ̉ÌÓÔÓÒÓ˜ÌË Ë ˜ÂÒÚÓ ÔÓÚË‚Ó˜˂Ë. ÑÛ„‡ Ò˙˘ÂÒÚ‚Â̇ Ô˘Ë̇  Á̇˜ËÚÂÎ̇ڇ ıÂÚÂÓ„ÂÌÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÓÔÓ„‡ÙÒ͇ ÎÓ͇ÎËÁ‡ˆËfl, ÏÓÙÓÎӄ˘ÂÌ ÒÛ·ÒÚ‡Ú, ‰ÓË „ÂÌÂÚ˘ÌÓ ‰ÂÚÂÏËÌË‡ÌË ÂÙÂÍÚË, ̇ÏË‡˘Ë ÓÚ‡ÊÂÌË ‚ ÏÌÓ„ÓÓ·‡ÁËÂÚÓ Ì‡ ÂÔËÎÂÔÒËËÚÂ Ë ÂÔËÎÂÔÚ˘ÌËÚ ÒË̉ÓÏË (24,50,69). íÛÍ Â ÏflÒÚÓÚÓ ‰‡ Ò ÓÚ·ÂÎÂÊË, ˜Â ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇, ‚Ò Ӣ ÌflÍÓË, ‰ÓË Ë ÂÎÂÏÂÌÚ‡ÌË ÍÓ΢ÂÒÚ‚ÂÌË ÏÂÚÓ‰Ë, Ì ҇ ̇ÏÂËÎË Ò‚ÓÂÚÓ ÛÚËÌÌÓ ÔËÎÓÊÂÌËÂ, ‚˙ÔÂÍË ˜Â ÍÓ΢ÂÒÚ‚Â̇ڇ ÖÖÉ Â ‰Ó͇Á‡Ì Ó·ÂÍÚË‚ÂÌ Ë ˜Û‚ÒÚ‚ËÚÂÎÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ڇ ̇ ˆÂÌÚ‡ÎÌËÚ ÂÙÂÍÚË Ì‡ ÎÂ͇ÒÚ‚‡Ú‡, ‚Íβ˜ËÚÂÎÌÓ Ë ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚËÚ (21,55,57). ։̇ Ò˙˘ÂÒÚ‚Â̇ Ô˘Ë̇ Â, ˜Â ÍÓ΢ÂÒÚ‚ÂÌËÚ ÂÙÂÍÚË Ì‡ Ôӂ˜ÂÚÓ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚË Ì ҇ ÓÔËÒ‡ÌË ÔÓ flÒÂÌ Ë Ì‰‚ÛÒÏËÒÎÂÌ Ì‡˜ËÌ. èӂ˜ÂÚÓ ÔÛ·ÎËÍÛ‚‡ÌË ÔÓÛ˜‚‡ÌËfl Ò‡ ÌÂÍÓÌÚÓÎË‡ÌË Ë ÓÚÍËÚË, Ò Ï‡Î˙Í ·ÓÈ Ô‡ˆËÂÌÚË, ÍÓ΢ÂÒÚ‚ÂÌËÚ ԇ‡ÏÂÚË Ë ‰ÓË Î˜ÂÌËÂÚÓ Ë Ò˙Ô˙ÚÒÚ‚‡˘‡Ú‡ ÚÂ‡ÔËfl, ı‡‡ÍÚÂËÒÚËÍËÚ ̇ Ô‡ˆËÂÌÚËÚÂ, ÔËÎÓÊÂÌËÚ ‰ÓÁË Ë Ì‡Î˘ËÂÚÓ Ì‡ ‰Û„Ë Ó͇Á‚‡˘Ë ‚ÎËflÌË هÍÚÓË, Ì ‚Ë̇„Ë Ò ÓÔËÒ‚‡Ú ‰ÓÒÚ‡Ú˙˜ÌÓ ËÌÙÓχÚË‚ÌÓ. çflÍÓË ÔÓÛ˜‚‡ÌËfl Ò‡ ÔÓ‚Âʉ‡ÌË ÔË ÛÒÎÓ‚ËflÚ‡ ̇ ‰ÓÔ˙ÎÌËÚÂÎ̇ ÚÂ‡ÔËfl ÔË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl, ‡ ‰Û„Ë Ò‡ ·ËÎË Ò ÂÍÒÔÂËÏÂÌÚ‡ÎÂÌ ‰ËÁ‡ÈÌ Ë Â‰ÌÓÍ‡ÚÌË ‰ÓÁË ÔË Á‰‡‚Ë ‰Ó·Ó‚ÓÎˆË (11). íÓ˜ÌËÚ ԇ‡ÏÂÚË Ë ÛÒÎÓ‚ËflÚ‡ ̇ ËÁÒΉ‚‡Ì Ì ‚Ë̇„Ë Ò‡ ‡‰ÂÍ‚‡ÚÌÓ ÔÓÒÓ˜ÂÌË, ˜ÂÒÚÓ ÂÁÛÎÚ‡ÚËÚ Ì ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ‚˙ÁÔÓËÁ‚‰ÂÌË (43,45). ë˙˘ÂÒÚ‚Û‚‡Ú ÏÌÓ„Ó Ï‡ÎÍÓ Ì‡ ·ÓÈ ÔÓÛ˜‚‡ÌËfl, ÓˆÂÌfl‚‡˘Ë ‰˙΄ÓÚ‡ÈÌËÚ ÂÙÂÍÚË ÓÚ Î˜ÂÌËÂÚÓ Ò ‡ÌÚËÂÔËÎÂÔÚ˘ÌË ÎÂ͇ÒÚ‚‡, ÍÓËÚÓ ·Ëı‡ ÏÓ„ÎË ‰‡ ·˙‰‡Ú Ô‰ÒÚ‡‚ËÚÂÎÌË Á‡ ÔÓ‰˙ÎÊËÚÂÎ̇ڇ ‡ÌÚËÂÔËÎÂÔÚ˘̇ ÚÂ‡ÔËfl ‚ ‡ÎÌËÚ ÛÒÎÓ‚Ëfl ̇ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇ (11). èêàãéÜÖçàÖ çÄ äéãàóÖëíÇÖçàü ÖÖÉ ÄçÄãàá Ç ÖèàãÖèëàüíÄ á̇˜ËÚÂÎÌËflÚ Ì‡Ô‰˙Í ‚ ÍÓÏÔ˛Ú˙ÌËÚ ÚÂıÌÓÎÓ„ËË ‰ÌÂÒ Ì ÔÓÏÂÌfl Ò˙˘ÂÒÚ‚ÂÌÓ ÓÎflÚ‡ ̇ ÖÖÉ, ÍÓflÚÓ ÓÒÚ‡‚‡ Á·ÚÂÌ Òڇ̉‡Ú ‚ ‰Ë‡„ÌÓÁ‡Ú‡ Ë Î˜ÂÌËÂÚÓ Ì‡ ÂÔËÎÂÔÒËËÚÂ, ͇ÚÓ Û‰Ó·ÂÌ, ·ÂÁÓÔ���ÒÂÌ, ËÍÓÌÓÏ˘ÂÌ Ë Ì‡È‚Â˜Â ÂÙÂÍÚË‚ÂÌ ÏÂÚÓ‰ ‰‡ Ò ‰ÂÏÓÌÒÚË‡Ú Ô‡ÚÓÙËÁËÓÎӄ˘ÌËÚ ÔÓfl‚Ë Ì‡ ‡·ÌÓÏ̇ ÍÓÚË͇Î̇ ‚˙Á·Û‰ËÏÓÒÚ, ÒÚÓfl˘Ë ‚ ÓÒÌÓ‚‡Ú‡ ̇ ÂÔËÎÂÔÒËflÚ‡. äÓ΢ÂÒÚ‚ÂÌËÚ ÖÖÉ ÏÂÚÓ‰Ë Á‡ ‡Ì‡ÎËÁ ‚Ò Ӣ ËÏ‡Ú Ò‡‚ÌËÚÂÎÌÓ Ó„‡Ì˘ÂÌÓ ÍÎËÌ˘ÌÓ ÔËÎÓÊÂÌË ‚ ӷ·ÒÚÚ‡ ̇ ÂÔËÎÂÔÚÓÎÓ„ËflÚ‡, ÌÓ ÔËÚÂʇ‚‡Ú ÌÂÓ·ıÓ‰ËÏËfl ÔÓÚÂ̈ˇΠ‰‡ ̇ÏÂflÚ ÏflÒÚÓ Í‡ÚÓ ‰ÓÔ˙ÎÌÂÌËÂ Ë ‡Á¯ËÂÌË ̇ ÛÚËÌ̇ڇ ÖÖÉ ‚ ‰ˈ‡ ÒÎÛ˜‡Ë (43):

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1. ä‡ÍÚÓ Â ËÁ‚ÂÒÚÌÓ, ‰Ë‡„ÌÓÁ‡Ú‡ ÂÔËÎÂÔÒËfl  Ô˙‚˘ÌÓ ÍÎËÌ˘̇. åÌÓ„Ó ˜ÂÒÚÓ Ó·‡˜Â ËÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ ÏÓÊ ‰‡ ·˙‰Â ÌÓχÎ̇. ç‡Î˘ËÂÚÓ Ì‡ ËÌÚÂËÍÚ‡Î̇ ‡ÍÚË‚ÌÓÒÚ ÔÓ‰ÂÔfl ‰Ë‡„ÌÓÁ‡Ú‡, ÌÓ ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ Úfl·‚‡ ‰‡ Ò Ëχ Ô‰‚ˉ ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ „ËÒÚË‡Ì ̇ ÂÔËÎÂÔÚËÙÓÏÂ̇ ‡ÍÚË‚ÌÓÒÚ ÔË 0.5% ÓÚ Á‰‡‚ËÚ ‚˙Á‡ÒÚÌË Ë̉˂ˉË, ‰Ó 5-8% ÓÚ ‰Âˆ‡Ú‡ Ë ‰Ó 2% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ‰Û„Ë Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl, ÌÓ ·ÂÁ ÍÎËÌ˘ÌË ‰‡ÌÌË Á‡ ÂÔËÎÂÔÒËfl. éÚ ‰Û„‡ ÒÚ‡Ì‡ ÎËÔÒ‡Ú‡ ̇ ÂÔËÎÂÔÚËÙÓÏÂ̇ ‡ÍÚË‚ÌÓÒÚ Ì ÓÚı‚˙Îfl ‰Ë‡„ÌÓÁ‡Ú‡, Ú˙È Í‡ÚÓ, ÒÔÓ‰ ÌflÍÓË ‡‚ÚÓË, Ò‡ÏÓ ÔË 50% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ‰Ó͇Á‡Ì‡ ÂÔËÎÂÔÒËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ڇ͇‚‡ ̇ Ô˙‚‡Ú‡ ÛÚËÌ̇ ÖÖÉ. èÓÒÚÓflÌÌÓ Ì„‡ÚË‚ÌË Ò‡ ÛÚËÌÌËÚ Á‡ÔËÒË ÔË 8% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ‰Ó͇Á‡Ì‡ ÍÎËÌ˘ÌÓ ‰Ë‡„ÌÓÁ‡ (2,5,20). èË·„‡ÈÍË ÍÓ΢ÂÒÚ‚ÂÌ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ, ÌflÍÓË ‡‚ÚÓË Ò ̇ÒÓ˜‚‡Ú Í˙Ï ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ‰ËÒÍÂÚÌË, ̉ÓÎÓ‚ËÏË ÔË ‚ËÁÛ‡ÎÌËfl ‡Ì‡ÎËÁ ‡ÒËÏÂÚËË, ÔË Ô‡ˆËÂÌÚË Ò ÌÓχÎÌË ËÌÚÂËÍÚ‡ÎÌË ÖÖÉ Ë ÛÒÚ‡ÌÓ‚fl‚‡Ú ÍÓ·ˆËfl Ò ÂÔËÎÂÔÚ˘ÌËfl ÙÓÍÛÒ. ê‡ÌÌË ÚÓÔÓ„‡ÙÒÍË ÔÓÛ˜‚‡ÌËfl Ò‡ ÔÓ͇Á‡ÎË Ì‡‡ÒÚ‚‡Ì ̇ ·˙Á‡Ú‡ Ë ·‡‚̇ڇ ‡ÍÚË‚ÌÓÒÚ ‚ „ËÓ̇ ̇ ÂÔËÎÂÔÚ˘ÌËfl ÙÓÍÛÒ Ë ÚÂÁË ÏÂÚÓ‰Ë Ò‡ ·ËÎË ÔÂÔÓ˙˜‚‡ÌË Á‡ ˉÂÌÚËÙˈË‡Ì ̇ ̉ÓÎÓ‚ËÏË Ì‡ ÛÚËÌ̇ڇ ÖÖÉ ÂÔËÎÂÔÚ˘ÌË ÙÓÍÛÒË, ͇ÍÚÓ Â Ì‡ÔËÏÂ ÔË ÂÔËÎÂÔÒËflÚ‡ ̇ ÙÓÌÚ‡ÎÌËfl ‰flÎ (16). Miyauchi et al. ÔÓ͇Á‚‡Ú „ÂÌÂ‡ÎËÁË‡ÌÓ Ì‡‡ÒÚ‚‡Ì ̇ ·‡‚̇ڇ ‡ÍÚË‚ÌÓÒÚ, ÍÓÂÚÓ Â ÍÓ΢ÂÒÚ‚ÂÌÓ ÔÓ-ËÁ‡ÁÂÌÓ ÔË Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, ͇ÍÚÓ Ë Ì‡Î˘Ë ̇ ‰ËÒÍÂÚÌË Î‡ÚÂ‡ÎËÁË‡ÌË ‡ÒËÏÂÚËË ‚ ‡ÎÙ‡2 Ë ·ÂÚ‡1 ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ ÒÚ‡Ì‡Ú‡ ̇ ÂÔËÎÂÔÚÓ„ÂÌÌËfl ÙÓÍÛÒ (41). Salinsky et al. Ô‡‚flÚ ËÁ‚Ó‰‡, ˜Â ÍÓ΢ÂÒÚ‚ÂÌËflÚ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ÏÓÊ ‰‡ ·˙‰Â ÔÓ‰˙ÎÊÂÌË ̇ ÍÓÌ‚Â̈ËÓ̇Î̇ڇ ÖÖÉ ÔË Ô‡ˆËÂÌÚË Ò ÌÓχÎ̇ ËÎË ÎÂÍÓ ‡·ÌÓÏ̇ ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ Á‡ ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ˆÂ·‡ÎÌË ÎÂÁËË (59). Tuunainen et al. ÔÓ͇Á‚‡Ú, ˜Â ÔË Ô‡ˆËÂÌÚËÚÂ Ò Ô‡ˆË‡ÎÂÌ ÔËÒÚ˙Ô ÔÓ-χÎÍÓ ÓÚ 20 ˜‡Ò‡ ÔÂ‰Ë ËÁÒΉ‚‡ÌÂÚÓ, ÏÓÊ ÍÓÂÍÚÌÓ ‰‡ Ò ·ÚÂ‡ÎËÁË‡ Ó„ÌˢÂÚÓ ˜ÂÁ ÔÓ-„ÓÎflÏÓÚÓ ÍÓ΢ÂÒÚ‚Ó ‡·ÒÓβÚ̇ ‰ÂÎÚ‡ ‡ÍÚË‚ÌÓÒÚ ‚ ÂÔËÎÂÔÚÓ„ÂÌÌËfl ÙÓÍÛÒ. Ä‚ÚÓËÚ ‚ ‰ÓÔ˙ÎÌÂÌË ‰ÂÏÓÌÒÚË‡Ú, ˜Â ÂÔËÎÂÔÚ˘ÌËÚ ÔËÒÚ˙ÔË Ò ÔˉÛʇ‚‡Ú ÓÚ ‰ËÒÍÂÚÌÓ ÙÓ͇ÎÌÓ Á‡·‡‚flÌ Á‡ ÏÌÓ„Ó ÔÓ-‰˙Î˙„ ÔÂËÓ‰ ÓÚ ‚ÂÏÂ, ÓÚÍÓÎÍÓÚÓ ÏÓÊ ‰‡ Ò ÛÒÚ‡ÌÓ‚Ë Ì‡ ÛÚËÌ̇ڇ ÖÖÉ (65). 2. èË Ì‡Î˘ËÂÚÓ Ì‡ ÂÔËÎÂÔÚËÙÓÏÂ̇ ‡ÍÚË‚ÌÓÒÚ, ÍÓ΢ÂÒÚ‚ÂÌËÚ ÖÖÉ ÏÂÚÓ‰Ë ÏÓ„‡Ú ‰‡ ‰ÓÔËÌÂÒ‡Ú ‰ÓÔ˙ÎÌËÚÂÎÌÓ Á‡ ÎÓ͇ÎËÁ‡ˆËflÚ‡ ̇ ÂÔËÎÂÔÚÓ„ÂÌÌËfl ÙÓÍÛÒ. ÑËÔÓÎÌËflÚ ‡Ì‡ÎËÁ ÔÓÁ‚ÓÎfl‚‡ ‰‡ Ò ÓÔ‰ÂÎË Ò˙Ò Á̇˜ËÚÂÎ̇ ÚÓ˜ÌÓÒÚ ÍÓÚË͇Î̇ڇ ÎÓ͇ÎËÁ‡ˆËfl ̇ ËÌÚÂËÍÚ‡Î̇ڇ Ë ËÍÚ‡Î̇ڇ ‡ÍÚË‚ÌÓÒÚ, ÔÓÒÓ͇ڇ ̇ ÔÓÔ‡„‡ˆËfl Ë Ò˙˘ÂÒÚ‚Û‚‡ÌÂÚÓ Ì‡ ÏÌÓÊÂÒÚ‚Ó ÓÚ‰ÂÎÌË ÒÔ‡ÈÍÓ‚Ë „ÂÌÂ‡ÚÓË. ÑËÔÓÎÌËflÚ ‡Ì‡ÎËÁ ÏÓÊ ‰‡ ·˙‰Â ÓÒÓ·ÂÌÓ ÔÓÎÂÁÂÌ ÔË Ô‡ˆËÂÌÚË, ÔÓ‰ÎÂʇ˘Ë ̇ ÓˆÂÌ͇ Á‡ ıËÛ„˘̇ ÚÂ‡ÔËfl ̇ ÂÔËÎÂÔÒËflÚ‡, ÔË ·ÂÌË„ÌÂ̇ڇ Ó·̉ӂ‡ ÂÔËÎÂÔÒËfl Á‡ ÓÚ‰ËÙÂÂ̈Ë‡Ì ̇ ÚËÔ˘ÌËÚ ÓÚ ‡ÚËÔ˘ÌËÚ ÙÓÏË Ë ÍÓÏÔÎÂÍÒÌËÚ ԇˆË‡ÎÌË ÔËÒÚ˙ÔË, ÍÓÂÚÓ Ëχ ÔÓ„ÌÓÒÚ˘̇ Ë ÚÂ‡Ô‚Ú˘̇ Á̇˜ËÏÓÒÚ (43,61). 3. Ç˙ÔÂÍË, ˜Â ÛÚËÌ̇ڇ ÖÖÉ ÓÒÚ‡‚‡ Á·ÚÌËflÚ Òڇ̉‡Ú Á‡ ÔÓÚ‚˙ʉ‡‚‡Ì ̇ ‰Ë‡„ÌÓÁ‡Ú‡ ÂÔËÎÂÔÒËfl, „ËÒÚË‡ÌÂÚÓ Ì‡ ÂÔËÎÂÔÚ˘ÂÌ ÔËÒÚ˙Ô ÔÓ ‚ÂÏ ̇ ‰ËÌ ÛÚËÌÂÌ Á‡ÔËÒ Â fl‰ÍÓ. ÑÓË ÒÂËÈÌË ÖÖÉ ÏÓ„‡Ú ‰‡ ÔÓÔÛÒÌ‡Ú ÂÔËÎÂÔÚËÙÓÏÂ̇ ‡ÍÚË‚ÌÓÒÚ ÔË ÓÍÓÎÓ 10% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ÍÎËÌ˘ÌÓ ‰Ó͇Á‡Ì‡ ‰Ë‡„ÌÓÁ‡ ÂÔËÎÂÔÒËfl. äÓ„‡ÚÓ ı‡‡ÍÚÂ˙Ú Ì‡ ÔËÒÚ˙ÔËÚ ËÎË ÚÓ˜ÌËflÚ ÂÔËÎÂÔÚ˘ÂÌ ÙÓÍÛÒ Ì ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ËÁflÒÌÂÌË Ò ÌflÍÓÎÍÓ ÔÓÒΉӂ‡ÚÂÎÌË ÛÚËÌÌË Á‡ÔËÒ‡, ÔÓ‰˙ÎÊËÚÂÎÌÓÚÓ ÖÖÉ ÏÓÌËÚÓË‡Ì ‚ ‡Ï·Û·ÚÓÌË ËÎË ÍÎËÌ˘ÌË ÛÒÎÓ‚Ëfl, ÓÒÓ-


‰ÂÍÂÏ‚Ë, 2007

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

·ÂÌÓ ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ‚ˉÂÓ-ÏÓÌËÚÓË‡ÌÂ Ë ‡‚ÚÓχÚ˘ÂÌ ‡Ì‡ÎËÁ ̇ Ò˙·ËÚËfl, ÏÓÊ ‰‡ ÓÒË„ÛË ÌÂÓ·ıÓ‰Ëχڇ ‰ÓÔ˙ÎÌËÚÂÎ̇ ËÌÙÓχˆËfl (5,20,68) 4. ÑÛ„Ë ÍÓ΢ÂÒÚ‚ÂÌË ÖÖÉ ÏÂÚÓ‰Ë Í‡ÚÓ ÍÓıÂÂÌÚÌËfl ‡Ì‡ÎËÁ ÏÓ„‡Ú ‰‡ ÔÓÏÓ„Ì‡Ú Á‡ ‡Á„‡Ì˘‡‚‡ÌÂÚÓ Ì‡ Ô˙‚˘ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ‡Áfl‰Ë ÓÚ ‚ÚÓ˘ÌÓ ·Ë·ÚÂ‡Î̇ ÒËÌıÓÌÌÓÒÚ, ˜ÂÁ ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ χÎÍË, ÂÔÓ‰ÛˆËÛÂÏË ËÌÚÂıÂÏËÒÙÂÌË ‡ÁÎËÍË ‚˙‚ ‚ÂÏÂÚÓ Ì‡ ‡Áfl‰ËÚÂ Ë ‡ÁÔ‰ÂÎÂÌËÂÚÓ Ì‡ χÍÒËχÎ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ë ÔÓ Ú‡Í˙‚ ̇˜ËÌ ‰‡ ̇ÒÓ˜Ë ËÁ·Ó‡ ̇ ‡ÌÚËÂÔËÎÂÔÚ˘ÂÌ Ï‰Ë͇ÏÂÌÚ ËÎË ‰‡ ÔÓ‰ÔÓÏÓ„Ì ÔÂıËÛ„˘̇ڇ ÎÓ͇ÎËÁ‡ˆËfl (43,68). äéãàóÖëíÇÖç ëèÖäíêÄãÖç ÄçÄãàá çÄ éëçéÇçÄíÄ ÄäíàÇçéëí èêà Åéãçà ë ÖèàãÖèëàü Clemens et al. (2006), ͇ÍÚÓ Ë ‰Û„Ë ‡‚ÚÓË, ̇ÏË‡Ú, ˜Â ‚ ‰ÂÈÒÚ‚ËÚÂÎÌÓÒÚ ÍÓ΢ÂÒÚ‚ÂÌ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÔË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl  ·ËÎ ÔÓ‚Âʉ‡Ì Ò‡ÏÓ ÓÚ Ó„‡Ì˘ÂÌ ·ÓÈ ËÁÒΉӂ‡ÚÂÎË, ͇ÚÓ Ôӂ˜ÂÚÓ ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡ Ò‡ ·ËÎË Ì‡ÒÓ˜ÂÌË Í˙Ï ÎÓ͇ÎËÁË‡Ì ̇ „ËÓ̇Î̇ڇ ÍÓÚË͇Î̇ ‰ËÒÙÛÌ͈Ëfl ÔË Ô‡ˆËÂÌÚËÚÂ Ò ÙÓ͇Î̇ ÂÔËÎÂÔÒËfl ËÎË Í˙Ï ÓˆÂÌ͇ ̇ ÂÙÂÍÚ‡ ̇ ‡ÌÚËÂÔËÎÂÔÚ˘ÌËÚ ÎÂ͇ÒÚ‚‡ ‚˙ıÛ Ô‡ÓÍÒËÁχÎ̇ڇ ‡ÍÚË‚ÌÓÒÚ (11). GÂlety et al. (1985), ‰ÂÏÓÌÒÚË‡Ú ÒË„ÌËÙË͇ÌÚÌÓ Á‡·‡‚flÌ ̇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡Ú‡ ÔË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl Ë ÌÓχÎ̇ ËÌÚÂËÍÚ‡Î̇ ÖÖÉ (·ÂÁ ‰‡ÌÌË Á‡ Â̈ÂÙ‡ÎÓÔ‡ÚËfl, ˜ÂÒÚË ÔËÒÚ˙ÔË, ÏÓÁ˙˜ÌË ÎÂÁËË ËÎË ÎÂ͇ÒÚ‚Â̇ ËÌÚÓÍÒË͇ˆËfl) ÒÔflÏÓ Á‰‡‚Ë ÍÓÌÚÓÎË, ͇ÚÓ ÚÂÁË ÔÓÏÂÌË Ò‡ ·ËÎË Á‡·ÂÎÂÊËÏË Ò‡ÏÓ Ì‡ ÒÔÂÍÚ‡ÎÌËfl ‡Ì‡ÎËÁ, ÌÓ ÌÂ Ë Ì‡ ÛÚËÌ̇ڇ ÖÖÉ. Ä‚ÚÓËÚ Á‡Íβ˜‡‚‡Ú, ˜Â ËÌÚÂËÍÚ‡ÎÌËÚ ÔÓÏÂÌË ‚ ÏÓÁ˙˜Ì‡Ú‡ ÙÛÌ͈Ëfl, ÍÓËÚÓ Ì Ò ‡ÁÍË‚‡Ú ÓÚ Òڇ̉‡Ú̇ڇ ÖÖÉ, ÏÓ„‡Ú ‰‡ Ò‡ Ò‚˙Á‡ÌË Ò ÔÓÏÂÌËÚ ‚ ËÌÚÂËÍÚ‡ÎÌÓÚÓ Ôӂ‰ÂÌËÂ Ë ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË Ë ˜Â ÍÓ΢ÂÒÚ‚ÂÌËflÚ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ÏÓÊ ‰‡ Ëχ ÒÚÓÈÌÓÒÚ ÔË ÓˆÂÌ͇ڇ ̇ Ô‡ˆËÂÌÚËÚÂ Ò ÂÔËÎÂÔÒËfl ÔÂ‰Ë Ë ÔÓ ‚ÂÏ ̇ ΘÂÌË (23). Drake et al. (1990, 1998), ‡ÁÍË‚‡Ú ÔÓÏÂÌË ‚ ÒÔÂÍÚ‡ÎÌËÚ ÔÓ͇Á‡ÚÂÎË ÔË Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË ÓÚ ÚÂÏÔÓ‡ÎÌËfl Ë ÙÓÌÚ‡ÎÌËfl ‰flÎ Ë ÌÓχÎÌË ËÌÚÂËÍÚ‡ÎÌË ÖÖÉ, ÔÓ‚Âʉ‡˘Ë ÏÓÌÓÚÂ‡ÔËfl Ò ‡Á΢ÌË ‡ÌÚËÂÔËÎÂÔÚ˘ÌË ÎÂ͇ÒÚ‚‡ - ÙÂÌÓ·‡·ËÚ‡Î, ÙÂÌËÚÓËÌ, ͇·‡Ï‡ÁÂÔËÌ Ë ‚‡ÎÔÓ‡Ú. Ä‚ÚÓËÚ ‰ÂÏÓÌÒÚË‡Ú, ˜Â ‰ÓË Ë ÔË Ô‡ˆËÂÌÚËÚÂ Ò ÌÓχÎ̇ ËÌÚÂËÍÚ‡Î̇ ÖÖÉ, ÔÓÒ‰ÒÚ‚ÓÏ ÍÓ΢ÂÒÚ‚ÂÌ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ÏÓ„‡Ú ‰‡ Ò ÛÒÚ‡ÌÓ‚flÚ ÔÓÏÂÌË Í‡ÚÓ Ì‡Ï‡ÎÂÌÓ Ò˙ÓÚÌÓ¯ÂÌË ̇ ÏÓ˘ÌÓÒÚÚ‡ ÏÂÊ‰Û ·˙ÁËfl Ë ·‡‚ÌËfl ‰Ë‡Ô‡ÁÓÌ, ÔÓ-„ÓÎflχ ‡ÒËÏÂÚËfl ̇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡Ú‡ Ë Ì‡Ï‡ÎÂ̇ ÒÔÂÍÚ‡Î̇ ÏÓ·ËÎÌÓÒÚ ÒÔflÏÓ ÍÓÌÚÓÎËÚ ̇ ÒÚ‡Ì‡Ú‡ ̇ ÂÔËÎÂÔÚÓ„ÂÌÌËfl ÙÓÍÛÒ. Ä‚ÚÓËÚ ÔÓ‰˜ÂÚ‡‚‡Ú, ˜Â ËÁÏÂ‚‡ÌÂÚÓ Ì‡ ‡ÏÔÎËÚÛ‰ÌËÚÂ Ë ˜ÂÒÚÓÚÌËÚ ÔÓ͇Á‡ÚÂÎË Ë Ì‡ ÒÔÂÍÚ‡ÎÌË ı‡‡ÍÚÂËÒÚËÍË Í‡ÚÓ ÏÓ·ËÎÌÓÒÚ, ÏÓÊ ‰‡ ‰ÂÏÓÌÒÚË‡ ̉ÓÎÓ‚ËÏË ÔÓ ‰Û„ ̇˜ËÌ ‡ÒËÏÂÚËË ‚ ËÌÚÂËÍÚ‡Î̇ڇ ÖÖÉ Ë ÔÓ Ú‡Í˙‚ ̇˜ËÌ ‰‡ ÒÔÓÏÓ„Ì ‚ ‰Ë‡„ÌÓÁ‡Ú‡ Ë Í·ÒËÙË͇ˆËflÚ‡ ̇ ÂÔËÎÂÔÒËflÚ‡ (16,17). Clemens et al. (2000), ‚˙Á ÓÒÌÓ‚‡ ÍÓ΢ÂÒÚ‚ÂÌ ‡Ì‡ÎËÁ ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÛÒÚ‡ÌÓ‚fl‚‡Ú ÏÌÓ„Ó ı‡‡ÍÚÂÂÌ ÒÔÂÍÚ‡ÎÂÌ ÔÓÙËΠ̇ ˉËÓÔ‡Ú˘̇ڇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËfl, Ô‰ÒÚ‡‚fl˘ ÒÂ Ò ÙÓÌÚ‡ÎÌÓ Ì‡‡ÒÚ‚‡Ì ̇ ‰ÂÎÚ‡ Ë ‰ËÙÛÁÌÓ Ì‡‡ÒÚ‚‡Ì ̇ ÚÂÚ‡ ‡ÍÚË‚ÌÓÒÚÚ‡, ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ‰ËÙÛÁÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ ·ÂÚ‡ ‡ÍÚË‚ÌÓÒÚÚ‡ Ò ÙÓÌÚ‡ÎÂÌ Ï‡ÍÒËÏÛÏ. Ä‚ÚÓËÚ ËÁ͇Á‚‡Ú ıËÔÓÚÂÁ‡Ú‡, ˜Â ÚÓÁË ÒÔÂÍÚ‡ÎÂÌ ÔÓÙËÎ Ô‰ÒÚ‡‚Îfl‚‡ ÓÚ‡ÊÂÌË ̇ ‡ÁÔÓÒÚ‡ÌÂ̇ڇ ÍÓÚË͇Î̇ ‰ËÒÙÛÌ͈Ëfl, ÍÓflÚÓ ÔÓ Ò˙˘ÂÒÚ‚Ó Â Ó·˘‡ Á‡ ‚Ò˘ÍË ËÁÒΉ‚‡ÌË ÒË̉ÓÏË Ì‡ ˉËÓÔ‡-

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Ú˘̇ „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËfl (13). ÉÂÌÂ‡ÎËÁË‡ÌÓÚÓ Ì‡‡ÒÚ‚‡Ì ̇ ·‡‚ÌÓ‚˙ÎÌÓ‚‡Ú‡ ‡ÍÚË‚ÌÓÒÚ Â Â‰Ì‡ ÓÚ Ì‡È-ÌÂÒÔˆËÙ˘ÌËÚÂ Ë Ì‡È-˜ÂÒÚÓ Ò¢‡ÌËÚ ̇ıÓ‰ÍË ‚ Â̈ÂÙ‡ÎÓ„‡Ï‡Ú‡. ç„ӂ‡Ú‡ ÍÓ΢ÂÒÚ‚Â̇ڇ ËÁ‡ÁÂÌÓÒÚ, Ó·‡˜Â, ÍÓÂÎË‡ Ò˙Ò ÒÚÂÔÂÌÚ‡ ̇ ÏÓÁ˙˜Ì‡Ú‡ ‰ËÒÙÛÌ͈Ëfl (1). Salynski et al. (2003), ‰ÂÏÓÌÒÚË‡Ú, ˜Â Òڇ̉‡Ú̇ڇ ÄÖå ÚÂ‡ÔËfl ÏÓÊ ‰‡ ·˙‰Â Ò‚˙Á‡Ì‡ Ò˙Ò ÒË„ÌËÙË͇ÌÚÌÓ Ì‡‡ÒÚ‚‡Ì ̇ ·‡‚ÌÓ‚˙ÎÌÓ‚‡Ú‡ ‡ÍÚË‚ÌÓÒÚ ÔË ÎËÔÒ‡Ú‡ ̇ ËÁfl‚Â̇ ÍÎËÌ˘ÌÓ Ì‚ÓÚÓÍÒ˘ÌÓÒÚ Ë ˜Â ÚÂÁË ÔÓÏÂÌË Ó·˘Ó ÍÓÂÎË‡Ú Ò ÔÓÏÂÌËÚ ‚ ÍÓ„ÌËÚË‚ÌËÚ ÔÓ͇Á‡ÚÂÎË Ë ÒÛ·ÂÍÚ˂̇ڇ ÓˆÂÌ͇ ̇ Ò˙ÒÚÓflÌËÂÚÓ. Ä‚ÚÓËÚ ÔÓ‚Âʉ‡Ú ÍÓ΢ÂÒÚ‚ÂÌ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÔË ·ÓÎÌË Ò ÂÔËÎÂÔÒËfl Ô‰Ë, ÔÓ ‚ÂÏÂ Ë ÒΉ ÒÔË‡Ì ̇ ‡ÌÚËÂÔËÎÂÔÚ˘̇ ÏÓÌÓÚÂ‡ÔËfl, ͇ÚÓ Ò‡‚Ìfl‚‡Ú ‰‡ÌÌËÚ Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË Ë Ò Ô‡ˆËÂÌÚË ·ÂÁ ÚÂ‡ÔËfl , ͇ÍÚÓ Ë Ò ‰‡ÌÌËÚ ÓÚ ÍÓ„ÌËÚË‚ÌËÚ ÚÂÒÚÓ‚Â. Ä‚ÚÓËÚ ‰Ó͇Á‚‡Ú, ˜Â Ô‡ˆËÂÌÚËÚÂ, ÔËÂχ˘Ë ÄÖå, ÏÓ„‡Ú ‰‡ ÔÓÎÛ˜‡Ú ÎÂ͇ÒÚ‚ÂÌÓ Ó·ÛÒÎÓ‚Â̇ ‰ËÒÙÛÌ͈Ëfl, ÍÓflÚÓ Ì  ÍÎËÌ˘ÌÓ ËÁfl‚Â̇ Ë ˜Â ÍÓ΢ÂÒÚ‚ÂÌËÚ ÔÓ͇Á‡ÚÂÎË Ì‡ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ·Ëı‡ ÏÓ„ÎË ‰‡ ·˙‰‡Ú ÔÓÎÂÁÂÌ ÏÂÚÓ‰ Á‡ Ë̉˂ˉۇÎÌÓ ÏÓÌËÚÓË‡Ì (56). Diaz et al. (1998), ÔË Ô‡ˆËÂÌÚË Ò ÙÓ͇Î̇ ÒËÏÔÚÓχÚ˘̇ ÂÔËÎÂÔÒËfl ÓÚ Ù��ÌÚ‡ÎÌËfl ËÎË ÚÂÏÔÓ‡ÎÌËfl ‰flÎ Ë ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰ËÙÛÁÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ ÏÓ˘ÌÓÒÚÚ‡ ‚ ‡ÎÙ‡ ‰Ë‡Ô‡ÁÓ̇, Ò‰̇ڇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡ Ë Ì‡‡ÒÚ‚‡ÌÂÚÓ ÏÓ˘ÌÓÒÚÚ‡ ‚ ÚÂÚ‡ ‰Ë‡Ô‡ÁÓ̇, ͇ÚÓ Ì ÛÒÚ‡ÌÓ‚fl‚‡Ú ÍÓ·ˆËfl ̇ ÚÂÁË ÔÓÏÂÌË Ò ÂÔËÎÂÔÚ˘ÌËfl ÒË̉ÓÏ Ë Ò ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓÚÓ Î˜ÂÌËÂ, ÌÓ Â ËÌÚÂÂÒÌÓ, ˜Â ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔÓÁËÚ˂̇ ÍÓ·ˆËfl ÏÂÊ‰Û ÒÚÂÔÂÌÚ‡ ̇ ÍÓ΢ÂÒÚ‚ÂÌËÚ ÖÖÉ ÔÓÏÂÌË Ë ÍÓ΢ÂÒÚ‚ÓÚÓ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÛÚËÌ̇ڇ ‰Ë„ËÚ‡Î̇ ÖÖÉ, ͇ÚÓ Ô‡‚flÚ Á‡Íβ˜ÂÌËÂÚÓ, ˜Â ÙÓ͇Î̇ڇ ÂÔËÎÂÔÚÓ„ÂÌÂÁ‡ ‚ÂÓflÚÌÓ Ëχ „ÂÌÂ‡ÎËÁË‡ÌÓ ÓÚ‡ÊÂÌË ‚˙ıÛ ÒÔÂÍÚ‡Î̇ڇ ÍÓÏÔÓÁˈËfl ̇ ÖÖÉ (15). éÔËÚ˙Ú ÔÓ͇Á‚‡, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò ÂÔËÎÂÔÒËfl ËÏ‡Ú ÔÓ-ÌËÒ͇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡ ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË. íÓ‚‡ ÓÚ˜‡ÒÚË Ò ÓÚ‰‡‚‡ ̇ ÔÓ‰ÎÂʇ˘‡Ú‡ ÏÓÁ˙˜Ì‡ Ô‡ÚÓÎÓ„Ëfl, ̇ Ò‡ÏËfl ÂÔËÎÂÔÚ˘ÂÌ ÔÓˆÂÒ, ͇ÍÚÓ Ë Ì‡ Ù‡ÍÚ‡, ˜Â ÌflÍÓË ÄÖå ÏÓ„‡Ú ‰‡ Ô‰ËÁ‚ËÍ‡Ú Á‡·‡‚flÌ ̇ ‡ÎÙ‡ ‡ÍÚË‚ÌÓÒÚÚ‡ (15,21,57,58,65). Clemens Ë Bassenyei (2003), ÔË ÌÂÎÂÍÛ‚‡ÌË ÌӂӉˇ„ÌÓÒÚˈË‡ÌË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl ÒÔflÏÓ Á‰‡‚Ë ÍÓÌÚÓÎË ÛÒÚ‡ÌÓ‚fl‚‡Ú ̇χÎÂ̇ ‡·ÒÓβÚ̇ Ë ÓÚÌÓÒËÚÂÎ̇ ÏÓ˘ÌÓÒÚ Ì‡ ‡ÎÙ‡ ‰Ë‡Ô‡ÁÓ̇, ÌÓ ÌÂ Ë ‡ÁÎË͇ ‚ Ò‰̇ڇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡ (12). ç‡‡ÒÚ‚‡Ì ‚ Ó͈ËÔËÚ‡Î̇ڇ ÔËÍÓ‚‡ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡  ·ËÎÓ Ì‡·Î˛‰‡‚‡ÌÓ ÒΉ ÒÔË‡Ì ̇ ‡ÌÚËÂÔËÎÂÔÚ˘ÌË ÎÂ͇ÒÚ‚‡, ÔÓ‰ÍÂÔfl˘Ó Ù‡ÍÚ‡, ˜Â e ‚˙ÁÏÓÊÌÓ Ì‡Ï‡Îfl‚‡ÌÂÚÓ Ì‡ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡Ú‡ ‰‡ Ò ‰˙ÎÊË Ì‡ ωË͇ÏÂÌÚÓÁÌÓ ‚˙Á‰ÂÈÒÚ‚Ë (18,56,58,65). êÂÁÛÎÚ‡ÚËÚ ̇ Larson Ë Kostov (2005), ÔÓ͇Á‚‡Ú Ò˙˘Ó ÒË„ÌËÙË͇ÌÚÌÓ ÔÓ-ÌËÒ͇ ÔËÍÓ‚‡ ˜ÂÒÚÓÚ‡ ̇ ‡ÎÙ‡-ËÚ˙χ ÔË ·ÓÎÌËÚÂ Ò ÂÔËÎÂÔÒËfl (37). àÌÚÂÂÒ˙Ú Í˙Ï ˜ÂÒÚÓÚÌËÚ ı‡‡ÍÚÂËÒÚËÍË Ì‡ ‡ÎÙ‡ ‰Ë‡Ô‡ÁÓ̇ ̇‡ÒÚ‚‡, Ú˙È Í‡ÚÓ ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ ‚Ò Ôӂ˜ ‡‚ÚÓË ÔÓÚ‚˙ʉ‡‚‡Ú ‚˙Á͇ڇ ÏÂÊ‰Û ÒÚÂÔÂÌÚ‡ ̇ Á‡·‡‚flÌ ̇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡Ú‡ Ë Ì‡Î˘ËÂÚÓ Ë ÒÚÂÔÂÌÚ‡ ̇ ÍÓ„ÌËÚ˂̇ ‰ËÒÙÛÌ͈Ëfl, ‡ ÓÚ ‰Û„‡ ÒÚ‡Ì‡ Ú ҇ ‰ÌË ÓÚ Ì‡È-ÒÚ‡·ËÎÌËÚ ÔÓ͇Á‡ÚÂÎË, ÍÓËÚÓ ÎÂÒÌÓ Ò ËÁÏÂ‚‡Ú. òËÓÍÓ ˆËÚË‡ÌÓ Â ÔÓÛ˜‚‡ÌÂÚÓ Ì‡ Frost JD et al.(1995), ‚ ÍÓÂÚÓ ‡‚ÚÓËÚ ‰Ó͇Á‚‡Ú, ˜Â ÔÓÌËʇ‚‡ÌÂÚÓ Ì‡ Ó͈ËÔËÚ‡Î̇ڇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡ Ò Ì‡‰ 0.5 çz Ӣ ‚ ̇˜‡ÎÓÚÓ Ì‡ ΘÂÌËÂÚÓ Â Ò‚˙Á‡ÌÓ Ò ÔÓ-ÎÓ¯Ë ÂÁÛÎÚ‡ÚË Ì‡ Wechsler Intelligence Scale for Children-Revised (WISC-R) ‰̇ „Ó‰Ë̇ ÔÓ-Í˙ÒÌÓ. Ä‚ÚÓËÚ ‰Ó͇Á‚‡Ú, ˜Â ÍÓ΢ÂÒÚ‚ÂÌËflÚ ‡Ì‡ÎËÁ ÏÓÊ ‰‡ Ò ËÁÔÓÎÁ‚‡ Á‡ ˉÂÌÚËÙˈË‡ÌÂÚÓ Ì‡ Ë̉˂ˉË, ÍÓËÚÓ Ò‡ Ò Ôӂ˯ÂÌ ËÒÍ ÓÚ ‡Á‚ËÚË ̇ Ò‚˙Á‡ÌË Ò ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚÌÓÚÓ Î˜ÂÌË ‰˙΄ÓÚ‡ÈÌË ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl (21). ᇷ‡‚flÌÂÚÓ Ì‡ ‡ÎÙ‡ ˜ÂÒÚÓ-


‰ÂÍÂÏ‚Ë, 2007

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

Ú‡Ú‡ ÏÓÊ ‰‡ ·˙‰Â ̇È-‡ÌÂÌ ÌÂÒÔˆËÙ˘ÂÌ ·Â΄ ̇ „ÂÌÂ‡ÎËÁË‡ÌÓ Ì‡Û¯ÂÌË ̇ ÏÓÁ˙˜Ì‡Ú‡ ÙÛÌ͈Ëfl, ÔÂ‰Ë ÔÓfl‚‡Ú‡ ̇ ‰Û„Ë ÔÓ-ÒÔˆËÙ˘ÌË ‡·ÌÓÏÌÓÒÚË. àÌÚÂıÂÏËÒÙÂÌË ‡ÁÎËÍË, ÔÓ-χÎÍË ÓÚ 1Hz Ò Û·‚flÚ ÚÛ‰ÌÓ ÓÚ ‚ËÁÛ‡ÎÌËfl ‡Ì‡ÎËÁ (1,2,19). ÄÍÓ Ò ̇Ô‡‚Ë ‡Ì‡ÎÓ„Ëfl Ò ˆËÚË‡Ì‡Ú‡ ÔÓ-„Ó ‚˙Á͇ ÏÂÊ‰Û ÍÓ„ÌËÚ˂̇ڇ ÓˆÂÌ͇ Ë ÒÌËʇ‚‡ÌÂÚÓ Ò 0.5Hz ̇ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡Ú‡, ÚÓ‚‡ ÔÓ͇Á‚‡, ˜Â Ò ÔÓÏÓ˘Ú‡ ̇ ÒÔÂÍÚ‡ÎÌËfl ‡Ì‡ÎËÁ, Ó˘Â Ì‡ ÒÛ·ÍÎËÌ˘ÌÓ ÌË‚Ó ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ‰ÓÎÓ‚ÂÌË ÔÓfl‚Ë Ì‡ Ì‚ÓÚÓÍÒ˘ÌÓÒÚ, ÔÂ‰Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÍÎËÌ˘̇ ÒËÏÔÚÓχÚË͇. ÑÛ„Ë ‡‚ÚÓË ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â Á‡·‡‚flÌÂÚÓ Ì‡ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡Ú‡ Ò Ôӂ˜ ÓÚ 0.5Hz Ô‰ÒÚ‡‚Îfl‚‡ ̇‰ÂʉÂÌ ·ËÓχÍÂ Á‡ ÍÓ„ÌËÚË‚ÌÓ Û‚Âʉ‡ÌÂ, ͇ÚÓ ÔÓ‰˜ÂÚ‡‚‡Ú, ˜Â ÍÎËÌ˘̇ڇ Á̇˜ËÏÓÒÚ Ì‡ Ú‡ÁË „‡Ìˈ‡ Ò Ò˙ÒÚÓË ‚ ÚÓ‚‡, ˜Â ̇χÎfl‚‡ÌÂÚÓ Ì‡ ‡ÎÙ‡-˜ÂÒÚÓÚ‡Ú‡ Ò Ôӂ˜ ÓÚ 0.5-0.6Hz ÒË„ÛÌÓ ÔÓ„ÌÓÁË‡ ̇ÒÚ˙Ô‚‡ÌÂÚÓ Ì‡ ÍÓ„ÌËÚË‚ÌÓ Ì‡Û¯ÂÌËÂ, ͇ÍÚÓ ÔË Á‰‡‚Ë Ë̉˂ˉË, ڇ͇ Ë ÔË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËfl. ë‡‚Ìfl‚‡ÈÍË Ì‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡ÌÂ Ë ÍÓ΢ÂÒÚ‚ÂÌËfl ÖÖÉ ‡Ì‡ÎËÁ, ÚÂ

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·„‡˘ÓÚÓ ÏÛ Ò ÏflÒÚÓ ‚ ÍÎËÌ˘̇ڇ Ô‡ÍÚË͇, ÌÓ ‚˙ÔÂÍË ÚÓ‚‡ ۷‰ËÚÂÎÌÓ Ì‡ÒÓ˜‚‡Ú Í˙Ï ‚˙ÁÏÓÊÌÓÚÓ ÏÛ ÛÒÔ¯ÌÓ ÔËÎÓÊÂÌË ‚ ӷ·ÒÚÚ‡ ̇ ÂÔËÎÂÔÚÓÎÓ„ËflÚ‡ Á‡ ÛÒÚ‡ÌÓ‚fl‚‡ÌÂ Ë ÔÓÒΉfl‚‡Ì ̇ ÔÓÏÂÌËÚ ‚ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÔË ·ÓÎÌËÚÂ Ò ÂÔËÎÂÔÒËfl, Á‡ ÏÓÌËÚÓË‡Ì ̇ ˆÂÌÚ‡ÎÌËÚ ÂÙÂÍÚË ÓÚ ‡ÌÚËÂÔËÎÂÔÚ˘ÌÓÚÓ Î˜ÂÌËÂ Ë ‡Ì̇ ‰ÂÚÂ͈Ëfl Ë ÔÓ„ÌÓÁË‡Ì ̇ Ì‚ÓÚÓÍÒ˘ÌË ÔÓfl‚Ë, ‰ÓË Ì‡ ÒÛ·ÍÎËÌ˘ÌÓ ÌË‚Ó, ‡ ڇ͇ Ò˙˘Ó Ë Í‡ÚÓ ‰ÓÔ˙ÎÌËÚÂÎÂÌ ÏÂÚÓ‰ ‚ ÍÎËÌ˘̇ڇ ÓˆÂÌ͇ ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË ÔË ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl ̇ ñçë. íÓ‚‡  ‚ Ò˙ÓÚ‚ÂÚÒÚ‚Ë Ò˙Ò ÒÚ‡ÌӂˢÂÚÓ Ì‡ ‰ˈ‡ ‡‚ÚÓË, ‡·ÓÚÂ˘Ë ËÁ‚˙Ì ÒÙÂ‡Ú‡ ̇ ÂÔËÎÂÔÚÓÎÓ„ËflÚ‡, ÍÓËÚÓ ÍÓÏÂÌÚË‡Ú, ˜Â „ÂÌÂÚ˘̇ ‰ÂÚÂÏËÌË‡ÌÓÒÚ Ì‡ ÖÖÉ ËÚÏËÚÂ Ë Á‡·ÂÎÂÊËÚÂÎ̇ڇ ËÌÚÂ- Ë ËÌÚ‡Ë̉˂ˉۇÎ̇ ÒÚ‡·ËÎÌÓÒÚ Ì‡ ÍÓ΢ÂÒÚ‚ÂÌËÚ ԇ‡ÏÂÚË ‚ ˜ÂÒÚÓÚÌËfl ‰ÓÏÂÈÌ Ó·ÓÒÌÓ‚‡‚‡Ú ÔËÎÓÊÂÌËÂÚÓ Ì‡ ÒÔÂÍÚ‡ÎÌËfl ÖÖÉ ‡Ì‡ÎËÁ ͇ÚÓ ÔÓÎÂÁÂÌ Ë ‰ÓÒÚÓ‚ÂÂÌ ÏÂÚÓ‰ Á‡ ‰ÂÚÂ͈Ëfl Ë ı‡‡ÍÚÂËÁË‡Ì ̇ ‰ËÒÍÂÚ̇ ÏÓÁ˙˜Ì‡ ‰ËÒÙÛÌ͈Ëfl, ÍÓÈÚÓ Úfl·‚‡ ‰‡ ·˙‰Â ÔË·„‡Ì ‚ ËÁÛ˜‡‚‡-

퇷Î.1 èÒËıÓÙ‡χÍÓÎӄ˘ÌË Í·ÒÓ‚Â ÒÔÓ‰ ÒÔÂÍÚ‡ÎÌËfl ÔÓÙËÎ ‰ÓË ‰Ó͇Á‚‡Ú Ô‚˙ÁıÓ‰ÒÚ‚ÓÚÓ Ì‡ ÔÓÒΉÌËfl ÏÂÚÓ‰ (56,57,58). ê‡ÁÔÓÁ̇‚‡ÌÂÚÓ Ì‡ ÍÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl ÔÓÒ‰ÒÚ‚ÓÏ ÔÓÒÚË Ë Ó·ÂÍÚË‚ÌË ÏÂÚÓ‰Ë ·Ë ·ËÎÓ ÔÓÎÂÁÌÓ ‚ ̇˜‡ÎÓÚÓ Ì‡ ΘÂÌËÂÚÓ, Ú˙È Í‡ÚÓ ‡ÌÌÓÚÓ ÔÓ„ÌÓÁË‡Ì ̇ ÎÂ͇ÒÚ‚ÂÌÓ-Ó·ÛÒÎÓ‚ÂÌÓÚÓ ÍÓ„ÌËÚË‚ÌÓ Ì‡Û¯ÂÌË  ÔÓ·ÎÂÏÌÓ Ì‡ ÚÓÁË ÂÚ‡Ô (11). ï‡‡ÍÚÂËÒÚËÍËÚ ̇ Ô‡ˆËÂÌÚ‡, ÂÔËÎÂÔÚ˘ÌËflÚ ÒË̉ÓÏ, ‰ÓÁ‡Ú‡ Ë ÒÂÛÏÌÓÚÓ ÌË‚Ó Ì‡ ‡ÌÚËÂÔËÎÂÔÚ˘ÌËڠωË͇ÏÂÌÚË ÌflÏ‡Ú ÔÓ„ÌÓÒÚ˘̇ ÒÚÓÈÌÓÒÚ (36). Salynski et al. (1994, 2002, 2003), ̇ÏË‡Ú, ˜Â ÏÂÊ‰Û ˜ÂÚËËÚ ËÁÒΉ‚‡ÌË ÓÚ Úflı ÔÓ͇Á‡ÚÂÎË ÓÚ Ó͈ËÔËÚ‡Î̇ڇ ӷ·ÒÚ (ÔËÍÓ‚‡ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡, ωˇÌ̇ ˜ÂÒÚÓÚ‡ ̇ ˆÂÎËfl ÒÔÂÍÚ˙, ÓÚÌÓÒËÚÂÎ̇ ‰ÂÎÚ‡ Ë ÚÂÚ‡ ÏÓ˘ÌÓÒÚ) ÔËÍÓ‚‡Ú‡ ‡ÎÙ‡ ˜ÂÒÚÓÚ‡  ̇È-˜Û‚ÒÚ‚ËÚÂÎÂÌ ÍÓ΢ÂÒÚ‚ÂÌ Ô‡‡ÏÂÚ˙ Í˙Ï ÂÙÂÍÚ‡ ̇ ÄÖå. Ä‚ÚÓËÚ ‰Ó͇Á‚‡Ú, ˜Â ӷ˘‡È̇ڇ ‡ÌÚËÂÔËÎÂÔÚ˘̇ ÚÂ‡ÔËfl ÏÓÊ ‰‡ ·˙‰Â Ò‚˙Á‡Ì‡ Ò˙Ò ÒË„ÌËÙË͇ÌÚÌÓ Á‡·‡‚flÌ ÔË ÎËÔÒ‡Ú‡ ̇ ÍÎËÌ˘ÌË ÔÓfl‚Ë Ì‡ Ì‚ÓÚÓÍÒ˘ÌÓÒÚ Ë ˜Â ÔÓÏÂÌËÚ ÍÓÂÎË‡Ú Ò ÔÓÏÂÌËÚ ‚ ÍÓ„ÌËÚË‚ÌËÚ ÔÓ͇Á‡ÚÂÎË Ë Ò˙Ò ÒÛ·ÂÍÚË‚ÌËÚ ÔÓfl‚Ë Ì‡ Ì‚ÓÚÓÍÒ˘ÌÓÒÚ (56,57,58). чÌÌËÚ ÓÚ ÎËÚÂ‡ÚÛ‡Ú‡ ÔÓ͇Á‚‡Ú, ˜Â ÍÓ΢ÂÒÚ‚ÂÌËflÚ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ ‚Ò Ӣ Ì Ò  ÛÚ‚˙‰ËΠ̇ ÔÓ-

ÌÂÚÓ Ì‡ ÏÓÁ˙͇ (9,22,31,32,46, 47,66). ãàíÖêÄíìêÄ 1.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÑÓˆ. ÄÎÂÍ҇̉˙ ÄÎÂÍÒË‚, ç‚ÓÎӄ˘̇ ÍÎËÌË͇ ìåÅÄã ◊ÄÎÂÍ҇̉Ó‚Ò͇” ÅÛÎ. ◊É. ëÓÙËÈÒÍË” ‹1, 1431 ëÓÙËfl 02 9230 465; e-mail: a.alexiev@yahoo.com


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é·ÁÓ ÑÖÉÖçÖêÄíàÇçÄ ãìåÅÄãçÄ ëèéçÑàãéãàëíÖáÄ è. ñ‚ÂÚ‡ÌÓ‚1, à. Ç˙ÎÍÓ‚1, Å. ëÚ‡ÏÂÌÓ‚1, Ç. êÓÒχÌÓ‚2, ã. âÓ‚˜Â‚‡2, ë. çfl„ÓÎÓ‚‡3, ç. íӈ‚3 K‡Ú‰‡ Ì‚ÓÎÓ„Ëfl Ë Ì‚ÓıËÛ„Ëfl, ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡, å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ-è΂ÂÌ 2 ä‡Ú‰‡ ÔÓ ÓÚÓÔ‰Ëfl Ë Âı‡·ËÎËÚ‡ˆËfl, ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡, å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ-è΂ÂÌ 3 ä‡Ú‰‡ ÔÓ ÂÌÚ„ÂÌÓÎÓ„Ëfl ‡‰ËÓÎÓ„Ëfl, ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡, å‰ˈËÌÒÍË ÛÌË‚ÂÒËÚÂÚ-è΂ÂÌ 1

SUMMARY DEGENERATIVE LUMBAR SPONDYLOLISTHESIS P. Tzvetanov, I. Volkov, B. Stamenov, V. Rossmanov, L. Yovcheva, S. Niagulova, N. Tocev Degenerative lumbar spondylolisthesis associated with spinal stenosis and instability is a common condition of the aging spine. Some aspects of diagnosis, appropriate nonsurgical treatment and necessity Óf surgical decompression are controversial. In other hand it is very important for neurologist to be familiar with early symptoms of lumbar spondylolisthesis. The aim of this review is to summarize the available literature data and to suggest the clear criteria for diagnosis and surgical treatment of lumbar spondylolisthesis. KEY WORDS: degenerative lumbar spondylolisthesis, spinal stenosis, and spinal instability. êÖáûåÖ Ñ„ÂÌÂ‡Ú˂̇ڇ ÎÛÏ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ Ë Ò˙ÔÓ‚Óʉ‡˘ËÚ fl ÒÔË̇Î̇ ÒÚÂÌÓÁ‡ Ë ÌÂÒÚ‡·ËÎÌÓÒÚ Ò‡ ˜ÂÒÚË Á‡·ÓÎfl‚‡ÌËfl ̇ „˙·Ì‡Í‡ Ò Ì‡Ô‰‚‡Ì ̇ ‚˙Á‡ÒÚÚ‡. éÚÌÓÒÌÓ ÌflÍÓË ‡ÒÔÂÍÚË Ì‡ ‰Ë‡„ÌÓÒÚË͇ڇ, ÔÓ‰ıÓ‰fl˘ÓÚÓ ÌÂıËÛ„˘ÌÓ Î˜ÂÌËÂ Ë ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ÓÚ ıËÛ„˘̇ ‰ÂÍÓÏÔÂÒËfl Ëχ ÔÓÚË‚ÓÂ˜Ë‚Ë ÒÚ‡Ìӂˢ‡. éÚ ‰Û„‡ ÒÚ‡Ì‡, Á‡ Ì‚ÓÎÓÁËÚ  ÓÚ ËÁÍβ˜ËÚÂÎ̇ ‚‡ÊÌÓÒÚ ‰‡ Ò ÔÓÁ̇‚‡Ú ‡ÌÌËÚ ÒËÏÔÚÓÏË Ì‡ ÎÛÏ·‡Î̇ڇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. ñÂΠ̇ ̇ÒÚÓfl˘Ëfl Ó·ÁÓ  ‰‡ Ò ӷӷ˘flÚ Ì‡Î˘ÌËÚ ÎËÚÂ‡ÚÛÌË ‰‡ÌÌË Ë ‰‡ Ò Ô‰ÎÓÊ‡Ú flÒÌË ÍËÚÂËË Á‡ ‰Ë‡„ÌÓÁ‡ Ë ıËÛ„˘ÌÓÚÓ Î˜ÂÌË ̇ ÎÛÏ·‡Î̇ڇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. äãûóéÇà Ñìåà: ‰Â„ÂÌÂ‡Ú˂̇ ÎÛÏ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡, ÒÔË̇Î̇ ÒÚÂÌÓÁ‡, ÒÔË̇Î̇ ÌÂÒÚ‡·ËÎÌÓÒÚ. è˙‚ÓÚÓ ÓÔËÒ‡ÌË ̇ ‰Â„ÂÌÂ‡Ú˂̇ڇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ ‰‡ÚË‡ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ 19ÚË ‚ÂÍ (1). H. Junghanns (8) Ӣ ‚ 1930 „. ÓÔ‰ÂÎfl “ÔËÔÎ˙Á‚‡ÌÂÚÓ” ̇ ÎÛÏ·‡ÎÌËÚ Ô¯ÎÂÌË ·ÂÁ ËÌÚÂ‡ÚËÍÛ·ÂÌ ‰ÂÙÂÍÚ Í‡ÚÓ “ÔÒ‚‰ÓÒÔÓ̉ËÎÓÎËÒÚÂÁ‡”. íÓ‚‡ Ò˙ÒÚÓflÌË ÔÓ-Í˙ÒÌÓ Â Í‡Ú„ÓËÁË‡ÌÓ Í‡ÚÓ ‰Â„ÂÌÂ‡Ú˂̇ ÎÛÏ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ ÓÚ P.H. Newman ‚ 1963 „. (12). ëÔÓ‰ ÚÓÁË ‡‚ÚÓ, ÔËÔÎ˙Á‚‡ÌÂÚÓ Ì‡ ÎÛÏ·‡ÎÌËÚ Ô¯ÎÂÌË ·ÂÁ Ò˙ÔÓ‚Óʉ‡˘ ‰ÂÙÂÍÚ Ì‡ ‰˙„ËÚ ̇ Ô¯ÎÂÌËÚ  ̇È-˜ÂÒÚÓ ÂÁÛÎÚ‡Ú ÓÚ ‰Â„ÂÌÂ‡ÚË‚ÂÌ ‡ÚËÚ Ì‡ ÎÛÏ·‡ÎÌËÚ هÒÂÚÌË ÒÚ‡‚Ë. ÖÚËÓÎÓ„ËflÚ‡ Ë Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ‰Â„ÂÌÂ‡Ú˂̇ڇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡  ÏÌÓ„ÓÙ‡ÍÚÓ̇. ífl Á‡Òfl„‡ Ô‰ËÏÌÓ ‚˙Á‡ÒÚÌË ·ÓÎÌË Ë Ò ̇·Î˛‰‡‚‡ ̇È-˜ÂÒÚÓ Ì‡ ÌË‚Ó L4L5. Ç Â‰ÌÓ Ó·ÒÚÓÈÌÓ Ë Á‡‰˙ηӘÂÌÓ ‡Ì‡ÚÓÏ˘ÌÓ Ë ÍÎËÌ˘ÌÓ ÔÓÛ˜‚‡Ì ̇ Ôӂ˜ ÓÚ 200 ·ÓÎÌË Ò ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â Úfl  ÔÂÚ ‰Ó ¯ÂÒÚ Ô˙ÚË ÔÓ-˜ÂÒÚ‡ ÔË ÊÂÌËÚÂ Ë ÚË Ô˙ÚË ÔÓ-˜ÂÒÚ‡ ÔË ‡ÙÓ‡ÏÂË͇̈ËÚ (14). è‰ÔÓ·„‡ ÒÂ, ˜Â ÓÒÌӂ̇ڇ Ô˘Ë̇ Á‡ ÚÓ‚‡ Ò‡ ıÓÏÓ̇ÎÌË Ù‡ÍÚÓË Ë ÔÓÏfl̇ ̇ Úflı̇ڇ ÍÓ̈ÂÌÚ‡ˆËfl ÔÂ‰Ë ÏÂÌÓÔ‡ÛÁ‡, Ô‰ËÁ‚ËÍ‚‡˘Ó „ÂÌÂ‡ÎËÁË‡Ì‡ ÎË„‡ÏÂÌÚÓÁ̇ ı‡ÎÚ‡‚ÓÒÚ. èÓ-‚ËÒÓ͇ڇ ˜ÂÒÚÓÚ‡ ÔË ‡ÙÓ‡ÏÂË͇̈ËÚ ÏÓÊ ‰‡ ·˙‰Â Ó·flÒÌÂ̇ Ò ËÁ„·‰Â̇ڇ ÎÛÏ·ÓÒ‡Í‡Î̇/Ò‡Í-

‡Î̇ ÎÓ‰ÓÁ‡ ÔË Ú‡ÁË ‡Ò‡, ͇ÍÚÓ Ë Ò Û‚Â΢Â̇ڇ ˜ÂÒÚÓÚ‡ ̇ Ò‡Í‡ÎËÁ‡ˆËfl ̇ ÔÂÚËfl ÎÛÏ·‡ÎÂÌ Ô¯ÎÂÌ (14). Ç ÂÁÛÎÚ‡Ú Ì‡ ÚÂÁË ‡Ì‡ÚÓÏ˘ÌË ÓÒÓ·ÂÌÓÒÚË Ò ۂÂ΢‡‚‡ ̇ÚÓ‚‡‚‡ÌÂÚÓ ‚˙ıÛ ËÌÚÂÔ‰ÛÌÍÛ·ÌËÚ ÒÚ‡‚Ë. é·˘‡ ˜ÂÚ‡ ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ‰Â„ÂÌÂ‡Ú˂̇ڇ ‚ÂÚ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡  ̇΢ËÂÚÓ Ì‡ Ò˙ÔÓ‚Óʉ‡˘ ‡ÚËÚ Ì‡ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë ÔË ÎËÔÒ‡ ̇ Ô‡ÚÓÎӄ˘ÌË ÔÓÏÂÌË ‚ ÓÒڇ̇ÎËÚ ‚ÂÚ·‡ÎÌË ÒÚÛÍÚÛË (îË„. 1). ãÛÏ·‡ÎÌËÚ هÒÂÚÌË ÒÚ‡‚Ë ÔÓ̇ÒflÚ ÓÚ 3 ‰Ó 35% ÓÚ

îË„. 1 ë˙Ô˙ÚÒÚ‚‡˘Ë ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡Ú‡ ÔÓÏÂÌË Ì‡ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë Ë ËÁ‡ÁÂ̇ ·ÚÂ‡Î̇ ÒÚÂÌÓÁ‡ ̇ ÌË‚Ó L5-S1 ÔË 77-„Ӊ˯̇ ·ÓÎ̇ ÒÚ‡Ú˘ÌÓÚÓ Ì‡ÚÓ‚‡‚‡Ì ‚ ÎÛÏ·‡ÎÌËfl Ò„ÏÂÌÚ Ì‡ „˙·Ì‡Í‡, ÍÓÂÚÓ ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÔÓÁˈËflÚ‡ ̇ „˙·Ì‡Í‡ ‰Ë̇Ï˘ÌÓ Ò Ôӂ˯‡‚‡ ‰Ó 33% ‚ ‡ÍÒˇÎÂÌ ÔÎ‡Ì (11, 16). Ç ÍÓÓ̇ÂÌ ÔÎ‡Ì Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë ÔÓ‰‰˙Ê‡Ú ÎÓ‰ÓÁ‡Ú‡ ̇ „˙·Ì‡Í‡ ÔË Ì„ӂ‡Ú‡ ÙÎÂÍÒËfl (4). L. Grobler Ë Ò˙‡‚Ú. (4) ÓÔËÒ‚‡Ú ÔÓ-˜ÂÒÚ‡ Ò‡„ËÚ‡Î̇ڇ ÓËÂÌÚ‡ˆËfl ̇ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë ÔË Ô‡ˆËÂÌÚË Ò ‰Â„ÂÌÂ‡Ú˂̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ ‚ Ò‡‚ÌÂÌËÂ Ò ÌÓχÎ̇ڇ ÔÓÔÛ·ˆËfl. èÓ‡‰Ë ÔÂӷ·‰‡‚‡˘‡Ú‡ ÍÓÓ̇̇ ÓËÂÌÚ‡ˆËfl ̇ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë ‚ L5-S1 „˙·Ì‡˜ÂÌ Ò„ÏÂÌÚ, ÔÓ-„ÓÎflχ ˜‡ÒÚ ÓÚ ‰Â„ÂÌÂ‡ÚË‚ÌËÚ ÒÚÂÌÓÁË Ò Ò¢‡Ú ̇ ÌË‚Ó L4-L5 (Í˙‰ÂÚÓ ÔÓ-˜ÂÒÚ‡  ҇„ËÚ‡Î̇ڇ ÓËÂÌÚ‡ˆËfl). ë˙˜ÂÚ‡ÌËÂÚÓ Ì‡ Ô‰‡ÁÔÓ·„‡˘Ë ıÓÏÓ̇ÎÌË Ë ÏÂı‡Ì˘ÌË Ù‡ÍÚÓË, Á‡Â‰ÌÓ Ò ‡Á‚ËÚËÂÚÓ Ì‡ ÓÒÚÂÓ‡ÚËÚ ‚Ó‰Ë ‰Ó ‡Á‚ËÚË ̇ Ô‰̇ ÒÛ·ÎÛÍÒ‡ˆËfl. è˙‚Ó̇˜‡ÎÌÓ Ì‡ÒÚ˙Ô‚‡Ú ‰Â„ÂÌÂ‡ÚË‚ÌË ÔÓÏÂÌË Ì‡ ËÌÚÂ‚ÂÚ·‡ÎÌËÚ ‰ËÒÍÓ‚Â, ÍÓÂÚÓ ÓÚ Ò‚Ófl ÒÚ‡Ì‡ ‚Ó‰Ë ‰Ó ‡Áı··‚‡Ì ̇ ligamentum flavum Ë ÌÂÒÚ‡·ËÎÌÓÒÚ ‚ Ò˙ÓÚ‚ÂÚÌËfl Ò„ÏÂÌÚ. ä‡ÚÓ ÒΉÒÚ‚Ë ÓÚ ÚÂÁË ÔÓÏÂÌË Ì‡ÒÚ˙Ô‚‡ ıËÔÂÚÓÙËfl ̇ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë Ë ‡ÁÚfl„‡Ì ̇ ÒÚ‡‚ÌËÚ ͇ÔÒÛÎË. èÓÒΉfl‚‡ÌÂÚÓ Ì‡ ·ÓÎÌËÚÂ Ò ÔÓ‰Ó·ÌË ‰Â„ÂÌÂ‡ÚË‚ÌË ÔÓÏÂÌË ÔÓ͇Á‚‡, ˜Â ÔË 30% ÓÚ Úflı Ò ‡Á‚Ë‚‡ ‰Â„ÂÌÂ‡Ú˂̇ ÎÛÏ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. êfl‰ÍÓ ÔËÔÎ˙Á‚‡ÌÂÚÓ Ì‡‰ı‚˙Îfl 25-30% ÓÚ ¯ËË̇ڇ ̇ ÔÓ‰ÎÂʇ˘Ëfl Ô¯ÎÂÌ Ë ıËÛ„˘̇ ËÌÚÂ‚Â̈Ëfl Ò Ô‰ÔËÂχ Ò‡ÏÓ ‚ ÒÎÛ˜‡ËÚÂ, ÔË ÍÓËÚÓ ÍÓÌÒÂ‚‡ÚË‚ÌÓÚÓ Î˜ÂÌË  ÌÂÂÙÂÍÚË‚ÌÓ


‰ÂÍÂÏ‚Ë, 2007

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

(10). äÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇ ̇ ÎËÒÚÂÁ‡Ú‡ Á‡‚ËÒË ÓÚ ÚÓ‚‡ ‰‡ÎË Úfl Ò Ò˙ÔÓ‚Óʉ‡ ÓÚ ÒÔË̇Î̇ ÒÚÂÌÓÁ‡ ̇ Û‚‰ÂÌÓÚÓ ÌË‚Ó. ÅÓÎÍËÚ ‚ ÎÛÏ·‡ÎÌËfl ÓÚ‰ÂÎ ˜ÂÒÚÓ Ò ‰˙ÎÊ‡Ú Ì‡ Ò˙ÔÓ‚Óʉ‡˘ ÎËÒÚÂÁ‡Ú‡ ‡ÚËÚ, ÍÓÈÚÓ Úfl·‚‡ ‰‡ Ò ÎÂÍÛ‚‡ ‰ÓË Ë ÔË ÓÚÒ˙ÒÚ‚Ë ̇ ÎËÒÚÂÁ‡ (6). ÅÓÎÌËÚÂ Ò ÔË·‡‚Â̇ Í˙Ï ÎËÒÚÂÁ‡Ú‡ ÒÔË̇Î̇ ÒÚÂÌÓÁ‡ ËÏ‡Ú ı‡‡ÍÚÂÌË Á‡ ÌÂfl ÒËÏÔÚÓÏË - ·ÓÎÍË ‚ Á‡‰Ì‡Ú‡ ˜‡ÒÚ Ì‡ ·Â‰ÓÚÓ Ë ÔÓ ıÓ‰‡ ̇ ‰ÓÎÌËfl Í‡ÈÌËÍ, Ó·ËÍÌÓ‚ÂÌÓ ‡ÒÓˆËË‡ÌË Ò ·ÓÎÍË ‚ Í˙ÒÚ‡. ï‡‡ÍÚÂÂÌ ·Â΄ ̇ ÚÂÁË ·ÓÎÍË Â ˜Â ‚˙ÁÌËÍ‚‡Ú ÔË ıÓ‰ÂÌÂ, Ó·ÎÂ͘‡‚‡Ú Ò ÔË ÔӘ˂͇ Ë ÔË Ì‡‚Âʉ‡Ì ̇ „˙·Ì‡Í‡ (ËÎË ÔË Ó·Îfl„‡ÌÂ). ÅÓÎ͇ڇ ‚ ‰ÓÎÌËfl Í‡ÈÌËÍ ÏÓÊ ‰‡  ÛÌË·ÚÂ‡Î̇ ËÎË ·Ë·ÚÂ‡Î̇, Ò ı‡‡ÍÚÂËÒÚË͇ ̇ Ì‚ÓÍ·ۉË͇ˆËÓ ËÎË ‡‰ËÍÛÎÂ̇ ·ÓÎ͇ (6). èË Ô‡ˆËÂÌÚË Ò˙Ò ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ Ë ÒÚÂÌÓÁ‡ ̇ L4-L5 ÌË‚Ó, Ó·ËÍÌÓ‚ÂÌÓ Ò Á‡Òfl„‡ L5 ÍÓÂÌ. èË ÚÂÊ͇ ÒÚÂÌÓÁ‡ ̇ ‚ÂÚ·‡ÎÌËfl ͇̇ΠÏÓÊ ‰‡ Ò Á‡Ò„ÌÂ Ë L4 ÍÓÂÌ. çÂÓ·ıÓ‰ËÏÓ Â Ó·‡˜Â ‰‡ Ò ËÁÍβ˜Ë ÔÂËÙÂÌÓ-Ò˙‰Ó‚Ó Á‡·ÓÎfl‚‡Ì ËÎË ÔÓÎËÌ‚ÓÔ‡ÚËfl, Ú˙È Í‡ÚÓ Ë ‰‚ÂÚ Ò˙ÒÚÓflÌËfl ÏÓ„‡Ú ‰‡ ÔÓÚ˘‡Ú Ò Ì‚ÓÍ·ۉË͇ˆËÓ. é·ËÍÌÓ‚ÂÌÓ ·ÓÎ͇ڇ ÔË ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ Ò ÔÓ‚ÓÍË‡ ÔË ÓÔ‰ÂÎÂ̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ. ÄÍÓ ·ÓÎÌËÚ ËÏ‡Ú ËÁ‡ÁÂÌ ·ÓÎÍÓ‚ ÒË̉ÓÏ ‚ ÔÓÍÓÈ, ÚÓ‚‡ ̇·„‡ ‰‡ Ò ËÁÍβ˜‡Ú ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl - ̇Ô. ÚÛÏÓ, Ò˙‰Ó‚Ó Á‡·ÓÎfl‚‡Ì (5, 6). èË ÙËÁË͇ÎÌÓÚÓ ËÁÒΉ‚‡Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ó„‡Ì˘ÂÌË ̇ Ô‡ÒË‚ÌËÚ ‰‚ËÊÂÌËfl ̇ „˙·Ì‡Í‡ ‚ ÎÛÏ·‡ÎÌËfl Ò„ÏÂÌÚ, ÓÒÓ·ÂÌÓ ÔË ÂÍÒÚÂÌÁËfl, Ú˙È Í‡ÚÓ ÒÔË̇ÎÌËflÚ Í‡Ì‡Î Ò ÒÚÂÌfl‚‡ Ӣ Ôӂ˜ ÔË ÂÍÒÚÂÌÁËfl. íÓ‚‡ Ó·flÒÌfl‚‡ ‰Ó· ËÁ‚ÂÒÚÌËfl ÍÎËÌ˘ÂÌ Ù‡ÍÚ, ˜Â ·ÓÎÍÓ‚ËflÚ ÒË̉ÓÏ Ò ӷÎÂ͘‡‚‡ ÔË Ì‡‚Âʉ‡Ì ̇Ô‰ (6). åÛÒÍÛÎ̇ Ò··ÓÒÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‚ ÓÍÓÎÓ 15-20% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ͇ÚÓ Úfl Ó·ËÍÌÓ‚ÂÌÓ Â ÛÏÂÂ̇ Ë Ì‡È-˜ÂÒÚÓ Â Ó·ÛÒÎÓ‚Â̇ ÓÚ ÎÂÁËfl ̇ L5 ÍÓÂÌ. ᇄ۷‡Ú‡ ̇ ÔÓÔËÓˆÂÔÚË‚ÌËÚ ÂÙÎÂÍÒË Â ÌÂÒÔˆËÙ˘ÂÌ ·Â΄, Ú˙È Í‡ÚÓ ÔË ‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË ÍÓÎÂÌÌËÚ ÂÙÎÂÍÒË ˜ÂÒÚÓ ÎËÔÒ‚‡Ú. ê‡ÁÒÚÂÊÌËÚ ·ÓÎÍÓ‚Ë ÙÂÌÓÏÂÌË (ı‡‡ÍÚÂÌË Á‡ Ô‡ˆËÂÌÚËÚÂ Ò ‰ËÒÍÓ‚‡ ıÂÌËfl) Ì ҇ ˜ÂÒÚË ÔË ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. í‡ÁÓ‚ÓÂÁÂ‚Ó‡ÌËÚ ‡ÁÒÚÓÈÒÚ‚‡ ÓÚ Ó‰‡ ̇ ËÌÍÓÌÚËÌÂ̈ËflÚ‡ ËÎË Ì‡Û¯ÂÌ ÍÓÌÚÓÎ Ò‡ ˜ÂÒÚË, ÌÓ Ì Ò ҂˙Á‚‡Ú Ò˙Ò ÒÚÂÌÓÁ‡ ̇ ͇̇·, ‡ ÔÓ-ÒÍÓÓ Ò ÓÚ‰‡‚‡Ú ̇ ‚˙Á‡ÒÚÓ‚Ë ÓÒÓ·ÂÌÓÒÚË (5, 6). ÇÌËχÚÂÎÌÓÚÓ ËÁÒΉ‚‡Ì ̇ ‰ÓÎÌËÚ Í‡ÈÌËˆË Â ÓÚ ÓÒÓ·Â̇ ‚‡ÊÌÓÒÚ, ͇ÚÓ Ì‡ÒÓ˜ÂÌÓ Ò Ú˙ÒflÚ Ò˙‰Ó‚Ë ÔÓ·ÎÂÏË ËÎË ·ÂÎÂÁË Ì‡ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ͇ÚÓ Ì‡ÔËÏÂ Á‡„Û·‡Ú‡ ̇ ÒÚ‡‚ÌÓ-ÏÛÒÍÛÎÂÌ ÛÒÂÚ. éÒÚÂÓ‡ÚËÚ˙Ú Ì‡ Ú‡Áӷ‰ÂÌËÚÂ Ë ÍÓÎÂÌÌË ÒÚ‡‚Ë Ò˙˘Ó ÏÓÊ ‰‡ Á‡Ï˙„ÎË ÍÎËÌ˘̇ڇ ͇ÚË̇ ̇ ‰Â„ÂÌÂ‡Ú˂̇ڇ ÒÚÂÌÓÁ‡, ÔÓ‡‰Ë ÍÓÂÚÓ ÂÌÚ„ÂÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì  Á‡‰˙ÎÊËÚÂÎÌÓ (6). ç‡ÎÓÊËÚÂÎÌÓ Â ÔÓ‚Âʉ‡Ì ̇ ÂÌÚ„ÂÌÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì ‚ ËÁÔ‡‚Â̇ ·ÚÂ‡Î̇ ÔÓÁˈËfl (‚ 15% ÓÚ Ô‡ˆËÂÌÚËÚ Ò ÓÚÍË‚‡ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡), ÍÓflÚÓ Ì Ò ‚ËÁÛ‡ÎËÁË‡ ‚ ΄̇· ÒÚ‡Ì˘̇ ÔÓÁˈËfl. îÎÂÍÒËÓÌÌÓ-ÂÍÒÚÂÌÁËÓÌÌÓ Î‡ÚÂ‡ÎÌËÚ ÂÌÚ„ÂÌÓ„‡ÙËË Ò‡ ÓÒÓ·ÂÌÓ ‚‡ÊÌË, Ú˙È Í‡ÚÓ ÔËÔÎ˙Á‚‡ÌÂÚÓ Ì‡ Ô¯ÎÂÌËÚ Ò ۂÂ΢‡‚‡ ÔË ÂÍÒÚÂÌÁËfl ̇ „˙·Ì‡Í‡. ç‡ÏË‡Ú Ò ۂÂ΢Â̇ Ú‡ÌÒ·ˆËÓÌ̇ ÔÓ‰‚ËÊÌÓÒÚ Ì‡ Ô¯ÎÂÌËÚÂ Ë ‡Ì„Û·̇ ÌÂÒÚ‡·ËÎÌÓÒÚ. í‡ÌÒ·ˆËÓÌ̇ڇ ÔÓ‰‚ËÊÌÓÒÚ Ì‡ Ô¯ÎÂÌËÚ Ò ҘËÚ‡ Á‡ Ô‡ÚÓÎӄ˘̇, ‡ÍÓ ÔË ÙÎÂÍÒËfl ̇ „˙·Ì‡Í‡  ÔÓ-„ÓÎflχ ÓÚ 4 mm. ÄÌ„Û·̇ڇ ÌÂÒÚ‡·ËÎÌÓÒÚ Ò ÔËÂχ Á‡ ̇΢̇ ÔË ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÏÂʉÛÔ¯ÎÂÌÂÌ ˙„˙Π̇ ËÁÒΉ‚‡ÌÓÚÓ ÌË‚Ó ÔÓ-„ÓÎflÏ Ò 100 ÓÚ ˙„ÎËÚ ̇‰ Ë ÔÓ‰ ÌË‚ÓÚÓ (6). äÓÏÔ˛Ú˙̇ڇ ÚÓÏÓ„‡ÙËfl ÓÒË„Ûfl‚‡ ‰ÂÚ‡ÈÎ̇ ËÌÙÓχˆËfl Á‡ Ô‡ÚÓÎÓ„ËflÚ‡ Ë ÓËÂÌÚ‡ˆËflÚ‡ ̇ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë (îË„. 1), ÌÓ ÔÓ‡‰Ë ÚÓ‚‡ ˜Â ̇È-˜ÂÒÚÓ Ò ÒÍÂÌË‡Ú ÓÚ L3 ‰Ó S1 ÌË‚‡, Ò ÔÓÔÛÒÍ‡Ú ÒÚÂÌÓÚ˘ÌËÚ ÔÓ-

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ÏÂÌË Ì‡ L2-L3 Ë Ì‡ ÔÓ-‚ËÒÓÍË ÌË‚‡ (Ò˙ÒÚ‡‚Îfl‚‡˘Ë 1520% ÓÚ ‚Ò˘ÍË ÒÚÂÌÓÁË) (5, 6). åËÂÎÓ„‡ÙËflÚ‡ ÓÒË„Ûfl‚‡ ˆflÎÓÒÚ̇ ͇ÚË̇ ÔÓ ÔÓÚÂÊÂÌË ̇ ˆÂÎËfl „˙·Ì‡Í Ë Â ÔÓ-ËÌÙÓχÚ˂̇ ‡ÍÓ Ò ÓÒ˙˘ÂÒÚ‚fl‚‡ ‚ ËÁÔ‡‚Â̇ ÔÓÁˈËfl (6). èÓÎÂÁÌË ‚ ‰Ë‡„ÌÓÒÚ˘ÌÓ ÓÚÌÓ¯ÂÌË ҇ Ë ‰Ë̇Ï˘ÌËÚ ÙÎÂÍÒËÓÌÌÓ-ÂÍÒÂÌÁËÓÌÌË „‡ÙËË, ÍÓËÚÓ Ò‡ ÔÓ-ËÌÙÓχÚË‚ÌË ÓÚ ÒÚ‡Ú˘ÌËÚ ÂÌÚ„ÂÌÓ„‡ÙËË. Ç 20% ÓÚ ÒÎÛ˜‡ËÚ ÒΉ ÏËÂÎÓ„‡ÙËfl Ó·‡˜Â Ò ̇·Î˛‰‡‚‡Ú ÒÚ‡Ì˘ÌË ‡͈ËË Í‡ÚÓ „·‚Ó·ÓÎËÂ Ë „‡‰ÂÌ (6). 凄ÌËÚÌÓ-ÂÁÓ̇ÌÒÌÓÚÓ ËÁÒΉ‚‡Ì ÓÒË„Ûfl‚‡ ÔˆËÁÌÓ ËÁÓ·‡Áfl‚‡Ì ̇ ÎÛÏ·‡ÎÌËfl „˙·Ì‡Í Ë Ï‰Û·ÌËfl ÍÓÌÛÒ. íÓÈ Â Ì‡È-‰Ó·ÓÚÓ ‰Ë‡„ÌÓÒÚ˘ÌÓ Ò‰ÒÚ‚Ó Á‡ ËÁÓ·‡Áfl‚‡Ì ̇ ÏÂÍËÚ Ú˙͇ÌË Ë Â Ò‰ÒÚ‚Ó Ì‡ ËÁ·Ó ÔË Ò˙ÏÌÂÌË Á‡ ÒÚÂÌÓÁ‡ ̇ ‚ÂÚ·‡ÎÌËfl ͇̇Π(5). ÖÎÂÍÚÓÏËÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡ÌÂ Ë ÒÓχÚÓÒÂÌÁÓÌËÚ ‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË Ò‡ ÔÓÎÂÁÌË ‰Ë‡„ÌÓÒÚ˘ÌË ÏÂÚÓ‰ËÍË Á‡ ÓÚ‰ËÙÂÂ̈Ë‡Ì ̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡Ú‡ ÓÚ Ì‚ÓÍ·ۉË͇ˆËÓÚÓ ÔË Ô‡ˆËÂÌÚË Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ (1, 5, 6). 燘‡ÎÌÓÚÓ Î˜ÂÌË ̇ Ô‡ˆËÂÌÚË Ò ·ÓÎÍË ‚ Í˙ÒÚ‡ Ë ‰ÓÎÌËfl Í‡ÈÌËÍ ÔË ‰Â„ÂÌÂ‡Ú˂̇ ÎÛÏ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ (Ò ËÎË ·ÂÁ ÒÔË̇Î̇ ÒÚÂÌÓÁ‡) Ò Ò˙ÒÚÓË ÓÚ Í‡Ú˙Í ÔÓÒÚÂÎÂÌ ÂÊËÏ (2-5 ‰ÌË) Ë ÔÓÚË‚Ó‚˙ÁÔ‡ÎËÚÂÎ̇ ÚÂ‡ÔËfl (9). çÂÒÚÂÓˉÌËÚ ÔÂÔ‡‡ÚË ÌflÏ‡Ú ‰Ó͇Á‡Ì‡ ÔÓ-‰Ó·‡ ÂÙÂÍÚË‚ÌÓÒÚ ÓÚ ÚÂ‡ÔËflÚ‡ Ò ‡ÒÔËËÌ (6). ÄÍÓ ·ÓÎÍÓ‚‡Ú‡ ÒËÏÔÚÓχÚË͇ ÔÂÒËÒÚË‡ Ò ÔËÒÚ˙Ô‚‡ Í˙Ï ÙËÁË͇Î̇ ÚÂ‡ÔËfl, ͇ÚÓ ÛÎÚ‡Á‚ÛÍ, ÚÓÔÎËÌÌË Ôӈ‰ÛË, Ï‡Ò‡Ê Ë ÂÍÒÚÂÌÁËfl. íÂÁË Ôӈ‰ÛË Ì‡Ï‡Îfl‚‡Ú Ôӂ˯ÂÌËfl ÏÛÒÍÛÎÂÌ ÚÓÌÛÒ Ì‡ Ô‡‡‚ÂÚ·‡Î̇ڇ ÏÛÒÍÛ·ÚÛ‡. èË ÌÂÔÓ‚ÎËfl‚‡ÌÂÚÓ Ì‡ ·ÓÎ͇ڇ Ò ÔÂÔÓ˙˜‚‡ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÎÛÏ·‡ÎÂÌ ÍÓÒÂÚ (6, 9). ÖÔˉÛ‡Î̇ڇ ËÌÙËÎÚ‡ˆËfl Ò˙Ò ÒÚÂÓˉ  ÔÓ͇Á‡Ì‡ ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒËÎ̇ Ë ÌÂÔÓ‚ÎËfl‚‡˘‡ Ò ‡‰ËÍÛ·΄Ëfl. Ç˙ÔÂÍË ˜Â Ì ҇ Ô‡‚ÂÌË Á‡‰˙ηӘÂÌË ÔÓÛ˜‚‡ÌËfl ̇ Ú‡ÁË Î˜·̇ Ôӈ‰Û‡, ·ÓÎÌËÚ ˜ÂÒÚÓ Ò˙Ó·˘‡‚‡Ú Á‡ Ó·ÎÂ͘‡‚‡Ì ̇ ·ÓÎ͇ڇ χ͇ Ë Ò ‚ÂÏÂÌÂÌ ÂÙÂÍÚ (9). lj̇„‡ ÒΉ ӂ·‰fl‚‡ÌÂÚÓ Ì‡ ÓÒÚ‡Ú‡ ·ÓÎÍÓ‚‡ ÒËÏÔÚÓχÚË͇ Ò ÔËÒÚ˙Ô‚‡ Í˙Ï ÙËÁ˘ÂÒÍË ÛÔ‡ÊÌÂÌËfl, ͇ÚÓ Ò ÒΉ‚‡Ú Ë̉˂ˉۇÎÌË ÔÓ„‡ÏË. íÂÁË ÔÓ„‡ÏË ‚Íβ˜‚‡Ú ‡ÂÓ·ÌË ÛÔ‡ÊÌÂÌËfl, ͇ÚÓ Ì‡Ô. ͇‡Ì ̇ ÒÚ‡ˆËÓ̇ÌÓ ÍÓÎÂÎÓ ËÎË ÔÎÛ‚‡ÌÂ. ÄÍÚË‚ÌËÚ ÙÎÂÍÒËÓÌÌË ÛÔ‡ÊÌÂÌËfl Ë ÎÂÍËÚÂ, ̇ÚÓ‚‡‚‡˘Ë ‡·‰ÓÏË̇Î̇ڇ Ë Ô‡‡‚ÂÚ·‡Î̇ڇ ÎÛÏ·‡Î̇ ÏÛÒÍÛ·ÚÛ‡ Ò ÔÂÔÓ˙˜‚‡Ú. 臈ËÂÌÚËÚ Úfl·‚‡ ‰‡ Ò ̇Ò˙˜‡‚‡Ú ‰‡ ÔÓ‰˙Ê‡Ú ÌÓχÎÌÓ ÚÂÎÂÒÌÓ Ú„ÎÓ, ÍÓÂÚÓ ÔÓÌflÍÓ„‡  ÚÛ‰ÌÓ ÔÓÒÚËÊËχ ˆÂÎ ÔË ‚˙Á‡ÒÚÌË ËÌ‰Ë‚Ë‰Ë (5, 6, 9). ä‡ÚÓ ˆflÎÓ ÔÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ‰Â„ÂÌÂ‡Ú˂̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ Ë ÒÔË̇Î̇ ÒÚÂÌÓÁ‡ Ò ÔÓ‰Ó·fl‚‡Ú ÓÚ ÔË·„‡Ì‡Ú‡ ÍÓÌÒÂ‚‡Ú˂̇ ÚÂ‡ÔËfl (6, 9). ñÂÎÚ‡ ̇ ıËÛ„˘ÌÓÚÓ Î˜ÂÌË  ӷÎÂ͘‡‚‡Ì ̇ ·ÓÎ͇ڇ, ˜‡ÒÚ˘̇ ËÎË Ô˙Î̇ ‰Û͈Ëfl ̇ Ì‚ÓÎӄ˘ÌËfl ‰ÂÙˈËÚ Ë ÔÓ‰Ó·ÂÌÓ Í‡˜ÂÒÚ‚Ó Ì‡ ÊË‚ÓÚ. èËÂÚË Ò‡ ÒΉÌËÚ Ë̉Ë͇ˆËË Á‡ ÓÔÂ‡ÚË‚ÌÓ Î˜ÂÌË ÔË ‰Â„ÂÌÂ‡Ú˂̇ ÒÚÂÌÓÁ‡ Ë ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ (6): 1. èÂÒËÒÚË‡˘‡ ËÎË ˆˉ˂Ë‡˘‡ ·ÓÎ͇ ‚ ‰ÓÎÌËfl Í‡ÈÌËÍ, ‚˙ÔÂÍË ÍÓÌÒÂ‚‡Ú˂̇ ÚÂ‡ÔËfl (ÏËÌËÏÛÏ 3 ÏÂÒˆ‡). 2. èÓ„ÂÒË‡˘ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ. ífl·‚‡ ‚ÌËχÚÂÎÌÓ ‰‡ Ò ËÁÍβ˜‡Ú ‚Ò˘ÍË ÓÒڇ̇ÎË ‚˙ÁÏÓÊÌË Ô˘ËÌË ÓÒ‚ÂÌ ÒÔË̇Î̇ڇ ÒÚÂÌÓÁ‡. 3. ëË„ÌËÙË͇ÌÚÌÓ ÒÌËÊÂÌË ̇ ͇˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ. 4.èÓÚ‚˙Ê‚‡˘Ë ÍÎËÌ˘̇ڇ ͇ÚË̇ Ì‚ÓËÁÓ·‡ÁËÚÂÎÌË ËÁÒΉ‚‡ÌËfl. ä·Ò˘ÂÒÍÓÚÓ ıËÛ„˘ÂÒÍÓ Î˜ÂÌË  ‰ÂÍÓÏÔÂÒ˂̇ڇ ·ÏËÌÂÍÚÓÏËfl. èÂÁ 1985 „. J. Lombardi Ë Ò˙‡‚Ú. (9)


‰ÂÍÂÏ‚Ë, 2007

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

‰ÓÍ·‰‚‡Ú ÂÁÛÎÚ‡ÚË ÔË 47 Ô‡ˆËÂÌÚ‡, ÍÓËÚÓ Ò‡ ÓÔÂË‡ÌË ÔÓ ÔÓ‚Ó‰ ̇ ÒÔË̇Î̇ ÒÚÂÌÓÁ‡ ‚ ÍÓÏ·Ë̇ˆËfl Ò ‰Â„ÂÌÂ‡Ú˂̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. í ÓÚ·ÂÎflÁ‚‡Ú, ˜Â ·ÓÎÌËÚ ÔË ÍÓËÚÓ ÓÒ‚ÂÌ ‡‰Ë͇Î̇ ·ÏËÌÂÍÚÓÏËfl Ò‡ ÓÚÒÚ‡Ìfl‚‡ÌË Ë Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë Ò‡ ËχÎË ÔÓ-ÎÓ¯ ËÁıÓ‰ Á‡ ‡ÁÎË͇ ÓÚ ÚÂÁË, ÔË ÍÓËÚÓ Ù‡ÒÂÚÌËÚ ÒÚ‡‚Ë Ò‡ Á‡Ô‡Á‚‡ÌË. ç‡È-‰Ó· Ò ÔÓ‚ÎËfl‚‡Ú Ô‡ˆËÂÌÚËÚÂ, ÔË ÍÓËÚÓ ÓÒ‚ÂÌ ‰ÂÍÓÏÔÂÒ˂̇ ·ÏËÌÂÍÚÓÏËfl Ë Á‡Ô‡ÁÂÌË Ù‡ÒÂÚÌË ÒÚ‡‚Ë Â ÓÒ˙˘ÂÒÚ‚fl‚‡Ì‡ ÔÓÒÚÂÓ·ÚÂ‡Î̇ ËÌÚÂÚ‡ÌÒ‚ÂÁ‡Î̇ ‡ÚÓ‰ÂÁ‡. èÂÁ 1985 „. ä. Johnsson Ë Ò˙‡‚Ú. (7) ‰ÓÍ·‰‚‡Ú 45 ÒÎÛ˜‡fl, ÔË ÍÓËÚÓ Â ÓÒ˙˘ÂÒÚ‚fl‚‡Ì‡ ‰ÂÍÓÏÔÂÒ˂̇ ÎËÌÂÍÚÓÏËfl ÔÓ ÔÓ‚Ó‰ ̇ ÒÔË̇Î̇ ÒÚÂÌÓÁ‡, ͇ÚÓ ‰‚‡‰ÂÒÂÚ ÓÚ Úflı Ò‡ ·ËÎË Ò ‰Â„ÂÌÂ‡Ú˂̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. èË ÚË̇‰ÂÒÂÚ ÓÚ Úflı ÔÓ-̇ڇÚ˙¯ÌÓÚÓ ÔÓÒΉfl‚‡Ì  ÔÓ͇Á‡ÎÓ ÔÓ‰˙Îʇ‚‡˘‡ ÎËÒÚÂÁ‡ ̇ Ô¯ÎÂÌËÚÂ, ͇ÚÓ ÔÓ‚ÚÓ̇ڇ ıËÛ„˘̇ ËÌÚÂ‚Â̈Ëfl Ì ·Ë· Ò ‰Ó·˙ ÂÁÛÎÚ‡Ú, ‡ ÔÓ‰Ó·ÂÌË  ÓÚ·ÂÎflÁ‡‚ÌÓ ‚ 52% ÓÚ ÒÎÛ˜‡ËÚÂ. èÂÁ 1991 „. H.N. Herkowitz, L.T. Kurz (5) Ò‡‚Ìfl‚‡Ú Ò‡ÏÓÒÚÓflÚÂÎ̇ڇ ‰ÂÍÓÏÔÂÒ˂̇ ·ÏËÌÂÍÚÓÏËfl Ë Ú‡ÁË Ò ÔÓÒÚÂÓ·ÚÂ‡Î̇ ‡ÚÓ‰ÂÁ‡ Ë ‡‚ÚÓ„ÂÌ̇ ÍÓÒÚ̇ ÔËÒ‡‰Í‡ ÓÚ ı˙ηӘ̇ ÍÓÒÚ. êÂÁÛÎÚ‡ÚËÚ ÔÓ͇Á‚‡Ú ÔÓ‰Ó·˙ ËÁıÓ‰, Ò ÔÓ-χÎ˙Í ËÒÍ ÓÚ ‡Á‚ËÚË ̇ ÌÓ‚‡ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ Ë ÎÛÏ·‡Î̇ ÌÂÒÚ‡·ËÎÌÓÒÚ ÔË ÍÓÏ·ËÌË‡Ì‡ ·ÏËÌÂÍÚÓÏËfl. ÇÒ˘ÍË ÔÓÒΉ‚‡˘Ë ÔÓÛ˜‚‡ÌËfl ÔÓÚ‚˙ʉ‡‚‡Ú ÔÓ-‰Ó·ËÚ ÂÁÛÎÚ‡ÚË ÔË ÍÓÏ·ËÌË‡Ì ̇ ·ÏËÌÂÍÚÓÏËflÚ‡ Ò ‡ÚÓ‰ÂÁ‡ (3, 13). äÓÏ·Ë̇ˆËflÚ‡ ̇ ·ÏËÌÂÍÚÓÏËfl Ò ‡ÚÓ‰ÂÁ‡ Ë ‡‚ÚÓ„ÂÌ̇ ÔËÒ‡‰Í‡ ��˜Â‚ˉÌÓ ÔÓ‰Ó·fl‚‡ ËÁıÓ‰‡ ÓÚ ÓÔÂ‡Ú˂̇ڇ ËÌÚÂ‚Â̈Ëfl. íÓ‚‡ Ó·‡˜Â Ì ÓÁ̇˜‡‚‡, ˜Â Ò‡ÏÓÒÚÓflÚÂÎ̇ڇ ‰ÂÍÓÏÔÂÒËfl Ì ÏÓÊ ‰‡ ‰‡‰Â ‰Ó·˙ ÂÁÛÎÚ‡Ú. èË Ô‡ˆËÂÌÚË Ò ËÁ‡ÁÂÌ ÓÒÚÂÓ‡ÚËÚ Ë ˄ˉÂÌ „˙·Ì‡Í ÔÓ„ÂÒË‡˘‡Ú‡ ÌÂÒÚ‡·ËÎÌÓÒÚ ÒΉ Ò‡ÏÓÒÚÓflÚÂÎ̇ ·ÏËÌÂÍÚÓÏËfl  Ì‚˙ÁÏÓÊ̇. ÑÓÍ·‰‚‡Ú Ò 31 ‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚ‡, ÔË ÍÓËÚÓ Â ËÁ‚˙¯Â̇ Ò‡ÏÓÒÚÓflÚÂÎ̇ ·ÏËÌÂÍÚÓÏËfl ÔÓ ÔÓ‚Ó‰ ̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡ Ë ‚ 84%  ‰ÓÍ·‰‚‡Ì ÛÒÔ¯ÂÌ ıËÛ„˘ÂÌ ÂÁÛÎÚ‡Ú, ͇ÚÓ ÔË ÌËÚÓ Â‰ËÌ ÒÎÛ˜‡È Ì ÔÓÒΉ‚‡Î‡ ÎËÒÚÂÁ‡ (15). LJÊÂÌ ‚˙ÔÓÒ ‚ Ô‡ÍÚ˘ÂÌ ÔÎ‡Ì Â ÚÓÁË Á‡ ÓÎflÚ‡ ̇ ÒÔË̇Î̇ڇ ËÌÒÚÛÏÂÌÚ‡ˆËfl ÔË Î˜ÂÌËÂÚÓ Ì‡ ·ÓÎÌËÚ Ò˙Ò ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. ñÂΠ̇ ‚˙Ú¯̇ڇ ÙËÍÒ‡ˆËfl  ‰‡ Ò ÛÒÍÓË Á‡Á‰‡‚fl‚‡ÌÂÚÓ Ë ‰‡ Ò ̇χÎË ·Ófl ̇ ÌË‚‡Ú‡, ̇·„‡˘Ë ËÁ‚˙¯‚‡Ì ̇ ÙËÍÒ‡ˆËfl Ë ‰‡ Ò Ò˙Í‡ÚË ‚ÂÏÂÚÓ Á‡ Âı‡·ËÎËÚ‡ˆËfl. á‰‡‚Ë̇ڇ Ë ÛÒÚÓȘ˂ÓÒÚÚ‡ ̇ ÙËÍÒ‡ˆËflÚ‡  Á̇˜ËÚÂÎÌÓ ÔÓ-‰Ó·‡ ÓÚ ·ËÓÏÂı‡Ì˘̇ „Ή̇ ÚӘ͇. çÂ˘Ó Ôӂ˜Â, ÎËÔÒ‡Ú‡ ̇ ·ÏË̇ ÒΉ ·ÏËÌÂÍÚÓÏËfl Ë Ì‡Î˘ËÂÚÓ Ì‡ ÓÒÚÂÓÔÂÌËfl ÔË ‚˙Á‡ÒÚÌË ·ÓÎÌË Â Ó˘Â Â‰ÌÓ ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ó·ÒÚÓflÚÂÎÒÚ‚Ó, ÍÓÂÚÓ ÓÔ‡‚‰‡‚‡ ÔËÎÓÊÂÌËÂÚÓ Ì‡ ÒÔË̇Î̇ڇ ËÌÒÚÛÏÂÌÚ‡ˆËfl. Ç ÎËÚÂ‡ÚÛ‡Ú‡ ˜ÂÒÚÓ Ò ‰ËÒÍÛÚË‡ ‚˙ÔÓÒ˙Ú, ‰‡ÎË ÍÓÏ·ËÌË‡ÌÂÚÓ Ì‡ ÒÔË̇Î̇ ËÌÒÚÛÏÂÌÚ‡ˆËfl Ò ËÌÚÂÚ‡ÌÒ‚ÂÁ‡Î̇ڇ ‡ÚÓ‰ÂÁ‡ ÔÓ‰Ó·fl‚‡ ÍÎËÌ˘ÌËfl ËÁıÓ‰ Ë Ò˙Í‡˘‡‚‡ ÓÁ‰‡‚ËÚÂÎÌËfl ÔÓÒÚÓÔÂ‡ÚË‚ÂÌ ÔÂËÓ‰? íÓ‚‡  ‚‡ÊÂÌ ÔÓ·ÎÂÏ, ͇ÚÓ Ò Ëχ Ô‰‚ˉ ÔÓ-„ÓÎÂÏËÚ ‡ÁıÓ‰Ë Ë ‚˙ÁÏÓÊÌËÚ ÛÒÎÓÊÌÂÌËfl ÒΉ ÒÔË̇Î̇ ËÌÒÚÛÏÂÌÚ‡ˆËfl (5, 6). í‚˙‰Â χÎÍÓ Ò‡ ÔÓÛ˜‚‡ÌËflÚ‡

218

Á‡ ÔÓÎÁ‡Ú‡ ÓÚ ÒÔË̇Î̇ ËÌÒÚÛÏÂÌÚ‡ˆËfl ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒÔË̇Î̇ ÒÚÂÌÓÁ‡ ‡ÒÓˆËË‡Ì‡ Ò ‰Â„ÂÌÂ‡Ú˂̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡. ä. Bridwell Ë Ò˙‡‚Ú. (2), ͇ÍÚÓ Ë T.A. Zdeblick (17) ÛÒÚ‡ÌÓ‚fl‚‡Ú, ˜Â ÔÓÒÚÓÔÂ‡ÚË‚ÌÓÚÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì  Á̇˜ËÏÓ ÔÓ-·˙ÁÓ ÔË ·ÓÎÌËÚÂ Ò ËÌÒÚÛÏÂÌÚ‡Î̇ ÙÛÁËfl. Ç Á‡Íβ˜ÂÌËÂ, ‰Â„ÂÌÂ‡Ú˂̇ڇ ÎÛÏ·‡Î̇ ÒÔÓ̉ËÎÓÎËÒÚÂÁ‡  ÏÌÓ„Ó ÔÓ-˜ÂÒÚ‡ ÔË ‚˙Á‡ÒÚÌË ÊÂÌË Ì‡‰ 50 „Ó‰ËÌË, ͇ÚÓ ·ÓίËÌÒÚ‚ÓÚÓ ÓÚ ÒÎÛ˜‡ËÚ Ò ÔÓ‚ÎËfl‚‡Ú ÓÚ ÔË·„‡Ì‡Ú‡ ÍÓÌÒÂ‚‡Ú˂̇ ÚÂ‡ÔËfl. ïËÛ„˘̇ڇ ËÌÚÂ‚Â̈Ëfl Ò ÔÂÔÓ˙˜‚‡ ÔË ÌÂÔÓ‚ÎËfl‚‡˘Ë Ò ·ÓÎÍË, ÔÓ„ÂÒË‡˘ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ Ë ‚ÎÓ¯ÂÌÓ Í‡˜ÂÒÚ‚Ó Ì‡ ÊË‚ÓÚ. ç‡È-ÂÙÂÍÚ˂̇  ÍÓÏ·Ë̇ˆËflÚ‡ ÓÚ ‰ÂÍÓÏÔÂÒ˂̇ ·ÏËÌÂÍÚÓÏËfl Ë ËÌÚÂÚ‡ÌÒ‚ÂÁ‡Î̇ ‡ÚÓ‰ÂÁ‡ Ò ‡‚ÚÓ„ÂÌÂÌ ÍÓÒÚÂÌ ÔËÒ‡‰˙Í. ÑÓÔ˙ÎÌËÚÂÎ̇ڇ ÒÔË̇Î̇ ËÌÒÚÛÏÂÌÚ‡ˆËfl ÛÒÍÓfl‚‡ ÔÓÒÚÓÔÂ‡ÚË‚ÌËÚ ‚˙ÁÒÚ‡ÌÓ‚ËÚÂÎÌË ÔÓˆÂÒË. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5.

