Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ë‰ÛÊÂÌËÂ ◊Å˙Î„‡ÒÍÓ ‰ÛÊÂÒÚ‚Ó ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ“ Official Journal of The Bulgarian Society of Neurology êÖÑÄäñàéççÄ äéãÖÉàü Publisher Color Studio® ÄÎÂÍÒËÂ‚ Ä. Å‡Î‰‡‡ÌÓ‚ Ñ. ÅÓÊËÌÓ‚ ëÚ. ÅÓÊËÌÓ‚‡ Ç Ç‡ÒËÎÂ‚‡ Ö. Ç‡ÒËÎÂ‚‡ í. É‡ÌÂ‚‡ É. ÉÂÓ„ËÂ‚ Ñ. ÉÂ‡ÒËÏÓ‚ Å. ÉÓÁÏ‡ÌÓ‚ É. ÉË„ÓÓ‚‡ é. ÑÂÎÂ‚‡ ç. á‡ı‡ËÂ‚ á. äÓÎÂ‚ é. äÓÎÂ‚ è. å‡Ì˜Â‚ à. å‡ÒÎ‡Ó‚ Ñ. åËÎ‡ÌÓ‚ à. åËÎ‡ÌÓ‚‡ å. åËÌ˜Â‚ Ñ. èÂÚÓ‚ à. èÂÚÓ‚‡ û. ê‡È˜Â‚ à. ê‡È˜Â‚‡ å. ëÚ‡ÏÂÌÓ‚ Å. íËÚﬂÌÓ‚‡ Ö. í‡ÈÍÓ‚ ã. í˙ÌÂ‚ à. ï‡‚ÂÁÓ‚‡ ã. ï‡‡Î‡ÌÓ‚ ã. ó‡Î˙ÍÓ‚‡ ç. óÂÌËÌÍÓ‚‡ ë. òÓÚÂÍÓ‚ è. üÌ˜Â‚‡ ë. ëÓÙËﬂ ëÓÙËﬂ èÎÂ‚ÂÌ ëÓÙËﬂ ëÓÙËﬂ èÎÓ‚‰Ë‚ ëÓÙËﬂ ëÓÙËﬂ ëÓÙËﬂ èÎÓ‚‰Ë‚ ëÓÙËﬂ Ç‡Ì‡ èÎÓ‚‰Ë‚ ëÓÙËﬂ ëÓÙËﬂ ëÚ.á‡„Ó‡ ëÓÙËﬂ ëÓÙËﬂ ëÓÙËﬂ Ç‡Ì‡ ëÓÙËﬂ ëÓÙËﬂ ëÓÙËﬂ ëÓÙËﬂ èÎÂ‚ÂÌ ëÓÙËﬂ ëÓÙËﬂ ëÓÙËﬂ Ç‡Ì‡ ëÓÙËﬂ èÎÓ‚‰Ë‚ ëÓÙËﬂ ëÓÙËﬂ ëÓÙËﬂ EDITORS Alexiev A. Baldaranov D. Bojinov St. Bojinoba V. Vassileva E. Vassileva T. Ganeva G. Georgiev D. Gerassimov B. Gozmanov G. Grigorova O. Deleva N. Zahariev Z. Kolev O. Kolev P. Manchev I. Maslarov D. Milanov I. Milanova M. Minchev D. Petrov I. Petrova U. Raychev I. Raycheva M. Stamenov B. Titianova E. Traykov L. Tarnev I. Havezova L. Haralanov L. Chalakova N. Charninkova S. Shotekov P. Yancheva S. Sofia Sofia Pleven Sofia Sofia Plovdiv Sofia Sofia Sofia Plovdiv Sofia Varna Plovdiv Sofia Sofia St. Zagora Sofia Sofia Sofia Varna Sofia Sofia Sofia Sofia Pleven Sofia Sofia Sofia Varna Sofia Plovdiv Sofia Sofia Sofia ÉãÄÇçà êÖÑÄäíéêà: à. åËÎ‡ÌÓ‚, è. ëÚ‡ÏÂÌÓ‚‡, à. ÇÂÎ˜Â‚‡ EDITOR IN CHIEF: I. Milanov, P. Stamenova, I. Velcheva ëÖäêÖíÄê: å. Ñ‡ÒÍ‡ÎÓ‚ SECRETARY: M. Daskalov Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ìäÄáÄçàÖ áÄ ÄÇíéêàíÖ Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Â ÓÙËˆË‡ÎÂÌ Ó„‡Ì Ì‡ ë‰ÛÊÂÌËÂ ◊Å˙Î„‡ÒÍÓ ‰ÛÊÂÒÚ‚Ó ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ“ Ë ÔÛ·ÎËÍÛ‚‡ ÒÚ‡ÚËË ÓÚ ‚ÒË˜ÍË Ó·Î‡ÒÚË Ì‡ ÌÂ‚ÓÎÓ„ËﬂÚ‡. ëÔËÒ‡ÌËÂÚÓ Ò˙‰˙Ê‡ ÒÎÂ‰ÌËÚÂ Û·ËÍË: ● êÂ‰‡ÍˆËÓÌÌ‡ ÒÚ‡ÚËﬂ Ò ÚÂÍÒÚ ‰Ó3 ÒÚ‡ÌËˆË. Ç˙ÁÎ‡„‡ ÒÂ ÓÚ êÂ‰ÍÓÎÂ„ËﬂÚ‡. ● éË„ËÌ‡ÎÌË ÒÚ‡ÚËË - ‰Ó 8 ÒÚ‡ÌËˆË, ‚ÍÎ˛˜ËÚÂÎÌÓ Ú‡·ÎËˆË, ÙË„ÛË, ÍÌË„ÓÔËÒ. ä˙Ï ÚÂÁË ÒÚ‡ÚËË ÒÂ ËÁËÒÍ‚‡ ÂÁ˛ÏÂ Ì‡ ·˙Î„‡ÒÍË Ë ‡Ì„ÎËÈÒÍË ÂÁËÍ Ó·˘Ó ‰Ó 40 Â‰‡, ÓÚÔÂ˜‡Ú‡ÌË Ì‡ ÓÚ‰ÂÎÌË ÒÚ‡ÌËˆË. ● êÂÁ˛ÏÂÚÓ Úﬂ·‚‡ ‰‡ Ò˙‰˙Ê‡ Á‡„Î‡‚ËÂ, ËÏÂÌ‡Ú‡ Ì‡ ‡‚ÚÓËÚÂ, ËÌÙÓÏ‡ˆËﬂ Á‡ ˆÂÎÚ‡ Ë Ó·ÂÍÚ‡ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ, ÏÂÚÓ‰ËÍËÚÂ, ÔÓÎÛ˜ÂÌËÚÂ ÂÁÛÎÚ‡ÚË. èÓÒÓ˜‚‡Ú ÒÂ ‰Ó 6 ÍÎ˛˜Ó‚Ë ‰ÛÏË. ● éË„ËÌ‡ÎÌËÚÂ ÒÚ‡ÚËË ‚ÍÎ˛˜‚‡Ú Í‡Ú˙Í Û‚Ó‰, ÍÓÌÚËÌ„ÂÌÚ, ÏÂÚÓ‰Ë, ÂÁÛÎÚ‡ÚË, Ó·Ò˙Ê‰‡ÌÂ Ë ÍÌË„ÓÔËÒ. ● á‡„Î‡‚Ì‡Ú‡ ÒÚ‡ÌËˆ‡ Ò˙‰˙Ê‡ Ô˙ÎÌÓ Ë Ò˙Í‡ÚÂÌÓ Á‡„Î‡‚ËÂ, ËÏÂÌ‡Ú‡ Ì‡ ‡‚ÚÓËÚÂ Ò ËÌËˆË‡ÎËÚÂ ËÏ, ÚÂıÌËÚÂ ‡Í‡‰ÂÏË˜ÌË ÒÚÂÔÂÌË Ë ÏÂÒÚÓ‡·ÓÚ‡, ‡‰ÂÒ Á‡ ÍÓÂÒÔÓÌ‰ÂÌˆËﬂ Ò ÚÂÎÂÙÓÌ, Ù‡ÍÒ Ë Â-mail. ● ä‡ÚÍË Ì‡Û˜ÌË Ò˙Ó·˘ÂÌËﬂ ‰Ó 3 ÒÚ‡ÌËˆË ● é·ÁÓÌË ÒÚ‡ÚËË ‰Ó 10 ÒÚ‡ÌËˆË ● àÌÙÓÏ‡ˆËË Ë ÂˆÂÌÁËË Ì‡ ÍÌË„Ë . ë˙‰˙Ê‡ ËÌÙÓÏ‡ˆËﬂ Á‡ ÍÓÌ„ÂÒË Ë ÍÓÌÙÂÂÌˆËË, ÌÓ‚Ë ÍÌË„Ë, ‡ÍÚÛ‡ÎÌ‡ ÎÂÍ‡ÒÚ‚ÂÌ‡ ËÌÙÓÏ‡ˆËﬂ, ÔÂ‰ÒÚÓﬂ˘Ë Ò˙·ËÚËﬂ. ● ëÚ‡ÌËˆË Ì‡ ˜ËÚ‡ÚÂÎﬂ. èÓÏÂÒÚ‚‡Ú ÒÂ ÔËÒÏ‡ ‰Ó Â‰‡ÍÚÓ‡, ‚ ÍÓËÚÓ ÒÂ ÍÓÏÂÌÚË‡Ú ÔÛ·ÎËÍÛ‚‡ÌË Ï‡ÚÂË‡ÎË, Í‡ÚÍË ÓÔËÒ‡ÌËﬂ Ì‡ ÒÎÛ˜‡Ë Ë ‰ËÒÍÛÒËË ÔÓ ‡ÍÚÛ‡ÎÌË ÔÓ·ÎÂÏË . ● ëÚ‡ÚËËÚÂ Úﬂ·‚‡ ‰‡ ·˙‰‡Ú ÔÂ‰ÒÚ‡‚ÂÌË Ì‡ CD/USB, Á‡ÔËÒ‡ÌË Ì‡ Â‰‡ÍÚÓÒÍ‡ ÔÓ„‡Ï‡ Word 6/Windows 96 ËÎË Windows Microsoft Word 97/2000XP, Ò Â‰ËÌ ÂÍÁÂÏÔÎﬂ ‡ÁÔÂ˜‡ÚÍ‡. ● í‡·ÎËˆËÚÂ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ Ì‡ ÓÚ‰ÂÎÂÌ ÎËÒÚ, ÌÓÏÂË‡ÌË, Í‡ÚÓ ‚ ÚÂÍÒÚ‡ ÒÂ ÓÚ·ÂÎÂÊË ÏﬂÒÚÓÚÓ ËÏ. í‡·ÎËˆËÚÂ ‰‡ ËÏ‡Ú Í‡ÚÍÓ Á‡„Î‡‚ËÂ. ● àÎ˛ÒÚ‡ˆËËÚÂ (ÙË„ÛË, ‰Ë‡„‡ÏË, ÙÓÏÛÎË) Úﬂ·‚‡ ‰‡ Ò‡ „ÓÚÓ‚Ë Á‡ ÌÂÔÓÒÂ‰ÒÚ‚ÂÌÓ ÔÓÎË„‡ÙË˜ÌÓ ‚˙ÁÔÓËÁ‚ÂÊ‰‡ÌÂ. íÂÍÒÚÓ‚ÂÚÂ ÔÓ‰ ÙË„ÛËÚÂ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ Ì‡ ÓÚ‰ÂÎÂÌ ÎËÒÚ ● äÌË„ÓÔËÒ˙Ú ‰‡ ÒÂ ÓÚÔÂ˜‡Ú‡ Ì‡ ÓÚ‰ÂÎÂÌ ÎËÒÚ. Ä‚ÚÓËÚÂ ‰‡ ÒÂ ÔÓ‰ÂÊ‰‡Ú ÔÓ ‡Á·Û˜ÂÌ Â‰, Í‡ÚÓ ‚ Ì‡˜‡ÎÓÚÓ ÒÂ ËÁ·Óﬂ‚‡Ú ËÁÚÓ˜ÌËˆËÚÂ Ì‡ ÍËËÎËˆ‡, ‡ ÒÎÂ‰ Úﬂı Ì‡ Î‡ÚËÌËˆ‡. á‡„Î‡‚ËﬂÚ‡ ‰‡ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ ËÁˆﬂÎÓ. Ç ÚÂÍÒÚ‡ ˆËÚË‡ÌËÚÂ ‡‚ÚÓË ‰‡ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ Ò ÔÓÂ‰ÂÌ ÌÓÏÂ ÓÚ ÍÌË„ÓÔËÒ‡. Ñ‡ÌÌËÚÂ ‚ ÍÌË„ÓÔËÒ‡ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ ÔÓ ÒÎÂ‰ÌËﬂ Ì‡˜ËÌ: ● ëÚ‡ÚËﬂ ÓÚ ÒÔËÒ‡ÌËÂ : Ä‚ÚÓ /Ë/. á‡„Î‡‚ËÂ Ì‡ ÒÚ‡ÚËﬂÚ‡. á‡„Î‡‚ËÂ Ì‡ ÒÔËÒ‡ÌËÂÚÓ / Ò˙Í‡ÚÂÌÓ ÔÓ Index medicus/, ÚÓÏ, „Ó‰ËÌ‡ Ì‡ ËÁ‰‡‚‡ÌÂ, ÌÓÏÂ Ì‡ ÍÌËÊÍ‡Ú‡, ÒÚ‡ÌËˆ‡ / ÓÚ-‰Ó/. èËÏÂ: Andersen, G., Vestergaard, K., Lauritzen, L. Effective treatment of poststroke depression with the selective serotonin reuptake inhibitor citalopram. Stroke, 25, 1994, 6, 1099-1104. ● äÌË„‡: Ä‚ÚÓ /Ë/ . á‡„Î‡‚ËÂ. èÓ‰Á‡„Î‡‚ÌË ‰‡ÌÌË. åÂÒÚÓËÁ‰‡‚‡ÌÂ, ËÁ‰‡ÚÂÒÚ‚Ó, „Ó‰ËÌ‡ Ì‡ ËÁ‰‡‚‡ÌÂ, ÒÚ‡ÌËˆ‡ /ÓÚ-‰Ó/.èËÏÂ : Calligaro,K., DeLaurentis, D., Baker, W. Management of Extracranial Cerebrovascular Disease.Philadelphia-New York, Lippincott - Raven Publishers, 1997, 217. ● èÛ·ÎËÍ‡ˆËË ÓÚ Ò·ÓÌËÍ : Ä‚ÚÓ /Ë/. á‡„Î‡‚ËÂ: - Ç: á‡„Î‡‚ËÂ Ì‡ Ò·ÓÌËÍ‡. àÁ‰‡ÚÂÎ /Ë/. åÂÒÚÓËÁ‰‡‚‡ÌÂ, „Ó‰ËÌ‡ Ì‡ ËÁ‰‡‚‡ÌÂ, ÒÚ‡ÌËˆ‡ / ÓÚ-‰Ó/. èËÏÂ : Binnie, C., Jeavsons , .M. Photosensitive epilepsies. In : Epileptic syndromes in infancy, childhood and adolescence , eds. J.Roger, M. Bureau, Ch.Dravet, F.E. Dreifuss, A.Perret , P.Wolf. London, John Libbey & Company, Ltd, 1992, 299-305. ● Ä‚ÚÓËÚÂ ÔÓ‰ÔËÒ‚‡Ú ‰ÂÍÎ‡‡ˆËﬂ, ˜Â ÔÂ‰ÓÒÚ‡‚ÂÌËﬂ Ï‡ÚÂË‡Î ÌÂ Â ÔÂ‰ÎÓÊÂÌ Á‡ ÔÂ˜‡Ú Ì‡ ‰Û„Ó ÏﬂÒÚÓ. ● èÂ‰ÓÒÚ‡‚ÂÌËÚÂ Ï‡ÚÂË‡ÎË Ë ÓÔËÒ‡ÌËÚÂ ‚ Úﬂı ËÁÒÎÂ‰‚‡ÌËﬂ Úﬂ·‚‡ ‰‡ ÓÚ„Ó‚‡ﬂÚ Ì‡ ÂÚË˜ÌËÚÂ ÒÚ‡Ì‰‡ÚË ● èË Ò˙‡‚ÚÓÒÚ‚Ó ÛÚ‚˙‰ÂÌ‡Ú‡ Á‡ ÔÂ˜‡Ú ÒÚ‡ÚËﬂ Úﬂ·‚‡ ‰‡ ·˙‰Â ÔÓ‰ÔËÒ‡Ì‡ ÓÚ ‚ÒË˜ÍË ‡‚ÚÓË. ê˙ÍÓÔËÒËÚÂ ‚ ‰‚‡ ÂÍÁÂÏÔÎﬂ‡ (ËÎË Â‰ËÌ ÂÍÁÂÏÔÎﬂ Ë CD/USB) ËÁÔ‡˘‡ÈÚÂ Ì‡ ‡‰ÂÒ: ëÓÙËﬂ 1504, ìÎ. 4 ÅﬂÎÓ ÏÓÂ " ‹ 8 åÅÄã " ñ‡Ëˆ‡ âÓ‡ÌÌ‡" - ÖÄÑ äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ èÓÙ. è.ëÚ‡ÏÂÌÓ‚‡ INSTRUCTION FOR AUTHORS Bulgarian Neurology is the official journal of the Bulgarian Society of Neurology. It will consider for publication papers in neurology and related areas in the following categories: ● Editorials, consisting of up to 3 pages, when approved by the Editorial Board ● Original papers - up to 8 pages, including tables, figures and references. An abstract in Bulgarian and English up to 40 rows on a separate sheet is required. The abstract should contain title, authors, objective, background, methods, results and conclusions plus up to 6 keywords. ● The original papers include short introduction, material, methods results, discussion and references. ● The title page should carry the full title, the short running title, the authors with their initials, academic degrees, institutional affiliations, address for correspondence, with telephone, fax and e-mail ● Short communications and case reports up to 3 pages ● Review articles up to 10 pages ● Book reviews and information. It includes information for new books, congresses and conferences, new drugs, future events in neurology ● Letters to the Editor with comments on previously published papers, short case reports and discussion on current problems ● The manuscripts should be submitted on CD/USB, using Word 6/Windows 96 or Windows Microsoft Word 97/2000XP with a printed copy. ● The tables should be presented on separate sheets with a short heading ● The illustrations /figures, diagrams, formulas/ should be ready for reproduction. Explanatory legends should be provided on a separate sheet of paper. References should be presented in alphabetic order on a separate sheet with all authors` names and full title of papers. In the text the authors should be indicated by the number from the reference list . ● The reference should be presented as follows: ● Journal paper: (1) author(s), (2) title, (3) journal name ( as abbreviated in Index Medicus ). (4) volume, (5) year of publication, (6) journal number, (7) inclusive pages. Example: Andersen, G., Vestergaard, K., Lauritzen, L. Effective treatment of poststroke depression with the selective serotonin reuptake inhibitor citalopram. Stroke, 25, 1994, 6, 1099-1104. ● Book: (1) author(s), (2) title, (3) city of publication, (4) publisher, (5) year of publication , (6) pages. Example: Calligaro,K., DeLaurentis, D., Baker, W. Management of Extracranial Cerebrovascular Disease. Philadelphia-New York, Lippincott - Raven Publishers, 1997, 217. ● References to books: (1) authors(s), (2) chapter title, (3) title of book, (4) editor(s), (5) city of publication, (6) publisher, (7) year of publication, (8) specific pages. Example: Binnie, C., Jeavsons, M. Photosensitive epilepsies. In: Epileptic syndromes in infancy, childhood and adolescence, eds. J.Roger, M. Bureau, Ch.Dravet, F.E. Dreifuss, A.Perret, P.Wolf. London, John Libbey & Company, Ltd, 1992, 299-305. ● The articles are considered for publication on the understanding that the presented material has not been published or is being submitted elsewhere before appearing in BULGARIAN NEUROLOGY. ● Authors should indicate that ethical approval of the study was granted and that informed consent was given. ● All authors should approve and sign the final manuscript. Manuscript with one copy (or a CD/USB and a copy) should be sent to the following address: Sofia 1504, Bulgaria, 8, Bialo more str. Department of Neurology University Hospital ◊ Queene Jovanna“ Prof. P. Stamenova Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ëöÑöêÜÄçàÖ CONTENTS éÅáéêà REVIEWS ëíÄíàçàíÖ - ÑöãÉéëêéóçÄ ëíêÄíÖÉàü Ç èêÖÇÖçñàüíÄçÄ åéáöóçéëöÑéÇÄíÄ ÅéãÖëí Ö‚„ÂÌËﬂ Ç‡ÒËÎÂ‚‡ . . . . . . . . . . . . . . . . . . . . . . . . . . . .143 STATINS AND PREVENTION OF CEREBROVASULAR DISORDERS Ö. Vassileva . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .143 ÇÄêàÄÅàãçéëí çÄ ëöêÑÖóçÄíÄ óÖëíéíÄ: åÖíéÑ áÄ éñÖçäÄ çÄ ëöêÑÖóçÄíÄ ÄÇíéçéåçÄ êÖÉìãÄñàü èêà çÖÇêéãéÉàóçà áÄÅéãüÇÄçàü ë. å‡ÌÚ‡Ó‚‡, ç. ÑÓÒ. ÑËÏËÚÓ‚, à. ÇÂÎ˜Â‚‡ . . . .148 HEART RATE VARIABILITY: A METHOD FOR ASSESSMENT OF CARDIAC AUTONOMIC REGULATION IN NEUROLOGICAL DISEASES S. Mantarova, N. Dos. Dimitrov, I. Velcheva . . . . . . . .148 ÑàëíêéîàçéèÄíàà- ÉÖçéíàè-îÖçéíàèçà äéêÖãÄñàà, äéÉçàíàÇçà çÄêìòÖçàü à ÑàÄÉçéëíàäÄ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡, å ê‡È˜Â‚‡, à. í˙ÌÂ‚‡ . . . . . . . . . . . . . .153 DYSTROPHINOPATHIES- GENOTYPE- PHENOTYPE CORRELATIONS AND COGNITIVE DISORDERS IN PATIENTS WITH DUCHENNE AND BECKER MUSCULAR DYSTROPHIES T Chamova, M Raytcheva, I Tournev . . . . . . . . . . . . . .153 ÑàëíêéîàçéèÄíàà- ëöÇêÖåÖççé ãÖóÖçàÖ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡, à. í˙ÌÂ‚ . . . . . . . . . . . . . . . . . . . . . . . . .160 DYSTROPHINOPATHIES- MODERN TREATMENT OF DUCHENNE AND BECKER MUSCULAR DYSTROPHIES T. Chamova, I. Tournev . . . . . . . . . . . . . . . . . . . . . . . .160 ÄÇíéáéåçé-ÑéåàçÄçíçà îÄåàãçà èÄêñàÄãçà ÖèàãÖèíàóçà ëàçÑêéåàäãàçàóçÄ, ÖãÖäíêéîàáàéãéÉàóçÄ à ÉÖçÖíàóçÄ ïÄêÄäíÖêàëíàäÄ ë. ÜÂÎﬂÁÍÓ‚‡ . à. í˙ÌÂ‚ . . . . . . . . . . . . . . . . . . . . .164 AUTOSOMAL-DOMINANT FAMILIAL PARTIAL EPILEPSIES-CLINICAL, ELECTROPHYSIOLOGICAL AND GENETIC CHARACTERISTICS. S. Zhelyazkova . I. Tournev . . . . . . . . . . . . . . . . . . . . .164 êÄççà äéÉçàíàÇçà çÄêìòÖçàü èêà ÅéãÖëííÄ çÄ èÄêäàçëéç èÂÚÓ‚‡ å, ê‡È˜Â‚‡ å,, í‡ÈÍÓ‚ ã. . . . . . . . . . . . . .170 EARLY COGNITIVE CHANGES IN PARKINSON’S DISEASE Petrova M., Raycheva M., Traykov L. . . . . . . . . . . . . . .170 éêàÉàçÄãçà ëíÄíàà ORIGINAL PAPERS ÖîàäÄëçéëí à èéçéëàåéëí çÄ LEVETIRACETAM èêà ãÖóÖçàÖ çÄ èÄñàÖçíà ë ÖèàãÖèëàü á. á‡ı‡ËÂ‚, Ö. ÇËÚÂ‚‡ . . . . . . . . . . . . . . . . . . . . . . .174 EFFICACY AND TOLERABILITY OF LEVETIRACETAM IN PATIENTS WITH EPILEPSY Z. Zahariev, E. Viteva . . . . . . . . . . . . . . . . . . . . . . . . .174 çÄóÄãçàüí ÑÇàÉÄíÖãÖç ëàåèíéå ÇãàüÖ Çöêïì äéÉçàíàÇçàü ëèÄÑ èêà ãéçÉàíìÑàçÄãçé èêéëãÖÑÖçà èÄñàÖçíà ë èÄêäàçëéçéÇÄ ÅéãÖëí ü. ÜÂÎÂ‚, å. ê‡È˜Â‚‡, å. èÂÚÓ‚‡, ã. í‡ÈÍÓ‚ . . . .183 INITIAL MOTOR SYMPTOM INFLUENCES COGNITIVE DECLINE IN A LONGITUDINALLY FOLLOWED PATIENTS WITH PARKINSON'S DISEASE Y.Zhelev, M. Raycheva, M. Petrova, L. Traykov . . . . . .183 äéÉçàíàÇçà çÄêìòÖçàü èêà åàéíéçàóçà Ñàëíêéîàà íàè STEINERT à PROMM. ÉÖçéíàè-îÖçéíàè äéêÖãÄñàà í. ó‡ÏÓ‚‡, å ê‡È˜Â‚‡, à. í˙ÌÂ‚ . . . . . . . . . . . . . . .187 COGNITIVE DISORDERS IN MYOTONIC DYSTROPHIES TYPE STEINERT AND PROMM. GENOTYPE CORRELATIONS T. Chamova, M. Raycheva, I. Tarnev . . . . . . . . . . . . . .187 ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 143 é·ÁÓ ëíÄíàçàíÖ - ÑöãÉéëêéóçÄ ëíêÄíÖÉàü Ç èêÖÇÖçñàüíÄ çÄ åéáöóçéëöÑéÇÄíÄ ÅéãÖëí Ö‚„ÂÌËﬂ Ç‡ÒËÎÂ‚‡ äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, ìåÅÄã ◊ñ‡Ëˆ‡ âÓ‡ÌÌ‡”, ëÓÙËﬂ SUMMARY STATINS AND PREVENTION OF CEREBROVASULAR DISORDERS Ö. Vassileva Lowering of cholesterol concentration with statins reduces the risk of stroke in high-risk populations and in patients with non-cardioembolic stroke or transient ischaemic attacks. In patients with coronary artery disease, statin treatment has been associated with reduction of risk of stroke. Evidence is now available that statin therapy also reduce stroke risk in patients without coronary artery disease but at high cardiovascvular risk or with diabetes mellitus. Intensive statin therapy started within 6 months after a cerebrovascular event significantly reduces stroke risk. They also have essential biological effects on stability of atherosclerotic plaque, chronic inflammation, thrombogenesis, immune regulation Statin therapy is the most important advance in stroke prevention scince the introduction of aspirin and antihypertensive treatments. KEY WORDS: statins, cerebrovascular disorder, prevention. êÖáûåÖ èÓÌËÊ‡‚‡ÌÂÚÓ Ì‡ ÍÓÌˆÂÌÚ‡ˆËﬂÚ‡ Ì‡ ıÓÎÂÒÚÂÓÎ‡ ÓÚ ÒÚ‡ÚËÌÓ‚ÓÚÓ ÎÂ˜ÂÌËÂ Ì‡Ï‡Îﬂ‚‡ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ ÓÚ ËÒıÂÏË˜ÌË ËÌÒÛÎÚË ‚˙‚ ‚ËÒÓÍÓËÒÍÓ‚‡ ÔÓÔÛÎ‡ˆËﬂ Í‡ÍÚÓ Ë ÔË ·ÓÎÌË Ò ÌÂÍ‡‰ËÓÂÏ·ÓÎË˜ÂÌ ËÌÒÛÎÚ ËÎË ÔÂıÓ‰ÌË Ì‡Û¯ÂÌËﬂ Ì‡ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÒÚ‡ÚËÌËÚÂ Ì‡Ï‡Îﬂ‚‡Ú ËÒÍ‡ ÓÚ Ô˙‚Ë ËÒıÂÏË˜ÂÌ ËÌÒÛÎÚ ÔË ·ÓÎÌË Ò ÍÓÓÌ‡Ì‡ Ô‡ÚÓÎÓ„Ëﬂ, Í‡ÍÚÓ Ë ÔË Ú‡ÍË‚‡ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. àÌÚÂÌÁË‚ÌÓÚÓ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘Ó ÎÂ˜ÂÌËÂ, Á‡ÔÓ˜Ì‡ÚÓ ‰Ó 6 ÏÂÒÂˆ‡ ÒÎÂ‰ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ËÒıÂÏË˜ÌËﬂ ËÌÒÛÎÚ ÒË„ÌËÙËÍ‡ÌÚÌÓ Ì‡Ï‡Îﬂ‚‡ ËÒÍ‡ ÓÚ ÂˆË‰Ë‚. ëÚ‡ÚËÌËÚÂ ÔËÚÂÊ‡‚‡Ú ‚‡ÊÌË ·ËÓÎÓ„Ë˜ÌË ÂÙÂÍÚË ‚˙ıÛ ÒÚ‡·ËÎËÚÂÚ‡ Ì‡ ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡Ú‡ ÔÎ‡Í‡, ÔÓˆÂÒËÚÂ Ì‡ ËÏÛÌÌ‡ Â„ÛÎ‡ˆËﬂ, ıÓÌË˜ÌÓÚÓ ‚˙ÁÔ‡ÎÂÌËÂ, ÚÓÏ·Ó„ÂÌÂÁ‡Ú‡. ëÚ‡ÚËÌÓ‚ÓÚÓ ÎÂ˜ÂÌËÂ Â Ì‡È-‚‡ÊÌËﬂ Ì‡ÔÂ‰˙Í ‚ ÔÂ‚ÂÌˆËﬂÚ‡ Ì‡ ÏÓÁ˙˜ÌËﬂ ËÌÒÛÎÚ ÒÎÂ‰ ‚˙‚ÂÊ‰‡ÌÂÚÓ Ì‡ ‡ÌÚË‡„Â„‡ÌÚÌ‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ë ‡ÌÚËıËÔÂÚÂÌÁË‚ÌÓÚÓ ÎÂ˜ÂÌËÂ. äãûóéÇà Ñìåà: ÒÚ‡ÚËÌË, ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ, ÔÂ‚ÂÌˆËﬂ. àÒıÂÏË˜ÌËÚÂ ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ò‡ Ì‡È-˜ÂÒÚËÚÂ ÓÒÚË Ì‡Û¯ÂÌËﬂ Ì‡ ÏÓÁ˙˜ÌÓÚÓ Í˙‚ÓÓ·‡˘ÂÌËÂ - 80% ÓÚ ‚ÒË˜ÍË ËÌÒÛÎÚË. ÄÚÂÓÒÍÎÂÓÁ‡Ú‡ Ë ‡ÚÂÓÚÓÏ·ÓÁ‡Ú‡ Ò‡ ÓÒÌÓ‚ÌËÚÂ Ô‡ÚÓÎÓ„Ë˜ÌË ÔÓˆÂÒË, ÔË˜ËÌﬂ‚‡˘Ë ËÒıÂÏË˜ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (àåà). ÄÚÂÓÒÍÎÂÓÁ‡Ú‡ ‚Ó‰Ë ‰Ó ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ ÔÓ ‰‚‡ ÓÒÌÓ‚ÌË ÏÂı‡ÌËÁÏ‡: ˜ÂÁ Ó·‡ÁÛ‚‡ÌÂ Ì‡ ÔÎ‡ÍË ÔÓ ‡ÓÚÌ‡Ú‡ ‰˙„‡ Ë ÒÚÂÌÓÁË‡ÌÂ Ì‡ ÂÍÒÚ‡Í‡ÌË‡ÎÌËÚÂ ÏÓÁ˙˜ÌË ‡ÚÂËË Ë ˜ÂÁ ‡Á‚ËÚËÂ Ì‡ ËÒıÂÏË˜Ì‡ ·ÓÎÂÒÚ Ì‡ Ò˙ˆÂÚÓ Ò ÔÓÒÎÂ‰‚‡˘ ÏËÓÍ‡‰ÂÌ ËÌÙ‡ÍÚ Ë ÔÂ‰Ò˙‰ÌÓ Ï˙Ê‰ÂÌÂ. éÍÓÎÓ 30% ÓÚ ÏÓÁ˙˜ÌËÚÂ ËÌÙ‡ÍÚË Ò‡ ÂÁÛÎÚ‡Ú ÓÚ ‡ÚÂÓÒÍÎÂÓÁ‡ Ë ‡ÚÂÓÚÓÏ·ÓÁ‡ Ì‡ ‡ÓÚÌ‡Ú‡ ‰˙„‡ Ë Ì‡ ÂÍÒÚ‡Í‡ÌË‡ÎÌËÚÂ ÏÓÁ˙˜ÌË ‡ÚÂËË. ëíÄíàçà à àëïÖåàóÖç àçëìãí ÑËÂÍÚÌ‡Ú‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ÒÂÛÏÌÓÚÓ ÌË‚Ó Ì‡ ıÓÎÂÒÚÂÓÎ‡ Ë ÏÓÁ˙˜ÌËﬂ ËÌÒÛÎÚ Â ·ËÎ‡ Ó·ÂÍÚ Ì‡ ÔÓÛ˜‚‡ÌÂ ÓÚ ÏÌÓÊÂÒÚ‚Ó ÏÂÚ‡‡Ì‡ÎËÁË, Ì‡Ô‡‚ÂÌË ÔÂÁ ÔÓÒÎÂ‰ÌËÚÂ ‰ÂÒÂÚ „Ó‰ËÌË. êÂÁÛÎÚ‡ÚËÚÂ ÓÚ Ô˙‚ËÚÂ ÓÚ Úﬂı ÌÂ ‰ÓÍ‡Á‚‡Ú ÍÓÂÎ‡ˆËﬂ ÏÂÊ‰Û ÒÂÛÏÌÓÚÓ ÌË‚Ó Ì‡ ıÓÎÂÒÚÂÓÎ‡ Ë ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ (1). èË˜ËÌËÚÂ Á‡ ÚÓ‚‡ Ò‡ ‡ÁÎË˜ÌË. í‡Í‡ Ì‡ÔËÏÂ ÌﬂÍÓË ÓÚ ÏÂÚ‡‡Ì‡ÎËÁËÚÂ, ‚˙ÔÂÍË ˜Â Ó·ı‚‡˘‡Ú ÏÌÓ„Ó·ÓÈÌË ÔÓÛ˜‚‡ÌËﬂ Ò ÏÌÓ„Ó „ÓÎﬂÏ ·ÓÈ ·ÓÎÌË, ËÁÒÎÂ‰‚‡Ú ‚˙ÁÍ‡Ú‡ Ì‡ ıÓÎÂÒÚÂÓÎ‡ Ò ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ò‡ÏÓ Ì‡ Ù‡Ú‡ÎÌË ÏÓÁ˙˜ÌË ËÌÒÛÎÚË (1,2). éÚÌÓÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ Ù‡Ú‡ÎÌËÚÂ ËÌÒÛÎÚË Ó·‡˜Â Â Ò‡ÏÓ 20% ÓÚ ‚ÒË˜ÍË ËÌÒÛÎÚË. éÒ‚ÂÌ ÚÓ‚‡ ‚ ÚÂÁË ‡Ì‡ÎËÁË ÒÂ ‚ÍÎ˛˜‚‡Ú ‚ÒË˜ÍË ÓÒÚË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë ËÌˆË‰ÂÌÚË, ‚ÍÎ˛˜ËÚÂÎÌÓ Ë ÏÓÁ˙˜ÌËÚÂ Í˙‚ÓËÁÎË‚Ë. çÂ ÒÂ ‚ÁÂÏ‡ ÔÓ‰ ‚ÌËÏ‡ÌËÂ Ì‡ÎË˜ËÂÚÓ Ì‡ ıÂÚÂÓ„ÂÌÌÓÒÚ Ì‡ àåà ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÂÚËÓÔ‡ÚÓ„ÂÌÂÁ‡Ú‡. á‡ ‰‡ ÔÓÍ‡ÊÂ ÓÎﬂÚ‡ Ì‡ ıÓÎÂÒÚÂÓÎ‡ ‚ Ô‡ÚÓÙËÁËÓÎÓ„ËﬂÚ‡ Ì‡ ÏÓÁ˙˜ÌËﬂ ËÌÒÛÎÚ, ÔÂÁ 2009 „Ó‰ËÌ‡ Amarenco Ë Labreuche (3) Ô‡‚ﬂÚ ÏÂÚ‡‡Ì‡ÎËÁ Ì‡ 24 ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËﬂ, ÔÛ·ÎËÍÛ‚‡ÌË ÏÂÊ‰Û 1994 Ë 2009 „. Ò ÓÒÌÓ‚ÌÓ ËÁÔÓÎÁ‚‡ÌËÚÂ ÒÚ‡ÚËÌË. ä‡Ò‡Â ÒÂ Á‡ ÓË„ËÌ‡ÎÌË ÔÓÛ˜‚‡ÌËﬂ, ‚ÒﬂÍÓ ÓÚ ÍÓËÚÓ Ò ÏËÌËÏÛÏ 1000 Ô‡ˆËÂÌÚË. åÂÚ‡‡Ì‡ÎËÁ˙Ú ËÁÒÎÂ‰‚‡ ÂÙÂÍÚ‡ Ì‡ ÒÚ‡ÚËÌËÚÂ Á‡ ÔÂ‚ÂÌˆËﬂ Ì‡ ËÌÒÛÎÚ Ó·˘Ó ÔË 165 000 ‚ËÒÓÍÓËÒÍÓ‚Ë ÎËˆ‡. èË Ô‡ˆËÂÌÚËÚÂ, ‡Ì‰ÓÏËÁË‡ÌË ‰‡ ÔÓÎÛ˜‡‚‡Ú ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËﬂ, ÏÂÚ‡-‡Ì‡ÎËÁ˙Ú ‰ÂÏÓÌÒÚË‡ Â‰ÛÍˆËﬂ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ‚ÒË˜ÍË ËÌÒÛÎÚË Ò 18% (ÙË„.1). ìÒÚ‡ÌÓ‚ﬂ‚‡ ÒÂ Ò˙˘Ó, ˜Â ‚ÒÂÍË 1 mmol/l (39 mg/dL) ÔÓÌËÊ‡‚‡ÌÂ Ì‡ ÍÓÌˆÂÌÚ‡ˆËﬂÚ‡ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡ ÒÂ ‡‚Ìﬂ‚‡ Ì‡ 21% Â‰ÛÍˆËﬂ Ì‡ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ Á‡ ËÌÒÛÎÚ (95% CI 6 3–33 5, p=0 009). Ç˙‚ ‚ÚÓË˜Ì‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ ÌÂÍ‡‰ËÓÂÏ·ÓÎË˜ÌËﬂ ËÌÒÛÎÚ, ËÌÚÂÌÁË‚Ì‡Ú‡ Â‰ÛÍˆËﬂ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡ ÔÓÒÂ‰ÒÚ‚ÓÏ ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËﬂ Ò˙˘Ó ‚Ó‰Ë ‰Ó ÒË„ÌËÙËÍ‡ÌÚÌ‡ Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ ÂˆË‰Ë‚ (ÓÚÌÓÒËÚÂÎÂÌ ËÒÍ 0 84, 0 71–0 99, p=0 03) Ë Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë ËÌˆË‰ÂÌÚË (0 80, 0 69–0 92, p=0 002). Ç˙ÔÂÍË ˜Â Â ÚÛ‰ÌÓ ‰‡ ·˙‰Â ÛÒÚ‡ÌÓ‚ÂÌ‡ ‰ËÂÍÚÌ‡Ú‡ îË„.1 äÓÂÎ‡ˆËﬂ ÏÂÊ‰Û Â‰ÛÍˆËﬂ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ Ë ˜ÂÒÚÓÚ‡ Ì‡ ÏÓÁ˙˜ÌË ËÌÒÛÎÚË ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ‚˙ÁÍ‡ ÏÂÊ‰Û ıËÔÂıÓÎÂÒÚÂÓÎÂÏËﬂÚ‡ Ë àåà, ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÔÓ‚Â˜ÂÚÓ ÔÓÛ˜‚‡ÌËﬂ ÔÓÍ‡Á‚‡Ú, ˜Â ÔÓÌËÊ‡‚‡ÌÂÚÓ Ì‡ ıÓÎÂÒÚÂÓÎ‡ ÔÓÌËÊ‡‚‡ Ë ËÒÍ‡ ÓÚ ÏÓÁ˙˜ÌË ËÌÒÛÎÚË ÔË ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË. ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà èêà èÄñàÖçíà ë äéêéçÄêçÄ èÄíéãéÉàü êÓÎﬂÚ‡ Ì‡ ÒÚ‡ÚËÌËÚÂ ‚ ÔÂ‚ÂÌˆËﬂÚ‡ Ì‡ ÏËÓÍ‡‰ÌËﬂ ËÌÙ‡ÍÚ ÔË ·ÓÎÌË Ò ÍÓÓÌ‡Ì‡ Ô‡ÚÓÎÓ„Ëﬂ Â ÓÚ‰‡‚Ì‡ ÛÒÚ‡ÌÓ‚ÂÌ‡ Ë ·ÂÁÒÔÓÌ‡, ÍÓÂÚÓ Ó·ÛÒÎ‡‚ﬂ „ÓÎﬂÏÓÚÓ ËÏ ÁÌ‡˜ÂÌËÂ Á‡ ÔÓÙËÎ‡ÍÚËÍ‡Ú‡ Ì‡ ÚÂÁË Á‡·ÓÎﬂ‚‡ÌËﬂ. Ç ÏÌÓ„Ó ÓÚ ÔÓÛ˜‚‡ÌËﬂÚ‡, ËÁÒÎÂ‰‚‡˘Ë ÁÌ‡˜ÂÌËÂÚÓ Ì‡ ÒÚ‡ÚËÌËÚÂ Á‡ ÔÂ‚ÂÌˆËﬂ Ì‡ ÏËÓÍ‡‰ÂÌ ËÌÙ‡ÍÚ ÔË ·ÓÎÌË Ò ËÁ‚ÂÒÚÌ‡ ÍÓÓÌ‡Ì‡ ·ÓÎÂÒÚ, ÒÂ ÓÚ˜ËÚ‡ ÚﬂıÌÓÚÓ ÁÌ‡˜ÂÌËÂ Ë ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ Ô˙‚Ë˜Ì‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ àåà. ìÒÚ‡ÌÓ‚ÂÌÓ ·Â, ˜Â ÚÓ‚‡ ÎÂ˜ÂÌËÂ ‰ÓÔËÌ‡Òﬂ ÓÒ‚ÂÌ Á‡ Ì‡Ï‡Îﬂ‚‡ÌÂ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÏËÓÍ‡‰ÌËﬂ ËÌÙ‡ÍÚ, Ó˘Â Ë Á‡ ÔÓÌËÊ‡‚‡ÌÂ Á‡·ÓÎÂ‚‡ÂÏÓÒÚÚ‡ ÓÚ ËÌÒÛÎÚË ‚ ÚÓÁË ÍÓÌÚËÌ„ÂÌÚ ·ÓÎÌË. í‡Í‡ Ì‡ÔËÏÂ, ‚ ÔÓÛ˜‚‡ÌÂÚÓ Heart Protecton Study (HPS) Ò‡ ‡Ì‰ÓÏËÁË‡ÌË Ó·˘Ó 20 536 ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË, 65% (13 386) ÓÚ ÍÓËÚÓ Ò ‰Ë‡„ÌÓÒÚËˆË‡Ì‡ ÍÓÓÌ‡Ì‡ Ô‡ÚÓÎÓ„Ëﬂ. äÓ„‡ÚÓ ÒÎÂ‰ 4,8 „Ó‰ËÌË ÒÂ‰ÌÓ ÔÓÒÎÂ‰ﬂ‚‡ÌÂ Â Ì‡Ô‡‚ÂÌ ‡Ì‡ÎËÁ Ì‡ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÒÚ‡ÚËÌÓ‚‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡, ‚ ÔÓÒÎÂ‰Ì‡Ú‡ „ÛÔ‡ Â ÛÒÚ‡ÌÓ‚ÂÌ‡ 25% Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ Ô˙‚Ë àåà (4). Ç ‰Û„Ó ÔÓ‰Ó·ÌÓ ÔÓÛ˜‚‡ÌÂ – Scandinavian Simvastatin Survival Study (4S) Ò‡ ‚ÍÎ˛˜ÂÌË 4444 ·ÓÎÌË Ò ËÒıÂÏË˜Ì‡ ·ÓÎÂÒÚ Ì‡ Ò˙ˆÂÚÓ (àÅë), ÍÓËÚÓ ÌÂ Ò‡ ÔÂÊË‚ﬂÎË àåà. á‡ ÔÂËÓ‰ ÓÚ 5,4 „Ó‰ËÌË ÒÂ‰ÌÓÚÓ ÌË‚Ó Ì‡ LDL-ıÓÎÂÒÚÂÓÎ ‚ „ÛÔ‡Ú‡, ÔÓÎÛ˜‡‚‡Î‡ ÒÚ‡ÚËÌÓ‚Ó ÎÂ˜ÂÌËÂ, Â Ì‡Ï‡ÎÂÌÓ Ò 35% ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ËÁıÓ‰ÌËÚÂ ÒÚÓÈÌÓÒÚË Ë Â ÓÚ˜ÂÚÂÌ‡ 30% Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ Ù‡Ú‡ÎÌË Ë ÌÂÙ‡Ú‡ÎÌË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë ËÌˆË‰ÂÌÚË (5). ëË„ÌËÙËÍ‡ÌÚÌÓÚÓ Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ ËÒÍ‡ ÓÚ àåà ÔË ·ÓÎÌË Ò àÅë ‚ ÂÁÛÎÚ‡Ú Ì‡ ÒÚ‡ÚËÌÓ‚‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ò˙˘Ó Â ÔÓÚ‚˙‰ÂÌÓ Ë ÓÚ ‰‚Â ‰Û„Ë ÔÓÛ˜‚‡ÌËﬂ – Cholesterol and Recurrent Events (CARE) Ë Long-term Intervention with Pravastatin in Ischemic Diseasese (LIPID) (6, 7). íÂ Ò‡‚Ìﬂ‚‡Ú 40 mg Ô‡‚‡ÒÚ‡ÚËÌ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÔÎ‡ˆÂ·Ó ÔË Ô‡ˆËÂÌÚË Ò àÅë Ë ÓÚ˜ËÚ‡Ú 22% Â‰ÛÍˆËﬂ Ì‡ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ ÔË ·ÓÎÌËÚÂ Ò˙Ò ÒÚ‡ÚËÌÓ‚Ó ÎÂ˜ÂÌËÂ. èÓÛ˜‚‡ÌÂÚÓ Treating to New Targets (íNí) (8) ËÁÒÎÂ‰‚‡ ÂÙÂÍÚË‚ÌÓÒÚÚ‡ Ì‡ ËÌÚÂÌÁË‚Ì‡Ú‡ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘‡ ÚÂ‡ÔËﬂ, ÍÓﬂÚÓ ‚Ó‰Ë ‰Ó Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ LDL- ıÓÎÂÒÚÂÓÎ‡ ÔÓ‰ 2,6 mmol/L (100 mg/dL). á‡ Ú‡ÁË ˆÂÎ 10 001 Ô‡ˆËÂÌÚË Ò ‰ÓÍÛÏÂÌÚË‡Ì‡ àÅë Ò‡ ÔÓÎÛ˜‡‚‡ÎË ÒÚ‡Ì‰‡ÚÌÓ ÎÂ˜ÂÌËÂ (Ò 10 mg ‡ÚÓ‚‡ÒÚ‡ÚËÌ) ËÎË ËÌÚÂÌÁË‚ÌÓ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘Ó ÎÂ˜ÂÌËÂ (Ò 80 mg ‡ÚÓ‚‡ÒÚ‡ÚËÌ). èË Ò‡‚ÌÂÌËÂ Ì‡ ‰‚ÂÚÂ „ÛÔË Â ÓÚ˜ÂÚÂÌ‡ 23% Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë ËÌˆË‰ÂÌÚË Ë 25% Ì‡ ËÒÍ‡ ÓÚ Ù‡Ú‡ÎÌË Ë ÌÂÙ‡Ú‡ÎÌË ËÌÒÛÎÚË ‚ ÔÓÎÁ‡ Ì‡ ËÌÚÂÌÁË‚ÌÓÚÓ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘Ó ÎÂ˜ÂÌËÂ. Ñ‡ÌÌËÚÂ ÓÚ Ó·˘Ó 26 ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËﬂ Ò‡ Ó·Ó·˘ÂÌË ÓÚ Amarenco et al. ‚ ÏÂÚ‡‡Ì‡ÎËÁ, ÍÓÈÚÓ ‚ÍÎ˛˜‚‡ Ó·˘Ó 90 000 Ô‡ˆËÂÌÚ‡ Ò àÅë Ë ‰ÓÍ‡Á‚‡, ˜Â ÒÚ‡ÚËÌËÚÂ Ì‡Ï‡Îﬂ‚‡Ú ËÒÍ‡ ÓÚ Ô˙‚Ë àåà ÔË ·ÓÎÌË Ò àÅë Ò 21% ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÔÎ‡ˆÂ·Ó (9). Ç Á‡ÍÎ˛˜ÂÌËÂ - ÓÎﬂÚ‡ Ì‡ ÒÚ‡ÚËÌËÚÂ Á‡ Ô˙‚Ë˜Ì‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ àåà ÔË ·ÓÎÌË Ò ÍÓÓÌ‡Ì‡ Ô‡ÚÓÎÓ„Ëﬂ Â ·ÂÁÒÔÓÌ‡. ÇÒË˜ÍË „ÓÎÂÏË ÔÎ‡ˆÂ·Ó-ÍÓÌÚÓÎË‡ÌË, ‡Ì‰ÓÏËÁË‡ÌË ÔÓÛ˜‚‡ÌËﬂ (4S, CARE, LIPID, HPS, TNT) (4-8) ‰ÓÍ‡Á‚‡Ú, ˜Â ÎÂ˜ÂÌËÂÚÓ Ò˙Ò ÒÚ‡ÚËÌË ÔÓÌËÊ‡‚‡ ËÒÍ‡ ÓÚ Ô˙‚Ë àåà ÔË Ô‡ˆËÂÌÚË Ò ÍÓÓÌ‡Ì‡ ·ÓÎÂÒÚ ÒÂ‰ÌÓ Ò 144 25%-35%. ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà èêà èÄñàÖçíà ëöë áÄïÄêÖç ÑàÄÅÖí êÓÎﬂÚ‡ Ì‡ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘‡Ú‡ ÚÂ‡ÔËﬂ Á‡ Ô˙‚Ë˜Ì‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ Ò˙‰Â˜ÌÓ-Ò˙‰Ó‚Ë Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ ÔË ·ÓÎÌË Ò ‚ÚÓË ÚËÔ Á‡ı‡ÂÌ ‰Ë‡·ÂÚ Â ÛÒÚ‡ÌÓ‚ÂÌ‡ Ó˘Â ÔÂÁ 2004 „Ó‰. èÓÛ˜‚‡ÌÂÚÓ Collaborative Atorvastatin Diabetes Study (CARDS) (10) ‡Ì‰ÓÏËÁË‡ 2838 Ô‡ˆËÂÌÚ‡ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ Ë ÔÓÌÂ Ó˘Â Â‰ËÌ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ åëÅ, ÌÓ ·ÂÁ ÔÂÍ‡‡ÌË ÓÒÚË Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë ËÌˆË‰ÂÌÚË. éÒ˙˘ÂÒÚ‚ÂÌÓ Â ÚﬂıÌÓÚÓ ÔÓÒÎÂ‰ﬂ‚‡ÌÂ ‰Ó ÏÓÏÂÌÚ‡ Ì‡ ‚˙ÁÌËÍ‚‡ÌÂ Ì‡ Ú‡Í˙‚ ËÌˆË‰ÂÌÚ. èÓÛ˜‚‡ÌÂÚÓ Ó·‡˜Â Â ÔÂÍ‡ÚÂÌÓ ‰‚Â „Ó‰ËÌË ÔÓ-‡ÌÓ ÓÚ ÔÂ‰‚Ë‰ÂÌËﬂ ÒÓÍ ÔÓ‡‰Ë ‰ÓÒÚË„‡ÌÂÚÓ Ì‡ Û·Â‰ËÚÂÎÌË ÂÁÛÎÚ‡ÚË. ÑÓÍ‡Á‡Ì‡ Â 48% Â‰ÛÍˆËﬂ ‚ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ ÓÚ àåà (95% CI,11% to 69%; p=0,016), 31% Â‰ÛÍˆËﬂ - Á‡ ÓÒÚË ÍÓÌÓ‡ÌË ËÌˆË‰ÂÌÚË Ë 31% - Á‡ ÍÓÓÌ‡Ì‡ Â‚‡ÒÍÛÎ‡ËÁ‡ˆËﬂ. èÓ‡‰Ë ÌÂÒ˙ÏÌÂÌËÚÂ ÔÓÎÁË Á‡ Ô‡ˆËÂÌÚËÚÂ Ò˙Ò Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, ÄÏÂËÍ‡ÌÒÍ‡Ú‡ ÄÒÓˆË‡ˆËﬂ ÔÓ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë, Í‡ÍÚÓ Ë Ú‡ÁË ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ ÔÂÁ 2006 „Ó‰ ÔËÂÏ‡Ú, ˜Â ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰Ë‡·ÂÚ, ÓÒÓ·ÂÌÓ ÔË Ì‡ÎË˜ËÂ Ë Ì‡ ‰Û„Ë ËÒÍÓ‚Ë Ù‡ÍÚÓË, Â ÔÂÔÓ˙˜ËÚÂÎÌÓ ‰Ó·‡‚ﬂÌÂ Ì‡ ÒÚ‡ÚËÌË ‚ ‰ÓÔ˙ÎÌÂÌËÂ Ì‡ ÒÚËÍÚÌËﬂ ÍÓÌÚÓÎ Ì‡ Í˙‚ÌÓÚÓ Ì‡Îﬂ„‡ÌÂ, Ò ˆÂÎ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ àåà (11). èÓÙËÎ‡ÍÚËÍ‡Ú‡ Ò˙Ò ÒÚ‡ÚËÌË Ì‡Ï‡Îﬂ‚‡ Ò Â‰Ì‡ ˜ÂÚ‚˙Ú ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÏËÓÍ‡‰ÌËÚÂ ËÌÙ‡ÍÚË, ËÒıÂÏË˜ÌËÚÂ ËÌÒÛÎÚË Ë Â‚‡ÒÍÛÎ‡ËÁ‡ˆËﬂÚ‡ ÔË ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË. ëíÄíàçà à èöêÇàóçÄ èêéîàãÄäíàäÄ çÄ àåà èêà èÄñàÖçíà ÅÖá ëöÑéÇÄ èÄíéãéÉàü éÒÓ·ÂÌ ËÌÚÂÂÒ ÔÂ‰ÒÚ‡‚Îﬂ‚‡ ‚˙ÔÓÒ˙Ú, ËÏ‡Ú ÎË ÏﬂÒÚÓ ÒÚ‡ÚËÌËÚÂ ‚ Ô˙‚Ë˜Ì‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ àåà ÔË Ô‡ˆËÂÌÚË ·ÂÁ ‰Ë‡„ÌÓÒÚËˆË‡Ì‡ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëﬂ ËÎË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. è˙‚ÓÚÓ ÔÓÛ˜‚‡ÌÂ, ÍÓÂÚÓ ÔÓÍ‡Á‚‡ ÒË„ÌËÙËÍ‡ÌÚÌÓ Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ àåà ÔË Ô‡ˆËÂÌÚËÚÂ ·ÂÁ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëﬂ Â Anglo-Scandinavian Cardiac Outcome Trial – Lipid Lowering Arm (ASCOT-LLA) (12). è‡ˆËÂÌÚËÚÂ, Ó·ÂÍÚ Ì‡ ËÁÒÎÂ‰‚‡ÌÂ, Ò‡ ‡ÒËÏÔÚÓÏ‡ÚË˜ÌË, ÌÓ ‚ËÒÓÍÓËÒÍÓ‚Ë ·ÓÎÌË - Ò ‡ÚÂË‡ÎÌ‡ ıËÔÂÚÓÌËﬂ Ë ÔÓÌÂ Ó˘Â ÚË ËÒÍÓ‚Ë Ù‡ÍÚÓ‡ Á‡ åëÅ. êÂÁÛÎÚ‡ÚËÚÂ ÔÓÍ‡Á‚‡Ú 27% Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ Ô˙‚Ë àåà ‚ ÂÁÛÎÚ‡Ú Ì‡ ÎÂ˜ÂÌËÂ Ò˙Ò ÒÚ‡ÚËÌË ÔË ÚÂÁË Ô‡ˆËÂÌÚË. éÚ ÓÒÓ·ÂÌÓ ÁÌ‡˜ÂÌËÂ Á‡ Ó·Ó„‡Úﬂ‚‡ÌÂ ÁÌ‡ÌËﬂÚ‡ Á‡ ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡Ú‡ Ò˙‰Ó‚‡ Ô‡ÚÓÎÓ„Ëﬂ Ë ÔÂ‚ÂÌˆËﬂÚ‡ Ì‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚‡Ú‡ ·ÓÎÂÒÚ Â Ì‡ÒÍÓÓ ÔÛ·ÎËÍÛ‚‡ÌÓÚÓ ÔÓÛ˜‚‡ÌÂ JUPITER (The Justification for the Use of Statins in Primary Prevention: an Intervention Trial Evaluating Rosuvastatin) (13). íÓ ‚ÍÎ˛˜‚‡ 89 890 Ô‡ˆËÂÌÚ‡, ÍÓËÚÓ ÌﬂÏ‡Ú ËÌ‰ËÍ‡ˆËË Á‡ ÎÂ˜ÂÌËÂ Ò˙Ò ÒÚ‡ÚËÌË. JUPITER Â ‡Ì‰ÓÏËÁË‡ÌÓ, ‰‚ÓÈÌÓ-ÒÎﬂÔÓ, ÔÎ‡ˆÂ·Ó-ÍÓÌÚÓÎË‡ÌÓ, ÏÌÓ„ÓˆÂÌÚÓ‚Ó ÔÓÛ˜‚‡ÌÂ, ËÁÒÎÂ‰‚‡˘Ó ÓÎﬂÚ‡ Ì‡ ÚÂ‡ÔËﬂÚ‡ Ò˙Ò ÒÚ‡ÚËÌË ÔË ÎËˆ‡ Ò ÌÓÏ‡ÎÌË ÌË‚‡ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ, ÌÓ Ò ÔÓ‚Ë¯ÂÌ Í‡‰ËÓ‚‡ÒÍÛÎ‡ÂÌ ËÒÍ, Ë‰ÂÌÚËÙËˆË‡Ì ÓÚ ÒÚÓÈÌÓÒÚË Ì‡ ‚ËÒÓÍÓ-ÒÂÌÁËÚË‚ÌËﬂ C-Â‡ÍÚË‚ÂÌ ÔÓÚÂËÌ (hsCRP _>2 mg/l). è˙‚Ë˜ÂÌ ÔÓÒÎÂ‰ﬂ‚‡Ì ÔÓÍ‡Á‡ÚÂÎ Â ‚ÂÏÂÚÓ ‰Ó ÔÓﬂ‚‡ Ì‡ Ô˙‚Ó Ò˙·ËÚËÂ (ËÌÙ‡ÍÚ Ì‡ ÏËÓÍ‡‰‡, àåà, ıÓÒÔËÚ‡ÎËÁ‡ˆËﬂ ÔÓ‡‰Ë ÌÂÒÚ‡·ËÎÌ‡ ÒÚÂÌÓÍ‡‰Ëﬂ, ‡ÚÂË‡ÎÌ‡ Â‚‡ÒÍÛÎ‡ËÁ‡ˆËﬂ ËÎË Í‡‰ËÓ‚‡ÒÍÛÎ‡Ì‡ ÒÏ˙Ú). èÂÁ ÔÂËÓ‰‡ Ì‡ ÔÓÒÎÂ‰ﬂ‚‡ÌÂ (ÒÂ‰ÌÓ 1,9 „Ó‰ËÌË, Ï‡ÍÒËÏÛÏ 5 „Ó‰ËÌË), rosuvastatin ‚Ó‰Ë ‰Ó ÒË„ÌËÙËÍ‡ÌÚÌ‡ Â‰ÛÍˆËﬂ Ì‡ ÒÂËÓÁÌËÚÂ Í‡‰ËÓ‚‡ÒÍÛÎ‡ÌË ËÌˆË‰ÂÌÚË (142 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ÒÔﬂÏÓ 251 ‚ „ÛÔ‡Ú‡ Ì‡ ÔÎ‡ˆÂ·Ó), ÌÂÁ‡‚ËÒËÏÓ ÓÚ Ù‡ÍÚ‡, ˜Â ÔÓ˜ÚË ‚ÒË˜ÍË Û˜‡ÒÚÌËˆË ËÏ‡Ú Ì‡˜‡ÎÌË ÌË‚‡ Ì‡ ÎËÔË‰ËÚÂ, ÌÂËÁËÒÍ‚‡˘Ë ÚÂ‡ÔËﬂ Ò˙„Î‡ÒÌÓ Ì‡ÒÚÓﬂ˘ËÚÂ ˙ÍÓ‚Ó‰ÒÚ‚‡ Á‡ ÔÂ‚ÂÌˆËﬂ (í‡·Î.1). ÅÓﬂÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ ÓÚ ËÁÒÎÂ‰‚‡ÌËﬂ ÍÓÌÚËÌ„ÂÌÚ, ÍÓËÚÓ Â ÌÂÓ·ıÓ‰ËÏÓ ‰‡ ÒÂ ÎÂÍÛ‚‡Ú Ò rosuvastatin ‚ ÔÓ‰˙ÎÊÂÌËÂ Ì‡ 2 „Ó‰ËÌË, Á‡ ‰‡ ÒÂ ÔÂ‰ÓÚ‚‡ÚË ÔÓﬂ‚‡Ú‡ Ì‡ Â‰ËÌ í‡·Î.1 êÂÁÛÎÚ‡ÚË Ò˙‰Ó‚ ËÌˆË‰ÂÌÚ, Â 95. á‡ ÔÂÚ„Ó‰Ë¯ÂÌ ÔÂËÓ‰ Â ÌÂÓ·ıÓ‰ËÏÓ ‰‡ ÒÂ ÎÂÍÛ‚‡Ú 25 Ô‡ˆËÂÌÚË, Á‡ ‰‡ ÒÂ ÔÂ‰ÓÚ‚‡ÚË ÔÓﬂ‚‡Ú‡ Ì‡ Â‰ÌÓ Ò˙·ËÚËÂ. éÚ ÓÒÓ·ÂÌÓ ÁÌ‡˜ÂÌËÂ Â Ù‡ÍÚ˙Ú, ˜Â Ó·˘ËﬂÚ ·ÓÈ Ì‡ Ò˙Ó·˘ÂÌË ÒÂËÓÁÌË ÌÂÊÂÎ‡ÌË Ò˙·ËÚËﬂ Â ÒıÓ‰ÂÌ ‚ „ÛÔ‡Ú‡ Ì‡ rosuvastatin Ë Ú‡ÁË Ò ÔÎ‡ˆÂ·Ó (Ò˙ÓÚ‚ÂÚÌÓ 1352 Ë 1377; P = 0.60). èÂ‰Ë¯ÌË ÔÓÛ˜‚‡ÌËﬂ Ò˙Ò ÒÚ‡ÚËÌË (ÔÓ‚Â˜ÂÚÓ ÓÚ ÍÓËÚÓ ËÁÔÓÎÁ‚‡Ú ÌË‚ÓÚÓ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ Í‡ÚÓ ÍËÚÂËÈ Á‡ ‚ÍÎ˛˜‚‡ÌÂ) Ò˙Ó·˘‡‚‡Ú Á‡ 20% Â‰ÛÍˆËﬂ Ì‡ Ò˙‰Ó‚Ëﬂ ËÒÍ Á‡ ‚ÒÂÍË 1 mmol/l ‡·ÒÓÎ˛ÚÌ‡ Â‰ÛÍˆËﬂ Ì‡ ÌË‚ÓÚÓ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡, ÍÓÂÚÓ ÔÂ‰ÔÓÎ‡„‡ ÔÓÔÓˆËÓÌ‡ÎÌ‡ Â‰ÛÍˆËﬂ Ì‡ ·Óﬂ Ì‡ Ò˙·ËÚËﬂÚ‡ ‚ JUPITER ÓÚ ÓÍÓÎÓ 25%. é·‡˜Â, Â‰ÛÍˆËﬂÚ‡ Ì‡ ËÒÍ‡ ‚ ÚÓ‚‡ ÔÓÛ˜‚‡ÌÂ, ÔË ÍÓÂÚÓ ÍËÚÂËÈ Á‡ ‚ÍÎ˛˜‚‡ÌÂ Â ÔÓ‚Ë¯ÂÌÓÚÓ ÌË‚Ó Ì‡ ‚ËÒÓÍÓ-˜Û‚ÒÚ‚ËÚÂÎÌËﬂ ë-Â‡ÍÚË‚ÂÌ ÔÓÚÂËÌ, ‡ ÌÂ ÔÓ‚Ë¯ÂÌÓÚÓ ÌË‚Ó Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡, Â ·ÎËÁÓ ‰‚‡ Ô˙ÚË ÔÓ-„ÓÎﬂÏ‡ Ë ÔÓÍ‡Á‚‡ ÔÓ-‚ËÒÓÍ‡ ÂÎ‡ÚË‚Ì‡ ÔÓÎÁ‡, ÓÚÍÓÎÍÓÚÓ Ú‡ÁË, ÛÒÚ‡ÌÓ‚ÂÌ‡ ‚ ÔÓ‚Â˜ÂÚÓ ÔÂ‰Ë¯ÌË ÔÓÛ˜‚‡ÌËﬂ Ò˙Ò ÒÚ‡ÚËÌË. Ç Ó·Ó˘ÂÌËÂ, ÎÂ˜ÂÌËÂÚÓ Ò rosuvastatin 20 mg ‚Ó‰Ë ‰Ó 44% Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ ÒÂËÓÁÌË Í‡‰ËÓ‚‡ÒÍÛÎ‡ÌË Ò˙·ËÚËﬂ (<0.00001), 54% Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ Ù‡Ú‡ÎÂÌ Ë ÌÂÙ‡Ú‡ÎÂÌ ËÌÙ‡ÍÚ Ì‡ ÏËÓÍ‡‰‡, 47% Â‰ÛÍˆËﬂ Ì‡ ‡ÚÂË‡ÎÌ‡Ú‡ Â‚‡ÒÍÛÎ‡ËÁ‡ˆËﬂ ËÎË ıÓÒÔËÚ‡ÎËÁ‡ˆËﬂ ÔÓ‡‰Ë ÌÂÒÚ‡·ËÎÌ‡ ÒÚÂÌÓÍ‡‰Ëﬂ, 47% Â‰ÛÍˆËﬂ Á‡ ÍÓÏ·ËÌË‡ÌËﬂ ÔÓÍ‡Á‡ÚÂÎ, ‚ÍÎ˛˜‚‡˘ Í‡‰ËÓ‚‡ÒÍÛÎ‡Ì‡ ÒÏ˙Ú, ÌÂÙ‡Ú‡ÎÂÌ ËÌÙ‡ÍÚ Ì‡ ÏËÓÍ‡‰‡ Ë ÌÂÙ‡Ú‡ÎÂÌ ËÌÒÛÎÚ. ãÂ˜ÂÌËÂÚÓ Ò rosuvastatin 20 mg ‚Ó‰Ë ‰Ó Â‰ÛÍˆËﬂ Ì‡ Ó·˘‡Ú‡ ÒÏ˙ÚÌÓÒÚ Ò 20% ‚ Ò‡‚ÌÂÌËÂ Ò ÔÎ‡ˆÂ·Ó (=0.02), ‚ ÔÓÔÛÎ‡ˆËﬂ ·ÂÁ ÛÒÚ‡ÌÓ‚ÂÌ‡ ÍÓÓÌ‡Ì‡ ·ÓÎÂÒÚ Ì‡ Ò˙ˆÂÚÓ. àÁ‚ÂÒÚÌÓ Â, ˜Â ÔÓ-ÒÚ‡ËÚÂ ÔÓÛ˜‚‡ÌËﬂ (WOSCOPS, AFCAPS/TexCAPS, PROSPER) (14-16) ÌÂ ÛÒÔﬂ‚‡Ú ‰‡ ‰ÓÍ‡Ê‡Ú Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ ËÒÍ‡ ÓÚ Ô˙‚Ë àåà ÔË Ô‡ˆËÂÌÚËÚÂ ·ÂÁ ÍÓÓÌ‡Ì‡ Ô‡ÚÓÎÓ„Ëﬂ. ëíÄíàçà à ÇíéêàóçÄ èêéîàãÄäíàäÄ çÄ àåà èÓÛ˜‚‡ÌÂÚÓ SPARCL (Stroke Prevention by Aggressive Reduction in Cholesterol Levels) (17) Â Ô˙‚ÓÚÓ ÔÓÛ˜‚‡ÌÂ, 145 ˆÂÎÚ‡ Ì‡ ÍÓÂÚÓ Â ‰‡ Ì‡Ô‡‚Ë ÔÓÒÔÂÍÚË‚Ì‡ ÓˆÂÌÍ‡ Ì‡ ÎÂ˜ÂÌËÂÚÓ Ò˙Ò ÒÚ‡ÚËÌË ÔË Ô‡ˆËÂÌÚË, ÔÂÍ‡‡ÎË àåà. ÇÍÎ˛˜ÂÌË Ò‡ 4 731 ·ÓÎÌË ÔÂÊË‚ÂÎË àåà ËÎË íàÄ ‚ ‡ÏÍËÚÂ Ì‡ 6 ÏÂÒÂˆ‡ ÓÚ Á‡ÔÓ˜‚‡ÌÂ Ì‡ ÒÚ‡ÚËÌÓ‚ÓÚÓ ÎÂ˜ÂÌËÂ. è‡ˆËÂÌÚËÚÂ Ò‡ ·ÂÁ ÍÓÓÌ‡Ì‡ Ô‡ÚÓÎÓ„Ëﬂ Ë Ò ÌÓÏ‡ÎÌÓ ıÓÎÂÒÚÂÓÎÓ‚Ó ÌË‚Ó. ç‡Ô‡‚ÂÌÓ Â ÔÓÒÎÂ‰ﬂ‚‡ÌÂ ‰Ó ‚˙ÁÌËÍ‚‡ÌÂ Ì‡ ÒÎÂ‰‚‡˘ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ ËÌˆË‰ÂÌÚ. êÂÁÛÎÚ‡ÚËÚÂ ÔÓÍ‡Á‚‡Ú, ˜Â ËÌÚÂÌÁË‚Ì‡Ú‡ ÚÂ‡ÔËﬂ Ò˙Ò ÒÚ‡ÚËÌË (atorvastatin 80 mg), ÔËÎÓÊÂÌ‡ ‚Â‰Ì‡„‡ ÒÎÂ‰ ËÒıÂÏË˜ÌËﬂ ÌÂÍ‡‰ËÓÂÏ·ÓÎË˜ÂÌ ÏÓÁ˙˜ÂÌ ËÌÒÛÎÚ, Ì‡Ï‡Îﬂ‚‡ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ ÓÚ ‚ÚÓË àåà Ò 16%. íÂÁË ‰‡ÌÌË ÔÓÚ‚˙Ê‰‡‚‡Ú ıËÔÓÚÂÁ‡Ú‡, ˜Â ÒÚ‡ÚËÌËÚÂ ÒÚ‡·ËÎËÁË‡Ú ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡Ú‡ ÔÎ‡Í‡ ‚ ‡ÌÌËﬂ ÒÎÂ‰ËÌÒÛÎÚÂÌ ÔÂËÓ‰, Í‡ÍÚÓ Ë ˜Â ËÏ‡Ú ÌÂ‚ÓÔÓÚÂÍÚË‚ÂÌ ÂÙÂÍÚ. éÒÌÓ‚‡‚‡ÈÍË ÒÂ Ì‡ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ SPARCL, ÄÏÂËÍ‡ÌÒÍ‡Ú‡ ÄÒÓˆË‡ˆËﬂ ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ ÔÂÔÓ˙˜‚‡ ÔËÎ‡„‡ÌÂ Ì‡ ËÌÚÂÌÁË‚Ì‡ ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËﬂ ÔË ·ÓÎÌËÚÂ Ò ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡ „ÂÌÂÁ‡ Ì‡ àåà ËÎË íàÄ, Á‡ Ì‡Ï‡Îﬂ‚‡ÌÂ ËÒÍ‡ ÓÚ ËÌÒÛÎÚ ËÎË Ò˙‰Â˜ÌÓ-Ò˙‰Ó‚Ë ËÌˆË‰ÂÌÚË (ÍÎ‡Ò 1, ÌË‚Ó Ç), ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÚÓ‚‡, ˜Â ÌﬂÏ‡Ú ‰ÓÍ‡Á‡Ì‡ àÅë (18). Ö‚ÓÔÂÈÒÍ‡Ú‡ ÄÒÓˆË‡ˆËﬂ ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ Ò˙˘Ó ÔÂÔÓ˙˜‚‡ ÒÚ‡ÚËÌÓ‚Ó ÎÂ˜ÂÌËÂ Á‡ ‚ÚÓË˜Ì‡ ÔÓÙËÎ‡ÍÚËÍ‡ ÔË ‚ÒË˜ÍË ·ÓÎÌË Ò ÌÂÍ‡‰ËÓÂÏ·ÓÎË˜ÂÌ àåà (19). äÄäÇà ëÄ èêàñÖãçàíÖ çàÇÄ áÄ LDL-ïéãÖëíÖêéã äÓÎÍÓÚÓ ÔÓ-ÌËÒÍ‡ Â ÍÓÌˆÂÌÚ‡ˆËﬂÚ‡ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡, ÚÓÎÍÓ‚‡ ÔÓ-ÁÌ‡˜ËÚÂÎÌ‡ Â Â‰ÛÍˆËﬂÚ‡ Ì‡ ËÒÍ‡ ÔÓ ÓÚÌÓ¯ÂÌËÂ Í‡ÍÚÓ Ì‡ Ô˙‚Ë˜Ì‡Ú‡, Ú‡Í‡ Ë Ì‡ ‚ÚÓË˜Ì‡Ú‡ ÔÂ‚ÂÌˆËﬂ Ì‡ ËÌÒÛÎÚ‡. åÂÚ‡‡Ì‡ÎËÁ˙Ú, ÔÓ‚Â‰ÂÌ ÓÚ Amarenco Ë Labreuche (3), ‚ÍÎ˛˜‚‡˘ 165 000 ‚ËÒÓÍÓËÒÍÓ‚Ë ÎËˆ‡ ‰ÂÏÓÌÒÚË‡, ˜Â ‚ÒÂÍË 1 mmol/l ÔÓÌËÊ‡‚‡ÌÂ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡ ÒÂ ‡‚Ìﬂ‚‡ Ì‡ 21% Â‰ÛÍˆËﬂ Ì‡ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ Á‡ ËÌÒÛÎÚ. êÂÁÛÎÚ‡ÚËÚÂ ÓÚ ‰Û„ ÏÂÚ‡‡Ì‡ÎËÁ (PROVE-IT, TNT, IDEAL, ALLIANCE, SEARCH) (8,20-22), ‚ÍÎ˛˜‚‡˘ 42 054 Ô‡ˆËÂÌÚË Ò àÅë ÒÓ˜‡Ú, ˜Â ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò ÒÚ‡Ì‰‡ÚÌ‡Ú‡ ÚÂ‡ÔËﬂ, ËÌÚÂÌÁË‚Ì‡Ú‡ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘‡ ÚÂ‡ÔËﬂ Ì‡Ï‡Îﬂ‚‡ ËÒÍ‡ ÓÚ ËÌÒÛÎÚ Ò 13%. Ç˙‚ ‚ÚÓË˜Ì‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ ÌÂÍ‡‰ËÓÂÏ·ÓÎË˜ÌËﬂ ËÌÒÛÎÚ ËÌÚÂÌÁË‚Ì‡Ú‡ Â‰ÛÍˆËﬂ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡ ÔÓÒÂ‰ÒÚ‚ÓÏ ÒÚ‡ÚËÌÓ‚‡ ÚÂ‡ÔËﬂ Ò˙˘Ó ‚Ó‰Ë ‰Ó ÒË„ÌËÙËÍ‡ÌÚÌ‡ Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ ÂˆË‰Ë‚. ÇÒﬂÍÓ Û‚ÂÎË˜‡‚‡ÌÂ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡ Ò 1 mmol/l Û‚ÂÎË˜‡‚‡ ËÒÍ‡ ÓÚ Ò˙‰Ó‚‡ ÒÏ˙Ú Ò 0,3 % (23). ÄÏÂËÍ‡ÌÒÍ‡Ú‡ ‡ÒÓˆË‡ˆËﬂ ÔÓ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë, Í‡ÍÚÓ Ë Ú‡ÁË ÔÓ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ ÔÂÔÓ˙˜‚‡Ú ÏÂ‰ËÍ‡ÏÂÌÚË ÓÚ „ÛÔ‡Ú‡ Ì‡ ÒÚ‡ÚËÌËÚÂ Á‡ Ô‡ˆËÂÌÚË Ò ÛÒÚ‡ÌÓ‚ÂÌ‡ àÅë ËÎË ÒËÏÔÚÓÏ‡ÚË˜Ì‡ ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡ ·ÓÎÂÒÚ, Í‡ÚÓ Úﬂ·‚‡ ‰‡ ÒÂ ÔÓÒÚË„Ì‡Ú ÔËˆÂÎÌË ÌË‚‡ Á‡ LDL-ıÓÎÂÒÚÂÓÎ ÔÓ‰ 2,6 mmol/l. á‡ Ô‡ˆËÂÌÚË Ò ÏÌÓ„Ó ‚ËÒÓÍ ËÒÍ Ë ÏÌÓÊÂÒÚ‚Ó ËÒÍÓ‚Ë Ù‡ÍÚÓË ÒÂ ÔÂÔÓ˙˜‚‡Ú ÌË‚‡ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ ÔÓ‰ 1,8 mmol/l. (ÍÎ‡Ò 1, ÌË‚Ó Ä) (11). éÚ ‚‡ÊÌÓ ÁÌ‡˜ÂÌËÂ Á‡ ÔÓÙËÎ‡ÍÚËÍ‡Ú‡ Ì‡ ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ËÚÂ ËÌˆË‰ÂÌÚË Â ‰ÓÒÚË„‡ÌÂÚÓ Ì‡ ÔËˆÂÎÌË ÌË‚‡ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡, ÍÓÂÚÓ ÒÂ ÔÓÒÚË„‡ Ò ‡ÁÎË˜Ì‡ ‰ÓÁËÓ‚Í‡ Ì‡ ËÁÔÓÎÁ‚‡ÌËÚÂ ‚ Ô‡ÍÚËÍ‡Ú‡ ÒÚ‡ÚËÌË. Ñ‡ÌÌËÚÂ ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ STELLAR ‰ÂÏÓÌÒÚË‡Ú, ˜Â ÔËˆÂÎÌÓÚÓ ÌË‚Ó Ì‡ LDL-ıÓÎÂÒÚÂÓÎ (<2,6 mmol/L) ÒÂ ÔÓÒÚË„‡ ÔË Ì‡È-‚ËÒÓÍ ÔÓˆÂÌÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÍÓËÚÓ ÔÓÎÛ˜‡‚‡Ú rosuvastatin ‚ Ò‡‚ÌÂÌËÂ Ò atorvastatin, simvastatin Ë pravastatin (ÙË„.2) (24). ëíÄíàçà à äÄêéíàÑçÄ ÄíÖêéëäãÖêéáÄ ÑÓ·Â ËÁ‚ÂÒÚÌÓ Â, ˜Â ÒÚ‡ÚËÌËÚÂ ÓÒ‚ÂÌ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘Ëﬂ ÒË ÂÙÂÍÚ – ÓÒÌÓ‚ÌÓ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ LDL-ıÓ- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology îË„. 