6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17.

Bolesta, M., Bohlman, H. Degenerative spondylolisthesis. In: Instructional Course Lecture. Prk Ridge, IL: American Academy of Orthopaedic Surgeons, 1989, 38, 1-20. Bridwell, K., Sedgewick, T., O,Brien, M, Lenke, L., Baldus, C. The role of fusion and instrumentation in the treatment of degeneratie spondylolsthesis with spinal stenosis. Journal of Spinal Disorders, 1993, 6, 467-472. Caputy, A., Lessenhop, A. Long term evaluation of decompressive surgery for degenrative lumbar stenosis. J Neurosurg, 1992, 77, 669-676. Grobler, L., Robertson, P., Novotny, J., Pope, M. Etiology of spondylolistesis. Assessment of the role plyed by lumbar facet joint morphology. Spine, 1993, 18, 80-92. Herkowitz, H.N., Kurtz, L.T. Degenerative lumbar pondyllistesis with spinal stenosis. A prospective study comparing decompression with decompression and intertransverse progressive arthrodesis. J Bone Joint Surg, 1991, 73, 802808. Herkowitz, H.N. Spinal stenosis: Clinical evaluation. In: American Academy of Orthopaedic Surgeons Instructional Course Lectures XLI. Eilert, R.E. ed. Park Ridge: American Academy of Orthopaedic Surgeons, 1992, 183-185. Johnsson, K., Wilner, S., Johnsson, K. Prospectve instability after decompression for lumbar spinal stenosis. Spine, 1986, 11, 107-110. Junghanns, H. Spondylisthesis ohne spalt in zwischengelenstuck. Archiv fr Orthopaedische und Unfall-Chirurgie, 1930, 29, 118-127. Lombardi, J., Wiltse, L., Reynolds, J., Widell, E.,Spencer, C. Treatment of degenerative sponylolisthesis. Spine, 1985, 10, 821-827. Matsunaga, S., Sakou, T., Morizono, Y., Masuda, A., Demirtas, A. Natural history of degenerative spondylolisthesis. Spine, 1990, 15, 1204-1210. Nachemson, A. Lumbar interdiscal pressure. Acta Orthop Scand, 1960, 43(Suppl), 140. Newman, P.H. Stenosis of the lumbar spine in spondylolisthesis. Clin Orthop, 1976, 115, 116-121. Postacchini, F., Cinnot, G. Bone regrowth after surgical decompression for lumbar spinal stenosis. J Bone Joint Surg, 1992, 74, 862-869. Rosenberg, N.J. Degenerative spondylolisthesis. J Bone Joint Surg, 1975, 57, 467-474. Sanderson, P.L., Wood, P.L. Surgery for lumbar spinal stenosis in old people, 1993, 75B, 393-397. Yang, K., King, A. Mecanism of facet load transmission as a hypothesis for low back pain. Spine, 1984, 9, 557-565. Zdeblick, T. A prospective randomized study of lumbar fusion. Spine, 1993, 18, 983-991.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- è·ÏÂÌ ñ‚ÂÚ‡ÌÓ‚ ÉÂÓ„Ë‚, ‰.Ï. è΂ÂÌ 5800, ÛÎ.”É. äӘ‚” 8Ä, ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡, ä‡Ú‰‡ ÔÓ ç‚ÓÎÓ„Ëfl Ë ç‚ÓıËÛ„Ëfl íeÎ.: (064835242, e-mail: tzvetanovplamen@hotmail.com


‰ÂÍÂÏ‚Ë, 2007

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

219

é·ÁÓ çÖîÄêåÄäéãéÉàóçÄ çÖÇêéèêéíÖäñàü èêà àëïÖåàóÖç åéáöóÖç àçëìãí Ñ. å‡Ò·Ó‚, Ç. ä‡Î‡È‰ÊË‚ è˙‚‡ åÅÄã – ëÓÙËfl, ç‚ÓÎӄ˘ÌÓ ÓÚ‰ÂÎÂÌËÂ

SUMMARY NON-PHARMACOLOGICAL NEURéPROTECTION IN ACUTE STROKE D. Maslarov, V. Kalaidjiev Neurology Department, First City Hospital - Sofia Despite many years of research, treatment and prevention of ischemic stroke continue to be one of the major challenges in current medicine. Ischemic stroke is a dynamic process, where a series of excitotoxic, inflammatory and microvascular mechanisms take place that lead to tissue necrosis. Pharmacological neuroprotective treatment has not yet demonstrated the clinical effectiveness observed in experimental models. The concept of neuroprotection in cerebral ischemia also involves a series of mechanisms that take place at the cerebral level following vascular occlusion. In this context, it should be borne in mind that a series of physiological functions usually involved in the cerebral metabolism (control of blood pressure, temperature, glycemia and arterial oxygen saturation) play a key role in modulation of the ischemic process. A relationship between hyperthermia and ischemic stroke has not been completely demonstrated and raises questions that have been yet to be resolved. There is currently no evidence from randomized trials to support the routine use of chemical or physical cooling therapy in acute stroke. Hyperglycemia is associated with a worse stroke outcome. Current data suggest that hyperglycemia is a predictor of outcome and mortality independently of age, stroke subtype and severity. The prognostic influence of blood pressure during the acute phase of ischemic stroke is still a matter of controversy. High blood pressure may induce the formation of brain edema, hemorrhagic transformation and further vascular damage. When treatment is indicated, lowering pressure should be done cautiously. Maintaining adequate tissue oxygenation seems to be of great importance during periods of acute cerebral ischemia in order to prevent hypoxia and potential worsening of the neurological injury. Closely related to arterial saturation is control of the volemia and viscosity of the blood. Non-pharmacolocical neuroprotection includes all interventions protecting the brain from pharmacological damage after cerebral vascular occlusion. The effectiveness of Stroke Units for the protocolized care of patients is well established now with a reduction of mortality, reduced evolution and a lower hospitalization rate. KEY WORDS: neuroprotection, hyperthermia, hyperglycemia, blood pressure, tissue oxygenation, Stroke Unit. êÖáûåÖ éÍÎÛÁËflÚ‡ ̇ ÌflÍÓfl ÓÚ ˆÂ·‡ÎÌËÚ ‡ÚÂËË ÒÚ‡ÚË‡ ͇Ò͇‰‡ ÓÚ ÍÎÂÚ˙˜ÌË Ë ÏÂÚ‡·ÓÎËÚÌË ÔÓˆÂÒË, ÍÓËÚÓ ‚ÎËflflÚ Í‡ÍÚÓ ‚˙ıÛ ÙÛÌ͈ËflÚ‡ Ë ÒÚÛÍÚÛ‡Ú‡ ̇ Ì‚ÓÌËÚÂ, „ÎËflÚ‡ Ë ẨÓÚÂÎÌËÚ ÍÎÂÚÍË, ڇ͇ Ë Ì‡

ÂÍÒÚ‡ˆÂÎÛ·ÌÓÚÓ ÔÓÒÚ‡ÌÒÚ‚Ó. ìÒËÎËflÚ‡ ̇ ËÁÒΉӂ‡ÚÂÎË Ë ÎÂ͇Ë ‰ÂÒÂÚÍË „Ó‰ËÌË Ò‡ Ò˙Ò‰ÓÚÓ˜ÂÌË ‚˙ıÛ Ó·Ó„‡Úfl‚‡Ì ̇ ÔÓÁ̇ÌËÂÚÓ ‚˙ıÛ ÍÓÏÔÎÂÍÒÌËÚ ÏÂı‡ÌËÁÏË Ì‡ ˆÂ·‡Î̇ڇ ËÒıÂÏËfl Ë ‚˙ÁÏÓÊÌÓÒÚËÚ Á‡ ÚflıÌÓÚÓ ÔÓ‚ÎËfl‚‡ÌÂ. î‡χÍÓÎӄ˘ÌËÚ Ì‚ÓÔÓÚÂÍÚÓË Ì ҇ ‰Ó͇Á‡ÎË Ô‰ÔÓ·„‡Âχڇ ÍÎËÌ˘̇ ÂÙÂÍÚË‚ÌÓÒÚ, ÍÓflÚÓ Â ‰Ó͇Á‡Ì‡ ÔË ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÏÓ‰ÂÎË. äÓ̈ÂÔˆËflÚ‡ Á‡ Ì‚ÓÔÓÚÂ͈Ëfl ÔË ˆÂ·‡Î̇ ËÒıÂÏËfl ‚Íβ˜‚‡ ‚˙ÁÏÓÊÌÓÒÚË Á‡ ÔÓ‚ÎËfl‚‡Ì ‚˙ıÛ ÒÂËfl ÓÚ ÏÂı‡ÌËÁÏË, ÍÓËÚÓ Ò ‡Á‚Ë‚‡Ú ̇ ÍÎÂÚ˙˜ÌÓ Ë ÒÛ·ÍÎÂÚ˙˜ÌÓ ÌË‚Ó ÒΉ Ò˙‰Ó‚‡ ÓÍÎÛÁËfl. Ç ÚÓÁË ÍÓÌÚÂÍÒÚ Úfl·‚‡ ‰‡ Ò ËÏ‡Ú Ô‰ ‚ˉ ÒÂËfl ÓÚ ÙÛÌ͈ËË, ÍÓËÚÓ Ë„‡flÚ Íβ˜Ó‚‡ ÓÎfl ‚ ÏÓ‰ÛÎË‡Ì ̇ ËÒıÂÏ˘ÌËfl ÔÓˆÂÒ: ÍÓÌÚÓΠ̇ ‡ÚÂˇÎÌÓÚÓ Ì‡Îfl„‡ÌÂ, ÚÂÏÔÂ‡ÚÛ‡Ú‡, ÌË‚ÓÚÓ Ì‡ Í˙‚̇ڇ Á‡ı‡ Ë ‡ÚÂˇÎ̇ڇ ÍËÒÎÓӉ̇ Ò‡ÚÛ‡ˆËfl. ÑËÂÍÚ̇ڇ ‚˙Á͇ ÏÂÊ‰Û ıËÔÂÚÂÏËfl Ë ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Ì  ‰ÌÓÔÓÒӘ̇ Ë ‚Ò Ӣ Ëχ ‚˙ÔÓÒË, ÍÓËÚÓ ˜‡Í‡Ú ÓÚ„Ó‚Ó. 阇͂‡Ú Ò ÂÁÛÎÚ‡ÚË ÓÚ ÍÓÌÚÓÎË‡ÌË ÔÓÛ˜‚‡ÌËfl, ÍÓËÚÓ ‰‡ ÔÓÚ‚˙‰flÚ ÛÚËÌ̇ڇ ÛÔÓÚ·‡ ̇ ıËÏ˘ÂÒ͇ ËÎË ÙËÁË͇Î̇ ıËÔÓÚÂÏËfl. ïËÔÂ„ÎËÍÂÏËflÚ‡ Ò ҂˙Á‚‡ Ò ‚ÎÓ¯‡‚‡Ì ̇ ËÁıÓ‰‡ ÓÚ ËÌÒÛÎÚ‡ Ë Ôӂ˯‡‚‡Ì ̇ ÒÏ˙ÚÌÓÒÚÚ‡ ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‚˙Á‡ÒÚ, ÔÓ‰‚ˉ‡ ̇ ËÌÒÛÎÚ‡ ËÎË ÚÂÊÂÒÚÚ‡. èÓ„ÌÓÒÚ˘̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ ‡ÚÂˇÎÌÓÚÓ Ì‡Îfl„‡Ì ÔÂÁ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ËÌÒÛÎÚ‡ ‚Ò Ӣ  Ô‰ÏÂÚ Ì‡ ÒÔÓÓ‚Â. ÇËÒÓÍÓÚÓ ‡ÚÂˇÎÌÓ Ì‡Îfl„‡Ì ÏÓÊ ‰‡ Ô‰ËÁ‚Ë͇ Ó·‡ÁÛ‚‡ÌÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ ÓÚÓÍ, ıÂÏÓ‡„˘̇ Ú‡ÌÒÙÓχˆËfl Ë ÔÓ-̇ڇÚ˙¯Ì‡ Ò˙‰Ó‚‡ Û‚‰‡. èÓ‰‰˙ʇÌÂÚÓ Ì‡ ‡‰ÂÍ‚‡Ú̇ Ú˙͇Ì̇ ÍËÒÎÓӉ̇ ÓÍÒË„Â̇ˆËfl Ò˙˘Ó Ëχ „ÓÎflÏÓ Á̇˜ÂÌË ‚ ÓÒÚËfl ÔÂËÓ‰ Ò Ó„Î‰ Ô‚Â̈Ëfl ̇ ÏÓÁ˙˜Ì‡Ú‡ ıËÔÓÍÒËfl. èflÍÓ Ò‚˙Á‡ÌË Ò „ÓÌÓÚÓ Ò‡ ÍÓÌÚÓ· ‚˙ıÛ ‚ÓÎÂÏËflÚ‡ Ë ‚ËÒÍÓÁËÚÂÚ‡ ̇ Í˙‚Ú‡. çÂÙ‡χÍÓÎӄ˘̇ڇ Ì‚ÓÔÓÚÂ͈Ëfl ‚Íβ˜‚‡ ‚Ò˘ÍË ËÌÚÂ‚Â̈ËË, ÍÓËÚÓ Ô‰ԇÁ‚‡Ú ÏÓÁ˙͇ ÓÚ Ù‡χÍÓÎӄ˘̇ Û‚‰‡ ÒΉ ÓÍÎÛÁËfl ̇ ˆÂ·‡Î̇ ‡ÚÂËfl. ÖÙÂÍÚË‚ÌÓÒÚÚ‡ ÓÚ Ò˙Á‰‡‚‡ÌÂÚÓ Ì‡ Stroke units ‚˜  ·ÂÁÒÔÓ̇, Ú˙È Í‡ÚÓ ÔË·„‡ÌÂÚÓ Ì‡ ÔÓÚÓÍÓÎÌË „ËÊË Á‡ Ô‡ˆËÂÌÚËÚ ‚ Úflı, ̇‰ Ò ‡‰ÂÍ‚‡ÚÌÓ ÚÂ‡Ô‚Ú˘ÌÓ Ôӂ‰ÂÌË ‚Ó‰flÚ ‰Ó ̇χÎfl‚‡Ì ̇ ÒÏ˙ÚÌÓÒÚÚ‡, Ó„‡Ì˘‡‚‡Ì ‚ÓβˆËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, Ò˙Í‡˘‡‚‡Ì ̇ ıÓÒÔËÚ‡ÎËÁ‡ˆËÓÌÌÓÚÓ ‚ÂÏÂ Ë Ò˙Á‰‡‚‡Ì ̇ ÔÓ-·Î‡„ÓÔËflÚ̇ ·‡Á‡ Á‡ ‡ÌÌÓ Á‡ÔÓ˜‚‡Ì ̇ Âı‡·ËÎËÚ‡ˆËfl. äãûóéÇà Ñìåà: Ì‚ÓÔÓÚÂ͈Ëfl, ıËÔÂÚÂÏËfl, ıËÔÂ„ÎËÍÂÏËfl, ‡ÚÂˇÎÌÓ Ì‡Îfl„‡ÌÂ, ÍËÒÎÓӉ̇ Ò‡ÚÛ‡ˆËfl, ÓÚ‰ÂÎÂÌËfl Á‡ ΘÂÌË ̇ ÓÒÚ˙ ËÌÒÛÎÚ. éÍÎÛÁËflÚ‡ ̇ ÌflÍÓfl ÓÚ ˆÂ·‡ÎÌËÚ ‡ÚÂËË ÒÚ‡ÚË‡ ͇Ò͇‰‡ ÓÚ ÍÎÂÚ˙˜ÌË Ë ÏÂÚ‡·ÓÎËÚÌË ÔÓˆÂÒË, ÍÓËÚÓ ‚ÎËflflÚ Í‡ÍÚÓ ‚˙ıÛ ÙÛÌ͈ËflÚ‡ Ë ÒÚÛÍÚÛ‡Ú‡ ̇ Ì‚ÓÌËÚÂ, „ÎËflÚ‡ Ë ẨÓÚÂÎÌËÚ ÍÎÂÚÍË, ڇ͇ Ë Ì‡ ÂÍÒÚ‡ˆÂÎÛ·ÌÓÚÓ ÔÓÒÚ‡ÌÒÚ‚Ó. ìÒËÎËflÚ‡ ̇ ËÁÒΉӂ‡ÚÂÎË Ë ÎÂ͇Ë ‰ÂÒÂÚÍË „Ó‰ËÌË Ò‡ Ò˙Ò‰ÓÚÓ˜ÂÌË ‚˙ıÛ Ó·Ó„‡Úfl‚‡Ì ̇ ÔÓÁ̇ÌËÂÚÓ ‚˙ıÛ ÍÓÏÔÎÂÍÒÌËÚ ÏÂı‡-


‰ÂÍÂÏ‚Ë, 2007

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

ÌËÁÏË Ì‡ ˆÂ·‡Î̇ڇ ËÒıÂÏËfl Ë ‚˙ÁÏÓÊÌÓÒÚËÚ Á‡ ÚflıÌÓÚÓ ÔÓ‚ÎËfl‚‡ÌÂ. èӂ‰ÂÌË Ò‡ Ôӂ˜ ÓÚ 140 ÍÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl Ò Ì‡‰ 250 ÎÂ͇ÒÚ‚‡, Ò‡ÏÓÒÚÓflÚÂÎÌÓ ËÎË ‚ ÍÓÏ·Ë̇ˆËË, „ÓÎflχ ˜‡ÒÚ ÓÚ ÍÓËÚÓ Ò‡ ÏÓÎÂÍÛÎË Ò Ô‰ÔÓ·„‡Âχ Ì‚ÓÔÓÚÂÍÚ˂̇ ‡ÍÚË‚ÌÓÒÚ. ꂇÒÍÛ·ËÁ‡ˆËflÚ‡, ÔÓÒÚ˄̇ڇ Ò ÛÚËÌÌÓÚÓ ‚˙‚Âʉ‡Ì ̇ ÚÓÏ·ÓÎËÚ˘̇ ÚÂ‡ÔËfl Ëχ ÏÌÓ„Ó Ó„‡Ì˘ÂÌËfl, ͇ÍÚÓ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ‚ÂÏ ̇ ÔËÎÓÊÂÌËÂ, ڇ͇ Ë ÔË ÔÓ‰·Ó‡ ̇ Ô‡ˆËÂÌÚË. Ç Ú‡ÁË ‚˙Á͇ Ù‡χÍÓÎӄ˘̇ڇ Ë ÌÂÙ‡χÍÓÎӄ˘̇ڇ Ì‚ÓÔÓÚÂ͈Ëfl ·Ë ·Ë· ËÁÍβ˜ËÚÂÎÌÓ ÔÓÎÂÁ̇ ‚ ÌflÍÓÎÍÓ Ì‡Ô‡‚ÎÂÌËfl: ● ÔÓ‰‰˙ʇÌ ÊËÁÌÂÒÔÓÒÓ·ÌÓÒÚÚ‡ ̇ ËÒıÂÏËÁË‡Ì‡Ú‡ Ú˙Í‡Ì ÔÓ-‰˙΄Ó, Ò ÍÓÂÚÓ Ò ۉ˙Îʇ‚‡ ÚÂ‡Ô‚Ú˘ÌËfl ÔÓÁÓˆ Á‡ ÚÓÏ·ÓÎËÁ‡; ● ËÌıË·Ë‡Ì ËÎË ÍÓÌÚÓÎË‡Ì ̇ ÒÚ‡Ì˘ÌËÚ ‡͈ËË, ÔÓËÁÎËÁ‡˘Ë ÓÚ ÂÔÂÙÛÁËflÚ‡; ● ̇χÎfl‚‡Ì ËÒ͇ ÓÚ ‡Á‚ËÚË ̇ ÏÓÁ˙˜Ì‡ ıÂÏÓ‡„Ëfl ÔË ÚÓÏ·ÓÎËÁË‡ÌË Ô‡ˆËÂÌÚË. èÂÚÂ̈ËË Á‡ Ì‚ÓÔÓÚÂÍÚ˂̇ ÂÙÂÍÚË‚ÌÓÒÚ Ò‡ Ô‰fl‚fl‚ËÎË ‡Á΢ÌË „ÛÔË ÎÂ͇ÒÚ‚‡: ÏÓ‰Û·ÚÓË Ì‡ C‡++ ͇̇ÎË, ÏÓ‰Û·ÚÓË Ì‡ Na++ ͇̇ÎË, ‡ÌÚ‡„ÓÌËÒÚË Ì‡ NMDA ˆÂÔÚÓË, ‡„ÓÌËÒÚË Ì‡ GABA ˆÂÔÚÓË, ‡ÌÚËÓÍÒˉ‡ÌÚË (ÔËı‚‡˘‡˜Ë ̇ Ò‚Ó·Ó‰ÌË ‡‰Ë͇ÎË), ÚÓÙ˘ÌË Ù‡ÍÚÓË (ÏÂÏ·‡ÌÌË ÒÚ‡·ËÎËÁ‡ÚÓË), ‡ÒÚÂÊÌË Ù‡ÍÚÓË, ˆËÚÓÍËÌË, ÒÎÂÔ‚‡˘Ë ÏÓÎÂÍÛÎË, ‡‰ÂÌÓÁËÌÓ‚Ë ‡ÌÚ‡/‡„ÓÌËÒÚË Ë ‰. ᇠÒ˙ʇÎÂÌË Ôӂ˜ÂÚÓ ÓÚ Úflı Ì ҇ ‰Ó͇Á‡ÎË Ô‰ÔÓ·„‡Âχڇ ÍÎËÌ˘̇ڇ ÂÙÂÍÚË‚ÌÓÒÚ, ÍÓflÚÓ Â ÓÚ·ÂÎflÁ‡Ì‡ ÔË ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÏÓ‰ÂÎË. Ç ÔÛ·ÎËÍÛ‚‡Ì ÔÂÁ 2004 „Ó‰Ë̇ ÏÂÚ‡-‡Ì‡ÎËÁ L. Labiche Ë J. Grotta (52) Ó·Ó·˘‡‚‡Ú ÂÁÛÎÚ‡ÚËÚ ÓÚ ÔÛ·ÎËÍÛ‚‡ÌËÚ ÍÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl. êÂÁÛÎÚ‡ÚËÚ ҇ Ô‰ËÏÌÓ ÌÂÛÚ‡ÎÌË ËÎË ÓÚˈ‡ÚÂÎÌË ÔÓ‡‰Ë ̉ÓÒÚ‡Ú˙˜ÂÌ ÂÙÂÍÚ, ÌÂÚÓ˜ÌÓ ÙÓÏÛÎË‡Ì‡ ‡‰ÂÍ‚‡Ú̇ ‰ÓÁ‡ ËÎË ‰ÓÁÓ-Á‡‚ËÒËÏË ÒÚ‡Ì˘ÌË ÂÙÂÍÚË, Ò ËÁÍβ˜ÂÌË ̇ ÔÓÎÓÊËÚÂÎÌËfl ÚẨ ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡ Ò Cerebrolysin Ë Citicoline. àÌÚÂÂÒ ·Û‰Ë Ù‡ÍÚ˙Ú, ˜Â Ì‚ÓÔÓÚÂÍÚË‚ÌËÚ Ò‰ÒÚ‚‡ ËÏ‡Ú ÔÓ-Ò˙˘ÂÒÚ‚ÂÌ ÂÙÂÍÚ ‚˙ıÛ ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜Ì‡Ú‡ ËÒıÂÏËfl ÔË ÂÍÒÔÂËÏÂÌÚ‡ÎÌË ÏÓ‰ÂÎË, ÍÓ„‡ÚÓ Ú Ò ÔË·„‡Ú ÔÂ‰Ë Ì‡˜‡ÎÓÚÓ Ì‡ ËÒıÂÏËflÚ‡. ëΉӂ‡ÚÂÎÌÓ ·Ë Úfl·‚‡ÎÓ ‰‡ Ò ӷ˙Ì ÒÔˆˇÎÌÓ ‚ÌËχÌË ̇ ‰ˈ‡ ÙËÁËÓÎӄ˘ÌË ÙÛÌ͈ËË, ÍÓËÚÓ Ò‡ ‚ Ôfl͇ ‚˙Á͇ Ò ˆÂ·‡ÎÌËfl ÏÂÚ‡·ÓÎËÁ˙Ï. ëÚ‡‚‡ ‰Ûχ Á‡ ÍÓÌÚÓΠ̇ ÒËÒÚÂÏÌÓÚÓ ‡ÚÂˇÎÌÓ Ì‡Îfl„‡Ì (Äç), ÚÂÎÂÒ̇ڇ ÚÂÏÔÂ‡ÚÛ‡, Í˙‚ÌÓÁ‡ı‡ÌÓÚÓ ÌË‚Ó, ÍËÒÎÓӉ̇ڇ Ò‡ÚÛ‡ˆËfl ̇ ‡ÚÂˇÎ̇ڇ Í˙‚ Ë ‰. ÇÒ˘ÍË Ú ˄‡flÚ Ò˙˘ÂÒÚ‚Â̇ ÓÎfl ‚ ÏÓ‰ÂÎË‡ÌÂÚÓ Ì‡ ËÒıÂÏ˘ÌËfl ÔÓˆÂÒ. éÚÍÎÓÌÂÌËflÚ‡ ÏÓ„‡Ú ‰‡ Ò ÍÓË„Ë‡Ú ˜ÂÁ ‡‰ÂÍ‚‡ÚÂÌ ÍÓÌÚÓΠ̇ ÚÂÁË ÔÓ͇Á‡ÚÂÎË, Ò ÍÓÂÚÓ ‰‡ Ò ÔÓ‚ÎËfl ‚˙ıÛ Í‡Ò͇‰‡Ú‡ ̇ ˆÂ·‡ÎÌÓÚÓ Û‚Âʉ‡ÌÂ. äéçíêéã çÄ íÖãÖëçÄíÄ íÖåèÖêÄíìêÄ ÇÁ‡ËÏÓÓÚÌÓ¯ÂÌËflÚ‡ ÏÂÊ‰Û ıËÔÂÚÂÏËflÚ‡ Ë ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ ‚Ò Ӣ Ì ҇ ̇Ô˙ÎÌÓ ËÁflÒÌÂÌË. íÓ‚‡ ÔÓ‚‰Ë„‡ ‰ˈ‡ ‚˙ÔÓÒË, ÍÓËÚÓ ‚ÂÓflÚÌÓ ˘Â ‰‡ ·˙‰‡Ú ¯‡‚‡ÌË ‚ ·˙‰Â˘Â. èË ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÏÓ‰ÂÎË Ì‡ ËÒıÂÏËfl Ò ‰Ó͇Á‚‡, ˜Â ıËÔÓÚÂÏËflÚ‡ ‰ۈË‡ ˆÂ·‡Î̇ڇ Û‚‰‡, ÍÓflÚÓ Ò ‡Á‚Ë‚‡ ‚ÒΉÒÚ‚Ë ÓÍÎÛÁËfl ̇ ˆÂ·‡Î̇ ‡ÚÂËfl (10, 15, 64, 83). ïËÔÓÚÂÏËflÚ‡ ̇χÎfl‚‡ ËÒıÂÏ˘̇ڇ Û‚‰‡ ÔË ÊË‚ÓÚËÌÒÍË ÏÓ‰ÂÎË. Ç ÌÂËÒıÂÏËÁË‡Ì‡ ÏÓÁ˙˜Ì‡ Ú˙Í‡Ì ÍÓÌÒÛχˆËflÚ‡ ̇ ÍËÒÎÓÓ‰ ̇χÎfl‚‡ Ò˙Ò 7% Á‡ ‚ÒÂÍË 1Óë ̇χÎfl‚‡Ì ̇ ÚÂÏÔÂ‡ÚÛ‡Ú‡. ëÚÂÔÂÌÚ‡ ̇ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ ıËÔÓÚÂÏËflÚ‡  ‚ Ôfl͇ ‚˙Á͇ Ò ÌÂÈÌÓÚÓ ‡ÌÌÓ ÔËÎÓÊÂÌË - ‚ Ô˙‚ËÚ 2 ˜‡Ò‡ (21), ͇ÍÚÓ Ë Ò ÔÓ-„ÓÎflχڇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ ÔË-

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·„‡ÌÂÚÓ È (Ôӂ˜ ÓÚ 24 ˜‡Ò‡) (22). èË ÂÍÒÔÂËÏÂÌÚ‡ÎÌÓ Ô‰ËÁ‚Ë͇ÌË ËÌÒÛÎÚË Û ÊË‚ÓÚÌË Â ‰Ó͇Á‡ÌÓ ‡Á‚ËÚË ̇ ıËÔÂÚÂÏËfl, ÍÓflÚÓ Ô‰ËÁ‚ËÍ‚‡ ÔÓ-„ÓÎflχ ˆÂ·‡Î̇ Û‚‰‡ (8, 20, 49). ïËÔÓÚÂÏËflÚ‡ Ô‰ÓÚ‚‡Úfl‚‡ ËÒıÂÏ˘̇ڇ ÏÓÁ˙˜Ì‡ Û‚‰‡ ÔÓ ÌflÍÓÎÍÓ ÏÂı‡ÌËÁχ: ● ‰ۈË‡Ì ̇Û¯‡‚‡ÌÂÚÓ Ì‡ Í˙‚ÌÓ-ÏÓÁ˙˜Ì‡Ú‡ ·‡ËÂ‡, ÍÓÂÚÓ Ò fl‚fl‚‡ ÒΉ ËÒıÂÏ˘ÌËfl Ë̈ˉÂÌÚ Ë ‚Ó‰Ë ‰Ó ÓÚÓÍ ÓÚ ÂÍÒÚ‡‚‡Á‡ˆËflÚ‡ (39); ● ÔÓ‰ÚËÒ͇Ì ÔÓ‰Û͈ËflÚ‡ ̇ Ò‚Ó·Ó‰ÌË ÍËÒÎÓÓ‰ÌË ‡‰Ë͇ÎË ‚ÒΉÒÚ‚Ë ‡ÍÚË‚Ë‡ÌÂÚÓ Ì‡ ÏËÍÓ„ÎËflÚ‡ Ë ‰Û„Ë ÍÎÂÚ˙˜ÌË ÚËÔÓ‚Â (74); ● ̇χÎfl‚‡Ì ̇ ÂÍÒˈËÚÓÚÓÍÒ˘ÌÓÚÓ Ì‚ÓÚ‡ÌÒÏËÚÂÌÓ ÓÒ‚Ó·Óʉ‡‚‡ÌÂ, ‚Ó‰Â˘Ó ‰Ó Ò‚˙ıÒÚËÏÛ·ˆËfl ̇ Ò˙Ò‰ÌËÚ Ì‚ÓÌË (58); ● Á‡·‡‚flÌ ̇ ÏÂÚ‡·ÓÎËÚÌËÚ ÔÓˆÂÒË, Ô‰ÓÚ‚‡Úfl‚‡˘Ó ‚ÂÌÚÛ‡ÎÌÓÚÓ ·˙ÁÓ ÂÌÂ„ËÈÌÓ ËÁ˜ÂÔ‚‡Ì (29) Ë ÔÓÚË‚Ó‚˙ÁÔ‡ÎËÚÂÎÌÓ ‰ÂÈÒÚ‚Ë (42). ïËÔÂÚÂÏËflÚ‡ Ò ÒÏflÚ‡ Á‡ ‚‡ÊÌÓ Ò˙·ËÚËÂ, ÍÓÂÚÓ ÒÚËÏÛÎË‡ ·ËÓıËÏ˘ÌËÚÂ Ë ‚˙ÁÔ‡ÎËÚÂÎÌË ËÒıÂÏ˘ÌË ÏÂı‡ÌËÁÏË ‚ ËÒıÂÏ˘̇ڇ ÔÂ̇Ϸ‡ Ë ÔÓ ÚÓÁË Ì‡˜ËÌ ‰ÓÔË̇Òfl Á‡ ÔÓ„ÂÒË‡Ì ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ (62). èӂ‰ÂÌË Ò‡ ÏÌÓ„Ó·ÓÈÌË ËÁÒΉ‚‡ÌËfl Á‡ ÔÓÛ˜‚‡Ì ÓÎflÚ‡ ̇ ÚÂÎÂÒ̇ڇ ÚÂÏÔÂ‡ÚÛ‡ ÔË Ô‡ˆËÂÌÚË Ò ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ – ‰Ó ͇͂‡ ÒÚÂÔÂÌ Úfl  ҂˙Á‡Ì‡ Ò Ï‡˘‡·‡ ̇ ËÒıÂÏ˘̇ڇ ÏÓÁ˙˜Ì‡ Û‚‰‡ (37, 75), ͇ÍÚÓ Ë ÔÓ ÓÚÌÓ¯ÂÌË ̇ Í‡ÈÌËfl ËÁıÓ‰, Ò˙ÓÚ‚ÂÚÌÓ ËÁÏÂ‚‡ÌË Ò ÚÂÊÂÒÚÚ‡ ̇ ËÌÒÛÎÚ‡ Ë ËÌÒÛÎÚ̇ڇ ÒÏ˙ÚÌÓÒÚ (7, 17). íÂÁË ËÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú ‚˙Á͇ ÏÂÊ‰Û Ôӂ˯Â̇ڇ ÚÂÎÂÒ̇ ÚÂÏÔÂ‡ÚÛ‡ ÔË ËÌÒÛÎÚÌÓ ·ÓÎÌËÚÂ Ë ÔÓ-„ÓÎÂÏËfl ‡ÁÏÂ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ (18, 24), ‡ ÓÚ ‰Û„‡ ‚˙Á͇ڇ ÏÂÊ‰Û Ôӂ˯Â̇ڇ ÚÂÏÔÂ‡ÚÛ‡ Ë ‚ÎÓ¯ÂÌËfl Í‡ÂÌ ËÁıÓ‰ ÓÚ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ (40, 79). Ö‰ËÌ ÏÂÚ‡-‡Ì‡ÎËÁ, ÍÓÈÚÓ ‚Íβ˜‚‡ 3790 Ô‡ˆËÂÌÚË, flÒÌÓ ÔÓ͇Á‚‡, ˜Â Ëχ ÔÓ-„ÓÎflχ ÏÓ·Ë‰ÌÓÒÚ Ë Ôӂ˯ÂÌ ÎÂÚ‡ÎËÚÂÚ ÔË Ô‡ˆËÂÌÚË Ò ˆÂ·‡ÎÂÌ ËÌÙ‡ÍÚ Ë ıËÔÂÚÂÏËfl, ÓÚÍÓÎÍÓÚÓ ‚ „ÛÔ‡Ú‡ ̇ Ô‡ˆËÂÌÚË Ò ˆÂ·‡ÎÂÌ ËÌÙ‡ÍÚ, ÌÓ ·ÂÁ ıËÔÂÚÂÏËfl (79). ÇÒÂ Ô‡Í Ò˙˘ÂÒÚ‚Û‚‡ Ë ÌÂÒ˙ÓÚ‚ÂÚÒÚ‚Ë ‚ ËÌÚÂÔÂÚ‡ˆËflÚ‡ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ̇˜‡ÎÓÚÓ Ë Á̇˜ÂÌËÂÚÓ Ì‡ ıËÔÂÚÂÏËflÚ‡ ‚ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ. åÓÏÂÌÚ˙Ú Ì‡ ÔÓfl‚‡Ú‡ ̇ ıËÔÂÚÂÏËfl  ÓÚ Á̇˜ÂÌËÂ: ıËÔÂÚÂÏËfl, ÍÓflÚÓ Á‡ÔÓ˜‚‡ ÔÂÁ Ô˙‚ËÚ 24 ˜‡Ò‡ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ËÌÒÛÎÚ‡, Ò ҂˙Á‚‡ Ò ÔÓ-„ÓÎflÏ ËÌÙ‡ÍÚÂÌ Ó·ÂÏ Ë ÔÓ-ÎÓ¯‡ Í‡È̇ ÔÓ„ÌÓÁ‡ (18), ͇ÍÚÓ Ë Ò ÔÓ-„ÓÎflÏ ÎÂÚ‡ÎËÚÂÚ (47). èÓÌflÍÓ„‡ ıËÔÂÚÂÏËflÚ‡ ÔÂÁ Ô˙‚ËÚ 72 ˜‡Ò‡ ÏÓÊ ‰‡ ·˙‰Â ÔÓ„ÌÓÒÚ˘ÂÌ ÔËÁÌ‡Í Á‡ ÎÓ¯ ËÁıÓ‰ Ë Á‡ Á̇˜ËÚÂÎÌÓ Ôӂ˯‡‚‡Ì ̇ ÔÓÒÚËÌÒÛÎÚÌËfl ÎÂÚ‡ÎËÚÂÚ. èÓÛ˜‚‡ÌÂ, Ôӂ‰ÂÌÓ ÓÚ Boysen Ë Christensen ÔÛ·ÎËÍÛ‚‡ÌÓ ÔÂÁ 2001 „Ó‰Ë̇ (12) ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â ÔÓ͇˜‚‡ÌÂÚÓ Ì‡ ÚÂÎÂÒ̇ڇ ÚÂÏÔÂ‡ÚÛ‡ ‚ Ô˙‚ËÚ ˜‡ÒÓ‚Â ÒΉ ÔÓfl‚‡Ú‡ ̇ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË Â ·ËÎÓ Ò‚˙Á‡ÌÓ Ò Ï‡Ò˂̇ ËÒıÂÏËfl, ÍÓÂÚÓ ÔÓ‰Ò͇Á‚‡, ˜Â ‡Ì̇ڇ ÔÓÒÚËÌÒÛÎÚ̇ ıËÔÂÚÂÏËfl ·Ë Ïӄ· ‰‡ ·˙‰Â ÂÁÛÎÚ‡Ú ÓÚ Ò‡ÏËfl ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ. ä‡ÚÓ Ò Ëχ Ô‰ ‚ˉ, ˜Â ÔÓÒÚËÌÒÛÎÚ̇ڇ ıËÔÂÚÂÏËfl  ҂˙Á‡Ì‡ Ò ÏÂı‡ÌËÁÏËÚ ̇ ˆÂ·‡Î̇ڇ ËÒıÂÏËfl Ë Ëχ ÓÚÌÓ¯ÂÌË Í˙Ï ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ ‚ ÁÓ̇ڇ ̇ ËÒıÂÏ˘ÌÓÚÓ fl‰Ó Ë Ì‡ ÔÂ̇Ϸ‡Ú‡, ·Ë ·ËÎÓ Ô‡‚ËÎÌÓ ‰‡ Ò ͇ÊÂ, ˜Â ıËÔÂÚÂÏËflÚ‡ ÔË ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ Â fl‚ÎÂÌË ‰ÌÓ‚ÂÏÂÌÌÓ Ë̉ۈË‡ÌÓ, ÌÓ Ë Ë̉ۈË‡˘Ó ıÓ‰‡ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ (61). éÒ‚ÂÌ ÚÓ‚‡, Ô‡ˆËÂÌÚËÚÂ Ò ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ÒÚ‡‚‡Ú ÊÂÚ‚‡ Ë Ì‡ ÒÛÔÂÔÓÌË‡ÌË ËÌÙÂ͈ËË (·ÂÎÓ‰Ó·ÌË Ë ÛË̇ÌË), ‡ ÚÂÁË ËÌÙÂ͈ËË ˜ÂÒÚÓ Ò‡ Ô˘Ë̇ ÓÚ ÔÂËÙÂÌÓ ÂÒÚÂÒÚ‚Ó Á‡ ıËÔÂÚÂÏËflÚ‡ ÔË ÚÂÁË Ô‡ˆËÂÌÚË. Ç˙ÔÂÍË, ˜Â ‡ÌÚËÔËÂÚˈËÚ ҇ Ó·˘ÓÔËÁ̇ÚË ÔË


‰ÂÍÂÏ‚Ë, 2007

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

ΘÂÌËÂÚÓ Ì‡ ıËÔÂÚÂÏËflÚ‡, Ìflχ ‰Ó͇Á‡ÚÂÎÒÚ‚‡ Á‡ ÚflıÌÓÚÓ ‚ÎËflÌË ‚˙ıÛ ËÁıÓ‰‡ ÓÚ ËÌÒÛÎÚ‡. European Stroke Initiative (EUSI) ÔÂÔÓ˙˜‚‡ ÔÓÌËʇ‚‡ÌÂÚÓ Ì‡ ÚÂÏÔÂ‡ÚÛ‡Ú‡ ‰‡ Ò ËÁ‚˙¯‚‡ ÔË ÒÚÓÈÌÓÒÚË Ì‡‰ 37.5Óë (31). íÂ‡Ô‚Ú˘ÌËÚ ÔÓ‰ıÓ‰Ë Ò ‡ÌÚËÔËÂÚËˆË Í‡ÚÓ Ì‡ÔËÏÂ Ô‡‡ˆÂÚ‡ÏÓÎ, Ò‡ ÔÓ͇Á‡ÎË ÂÙÂÍÚË‚ÌÓÒÚ Â‰ËÌÒÚ‚ÂÌÓ ÔË ÔÓÌËʇ‚‡ÌÂÚÓ Ì‡ ÚÂÎÂÒ̇ڇ ÚÂÏÔÂ‡ÚÛ‡ (27), ÌÓ ÌÂ Ë ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ì‚ÓÎӄ˘ÌËfl ÙÛÌ͈ËÓ̇ÎÂÌ Ë ÔÓ„ÌÓÒÚ˘ÂÌ ÒÚ‡ÚÛÒ (50). èÓ‡‰Ë χÎÍËfl ·ÓÈ Ì‡ Ô‡ˆËÂÌÚËÚÂ, ÔË ÍÓËÚÓ Â ÔÓÛ˜‚‡Ì ÂÙÂÍÚ‡ ÓÚ ÔË·„‡ÌÂÚÓ Ì‡ ‡ÌÚËÔËÂÚËˆË ‚ ÓÒÚËfl ÒÚ‡‰ËÈ Ì‡ ËÌÒÛÎÚ‡, Ì ÏÓ„‡Ú ‰‡ Ò ̇Ô‡‚flÚ ‰ÂÙËÌËÚË‚ÌË Á‡Íβ˜ÂÌËfl Á‡ ÔÓÎÁ‡Ú‡ ̇ ÚÂÁË ÔÂÔ‡‡ÚË ÔË ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ. ÇÒ ԇÍ, ‰Ó ÔÓÎÛ˜‡‚‡ÌÂÚÓ Ì‡ ÂÁÛÎÚ‡ÚË ÓÚ ‡Ì‰ÓÏËÁË‡ÌË ÍÎËÌ˘ÌË ËÁÒΉ‚‡ÌËfl, ÔË·„‡ÌÂÚÓ Ì‡ ‡ÌÚËÔËÂÚËˆË Ò ÔÂÔÓ˙˜‚‡ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÓÒÚ˙ ËÌÒÛÎÚ Ò ÚÂÏÔÂ‡ÚÛ‡ ̇‰ 37.5Óë, ‰ÌÓ‚ÂÏÂÌÌÓ Ò ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ·˙Á‡ ‰Ë‡„ÌÓÁ‡ ̇ ÒÛÔÂÔÓÌË‡ÌËÚ ËÌÙÂ͈ËË Ë ÚflıÌÓÚÓ ÂÚËÓÎӄ˘ÌÓ Î˜ÂÌËÂ. éÚ ‰Û„‡ ÒÚ‡Ì‡, ÔË·„‡ÌÂÚÓ Ì‡ ÚÂ‡Ô‚Ú˘̇ ıËÔÓÚÂÏËfl ÔË Î˜ÂÌËÂÚÓ Ì‡ ÓÒÚËfl ÒÚ‡‰ËÈ Ì‡ ËÌÒÛÎÚ‡,  ‚Ò Ӣ ‚ ÂÍÒÔÂËÏÂÌÚ‡ÎÂÌ ÒÚ‡‰ËÈ Ë Í‡Ú„Ó˘ÌË ‰Ó͇Á‡ÚÂÎÒÚ‚‡ Á‡ Ì„ӂ‡Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ ‰ÓÒ„‡ Ì ҇ ÔÓÎÛ˜ÂÌË. Ç ÌflÍÓÎÍÓ ÔÓÛ˜‚‡ÌËfl ÔË Ô‡ˆËÂÌÚË Ò ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ·ÓÎÌËÚ ҇ ıËÔÓÚÂÏË‡ÌË Ò Ó„Î‰ ÓÒË„Ûfl‚‡Ì ̇ Ì‚ÓÔÓÚÂ͈Ëfl (32, 46, 51, 59, 68, 69). íÂÁË ËÁÒΉ‚‡ÌËfl Ò‡ χÎÍÓ Ì‡ ·ÓÈ Ë ıÂÚÂÓ„ÂÌÌË ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ôӂ˜ÂÚÓ Ô‡‡ÏÂÚË, ÍÓÂÚÓ Á‡ÚÛ‰Ìfl‚‡ ÚÓ˜ÌÓÚÓ ÓÔ‰ÂÎflÌ ̇ ÔÓÎÛ˜ÂÌËfl ÂÙÂÍÚ ÓÚ Óı·ʉ‡ÌÂÚÓ ÔË ËÌÒÛÎÚÌÓ ·ÓÎÌËÚÂ. èË ‰ÓÒ„‡¯ÌËÚ ËÁÒΉ‚‡ÌËfl Á‡ Á̇˜ÂÌËÂÚÓ Ì‡ ÚÂ‡Ô‚Ú˘̇ڇ ıËÔÓÚÂÏËfl, ̇·Î˛‰‡‚‡ÌËÚ ԇ‡ÏÂÚË Ò‡ ‡Á΢ÌË - ̇ÔËÏÂ ‚ÂÏÂÚÓ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ÒËÏÔÚÓÏËÚ ‰Ó Á‡ÔÓ˜‚‡Ì ̇ ÚÂ‡ÔËflÚ‡; ‡Á΢̇  ·Ë· Ë ÚÂÏÔÂ‡ÚÛ‡Ú‡, ÍÓflÚÓ Â ·Ë· Ô‰‚‡ËÚÂÎÌÓ ÓÔ‰ÂÎfl̇ ͇ÚÓ Á‡‰‡‚‡ÌÓ ÌË‚Ó ÔË ·ÓÎÌËÚÂ, ͇ÍÚÓ Ë Ò‡Ï‡Ú‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒ�� ̇ ıËÔÓÚÂÏËflÚ‡. íÂÏÔÂ‡ÚÛ‡Ú‡, ÍÓflÚÓ Â ·Ë· Á‡‰‡‚‡Ì‡ ‚ Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë,  ·Ë· 32Óë – 33Óë. íÂ‡Ô‚Ú˘ÌËflÚ ÔÓÁÓˆ  ·ËÎ 3 – 8 ˜‡Ò‡, ‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ ıËÔÓÚÂÏËflÚ‡ ‚‡Ë‡ ÓÚ 6 ˜‡Ò‡ ‰Ó ÌflÍÓÎÍÓ ‰ÌË. ãÂÒ̇ڇ ÓÒ˙˘ÂÒÚ‚ËÏÓÒÚ Ë ·ÂÁÓÔ‡ÒÌÓÒÚÚ‡ ̇ ڇ͇‚‡ ÚÂ‡ÔËfl Ó·‡˜Â Ì ÏÓ„‡Ú ‰‡ Ô‰ÓÚ‚‡ÚflÚ ÔÓfl‚‡Ú‡ Ë Ì‡ ÒÚ‡Ì˘ÌË ÂÙÂÍÚË (38). ëÔ‡‰‡Ì ̇ Í˙‚ÌÓÚÓ Ì‡Îfl„‡ÌÂ, Ò˙‰Â˜ÌËÚ ‡ËÚÏËË Ë ÔÌ‚ÏÓÌËËÚ Ò Ò¢‡Ú ˜ÂÒÚÓ, ÓÒÓ·ÂÌÓ ÔË ‡ÌÂÒÚÂÁË‡ÌË Ô‡ˆËÂÌÚË Ò ÚÂÏÔÂ‡ÚÛ‡ 32Óë – 33Óë (61). çflχ ‰‡ÌÌË ÓÚ ‡Ì‰ÓÏËÁË‡ÌË ËÁÒΉ‚‡ÌËfl, ÍÓËÚÓ ‰‡ ÔÓ‰ÍÂÔflÚ ÛÚËÌÌÓÚÓ ÔË·„‡Ì ̇ ıËÏ˘ÂÒ͇ (·‡·ËÚÛÓ‚‡) ËÎË ÙËÁË͇Î̇ ÚÂ‡ÔËfl Á‡ Óı·ʉ‡Ì ÔË ÓÒÚËfl ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ (23). ÇÒ ԇÍ, ÒΉ ͇ÚÓ ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ËÁÒΉ‚‡ÌËfl Ò‡ ÔÓ͇Á‡ÎË Ì‚ÓÔÓÚÂÍÚË‚ÂÌ ÂÙÂÍÚ ÓÚ ÔË·„‡ÌÂÚÓ Ì‡ ıËÔÓÚÂÏËfl ÔË ˆÂ·‡Î̇ ËÒıÂÏËfl (68), ÔÓ-̇ڇÚ˙¯ÌËÚ ÔÓÛ˜‚‡ÌËfl Ò Óı·ʉ‡˘‡ ÚÂ‡ÔËfl ÔË ·ÓÎÌË ‚ ÓÒÚËfl ÒÚ‡‰ËÈ Ì‡ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ ÔÓ‰˙Îʇ‚‡Ú. äéçíêéã çÄ äêöÇçéáÄïÄêçéíé çàÇé èӂ˯ÂÌÓÚÓ Í˙‚ÌÓÁ‡ı‡ÌÓ ÌË‚Ó Â ˜ÂÒÚÓ Ò¢‡ÌÓ ‚ ‡Ì̇ڇ Ù‡Á‡ ̇ ËÌÒÛÎÚ‡. ïËÔÂ„ÎËÍÂÏËfl ̇‰ 6 mmol/l Ò Ò¢‡ ÔË ‰‚ ÚÂÚË ÓÚ Ô‡ˆËÂÌÚË Ò ‡Á΢ÌËÚ ÔÓ‰ÚËÔÓ‚Â ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ‚ ÏÓÏÂÌÚ‡ ̇ ÔËÂχ ̇ Ô‡ˆËÂÌÚËÚ ‚ ·ÓÎÌˈ‡ (70). ÖÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÏÓ‰ÂÎË Ì‡ ÙÓ͇Î̇ ˆÂ·‡Î̇ ËÒıÂÏËfl ÔË ÊË‚ÓÚÌË ‰‡‚‡Ú ÓÒÌÓ‚‡ÌË ‰‡ Ò ÒÏflÚ‡, ˜Â ıËÔÂ„ÎËÍÂÏËflÚ‡ ÏÓÊ ‰‡ ÔÓ‚ÎËfl Ì‚Ó̇Î̇ڇ Û‚‰‡ ˜ÂÁ Á‡‰˙ηӘ‡‚‡Ì ̇ Ú˙͇Ì̇ڇ ‡ˆË‰ÓÁ‡ Ë „ÂÌÂË‡ÌÂ-

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ÚÓ Ì‡ ·ÍÚ‡Ú (65, 82). ìÏÂÂ̇ڇ Ë ‚ËÒÓÍÓÒÚÂÔÂÌ̇ڇ ıËÔÂ„ÎËÍÂÏËfl ‰ÓÔ˙ÎÌËÚÂÎÌÓ ‚ÎÓ¯‡‚‡Ú ÏÂÚ‡·ÓÎËÚÌËfl ÒÚ‡ÚÛÒ Ë ÏËÚÓıÓ̉ˇÎ̇ڇ ÙÛÌ͈Ëfl ‚ ӷ·ÒÚÚ‡ ̇ ËÒıÂÏ˘̇ڇ ÔÂ̇Ϸ‡ (5). ä˙‚ÌÓ-ÏÓÁ˙˜Ì‡Ú‡ ·‡ËÂ‡  ÛflÁ‚Ëχ ÔË ıËÔÂ„ÎËÍÂÏ˘ÌË Ò˙ÒÚÓflÌËfl, ‚ÂÓflÚÌÓ ÔÓ‡‰Ë ÓÒ‚Ó·Óʉ‡‚‡ÌÂÚÓ Ì‡ ·ÍÚ‡Ú Ë Ò‚Ó·Ó‰ÌË ‡‰Ë͇ÎË (57). ᇠÔËÏÂ ÏÓÊ ‰‡ ÔÓÒÎÛÊË ÏÓ‰ÂΠ̇ ÓÍÎÛÁËfl ̇ ‰̇ڇ ÓÚ ‰‚ÂÚ aa. cerebri media Û ıËÔÂ„ÎËÍÂÏ˘ÌË ÍÓÚÍË, ÔË ÍÓÈÚÓ Ò  fl‚ËÎÓ ÔÂÚÍ‡ÚÌÓ Ì‡‡ÒÚ‚‡Ì ̇ Ó·Âχ ̇ ËÌÙ‡ÍÚ̇ڇ ÁÓ̇ ‚ Ò‡‚ÌÂÌËÂ Ò „ÓÎÂÏË̇ڇ ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ ÔË ÔÓ‰Ó·ÂÌ ÏÓ‰ÂÎ Û ÌÓÏÓ„ÎËÍÂÏ˘ÌË ÍÓÚÍË (25). ÖÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÔÓÛ˜‚‡ÌËfl Ò‡ ÛÒÚ‡ÌÓ‚ËÎË, ˜Â ‚˙‚Âʉ‡ÌÂÚÓ Ì‡ ËÌÒÛÎËÌ Ë „βÍÓÁ‡ ÔÓ ‚ÂÏ ̇ ‰̇ ÙÓ͇Î̇ ˆÂ·‡Î̇ ËÒıÂÏËfl, ÏÓÊ ‰‡ ‰ۈË‡ ‡ÁÏÂ‡ ̇ ˆÂ·‡ÎÌÓÚÓ ËÌÙ‡ˆË‡Ì (35). ïËÔÂ„ÎËÍÂÏËflÚ‡  ҂˙Á‡Ì‡ Ò ÔÓ-ÎÓ¯ ËÌÒÛÎÚÂÌ ËÁıÓ‰. ä‡ÍÚÓ ÔÓÒÔÂÍÚË‚ÌËÚÂ, ڇ͇ Ë ÍÓÌÚÓÎË‡ÌËÚ ÔÓÛ˜‚‡ÌËfl ÒÚË„‡Ú ‰Ó ËÁ‚Ó‰‡, ˜Â ıËÔÂ„ÎËÍÂÏËflÚ‡  ÔÓ„ÌÓÒÚ˘ÂÌ ÔËÁÌ‡Í Á‡ ËÁıÓ‰‡ Ë ÎÂÚ‡ÎËÚÂÚ‡ ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‚˙Á‡ÒÚÚ‡, ËÌÒÛÎÚÌËfl ÔÓ‰ÚËÔ Ë ÚÂÊÂÒÚÚ‡ ̇ Ë̈ˉÂÌÚ‡ (14, 43, 48, 80). ÑÛ„Ë ÔÓÛ˜‚‡ÌËfl Ó·‡˜Â Ì ҇ ‰Ó͇Á‡ÎË ÔÓ„ÌÓÒÚ˘̇ڇ ÓÎfl ̇ ıËÔÂ„ÎËÍÂÏËflÚ‡ ÔË ËÁıÓ‰‡ ÓÚ ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ͇ÚÓ Ò ËÁ͇Á‚‡ ÚÂÁ‡Ú‡, ˜Â ıËÔÂ„ÎËÍÂÏËflÚ‡ ÔÓÒÚÓ ÓÚ‡Áfl‚‡ ͇ÚÂıÓ·ÏËÌÓ‚Ó-·‡ÁË‡Ì ÒÚÂÒÓ‚ ÓÚ„Ó‚Ó ÔË ËÌÒÛÎÚÌÓ ·ÓÎÌË. Ö‰ÌÓ ÂÚÓÒÔÂÍÚË‚ÌÓ ÔÓÛ˜‚‡Ì ÛÒÚ‡ÌÓ‚fl‚‡ Ôӂ˯‡‚‡Ì ̇ Í˙‚ÌÓÁ‡ı‡ÌËÚ ÌË‚‡ ÔÂÁ Ô˙‚ËÚ 12 ˜‡Ò‡ ÒΉ ̇ÒÚ˙Ô‚‡ÌÂÚÓ Ì‡ ËÌÒÛÎÚ‡, ÍÓÂÚÓ Â ÔÓ-Á̇˜ËÚÂÎÌÓ ‚ ÔÓÚÂÊÍËÚ ÒÎÛ˜‡Ë Ë Â Ò‚˙Á‡ÌÓ Ò ‡ÌÂÌ ÎÂÚ‡ÎËÚÂÚ. èË ÚÓ‚‡,  ̇·Î˛‰‡‚‡ÌÓ ÒÔÓÌÚ‡ÌÌÓ ÔÓÌËʇ‚‡Ì ̇ Í˙‚ÌÓÁ‡ı‡ÌËÚ ÌË‚‡ ÔÂÁ Ô˙‚ËÚ 24 ˜‡Ò‡ ̇ ËÌÒÛÎÚ‡ (33). LJÊÌÓ Â ‰‡ Ò ÔÓ‰˜ÂÚ‡Â, ˜Â ÔÂÒËÒÚË‡ÌÂÚÓ Ì‡ ıËÔÂ„ÎËÍÂÏËflÚ‡ ÔÂÁ Ô˙‚ËÚ 7 ‰ÌË ÒΉ ̇ÒÚ˙Ô‚‡ÌÂÚÓ Ì‡ ËÌÒÛÎÚ‡  ҂˙Á‡ÌÓ Ò ÔÓ-„ÓÎflÏ Í‡ÂÌ ËÌÙ‡ÍÚÂÌ Ó·ÂÏ Ë Ò ‚ÎÓ¯‡‚‡Ì ̇ ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (9). ÇËÒÓÍÓÚÓ Í˙‚ÌÓÁ‡ı‡ÌÓ ÌË‚Ó Ë ÔÓ ‚ÂÏ ̇ Â͇̇ÎËÁ‡ˆËflÚ‡ ̇ Á‡ÔÛ¯Â̇ڇ ÏÓÁ˙˜Ì‡ ‡ÚÂËfl  ҂˙Á‡ÌÓ Ò˙˘Ó Ò ÔÓ-ÎÓ¯ ËÁıÓ‰ (4). Ç˙ÔÂÍË, ˜Â Ӣ ÎËÔÒ‚‡Ú Í‡ÈÌË ÂÁÛÎÚ‡ÚË ÓÚ ÍÓÌÚÓÎË‡ÌËÚ ÍÎËÌ˘ÌË ÔÓÛ˜‚‡ÌËfl, ÍÓËÚÓ ÓˆÂÌfl‚‡Ú ËÌÒÛÎËÌÓ‚‡Ú‡ ÚÂ‡ÔËfl ÔË ËÌÒÛÎÚÌÓ ·ÓÎÌË, ÂÁÛÎÚ‡ÚËÚ ÓÚ ÔÓÛ˜‚‡ÌËflÚ‡ ÔË ÊË‚ÓÚÌË Ë ÍÎËÌ˘ÌËÚ ӷÁÂ‚‡ˆËÓÌÌË ÔÓÛ˜‚‡ÌËfl ÔÓ‰˜ÂÚ‡‚‡Ú ÔÓÎÁ‡Ú‡ ÓÚ ËÁ·fl„‚‡ÌÂÚÓ Ì‡ ‡Ì̇ ıËÔÂ„ÎËÍÂÏËfl ÔË Ô‡ˆËÂÌÚËÚÂ Ò Ì·ÍÛ̇ÌË ËÌÙ‡ÍÚË Ë „ÎÓ·‡Î̇ ËÒıÂÏËfl (45). àÌÚÂÌÁË‚ÌÓÚÓ Î˜ÂÌËÂ Ò ËÌÒÛÎËÌ Á‡ ÔÓ‰‰˙ʇÌÂÚÓ Ì‡ Í˙‚ÌÓÁ‡ı‡ÌË ÌË‚‡ ÏÂÊ‰Û 4 Ë 6 mmol/l ÔË Ô‡ˆËÂÌÚËÚ ‚ ÍËÚ˘ÌÓ Ò˙ÒÚÓflÌË Ò  Ó͇Á‡ÎÓ ·ÂÁÓÔ‡ÒÌÓ Ë Á̇˜ËÚÂÎÌÓ Â ‰ÓÔËÌÂÒÎÓ Á‡ ̇χÎfl‚‡Ì ̇ ÎÂÚ‡ÎËÚÂÚ‡ Ë ‚˙Ú·ÓÎÌ˘ÌËÚ ÍÓÏÔÎË͇ˆËË (77). àÁÒΉ‚‡ÌÂÚÓ GIST (Glucose Insulin in Stroke Trial) ‰ÂÏÓÌÒÚË‡ ·ÂÁÓÔ‡ÒÌÓÒÚÚ‡ Ë ˆÂÎÂÒ˙Ó·‡ÁÌÓÒÚÚ‡ ̇ ÔËÎÓÊÂÌËÂÚÓ Ì‡ „βÍÓÁÌÓ-͇ÎË‚Ó-ËÌÒÛÎËÌÓ‚Ë ËÌÙÛÁËË ‚ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ Í‡ÚÓ ˆÂÎÚ‡  ‰‡ Ò ÔÓ‰‰˙Ê‡Ú Í˙‚ÌÓÁ‡ı‡ÌË ÌË‚‡ ÏÂÊ‰Û 4 Ë 7 mmol/l (70). EUSI ÔÂÔÓ˙˜‚‡ ÔÓ‰‰˙ʇÌ ̇ ÒÚÓÈÌÓÒÚË ÔÓ‰ 10 mmol/l (31). Ç Í‡È̇ ÒÏÂÚ͇ ¯ÂÌËÂÚÓ Á‡ ËÌÚÂÌÁË‚ÌÓ ËÌÒÛÎËÌÓ‚Ó Î˜ÂÌË ̇ ÓÚ‰ÂÎÌËfl Ô‡ˆËÂÌÚ Úfl·‚‡ ‰‡ Ò ‚ÁÂχ, ÒΉ ͇ÚÓ Ò ÔˆÂÌË Ë̉˂ˉۇÎÌËfl Ó·˘ ÍÎËÌ˘ÂÌ ÙÓÌ. äéçíêéã çÄ äêöÇçéíé çÄãüÉÄçÖ ñÂ·‡Î̇ڇ ÔÂÙÛÁËfl ÌÓχÎÌÓ Ò ÓÔ‰ÂÎfl ÓÚ ÎÓ͇ÎÌËÚ ÏÓÁ˙˜ÌË ÏÂÚ‡·ÓÎËÚÌË ÌÛÊ‰Ë Ë Â ÓÚÌÓÒËÚÂÎÌÓ ÌÂÁ‡‚ËÒËχ ÓÚ ÒËÒÚÂÏÌÓÚÓ Äç Ò ËÁÍβ˜ÂÌË ̇ ÒÎÛ˜‡ËÚÂ Ò ÏÌÓ„Ó ÌËÒÍË ËÎË ÏÌÓ„Ó ‚ËÒÓÍË ÌË‚‡ (Ú. ̇. ˆÂ·‡Î̇ ‡‚ÚÓ„Û·ˆËfl). èË ÓÒÚËfl ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‡‚ÚÓ„Û·ˆËflÚ‡ Ò Á‡„Û·‚‡ Ë ÔÂÙÛÁËflÚ‡ ÒÚ‡‚‡ ÔflÍÓ Á‡-


‰ÂÍÂÏ‚Ë, 2007

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

‚ËÒËχ ÓÚ Ì‡Îfl„‡ÌÂÚÓ. é·ÁÂ‚‡ˆËÓÌÌËÚ ËÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ÔË·ÎËÁËÚÂÎÌÓ 75 % ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ËÏ‡Ú Ôӂ˯ÂÌÓ Äç ÔË ËÁÏÂ‚‡ÌÂÚÓ ÏÛ ‚ ‡ÏÍËÚ ̇ Ô˙‚ËÚ 24 ‰Ó 48 ˜‡Ò‡ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ Ë̈ˉÂÌÚ‡. ë˜ËÚ‡ ÒÂ, ˜Â ÒËÒÚÂÏ̇ڇ ıËÔÂÚÓÌËfl ÔÓ ‚ÂÏÂÚÓ Ì‡ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ Â ÙËÁËÓÎӄ˘ÂÌ ÓÚ„Ó‚Ó, ÍÓÈÚÓ ÔÓ‰‰˙ʇ ‡‰ÂÍ‚‡Ú̇ڇ ˆÂ·‡Î̇ ÔÂÙÛÁËfl ‚ ËÒıÂÏ˘̇ڇ ÔÂ̇Ϸ‡ (64). èÓ„ÌÓÒÚ˘ÌÓÚÓ ‚ÎËflÌË ̇ Äç ÔÓ ‚ÂÏ ̇ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ Â ‚Ò Ӣ ӷÂÍÚ Ì‡ ÒÔÓÓ‚Â. éÚ Â‰Ì‡ ÒÚ‡Ì‡, Ú‚˙‰Â ‚ËÒÓÍÓÚÓ Äç ÏÓÊ ‰‡ Ë̉ۈË‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ ÓÚÓÍ, ıÂÏÓ‡„˘̇ڇ Ú‡ÌÒÙÓχˆËfl ̇ ÏÓÁ˙˜ÌËfl ËÌÙ‡ÍÚ Ë ÔÓ-̇ڇÚ˙¯Ì‡Ú‡ Ò˙‰Ó‚‡ Û‚‰‡. éÚ ‰Û„‡ ÒÚ‡Ì‡ Ó·‡˜Â, ÌËÒÍÓÚÓ Äç ÏÓÊ ‰‡ Ô‰ËÁ‚Ë͇ ‚ÚÓ˘ÌÓ ‰ۈË‡Ì ̇ ÔÂÙÛÁËflÚ‡ ‚ ӷ·ÒÚÚ‡ ̇ ËÒıÂÏËflÚ‡ (64). ä‡ÍÚÓ Ú‚˙‰Â ‚ËÒÓÍÓÚÓ, ڇ͇ Ë Ú‚˙‰Â ÌËÒÍÓÚÓ Äç, ͇ÍÚÓ Ë ‰‡ÒÚ˘ÌÓÚÓ ÒÔ‡‰‡Ì ̇ ̇Îfl„‡ÌÂÚÓ, Ò‡ ÎÓ¯ ÔÓ„ÌÓÒÚ˘ÂÌ Ù‡ÍÚÓ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ (19, 55, 78). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ‰‡ÒÚ˘ÌÓÚÓ ‰ۈË‡Ì ̇ Äç ‚ Ô˙‚ËÚ 24 ˜‡Ò‡ ̇ ËÌÒÛÎÚ‡,  ҇ÏÓÒÚÓÈÌÓ Ò‚˙Á‡ÌÓ Ò ÔÓÎÓ¯ Ì‚ÓÎӄ˘ÂÌ ËÁıÓ‰ ‰Ó 3-Ëfl ÏÂÒˆ ÓÚ ËÌÒÛÎÚ‡ (60) Ë Ò ‚ÎÓ¯‡‚‡Ì ‚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ (19). ìÔÓÚ·‡Ú‡ ̇ ÒÛ·ÎËÌ„‚‡ÎÂÌ ÌËÙ‰ËÔËÌ Â ·Ë· Ô‰ÏÂÚ Ì‡ ÓÒÓ·ÂÌÓ ‚ÌËχÚÂÎÌÓ Ì‡·Î˛‰ÂÌË ÔÓ‡‰Ë ÚẨÂ̈ËflÚ‡ ÏÛ ‰‡ Ô‰ËÁ‚ËÍ‚‡ ·˙ÁË, ˜ÂÒÚÓ Ô˙ÚË ÒÚÂτ·‚Ë Ô‡‰Ó‚ ̇ Äç. Ö‰ËÌ ‚ÚÓ˘ÂÌ ‡Ì‡ÎËÁ ̇ ÌËÏÓ‰ËÔËÌÓ‚Ëfl ÂÙÂÍÚ ÔË ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ÔÂÁ Ô˙‚ËÚ 24 ˜‡Ò‡ ÔÓ͇Á‚‡, ˜Â ̇χÎfl‚‡ÌÂÚÓ Ì‡ ‰Ë‡ÒÚÓ΢̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ Äç Ò ÓÍÓÎÓ 15 ÏÏ çg  ҂˙Á‡ÌÓ Ò ÎÓ¯ Í‡ÂÌ ËÁıÓ‰ (3). çflÍÓÎÍÓ Ì‡·Î˛‰‡ÚÂÎÌË ÔÓÛ˜‚‡ÌËfl Ò‡ ÛÒÚ‡ÌÓ‚ËÎË ‚˙Á͇ڇ ÏÂÊ‰Û ‰‡ÒÚ˘ÌÓ Ôӂ˯ÂÌËÚ ÌË‚‡ ̇ Äç Ë ÎÓ¯Ëfl Í‡ÂÌ ËÁıÓ‰ (16, 44, 54, 66, 81). чÌÌËÚ ÓÚ International Stroke Trial (IST) ÔÓÚ‚˙ʉ‡‚‡Ú, ˜Â ËÒÍ˙Ú ÓÚ ‡Ì̇ Ë ÔÓ-Í˙Ò̇ ÒÏ˙Ú, ͇ÍÚÓ Ë ÓÚ ‡Á‚ËÚËÂÚÓ Ì‡ ÚÂÊ͇ ËÌ‚‡ÎˉÌÓÒÚ Â Ò‡ÏÓÒÚÓÈÌÓ Ò‚˙Á‡Ì Ò ÔÓ͇˜‚‡ÌÂÚÓ Ì‡ ÒËÒÚÓÎ̇ڇ ÒÚÓÈÌÓÒÚ Ì‡ Äç Û 17398 ·ÓÎÌË (55). ëËÒÚÂÏÌËflÚ Ô„Ή ̇ 32 ÔÓÛ˜‚‡ÌËfl, ‚Íβ˜‚‡˘Ë 10892 ÔÓˆËÂÌÚ‡, ÒÚË„‡ ‰Ó Á‡Íβ˜ÂÌËÂÚÓ, ˜Â ‰‡ÒÚ˘ÌÓ Ôӂ˯ÂÌÓÚÓ Äç ÔË ÓÒÚËfl ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Â Ò‚˙Á‡ÌÓ Ò ÔÓÒΉ‚‡˘‡ ÒÏ˙Ú, ‚ÎÓ¯‡‚‡Ì ËÎË ‡Á‚ËÚË ̇ ÚÂÊ͇ ËÌ‚‡ÎˉÌÓÒÚ, ‰Ó͇ÚÓ ÛÏÂÂÌÓÚÓ ÔÓÌËʇ‚‡Ì ̇ Äç ·Ë ÏÓ„ÎÓ ‰‡ ÔÓ‰Ó·Ë ËÁıÓ‰‡ ÓÚ Á‡·ÓÎfl‚‡ÌÂÚÓ. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË Ò ËÒıÂÏ˘ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ ÔÓÎÛ˜‡‚‡Ú ‡ÌÚËıËÔÂÚÂÌÁË‚ÌË ÔÂÔ‡‡ÚË, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÎËÔÒ‡Ú‡ ̇ ÚÂÊ͇ ıËÔÂÚÓÌËfl (56). çflχ Ó·˘ÓÔËÂÚÓ ÒÚ‡Ìӂˢ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÓ‚‡ Í‡Í Úfl·‚‡ ‰‡ Ò ÔÓ‰˙ʇ Äç ‚ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ. ç‡ÒÚÓfl˘ËÚ ÏÌÂÌËfl ‚‡Ë‡Ú ÓÚ Â‰Ì‡Ú‡ Í‡ÈÌÓÒÚ Ì‡ Ô˙ÎÌÓÚÓ Ì„ÎËÊË‡Ì ‰Ó ÔÂÒÚ‡‡‚‡ÌÂÚÓ ÔË ÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‡ÌÚËıËÔÂÚÂÌÁË‚ÌË Ò‰ÒÚ‚‡ (72). Ç˙ÔÂÍË, ˜Â Ò‚‡ÎflÌÂÚÓ Ì‡ Äç ËÁ„ÎÂʉ‡ ÔË‚ÎÂ͇ÚÂÎÌÓ, Úfl·‚‡ ‰‡ Ò Ëχ Ô‰‚ˉ, ˜Â ˆÂ·‡Î̇ڇ ‡‚ÚÓ„Û·ˆËfl ÔË ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ Ò Á‡„Û·‚‡ Ë ‚ÒΉÒÚ‚Ë ̇ ÚÓ‚‡ ÏÓÁ˙˜Ì‡Ú‡ ÔÂÙÛÁËfl  ‰ËÂÍÚÌÓ ÔÓÔÓˆËÓ̇Î̇ ̇ ̇Îfl„‡ÌÂÚÓ, ڇ͇ ˜Â ÔÂÍÓÏÂÌÓÚÓ ÔÓÌËʇ‚‡Ì ̇ Äç ÏÓÊ ‰‡ ‰Ó‚‰ ‰Ó ‚ÎÓ¯‡‚‡Ì ̇ Í‡ÈÌËfl ËÁıÓ‰. ãÂÍÓ ‰Ó ÛÏÂÂÌÓ Ôӂ˯ÂÌÓÚÓ Äç ˜ÂÒÚÓ ÒÔ‡‰‡ ÒÔÓÌÚ‡ÌÌÓ ÔÂÁ Ô˙‚ËÚ ÏËÌÛÚË ËÎË ˜‡Òӂ ̇ ÙÓ͇Î̇ڇ ËÒıÂÏËfl Ë Ó·ËÍÌÓ‚ÂÌÓ Ì ËÁËÒÍ‚‡ ÒÔ¯ÌÓ Ù‡χÍÓÎӄ˘ÌÓ Î˜ÂÌË (13). 燷β‰ÂÌËflÚ‡ ÔÓ͇Á‚‡Ú, ˜Â Ôӂ˯ÂÌÓÚÓ Äç  ÌÂÓ·ıÓ‰ËÏÓ Á‡ ÔÓ‰˙ʇÌÂÚÓ Ì‡ ÔÂÙÛÁËfl ‚ ËÒıÂÏËÁË‡Ì‡Ú‡ ÏÓÁ˙˜Ì‡ Ú˙Í‡Ì ‰Ó ÏÓÏÂÌÚ‡ ̇ Â͇̇ÎËÁË‡ÌÂÚÓ, ‡ ÒΉ ÚÓ‚‡ ‡ÚÂˇÎÌÓÚÓ Ì‡Îfl„‡Ì ÒÔ‡‰‡ ÒÔÓÌÚ‡ÌÌÓ ·ÂÁ ΘÂÌËÂ. åÓÊ ·Ë  ÌÂÔÓ‰ıÓ‰fl˘Ó ‰‡ Ò ҂‡Îfl Äç, ‡ÍÓ Ì Ò Á̇ ‰‡ÎË Á‡ÔÛ¯Â̇ڇ ‡ÚÂËfl ‚˜ Ò  Â͇̇ÎËÁË‡Î‡. é·˘ÓÔËÂÚÓÚÓ ÏÌÂÌË Â, ˜Â Úfl·‚‡ ‰‡ Ëχ ‚˙Á‰˙ʇÌ ÓÚ ÔË·„‡Ì ̇ ‡Ì-

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ÚËıËÔÂÚÂÌÁË‚ÌË ÔÂÔ‡‡ÚË, ‡ÍÓ Í˙‚ÌÓÚÓ Ì‡Îfl„‡Ì Ì Ô‚˯‡‚‡ 185 mm Hg Á��� ÒËÒÚÓÎÌÓÚÓ Ë 110 – Á‡ ‰Ë‡ÒÚÓÎÌÓÚÓ, ‚Íβ˜ËÚÂÎÌÓ Ë ÔË Ô‡ˆËÂÌÚËÚÂ, ÍÓËÚÓ ÔÓÎÛ˜‡‚‡Ú ÂÍÓÏ·Ë̇ÌÚÂÌ Ú˙͇ÌÂÌ Ô·ÁÏËÌÓ„ÂÌÂÌ ‡ÍÚË‚‡ÚÓ ÔÓ‡‰Ë ËÒ͇ ÓÚ ‚ÚÓ˘ÌÓÚÓ ‡Á‚ËÚË ̇ Ô‡ÂÌıËÏÂÌ Í˙‚ÓËÁÎË‚ (53,76). äÓ„‡ÚÓ Ò ÔËÒÚ˙Ô‚‡ Í˙Ï Î˜ÂÌË ̇ Ôӂ˯ÂÌÓÚÓ Äç, ÚÓ ÔÓÌËʇ‚‡ÌÂÚÓ Ì‡ ÒÚÓÈÌÓÒÚËÚ ÏÛ Úfl·‚‡ ‰‡ Ò Ô‡‚Ë Ô‰ԇÁÎË‚Ó. èÓÛ˜‚‡ÌËfl Ò Í‡ÎˆËÂ‚Ë ‡ÌÚ‡„ÓÌËÒÚË, Ôӂ‰ÂÌË ‚ ÓÒÚ‡Ú‡ Ù‡Á‡ ̇ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ, Ò‡ ÔÓ͇Á‡ÎË ÒÔÓÒÓ·ÌÓÒÚÚ‡ ËÏ ‰‡ ÔÓÌËʇ‚‡Ú ͇ÍÚÓ ÒËÒÚÓÎÌËÚÂ, ڇ͇ Ë ‰Ë‡ÒÚÓÎÌËÚ ÒÚÓÈÌÓÒÚË Ì‡ Í˙‚ÌÓÚÓ Ì‡Îfl„‡ÌÂ, ÌÓ Ú ҇ ÔÓ͇Á‡ÎË Ò˙˘Ó Ë Ì„‡ÚË‚ÌË ÂÙÂÍÚË ÔÓ ÓÚÌÓ¯ÂÌË ̇ Í‡ÈÌËfl ËÁıÓ‰ ÓÚ ËÌÒÛÎÚ‡ (3). ÄëÖ ËÌıË·ËÚÓË Ò‡ ÔÓ͇Á‡ÎË Ò‚ÓÈÒÚ‚ÓÚÓ ‰‡ ÔÓÌËʇ‚‡Ú Í˙‚ÌÓÚÓ Ì‡Îfl„‡ÌÂ, ÌÓ ·ÂÁ ‰‡ ÏÓ‰ÛÎË‡Ú Ò˙˘ÂÒÚ‚ÂÌÓ ˆÂ·‡ÎÌËfl Í˙‚ÂÌ ÚÓÍ. Ö‰ËÌÒÚ‚ÂÌËflÚ ‡ÌÚËıËÔÂÚÂÌÁË‚ÂÌ ÔÂÔ‡‡Ú ÓÚ ÌÓ‚Ó ÔÓÍÓÎÂÌËÂ, Á‡ ÍÓÈÚÓ Â Ôӂ‰ÂÌÓ ÔÓ‰Ó·ÌÓ ÔÓÛ˜‚‡Ì ‚ ÓÒÚËfl ÒÚ‡‰ËÈ Ì‡ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ,  candesartan (26). èË·„‡ÌÂÚÓ ÏÛ ÔÂÁ Ô˙‚ËÚ 7 ‰ÌË Ò  Ó͇Á‡ÎÓ ·ÂÁÓÔ‡ÒÌÓ, ‡ ÓÒ‚ÂÌ ÚÓ‚‡  ÔÓ͇Á‡Î Ë ·Î‡„ÓÔËflÚÂÌ ÂÙÂÍÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ Í‡ÈÌËfl ËÁıÓ‰ Ë ÎÂÚ‡ÎËÚÂÚ‡ ‰Ó 12-Ëfl ÏÂÒˆ ÓÚ Ì‡ÒÚ˙Ô‚‡ÌÂÚÓ Ì‡ ËÌÒÛÎÚ‡ (66). Cochrane Review Ó·Ó·˘‡‚‡ 5 Ó„‡Ì˘ÂÌË ÔÓÛ˜‚‡ÌËfl, ‚Íβ˜‚‡˘Ë Ó·˘Ó 218 ·ÓÎÌË, ‡Ì‰ÓÏËÁË‡ÌË ÒÔflÏÓ ÛÔÓÚ·‡Ú‡ ̇ ÌËÏÓ‰ËÔËÌ, ÌË͇‰ËÔËÌ, ͇ÔÚÓÔËÎ, ÔÂË̉ÓÔËÎ, ÍÎÓÌˉËÌ ËÎË „ÎˈÂÓÎ ÚËÌËÚ‡Ú, Ò˙ÔÓÒÚ‡‚ÂÌË ÒÔflÏÓ Ô·ˆÂ·Ó ËÎË ÍÓÌÚÓÎÌË „ÛÔË. ç‡Ô‡‚ÂÌËflÚ ËÁ‚Ó‰ Â, ˜Â ‰‡ÌÌËÚ ҇ ̉ÓÒÚ‡Ú˙˜ÌË Á‡ ˆflÎÓÒÚ̇ ÓˆÂÌ͇ ̇ ‚Á‡ËÏÓÓÚÌÓ¯ÂÌËflÚ‡ ÏÂÊ‰Û Äç Ë ÍÎËÌ˘ÌËfl ËÁıÓ‰ ÔË Ô‡ˆËÂÌÚË ‚ ‡ÏÍËÚ ̇ Ô˙‚ËÚ 2 Ò‰ÏËˆË ÓÚ Ì‡ÒÚ˙Ô‚‡ÌÂÚÓ Ì‡ ËÒıÂÏ˘ÌËfl ËÌÒÛÎÚ (11). ÄÍÚÛ‡ÎÌËÚ ̇ÒÓÍË ÔÓ͇Á‚‡Ú, ˜Â ÌflÍÓË Ô‡ÂÌÚÂ‡ÎÌË ‡„ÂÌÚË Í‡ÚÓ labetalol, Ò ÚËÚË‡Ú ÎÂÒÌÓ Ë ËÏ‡Ú ÏËÌËχÎÂÌ ‚‡ÁӉ˷ڇÚË‚ÂÌ ÂÙÂÍÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ˆÂ·‡ÎÌËÚ Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚Â. Ç ÌflÍÓË ÒÎÛ˜‡Ë ̇ ÂÍÒˆÂÒË‚ÌÓ ÔÓ͇˜‚‡Ì ̇ ‡ÚÂˇÎÌÓÚÓ Ì‡Îfl„‡ÌÂ, ËÌÚ‡‚ÂÌÓÁ̇ڇ ËÌÙÛÁËfl ̇ natrium nitropruside ÏÓÊ ‰‡ Ò Ó͇Ê ÌÂÓ·ıÓ‰Ëχ Á‡ ‡‰ÂÍ‚‡ÚÌËfl ÍÓÌÚÓΠ̇ Äç. éÒÌÓ‚ÌË Ò‰ÒÚ‚‡ ‚ ·ÂÁÓÔ‡ÒÌÓÚÓ ÚÂÚË‡Ì ̇ ·ÓÎÌËÚ ÓÒÚ‡‚‡Ú ÄëÖ-ËÌıË·ËÚÓËÚ (1). äéçíêéã çÄ ÄêíÖêàÄãçÄíÄ äàëãéêéÑçÄ ëÄíìêÄñàü èÓ‰‰˙ʇÌÂÚÓ Ì‡ ‡‰ÂÍ‚‡Ú̇ Ú˙͇Ì̇ ÓÍÒË„Â̇ˆËfl  ÓÚ „ÓÎflÏÓ Á̇˜ÂÌË ÔÂÁ ÔÂËÓ‰‡ ̇ ÓÒÚ‡ ÏÓÁ˙˜Ì‡ ËÒıÂÏËfl, Ò Ó„Î‰ Ô‰ÓÚ‚‡Úfl‚‡ÌÂÚÓ Ì‡ ıËÔÓÍÒËflÚ‡ Ë ÔÓÚÂ̈ˇÎÌÓÚÓ ‚ÎÓ¯‡‚‡Ì ̇ Ì‚ÓÎӄ˘̇ڇ Û‚‰‡. 臈ËÂÌÚËÚÂ Ò ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ Úfl·‚‡ ‰‡ ·˙‰‡Ú ÏÓÌËÚÓË‡ÌË ˜ÂÁ Í˙‚ÌÓ„‡ÁÓ‚ ‡Ì‡ÎËÁ Ë ÔÛÎÒÓ‚‡ ÓÍÒËÏÂÚËfl, ͇ÚÓ ˆÂÎÚ‡  ÔÓÒÚË„‡ÌÂÚÓ Ì‡ ÍËÒÎÓÓ‰ÌÓ Ò‡ÚÛ‡ˆËÓÌÌÓ ÌË‚Ó Ì‡‰ 95%. èÂÔÓ˙˜‚‡ Ò Ò˙˘Ó ÔÓ‰‡‚‡ÌÂÚÓ Ì‡ ‰ÓÔ˙ÎÌËÚÂÎÂÌ ÍËÒÎÓÓ‰ ̇ ıËÔÓÍÒÂÏ˘ÌËÚ ԇˆËÂÌÚË. ôÓ Ò ÓÚ̇Òfl ‰Ó ıËÔÂ·‡̇ڇ ÍËÒÎÓӉ̇ ÏÂÚÓ‰Ë͇, ÚÓ ‰‡ÌÌËÚ Á‡ ÔÓÎÁÓÚ‚ÓÌÓÒÚÚ‡ Ò‡ ‡·ÒÓβÚÌÓ Ì‰ÓÒÚ‡Ú˙˜ÌË, Á‡ ‰‡ ÏÓÊ ‰‡ Ò ̇Ô‡‚Ë Ó·ÓÒÌÓ‚‡Ì‡ ÔÂÔÓ˙͇ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÌÂÈÌÓÚÓ ÔËÎÓÊÂÌË (1, 2, 31) èéÇãàüÇÄçÖ çÄ ÇéãÖåàüíÄ à ÇàëäéáàíÖíÄ çÄ äêöÇíÄ èÓÛ˜‚‡ÌËflÚ‡, ̇Ô‡‚ÂÌË ‚˙ıÛ ·ÓÎÌË Ì‡ ÍÓËÚÓ Ò‡ ÔË·„‡ÌË ËÁÓ‚ÓÎÂÏ˘̇ ıÂÏÓ‰ËÎÛˆËfl Ò ‰ÂÍÒÚ‡Ì (72), ͇ÍÚÓ Ë ÔÓ-Í˙ÒÌËÚ ÔÓÛ˜‚‡ÌËfl ‚˙ıÛ ÒÎÛ˜‡Ë ̇ ÔË·„‡Ì‡ ıËÔÂ‚ÓÎÂÏ˘̇ ıÂÏÓ‰ËÎÛˆËfl ˜ÂÁ ‚Íβ˜‚‡Ì ̇ ÔÂÌÚ‡ÒÍÓ·fl· (36), Ì ҇ ‰‡ÎË ÔÓÁËÚË‚ÂÌ ÂÁÛÎÚ‡Ú. åÂÚ‡-


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

‰ÂÍÂÏ‚Ë, 2007

‡Ì‡ÎËÁ˙Ú Ì‡ 18 ÍÓÌÚÓÎÌË ÔÓÛ˜‚‡ÌËfl (6) ÔÓ͇Á‚‡, ˜Â ÔÓÒÓ˜ÂÌËÚ Ôӈ‰ÛË Ì ҇ ‰‡ÎË Ò˙˘ÂÒÚ‚ÂÌÓ ÓÚ‡ÊÂÌË ‚˙ıÛ ÒÏ˙ÚÌÓÒÚÚ‡ ËÎË ÒÚÂÔÂÌÚ‡ ̇ ËÌ‚‡ÎˉËÁ‡ˆËfl.

5. 6. 7.

ÑÖäéåèêÖëàÇçÄ ïÖåàäêÄçàÖäíéåàü ÖÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÏÓ‰ÂÎË ÔÓ͇Á‚‡Ú, ˜Â ‡Ì̇ڇ ıÂÏË͇ÌËÂÍÚÓÏËfl Ëχ ÔÓÎÓÊËÚÂÎÂÌ Ì‚ÓÔÓÚÂÍÚË‚ÂÌ ÂÙÂÍÚ, ‰ۈË‡˘ Ó·Âχ ̇ ËÌÙ‡ÍÚ̇ڇ ÁÓ̇ Ë ÓÒÚ‡Ú˙˜ÌËfl Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ. íÓ‚‡ Ò ÓÚ‰‡‚‡ ̇ ÔÓ‰Ó·Â̇ڇ ıÂÏÓ‰Ë̇ÏË͇, ‰˙Îʇ˘‡ Ò ̇ ÔÓ‰Ó·ÂÌÓÚÓ ÏÓÁ˙˜ÌÓ ÔÂÙÛÁËÓÌÌÓ Ì‡Îfl„‡Ì ÔÓ ÎÂÔÚÓÏÂÌË̄‡ÎÌËÚ ÍÓ·ÚÂ‡ÎË (28, 30). èÂ‰Ë ÌflÍÓÎÍÓ ‰ÂÒÂÚËÎÂÚËfl ·Â¯Â ÓÒ˙˘ÂÒÚ‚Â̇ Ô˙‚‡Ú‡ ‰ÂÍÓÏÔÂÒ˂̇ ıÂÏËÍ‡ÌËÂÍÚÓÏËfl, ‡ ÂÁÛÎÚ‡ÚËÚ ÓÚ ÂÔËÁӉ˘ÌËÚ ÔÛ·ÎË͇ˆËË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ò‡ Ó·Ó·˘ÂÌË ÔÂÁ 1995 „Ó‰Ë̇ ÓÚ Ú. ̇. „ÛÔ‡ ÓÚ ï‡È‰ÂηÂ„ Ë ÔÓ͇Á‚‡Ú ÔÓ-ÌËÒ͇ ÒÏ˙ÚÌÓÒÚ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ÂÍÓÏÔÂÒ˂̇ Í‡ÌËÂÍÚÓÏËfl, ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎ̇ڇ „ÛÔ‡. å‡ÒË‚ÌËflÚ ÛÌË·ÚÂ‡ÎÂÌ ıÂÏËÒÙÂˇÎÂÌ ËÌÙ‡ÍÚ ‚ ·‡ÒÂÈ̇ ̇ ÌflÍÓfl ÓÚ Ò‰ÌËÚ ÏÓÁ˙˜ÌË ‡ÚÂËË ˜ÂÒÚÓ Ô‰ËÁ‚ËÍ‚‡ ÔÓ„ÂÒË‚ÂÌ ÔÓÒÚËÒıÂÏ˘ÂÌ Â‰ÂÏ, Ò Ï‡ÎË„ÌÂÌÓ ‡Á‚ËÚË ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÒÏ˙ÚÌÓÒÚ Ì‡‰ 80% ÔË ÍÓÌ‚Â̈ËÓ̇Î̇ڇ ÚÂ‡ÔËfl. ç‡ ÚÓÁË ÂÚ‡Ô ‚Ò Ӣ Ìflχ Ó·fl‚ÂÌ ÂÁÛÎÚ‡Ú ÓÚ ‡Ì‰ÓÏËÁË‡ÌË ÍÓÌÚÓÎË‡ÌË ÔÓÛ˜‚‡ÌËfl Á‡ ÔÓÎÁ‡Ú‡ ÓÚ ‰ÂÍÓÏÔÂÒ˂̇ڇ ıËÛ„Ëfl ÔË Ï‡ÎË„ÌÂÌË ËÌÙ‡ÍÚË. êÂÁÛÎÚ‡ÚËÚ ÓÚ HAMLET (Hemicraniectomy After MCA Infarction with Life-Treatening Edema Trial) ‚ÂÓflÚÌÓ ˘Â ÌË ÔÓÏÓ„Ì‡Ú ‚ ·˙‰Â˘ÂÚÓ Ôӂ‰ÂÌËÂ. Ç Ú‡ÁË ‚˙Á͇, Ó˜‡Í‚‡Ï ÔÛ·ÎËÍÛ‚‡ÌÂÚÓ Ì‡ ÂÁÛÎÚ‡ÚËÚ ÓÚ CoolStroke Trial, ÍÓÂÚÓ ÍÓÏ·ËÌË‡ ÔË·„‡ÌÂÚÓ Ì‡ ‡Ì̇ ÛÏÂÂ̇ ıËÔÓÚÂÏËfl (ÔÂÁ Ô˙‚ËÚ 24 ˜‡Ò‡) Ë ıÂÏËÍ‡ÌËÂÍÚÓÏËfl ÔË Ô‡ˆËÂÌÚË ÔÓ‰ 65 „Ó‰ËÌË Ë ‚ËÒÓÍ ËÒÍ ÓÚ ‡Á‚ËÚË ̇ χÎË„ÌÂÌ ËÌÙ‡ÍÚ. çÂÙ‡χÍÓÎӄ˘̇ڇ Ì‚ÓÔÓÚÂ͈Ëfl ‚Íβ˜‚‡ ‚Ò˘ÍË ËÌÚÂ‚Â̈ËË, ÍÓËÚÓ Ô‰ԇÁ‚‡Ú ÏÓÁ˙͇ ÓÚ Ù‡χÍÓÎӄ˘̇ Û‚‰‡ ÒΉ ÓÍÎÛÁËfl ̇ ˆÂ·‡Î̇ ‡ÚÂËfl. óÂÚËË Ò‡ ÙËÁËÓÎӄ˘ÌËÚ ԇ‡ÏÂÚË (Äç, Í˙‚ÌÓÁ‡ı‡ÌÓ ÌË‚Ó, ÚÂÎÂÒ̇ ÚÂÏÔÂ‡ÚÛ‡ Ë ÍËÒÎÓӉ̇ Ò‡ÚÛ‡ˆËfl), Á‡ ÍÓËÚÓ Ò ҘËÚ‡, ˜Â Ò‡ ÌÂÁ‡‚ËÒËÏË ÔÓ„ÌÓÒÚ˘ÌË Ù‡ÍÚÓË ÔË ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ, ÍÓÂÚÓ Â ÓÒÌÓ‚‡ÌË ‚Ò˘ÍË ˙ÍÓ‚Ó‰ÒÚ‚‡ Á‡ ΘÂÌË ̇ ÓÒÚ˙ ËÒıÂÏ˘ÂÌ ËÌÒÛÎÚ ‰‡ ÔÂÔÓ˙˜‚‡Ú ÔÓÒÚË„‡ÌÂ Ë ÔÓ‰‰˙ʇÌ ̇ ÓÔÚËχÎÂÌ ÍÓÌÚÓÎ ‚˙ıÛ ÚÂÁË Ô‡‡ÏÂÚË - Ô‰ıÓÒÔËÚ‡ÎËÁ‡ˆËÓÌÌÓ, ÔÓ ‚ÂÏ ̇ Ú‡ÌÒÔÓÚË‡ÌÂÚÓ Ë ‚ ÓÚ‰ÂÎÂÌËflÚ‡ Á‡ ΘÂÌË ̇ ÓÒÚ˙ ËÌÒÛÎÚ (Stroke units), Í˙‰ÂÚÓ ÚÂÁË Ô‡‡ÏÂÚË ‰‡ Ò ÔÓÚÓÍÓÎË‡Ú, Ò˙„·ÒÌÓ ÔËÂÚËÚ ÔÂÔÓ˙ÍË Ë ˙ÍÓ‚Ó‰ÒÚ‚‡. åÂÚ‡-‡Ì‡ÎËÁËÚ ÔÓ͇Á‚‡Ú, ˜Â ÂÙÂÍÚË‚ÌÓÒÚÚ‡ ÓÚ Ò˙Á‰‡‚‡ÌÂÚÓ Ì‡ Stroke units ‚˜  ·ÂÁÒÔÓ̇ (Stroke Trialis’s Collaboration) (41, 73), Ú˙È Í‡ÚÓ ÔË·„‡ÌÂÚÓ Ì‡ ÔÓÚÓÍÓÎÌË „ËÊË Á‡ Ô‡ˆËÂÌÚËÚ ‚ Úflı, ̇‰ Ò ‡‰ÂÍ‚‡ÚÌÓ ÚÂ‡Ô‚Ú˘ÌÓ Ôӂ‰ÂÌË ‚Ó‰flÚ ‰Ó ̇χÎfl‚‡Ì ̇ ÒÏ˙ÚÌÓÒÚÚ‡, Ó„‡Ì˘‡‚‡Ì ‚ÓβˆËflÚ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, Ò˙Í‡˘‡‚‡Ì ̇ ıÓÒÔËÚ‡ÎËÁ‡ˆËÓÌÌÓÚÓ ‚ÂÏÂ Ë Ò˙Á‰‡‚‡Ì ̇ ÔÓ-·Î‡„ÓÔËflÚ̇ ·‡Á‡ Á‡ ‡ÌÌÓ Á‡ÔÓ˜‚‡Ì ̇ Âı‡·ËÎËÚ‡ˆËfl.

8. 9.

10. 11. 12. 13. 14. 15.

16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27.

28.

29.

30.

31.

32.

ãàíÖêÄíìêÄ 1.

2. 3. 4.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- ÑËÏËÚ˙ å‡Ò·Ó‚, ‰ÓÍÚÓ è˙‚‡ åÅÄã – ëÓÙËfl, ç‚ÓÎӄ˘ÌÓ ÓÚ‰ÂÎÂÌË ·ÛÎ. “è‡Úˇı Ö‚ÚËÏËÈ” ‹ 37 ëÓÙËfl 1000 íÂÎ. 981 08 01 E-mail: maslarovdb@abv.bg


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éË„Ë̇ÎÌË ÒÚ‡ÚËË çÖÇêéçÄãçà ñÖêéàÑãàèéîìëñàçéáà Ç ÑÖíëäÄ ÇöáêÄëí: çÖ íéãäéÇÄ êÖÑäà, çé íêìÑçé êÄáèéáçÄÇÄçà Ç ÅöãÉÄêàü èeÚfl ÑËÏÓ‚‡1, ÇÂÌÂÚ‡ ÅÓÊËÌÓ‚‡1, áÓÎÚ‡Ì ãÛ͇˜2 ÑÂÚÒ͇ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇, ìëÅÄãçè “ë‚. ç‡ÛÏ”, ëÓÙËfl, Å˙΄‡Ëfl 2åÂÚ‡·ÓÎËÚ̇ ··Ó‡ÚÓËfl ̇ äÎËÌË͇ ÔÓ è‰ˇÚËfl, ìÌË‚ÂÒËÚÂÚ ï‡Ï·Û„, ÉÂχÌËfl 1

SUMMARY NEURONAL CEROID LIPOFUSCINOSES IN CHILDHOOD: NOT SO RARE, BUT DIFFICULT DIAGNOSIS IN BULGARIA Petia Dimova1, Veneta Bojinova1, Zoltan Lukacs2 1 Clinic of Child Neurology, St. Naum University Hospital of Neurology, Sofia 2Clinic of Pediatrics with Metabolic Laboratory, University of Hamburg, Germany We present four cases with confirmed diagnosis of neuronal ceroid lipofuscinosis - type I (NCL1) in one, and type II (NCL2) - in 3 cases. In all children progressive pyramidal, cerebellar, myoclonic and epileptic syndromes were present, as well as severe dementia, blindness and deafness. These symptoms developed more rapidly in NCL1 (for about 8 months) than in NCL2 (for 1 to 3 years). The neuroimaging examinations showed initial cerebellar atrophy with subsequent global brain atrophy (NCL2) and initial total brain atrophy (NCL1). The EEG demonstrated abnormal background and generalized paroxysmal activity. The diagnosis NCL was established in all patients by means of verifying the decreased enzymatic activity of tripeptidyl-peptidasis in the patients with NCL2 and of palmitoyl-protein-thioesterasis in the child with NCL1, respectively. The examination was performed in a dry blood spot on a filter paper. KEY WORDS: neuronal, ceroidlipofuscinosis, epilepsy, myoclonic, progressive, EEG. êÖáûåÖ è‰ÒÚ‡‚flÏ ˜ÂÚËË ÒÎÛ˜‡fl Ò ÔÓÚ‚˙‰Â̇ ‰Ë‡„ÌÓÁ‡ ç‚Ó̇Î̇ ˆÂÓˉÎËÔÓÙÛÒˆËÌÓÁ‡: I ÚËÔ (NCL1) ÔË Â‰ÌÓ ‰ÂÚÂ Ë ¨¨ ÚËÔ (NCL2) - ÔË 3. èË ‚Ò˘ÍË ‰Âˆ‡ Ò ̇·Î˛‰‡‚‡Ú ÔÓ„ÂÒË‚ÂÌ Í‚‡‰ËÔË‡ÏˉÂÌ, ˆÂ·Â·ÂÌ, ÏËÓÍÎÓÌ˘ÂÌ Ë ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏË, ÛÒÔÓ‰ÌÓ Ò ÚÂÊ͇ ‰ÂÏÂ̈Ëfl, ÒÎÂÔÓÚ‡ Ë „ÎÛıÓÚ‡. Ö‚ÓβˆËflÚ‡  ÔÓ-·˙Á‡ ÔË NCL1 (Á‡ 8 ÏÂÒˆ‡), ÓÚÍÓÎÍÓÚÓ ÔË NCL2 (ÏÂÊ‰Û 1 Ë 3 „Ó‰ËÌË). ç‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl ‡ÁÍË‚‡Ú ̇˜‡Î̇ ˆÂ·Â·̇ ‡ÚÓÙËfl Ò ÔÓÒΉ‚‡˘‡ ÚÂÊ͇ „ÎÓ·‡Î̇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl (NCL2) ËÎË Ì‡˜‡Î̇ Ó·˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl (NCL1), ‡ ÖÖÉ ‰ÂÏÓÌÒÚË‡ ‡·ÌÓÏ̇ ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ Ë „ÂÌÂ‡ÎËÁË‡ÌË Ô‡ÓÍÒËÁÏË. Ñˇ„ÌÓÁ‡Ú‡ NCL ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË ·Â ÔÓÚ‚˙‰Â̇ ˜ÂÁ ‰Ó͇Á‚‡Ì ̇ ÔÓÌËÊÂ̇ ÂÌÁËÏ̇ ‡ÍÚË‚ÌÓÒÚ, Ò˙ÓÚ‚ÂÚÌÓ Ì‡ ÚËÔÂÔÚˉËÎ-ÔÂÔÚˉ‡Á‡ ÔË ‰Âˆ‡Ú‡ Ò NCL2 Ë Ì‡ Ô‡ÎÏËÚÓËÎÔÓÚÂËÌ-ÚËÓÂÒÚÂ‡Á‡ ÔË ‰ÂÚÂÚÓ Ò NCL1, ÍÓÂÚÓ ·Â ËÁ‚˙¯ÂÌÓ ‚ ÒÛı‡ ͇Ô͇ Í˙‚ ‚˙ıÛ ÙËÎÚ˙̇ ı‡ÚËfl. äãûóéÇà Ñìåà: Ì‚Ó̇Î̇, ˆÂÓˉÎËÔÓÙÛÒˆËÌÓÁ‡, ÂÔËÎÂÔÒËfl, ÖÖÉ, ÔÓ„ÂÒ˂̇, ÏËÓÍÎÓÌ˘̇. ÇöÇÖÑÖçàÖ ç‚Ó̇ÎÌËÚ ˆÂÓˉÎËÔÓÙÛÒˆËÌÓÁË (NCLs) Ò‡ „ÛÔ‡ ÓÚ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌË Â̈ÂÙ‡ÎÓÔ‡ÚËË, ı‡‡ÍÚÂËÁË‡˘Ë ÒÂ Ò Ì‡ÚÛÔ‚‡Ì ̇ ‡‚ÚÓÙÎÛÓÂÒˆÂÌÚÂÌ ÎËÔÓÔË„ÏÂÌÚ ‚ Ì‚ÓÌËÚÂ Ë ‰Û„Ë ÍÎÂÚ˙˜ÌË ÚËÔÓ‚Â (4,6-9,14). í ÒÂ

Í·ÒËÙˈË‡Ú ‚ ˜ÂÚËË ÓÒÌÓ‚ÌË ÔÓ‰‚ˉ‡ ÒÔÓ‰ ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓ, ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË Ë ÛÎÚ‡ÒÚÛÍÚÛÌËÚ ı‡‡ÍÚÂËÒÚËÍË Ì‡ ̇ÚÛÔ‡ÌËfl ÍÎÂÚ˙˜ÂÌ Ï‡ÚÂˇΠ(4,6-9,14). ç‡È-˜ÂÒÚË Ë ‰Ó· ÔÓÁ̇ÚË Ò‡ ÒΉÌËÚ ◊‰ÂÚÒÍË” ÙÓÏË: ‡Ì̇ڇ ‰ÂÚÒ͇ (ËÎË ËÌÙ‡ÌÚËÎ̇) NCL1 (INCL), ÛÎÚ‡ÒÚÛÍÚÛÌÓ ı‡‡ÍÚÂËÁË‡˘‡ ÒÂ Ò „‡ÌÛ·ÌË ÓÒÏËÓÙËÎÌË ‰ÂÔÓÁËÚË (GRODs); Í·Ò˘ÂÒ͇ڇ Í˙Ò̇ ‰ÂÚÒ͇ NCL2 (LINCL), ÔË ÍÓflÚÓ Ò ��ÒÚ‡ÌÓ‚fl‚‡Ú ‚˙ÚÂÍÎÂÚ˙˜ÌË ÍÛ‚ËÎË̇ÌË ÚÂΈ‡ (CVBs); Ë ˛‚ÂÌËÎ̇ڇ NCL3 (JNCL), ÔË ÍÓflÚÓ Ò ‰ÂÏÓÌÒÚË‡Ú Ú.̇. ◊fingerprint profiles” (FPPs) (6,9). éÒ‚ÂÌ Úflı Ë ÏÌÓ„Ó fl‰Í‡Ú‡ ·ÓÎÂÒÚ Ì‡ Kufs (NCL ÔË ‚˙Á‡ÒÚÌË), ‚ ÔÓÒΉÌËÚ 5-10 „Ó‰ËÌË ·flı‡ ÔÛ·ÎËÍÛ‚‡ÌË Ó˘Â ÌflÍÓÎÍÓ ÔÓ‰ÍË ÙÓÏË Ë ‚‡ˇÌÚË, ÍÓÂÚÓ Ì‡ÎÓÊË ÌÓ‚‡ Í·ÒËÙË͇ˆËfl ̇ ‚˜ ËÁ‚ÂÒÚÌËÚ 10 ÚËÔ‡ (3,4, 6-9). ÑÂÚÒÍËÚ NCLs ͇ÚÓ „ÛÔ‡ Ò‡ ̇È-˜ÂÒÚ‡Ú‡ Ô˘Ë̇ Á‡ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡Ì ‚ Ú‡ÁË ‚˙Á‡ÒÚ. Ç˙ÔÂÍË ÏÌÓ„ÓÓ·‡ÁËÂÚÓ ÒË, ◊‰ÂÚÒÍËÚ” NCL ËÏ‡Ú ÏÌÓ„Ó Ó·˘Ë ı‡‡ÍÚÂËÒÚËÍË. ÇÒ˘ÍË Ú ÒÔ‡‰‡Ú Í˙Ï „ÛÔ‡Ú‡ ̇ ÔÓ„ÂÒË‚ÌËÚ ÏËÓÍÎÓÌ˘ÌË ÂÔËÎÂÔÒËË (4,6-8,12) Ë ‚Ó‰flÚ ‰Ó ÁËÚÂÎÌË Ì‡Û¯ÂÌËfl, ‚ÍÎ. ÒÎÂÔÓÚ‡. ÑÂÏÂ̈Ëfl ‚ ‡Á΢̇ ÒÚÂÔÂÌ Ò ̇·Î˛‰‡‚‡ ‚Ë̇„Ë, ͇ÍÚÓ Ë ÔË‡ÏˉÌË Ë/ËÎË ÂÍÒÚ‡ÔË‡ÏˉÌË Ì‡Û¯ÂÌËfl Ò ËÁfl‚‡ ‚ ‡Á΢ÌË ÂÚ‡ÔË ÓÚ ·ÓÎÂÒÚÚ‡. ñÂ·Â·̇ ‡Ú‡ÍÒËfl Ò ̇·Î˛‰‡‚‡ ‚ ̇ÈÚÂÊ͇ ÒÚÂÔÂÌ ÔË Í˙Ò̇ڇ ‰ÂÚÒ͇ ÙÓχ (LINCL). ç‚ÓÙËÁËÓÎӄ˘ÌËÚÂ Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ËÁÒΉ‚‡ÌËfl Ò‡ Ò Ô‡ÚÓÎӄ˘ÌË Ì‡ıÓ‰ÍË, ˜ÂÒÚÓ ı‡‡ÍÚÂÌË Á‡ ‚ÒÂÍË ÚËÔ NCL, ÍÓÂÚÓ ÔÓ‰ÔÓχ„‡ ‰Ë‡„ÌÓÁ‡Ú‡. ÑÓ ÒÍÓÓ Úfl Ò ÔÓÚ‚˙ʉ‡‚‡¯Â ‰ËÌÒÚ‚ÂÌÓ ˜ÂÁ ‰Ó͇Á‚‡ÌÂÚÓ Ì‡ ÎËÔÓÙÛÒˆËÌÓ‚Ë ‚Íβ˜‚‡ÌËfl ‚ ‡Á΢ÌË Ú˙͇ÌË, ‡ ̇ÔÓÒΉ˙Í Ë ˜ÂÁ ‰Ó͇Á‚‡ÌÂÚÓ Ì‡ ı‡‡ÍÚÂÌËfl Á‡ ‚Òfl͇ ÙÓχ „ÂÌÂÚ˘ÂÌ ‰ÂÙÂÍÚ ËÎË Ì‡ ÏÂÚ‡·ÓÎËÚÂÌ ‰ÂÙˈËÚ, ͇ÚÓ ÔÓÒΉÌÓÚÓ Â ‚˙ÁÏÓÊÌÓ Ò‡ÏÓ ÔË ÌflÍÓË ÓÚ ÙÓÏËÚ (3,4,5,12). Ñ‚ÂÚ ̇È-˜ÂÒÚË ÙÓÏË - NCL1 Ë NCL2, Ò Ô˘ËÌfl‚‡Ú ÓÚ ‰ÂÙˈËÚ Ì‡ ÎËÁÓÁÓÏÌË ÂÌÁËÏË, Ò˙ÓÚ‚ÂÚÌÓ Ô‡ÎÏËÚÓËÎ-ÔÓÚÂËÌ-ÚËÓÂÒÚÂ‡Á‡ 1 (PPT1) Ë ÚËÔÂÔÚˉËÎ-ÔÂÔÚˉ‡Á‡ 1 (TPP1), ˜ËÂÚÓ ‰Ó͇Á‚‡Ì ‚Ò Ӣ Ò ËÁ‚˙¯‚‡ ‚ χÎÍÓ Î‡·Ó‡ÚÓËË ÔÓ Ò‚ÂÚ‡ (5). çÂÁ‡‚ËÒËÏÓ ÓÚ Òı‚‡˘‡ÌÂÚÓ Á‡ ÔÂӷ·‰‡‚‡Ì ̇ NCL Ò‰ ‰ÂÚÒÍËÚ ÔÓ„ÂÒË‚ÌË Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌË Á‡·ÓÎfl‚‡ÌËfl, ‰Ó ÒÍÓÓ Û Ì‡Ò Ìflχ¯Â ÓÔËÒ‡ÌË ÒÎÛ˜‡Ë, ‚ÍÎ. Ò „ÂÌÂÚ˘ÌÓ ËÎË ÏÂÚ‡·ÓÎËÚÌÓ ÔÓÚ‚˙‰Â̇ ‰Ë‡„ÌÓÁ‡. çË Ô‰ÒÚ‡‚flÏ Ô˙‚ËÚ ˜ÂÚËË ‰Âˆ‡ ‚ Å˙΄‡Ëfl, ÔË ÍÓËÚÓ ‰Ë‡„ÌÓÁ‡Ú‡ Ë ÒË̉ÓÏ̇ڇ Í·ÒËÙË͇ˆËfl ·flı‡ ÔÓÚ‚˙‰ÂÌË ˜ÂÁ ÏÂÚ‡·ÓÎËÚÌÓ ËÁÒΉ‚‡Ì ̇ ÒÛı‡ ͇Ô͇ Í˙‚ ‚˙ıÛ ÙËÎÚ˙̇ ı‡ÚËfl Ë ‰Ó͇Á‚‡Ì ̇ ÂÌÁËÏÌËfl ‰ÂÙˈËÚ. åÄíÖêàÄãà à åÖíéÑà ÇÒ˘ÍË Ô‡ˆËÂÌÚË (‚˙Á‡ÒÚ 1 1/2 - 6 „.) Ò‡ ÔÓÒΉfl‚‡ÌË ‚ Ñçä Á‡ ÔÂËÓ‰ ÓÚ 2 ‰Ó 6 „Ó‰ËÌË, ÔÓ ‚ÂÏ ̇ ÏÌÓ„ÓÍ‡ÚÌË ıÓÒÔËÚ‡ÎËÁ‡ˆËË Ë ‡Ï·Û·ÚÓÌË Ô„ÎÂ‰Ë ÓÚ 2003 „. ‰Ó ‡‚„ÛÒÚ 2007 „. èË ÚË ÓÚ ‰Âˆ‡Ú‡ (ÏÓϘÂÚ‡)  ÔÓÚ‚˙‰Â̇ ‰Ë‡„ÌÓÁ‡Ú‡ NCL2, ‡ Ò‡ÏÓ Â‰ÌÓ ‰ÂÚ (ÏÓÏ˘Â) Â Ò ÛÒÚ‡ÌÓ‚Â̇ NCL1. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ Ôӂ‰ÂÌË Ï‡„ÌËÚÌÓ-ÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl (åêí) Ë ÍÓÏ-


‰ÂÍÂÏ‚Ë, 2007

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

Ô˛Ú˙ÌÓ-ÚÓÏÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì ̇ „·‚ÂÌ ÏÓÁ˙Í, ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙËfl (ÖÖÉ), ËÁÒΉ‚‡Ì ̇ ÁËÚÂÎÌË Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË (áÖè) Ë/ËÎË ÒÚ‚ÓÎÓ‚Ë ÒÎÛıÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË (ëëÖè), ÏÂÚ‡·ÓÎËÚÂÌ ÒÍËÌËÌ„, ÂÎÂÍÚÓÏËÓ„‡ÙËfl Ë ÂÎÂÍÚÓÌ‚Ó„‡ÙËfl (ÖåÉË ÖçÉ), ͇ÍÚÓ Ë ËÁÒΉ‚‡Ì ̇ ÂÌÁËÏ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì‡ PPT1 Ë TPP1. ÖåÉ Ë ÖçÉ Ò‡ Ôӂ‰ÂÌË Ò ‰ËÙÂÂ̈ˇÎÌÓ-‰Ë‡„ÌÓÒÚ˘̇ ˆÂÎ ‚ ̇ÒÓ͇ ÌflÍÓË Î‚ÍÓ‰ËÒÚÓÙËË Ë ÏËÚÓıÓ̉ˇÎÌË Â̈ÂÙ‡ÎÓÏËÓÔ‡ÚËË, ͇ÚÓ Ì ҇ ÔÓ͇Á‡ÎË ÓÚÍÎÓÌÂÌËfl ÓÚ ÌÓχڇ Á‡ ‚˙Á‡ÒÚÚ‡ ÔË ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚÂ. åÂÚ‡·ÓÎËÚÌËflÚ ÒÍËÌËÌ„, Ôӂ‰ÂÌ Û Ì‡Ò ‚ ̇ÒÓ͇ ̇È-˜ÂÒÚË Î‚ÍÓ‰ËÒÚÓÙËË, ÎËÁÓÁÓÏÌË ·ÓÎÂÒÚË, ‡ÏËÌÓ‡ˆË‰ÛËË Ë Ó„‡Ì˘ÌË ‡ˆË‰ÛËË,  ·ËÎ ·ÂÁ ÓÚÍÎÓÌÂÌËfl ÔË ‚Ò˘ÍË ‰Âˆ‡. àÁÒΉ‚‡ÌÂÚÓ Ì‡ ÂÌÁËÏ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì‡ ‰‚‡Ú‡ „ÓÂÔÓÒÓ˜ÂÌË ÂÌÁËχ ·Â ËÁ‚˙¯ÂÌÓ ‚ åÂÚ‡·ÓÎËÚ̇ڇ ··Ó‡ÚÓËfl ̇ ÑÂÚÒ͇ ÍÎËÌË͇ Í˙Ï ìÌË‚ÂÒËÚÂÚ‡ ‚ ï‡Ï·Û„, ‚ ÒÛı‡ ͇Ô͇ Í˙‚ ‚˙ıÛ Gouthrie ͇Ú‡ (ÙËÎÚ˙̇ ı‡ÚËfl, ËÁÔÓÎÁ‚‡Ì‡ ӷ˘‡ÈÌÓ Á‡ ‰Ó͇Á‚‡Ì ̇ ˜ÂÒÚË ÏÂÚ‡·ÓÎËÚÌË ‰ÂÙÂÍÚË ÔË ÌÓ‚ÓÓ‰ÂÌË). çÓχÎ̇ڇ ‡ÍÚË‚ÌÓÒÚ Ì‡ íêê1  0.1-2.28 ÌÏÓÎ/ÔÂÚÌÓ, ‡ ̇ êêí1 0.25-2.4 ÌÏÓÎ/ÔÂÚÌÓ, ͇ÚÓ Ë ‰‚‡Ú‡ ÂÌÁËχ Ò ËÁÒΉ‚‡Ú ÔË ÍÓÌÚÓ· – ËÁÒΉ‚‡Ì ̇ ÂÌÁËχ ·ÂÚ‡-„‡Î‡ÍÚÓÁˉ‡Á‡. èË ‚Ò˘ÍË ‰Âˆ‡ ‰Ó͇Á‚‡ÌÂÚÓ Ì‡ ÂÌÁËÏÌËfl ‰ÂÙˈËÚ, ‡ Ò ÚÓ‚‡ Ë ÔÓÚ‚˙ʉ‡‚‡ÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡ ·ÂÁ ËÁ‚˙¯ÂÌÓ ÔÂÁ ÔÂËÓ‰‡ Ï‡È 2005 „. – ˛ÌË 2007 „. êÖáìãíÄíà é·Ó·˘ÂÌÓ ÍÎËÌ˘ÌËÚ ı‡‡ÍÚÂËÒÚËÍË Ë ıÓ‰‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÂÁÛÎÚ‡ÚËÚ ÓÚ Ôӂ‰ÂÌËÚ ËÁÒΉ‚‡ÌËfl ÔË ÓÚ‰ÂÎÌËÚ ԇˆËÂÌÚË Ò‡ Ô‰ÒÚ‡‚ÂÌË ‚ 퇷Îˈ‡ 1. àβÒÚ‡ˆËË Ì‡ ‰ÂÏÓÌÒÚ‡ÚË‚ÌË åêí Ë ÖÖÉ Ì‡ıÓ‰ÍË ÔË ÌflÍÓË ÓÚ Ô‡ˆËÂÌÚËÚ ҇ ÔÓ͇Á‡ÌË ‚˙‚ îË„ÛË 1‡ ‰Ó 3b. (ëΉ‚‡ 퇷Îˈ‡ 1; ÒΉ ÌÂfl îË„ÛË 1‡ ‰Ó 1Ò). éÅëöÜÑÄçÖ Ñˇ„ÌÓÁ‡Ú‡ NCL Ò ÔÓÒÚ‡‚fl ‚˙Á ÓÒÌÓ‚‡ ̇ ı‡‡ÍÚÂ̇ڇ ÍÓÏ·Ë̇ˆËfl ÓÚ ÍÎËÌ˘ÌË ı‡‡ÍÚÂËÒÚËÍË Ë Ô‡ÚÓÎӄ˘ÌË ÓÚÍÎÓÌÂÌËfl ‚ ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌËÚÂ, Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚÂ, Ô‡ÚÓıËÒÚÓÎӄ˘ÌË, ‡ ̇ÔÓÒΉ˙Í Ë ÏÂÚ‡·ÓÎËÚÌË Ë/ËÎË „ÂÌÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl. ÖÖÉ ‰ÂÏÓÌÒÚË‡ ‚Ë̇„Ë ‡·ÌÓÏ̇ ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ, ͇ÍÚÓ Ë Ó„ÌˢÌË Ë „ÂÌÂ‡ÎËÁË‡ÌË Ô‡ÓÍÒËÁχÎÌË ÔÓfl‚Ë. ëÔˆËÙ˘ÌË Á‡ INCL Ò‡ ÎËÔÒ‡Ú‡ ̇ ÔÓÚËÒ͇Ì ̇ ÓÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÔË ÓÚ‚‡flÌ ̇ Ó˜ËÚÂ Ë ËÁ˜ÂÁ‚‡ÌÂÚÓ Ì‡ Ò˙ÌÌËÚ ‚ÂÚÂ̇ ÓÍÓÎÓ 1-2 „. ‚˙Á‡ÒÚ (1,4,7,8,9,14). ëΉ‚‡ ÔÓ„ÂÒË‚ÌÓ ÒÌËʇ‚‡Ì ̇ ÖÖÉ ‡ÏÔÎËÚÛ‰‡Ú‡ ‰Ó Ô˙Î̇ ÎËÔÒ‡ ̇ ‡ÍÚË‚ÌÓÒÚ Í˙Ï 3 „. ‚˙Á‡ÒÚ (1,8,11,14). (îË„ÛË 2‡ Ë 2b). èË LINCL ÒÔˆËÙ˘̇ ̇ıӉ͇ Ò‡ ‚ËÒÓÍÓ‡ÏÔÎËÚÛ‰ÌË ÔÓÎËÙ‡ÁÌË ÓÒÚËfl ‚ Á‡‰ÌËÚ ÓÚ‚Âʉ‡ÌËfl, Ô‰ËÁ‚Ë͇ÌË ÓÚ ÙÓÚÓÒÚËÏÛ·ˆËfl Ò ÌËÒÍË ˜ÂÒÚÓÚË Ë ˜ÂÒÚÓ Ò˙ÔÓ‚Ó‰ÂÌË ÓÚ ÏËÓÍÎÓÌ˘ÌË ÔÓÚÂÔ‚‡ÌËfl, ÍÓËÚÓ Ò ̇·Î˛‰‡‚‡Ú ÓÍÓÎÓ 3 „. ‚˙Á‡ÒÚ Ë Ô‰ÒÚ‡‚Îfl‚‡Ú „Ë„‡ÌÚÒÍË áÖè (1,8,9,11). áÖè Ë ÂÚËÌÓ„‡Ï‡Ú‡ (êÖÉ) ‚Ë̇„Ë Ò‡ ‡·ÌÓÏÌË, Ú˙È Í‡ÚÓ ÔË ‚Ò˘ÍË NCL  ̇Îˈ ÂÚË̇Î̇ ‰Â„ÂÌÂ‡ˆËfl Ò ÔÓÒÚÂÔÂÌÌÓ ÔÓÚËÒ͇Ì ‰Ó Ô˙ÎÌÓ ËÁ˜ÂÁ‚‡Ì ̇ ÌÓχÎÌËÚ ÔÓÚÂ̈ˇÎË, ÍÓÂÚÓ Â Ì‡È-‡ÌÌÓ ÔË INCL (3-4 „.). èË LINCL ÒÔˆËÙ˘ÌË Ò‡ „Ë„‡ÌÚÒÍËÚ áÖè, Ô‰ËÁ‚Ë͇ÌË Ò‡ÏÓ ÓÚ Ù·¯-ÒÚËÏÛ·ˆËfl, ÛÒÔÓ‰ÌÓ Ì‡ ËÁfl‚‡Ú‡ ̇ ÔÓÒÚÂËÓÌËÚ ÓÒÚËfl ‚ ÖÖÉ (1,7,8,9,11,14). åêí ‰ÂÏÓÌÒÚË‡ ‡·ÌÓÏÌÓÒÚË ÔË INCL ‚˙‚ ‚˙Á‡ÒÚÚ‡ ÏÂÊ‰Û 7 Ë 11 ÏÂÒˆ‡, ÍÓ„‡ÚÓ Ò ÔÓfl‚fl‚‡ ËÁ‡ÁÂÌ í2-ıËÔÓËÌÚÂÌÁËÚÂÚ Ì‡ ڇ·ÏÛÒËÚ ÒÔflÏÓ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË Ë ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó, ‡ ÛÒÔÓ‰ÌÓ Ò ۂÂ΢‡‚‡ ËÌÚÂÌÁËÚÂÚ˙Ú Ì‡ ÔÂË‚ÂÌÚËÍÛÎÌÓÚÓ ·flÎÓ ‚¢ÂÒÚ‚Ó (2,4,7,8-10,12). ä˙Ï 13 ÏÂÒ. ‚˙Á‡ÒÚ Ò ËÁfl‚fl‚‡ „ÂÌÂ‡ÎË-

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ÁË‡Ì‡Ú‡ ˆÂ·‡Î̇ Ë ˆÂ·Â·̇ ‡ÚÓÙËfl. ä·Ò˘ÂÒ͇ڇ LINCL ÏÓÊ ‰‡ Ò ‚ËÁÛ‡ÎËÁË‡ Ò ÎÂ͇ ˆÂ·‡Î̇ ‡ÚÓÙËfl ‚ ‡ÌÌËÚ ÒÚ‡‰ËË, ÍÓflÚÓ ÒΉ ÚÓ‚‡ ÔÓ„ÂÒË‡ ÏÌÓ„Ó ·˙ÁÓ, ÓÒÓ·ÂÌÓ ËÌÙ‡ÚÂÌÚÓˇÎÌÓ (2,7,8-10). åêí ‰ÂÏÓÌÒÚË‡ ËÁ‡ÁÂÌ í2-ıËÔÂËÌÚÂÌÁËÚÂÚ Ì‡ ·flÎÓÚÓ ‚¢ÂÒÚ‚Ó, Ô˙‚Ó̇˜‡ÎÌÓ ÔÂË‚ÂÌÚËÍÛÎÌÓ, ‡ ÔÓ-Í˙ÒÌÓ – „ÂÌÂ‡ÎËÁË‡ÌÓ (2,7,8-10,14). ç‡È-‚‡ÊÌË Ò‡ Á‡ ‰Ë‡„ÌÓÒÚˈË‡ÌÂÚÓ Ì‡ NCL Ò‡ ÑçäËÁÒΉ‚‡ÌËflÚ‡, ÓÔ‰ÂÎflÌÂÚÓ Ì‡ ÂÌÁËÏÌËfl ‰ÂÙÂÍÚ Ë ·ËÓıËÏ˘ÂÌ ‡Ì‡ÎËÁ ̇ ̇ÚÛÔ‡ÌËfl ‚ ‡Á΢ÌË Ú˙͇ÌË Ï‡ÚÂˇÎ. í ÔÓÚ‚˙ʉ‡‚‡Ú ÍÎËÌ˘̇ڇ, Ì‚Ó‡‰ËÓÎӄ˘̇ Ë Ì‚ÓÙËÁËÓÎӄ˘̇ ‰Ë‡„ÌÓÁ‡. êÛÚËÌÌËÚ ··Ó‡ÚÓÌË ËÁÒΉ‚‡ÌËfl Ò‡ ·ÂÁ ÓÒÓ·Â̇ ÒÚÓÈÌÓÒÚ ÔË Ôӂ˜ÂÚÓ NCL, Ú˙È Í‡ÚÓ Ò‡ÏÓ ÔË JNCL Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‚‡ÍÛÓÎËÁË‡ÌË ÎËÏÙÓˆËÚË (7,8). Ç ‡ÌÌËÚ ÒÚ‡‰ËË Ì‡ INCL ÍÎËÌ˘̇ڇ ‰Ë‡„ÌÓÁ‡ Ò ·‡ÁË‡ ̇ åêí ̇ıӉ͇ڇ, ÍÓflÚÓ ÔË Ò˙ÏÌËÚÂÎÌË ÒÎÛ˜‡Ë ÏÓÊ ‰‡ ·˙‰Â ÔÓ‚ÂÂ̇ Ë ˜ÂÁ ËÁ‚˙¯‚‡Ì ̇ ·ËÓÔÒËfl, ‡ Í˙Ï 2 „. ‚˙Á‡ÒÚ – ‰ÓÔ˙ÎÌÂ̇ Ë ÓÚ ÂÁÛÎÚ‡ÚËÚ ̇ Ì‚ÓÙËÁËÓÎӄ˘ÌËÚÂ Ë ÓÙÚ‡ÎÏÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl (8). ä‡Ú„Ó˘ÌÓ ÔÓÚ‚˙ʉÂÌË ̇ ‰Ë‡„ÌÓÁ‡Ú‡  ÛÒÚ‡ÌÓ‚fl‚‡ÌÂÚÓ Ì‡ GRODs ‚ ÍÎÂÚÍË ÓÚ ÌÂ‚̇ ËÎË ‰Û„‡ Ú˙Í‡Ì (ÍÓʇ), ͇ÍÚÓ Ë Ì‡ „ÂÌÌËfl ‰ÂÙÂÍÚ, Á‡Òfl„‡˘ CLN1 „Â̇, ‡ÁÔÓÎÓÊÂÌ ‚˙ıÛ ıÓÏÓÁÓχ 1p32 Ë ÍÓ‰Ë‡˘ PPT1 (9,14). èË Í·Ò˘ÂÒ͇ڇ LINCL ÍÎËÌ˘̇ڇ ‰Ë‡„ÌÓÁ‡ Ò ÓÒÌÓ‚‡‚‡ ̇ ı‡‡ÍÚÂÌËÚ Ì‚ÓÙËÁËÓÎӄ˘ÌË Ì‡ıÓ‰ÍË Ë Ò ÔÓÚ‚˙ʉ‡‚‡ ÓÚ ‰ÂÏÓÌÒÚË‡ÌÂÚÓ Ì‡ ı‡‡ÍÚÂÌËÚ ÍÎÂÚ˙˜ÌË ‚Íβ˜‚‡ÌËfl (CVBs Ò/·ÂÁ FPPs) ÔË ÔÓ‚Âʉ‡Ì ̇ ÍÓÊ̇ ËÎË ÂÍÚ‡Î̇ ÏÛÍÓÁ̇ ·ËÓÔÒËfl, ËÎË ÓÚ ‰Ó͇Á‚‡ÌÂÚÓ Ì‡ ÂÌÁËÏÌËfl Ë/ËÎË „ÂÌÂÚ˘ÂÌ ‰ÂÙÂÍÚ (5,7,8,9,14). ÉÂÌ˙Ú, ÓÚ„Ó‚ÓÂÌ (CLN2) Ò ̇ÏË‡ ‚˙ıÛ ıÓÏÓÁÓχ 11p15 Ë ÍÓ‰Ë‡ íêê1. è‰ÒÚ‡‚ÂÌËÚ ÓÚ Ì‡Ò ÒÎÛ˜‡Ë Ò‡ Ò ÚËÔ˘ÌÓ ÍÎËÌ˘ÌÓ ÔÓÚ˘‡Ì Á‡ NCL (‚ËÊ í‡·Îˈ‡). àÁfl‚‡Ú‡ ̇ ÔÓ„ÂÒË‡˘Ë Í‚‡‰ËÔ‡ÂÚ˘ÂÌ Ë ˆÂ·Â·ÂÌ ÒË̉ÓÏË ‚ ‡Á΢ÂÌ ‰ ̇ ËÁfl‚‡ ÔË NCL1 Ë NCL2, ͇ÍÚÓ Ë ÔË Ì‡¯ËÚ ԇˆËÂÌÚË, ËÌÚÂÎÂÍÚÛ‡ÎÂÌ „ÂÒ, ‡Ï·ÎËÓÔËfl, ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ Ë ‡ÍˆËÓÌÌË ÏËÓÍÎÓÌËË ÔË ËÁıÓ‰ÌÓ Á‰‡‚Ó ‰ÂÚ ̇ÒÓ˜‚‡Ú Í˙Ï Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ. ᇠ҂Ó‚ÂÏÂÌÌÓÚÓ ‰Ë‡„ÌÓÒÚˈË‡Ì  ÌÂÓ·ıÓ‰ËÏÓ ËÁÍβ˜‚‡Ì ̇ ΂ÍÓ‰ËÒÚÓÙËfl ˜ÂÁ åêí ÔË ÎËÔÒ‡ ̇ ı‡‡ÍÚÂÌËÚ ӷ¯ËÌË ÔÓ‰ÍÓÓ‚Ë ‰ÂÏËÂÎËÌËÁ‡ˆËË. ᇉ˙ÎÊËÚÂÎ̇ڇ ̇ıӉ͇ Á‡ NCL  ÔÓ„ÂÒË‡˘‡Ú‡ „ÎÓ·‡Î̇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò ËÁ‡ÁÂÌÓ Û˜‡ÒÚË ̇ χÎÍËfl ÏÓÁ˙Í ÔË Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ËÁÒΉ‚‡ÌËfl. 燘‡Î̇ڇ χÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl  ‚˙ÁÏÓÊ̇ ÔË LINCL, ͇ÍÚÓ ÔË Ì‡¯ËÚ ÒÎÛ˜‡Ë 2 Ë 3. ÉÎÓ·‡Î̇ڇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl ‚Ó‰Ë ‰Ó ıˉÓˆÂÙ‡ÎËfl ÂÍÒ ‚‡ÍÛÓ, ÔÓ„¯ÌÓ ËÌÚÂÔÂÚË‡Ì‡ ÔË Ì‡¯Ëfl Ô‡ˆËÂÌÚ Ò INCL ͇ÚÓ ◊ÌÂÓÍÎÛÁ˂̇ ıˉÓˆÂÙ‡ÎËfl Ò ‚ÚÓ˘̇ ‡Ï‡‚ÓÁ‡” (!?). Ç ÚÓÁË ÒÎÛ˜‡È ÔÓÒÚ‡‚Â̇ڇ ‚ÂÌÚËÍÛÎÓ-ÔÂËÚÓ̇Î̇ ‡Ì‡ÒÚÓÏÓÁ‡  ·ÂÁÒÏËÒÎÂ̇ ËÌÚÂ‚Â̈Ëfl, ÍÓflÚÓ Â ·ÂÁ ÂÙÂÍÚ ‚˙ıÛ Ò˙ÒÚÓflÌËÂÚÓ Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘‡Ú‡ ̇ıӉ͇, ÔÓÒΉÂ̇ ˜ÂÁ äí Ë åêí. (ëΉ‚‡Ú îË„ÛË 3‡ ‰Ó 3c). äÎËÌ˘̇ڇ ‰Ë‡„ÌÓÁ‡ ̇ NCL Ò ÓÒÌÓ‚‡‚‡ ̇ ÔÓÒÓ˜ÂÌÓÚÓ Ò˙˜ÂÚ‡ÌË ÓÚ ‡Á΢ÌË ÒËÏÔÚÓÏË Ë ÒË̉ÓÏË Ì‡ Û‚‰‡ ̇ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ, ̇ÒÚ˙Ô‚‡˘Ë ‚ ÓÔ‰ÂÎÂ̇ ‚˙Á‡ÒÚ Ë ÔÓ͇Á‚‡˘Ë ÔÓÒÚÓflÌ̇, ˜ÂÒÚÓ ·˙Á‡ ÔÓ„ÂÒËfl. í˙ÒÂÌÂÚÓ Ì‡ ÚËÔ˘ÌËÚ ÖÖÉ, áÖè Ë êÖÉ Úfl·‚‡ ‰‡ ·˙‰Â ‚Íβ˜ÂÌÓ ‚ ‰Ë‡„ÌÓÒÚ˘ÌËfl ÔÎ‡Ì ÔË ‚ÒflÍÓ ‰ÂÚ Ò˙Ò Ò˙ÏÌÂÌË Á‡ NCL. éÔËÒ‡ÌËÚ ÓÚ Ì‡Ò ÒÎÛ˜‡Ë Ò NCL Ò‡ Ò ÚËÔ˘̇ڇ ÍÎËÌË͇ ̇ ÔÓ„ÂÒ˂̇ ÏËÓÍÎÓÌ˘̇ ÂÔËÎÂÔÒËfl, ÍÓflÚÓ ‚Ë̇„Ë Â ÒËÏÔÚÓχÚ˘̇. èË ÒÎÛ˜‡È 2 Ë 3  ̇‰ˆÂÌÂÌ ÚÂ‡Ô‚Ú˘ÌÓ-ÂÁËÒÚÂÌÚÌËflÚ ÂÔËÎÂÔÚ˘ÂÌ ÒË̉ÓÏ, ‡ ÔÓ„ÂÒË‡˘ËflÚ ÔÓÎËÏÓÙÂÌ Ì‚ÓÎӄ˘ÂÌ ‰ÂÙˈËÚ Ë ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò‡ ÔÓ„¯ÌÓ ËÌÚÂÔÂÚË‡ÌË Í‡ÚÓ ÒΉÒÚ‚Ë ̇ “ÚÂ‡Ô‚Ú˘ÌÓ-ÂÁËÒÚÂÌÚ̇ ÍËÔÚÓ„ÂÌ̇ ÂÔËÎÂÔÒËfl”. ë˜ËÚ‡ÏÂ, ˜Â Ô‰Ò-


Ç ÌÓχ

Ç ÌÓχ

ᇷ‡‚ÂÌÓ èåê

å, 7 „.

å, 6 „.

Ü, 4 „.

Ü, 3 „.

ÅÂÏÂÌÌÓÒÚ, ‡Ê‰‡ÌÂ Ë ‡ÌÌÓ èåê Ç ÌÓχ

ëÎÛ˜‡È

3 „.

17 ÏÂÒ.

燘‡ÎÓ

èÓ-„ÓÎflÏ 3 „. ·‡Ú - exitus ̇ 7 „. Ò Ú‚˙‰Â ÒıÓ‰ÌÓ, ÌÓ ÌÂËÁflÒÌÂÌÓ Á‡·ÓÎfl‚‡ÌÂ

Ң‡ –ÌÓ˘ÌË 3 „. ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË (3-6 „.)

çÂflÒ̇ (‡‰ÓÔÚË‡Ì)

ãËÔÒ‚‡

î‡ÏËÎÌÓÒÚ ä‚‡‰ËÔ‡ÂÁ‡ Ñ>ã (17 ÏÂÒ.) åËÓÍÎÓÌËË (19 ÏÂÒ.) àÌ‚ÂÒËfl ̇ Ò˙Ìfl (20 ÏÂÒ.) ÄÚ‡ÍÒËfl, ÏÛÚËÁ˙Ï (20 ÏÂÒ.) ëÎÂÔÓÚ‡ Ë „ÎÛıÓÚ‡ (22 ÏÂÒ.) ÖÔËÎÂÔÒËfl – Éíäè (3 „.); ‡·Ò‡ÌÒË, åÄ, ÚÓÌ˘ÌË, Éíäè-ÒÚ‡ÚÛÒË, ‚„ÂÚ‡ÚË‚ÌË ÍËÁË (ÒΉ 4 „.) ÄÚ‡ÍÒËfl, ÚÂÏÓ, ÏËÓÍÎÓÌËË – ÔË êÇ (3 „.4 Ï.) èÓ„ÂÒ˂̇ ‰ÂÏÂ̈Ëfl (4 „.) éÔÚËÍÛÒ-‡ÚÓÙËfl (4 „.), ‡Ï‡‚ÓÁ‡ (5 „.) 䂇‰ËÔ‡ÂÁ‡, „ÎÛıÓÚ‡ (5 „.) ïÓÂÓ-‡ÚÂÚÓÁ‡ (6 „.) ÖÔËÎÂÔÒËfl – Éíäè (3 „.) ÄÚ‡ÍÒËfl – ÔË êÇ (3 „. 3 Ï.), ÚÂÏÓ (3 1/2 „.) ÄÚÓÌ˘ÌË; åËÓÍÎÓÌËË (4 „.) 䂇‰ËÔË‡ÏˉÂÌ (4 1/2 „.) ÑÂÏÂ̈Ëfl (4 1/2 „.) Äχ‚ÓÁ‡ (4 1/2 „.) ÑËÒÍÂÚ̇ ‡Ú‡ÍÒËfl (3 „.) çÂÔÓÒÚÓflÌÌË ÏËÓÍÎÓÌËË (3 1/2 „.) – ÔË ËÌÙÂ͈ËË Ñ‚‡ Éíäè (3 „.) Ñ‚‡ Éíäè (3 „.) åÄ Ë ‡ÚÓÌ˘ÌË (3 1/2 „.)

ëËÏÔÚÓÏ Ë ‚˙Á‡ÒÚ Ì‡ ËÁfl‚‡

åêí (4 „.) – ·.Ó. äí (4 1/2 „.) – ˆÂ·Â·̇ ‡ÚÓÙËfl

äí – ˆÂ·Â·̇ ‡ÚÓÙËfl (3 „.) åêí – „ÂÌÂ‡ÎËÁË‡Ì‡, ÓÒÓ·ÂÌÓ ˆÂ·Â·̇ ‡ÚÓÙËfl (4 „.)

äí ̇ 10 ÏÂÒ.* Ë Ì‡ 3 „.- ·.Ó. äí ̇ 4 „. - ˆÂ·ÂÎÓ -ÔÓÌÚËÌ̇ ‡ÚÓÙËfl åêí (5,6,7 „.) „ÂÌÂ‡ÎËÁË‡Ì‡, ÔÓ„ÂÒ˂̇ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl

ÉÂÌÂ‡ÎËÁË‡Ì‡ ‡ÚÓÙËfl (20 ÏÂÒ.), åêí í2-ıËÔÓËÌÚÂÌÁÌË Ú‡Î‡ÏÛÒË

äí Ë åêí

ãËÔÒ‡ (4-5 „.)

áÖè Ë/ËÎË ëëÖè ãËÔÒ‡ (2 „.)

ᇷ‡‚Â̇, „ÂÌÂ‡ÎË -ÁË‡ÌË SW Ë PSW, îèé

Å.Ó. (4 „.)

3 ïˆ SW, ãËÔÒ‡ îèé-3 „. ᇷ‡‚Â̇, (4-5 „.) „ÂÌÂ‡ÎË-ÁË‡ÌË SW Ë PSW -4 „.

ᇷ‡‚Â̇, „ÂÌÂ‡ÎË-ÁË‡ÌË SW Ë PSW, îèé ** (ÎËÔÒ‚‡˘ Í˙Ï 4 „.)

ÅÂÁ Ò˙ÌÌË Ô‡ÚÂÌË (24 ÏÂÒ.) çËÒÍÓ‚ÓÎÚ‡Ê̇ (4 „.)

ÖÖÉ

퇷Îˈ‡ 1: äÎËÌ˘ÌË ı‡‡ÍÚÂËÒÚËÍË Ë ÂÁÛÎÚ‡ÚË ÓÚ ËÁÒΉ‚‡ÌËflÚ‡ ÔË Ô‡ˆËÂÌÚËÚÂ

VêÄ - ˜‡ÒÚ˘ÂÌ

êÇ-100%; VêÄ – ˜‡ÒÚ˘ÂÌ íêå, CZP, ESM - NE LTG, äë– ‚ÂÏÂÌÂÌ

CZP, NZP, VêÄ, LTG, LEV – NE êÇ – ˜‡ÒÚ˘ÂÌ; CBZ – ‡„‡‚‡ˆËfl VêÄ – ˜‡ÒÚ˘ÂÌ, LTG, CZP – NE

ã˜ÂÌËÂ

Ñˇ„ÌÓÁ‡

ä·Ò˘ÂÒ͇ Í˙Ò̇ ‰ÂÚÒ͇ NCL (NCL 2) ä·Ò˘ÂÒ͇ Í˙Ò̇ ‰ÂÚÒ͇ NCL (NCL 2).

TPP1 – 0.02 ÌÏÓÎ/ÔÂÚÌÓ

TPP1 – 0.00 Ë 0.01 ÌÏÓÎ/ ÔÂÚÌÓ (‰‚ÛÍ‡ÚÌÓ ËÁÒΉ‚‡Ì*)

ä·Ò˘ÂÒ͇ ‡Ì̇ ‰ÂÚÒ͇ NCL (NCL 1) TPP1 - ÎËÔÒ‚‡ ä·Ò˘ÂÒ͇ /0.00 ÌÏÓÎ/ÔÂÚÌÓ/ Í˙Ò̇ ‰ÂÚÒ͇ NCL (NCL 2).

êêí1 - 0.04 ÌÏÓÎ/ÔÂÚÌÓ

ÖÌÁËÏÌÓ ËÁÒΉ‚‡ÌÂ

‰ÂÍÂÏ‚Ë, 2007

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology 227


‰ÂÍÂÏ‚Ë, 2007

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

228

Ú‡‚ÂÌËÚ ÓÚ Ì‡Ò ÒÎÛ˜‡Ë ÔÓÚ‚˙ʉ‡‚‡Ú ‚ ÏÌÓ„Ó ÓÚÌÓ¯ÂÌËfl ‚Ò Ӣ Ì‰ÓÒÚ‡Ú˙˜ÌÓÚÓ ÂÚËÓÎӄ˘ÌÓ ËÁflÒÌfl‚‡Ì ̇ ‰ˈ‡ Ú.̇. ”ÚÂ‡Ô‚Ú˘ÌÓ-ÂÁËÒÚÂÌÚÌË”, ‚ÍÎ. “ÔÓ„ÂÒË‚ÌË ÏËÓÍÎÓÌ˘ÌË ÂÔËÎÂÔÒËË”. èÓ‚Âʉ‡ÌÂÚÓ Ì‡ ·ËÓÔÒËfl, ‰ÓË Ò‡ÏÓ ÍÓÊ̇, Ò ˆÂÎ ÔÓÎÛ˜‡‚‡Ì ̇ χÚÂˇΠÁ‡ ÂÎÂÍÚÓÌÌÓ-ÏËÍÓÒÍÓÔÒÍÓ ËÁÒΉ‚‡ÌÂ, Ò ÔËÂχ ÚÛ‰ÌÓ ÓÚ Ó‰ËÚÂÎËÚÂ Ë Ì ‚Ë̇„Ë ÏÓÊ ‰‡ ÔÓÚ‚˙‰Ë ‰Ë‡„ÌÓÁ‡Ú‡ NCL. Ä ÚӘ̇ڇ ‰Ë‡„ÌÓÁ‡  ‡·ÒÓβÚÌÓ ÌÂÓ·ıÓ‰Ëχ, Á‡˘ÓÚÓ ÓÔ‰ÂÎfl ÔÓ-̇ڇÚ˙¯Ì‡Ú‡ ÔÓ„ÌÓÁ‡ ̇ Ô‡ˆËÂÌÚ‡ Ë Ò˙‰·‡Ú‡ ̇ ÒÂÏÂÈÒÚ‚ÓÚÓ ÔË Ê·ÌË Á‡ ‰Âˆ‡, Á‡ ÍÓÂÚÓ Â ÌÂÓ·ıÓ‰ËÏÓ ÔÓ‚Âʉ‡ÌÂÚÓ Ì‡ „ÂÌÂÚ˘ÌÓ Ë Â‚ÂÌÚÛ‡ÎÌÓ ÔÂ̇ڇÎÌÓ ËÁÒΉ‚‡Ì (3,13). èÓÚ‚˙ʉÂÌË ̇ ÚÓ‚‡  ̇¯ËflÚ ÒÎÛ˜‡È 4, Í˙‰ÂÚÓ ËÁfl‚‡Ú‡ ̇ LINCL ̇È-‚ÂÓflÚÌÓ Â Ù‡ÏËÎ̇: ̇Îˈ  ÒÏ˙ÚÂÌ ËÁıÓ‰ ̇ ÔÓ-„ÓÎflÏÓ ‰ÂÚ ‚ ÒÂÏÂÈÒÚ‚ÓÚÓ ÓÚ Ë‰ÂÌÚ˘ÌÓ ÔÓ ı‡‡ÍÚÂ Ë ıÓ‰ Á‡·ÓÎfl‚‡ÌÂ, ÌÓ Á‡ Ò˙ʇÎÂÌË ‰Ë‡„ÌÓÁ‡Ú‡ Ì  ·Ë· ÛÚÓ˜ÌÂ̇ Ë ÒΉ‚‡˘ÓÚÓ ‰ÂÚ ÔÓ̇ÒÚÓfl˘ÂÏ Ï‡ÌËÙÂÒÚË‡ ·ÓÎÂÒÚÚ‡, χ͇ Ë Ò ÔÓ-·‡‚ÌÓ ÔÓ„ÂÒË‡˘ ıÓ‰.

îË„Û‡ 1‡. äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ ëÎÛ˜‡È 1 (NCL1) ̇ 7 ÏÂÒ. ‚˙Á‡ÒÚ: åÓÁ˙˜Ì‡ Ë Ï‡ÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò Ì‡Û¯Â̇ ‰ËÙÂÂ̈ˇˆËfl ÏÂÊ‰Û ÒË‚Ó Ë ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó.

áÄäãûóÖçàÖ ä‡ÍÚÓ Ò ‚Ëʉ‡ ÓÚ Ô‰ÒÚ‡‚ÂÌËÚ ‰‡ÌÌË, Á‡ ÔÂËÓ‰ Ò‡ÏÓ ÓÚ ‰‚ „Ó‰ËÌË ‚ ̇¯‡Ú‡ ÍÎËÌË͇ ‰Ë‡„ÌÓÁ‡Ú‡ NCL ·Â ÔÓÚ‚˙‰Â̇ ÔË 4 ‰Âˆ‡. íÓ‚‡ Òڇ̇ ‚˙ÁÏÓÊÌÓ ˜ÂÁ ‚ÌËχÚÂÎÌÓÚÓ Ó·Ò˙ʉ‡Ì ̇ Ò˙˜ÂÚ‡ÌËÂÚÓ ÓÚ Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË Ë ÒË̉ÓÏË, Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ̇ıÓ‰ÍË Ë ÂÁÛÎÚ‡ÚË ÓÚ Ì‚ÓÙËÁËÓÎӄ˘ÌËÚ ËÁÒΉ‚‡ÌËfl, ÔË ÓÚ˜Ëڇ̠̇ Úflı̇ڇ ‰Ë̇ÏË͇ ‚˙‚ ‚ÂÏÂÚÓ, ͇ÚÓ ÚÓ‚‡ ̇ÒÓ˜Ë Í˙Ï ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ÓÚ ÔÓ‚Âʉ‡Ì ̇ ÏÂÚ‡·ÓÎËÚÌÓ ËÁÒΉ‚‡Ì ‚ ̇ÒÓ͇ ̇ „Ó̇ڇ ‰Ë‡„ÌÓÁ‡. ÑÓ͇Á‚‡ÌÂÚÓ Ì‡ ÂÌÁËÏÌËfl ‰ÂÙˈËÚ ÔË ‰‚ÂÚ ̇È-˜ÂÒÚË NCL ÙÓÏË ‚ ‰ÂÚÒÚ‚ÓÚÓ Â ÛÌË͇ÎÌÓ Ó·ÎÂ͘ÂÌË Á‡ ÎÂ͇fl Ë ÒÂÏÂÈÒÚ‚ÓÚÓ Ì‡ Ô‡ˆËÂÌÚ‡. èÓÚ‚˙ʉ‡‚‡ÌÂÚÓ ËÎË ÓÚı‚˙ÎflÌÂÚÓ Ì‡ Ò˙ÓÚ‚ÂÚÌËfl ÏÂÚ‡·ÓÎËÚÂÌ ‰ÂÙÂÍÚ ‰‡‚‡ ̇ÒÓ͇ Á‡ ÔÓ-̇ڇÚ˙¯ÌÓ ËÁÒΉ‚‡Ì ̇ Ó‰ËÚÂÎËÚÂ Ë ÓÔ‰ÂÎflÌ ‚ÂÓflÚÌÓÒÚÚ‡ Á‡ Ô‰‡‚‡Ì ̇ ·ÓÎÂÒÚÚ‡ ‚ ÒΉ‚‡˘Ë ÔÓÍÓÎÂÌËfl. åÂÚÓ‰˙Ú Ì‡ ÓÔ‰ÂÎflÌ ̇ PPT1- Ë TPP1-‡ÍÚË‚ÌÓÒÚÚ‡ ÔÓ Ì‡˜ËÌ, ËÁ‚˙¯‚‡Ì ÛÚËÌÌÓ ÔË ÌÓ‚ÓÓ‰ÂÌË Á‡ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ ‰Û„Ë ÏÂÚ‡·ÓÎËÚÌË ‰ÂÙÂÍÚË, ÔÓÁ‚ÓÎfl‚‡ ·˙ÁÓ Ë ÚÓ˜ÌÓ ‡Á„‡Ì˘‡‚‡Ì ̇ Á‰‡‚Ë, ̇ ÒÚ‡‰‡˘Ë ÓÚ CLN1 Ë CLN2, Ë Ì‡ ıÂÚÂÓÁË„ÓÚÌË ÌÓÒËÚÂÎË (5). è‰ËÏÒÚ‚Ó Ì‡ ÏÂÚÓ‰‡ Â Ë Á̇˜ËÚÂÎÌÓ ÔÓ-ÌËÒ͇ڇ ÏÛ ˆÂ̇ ‚ Ò‡‚ÌÂÌËÂ Ò ËÁÒΉ‚‡ÌËfl ̇

îË„Û‡ 1b. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ Ò˙˘ÓÚÓ ‰ÂÚ ÓÍÓÎÓ 1 ÏÂÒ. ÔÓ-Í˙ÒÌÓ: é·˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl; Ô‡‚flÚ ‚Ô˜‡ÚÎÂÌË ıËÔÓËÌÚÂÌÁËÚÂÚ˙Ú Ì‡ ڇ·ÏÛÒËÚ ÒÔflÏÓ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË Ë ·flÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó, ͇ÍÚÓ Ë ıËÔÂËÌÚÂÌÁÌËÚ ÁÓÌË ÔÂË‚ÂÌÚËÍÛÎÌÓ.


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îË„Û‡ 1c. äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ Ò˙˘Ëfl Ô‡ˆËÂÌÚ 1 ÏÂÒ. ÔÓ-Í˙ÒÌÓ: èÓ„ÂÒËfl ̇ ÏÓÁ˙˜Ì‡Ú‡ ‡ÚÓÙËfl, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÔÓÒÚ‡‚Â̇ڇ ‚ÂÌÚËÍÛÎÓÔÂËÚÓ̇Î̇ ‡Ì‡ÒÚÓÏÓÁ‡.

îË„Û‡ 2‡. ÖÖÉ Ì‡ ëÎÛ˜‡È 1 (NCL1) ̇ 2 „. ‚˙Á‡ÒÚ: ÑÂÁÓ„‡ÌËÁË‡Ì‡ Ë Á‡·‡‚Â̇ ‡ÍÚË‚ÌÓÒÚ Ò ˜ÂÚËËÍ‡ÚÌË „ÂÌÂ‡ÎËÁË‡ÌË ÏËÓÍÎÓÌËË, ÔË ÍÓËÚÓ ‚ ÖÖÉ Ò ̇·Î˛‰‡‚‡Ú Ô‰ËÏÌÓ ‡ÚÂÙ‡ÍÚË Ë Ò‡ÏÓ ÙÓÌÚÓˆÂÌÚ‡ÎÌË (‰Ó ÚÂÏÔÓ‡ÎÌË) ‚ËÒÓÍË ÓÒÚË Ë ·‡‚ÌË ‚˙ÎÌË.

îË„Û‡ 2b. ÖÖÉ Ì‡ Ò˙˘Ëfl Ô‡ˆËÂÌÚ Ì‡ 4 „. ‚˙Á‡ÒÚ. ÑÂÁÓ„‡ÌËÁË‡Ì‡, ÌËÒÍÓ‚ÓÎÚ‡Ê̇ ‡ÍÚË‚ÌÓÒÚ. äÎËÌ˘ÌÓ: ‰ËÒÚ‡ÎÌË Ë ‰ÍË „ÂÌÂ‡ÎËÁË‡ÌË ÏËÓÍÎÓÌËË, ·ÂÁ ÖÖÉ ÍÓ·ˆËfl. ÂÌÁËÏËÚ ‚ EDTA-Í˙‚ (5), Ú˙È Í‡ÚÓ ÂÌÁËÏ̇ڇ ‡ÍÚË‚ÌÓÒÚ ÓÒÚ‡‚‡ ÒÚ‡·ËÎ̇ ÌflÍÓÎÍÓ ‰ÌË Ë Ï‡ÚÂˇÎ˙Ú ÏÓÊ ·˙ÁÓ ‰‡ ·˙‰Â ËÁÔ‡ÚÂÌ ÔÓ ÔÓ˘‡Ú‡. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5.

6.

îË„Û‡ 3‡. äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ ëÎÛ˜‡È 2 (NCL2) ̇ 3 „. ‚˙Á‡ÒÚ: 燘‡Î̇ χÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl, ÔÓ‰˜Âڇ̇ ‚˙‚ ‚ÂÏËÒ̇ڇ ӷ·ÒÚ.

Binelli, S., Canafoglia, L., Panzica, F., Pozzi, A., Franceschetti, S. Electroencephalographic features in a series of patients with neuronal ceroid lipofuscinoses. Neurol Sci 2000; 21(3 Suppl): S83–S87. D’Incerti, L. MRI in neuronal ceroid lipofuscinosis. Neurol Sci 2000; 21: S71-S73. Gardiner, R.M. The molecular genetic basis of the neuronal ceroid lipofuscinoses. Neurol Sci 2000; 21(3 Suppl): S15-S19. Goebel, H.H., Wisniewski, K.E. Current state of clinical and morphological features in human NCL. Brain Pathol 2004; 14:61–69. Lukacs, Z., Santavuori, P., Keil, A., Steinfeld, R., Kohlschutter, A. Rapid and simple assay for the determination of tripeptidyl peptidase and palmitoyl protein thioesterase activities in dried blood spots. Clin Chem 2000; 49: 509–511. Mole, S., Williams, R., Goebel, H. Correlations between genotype, ultrastructural morphology and clinical phenotype in the neuronal ceroid lipofuscinoses. Neurogenetics 2005; 6: 107–126.


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îË„Û‡ 3b. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ Ò˙˘Ëfl Ô‡ˆËÂÌÚ 2 „. ÔÓ-Í˙ÒÌÓ: é·˘‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò ÔÓfl‚‡ ̇ ıËÔÂËÌÚÂÌÁÌË ÁÓÌË ÔÂË‚ÂÌÚËÍÛÎÌÓ Ë Á‡Î˘‡‚‡Ì ̇ „‡Ìˈ‡Ú‡ ÏÂÊ‰Û ÒË‚Ó Ë ·flÎÓ ÏÓÁ˙˜ÌÓ ‚¢ÂÒÚ‚Ó. 7. 8. 9. 10. 11. 12. 13.

Mole, S. Neuronal ceroid lipofuscinoses (NCL). Eur J Paediatr Neurol 2004; 8: 101–103. Santavuori, P., Lauronen, L., Kirveskari, E., Aberg, L., Sainio, K., Autti, T. Neuronal ceroid lipofuscinosis in childhood. Neurol Sci 2000; 21(3 Suppl): S35-S41. Serratosa, J.M. The progressive myoclonus epilepsies. In: Wyllie E., ed. The treatment of Epilepsy: Principles and Practice. Philadelphia: Lippincott Williams and Wilkins, 2001: 509-524. Vanhanen, S.L., Puranen, J., Autti, T., et al. Neuroradiological findings (MRS, MRI, SPECT) in infantile neuronal ceroid-lipofuscinosis (infantile CLN1) at different stages of the disease. Neuropediatrics 2004; 35: 27–35. Veneselli, E., Biancheri, R., Perrone, M.V., Buoni, S., Fois, A. Neuronal ceroid lipofuscinoses: clinical and EEG findings in a large study of Italian cases. Neurol Sci 2000; 21(3 Suppl): S75–S81. Wisniewski, K., Zhong, N., Philippart, M. Pheno/genotypic correlations of neuronal ceroid lipofuscinoses. Neurology 2001; 57: 576–581. Young, E.P., Worthington, V.C., Jackson, M., Winchester, B.G. Pre- and postnatal diagnosis of patients with CLN1 and CLN2 by assay of palmitoylprotein thioesterase and tripeptidyl-peptidase I activities. Eur J Paediatr Neurol 2001; 5(Suppl A): 193–196.

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îË„Û‡ 3Ò. äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ Ò˙˘Ëfl Ô‡ˆËÂÌÚ Ì‡ 6 „. ‚˙Á‡ÒÚ: íÂÊ͇ ÏÓÁ˙˜Ì‡ Ë Ï‡ÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl, ÔË Ô˙Î̇ ÎËÔÒ‡ ̇ ‰ËÙÂÂ̈ˇˆËfl ÏÂÊ‰Û ÍÓ‡ Ë ÔÓ‰ÍÓËÂ. 14. Zupanc, M.L., Legros, B. Progressive myoclonic epilepsy. Cerebellum 2004; 3: 156-171.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- èÂÚfl ëÚ. ÑËÏÓ‚‡, ‰Ï ÑÂÚÒ͇ Ì‚ÓÎӄ˘̇ ÍÎËÌË͇, ìëÅÄãçè “ë‚. ç‡ÛÏ” ìÎ. “Ñ- ã. êÛÒ‚” ‹1 1113 ëÓÙËfl Ö-mail: pdimova@cablebg.net


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éË„Ë̇ÎÌË ÒÚ‡ÚËË ïêéçàóçÄ óÖêçéÑêéÅçÄ ÖçñÖîÄãéèÄíàü èêà èÄñàÖçíà ë óÖêçéÑêéÅçÄ ñàêéáÄ å. чÒ͇ÎÓ‚1, ç. ñÓÎÓ‚‡2, å. ꇯ‚‡1, ë. ëÚÓÈÌÓ‚2, Ä. ÄÎÂÍ҇̉Ó‚3 1 äÎÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇” 2äÎËÌË͇ ÔÓ „‡ÒÚÓÂÌÚÂÓÎÓ„Ëfl, ìåÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇” 3àÌÒÚËÚÛÚ ÔÓ ·ËÓÙËÁ��͇, Å˙΄‡Ò͇ Ä͇‰ÂÏËfl ̇ ̇ÛÍËÚÂ, ëÓÙËfl SUMMARY CHRONIC HEPATIC ENCEPHALOPATHY IN PATIENTS WITH LIVER CIRRHOSIS – CLINICAL, NEUROPSYCHOLOGICAL AND EEG STUDY M. Daskalov, N. Tzolova, M. Rasheva, S. Stoinov, A. Alexandrov Hepatic encephalopathy is the most frequent and important neurological complication in patients with ‡cute, subacute and chronic hepatocellular insufficiency. The aim of the present study is to perform clinical, neuropsychological and EEG examination of patients with liver cirrhosis in order to diagnose clinically manifested and subclinical forms of chronic hepatic encephalopathy. Clinical contingent consists of 22 patients, 12 males and 10 females, with viral, alcoholic, mixed or cryptogenic aetiology of liver cirrhosis. All patients had specific history, and detailed neurological examination was carried out. Neuropsychological evaluation included MMSE. EEG recordings were carried out using a standard 20-chanal EEG device, “Galileo”. Various CNS signs and syndromes were observed in 13 patients (59%). MMSE scores were abnormal in 13 patients (59%). Most of the patients had borderline MMSE values or mild cognitive deficit. EEG recordings were within normal limits in 9 patients (40,9%). The degree of diffuse EEG changes increases as the liver diseases progresses, when the lower alpha-rhythm waves (7,5-8 Hz) were observed. Focal changes were found in 27,3% of the cases. Focal or generalized paroxysmal activity was registered in 5 patients (22,7%). The severity of EEG changes was related to the stage of liver cirrhosis and psychometric scores. KEY WORDS: Chronic hepatic encephalopathy, EEG, neuropsychological study. êÖáûåÖ óÂÌÓ‰ӷ̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl Â Ì‡È ˜ÂÒÚÓÚÓ Ë Á̇˜ËÏÓ Ì‚ÓÎӄ˘ÌÓ ÛÒÎÓÊÌÂÌË ÔË Ô‡ˆËÂÌÚË Ò ÓÒÚ‡, ÔÓ‰ÓÒÚ‡ Ë ıÓÌ˘̇ ıÂÔ‡ÚÓˆÂÎÛ·̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. ñÂÎÚ‡ ̇ ̇ÒÚÓfl˘ÂÚÓ ËÁÒΉ‚‡Ì  ÔÓ‚Âʉ‡Ì ̇ ÍÎËÌ˘ÌÓ, Ì‚ÓÔÒËıÓÎӄ˘ÌÓ Ë ÖÖÉ ËÁÒΉ‚‡Ì ÔË Ô‡ˆËÂÌÚË Ò ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ Ò Ó„Î‰ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ ÍÎËÌ˘ÌÓ ÔÓfl‚ÂÌË ËÎË ÒÛ·ÍÎËÌ˘ÌË ÙÓÏË Ì‡ ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl. äÎËÌ˘ÌËflÚ ÍÓÌÚËÌ„ÂÌÚ Ò Ò˙ÒÚÓË ÓÚ 22 ·ÓÎÌË /12 Ï˙ÊÂ Ë 10 ÊÂÌË/ Ò ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ Ò ‚ËÛÒ̇, ‡ÎÍÓıÓÎ̇, ÒÏÂÒÂ̇ ËÎË ÍËÔÚÓ„ÂÌ̇ ÂÚËÓÎÓ„Ëfl. èË ‚Ò˘ÍËÚ ҇ ÒÌÂÚË Ì‡ÒÓ˜Â̇ ‡Ì‡ÏÌÂÁ‡ Ë Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ. ç‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì  Ôӂ‰ÂÌÓ Ò ÔÓÏÓ˘Ú‡ ̇ ååSÖ. ÖÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡Ì  ÓÒ˙˘ÂÒÚ‚ÂÌÓ Ò 20-͇̇ÎÂÌ ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡Ù Galileo. ìÒÚ‡ÌÓ‚fl‚‡ Ò ‡ÁÌÓÓ·‡Á̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÔË 13 ·ÓÎÌË (59%). ç‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ÔÓ MMSE ÔÓ͇Á‚‡ ÓÚÍÎÓÌÂÌË ÓÚ ÌÓχÎÌËÚ ÒÚÓÈÌÓÒÚË ÔË 13 ·ÓÎÌË (59%), ͇ÚÓ ÔÓ˜ÂÒÚÓ Ò ͇҇ Á‡ „‡Ì˘ÌË ÒÚÓÈÌÓÒÚË ËÎË ÎÂÍ ÍÓ„ÌËÚË-

‚ÂÌ ‰ÂÙˈËÚ. ÖÖÉ Ò „ËÒÚË‡ ‚ „‡ÌˈËÚ ̇ ÌÓχڇ Á‡ ‚˙Á‡ÒÚÚ‡ ÔË 9 ÓÚ ·ÓÎÌËÚ (40,9%). ëÚÂÔÂÌÚ‡ ̇ ‰ËÙÛÁÌËÚ ÔÓÏÂÌË Ì‡‡ÒÚ‚‡ Ò Ì‡Ô‰‚‡Ì ̇ ˜ÂÌÓ‰Ó·ÌÓÚÓ Á‡·ÓÎfl‚‡ÌÂ, ÍÓ„‡ÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ë ÔÓ-ÌËÒÍË ˜ÂÒÚÓÚË Ì‡ α – ËÚ˙χ (7,5-8 Hz). îÓ͇ÎÌËÚ ÔÓÏÂÌË Ò ̇·Î˛‰‡‚‡Ú Ò‡‚ÌËÚÂÎÌÓ fl‰ÍÓ – ÔË 27,3% ÓÚ ÒÎÛ˜‡ËÚÂ. èË 5 ·ÓÎÌË (22,7%) Ò „ËÒÚË‡ ÙÓ͇Î̇ ËÎË „ÂÌÂ‡ÎËÁË‡Ì‡ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ. íÂÊÂÒÚÚ‡ ̇ ÖÖÉ ÔÓÏÂÌËÚ ÓÚ„Ó‚‡flÚ Ì‡ ÒÚ‡‰Ëfl ̇ ˜ÂÌÓ‰ӷ̇ڇ ˆËÓÁ‡ Ë Ì‡ ÔÒËıÓÏÂÚ˘ÌËfl ÒÍÓ. äãûóéÇà Ñìåà: ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl, ÖÖÉ, Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ. èË Ô‡ˆËÂÌÚË Ò ˜ÂÌÓ‰Ó·ÌÓ Á‡·ÓÎfl‚‡Ì ˜ÂÒÚÓ Ò ̇·Î˛‰‡‚‡Ú Ì‚ÓÎӄ˘ÌË ÛÒÎÓÊÌÂÌËfl, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ Ò ‰˙ÎÊ‡Ú Ì‡ Á‡Òfl„‡Ì ̇ ˆÂÌÚ‡Î̇ڇ ÌÂ‚̇ ÒËÒÚÂχ (ñçë) ËÎË Ì‡ ÔÂËÙÂ̇ڇ ÌÂ‚̇ ÒËÒÚÂχ (èçë) (14). ç‡È-¯ËÓÍÓ ‡ÁÔÓÒÚ‡ÌÂÌÓÚÓ Ë Ì‡È-‰Ó· ÔÓÁ̇ÚÓ ÛÒÎÓÊÌÂÌË ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÔË ıÓÌ˘ÌË ˜ÂÌÓ‰Ó·ÌË Á‡·ÓÎfl‚‡ÌËfl  ˜ÂÌÓ‰ӷ̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (9). ífl Ò Ô˘ËÒÎfl‚‡ Í˙Ï „ÛÔ‡Ú‡ ̇ ÏÂÚ‡·ÓÎËÚÌËÚ Â̈ÂÙ‡ÎÓÔ‡ÚËË, ÍÓËÚÓ Ò‡ ÔÓÒΉˈ‡ ̇ ‡ÁÒÚÓÈÒÚ‚Ó Ì‡ ÏÓÁ˙˜Ì‡Ú‡ Ó·Ïfl̇ ̇ ‚¢ÂÒÚ‚‡Ú‡ Ë Ì Ò ‰˙ÎÊ‡Ú Ì‡ ÒÚÛÍÚÛÌË ÏÓÁ˙˜ÌË ÎÂÁËË (15). Ç ÂÁÛÎÚ‡Ú Ì‡ ̇Û¯Â̇ڇ ÙÛÌ͈Ëfl ̇ ˜ÂÌÓ‰Ó·ÌËÚ ÍÎÂÚÍË, ˜ÂÌËflÚ ‰Ó· Ì ÏÓÊ ‰‡ ËÁÔ˙ÎÌfl‚‡ Ò‚ÓËÚ ÏÂÚ‡·ÓÎËÚÌË ÙÛÌ͈ËË – Ì  ‚ Ò˙ÒÚÓflÌË ‰‡ ÏÂÚ‡·ÓÎËÁË‡ ‰ˈ‡ Ó·‡ÁÛ‚‡ÌË ‚ ˜Â‚‡Ú‡ ÚÓÍÒËÌË Í‡ÚÓ ‡ÏÓÌflÍ, ÏÂ͇ÔÚ‡Ì, ÙÂÌÓÎ, Í˙ÒÓ‚ÂËÊÌË Ï‡ÒÚÌË ÍËÒÂÎËÌË Ë ‰. èË ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ ÚÂÁË ÔÓ‰ÛÍÚË Ôӂ˯‡‚‡Ú Ò‚ÓflÚ‡ ÒÂÛÏ̇ ÍÓ̈ÂÌÚ‡ˆËfl, ‡ ÒΉ ÚÓ‚‡ ÔÂÏË̇‚‡Ú ÔÂÁ Í˙‚ÌÓ-ÏÓÁ˙˜Ì‡Ú‡ ·‡ËÂ‡ ‚ ÏÓÁ˙͇ (10). è‡ÚÓ„ÂÌÂÁ‡Ú‡ ̇ ˜ÂÌÓ‰ӷ̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl  ÏÛÎÚËÙ‡ÍÚÓˇÎ̇ ͇ÚÓ Ò ӷÒ˙ʉ‡Ú ‰ˈ‡ ıËÔÓÚÂÁË. èÓ̇ÒÚÓfl˘ÂÏ Í‡ÚÓ Ì‡È-Ò˙˘ÂÒÚ‚ÂÌË Ò ÒÏflÚ‡Ú ÚÂÓËËÚ Á‡ Ôӂ˯ÂÌËÚ ÍÓ̈ÂÌÚ‡ˆËË Ì‡ ‡ÏÓÌfl͇ ‚ „·‚ÌËfl ÏÓÁ˙Í Ë Á‡ÒËÎÂ̇ڇ ÉÄåä-ωËË‡Ì‡ Ì‚ÓÚ‡ÌÒÏËÒËfl (10) íÂÏËÌ˙Ú ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ‚Íβ˜‚‡ ‚ Ò· ÒË Ì‚ÓÔÒËıˇÚ˘ÌËÚ ÒËÏÔÚÓÏË Ë ÒË̉ÓÏË, ÍÓËÚÓ Ò ‡Á‚Ë‚‡Ú ͇ÚÓ ÛÒÎÓÊÌÂÌË ̇ ÓÒÚ‡Ú‡, ÔÓ‰ÓÒÚ‡Ú‡ Ë ıÓÌ˘̇ڇ ıeÔ‡ÚÓˆÂÎÛ·̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. ê‡Á„‡Ì˘‡‚‡Ú Ò ÓÒÚ‡, ÔÓ‰ÓÒÚ‡ Ë ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (9). ç‡È-‡ÌÌËÚ ÔÓÏÂÌË ÔË ÌÂfl Ò‡ ‰ËÒÍÂÚÌË ÔÒËı˘ÌË Ë Ôӂ‰Â̘ÂÒÍË ÔÓÏÂÌË, ÍÓËÚÓ ÚÛ‰ÌÓ Ò ‰Ó·‚flÚ ÓÚ ÎÂ͇fl, ‡ ÔÓ-ÒÍÓÓ ÓÚ ·ÎËÁÍËÚ ̇ Ô‡ˆËÂÌÚ‡. ìÒÚ‡ÌÓ‚fl‚‡Ú Ò ÒÏÛ˘ÂÌËfl ‚ ÍÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ ‚ÌËχÌËÂÚÓ, ̇Û¯ÂÌËfl ‚ Ò˙Ìfl, ÌÂÒÔÓÍÓÈÒÚ‚Ë (8). èÓ-̇ڇÚ˙Í Ò ÔË·‡‚flÚ Ì‡Ï‡ÎÂ̇ ÔÒËıÓÏÓÚÓ̇ ‡ÍÚË‚ÌÓÒÚ, Ò˙ÒÚÓflÌËfl ̇ Ó·˙͇ÌÓÒÚ, Ò˙ÌÎË‚ÓÒÚ. èÂÁ ÒΉ‚‡˘ËÚ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÍÓ΢ÂÒÚ‚ÂÌËÚ ̇Û¯ÂÌËfl ‚ Ò˙Á̇ÌËÂÚÓ ÔÂ‡ÒÚ‚‡Ú ÔÓÒÚÂÔÂÌÌÓ ‚ ÒÓÔÓ Ë ÍÓχ. é˘Â ÔÂ‰Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÍÓχڇ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ó„ÌË˘Ì‡ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ (4). Ç ÔÓ‡ÌÌËÚ ÒÚ‡‰ËË ÏÓ„‡Ú ‰‡ Ò „ËÒÚË‡Ú ·ÓÎÂÒÚÌÓ ÛÒËÎÂÌË ÒÛıÓÊËÎÌË Ë Ì‡‰ÍÓÒÚÌË ÂÙÎÂÍÒË, ‡ ÔÓ-̇ڇÚ˙Í ÒÂ


‰ÂÍÂÏ‚Ë, 2007

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

ÔË·‡‚flÚ Ô‡ÚÓÎӄ˘ÂÌ ÂÙÎÂÍÒ Ì‡ Ň·ËÌÒÍË, ÒÏÛ͇ÚÂÎÂÌ Ë ı‚‡Ú‡ÚÂÎÂÌ ÂÙÎÂÍÒ Ì‡ üÌ˯‚ÒÍË, Ô‡ˆË‡ÎÌË Ë ‚ÚÓ˘ÌÓ-„ÂÌÂ‡ÎËÁË‡ÌË ÂÔËÎÂÔÚ˘ÌË ÔËÔ‡‰˙ˆË (14). íÂÏËÌ˙Ú ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ˜ÂÒÚÓ Ò ËÁÔÓÎÁ‚‡ ÔË ·ÓÎÌË Ò ˆËÓÁ‡ Ë Á̇˜ËÚÂÎÌÓ ÔÓÚÓÒËÒÚÂÏÌÓ ¯˙ÌÚË‡ÌÂ. ç‡È-˜ÂÒÚ‡Ú‡ Ô˘Ë̇ Á‡ ıÓÌ˘̇ڇ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ ‚ á‡Ô‡‰Ì‡ Ö‚ÓÔ‡ Ë ë‚Â̇ ÄÏÂË͇  ıÓÌ˘ÌËfl ‡ÎÍÓıÓÎËÁ˙Ï Ò ‡Á‚ËÚË ̇ ‡ÎÍÓıÓÎ̇ ˆËÓÁ‡. ïÓÌ˘̇ڇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ÏÓÊ ‰‡ Ëχ ÔÓ„ÂÒË‡˘Ó, ıÓÌ˘ÌÓ-ÂÏËÚË‡˘Ó ËÎË ÔÂÒËÒÚË‡˘Ó ÔÓÚ˘‡ÌÂ. çflÍÓË ÔÓ‚ÓÍË‡˘Ë Ù‡ÍÚÓË ÏÓ„‡Ú ‰‡ ‚ÎÓ¯‡Ú Ò˙ÒÚÓflÌËÂÚÓ Ì‡ ·ÓÎÌËfl. í‡ÍË‚‡ Ò‡ ÔÂ͇ÎÂÌÓ ‚ËÒÓÍÓ Ò˙‰˙ʇÌË ̇ ·ÂÎÚ˙ˆË ‚ ı‡Ì‡Ú‡, ıÂÏÓ‡„Ëfl ‚ ÒÚÓχ¯ÌÓ-˜‚ÌËfl Ú‡ÍÚ, ıËÔÓÍÒËfl, ıËÔÓ͇ÎËÂÏËfl, ÏÂÚ‡·ÓÎËÚ̇ ‡Î͇ÎÓÁ‡ Ë ‰. éÚÒÚ‡Ìfl‚‡ÌÂÚÓ ËÏ ÔÓ‰Ó·fl‚‡ Ì‚Ó-ÔÒËı˘ÌËfl ÒÚ‡ÚÛÒ. äÓ„‡ÚÓ ıÓÌ˘̇ڇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ‰ÓÒÚË„Ì ÒÚ‡‰ËÈ Ì‡ ÍÓχÚÓÁÌÓ Ò˙ÒÚÓflÌËÂ, ‰̇ ÔÓ-χÎ͇ ˜‡ÒÚ ÓÚ ·ÓÎÌËÚ Ò ‚˙ÁÒÚ‡ÌÓ‚fl‚‡, ‰Ó͇ÚÓ ÔË ÓÒڇ̇ÎËÚ ÓÒÚ‡‚‡Ú ‡Á΢ÌÓ ËÁ‡ÁÂÌË ÂÁˉۇÎÌË Ì‚ÓÎӄ˘ÌË Ë ÔÒËı˘ÌË ÒËÏÔÚÓÏË, ÍÓËÚÓ ‚Ó‰flÚ ‰Ó ËÌ‚‡ÎˉËÁ‡ˆËfl (15). ᇠÒÛ·ÍÎËÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl Ò „Ó‚ÓË, ÍÓ„‡ÚÓ Â‰ËÌ Ô‡ˆËÂÌÚ Ò ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ ·ÓÎÂÒÚ (̇Ô. ˆËÓÁ‡) Ëχ ÌÓχÎÂÌ Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ (7). Ç Ú‡ÍË‚‡ ÒÎÛ˜‡Ë Ò ËÁÔÓÎÁ‚‡ Ò˙˘Ó ڇ͇ ÚÂÏË̇ ◊ÏËÌËχÎ̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl” (1). èË ÔÓ‚Âʉ‡Ì ̇ ‰ˈ‡ ÔÒËıÓÏÂÚ˘ÌË ÚÂÒÚÓ‚Ë Ë/ËÎË Ì‚ÓÙËÁËÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl (̇È-˜ÂÒÚÓ ÖÖÉ) ÔÓÒΉÌËÚ ÔÓ͇Á‚‡Ú ‡·ÌÓÏÌË ÂÁÛÎÚ‡ÚË. íÓ‚‡ Ëχ „ÓÎflÏÓ Á̇˜ÂÌËÂ, Ú˙È Í‡ÚÓ ÏÓÊ ‰‡ Ò Á‡ÔӘ̠‡ÌÌÓ Î˜ÂÌË ̇ ˜ÂÌÓ‰ӷ̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (7). èÓ‚Âʉ‡ÌÂÚÓ Ì‡ ÔÓ‰Ó·ÌË Í˙‚ÌË Ë ·ËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl ÔÓÁ‚ÓÎfl‚‡Ú ËÁÍβ˜‚‡ÌÂÚÓ Ì‡ ‰Û„ ‚ˉ ÏÂÚ‡·ÓÎËÚ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl. äÓ̈ÂÌÚ‡ˆËflÚ‡ ̇ ÒÂÛÏÌËfl ‡ÏÓÌflÍ, ËÁÏÂÂÌ ‚ ÌflÍÓÎÍÓ ÔÓ·Ë ÓÚ ‡ÚÂˇÎ̇ Í˙‚  Ôӂ˯Â̇, ÌÓ ÍÓ·ˆËflÚ‡ ÏÂÊ‰Û Ì„ӂËÚ ÒÚÓÈÌÓÒÚË, Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ Ë ÖÖÉ – ÔÓÏÂÌËÚ  Ò··Ó ËÁ‡ÁÂ̇ (16).äí Ì ÏÓÊ ‰‡ ÔÓÒÚ‡‚Ë ‰Ë‡„ÌÓÁ‡Ú‡ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl, ÌÓ Â ÔÓÎÂÁ̇ ÔË ËÁÍβ˜‚‡ÌÂÚÓ Ì‡ Ó„‡Ì˘ÌË ÏÓÁ˙˜ÌË ÎÂÁËË Ò ‰Û„ ÔÓËÁıÓ‰. èË·„‡ÌÂÚÓ Ì‡ ÔÒËıÓÏÂÚ˘ÌË ÚÂÒÚÓ‚Â Â ÔÓ͇Á‡ÌÓ ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒÛ·ÍÎËÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl Ë ÌÓχÎÂÌ Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ. çflχ ‰ËÌӉۯˠÍÓÈ ÓÚ Úflı Úfl·‚‡ ‰‡ ·˙‰Â Ô‰ÔÓ˜ÂÚÂÌ ÔË ÚÓ‚‡ Á‡·ÓÎfl‚‡Ì (9). ÖÖÉ ËÁÒΉ‚‡ÌÂÚÓ Â ˜Û‚ÒÚ‚ËÚÂÎÌÓ Ë Ì‡‰ÂʉÌÓ Ò‰ÒÚ‚Ó ÔË ÓÔ‰ÂÎflÌ ÒÚ‡‰Ëfl ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. íÓ ÏÓÊ ‰‡  ‡·ÌÓÏÌÓ Ë ÔË ÒÛ·ÍÎËÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl. éÒÌÓ‚ÌËÚ ÖÖÉ-‡·ÌÓχÎËÚÂÚË ÔË ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl Ò‡ ÔÓ„ÂÒË‚ÌÓ, ·Ë·ÚÂ‡ÎÌÓ, ÒËÌıÓÌÌÓ Ì‡Ï‡Îfl‚‡Ì ̇ ‚˙ÎÌÓ‚‡Ú‡ ˜ÂÒÚÓÚ‡ Ë Â‰ÌÓ Û‚Â΢ÂÌË ̇ ‡ÏÔÎËÚÛ‰‡Ú‡ ̇ ‚˙ÎÌËÚÂ. ë˙˘Ó ڇ͇ Ò ̇·Î˛‰‡‚‡Ú Ô‡ÓÍÒËÁχÎÌË ÚËÙ‡ÁÌË ‚˙ÎÌË, ‰ÓË Ë ‚ ‡ÌÌËÚ ÒÚ‡‰ËË. ífl·‚‡ ‰‡ Ò ÓÚ·ÂÎÂÊË Ó·‡˜Â, ˜Â Ú Ò Ò¢‡Ú Ë ÔË ‰Û„ËÚ ÏÂÚ‡·ÓÎËÚÌË Â̈ÂÙ‡ÎÓÔ‡ÚËË (3, 5). ñÂÎÚ‡ ̇ ̇ÒÚÓfl˘ÂÚÓ ËÁÒΉ‚‡Ì  ÔÓ‚Âʉ‡Ì ̇ ÍÎËÌ˘ÌÓ, Ì‚ÓÔÒËıÓÎӄ˘ÌÓ Ë ÖÖÉ ËÁÒΉ‚‡Ì ÔË Ô‡ˆËÂÌÚË Ò ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ Ò Ó„Î‰ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ ÍÎËÌ˘ÌË Ë ÒÛ·ÍÎËÌ˘ÌË ÙÓÏË Ì‡ ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl. äãàçàóÖç äéçíàçÉÖçí èÓÛ˜ÂÌË Ò‡ 22 ·ÓÎÌË Ò ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡, 12 Ï˙ÊÂ Ë 10 ÊÂÌË, Ò˙Ò Ò‰̇ ‚˙Á‡ÒÚ 57.63 „. (ÓÚ 30 ‰Ó 75 „.). ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ÊÂÌËÚ  61 „., ‡ ̇ Ï˙ÊÂÚ – 54.8 „. ÖÚËÓÎÓ„ËflÚ‡ ÔË 6 ·ÓÎÌË Â çÇV ËÌÙÂ͈Ëfl (27.3%),

232

çëV ËÌÙÂ͈Ëfl ÔË 3-χ (13.6%), ÒÏÂÒÂ̇ ÂÚËÓÎÓ„Ëfl – ‚ËÛÒ̇ + ‡ÎÍÓıÓÎ̇ ÔË 2-χ (9.1%), ‡‚ÚÓËÏÛÌ̇ – 4 (18.2%), ÍËÔÚÓ„ÂÌ̇ – 2 (9.1%) Ë ‡ÎÍÓıÓÎ̇ ÔË 5 ·ÓÎÌË (22.7%). Ñˇ„ÌÓÁ‡Ú‡  ÔÓÒÚ‡‚Â̇ ‚˙Á ÓÒÌÓ‚‡ ̇ ÍÎËÌ˘ÌË, ··Ó‡ÚÓÌË Ë ËÌÒÚÛÏÂÌÚ‡ÎÌË ËÁÒΉ‚‡ÌËfl Ë Í·ÒËÙˈË‡Ì‡ ÔÓ Child: 7 ·ÓÎÌË Ò Child Ä (31.8%); Child Ç – 9 (40.9%) Ë Child ë – 6 (27.3%) (Ú‡·Î. 1). 퇷Îˈ‡ 1. äËÚÂË�� ̇ Child Á‡ ÚÂÊÂÒÚÚ‡ ̇ ˆËÓÁ‡Ú‡ èéäÄáÄíÖã ëÂÛÏÂÌ ·ËÎËÛ·ËÌ (µmol/l) ëÂÛÏÂÌ ‡Î·ÛÏËÌ (g/l) èÓÚÓÏ·ËÌÓ‚Ó ‚ÂÏ (Ë̉ÂÍÒ) ÄÒˆËÚ Ö̈ÂÙ‡ÎÓÔ‡ÚËfl

ëÚ‡‰ËÈ Ä < 35 > 36 > 70 % ãËÔÒ‚‡ ãËÔÒ‚‡

ëÚ‡‰ËÈ Ç 35-50 30-35 40-70 % ìÏÂÂÌ I-II ÒÚ.

ëÚ‡‰ËÈ ë > 50 < 30 < 40 % å‡ÒË‚ÂÌ III-IV ÒÚ.

ÇÒ˘ÍË ·ÓÎÌË Ò‡ ËÁÒΉ‚‡ÌË Ë ÎÂÍÛ‚‡ÌË ‚ äÎËÌË͇ ÔÓ „‡ÒÚÓÂÌÚÂÓÎÓ„Ëfl ÔË ìåÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇”, Í˙‰ÂÚÓ Â ÔÓÒÚ‡‚Â̇ ‰Ë‡„ÌÓÁ‡Ú‡ ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ Ë ÓÔ‰ÂÎÂÌË ÌÂÈ̇ڇ ÂÚËÓÎÓ„Ëfl Ë Í·ÒËÙˈË‡Ì ÔÓ Child. èË Ô‡ˆËÂÌÚËÚ Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡Ì‡ÏÌÂÒÚ˘ÌË, ÍÎËÌ˘ÌË Ë Ô‡‡ÍÎËÌ˘ÌË ‰‡ÌÌË Á‡ ‰Û„Ë ÚÂÊÍË ÒÓχÚ˘ÌË Á‡·ÓÎfl‚‡ÌËfl, Ó„‡Ì˘ÌË ËÎË ‰Û„Ë ÏÂÚ‡·ÓÎËÚÌË Â̈ÂÙ‡ÎÓÔ‡ÚËË, ͇ÍÚÓ Ë Á‡ ÔËÂχÌ ̇ ωË͇ÏÂÌÚË Ò ‰ÂÈÒÚ‚Ë ‚˙ıÛ ñçë. åÖíéÑà çÄ àáëãÖÑÇÄçÖ èË ‚Ò˘ÍË ·ÓÎÌË Ò‡ ÒÌÂÚË Ì‡ÒÓ˜Â̇ ‡Ì‡ÏÌÂÁ‡ Ë Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ, ͇ÍÚÓ Ë Ôӂ‰ÂÌË Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ Ò ÔÓÏÓ˘Ú‡ ̇ MMSE Ë ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì ̇ 20-͇̇ÎÂÌ ÂÎÂÍÚÓÂ̈ÂÙ‡ÎÓ„‡Ù Galileo. éˆÂÌfl‚‡ÌË ·flı‡ Ò ÛÚËÌÂÌ ‚ËÁÛ‡ÎÂÌ ‡Ì‡ÎËÁ Ò‰̇ڇ ˜ÂÒÚÓÚ‡ ̇ ‡ÎÙ‡-‚˙ÎÌËÚÂ Ë ÒÚÂÔÂÌÚ‡ ̇ ËÁ‡ÁÂÌÓÒÚ Ì‡ ‰ËÙÛÁÌËÚ ÔÓÏÂÌË. èË ÌÂÓ·ıÓ‰ËÏÓÒÚ ÔË ÌflÍÓË ·ÓÎÌË Ò‡ Ôӂ‰ÂÌË ‰ÓÔ˙ÎÌËÚÂÎÌË ËÁÒΉ‚‡ÌË Í‡ÚÓ äí ̇ „·‚ÂÌ ÏÓÁ˙Í Ë ÖåÉ Ò Ó„Î‰ ËÁÍβ˜‚‡Ì ̇ ‰Û„Ë Á‡·ÓÎfl‚‡ÌËfl ̇ ñçë Ë èçë. êÖáìãíÄíà чÌÌËÚ ÓÚ ‡Ì‡ÏÌÂÁ‡Ú‡ ÔÓ͇Á‚‡Ú, ˜Â ̇‰ 50% ÓÚ ·ÓÎÌËÚ ËÏ‡Ú ‡Á΢ÌË ÓÔ·͂‡ÌËfl, ̇È-˜ÂÒÚÓ ÛÏÓ‡, ÓÚÔ‡‰Ì‡ÎÓÒÚ Ë ·ÂÁÒËÎË (6 ·ÓÎÌË – 27.2%), ͇ÚÓ ÚÓ‚‡  ‰ËÌ ÓÚ ‡ÌÌËÚ ÒËÏÔÚÓÏË Ë Ò ̇·Î˛‰‡‚‡ ÔÓ-˜ÂÒÚÓ ‚ ÒÚ‡‰ËÈ Ä (ÔË 4 ÓÚ 7 Ô‡ˆËÂÌÚË). éÚ „·‚Ó·ÓÎË Ò ÓÔ·͂‡Ú ÚËχ ·ÓÎÌË (13.6%). íÓ Ò „ËÒÚË‡ ‚˙‚ ‚Ò˘ÍË ÒÚ‡‰ËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ᇠËÁÚ˙Ô‚‡ÌÂ, Ô‡ÂÒÚÂÁËË, Í‡ÏÔË Ë ·ÓÎÍË Ô‰ËÏÌÓ ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Ò˙Ó·˘‡‚‡Ú ÚËχ ÓÚ ·ÓÎÌËÚ (13.6%). ê‰ÍË ÒËÏÔÚÓÏË Ò‡ ·ÂÁÒ˙ÌËÂÚÓ (ÔË Â‰ËÌ ·ÓÎÂÌ ‚ ÒÚ‡‰ËÈ Ç) Ë Ò‚ÂÚÓ‚˙ÚÂʇ (‰ËÌ ·ÓÎÂÌ ‚ ÒÚ‡‰ËÈ Ä). è‡‚Ë ‚Ô˜‡ÚÎÂÌËÂ, ˜Â Ò‡ÏÓ Â‰Ì‡ Ô‡ˆËÂÌÚ͇ ÓÚ ˆÂÎËfl ÍÎËÌ˘ÂÌ ÍÓÌÚËÌ„ÂÌÚ Ò˙Ó·˘‡‚‡ Á‡ ÎÂÒÌÓ Á‡·‡‚flÌÂ, Á‡·‡‚ÂÌ „Ó‚Ó Ë ËÌÍÓÌÚËÌÂ̈Ëfl ̇ ÛË̇ڇ. èË Ó·ÂÍÚË‚ÌÓÚÓ Ì‚ÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì Ò ‡ÁÍË‚‡Ú ‰‡ÌÌË Á‡ ‚˙‚΢‡Ì ̇ ñçë ÔË 13 ·ÓÎÌË (59%). ãÂÍ ÔÒ‚‰Ó·Ûη‡ÂÌ ÒË̉ÓÏ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 5, Í‚‡‰ËÔË‡ÏˉÂÌ ÒË̉ÓÏ – ÔË 3-χ, ÔÓÒÚÛ‡ÎÂÌ Ë ËÌÚÂ̈ËÓÌÂÌ ÚÂÏÓ – ÔË 4-χ, ‡ ·‡‰ËÍËÌÂÁËfl Ë ‰ÂÏÂ̈Ëfl Ò ËÌÍÓÌÚËÌÂ̈Ëfl ̇ ÛË̇ڇ ÔË Â‰Ì‡ ·ÓÎ̇ ̇ 67 „Ó‰ËÌË. ëÚÂÔÂÌÚ‡ ̇ ‚˙‚΢‡Ì ̇ ñçë ̇‡ÒÚ‚‡ Ò Ì‡Ô‰‚‡Ì ̇ ˜ÂÌÓ‰ӷ̇ڇ ˆËÓÁ‡ (Ú‡·Î. 2). ì‚Âʉ‡Ì ̇ ÔÂËÙÂ̇ڇ ÌÂ‚̇ ÒËÒÚÂχ Ò ̇·Î˛‰‡‚‡ ÔË 9 ·ÓÎÌË (40.9%). èË 6 ÓÚ Úflı Ò ͇҇ Á‡ ‡ÎÍÓıÓÎ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ‡


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

‰ÂÍÂÏ‚Ë, 2007

퇷Îˈ‡ 2. Ç˙‚΢‡Ì ̇ ñçë ÒÔÓ‰ ÒÚ‡‰Ëfl ̇ ˆËÓÁ‡Ú‡ (n = 22) ñçë ëÚ‡‰ËÈ Ä Ç ë ÇÒ˘ÍÓ

ÅÂÁ Û˜‡ÒÚË ̇ ñçë 5 (71.4%) 4 (44.4%) 0 9

Ç˙‚΢‡Ì ̇ ñçë 2 (28.6%) 5 (55.6%) 6 (100%) 13 (59%)

ÇÒ˘ÍÓ 7 9 6 22

ÔË 3-χ – Á‡ ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. ÇÒ˘ÍË Ú ҇ ÓÚ ÒÂÚË‚ÂÌ, ‰ËÒÚ‡ÎÂÌ Ë ÒËÏÂÚ˘ÂÌ ÚËÔ. Ç ÒÚ‡‰ËÈ Ä Ëχ ‰ËÌ ÒÎÛ˜‡È Ò ÔÓÎËÌ‚ÓÔ‡ÚËfl, ‡ ÓÒڇ̇ÎËÚ 8 Ò‡ ‚ ÒÚ‡‰ËÈ Ç Ë ë (5 ‚ ÒÚ‡‰ËÈ Ç Ë 3 ‚ ÒÚ‡‰ËÈ ë). çÓχÎÂÌ Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË ÚËχ ·ÓÎÌË. éÚ Úflı ‰‚‡Ï‡ Ò‡ ‚ ÒÚ‡‰ËÈ Ä Ë Â‰ËÌ – ‚ ÒÚ‡‰ËÈ Ç. Ö‰ÌÓ‚ÂÏÂÌÌÓ Á‡Òfl„‡Ì ̇ ñçë Ë èçë Ò ̇·Î˛‰‡‚‡ ÔË ÚËχ ·ÓÎÌË, ‚ ÒÚ‡‰ËÈ Ç (‰‚‡Ï‡ ·ÓÎÌË) Ë ÒÚ‡‰ËÈ ë (‰ËÌ ·ÓÎÂÌ). çÖÇêéèëàïéãéÉàóçé àáëãÖÑÇÄçÖ ë ËÁÍβ˜ÂÌË ̇ Ô‡ˆËÂÌÚ͇ڇ Ò ‰ÂÏÂ̈Ëfl, ÔË ÌËÚÓ Â‰ËÌ ÓÚ ÓÒڇ̇ÎËÚ ·ÓÎÌË Ì Ò „ËÒÚË‡Ú ÓÔ·͂‡ÌËfl ÓÚ ÒÏÛ˘ÂÌËfl ‚ Ô‡ÏÂÚÚ‡, ‚ÌËχÌËÂÚÓ, ÓËÂÌÚ‡ˆËflÚ‡, ˜ڇ, ÏËÒÎÂÌÂÚÓ Ë ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ Âʉ̂ÌËÚ ‰ÂÈÌÓÒÚË. èÒËıÓÎӄ˘ÌËflÚ ÒÚ‡ÚÛÒ ÔÓ͇Á‚‡, ˜Â ·ÓÎÌËÚ ҇ ‚ flÒÌÓ Ò˙Á̇ÌËÂ Ë ‡ÔÒËıÓÚ˘ÌË. ç‚ÓÔÒËıÓÎӄ˘̇ڇ ÓˆÂÌ͇ ÔÓ Ò͇·ڇ MMSE ÛÒÚ‡ÌÓ‚fl‚‡ ÓÚÍÎÓÌÂÌËfl ÓÚ ÌÓχÎÌËÚ ÒÚÓÈÌÓÒÚË (28-30 ÚÓ˜ÍË) ÔË 13 ·ÓÎÌË (59%), ͇ÚÓ ÔÓ-˜ÂÒÚÓ Ò ͇҇ Á‡ „‡Ì˘ÌË ÒÚÓÈÌÓÒÚË ‰Ó ÎÂÍ ÍÓ„ÌË퇷Îˈ‡ 3. ç‚ÓÔÒËıÓÎӄ˘̇ ÓˆÂÌ͇ ̇ MMSE ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÒÚ‡‰Ëfl ̇ ˆËÓÁ‡Ú‡ (n = 22) ëÚ‡‰ËÈ/MMSE 28-30 25-27 15-24 ÇÒ˘ÍÓ Ä 6 (85.7%) 1 (14.3%) 0 7 Ç 3 (33.3%) 4 (44.5%) 2 (22.2%) 9 ë 0 4 (66.7%) 2 (33.3%) 6 ÇÒ˘ÍÓ 9 (40.9%) 9 (40.9%) 4 (18.2%) 22 ÚË‚ÂÌ ‰ÂÙˈËÚ. ë‡ÏÓ Â‰Ì‡ Ô‡ˆËÂÌÚ͇ ̇ 67 „Ó‰. Â Ò ÛÏÂÂÌÓ ËÁ‡ÁÂ̇ ‰ÂÏÂ̈Ëfl (15 ÚÓ˜ÍË). 燷≇‚‡ Ò ÓÔ‰ÂÎÂ̇ ÚẨÂ̈Ëfl Á‡ ̇‡ÒÚ‚‡Ì ̇ ÍÓ„ÌËÚË‚ÌËÚ ‡ÁÒÚÓÈÒÚ‚‡ Ò Ì‡Ô‰‚‡Ì ̇ ˜ÂÌÓ‰ӷ̇ڇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (Ú‡·Î. 3). äÓÌÚÓÎ˙Ú Ì‡ Ù‡ÍÚÓË ‚˙Á‡ÒÚ ÔÓ͇Á‚‡, ˜Â ÍÓ„ÌËÚË‚ÌËflÚ ‰ÂÙˈËÚ Ì Á‡‚ËÒË ÓÚ ‚˙Á‡ÒÚÚ‡ (Ú‡·Î. 4). ë ̇È-‰Ó·‡ ÓˆÂÌ͇ ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË Ò‡ Ô‡ˆËÂÌÚËÚ ‚˙‚ ‚˙Á‡ÒÚÓ‚Ëfl ËÌÚÂ‚‡Î ÓÚ 61-70 „Ó‰ËÌË. 퇷Îˈ‡ 4. ç‚ÓÔÒËıÓÎӄ˘̇ ÓˆÂÌ͇ ̇ MMSE ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ‚˙Á‡ÒÚÚ‡ (n = 22) Ç˙Á‡ÒÚ 28-30 25-27 15-24 ÇÒ˘ÍÓ ‰Ó 50 „Ó‰. 1 (25%) 2 (50%) 1 (25%) 4 51-60 „Ó‰. 3 (33.3%) 4 (44.5%) 2 (22.2%) 9 61-70 „Ó‰. 5 (62.5%) 2 (25%) 1 (12.5%) 8 > 70 „Ó‰. 1 1 ÇÒ˘ÍÓ 9 9 4 22 ÖÖÉ àáëãÖÑÇÄçÖ ÖÖÉ Ò „ËÒÚË‡ ‚ „‡ÌËˆË Ì‡ ÌÓχڇ Á‡ ‚˙Á‡ÒÚÚ‡ ÔË 9 ÓÚ ·ÓÎÌËÚ (40.9%), ̇È-˜ÂÒÚÓ ÔË ÚÂÁË ‚ ÒÚ‡‰ËÈ Ä Ì‡ ˜ÂÌÓ‰ӷ̇ڇ ˆËÓÁ‡ (Ú‡·Î. 5). ëÚÂÔÂÌÚ‡ ̇ ‰ËÙÛÁÌËÚ ÔÓÏÂÌË Ì‡‡ÒÚ‚‡Ú Ò Ì‡Ô‰‚‡Ì ̇ ˜ÂÌÓ‰Ó·ÌÓÚÓ Á‡·ÓÎfl‚‡ÌÂÚÓ. íÂÁË ‰‡ÌÌË Ò ÔÓÚ‚˙ʉ‡‚‡Ú ÔË Ò‡‚ÌËÚÂÎ̇ ÓˆÂÌ͇ ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‡ÎÙ‡-‚˙ÎÌËÚ ‚ Á‡-

233

퇷Îˈ‡ 5. ëÚÂÔÂÌ Ì‡ ËÁ‡ÁÂÌÓÒÚ Ì‡ ‰ËÙÛÁÌËÚ ÖÖÉ ÔÓÏÂÌË ÒÔÓ‰ ÒÚ‡‰Ëfl ̇ ˆËÓÁ‡Ú‡ (n = 22) ÖÖÉ ÔÓÏÂÌË çÓχÎ̇ ãÂÍË ‰ËÙÛÁÌË àÁ‡ÁÂÌË ÇÒ˘ÍÓ íÂÊÂÒÚ Ì‡ ̇ıӉ͇ ÔÓÏÂÌË ‰ËÙÛÁÌË ˆËÓÁ‡Ú‡ ÔÓÏÂÌË ëÚ‡‰ËÈ Ä 5 (71.4 %) 2 (28.6 %) 0 7 ëÚ‡‰ËÈ Ç 3 (33.3 %) 2(22.2 %) 4 (44.5 %) 9 ëÚ‡‰ËÈ ë 1 (16.7 %) 0 5 (83.3 %) 6 ÇÒ˘ÍÓ 9 (40.9 %) 4 (18.2 %) 9 (40.9 %) 22 ‚ËÒËÏÓÒÚ ÓÚ ÒÚ‡‰Ëfl ̇ ˆËÓÁ‡Ú‡ (Ú‡·Î. 6). Ç ÒÚ‡‰ËÈ ë ÔÂӷ·‰‡‚‡Ú Ô‡ˆËÂÌÚËÚÂ Ò ÌËÒÍË ˜ÂÒÚÓÚË Ì‡ ‡ÎÙ‡ËÚ˙χ ÓÚ 7.5 ‰Ó 8 ïˆ – 83.3%, ‰Ó͇ÚÓ ‚ ÒÚ‡‰ËÈ Ä – 85.7% Ò‡ Ò ÌÓχÎÂÌ Á‡ ‚˙Á‡ÒÚÚ‡ ‡ÎÙ‡-ËÚ˙Ï. à ‚ ÚÓÁË ÒÎÛ˜‡È ͇ÍÚÓ ÔË ÓˆÂÌ͇ ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË, ÖÖÉ ÂÁÛÎÚ‡ÚËÚ Ì ҇ ÓÚ‡ÊÂÌË ̇ Ù‡ÍÚÓ‡ ‚˙Á‡ÒÚ (Ú‡·Î. 7). éÚ 4 Ô‡ˆËÂÌÚË ‰Ó 50-„Ӊ˯̇ ‚˙Á‡ÒÚ, ‰‚‡Ï‡ Ò‡ Ò ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË, ÓÚ 8 ·ÓÎÌË ÏÂÊ‰Û 61 Ë 70 „Ó‰ËÌË – 4 Ò‡ Ò ÌÓχÎ̇ ̇ıӉ͇ Ë 4 – Ò ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË. Ç „ÛÔ‡Ú‡ ÏÂÊ‰Û 51 Ë 60 „Ó‰ËÌË ‡ÁÔ‰ÂÎÂÌËÂÚÓ ÏÂÊ‰Û ÌÓχÎ̇ ̇ıӉ͇, ÎÂÍË ‰ËÙÛÁÌË Ë ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË Â ‡‚ÌÓÏÂÌÓ. 臈ËÂÌÚ˙Ú Ì‡ 75 „Ó‰ËÌË Â Ò ÌÓχÎ̇ ÖÖÉ Ì‡ıӉ͇ Ë ‰Ó· Ó„‡ÌËÁË‡Ì ‡ÎÙ‡-ËÚ˙Ï Ò ˜ÂÒÚÓÚ‡ 10.5 ïˆ. ë‡ÏÓ ÔË ‰‚‡Ï‡ ÓÚ ·ÓÎÌËÚÂ, Ë ‰‚‡Ï‡Ú‡ ‚ ÒÚ‡‰ËÈ ë, Ò „ËÒÚË‡ Á‡ÔËÒ ÓÚ ÚËÙ‡ÁÌË ÓÒÚË ‚˙ÎÌË, Ò˜ËÚ‡Ì Á‡ ‰ËÌ ÓÚ ÚËÔ˘ÌËÚ ÖÖÉ Ô‡ÚÂÌË Á‡ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (ÙË„. 1 Ë 2). îÓ͇ÎÌËÚ ÖÖÉ ÔÓÏÂÌË Ò ̇·Î˛‰‡‚‡Ú Ò‡‚ÌËÚÂÎÌÓ í‡·Îˈ‡ 6. ë‰̇ ˜ÂÒÚÓÚ‡ ̇ α-‚˙ÎÌËÚ ‚ ÖÖÉ ÒÔÓ‰ ÒÚ‡‰Ëfl ̇ ˆËÓÁ‡Ú‡ (n = 22) ÖÖÉ ÔÓÏÂÌË íÂÊÂÒÚ̇ˆËÓÁ‡Ú‡ ëÚ‡‰ËÈ Ä ëÚ‡‰ËÈ Ç ëÚ‡‰ËÈ ë ÇÒ˘ÍÓ

ÑÓ 8 Hz

éÚ 8 – 9 Hz 燉 9 Hz ÇÒ˘ÍÓ

1 (14.3 %) 5 (55.6%) 5 (83.3%) 11 (50.0 %)

1 (14.3 %) 3 (33.3 %) 1 (16.7 %) 5 (22.7 %)

5 (71.4 %) 1 (11.1 %) 0 6 (27.3 %)

7 9 6 22

fl‰ÍÓ – ÔË 27.3% ÓÚ ÒÎÛ˜‡ËÚÂ, ͇ÚÓ ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚ Ì  ‚ ÒÚ‡‰ËÈ Ä. Ç˙ÔÂÍË ˜Â ÔË ·ÓÎÌËÚ ÌflχÏ ÍÎËÌ˘ÌË ‰‡ÌÌË Á‡ ÂÔËÎÂÔÚ˘ÌË ÔËÔ‡‰˙ˆË, ÔË 5 ·ÓÎÌË (22.7%) Ò „ËÒÚË‡ı‡ ÙÓ͇Î̇ ËÎË „ÂÌÂ‡ÎËÁË‡Ì‡ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ ‚ ‡Á΢̇ ÒÚÂÔÂÌ Ì‡ ËÁ‡ÁÂÌÓÒÚ (ÙË„. 3). ч‚ÌÓÒÚÚ‡ ̇ ˆËÓÁ‡Ú‡ Ì ÔÓ͇Á‚‡ ÍÓ·ˆËfl Ò ÖÖÉ Ì‡ıӉ͇ڇ. àÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË Ò ̇·Î˛‰‡‚‡Ú ͇ÍÚÓ ÔË Ô‡ˆËÂÌÚË Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎfl‚‡ÌÂ퇷Îˈ‡ 7. ÖÖÉ Ì‡ıӉ͇ ÒÔÓ‰ ‚˙Á‡ÒÚÚ‡ (n = 22) ÖÖÉ ÔÓÏÂÌË Ç˙Á‡ÒÚ ÑÓ 50 „Ó‰. éÚ 51 ‰Ó 60 „Ó‰. éÚ 61 ‰Ó 70 „Ó‰. 燉 71 „Ó‰. ÇÒ˘ÍÓ

çÓχÎ̇ ãÂÍË Ì‡ıӉ͇ ‰ËÙÛÁÌË ÔÓÏÂÌË 2 (50 %) 2 (22.2 %) 4 (44.5 %) 4 (50 %) 1 (100 %) 9 4

àÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË 2 (50 %) 3 (33.3 %) 4 (50 %) 9

ÇÒ˘ÍÓ 4 9 8 1 22

ÚÓ Ôӂ˜ ÓÚ Â‰Ì‡ „Ó‰Ë̇ (55.6%), ڇ͇ Ë ÔË Ô‡ˆËÂÌÚË Ò ÔÓ-Í‡Ú͇ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ – ‰Ó 1/2 „Ó‰ËÌË (28.6%) Ë ÓÚ 1/2 ‰Ó 1 „Ó‰Ë̇ (33.3%). ë˙ÔÓÒÚ‡‚͇ڇ ̇ Ò‰̇ڇ ˜ÂÒÚÓÚ‡ ̇ ‡ÎÙ‡-‚˙ÎÌËÚ  ӈÂÌ͇ڇ ̇ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË ÔÓ Ò͇·ڇ MMSE


‰ÂÍÂÏ‚Ë, 2007

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

234

îË„ 1‡

îË„. 1· îË„. 1. è. É. Ñ. ̇ 52 „., ÒÚ. ë ÖÖÉ Ò ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË, ÔÓ-ËÁ‡ÁÂÌË ‚ Îfl‚Ó Ò ÔÂËӉ˘ÂÌ ÚËÔ ‡ÍÚË‚ÌÓÒÚ ÓÚ ÚËÙ‡ÁÌË ÓÒÚË ‚˙ÎÌË ÔÓ͇Á‚‡ ̇΢ˠ̇ Á‡·‡‚flÌ ̇ ÓÒÌÓ‚ÌÓÚÓ Ú‡ÒÂ Ë ÔË ÒÎÛ˜‡ËÚÂ Ò ÌÓχÎÌË ÒÚÓÈÌÓÒÚË Ì‡ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË ÔÓ MMSE (33.2%), χ͇ Ë ÔÓ-fl‰ÍÓ ‚ Ò‡‚ÌÂÌË Ò˙Ò ÒÎÛ˜‡ËÚÂ Ò „‡Ì˘ÌË ÒÚÓÈÌÓÒÚË Ë ÎÂÍ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ (ÂÒÔ. 66.7% Ë 50%). éÅëöÜÑÄçÖ ç‡¯ËÚ ÂÁÛÎÚ‡ÚË ÔÓÚ‚˙ʉ‡‚‡Ú ÎËÚÂ‡ÚÛÌËÚ ‰‡ÌÌË Á‡ ‡ÁÌÓÓ·‡Á̇, ÌÓ ÎÂÍÓ ËÁ‡ÁÂ̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÔË ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Û ·ÓÎÌË Ò ˆËÓÁ‡ ̇ ˜ÂÌËfl ‰Ó· (15). ë‡‚ÌËÚÂÎÌÓ „ÓÎÂÏËflÚ ·ÓÈ ·ÓÎÌË (13), ÔË ÍÓËÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‡Á΢ÌË Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË Ë ÒË̉ÓÏË Ò Ú‡Í˙‚ ÔÓËÁıÓ‰, ·Ë ÏÓ„˙Î ‰‡ Ò ӷflÒÌË ÓÒ‚ÂÌ Ò ˜ÂÌÓ‰ӷ̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl Ë Ò Ô‡ÚÓ„ÂÌÂÚ˘̇ڇ ÓÎfl ̇ ıÓÌ˘ÌËfl ÂÚËÎËÁ˙Ï. èË Ò‰ÂÏ ·ÓÎÌË (31.8%) ÁÎÓÛÔÓÚ·‡Ú‡ Ò ‡ÎÍÓıÓÎ Ó·ÛÒ·‚fl ‡Á‚ËÚËÂÚÓ Ì‡ ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡. ïÓÌ˘ÌËflÚ ÂÚËÎËÁ˙Ï ·Ë ÏÓ„˙Î ‰‡ ‰Ó‚‰ ‰Ó ‡Á‚ËÚËÂÚÓ Ì‡ ‰ˈ‡ ÛÒÎÓÊÌÂÌËfl ÓÚ ÒÚ‡Ì‡ ̇

ñçë, ÍÓËÚÓ Ò‡ ÒΉÒÚ‚Ë ̇ ‰ËÂÍÚÌÓÚÓ ÚÓÍÒ˘ÌÓ ‰ÂÈÒÚ‚Ë ̇ ‡ÎÍÓıÓ· Ë Ì„ӂËÚ ‡Á„‡‰ÌË ÔÓ‰ÛÍÚË ‚˙ıÛ ÌÂfl, ͇ÍÚÓ Ë Ì‡ ‰ˈ‡ ̉ÓËÏ˙˜ÌË Ù‡ÍÚÓË. í‡ÍË‚‡ ÛÒÎÓÊÌÂÌËfl Ò‡ Â̈ÂÙ‡ÎÓÔ‡ÚËflÚ‡ ̇ ÇÂÌËÍÂ, χÎÍÓÏÓÁ˙˜Ì‡Ú��� ‡ÚÓÙËfl, ‡ÎÍÓıÓÎ̇ڇ ‰ÂÏÂ̈Ëfl, ÏËÂÎÓÔ‡ÚËfl Ë ‰. í ‚Ò˙˘ÌÓÒÚ ÏÓ„‡Ú ‰‡ Ò ÒÛÔÂÔÓÌË‡Ú ͇ÚÓ ÓÎË„ÓÒËÏÔÚÓÏÌË ËÎË ÙÛÒÚÌË ÙÓÏË ‚˙ıÛ ÔÓfl‚ËÚ ̇ ıÓÌ˘̇ڇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ˜ÂÌÓ‰ӷ̇ڇ ˆËÓÁ‡. ç‡Î˘ËÂÚÓ Ì‡ ıÓÌ˘ÂÌ ÂÚËÎËÁ˙Ï ÔË Ò‰ÂÏ Ô‡ˆËÂÌÚË Ë Ì‡ Á‡ı‡ÂÌ ‰Ë‡·ÂÚ ÔË ‰Û„Ë ÚËχ Ó·flÒÌfl‚‡ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ‡ÎÍÓıÓÎ̇ Ë ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl ÔË Ó·˘Ó 9 ·ÓÎÌË. èË ÌËÚÓ Â‰ËÌ ·ÓÎÂÌ Ì ̇·Î˛‰‡‚‡ıÏ ÙÂÌÓÏÂ̇ ̇ ÌÂÔÂÍ˙Ò̇ڇ ÏÛÒÍÛÎ̇ ÍÓÌÚ‡ÍˆËfl – Ú.̇. ‡ÒÚÂËÍÒËÒ, ÍÓÈÚÓ Â ÏÌÓ„Ó ı‡‡ÍÚÂÂÌ Á‡ ıÓÌ˘̇ڇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl (9). ᇠ‡ÁÎË͇ ÓÚ ‰Û„ËÚ ÏÂÚ‡·ÓÎËÚÌË Â̈ÂÙ‡ÎÓÔ‡ÚËË, ÍÎËÌ˘̇ڇ ͇ÚË̇ ̇ ıÓÌ˘̇ڇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ÏÓÊ ‰‡ ‚Íβ˜‚‡ ‰ˈ‡ ÂÍÒÚ‡ÔË‡ÏˉÌË ÒËÏÔÚÓÏË ‰Ó ÓÙÓÏflÌÂÚÓ Ì‡ ‰ËÌ


‰ÂÍÂÏ‚Ë, 2007

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îË„ 2‡

îË„. 2· îË„. 2. ë. ä. å. ̇ 67 „., ÒÚ. ë ÖÖÉ Ò ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ÔÓÏÂÌË, Ò ÔÂËӉ˘ÂÌ ÚËÔ ‡ÍÚË‚ÌÓÒÚ ÓÚ ÚËÙ‡ÁÌË ÓÒÚË ‚˙ÎÌË Ò ˆÂÌÚÓ-ÚÂÏÔÓ‡ÎÌËÚ ‡ÈÓÌË Ô‡ÍËÌÒÓÔÓ‰Ó·ÂÌ ÒË̉ÓÏ (14). Ç Ì‡¯Ëfl ÍÎËÌ˘ÂÌ ÍÓÌÚËÌ„ÂÌÚ Ì‡·Î˛‰‡‚‡ıÏ ҇ÏÓ ÔË Â‰Ì‡ ·ÓÎ̇ ·‡‰ËÍËÌÂÁËfl, Ò˙˜Âڇ̇ Ò ÛÏÂÂÌÓ ËÁ‡ÁÂ̇ ‰ÂÏÂ̈Ëfl. ífl ·Â¯Â ‚ ÒÚ‡‰ËÈ ë ̇ ˆËÓÁ‡Ú‡. èӂ‰ÂÌÓÚÓ äí ËÁÒΉ‚‡Ì ̇ „·‚ÌËfl ÏÓÁ˙Í ÔË ÌÂfl Ì ‡ÁÍË ÍÓÓ‚‡ ‡ÚÓÙËfl, ‰‡ÌÌË Á‡ ÏÛÎÚËËÌÙ‡ÍÚ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ËÎË ‰Û„‡ Ô˘Ë̇ Á‡ ‰ÂÏÂ̈Ëfl. Ç ÑÑ-ÓÚÌÓ¯ÂÌË ÓÒÚ‡‚‡ ‚˙ÁÏÓÊÌÓÒÚÚ‡ ‰‡ Ò ËÁÍβ˜Ë ‚ ·˙‰Â¯Â ·ÓÎÂÒÚÚ‡ ̇ ÄΈı‡ÈÏÂ. 燷≇‚‡Ì‡Ú‡ ‰ËÒӈˇˆËfl ÏÂÊ‰Û Ò‡‚ÌËÚÂÎÌÓ ÎÂÍËÚ Ì‚ÓÎӄ˘ÌË ÔÓfl‚Ë ÓÚ ÒÚ‡Ì‡ ̇ ñçë ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ Ë Ì‡Ô‰̇ÎËfl ÒÚ‡‰ËÈ Ì‡ ˆËÓÁ‡Ú‡ (ë) ÓÚ ‰Û„‡, ÔË 5 ÓÚ 6-Ú ·ÓÎÌË ‚ ÒÚ‡‰ËÈ ë, ·Ë Ïӄ· ‰‡ Ò ӷflÒÌË Ò Ô‡‚ËÎÌÓ ÔÓ‚Âʉ‡ÌËÚ ̇˜ËÌ Ì‡ ÊË‚ÓÚ Ë ı‡ÌÂÌÂ, ͇ÍÚÓ Ë Ì‡ ‡‰ÂÍ‚‡ÚÌÓÚÓ Î˜ÂÌË ÔË ÚÂÁË ·ÓÎÌË. íÓ‚‡ Ò ÔÓÚ‚˙ʉ‡‚‡ Ë ÓÚ Ù‡ÍÚ‡, ˜Â ÔË ÌËÚÓ Â‰ËÌ Ô‡ˆËÂÌÚ ‚ ÒÚ‡‰ËÈ Ç ËÎË ë Ì Ò „ËÒÚË‡ı‡ ÍÓ΢ÂÒÚ‚ÂÌË Ì‡Û¯ÂÌËfl ‚ Ò˙Á̇ÌËÂÚÓ (ÒÓÏÌÓÎÂÌÚÌÓÒÚ, ÒÓÔÓ ËÎË ÍÓχ).

çflχ¯Â ·ÓÎÌË, ÍÓËÚÓ ‰‡ Ò‡ ‰ÂÁÓËÂÌÚË‡ÌË Á‡ ‚ÂÏÂ, ÏflÒÚÓ Ë ÒÓ·ÒÚ‚Â̇ ΢ÌÓÒÚ. íËÔ˘ÌË ÓÔ·͂‡ÌËfl ÔË Ì‡¯ËÚ ԇˆËÂÌÚË ·flı‡ ˜Û‚ÒÚ‚ÓÚÓ Á‡ ÛÏÓ‡, ÓÚÔ‡‰Ì‡ÎÓÒÚ Ë ·ÂÁÒ˙ÌËÂ. èË ÔÓ‚Âʉ‡ÌÂÚÓ Ì‡ Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ÔÓ MMSE Ò͇·ڇ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÓÚÍÎÓÌÂÌË ÓÚ ÌÓχڇ ÔË 13 ·ÓÎÌË (59%), ͇ÚÓ Ì‡È-˜ÂÒÚÓ Ò ͇҇ Á‡ „‡Ì˘ÌË ÒÚÓÈÌÓÒÚË ‰Ó ÎÂÍ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ. éÚ˜ËÚ‡ Ò ÓÔ‰ÂÎÂ̇ ÚẨÂ̈Ëfl Á‡ ̇‡ÒÚ‚‡Ì ̇ ÍÓ„ÌËÚË‚ÌËÚ ‡ÁÒÚÓÈÒÚ‚‡ Ò Ì‡Ô‰‚‡Ì ̇ ˜ÂÌÓ‰ӷ̇ڇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÍÓÂÚÓ Ò ÔÓ‰˜ÂÚ‡‚‡ Ë ÓÚ ‰Û„Ë ‡‚ÚÓË (9). Ç ÒÎÛ˜‡fl Ì ÏÓÊ ‰‡ Ò ËÁÍβ˜Ë ‰ÓÔ˙ÎÌËÚÂÎÌÓ ÓÎflÚ‡ ̇ ıÓÌ˘̇ڇ ‡ÎÍÓıÓÎ̇ ËÌÚÓÍÒË͇ˆËfl ÔË Îˈ‡Ú‡ Ò ıÓÌ˘ÂÌ ÂÚËÎËÁ˙Ï. ë‡ÏÓ Ô‡ˆËÂÌÚ͇ڇ Ò ‰ÂÏÂ̈Ëfl Ò ÓÔ·͂‡¯Â ÓÚ Ì‡Û¯ÂÌË ‚ Ô‡ÏÂÚÚ‡ Á‡ ‡ÁÎË͇ ÓÚ ÓÒڇ̇ÎËÚ 21 Ô‡ˆËÂÌÚË. êÂÁÛÎÚ‡ÚËÚ ÓÚ ÖÖÉ ËÁÒΉ‚‡ÌËfl ÔÓÚ‚˙ʉ‡‚‡Ú


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îË„. 3· îË„. 3. ñ. à. í. ̇ 59 „. ÒÚ. Ç ÖÖÉ Ò Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ ÓÚ ˜ÂÒÚË Í‡ÚÍÓÚ‡ÈÌË, ·Ë·ÚÂ‡ÎÌÓ ÒËÌıÓÌËÁË‡ÌË ‡Áfl‰Ë ÓÚ ÔÓÎËÒÔ‡ÈÍ-‚˙Î̇ ÍÓÏÔÎÂÍÒË, Ò ‡ÏÔÎËÚÛ‰ÂÌ Ï‡ÍÒËÏÛÏ ‚ ˆÂÌÚ‡ÎÌËÚ ‡ÈÓÌË. ̇·Î˛‰ÂÌËflÚ‡ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ (2,6,13), ˜Â ÖÖÉ ÔË ıÓ- Ú „Ó‰ËÌË Ò ÔÂÔÓ˙˜‚‡Ú ÍÓ΢ÂÒÚ‚ÂÌË ÏÂÚÓ‰Ë Ì‡ ‡Ì‡Ì˘̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl ‰ÂÏÓÌÒÚË‡ ¯ËÓÍ ÎËÁ ͇ÚÓ ÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ Ë ‰. (2,13) ÒÔÓ‰ ‰ˈ‡ ‡‚ÒÔÂÍÚ˙ ÓÚ ÔÓÏÂÌË. èË ÚÓ‚‡ ÚÂÁË ÔÓÏÂÌË Ì ҇ ‰- ÚÓË (11,17) Í·Ò˘ÂÒ͇ڇ ÓˆÂÌ͇ ˜ÂÁ ÛÚËÌÂÌ ÁËÚÂÍË Ë Ò „ËÒÚË‡Ú ÔË Ì‡‰ ÔÓÎÓ‚Ë̇ڇ ÓÚ Ì‡¯ËÚ ÒÎÛ- ÎÂÌ ‡Ì‡ÎËÁ ̇ ÖÖÉ Â ‰ÓÒÚ‡Ú˙˜ÌÓ Ì‡‰Âʉ̇ ͇ÚÓ ÏÂÚÓ‰ Á‡ ˜‡Ë, ÍÓÂÚÓ Â ‚ Ò˙„·ÒËÂ Ò ÎËÚÂ‡ÚÛÌËÚ ‰‡ÌÌË (4). ä‡- ÓÔ‰ÂÎflÌ ̇ ‰ËÙÛÁÌËÚ ÔÓÏÂÌË ‚ ÖÖÉ. ÚÓ ÏÂÚÓ‰Ë͇, ÓÚ‡Áfl‚‡˘‡ ÙÛÌ͈ËÓ̇ÎÌÓÚÓ Ò˙ÒÚÓflÌË ë‡‚ÌËÚÂÎ̇ڇ ÓˆÂÌ͇ ̇ ÖÖÉ Ò Ì‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ Ì‡ ÏÓÁ˙͇, ÖÖÉ ÔÓ͇Á‚‡ ÔÓÏÂÌË, ÚflÒÌÓ Ò‚˙Á‡ÌË Ò˙Ò ËÁÒΉ‚‡Ì ÔÓ MMSE Ò͇·ڇ ‰Ó͇Á‚‡, ˜Â ÖÖÉ Â ÔÓÒÚÂÔÂÌÚ‡ ̇ ˜ÂÌÓ‰ӷ̇ڇ ‰ËÒÙÛÌ͈Ëfl (3,12). ÑËÙÛÁÌË- ˜Û‚ÒÚ‚ËÚÂÎÂÌ ÏÂÚÓ‰ Á‡ ÛÒÚ‡ÌÓ‚fl‚‡Ì ̇ ‡ÌÌË ÒÛ·ÍÎËÚ ÔÓÏÂÌË, Á‡·‡‚flÌÂÚÓ Ì‡ Ò‰̇ڇ ˜ÂÒÚÓÚ‡ ̇ ‡ÎÙ‡- Ì˘ÌË ‰ËÒÙÛÌ͈ËË Ì‡ ñçë ÔË ıÓÌ˘̇ ˜ÂÌÓ‰ӷ̇ Ì‚˙ÎÌËÚÂ, ͇ÍÚÓ Ë Ì‡Î˘ËÂÚÓ Ì‡ ÔÂËӉ˘ÂÌ ÚËÔ ‡ÍÚË‚- ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ. ÌÓÒÚ ‚ ÖÖÉ Ò Ò¢‡Ú ÔÓ-˜ÂÒÚÓ ‚ Í˙ÒÌËÚ ÒÚ‡‰ËË Ì‡ ˆËíËÙ‡ÁÌË ÓÒÚË ‚˙ÎÌË, ÓÔËÒ‚‡ÌË Í‡ÚÓ Â‰ËÌ ÓÚ ÒÔˆËÓÁ‡Ú‡. èË·„‡ÌËÚ ‚ ÎËÚÂ‡ÚÛ‡ Ò͇ÎË Á‡ ‚ËÁÛ‡ÎÂÌ ÍÓ- Ù˘ÌËÚ ԇÚÂÌË Á‡ ‰ËÒÏÂÚ‡·ÓÎËÚÌË Â̈ÂÙ‡ÎÓÔ‡ÚËË Î˘ÂÒÚ‚ÂÌ ‡Ì‡ÎËÁ (4,12) ÔÓÁ‚ÓÎfl‚‡Ú ÔÓ-ÚӘ̇ ÓˆÂÌ͇ ̇ (5,6,11) ̇·Î˛‰‡‚‡ıÏ Ò‡‚ÌËÚÂÎÌÓ fl‰ÍÓ (ÔÓ‰ 10% ÓÚ ÒÚÂÔÂÌÚ‡ ̇ ‰ËÙÛÁÌËÚ ÔÓÏÂÌË Ë ‰‡‚‡Ú ‚˙ÁÏÓÊÌÓÒÚ Á‡ ÒÎÛ˜‡ËÚÂ), ÍÓÂÚÓ Ò ӷflÒÌfl‚‡ Ò Ù‡ÍÚ‡, ˜Â Ôӂ˜ÂÚÓ ·ÓÎÌË ‡ÁÍË‚‡Ì ̇ ̇˜‡ÎÌË, ÒÛ·ÍÎËÌ˘ÌË ÔËÁ̇ˆË ̇ ‚˙‚΢‡- Ò‡ ‚ ÒÚ‡‰ËÈ Ì‡ ÍÓÏÔÂÌÒ‡ˆËfl Ë ÔË ÌËÚÓ Â‰ËÌ ÓÚ Úflı Ì Ò Ì ̇ ñçë ‚ ·ÓÎÂÒÚÌËfl ÔÓˆÂÒ. Ç˙ÔÂÍË ˜Â ÔÂÁ ÔÓÒΉÌË- ̇·Î˛‰‡‚‡ı‡ ÍÓ΢ÂÒÚ‚ÂÌË Ì‡Û¯ÂÌËfl ‚ Ò˙Á̇ÌËÂÚÓ.


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ç‡Î˘ËÂÚÓ Ì‡ ÙÓ͇ÎÌÓ Á‡·‡‚flÌ ‚ ÖÖÉ, ̇ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ (ÙÓ͇Î̇ ËÎË „ÂÌÂ‡ÎËÁË‡Ì‡) ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ ÏÓÊ ‰‡ Ò ҂˙ÊÂ Ò ˜ÂÌÓ‰ӷ̇ڇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl, ‡ ÓÚ ‰Û„‡ – ‰‡  ÂÁÛÎÚ‡Ú Ì‡ ÛÒÎÓÊÌÂÌËfl ̇ ıÓÌ˘ÌËfl ÂÚËÎËÁ˙Ï (ıÓÌ˘̇ ËÌÚÓÍÒË͇ˆËfl, ÔÂ͇‡ÌË óåí), Á‡ı‡ÂÌ ‰Ë‡·ÂÚ (Ò ‰Ó·‡‚˙˜Ì‡ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëfl) Ë ‰. íÂÊÂÒÚÚ‡ ̇ ÖÖÉ ÔÓÏÂÌËÚ ÓÚ„Ó‚‡fl ̇ ÒÚ‡‰Ëfl ̇ ˜ÂÌÓ‰ӷ̇ڇ ˆËÓÁ‡ (ÔÓ Child) Ë Ì‡ ÔÒËıÓÏÂÚ˘ÌËfl ÒÍÓ, ÍÓÂÚÓ Â ‚ Ò˙ÓÚ‚ÂÚÒÚ‚ËÂ Ò ÎËÚÂ‡ÚÛÌËÚ ‰‡ÌÌË (2). ífl·‚‡ ‰‡ Ò ÔÓ‰˜ÂÚ‡Â, ˜Â Ë ÔË ˜‡ÒÚ ÓÚ ÒÎÛ˜‡ËÚ ‚ ÒÚ‡‰ËÈ Ä ÔË ÎËÔÒ‡ ̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ Ë ÔË ÌÓχÎÂÌ ËÎË „‡Ì˘ÂÌ ÒÍÓ ÔÓ MMSÖ, Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰ËÙÛÁÌË ÔÓÏÂÌË Ò˙Ò Á‡·‡‚flÌ ̇ Ò‰̇ڇ ‡ÎÙ‡-˜ÂÒÚÓÚ‡. íÓ‚‡ ÔÓ͇Á‚‡, ˜Â ÖÖÉ Í‡ÚÓ Â‰ËÌ ÌÂËÌ‚‡ÁË‚ÂÌ Ë ÌÂÒÍ˙Ô ÏÂÚÓ‰ Á‡ ÓˆÂÌ͇ ̇ ÏÓÁ˙˜ÌËÚ ‰ËÒÙÛÌ͈ËË ÔË ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡ Â Ò ‚˙ÁÏÓÊÌÓÒÚË Á‡ ‡ÌÌÓ ‡ÁÍË‚‡Ì ̇ ÒÛ·ÍÎËÌ˘ÌËÚ ÔÓfl‚Ë Ì‡ ̇˜‡Î̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl Ë ÔÓÒΉfl‚‡Ì ̇ ‡Á‚ËÚËÂÚÓ Ì‡ ·ÓÎÂÒÚÌËfl ÔÓˆÂÒ, ͇ÍÚÓ Ë Ì‡ ÂÙÂÍÚ‡ ÓÚ Ôӂ‰ÂÌÓÚÓ Î˜ÂÌËÂ. ãàíÖêÄíìêÄ 1.

2.

3.

Äڇ̇ÒÓ‚‡ è, ä˙ÒÚ‚ ç, å‡Ò‡Î‰ÊË‚‡ ê, Ňı˜Â‚‡ÌÓ‚ ä, åËı‡ÈÎÓ‚‡ Ä, åÛÁË͇‰ÊË‚‡ É, è‡ÛÌÓ‚‡ ï, äÓÒÚ‡‰ËÌÓ‚‡ å, ÉÂÓ„Ë‚ Ñ. åËÌËχÎ̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙÓÎÓÔ‡ÚËfl ÔË Ô‡ˆËÂÌÚË Ò ˜ÂÌÓ‰ӷ̇ ˆËÓÁ‡. Å˙΄‡Ò͇ Ì‚ÓÎÓ„Ëfl, 7, 2007, 80-84. Amodio P, Pellegrini A, Ubiali E, Mathy I, Del Piccolo F, Orsato E, Gatta A, Guerit JM. The EEG assessment of low-grade hepatic encephalopathy: Comparison of an artificial neural network-expert system (ANNES) based evaluation with visual EEG readings and EEG spectral analysis. Clinical Neurolphysiology, 117, 2006, 2243-2251. Bickford RG, Butt HR. Hepatic coma: the electroencephalographic pattern. J Clin Invest, 34, 1955, 790-791.

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12. 13. 14. 15. 16. 17.

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Conn HO. Quantifying the severity of hepatic encephalopathy. In: Conn H, Bircher CH (eds) Hepatic encephalopathy. Bloomington : Medi – Ed Press, 1994, 13-26. Fish BJ. Fish & Spehlmann's EEG Primer: Basic Principles of Digital and Analog EEG, 3rd Edition ed, Elsevier, Amsterdam, 1999. Fisch BJ, Klass DW. The diagnostic specificity of triphasic waves. Electroencephalogr Clin Neurophisiol, 70, 1988, 1-8. Gitlin N, Lewis D, Hinklev L. The diagnosis and prevalence of subclinical hepatic encephalopathy in apparently healthy ambulant non – shunted patients with cirrhosis. J Hepatol, 3, 1986, 75-82. Haussinger D. Low grade cerebral edema and the pathogenesis of hepatic encephalopathy in cirrhosis. Hepatology, 43, 2006, 1187-1190. Jones, E.A., Weissenborn, K. Neurology and the liver. J. Neurol. Neurosurg. Psychiatry, 63, 1997, 279-293 Mullen KD, Dasarathy S. Hepatic encephalopathy. In: Sorrell M, Maddrey W (eds) Schiff’s diseases of the liver, 8th edition. Philadelphia Lippincott – Raven, 1999, 545-581. Niedermeyer E. Metabolic central nervous system disorders. In: Niedermeyer E, Da Silva FL (eds). Electroencephalography – basic principles, clinical applications, and related fields. Baltimore MA USA, Williams & Wilkins 1999a, 416-431. Parsons-Simith BG, Summerskill WHJ, Dawson AM, Sherlock S. The electroencephalograph in liver disease. Lancet, 2, 1957, 867-871. Reeves RR, Struve FA, Burke RS. Quantitative EEG analysis before and after liver transplantation. Clinical EEG and Neuroscience, 37, 2006, 34-40 Ropper A, Brown R. The acquired metabolic disorders of the nervous system. In: H, Brown RH (eds) Adams and Victor’s principles of neurology. McGraw – Hill, Medical Publishing Division New York, 2005, 959-982. Schulz JB. Metabolische und toxische Enzephalopathien. In: In: Brandt Th, Dichgans J, Diener H-Ch (Hrsg) Therapie und Verlauf neurologischer Erkrankungen 4. Auflage. Verlag W. Kohlhammer, 2003, 310-327 Stahl J. Studies of the blood ammonia in liver disease. Its diagnostic, prognostic and therapeutic significance. Ann Intern Med, 58, 1963, 1-24. Weissenborn K, Scholz M, Hinrichs H, Wiltfang J, Schmidt FW, Kunkel H. Neurophysiological assessment of early hepatic encephalopathy. Electroencephalogr Clin Neurophisiol, 75, 1990, 289-295.

éË„Ë̇ÎÌË ÒÚ‡ÚËË ÖÑàçàóçà à åçéÉéäêÄíçà Ä-Çöãçà èêà èÄñàÖçíà ë ÑàÄÅÖíçÄ çÖÇêéèÄíàü 1

Ä. ë‚. ÄÎÂÍ҇̉Ó‚1, ç. åÛ‡‰flÌ2, å. чÒ͇ÎÓ‚2, Å. à¯ÔÂÍÓ‚‡2 àÌÒÚËÚÛÚ ÔÓ ·ËÓÙËÁË͇, Å˙΄‡Ò͇ Ä͇‰ÂÏËfl ̇ ç‡ÛÍËÚÂ, ëÓÙËfl 2äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇”, ëÓÙËfl

SUMMARY SINGLE AND MULTIPLE A-WAVES IN PATIENTS WITH DIABETIC NEUROPATHY A. Alexandrov, N. Muradyan , M. Daskalov , B. Ishpekova Diabetic neuropathy is a nerve disorder caused by diabetes. A-waves are late responses of the muscle following the Mwave on electrical stimulation of a motor nerve. The aim of this study is recording and investigation of single and multiple A-waves in patients with diabetic neuropathy. The investigation was carried out on 66 patients with diabetic polyneuropathy: 16 women with age range of 17 to 72 years (mean age 55.8+ _12.4) and 38 men with age range of 33 to 79 years (mean age 66.7+ _10.2). All studies were performed at the University Hospital “Tzaritza Joanna”, Department of Neurology, Sofia, Bulgaria. A-waves were obtained during conventional F wave investigation. Sixteen supramaximal electrical stimulations with square wave pulses 0.2 ms in duration and a frequency below 1Hz were applied via a bipolar surface electrode. The A-waves were recorded with concentric needle electrode (diameter of 0.45 mm) at amplification of 200 mV/div from mm. frontalis, orbicularis oris, orbicularis oculi, and surface electrode from mm. abductor digiti minimi, abductor pollicis brevis, extensor digitorum brevis, abductor hallucis. Single A-

waves were observed only in 44 (66.7%) patients and multiple A-waves in 20 (24.2%) patients. Only in 6 (9.1%) patients with diabetic neuropathy were observed single and multiple A-waves. In patients with diabetic neuropathy A-waves were observed in the absence of F-waves. A-waves were present in patients depending on insulin or independently of insulin. In some patients with evidence of diabetic neuropathy A-waves were not observed. Although the mechanism of A-waves is completely unclear and its clinical importance hasn’t been revealed fully, it can be stated that A-waves have to be heeded in routine examinations and specified in EMG reports. KEY WORDS: A wave, multiple A-waves, diabetic neuropathy, EMG. êÖáûåÖ Ñˇ·ÂÚ̇ڇ Ì‚ÓÔ‡ÚËfl  ‰ÌÓ ÓÚ Ì‡È-˜ÂÒÚËÚ ÛÒÎÓÊÌÂÌËfl ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ. Ä-‚˙ÎÌË Ò‡ ̇·Î˛‰‡‚‡ÌË ÔË ÛÚËÌÌÓ ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡Ì ̇ F ‚˙Î̇ڇ. Ä-‚˙ÎÌËÚ Ò ‡Á΢‡‚‡Ú ÓÚ F ‚˙Î̇ڇ Ò ÔÓ-ÌËÒ͇ ‡ÏÔÎËÚÛ‰‡, ÍÓÌÒÚ‡ÌÚ̇ ÙÓχ Ë ÔÓ-Í˙Ò‡ ·ÚÂ̈Ëfl ̇ ÔÓÚÂ̈ˇ·. ÄÏÔÎËÚÛ‰‡Ú‡ ̇ Ä ‚˙Î̇ڇ Ì Á‡‚ËÒË ÓÚ ËÌÚÂÌÁËÚÂÚ‡ ̇ ÒÚËÏÛÎÌËfl ËÏÔÛÎÒ. èÓÛ˜‚‡ÌÂÚÓ Â Ôӂ‰ÂÌÓ ÔË 66 ·ÓÎÌË Ò ‰Ë‡·ÂÚ̇ ÔÓÎË-


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Ì‚ÓÔ‡ÚËfl, ÓÚ ÍÓËÚÓ 28 ÊÂÌË Ì‡ ‚˙Á‡ÒÚ ÓÚ 17 ‰Ó 72 „. (Ò‰̇ ‚˙Á‡ÒÚ 55.8+ _12.4) Ë 38 Ï˙Ê ̇ ‚˙Á‡ÒÚ ÓÚ 33 ‰Ó 79 „. (Ò‰̇ ‚˙Á‡ÒÚ 66.7+ _10.2). Ä-‚˙ÎÌËÚ ҇ ̇·Î˛‰‡‚‡ÌË ÔË ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡Ì ̇ F ‚˙Î̇ڇ Ò 16 ÒÛÔ‡Ï‡ÍÒËχÎÌË ÒÚËÏÛÎË Ò ˜ÂÒÚÓÚ‡ ÓÚ 1 Hz. ÖÎÂÍÚÓÒÚËÏÛ·ڇ‡ ÔÓ‰‡‚‡ Ô‡‚Ó˙„˙ÎÂÌ ËÏÔÛÎÒ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ 0.2 ms ̇ ·ËÔÓÎflÌËfl ÔÓ‚˙ıÌÓÒÚÂÌ ÂÎÂÍÚÓ‰ ÙËÍÒË‡Ì ‚˙ıÛ ÌÂ‚‡. Ä‚˙ÎÌËÚ ·flı‡ Á‡ÔËÒ‡ÌË Ò ÍÓ̈ÂÌÚ˘ÌË Ë„ÎÓ‚Ë ÂÎÂÍÚÓ‰Ë (‰Ë‡ÏÂÚ˙ 0.45mm Ë ÛÒË΂‡Ì 200 mV/div) ÓÚ mm. frontalis, orbicularis oris, orbicularis oculi, Ë Ò ÔÓ‚˙ıÌÓÒÚÌË ÂÎÂÍÚÓ‰Ë ÓÚ mm. abductor digiti minimi, abductor pollicis brevis, extensor digitorum brevis, abductor hallucis. ÇÒ˘ÍË ËÁÒΉ‚‡ÌËfl Ò‡ ËÁ‚˙¯ÂÌË Ò ÔÓÏÓ˘Ú‡ ̇ ÂÎÂÍÚÓÏËÓ„‡Ù PHASIS II ‚ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇”, ëÓÙËfl. éÚ ËÁÒΉ‚‡ÌËÚ ·ÓÎÌË ‚ 44 (66.7%) Ò ̇·Î˛‰‡‚‡Ú Ò‡ÏÓ Â‰ËÌ˘ÌË Ä-‚˙ÎÌË, ‚ (20) 24.2% ÏÌÓ„ÓÍ‡ÚÌË Ë Ò‡ÏÓ ‚ 6 (9.1%) ‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË (ÔË ‰‚‡ ‡Á΢ÌË ÏÛÒÍÛ· ̇ ‰ËÌ Ë Ò˙˘Ë Ô‡ˆËÂÌÚ). ÅÓfl ̇ Ä-‚˙ÎÌËÚ Ì Á‡‚ËÒË ÓÚ ÚËÔ‡ Ë ‰‡‚ÌÓÒÚÚ‡ ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ. Ö‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË Ò ̇·Î˛‰‡‚‡Ú ͇ÍÚÓ ÔË ‡ÌÌÓ Á‡Ò„̇ÚË, ڇ͇ Ë ÔË ‰˙Î„Ó Á‡Ò„̇ÚË Ô‡ˆËÂÌÚË ÓÚ ‰Ë‡·ÂÚ. èÓfl‚‡Ú‡ ̇ Ä‚˙ÎÌË ‚ÂÓflÚÌÓ Á‡ÒËÒË ÓÚ ÚÓ‚‡ ÍÓÎÍÓ ·˙ÁÓ Ë ‚ ͇͂‡ ÒÚÂÔÂÌ ‰Ë‡·ÂÚ̇ڇ ÔÓÎËÌ‚ÓÔ‡ÚËfl  ̇ÌÂÒ· Ò‚ÓÈÚ ÔÓ‡ÊÂÌËfl. àÌÚÂÂÒÂÌ Ù‡ÍÚ Â,˜Â Ä-‚˙ÎÌË Ì Ò ̇·Î˛‰‡‚‡Ú ÔË ‚Ò˘ÍË ‰ÓÍÓÁ‡ÌË Ô‡ˆËÂÌÚË Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. í˙ÒÂÌÂÚÓ Ì‡ ÚÓ˜ÌËfl ÙËÁËÓÎӄ˘ÂÌ ÏÂı‡ÌËÁ˙Ï ˘Â ‰‡‰Â ÓÚ„Ó‚Ó ̇ ÏÌÓ„Ó ÓÚ ÌÂflÒÌËڠ̢‡ ÓÍÓÎÓ ÔÓfl‚‡Ú‡ ̇ Ä-‚˙ÎÌË ÔË ‰Ë‡·ÂÚ̇ڇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. äãûóéÇà Ñìåà: Ä ‚˙ÎÌË, ÏÌÓ„ÓÍ‡ÚÌË Ä ‚˙ÎÌË, ‰Ë‡·ÂÚ̇ Ì‚ÓÔ‡ÚËfl, ÖåÉ. ÇöÇÖÑÖçàÖ Ñˇ·ÂÚ̇ڇ Ì‚ÓÔ‡ÚËfl (Ñç) Á‡Âχ Ô˙‚Ó ÏflÒÚÓ ÔÓ ˜ÂÒÚÓÚ‡ ÔÂ‰Ë ‡ÎÍÓıÓÎ̇ڇ ÔÓÎËÌ‚ÓÔ‡ÚËfl ‚ ‡Á‚ËÚËÚ ÒÚ‡ÌË ÒÔÓ‰ ‡Á΢ÌËÚ ÂÔˉÂÏËÓÎӄ˘ÌË ÔÓÛ˜‚‡ÌËfl. óÂÒÚÓÚ‡Ú‡ ̇ Ñç Ò ۂÂ΢‡‚‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ (10, 11, 12). èË·ÎËÁËÚÂÎÌÓ 2/3 ÓÚ Ô‡ˆËÂÌÚËÚ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ ËÏ‡Ú ÍÎËÌ˘ÌË ËÎË ÒÛ·ÍÎËÌ˘ÌË ‰‡ÌÌË Á‡ Ì‚ÓÔ‡ÚËfl. ífl ÏÓÊ ‰‡ Ò ‡Á‚Ë ÔË ‚Ò˘ÍË ÚËÔÓ‚Â Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. ê‡Á„‡Ì˘‡‚‡Ú Ò ÒËÏÂÚ˘ÌË Ë ‡ÒËÏÂÚ˘ÌË ÙÓÏË Ì‡ Ñç. ÑËÒÚ‡Î̇ڇ ÒËÏÂÚ˘̇ ÒÂÚË‚ÌÓ-ÏÓÚÓ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl  ̇È-˜ÂÒÚ‡Ú‡ ÙÓχ ̇ Ñç, Ë ‚ÂÓflÚÌÓ Ò˙ÒÚ‡‚Îfl‚‡ 75% ÓÚ ‚Ò˘ÍË Ô‡ˆËÂÌÚË. èÓ-‰ÍË ÙÓÏË Ì‡ Ñç Ò‡ ‡ÒËÏÂÚ˘ÌËÚ (ÙÓ͇ÎÌË ÙÓÏË, ̇ÔËÏÂ ÎÂÁËflÚ‡ ̇ III, VI ËÎË VII ˜ÂÂÔÌÓ ÏÓÁ˙˜ÌË ÌÂ‚Ë). ÑËÒÚ‡Î̇ڇ ÒËÏÂÚ˘̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl Ò ‡Á‚Ë‚‡ ·‡‚ÌÓ Ë ÔÓÒÚÂÔÂÌÌÓ. ë¢‡ Ò ÔË ‚˙Á‡ÒÚÌË Ë ‰Âˆ‡. Ç Ôӂ˜ÂÚÓ ÒÎÛ˜‡Ë Úfl Ò˙˜ÂÚ‡‚‡ ‡ÁÒÚÓÈÒÚ‚Ó ‚˙‚ ÙÛÌ͈ËËÚ ̇ ÒÂÚË‚ÌËÚÂ, ÏÓÚÓÌË-

îË„Û‡ 1. èÓˆÂÌÚÌÓ ‡ÁÔ‰ÂÎÂÌË ̇ ‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÔË ËÁÒΉ‚‡ÌËÚ ‰Ë‡·ÂÚ˘ÌÓ ·ÓÎÌË: (1) 44 (66.7 %) ËÏ‡Ú Ò‡ÏÓ Â‰ËÌ˘ÌË Ä-‚˙ÎÌË, (2) 20 (24.2 %) ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË Ë (3) 6 (9.1 %) ‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË (ÔË ‰‚‡ ‡Á΢ÌË ÏÛÒÍÛ· ̇ ‰ËÌ Ë Ò˙˘Ë Ô‡ˆËÂÌÚ).

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îË„Û‡ 2. Ö‰ËÌ˘̇ Ä-‚˙Î̇ ÓÚ n.tibialis ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. ÚÂ Ë ‡‚ÚÓÌÓÏÌËÚ ‚·Í̇. Ç Ì‡˜‡ÎÌËÚ ÒÚ‡‰ËË Ò Á‡Òfl„‡Ú Í‡Í‡Ú‡, ‡ ˙ˆÂÚ Ò ‡Ì„‡ÊË‡Ú ÔÓ-Í˙ÒÌÓ Ë ‚ ÔÓ-‰ÍË ÒÎÛ˜‡Ë. éÚ ÒÛ·ÛÍÚË‚ÌËÚ ÓÔ·͂‡ÌËfl ÔÂӷ·‰‡‚‡Ú ÒΉÌËÚ ÒËÏÔÚÓÏË: ËÁÚ˙Ô‚‡ÌÂ, Ï‡‚͇ۘÌÂ, Ô‡ÂÌÂ, Í‡ÏÔË, Ò··ÓÒÚ – ÔÓfl‚ÂÌË Ì‡È-̇Ô‰ ‚ ‰ËÒÚ‡ÎÌËÚ ۘ‡ÒÚ˙ˆË ̇ Í‡Í‡Ú‡ (13). óÂÁ Ë„ÎÂ̇ ÂÎÂÍÚÓÏËÓ„‡ÙËfl ÔË Ñç Ò ‰Ó͇Á‚‡Ú ‰ÂÌÂ‚‡ˆËÓÌÌË ÔÓÏÂÌË ‚ Á‡Ò„̇ÚËÚ ÏÛÒÍÛÎË. îË·Ë·ˆËË Ë ÔÓÎÓÊËÚÂÎÌË ÓÒÚË ‚˙ÎÌË Ò ̇·Î˛‰‡‚‡Ú ˜ÂÒÚÓ ‚ ‰ËÒÚ‡ÎÌËÚ ÏÛÒÍÛÎË ÔË ÓÚÒ˙ÒÚ‚Ë ̇ ËÁ‡ÁÂ̇ Ò··ÓÒÚ Ë ÛÏÓflÂÏÓÒÚ Û Ô‡ˆËÂÌÚËÚ (1, 2, 3). èË ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ̇ ÔÓ‚Âʉ‡ÌÂÚÓ Ì‡ ÔÂËÙÂÌËÚ ÏÓÚÓÌË ÌÂ‚Ë ÏÓ„‡Ú ‰‡ Ò ̇·Î˛‰‡‚‡Ú ‡Á΢ÌË ‚ˉӂ Ô‰ËÁ‚Ë͇ÌË ÓÚ„Ó‚ÓË Ò Î‡ÚÂÌÚÌÓÒÚË ÔÓ-Û‰˙ÎÊÂÌË ÓÚ Î‡ÚÂÌÚÌÓÒÚÚ‡ ̇ Ô‰ËÁ‚Ë͇ÌËfl ÒÛχÂÌ ÏÛÒÍÛÎÂÌ ‡ÍˆËÓÌÂÌ ÔÓÚÂ̈ˇΠ(ëåÄè). íÂÁË ÓÚ„Ó‚ÓË ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ‡Á„‡Ì˘ÂÌË ÒÔÓ‰ ÔÓÏfl̇ڇ ̇ ÚÂıÌËÚ ԇ‡ÏÂÚË (·ÚÂÌÚÌÓÒÚ, ‡ÏÔÎËÚÛ‰‡ Ë ˜ÂÒÚÓÚ‡ ̇ ÔÓfl‚‡), ÏflÒÚÓÚÓ Ì‡ ÒÚËÏÛ·ˆËfl ÔÓ ÔÂËÙÂÌËfl ÌÂ‚, ËÌÚÂÌÁËÚÂÚ‡ ̇ ÒÚËÏÛÎÌËfl ËÏÔÛÎÒ Ë Ôӂ‰ÂÌËÂÚÓ ËÏ ÔË ÒÚËÏÛ·ˆËfl Ò ‰‚ÓÈ͇ ËÏÔÛÎÒË. è˙‚ËflÚ ‚ˉ Ä-‚˙ÎÌË Ò‡ ÓÔËÒ‡ÌË ÓÚ Fullerton et al., (7) ͇ÚÓ ‡ÍÒÓÌ ÂÙÎÂÍÒ Ë Ò ı‡‡ÍÚÂËÁË‡ Ò ÍÓÌÒÚ‡ÌÚ̇

îË„Û‡ 3. Ö‰ËÌ˘̇ Ä-‚˙Î̇ ÓÚ n.tibialis ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ÍÓÈÚÓ Â Ì‡ ΘÂÌËÂ Ò ËÌÒÛÎËÌ ÓÚ 12 „Ó‰.


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ÚÂÚ‡ ̇ ÒÚËÏÛ·. Ç ÔÓÌflÚËÂÚÓ Ä-‚˙ÎÌË (AAEM, Glossary of Terms, 2001) Ò ‚Íβ˜‚‡Ú Ë ‡·ÌÓχÎÌË Í˙ÒÌË ÓÚ„Ó‚ÓË, ÍÓËÚÓ Ì ҇ ‡ÍÒÓÌ ÂÙÎÂÍÒË Ë Ò‡ ̇·Î˛‰‡‚‡ÌË ÔË ÛÚËÌÌÓ ËÁÒΉ‚‡Ì ̇ F-‚˙Î̇ڇ ÔË ÓÚ‚Âʉ‡ÌÂ Ò ÔÓ‚˙ıÌÓÒÚÌË ÂÎÂÍÚÓ‰Ë. Tomasulo (14) ÓÔËÒ‚‡ ÏÂʉËÌÂÌ ÓÚ„Ó‚Ó ÏÂÊ‰Û ÏÓÚÓÌËfl å-ÓÚ„Ó‚Ó Ë F-‚˙Î̇ Ë fl ̇˘‡ Í˙Ò̇ ÔÂËÙÂ̇ ‚˙Î̇, ‡ Bischoff et al., (6) ̇·Î˛‰‡‚‡Ú ÚÓÁË ÓÚ„Ó‚Ó Ë ÒΉ F-‚˙Î̇. Ä-‚˙ÎÌËÚ Ò ‡Á΢‡‚‡Ú ÓÚ F-‚˙Î̇ڇ Ò ÔÓ-ÌËÒ͇ڇ ÒË ‡ÏÔÎËÚÛ‰‡, ÔÓ-Í˙Ò‡ ·ÚÂ̈Ëfl Ë ÍÓÌÒÚ‡ÌÚ̇ ÙÓχ. ÄÏÔÎËÚÛ‰‡Ú‡ ̇ Ä-‚˙Î̇ڇ Ì Á‡‚ËÒË ÓÚ ËÌÚÂÌÁËÚÂÚ‡ ̇ ÒÚËÏÛÎÌËfl ËÏÔÛÎÒ (ÓÚ Ô‡„Ó‚ ‰Ó ÒÛÔ‡Ï‡ÍÒËχÎÂÌ ËÌÚÂÌÁËÚÂÚ). ñÂÎÚ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ Â Á‡ÔËÒ Ë ËÁÒΉ‚‡Ì ̇ ‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÔË Ô‡ˆËÂÌÚË Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl.

îË„Û‡ 4. Ö‰ËÌ˘̇ Ä-‚˙Î̇ ÓÚ n.peroneus ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl.

îË„Û‡ 5. Ö‰ËÌ˘̇ Ä-‚˙Î̇ ÓÚ n.medianus ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. ÙÓχ ̇ ÔÓÚÂ̈ˇ·, ÌÓ ÔÓfl‚‡Ú‡ ÏÛ Á‡‚ËÒË ÓÚ ËÌÚÂÌÁË-

äãàçàóÖç äéçíàçÉÖçí à åÖíéÑàäÄ èÓÛ˜‚‡ÌÂÚÓ Â Ôӂ‰ÂÌÓ ÔË 66 ·ÓÎÌË Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ÓÚ ÍÓËÚÓ 28 ÊÂÌË Ì‡ ‚˙Á‡ÒÚ ÓÚ 17 ‰Ó 72 „Ó‰. (Ò‰̇ ‚˙Á‡ÒÚ 55.8±12.4 „Ó‰.) Ë 38 Ï˙Ê ̇ ‚˙Á‡ÒÚ ÓÚ 33 ‰Ó 79 „Ó‰. (Ò‰̇ ‚˙Á‡ÒÚ 61.1±12.9 „Ó‰.). ч‚ÌÓÒÚ Ì‡ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ: ‰Ó 5 „Ó‰ËÌË – 22 ·ÓÎÌË; ÓÚ 5 ‰Ó 10 „Ó‰ËÌË – 18 ·ÓÎÌË; ÓÚ 10 ‰Ó 20 „Ó‰ËÌË – 20 ·ÓÎÌË Ë Ì‡‰ 20 „Ó‰ËÌË – 6 ·ÓÎÌË. ÇÒ˘ÍË ËÁΉ‚‡ÌËfl Ò‡ ËÁ‚˙¯ÂÌË Ò ÔÓÏÓ˘Ú‡ ̇ ÂÎÂÍÚÓÏËÓ„‡Ù PHASIS II ‚ äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ìåÅã “ñ‡ˈ‡ âÓ‡Ì̇”, ëÓÙËfl. ÇÒflÍÓ ËÁÒΉ‚‡Ì  Ôӂ‰ÂÌÓ ÔË ÍÓÊ̇ ÚÂÏÔÂ‡ÚÛ‡ ̇‰ 30 0C, ÔË ÒÚ‡È̇ ÚÂÏÔÂ‡ÚÛ‡ ÏÂÊ‰Û 23 0C Ë 26 0C. Ä-‚˙ÎÌËÚ ҇ ̇·Î˛‰‡‚‡ÌË ÔË ÛÚËÌÌÓ ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡Ì ̇ F-‚˙Î̇ڇ Ò 16 ÒÛÔ‡Ï‡ÍÒËχÎÌË ÒÚËÏÛÎË Ò ˜ÂÒÚÓÚ‡ ÔÓ-ÌËÒ͇ ÓÚ 1 Hz. ÖÎÂÍÚÓÒÚËÏÛ·ÚÓ‡ ÔÓ‰‡‚‡ Ô‡‚Ó˙„˙ÎÂÌ ËÏÔÛÎÒ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ 0.2 ms ̇ ·ËÔÓÎflÌËfl ÔÓ‚˙ıÌÓÒÚÂÌ ÂÎÂÍÚÓ‰ ÙËÍÒË‡Ì ‚˙ıÛ ÌÂ‚‡. ëÛχÌËÚ ÏÛÒÍÛÎÌË ‡ÍˆËÓÌÌË ÔÓÚÂ̈ˇÎË ·flı‡ Á‡ÔËÒ‚‡ÌË Ò ÍÓ̈ÂÌÚ˘ÌË Ë„ÎÓ‚Ë ÂÎÂÍÚÓ‰Ë (frontalis and orbicularis oris muscles, ‰Ë‡ÏÂÚ˙ 0.45 mm) Ë ÛÒË΂‡Ì 200 mV/div Ë ÔÓ‚˙ıÌÓÒÚÌË ÂÎÂÍÚÓ‰Ë (abductor digiti minimi, abductor pollicis brevis, extensor digitorum brevis, abductor hallucis muscles). êÖáìãíÄíà à éÅëöÜÑÄçÖ ç‡ îË„Û‡ 1  Ô‰ÒÚ‡‚ÂÌÓ ÔÓˆÂÌÚÌÓÚÓ ‡ÁÔ‰ÂÎÂÌË ÔË ËÁÒΉ‚‡ÌËÚ ‰Ë‡·ÂÚ˘ÌÓ ·ÓÎÌË. ìÒÚ‡ÌÓ‚fl‚‡ ÒÂ, ˜Â ‚ 44 (66.7 %) ÓÚ Ô‡ˆËÂÌÚËÚ Ëχ Ò‡ÏÓ Â‰ËÌ˘ÌË Ä-

îË„Û‡ 6. åÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÓÚ n. peroneus ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl.


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îË„Û‡ 7. åÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÓÚ n. facialis ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. ‚˙ÎÌË, ‚ 20 (24.2 %) ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË Ë Ò‡ÏÓ ‚ 6 (9.1 %) ‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË (ÔË ‰‚‡ ‡Á΢ÌË ÏÛÒÍÛ· ̇ ‰ËÌ Ë Ò˙˘Ë Ô‡ˆËÂÌÚ). ç‡ îË„ÛË 2 Ë 3 Ò‡ ÔÓ͇Á‡ÌË Â‰ËÌ˘ÌË Ä-‚˙ÎÌË ÓÚ n.tibialis ÔË ‰‚‡Ï‡ Ô‡ˆËÂÌÚË ÓÚ ÍÓËÚÓ ‚ÚÓËfl  ̇ ΘÂÌËÂ Ò ËÌÒÛÎËÌ ÓÚ 12 „Ó‰ËÌË (îË„Û‡ 3). Ö‰ËÌ˘ÌË Ä-‚˙ÎÌË ÓÚ n.peroneus (îË„Û‡ 4) Ë n.medianus (îË„Û‡ 5) Ò‡ ÔÓÎÛ˜ÂÌË ÔË ‰Ë‡·ÂÚÌÓ ·ÓÎÌË, ÍÓËÚÓ Ò‡ Ò ÌÂ-ËÌÒÛÎËÌÓÁ‡‚ËÒËÏ ‰Ë‡·ÂÚ. ç‡ îË„ÛË 6 Ë 7 Ò‡ ÔÓ͇Á‡ÌË ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÔË ËÁÒΉ‚‡ÌËÚ ÓÚ Ì‡Ò Ô‡ˆËÂÌÚË ÓÚ n.peroneus Ë n.facialis, ÍÓËÚÓ Ò‡ Ò ÌÂ-ËÌÒÛÎËÌÓÁ‡‚ËÒËÏ ‰Ë‡·ÂÚ. ç‡ îË„Û‡ 8 ÏÌÓ„ÓÍ‡ÚÌËÚ Ä-‚˙ÎÌË ÓÚ n.facialis ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ÍÓÈÚÓ Â Ì‡ ΘÂÌËÂ Ò ËÌÒÛÎËÌ. ç‡ îË„Û‡ 9 ÔË Ô‡ˆËÂÌÚ‡ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl ËχÏ ‰ËÌ˘̇ Ä-‚˙Î̇ ÓÚ n. tibialis Ë ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÔÓÎÛ˜ÂÌË ÓÚ n. peroneus. ÇÒ˘ÍËÚ ÚÂÁË Á‡ÔËÒË Ò‡ ÔÓÎÛ˜ÂÌË ‚ ÔËÒ˙ÒÚ‚ËÂ Ë Ì‡ F ‚˙ÎÌË. Ç ÎËÚÂ‡ÚÛ‡Ú‡ Ëχ ‰‡ÌÌË (5, 8, 9), Í˙‰ÂÚÓ Ò ÔÓÒÓ˜‚‡, ˜Â Ò‡ Óڂ‰ÂÌË ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÔË Ô‡ˆËÂÌÚË Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. èË Ì‡¯ËÚ ԇˆËÂÌÚË Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl

îË„Û‡ 8. åÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÓÚ n. facialis ÔË Ô‡ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl ̇ ΘÂÌËÂ Ò ËÌÒÛÎËÌ.

îË„Û‡ 9. Ö‰ËÌ˘̇ Ä-‚˙Î̇ ÓÚ n.tibialis Ë ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË ÓÚ n. peroneus ÔË Ô���ˆËÂÌÚ Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl. ̇·Î˛‰‡‚‡ıÏ Ä-‚˙ÎÌË Ë ÔË ÎËÔÒ‡ ̇ F ‚˙ÎÌË. Ç˙ÔÂÍË ‰Ó͇Á‡Ì‡Ú‡ ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl ÔË ÌflÍÓË ÓÚ Úflı Ì Óڂ‰ÓıÏ Ä-‚˙ÎÌË ÔË ÔÓ‰Ó·ÌÓ ÂÎÂÍÚÓÏËÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ. Ö‰ËÌ˘ÌË Ë ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË Ò ̇·Î˛‰‡‚‡Ú ÔË Ô‡ˆËÂÌÚË Ò ‰Ë‡·ÂÚ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ͇ÍÚÓ Ò ÔÓ-χÎ͇, ڇ͇ Ë Ò ÔÓ-„ÓÎflχ ‰‡‚ÌÓÒÚ Ì‡ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ. Ç „ÓÎflχ ˜‡ÒÚ ÓÚ Ì‡¯ËÚ ԇˆËÂÌÚË Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ Ò ̇·Î˛‰‡‚‡Ú ‰ËÌ˘ÌË Ä-‚˙ÎÌË (Ò ‰‡‚ÌÓÒÚ ‰Ó 5 „Ó‰ËÌË ËχÏ ÔË 13 (19.7 %), ÏÂÊ‰Û 6 Ë 10 „Ó‰ËÌË – 12 (18.2%), ÏÂÊ‰Û 11 Ë 20 „Ó‰ËÌË – 13 (19.7%) Ë Ì‡‰ 20 „Ó‰ËÌË ÔË 3 (4.5%) Ô‡ˆËÂÌÚ‡). èË Ô‡ˆËÂÌÚËÚÂ Ò Â‰ËÌ˘ÌË Ä-‚˙ÎÌË Ì‡ÏË‡Ï ÍÓ·ˆËÓÌ̇ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ‚˙Á‡ÒÚÚ‡ Ë ‰‡‚ÌÓÒÚÚ‡ ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ. äÓ·ˆËÓÌ̇ڇ Á‡‚ËÒËÏÓÒÚ Ò ‰‡‚ÌÓÒÚ ‰Ó 5 „Ó‰ËÌË Â r = 0.37, ÏÂÊ‰Û 6 Ë 10 „Ó‰ËÌË Â r = 0.54, ÏÂÊ‰Û 11 Ë 20 „Ó‰ËÌË r = 0.47, ̇‰ 20 „Ó‰ËÌË r = 0.79. èË ÍÓÂÙˈËÂÌÚË r = 0.37 Ë r = 0.47 Ò ӷÒ˙ʉ‡ ÛÏÂÂ̇, ÔË r = - 0.54 ËχÏ Á̇˜ËÚÂÎ̇ Ë ÔË r = 0.79 „ÓÎflχ ÍÓ·ˆËÓÌ̇ Á‡‚ËÒËÏÓÒÚ. Ç „ÛÔ‡Ú‡ ÏÂÊ‰Û 6 Ë 10 „Ó‰ËÌË ÍÓÂÙˈËÂÌÚ‡ ̇ ÍÓ·ˆËfl Ò ÔˉÛʇ‚‡ ÓÚ ÓÚˈ‡ÚÂ-


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ÎÂÌ Á̇Í, Ú.Â. ËχÏ ӷ‡Ú̇ ‚˙Á͇. èË Ô‡ˆËÂÌÚËÚÂ Ò ÏÌÓ„ÓÍ‡ÚÌË Ä-‚˙ÎÌË Ì‡ÏË‡Ï ÍÓ·ˆËÓÌ̇ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ‚˙Á‡ÒÚÚ‡ Ë ‰‡‚ÌÓÒÚÚ‡ ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ ‚ „ÛÔ‡Ú‡ ÏÂÊ‰Û 5 Ë 10 „Ó‰ËÌË, Í˙‰ÂÚÓ r = 0.41 Ë „Ó‚ÓËÏ Á‡ ÛÏÂÂ̇ ÍÓ·ˆËÓÌ̇ Á‡‚ËÒËÏÓÒÚ. èÓ‡‰Ë ÚÓ‚‡, ˜Â ‰Û„ËÚ „ÛÔË Ò‡ Ò˙Ò Ï‡Î˙Í ·ÓÈ Ô‡ˆËÂÌÚË Ì ÒÏ Ú˙ÒËÎË ÍÓ·ˆËÓÌ̇ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ‚˙Á‡ÒÚÚ‡ Ë ‰‡‚ÌÓÒÚÚ‡ ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ. èÓfl‚‡Ú‡ ̇ Ä-‚˙ÎÌËÚ ‚ÂÓflÚÌÓ Á‡‚ËÒË ÓÚ ÚÓ‚‡ ÍÓÎÍÓ ·˙ÁÓ Ë ‚ ͇͂‡ ÒÚÂÔÂÌ Ò  ‡Á‚Ë· ‰Ë‡·ÂÚ̇ڇ Ì‚ÓÔ‡ÚËfl. Ä-‚˙ÎÌË Ì Ò ̇·Î˛‰‡‚‡Ú ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ‰Ó͇Á‡Ì‡ ‰Ë‡·ÂÚ̇ Ì‚ÓÔ‡ÚËfl. ÅÓfl ̇ Ä-‚˙ÎÌËÚ Ì Á‡‚ËÒË ÓÚ ÚËÔ‡ ̇ Á‡ı‡ÌËfl ‰Ë‡·ÂÚ: ËÌÒÛÎËÌÓÁ‡‚ËÒËÏ ËÎË ÌÂ-ËÌÒÛÎËÌÓÁ‡‚ËÒËÏ Ë ÓÚ Ì„ӂ‡Ú‡ ‰‡‚ÌÓÒÚ. ê‡ÁÍË‚‡ÌÂÚÓ Ì‡ ÚÓ˜ÌËfl ÙËÁËÓÎӄ˘ÂÌ ÏÂı‡ÌËÁ˙Ï ˘Â ‰‡‰Â ÓÚ„Ó‚Ó ̇ ÌÂËÁflÒÌÂÌËڠ̢‡ ÓÍÓÎÓ ÔÓfl‚‡Ú‡ ̇ Ä-‚˙ÎÌË ÔË ‰Ë‡·ÂÚ̇ڇ Ì‚ÓÔ‡ÚËfl. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7. 8.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: ÄÎÂÍ҇̉˙ ÄÎÂÍ҇̉Ó‚, Ì.Ò. I ÒÚ., Í.·.Ì. àÌÒÚËÚÛÚ ÔÓ ·ËÓÙËÁË͇ Å˙΄‡Ò͇ Ä͇‰ÂÏËfl ̇ ç‡ÛÍËÚ ìÎ. Ä͇‰ É. ÅÓ̘‚, ·Î- 21, ëÓÙËfl 1113 Tel. +359 2 979 3791 E mail: alex@bio.bas.bg ËÎË asalexandrov@abv.bg Address for correspondence: Alexander S. Alexandrov, Ph.D. Institute of Biophysics Bulgarian Academy of Sciences Acad. G. Bonchev Str, Bldg. 21 Sofia 1113, Bulgaria Phone: +359 2 979 3791 E mail: alex@bio.bas.bg ËÎË asalexandrov@abv.bg


‰ÂÍÂÏ‚Ë, 2007

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

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éË„Ë̇ÎÌË ÒÚ‡ÚËË äéçÉÖçàíÄãÖç åàÄëíÖçÖç ëàçÑêéå íàè IÄ (îÄåàãçÄ àçîÄçíàãçÄ åàÄëíÖçàü) èêà ÅöãÉÄêëäàíÖ êéåà Ç. ÉÂ„ÂΘ‚‡1,10, à. ãËÚ‚ËÌÂÌÍÓ2, ë. óÂÌËÌÍÓ‚‡1, Ç. ÅÓÊËÌÓ‚‡1, Ç. åËı‡ÈÎÓ‚‡1, Å. à¯ÔÂÍÓ‚‡1, ã. ÄÌ„ÂÎÓ‚‡3, à. à‚‡ÌÓ‚4, è. ÑËÏÓ‚‡1, Ä. éÒ͇1, Ä. âÓ‰‡ÌÓ‚‡5, Å. Öχ6, Ä. ÖÌ„ÂÎ7, ï. ãÓÍÏ˛ÎÂ8, ã. ä‡Î‡È‰ÊË‚‡9, à. í˙Ì‚1,10 1 ä‡Ú‰‡ ÔÓ Ì‚ÓÎÓ„Ëfl, 2 ä‡Ú‰‡ ÔÓ Ô‰ˇÚËfl, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ - ëÓÙËfl; 3 ä‡Ú‰‡ ÔÓ Ô‰ˇÚËfl Ë Ï‰ˈËÌÒ͇ „ÂÌÂÚË͇, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ – LJ̇, 4 ä‡Ú‰‡ ÔÓ Ô‰ˇÚËfl, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ - èÎÓ‚‰Ë‚; 5 燈ËÓ̇Î̇ „ÂÌÂÚ˘̇ ··Ó‡ÚÓËfl, ëÅÄãÄÉ “å‡È˜ËÌ ‰ÓÏ”, 6 àÌÒÚËÚÛÚ ÔÓ ÏËÓÎÓ„Ëfl “Ň·ËÌÒÍË”, è‡ËÊ; 7 ä‡Ú‰‡ ÔÓ Ì‚ÓÎÓ„Ëfl, å‡ÈÓ ÍÎËÌË͇, êÓ˜ÂÒÚÂÒÍË ÛÌË‚ÂÒËÚÂÚ, ëÄô; 8 îˉËı ŇÛÂ àÌÒÚËÚÛÚ‡, å˛ÌıÂÌ, ÉÂχÌËfl; 9 ㇷÓ‡ÚÓËfl ÔÓ ÏÓÎÂÍÛÎfl̇ „ÂÌÂÚË͇, á‡Ô‡‰ÌÓ-Ä‚ÒÚ‡ÎËÈÒÍË ËÌÒÚËÚÛÚ Á‡ ωˈËÌÒÍË ËÁÒΉ‚‡ÌËfl, ìÌË‚ÂÒËÚÂÚ Ì‡ á‡Ô‡‰Ì‡ ÄÌÒÚ‡ÎËfl, è˙Ú, Ä‚ÒÚ‡ÎËfl; 10 îÓ̉‡ˆËfl “á‰‡‚ÌË ÔÓ·ÎÂÏË Ì‡ χΈËÌÒÚ‚‡Ú‡”, ëÓÙËfl SUMMARY CONGENITAL MYASTHENIC SYNDROME TYPE IA (FAMILIAL INFANTILE MYASTHENIA) IN BULGARIAN ROMA V. Guergueltcheva, I. Litvinenko, S. Cherninkova, V. Bojinova, V. Mihaylova, B. Ishpekova, L. Angelova, I. Ivanov, P. Dimova, A. Oscar, A. Jordanova, B. Eymard, A. Engel, H. Lochmuller, L. Kalaydjieva, I. Tournev A hundred and eight patients with congenital myasthenic syndrome type Ia were investigated from 49 non-related families (56 men and 52 women). The mean age of investigation of the patients is 11.5 years (age range 0-67 years). Ptosis and ophthalmoparesis are the most constant features of the disease regardless of the age of the patients. Ophthalmoparesis without diplopia is encountered in 108 patients and ptosis – in 106 patients. Fatigable weakness of the bulbar muscles is established in 87 patients and of the proximal limb muscles – in 81 patients. All patients show fluctuating weakness during the day and on exertion. Most of the patients report no progression of the disease. EMG study shows a decremental response at repetitive stimulation. The patients demonstrate a positive response to anticholinesterase treatment. The homozygous ε1267delG mutation was identified in all patients. KEY WORDS: Congenital myasthenic syndrome, Roma, autosomal recessive inheritance êÖáûåÖ àÁÒΉ‚‡ÌË Ò‡ 108 Ô‡ˆËÂÌÚË Ò äÓÌ„ÂÌËÚ‡ÎÂÌ ÏˇÒÚÂÌÂÌ ÒË̉ÓÏ ÚËÔ ¨‡ ÓÚ 49 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË (56 Ï˙ÊÂ Ë 52 ÊÂÌË). ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ËÁÒΉ‚‡ÌËÚ Îˈ‡  11.5 „Ó‰ËÌË /0-67 „Ó‰ËÌË/. èÚÓÁ‡Ú‡ Ë ‚˙̯̇ڇ ÓÙÚ‡ÎÏÓÔ‡ÂÁ‡ Ò‡ ̇È-ÔÓÒÚÓflÌÌËÚ ÒËÏÔÚÓÏË ÔË Á‡·ÓÎfl‚‡ÌÂÚÓ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‚˙Á‡ÒÚÚ‡ ̇ Ô‡ˆËÂÌÚËÚÂ. Ç˙̯̇ ÓÙÚ‡ÎÏÓÔ‡ÂÁ‡ ·ÂÁ ‰ËÔÎÓÔËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 108 Ô‡ˆËÂÌÚË, ‡ ÔÚÓÁ‡ – ÔË 106 Ô‡ˆËÂÌÚË. ÅÛη‡̇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 87 Ô‡ˆËÂÌÚË, ‡ ÔÓÍÒËχÎ̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ – ÔË 81 Ô‡ˆËÂÌÚË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Ì‡Îˈ ‰Ë̇ÏË͇ ÔÂÁ ‰ÂÌfl Ë ÔË ÙËÁ˘ÂÒÍË ÛÒËÎËfl. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË Ì Ò˙Ó·˘‡‚‡Ú Á‡ ÔÓ„ÂÒËfl ‚ ÔÓÚ˘‡ÌÂÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÖÎÂÍÚÓÏËÓ„‡ÙÒÍË ÔË ‚Ò˘ÍË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ̇Û¯ÂÌË ‚ Ì‚Ó-ÏÛÒÍÛÎÌÓÚÓ Ô‰‡‚‡Ì ÓÚ ÏˇÒÚÂÌÂÌ ÚËÔ. 燷≇‚‡ Ò ‰Ó·Ó ÚÂ‡Ô‚Ú˘ÌÓ ÔÓ‚ÎËfl‚‡Ì ÓÚ ‡ÌÚËıÓÎËÌÂÒÚÂ‡ÁÌË Ï‰Ë͇ÏÂÌÚË. èË ‚Ò˘ÍË Á‡Ò„̇ÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÏÛÚ‡ˆËflÚ‡ ε1267delG ‚ ıÓÏÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌËÂ. äãûóéÇà Ñìåà: äÓÌ„ÂÌËÚ‡ÎÂÌ ÏˇÒÚÂÌÂÌ ÒË̉ÓÏ, ÓÏË, ‡‚ÚÓÁÓÏÌÓˆÂÒË‚ÌÓ Û̇ÒΉfl‚‡ÌÂ.