2. ÑﬂÎ Ô‡ˆËÂÌÚË (%), ÔÓÒÚË„Ì‡ÎË ÔÓˆÂÎÌË ÒÚÓÈÌÓÒÚË Ì‡ ıÓÎÂÒÚÂÓÎ‡, Ò˙„Î‡ÒÌÓ NCEP Adult Treatment Panel 3 ÎÂÒÚÂÓÎ‡, ËÏ‡Ú Ë ‰Û„Ë ÏÌÓ„Ó ‚‡ÊÌË – ÔÎÂÈÓÚÓÔÌË Ò‚ÓÈÒÚ‚‡. ëÚ‡ÚËÌËÚÂ ÒÚ‡·ËÎËÁË‡Ú ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡Ú‡ ÔÎ‡Í‡ ÔÓ ÏÌÓ„Ó Ì‡˜ËÌË: ÔÓ‰Ó·ﬂ‚‡Ú ÂÌ‰ÓÚÂÎÌ‡Ú‡ ‰ËÒÙÛÌÍˆËﬂ, ËÏ‡Ú ‡ÌÚËËÌÙÎ‡Ï‡ÚÓÌÓ ‰ÂÈÒÚ‚ËÂ Ë ‡ÌÚËÔÓÎËÙÂ‡ÚË‚ÌÓ ‰ÂÈÒÚ‚ËÂ, Ì‡Ï‡Îﬂ‚‡Ú ÚÓÏ·Ó„ÂÌÌËﬂ ÔÓÚÂÌˆË‡Î Ì‡ ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡Ú‡ ÔÎ‡Í‡. íÓ‚‡ Ó·ÛÒÎ‡‚ﬂ „ÓÎﬂÏ‡Ú‡ ÔÓÎÁ‡ ÓÚ Úﬂı Á‡ ÔÓÙËÎ‡ÍÚËÍ‡Ú‡ Ì‡ Ò˙‰Ó‚Ë ËÌˆË‰ÂÌÚË ÔË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â Ì‡ÎËˆÂ ‡ÚÂÓÒÍÎÂÓÚË˜ÂÌ ÔÓˆÂÒ. ÅÂÁÒÔÓÌÓ Â, ˜Â ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡ Â ·‡‚ÂÌ ÔÓˆÂÒ, ‡Á‚Ë‚‡˘ ÒÂ ‚ ÔÓ‰˙ÎÊÂÌËÂ Ì‡ ‰ÂÒÂÚÍË „Ó‰ËÌË, ÔÓ‡‰Ë ÍÓÂÚÓ, ‡ÍÓ Úﬂ·‚‡ ‰‡ ÒÂ ÓˆÂÌË ‚˙Á‰ÂÈÒÚ‚ËÂÚÓ Ì‡ ‡ÁÎË˜ÌË Ù‡ÍÚÓË ‚˙ıÛ ÌÂ„Ó‚‡Ú‡ ÔÓ„ÂÒËﬂ, Â ÌÂÓ·ıÓ‰ËÏÓ ÔÓÒÎÂ‰ﬂ‚‡ÌÂ Ì‡ „ÓÎﬂÏ ·ÓÈ Ô‡ˆËÂÌÚË ‚ ÔÓ‰˙ÎÊÂÌËÂ Ì‡ ÏÌÓ„Ó „Ó‰ËÌË. íÓ‚‡ Ì‡Î‡„‡ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÔÂˆËÙË˜ÌË Ï‡ÍÂË Á‡ ÒÚÂÔÂÌÚ‡ Ì‡ ‡ÚÂÓÒÍÎÂÓÁ‡ Ë ÌÂÈÌ‡Ú‡ ÔÓ„ÂÒËﬂ. åÂÊ‰Û ÏÌÓ„ÓÚÓ ËÁÔÓÎÁ‚‡ÌË Ú‡ÍË‚‡ Ï‡ÍÂË, Ì‡È-‰Ó·ËﬂÚ ÏÂÚÓ‰ ÒÂ ·‡ÁË‡ Ì‡ ËÁÏÂ‚‡ÌÂÚÓ Ì‡ Í‡ÓÚË‰ÌËﬂ ËÌÚËÏ‡-ÏÂ‰Ëﬂ ÍÓÏÔÎÂÍÒ (intima-media thickness, IMT). íÓÁË Ï‡ÍÂ ÒÂ ËÁÏÂ‚‡ ÔË ‰ÛÔÎÂÍÒ ÒÍÂÌË‡ÌÂ, Í˙‰ÂÚÓ ÒÚÂÌ‡Ú‡ Ì‡ Ó·˘‡Ú‡ Ò˙ÌÌ‡ ‡ÚÂËﬂ ËÏ‡ ı‡‡ÍÚÂÂÌ Ó·‡Á – Ò ‰‚Â ÂıÓ„ÂÌÌË ÔÓ‚˙ıÌÓÒÚË. Ç˙ÚÂ¯Ì‡Ú‡ ÔÓ‚˙ıÌÓÒÚ ÓÚ„Ó‚‡ﬂ Ì‡ „‡ÌËˆ‡Ú‡ Í˙‚ - ËÌÚËÏ‡, ‡ ‚˙Ì¯Ì‡Ú‡ - Ì‡ „‡ÌËˆ‡Ú‡ ÏÂ‰Ëﬂ – ‡‰‚ÂÌÚËˆËﬂ. IMT ÒÂ Û‚ÂÎË˜‡‚‡ ÔË Ô‡ˆËÂÌÚË Ò ˆÂÂ·Ó‚‡ÒÍÛÎ‡ÌË ËÒÍÓ‚Ë Ù‡ÍÚÓË: ıËÔÂıÓÎÂÒÚÂÓÎÂÏËﬂ, Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, Ú˛Ú˛ÌÓÔÛ¯ÂÌÂ, ‡ÚÂË‡ÎÌ‡ ıËÔÂÚÓÌËﬂ. ì‚ÂÎË˜‡‚‡ÌÂÚÓ Ì‡ IMT Â ·ÂÎÂ„ Á‡ ‡ÌÌ‡ ‡ÚÂÓÒÍÎÂÓÁ‡, Á‡ ÒÚÂÔÂÌÚ‡ Ì‡ ÌÂÈÌÓÚÓ ‡Á‚ËÚËÂ Ë ÔÓ„ÂÒËﬂ Ë Â ÌÂÁ‡‚ËÒËÏ ÔÂ‰ËÍÚÓ Ì‡ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Ë ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ (25-30). åÌÓÊÂÒÚ‚Ó ÔÓÛ˜‚‡ÌËﬂ (31-40) Ò‡ ÔÓÒ‚ÂÚÂÌË Ì‡ ËÁÒÎÂ‰‚‡ÌÂ ‚˙ÁÍ‡Ú‡ ÏÂÊ‰Û ‰Â·ÂÎËÌ‡Ú‡ Ì‡ IMT Ë ÒÚ‡ÚËÌÓ‚ÓÚÓ ÎÂ˜ÂÌËÂ. èÓ˜ÚË ‚ÒË˜ÍË ÔÓÍ‡Á‚‡Ú Á‡·‡‚ﬂÌÂ ÔÓ„ÂÒËﬂÚ‡ Ì‡ IMT ÒÂ‰ ÎÂÍÛ‚‡ÌËÚÂ ·ÓÎÌË ‚ Ò‡‚ÌÂÌËÂ Ò ÔÎ‡ˆÂ·Ó. åÂÚ‡‡Ì‡ÎËÁ (41), Ó·Ó·˘‡‚‡˘ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ‰Â‚ÂÚ ÓÚ Úﬂı, ÔÓÍ‡Á‚‡ Ì‡ÎË˜ËÂÚÓ Ì‡ ÒÚÓ„‡ ÍÓÂÎ‡ˆËﬂ ÏÂÊ‰Û ÒÚÂÔÂÌÚ‡ Ì‡ Â‰ÛÍˆËﬂ Ì‡ LDL- ıÓÎÂÒÚÂÓÎ‡ Ë Ú‡ÁË Ì‡ IMT (r=0.65; P=0.004). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ‚ÒﬂÍÓ ‰ÂÒÂÚÔÓˆÂÌÚÌÓ Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ LDL-ıÓÎÂÒÚÂÓÎ‡ Ì‡Ï‡Îﬂ‚‡ IMT Ò 0,73% Ì‡ „Ó‰ËÌ‡. èÓ‚Â˜ÂÚÓ ÓÚ ÚÂÁË ÔÓÛ˜‚‡ÌËﬂ ‚ÍÎ˛˜‚‡Ú ‚ËÒÓÍÓËÒÍÓ‚Ë Ô‡ˆËÂÌÚË Ò ÔÓ‚Ë¯ÂÌ LDL-ıÓ- 146 ÎÂÒÚÂÓÎ ËÎË Ú‡ÍË‚‡, ÔÂÍ‡‡ÎË àåà. Ç ÔÓÛ˜‚‡ÌÂÚÓ METEOR (42) ÒÂ ËÁÔÓÎÁ‚‡ B-mode ÛÎÚ‡Á‚ÛÍ Á‡ ‰‡ ÒÂ ÓÔÂ‰ÂÎË ‰‡ÎË ÎÂ˜ÂÌËÂÚÓ Ò rosuvastatin ÏÓÊÂ ‰‡ Á‡·‡‚Ë ÔÓ„ÂÒËﬂÚ‡ ËÎË ‰‡ ÔÂ‰ËÁ‚ËÍ‡ Â„ÂÒËﬂ Ì‡ Í‡ÓÚË‰Ì‡Ú‡ IMT ÔË ‡ÒËÏÔÚÓÏ‡ÚË˜ÌË Ô‡ˆËÂÌÚË Ò ÌËÒ˙Í Ò˙‰Ó‚ ËÒÍ. ãÂ˜ÂÌËÂÚÓ Ò rosuvastatin 40 mg Á‡ 2-„Ó‰Ë¯ÂÌ ÔÂËÓ‰ ÁÌ‡˜ËÏÓ Á‡·‡‚ﬂ ÒÚÂÔÂÌÚ‡ Ì‡ ‡Á‚ËÚËÂ Ì‡ IMT ‚ Ò‡‚ÌÂÌËÂ Ò ÔÎ‡ˆÂ·Ó. èÓÛ˜‚‡ÌÂÚÓ METEOR Â ‚‡ÊÌÓ, Ú.Í. Â Â‰ËÌÒÚ‚ÂÌÓÚÓ ÔÎ‡ˆÂ·Ó ÍÓÌÚÓÎË‡ÌÓ, ÍÓÂÚÓ ÔÓÍ‡Á‚‡ ÔÓÁËÚË‚ÂÌ ÂÙÂÍÚ ‚˙ıÛ ‡ÚÂÓÒÍÎÂÓÁ‡Ú‡ ÔË Ô‡ˆËÂÌÚË Ò ÚÓÎÍÓ‚‡ ‡ÌÌË ·ÂÎÂÁË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë Ò ÌËÒ˙Í ËÒÍ ÓÚ ÍÓÓÌ‡ÌË ËÌˆË‰ÂÌÚË (Framingham 10 „Ó‰Ë¯ÂÌ ËÒÍ < 10%). Rosuvastatin ÔÓÌËÊ‡‚‡ LDL-ıÓÎÂÒÚÂÓÎ‡ Ò 48,8% Ë ÔÓÍ‡˜‚‡ HDL–ıÓÎÂÒÚÂÓÎ‡ Ò 8,0% ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ËÁıÓ‰ÌËÚÂ ÒÚÓÈÌÓÒÚË. íÓ ÌÂ ÓÚ˜ËÚ‡ Â„ÂÒËﬂ Ì‡ ‡ÚÂÓÒÍÎÂÓÚË˜ÌËﬂ ÔÓˆÂÒ ‚ Í‡ÓÚË‰ÌËÚÂ ‡ÚÂËË, Á‡ ‡ÁÎËÍ‡ ÓÚ ÔÓÛ˜‚‡ÌÂÚÓ ASTEROID, ÍÓÂÚÓ ÔÓÍ‡Á‚‡ Â„ÂÒËﬂ Ì‡ ÍÓÓÌ‡Ì‡Ú‡ ‡ÚÂÓÒÍÎÂÓÁ‡ ÔË ÔËÎÓÊÂÌËÂ Ì‡ rosuvastatin 40 mg, ÓÚ˜ÂÚÂÌ‡ ˜ÂÁ ËÌÚ‡‚‡Á‡ÎÌÓ ÛÎÚ‡Á‚ÛÍÓ‚Ó ËÁÒÎÂ‰‚‡ÌÂ (43). è‡ˆËÂÌÚËÚÂ ÓÚ ASTEROID Ò‡ Ò ‡ÚÂÓÒÍÎÂÓÚË˜ÂÌ ÔÓˆÂÒ ‚ Ì‡ÔÂ‰Ì‡Î‡ Ù‡Á‡, ÍÓÂÚÓ ÔÓÁ‚ÓÎﬂ‚‡ ‰‡ ÒÂ ÓÚ˜ÂÚÂ Ë Â„ÂÒËﬂÚ‡, ‰ÓÍ‡ÚÓ Ú‡Í˙‚ ÎËÔÒ‚‡ ‚ METEOR. óÂÁ ÌÂËÌ‚‡ÁË‚ÌË ËÁÒÎÂ‰‚‡ÌËﬂ Ì‡ Í‡ÓÚË‰ÌËÚÂ ‡ÚÂËË (‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËﬂ, ﬂ‰ÂÌÓÏ‡„ÌËÚÂÌ ÂÁÓÌ‡ÌÒ) ÒÂ ÔÓÎÛ˜‡‚‡ ËÌÙÓÏ‡ˆËﬂ Á‡ Ì‡ÎË˜ËÂÚÓ, „ÓÎÂÏËÌ‡Ú‡, ÏÂÒÚÓÔÓÎÓÊÂÌËÂÚÓ Ë ÒÚÛÍÚÛ‡Ú‡ Ì‡ ‡ÚÂÓÒÍÎÂÓÚË˜ÌËÚÂ ÔÎ‡ÍË. èÓÒÎÂ‰ÌËÚÂ ËÏ‡Ú ‡ÁÎË˜ÂÌ Ò˙ÒÚ‡‚, ÔÓ‡‰Ë ÍÓÂÚÓ ˜ÂÁ Ç-mode ÒÂ ‚ËÁÛ‡ÎËÁË‡Ú Í‡ÚÓ ıËÔÂÂıÓ„ÂÌÌË, ıËÔÓÂıÓ„ÂÌÌË ËÎË ıÂÚÂÓ„ÂÌÌË. ë˜ËÚ‡ ÒÂ, ˜Â ÓÒÌÓ‚ÌËﬂÚ Ù‡ÍÚÓ ÍÓÈÚÓ ÓÔÂ‰ÂÎﬂ ÔÎ‡ÍÓ‚‡Ú‡ ÒÚ‡·ËÎÌÓÒÚ, Â ÔÎ‡ÍÓ‚‡Ú‡ ÒÚÛÍÚÛ‡, ‡ ÌÂ ÚÓÎÍÓ‚‡ ÔÎ‡ÍÓ‚‡Ú‡ „ÓÎÂÏËÌ‡ (44). ïËÔÂÂıÓ„ÂÌÌËÚÂ ÔÎ‡ÍË Ò‡ ·Ó„‡ÚË Ì‡ ÙË·ËÌ Ë Í‡ÎˆËÂ‚Ë ÓÚÎ‡„‡ÌËﬂ. ïËÔÓÂıÓ„ÂÌÌËÚÂ ÔÎ‡ÍË ËÏ‡Ú ·Ó„‡ÚÓ ÎËÔË‰ÌÓ Ò˙‰˙Ê‡ÌËÂ Ë Ì‡ÎË˜ËÂ Ì‡ ÌÂÍÓÚË˜ÌË Û˜‡ÒÚ˙ˆË. íÂ Ò‡ ÔÓ-‡ÌËÏË, Ò ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ÛÔÚÛ‡ Ë ÎÂÒÌÓ ÒÂ ÔÂ‚˙˘‡Ú ‚ ËÁÚÓ˜ÌËÍ Ì‡ ÏÓÁ˙˜ÂÌ ÂÏ·ÓÎËÁ˙Ï. è‡ˆËÂÌÚËÚÂ Ò ıËÔÓÂıÓ„ÂÌÌË ÔÎ‡ÍË ËÏ‡Ú ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ àåà, ÔÓ-‚ËÒÓÍ‡ ˜ÂÒÚÓÚ‡ Ì‡ ‡ÒËÏÔÚÓÏÌË ÏÓÁ˙˜ÌË ËÌÙ‡ÍÚË Ë ÍÓÓÌ‡ÌË ËÌˆË‰ÂÌÚË. ëÌËÊ‡‚‡ÌÂÚÓ Ì‡ HDL- ıÓÎÂÒÚÂÓÎ‡ Û‚ÂÎË˜‡‚‡ ËÒÍ‡ ÓÚ Ó·‡ÁÛ‚‡ÌÂ Ì‡ ıËÔÓÂıÓ„ÂÌÌË ÔÎ‡ÍË Ì‡‰ 8 Ô˙ÚË (45-48). çﬂÍÓÎÍÓ Ï‡ÎÍË ÔÓÛ˜‚‡ÌËﬂ ÔÓÍ‡Á‚‡Ú, ˜Â ÎÂ˜ÂÌËÂÚÓ Ò˙Ò ÒÚ‡ÚËÌË ‚Ó‰Ë ‰Ó ÔÓÏﬂÌ‡ ‚ ÔÎ‡ÍÓ‚‡Ú‡ ÏÓÙÓÎÓ„Ëﬂ (48-51). ç‡Ï‡Îﬂ‚‡ÌÂÚÓ Ì‡ ÔÎ‡ÁÏÂÌËÚÂ ÌË‚‡ Ì‡ ıÓÎÂÒÚÂÓÎ‡ ÔÂ‰ËÁ‚ËÍ‚‡ ÔÓÏÂÌË „Î‡‚ÌÓ ‚ ÍÓÏÔÓÌÂÌÚËÚÂ Ì‡ ÔÎ‡ÍËÚÂ Ò˙Ò ÁÌ‡˜ËÚÂÎÌÓ Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ ÎËÔË‰ÌÓÚÓ ËÏ Ò˙‰˙Ê‡ÌËÂ Ë ÔÓ-Ï‡ÎÍÓ ‚ÎËﬂÌËÂ ‚˙ıÛ ÙË·ÓÁÌ‡Ú‡ ËÏ Ó·‚Ë‚Í‡. í‡Í‡ Ì‡ÔËÏÂ ÔÓÒÂ‰ÒÚ‚ÓÏ ‰ÛÔÎÂÍÒ ‰ÓÔÎÂÓ‚‡ ÒÓÌÓ„‡ÙËﬂ Â ÓÚ˜ÂÚÂÌÓ Û‚ÂÎË˜‡‚‡ÌÂ Ì‡ ÔÎ‡ÍÓ‚‡Ú‡ ÂıÓ„ÂÌÌÓÒÚ ÒÎÂ‰ ÔÓ‰˙ÎÊËÚÂÎÌÓ ÎÂ˜ÂÌËÂ (50). ÑÛ„Ó ÌÓ‚Ó Ï‡ÎÍÓ ÔÓÛ˜‚‡ÌÂ ‰ÂÏÓÌÒÚË‡, ˜Â ËÌÚÂÌÁË‚Ì‡Ú‡ ÎËÔË‰ÓÔÓÌËÊ‡‚‡˘‡ ÚÂ‡ÔËﬂ Á‡ 6-ÏÂÒÂ˜ÂÌ ÔÂËÓ‰ ÓÚ ‚ÂÏÂ ÔË ·ÓÎÌË Ò Ì‡‰ 40% ÒÚÂÌÓÁË ·ÂÁ ËÌ‰ËÍ‡ˆËË Á‡ ËÌÚÂ‚ÂÌˆËﬂ, ÔÓ‰Ó·ﬂ‚‡ ÔÎ‡ÍÓ‚‡Ú‡ ÂıÓ„ÂÌÌÓÒÚ, ÓÚ˜ÂÚÂÌ‡ ˜ÂÁ GSM (GrayScale Median) (51). àÁÔÓÎÁ‚‡ÈÍË ﬂ‰ÂÌÓÏ‡„ÌËÚÂÌ ÂÁÓÌ‡ÌÒ R. Hunter et al. (52) ÔÓÍ‡Á‚‡Ú, ˜Â 24 ÏÂÒÂ˜ÌÓÚÓ ÎÂ˜ÂÌËÂ Ò rosuvastatin ‚Ó‰Ë ‰Ó Ì‡Ï‡Îﬂ‚‡ÌÂ Ì‡ ‚˙ÚÂÔÎ‡ÍÓ‚ËÚÂ ÎËÔË‰ÌË ÌÂÍÓÚË˜ÌË ﬂ‰‡. èË ËÏÛÌÓıËÒÚÓıËÏË˜ÂÒÍÓ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ÓÚ‰ÂÎÂÌËÚÂ ÔË Í‡ÓÚË‰Ì‡ ÂÌ‰‡ÚÂÂÍÚÓÏËﬂ ÔÎ‡ÍË Â ÛÒÚ‡ÌÓ‚ÂÌ‡ ÁÌ‡˜ËÏ‡ ‡ÁÎËÍ‡ ÔÓ ÓÚÌÓ¯ÂÌËÂ ËÌÙËÎÚ‡ˆËﬂÚ‡ ÓÚ Ï‡ÍÓÙ‡„Ë Ë ÂÍÒÔÂÒËﬂÚ‡ Ì‡ Ï‡ÚËÍÒÌ‡Ú‡ ÏÂÚ‡ÎÓÔÓÚÂËÌ‡Á‡ (53, 54). è‡ˆËÂÌÚËÚÂ ÔÓ‚ÂÊ‰‡ÎË ÒÚ‡ÚËÌÓ‚Ó ÎÂ˜ÂÌËÂ, ËÏ‡Ú ÒË„ÌËÙËÍ‡ÌÚÌÓ ÔÓ-Ï‡Î˙Í ÔÓˆÂÌÚ Ì‡ ËÌÙËÎÚ‡ˆËﬂ ÓÚ Ï‡ÍÓÙ‡„Ë Ë ÂÍÒÔÂÒËﬂ Ì‡ Ï‡ÚËÍÒÌ‡Ú‡ ÏÂÚ‡ÎÓÔÓÚÂËÌ‡Á‡ ‚ Ò‡‚ÌÂÌËÂ Ò ÌÂÎÂÍÛ‚‡ÌË ·ÓÎÌË. íÂÁË ‰‚‡ Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ÔÓÍ‡Á‡ÚÂÎﬂ Ò‡ Ï‡ÍÂË Á‡ ‚ËÒÓÍÓËÒÍÓ‚Ë ‡ÚÂÓÒÍÎÂÓÚË˜ÌË ÔÎ‡ÍË Ë ÔÎ‡ÍÓ‚‡ ÌÂÒÚ‡·ËÎÌÓÒÚ. êÂÁÛÎÚ‡ÚËÚÂ ÔÓÚ‚˙Ê‰‡‚‡Ú ÚÓ‚‡, ˜Â ÒÚ‡ÚËÌËÚÂ ‚ÎËﬂﬂÚ ÌÂ Ò‡ÏÓ ‚˙ıÛ ÎËÔË‰ÌÓÚÓ Ò˙‰˙ÊËÏÓ, ÌÓ ÔÓ‚ÎËﬂ‚‡Ú Ë ËÌÙÎ‡Ï‡ÚÓÌËÚÂ Ë „Î‡‰ÍÓÏÛÒÍÛÎÌË ÍÎÂÚÍË, ÏÂÚ‡ÎÓÔÓÚÂËÌ‡ÁÌ‡Ú‡ ‡ÍÚË‚ÌÓÒÚ Ë ÓÍÒË‰‡ÚË‚ÌËﬂ ÒÚÂÒ. èÓÛ˜‚‡ÌÂÚÓ SPARCLE ÔÓÍ‡Á‚‡ ÔÓ-„ÓÎﬂÏ‡ ÔÓÎÁ‡ ÔÓ ÓÚÌÓ¯ÂÌËÂ Â‰ÛÍˆËﬂ Ì‡ ËÒÍ‡ ÓÚ àåà ÔË ·ÓÎÌËÚÂ Ò Í‡ÓÚË‰ÌË ÒÚÂÌÓÁË, Í‡ÚÓ ÒÚ‡ÚËÌÓ‚ÓÚÓ ÎÂ˜ÂÌËÂ Ì‡Ï‡Îﬂ‚‡ ÂÎ‡ÚË‚ÌËﬂ ËÒÍ Ò 33%. áÄäãûóÖçàÖ ëÚ‡ÚËÌËÚÂ ËÏ‡Ú ‡ÁÎË˜ÌË ÂÙÂÍÚË ‚˙ıÛ ‡ÚÂÓÒÍÎÂÓÚË˜Ì‡Ú‡ ÔÎ‡Í‡, Í‡ÚÓ ÔÓÏÂÌËÚÂ ‚ ÂÌ‰ÓÚÂÎÌ‡Ú‡ ‰ËÒÙÛÌÍˆËﬂ Ë ÔÎ‡ÍÓ‚‡Ú‡ ÒÚÛÍÚÛ‡ ‰ÓÔËÌ‡ÒﬂÚ Á‡ ÔÂ‚‡ÌÚË‚ÌÓÚÓ ËÏ ‰ÂÈÒÚ‚ËÂ ÔË ÏÓÁ˙˜ÌÓÒ˙‰Ó‚ËÚÂ Á‡·ÓÎﬂ‚‡ÌËﬂ. èÓÙËÎ‡ÍÚËÍ‡Ú‡ Ò˙Ò ÒÚ‡ÚËÌË ËÏ‡ ¯ËÓÍÓ ÔËÎÓÊÂÌËÂ Í‡ÍÚÓ ‚ Ô˙‚Ë˜Ì‡Ú‡, Ú‡Í‡ Ë ‚˙‚ ‚ÚÓË˜Ì‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ åëÅ. íﬂ Â Ì‡È-‚‡ÊÌÓÚÓ ÔÓÒÚËÊÂÌËÂ ‚ ÔÂ‚ÂÌˆËﬂÚ‡ Ì‡ ËÒıÂÏË˜ÌËﬂ ËÌÒÛÎÚ ÓÚ ‚˙‚ÂÊ‰‡ÌÂÚÓ Ì‡ ‡ÒÔËËÌ Ë ‡ÌÚËıËÔÂÚÂÌÁË‚ÌÓÚÓ ÎÂ˜ÂÌËÂ. ãàíÖêÄíìêÄ 1. 2. 3. 4 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 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American Heart Journal, 155, 3, 584.e1-584.e8, 148 53. Crisby M., Nordin-Fredriksson G., Shah P. et al. Pravastatin Treatment Increases Collagen Content and Decreases Lipid Content, Inflammation, Metalloproteinases, and Cell Death in Human Carotid Plaques. Implications for Plaque Stabilization. Circulation. 2001, 103, 926. 54. Kadoglou N P, Sailer N, Moumtzouoglou A, et al. Aggressive lipid-lowering is more effective than moderate lipid-lowering treatment in carotid plaque stabilization. 2009 Vascular Annual Meeting; June 11, 2009; Denver, CO. Ä‰ÂÒ Á‡ ÍÓÂÒÔÓÌ‰ÂÌˆËﬂ: ‰Óˆ. ‰- Ö‚„ÂÌËﬂ Ç‡ÒËÎÂ‚‡, ‰.Ï. äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, ìåÅÄã ◊ñ‡Ëˆ‡ âÓ‡ÌÌ‡”, ëÓÙËﬂ i-mail: [email protected] ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 149 é·ÁÓ ÇÄêàÄÅàãçéëí çÄ ëöêÑÖóçÄíÄ óÖëíéíÄ: åÖíéÑ áÄ éñÖçäÄ çÄ ëöêÑÖóçÄíÄ ÄÇíéçéåçÄ êÖÉìãÄñàü èêà çÖÇêéãéÉàóçà áÄÅéãüÇÄçàü ë. å‡ÌÚ‡Ó‚‡, ç. ÑËÏËÚÓ‚, à. ÇÂÎ˜Â‚‡ çÂ‚ÓÎÓ„Ë˜Ì‡ ÍÎËÌËÍ‡, ëÔÂˆË‡ÎËÁË‡Ì‡ ·ÓÎÌËˆ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ Ë ÔÒËıË‡ÚËﬂ ◊ë‚. ç‡ÛÏ”, åÂ‰ËˆËÌÒÍË ÛÌË‚ÂÒËÚÂÚ - ëÓÙËﬂ SUMMARY HEART RATE VARIABILITY: A METHOD FOR ASSESSMENT OF CARDIAC AUTONOMIC REGULATION IN NEUROLOGICAL DISEASES S. Mantarova, N. Dos. Dimitrov, I. Velcheva Dept of Neurology, University Hospital of Neurology and Psychiatry “St. Naum”, Medical University- Sofia The heart rate variability (HRV) is a noninvasive and reliable method with high reproducibility, by which, through measuring the variations of the heart intervals in short-term or 24-hour records the sympathovagal balance at sinoatrial level is estimated. The standard clinical parameters of the method are measurements in the frequency and time domain. Recently HRV is examined not only at rest but also after applying of different stimuli: deep breathing, body tilting, mental tasks, ect). A number of studies have shown certain diagnostic value of HRV in patients with diabetic neuropathy, Parkinson disease, stroke and multiple sclerosis. Its evaluation contributes for detecting early asymptomatic cardiac autonomic dysfunctions and correlates with basic clinical symptoms of the diseases: the hemoglobin A1C in diabets mellitus, the severity of the movement disorders in Parkinson`s disease, the degree of fatigue in multiple sclerosis, the type of the seizures in epilepsy. The impairment of the HRV is associated with increased risk of cardiac rhythm disturbances and of sudden cardiac death. The changes in HRV are associated with impairment of the cerebral autonomic structures and disturbance of their descending projections and neurotransmitter systems. The elucidation of their diagnostic value in different neurological diseases needs further studies. KEY WORDS: cardiac autonomic regulation, heart rate variability, neurological diseases. êÖáûåÖ Ç‡Ë‡·ËÎÌÓÒÚÚ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ (Çëó) Â ÌÂËÌ‚‡ÁË‚ÂÌ Ë Ì‡‰ÂÊ‰ÂÌ ÏÂÚÓ‰ Ò ‚ËÒÓÍ‡ ÂÔÓ‰ÛÍÚË‚ÌÓÒÚ, ÔË ÍÓÈÚÓ ˜ÂÁ ËÁÏÂ‚‡ÌÂ Ì‡ ‚‡Ë‡ˆËËÚÂ Ì‡ Ò˙‰Â˜ÌËÚÂ ËÌÚÂ‚‡ÎË ÔË Í‡ÚÍÓÚ‡ÈÌË ËÎË ÔÓ‰˙ÎÊËÚÂÎÌË Á‡ÔËÒË ÒÂ ÓÔÂ‰ÂÎﬂ ÒËÏÔ‡ÚËÍÓ‚‡„‡ÎÌËﬂ ·‡Î‡ÌÒ Ì‡ ÒËÌÓ‡ÚË‡ÎÌÓ ÌË‚Ó. ëÚ‡Ì‰‡ÚÌËÚÂ ÍÎËÌË˜ÌË Ô‡‡ÏÂÚË Ì‡ ÏÂÚÓ‰‡ Ò‡ ËÁÏÂ‚‡ÌËﬂ ‚ ˜ÂÒÚÓÚÌ‡Ú‡ Ë ‚˙‚ ‚ÂÏÂ‚‡Ú‡ Ó·Î‡ÒÚ. ç‡ÔÓÒÎÂ‰˙Í Çëó ÒÂ ËÁÒÎÂ‰‚‡ ÌÂ Ò‡ÏÓ ‚ ÔÓÍÓÈ, ÌÓ Ë ÒÎÂ‰ ÔËÎ‡„‡ÌÂ Ì‡ ‡ÁÎË˜ÌË ÒÚËÏÛÎË (‰˙Î·ÓÍÓ ‰Ë¯‡ÌÂ, ÔÓ‚‰Ë„‡ÌÂ Ì‡ ÚﬂÎÓÚÓ ÔÓ‰ ÓÔÂ‰ÂÎÂÌ ˙„˙Î Ë ‰Û„Ë). ÉÓÎﬂÏ ·ÓÈ ÔÓÛ˜‚‡ÌËﬂ Ò‡ ÔÓÍ‡Á‡ÎË ‰Ë‡„ÌÓÒÚË˜Ì‡Ú‡ ÒÚÓÈÌÓÒÚ Ì‡ Çëó ÔË ·ÓÎÌË Ò ‰Ë‡·ÂÚÌ‡ ÌÂ‚ÓÔ‡ÚËﬂ, Ô‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ÏÓÁ˙˜ÌË ËÌÒÛÎÚË Ë ÏÌÓÊÂÒÚ‚ÂÌ‡ ÒÍÎÂÓÁ‡. çÂÈÌÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ ‰ÓÔËÌ‡Òﬂ Á‡ ÓÚÍË‚‡ÌÂ Ì‡ ‡ÌÌ‡ ‡ÒËÏÔÚÓÏÌ‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏÌ‡ ‰ËÒÙÛÌÍˆËﬂ Ë ÍÓÂÎË‡ Ò ÓÒÌÓ‚ÌË ÍÎËÌË˜ÌË ÔÓﬂ‚Ë Ì‡ Á‡·ÓÎﬂ‚‡ÌËﬂÚ‡: ÌË‚Ó Ì‡ ıÂÏÓ„ÎÓ·ËÌ A1C ÔË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ, ÚÂÊÂÒÚ Ì‡ ‰‚Ë„‡ÚÂÎÌËÚÂ Ì‡Û¯ÂÌËﬂ ÔË Ô‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ‚Ë‰ Ì‡ ÔËÒÚ˙ÔËÚÂ ÔË ÂÔËÎÂÔÒËﬂ, ÒËÌ‰ÓÏ Ì‡ ÛÏÓ‡ ÔË ÏÌÓÊÂÒÚ‚ÂÌ‡ ÒÍÎÂÓÁ‡. ç‡ÎË˜ËÂÚÓ ËÏ Â Ò‚˙Á‡ÌÓ Ò ÔÓ‚Ë¯ÂÌ ËÒÍ ÓÚ Ò˙‰Â˜ÌË ËÚ˙ÏÌË Ì‡Û¯ÂÌËﬂ Ë ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. èÓÏÂÌËÚÂ ‚˙‚ Çëó Ò‡ Ò‚˙Á‡Ì‡ Ò Û‚ÂÊ‰‡ÌÂ Ì‡ ÏÓÁ˙˜ÌËÚÂ ‡‚ÚÓÌÓÏÌË ÒÚÛÍÚÛË Ë Ì‡Û¯‡‚‡ÌÂ Ì‡ ÚÂıÌËÚÂ ‰ÂÒˆÂÌ‰ÂÌÚÌË ÔÓÂÍˆËË Ë ÙÛÌÍˆËÓÌ‡ÎÌË ‚˙ÁÍË. Ç „ÂÌÂÁ‡Ú‡ ËÏ Û˜‡ÒÚ‚‡Ú Ë Ì‡Û¯ÂÌËﬂ ‚ ËÏÛÌÌ‡Ú‡ Ë ÌÂ‚ÓÚ‡ÌÒÏËÚÂÌËÚÂ ÒËÒÚÂÏË. àÁﬂÒÌﬂ‚‡ÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÒÚË˜Ì‡Ú‡ ËÏ ÒÚÓÈÌÓÒÚ ÔË ÓÚ‰ÂÎÌËÚÂ ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ Ì‡Î‡„‡ ÔÓ‚ÂÊ‰‡ÌÂ Ì‡ ÔÓ-Ì‡Ú‡Ú˙¯ÌË ËÁÒÎÂ‰‚‡ÌËﬂ. äãûóéÇà Ñìåà: ‚‡Ë‡·ËÎÌÓÒÚ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡, ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ, Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏÌ‡ Â„ÛÎ‡ˆËﬂ. ë˙‰Â˜ÌËﬂÚ ËÚ˙Ï ÔÓ‰ÎÂÊË Ì‡ ÒËÏÔ‡ÚËÍÓ‚ Ë Ô‡‡ÒËÏÔ‡ÚËÍÓ‚ ÍÓÌÚÓÎ ÏÓ‰ÛÎË‡Ì ÓÚ ˆÂÌÚ‡ÎÌË Ë ÔÂËÙÂÌË ‡‚ÚÓÌÓÏÌË ÒÚÛÍÚÛË (1). èÂÁ ÔÓÒÎÂ‰ÌËÚÂ „Ó‰ËÌË Á‡ ‰Ë‡„ÌÓÒÚËˆË‡ÌÂ Ì‡ Ò˙‰Â˜ÌËÚÂ ‡‚ÚÓÌÓÏÌË ‰ËÒÙÛÌÍˆËË ÒÂ ÔËÎ‡„‡Ú ÌÂËÌ‚‡ÁË‚ÌË ËÁÒÎÂ‰‚‡ÌËﬂ, ·‡ÁË‡ÌË Ì‡ ‡Ì‡ÎËÁ‡ Ì‡ ÓÒÌÓ‚ÌË ÙËÁËÓÎÓ„Ë˜ÌË Ô‡‡ÏÂÚË. í‡ÍË‚‡ Ò‡ ‚‡Ë‡·ËÎÌÓÒÚ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ (Çëó), ·‡ÓÂÙÎÂÍÒÌ‡ ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ, ‰˙ÎÊËÌ‡ Ì‡ QT ËÌÚÂ‚‡Î‡, ÍÓÒÒÔÂÍÚ‡ÎÂÌ ‡Ì‡ÎËÁ Ë ‰. éˆÂÌÍ‡Ú‡ Ì‡ Çëó Â ·˙Á, ÌÂËÌ‚‡ÁË‚ÂÌ, ÎÂÒÌÓ ‰ÓÒÚ˙ÔÂÌ Ë Ì‡‰ÂÊ‰ÂÌ ÏÂÚÓ‰ Ò ‚ËÒÓÍ‡ ÂÔÓ‰ÛÍÚË‚ÌÓÒÚ, ˜ÂÁ ÍÓÈÚÓ ÏÓÊÂ ‰‡ ÒÂ ÓÔÂ‰ÂÎË ÒËÏÔ‡ÚËÍÓ‚‡„‡ÎÌËﬂ ·‡Î‡ÌÒ Ì‡ ÒËÌÓ‡ÚË‡ÎÌÓ ÌË‚Ó (60). íÓÈ ÒÂ ËÁÔÓÎÁ‚‡ ÛÚËÌÌÓ Í‡ÍÚÓ ‚ Í‡‰ËÓÎÓ„Ë˜Ì‡Ú‡ Ô‡ÍÚËÍ‡, Ú‡Í‡ Ë ÔË Â‰Ëˆ‡ ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ ÔË ÍÓËÚÓ ÒÂ Ì‡·Î˛‰‡‚‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏÌ‡ ‰ËÒÙÛÌÍˆËﬂ – ‰Ë‡·ÂÚÌ‡ ÔÓÎËÌÂ‚ÓÔ‡ÚËﬂ, ÂÔËÎÂÔÒËﬂ, Ô‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, ÏÓÁ˙˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ Ë ‰. óÂÒÚÓ ÛÒÚ‡ÌÓ‚ÂÌËÚÂ ÌÛ¯ÂÌËﬂ ÍÓÂÎË‡Ú Ò ÚÂÊÂÒÚÚ‡ Ë ÔÓ„ÌÓÁ‡Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (1). ÄÌ‡ÎËÁ˙Ú Ì‡ Çëó ÒÂ ÓÒÌÓ‚‡‚‡ Ì‡ ÒÂËﬂ ÓÚ ËÁÏÂ‚‡ÌËﬂ Ì‡ ‚‡Ë‡ˆËËÚÂ ‚ Ò˙‰Â˜ÌËÚÂ ËÌÚÂ‚‡ÎË Ò˙Ò ÒËÌÛÒÓ‚ ÔÓËÁıÓ‰, ÍÓËÚÓ ÒÂ Ó·ÓÁÌ‡˜‡‚‡Ú Í‡ÚÓ NN ËÌÚÂ‚‡ÎË. åÓ„‡Ú ‰‡ ÒÂ ËÁÔÓÎÁ‚‡Ú ÔÓ‰˙ÎÊËÚÂÎÌË 24 ˜‡ÒÓ‚Ë ıÓÎÚÂ Á‡ÔËÒË ËÎË ÔÓ – Í˙ÒË ÂÎÂÍÚÓÍ‡‰ËÓ„‡ÙÒÍË Á‡ÔËÒË ‚ ‡ÏÍËÚÂ ÓÚ 0.5 ‰Ó 5 ÏËÌÛÚË, ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ˆÂÎËÚÂ Ì‡ ËÁÒÎÂ‰‚‡ÌÂÚÓ (60). ê‡ÁÎË˜ÌË ÙËÁËÓÎÓ„Ë˜ÌË Ù‡ÍÚÓË Í‡ÚÓ ÔÓÎ, ‚˙Á‡ÒÚ, ÚÂÎÂÒÌÓ ÚÂ„ÎÓ, ÂÚÌÓÒ, ‰Ë¯‡ÌÂ, ÙËÁË˜ÂÒÍ‡ ‡ÍÚË‚ÌÓÒÚ, ‚ÂÏÂ ÓÚ ‰ÂÌÓÌÓ˘ËÂÚÓ, ÔÓÎÓÊÂÌËÂ Ì‡ ÚﬂÎÓÚÓ, ÔËÂÏ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚË, ÂÏÓˆËË Ë ‰Û„Ë ÏÓ„‡Ú ‰‡ ÓÍ‡Á‚‡Ú ‚ÎËﬂÌËÂ ‚˙ıÛ ËÁÒÎÂ‰‚‡ÌËÚÂ Ô‡‡ÏÂÚË(1,10). Çëó Â ÔÓ- ÌËÒÍ‡ ÔË ÊÂÌË, ÔË ÎËˆ‡ Ò Ì‡‰ÌÓÏÂÌÓ ÚÂ„ÎÓ ËÎË ‚ Ì‡ÔÂ‰Ì‡Î‡ ‚˙Á‡ÒÚ (10, 57). èÂÁ 1996 í‡ÒÍ Force of European Society of Cardiology Ë North American Society of Pacing and Electrophysiology Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ÔËÂı‡ ÒÚ‡Ì‰‡ÚË Á‡ ËÁÏÂ‚‡ÌÂ, ËÌÚÂÔÂÚ‡ˆËﬂ Ë ÍÎËÌË˜ÌÓ ÔËÎÓÊÂÌËÂ Ì‡ Çëó. ëÚ‡Ì‰‡ÚÌËÚÂ ÍÎËÌË˜ÌË Ô‡‡ÏÂÚË Ò‡ ËÁÏÂ‚‡ÌËﬂ ‚ ˜ÂÒÚÓÚÌ‡Ú‡ Ë ‚˙‚ ‚ÂÏÂ‚‡Ú‡ Ó·Î‡ÒÚ, Í‡ÍÚÓ Ë „ÂÓÏÂÚË˜ÌË ÔÓÍ‡Á‡ÚÂÎË (61). ç‡ Ú‡·ÎËˆ‡ 1. Ò‡ ÔÂ‰ÒÚ‡‚ÂÌË Ì‡È – ˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚÂ ‚ÂÏÂ‚Ë Ë ˜ÂÒÚÓÚÌË Ô‡‡ÏÂÚË Ë ÚﬂıÌ‡Ú‡ ı‡‡ÍÚÂËÒÚËÍ‡. ëÔÂÍÚ‡ÎÌËﬂÚ ‡Ì‡ÎËÁ Ì‡ ˜ÂÒÚÓÚÌËÚÂ Ô‡‡ÏÂÚË ÒÂ ÓÒ˙˘ÂÒÚ‚ﬂ‚‡ ˜ÂÁ ÌÂÔ‡‡ÏÂÚË˜ÌË – ·˙Á‡ Ú‡ÌÒÙÓÏ‡ˆËﬂ Ì‡ îÛËÂ Ë Ô‡‡ÏÂÚË˜ÌË ÏÂÚÓ‰Ë - ‡‚ÚÓÂ„ÂÒË‚ÂÌ ‡Ì‡ÎËÁ. è‡‡ÏÂÚËÚÂ ÓÚ ˜ÂÒÚÓÚÌ‡Ú‡ Ó·Î‡ÒÚ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÍÎ‡ÒËÙËˆË‡ÌË ‚ ˜ÂÚËË Í‡ÚÂ„ÓËË ÛÎÚ‡ÌËÒÍË (UVL), ÏÌÓ„Ó ÌËÒÍË (VLF), ÌËÒÍË (LF) Ë ‚ËÒÓÍË (HF) ˜ÂÒÚÓÚË. (í‡·ÎËˆ‡ 1). èË Í‡ÚÍÓÚ‡ÈÌËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ ÒÂ ËÁÔÓÎÁ‚‡Ú VLF, LF Ë HF, ‰ÓÍ‡ÚÓ ÔË ‰˙Î„ÓÚ‡ÈÌËÚÂ ÒÂ ‚ÍÎ˛˜‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ UVL. àÁ˜ËÒÎﬂ‚‡Ú ÒÂ Ò ÔÓÏÓ˘Ú‡ Ì‡ ‡ÁÎË˜ÌË ÍÓÏÔ˛Ú˙ÌË ÔÓ„‡ÏË (îË„Û‡ 1). LF Ë HF ÏÓ„‡Ú ‰‡ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ ‚ ‡·ÒÓÎ˛ÚÌË ËÎË ‚ ÌÓÏ‡ÎËÁË‡ÌË Â‰ËÌËˆË. èÓÒÎÂ‰ÌËÚÂ Ò‡ ÔÓÎÂÁÌË ÔË ÓˆÂÌÍ‡ ‚ÎËﬂÌËÂÚÓ Ì‡ ‡ÁÎË˜ÌË ÒÚËÏÛÎË ËÎË ÔË Ò‡‚ÌËÚÂÎÂÌ ‡Ì‡ÎËÁ Ì‡ ‰‡ÌÌË ÓÚ Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ ÒÂ Ì‡·Î˛‰‡‚‡ „ÓÎﬂÏ‡ ‡ÁÎËÍ‡ ‚ Ó·˘ËÚÂ ÏÓ˘ÌÓÒÚË Ì‡ ÒÔÂÍÚ˙‡. ÇÒÂ ÔÓ – ˜ÂÒÚÓ ÒÂ ËÁÒÎÂ‰‚‡ Çëó ÌÂ Ò‡ÏÓ ‚ ÔÓÍÓÈ, ÌÓ Ë ÒÎÂ‰ ÔËÎ‡„‡ÌÂ Ì‡ ‡ÁÎË˜ÌË ÒÚËÏÛÎË – ‰˙Î·ÓÍÓ ‰Ë¯‡ÌÂ, ÔÓ‚‰Ë„‡ÌÂ Ì‡ ÚﬂÎÓÚÓ ÔÓ‰ ÓÔÂ‰ÂÎÂÌ ˙„˙Î, ÔË ÙËÁË˜ÂÒÍÓ ÛÒËÎËÂ, ÒÎÛıÓ‚Ë Ë ÁËÚÂÎÌË ÒÚËÏÛÎË, ÏÂÌÚ‡ÎÌË Á‡‰‡˜Ë, ÔË Ù‡Ï‡ÍÓÎÓ„Ë˜ÌÓ ‚˙Á‰ÂÈÒÚ‚ËÂ ËÎË ‚ ÍÓÏ·ËÌ‡ˆËﬂ Ò˙Ò ÒˆËÌÚË„‡ÙÒÍË ÏÂÚÓ‰Ë (60). ëÔÂÍÚ‡ÎÌËﬂÚ ‡Ì‡ÎËÁ Ì‡ Çëó Â Ì‡‰ÂÊ‰ÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌÍ‡ Ì‡ ‡‚ÚÓÌÓÏÌ‡Ú‡ ‰Ë‡·ÂÚÌ‡ ‰ËÒÙÛÌÍˆËﬂ (49). ë ÔËÎ‡„‡ÌÂÚÓ ÏÛ ÔË ‰Âˆ‡ ·ÓÎÌË ÓÚ ‰Ë‡·ÂÚ ÏÓÊÂ ‰‡ ÓÚÍËﬂÚ ‡ÌÌË ‡ÒËÏÔÚÓÏÌË ‡‚ÚÓÌÓÏÌË Ì‡Û¯ÂÌËﬂ, ÍÓËÚÓ Ò‡ ÔÂ‰ËÏÌÓ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë Ë Á‡‚ËÒﬂÚ ÓÚ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ‡‰ÂÍ‚‡ÚÌËﬂ ÏÂÚ‡·ÓÎËÚÂÌ ÍÓÌÚÓÎ (9). ëÌËÊ‡‚‡ÌÂÚÓ Ì‡ Ô‡‡ÏÂÚËÚÂ Ì‡ Çëó ÏÓ- 150 ÊÂ ‰‡ ÔÂ‰¯ÂÒÚ‚‡ ÍÎËÌË˜Ì‡Ú‡ ËÁﬂ‚‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ‡‚ÚÓÌÓÏÌ‡ ÌÂ‚ÓÔ‡ÚËﬂ, ÍÓﬂÚÓ Â ÚﬂÒÌÓ Ò‚˙Á‡Ì‡ Ò ËÒÍ ÓÚ ÏËÓÍ‡‰Ì‡ ËÒıÂÏËﬂ, ‡ËÚÏËË Ë ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú (32, 43). èË ·ÓÎÌË Ò ‰Ë‡·eÚ ÚËÔ 1 Ë ÓÒÓ·ÂÌÓ ÔË ÚÂÁË Ò˙Ò Ò˙Ô˙ÚÒÚ‚‡˘‡ ÌÂÙÓÔ‡ÚËﬂ Â ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â Ì‡Û¯ÂÌËÂÚÓ ‚˙‚ Çëó Â ÌÂÁ‡‚ËÒËÏ ËÒÍÓ‚ Ù‡ÍÚÓ Á‡ Ò˙‰Â˜ÌÓÒ˙‰Ó‚Ë Á‡·ÓÎﬂ‚‡ÌËﬂ Ë ÒÏ˙ÚÌÓÒÚ ÓÚ Úﬂı (5, 41). èÓÒÔÂÍÚË‚ÌÓ ÔÓÛ˜‚‡ÌÂ Â ÔÓÍ‡Á‡ÎÓ Ò˙˘Ó ˜ÂÒÚ‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏÌ‡ ‰ËÒÙÛÌÍˆËﬂ (ëÄÑ) ÔË ·ÓÎÌË Ò ‰Ë‡·ÂÚ 2 ÚËÔ, ÍÓËÚÓ Ò‡ ÔÓ – ‚˙Á‡ÒÚÌË, Ò ÔÓ – „ÓÎﬂÏ‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ËÏ‡Ú ıËÔÂÚÓÌËﬂ, ÂÚËÌÓÔ‡ÚËﬂ Ë ÏËÍÓ‡Î·ÛÏËÌÛËﬂ. èË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ 2 ÚËÔ ëÄÑ ÍÓÂÎË‡ Ò ÌË‚ÓÚÓ Ì‡ ıÂÏÓ„ÎÓ·ËÌ A1C (33). èË Ò˙˘Ëﬂ ÍÓÌÚËÌ„ÂÌÚ ·ÓÎÌË Ì‡ÏÂÂÌ‡Ú‡ Á‡‚ËÒËÏÓÒÚ ÏÂÊ‰Û ÒÌËÊÂÌË ÒÚÓÈÌÓÒÚË Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚÂ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡ Ë Á‡‰Â·ÂÎﬂ‚‡ÌÂÚÓ Ì‡ ËÌÚËÏ‡-ÏÂ‰Ëﬂ ÍÓÏÔÎÂÍÒ‡ ‚ Ó·Î‡ÒÚÚ‡ Ì‡ Í‡ÓÚË‰Ì‡Ú‡ ·ËÙÛÍ‡ˆËﬂ ÔÓ‰ÒÍ‡Á‚‡ ‚˙ÁÍ‡Ú‡ Ì‡ Ì‡Û¯ÂÌ‡Ú‡ Çëó Ò Ì‡ÎË˜ËÂÚÓ Ë ÔÓ„ÂÒËﬂÚ‡ Ì‡ Í‡ÓÚË‰Ì‡ ‡ÚÂÓÒÍÎÂÓÁ‡ (20). èË ·ÓÎÌË Ò èÅ ˜ÂÒÚÓ ÒÂ Ì‡·Î˛‰‡‚‡ Ò˙‰Â˜Ì‡ ‡‚ÚÓÌÓÏÌ‡ ‰ËÒÙÛÌÍˆËﬂ. Mihci E. et al. (47) ÔË 24 ˜‡ÒÓ‚Ó ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ‚‡Ë‡·ËÎÌÓÒÚÚ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Â„ËÒÚË‡Ú ÔÓ – ÌËÒÍË ÒÚÓÈÌÓÒÚË, Í‡ÍÚÓ Á‡ ‚ÂÏÂ‚ËÚÂ, Ú‡Í‡ Ë Á‡ ˜ÂÒÚÓÚÌËÚÂ Ô‡‡ÏÂÚË. Ç ÍÎËÌË˜Ì‡Ú‡ Ô‡ÍÚËÍ‡ Í‡‰ËÓ‚‡ÒÍÛÎ‡ÌËÚÂ ÚÂÒÚÓ‚Â ÏÓ„‡Ú ‰‡ ÔÓ‰ÔÓÏÓ„Ì‡Ú ‰ËÙÂÂÌˆË‡ÎÌ‡Ú‡ ‰Ë‡„ÌÓÁ‡ Ì‡ Ô‡ÍËÌÒÓÌÓ‚ËÚÂ ÒËÌ‰ÓÏË. ç‡Ï‡ÎÂÌ‡ Çëó ÒÂ Ì‡·Î˛‰‡‚‡ ÔË ·ÓÎÌË Ò ÏÌÓÊÂÒÚ‚ÂÌ‡ ÒËÒÚÂÏÌ‡ ‡ÚÓÙËﬂ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÚﬂıÌ‡Ú‡ ‚˙Á‡ÒÚ Ë ÓÚ ‰‡‚ÌÓÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. èË èÅ ÔÓ‰Ó·Ì‡ ÍÓÌÒÚÂÎ‡ˆËﬂ ÒÂ ÒÂ˘‡ Ò‡ÏÓ ‚ Ì‡ÔÂ‰Ì‡Î‡ ‚˙Á‡ÒÚ Ë ÔË „ÓÎﬂÏ‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (29). èË ÌÓ‚ÓÓÚÍËÚË ÌÂÎÂÍÛ‚‡ÌË ·ÓÎÌË Ò èÅ Â Ì‡ÎËˆÂ Î‡ÚÂÌÚÌ‡ Ò˙‰Â˜Ì‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‰ËÒÙÛÌÍˆËﬂ, ÍÓﬂÚÓ Ì‡È – ‚ÂÓﬂÚÌÓ ÒÂ ‰˙ÎÊË Ì‡ Û‚ÂÊ‰‡ÌÂ Ì‡ ÒËÌÛÒÓ‚Ëﬂ ‚˙ÁÂÎ. è‡- í‡·ÎËˆ‡ 1. è‡‡ÏÂÚË ÓÚ ‚ÂÏÂ‚‡Ú‡ Ë ˜ÂÒÚÓÚÌ‡Ú‡ Ó·Î‡ÒÚ è‡‡ÏÂÚË ÓÚ ‚ÂÏÂ‚‡Ú‡ Ó·Î‡ÒÚ SDNN SDANN Ö‰ËÌËˆË ï‡‡ÍÚÂËÒÚËÍ‡ ms ms SD(ËÎËSDSD) ms RMSSD ms Pnn50 % è‡‡ÏÂÚË ÓÚ ˜ÂÒÚÓÚÌ‡Ú‡ Ó·Î‡ÒÚ é·˘‡ ÏÓ˘ÌÓÒÚ Ö‰ËÌËˆË ëÚ‡Ì‰‡ÚÌÓ ÓÚÍÎÓÌÂÌËÂ Ì‡ ‚ÒË˜ÍË NN ËÌÚÂ‚‡ÎË,Ó·˘ ÔÓÍ‡Á‡ÚÂÎ, ‚ÎËﬂ ÒÂ ÓÚ ˆËÍ‡‰ÌËÚÂ ËÚÏË. ëÚ‡Ì‰‡ÚÌÓ ÓÚÍÎÓÌÂÌËÂ Ì‡ ÒÂ‰ÌËÚÂ NN ËÌÚÂ‚‡ÎË ÓÚ ‚ÒÂÍË 5 ÏËÌÛÚÂÌ ÒÂ„ÏÂÌÚ ÓÚ Á‡ÔËÒ‡, ˜Û‚ÒÚ‚ËÚÂÎÂÌ ÔÓÍ‡Á‡ÚÂÎ Á‡ ÔÓ – ÌËÒÍËÚÂ ˜ÂÒÚÓÚË, Á‡‚ËÒÂ˘ ÓÚ ÙËÁË˜ÂÒÍ‡Ú‡ ‡ÍÚË‚ÌÓÒÚ, ˆËÍ‡‰ÌËÚÂ ËÚÏË, ÔÓÏﬂÌ‡ ‚ ÔÓÁËˆËﬂÚ‡ Ì‡ ÚﬂÎÓÚÓ. ëÚ‡Ì‰‡ÚÌÓ ÓÚÍÎÓÌÂÌËÂ Ì‡ ‡ÁÎËÍËÚÂ ÏÂÊ‰Û Ò˙ÒÂ‰ÌË NN ËÌÚÂ‚‡ÎË, ÓÚ‡Áﬂ‚‡˘ ‰ÂÌÓÌÓ˘ÌËÚÂ ÔÓÏÂÌËÚÂ ‚ HRV. ëÂ‰ÌÓ Í‚‡‰‡ÚË˜ÌÓ Ì‡ ‡ÁÎËÍËÚÂ ÏÂÊ‰Û ‚ÒÂÍË ‰‚‡ ÔÓÒÎÂ‰Ó‚‡ÚÂÎÌË NN ËÌÚÂ‚‡ÎË, Á‡ Í‡ÚÍÓ‚ÂÏÂÌÌË ÔÓÏÂÌË ‚ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Ë ÌÂÁ‡‚ËÒËÏ ÓÚ ˆËÍ˙Î‡ ‰ÂÌ/ÌÓ˘. èÓˆÂÌÚ‡ Ì‡ ÔÓÒÎÂ‰Ó‚‡ÚÂÎÌËÚÂ ËÌÚÂ‚‡ÎË, ÍÓËÚÓ ÒÂ ‡ÁÎË˜‡‚‡Ú Ò ÔÓ‚Â˜Â ÓÚ 50 ms, Í‡ÚÍÓ‚ÂÏÂÌÌË ÔÓÏÂÌË ‚ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Ë ÌÂÁ‡‚ËÒËÏ ÓÚ ˆËÍ˙Î‡ ‰ÂÌ/ÌÓ˘. èÓ – ÌÂÒÚ‡·ËÎÂÌ ÔÓÍ‡Á‡ÚÂÎ ÓÚ RMSSD. ï‡‡ÍÚÂËÒÚËÍ‡ óÂÒÚÓÚÂÌ ‰Ë‡Ô‡ÁÓÌ ms2 UVL ms2 VLF ms2 LF ms2 HF ms2 LF/HF Ò˙ÓÚÌÓ¯ÂÌËÂ Ç‡Ë‡ˆËﬂ Ì‡ ‚ÒË˜ÍË NN ËÌÚÂ‚‡ÎË, Ó·˘‡ ‚‡Ë‡·ËÎÌÓÒÚ, ÍÓÂÒÔÓÌ‰Ë‡ Ò˙Ò ÒÛÏ‡Ú‡ ÓÚ ‚ÒË˜ÍË ÒÔÂÍÚ‡ÎÌË ÍÓÏÔÓÌÂÌÚË. ìÎÚ‡ÌËÒÍË ˜ÂÒÚÓÚË, ÓÚ‡Áﬂ‚‡ ‚ÎËﬂÌËÂÚÓ Ì‡ ˆËÍ‡‰ÌËÚÂ Ë ÌÂ‚ÓÂÌ‰ÓÍËÌÌË ËÚÏË. åÌÓ„Ó ÌËÒÍË ˜ÂÒÚÓÚË., Ò‚˙Á‡Ì Ò ‰˙Î„ÓÚ‡ÈÌË ÔÓÏÂÌË, Á‡‚ËÒË ÓÚ ÙËÁË˜ÂÒÍ‡Ú‡ ‡ÍÚË‚ÌÓÒÚ, Ï‡ÍÂ Á‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ. çËÒÍË ˜ÂÒÚÓÚË, ËÁ‡Á Í‡ÍÚÓ Ì‡ ÒËÏÔ‡ÚËÍÓ‚Ë, Ú‡Í‡ Ë Ì‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë ‚ÎËﬂÌËﬂ, Í‡ÚÓ ‚ ÌÓÏ‡ÎËÁË‡ÌË Â‰ËÌËˆË ÓÚ‡Áﬂ‚‡ ÒËÏÔ‡ÚËÍÓ‚‡Ú‡ ‡ÍÚË‚ÌÓÒÚ. ÇËÒÓÍË ˜ÂÒÚÓÚË, ÂÒÔË‡ÚÓÌÓ Á‡‚ËÒËÏ Ï‡ÍÂ Á‡ ‚‡„ÛÒÓ‚ËÚÂ ‚ÎËﬂÌËﬂ, ÓÔÂ‰ÂÎﬂ ÒÂ ÓÚ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ‰Ë¯‡ÌÂÚÓ. ë˙ÓÚÌÓ¯ÂÌËÂ Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚ Í˙Ï ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËÚÂ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡, ÓÚ‡Áﬂ‚‡ ÒËÏÔ‡ÚËÍÓ‚‡„‡ÎÌËﬂ ·‡Î‡ÌÒ. < 0.4 Hz <0.003 Hz <0.003-0.4 Hz 0.04 – 0.15 Hz 0.15 – 0.4 Hz ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 151 îË„. 1 èÓ„‡Ï‡ Á‡ ËÁ˜ËÒÎﬂ‚‡ÌÂ Ì‡ ÒÔÂÍÚ‡ÎÌËÚÂ ‰‡ÌÌË Ì‡ Çëó ‡ÒËÏÔ‡ÚËÍÓ‚‡Ú‡ Í‡‰ËÓ‚‡ÒÍÛÎ‡Ì‡ Â„ÛÎ‡ˆËﬂ Ò˙˘Ó ÌÂ Â ÔÓ˘‡‰ÂÌ‡ ÔË ÚÓÁË ÍÓÌÚËÌ„ÂÌÚ ·ÓÎÌË; ÔÓÏÂÌËÚÂ Ò‡ Ì‡È – ÒËÎÌÓ ËÁ‡ÁÂÌË ÔÂÁ ÌÓ˘ÌËÚÂ ˜‡ÒÓ‚Â (55). èË Ô‡‡ÎÂÎÌÓ ÔÓ‚ÂÊ‰‡ÌÂ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ÒˆËÌÚË„‡ÙËﬂ Ò I123 MIBG Ò‚˙Á‚‡ÌÂÚÓ Ì‡ ËÁÓÚÓÔ‡ ÔÓ„ÂÒË‚ÌÓ Ì‡Ï‡Îﬂ‚‡ Ò Ì‡‡ÒÚ‚‡ÌÂ Ì‡ ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (48). è‡‡ÏÂÚËÚÂ Ì‡ Çëó ÍÓÂÎË‡Ú Ò ÚÂÊÂÒÚÚ‡ Ì‡ ‰‚Ë„‡ÚÂÎÌËÚÂ Ì‡Û¯ÂÌËﬂ ÔË èÅ , ÌÓ ÌÂ ÔÓÍ‡Á‚‡Ú Á‡‚ËÒËÏÓÒÚ ÓÚ ÔÓﬂ‚ËÚÂ Ì‡ ÚÂÏÓ Ë Ë„Ë‰ÌÓÒÚ. èË ·ÓÎÌËÚÂ Ò ÚÂÊ˙Í ‰‚Ë„‡ÚÂÎÂÌ ‰ÂÙËˆËÚ ÒÂ Ì‡·Î˛‰‡‚‡ ÒÌËÊÂÌËÂ Ì‡ ÒÚÓÈÌÓÒÚËÚÂ Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚÂ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡, ‡ ÔÓ- ÚÂÊÍÓ ËÁ‡ÁÂÌ‡ ıËÔÓÍËÌÂÁËﬂ Â Ò‚˙Á‡Ì‡ Ò ÔÓ- ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËÚÂ ÍÓÏÔÓÌÂÌÚË (22). éÔÂ‰ÂÎﬂÌÂÚÓ Ì‡ ‰ÓÏËÌË‡˘‡Ú‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚‡ ËÎË ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ ÔÂÁ ‡ÁÎË˜ÌË Ù‡ÁË Ì‡ Ò˙Ìﬂ Â ˜Û‚ÒÚ‚ËÚÂÎÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌÍ‡ Ì‡ ÌÓ˘ÌË ëÄÑ ÔË èÅ. êÂ‡ÍÚË‚ÌÓÒÚÚ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ ÔË ‰‚ËÊÂÌËÂ Ì‡ ÚﬂÎÓÚÓ ÔË ÚÂÁË ·ÓÎÌË Â ÒÌËÊÂÌ‡ ÒÔﬂÏÓ Á‰‡‚ËË ÎËˆ‡, ÓÒÓ·ÂÌÓ ÔÂÁ êÖå ÒÚ‡‰Ëﬂ Ì‡ Ò˙Ìﬂ. èË Úﬂı Ò‡ Ì‡Ï‡ÎÂÌË ‚ËÒÓÍÓ˜ÂÒÚÓÚÌËÚÂ ÍÓÏÔÓÌÂÌÚË Í‡ÍÚÓ ÔË Ò˙·ÛÊ‰‡ÌÂ, Ú‡Í‡ Ë ÔÂÁ ÌÂ- êÖå Ò˙Ìﬂ; Ì‡Û¯ÂÌ Â Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ëﬂ Í‡‰ËÓ‚‡ÒÍÛÎ‡ÂÌ ÍÓÌÚÓÎ (31). èËÂÏ˙Ú Ì‡ ÎÂ‚Ó‰ÓÔ‡ ÏÂ‰ËÍ‡ÏÂÌÚË ÔË èÅ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ‚ÎÓ¯‡‚‡ Ì‡Û¯ÂÌËﬂ ‡‚ÚÓÌÓÏÂÌ ÍÓÌÚÓÎ Ì‡ ‡ÚÂË‡ÎÌÓÚÓ Ì‡Îﬂ„‡ÌÂ Ë Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ (8). Ç Ò‡‚ÌÂÌËÂ Ò ÌÂÎÂÍÛ‚‡ÌË ·ÓÎÌË Ò˙Ò ÒÎ‡·Ó ËÁ‡ÁÂÌ ‰‚Ë„‡ÚÂÎÂÌ ‰ÂÙËˆËÚ, LF Ë Ò˙ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF, ËÁÏÂÂÌË ÔÂÁ ‰ÌÂ‚ÌËÚÂ ˜‡ÒÓ‚Â, Ò‡ ÒÌËÊÂÌË ÔË ·ÓÎÌË Ò˙Ò ÒÂ‰Ì‡ ÚÂÊÂÒÚ Ì‡ èÅ Ë ÔËÂÏ Ì‡ ÎÂ‚Ó‰ÓÔ‡, Í‡ÍÚÓ Ë ÔË ÚÂÁË Ò ÛÒÎÓÊÌÂÌËﬂ ÓÚ ÎÂ˜ÂÌËÂÚÓ. Ç‡„ÛÒÓ‚ËÚÂ Ï‡ÍÂË - HF Ë pNN50 Ò‡ Ì‡Ï‡ÎÂÌË ÔÂÁ ÌÓ˘Ú‡ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ËÒÍËÌÂÁËË Ë ÍÓÂÎË‡Ú Ò ÏÂ‰ËÍ‡ÏÂÌÚÓÁÌÓ Ò‚˙Á‡ÌËÚÂ “off” ÔÂËÓ‰Ë, ÌÓ ÌÂ Ë Ò “on” ÔÂËÓ‰ËÚÂ (12). á‡ ‡ÁÎËÍ‡ ÓÚ ÎÂ‚Ó‰ÓÔ‡ ÏÂ‰ËÍ‡ÏÂÌÚËÚÂ, COåí – ËÌıË·ËÚÓËÚÂ (tolcapone) ÌÂ ÔÓ‚ÎËﬂ‚‡Ú Í‡‰ËÓ‚‡ÒÍÛÎ‡ÌËÚÂ ÙÛÌÍˆËË ÔË Ô‡ˆËÂÌÚË Ò èÅ (44). àÁÒÎÂ‰‚‡ÌÂÚÓ Ì‡ Äçë Ë Çëó ÔË ·ÓÎÌË Ò ÂÔËÎÂÔÒËﬂ ‰ÓÔËÌ‡Òﬂ Á‡ ÓÚÍË‚‡ÌÂ Ì‡ ‡ÁÎË˜ÌË ‡‚ÚÓÌÓÏÌË ÔÓﬂ‚Ë Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, Á‡ ÔÓ‰Ó·ﬂ‚‡ÌÂ Ì‡ ÌÂÈÌ‡Ú‡ ‰Ë‡„ÌÓÒÚËÍ‡ Ë Á‡ ËÁ·Ó‡ Ì‡ ÔÓ – ÂÙÂÍÚË‚Ì‡ ÚÂ‡ÔËﬂ. (18). ç‡Û¯ÂÌËﬂÚ‡ ‚ ‡‚ÚÓÌÓÏÌËﬂ Ò˙‰Â˜ÌÓÒ˙‰Ó‚ ÍÓÌÚÓÎ Ë„‡ﬂÚ ‚‡ÊÌ‡ ÓÎﬂ Á‡ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú ÔË ·ÓÎÌË ÓÚ ÂÔËÎÂÔÒËﬂ. íﬂ Ì‡ÒÚ˙Ô‚‡ Ì‡È – ˜ÂÒÚÓ ÌÓ˘ÂÏ, ÍÓ„‡ÚÓ ÔÓÏÂÌË ‚˙‚ Çëó Ò‡ ÔÓ – ÒËÎÌÓ ËÁ‡ÁÂÌË (51). èÓÛ˜‚‡ÌËﬂ Ì‡ ‡ÁÎË˜ÌË ‡‚ÚÓË ÔÓÍ‡Á‚‡Ú Á‡‚ËÒËÏÓÒÚ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ‡‚ÚÓÌÓÏÌ‡ ‰ËÒÙÛÌÍˆËﬂ Ë Ì‡ Çëó ÓÚ ÎÓÍ‡ÎËÁ‡ˆËﬂÚ‡ Ì‡ ÂÔËÎÂÔÚË˜ÌÓÚÓ Ó„ÌË˘Â Ì‡ ÂÎÂÍÚÓÂÌˆÂÙ‡ÎÓ„‡Ï‡Ú‡ Ë ÓÚ ‚Ë‰‡ Ì‡ ÔËÒÚ˙ÔËÚÂ. í‡Í‡ ÔË Ô‡ˆËÂÌÚË Ò ÙÓÌÚ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ÒÂ Ì‡·Î˛‰‡‚‡ Ì‡Ï‡ÎÂÌ‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ (26), ‰ÓÍ‡ÚÓ ÔË ÚÂÁË Ò „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌË˜ÌÓ – ÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË Ë Ò ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÔÓ‚Ë¯ÂÌ‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ (15). ëÌËÊÂÌ‡ Çëó, ÓÒÓ·ÂÌÓ ‚ ÌÓ˘ÌËÚÂ ˜‡ÒÓ‚Â, ÒÂ ÓÚÍË‚‡ ÔË Ô‡ˆËÂÌÚË Ò ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ. èÓÏÂÌËÚÂ ÔÂÓ·Î‡‰‡‚‡Ú ‚ ÒÎÛ˜‡ËÚÂ Ò ‰ÂÒÂÌ ÖÖÉ ÙÓÍÛÒ, Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ Ò˙‰Â˜Ì‡ ‡ËÚÏËﬂ Ë Ò‡ Ò‚˙Á‡ÌË Ò ‚ËÒÓÍ ËÒÍ ÓÚ ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. ç‡Û¯ÂÌËﬂÚ‡ ‚˙‚ Çëó ÔË ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ÌÂ Á‡‚ËÒﬂÚ ÓÚ ÂÙÂÍÚ‡ Ì‡ ÔÓ‚ÂÊ‰‡ÌÓÚÓ ÎÂ˜ÂÌËÂ (59). èË ÂÁËÒÚÂÌÚÌ‡ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚÓÁÌÓ ÎÂ˜ÂÌËÂ ÂÔËÎÂÔÒËﬂ Ì‡ÎË˜ËÂÚÓ ËÏ Â Ò‚˙Á‡ÌÓ Ë Ò ÌÂ·Î‡„ÓÔËﬂÚÌ‡ ÔÓ„ÌÓÁ‡ ÔË Â‚ÂÌÚÛ‡ÎÌ‡ ıËÛ„Ë˜Ì‡ Ì‡ÏÂÒ‡ (52). èË ‰Âˆ‡ Ò ÂÔËÎÂÔÒËﬂ ÌÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú Á‡‚ËÒËÏÓÒÚË ÏÂÊ‰Û ı‡‡ÍÚÂÌËÚÂ Á‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÖÖÉ ÔÓÏÂÌË Ë Çëó (4). èË ÚÂÁË Ò ÂÙ‡ÍÚÂÌ‡ „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔ- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÒËﬂ ÒÂ Ì‡·Î˛‰‡‚‡ Â‰ÛÍˆËﬂ Ì‡ Çëó ÔÂ‰ËÏÌÓ ‚˙‚ ‚ËÒÓÍÓ˜ÂÒÚÓÚÌ‡Ú‡ Ó·Î‡ÒÚ (25). èÓ‰Ó·ÌÓ Ì‡ ‚˙Á‡ÒÚÌËÚÂ, ÔË ‰Âˆ‡Ú‡ Ò Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ÔÓ ‚ÂÏÂ Ì‡ Ò˙Ì ÒÂ ÔÓﬂ‚ﬂ‚‡ ÚÂÌ‰ÂÌˆËﬂ Á‡ ÒÌËÊÂÌËÂ Ì‡ ÔÓÍ‡Á‡ÚÂÎËÚÂ Ì‡ Çëó Í‡ÍÚÓ ‚˙‚ ‚ÂÏÂ‚‡Ú‡, Ú‡Í‡ Ë ‚ ˜ÂÒÚÓÚÌ‡Ú‡ Ó·Î‡ÒÚ. íÓ Â Ì‡È- ‰Ó·Â ËÁ‡ÁÂÌÓ ‚ ÒÚ‡‰ËÈ 3 Ë 4 Ì‡ êÖå Ò˙Ìﬂ (17). ëÌËÊÂÌËÂÚÓ ÌÂ Á‡‚ËÒË ÓÚ Ì‡ÎË˜ËÂÚÓ ËÎË ÓÚÒ˙ÒÚ‚ËÂÚÓ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚÓÁÌÓ ÎÂ˜ÂÌËÂ. èË ‰Âˆ‡Ú‡ ·ÂÁ ÔÓ‚ÂÊ‰‡ÌÓ ‡ÌÚËÂÔËÎÂÔÚË˜ÌÓ ÎÂ˜ÂÌËÂ ÔÂÓ·Î‡‰‡‚‡ Ò˙˘Ó ÒÌËÊÂÌËÂ Ì‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚ËÚÂ ÍÓÏÔÓÌÂÌÚË Ì‡ Çëó (24). ÑÓÔÛÒÍ‡ÌÂÚÓ Ì‡ O. Hallioglu et al. (24), ˜Â ‡ÌÚËÂÔËÎÂÔÚË˜ÌÓÚÓ ÎÂ˜ÂÌËÂ ÏÓÊÂ ‰‡ ‰Ó‚Â‰Â ‰Ó ÔÓ‰Ó·ﬂ‚‡ÌÂ Ì‡ ‡‚ÚÓÌÓÏÌËÚÂ Ò˙‰Â˜ÌË ÙÛÌÍˆËË, ÒÂ ÔÓÚ‚˙Ê‰‡‚‡ ÓÚ ‰‡ÌÌËÚÂ Á‡ ·Î‡„ÓÔËﬂÚÂÌ ÂÙÂÍÚ ‚˙ıÛ Ì‡Û¯ÂÌ‡Ú‡ Çëó ÓÚ ÎÂ˜ÂÌËÂÚÓ Ò Ääíï ÔË ÒËÌ‰ÓÏ‡ Ì‡ West (27) Ë ÓÚ ÒÚËÏÛÎ‡ˆËﬂÚ‡ Ì‡ n. vagus ÔË Ô‡ˆËÂÌÚË Ò˙Ò ÒËÌ‰ÓÏ Ì‡ Lennox-Gastaut (35). éÚ ‰Û„‡ ÒÚ‡Ì‡, ÔÓ‰˙ÎÊËÚÂÎÌÓÚÓ ÎÂ˜ÂÌËÂ Ò ‡ÌÚËÂÔËÎÂÔÚË˜ÌË ÏÂ‰ËÍ‡ÏÂÌÚË, ÓÒÓ·ÂÌÓ Í‡·‡Ï‡ÁÂÔËÌ ÒÂ Ò‚˙Á‚‡ Ò ÔÓÌËÊÂÌ‡ Çëó ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò ÒÚÓÈÌÓÒÚËÚÂ È ÔË ÌÓ‚Ó‰Ë‡„ÌÓÒÚËˆË‡ÌË ÌÂÎÂÍÛ‚‡ÌË Ô‡ˆËÂÌÚË (50). èÓÒÚÂÔÂÌÌÓÚÓ ÓÚÌÂÏ‡ÌÂ Ì‡ ÚÓÁË ÏÂ‰ËÍ‡ÏÂÌÚ ÓÍ‡Á‚‡ ·Î‡„ÓÔËﬂÚÂÌ ÂÙÂÍÚ Í‡ÍÚÓ ‚˙ıÛ ÒËÏÔ‡ÚËÍÓ‚ËÚÂ, Ú‡Í‡ Ë ‚˙ıÛ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚ËÚÂ ÙÛÌÍˆËË (40). ÇÌÂÁ‡ÔÌÓÚÓ ÒÔË‡ÌÂ Ì‡ ÔËÎÓÊÂÌËÂÚÓ ÏÛ Ó·‡˜Â ‚Ó‰Ë ‰Ó ÔÓ‚Ë¯ÂÌ‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ ÔÓ ‚ÂÏÂ Ì‡ Ò˙Ì, ÍÓÂÚÓ ÔÂ‰‡ÁÔÓÎ‡„‡ Í˙Ï ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú (28). èË ·ÓÎÌËÚÂ Ò ÓÒÚ˙ ËÒıÂÏË˜ÂÌ ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ ÔÓÏÂÌË ‚˙‚ Çëó ÒÂ Ì‡·Î˛‰‡‚‡Ú Ì‡È-˜ÂÒÚÓ ÔË ÎÓÍ‡ÎËÁ‡ˆËﬂ Ì‡ ËÌÙ‡ÍÚ‡ ‚ ‰ﬂÒÌ‡Ú‡ ËÌÒÛÎ‡. íÂ ÒÂ ÔÓﬂ‚ﬂ‚ﬂÚ Ò˙Ò ÁÌ‡˜ËÏÓ ÒÌËÊÂÌË ÒÚÓÈÌÓÒÚË Ì‡ SDNN Ë Ì‡ rMSSD Ë ÔÓ‚ËÒÓÍË ÒÚÓÈÌÓÒÚË Ì‡ ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF. èË ÚÂÁË ·ÓÎÌË ÔÓ-˜ÂÒÚÓ Ì‡ÒÚ˙Ô‚‡Ú ËÚ˙ÏÌË Ì‡Û¯ÂÌËﬂ (11). èËÂÏ‡ ÒÂ, ˜Â ÔÓÏÂÌËÚÂ Ì‡ Çëó, ÓÒÓ·ÂÌÓ Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚÂ È ÍÓÏÔÓÌÂÚË, Ò‡ ÌÂÁ‡‚ËÒËÏ ÔÓ„ÌÓÒÚË˜ÂÌ Ù‡ÍÚÓ Á‡ ËÁıÓ‰‡ ÓÚ ÓÒÚ˙ ÚÂÊ˙Í ÏÓÁ˙˜ÂÌ ËÌÙ‡ÍÚ (21). éˆÂÌÍ‡Ú‡ ËÏ ÔÂ‰Ë Á‡ÔÓ˜‚‡ÌÂ Ì‡ Âı‡·ËÎËÚ‡ˆËﬂÚ‡ Ì‡ ÚÂÁË ·ÓÎÌË ÏÓÊÂ ‰‡ ‰‡‰Â ËÌÙÓÏ‡ˆËﬂ Á‡ Ó˜‡Í‚‡Ì‡Ú‡ ÒÚÂÔÂÌ Ì‡ ÙÛÌÍˆËÓÌ‡ÎÌÓ ‚˙ÁÒÚ‡ÌÓ‚ﬂ‚‡ÌÂ. íﬂ Â ÌÂ·Î‡„ÓÔËﬂÚÌ‡ ÔË ÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ SDNN (6). èÓÍ‡Á‡ÌÓ Â, ˜Â Çëó ÓÒÚ‡‚‡ ÁÌ‡˜ËÏÓ ÔÓ-ÌËÒÍ‡ ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò Á‰‡‚Ë ÎËˆ‡ Ë ÒÎÂ‰ ÔÂÏËÌ‡‚‡ÌÂ Ì‡ ÓÒÚ‡Ú‡ Ù‡Á‡ Ì‡ ÏÓÁ˙˜ÌËﬂ ËÌÙ‡ÍÚ (38). á‡ ÔÓÏÂÌËÚÂ È ‰ÓÔËÌ‡Òﬂ Ë Ì‡ÎË˜ËÂÚÓ Ì‡ ‰ÂÔÂÒËﬂ ÒÎÂ‰ ÔÂÊË‚ÂÌËﬂ ÏÓÁ˙˜ÂÌ Ò˙‰Ó‚ ËÌˆË‰ÂÌÚ (58). ëÌËÊÂÌ‡ Çëó ÒÂ Ì‡·Î˛‰‡‚‡ ÔË ÔË‰ÛÊ‡‚‡˘‡ ÏÓÁ˙˜ÌËﬂ ËÌÙ‡ÍÚ Í‡ÓÚË‰Ì‡ ‡ÚÂÓÒÍÎÂÓÁ‡ (37). Ç ÂÁÛÎÚ‡Ú ÓÚ Ò‚ÓÂ ÔÓÛ˜‚‡ÌÂ ‚˙ıÛ Çëó ÔË ·ÓÎÌË Ò ÏÌÓÊÂÒÚ‚ÂÌ‡ ÒÍÎÂÓÁ‡ J.Diamond et al. (13) ÔËÂÏ‡Ú, ˜Â ÂÁÛÎÚ‡ÚËÚÂ, ÔÓÎÛ˜ÂÌË Ò ÚÓÁË ÏÂÚÓ‰ Ò‡ ËÁÚÓ˜ÌËÍ Ì‡ ÔÓÎÂÁÌ‡ ËÌÙÓÏ‡ˆËﬂ Á‡ ÓˆÂÌÍ‡ Ì‡ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ëﬂ ÍÓÌÚÓÎ Ì‡ Ò˙ˆÂÚÓ, Í‡ÍÚÓ Ë Á‡ ËÁ·Ó‡ Ì‡ ÔÓ – ÂÙÂÍÚË‚ÌÓ ÎÂ˜ÂÌËÂ Ë eı‡·ËÎËÚ‡ˆËÓÌÌ‡ ÒÚ‡ÚÂ„Ëﬂ. Ç‡Ë‡·ËÎÌÓÒÚÚ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Ì‡Ï‡Îﬂ‚‡ Ò Û‚ÂÎË˜‡‚‡ÌÂ Ì‡ ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë Ì‡ ÌÂ„Ó‚‡Ú‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ (42). èË ·ÓÎÌË Ò ‚ÚÓË˜ÌÓ ÔÓ„ÂÒË‡˘‡ ÙÓÏ‡ Ì‡ ÏÌÓÊÂÒÚ‚ÂÌ‡ ÒÍÎÂÓÁ‡ Merico et al. (45) Ì‡ÏË‡Ú ÒÚ‡ÚËÒÚË˜ÌÓ ÁÌ‡˜ËÏË Á‡‚ËÒËÏÓÒÚË ÏÂÊ‰Û Ô‡‡ÏÂÚËÚÂ Ì‡ Çëó Ë ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÒÍ‡Î‡Ú‡ Á‡ ÓˆÂÌÍ‡ Ì‡ ÛÏÓ‡; ÔË Ò˙˘ËÚÂ ·ÓÎÌË ÓÚÒ˙ÒÚ‚‡ Á‡‚ËÒËÏÓÒÚ Ì‡ Çëó Ò ‚˙Á‡ÒÚÚ‡ Ë EDSS ÒÍ‡Î‡Ú‡. ç‡·Î˛‰‡‚‡ÌËÚÂ ÁÌ‡˜ËÏË ‡ÁÎËÍË ‚ ÒÚÂÔÂÌÚ‡ Ì‡ ÛÏÓ‡ ÏÂÊ‰Û Ô‡ˆËÂÌÚËÚÂ Ò Ë ·ÂÁ ‡‚ÚÓÌÓÏÌË Ì‡Û¯ÂÌËﬂ ÔÓ‰ÒÍ‡Á‚‡Ú ‰ÓÔ˙ÎÌËÚÂÎÌÓÚÓ È ÔÓ‚ÎËﬂ‚‡ÌÂ ÓÚ ‡‚ÚÓÌÓÏÌ‡ ‰ËÒÙÛÌÍˆËﬂ (46). àÁÒÎÂ‰‚‡ÌÂÚÓ Ì‡ ÔÓÏÂÌËÚÂ ‚˙‚ Çëó ÌÂ ÍÓÂÎË‡ Ò ÂÁÛÎÚ‡ÚËÚÂ ÓÚ I-123 MIBG ÒˆËÌÚË„‡ÙËﬂ Ì‡ ÏËÓÍ‡‰‡. ë˙˜ÂÚ‡ÌÓÚÓ ËÁÔÓÎÁ‚‡ÌÂ Ì‡ ÚÂÁË ÏÂÚÓ‰Ë ÔË ·ÓÎÌË Ò ‚ÚÓË˜ÌÓ ÔÓ„ÂÒË‡˘‡ ÏÌÓ- 152 ÊÂÒÚ‚ÂÌÌ‡ ÒÍÎÂÓÁ‡ ÏÓÊÂ ‰‡ ‰ÓÔËÌÂÒÂ Á‡ ÓˆÂÌﬂ‚‡ÌÂ Ì‡ ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (39). èË Â‰Ëˆ‡ ‰Û„Ë ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ Ò‡ Ô‡‚ÂÌË Â‰ËÌË˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ Ì‡ ‚‡Ë‡·ËÎÌÓÒÚÚ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡, ‰ÓÍ‡Á‚‡˘Ë Ì‡ÎË˜ËÂ Ì‡ ‡‚ÚÓÌÓÏÌË Ì‡Û¯ÂÌËﬂ. ÑÓÍ‡ÚÓ Allan L. et al. (2) ÌÂ Ì‡ÏË‡Ú ÔÓÏÂÌË ‚˙‚ Çëó ÔË ·ÓÎÂÒÚÚ‡ Ì‡ ÄÎˆı‡ÈÏÂ, ‰Û„Ë ‡‚ÚÓË (19) ÓÚÍË‚‡Ú ÁÌ‡˜ËÏÓ ÒÌËÊÂÌËÂ Ì‡ ‚ÒË˜ÍË ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡, ÓÒÓ·ÂÌÌÓ Ì‡ LF Ë LF/HF ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò Á‰‡‚Ë ÎËˆ‡. íÂ ‰ÓÔÛÒÍ‡Ú, ˜Â Ì‡Û¯ÂÌËÂÚÓ Ì‡ Ò˙‰Â˜ÌËÚÂ ‡‚ÚÓÌÓÏÌË ÙÛÌÍˆËË ÒÂ ‰˙ÎÊË Ì‡ ıÓÎËÌÂ„Ë˜ÂÌ ‰ÂÙËˆËÚ, ÍÓÈÚÓ ‚ ÌﬂÍÓË ÒÎÛ˜‡Ë ÏÓÊÂ ‰‡ ÒÂ ÍÓË„Ë‡ ˜ÂÁ ÎÂ˜ÂÌËÂ Ò ıÓÎËÌÂÒÚÂ‡ÁÌË ËÌıË·ËÚÓË. Çëó Â ÔÓÎÂÁÂÌ Ë ˆÂÌÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌÍ‡ Ì‡ ÙÛÌÍˆËÓÌ‡ÎÌÓÚÓ Ò˙ÒÚÓﬂÌËÂ Ì‡ Äçë, ÓÒÓ·ÂÌÓ ‚ ‡ÌÌËÚÂ ÒÚ‡‰ËË Ì‡ ÓÎË‚ÓÔÓÌÚÓˆÂÂ·ÂÎ‡Ì‡ ‰Â„ÂÌÂ‡ˆËﬂ (36). èË ÒÔËÌÓˆÂÂ·ÂÎ‡Ì‡ ‰Â„ÂÌÂ‡ˆËﬂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÔÂ‰ËÏÌÓ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë Ì‡Û¯ÂÌËﬂ Ë ÚÂÊÂÒÚÚ‡ ËÏ ÍÓÂÎË‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (54). èË Ô‡ˆËÂÌÚË Ò Î‡ÚÂ‡ÎÌ‡ ‡ÏËÓÚÓÙË˜Ì‡ ÒÍÎÂÓÁ‡ ÒÂ Ì‡·Î˛‰‡‚‡ ÔÓ‚Ë¯ÂÌ‡ Ò˙‰Â˜Ì‡ ˜ÂÒÚÓÚ‡, Ì‡Ï‡ÎÂÌÓ SDNN Ë Û‚ÂÎË˜‡‚‡ÌÂ Ì‡ ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF, ÍÓËÚÓ ÌÂ ÍÓÂÎË‡Ú Ò ÍÎËÌË˜ÌËÚÂ ÒËÏÔÚÓÏË Ë ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. êÂ„ËÒÚË‡ÌÓÚÓ ÒÛ·ÍÎËÌË˜ÌÓ ‡‚ÚÓÌÓÏÌÓ Ì‡Û¯ÂÌËÂ Á‡Òﬂ„‡ ÔÂ‰ËÏÌÓ Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ëﬂ Ò˙‰Â˜ÂÌ ÍÓÌÚÓÎ (53). èË ÚÂÁË ·ÓÎÌË ËÏ‡ ‰‡ÌÌË Á‡ ÔÓÒÚ„‡Ì„ÎËÓÌ‡Ì‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‡‰ÂÌÂ„Ë˜Ì‡ ‰ÂÌÂ‚‡ˆËﬂ, ‰ÓÍ‡Á‡Ì‡ ˜ÂÁ I123 MIBG ÒˆËÌÚË„‡ÙËﬂ Ë Á‡ Í‡‰ËÓ‚‡„‡ÎÌ‡ ‰ÂÌÂ‚‡ˆËﬂ, ÛÒÚ‡ÌÓ‚ÂÌ‡ ˜ÂÁ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ Çëó (14). èË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ Ì‡ ï˙ÌÚËÌ„ÚÓÌ, ÍÓËÚÓ Ò‡ ‡ÒËÏÔÚÓÏÌË „ÂÌÌË ÌÓÒËÚÂÎË ËÎË Ò‡ Ò ÛÏÂÂÌÓ ËÁ‡ÁÂÌË ÍÎËÌË˜ÌË ÔÓﬂ‚Ë, ÒÂ Ì‡·Î˛‰‡‚‡ ÔÓ‚Ë¯ÂÌ‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‡ÍÚË‚ÌÓÒÚ, ÍÓﬂÚÓ Â ‚ ÍÓÌÚ‡ÒÚ Ò˙Ò ÒÌËÊÂÌ‡Ú‡ ‡‚ÚÓÌÓÏÌ‡ Â„ÛÎ‡ˆËﬂ ‚ Ì‡ÔÂ‰Ì‡ÎËﬂ ÒÚ‡‰ËÈ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (34). ÅÓÎÌËÚÂ Ò ÛÏÂÂÌÓ ‰Ó ÚÂÊÍÓ ËÁ‡ÁÂÌ‡ ·ÓÎÂÒÚ Ì‡ ï˙ÌÚËÌ„ÚÓÌ ËÏ‡Ú Í‡ÍÚÓ ÒËÏÔ‡ÚËÍÓ‚Ë, Ú‡Í‡ Ë Ô‡‡ÒËÏÔ‡ÚËÍÓ‚Ë ‰ËÒÙÛÌÍˆËË (3). ãÓÌ„ËÚÛ‰ËÌ‡ÎÂÌ ‚ÂÏÂ‚Ë Ë ˜ÂÒÚÓÚÂÌ ‡Ì‡ÎËÁ Ì‡ Çëó ÔË ·ÓÎÌË Ò ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÚËÔ Ñ˛¯ÂÌ Â ÔÓÍ‡Á‡Î ÔÓ„ÂÒË‚ÌÓ ÒÌËÊÂÌËÂ Ì‡ Ô‡‡ÏÂÚËÚÂ ÓÚ ˜ÂÒÚÓÚÌ‡Ú‡ Ó·Î‡ÒÚ, ÓÒÓ·ÂÌÌÓ ‚ ÌÓ˘ÌËÚÂ ˜‡ÒÓ‚Â, ‰ÓÍ‡ÚÓ ‚ÂÏÂ‚ËÚÂ Ô‡‡ÏÂÚË Ò‡ ÔÓÍ‡Á‡ÎË ÒÌËÊÂÌËÂ ÔÂÁ ˆﬂÎÓÚÓ ‰ÂÌÓÌÓ˘ËÂ (62). èË ÚÂÁË ·ÓÎÌË ÒÂ Ì‡·Î˛‰‡‚‡ Ì‡Û¯ÂÌ‡ ‡‚ÚÓÌÓÏÌ‡ ÙÛÌÍˆËﬂ, ÍÓﬂÚÓ Á‡Òﬂ„‡ ÓÒÌÓ‚ÌÓ Ó·˘‡Ú‡ ‚‡Ë‡·ËÎÌÓÒÚ ÔË ÓÚÒ˙ÒÚ‚ËÂ Ì‡ ‡·ÌÓÏÌË ÔÓÏÂÌË ‚ ÍÓÌ‚ÂÌˆËÓÌ‡ÎÌÓÚÓ ÍÎËÌË˜ÌÓ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ÙÛÌÍˆËﬂ. Ä‚ÚÓËÚÂ ÔÂ‰Î‡„‡Ú ËÁÏÂ‚‡ÌÂ Ì‡ ÒÂ‰Ì‡Ú‡ Ò˙‰Â˜Ìa ˜ÂÒÚÓÚ‡ ÔÂÁ ÌÓ˘Ú‡ Í‡ÚÓ Ì‡È-ÎÂÒÂÌ ÏÂÚÓ‰ Á‡ ÓˆÂÌÍ‡ Ì‡ ‡‚ÚÓÌÓÏÌ‡Ú‡ ÙÛÌÍˆËﬂ ÔË Ú‡ÍË‚‡ Ô‡ˆËÂÌÚË (30). èË ·ÓÎÌË Ò ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ 1 ÚËÔ ÔË 24 – ˜‡ÒÓ‚Ë Á‡ÔËÒË SDNN Ë Ó·˘‡Ú‡ ‚‡Ë‡·ËÎÌÓÒÚ Ò‡ ÁÌ‡˜ËÏÓ ÔÓ – ÌËÒÍË ÒÔﬂÏÓ Á‰‡‚ËÚÂ. éÒÚ‡Ì‡ÎËÚÂ Ô‡‡ÏÂÚË Ò˙˘Ó ÔÓÍ‡Á‚‡Ú ÚÂÌ‰ÂÌˆËﬂ Í˙Ï ÒÌËÊÂÌËÂ. Ñ‡ÌÌËÚÂ ÔÓ‰ÒÍ‡Á‚‡Ú Ì‡ÎË˜ËÂ Ì‡ ÒËÏÔ‡ÚËÍÓ‚‡ ‰ËÒÙÛÌÍˆËﬂ Ë ÔÂ‚‡ÎË‡ÌÂ Ì‡ ‚‡„ÛÒÓ‡Ú‡ ‡ÍÚË‚ÌÓÒÚ ÔË ÚÂÁË ·ÓÎÌË. çÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ Á‡‚ËÒËÏÓÒÚ Ì‡ ëÄÑ ÓÚ ‚˙Á‡ÒÚÚ‡ Ì‡ ·ÓÎÌËÚÂ Ë ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ(56). àÁÒÎÂ‰‚‡ÌÂÚÓ Ì‡ Çëó ÒÎÂ‰ ÔÂÍ‡‡ÌË ÚÂÊÍË ˜ÂÂÔÌÓÏÓÁ˙˜ÌË Ú‡‚ÏË (óåí) Â Ò‚˙Á‡ÌÓ Ò ‰ÓÍ‡Á‡Ì‡ Â‰ÛÍˆËﬂ Ì‡ TP Ë LF/HF Ë ÌÂ·Î‡„ÓÔËﬂÚÌ‡ ÔÓ„ÌÓÁ‡ Á‡ ·ÓÎÌËÚÂ (23). èË ‰Âˆ‡ Ò óåí ‡Ì‡ÎËÁ˙Ú Ì‡ Çëó ÒÎÂ‰ Ô‡ÒË‚ÌÓ ËÁÔ‡‚ﬂÌÂ ‰ÓÔËÌ‡Òﬂ Á‡ ‰Ë‡„ÌÓÒÚËˆË‡ÌÂ Ì‡ ÌÂ‚ÓÏÂ‰ËË‡ÌË ÒËÌÍÓÔË. ìÒÚ‡ÌÓ‚ÂÌËﬂÚ ıËÔÂÂ‡ÍÚË‚ÂÌ ÓÚ„Ó‚Ó Ë„‡Â ÓÎﬂ ‚ ‡Á‚ËÚËÂÚÓ Ì‡ Ô‡ÚÓÎÓ„Ë˜ÌË ‡‚ÚÓÌÓÏÌË ÂÙÎÂÍÒË (16). ÉÓÎÂÏËﬂÚ ·ÓÈ ÔÓÛ˜‚‡ÌËﬂ Ë Ì‡ÚÛÔ‡ÌËﬂÚ ÓÔËÚ ÓÚ Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ËÁÒÎÂ‰‚‡ÌÂÚÓ Ì‡ Çëó ÔË ‡ÁÎË˜ÌË ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ ÌË ÔÓÁ‚ÓÎﬂ‚‡ ‰‡ ÔËÂÏÂÏ, ˜Â ÚÓÁË ÌÂËÌ‚‡ÁË‚ÂÌ, ÎÂÒÂÌ Á‡ ËÁÔ˙ÎÌÂÌËÂ Ë ËÌÚÂÔÂÚ‡ˆËﬂ ÏÂÚÓ‰ Á‡ ÓˆÂÌÍ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ‡‚ÚÓÌÓÏÌ‡ ÙÛÌÍˆËﬂ ËÏ‡ ÓÔÂ‰ÂÎÂÌ‡ ‰Ë‡„ÌÓÒÚË˜Ì‡ ÒÚÓÈÌÓÒÚ ÔË ÚÂÁË Á‡·ÓÎﬂ‚‡ÌËﬂ. ç‡È-˜ÂÒÚÓ ÔËÎ‡„‡ÌÂÚÓ ÏÛ Â Ò‚˙Á‡ÌÓ Ò˙Ò ÒÌËÊÂÌËÂ Ì‡ Ó·˘‡Ú‡ ‚‡Ë‡·ËÎÌÓÒÚ, Ì‡ ÌËÒÍÓ˜ÂÒÚÓÚÌËÚÂ ÍÓÏÔÓÌÂÌÚË Ì‡ ÒÔÂÍÚ˙‡ Ë Ì‡ ÓÚÌÓ¯ÂÌËÂÚÓ LF/HF, ÍÓËÚÓ ÔÓÍ‡Á‚‡Ú Ì‡Û¯ÂÌ‡ ÒËÏÔ‡ÚËÍÓ‚‡ ÙÛÌÍˆËﬂ. èÓﬂ‚‡Ú‡ ËÏ Â Ò‚˙Á‡Ì‡ Ò Û‚ÂÊ‰‡ÌÂ Ì‡ ÏÓÁ˙˜ÌË ‡‚ÚÓÌÓÏÌË ÒÚÛÍÚÛË (ÎËÏ·Ë˜ÌË, ËÌÒÛÎ‡Ì‡ ÍÓ‡, ıËÔÓÚ‡Î‡ÏÛÒ, ÒÚ‚ÓÎÓ‚Ë Ë „˙·Ì‡˜ÌÓÏÓÁ˙˜ÌË ‡‚ÚÓÌÓÏÌË ﬂ‰‡), Í‡ÍÚÓ Ë Ò Ì‡Û¯‡‚‡ÌÂ Ì‡ ÚÂıÌËÚÂ ‰ÂÒˆÂÌ‰ÂÌÚÌË ÔÓÂÍˆËË Ë ÙÛÌÍˆËÓÌ‡ÎÌË ‚˙ÁÍË. Ç „ÂÌÂÁ‡Ú‡ Ì‡ ÔÓÏÂÌËÚÂ Ì‡ Çëó Û˜‡ÒÚ‚‡Ú Ë Ì‡Û¯ÂÌËﬂ ‚ ËÏÛÌÌ‡Ú‡ Ë ÌÂ‚ÓÚ‡ÌÒÏËÚÂÌËÚÂ ÒËÒÚÂÏË. èÂˆËÁË‡ÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÒÚË˜Ì‡Ú‡ ÒÚÓÈÌÓÒÚ Ì‡ Çëó ÔË ÓÚ‰ÂÎÌËÚÂ ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ Ì‡Î‡„‡ ÔÓ‚ÂÊ‰‡ÌÂ Ì‡ ÔÓ-Ì‡Ú‡Ú˙¯ÌË ËÁÒÎÂ‰‚‡ÌËﬂ Ò ÚÓÁË ÏÂÚÓ‰. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. ÇÂÎ˜Â‚‡ à. ÑËÏËÚÓ‚ ÑÓÒ. Ñ., å‡ÌÚ‡Ó‚‡ ë. Ñ‡ÏﬂÌÓ‚ è. ÑËÏËÚÓ‚ ÑËÏ Ñ., á‡ÔﬂÌÓ‚‡ ã., àÁÒÎÂ‰‚‡ÌÂ Ì‡ ‡‚ÚÓÌÓÏÌËﬂ ·‡Î‡ÌÒ ÔË ÌﬂÍÓË ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ ˜ÂÁ ‡Ì‡ÌÎËÁ Ì‡ ‚‡Ë‡·ËÎÌÓÒÚÚ‡ Ì‡ Ò˙‰Â˜Ì‡Ú‡ ˜ÂÒÚÓÚ‡. Å˙Î„‡ÒÍ‡ ÌÂ‚ÓÎÓ„Ëﬂ. 9, 2009, 1, 30-32. Allan LM, Kerr SR, Ballard CG, Allen J, Murray A, McLaren AT, Kenny RA. Autonomic function assessed by heart rate variability is normal in Alzheimer's disease and vascular dementia. Dement Geriatr Cogn Disord., 2005, 19,140-144. Andrich J, Schmitz T, Saft C, Postert T, Kraus P, Epplen JT, Przuntek H, Agelink MW. Autonomic nervous system function in Huntington's disease. 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Task Force of the European Society of Cardiology and the North American Society of Pacing and Electrophysiology. Heart ratevariability. Standards of measurement, physiological interpretation,and clinical use. Circulation, 93, 1996, 1043–1065. 62. Yotsukura M, Fujii K, Katayama A, Tomono Y, Ando H, Sakata K, Ishihara T, Ishikawa K.Nine-year follow-up study of heart rate variability in patients with Duchenne-type progressive muscular dystrophy. Am Heart J., 136, 1998, 289-296. Ä‰ÂÒ Á‡ ÍÓÂÒÔÓÌ‰ÂÌˆËﬂ: Ñ- ëÚÂÙÍ‡ å‡ÌÚ‡Ó‚‡, ÇÚÓ‡ ÌÂ‚ÓÎÓ„Ë˜Ì‡ ÍÎËÌËÍ‡ ëÅÄãçè ◊ë‚. ç‡ÛÏ” ëÓÙËﬂ ÛÎ. ◊ã. êÛÒÂ‚” ‹ 1, 1113 ëÓÙËﬂ, ÚÂÎ. 9702 214 e-mail: [email protected] 154 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 155 é·ÁÓ ÑàëíêéîàçéèÄíàà- ÉÖçéíàè-îÖçéíàèçà äéêÖãÄñàà, äéÉçàíàÇçà çÄêìòÖçàü à ÑàÄÉçéëíàäÄ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡1, å ê‡È˜Â‚‡1, à. í˙ÌÂ‚1,2 çÂ‚ÓÎÓ„Ë˜Ì‡ ÍÎËÌËÍ‡, ìåÅÄã ◊ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, åî –ëÓÙËﬂ 2çÓ‚ ·˙Î„‡ÒÍË ÛÌË‚ÂÒËÚÂÚ, ä‡ÚÂ‰‡Ú‡ ÔÓ äÓ„ÌËÚË‚Ì‡ Ì‡ÛÍ‡ Ë ÔÒËıÓÎÓ„Ëﬂ 1 SUMMARY DYSTROPHINOPATHIES- GENOTYPE- PHENOTYPE CORRELATIONS AND COGNITIVE DISORDERS IN PATIENTS WITH DUCHENNE AND BECKER MUSCULAR DYSTROPHIES T Chamova1, M Raytcheva1, I Tournev1,2 1 Neurology clinic, University hospital “Alexandrovska”, Medical Faculty 2 New Bulgarian University, Chair of cognitive sciences and psychology Duchenne and Becker progressive muscular dystrophies (DMD and BMD) are allelic X-linked genetic myopathies, caused by mutations in the dystrophin gene. They are clinically characterized by progressive muscle wasting, loss of walking ability leading to complete wheelchair dependence, cardiomyopathy, and in some of the cases by mental retardation and nanismus. These two forms of dystrophinopathy differ from one another by the age of onset, severity of the clinical features and the progession of the disorder. The non-progressive cognitive disorders with lower scores for verbal skills and short-term memory are specific clinical features for some of the patients with DMD , whereas the patients with BMD have a more heterogeneous cognitive profile, which encompass learning difficulties as well as autism, behavioral and attention problems. The pathogenesis of this disorders is complex, mainly due to mutations in the second part of the gene, that disturb the regulation of the gene expression of the dystrophin isoforms, localized in the central nervous system (Dp140, Dp71, Dp116) and subsequently cause disruption of the neuronal maturation and development, the synaptogenesis and the intracellular homeostasis of the neurons and glial cells.The diagnostic algorithm of dystrophinopathies is complex as the genetic tests are an important diagnostic procedure, used to identify the mutations in the dystrophin gene in patients and in female carriers in order to perform prenatal diagnosis. KEY WORDS: Dystrophinopathies; dystrophin isoforms; cognitive disorders. êÖáûåÖ èÓ„ÂÒË‚ÌËÚÂ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ Ò‡ ï-Ò‚˙Á‡ÌË Ì‡ÒÎÂ‰ÒÚ‚ÂÌË ÏËÓÔ‡ÚËË, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÏÛÚ‡ˆËË, Á‡Òﬂ„‡˘Ë ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ. äÎËÌË˜ÌÓ ÒÂ ÔÓﬂ‚ﬂ‚‡Ú Ò ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ ‰Ó ÚÂÊÍ‡ ËÌ‚‡ÎË‰ËÁ‡ˆËﬂ, Í‡‰ËÓÏËÓÔ‡ÚËﬂ, ‡ ‚ ÌﬂÍÓË ÓÚ ÒÎÛ˜‡ËÚÂ Ë Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë Ì‡ÌËÁ˙Ï. ê‡ÁÎË˜‡‚‡Ú ÒÂ ÔÓÏÂÊ‰Û ÒË ÔÓ Ì‡˜‡ÎÓÚÓ, ÚÂÊÂÒÚÚ‡ Ì‡ ÍÎËÌË˜Ì‡Ú‡ ÒËÏÔÚÓÏ‡ÚËÍ‡ Ë ÒÍÓÓÒÚÚ‡ Ì‡ ÔÓ„ÂÒËﬂ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. äÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ Ò ÔÓ-ÚÂÊÍÓ Á‡Òﬂ„‡ÌÂ Ì‡ ‚Â·‡ÎÌËÚÂ ÛÏÂÌËﬂ Ë Í‡ÚÍÓÒÓ˜Ì‡Ú‡ Ô‡ÏÂÚ Ò‡ ÒÔÂˆËÙË˜Ì‡ ÍÎËÌË˜Ì‡ ÓÒÓ·ÂÌÓÒÚ ÔË ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ, ‰Ó- Í‡ÚÓ Á‡ Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ ÍÓ„ÌËÚË‚ÌËﬂÚ ÔÓÙËÎ Â ÔÓ-ıÂÚÂÓ„ÂÌÂÌ Ë ‚ÍÎ˛˜‚‡ Á‡ÚÛ‰ÌÂÌËﬂ ‚ Ó·Û˜ÂÌËÂÚÓ, Í‡ÍÚÓ Ë ‡ÛÚËÁ˙Ï, ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ, ıËÔÂ‡ÍÚË‚ÌÓ ÔÓ‚Â‰ÂÌËÂ Ë ‰ÂÙËˆËÚ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ. íÂÁË ÒÏÛ˘ÂÌËﬂ Ì‡È-‚ÂÓﬂÚÌÓ Ò‡ Ò ÍÓÏÔÎÂÍÒÌ‡ „ÂÌÂÁ‡, Í‡ÚÓ ÓÒÌÓ‚ÌÓ ÁÌ‡˜ÂÌËÂ ËÏ‡Ú ÏÛÚ‡ˆËË, ‚˙‚ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚ËÌ‡ „ÂÌ‡, ÍÓËÚÓ Ì‡Û¯‡‚‡Ú Â„ÛÎ‡ˆËﬂÚ‡ Ì‡ „ÂÌÌ‡Ú‡ ÂÍÒÔÂÒËﬂ Ì‡ ‰ËÒÚÓÙËÌÓ‚ËÚÂ ËÁÓÙÓÏË, ÎÓÍ‡ÎËÁË‡ÌË ‚ ñçë (Dp140, Dp71, Dp116) Ë ÔÓ ÚÓÁË Ì‡˜ËÌ Ò˙Áﬂ‚‡ÌÂÚÓ Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÌÂ‚ÌËÚÂ ÍÎÂÚÍË, ÒËÌ‡ÔÚÓ„ÂÌÂÁ‡Ú‡ Ë ‚˙ÚÂÍÎÂÚ˙˜Ì‡Ú‡ ıÓÏÂÓÒÚ‡Á‡ Ì‡ ÌÂ‚ÓÌËÚÂ Ë „ÎËﬂÚ‡. á‡ ‰Ë‡„ÌÓÒÚËˆË‡ÌÂÚÓ Ì‡ èåÑ ÓÒ‚ÂÌ ·ËÓıËÏË˜ÌËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ, ÖåÉ Ë ËÏÛÌÓıËÒÚÓıËÏË˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ ÓÚ Ò˙˘ÂÒÚ‚ÂÌÓ ÁÌ‡˜ÂÌËÂ Ò‡ Ò˙‚ÂÏÂÌÌËÚÂ „ÂÌÂÚË˜ÌË ÏÂÚÓ‰Ë, ‡ÁÍË‚‡˘Ë „ÂÌÂÌ ‰ÂÙÂÍÚ Û ·ÓÎÌË, ÌÓÒËÚÂÎÍË Ë ‚ ÛÒÎÓ‚ËﬂÚ‡ Ì‡ ÔÂÌ‡Ú‡ÎÌ‡ ‰Ë‡ÌÓÁ‡. äãûóéÇà Ñìåà:ÑËÒÚÓÙËÌÓÔ‡ÚËË; ‰ËÒÚÓÙËÌÓ‚Ë ËÁÓÙÓÏË; ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ. èÓ„ÂÒË‚ÌËÚÂ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË (èåÑ) ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ Ò‡ Ì‡ÒÎÂ‰ÒÚ‚ÂÌË ÏËÓÔ‡ÚËË, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÏÛÚ‡ˆËË, Á‡Òﬂ„‡˘Ë ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ, ÎÓÍ‡ÎËÁË‡Ì ‚ ï ıÓÏÓÁÓÏ‡Ú‡, ÍÎËÌË˜ÌÓ ÔÓﬂ‚ﬂ‚‡˘Ë ÒÂ Ò ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ ‰Ó ÚÂÊÍ‡ ËÌ‚‡ÎË‰ËÁ‡ˆËﬂ, Í‡‰ËÓÏËÓÔ‡ÚËﬂ, ‡ ‚ ÌﬂÍÓË ÓÚ ÒÎÛ˜‡ËÚÂ Ë Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë Ì‡ÌËÁ˙Ï. ê‡ÁÎË˜‡‚‡Ú ÒÂ ÔÓÏÂÊ‰Û ÒË ÔÓ Ì‡˜‡ÎÓÚÓ, ÚÂÊÂÒÚÚ‡ Ì‡ ÍÎËÌË˜Ì‡Ú‡ ÒËÏÔÚÓÏ‡ÚËÍ‡ Ë ÒÍÓÓÒÚÚ‡ Ì‡ ÔÓ„ÂÒËﬂ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. è˙‚ÓÚÓ ÔÓ‰Ó·ÌÓ ÍÎËÌË˜ÌÓ Ë ·ËÓÔÒË˜ÌÓ Ô‡ÚÓ‡Ì‡ÚÓÏË˜ÌÓ ÓÔËÒ‡ÌËÂ Ì‡ ÔÓ„ÂÒË‚Ì‡Ú‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ Í‡ÚÓ ◊èÒÂ‚‰ÓıËÔÂÚÓÙË˜Ì‡ ÏÛÒÍÛÎÌ‡ Ô‡‡ÎËÁ‡” e Ì‡ ÙÂÌÒÍËﬂ ÌÂ‚ÓÎÓ„ Guillaume Benjamin Amand Duchenne (1868) „. Gowers ÓÔËÒ‚‡ ÙÓÏ‡Ú‡ Ò ÔÓ-·‡‚ÌÓ ‡Á‚ËÚËÂ ÓÍÓÎÓ Ë ÒÎÂ‰ ÔÛ·ÂÚÂÚ‡, ÍÓﬂÚÓ Â ÔÓ‰Ó·ÌÓ ı‡‡ÍÚÂËÁË‡Ì‡ ÓÚ E.Becker (1955). éÚÍËÚËÂÚÓ Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ (Monaco Ë Ò˙‡‚Ú. 1986) Â ÓÒÌÓ‚‡Ú‡ Á‡ Ò˙‚ÂÏÂÌÌÓÚÓ ‡Á·Ë‡ÌÂ Ì‡ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ë ‚˙ÁÏÓÊÌÓÒÚËÚÂ Á‡ ÚÂ‡ÔËﬂ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. ÖèàÑÖåàéãéÉàü èåÑ ÚËÔ Ñ˛¯ÂÌ ÒÂ ÒÂ˘‡ Ò ˜ÂÒÚÓÚ‡ 1 Ì‡ 3500 ÊË‚ÓÓ‰ÂÌË ÏÓÏ˜ÂÚ‡. èË 30% ÓÚ Úﬂı ÏÛÚ‡ˆËﬂÚ‡ ‚˙ÁÌËÍ‚‡ de novo. èåÑ ÚËÔ ÅÂÍÂ ÒÂ ÔÓﬂ‚ﬂ‚‡ Ò ˜ÂÒÚÓÚ‡ 1 Ì‡ 30 000 ÊË‚ÓÓ‰ÂÌË ÏÓÏ˜ÂÚ‡. èË 50% ÏÛÚ‡ˆËËÚÂ Ò‡ de novo. ÖíàéãéÉàü ÉÂÌ˙Ú ÓÚ„Ó‚ÓÂÌ Á‡ èåÑ ÚËÔ Ñ˛¯ÂÌ Ë ÅÂÍÂ Â ÍÎÓÌË‡Ì Ë Í‡ÚË‡Ì ÔÂÁ 1986 „. (60) Ë ÔÓ‰ÛÍÚ˙Ú ÏÛ- ÔÓÚÂËÌ˙Ú ‰ËÒÚÓÙËÌ Â ÓÔËÒ‡Ì ÓÚ ÂÍËÔ‡ Ì‡ Kunkel ÔÂÁ 1987 (44). ÑËÒÚÓÙËÌÓ‚ËﬂÚ „ÂÌ ÒÂ Ì‡ÏË‡ Ì‡ ï-ıÓÏÓÁÓÏ‡Ú‡ ‚ ÎÓÍÛÒ ï21. ë˙ÒÚÓË ÒÂ 2,4 ÏÂ„‡·‡ÁË (mb) ÓÚ Ñçä, ÔÂ‰Ò- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology Ú‡‚Îﬂ‚‡ 1% ÓÚ ï ıÓÏÓÁÓÏ‡Ú‡ Ë Â Â‰ËÌ ÓÚ Ì‡È-„ÓÎÂÏËÚÂ „ÂÌË ‚ ˜Ó‚Â¯ÍËﬂ „ÂÌÓÏ. äÓ‰Ë‡˘‡Ú‡ ˜‡ÒÚ Á‡ ÒËÌÚÂÁ‡ Ì‡ ‰ËÒÚÓÙËÌ Â ÓÚ ÓÍÓÎÓ 15 ÍËÎÓ·‡ÁË ËÎË 79 ÂÍÁÓÌ‡ Ë Ò˙ÒÚ‡‚Îﬂ‚‡ Ò‡ÏÓ 0,6 % ÓÚ „ÂÌ‡ (38, 64). ÖÍÁÓÌËÚÂ Ò‡ ÓÚ„‡ÌË˜ÂÌË Â‰ËÌ ÓÚ ‰Û„ ˜ÂÁ ËÌÚÓÌË, ÍÓËÚÓ Ò‡ Ò‚˙Á‡ÌË Ò Â„ÛÎ‡ˆËﬂÚ‡ Ì‡ „ÂÌÂÚË˜Ì‡Ú‡ ÂÍÒÔÂÒËﬂ Ë Â‚ÓÎ˛ˆËﬂÚ‡ Ì‡ „ÂÌÂÚË˜ÌËﬂ ÍÓ‰. ÉÂÌ˙Ú ÒÂ ı‡‡ÍÚÂËÁË‡ Ò˙Ò ÒÎÓÊÌ‡ Â„ÛÎ‡ˆËﬂ Ì‡ Ú‡ÌÒÍË·ˆËﬂÚ‡, ÔÓ‡‰Ë Ì‡ÎË˜ËÂÚÓ Ì‡ ÓÒÂÏ ‡ÁÎË˜ÌË ÔÓÏÓÚÓ‡ ÔÓ ˆﬂÎ‡Ú‡ ÏÛ ‰˙ÎÊËÌ‡, Â„ÛÎË‡˘Ë ÒËÌÚÂÁ‡Ú‡ Ì‡ ‡ÁÎË˜ÌË ÔÓ ‰˙ÎÊËÌ‡ ‰ËÒÚÓÙËÌÓ‚Ë ËÁÓÙÓÏË. åÛÚ‡ˆËËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ èåÑ ÚËÔ Ñ˛¯ÂÌ (31;63) Ò‡ „Î‡‚ÌÓ ‰ÂÎÂˆËË (ÓÍÓÎÓ 60%), ÔÂ‰ËÏÌÓ ÓÍÓÎÓ ËÌÚÓÌ 7 Ë ‰ÛÔÎËÍ‡ˆËË (ÓÍÓÎÓ 8%); ÓÍÓÎÓ 30% Ò‡ ÚÓ˜ÍÓ‚Ë ÏÛÚ‡ˆËË, Â‰ÌÓÌÛÍÎÂÓÚË‰ÌË Á‡ÏÂÌË, ËÌÒÂˆËË, Ï‡ÎÍË ‰ÂÎÂˆËË Ë ‰ÛÔÎËÍ‡ˆË. èË èåÑ ÚËÔ ÅÂÍÂ 80% ÓÚ ÏÛÚ‡ˆËËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ ·ÓÎÂÒÚÚ‡ Ò‡ ‰ÂÎÂˆËË ‚ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ. ç‡È-˜ÂÒÚÓ ÏÛÚ‡ˆËËÚÂ ÒÂ ÎÓÍ‡ÎËÁË‡Ú ‚ Ó·Î‡ÒÚÚ‡ Ì‡ Ô˙‚ËÚÂ 20 ÂÍÁÓÌ‡ (ÓÒÌÓ‚ÌÓ ÓÍÓÎÓ 7 ÂÍÁÓÌ) Ë ÏÂÊ‰Û 44 Ë 45 ÂÍÁÓÌ, Í˙‰ÂÚÓ ËÌÚÓÌËÚÂ Ò‡ Ò ÔÓ-„ÓÎﬂÏ‡ ÌÛÍÎÂÓÚË‰Ì‡ ÔÓÒÎÂ‰Ó‚‡ÚÂÎÌÓÒÚ. Ç ËÌÚÓÌ 50 Ò‡ Â„ËÒÚË‡ÌË Ì‡È-ÏÌÓ„Ó ÚÓ˜ÍË Ì‡ ‡ÁÍ˙Ò‚‡ÌÂ ‚ ÔÓÔÛÎ‡ˆËﬂÚ‡ Ì‡ ·‡ÎÍ‡ÌÒÍËﬂ ÔÓÎÛÓÒÚÓ‚ (3). Ñ‚Â ÓÒÌÓ‚ÌË ÂÍÓÏ·ËÌ‡ˆËÓÌÌË Ò˙·ËÚËﬂ ‚Ó‰ﬂÚ ‰Ó „ÂÌÂË‡ÌÂ Ì‡ ‰ÂÎÂˆËË Ë ËÁﬂ‚‡ Ì‡ „ÂÌÂÚË˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ: ıÓÏÓÎÓÊÌ‡ ÌÂÂˆËÔÓ˜Ì‡ ÂÍÓÏ·ËÌ‡ˆËﬂ, ÔÓÓ‰ÂÌ‡ ÓÚ ÒıÓ‰ÌË „ÂÌÌË ÔÓÒÎÂ‰Ó‚‡ÚÂÎÌÓÒÚË ËÎË ÓÚ ÔÓ‚ÚÓÂÌË ÂÎÂÏÂÌÚË Ë ÌÂıÓÏÓÎÓÊÌ‡ ÂÍÓÏ·ËÌ‡ˆËﬂ, ‚ÍÎ˛˜‚‡˘‡ Û˜‡ÒÚ˙ˆË Ò ÏËÌËÏ‡ÎÌ‡ ÒÂÍ‚ÂÌˆËÓÌÌ‡ ıÓÏÓÎÓ„Ëﬂ. ÉÓÎÂÏËÌ‡Ú‡ Ì‡ ‰ÂÎÂˆËﬂÚ‡ ÌÂ ‚ËÌ‡„Ë ÍÓÂÎË‡ Ò ÔÓÚÂÊÍ‡ ÍÎËÌË˜Ì‡ ËÁﬂ‚‡. ïËÔÓÚÂÁ‡Ú‡ Ì‡ Monaco Ë Ò˙‡‚Ú. (1986) Ô‡‚Ë ÓÔËÚ ‰‡ Ó·ﬂÒÌË ‡ÁÎËÍËÚÂ ‚˙‚ ÙÂÌÓÚËÔÌ‡Ú‡ ËÁﬂ‚‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. äÓ„‡ÚÓ ‰ÂÎÂˆËﬂÚ‡ Ì‡Û¯Ë ‡ÏÍ‡Ú‡ Ì‡ ˜ÂÚÂÌÂ Ò ÔÓﬂ‚‡ Ì‡ ÒÚÓÔ ÍÓ‰ÓÌ, ÒÂ ÒËÌÚÂÁË‡ ‰ÂÙÂÍÚÂÌ, ÔÓ-Í˙Ò ‰ËÒÚÓÙËÌ Ò ÎËÔÒ‡ Ì‡ ë-ÚÂÏËÌ‡Î, ÍÓÈÚÓ ·˙ÁÓ ÒÂ ‡ÁÛ¯‡‚‡. îÂÌÓÚËÔÌ‡Ú‡ ËÁﬂ‚‡ Ì‡ Ú‡Í˙‚ ÚËÔ ÏÛÚ‡ˆËË Â èåÑ ÚËÔ Ñ˛¯ÂÌ. äÓ„‡ÚÓ ÌÂ ÒÂ Ì‡Û¯Ë ‡ÏÍ‡Ú‡ Ì‡ ˜ÂÚÂÌÂ ÒÎÂ‰ ÏÛÚ‡ˆËﬂÚ‡, ÒÂ ÒËÌÚÂÁË‡ ‰ËÒÚÓÙËÌ Ò ‡ÁÎË˜Ì‡ ÔÓ ‰˙ÎÊËÌ‡ Á‡„Û·‡ Ì‡ ‚˙ÚÂ¯ÌËﬂ ÒÂ„ÏÂÌÚ, ÌÓ Á‡Ô‡ÁÂÌË ë Ë NÚÂÏËÌ‡ÎË, ÍÓÂÚÓ ÌÂ ÒÏÛ˘‡‚‡ ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂÚÓ Ò ‰Û„ËÚÂ ·ÂÎÚ˙ˆË ÓÚ „ÎËÍÓÔÓÚÂËÌ-‡ÒÓˆËË‡ÌËﬂ ÍÓÏÔÎÂÍÒ Ë Á‡Ô‡Á‚‡ ÓÚÌÓÒËÚÂÎÌ‡Ú‡ ÙÛÌÍˆËÓÌ‡ÎÌÓÒÚ Ì‡ ‰ËÒÚÓÙËÌ‡. íÓ‚‡ Ó·ÛÒÎ‡‚ﬂ ÔÓ-ÎÂÍ‡Ú‡ ÙÂÌÓÚËÔÌ‡ ËÁﬂ‚‡ Ì‡ èåÑ ÚËÔ ÅÂÍÂ (32; 65). àÁÍÎ˛˜ÂÌËﬂ ÓÚ Ô‡‚ËÎÓÚÓ Ò Ì‡Û¯‡‚‡ÌÂ ËÎË Á‡Ô‡Á‚‡ÌÂ Ì‡ ‡ÏÍ‡Ú‡ Ì‡ ˜ÂÚÂÌÂ Ô‡‚ﬂÚ ÒÎÛ˜‡ËÚÂ, ÔË ÍÓËÚÓ ‰ÂÎÂˆËﬂÚ‡ Á‡Òﬂ„‡ ÔÓÚÂËÌ-Ò‚˙Á‚‡˘ËÚÂ ‰ÓÏÂÌË (14). í‡ÍË‚‡ ÏÛÚ‡ˆËË, ÌÂÁ‡‚ËÒËÏÓ ÓÚ Á‡Ô‡Á‚‡ÌÂÚÓ Ì‡ ‡ÏÍ‡Ú‡ Ì‡ ˜ÂÚÂÌÂ, ‰Ó‚ÂÊ‰‡Ú ‰Ó ÚÂÊÍÓ Á‡Òﬂ„‡ÌÂ Ì‡ ÙÛÌÍˆËﬂÚ‡ Ì‡ ‰ËÒÚÓÙËÌ‡, ÂÒÔ. ËÁﬂ‚‡ Ì‡ èåÑ ÚËÔ Ñ˛¯ÂÌ (45). èË ÂÍÁÓÌ-ÔÂÒÍ‡˜‡˘ËÚÂ ÏÛÚ‡ˆËË ‰ÂÎÂˆËËÚÂ, Ì‡Û¯‡‚‡˘Ë ‡ÏÍ‡Ú‡ Ì‡ ˜ÂÚÂÌÂ ÏÓ„‡Ú ‰‡ ÒÂ ÔÓﬂ‚ﬂ‚‡Ú ÙÂÌÓÚËÔÌÓ, Í‡ÚÓ Ú‡ÍË‚‡ Ò˙Ò Á‡Ô‡ÁÂÌ‡ ‡ÏÍ‡ Ì‡ ˜ÂÚÂÌÂ Ë Ó·‡ÚÌÓ (22). èË èåÑ ÏÛÚ‡ˆËËÚÂ ÒÂ ÛÌ‡ÒÎÂ‰ﬂ‚‡Ú ï-ÂˆÂÒË‚ÌÓ Ë ·ÓÎÂ‰Û‚‡Ú ÓÒÌÓ‚ÌÓ ÏÓÏ˜ÂÚ‡, Á‡˘ÓÚÓ Ò‡ ıÂÏËÁË„ÓÚË ÔÓ ï-ıÓÏÓÁÓÏ‡Ú‡. ÜÂÌËÚÂ Ò‡ ÌÓÒËÚÂÎÍË Ë ÔÂ‰‡‚‡Ú Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ì‡ Ò‚ÓËÚÂ ÒËÌÓ‚Â Ò ËÒÍ ÓÚ 50% ÓÚ ‡Ê‰‡ÌÂ Ì‡ ·ÓÎÌÓ ÏÓÏ˜Â, ‡ 50% ÓÚ ‰˙˘ÂËÚÂ Ì‡ Ú‡Í‡‚‡ ÊÂÌ‡ Ò˙˘Ó Ò‡ ÌÓÒËÚÂÎÍË. Ç ÏÌÓ„Ó Â‰ÍË ÒÎÛ˜‡Ë ÊÂÌË ÏÓ„‡Ú ‰‡ ·˙‰‡Ú Á‡ÒÂ„Ì‡ÚË, ÌÓ Ò ÔÓ-ÎÂÍ‡ ÍÎËÌË˜Ì‡ ËÁﬂ‚‡ ÔË ÒËÌ‰ÓÏ Ì‡ í˙Ì˙ 45ï0, ‡ÒËÏÂÚË˜Ì‡ ËÌ‡ÍÚË‚‡ˆËﬂ Ì‡ Á‰‡‚‡Ú‡ ï-ıÓÏÓÁÓÏ‡ Ë Â‰ÌÓÓ‰ËÚÂÎÒÍ‡ ‰ËÁÓÏËﬂ (2, 4, 30) èÄíéÉÖçÖáÄ çÄ åìëäìãçÄíÄ ìÇêÖÑÄ à äéÉçàíàÇçàíÖ çÄêìòÖçàü èêà èåÑ ÑËÒÚÓÙËÌ˙Ú Â ÔÓÚÂËÌ Ò ÏÓÎÂÍÛÎÌ‡ Ï‡Ò‡ ÓÚ 427 kDa (ÍËÎÓ‰‡ÎÚÓÌ‡). Ç ‡ÁÎË˜ÌËÚÂ ÒË ËÁÓÙÓÏË ÒÂ ÓÚÍË‚‡ ‚ ÏÛÒÍÛÎËÚÂ, ÂÚËÌ‡Ú‡, ÌÂ‚ÓÌËÚÂ Ë „ÎË‡ÎÌËÚÂ ÍÎÂÚÍË ‚ 156 ñçë, ‚ ÍÎÂÚÍËÚÂ Ì‡ ·˙·ÂˆËÚÂ, ˜ÂÌËﬂ ‰Ó·, ÂÚËÌ‡Ú‡, Ô‡ÌÍÂ‡Ò‡, ·ÂÎËÚÂ ‰Ó·Ó‚Â Ë ÚÂÒÚËÒËÚÂ (61). èÓ‚Â˜ÂÚÓ ËÁÓÙÓÏË Ò‡ ÚËÔË˜ÌË Á‡ ‰‡‰ÂÌ ÍÎÂÚ˙˜ÂÌ ÚËÔ, Dp71 ÒÂ ÓÚÍË‚‡ ‚ „ÓÎﬂÏ‡ ˜‡ÒÚ ÓÚ ËÁ·ÓÂÌËÚÂ Ú˙Í‡ÌË, ÌÓ ÌÂ Ë ‚ Ì‡ÔÂ˜ÌÓÌ‡·‡Á‰ÂÌËÚÂ ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡. ÑÛ„ËÚÂ ËÁÓÙÓÏË (Dp71, Dp116, Dp140 Ë Dp260) ËÏ‡Ú ÔÓÏÓÚÓË ‡ÁÎË˜ÌË ÓÚ ÚÂÁË Ì‡ ‰ËÒÚÓÙËÌ‡ Ì‡ Ì‡ÔÂ˜ÌÓÌ‡·‡Á‰ÂÌËÚÂ ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡. ÑËÒÚÓÙËÌ˙Ú Â ˆËÚÓÒÍÂÎÂÚÂÌ ÔÓÚÂËÌ, Ò ‚ËÒÓÍ‡ ÂÍÒÔÂÒËﬂ ‚ ÌÂ‚ÌÓÏÛÒÍÛÎÌËÚÂ ÒËÌ‡ÔÒË Ë ÏÛÒÍÛÎÌÓ-ÒÛıÓÊËÎÌËÚÂ ‚˙ÁÍË (20). ãÓÍ‡ÎËÁË‡Ì Â ÔÓ ‚˙ÚÂ¯Ì‡Ú‡ ÔÓ‚˙ıÌÓÒÚ Ì‡ Ò‡ÍÓÎÂÏ‡Ú‡. ä‡ÚÓ ıÓÏÓÚÂÚ‡ÏÂ (25) ÒÂ Ò˙ÒÚÓË ÓÚ: ● ÉÎÓ·ÛÎ‡ÂÌ ‡ÏËÌÓ-Í‡È, ˜ÂÁ ÍÓÈÚÓ ÒÂ Ò‚˙Á‚‡ Ò˙Ò Ò‡ÍÓÏÂÌËﬂ ·ÂÎÚ˙Í ‡ÍÚËÌ; ● ñÂÌÚ‡ÎÂÌ ‰ÓÏÂÌ Ò 24 ÔÓ‚ÚÓ‡, ÔÓ‰Ó·ÂÌ Ì‡ ˆËÚÓÒÍÂÎÂÚÌËﬂ ÔÓÚÂËÌ ÒÔÂÍÚËÌ. èÓ‚ÚÓËÚÂ ÒÂ ÔÂÍ˙Ò‚‡Ú ÓÚ ˜ÂÚËË, ·Ó„‡ÚË Ì‡ ÔÓÎËÌ ÌÂÔÓ‚Ú‡ﬂ˘Ë ÒÂ Â„ËÓÌË, Ì‡Â˜ÂÌË Ò‚˙Á‚‡˘Ë; ● ÑÓÏÂÌ, ·Ó„‡Ú Ì‡ ˆËÒÚÂËÌ; ● ë-ÚÂÏËÌ‡ÎÂÌ ‰ÓÏÂÌ, Ó·‡ÁÛ‚‡˘ ‰ËÒÚÓÙËÌ-‡ÒÓˆËË‡Ì ÍÓÏÔÎÂÍÒ ˜ÂÁ Ò‚˙Á‚‡ÌÂ Ì‡ ‰ËÒÚÓÙËÌ‡ Ò β-‰ËÒÚÓ„ÎËÍ‡Ì‡. ÑËÒÚÓÙËÌ-‡ÒÓˆËË‡ÌËﬂ „ÎËÍÓÔÓÚÂËÌÓ‚ ÍÓÏÔÎÂÍÒ Â ÒÎÓÊÌ‡ Ì‡‰ÏÓÎÂÍÛÎÌ‡ Ó„‡ÌËÁ‡ˆËﬂ, ‚ÍÎ˛˜‚‡˘‡ ‰ËÒÚÓÙËÌ, ‰ËÒÚÓ„ÎËÍ‡ÌÓ‚, Ò‡ÍÓ„ÎËÍ‡ÌÓ‚ Ë ÒËÌÚÓÙËÌÓ‚ ÒÛ·ÍÓÏÔÎÂÍÒË. íÓÁË ÍÓÏÔÎÂÍÒ Ë„‡Â ÓÎﬂÚ‡ Ì‡ ÏÓÒÚ ÏÂÊ‰Û ˆËÚÓÒÍÂÎÂÚ‡, Ò‡ÍÓÎÂÏ‡Ú‡ Ë ·‡Á‡ÎÌ‡Ú‡ ÏÂÏ·‡Ì‡ Ë Ì‡ ÒÚ‡·ËÎËÁ‡ÚÓ Ì‡ Ò‡ÍÓÎÂÏ‡Ú‡ ÔË ÏÛÒÍÛÎÌ‡ ÍÓÌÚ‡ÍˆËﬂ, Í‡ÚÓ ÔÂ‰Ô‡Á‚‡ ÏËÓÙË·ËÎËÚÂ ÓÚ ÌÂÍÓÁ‡. èË èåÑ ÚËÔ Ñ˛¯ÂÌ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÔÓ˜ÚË Ô˙ÎÌ‡ ÎËÔÒ‡ Ì‡ ‰ËÒÚÓÙËÌ. èË èåÑ ÚËÔ ÅÂÍÂ ÚÓÈ Â ‡·ÌÓÏÂÌ ËÎË Ì‡Ï‡ÎÂÌ. íÂÁË Ù‡ÍÚÓË Ó·ÛÒÎ‡‚ﬂÚ Ì‡Û¯ÂÌ‡Ú‡ ÒÚ‡·ËÎÌÓÒÚ Ì‡ ‰ËÒÚÓÙËÌ „ÎËÍÓÔÓÚÂËÌ-‡ÒÓˆËË‡ÌËﬂ ÍÓÏÔÎÂÍÒ. è‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÔÓÏÂÌËÚÂ ‚ Ì‡ÔÂ˜ÌÓÌ‡·‡Á‰ÂÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ ÔË èåÑ ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÎËÔÒ‡Ú‡ ËÎË ‰ÂÙËˆËÚ‡ Ì‡ ‰ËÒÚÓÙËÌ ÒÂ Ó·ﬂÒÌﬂ‚‡Ú Ò ‰‚Â ÓÒÌÓ‚ÌË ıËÔÓÚÂÁË. ● ä‡ÎˆËÂ‚‡ ıËÔÓÚÂÁ‡ (15): ÑËÒÚÓÙËÌÓ‚ËﬂÚ ‰ÂÙËˆËÚ ‚Ó‰Ë ‰Ó ÒÏÛ˘ÂÌËÂ ‚ Í‡ÎˆËÂ‚‡Ú‡ ıÓÏÂÓÒÚ‡Á‡, ÔÓ‡‰Ë Ì‡Û¯‡‚‡ÌÂ Ì‡ ÙÛÌÍˆËﬂÚ‡ Ì‡ ÏÂÏ·‡ÌÌËÚÂ ÈÓÌÌËÚÂ Í‡Ì‡ÎË. ìÒÚ‡ÌÓ‚ﬂ‚‡ ÒÂ ÔÓ‚Ë¯ÂÌ Í‡ÎˆËÂ‚ ËÌÙÎÛÍÒ ‚ Á‡ÒÂ„Ì‡ÚËÚÂ ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡, ÍÓÈÚÓ ‚ÂÓﬂÚÌÓ ÔË˜ËÌﬂ‚‡ ÚﬂıÌ‡Ú‡ ÌÂÍÓÁ‡, ‚ÒÎÂ‰ÒÚ‚ËÂ ËÌÚÂÌÁËÙËˆË‡ÌÂ Ì‡ ÔÓˆÂÒËÚÂ Ì‡ ÔÓ„‡ÏË‡Ì‡ ÍÎÂÚ˙˜Ì‡ ÒÏ˙Ú. èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â Í‡ÎˆËﬂÚ ‡ÍÚË‚Ë‡ ë‡-Á‡‚ËÒËÏË ÔÓÚÂ‡ÁË Ë ÓÚÍÎ˛˜‚‡ ‚˙ÁÔ‡ÎËÚÂÎÂÌ ÔÓˆÂÒ. ÑËÒÚÓÙËÌ˙Ú Ë„‡Â ÒÚÛÍÚÛÌ‡ ÓÎﬂ Ë ÓÒË„Ûﬂ‚‡ ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂÚÓ ÏÂÊ‰Û Ò‡ÍÓÎÂÏ‡Ú‡ Ë ÂÍÒÚ‡ˆÂÎÛÎ‡ÌËﬂ Ï‡ÚËÍÒ (14). ãËÔÒ‡Ú‡ ÏÛ ‚Ó‰Ë ‰Ó ÓÒÏÓÚË˜Ì‡ ÌÂÒÚ‡·ËÎÌÓÒÚ ÔË ÏÛÒÍÛÎÌÓ Ò˙Í‡˘ÂÌËÂ Ë ÔÓÒÎÂ‰‚‡˘‡ ÌÂÍÓÁ‡. ÑËÒÚÓÙËÌ˙Ú ÒÂ ÓÚÍË‚‡ Ë ‚ Ò˙‰Â˜ÌËÚÂ Ë „Î‡‰ÍËÚÂ ÏÛÒÍÛÎÌË ÍÎÂÚÍË (62). ë ‰ÂÙËˆËÚ‡ Ì‡ ‰ËÒÚÓÙËÌ ÒÂ Ó·ﬂÒÌﬂ‚‡ Í‡‰ËÓÏËÓÔ‡ÚËﬂÚ‡ ÔË 50% ‰Ó 80% ÓÚ èåÑ ÚËÔ Ñ˛¯ÂÌ (73) Ë ‰ËÒÙÛÌÍˆËﬂÚ‡ Ì‡ „Î‡‰Í‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ ‚ ÂÁÛÎÚ‡Ú ÓÚ ÌÂÍÓÁ‡ Ì‡ ÏÛÒÍÛÎÌËÚÂ ‚Î‡ÍÌ‡, ‰Â„ÂÌÂ‡ˆËﬂ Ì‡ ÍÎÂÚ˙˜ÌËÚÂ ËÏ ﬂ‰‡ Ë Ï‡ÒÚÌ‡ ËÌÙËÎÚ‡ˆËﬂ. è‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ ÔË ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Ò˙˘Ó Â Ò‚˙Á‡Ì‡ ÓÒÌÓ‚ÌÓ Ò ‰ËÒÚÓÙËÌÓ‚Ëﬂ ‰ÂÙËˆËÚ. Ç ñçë Ò‡ ËÁÓÎË‡ÌË ‡ÁÎË˜ÌË ËÁÓÙÓÏË Ì‡ ‰ËÒÚÓÙËÌ ÓÚ ÍÓÚËÍ‡ÎÌË ÌÂ‚ÓÌË (10; 16), ÍÎÂÚÍËÚÂ Ì‡ Purkinje, (40), „ÎË‡ÎÌË ÍÎÂÚÍË (40), ¯‚‡ÌÓ‚Ë ÍÎÂÚÍË (20) (í‡·ÎËˆ‡ 1). í‡·ÎËˆ‡ 1. ãÓÍ‡ÎËÁ‡ˆËﬂ Ì‡ ËÁÓÙÓÏËÚÂ Ì‡ ‰ËÒÚÓÙËÌ‡ ‚ ñçë (26,39) Ç ñçë ÒÂ ÂÍÒÔÂÒË‡Ú ‰‚Â ‡ÎÚÂÌ‡ÚË‚ÌË ËÁÓÙÓÏË Ì‡ ‰ËÒÚÓÙËÌ‡ Ò Ô˙ÎÌ‡ ‰˙ÎÊËÌ‡ Ë ÚË- ÍÓ‰Ë‡ÌË ÓÚ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ‰ËÒÚ‡ÎÌ‡Ú‡ ˜‡ÒÚ Ì‡ „ÂÌ‡ (Dp140, Dp71, Dp116). åÓÁ˙˜ÌËﬂÚ ‰ËÒÚÓÙËÌ ËÏ‡ ‚‡ÊÌÓ ÁÌ‡˜ÂÌËÂ Á‡ Ò˙Áﬂ‚‡ÌÂÚÓ Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÌÂ‚ÌËÚÂ ÍÎÂÚÍË. ÇÂÓﬂÚÌÓ Û˜‡ÒÚ‚‡ ‚ ÒËÌ‡ÔÚÓ„ÂÌÂÁ‡Ú‡ Ë ÍÎÂÚ˙˜Ì‡Ú‡ ÏË„‡ˆËﬂ ÔÓ ‚ÂÏÂ Ì‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÌÂ‚Ì‡Ú‡ ÒËÒÚÂÏ‡ (35). àÏ‡ ÚÂÓËË, ˜Â ÔË Á‡‚˙¯ÂÌË ÔÓˆÂÒË Ì‡ ‡Á‚ËÚËÂ Ë Ò˙Áﬂ‚‡ÌÂ, ‰ËÒÚÓÙËÌ˙Ú Û˜‡ÒÚ‚‡ ‚ ‰ËÌ‡ÏË˜Ì‡Ú‡ ÒËÌ‡ÔÚË˜Ì‡ ÏÓ‰ÛÎ‡ˆËﬂ (56; 57) Ë ÔÓ ÚÓÁË Ì‡˜ËÌ ‚ÎËﬂÂ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ ÙÛÌÍˆËË. ë˙˘ÂÒÚ‚Û‚‡Ú ‰ÓÍ‡Á‡ÚÂÎÒÚ‚‡, ˜Â ÎËÔÒ‡Ú‡ ËÎË ‰ÂÙËˆËÚ˙Ú Ì‡ ‰ËÒÚÓÙËÌ ‚Ó‰ﬂÚ ‰Ó ÒÂËÓÁÌË Ì‡Û¯ÂÌËﬂ Ì‡ ‚˙ÚÂÍÎÂÚ˙˜Ì‡Ú‡ Í‡ÎˆËÂ‚‡ ıÓÏÂÓÒÚ‡Á‡. íÓ‚‡ ÏÓÊÂ ‰‡ ÒÏÛÚË Â„ÛÎ‡ˆËﬂÚ‡ Ì‡ ÍÎÂÚ˙˜ÌËﬂ ˆËÍ˙Î, ÊËÁÌÂÒÔÓÒÓ·ÌÓÒÚÚ‡ Ì‡ ÍÎÂÚÍËÚÂ, ÌÂ‚Ó·Î‡ÒÚÌ‡Ú‡ ÏË„‡ˆËﬂ ‚ ÔÂËÓ‰‡ Ì‡ ‡Á‚ËÚËÂ Ë ÔÓÒÎÂ‰‚‡˘‡Ú‡ ÒËÌ‡ÔÚË˜Ì‡ ÒË„Ì‡ÎËÁ‡ˆËﬂ ‚ ÔÂËÓ‰‡ Ì‡ ÁﬂÎÓÒÚ Ì‡ çë (36). KÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ Ë ÛÏÒÚ‚ÂÌ ‰ÂÙËˆËÚ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú Ò ‚‡Ë‡·ËÎÌ‡ ˜ÂÒÚÓÚ‡ ÔË ·ÓÎÌËÚÂ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËË (26, 74) Ë Ò‡ ÔÓ-˜ÂÒÚË ÔË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ, ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ (8). äÓ„ÌËÚË‚ÌËÚÂ ÒÏÛ˘ÂÌËﬂ Á‡Òﬂ„‡Ú ÏÂÊ‰Û 20% Ë 50% ÓÚ ·ÓÎÌËÚÂ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò 9% Ô‡ˆËÂÌÚË Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ ÓÚ Ó·˘‡Ú‡ ÔÓÔÛÎ‡ˆËﬂ (35; 68). èË ·ÓÎÌË Ò èåÑ ÚËÔ ÅÂÍÂ Ò˙˘Ó ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ë ÔÒËıË˜ÂÒÍË ÓÚÍÎÓÌÂÌËﬂ (26, 74). äÓ„ÌËÚË‚ÌËﬂÚ ‰ÂÙËˆËÚ ÌÂ ÍÓÂÎË‡ Ò ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÌÂ ÔÓ„ÂÒË‡ (16; 40; 58). á‡ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÚÓÁË ÚËÔ ÒÏÛ˘ÂÌËﬂ ÔË ÚÂÁË Ô‡ˆËÂÌÚË Ò˙˘ÂÒÚ‚Û‚‡Ú ‡ÁÎË˜ÌË ıËÔÓÚÂÁË, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ ÒÂ „ÛÔË‡Ú ‚ 3 ÓÒÌÓ‚ÌË Ì‡Ô‡‚ÎÂÌËﬂ: ‰ÂÙËˆËÚ, ÎËÔÒ‡ ËÎË ‡·ÌÓÏÂÌ ‰ËÒÚÓÙËÌ ‚ ÌÂ‚ÓÌËÚÂ Ì‡ ñçë, Á‡ ÍÓÂÚÓ Ò‡ ÔÓ‚ÂÊ‰‡ÌË Ì‡È-ÏÌÓ„Ó ËÁÒÎÂ‰‚‡ÌËﬂ; ÏÛÚ‡ˆËË ‚ „ÂÌË, ÎÓÍ‡ÎËÁË‡ÌË ‚ Ò˙ÒÂ‰ÒÚ‚Ó Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ ‚ ï ıÓÏÓÁÓÏ‡Ú‡; ÏÓÁ˙˜Ì‡ ıËÔÓÍÒËﬂ ‚ÒÎÂ‰ÒÚ‚ËÂ Ì‡ ‰Ëı‡ÚÂÎÌ‡Ú‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ. ‡) ÑÂÙËˆËÚ, ÎËÔÒ‡ ËÎË ‡·ÌÓÏÂÌ ‰ËÒÚÓÙËÌ ‚ ÌÂ‚ÓÌËÚÂ Ì‡ ñçë ● åÛÚ‡ˆËËÚÂ (‰ÂÎÂˆËË Ë ‰ÛÔÎËÍ‡ˆËË) ‚˙‚ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚ËÌ‡ Ì‡ „ÂÌ‡ ÔÓ-˜ÂÒÚÓ ‚Ó‰ﬂÚ ‰Ó ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËﬂ (18; 58), Ï‡Í‡ ˜Â Ë ÔË Ô‡ˆËÂÌÚË Ò ÏÛÚ‡ˆËË ‚ ÔÓÍÒËÏ‡ÎÌ‡Ú‡ ˜‡ÒÚ Ì‡ „ÂÌ‡ Ò˙˘Ó ÏÓÊÂ ‰‡ ÒÂ ÛÒÚ‡ÌÓ‚Ë ‡ÁÎË˜ÂÌ ÔÓ ÒÚÂÔÂÌ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ. ● ãËÔÒ‡Ú‡ Ì‡ Ô˙ÎÌÓ‚ÂËÊÂÌ ‰ËÒÚÓÙËÌ ÏÓÊÂ ‰‡ ‰Ó‚Â‰Â ‰Ó ÛÏÂÂÌÓ Ì‡Û¯ÂÌËÂ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ ÙÛÌÍˆËË(21; 33; 53; 58). ● à‰ÂÌÚËÙËˆË‡ÌË Ò‡ ÚË ‰ËÒÚ‡ÎÌË ‰ËÒÚÓÙËÌÓ‚Ë ËÁÓÙÓÏË: Dp140, Dp71, Dp116. - Dp140 Â ÏÓÁ˙˜Ì‡ ‰ËÒÚ‡ÎÌ‡ ËÁÓÙÓÏ‡, ÂÍÒÔÂÒË‡˘‡ ÒÂ ÔÓ ‚ÂÏÂ Ì‡ ÂÏ·ËÓÌ‡ÎÌËﬂ ÔÂËÓ‰ Ì‡ ‡Á‚ËÚËÂ (8; 55; 58). éÚÍË‚‡ ÒÂ Ë ‚ ·˙·ÂˆËÚÂ. ë˙ÒÚÓË ÒÂ ÓÚ ‰ËÒÚ‡ÎÌ‡Ú‡ ˜‡ÒÚ Ì‡ ˆÂÌÚ‡ÎÌËﬂ ‰ÓÏÂÌ Ë ÓÚ ë-ÚÂÏËÌ‡ÎÌËﬂ ‰ÓÏÂÌ, ‚ÍÎ˛˜‚‡ Ú. Ì‡. Ò‚˙Á‚‡˘ Â„ËÓÌ, ÍÓÈÚÓ ÏÓÊÂ ‰‡ Û˜‡ÒÚ‚‡ ‚ ÍÎÂÚ˙˜Ì‡Ú‡ ÒË„Ì‡ÎËÁ‡ˆËﬂ ËÎË ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂÚÓ Ò ‰Û„Ë ÔÓÚÂËÌË. èË Dp140 Ì‡˜‡ÎÌËﬂÚ Â„ËÓÌ Ì‡ Ú‡ÌÒÎ‡ˆËﬂ ÒÂ Ì‡ÏË‡ ‚ ÂÍÁÓÌ 51, ÌÓ ÌÂ„Ó‚ËﬂÚ ÔÓÏÓÚÓ Ë Ô˙‚ËﬂÚ ÂÍÁÓÌ ÒÂ Ì‡ÏË‡Ú ‚ „ÓÎÂÏËﬂ ËÌÚÓÌ ÏÂÊ‰Û 44 Ë 45 ÂÍÁÓÌË. íÓ‚‡ Â Â„ËÓÌ, ‚ ÍÓÈÚÓ ˜ÂÒÚÓ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ‰ÂÎÂˆËË ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ (33). - Dp71 Â ÓÒÌÓ‚Ì‡Ú‡ ‰ËÒÚÓÙËÌÓ‚‡ ËÁÓÙÓÏ‡, ÂÍÒÔÂÒË‡Ì‡ ‚ ÏÓÁ˙Í‡ (19; 69; 71). èË Dp71 ÔÓÏÓÚÓ˙Ú Â ÎÓÍ‡ÎËÁË‡Ì ‚ ËÌÚÓÌ 62, ‡ Ì‡˜‡ÎÌËﬂÚ Â„ËÓÌ Ì‡ Ú‡ÌÒÎ‡ˆËﬂ Á‡ÔÓ˜‚‡ ÓÚ ÂÍÁÓÌ 63 (13). Dp71 Â ÚﬂÒÌÓ Ò‚˙Á‡Ì Ò˙Ò ÒËÌ‡ÔÚË˜Ì‡Ú‡ ÔÎ‡ÁÏÂÌ‡ ÏÂÏ·‡Ì‡ Ë Ú‡Í‡ ‚ÂÓﬂÚÌÓ Û˜‡ÒÚ‚‡ ‚ ÔÂ‰‡‚‡ÌÂÚÓ Ì‡ ÌÂ‚ÌËﬂ ËÏÔÛÎÒ (58). èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â ÔÓ‡‰Ë ‡ÌÌ‡Ú‡ ÏÛ ÂÍÒÔÂÒËﬂ ‚ ÌÂ‚ÓÌËÚÂ ÔÓ ‚ÂÏÂ Ì‡ ÂÏ·ËÓÌ‡ÎÌÓÚÓ ‡Á‚ËÚËÂ, ÚÓÈ ËÏ‡ ÓÒÌÓ‚ÓÔÓÎ‡„‡˘‡ ÓÎﬂ ‚ ÏÓÁ˙˜ÌÓ- 157 í‡·ÎËˆ‡ 1. ãÓÍ‡ÎËÁ‡ˆËﬂ Ì‡ ËÁÓÙÓÏËÚÂ Ì‡ ‰ËÒÚÓÙËÌ‡ ‚ ñçë (26,39) àÁÓÙÓÏË äÎÂÚ˙˜Ì‡ ÎÓÍ‡ÎËÁ‡ˆËﬂ (ÓÎﬂ) Ì‡ ‰ËÒÚÓÙËÌ‡ Dp427M ÉÎË‡ÎÌË ÍÎÂÚÍË Ë Ò˙‰Ó‚Ë ÂÌ‰ÓÚÂÎÌË ÍÎÂÚÍË Dp427C äÓÚËÍ‡ÎÌË Ë ıËÔÓÍ‡ÏÔ‡ÎÌË ÔË‡ÏË‰ÌË ÌÂ‚ÓÌË Dp427P îÂÚ‡ÎÂÌ ˆÂÂ·‡ÎÂÌ ÍÓÚÂÍÒ ÍÎÂÚÍË Ì‡ Purkinje ‚ ˆÂÂ·ÂÎÛÏ‡ Dp260 êÂÚËÌ‡ Dp140 ● ÄÒÚÓ„ÎË‡ÎÌË ÔÓˆÂÒË ÔÓ ‚ÂÏÂ Ì‡ ‡Á‚ËÚËÂÚÓ ● èÓ‚˙ıÌÓÒÚ Ì‡ ÎÂÔÚÓÏÂÌËÌ„ËÚÂ ● èÓ ıÓ‰‡ Ì‡ ÔÓÌËÍ‚‡˘ËÚÂ Í˙‚ÓÌÓÒÌË Ò˙‰Ó‚Â ● çÂ‚ÓıËÔÓÙËÁ‡ Dp116 ● Å‡Á‡ÎÌË „‡Ì„ÎËË ● åËÂÎËÌÓ‚ËÚÂ Ó·‚Ë‚ÍË Ì‡ ÔÂËÙÂÌËÚÂ ÌÂ‚Ë Dp71 ● ê‡ÌÌË ÂÏ·ËÓÌ‡ÎÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË ● åÂÁÂÌıËÏÌÓ-ÂÔËÚÂÎÌË ‚˙ÁÍË ● ÖÏ·ËÓÌ‡ÎÂÌ ÏÓÁ˙˜ÂÌ ÒÚ‚ÓÎ ● ÑËÙÂÂÌˆË‡ˆËﬂ Ì‡ ÏÓÁ˙Í‡ ‚ ÔÂËÌ‡Ú‡ÎÌËﬂ ÔÂËÓ‰ ● êÂÚËÌ‡ ● ÖÏ·ËÓÌ‡ÎÂÌ ÔÂ‰ÂÌ ÏÓÁ˙Í ● åÓÁ˙˜Ì‡ ÍÓ‡ ● á˙ÌÂÒÚË ÍÎÂÚÍË ‚ ıËÔÓÍ‡ÏÔ‡ Ë gyrus dentatus ● ïËÔÓÙËÁ‡ ÚÓ Ò˙Áﬂ‚‡ÌÂ (47; 50). éÒÌÓ‚Ì‡Ú‡ ÎÓÍ‡ÎËÁ‡ˆËﬂ Ì‡ Dp71 Â ‚ ıËÔÓÍ‡ÏÔ‡ÎÌËﬂ gyrus dentatus, ÍÓÂÚÓ Ì‡Î‡„‡ Òı‚‡˘‡ÌÂÚÓ, ˜Â Ú‡ÁË ‰ËÒÚÓÙËÌÓ‚‡ ËÁÓÙÓÏ‡ Â Ò‚˙Á‡Ì‡ Ò Ô‡ÏÂÚÓ‚ËÚÂ ÙÛÌÍˆËË (41). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÏÛÚ‡ˆËË, Á‡Òﬂ„‡˘Ë ÔÓÏÓÚÓÌËﬂÚ Û˜‡ÒÚ˙Í Ì‡ Dp71„ÂÌ‡, ÔÓ„Â¯ÌÓÒÏËÒÎÂÌË ÏÛÚ‡ˆËË Ë Ú‡ÍË‚‡, ‚Ó‰Â¯Ë ‰Ó ÚÂÏËÌ‡ˆËﬂ Ì‡ Ú‡ÌÒÎ‡ˆËﬂÚ‡ Ò‡ Ò‚˙Á‡ÌË Ò ÛÏÂÂÌÓ ËÎË ÚÂÊÍÓ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ Ì‡ Ô‡ˆËÂÌÚËÚÂ. - äÓ‰Ë‡˘‡Ú‡ ÒÂÍ‚ÂÌˆËﬂ Á‡ Dp116 Á‡ÔÓ˜‚‡ ÓÚ ÂÍÁÓÌ 57. Dp116 ‰ËÒÚÓÙËÌ˙Ú ‚ÍÎ˛˜‚‡ ‰ËÒÚ‡ÎÌ‡Ú‡ ˜‡ÒÚ ÓÚ ˆÂÌÚ‡ÎÌËﬂ ‰ÓÏÂÌ (‰‚‡ Ô˙ÎÌË ÔÓ‚ÚÓ‡), ·Ó„‡ÚËﬂ Ì‡ ˆËÒÚÂËÌ Â„ËÓÌ Ë Í‡·ÓÍÒË-ÚÂÏËÌ‡Î‡. ãËÔÒ‡Ú‡ Ì‡ ‡ÍÚËÌ-Ò‚˙Á‚‡˘ ‰ÓÏÂÌ ÔÂ‰ÔÓÎ‡„‡, ˜Â ÓÒÌÓ‚Ì‡Ú‡ ÙÛÌÍˆËﬂ Ì‡ Ú‡ÁË ËÁÓÙÓÏ‡ ÒË„Ì‡ÎÌ‡Ú‡ Ú‡ÌÒ‰ÛÍˆËﬂ Ë ÌﬂÏ‡ ÁÌ‡˜ËÏ‡ ÓÎﬂ Á‡ ÏÂı‡ÌË˜Ì‡Ú‡ ÒÚ‡·ËÎËÁ‡ˆËﬂ Ì‡ Ò‡ÍÓÎÂÏ‡Ú‡ Ì‡ ÏÛÒÍÛÎÌËÚÂ ÍÎÂÚÍË. éÚÍË‚‡ ÒÂ ‚ ‚ ·‡Á‡ÎÌËÚÂ „‡Ì„ÎËË ÔÓ ‚ÂÏÂ Ì‡ Í˙ÒÌËÚÂ ÂÚ‡ÔË Ì‡ ÂÏ·ËÓÌ‡ÎÌÓÚÓ ‡Á‚ËÚËÂ Ì‡ ÏÓÁ˙Í‡. (57; 59). àÁÒÎÂ‰‚‡ÌËﬂ, Ì‡Ô‡‚ÂÌË ‚ ÔÓÒÎÂ‰ÌËÚÂ „Ó‰ËÌË ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÍÓÂÎ‡ˆËﬂ ÏÂÊ‰Û ÍÓ„ÌËÚË‚ÌËﬂ ‰ÂÙËˆËÚ Ë ÏÛÚ‡ˆËËÚÂ ‚ Dp71, Dp116 Ë Dp140 ËÁÓÙÓÏËÚÂ. çÂ ﬂ‰ÍÓ ‰ÂÎÂˆËË ‚ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ Á‡Òﬂ„‡Ú Â„ÛÎ‡ÚÓÌËﬂ Â„ËÓÌ Ì‡ Dp71 Ë ÍÓ‰Ë‡˘ËÚÂ ÒÂÍ‚ÂÌˆËË Ì‡ Dp116 Ë Dp140, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, ÔÓ‡‰Ë ÎËÔÒ‡Ú‡ Ì‡ ÏÓÁ˙˜ÌËÚÂ ËÁÓÙÓÏË ‚ ÌÂ‚ÓÌËÚÂ (35). çﬂÍÓË ‡‚ÚÓË ÔÓ‰˜ÂÚ‡‚‡Ú ÚﬂÒÌ‡Ú‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ÏÛÚ‡ˆËËÚÂ ‚ Â„ÛÎ‡ÚÓÌËﬂ Â„ËÓÌ Ì‡ Dp140 Ë ÛÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡ÌÂ ÔË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ ÅÂÍÂ (9). ÑÂÙËˆËÚ˙Ú ËÎË ÎËÔÒ‡Ú‡ Ì‡ ‰ËÒÚÓÙËÌ ‚ÂÓﬂÚÌÓ Ò‡ ‚ ÓÒÌÓ‚‡Ú‡ Ì‡ ÏÓÁ˙˜ÌËÚÂ ÒÚÛÍÚÛÌË ‡·ÌÓÏÌÓÒÚË ÔË Ú‡ÍË‚‡ Ô‡ˆËÂÌÚË: Á‡„Û·‡ Ì‡ ÌÂ‚ÓÌË, ıÂÚÂÓÚÓÔËË, „ÎËÓÁ‡ ÌÂ‚ÓÙË·ËÎÂÌË ‚ÍÎ˛˜‚‡ÌËﬂ, Á‡„Û·‡ Ì‡ ÍÎÂÚÍË Ì‡ Purkinje, ‰ÂÌ‰ËÚÌË ‡·ÌÓÏÌÓÒÚË, Ì‡Û¯ÂÌ‡ ˆËÚÓ‡ıËÚÂÍÚÓÌËÍ‡, ÔÂËÌÛÍÎÂ‡Ì‡ ‚‡ÍÛÓÎËÁ‡ˆËﬂ (6; 46). ● èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â ‰ËÒÚÓÙËÌÓÔÓ‰Ó·ÌËÚÂ Ë ‰ËÒÚÓÙËÌ-‡ÒÓˆËË‡ÌËÚÂ ÔÓÚÂËÌË Ò˙˘Ó ËÏ‡Ú ÓÚÌÓ¯ÂÌËÂ Í˙Ï ÍÓ„ÌËÚË‚ÌËÚÂ ÙÛÌÍˆËË. íÓ‚‡ Ò‡ Î‡ÏËÌËÌ, ÏÂÓÁËÌ, ÔÂÎÂÍ‡Ì, ‡„ËÌ, ·Ë„ÎËÍ‡Ì Ë ÌÓ‚ÓÓÚÍËÚËﬂÚ ÌÂ‚ÂÍÒËÌ, ÍÓËÚÓ ËÏ‡Ú ÓÚÌÓ¯ÂÌËÂ Í˙Ï ÏÂÊ‰ÛÍÎÂÚ˙˜Ì‡Ú‡ ÒË„Ì‡ÎËÁ‡ˆËﬂ (42; 66; 69). ÑËÒÚÓÙËÌ‡ÒÓˆËË‡ÌËÚÂ ÔÓÚÂËÌË α Ë β-‰ËÒÚÓ·Â‚ËÌ Ò‡ ‡ÁÔÓÒÚ‡ÌÂÌË ‚ ÏÓÁ˙Í‡. α-ÑËÒÚÓ·Â‚ËÌ˙Ú ÒÂ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ËÁÓÎË‡ ÓÚ „ÎË‡ÎÌËÚË ÍÎÂÚÍË, ‰ÓÍ‡ÚÓ β-‰ËÒÚÓ·Â‚ËÌ˙Ú- ÓÚ ÌÂ‚ÓÌË Ë ËÏ‡ ‚ÂÓﬂÚÌ‡ ÒË„Ì‡ÎÌ‡ ÙÛÌÍˆËﬂ. ÑËÒ·Ë‰ËÌ˙Ú Â Ò‚˙Á‡Ì Ò β-‰ËÒÚÓ·Â‚ËÌ‡ ‚ ÏÓÁ˙Í‡ Ë Ò α-‰ËÒÚÓ·Â‚ËÌ‡ ‚ Ì‡ÔÂ˜ÌÓÌÓ·‡Á‰ÂÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡. ● êÂ‰ÛˆË‡Ì Â „Î˛ÍÓÁÌËﬂÚ ÏÂÚ‡·ÓÎËÁ˙Ï ‚ Ó·Î‡ÒÚË Ì‡ ñçë Ò ‚ËÒÓÍ‡ ÂÍÒÔÂÒËﬂ Ì‡ ‰ËÒÚÓÙËÌ Í‡ÚÓ ÏÂ‰Ë‡ÎÌËÚÂ ÚÂÏÔÓ‡ÎÌË ÒÚÛÍÚÛË, Ï‡ÎÍËﬂ ÏÓÁ˙Í, ÒÂÌÁÓÏÓÚÓÌËﬂ Ë Î‡ÚÂ‡ÎÌËﬂ ÚÂÏÔÓ‡ÎÂÌ ÍÓÚÂÍÒ ‚ ‰ﬂÒÌ‡Ú‡ ıÂÏËÒÙÂ‡. êÓÎﬂÚ‡ Ì‡ ‰ËÒÚÓÙËÌ‡ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ „Î˛ÍÓÁÌËﬂ ÏÂÚ‡·ÓÎËÁ˙Ï ÓÒÚ‡‚‡ ÌÂﬂÒÌ‡. ë˙˘ÂÒÚ‚Û‚‡ ÚÂÓËﬂ, ÒÔÓÂ‰ ÍÓﬂÚÓ ‰ËÒÚÓÙËÌ˙Ú ÏÓÊÂ ‰‡ Û˜‡ÒÚ‚‡ ‚ ÒË„Ì‡ÎÌ‡Ú‡ Ú‡ÌÒ‰ÛÍˆËﬂ ÓÚ ÂÍÒÚ‡ˆÂÎÛÎ‡ÌÓÚÓ ÔÓÒÚ‡ÌÒÚ‚Ó Í˙Ï ÍÎÂÚ˙˜ÌÓÚÓ ﬂ‰Ó. ● èÓ‚Ë¯ÂÌÓ Â ÌË‚ÓÚÓ Ì‡ ÌÂÓ„‡ÌË˜ÌËÚÂ ÙÓÒÙ‡ÚË Ë Â Ì‡Ï‡ÎÂÌÓ ÍÓÎË˜ÂÒÚ‚ÓÚÓ Ì‡ Äíî ‚ ÌÂ‚ÌËÚÂ ÍÎÂÚÍË. ìÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒÂ ‚ËÒÓÍË ÌË‚‡ Ì‡ ıÓÎËÌ-Ò˙‰˙Ê‡˘Ë ÒÛ·ÒÚ‡ÌˆËË ‚˙‚ ÙÓÌÚ‡ÎÌËﬂ ÍÓÚÂÍÒ (7). ● èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â Ò˙˘ÂÒÚ‚Û‚‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ÙÛÌÍˆËÓÌË‡ÌÂÚÓ Ì‡ ÉÄåäÄ ÂˆÂÔÚÓËÚÂ („‡Ï‡-‡ÏËÌÓÏ‡ÒÎÂÌ‡ ÍËÒÂÎËÌ‡) Ë ÏÓÁ˙˜ÌËÚÂ ‰ËÒÚÓÙËÌË, ÛÒÚ‡ÌÓ‚ÂÌ‡ ÔË ÓÔËÚÌË ÏË¯ÍË-ÏÓ‰ÂÎË. íÂÁË ÂˆÂÔÚÓË ËÏ‡Ú ÁÌ‡˜ÂÌËÂ Á‡ ÔÓ‰˙Ê‡ÌÂÚÓ Ì‡ ÓÚ‰ÂÎÌËÚÂ Ù‡ÁË Ì‡ Ò˙Ìﬂ, ‚˙Á·Û‰ËÏÓÒÚÚ‡ Ë ‡ÍÚË‚ÌÓÒÚÚ‡ Ì‡ ÌÂ‚ÓÌËÚÂ. ëÎÂ‰Ó‚‡ÚÂÎÌÓ ‰ËÒÙÛÌÍˆËﬂÚ‡ Ì‡ ÉÄåäÄ ÂˆÂÔÚÓËÚÂ ÏÓÊÂ ‰‡ Â ÔË˜ËÌ‡ Á‡ ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËË (49). ·) åÛÚ‡ˆËË ‚ „ÂÌË, ÎÓÍ‡ÎËÁË‡ÌË ‚ Ò˙ÒÂ‰ÒÚ‚Ó Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ ‚ ï ıÓÏÓÁÓÏ‡Ú‡, ÍÓËÚÓ ËÏ‡Ú ÁÌ‡˜ÂÌËÂ Á‡ ÛÏÒÚ‚ÂÌÓÚÓ ‡Á‚ËÚËÂ ( XLRM, ACSL4, NXT2, GUCY2F, KCNE1L)( 12). ‚) åÓÁ˙˜Ì‡ ıËÔÓÍÒËﬂ, ‚ÒÎÂ‰ÒÚ‚ËÂ ÔÓ„ÂÒË‡˘‡Ú‡ ÒÎ‡·ÓÒÚ Ì‡ ‰Ëı‡ÚÂÎÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ (37) ËÏ‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ÁÌ‡˜ÂÌËÂ Ì‡ ÔÓ-Í˙ÒÌËÚÂ ÂÚ‡ÔË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÒÎÂ‰ Á‡„Û·‡Ú‡ Ì‡ Ò‡ÏÓÒÚÓﬂÚÂÎÌ‡ ÔÓıÓ‰Í‡. äãàçàóçÄ ïÄêÄäíÖêàëíàäÄ çÄ ÑàëíêéîàçéèÄíààíÖ èåÑ íàè ÑûòÖç èÓ˜ÚË Ô˙ÎÌ‡Ú‡ ÎËÔÒ‡ Ì‡ ‰ËÒÚÓÙËÌ ‚ Ì‡ÔÂ˜ÌÓÌ‡·‡Á‰ÂÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ ÓÔÂ‰ÂÎﬂ ı‡‡ÍÚÂÌ‡Ú‡ Ë ‰Ó·Â ËÁ‚ÂÒÚÌ‡ ÍÎËÌËÍ‡ Ì‡ èåÑ ÚËÔ Ñ˛¯ÂÌ Ò ‡ÌÂÌ Ë ·˙ÁÓ ÔÓ„ÂÒË‡˘ ÒËÏÂÚË˜ÂÌ ÏËÓÔ‡ÚÂÌ ÒËÌ‰ÓÏ Ò ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ Ò Ì‡˜‡ÎÓ ÔÂ‰Ë 5-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ Ë Á‡„Û·‡ Ì‡ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ ıÓ‰ﬂÚ Ò‡ÏÓÒÚÓﬂÚÂÎÌÓ ÔÂ‰Ë 13 „.‚˙Á‡ÒÚ. ÄÌ„‡ÊË‡ ÒÂ ÔÂ‰ËÏÌÓ ÔÓÍÒËÏ‡ÎÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ Ô˙‚Ó Ì‡ ‰ÓÎÌËÚÂ Í‡ÈÌËˆË, ‡ ‚ ÔÓÒÎÂ‰ÒÚ‚ËÂ Ë Ì‡ „ÓÌËÚÂ Í‡ÈÌËˆË, Ô‡‡‚ÂÚÂ·‡ÎÌ‡Ú‡ Ë ¯ËÈÌ‡ ÏÛÒÍÛÎ‡ÚÛ‡. ï‡Ô‡ÍÚÂÌË Ò‡ ÔÒÂ‚‰ÓıËÔÂÚÓÙËﬂ Ì‡ m. triceps surae, ‡ Ì‡ ÔÓ-Í˙ÒÌËÚÂ ÂÚ‡ÔË ‡ÚÓÙËËÚÂ Ì‡ ‰ËÒÚ‡ÎÌËÚÂ ÏÛÒÍÛÎË Ì‡ ‰ÓÎÌËÚÂ, „ÓÌËÚÂ Í‡ÈÌËˆË Ë ÙÎÂÍÒÓËÚÂ Ì‡ ¯ËﬂÚ‡, ÍÓÌÚ‡ÍÚÛËÚÂ Ì‡ „ÎÂÁÂÌÌËÚÂ ÒÚ‡‚Ë Ë ‡ıËÎÂÒÓ‚ÓÚÓ ÒÛıÓÊËÎËÂ, ‡ ‚ ÔÓÒÎÂ‰ÒÚ‚ËÂ Ë ÍÓÎÂÌÌË, Î‡Í˙ÚÌË, ‡ÏÂÌÌË, Ú‡ÁÓ·Â‰ÂÌË Ë ËÌÚÂ‚ÂÚÂ·‡ÎÌË ÒÚ‡‚Ë (1). äËÙÓÒÍÓÎËÓÁ‡Ú‡ Ë ÒÎ‡·ÓÒÚÚ‡ Ì‡ ËÌÚÂÍÓÒÚ‡ÎÌËÚÂ ÏÛÒÍÛÎË Ë ‰Ë‡Ù‡„Ï‡Ú‡ Ì‡Û¯‡‚‡Ú ‚˙Ì¯ÌÓÚÓ ‰Ë¯‡ÌÂ. ê‡Á‚Ë‚‡ ÒÂ ÂÒÚËÍÚË‚ÂÌ ÚËÔ Ì‡Û¯ÂÌËÂ Ì‡ ‰Ë¯‡ÌÂÚÓ, ÔÓ„ÂÒË‡˘‡ ıËÔÓÍÒÂÏËﬂ Ë ıËÔÂÍ‡ÔÌËﬂ Ò ÎÂÒÌ‡ ÛÏÓﬂÂÏÓÒÚ, Ì‡Û¯ÂÌËﬂ Ì‡ Ò˙Ìﬂ Ë „Î‡‚Ó·ÓÎËÂ. ç‡‡ÒÚ‚‡ ËÒÍ˙Ú ÓÚ ÒÏ˙Ú, ÔÓ‡‰Ë ÓÒÚ‡ ‰Ëı‡ÚÂÎÌ‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÔÓ‚ÓÍË‡Ì‡ ÓÚ ËÌÚÂÍÛÂÌÚÌË ËÌÙÂÍˆËË. è‡ˆËÂÌÚËÚÂ Ò‡ Ò˙Ò ÒÌËÊÂÌ ‚ËÚ‡ÎÂÌ Í‡Ô‡ˆËÚÂÚ, ˜ÂÒÚË ÔÌÂ‚ÏÓÌËË Ë ‡Á‚Ë‚‡Ú ‰Ëı‡ÚÂÎÌ‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÍÓÂÚÓ Ì‡Î‡„‡ ‰Ëı‡ÚÂÎÌ‡ 158 Â‡ÌËÏ‡ˆËﬂ ‚ Í‡ÈÌËÚÂ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡. ä‡‰ËÓÏËÓÔ‡ÚËﬂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÔË 50% ‰Ó 80% ÓÚ Ô‡ˆËÂÌÚËÚÂ ‚ ıÓ‰‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ò Í‡‰ËÓÏÂ„‡ÎËﬂ, ÔÂÒËÒÚË‡˘‡ Ú‡ıËÍ‡‰Ëﬂ, ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËﬂ Ë ÓÒÚË ÏËÓÍ‡‰ÌË ËÌÙ‡ÍÚË (73). ëËÏÔÚÓÏËÚÂ ÓÚ ‰ËÒÙÛÌÍˆËﬂ Ì‡ „Î‡‰Í‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ ÒÂ ËÁﬂ‚ﬂ‚‡Ú Ò ıËÔÓÏÓÚËÎËÚÂÚ Ì‡ ÒÚÓÏ‡ı‡ Ë/ËÎË ı‡ÌÓÔÓ‚Ó‰‡, Ï‡Î‡·ÒÓ·ˆËÓÌÂÌ ÒËÌ‰ÓÏ, ÏÂ„‡ÍÓÎÓÌ Ë ÍÓÌÒÚËÔ‡ˆËﬂ. èË „ÓÎﬂÏ‡ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ. ëÂ‰ÌËﬂÚ ÍÓÂÙËˆËÂÌÚ Ì‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ (äà) Â 85, Ò ‚‡Ë‡ˆËË ÏÂÊ‰Û 40 Ë 130 (23, 59). èË·ÎËÁËÚÂÎÌÓ 25% Ò‡ Ò äà ÔÓ‰ 70. èÓ-ÚÂÊÍÓ Ò‡ Á‡ÒÂ„Ì‡ÚË ‚Â·‡ÎÌËÚÂ ÛÏÂÌËﬂ, ‚ Ò‡‚ÌÂÌËÂ Ò ÌÂ‚Â·‡ÎÌËÚÂ (58). íÓÁË ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ ÒÂ Ò‚˙Á‚‡ Ò Ì‡Û¯ÂÌËﬂ ‚ ÌÓÏ‡ÎÌ‡Ú‡ ‡ıËÚÂÍÚÓÌËÍ‡ Ì‡ ÍÓÚËÍ‡ÎÌËÚÂ ÌÂ‚ÓÌË Ë ÚÂıÌËÚÂ ‰ÂÌ‰ËÚË, ÍÓËÚÓ ÌÓÏ‡ÎÌÓ ÂÍÒÔÂÒË‡Ú ‰ËÒÚÓÙËÌ. ç‡ ÌÂ‚ÓËÁÓ·‡Áﬂ‚‡˘ËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËﬂ. íÂÊÂÒÚÚ‡ Ì‡ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ ÌÂ ÍÓÂÎË‡ Ò ÚÂÊÂÒÚÚ‡ Ì‡ ÏÛÒÍÛÎÌÓ Á‡Òﬂ„‡ÌÂ Ë Á‡ ‡ÁÎËÍ‡ ÓÚ ÏÛÒÍÛÎÌ‡Ú‡ ÒÎ‡·ÓÒÚ ÌÂ ÔÓ„ÂÒË‡ Ò ‚ÂÏÂÚÓ (72). Hinton Ë Ò˙‡‚Ú. (2000) ËÁÍ‡Á‚‡Ú ÚÂÓËﬂÚ‡, ˜Â ÔÓ-ÚÂÊÍÓÚÓ Ì‡Û¯ÂÌËÂ Ì‡ ‚Â·‡ÎÌËÚÂ ÛÏÂÌËﬂ Â ÂÁÛÎÚ‡Ú ÓÚ Á‡Òﬂ„‡ÌÂ Ì‡ Í‡ÚÍÓÒÓ˜Ì‡Ú‡ Ô‡ÏÂÚ. Ñ˙Î„ÓÒÓ˜Ì‡Ú‡ Ô‡ÏÂÚ ÌÂ Â ÚÂÊÍÓ Á‡ÒÂ„Ì‡Ú‡. Wicksell Ë Ò˙‡‚Ú.(2004) ÓÚÍË‚‡Ú ‰ÂÙËˆËÚ ‚ ‡ÍÚË‚Ì‡Ú‡ ‡·ÓÚÌ‡ Ô‡ÏÂÚ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË. íÂ ËÁÍ‡Á‚‡Ú ÚÂÓËﬂ Á‡ Â‰Ì‡Í‚Ó ÚÂÊÍÓ Á‡Òﬂ„‡ÌÂ Ì‡ ÍÓÌÒÚÛÍÚË‚ÌËÚÂ Ë ‚Â·‡ÎÌËÚÂ ÛÏÂÌËﬂ. è‡ˆËÂÌÚËÚÂ ËÁÓÒÚ‡‚‡Ú ‚ ÔÓˆÂÒ‡ Ì‡ Ì‡Û˜‡‚‡ÌÂ ‰‡ ˜ÂÚ‡Ú, ÔË¯‡Ú Ë ‚ Ï‡ÚÂÏ‡ÚË˜ÂÒÍËÚÂ ÒË ÛÏÂÌËﬂ (48; 52). íÛ‰ÌÓÒÚËÚÂ ÔË ˜ÂÚÂÌÂ ÒÂ ‡ÒÓˆËË‡Ú Ò ‡·ÌÓÏÌÓÒÚË ‚ ‰ÂÒÌËﬂ ˆÂÂ·ÂÎÛÏ (28). ÑËÒÎÂÍÒËﬂÚ‡, ÍÓﬂÚÓ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÔË ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ, ‚ÂÓﬂÚÌÓ Â Ò‚˙Á‡Ì‡ Ò˙Ò ÒÏÛ˘ÂÌËﬂ ‚ ÒËÌ‡ÔÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÌË‚Ó ˆÂÂ·ÂÎÛÏ ÔË ‡·ÌÓÏÌ‡ ÂÍÒÔÂÒËﬂ Ì‡ ‡-‰ËÒÚÓ„ÎËÍ‡Ì‡ (66). Ç Â‰ÍË ÒÎÛ˜‡Ë Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ÏÓ„‡Ú ‰‡ ËÏ‡Ú Ë ‰Û„Ë ï-Ò‚˙Á‡ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ, Í‡ÚÓ ÔË„ÏÂÌÚÂÌ ÂÚËÌËÚ, ıÓÌË˜Ì‡ „‡ÌÛÎÓÏ‡ÚÓÁÌ‡ ·ÓÎÂÒÚ Ë ·ÓÎÂÒÚ Ì‡ McLeod (ÒËÌ‰ÓÏ Ì‡ ÌÂ‚Ó‡Í‡ÌÚÓˆËÚÓÁ‡), „ÎËˆÂÓÎ-ÍËÌ‡ÁÂÌ ‰ÂÙËˆËÚ ËÎË ‡‰ÂÌ‡ÎÌ‡ ıËÔÓÔÎ‡ÁËﬂ, ‚ ÂÁÛÎÚ‡Ú Ì‡ ‡ÁÎË˜ÌË ‰ÂÎÂˆËË. èÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ ÊË‚ÓÚ‡ ÔË ÏÓÏ˜ÂÚ‡ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ Â ÒÂ‰ÌÓ 20-25 „Ó‰ËÌË. ç‡È-˜ÂÒÚÓ ÔË˜ËÌË Á‡ ÎÂÚ‡ÎÌËﬂ ËÁıÓ‰ Ò‡ ·ÂÎÓ‰Ó·ÌË ËÌÙÂÍˆËË Ë Ò˙‰Â˜Ì‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ. èåÑ íàè ÅÖäÖê ï‡‡ÍÚÂËÁË‡ ÒÂ Ò ÔÓ-Í˙ÒÌÓÚÓ Ì‡˜‡ÎÓ Ë ÔÓ-ÎÂÍ‡Ú‡ ÍÎËÌË˜Ì‡ Í‡ÚËÌ‡ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ÔÓ‡‰Ë Ì‡ÎË˜ËÂÚÓ Ì‡ ‰ËÒÚÓÙËÌ ‚ Â‰ÛˆË‡ÌË ÍÓÎË˜ÂÒÚ‚‡ ËÎË ‡·ÌÓÏÂÌ ÔÓ ÒÚÛÍÚÛ‡. ç‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â ÒÎÂ‰ 7-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. èÓÒÚÂÔÂÌÌÓ ÒÂ ‡Á‚Ë‚‡ ÔÓ„ÂÒË‚Ì‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ Í‡ÚÓ ÔÓÍÒËÏ‡ÎÌËÚÂ ÏÛÒÍÛÎË Ò‡ ÔÓ-ÚÂÊÍÓ Á‡ÒÂ„Ì‡ÚË ÓÚ ‰ËÒÚ‡ÎÌËÚÂ, ‡ ‰ÓÎÌËÚÂ Í‡ÈÌËˆË- ÔÓ-‡ÌÓ ÓÚ „ÓÌËÚÂ. óÂÒÚ ÒËÏÔÚÓÏ Â ÔÒÂ‚‰ÓıËÔÂÚÓÙËﬂÚ‡ Ì‡ ÔÓ‰·Â‰ËˆËÚÂ. ëÎ‡·ÓÒÚÚ‡ ‚ m. quadriceps femoris ÏÓÊÂ ‰˙Î„Ó ‚ÂÏÂ ‰‡ ·˙‰Â Â‰ËÌÒÚ‚ÂÌ‡Ú‡ ËÁﬂ‚‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. Ç ÒÎÛ˜‡ËÚÂ, ÔË ÍÓËÚÓ „ÂÌÌËÚÂ ÏÛÚ‡ˆËË ‚Ó‰ﬂÚ ‰Ó ‰ÂÙÂÍÚË ‚ ‡ÏËÌÓ-Í‡ﬂ Ì‡ ‰ËÒÚÓÙËÌÓ‚‡Ú‡ ÏÓÎÂÍÛÎ‡, Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÏÓÊÂ ‰‡ ÒÂ ÔÓﬂ‚Ë Ò ÏÛÒÍÛÎÌË Í‡ÏÔË, ÔÓ-ÎÂÒÌ‡ ÛÏÓﬂÂÏÓÒÚ ÔË ÙËÁË˜ÂÒÍË ÛÒËÎËﬂ, ÏËÓ„ÎÓ·ËÌÛËﬂ Ë ‡ÒËÏÔÚÓÏÌÓ Û‚ÂÎË˜ÂÌ‡ äîä (ÍÂ‡ÚËÌÙÓÒÙÓÍËÌ‡Á‡). ëÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚÓﬂÚÂÎÌ‡ ÔÓıÓ‰Í‡ ÒÂ „Û·Ë ÒÎÂ‰ 16-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ, ‚ ÌﬂÍÓË ÒÎÛ˜‡Ë ÒÎÂ‰ 30-35 „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. èË ÚÂÁË Ô‡ˆËÂÌÚË Í‡‰ËÓÏËÓÔ‡ÚËËÚÂ Ë ÛÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡ÌÂ Ò‡ ÔÓ- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology Â‰ÍË Ë ÔÓ-ÎÂÍË. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ ËÏ‡Ú ÔÓ-Ï‡ÎÍÓ ıÓÏÓ„ÂÌÂÌ ÍÓ„ÌËÚË‚ÂÌ ÙÂÌÓÚËÔ ‚ Ò‡‚ÌÂÌËÂ Ò ÚÂÁË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ. Ç Ò‡‚ÌÂÌËÂ Ò Ó·˘‡Ú‡ ÔÓÔÛÎ‡ˆËﬂ, Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ, Ò‡ Ò ÔÓ-‚ËÒÓÍ‡ ˜ÂÒÚÓÚ‡ Ì‡ Á‡ÚÛ‰ÌÂÌËﬂ ‚ Ó·Û˜ÂÌËÚÓ, ‡ÛÚËÁ˙Ï, ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ, ıËÔÂ‡ÍÚË‚ÌÓ ÔÓ‚Â‰ÂÌËÂ Ë ‰ÂÙËˆËÚ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ. èË ÔÓ‚ÂÊ‰‡ÌÂÚÓ Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÂÒÍË ËÁÒÎÂ‰‚‡ÌËﬂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÔÓ-ÒÎ‡·Ó ÔÂ‰ÒÚ‡‚ﬂÌÂ Ì‡ Á‡‰‡˜Ë, Ò‚˙Á‡ÌË Ò ‚Â·‡ÎÌ‡ Ô‡ÏÂÚ Ë ÏÓÚÓÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌË ÛÏÂÌËﬂ (9; 74). àÌÚÂÏÂ‰ËÂÌËﬂÚ ÚËÔ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÒÂ ı‡‡ÍÚÂËÁË‡ Ò Ì‡˜‡ÎÓ Ì‡ ÍÎËÌË˜Ì‡Ú‡ ÒËÏÔÚÓÏ‡ÚËÍ‡ ÏÂÊ‰Û 5 Ë 7-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ Ë Á‡„Û·‡ Ì‡ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚÓﬂÚÂÎÌ‡ ÔÓıÓ‰Í‡ ÏÂÊ‰Û 13 Ë 16-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. ÑàÄÉçéëíàñàêÄçÖ à èêéëãÖÑüÇÄçÖ çÄ èêéÉêÖëàÇçàíÖ åìëäìãçà Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê ÑË‡„ÌÓÁ‡Ú‡ ÒÂ ÔÓÒÚ‡‚ﬂ ‚˙Á ÓÒÌÓ‚‡ Ì‡ ı‡‡ÍÚÂÌ‡Ú‡ ÍÎËÌËÍ‡ Ë Ô‡‡ÍÎËÌË˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ. ● ÅËÓıËÏË˜ÌËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ Ò‡ Ò ‰‡ÌÌË Á‡ ÔÓ‚Ë¯ÂÌË ÒÚÓÈÌÓÒÚË Ì‡ äîä, ÄëÄí, ÄãÄí, Ë ãÑï. èË Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ Ñ˛¯ÂÌ äîä ÒÂ ÔÓ‚Ë¯‡‚‡ Ì‡ÈÏ‡ÎÍÓ 10 Ô˙ÚË Ì‡‰ ÌÓÏ‡Ú‡ (Ì‡È-˜ÂÒÚÓ ÏÂÊ‰Û 25 Ë 200 Ô˙ÚË), ‡ ÔË èåÑ ÚËÔ ÅÂÍÂ- Ì‡È-Ï‡ÎÍÓ 5 Ô˙ÚË. èÓ‚Ë¯ÂÌËÚÂ ÒÚÓÈÌÓÒÚË Ì‡ äîä, ÍÓËÚÓ Ò‡ ÒÎÂ‰ÒÚ‚ËÂ Ì‡ ÌÂÍÓÁ‡ Ì‡ ÏÛÒÍÛÎÌËÚÂ ‚Î‡ÍÌ‡, Ò˙ÓÚ‚ÂÚÒÚ‚‡Ú Ì‡ ‚˙Á‡ÒÚÚ‡ Ë Ò˙ÒÚÓﬂÌËÂÚÓ Ì‡ Ô‡ˆËÂÌÚ‡ (5). ● ÖåÉ (ÂÎÂÍÚÓÏËÓ„‡ÙËﬂ) ÛÒÚ‡ÌÓ‚ﬂ‚‡ ı‡‡ÍÚÂÌ‡ „ÂÌÂ‡ÎËÁË‡Ì‡ ÏËÓ„ÂÌÌ‡ Û‚Â‰‡ ‡ ‚ Í‡ÈÌËÚÂ ÒÚ‡‰ËË Ì‡ èåÑ Ó·˘ÓÚÓ ˜ËÒÎÓ Ì‡ ‡ÍˆËÓÌÌËÚÂ ÔÓÚÂÌˆË‡ÎË ÒÂ Â‰ÛˆË‡ Ò ÓÍÓÎÓ 35%. ● óÂÁ ÖäÉ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ‡ÁÎË˜ÌË ÙÓÏË Ì‡ ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËﬂ (Ô˙ÎÂÌ ‰ÂÒÂÌ ·Â‰ÂÌ ·ÎÓÍ, ÒËÌÛÒÓ‚‡ Ú‡ıËÍ‡‰Ëﬂ, ÔÂ‰Ò˙‰ÌË Ë Í‡ÏÂÌË ÂÍÒÚ‡ÒËÒÚÓÎË). ÖıÓÍ‡‰ËÓ„‡ÙËﬂÚ‡ ÒÂ ÔËÎ‡„‡ Á‡ ÔÓÒÎÂ‰ﬂ‚‡ÌÂ Ì‡ Ò˙‰Â˜ÌËﬂ ÒÚ‡ÚÛÒ. ÇËÁÛ‡ÎËÁË‡Ú ÏËÓÍ‡‰Ì‡ ÙË·ÓÁ‡, ıËÔÓÍËÌÂÁËﬂ Ì‡ Ò˙ˆÂÚÓ, ‰ËÎ‡Ú‡ÚË‚Ì‡ Í‡‰ËÓÏËÓÔ‡ÚËﬂ, Ì‡Ï‡ÎÂÌ‡ Ù‡ÍˆËﬂ Ì‡ ËÁÚÎ‡ÒÍ‚‡ÌÂ. ÑÓ ‰ÂÒÂÚ „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ ÒÂ ÔÂÔÓ˙˜‚‡ ÖäÉ Ë ÂıÓÍ‡‰ËÓ„‡ÙËﬂ ‰‡ ÒÂ ÔÓ‚ÂÊ‰‡Ú ‚Â‰Ì˙Ê Ì‡ ‰‚Â „Ó‰ËÌË, ‡ ÒÎÂ‰ ÚÓ‚‡ ‚Â‰Ì˙Ê „Ó‰Ë¯ÌÓ. ● îÛÌÍˆËÓÌ‡ÎÌÓ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ‰Ë¯‡ÌÂÚÓ. èË Ô‡ˆËÂÌÚËÚÂ ÒÂ ËÁÒÎÂ‰‚‡Ú îÓÒË‡Ì ÇËÚ‡ÎÂÌ ä‡Ô‡ˆËÚÂÚ (îÇä) ÔÓ‡‰Ë ÂÒÚËÍÚË‚ÂÌ ÚËÔ Ì‡Û¯ÂÌËÂ Ì‡ ‰Ë¯‡ÌÂÚÓ Ò˙Ò ÒÌËÊÂÌË ÒÚÓÈÌÓÒÚË Ì‡ îÖé1 Ë îÇä Ë ÌÓÏ‡ÎÂÌ ÍÓÂÙËˆËÂÌÚ Ì‡ íËÙÌÓ îÇä/îÖé1 (67). éÔÂ‰ÂÎﬂÚ ÒÂ ÍËÒÎÓÓ‰Ì‡Ú‡ Ò‡ÚÛ‡ˆËﬂ Ë Ô‡ˆË‡ÎÌËÚÂ Ì‡Îﬂ„‡ÌËﬂ Ì‡ ÍËÒÎÓÓ‰‡ Ë ‚˙„ÎÂÓ‰ÌËﬂ ‰ËÓÍÒË‰ ‚ Í˙‚Ú‡, ˜ËËÚÓ ÔÓÏÂÌË Ò‡ ‚ ÚﬂÒÌ‡ ÍÓÂÎ‡ˆËﬂ Ò ÔÓÏÂÌËÚÂ Ì‡ ÙÛÌÍˆËÓÌ‡ÎÌËÚÂ ÔÓÍ‡Á‡ÚÂÎË. ● åÛÒÍÛÎÌ‡ ·ËÓÔÒËﬂ. ïËÒÚÓÎÓ„Ë˜ÌÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ ‚ ‡ÌÌËÚÂ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ‡ÁÍË‚‡ ‰Â„ÂÌÂ‡ˆËﬂ Ë Ù‡„ÓˆËÚÓÁ‡ Ì‡ Â‰ËÌË˜ÌË ËÎË „ÛÔË ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡, ÌÂÍÓÚË˜ÌË Ë Â„ÂÌÂ‡ÚÓÌË ÔÓÏÂÌË. Ç Í˙ÒÌËÚÂ ÒÚ‡‰ËË Ì‡ ÏÛÒÍÛÎÌËÚÂ ‰ËÒÚÓÙËË ÒÂ ÓÚÍË‚‡Ú: Á‡„Û·‡ Ì‡ ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡, ÏËÓˆËÚË Ò ÔÓÏ‡ÎÍË ËÎË ÔÓ-„ÓÎÂÏË ‡ÁÏÂË ÓÚ ÌÓÏ‡ÎÌËÚÂ Ë ·ÂÁ‡Á·ÓÌÓ ‡ÁÔÓÎÓÊÂÌËÂ, ‡Á‡ÒÚ‚‡ÌÂ ÂÌ‰ÓÏËÁË‡ÎÌÓ Ì‡ Ï‡ÒÚÌ‡ Ë Ò˙Â‰ËÌËÚÂÎÌ‡ Ú˙Í‡Ì. àÏÛÌÓıËÒÚÓıËÏË˜ÌÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ Ë Western blot ÔÓÍ‡Á‚‡Ú ÎËÔÒ‡ Ì‡ ‰ËÒÚÓÙËÌ ÔË èåÑ ÚËÔ Ñ˛¯ÂÌ, Â‰ÛˆË‡Ì ËÎË ‡·ÌÓÏÂÌ- ÔË ÚËÔ ÅÂÍÂ. ● çÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ ‚ÍÎ˛˜‚‡Ú Ó·˘Ó 159 ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ ÙÛÌÍˆËË, ÚÂÒÚÓ‚ÂÚÂ Á‡ Ô‡ÏÂÚÓ‚Ë ÙÛÌÍˆËË, ÂÍÁÂÍÛÚË‚ÌË ÙÛÌÍˆËË, ÔÓ‚Â‰ÂÌ˜ÂÒÍËÚÂ Ì‡Û¯ÂÌËﬂ (54). ● é·‡ÁÌË ËÁÒÎÂ‰‚‡ÌËﬂ Ì‡ ñçë (äÄí Ë åêí), Í‡ÍÚÓ Ë ÙÛÌÍˆËÓÌ‡ÎÌË ËÁÒÎÂ‰‚‡ÌËﬂ Ì‡ ÏÓÁ˙˜ÌËﬂ ÏÂÚ‡·ÓÎËÁ˙Ï (SPECT Ë PET). èË ÌﬂÍÓË Ô‡ˆËÂÌÚË ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú Ì‡Ï‡ÎÂÌ Ó·ÂÏ Ì‡ ÏÓÁ˙˜ÌÓÚÓ ‚Â˘ÂÒÚ‚Ó Ë ‡Á¯ËÂÌË ‚ÂÌÚËÍÛÎË (17; 68) èË êÖí ÒÂ ‚ËÁÛ‡ÎËÁË‡Ú Ì‡Ï‡ÎÂÌ „Î˛ÍÓÁÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï ‚ ‰ÂÒÌËÚÂ gyrus postcentralis Ë gyrus temporalis medius, uncus, ÎÂ‚Ëﬂ gyrus hyppocampalis Ë Ï‡ÎÍËﬂ ÏÓÁ˙Í (17; 51), ÍÓÂÚÓ ‚ÂÓﬂÚÌÓ ÓÚ‡Áﬂ‚‡ ÒÏÛÚÂÌ‡ ÌÂ‚ÓÌ‡ÎÌ‡ ÏË„‡ˆËﬂ Ë ÍÎÂÚ˙˜Ì‡ ‰ËÙÂÂÌˆË‡ˆËﬂ, ÍÓËÚÓ ÒÂ Â„ÛÎË‡Ú ÓÚ ‰ËÒÚÓÙËÌ. ● ÉÂÌÂÚË˜ÌÓ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ·ÓÎÌË ˜ÂÁ ‰ËÂÍÚÂÌ Ë ËÌ‰ËÂÍÚÂÌ Ñçä-‡Ì‡ÎËÁ ÄçÄãàá çÄ ÑÖãÖñàà à ÑìèãàäÄñàà åÛÎÚËÔÎÂÍÒÌ‡Ú‡ PCR, Southern blot Ë FISH ÒÂ ËÁÔÓÎÁ‚‡Ú Á‡ ÓÚÍË‚‡ÌÂ Ì‡ ‰ÂÎÂˆËË ‚ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ. èË·ÎËÁËÚÂÎÌÓ 98% ÓÚ ‰ÂÎÂˆËËÚÂ ÒÂ ÓÚÍË‚‡Ú Ò ÚÂÁË ÏÂÚÓ‰ËÍË. Southern blot Ë ÍÓÎË˜ÂÒÚ‚ÂÌ‡ PCR ÒÂ ÔËÎ‡„‡Ú Á‡ ÓÚÍË‚‡ÌÂ Ì‡ ‰ÛÔÎËÍ‡ˆËË. MLPA (Multiple ligation probe amplification) ÒÂ ËÁÔÓÎÁ‚‡ Á‡ ‡Ì‡ÎËÁ Ì‡ ‰ÂÎÂˆËË Ë ‰ÛÔÎËÍ‡ˆËË ÔË ·ÓÎÌË Ë ÊÂÌË-ÌÓÒËÚÂÎÍË (70). - ëÍ‡ÌË‡ÌÂÚÓ Ì‡ ÏÛÚ‡ˆËËÚÂ Ë ÒÂÍ‚ÂÌË‡ÌÂÚÓ ÓÚÍË‚‡Ú Ï‡ÎÍË ‰ÂÎÂˆËË, ËÌÒÂˆËË, ÚÓ˜ÍÓ‚Ë Ë ÒÔÎ‡ÈÒËÌ„ ÏÛÚ‡ˆËË (11, 29, 70). àÁÔÓÎÁ‚‡Ú ÒÂ ‡Ì‡ÎËÁ Ì‡ ÔÓÎËÏÓÙËÁÏË ‚ ÍÓÌÙÓÏ‡ˆËﬂ Ì‡ Â‰ÌÓ‚ÂËÊÌËÚÂ (SSCP) Ë ‰‚ÓÈÌÓ‚ÂËÊÌËÚÂ (DSCP) Ñçä Ù‡„ÏÂÌÚË, ıÂÚÂÓ‰ÛÔÎÂÍÒÂÌ ‡Ì‡ÎËÁ, ÒÂÍ‚ÂÌË‡ÌÂ, ÔÂÊ‰Â‚ÂÏÂÌÌÓ ÔÂÍ‡Úﬂ‚‡ÌÂ Ì‡ ·ÂÎÚ˙˜Ì‡Ú‡ Ú‡ÌÒÎ‡ˆËﬂ (Protein truncation test). çÓ‚Ë ÏÂÚÓ‰Ë Í‡ÚÓ SCAIP (single-condition amplification internal primer sequencing) Ë DGGE ‰ÂÌ‡ÚÛË‡˘ „ÂÎ ÂÎÂÍÚÓÙÓÂÁ‡- ·‡ÁË‡ÌÓ ÒÍ‡ÌË‡ÌÂ Ì‡ „ÂÌ‡ ËÏ‡Ú Á‡ ˆÂÎ ‰‡ ÓÚÍËﬂÚ ÓÒÚ‡Ì‡ÎËÚÂ 30-35% ÓÚ ÏÛÚ‡ˆËËÚÂ ÔË èåÑ ÚËÔ Ñ˛¯ÂÌ. àÁÒÎÂ‰‚‡ÌÂÚÓ Ì‡ ·ËÓÔÒË˜ÂÌ Ï‡ÚÂË‡Î ÓÚ ÏÛÒÍÛÎ ˜ÂÁ ÔÓÚÂËÌÂÌ Ë êçä-·‡ÁË‡Ì ‡Ì‡ÎËÁ ‰ÓÔ˙Î‚‡ ‰ËÂÍÚÌÓÚÓ Ñçä-ÒÂÍ‚ÂÌË‡ÌÂ (27). ● ÉÂÌÂÚË˜ÌÓ ËÁÒÎÂ‰‚‡ÌÂ Á‡ ÌÓÒËÚÂÎÒÚ‚Ó àÁÒÎÂ‰‚‡Ú ÒÂ ÊÂÌË, ‚ ÒÂÏÂÈÒÚ‚‡Ú‡ Ì‡ ÍÓËÚÓ ËÏ‡ ·ÓÎÂÌ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ. äÓ„‡ÚÓ ÏÛÚ‡ˆËﬂÚ‡ Ì‡ Ô‡ˆËÂÌÚ‡ Ò ÑåÑ Â ËÁ‚ÂÒÚÌ‡, ËÁÒÎÂ‰‚‡ÌÂÚÓ Á‡ ÌÓÒËÚÂÎÒÚ‚Ó Ì‡ ÏÛÚ‡ˆËﬂ ÓÚ ÚËÔ‡ ‰ÂÎÂˆËﬂ ËÎË ‰ÛÔÎËÍ‡ˆËﬂ ÏÓÊÂ ‰‡ ·˙‰Â ËÁ‚˙¯ÂÌÓ ˜ÂÁ ÍÓÎË˜ÂÒÚ‚ÂÌ ‡Ì‡ÎËÁ Ì‡ „ÂÌÌËﬂ ‰ÓÁ‡Ê. íÓÁË ÏÂÚÓ‰ ÏÓÊÂ ‰‡ Â ÚÛ‰ÂÌ Á‡ ÓÒ˙˘ÂÒÚ‚ﬂ‚‡ÌÂ Ë ËÌÚÂÔÂÚ‡ˆËﬂ. àÁÔÓÎÁ‚‡Ú ÒÂ Ò˙˘Ó Ú‡Í‡ PCR (Á‡ ‰ÂÎÂˆËË Ë ‰ÛÔÎËÍ‡ˆËË), FISH (Á‡ ‰ÂÎÂˆËË) Ë ÒÂÍ‚ÂÌË‡ÌÂ (Á‡ ÚÓ˜ÍÓ‚Ë ÏÛÚ‡ˆËË). äÓ„‡ÚÓ ÏÛÚ‡ˆËﬂÚ‡ Ì‡ Ô‡ˆËÂÌÚ‡ Ò ÑåÑ ÌÂ Â ËÁ‚ÂÒÚÌ‡ ÏÓÊÂ ‰‡ ÒÂ ËÁÔÓÎÁ‚‡ Linkage analysis, ÍÓ„‡ÚÓ ‚ ÒÂÏÂÈÒÚ‚ÓÚÓ Ì‡ ËÁÒÎÂ‰‚‡ÌËÚÂ ÊÂÌË ËÏ‡ ÔÓ‚Â˜Â ÓÚ Â‰ËÌ Á‡ÒÂ„Ì‡Ú Ò˙Ò ÒË„ÛÌ‡ ‰Ë‡„ÌÓÁ‡ èåÑ ÚËÔ Ñ˛¯ÂÌ/ ÅÂÍÂ ËÎË Ñ˛¯ÂÌ-‡ÒÓˆËË‡Ì‡ Í‡‰ËÓÏËÓÔ‡ÚËﬂ. ● à‰ÂÌÚËÙËˆË‡ÌÂ Ì‡ de novo ÏÛÚ‡ˆËË. Ç ÒÂÏÂÈÒÚ‚‡ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ ÔË Ò‡ÏÓ Â‰ËÌ ˜ÎÂÌ Ì‡ ÒÂÏÂÈÒÚ‚ÓÚÓ, ÔÓËÁıÓ‰˙Ú Ì‡ de novo ÏÛÚ‡ˆËﬂÚ‡ ÏÓÊÂ ‰‡ ·˙‰Â Ë‰ÂÌÚËÙËˆË‡Ì ˜ÂÁ ‰ÂÎÂˆËÓÌÂÌ/ ‰ÛÔÎËÍ‡ˆËÓÌÂÌ ‡Ì‡ÎËÁ ËÎË ÒÂÍ‚ÂÌË‡ÌÂ ˜ÂÁ linkage analysis. ï‡ÔÎÓÚËÔ˙Ú, ‡ÒÓˆËË‡Ì Ò ÏÛÚË‡ÎËﬂ ‡ÎÂÎ ÔË Á‡ÒÂ„Ì‡ÚËﬂ ËÌ‰Ë‚Ë‰ ÏÓÊÂ ‰‡ ·˙‰Â ÔÓÒÎÂ‰ÂÌ ÔË Ï‡ÈÍ‡Ú‡ Ë ‡ÍÓ Â ÌÂÓ·ıÓ‰ËÏÓ ÔË ÌÂÈÌËÚÂ Ó‰ËÚÂÎË (34). ● èÂÌ‡Ú‡ÎÌ‡Ú‡ Ë ÔÂÍÓÌˆÂÔˆËÓÌÌ‡Ú‡ ‰Ë‡„ÌÓÒÚËÍ‡ ‚ Á‡ÒÂ„Ì‡ÚËÚÂ ÒÂÏÂÈÒÚ‚‡ Ò‡ ÓÒÌÓ‚ÂÌ ÂÎÂ- Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ÏÂÌÚ ‚ ÔÓÙËÎ‡ÍÚËÍ‡Ú‡ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ. íﬂ ÒÂ ÔÓ‚ÂÊ‰‡ Ò‡ÏÓ ÔË ËÌÙÓÏ‡ÚË‚ÌË ÒÂÏÂÈÒÚ‚‡ Ò ÛÚÓ˜ÌÂÌ‡ ÏÛÚ‡ˆËﬂ Ë ËÁﬂÒÌÂÌË ËÌÙÓÏ‡ÚË‚ÌË ÔÓÎËÏÓÙÌË Ñçä-Ï‡ÍÂË. èÂÌ‡Ú‡ÎÌ‡Ú‡ ‰Ë‡„ÌÓÒÚËÍ‡ Â ÔÓÍ‡Á‡Ì‡ ÔË ÊÂÌË Ò ‰ÓÍ‡Á‡Ì ÌÓÒËÚÂÎÒÍË ÒÚ‡ÚÛÒ (ÔÓ‡‰Ë ËÒÍ‡ ÓÚ 50% ÓÚ ‡Ê‰‡ÌÂ Ì‡ ·ÓÎÌÓ ÏÓÏ˜Â), ÔË ÊÂÌË, Ó‰ËÎË ‰Âˆ‡ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ, ÌÓ Ò ÌÂËÁﬂÒÌÂÌ ÌÓÒËÚÂÎÒÍË ÒÚ‡ÚÛÒ (ÔÓ‡‰Ë ËÒÍ ÓÚ ÔÓÎÓ‚ ÏÓÁ‡ËˆËÁ˙Ï). ä‡ÚÓ Ô˙‚Ë ÂÚ‡Ô Ì‡ ÔÂÌ‡Ú‡ÎÌ‡Ú‡ ‰Ë‡„ÌÓÒÚËÍ‡ Â ÓÔÂ‰ÂÎﬂÌÂÚÓ Ì‡ ÔÓÎ‡ ˜ÂÁ ˆËÚÓ„ÂÌÂÚË˜ÂÌ ËÎË Ñçä-‡Ì‡ÎËÁ. ÄÍÓ ÔÎÓ‰˙Ú Â Ï˙ÊÍË ÒÂ ÔÓ‚ÂÊ‰‡ ‰ËÂÍÚÂÌ ËÎË ËÌ‰ËÂÍÚÂÌ Ñçä-‡Ì‡ÎËÁ ˜ÂÁ „ÓÂÔÓÒÓ˜ÂÌËÚÂ ÏÂÚÓ‰Ë. áÄäãûóÖçàÖ äÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ ÔË ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Ò‡ ÔÓ-˜ÂÒÚË ÔË ÏÛÒÍÛÎÌËÚÂ ‰ËÒÚÓÙËË ÚËÔ Ñ˛¯ÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò ÅÂÍÂ. íÂÁË ÒÏÛ˘ÂÌËﬂ Ò‡ Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ, Í‡ÚÓ ‚Â·‡ÎÌËÚÂ ÛÏÂÌËﬂ Ë Í‡ÚÍÓÒÓ˜Ì‡Ú‡ Ô‡ÏÂÚ Ò‡ ÔÓ-ÚÂÊÍÓ Á‡ÒÂ„Ì‡ÚË. èË Ô‡ˆËÂÌÚËÚÂ Ò èåÑ ÚËÔ ÅÂÍÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÔÒËıË˜ÌË Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË ÓÚÍÎÓÌÂÌËﬂ, Í‡ÚÓ ‡ÛÚËÁ˙Ï, ıËÔÂ‡ÍÚË‚ÌÓ ÔÓ‚Â‰ÂÌËÂ Ë ‰ÂÙËˆËÚ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ. íﬂıÌ‡Ú‡ „ÂÌÂÁ‡ ‚ÂÓﬂÚÌÓ Â ÍÓÏÔÎÂÍÒÌ‡, Ó·ÛÒÎÓ‚ÂÌ‡ ÓÚ ÒÔÂˆËÙË˜ÌË ÔÓ ÎÓÍ‡ÎËÁËˆËﬂ ÏÛÚ‡ˆËË ‚ ‡ÏÍËÚÂ Ì‡ ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ, ‰ËÒÙÛÌÍˆËﬂ Ì‡ ‰ËÒÚÓÙËÌÓÔÓ‰Ó·ÌËÚÂ Ë ‰ËÒÚÓÙËÌ-‡ÒÓˆËË‡ÌËÚÂ ÔÓÚÂËÌË, Â‰ÛˆË‡Ì „Î˛ÍÓÁÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï, ÏÛÚ‡ˆËË Á‡Òﬂ„‡˘Ë ‰Û„Ë „ÂÌË, ÎÓÍ‡ÎËÁË‡ÌË ‚˙ıÛ ï-ıÓÏÓÁÓÏ‡Ú‡, ÏÓÁ˙˜Ì‡ ıËÔÓÍÒËﬂ. Ç Å˙Î„‡Ëﬂ ÌÂ Ò‡ ÔÓ‚ÂÊ‰‡ÌË „ÂÌÓÚËÔ-ÙÂÌÓÚËÔÌË ÍÓÂÎ‡ˆËË, ÔË ‰ÂÚ‡ÈÎÌÓÚÓ „ÂÌÓÚËÔËÁË‡ÌÂ ˜ÂÁ åLPA Ì‡ ‚Ë‰‡ Ë ÒÚÂÔÂÌÚ‡ Ì‡ ËÁ‡ÁÂÌÓÒÚ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ, Í‡ÍÚÓ Ë ÍÓÂÎ‡ˆËËÚÂ Ò åêí Ì‡ „Î‡‚ÌËﬂ ÏÓÁ˙Í. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. ÅÓÊËÌÓ‚ ë., É˙Î˙·Ó‚ É. Ë Ò˙Ú. åËÓÔ‡ÚËË. äÎËÌË˜ÌÓ, ·ËÓıËÏË˜ÌÓ, ıËÒÚÓÎÓ„Ë˜ÌÓ, ıËÒÚÓıËÏË˜ÌÓ Ë ÂÎÂÍÚÓÌÌÓÏËÍÓÒÍÓÔÒÍÓ ËÁÒÎÂ‰‚‡ÌÂ. åÂ‰ËˆËÌ‡ Ë ÙËÁÍÛÎÚÛ‡ 1973:278Ò ÉÂÓ„ËÂ‚ à., ñ‚ÂÚ‡ÌÓ‚‡ Ö., ÅÓÊËÌÓ‚‡ í., Ç‡ÌÂ‚‡ Ñ. éÚÍË‚‡ÌÂ Ì‡ ÌÓÒËÚÂÎË Ì‡ Ì‡ÒÎÂ‰ÒÚ‚ÂÌÓÒÚÚ‡ ÔË ÔÓ„ÂÒË‚Ì‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ. èÂ‰Ë‡ÚËﬂ 1971;10: 264-269 íÓ‰ÓÓ‚‡ Ä. åÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ Ñ˛¯ÂÌ/ ÅÂÍÂ ‚ Å˙Î„‡Ëﬂ ÏÓÎÂÍÛÎÌË ı‡‡ÍÚÂËÒÚÍË, ÏÂı‡ÌËÁÏË Ì‡ ÏÛÚ‡„ÂÌÂÁ‡ Ë ÔÓÙËÎ‡ÍÚËÍ‡. ÑËÒÂÚ‡ˆËﬂ Á‡ ÔËÒ˙Ê‰‡ÌÂ Ì‡ Ó·‡ÁÓ‚‡ÚÂÎÌ‡ ÒÚÂÔÂÌ ‰ÓÍÚÓ. ëÓÙËﬂ, 1999. ñ‡˜Â‚‡ å., Å‡Î‰‡‡ÌÓ‚ Ñ., íÓ‰ÓÓ‚‡ Ä., í˙ÌÂ‚ à., ñ‡ÌÍÓ‚ Ç., ÑËÏËÚÓ‚ Ñ., äÂÏÂÌÒÍË à. ÖÍÒÔÂÒËﬂ Ì‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÚËÔ Ñ˛¯ÂÌ Û ÊÂÌ‡ ıÂÚÂÓÁË„ÓÚÂÌ ÌÓÒËÚÂÎ. 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ÉÂÓ„Ë ëÓÙËÈÒÍË ‹1. e-mail: [email protected] Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 162 é·ÁÓ ÑàëíêéîàçéèÄíàà- ëöÇêÖåÖççé ãÖóÖçàÖ èêà èêéÉêÖëàÇçàíÖ åìëäìãçÄ Ñàëíêéîàà íàè ÑûòÖç à ÅÖäÖê í. ó‡ÏÓ‚‡1, à. í˙ÌÂ‚1,2 1çÂ‚ÓÎÓ„Ë˜Ì‡ 2çÓ‚ ÍÎËÌËÍ‡, ìåÅÄã ◊ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, åî –ëÓÙËﬂ ·˙Î„‡ÒÍË ÛÌË‚ÂÒËÚÂÚ, ä‡ÚÂ‰‡Ú‡ ÔÓ äÓ„ÌËÚË‚Ì‡ Ì‡ÛÍ‡ Ë ÔÒËıÓÎÓ„Ëﬂ SUMMARY DYSTROPHINOPATHIES- MODERN TREATMENT OF DUCHENNE AND BECKER MUSCULAR DYSTROPHIES Teodora Chamova1, Ivailo Tournev1,2 1 Neurology clinic, University hospital “Alexandrovska”, Medical Faculty 2New Bulgarian University, Chair of cognitive science and psychology Duchenne and Becker muscular dystrophies are genetic disorders, characterized by progressive muscle wasting, loss of walking ability leading to complete wheelchair dependence, cognitive disorders, as well as cardiomyopathy and respiratory insufficiency, which are the main causes of death. The therapy of these diseases relies on multidisciplinary management and aims to slow the progression of the disorder and to improve the life quality of these patients. The key areas of the symptomatic treatment are physiotherapy, respiratory management, management of cardiomyopathy and orthopedic interventions. The glucocorticosteroids are the gold standard of treatment for muscle weakness in ambulant children with DMD, although the specific cellular events responsible for their beneficial effects are not known. The eagerly awaited pathogenic approaches based on gene therapy are at a stage of in vitro clinical trials and clinical trials on animal models. These advanced technologies encompass influencing the gene expression by gene replacement therapy with stem cells and delivery of dystrophin minigenes; DNA/RNA manipulation; pharmacological reagents (drugs to turn on the urotrophin gene expression, drugs to cause read-through of premature stop codon, viral delivery of growth factors, protease inhibitors, antiinflamatory reagents). The gene modification approaches such as DNA/RNA manipulation with antisense oligonucleotides resulting in exon skipping and short forms of dystrophin, which attempts to turn a Duchenne mutation into a less severe Becker form are the most promising techniques. KEY WORDS: Dystrophinopathies, Duchenne muscular dystrophy, Becker muscular dystrophy, physiotherapy, respiratory management, drug therapy of cardiomyopathy, orthopedic interventions, corticosteroids, gene replacement therapy with stem cells, DNA/RNA manipulation; pharmacologic agents. êÖáûåÖ èÓ„ÂÒË‚ÌËÚÂ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË ÚËÔ Ñ˛¯ÂÌ Ë ÅÂÍÂ Ò‡ „ÂÌÂÚË˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ, ˜ËﬂÚÓ ÍÎËÌË˜Ì‡ Í‡ÚËÌ‡ ‚ÍÎ˛˜‚‡ ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ ‰Ó ÚÂÊÍ‡ ËÌ‚‡ÎË‰ËÁ‡ˆËﬂ, ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ, Í‡ÍÚÓ Ë Í‡‰ËÓÏËÓÔ‡ÚËﬂ Ë ‰Ëı‡ÚÂÎÌ‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ, ÍÓËÚÓ Ò‡ Ì‡È˜ÂÒÚËÚÂ ÌÂÔÓÒÂ‰ÒÚ‚ÂÌË ÔË˜ËÌË Á‡ ÎÂÚ‡ÎÂÌ ËÁıÓ‰. ìÚ‚˙‰ÂÌ‡Ú‡ Ì‡ ÚÓÁË ÂÚ‡Ô ÚÂ‡ÔËﬂ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Â ÍÓÏÔÎÂÍÒÌ‡ Ë ËÏ‡ Á‡ ˆÂÎ ‰‡ Á‡·‡‚Ë ÔÓ„ÂÒËﬂÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÔÓ‰Ó·Ë Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ ·ÓÎÌËÚÂ. éÒÌÓ‚ÌË ÂÎÂÏÂÌÚË Ì‡ ÒËÏÔÚÓÏ‡ÚË˜ÌÓÚÓ ÎÂ˜ÂÌËÂ Ò‡ ÙËÁËÓÚÂ‡ÔËﬂÚ‡, ‰Ëı‡ÚÂÎÌ‡Ú‡ Â‡ÌËÏ‡ˆËﬂ, ÏÂ‰ËÍ‡ÏÂÌÚÓÁÌ‡Ú‡ ÚÂ‡ÔËﬂ Ì‡ Í‡‰ËÓÏËÓÔ‡ÚËﬂÚ‡ Ë ÓÚÓÔÂ‰Ë˜Ì‡Ú‡ ıËÛ„Ëﬂ. äÓÚËÍÓÒÚÂÓË‰ËÚÂ Ò‡ ÁÎ‡ÚÂÌ ÒÚ‡Ì‰‡Ú ‚ ÎÂ˜ÂÌËÂÚÓ Ì‡ ÔÓ„ÂÒË‚ÌËÚÂ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË Ñ˛¯ÂÌ, ‚˙ÔÂÍË ˜Â ÚÓ˜ÌËﬂÚ ËÏ ÏÂı‡ÌËÁ˙Ï Ì‡ ‰ÂÈÒÚ‚ËÂ Â ‚ÒÂ Ó˘Â ÌÂﬂÒÂÌ. ëÂËÓÁÌË Ì‡‰ÂÊ‰Ë ÒÂ ‚˙ÁÎ‡„‡Ú Ì‡ Ô‡ÚÓ„ÂÌÂÚË˜Ì‡Ú‡ „ÂÌÌ‡ ÚÂ‡ÔËﬂ, ˜ËËÚÓ ‡ÁÎË˜ÌË ÔÓ‰ıÓ‰Ë Ò‡ ‚ÒÂ Ó˘Â ‚ ÂÚ‡Ô Ì‡ ÍÎËÌË˜ÌË ÔÓÛ˜‚‡ÌËﬂ ‚˙ıÛ ÊË‚ÓÚËÌÒÍË ÏÓ‰ÂÎË Ë ‚ in vitro ÛÒÎÓ‚Ëﬂ. íÂÁË ‡‚‡Ì„‡‰ÌË ÚÂıÌÓÎÓ„ËË ‚ÍÎ˛˜‚ﬂÚ ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ „ÂÌÌ‡Ú‡ ÂÍÒÔÂÒËﬂ Ì‡ ‰ËÒÚÓÙËÌ ˜ÂÁ: „ÂÌ-Á‡ÏÂÒÚËÚÂÎÌ‡ ÚÂ‡ÔËﬂ Ò˙Ò ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË (ÏÛÒÍÛÎÌË Ë ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË, ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚÓÏ‡ÎÌË ÍÎÂÚÍË); Ñçä/êçä Ï‡ÌËÔÛÎË‡ÌÂ; Ù‡Ï‡ÍÓÎÓ„Ë˜ÌË ‡„ÂÌÚË (ÏÂ‰ËÍ‡ÏÂÌÚË, ÒÚËÏÛÎË‡˘Ë „ÂÌÌ‡Ú‡ ÂÍÒÔÂÒËﬂ Ì‡ ÛÓÚÓÙËÌ; ÏÂ‰ËÍ‡ÏÂÌÚË, ÓÒË„Ûﬂ‚‡˘Ë Ú‡ÌÒÍËÔˆËﬂ Ë ÒÎÂ‰ ÔÂÊ‰Â‚ÂÏÂÌÌÓ ÔÓﬂ‚ËÎ ÒÂ ÒÚÓÔ ÍÓ‰ÓÌ; ‚Ì‡ÒﬂÌÂ ˜ÂÁ ‚ËÛÒÌË ‡„ÂÌÚË Ì‡ ‡ÒÚÂÊÌË Ù‡ÍÚÓË; ÔÓÚÂ‡ÁÌË ËÌıË·ËÚÓË, Á‡·‡‚ﬂ˘Ë ÏÛÒÍÛÎÌ‡Ú‡ Û‚Â‰‡; Â‡„ÂÌÚË, ÔÓ‰ÚËÒÍ‡˘Ë ‚˙ÁÔ‡ÎÂÌËÂÚÓ). ç‡È-Ó·Â˘‡‚‡˘Ë Á‡ ÒÂ„‡ Ò‡ „ÂÌÌÓ- ÏÓ‰ËÙËˆË‡˘ËÚÂ ÏÂÚÓ‰Ë - Ñçä/êçä Ï‡ÌËÔÛÎË‡ÌÂ Ò ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚË‰Ë - ÔÂÒÍ‡˜‡ÌÂÚÓ Ì‡ ÂÍÁÓÌ, ÒËÌÚÂÁ Ì‡ Í˙ÒË ‰ËÒÚÓÙËÌÓ‚Ë ÙÓÏË Ë ÔÂÏËÌ‡‚‡ÌÂ ÓÚ ÚÂÊÍ‡ ÏÛÚ‡ˆËﬂ Ò ÍÎËÌËÍ‡ Ì‡ Ñ˛¯ÂÌ ‚ ÔÓ-ÎÂÍ‡ Ò ÙÂÌÓÚËÔ Ì‡ èåÑ ÚËÔ ÅÂÍÂ. äãûóéÇà Ñìåà: ÑËÒÚÓÙËÌÓÔ‡ÚËË, ÔÓ„ÂÒË‚Ì‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÚËÔ Ñ˛¯ÂÌ, ÔÓ„ÂÒË‚Ì‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÚËÔ ÅÂÍÂ, ÙËÁËÓÚÂ‡ÔËﬂÚ‡, ‰Ëı‡ÚÂÎÌ‡Ú‡ Â‡ÌËÏ‡ˆËﬂ, ÏÂ‰ËÍ‡ÏÂÌÚÓÁÌ‡Ú‡ ÚÂ‡ÔËﬂ Ì‡ Í‡‰ËÓÏËÓÔ‡ÚËﬂÚ‡, ÓÚÓÔÂ‰Ë˜Ì‡Ú‡ ıËÛ„Ëﬂ, ÍÓÚËÍÓÒÚÂÓË‰Ë, „ÂÌ-Á‡ÏÂÒÚËÚÂÎÌ‡ ÚÂ‡ÔËﬂ Ò˙Ò ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË, Ñçä/êçä Ï‡ÌËÔÛÎË‡ÌÂ; Ù‡Ï‡ÍÓÎÓ„Ë˜ÌË ‡„ÂÌÚË. èÓ„ÂÒË‚ÌËÚÂ ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË ÚËÔ Ñ˛¯ÂÌ Ë ÚËÔ ÅÂÍÂ Ò‡ Ì‡ÒÎÂ‰ÒÚ‚ÂÌË ÏËÓÔ‡ÚËË, Ó·ÛÒÎÓ‚ÂÌË ÓÚ ÏÛÚ‡ˆËË, Á‡Òﬂ„‡˘Ë ‰ËÒÚÓÙËÌÓ‚Ëﬂ „ÂÌ, ÎÓÍ‡ÎËÁË‡Ì ‚ ï ıÓÏÓÁÓÏ‡Ú‡ (2). èÂÁ ÔÓÒÎÂ‰ÌËÚÂ „Ó‰ËÌË ÓÒ‚ÂÌ ÛÚ‚˙‰ÂÌ‡Ú‡ ÍÓÏÔÎÂÍÒÌ‡ ÒËÏÔÚÓÏ‡ÚË˜Ì‡ ÚÂ‡ÔËﬂ Á‡ ÎÂ˜ÂÌËÂ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ (11,13,17), Ò˙˘ÂÒÚ‚ÂÌ Â Ì‡ÔÂ‰˙Í˙Ú ‚ ÂÚËÓÔ‡ÚÓ„ÂÌÂÚË˜ÌÓÚÓ ËÏ ÎÂ˜ÂÌËÂ, ‚ÍÎ˛˜‚‡˘Ó „ÂÌÌ‡ ÚÂ‡ÔËﬂ (3,29,32). á‡ ÒÂ„‡ „ÂÌÌ‡Ú‡ ÚÂ‡ÔËﬂ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Â ‚ ÂÚ‡Ô Ì‡ ÂÍÒÔÂËÏÂÌÚ‡ÎÌË ËÁÒÎÂ‰‚‡ÌËﬂ (5,6,10,20,26,27,28) Ë ÍÎËÌË˜ÌË ÔÓÛ˜‚‡ÌËﬂ (3,7,19,22,23). I. äéåèãÖäëçÄ íÖêÄèà çÄ ÑàëíêéîàçéèÄíààíÖ ç‡ ÚÓÁË ÂÚ‡Ô Ò˙˘ÂÒÚ‚Û‚‡Ú ˜ÂÚËË ÓÒÌÓ‚ÌË Ì‡ÒÓÍË ‚ ÍÓÏÔÎÂÍÒÌ‡Ú‡ ÚÂ‡ÔËﬂ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ, ÓÚ‡ÁÂÌ‡ ‚ ÔËÂÚË Ô‡‚ËÎ‡ Ë ÍÓÌÒÂÌÒÛÒË (1,13,14): ● èÓ‰˙Ê‡ÌÂ Ë ÔÓ‰Ó·ﬂ‚‡ÌÂ Ì‡ ÏÛÒÍÛÎÌ‡Ú‡ ÒËÎ‡ Ë ÙÛÌÍˆËﬂ ● èÂ‚ÂÌˆËﬂ Ì‡ ‰ÂÙÓÏ‡ˆËËÚÂ Ì‡ „˙·Ì‡˜ÌËﬂ ÒÚ˙Î· ● èÓÙËÎ‡ÍÚËÍ‡ Ì‡ ‰Ëı‡ÚÂÎÌËÚÂ ÛÒÎÓÊÌÂÌËﬂ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ● ãÂ˜ÂÌËÂ Ì‡ Í‡‰ËÓÏËÓÔ‡ÚËﬂÚ‡ Ç Ì‡ÒÚÓﬂ˘Ëﬂ ÏÓÏÂÌÚ ÓÒÌÓ‚ÌÓ ÔËÎÓÊÂÌËÂ Ì‡ÏË‡ ÚÂ‡ÔËﬂÚ‡ Ò ÍÓÚËÍÓÒÚÂÓË‰Ë, ÄëÖ-ËÌıË·ËÚÓË, ‚·ÎÓÍÂË, ÓÚÓÔÂ‰Ë˜Ì‡Ú‡ ıËÛ„Ëﬂ Ë ÙËÁËÓÚÂ‡ÔËﬂÚ‡, ˜ÂÁ ÍÓËÚÓ ÒÂ ˆÂÎË ÔÓ‰Ó·ﬂ‚‡ÌÂ Ì‡ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ. 1. äéêíàäéëíÖêéàÑà (äë). ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â Prednisolone Ë Deflazacort Ò‡ ÁÎ‡ÚÂÌ ÒÚ‡Ì‰‡Ú ‚ ÎÂ˜ÂÌËÂÚÓ Ì‡ ÏÛÒÍÛÎÌ‡Ú‡ ÒÎ‡·ÓÒÚ ÔË ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ (17,24). ÖÊÂ‰ÌÂ‚ÌÓÚÓ ËÏ ÔËÎÓÊÂÌËÂ ËÎË ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‡ÎÚÂÌË‡˘Ë ÒıÂÏË ‚ÂÏÂÌÌÓ Á‡·‡‚ﬂ ÔÓ„ÂÒËﬂÚ‡ Ì‡ ÏÛÒÍÛÎÌ‡Ú‡ ‰ËÒÚÓÙËﬂ, ÒÚ‡·ËÎËÁË‡ ÏÛÒÍÛÎÌ‡Ú‡ ÒËÎ‡ Ë ÙÛÌÍˆËﬂ, Ì‡Ï‡Îﬂ‚‡ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÛÒÎÓÊÌÂÌËﬂÚ‡ Í‡ÚÓ ÒÍÓÎËÓÁ‡, ÔÓ‰Ó·ﬂ‚‡ ‰Ëı‡ÚÂÎÌ‡Ú‡ ÙÛÌÍˆËﬂ, ËÁÏÂÂÌ‡ ˜ÂÁ ÙÓÒË‡ÌËﬂ ‚ËÚ‡ÎÂÌ Í‡Ô‡ˆËÚÂÚ (îÇä) Ë Ì‡Ï‡Îﬂ‚‡ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ Ò˙‰Â˜ÌËÚÂ ÛÒÎÓÊÌÂÌËﬂ. í˙È Í‡ÚÓ ÔÓ‰˙ÎÊËÚÂÎÌ‡Ú‡ ËÏ ÛÔÓÚÂ·‡ ÏÓÊÂ ‰‡ ‰Ó‚Â‰Â ‰Ó ÒÂËÓÁÌË ÌÂÊÂÎ‡ÌË ÎÂÍ‡ÒÚ‚ÂÌË Â‡ÍˆËË (çãê) Í‡ÚÓ: ‡ÚÂË‡ÎÌ‡ ıËÔÂÚÓÌËﬂ, Á‡ÒÚÓÈÌ‡ Ò˙‰Â˜Ì‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ, ﬂÁ‚ÂÌ‡ ·ÓÎÂÒÚ, ˜ÂÌÓ‰Ó·Ì‡ ÒÚÂ‡ÚÓÁ‡, Ô‡ÌÍÂ‡ÚËÚ, ﬂÚÓ„ÂÌÂÌ ıËÔÂÍÓÚËˆËÁ˙Ï Ò ˆÂÌÚËÔÂÚ‡ÎÌÓ Á‡ÚÎ˙ÒÚﬂ‚‡ÌÂ, facies lunata, ‡ÍÌÂ, ıËÁÛÚËÁ˙Ï, ÒÚÂÓË‰ÂÌ ‰Ë‡·ÂÚ, ‰ËÒÎËÔË‰ÂÏËﬂ, ıËÔÓÍ‡ÎËÂÏËﬂ, ÏÂÚ‡·ÓÎËÚÌ‡ ‡ÎÍ‡ÎÓÁ‡, ÓÒÚÂÓÔÓÓÁ‡ Ò ÍÓÏÔÂÒËÓÌÌË Ù‡ÍÚÛË (8), ÍÓÌ‚ÛÎÒËË, ·ÂÌË„ÌÂÌ‡ ËÌÚ‡Í‡ÌË‡ÎÌ‡ ıËÔÂÚÂÌÁËﬂ, ÂÏÓˆËÓÌ‡ÎÌ‡ Î‡·ËÎÌÓÒÚ Ë ÔÒËıÓÁË, Í‡Ú‡‡ÍÚ‡ Ë ËÏÛÌÓÒÛÔÂÒËﬂ Ò ÂˆË‰Ë‚Ë‡˘Ë ËÌÙÂÍˆËË, ÔËÎÓÊÂÌËÂÚÓ ËÏ ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ, ÒÎÂ‰‚‡ ‰‡ ·˙‰Â ‚ÌËÏ‡ÚÂÎÌÓ ÔÂˆÂÌÂÌÓ. åÂı‡ÌËÁÏËÚÂ Ì‡ ‰ÂÈÒÚ‚ËÂ Ì‡ äë Ì‡ ÍÎÂÚ˙˜ÌÓ ÌË‚Ó, ÍÓËÚÓ ‚Ó‰ﬂÚ ‰Ó Á‡·‡‚ﬂÌÂ Ì‡ ÔÓ„ÂÒËﬂÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, ÓÒÚ‡‚‡Ú ÌÂﬂÒÌË. ë˙˘ÂÒÚ‚Û‚‡Ú ‡ÁÎË˜ÌË ÚÂÓËË, ÔÓÛ˜ÂÌË ÔË ÂÍÒÔÂËÏÂÌÚ‡ÎÌË ÏÓ‰ÂÎË Ì‡ ÏË¯ÍË (14): ● èÓÏﬂÌ‡ ‚ ÍÓÎË˜ÂÒÚ‚ÓÚÓ Ì‡ Ëêçä (ËÌÙÓÏ‡ˆËÓÌÌ‡ êçä) Á‡ ÒÚÛÍÚÛÌË, ÒË„Ì‡ÎÌË Ë „ÂÌË, Û˜‡ÒÚ‚‡˘Ë ‚ ËÏÛÌÌËﬂ ÓÚ„Ó‚Ó ● ç‡Ï‡Îﬂ‚‡ÌÂ ·Óﬂ Ì‡ ˆËÚÓÚÓÍÒË˜ÌËÚÂ í-ÍÎÂÚÍË ● é„‡ÌË˜‡‚‡ÌÂ Ì‡ Í‡ÎˆËÂ‚Ëﬂ ËÌÙÎÛÍÒ Ë ‚˙ÚÂÍÎÂÚ˙˜Ì‡Ú‡ Í‡ÎˆËÂ‚‡ ÍÓÌˆÂÌÚ‡ˆËﬂ ● èÓ‚Ë¯‡‚‡ÌÂ Ì‡ ÂÍÒÔÂÒËﬂÚ‡ Ì‡ Î‡ÏËÌËÌ Ë ÒÚËÏÛÎË‡ÌÂ Ì‡ ÏÛÒÍÛÎÌÓÚÓ ‚˙ÁÒÚ‡ÌÓ‚ﬂ‚‡ÌÂ ● éÚÒÓ˜‚‡ÌÂ ‚˙‚ ‚ÂÏÂÚÓ Ì‡ ‡ÔÓÔÚÓÁ‡Ú‡ Ì‡ ÏÛÒÍÛÎÌËÚÂ ‚Î‡ÍÌ‡ Ë ÍÎÂÚ˙˜Ì‡Ú‡ ËÌÙËÎÚ‡ˆËﬂ ● èÓ‚Ë¯‡‚‡ÌÂ Ì‡ ÂÍÒÔÂÒËﬂÚ‡ Ì‡ ‰ËÒÚÓÙËÌ ● èÓ‚ÎËﬂ‚‡ÌÂ ÌÂ‚ÌÓÏÛÒÍÛÎÌÓÚÓ ÔÂ‰‡‚‡ÌÂ ● èÂ‰Ô‡Á‚‡ÌÂ Ì‡ ÏÛÒÍÛÎÌËÚÂ ‚Î‡ÍÌ‡ ÓÚ ÏÂı‡ÌË˜Ì‡ Û‚Â‰‡ ● ç‡Ï‡Îﬂ‚‡ÌÂ ÌÂÍÓÁ‡Ú‡ Ì‡ ÏËÓˆËÚËÚÂ ● èÓ‚Ë¯‡‚‡ÌÂ Ì‡ ÌË‚‡Ú‡ Ì‡ ÏÛÒÍÛÎÌËﬂ Ú‡ÛËÌ Ë ÍÂ‡ÚËÌ ë˙˘ÂÒÚ‚Û‚‡Ú ‡ÁÎË˜ÌË ÒıÂÏË Ì‡ ÔËÎÓÊÂÌËÂ Ì‡ äë, ÔË ÍÓËÚÓ ÚÂ ÏÓ„‡Ú ‰‡ ÒÂ ÔËÎ‡„‡Ú ‚ÒÂÍË ‰ÂÌ ËÎË ‡ÎÚÂÌË‡˘Ó. Prednisolone ÒÂ ‰ÓÁË‡ ÔÓ 0,75 Ï„/Í„/‰ÌÂ‚ÌÓ, Deflazacort ÔÓ 0,9 Ï„/Í„/‰ÌÂ‚ÌÓ (25). äÎËÌË˜ÌËÚÂ ÔÓÛ˜‚‡ÌËﬂ ÔÓÍ‡Á‚‡Ú, ˜Â ÚÂÁË Ì‡˜ËÌË Ì‡ ÔËÎÓÊÂÌËÂ ËÏ‡Ú ‰Ó·˙ ÂÙÂÍÚ ‚˙ıÛ ÏÛÒÍÛÎÌ‡Ú‡ ÒËÎ‡ Ë ‰Ëı‡ÚÂÎÌ‡Ú‡ ÙÛÌÍˆËﬂ ‚ Í‡ÚÍÓÒÓ˜ÂÌ ÔÎ‡Ì (‰˙Î„ÓÒÓ˜ÌËÚÂ ÂÙÂÍÚË ÒÎÂ‰‚‡ ‰‡ ÒÂ ÓÚ˜ËÚ‡Ú ‚ ·˙‰Â˘Â). ÑÛ„Ë ÒıÂÏË, ÓÔËÒ‡ÌË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ò‡: Prednisolone 0,3-1,5 Ï„/Í„/‰ÌÂ‚ÌÓ. èË ÔÓ-ÌËÒÍËÚÂ ‰ÓÁËÓ‚ÍË 0,3-0,5 Ï„/Í„ ËÏ‡ ÔÓ-Ï‡Î˙Í ËÒÍ ÓÚ çãê, ÌÓ Ë ÔÓ-ÒÎ‡· ÚÂ‡ÔÂ‚ÚË˜ÂÌ ÂÙÂÍÚ. ÄÎÚÂÌË‡˘Ë ÒıÂÏË: Prednisolone 0,75 Ï„/Í„/‰ÌÂ‚ÌÓ Ò Â‰Û‚‡ÌÂ Ì‡ ÔÂËÓ‰Ë ÓÚ 10 ‰ÂÌ‡ ÔËÂÏ Ë 10 ‰ÂÌ‡ ÔÓ˜Ë‚Í‡. Prednisolone 0,65 Ï„/Í„/‰ÌÂ‚ÌÓ 20 ‰ÂÌ‡ ÔËÂÏ 10 ‰ÂÌ‡ ÔÓ˜Ë‚Í‡. èÂÔÓ˙˜‚‡ ÒÂ ÚÂ‡ÔËﬂÚ‡ ‰‡ ÒÂ Á‡ÔÓ˜ÌÂ, ‰ÓÍ‡ÚÓ 163 ÏÓÏ˜ÂÚÓ ÌÂ Â Á‡„Û·ËÎÓ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ÒË ‰‡ ıÓ‰Ë (Ì‡È˜ÂÒÚÓ ÒÎÂ‰ 4-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ). Prednisolone Ë Deflazacort ÔÓÍ‡Á‚‡Ú ÒıÓ‰Ì‡ ÂÙÂÍÚË‚ÌÓÒÚ, ÌÓ ÒÂ ‡ÁÎË˜‡‚‡Ú ÔÓ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ çêã, ÍÓËÚÓ ÔË˜ËÌﬂ‚‡Ú. Deflazacort ÔÓ-ﬂ‰ÍÓ ‚Ó‰Ë ‰Ó ÔÓ‚Ë¯‡‚‡ÌÂ Ì‡ ÚÂÎÂÒÌ‡Ú‡ Ï‡Ò‡, ÌÓ ˜ÂÒÚÓ ÔË˜ËÌﬂ‚‡ ‡ÒËÏÔÚÓÏÌ‡ Í‡Ú‡‡ÍÚ‡ (9,14). èÓﬂ‚‡Ú‡ Ì‡ ÒÂËÓÁÌË çãê, Í‡ÚÓ „ÓÂËÁ·ÓÂÌËÚÂ Â ËÌ‰ËÍ‡ˆËﬂ Á‡ ÔÂÛÒÚ‡ÌÓ‚ﬂ‚‡ÌÂ Ì‡ ÚÂ‡ÔËﬂÚ‡ Ò ÍÓÚËÍÓÒÚÂÓË‰Ë. Ç ÒÎÛ˜‡È, ˜Â Ú‡ÍË‚‡ ÌÂ Ò‡ Ì‡ÎËˆÂ, ÚÂ‡ÔËﬂÚ‡ ÔÓ‰˙ÎÊ‡‚‡ ‰ÓÍ‡ÚÓ Ô‡ˆËÂÌÚËÚÂ Á‡„Û·ﬂÚ ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ ÒÂ ÔË‰‚ËÊ‚‡Ú Ò‡ÏÓÒÚÓﬂÚÂÎÌÓ. ÉÓÎÂÏËﬂÚ ·ÓÈ çãê, ‰˙ÎÊ‡˘Ë ÒÂ Ì‡ ÔÓ‰˙ÎÊËÚÂÎÌ‡ ÚÂ‡ÔËﬂ Ò äë, Ì‡Î‡„‡ Ô‡ˆËÂÌÚËÚÂ ‰‡ ·˙‰‡Ú Â‰Ó‚ÌÓ ÔÓÒÎÂ‰ﬂ‚‡ÌË, Í‡ÚÓ ÒÂ ÒÎÂ‰ﬂÚ: ‚ËÒÓ˜ËÌ‡, ÚÂÎÂÒÌ‡ Ï‡Ò‡, ÄÚÂË‡ÎÌÓ Ì‡Îﬂ„‡ÌÂ, „Î˛ÍÓÁ‡Ú‡ ‚ ÛËÌ‡Ú‡, ÔÓ‚Â‰ÂÌËÂÚÓ Á‡ Ò˙ÓÚ‚ÂÚÌË ÓÚÍÎÓÌÂÌËﬂ, Ó˜ÂÌ ÒÚ‡ÚÛÒ, ı‡ÌËÚÂÎÂÌ ÂÊËÏ, ‰ÓÒÚ‡Ú˙˜ÂÌ ÔËÂÏ Ì‡ Í‡ÎˆËÈ Ë Vitamin D, DEXA (Dual Energy X-Ray Absorbtiometry). åÌÓ„Ó ˜ÂÒÚÓ Á‡„Û·‡Ú‡ Ì‡ ÒÔÓÒÓ·ÌÓÒÚ‡ Á‡ Ò‡ÏÓÒÚÓﬂÚÂÎÌÓ ıÓ‰ÂÌÂ Ò‡ ﬂ‚ﬂ‚‡ Â‰ËÌ ÓÚ Ù‡ÍÚÓËÚÂ, Ó·ÛÒÎ‡‚ﬂ˘Ë ÌËÒÍ‡Ú‡ ÍÓÒÚÌ‡ ÔÎ˙ÚÌÓÒÚ Ë ÔÓÒÎÂ‰‚‡˘Ë Ù‡ÍÚÛË, Ì‡È-˜ÂÒÚÓ ÔÂ¯ÎÂÌÌË. äÓÚËÍÓÒÚÂÓË‰ËÚÂ Ò˙˘Ó ËÏ‡Ú ÌÂ·Î‡„ÓÔËﬂÚÂÌ ÂÙÂÍÚ ‚˙ıÛ ÍÓÒÚÌ‡Ú‡ ÒËÒÚÂÏ‡, Í‡ÚÓ ÔÓÛ˜‚‡ÌËﬂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú, ˜Â Â‰ÛÍˆËﬂÚ‡ Ì‡ ÍÓÒÚÌ‡Ú‡ ÔÎ˙ÚÌÓÒÚ, Ó·ÛÒÎÓ‚ÂÌ‡ ÓÚ Ú‡ÁË ÚÂ‡ÔËﬂ, Â Ì‡È-ÒËÎÌÓ ËÁ‡ÁÂÌ‡ ‚ ‰ËÒÚ‡ÎÌËÚÂ ˜‡ÒÚË Ì‡ ÏÂÚ‡ÙËÁËÚÂ Ì‡ ÍÓÒÚËÚÂ. á‡ ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ ÍÓÚËÍÓÒÚÂÓË‰-ËÌ‰ÛˆË‡Ì‡Ú‡ ÓÒÚÂÓÔÓÓÁ‡ Ë ÍÓÏÔÂÒËÓÌÌË Ù‡ÍÚÛË ‚ Ò˙Ó·‡ÊÂÌËÂ ‚ÎËÁ‡Ú ÒÎ˙Ì˜Â‚‡ ÂÍÒÔÓÁËˆËﬂ, ‰ËÂÚ‡, ·Ó„‡Ú‡ Ì‡ Í‡ÎˆËÈ, ÚÂ‡ÔËﬂÚ‡ Ò Vitamin D (‰Ó 400Ö/‰ÂÌ) + ä‡ÎˆËÈ (1000Ï„/‰ÂÌ), ËÎË ·ËÙÓÒÙÓÌ‡ÚË. àÏÛÌÓÒÛÔÂÒË‚ÌËﬂÚ ÂÙÂÍÚ Ì‡ ÍÓÚËÍÓÒÚÂÓË‰ËÚÂ Ì‡Î‡„‡ ÔÂ‰Ë Ì‡˜‡ÎÓÚÓ Ì‡ ÚÂ‡ÔËﬂÚ‡ ‰‡ ÒÂ ËÁÍÎ˛˜Ë ‡ÍÚË‚Ì‡ ÚÛ·ÂÍÛÎÓÁÌ‡ ËÌÙÂÍˆËﬂ, ‡ ‚˙‚ ÇÂÎËÍÓ·ËÚ‡ÌËﬂ ÒÂ ÔËÎ‡„‡ Ë ‚‡ÍÒËÌ‡ˆËﬂ ÒÂ˘Û ‚‡ËˆÂÎ‡. èÓ ‚ÂÏÂ Ì‡ ÍÓÚËÍÓÒÚÂÓË‰Ì‡Ú‡ ÚÂ‡ÔËﬂ ÒÂ ÔÓÒÎÂ‰ﬂ‚‡ Ó·ÂÍÚË‚ÌÓ ÏÛÒÍÛÎÌ‡Ú‡ ÒËÎ‡, ‰Ëı‡ÚÂÎÌ‡Ú‡, Ò˙‰Â˜ÌÓÒ˙‰Ó‚ËÚÂ ÙÛÌÍˆËË Ë ÔÒËıË˜ÂÒÍÓÚÓ Ò˙ÒÚÓﬂÌËÂ, ÔÓﬂ‚‡Ú‡ Ì‡ çêã. èË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ ÅÂÍÂ ÂÙÂÍÚ˙Ú Ì‡ ÚÂ‡ÔËﬂ Ò ÍÓÚËÍÓÒÚÂÓË‰Ë ÌÂ Â ÔÓÛ˜ÂÌ, ÔÓ‡‰Ë ÔÓ-·‡‚Ì‡Ú‡ ÔÓ„ÂÒËﬂ Ë ÔÓ-Í˙ÒÌ‡Ú‡ ËÌ‚‡ÎË‰ËÁ‡ˆËﬂ. 2. îàáàéíÖêÄèàüíÄ C˙˘Ó Â ‚‡ÊÂÌ ÂÎÂÏÂÌÚ ÓÚ ÍÓÏÔÎÂÍÒÌÓÚÓ ÎÂ˜ÂÌËÂ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ. èËÎ‡„‡ ÒÂ ‚˙‚ ‚ÒÂÍË Â‰ËÌ ÏÓÏÂÌÚ ÓÚ ÔÓ„ÂÒËﬂÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Í‡ÍÚÓ ÔË ÔÓ‰‚ËÊÌË Ú‡Í‡ Ë ÔË Á‡„Û·ËÎË ÒÔÓÒÓ·ÌÓÒÚÚ‡ ÒË ‰‡ ıÓ‰ﬂÚ Ô‡ˆËÂÌÚË. éÒÌÓ‚ËÚÂ È ˆÂÎË Ò‡ ‰‡ Á‡·‡‚Ë Ì‡Ï‡ÎÂÌËÂÚÓ Ì‡ ÏÛÒÍÛÎÌ‡Ú‡ ÒËÎ‡ Ë Ï‡Ò‡, ‰‡ ÔÓÙËÎ‡ÍÚË‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÒÚ‡‚ÌËÚÂ ÍÓÌÚ‡ÍÚÛË Ë ‰‡ ÔÓ‰Ó·Ë Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ ·ÓÎÌËÚÂ. îËÁËÓÚÂ‡ÔËﬂ ÒÎÂ‰‚‡ ‰‡ ÒÂ Á‡ÔÓ˜ÌÂ ÌÂÔÓÒÂ‰ÒÚ‚ÂÌÓ ÒÎÂ‰ ÔÓÒÚ‡‚ﬂÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡. è˙‚ÓÌ‡˜‡ÎÂÌ ÔËÓËÚÂÚ Â ÔÂ‚ÂÌˆËﬂÚ‡ Ì‡ ‚˙ÁÌËÍ‚‡ÌÂÚÓ Ì‡ ‡ÒËÏÂÚË˜ÌË ÍÓÌÚ‡ÍÚÛË Ì‡ ‡ıËÎÂÒÓ‚ËÚÂ ÒÛıÓÊËÎËﬂ, ÍÓÎÂÌÌËÚÂ Ë Ú‡ÁÓ·Â‰ÂÌËÚÂ ÒÚ‡‚Ë, Í‡ÚÓ ÔÓ ÚÓÁË Ì‡˜ËÌ ÒÂ Ì‡Ï‡Îﬂ‚‡ ËÒÍ‡ ÓÚ ÔÓÒÎÂ‰‚‡˘‡ ÒÍÓÎËÓÁ‡. è‡ÒË‚ÌËÚÂ ‡ÁÚﬂ„‡ÌËﬂ Ë ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ „ÎÂÁÂÌÌË ÓÚÂÁË Ò‡ ÓÒÌÓ‚ÂÌ ÏÓÏÂÌÚ ÓÚ ÎÂ˜ÂÌËÂÚÓ ‚ ÂÚ‡Ô, ÍÓ„‡ÚÓ Ô‡ˆËÂÌÚËÚÂ Ò‡ ‚ÒÂ Ó˘Â ÔÓ‰‚ËÊÌË. ÉÎÂÁÂÌÌËÚÂ ÓÚÂÁË ÒÂ ÔÓÒÚ‡‚ﬂÚ ÌÓ˘ÂÏ, Á‡˘ÓÚÓ Á‡ÚÛ‰Ìﬂ‚‡Ú ıÓ‰ÂÌÂÚÓ ÔÂÁ ‰ÂÌﬂ. åÓÏ˜ÂÚ‡Ú‡ ÒÎÂ‰‚‡ ‰‡ ÒÂ ÒÚËÏÛÎË‡Ú ‰‡ Ô‡ÍÚËÍÛ‚‡Ú ÔÓ‰ıÓ‰ﬂ˘Ë ÙËÁË˜ÂÒÍË ÛÔ‡ÊÌÂÌËﬂ Ë ıË‰ÓÚÂ‡ÔËﬂ. àÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÍÓÎﬂÌÌÓ-„ÎÂÁÂÌÌË ÓÚÂÁË ÏÓÊÂ ‰‡ Û‰˙ÎÊË ‚˙‚ ‚ÂÏÂÚÓ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚﬂÚÂÎÌÓ ıÓ‰ÂÌÂ Ë ÒÚÓÂÌÂ Ô‡‚. çÂ ﬂ‰ÍÓ ÚﬂıÌ‡Ú‡ ÛÔÓÚÂ·‡ ÒÂ ÔÂ‰¯ÂÒÚ‚‡ ÓÚ ÚÂÌ‰ÓÂÎÓÌ„‡ˆËË Ì‡ ‡ıËÎÂÒÓ‚ËÚÂ ÒÛıÓÊËÎËﬂ. ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 3. éêíéèÖÑàóçÄ ïàêìêÉàü éÍÓÎÓ 90% ÓÚ ÏÓÏ˜ÂÚ‡Ú‡ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ ‡Á‚Ë‚‡Ú ÍÎËÌË˜ÌÓ ÁÌ‡˜ËÏ‡ ÒÍÓÎËÓÁ‡. èÓ‰ıÓ‰ﬂ˘‡Ú‡ ÔÓÙËÎ‡ÍÚËÍ‡ ÒÂ˘Û „˙·Ì‡˜ÌËÚÂ ËÁÍË‚ﬂ‚‡ÌËﬂ ÔË ÚÂÁË Ô‡ˆËÂÌÚË Úﬂ·‚‡ ‰‡ Á‡ÔÓ˜ÌÂ ÔÂ‰Ë ‰‡ Ò‡ ËÁ„Û·ËÎË ÒÔÓÒÓ·ÌÓÒÚÚ‡ ÒË ‰‡ ÒÂ ‰‚ËÊ‡Ú Ë ‚ÍÎ˛˜‚‡ ÙËÁËÓÚÂ‡ÔÂ‚ÚË˜ÌË ÔÓˆÂ‰ÛË Ë ÔÓ‰ıÓ‰ﬂ˘‡ ÔÓÁ‡, ËÁ·ﬂ„‚‡˘‡ Ú‡ÁÓ‚‡Ú‡ ‡ÒËÏÂÚËﬂ. ÇÂ‰Ì˙Ê ÔÓﬂ‚ËÎ‡ ÒÂ, ÒÍÓÎËÓÁ‡Ú‡ ÏÓÊÂ ‰‡ ·˙‰Â ÍÓË„Ë‡Ì‡ ıËÛ„Ë˜ÌÓ. ë Ì‡È-‰Ó·˙ ÂÙÂÍÚ Ò‡ ÚÂÁË ËÌÚÂ‚ÂÌˆËË, ËÁ‚˙¯ÂÌË Ì‡ ÂÚ‡Ô, ÍÓ„‡ÚÓ „˙·Ì‡˜ÌËﬂÚ ÒÚ˙Î· Â ‚ÒÂ Ó˘Â ÔÓ‰‚ËÊÂÌ Ë ˙„˙Î˙Ú Ì‡ äÓ· Â 20-401 (15). Ç ÚÂÁË ÔÓ‡ÌÌË ÒÚ‡‰ËË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ‰Ëı‡ÚÂÎÌ‡Ú‡ Ë Ò˙‰Â˜Ì‡Ú‡ ÙÛÌÍˆËË Ì‡ ÚÂÁË ·ÓÎÌË Ò‡ ‰ÓÒÚ‡Ú˙˜ÌÓ Ò˙ı‡ÌÂÌË, Á‡ ‰‡ ÏÓÊÂ Ó„‡ÌËÁÏ˙Ú ËÏ ‰‡ ÔÓÌÂÒÂ ÓÔÂ‡ÚË‚Ì‡ ËÌÚÂ‚ÂÌˆËﬂ. èË Ô‡ˆËÂÌÚË, ÍÓÌÚ‡ËÌ‰ËˆË‡ÌË Á‡ ÓÔÂ‡ˆËË ÒÂ ËÁÔÓÎÁ‚‡Ú Ò˙ÓÚ‚ÂÚÌË ÒÚ‡·ËÎËÁË‡˘Ë ÍÓÎ‡ÌË Ë ÍÓÒÂÚË. íÂÌ‰ÓÂÎÓÌ„‡ˆËËÚÂ Ì‡ ÄıËÎÂÒÓ‚ËÚÂ ÒÛıÓÊËÎËﬂ Ò˙˘Ó ËÏ‡Ú ÁÌ‡˜ÂÌËÂ Á‡ Û‰˙ÎÊ‡‚‡ÌÂ ‚˙‚ ‚ÂÏÂÚÓ Ì‡ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Ì‡ ÚÂÁË ·ÓÎÌË ‰‡ ıÓ‰ﬂÚ Ò‡ÏÓÒÚÓﬂÚÂÎÌÓ. 4. åÖÑàäÄåÖçíéáçÄ íÖêÄèàü çÄ äÄêÑàéåàéèÄíàüíÄ ä‡‰ËÓÏËÓÔ‡ÚËﬂÚ‡ Â ˜ÂÒÚÓ ÛÒÎÓÊÌËÂÌËÂ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËË. èÓ-Í˙ÒÌ‡Ú‡ È ËÁﬂ‚‡ ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò Á‡Òﬂ„‡ÌÂÚÓ Ì‡ ÒÍÂÎÂÚÌËÚÂ ÏÛÒÍÛÎË ÒÂ Ó·ÛÒÎ‡‚ﬂ ÓÚ Ì‡Ï‡ÎÂÌ‡Ú‡ ÙËÁË˜ÂÒÍ‡ ‡ÍÚË‚ÌÓÒÚ ÔË Ô‡ˆËÂÌÚËÚÂ, ‚ÒÎÂ‰ÒÚ‚ËÂ Á‡„Û·‡Ú‡ Ì‡ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ Ò‡ÏÓÒÚÓﬂÚÂÎÌ‡ ÔÓıÓ‰Í‡. ë˙‰Â˜ÌÓÚÓ Á‡Òﬂ„‡ÌÂ Â ÌÂÔÓÒÂ‰ÒÚ‚ÂÌ‡ ÔË˜ËÌ‡ Á‡ ÒÏ˙ÚÚ‡ ÔË ÓÍÓÎÓ 10% ÓÚ ÚÂÁË Ô‡ˆËÂÌÚË (12). èÂÔÓ˙˜‚‡ÌËÚÂ ÏÂ‰ËÍ‡ÏÂÌÚË Á‡ ÚÂ‡ÔËﬂ Í‡‰ËÓÏËÓÔ‡ÚËﬂÚ‡ ÔË Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ Ò‡: ÄëÖ-ËÌıË·ËÚÓË ËÎË ÄÌ„ËÓÚÂÌÁËÌ-ÂˆÂÔÚÓÌË ·ÎÓÍÂË, ·ËÏÍÓ‚Ë ‰ËÛÂÚËˆË, ÒÔËÓÌÓÎ‡ÍÚÓÌ Ë ‚ ‰ÓÔ˙ÎÌÂÌËÂ ÌÂÒÂÎÂÍÚË‚ÌË ‚-·ÎÓÍÂË ‚ ÔÓ-Ì‡ÔÂ‰Ì‡ÎËÚÂ ÒÚ‡‰ËË Ì‡ Ò˙‰Â˜ÌÓ Á‡Òﬂ„‡ÌÂ. íﬂ ÒÂ ÔËÎ‡„‡ Á‡ Á‡·‡‚ﬂÌÂ Ì‡ ÔÓ„ÂÒËﬂÚ‡ Ì‡ Ò˙‰Â˜ÌÓÚÓ Á‡Òﬂ„‡ÌÂ Ë Á‡ ÔÓ‰Ó·ﬂ‚‡ÌÂ ÔÓ„ÌÓÁ‡Ú‡ Ì‡ Ô‡ˆËÂÌÚË Ò ·ÂÁÒËÏÔÚÓÏÌ‡ ÎÂ‚ÓÍ‡ÏÂÌ‡ ıËÔÂÚÓÙËﬂ (12). ÄëÖ-ËÌıË·ËÚÓËÚÂ Ò‡ ‰ÓÍ‡Á‡ÎË Ò‚ÓﬂÚ‡ ÂÙÂÍÚË‚ÌÓÒÚ ‚ ÔÂ‚ÂÌˆËﬂÚ‡ Ì‡ ÂÏÓ‰ÂÎË‡ÌÂÚÓ Ì‡ ÒÚÂÌ‡Ú‡ Ì‡ Îﬂ‚‡ Í‡ÏÂ‡, ‡Á‡ÒÚ‚‡ÌÂ Ì‡ ÙË·ÓÁ‡ Ë Ó„‡ÌË˜‡‚‡ÌÂÚÓ Ì‡ ÎÂ‚ÓÍÓÏÂÌ‡Ú‡ ıËÔÂÚÓÙËﬂ, ÍÓÂÚÓ Ì‡Ï‡Îﬂ‚‡ ËÒÍ‡ ÓÚ Í‡ÏÂÌË ‡ËÚÏËË Ë ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. äÎËÌË˜ÌË ÔÓÛ˜‚‡ÌËﬂ ÔÓÚ‚˙Ê‰‡‚‡Ú ÂÙÂÍÚË‚ÌÓÒÚÚ‡ Ì‡ ÍÓÏ·ËÌË‡Ì‡ ÚÂ‡ÔËﬂ ÓÚ ÄëÖ-ËÌıË·ËÚÓËÚÂ Ë ‚-·ÎÓÍÂË Á‡ Û‚ÂÎË˜‡‚‡ÌÂ Ì‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ ÊË‚ÓÚ‡ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ÎÂ‚ÓÍÓÏÂÌ‡ ‰ËÒÙÛÌÍˆËﬂ (16). 5. èéÑöêÜÄçÖ çÄ ÑàïÄíÖãçàíÖ îìçäñàà óÂÒÚËÚÂ ‰Ëı‡ÚÂÎÌË ËÌÙÂÍˆËË Ë ÔÓ„ÂÒË‡˘‡Ú‡ ‰Ëı‡ÚÂÎÌ‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ ‚ ÔÓ-Ì‡ÔÂ‰Ì‡ÎËÚÂ ÒÚ‡‰ËË Ì‡ èåÑ ÚËÔ Ñ˛¯ÂÌ Ì‡Î‡„‡Ú ÔÂËÓ‰Ë˜ÌÓ ÏÓÌËÚÓË‡ÌÂ Ì‡ ‰Ëı‡ÚÂÎÌËÚÂ ÙÛÌÍˆËË ˜ÂÁ ËÁÏÂ‚‡ÌÂ Ì‡ îÇä Ë Í˙‚ÌËÚÂ „‡ÁÓ‚Â, Í‡ÍÚÓ Ë Ò˙ÓÚ‚ÂÚÌ‡ ÚÂ‡ÔËﬂ. é·ÂÁÔÓÍÓﬂ‚‡˘Ë ÒËÏÔÚÓÏË, Ì‡Î‡„‡˘Ë ÚÂ‡ÔËﬂ Ò‡: ·ÂÎÂÁË Ì‡ ÌÓ˘Ì‡ ıËÔÓ‚ÂÌÚËÎ‡ˆËﬂ (Ì‡Û¯ÂÌËﬂ ‚ Ò˙Ìﬂ, ‰ÌÂ‚Ì‡ ÛÏÓ‡, Ì‡Û¯ÂÌ‡ ÍÓÌˆÂÌÚ‡ˆËﬂ), Ì‡Ï‡ÎÂÌ ‡ÔÂÚËÚ, Ò‡ÚÛ‡ˆËﬂ Ì‡ O2 <92%, îÇä<60% ÓÚ Ó˜‡Í‚‡ÌËﬂ, ˜ÂÒÚË ‰Ëı‡ÚÂÎÌË ËÌÙÂÍˆËË. Ç ÔÓ-‡ÌÌËÚÂ ÒÚ‡‰ËË ‰Ó·˙ ËÏ‡Ú ‰Ëı‡ÚÂÎÌ‡Ú‡ „ËÏÌ‡ÒÚËÍ‡ Ë ÔÓÒÚÛ‡ÎÌËﬂÚ ‰ÂÌ‡Ê. óÂÒÚËÚÂ ‰Ëı‡ÚÂÎÌË ËÌÙÂÍˆËË ËÁËÒÍ‚‡Ú ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÔÓÚË‚Ó„ËÔÌË Ë ÔÌÂ‚ÏÓÍÓÍÓ‚Ë ‚‡ÍÒËÌË Í‡ÚÓ ÔÓÙËÎ‡ÍÚËÍ‡, Í‡ÍÚÓ Ë ‡‰ÂÍ‚‡ÚÌ‡ ‡ÌÚË·ËÓÚË˜Ì‡ ÚÂ‡ÔËﬂ Á‡ ÎÂ˜ÂÌËÂ (4). àÁÍÛÒÚ‚ÂÌ‡Ú‡ ‚ÂÌÚËÎ‡ˆËﬂ Ò ÔÓÁËÚË‚ÌÓ Ì‡Îﬂ„‡ÌÂ Ì‡È-˜ÂÒÚÓ Á‡ÔÓ˜‚‡ ‰‡ ÒÂ ÔËÎ‡„‡ ÓÍÓÎÓ 17-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. éÒÌÓ‚Ì‡ ËÌ‰ËÍ‡ˆËﬂ Á‡ ÔËÎÓÊÂÌËÂÚÓ Ì‡ ÌÂËÌ‚‡ÁË‚Ì‡ ÌÓ˘Ì‡ ‚ÂÌÚËÎ‡ˆËﬂ Â ÛÒÚ‡ÌÓ‚ﬂ‚‡ÌÂÚÓ Ì‡ ÍÎËÌË˜ÌÓ ËÁﬂ‚ÂÌ‡ ÌÓ˘Ì‡ ıËÔÓ‚ÂÌÚËÎ‡- 164 ˆËﬂ. ÖÏÔËË˜ÌÓ Â ÛÒÚ‡ÌÓ‚ÂÌÓ, ˜Â ÌÓ˘Ì‡Ú‡ ‚ÂÌÚËÎ‡ˆËﬂ (ÌÂËÌ‚‡ÁË‚Ì‡ ËÎË ËÌ‚‡ÁË‚Ì‡ ˜ÂÁ Ú‡ıÂÓÒÚÓÏËﬂ) ‚Ó‰Ë ‰Ó ‰˙Î„ÓÒÓ˜Ì‡ ÒÚ‡·ËÎËÁ‡ˆËﬂ Ì‡ ‰Ëı‡ÚÂÎÌËÚÂ ÙÛÌÍˆËË, Â‰ÛÍˆËﬂ Ì‡ ‰Ëı‡ÚÂÎÌËÚÂ ËÌÙÂÍˆËË Ë Û‚ÂÎË˜ÂÌËÂ Ì‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ ÊË‚ÓÚ‡ (18). ë Ì‡ÔÂ‰‚‡ÌÂ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÔÂËÓ‰ËÚÂ Ì‡ ËÁÍÛÒÚ‚ÂÌ‡ ‚ÂÌÚËÎ‡ˆËﬂ ‚ ‡ÏÍËÚÂ Ì‡ ‰ÂÌÓÌÓ˘ËÂÚÓ ÒÂ Û‰˙ÎÊ‡‚‡Ú. II. ÉÖççÄ íÖêÄèàü 1. íÖêÄèàü, èéÇãàüÇÄôÄ ÉÖççÄíÄ ÖäëèêÖëàü çÄ Ñàëíêéîàç èÂÁ ÔÓÒÎÂ‰ÌËÚÂ „Ó‰ËÌË ÒÂ ‡Á‚Ë‚‡Ú ‡ÁÎË˜ÌË ÔÓ‰ıÓ‰Ë Á‡ ÍÓÂÍˆËﬂ Ì‡ „ÂÌÂÚË˜ÌËﬂ ‰ÂÙÂÍÚ Ë ‚˙ÁÒÚ‡ÌÓ‚ﬂ‚‡ÌÂ Ì‡ ‰ËÒÚÓÙËÌÓ‚‡Ú‡ ÂÍÒÔÂÒËﬂ, ÍÓËÚÓ ·Ëı‡ Á‡·‡‚ËÎË ÔÓ„ÂÒËﬂÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ·Ëı‡ ÔÓ‰Ó·ËÎË Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ ·ÓÎÌËÚÂ (29, 30, 21). íÂ Ò‡ ‚ÒÂ Ó˘Â ÂÍÒÔÂËÏÂÌÚ‡ÎÌË. íÂÁË ÔÓ‰ıÓ‰Ë ÒÂ Í‡ÚÂ„ÓËÁË‡Ú ‚ ÚË ÓÒÌÓ‚ÌË ÍÎ‡Ò‡: „ÂÌÂÚË˜ÌË, ÍÎÂÚ˙˜ÌÓ-·‡ÁË‡ÌË Ë Ù‡Ï‡ÍÓÎÓ„Ë˜ÌË (í‡·ÎËˆ‡ 1). í‡·ÎËˆ‡ 1. éÒÌÓ‚ÌË ÚÂ‡ÔÂ‚ÚË˜ÌË ÒÚ‡ÚÂ„ËË Á‡ „ÂÌÌ‡Ú‡ ÚÂ‡ÔËﬂ (32) ÉÂÌ-Á‡ÏÂÒ- ëÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË åÛÒÍÛÎÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË, ÚËÚÂÎÌ‡ Ì‡Â˜ÂÌË Ó˘Â Ò‡ÚÂÎËÚÌË ÚÂ‡ÔËﬂ åÛÒÍÛÎÌË Ë ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË äÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚÓÏ‡ÎÌË ÍÎÂÚÍË ÇÌ‡ÒﬂÌÂ ‚ Ó„‡ÌËÁÏ‡ Ì‡ Ä‰ÂÌÓ‡ÒÓˆËË‡ÌË ‚ËÛÒË ‰ËÒÚÓÙËÌÓ‚Ë ÏËÌË„ÂÌË ˜ÂÁ: ÇËÛÒË ÓÚ Ó‰ Lentivirus Ñçä ÔÎ‡ÁÏË‰Ë Ñçä/êçä ÄÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚË‰Ë, Ï‡ÌËÔÛ- ÍÓËÚÓ ‚Ó‰ﬂÚ ‰Ó ÔÂÒÍ‡˜‡ÌÂ ÎË‡ÌÂ Ì‡ ÂÍÁÓÌ Ë ÒËÌÚÂÁ Ì‡ Í˙ÒË ‰ËÒÚÓÙËÌÓ‚Ë ÙÓÏË. Ñçä ıËÏÂ‡ÔÎ‡ÒÚË Á‡ ÍÓÂÍˆËﬂ Ì‡ ÚÓ˜ÍÓ‚Ë ÏÛÚ‡ˆËË Ë Ï‡ÎÍË ‰ÂÎÂˆËË ÇËÛÒÌÓ-Ì‡ÒÓ˜ÂÌÓ ÔÂÒÍ‡˜‡ÌÂ Ì‡ ÂÍÁÓÌ î‡Ï‡ÍÓ- åÂ‰ËÍ‡ÏÂÌÚË, ÒÚËÏÛÎË‡˘Ë Heregulin Ë ‰Û„Ë Ò˙Â‰ËÌÂÌËﬂ ‚ ÎÓ„Ë˜ÌË „ÂÌÌ‡Ú‡ ÂÍÒÔÂÒËﬂ ÂÚ‡Ô Ì‡ ÔÓÛ˜‚‡ÌÂ ‡„ÂÌÚË Ì‡ ÛÓÚÓÙËÌ åÂ‰ËÍ‡ÏÂÌÚË, ÓÒË„Ûﬂ‚‡˘Ë ÄÏËÌÓ„ÎËÍÓÁË‰Ë Ú‡ÌÒÍ‡ÔˆËﬂ Ë ÒÎÂ‰ ÔÂÊ‰Â‚- êíë124 ÂÏÂÌÌÓ ÔÓﬂ‚ËÎ ÒÂ ÒÚÓÔ ÍÓ‰ÓÌ ÑÛ„Ë Ò˙Â‰ËÌÂÌËﬂ ‚ ÂÚ‡Ô Ì‡ ÔÓÛ˜‚‡ÌÂ åÂ‰ËÍ‡ÏÂÌÚË Ë Ù‡ÍÚÓË, ÍÓËÚÓ ÓÒË„Ûﬂ‚‡Ú ÏÛÒÍÛÎÌÓÚÓ ‚˙ÁÒÚ‡ÌÓ‚ﬂ‚‡ÌÂ ËÎË Ì‡Ï‡Îﬂ‚‡Ú ÏÛÒÍÛÎÌ‡Ú‡ Û‚Â‰‡ ÇÌ‡ÒﬂÌÂ ˜ÂÁ ‚ËÛÒÌË ‡„ÂÌÚË IGF 1 Ì‡ ‡ÒÚÂÊÌË Ù‡ÍÚÓË åËÓÒÚ‡ÚËÌ ËÌıË·ËˆËﬂ èÓÚÂ‡ÁÌË ËÌıË·ËÚÓË, Leupeptin-ËÌıË·ËÚÓ Ì‡ Á‡·‡‚ﬂ˘Ë ÏÛÒÍÛÎÌ‡Ú‡ Û‚Â‰‡ Í‡ÎÔ‡ËÌ‡ BBIC-ÒÂËÌ ÔÓÚÂ‡ÁÂÌ ËÌıË·ËÚÓ MG-132-ÔÓÚÂÓÁÓÏÂÌ ËÌıË·ËÚÓ êÂ‡„ÂÌÚË, ÔÓ‰ÚËÒÍ‡˘Ë Cyclosporine A ‚˙ÁÔ‡ÎÂÌËÂÚÓ Enbrel Remicade NFkB nNOS Â„ÛÎ‡ˆËﬂ Ì‡„ÓÂ Pentoxifillin ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÉÖçÖç íêÄçëîÖê îÛÌÍˆËÓÌ‡ÎÂÌ ‰ËÒÚÓÙËÌÓ‚ „ÂÌ ÏÓÊÂ ‰‡ ·˙‰Â ‚˙‚Â‰ÂÌ ‚ ÏÛÒÍÛÎÌËÚÂ ÍÎÂÚÍË Ì‡ Ô‡ˆËÂÌÚË Ò ‰ËÒÚÓÙËÌÓÔ‡ÚËﬂ, Á‡ ‰‡ Á‡ÏÂÒÚË ‰ÂÙÂÍÚÌÓÚÓ ÍÓÔËÂ. êÂÍÓÏ·ËÌ‡ÌÚÌËÚÂ ‡‰ÂÌÓ‡ÒÓˆËË‡ÌË ‚ËÛÒË ËÏ‡Ú ÌﬂÍÓË ÔÂ‰ËÏÒÚ‚‡ ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌ‚ÂÌˆËÓÌ‡ÎÌËÚÂ ‡‰ÂÌÓ‚ËÛÒÌË Ë ÂÚÓ‚ËÛÒÌË ‚ÂÍÚÓË Ë ÒÂ ËÁÔÓÎÁ‚‡Ú ‚ÒÂ ÔÓ-˜ÂÒÚÓ Á‡ Ú‡ÌÒÙÂ Ì‡ ÏËÌË Ë ÏËÍÓ‰ËÒÚÓÙËÌÓ‚Ë Ë ÛÓÚÓÙËÌÓ‚Ë „ÂÌË ‚ Ì‡ÔÂ˜ÌÓÌ‡·‡Á‰ÂÌË Ë Ò˙‰Â˜ÌË ÏËÓˆËÚË Ì‡ ÏË¯ÍË- ÏÓ‰ÂÎË. çÂÒÔÂˆËÙË˜ÌË CMV Ë ÒÔÂˆËÙË˜ÌË ÏÛÒÍÛÎÌË ÔÓÏÓÚÂË ÒÂ ËÁÔÓÎÁ‚‡Ú Á‡ ÍÓÌÚÓÎ Ì‡ ÂÍÒÔÂÒËﬂÚ‡ Ì‡ ÚÂÁË „ÂÌË. ÑÛ„ ÏÂÚÓ‰ Â ËÌÊÂÍÚË‡ÌÂÚÓ Ì‡ „ÓÎË Ñçä ÔÎ‡ÁÏË‰Ë, ÌÓÒÂ˘Ë „ÂÌ‡, ‚ ÏÛÒÍÛÎÌËÚÂ ÍÎÂÚÍË (23). á‡ ˆÂÎÚ‡ ÒÂ ËÁÔÓÎÁ‚‡Ú ÂÎÂÍÚÓÚ‡ÌÒÙÂ Ë ıË‡ÎÛÓÌË‰‡Á‡. ÉÖççÄ åéÑàîàäÄñàü ñÂÎÚ‡ Ì‡ „ÂÌÌ‡Ú‡ ÏÓ‰ËÙËÍ‡ˆËﬂ Â ‰‡ Á‡ÏÂÌË ËÎË ‚˙ÁÒÚ‡ÌÓ‚Ë „ÂÌÌËÚÂ ÏÛÚ‡ˆËË. Ö‰ËÌ ÓÚ Ì‡È-Ó·Â˘‡‚‡˘ËÚÂ ÏÂÚÓ‰Ë Â ÔÂÒÍ‡˜‡ÌÂÚÓ Ì‡ ÂÍÁÓÌ, ˆÂÎÚ‡, Ì‡ ÍÓÈÚÓ Â ‰‡ ÏÓ‰ËÙËˆË‡ Â‰Ì‡ ÔÓ-ÚÂÊÍ‡ ÏÛÚ‡ˆËﬂ, ‚Ó‰Â˘‡ ‰Ó ÍÎËÌËÍ‡ Ì‡ Ñ˛¯ÂÌ ‚ ÔÓ-ÎÂÍ‡ Ò ÙÂÌÓÚËÔ Ì‡ èåÑ ÚËÔ ÅÂÍÂ (3). ÖÍËÔË ÓÚ ÏÌÓ„Ó ÒÚ‡ÌË ËÁ‚˙¯‚‡Ú ÍÎËÌË˜ÌË ÔÓÛ˜‚‡ÌËﬂ ‚ Ú‡ÁË Ì‡ÒÓÍ‡, Í‡ÚÓ ÒÂ ËÁÔÓÎÁ‚‡Ú ÍÓÏ·ËÌ‡ˆËË ÓÚ ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚË‰Ë. àÁÒÎÂ‰‚‡ÌËﬂ ÔÓÍ‡Á‚‡Ú, ˜Â ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚË‰Ë, ÍÓËÚÓ ÓÒË„Ûﬂ‚‡Ú ÔÂÒÍ‡˜‡ÌÂ Ì‡ Â„ËÓÌ ÏÂÊ‰Û 45 Ë 51 ÂÍÁÓÌË (ÏÓÎÂÍÛÎÌË Í˙ÔÍË), ÏÓÊÂ ‰‡ Â ÔÓ‰ıÓ‰ﬂ˘Ó ÎÂ˜ÂÌËÂ Á‡ „ÓÎﬂÏ‡ ˜‡ÒÚ (ÓÍÓÎÓ 80%) ÓÚ Ô‡ˆËÂÌÚËÚÂ (7,19). ÑÛ„ ÚËÔ „ÂÌÌ‡ ÚÂ‡ÔËﬂ Ò‡ ıËÏÂ‡ÔÎ‡ÒÚËÚÂ, ÔË ÍÓËÚÓ ÒÂ ËÁÔÓÎÁ‚‡Ú Ñçä ÔÓÒÎÂ‰Ó‚‡ÚÂÎÌÓÒÚË, Ò‚˙Á‡ÌË Ò êçä, ÍÓËÚÓ ÒÂ Ò‚˙Á‚‡Ú Ò˙Ò ÒÓ·ÒÚ‚ÂÌ‡Ú‡ Ñçä Ì‡ Ô‡ˆËÂÌÚ‡ Ë ‡ÍÚË‚Ë‡Ú ÏÂı‡ÌËÁÏËÚÂ Ì‡ ‚˙ÁÒÚ‡ÌÓ‚ﬂ‚‡ÌÂ. êÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÚÓÁË ÏÂÚÓ‰ ÌÂ Ò‡ ÏÌÓ„Ó ‰Ó·Ë (22). ÄÌÚË·ËÓÚËˆË Í‡ÚÓ ‡ÏËÌÓ„ÎËÍÓÁË‰ËÚÂ ÏÓ„‡Ú ‰‡ ÔÓ‚ÎËﬂﬂÚ ÔÓˆÂÒËÚÂ Ì‡ Ú‡ÌÒÎ‡ˆËﬂ, Í‡ÚÓ ÒÂ Ë„ÌÓË‡ ÔÂÊ‰Â‚ÂÏÂÌÌÓ ‚˙ÁÌËÍÌ‡ÎËﬂ ÒÚÓÔ ÍÓ‰ÓÌ Ë ÒÂ ÒËÌÚÂÁË‡ ÌÓÏ‡ÎÂÌ ‰ËÒÚÓÙËÌ (6, 26). è‡‚ﬂÚ ÒÂ ÓÔËÚË Ò Gentamicin, Negamicin Ë PTC124 ÔË ÏË¯ÍË- ÏÓ‰ÂÎË Ë ÔË ıÓ‡ Ò Ó·Â˘‡‚‡˘Ë Ì‡ ÚÓÁË ÂÚ‡Ô ÂÁÛÎÚ‡ÚË (5). äãÖíöóçÄ íÖêÄèàü çÓÏ‡ÎÌ‡Ú‡ ÏÛÒÍÛÎÌ‡ ÙÛÌÍˆËﬂ Á‡‚ËÒË ÓÚ ËÁ‰˙ÊÎË‚ÓÒÚÚ‡ Ë Â„ÂÌÂ‡ˆËﬂÚ‡ Ì‡ ÏËÓÙË·ËÚÂ. íÓ‚‡ Â Â‰ËÌ ÒÎÓÊÌÓ Â„ÛÎË‡Ì ÔÓˆÂÒ, Á‡ÔÓ˜‚‡˘ Ò ‡ÍÚË‚‡ˆËﬂÚ‡ Ì‡ ÔÂÍÛÒÓÌË ÍÎÂÚÍË, ÍÓËÚÓ ‚ÔÓÒÎÂ‰ÒÚ‚ËÂ ÙÓÏË‡Ú ÔÓ„ÂÌËÚÓÌË ÍÎÂÚÍË, ÍÓËÚÓ ÒÎÂ‰ ÒÎË‚‡ÌÂÚÓ ÒË, Ó·‡ÁÛ‚‡Ú ‰ËÙÂÂÌˆË‡ÌË ÏËÓÙË·Ë. í‡ÌÒÔÎ‡ÌÚ‡ˆËﬂÚ‡ Ì‡ ‡ÁÎË˜ÌË ‚Ë‰Ó‚Â ÏÛÒÍÛÎÌË ÔÂÍÛÒÓÌË ÍÎÂÚÍË ËÏ‡ Á‡ ˆÂÎ ‰‡ Û‚ÂÎË˜Ë ÒËÎ‡Ú‡ Ì‡ ‰ËÒÚÓÙË˜ÌËÚÂ ÏÛÒÍÛÎË. èÓÒÚË„Ì‡ÚËÚÂ ÂÁÛÎÚ‡ÚË Ò‡ ‡ÁÓ˜‡Ó‚‡˘Ë, ÔÓ‡‰Ë ‡Á‚Ë‚‡˘‡Ú‡ ÒÂ ‚˙ÁÔ‡ÎËÚÂÎÌ‡ Â‡ÍˆËﬂ Ë ÒÎ‡·‡Ú‡ ÏË„‡ˆËÓÌÌ‡ ÒÔÓÒÓ·ÌÓÒÚ Ì‡ ÏËÓ·Î‡ÒÚËÚÂ (20,28). ë‡ÚÂÎËÚÌËÚÂ ÍÎÂÚÍË, ‡ÁÔÓÎÓÊÂÌË ÏÂÊ‰Û ÔÎ‡ÁÏÂÌ‡Ú‡ ÏÂÏ·‡Ì‡ Ì‡ ÏÛÒÍÛÎÌËÚÂ ‚Î‡ÍÌ‡ Ë ·‡Á‡ÎÌ‡Ú‡ ÏÂÏ·‡Ì‡, ÒÂ Ò˜ËÚ‡Ú Á‡ ÏÛÒÍÛÎÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÍÚË. íÂ ÂÍÒÔÂÒË‡Ú CD34, ‡ ÒÎÂ‰ ‡ÍÚË‚‡ˆËﬂ Ë MyoD, Myf-5, M-Cadherin. ÇÒﬂÍÓ ÔÂ˜ËÒÚÂÌÓ Â‰ËÌË˜ÌÓ ËÌÚ‡ÍÚÌÓ ÏÛÒÍÛÎÌÓ ‚Î‡ÍÌÓ Â ‡ÒÓˆËË‡ÌÓ Ò ÓÍÓÎÓ 7-22 Ò‡ÚÂÎËÚÌË ÍÎÂÚÍË Ë Ú‡ÌÒÔÎ‡ÌÚË‡ÌË ‚ ËÏÛÌÓÒÛÔÂÒË‡ÌË ÏË¯ÍË-ÏÓ‰ÂÎË, ‰‡‚‡Ú Ì‡˜‡ÎÓÚÓ Ì‡ ÓÍÓÎÓ 100 ÌÓ‚Ë ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡. ë‡ÚÂÎËÚÌËÚÂ ÍÎÂÚÍË Ò‡ ‚ Ò˙ÒÚÓﬂÌËÂ ‰‡ ÓÒË„ÛﬂÚ Â„ÂÌÂ‡ˆËﬂ Ì‡ Û‚Â‰ÂÌË ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡ in vivo. Ç Ò‡‚ÌÂÌËÂ Ò ÚÂÁË ÍÎÂÚÍË, ‰Û„ËÚÂ ËÁÔÓÎÁ‚‡ÌË ÏÛÒÍÛÎÌË ÒÚ‚ÓÎÓ‚Ë Ë ÔÓ„ÂÌËÚÓÌË ÍÎÂÚÍË ËÏ‡Ú Ï‡Î˙Í ÏËÓ„ÂÌÂÌ ÔÓÚÂÌˆË‡Î (31). è‡‚ﬂÚ ÒÂ ÓÔËÚË Ò ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ıÂÏÓÔÓÂÚË˜ÌË Ë ÒÚ¸ÓÏ‡ÎÌË ÍÎÂÚÍË, ËÏ‡˘Ë ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ 165 ÔÂÏËÌ‡‚‡Ú ÓÚ Í˙‚ÓÌÓÒÌËÚÂ Ò˙‰Ó‚Â Í˙Ï ÏÛÒÍÛÎÌËÚÂ ÍÎÂÚÍË, Ò ‚ÒÂ Ó˘Â ÌÂÒË„ÛÂÌ ÂÁÛÎÚ‡Ú. éÒÌÓ‚ÂÌ ÔÓ·ÎÂÏ, Ò ÍÓÈÚÓ ÒÂ Ò·Î˙ÒÍ‚‡ ÍÎÂÚ˙˜Ì‡Ú‡ ÚÂ‡ÔËﬂ Â Â‡ÍˆËﬂÚ‡ Ì‡ ÓÚı‚˙ÎﬂÌÂ. àÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÓ·ÒÚ‚ÂÌË ÍÎÂÚÍË Ì‡ Ô‡ˆËÂÌÚ‡ Â Â‰ÌÓ ÓÚ ‚˙ÁÏÓÊÌËÚÂ Â¯ÂÌËﬂ. ìêéíêéîàç ìÓÚÓÙËÌ˙Ú Â ÔÓÚÂËÌ Ò ·ÎËÁÍ‡ ‰Ó ‰ËÒÚÓÙËÌ‡ ÒÚÛÍÚÛ‡ Ë ÒÂ ÓÚÍË‚‡ ‚˙‚ ‚ËÒÓÍË ÍÓÌˆÂÌÚ‡ˆËË ‚ ÏÛÒÍÛÎÌËÚÂ ÍÎÂÚÍË ÔÓ ‚ÂÏÂ Ì‡ ÂÏ·ËÓÌ‡ÎÌÓÚÓ ‡Á‚ËÚËÂ. ëÎÂ‰ ‡Ê‰‡ÌÂÚÓ ÒÂ ÂÍÒÔÂÒË‡ ÓÒÌÓ‚ÌÓ ‚ ÌÂ‚ÓÏÛÒÍÛÎÌËÚÂ Ò˙˜ÎÂÌÂÌËﬂ. èË Ô‡ˆËÂÌÚË Ò èåÑ ÚËÔ Ñ˛¯ÂÌ ÂÍÒÔÂÒËﬂÚ‡ Ì‡ ÛÓÚÓÙËÌ‡ Â ÔÓ‚Ë¯ÂÌ‡ Í‡ÚÓ ÍÓÏÔÂÌÒ‡ÚÓÌ‡ Â‡ÍˆËﬂ ÒÔﬂÏÓ Ì‡Ï‡ÎÂÌËﬂ ‰ËÒÚÓÙËÌ. èÓ‰ıÓ‰Ë ‚ ÚÂ‡ÔËﬂÚ‡ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Ò‡ ‚˙‚ÂÊ‰‡ÌÂ Ì‡ ÔÓÏÓÚÓ ËÎË ‚Â˘ÂÒÚ‚Ó, ÍÓËÚÓ ÔÓ‚Ë¯‡‚‡Ú ÂÍÒÔÂÒËﬂÚ‡ Ì‡ ÛÓÚÓÙËÌÓ‚Ëﬂ „ÂÌ (10, 27). îÄêåÄäéãéÉàóçà èéÑïéÑà àÁÔÓÎÁ‚‡Ú ÒÂ ÏÂ‰ËÍ‡ÏÂÌÚË, ÛÚ‚˙‰ÂÌË Á‡ ÎÂ˜ÂÌËÂÚÓ Ì‡ ‰Û„Ë Á‡·ÓÎﬂ‚‡ÌËﬂ (‚Ê. ÍÓÚËÍÓÒÚÂÓË‰Ì‡ ÚÂ‡ÔËﬂ). ñÂÎÚ‡ ËÏ Â ÔÓ‰‰˙Ê‡Ú Í‡ÎˆËÂ‚‡Ú‡ ıÓÏÂÓÒÚ‡Á‡, ‰‡ ÔÓ‰Ó·ﬂÚ ÏÛÒÍÛÎÌ‡Ú‡ ÒËÎ‡, ‰‡ ÒÛÔÂÒË‡Ú ÒÚÓÔ ÍÓ‰ÓÌËÚÂ, ‰‡ ÒÚËÏÛÎË‡Ú ÛÓÚÓÙËÌÓ‚‡Ú‡ ÂÍÒÔÂÒËﬂ. áÄäãûóÖçàÖ ãÂ˜ÂÌËÂÚÓ Ì‡ ‰ËÒÚÓÙËÌÓÔ‡ÚËËÚÂ Â Ò ÍÓÚËÍÓÒÚÂÓË‰Ë (Prednisolone Ë Deflazacort) Â ÁÎ‡ÚÂÌ ÒÚ‡Ì‰‡Ú, Ú˙È Í‡ÚÓ ÚÂ ÔÓ ÌÂ Ì‡Ô˙ÎÌÓ ËÁﬂÒÌÂÌË ÍÎÂÚ˙˜ÌË ÏÂı‡ÌËÁÏË ‚Ó‰ﬂÚ ‰Ó Á‡·‡‚ﬂÌÂ Ì‡ ÔÓ„ÂÒËﬂÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. íÓÁË ÚËÔ ÎÂ˜ÂÌËÂ ÌÂ Â ÔËÎ‡„‡ÌÓ Ï‡ÒÓ‚Ó ‚ Å˙Î„‡Ëﬂ, ‡ Â ÌÂÓ·ıÓ‰ËÏÓ ‰‡ ÒÂ ËÁÔÓÎÁ‚‡ ÔÓ‡‰Ë ‰ÓÒÚ˙ÔÌÓÒÚÚ‡ Ë ÔÂÒÔÂÍÚË‚‡Ú‡ Á‡ Û‰˙ÎÊ‡‚‡ÌÂ ÔÂËÓ‰‡ Ì‡ ‰ÓÒÚË„‡ÌÂ ‰Ó ÚÂÊÍ‡ ËÌ‚‡ÎË‰ËÁ‡ˆËﬂ. ãÂ˜ÂÌËÂÚÓ Ò ‡‚‡Ì„‡‰ÌË ÚÂıÌÓÎÓ„ËË, ‰ÂÈÒÚ‚‡˘Ë Ì‡ Ô‡ÚÓ„ÂÁ‡Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ò ÔÓ‚ÎËﬂ‚‡ÌÂ „ÂÌÌ‡Ú‡ ÂÍÒÔÂÒËﬂ Ì‡ ‰ËÒÚÓÙËÌ ‚ÍÎ˛˜‚‡: „ÂÌ-Á‡ÏÂÒÚËÚÂÎÌ‡ ÚÂ‡ÔËﬂ Ò˙Ò ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË (ÏÛÒÍÛÎÌË Ë ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚ‚ÓÎÓ‚Ë ÍÎÂÚÍË, ÍÓÒÚÌÓÏÓÁ˙˜ÌË ÒÚÓÏ‡ÎÌË ÍÎÂÚÍË); Ñçä/êçä Ï‡ÌËÔÛÎË‡ÌÂ Ë Ù‡Ï‡ÍÓÎÓ„Ë˜ÌË ‡„ÂÌÚË (ÏÂ‰ËÍ‡ÏÂÌÚË, ÒÚËÏÛÎË‡˘Ë „ÂÌÌ‡Ú‡ ÂÍÒÔÂÒËﬂ Ì‡ ÛÓÚÓÙËÌ; ÏÂ‰ËÍ‡ÏÂÌÚË, ÓÒË„Ûﬂ‚‡˘Ë Ú‡ÌÒÍ‡ÔˆËﬂ Ë ÒÎÂ‰ ÔÂÊ‰Â‚ÂÏÂÌÌÓ ÔÓﬂ‚ËÎ ÒÂ ÒÚÓÔ ÍÓ‰ÓÌ; ‚Ì‡ÒﬂÌÂ ˜ÂÁ ‚ËÛÒÌË ‡„ÂÌÚË Ì‡ ‡ÒÚÂÊÌË Ù‡ÍÚÓË; ÔÓÚÂ‡ÁÌË ËÌıË·ËÚÓË, Á‡·‡‚ﬂ˘Ë ÏÛÒÍÛÎÌ‡Ú‡ Û‚Â‰‡; Â‡„ÂÌÚË, ÔÓ‰ÚËÒÍ‡˘Ë ‚˙ÁÔ‡ÎÂÌËÂÚÓ). ç‡È-Ó·Â˘‡‚‡˘Ó Á‡ ÒÂ„‡ Â Ñçä/êçä Ï‡ÌËÔÛÎË‡ÌÂÌÓ Ò ‡ÌÚËÒÂÌÒ ÓÎË„ÓÌÛÍÎÂÓÚË‰Ë, ÔË ÍÓÂÚÓ ÒÂ ÒËÌÚÂÁË‡Ú ÒÂ Í˙ÒË ‰ËÒÚÓÙËÌÓ‚Ë ÙÓÏË Ë ÒÂ ÔÂÏËÌ‡‚‡ ÓÚ ÚÂÊÍ‡ ÏÛÚ‡ˆËﬂ Ò ÍÎËÌËÍ‡ Ì‡ Ñ˛¯ÂÌ ‚ ÔÓ-ÎÂÍ‡ Ò ÙÂÌÓÚËÔ Ì‡ èåÑ ÚËÔ ÅÂÍÂ. èË ÔÓÁËÚË‚ÌË ÂÁÛÎÚ‡ÚË ÓÚ ÂÍÒÔÂËÏÂÌÚ‡ÎÌËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ Ë ÍÎËÌË˜ÌË ÔÓÛ˜‚‡ÌËﬂ, ÌﬂÍÓË ÓÚ Úﬂı ·Ëı‡ ÏÓ„ÎË ‰‡ ÒÂ ËÁÔÓÎÁ‚‡Ú Ë ÔË ·˙Î„‡ÒÍË Ô‡ˆËÂÌÚË. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7. ç‡ˆËÓÌ‡ÎÂÌ ÍÓÌÒÂÌÒÛÒ Á‡ ‰Ë‡„ÌÓÒÚËÍ‡, ÎÂ˜ÂÌËÂ Ë ÔÓÙËÎ‡ÍÚËÍ‡ Ì‡ ÌÂ‚Ó-ÏÛÒÍÛÎÌËÚÂ Á‡·ÓÎﬂ‚‡ÌËﬂ. 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E-mail: [email protected] Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 167 é·ÁÓ ÄÇíéáéåçé-ÑéåàçÄçíçà îÄåàãçà èÄêñàÄãçà ÖèàãÖèíàóçà ëàçÑêéåà-äãàçàóçÄ, ÖãÖäíêéîàáàéãéÉàóçÄ à ÉÖçÖíàóçÄ ïÄêÄäíÖêàëíàäÄ ë.ÜÂÎﬂÁÍÓ‚‡1. à.í˙ÌÂ‚1,2 1ìåÅÄã 2çÓ‚ ◊ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, åî –ëÓÙËﬂ ·˙Î„‡ÒÍË ÛÌË‚ÂÒËÚÂÚ, ä‡ÚÂ‰‡ ÔÓ äÓ„ÌËÚË‚Ì‡ Ì‡ÛÍ‡ Ë ÔÒËıÓÎÓ„Ëﬂ SUMMARY AUTOSOMAL-DOMINANT FAMILIAL PARTIAL EPILEPSIES-CLINICAL, ELECTROPHYSIOLOGICAL AND GENETIC CHARACTERISTICS. S.Zhelyazkova1. I.Tournev1,2 1 University Hospital “Alexandrovska”, Neurological Clinic, Medical faculty-Sofia 2New Bulgarian University, Department of Cognitive Science and Psychology Genetic factors are thought to play a role in the pathogenesis of approximately 40% of the epilepsies. While the significance of these factors for the generalized epilepsies is wellknown, the concept of genetic focal epilepsies is relatively new. In the last decade, the progress, achieved in the field of molecular genetics and phenotype defining contributed to the identification of several new familial partial epilepsies with autosomal-dominant inheritance. They are characterized by incomplete penetrance, variable expressivity in terms of age of onset, type and frequency of seizures, and duration of the disorder. In some of these syndromes, the responsible genes have been identified while in others, new genes are expected to be discovered. The fact that the clinical presentation in all familial partial epilepsy syndromes is similar to that in sporadic patients, necessitates the taking of detailed family history by the physicians for determining the exact diagnosis. The aim of this review is to present the main clinical, electrophysiological and genetic features of some of the newly discovered in the last decade familial partial epilepsy syndromes with autosomal-dominant inheritance. KEY WORDS: genetic, autosomal-dominant inheritance, familial focal epilepsies. êÖáûåÖ ÉÂÌÂÚË˜ÌËÚÂ Ù‡ÍÚÓË Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÓÍÓÎÓ 40% ÓÚ ÂÔËÎÂÔÒËËÚÂ. ÑÓÍ‡ÚÓ ÚﬂıÌÓÚÓ ÁÌ‡˜ÂÌËÂ ÔË „ÂÌÂ‡ÎËÁË‡ÌËÚÂ ÂÔËÎÂÔÒËË Â ÓÚ‰‡‚Ì‡ ËÁ‚ÂÒÚÌÓ, ÚÓ Ë‰ÂﬂÚ‡ Á‡ Ò˙˘ÂÒÚ‚Û‚‡ÌÂ Ì‡ „ÂÌÂÚË˜ÌË ÙÓÏË Ì‡ ÙÓÍ‡ÎÌË ÂÔËÎÂÔÒËË Â Ò‡‚ÌËÚÂÎÌÓ ÌÓ‚‡. Ç ÔÓÒÎÂ‰ÌËÚÂ 10 „Ó‰ËÌË ·Î‡„Ó‰‡ÂÌËÂ Ì‡ ÔÓÒÚËÊÂÌËﬂÚ‡ ‚ Ó·Î‡ÒÚÚ‡ Ì‡ ÏÓÎÂÍÛÎﬂÌ‡Ú‡ „ÂÌÂÚËÍ‡ Ë ÔÓ-ÔÂˆËÁÌÓÚÓ ÙÂÌÓÚËÔÌÓ ‰ÂÙËÌË‡ÌÂ, ·ﬂı‡ Ë‰ÂÌÚËÙËˆË‡ÌË ÌﬂÍÓÎÍÓ ÌÓ‚Ë Ô‡ˆË‡ÎÌË, Ù‡ÏËÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏ‡ Ò ‡‚ÚÓÁÓÏÌÓ - ‰ÓÏËÌ‡ÌÚÓ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ. íÂ ÒÂ ı‡‡ÍÚÂËÁË‡Ú Ò ÌÂÔ˙ÎÌ‡ ÔÂÌÂÚ‡ÌÌÓÒÚ, ‚‡Ë‡·ËÎÌ‡ ÂÍÒÔÂÒË‚ÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ‚˙Á‡ÒÚ Ì‡ Ì‡˜‡ÎÓ, ÚËÔ Ë ˜ÂÒÚÓÚ‡ Ì‡ ÂÔËÎÂÔÚË˜ÌËÚÂ ÔËÒÚ˙ÔË, Ë ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. èË ÌﬂÍÓË ÓÚ Úﬂı ‚Â˜Â Ò‡ Ë‰ÂÌÚËÙËˆË‡ÌË ÓÚ„Ó‚ÓÌË Á‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ „ÂÌË, ‡ ÔË ‰Û„Ë ÚÓ‚‡ ÚÂÔ˙‚‡ ÔÂ‰ÒÚÓË. äÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡ Ì‡ ‚ÒË˜ÍË Ù‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË Â ÒıÓ‰Ì‡ Ò Ú‡ÁË Ì‡ ÒÔÓ‡‰Ë˜ÌËÚÂ ÙÓÏË, ÂÚÓ Á‡˘Ó Ò‡ÏÓ Ì‡ÒÓ˜ÂÌÓÚÓ Ú˙ÒÂÌÂ Ì‡ Ù‡ÏËÎÌ‡ ‡Ì‡ÏÌÂÁ‡ ÓÚ ÒÚ‡Ì‡ Ì‡ ÍÎËÌËˆËÒÚËÚÂ, ·Ë ÔÓÏÓ„Ì‡ÎÓ Á‡ ÔÓÒÚ‡‚ﬂÌÂ Ì‡ ÚÓ˜Ì‡ ‰Ë‡„ÌÓÁ‡. ñÂÎÚ‡ Ì‡ ÚÓÁË Ó·ÁÓ Â ‰‡ ÒÂ ÔÂ‰ÒÚ‡‚ﬂÚ ÓÒÌÓ‚ÌËÚÂ ÍÎËÌË˜ÌË, ÂÎÂÍÚÓÙËÁËÓÎÓ„Ë˜ÌË Ë „ÂÌÂÚË˜ÌË ı‡‡ÍÚÂËÒÚËÍË Ì‡ ÌﬂÍÓË ÓÚ ÌÓ‚ÓÓÚÍËÚËÚÂ ‚ ÔÓÒÎÂ‰ÌÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ Ù‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË Ò ‡‚ÚÓÁÓÏÌÓ – ‰ÓÏËÌ‡ÌÚÓ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ. äãûóéÇà Ñìåà: „ÂÌÂÚËÍ‡, ‡‚ÚÓÁÓÏÌÓ ‰ÓÏËÌ‡ÌÚÓ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ, Ù‡ÏËÎÌË ÙÓÍ‡ÎÌË ÂÔËÎÂÔÒËË. ÖÔËÎÂÔÒËﬂÚ‡ Â Â‰ÌÓ ÓÚ Ì‡È-˜ÂÒÚËÚÂ ıÓÌË˜ÌË ÌÂ‚ÓÎÓ„Ë˜ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ, ÍÓÂÚÓ ÒÂ ı‡‡ÍÚÂËÁË‡ Ò ËÌ‰Ë‚Ë‰Û‡ÎÌ‡ ÔÂ‰‡ÁÔÓÎÓÊÂÌÓÒÚ Í˙Ï ÔÂËÓ‰Ë˜ÌÓ ÔÓ‚Ú‡ﬂ˘Ë ÒÂ ÔÓﬂ‚Ë, Ì‡Â˜ÂÌË ÂÔËÎÂÔÚË˜ÌË ÔËÒÚ˙ÔË. ÖÔËÎÂÔÒËﬂÚ‡ Â ‡ÁÔÓÒÚ‡ÌÂÌ‡ ‚˙‚ ‚ÒË˜ÍË ‡ÒË Ë Á‡Òﬂ„‡ ÔÓ˜ÚË Â‰Ì‡Í‚Ó ‰‚‡Ú‡ ÔÓÎ‡. á‡·ÓÎÂ‚‡ÂÏÓÒÚÚ‡ ÓÚ ÂÔËÎÂÔÒËﬂ Â 2070/100 000, Í‡ÚÓ Â Ì‡È-‚ËÒÓÍ‡ ‚ ‰ÂÚÒÍ‡Ú‡ (120-240/100 000) Ë ‚ ÒÚ‡˜ÂÒÍ‡Ú‡ ‚˙Á‡ÒÚ (140-150/100 000 ÒÎÂ‰ 70 „.) (1). íﬂ ÔÂ‰ÒÚ‡‚Îﬂ‚‡ ÏÛÎÚËÙ‡ÍÚÓÌÓ Á‡·ÓÎﬂ‚‡ÌÂ, ÍÓÂÚÓ ÒÂ ‡Á‚Ë‚‡ ÔË ‚Á‡ËÏÓ‰ÂÈÒÚ‚ËÂ Ì‡ ÌﬂÍÓÎÍÓ Ù‡ÍÚÓ‡, ËÏ‡˘Ë ‡ÁÎË˜Ì‡ ÚÂÊÂÒÚ ÔË ÓÚ‰ÂÎÌËÚÂ ÙÓÏË Ë ‚Ó‰Â˘Ë ‰Ó ÙÓÏË‡ÌÂ Ì‡ ÂÔËÎÂÔÚÓ„ÂÌÌÓ Ó„ÌË˘Â, Í‡ÚÓ ËÁ‡Á Ì‡ ÏËÍÓ- ËÎË Ï‡ÍÓÒÍÓÔÒÍË ÏÓÁ˙˜ÌË ÎÂÁËÁË. á‡ Û˜‡ÒÚËÂÚÓ Ì‡ „ÂÌÂÚË˜ÌË Ù‡ÍÚÓË ‚ ÂÚËÓÎÓ„ËﬂÚ‡ Ì‡ ÂÔËÎÂÔÒËËÚÂ ÒÂ Â ÁÌ‡ÂÎÓ Ó˘Â ÓÚ ‰Â‚ÌÓÒÚÚ‡ Ë ÚÓ‚‡ Â ‰ÓÔËÌÂÒÎÓ Á‡ ÒÚË„Ï‡ÚËÁ‡ˆËﬂÚ‡ Ì‡ ıÓ‡Ú‡ Ò ÂÔËÎÂÔÒËﬂ (33). ç‡È–„ÓÎÂÏËﬂÚ Ì‡ÔÂ‰˙Í Á‡ ‡ÁÍË‚‡ÌÂ „ÂÌÂÚËÍ‡Ú‡ Ì‡ ÂÔËÎÂÔÒËËÚÂ Á‡ÔÓ˜‚‡ ÓÚ 1930„. Ò ‚˙‚ÂÊ‰‡ÌÂÚÓ ÓÚ ìËÎﬂÏ ãÂÌÓÍÒ Ì‡ Ì‡Û˜ÌËﬂÚ ÏÂÚÓ‰ Á‡ ËÁÛ˜‡‚‡ÌÂÚÓ È. éÚ ÚÓ„‡‚‡ ‰Ó ‰ÌÂÒ ‚ ÂÁÛÎÚ‡Ú Ì‡ ÔÓÛ˜‚‡ÌËﬂ ‚˙ıÛ Á‡ÒÂ„Ì‡ÚË Ù‡ÏËÎËË Ë ÒÔÓÌÚ‡ÌÌË ËÎË ËÌ‰ÛˆË‡ÌË ÊË‚ÓÚËÌÒÍË ÏÓ‰ÂÎË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ì‡‰ 70 „ÂÌË Û˜‡ÒÚ‚‡˘Ë ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÂÔËÎÂÔÒËﬂÚ‡. ç‡È-„ÓÎﬂÏ ·ÓÈ ÓÚ Ë‰ÂÌÚËÙËˆË‡ÌËÚÂ „ÂÌË ÍÓ‰Ë‡Ú ÒÛ·Â‰ËÌËˆË Ì‡ ‚ÓÎÚ‡Ê Á‡‚ËÒËÏËÚÂ Ë ÎË„‡Ì‰ Á‡‚ËÒËÏËÚÂ ÈÓÌÌË Í‡Ì‡ÎË. çﬂÍÓÎÍÓ ÏÛÚ‡ˆËË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‚˙‚ ‚ÒÂÍË ÓÚ ÚÂÁË „ÂÌË, ÍÓÂÚÓ ÔÓÍ‡Á‚‡ ‡ÎÂÎÌ‡ ıÂÚÂÓ„ÂÌÌÓÒÚ. èÓ Ì‡ÒÚÓﬂ˘ÂÏ ÒÂ ÔËÂÏ‡, ˜Â „ÂÌÂÚË˜ÌËÚÂ Ù‡ÍÚÓË Û˜‡ÒÚ‚‡Ú ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÓÍÓÎÓ 40% ÓÚ ÂÔËÎÂÔÒËËÚÂ (59). ç‡È-˜ÂÒÚËÚÂ Ù‡ÏËÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË ÔÓÍ‡Á‚‡Ú ÍÓÏÔÎÂÍÒÌÓ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ. å‡Î˙Í Â ·Óﬂ Ì‡ ◊ ˜ËÒÚËÚÂ” ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË, ÍÓËÚÓ ÒÂ ÛÌ‡ÒÎÂ‰ﬂ‚‡Ú ÏÓÌÓ„ÂÌÌÓ Ë ÏÓ„‡Ú ‰‡ ·˙‰‡Ú „ÂÌÂ‡ÎËÁË‡ÌË ( Ì‡Ô. ·ÂÌË„ÌÂÌËÚÂ Ù‡ÏËÎÌË ÌÂÓÌ‡Ú‡ÎÌË ÍÓÌ‚ÛÎÒËË) ËÎË Ô‡ˆË‡ÎÌË (Ì‡Ô. ‡‚ÚÓÁÓÏÌÓ ‰ÓÏËÌ‡ÌÚÌ‡ ÌÓ˘Ì‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÙÓÌÚ‡ÎÌËﬂ ‰ﬂÎ). íÂ Ò‡ ‡‚ÌËÚÂÎÌÓ Â‰ÍË Ë ÒÂ ÓÚÍË‚‡Ú ‚ ÓÍÓÎÓ 1% ÓÚ ‚ÒË˜ÍË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËﬂ (59). äÓÌˆÂÔˆËﬂÚ‡ Á‡ Ì‡ÎË˜ËÂ Ì‡ Ù‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÒËÌ‰ÓÏË Â Ò‡‚ÌËÚÂÎÌÓ ÌÓ‚‡. Ñ˙Î„Ó ‚ÂÏÂ ÒÂ Â Ò˜ËÚ‡ÎÓ, ˜Â Ô‡ˆË‡ÎÌËÚÂ ÂÔËÎÂÔÒËË ÒÂ ‰˙ÎÊ‡Ú Ì‡ Ù‡ÍÚÓË Ì‡ ÓÍÓÎÌ‡Ú‡ ÒÂ‰‡ Í‡ÚÓ Ó‰Ó‚Ë Ë ÔÓÒÚ‡ÌÚ‡ÎÌË Ú‡‚ÏË, ËÌÙÂÍˆËË Ë ÏÓÁ˙˜ÌË ÎÂÁËË Ì‡Ô. ÚÛÏÓË Ë Ò˙‰Ó‚Ë ËÌÒÛÎÚË. Ç˙ÔÂÍË, ˜Â Ò‡ ÓÚÍË‚‡ÌË Ù‡ÏËÎËË Ò Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ, ÒÂ Â Ò˜ËÚ‡ÎÓ, ˜Â ÚÂ Ò‡ Ò ÍÓÏÔÎÂÍÒÌÓ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ. á‡ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ÚﬂıÌ‡Ú‡ „ÂÌÂÚËÍ‡ Ò‡ ËÁÔÓÎÁ‚‡ÌË ‡ÁÎË˜ÌË ÔÓ‰ıÓ‰Ë: ÂÔË‰ÂÏËÓÎÓ„Ë˜ÌËﬂ ÔÓ‰ıÓ‰ „Ë ‡Á„ÎÂÊ‰‡ Í‡ÚÓ ıÓÏÓ„ÂÌÌ‡ „ÛÔ‡; ÎÓ·‡ÌËﬂÚ ÔÓ‰ıÓ‰ ÍÎ‡ÒËÙËˆË‡ Ô‡ˆËÂÌÚËÚÂ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 168 ÒÔÓÂ‰ ÔÂ‰ÔÓÎ‡„‡ÂÏ‡ ËÎË ‰ÓÍ‡Á‡Ì‡ ‡Ì‡ÚÓÏË˜Ì‡ ÎÓÍ‡ÎËÁ‡ˆËﬂ; EEG – ÔÓ‰ıÓ‰‡ Ó·Â‰ËÌﬂ‚‡ ‰‡ÌÌËÚÂ ÓÚ ÔÓ‚Â‰ÂÌËÚÂ ÖÖÉ ËÁÒÎÂ‰‚‡ÌËﬂ; ÔË ÔÓ‰ıÓ‰‡ Ò ËÁÔÓÎÁ‚‡ÌÂ Ì‡ „ÓÎÂÏË Ù‡ÏËÎËË ÒÂ ÔÓ‚ÂÊ‰‡Ú ‡Ì‡ÎËÁË Á‡ ÒÍ‡˜ÂÌÓÒÚ Ë Ú˙ÒÂÌÂ Ì‡ „ÂÌËÚÂ ÓÚ„Ó‚ÓÌË Á‡ ‡Á‚ËÚËÂ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ(33). Ç ÔÓÒÎÂ‰ÌÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ Ò‡ Ë‰ÂÌÚËÙËˆË‡ÌË ÌﬂÍÓÎÍÓ Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏ‡ ÛÌ‡ÒÎÂ‰ﬂ‚‡˘Ë ÒÂ ‡‚ÚÓÁÓÏÌÓ - ‰ÓÏËÌ‡ÌÚÌÓ Ë ÚÂ Ò‡ ‚ÍÎ˛˜ÂÌË ‚ ÔÂ‰ÎÓÊÂÌËÂÚÓ Á‡ ÌÓ‚‡ ÍÎ‡ÒËÙËÍ‡ˆËﬂ Ì‡ ÂÔËÎÂÔÚË˜ÌËÚÂ ÒËÌ‰ÓÏË Ì‡ ILAE (International League Against Epilepsy) (28). íÓ‚‡ Ò‡: ÄÑ ÌÓ˘Ì‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÙÓÌÚ‡ÎÌËﬂ ÎÓ·, ÄÑ Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË, Ù‡ÏËÎÌ‡Ú‡ ÏÂÁË‡ÎÌ‡ ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ, Ù‡ÏËÎÌ‡Ú‡ ÓÍˆËÔËÚÓÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ, Ù‡ÏËÎÌ‡Ú‡ Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ò ÔÓÏÂÌÎË‚Ë Ó„ÌË˘‡ Ë Ô‡ˆË‡ÎÌ‡Ú‡ ÂÔËÎÂÔÒËﬂ Ò ÔÂËˆÂÌÚ‡ÎÌË ÒÔ‡ÈÍÓ‚Â (í‡·Î. 1). ÔÓ-ﬂ‰ÍÓ ‰Û„Ë ÒÂÚË‚ÌË (ÒÎÛıÓ‚Ë, ‚ÂÚË„ËÌÓÁÌË, ÁËÚÂÎÌË, Ó·ÓÌﬂÚÂÎÌË, ‚ÍÛÒÓ‚Ë) Ë ÔÒËıË˜ÌË (ÒÚ‡ı, deja vu) ÙÂÌÓÏÂÌË (60, 61). èË ‰‚Â ÚÂÚË ÓÚ Ô‡ˆËÂÌÚËÚÂ ÒÂ ‡Á‚Ë‚‡Ú ‚ÚÓË˜ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌË˜ÌÓ – ÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË. Ç˙ÁÏÓÊÌË Ò‡ Ë ‰ÌÂ‚ÌË ÔËÒÚ˙ÔË ÓÒÓ·ÂÌÓ ÔË Ô‡ˆËÂÌÚË Ò ÌÂ‰Ó·˙ ÍÓÌÚÓÎ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. ÖÖÉ ËÁ‚˙Ì ÔËÒÚ˙Ô Ó·ËÍÌÓ‚ÂÌÓ Â ÌÓÏ‡ÎÌ‡, ÌÓ ‚ 1265% ÓÚ ÒÎÛ˜‡ËÚÂ ÒÂ Â„ËÒÚË‡Ú ÙÓÌÚ‡ÎÌË ÂÔËÎÂÔÚËÙÓÏÂÌË ‡·ÌÓÏÌÓÒÚË, „Î‡‚ÌÓ ÔÓ ‚ÂÏÂ Ì‡ Ò˙Ì. àÍÚ‡ÎÌ‡Ú‡ ÖÖÉ Ò˙˘Ó ˜ÂÒÚÓ Â ÌÓÏ‡ÎÌ‡, ÌÓ ‚ 44%-88% ÒÂ Â„ËÒÚË‡Ú ÙÓÌÚ‡ÎÌË ÓÒÚËÂÚ‡, ÓÒÚËÂ-·‡‚Ì‡ ‚˙ÎÌ‡ ËÎË ËÚÏË˜ÌË ÚÂÚ‡ ‚˙ÎÌË Â‰ÌÓÒÚ‡ÌÌÓ ËÎË ‰‚ÛÒÚ‡ÌÌÓ (53). éÚ ÌÂ‚ÓËÁÓ·‡Áﬂ‚‡˘ËÚÂ ÏÂÚÓ‰Ë åêí Ó·ËÍÌÓ‚ÂÌÓ ÌÂ ÔÓÍ‡Á‚‡ ÓÚÍÎÓÌÂÌËﬂ ÓÚ ÌÓÏ‡Ú‡, ÌÓ ÔË PET/ SPECT ËÁÒÎÂ‰‚‡ÌÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÛÌËÎ‡ÚÂ‡ÎÌË ‡·ÌÓÏÌÓÒÚË ‚˙‚ ÙÓÌÚ‡ÎÌËﬂ ‰ﬂÎ (30). ç‡·Î˛‰‡‚‡Ú ÒÂ ËÁ‡ÁÂÌË ËÌÚ‡Ù‡ÏËÎÌË ‡ÁÎË˜Ëﬂ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ‚˙Á‡ÒÚ Ì‡ Ì‡˜‡ÎÓ, ÒÂÏËÓÎÓ„Ëﬂ Ì‡ ÔËÒÚ˙ÄÇíéáéåçé ÑéåàçÄçíçÄ çéôçÄ ÖèàãÖèëàü ÔËÚÂ Ë ÚÂÊÂÒÚ Ì‡ ÔÓÚË˜‡ÌÂ. èÓ‡‰Ë ÎÂÍ‡Ú‡ ÒÚÂÔÂÌ Ì‡ çÄ îêéçíÄãçàü Ñüã (ADNFLE) ADNFLE Â Ô˙‚‡Ú‡ ÓÔËÒ‡Ì‡ Ù‡ÏËÎÌ‡ Ô‡ˆË‡ÎÌ‡ ÂÔË- Á‡Òﬂ„‡ÌÂ Ì‡ Ó‰ÒÚ‚ÂÌËˆËÚÂ, Ù‡ÏËÎÌËﬂ ı‡‡ÍÚÂ Ì‡ Ò˙ÒÎÂÔÒËﬂ, ÔË ÍÓﬂÚÓ Â ÛÒÚ‡ÌÓ‚ÂÌ Â‰ËÌÒÚ‚ÂÌ „ÂÌ Í‡ÚÓ ÔË- ÚÓﬂÌËÂÚÓ ˜ÂÒÚÓ ÌÂ ÒÂ ‡ÁÔÓÁÌ‡‚‡. èËÒÚ˙ÔËÚÂ ÏÓ„‡Ú ˜ËÌ‡ Á‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (60, 61). ë‡ÏÓ ‰ÂÒÂÚËÎÂÚËÂ ÔÓ-Í˙Ò- ‰‡ ÒÂ Ò·˙Í‡Ú Ò Ô‡‡ÒÓÏÌËË, ÌÓ ÔÓÒÎÂ‰ÌËÚÂ Ò‡ ÔÓ-Â‰ÍË, ÌÓ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ (21) Ò‡ Ò˙Ó·˘ÂÌË Ì‡‰ 109 Ó‰ÓÒÎÓ‚Ëﬂ ÌÂ ÒÂ „ÛÔË‡Ú Ë ËÏ‡Ú ÒÍÎÓÌÌÓÒÚ ‰‡ ÓÚÁ‚Û˜‡‚‡Ú ‰Ó 18 Ò ADNFLE Ë ‚˙Á ÓÒÌÓ‚‡ Ì‡ Úﬂı Â ÛÒÚ‡ÌÓ‚ÂÌÓ ‡‚ÚÓÁÓÏÌÓ „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. äÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡ Ì‡ ADNFLE Â ÌÂ– ‰ÓÏËÌ‡ÌÚÌÓ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ Ò 84% ÔÂÌÂÚ‡ÌÌÓÒÚ Ë Â‰- ‡ÁÎË˜ËÏ‡ ÓÚ ÒÔÓ‡‰Ë˜ÌËÚÂ ÙÓÏË Ì‡ ÌÓ˘Ì‡Ú‡ ÂÔËÎÂÔÒËﬂ Ì‡ ˜ÂÎÌËﬂ ‰ﬂÎ, Í˙‰ÂÚÓ ËÌÚÂËÍÚ‡ÎÌ‡Ú‡ ÖÖÉ ˜ÂÒÚÓ Â Ì‡Í‚‡ ˜ÂÒÚÓÚ‡ Ì‡ Á‡Òﬂ„‡ÌÂ Ì‡ ‰‚‡Ú‡ ÔÓÎ‡. ç‡˜‡ÎÓÚÓ Â ‚ 1-2 ‰ÂÒÂÚËÎÂÚËÂ (ÒÂ‰ÌÓ 8-12 „Ó‰. ‚˙Á- ÌÓÏ‡ÎÌ‡, ‡ ËÍÚ‡ÎÌ‡Ú‡ ÖÖÉ Â Ï‡ÒÍË‡Ì‡ ÓÚ ÏÌÓÊÂÒÚ‚Ó ‡ÒÚ), Í‡ÚÓ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÔËÒÚ˙ÔËÚÂ Ì‡Ï‡Îﬂ‚‡ Ò ‚˙Á- ‡ÚÂÙ‡ÍÚË. èÓ„ÌÓÁ‡Ú‡ Â ‰Ó·‡. Ç˙ÁÏÓÊÌË Ò‡ ÒÔÓÌÚ‡ÌÌË ÂÏËÒËË. ‡ÒÚÚ‡. äÎËÌË˜ÌËÚÂ ÔÓﬂ‚Ë Ò‡ Í‡ÚÍË (20-50 ÒÂÍ.) Ë „Ûé·ËÍÌÓ‚ÂÌÓ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÔËÒÚ˙ÔËÚÂ Ì‡Ï‡Îﬂ‚‡ Í˙Ï ÔË‡ÌË ÏÓÚÓÌË ÔËÒÚ˙ÔË ÓÚ ıËÔÂÍËÌÂÚË˜ÂÌ ËÎË ÚÓÔÂÚÓÚÓ – ¯ÂÒÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ ÓÚ ÊË‚ÓÚ‡. ë‡ÏÓ ÔË ÌË˜ÂÌ ÚËÔ, ‚˙ÁÌËÍ‚‡˘Ë ÔÓ ‚ÂÏÂ Ì‡ çêÖå Ò˙Ìﬂ (ÓÒÓ·ÂÌÓ 2 ÒÚ‡‰ËÈ). éÔËÒ‡ÌË Ò‡ ‡ÁÎË˜ÌË ËÚÏË˜ÌË Ë ÔÓ‚Ú‡ﬂ˘Ë ÓÚ‰ÂÎÌË Ô‡ˆËÂÌÚË ÒÂ ÓÔËÒ‚‡Ú ˜ÂÒÚË, ÂÁËÒÚÂÌÚÌË Ì‡ ÎÂ˜ÂÌËÂ ÔËÒÚ˙ÔË. í‡·ÎËˆ‡ 1. î‡ÏËÎÌË Ô‡ˆË‡ÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË ëÂ‰Ì‡ ‚˙Á‡ÒÚ Ì‡ Ì‡˜‡ÎÓ ÖÔËÎÂÔÚË˜ÂÌ ÙÂÌÓÚËÔ ãÓÍ‡ÎËÁ‡ˆËﬂ Ì‡ „ÂÌ‡ Ä‚ÚÓÁÓÏÌÓ –‰ÓÏËÌ‡ÌÚÌ‡ ÌÓ˘Ì‡ ÂÔËÎÂÔÒËﬂ 1-2 ‰ÂÒÂÚËÎÂÚËÂ çÓ˘ÌË ÏÓÚÓÌË ÔËÒÚ˙ÔË CHRNA 4 Ì‡ ÙÓÌÚ‡ÎÌËﬂ ‰ﬂÎ ÓÚ ıËÔÂÍËÌÂÚË˜ÂÌ ÚËÔ, CHRNB2 ˜ÂÒÚÓ ÔË Á‡Ô‡ÁÂÌÓ Ò˙ÁÌ‡ÌËÂ CHRNA2 Ä‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÌ‡ Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ 2 - 4 ÚÓ ‰ÂÒÂÚËÎÂÚËÂ ÄÛ‡ Ò˙Ò ÒÎÛıÓ‚Ë, ÁËÚÂÎÌË, LGI1 Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË ‰ËÒÙ‡ÁË˜ÌË ÒËÏÔÚÓÏË î‡ÏËÎÌË ÏÂÁË‡ÎÌË ÂÔËÎÂÔÒËË Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ 2 – 4ÚÓ ‰ÂÒÂÚËÎÂÚËÂ Deja vu, ‡‚ÚÓÌÓÏÌ‡ Ë ‡ÙÂÍÚË‚Ì‡ 4q* - ·ÂÁ HS, ·ÂÁ ÙÂ·ËÎÌË ÔËÒÚ˙ÔË ‡Û‡ Ë ‰Ó·ÓÍ‡˜ÂÒÚ‚ÂÌ ıÓ‰ - Ò HS, +/- ÙÂ·ËÎÌË ÔËÒÚ˙ÔË 1 – 3 ÚÓ ‰ÂÒÂÚËÎÂÚËÂ Deja vu, ‡‚ÚÓÌÓÏÌ‡ Ë ‡ÙÂÍÚË‚Ì‡ ÌÂËÁ‚ÂÒÚÂÌ ‡Û‡, ˜ÂÒÚÓ ÂÙ‡ÍÚÓÌ‡ - ·ÂÁ HS, Ò ÙÂ·ËÎÌË ÔËÒÚ˙ÔË 1 – 2 Ó ‰ÂÒÂÚËÎÂÚËÂ Deja vu, ‡‚ÚÓÌÓÏÌ‡ ‡Û‡ 18q*, 1q* î‡ÏËÎÌ‡ ÓÍˆËÔËÚÓÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ 1 - 4 ÚÓ ‰ÂÒÂÚËÎÂÚËÂ ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, 9q* ÏË„ÂÌ‡, ‡ÁÌÓÓ·‡ÁÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË î‡ÏËÎÌ‡ Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ò ÔÓÏÂÌÎË‚Ë Ó„ÌË˘‡ 1 – 3 ÚÓ ‰ÂÒÂÚËÎÂÚËÂ ÙÓÌÚ‡ÎÌË, ÚÂÏÔÓ‡ÎÌË, ÓÍˆËÔËÚ‡ÎÌË, ˆÂÌÚÓÚÂÏÔÓ‡ÎÌË ÔËÒÚ˙ÔË 22q12 è‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ò ÔÂËˆÂÌÚ‡ÎÌË ÒÔ‡ÈÍÓ‚Â 1 – 2 Ó ‰ÂÒÂÚËÎÂÚËÂ ïÂÏËÍÓÌ‚ÛÎÒËË ˜ÂÒÚÓ ÌÓ˘ÂÏ, ‰Û„Ë 415* Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË *ëÍ‡˜ÂÌÓÒÚ Â ÛÒÚ‡ÌÓ‚ÂÌ‡ ÔË Â‰Ì‡ Ù‡ÏËÎËﬂ, HS – ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡ *åÓ‰ËÙËÍ‡ˆËﬂ ÔÓ Helbig et al. (2008)3 ÒÂ ÔÓÚÂÔ‚‡ÌËﬂ Ì‡ ˙ˆÂÚÂ Ë Í‡Í‡Ú‡, ·˙ÁË ÌÂÍÓÓ‰ËÌË‡ÌË ‰‚ËÊÂÌËﬂ Ò ‰ËÒÚÓÌË˜ÂÌ ËÎË ‰ËÒÍËÌÂÚË˜ÂÌ ÍÓÏÔÓÌÂÌÚ, ÒÎÓÊÌË ÏÓÚÓÌË ‰ÂÈÌÓÒÚË, ‚ÌÂÁ‡ÔÌË ÔÓ‚‰Ë„‡ÌËﬂ Ì‡ ÚﬂÎÓÚÓ Ë „Î‡‚‡Ú‡ Ò˙ÔÓ‚Ó‰ÂÌË ÓÚ ˜Û‚ÒÚ‚Ó Ì‡ ÒÚ‡ı Ë ‚ÓÍ‡ÎËÁ‡ˆËﬂ. ë˙ÌﬂÚ ﬂÁÍÓ ÒÂ ÔÂÍ˙Ò‚‡, ‡ ÒÎÂ‰ ÔËÒÚ˙Ô‡ ÒÂ ‚˙Á‚˙˘‡ ÓÚÌÓ‚Ó. è‡ˆËÂÌÚËÚÂ ˜ÂÒÚÓ Ò˙Ó·˘‡‚‡Ú Á‡ ‡Û‡, ‡ Ò˙ÁÌ‡ÌËÂÚÓ ‚ ÏÌÓ„Ó ÓÚ ÒÎÛ˜‡ËÚÂ Â Á‡Ô‡ÁÂÌÓ. ÄÛ‡Ú‡ Â ÌÂÒÔÂˆËÙË˜Ì‡ – „ÂÌÂ‡ÎËÁË‡ÌÓ ËÁÚ˙Ô‚‡ÌÂ Ë ÚÂÔÂÂÌÂ, ˆÂÙ‡Î„Ë˜ÌË, ÚÓ‡Í‡ÎÌË Ë ÂÔË„‡ÒÚ‡ÎÌË ÛÒÂ˘‡ÌËﬂ, èÓÌ‡ÒÚÓﬂ˘ÂÏ Ò‡ Ë‰ÂÌÚËÙËˆË‡ÌË ÚË ÎÓÍÛÒ‡ Á‡ ADNFLE. íÂ Ò‡ Í‡ÚË‡ÌË Ì‡ ıÓÏÓÁÓÏË 20q13 (ENFL1), 15q24 ( ENFL2), 1q21 (ENFL3). ë‡ÏÓ ÔË ‰‚‡ ÓÚ Úﬂı (ENFL1 Ë ENFL3) Â Ë‰ÂÌÚËÙËˆË‡Ì „ÂÌ ÓÚ„Ó‚ÓÂÌ Á‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. éÚ ÓÚÍË‚‡ÌÂÚÓ Ì‡ Ô˙‚Ëﬂ ÎÓÍÛÒ ENFL1 ‰Ó ÒÂ„‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ˜ÂÚËË ‡ÁÎË˜ÌË ÏÛÚ‡ˆËË ‚ CHRNA4 „ÂÌ‡ ÍÓ‰Ë‡˘ α4 ÒÛ·Â‰ËÌËˆ‡Ú‡ Ì‡ ÌÂ‚ÓÌ‡ÎÌËﬂ ÌËÍÓÚËÌÓ‚ ‡ˆÂÚËÎıÓÎËÌÓ‚ ÂˆÂÔÚÓ (nAChR). íË Ò‡ ÏËÒÒÂÌÒ ÏÛÚ‡ˆËË (S252F, S256L Ë T265I), ‡ Â‰Ì‡ Â ËÌÒÂˆËﬂ Ì‡ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÎÂ‚ˆËÌ (263InsL). íÂ Ò‡ Ò˙Ó·˘ÂÌË Ò‡ÏÓ ‚ 10 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË (21, 35, 46, 47) Ë ÌÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÔË ÓÒÚ‡Ì‡ÎËÚÂ ADNFE Ù‡ÏËÎËË (ÓÍÓÎÓ 90% ) (22, 25, 50, 54, 55), ÍÓÂÚÓ ÔÂ‰ÔÓÎ‡„‡, ˜Â ÏÛÚ‡ˆËËÚÂ ‚ CHRNA4 „ÂÌ‡ ËÏ‡Ú Ï‡Î˙Í ÔËÌÓÒ ÔË ADNFE. Ç˙‚ ‚ÒË˜ÍËÚÂ 10 Ù‡ÏËÎËË ÏÛÚ‡ˆËËÚÂ Ò‡ ·ËÎË ‚ ıÂÚÂÓÁË„ÓÚÌÓ Ò˙ÒÚÓﬂÌËÂ, ÍÓÂÚÓ Â ‚ Ò˙ÓÚ‚ÂÚÒÚ‚ËÂ Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÌËﬂ ÏÓ‰ÂÎ Ì‡ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ. èÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ENFL3 ÎÓÍÛÒ‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ‰‚Â ‡ÁÎË˜ÌË ÏÛÚ‡ˆËË (V287M and V287L) ‚ CHRNB2 „ÂÌ‡, ÍÓ‰Ë‡˘ β2 ÒÛ·Â‰ËÌËˆ‡Ú‡ Ì‡ ÌËÍÓÚËÌÓ‚Ëﬂ ‡ˆÂÚËÎıÓÎËÌÓ‚ ÂˆÂÔÚÓ ÓÚÍËÚË ‚ ÚË Ö‚ÓÔÂÈÒÍË Ù‡ÏËÎËË (22, 25, 54). àÁ‚ÂÒÚÌËÚÂ ÏÛÚ‡ˆËË ËÏ‡Ú ‡ÁÎË˜ÂÌ ÂÙÂÍÚ ‚˙ıÛ Ò‚ÓÈÒÚ‚‡Ú‡ Ì‡ ÌÂ‚ÓÌ‡ÎÌËﬂ ÌËÍÓÚËÌÓ‚ ‡ˆÂÚËÎıÓÎËÌÓ‚ ÂˆÂÔÚÓ, ÌÓ Í‡ÈÌËﬂÚ ÂÁÛÎÚ‡Ú Â Ì‡Ï‡ÎÂÌ ÔÂÏÂ‡·ËÎËÚÂÚ Á‡ ë‡++, Û‚ÂÎË˜ÂÌ ÔÂËÓ‰ Ì‡ ËÌ‡ÍÚË‚ËÚÂÚ, Ì‡Ï‡ÎÂÌÓ ‚ÂÏÂ Ì‡ ÓÚ‚‡ﬂÌÂ Ì‡ Í‡Ì‡Î‡ Ë ÔÓ‚Ë¯ÂÌ‡ ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ Í˙Ï ‡„ÓÌËÒÚË (56). é·˘Ó ÏÛÚ‡ˆËËÚÂ ‚ CHRNA4 Ë CHRNB2 „ÂÌËÚÂ ÒÂ ÓÚÍË‚‡Ú Ò‡ÏÓ ‚ 12% ÓÚ Ù‡ÏËÎËËÚÂ Ò ADNFE, ‰ÓÍ‡ÚÓ ÔË ÓÒÚ‡Ì‡ÎËÚÂ 88% ÓÚ ÒÎÛ˜‡ËÚÂ „ÂÌÂÚË˜ÌËﬂÚ ‰ÂÙÂÍÚ ÓÒÚ‡‚‡ ÌÂËÁ‚ÂÒÚÂÌ. èÂ‰ÔÓÎ‡„‡ ÒÂ Ò˙˘ÂÒÚ‚Û‚‡ÌÂ Ë Ì‡ ˜ÂÚ‚˙ÚË ÎÓÍÛÒ Ò „ÂÌ ‡ÁÎË˜ÂÌ ÓÚ ÚÂÁË ÍÓ‰Ë‡˘Ë ÒÛ·Â‰ËÌËˆË Ì‡ ÌÂ‚ÓÌ‡ÎÌËﬂ ÌËÍÓÚËÌÓ‚ ‡ˆÂÚËÎıÓÎËÌÓ‚ ÂˆÂÔÚÓ (13). ë˙˘ÂÒÚ‚Û‚‡ÌÂÚÓ Ì‡ ‡ÁÎË˜ÌË ÎÓÍÛÒË, Í‡ÍÚÓ Ë ‚‡Ë‡·ËÎÌ‡Ú‡ ÂÍÒÔÂÒË‚ÌÓÒÚ Ì‡ ÒËÏÔÚÓÏËÚÂ Ì‡·Î˛‰‡‚‡Ì‡ ÒÂ‰ ˜ÎÂÌÓ‚ÂÚÂ Ì‡ Â‰Ì‡ Ë Ò˙˘‡ Ù‡ÏËÎËﬂ Ò Â‰ËÌ Ë Ò˙˘ „ÂÌÂÚË˜ÂÌ ‰ÂÙÂÍÚ ÔÂ‰ÔÓÎ‡„‡ Ì‡ÎË˜ËÂÚÓ Ì‡ ÏÓ‰ËÙËˆË‡˘Ë „ÂÌË Ë ÌÂ„ÂÌÂÚË˜ÌË Ù‡ÍÚÓË ÏÓ‰ÛÎË‡˘Ë ÙÂÌÓÚËÔ‡ Ì‡ ‚ÒÂÍË ËÌ‰Ë‚Ë‰ (21). îÄåàãçà ÖèàãÖèëàà çÄ íÖåèéêÄãçàü Ñüã ë˙˘ÂÒÚ‚Û‚‡Ú ‰‚‡ ÓÒÌÓ‚ÌË ÚËÔ‡ Ù‡ÏËÎÌË ÂÔËÎÂÔÒËË Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ÒÂÏËÓÎÓ„ËﬂÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ, „ÂÌÂÚË˜ÌËÚÂ Ë åêí ı‡‡ÍÚÂËÒÚËÍË: Î‡ÚÂ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ (FLTLE, Ì‡Â˜ÂÌ‡ Ó˘Â ÄÑ Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË) Ë ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ (FMTLE). ãÄíÖêÄãçÄ ÖèàãÖèëàü çÄ íÖåèéêÄãçàü Ñüã (ÄÑ èÄêñàÄãçÄ ÖèàãÖèëàü ëöë ëãìïéÇà ëàåèíéåà) íÓ‚‡ Â ·ÂÌË„ÌÂÌ ÂÔËÎÂÔÚË˜ÂÌ ÒËÌ‰ÓÏ ÓÔËÒ‡Ì Á‡ Ô˙‚Ë Ô˙Ú ‚ ÎËÚÂ‡ÚÛ‡Ú‡ ÔÂÁ 1995„. ÓÚ Ottman et al. (51). ìÌ‡ÒÎÂ‰ﬂ‚‡ ÒÂ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÌÓ Ò 50-80% ÔÂÌÂÚ‡ÌÌÓÒÚ (58). Ç˙Á‡ÒÚÚ‡ Ì‡ Ì‡˜‡ÎÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚˙‚ ‚ÚÓÓÚÓ Ë ÚÂÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ. Ñ‚‡Ú‡ ÔÓÎ‡ ÒÂ Á‡Òﬂ„‡Ú Ò Â‰Ì‡Í‚‡ ˜ÂÒÚÓÚ‡. èÓﬂ‚ﬂ‚‡ ÒÂ Ò ÔÓÒÚË ÒÎÛıÓ‚Ë ı‡Î˛ˆËÌ‡ˆËË ÔÓ‰ ÙÓÏ‡Ú‡ Ì‡ ·˙Ï˜ÂÌÂ, Á‚˙ÌÂÌÂ, ˘‡Í‡ÌÂ Ë ÌÂı‡‡ÍÚÂÂÌ ¯ÛÏ. éÔËÒ‡ÌË Ò‡ Ë ‰Û„Ë ÔÒËıË˜ÌË, ÒÂÚË‚ÌË (ÁËÚÂÎÌË, Ó·ÓÌﬂÚÂÎÌË, ‚ÂÚË„ËÌÓÁÌË ) Ë ÏÓÚÓÌË ÙÂÌÓÏÂÌË, ÌÓ ‡Û‡Ú‡ Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË Â Ì‡È- ı‡‡ÍÚÂÌ‡Ú‡ ÔÓﬂ‚‡ Ì‡ ÚÓÁË ÒËÌ‰ÓÏ. ë˙Ó·˘ÂÌË Ò‡ Ù‡ÏËÎËË ÔË ÍÓËÚÓ ÔÂÓ·Î‡‰‡‚‡˘‡ ÍÎËÌË˜Ì‡ ËÁﬂ‚‡ Ì‡ ÒËÌ‰ÓÏ‡ Â ËÍÚ‡ÎÌ‡ ÒÂÌÁÓÌ‡ ‡Ù‡ÁËﬂ (15, 16, 39), Ú‡ÍË‚‡ ÔË ÍÓËÚÓ ÔËÒÚ˙ÔËÚÂ ÒÂ ÔÓ‚ÓÍË‡Ú ÓÚ ÚÂÎÂÙÓÌÂÌ Á‚˙Ì (48, 66), Í‡ÍÚÓ Ë Ú‡ÍË‚‡ Ò ˜ÂÒÚË ‚ÚÓË˜ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌË˜ÌÓ-ÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË (15, 48, 66). çÂ‚ÓÔÒËıË˜ÌÓÚÓ ‡Á‚ËÚËÂ Â ÌÓÏ‡ÎÌÓ. ÖÖÉ Ì‡È-˜ÂÒÚÓ Â ÌÓÏ‡ÎÌ‡ ËÎË Ò ÂÔËÎÂÔÚËÙÓÏÂÌË ‡Áﬂ‰Ë Á‡‰ÌÓ ÚÂÏÔÓ‡ÎÌÓ. åêí ËÁÒÎÂ‰‚‡ÌÂÚÓ Â ÌÓÏ‡ÎÌÓ ËÎË ÔÓÍ‡Á‚‡ Ï‡ÎÙÓÏ‡ˆËË ‚ Î‡ÚÂ‡ÎÌËÚÂ Ó·Î‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ ÔË ÓÍÓÎÓ 45% ÓÚ Á‡ÒÂ„Ì‡ÚËÚÂ ËÌ‰Ë‚Ë‰Ë ( ‚ÍÎ. Ë ÔË ‡ÒËÏÔÚÓÏÂÌ ÌÓÒËÚÂÎ Ì‡ ÏÛÚ‡ˆËﬂ) (42). ãÂ‚ËﬂÚ ÚÂÏÔÓ‡ÎÂÌ ‰ﬂÎ Ì‡ ÚÂÁË Ô‡ˆËÂÌÚË ËÁ„ÎÂÊ‰‡ Û„ÓÎÂÏÂÌ Ë ÔÓÌﬂÍÓ„‡ ÒÂ Ì‡·Î˛‰‡‚‡ ÔÓÚÛÁËﬂ Ì‡ ÏÓÁ˙˜ÌËﬂ Ô‡ÂÌıËÏ Î‡ÚÂ‡ÎÌÓ (ÂÌˆÂÙ‡ÎÓˆÂÎÂ ÔÓ‰Ó·ÂÌ ‚Ë‰). 