ìÇéÑ äÓÌ„ÂÌËÚ‡ÎÌËflÚ ÏˇÒÚÂÌÂÌ ÒË̉ÓÏ ÚËÔ I‡ (äåë) ËÎË Ù‡ÏËÎ̇ڇ ËÌÙ‡ÌÚËÎ̇ ÏˇÒÚÂÌËfl /ÔÓ ä·ÒËÙË͇ˆËflÚ‡ ̇ ÍÓÌ„ÂÌËÚ‡ÎÌËÚ ÏˇÒÚÂÌÌË ÒË̉ÓÏË Ì‡ Ö‚ÓÔÂÈÒÍËfl Ì‚ÓÏÛÒÍÛÎÂÌ ˆÂÌÚ˙, 1998/  ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ˉÂÌÚËÙˈË‡ÌÓ Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Greer Ë Schotland (8). èÓ-Í˙ÒÌÓ ‰Û„Ë ‡‚ÚÓË (3, 6, 7, 15, 16) ÔÛ·ÎËÍÛ‚‡Ú ÍÎËÌ˘ÌËÚ ÒË Ì‡·Î˛‰ÂÌËfl ‚˙ıÛ ÒË̉Óχ. èӂ‰ÂÌËÚ in vitro ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌË Ë ÏÓÙÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl ÔË Ô‡ˆËÂÌÚË Ò äåë ÔÓ͇Á‚‡Ú, ˜Â ̇È-‚ÂÓflÚÌÓ Â Ì‡Îˈ ÔÂÒË̇ÔÚ˘ÂÌ ‰ÂÙÂÍÚ Ì‡ ‡ˆÂÚËÎıÓÎËÌÓ‚‡Ú‡ ÒËÌÚÂÁ‡ ËÎË ÏÓ·ËÎËÁ‡ˆËfl (12). Christodoulou K. Ë Ò˙Ú. (2) Ò˙·Ë‡Ú „ÓÎflχ „ÛÔ‡ ÓÚ Ô‡ˆËÂÌÚË Ò äåë ÓÚ ‡Á΢ÌË ‰˙ʇ‚Ë Ë ÔÓ‚Âʉ‡Ú ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì Á‡ Ò͇˜ÂÌÓÒÚ. í ͇ÚË‡Ú „Â̇, ÓÚ„Ó‚ÓÂÌ Á‡ äåë, ‚ ÚÂÎÓÏÂÌËfl ‡ÈÓÌ Ì‡ 17 ıÓÏÓÁÓχ. Middleton L. Ë Ò˙Ú. (10) ÓÚÍË‚‡Ú, ˜Â ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò äåë ËÏ‡Ú ÏÛÚ‡ˆËË ‚ ε Ò۷‰ËÌˈ‡Ú‡ ̇ ‡ˆÂÚËÎıÓÎËÌÓ‚Ëfl ˆÂÔÚÓ. ìÒÚ‡ÌÓ‚fl‚‡ ÒÂ, ˜Â 4 ÓÚ 12 ËÁÒΉ‚‡ÌË Ù‡ÏËÎËË, ÍÓËÚÓ Ò‡ Ò ˆË„‡ÌÒÍË ÔÓËÁıÓ‰ ÓÚ É˙ˆËfl Ë íÛˆËfl, ËÏ‡Ú Ó·˘‡ ÏÛÚ‡ˆËfl ε1267delG ‚ ıÓÏÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌËÂ, ̇Û¯‡‚‡˘‡ ‡Ï͇ڇ ̇ ˜ÂÚÂÌÂ. à‰ÂÌÚËÙˈË‡ÌË Ò‡ ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ „Â̇, ÓÚ„Ó‚‡ÂÌ Á‡ ε Ò۷‰ËÌˈ‡Ú‡, ‚Ó‰Â˘Ë ‰Ó ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌË ÍÓÌ„ÂÌËÚ‡ÎÌË ÏˇÒÚÂÌÌË ÒË̉ÓÏË (5, 10, 11, 14). Abicht A. Ë Ò˙Ú. (1) ÔÓ‚Âʉ‡Ú ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì ̇ 13 Ô‡ˆËÂÌÚË Ò äåë ÓÚ 11 ÌÂÒ‚˙Á‡ÌË ˆË„‡ÌÒÍË Ù‡ÏËÎËË ÓÚ ‡Á΢ÌË ˛„ÓËÁÚÓ˜ÌÓ-‚ÓÔÂÈÒÍË ‰˙ʇ‚Ë (ìÌ„‡Ëfl, ë˙·Ëfl, å‡Í‰ÓÌËfl, íÛˆËfl Ë äÓÒÓ‚Ó). Ä‚ÚÓËÚ ÓÚÍË‚‡Ú ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË ıÓÏÓÁË„ÓÚ̇ ‰ÂΈËfl ̇ G ÌÛÍÎÂÓÚˉ‡ ‚ 1 ÂÍÁÓÌ Ì‡ „Â̇, ÓÚ„Ó‚ÓÂÌ Á‡ ? Ò۷‰ËÌˈ‡Ú‡ (ε1267delG). ÉÂÌÓÚËÔÌËflÚ ‡Ì‡ÎËÁ ÔÓ͇Á‚‡, ˜Â ‚Ò˘ÍË ËÁÒΉ‚‡ÌË ˆË„‡ÌÒÍË Ù‡ÏËÎËË Ò äåë ̇È-‚ÂÓflÚÌÓ ÔÓËÁıÓʉ‡Ú ÓÚ Ó·˘ Ô‡Ó‰ËÚÂÎ. Ç Å˙΄‡Ëfl í˙Ì‚ Ë Ò˙Ú. (1997) Ô˙‚Ë ÓÚÍË‚‡Ú ˆË„‡ÌÒ͇ Ù‡ÏËÎËfl Ò äåë ‚ „. ê‡ÍËÚÓ‚Ó. åÄíÖêàÄã à åÖíéÑà àÁÒΉ‚‡ÌË Ò‡ 108 Ô‡ˆËÂÌÚË ÓÚ 49 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË (56 Ï˙ÊÂ Ë 52 ÊÂÌË), ÔË̇‰ÎÂʇ˘Ë Í˙Ï ‡Á΢ÌË ÓÏÒÍË „ÛÔË (ÙË„. 1). ë‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ËÁÒΉ‚‡ÌËÚ Îˈ‡  11.5 „Ó‰ËÌË /0-67 „Ó‰ËÌË/. ÑËÁ‡ÈÌ˙Ú Ì‡ ËÁÒΉ‚‡Ì ‚Íβ˜‚‡: Ô˙ÎÌÓ Ì‚ÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ; ·ËÓıËÏ˘ÌÓ ËÁÒΉ‚‡Ì – èää, ÏÛÒÍÛÎÌË ÂÌÁËÏË; ÂÎÂÍÚÓÏËÓ„‡ÙÒÍÓ ËÁÒΉ‚‡Ì – ÂÎÂÍÚÓÌ‚Ó„‡ÙËfl, Ë„ÎÂ̇ ÂÎÂÍÚÓÏËÓ„‡ÙËfl, ÂÔÂÚ‡Ú˂̇ ÒÚËÏÛ·ˆËfl Ò ‡Á΢ÌË ˜ÂÒÚÓÚË; ÖäÉ Ë ÖıÓäÉ; äÄí ̇ ωˇÒ-


‰ÂÍÂÏ‚Ë, 2007

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

ÚËÌÛÏ; ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡Ì - ËÁÔÓÎÁ‚‡Ì  SSCP Ë DNA ÒÂÍ‚Â̈ËÓÌÂÌ ‡Ì‡ÎËÁ Á‡ ˉÂÌÚËÙˈË‡Ì ̇ ÏÛÚ‡ˆËflÚ‡. êÖáìãíÄíà ÅÂÏÂÌÌÓÒÚÚ‡ Ë ‡Ê‰‡ÌÂÚÓ ÔË ‚Ò˘ÍË Á‡Ò„̇ÚË ËÌ‰Ë‚Ë‰Ë Ò‡ ÌÓχÎÌË. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ ̇Îˈ ‡Ì‡ÏÌÂÒÚ˘ÌË ‰‡ÌÌË, ÔÓ‰‡‰ÂÌË ÓÚ Ó‰ËÚÂÎËÚÂ, Á‡ ÒËÏÔÚÓÏË, ÛÒÚ‡ÌÓ‚ÂÌË ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ ÒΉ ‡Ê‰‡ÌÂÚÓ ËÎË ‚ ‡ÌÌÓ ‰ÂÚÒÚ‚Ó. ëÔ‡‰‡Ì ̇ ÍÎÂÔ‡˜ËÚÂ Ë Á‡ÚÛ‰ÌÂÌË Ó˜ÌË ‰‚ËÊÂÌËfl, Ò·· Ô·˜, Á‡ÚÛ‰ÌÂÌÓ ÒÛ͇ÌÂ Ë „˙Îڇ̠ҠÒ˙Ó·˘‡‚‡Ú ‚ ‡Á΢̇ ÒÚÂÔÂÌ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ë Ò‡ ÔÓ-ËÁ‡ÁÂÌË ‚ ‡ÌÌÓ ‰ÂÚÒÚ‚Ó. ÇÒ˘ÍË Ô‡ˆËÂÌÚË ÔÓıÓʉ‡Ú ̇‚ÂÏÂ, ÌÓ ‰‡‚‡Ú ‡Ì‡ÏÌÂÒÚ˘ÌË ‰‡ÌÌË Á‡ ÛÏÓflÂÏÓÒÚ ÔË ıÓ‰ÂÌÂ Ë ‚ ˜‡ÒÓ‚ÂÚ ÔÓ ÙËÁ˘ÂÒÍÓ ‚˙ÁÔËÚ‡ÌË ‚ Û˜ËÎË˘Ì‡ ‚˙Á‡ÒÚ. èË ‚Ò˘ÍË Â Ì‡Îˈ ÔÓ‰˜Âڇ̇ ‰Ë̇ÏË͇ ̇ ÓÔ·͂‡ÌËflÚ‡, ͇ÚÓ Ú Ò Á‡ÒË΂‡Ú ÔÂÁ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚Ë̇ ̇ ‰ÂÌfl Ë ÔË ÙËÁ˘ÂÒÍË ÛÒËÎËfl. èÂÁ Ô˙‚‡Ú‡ „Ó‰Ë̇ ÓÚ ÊË‚ÓÚ‡ ÍÎËÌ˘̇ڇ ÒËÏÔÚÓχÚË͇  ̇È-ÚÂÊ͇ Ë Ò˙˘ÂÒÚ‚Û‚‡ ̇È-‚ËÒÓÍ ËÒÍ Á‡ ÊË‚ÓÚ‡ ̇ Ô‡ˆËÂÌÚËÚÂ. 25% ÓÚ Úflı ·ÓΉۂ‡Ú ˜ÂÒÚÓ ÓÚ ÂÒÔË‡ÚÓÌË ËÌÙÂ͈ËË, ‡ 5 Ô‡ˆËÂÌÚË ÔÂ͇‚‡Ú ÂÒÔË‡ÚÓÂÌ ‰ËÒÒÚÂÒ ÒË̉ÓÏ. Ç ıÓ‰‡ ̇ ËÁ‡ÒÚ‚‡ÌÂÚÓ Ë ÓÒÓ·ÂÌÓ ‚ Áfl· ‚˙Á‡ÒÚ Ò ̇·Î˛‰‡‚‡ ÔÓÒÚÂÔÂÌÌÓ ÒÚ‡·ËÎËÁË‡Ì ̇ Ò˙ÒÚÓflÌËÂÚÓ, ÌflÍÓË ÓÚ ÓÔ·͂‡ÌËflÚ‡ ̇χÎfl‚‡Ú, ‡ ‰Û„Ë ‰ÓË ËÁ˜ÂÁ‚‡Ú. çflÍÓË ÔÓ‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË ‰ÓË ‡·ÓÚflÚ ÚÂÊ͇ ÙËÁ˘ÂÒ͇ ‡·ÓÚ‡ Ë ËÏ‡Ú ÏËÌËχÎÌË ÓÔ·͂‡ÌËfl, ÔË ÚÓ‚‡ ·ÂÁ ‰‡ ÔÓ‚Âʉ‡Ú ‡ÌÚËıÓÎËÌÂÒÚÂ‡ÁÌÓ Î˜ÂÌËÂ. èË ÚË Ô‡ˆËÂÌÚÍË Ì‡ ‚˙Á‡ÒÚ 7, 21 Ë 41 „. Á‡·ÓÎfl‚‡ÌÂÚÓ ÔÓÚ˘‡ ÔÓ-ÚÂÊÍÓ ÓÚ ÓÒڇ̇ÎËÚ ԇˆËÂÌÚË Í‡ÚÓ ÔË Úflı  Á‡ÚÛ‰ÌÂ̇ ÔÓıӉ͇ڇ ÔÓ‡‰Ë ÒËÎÌÓ ËÁ‡ÁÂ̇ڇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ. éÚ ËÁÒΉ‚‡ÌÂÚÓ Ì‡ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ Ò ÛÒÚ‡ÌÓ‚fl‚‡, ˜Â ÔÚÓÁ‡Ú‡ Ë ‚˙̯̇ڇ ÓÙÚ‡ÎÏÓÔ‡ÂÁ‡ Ò‡ ̇È-ÔÓÒÚÓflÌÌËÚ ÒËÏÔÚÓÏË ÔË Á‡·ÓÎfl‚‡ÌÂÚÓ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‚˙Á‡ÒÚÚ‡ ̇ Ô‡ˆËÂÌÚËÚÂ. Ç˙̯̇ ÓÙÚ‡ÎÏÓÔ‡ÂÁ‡ ·ÂÁ ‰ËÔÎÓÔËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ 108 Ô‡ˆËÂÌÚË, ‡ ÔÚÓÁ‡ – ÔË 106 Ô‡ˆËÂÌÚË. ÅÛη‡̇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ Ò ÔÓfl‚Ë Ì‡ ‰ËÒÙ‡„Ëfl, ‰ËÒÙÓÌËfl Ë ÎÂ͇ ‰ËÁ‡ÚËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 87 Ô‡ˆËÂÌÚË, ‡ ÔÓÍÒËχÎ̇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ – ÔË 81 Ô‡ˆËÂÌÚË. èË ÚË Ô‡ˆËÂÌÚÍË Ò ÔÓ-ÚÂÊÍÓ ÔÓfl‚ÂÌ ÏˇÒÚÂÌÂÌ ÒË̉ÓÏ, ÔÓÍÒËχÎ̇ڇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ Â Ò˙˜Âڇ̇ Ë Ò ÏÛÒÍÛÎ̇ ‡ÚÓÙËfl, ÍÓflÚÓ Ì‡È-‚ÂÓflÚÌÓ Â ‚ÚÓ˘̇. èË ÚÂÁË Ô‡ˆËÂÌÚÍË ÔÓıӉ͇ڇ  ÒËÎÌÓ Á‡ÚÛ‰ÌÂ̇ ÔÓ ÏËÓÔ‡ÚÂÌ ÚËÔ. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Â Ì‡Îˈ ‰Ë̇ÏË͇ ÔÂÁ ‰ÂÌfl Ë ÔË ÙËÁ˘ÂÒÍË ÛÒËÎËfl. çËÚÓ Â‰ËÌ Ô‡ˆËÂÌÚ Ì Ò˙Ó·˘‡‚‡ Á‡ ÔÓ„ÂÒËfl ‚ ÔÓÚ˘‡ÌÂÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÅÛη‡ÌËÚ ÒËÏÔÚÓÏË Ë ÔÓÍÒËχÎ̇ڇ ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ò‡ Ò ÚẨÂ̈Ëfl ‰‡ ̇χÎfl‚‡Ú, ‡ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË Ë ‰‡ ËÁ˜ÂÁ‚‡Ú ‚ ÔÓ-Í˙Ò̇ ‚˙Á‡ÒÚ. 85 % ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò äåë ‚˙‚ ‚˙Á‡ÒÚÓ‚Ëfl ‰Ë‡Ô‡ÁÓÌ 0-10 „. ‰ÂÏÓÌÒÚË‡Ú ·Ûη‡̇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ, Á‡ ‡ÁÎË͇ ÓÚ Ô‡ˆËÂÌÚËÚ ‚˙‚ ‚˙Á‡ÒÚÓ‚ËÚ ‰Ë‡Ô‡ÁÓÌË 11-20 „. Ë 21-67 „., Í˙‰ÂÚÓ ÔÓˆÂÌÚËÚ ҇ ÂÒÔÂÍÚË‚ÌÓ 70 % Ë 34 %. èÓÍÒËχÎ̇ڇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ Ò˙˘Ó Ò ̇·Î˛‰‡‚‡ ‚ ̇È-‚ËÒÓÍ ÔÓˆÂÌÚ (89 %) ‚˙‚ ‚˙Á‡ÒÚÓ‚Ëfl ‰Ë‡Ô‡ÁÓÌ 0-10 „. Ë Ì‡Ï‡Îfl‚‡ ‚ ‰Û„ËÚ ‰‚‡ ‚˙Á‡ÒÚÓ‚Ë ‰Ë‡Ô‡ÁÓÌË - ÂÒÔÂÍÚË‚ÌÓ ‰Ó 77 % Ë 45%. êÂÒÔË‡ÚÓÂÌ ‰ËÒÒÚÂÒ ÒË̉ÓÏ Â Ì‡·Î˛‰‡‚‡Ì ÔË ÔÂÚ ‰Âˆ‡ ‚ Ô˙‚‡Ú‡ „Ó‰Ë̇ ÓÚ ÊË‚ÓÚ‡. çËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚ ‚ ÔÓ-Í˙Ò̇ ‚˙Á‡ÒÚ Ìflχ ‰Ëı‡ÚÂÎÌË Ì‡Û¯ÂÌËfl. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Á̇˜ËÏË ‡Á΢Ëfl ͇ÍÚÓ ÏÂÊ‰Û ‰‚‡Ú‡ ÔÓ·, ڇ͇ Ë ÏÂÊ‰Û Ù‡ÏËÎÌËÚÂ Ë ÒÔÓ‡‰Ë˜ÌËÚ ÒÎÛ˜‡Ë. Ç Ò˙˘ÓÚÓ ‚ÂÏ ӷ‡˜Â Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ËÌÚÂ- Ë ËÌÚ‡Ù‡ÏËÎÌË ÙÂÌÓÚËÔÌË ‚‡ˇˆËË ‚ ÔÓÚ˘‡ÌÂÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ÅËÓıËÏ˘ÌËÚ ËÁÒΉ‚‡ÌËfl ‚ÍÎ. Ë ÒÚÓÈÌÓÒÚËÚ ̇ Í‡ÚËÌÙÓÒÙÓÍË̇Á‡Ú‡ Ò‡ ‚ ÌÓχ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË.

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ÖÎÂÍÚÓÏËÓ„‡ÙÒÍÓÚÓ ËÁÒΉ‚‡Ì ̇ Îˈ‚ ÏÛÒÍÛÎ (m.nasalis) ÔË ‚Ò˘ÍË ÛÒÚ‡ÌÓ‚fl‚‡ ̇Û¯ÂÌË ‚ Ì‚ÓÏÛÒÍÛÎÌÓÚÓ Ô‰‡‚‡Ì ÓÚ ÏˇÒÚÂÌÂÌ ÚËÔ. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰‚ÓÈÌË å-ÓÚ„Ó‚ÓË ÔË Â‰ËÌ˘̇ ÒÚËÏÛ·ˆËfl ̇ ÌÂ‚‡. ÖäÉ Ë ÖıÓäÉ, ÓÒ˙˘ÂÒÚ‚ÂÌË ÔË 19 Ô‡ˆËÂÌÚË, Ò‡ ‚ ÌÓχ. èӂ‰ÂÌËÚ äÄí ̇ ωˇÒÚËÌÛχ ÔË 10 Ô‡ˆËÂÌÚË Ë äÄí ̇ „·‚ÂÌ ÏÓÁ˙Í ÔË 5 Ì ÛÒÚ‡ÌÓ‚fl‚‡Ú Ô‡ÚÓÎÓ„Ëfl. èË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‰Ó·Ó ÚÂ‡Ô‚Ú˘ÌÓ ÔÓ‚ÎËfl‚‡Ì ÓÚ ‡ÌÚËıÓÎËÌÂÒÚÂ‡ÁÌË Ï‰Ë͇ÏÂÌÚË. íÓ Â ÔÓ-ÓÚ˜ÂÚÎË‚Ó ‚ ‡Ì̇ڇ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ. Ç Ì‡È-„ÓÎflχ ÒÚÂÔÂÌ Ò ÔÓ‚ÎËfl‚‡ ·Ûη‡̇ڇ Ë ÔÓÍÒËχÎ̇ڇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ, ‚ ÔÓ-χÎ͇ ÒÚÂÔÂÌ – ÔÚÓÁ‡Ú‡. ç‡È-ÂÁËÒÚÂÌÚ̇ ̇ ‡ÌÚËıÓÎËÌÂÒÚÂ‡Á̇ڇ ÚÂ‡ÔËfl Ò Ó͇Á‚‡ ÓÙÚ‡ÎÏÓÔ‡ÂÁ‡Ú‡. êÓ‰ËÚÂÎËÚ ̇ ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ Á‰‡‚Ë Ë Ì ÔÓ͇Á‚‡Ú ÏˇÒÚÂÌ̇ ÒËÏÔÚÓχÚË͇. ç‚ÓÎӄ˘ÌËflÚ ÒÚ‡ÚÛÒ Ì‡ ‚Ò˘ÍË Ó‰ËÚÂÎË Â ·ÂÁ ÓÚÍÎÓÌÂÌËfl. èË ‰‚ Ó‰ËÚÂÎÒÍË ‰‚ÓÈÍË Â Ôӂ‰ÂÌÓ Ë ÂÎÂÍÚÓÏËÓ„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ Ë Ì  ÛÒÚ‡ÌÓ‚Â̇ ÏˇÒÚÂÌ̇ ‡͈Ëfl. ä˙‚ÌÓÓ‰ÒÚ‚ÂÌË ·‡ÍÓ‚Â Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ÔË 7 Ù‡ÏËÎËË Ò äåë ÚËÔ I‡. êÓ‰ÓÒÎÓ‚ÌËflÚ ‡Ì‡ÎËÁ ̇ Ù‡ÏËÎËËÚÂ Ò äåë ÔÓ͇Á‚‡ ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÂÌ ÚËÔ Ì‡ Û̇ÒΉfl‚‡ÌÂ. éÚ ‚Ò˘ÍË ·ÓÎÌË Ò äåë Ë ÚÂıÌËÚ Á‰‡‚Ë Ó‰ÌËÌË Â ‚ÁÂÚ‡ Í˙‚ Ë Â ÂÍÒÚ‡ıË‡Ì‡ Ñçä. åÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì  ÓÒ˙˘ÂÒÚ‚ÂÌÓ ‚ êÓ˜ÂÒÚÂÒÍËfl ÛÌË‚ÂÒËÚÂÚ, ëÄô, àÌÒÚËÚÛÚ‡ ÔÓ ÏËÓÎÓ„Ëfl “Ň·ËÌÒÍË”, è‡ËÊ, î‡ÌˆËfl, îˉËı ŇÛÂ àÌÒÚËÚÛÚ‡, å˛ÌıÂÌ, ÉÂχÌËfl Ë ç‡ˆËÓ̇Î̇ڇ „ÂÌÂÚ˘̇ ··Ó‡ÚÓËfl ̇ ìëÅÄãÄÉ “å‡È˜ËÌ ‰ÓÏ”. èË ‚Ò˘ÍË Á‡Ò„̇ÚË, Ù‡ÏËÎÌË Ë ÒÔÓ‡‰Ë˜ÌË ÒÎÛ˜‡Ë, Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÏÛÚ‡ˆËflÚ‡ ε1267delG ‚ ıÓÏÓÁË„ÓÚÌÓ, ‡ ÔË ÚÂıÌËÚ Ó‰ËÚÂÎË – ‚ ıÂÚÂÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌËÂ. ᇷÓÎfl‚‡ÌÂÚÓ Â Ë‰ÂÌÚËÙˈË‡ÌÓ ‚ 18 ÓÚ 28 ӷ·ÒÚË Ì‡ ÒÚ‡Ì‡Ú‡, Ò‰ ‚Ò˘ÍË ÓÏÒÍË „ÛÔË ‚ Å˙΄‡Ëfl, ‚˙ÔÂÍË ˜Â ÔÓ-„ÓÎflχڇ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚ ÔË̇‰ÎÂÊ‡Ú Ì‡ „ÓÎflχڇ „ÛÔ‡ ̇ ÈÂÎËËÚÂ. éÅëöÜÑÄçÖ äÓÌ„ÂÌËÚ‡ÎÌËÚ ÏˇÒÚÂÌÌË ÒË̉ÓÏË Ò‡ ÍÎËÌ˘ÌÓ Ë „ÂÌÂÚ˘ÌÓ ıÂÚÂÓ„ÂÌ̇ „ÛÔ‡ ÓÚ Ì‡‚ÚÓËÏÛÌÌË Á‡·ÓÎfl‚‡ÌËfl, Á‡Òfl„‡˘Ë Ì‚ÓÏÛÒÍÛÎÌÓÚÓ Ô‰‡‚‡ÌÂ Ò ˜ÂÒÚÓÚ‡ ÔÓ-χÎÍÓ ÓÚ 1/500 000 (9,10). àÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ Ò˙‚ÂÏÂÌÌËÚ ‚˙ÁÏÓÊÌÓÒÚË Ì‡ ÂÎÂÍÚÓÌÌÓ-ÏËÍÓÒÍÓÔÒÍËÚÂ Ë in vitro ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl ‚ ÔÓÒΉÌËÚ „Ó‰ËÌË ÔÓÁ‚ÓÎË ‰‡ Ò ̇‚ÎÂÁ ‚ ‰˙ηӘË̇ ‚ Ô‡ÚÓ„ÂÌÂÚ˘ÌËÚ ÏÂı‡ÌËÁÏË Ì‡ ÍÓÌ„ÂÌËÚ‡ÎÌËÚ ÏˇÒÚÂÌÌË ÒË̉ÓÏË. ê‡Á΢‡‚‡Ú Ò ÔÂÒË̇ÔÚ˘ÌË, ÒË̇ÔÚ˘ÌË Ë ÔÓÒÚÒË̇ÔÚ˘ÌË äåë. ÑÓÒ„‡ Ò‡ ˉÂÌÚËÙˈË‡ÌË Ò‡ÏÓ ‰‚‡ ÔÂÒË̇ÔÚ˘ÌË äåë: ‡/ Ò ‰ÂÙÂÍÚ ‚ ÂÒËÌÚÂÁ‡Ú‡ ËÎË Ô‡ÍÂÚË‡ÌÂÚÓ Ì‡ ‡ˆÂÚËÎıÓÎË̇ (Ù‡ÏËÎ̇ ËÌÙ‡ÌÚËÎ̇ ÏˇÒÚÂÌËfl) Ë ·/ Ò ‡ÁÂʉ‡Ì ̇ ÒË̇ÔÚ˘ÌËÚ ‚ÂÁËÍÛÎË Ë Ì‡Ï‡ÎÂÌÓ Í‚‡ÌÚÓ‚Ó ÓÚ‰ÂÎflÌÂ. î‡ÏËÎ̇ڇ ËÌÙ‡ÌÚËÎ̇ ÏˇÒÚÂÌËfl Ò ‰˙ÎÊË Ì‡ ÏÛÚ‡ˆËË ‚ „Â̇ Á‡ ε-Ò۷‰ËÌˈ‡Ú‡ ‚ 17p13 ıÓÏÓÁÓχ. ëË̇ÔÚ˘ÌËÚ äåë Ò‡ Ò‚˙Á‡ÌË Ò ‡ˆÂÚËÎıÓÎËÌ-ÂÒÚÂ‡ÁÂÌ ‰ÂÙˈËÚ Ì‡ ÏÓÚÓ̇ڇ ÔÎӘ͇ Ë Ò ‰˙ÎÊ‡Ú Ì‡ ÏÛÚ‡ˆËË ‚ COLQ „Â̇ ‚ 3p24.2 ıÓÏÓÁÓχ èÓÒÚÒË̇ÔÚ˘ÌËÚ äåë Ò ӷÛÒ·‚flÚ „·‚ÌÓ ÓÚ ÍËÌÂÚ˘ÌË Ì‡Û¯ÂÌËfl ËÎË ‰ÂÙˈËÚ Ì‡ ‡ˆÂÚËÎıÓÎËÌÓ‚ËÚ ˆÂÔÚÓË. äËÌÂÚ˘ÌËÚ ̇Û¯ÂÌËfl ÔÓÌËʇ‚‡Ú ËÎË Ôӂ˯‡‚‡Ú ÓÚ„Ó‚Ó‡ Í˙Ï ‡ˆÂÚËÎıÓÎË̇ Ë Ô˘ËÌfl‚‡Ú ‡Á΢ÌË ·‡‚ÌÓ- Ë ·˙ÁÓ-͇̇ÎÌË äåë. í ҇ „ÂÌÂÚ˘ÌÓ Ë ÙËÁËÓÎӄ˘ÌÓ ıÂÚÂÓ„ÂÌÌË Ë Ò ‰˙ÎÊ‡Ú Ì‡ ÏÛÚ‡ˆËË ‚ „ÂÌËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ ÓÚ‰ÂÎÌËÚ Ò۷‰ËÌËˆË Ì‡ ‡ˆÂÚËÎıÓÎËÌÓ‚Ëfl ˆÂÔÚÓ: „ÂÌ Á‡ α-Ò۷‰ËÌˈ‡Ú‡ – 2q24-q32 ıÓÏÓÁÓχ; „ÂÌ Á‡ β-Ò۷‰ËÌˈ‡Ú‡ – 17p11-p12 ıÓÏÓÁÓχ; „ÂÌ Á‡ ε-Ò۷‰ËÌˈ‡Ú‡ – 17p13 ıÓÏÓÁÓχ. àÁ‚ÂÒÚÌË Ò‡


‰ÂÍÂÏ‚Ë, 2007

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

̇‰ 60 ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ ÚËÚ „Â̇. Ň‚ÌÓ-͇̇ÎÌËÚ ÏÛÚ‡ˆËË Û‰˙Îʇ‚‡Ú ÂÔËÁÓ‰ËÚ ̇ ÓÚ‚‡flÌ ̇ ͇̇·, Ë Ò‡ ÎÓ͇ÎËÁË‡ÌË ‚ å1 Ë å2 ‰ÓÏÂÌËÚ ̇ α-, β- Ë ε- Ò۷‰ËÌˈËÚ ̇ ‡ˆÂÚËÎıÓÎËÌÓ‚Ëfl ˆÂÔÚÓ. íÂÁË ÏÛÚ‡ˆËË Ò Û̇ÒΉfl‚‡Ú ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌÓ Ë Ô˘ËÌfl‚‡Ú Á‡„Û·‡ ̇ ÙÛÌ͈Ëfl. Å˙ÁÓ-͇̇ÎÌËÚ ÏÛÚ‡ˆËË ÒÍ˙Òfl‚‡Ú ÂÔËÁÓ‰ËÚ ̇ ÓÚ‚‡flÌ ̇ ͇̇· Ë ‚˙ÁÌËÍ‚‡Ú ̇ ÏflÒÚÓÚÓ Ì‡ Ò‚˙Á‚‡Ì ̇ ε- Ò۷‰ËÌˈ‡Ú‡. ÉÂÌ˙Ú, ÓÚ„Ó‚ÓÂÌ Á‡ ε-Ò۷‰ËÌˈ‡Ú‡ ̇ ‡ˆÂÚËÎıÓÎËÌÓ‚Ëfl ˆÂÔÚÓ,  ÓÒÌÓ‚ÂÌ ÙÓÍÛÒ Á‡ ÏÛÚ‡ˆËË, Ó·ÛÒ·‚fl˘Ë ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌË ÔÂÒË̇ÔÚ˘ÌË Ë ÔÓÒÚÒË̇ÔÚ˘ÌË äåë. éÔËÒ‡ÌË Ò‡ ̇‰ 30 ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ „Â̇ Á‡ ε-Ò۷‰ËÌˈ‡Ú‡. ó‡ÒÚ ÓÚ ÚÂÁË ÏÛÚ‡ˆËË ‚Ó‰flÚ ‰Ó Ù‡ÏËÎ̇ڇ ËÌÙ‡ÌÚËÎ̇ ÏˇÒÚÂÌËfl (äåë ÚËÔ I‡). Ñ‚Â Ú‡ÍË‚‡ ÏÛÚ‡ˆËË, Á‡Òfl„‡˘Ë ‰˙΄‡Ú‡ ËÌÚ‡ˆËÚÓÔ·ÁχÚ˘̇ ÔËÏ͇, Ò‡ ˉÂÌÚËÙˈË‡ÌË ÔË ÚË ÚÛÒÍË Ù‡ÏËÎËË (IVS10 +2T → G) Ë ÔË Â‰Ì‡ ËڇΡÌÒ͇ Ù‡ÏËÎËfl (εt411P). ÑÛ„Ë ÏÛÚ‡ˆËË ÔË äåë Ò‡: ε723DLC ‚ å1 ÂÍÒÚ‡ˆÂÎÛ·ÌËfl ‰ÓÏÂÌ, ÓÚÍËÚ‡ ÔË ÈÓ‰‡ÌÒ͇ Ù‡ÏËÎËfl, ε760ins8 ‚ å2 Ú‡ÌÒÏÂÏ·‡ÌÌËfl ‰ÓÏÂÌ Ë T51P ‚ ÂÍÒÚ‡ˆÂÎÛ·ÌËfl ‰ÓÏÂÌ, ÛÒÚ‡ÌÓ‚ÂÌË ÔË ÚÛÒÍË Ù‡ÏËÎËË. Middleton L. Ë Ò˙Ú. (10) ÓÚÍË‚‡Ú ÏÛÚ‡ˆËflÚ‡ ε1267delG ÔË 4 ˆË„‡ÌÒÍË Ù‡ÏËÎËË ÓÚ É˙ˆËfl Ë íÛˆËfl. Croxen Ë Ò˙Ú. (1998) (4) ˉÂÌÚËÙˈË‡Ú Ò˙˘‡Ú‡ ÏÛÚ‡ˆËfl ÔË 5 ÌÂÒ‚˙Á‡ÌË Ô‡ˆËÂÌÚË ÓÚ ‡Á΢ÌË „ÂÓ„‡ÙÒÍË ‡ÈÓÌË Í‡ÚÓ è‡ÍËÒÚ‡Ì, à̉Ëfl, Ö„ËÔÂÚ Ë É˙ˆËfl. í ӷÒ˙ʉ‡Ú ÙÛÌ͈ËÓ̇ÎÌËfl ÂÙÂÍÚ Ì‡ ÏÛÚ‡ˆËflÚ‡, ÎÓ͇ÎËÁË‡Ì‡ ‚ å3-å4 ˆËÚÓÔ·ÁχÚ˘̇ڇ ÔËÏ͇ Ë ÓÚÍË‚‡Ú, ˜Â Á‡„Û·‡Ú‡ ̇ å4 Ú‡ÌÒÏÂÏ·‡ÌÌËfl ‰ÓÏÂÌ ÏÓÊ ‰‡ Ëχ χÎ˙Í ÂÙÂÍÚ ‚˙ıÛ ÈÓÌÌËÚ ͇̇ÎË. íÓÁË Ù‡ÍÚ ËβÒÚË‡ ÏÂı‡ÌËÁ˙Ï Ì‡ ÏÓÎÂÍÛÎfl̇ ‚ÓβˆËfl ͇ÚÓ ÍÓÏ·ËÌË‡ÌÂÚÓ Ì‡ ÏÛÚ‡ˆËfl Ò˙Ò Á‡Ô‡Á‚‡ÌÂÚÓ Ì‡ ËÌÚÓÌ „ÂÌÂË‡ ÌÓ‚ ÙÛÌ͈ËÓ̇ÎÂÌ ÔÓÎËÔÂÔÚˉ. Abicht A. Ë Ò˙Ú. (1) Ò˙˘Ó ˉÂÌÚËÙˈË‡Ú ε1267dÂlG ÔË 13 Ô‡ˆËÂÌÚË Ò äåë ÓÚ 11 ÌÂÒ‚˙Á‡ÌË ˆË„‡ÌÒÍË Ù‡ÏËÎËË ÓÚ ‡Á΢ÌË ˛„ÓËÁÚÓ˜ÌÓ-‚ÓÔÂÈÒÍË ‰˙ʇ‚Ë. Ä‚ÚÓËÚ ÛÒÚ‡ÌÓ‚fl‚‡Ú Ú‡ÁË ÏÛÚ‡ˆËfl ÔË 31% ÓÚ Ù‡ÏËÎËËÚÂ Ò äåë ÚËÔ ¨‡ (ÔË 50% ÓÚ Ù‡ÏËÎËËÚÂ Ò ÌÂ-ÌÂÏÒÍË Ë ÔË ÌËÚÓ Â‰Ì‡ Ù‡ÏËÎËfl Ò ÌÂÏÒÍË ÂÚÌ˘ÂÌ ÔÓËÁıÓ‰). åÛÚ‡ˆËflÚ‡ ε1267dÂlG Á‡Òfl„‡ ‡ÈÓÌ ‚ ε-Ò۷‰ËÌˈ‡Ú‡, Ô‰‡ÁÔÓÎÓÊÂÌ Í˙Ï ÏÛÚ‡ˆËË. ÑÂΈËflÚ‡ „‡ÌË˜Ë Ò ËÌÚÓÌ 11 Ë ÂÍÁÓÌ 12 Ë ‚Ó‰Ë ‰Ó ̇Û¯ÂÌË ̇ ‡Ï͇ڇ ̇ ˜ÂÚÂÌ ÒΉ 422 ÍÓ‰ÓÌ, ÔÓÒΉ‚‡ÌÓ ÓÚ ÒÚÓÔ-ÍÓ‰ÓÌ. èÓ ÚÓ-

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ÁË Ì‡˜ËÌ ε1267dÂlG ‡ÁÛ¯‡‚‡ ˆËÚÓÔ·ÁχÚ˘̇ڇ ‚ÂË„‡ Ë ˜ÂÚ‚˙ÚËfl Ú‡ÌÒÏÂÏ·‡ÌÂÌ ‰ÓÏÂÌ (å4) ̇ ε-Ò۷‰ËÌˈ‡Ú‡. Ç˙ÁÏÓÊÌÓ Â Ò˙˘Ó ‰‡ ‚Ó‰Ë Ë ‰Ó ‰ÂÙÂÍÚÂÌ ÒÔ·ÈÒËÌ„. îÛÌ͈ËÓ̇ÎÌËÚ ÔÓÒΉÒÚ‚Ëfl ÓÚ ÏÛÚ‡ˆËflÚ‡ Ò‡: ÌÂÔ˙ÎÌÓ Ó„‡ÌËÁË‡Ì ̇ ˆÂÔÚÓÌËÚ Ò۷‰ËÌˈË, ‰ۈË‡Ì‡ ÂÍÒÔÂÒËfl ̇ ˆÂÔÚÓËÚÂ, Ò˙‰˙ʇ˘Ë ÏÛÚ‡ÌÚÌËÚ ε-Ò۷‰ËÌËˆË ËÎË ‡ÁÔ‡‰‡Ì ̇ ÌÂÒÚ‡·ËÎÌËÚ ˆÂÔÚÓË (1). äÎËÌ˘ÌËflÚ ÙÂÌÓÚËÔ Ì‡ ·˙΄‡ÒÍËÚ ˆË„‡ÌÒÍË Ù‡ÏËÎËË Ò ε1267dÂlG ÏÛÚ‡ˆËfl Ì Ò ‡Á΢‡‚‡ ÓÚ ÚÓÁË ÔË ÓÔËÒ‡ÌËÚ ‰ÓÒ„‡ Ù‡ÏËÎËË Ò Ú‡ÁË ÏÛÚ‡ˆËfl: ̇˜‡ÎÓ ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ ÒΉ ‡Ê‰‡ÌÂÚÓ, ÔÚÓÁ‡ Ë ‚˙̯̇ ÓÙÚ‡ÎÏÓÔ΄Ëfl, Ô‡ÚÓÎӄ˘̇ ·Ûη‡̇ Ë ÔÓÍÒËχÎ̇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ Ò ‰ÂÌÓÌÓ˘Ì‡ ‰Ë̇ÏË͇ Ë Á‡ÒË΂‡˘‡ Ò ÔË ÙËÁ˘ÂÒÍË ÛÒËÎËfl. åˇÒÚÂÌ̇ڇ ÒËÏÔÚÓχÚË͇  ̇ÈÚÂÊÍÓ ÔÓfl‚Â̇ ‚ Ô˙‚ËÚ „Ó‰ËÌË ÒΉ ‡Ê‰‡ÌÂÚÓ. ë˙Ò Ò˙Áfl‚‡ÌÂÚÓ Ì‡ÒÚ˙Ô‚‡ ÒÚ‡·ËÎËÁË‡Ì ‚ Ò˙ÒÚÓflÌËÂÚÓ Ì‡ ·ÓÎÌËÚÂ, ‡ ‚ ÔÓ-Í˙Ò̇ ‚˙Á‡ÒÚ ÌflÍÓË ÓÚ ÒËÏÔÚÓÏËÚ (·Ûη‡Î̇ Ë ÔÓÍÒËχÎ̇ ÏÛÒÍÛÎ̇ ÛÏÓflÂÏÓÒÚ) ËÁ·Î‰Ìfl‚‡Ú Ë ËÁ˜ÂÁ‚‡Ú. Ç˙̯̇ڇ ÓÙÚ‡ÎÏÓÔ΄Ëfl  ̇È-ÔÓÒÚÓflÌÌËfl ÒËÏÔÚÓÏ ÔË ÚÓ‚‡ Á‡·ÓÎfl‚‡ÌÂ. èË ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚ Ì Ò ÛÒÚ‡ÌÓ‚Ë Á‡Òfl„‡Ì ̇ ˆÂ‚Ë͇ÎÌËÚÂ, Ò͇ÔÛ·ÌËÚÂ Ë ÂÍÒÚÂÌÁÓÌËÚ ÏÛÒÍÛÎË Ì‡ Ô˙ÒÚËÚÂ, ÚËÔ˘ÌÓ Á‡ ·‡‚ÌÓ-͇̇ÎÌËÚ äåë (9). ìÒÚ‡ÌÓ‚fl‚‡Ú Ò ËÌÚÂ- Ë ËÌÚ‡-Ù‡ÏËÎÌË ‚‡ˇˆËË ‚ ÔÓÚ˘‡ÌÂÚÓ Ì‡ äåë, ÍÓËÚÓ Ì‡È-‚ÂÓflÚÌÓ Ò‡ Ò‚˙Á‡ÌË Ò ‰Û„Ë „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË ËÎË Ù‡ÍÚÓË Ì‡ Ò‰‡Ú‡. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Á̇˜ËÏË ‡Á΢Ëfl ‚˙‚ ÙÂÌÓÚËÔ‡ ̇ Ù‡ÏËÎÌËÚÂ Ë ÒÔÓ‡‰Ë˜ÌËÚ ÒÎÛ˜‡Ë Ò äåë ÚËÔ I‡, ͇ÍÚÓ Ë ‡Á΢Ëfl, Ò‚˙Á‡ÌË Ò ÔÓ·. ê‡Á„‡Ì˘‡‚‡ÌÂÚÓ Ì‡ äåë ÓÚ ÏˇÒÚÂÌËfl „‡‚ËÒ Ëχ Ô‡ÍÚ˘ÂÒÍÓ Á̇˜ÂÌËÂ, Ú˙È Í‡ÚÓ ÔË äåë Ì ‚ÎËÁ‡Ú ‚ Ò˙Ó·‡ÊÂÌË ÚËÏÂÍÚÓÏËflÚ‡ Ë ÍÓÚËÍÓÒÚÂÓˉ̇ڇ ÚÂ‡ÔËfl. è‡‚Ë ‚Ô˜‡ÚÎÂÌËÂ, ˜Â Á‡·ÓÎfl‚‡ÌÂÚÓ Â ¯ËÓÍÓ ‡ÁÔÓÒÚ‡ÌÂÌÓ Ò‰ ·˙΄‡ÒÍËÚ ÓÏË Ë Á‡Òfl„‡ Ë ‰‚ÂÚ ÓÒÌÓ‚ÌË ÓÏÒÍË „ÛÔË ‚ Å˙΄‡Ëfl – „ÛÔ‡Ú‡ ̇ ÈÂÎËËÚÂ Ë Ç·¯Í‡Ú‡ „ÛÔ‡ ÓÏË. èӂ‰ÂÌÓ Â Ï‡˘‡·ÌÓ „ÂÌÂÚ˘ÌÓ ÂÔˉÂÏËÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ̇ äåë ÚËÔ I‡. àÁÒΉ‚‡ÌË Ò‡ ̇‰ 800 Á‰‡‚Ë ËÌ‰Ë‚Ë‰Ë ÓÚ ‰ÂÒÂÚ ‡Á΢ÌË Ẩӄ‡ÏÌË ÓÏÒÍË „ÛÔË Á‡ ÌÓÒËÚÂÎÒÚ‚Ó Ì‡ „ÂÌÂÚ˘ÌËfl ‰ÂÙÂÍÚ. óÂÒÚÓÚ‡Ú‡ ̇ ÌÓÒËÚÂÎÒÚ‚ÓÚÓ ‚ ÓÚ‰ÂÎÌËÚ „ÛÔË ‚‡Ë‡ ÓÚ 0 % ‰Ó 7.94 %. íÂÁË ÂÁÛÎÚ‡ÚË ËÁÚ˙Í‚‡Ú ‚‡ÊÌÓÒÚÚ‡ ̇ ÓÔ‰ÂÎflÌÂÚÓ Ì‡ ÒÓˆËÓ-‡ÌÚÓÔÓÎӄ˘ÂÒ͇ڇ ı‡-

îË„. 1. 臈ËÂÌÚË Ò äåë ÚËÔ I‡


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

‰ÂÍÂÏ‚Ë, 2007

‡ÍÚÂËÒÚË͇ ̇ ÓÏÒ͇ڇ „ÛÔ‡ ÔË ÓÒ˙˘ÂÒÚ‚fl‚‡Ì ̇ ÒÂÎÂÍÚË‚ÌË „ÂÌÂÚ˘ÌË ÒÍËÌËÌ„Ó‚Ë ÔÓ„‡ÏË. äåë ÚËÔ ¨‡ Ò˙Ò Ò˙˘‡Ú‡ „ÂÌÂÚ˘̇ ÏÛÚ‡ˆËfl ε1267dÂlG  ÓÚÍËÚ Ë ÔË ˆË„‡ÌÒÍË Ù‡ÏËÎËË ÓÚ ‡Á΢ÌË ˛„ÓËÁÚÓ˜ÌË Â‚ÓÔÂÈÒÍË (É˙ˆËfl, íÛˆËfl, Å˙΄‡Ëfl, å‡Í‰ÓÌËfl, ë˙·Ëfl, äÓÒÓ‚Ó Ë ìÌ„‡Ëfl), Ë Á‡Ô‡‰ÌÓ-‚ÓÔÂÈÒÍË ‰˙ʇ‚Ë (ÉÂχÌËfl, î‡ÌˆËfl, àÒÔ‡ÌËfl), ͇ÍÚÓ Ë Ò‰ Ù‡ÏËÎËË ÓÚ è‡ÍËÒÚ‡Ì, à̉Ëfl Ë Ö„ËÔÂÚ. èӂ‰ÂÌËflÚ ı‡ÔÎÓÚËÔÂÌ ‡Ì‡ÎËÁ ÔÓ͇Á‚‡, ˜Â ÏÛÚ‡ˆËflÚ‡ Ëχ Ó·˘ Ô‡Ó‰ËÚÂÎ Ë Â ‚˙ÁÌËÍ̇· ÔÂ‰Ë ÓÍÓÎÓ 900-1000 „Ó‰ËÌË (32-40 ÔÓÍÓÎÂÌËfl) ÔÓ ‚ÂÏ ̇ ÙÓÏË‡ÌÂÚÓ Ì‡ ÔÓÚÓ-ˆË„‡ÌÒ͇ڇ ÔÓÔÛ·ˆËfl (13). ÑÌÂÒ Úfl  ‡ÁÔÓÒÚ‡ÌÂ̇, ͇ÍÚÓ Ò‰ ‡Á΢ÌË ÓÏÒÍË „ÛÔË ‚ ˆfl· Ö‚ÓÔ‡, ڇ͇ Ë Ò‰ ‡Á΢ÌË Ó·˘ÌÓÒÚË ‚ à̉Ëfl Ë è‡ÍËÒÚ‡Ì, ‡Á΢‡‚‡˘Ë Ò ÔÓ ÂÎË„Ëfl, ÂÁËÍ Ë Í‡ÒÚ‡. íÓ‚‡  ̇È-ÒËÎÌÓÚÓ ‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ Ë̉ËÈÒÍËfl ÔÓËÁıÓ‰ ̇ ÓÏËÚÂ Ë ˜Â ‰‡‚ÌÓÒÚÚ‡ ̇ ÏÛÚ‡ˆËflÚ‡ Ô‰¯ÂÒÚ‚‡ Úflı̇ڇ ÏË„‡ˆËfl ÓÚ ë‚ÂÓ-á‡Ô‡‰Ì‡ à̉Ëfl.

4.

5.

6. 7. 8. 9. 10. 11.

12. 11.

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2.

3.

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éË„Ë̇ÎÌË ÒÚ‡ÚËË óÖëíéíÄ çÄ ε4 ÄãÖã çÄ ÄèéãàèéèêéíÖàç Ö èêà ÅöãÉÄêëäà èÄñàÖçíà ë ÅéãÖëí çÄ ÄãñïÄâåÖê ã. í‡ÈÍÓ‚1, ò. åÂı‡·Ë‡Ì1, Ä. âÓ‰‡ÌÓ‚‡2, å. ê‡È˜Â‚‡1, å. èÂÚÓ‚‡1, ü. ÜÂ΂1, à. âÓ‰‡ÌÓ‚‡2, à. äÂÏÂÌÒÍË2 1 äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, åÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËfl 2ㇷÓ‡ÚÓËfl ÔÓ åÓÎÂÍÛÎfl̇ è‡ÚÓÎÓ„Ëfl, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËfl SUMMARY APOLIPOPROTEIN E α4 ALLELE FREQUENCY IN BULGARIAN PATIENTS WITH ALZHEIMER’S DISEASE. L. Traykov, S. Mehrabian, A. Jordanova, M. Raycheva, M. Petrova, Y. Jelev, I. Jordanova, I. Kremensky Department of Neurology, “Alexandrovska” Hospital Medical University, Sofia, Bulgaria Laboratory of Molecular Pathology, Medical University, Sofia, Bulgaria Mutations in three genes that increase the production of amyloid-beta (Aβ) peptides—amyloid precursor protein (APP), presenilin-1 (PSEN1), and presenilin-1 (PSEN2)— have been genetically linked to early-onset (<65 years old) familial Alzheimer’s disease (EOAD), which accounts for less than 5% of AD cases. Apolipoprotein E (ApoE)- ε4 has been genetically linked to late-onset (>65 years old) familial (LOAD) and sporadic AD, which account for more than 95% of AD cases, and it has a gene-dose effect on the risk and age at onset of the disease. The ApoE ε4 allele was shown to be a risk factor for AD, while the possible role of the epsilon2 allele in AD is controversial. The objective of this study was to compare the ApoE ε4 allele frequency in patients with AD (EOAD, LOAD) and NC.

Study patients were selected among subjects who sought consultation at the University Hospital “Alexandrovska” in Sofia, because of memory problems or other symptoms of cognitive deterioration. We investigated 68 patients with LOAD, 63 with EOAD and 43 normal controls (NC). ApoE phenotyping was performed in all subjects by an agarose isoelectric focusing immunoblot method. The ε4 allele frequency showed significant differences between LOAD and NC (χ2 =8.9; p<0.002). The ε4 frequency in the EOAD group did not differ significantly from that in NC. Patients containing at least one ε2 allele were more frequent among the LOAD (30.2%) than among NC (9.3 %) and EOAD (14.3%) groups. The ε2 allele frequency showed significant decrease in LOAD (χ 2=7.41; p<0.006), and EOAD (χ2=5.11; p<0.02) groups in comparison with NC. In conclusion, we have confirmed that the ApoE ε4 allele occurs frequently in late-onset AD, while we have found no similar association in patients with EOAD. The patients could with at least one ApoE ε4 allele could be at risk of developing AD by an ε4 -dependent pathway. The findings should contribute to the assessment of dementia risk profile in the elderly. The significant decrease of ε2 allele frequency in the AD group supports the hypothesis of a possible protective role of ε2 allele in AD. KEY WORDS: LOAD, EOAD, Apolipoprotein E.


‰ÂÍÂÏ‚Ë, 2007

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

êÖáûåÖ åÛÚ‡ˆËËÚ ‚ ÚË „Â̇, ÍÓËÚÓ Û‚Â΢‡‚‡Ú ÔÓ‰Û͈ËflÚ‡ ̇ βA4 ÔÓÚÂËÌ, Amyloid Precursor Protein (APP) Presenilin 1 (PSEN1) Ë Presenilin 2 (PSEN2) Ò‡ ‡ÒÓˆËË‡ÌË Ò Ù‡ÏËÎ̇ ·ÓÎÂÒÚ Ì‡ ÄΈı‡ÈÏÂ (ÅÄ) Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (<65 „.). íÂÁË ÏÛÚ‡ˆËË Ò‡ Ô˘Ë̇ Á‡ ÓÍÓÎÓ 5% ÓÚ ‚Ò˘ÍË ÒÎÛ˜‡Ë ̇ ÅÄ. î‡ÏËÎÌËÚÂ Ë ÒÔÓ‡‰Ë˜ÌËÚ ÙÓÏË Ì‡ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (>65 „.), ÓÍÓÎÓ 95% ÓÚ ÒÎÛ˜‡ËÚÂ, Ò‡ „ÂÌÂÚ˘ÌÓ Ò‚˙Á‡ÌË Ò Ì‡Î˘ËÂÚÓ Ì‡ ε4 ‡ÎÂΠ̇ ÄÔÓÎËÔÓÔÓÚÂËÌ Ö (ÄÔÓÖ). AÔÓE-ε4 ‡ÎÂÎ˙Ú Ëχ ‰ÓÁÓ‚ ÂÙÂÍÚ ‚˙ıÛ ËÒ͇ ÓÚ ‡Á‚ËÚË ̇ ÅÄ Ë ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ‰Ó͇ÚÓ ËÁÒΉ‚‡ÌËflÚ‡ Á‡ ÓÎflÚ‡ ̇ AÔÓE ε2 ‡Î· ÔÓ͇Á‚‡Ú ÔÓÚË‚ÓÂ˜Ë‚Ë ÂÁÛÎÚ‡ÚË. ñÂÎÚ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ Â ËÁÒΉ‚‡Ì ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ‡ÎÂÎËÚ ̇ ÄÔÓÖ ÔË Ô‡ˆËÂÌÚË Ò ÅÄ (Ò ‡ÌÌÓ Ë Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ) Ë ÔË Á‰‡‚Ë ‚˙Á‡ÒÚÌË Îˈ‡ ·ÂÁ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ‚ Å˙΄‡Ëfl. àÁÒΉ‚‡ÌËÚ ·ÓÎÌË Ò‡ ÔÓ‰·‡ÌË Ò‰ Îˈ‡Ú‡ ÍÓÌÒÛÎÚË‡ÌË ‚ ·ÓÎÌˈ‡ ◊ÄÎÂÍ҇̉Ó‚Ò͇” (ëÓÙËfl, Å˙΄‡Ëfl) ÔÓ ÔÓ‚Ó‰ Ô‡ÏÂÚÓ‚Ë ÔÓ·ÎÂÏË ËÎË ‰Û„Ë ÒËÏÔÚÓÏË Ì‡ ÍÓ„ÌËÚË‚ÌÓ Ì‡Û¯ÂÌËÂ. àÁÒΉ‚‡ÌË Ò‡ 68 Ô‡ˆËÂÌÚË Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ, 63 Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë 43 ÍÎËÌ˘ÌÓ Á‰‡‚Ë Îˈ‡. AÔoE ÙÂÌÓÚËÔ˙Ú Â ÓÔ‰ÂÎflÌ ˜ÂÁ ÏÂÚÓ‰‡ ̇ ËÁÓÂÎÂÍÚÓÙÓ͇ÎËÁ‡ˆËfl ‚˙ıÛ Í˙‚̇ Ô·Áχ. èÓÛ˜‚‡ÌÂÚÓ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ ε4 ‡Î· ÔÓ͇Á‚‡ Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ Ë Á‰‡‚ËÚ ÍÓÌÚÓÎË (χ2=8.9; p<0.002). óÂÒÚÓÚ‡Ú‡ ̇ ε4 ‡Î· ‚ „ÛÔ‡Ú‡ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ì Ò ‡Á΢‡‚‡ Á̇˜ËÏÓ ÓÚ Ú‡ÁË ‚ „ÛÔ‡Ú‡ Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË. ãˈ‡Ú‡, ÌÓÒËÚÂÎË Ì‡ ÔÓÌ ‰ËÌ ε4 ‡ÎÂÎ, Ò Ò¢‡Ú Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚÓ Ò‰ ·ÓÎÌËÚ ÒÚ‡‰‡˘Ë ÓÚ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (30.2 %), ÓÚÍÓÎÍÓÚÓ Ò‰ ·ÓÎÌËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (14.3%) Ë Á‰‡‚ËÚ Îˈ‡ (9.3 %). óÂÒÚÓÚ‡Ú‡ ̇ ε2 ‡Î·  Á̇˜ËÚÂÎÌÓ ÔÓ-ÌËÒ͇ ‚ „ÛÔ‡Ú‡ ̇ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (χ2=7.41; p<0.006) Ë ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (χ2=5.11; p<0.02) ‚ Ò‡‚ÌÂÌË Ò˙Ò Á‰‡‚ËÚ ÍÓÌÚÓÎË. Ç Á‡Íβ˜ÂÌËÂ, ÌË ÔÓÚ‚˙ʉ‡‚‡ÏÂ, ˜Â ε4 ‡Î· ̇ ÄÔÓÖ Ò Ò¢‡ Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚÓ ÔË ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ, ‰Ó͇ÚÓ Ú‡Í‡‚‡ Á‡‚ËÒËÏÓÒÚ Ì Ò ̇·Î˛‰‡‚‡ ÔË ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ. èË Ô‡ˆËÂÌÚË ÌÓÒËÚÂÎË Ì‡ ÔÓÌ ‰ËÌ ε4 ‡ÎÂÎ Ò Ôӂ˯‡‚‡ Á̇˜ËÏÓ ËÒ͇ ÓÚ ‡Á‚ËÚËÂÚÓ Ì‡ ÅÄ. íÂÁË ÂÁÛÎÚ‡ÚË ·Ëı‡ ÏÓ„ÎË ‰‡ ‰ÓÔËÌÂÒ‡Ú Á‡ ÓˆÂÌ͇ڇ ̇ ÔÓÙË· ̇ ËÒ͇ Á‡ ÔÓfl‚‡ ̇ ‰ÂÏÂ̈Ëfl ÔË ‚˙Á‡ÒÚÌË Îˈ‡. ëË„ÌËÙË͇ÌÚÌÓÚÓ ÒÌËÊÂÌË ̇ ε2 ‡Î· ‚ „ÛÔËÚ ̇ ÅÄ Ò Í˙ÒÌÓ Ë ‡ÌÌÓ Ì‡˜‡ÎÓ ÔÓ‰ÍÂÔfl ıËÔÓÚÂÁ‡Ú‡ Á‡ ÔÓÚÂÍÚ˂̇ڇ ÏÛ ÓÎfl ÔË ‡Á‚ËÚË ̇ ÅÄ. äãûóéÇà Ñìåà: ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ, ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ,ÄÔÓÎËÔÓÔÓÔÓÚÂËÌ Ö. ÇöÇÖÑÖçàÖ ÅÓÎÂÒÚÚ‡ ̇ ÄΈı‡ÈÏÂ (ÅÄ)  ÔÓ„ÂÒË‡˘Ó Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ Ò ÍÓÏÔÎÂÍÒ̇ ÂÚËÓÎÓ„Ëfl. ÉÂÌÂÚ˘ÌË Ù‡ÍÚÓË Ë Ù‡ÍÚÓË ÓÚ ÓÍÓÎ̇ڇ Ò‰‡ Ë„‡flÚ ‚‡Ê̇ ÓÎfl Á‡ ÂÍÒÔÂÒËflÚ‡ ̇ ÙÂÌÓÚËÔ‡. ÅÄ Â ÓÒÌӂ̇ Ô˘Ë̇ Á‡ ‰ÂÏÂ̈Ëfl Ë ÏÓÊ ‰‡ Ò ÔÓfl‚Ë Í‡ÚÓ Ù‡ÏËÎ̇ Ë ÒÔÓ‡‰Ë˜Ì‡ ÙÓχ (14). éÚ 1991 „Ó‰Ë̇ ÂÁÛÎÚ‡ÚËÚ ÓÚ „ÂÌÂÚ˘ÌËÚ ÔÓÛ˜‚‡ÌËfl Ò‡ ‰Ó‚ÂÎË ‰Ó ˉÂÌÚËÙˈË‡Ì ̇ ÏÛÚ‡ˆËË Ë ÔÓÎËÏÓÙËÁÏË, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ Ô˘ËÌflÚ ÅÄ ËÎË ‰‡ Û‚Â΢‡Ú ËÒ͇ ÓÚ ‡Á‚ËÚË ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ (31). åÛÚ‡ˆËË ‚ ÚË „Â̇: „ÂÌ Á‡ ÒËÌÚÂÁ‡ ̇ Ämyloid êrecursor êrotein (APP, Goate, et al., 1991), Presenilin 1 (PSEN1, Sherrington, et al., 1995) Ë Presenilin 2 (PSEN2, Levy – L‡had, et al., 1995a) Á‡ ÏÓÏÂÌÚ‡ Ò‡ ‡ÒÓˆËË‡ÌË Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ. íÂÁË ÏÛÚ‡ˆËË Ò‡ Ô˘Ë̇ Á‡ 30-50% ÓÚ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË̇ÌÚÌËÚ ÒÎÛ˜‡Ë Ë ÓÍÓÎÓ 5% ÓÚ ‚Ò˘ÍË ÒÎÛ˜‡Ë Ò ÅÄ.

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èË Ô‡ˆËÂÌÚË Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ Ì ҇ ÓÚÍËÚË ÏÛÚ‡ˆËË ‚ Äêê, PSEN1 Ë PSEN2 „ÂÌËÚÂ. òËÓÍÓχ˘‡·ÂÌ „ÂÌÓÏÂÌ ‡Ì‡ÎËÁ ‚ „ÓÎÂÏË ÒÂÏÂÈÒÚ‚‡ Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ ÓÚÍË‚‡ ‰‡ÌÌË Á‡ Ò͇˜ÂÌÓÒÚ Ò ıÓÏÓÁÓÏÂÌ Û˜‡ÒÚ˙Í 19q13.2 (18). èÓ Ò˙˘ÓÚÓ ‚ÂÏ Ì‚Ó·ËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ‚ Ô·ÍËÚ ̇ Ô‡ˆËÂÌÚË Ò ÅÄ Ëχ ‚ËÒÓÍË ÍË̈ÂÌÚ‡ˆËË Ì‡ ÄÔÓÎËÔÓÔÓÚÂËÌ Ö (ÄÔoE), ÍÓ‰Ë‡Ì ÓÚ AÔÓE „Â̇ (17). Ç „˙·Ì‡˜ÌÓ-ÏÓÁ˙˜Ì‡Ú‡ Ú˜ÌÓÒÚ ÄÔoE Ò ҂˙Á‚‡ Ò ‚ËÒÓÍ ‡ÙËÌËÚÂÚ Í˙Ï ËÏÓ·ËÎËÁË‡Ì β-‡ÏËÎÓˉ (29). íÂÁË ‰‚‡ ‚ˉ‡ ËÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â AÔÓE  ÙÛÌ͈ËÓ̇ÎÂÌ Í‡Ì‰Ë‰‡Ú-„ÂÌ Á‡ ÅÄ, ÍÓÈÚÓ Ò ͇ÚË‡ ‚ 19q13-‡ÈÓ̇. Ç ÍÓ‰Ë‡˘‡Ú‡ ӷ·ÒÚ Ì‡ ÚÓÁË „ÂÌ Ò‡ ÓÚÍËÚË ‰‚‡ „ÂÌÂÚ˘ÌË ÔÓÎËÏÓÙËÁχ ‚ ‡ÏËÌÓÍËÒÂÎËÌÌË ÔÓÁˈËË 112 (Cys) Ë 158 (Arg). í ‚Ó‰flÚ ‰Ó ÚË ÓÒÌÓ‚ÌË ·ÂÎÚ˙˜ÌË ËÁÓÙÓÏË. AÔÓE-ε3 (Cys112Arg158)  ̇ȘÂÒÚ‡Ú‡ ËÁÓÙÓχ, Ò ‡ÎÂÎ̇ ˜ÂÒÚÓÚ‡ 75% ‚ 䇂͇Á͇ڇ ÔÓÔÛ·ˆËfl. ÑÛ„ËÚ ‰‚‡ ˜ÂÒÚË ‡Î· Ò‡ AÔÓE-ε2 (Cys112Cys158) Ë AÔÓE-ε4 (Arg112Arg158) Ò ˜ÂÒÚÓÚË Ò˙ÓÚ‚ÂÚÌÓ 10% Ë 15%. ÉÂÌÂÚ˘̇ ‡ÒӈˇˆËfl  ÓÚÍËÚ‡ ÏÂÊ‰Û AÔÓE-ε4 ‡Î· Ë Ù‡ÏËÎÌË Ë ÒÔÓ‡‰Ë˜ÌË ÒÎÛ˜‡Ë Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (23, 32). çflÍÓÎÍÓ ÌÂÁ‡‚ËÒËÏË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â AÔÓE-ε4 ‡Î· Ëχ ‰ÓÁÓ‚ ÂÙÂÍÚ ‚˙ıÛ ËÒ͇ ÓÚ ‡Á‚ËÚË ̇ ÅÄ, ‰Ó͇ÚÓ AÔÓE-ε2 ‡Î· Ëχ ÔÓÚÂÍÚË‚ÂÌ ÂÙÂÍÚ (7, 8, 30). ç‡Î˘ËÂÚÓ Ì‡ ÄÔÓÖ e4 Ëχ ‰ËÂÍÚ̇ ‚˙Á͇ Ë Ò Ì‡Û¯ÂÌËfl ‚ ıÓÎËÌÂ„˘̇ڇ ωˇˆËfl ‚ ÚÂÏÔÓ‡ÎÌËfl ‰flÎ (20), Ò ÔÓ-ÚÂÊÍË ‡ÚÓÙ˘ÌË ÔÓÏÂÌË ‚ ıËÔÓ͇ÏÔ‡ Ë ÂÌÚÓˇÎÌËfl ÍÓÚÂÍÒ Ë ÔÓ-ÚÂÊÍË Ì‡Û¯ÂÌËfl ̇ ÂÔËÁӉ˘̇ڇ Ô‡ÏÂÚ. åÛÎÚˈÂÌÚÓ‚Ó ÔÓÛ˜‚‡ÌÂ, Ôӂ‰ÂÌÓ ÔÂÁ 1997„., ÔÓ͇Á‚‡ ˜Â ÔË ıÂÚÂÓÁË„ÓÚÌËÚ ÌÓÒËÚÂÎË ÓÚ ä‡‚Í‡Á͇ڇ ‡Ò‡ ̇ ‰ËÌ AÔÓE-ε4 ‡ÎÂÎ Ëχ ÚË Ô˙ÚË ÔÓ-‚ËÒÓÍ ËÒÍ, ‡ ÔË ıÓÏÓÁË„ÓÚÌËÚ ÌÓÒËÚÂÎË ÓÚ Ò˙˘‡Ú‡ ‡Ò‡ – 8 Ô˙ÚË ÔÓ-‚ËÒÓÍ ËÒÍ Á‡ ‡Á‚ËÚË ̇ ÅÄ (6). AÔÓE-ε4 ‡ÎÂÎ Â ‡ÒÓˆËË‡Ì Ò ÔÓ-‡Ì̇ڇ ‚˙Á‡ÒÚ Ì‡ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. ç‡Î˘ËÂÚÓ Ì‡ ‚ÒÂÍË ‡ÎÂÎ Ô‰ËÁ‚ËÍ‚‡ ̇χÎÂÌË ̇ ‚˙Á‡ÒÚÚ‡ ̇ ‰Â·˛Ú‡ Ò ÓÍÓÎÓ 5 „Ó‰ËÌË. í‡ÁË ‚˙Á͇ ̇ ÄÔÓÖ ÔÓÎËÏÓÙËÁÏËÚÂ Ò ÅÄ Â Á̇˜Ëχ Ò‡ÏÓ ‚ ͇‚͇Á͇ڇ ÔÓÔÛ·ˆËfl. èË ˜ÂÌÓÍÓÊËÚ ڇ͇‚‡ Á‡‚ËÒËÏÓÒÚ Ì  ÛÒÚ‡ÌÓ‚Â̇ (12). ÇÒ Ӣ ÎËÔÒ‚‡Ú ‰‡ÌÌË Á‡ ‡ÒӈˇˆËflÚ‡ ̇ ε4 ‡Î· Ò ÅÄ Ò‰ ÔÓÔÛ·ˆËfl ÓÚ ·˙΄‡ÒÍË Ô‡ˆËÂÌÚË, ‚˙ÔÂÍË ÏÌÓÊÂÒÚ‚ÓÚÓ Ôӂ‰ÂÌË ÔÓÛ˜‚‡ÌËfl ‚ ‡Á΢ÌË Â‚ÓÔÂÈÒÍË ÒÚ‡ÌË. ñÂÎÚ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ Â ËÁÒΉ‚‡Ì ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÙÂÌÓÚËÔÓ‚ÂÚÂ Ë ‡ÎÂÎËÚ ̇ ÄÔÓÖ ÔË Ô‡ˆËÂÌÚË Ò ÅÄ (Ò ‡ÌÌÓ Ë Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ) Ë ÔË Á‰‡‚Ë ‚˙Á‡ÒÚÌË Îˈ‡ ·ÂÁ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl ‚ Å˙΄‡Ëfl. äéçíàçÉÖçí à åÖíéÑà èÓÛ˜‚‡ÌÂÚÓ Â Ôӂ‰ÂÌÓ ‚ ·ÓÎÌˈ‡ ◊ÄÎÂÍ҇̉Ó‚Ò͇” (ëÓÙËfl, Å˙΄‡Ëfl) ÔË 68 ‰ÂÏÂÌÚÌÓ ·ÓÎÌË ÒÚ‡‰‡˘Ë ÓÚ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ, 63 ·ÓÎÌË Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÔË 43 ÍÎËÌ˘ÌÓ Á‰‡‚Ë Îˈ‡. Ñˇ„ÌÓÁ‡Ú‡  ÔÓÒÚ‡‚fl̇ ‚˙Á ÓÒÌÓ‚‡ ̇ ÔÓ‰ӷ̇ ‡Ì‡ÏÌÂÁ‡, ÒÓχÚ˘ÂÌ Ë Ì‚ÓÎӄ˘ÂÌ Ô„Ή, ··Ó‡ÚÓÌË ËÁÒΉ‚‡ÌËfl Ë ÍÓÏÔ˛Ú˙̇ ÚÓÏÓ„‡ÙËfl (CT) Ë/ËÎË Ï‡„ÌËÚÌÓ ÂÁÓ̇ÌÒ̇ ÚÓÏÓ„‡ÙËfl (åêí) ̇ ÏÓÁ˙͇. Ñˇ„ÌÓÒÚ˘̇ڇ Ôӈ‰Û‡  ÓÔË҇̇ ÔÓ‰Ó·ÌÓ ‚ Ô‰˯ÌË Ì‡¯Ë ÔÓÛ˜‚‡ÌËfl (1, 2). ÅÓÎÌËÚ ÒÚ‡‰‡˘Ë ÓÚ ÅÄ Ò‡ ‰Ë‡„ÌÓÒÚˈË‡ÌË Ì‡ ·‡Á‡Ú‡ ̇ DSM-IV (3) ÍËÚÂËËÚ Á‡ "ÑÂÏÂ̈Ëfl ÓÚ ÄΈı‡ÈÏÂÓ‚ ÚËÔ" Ë NINCDS/ADRDA (16) ÍËÚÂËËÚ Á‡ ‚ÂÓflÚ̇ ·ÓÎÂÒÚ Ì‡ ÄΈı‡ÈÏÂ. чÌÌËÚ Á‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÄÔÓÖ ‡ÎÂÎËÚ ‚ ‰‚ÂÚ „ÛÔË ·ÓÎÌË Ò‡ Ò‡‚Ìfl‚‡ÌË Ò ÚÂÁË Ì‡ ÍÓÌÚÓÎ̇ „ÛÔ‡ Ò˙ÒÚ‡‚Â̇ ÓÚ ÍÎËÌ˘ÌÓ Á‰‡‚Ë Îˈ‡, ÍÓËÚÓ ÓÚ„Ó‚‡flÚ ÔÓ ÔÓÎ,


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

‰ÂÍÂÏ‚Ë, 2007

‚˙Á‡ÒÚ Ë Ó·‡ÁÓ‚‡ÚÂÎÂÌ ˆÂÌÁ ̇ Ô‡ˆËÂÌÚËÚÂ. í Ì Ò˙Ó·˘‡‚‡Ú ‰‡ÌÌË, ÌËÚÓ ËÏ‡Ú ÒËÏÔÚÓÏË Á‡ ͇͂ÓÚÓ Ë ‰‡  Ì‚ÓÎӄ˘ÌÓ ËÎË ÔÒËıˇÚ˘ÌÓ Á‡·ÓÎfl‚‡ÌÂ Ë Ì‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì Ì ÔÓ͇Á‚‡ ̇Û¯ÂÌË ̇ ÍÓ„ÌËÚË‚ÌËÚ ËÏ ÙÛÌ͈ËË. AÔoE ÙÂÌÓÚËÔ˙Ú Â ÓÔ‰ÂÎflÌ ˜ÂÁ ÏÂÚÓ‰‡ ̇ ËÁÓÂÎÂÍÚÓÙÓ͇ÎËÁ‡ˆËfl ‚˙ıÛ Í˙‚̇ Ô·Áχ (4). ᇠˆÂÎÚ‡ ̇ ÔÓÛ˜‚‡ÌÂÚÓ Ò ÔËÂχ, ˜Â ÙÂÌÓÚËÔ˙Ú ÓÚ‡Áfl‚‡ „ÂÌÓÚËÔ‡. éÔ‰ÂÎflÌÂÚÓ Ì‡ ÙÂÌÓÚËÔ‡  ËÁ‚˙¯‚‡ÌÓ Ì‡ ÒÎflÔÓ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÍÎËÌ˘̇ڇ ‰Ë‡„ÌÓÁ‡ Ë Ó·‡ÚÌÓÚÓ. ê‡ÁÎËÍËÚ ÏÂÊ‰Û „ÛÔËÚ ‚˙‚ ‚˙Á‡ÒÚÚ‡ Ë ÂÁÛÎÚ‡ÚËÚ ÓÚ MMS Ò‡ ÓˆÂÌfl‚‡ÌË Ò ÔÓÏÓ˘Ú‡ ̇ ANOVA (oneway ‚‡ˇˆËÓÌÂÌ ‡Ì‡ÎËÁ). èË Ì‡Î˘Ë ̇ Á̇˜ËÏ ÂÙÂÍÚ ÔË ANOVA, ‡ÁÎËÍËÚ ÏÂÊ‰Û „ÛÔËÚ ҇ ‡Ì‡ÎËÁË‡ÌË post-hoc ‰‚ ÔÓ ‰‚ Ò˙Ò Student-Newman-Keuls ÚÂÒÚ (p<0.05). óÂÒÚÓÚ‡Ú‡ ̇ ‡ÎÂÎËÚ  ÓÔ‰ÂÎfl̇ ˜ÂÁ ‡Ì‡ÎËÁ ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÙÂÌÓÚËÔÓ‚ÂÚÂ. íflıÌÓÚÓ ‡ÁÔ‰ÂÎÂÌË  ӈÂÌfl‚‡ÌÓ ˜ÂÁ Pearsons χ2 ÚÂÒÚ Ë Fisher's exact ÚÂÒÚ ÔË ÌÂÓ·ıÓ‰ËÏÓÒÚ êÖáìãíÄíà ï‡‡ÍÚÂËÒÚËÍËÚ ̇ ‚Íβ˜ÂÌËÚ „ÛÔË Ò‡ Ô‰ÒÚ‡‚ÂÌË Ì‡ Ú‡·Îˈ‡ 1. ëÚ‡ÚËÒÚ˘ÂÒÍËflÚ ‡Ì‡ÎËÁ ÔÓ͇Á‚‡ Á̇˜Ëχ ‡ÁÎË͇ Á‡ T‡·Îˈ‡ 1: ï‡‡ÍÚÂËÒÚËÍË Ì‡ ‚Íβ˜ÂÌËÚ „ÛÔË ï‡‡ÍÚÂËÒÚËÍË ÅÄ Ò Í˙ÒÌÓ ÅÄ Ò ‡ÌÌÓ äÓÌÚÓÎË Ì‡˜‡ÎÓ (n=68) ̇˜‡ÎÓ (n=63) (n=43) èÓÎ (Ü/M) 43/25 32/31 32/11 Ç˙Á‡ÒÚ 74.50 (4.4) 61.18 (4.1) 64.32 (8.9) MMS/30 18.27 (5.8) 19 (4.8) 29.12 (0.8) ÅA = ÅÓÎÂÒÚ Ì‡ ÄΈı‡ÈÏÂ; MMS = Mini Mental State; чÌÌËÚ ҇ Ò‰ÌË ‡ËÚÏÂÚ˘ÌË (±SD). MMS ÏÂÊ‰Û ·ÓÎÌËÚÂ Ë Á‰‡‚ËÚ ÍÓÌÚÓÎË, ÌÓ Ì ÏÂÊ‰Û ‰‚ÂÚ „ÛÔË ·ÓÎÌË. èÓ ÓÚÌÓ¯ÂÌË ̇ ‚˙Á‡ÒÚÚ‡ Ò ÓÚ˜ËÚ‡ Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û Ô‡ˆËÂÌÚËÚÂ Ò ÅÄ Ò Í˙‰ÌÓ Ì‡˜‡ÎÓ Ë ÓÒڇ̇ÎËÚ ‰‚ „ÛÔË. ê‡ÁÔ‰ÂÎÂÌËÂÚÓ Ì‡ ÙÂÌÓÚËÔÓ‚ÂÚÂ Ë ‡ÎÂÎËÚ ̇ ÄÔÓÖ ÔË ·ÓÎÌËÚÂ Ë ÔË Á‰‡‚ËÚ Îˈ‡  Ô‰ÒÚ‡‚ÂÌÓ Ì‡ Ú‡·Îˈ‡ 2. T‡·Îˈ‡ 2: óÂÒÚÓÚ‡ ̇ ‡ÎÂÎËÚÂ Ë ÙÂÌÓÚËÔÓ‚ÂÚ ̇ ÄÔÓÖ ï‡‡ÍÚÂËÒÚËÍË ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (n=68) E2/E2 n (%) 0 E2/E3 n (%) 3 (4.4) E3/E3 n (%) 30 (44.1) E3/E4 n (%) 29 (42.6) E4/E2 n (%) 0 E4/E4 n (%) 6(8.8) ÄÎÂÎË ε2 n (%) 3 (2.2) ε3n (%) 92(67.7) ε4 n (%) 41(30.2) ÅA = ÅÓÎÂÒÚ Ì‡ ÄΈı‡ÈÏÂ;

ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (n=63) 0 4 (6.3) 47(74.6) 10 (15.9) 0 2(3.2)

äÓÌÚÓÎË (n=43) 0 8(18.6) 27 (62.8) 6 (13.9) 2 (4.7) 0

4 (3.2) 104 (82.5) 18 (14.3)

10 (11.3) 68(79.1) 8 (9.3)

èÓÛ˜‚‡ÌÂÚÓ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ ε4 ‡Î· ÔÓ͇Á‚‡ Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ Ë „ÛÔ‡Ú‡ ̇ ·ÓÎÌËÚÂ Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (χ2=5.67; p<0.01), ‰Ó͇ÚÓ Ú‡Í‡‚‡ Á̇˜ËÏÓÒÚ Ì Ò ̇·Î˛‰‡‚‡ ‚ „ÛÔ‡Ú‡ ̇ ·ÓÎÌËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ (χ2 =0.002; p<0.9). ãˈ‡Ú‡, ÌÓÒËÚÂÎË Ì‡ ÔÓÌ ‰ËÌ ε4 ‡ÎÂÎ, Ò Ò¢‡Ú Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚÓ ‚Ò‰ ·ÓÎÌËÚ ÒÚ‡‰‡˘Ë ÓÚ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (51.5%), ÓÚÍÓÎÍÓÚÓ ÔË ·ÓÎÌËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (19%) Ë Ò‰ Á‰‡‚ËÚ Îˈ‡ (18.6%). îÂÌÓÚËÔ˙Ú

247

E4/E4 Ò Ò¢‡ ÔÓ-˜ÂÒÚÓ ÔË ·ÓÎÌËÚÂ Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (6 ÓÚ 68 ·ÓÎÌË; 8.8%), ÓÚÍÓÎÍÓÚÓ ÔË ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (2 oÚ 63 ·ÓÎÌË; 3.2%) Ë ÔË Á‰‡‚ËÚ Îˈ‡ (0 oÚ 43). èÓÛ˜‚‡ÌÂÚÓ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ ε2 ‡Î· ÔÓ͇Á‚‡ Á̇˜Ëχ ‡ÁÎË͇ ÏÂÊ‰Û ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ Ë „ÛÔ‡Ú‡ ̇ ·ÓÎÌËÚÂ Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (χ2=7.41; p<0.006), Ë ÏÂÊ‰Û ÍÓÌÚÓÎ̇ڇ „ÛÔ‡ Ë „ÛÔ‡Ú‡ ̇ ·ÓÎÌËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (χ2=5.11; p<0.02). í‡Í‡‚‡ ‡ÁÎË͇ Ì Ò ̇·Î˛‰‡‚‡ ÏÂÊ‰Û Ô‡ˆËÂÌÚËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÚÂÁË Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (χ2 =0.02; p<0.63). ãˈ‡Ú‡, ÌÓÒËÚÂÎË Ì‡ ÔÓÌ ‰ËÌ ε2 ‡ÎÂÎ, Ò Ò¢‡Ú Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚÓ Ò‰ Á‰‡‚ËÚ Îˈ‡ (23.3%), ÓÚÍÓÎÍÓÚÓ ÔË ·ÓÎÌËÚ ÒÚ‡‰‡˘Ë ÓÚ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (4.8%) Ë ·ÓÎÌËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (6.3%). ë ˆÂÎ ËÁflÒÌfl‚‡Ì ̇ ÓÎflÚ‡ ̇ ÄÔÓ Ö- ε4 ‚˙ıÛ ‚˙Á‡ÒÚÚ‡ ̇ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ‚˙‚ ‚Òfl͇ „ÛÔ‡ ÔÓÛ˜‚‡Ï Ò‰̇ڇ ‚˙Á‡ÒÚ Ì‡ ‰Â·˛Ú��� ̇ ·ÓÎÂÒÚÚ‡ ÔË Ô‡ˆËÂÌÚË Ò ÔÓÌ ‰ËÌ ε4- ‡ÎÂÎ Ë Ô‡ˆËÂÌÚË ·ÂÁ ε4- ‡ÎÂÎ ‚˙‚ ‚Òfl͇ ‰̇ ÓÚ ‰‚ÂÚ „ÛÔË ·ÓÎÌË. èË ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ Ò‰̇ڇ ‚˙Á‡ÒÚ Ì‡ Ô‡ˆËÂÌÚËÚ ·ÂÁ ε4- ‡ÎÂÎ Â 71.3 „., ÔË ·ÓÎÌË Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ Ò ÔÓÌ ‰ËÌ ε4- ‡ÎÂÎ Â 72.5 „; ÔË ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ ‰‡ÌÌËÚ ҇ Ò˙ÓÚ‚ÂÚÌÓ 59.3 „. Ë 58.1 „. íÂÁË ÂÁÛÎÚ‡ÚË Ì ÔÓ͇Á‚‡Ú Á̇˜Ëχ ‡ÁÎË͇ ‚ ‰‚ÂÚ „ÛÔË. éÅëöÜÑÄçÖ óÂÒÚÓÚ‡Ú‡ ̇ ÄÔÓ ε4  ‰Ó· ËÁ‚ÂÒÚÂÌ Ù‡ÍÚÓ Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (23, 32), ‰Ó͇ÚÓ Á‡ Ì„ӂËflÚ ÂÙÂÍÚ ‚˙ıÛ ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ëχ ÔÓÚË‚ÓÂ˜Ë‚Ë ÂÁÛÎÚ‡ÚË. çflÍÓË ‡‚ÚÓË ÔË ËÁÒΉ‚‡Ì ̇ Ô‡ˆËÂÌÚË Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ ÔÓ͇Á‚‡Ú ÒıӉ̇ ‡ÒoˆË‡ˆËfl ̇ ÄÔÓÖ ε4 ͇ÍÚÓ Ú‡ÁË Ì‡ Ô‡ˆËÂÌÚË Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (27, 21). ê‰ˈ‡ ‰Û„Ë ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú (26, 15), ˜Â ˜ÂÒÚÓÚ‡Ú‡ ÄÔÓÖ ε4 ‚ „ÛÔ‡Ú‡ ̇ ·ÓÎÌË Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ìflχ Á̇˜Ëχ ‡ÁÎË͇ Ò˙Ò Á‰‡‚ËÚ ÍÓÌÚÓÎË. êÂÁÛÎÚ‡ÚËÚ ÓÚ Ì‡¯ÂÚÓ ÔÓÛ˜‚‡Ì ÔÓ͇Á‚‡Ú Á̇˜ËÚÂÎÌÓ ‡ÒÓˆËË‡Ì ̇ ÄÔÓÖ ε4 ‡Î· Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ, ‚ Ò‡‚ÌÂÌËÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÍÎËÌ˘ÌÓ Á‰‡‚ËÚ Îˈ‡. ìÒÚ‡ÌÓ‚Â̇ڇ ˜ÂÒÚÓÚ‡ ̇ ÄÔÓÖ ‡ÎÂÎËÚ ‚ „ÛÔ‡Ú‡ ·ÓÎÌË Ò ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (ε2 - 0.02; ε3 - 0.68; ε4 - 0.30), ËÁ˜ËÒÎÂ̇ ‚˙Á ÓÒÌÓ‚‡ ̇ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÙÂÌÓÚËÔÓ‚ÂÚÂ,  ÏÌÓ„Ó ·ÎËÁ͇ ‰Ó Ú‡ÁË Ò˙Ó·˘Â̇ ÓÚ ë. Harrington Ë Ò˙‡‚Ú. (11) ‚ ÔÓÔÛ·ˆËfl ÓÚ Ô‡ÚӇ̇ÚÓÏ˘ÌÓ ‚ÂËÙˈË‡ÌË ÒÎÛ˜‡Ë Ò ÅÄ (ε2 - 0.03; ε3- 0.64; ε4- 0.33) Ë flÒÌÓ ÔÓ͇Á‚‡ Û‚Â΢ÂÌ ËÒÍ Á‡ ÔÓfl‚‡Ú‡ ̇ ÅÄ ÔË ÌÓÒËÚÂÎËÚ ̇ ε4 ‡Î·. Ç „ÛÔ‡Ú‡ ̇ Á‰‡‚ËÚ ÍÓÌÚÓÎË, ˜ÂÒÚÓÚ‡Ú‡ ̇ ε2, ε3 Ë Aε4 ‡ÎÂÎËÚ  Ò˙ÓÚ‚ÂÚÌÓ 11.4%, 79.2% Ë 9.4%. íÂÁË ‰‡ÌÌË Ò‡ ÏÌÓ„Ó ÒıÓ‰ÌË Ò ÎËÚÂ‡ÚÛÌËÚ ‰‡ÌÌË Á‡ ˜ÂÒÚÓÚ‡Ú‡ ̇ ÄÔÓ Ö ‡ÎÂÎËÚ ÔË Í‡‚͇Á͇ڇ ‡Ò‡ (28). êÂÁÛÎÚ‡ÚËÚ ÓÚ ÚÓ‚‡ ÔÓÛ˜‚‡Ì ÔÓ͇Á‚‡Ú, ˜Â Îˈ‡Ú‡, ÌÓÒËÚÂÎË Ì‡ ÔÓÌ ‰ËÌ ε2 ‡ÎÂÎ, Ò Ò¢‡Ú Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚÓ Ò‰ Á‰‡‚ËÚ Îˈ‡ (11.3%), ÓÚÍÓÎÍÓÚÓ ÔË ·ÓÎÌËÚ ÒÚ‡‰‡˘Ë ÓÚ ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ (2.2%) Ë ·ÓÎÌËÚÂ Ò ÅÄ Ò ‡ÌÌÓ Ì‡˜‡ÎÓ (3.2%). íÂÁË ÂÁÛÎÚ‡ÚË Ò˙‚Ô‡‰‡Ú Ò Ôӂ˜ÂÚÓ ÎËÚÂ‡ÚÛÌË ‰‡ÌÌË (28, 23) Á‡ ÔÓÚÂÍÚË‚ÌËflÚ ÂÙÂÍÚ Ì‡ ε2 ‡Î· ÔË ‡Á‚ËÚË ̇ ÅÄ. Ç ÔÓÒΉÌËÚ „Ó‰ËÌË Ò‡ ÓÚÍËÚË ÌÓ‚Ë ÔÓÎËÏÓÙËÁÏË ‚ ÔÓÏÓÚÓÌËfl Û˜‡ÒÚ˙Í Ì‡ ÄÔÓÖ „Â̇, ÍÓËÚÓ Ò‡ ‡ÒÓˆËË‡ÌË Ò ÅÄ. íÓ‚‡ ÔÓ͇Á‚‡, ˜Â ÌÂÁ‡‚ËÒËÏÓ ÓÚ Ì‡Î˘ÌËÚ ËÁÓÙÓÏË, ÂÒÍÔÂÒËflÚ‡ ̇ ÄÔÓÖ „Â̇ ҇χ ÔÓ Ò· ÒË Ëχ Á̇˜ÂÌË Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÅÄ. íÓ˜ÌËflÚ ÏÂı‡ÌËÁ˙Ï, ÔÓ ÍÓÈÚÓ ‡ÔÓÎËÔÓÔÓÚÂËÌ Ö ‚ÎËfl ‚˙ıÛ ÔÓfl‚‡Ú‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ‚Ò Ӣ Ì  ̇Ô˙ÎÌÓ ÛÚÓ˜ÌÂÌ, ÌÓ ‚˙ÔÂÍË ÚÓ‚‡ ̇˜‡ÎÌË ÔÓÛ˜‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â Û‚Â΢Â̇ڇ ˜ÂÒÚÓÚ‡ ̇ ÄÔÓÖ ε4 ‡ÎÂÎa ‚ ‰‡‰Â̇ ÔÓÔÛ·ˆËfl ÓÚ ·ÓÎÌË, ·Ë Ë„‡Î‡ ÓÎflÚ‡ ̇ Ë̉Ë͇ÚÓ Á‡ ‚ÂÓflÚ̇ ‰Â„ÂÌÂ‡Ú˂̇ ÂÚËÓ-


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

‰ÂÍÂÏ‚Ë, 2007

ÎÓ„Ëfl. ÅËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl ‰ÂÏÓÌÒÚË‡Ú, ˜Â ÄÔÓÖ ε4 ËÁÓÙÓχڇ Ò ҂˙Á‚‡ Ò ·ÂÚ‡-‡ÏËÎÓˉ ÏÌÓ„Ó ÔÓ-·˙ÁÓ ÓÚÍÓÎÍÓÚÓ ÄÔÓÖ ε2 (29). ç‚Ó·ËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl ÔÓ͇Á‚‡Ú, ˜Â ‚ ÏÓÁ˙˜ÌËÚ ÎÂÁËË Ì‡ ÅÄ ·ÓÎÌËÚ Ëχ ‚ËÒÓÍË ÍÓ̈ÂÌÚ‡ˆËË Ì‡ ÄÔÓÖ, ÍÓ‰Ë‡Ì ÓÚ ÄÔÓÖ „Â̇ (17). Ç Á‡Íβ˜ÂÌËÂ, ÌË ÔÓÚ‚˙ʉ‡‚‡ÏÂ, ˜Â ÄÔÓÖ ε4 ‡ÎÂÎ˙Ú Ò Ò¢‡ ÔÓ-˜ÂÒÚÓ ÔË ÅÄ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ. íÓ‚‡ ·Ë ÏÓ„ÎÓ ‰‡ ÓÁ̇˜‡‚‡ Ô‰‡ÁÔÓÎÓÊÂÌË Á‡ ÔÓfl‚‡Ú‡ ̇ ÅÄ ‚ Ú‡ÁË „ÛÔ‡. íÂÁË ÂÁÛÎÚ‡ÚË ·Ëı‡ ÏÓ„ÎË ‰‡ ‰ÓÔËÌÂÒ‡Ú Á‡ ÓˆÂÌ͇ڇ ̇ ÔÓÙË· ̇ ËÒ͇ Á‡ ÔÓfl‚‡ ̇ ‰ÂÏÂ̈Ëfl ÔË ‚˙Á‡ÒÚÌË Îˈ‡, ÔÓ͇Á‚‡ÈÍË, ˜Â ÔË ÌÓÒËÚÂÎËÚ ̇ ε4 ‡Î· Ëχ ÔÓ-„ÓÎflÏ ËÒÍ ÓÚ ‡Á‚ËÚË ̇ ÅÄ. ãàíÖêÄíìêÄ 1. 2. 3. 4.

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ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: èÓÙ. ‰- ã˙˜ÂÁ‡ ÑË̘ӂ í‡ÈÍÓ‚, ‰.Ï.Ì. ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, ·ÛÎ. “ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËfl 1431 ÚÂÎ.: 92 30 752 e-mail: traykov_l@yahoo.com


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

‰ÂÍÂÏ‚Ë, 2007

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éË„Ë̇ÎÌË ÒÚ‡ÚËË àçíÖêåÖÑàÖêçÄ îéêåÄ çÄ ÅéãÖëí çÄ NIEMANN-PICK èêà ÅöãÉÄêëäà êéåà Ç.åËı‡ÈÎÓ‚‡1, à.í˙Ì‚1, ü.ï‡ÌÚÍÂ2, à.ëËÌË„ÂÒ͇3, ë.óÂÌËÌÍÓ‚‡1, å.ꇉËÓÌÓ‚‡ , å.ê‡È˜Â‚‡1, ë. ä˙ÒÚ‚4, à.í‡ÈÍÓ‚‡1, Ä.òχÓ‚1, Ç. ɇ‚ËÍÓ‚‡5, Å.åË΂5, ê.íË̘‚‡6, Ñ. äÛ˛Ï‰ÊË‚7, ã. ä‡Î‡È‰ÊË‚‡2 1 ç‚ÓÎӄ˘̇ ÍÎËÌË͇, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, ëÓÙËfl 2 Centre for Medical Research and Western Australian Institute for Medical Research, The University of Western Australia, Perth, Australia 3 ㇷÓ‡ÚÓËfl ÔÓ ÏÓÎÂÍÛÎfl̇ Ô‡ÚÓÎÓ„Ëfl, ëÅÄãÄÉ “å‡È˜ËÌ ‰ÓÏ”, ëÓÙËfl 4 äÎËÌË͇ ÔÓ ÔÒËıˇÚËfl, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, ëÓÙËfl 5 ä‡Ú‰‡ ÔÓ ÂÌÚ„ÂÌÓÎÓ„Ëfl, ìåÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, ëÓÙËfl 6 ìÌË‚ÂÒËÚÂÚÒ͇ ·ÓÎÌˈ‡ ÔÓ Ô‰ˇÚËfl, ëÓÙËfl 7 äÎËÌË͇ ÔÓ Ô‰ˇÚËfl, åÅÄã „. è‡Á‡‰ÊËÍ 1

SUMMARY INTERMEDIATE TYPE OF NIEMANN-PICK DISEASE IN BULGARIAN ROMA V. Mihaylova1, I. Tournev1, J. Hantke2, I. Sinigerska3, S. Cherninkova1, M. Radionova1 M. Raicheva1, S. Krastev4, I. Traikova1, A. Shmarov1, V. Gavrikova5, B. Milev5, R. Tincheva6, D. Khuyomdziev7, L. Kalaydjieva2 1 Department of Neurology, University Hospital “Alexandrovska”, Medical University, Sofia, Bulgaria 2 Centre for Medical Research and Western Australian Institute for Medical Research, The University of Western Australia, Perth, Australia 3 National Genetic Laboratory, Medical University, Sofia, Bulgaria 4 Department of Psychiatry, Medical University, Sofia, Bulgaria 5 Department of Radiology, Medical University, Sofia, Bulgaria 6 Department of Pediatrics, Medical University, Sofia, Bulgaria 7 Department of Pediatrics, Regional Hospital Pazardjik, Bulgaria Niemann-Pick disease (NPD) is an autosomal-recessive disorder, caused by acid sphingomyelinase deficiency resulting from mutations in SMPD1 gene. Along with the traditional type A and type B phenotypes, intermediate forms have also been reported. We studied 20 Bulgarian Roma patients with intermediate type of Niemann-Pick disease. Detailed physical, neurological and psychiatric examinations, neuropsychological testing, routine hematological and biochemical analyses, electrophysiological studies, radiological investigations and geneting testing were performed. Evidence of nervous system involvement was found in 95% of the patients. Interestingly, there was an extreme variability in terms of severity and localization of the symptoms. While some patients demonstrated only macular halos, others presented with overt neurological and psychiatric symptoms. Sequencing of SMPD1 gene detected a T>G transversion at position 1177 leading to the substitution of glycine for the conserved tryptophane residue at amino acid position 391(W391G). All our NPD patients were homozygous for this mutation. Nevertheless they showed a high degree of variation in nervous system involvement suggesting that yet unidentified factors are likely to shape the phenotype. KEY WORDS: intermediate type of Niemann-Pick disease, macular halo, W391G.

êÖáûåÖ ÅÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ Â ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÂÁÛÎÚ‡Ú ÓÚ ‰ÂÙˈËÚ Ì‡ ÎËÁÓÁÓÏ̇ڇ ÒÙËÌ„ÓÏËÂÎË̇Á‡, ÔÓ‡‰Ë ÏÛÚ‡ˆËË ‚ „Â̇ SMPD1. ç‡‰ Ò Í·Ò˘ÂÒÍËÚ ÙÓÏË (çËχÌ-èËÍ ÚËÔ Ä Ë ÚËÔ Å), Ò‡ ÓÔËÒ‡ÌË Ë ‡ÚËÔ˘ÌË ËÌÚÂωËÂÌË ÒÎÛ˜‡Ë. Ç Ì‡ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡Ì ҇ ÓÔËÒ‡ÌË ı‡‡ÍÚÂËÒÚËÍËÚ ̇ ËÌÚÂωËÂ̇ڇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ ÔË 20 ·˙΄‡ÒÍË ÓÏË. èË Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë Ò‡ Ôӂ‰ÂÌË ÔÓ‰Ó·ÂÌ ÒÓχÚ˘ÂÌ Ë Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ, Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ, ÔÒËı˘ÌÓ ËÌÚÂ‚˛, ÛÚËÌÌË Í˙‚ÌË Ë ·ËÓıËÏ˘ÌË ËÁÒΉ‚‡ÌËfl, ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌË, ‡‰ËÓÎӄ˘ÌË Ë „ÂÌÂÚ˘ÌË ËÁÒΉ‚‡ÌËfl èË 95% ÓÚ ·ÓÎÌËÚ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Á‡Òfl„‡Ì ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ, ͇ÚÓ Â Ì‡Îˈ ËÁÍβ˜ËÚÂÎ̇ ‚‡ˇ·ËÎÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÂÊÂÒÚÚ‡ Ë ÎÓ͇ÎËÁ‡ˆËflÚ‡: ÓÚ ËÁÓÎË‡ÌÓ Ï‡ÍÛÎÌÓ ı‡ÎÓ ÔË ÌflÍÓË Ô‡ˆËÂÌÚË ‰Ó ËÁfl‚Â̇ Ì‚ÓÎӄ˘̇ Ë ÔÒËı˘̇ ÒËÏÔÚÓχÚË͇ ÔË ‰Û„Ë. ÑËÂÍÚÌÓÚÓ ÒÂÍ‚ÂÌË‡Ì ̇ SMPD1 „Â̇ ÛÒÚ‡ÌÓ‚Ë Ú‡ÌÒ‚ÂÁËflÚ‡ T1711>G, ÍÓflÚÓ ‚Ó‰Ë ‰Ó Á‡Ïfl̇ ̇ ÍÓÌÒÂ‚‡ÚË‚ÂÌ ÚËÔÚÓÙ‡Ì Á‡ „ÎˈËÌ Ì‡ 391 ÔÓÁˈËfl (W391G). ÇÒ˘ÍË Ì‡¯Ë Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚ Ì‡ çËχÌ-èËÍ Ò‡ ıÓÏÓÁË„ÓÚË ÔÓ Ú‡ÁË ÏÛÚ‡ˆËfl. Ç˙ÔÂÍË ÚÓ‚‡ ·ÓÎÌËÚ ÔÓ͇Á‚‡Ú ËÁÍβ˜ËÚÂÎ̇ ‚‡ˇ·ËÎÌÓÒÚ ‚ Ì‚ÓÎӄ˘̇ڇ ÒËÏÔÚÓχÚË͇, ÍÓÂÚÓ Ô‰ÔÓ·„‡ ̇΢ËÂÚÓ Ì‡ ‚Ò Ӣ ÌÂˉÂÌÚËÙˈË‡ÌË Ù‡ÍÚÓË, ÍÓËÚÓ ÏÓ‰ËÙˈË‡Ú ÙÂÌÓÚËÔ‡. äãûóéÇà Ñìåà: ËÌÚÂωËÂÂÌ ÚËÔ Ì‡ ·ÓÎÂÒÚ Ì‡ çËχÌ-èËÍ, χÍÛÎÌÓ ı‡ÎÓ, W391G. ÇöÇÖÑÖçàÖ ÅÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ Â ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ı‡‡ÍÚÂËÁË‡˘Ó ÒÂ Ò ‰ÂÙˈËÚ Ì‡ ÍËÒ· ÒÙËÌ„ÓÏËÂÎË̇Á‡, ÔÓ‡‰Ë ÏÛÚ‡ˆËË ‚ SMPD1 „Â̇. äËÒ·ڇ ÒÙËÌ„ÓÏËÂÎË̇Á‡  ÎËÁÓÁÓÏ̇ ıˉÓ·Á‡, ÌÂÓ·ıÓ‰Ëχ Á‡ ‡Á„‡Ê‰‡ÌÂÚÓ Ì‡ ÒÙËÌ„ÓÏËÂÎË̇ ‰Ó ˆÂ‡Ïˉ Ë ÙÓÒÙÓıÓÎËÌ, ÔÓ‡‰Ë ÍÓÂÚÓ ÔË ‰ÂÙˈËÚ Ì‡ ÚÓÁË ÂÌÁËÏ Ò ̇ÚÛÔ‚‡Ú ÒÙËÌ„ÓÏËÂÎËÌ Ë ‰Û„Ë ÎËÔË‰Ë ‚ ÍÎÂÚÍËÚ ̇ ååë Ë „‡Ì„ÎËÈÌËÚ ÍÎÂÚÍË Ì‡ ñçë (1). ÅÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ Ò Í·ÒËÙˈË‡ ̇ Ì‚ÓÌÓÔ‡ÚÂÌ ÚËÔ Ä, Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÒÏ˙Ú ‰Ó 2-3 „Ó‰Ë̇ Ë ·‡‚ÌÓÔÓ„ÂÒË‡˘ ÚËÔ Å, Ò ËÁÓÎË‡Ì‡ ‚ËÒˆÂ‡Î̇ ÒËÏÔÚÓχÚË͇ Ë ÒÏ˙Ú ‚ Áfl· ‚˙Á‡ÒÚ (1). Ç˙ÔÂÍË ÚÓ‚‡ Ò‡ ÓÔËÒ‡ÌË Ô‡ˆËÂÌÚË Ò ÔÓÚ‡ıË‡Ì ıÓ‰ Ë ‡ÁÌÓÓ·‡ÁÌË Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË. (2-8). ç‡ÒÍÓÓ ÔÛ·ÎËÍÛ‚‡ÌÓ ÔÓÛ˜‚‡Ì ̇ Ô‡ˆËÂÌÚË ÓÚ ÉÂχÌËfl, óÂıËfl Ë ëÎÓ‚‡ÍËfl, ÔÓ͇Á‚‡ ˜Â ËÌÚÂωËÂÌËfl ÙÂÌÓÚËÔ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â Á̇˜ËÚÂÎÌÓ ÔÓ-˜ÂÒÚ (9). ÑÛ„Ó ÔÓÛ˜‚‡Ì ‚ ëÄô (10) ÔÓ͇Á‚‡ ̇΢ˠ̇ ÔÓ„ÂÒ˂̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇ ÔË 8% Ë ÎÂÍË Ì‚ÓÎӄ˘ÌË ‡·ÌÓÏÌÓÒÚË ÔË 22%


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6

2

5

̉

+

+

+

151/131

45/26

123/97

19/13

15/8

+

̉

̉

̉

8

11

7

7

4

̉

+

+

+

+

+

+

+

+_

+

̉

̉

̉

чÌÌË ÓÚ ÔÓÒΉ̇ڇ ıÓÒÔËÚ‡ÎËÁ‡ˆËfl è·ÁÏÂ̇ Ç˙Á‡ÒÚ çËÒ˙Í ıÂÔ‡ÚÓ- AëÄí/AãÄT5 ëÔÎÂÌÓÏÂ- ïËÔÂÒÔ΄‡ÎËfl4 ÌËÁ˙Ï ıËÚÓ(„Ó‰ËÌË) ˙ÒÚ Ï„‡ÎËfl4 ÚËÓÁˉ‡Á‡2

̉

̉

̉

̉

ëÙËÌ„ÓÏËÂÎË̇Á̇ ‡ÍÚË‚ÌÓÒÚ ‚ ΂ÍÓ ˆËÚË Ë ÙË·ӷ·ÒÚË 1 ̉

퇷Îˈ‡ 1. ÇËÒˆÂ‡Î̇ ÒËÏÔÚÓχÚË͇ ÔË ·ÓÎÌËÚ ıÓÏÓÁË„ÓÚË ÔÓ W391G ‚ SMPD1

-

+

-

+

-

̉

̉

+

+

èÂÌÂÒÚË ÍÎÂÚÍË Ì‡ ÏËÂÎÓ„‡Ï‡Ú‡

+

+

+

-

+/0.44 ìÔÎ˙ÚÌÂÌË ÒÚÂÌË Ì‡ aorta ascendens, ËÁÓÒÚ‡‚‡Ì ‚ ÍÓÒÚÌËfl ‡ÒÚÂÊ, ÓÒÚÂÓÔÓÓÁ‡

+/0.52 ÌÂÙÓÎËÚˇÁ‡ +/0.96 èÚÓÁ‡ Ì ‰ÂÒÌËfl ·˙·ÂÍ +/0.5

ÅÂÎÓ‰- Ä·ÌÓ- ÑÛ„Ó ӷ̇ ÏÂÌ ËÌÙËÎÚ- ÎËÔˉÂÌ ‡ˆËfl ÔÓÙËÎ/ HDLıÓÎ6 ̉ ̉ ÉÛ·Ë ˜ÂÚË Ì‡ ÎˈÂÚÓ Ì‰ ̉ ÄÌÍËÎÓÁ‡ ̇ ·Í˙ÚÌË Ë ÍÓÎÂÌÌË ÒÚ‡‚Ë Ì‰ ̉ ÉÛ·Ë ˜ÂÚË Ì‡ ÎˈÂÚÓ Ì‰ ̉ ÉÛ·Ë ˜ÂÚË Ì‡ ÎˈÂÚÓ Ì‰ àıÚËÓÁËÒ ‚Û΄‡ËÒ

‰ÂÍÂÏ‚Ë, 2007

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology 250


Ï

Ï

Ï

Ï

11

12

13

14

éÒÚ˙ ·ÓÌıËÚ, ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl ïÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl

éÒÚ˙ ·ÓÌıËÚ, ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl êÂÒÔË‡ÚÓÌË ËÌÙÂ͈ËË, ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl

+

-

-

671

441 146

26F 2463

2L 19F

14L 23L 32F 12

182

35

15

17

<3

16

15

<3

<3

13

<3

7

6

6

̉

6

93/108

67/89

194/130

9

33/38

*

7

8

+_

7

-

-

+

̉

-

̉

̉

+

чÌÌË ÓÚ ÔÓÒΉ̇ڇ ıÓÒÔËÚ‡ÎËÁ‡ˆËfl è·ÁÏÂ̇ Ç˙Á‡ÒÚ çËÒ˙Í ıÂÔ‡- AëÄí/AãÄT5 ëÔÎÂÌÓÏÂ- ïËÔÂÒÔÎÂ- èÂÌÂÒÚË ıËÚÓ(„Ó‰ËÌË) ˙ÒÚ ÚÓ„‡ÎËfl4 ÌËÁ˙Ï ÍÎÂÚÍË ÚËÓÁËÏ„‡ ̇ ÏËÂÎÓ„ÎËfl4 ‡Ï‡Ú‡ ‰‡Á‡2

ëÙËÌ„ÓÏËÂÎË̇Á̇ ‡ÍÚË‚ÌÓÒÚ ‚ ΂ÍÓ ˆËÚË Ë ÙË·ӷ·ÒÚË 1 ̉

̉

+

+

+

+

ÅÂÎÓ‰ӷ̇ ËÌÙËÎÚ‡ˆËfl

̉

+

+/0.18

ËÁÓÒÚ‡‚‡Ì ‚ ÍÓÒÚÌËfl ‡ÒÚÂÊ, ÓÒÚÂÓÔÓÓÁ‡

ìÔÎ˙ÚÌÂÌË ÒÚÂÌË Ì‡ aorta ascendens, ÔÓÎËÔÓÁ‡ ̇ ÊÎ˙˜ÌËfl ÏÂıÛ +/0.52

Ä·ÌÓ- ÑÛ„Ó ÏÂÌ ÎËÔˉÂÌ ÔÓÙËÎ/ HDLıÓÎ6 +/0.51 àıÚËÓÁË Ò ‚Û΄‡Ë Ò ËÁÓÒÚ‡‚‡Ì ‚ ÍÓÒÚÌËfl ‡ÒÚÂÊ, ÌÂÙÓÎËÚˇÁ‡ +/0.59

15 16

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óÂÒÚË ËÌÙÂ͈ËË Ì‰ 36L 1880 21 <3 6 ̉ 9 ̉ ̉ ̉ óÂÒÚË ËÌÙÂ͈ËË 20L 155 17 5 7 82/117 9 ̉ + +/0.43 ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl 36F 17 Ï 1 óÂÒÚË ËÌÙÂ͈ËË + ̉ 617 1.5 + + 273/128 + + ̉ ̉ ̉ ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl 18 Ê 2 óÂÒÚË ËÌÙÂ͈ËË + 20L 1444 2.5 + + 299/250 + ̉ ̉ ̉ ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl 29F 19 Ï <1 óÂÒÚË ËÌÙÂ͈ËË + 17F 1241 0.6 + + 179/111 + + ̉ ̉ ̉ ıÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl 20 Ï <1 ïÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl + ̉ 614 1 + + 245/177 + ̉ ̉ ̉ Ê – ÊÂ̇, Ï – Ï˙Ê; + ‰‡; - ÌÂ; ̉ Ìflχ ‰‡ÌÌË; 1 ëÙËÌ„ÓÏËÂÎË̇Á̇ ‡ÍÚË‚ÌÓÒÚ ËÁÏÂÂ̇ Ò ËÁÍÛÒÚ‚ÂÌËfl ÒÛ·ÒÚ‡Ú 2-(N- Hexadecanoylamino)-4-nitrophenyl phosphocholine ‚ ΂ÍÓˆËÚËÚ (L), ÂÙÂÂÌÚÌË ÒÚÓÈÌÓÒÚË 24-135 nmol/17h/mg ÔÓÚÂËÌ Ë ‚˙‚ ÙË·ӷ·ÒÚËÚ (F), ÂÙÂÂÌÚÌË ÒÚÓÈÌÓÒÚË 102-590 nmol/h/mg ÔÓÚÂËÌ 2 êÂÙÂÂÌÚÌË ÒÚÓÈÌÓÒÚË 34±49 nmol/h/ml. è·ÁÏÂ̇ڇ ıËÚÓÚËÓÁˉ‡Á‡ ÔÓËÁıÓʉ‡ ÓÚ ‡ÍÚË‚Ë‡ÌËÚ χÍÓÙ‡„Ë Ë Ò ËÁÔÓÎÁ‚‡ ͇ÚÓ Ï‡ÍÂ Á‡ ÙÓÏË‡ÌÂÚÓ Ì‡ χÍÓÙ‡„Ë ËÁÔ˙ÎÌÂÌË Ò ÎËÔË‰Ë ÔË ·ÓÎÂÒÚ Ì‡ çËχÌ-èËÍ (Brinkman Ë Ò˙‡‚Ú., 2005) 3 Ç ÔÂÒ‡ÌÚËÎË Á‡ Ò˙ÓÚ‚ÂÚ̇ڇ ‚˙Á‡ÒÚ Ë ÔÓÎ ÒÔflÏÓ ÂÙÂÂÌÚÌËÚ ÌÓÏË Á‡ Å˙΄‡Ò͇ڇ ÔÓÔÛ·ˆËfl. ë (+) Ò‡ ÓÁ̇˜ÂÌË Ô‡ˆËÂÌÚËÚÂ, ÔË ÍÓËÚÓ ‚ ̇΢̇ڇ ‰ÓÍÛÏÂÌÚ‡ˆËfl  ÓÚ·ÂÎflÁ‡ÌÓ ËÁÓÒÚ‡‚‡Ì ‚ ‡ÒÚÂʇ ËÎË ÌËÒ˙Í ˙ÒÚ, ÌÓ ·ÂÁ ÚÓ˜ÌË ‰‡ÌÌË 4 ä‡ÚÓ ÒÏ ÔÓ‰ ·Â̇ڇ ‰˙„‡ ËÎË Í‡ÚÓ Ì‡Î˘Ë (+) ÔË ÎËÔÒ‡ ̇ ÔÓ‰Ó·ÌË ‰‡ÌÌË *èË Ô‡ˆËÂÌÚ 12  ÓÒ˙˘ÂÒÚ‚Â̇ ÒÔÎÂÌÂÍÚÓÏËfl. 5 êÂÙÂÂÌÚÌË ÒÚÓÈÌÓÒÚË ÄãÄí<41 U/l, ÄëÄí<37U/l 6 HDL-ıÓÎÂÒÚÂÓÎ (mmol/L): ш‡ Ê·ÌÓ >1.2mmol/l, ÌËÒÍÓ <0.9mmol/l; Ç˙Á‡ÒÚÌË Ê·ÌÓ ?1.6mmol/l, ÌËÒÍÓ <1mmol/l

7

~3

<1

<1

-

+

ïÂÔ‡ÚÓÒÔÂÌÓÏ„‡ÎËfl, ‡ÌÂÏËfl

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ᇷ‡‚ÂÌÓ ÔÒËıÓ ÏÓÚÓÌÓ ‡Á‚ËÚËÂ

臈ËÂÌÚ # èÓÎ Ç˙Á‡ÒÚ ç‡˜‡ÎÂÌ ÒËÏÔÚÓÏ Ì‡ ̇˜‡ÎÓ („Ó‰ËÌË)

‰ÂÍÂÏ‚Ë, 2007

Å˙΄‡Ò͇ ç‚ÓÎÓ„Ëfl Bulgarian Neurology 251


‰ÂÍÂÏ‚Ë, 2007

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

ÓÚ ·ÓÎÌËÚ Í·ÒËÙˈË‡ÌË Í‡ÚÓ ÚËÔ Å. íÂÁË ‰‡ÌÌË ÔÓ͇Á‚‡Ú, ˜Â ÔË ÏÌÓ„Ó Ô‡ˆËÂÌÚË Ò ‰ÂÙˈËÚ Ì‡ ÒÙËÌ„ÓÏËÂÎË̇Á‡ ÙÂÌÓÚËÔ˙Ú Ì ÓÚ„Ó‚‡fl ̇ Ú‡‰ËˆËÓÌ̇ڇ Í·ÒËÙË͇ˆËfl, ‡ ÍÎËÌ˘ÌËÚ ÒËÏÔÚÓÏË ÔÂÏË̇‚‡Ú Ô·‚ÌÓ, ·ÂÁ ‰‡ ÓÙÓÏflÚ ‰‚‡ Í‡ÈÌÓ ‡Á΢ÌË ÙÂÌÓÚËÔ‡. é„‡Ì˘ÂÌËflÚ ·ÓÈ ·ÓÎÌË Ò Ë‰ÂÌÚ˘ÂÌ „ÂÌÓÚËÔ, ͇ÍÚÓ Ë Ì‡Î˘ËÂÚÓ Ì‡ ËÁÍβ˜ËÚÂÎ̇ ‡ÎÂÎ̇ ıÂÚÂÓ„ÂÌÌÓÒÚ Á‡ÚÛ‰Ìfl‚‡Ú „ÂÌÓÚËÔ-ÙÂÌÓÚËÔ ÍÓ·ˆËËÚÂ. ÑÓ ÏÓÏÂÌÚ‡, ̇È-˜ÂÒÚ‡Ú‡ ÏÛÚ‡ˆËfl ‚ SMPD1 ‡Òӈˇ‡Ì‡ Ò ËÌÚÂωËÂÂÌ ÙÂÌÓÚËÔ Â Q292K, ÍÓflÚÓ Â ÓÔË҇̇ ‚ ıÓÏÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌË ÔË 6 Ë ÔË 13 ·ÓÎÌË ‚ ÍÓÏ·Ë̇ˆËfl Ò ‰Û„Ë ÏÛÚ‡ˆËË (9-11). Ç Ì‡ÒÚÓfl˘ÂÚÓ ÔÓÛ˜‚‡Ì Á‡ Ô˙‚Ë Ô˙Ú Ò ÓÔËÒ‚‡Ú ÙÂÌÓÚËÔÌËÚ ı‡‡ÍÚÂËÒÚËÍË Ì‡ ËÌÚÂωËÂ̇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ çËχÌ-èËÍ ÔË ·˙΄‡ÒÍË ÓÏË. èÓ‰Ó·ÌÓ Ì‡ ‰Û„Ë ‡‚ÚÓÁÓÏÌÓ-ˆÂÒË‚ÌË Á‡·ÓÎfl‚‡ÌËfl ‚ Ú‡ÁË ÔÓÔÛ·ˆËfl, ·ÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ Â ÂÁÛÎÚ‡Ú ÓÚ Â‰Ì‡ ‰ËÌÒÚ‚Â̇ ÏÛÚ‡ˆËfl W391G ‚ SMPD1. ÉÂÌÂÚ˘̇ڇ ıÓÏÓ„ÂÌÌÓÒÚ Ì‡ ̇¯ËÚ ԇˆËÂÌÚË Ô‰ÓÒÚ‡‚Ë ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ÓÔ‰ÂÎflÌ ̇ ÙÂÌÓÚËÔÌËfl ÒÔÂÍÚ˙ ̇ ËÌÚÂωËÂ̇ڇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ. Ç˙ÔÂÍË Ë‰ÂÌÚ˘ÌËfl „ÂÌÓÚËÔ, Ô‡ˆËÂÌÚËÚ ÔÓ͇Á‚‡Ú ËÁÍβ˜ËÚÂÎ̇ ‚‡ˇ·ËÎÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÚÂÊÂÒÚÚ‡ Ë ÎÓ͇ÎËÁ‡ˆËflÚ‡ ̇ Á‡Òfl„‡Ì ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ. äéçíàçÉÖçí à åÖíéÑà Ç ÔÓÛ˜‚‡ÌÂÚÓ Û˜‡ÒÚ‚‡Ú 20 ·˙΄‡ÒÍË ÓÏË (15 Ï˙ÊÂ, 5 ÊÂÌË) ÓÚ 16 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË Ò ·ÓÎÂÒÚ Ì‡ çËχÌèËÍ. è˙‚Ó̇˜‡ÎÌÓ ‰Ë‡„ÌÓÁ‡Ú‡  ÔÓÒÚ‡‚Â̇ ‚˙Á ÓÒÌÓ‚‡ ̇ ÍÎ��Ì˘ÌËÚ ‰‡ÌÌË Ë ÂÁÛÎÚ‡ÚËÚ ÓÚ ·ËÓıËÏ˘ÌÓÚÓ ËÁÒΉ‚‡Ì (‡ÍÚË‚ÌÓÒÚ Ì‡ ÍËÒ· ÒÙËÌ„ÓÏËÂÎË̇Á‡ ‚ ΂ÍÓˆËÚË Ë ÍÛÎÚË‚Ë‡ÌË ÙË·ӷ·ÒÚË). èÓ ‚ÂÏ ̇ ÔÓÒΉÌÓÚÓ ÍÎËÌ˘ÌÓ ËÁÒΉ‚‡Ì ·ÓÎÌËÚ ҇ ̇ ‚˙Á‡ÒÚ ÓÚ 7 ÏÂÒˆ‡ ‰Ó 35 „Ó‰ËÌË. 臈ËÂÌÚËÚ 5 (ÔÓ˜Ë̇Î) Ë 6, ͇ÍÚÓ Ë 1,2,3,4 (‚Ò˘ÍË ÔÓ˜Ë̇ÎË) Ò‡ ·‡Úfl Ë ÒÂÒÚË. 臈ËÂÌÚË 1-4 Ò‡ Ô˙‚ËÚ ÓÔËÒ‡ÌË ‡ÚËÔ˘ÌË ÒÎÛ˜‡Ë ̇ çËχÌèËÍ ‚ Å˙΄‡Ëfl (12). èÓ‰ӷ̇ ‡Ì‡ÏÌÂÁ‡, ÒÓχÚ˘ÂÌ Ë Ì‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ, ÛÚËÌÌË ıÂχÚÓÎӄ˘ÌË Ë ·ËÓıËÏ˘ÌË ÔÓ͇Á‡ÚÂÎË, Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ, ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl (ÖçÉ, Ï.Ò.ëÖè, ÖÖÉ), ÂÌÚ„ÂÌÓ„‡ÙËfl ̇ ·ÂÎË ‰Ó·Ó‚Â Ë ˙ˆÂ, ÂıÓ„‡ÙËfl ̇ ÍÓÂÏ, äí Ë åêí ̇ „·‚‡ Ò‡ ÓÒ˙˘ÂÒÚ‚ÂÌË ÔË Ôӂ˜ÂÚÓ ·ÓÎÌË. ÑËÂÍÚÌÓ ÒÂÍ‚ÂÌË‡Ì ̇ SMPD1 e ÓÒ˙˘ÂÒÚ‚ÂÌÓ ÔË 16 ·ÓÎÌË, ͇ÍÚÓ Ë ÔË Ï‡È͇ڇ Ë ÊË‚Ëfl ·‡Ú ̇ Ô‡ˆËÂÌÚË 1-4. êÖáìãíÄíà ÇËÒˆÂ‡Î̇ ÒËÏÔÚÓχÚË͇ (Ú‡·Î. 1) 燘‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÔÂÁ Ô˙‚ËÚ 2 „Ó‰ËÌË ÔË ÔÓ˜ÚË ‚Ò˘ÍË Ô‡ˆËÂÌÚË, ÌÓ ‰Ë‡„ÌÓÁ‡Ú‡  ÔÓÒÚ‡‚Â̇ ‚ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó Â‰‚‡ ÔË 7 ÓÚ Úflı Ë Â Á‡·‡‚Â̇ ÓÚ 9 ‰Ó 27 „Ӊ˯̇ ‚˙Á‡ÒÚ ÔË ‚Ò˘ÍË ÓÒڇ̇ÎË (ÔË Ô‡ˆËÂÌÚ 5 ‰Ë‡„ÌÓÁ‡Ú‡  ÔÓÒÚ‡‚Â̇ ÂÚÓÒÔÂÍÚË‚ÌÓ, ÒΉ Ì„ӂ‡Ú‡ ÒÏ˙Ú). 燘‡ÎÌËÚ ÒËÏÔÚÓÏË Ò‡ ıÂÔ‡ÚÓÒÔÎÂÌÓÏ„‡ÎËfl, ˜ÂÒÚË ÂÒÔË‡ÚÓÌË ËÌÙÂ͈ËË Ë Á‡·‡‚ÂÌÓ ÔÒËıÓÏÓÚÓÌÓ ‡Á‚ËÚËÂ. óÂÒÚ‡ ̇ıӉ͇  ÌËÒÍËflÚ ˙ÒÚ: ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò‡ ÔÓ‰ 25ÚË ÔÂÒ‡ÌÚËÎ, ‡ 5 ·ÓÎÌË Ò‡ ÔÓ‰ 3ÚË ÔÂÒ‡ÌÚËΠ̇ ÌÓχڇ. ïÂÔ‡ÚÓÒÔÎÂÌÓÏ„‡ÎËfl Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË. ëÂÛÏÌËÚ ‡ÏËÌÓÚ‡ÌÒÙÂ‡ÁË (ÄëÄí Ë ÄãÄí) Ò‡ Ôӂ˯ÂÌË ÔË 11/14 ·ÓÎÌË. ïËÔÂÒÔÎÂÌËÁ˙Ï ËÏ‡Ú 9/16 ·ÓÎÌË. èË ÔÓÒΉÌÓÚÓ ËÁÒΉ‚‡Ì Ì Ò ̇·Î˛‰‡‚‡ ̇Û¯Â̇ ÒËÌÚÂÚ˘̇ ÙÛÌ͈Ëfl ̇ ˜ÂÌËfl ‰Ó· ÔË ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÌÓ ÔÓ ‰‡ÌÌË ÓÚ Ô‰ıÓ‰ÌË ÂÔËÍËÁË ÔÓÚÓÏ·ËÌÓ‚ÓÚÓ ‚ÂÏ  ·ËÎÓ Ì‡Ï‡ÎÂÌÓ ÔË 3 ·ÓÎÌË. èӂ˜ÂÚÓ ·ÓÎÌË ËÏ‡Ú ˜ÂÒÚË

252

ÂÒÔË‡ÚÓÌË ËÌÙÂ͈ËË. ÅÂÎÓ‰Ó·ÌËÚ ÂÌÚ„ÂÌÓ„‡ÙËË ÔÓ͇Á‚‡Ú ÛÒËÎÂÌ ËÒÛÌ˙Í ÔË 7/10 ·ÓÎÌË. HDL-ıÓÎÂÒÚÂÓÎ˙Ú Â ÔÓÌËÊÂÌ ÔË ‚Ò˘ÍË 9 ·ÓÎÌË, ÔË ÍÓËÚÓ Â ËÁÒΉ‚‡Ì (7 ÓÚ Úflı Ò‡ ‰Âˆ‡). éÒ‚ÂÌ ÚÓ‚‡ ÔË ‰Âˆ‡Ú‡ Ò ̇·Î˛‰‡‚‡ Ôӂ˯ÂÌ Ó·˘ ıÓÎÂÒÚÂÓÎ, LDL-ıÓÎÂÒÚÂÓÎ Ë ÚË„ÎˈÂˉË. Ç ÏËÂÎÓ„‡ÏËÚ ̇ 7 ·ÓÎÌË Ò ÓÚÍË‚‡Ú ı‡‡ÍÚÂÌËÚ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ ÔÂÌÂÒÚË ÍÎÂÚÍË, ÌÓ Ú‡ÍË‚‡ Ì Ò ̇·Î˛‰‡‚‡Ú ÔË 3 Ô‡ˆËÂÌÚË Ì‡ ‚˙Á‡ÒÚ 7 ÏÂÒˆ‡, 2 Ë 13 „Ó‰ËÌË. ÄÍÚË‚ÌÓÒÚÚ‡ ̇ ÍËÒ·ڇ ÒÙËÌ„ÓÏËÂÎË̇Á‡ ‚ ΂ÍÓˆËÚËÚÂ, ËÁÏÂÂ̇ Ò ËÁÍÛÒÚ‚ÂÌËfl ÒÛ·ÒÚ‡Ú 2-N-Hexadecanoylamino-4-nitrophenylphosphocholine, Ì  ̇‰ÂʉÂÌ ÔÓ͇Á‡ÚÂÎ Á‡ ÔÓÒÚ‡‚flÌ ̇ ‰Ë‡„ÌÓÁ‡Ú‡, Ú˙È Í‡ÚÓ Â Ì‡Ï‡ÎÂ̇ Ò‡ÏÓ ÔË 3/8 ·ÓÎÌË, ͇ÚÓ Á̇˜ËÚÂÎÌÓ Â Ì‡Ï‡ÎÂÌËÂÚÓ Ò‡ÏÓ ÔË Ô‡ˆËÂÌÚ 11. èÓ-ËÌÙÓχÚË‚ÌÓ Â ËÁÏÂ‚‡ÌÂÚÓ Ì‡ ÂÌÁËÏ̇ڇ ‡ÍÚË‚ÌÓÒÚ ‚ ÍÛÎÚË‚Ë‡ÌË ÙË·ӷ·ÒÚË: ̇χÎÂ̇ ‡ÍÚË‚ÌÓÒÚ ËÏ‡Ú ‚Ò˘ÍËÚ 8 ·ÓÎÌË, ÔË ÍÓËÚÓ Â ËÁÏÂ‚‡Ì‡, Ò ‚‡ˇˆËË ÓÚ 15 ‰Ó 60% ÓÚ ÌÓχڇ. è·ÁÏÂ̇ڇ ıËÚÓÚËÓÁˉ‡Á‡  ̇‰ÂʉÂÌ ‰Ë‡„ÌÓÒÚ˘ÂÌ ÔÓ͇Á‡ÚÂÎ, Ôӂ˯Â̇ ‡ÍÚË‚ÌÓÒÚ Ò ̇·Î˛‰‡‚‡ ÔË ‚Ò˘ÍË ËÁÒΉ‚‡ÌË 15 ·ÓÎÌË. áÄëüÉÄçÖ çÄ çÖêÇçÄíÄ ëàëíÖåÄ á‡Òfl„‡Ì ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ Ò ̇·Î˛‰‡‚‡ ÔË 19 ÓÚ Ì‡¯ËÚ 20 Ô‡ˆËÂÌÚË, ͇ÚÓ ÎÓ͇ÎËÁ‡ˆËflÚ‡ Ë ÚÂÊÂÒÚÚ‡ ̇ Ì‚ÓÎӄ˘̇ڇ ÒËÏÔÚÓχÚË͇ Ò‡ ËÁÍβ˜ËÚÂÎÌÓ ‚‡ˇ·ËÎÌË: ÓÚ ËÁÓÎË‡ÌÓ Ï‡ÍÛÎÌÓ ı‡ÎÓ ‰Ó ËÁ‡ÁÂ̇ ‡Ú‡ÍÒËfl, ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙˈËÚ Ë ÔÒËıÓÁ‡ (Ú‡·Î. 2). ãËÔÒ‡ ̇ Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË Ë Ì‡ χÍÛÎÌÓ ı‡ÎÓ Ò ̇·Î˛‰‡‚‡ Ò‡ÏÓ ÔË Â‰ËÌ Ô‡ˆËÂÌÚ 19 (7-ÏÂÒ˜ÌÓ ·Â·Â). çÖÇêééîíÄãåéãéÉàóçÄ ëàåèíéåÄíàäÄ å‡ÍÛÎÌÓÚÓ ı‡ÎÓ Â Ì‡È-ı‡‡ÍÚÂ̇ڇ ̇ıӉ͇, Ú˙È Í‡ÚÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÔË 16/18 ·ÓÎÌË, ÔË ÍÓËÚÓ Â Ôӂ‰Â̇ ÓÙÚ‡ÎÏÓÒÍÓÔËfl. å‡ÍÛÎÌÓ ı‡ÎÓ Ì Ò ̇·Î˛‰‡‚‡ ÔË Ô‡ˆËÂÌÚË 18 Ë 19, ̇ ‚˙Á‡ÒÚ Ò˙ÓÚ‚. 2.5 „Ó‰ËÌË Ë 7 ÏÂÒˆ‡. 燷≇‚‡ Ò ‚˙Á‡ÒÚÓ‚Ó Á‡‚ËÒËχ ÔÓ„ÂÒËfl ‚ ‡ÁÏÂ‡ ̇ χÍÛÎÌÓÚÓ ı‡ÎÓ. éÚÌÓ¯ÂÌËÂÚÓ Ì‡ ‚˙̯ÌËfl ‰Ë‡ÏÂÚ˙ ̇ ı‡ÎÓÚÓ ÒÔflÏÓ Ô‡ÔËÎÂÌËfl ‰Ë‡ÏÂÚ˙ Á‡ ˆfl·ڇ „ÛÔ‡ ·ÓÎÌË Â 0.75?0.28, ͇ÚÓ ‚‡Ë‡ ÓÚ 0.33 ÔË Ì‡È-Ï·‰Ëfl Ô‡ˆËÂÌÚ (̇ 5 „Ó‰ËÌË) ‰Ó 1.1 Ë 1.2 ÔË Ì‡È-‚˙Á‡ÒÚÌËÚ ԇˆËÂÌÚË (̇ 26 Ë 35 „Ó‰ËÌË). äÓË„Ë‡Ì‡Ú‡ ÁËÚÂÎ̇ ÓÒÚÓÚ‡  ÌÓχÎ̇ ÔË ‚Ò˘ÍË ·ÓÎÌË. áÄëüÉÄçÖ çÄ ñçë ñÂ·Â·̇ ÒËÏÔÚÓχÚË͇ ËÏ‡Ú 4 ·ÓÎÌË. ÄÚ‡ÍÒËfl  ‚Ӊ¢ ÒËÏÔÚÓÏ ÔË Ô‡ˆËÂÌÚ 5, Ô˘Ë̇ Á‡ ËÌ‚‡ÎˉËÁ‡ˆËfl ‚ Ï·‰‡ ‚˙Á‡ÒÚ. èÓ-ÎÂ͇ χÎÍÓÏÓÁ˙˜Ì‡ ÒËÏÔÚÓχÚË͇ (‰ËÒÏÂÚËfl Ë ·Ë·ÚÂ‡ÎÂÌ ËÌÚÂ̈ËÓÌÂÌ ÚÂÏÓ ÔË ÌÓÒÓ-ÔÓ͇Á‡Î˜̇ڇ ÔÓ·‡) Ò ̇·Î˛‰‡‚‡ ÔË Ô‡ˆËÂÌÚ 8 (̇ 16 „Ó‰ËÌË) Ë 12 (̇ 35 „Ó‰ËÌË). 臈ËÂÌÚ 12 Ëχ Ó˘Â Ë ·Ë·ÚÂ‡ÎÂÌ ËÌÚÂ̈ËÓÌÂÌ ÚÂÏÓ ÔË ÍÓÎflÌÌÓÒÚ˙Ô‡Î̇ڇ ÔÓ·‡ Ë ‰Ë҉ˇ‰ÓıÓÍËÌÂÁËfl. àÌÚÂ̈ËÓÌÂÌ ÚÂÏÓ ÔË ÍÓÎflÌÌÓ-ÒÚ˙Ô‡Î̇ڇ ÔÓ·‡ Ò ̇·Î˛‰‡‚‡ Ë ÔË Ô‡ˆËÂÌÚ 15. åêí ̇ „·‚ÌËfl ÏÓÁ˙Í ÔÓ͇Á‚‡ χÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ë ‡ÚÓÙËfl ̇ ÔÓÌÒ‡ ÔË Ô‡ˆËÂÌÚ 8, χÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ë Û‚Â΢ÂÌ ‡ÁÏÂ ̇ ˆËÒÚÂ̇ χ„̇ ÔË Ô‡ˆËÂÌÚ 12. èË‡Ïˉ̇ ÒËÏÔÚÓχÚË͇ ËÏ‡Ú ÚËχ ·ÓÎÌË. èË Ô‡ˆËÂÌÚË 5 Ë 6 Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÎËÔÒ‡ ËÎË ‡ÒËÏÂÚËfl ̇ ÍÓÊÌËÚ ÍÓÂÏÌË ÂÙÎÂÍÒË. èË Ô‡ˆËÂÌÚ 11 Ò ̇·Î˛‰‡‚‡ ‰ÂÒÌÓÒÚ‡ÌÂÌ Å‡·ËÌÒÍË Ò ˆÂÌÚ‡Î̇ ‰flÒ̇ ‰ÓÎ̇ ÏÓÌÓÔ‡ÂÁ‡ Ë ÔÂÒ ÂÍ‚ËÌÓ‚‡ÛÒ. ÖÍÒÚ‡ÔË‡Ïˉ̇ ÒËÏÔÚÓχÚË͇ Ëχ Ò‡ÏÓ ÔË Ô‡ˆËÂÌÚ 5 – ‡ÚÂÚÓÁ‡ ‚ ˜ÂÚËËÚ Í‡ÈÌˈË.


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

‰ÂÍÂÏ‚Ë, 2007

253

퇷Îˈ‡ 2 á‡Òfl„‡Ì ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ ÄÌ„‡ÊË‡Ì ̇ ñçë 臈ËÂÌÚ Ç˙Á‡ÒÚ # („Ó‰ËÌË) ÔË ÔÓÒΉÌÓÚÓ ËÁÒΉ‚‡Ì 1 12 2 20 3 16 4 10 5 23

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++ ‰‡ ‰‡ ‰‡ ++

‡Ú‡ÍÒËfl, ÔË‡ÏˉÌË Ë ÂÍÒÚ‡ÔË‡ÏˉÌË ÒËÏÔÚÓÏË ÔË‡ÏˉÌË ÒËÏÔÚÓÏË ‡Ú‡ÍÒËfl ÔË‡ÏˉÌË ÒËÏÔÚÓÏË ÄÚ‡ÍÒËfl ãÂ͇ ‡Ú‡ÍÒËfl ̉ ̉

ÄÌ„‡ÊË‡Ì ̇ ÔÂËÙÂ̇ڇ ÌÂ‚̇ ÒËÒÚÂχ åÂÌÚ‡Î̇ èÒËı˘ÌË ÖÖÉ Ä·ÌÓÏÌÓ äÎËÌ˘ÂÌ ç‡Ï‡ÎÂ̇ ç‡Ï‡ÎÂÌË ÂÚ‡‰‡- ÒËÏÔÚÓÏË ÔÓÏÂÌË Ì‚ÓËÁÓ·- Ô„Ή ëè CMAè/ ˆËfl ‡Áfl‚‡Ì ëçAè ‡ÏÔÎËÚÛ‰Ë ++ ̉ ̉ ̉ ̉ ̉ +++ ̉ ̉ ̉ ̉ ̉ + ̉ ̉ ̉ ̉ ̉ + ̉ ̉ ̉ ̉ ̉ + + ++ ̉ -

6 26 +++ ++ ++ ++ + 7 14 ++ ± ̉ + + 8 16 ++ ++ ± + + + + 9 5 + ± ̉ + 10 17 ‰‡ ++ ± 11 15 ++ + + ++ 12 35 +++ ++ ± + + 13 16 ++ ± + + 14 12 ̉ +++ ̉ ̉ ̉ 15 21 ч ̉ ̉ + ̉ 16 17 +++ + + 17 1.5 ‰‡ +* ̉ ̉ ̉ ̉ 18 2.5 +* ̉ ̉ ̉ ̉ 19 0.6 -* ̉ ̉ ̉ ̉ 20 1 ̉ +* ̉ ̉ ̉ ̉ ̉ – Ìflχ ‰‡ÌÌË; + ‰‡; - ÌÂ; å‡ÍÛÎÌÓ ı‡ÎÓ: +<0.5 èÑ (Ô‡ÔËÎÂÌ ‰Ë‡ÏÂÚ˙); ++ 0.5-0.75 èÑ; +++ >0.75; “‰‡” ÔÓ͇Á‚‡ ̇΢ËÂÚÓ Ì‡ ı‡ÎÓ, ÌÓ ·ÂÁ ÍÓ΢ÂÒÚ‚Â̇ ÓˆÂÌ͇ åÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl (ÒÚÓÈÌÓÒÚË Ì‡ IQ): - ÌÓχÎÌË/„‡Ì˘ÌË (85-70); + ÎÂ͇ (<70); ++ ÛÏÂÂ̇ (<50); +++ ÚÂÊ͇ (<30); +* Á‡·‡‚ÂÌÓ ÔÒËı˘ÂÒÍÓ ‡Á‚ËÚË ÒÔÓ‰ ÎÂÍÛ‚‡˘ËÚ Ô‰ˇÚË, ÌÓ ÎËÔÒ‚‡ Ì‚ÓÔÒËıËÎӄ˘ÌÓ ËÁÒΉ‚‡Ì èÒËı˘ÌË ÒËÏÔÚÓÏË: Ô‡ˆËÂÌÚ 8 (?) ̇΢ˠ̇ ÔÒËıÓÚ˘ÌË ÂÔËÁÓ‰Ë ‚ ıÓ‰‡ ̇ ˜ÂÌÓ‰ӷ̇ Â̈ÂÙ‡ÎÓÔ‡ÚËfl EEÉ ÔÓÏÂÌË: (?) ÎÂÍË ÔÓÏÂÌË Ò ‰ËÙÛÁÌË ? Ë ÓÒÚË ‚˙ÎÌË; (+) „ÂÌÂ‡ÎËÁË‡Ì‡ ·‡‚ÌÓ‚˙ÎÌÓ‚‡ ‡ÍÚË‚ÌÓÒÚ ËÎË ÙÓÍÛÒ ÓÚ ÓÒÚË ‚˙ÎÌË; (+ +) Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ ËÎË „Û·Ó Ì‡Û¯Â̇ ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ ç‚ÓËÁÓ·‡Áfl‚‡ÌÂ: Ô‡ˆËÂÌÚ 6 – „ÂÌÂ‡ÎËÁË‡Ì‡ ‡ÚÓÙËfl; Ô‡ˆËÂÌÚ 8 – ÔÓÌÚÓˆÂ·Â·̇ ‡ÚÓÙËfl; Ô‡ˆËÂÌÚ 12 – χÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl èëàïàóçÄ ëàåèíéåÄíàäÄ èӂ‰Â̘ÂÒÍË ÓÚÍÎÓÌÂÌËfl (ËÏÔÛÎÒË‚ÌÓÒÚ, ÒÂÍÒÛ‡Î̇ Ë ‡„ÂÒ˂̇ ‡ÁÚÓÏÓÁÂÌÓÒÚ) Ò ̇·Î˛‰‡‚‡Ú ÔË Ô‡ˆËÂÌÚ 5 (̇ 23 „Ó‰ËÌË) Ë Ô‡ˆËÂÌÚ 11 (̇ 15 „Ó‰ËÌË), ÍÓËÚÓ ËÏ‡Ú ÎÂ͇ ÒÚÂÔÂÌ Ì‡ ÛÏÒÚ‚Â̇ ËÁÓÒڇ̇ÎÓÒÚ. 臈ËÂÌÚ 12  ‰Ë‡„ÌÓÒÚˈË‡Ì Ò ‡ÙÂÍÚË‚ÌÓ ‡ÁÒÚÓÈÒÚ‚Ó (ÔÂËӉ˘̇ ‰ÂÔÂÒËfl) ̇ 30 „Ó‰ËÌË. íÓÈ Ëχ ÚË ‰ÂÔÂÒË‚ÌË ÂÔËÁÓ‰Ë Ò˙Ò ÒÚ‡ıÓ‚Ó-ÔÒËıÓÚ˘ÂÌ ı‡‡ÍÚÂ Ë ‚Ó‰Â˘Ó ÔÂÊË‚fl‚‡Ì ̇ ‚ËÚ‡Î̇ Á‡ÒÚ‡¯ÂÌÓÒÚ. èÓ ‚ÂÏ ̇ ÚÂÚËfl ÂÔËÁÓ‰  ̇Á‡Ì‡˜ÂÌÓ Î˜ÂÌËÂ Ò êËÒÔÂˉÓÌ, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó Ô˙Î̇ ÂÏËÒËfl. éÚ 23 „Ӊ˯̇ ‚˙Á‡ÒÚ Ô‡ˆËÂÌÚ 6 Ëχ ı‡Î˛ˆË̇ˆËË Ë Ô‡‡ÌÓˉÌË Ì‡ÎÛ‰ÌÓÒÚË, ÔÓ‡‰Ë ÍÓÂÚÓ Â ÎÂÍÛ‚‡Ì‡ Ò ‡Á΢ÌË Ì‚ÓÎÂÔÚˈË. èË ÔÓÒΉ̇ڇ È ıÓÒÔËÚ‡ÎËÁ‡ˆËfl ‚ ÍÎËÌË͇ڇ ̇ 26 „Ӊ˯̇ ‚˙Á‡ÒÚ Ô‡ˆËÂÌÚ͇ڇ Ëχ ‡ÍÚ˂̇ ‚Â·‡Î̇ ı‡Î˛ˆËÌÓÁ‡, Ô‰ËÏÌÓ Ò ÍÓÏÂÌÚË‡˘ ı‡‡ÍÚÂ, ÒÓχÚ˘ÌË ı‡Î˛ˆË̇ˆËË, ̇ÎÛ‰ÌÓÒÚË Ò Ô‡‡ÌÓˉÌÓ Ò˙‰˙ʇÌËÂ. èÓÒÚ‡‚Â̇  ‰Ë‡„ÌÓÁ‡Ú‡ ¯ËÁÓÙÂÌÓÔӉӷ̇ ÔÒËıÓÁ‡ Ë Á‡ÔӘ̇ڇ ÚÂ‡ÔËfl Ò êËÒÔÂˉÓÌ, ͇ÚÓ ÒΉ 3 ÏÂÒˆ‡  ̇Îˈ ÏÌÓ„Ó ÎÂÍÓ ÔÓ‰Ó·ÂÌËÂ. èË Ú‡ÁË Ô‡ˆËÂÌÚ͇ äí ̇ ÏÓÁ˙͇ ̇ 10 „Ӊ˯̇ ‚˙Á‡ÒÚ Â ÌÓχÎ̇, ÌÓ Ì‡ 26 „Ó‰ËÌË äí Ë åêí ̇ „·‚‡Ú‡ ‰ÂÏÓÌÒÚË‡Ú „ÂÌÂ‡ÎËÁË‡Ì‡ ÍÓÓ‚‡ ‡ÚÓÙËfl. çÖÇêéèëàïéãéÉàóçé àáëãÖÑÇÄçÖ éÚ 16 ·ÓÎÌË, ÔË ÍÓËÚÓ Â ÓÒ˙˘ÂÒÚ‚ÂÌÓ Ì‚ÓÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡ÌÂ, 6 (Ô‡ˆËÂÌÚË 7, 9, 12, 13, 15, 16) ËÏ‡Ú Ò‡-

+ + + + + + + ̉ ̉ + ̉ ̉ ̉ ̉

ÏÓÒÚÓflÚÂÎÌÓ ÒӈˇÎÌÓ ÙÛÌ͈ËÓÌË‡ÌÂ Ë IQ ‚ ‡ÏÍËÚ ̇ ÌËÒ͇ڇ ÌÓχ ËÎË „‡Ì˘ÌËÚ ÒÚÓÈÌÓÒÚË (70-85). ÑÂÒÂÚ ·ÓÎÌË ËÏ‡Ú ÛÏÒÚ‚Â̇ ËÁÓÒڇ̇ÎÓÒÚ ÒÔÓ‰ ICD-10 ÍËÚÂËËÚÂ. 臈ËÂÌÚË 3,4,5 Ë 11 ËÏ‡Ú ÎÂ͇ ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl (IQ<70) Ë ÌÂ̇Ô˙ÎÌÓ ÌÂÁ‡‚ËÒËÏÓ ÒӈˇÎÌÓ ÙÛÌ͈ËÓÌË‡ÌÂ. 臈ËÂÌÚË 1,6,8 Ë 10 ËÏ‡Ú ÛÏÂÂ̇ ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl (IQ<50). Ñ‚‡Ï‡ ·ÓÎÌË (Ô‡ˆËÂÌÚË 2 Ë 14) ËÏ‡Ú ÚÂÊ͇ ÛÏÒÚ‚Â̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (IQ<30) Ò Ì‚˙ÁÏÓÊ̇ ‚Â·‡Î̇ Ë Ì‚Â·‡Î̇ ÍÓÏÛÌË͇ˆËfl, Ò ÏÓÚÓÌË ÒÚÂÂÓÚËÔË Ë Ì‡Ô˙ÎÌÓ Á‡‚ËÒËÏÓ ÒӈˇÎÌÓ ÙÛÌ͈ËÓÌË‡ÌÂ. ÖÖÉ ëڇ̉‡Ú̇ ÖÖÉ Ë ÏÓÁ˙˜Ì‡ ͇ÚÓ„‡ÙËfl Ò‡ Ôӂ‰ÂÌË ÔË 10 ·ÓÎÌË (Ô‡ˆËÂÌÚË 6 Ë 12 Ò‡ ̇ ÚÂ‡ÔËfl Ò êËÒÔÂˉÓÌ ÔÓ ‚ÂÏ ̇ ËÁÒΉ‚‡ÌÂÚÓ). éÒÌӂ̇ڇ ‡ÍÚË‚ÌÓÒÚ Â ÌÓχÎ̇ ÔË ‚Ò˘ÍË Ò ËÁÍβ˜ÂÌË ̇ Ô‡ˆËÂÌÚ 5. èË 8 ·ÓÎÌË Ò ̇·Î˛‰‡‚‡ ‰ËÙÛÁ̇ ·‡‚ÌÓ‚˙ÎÌÓ‚‡ ‡ÍÚË‚ÌÓÒÚ ÓÚ Θ ‚˙ÎÌË Ë ÓÒÚË ‚˙ÎÌË. å‡ÌËÙÂÒÚÌÓ ‡·ÌÓÏÌÓ ÖÖÉ ËÏ‡Ú 5 Ô‡ˆËÂÌÚË. èË Ô‡ˆËÂÌÚ 5  ̇Îˈ ÒËÎÌÓ ‰ÂÁÓ„‡ÌËÁË‡Ì‡ ÓÒÌӂ̇ ‡ÍÚË‚ÌÓÒÚ Ò ÏÌÓÊÂÒÚ‚Ó ‰ËÙÛÁÌÓ ‡ÁÔÓÒÚ‡ÌÂÌË Θ Ë δ ‚˙ÎÌË, Ô‰ËÏÌÓ ‚˙‚ ÙÓÌÚ‡ÎÌËÚ ÓÚ‚Âʉ‡ÌËfl; Ô‡ˆËÂÌÚ 16 Ëχ „ÂÌÂ‡ÎËÁË‡Ì‡ ·‡‚ÌÓ‚˙ÎÌÓ‚‡ ‡ÍÚË‚ÌÓÒÚ (4Hz) Ô‰ËÏÌÓ ÙÓÌÚ‡ÎÌÓ Ë Ó͈ËÔËÚ‡ÎÌÓ. èË Ô‡ˆËÂÌÚË 8 Ë 11  ̇Îˈ ÙÓÍÛÒ ÓÚ ÓÒÚË ‚˙ÎÌË, Ò˙ÓÚ‚. ‚ ‰flÒÌÓ ÚÂÏÔÓ‡ÎÌÓ Ë ˆÂÌÚÓÔ‡ËÂÚ‡ÎÌÓ. è‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ ËÏ‡Ú ‰‚‡Ï‡ ·ÓÎÌË: Ô‡ˆËÂÌÚ 6 Ëχ „ÂÌÂ‡ÎËÁË‡Ì‡ Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ Ò ·ËÙÓÌÚ‡ÎÂÌ Ï‡ÍÒËÏÛÏ, ‡ Ô‡ˆËÂÌÚ 11 Ëχ Ô‡ÓÍÒËÁχÎÂÌ ÙÓÍÛÒ Ô‡-


‰ÂÍÂÏ‚Ë, 2007

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

ËÂÚ‡ÎÌÓ Ò ÍÓÌÚ‡Î‡ÚÂ‡Î̇ Ë‡‰Ë‡ˆËfl. åÓÁ˙˜ÌÓÒÚ‚ÓÎÓ‚Ë ÒÎÛıÓ‚Ë Â‚ÓÍË‡ÌË ÔÓÚÂ̈ˇÎË (Ï.Ò.ëÖè) å.Ò.ëÖè Ò‡ ÌÓχÎÌË ÔË 7/7 ·ÓÎÌË. ë‰̇ڇ ·ÚÂ̈Ëfl ̇ ‚˙Î̇ I  1.57+ _0.11 (ÌÓχ 1.6+ _0.2), ̇ ‚˙Î̇ III  3.74+ _0.13 (ÌÓχ 3.7+ _0.2), ̇ ‚˙Î̇ V  5.69+ _0.1 (ÌÓχ 5.8+ _0.2). àÌÚÂÔËÍÓ‚ËÚ ·ÚÂ̈ËË Ò‡ Ò˙˘Ó ‚ ÌÓχ: I-III 2.2+ _0.1 (ÌÓχ 2.2+ _1.48), III-V 1.96+ _0.17 (ÌÓχ 1.95+ _1.49), I-V 4.06+ _0.11 (ÌÓχ 4.15+ _0.22). éÚÌÓ¯ÂÌËÂÚÓ Ì‡ V/I ‚˙Î̇ Ò˙˘Ó  ÌÓχÎÌÓ ÔË ‚Ò˘ÍË ËÁÒΉ‚‡ÌË ·ÓÎÌË. áÄëüÉÄçÖ çÄ èÖêàîÖêçÄíÄ çÖêÇçÄ ëàëíÖåÄ äÎËÌ˘ÌË ‰‡ÌÌË Á‡ ÎÂ͇, Ô‰ËÏÌÓ ÏÓÚÓ̇ ÔÓÎËÌ‚ÓÔ‡ÚËfl, ËÏ‡Ú 2 ·ÓÎÌË. èË Ô‡ˆËÂÌÚ 7 Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‡ÂÙÎÂÍÒËfl ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Ë ıËÔÓÂÙÎÂÍÒËfl ‚ „ÓÌËÚ Í‡ÈÌˈË. èË Ò˙˘Ëfl Ô‡ˆËÂÌÚ ÎÂÍÓ Â Ì‡Ï‡ÎÂÌ Ë ‚Ë·‡ˆËÓÌÌËflÚ ÛÒÂÚ. ïËÔÓÂÙÎÂÍÒËfl ‚ „ÓÌËÚ Í‡ÈÌËˆË Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ë ÔË Ô‡ˆËÂÌÚ 8. èË Ô‡ˆËÂÌÚË 13 Ë 15 Ò ̇·Î˛‰‡‚‡ ‰ËÒÚ‡ÎÌÓ Á‡ÒË΂‡˘‡ Ò ıËÔÂÂÒÚÂÁËfl ÔË ËÁÒΉ‚‡Ì ̇ Ú‡ÍÚËÎÌËfl ÛÒÂÚ. ÖÎÂÍÚÓÌ‚Ó„‡ÙÒÍËÚ ËÁÒΉ‚‡ÌËfl Ò‡ ‡·ÌÓÏÌË ÔË 8/10 ·ÓÎÌË. èË 6 ·ÓÎÌË Ò ̇·Î˛‰‡‚‡ ÎÂÍÓ Ì‡Ï‡ÎÂ̇ ÒÍÓÓÒÚ Ì‡ ÔÓ‚Âʉ‡Ì (ÂÁÛÎÚ‡ÚËÚ ̇ Ô‡ˆËÂÌÚË 12 Ë 13 Ì ҇ ‚Íβ˜ÂÌË, ÔÓ‡‰Ë ̇΢ˠ̇ ‰ËÒÍÓ‚Ë ıÂÌËË Ì‡ L4-L5 Ë L5-S1). ë‰̇ڇ ÒÍÓÓÒÚ Ì‡ ÔÓ‚Âʉ‡Ì Á‡ n. medianus  43.68+ _0.75Ï/ÒÂÍ (ÌÓχ >46 Ï/ÒÂÍ), Á‡ n. ulnaris  49.5+ _2.54 Ï/ÒÂÍ (ÌÓχ >54 Ï/ÒÂÍ), ‡ Á‡ n. eroneus  37.48+ _4.57 Ï/ÒÂÍ (ÌÓχ >46.2 Ï/ÒÂÍ). ÑËÒÚ‡ÎÌËÚ ·ÚÂÌÚÌË ‚ÂÏÂ̇ Ò‡ ÌÓχÎÌË ÔË ‚Ò˘ÍË ·ÓÎÌË. çËÒÍÓ‡ÏÔÎËÚÛ‰ÌË ëåÄè Ò ̇·Î˛‰‡‚‡Ú ÔË 7 ·ÓÎÌË, Ò‰̇ڇ ‡ÏÔÎËÚÛ‰‡ Á‡ n. medianus  1.21+ _0.99mV (ÌÓχ >4mV), Á‡ n. eroneus 2.73+ _0.61mV (ÌÓχ >5mV) Ë Á‡ n. tibialis  2.53+ _1.26mV (ÌÓχ >5mV). çËÒÍÓ‡ÏÔÎËÚÛ‰ÌË ëçÄè Ò ̇·Î˛‰‡‚‡Ú ÔË 8 Ô‡ˆËÂÌÚË, ͇ÚÓ ÒÚÓÈÌÓÒÚËÚ Á‡ n. medianus Ò‡ ÓÚ 12.1 ‰Ó 17.2+ _V ÔË 3 ·ÓÎÌË, Ò‰ÌËÚ ÒÚÓÈÌÓÒÚË Á‡ n. ulnaris Ò‡ 13.86+ _1.97 µV, ‡ Á‡ n. suralis 10.36+ _4.23 µV (ÌÓχ >20 µV Á‡ ‚Ò˘ÍË ËÁÒΉ‚‡ÌË ÌÂ‚Ë). èË Ô‡ˆËÂÌÚ 16 Ì Ò ÔÓÎÛ˜‡‚‡ ëçÄè ÓÚ n. suralis. åéãÖäìãüêçé-ÉÖçÖíàóçé àáëãÖÑÇÄçÖ ÑËÂÍÚÌÓÚÓ ÒÂÍ‚ÂÌË‡Ì ̇ SMPD1 ÛÒÚ‡ÌÓ‚Ë Ú‡ÌÒ‚ÂÁËflÚ‡ T1711>G, ÍÓflÚÓ ‚Ó‰Ë ‰Ó Á‡Ïfl̇ ̇ ÍÓÌÒÂ‚‡ÚË‚ÂÌ ÚËÔÚÓÙ‡Ì Á‡ „ÎˈËÌ Ì‡ 391 ÔÓÁˈËfl (W391G). ÇÒ˘ÍË Ì‡¯Ë ÊË‚Ë Ô‡ˆËÂÌÚË Ò‡ ıÓÏÓÁË„ÓÚË ÔÓ Ú‡ÁË ÏÛÚ‡ˆËfl. Ä̇ÎËÁ˙Ú Ì‡ Ò˙ı‡ÌÂ̇ Ñçä ÓÚ Ô‡ˆËÂÌÚ 5 Ò˙˘Ó ÔÓ͇Á‚‡ ̇΢ˠ̇ ÏÛÚ‡ˆËflÚ‡ ‚ ıÓÏÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌËÂ. éÚÍË‚‡ÌÂÚÓ Ì‡ W391G ‚ ıÂÚÂÓÁË„ÓÚÌÓ Ò˙ÒÚÓflÌË ÔË Ï‡È͇ڇ Ë ÊË‚Ëfl ·‡Ú ̇ Ô‡ˆËÂÌÚË 1-4 ÍÓÒ‚ÂÌÓ ÔÓ͇Á‚‡, ˜Â ÚÂÁË ·ÓÎÌË Ò˙˘Ó Ò‡ ıÓÏÓÁË„ÓÚË ÔÓ W391G . éÅëöÜÑÄçÖ ç‡¯ËÚ W391G ıÓÏÓÁË„ÓÚË ÔÓ͇Á‚‡Ú ËÌÚÂωËÂ̇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ. ÇËÒˆÂ‡Î̇ڇ ÒËÏÔÚÓχÚË͇  ÚÂÊ͇ Ë ‡ÁÌÓÓ·‡Á̇ Ë Ì Ò ‡Á΢‡‚‡ ÓÚ ÔÛ·ÎËÍÛ‚‡ÌËÚ çËχÌ-èËÍ ÚËÔ Å Ô‡ˆËÂÌÚË Ò ‡Á΢ÌË ÏÛÚ‡ˆËË ‚ SMPD1 (13, 14). Ç˙ÔÂÍË Ë‰ÂÌÚ˘ÌËfl „ÂÌÓÚËÔ, ̇¯ËÚ ԇˆËÂÌÚË ËÏ‡Ú ËÁÍβ˜ËÚÂÎÌÓ ‡ÁÌÓÓ·‡Á̇ Ì‚ÓÎӄ˘̇ ÒËÏÔÚÓχÚË͇. è‰ıÓ‰ÌÓ ÔÛ·ÎËÍÛ‚‡ÌËÚ ÔÓÛ˜‚‡ÌËfl ÓÔËÒ‚‡Ú ‡ÁÌÓÓ·‡ÁÌË ÍÎËÌ˘ÌË Ë ÒÛ·ÍÎËÌ˘ÌË Ï‡ÌËÙÂÒÚ‡ˆËË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ, ͇ÚÓ ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl, ÖÖÉ ‡·ÌÓÏÌÓÒÚË Ë ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË, ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl, ÔÒËıÓÁË Ë Ú.Ì. (2-9). àÌÚÂÔÂÚ‡ˆËflÚ‡ ̇ ÚÂÁË ‰‡ÌÌË Ó·‡˜Â  ÒÎÓÊ̇ ÔÓ‡‰Ë ‡ÁÌÓÓ·‡ÁÌËÚ ÏÛÚ‡ˆËË Ë ÎËÔÒ‡Ú‡ ̇ ÏÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓ ËÁÒΉ‚‡ÌÂ. ÅÓÎÌËÚ ıÓÏÓÁË„ÓÚË ÔÓ W391G ‰ÂÏÓÌÒÚË‡Ú ‡ÁÌÓÓ·‡ÁÌË ÒËÏÔÚÓÏË, ÍÓËÚÓ ÔÓÍË‚‡Ú ˆÂÎËfl ÒÔÂÍÚ˙ ̇·Î˛‰‡‚‡Ì ÔË ·ÓÎÌË Ò ‰Û„Ë ÏÛÚ‡ˆËË Ë ‡ÁÎË-

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˜ÂÌ ÂÚÌ˘ÂÌ ÔÓËÁıÓ‰. Ç Â‰ËÌËfl Í‡È ̇ ÚÓÁË ÒÔÂÍÚ˙ W391G ıÓÏÓÁË„ÓÚËÚ ҇ ÌÂ‡Á΢ËÏË ÓÚ Í·Ò˘ÂÒÍËfl ‚ËÒˆÂ‡ÎÂÌ çËχÌ-èËÍ ÚËÔ Å. íÂÁË Ô‡ˆËÂÌÚË ÌflÏ‡Ú Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË ËÎË ËÏ‡Ú Ò‡ÏÓ ÒÛ·ÍÎËÌ˘ÌÓ ÂÚË̇ÎÌÓ ‡Ì„‡ÊË‡Ì (χÍÛÎÌÓ ı‡ÎÓ) ·ÂÁ ÙÛÌ͈ËÓ̇ÎÌÓ Á̇˜ÂÌËÂ, ÍÓÂÚÓ ËÁ„ÎÂʉ‡  ˜ÂÒÚ‡ ̇ıӉ͇ ÔË çËχÌ-èËÍ ÚËÔ Å (15). Ç Ú‡ÁË „ÛÔ‡ ÏÓÊ ‰‡ ÓÚÌÂÒÂÏ Ô‡ˆËÂÌÚ 19. èÂËÙÂÌÓ-ÌÂ‚ÌÓ ‡Ì„‡ÊË‡ÌÂ, Ò˙Ò Á‡Òfl„‡Ì ̇ ò‚‡ÌÓ‚ËÚ ÍÎÂÚÍË Ë ‡ÍÒÓÌËÚÂ, ‚Ó‰Â˘Ó ‰Ó ÒÛ·ÍÎËÌ˘̇ ËÎË ÎÂ͇ ÔÂËÙÂ̇ Ì‚ÓÔ‡ÚËfl Ò ̇·Î˛‰‡‚‡ ÔË Ôӂ˜ÂÚÓ Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â ÓÒ˙˘ÂÒÚ‚ÂÌÓ ÂÎÂÍÚÓÌ‚Ó„‡ÙÒÍÓ ËÁÒΉ‚‡ÌÂ. èË Ô‡ˆËÂÌÚ 9 ÚÓ‚‡  ‰ËÌÒÚ‚Â̇ڇ ‰ÓÔ˙ÎÌËÚÂÎ̇ Í˙Ï Ï‡ÍÛÎÌÓÚÓ ı‡ÎÓ Ì‡ıӉ͇, ÍÓflÚÓ ‡Á΢‡‚‡ ÙÂÌÓÚËÔ‡ ÓÚ ˜ËÒÚÓ ‚ËÒˆÂ‡ÎÌËfl çËχÌ-èËÍ ÚËÔ Å. äÎËÌ˘ÌË ‰‡ÌÌË Á‡ Á‡Òfl„‡Ì ̇ ñçë Ëχ ÔË 6/18 ·ÓÎÌË. ñÂ·Â·̇ ‡Ú‡ÍÒËfl Ò ̇·Î˛‰‡‚‡ ÔË 4 ·ÓÎÌË Ë Â ‚Ӊ¢‡ ÒËÏÔÚÓχÚË͇ ÔË Â‰ËÌ ÓÚ Úflı. å‡ÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò ̇·Î˛‰‡‚‡ ÔË 2 ÓÚ ·ÓÎÌËÚÂ Ò ‡Ú‡ÍÒËfl, ÔË ÍÓËÚÓ Â Ôӂ‰ÂÌÓ Ì‚ÓËÁÓ·‡Áfl‚‡˘Ó ËÁÒΉ‚‡ÌÂ. ñÂ·Â·ÌË ÒËÏÔÚÓÏË Ë åêí-‰‡ÌÌË Á‡ ˆÂ·Â·̇ ‡ÚÓÙËfl Ò‡ ÓÔËÒ‡ÌË Ë ÓÚ Pavlu-Pereira Ë Ò˙‡‚Ú. (2005), ‡ Á‡„Û·‡Ú‡ ̇ ÍÎÂÚÍË Ì‡ èÛÍËÌËÂ Ë Ó·˘‡ χÎÍÓÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl Ò‡ ÓÒÌÓ‚ÌËÚ ԇÚÓÎӄ˘ÌË Ì‡ıÓ‰ÍË ‚ Ï˯Ëfl ÏÓ‰ÂΠ̇ ÒÙËÌ„ÓÏËÂÎË̇Á‡ ‰ÂÙˈËÚÌËfl çËχÌ-èËÍ (16). чÌÌËÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ë ÊË‚ÓÚËÌÒÍËfl ÏÓ‰ÂÎ ÔÓ͇Á‚‡Ú ËÁÍβ˜ËÚÂÎ̇ ÒÂÌÁËÚË‚ÌÓÒÚ Ì‡ χÎÍËfl ÏÓÁ˙Í ÒÔflÏÓ ‰ÂÙˈËÚ‡ ̇ ÒÙËÌ„ÓÏËÂÎË̇Á‡. èË‡ÏˉÌËÚ ÒËÏÔÚÓÏË Ò‡ ÒΉ‚‡˘‡Ú‡ ̇È-˜ÂÒÚ‡ χÌËÙÂÒÚ‡ˆËfl, ̇·Î˛‰‡‚‡ÌË ÔË 3 ÓÚ ·ÓÎÌËÚÂ. ÖÖÉ ÔÓÏÂÌË Ò ̇·Î˛‰‡‚‡Ú ÔË 8/10 Ô‡ˆËÂÌÚË, ‚‡Ë‡˘Ë ÓÚ ‰ËÙÛÁÌË ÎÂÍË ‡·ÌÓÏÌÓÒÚË ‰Ó ÙÓ͇Î̇ Ë Ô‡ÓÍÒËÁχÎ̇ ‡ÍÚË‚ÌÓÒÚ, ÌÓ ·ÂÁ ÂÔËÎÂÔÚ˘ÌË ÔËÒÚ˙ÔË. èÒËı˘ÌË ÒËÏÔÚÓÏË, ̇·„‡˘Ë ıÓÒÔËÚ‡ÎËÁ‡ˆËfl Ë Î˜ÂÌËÂ Ò Ì‚ÓÎÂÔÚˈË, Ò ̇·Î˛‰‡‚‡Ú ÔË 2 ·ÓÎÌË: Ô‡ˆËÂÌÚ 6 Ò ¯ËÁÓÙÂÌÓÔӉӷ̇ ÔÒËıÓÁ‡ Ë Ô‡ˆËÂÌÚ 12 Ò ÔÂËӉ˘̇ ‰ÂÔÂÒËfl. äÓ„ÌËÚË‚ÌËÚ ̇Û¯ÂÌËfl Ò‡ ˜ÂÒÚË ÔË W391G ıÓÏÓÁË„ÓÚËÚÂ, ÛÒÚ‡ÌÓ‚fl‚‡Ú Ò ÔË 10/16 ·ÓÎÌË ‚ ̇ÒÚÓfl˘ÓÚÓ ÔÓÛ˜‚‡ÌÂ. íÂÁË ‰‡ÌÌË Úfl·‚‡ ‰‡ Ò ËÌÚÂÔÂÚË‡Ú Ô‰ԇÁÎË‚Ó, ËχÈÍË Ô‰‚ˉ ÒӈˇÎ̇ڇ ËÁÓ·ˆËfl, ÎËÔÒ‡Ú‡ ̇ ‰ÓÒÚ‡Ú˙˜ÌË ÔÓÁ̇ÌËfl ÔÓ ·˙΄‡ÒÍË ÂÁËÍ, ÎËÔÒ‡Ú‡ ̇ ͇ÎË·Ë‡Ì‡ ÏÂÚÓ‰Ë͇ Á‡ ÔÒËıÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ̇ ÓÏÒ͇ڇ ÔÓÔÛ·ˆËfl. Ç˙ÔÂÍË ÚÓ‚‡ ̇¯ËÚ ‰‡ÌÌË Ò‡ ͇Ú„Ó˘ÌË ÔË ‰‚‡Ï‡ ·ÓÎÌË Ò ÚÂÊ͇ ÏÂÌÚ‡Î̇ ÂÚ‡‰‡ˆËfl Ë ÔË ‰Û„Ë ˜ÂÚËËχ Í·ÒËÙˈË‡ÌË Í‡ÚÓ ÛÏÂÂÌÓ ËÁÓÒڇ̇ÎË. 燯ËÚ ‰‡ÌÌË Ì ÔÓÁ‚ÓÎfl‚‡Ú ÓˆÂÌ͇ ̇ ‚ÓβˆËflÚ‡ ̇ Á‡Òfl„‡Ì ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ ÔË W391G ıÓÏÓÁË„ÓÚËÚÂ. èÓ„ÂÒËfl ̇ ÎËÔˉÌÓÚÓ Ì‡ÚÛÔ‚‡Ì Ò ÔÓ‰ÍÂÔfl ÓÚ Ì‡·Î˛‰‡‚‡ÌÓÚÓ Û‚Â΢ÂÌË ̇ ‡ÁÏÂ‡ ̇ ı‡ÎÓÚÓ Ò ‚˙Á‡ÒÚÚ‡, ÍÓÂÚÓ Â ÓÔËÒ‡ÌÓ ÔË ÔÓÒΉfl‚‡ÌÂÚÓ Ì‡ Ô‡ˆËÂÌÚË Ë ÓÚ ‰Û„Ë ‡‚ÚÓË (15,17). çflÍÓË ÓÚ Ì‡¯ËÚ ·ÓÎÌË ÔÓ͇Á‚‡Ú ‰ÓÔ˙ÎÌËÚÂÎÌË ·ÂÎÂÁË Ì‡ ÔÓ„ÂÒ˂̇ Ì‚Ӊ„ÂÌÂ‡ˆËfl. èË Ô‡ˆËÂÌÚ 5 ˆÂ·Â·̇ڇ ‡Ú‡ÍÒËfl  ̇Îˈ ÓÚ ‰ÂÚÒÚ‚ÓÚÓ, ̇ÒÚ˙Ô‚‡ ‚ÎÓ¯‡‚‡Ì ̇ 13 „Ó‰ËÌË Ë ‰ÓÔ˙ÎÌËÚÂÎ̇ ÔÓfl‚‡ ̇ ÖÖÉ ‡·ÌÓÏÌÓÒÚË, ‡ ÔÓ-Í˙ÒÌÓ Ò ̇·Î˛‰‡‚‡Ú Ë ÔË‡ÏˉÌË, ÂÍÒÚ‡ÔË‡ÏˉÌË Ë Ôӂ‰Â̘ÂÒÍË ÔÓÏÂÌË. ëÂÒÚ‡Ú‡ ̇ ÚÓÁË Ô‡ˆËÂÌÚ (Ô‡ˆËÂÌÚ 6) Ìflχ Ì‚ÓÎӄ˘ÌË ÒËÏÔÚÓÏË Ì‡ 10 „Ó‰ËÌË, ̇ 23 „Ó‰ËÌË ‡Á‚Ë‚‡ ÔÒËıÓÁ‡, ‡ ÔÓ-Í˙ÒÌÓ (̇ 27 „Ó‰ËÌË) Ò ̇·Î˛‰‡‚‡Ú ÔË‡ÏˉÌË ·ÂÎÂÁË, ÖÖÉ ÔÓÏÂÌË Ë ÏÓÁ˙˜Ì‡ ‡ÚÓÙËfl. éÚ ‰Û„‡ ÒÚ‡Ì‡ ‰‚‡Ï‡ Ô‰ıÓ‰ÌÓ ÔÛ·ÎËÍÛ‚‡ÌË ıÓÏÓÁË„ÓÚË ÔÓ W391G ÓÚ ë˙·Ëfl ̇ ‚˙Á‡ÒÚ 28 Ë 31 „Ó‰ËÌË, ÔÓ͇Á‚‡Ú ÒÚ‡·ËÎÂÌ ÍÎËÌ˘ÂÌ ıÓ‰, ·ÂÁ ‰‡ÌÌË Á‡ Ì‚ÓÎӄ˘ÌÓ Á‡Òfl„‡Ì (17). àÁ‡ÁÂÌË ÍÎËÌ˘ÌË ‡Á΢Ëfl ÔË Ì‡Î˘Ë ̇ ‰̇Í˙‚ „ÂÌÓÚËÔ Ò ̇·Î˛‰‡‚‡Ú Ë ÔË ‰Û„Ë ÏÓÌÓ„ÂÌÌË Á‡·ÓÎfl‚‡ÌËfl, ÍÓÂÚÓ ÏÓÊ ‰‡  ÂÁÛÎÚ‡Ú ÓÚ ‰ÂÈÒÚ‚ËÂÚÓ Ì‡ ‡Á΢ÌË ÏÓ‰ËÙË͇ÚÓË. ç‡Î˘ËÂÚÓ Ì‡ ‰ÓÔ˙ÎÌËÚÂÎÌË „ÂÌÂÚ˘ÌË Ù‡ÍÚÓË, ÍÓËÚÓ ÏÓ‰ËÙˈË‡Ú Ì‚ÓÎӄ˘̇ڇ


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

‰ÂÍÂÏ‚Ë, 2007

ÒËÏÔÚÓχÚË͇ ÔË ÒÙËÌ„ÓÏËÂÎË̇Á‡-‰ÂÙˈËÚÌËfl çËχÌ-èËÍ Ò ÔÓ‰ÍÂÔfl ÓÚ Ì‡Î˘ËÂÚÓ Ì‡ ÚẨÂ̈Ëfl Á‡ ÒıÓ‰ÒÚ‚Ó ‚ ÔÓÚ˘‡ÌÂÚÓ ÏÂÊ‰Û Á‡Ò„̇ÚËÚ ËÌ‰Ë‚Ë‰Ë ÓÚ Â‰Ì‡ Ë Ò˙˘‡ Ù‡ÏËÎËfl (Ô‡ˆËÂÌÚË 1-4, Ô‡ˆËÂÌÚË 5,6). 燯ËÚ ‰‡ÌÌË ËÏ‡Ú Ô‡ÍÚ˘ÂÒÍÓ Á̇˜ÂÌË Á‡ ‰Ë‡„ÌÓÁ‡Ú‡ Ë Î˜ÂÌËÂÚÓ Ì‡ ·ÓÎÌË Ò ·ÓÎÂÒÚ Ì‡ çËχÌ-èËÍ. ÄÚËÔ˘ÌËflÚ ıÓ‰, ͇ÍÚÓ Ë Ú‡‰ËˆËÓÌ̇ڇ Ô‰ÒÚ‡‚‡ Á‡ ̇΢ËÂÚÓ Ì‡ ËÌÙ‡ÌÚËÎÂÌ Ì‚ÓÌÓÔ‡ÚÂÌ ÚËÔ Ä Ë ıÓÌ˘ÂÌ ‚ËÒˆÂ‡ÎÂÌ ÚËÔ Å, ÏÓ„‡Ú ‰‡ ‰Ó‚‰‡Ú ‰Ó Á̇˜ËÚÂÎÌË ‰Ë‡„ÌÓÒÚ˘ÌË Á‡ÚÛ‰ÌÂÌËfl ÔË W391G ıÓÏÓÁË„ÓÚË. çÂ̇‰ÂʉÌËÚ ÂÁÛÎÚ‡ÚË ÓÚ ËÁÏÂ‚‡ÌÂÚÓ Ì‡ ‡ÍÚË‚ÌÓÒÚÚ‡ ̇ ÍËÒ·ڇ ÒÙËÌ„ÓÏËÂÎË̇Á‡ Ò ËÁÍÛÒÚ‚ÂÌ ÒÛ·ÒÚ‡Ú Ë ÎËÔÒ‡Ú‡ ̇ ÔÂÌÂÒÚË ÍÎÂÚÍË ‰ÓÔ˙ÎÌËÚÂÎÌÓ Á‡ÚÛ‰Ìfl‚‡Ú ÔÓÒÚ‡‚flÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡. åÓÎÂÍÛÎflÌÓ-„ÂÌÂÚ˘ÌÓÚÓ ËÁÒΉ‚‡Ì Á‡ W391G ÛÎÂÒÌfl‚‡ ‰Ë‡„ÌÓÒÚ˘ÌËfl ÔÓˆÂÒ, ‡ÍÓ Ò ÔËÎÓÊË Ì‡ ‚ÒÂÍË ÓÏÒÍË Ô‡ˆËÂÌÚ Ò˙Ò ÒÔÎÂÌÓÏ„‡ÎËfl Ë Û‚Â΢Â̇ Ô·ÁÏÂ̇ ıËÚÓÚËÓÁˉ‡Á‡, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‚˙Á‡ÒÚÚ‡ Ë Ì‡Î˘ËÂÚÓ Ì‡ Ì‚ÓÎӄ˘ÌÓ Á‡Òfl„‡ÌÂ. ãËÔÒ‡Ú‡ ̇ ÍÓ·ˆËfl ÏÂÊ‰Û SMPD1 „ÂÌÓÚËÔ‡ Ë ‡Ì„‡ÊË‡ÌÂÚÓ Ì‡ ñçë, ÍÓÂÚÓ Ò ̇·Î˛‰‡‚‡ ÔË Ì‡¯ËÚ ԇˆËÂÌÚË,  ÓÚ ËÁÍβ˜ËÚÂÎ̇ ‚‡ÊÌÓÒÚ Á‡ ıÓÌ˘̇ڇ Ì‚Ó‚ËÒˆÂ‡Î̇ ÙÓχ ̇ ·ÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ. ç‚˙ÁÏÓÊÌÓÒÚÚ‡ ‰‡ Ò Ô‰‚Ë‰Ë ‡Ì„‡ÊË‡ÌÂÚÓ Ì‡ ñçë, ‰‡‚‡ ÌÓ‚Ë Ì‡ÒÓÍË Á‡ ÔË·„‡Ì ̇ ÂÌÁËÏ Á‡ÏÂÒÚËÚÂÎ̇ڇ ÚÂ‡ÔËfl Ë ÔË ËÌÚÂωËÂ̇ڇ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. ãàíÖêÄíìêÄ 1.

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al. (1978) Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation. Eur J Pediatr 128:235-240. Elleder M and Cihula J (1983) Niemann-Pick disease (Variation in the sphingomyelinase deficient group) Eur J Pediatr 140:323-328. Shah MD, Desai AP, Jain MK, Kulkarni V, Patel P, Waradkar AM, et al. (1983) Niemann-Pick disease type B with oculoneural involvement. Indian Pediatrics 20:521-522. Matthews JD, Weiter JJ, Kolodny EH (1986) Macular halos associated with Niemann-Pick type B disease. Ophthalmology 93:933-937. Elleder M, Nevoral J, Spicakova V, Hyniova H, Kraus J, Krasny J, et al. (1986) A new variant of sphingomyelinase deficiency (Niemann-Pick): visceromegaly, minimal neurological lesions and low in-vivo degradation rate of sphingomyelin. J Inher Metab Dis 9:357-366. Dubois G, Mussini J-M, Auclair M, Battesti J, Boutry J-M, Kemeny J-L, et al. (1990) Adult sphingomyelinase deficiency: report of 2 patients who initially presented with psychiatric disorders. Neurology 40:132-136. Pavlu-Pereira H, Asfaw B, Poupetova H, Ledvinova J, Sikora J, Vanier M, et al. (2005) Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study. J Inherit Metab Dis 28:203-227. Wasserstein M, Aron A, Brodie S, Simonaro C, Desnick R, McGovern M (2006) Acid sphingomyelinase deficiency: prevalence and characterization of an intermediate phenotype of Niemann-Pick disease. J Pediatr; 149:554-9 Harzer K, Rolfs A, Bauer P, Zschiesche M, Mengel E, Backes J, et al. (2003) Niemann-Pick disease disease types A and B are clinically but also enzymatically heterogeneous: pitfall in the laboratory diagnosis of sphingomyelinase deficiency associated with the mutation Q292K. Neuropediatrics 34:301-306. äÛ˛Ï‰ÊË‚ Ñ, Åӷӯ‚ÒÍË ã (1979) èËÌÓÒ Í˙Ï ËÁÛ˜‡‚‡ÌÂÚÓ Ì‡ ·ÓÎÂÒÚÚ‡ ̇ çËχÌ-èËÍ ˜ÂÁ ÔÓÛ˜‚‡Ì ̇ ‰ÌÓ ÒÂÏÂÈÒÚ‚Ó è‰ˇÚËfl 18:382-391 Simonaro CM, Desnick RJ, McGovern MM, Wasserstein MP, Schuchman EH (2002) The demographics and distribution of type B Niemann-Pick Disease: novel mutations lead to new genotype/phenotype correlations. Am J Hum Genet 71:1413-1419. Wasserstein M, Desnick R, Schuchman E, Hossain S, Wallenstein S, Lamm C, et al. (2004) The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study. Pediatrics 114; 672-677 McGovern MM, Wasserstein MP, Aron A, Desnick RJ, Schuchman EH, Brodie SE (2004) Ocular manifestations of Niemann-Pick disease type B. Ophthalmology 111:1424-1427. Horinouchi K, Erlich S, Perl DP, Ferlinz K, Bisgaier CL, Sandhoff K, et al. (1995) Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease. Nat Genet 10:288-293. Sperl W, Bart G, Vanier M, Christomanou H, Baldissera I, Steichen-Gersdorf E, et al. (1994) A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate. J Inher Metab Dis 17:93-103.

éË„Ë̇ÎÌË ÒÚ‡ÚËË êÄÅÑéåàéãàáÄ ë èêàçéë çÄ ÑÇÄ ëãìóÄü – êÄáãàóçÄ ÖíàéãéÉàü, èÄíéÉÖçÖáÄ à ãÖóÖçàÖ, ëÇöêáÄçà ë êÄáãàóÖç àáïéÑ å. äÎËÒÛÒÍË, Ö. LJÒË΂‡, Å. ÑÂÎËÈÒ͇1, ü. ïËÒÚÓ‚, Ñ. Äڇ̇ÒÓ‚‡, ñ. ŇΉ‚2, ñ.ÇÂÎÍÓ‚ÒÍË2, å. åË·ÌÓ‚‡ ä‡Ú‰‡ ÔÓ Ì‚ÓÎÓ„Ëfl, åì – ëÓÙËfl, åÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” – ëÓÙËfl, äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl, 1äÎËÌË͇ ÔÓ ÌÂÙÓÎÓ„Ëfl, 2éÚ‰ÂÎÂÌË ÔÓ ıÂÏӉˇÎËÁ‡ SUMMARY TWO CASES OF RHABDOMYOLYSIS WITH DIFFERENT ETIOLOGY, PATHOGENESIS, TREATMENT AND FINAL OUTCOME M. Klissurski, Ö. Vassileva, B. Deliiska1, Y. Hristov, D. Atanasova, Tz. Baldev2, Tz. Velkovski2, M.Milanova We describe the case history, clinical picture and examinations of two patients with rhabdomyolysis (RML), who were treated differently and had different outcome. The first patient was 72-year old man with severe quadriparesis due to a toxic RML complicated with acute renal failure and successfully treated with haemodialysis. The second patient was 36-yer old man with prolonged pulmonary TBC infection, formation of abscess and sepsis which lead to RML acute renal failure, metabolic disturbances and subsequent multiple organ failure. The different predisposing factors, history, clinical and laboratory data and therapeutic options for this rare and serious disorder were compared and discusses KEY WORDS: rhabdomyolysis, acute renal failure, intensive care.

êÖáûåÖ è‰ÒÚ‡‚Â̇  ‡Ì‡ÏÌÂÁ‡Ú‡, ÍÎËÌ˘̇ڇ ͇ÚË̇, Ô‡‡ÍÎËÌ˘ÌËÚ ËÁÒΉ‚‡ÌËfl Ë ÚÂ‡ÔËflÚ‡ ÔË ‰‚‡ ÒÎÛ˜‡fl ̇ ÚÂÊ͇ êåã, ÍÓËÚÓ ÔÓÚ˘‡Ú Ò ‡Á΢ÂÌ ËÁıÓ‰. è˙‚ËflÚ ÒÎÛ˜‡È  72 „Ӊ˯ÂÌ ·ÓÎÂÌ Ò ‡Á‚ËÚË ̇ Í‚‡‰ËÔ‡ÂÁ‡ ÔË ÚÂÊ͇ ÚÓÍÒ˘̇ êåã, ÛÒÎÓÊÌÂ̇ Ò Í‡ÚË̇ڇ ̇ ÓÒÚ‡ ·˙·˜̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (éÅç), Ë ÛÒÔ¯ÌÓ ÍÓÏÔÎÂÍÒÌÓ Î˜ÂÌËÂ, ‚Íβ˜‚‡˘Ó ıÂÏӉˇÎËÁ‡. ÇÚÓËflÚ Ô‡ˆËÂÌÚ Â 36 „Ӊ˯ÂÌ ·ÓÎÂÌ Ò ·ÂÎÓ‰Ó·ÂÌ íÅä ÔÓˆÂÒ, ‰Ó‚ÂÎ ‰Ó ÙÓÏË‡Ì ̇ ‡·ÒˆÂÒ, ÔÓÒΉ‚‡˘ ÒÂÔÒËÒ Ë êåã ‚ ÔÓÒΉÌËÚ ‰ÌË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. èË ÌÂ„Ó ‚˙ÁÌËÍ‚‡ éÅç Ë ·˙ÁÓ Ì‡Ô‰‚‡˘‡ ÔÓÎËÓ„‡Ì̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ò Ù‡Ú‡ÎÂÌ Á‡‚˙¯ÂÍ. ë‡‚Ìfl‚‡Ú ÒÂ Ë Ò ‰ËÒÍÛÚË‡Ú ‡Á΢ÌËÚ Ô‰‡ÁÔÓ·„‡˘Ë, ‡Ì‡ÏÌÂÒÚ˘ÌË Ë ÍÎËÌ˘ÌË Ù‡ÍÚÓË, ̇˜Ë̇ ̇ ΘÂÌËÂ Ë Ôӂ‰ÂÌËÂ, ͇ÍÚÓ Ë Ô˘ËÌËÚ Á‡ Í‡ÈÌËfl ËÁıÓ‰ ÔË ÚÓ‚‡ fl‰ÍÓ Ò¢‡ÌÓ ÒÂËÓÁÌÓ Á‡·ÓÎfl‚‡ÌÂ. äãûóéÇà Ñìåà: ‡·‰ÓÏËÓÎËÁ‡, ÓÒÚ‡ ·˙·˜̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ËÌÚÂÌÁË‚ÌÓ Î˜ÂÌËÂ.