169 áÌ‡˜ÂÌËÂÚÓ Ì‡ ÚÂÁË ÒÚÛÍÚÛÌË ‡ÌÓÏ‡ÎËË Á‡ ÂÔËÎÂÔÚÓ„ÂÌÂÁ‡Ú‡ Â ‚ÒÂ Ó˘Â ÌÂËÁ‚ÂÒÚÌÓ. ÑËÙÂÂÌˆË‡ÎÌ‡ ‰Ë‡„ÌÓÁ‡ ÒÂ Ô‡‚Ë Ò˙Ò ÒËÏÔÚÓÏ‡ÚË˜Ì‡ Î‡ÚÂ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ, Í‡ÍÚÓ Ë Ù‡ÏËÎÌ‡ ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ. èÓ„ÌÓÁ‡Ú‡ Â ‰Ó·‡, ÔËÒÚ˙ÔËÚÂ Ò‡ ÎÂÍË, Â‰ÍË Ë ÒÂ ÍÓÌÚÓÎË‡Ú ‰Ó·Â Ò ‡ÌÚËÂÔËÎÂÔÚË˜ÌË ÏÂ‰ËÍ‡ÏÂÌÚË. èË ÓÍÓÎÓ 50% ÓÚ Ù‡ÏËÎËËÚÂ Ò Î‡ÚÂ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÏÛÚ‡ˆËﬂ ‚ leucin-rich, glioma inactivated 1 „ÂÌ‡ ( LGI1 ) ÎÓÍ‡ÎËÁË‡Ì Ì‡ ‰˙Î„ÓÚÓ ‡ÏÓ Ì‡ 10 ıÓÏÓÁÓÏ‡-10q (10, 38, 48, 51). ç‡‰ 20 ‡ÁÎË˜ÌË ÏÛÚ‡ˆËË ‚ ÚÓÁË „ÂÌ Ò‡ Ò˙Ó·˘ÂÌË ‰Ó ÒÂ„‡, ÔÓ‚Â˜ÂÚÓ ÓÚ ÍÓËÚÓ Ò‡ ◊˜‡ÒÚÌË” Ú.Â ÒÔÂˆËÙË˜ÌË Á‡ ÓÚ‰ÂÎÌËÚÂ Ù‡ÏËÎËË (52). LGI1 „ÂÌ‡ ÍÓ‰Ë‡ ÏÂÏ·‡ÌÌÓ Ò‚˙Á‡Ì ÔÓÚÂËÌ Ò ÌÂËÁ‚ÂÒÚÌ‡ ÙÛÌÍˆËﬂ. ï‡‡ÍÚÂËÁË‡ ÒÂ Ò ˆÂÌÚ‡ÎÂÌ ·Ó„‡Ú Ì‡ ÎÂ‚ˆËÌ Â„ËÓÌ, ÍÓÈÚÓ ‚ÂÓﬂÚÌÓ Û˜‡ÒÚ‚‡ ‚ Â„ÛÎ‡ˆËﬂÚ‡ Ì‡ ÍÎÂÚ˙˜ÌËﬂ ‡ÒÚÂÊ, ‡‰ıÂÁËﬂ Ë ÏË„‡ˆËﬂ (36). äÎÓÌË‡Ì Â ÓÚ „ÎËÓ·Î‡ÒÚÓÏÌ‡ ÍÎÂÚ˙˜Ì‡ ÎËÌËﬂ Ë ‚ÂÓﬂÚÌÓ ÔÂ‰ÒÚ‡‚Îﬂ‚‡ ÚÛÏÓ-ÒÛÔÂÒÓÂÌ „ÂÌ (19). ÄÒÓˆËË‡ÌÂÚÓ ÏÛ Ò Ï‡ÎÙÓÏ‡ˆËË Ì‡ Î‡ÚÂ‡ÎÌËÚÂ Ó·Î‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ ÔË ÌﬂÍÓË Ù‡ÏËÎËË ÔÂ‰ÔÓÎ‡„‡ ‚ÂÓﬂÚÌÓÚÓ ÏÛ ÁÌ‡˜ÂÌËÂ Á‡ ‡Á‚ËÚËÂÚÓ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ (42). åÛÚ‡ˆËËÚÂ ‚ LGI1 „ÂÌ‡ ËÁ„ÎÂÊ‰‡ Ò‡ ÒÔÂˆËÙË˜ÌË Á‡ ÒËÌ‰ÓÏ‡, ÌÓ Ë‰ÂÌÚËÙËˆË‡ÌÂÚÓ ËÏ Ò‡ÏÓ ÔË ÔÓÎÓ‚ËÌ‡Ú‡ ÓÚ Ù‡ÏËÎËËÚÂ Ò ÚËÔË˜ÂÌ ÙÂÌÓÚËÔ ÔÓÍ‡Á‚‡ „ÂÌÂÚË˜Ì‡Ú‡ ıÂÚÂÓ„ÂÌÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (10). Bisulli et al. ( 2004 )(12) ÓÔËÒ‚‡Ú 53 ÒÔÓ‡‰Ë˜ÌË ÒÎÛ˜‡ﬂ Ì‡ Î‡ÚÂ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ. ëËÌ‰ÓÏ˙Ú Â Ì‡Â˜ÂÌ ÓÚ ‡‚ÚÓËÚÂ Ë‰ËÓÔ‡ÚË˜Ì‡ Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ò˙Ò ÒÎÛıÓ‚Ë ÒËÏÔÚÓÏË Ë ÌÂ ÒÂ ‡ÁÎË˜‡‚‡ ÍÎËÌË˜ÌÓ ÓÚ Ù‡ÏËÎÌ‡Ú‡ ÙÓÏ‡. èË ÌÂ„Ó Ó·‡˜Â ÎËÔÒ‚‡ ÏÛÚ‡ˆËﬂ ‚ LGI1 „ÂÌ‡. îÄåàãçÄ åÖáàÄãçÄ ÖèàãÖèëàü çÄ íÖåèéêÄãçàü Ñüã (FMTLE) éÔËÒ‡Ì‡ Â Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Bercovic et al. (7, 8) ÔÂÁ 1994„. Í‡ÚÓ ·ÂÌË„ÌÂÌ ÂÔËÎÂÔÚË˜ÂÌ ÒËÌ‰ÓÏ. Ç ÔÓÒÎÂ‰ÒÚ‚ËÂ ÒÂ ÓÚÍË‚‡Ú Ù‡ÏËÎËË Ò ÌÂ ÚÓÎÍÓ‚‡ ‰Ó·ÓÍ‡˜ÂÒÚ‚ÂÌÓ ÔÓÚË˜‡ÌÂ Ì‡ ÂÔËÎÂÔÒËﬂÚ‡ Ë ÔÂ‰¯ÂÒÚ‚‡˘Ë ÛÒÎÓÊÌÂÌË ÙÂ·ËÎÌË ÔËÒÚ˙ÔË, Í‡ÍÚÓ Ë ËÁ‡ÁÂÌ‡ ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡. íÓ‚‡ ‰Ó‚ÂÊ‰‡ ‰Ó ‡Á‰ÂÎﬂÌÂÚÓ Ì‡ ÒËÌ‰ÓÏ‡ Ì‡ ÚË ÓÒÌÓ‚ÌË „ÛÔË: ● ·ÂÌË„ÌÂÌ‡ Ù‡ÏËÎÌ‡ ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ: ë˙ÓÚ‚ÂÚÒÚ‚‡ Ì‡ ÓË„ËÌ‡ÎÌÓÚÓ ÓÔËÒ‡ÌËÂ Ì‡ Bercovic et al.(1994, 1996) (7, 8). ìÌ‡ÒÎÂ‰ﬂ‚‡ ÒÂ ‡‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÌÓ Ò ÌÂÔ˙ÎÌ‡ ÔÂÌÂÚ‡ÌÌÓÒÚ. ÜÂÌËÚÂ ÒÂ Á‡Òﬂ„‡Ú ÔÓ˜ÂÒÚÓ ÓÚ Ï˙ÊÂÚÂ. ç‡˜‡ÎÓÚÓ Â ÏÂÊ‰Û ‚ÚÓÓÚÓ Ë ˜ÂÚ‚˙ÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ ÓÚ ÊË‚ÓÚ‡ (ÌÂ ÔÓ-‡ÌÓ ÓÚ 10 „Ó‰. ‚˙Á‡ÒÚ, ÒÂ‰ÌÓ 25 „Ó‰ ). ãËÔÒ‚‡ ‡Ì‡ÏÌÂÁ‡ Á‡ ÔÂ‰¯ÂÒÚ‚‡˘Ë ÙÂ·ËÎÌË ÔËÒÚ˙ÔË, ‡ „Î‡‚ÌËÚÂ ÂÔËÎÂÔÚË˜ÌË ÔÓﬂ‚Ë ‚ Á‡ÒÂ„Ì‡ÚËÚÂ ËÌ‰Ë‚Ë‰Ë Ò‡ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò ÔÒËıË˜ÌË Ë ‡‚ÚÓÌÓÏÌË ÒËÏÔÚÓÏË, ÔÂ‰ÔÓÎ‡„‡˘Ë ÔÓËÁıÓ‰ ÓÚ ÏÂÁË‡ÎÌËﬂ ‰ﬂÎ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ÎÓ·. ç‡È-˜ÂÒÚËÚÂ ÒËÏÔÚÓÏË Ì‡ ÂÔËÎÂÔÚË˜Ì‡Ú‡ ‡Û‡ Ò‡ ÙÂÌÓÏÂÌË Ì‡ deja-vu Ë jamais vu Ë ÔÓ-ﬂ‰ÍÓ „‡‰ÂÌÂ, Ú‡ıËÍ‡‰Ëﬂ, ÒÚ‡ı, ˜Û‚ÒÚ‚Ó Á‡ Á‡·‡‚ÂÌÓÒÚ Ì‡ ‰‚ËÊÂÌËﬂÚ‡ Ë ÒÍÓ‚‡ÌÓÒÚ, ‰ÂÙÓÏË‡ÌÓ ‚˙ÁÔËÂÏ‡ÌÂ Ì‡ Ó·ÂÍÚËÚÂ Ë Á‚ÛˆËÚÂ, ‰ËÙÛÁÌË Ô‡ÂÒÚÂÁË. ãËÔÒ‚‡ ÂÔË„‡ÒÚ‡ÎÌ‡ ‡Û‡. èË ÔÓ‚Â˜ÂÚÓ Á‡ÒÂ„Ì‡ÚË ËÌ‰Ë‚Ë‰Ë ÂÔËÎÂÔÒËﬂÚ‡ ÔÓÚË˜‡ Ò‡ÏÓ Ò ÔÓ‚Ú‡ﬂ˘Ë ÒÂ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò „ÓÂÓÔËÒ‡Ì‡Ú‡ ı‡‡ÍÚÂËÒÚËÍ‡, ÍÓËÚÓ ˜ÂÒÚÓ ÌÂ ÒÂ ‡ÁÔÓÁÌ‡‚‡Ú Í‡ÚÓ ÂÔËÎÂÔÚË˜ÌË. ë˙Ó·˘‡‚‡Ú ÒÂ Ë Â‰ÍË, ÎÂÍË ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ë ‚ÚÓË˜ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌË˜ÌÓ – ÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË ÎÂÒÌÓ ÍÓÌÚÓÎË‡˘Ë ÒÂ Ò ‡ÌÚËÂÔËÎÂÔÚË˜ÌË ÏÂ‰ËÍ‡ÏÂÌÚË. çÂ‚ÓÎÓ„Ë˜ÌÓÚÓ Ë ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÔÒËıË˜ÌÓÚÓ ‡Á‚ËÚËÂ Ò‡ ÌÓÏ‡ÎÌË. àÌÚÂËÍÚ‡ÎÌ‡Ú‡ ÖÖÉ Â Ó·ËÍÌÓ‚ÂÌÓ ÌÓÏ‡ÎÌ‡, ËÎË Ò ÙÓÍ‡ÎÌË ·‡‚ÌË ‚˙ÎÌË, ËÎË Â‰ÍË ÍÓÏÔÎÂÍÒË ÓÒÚËÂ-·‡‚Ì‡ ‚˙ÎÌ‡ ÎÓÍ‡ÎËÁË‡ÌË Â‰ÌÓÒÚ‡ÌÌÓ ÚÂÏÔÓ‡ÎÌÓ. åêí ËÁÒÎÂ‰‚‡ÌÂÚÓ Â ÌÓÏ‡ÎÌÓ. èÓ„ÌÓÁ‡Ú‡ Â ÓÚÌÓÒËÚÂÎÌÓ ·Î‡„ÓÔËﬂÚÌ‡. èÓ-Í˙ÒÌÓ ÔÓ‰Ó·ÌË Ù‡ÏËÎËË Ò‡ Ò˙Ó·˘ÂÌË Ë ÓÚ ‰Û„Ë ‡‚ÚÓË (29, 57, 65), Í‡ÚÓ ‚ Â‰ËÌË˜Ì‡ „ÓÎﬂÏ‡ Ù‡ÏËÎËﬂ Â ÛÒÚ‡ÌÓ‚ÂÌ‡ ÒÍ‡˜ÂÌÓÒÚ Ò ıÓÏÓÁÓÏ‡ 4q13.2-q21.3 (31). ● Ù‡ÏËÎÌ‡ ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ ‡ÒÓˆËË‡Ì‡ Ò ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡: ç‡˜‡ÎÓÚÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚ Ô˙‚ËÚÂ ÚË ‰ÂÒÂÚËÎÂÚËﬂ ÓÚ ÊË‚ÓÚ‡ (42). ëÂÏËÓÎÓ„ËﬂÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ Â ı‡‡ÍÚÂÌ‡ Á‡ ÏÂÁË‡ÎÌËÚÂ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ - ÂÔË„‡ÒÚ‡ÎÌË ÛÒÂ˘‡ÌËﬂ ËÁ‰Ë„‡˘Ë ÒÂ Í˙Ï „˙ÎÓÚÓ Ë ÔÒËıË˜ÌË ÙÂÌÓÏÂÌË. óÂÒÚË Ò‡ ÍÓÏÔÎÂÍÒÌËÚÂ Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò Ó‡ÎÌË ËÎË ÊÂÒÚÓ‚Ë ‡‚ÚÓÏ‡ÚËÁÏË Ë ËÁ‡ÁÂÌÓ ÔÓÒÚËÍÚ‡ÎÌÓ Ó·˙Í‚‡ÌÂ ( ÔÓ‰Ó·ÌÓ Ì‡ ÚÓ‚‡ Ì‡·Î˛‰‡‚‡ÌÓ ÔË Ô‡ˆËÂÌÚË Ò ÌÂÙ‡ÏËÎÌ‡ ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ). ÇÚÓË˜ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌË˜ÌÓ-ÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË ÒÂ Ì‡·Î˛‰‡‚‡Ú Ó·ËÍÌÓ‚ÂÌÓ ‚ Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, ÔÂ‰Ë Á‡ÔÓ˜‚‡ÌÂ Ì‡ ÎÂ˜ÂÌËÂÚÓ. ÄÌ‡ÏÌÂÁ‡ Á‡ ÙÂ·ËÎÌË ÔËÒÚ˙ÔË ‚ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó ÒÂ ÓÚÍË‚‡ ÔÓﬂ‰ÍÓ ( ÓÍÓÎÓ 11% ) (42) ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚË Ò FMTLE ÔÓÒÎÂ‰ﬂ‚‡ÌË ‚ ıËÛ„Ë˜ÌË ÓÚ‰ÂÎÂÌËﬂ ( 30% ) (43) Ë Ó˘Â ÔÓ-ﬂ‰ÍÓ ÔË Ú‡ÍË‚‡ Ò ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ÌÂÒÂÎÂÍÚË‡ÌË Á‡ Ù‡ÏËÎÌ‡ ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ( 40% ) (2). àÌÚÂËÍÚ‡ÎÌ‡Ú‡ Ë ËÍÚ‡ÎÌ‡ ÖÖÉ ÔÓÍ‡Á‚‡Ú Â‰ÌÓÒÚ‡ÌÌË ËÎË ‰‚ÛÒÚ‡ÌÌË ÂÔËÎÂÔÚËÙÓÏÂÌË ﬂÁﬂ‰Ë ‚ ÏÂÁË‡ÎÌËÚÂ ÚÂÏÔÓ‡ÎÌË Ó·Î‡ÒÚË, ‚˙ÔÂÍË, ˜Â ÔË ÌﬂÍÓË Ô‡ˆËÂÌÚË ÖÖÉ Ì‡ıÓ‰Í‡Ú‡ ÏÓÊÂ ‰‡ Â ÌÓÏ‡ÎÌ‡. åêí ËÁÒÎÂ‰‚‡ÌÂÚÓ ‡ÁÍË‚‡ ‡ÁÎË˜Ì‡ ÔÓ ÒÚÂÔÂÌ ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡ ‰ÓË Ë ÔË ‡ÒËÏÔÚÓÏÌË ˜ÎÂÌÓ‚Â Ì‡ Á‡ÒÂ„Ì‡ÚËÚÂ Ù‡ÏËÎËË (44). íﬂ Ó·‡˜Â Â Ì‡È-˜ÂÒÚ‡ Ë Ì‡È-ÚÂÊÍÓ ËÁ‡ÁÂÌ‡ ÔË Ô‡ˆËÂÌÚË Ò ÂÙ‡ÍÚÂÌË Ì‡ ÎÂ˜ÂÌËÂ ÔËÒÚ˙ÔË (45). ç‡ÎË˜ËÂÚÓ Ì‡ åêí ‰‡ÌÌË Á‡ ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡ Í‡ÍÚÓ ÔË ‡ÒËÏÔÚÓÏÌË, Ú‡Í‡ Ë ÔË ·ÓÎÌË ˜ÎÂÌÓ‚Â Ì‡ Ù‡ÏËÎËﬂÚ‡ ÔÂ‰ÔÓÎ‡„‡ ‚˙ÁÏÓÊÌÓÒÚÚ‡ ÚÂÁË ‡·ÌÓÏÌÓÒÚË ‰‡ ÒÂ ÛÌ‡ÒÎÂ‰ﬂ‚‡Ú ·ÂÁ Á‡‰˙ÎÊËÚÂÎÌÓ ‰‡ ‚Ó‰ﬂÚ ‰Ó ‡Á‚ËÚËÂ Ì‡ ÂÔËÎÂÔÒËﬂ (44). ïÓ‰˙Ú Ì‡ ÔÓÚË˜‡ÌÂ Â ÓÚÌÓÒËÚÂÎÌÓ ·Î‡„ÓÔËﬂÚÂÌ Ò˙Ò ÒÔÓÌÚ‡ÌÌË ÂÏËÒËË. êÂÙ‡ÍÚÓÌË ÔËÒÚ˙ÔË ÒÂ ÓÔËÒ‚‡Ú ÔË ÓÍÓÎÓ 29% ÓÚ Ô‡ˆËÂÌÚËÚÂ (45). ÑÓ ÏÓÏÂÌÚ‡ ÌÂ Â Ë‰ÂÌÚËÙËˆË‡Ì ÓÚ„Ó‚ÓÂÌ Á‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ „ÂÌ. Ç˙ÔÂÍË, ˜Â Ò‡ ÓÔËÒ‚‡ÌË ÔÓÎËÏÓÙËÁÏË ÔË Ô‡ˆËÂÌÚË Ò ÏÂÁË‡ÎÌ‡ ÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ( prodynorphin „ÂÌ, Apoε „ÂÌ, GABAÇ „ÂÌ, prion protein „ÂÌ ) Ú‡ÍË‚‡ ÌÂ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÒÂ‰ Ó‰ÓÒÎÓ‚Ëﬂ Ò Ù‡ÏËÎÌ‡ ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ (18, 63). ● Ù‡ÏËÎÌ‡ ÏÂÁË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ ‡ÒÓˆËË‡Ì‡ Ò ÙÂ·ËÎÌË ÔËÒÚ˙ÔË, ·ÂÁ åêí ‰‡ÌÌË Á‡ ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡: Baulac et al. (2001) (6) ÓÔËÒ‚‡Ú ÙÂÌÒÍ‡ Ù‡ÏËÎËﬂ ÔË ÍÓﬂÚÓ ÒÂ Ì‡·Î˛‰‡‚‡Ú ÙÂ·ËÎÌË ÔËÒÚ˙ÔË ‡ÒÓˆËË‡ÌË Ò ÂÔËÎÂÔÒËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ, ·ÂÁ åêí ‰‡ÌÌË Á‡ ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡. Depondt et al. (2002„.) (23) ÓÔËÒ‚‡Ú „ÓÎﬂÏ‡ ·ÂÎ„ËÈÒÍ‡ Ù‡ÏËÎËﬂ Ò ÔÓ‰Ó·ÂÌ ÙÂÌÓÚËÔ. ìÌ‡ÒÎÂ‰ﬂ‚‡ÌÂÚÓ Â ‡‚ÚÓÁÓÏÌÓ ‰ÓÏËÌ‡ÌÚÓ Ò 80 % ÔÂÌÂÚ‡ÌÌÓÒÚ, Í‡ÚÓ Â Ì‡ÎËˆÂ Ë ËÁ‡ÁÂÌ‡ ÙËÌÓÚËÔÌ‡ ıÂÚÂÓ„ÂÌÌÓÒÚ. ëÂ‰Ì‡Ú‡ ‚˙Á‡ÒÚ Ì‡ Ì‡˜‡ÎÓ Ì‡ ‡ÙÂ·ËÎÌËÚÂ ÔËÒÚ˙ÔË Â 8 „Ó‰ËÌË. ëÂÏËÓÎÓ„ËﬂÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ Â ı‡‡ÍÚÂÌ‡ Á‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ, Í‡ÚÓ Ì‡È-˜ÂÒÚËÚÂ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò‡ ‚ËÒˆÂÓÒÂÌÁÓÌË, ÔÒËıË˜ÌË Ë ÓÚ ÒÚ‡Ì‡ Ì‡ ñçë. çÂ ÒÂ Ò˙Ó·˘‡‚‡Ú deja vu ÙÂÌÓÏÂÌË, ÒÎÛıÓ‚Ë ËÎË ÁËÚÂÎÌË ı‡Î˛ˆËÌ‡ˆËË. äÓÏÔÎÂÍÒÌËÚÂ Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË ÔÓÚË˜‡Ú Ò Ì‡Û¯ÂÌÓ Ò˙ÁÌ‡ÌËÂ, ‚ÚÂÌ˜‚‡ÌÂ, ÓÚÍÎÓÌﬂ‚‡ÌÂ Ì‡ „Î‡‚‡Ú‡, ıËÔÂ‚ÂÌÚËÎ‡ˆËﬂ, ‚ÓÍ‡ÎËÁ‡ˆËﬂ, ‰ËÒÚÓÌË˜ÌË ÔÓÁË, ‡‚ÚÓÏ‡ÚËÁÏË Ë ÛÌËÎ‡ÚÂ‡ÎÌË ÍÓÌ‚ÛÎÒËË. èËÒÚ˙ÔËÚÂ Ò‡ ÔÂ‰ËÏÌÓ ÔÂÁ ‰ÂÌﬂ. óÂÒÚÓÚ‡Ú‡ Ì‡ ÙÂ·- 170 ËÎÌËÚÂ ÔËÒÚ˙ÔË Â ‚ËÒÓÍ‡ ÓÚ 50% (23) ‰Ó 100% (6), Í‡ÚÓ Ú‡ÍË‚‡ ÌÂ ÒÂ ÓÔËÒ‚‡Ú ÒÎÂ‰ 6 „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. àÌÚÂËÍÚ‡ÎÌ‡Ú‡ ÖÖÉ Â ËÎË ÌÓÏ‡ÎÌ‡ ËÎË Ò ÂÔËÎÂÔÚËÙÓÏÂÌË ‡·ÌÓÏÌÓÒÚË ‚ ÚÂÏÔÓ‡ÎÌËÚÂ ÓÚ‚ÂÊ‰‡ÌËﬂ. åêí Ì‡ „Î‡‚ÂÌ ÏÓÁ˙Í Â ·ÂÁ ‰‡ÌÌË Á‡ ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡. èÓ„ÌÓÁ‡Ú‡ Ó·ËÍÌÓ‚ÂÌÓ Â ‰Ó·‡ (20). èÓ‚Â‰ÂÌËﬂÚ „ÂÌÓÏÂÌ ÒÍ‡Ì Ì‡ ÙÂÌÒÍ‡Ú‡ Ù‡ÏËÎËﬂ Ë‰ÂÌÚËÙËˆË‡ ‰‚‡ ‡ÁÎË˜ÌË ÎÓÍÛÒ‡ Ì‡ ıÓÏÓÁÓÏË 18qter Ë 1q25-q31, ·ÂÁ ‰‡ Â ÛÒÚ‡ÌÓ‚ÂÌ ÓÚ„Ó‚ÓÂÌ „ÂÌ. Depondt et al. (2002„) (23) ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒÍ‡˜ÂÌÓÒÚ Ò ıÓÏÓÁÓÏ‡ 12q2223.3. îÄåàãçÄ èÄêñàÄãçÄ ÖèàãÖèëàü ë èêéåÖçãàÇà éÉçàôÄ (FPEVF) íÓ‚‡ Â ÂÔËÎÂÔÚË˜ÂÌ ÒËÌ‰ÓÏ ı‡‡ÍÚÂËÁË‡˘ ÒÂ Ò ‡ÁÎË˜ÂÌ ÚËÔ Ë ÍÓÓ‚‡ ÎÓÍ‡ÎËÁ‡ˆËﬂ Ì‡ Ô‡ˆË‡ÎÌËÚÂ ÔËÒÚ˙ÔË ÔË ÓÚ‰ÂÎÌËÚÂ Ù‡ÏËÎËË, ÔË ÓÚÒ˙ÒÚ‚ËÂÚÓ Ì‡ Í‡Í‚ËÚÓ Ë ‰‡ ·ËÎÓ ÒÚÛÍÚÛÌË ÎÂÁËË ‚ ÏÓÁ˙˜ÌËﬂ Ô‡ÂÌıËÏ (3). èÓÌ‡ÒÚÓﬂ˘ÂÏ ‚ ÎËÚÂ‡ÚÛ‡Ú‡ Ò‡ ÓÔËÒ‡ÌË ÓÍÓÎÓ 10 ÌÂÒ‚˙Á‡ÌË Ù‡ÏËÎËË. èËÒÚ˙ÔËÚÂ Ë ÖÖÉ ÔÓÏÂÌËÚÂ Ò‡ ÔÓÒÚÓﬂÌÌË ‚˙‚ ‚ÂÏÂÚÓ ÔË ‚ÒÂÍË ÓÚ Á‡ÒÂ„Ì‡ÚËÚÂ ˜ÎÂÌÓ‚Â Ì‡ Ù‡ÏËÎËËÚÂ Ë ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÙÓÌÚ‡ÎÌË, ÚÂÏÔÓ‡ÎÌË ËÎË ÓÍˆËÔËÚ‡ÎÌË. éÒ‚ÂÌ ËÁ‡ÁÂÌËÚÂ ËÌÚÂÙ‡ÏËÎÌË ‚‡Ë‡ˆËË ‚ ÍÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡ Ò‡ Ì‡ÎËˆÂ Ë ËÌÚ‡Ù‡ÏËÎÌË ‚‡Ë‡ˆËË, ÍÓÂÚÓ ˜ÂÒÚÓ ‚Ó‰Ë ‰Ó ÔÓ„Â¯Ì‡ ‰Ë‡„ÌÓÁ‡ Ò ‰Û„Ë Ù‡ÏËÎÌË ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË. ìÌ‡ÒÎÂ‰ﬂ‚‡ÌÂÚÓ Â ‡‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÌÓ Ò 70% ÔÂÌÂÚ‡ÌÌÓÒÚ, Í‡ÚÓ ‰‚‡Ú‡ ÔÓÎ‡ ÒÂ Á‡Òﬂ„‡Ú Ò Â‰Ì‡Í‚‡ ˜ÂÒÚÓÚ‡. ç‡˜‡ÎÓ Ì‡ ÔËÒÚ˙ÔËÚÂ ‚‡Ë‡ ÁÌ‡˜ËÚÂÎÌÓ, ÌÓ Ó·ËÍÌÓ‚ÂÌÓ Â ‚ Ô˙‚ËÚÂ ÚË ‰ÂÒÂÚËÎÂÚËﬂ ÓÚ ÊË‚ÓÚ‡, Í‡ÚÓ ÒÂ ÓÙÓÏﬂÚ ‰‚‡ ÔËÍ‡ Ì‡ 5 Ë Ì‡ 25 „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ (67). èËÒÚ˙ÔËÚÂ ÔÓËÁÚË˜‡˘Ë ÓÚ Ó„ÌË˘Â ‚˙‚ ÙÓÌÚ‡ÎÌËﬂ ‰ﬂÎ Ò‡ Ì‡È-˜ÂÒÚÓ ÓÔËÒ‚‡ÌËÚÂ ÔË ÚÓÁË ÒËÌ‰ÓÏ. íÂ ÒÂ ‡ÁÎË˜‡‚‡Ú ÓÚ ÚÂÁË ÔË ADNFLE ÔÓ ÚÓ‚‡, ˜Â ÔÓ-ﬂ‰ÍÓ ÒÂ „ÛÔË‡Ú, ˜ÂÒÚÓÚ‡Ú‡ ËÏ Â ÔÓ-ÌËÒÍ‡, ‚˙ÁÏÓÊÌË Ò‡ Í‡ÍÚÓ ÌÓ˘ÌË Ú‡Í‡ Ë ‰ÌÂ‚ÌË ÔËÒÚ˙ÔË, Í‡ÚÓ ÔÓÒÎÂ‰ÌËÚÂ ÏÓ„‡Ú ‰‡ ÔÂÓ·Î‡‰‡‚‡Ú, ﬂ‰ÍÓ ËÏ‡ ‡Û‡, ˜ÂÒÚ‡ Â ‚ÚÓË˜Ì‡Ú‡ „ÂÌÂ‡ÎËÁ‡ˆËﬂ (11, 67). ÇÚÓË ÔÓ ˜ÂÒÚÓÚ‡ Ò‡ ÔËÒÚ˙ÔËÚÂ ÔÓËÁÚË˜‡˘Ë ÓÚ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ, ‰ÓÍ‡ÚÓ ÚÂÁË Ò ˆÂÌÚÓÔ‡ËÂÚ‡ÎÂÌ Ë ÓÍˆËÔËÚ‡ÎÂÌ ÔÓËÁıÓ‰ Á‡ Â‰ÍË. àÌÚÂËÍÚ‡ÎÌ‡Ú‡ ÖÖÉ ÔÓÍ‡Á‚‡ Ó„ÌË˘ÌË ÂÔËÎÂÔÚËÙÓÏÂÌË ‡·ÌÓÏÌÓÒÚË ÔË ÔÓ‚Â˜ÂÚÓ Ô‡ˆËÂÌÚË, ÎÓÍ‡ÎËÁË‡ÌË ‚ ÚÂÏÔÓ‡ÎÌËÚÂ, ÙÓÌÚ‡ÎÌËÚÂ, ˆÂÌÚÓÚÂÏÔÓ‡ÎÌËÚÂ ËÎË ÓÍˆËÔËÚ‡ÎÌËÚÂ Â„ËÓÌË. èË Á‰‡‚Ë ˜ÎÂÌÓ‚Â Ì‡ Ù‡ÏËÎËËÚÂ Ò˙˘Ó ÏÓ„‡Ú ‰‡ ÒÂ Â„ËÒÚË‡Ú ÖÖÉ ÔÓÏÂÌË, ÍÓÂÚÓ ‚ÂÓﬂÚÌÓ Â ı‡‡ÍÚÂÂÌ Á‡ ÒËÌ‰ÓÏ‡ ·ÂÎÂ„ (53). íÂÊÂÒÚÚ‡ Ì‡ ÖÖÉ ÔÓÏÂÌËÚÂ ‚‡Ë‡ ÏÂÊ‰Û ÓÚ‰ÂÎÌËÚÂ ËÌ‰Ë‚Ë‰Ë Ë ÌÂ ÍÓÂÎË‡ Ò ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÔËÒÚ˙ÔËÚÂ. àÍÚ‡ÎÌ‡Ú‡ ÖÖÉ ÔÓÍ‡Á‚‡ Ó„ÌË˘ÌË ÔÓÏÂÌË. åêí Ì‡ „Î‡‚ÂÌ ÏÓÁ˙Í Ó·ËÍÌÓ‚ÂÌÓ Â ÌÓÏ‡ÎÌ‡. àÏ‡ ËÁ‡ÁÂÌË ËÌÚ‡Ù‡ÏËÎÌË ‚‡Ë‡ˆËË Ë ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÔÓ„ÌÓÁ‡Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. èË Ô˙‚ÓÚÓ ÓÔËÒ‡ÌÓ Ó‰ÓÒÎÓ‚ËÂ Ò ÚÓÁË ÒËÌ‰ÓÏ (‡‚ÒÚ‡ÎËÈÒÍ‡ Ù‡ÏËÎËﬂ) Â Ë‰ÂÌÚËÙËˆË‡Ì ÎÓÍÛÒ Ì‡ ıÓÏÓÁÓÏ‡ 2q (64), ÌÓ ÔÓ‡‰Ë Ï‡ÎÍËﬂ ÏÛ ‡ÁÏÂ ÔÓ-Ì‡Ú‡Ú˙¯ÌË ËÁÒÎÂ‰‚‡ÌËﬂ ÌÂ Ò‡ Ô‡‚ÂÌË. ëÍ‡˜ÂÌÓÒÚ Ò ıÓÏÓÁÓÏ‡ 22q Â ÛÒÚ‡ÌÓ‚ÂÌ‡ ‚ ÔÂÚ Ù‡ÏËÎËË (11,17, 67). íË ÓÚ Ù‡ÏËÎËËÚÂ Ò‡ ÓÚ ÙÂÌÒÍÓ-Í‡Ì‡‰ÒÍË ÔÓËÁıÓ‰ (11, 67) Ë ËÏ‡Ú Â‰ËÌ Ë Ò˙˘ ı‡ÔÎÓÚËÔ, ÍÓÂÚÓ ÔÂ‰ÔÓÎ‡„‡ ◊ÂÙÂÍÚ Ì‡ Ô‡Ó‰ËÚÂÎﬂ”. Ç ÔÓÒÎÂ‰ÒÚ‚ËÂ Ò‡ ÓÔËÒ‡ÌË Â‰Ì‡ ËÒÔ‡ÌÒÍ‡ (11) Ë Â‰Ì‡ ‰‡ÚÒÍ‡ (62) Ù‡ÏËÎËﬂ Ë ÒÍ‡˜ÂÌÓÒÚ Ò˙Ò Ò˙˘Ëﬂ ıÓÏÓÁÓÏÂÌ ÎÓÍÛÒ 22q, ÌÓ Ò ‡ÁÎË˜ÂÌ ı‡ÔÎÓÚËÔ. îÄåàãçÄ éäñàèàíéíÖåèéêÄãçÄ ÖèàãÖèëàü Deprez et al. (2007) (24) ÓÔËÒ‚‡Ú „ÓÎﬂÏ‡ ·ÂÎ„ËÈÒÍ‡ Ù‡ÏËÎËﬂ Ò ÓÍˆËÔËÚÓÚÂÏÔÓ‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ ‡ÒÓˆËË‡Ì‡ Ò ÏË„ÂÌ‡ Ò˙Ò ÁËÚÂÎÌ‡ ‡Û‡. ìÌ‡ÒÎÂ‰ﬂ‚‡ÌÂÚÓ Â ‡‚ÚÓÁÓÏÌÓ-‰ÓÏË- Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 Ì‡ÌÚÓ Ò 75% ÔÂÌÂÚ‡ÌÌÓÒÚ. Ç˙Á‡ÒÚÚ‡ Ì‡ Ì‡˜‡ÎÓ ‚‡Ë‡ ÏÂÊ‰Û 7 ÏÂÒÂˆ‡ Ë 63 „Ó‰ËÌË (ÒÂ‰ÌÓ 21 „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ). èËÒÚ˙ÔËÚÂ Ò‡ ÔÂ‰ËÏÌÓ ‰ÌÂ‚ÌË. éÔËÒ‡ÌË Ò‡ ÔÓÒÚË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË - Ì‡È-˜ÂÒÚÓ Ò˙Ò ÁËÚÂÎÌË ÒËÏÔÚÓÏË (60%), ÍÓ„ÌËÚË‚ÌË ÒËÏÔÚÓÏË (50%), ‡‚ÚÓÌÓÏÌË ÒËÏÔÚÓÏË, ÒÚ‡ı, ÏÓÚÓÌË, ‚ÂÚË„ËÌÓÁÌË Ë ÒÓÏ‡ÚÓÒÂÌÁÓÌË ÒËÏÔÚÓÏË Ë Ó·ÓÌﬂÚÂÎÌË ËÎË ÒÎÛıÓ‚Ë ı‡Î˛ˆËÌ‡ˆËË; ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË Ë ‚ÚÓË˜ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË. ãËÔÒ‚‡ ‡Ì‡ÏÌÂÁ‡ Á‡ ÙÂ·ËÎÌË ÔËÒÚ˙ÔË. èË 50% ÓÚ ˜ÎÂÌÓ‚ÂÚÂ Ì‡ Ù‡ÏËÎËﬂÚ‡ ÓÍˆËÔËÚÓÚÂÏÔÓ‡ÎÌ‡Ú‡ ÂÔËÎÂÔÒËﬂ ÒÂ ‡ÒÓˆËË‡ Ò ÏË„ÂÌ‡ Ò ‡Û‡. ëÂ‰Ì‡Ú‡ ‚˙Á‡ÒÚ Ì‡ Ì‡˜‡ÎÓ Ì‡ ÏË„ÂÌÓÁÌËÚÂ ÔËÒÚ˙ÔË Â 42 „Ó‰ËÌË ( ÏÂÊ‰Û 30 ‰Ó 65 „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ ). ç‡È-˜ÂÒÚ‡Ú‡ ÁËÚÂÎÌ‡ ‡Û‡ ÒÂ ËÁ‡Áﬂ‚‡ ‚ ÔÓﬂ‚‡ Ì‡ ‰‚ÓÈÌÓ ‚ËÊ‰‡ÌÂ, Ò‚ÂÚÍ‡‚ËˆË Ë ÒÍÓÚÓÏ. ëÂ‰Ì‡Ú‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ ‡Û‡Ú‡ Â 20 ÏËÌÛÚË. ÖÔËÎÂÔÚË˜ÌËÚÂ Ë ÏË„ÂÌÓÁÌËÚÂ ÔËÒÚ˙ÔË ÔË ÔÓ‚Â˜ÂÚÓ Á‡ÒÂ„Ì‡ÚË ËÌ‰Ë‚Ë‰Ë ÔÓÚË˜‡Ú ÌÂÁ‡‚ËÒËÏÓ Â‰ÌË ÓÚ ‰Û„Ë ‚˙‚ ‚ÂÏÂÚÓ. çÂ‚ÓÎÓ„Ë˜ÌËﬂ ÒÚ‡ÚÛÒ Ó·ËÍÌÓ‚ÂÌÓ Â ÌÓÏ‡ÎÂÌ. àÌÚÂËÍÚ‡ÎÌ‡Ú‡ ÖÖÉ Ì‡È-˜ÂÒÚÓ Â ÌÓÏ‡ÎÌ‡. åêí ËÁÒÎÂ‰‚‡ÌÂÚÓ Ò˙˘Ó Â ÌÓÏ‡ÎÌÓ. èË ÔÓ‚Â‰ÂÌËﬂ ‡Ì‡ÎËÁ Á‡ ÒÍ‡˜ÂÌÓÒÚ Â Ë‰ÂÌÚËÙËˆË‡Ì ÎÓÍÛÒ Ì‡ ıÓÏÓÁÓÏ‡ 9q21-q22, ÓÚ ÍÓÂÚÓ ÏÓÊÂ ‰‡ ÒÂ ÔÂ‰ÔÓÎÓÊË, ˜Â ÏÛÚ‡ˆËﬂ ‚ Â‰ËÌÒÚ‚ÂÌ Ó·˘ „ÂÌ Â ÓÚ„Ó‚ÓÂÌ Â‰ÌÓ‚ÂÏÂÌÌÓ Á‡ ‰‚ÂÚÂ Á‡·ÓÎﬂ‚‡ÌËﬂ ( ÂÔËÎÂÔÒËﬂ Ë ÏË„ÂÌ‡ ). èÄêñàÄãçÄ ÖèàãÖèëàü ë èÖêàñÖçíêÄãçà ëèÄâäéÇÖ ëËÌ‰ÓÏ˙Ú Â ÓÔËÒ‡Ì Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Kinton Ât al. 2002„. (40) ÔË ·‡ÁËÎÒÍ‡ Ù‡ÏËÎËﬂ. ç‡˜‡ÎÓÚÓ Â ‚ Ô˙‚ËÚÂ ‰‚Â ‰ÂÒÂÚËÎÂÚËﬂ ÓÚ ÊË‚ÓÚ‡, Í‡ÚÓ ÔËÒÚ˙ÔËÚÂ Ó·ËÍÌÓ‚ÂÌÓ ÔÂÒËÒÚË‡Ú ‰Ó 70 „Ó‰. ‚˙Á‡ÒÚ, ‡ ÖÖÉ ÔÓÏÂÌËÚÂ ‰Ó 30 „Ó‰. ‚˙Á‡ÒÚ. èË Á‡ÒÂ„Ì‡ÚËÚÂ ˜ÎÂÌÓ‚Â ÓÚ Ù‡ÏËÎËﬂÚ‡ ÒÂ Ì‡·Î˛‰‡‚‡Ú ‡ÁÎË˜ÌË ‚Ë‰Ó‚Â ÂÔËÎÂÔÚË˜ÌË ÔËÒÚ˙ÔË – ıÂÏËÍÎÓÌË˜ÌË, ıÂÏËÚÓÌË˜ÌË, Éíäè, ÔÓÒÚË ÙÓÍ‡ÎÌË ( ÒÚÂÂÓÚËÔÌË ÂÔËÁÓ‰Ë ÓÚ ÂÔË„‡ÒÚ‡ÎÌ‡ ·ÓÎÍ‡) Ë ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ò˙Ò ÒÂÏËÓÎÓ„Ëﬂ Ò˙ÓÚ‚ÂÚÒÚ‚‡˘‡ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ. ï‡‡ÍÚÂÂÌ ·ÂÎÂ„ Ì‡ ÚÓÁË ÒËÌ‰ÓÏ Ò‡ ÖÖÉ ÔÓÏÂÌËÚÂ ÓÚ ÓÒÚËÂÚ‡ ËÎË ·‡‚ÌË ‚˙ÎÌË ‚ ÔÂËˆÂÌÚ‡ÎÌËÚÂ Â„ËÓÌË (ˆÂÌÚÓÔ‡ËÂÚ‡ÎÌÓ, ˆÂÌÚÓÙÓÌÚ‡ÎÌÓ ËÎË ˆÂÌÚ‡ÚÂÏÔÓ‡ÎÌÓ). ÑË‡„ÌÓÁ‡Ú‡ ˜ÂÒÚÓ ÒÂ Á‡ÚÛ‰Ìﬂ‚‡ ÓÚ ‚‡Ë‡·ËÎÌ‡Ú‡ ÔÂÌÂÚ‡ÌÌÓÒÚ Ë ÍÎËÌË˜Ì‡ Í‡ÚËÌ‡ ÒÂ‰ Á‡ÒÂ„Ì‡ÚËÚÂ ˜ÎÂÌÓ‚Â Ì‡ Ù‡ÏËÎËﬂÚ‡. ïÓ‰˙Ú Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â ·Î‡„ÓÔËﬂÚÂÌ. ìÒÚ‡ÌÓ‚ÂÌ‡ Â ÒÍ‡˜ÂÌÓÒÚ Ò ıÓÏÓÁÓÏ‡ 415. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. áÄäãûóÖçàÖ Ç˙ÔÂÍË, ˜Â „ÓÂÓÔËÒ‡ÌËÚÂ ÌÓ‚Ë ÒËÌ‰ÓÏË ÒÂ ÒÂ˘‡Ú Ò‡‚ÌËÚÂÎÌÓ ﬂ‰ÍÓ, ÔÓÁÌ‡‚‡ÌÂÚÓ ËÏ Ë Ì‡ÒÓ˜ÂÌÓÚÓ Ú˙ÒÂÌÂ Ì‡ Ù‡ÏËÎÌ‡ ‡Ì‡ÏÌÂÁ‡ Â ÓÚ „ÓÎﬂÏÓ ÁÌ‡˜ÂÌËÂ Á‡ ÍÎËÌËˆËÒÚËÚÂ. íÓ‚‡ ˘Â ÛÎÂÒÌË ÔÓÒÚ‡‚ﬂÌÂÚÓ Ì‡ ÚÓ˜Ì‡ ‰Ë‡„ÌÓÁ‡ Ë ˘Â ‰Ó‚Â‰Â ‰Ó ÔÓ‚ÂÊ‰‡ÌÂ Ì‡ ÔÓ‰ıÓ‰ﬂ˘Ó ÎÂ˜ÂÌËÂ, ÓÒË„Ûﬂ‚‡ÌÂ Ì‡ ÔÓ-‰Ó·Ë „ËÊË Á‡ Ô‡ˆËÂÌÚËÚÂ Ë ÔÓ-ÚÓ˜Ì‡ ÔÓ„ÌÓÁ‡ Á‡ ıÓ‰‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. à‰ÂÌÚËÙËˆË‡ÌÂÚÓ Ì‡ „ÂÌËÚÂ, ÓÚ„Ó‚ÓÌË Á‡ ‡ÁÎË˜ÌËÚÂ ÂÔËÎÂÔÚË˜ÌË ÒËÌ‰ÓÏË ˘Â ‰ÓÔËÌÂÒÂ Á‡ ÔÓ-‰Ó·Ó ‡Á·Ë‡ÌÂ Ì‡ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ë ‰Ó ÔÓ-ÂÙÂÍÚË‚ÌÓ, „ÂÌÂÚË˜ÌÓ Ó·ÓÒÌÓ‚‡ÌÓ ÎÂ˜ÂÌËÂ Ì‡ ÂÔËÎÂÔÒËËÚÂ, ‡ Ò˙˘Ó Ë Á‡ ÔÓ-Ì‡Ú‡Ú˙¯ÌÓÚÓ ËÁﬂÒÌﬂ‚‡ÌÂ Ì‡ ÌÓÏ‡ÎÌÓÚÓ ÙÛÌÍˆËÓÌË‡ÌÂ Ì‡ ÏÓÁ˙Í‡. 30. 31. 32. 33. 34. 35. 36. 37. ãàíÖêÄíìêÄ 1. 2. 3. 4. á‡ı‡ËÂ‚, á., É‡ÌÂ‚‡, É ÖÔËÎÂÔÒËﬂ.- ‚ çÂ‚ÓÎÓ„Ëﬂ ÔÓ‰ Â‰‡ÍˆËﬂÚ‡ Ì‡ ÔÓÙ.åËÎ‡ÌÓ‚ Ë ÔÓÙ.üÌ˜Â‚‡. ëÓÙËﬂ: åÂ‰ËˆËÌ‡ Ë ÙËÁÍÛÎÚÛ‡ 2007; 545-546 Abou-Khalil B, Andermann E, Andermann F, et al. 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Seizure 2002; 11:16–19 66. Winawer MR, Ottman R, Hauser WA, Pedley TA. Autosomal dominant partial epilepsy with auditory features: defining the phenotype. Neurology 2000; 54:2173–2176. 67. Xiong L, Labuda M, Li DS, et al. Mapping of a gene determining familial partial epilepsy with variable foci to chromosome 22q11-q12. Am J Hum Genet 1999; 65:1698–710. Ä‰ÂÒ Á‡ ÍÓÂÒÔÓÌ‰ÂÌˆËﬂ: Ñ- ë. ÜÂÎﬂÁÍÓ‚‡ ìåÅÄã ◊ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡” äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, ·ÛÎ. ◊ ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËﬂ 1431 ÚÂÎ. 02/ 92 30 388 e-mail: [email protected] ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 173 é·ÁÓ êÄççà äéÉçàíàÇçà çÄêìòÖçàü èêà ÅéãÖëííÄ çÄ èÄêäàçëéç å. èÂÚÓ‚‡, å. ê‡È˜Â‚‡, ã. í‡ÈÍÓ‚ ìåÅÄã “ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, åÂ‰ËˆËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËﬂ SUMMARY EARLY COGNITIVE CHANGES IN PARKINSON’S DISEASE M. Petrova, M. Raycheva, L. Traykov Parkinson’s disease (PD) is a slowly progressive neurodegenerative disorder mainly characterized by degeneration of dopaminergic neurons in the substantia nigra and the ventral tegmental area, in combination with a varying loss of central noradrenergic, cholinergic and serotonergic integrity, leading to a multitude of motor and non-motor disturbances (such as cognitive impairment and dementia). Studies describing cognitive dysfunction in PD tend to focus on the dementia, although it is important to establish the milder degrees of cognitive impairment, which also impact significantly on quality of life and increase significant the risk of dementia development. Cognitive impairments are often associated with PD, although deficits may be relatively subtle and not clinically apparent, or not overtly affect daily functioning. However, when compared to controls, subtle to prominent cognitive impairment is almost always found in PD patients. A wide variety of cognitive deficits has been described in nondemented PD patients, the most prominent of which is a deficit in executive function. Besides executive dysfunction, there is considerable evidence of deficits in visuospatial functions, constructional abilities, language and memory in nondemented PD. In this article, we review profoundly changes of different cognitive domains in non-demented PD in order to establish the specific profile of these early cognitive changes in PD. KEY WORDS: early cognitive changes, Parkinson’s disease. êÖáûåÖ ÅÓÎÂÒÚÚ‡ Ì‡ è‡ÍËÌÒÓÌ (Åè) Â ·‡‚ÌÓ ÔÓ„ÂÒË‡˘Ó ÌÂ‚Ó‰Â„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎﬂ‚‡ÌÂ, ÍÓÂÚÓ ÒÂ ı‡‡ÍÚÂËÁË‡ ÓÒÌÓ‚ÌÓ Ò ‰Â„ÂÌÂ‡ˆËﬂ Ì‡ ‰ÓÔ‡ÏËÌÂ„Ë˜ÌËÚÂ ÌÂ‚ÓÌË ‚ ÒÛ·ÒÚ‡ÌˆËﬂ ÌË„‡ Ë ‚ÂÌÚ‡ÎÌ‡Ú‡ ÚÂ„ÏÂÌÚ‡ÎÌ‡ Ó·Î‡ÒÚ. ÑÓÔ‡ÏËÌÂ„Ë˜ÌËﬂÚ ‰ÂÙËˆËÚ ÒÂ ÍÓÏ·ËÌË‡ Ë Ò˙Ò Á‡„Û·‡ Ì‡ ÌÓ‡‰ÂÌÂ„Ë˜ÌË, ıÓÎËÌÂ„Ë˜ÌË Ë ÒÂÓÚÓÌËÌÂ„Ë˜ÌË ÔÓÂÍˆËË, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÏÌÓÊÂÒÚ‚Ó ÏÓÚÓÌË Ë ÌÂÏÓÚÓÌË Ì‡Û¯ÂÌËﬂ (Í‡ÚÓ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ Ë ‰ÂÏÂÌˆËﬂ). íÂÌ‰ÂÌˆËﬂÚ‡ ÔË ÔÓÛ˜‚‡ÌËﬂÚ‡, ÓÔËÒ‚‡˘Ë ÍÓ„ÌËÚË‚ÌËÚÂ ‰ÂÙËˆËÚË ÔË Åè, Â ‰‡ ÒÂ ÙÓÍÛÒË‡Ú ÔÂ‰ËÏÌÓ ‚˙ıÛ ‰ÂÏÂÌˆËﬂÚ‡. ë˙˘Ó Ú‡Í‡ Ó·‡˜Â Â ‚‡ÊÌÓ ÛÒÚ‡ÌÓ‚ﬂ‚‡ÌÂÚÓ Ì‡ ÔÓ-ÎÂÍËÚÂ ÒÚÂÔÂÌË Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ ÔË Åè, ÍÓËÚÓ Ò˙˘Ó ÓÍ‡Á‚‡Ú ÁÌ‡˜ËÏÓ ‚ÎËﬂÌËÂ ‚˙ıÛ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ë ÔÓ‚Ë¯‡‚‡Ú ÁÌ‡˜ËÚÂÎÌÓ ËÒÍ‡ ÓÚ ÔÓÒÎÂ‰‚‡˘‡ ‰ÂÏÂÌˆËﬂ. äÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ Ò‡ ˜ÂÒÚÓ ‡ÒÓˆËË‡ÌË Ò Åè, ‚˙ÔÂÍË ˜Â ‰ÂÙËˆËÚËÚÂ ÏÓ„‡Ú ‰‡ Ò‡ ÓÚÌÓÒËÚÂÎÌÓ ÎÂÍË, ‰‡ ÌÂ Ò‡ ÍÎËÌË˜ÌÓ ËÁﬂ‚ÂÌË Ë ‰‡ ÌÂ Á‡Òﬂ„‡Ú ÓÚ˜ÂÚÎË‚Ó ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ ÂÊÂ‰ÌÂ‚ÌËÚÂ ÙÛÌÍˆËË Ì‡ Ô‡ˆËÂÌÚËÚÂ. èË ‚ÒÂ ÚÓ‚‡ ÍÓ„‡ÚÓ ÒÂ Ò‡‚Ìﬂ‚‡Ú Ò ÍÓÌÚÓÎÌË ÎËˆ‡, Ô‡ˆËÂÌÚËÚÂ Ò Åè ÔÓÍ‡Á‚‡Ú ÔÓ˜ÚË ‚ËÌ‡„Ë ÓÚ Â‰‚‡ ‰ÓÎÓ‚ËÏË ‰Ó ËÁ‡ÁÂÌË ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ. òËÓÍ ‰Ë‡Ô‡ÁÓÌ ÓÚ ÍÓ„ÌËÚË‚ÌË ‰ÂÙËˆËÚË Ò‡ ÓÔËÒ‡ÌË ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè, Í‡ÚÓ Ì‡È-ËÁ‡ÁÂÌË ÓÚ Úﬂı Ò‡ ÚÂÁË ‚ Ó·Î‡ÒÚÚ‡ Ì‡ ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË. ç‡Â‰ Ò Ì‡Û¯ÂÌËﬂÚ‡ ‚ ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË, Ì‡ÎËˆÂ Ò‡ Ò˙˘Ó Ë ÒÂËÓÁÌË ‰ÓÍ‡Á‡ÚÂÎÒÚ‚‡ Á‡ ‰ÂÙËˆËÚË ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÙÛÌÍˆËË, ÍÓÌÒÚÛÍÚË‚ÌËÚÂ ÛÏÂÌËﬂ, ÂÁËÍ‡ Ë Ô‡ÏÂÚÚ‡ ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè. Ç Ì‡ÒÚÓﬂ˘‡Ú‡ ÒÚ‡ÚËﬂ ˘Â Ì‡Ô‡‚ËÏ Á‡‰˙Î·Ó˜ÂÌ ÔÂ„ÎÂ‰ Ì‡ ÔÓÏÂÌËÚÂ ‚ ÓÚ‰ÂÎÌËÚÂ ÍÓ„ÌËÚË‚ÌË Ó·Î‡ÒÚË ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè Ò Ó„ÎÂ‰ Ó˜ÂÚ‡‚‡ÌÂÚÓ Ì‡ ÒÔÂˆËÙË˜ÌËﬂ ÔÓÙËÎ Ì‡ ÚÂÁË ‡ÌÌË ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ ÔË Åè. äãûóéÇà Ñìåà: ‡ÌÌË ÍÓ„ÌËÚË‚Ì Ì‡Û¯ÂÌËﬂ, ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ. ÅÓÎÂÒÚÚ‡ Ì‡ è‡ÍËÌÒÓÌ (Åè) Â ·‡‚ÌÓ ÔÓ„ÂÒË‡˘Ó ÌÂ‚Ó‰Â„ÂÌÂ‡ÚË‚ÌÓ Á‡·ÓÎﬂ‚‡ÌÂ, ÍÓÂÚÓ ÒÂ ı‡‡ÍÚÂËÁË‡ ÓÒÌÓ‚ÌÓ Ò ‰Â„ÂÌÂ‡ˆËﬂ Ì‡ ‰ÓÔ‡ÏËÌÂ„Ë˜ÌËÚÂ ÌÂ‚ÓÌË ‚ ÒÛ·ÒÚ‡ÌˆËﬂ ÌË„‡ Ë ‚ÂÌÚ‡ÎÌ‡Ú‡ ÚÂ„ÏÂÌÚ‡ÎÌ‡ Ó·Î‡ÒÚ. ÑÓÔ‡ÏËÌÂ„Ë˜ÌËﬂÚ ‰ÂÙËˆËÚ ÒÂ ÍÓÏ·ËÌË‡ Ë Ò˙Ò Á‡„Û·‡ Ì‡ ÌÓ‡‰ÂÌÂ„Ë˜ÌË (locus coeruleus), ıÓÎËÌÂ„Ë˜ÌË (nucleus basalis of Meynert) Ë ÒÂÓÚÓÌËÌÂ„Ë˜ÌË (dorsal raphe nuclei) ÔÓÂÍˆËË (35), ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ÏÌÓÊÂÒÚ‚Ó ÏÓÚÓÌË Ë ÌÂÏÓÚÓÌË Ì‡Û¯ÂÌËﬂ (Í‡ÚÓ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ Ë ‰ÂÏÂÌˆËﬂ). èÓÛ˜‚‡ÌËﬂÚ‡, ÍÓËÚÓ ËÁÒÎÂ‰‚‡Ú ÍÓ„ÌËÚË‚ÌËÚÂ ÙÛÌÍˆËË ÔË Åè ËÏ‡Ú ÚÂÌ‰ÂÌˆËﬂ ‰‡ ÒÂ ÙÓÍÛÒË‡Ú ‚˙ıÛ ‡Á‚ËÚËÂÚÓ Ì‡ ‰ÂÏÂÌˆËﬂ, ‚˙ÔÂÍË ˜Â Â ‚‡ÊÌÓ ‰‡ ÒÂ ÛÒÚ‡ÌÓ‚ﬂÚ ÔÓ-ÎÂÍËÚÂ ÒÚÂÔÂÌË Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ, ÍÓËÚÓ ÓÒ‚ÂÌ ˜Â ÔÓ‚Ë¯‡‚‡Ú ËÒÍ‡ ÓÚ ÔÓÒÎÂ‰‚‡˘‡ ‰ÂÏÂÌˆËﬂ (34), Ò˙˘Ó ‚ÎËﬂﬂÚ ÁÌ‡˜ËÚÂÎÌÓ ‚˙ıÛ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ (36). äÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ Ò‡ ˜ÂÒÚÓ ‡ÒÓˆËË‡ÌË Ò Åè, ‚˙ÔÂÍË ˜Â ‰ÂÙËˆËÚËÚÂ ÏÓ„‡Ú ‰‡ Ò‡ ÓÚÌÓÒËÚÂÎÌÓ ÎÂÍË, ‰‡ ÌÂ Ò‡ ÍÎËÌË˜ÌÓ ËÁﬂ‚ÂÌË Ë ‰‡ ÌÂ Á‡Òﬂ„‡Ú ÓÚ˜ÂÚÎË‚Ó ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ ÂÊÂ‰ÌÂ‚ÌËÚÂ ÙÛÌÍˆËË Ì‡ Ô‡ˆËÂÌÚËÚÂ. äÓ„‡ÚÓ ÒÂ Ò‡‚Ìﬂ‚‡Ú Ò ÍÓÌÚÓÎÌË ÎËˆ‡, Ô‡ˆËÂÌÚËÚÂ Ò Åè ÔÓÍ‡Á‚‡Ú ˜ÂÒÚÓ ÓÚ Â‰‚‡ ‰ÓÎÓ‚ËÏ ‰Ó ËÁ‡ÁÂÌ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ (14). ç‡Û¯ÂÌËﬂÚ‡ ‚ ‡ÁÎË˜ÌËÚÂ ÍÓ„ÌËÚË‚ÌË Ó·Î‡ÒÚË Í‡ÚÓ ‚ÌËÏ‡ÌËÂ, ÂÍÁÂÍÛÚË‚ÌË ÙÛÌÍˆËË, Ô‡ÏÂÚ, Â˜ Ë ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌË ÛÏÂÌËﬂ, Ò‡ Ó·ÂÍÚ Ì‡ Â‰Ëˆ‡ ÔÓÛ˜‚‡ÌËﬂ ÔË Ô‡ˆËÂÌÚË Ò Åè, ‰ÓË Ë ‚ Ì‡˜‡ÎÌËÚÂ ÒÚ‡‰ËË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, ÌÓ ÂÁÛÎÚ‡ÚËÚÂ ˜ÂÒÚÓ Ò‡ ÔÓÚË‚ÓÂ˜Ë‚Ë (37, 38, 56, 26, 28, 44, 47). Ç Ì‡ÒÚÓﬂ˘‡Ú‡ ÒÚ‡ÚËﬂ ˘Â ·˙‰‡Ú ‡Á„ÎÂ‰‡ÌË ÔÓÓÚ‰ÂÎÌÓ Ì‡Û¯ÂÌËﬂÚ‡ ‚ ÓÚ‰ÂÎÌËÚÂ ÍÓ„ÌËÚË‚ÌË ÒÙÂË ÔË ÌÂ‰ÂÏÂÌÚÌËÚÂ Ô‡ˆËÂÌÚË Ò Åè. 1. çÄêìòÖçàü çÄ ÇçàåÄçàÖíé îÂÌÓÏÂÌËÚÂ, Ò‚˙Á‡ÌË Ò ‚ÌËÏ‡ÌËÂÚÓ, ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ‡Á‰ÂÎÂÌË Ì‡ ‰‚Â „Î‡‚ÌË „ÛÔË. è˙‚‡Ú‡ „ÛÔ‡ ÓÚ„Ó‚‡ﬂ Ì‡ Ú. Ì‡. vigilance Ë ÒÂ ËÁÒÎÂ‰‚‡ Ò ÚÂÒÚÓ‚Â, ‚ÍÎ˛˜‚‡˘Ë ËÁÏÂ‚‡ÌÂ Ì‡ ‚ÂÏÂÚÓ, ÌÂÓ·ıÓ‰ËÏÓ Á‡ Ó·ËÍÌÓ‚ÂÌËÚÂ Â‡ÍˆËË. ÇÚÓ‡Ú‡ ÔÂ‰ÒÚ‡‚Îﬂ‚‡ ÒÂÎÂÍÚË‚ÌÓÚÓ ‚ÌËÏ‡ÌËÂ, ÍÓÂÚÓ ËÏ‡ ‰‚‡ ‡ÒÔÂÍÚ‡ – Ì‡ÒÓ˜ÂÌÓ Ë ‡ÁÔÂ‰ÂÎÂÌÓ ‚ÌËÏ‡ÌËÂ (52). ç‡ÒÓ˜ÂÌÓÚÓ ‚ÌËÏ‡ÌËÂ Â ÓÚ„Ó‚ÓÌÓ Á‡ ËÁ·Ó‡ Ì‡ Â‰ËÌ ËÁÚÓ˜ÌËÍ Ì‡ ËÌÙÓÏ‡ˆËﬂ Ë Ë„ÌÓË‡ÌÂÚÓ Ì‡ ‰Û„ËÚÂ. ê‡ÁÔÂ‰ÂÎÂÌÓÚÓ ‚ÌËÏ‡ÌËÂ ÔÓÏ‡„‡ Á‡ ÛÒÔÂ¯Ì‡Ú‡ Ó·‡- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ·ÓÚÍ‡ Ì‡ ËÌÙÓÏ‡ˆËﬂÚ‡, Ë‰‚‡˘‡ ÓÚ ÔÓ‚Â˜Â ÓÚ Â‰ËÌ ËÁÚÓ˜ÌËÍ. ë˙˘ÂÒÚ‚Û‚‡ Ë ÚÂÚË ÚËÔ – ÔÓ‰‰˙Ê‡˘Ó ‚ÌËÏ‡ÌËÂ (ÌÂÓ·ıÓ‰ËÏÓÒÚÚ‡ ‰‡ ÒÂ Â‡„Ë‡ ‚ÒÂÍË Ô˙Ú Ì‡ ﬂ‰ÍÓ ÒÎÛ˜‚‡˘Ë ÒÂ Ò˙·ËÚËﬂ). ê‡ÁÔÂ‰ÂÎÂÌÓÚÓ ‚ÌËÏ‡ÌËÂ Ë ‡·ÓÚÌ‡Ú‡ Ô‡ÏÂÚ ÌÂ ·Ë Úﬂ·‚‡ÎÓ ‰‡ ·˙‰‡Ú ‚˙ÁÔËÂÏ‡ÌË Í‡ÚÓ Â‰ËÌÌË ÒËÒÚÂÏË Ò ÙËÍÒË‡Ì Í‡Ô‡ˆËÚÂÚ. Ö‰ËÌ ÏÓ‰ÂÎ Á‡ ÍÓÌˆÂÔÚÛ‡ÎËÁ‡ˆËﬂ Ì‡ ‡·ÓÚÌ‡Ú‡ Ô‡ÏÂÚ Â ÚËÍÓÏÔÓÌÂÌÚÌ‡Ú‡ ÒËÒÚÂÏ‡ (9). íﬂ Ó·ı‚‡˘‡˘‡ ÙÓÌÓÎÓ„Ë˜ÌËﬂ Í˙„ Á‡ ÔÓ‰‰˙Ê‡ÌÂ Ì‡ ‚Â·‡ÎÌ‡Ú‡ ËÌÙÓÏ‡ˆËﬂ, (sketchpad) ÔÓ‰ÒËÒÚÂÏ‡Ú‡ Á‡ ÔÓ‰‰˙Ê‡ÌÂ Ì‡ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌ‡Ú‡ ËÌÙÓÏ‡ˆËﬂ (7) Ë ˆÂÌÚ‡ÎÌ‡Ú‡ ÂÍÁÂÍÛÚË‚Ì‡ ÒËÒÚÂÏ‡ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ (central executive attentional system), ÍÓﬂÚÓ ÍÓÓ‰ËÌË‡ ‰‚ÂÚÂ ÔÓ‰‰˙Ê‡˘Ë ÒËÒÚÂÏË ˜ÂÁ Ó·ÌÓ‚ﬂ‚‡ÌÂ Ì‡ ÒÍÎ‡‰Ë‡˘Ëﬂ ·ÛÙÂ (storage buffer), ÍÓ„‡ÚÓ Â Ì‡ÎËˆÂ ÌÓ‚‡ Ò˙ÓÚ‚ÂÚÒÚ‚‡˘‡ ËÌÙÓÏ‡ˆËﬂ (8, 50). ç‡ÒÍÓÓ ·Â¯Â ‰Ó·‡‚ÂÌ ˜ÂÚ‚˙ÚË ÍÓÏÔÓÌÂÌÚ, ÂÔËÁÓ‰Ë˜ÂÌ ·ÛÙÂ, ÍÓÈÚÓ ËÌÚÂ„Ë‡ ËÌÙÓÏ‡ˆËﬂÚ‡ ÓÚ ‡ÁÎË˜ÌË ÏÓ‰‡ÎÌÓÒÚË Ë ÓÒË„Ûﬂ‚‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ÔÓ‰ÒËÒÚÂÏËÚÂ Ë ‰˙Î„ÓÒÓ˜Ì‡Ú‡ Ô‡ÏÂÚ (6, 8). èË Ô‡ˆËÂÌÚË Ò Åè, ËÁÔÓÎÁ‚‡ÈÍË ÚÓÁË ˜ÂÚËËÍÓÏÔÓÌÂÌÚÂÌ ÏÓ‰ÂÎ Ì‡ ‡·ÓÚÌ‡Ú‡ Ô‡ÏÂÚ, ÒÂ Ò˙Ó·˘‡‚‡ Á‡ Â‰ÛˆË‡ÌÂ Ì‡ ÂÙÂÍÚË‚ÌÓÒÚÚ‡ Ì‡ ˆÂÌÚ‡ÎÌËﬂ ÂÍÁÂÍÛÚË‚ÂÌ Ë ÂÔËÁÓ‰Ë˜ÂÌ ·ÛÙÂ ÔË Ò˙ı‡ÌÂÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ Ì‡ ‚Â·‡ÎÌËﬂ Ë ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËﬂ ·ÛÙÂ (2). åÓÁ˙˜ÌË ÒÚÛÍÚÛË, ÍÓËÚÓ ‚ÁËÏ‡Ú Û˜‡ÒÚËÂ ‚ ÔÓˆÂÒËÚÂ Ì‡ ‚ÌËÏ‡ÌËÂ, Ò‡ ˜ÂÎÌËÚÂ Ë Ô‡ËÂÚ‡ÎÌËÚÂ ÍÓÓ‚Ë ÁÓÌË, ËÌÚ‡Î‡ÏËÌ‡ÌËÚÂ Ë ÂÚËÍÛÎ‡ÌË ﬂ‰‡ Ì‡ Ú‡Î‡ÏÛÒ‡ Ë ÏÓÁ˙˜ÌËﬂ ÒÚ‚ÓÎ (‡ÒˆÂÌ‰ÂÌÚÌ‡Ú‡ ‡ÍÚË‚Ë‡˘‡ ÂÚËÍÛÎ‡Ì‡ ÙÓÏ‡ˆËﬂ Ë ÔËÎÂÊ‡˘ËÚÂ ﬂ‰‡) (46). ç‡Û¯ÂÌËﬂÚ‡ ‚˙‚ ‚ÌËÏ‡ÌËÂÚÓ Ò‡ ˜ÂÒÚÓ ÒÂ˘‡ÌË ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè ‚ÍÎ˛˜ËÚÂÎÌÓ Ë ‚ Ì‡È-‡ÌÌËÚÂ ÒÚ‡‰ËË Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ‰ÂÙËˆËÚ (41). Ç Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎÌË Á‰‡‚Ë ÎËˆ‡ Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË ÔÓ-„ÓÎÂÏË ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ë ÌÂÔÓÒÚÓﬂÌÒÚ‚Ó ‚ Â‡ÍˆËÓÌÌËÚÂ ‚ÂÏÂÌ‡ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè (18, 19, 24, 48). ç‡·Î˛‰‡‚‡ÌÓ Â Ò˙˘Ó, ˜Â Ô‡ˆËÂÌÚËÚÂ ‚ Ì‡ÔÂ‰Ì‡ÎËÚÂ ÒÚ‡‰ËË Ì‡ Åè Ò‡ ÔÓ-·‡‚ÌË Ë Ò ÔÓ„ÓÎﬂÏ‡ ‚‡Ë‡·ËÎÌÓÒÚ ‚ ËÁÔ˙ÎÌÂÌËﬂÚ‡ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚÂ ‚ ÎÂÍËÚÂ ÒÚ‡‰ËË Ì‡ ·ÓÎÂÒÚÚ‡ (24). èË ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ÌÂÎÂÍÛ‚‡ÌË Ô‡ˆËÂÌÚË Ò Åè ÌÂ ÒÂ ÓÚÍË‚‡Ú ÍÓÂÎ‡ˆËË ÏÂÊ‰Û ËÁÏÂÂÌËÚÂ Â‡ÍˆËÓÌÌË ‚ÂÏÂÌ‡ Ë ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ËÁÒÎÂ‰‚‡ÌËﬂÚ‡ Ì‡ „ÎÓ·‡ÎÌËÚÂ ÏÓÚÓÌË Ë ÍÓ„ÌËÚË‚ÌË ÙÛÌÍˆËË, ÔÂ‰ÔÓÎ‡„‡ÈÍË, ˜Â ÚÂÁË Ì‡Û¯ÂÌËﬂ ·Ëı‡ ÏÓ„ÎË ‰‡ Ò‡ ÓÚ‰ÂÎÂÌ Ï‡ÍÂ ÔË Åè (19). ÑÂÙËˆËÚËÚÂ ‚˙‚ ‚ÌËÏ‡ÌËÂÚÓ Ò‡ Ò‚˙Á‡ÌË ËÁ„ÎÂÊ‰‡ Ò Ì‡Û¯ÂÌËﬂÚ‡ ‚ ÔÓıÓ‰Í‡Ú‡ Ë ‡‚ÌÓ‚ÂÒËÂÚÓ. èË ÓÚ‚ÎË˜‡ÌÂ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ Ì‡ Åè Ô‡ˆËÂÌÚË Ò Ô‡‰‡ÌËﬂ Â ÛÒÚ‡ÌÓ‚ÂÌÓ Á‡ÒËÎ‚‡ÌÂ Ì‡ ÔÓÒÚÛ‡ÎÌ‡Ú‡ ËÏ ÌÂÒÚ‡·ËÎÌÓÒÚ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚË ·ÂÁ Ô‡‰‡ÌËﬂ (3), Í‡ÍÚÓ Ë Á‡ÒËÎ‚‡ÌÂ Ì‡ ÙËÈÁËÌ„ ÙÂÌÓÏÂÌ‡ Ë Á‡„Û·‡ Ì‡ ‡‚ÌÓ‚ÂÒËÂ ÔË ıÓ‰ÂÌÂ (42). ë˙Ó·˘‡‚‡ ÒÂ, ˜Â Åè Ô‡ˆËÂÌÚËÚÂ Ò ËÁ‡ÁÂÌ‡ ÔÓÒÚÛ‡ÎÌ‡ ÌÂÒÚ‡·ËÎÌÓÒÚ Ë Ì‡Û¯ÂÌËﬂ ‚˙‚ ‚ÌËÏ‡ÌËÂÚÓ Ò‡ Ò ÔÓ‚Ë¯ÂÌ‡ ˜ÂÒÚÓÚ‡ Ì‡ Ô‡‰‡ÌËﬂÚ‡ (1). 2. çÄêìòÖçàü çÄ ÖäáÖäìíàÇçàíÖ îìçäñàà ÖÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË Ó·ı‚‡˘‡Ú Ò˙‚ÍÛÔÌÓÒÚ ÓÚ ÍÓ„ÌËÚË‚ÌË ÔÓˆÂÒË, ÌÂÓ·ıÓ‰ËÏË Á‡ ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ ÒÎÂ‰ÌËÚÂ Á‡‰‡˜Ë: ËÁ‡·ÓÚ‚‡ÌÂ Ì‡ ÔÎ‡Ì, ‚ÁËÏ‡ÌÂ Ì‡ Â¯ÂÌËÂ, ÔÂˆÂÌÍ‡ Ë ‡‚ÚÓÔÂˆÂÔˆËﬂ (ËÎË feedback) (17, 57). èËÂÏ‡ ÒÂ, ˜Â Ì‡Û¯ÂÌËﬂÚ‡ ‚ ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË Ò‡ ‚ ÓÒÌÓ‚‡Ú‡ Ì‡ ÔÓ-„ÓÎﬂÏ‡Ú‡ ˜‡ÒÚ ÓÚ ÍÓ„ÌËÚË‚ÌËÚÂ ÔÓÏÂÌË ÔË Ô‡ˆËÂÌÚË Ò Åè. çÂ‰ÂÏÂÌÚÌËÚÂ Ô‡ˆËÂÌÚË Ò Åè ËÁÔ˙ÎÌﬂ‚‡Ú ÔÓ-ÎÓ¯Ó Á‡‰‡˜Ë, ÍÓËÚÓ ËÁËÒÍ‚‡Ú „ÂÌÂË‡ÌÂÚÓ Ì‡ Ô‡ÏÂÚÓ‚ ÒÂÚ, ‡‰ÂÍ‚‡ÚÌÓ ÔÎ‡ÌË‡ÌÂ Ë ÍÓ„ÌËÚË‚ÌÓ ÔÓÒÎÂ‰ﬂ‚‡ÌÂ (16, 26). Ö‰ËÌ ˜ÂÒÚÓ Ò˙Ó·˘‡‚‡Ì ‰ÂÙËˆËÚ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ Â ‚ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ ÔÂ‚ÍÎ˛˜‚‡ÌÂ 174 ÓÚ Â‰ËÌ ÔÎ‡Ì Í˙Ï ‰Û„, Ú.Â. ÒÔÓÒÓ·ÌÓÒÚÚ‡ ‰‡ ÒÂ ÔÓÏÂÌﬂ ÔÓ‚Â‰ÂÌËÂÚÓ Ò˙Ó·‡ÁÌÓ ÔÓÏﬂÌ‡Ú‡ ‚ Ò˙ÓÚ‚ÂÚÌËﬂ ÒÚËÏÛÎ. é·‡˜Â ÔË˜ËÌËÚÂ Á‡ ÚÂÁË Ì‡Û¯ÂÌËﬂ ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ ÌÂ Ò‡ Ì‡Ô˙ÎÌÓ ËÁﬂÒÌÂÌË. Ö‰ÌË ‡‚ÚÓË Ì‡ÏË‡Ú, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò Åè ËÏ‡Ú Ì‡Û¯ÂÌËÂ ‚ ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ ÔÂ‚ÍÎ˛˜‚‡ÌÂ, ÌÓ Ò‡ÏÓ ÍÓ„‡ÚÓ Ò‡ ÌÂÓ·ıÓ‰ËÏË ‚˙ÚÂ¯ÌÓ „ÂÌÂË‡ÌÂ ËÎË ‚˙ÚÂ¯ÂÌ ÍÓÌÚÓÎ, Á‡„‡Ú‚‡ÈÍË Á‡‚ËÒËÏÓÒÚÚ‡ ÓÚ ÏÂı‡ÌËÁÏËÚÂ Ì‡ ‡·ÓÚÌ‡Ú‡ Ô‡ÏÂÚ (20, 54). ÑÛ„Ë ‡‚ÚÓË ÓÚ Ò‚Óﬂ ÒÚ‡Ì‡ ÔÓÍ‡Á‚‡Ú, ˜Â ‰ÂÙËˆËÚËÚÂ ‚ ÔÂ‚ÍÎ˛˜‚‡ÌÂÚÓ Ò˙˘Ó ÏÓ„‡Ú ‰‡ ÒÂ Ì‡·Î˛‰‡‚‡Ú Ë ÔË ‚˙Ì¯ÂÌ ÍÓÌÚÓÎ Ì‡ ÔÂ‚ÍÎ˛˜‚‡ÌÂÚÓ, ÍÓÂÚÓ ‰‡ÒÚË˜ÌÓ Ì‡Ï‡Îﬂ‚‡ ‚ÎËﬂÌËÂÚÓ Ì‡ ‡·ÓÚÌ‡Ú‡ Ô‡ÏÂÚ (21). íÂ ÓÚÍË‚‡Ú Ò˙˘Ó Ú‡Í‡, ˜Â ‰ÂÙËˆËÚ˙Ú ‚ ÔÂ‚ÍÎ˛˜‚‡ÌÂÚÓ ÔË Ô‡ˆËÂÌÚËÚÂ ÒÂ Ì‡·Î˛‰‡‚‡ Ò‡ÏÓ ‚ ÚÂÁË ÔÓ‰Á‡‰‡˜Ë, ÔË ÍÓËÚÓ ÒÂ ËÁËÒÍ‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ì‡ÎË˜ËÂÚÓ Ì‡ ÒÂÎÂÍÚË‚ÌË ÏÂı‡ÌËÁÏË, ÌÂÓ·ıÓ‰ËÏË Á‡ ÔÂ‚ÍÎ˛˜‚‡ÌÂÚÓ ‚ ÛÒÎÓ‚ËﬂÚ‡ Ì‡ ‡ÁÒÂÈ‚‡ÌÂ. êÂ‰Ëˆ‡ ÔÓÛ˜‚‡ÌËﬂ Ò˙˘Ó Ò˙Ó·˘‡‚‡Ú Á‡ ËÁ‡ÁÂÌË Ì‡Û¯ÂÌËﬂ ‚ ÚÂÒÚÓ‚ÂÚÂ, ÓˆÂÌﬂ‚‡˘Ë ÒÔÓÒÓ·ÌÓÒÚÚ‡ Á‡ ËÌıË·Ë‡ÌÂ Ì‡ ÛÚËÌÌËÚÂ Ë ÌÂ‡‰ÂÍ‚‡ÚÌË Á‡ ÍÓÌÍÂÚÌ‡ ÒËÚÛ‡ˆËﬂ ‡ÁÒÂÈ‚‡˘Ë ÒÚËÏÛÎ‡ˆËË Ë Ò˙ÓÚ‚ÂÚÌËÚÂ ‡‚ÚÓÏ‡ÚËÁË‡ÌË ÓÚ„Ó‚ÓË ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè (60, 61). çﬂÍÓË ‡‚ÚÓË Ò˙Ó·˘‡‚‡Ú Ò˙˘Ó, ˜Â ‰ÂÙËˆËÚËÚÂ ‚ ÚÂÁË ÚÂÒÚÓ‚Â Ò‡ Ó·‚˙Á‡ÌË Ò ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ÔÓÒÎÂ‰‚‡˘ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ Ë ‰ÂÏÂÌˆËﬂ (40, 62) ç‡Û¯ÂÌËﬂÚ‡ ‚˙‚ ‚Â·‡ÎÌ‡Ú‡ ÙÎÛË‰ÌÓÒÚ, ÍÓËÚÓ ÓÚ˜‡ÒÚË ÓÚ‡Áﬂ‚‡Ú ‰ÂÙËˆËÚËÚÂ ‚ ÂÍÁÂÍÛÚË‚ÌÓÚÓ Ú˙ÒÂÌÂ Ë ÔÓˆÂÒËÚÂ Ì‡ ËÁ‚ÎË˜‡ÌÂ, Ò‡ Ò˙˘Ó ‰Ó·Â ÓÔËÒ‡ÌË ÔË Åè. èÓ-ÒÍÓÓ ÎËÚÂ‡ÎÌ‡Ú‡ ÓÚÍÓÎÍÓÚÓ ÒÂÏ‡ÌÚË˜Ì‡Ú‡ ÙÎÛË‰ÌÓÒÚ Â ÚËÔË˜ÌÓ Ì‡Û¯ÂÌ‡ ÔË Åè (5, 21, 27, 40), Ï‡Í‡ Ë ÚÂÁË ‰‡ÌÌË ‰‡ ÌÂ Ò‡ ÒËÒÚÂÏÌÓ Ì‡·Î˛‰‡‚‡ÌË (30, 43). ç‡ÎËˆÂ Â ËÁ‚ÂÒÚÌÓ ÔËÔÓÍË‚‡ÌÂ ÓÚ „ÎÂ‰Ì‡ ÚÓ˜Í‡ Ì‡ ÔÓ‰ÎÂÊ‡˘‡Ú‡ ÌÂ‚Ó·ËÓÎÓ„Ë˜Ì‡ ·‡Á‡ Ì‡ ‰ÂÙËˆËÚËÚÂ ‚ ÎËÚÂ‡ÎÌ‡ Ë ÒÂÏ‡ÌÚË˜Ì‡ ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ, ËÏ‡ÈÍË ÔÂ‰‚Ë‰, ˜Â Ë ‰‚‡Ú‡ ÚËÔ‡ ÙÎÛË‰ÌÓÒÚË ‡Á˜ËÚ‡Ú Ì‡ ÙÓÌÚ‡ÎÌÓ ·‡ÁË‡ÌË ÂÍÁÂÍÛÚË‚ÌË ÒÚ‡ÚÂ„ËË. 3. çÄêìòÖçàü çÄ èÄåÖííÄ Ö‰ÌÓ ÓÚ Ì‡È-ÒÚ‡ËÚÂ ‡Á‰ÂÎÂÌËﬂ Ì‡ Ô‡ÏÂÚÚ‡ Â ÚÓ‚‡ Ì‡ Ô˙‚Ë˜Ì‡ ËÎË Í‡ÚÍÓÒÓ˜Ì‡ Ô‡ÏÂÚ (äëè) Ë ‚ÚÓË˜Ì‡ ËÎË ‰˙Î„ÓÒÓ˜Ì‡ Ô‡ÏÂÚ (Ñëè). äëè Â ÒËÒÚÂÏ‡ Ò˙Ò Ò‡ÏÓÒÚÓﬂÚÂÎÂÌ ‡Ì‡ÚÓÏË˜ÂÌ ÒÛ·ÒÚ‡Ú, ÍÓﬂÚÓ Ò˙ÁÌ‡ÚÂÎÌÓ Ò˙ı‡Ìﬂ‚‡ ËÌÙÓÏ‡ˆËﬂÚ‡ Á‡ Ó„‡ÌË˜ÂÌÓ ‚ÂÏÂ (9). Ñëè ÔÂ‰ÒÚ‡‚Îﬂ‚‡ ÒËÒÚÂÏ‡ ÓÚ ÔÓˆÂÒË, ÔÓÁ‚ÓÎﬂ‚‡˘Ë ÔÓ-‰˙Î„ÓÚ‡ÈÌÓÚÓ ÒÍÎ‡‰Ë‡ÌÂ Ì‡ ËÌÙÓÏ‡ˆËﬂ, Í‡ÍÚÓ Ë ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÔÂ‰Ë¯ÌË ÁÌ‡ÌËﬂ (58). Ö‰ÌÓ ÓÚ Ô˙‚ËÚÂ ÙÛÌ‰‡ÏÂÌÚ‡ÎÌË ‡Á‰ÂÎÂÌËﬂ Ì‡ Ñëè ÔÓÚË‚ÓÔÓÒÚ‡‚ﬂ ÂÔËÁÓ‰Ë˜Ì‡Ú‡ Ì‡ ÒÂÏ‡ÌÚË˜Ì‡ Ô‡ÏÂÚ. ÖÔËÁÓ‰Ë˜Ì‡Ú‡ Ô‡ÏÂÚ Í‡Ò‡Â Ô‡ÏÂÚÚ‡ Á‡ Ò˙·ËÚËﬂÚ‡, ÍÓËÚÓ ÏÓ„‡Ú ‰‡ ·˙‰‡Ú ÒËÚÛË‡ÌË ‚ ÒÚÓ„Ó ÓÔÂ‰ÂÎÂÌ ÔÓÒÚ‡ÌÒÚ‚ÂÌ Ë ‚ÂÏÂ‚Ë ÍÓÌÚÂÍÒÚ. ëÂÏ‡ÌÚË˜Ì‡Ú‡ Ô‡ÏÂÚ Â Ô‡ÏÂÚÚ‡ Á‡ Ù‡ÍÚËÚÂ, Ô‡‚ËÎ‡Ú‡ Ë ÍÓÌˆÂÔˆËËÚÂ. Ç ÎËÚÂ‡ÚÛ‡Ú‡ ÒÂ Ò˙Ó·˘‡‚‡Ú Â‰Ëˆ‡ ‰‡ÌÌË, ˜Â Ì‡Û¯ÂÌËﬂÚ‡ ‚ ÂÔËÁÓ‰Ë˜Ì‡Ú‡ Ô‡ÏÂÚ, ‚ÍÎ˛˜‚‡˘Ë ‰ÂÙËˆËÚË ‚ ËÁ‚ÎË˜‡ÌÂÚÓ, Ò‡ ÚÓÎÍÓ‚‡ ˜ÂÒÚË, ÍÓÎÍÓÚÓ Ë ÚÂÁË ‚˙‚ ‚ÌËÏ‡ÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË Ó˘Â ‚ Ì‡˜‡ÎÌËﬂ ÒÚ‡‰ËÈ Ì‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ ÔË Ô‡ˆËÂÌÚË Ò Åè (41). èË ÌÂ‰ÂÏÂÌÚÌËÚÂ Ô‡ˆËÂÌÚË Ó·Ë˜‡ÈÌÓ ÒÂ Ì‡·Î˛‰‡‚‡ ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-ÒÎ‡·Ó Á‡Òﬂ„‡ÌÂ Ì‡ Á‡Û˜‡‚‡ÌÂÚÓ Ì‡ ÌÓ‚‡ ËÌÙÓÏ‡ˆËﬂ Ë ‡ÁÔÓÁÌ‡‚‡ÌÂÚÓ Ì‡ ‚Â˜Â Á‡Û˜ÂÌ‡ Ú‡Í‡‚‡, ÍÓÂÚÓ ÔÓ‰ÒÍ‡Á‚‡ ‰ÂÙËˆËÚ ‚ „ÂÌÂË‡ÌÂÚÓ Ì‡ ÍÓ‰Ë‡˘Ë Ë ËÁ‚ÎË˜‡˘Ë ÒÚ‡ÚÂ„ËË Ò ÓÚÌÓÒËÚÂÎÌÓ Á‡Ô‡Á‚‡ÌÂ Ì‡ ÒÍÎ‡‰Ë‡Ì‡Ú‡ ËÌÙÓÏ‡ˆËﬂ, ı‡‡ÍÚÂÌÓ Á‡ ÔÓ‰ÍÓÓ‚Ëﬂ ÚËÔ Ô‡ÏÂÚÓ‚Ë Ì‡Û¯ÂÌËﬂ (54, 55). ç‡Û¯ÂÌËﬂ ‚˙‚ ‚Â·‡ÎÌ‡Ú‡ ÙÎÛË‰ÌÓÒÚ, ÍÓËÚÓ Ò‡ Ò˙˘Ó ·ÂÎÂ„ Á‡ ÒÂÏ‡ÌÚË˜ÌÓ Á‡Òﬂ„‡ÌÂ Ò‡ ‰Ó·Â ÓÔËÒ‡ÌË ÔË Åè (30) Ë Â‰Ëˆ‡ ‡‚ÚÓË Ë‰ÂÌ- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÚËÙËˆË‡Ú ‰ÂÙËˆËÚËÚÂ ‚˙‚ ‚Â·‡ÎÌ‡Ú‡ ÙÎÛË‰ÌÓÒÚ Í‡ÚÓ ÔÂ‰ËÍÚÓË Á‡ ÔÓÒÎÂ‰‚‡˘‡ ÔÓﬂ‚‡ Ì‡ ‰ÂÏÂÌˆËﬂ (32, 39, 40). ç‡ÒÍÓÓ ÔÓ‚Â‰ÂÌ ÏÂÚ‡-‡Ì‡ÎËÁ ‰ÂÏÓÌÒÚË‡, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò Åè ÔÓÍ‡Á‚‡Ú ÏÌÓ„Ó ÔÓ-„ÓÎÂÏË Ì‡Û¯ÂÌËﬂ ‚ ÚÂÒÚÓ‚ÂÚÂ Á‡ ÒÂÏ‡ÌÚË˜Ì‡, ÓÚÍÓÎÍÓÚÓ Á‡ ÎËÚÂ‡ÎÌ‡ ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ, ÍÓÂÚÓ ÔÂ‰ÔÓÎ‡„‡, ˜Â Ô‡ÚÓÎÓ„ËﬂÚ‡ ‚ ÚÂÏÔÓ‡ÎÌËﬂ ÎÓ· ÏÓÊÂ ‰‡ ‰ÓÔËÌ‡Òﬂ Á‡ Ì‡·Î˛‰‡‚‡ÌËÚÂ Ì‡Û¯ÂÌËﬂ ‚˙‚ ÙÎÛË‰ÌÓÒÚÚ‡ (30). Ç ÔÓ‰ÍÂÔ‡ Ì‡ ‰‡ÌÌËÚÂ Á‡ ‡ÌÌË Ô‡ÏÂÚÓ‚Ë ‰ÂÙËˆËÚË ÔË Åè, Ò‡ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÔÓ‚Â‰ÂÌË ÔÂÁ ÔÓÒÎÂ‰ÌËÚÂ „Ó‰ËÌË ÌÂ‚ÓËÁÓ·‡Áﬂ‚‡˘Ë Ë ÌÂ‚ÓÔ‡ÚÓÎÓ„Ë˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ. ë˙Ó·˘‡‚‡ ÒÂ Á‡ ËÁ‡ÁÂÌË ‰ËÙÛÁÌË ıÓÎËÌÂ„Ë˜ÌË ‰ÂÙËˆËÚË ÔË Åè (12), Í‡ÍÚÓ Ë Á‡ Ì‡ÎË˜ËÂÚÓ Ì‡ ÛÒÚ‡ÌÓ‚ÂÌË Ô‡ÚÓÎÓ„Ë˜ÌË ÔÓÏÂÌË ‚˙‚ ÙÓÌÚ‡ÎÌ‡Ú‡ ·‡Á‡ÎÌ‡ ÍÓ‡ Ë ‚ ıËÔÓÍ‡ÏÔ‡ÎÌ‡Ú‡ ÙÓÏ‡ˆËﬂ ÓÚÌÓÒËÚÂÎÌÓ ‡ÌÓ ‚ ıÓ‰‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (15). 4. çÄêìòÖçàü çÄ êÖóíÄ à ÖáàäÄ èÓ‚Â˜ÂÚÓ ÓÚ ‡‚ÚÓËÚÂ Ò‡ Â‰ËÌÓ‰Û¯ÌË, ˜Â Ì‡Û¯ÂÌËﬂÚ‡ ‚ Â˜Ú‡ ÌÂ Ò‡ ÓÒÓ·ÂÌÓ ı‡‡ÍÚÂÌË Á‡ ÔÂ‰ÂÏÂÌÚÌËﬂ ÒÚ‡‰ËÈ Ì‡ Åè (11, 23, 29). èË ‚ÒÂ ÚÓ‚‡ Ó·‡˜Â Ì‡Û¯ÂÌËﬂ ‚ ÒÂÏ‡ÌÚË˜Ì‡Ú‡ ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ (4), ÙÎÛË‰ÌÓÒÚÚ‡ Á‡ Ì‡ÁÓ‚‡‚‡ÌÂ Ì‡ „Î‡„ÓÎË (45) Ë ‡ÎÚÂÌ‡ÚË‚Ì‡Ú‡ ÙÎÛË‰ÌÓÒÚ (22, 25) Ò‡ Ò˙Ó·˘‡‚‡ÌË ÔË Ô‡ˆËÂÌÚË Ò Åè ‚ ÌﬂÍÓË ÓÚ ÔÓÛ˜‚‡ÌËﬂÚ‡. Lee and Smith (37) Ì‡ÏË‡Ú, ˜Â Ô‡ˆËÂÌÚËÚÂ Ò ‡ÌÌ‡ Åè ‰‡‚‡Ú Ì‡ Á‡‰‡˜‡Ú‡ Á‡ ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ ÔÓ-„ÓÎﬂÏ ·ÓÈ ÔÓ‚Ú‡ﬂ˘Ë ÒÂ ËÌÚÛÁËË (ËÁÎË¯ÌË ‰ÛÏË) ‚ Ò‡‚ÌÂÌËÂ Ò ÍÓÌÚÓÎÌ‡ „ÛÔ‡ Á‰‡‚Ë ÎËˆ‡. Ä‚ÚÓËÚÂ ÔÂ‰ÔÓÎ‡„‡Ú, ˜Â ÚÂÁË ÂÁÛÎÚ‡ÚË ÒÂ ‰˙ÎÊ‡Ú Ì‡ ÚÛ‰ÌÓÒÚËÚÂ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ò Åè ‚ ÔÂ‚ÍÎ˛˜‚‡ÌÂÚÓ ÏÂÊ‰Û Í‡ÚÂ„ÓËËÚÂ ÔË Ì‡ÎË˜ËÂ Ì‡ ‚ÂÏÂ‚Ó Ó„‡ÌË˜ÂÌËÂ. éÚ Ò‚Óﬂ ÒÚ‡Ì‡ Cooper Ë Ò˙Ú. (22), ËÁÔÓÎÁ‚‡ÈÍË ÚÂÒÚÓ‚Â Á‡ Ì‡ÁÓ‚‡‚‡ÌÂ, ‡Á·Ë‡ÌÂ, ÂÁËÍÓ‚‡ ÂÍÒÔÂÒËﬂ Ë ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ (ÒÂÏ‡ÌÚË˜Ì‡ Ë ‡ÎÚÂÌ‡ÚË‚Ì‡), ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú Ì‡ÎË˜ËÂÚÓ Ò‡ÏÓ Ì‡ ÎÂÍË Ì‡Û¯ÂÌËﬂ ‚ ÂÁËÍÓ‚‡Ú‡ ÂÍÒÔÂÒËﬂ Ë ‚ ÌﬂÍÓË ÓÚ Á‡‰‡˜ËÚÂ Á‡ ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ ÔË ËÁÒÎÂ‰‚‡ÌËÚÂ ÓÚ Úﬂı ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè. Cummings Ë Ò˙Ú. (23) Ò‡‚Ìﬂ‚‡Ú „Ó‚ÓÌËÚÂ Ë ÂÁËÍÓ‚Ë Ì‡Û¯ÂÌËﬂ ÔË Ô‡ˆËÂÌÚË Ò Åè Ò Ë ·ÂÁ ‰ÂÏÂÌˆËﬂ ˜ÂÁ ÔÓ‰Ó·Ì‡ ·‡ÚÂËﬂ ÓÚ 37 ÔÓ‰ÒÍ‡ÎË Á‡ ÓˆÂÌÍ‡. è‡ˆËÂÌÚËÚÂ Ò Åè ·ÂÁ ‰ÂÏÂÌˆËﬂ Ò‡ ÔÓÍ‡Á‚‡ÎË Ì‡Ï‡ÎÂÌËÂ Ì‡ ËÌÙÓÏ‡ˆËÓÌÌÓÚÓ Ò˙‰˙Ê‡ÌËÂ Ì‡ ÒÔÓÌÚ‡ÌÌ‡Ú‡ Â˜, Í‡ÍÚÓ Ë ‚ ‡Á·Ë‡ÌÂÚÓ Ì‡ ÍÓÏÔÎÂÍÒÌË ÍÓÏ‡Ì‰Ë, ÌÓ Ì‡Û¯ÂÌËﬂÚ‡ ÌÂ Ò‡ ·ËÎË ÚÓÎÍÓ‚‡ ÔÓ‰˜ÂÚ‡ÌË, ÍÓÎÍÓÚÓ ÚÂÁË ‚ Â˜Ú‡ Ë ÏÂı‡ÌËÍ‡Ú‡ Ì‡ ÔËÒ‡ÌÂ. éÚ ‰Û„‡ ÒÚ‡Ì‡ Ú‡ÁË „ÛÔ‡ Ô‡ˆËÂÌÚË ÌÂ Ò‡ ÔÓÍ‡Á‡ÎË Ì‡Û¯ÂÌËﬂ ‚ ‡Á·Ë‡ÌÂÚÓ, ÔÓ‚ÚÓÂÌËÂÚÓ, Ì‡ÁÓ‚‡‚‡ÌÂÚÓ, ˜ÂÚÂÌÂÚÓ ËÎË ‡‚ÚÓÏ‡ÚËÁË‡Ì‡Ú‡ Â˜. ÑÛ„Ë ‡‚ÚÓË, ËÁÒÎÂ‰‚‡˘Ë ÂÁËÍÓ‚ËÚÂ ÙÛÌÍˆËË ÔË Ô‡ˆËÂÌÚË Ò Åè ·ÂÁ ‰ÂÏÂÌˆËﬂ, Ò˙Ó·˘‡‚‡Ú Á‡ ‰ÂÙËˆËÚË ‚ Ì‡ÏË‡ÌÂÚÓ Ì‡ ‰ÛÏË ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè ‚ Á‡‰‡˜ËÚÂ Á‡ ÁËÚÂÎÌÓ ÍÓÌÙÓÌÚ‡ˆËÓÌÌÓ Ì‡ÁÓ‚‡‚‡ÌÂ, ÍÓÂÚÓ Ó·‡˜Â ÒÂ ÛÎÂÒÌﬂ‚‡ ˜ÂÁ ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ÒÂÏ‡ÌÚË˜ÌÓ ËÎË ÙÓÌÂÚË˜ÌÓ ÔÓ‰ÔÓÏ‡„‡ÌÂ (43). íÂÁË ‡‚ÚÓË Ò˙˘Ó Ì‡·Î˛‰‡‚‡Ú ‰ËÒÓˆË‡ˆËﬂ ÏÂÊ‰Û Ì‡Û¯ÂÌËﬂÚ‡ ‚ ÎËÚÂ‡ÎÌ‡ Ë ÒÂÏ‡ÌÚË˜Ì‡ ‚Â·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Ì‡Û¯ÂÌËﬂ ‚ ÒÂÏ‡ÌÚË˜Ì‡Ú‡ (ËÁ‚ÎË˜‡ÌÂ Ì‡ ‰ÛÏË ÓÚ ÓÔÂ‰ÂÎÂÌ‡ ÒÂÏ‡ÌÚË˜Ì‡ Í‡ÚÂ„ÓËﬂ), ÌÓ ÌÂ Ë ‚ ÎËÚÂ‡ÎÌ‡Ú‡ ‚Â·‡ÎÌ‡ ÙÎÛÂÌÚÌÓÒÚ (Ì‡ÁÓ‚‡‚‡ÌÂ Ì‡ ‰ÛÏË, Á‡ÔÓ˜‚‡˘Ë Ò ÓÔÂ‰ÂÎÂÌ‡ ·ÛÍ‚‡). 5. áêàíÖãçé-èêéëíêÄçëíÇÖçà à äéçëíêìäíàÇçà çÄêìòÖçà êÂ‰Ëˆ‡ ‡‚ÚÓË Ò˙Ó·˘‡‚‡Ú Á‡ Ì‡ÎË˜ËÂ Ì‡ ‰ÂÙËˆËÚË ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÙÛÌÍˆËË ÔË ÌÂ‰ÂÏÂÌÚÌË 175 Ô‡ˆËÂÌÚË Ò Åè, ‰ÓË ÍÓ„‡ÚÓ ËÁÔÓÎÁ‚‡ÌËÚÂ ÚÂÒÚÓ‚Â ËÏ‡Ú Ï‡ÎÍÓ ÏÓÚÓÌË ÍÓÏÔÓÌÂÌÚË (13, 31). ê‡ÌÌËÚÂ Ì‡Û¯ÂÌËﬂ ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌ‡Ú‡ Ó„‡ÌËÁ‡ˆËﬂ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Åè ÒÂ Ò˙Ó·˘‡‚‡Ú ÓÚ ÌﬂÍÓË ‡‚ÚÓË Í‡ÚÓ ‚˙ÁÏÓÊÌË ÔÂ‰ËÍÚÓË Ì‡ ÔÓ-Í˙ÒÌÓÚÓ ‡Á‚ËÚËÂ Ì‡ ‰ÂÏÂÌˆËﬂ (33, 39). Clock Drawing Test (CDT) Â Â‰ËÌ ÓÚ ˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚÂ ÚÂÒÚÓ‚Â Á‡ ÓˆÂÌÍ‡ Ì‡ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÒÔÓÒÓ·ÌÓÒÚË Ì‡ ÚÂÁË Ô‡ˆËÂÌÚË, ÌÓ ‰ÓÍ‡ÚÓ Â‰ÌË ‡‚ÚÓË Ò˙Ó·˘‡‚‡Ú Á‡ ÁÌ‡˜ËÏË Ì‡Û¯ÂÌËﬂ ÔË ÌÂ‰ÂÏÂÌÚÌËÚÂ Ô‡ˆËÂÌÚË Ò Åè (51), ÚÓ ‰Û„Ë ÌÂ ÓÚÍË‚‡Ú ÔÓ‰Ó·ÌË ‰ÂÙËˆËÚË (44). Clock Drawing Test, Ô˙‚ÓÌ‡˜‡ÎÌÓ Á‡ÏËÒÎÂÌ ‰‡ ‚ÂËÙËˆË‡ ÓÔÂ‰ÂÎÂÌË ÍÓÚËÍ‡ÎÌË ÙÛÌÍˆËË ÔË Ô‡ˆËÂÌÚËÚÂ Ò ·ÓÎÂÒÚ Ì‡ ÄÎˆı‡ÈÏÂ (53), ‰ÓÔ˙ÎÌËÚÂÎÌÓ Ë‰ÂÌÚËÙËˆË‡ ÌﬂÍÓÎÍÓ Ô‡ÏÂÚÓ‚Ë ÔÓˆÂÒ‡, Ò‚˙Á‡ÌË Ò ÔÂÙÓÌÚ‡ÎÌËÚÂ Ë ÒÛ·ÍÓÚËÍ‡ÎÌËÚÂ ÏÂı‡ÌËÁÏË. íÂÁË ÔÓˆÂÒË ‚ÍÎ˛˜‚‡Ú ÔÓ‚Â‰ÂÌ˜ÂÒÍ‡Ú‡ Ó„‡ÌËÁ‡ˆËﬂ, Ô‡ÏÂÚÓ‚‡Ú‡ ÙÎÂÍÒË·ËÎÌÓÒÚ, ÁËÚÂÎÌÓ-ÍÓÌÒÚÛÍÚË‚ÌÓÚÓ ËÁÔ˙ÎÌÂÌËÂ, ÍÓ„ÌËÚË‚ÌÓÚÓ ÔÓ„‡ÏË‡ÌÂ Ì‡ ÔÒËıÓÏÓÚÓÌ‡Ú‡ ‡ÍÚË‚ÌÓÒÚ Ë ËÌÚÂ„‡ˆËﬂÚ‡ Ì‡ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÙÛÌÍˆËË (10, 49, 59). Ç Á‡ÍÎ˛˜ÂÌËÂ ¯ËÓÍ ‰Ë‡Ô‡ÁÓÌ ÓÚ ÍÓ„ÌËÚË‚ÌË ‰ÂÙËˆËÚË Ò‡ ÓÔËÒ‡ÌË ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè, Í‡ÚÓ Ì‡ÈËÁ‡ÁÂÌË ÓÚ Úﬂı Ò‡ ÚÂÁË ‚ Ó·Î‡ÒÚÚ‡ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË. ç‡Â‰ Ò Ì‡Û¯ÂÌËﬂÚ‡ ‚˙‚ ‚ÌËÏ‡ÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË, Ì‡ÎËˆÂ Ò‡ Ò˙˘Ó Ë ÒÂËÓÁÌË ‰ÓÍ‡Á‡ÚÂÎÒÚ‚‡ Á‡ ‰ÂÙËˆËÚË ‚ Ô‡ÏÂÚÚ‡, ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÙÛÌÍˆËË, ÍÓÌÒÚÛÍÚË‚ÌËÚÂ ÛÏÂÌËﬂ Ë Â˜Ú‡ ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Åè. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. Allcock, L.M., Rowan, E.N., Steen, I.N., Wesnes, K., Kenny, R.A., Burn, D.J. Impaired attention predicts falling in Parkinson's disease. Parkinsonism Relat Disord, 2009, 15, 110-5. Altgassen, M., Phillips, L., Kopp, U., Kliegel, M. Role of working memory components in planning performance of individuals with Parkinson's disease. Neuropsychologia, 2007, 45, 2393-7. Ashburn, A., Stack, E., Pickering, R.M., Ward, C.D. 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Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ, 2007, 7, 2, 77-80. ê‡È˜Â‚‡ å., èÂÚÓ‚‡ å., åÂı‡·Ë‡Ì ò, ÜÂÎÂ‚ ü., äËÍÓ‚‡ Ç., í‡ÈÍÓ‚ ã. ç‡Û¯ÂÌËﬂ ‚ ÂÍÁÂÍÛÚË‚ÌËﬂ ÍÓÌÚÓÎ ÔË Ô‡ˆËÂÌÚË Ò ÎÂÍÓ ÍÓ„ÌËÚË‚ÌÓ Ì‡Û¯ÂÌËÂ Ò Ë ·ÂÁ ·ÓÎÂÒÚ Ì‡ è‡ÍËÌÒÓÌ. IV-ÚË Ì‡ˆËÓÌ‡ÎÂÌ ÔÓ ÔÒËıÓÎÓ„Ëﬂ, 31.10.2008-02.11.2008, ëÓÙËﬂ, Å˙Î„‡Ëﬂ. ÜÂÎÂ‚ ü., èÂÚÓ‚‡ å., ê‡È˜Â‚‡ å., í‡ÈÍÓ‚ ã. çÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌË ÔÂ‰ËÍÚÓË Á‡ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ ÔË Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡ÚË˜Ì‡ è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ, Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ, 2009, 1, 20-23. Ä‰ÂÒ Á‡ ÍÓÂÒÔÓÌ‰ÂÌˆËﬂ: Ñ- å. èÂÚÓ‚‡, ìåÅÄã “ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, ·ÛÎ. “ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËﬂ 1431 ÚÂÎ: 02/92 30 544 Â-mail: [email protected] ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 177 éË„ËÌ‡ÎÌË ÒÚ‡ÚËË ÖîàäÄëçéëí à èéçéëàåéëí çÄ LEVETIRACETAM èêà ãÖóÖçàÖ çÄ èÄñàÖçíà ë ÖèàãÖèëàü á. á‡ı‡ËÂ‚, Ö. ÇËÚÂ‚‡ åÂ‰ËˆËÌÒÍË ìÌË‚ÂÒËÚÂÚ - èÎÓ‚‰Ë‚, äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ SUMMARY EFFICACY AND TOLERABILITY OF LEVETIRACETAM IN PATIENTS WITH EPILEPSY Z. Zahariev, E. Viteva AIMS: To evaluate long-term effectiveness of Levetiracetam (LEV) as polytherapy in patients with epilepsy – refractory or not. PATIENTS AND METHODS: A prospective study of 65 patients (36,92% men and 63,08% women) of median age 32,68±14,09 years and with median disease duration 18,37±11,62 years for a period of 1 year. 71,43% of them were with partial, 28,57% - with generalized epilepsy, 20% had idiopathic, 55% - symptomatic and 25% - cryptogenic epilepsy. The mean frequency of seizures before starting LEV was 4,1±6,4 per week for the partial seizures and 3,7±9,4 for the generalized ones. According to the applied dose of LEV, 4 groups were made out: 1000 mg/d, 1500 mg/d, 2000 mg/d and 3000 mg/d. LEV was given as polytherapy most often in combination with Valproate – in 16,15% and VPA+OCBZ – in 13,85%. The treatment was terminated in 3 patients due to adverse events or increase in seizure frequency. RESULTS: Reduction of seizure frequency _>50% was recorded in 57,89% of cases. The most frequently used dosage was 1500 mg/d – in 52,31%, the most effective - 1000mg/d – it resulted in seizure reduction _>50% in all patients. There was a minimal difference of the total effectiveness of the dosages 1500 and 2000 mg/d – the reduction of seizure frequency _>50% was in 53,33% and 52,63% of cases respectively. In 18,42% there was no change in the frequency of clinical manifestations, while in 15,79% there was worsening. The clinical effect was most marked in the 6-th month for the partial seizures, when the mean month frequency showed more than double frequency reduction and at the end of the first year for the generalized seizures – reduction of 59%. It was proven the most rapid and highest reduction for partial seizures and a gradual frequency reduction for generalized seizures. At the study end 2 of 4 patients with myoclonic seizures were with seizure frequency reduction higher than 50%. CONCLUSION: LEV is highly effective as polytherapy in patients with partial and generalized epilepsies with all types of etiology. KEY WORDS: Levetiraceram, effectiveness, side effects, polytherapy. êÖáûåÖ ñÖãà: Ñ‡ ÒÂ ÓˆÂÌË ‰˙Î„ÓÒÓ˜Ì‡Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ Levetiracetam (LÖV) ‚ ÔÓÎËÚÂ‡ÔËﬂ ÔË Ô‡ˆËÂÌÚË Ò ÂÔËÎÂÔÒËﬂ – ÂÙ‡ÍÚÂÌ‡ ËÎË ÌÂ. èÄñàÖçíà à åÖíéÑà: èÓÒÔÂÍÚË‚ÌÓ ÔÓÛ˜‚‡ÌÂ Ì‡ 65 Ô‡ˆËÂÌÚ‡ (36,92% Ï˙ÊÂ Ë 63,08% ÊÂÌË) Ì‡ ÒÂ‰Ì‡ ‚˙Á‡ÒÚ 32,68 ± 14,09 „., Ò˙Ò ÒÂ‰Ì‡ ‰‡‚ÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ 18,37 ± 11,62 „. Á‡ ÔÂËÓ‰ ÓÚ 1 „Ó‰ËÌ‡. éÚ Úﬂı 71,43% Ò‡ Ò Ô‡ˆË‡ÎÌ‡, 28,57% - Ò „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËﬂ, 20% - Ò Ë‰ËÓÔ‡ÚË˜Ì‡, 55% - Ò˙Ò ÒËÏÔÚÓÏ‡ÚË˜Ì‡ Ë 25% - Ò ÍËÔÚÓ„ÂÌÌ‡ ÂÔËÎÂÔÒËﬂ. éÚ˜ÂÚÂÌ‡ Â ÒÂ‰Ì‡ ˜ÂÒÚÓÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ ÔÂ‰Ë ‚ÍÎ˛˜‚‡ÌÂ Ì‡ LEV 4,1 ± 6,4 ÒÂ‰ÏË˜ÌÓ Á‡ Ô‡ˆË‡ÎÌËÚÂ Ë 3,7 ± 9,4 ÏÂÒÂ˜ÌÓ Á‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ. éÙÓÏÂÌË Ò‡ 4 „ÛÔË ÒÔÓÂ‰ ÔËÎ‡„‡Ì‡Ú‡ ‰ÓÁ‡ LEV: 1000 mg/d, 1500 mg/d, 2000 mg/d Ë 3000 mg/d. èÓ‚ÂÊ‰‡Ì‡ Â ÔÓÎËÚÂ‡ÔËﬂ Ì‡È-˜ÂÒÚÓ Ò Valproate – ÔË 16,15% Ë VPA + OCBZ – ÔË 13,85%. èË 3-Ï‡ ÓÚ ·ÓÎÌËÚÂ ÎÂ˜ÂÌËÂÚÓ Ò LEV e ÔÂÍ‡ÚÂÌÓ ÔÓ‡‰Ë ÓÚ˜ÂÚÂÌËÚÂ ÌÂÊÂÎ‡ÌË ÒÚ‡ÌË˜ÌË ﬂ‚ÎÂÌËﬂ ËÎË ‚ÎÓ¯‡‚‡ÌÂ Ì‡ ÍÎËÌË˜ÌËÚÂ ÔÓﬂ‚Ë. êÖáìãíÄíà: éÚ˜ÂÚÂ ÒÂ Ó·˘‡ Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔËÚÂ _>50% ÔË 57,89% ÓÚ ÒÎÛ˜‡ËÚÂ. ç‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡Ì‡Ú‡ ‰ÓÁ‡ Â 1500mg/d – ÔË 52,31%, ‡ Ì‡È-ÂÙÂÍÚË‚Ì‡ Â 1000mg/d – Â„ËÒÚË‡ ÒÂ ÔËÒÚ˙ÔÌ‡ Â‰ÛÍˆËﬂ _>50% - ÔË ‚ÒË˜ÍË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â ÔËÎ‡„‡Ì‡. åËÌËÏ‡ÎÌ‡ Â ‡ÁÎËÍ‡Ú‡ Á‡ Ó·˘‡Ú‡ Í‡ÈÌ‡ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ ‰ÓÁËÚÂ 1500 Ë 2000 mg/d - Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔËÚÂ _>50% Ò˙ÓÚ‚ÂÚÌÓ ÔË 53,33% Ë 52,63%. èË 18,42% ÓÚ Ô‡ˆËÂÌÚËÚÂ ÌÂ ÒÂ Ì‡·Î˛‰‡‚‡ ÔÓÏﬂÌ‡ ‚ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÍÎËÌË˜ÌËÚÂ ÔÓﬂ‚Ë, ‡ ÔË 15,79% ÒÂ ÓÚ˜ËÚ‡ ‚ÎÓ¯‡‚‡ÌÂ. äÎËÌË˜ÌËﬂÚ ÂÙÂÍÚ Â Ì‡ÈËÁ‡ÁÂÌ Ì‡ ¯ÂÒÚËﬂ ÏÂÒÂˆ Á‡ Ô‡ˆËÂÌÚËÚÂ Ò Ô‡ˆË‡ÎÌËÚÂ ÔËÒÚ˙ÔË, ÍÓ„‡ÚÓ ÒÂ‰Ì‡Ú‡ ÏÂÒÂ˜Ì‡ ˜ÂÒÚÓÚ‡ Â Ò Â‰ÛÍˆËﬂ ÔÓ‚Â˜Â ÓÚ 2 Ô˙ÚË Ë ‚ Í‡ﬂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡ Á‡ ÚÂÁË Ò „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË - Â‰ÛÍˆËﬂ Ò 59%. ÑÓÍ‡Á‡Ì‡ Â Ì‡È-·˙Á‡ Ë ‚ËÒÓÍ‡ Í‡ÈÌ‡ Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ÔË Ô‡ˆË‡ÎÌËÚÂ ÔËÒÚ˙ÔË Ë ÔÓ-ÔÓÒÚÂÔÂÌÌÓ Ì‡‡ÒÚ‚‡˘‡ Â‰ÛÍˆËﬂ ÔË „ÂÌÂ‡ÎËÁË‡ÌËÚÂ ÔËÒÚ˙ÔË. éÚ 4ÚÂ Ô‡ˆËÂÌÚ‡ Ò ÏËÓÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË 2-Ï‡ Ò‡ Ò ÔËÒÚ˙ÔÌ‡ Â‰ÛÍˆËﬂ >50% ‚ Í‡ﬂ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ. áÄäãûóÖçàÖ: LEV Â ‚ËÒÓÍÓ eÙÂÍÚË‚ÂÌ ÏÂ‰ËÍ‡ÏÂÌÚ ‚ ÔÓÎËÚÂ‡ÔËﬂ ÔË Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡ÎÌË Ë „ÂÌÂ‡ÎËÁË‡ÌË ÂÔËÎÂÔÒËË, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÚﬂıÌ‡Ú‡ ÂÚËÓÎÓ„Ëﬂ. äãûóéÇà Ñìåà: Levetiracetam, ÂÙÂÍÚË‚ÌÓÒÚ, ÒÚ‡ÌË˜ÌË ÂÙÂÍÚË, ÔÓÎËÚÂ‡ÔËﬂ. ÇöÇÖÑÖçàÖ Levetiracetam (Keppra) ÔËÌ‡‰ÎÂÊË Í˙Ï „ÛÔ‡Ú‡ Ì‡ ÔÓÌÓ‚ËÚÂ ‡ÌÚËÂÔËÎÂÔÚË˜ÌË ÏÂ‰ËÍ‡ÏÂÌÚË (ÄÖå) Ë Ì‡ÏË‡ ‚ÒÂ ÔÓ-¯ËÓÍÓ ÔËÎÓÊÂÌËÂ ‚ ÍÎËÌË˜Ì‡Ú‡ Ô‡ÍÚËÍ‡. èÂ‰ÒÚ‡‚Îﬂ‚‡ S-ÂÌ‡ÌÚËÓÏÂ Ì‡ ÔË‡ˆÂÚ‡Ï‡, ÍÓÈÚÓ ÔËÚÂÊ‡‚‡ ÏÌÓ„Ó ÓÚ Ò‚ÓÈÒÚ‚‡Ú‡ Ì‡ Ë‰Â‡ÎÌËﬂ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚ. éÒÌÓ‚ÌËÚÂ ÏÛ ÔÂ‰ËÏÒÚ‚‡ Ò‡ ·˙Á‡ ‡·ÒÓ·ˆËﬂ, ÎËÌÂÈÌ‡ Ù‡Ï‡ÍÓÍËÌÂÚËÍ‡, ÌÂÁÌ‡˜ËÚÂÎÌË ÎÂÍ‡ÒÚ‚ÂÌË ‚Á‡ËÏÓ-‰ÂÈÒÚ‚Ëﬂ, ÌÂÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ ˜ÂÌÓ‰Ó·ÌËÚÂ ÂÌÁËÏË Ë ·˙·Â˜ÌÓ ÂÎËÏËÌË‡ÌÂ (20). çÂÁ‡‰˙ÎÊËÚÂÎÌÓÚÓ ËÎË ·˙ÁÓ ÚËÚË‡ÌÂ Ë ‰‚ÛÍ‡ÚÌËﬂÚ ÔËÂÏ Ò˙˘Ó ÛÎÂÒÌﬂ‚‡Ú ÌÂ„Ó‚ÓÚÓ ÔËÎ‡„‡ÌÂ. ÄÌÚËÍÓÌ‚ÛÎÒË‚ÌËﬂÚ ÂÙÂÍÚ Ì‡ LEV ÒÂ ‰˙ÎÊË Ì‡ ÌﬂÍÓÎÍÓ ÒÔÂˆËÙË˜ÌË ÏÂı‡ÌËÁÏ‡: 1. ë‚˙Á‚‡ÌÂ Ò˙Ò ÒËÌ‡ÔÚË˜ÌËÚÂ ‚ÂÁËÍÛÎÌË ÔÓÚÂËÌË SV2A; 2. êÂ‰ÛÍˆËﬂ Ì‡ ‚ËÒÓÍÓ‚ÓÎÚ‡ÊÌÓ ‡ÍÚË‚Ë‡˘ËÚÂ ÒÂ N-ÚËÔ Ë ˜‡ÒÚË˜ÌÓ - P/Q-ÚËÔ Í‡ÎˆËÂ‚Ë ÔÓÚÓˆË; 3. ç‡Ï‡ÎÂÌÓ ÓÒ‚Ó·ÓÊ‰‡‚‡ÌÂ Ì‡ Í‡ÎˆËÈ ÓÚ ‚˙ÚÂÍÎÂÚ˙˜ÌËÚÂ ÌÂ‚ÓÌÌË ‰ÂÔ‡; 4. êÂ‰ÛÍˆËﬂ Ì‡ ËÌıË·ËÚÓÌËﬂ ÂÙÂÍÚ Ì‡ ˆËÌÍ Ë β-Í‡·ÓÎËÌËÚÂ ‚˙ıÛ ıÎÓË‰ÌËﬂ ËÌÙÎÛÍÒ ˜ÂÁ ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ ÉÄåä- Ë „ÎËˆËÌÓ‚ËÚÂ ÂˆÂÔÚÓË (4). ÉÓÎﬂÏ ·ÓÈ ÔÓÛ˜‚‡ÌËﬂ ÔÓÍ‡Á‚‡Ú ÂÙËÍ‡ÒÌÓÒÚÚ‡ Ì‡ LEV Í‡ÚÓ ÏÓÌÓ- Ë ÔÓÎËÚÂ‡ÔËﬂ ÔË Ô‡ˆË‡ÎÌË, ÏËÓÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË, Ô˙‚Ë˜ÌÓ ËÎË ‚ÚÓË˜ÌÓ „ÂÌÂ‡ÎËÁË‡ÌË ÚÓÌË˜ÌÓ-ÍÎÓÌË˜ÌË (Éíäè), ÙÓÚÓÒÂÌÁËÚË‚ÌË ÔËÒÚ˙ÔË Ë ‡·Ò‡ÌÒË (7, 18, 19, 27, 33, 34, 35). èÓÚ‚˙‰ÂÌ‡ Â Ë ÌÂ„Ó‚‡- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ Í‡ÚÓ ÏÂ‰ËÍ‡ÏÂÌÚ ÓÚ Ô˙‚Ë Â‰ ÔË ÌÓ‚Ó‰Ë‡„ÌÓÒÚËˆË‡ÌË Ô‡ˆËÂÌÚË Ò Ô˙‚Ë˜ÌÓ Éíäè ËÎË ÔËÒÚ˙ÔË Ò Ô‡ˆË‡ÎÌÓ Ì‡˜‡ÎÓ, ÍÓﬂÚÓ Â ÒıÓ‰Ì‡ Ò Ú‡ÁË Ì‡ CBZ ÔË ÔÓ-‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ (14, 15). èÓ‰ıÓ‰ﬂ˘ Â Ë ÔË ÁÎÓÍ‡˜ÂÒÚ‚ÂÌËÚÂ ‰ÂÚÒÍË ÂÔËÎÂÔÒËË – ÒËÌ‰ÓÏ Ì‡ West, Lennox-Gastaut Ë ÔÓ„ÂÒË‚Ì‡ ÏËÓÍÎÓÌÛÒ ÂÔËÎÂÔÒËﬂ, ÂÔËÎÂÔÒËﬂ Ò ÂÎÂÍÚË˜ÂÌ ÒÚ‡ÚÛÒ ÂÔËÎÂÔÚËÍÛÒ ÔÓ ‚ÂÏÂ Ì‡ ·‡‚ÌÓ‚˙ÎÌÓ‚Ëﬂ Ò˙Ì, ÔË ÍÓËÚÓ Ò˙˘Ó ÒÂ ÓÚ˜ËÚ‡ ËÁ‚ÂÒÚÂÌ ·Î‡„ÓÔËﬂÚÂÌ ÍÎËÌË˜ÂÌ Ë ÂÎÂÍÚÓÙËÁËÓÎÓ„Ë˜ÂÌ ÂÙÂÍÚ (2, 3, 7, 8, 16). ì‰‡˜ÌÓ Â ÔËÎ‡„‡ÌÂÚÓ ÏÛ ‚ ÏÓÌÓ- ËÎË ÔÓÎËÚÂ‡ÔËﬂ ÔË ‰Âˆ‡ Ë ÔÓ-‚˙Á‡ÒÚÌË Ô‡ˆËÂÌÚË, ÔÂ‰‚Ë‰ ‰Ó·‡Ú‡ ÔÓÌÓÒËÏÓÒÚ Ë ÔÓÁËÚË‚ÌËﬂ ÂÙÂÍÚ ‚˙ıÛ Í‡˜ÂÒÚ‚ÓÚÓ Ì‡ ÊË‚ÓÚ (10, 13). èÓ-ı‡‡ÍÚÂÌË ÌÂÊÂÎ‡ÌË ÒÚ‡ÌË˜ÌË ‰ÂÈÒÚ‚Ëﬂ Ò‡ ‰ÓÁÓÓ„‡ÌË˜‡‚‡˘ËÚÂ ‚ ÌﬂÍÓË ÒÎÛ˜‡Ë ÛÏÓﬂÂÏÓÒÚ Ë Ò˙ÌÎË‚ÓÒÚ, Í‡ÍÚÓ Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË ÓÚÍÎÓÌÂÌËﬂ, ÓÒÓ·ÂÌÓ ËÁ‡ÁÂÌË ÔË ·ÓÎÌË Ò ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ Ë/ËÎË ÔÂ‰¯ÂÒÚ‚‡˘‡ ÔÒËıË‡ÚË˜Ì‡ ÒËÏÔÚÓÏ‡ÚËÍ‡. ñÂÎÚ‡ Ì‡ Ì‡ÒÚÓﬂ˘ÂÚÓ ÔÓÛ˜‚‡ÌÂ Â ‰‡ ÒÂ ÓÔÂ‰ÂÎË ÓÔÚËÏ‡ÎÌ‡Ú‡ ‰ÓÁ‡ Ë ‰‡ ÒÂ ÓÚ˜ÂÚÂ ÂÙËÍ‡ÒÌÓÒÚÚ‡ Ë ÔÓÌÓÒËÏÓÒÚÚ‡ Ì‡ Levetiracetam (LEV) Á‡ ÒÓÍ ÓÚ 12 ÏÂÒÂˆ‡ ÔË ÔÓÎËÚÂ‡ÔËﬂ Ì‡ Ô‡ˆËÂÌÚË Ò Ô‡ˆË‡ÎÌ‡ Ë „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËﬂ – ÂÁËÒÚÂÌÚÌ‡ ËÎË ÌÂ. èÄñàÖçíà à åÖíéÑà éÒ˙˘ÂÒÚ‚ËıÏÂ ÔÓÒÔÂÍÚË‚ÌÓ Ì‡·Î˛‰ÂÌËÂ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ ÓÚ 6 ÏÂÒÂˆ‡ ‰Ó 1 „. Ì‡ 65 Ô‡ˆËÂÌÚ‡ Ò ÂÔËÎÂÔÒËﬂ (ÛÚ‚˙‰ÂÌ‡ ËÎË ÌÂ Í‡ÚÓ ÂÁËÒÚÂÌÚÌ‡/ Ì‡ ÔÓÎËÚÂ‡ÔËﬂ Ò LEV. ëÂ‰Ì‡Ú‡ ‚˙Á‡ÒÚ Ì‡ Ô‡ˆËÂÌÚËÚÂ Â 32,68 ± 14,09 „. (ÓÚ 14 ‰Ó 57 „.), ‡ ÒÂ‰Ì‡Ú‡ ‰‡‚ÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ - 18,37 ± 11,62 „. (ÓÚ 22 ‰ÌË ‰Ó 40 „.). ëËÒÚÂÏ‡ÚËÁË‡ıÏÂ Û˜‡ÒÚÌËˆËÚÂ Ò˙Ó·‡ÁÌÓ: ÔÓÎ, ‚˙Á‡ÒÚ, ‰‡‚ÌÓÒÚ, ÂÚËÓÎÓ„Ëﬂ, ‚Ë‰ Ì‡ ÂÔËÎÂÔÒËﬂÚ‡, ÚËÔ Ë ˜ÂÒÚÓÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ, ÔÂ‰¯ÂÒÚ‚‡˘‡ Ë Ò˙Ô˙ÚÒÚ‚‡˘‡ ÚÂ‡ÔËﬂ Ë „Ë ÔÓÒÎÂ‰ËıÏÂ Ì‡ 6 ÏÂÒÂˆ‡ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÒÓÏ‡ÚË˜ÂÌ, ÌÂ‚ÓÎÓ„Ë˜ÂÌ, ÔÒËıË˜ÂÌ ÒÚ‡ÚÛÒ, ÖÖÉ. èË ÌÂÓ·ıÓ‰ËÏÓÒÚ ÓÒ˙˘ÂÒÚ‚ËıÏÂ äÄí Ë üåê (í‡·ÎËˆ‡ 1). èÓÒÎÂ‰ËıÏÂ ‚ ‰ËÌ‡ÏËÍ‡ ÂÙÂÍÚ‡ ÓÚ ÔËÎÓÊÂÌËÂÚÓ Ì‡ LEV, ÓÚ˜ËÚ‡ÈÍË ˜ÂÒÚÓÚ‡Ú‡ Ë ÚÂÊÂÒÚÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ, Í‡ÚÓ ËÁÔÓÎÁ‚‡ıÏÂ LEV Í‡ÚÓ ÔÓÎËÚÂ‡ÔËﬂ ‚ ‰ÓÁ‡ ÓÚ 1000 ‰Ó 3000 Ï„, ÒÂ‰Ì‡ ‰ÓÁ‡ 1646 ± 359 mg/d. ê‡ÁÔÂ‰ÂÎËıÏÂ Ô‡ˆËÂÌÚËÚÂ ‚ 4 „ÛÔË ÒÔÓÂ‰ ‰ÌÂ‚Ì‡Ú‡ ‰ÓÁ‡ – 1000 mg/d – 7 (10,77%) 1500 mg/d – 34 (52,31%), 2000 mg/d – 23 (35,38%) Ë 3000 mg/d – 1 (1,54%). ëÔÓÂ‰ ÔÓ‚ÎËﬂ‚‡ÌÂÚÓ Ì‡ ÚÂÊÂÒÚÚ‡ Ë ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÂÔËÎÂÔÚË˜ÌËÚÂ ÔËÒÚ˙ÔË ‚ Í‡ﬂ Ì‡ Ì‡·Î˛‰ÂÌËÂÚÓ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‰ÓÁ‡Ú‡ Ì‡ LEV Ë ÚËÔ‡ ÔËÒÚ˙ÔË, ‡ÁÔÂ‰ÂÎËıÏÂ Ô‡ˆËÂÌÚËÚÂ ‚ 6 „ÛÔË – Ò ‚ÎÓ¯‡‚‡ÌÂ, ·ÂÁ ÔÓÏﬂÌ‡, Â‰ÛÍˆËﬂ <50%, Â‰ÛÍˆËﬂ 50%, Â‰ÛÍˆËﬂ 51-99%, Â‰ÛÍˆËﬂ 100%. á‡ ÓÚ„Ó‚ÓËÎË Ì‡ ÎÂ˜ÂÌËÂÚÓ /ËÎË ÂÒÔÓÌ‰ÂË/ ÔËÂıÏÂ Ô‡ˆËÂÌÚËÚÂ Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ _>50%, ‡ Á‡ ÌÂÓÚ„Ó‚ÓËÎË – Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ÔÓ‰ 50%. éÚ˜ÂÚÓıÏÂ Ë ÌÂÊÂÎ‡ÌËÚÂ ÒÚ‡ÌË˜ÌË ÂÙÂÍÚË. èËÎÓÊËıÏÂ ‚‡Ë‡ˆËÓÌÂÌ Ë „‡ÙË˜ÂÌ ‡Ì‡ÎËÁ Á‡ ÒÚ‡ÚËÒÚË˜ÂÒÍ‡ Ó·‡·ÓÚÍ‡. ëÂ‰Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ ÔÂ‰Ë LEV Â 4,1 ± 6,4 ÒÂ‰ÏË˜ÌÓ /ÓÚ 1 „Ó‰. ‰Ó 35 ÒÂ‰ÏË˜ÌÓ (Á‡ Ô‡ˆË‡ÎÌËÚÂ Ë 3,7 ± 9,4 ÏÂÒÂ˜ÌÓ) ÓÚ 1 Ì‡ 4 ÏÂÒ. ‰Ó 40 ÏÂÒÂ˜ÌÓ Á‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ. ìÒÚ‡ÌÓ‚ËıÏÂ ÓÚÍÎÓÌÂÌËﬂ ‚ ÌÂ‚ÓÎÓ„Ë˜ÌËﬂ ÒÚ‡ÚÛÒ ÔË 10 (15,38%) Ô‡ˆËÂÌÚ‡, ‡ ‚ ÔÒËıË˜ÌËﬂ ÒÚ‡ÚÛÒ - ÔË 21 (32,31%) - ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, ÍÓ„ÌËÚË‚ÌË Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ. ÇÒË˜ÍË Û˜‡ÒÚÌËˆË Ò‡ ÔËÂÏ‡ÎË ÔÂ‰Ë ÚÓ‚‡ ÄÖå. éÒÂÏ (12,31%) ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò‡ ÒÂ ÎÂÍÛ‚‡ÎË Ò‡ÏÓ Ò 1 ÄÖå, ÓÒÚ‡Ì‡ÎËÚÂ 57 (87,69%) - Ò ÂÁËÒÚÂÌÚÌ‡ ÂÔËÎÂÔÒËﬂ - Ò‡ ·ËÎË Ì‡ ÔÓÎËÚÂ‡ÔËﬂ Ò ‰Ó 5 ÏÂ‰ËÍ‡ÏÂÌÚ‡ (VPA, CBZ, PB, CZP, ESM, Diazepam, PHT, OCBZ, TPM, LTG, 178 í‡·ÎËˆ‡ 1: ï‡‡ÍÚÂËÒÚËÍ‡ Ì‡ Ô‡ˆËÂÌÚËÚÂ èÓÍ‡Á‡ÚÂÎ èÓÎ - Ï˙ÊÂ - ÊÂÌË Ñ‡‚ÌÓÒÚ Ì‡ ÂÔËÎÂÔÒËﬂÚ‡ /Á‡ 63 ·ÓÎÌË/ - ‰Ó 1 „. |- ÓÚ 1 ‰Ó 3 „. - Ì‡‰ 3 „. ÖÚËÓÎÓ„Ëﬂ Ì‡ ÂÔËÎÂÔÒËﬂÚ‡ /ÛÚÓ˜ÌÂÌ‡ ÔË 60 ·ÓÎÌË/ à‰ËÓÔ‡ÚË˜Ì‡ äËÔÚÓ„ÂÌÌ‡ ëËÏÔÚÓÏ‡ÚË˜Ì‡ /óåí, ‚˙ÁÔ‡ÎËÚÂÎÌ‡, Ó·ÂÏÂÌ ÔÓˆÂÒ, ÔÂËÌ‡Ú‡ÎÌ‡ ‡ÒÙËÍÒËﬂ, ÂÌˆÂÙ‡ÎÓÔ‡ÚËﬂ, ÚÓÍÒË˜Ì‡ /‡ÎÍÓıÓÎÌ‡/, óåí + ‚˙ÁÔ‡ÎËÚÂÎÌ‡, Ò˙‰Ó‚‡, óåí + ÏÂÁË‡ÎÌ‡ „ÎËÓÁ‡, óåí + ‚˙ÁÔ‡ÎËÚÂÎÌ‡, ÚÛ·ÂÓÁÌ‡ ÒÍÎÂÓÁ‡, ıËÔÓÍ‡ÏÔ‡ÎÌ‡ ÒÍÎÂÓÁ‡, ÎÓÍ‡ÎÌ‡ ÏÓÁ˙˜Ì‡ ‡„ÂÌÂÁËﬂ, ÒËÌ‰ÓÏ Ì‡ êÂÈ/ ÇË‰ Ì‡ ÂÔËÎÂÔÒËﬂÚ‡ /ÛÚÓ˜ÌÂÌ‡ ÔË 63 Ô‡ˆËÂÌÚ‡/ - Ô‡ˆË‡ÎÌ‡ /ÖÔËÎÂÔÒËﬂ Ô‡ˆË‡ÎËÒ ÍÓÌÚËÚÛ‡ Ì‡ äÓÊÂ‚ÌËÍÓ‚ – ÔË 1/ - „ÂÌÂ‡ÎËÁË‡Ì‡ /ûåÖ – ÔË 1, èÓ„ÂÒË‚Ì‡ ÏËÓÍÎÓÌÛÒ ÂÔËÎÂÔÒËﬂ – ÔË 1/ ÇË‰ Ì‡ ÔËÒÚ˙ÔËÚÂ /Á‡ 63 Ô‡ˆËÂÌÚ‡/ 1. è‡ˆË‡ÎÌË Ç ÚÓ‚‡ ˜ËÒÎÓ: - èèè - äèè - è‡ˆË‡ÎÌË Ò ÇÉ 2. ÉÂÌÂ‡ÎËÁË‡ÌË - Éíäè 3. äÓÏ·ËÌË‡ÌË n % Sp 24 41 36,92 63,08 6,13 6,13 1 3 59 1,75 5,25 93 1,67 2,83 3,24 12 15 33 20 25 55 5,21 5,64 6,48 45 71,43 5,74 18 28,57 5,74 28 44,45 6,31 2 7 19 3,17 11,12 30,16 2,23 3,99 5,83 13 22 20,63 34,92 5,14 6,05 GBP, TGB, ÍÓËÚÓ Ò‡ ÒÂ ÓÍ‡Á‡ÎË Ò ÌÂ‰ÓÒÚ‡Ú˙˜Ì‡ ËÎË ‚ÂÏÂÌÌ‡ ÂÙÂÍÚË‚ÌÓÒÚ. èË ‚ÒË˜ÍË ·ÓÎÌË LEV Â ‚ÍÎ˛˜ÂÌ Í‡ÚÓ ‰Ó·‡‚˙˜Ì‡ ÚÂ‡ÔËﬂ, Í‡ÚÓ ÔË 5 ·ÓÎÌË Â Á‡ÏÂÌËÎ ÔËÂÏ‡ÌË ‰Ó ÚÓÁË ÏÓÏÂÌÚ ÄÖå. çÂ e ÔËÎ‡„‡Ì Í‡ÚÓ ÏÓÌÓÚÂ‡ÔËﬂ. àÁÔÓÎÁ‚‡Ì Â ‚ ÍÓÏ·ËÌ‡ˆËﬂ Ò VPA – ÔË 17 (16,15%), Ò VPA Ë OCBZ – ÔË 9 (13,85%), CBZ – ÔË 5 (7,69%) Ë Ò OCBZ – ÔË 5 (7,69%). èË 6 (9,23%) (Ô‡ˆËÂÌÚË Ò ‚ËÒÓÍÓ ÂÁËÒÚÂÌÚÌ‡ ÂÔËÎÂÔÒËﬂ) LEV Â ‚ ˜ÂÚ‚ÓÌ‡ ÍÓÏ·ËÌ‡ˆËﬂ ÓÚ ÄÖå – îË- „Û‡ 1. êÖáìãíÄíà êÖÑìäñàü çÄ ÖèàãÖèíàóçàíÖ èêàëíöèà ê‡ÁÔÓÎ‡„‡ÏÂ Ò ËÌÙÓÏ‡ˆËﬂ Á‡ 38 ÓÚ Ô‡ˆËÂÌÚËÚÂ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÍÎËÌË˜ÌËﬂ ÂÙÂÍÚ Ë ÒÚ‡ÌË˜ÌËÚÂ ‰ÂÈÒÚ‚Ëﬂ. ìÚÂÊÌﬂ‚‡ÌÂ Ì‡ ÍÎËÌË˜ÌËÚÂ ËÁﬂ‚Ë ÓÚ˜ËÚ‡ÏÂ ÔË 6 (15,79%) Û˜‡ÒÚÌËÍ‡, ÔÓÏﬂÌ‡ ÌÂ Ì‡ÒÚ˙Ô‚‡ ÔË 7 (18,42%), ÒÎ‡·Ó ÔÓ‚ÎËﬂÌË – Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ÔÓ‰ 50% Ò‡ 3 (7,89%) ËÎË ÌÂÓÚ„Ó‚ÓËÎË Ì‡ ÎÂ˜ÂÌËÂÚÓ Ò LEV Ò‡ Ó·˘Ó 16 (42,11%) ·ÓÎÌË. êÂ‰ÛÍˆËﬂ Ì‡ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÔËÒÚ˙ÔËÚÂ Ò 50% Ì‡·Î˛‰‡‚‡ÏÂ ÔË 8 (21,05%), ÔË 11 (28,96%) Â„ËÒÚË‡ÏÂ Â‰ÛÍˆËﬂ ÔÓ-„ÓÎﬂÏ‡ ÓÚ 50% Ë ÔÓ‰ 100%, 3 (7,89%) ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò‡ Ò‚Ó·Ó‰ÌË ÓÚ ÔËÒÚ˙ÔË ‚ Í‡ﬂ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ, Ú.Â. 22 (57,89%) ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò‡ ÓÚ„Ó‚ÓËÎË Ì‡ ÎÂ˜ÂÌËÂÚÓ Ò LEV – îË„. 2. èéÇãàüÇÄçÖ çÄ ÖèàãÖèíàóçàíÖ èêàëíöèà ëèéêÖÑ ÑéáÄíÄ LEV ç‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡ÌËÚÂ ÓÚ Ì‡Ò ‰ÓÁË Á‡ ÔÓÎËÚÂ‡ÔËﬂ Ò‡ 2000 Ë 1500 mg/d, ‡ Ì‡È-ÂÙÂÍÚË‚Ì‡ Â 1000 mg/d – ÔÓÒÚË„Ì‡Ú‡ Â Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ _>50% ÔË ‚ÒË˜ÍË Ô‡ˆËÂÌÚË, ÔË ÍÓËÚÓ Â ÔËÎ‡„‡Ì‡ (Ò Ô‡ˆË‡ÎÌË Ë „ÂÌÂ‡ÎËÁË‡ÌË, ÌÓ ÌÂ Ë Ò ÍÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË) – îË„Û‡ 3. èË ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ ‰ÓÁ‡Ú‡ 1500 mg/d ÂÒÔÓÌ‰ÂË Ò‡ 50% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, 50% - Ò „ÂÌÂ‡ÎËÁË‡ÌË Ë 71,43% - Ò ÍÓÏ·ËÌË‡ÌË. á‡ ‰ÓÁ‡Ú‡ ÓÚ 2000 mg/d Ò˙ÓÚ‚ÂÚÌÓÚÓ ÔÓ‚ÎËﬂ‚‡ÌÂ Â 50% Á‡ Û˜‡ÒÚÌËˆËÚÂ Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË Ë 71,43% ÓÚ ÚÂÁË Ò ÍÓÏ·ËÌË‡ÌË. ÅÓÎÌËÚÂ Ò „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË Ì‡ Ú‡ÁË ‰ÓÁ‡ Ò‡ ·ÂÁ ÔÓÏﬂÌ‡ ‚ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ËÎË Ò ‚ÎÓ¯‡‚‡ÌÂ. ÑàçÄåàóçé èêéëãÖÑüÇÄçÖ çÄ ÖîÖäíÄ éí èéãàíÖêÄèàüíÄ ë LEV ëèéêÖÑ íàèÄ ÖèàãÖèíàóçà èêàëíöèà èÄêñàÄãçà èêàëíöèà ë‡ÏÓ Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË ÒÂ Ì‡·Î˛‰‡‚‡Ú ÔË 28 (44,45%) ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÌÓ ÔË 16 Â ÓÚ˜ÂÚÂÌ ÂÙÂÍÚ‡ Ì‡ 6-ÚËﬂ Ë ÔË 14 – Ì‡ 12-ÚËﬂ ÏÂÒÂˆ. éÚ˜ËÚ‡ÏÂ ‰Ó·Ó ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ Ë ÚÂÊÂÒÚÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ. èË 3-Ï‡ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ì‡ 6-ÚËﬂ ÏÂÒÂˆ Ë ÔË 2-Ï‡ Ì‡ 1 „Ó‰ËÌ‡ ËÏ‡ ÔÓ‚Ë¯‡‚‡ÌÂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡. ç‡ 6ÚËﬂ ÏÂÒÂˆ Ë 1 „. ÓÚ ÎÂ˜ÂÌËÂÚÓ ÔË 5 Ô‡ˆËÂÌÚ‡ ÌÂ ÓÚ˜ËÚ‡ÏÂ ÔÓÏﬂÌ‡ ‚ ÍÎËÌË˜ÌËÚÂ ÔÓﬂ‚Ë. Ç Í‡ﬂ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ ÌÂÓÚ„Ó‚ÓËÎË Ò‡ 8 (57,15%) ÓÚ 14 Ô‡ˆËÂÌÚ‡. éÚÌÓ- 179 ÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ Û˜‡ÒÚÌËˆËÚÂ Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔËÚÂ _>50% Â 50% Ì‡ 6-ÚËﬂ ÏÂÒÂˆ, ÒÎÂ‰ ÍÓÂÚÓ Ì‡Ï‡Îﬂ‚‡ ‰Ó 42,85% ‚ Í‡ﬂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡. ç‡ Ô˙‚‡Ú‡ ‚ËÁËÚ‡ ÓÚ ‚ÍÎ˛˜‚‡ÌÂÚÓ Ì‡ LEV Ò‚Ó·Ó‰ÌË ÓÚ ÔËÒÚ˙ÔË Ò‡ 12,5% ÓÚ Û˜‡ÒÚÌËˆËÚÂ, ‚ÔÓÒÎÂ‰ÒÚ‚ËÂ ÓÚÌÓÒËÚÂÎÌËﬂÚ ËÏ ‰ﬂÎ Ì‡Ï‡Îﬂ‚‡ ‰Ó 7,14% Ì‡ 1 „. (îË„. 4). ÉÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË. ÉÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙ÔË ÒÂ Ì‡·Î˛‰‡‚‡Ú ÔË 13 (20,63%) ÓÚ Û˜‡ÒÚÌËˆËÚÂ ‚ ÔÓÛ˜‚‡ÌÂÚÓ. ëÂ‰ÂÏ ÓÚ Úﬂı ÒÂ ÔÓÒÎÂ‰ﬂ‚‡Ú Á‡ ÔÂËÓ‰ ÓÚ 6 ÏÂÒÂˆ‡ ‰Ó 1 „Ó‰ËÌ‡. ÖÙÂÍÚ˙Ú ÓÚ ÎÂ˜ÂÌËÂÚÓ Ì‡ 6-ÚËﬂ ÏÂÒÂˆ ÒÂ Ò‚ÂÊ‰‡ ‰Ó 57,15% ÌÂÓÚ„Ó‚ÓËÎË, ÓÚ ÍÓËÚÓ 28,57% Ò‡ Ò ‚ÎÓ¯‡‚‡ÌÂ Ì‡ ÍÎËÌË˜ÌËÚÂ ËÁﬂ‚Ë, ÔË 14,29% ÎËÔÒ‚‡ ÍÎËÌË˜ÂÌ ÂÙÂÍÚ, ‡ 14,29% Ò‡ Ò˙Ò ÒÎ‡· ÓÚ„Ó‚Ó – ÔÓ‰ 50% Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡. éÒÚ‡Ì‡ÎËÚÂ 42,85% Ò‡ Ò _>50% Â‰ÛÍˆËﬂ Ì‡ ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÔËÒÚ˙ÔËÚÂ. Ç ÔÓˆÂÒ‡ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ ÓÚÌÓÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ ÓÚ„Ó‚ÓËÎËÚÂ Ì‡‡ÒÚ‚‡ Ì‡ 57,13% ‚ Í‡ﬂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡. éÔËÒ‡ÌËÚÂ Û˜‡ÒÚÌËˆË Ò ÛÚÂÊÌﬂ‚‡ÌÂ Ì‡ ÍÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡ Ì‡ 6-ÚËﬂ ÏÂÒÂˆ Ì‡Ï‡Îﬂ‚‡Ú Ì‡ ÒÎÂ‰‚‡˘‡Ú‡ ‚ËÁËÚ‡ Ò 50%, ‡ ÚÂÁË ·ÂÁ ÔÓ‚ÎËﬂ‚‡ÌÂ ÓÒÚ‡‚‡Ú Ò˙˘ËﬂÚ ·ÓÈ. àÌÚÂÂÒÌ‡ Â Ë ‰ËÌ‡ÏËÍ‡Ú‡ ‚ ÓÚÌÓÒËÚÂÎÌËﬂ ·ÓÈ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ò‚Ó·Ó‰ÌË ÓÚ ÔËÒÚ˙ÔË - Ì‡ 6-ÚËﬂ ÏÂÒÂˆ Â 28,57%, Ì‡ 1 „. – Ì‡‡ÒÚ‚‡ ‰Ó 42,84 (îË„. 5). ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology äÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË. ë ÍÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË Ò‡ 22 (34,92%) ÓÚ Û˜‡ÒÚÌËˆËÚÂ ‚ ÔÓÛ˜‚‡ÌÂÚÓ. èË 15 ÓÚ Úﬂı ÔÓÒÎÂ‰ËıÏÂ ÚÂÊÂÒÚÚ‡ Ë ˜ÂÒÚÓÚ‡Ú‡ Ì‡ ÔËÒÚ˙ÔËÚÂ ‚ ‰ËÌ‡ÏËÍ‡. ç‡ 6-ÚËﬂ ÏÂÒÂˆ ÓÚ ÔÓÒÎÂ‰ﬂ‚‡ÌÂÚÓ Â‰ÛÍˆËﬂÚ‡ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ Ò > _50% Â Ì‡ÎË˜Ì‡ ÔË 60% ÓÚ Ô‡ˆËÂÌÚËÚÂ. òÂÒÚ ÏÂÒÂˆ‡ ÔÓ-Í˙ÒÌÓ ÓÚÌÓÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ Û˜‡ÒÚÌËˆËÚÂ Ò Ú‡Í‡‚‡ ÔËÒÚ˙ÔÌ‡ Â‰ÛÍˆËﬂ Ì‡‡ÒÚ‚‡ Ì‡ 66,67%. ÄÌ‡ÎÓ„Ë˜Ì‡ Â ‰ËÌ‡ÏËÍ‡Ú‡ ‚ ÓÚÌÓÒËÚÂÎÌËﬂ ‰ﬂÎ Ì‡ ÌÂÓÚ„Ó‚ÓËÎËÚÂ Ì‡ ÎÂ˜ÂÌËÂÚÓ. ç‡ 6-ÚËﬂ ÏÂÒÂˆ ÚÓÈ Â 40%, ÓÚ ÍÓËÚÓ 13,33% Ò‡ Ò ÛÚÂÊÌﬂ‚‡ÌÂ Ì‡ ÍÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡, 20% Ò‡ ·ÂÁ ÓÚ˜ÂÚÂÌ ÍÎËÌË˜ÂÌ ÂÙÂÍÚ Ë 6,67% Ò˙Ò ÒÎ‡·Ó ÔÓ‚ÎËﬂ‚‡ÌÂ – Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ Ò ÔÓ-Ï‡ÎÍÓ ÓÚ 50%. Ç ÔÓˆÂÒ‡ Ì‡ Ì‡·Î˛‰ÂÌËÂ ÛÚÂÊÌﬂ‚‡ÌÂ Ì‡ ÍÎËÌË˜ÌËÚÂ ÔÓﬂ‚Ë ÒÂ Â„ËÒÚË‡ ÔË 13,33% ‚ Í‡ﬂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡. ãËÔÒ‚‡Ú Ô‡ˆËÂÌÚË Ò Ô˙ÎÌÓ ÍÛÔË‡ÌÂ Ì‡ ÔËÒÚ˙ÔËÚÂ Ì‡ 6-ÚËﬂ ÏÂÒÂˆ ÔÓ ‚ÂÏÂ Ì‡ ˆÂÎËﬂ ÔÂËÓ‰ Ì‡ Ì‡·Î˛‰ÂÌËÂ (îË„. 