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

‰ÂÍÂÏ‚Ë, 2007

ìÇéÑ ê‡·‰ÓÏËÓÎËÁ‡Ú‡ (êåã)  Á‡·ÓÎfl‚‡ÌÂ Ò ‡Á΢̇ ÂÚËÓÎÓ„Ëfl, ÔË ÍÓÂÚÓ ‚˙ÁÌËÍ‚‡ ‡ÁÍ˙Ò‚‡Ì ̇ ̇Ô˜ÌÓ Ì‡·‡Á‰ÂÌËÚ ÏÛÒÍÛÎÌË ‚·Í̇ Ò ËÁÎË‚‡Ì ̇ ÔÓÚÂ̈ˇÎÌÓ ÚÓÍÒ˘ÌË ÍÎÂÚ˙˜ÌË ÍÓÏÔÓÌÂÌÚË ‚ ÒËÒÚÂÏÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌË (1-28). ÑÂÒÚÛ͈ËflÚ‡ ̇ ÒÓχÚ˘̇ڇ ÏÛÒÍÛ·ÚÛ‡, ÍÓflÚÓ Ô‰ÒÚ‡‚Îfl‚‡˘‡ 40-50% ÓÚ Ó·˘‡Ú‡ ÚÂÎÂÒ̇ χ҇, Ò Ò¢‡ fl‰ÍÓ Í‡ÚÓ Ô˙‚˘ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÔÓ‡‰Ë „ÓÎÂÏËÚ „ÂÌÂ‡ÚÓÌË ‚˙ÁÏÓÊÌÓÒÚË Ë ÛÒÚÓȘ˂ÓÒÚ Ì‡ ÏËÓˆËÚËÚ Í˙Ï ‡Á΢ÌË Ô‡ÚÓÎӄ˘ÌË ‚ÎËflÌËfl (21). ä‡ÈÌËflÚ Ó·˘ Ô˙Ú Ì‡ êåã  ۂ‰‡ ̇ ͇ΈË‚‡Ú‡ ÏËÓˆËÚ̇ ıÓÏÂÓÒÚ‡Á‡ (16, 21, 28). íÓ‚‡ ÔÓÚÂ̈ˇÎÌÓ ÓÔ‡ÒÌÓ Ò˙ÒÚÓflÌË ̇ ÏÛÒÍÛÎ̇ڇ Ú˙Í‡Ì Úfl·‚‡ ‰‡ Ò ÔÓÁ̇‚‡ Ë ÎÂÍÛ‚‡ ÓÚ ¯ËÓÍ Í˙„ ÒÔˆˇÎËÒÚË ÔÓ ‚˙Ú¯̇ ωˈË̇, ‡ÌËχˆËfl, ËÌÚÂÌÁ˂̇ ωˈË̇, Ú‡‚χÚÓÎÓ„Ëfl Ë Ì‚ÓÎÓ„Ëfl, ÔÓ‡‰Ë ‚ËÒÓ͇ڇ ÒÏ˙ÚÌÓÒÚ Ë ÍÓÏÔÎÂÍÒ ÓÚ ÔÓfl‚Ë Ë ÛÒÎÓÊÌÂÌËfl. ñÖã ñÂΠ̇ ̇ÒÚÓfl˘‡Ú‡ ÔÛ·ÎË͇ˆËfl  ‰‡ Ô‰ÒÚ‡‚ËÏ ‡Ì‡ÏÌÂÁ‡Ú‡, ÍÎËÌ˘̇ڇ ͇ÚË̇, Ô‡‡ÍÎËÌ˘ÌËÚÂ, ÂÎÂÍÚÓÙËÁËÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl Ë ÚÂ‡Ô‚Ú˘̇ڇ ÒÚ‡Ú„Ëfl ÔË ‰‚‡ ÒÎÛ˜‡fl ̇ ÚÂÊ͇ êåã. í ËÏ‡Ú ‡Á΢̇ ÂÚËÓÔ‡ÚÓ„ÂÌÂÁ‡, ΘÂÌËÂ Ë ÔÓÚ˘‡Ú Ò ‡Á΢ÂÌ ËÁıÓ‰. è˙‚ËflÚ ÒÎÛ˜‡È  72 „Ӊ˯ÂÌ ·ÓÎÂÌ Ò ‡Á‚ËÚË ̇

256

·ÓÎfl‚‡ÌÂÚÓ ÒË. èË ÌÂ„Ó Ò˙˘Ó Ò ‡Á‚Ë‚‡ éÅç Ë ·˙ÁÓ Ì‡Ô‰‚‡˘‡ ÔÓÎËÓ„‡Ì̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ò Ù‡Ú‡ÎÂÌ Á‡‚˙¯ÂÍ. ë‡‚Ìfl‚‡Ú ÒÂ Ë Ò ‰ËÒÍÛÚË‡Ú ‡Á΢ÌËÚ Ô‰‡ÁÔÓ·„‡˘Ë, ‡Ì‡ÏÌÂÒÚ˘ÌË Ë ÍÎËÌ˘ÌË Ù‡ÍÚÓË, ̇˜Ë̇ ̇ ΘÂÌËÂ Ë Ôӂ‰ÂÌËÂ, ͇ÍÚÓ Ë Ô˘ËÌËÚ Á‡ Í‡ÈÌËfl ËÁıÓ‰ ÔË ÚÓ‚‡ fl‰ÍÓ Ò¢‡ÌÓ ÒÂËÓÁÌÓ Á‡·ÓÎfl‚‡ÌÂ. äãàçàóÖç ëãìóÄâ ‹1 (èÄñàÖçí ã.ç.ã., àá ‹ 14273 éí 06.08.2007, àá ‹ 15104 éí 26.08.2007) 72-„Ӊ˯ÂÌ Ô‡ˆËÂÌÚ, ÍÓÈÚÓ Â ·ËÎ ‚ ‰Ó·Ó Ó·˘Ó Ò˙ÒÚÓflÌËÂ, Ò˙Ò Á‡Ô‡ÁÂ̇ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓıӉ͇,  ÔÓ‚ÂÎ ‚ÂÎÓÂ„ÓÏÂÚ˘ÂÌ ÚÂÒÚ Ì‡ 01.08.07 ÔÓ ÔÓ‚Ó‰ ÚËÍÎÓÌÓ‚‡ ·ÓÎÂÒÚ ‚ ͇‰ËÓÎӄ˘̇ ÍÎËÌË͇. ç‡ 01.08.07 ‚ ÒΉӷ‰ÌËÚ ˜‡ÒÓ‚Â Ò ÔÓfl‚Ë· Ò··ÓÒÚ Ë ·ÓÎÂÁÌÂÌÓÒÚ ‚ ·Â‰Â̇ڇ ÏÛÒÍÛ·ÚÛ‡ ‰‚ÛÒÚ‡ÌÌÓ, ÍÓflÚÓ Á‡ ‰‚‡ ‰ÌË Ó·ı‚‡Ì‡Î‡ Ë ‰‚ÂÚ ÔÓ‰·Â‰ËˆË Ë Ï˯ÌˈË. éÚ 05.08.07 ·ÓÎÌËflÚ Ì ÏÓÊÂÎ ‰‡ ıÓ‰Ë Ò‡ÏÓÒÚÓflÚÂÎÌÓ Í‡ÍÚÓ Ë Ò ÔÓÏÓ˘ÌË Ò‰ÒÚ‚‡, ÔÓ‡‰Ë Ò··ÓÒÚ ‚ ‰ÓÎÌËÚ Í‡ÈÌËˆË Ë Ô‡‰Ì‡Î ‰‚ÛÍ‡ÚÌÓ ÔË ÓÔËÚ Á‡ ÒÚ‡‚‡ÌÂ. ç  ËχΠÁ‡ „‡‰ÂÌÂ, „·‚Ó·ÓÎËÂ Ë ÔÓ‚˙˘‡ÌÂ. ç Ò˙Ó·˘‡‚‡ Á‡ ËÌÙÂ͈ËÓÁÌÓ Á‡·ÓÎfl‚‡ÌÂ, Ôӂ˯Â̇ ÚÂÏÔÂ‡ÚÛ‡, Ûı‡Ô‚‡Ì ÓÚ Í˙ÎÂÊ, ÁÎÓÛÔÓÚ·‡ Ò ‡ÎÍÓıÓÎ ËÎË Ï‰Ë͇ÏÂÌÚË, ÔÂÁ ÔÓÒΉÌËÚ ÏÂÒˆË. åË̇ÎË Á‡·ÓÎfl‚‡ÌËfl: àÅë, ÚËÍÎÓÌÓ‚‡ ·ÓÎÂÒÚ Ò˙ÒÚÓflÌË ÒΉ ÒÚÂÌÚË‡Ì – Ï‡È 2007, ‡ÚÂË-

퇷Îˈ‡ 1. äÎËÌ˘ÌË Ë Ô‡‡ÍÎËÌ˘ÌË ÔÓ͇Á‡ÚÂÎË ÔË ‰‚‡Ï‡Ú‡ ·ÓÎÌË Ò êåã. ï‡‡ÍÚÂËÒÚË͇ / èÓ͇Á‡ÚÂÎ Ç˙Á‡ÒÚ è‰¯ÂÒÚ‚‡˘Ë ·ÓÎÂÒÚË Ë ÓÔÂ‡ÚË‚ÌË Ì‡ÏÂÒË ç‡˜‡ÎÓ Á‡ Á‡·ÓÎfl‚‡ÌÂÚÓ 3 ‰ÌË Ò˙Ò ÌÓ‚‡ Ò··ÓÒÚ ‚ ÔÓÍÒËχÎ̇ڇ ÏÛÒÍÛ·ÚÛ‡ ̇ Í‡Í‡Ú‡ èËÂÏ Ì‡ Ô‰¯ÂÒÚ‚‡˘Ë ωË͇ÏÂÌÚË ‰Ó 2 ÏÂÒˆ‡ àÌÙÂ͈Ëfl / íÂÏÔÂ‡ÚÛ‡ ÄÌÂÏËfl / àÌÚÓÍÒË͇ˆËfl ç‚ÓÎӄ˘ÂÌ ÒÚ‡ÚÛÒ ÔË Ô‰ÒÚ‡‚flÌÂÚÓ ‚ ÍÎËÌË͇ڇ É‡ÙËfl / äí ̇ ÅÑë ‡·ÒˆÂÒ ÖåÉ èää ëìÖ / ëRP ëêä LDH ASAT / ALAT ä‡ÚËÌËÌ ìÂfl ä+ / Na+ èËÍӘ̇ ÍËÒÂÎË̇ éÅç ëÂÒÔËÒ äÓ‡„ÛÎÓÔ‡ÚËfl àÅÇ ïÖåéÑàÄãàáÄ ä‡ÂÌ ËÁıÓ‰

臈ËÂÌÚ ‹ 1 72 „ Äï, àÅë, ÒÚÂÌÚË‡Ì ̇ ÍÓÓ̇ÌË ‡ÚÂËË, Ö̉ÓÔÓÚÂÁ‡ ̇ ‰flÒ̇ Ú‡Áӷ‰Â̇ ÒÚ‡‚‡, Ñá éÒÚÓ Á‡ 2 ‰Â̇

臈ËÂÌÚ ‹2 36 „ OÚ 1,5 ÏÂÒˆ Ëχ ÌÂÛÚÓ˜ÌÂÌÓ ÂÚËÓÎӄ˘ÌÓ ËÌÙËÎÚ‡ÚË‚ÌÓ Á‡ÒÂ̘‚‡Ì ‚ ·ÂÎËfl ‰Ó· èÓÒÚÂÔÂÌÌÓ – 20 ‰ÌË, Ò Ïˇ΄Ëfl Ë ÓÒÚÓ ÓÚ

plavix, atenolol, zocor, norvasc, isodinit, , ·ËÎÍË Ë·ÂÌÓ Ï‡ÒÎÓ ç ç è‰ËÏÌÓ ÔÓÍÒËχÎ̇ ÔÂËÙÂ̇ Í‚‡‰ËÔ‡ÂÁ‡, ååí 2/5 çÓχ

tercef, tavanik, tubocin, rimicid, isoniazid

ꇉËÍÛÎÓÌ‚ËÚ̇ Û‚‰‡ ÅÂÁ ÓÒÓ·ÂÌË ÔÓÏÂÌË 25-40 ÏÏ / 4,8 Ï„/‰Î 79613 8146 432 / 345 436 52 6,14 – 8,4 / 135-141 819 ч 1-2 ÒÚ ç ç ç ч 5-Í‡ÚÌÓ Ç˙ÁÒÚ‡ÌÓ‚fl‚‡ÌÂ

èÓÎË‡‰ËÍÛÎÓÌ‚ÓÚ̇ Û‚‰‡ - ÚÂÊ͇ ã‚ÍÓˆËÚÓÁ‡ Ò Ó΂fl‚‡Ì 98 ÏÏ 80848 13308 2385 / 319 264 17,6 8,4 / 126 ч ч ç ч ч - ‰ÌÓÍ‡ÚÌÓ ãÂÚ‡ÎÂÌ

Í‚‡‰ËÔ‡ÂÁ‡ ÔË ÚÂÊ͇ ÚÓÍÒ˘̇ êåã, ÛÒÎÓÊÌÂ̇ Ò Í‡ÚË̇ڇ ̇ ÓÒÚ‡ ·˙·˜̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (éÅç), Ë ÛÒÔ¯ÌÓ ÍÓÏÔÎÂÍÒÌÓ Î˜ÂÌËÂ, ‚Íβ˜‚‡˘Ó ıÂÏӉˇÎËÁ‡. ÇÚÓËflÚ Ô‡ˆËÂÌÚ Â 36 „Ӊ˯ÂÌ ·ÓÎÂÌ Ò ÔÓÚ‡ıË‡Ì ·ÂÎÓ‰Ó·ÂÌ ‚˙ÁÔ‡ÎËÚÂÎÂÌ ÔÓˆÂÒ, ‰Ó‚ÂÎ ‰Ó ÙÓÏË‡Ì ̇ ‡·ÒˆÂÒ, ÔÓÒΉ‚‡˘ ÒÂÔÒËÒ Ë êåã ‚ ÔÓÒΉÌËÚ ‰ÌË Ì‡ Á‡-

ч ç ëËÏÂÚ˘̇ ‚fl· Í‚‡‰ËÔ‡ÂÁ‡, ååí 1/5 ÅÂÎÓ‰Ó·ÂÌ ‚˙ÁÔ‡ÎËÚÂÎÂÌ ÔÓˆÂÒ, ‚˙ıÓ‚

‡Î̇ ıËÔÂÚÓÌËfl 2 ÒÚ., ‰ÛÓ‰Â̇Î̇ flÁ‚‡. ë˙ÒÚÓflÌË ÒΉ ẨÓÔÓÚÂÁË‡Ì ̇ ‰flÒ̇ Ú‡Áӷ‰Â̇ ÒÚ‡‚‡ - 2005 „. á‡ı‡ÂÌ ‰Ë‡·ÂÚ Ò ‰‡‚ÌÓÒÚ ÓÚ ‰‚‡ ÏÂÒˆ‡, ̇ ‰ËÂÚ‡. èËÂχÌË Ï‰Ë͇ÏÂÌÚË: ÁÓÍÓ, ‡ÚÂÌÓÎÓÎ, ËÁÓ‰ËÌËÚ, Ô·‚ËÍÒ, ÌÓ‚‡ÒÍ, Ë·ÂÌÓ ÓÎËÓ, ‡Á΢ÌË ·ËÎÍË. ëÓχÚ˘ÂÌ ÒÚ‡ÚÛÒ: ãÂÍÓ Û‚‰ÂÌÓ Ó·˘Ó Ò˙ÒÚÓflÌËÂ,


‰ÂÍÂÏ‚Ë, 2007

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

‡Ù·ËÂÎÂÌ, ÚÂÎÂÒ̇ χ҇ 105 Í„, ˙ÒÚ 175 ÒÏ. óËÒÚÓ ‚ÂÁËÍÛ·ÌÓ ‰Ë¯‡Ì ‰‚ÛÒÚ‡ÌÌÓ. êËÚÏ˘̇ Ò˙‰Â˜Ì‡ ‰ÂÈÌÓÒÚ ÔÛÎÒ 78 Û./ÏËÌ., RR-120/65. äÓÂÏ ÏÂÍ Ì·ÓÎÂÁÌÂÌ. ä‡ÈÌËˆË ÎÂÍÓ ÓÚÓ˜ÌË ·ÂÁ ‚ÂÌÓÁÌË ÔÓÏÂÌË. éÚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ: Çfl· Í‚‡‰ËÔ‡ÂÁ‡ ‰Ó Ô΄Ëfl Á‡ Í‡Í‡Ú‡, ÏÛÒÍÛÎ̇ ÒË· ‚ ˜ÂÚËËÚ Í‡ÈÌË͇ ÔÓ ååí 1/5 ÔÓÍÒËχÎÌÓ Á‡ Í‡Í‡Ú‡, 2,5/5 Á‡ ˙ˆÂÚ ‰ËÒÚ‡ÎÌÓ; Ô‡ÎÔ‡ÚÓ̇ ·ÓÎÂÁÌÂÌÓÒÚ ÔÓ ÏÛÒÍÛ·ÚÛ‡ ̇ ‰‚ÂÚ ·Â‰‡ Ë Ô‰Ï˯ÌˈË, ÍÓÎÂÌ̇ Ë ‡ıËÎÓ‚‡ ‡ÂÙÎÂÍÒËfl, Ô‡ÚÓÎӄ˘ÌË ÓÚ „. ̇ Ň·ËÌÒÍË ÎËÔÒ‚‡Ú ‰‚ÛÒÚ‡ÌÌÓ. äÓÓ‰Ë̇ˆËfl Ë ÒÂÚË‚ÌÓÒÚ - Ò˙ı‡ÌÂÌË. íêî Ë Çäî – ·.Ó. èӂ‰ÂÌË ËÁÒΉ‚‡ÌËfl Ë ÍÓÌÒÛÎÚ‡ˆËË: ï· 123-95, ïÚ 0.34, ÖËÚÓˆËÚË 3.53, ΂ÍÓˆËÚË 13.3-11.3, ÚÓÏ·ÓˆËÚË 213 ıËÎfl‰Ë, èíÇ 79%, ÄëÄí 67-299-97 - 34 U/L, ÄãÄí 275-345-138-42 U/L, ëêä 53506-58103-62241-79613-232633643-283 U/L, ãÑç 6981-4979-7108-8146-1225-694 U/L, Í‡ÚËÌËÌ 240-313.2-435-354-195 µmol/l, ÛÂfl 26.1-3746.5 mmol/l, ÚÓÔÓÌËÌ ÓÚˈ‡ÚÂÎÂÌ, ͇ÎËÈ 6.14-6.81-8.46.0-3.46 mmol/l, CRP 4.8; ÛË̇ Ú˙ÏÌÓ Í‡ÙÂÌË͇‚Ó-˜Â‚ÂÌË͇‚‡: ç 5, ÒÔˆ. Ú„ÎÓ 1015, ·ÂÎÚ˙Í /++/, Á‡ı‡ /-/, ÍÂÚÓ Ú· /-/, ÛÓ·ËÎËÌÓ„ÂÌ ÌÓχ, ·ËÎËÛ·ËÌ /-/, Í˙‚ /+/, ‚ Ò‰ËÏÂÌÚ‡: Ô‡‚‡Ê ÓÚ ÂËÚÓˆËÚË Ë Î‚ÍÓˆËÚË. àÁΉ‚‡Ì ̇ ÎËÍ‚Ó – ·ÂÁˆ‚ÂÚÂÌ, ΂ÍÓˆËÚË 3/3, ÂËÚÓˆËÚË 0/3, è‡Ì‰Ë /+/, ÍÓÎ. ·ÂÎÚ˙Í 27.4 Ï„%, ÓÍÒË ï· 0 ‰, ·ËÎËÛ·ËÌ 0 ‰. ÑËÙÂÂ̈ˇÎÌÓ ·ÓÂÌ ̇ ÎËÍ‚Ó – ÎËÏÙÓˆËÚË 32%, ‡ÍÚË‚ÌË ÎËÏÙÓˆËÚË 8%, ÏÓÌÓˆËÚË 52%, ÂÚËÍÛÎÓˆËÚË 4%, Ò„ÏÂÌÚË 4%. ëÂÛÏ ÓÚˈ‡ÚÂÎÂÌ Á‡ ÎÂÔÚÓÒÔËÓÁ‡. äÓÍÒ‡ÍË ‚ËÛÒË ÓÚˈ‡ÚÂÎ̇ ̇ıӉ͇ ‚ ÒÂÛχ. ïÂÏÓÍÛÎÚÛË Ë ÛÓÍÛÎÚÛ‡ – ÚËÍ‡ÚÌÓ ÓÚˈ‡ÚÂÎÌË. êÂÌÚ„ÂÌ Ì‡ ·flÎ ‰Ó· Ë Ò˙ˆÂ – ·ÂÁ ‰‡ÌÌË Á‡ ‡ÍÚË‚ÌË Ô‡ÚÓÎÓ˘ÌË ÔÓÏÂÌË. ÖåÉ ‰‡ÌÌË Á‡ Ì‚Ó„ÂÌ̇ ‡ÚÓÙËfl – ‡‰ËÍÛÎÓÌ‚ËÚ̇ Û‚‰‡. ç Ò „ËÒÚË‡ ‰ÂÌÂ‚‡ˆËfl. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰‡ÌÌË Á‡ ÏËÓ„ÂÌ̇ Û‚‰‡. è‡ÔËÎË Ò flÒÌË „‡ÌËˆË Ì‡ ÌË‚ÓÚÓ Ì‡ ÂÚËÌËÚÂ, ‡Ì„ËÓÒÍÎÂÓÁËÒ ÂÚËÌ ıËÔÂÚÓÌË͇. ìá ̇ ÍÓÂÏÌË Ó„‡ÌË - ‰ÂÒÂÌ ·˙·ÂÍ 101 ÏÏ, Îfl‚ 106 ÏÏ. Ç ‰ÂÒÂÌ ·˙·ÂÍ Ì‡Î˘̇ ÂıÓ„ÂÌ̇ ÍËÒÚ‡ Ò ÎÂÍÓ Ôӂ˯Â̇ ÔÎ˙ÚÌÓÒÚ. Ç Ô˙‚ËÚ ‰ÌË ÓÚ ÔÂÒÚÓfl ‚ ÍÎËÌË͇ڇ Ò ۂÂ΢Ëı‡ ÒÚÓÈÌÓÒÚËÚ ̇ ëêä ‰Ó 79613 U/L. èÓ‡‰Ë ‡Á‚ËÚËÂÚÓ Ì‡ éÅç Ò Ì‡Ï‡ÎÂÌË ̇ ‰ËÛÂÁ‡Ú‡, Ôӂ˯ÂÌË ̇ ÒÂÛÏÌËfl ͇ÎËÈ ‰Ó 8.5 mmol/l, Í‡ÚËÚËÌ ‰Ó 435.7 µmol/l Ë ÛÂfl ‰Ó 51.9 mmol/l, ÔË ÌÂ„Ó Ò Ôӂ‰ ıÂÏӉˇÎËÁÌÓ Î˜ÂÌËÂ Ò ‰Ó·Ó ÔÓ‚ÎËfl‚‡Ì ̇ ··Ó‡ÚÓÌËÚ ÔÓ͇Á‡ÚÂÎË Ë Ó·‡ÚÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÏÛÒÍÛÎ̇ڇ Ò··ÓÒÚ (퇷Îˈ‡ Ë îË„Û‡ 1). èӂ‰ÓıÏ ÍÓÌÒÂ‚‡ÚË‚ÌÓ Î˜ÂÌËÂ Ò ‚ÎË‚‡ÌËfl ̇ ÙËÁËÓÎӄ˘ÂÌ ÒÂÛÏ, 5% „βÍÓÁ‡, ÒÂÛÏ „βÍÓÁÂ, ËÌ„Â ·ÍÚ‡Ú 2-3 ÎËÚ‡ ‰Ì‚ÌÓ, NaHCO3, mannitol, furanthril, plavix, atenolol, penicillin 24 MIU, heparin 2 x 2500 UI, norvasc, isodinit, methylprednisolon ÓÚ 150 Ï„ ‚ ÌËÒıÓ‰fl˘‡ ‰ÓÁ‡ 5 ‰ÌË Ë Âı‡·ËÎËÚ‡ˆËfl. ÅÓÎÌËflÚ Ò ËÁÔËÒ‡ ·ÂÁ ·ÓÎÍË, ‡Ù·ËÎÂÌ, ‡‰ÂÍ‚‡ÚÂÌ, Ò ‚˙ÁÒÚ‡ÌÓ‚Â̇ ÏÛÒÍÛÎ̇ ÒË· ‚ ˜ÂÚËËÚ Í‡ÈÌË͇ ‰Ó ååí 3,5-4/5 Ô‰ËÏÌÓ ÔÓÍÒËχÎÌÓ. 臈ËÂÌÚ˙Ú ÏÓʯ ‰‡ Òfl‰‡ ‚ ΄ÎÓÚÓ Ë Ëχ¯Â ‡ÍÚË‚ÌË ‰‚ËÊÂÌËfl ‚ ˜ÂÚËËÚ Í‡ÈÌË͇. ç‡ÒÓ˜ÂÌ ·Â¯Â Á‡ ÔÓ‰˙Îʇ‚‡Ì ̇ ΘÂÌËÂÚÓ Ë ÔÓÒΉfl‚‡Ì ̇ Ò˙ÒÚÓflÌËÂÚÓ ‚ ÌÂÙÓÎӄ˘̇ ÍÎËÌË͇, Í˙‰ÂÚÓ Âı‡·ËÎËÚ‡ˆËflÚ‡ ÔÓ‰˙Îʇ‚‡ Ë ÔÂÚ ‰ÌË ÔÓ-Í˙ÒÌÓ ÚÓÈ Â ËÁÔËÒ‡Ì Ò˙Ò Ò‡ÏÓÒÚÓflÚÂÎ̇ χ͇ Ë ·‡‚̇ ÔÓıӉ͇ ‰Ó 50 Ï. ÅÓÎÌËflÚ Ì Ò ÌÛʉ‡Â¯Â ÓÚ ÔÓ‰˙Îʇ‚‡Ì ̇ ‰Ë‡ÎËÁÌÓÚÓ Î˜ÂÌËÂ, Ú˙È Í‡ÚÓ ÒÂÛÏÌËflÚ Í‡ÚËÌËÌ ÒÔ‡‰‡ ‰Ó 186 µmol/l, ÛÂflÚ‡ ‰Ó 7,2 mmol/l. ëÚÓÈÌÓÒÚËÚ ̇ ëêä ÒÔ‡‰Ì‡ı‡ ‰Ó 217, ˜ÂÌÓ‰Ó·ÌËÚ ÂÌÁËÏË Ò ÌÓχÎËÁË‡ı‡. äãàçàóÖç ëãìóÄâ ‹2 (èÄñàÖçí Ä.Ñ.ä., àá ‹ 12192 éí 29.06.2007) 36 „Ӊ˯ÂÌ Ô‡ˆËÂÌÚ ·ÂÁ ÓÒÓ·ÂÌË ÏË̇ÎË Ë Ò˙Ô˙ÚÒÚ‚‡-

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˘Ë Á‡·ÓÎfl‚‡ÌËfl, ÍÓÈÚÓ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ Ï‡È 2007 ÔÓÎÛ˜‡‚‡ ÒÚfl„‡Ì ‚ „˙‰ËÚÂ. ëΉ ÂÌÚ„ÂÌÓÎӄ˘ÌÓ ËÁÒΉ‚‡Ì ̇ ·ÂÎËÚ ‰Ó·Ó‚Â Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú ‰‡ÌÌË Á‡ ËÌÙËÎÚ‡ÚË‚ÌÓ Á‡ÒÂ̘‚‡Ì ̇ ‰flÒÌÓ Ò‰ÌÓ ·ÂÎÓ‰Ó·ÌÓ ÔÓÎÂ, Ò ÔÓÏÂÌË Ò˙ÓÚ‚ÂÚÒÚ‚‡˘Ë ̇ Ô˙‚˘ÂÌ íÅä ÍÓÏÔÎÂÍÒ. Ç ‰ËÙÂÂ̈ˇÎÌÓ ÓÚÌÓ¯ÂÌË  ӷÒ˙‰Â̇ ·‡ÍÚÂˇÎ̇ ÔÌ‚ÏÓÌËfl. èÓ‚ÂÎ Â ‡Ï·Û·ÚÓÌÓ ‰ÂÒÂÚ ‰Ì‚ÌÓ Î˜ÂÌËÂ Ò ÚÂˆÂÙ Ë ‰‚ÛÒ‰Ï˘ÌÓ Ò Ú‡‚‡ÌËÍ, Ë ÓÒÂωÌ‚ÌÓ Ò ÚÛ·ÓˆËÌ 600 Ï„ Ë ËÏˈˉ 300 Ï„ ‰Ì‚ÌÓ. èÂÁ ÚÓ‚‡ ‚ÂÏ Ò ÔÓfl‚ËÎË ·ÓÎÍË ÔÓ ÏÛÒÍÛÎËÚ ̇ Í‡ÈÌˈËÚÂ, Ó·˘‡ ÛÏÓ‡ Ë Ò··ÓÒÚ, ÔÓ-ËÁ‡ÁÂ̇ Á‡ ÔÓÍÒËχÎ̇ڇ ÏÛÒÍÛ·ÚÛ‡ ̇ Í‡Í‡Ú‡. ç‡ 04.06.2007  Ôӂ‰Â̇ äí ̇ ·flÎ ‰Ó· Ë ·ÓÌıÓÒÍÓÔËfl Ò Î‡‚‡Ê. êÂÁÛÎÚ‡Ú˙Ú Â ÓÚˈ‡ÚÂÎÂÌ Á‡ íÅä. èÂÁ ÒΉ‚‡˘ËÚ 20 ‰ÌË ÒËÏÔÚÓÏËÚ ÔÓ„ÂÒË‡ÎË Ò ÛÒË΂‡Ì ̇ ·ÓÎÍËÚÂ Ë Ò··ÓÒÚÚ‡ ‚ Í‡ÈÌˈËÚÂ. ç‡ 26.06.2007  ıÓÒÔËÚ‡ÎËÁË‡Ì Ò˙Ò Ò˙ÏÌÂÌË Á‡ ËÌÙËÎÚ‡ÚË‚ÌÓ ÔÌ‚ÏÓÌ˘̇ ÙÓχ ̇ ÚÛ·ÂÍÛÎÓÁ‡, ͇ÚÓ Â Á‡ÔӘ̇ÚÓ Ë ÔÓÚË‚ÓÚÛ·ÂÍÛÎÓÁÌÓ Î˜ÂÌËÂ Ò Ú‡·ÎÂÚÍË ÚÛ·ÓˆËÌ Ë ËÁÓÌˉ. ë˙˘ËflÚ ‰ÂÌ ‚˙ÁÌËÍ‚‡ Ú‡ıˉËÒÔÌÂfl, ‡ÌÛËfl, ÚÂÊ͇ Í‚‡‰ËÔ‡ÂÁ‡ Ë ‰ËÒÙ‡„Ëfl. ëÚÓÈÌÓÒÚÚ‡ ̇ ÒÂÛÏ̇ڇ ëêä  6000 U/L. ëΉ ÍÓÌÒÛÎÚ‡ˆËfl Ò Ì‚ÓÎÓ„, ·ÓÎÌËflÚ Â ‰Ó͇‡Ì ÔÓ ÒÔ¯ÌÓÒÚ ‚ ËÌÚÂÌÁË‚ÌÓÚÓ ÓÚ‰ÂÎÂÌË ̇ ÍÎËÌË͇ڇ ÔÓ Ì‚ÓÎÓ„Ëfl ÔË åÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇” - ëÓÙËfl. ëÓχÚ˘ÌÓ ‚ Í‡ÈÌÓ ÚÂÊÍÓ Û‚‰ÂÌÓ Ò˙ÒÚÓflÌËÂ, ÍÓÌÚ‡ÍÚÂÌ, ÒÛ·Ù·ËÎÂÌ, Ò Ú‡ıˉËÒÔÌÂfl Ë ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ËÌÚÛ·Ë‡Ì ‚ ÔÓÚË‚Ó¯ÓÍÓ‚‡ Á‡Î‡, ̇ ‡Ô‡‡ÚÌÓ ‰Ë¯‡ÌÂ, ÔÛÎÒ 128, êê 130/90. éÚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ: ëêåÑ ÎËÔÒ‚‡, óåç ‚ ÌÓχ. íÂÊ͇ ‚fl· Í‚‡‰ËÔ‡ÂÁ‡ ÔÓ-ËÁ‡ÁÂ̇ ÔÓÍÒËχÎÌÓ, ‰Ó Ô΄Ëfl Á‡ Í‡Í‡Ú‡, ÔË ÏÛÒÍÛÎ̇ ıËÔÓÚÓÌËfl. ÄÂÙÎÂÍÒËfl Á‡ Í‡ÈÌˈËÚÂ, ·ÂÁ Ô‡ÚÓÎӄ˘ÌË ÂÙÎÂÍÒË ÓÚ „ÛÔ‡Ú‡ ̇ Ň·ËÌÒÍË. ëÂÚË‚ÌÓÒÚÚ‡ Ë ÍÓÓ‰Ë̇ˆËflÚ‡ Ì ҇ ÔˆËÁË‡ÌË, ÔÓ‡‰Ë ÚÂÊÍÓÚÓ Ò˙ÒÚÓflÌË ̇ ·ÓÎÌËfl. ä‡ÚÂÚÂËÁË‡Ì Ò ‡ÌÛËfl. Çäî - ËÁÔ˙ÎÌfl‚‡ ̇Âʉ‡ÌËfl. èӂ‰ÂÌË ËÁÒΉ‚‡ÌËfl Ë ÍÓÌÒÛÎÚ‡ˆËË (퇷Îˈ‡ 1): ÖËÚÓˆËÚË 6,82; ï· 155; ïÚ 0,45; ÚÓÏ·ÓˆËÚË 146; ΂ÍÓˆËÚË 20,7, èíÇ 77% /INR 1,15/ ̇ÚËÈ 126 mmol/l, ͇ÎËÈ 8,4 mmol/l; ıÎÓ 100 mmol/l, ASAT 2385 U/L, ALAT 319 U/L, CPK 80848 U/L, LDH 13308 U/L; ‡ÏË·Á‡ 75 U/L; Ó·˘ ·ÂÎÚ˙Í 43; ‡Î·ÛÏËÌ 24,5; „βÍÓÁ‡ 9,47 mmol/l; ÛÂfl 17,6 mmol/l, Í‡ÚËÌËÌ 264 µmol/l. êÂÌÚ„ÂÌÓ„‡ÙËflÚ‡ ̇ ·flÎ ‰Ó· ÔÓ͇Á‚‡ Á‡ÒÂ̘‚‡Ì ̇ ‰flÒÌÓ Ò‰ÌÓ ·ÂÎÓ‰Ó·ÌÓ ÔÓÎÂ. äÓÌÒÛÎÚ‡ˆËfl Ò Í‡‰ËÓÎÓ„ ̇‰Í‡ÏÂ̇ Ú‡ıË͇‰Ëfl, ·ÂÁ ê-‚˙ÎÌË, ‚˙ÁÏÓÊÌÓ Ô‰Ò˙‰ÌÓ Ï˙ʉÂÌÂ Ò Ä-V ·ÎÓÍ 2:1, ÌÂÔ˙ÎÂÌ ãÅÅ. äÓÌÒÛÎÚ‡ˆËfl Ò ÌÂÙÓÎÓ„: Ô‡ˆËÂÌÚ˙Ú Â ·ÂÁ ‡Ì‡ÏÌÂÁ‡ Á‡ Ô‰ıÓʉ‡˘Ó ·˙·˜ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ̇Îˈ  ÓÎË„Ó-‡ÌÛËfl Ò ÌÂËÁ‚ÂÒÚ̇ ‰‡‚ÌÓÒÚ, ıËÔÂ͇ÎËÂÏËfl Ë Ò˙ÓÚ‚ÂÚÌË ÖäÉ ÔÓÏÂÌË. èË ·ÓÎÌËfl  Ôӂ‰Â̇ ‰ÌÓÍ‡Ú̇ ÒÔ¯̇ ıÂÏӉˇÎËÁ‡ Ë ËÌÚÂÌÁË‚ÌÓ Î˜ÂÌËÂ Ò ‚ÎË‚‡ÌËfl ̇ ‚Ó‰ÌÓ-ÒÓÎÂ‚Ë Ë Á‡ı‡ÌË ‡ÁÚ‚ÓË, ˆËÔËÌÓÎ, ‡Ô‡‡Ú̇ ‚ÂÌÚË·ˆËfl. èӂ‰ Ò ‰ËÌÒÚ‚ÂÌÓ ÂÎÂÍÚÓÌ‚Ó„‡ÙËfl Ò ÔÓ‚˙ıÌÓÒÚÌË ÂÎÂÍÚÓ‰Ë ÌÂÔÓÒ‰ÒÚ‚ÂÌÓ ÔÂ‰Ë ÒÏ˙ÚÌËfl ËÁıÓ‰. àÁÒΉ‚‡ÌÂÚÓ ÔÓ͇Á‡ ‰‡ÌÌË Á‡ ÚÂÊÍÓ ËÁ‡ÁÂ̇ ÔÂËÙÂÌÓ-ÒÚ‚ÓÎÓ‚‡ Û‚‰‡ ̇ ÌÂ‚ËÚ ̇ ˜ÂÚËËÚ Í‡ÈÌË͇ – ÒÏÂÒÂÌ ÚËÔ. Ä̇ÏÌÂÁ‡Ú‡ Á‡ ·ÓÎÍË Ë Ò··ÓÒÚ ÔÓ ÏÛÒÍÛÎËÚÂ, ÂÌÚ„ÂÌÓ‚‡Ú‡ Ë äí ̇ıӉ͇ ̇ ·flÎ ‰Ó·, ‰‡ÌÌËÚ Á‡ ÒÛ·Ù·ËÎËÚÂÚ, ΂ÍÓˆËÚÓÁ‡ Ò Ó΂fl‚‡ÌÂ, Ô‡‡ÍÎËÌ˘ÌËÚÂ Ë ÖåÉ ÔÓÏÂÌËÚ ÌË ‰‡‚‡Ú ÓÒÌÓ‚‡ÌË ‰‡ ÔËÂÏÂÏ ËÌÙÂ͈ËÓÁÌÓ ÒÂÔÚ˘ÌÓ Ò˙ÒÚÓflÌË ÔË ‚˙ÁÏÓÊ̇ ·ÂÎÓ‰ӷ̇ íÅä, Ò ÔÓÒΉ‚‡˘Ó ‚˙ÁÌËÍ‚‡Ì ̇ ‰Ëı‡ÚÂÎ̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ë êåã (ÒÚÓÈÌÓÒÚ Ì‡ ëêä ‰Ó 80000 U/L), ÔÓÎËÌ‚ÓÔ‡ÚËfl Ë ÔÓÎËÓ„‡Ì̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ. 䂇‰ËÔ‡ÂÁ‡Ú‡ Ò ‚ÎÓ¯Ë ‰Ó Í‚‡‰ËÔ΄Ëfl, éÅç ‰Ó ‡ÌÛËfl, ıËÔÂ͇ÎËÂÏËfl, ıËÔÓ̇ÚËÂÏËfl Ë ẨÓÚÓÍÒ˘ÂÌ ¯ÓÍ. ÑËı‡ÚÂÎ̇ڇ Ë Ò˙‰Â˜ÌÓ-Ò˙‰Ó‚‡ Ò··ÓÒÚ ÔÓ„ÂÒË‡ı‡ Ç˙ÔÂÍË Ôӂ‰ÂÌËÚ ‡ÌËχˆËÓÌÌË ÏÂÓÔËflÚËfl, àÅÇ, ‚Íβ˜‚‡ÌÂÚÓ Ì‡


‰ÂÍÂÏ‚Ë, 2007

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

‰ÓÔ‡ÏËÌ Ë ‡‰Â̇ÎËÌ ‚ÂÌÓÁÌÓ Ë ËÌÚ‡Í‡‰Ë‡ÎÌÓ, Ë̉ËÂÍÚÂÌ Ò˙‰Â˜ÂÌ Ï‡Ò‡Ê Ë ‰ÂÙË·Ë·ˆËfl, ·ÓÎÌËflÚ Ì‡Ô‡‚Ë ÎÂÚ‡ÎÂÌ ËÁıÓ‰ ÌflÍÓÎÍÓ ˜‡Ò‡ ÒΉ ÔËÂχÌÂÚÓ ‚ ÍÎËÌË͇ڇ. èË Ô‡ÚӇ̇ÚÓÏ˘ÌÓÚÓ ËÁÒΉ‚‡Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÚÂÊÍÓ ËÁ‡ÁÂ̇ ËÌÙËÎÚ‡ÚË‚ÌÓ ÔÌ‚ÏÓÌ˘̇ ·ÂÎÓ‰ӷ̇ ÚÛ·ÂÍÛÎÓÁ‡. Ñàëäìëàü êåã  ҂˙Á‡Ì‡ Ò Û‚Âʉ‡ÌÂ Ë ÌÂÍÓÁ‡ ̇ ÏÛÒÍÛÎËÚÂ Ë ÓÚ‰ÂÎflÌ ̇ ÏÛÒÍÛÎÌË ÔÓÚÂËÌË, ÏËÓ„ÎÓ·ËÌ Ë ‰Û„Ë ÍÎÂÚ˙˜ÌË ‚¢ÂÒÚ‚‡, ‚Íβ˜ËÚÂÎÌÓ Í‡ÚËÌÍË̇Á‡ ‚ ÒËÒÚÂÏ̇ڇ ˆËÍÛ·ˆËfl, ͇ÚÓ ÔË ÓÔ‰ÂÎÂÌË ÛÒÎÓ‚Ëfl Ú ÔˆÂÔËÚË‡Ú ‚ ·˙·ˆËÚÂ Ë Ô˘ËÌflÚ éÅç (3, 17, 18, 21, 28). Ç ÏÂı‡ÌËÁχ ̇ Û‚Âʉ‡ÌËflÚ‡ Û˜‡ÒÚ‚‡Ú Ó˘Â Ì‡ÚÛÔ‚‡Ì ̇ ‚˙ÚÂÍÎÂÚ˙˜ÂÌ Í‡ÎˆËÈ, ‡ÍÚË‚Ë‡Ì ̇ ÔÓÚ‡ÁË Ë ÎËÔ‡ÁË, ÓÚ‰ÂÎflÌ ̇ Ò‚Ó·Ó‰ÌË ‡‰Ë͇ÎË Ë ËÌÙËÎÚ‡ˆËfl Ò ‚˙ÁÔ‡ÎËÚÂÎÌË ÍÎÂÚÍË. ç‡ÒÚ˙Ô‚‡ÌÂÚÓ Ì‡ ÏËÓ„ÎÓ·ÛÎËÌÛËÌÓ‚‡ éÅç ˜ÂÒÚÓ Â Ò‚˙Á‡ÌË Ò ÏËÓ„ÎÓ·Ë̇, ÓÚ‰ÂÎÂÌ ÓÚ ÏÛÒÍÛÎËÚÂ Ë ıËÔÓ‚ÓÎÂÏËfl Ë ·˙·˜̇ ıËÔÓÔÂÙÛÁËfl. (15,16,21). ãÂ͇ڇ Ë ÛÏÂÂ̇ڇ êåã Ô˘ËÌfl‚‡Ú ÏÂÚ‡·ÓÎËÚÌË Ì‡Û¯ÂÌËfl ͇ÚÓ ıËÔÂ̇ÚËÂÏËfl, ıËÔÂ͇ÎËÂÏËfl, ıËÔÂÙÓÒÙ‡ÚÂÏËfl, ıËÔÓ͇ΈÂÏËfl, ·ÍÚ‡ˆË‰ÓÁ‡ Ë ıËÔÂÛËÍÂÏËfl, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ Ò Á‡ÒËÎflÚ Ò ÔÓfl‚‡Ú‡ ̇ éÅç. èË ÔÓ-ÚÂÊÍËÚ Ò˙ÒÚÓflÌËfl ÏÓÊ ‰‡ Ò ‰ÓÒÚË„Ì ‰Ó ‰Ó ÎÂÚ‡ÎÂÌ ËÁıÓ‰ (7, 10, 12). óÂÒÚË ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ êåã Ò‡ ‡ÎÍÓıÓÎ̇ ÁÎÓÛÔÓÚ·‡ (67%), ÏÂÍÓÚ˙͇Ì̇ ÍÓÏÔÂÒËfl (39%), ÙËÁ˘ÂÒÍÓ ÔÂ̇ÚÓ‚‡‚‡Ì Ë/ËÎË „˙˜Ó‚‡ ‡ÍÚË‚ÌÓÒÚ (5-24%), Ú‡‚χ (17-38%), ÎÂ͇ÒÚ‚Â̇ ÁÎÓÛÔÓÚ·‡ (7-15%), ÏÛÒÍÛÎ̇ ËÒıÂÏËfl (14%), ÏÂÚ‡·ÓÎËÚÌË Ì‡Û¯ÂÌËfl (8%), ıËÔÓÚÂÏËfl (4%), „ËÔÓÔÓ‰Ó·ÌÓ Á‡·ÓÎfl‚‡Ì (3%), ÒÂÔÒËÒ (23%), „‡Ì„Â̇ (1%), ÔÓÎËÏËÓÁËÚ (8%), ËÁ„‡flÌ (5%), ıÂ‰ËÚ‡ÌË Á‡·ÓÎfl‚‡ÌËfl (3%) (10, 28). èÓ̇ÒÚÓfl˘ÂÏ ÌÂÚ‡‚χÚ˘ÌËÚ Ô˘ËÌË Á‡ êåã Ò‡ ÔÂÚ Ô˙ÚË ÔÓ-˜ÂÒÚË ÓÚ ÔÓÒÚÚ‡‚χÚ˘ÌËÚ (18, 27). êåã Ô‰ËÁ‚ËÍ‚‡ 715% ÓÚ ÒÎÛ˜‡ËÚ ̇ éÅç ‚ ëÄô, ͇ÚÓ 30-45% ÓÚ ·ÓÎÌËÚÂ Ò êåã ÒÚË„‡Ú ‰Ó éÅç (7). èÓ ‰ÂÙËÌˈËfl, Á‡ ‰‡ Ò ÔÓÒÚ‡‚Ë ‰Ë‡„ÌÓÁ‡ êåã, Úfl·‚‡ ‰‡ Ëχ ÒÚÓÈÌÓÒÚË Ì‡ ëêä 5-10 ̇‰ ÌÓχڇ, ÚËÔ˘ÌË ÒËÏÔÚÓÏË Ë ËÒÍÓ‚Ë Ù‡ÍÚÓË ÓÚ ‡Ì‡ÏÌÂÁ‡Ú‡ (10, 16, 27). é·ËÍÌÓ‚ÂÌÓ ëêä  ̇��� 6000 U/L ÔË ‰Ë‡Ô‡ÁÓÌ ÓÚ 10000 ‰Ó 200 000 ÔË Ï‡ÍÒËχÎÌÓ ÓÔËÒ‡ÌË ÒÚÓÈÌÓÒÚË ÓÚ 3 ÏËÎËÓ̇ U/L (20). çflχ ‰Û„Ó Á‡·ÓÎfl‚‡ÌÂ, ÔË ÍÓÂÚÓ ëêä ‰‡ ‰ÓÒÚË„Ì ڇÍË‚‡ ‚Â΢Ë̇. ê‡ÌÌËÚ ÍÎËÌ˘ÌË ÔÓfl‚Ë Ì‡ êåã Ò‡ ÌÂÒÔˆËÙ˘ÌË - ÏÛÒÍÛÎ̇ Ò··ÓÒÚ Ë ·ÓÎÂÁÌÂÌÓÒÚ, „ÂÌÂ‡ÎËÁË‡Ì‡ ÛÏÓ‡, ÚÂÏÔÂ‡ÚÛ‡ Ë „ËÔÓÔÓ‰Ó·ÌË ÔÓfl‚Ë, „‡‰ÂÌÂ Ë ‰. (10, 12, 28). èË 50% ÓÚ ·ÓÎÌËÚ ‚ ̇˜‡ÎÓÚÓ Ì‡ êåã Ëχ ÏÛÒÍÛÎÌË ·ÓÎÍË (10), ÔË 97% Ò˙Ó·˘‡‚‡Ú Á‡ ÔÓÌ 1 ËÒÍÓ‚ Ù‡ÍÚÓ, ‡ 59% Á‡ ÏÌÓÊÂÒÚ‚Ó ËÒÍÓ‚Ë Ù‡ÍÚÓË Á‡ êåã. íÂÁË ÒËÏÔÚÓÏË ÎÂÒÌÓ ÏÓ„‡Ú ‰‡ Ò ÔÓÔÛÒÌ‡Ú Ë Ì‰ÓÓˆÂÌflÚ, ÓÒÓ·ÂÌÓ ÔË ÚÂÊÍÓ ·ÓÎÌË Ô‡ˆËÂÌÚË (2, 7, 8, 12). äÎËÌ˘ÌËÚ ÔÓÒΉÒÚ‚Ëfl ̇ êåã ‚Íβ˜‚‡Ú ÒΉÌËÚ ÓÒÌÓ‚ÌË Ò˙ÒÚÓflÌËfl: ıËÔÓ‚ÓÎÂÏËfl (ÔÓ‡‰Ë ÒÂÍ‚ÂÒÚ‡ˆËfl ̇ Ô·ÁÏÂÌÓÚÓ Ò˙‰˙ʇÌË ̇ ‚Ó‰‡ ‚ Û‚‰ÂÌËÚ ÏËÓˆËÚË), ıËÔÂ͇ÎËÂÏËfl, ÏÂÚ‡·ÓÎËÚ̇ ‡ˆË‰ÓÁ‡ (ÓÒ‚Ó·Óʉ‡‚‡ ̇ ÍÎÂÚ˙˜ÌË ÙÓÒÙ‡ÚË Ë ÒÛÎÙ‡ÚË), ÓÒÚ‡ ·˙·˜̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ (éÅç, ÔÓ‡‰Ë ÌÂÙÓÚÓÍÒ˘ÌËÚ ÂÙÂÍÚË Ì‡ ÏËÓ„ÎÓ·Ë̇ Ë ‰Û„ËÚ ‡ÁÔ‡‰ÌË ÏËÓˆËÚÌË ÔÓ‰ÛÍÚË) Ë ‡Á‚ËÚË ̇ ËÌÚ‡‚‡Á‡Î̇ ‰ËÒÂÏËÌË‡Ì‡ ÍÓ‡„ÛÎÓÔ‡ÚËfl (DIC-ÒË̉ÓÏ) (7). èË ‰‚‡Ï‡Ú‡ ̇¯Ë ·ÓÎÌË Ì‡·Î˛‰‡‚‡ıÏÂ Ë ÚËÚ ÛÒÎÓÊÌÂÌËfl ̇ êåã, ·ÂÁ ‡Á‚ËÚËÂÚÓ Ì‡ Ñàä ÒË̉ÓÏ. ÖÚËÓÎӄ˘ÌËÚ Ô˘ËÌË Á‡ ‡Á‚ËÚË ̇ êåã Ò‡ ÏÌÓ„Ó·ÓÈÌË, ÌÓ Ì‡È-˜ÂÒÚËÚ ҇ ‰ËÂÍÚ̇ ÏÛÒÍÛÎ̇ Ú‡‚χ; ÙËÁ˘ÂÒÍÓ ÔÂ̇ÚÓ‚‡‚‡Ì (χ‡ÚÓÌ, ÂÔËÎÂÔÚ˘ÂÌ ÒÚ‡ÚÛÒ, ÚÂÊ͇ ‰ËÒÚÓÌËfl, ÓÒÚ‡ ÔÒËıÓÁ‡); ÏÛÒÍÛÎ̇ ËÒıÂ-

258

ÏËfl; ÚÓÍÒËÍÓÎӄ˘ÌË, ÓÚÓ‚Ë Ë ÏËÓÚÓÍÒ˘ÌË ÒÛ·Òڇ̈ËË, ͇ÚÓ Âthanol, Ïethanol, Âthylene glycol, ‚˙„ÎÂÓ‰ÂÌ ÓÍËÒ, toluene, isopropanol, ıÂÓËÌ, Ïethadone, ‚arbiturates, Òocaine, ‡mphetamine, hencyclidine (MDMA, ecstasy), LSD; ÓÚÓ‚Ë ÓÚ Ô‡flˆË, ÁÏËË, ÓÒË; ÏÂÚ‡·ÓÎËÚÌË, ÂÎÂÍÚÓÎËÚÌË Ë ẨÓÍËÌÌË Ì‡Û¯ÂÌËfl (ıËÔÓ- ËÎË ıËÔÂ̇ÚËÂÏËfl, ıËÔÓ͇ÎËÂÏËfl, ıËÔÓÙÓÒÙ‡Ú‚ÏËfl, ıËÔÓ- Ë ıËÔÂÚËÓˉËÁ˙Ï, ‰Ë‡·ÂÚ̇ ÍÂÚÓ‡ˆË‰ÓÁ‡, ÌÂÍÂÚÓ„ÂÌ̇ ıËÔÂÓÒÏÓ·̇ ‰Ë‡·ÂÚ̇ ÍÓχ); ËÌÙÂ͈ËÓÁÌË (·‡ÍÚÂËË Francisella tularensis, Streptococcus pneumoniae, Group B streptococci, Streptococcus pyogenes, Staphylococcus epidermidis, Escherichia coli, Borrelia burgdorferi, Clostridium perfringens, Clostridium tetani, Viridans streptococci, Plasmodium, Rickettsia, Salmonella, Listeria, Legionella, Mycoplasma, Vibrio, Brucella, Bacillus, Leptospira; ‚ËÛÒË – „ËÔÌË ‚ËÛÒË ÚËÔ A Ë B (̇È-˜ÂÒÚÓ), HIV, ÍÓÍÒ‡ÍË, EBV, Echovirus, Parainfluenza virus, VZV, CMV, Adenovirus, HSV, Ë ÌflÍÓË „˙·Ë˜ÌË ‡„ÂÌÚË, ͇ÚÓ Candida species Aspergillus), ËÏÛÌÓÎӄ˘ÌË, Ò‚˙Á‡ÌË Ò Ù‡ÍÚÓË ÓÚ ÓÍÓÎ̇ڇ Ò‰‡ – Ô„fl‚‡ÌÂ, Óı·ʉ‡ÌÂ, ÂÎÂÍÚ˘ÂÒÍÓ ËÁ„‡flÌÂ, Û‰‡‚flÌÂ Ë ÔÓ fl‰ÍÓ ÔË ÌflÍÓË Ì‡ÒΉÒÚ‚ÂÌË Á‡·ÓÎfl‚‡ÌËfl. çflÍÓË ÓÚ ÎÂ͇ÒÚ‚‡Ú‡ Ò‚˙Á‡ÌË Ò êåã Ò‡ ‡ÌÚËıËÒÚ‡ÏËÌË, Ò‡ÎˈË·ÚË, ÍÓÙÂËÌ, ÙË·‡ÚË, clofibrate, fenofibrate, gemfibrozil, Ì‚ÓÎÂÔÚˈË, ‡ÌÂÒÚÂÚˈË, propofol, amphotericin B, ıËÌËÌ, ÍÓÚËÍÓÒÚÂÓˉË, ÒÚ‡ÚËÌË - ‡torvastatin, fluvastatin, lovastatin, pitavastatin, pravastatin, rosuvastatin, simvastatin, cerivastatin, theophylline, ‡ÌÚˉÂÔÂÒ‡ÌÚË ÓÔÂ‡ÚË‚ÌË ËÌÚÂ‚Â̈ËË, ÓÒÓ·ÂÌÓ ÔË ·ÓÎÌË Ò Ì‡‰ÌÓÏÂÌÓ Ú„ÎÓ, ÚÓÍÒ˘ÌË ÔÓ‰ÛÍÚË ÓÚ Ë·ÌË ÔÓ‰ÛÍÚË Ë ‰. (1-6, 8, 10, 13, 14, 16-19, 23-26). èË Ô‡ˆËÂÌÚ ‹1 ÒΉ Ôӂ‰ÂÌËÚ ÍÓÏÔÎÂÍÒÌÓ ËÁÒΉ‚‡ÌÂ Ë ÏÌÓ„Ó·ÓÈÌË ÍÓÌÒÛÎÚ‡ˆËË, Ì ÓÚÍËıÏ ‰Û„‡ Ô˘Ë̇ Á‡ ‡Á‚ËÚË ̇ êåã ÓÒ‚ÂÌ ÍÓÏ·ËÌË‡ÌÂÚÓ Ì‡ ÚË Ù‡ÍÚÓ‡: 1) ÛÔÓÚ·‡ ̇ ÏÌÓÊÂÒÚ‚Ó ÎÂ͇ÒÚ‚‡, ÏÂÊ‰Û ÍÓËÚÓ Ë ÁÓÍÓ, Ô·‚ËÍÒ Ë ÔËÂÏ ‚ ‰Óχ¯ÌË ÛÒÎÓ‚Ëfl ̇ ı‡ÌËÚÂÎ̇ ‰Ó·‡‚͇, Ò˙‰˙ʇ˘‡ Ë·ÂÌÓ Ï‡ÒÎÓ 2) ̇‰ÂÍ‚‡ÚÌÓ Á‡ ‚˙Á‡ÒÚÚ‡ ÙËÁ˘ÂÒÍÓ ÔÂ̇ÚÓ‚‡‚‡Ì (‚ÂÎÓÂ„ÓÏÂÚËfl, ͇ÚÓ ÒÔÓ‰ ‡Ì‡ÏÌÂÁ‡Ú‡ ·ÓÎÌËflÚ Â ÔÓ‚ÚÓËÎ ÚÂÒÚ‡ Ò Í‡‡Ì ̇ ÍÓÎÂÎÓ ‚ ‰Óχ ÒË Ò˙˘Ëfl ‰ÂÌ ‚˜ÂÚ‡ – ÍÎËÌ˘ÌÓ ÏÛÒÍÛ·ÚÛ‡Ú‡ ̇ Í‡Í‡Ú‡ ÏÛ ·Â¯Â Á‡Ò„̇ڇ ̇È-ÏÌÓ„Ó) Ë 3) Ô‚‰ÂÌË ÒÍÓÓ ‰‚ ÓÔÂ‡ÚË‚ÌË Ì‡ÏÂÒË, Ô˙‚‡Ú‡ ÒÚÂÌÚË‡Ì Á‡ àÅë Ò ÚËÍÎÓÌÓ‚‡ ·ÓÎÂÒÚ Ë ‚ÚÓ‡Ú‡ – ẨÓÔÓÚÂÁË‡Ì ̇ ‰flÒ̇ Ú‡Áӷ‰Â̇ ÒÚ‡‚‡. íÓ‚‡, ˜Â ÔÓ ÎËÚÂ‡ÚÛÌË ‰‡ÌÌË (22, 26) ÔËÎÓÊÂÌËÂÚÓ Ì‡ ÒÚ‡ÚËÌË Â Ò‚˙Á‡ÌÓ Ò ‡Á‚ËÚË ̇ êåã, Ì ÓÁ̇˜‡‚‡ ˜Â ÔË Ì‡¯Ëfl ·ÓÎÂÌ Ëχ flÒÌÓ ‰Ó͇Á‡Ì‡ Ô˘ËÌÌÓ ÒΉÒÚ‚Â̇ ‚˙Á͇ ωË͇ÏÂÌÚ / ·ÓÎÂÒÚ. éÚ˜ÂÚÂ̇  êåã Ë éÅç ÔË ÔË·„‡Ì ̇ ÒËÏ‚‡ÒÚ‡ÚËÌ, ‡ÏËÓ‰‡ÓÌ Ë ‡Ú‡Á‡Ì‡‚Ë. êËÒÍ˙Ú ÓÚ êåã Ò Á‡ÒË΂‡ ÔË Â‰ÌÓ‚ÂÏÂÌÌÓ ÔË·„‡Ì ̇ ωË͇ÏÂÌÚË, ÍÓËÚÓ ÔÓ‰ÚËÒÍ‡Ú ÏÂÚ‡·ÓÎËÁχ ̇ ÒËÏ‚‡ÒÚ‡ÚË̇. èÓÒΉÌËflÚ Ò ÏÂÚ‡·ÓÎËÁË‡ ˜ÂÁ CYP3A4. è‡‚‡ÒÚ‡ÚËÌ, ÙÎÛ‚‡ÒÚ‡ÚËÌ Ë ÓÒÛ‚‡ÒÚ‡ÚËÌ Ò‡ Ò Ì‡È-ÌËÒ˙Í ËÒÍ ÓÚ ÎÂ͇ÒÚ‚ÂÌË ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ëfl, ‡ÚÓ‚‡ÒÚ‡ÚËÌ Â Ò ÛÏÂÂÌ ËÒÍ, ‰Ó͇ÚÓ ÒËÏ‚‡ÒÚ‡ÚËÌ Ë ÎÓ‚‡ÒÚ‡ÚËÌ Ò‡ Ò Ì‡È-‚ËÒÓÍ ËÒÍ ÓÚ Ì··„ÓÔËflÚÌË ‚Á‡ËÏÓ‰ÂÈÒÚ‚Ëfl Ë ·Ë Úfl·‚‡ÎÓ ‰‡ Ì Ò ÔË·„‡Ú ÔË ·ÓÎÌË, ÔËÂχ˘Ë ËÌıË·ËÚÓË Ì‡ CYP3A4 (11). ëÔÓ‰ ÔÓÛ˜‚‡ÌÂÚÓ PHARMO ·‡ÁË‡ÌÓ ‚˙ıÛ Ì‡‰ 2 ÏËÎËÓ̇ ‰‡Ú˜‡ÌË, ÒÎÛ˜‡ËÚ ̇ ıÓÒÔËÚ‡ÎËÁ‡ˆËË ÔÓ ÔÓ‚Ó‰ Ìa ÏËÓÔ‡ÚËfl, êåã, éÅç Ë ˜ÂÌÓ‰Ó·ÌË Û‚Âʉ‡ÌËfl Ò‡ ÔÓ‰ 1 ̇ 3000 ‰Û¯Ë Á‡ „Ó‰Ë̇ (11, 22, 26). Ç ‰ËÙÂÂ̈ˇÎÌÓ ‰Ë‡„ÌÓÒÚ˘ÂÌ ÔÎ‡Ì ÔË Ô‡ˆËÂÌÚ ‹1 Ò ӷÒ˙‰Ë ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ÓÒÚ˙ ÔÓÎË‡‰ËÍÛÎÓÌ‚ËÚ ÚËÔ ÉËÎÂÌ-ŇÂ, ıËÔÂ͇ÎËÂÏ˘̇ èçè, ÔÓÎËÏËÓÁËÚ, ÚÓÍÒ˘̇ èçè, ÔÓÙËËÌÓ‚‡ èçè. àÌÙÂ͈ËÓÁ̇ „ÂÌÂÁ‡ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ ·Â¯Â ÓÚı‚˙ÎÂ̇ ÔÓ‡‰Ë ÎËÔÒ‡Ú‡ ̇ ËÌÙÂ͈ËÓÁ̇ ‡Ì‡ÏÌÂÁ‡, Ù·ËÎËÚÂÚ, ÚÓÍÒÓ-ËÌÙÂ͈ËÓÁÂÌ


‰ÂÍÂÏ‚Ë, 2007

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

ÒË̉ÓÏ, ËÁ‡ÁÂ̇ ΂ÍÓˆËÚÓÁ‡ Ò Ó΂fl‚‡ÌÂ, Ë Ì„‡ÚË‚ÌË ÏËÍÓ·ËÓÎӄ˘ÌË ÔÓÒ‚ÍË ÓÚ ÒÂÛÏ Ë ÎËÍ‚Ó. êåã ÔË Ô‡ˆËÂÌÚ ‹2  Ò˙Ò ÒÂÔÚ˘ÌÓ-ËÌÙÂ͈ËÓÁ̇ „ÂÌÂÁ‡. ìÚÂÊÌfl‚‡˘ Ô‡ÚÓ„ÂÌÂÚ˘ÂÌ ÏÂı‡ÌËÁ˙Ï ÔË ÌÂ„Ó ·Â¯Â ÌÂÔÓ‚ÎËfl̇ڇ ÓÚ ‡ÌÚË·ËÓÚËˆË Ë ÚÛ·ÂÍÛÎÓÒÚ‡ÚËˆË íÅä ËÌÙÂ͈Ëfl, Ò ‡Á‚ËÚË ̇ ÒÂÔÒËÒ, ıËÔÓ̇ÚËÂÏËfl Ë ÔÓÎËÓ„‡Ì̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ, ˜‡ÒÚ ÓÚ ÍÓflÚÓ Â Ë Û‚‰‡ ̇ ÒÍÂÎÂÚ̇ڇ ÏÛÒÍÛ·ÚÛ‡ Ò Í‡ÚË̇ ̇ êåã, ıËÔÂ͇ÎËÂÏËfl Ë éÅç. èÓ‡‰Ë Í‡ÚÍËfl ÔÂÒÚÓÈ ‚ ÍÎËÌË͇ڇ Ì ÛÒÔflıÏ ‰‡ ‰Ó͇ÊÂÏ ÌflÍÓÈ ÓÚ ËÁ·ÓÂÌËÚ ÔÓ-„Ó ÂÚËÓÎӄ˘ÌË Ô˘ËÌËÚÂÎË. í‚˙‰Â ‚˙ÁÏÓÊÌÓ Â ‚˙ÁÔ‡ÎËÚÂÎÌËflÚ ·ÂÎÓ‰Ó·ÂÌ ÔÓˆÂÒ ÔË ÌÂ„Ó ‰‡  ҂˙Á‡Ì Ò ‡ÚËÔ˘ÂÌ íÅä ‡„ÂÌÚ, Ú˙È Í‡ÚÓ Ì  ËχÎÓ ÂÙÂÍÚ ÓÚ Ôӂ‰ÂÌÓÚÓ ¯ËÓÍÓÒÔÂÍÚ˙ÌÓ ‡ÌÚË·ËÓÚ˘ÌÓ Ë ÚÛ·ÂÍÛÎÓÒÚ‡Ú˘ÌÓ Î˜ÂÌË ÔÂ‰Ë êåã. EåÉ ÔÓÏÂÌË ÔË ·ÓÎÌË Ò êåã Ì ҇ ÓÔËÒ‚‡ÌË ‚ ̇ÒÍÓÓ ‚ ·˙΄‡Ò͇ڇ ÎËÚÂ‡ÚÛ‡ (1). èË Ô˙‚Ëfl ·ÓÎÂÌ Ë„ÎÓ‚‡Ú‡ ÖåÉ Â Ì‡Ô‡‚Â̇ ̇ 6-fl ‰ÂÌ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ÓÔ·͂‡ÌËflÚ‡, ÔË Í‚‡‰ËÔ‡ÂÁ‡ ååí 1/5 Á‡ Í‡Í‡Ú‡ Ë 2,5/5 ‰ËÒÚ‡ÎÌÓ Á‡ Ô˙ÒÚËÚ ̇ ˙ˆÂÚÂ, ëêä 58103 U/L, ä+ 6,14 mmol/l Ë Í‡ÚËÌËÌ 240 µmol/l. ÖåÉ ‰ÂÏÓÌÒÚË‡ ÒËÎÌÓ Ì‡Ï‡ÎÂÌË ÏÓÚÓÌË Â‰ËÌˈË, Á‡·‡‚Â̇ ÔÓ‚Ó‰ËÏÓÒÚ ÔÓ ‰‚Ë„‡ÚÂÎÌËÚÂ Ë ÏÓÚÓÌË ‚·Í̇ ÓÚ ÒÏÂÒÂÌ ÚËÔ, ̇χÎÂÌË ‰Ó ÎËÔÒ‚‡˘Ë F-‚˙ÎÌË ÓÚ Í‡Í‡Ú‡, ·ÂÁ ̇΢ˠ̇ ‰ÂÌÂ‚‡ˆËfl. èË ‚ÚÓËfl ·ÓÎÂÌ Ì ÏÓʇ ‰‡ Ò ÓÒ˙˘ÂÒÚ‚Ë Ë„ÎÂ̇ ÂÎÂÍÚÓÏËÓ„‡ÙËfl Ë Ò˙ÓÚ‚ÂÚÌÓ ‰‡ Ò ‰ÓÍ‡Ê‡Ú ‰‡ÌÌË Á‡ ÏËÓ„ÂÌ̇ Û‚‰‡. àÌÚÂÂÒÂÌ Â Ù‡ÍÚ˙Ú, ˜Â Ì Ò ‰ÂÏÓÌÒÚË‡Ú ‰‡ÌÌË Á‡ ÏËÓ„ÂÌÌÓ Á‡·ÓÎfl‚‡ÌÂ, ‡ Á‡ Ì‚Ó„ÂÌÌÓ Ë ÔË ‰‚‡Ï‡Ú‡ ·ÓÎÌË. è‡ÚÓ„ÂÌÂÚ˘ÌÓ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÚÂÊ͇ ÏËÓÔ‡ÚËfl, ÔÓ‡‰Ë ÏÂÏ·‡ÌÌË Ì‡Û¯ÂÌËfl Ë ‡ÁÍ˙Ò‚‡Ì ̇ ̇Ô˜ÌÓ̇·‡Á‰ÂÌËÚ ÏÛÒÍÛÎÌË ‚·Í̇. ÇÂÓflÚÌÓ ÎËÔÒ‡Ú‡ ̇ ÚËÔ˘ÌË ÖåÉ ÔÓfl‚Ë ÔË ÚÂÁË ·ÓÎÌË Ò ıËÔÂ͇ÎËÂÏËfl, ÏÂÚ‡·ÓÎËÚÌË Ì‡Û¯ÂÌËfl Ë Á‡Òfl„‡ÌÂ Ë Ì‡ èçëÑ ‚Ó‰flÚ ‰Ó Á‡Íβ˜ÂÌË Á‡ ÔÓÎË‡‰ËÍÛÎÓÌ‚ËÚ̇, ‡ Ì ˜ËÒÚÓ ÏËÓ„ÂÌ̇ Û‚‰‡ (1). ë˙˘ÂÒÚ‚ÂÌÓ Á‡ ΘÂÌËÂÚÓ Ì‡ êåã  ıˉ‡ÚË‡ÌÂÚÓ Ì‡ ·ÓÎÌËfl Ë ·Ó·‡Ú‡ Ò ıËÔÓ‚ÓÎÂÏËflÚ‡, ÔËÎÓÊÂÌËÂÚÓ Ì‡ ·Ë͇·Ó̇ÚË, χÌËÚÓÎ, ÔÓ‰‰˙ʇÌÂÚÓ Ì‡ ‡‰ÂÍ‚‡Ú̇ ‰ËÛÂÁ‡, ÏÓÌËÚÓË‡Ì ̇ Ääê, ç ̇ ÛË̇ڇ (8). àχ ÛÒÚ‡ÌÓ‚ÂÌË ÔÓÚÓÍÓÎË Ì‡ ΘÂÌËÂ Ë Ôӂ‰ÂÌË ‚ ‡Á΢ÌËÚ ÂÚ‡ÔË ÓÚ êåã (3, 12, 16 - ˆËÚË‡ R. Muthins, M.Meinninger. Trauma Servce: Rhabdomyolysis Algorithm. Oregon Health & Science University, Portland, Oregon 1998). ëÚÓÈÌÓÒÚÚ‡ ̇ ëêä  ÌÂÓ·ıÓ‰ËÏÓ ‰‡ Ò ÒÎÂ‰Ë Ì‡ 8-12 ˜‡Ò‡ ‰Ó ÚË ÔÓÒΉӂ‡ÚÂÎÌÓ Ì‡Ï‡Îfl‚‡˘Ë ÒÚÓÈÌÓÒÚË, ‰ËÛÂÁ‡Ú‡ ̇ ‚ÒÂÍË ˜‡Ò, ç ̇ ÛË̇ڇ ̇ 4 ˜‡Ò‡ (3, 7, 16, 21). çË ÔÓÒΉfl‚‡ıÏ Âʉ̂ÌÓ èää, ·ËÓıËÏËflÚ‡ Ääê, ÂÎÂÍÚÓÎËÚËÚÂ, ‰ËÛÂÁ‡Ú‡ Ë ÍÓ‡„Û·ˆËÓÌÌËÚ ÔÓ͇Á‡ÚÂÎË ÔË Ì‡¯Ëfl ·ÓÎÂÌ. èËÍ˙Ú Ì‡ ëêä ÔË Ô˙‚Ëfl ̇¯ ·ÓÎÂÌ ·Â¯Â ÔÓ˜ÚË 80 000 (̇ 9-fl ‰ÂÌ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ÓÔ·͂‡ÌËflÚ‡), ͇ÚÓ ÒΉ ‚ÚÓ‡Ú‡ ıÂÏӉˇÎËÁ‡ ÚÓÁË Ï‡ÍÒËÏÛÏ Á‡ÔӘ̇ Á‡ ̇χÎfl‚‡, ͇ÚÓ ÔÓ˜ÌË ‰ÓÒÚ˄̇ ÌÓχڇ ̇ ÔÂÚËfl ‰ÂÌ ÒΉ 5-Ú‡, ÔÓÒΉ̇ ‰Ë‡ÎËÁ̇ Ôӈ‰Û‡. äÎËÌ˘ÌÓÚÓ ÒÚ‡·ËÎËÁË‡Ì ̇ Ô‡ÂÚ˘ÌËÚ fl‚ÎÂÌËfl (Ò··ÓÒÚÚ‡ ‚ ÔÓÍÒËχÎÌËÚ ÏÛÒÍÛÎË Ì‡ Í‡ÈÌˈËÚÂ) Ë ‰ÓË ÎÂÍÓÚÓ ÍÎËÌ˘ÌÓ ÔÓ‰Ó·ÂÌË ̇ ÒË·ڇ Á‡ ˙ˆÂÚ ·Â¯Â ̇·Î˛‰‡‚‡ÌÓ ÔË Ì‡‡ÒÚ‚‡Ì ̇ ÒÚÓÈÌÓÒÚËÚ ̇ ëêä ÓÚ 50000 ‰Ó 79610. ÏÂÊ‰Û 7-10 ‰ÂÌ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ ÒËÏÔÚÓÏËÚÂ. èË Ô‡ˆËÂÌÚ ‹1 ̇È-Ò˙˘ÂÒÚ‚ÂÌÓ Á‡ ÔÂÓ‰ÓÎfl‚‡Ì ̇ ıËÔÂ͇ÎËÂÏËflÚ‡, ÏËÓ„ÎÓ·ËÌÛËflÚ‡ Ë éÅç ·Â¯Â ÔÓ‚Âʉ‡ÌÂÚÓ Ì‡ ıÂÏӉˇÎËÁ‡, ͇ÍÚÓ Â ÔÓ͇Á‡ÌÓ Ì‡ îË„Û‡ 1. ìÔÓÚ·‡Ú‡ ̇ ÍÓÚËÍÓÒÚÂÓË‰Ë ÔË Î˜ÂÌËÂÚÓ Ì‡ êåã  ÒÔÓÌÓ Ë Ìflχ ÓÔ‰ÂÎÂ̇ ‰ÓÁËӂ͇ (8, 20, 27), ÓÚ Â‰Ì‡ ÒÚ‡Ì‡ Ú ÒÚ‡·ËÎËÁË‡Ú ÎËÁÓÁÓÏÌËÚ ÏÂÏ·‡ÌË, Á‡‰˙Ê‡Ú ‚˙ÁÔ‡ÎËÚÂÎÌËÚ ÔÓˆÂÒË Ë ÍÎÂÚ˙˜Ì‡Ú‡ ‰ÂÒÚÛ͈ËflÚ‡, ÌÓ ÓÚ ‰Û„‡ Á‡ÒË΂‡Ú ͇ڇ·ÓÎÌËÚ ÔÓˆÂÒË Ë „ÂÌÂ-

259

‡ˆËfl ̇ ÏÛÒÍÛÎËÚ (23). èË Ô˙‚Ëfl ÓÚ Ì‡¯ËÚ ·ÓÎÌË ÔËÎÓÊËıÏ 5 ‰Ì‚ÌÓ äë ΘÂÌË ÓÚ 120 Ï„ ‚ÂÌÓÁÌÓ Ò ‰Ó·˙ ÂÙÂÍÚ Ë Ô‡‡ÎÂÎÌÓ Ò ÔÓ‰Ó·ÂÌË ̇ Ò··ÓÒÚÚ‡. îË„Û‡ 1. É‡ÙË͇ ̇ ÔÓÏÂÌËÚ ̇ CPK, LDH, K+, Òreatinin Ë ÏÛÒÍÛÎ̇ڇ ÒË· Á‡ Í‡Í‡Ú‡ (ååí – χÌÛ‡ÎÌÓ ÏÛÒÍÛÎÌÓ ÚÂÒÚÛ‚‡ÌÂ) ÔË ·ÓÎÂÌ ‹1 Ò êåã, ÔÓ‰ ‚ÎËflÌË ̇ ΘÂÌËÂÚÓ Ë ıÂÏӉˇÎËÁ‡Ú‡ (ïÑ) ‚˙‚ ‚ÂÏÂÚÓ.

îË„Û‡ 1. É‡ÙË͇ ̇ ÔÓÏÂÌËÚ ̇ CPK, LDH, K+, Òreatinin Ë ÏÛÒÍÛÎ̇ڇ ÒË· Á‡ Í‡Í‡Ú‡ (ååí – χÌÛ‡ÎÌÓ ÏÛÒÍÛÎÌÓ ÚÂÒÚÛ‚‡ÌÂ) ÔË ·ÓÎÂÌ ‹1 Ò êåã, ÔÓ‰ ‚ÎËflÌË ̇ ΘÂÌËÂÚÓ Ë Ôӈ‰ÛË Ì‡ ıÂÏӉˇÎËÁ‡ (ÒÚÂÎÍËÚÂ) ‚˙‚ ‚ÂÏÂÚÓ. èÓ ÓÚÌÓ¯ÂÌË ̇ ÓÔ‡ÒÌÓÒÚÚ‡ ÓÚ ÍÓ‡„Û·ˆËÓÌÌË ÛÒÎÓÊÌÂÌËfl ÔË êåã, ÔË Ô‡ˆËÂÌÚ ‹ 1 ̇·Î˛‰‡‚‡ıÏ ÒÔ‡‰‡Ì ̇ èíÇ ‰Ó 21%, Ë ÓÍÓÎÓ 12 ˜‡Ò‡ ÎÂÍÓ Í˙‚ÂÌ ÓÚ ÏflÒÚÓ Ì‡ ËÌÊÂÍÚË‡Ì ‚˙ıÛ ÍÓʇڇ, ÔÓ ‚ÂÏ ̇ ËÁ‚˙¯‚‡Ì ̇ ïÑ (‰ÓÔ˙ÎÌËÚÂÎÌÓ ËÁÔÓÎÁ‚‡Ì ̇ ıÂÔ‡ËÌ) Ë ÔÓÙË·ÍÚ˘ÌÓ ÔËÎÓÊÂÌË ̇ ıÂÔ‡ËÌ (Á‡ ÔÓÙË·ÍÚË͇ ̇ ÑÇí ÔË ÚÂÊ͇ Ô‡‡Ô‡ÂÁ‡ Ë Ì‡‰ÌÓÏÂÌÓ Ú„ÎÓ, 2 ı 2500 ‰Ì‚ÌÓ) Ë Ô·‚ËÍÒ 75 Ï„ ‰Ì‚ÌÓ. ëΉ ÚˉÌ‚ÌÓ ÒÔË‡Ì ̇ ıÂ‡ÔË̇ Ë Ô·‚ËÍÒ‡ ıÂÏÓ‡„ËËÚ ÒÔflı‡, èíÇ ‰ÓÒÚ˄̇ 71% Ë ÚÂ‡ÔËflÚ‡ Ò Ô·‚ËÍÒ, ͇ÚÓ ÔÓÙË·ÍÚË͇ ̇ àÅë Ë ÒÚÂÌÚÓ‚ÂÚÂ, ·Â¯Â ‚˙ÁÓ·ÌÓ‚Â̇ ·ÂÁ ÛÒÎÓÊÌÂÌËfl. ä‡ÚÓ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ÛÒÎÓÊÌÂÌË ‚ ÎÂ͇ ÒÚÂÔÂÌ ÔË Ì‡¯Ëfl Ô‡ˆËÂÌÚ Ì‡·Î˛‰‡‚‡ıÏ ‡Á‚ËÚË ̇ Ú.̇. Compartment-ÒË̉ÓÏ Ì‡ ˙ˆÂÚÂ, ÔÓ-ËÁ‡ÁÂÌ Á‡ ‰flÒ̇ڇ ˙͇. ÅÓÎÌËflÚ Ò ÓÔ·͂‡¯Â ÓÚ ·ÓÎ͇, ̇Ô„̇ÚÓÒÚ Ë ÔÓ‰Û‚‡Ì ̇ Ú˙͇ÌËÚ ̇ Ô‰Ï˯Ìˈ‡Ú‡ ÓÚ Î‡Í˙Úfl Á‡ Ô˙ÒÚËÚÂ. Ç ÎËÚÂ‡ÚÛ‡Ú‡ ÚÓ‚‡ Ò ӷflÒÌfl‚‡ Ò ÔËÚËÒ͇ÌÂ Ë ÓÚÓÍ Ì‡ ÏÂÍËÚ Ú˙͇ÌË, ÔÓ‡‰Ë ÓÚÓÍ Ë Ì‡Î˘Ë ̇ ÓÒÏÓ·ÂÌ „‡‰ËÂÌÚ Ì‡‚˙Ú Í˙Ï ÏÛÒÍÛÎËÚÂ, Ôӂ˯ÂÌÓ Ì‡Îfl„‡Ì ÏÂÊ‰Û Ù‡ÒˆËËÚÂ, ̇χÎÂÌÓ Í˙‚ÓÒ̇·‰fl‚‡ÌÂ Ë ‚ÂÌÓÁÂÌ Á‡ÒÚÓÈ (16). ÑflÒ̇ڇ ˙͇ ̇ ·ÓÎÌËfl ·Â¯Â ËÁÔÓ-


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

‰ÂÍÂÏ‚Ë, 2007

ÎÁ‚‡Ì‡ Á‡ ÔÓÒÚ‡‚flÌ ̇ ‚ÂÌÓ-‡ÚÂˇÎÌËfl ¯˙ÌÚ, ÌÓ Ò ‰ËÛÂÚ˘̇ڇ ÚÂ‡ÔËfl, ÍÓÏÔÂÒË Ë ïÑ ÚÂÁË fl‚ÎÂÌËfl ÓÚ¯ÛÏflı‡, ·ÂÁ ‰‡ Ò ÒÚË„‡ ‰Ó ıËÛ„˘̇ Ë̈ËÁËfl ͇ÍÚÓ Ò ÓÔËÒ‚‡ ÓÚ ‰Û„Ë ‡‚ÚÓË (18). áÄäãûóÖçàÖ é·˘‡Ú‡ ˜ÂÒÚÓÚ‡ ̇ ÒÏ˙ÚÌÓÒÚ ÔË ·ÓÎÌË Ò êåã  ÔË·ÎËÁËÚÂÎÌÓ 5%; ‚˙ÔÂÍË ˜Â ÔË ‚ÒÂÍË ÍÓÌÍÂÚÂÌ Ô‡ˆËÂÌÚ ÚÓ‚‡ Á‡‚ËÒË ÓÚ ÂÚËÓÎÓ„ËflÚ‡ Ë ‰ÓÔ˙ÎÌËÚÂÎ̇ڇ ÍÓÏÓ·Ë‰ÌÓÒÚ (7, 16). 燂ÂÏÂÌ̇ڇ ‰Ë‡„ÌÓÁ‡, ‡‰ÂÍ‚‡ÚÌÓÚÓ ËÌÙÛÁËÓÌ̇ ΘÂÌËÂ Ë Ôӂ‰ÂÌËÚ ıÂÏӉˇÎËÁÌË Ôӈ‰ÛË Ò‡ Ô‰ÔÓÒÚ‡‚͇ Á‡ ·Î‡„ÓÔËflÚÌËfl ËÁıÓ‰ ÔË Ô‡ˆËÂÌÚ ‹ 1. ç··„ÓÔËflÚÌËflÚ ËÁıÓ‰ ÔË Ô‡ˆËÂÌÚ ‹2, ÍÓÈÚÓ Â ÔÓ-Ï·‰,  ҂˙Á‡Ì Ò Í˙ÒÌÓÚÓ ‰Ë‡„ÌÓÒÚˈË‡Ì ̇ ÙÛ‰ÓflÌÚÌÓ ÔÓÚ˘‡˘‡ ‚ ÔÓÒΉÌËÚ ‰ÌË ÚÛ·ÂÍÛÎÓÁ‡, ͇ÚÓ ËÌÙÂ͈ËÓÁ̇ Ô˘Ë̇ Á‡ êåã, Ë Í˙ÒÌÓÚÓ ÏÛ Ô‚Âʉ‡Ì ‚ ÓÚ‰ÂÎÂÌË Á‡ ‡ÌËχˆËfl Ë ËÌÚÂÌÁË‚ÌË „ËÊË. ê‡Á‚ËÚËÂÚÓ Ì‡ ÒÂÔÒËÒ, Á‡Òfl„‡ÌÂÚÓ Ì‡ ÒÍÂÎÂÚ̇ڇ ÏÛÒÍÛ·ÚÛ‡ Ë ÔÂËÙÂ̇ ÌÂ‚̇ ÒËÒÚÂχ ÔË êåã Ë Ò‚˙Á‡ÌËÚ éÅç, ÏÂÚ‡·ÓÎËÚÌË Ì‡Û¯ÂÌËfl Ë Ì‡Ô‰‚‡˘‡Ú‡ ÔÓÎËÓ„‡Ì̇ ̉ÓÒÚ‡Ú˙˜ÌÓÒÚ Ò‡ ÍÓÏÔÎÂÍÒÌË Ô˘ËÌË ‚ ڇ̇ÚÓ„ÂÌÂÁ‡Ú‡ ÔË ÚÓÁË Ô‡ˆËÂÌÚ. ëÍÂÎÂÚÌËÚ ÏÛÒÍÛÎË Ò ‚˙ÁÒÚ‡ÌÓ‚flÚ ÏÌÓ„Ó ‰Ó·Â, ÔË ÏËÌËχÎÌË ÓÒÚ‡Ú˙˜ÌË Ì‡Û¯ÂÌËfl, ÒΉ ÛÒÔ¯ÌÓ ÎÂÍÛ‚‡Ì‡ êåã, ͇ÍÚÓ ÚÓ‚‡ ̇·Î˛‰‡‚‡ıÏ ÔË Ì‡¯Ëfl Ô‡ˆËÂÌÚ ‹1. ÖÚÓ Á‡˘Ó ‡‚ÚÓËÚ ӷ˙˘‡Ú ÒÔˆˇÎÌÓ ‚ÌËχÌË ̇ ‡ÌÌÓÚÓ ‡ÁÔÓÁ̇‚‡Ì ̇ ·ÓÎÂÒÚÚ‡ Ë Á‡ÔÓ˜‚‡Ì ̇ ‡„ÂÒË‚ÌÓ Î˜ÂÌËÂ, ͇ÚÓ Ô‰ÔÓÒÚ‡‚͇ Á‡ ̇χÎfl‚‡Ì ̇ ÒÏ˙ÚÌÓÒÚÚ‡ Ë Û‚Â΢‡‚‡Ì ˜ÂÒÚÓÚ‡Ú‡ ̇ ÔÂÊË‚flÂÏÓÒÚ ÔË êåã (4, 8, 9, 12, 16, 21, 27, 29). ãàíÖêÄíìêÄ 1.

2. 3. 4. 5. 6. 7. 8. 9.

à. èÂÚÓ‚, ä. äÓÒÚÓ‚, à. à‚‡ÌÓ‚, à. à‚‡ÌÓ‚‡. éÒÚ‡ ˆˉ˂Ë‡˘‡ ˉËÓÔ‡Ú˘̇ ‡·‰ÓÏËÓÎËÁ‡ Ò ÔËÌÓÒ Ì‡ ‰ËÌ ÒÎÛ˜‡È Ë Ô„Ή ̇ ÎËÚÂ‡ÚÛ‡Ú‡. éÒÌÓ‚ÌË ÔÓ·ÎÂÏË ‚ Ì‚ÓÎÓ„ËflÚ‡. 1994, ÔÓ‰ ê‰. Ö.ÊËÔÂÚÓ‚‡. èÎÓ‚‰Ë‚, èÓÎË„‡ÙËfl ÖÄÑ, 188-190. Berger RP, Wadowksy RM: Rhabdomyolysis associated with infection by Mycoplasma pneumoniae: a case report. Pediatrics 2000 Feb; 105(2): 433 Better OS, Stein JH: Early management of shock and prophylaxis of acute renal failure in traumatic rhabdomyolysis. N Engl J Med 1990 Mar 22; 322(12): 825-9 Burke J, Seda G, Allen D, Knee TS. A case of severe exercise-induced rhabdomyolysis associated with a weight-loss dietary supplement. Mil Med. 2007 Jun; 172 (6):656-8. Chariot P, Ruet E, Authier FJ, et al: Acute rhabdomyolysis in patients infected by human immunodeficiency virus. Neurology 1994 Sep; 44(9): 1692-6. Clarkson PM. Exertional rhabdomyolysis and acute renal failure in marathon runners. Sports Med. 2007; 37(4-5):361-3. Craig S. Rhabdomyolysis. November 30, 2006. Ö-Medicine.com Dayer-Berenson L. Rhabdomyolysis: a comprehensive guide. ANNA J. 1994; 21:15–18. Esnault VL, Nakhla M, Delcroix C, Moutel MG, Couturier O. What is the value of Tc-99m hydroxymethylene diphosphonate scintigraphy for the etio-

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logical diagnosis of mild rhabdomyolysis? Clin Nucl Med. 2007 Jul; 32(7):519-23. 10. Gabow PA, Kaehny WD, Kelleher SP: The spectrum of rhabdomyolysis. Medicine (Baltimore) 1982 May; 61(3): 141-52. 11. Goettsch WG, Heintjes EM, Kastelein JJ, Rabelink TJ, Johansson S, Herings RM. Results from a rosuvastatin historical cohort study in more than 45,000 Dutch statin users, a PHARMO study. Pharmacoepidemiol Drug Saf. 2006 Jul; 15(7):435-43. 12. Haskins N. Rhabdomyolysis and acute renal failure in intensive care. Nurs Crit Care. 1998; 3:283–288. 13. Janssen van Doorn K, Pierard D, Spapen H. Acute renal dysfunction in salmonella gastroenteritis. J Clin Gastroenterol. 2006 Nov-Dec;40(10):9102.Labidi J, Fdhila W, Battikh R, Ellouze S, Ben Abdelhafidh N, Louzir B, M'sadek F, Othmani S. Legionnaire's disease complicated by acute renal failure due to rhabdomyolosis: a case report. Med Mal Infect. 2006 Sep; 36(9):476-8. 14. Karcher C, Dieterich HJ, Schroeder TH Rhabdomyolysis in an obese patient after total knee arthroplasty. Br J Anaesth. 2006 Dec; 97(6):822-4. 15. Lappalainen H, Tiula E, Uotila L: Elimination kinetics of myoglobin and creatine kinase in rhabdomyolysis: implications for follow-up. Crit Care Med 2002 Oct; 30(10): 2212-5. 16. Laura M. Criddle. Rhabdomyolysis. Pathophysiology, Recognition, and Management. Critical Care Nurse Vol 23, No 6, Dec 2003: 14-32. 17. Makaryus JN, Catanzaro JN, Katona KC. Exertional rhabdomyolysis and renal failure in patients with sickle cell trait: is it time to change our approach? Hematology. 2007 Aug; 12(4):349-52. 18. Malinoski DJ, Slater MS, Mullins RJ: Crush injury and rhabdomyolysis. Crit Care Clin 2004 Jan; 20(1): 171-92. 19. Rehman J, Boglia J, Chughtai B, Sukkarieh T, Khan SA, Lewis R, Darras F, Wadhwa NK, Samadi DB, Waltzer WC. High body mass index in muscular patients and flank position are risk factors for rhabdomyolysis: case report after laparoscopic live-donor nephrectomy. J Endourol 2006 Sep;20(9):64650. 20. Rosa NG, Silva G, Teixeira A, Rodrigues F, Araujo JA. Rhabdomyolysis. Acta Med Port. 2005 Jul-Aug;18(4):271-81. 21. Russell TA. Acute renal failure related to rhabdomyolysis: pathophysiology, diagnosis, and collaborative management. Nephrol Nurs J. 2000; 27:567–577. 22. Schmidt GA, Hoehns JD, Purcell JL, Friedman RL, Elhawi Y. Severe rhabdomyolysis and acute renal failure secondary to concomitant use of simvastatin, amiodarone, and atazanavir. J Am Board Fam Med. 2007 JulAug;20(4):411-6 23. Sever SN. Crush syndrune. Rhabdomyolysis. pp. 70-75 Karger 2000 24. Sinert R, Kohl L, Rainone T, Scalea T: Exercise-induced rhabdomyolysis. Ann Emerg Med 1994 Jun; 23(6): 1301-6. 25.Singh U, Scheld WM: Infectious etiologies of rhabdomyolysis: three case reports and review. Clin Infect Dis 1996 Apr; 22(4): 642-9. 26. Tufan A, Dede DS, Cavus S, Altintas ND, Iskit AB, Topeli A. Rhabdomyolysis in a patient treated with colchicine and atorvastatin. Ann Pharmacother. 2006 Jul-Aug; 40(7-8):1466-9 27. Visweswaran P, Guntupalli J: Rhabdomyolysis. Crit Care Clin 1999 Apr; 15(2): 415-28, ix-x. 28. Ward MM: Factors predictive of acute renal failure in rhabdomyolysis. Arch Intern Med 1988 Jul; 148(7): 1553-7. 29. Watanabe N, Inaoka T, Shuke N, Takahashi K, Aburano T, Chisato N, Nochi H, Go K. Acute rhabdomyolysis of the soleus muscle induced by a lightning strike: magnetic resonance and scintigraphic findings. Skeletal Radiol. 2007 Jul; 36(7):671-5.

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl: Ñ- å. äÎËÒÛÒÍË, ‰Ï äÎËÌË͇ ÔÓ Ì‚ÓÎÓ„Ëfl ìåÅÄã “ñ‡ˈ‡ âÓ‡Ì̇” ëÓÙËfl ìÎ. “ÅflÎÓ ÏÓ”‹ 8, 1527 ëÓÙËfl íÂÎ: (02) 9432 –597 \ 160 Ö-mail to mklissurski@yahoo.com


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

‰ÂÍÂÏ‚Ë, 2007

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éË„Ë̇ÎÌË ÒÚ‡ÚËË äêöÇéëçÄÅÑüÇÄçÖ çÄ åéáöóçà íêÄçëèãÄçíÄíà âÓ‰‡Ì è‡ÌÓ‚ äÎËÌË͇ ÔÓ Ì‚ÓıËÛ„Ëfl åÅÄã “ñ‡ˈ‡ âÓ‡Ì̇”, ëÓÙËfl

SUMMARY NEOVASCULARIZATION OF THE BRAIN’S GRAFTS Y. Panov The aim of this study was to investigate vascularization of the solid grafts and cell suspension after intraventricular and intracerebral transplantation. MATERIALS AND METHODS: 106 Wistar rats weighing 200-250g are divided into 7 groups according to localization, volume and embryonic age of the transplant. Fragments and cell suspension of cells from subventricular zone of the brain to be used for transplantation were obtained from 15, 18, 21 day-old embryos. Histological and immunohistochemical examination was carried out 1 to 6 month after transplantation. RESULTS: In brains without necrosis of the transplant we find neovascularization in 40-80% of the cases. In the peripheral portion of the graft, neovascularization is the result of two processes: ingrowths of capillaries from the recipient brain to the graft, and neoformation of capillaries from pre-existing graft’s vessels. We investigated the expression of intercellular adhesion, which is obligatory condition for neovascularization of the intraventricular grafts. CONCLUSION: The growth of new blood vessels contributes to graft survival. Alteration seems to be an important factor in the vascularization of cerebral grafts. Mediated via formation of artificial cavity or transependymal invasion alteration stimulates vascular endothelial growth factor secretion, which plays a crucial role in angiogenesis. KEY WORDS: brain, transplantation, graft.

ÏÂʉÛÍÎÂÚ˙˜Ì‡ ‡‰ıÂÁËfl. á‡Íβ˜ÂÌËÂ: èÓ‡ÒÚ‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ‰ÓÔË̇Òfl Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡. ÄÎÚÂ‡ˆËflÚ‡ ̇ ÏÓÁ˙˜Ì‡Ú‡ Ú˙Í‡Ì Ì‡ ˆËÔËÂÌÚ‡  ‚‡ÊÂÌ Ù‡ÍÚÓ Á‡ ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡ ̇ ‰ÓÌÓ̇ڇ Ú˙͇Ì. è‰ËÁ‚Ë͇̇ ˜ÂÁ ÓÙÓÏflÌ ̇ ‡ÚËÙˈˇÎ̇ ÍÛıË̇ ËÎË ÓÚ Ú‡ÌÒÂÔẨËχÎ̇ڇ ËÌ‚‡ÁËfl, ‡ÎÚÂ‡ˆËflÚ‡ ÒÚËÏÛÎË‡ ÒÂÍˆËflÚ‡ ̇ ẨÓÚÂÎÌË ‚‡ÒÍÛ·ÌË Ù‡ÍÚÓË Ëχ˘Ë ‚‡Ê̇ ÓÎfl ‚ ‡Ì„ËÓ„ÂÌÂÁ‡Ú‡. äãûóéÇà Ñìåà: „·‚ÂÌ ÏÓÁ˙Í, Ú‡ÌÒÔ·ÌÚ‡ˆËfl, Ú‡ÌÒÔ·ÌÚ‡Ú. èÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË Ò ̇ÚÛÔ‡ı‡ ÂÍÒÔÂËÏÂÌÚ‡ÎÌË ‰‡ÌÌË Á‡ ‚˙ÁÏÓÊÌËfl ÔÓÎÓÊËÚÂÎÂÌ ÂÙÂÍÚ ÓÚ Ú‡ÌÒÔ·ÌÚ‡ˆËflÚ‡ ̇ ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ÔË ‡Á΢ÌË Á‡·ÓÎfl‚‡ÌËfl ̇ ÌÂ‚̇ڇ ÒËÒÚÂχ. ÑËÙÂÂ̈ˇˆËflÚ‡ ËÏ ‚ ‡Á΢ÌË ÍÎÂÚ˙˜ÌË ÚËÔÓ‚Â Ë ÚÓÙ˘ÌÓÚÓ ‚˙Á‰ÂÈÒÚ‚Ë ‚˙ıÛ ÏÓÁ˙͇ ̇ ˆËÔËÂÌÚ‡, ‰Ó‚Âʉ‡ ‰Ó ÔÓ-‰Ó·Ó ÙÛÌ͈ËÓ̇ÎÌÓ ‚˙ÁÒÚ‡ÌÓ‚fl‚‡ÌÂ. ÑÓ͇Á‚‡ ÒÂ, ˜Â ‡Ì„ËÓ„ÂÌÂÁ‡Ú‡ ‚ „‡Ì˘̇ڇ ÁÓ̇ ̇ ÎÂÁËflÚ‡ Ë ÂÒÔÂÍÚË‚ÌÓ Ì‡ Ú‡ÌÒÔ·ÌÚ‡ˆËfl, ͇ÍÚÓ Ë ‚ Ò‡ÏËfl Ú‡ÌÒÔ·ÌÚ‡Ú Â ÓÚ ¯‡‚‡˘Ó Á̇˜ÂÌË Á‡ ÔÂÊË‚flÂÏÓÒÚÚ‡ ̇ ÍÎÂÚÍËÚ (12). àÁÛ˜‡‚‡Ì ̇ ÛÒÎÓ‚ËflÚ‡ Á‡ ÔÓÒÚË„‡Ì ̇ ÔÓ- ‰Ó·Ó Í˙‚ÓÒ̇·‰fl‚‡Ì ҇ ‚ ÓÒÌÓ‚‡Ú‡ ̇ ÛÒÔ¯̇ڇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl. ñÖã ч Ò ÛÒÚ‡ÌÓ‚Ë Ì‡Î˘ËÂÚÓ Ì‡ ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌË Ò˙‰Ó‚ ‚ „‡Ì˘̇ڇ ÁÓ̇ ‰ÓÌÓ̇ Ú˙͇̖ ˆËÔËÂÌÚ ÔË ËÌÚ‡‚ÂÌÚËÍÛÎ̇ Ë ËÌÚ‡Ô‡ÂÌıËÏ̇ ÎÓ͇ÎËÁ‡ˆËfl ̇ ÒÓÎˉÌË Ú‡ÌÒÔ·ÌÚ‡ÚË Ë ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl Ë ÛÒÎÓ‚ËflÚ‡ Á‡ ÔÓ‰Ó·‡ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl ̇ Ú‡ÌÒÔ·ÌÚ‡ÚËÚÂ.

êÖáûåÖ ñÖã: ч Ò ËÁÒΉ‚‡ ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡ ̇ ÒÓÎˉÌË Ú‡ÌÒÔ·ÌÚ‡ÚË Ë ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl ÒΉ ËÌÚ‡‚ÂÌÚËÍÛÎ̇ Ë ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl. åÄíÖêàÄã à åÖíéÑ: 106 ÔÎ˙ı‡ Wistar Ò Ú„ÎÓ 200250„ Ò‡ ‡Á‰ÂÎÂÌË ‚ 7 „ÛÔË ÒÔÓ‰ ÎÓ͇ÎËÁ‡ˆËflÚ‡, Ó·Âχ Ë ÂÏ·ËÓ̇Î̇ڇ ‚˙Á‡ÒÚ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡. î‡„ÏÂÌÚË Ë ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl ÓÚ ÍÎÂÚÍË ÓÚ ÒÛ·‚ÂÌÚËÍÛÎ̇ ÁÓ̇ ̇ ÂÏ·ËÓ̇Î̇ ÏÓÁ˙˜Ì‡ Ú˙Í‡Ì Ò‡ ÔÓÎÛ˜ÂÌË ÓÚ ÂÏ·ËÓÌË ÔË Á‡ÔÎÓʉ‡Ì ̇ ÔÎ˙ıÓ‚ÂÚÂ Ò ÓÚ˜Ëڇ̠̇ 15, 18, 21, /Ö15/, /Ö18/, /Ö21/ ‰ÂÌ Ì‡ ·ÂÏÂÌÌÓÒÚÚ‡. ïËÒÚÓÎӄ˘ÌÓ, ıËÒÚÓıËÏ˘ÌÓ ËÁÒΉ‚‡Ì  ËÁ‚˙¯ÂÌÓ 1 ‰Ó 6 ÏÂÒˆ‡ ÒΉ Ú‡ÌÒÔ·ÌÚ‡ˆËflÚ‡. êÖáìãíÄíà: èË ‡Á‚ËÚËÂ Ë ‰ËÙÂÂ̈ˇˆËfl ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡ ‚ ‡Á΢ÌËÚ „ÛÔË Ò ÓÚÍË‚‡Ú ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌË Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ‚ 40 ‰Ó 80% ÓÚ ÒÎÛ˜‡ËÚÂ. Ç ÔÂËÙÂËflÚ‡ ̇ ‚ËÚ‡ÎÌËÚ Ú‡ÌÒÔ·ÌÚ‡ÚË ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡  ÂÁÛÎÚ‡Ú Ì‡ ‰‚‡ ÔÓˆÂÒ‡: ÔÓ‡ÒÚ‚‡Ì ̇ ͇ÔËÎflË ÓÚ ÏÓÁ˙͇ ̇ ˆËÔËÂÌÚ‡ Í˙Ï Ú‡ÌÒÔ·ÌÚ‡Ú‡ Ë ÓÙÓÏflÌ ̇ ͇ÔËÎflË ÓÚ Ò˙˘ÂÒÚ‚Û‚‡˘Ë Ò˙‰Ó‚ ̇ ÔËÒ‡‰˙͇. èË ËÌÚ‡‚ÂÌÚËÍÛÎÌË Ú‡ÌÒÔ·ÌÚ‡ÚË Á‡‰˙ÎÊËÚÂÎÌÓ ÛÒÎÓ‚Ë Á‡ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl  ÛÒÚ‡ÌÓ‚Â̇ڇ

åÖíéÑà ä‡ÚÓ ˆËÔËÂÌÚË Ò‡ ËÁÔÓÎÁ‚‡ÌË 106 ÔÎ˙ı‡ Wistar Ò Ú„ÎÓ 200- 250„. EÏ·ËÓ̇Î̇ ÏÓÁ˙˜Ì‡ Ú˙Í‡Ì Â ÔÓÎÛ˜Â̇ ÓÚ ÂÏ·ËÓÌË ÔË Á‡ÔÎÓʉ‡Ì ̇ ÔÎ˙ıÓ‚Â Ò ÓÚ˜Ëڇ̠̇ 15, 18, 21, /Ö15/, /Ö18/, /Ö21/ ‰ÂÌ Ì‡ ·ÂÏÂÌÌÓÒÚÚ‡ Ò ÚÓ˜ÌÓÒÚ ‰Ó 12 ˜‡Ò‡. àÁ‚˙¯‚‡¯Â Ò ·ԇÓÚÓÏËfl ÔÓ‰ Ó·˘‡ ‡ÌÂÒÚÂÁËfl Ë ÂÏ·ËÓÌËÚ Ò ÓÚ‰ÂÎflı‡ ‚ ÒÚÂËÎÌË ÔÂÚËÚ‡ ‚ ‡ÁÚ‚Ó ̇ ï‡ÚχÌ. èÓ‰ Û‚Â΢ÂÌË ̇ ÓÔÂ‡ˆËÓÌÂÌ ÏËÍÓÒÍÓÔ Ò ÓÙÓÏflı‡ Ù‡„ÏÂÌÚË ÓÚ ÒÛ·‚ÂÌÚËÍÛÎ̇ ÁÓ̇. Ç ˜‡ÒÚ ÓÚ ÂÍÒÔÂËÏÂÌÚËÚÂ, Ù‡„ÏÂÌÚËÚ Ò Ú‡ÌÒÔ·ÌÚË‡ı‡ ‰ËÂÍÚÌÓ, ‡ ‚ ÓÒڇ̇ÎËÚ ÒÎÛ˜‡Ë Ò ËÁ„ÓÚ‚fl¯Â ÍÎÂÚ˙˜ÌÓ ÍÛÎÚË‚Ë‡Ì‡ ÒÛÒÔÂÌÁËfl ÔÓ Òڇ̉‡ÚËÁË‡Ì ÔÓÚÓÍÓÎ: î‡„ÏÂÌÚËÚ Ò ÔÓÒÚ‡‚flÚ ‚ ·‡Î‡ÌÒË‡Ì, ÚÂÏÔÂË‡Ì ÒÓÎÂ‚Ë ‡ÁÚ‚Ó ̇ ï‡ÌÍ. ëΉ ‰‚ÛÍ‡ÚÌÓ ÔÓÏË‚‡Ì ‚ Ò˙˘Ëfl ‡ÁÚ‚Ó ÚÓÍËÌ„‡  ÔÓÒÚ‡‚Â̇ ‚ Ò‰‡ ̇ àÒÍÓ‚ Ò 10% ÙÂÚ‡ÎÂÌ ÚÂίÍË ÒÂÛÏ. ëÛÒÔÂÌÁËflÚ‡  ÍÛÎÚË‚Ë‡Ì‡ ̇ 37 „‡‰ÛÒ‡ ‚ ËÌÍÛ·‡ÚÓ ‚ Ô·ÒÚχÒÓ‚Ë Ï‡Ú‡ˆË. ᇠÚ‡ÌÒÔ·ÌÚ‡ˆËfl  ËÁÔÓÎÁ‚‡Ì‡ ı 5 ÏËÍÓÎËÚ‡ ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl Ò ÍÓ̈ÂÌÚ‡ˆËfl ̇ ÍÎÂÚÍËÚ 1000 000 ̇ ÏËÍÓÎËÚ˙ Ë ‚ËÚ‡ÎÌÓÒÚ- 96%. ÜË‚ÓÚÌËÚ ҇ ‡Á‰ÂÎÂÌË ‚ 7 „ÛÔË.


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

‰ÂÍÂÏ‚Ë, 2007

1û„ÛÔ‡- ËÌÚ‡‚ÂÌÚËÍÛÎ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl- ÔË 13 ÔÎ˙ı‡, Ù‡„ÏÂÌÚË ÓÚ 5ÏÏ3, 13 ÔÎ˙ı‡ - 10ÏÏ3, Ö21; 2 „ÛÔ‡- ‰ËÂÍÚ̇ ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËflÔË 15 ÊË‚ÓÚÌË, Ù‡„ÏÂÌÚË ÓÚ 2ÏÏ3, Ö18; 3 „ÛÔ‡- ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ̇ ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl ÔË 20 ÊË‚ÓÚÌË - 5 ÏËÍÓÎËÚ‡, Ö21 4 „ÛÔ‡- ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ‚ ‡ÚËÙˈˇÎ̇ ÍÛıË̇- 11 ÔÎ˙ı‡- 5 ÏÏ3, Ö21 5 „ÛÔ‡- ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ‚ ‡ÚËÙˈˇÎ̇ ÍÛıË̇- 15 ÔÎ˙ı‡- 2 ÏÏ3, Ö18 6 „ÛÔ‡- ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ‚ ‡ÚËÙˈˇÎ̇ ÍÛıË̇- 10 ÔÎ˙ı‡- 7 ÏÏ3, Ö15 7 „ÛÔ‡- ËÌÚ‡ˆÂ·‡Î̇ ‚ ‡ÚËÙˈˇÎ̇ ÍÛıË̇ – 9 ÔÎ˙ı‡- 7ÏÏ3, Ö 21 íÂıÌË͇ڇ ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ÔÓ‰Ó·ÌÓ Â ÓÔË҇̇ ‚ Ô‰ıÓ‰ÌË ÔÛ·ÎË͇ˆËË (8,9). ïËÒÚÓÎӄ˘ÌÓ, ıËÒÚÓıËÏ˘ÌÓ, ËÁÒΉ‚‡Ì  ËÁ‚˙¯ÂÌÓ 1 ‰Ó 6 ÏÂÒˆ‡ ÒΉ Ú‡ÌÒÔ·ÌÚ‡ˆËflÚ‡. ëÚ‡ÚËÒÚ˘ÂÒ͇ Ó·‡·ÓÚ͇  ̇Ô‡‚Â̇ ÔÓ ÏÂÚÓ‰‡ Á‡ ÓˆÂÌ͇ ̇ ‡ÁÎËÍËÚ ÏÂÊ‰Û ‰‚ ˜ÂÒÚÓÚË Ì‡ ÔÓfl‚‡ ÔË ‡ÎÚÂ̇ÚË‚ÌË Ò˙‚ÓÍÛÔÌÓÒÚË. êÖáìãíÄíà èË ‡Á‚ËÚËÂ Ë ‰ËÙÂÂ̈ˇˆËfl ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡ Ó·˘Ó ‚ 60% ÓÚ ÒÎÛ˜‡ËÚ Ò ÓÚÍË‚‡Ú ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌË Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚Â. èË ËÌÚ‡‚ÂÌÚËÍÛÎÌËÚ Ú‡ÌÒÔ·ÌÚ‡ÚË Ú‡ÍË‚‡ Ò˙‰Ó‚ Ò ÓÚÍË‚‡Ú ‚ ÁÓ̇ڇ ̇ ‡‰ıÂÁËfl Ë ˜‡ÒÚ˘̇ ËÌ‚‡ÁËfl Í˙Ï ÒÚÂ̇ڇ ̇ ‚ÂÌÚËÍÛ·, Í˙‰ÂÚÓ ÎËÔÒ‚‡ ÂÔẨËÏ /ÙË„.1/ ÔË 23 ÊË‚ÓÚÌË ËÎË 80% ÓÚ Ú‡ÌÒÔ·ÌÚË‡ÌËÚÂ. ä˙‚ÓÌÓÒÌË Ò˙‰Ó‚ Ò ÛÒÚ‡ÌÓ‚fl‚‡Ú Ë ‚ ‰˙ηӘË̇ ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡. èË Ú‡ÌÒÔ·ÌÚË‡Ì ̇ χÎÍË Ó·ÂÏË ÒÓÎˉÌË Ú‡ÌÒÔ·ÌÚ‡ÚË Ë ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl – 2 Ë 3 „ÛÔ‡, Ò‡ ÓÚÍË‚‡ÌË Â‰ËÌ˘ÌË Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ‚ 40% ÓÚ ËÁ‚˙¯ÂÌËÚ Ú‡ÌÒÔ·ÌÚ‡ˆËË /ÙË„. 2/. íÓ‚‡ Ò‡ „ÛÔËÚÂ Ò Ì‡È-ÌËÒ˙Í ÔÓˆÂÌÚ Ì‡ ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌË Ò˙‰Ó‚Â, ‚˙ÔÂÍË ˜Â ÚÂıÌ˘ÂÒÍË Ú‡ÌÒÔ·ÌÚ‡ˆËËÚ ҇ ÔÓ-χÎÍÓ Ú‡‚χÚ˘ÌË ‚ Ò‡‚ÌÂÌËÂ Ò ÓÒڇ̇ÎËÚÂ. Ç „ÛÔËÚ ÓÚ ˜ÂÚ‚˙Ú‡ ‰Ó Ò‰χ ‰ÓÌÓ̇ڇ Ú˙Í‡Ì Â Ú‡ÌÒÔ·ÌÚË‡Ì‡ ‚ ‡ÚËÙˈˇÎ̇ ÍÛıË̇. ê‡ÁÎËÍËÚ ҇ ‚ Ó·Âχ Ë ÂÏ·ËÓ̇Î̇ڇ ‚˙Á‡ÒÚ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡ ÒÚ‡ÚËÒÚ˘ÂÒÍË Á̇˜Ëχ ‡ÁÎË͇ ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ó·ÂÏ Ë ÂÏ·ËÓ̇Î̇ ‚˙Á‡ÒÚ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡ Ë ·ÓÈ Ì‡ ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌË Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ̇ ÒÂÁ. ç Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‡ÁÎË͇ Ë ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÔÂÊË‚flÂÏÓÒÚÚ‡ ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡ ‚ ÔÓÒÓ˜ÂÌËÚ „ÛÔË. èË Ú‡ÌÒÔ·ÌÚ‡ˆËËÚ ‚ ‡ÚËÙˈˇÎ̇ ÍÛıË̇ ÂÁÛÎÚ‡ÚËÚ ҇ ÒıÓ‰ÌË- ‚ 60% Ò ÓÚÍË‚‡Ú Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ‚ „‡Ì˘̇ڇ ÁÓ̇ ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡ /ÙË„ 3,4/. éÅëöÜÑÄçÖ Ç ÒÎÛ˜‡ËÚ ̇ ÛÒÔ¯̇ ‚ÂÌÚËÍÛÎ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ÔËÂχÏÂ, ˜Â ÂÔẨËÏ˙Ú Â Ì‡Û¯ÂÌ ‚ÚÓ˘ÌÓ, ‡ Ì ‚ ÂÁÛÎÚ‡Ú Ì‡ Ú‡‚χڇ ÔË ÔÓÌËÍ‚‡ÌÂÚÓ ‚˙‚ ‚ÂÌÚËÍÛ·, Ú˙È Í‡ÚÓ ‡‰ıÂÁËfl ̇·Î˛‰‡‚‡ıÏ ̇ ÏÌÓ„Ó ÔÓ-¯ËÓ͇ ÓÒÌÓ‚‡. àÌÚ‡‚ÂÌÚËÍÛÎÌËÚ Ú‡ÌÒÔ·ÌÚ‡ÚË ‚ ̇¯‡Ú‡ ÒÂËfl Ò‡ ̇È-„ÓÎÂÏËÚ ‚ËÚ‡ÎÌË Ú‡ÌÒÔ·ÌÚ‡ÚË. èËÂχÏÂ, ˜Â ÛÒÎÓ‚ËflÚ‡ Á‡ ÚÓ‚‡ Ò‡ Ò‚Ó·‰ÌÓÚÓ ÔÓÒÚ‡ÌÒÚ‚Ó Á‡ ‡ÒÚÂÊ Ë ‰Ó·‡Ú‡ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl. èË ÎËÔÒ‡ ̇ ‡‰ıÂÁËfl, ÒÓÎˉÌËÚ ËÌÚ‡‚ÂÌÚËÍÛÎÌË Ú‡ÌÒÔ·ÌÚ‡ÚË ÌÂÍÓÚËÁË‡Ú. åÓÊ ‰‡ Ò ‰ÓÔÛÒÌÂ, ˜Â ‰ËÌ˘ÌË ÍÎÂÚÍË ÓÒÚ‡‚‡Ú ‚ËÚ‡ÎÌË ËÁı‡Ì‚‡ÌË ÓÚ ÎËÍ‚Ó‡, ÌÓ fl‚ÌÓ ÚÓ‚‡

262

Ì  ‰ÓÒÚ‡Ú˙˜ÌÓ Á‡ ÔÓ- Ó·ÂÏÌË Ú‡ÌÒÔ·ÌÚ‡ÚË. èÓ ‚Òfl͇ ‚ÂÓflÚÌÓÒÚ Ï‡ÎÍËÚ - ‰Ó 2 ÏÏ3- ‚ËÚ‡ÎÌË Ú‡ÌÒÔ·ÌÚ‡ÚË Ë Â‰ËÌ˘ÌË ‰ÓÌÓÌË ÍÎÂÚÍË Ò ËÁı‡Ì‚‡Ú ÔÓ Ò˙Ò‰ÒÚ‚Ó Ë ·ÂÁ Ó·‡ÁÛ‚‡Ì ̇ ÌÓ‚Ë Ò˙‰Ó‚Â. Ç ÚÂÁË ÒÎÛ˜‡Ë, Ó·‡˜Â, Ì ÏÓÊ ‰‡ Ò ‡Á˜ËÚ‡ ̇ ‚ÂÌÚÛ‡ÎÂÌ ÙÛÌ͈ËÓ̇ÎÂÌ ÂÙÂÍÚ ÔÓ‡‰Ë χÎÍËfl ·ÓÈ ÍÎÂÚÍË ÒÔflÏÓ ÌÂÓ·ıÓ‰ËÏËfl. íÂıÌË͇ڇ ̇ Ú‡ÌÒÔ·ÌÚË‡Ì ‚ ÚÂÁË ÒÎÛ˜‡Ë Ó·ÛÒ·‚fl ÏËÌËχÎ̇ڇ Ú‡‚χ ̇ ÏÓÁ˙͇ ̇ ˆËÔËÂÌÚ‡, ÌÓ ÚÓ‚‡ Ò fl‚fl‚‡ ̉ÓÒÚ‡Ú˙Í ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡. ë˜ËÚ‡ÏÂ, ˜Â ÓÒÌӂ̇ Ô˘Ë̇ Á‡ ÌÂÍÓÁ‡Ú‡ ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡  Ì‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ Ò‚Ó‚ÂÏÂÌ̇ ‚‡ÒÍÛ·ËÁ‡ˆËfl, ÌÓ ‚ ˜‡ÒÚ ÓÚ ÒÎÛ˜‡ËÚ Úfl  ÒΉÒÚ‚Ë ̇ ̉ÓÒÚ‡Ú˙ˆË ‚˙‚ ‚Ò˘ÍË ÂÚ‡ÔË Ì‡ Ú‡ÌÒÔ·ÌÚ‡ˆËflÚ‡ – ÓÚ ÔÓ‰„ÓÚӂ͇ڇ ̇ ‰ÓÌÓ̇ڇ Ú˙͇Ì, ÚÂıÌË͇ ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl Ë ÒΉÓÔÂ‡ÚË‚ÂÌ ÔÂËÓ‰. ÑÓÌÓ̇ Ú˙Í‡Ì Ò Ó·ÂÏ ÓÚ 7ÏÏ3 Ò fl‚fl‚‡ „‡Ì˘ÂÌ ÒÔflÏÓ ËÌÚ‡Í‡ÌˇÎÌËÚ ӷÂÏË Ì‡ ÂÍÒÔÂËÏÂÌÚ‡ÎÌÓÚÓ ÊË‚ÓÚÌÓ. èË ÓÔËÚË Á‡ ËÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ̇ ÔÓ-„ÓÎÂÏË Ó·ÂÏË ÓÚ 7ÏÏ3 ÂÁÛÎÚ‡ÚËÚ ÔÓ„ÂÒË‚ÌÓ Ò ‚ÎÓ¯‡‚‡Ú. éÚ Â‰Ì‡ ÒÚ‡Ì‡ Ú‡‚χڇ  Á̇˜ËÚÂÎ̇, ÓÚ ‰Û„‡ Í˙‚ÓÒ̇·‰fl‚‡ÌÂÚÓ ÓÒÚ‡‚‡ ÌÂÔ˙ÎÌÓˆÂÌÌÓ. ÑÓÒÚ‡Ú˙˜ÂÌ ÎË Â ÔÓÚÂ̈ˇÎ˙Ú Ì‡ Ú‡ÌÒÔ·ÚË‡Ì‡Ú‡ Ú˙Í‡Ì ‰‡ Ó·‡ÁÛ‚‡ Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚Â, ÍÓËÚÓ ‰‡ ̇‚ÎËÁ‡Ú ‚ ÏÓÁ˙˜Ì‡Ú‡ Ú˙Í‡Ì Ì‡ ˆËÔËÂÌÚ‡ Ë ÓÚ ‰Û„‡ ÒÚ‡Ì‡ ̇ ÏÓÁ˙͇ ̇ ˆËÔËÂÌÚ‡, ÍÓÈÚÓ Úfl·‚‡ ‰‡ ‚‡ÒÍÛ·ËÁË‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡? èË Ú‡ÌÒÔ·ÌÚË‡Ì ̇ ÒÛÒÔÂÌÁËfl ÓÚ ÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ‚ χÎ˙Í Ó·ÂÏ Ú‡ÁË ‚˙ÁÏÓÊÌÓÒÚ Â Ï‡Î͇. éÚ Â‰Ì‡ ÒÚ‡Ì‡ Ìflχ ‰ÓÒÚ‡Ú˙˜Ì‡ ÎÂÁËfl ̇ ÏÓÁ˙˜Ì‡ Ú˙Í‡Ì , ÍÓflÚÓ ‰‡ ÔÓ‚ÓÍË‡ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl , ‡ ÓÚ ‰Û„‡ ‚ ҇χڇ ÍÎÂÚ˙˜Ì‡ ÍÛÎÚÛ‡ ÎËÔÒ‚‡Ú ÔÓ„ÂÌËÚÓÌË ẨÓÚÂÎÌË ÍÎÂÚÍË. á‡ÚÓ‚‡ ̇ÔÓÒΉ˙Í Ò Ô‡‚flÚ ÓÔËÚË Á‡ ”Ó·Ó„‡Úfl‚‡Ì” ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡ Ò Ù‡ÍÚÓË ÔÓ‚ÎËfl‚‡˘Ë ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡. èË ÔÓÒÚ‡‚flÌ ̇ 14 ‰Ì‚̇ ÏÂÁÂ̈ÂÙ‡Î̇ ÂÏ·ËÓ̇Î̇ Ú˙Í‡Ì Á‡ 6 ‰ÌË ‚ ‡ÁÚ‚Ó Ò ÂËÚÓÔÓÂÚËÌ Ë GDNF Ë ÔÓÒΉ‚‡˘‡ Ú‡ÌÒÔ·ÌÚ‡ˆËfl Ò ÔÓÎÛ˜‡‚‡ Á̇˜ËÚÂÎÌÓ ÔÓ- ‰Ó·‡ ͇ÔËÎfl̇ ÏÂʇ ‚ Ú‡ÌÒÔ·ÌÚ‡Ú‡ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓ·ڇ (7). èË ‡‚ÚÓÚ‡ÌÒÔ·ÌÚ‡ˆËfl ̇ ‡‰ÂÌÓıËÔÓÙËÁ‡ ̇ 3 ÏÂÒ˜ÌË ÔÎ˙ıÓ‚Â ÔÓ‰ ͇ÔÒÛ· ̇ ·˙·ÂÍ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‡Ì„ËÓ„ÂÌÂÁ‡ ‚ Ú‡ÌÒÔ·ÌÚ‡Ú‡, ͇ÍÚÓ Ë ÌÓ‚Ó‚˙ÁÌËÍ̇ÎË Ò˙‰Ó‚ ÓÚ ·˙·Â͇, ÌÓ Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ë ˆÂÌÚ‡Î̇ ÌÂÍÓÁ‡, „Ó‚Ó¢‡ Á‡ ÌÂÔ˙ÎÌÓˆÂÌÌÓ Í˙‚ÓÒ̇·‰fl‚‡Ì (6). ÖÍÒÔÂËÏÂÌÚ˙Ú Â ÔËÍβ˜ËÎ Á‡ 3 Ò‰ÏËˆË - ̉ÓÒÚ‡Ú˙˜ÌÓ ‚ÂÏ Á‡ ÔˆÂÌ͇ ̇ ‚ËÚ‡ÎÌÓÒÚÚ‡ ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡. çË ҘËÚ‡ÏÂ, ˜Â ÏËÌËχÎÌËflÚ ÒÓÍ Á‡ ÔÓÒΉfl‚‡Ì  ‰ËÌ ÏÂÒˆ. ‚ ÍÓÈÚÓ ËÎË Ì‡ÒÚ˙Ô‚‡ ‰Â„ÂÌÂ‡ˆËfl ̇ Ú‡ÌÒÔ·ÌÚ‡Ú‡ ËÎË ‚‡ÒÍÛ·ËÁË‡ÌÂ Ë ‰ËÙÂÂ̈ˇˆËflÚ‡ ÏÛ. èÓÒÓ˜ÂÌËÚ ÔÓ- „Ó ÂÍÒÔÂËÏÂÌÚË ÔÓ͇Á‚‡Ú ̇΢ËÂÚÓ Ì‡ ÒÓ·ÒÚ‚ÂÌ ÔÓÚÂ̈ˇΠÁ‡ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl ̇ ÒÓÎˉÌËÚ Ú‡ÌÒÔ·ÌÚ‡ÚË, Ë ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ÓÚ ÒÚËÏÛÎË‡˘Ë ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡ Ù‡ÍÚÓË ÔË Ú‡ÌÒÔ·ÌÚ‡ˆËfl ̇ ÒÛÒÔÂÌÁËfl ÓÚ ÍÎÂÚÍË. ë˙„·ÒÌË ÒÏÂ Ò ‡‚ÚÓËÚÂ, ˜Â ıËÔÓÍÒËflÚ‡  ÓÒÌӂ̇ Ô˘Ë̇ Á‡ ÒÂÍˆËflÚ‡ ̇ ‚‡ÒÍÛ·ÂÌ ẨÓÚÂÎÂÌ ‡ÒÚÂÊÂÌ Ù‡ÍÚÓ. Ç Ì‡¯ËÚ ÂÍÒÔÂËÏÂÌÚË ‰Ó·Ë ÂÁÛÎÚ‡ÚË Ò‡ ÔÓÒÚ˄̇ÚË ÔË „ÓÎÂÏË ÒÓÎˉÌË Ú‡ÌÒÔ·ÌÚ‡ÚË ‰ÂÔÓÌË‡ÌË ‚ ÍÛıË̇ – ̇Îˈ ҇ ‰‚‡Ú‡ ÓÒÌÓ‚ÌË Ù‡ÍÚÓ‡ – ͇ÍÚÓ Ì‡Î˘ËÂÚÓ Ì‡ ẨÓÚÂÎÌË ÍÎÂÚÍË ‚ Ú‡ÌÒÔ·ÌÚ‡Ú‡, ÓÚ ÍÓËÚÓ Ò ‡Á‚Ë‚‡Ú Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚Â, ڇ͇ Ë ‰ÓÒÚ‡Ú˙˜Ì‡Ú‡ ÎÂÁËfl ̇ ÏÓÁ˙͇ ̇ ˆËÔËÂÌÚ‡ ‡ÍÚË‚Ë‡˘‡ ÏËÍÓ„ÎËflÚ‡ Ë ‡ÒÚÓˆËÚËÚ Á‡ ÓÒ‚Ó·Óʉ‡‚‡Ì ̇ ẨÓÚÂÎËÌË. Ç ÚÂÁË ÂÍÒÔÂËÏÂÌÚË Ò ‰Ó-


‰ÂÍÂÏ‚Ë, 2007

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

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îË„. 3 àÌÚ‡ˆÂ·‡ÎÂÌ Ú‡ÌÒÔ·ÌÚ‡Ú, Óˆ‚.ïÖ, Û‚. 100. ä˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ÔÓ͇Á‡ÌË Ò˙Ò ÒÚÂÎÍË.

îË„. 1 àÌÚ‡‚ÂÌÚËÍÛÎÂÌ Ú‡ÌÒÔ·ÌÚ‡Ú- ‡‰ıÂÁËfl Ë ˜‡ÒÚ˘̇ ËÌ‚‡ÁËfl Í˙Ï ÒÚÂ̇ڇ ̇ ‚ÂÌÚËÍÛ·, Óˆ‚. ïÖ, Û‚. 40. çÓ‚ÓÓ·‡ÁÛ‚‡Ì Ò˙‰ ÔÓ͇Á‡Ì Ò˙Ò ÒÚÂÎÍË. ͇Á‚‡ Ë ‰Ó·‡ ‚ËÚ‡ÎÌÓÒÚ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡ ·ÂÁ ÌÂÍÓÁ‡ Ë Á‡ 6 ÏÂÒ˜ÂÌ ÔÂËÓ‰ ̇ ̇·Î˛‰ÂÌËÂ. èÓÚË‚ÓÂ˜Ë‚Ë Ò‡ ‰‡ÌÌËÚ ÓÚ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ Í˙‚ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ÔË ÏÓ‰ÂÎË Ì‡ ÏÓÁ˙˜ÌË Á‡·ÓÎfl‚‡ÌËfl Á‡ ÔÓÒÚË„‡Ì ̇ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl. èË Ú‡ÌÒÔ·ÌÚ‡ˆËfl ̇ ÔÂËÙÂÌË Í˙‚ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ‚ ËÒıÂÏ˘ÂÌ ÏÓÁ˙Í Ì‡ ÔÎ˙ıÓ‚Â Ò ÛÒÚ‡ÌÓ‚fl‚fl ÔÓ- ‰Ó·‡ ‡Ì„ËÓ„ÂÌÂÁ‡ Ë Ì‚ÓÎӄ˘ÌÓ ÔÓ‰Ó·ÂÌË ĂÚÓËÚ ӷflÒÌfl‚‡Ú ÚÓ‚‡ Ò ÂÍÒÔÂÒËflÚ‡ ̇ ÏËÍÓ„ÎËfl Ë ·ÂÚ‡ 1 ËÌÚ„ËÌË ÓÚ ÒÚ‚ÓÎÓ‚ËÚ ÍÎÂÚÍË, ÌÓ ÌÂ Ë Ì‡ Ì‚Ó̇Î̇ڇ ËÏ Ú‡ÌÒÙÓχˆËfl (10). ë˙˘ÓÚÓ Â ÒÚ‡ÌӂˢÂÚÓ Ë Ì‡ (5) - ÍÓÒÚÌÓÏÓÁ˙˜ÌËÚ ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË Ò ‰ËÙÂÂ̈Ë‡Ú Ò‡ÏÓ ‰Ó ÏËÍÓ„ÎËfl Ë ÔÂË‚‡ÒÍÛ·ÌË ÍÎÂÚÍË. ÑÛ„ ÍÓÎÂÍÚË‚ (4) ËÁÒΉ‚‡Ú Ôӂ‰ÂÌËÂÚÓ Ì‡ ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÔÓ„ÂÌËÚÓÌË ẨÓÚÂΡÎÌË ÍÎÂÚÍË ‚ ñçë- „·‚ÂÌ, „˙·Ì‡˜ÂÌ ÏÓÁ˙Í Ë ÂÚË̇. ç ÛÒÚ‡ÌÓ‚fl‚‡Ú Û˜‡ÒÚËÂÚÓ ËÏ ‚ ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌÂÚÓ Ì‡ Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚Â. Ö̉ÓÚÂΡÎÌË ÔÓ„ÂÌËÚÓÌË ÍÎÂÚÍË Ò‡ ËÁÓÎË‡ÌË ÓÚ ÔÂËÙÂ̇ Í˙‚ - CD34, Flk-1, ËÎË AC133 ‡ÌÚË„ÂÌ- ÔÓÁËÚË‚ÌË ÍÎÂÚÍË, ͇ÚÓ Ò ‰Ó͇Á‚‡ Úflı̇ڇ ÍÓ̈ÂÌÚ‡ˆËfl

îË„. 2 àÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl ̇ ÍÎÂÚ˙˜Ì‡ ÒÛÒÔÂÌÁËfl- 5 ÏËÍÓÎËÚ‡, Ö21 Óˆ‚. ïÖ, Û‚. 100. ä˙‚ÓÌÓÒÂÌ Ò˙‰ ÔÓ͇Á‡Ì Ò˙Ò ÒÚÂÎ͇.

‚ ÏflÒÚÓÚÓ Ì‡ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËfl Á‡ ‡Á΢ÌË Ó„‡ÌË (1). íÓ‚‡ Ì  ‰ÓÒÚ‡Ú˙˜ÌÓ ‰‡ Ò ÔËÂÏÂ, ˜Â Û˜‡ÒÚ‚‡Ú ‚ ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡. íÂÁË ËÁÒΉ‚‡ÌËfl ÔÓ-ÒÍÓÓ Óı·ʉ‡Ú ÂÌÚÛÒˇÁχ Á‡ χÒÓ‚Ó ÔËÎÓÊÂÌË ̇ ÍÓÒÚÌÓÏÓÁ˙˜ÌËÚ ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ‚ ΘÂÌËÂÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌËfl ̇ ñçë. Ä‚ÚÓÒÍË ÍÓÎÂÍÚË‚Ë ÓÚÌÓ‚Ó Ò ӷ˙˘‡Ú, ‚˙ÔÂÍË ÚÛ‰ÌÓÒÚËÚÂ, Í˙Ï ÂÍÒÔÂËÏÂÌÚË Ë Ì‡˜‡ÎÌË ÍÎËÌ˘ÌË ÔËÎÓÊÂÌËfl ̇ ÂÏ·ËÓ̇Î̇ ÌÂ‚̇ Ú˙͇Ì. MÌÓ„Ó ‰Ó·Ó ‡Á‚ËÚË (3) ̇ ÂÏ·ËÓ̇ÎÌË ÌÂ‚ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË Ò ‰Ó-

îË„. 4 àÌÚ‡ˆÂ·‡Î̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl. É‡Ì˘̇ ÁÓ̇ ‰ÓÌÓˆËÔËÂÌÚ Ò ÌÓ‚ÓÓ·‡ÁÛ‚‡ÌË Ò˙‰Ó‚Â, Óˆ‚. ïÖ, Û‚. 100. ä˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ÔÓ͇Á‡ÌË Ò˙Ò ÒÚÂÎÍË. ͇Á‚‡, ͇ÍÚÓ ËÌ ‚ËÚÓ, ڇ͇ Ë ËÌ ‚Ë‚Ó. ìÒÚ‡ÌÓ‚ÂÌÓ Â ‡Á‚ËÚË ̇ Ú‡ÌÒÔ·ÌÚË‡ÌË Ì‚ÓÌË ‚ ÎÂÁËfl ̇ ÏÓÚÓ̇ ÍÓ‡, ͇ÍÚÓ Ë ÔÓ-‰Ó·Ó ‚˙ÁÒÚ‡ÌÓ‚fl‚‡Ì ̇ ÏÓÚÓÂÌ ‰ÂÙˈËÚ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎ̇ „ÛÔ‡. éÚÌÓ‚Ó Ò ÔÓÚ‚˙ʉ‡‚‡Ú ‰Ó͇Á‡ÌËÚ ÓÚ 20 „Ó‰ËÌË Ô‰ËÏÒÚ‚‡ ̇ ÂÏ·ËÓ̇Î̇ڇ ÌÂ‚̇ Ú˙Í‡Ì Á‡ ÂÍÓÌÒÚÛ͈Ëfl ̇ ÏÓÁ˙͇. Ç ÔˆËÁÂÌ ÂÍÒÔÂËÏÂÌÚ (11) e ‰ÂÏÓÌÒÚË‡Ì‡ ‰Ó·‡ ‚‡ÒÍÛ·ËÁ‡ˆËfl ̇ ÔË̇Î̇ ÊÎÂÁ‡ ÒΉ ‡‚ÚÓÚ‡ÌÒÔ·ÌÚ‡ˆËfl ‚ ÏÓÁ˙˜ÂÌ Ô‡ÂÌıËÏ. Ä‚ÚÓËÚ Á‡Íβ˜‚‡Ú, ˜Â ÒÓÎˉÌËflÚ Ú‡ÌÒÔ·ÌÚ‡Ú Ò˙Ò Ò‚ÓËÚ ÌÂ̇Û¯ÂÌË ÏÂʉÛÍÎÂÚ˙˜ÌË ‚˙ÁÍË Ë Ì‡Î˘ÌË Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ӷÎÂ͘‡‚‡Ú ÚÓÁË ÔÓˆÂÒ. ÑÛ„ ÍÓÎÂÍÚË‚ (2) ÒΉ ÒÂËfl ÂÍÒÔÂËÏÂÌÚË ËÁÔÓÎÁ‚‡ÈÍË Ì‚Ó̇Î̇ ÍÎÂÚ˙˜Ì‡ ÍÛÎÚÛ‡ ÓÚ 15 ‰Ì‚ÂÌ ÔÎ˙¯Ë ÏÂÁÂ̈ÂÙ‡ÎÓÌ Ë ˜Ó‚¯ÍË ẨÓÚÂΡÎÂÌ ‡ÒÚÂÊÂÌ Ù‡ÍÚÓ ‰ÓÍ·‰‚‡, ˜Â ÌÂÓ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡ ÓÔ‰ÂÎfl ÒÚÂÔÂÌÚ‡ ̇ ÔÂÊË‚flÂÏÓÒÚ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡. íÂÁË ‚˙Á„ÎÂ‰Ë ÒÔÓ‰ÂÎflÏÂ Ë ÌË ÒΉ ‡Ì‡ÎËÁ‡ ̇ ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚ ÔÓÒÚ‡ÌÓ‚ÍË. è‰ÒÚ‡‚flÌÂÚÓ Ì‡ Í˙‚ÓÒ̇·‰ÂÌË ‚ËÚ‡ÎÌË Ú‡ÌÒÔ·ÌÚ‡ÚË, ÒÔÓ‰ Ì‡Ò Â ‚‡ÊÌÓ


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

‰ÂÍÂÏ‚Ë, 2007

‰Ó͇Á‡ÚÂÎÒÚ‚Ó Á‡ ÛÒÔÂı‡ ̇ ‰̇ ËÌÚ‡Í‡ÌˇÎ̇ Ú‡ÌÒÔ·ÌÚ‡ˆËfl. áÄäãûóÖçàÖ èÓ‡ÒÚ‚‡ÌÂÚÓ Ì‡ ÌÓ‚Ë Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚ ‰ÓÔË̇Òfl Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ Ú‡ÌÒÔ·ÌÚ‡Ú‡. ÄÎÚÂ‡ˆËflÚ‡ ̇ ÏÓÁ˙˜Ì‡Ú‡ Ú˙Í‡Ì Ì‡ ˆËÔËÂÌÚ‡  ‚‡ÊÂÌ Ù‡ÍÚÓ Á‡ ‚‡ÒÍÛ·ËÁ‡ˆËflÚ‡ ̇ ‰ÓÌÓ̇ڇ Ú˙͇Ì. è‰ËÁ‚Ë͇̇ ˜ÂÁ ÓÙÓÏflÌ ̇ ‡ÚeÙˈˇÎ̇ ÍÛıË̇ ËÎË ÓÚ Ú‡ÌÒÂÔẨËχÎ̇ڇ ËÌ‚‡ÁËfl, ‡ÎÚÂ‡ˆËflÚ‡ ÒÚËÏÛÎË‡ ÒÂÍˆËflÚ‡ ̇ ẨÓÚÂÎÌË ‚‡ÒÍÛ·ÌË Ù‡ÍÚÓË Ëχ˘Ë ‚‡Ê̇ ÓÎfl ‚ ‡Ì„ËÓ„ÂÌÂÁ‡Ú‡. ãàíÖêÄíìêÄ 1. 2.

3.

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Asahara T, Kawamoto A. Endothelial progenitor cells for postnatal vasculogenesis. Am J Physiol Cell Physiol. 2004 Sep;287(3):C572-9. Casper D, Engstrom SJ, Mirchandani GR, Pidel A, Palencia D, Cho PH, Brownlee M, Edelstein D, Federoff HJ, Sonstein WJ. Enhanced vascularization and survival of neural transplants with ex vivo angiogenic gene transfer. Cell Transplant. 2002;11(4):331-49 Chiba S, Ikeda R, Kurokawa MS, Yoshikawa H, Takeno M, Nagafuchi H, Tadokoro M, Sekino H, Hashimoto T, Suzuki N. Anatomical and functional recovery by embryonic stem cell-derived neural tissue of a mouse model of brain damage. J Neurol Sci. 2004 Apr 15;219(1-2):107-17 Galimi F, Summers RG, van Praag H, Verma IM, Gage FH. A role for bone marrow-derived cells in the vasculature of noninjured CNS. Blood. 2005 Mar 15;105(6):2400-2. Epub 2004 Nov 30.

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Hess DC, Abe T, Hill WD, Studdard AM, Carothers J, Masuya M, Fleming PA, Drake CJ, Ogawa M. Hematopoietic origin of microglial and perivascular cells in brain. Exp Neurol. 2004 Apr;186(2):134-44. 6. Lombardero M, Quintanar-Stephano A, Vidal S, Horvath E, Kovacs K, Lloyd RV, Scheithauer BW.Vascularization of rat pituitary autografts. J Anat. 2006 May;208(5):587-93 7. McLeod M, Hong M, Mukhida K, Sadi D, Ulalia R, Mendez I. Erythropoietin and GDNF enhance ventral mesencephalic fiber outgrowth and capillary proliferation following neural transplantation in a rodent model of Parkinson's disease. Eur J Neurosci. 2006 Jul;24(2):361-70. 8. Panov Y , Thencheva Z., A modified approach to transplantation in the lateral ventricle of rats. Comptes rendus de L’Academie bulgare des Sciences 2005,(58) ‹ 7 , 855- 860 9. Panov Y. Physiological limit of intracerebral transplantation in rats. Comptes rendus de L’Academie bulgare des Sciences 2006,(59) ‹ 10 1083-1088 10. Shyu WC, Lin SZ, Chiang MF, Su CY, Li H. Intracerebral peripheral blood stem cell (CD34+) implantation induces neuroplasticity by enhancing beta1 integrin-mediated angiogenesis in chronic stroke rats. J Neurosci. 2006 Mar 29;26(13):3444-53. 11. Villegas J. Time course of angiogenesis in solid pineal autografts. Cell Tissue Res. 2004 Mar;315(3):349-59. Epub 2004 Jan 15 12. Wei L, Keogh CL, Whitaker VR, Theus MH, Yu SP. Angiogenesis and stem cell transplantation as potential treatments of cerebral ischemic stroke. Pathophysiology. 2005 Jul;12(1):47-62

ĉÂÒ Á‡ ÍÓÂÒÔÓ̉Â̈Ëfl Ñ- âÓ‰‡Ì è‡ÌÓ‚ Ñå äÎËÌË͇ ÔÓ Ì‚ÓıËÛ„Ëfl åÅÄã ◊ñ‡ˈ‡ âÓ‡Ì̇” ìÎ. ÅflÎÓ ÏÓ ‹ 8 1504 ëÓÙËfl


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

‰ÂÍÂÏ‚Ë, 2007

265

éÔËÒ‡ÌË ̇ ÍÎËÌ˘ÂÌ ÒÎÛ˜‡È çÖÇêéÑÖÉÖçÖêÄñàü ë éíãÄÉÄçÖ çÄ ÜÖãüáé Ç åéáöäÄ (ëàçÑêéå çÄ HALLERVORDEN-SPATZ) ã. í‡ÈÍÓ‚1, ò. åÂı‡·Ë‡Ì1, å. ê‡È˜Â‚‡1, å. èÂÚÓ‚‡1, ü. ÜÂ΂1, é„ÌflÌ ä˙΂2 äÎËÌË͇ ÔÓ ç‚ÓÎÓ„Ëfl, åÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇”, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËfl 2 ㇷÓ‡ÚÓËfl ÔÓ ç‚ÓÔ‡ÚÓÎÓ„Ëfl, å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËfl

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SUMMARY NEURODEGENERATION WITH BRAIN IRON A CCUMULATION (HALLERVORDEN-SPATZ) L. Traykov, S. Mehrabian, M. Raycheva, M. Petrova, Y. Zhelev, O. Kalev Department of Neurology, UH “Alexandrovska”, Medical University, Sofia, Bulgaria Laboratory of Pathology, Medical University, Sofia, Bulgaria Neurodegeneration with brain iron accumulation, also known as a Hallervorden-Spatz syndrome, is a rare neurodegenerative autosomal recessive disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. Many patients with this disease have mutations in the gene encoding pantothenate kinase 2 (PANK2); these patients are said to have pantothenate kinase-associated neurodegeneration. In this study we present the clinical and radiographic features of a Bulgarian patient with Hallervorden-Spatz syndrome. The patient is a 43 years old man. The first symptoms of mood disturbances, disorientation, social withdrawal and loss of motivation were noticed at the age of 28. About 6 years after the disease onset, gait troubles occurred accompanied with slowed movements. He had reduced spontaneous speech with changes in voice and speech. During the last years the complaints were with more rapid progression and the memory and orientation disturbances worsened, as well as the impossibility of managing with daily activities. A pigmentary degeneration of the retina was diagnosed. The neurological exam reveals an apathetic and bradykinetic man with severely impaired cognitive functions. He has dysphonia, dysarthria, dystonia with rigidly increased muscle tone in the right upper limb and pathologic reflex of Babinski in the right. The neuropsychological assessment shows severe dementia (MMSE =7). MRI of the brain shows a specific pattern of hyperintensity within the hypointense medial globus pallidus. A skin biopsy is performed aiming definitive neuropathological diagnosis based on electron microscopy findings. He has no familial history for similar disease, but positive familial history for pigmentary degeneration of the retina. In conclusion, the diagnosis of neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome) is reported on the basis of clinical and neuroimaging assessment as having atypical disease (later onset with slow progression). Although we are waiting for genetic results, the literature data show that a specific MRI pattern distinguishes patients with PANK2 mutations. This patient is the first case in Bulgaria of Hallervorden-Spatz syndrome. KEY WORDS: Hallervorden-Spatz syndrome, clinical assessment, MRI and pathlogy findings

êÖáûåÖ ç‚Ӊ„ÂÌÂ‡ˆËfl Ò ÓÚ·„‡Ì‡ ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇, ËÁ‚ÂÒÚÂÌ Í‡ÚÓ ÒË̉ÓÏ Ì‡ Hallervorden-Spatz  fl‰ÍÓ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ Ò ‡‚ÚÓÁÓÏÌÓ-ÂÒˆÂÒË‚ÌÓ Û̇ÒΉfl‚‡ÌÂ, ı‡‡ÍÚÂËÁË‡˘Ó Ò ‰ËÒÚÓÌËfl, Ô‡ÍËÌÒÓÌËÁ˙Ï Ë ‡ÍÛÏÛ·ˆËfl ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇. èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË ËÏ‡Ú ÏÛÚ‡ˆËË ‚ „ÂÌ, ÍÓ‰Ë‡˘ Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡ 2 (PANK 2), ÍÓËÚÓ Ò ‰Ë‡„ÌÓÒÚˈË‡Ú ͇ÚÓ Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡-Ò‚˙Á‡Ì‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl. è‰ÒÚ‡‚flÏ ÍÎËÌ˘̇ Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘‡ ͇ÚË̇ ̇ ·˙΄‡ÒÍË Ô‡ˆËÂÌÚ Ò Hallervorden-Spatz ÒË̉ÓÏ. 臈ËÂÌÚ˙Ú Â 43 „Ӊ˯ÂÌ Ï˙Ê Ò ÔÓfl‚Ë Ì‡ ‰ÂÁÓËÂÌÚ‡ˆËfl, ÔÓÏÂÌË ‚ ̇ÒÚÓÂÌËÂÚÓ, ÒӈˇÎÌÓ ËÁÓÎË‡ÌÂ Ë ÎËÔÒ‡ ̇ ÏÓÚË‚‡ˆËfl, ‰Â·˛ÚË‡˘Ë ̇ 28 „Ӊ˯̇ ‚˙Á‡ÒÚ. òÂÒÚ „Ó‰ËÌË ÒΉ ̇˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Ò ÔÓfl‚fl‚‡Ú ÔÓÏÂÌË ‚ ÔÓıӉ͇ڇ Ë ÔÓÁ‡Ú‡ Ò˙Ò Á‡·‡‚flÌ ̇ ‰‚ËÊÂÌËflÚ‡, ÒËÎÌÓ Ó·Â‰Ìfl‚‡Ì ̇ ˜ÌË͇ Ë ÔÓÏÂÌË ‚ „·҇ Ë „Ó‚Ó‡. èÂÁ ÔÓÒΉÌËÚ „Ó‰ËÌË ·˙ÁÓ ÔÓ„ÂÒË‡Ú ̇Û¯ÂÌËflÚ‡ ‚ Ô‡ÏÂÚÚ‡ Ë ÓËÂÌÚ‡ˆËflÚ‡ Ò˙Ò Á‡ÚÛ‰ÌÂÌËfl ‚ Ò‡ÏÓÒÚÓflÚÂÎÌÓÚÓ ÒÔ‡‚flÌÂ Ò Âʉ̂ÌËÚ ‰ÂÈÌÓÒÚË. ç‚ÓÎӄ˘ÌËflÚ Ô„Ή ÔÓ͇Á‚‡ ‡Ô‡Ú˘ÂÌ Ë ·‡‰ËÍËÌÂÚ˘ÂÌ Ï˙Ê Ò ÚÂÊÍÓ Ì‡Û¯ÂÌË ‚ ÍÓ„ÌËÚË‚ÌËÚ ÙÛÌ͈ËË. ç‡Îˈ  ‰ËÒÙÓÌËfl, ‰ËÁ‡ÚËfl, ‰ËÒÚÓÌËfl Ò ˄ˉÌÓ Ôӂ˯ÂÌ ÏÛÒÍÛÎÂÌ ÚÓÌÛÒ ‚ ‰flÒ̇ڇ ˙͇ Ë Ô‡ÚÓÎӄ˘ÂÌ ÂÙÎÂÍÒ Ì‡ Ň·ËÌÒÍË ‚ ‰flÒÌÓ. ç‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‚‡Ì ÓÚÍË‚‡ ÚÂÊ˙Í ‰ÂÏÂÌÚÂÌ ÒË̉ÓÏ (MMSE =7). åêí ̇ „·‚ÂÌ ÏÓÁ˙Í ÔÓ͇Á‚‡ ÒÔˆËÙ˘̇ ͇ÚË̇ Ò ıËÔÂËÌÚÂÌÁ̇ ÁÓ̇ Á‡Ó·ËÍÓÎÂÌ ÓÚ ıËÔÓËÌÚÂÌÁÂÌ „ËÓÌ ‚ ωˇÎÂÌ „ÎÓ·ÛÒ Ô‡ÎˉÛÒ. è‰ÔËÂÚ‡  ÍÓÊ̇ ·ËÓÔÒËfl Ò ˆÂÎ ‰ÂÙËÌËÚ˂̇ Ì‚ÓÔ‡ÚÓÎӄ˘̇ ‰Ë‡„ÌÓÁ‡ ̇ ·‡Á‡Ú‡ ̇ ÂÎÂÍÚÓÌÌÓÏËÍÓÒÍÓÔÒ͇ڇ ̇ıӉ͇. 臈ËÂÌÚ˙Ú Ìflχ Ù‡ÏËÎ̇ Ó·ÂÏÂÌÂÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ ÔÓ‰Ó·ÌÓ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÌÓ Ëχ Ù‡ÏËÎÌË ‰‡ÌÌË Á‡ ÔË„ÏÂÌÚÓÁÂÌ ÂÚËÌËÚ. Ç Á‡Íβ˜ÂÌËÂ, ‰Ë‡„ÌÓÁ‡Ú‡ ◊‡ÚËÔ˘̇ ÙÓχ” ̇ ç‚Ӊ„ÂÌÂ‡ˆËfl Ò ÓÚ·„‡Ì‡ ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇, ËÁ‚ÂÒÚÂÌ Í‡ÚÓ ÒË̉ÓÏ Ì‡ Hallervorden-Spatz Ò ÔÓÒÚ‡‚fl ̇ ·‡Á‡Ú‡ ̇ ÍÎËÌ˘ÌËÚ ‰‡ÌÌË (Ò ÔÓ-Í˙ÒÌÓ Ì‡˜‡ÎÓ Ë ÔÓ-·‡‚̇ ÔÓ„ÂÒËfl). Ç˙ÔÂÍË ˜Â Ó˜‡Í‚‡Ï „ÂÌÂÚ˘ÌËÚ ÂÁÛÎÚ‡ÚË, ÒÔˆËÙ˘̇ڇ åêí ͇ÚË̇ Ò ̇·Î˛‰‡‚‡ Ò‡ÏÓ ÔË Ô‡ˆËÂÌÚË Ò ÏÛÚ‡ˆËË ‚ PANK2. íÓÁË Ô‡ˆËÂÌÚ Â Ô˙‚ËflÚ ÒÎÛ˜‡È ̇ Hallervorden-Spatz ÒË̉ÓÏ ‚ Å˙΄‡Ëfl äãûóéÇà Ñìåà: ëË̉ÓÏ Ì‡ Hallervorden-Spatz, ÍÎËÌ˘̇ ͇ÚË̇, åêí Ë Ì‚ÓÔ‡ÚÓÎӄ˘ÌË ‰‡ÌÌË. ÇöÇÖÑÖçàÖ ç‚Ӊ„ÂÌÂ‡ˆËflÚ‡ Ò ÓÚ·„���Ì ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇, ËÁ‚ÂÒÚ̇ Ӣ ͇ÚÓ ÒË̉ÓÏ Ì‡ Hallervorden-Spatz,  fl‰ÍÓ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ Ò ÔÓ„ÂÒË‚ÂÌ ıÓ‰ ̇


‰ÂÍÂÏ‚Ë, 2007

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

ÔÓÚ˘‡ÌÂ, ı‡‡ÍÚÂËÁË‡˘Ó ÒÂ Ò ‰ËÒÚÓÌËfl, Ô‡ÍËÌÒÓÌËÁ˙Ï Ë Ì‡ÚÛÔ‚‡Ì ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇. éÔËÒ‡ÌÓ Â ÓÚ Hallervorden Ë Spatz ÔÂÁ 1922„. (4). èÓ‡‰Ë ÌÂÂÚ˘ÌË ÔÓÒÚ˙ÔÍË ÓÚ ÒÚ‡Ì‡ ̇ ‡‚ÚÓËÚ ÔÂÁ ÇÚÓ‡Ú‡ Ò‚ÂÚӂ̇ ‚ÓÈ̇  Ô‰ÎÓÊÂÌÓ Ì‡ËÏÂÌÓ‚‡ÌËÂÚÓ “Ì‚Ӊ„ÂÌÂ‡ˆËfl Ò ÓÚ·„‡Ì ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇” (7). èӂ˜ÂÚÓ Ô‡ˆËÂÌÚË ËÏ‡Ú ÏÛÚ‡ˆËË ‚ „ÂÌ, ÍÓ‰Ë‡˘ Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡ 2 (PANK2) Ò ‡‚ÚÓÁÓÏÌÓ-ÂÒˆÂÒË‚ÌÓ Û̇ÒΉfl‚‡ÌÂ. íÓÁË „ÂÌ Â ÎÓ͇ÎËÁË‡Ì ‚˙ıÛ 2013 ıÓÏÓÁÓχ Ë Â ÓÚÍËÚ ÔÂÁ 2001 „. (11,15). íÂÁË ÒÎÛ˜‡Ë Ò ‰Ë‡„ÌÓÒÚˈË‡Ú ͇ÚÓ ◊Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡-Ò‚˙Á‡Ì‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl”. è‰ÔÓ·„‡ ÒÂ, ˜Â PANK2 ÏÛÚ‡ˆËËÚ ‚Ó‰flÚ ‰Ó ËÁ˜ÂÔ‚‡Ì ̇ ÍÓÂÌÁËÏ Ä, ‡ÒÓˆËË‡ÌÓ Ò ‰ÂÙÂÍÚ̇ ·ËÓÒËÌÚÂÁ‡ ̇ ÏÂÏ·‡Ì‡Ú‡. ÄÍÛÏÛÎË‡ÌËflÚ ˆËÒÚÂËÌ Ó·‡ÁÛ‚‡ ÍÓÏÔÎÂÍÒË Ò ÊÂÎflÁÓÚÓ, ÍÓÂÚÓ Ô‰ËÁ‚ËÍ‚‡ ÓÍÒˉ‡ÚË‚ÌÓ Û‚Âʉ‡Ì ̇ ÏÓÁ˙͇ (7). Ñˇ„ÌÓÁ‡Ú‡ ◊ëË̉ÓÏ Ì‡ Hallervorden-Spatz” ‚Íβ˜‚‡ ‡ÁÌÓÓ·‡ÁÂÌ ÒÔÂÍÚ˙ ÓÚ ÍÎËÌ˘ÌË Ô‰ÒÚ‡‚flÌËfl, ÍÓËÚÓ ËÏ‡Ú Ó·˘‡ Ì‚Ӊ„ÂÌÂ‡Ú˂̇ ͇ÚË̇ Ë ÓÚ·„‡Ì ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇. ê‡Á΢‡‚‡Ú Ò ÚËÔ˘ÌË Ë ‡ÚËÔ˘ÌË ÙÓÏË Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ. éË„Ë̇Î̇ڇ Í·Ò˘ÂÒ͇ ÙÓχ Ò Ô‰ÒÚ‡‚fl Ò ‡ÌÌÓ Ì‡˜‡ÎÓ Ë ·˙Á‡ ÔÓ„ÂÒËfl. Ñ·˛Ú˙Ú È Â ‚ ‰ÂÚÒ͇ ‚˙Á‡ÒÚ (Ò‰ÌÓ 3.4±3„. ‚ ‰Ë‡Ô‡ÁÓÌ 0.5-12) Ò ÔÓ„ÂÒË‚ÂÌ ıÓ‰ Ë ÔÓfl‚Ë Ì‡ ‰ËÒÚÓÌËfl, ‰ËÁ‡ÚËfl Ë ˄ˉÌÓÒÚ, Á‡‚˙¯‚‡ÈÍË Ò ·˙Á Ù‡Ú‡ÎÂÌ ËÁıÓ‰. ë˙Ô˙ÚÒ‚‡˘ ÒËÏÔÚÓÏ ÏÓÊ ‰‡ ·˙‰Â ÔË„ÏÂÚÓÁ̇ ‰Â„ÂÌÂ‡ˆËfl ̇ ÂÚË̇ڇ. éÚ·„‡ÌÂÚÓ Ì‡ ÊÂÎflÁÓ ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎË Â flÒÌÓ ËÁ‡ÁÂÌÓ Ì‡ åêí ËÁÒΉ‚‡ÌÂ Ë ÔÓÒÚÏÓÚÂÏ. èË ◊‡ÚËÔ˘ÌËÚ” ÙÓÏË Ì‡ ëË̉ÓÏ˙Ú Ì‡ Hallervorden-Spatz, ‰Â·˛Ú˙Ú Ì‡ Á‡·ÓÎfl‚‡ÌÂÚÓ Â ÔÓ-Í˙ÒÌÓ (13.7±5.9„. Ò ‰Ë‡Ô‡ÁÓÌ 1-28) Ë ÔÓ„ÂÒËflÚ‡  ÔÓ-·‡‚̇ Ë ÔÓ-‚‡ˇ·ËÎ̇, Ò˙ÔÓ‚Ó‰Â̇ Ò ÓÚ·„‡Ì ̇ ÊÂÎflÁÓ ‚ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË. éÚÍË‚‡Ú Ò ÏÛÚ‡ˆËË Ì‡ PANK 2 „Â̇ ÔË ‚Ò˘ÍË Ô‡ˆËÂÌÚË Ò Í·Ò˘ÂÒ͇ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ Ë ÔË Â‰Ì‡ ÚÂÚ‡ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ‡ÚËÔ˘̇ ÙÓχ (2,6,8,9). äËÚÂËËÚ Á‡ ÔÓÒÚ‡‚flÌ ̇ ‰Ë‡„ÌÓÁ‡Ú‡ ◊ëË̉ÓÏ Ì‡ Hallervorden-Spatz”Ò‡ ÒΉÌËÚ (12): Á‡‰˙ÎÊËÚÂÎÌËÚ ÒËÏÔÚÓÏË Ò‡ ̇˜‡ÎÓ ÔÂÁ Ô˙‚ËÚ ‰‚ ‰Â͇‰Ë, ÔÓ„ÂÒË‚ÂÌ ıÓ‰ Ë ÂÍÒÚ‡ÔË‡ÏˉÌË ·ÂÎÂÁË. èÓ‰ÍÂÔfl˘ËÚ ÒËÏÔÚÓÏË Ò‡ ÔË‡ÏˉÌË ·ÂÎÂÁË, ÔÓ„ÂÒË‚ÌË ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËfl, åêí ‰‡ÌÌË Á‡ ◊Ó˜Ë Ì‡ ÚË„˙”, „˙˜Ó‚Â, ÔË„ÏÂÌÚÓÁÂÌ ÂÚËÌËÚ Ë ÓÔÚ˘̇ ‡ÚÓÙËfl, Ù‡ÏËÎ̇ Ó·ÂÏÂÌÂÌÓÒÚ, ÓÚÍË‚‡Ì ̇ ‡·ÌÓÏÌË ˆËÚÓÁÓÏË ‚ ˆËÍÛÎË‡˘Ë ÎËÏÙÓˆËÚË Ë ÒËÌË ıËÒÚËÓˆËÚË ‚ ÍÓÒÚÌËfl ÏÓÁ˙Í (Ú‡·Îˈ‡ 1). ÑÂÙËÌËÚ˂̇ڇ ‰Ë‡„ÌÓÁ‡ Ò ÔÓÒÚ‡‚fl Ò „ÂÌÂÚ˘̇ ‚ÂËÙË͇ˆËfl ËÎË ÔÓÒÚÏÓÚÂÏ. ïËÒÚÓÔ‡ÚÓÎӄ˘ÌÓ Ò ̇·Î˛‰‡‚‡Ú ͇Ùfl‚Ë ÔË„ÏÂÌÚ‡ˆËË Ì‡ „ÎÓ·ÛÒ Ô‡ÎˉÛÒ Ë ÒÛ·Òڇ̈Ëfl ÌË„‡. ÜÂÎÂÁÌË „‡ÌÛÎË Ò ÓÚÍË‚‡Ú ‚ Ì‚ÓÌË, ÏËÍӄΡÎÌË ÍÎÂÚÍË Ë ‰ÓË ÂÍÒÚ‡ˆÂÎÛ·ÌÓ. ç‡Î˘Ë ̇ ÒÙÂÓˉÌË ÚÂΈ‡, ͇ÚÓ ‡ÍÒÓ̇ÎÌË ËÁ‡ÒÚ˙ˆË Ë ÔÓÌfl-

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ÍÓ„‡ ‚˙ÚÂÍÎÂÚ˙˜ÌË ‚Íβ˜‚‡ÌËfl Ò ‡ÎÙ‡-ÒËÌÛÍÎÂËÌ (10,15). ñÂÎÚ‡ ̇ ̇¯ÂÚÓ ÔÓÛ˜‚‡Ì  ËÁÒΉ‚‡Ì ̇ ÙÂÌÓÚËÔ‡ ̇ ·˙΄‡ÒÍË Ô‡ˆËÂÌÚ Ò ‡ÚËÔ˘̇ ÙÓχ ̇ ◊ëË̉ÓÏ Ì‡ Hallervorden-Spatz”. èӂ‰ÂÌË Ò‡ ÔÓ‰Ó·ÌË ÍÎËÌ˘ÌË, Ì‚ÓÔÒËıÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl, åêí ̇ „·‚ÂÌ ÏÓÁ˙Í, ÓÙÚ‡ÎÏÓÎӄ˘̇ ÍÓÌÒÛÎÚ‡ˆËfl Ë „ÂÌÂÚ˘ÌÓ ÚÂÒÚÛ‚‡ÌÂ. éèàëÄçàÖ çÄ äãàçàóÖç ëãìóÄâ è‰ÒÚ‡‚flÏ ԇˆËÂÌÚ Ì‡ 43 „Ó‰ËÌË Ò ÓÒÌÓ‚ÌÓ Ó·‡ÁÓ‚‡ÌËÂ, ıÓÒÔËÚ‡ÎËÁË‡Ì ‚ ÍÎËÌË͇ڇ ÔÓ Ì‚ÓÎÓ„Ëfl ̇ åÅÄã “ÄÎÂÍ҇̉Ó‚Ò͇” ÔÂÁ ‡ÔËÎ 2007„ ÔÓ ÔÓ‚Ó‰ ÍÓ„ÌËÚË‚ÌË Ë ‰‚Ë„‡ÚÂÎÌË Ì‡Û¯ÂÌËfl. éÚ 13-14 „Ӊ˯̇ ‚˙Á‡ÒÚ Â Ò Ì‡Ï‡ÎÂÌË ̇ ÁÂÌËÂÚÓ, Ò ÔÓ„ÂÒË‚ÂÌ ı‡‡ÍÚÂ. ꇷÓÚËΠ ͇ÚÓ ¯ÓÙ¸Ó. éÚ 30 „Ӊ˯̇ ‚˙Á‡ÒÚ Ò ÔÓfl‚fl‚‡Ú ı‡‡ÍÚÂÓ‚Ë ÔÓÏÂÌË, ͇ÚÓ ÔÓÚËÒ̇ÚÓÒÚ, ÎËÔÒ‡ ̇ Ê·ÌËÂ Ë ËÌÚÂÂÒË, ‡Ô‡ÚËfl, ÒӈˇÎÌÓ ËÁÓÎË‡ÌÂ, ÔÂÌ·„‚‡Ì ̇ ‚˙̯ÌËfl ‚ˉ. éÚ 2002„  ÓÒ‚Ó·Ó‰ÂÌ ÓÚ ‡·ÓÚ‡, ̇ÒÚ˙Ô‚‡Ú ÔÓ„ÂÒË‚ÌË Ô‡ÏÂÚÓ‚Ë Ì‡Û¯ÂÌËfl Ë Ì‡Û¯ÂÌË ‚ ÓËÂÌÚ‡ˆËflÚ‡. ÅÎËÁÍËÚ Á‡·ÂÎflÁ‚‡Ú Á‡·‡‚flÌ ̇ ‰‚ËÊÂÌËflÚ‡ Ë Á‡·‡‚flÌ ̇ ÏËÒÎÓ‚ÌËfl ÔÓˆÂÒ. éÚ 2003„ Ò ÔÓfl‚fl‚‡Ú ÒËÎÌÓ Ó·Â‰Ìfl‚‡Ì ̇ ˜ÌË͇, ÔÓÏÂÌË ‚ „·҇ Ë „Ó‚Ó‡, ÔÓÏÂÌË ‚ ÔÓıӉ͇ڇ Ë ÔÓÁ‡Ú‡, ÔÓ„ÂÒË‚ÌÓ ÓÚÒ··‚‡ÌÂ Ò ÓÍÓÎÓ 40 Í„, ÔÓfl‚‡ ̇ “ÊÂÎflÁ̇ χÒ͇”, Á‡ÚÛ‰ÌÂÌËfl ‚ Ò‡ÏÓÒÚÓflÚÂÎÌÓÚÓ ÒÔ‡‚flÌÂ Ò Âʉ̂ÌËÚ ‰ÂÈÌÓÒÚË. Ç ÏÓÏÂÌÚ‡ Ëχ ÌÛʉ‡ ÓÚ ÔÓÏÓ˘ Ë Ì‡‰ÁÓ ÔË ËÁÔ˙ÎÌÂÌË ̇ ‚Ò˘ÍË Âʉ̂ÌË ‰ÂÈÌÓÒÚË, ‚Íβ˜ËÚÂÎÌÓ Ë ÔË ÔÓ‰‰˙ʇÌÂÚÓ Ì‡ ÒÓ·ÒÚ‚ÂÌËfl ÚÓ‡ÎÂÚ. èÂÁ 2003 „ Ò ‰Ó͇Á‚‡ ‰Ë‡„ÌÓÁ‡Ú‡ ‰Â„ÂÌÂ‡ˆËÓ ÂÚËÌ ÔË„ÏÂÌÚÓÁ‡, ‰‚ÛÒÚ‡Ì̇ ͇ڇ‡ÍÚ‡. íÓ„‡‚‡ ÔÓÎÛ˜‡‚‡ ¯ÂÌË ̇ íÖãä- Ô˙‚‡ „ÛÔ‡ ËÌ‚‡ÎˉÌÓÒÚ ·ÂÁ Ô‡‚Ó Ì‡ ÔˉÛÊËÚÂΠ̇ ·‡Á‡Ú‡ ̇ ÓÙÚ‡ÎÏÓÎӄ˘ÂÌ Ô„Ή Ë ‰‡ÌÌË Á‡ ‰ÂÏÂ̈Ëfl ÓÚ Ôӂ‰Â̇ ÍÓÌÒÛÎÚ‡ˆËfl Ò ÔÒËıˇÚ˙ (Ì ҇ Ôӂ‰ÂÌË Ì‚ÓÔÒËıÓÎӄ˘ÌË Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ËÁÒΉ‚‡ÌËfl). èË Ô‡ˆËÂÌÚ Ì Ò ÛÒÚ‡ÌÓ‚fl‚‡ Ù‡ÏËÎ̇ Ó·ÂÏÂÌÂÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌË ̇ Ì‚ÓÎӄ˘ÌË Á‡·ÓÎfl‚‡ÌËfl. Ң‡ ÏÛ Â ËχΠÔË„ÏÂÌÚÓÁ̇ ‰Â„ÂÌÂ‡ˆËfl ̇ ÂÚË̇ڇ. ÅÎËÁÍËÚ Ò˙Ó·˘‡‚‡Ú Á‡ ÒËÎÌÓ Ì‡Ï‡ÎÂÌË ̇ ÁÂÌËÂÚÓ ÔË ˜Ë˜ÓÚÓ Ë ·‡·‡Ú‡ ̇ Ô‡ˆËÂÌÚ‡ ÔÓ ·‡˘Ë̇ ÎËÌËfl. éÚ Ì‚ÓÎӄ˘ÌËfl ÒÚ‡ÚÛÒ Ò ÛÒÚ‡ÌÓ‚fl‚‡ ‡Ô‡Ú˘ÌÓ‡‰Ë̇Ï˘ÂÌ Ï˙Ê Ò˙Ò ÒËÎÌÓ Ì‡Ï‡ÎÂÌË ̇ ÁÂÌËÂÚÓ Ë Ò˙ÏÌÂÌË Á‡ ‚ÂÚË͇Î̇ ÔӄΉ̇ Ô‡ÂÁ‡; ‰ËÒÙÓÌËfl Ë ‰ËÁ‡ÚËfl; ÓÚÒ··ÂÌË ëçê, Ôӂ˜ ‚ „ÓÌËÚ Í‡ÈÌËˆË (ã>Ñ); ‚Ô˜‡ÚÎÂÌË Á‡ Ň·ËÌÒÍË ‚ ‰flÒÌÓ; ËÁ‡ÁÂ̇ ·‡‰ËÏËÏËfl, ·‡‰ËÍËÌÂÁËfl, ÎÂÍÓ Ô˂‰Â̇ ÔÓÁ‡, ÎÂÍË ‰ËÒÚÓÌ˘ÌË ÔÓfl‚Ë Ò „ËχÒÌ˘ÂÌÂ, ÎÂ͇ ˄ˉÌÓÒÚ ‚ ‰flÒ̇ڇ ˙͇, ÒËÌÍËÌÂÁËËÚ Ì ҇ Ò˙ı‡ÌÂÌË; ‡Ú‡ÍÚ˘̇ ÔÓıӉ͇ ̇ ¯ËÓ͇ ÓÒÌÓ‚‡. íÂÊÍÓ ËÁ‡ÁÂÌ ‰ÂÏÂÌÚÂÌ ÒË̉ÓÏ (MMSE=7). è‡‡ÍÎËÌ˘ÌËÚ ËÁÒΉ‚‡ÌËfl, TSH, ‚ËÚ Ç12, ÙÓÎË‚‡ ÍËÒÂÎË̇, ÌË‚ÓÚÓ Ì‡ ˆÂÛÎÓÔ·ÁÏËÌ Ë Ï‰ ‚ Í˙‚ Ë ÛË̇

퇷Îˈ‡ 1. äÎËÌ˘̇ ͇ÚË̇ ̇ ÚËÔ˘̇ Ë ‡ÚËÔ˘̇ ÙÓχ ̇ ◊Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡ Ò‚˙Á‡Ì‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl” äÎËÌ˘̇ ͇ÚË̇ Ñ·˛Ú éÒÌӂ̇ Ì‚ÓÎӄ˘̇ ͇ÚË̇ èË„ÏÂÌÚÓÁ̇ ÂÚËÌÓÔ‡ÚËfl ïÓ‰ ̇ ÔÓÚ˘‡Ì åêí ‰‡ÌÌË

ä·Ò˘ÂÒ͇ ÙÓχ Ô˙‚‡ ‰Â͇‰‡, ‡Ì̇ ÔÓ„ÂÒËfl ÂÍÒÚ‡ÔË‡ÏˉÌË ÔÓfl‚Ë, ÍÓÚËÍÓ-ÒÔË̇ÎÌÓ Á‡Òfl„‡Ì åÌÓ„Ó ˜ÂÒÚÓ á‡„Û·‡ ̇ ‚˙ÁÏÓÊÌÓÒÚ Á‡ Ôˉ‚ËÊ‚‡Ì Á‡ 10-15 „Ó‰ËÌË é˜Ë ̇ ÚË„˙

ÄÚËÔ˘̇ ÙÓχ ÇÚÓ‡ ËÎË ÚÂÚ‡ ‰Â͇‰‡, ·‡‚̇ ÔÓ„ÂÒËfl ÉÓ‚ÓÌË Ì‡Û¯ÂÌËfl, ÔÒËıˇÚ˘ÌË ÔÓfl‚Ë, Öè-ÔÓfl‚Ë, ÍÓÚËÍÓ-ÒÔË̇ÎÌÓ Á‡Òfl„‡Ì êfl‰ÍÓ á‡„Û·‡ ̇ ‚˙ÁÏÓÊÌÓÒÚ Á‡ Ôˉ‚ËÊ‚‡Ì Á‡ 15-40 „Ó‰ËÌË é˜Ë ̇ ÚË„˙


‰ÂÍÂÏ‚Ë, 2007

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

Ò‡ ‚ „‡Ìˈ‡Ú‡ ̇ ÌÓχڇ. ëÂÓÎӄ˘ÌË ËÁÒΉ‚‡ÌËfl ‚ ̇ÒÓ͇ HIV, ÒËÙËÎËÒ Ë ıÂÔÂÒÌË ‚ËÛÒË (ÒÂÛÏ Ë ÎËÍ‚Ó) Ò‡ ÓÚˈ‡ÚÂÎÌË. é·˘ÓÚÓ ËÁÒΉ‚‡Ì ̇ ÎËÍ‚Ó‡ ÔÓ͇Á‚‡ ÎÂÍÓ Í˙‚‡‚ ÎËÍ‚Ó, Ö 7.3 ı 10/9 /Î; ã‚ 6 ı 10/6; ÅÂÎÚ˙Í 0.77 „/Î. ãËÍ‚Ó̇ڇ ÂÎÂÍÚÓÙÓÂÁ‡ Â Ò ‰‡ÌÌË Á‡ ÒËÎÌÓ Û‚Â΢ÂÌË ‡ÎÙ‡-2 Ë „‡Ï‡ „ÎÓ·ÛÎËÌË. ÖÍÒÚ‡Í‡ÌˇÎ̇ Ë Ú‡ÌÒÍ‡ÌˇÎ̇ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËfl Ò‡ ‚ ÌÓχ. äí ̇ „·‚ÂÌ ÏÓÁ˙Í ‰ÓÍÛÏÂÌÚË‡ ËÁ‡ÁÂ̇ ÍÓÓ‚‡ ‡ÚÓÙËfl, Ô‰ËÏÌÓ ‚ Îfl‚‡ ÚÂÏÔÓ‡Î̇ ӷ·ÒÚ. åêí ̇ „·‚ÂÌ ÏÓÁ˙Í (ÙË„Û‡ 1) ÔÓ͇Á‚‡ ÒËÒÚÂÏ̇ Ô‡ÂÌıËÏ̇ ‡ÚÓÙËfl, ‰Ë·ÚË‡ÌË Ò‡ ‡‡ıÌÓˉÌËÚ ÔÓÒÚ‡ÌÒÚ‚‡ ÔÓ ÍÓÌ‚ÂÍÒËÚÂÚ‡ Ë ‚ÂÌÚËÍÛÎ̇ڇ ÒËÒÚÂχ. ÑËÒÍÂÚÌË ıËÔÂËÌÚÂÌÁÌË ÙÓÍÛÒË ‚˙‚ FLAIR ÓÚ ·‡Á‡ÎÌËÚ fl‰‡; ÔÓ‰˜ÂÚ‡ÌÓ ıËÔÓËÌÚÂÌÁÌË Ò˄̇ÎË ‚ ‰‚‡Ú‡ „ÎÓ·ÛÒ Ô‡ÎˉÛÒ ÚËÔ ◊Ó˜Ë Ì‡ ÚË„˙”. ÖÖÉ ÓÚÍË‚‡ ÎÂÍË Í˙Ï ÛÏÂÂÌÓ ËÁ‡ÁÂÌË ·‡‚ÌÓ‚˙ÎÌÓ‚Ë ÔÓÏÂÌË. ÖåÉ ËÁÒΉ‚‡Ì ÔÓ͇Á‚‡ ÌÓχÎ̇ ÒÍÓÓÒÚ Ì‡ ÔÓ‚Âʉ‡Ì ÔÓ ËÁÒΉ‚‡ÌËÚ ÌÂ‚Ë Ì‡ ˙ˆÂ Ë Í‡Í‡. ÖıÓ͇‰ËÓ„‡ÙËflÚ‡ Â Ò ‰‡ÌÌË Á‡ ÎÂÍË ‰Â„ÂÌÂ‡ÚË‚ÌË ÔÓÏÂÌË ÔÓ Í·ÔËÚÂ. ç‚ÓÔÒËıÓÎӄ˘ÌÓÚÓ ËÁÒΉ‡‚Ì ÛÒÚ‡ÌÓ‚fl‚‡ ÚÂÊ˙Í

267

ÍË. 阇͂‡Ú Ò ÂÁÛÎÚ‡ÚËÚ ÓÚ „ÂÌÂÚ˘ÌËfl ‡Ì‡ÎËÁ Ë ÂÎÂÍÚÓÌÌÓÏËÍÓÒÍÓÔÒÍÓ ÔÓÚ‚˙ʉÂÌË ̇ ‰Ë‡„ÌÓÁ‡Ú‡. éÅëöÜÑÄçÖ è‰ÒÚ‡‚flÏ ÒÎÛ˜‡È ̇ Ô‡ˆËÂÌÚ Ò ◊‡ÚËÔ˘̇” ÙÓχ ̇ ÒË̉ÓÏ Ì‡ Hallervorden-Spatz, ı‡‡ÍÚÂËÁË‡˘ ÒÂ Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ Ë Ò‡‚ÌËÚÂÎÌÓ ·‡‚̇ ÔÓ„ÂÒËfl, ̇΢ˠ̇ ÚÂÊ͇ ‰ÂÏÂ̈Ëfl Ë Ôӂ‰Â̘ÂÒÍË ‡ÁÒÚÓÈÒÚ‚‡ ͇ÚÓ ‚Ӊ¢‡ ÍÎËÌ˘̇ ͇ÚË̇ Ë ÔË„ÏÂÌÚÓÁÂÌ ÂÚËÌËÚ, ÂÍÒÚ‡ÔË‡ÏˉÌË ·ÂÎÂÁË, ‰ËÒÚÓÌËfl, ‰ËÁ‡ÚËfl Ë ‡Ú‡ÍÒËfl, åêí ‰‡ÌÌË Á‡ ÚËÔ˘ÌÓ ‡Ì„‡ÊË‡Ì ̇ ·‡Á‡ÎÌËÚ fl‰‡ ÚËÔ “Ó˜Ë Ì‡ ÚË„˙”, ˆÂ·‡Î̇ Ë ˆÂ·Â·̇ ‡ÚÓÙËfl. 燯ËflÚ ÒÎÛ˜‡È Ô‰ÒÚ‡‚Îfl‚‡ ËÌÚÂÂÒ ‚ ̇ÒÓ͇ fl‰ÍÓ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ, ÍÓÂÚÓ Â ÓÚÍËÚÓ Á‡ Ô˙‚Ë Ô˙Ú ‚ Å˙΄‡Ëfl. èӂ‰ÂÌË Ï‡˘‡·ÌË ÍÎËÌ˘ÌË Ë Ì‚ÓËÁÓ·‡Áfl‚‡˘Ë ËÁÒΉ‡‚ÌËfl ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒË̉ÓÏ Ì‡ HallervordenSpatz (7) Ë Ò˙ÔÓÒÚ‡‚flÌÂ Ò ‰‡ÌÌËÚ ÓÚ „ÂÌÂÚ˘ÌËÚ ‡Ì‡ÎËÁË ÔÓ͇Á‚‡Ú, ˜Â ‚Ò˘ÍËÚ ·ÓÎÌË Ò Ú˘Ë̇ Ë ‡ÚËÔ˘̇ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÍÓËÚÓ ËÏ‡Ú åêí ‰‡ÌÌË Á‡ ÒÔˆËÙ˘ÌË ÔÓÏÂÌË ÚËÔ «Ó˜Ë ̇ ÚË„˙» Ò‡ ÌÓÒËÚÂÎË Ì‡ ÏÛ-

îË„Û‡-1 åêí ̇ „·‚ÂÌ ÏÓÁ˙Í Ì‡ Ô‡ˆËÂÌÚ‡. ‰ÂÏÂÌÚÂÌ ÒË̉ÓÏ (MMSE=7). ÇÂ·‡Î̇ڇ ÂÔËÁӉ˘̇ Ô‡ÏÂÚ Â ÚÂÊÍÓ Ì‡Û¯Â̇. ìÏÂÂÌÓ Â Ì‡Û¯Â̇ ÓËÂÌÚ‡ˆËflÚ‡ Á‡ ‚ÂÏÂ Ë ÏflÒÚÓ. ÖÁËÍÓ‚‡Ú‡ ÍÓÏÛÌË͇ˆËfl  ÚÂÊÍÓ Ì‡Û¯Â̇, ͇ÚÓ ‚Ӊ¢ËflÚ ‰ÂÙˈËÚ Â ‚ ‡Á·Ë‡ÌÂÚÓ, ̇ÎËˆÂ Â Ë ‡„‡ÙËfl, ‡Í‡ÎÍÛÎËfl Ë ÚÂÊ͇ ‡Ô‡ÍÒËfl. ç‡Ô˙ÎÌÓ ÌÂÒ‡ÏÓÒÚÓflÚÂÎÂÌ Ë ‡Ô‡Ú˘ÂÌ. èӂ‰Â̇  ÍÓÌÒÛÎÚ‡ˆËfl Ò Ì‚ÓÓÙÚ‡ÎÏÓÎÓ„ Ò ‰‡ÌÌË Á‡ ÔÚÂË„ËÛÏ ‚ÂÛÏ Ì‡ Ô‰ÂÌ Ó˜ÂÌ Ò„ÏÂÌÚ ‚ ‰flÒÌÓ. 㢇ڇ  Ï˙Ú̇ Ë ÚÛ‰ÌÓ Ò ӄÎÂʉ‡Ú Ó˜ÌËÚ ‰˙̇. è‡ÔËÎËÚ ҇ Ò flÒÌË Ó˜ÂÚ‡ÌËfl Ë Ò ‰Â„ÂÌÂ‡ˆËfl ̇ ÂÚË̇ڇ. éÚÓÌ‚ÓÎӄ˘ÌÚ‡ ÍÓÒÛÎÚ‡ˆËfl ÓÚÍË‚‡ ˆÂÌÚ‡ÎÂÌ ‚ÂÒÚË·Û·ÂÌ ÒË̉ÓÏ. ç‡Ô‡‚Â̇  ÍÓÊ̇ ·ËÓÔÒËfl ÓÚ ‰flÒ̇ ‰Î‡Ì Ò ˆÂÎ ÔÓÔ‡‰‡Ì ̇ Û˜‡ÒÚ˙Í, ·ÎËÁÓ ‰Ó ÔÓÚÌË ÊÎÂÁË, ·Ó„‡Ú ̇ ÌÂ‚ÌË ÓÍÓ̘‡ÌËfl. ç‡ ÔÓÎÛÚ˙ÌÍËfl ıËÒÚÓÎӄ˘ÂÌ ÒÂÁ, ËÁ„ÓÚ‚ÂÌ ÓÚ Í‡ÔÒÛ·ڇ Á‡ ÂÎÂÍÚÓÌÌÓ ÏËÍÓÒÍÓÔÒÍÓ ËÁÒΉ‚‡Ì Ò ̇·Î˛‰‡‚‡Ú χÒÚÌË ÊÎÂÁË Ë ÓÍÓÎ̇ Ò˙‰ËÌËÚÂÎ̇ Ú˙Í‡Ì Ì‡ ‰Âχڇ. ÇËÁÛ‡ÎËÁË‡ÌÂÚÓ Ì‡ ÓÚÓ˜ÌË ÌÂ‚ÌË ÓÍÓ̘‡ÌËfl (ÒÙÂÓˉË)  ‚˙ÁÏÓÊÌÓ Ò‡ÏÓ ÂÎÂÍÚÓÌÌÓÏËÍÓÒÍÓÔÒ-

Ú‡ˆËfl ‚ PKAN 2 „Â̇ Ë Ó·‡ÚÌÓ, ÚÓÁË ÒÔˆËÙ˘ÂÌ ·Â΄ Ì  ÓÚÍËÚ ÔË ÌËÚÓ Â‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÍÓËÚÓ Ì ҇ ÌÓÒËÚÂÎË Ì‡ ÏÛÚ‡ˆËfl. éÚ ‰Û„‡ ÒÚ‡Ì‡, ÏÂÊ‰Û Ô‡ˆËÂÌÚËÚÂ Ò ‡ÚËÔ˘̇ ÍÎËÌ˘̇ ͇ÚË̇ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÚÂÁË ÍÓËÚÓ Ò‡ ÌÓÒËÚÂÎË Ì‡ ÏÛÚ‡ˆËfl Ò‡ Ò ÔÓ-˜ÂÒÚË Ôӂ‰Â̘ÂÒÍË ‡ÁÒÚÓÈÒÚ‚‡ Ë „Ó‚ÓÌË Ì‡Û¯ÂÌËfl ÓÚÍÓÎÍÓÚÓ ÚÂÁË ·ÂÁ ÏÛÚ‡ˆËfl (7,13,14). ëÔÓ‡‰Ë˜ÌËflÚ ı‡‡ÍÚÂ ̇ ̇¯Ëfl ÒÎÛ˜‡È, ÍÓÈÚÓ Â ‚ ÔÓ‰ÍÂÔ‡ ̇ ‡‚ÓÁÓÏÌÓ-ÂÒˆÂÒË‚ÌÓÚÓ Û̇ÒΉfl‚‡ÌÂ, åêí ‰‡ÌÌËÚÂ Ë ÒËÎÌÓÚÓ ÔËÒ˙ÒÚ‚Ë ̇ Ôӂ‰Â̘ÂÒÍË Ë „Ó‚ÓÌË/ÂÁËÍÓ‚Ë Ì‡Û¯ÂÌËfl, ÔÓ‰ÍÂÔflÚ ‰Ë‡„ÌÓÁ‡Ú‡ ◊Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡-Ò‚˙Á‡Ì‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl”, ‰ÓË ÔÂ‰Ë „ÂÌÂÚ˘̇ڇ ‚ÂËÙË͇ˆËfl ̇ ÒÎÛ˜‡fl. ÉÂÌÂÚ˘̇ڇ ‚ÂËÙË͇ˆËfl ˘Â ‰ÓÔËÌÂÒÂ Ë Á‡ ‚˙ÁÏÓÊÌÓÒÚ Á‡ ÚÂ‡Ô‚Ú˘̇ ËÌÚÂ‚Â̈Ëfl, ÔÓ‡‰Ë Ù‡ÍÚ‡, ˜Â Ôӂ˜ÂÚÓ Ô‡ˆËÂÌÚË Ò ‡ÚËÔ˘̇ ÙÓχ Ë ◊Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡-Ò‚˙Á‡Ì‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl” ËÏ‡Ú ÔÓ„¯ÌÓ ÒÏËÒÎÓ‚‡ ÏÛÚ‡ˆËË Ë Â ‚˙ÁÏÓÊÌÓ ‰‡ ËÏ‡Ú ÂÁˉۇÎ̇ ‡ÍÚË‚ÌÓÒÚ Ì‡ Ô‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡ 2. ë˙˘ÂÒÚ‚Û‚‡ ıËÔÓÚÂÁ‡ Á‡ Á‡·‡‚flÌ ̇ ÒËÏÔÚÓÏËÚ ˜ÂÁ ‰Ó·‡‚͇ ̇ Ô‡ÌÚÓÚÂÌ‡Ú (‚ËÚ Ç5), ÍÓÈÚÓ ÏÓÊ ˜‡ÒÚ˘ÌÓ ‰‡ ÍÓÏÔÂÌÒË‡ ‰Â-


‰ÂÍÂÏ‚Ë, 2007

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

ÙˈËÚ‡ ̇ ÚÓÁË ÂÌÁËÏ. ç‡Î˘ËÂÚÓ Ì‡ ÔË„ÏÂÌÚÓÁ̇ڇ ÂÚËÌÓÔ‡ÚËfl ̇·„‡ ÓÚ‰ÂÎ̇ ‰ËÒÍÛÒËfl ÔË Ì‡¯Ëfl Ô‡ˆËÂÌÚ, Ӣ ÔÂ‰Ë „ÂÌÂÚ˘ÌÓÚÓ ‚ÂËÙˈË‡Ì ̇ ÒÎÛ˜‡fl. èË„ÏÂÌÚÓÁ̇ڇ ÂÚËÌÓÔ‡ÚËfl  fl‰˙Í, ‡ÌÌÓ ÔÓfl‚fl‚‡˘ Ò ÒËÏÔÚÓÏ ÔË ‡ÚËÔ˘̇ڇ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ. éÚ ‰Û„‡ ÒÚ‡Ì‡, ÔË Ì‡¯Ëfl Ô‡ˆËÂÌÚ Ì‡Î˘ËÂÚÓ Ì‡ Ò‡ÏÓÒÚÓflÚÂÎ̇ ÔÓÎÓÊËÚÂÎ̇ Ù‡ÏËÎ̇ Ó·ÂÏÂÌÂÌÓÒÚ Á‡ ÔË„ÏÂÌÚÓÁ̇ ÂÚËÌÓÔ‡ÚËfl ‚Ó‰Ë ‰Ó Ò˙ÏÌÂÌË Á‡ Ò˙Ô˙ÚÒÚ‚‡˘‡ ̇ÒΉÒÚ‚Â̇ ÔË„ÏÂÌÚÓÁ̇ ÂÚËÌÓÔ‡ÚËfl ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÒË̉Óχ Hallervorden-Spatz. èÓ Ú‡ÁË Ô˘Ë̇  Ô‰‚ˉÂÌ „ÂÌÂÚ˘ÂÌ ‡Ì‡ÎËÁ Ë ‚ ̇ÒÓ͇ ̇ÒΉÒÚ‚Â̇ ÔË„ÏÂÌÚÓÁ̇ ÂÚËÌÓÔ‡ÚËËfl. éÚ „ÓÎflÏÓ Á̇˜ÂÌË  ‰‡ Ò ÛÒÚ‡ÌÓ‚Ë ÒÔˆËÙË͇ڇ ̇ ÍÎËÌ˘ÌËÚ ÒË̉ÓÏË Ò ‰ÂÏÂ̈Ëfl Ë/ËÎË ‰‚Ë„‡ÚÂÎÌË ‡ÁÒÚÓÈÒÚ‚‡ ‚ Ò˙ÓÚ‚ÂÚÒÚ‚ËÂ Ò Ì‚ÓÔ‡ÚÓÎӄ˘̇ڇ ‰Ë‡„ÌÓÁ‡ ̇ ‰‡‰ÂÌÓ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎfl‚‡ÌÂ. äÎËÌ˘̇ڇ ‰ËÙÂÂ̈ˇÎ̇ ‰Ë‡„ÌÓÁ‡ ˜ÂÒÚÓ Â ÏÌÓ„Ó Úۉ̇, Á‡˘ÓÚÓ Â‰ËÌ Ë Ò˙˘ ÍÎËÌ˘ÂÌ ‰ÂÏÂÌÚÂÌ ÒË̉ÓÏ ÏÓÊ ‰‡ Ò ̇·Î˛‰‡‚‡ ÔË ÌflÍÓÎÍÓ Á‡·ÓÎfl‚‡ÌËfl. Ç ÒÎÛ˜‡fl, Ì‚ÓËÁÓ·‡Áfl‚‡˘ËÚ ÏÂÚÓ‰Ë Ò‡ ÓÒÌÓ‚ÂÌ ËÌÒÚÛÏÂÌÚ Á‡ ÔÓÒÚ‡‚flÌ ̇ ‰Ë‡„ÌÓÁ‡Ú‡, ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ÍÎËÌ˘ÌËÚ ‰‡ÌÌË Ë „ÂÌÂÚ˘ÌËÚ ËÁÒΉ‚‡ÌËfl. é˘Â Ôӂ˜Â, ˜Â åêí ÔÓÏÂÌËÚ ÔÓÌflÍÓ„‡ ̇ÒÚ˙Ô‚‡Ú ÔÂ‰Ë ÔÓfl‚‡Ú‡ ̇ ÒËÏÔÚÓÏËÚÂ. àχ Ò˙Ó·˘ÂÌË ̇ åêí ‰‡ÌÌË Á‡ ÚËÔ˘ÌÓ ‡Ì„‡ÊË‡Ì ̇ ·‡Á‡ÎÌËÚ „‡Ì„ÎË ÔË ÔÂÒËÏÔÚÓχÚ˘ÌË Ó‰ÌËÌË Ì‡ Á‡Ò„̇ÚË Ô‡ˆËÂÌÚË (7). è‡ÌÚÓÚÂÌ‡Ú ÍË̇Á‡-Ò‚˙Á‡Ì‡Ú‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl  ‰ÌÓ ÓÚ ÚËÚ ÂÍÒÚ‡ÔË‡ÏˉÌË Á‡·ÓÎfl‚‡ÌËfl, ‡ÒÓˆËË‡ÌË Ò Ôӂ˯ÂÌÓ ÓÚ·„‡Ì ̇ ÊÂÎflÁÓ ‚ ÏÓÁ˙͇. ÑÛ„ËÚ ‰‚ ҇ Ì‚ÓÙÂËÚËÌÓÔ‡ÚËflÚ‡ Ò ÏÛÚ‡ˆËfl ‚ ÎÂÍÓ ‚ÂËÊÌËfl ÙÂËÚËÌ „ÂÌ Ë ‡ˆÂÛÎÓÔ·ÁÏËÌÂÏËfl Ò ÏÛÚ‡ˆËfl ‚ „Â̇ Á‡ ˆÂÛÎÓÔ·ÁÏËÌ, ÌÓ Ë ‰‚ÂÚ ҇ Ò ‰Â·˛Ú ‚ Áfl· ‚˙Á‡ÒÚ Ë ÏÓ„‡Ú ‰‡ Ò ‰ËÙÂÂ̈Ë‡Ú ÓÚ Ô‡ÌÚÓÚÂ̇Ú-ÍË̇Á‡ Ò‚˙Á‡Ì‡Ú‡ Ì‚Ӊ„ÂÌÂ‡ˆËfl ̇ ·‡Á‡Ú‡ ̇ ÍÎËÌ˘ÌË ÔÓfl‚Ë, åêí ‰‡ÌÌË Ë „ÂÌÂÚ˘ÌÓ ÚÂÒÚÛ‚‡Ì (1,7). Ç Á‡Íβ˜ÂÌËÂ, ÓÔËÒ‚‡Ï ÒÎÛ˜‡È Ò ‡ÚËÔ˘̇ ÙÓχ ̇ ◊ëË̉ÓÏ Ì‡ Hallervorden-Spatz” Ò Í˙ÒÌÓ Ì‡˜‡ÎÓ, ·‡‚̇ ÔÓ„ÂÒËfl Ò ÚËÔ˘ÌÓ ‡Ì„‡ÊË‡Ì ̇ ·‡Á‡ÎÌËÚ „‡Ì„ÎËË ÚËÔ ◊Ó˜Ë Ì‡ ÚË„˙”, ‚ Ò˙˜ÂÚ‡ÌËÂ Ò ‰ÂÏÂ̈Ëfl, Ôӂ‰Â̘ÂÒÍË ‡ÁÒÚÓÈÒÚ‚‡ Ë ÔË„ÏÂÌÚÓÁ̇ ÂÚËÌÓÔ‡ÚËfl. íÓ‚‡  Ô˙‚ËflÚ ÒÎÛ˜‡È ̇ ◊ëË̉ÓÏ Ì‡ Hallervorden-Spatz” ‚ Å˙΄‡Ëfl. 臈ËÂÌÚËÚÂ, ÍÓËÚÓ Ò‡ Ò ‡ÚËÔ˘̇ ÙÓχ ̇ Á‡·ÓÎfl‚‡ÌÂÚÓ, ÔˉÛÊÂÌË ÓÚ „Ó‚ÓÌË, ÂÁËÍÓ‚Ë Ë ÔÓ‚Â-

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‰Â̘ÂÒÍË Ì‡Û¯ÂÌËfl Ë åêí ‰‡ÌÌË Á‡ ◊Ó˜Ë Ì‡ ÚË„˙” ÔÓ‰ÎÂÊ‡Ú Ì‡ „ÂÌÂÚ˘ÌÓ ÚÂÒÚÛ‚‡Ì Á‡ ÏÛÚ‡ˆËË ‚ PANK 2 „Â̇. êÂÁÛÎÚ‡ÚËÚÂ, ÔÓÎÛ˜ÂÌË ÓÚ ÏÓÎÂÍÛÎflÌÓ „ÂÌÂÚ˘ÌËÚ ËÁÒΉ‚‡ÌËfl, ÓÚÍË‚‡Ú ÔÂÒÔÂÍÚË‚‡ Ë Á‡ ÔÓ-ÂÙË͇ÒÌÓ ÚÂ‡Ô‚Ú˘ÌÓ ‚˙Á‰ÂÈÒÚ‚ËÂ. Å·„Ó‰‡ÌÓÒÚË èÓ-„Ó·χڇ ˜‡ÒÚ ÓÚ Ò‰ÒÚ‚‡Ú‡ Á‡ ÓÒ˙˘ÂÒÚ‚fl‚‡Ì ̇ ‰Ë‡„ÌÓÒÚ˘ÌËfl ÔÎ‡Ì ·flı‡ ÓÒË„ÛÂÌË ÔÓ ÔÓÂÍÚ ◊åÛÎÚˉËÒˆËÔÎË̇ÌÓ ÔÓÛ˜‚‡Ì Á‡ ÒÍËÌËÌ„, ‰Ë‡„ÌÓÒÚË͇ Ë ÍÓ„ÌËÚ˂̇ Âı‡·ËÎËÚ‡ˆËfl ̇ Ì‚Ӊ„ÂÌÂ‡ÚË‚ÌËÚ Á‡·ÓÎfl‚‡ÌËfl Ò ‰ÂÏÂÌÚÂÌ ÒË̉ÓÏ ‚ Å˙΄‡Ëfl” Á‡ 2006 „., ÙË̇ÌÒË‡Ì ÓÚ ÂÍÚÓ‡Ú‡ ̇ å‰ˈËÌÒÍË ìÌË‚ÂÒËÚÂÚëÓÙËfl. ãàíÖêÄíìêÄ 1.

2. 3. 4. 5. 6. 7. 8. 9. 10.

11. 12. 13. 14. 15.

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