6). éÚ 6 ÎËˆ‡ (9,52%) Ò ÏËÓÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË ÔÓÒÎÂ‰ËıÏÂ ‚ ‰ËÌ‡ÏËÍ‡ ÂÙÂÍÚ‡ ÓÚ ÎÂ˜ÂÌËÂÚÓ ÔË 4 ·ÓÎÌË. ç‡ 6-ÚËﬂ ÏÂÒÂˆ ÓÚ Ì‡·Î˛‰ÂÌËÂÚÓ 3-Ï‡ Ô‡ˆËÂÌÚË Ò‡ ·ÂÁ ÔÓ‚ÎËﬂ‚‡ÌÂ, ‡ ÔË 1 – ÔËÒÚ˙ÔËÚÂ Ò‡ ÍÛÔË‡ÌË Ì‡Ô˙ÎÌÓ. Ç ÒÎÂ‰‚‡˘ËÚÂ 6 ÏÂÒÂˆ‡ ÔÓ‚ÎËﬂ‚‡ÌÂ ÌÂ ÒÂ ÓÚ˜ËÚ‡ ÔË 2-Ï‡. èË 1 Ô‡ˆËÂÌÚ Â‰ÛÍˆËﬂÚ‡ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ Â Ì‡‰ 50%, ‡ 1 ·ÓÎÂÌ ÓÒÚ‡‚‡ ·ÂÁ ÔËÒÚ˙ÔË. Ç Ò‡‚ÌËÚÂÎÂÌ ÔÎ‡Ì ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÔÓ‚ÎËﬂ‚‡ÌÂÚÓ Ì‡ ‡ÁÎË˜ÌËÚÂ ÚËÔÓ‚Â ÔËÒÚ˙ÔË ‚ ‰ËÌ‡ÏËÍ‡, Ì‡ 6-ÚËﬂ ÏÂÒÂˆ Ô‡‚Ë ‚ÔÂ˜‡ÚÎÂÌËÂ Ì‡È-ÁÌ‡˜ËÚÂÎÌÓ ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ ÍÓÏ·ËÌË‡ÌËÚÂ ÔËÒÚ˙ÔË – ‚ 60% ÓÚ ÒÎÛ˜‡ËÚÂ, Ì‡ ‚ÚÓÓ ÏﬂÒÚÓ Ò‡ Ô‡ˆË‡ÎÌËÚÂ – ‚ 50%, ‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ Ò‡ ÔÓ‚ÎËﬂÌË ‚ 42,86% ÓÚ ÒÎÛ˜‡ËÚÂ. Ç Í‡ﬂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡ ÓÚÌÓÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ ÂÒÔÓÌ‰ÂËÚÂ Ì‡‡ÒÚ‚‡ Ò˙ÓÚ- ‚ÂÚÌÓ ‰Ó 66,67% Á‡ ÍÓÏ·ËÌË‡ÌËÚÂ Ë 57,13% Á‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ Ë Ì‡Ï‡Îﬂ‚‡ ‰Ó 42,85% Á‡ Ô‡ˆË‡ÎÌËÚÂ. (îË„. 7). èÓÒÎÂ‰ﬂ‚‡ÏÂ ‚ ‰ËÌ‡ÏËÍ‡ Ë ÔÓÏﬂÌ‡Ú‡ ‚ ÒÂ‰Ì‡Ú‡ ÏÂÒÂ˜Ì‡ ˜ÂÒÚÓÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ. ç‡È-Ò˙˘ÂÒÚ‚ÂÌ‡ Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ Á‡ Ô‡ˆË‡ÎÌËÚÂ ÔËÒÚ˙ÔË (ÔÓ‚Â˜Â ÓÚ 2 Ô˙ÚË) ÓÚ˜ËÚ‡ÏÂ Ì‡ 6-ÚËﬂ ÏÂÒÂˆ ÓÚ ÎÂ˜ÂÌËÂÚÓ – ÒÂ‰ÌÓ 7,3 ÔËÒÚ˙Ô‡ ÏÂÒÂ˜ÌÓ. Ç Í‡ﬂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡ ÒÂ‰Ì‡Ú‡ ÏÂÒÂ˜Ì‡ ˜ÂÒÚÓÚ‡ Ì‡ ÔËÒÚ˙ÔËÚÂ Â 6,6, Ú.Â ÓÚ˜ËÚ‡ÏÂ Ó·˘‡ Â‰ÛÍˆËﬂ 2,48 Ô˙ÚË. á‡ 180 „ÂÌÂ‡ÎËÁË‡ÌËÚÂ ÔËÒÚ˙ÔË Â‰ÛÍˆËﬂÚ‡ ÔÂÁ Ô˙‚ËÚÂ 6 ÏÂÒÂˆ‡ Â ÏËÌËÏ‡ÎÌ‡ (Ò ÓÍÓÎÓ 3 %) - 3,6 ÔËÒÚ˙Ô‡ ÏÂÒÂ˜ÌÓ Ë ÔÓ-ÁÌ‡˜ËÚÂÎÌ‡ ‚˙‚ ‚ÚÓ‡Ú‡ ÔÓÎÓ‚ËÌ‡ Ì‡ Ô˙‚‡Ú‡ „Ó‰Ë8 Ì‡ ÓÚ ÎÂ˜ÂÌËÂÚÓ – (Ò 39%) - 2,2 ÔËÒÚ˙Ô‡ ÏÂÒÂ˜ÌÓ - îË„Û‡ 8. é·˘Ó ÔË 6 (15,78%) ÓÚ Ì‡·Î˛‰‡‚‡ÌËÚÂ Ô‡ˆËÂÌÚË ÓÚ˜ËÚ‡ÏÂ ÌÂÊÂÎ‡ÌË ÒÚ‡ÌË˜ÌË ‰ÂÈÒÚ‚Ëﬂ - „Î‡‚Ó·ÓÎËÂ Ë ÓÚÔ‡‰Ì‡ÎÓÒÚ – ÔË 2-Ï‡ , Á‡ÔÂÍ – 1, ÒÚÓÏ‡¯ÌÓ ‰‡ÁÌÂÌÂ - 1, „‡‰ÂÌÂ Ë Ò‚ÂÚÓ‚˙ÚÂÊ – 1, ‡„ÂÒË‚ÌÓÒÚ – 1 (Ì‡ÎÓÊËÎÓ ÒÂ Â ‚ÍÎ˛˜‚‡ÌÂ Ì‡ ıÎÓÔÓÚËÍÒÂÌ). íÂ Ò‡ ‰Ó‚ÂÎË ‰Ó ËÁÍÎ˛˜‚‡ÌÂ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚ‡ ÔË 2-Ï‡ ÓÚ ·ÓÎÌËÚÂ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‰Ó·Ëﬂ ÍÎËÌË˜ÂÌ ÂÙÂÍÚ, ‡ ÔË 1 Û˜‡ÒÚÌËÍ LEV Â Á‡ÏÂÌÂÌ Ò ‰Û„ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚ ÔÓ‡‰Ë ÁÌ‡˜ËÚÂÎÌÓ Û‚ÂÎË˜‡‚‡ÌÂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡. éÅëöÜÑÄçÖ èÓ‚Â‰ÂÌÓÚÓ ÓÚ Ì‡Ò ÔÓÛ˜‚‡ÌÂ ‚ÍÎ˛˜‚‡ 65 Ô‡ˆËÂÌÚ‡ Ì‡ ‚˙Á‡ÒÚ ÏÂÊ‰Û 14 Ë 57 „. Ò˙Ò ÒÂ‰Ì‡ ‰‡‚ÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÓÍÓÎÓ 18 „., ÍÓËÚÓ ÔÓÎÛ˜‡‚‡Ú Ô‡ˆË‡ÎÌË, „ÂÌÂ‡ÎËÁË‡ÌË Ë ÍÓÏ·ËÌË‡ÌË ÔËÒÚ˙ÔË ÔË ÔÓ-‚ËÒÓÍ ÓÚÌÓÒËÚÂÎÂÌ ‰ﬂÎ Ì‡ ·ÓÎÌËÚÂ Ò˙Ò ÒËÏÔÚÓÏ‡ÚË˜Ì‡ ÂÔËÎÂÔÒËﬂ (55%) Ë ÍËÔÚÓ„ÂÌÌ‡ ÂÔËÎÂÔÒËﬂ (25%). íÂ Ò‡ ÎÂÍÛ‚‡ÌË ·ÂÁ Á‡‰Ó‚ÓÎËÚÂÎÂÌ ÂÙÂÍÚ Ò 1 ‰Ó 6 ÄÖå, Ì‡ ÙÓÌ‡ Ì‡ ÍÓËÚÓ ÒÂ‰Ì‡Ú‡ ÔËÒÚ˙ÔÌ‡ ˜ÂÒÚÓÚ‡ ÔÂ‰Ë ‚ÍÎ˛˜‚‡ÌÂ Ì‡ LEV Â 4,1 Ô‡ˆË‡ÎÌË ÔËÒÚ˙Ô‡ ÒÂ‰ÏË˜ÌÓ Ë 3,7 „ÂÌÂ‡ÎËÁË‡ÌË ÔËÒÚ˙Ô‡ ÏÂÒÂ˜ÌÓ. 87,69% ÓÚ Û˜‡ÒÚÌËˆËÚÂ Ò‡ Ò ÂÁËÒÚÂÌÚÌ‡ ÙÓÏ‡ Ì‡ ÂÔËÎÂÔÒËﬂ. ç‡È-˜ÂÒÚÓ ËÁÔÓÎÁ‚‡Ì‡Ú‡ ‰ÓÁ‡ LEV Â 1500 mg/d, ÒÎÂ‰‚‡Ì‡ ÓÚ 2000 mg/d, ‡ Ì‡È-ÂÙÂÍÚË‚Ì‡ - 1000 mg/d – ÔÓÒÚË„Ì‡Ú‡ Â Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔËÚÂ >_50% ÔË 100% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÔË ÍÓËÚÓ Â ÔËÎ‡„‡Ì‡. åËÌËÏ‡ÎÌ‡ Â ‡ÁÎËÍ‡Ú‡ Á‡ Ó·˘‡Ú‡ Í‡ÈÌ‡ ÂÙÂÍÚË‚ÌÓÒÚ Ì‡ ‰ÓÁËÚÂ 1500 Ë 2000 mg/d - Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔËÚÂ >_50% Ò˙ÓÚ‚ÂÚÌÓ ÔË 53,33% Ë 52,63%. è‡‚Ë ‚ÔÂ˜‡ÚÎÂÌËÂ ÔÓ-‰Ó·ÓÚÓ ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ „ÂÌÂ‡ÎËÁË‡ÌËÚÂ ÔËÒÚ˙ÔË ÓÚ ‰ÓÁ‡ 1500 mg/d ‚ Ò‡‚ÌÂÌËÂ Ò 2000mg/d. ëÂ‰Ì‡Ú‡ ‰ÓÁ‡ Á‡ ÔÓÛ˜‚‡ÌÂÚÓ Â 1646mg/d ÔË ‰Ë‡Ô‡ÁÓÌ 1000-3000mg/d. Ç ‰Û„Ë ÔÓÛ˜‚‡ÌËﬂ ÔËÎ‡„‡Ì‡Ú‡ ÒÂ‰Ì‡ ‰ÓÁ‡ Á‡ ÔÓÎËÚÂ‡ÔËﬂ ‚‡Ë‡ ÓÚ 1100 ‰Ó 2281 mg/d (9, 17, 22, 39) . èË ÔÓÛ˜‚‡ÌÂ Ì‡ Shorvon Ë Ò˙Ú. 22,8% ÓÚ ·ÓÎÌËÚÂ Ì‡ 1000 mg/d Ò‡ ÓÚ„Ó‚ÓËÎË Ì‡ ÎÂ˜ÂÌËÂÚÓ, ‡ ÔË ‰ÓÁ‡ 2000 mg/d – 31,6% (1). Verdru Ë Ò˙Ú. ÔËÎ‡„‡Ú ‰ÓÁ‡ ÓÚ 3000 mg/d ÔË Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡ÚË˜Ì‡ „ÂÌÂ‡ÎËÁË‡Ì‡ ÂÔËÎÂÔÒËﬂ Ë ÏËÓÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË Ë ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú, ˜Â 58,3% ÓÚ Û˜‡ÒÚÌËˆËÚÂ Ò‡ Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ÔÓÌÂ 50% ÔË ‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ (42). íÂÁË ‰‡ÌÌË ÔÓÚ‚˙Ê‰‡‚‡Ú ‰ÓÁÓÁ‡‚ËÒËÏËﬂ ÂÙÂÍÚ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚ‡. èË ·ÓÎÌËÚÂ Ò Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË ÂÒÔÓÌ‰ÂË Ò‡ 42,85%, Ò „ÂÌÂ‡ÎËÁË‡ÌË – 57,13%, ‡ Ò ÍÓÏ·ËÌË‡ÌË – 66,67%. éÚ Úﬂı Ò˙ÓÚ‚ÂÚÌÓ 7,14% Ë 42,84% Ò‡ Ò Ô˙ÎÌÓ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÍÛÔË‡ÌÂ Ì‡ ÔËÒÚ˙ÔÌËÚÂ ÔÓﬂ‚Ë. ç‡¯ËÚÂ ÂÁÛÎÚ‡ÚË ÒÂ ‰Ó·ÎËÊ‡‚‡Ú ‰Ó ÚÂÁË Ì‡ ‰Û„Ë ‡‚ÚÓË (9, 31, 39, 40) . ÄÎÂÍÒËÂ‚ Ë Ò˙Ú., Í‡ÍÚÓ Ë Cuba Ë Ò˙Ú. ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒıÓ‰ÌË ÂÁÛÎÚ‡ÚË Á‡ ÔÓÒÚËÚÂ Ë ÍÓÏÔÎÂÍÒÌË Ô‡ˆË‡ÎÌË ÔËÒÚ˙ÔË, Í‡ÚÓ ‰ÌËÚÂ ·ÂÁ ÔËÒÚ˙ÔË Ì‡‡ÒÚ‚‡Ú Ò 6,8 Ì‡ ÚÂÚËﬂ ÏÂÒÂˆ ÓÚ ÎÂ˜ÂÌËÂÚÓ Ò LEV (1, 26). á‡Â‰ÌÓ Ò ÚÓ‚‡ Ì‡Ï‡Îﬂ‚‡ ‚ÚÓË˜Ì‡Ú‡ „ÂÌÂ‡ÎËÁ‡ˆËﬂ Ì‡ ÔËÒÚ˙ÔËÚÂ. Ö‰ËÌ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ÏËÓÍÎÓÌË˜ÌË ÔËÒÚ˙ÔË ‚ Ì‡¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ ÓÒÚ‡‚‡ ·ÂÁ ÔËÒÚ˙ÔË, ‡ 1 Â Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ Ì‡‰ 50%. Jongsma Ë Ò˙Ú., Resor Ë Ò˙Ú., Czapinski Ë Ò˙Ú., Krauss Ë Ò˙Ú., Greenhil Ë Ò˙Ú., Gilland Ë Ò˙Ú., Fattouch Ë Ò˙Ú., Hirsch Ë Ò˙Ú., Husain Ë Ò˙Ú, Meencke Ë Ò˙Ú., Mohanraj ÔÓÒÚË„‡Ú Ò‚Ó·Ó‰‡ ÓÚ ÔËÒÚ˙ÔË ÔË Ì‡‰ 60% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò ˛‚ÂÌËÎÌ‡ ÏËÓÍÎÓÌÛÒ ÂÔËÎÂÔÒËﬂ (5, 24, 25), ÔË Ì‡·Î˛‰ÂÌËÂ Ì‡ ê‡¯Â‚‡ Ë Ò˙Ú. 84% ÓÚ ·ÓÎÌËÚÂ Ò Ú‡Í‡‚‡ ÙÓÏ‡ Ì‡ ÂÔËÎÂÔÒËﬂ Ò‡ Ò Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ > _ 50% (7, 8). Ç Í‡ﬂ Ì‡ Ì‡¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ, ÌÂÁ‡‚ËÒËÏÓ ÓÚ ‰ÓÁ‡Ú‡ Ë ÚËÔ‡ Ì‡ ÂÔËÎÂÔÚË˜ÌËÚÂ ÔËÒÚ˙ÔË, ÓÚÌÓÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ ÓÚ„Ó‚ÓËÎËÚÂ Â 57,89%. íÂÁË ÂÁÛÎÚ‡ÚË ÒÂ ‰Ó·ÎËÊ‡‚‡Ú ‰Ó ÓÚ˜ÂÚÂÌ‡Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ ÔË ÔÓÛ˜‚‡ÌËﬂ Ì‡ ÄÎÂÍÒËÂ‚ Ë Ò˙Ú., ÅÓÊËÌÓ‚‡ Ë Ò˙Ú., É‡ÌÂ‚‡ Ë Ò˙Ú., ê‡¯Â‚‡ Ë Ò˙Ú., Allder Ë Ò˙Ú, Pellock Ë Ò˙Ú., Sake Ë Ò˙Ú., Meencke Ë Ò˙Ú., Ben-Menachen Ë Ò˙Ú., ÔË ÍÓËÚÓ ÓÚÌÓÒËÚÂÎÌËﬂÚ ‰ﬂÎ Ì‡ ÂÒÔÓÌ‰ÂËÚÂ ‚‡Ë‡ ÏÂÊ‰Û 29,8 Ë 95,7% (1, 3, 4, 5, 6, 7, 9, 11, 12, 32, 36, 39). êÂ‰ÛÍˆËﬂÚ‡ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ÒÂ Ò˙ÔÓ‚ÓÊ‰‡ Ë Ò˙Ò ÁÌ‡˜ËÚÂÎÌÓ ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ ÂÔËÎÂÔÚË˜Ì‡Ú‡ ‡ÍÚË‚ÌÓÒÚ Ì‡ ÖÖÉ (2, 3, 5). ä‡ÚÓ ÂÁÛÎÚ‡Ú ÓÚ Ì‡¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ ÓÚ˜ËÚ‡ÏÂ Ì‡È·˙Á‡ Ë ‚ËÒÓÍ‡ Í‡ÈÌ‡ Â‰ÛÍˆËﬂ Ì‡ ÔËÒÚ˙ÔÌ‡Ú‡ ˜ÂÒÚÓÚ‡ ÔË Ô‡ˆË‡ÎÌËÚÂ ÔËÒÚ˙ÔË Ë ÔÓ-ÔÓÒÚÂÔÂÌÌÓ Ì‡‡ÒÚ‚‡˘‡ Â‰ÛÍˆËﬂ ÔË „ÂÌÂ‡ÎËÁË‡ÌËÚÂ ÔËÒÚ˙ÔË. Ç‡ÊÂÌ ÔÓÍ‡Á‡ÚÂÎ Á‡ ÂÙËÍ‡ÒÌÓÒÚ Ë ÔÓÌÓÒËÏÓÒÚ Ì‡ Â‰ËÌ ÏÂ‰ËÍ‡ÏÂÌÚ Â ÔÓˆÂÌÚ˙Ú Ô‡ˆËÂÌÚË ÔÓ‰˙ÎÊËÎË ÎÂ˜ÂÌËÂÚÓ Ò ÌÂ„Ó. Ç ıÓ‰‡ Ì‡ Ì‡¯ÂÚÓ Ì‡·Î˛‰ÂÌËÂ Ò‡ÏÓ ÔË 3-Ï‡ ·ÓÎÌË (7,89%) ÒÂ ÔÂÍ˙Ò‚‡ ÔËÎ‡„‡ÌÂÚÓ Ì‡ LEV. èË 1 ÓÚ Úﬂı ÚÓ‚‡ ÒÂ ‰˙ÎÊË Ì‡ Û‚ÂÎË˜ÂÌ‡Ú‡ ÔËÒÚ˙ÔÌ‡ ˜ÂÒÚÓÚ‡. Ç Ò‚ÓÂ ÔÓÛ˜‚‡ÌÂ Soni Ë Ò˙Ú. ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒÚÓÈÌÓÒÚË Ì‡ ÚÓÁË ÔÓÍ‡Á‡ÚÂÎ ÓÚ 70%, ‡ ÔË Ì‡·Î˛‰ÂÌËﬂ Ì‡ ‰Û„Ë ‡‚ÚÓË ÚÓÈ ‚‡Ë‡ ÓÚ 84 ‰Ó 50% ‚ Á‡‚ËÒËÏÓÒÚ ÓÚ ËÁÏËÌ‡ÎÓÚÓ ‚ÂÏÂ ÓÚ ‚ÍÎ˛˜‚‡ÌÂÚÓ (41). Ç ÔÓÛ˜‚‡ÌÂ Ì‡ ê‡¯Â‚‡ Ë Ò˙Ú. – 88,9% ÓÚ Ô‡ˆËÂÌÚËÚÂ ÓÒÚ‡‚‡Ú Ì‡ ÎÂ˜ÂÌËÂ Ò LEV, ÍÓÂÚÓ Â ‰ÓÍ‡Á‡ÚÂÎÒÚ‚Ó Á‡ ÌÂ„Ó‚‡Ú‡ ÂÙÂÍÚË‚ÌÓÒÚ Ë ‰Ó·‡ ÔÓÌÓÒËÏÓÒÚ (6, 26, 40). Marson Ë Ò˙Ú. ‰ÓÍ‡Á‚‡Ú ÓÚÌÓÒËÚÂÎÂÌ ËÒÍ Á‡ ÔÂÍ‡Úﬂ‚‡ÌÂ Ì‡ ÎÂ˜ÂÌËÂÚÓ Ò LEV 1,21 Ò Ì‡È-‰Ó·Ó Ò˙ÓÚÌÓ¯ÂÌËÂ ÂÒÔÓÌ‰ÂË ÔÂÍ˙ÒÌ‡ÎË ÎÂ˜ÂÌËÂÚÓ ‚ Ò‡‚ÌÂÌËÂ Ò OCBZ, Remacemide Ë Zonizamide ÔË Ô‡ˆË‡ÎÌ‡ ÂÔËÎÂÔÒËﬂ (29). èË 6 (15,78%) ÓÚ Ì‡¯ËÚÂ Ô‡ˆËÂÌÚË ÓÚ˜ËÚ‡ÏÂ ÒÚ‡ÌË˜ÌË ÂÙÂÍÚË („Î‡‚Ó·ÓÎËÂ Ë ÓÚÔ‡‰Ì‡ÎÓÒÚ, ÒÏÛ˘ÂÌËﬂ ÓÚ ÒÚ‡Ì‡ Ì‡ ÒÚÓÏ‡¯ÌÓ-˜Â‚ÌËﬂ Ú‡ÍÚ, Ò‚ÂÚÓ‚˙ÚÂÊ, ‡„ÂÒË‚ÌÓÒÚ). íÂ Ò‡ ‰Ó‚ÂÎË ‰Ó ÒÔË‡ÌÂ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚ‡ ÔË 2-Ï‡ ÓÚ Úﬂı. ç‡È-˜ÂÒÚËÚÂ ÌÂÊÂÎ‡ÌË ÒÚ‡ÌË˜ÌË ‰ÂÈÒÚ‚Ëﬂ ÓÔËÒ‡ÌË ‚ ÎËÚÂ‡ÚÛ‡Ú‡ (ÔË ‰Ó 70% ÓÚ Ô‡ˆËÂÌÚËÚÂ) Ò‡: ÛÏÓ‡, Á‡Ï‡È‚‡ÌÂ, ÒÓÏÌÓÎÂÌÚÌÓÒÚ, ‡Ú‡ÍÒËﬂ, ‡ÎÂ„Ë˜Ì‡ Â‡ÍˆËﬂ, Á‡„Û·‡ Ì‡ ‡ÔÂÚËÚ, ÔÓ‚Ë¯‡‚‡ÌÂ Ì‡ ÚÂ„ÎÓÚÓ, ‰Ë‡Ëﬂ, ·ÂÁÒ˙ÌËÂ, ËÌÓÙ‡ËÌ„ËÚ, Ì‡Û¯ÂÌËﬂ Ì‡ Ì‡ÒÚÓÂÌËÂÚÓ Ë ÔÓ‚Â‰ÂÌËÂÚÓ, ‡Á‰‡ÁÌËÚÂÎÌÓÒÚ, ‡„ÂÒË‚ÌÓÒÚ, ‰ÂÔÂÒËﬂ, ÚÂ‚ÓÊÌÓÒÚ, ÔÒËıÓÚË˜ÌË ÔÓﬂ‚Ë (1, 2, 9, 10, 12, 14, 17, 21, 22, 23, 28, 31, 37, 38, 40). ç‡È˜ÂÒÚÓ ÚÂ Ò‡ ÎÂÍÓ ËÁ‡ÁÂÌË Ë ·˙ÁÓ ÔÂıÓ‰ÌË (1, 36). é·ËÍÌÓ‚ÂÌÓ Ò‡ Ò‚˙Á‡ÌË Ò ‚ËÒÓÍ‡ Ì‡˜‡ÎÌ‡ ‰ÓÁ‡ (23). íÂ ÒÚ‡‚‡Ú ÔË˜ËÌ‡ Á‡ ÔÂÍ‡Úﬂ‚‡ÌÂ Ì‡ ÎÂ˜ÂÌËÂÚÓ ‚ ‰Ó 14,4% ÓÚ ÒÎÛ˜‡ËÚÂ Ë Ò‡ ÔÓ-ÒÎ‡·Ó ÔÓﬂ‚ÂÌË ÔË ÔÓ-·‡‚ÌÓ ‚˙‚ÂÊ‰‡ÌÂ Ì‡ ÏÂ‰ËÍ‡ÏÂÌÚ‡ (14). Ç Á‡ÍÎ˛˜ÂÌËÂ LEV Â ÏÂ‰ËÍ‡ÏÂÌÚ Ò ‚ËÒÓÍ‡ Ë ‰˙Î„ÓÒÓ˜Ì‡ ÒÚ‡·ËÎÌ‡ ÂÙËÍ‡ÒÌÓÒÚ ÔË Ô‡ˆË‡ÎÌË Ë „ÂÌÂ‡ÎËÁË- 181 ‡ÌË ÂÔËÎÂÔÒËË. íÓÈ Â ‰Ó·Â ÔÓÌÓÒËÏ, Ò ÎÂÍË Ë ‚ÂÏÂÌÌË ÌÂÊÂÎ‡ÌË ÒÚ‡ÌË˜ÌË ‰ÂÈÒÚ‚Ëﬂ, ﬂ‰ÍÓ ‰Ó‚ÂÊ‰‡˘Ë ‰Ó ÔÂÍ˙Ò‚‡ÌÂ Ì‡ ÎÂ˜ÂÌËÂÚÓ. ãàíÖêÄíìêÄ 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. ÄÎÂÍÒËÂ‚, Ä., Ä. ä‡ÔÂÎﬂÌ, Ñ. åËÌ˜Â‚, ã. ï‡‚ÂÁÓ‚‡, å. ê‡¯Â‚‡, ç. ÑÂÎÂ‚‡, è. âÓ‰‡ÌÓ‚‡, è. òÓÚÂÍÓ‚, è. ëÚ‡ÏÂÌÓ‚‡, ê. ä‡ÎÔ‡˜ÍË, ë. ñÂÍÓ‚, ë. É‡ÌÂ‚‡, Ç. âÓÎÓ‚‡, èÓÛ˜‚‡ÌÂ Ì‡ ‰˙Î„ÓÚ‡ÈÌËﬂ ÂÙÂÍÚ Ì‡ Levetiracetam Í‡ÚÓ ‰Ó·‡‚˙˜Ì‡ ÚÂ‡ÔËﬂ ÔË ÂÙ‡ÍÚÂÌË Ô‡ˆË‡ÎÌË ÂÔËÂÔÒËË, Å˙Î„‡ÒÍ‡ ÌÂ‚ÓÎÓ„Ëﬂ, 2004, ÚÓÏ 4, ·. 3, 131-135. ÅÓÊËÌÓ‚‡, Ç., è. ÑËÏÓ‚‡, Levetiracetam (Keppra) – ÌÓ‚ ¯ËÓÍÓÒÔÂÍÚ˙ÂÌ ‡ÌÚËÍÓÌ‚ÛÎÒ‡ÌÚ ‚ ‰ÂÚÒÍ‡Ú‡ ‚˙Á‡ÒÚ – ÂÙÂÍÚË‚ÌÓÒÚ, ÔÓÌÓÒËÏÓÒÚ, ËÌ‰ËÍ‡ˆËË, ç‡ˆËÓÌ‡ÎÌ‡ ÍÓÌÙÂÂÌˆËﬂ ÔÓ ‰ÂÚÒÍ‡ ÌÂ‚ÓÎÓ„Ëﬂ, ÔÒËıË‡ÚËﬂ Ë ÔÒËıÓÎÓ„Ëﬂ Ì‡ ‡Á‚ËÚËÂÚÓ, 26-28.10.2006, 23-25. ÅÓÊËÌÓ‚‡, Ç., è. 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Sadek, A., A. Fix, J. French, Levetiracetam-related behavioural adverse events: a post-marketing study, Epilepsia, 2002, 43 (8), 151. 39. Sake, J., M. De Decker, G. Janssen, Efficacy of levetiracetam: an open label assessment, Epilepsia, 2002, 43 (8), 117. 40. Smith, D., A. Nicolson, Prospective analysis of the outcome of levetiracetam treatment, Epilepsia, 2002, 43 (8), 117. 41. Soni, S., M. O’Rourke, Levetiracetam in learning disability adults with tonicclonic seizures, Epilepsia, 2002, 43 (8), 181. 42. Verdru, P., A. Wajgt, J. Schiemann Delgado, S. Noachtar, Efficacy and Safety of Levetiracetam 3000 mg/d as Adjunctive Treatment in adolescents and Adults Suffering from Idiopathic Generalized Epilepsy with Myoclonic seizures, presented in Paris, 26-th ICG Proceedings. ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology 183 éË„ËÌ‡ÎÌË ÒÚ‡ÚËË çÄóÄãçàüí ÑÇàÉÄíÖãÖç ëàåèíéå ÇãàüÖ Çöêïì äéÉçàíàÇçàü ëèÄÑ èêà ãéçÉàíìÑàçÄãçé èêéëãÖÑÖçà èÄñàÖçíà ë èÄêäàçëéçéÇÄ ÅéãÖëí ü. ÜÂÎÂ‚, å. ê‡È˜Â‚‡, å. èÂÚÓ‚‡, ã. í‡ÈÍÓ‚ ìåÅÄã “ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, åÂ‰ËˆËÌÒÍË ìÌË‚ÂÒËÚÂÚ, ëÓÙËﬂ SUMMARY INITIAL MOTOR SYMPTOM INFLUENCES COGNITIVE DECLINE IN A LONGITUDINALLY FOLLOWED PATIENTS WITH PARKINSON'S DISEASE Y.Zhelev, M. Raycheva, M. Petrova, L. Traykov University Hospital 'Alexandrovska', Neurology, Sofia, Bulgaria Parkinson's disease (PD) is often associated with cognitive impairment. Some patients exhibit severe mental deterioration over time while others show subtle cognitive decrease. Research has shown that several clinical disease parameters may correlate with cognitive deterioration. The present study examined the association between type of motor symptom at disease onset and progression of cognitive decline in a longitudinally followed group of patients with idiopathic Parkinson's disease. We investigated 27 nondemented patients with PD. All subjects underwent a comprehensive neuropsychological assessment including tests of episodic memory, language, constructional praxis, attention and executive functions. Information concerning type of initial motor symptom was obtained from subject report during clinical interview and from medical documentation. Subjects were divided into two groups - having tremor (n=15) and having bradykinesia/rigidity (n=12) as initial motor presentation of disease. We reassessed PD subjects 20±9 months later. Bradykinesia/rigidity group demonstrated significant deterioration in conceptualization subscale of Mattis Dementia Rating Scale and Digit Span Forward. Tremor group do not exhibit significant decries in performance of neuropsychological tests. In this group initial motor symptom determines progression of cognitive deterioration. A decrease in attention/executive tests performance was found in the bradykinesia/rigidity group. Tremor group showed cognitive stability over the follow-up period. The type of motor symptom at disease onset might predetermine cognitive decline in PD. êÖáûåÖ è‡ÍËÌÒÓÌÓ‚‡Ú‡ ·ÓÎÂÒÚ (èÅ) ˜ÂÒÚÓ Â Ò˙ÔÓ‚Ó‰ÂÌ‡ ÓÚ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ. çﬂÍÓË ÓÚ Ô‡ˆËÂÌÚËÚÂ ÔÓÍ‡Á‚‡Ú ÚÂÊ˙Í ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ ‚˙‚ ‚ÂÏÂÚÓ ‰ÓÍ‡ÚÓ ‰Û„Ë ÔÓﬂ‚‡‚‡Ú Ò‡ÏÓ ÎÂÍË ÍÓ„ÌËÚË‚ÌË ÒÏÛ˘ÂÌËﬂ Á‡ Ò˙˘Ëﬂ ÔÂËÓ‰ ÓÚ ‚ÂÏÂ. ç‡ÚÛÔ‡ÌËÚÂ ‰Ó ÏÓÏÂÌÚ‡ ËÁÒÎÂ‰Ó‚‡ÚÂÎÒÍË ‰‡ÌÌË ÔÓÍ‡Á‚‡Ú Ì‡ÎË˜ËÂÚÓ Ì‡ ÍÓÂÎ‡ˆËﬂ ÏÂÊ‰Û ÌﬂÍÓË ÓÚ ÍÎËÌË˜ÌËÚÂ Ô‡‡ÏÂÚË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰. ç‡ÒÚÓﬂ˘ÂÚÓ ÔÓÛ˜‚‡ÌÂ ËÁÒÎÂ‰‚‡ ‚˙ÁÍ‡Ú‡ ÏÂÊ‰Û ÚËÔ‡ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÔÓ„ÂÒËﬂÚ‡ Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰ ‚ ÎÓÌ„ËÚÛ‰ËÌ‡ÎÌÓ ÔÓÒÎÂ‰ÂÌ‡ „ÛÔ‡ Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡ÚË˜Ì‡ è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ. àÁÒÎÂ‰‚‡ÌË Ò‡ 27 ÌÂ‰ÂÏÂÌÚÌË ÎËˆ‡ Ò èÅ. àÁÔÓÎÁ‚‡Ì‡ Â ÔÓ‰Ó·Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜Ì‡ ·‡ÚÂËﬂ ‚ÍÎ˛˜‚‡˘‡ ÚÂÒÚÓ‚Â Á‡ Ô‡ÏÂÚ, ÂÁËÍ, ÍÓÌÒÚÛÍÚË‚ÂÌ Ô‡ÍÒËÒ, ‚ÌËÏ‡ÌËÂ Ë ÂÍÁÂÍÛÚË‚ÌË ÙÛÌÍˆËË. àÌÙÓÏ‡ˆËﬂÚ‡ ÓÚÌÓÒÌÓ ÚËÔ‡ ‰Â·˛ÚÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Â ÔÓÎÛ˜ÂÌ‡ ÓÚ ÍÎËÌË˜ÌÓ ËÌÚÂ‚˛ Ò Ô‡ˆËÂÌÚ‡ Ë Â ÔÓÚ‚˙‰ÂÌ‡ ÓÚ Ì‡ÎË˜Ì‡Ú‡ ÏÂ‰ËˆËÌÒÍ‡ ‰ÓÍÛÏÂÌÚ‡ˆËﬂ. ãËˆ‡Ú‡ Ò‡ ‡Á‰ÂÎÂÌË Ì‡ ‰‚Â „ÛÔË – ËÏ‡˘Ë ÚÂÏÓ Ë (n=15) Ë ËÏ‡˘Ë ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ (n=12) Í‡ÚÓ Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. è‡ˆËÂÌÚËÚÂ Ò‡ ÔÓ‰ÎÓÊÂÌË Ì‡ ÔÓ‚ÚÓÌÓ ÚÂÒÚÛ‚‡ÌÂ Ò˙Ò Ò˙˘‡Ú‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜Ì‡ ·‡ÚÂËﬂ ÒÎÂ‰ 20±9 ÏÂÒÂˆ‡. ÉÛÔ‡Ú‡ Ò ‰Â·˛Ú ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ ÔÓÍ‡Á‡ ÁÌ‡˜ËÏ ÒÔ‡‰ ‚ ÔÓ‰ÒÍ‡Î‡Ú‡ Á‡ ÍÓÌˆÂÔÚÛ‡ÎËÁ‡ˆËﬂ ÓÚ Mattis Dementia Rating Scale Í‡ÍÚÓ Ë Digit Span Forward. ÉÛÔ‡Ú‡ Ò ‰Â·˛Ú ÚÂÏÓ ÌÂ ÔÓÍ‡Á‡ ÁÌ‡˜ËÏ ÒÔ‡‰ Ì‡ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÔËÎÓÊÂÌËÚÂ ÚÂÒÚÓ‚Â. Ç ËÁÒÎÂ‰‚‡Ì‡Ú‡ „ÛÔ‡ Ô‡ˆËÂÌÚË Ì‡˜‡ÎÌËﬂ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÓÔÂ‰ÂÎﬂ ÔÓ„ÂÒËﬂÚ‡ Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰. ìÒÚ‡ÌÓ‚ﬂ‚‡ ÒÂ ÒÔ‡‰ ‚ ÚÂÒÚÓ‚ÂÚÂ ÓˆÂÌﬂ‚‡˘Ë ‚ÌËÏ‡ÌËÂ/ÂÍÁÂÍÛÚË‚ÌË ÙÛÌÍˆËË Á‡ „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÓ ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ. ÉÛÔ‡Ú‡ Ò Ì‡˜‡ÎÓ ÚÂÏÓ ÔÓÍ‡Á‡ ÒÚ‡·ËÎÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÍÓ„ÌËˆËﬂÚ‡ Á‡ ËÁÒÎÂ‰‚‡ÌËﬂ ÔÂËÓ‰ ÓÚ ‚ÂÏÂ. íËÔ˙Ú ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ‚ Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ‚ÂÓﬂÚÌÓ ÏÓÊÂ ‰‡ ÔÂ‰ÓÔÂ‰ÂÎË ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰ ÔË èÅ. ÇöÇÖÑÖçàÖ äÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ Ò˙Ô˙ÚÒÚ‚‡Ú è‡ÍËÌÒÓÌÓ‚‡Ú‡ ·ÓÎÂÒÚ (èÅ). èÂˆËÁÌÓÚÓ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌÓ ËÁÒÎÂ‰‚‡ÌÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÒÏÛ˘ÂÌËﬂ ‚ ÍÓ„ÌËˆËﬂÚ‡ ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ó˘Â ‚ ‡ÌÌËÚÂ ÂÚ‡ÔË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. çﬂÍÓÈ ·ÓÎÌË ·˙ÁÓ ‡Á‚Ë‚‡Ú ËÎË Á‡‰˙Î·Ó˜‡‚‡Ú Ì‡ÎË˜ÌËﬂ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ, ‰ÓÍ‡ÚÓ ‰Û„Ë ÔÓÍ‡Á‚‡Ú ÓÚÌÓÒËÚÂÎÌ‡ ÒÚ‡·ËÎÌÓÒÚ ‚˙‚ ‚ÂÏÂÚÓ. ÇÎËﬂÌËÂÚÓ Ì‡ ÌﬂÍÓË ÍÎËÌË˜ÌË Ô‡‡ÏÂÚË ‚˙ıÛ ıÓ‰‡, ÔÓ„ÂÒËﬂÚ‡ Ë ÔÓ„ÌÓÁ‡Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â ËÁÛ˜‡‚‡ÌÓ ÓÚ Â‰Ëˆ‡ ‡‚ÚÓË. èÓ‰Ó·ÂÌ Â ÔÓ‰ıÓ‰˙Ú Ì‡ÒÓ˜ÂÌ Í˙Ï Ú˙ÒÂÌÂ Ì‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ‰ÓÏËÌË‡˘ËÚÂ ÏÓÚÓÌË ÒËÏÔÚÓÏË Ë ÚÂÊÂÒÚÚ‡ Ì‡ ÏÌÂÒÚË˜ÌËÚÂ Ì‡Û¯ÂÌËﬂ. Ç ÔÓ-„ÓÎﬂÏ‡Ú‡ ÒË ˜‡ÒÚ ÔÓÛ˜‚‡ÌËﬂ ‚ Ú‡ÁË Ì‡ÒÓÍ‡ ÔÓÒÓ˜‚‡Ú Ì‡ÎË˜ËÂÚÓ Ì‡ ÍÓÂÎ‡ˆËﬂ ÏÂÊ‰Û ·‡‰ËÍËÌÂÁËﬂÚ‡ Ë/ËÎË Ë„Ë‰ÌÓÒÚÚ‡ Ë ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ, ‰ÓÍ‡ÚÓ Á‡ ÚÂÏÓ˙Ú Ú‡Í‡‚‡ ‡ÒÓˆË‡ˆËﬂ ËÎË ÌÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ËÎË ÌÂ„Ó‚ÓÚÓ ‰ÓÏËÌË‡ÌÂ Ì‡ÒÓ˜‚‡ Í˙Ï ÓÚÌÓÒËÚÂÎÌÓ ÔÓ-ÌËÒ˙Í ËÒÍ Á‡ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ (1), (2), (3), (4), (5), (6), (7), (8). Ç ÔÓ-„ÓÎﬂÏ‡Ú‡ ÒË ˜‡ÒÚ ÔÓÛ˜‚‡ÌËﬂÚ‡ ‡ÍˆÂÌÚË‡Ú ‚˙ıÛ Ú˙ÒÂÌÂÚÓ Ì‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ‡ÍÚÛ‡ÎÌËÚÂ ÍÎËÌË˜ÌË ÏÓÚÓÌË ÒËÏÔÚÓÏË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÓÒÓ·ÂÌÓÒÚËÚÂ Ë ÔÓ„ÂÒËﬂÚ‡ Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌËÚÂ Ì‡Û¯ÂÌËﬂ. íÓÁË ÔÓ‰ıÓ‰ ËÏ‡ ÌﬂÍÓË ÌÂ‰ÓÒÚ‡Ú˙ˆË. í‡Í‡ Ì‡ÔËÏÂ, Ò ÔÓ„ÂÒËﬂ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÒÚ‡‚‡ ‚ÒÂ ÔÓ-ÚÛ‰ÌÓ ‰‡ ÒÂ ÓÔÂ‰ÂÎË ÍÓÈ ÒËÏÔÚÓÏ Â ‰ÓÏËÌË‡˘. ëÎÂ‰ Á‡ÔÓ˜‚‡ÌÂ Ì‡ ÎÂ˜ÂÌËÚÓ Ú‡ÁË ÔÂˆÂÌÍ‡ ÒÂ Á‡ÚÛ‰Ìﬂ‚‡ ‰ÓÔ˙ÎÌËÚÂÎÌÓ ÔÓ‡‰Ë ÌÂÔÓÔÓˆËÓÌ‡ÎÌÓÚÓ ÚÂ‡ÔÂ‚ÚË˜ÌÓ ÔÓ‚ÎËﬂ‚‡ÌÂ Ì‡ ÓÚ‰ÂÎÌËÚÂ ÒËÏÔÚÓÏË. ë˙˘Ó Ú‡Í‡ Úﬂ·‚‡ ‰‡ ÒÂ ËÏ‡ ‚ ÔÂ‰‚Ë‰, ˜Â ÒÛÏ‡Ì‡ ÓˆÂÌÍ‡, ÍÓﬂÚÓ ÒÂ ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÔÓÎÛ˜‡‚‡ ÔË ËÁÔÓÎÁ‚‡ÌÂÚÓ Ì‡ Ò˙ÓÚ‚ÂÚÌËÚÂ ÒÍ‡ÎË Â ÌÂËÌÙÓÏ‡ÚË‚Ì‡ Á‡ ÚÂÊÂÒÚÚ‡ Ì‡ ÓÚ‰ÂÎÌËﬂ ÒËÏÔÚÓÏ, àÁÛ˜‡‚‡ÌÂÚÓ Ì‡ ‚˙ÁÍ‡Ú‡ ÏÂÊ‰Û ÍÓ„ÌËˆËﬂÚ‡ Ë Ì‡˜‡ÎÌËﬂ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ, ‡ ÌÂ ‡ÍÚÛ‡ÎÌËÚÂ ÏÓÚÓÌË ÒËÏÔÚÓÏË Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, ‚ÂÓﬂÚÌÓ Â Ì‡‰ÂÊ‰ÂÌ ÔÓ‰ıÓ‰ ÔÓ‡‰Ë Á‡Ó·ËÍ‡ÎﬂÌÂ Ì‡ ËÁ·ÓÂÌËÚÂ ‚˙ÁÏÓÊÌÓÒÚË Á‡ ÌÂÍÓÂÍÚÌ‡ ÓˆÂÌÍ‡ Ì‡ ÏÓÚÓÌ‡Ú‡ ÒËÏÔÚÓÏ‡ÚËÍ‡. åÓÊÂ ‰‡ ÒÂ ÔÂ‰ÔÓÎÓÊË, ˜Â Ì‡˜‡ÎÌËﬂÚ ‰‚Ë„‡ÚÂÎÂÌ ÒËÏÔÚÓÏ ·Ë ÏÓ„˙Î ‰‡ ËÏ‡ ÁÌ‡˜ËÏ‡ ÔÓ„ÌÓÒÚË˜Ì‡ ÒÚÓÈÌÓÒÚ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰, ÓÒÓ·ÂÌÓ ‚ Ô˙‚ËÚÂ ÌﬂÍÓÎÍÓ „Ó‰ËÌË ÒÎÂ‰ ÔÓÒÚ‡‚ﬂÌÂÚÓ Ì‡ ‰Ë‡„ÌÓÁ‡Ú‡. ñÖã ñÂÎÚ‡ Ì‡ ÔÓÛ˜‚‡ÌÂÚÓ Â ‰‡ ËÁÒÎÂ‰‚‡ ‚˙ÁÍ‡Ú‡ ÏÂÊ‰Û ÚËÔ‡ Ì‡ ÏÓÚÓÌËﬂ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÔÓÒÎÂ‰‚‡˘Ëﬂ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰ ‚ ÎÓÌ„ËÚÛ‰ËÌ‡ÎÌÓ ÔÓÒÎÂ‰ÂÌ‡ „ÛÔ‡ ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË Ò Ë‰ËÓÔ‡ÚË˜Ì‡ èÅ. äéçíàçÉÖçí à åÖíéÑàäÄ èÓÛ˜‚‡ÌÂÚÓ Â ÔÓ‚Â‰ÂÌÓ ‚ ÍÎËÌËÍ‡Ú‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ Ì‡ ìåÅÄã "ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡" ëÓÙËﬂ ÔË 27 ·ÓÎÌË Ò èÅ. èÓ‰·‡ÌË Ò‡ ÎËˆ‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÌÂ ÔÓ‚Â˜Â ÓÚ 5 „Ó‰ËÌË. ÇÒË˜ÍË Û˜‡ÒÚÌËˆË Ò‡ ÔÓ‰Ó·ÌÓ ÍÎËÌË˜ÌÓ Ë ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌÓ ËÁÒÎÂ‰‚‡ÌË ÔË ‚ÍÎ˛˜‚‡ÌÂ ‚ ÔÓÛ˜‚‡ÌÂÚÓ Ë ÔÓ‚ÚÓÌÓ Ò‡ ÚÂÒÚÛ‚‡ÌË ÒÎÂ‰ ÒÂ‰ÌÓ 20,3 ± 9,4 ÏÂÒÂˆ‡. Ç˙Á ÓÒÌÓ‚‡ Ì‡ ÏÓÚÓÌËﬂ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ò‡ ÙÓÏË‡ÌË ‰‚Â „ÛÔË – Ô‡ˆËÂÌÚË ËÏ‡˘Ë ·‡‡‰ËÍËÌÂÁËﬂ Ë/ËÎË Ë„Ë‰ÌÓÒÚ Ë „ÛÔ‡ Ò Â‰ËÌÒÚ‚ÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ. ÑË‡„ÌÓÁ‡Ú‡ Â ÔÓÒÚ‡‚ﬂÌ‡ ‚˙Á ÓÒÌÓ‚‡ Ì‡ ÔÓ‰Ó·Ì‡ ‡Ì‡ÏÌÂÁ‡, ÒÓÏ‡ÚË˜ÂÌ Ë ÌÂ‚ÓÎÓ„Ë˜ÂÌ ÔÂ„ÎÂ‰, Î‡·Ó‡ÚÓÌË Ë ÌÂ‚ÓËÁÓ·‡Áﬂ‚‡˘Ë ËÁÒÎÂ‰‚‡ÌËﬂ (ÍÓÏÔ˛Ú˙Ì‡ ÚÓÏÓ„‡ÙËﬂ ËÎË Ï‡„ÌËÚÌÓ ÂÁÓÌ‡ÌÒÌ‡ ÚÓÏÓ„‡ÙËﬂ Ì‡ ÏÓÁ˙Í‡), Í‡ÍÚÓ Ë ÔÓ‰Ó·Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜Ì‡ ÓˆÂÌÍ‡. çÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ ‚ÍÎ˛˜‚‡ ÒÍËÌËÌ„Ó‚Ë ÒÍ‡ÎË Ë ÔÓ‰Ó·Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜Ì‡ ·‡ÚÂËﬂ ÓÚ ÚÂÒÚÓ‚Â Ë Ò˙Í‡ÚÂÌË ‚ÂÒËË Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌË ÏÂÚÓ‰ËÍË. é·˘ËﬂÚ ÍÓ„ÌËÚË‚ÂÌ Í‡Ô‡ˆËÚÂÚ Â ËÁÒÎÂ‰‚‡Ì Ò: Mini Mental State (MMS) Ë Dementia Rating Scale (DRS). è‡ÏÂÚÚ‡ Â ËÁÒÎÂ‰‚‡Ì‡ Ò: Digit Span (forward) Á‡ ÓˆÂÌÍ‡ Ì‡ Í‡ÚÍÓÒÓ˜Ì‡ ‚Â·‡ÎÌ‡ Ô‡ÏÂÚ, Í‡ÍÚÓ Ë Ò Buschke Free and Cued Selective Reminding Test (FCSRT) Á‡ ÓˆÂÌÍ‡ Ì‡ ‚Â·‡ÎÌ‡ ÂÔËÁÓ‰Ë˜Ì‡ Ô‡ÏÂÚ ˜ÂÁ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ Ò‚Ó·Ó‰ÌÓÚÓ ÔËÔÓÏÌﬂÌÂ, Ó·˘ÓÚÓ (Ò‚Ó·Ó‰ÌÓ Ë ÛÎÂÒÌÂÌÓ) ÔËÔÓÏÌﬂÌÂ Ë ‡ÁÔÓÁÌ‡‚‡ÌÂÚÓ. ÇÌËÏ‡ÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË Ò‡ ËÁÒÎÂ‰‚‡ÌË Ò: Trail Making Test - A (TMT-A) Ë Ç (TMT-B); Modified Card Sorting Test (MCST); ÇÂ·‡ÎÌ‡ ÙÎÛÂÌÚÌÓÒÚ (ÙÓÌÂÏÌ‡), Ë Stroop Test. êÂ˜Ú‡ Â ËÁÒÎÂ‰‚‡Ì‡ Ò Í‡ÚÍ‡ ‚ÂÒËﬂ Ì‡ Boston Naming Test (BNT) Ë c ÇÂ·‡ÎÌ‡ ÙÎÛË‰ÌÓÒÚ (Í‡ÚÂ„ÓË‡ÎÌ‡). ê‡ÁÎËÍËÚÂ ÏÂÊ‰Û „ÛÔËÚÂ ‚˙‚ ‚˙Á‡ÒÚÚ‡, Ó·‡ÁÓ‚‡ÌËÂÚÓ, ÂÁÛÎÚ‡ÚËÚÂ ÓÚ MMS, DRS Ë ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌËÚÂ ÚÂÒÚÓ‚Â Ò‡ ÓˆÂÌﬂ‚‡ÌË Ò ÔÓÏÓ˘ Ú‡ Ì‡ non-paired ttest. á‡ ÓˆÂÌÍ‡ ÒÚÂÔÂÌÚ‡ Ì‡ ÔÓÏﬂÌ‡ ‚ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ Â ËÁÔÓÎÁ‚‡Ì paired ttest, ‚ÁÂÏ‡˘ ‚ ÔÂ‰‚Ë‰ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ Ô˙‚ÓÚÓ Ë ‚ÚÓÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ. êÖáìãíÄíà ãËÔÒ‚‡Ú ÒÚ‡ÚËÒÚË˜ÂÒÍË ÁÌ‡˜ËÏË ‡ÁÎËÍË ÏÂÊ‰Û „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÓÚÓÏ ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ (èÅÅ/ê) Ë „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ (èÅ-í) ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÔÓÏÂÌÎË‚ËÚÂ ‚˙Á‡ÒÚ (èÅ-Å/ê - 66,9 (9,3), èÅ-í - 66,5 (6,3)), Ó·‡ÁÓ‚‡ÌËÂ (èÅ-Å/ê - 14,3 (2,9), èÅ-í - 12,9 (3,0)), ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ 184 (èÅ-Å/ê - 2,6 (1,6), èÅ-í - 2,5 (1,3)) Í‡ÍÚÓ Ë ·‡ÁËÒÌËÚÂ ÂÁÛÎÚ‡ÚË ÓÚ ÒÍ‡ÎËÚÂ Á‡ „ÎÓ·‡ÎÌÓ ÍÓ„ÌËÚË‚ÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ MMSE (èÅ-Å/ê - 27,9 (1,6), èÅ-í - 26,6 (2,0)), DRS (èÅ-Å/ê - 135,7 (4,3), èÅ-í - 131,9 (7,6)). çÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒÚ‡ÚËÒÚË˜ÂÒÍË ÁÌ‡˜ËÏË ‡ÁÎËÍË Ë ‚ ·‡ÁËÒÌËÚÂ ÂÁÛÎÚ‡ÚË ÓÚ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌËÚÂ ÚÂÒÚÓ‚Â ÏÂÊ‰Û ‰‚ÂÚÂ „ÛÔË. êÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÓˆÂÌﬂ‚‡˘Ëﬂ ‡ÍÚÛ‡ÎÌËﬂ Ô‡ÏÂÚÓ‚ Í‡Ô‡ˆËÚÂÚ ÚÂÒÚ (FCSRT) Ò‡ ËÁÓ·‡ÁÂÌË Í‡ÚÓ ÔÓˆÂÌÚ Ì‡ ÒÔ‡‰ ÒÔﬂÏÓ ·‡ÁËÒÌÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ (ÙË„. 1). Ñ‡ÌÌËÚÂ ÓÚ ‰Û„ËÚÂ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌË ÚÂÒÚÓ‚Â Ò‡ ÔÂ‰ÒÚ‡‚ÂÌË Ì‡ Ú‡·ÎËˆ‡ 1. Ç ‰‚ÂÚÂ „ÛÔË ÌÂ ÒÂ Ì‡·Î˛‰‡‚‡ ÁÌ‡˜ËÏ ÒÔ‡‰ ‚ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÚÂÒÚÓ‚ÂÚÂ Á‡ Ó·˘Ó ÍÓ„ÌËÚË‚ÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ Í‡ÍÚÓ Ë ‚ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÚÂÒÚ‡ Á‡ ‚Â·‡ÎÌ‡ ÂÔËÁÓ‰Ë˜Ì‡ Ô‡ÏÂÚ (FCSRT). èÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÚÂÒÚÓ‚ÂÚÂ, ÍÓËÚÓ ËÁÒÎÂ‰‚‡Ú ‡ÁÎË˜ÌË ‡ÒÔÂÍÚË Ì‡ ‚ÌËÏ‡ÌËÂÚÓ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË, ÒÂ Ì‡·Î˛‰‡‚‡Ú ÒÚ‡ÚËÒÚË˜ÂÒÍË ÁÌ‡˜ËÏË ‡ÁÎËÍË ÔË Digit Span Forward Ë ÂÍÁÂÍÛÚË‚Ì‡Ú‡ ÔÓ‰ÒÍ‡Î‡ Ì‡ DRS (ÒÔÓÒÓ·ÌÓÒÚ Á‡ ÙÓÏË‡ÌÂ Ì‡ ÍÓÌˆÂÔˆËË) Â‰ËÌÒÚ‚ÂÌÓ ‚ „ÛÔ‡Ú‡ ËÏ‡˘‡ ‰Â·˛ÚÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ·‡‰ËÍËÌÂÁËﬂ Ë/ËÎË Ë„Ë‰ÌÓÒÚ. ÉÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ ÔÓÍ‡Á‚‡ ÒÚ‡·ËÎÌÓÒÚ ‚˙‚ ‚ÂÏÓÚÓ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÔËÎÓÊÂÌËÚÂ ÍÓ„ÌËÚË‚ÌË ÒÍ‡ÎË. éÅëöÜÑÄçÖ ÄÌ‡ÎËÁ˙Ú Ì‡ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ËÁÒÎÂ‰‚‡ÌËÚÂ „ÛÔË ÔÓÍ‡Á‚‡Ú, ˜Â ÏÓÚÓÌËﬂÚ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ‚ÂÓﬂÚÌÓ ËÏ‡ ÔÓ„ÌÓÒÚË˜ÌÓ ÁÌ‡˜ÂÌËÂ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÔÓÒÎÂ‰‚‡˘Ëﬂ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰. è‡ˆËÂÌÚË, ˜ËËÚÓ Ô˙‚Ë ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â ·‡‰ËÍËÌÂÁËﬂ Ë/ËÎË Ë„Ë‰ÌÓÒÚ, ËÏ‡Ú ÚÂÌ‰ÂÌˆËﬂ Á‡ ÒÚ‡ÚËÒÚË˜ÂÒÍË ÁÌ‡˜ËÏ ÒÔ‡‰ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÌﬂÍÓË ÒÙÂË Ì‡ ÍÓ„í‡·ÎËˆ‡ 1. êÂÁÛÎÚ‡ÚË ÓÚ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌÓÚÓ ÚÂÒÚÛ‚‡ÌÂ ÔË Ô˙‚ÓÚÓ (1) Ë ÔË ÔÓ‚ÚÓÌÓÚÓ (2) ËÁÒÎÂ‰‚‡ÌÂ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ò è‡ÍËÌÒÓÌÓ‚‡ ·ÓÎÂÒÚ Ò ‰Â·˛Ú ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ (èÅ-Å/ê) Ë ÚÂÏÓ (èÅ-í) íÂÒÚÓ‚Â èÅ-Å/ê 1 93,3 (59,1) 172,5 (81,6) 29,3 (13,8 ) 4,8 (2,0) 37,3 (0,7) 9,6 (3,8) (n=12) 2 97,6 (53,0) 211,5 (92,5) 36,1 (12,2) 4,9 (1,9) 34,5 (3,2)* 9,3 (4,6) èÅ-í (n=15) 1 2 77,6 (37,4) 85,9 (42,6) 201,9 (86,8) 223,6 (96,4) 28,8 (11,8) 30,1 (12,5) 4,9 (1,6) 4,6 (2,2) 36,2 (3,3) 36,4 (2,3) 7,7 (2,5) 7,8 (4,2) TMT Ä (ÒÂÍ.) TMT B (ÒÂÍ.) Stroop Test MCST (Í‡Ú.) CONCEPT ÇÂ·‡ÎÌ‡ (å) ÙÎÛË‰ÌÓÒÚ Digit span 4,0 (0,7) 3,7 (0,8) 3,5 (0,8) 3,8 (0,9) (backward) Digit Span 5,7 (0,9) 5,1 (0,7)* 5,2 (1,1) 5,3 (0,9) (forward) ÇÂ·‡ÎÌ‡ 18,8 (5,2) 16,8 (5,3) 17,1 (4,4) 16,4 (5,0) ÙÎÛË‰ÌÓÒÚ (ÊË‚ÓÚÌË) BNT 14,3 (1,0) 14,8 (0,6) 14,5 (1,3) 14,4 (1,0) Ñ‡ÌÌËÚÂ Ò‡ ÒÂ‰ÌÓ‡ËÚÏÂÚË˜ÌË (SD); TMT Ä = Trail Making Test Ä; TMT B = Trail Making Test B; MCST = Modified Card Sorting Test (Í‡ÚÂ„ÓËË); CONCEPT = DRS-Conceptualization; BNT = Boston Naming Test; *-P<0.05 ÌËÚË‚ÌÓÚÓ ÙÛÌÍˆËÓÌË‡ÌÂ. äÓÌÍÂÚÌÓ ÚÓ‚‡ Ò‡ ‚˙ÁÏÓÊÌÓÒÚËÚÂ Á‡ ÙÓÏË‡ÌÂ Ì‡ ÍÓÌˆÂÔˆËË (‚ Ì‡¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ ËÁÒÎÂ‰‚‡Ì‡ Ò ÔÓ‰ÒÍ‡Î‡ Ì‡ DRS) Ë ‚ÌËÏ‡ÌËÂ/Í‡ÚÍÓÒÓ˜Ì‡ Ô‡ÏÂÚ. éÚ ‰Û„‡ ÒÚ‡Ì‡, ‚ÁËÏ‡ÈÍË ÔÂ‰‚Ë‰ ÎËÔÒ‡Ú‡ Ì‡ ÒÔ‡‰ ‚ „ÛÔ‡Ú‡ Ì‡ ÎËˆ‡Ú‡ ËÏ‡˘Ë ÏÓÚÓÂÌ ‰Â·˛Ú Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ë‚è = Ò‚Ó·Ó‰ÌÓ ÔËÔÓÏÌﬂÌÂ, é·è= Ó·˘Ó ÔËÔÓÏÌﬂÌÂ (Ò‚Ó·Ó‰ÌÓ Ë ÔÓ‰ÔÓÏÓ„Ì‡ÚÓ), ë‚éÚ‰ = Ò‚Ó·Ó‰ÌÓ ÓÚ‰‡ÎÂ˜ÂÌÓ ÔËÔÓÏÌﬂÌÂ, é·éÚ‰ = Ó·˘Ó ÓÚ‰‡ÎÂ˜ÂÌÓ ÔËÔÓÏﬂÌÂ, ê‡Á = ‡ÁÔÓÁÌ‡‚‡ÌÂ îË„.1 Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÚÂÏÓ, ·ËıÏÂ ÏÓ„ÎË ‰‡ Á‡ÍÎ˛˜ËÏ, ˜Â ÚÓÁË ‚Ë‰ ÒËÏÔÚÓÏ ‚ Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â Ò‚˙Á‡Ì Ò ÔÓÒÎÂ‰‚‡˘‡ ÓÚÌÓÒËÚÂÎÌ‡ ÍÓ„ÌËÚË‚Ì‡ Ò˙ı‡ÌÂÌÓÒÚ. éÚ˜ËÚ‡ÈÍË ÔÓ‰·Ó‡ Ì‡ ÎËˆ‡Ú‡ ‚ÍÎ˛˜ÂÌË ‚ Ì‡ÒÚÓﬂ˘Ëﬂ ‡Ì‡ÎËÁ, ‡ ËÏÂÌÌÓ ‚ÂÏÂ‚Ó ÓÚÒÚÓﬂÌËÂ ÓÚ Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ‰Ó ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ ÌÂ ÔÓ‚Â˜Â ÓÚ 5 „Ó‰ËÌË, ÚÓ Ú‡ÁË ËÌÚÂÔÂÚ‡ˆËﬂ ËÏ‡ ÔÓ„ÌÓÒÚË˜Ì‡ ‚ÂÏÂ‚‡ ‰ÓÒÚÓ‚ÂÌÓÒÚ ‚ Ô˙‚ËÚÂ ÌﬂÍÓÎÍÓ „Ó‰ËÌË ÒÎÂ‰ Ô˙‚ËÚÂ ÍÎËÌË˜ÌË ÔÓﬂ‚Ë Ì‡ ·ÓÎÂÒÚÚ‡. èÓÎÛ˜ÂÌËÚÂ ÂÁÛÎÚ‡ÚË Ò‡ ‚ ÛÌËÒÓÌ Ò ÔÓ‚Â˜ÂÚÓ ÔÓÛ˜‚‡ÌËﬂ ‰ÂÏÓÌÒÚË‡˘Ë Ì‡ÎË˜ËÂÚÓ Ì‡ ‚˙ÁÍ‡ ÏÂÊ‰Û ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ Ë ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ Í‡ÍÚÓ Ë ‡ÒÓˆË‡ˆËﬂ Ì‡ ÚÂÏÓ‡ Ò ÓÚÌÓÒËÚÂÎÌ‡ Ò˙ı‡ÌÂÌÓÒÚ Ì‡ ÍÓ„ÌËˆËﬂÚ‡ (9), (2), (10). åÓÊÂ ‰‡ ÒÂ ÔÂ‰ÔÓÎÓÊË, ˜Â ÍÓ„ÌËÚË‚ÌËﬂÚ ‰ÂÙËˆËÚ ÔË èÅ ÒÂ ÓÔÂ‰ÂÎﬂ ‰Ó „ÓÎﬂÏ‡ ÒÚÂÔÂÌ ÓÚ Ò˙˘ËÚÂ Ô‡ÚÓÙËÁËÓÎÓ„Ë˜ÌË ÏÂı‡ÌËÁÏË, ÍÓËÚÓ ÒÂ ‡ÒÓˆËË‡Ú Ò ·‡‰ËÍËÌÂÁËﬂÚ‡ Ë Ë„Ë‰ÌÓÒÚÚ‡. ÑÓÔ‡ÏËÌÓ‚ËﬂÚ ‰ÂÙËˆËÚ ‚ ·‡Á‡ÎÌËÚÂ „‡Ì„ÎËË Ì‡Û¯‡‚‡ ÙÛÌÍˆËËÚÂ Ì‡ ÌﬂÍÓÎÍÓ ÌÂ‚Ó‡Ì‡ÚÓÏË˜ÌË Í˙„‡ Ò‚˙Á‚‡˘Ë ÒÚË‡ÚÛÏ‡ Ò ÔÓ‰ÍÓÓ‚Ë ﬂ‰‡ Ë ÍÓÚËÍ‡ÎÌËÚÂ Ó·Î‡ÒÚË. èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â ‚ÒÂÍË ÓÚ Í‡‰ËÌ‡ÎÌËÚÂ ÒËÏÔÚÓÏË Ì‡ èÅ ÒÂ ‡Á‚Ë‚‡ ÌÂÁ‡‚ËÒËÏÓ, Í‡ÚÓ ÒÎÂ‰ÒÚ‚ËÂ Ì‡ ÙÛÌÍˆËÓÌ‡ÎÌ‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ Ì‡ ÓÔÂ‰ÂÎÂÌË ÌÂ‚ÓÓ‡Ì‡ÚÓÏË˜ÌË ÔÓ‰Í˙„Ó‚Â (11), (12). Ç˙ÁÏÓÊÌÓ Â Ô‡ÚÓÙËÁËÓÎÓ„ËﬂÚ‡ Ì‡ ÚÂÏÓ‡ ‰‡ ‡Ì„‡ÊË‡ Ô˙ÚË˘‡, ÍÓËÚÓ ÌÂ Ò‡ ‰ËÂÍÚÌÓ Ò‚˙Á‡ÌË Ò ÍÓ„ÌËÚË‚ÌÓÚÓ ÙÛÌÍˆËÓÌË‡ÌÂ, ‰ÓÍ‡ÚÓ ·‡‰ËÍËÌÂÁËﬂÚ‡ Ë Ë„Ë‰ÌÓÒÚÚ‡ ‰‡ Ò‡ ÌÂ‚Ó‡Ì‡ÚÓÏË˜ÌÓ Ë ÌÂ‚ÓÏÂ‰Ë‡ÚÓÌÓ Ò‚˙Á‡ÌË Ò ÔÓÂÍˆËË Í˙Ï ‰ÓÁÓÎ‡ÚÂ‡ÎÌËﬂ ÔÂÙÓÌÚ‡ÎÂÌ Í˙„ Û˜‡ÒÚ‚‡˘ ‚ ÍÓ„ÌËÚË‚ÌËÚÂ ÔÓˆÂÒË (13), (14). ïËÔÓÚÂÁ‡Ú‡ Á‡ ‰ÓÔ‡ÏËÌÂ„Ë˜ÌËﬂ ‰ÂÙËˆËÚ Í‡ÚÓ Ì‡˜ËÌ ‰‡ ÒÂ Ó·ﬂÒÌﬂÚ ÒÔÂˆËÙË˜ÌËÚÂ ◊ÙÓÌÚ‡ÎÌË” ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ ÔË ÌÂ‰ÂÏÂÌÚÌË Ô‡ˆËÂÌÚË ‚ Ì‡˜‡ÎÓÚÓ Ì‡ èÅ, ÍÓ„‡ÚÓ ÔÓˆÂÒ˙Ú Ì‡ ÌÂ‚Ó‰Â„ÂÌÂ‡ˆËﬂ ÒÂ ÒÏﬂÚ‡ Á‡ ÔÂ‰ËÏÌÓ Ó„‡ÌË˜ÂÌ ‚ ‰ÓÔ‡ÏÂÌÂ„Ë˜ÌËÚÂ ÌÂ‚ÓÌË, ËÁ„ÎÂÊ‰‡ ÎÓ„Ë˜Ì‡. Ç ÎÓÌ„ËÚÛ‰ËÌ‡ÎÌÓ ÔÓÛ˜‚‡ÌÂ ‚˙ıÛ 20 Ô‡ˆËÂÌÚË Ò èÅ Ò ÓÚÌÓÒËÚÂÎÌÓ ÒÍÓÓ ÔÓÒÚ‡‚ÂÌ‡ ‰Ë‡„ÌÓÁ‡ Â ÓÚ˜ÂÚÂÌÓ ÌÂÔ˙ÎÌÓ ÔÓ‰Ó·ÂÌËÂ ‚ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÔÓ‚Â˜ÂÚÓ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌË ÚÂÒÚÓ‚Â, Í‡ÍÚÓ Ë Ì‡ ÏÓÚÓÌËÚÂ ÒËÏÔÚÓÏË, ÒÎÂ‰ Ì‡˜‡ÎÓÚÓ Ì‡ ÚÂ‡ÔËﬂ Ò ÎÂ‚Ó‰ÓÔ‡ Ë ÔÂ„ÓÎË‰ (15). èÓ‰Ó·ÂÌËÂÚÓ Â ÓÚ˜ÂÚÂÌÓ ÔÂ‰ËÏÌÓ Á‡ ◊ÙÓÌÚ‡ÎÌËÚÂ” ÚÂÒÚÓ‚Â, ÌÓ Ò˙˘Ó Ë Á‡ ÚÂÒÚÓ‚Â ÓˆÂÌﬂ‚‡˘Ë ‰˙Î„ÓÒÓ˜Ì‡Ú‡ ‚Â·‡ÎÌ‡ Ë ÁËÚÂÎÌ‡ Ô‡ÏÂÚ, Ë ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÒÔÓÒÓ·ÌÓÒÚË. î‡ÍÚ˙Ú, ˜Â ‚˙ÔÂÍË ÔÓ‰Ó·ÂÌËÚÂ ÍÓ„ÌËÚË‚ÌË ÒÔÓÒÓ·ÌÓÒÚË Ô‡ˆËÂÌÚËÚÂ ÌÂ ‰ÓÒÚË„‡Ú ÌÓÏ‡ÎÌËÚÂ Ô‡‡ÏÂÚË, Í‡ÍÚÓ Ë ÎËÔÒ‡Ú‡ Ì‡ ÔÓÏﬂÌ‡ ‚ ÌﬂÍÓË ÓÚ ÚÂÒÚÓ‚ÂÚÂ ‚˙ÔÂÍË ÎÂ˜ÂÌËÂÚÓ, Ò‡ ÔË˜ËÌ‡ ‰‡ ÒÂ ÔÂ‰ÔÓÎÓÊË ˜Â ÍÓ„ÌËÚË‚ÌËﬂÚ ‰ÂÙËˆËÚ ÔË èÅ ÌÂ Â ËÁˆﬂÎÓ ÒÎÂ‰ÒÚ‚ËÂ ÓÚ ‰ÓÔ‡ÏËÌÂ„Ë˜ÌÓÚÓ ËÁ˜ÂÔ‚‡ÌÂ, ‡ ‚Â- 185 ÓﬂÚÌÓ ÒÂ Ì‡ÏÂÒ‚‡Ú Ë ÌÂ‰ÓÔ‡ÏËÌÂ„Ë˜ÌË Ù‡ÍÚÓË (15). Ñ‡ÌÌËÚÂ ÓÚ ÚÓ‚‡ ÔÓÛ˜‚‡ÌÂ ÓÚ ˜‡ÒÚË Ò‡ ‚ ÔÓÚË‚Ó‚ÂÒ Ò ‰‡ÌÌËÚÂ ÓÚ ÔÓÛ˜‚‡ÌÂ Ò ÂÚÓÒÔÂÍÚË‚ÂÌ ‰ËÁ‡ÈÌ ÔÓ‚Â‰ÂÌÓ ‚˙ıÛ Ô‡ˆËÂÌÚË ‚ ÔÓ-Ì‡ÔÂ‰Ì‡Î‡ Ù‡Á‡ Ì‡ èÅ. Ç ÌÂ„Ó ËÁÒÎÂ‰Ó‚‡ÚÂÎËÚÂ Ò˙Ó·˘‡‚‡Ú Á‡ ÁÌ‡˜ËÏ‡ ÔÂ‰ËÍÚË‚Ì‡ ÓÎﬂ Ì‡ ‚˙Á‡ÒÚÚ‡ Ë ÚÂÏÓ˙Ú Í‡ÚÓ Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ Á‡ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ (16). ëÔÓÂ‰ ÚÂıÌËÚÂ ÂÁÛÎÚ‡ÚË, Ô‡ˆËÂÌÚËÚÂ Ò ÚÂÏÓ ÔË ‰Â·˛Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â ÔÓ-‚ÂÓﬂÚÌÓ ‰‡ ‡Á‚ËﬂÚ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ ÓÚÍÓÎÍÓÚÓ Ô‡ˆËÂÌÚË ËÏ‡˘Ë ‡ÍËÌÂÁËﬂ ËÎË Ë„Ë‰ÌÓÒÚ ‚ Ì‡˜‡ÎÓÚÓ Ì‡ ÒÚ‡‰‡ÌËÂÚÓ. íÂÁË Ì‡Û¯ÂÌËﬂ Ò‡ ·ËÎË Ó„‡ÌË˜ÂÌË ‚ ÒÙÂËÚÂ Ì‡ Ô‡ÏÂÚ/‚ÌËÏ‡ÌËÂ Ò ÏËÌËÏ‡ÎÌË ËÎË ÎËÔÒ‚‡˘Ë ÒÏÛ¯ÂÌËﬂ ‚ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÒÔÓÒÓ·ÌÓÒÚË Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË Ò˙ÓÚ‚ÂÚÌÓ. ëÂ‰Ì‡Ú‡ ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÔË ÎËˆ‡Ú‡ ‚ÁÂÎË Û˜‡ÒÚËÂ ‚ ÔÓÛ˜‚‡ÌÂÚÓ Â 12,7 „Ó‰ËÌË. çÂ‚ÓÔ‡ÚÓÎÓ„Ë˜ÌË ÔÓÛ˜‚‡ÌËﬂ ÔÓÍ‡Á‚‡Ú ‰Â„ÂÌÂ‡ˆËﬂ ‚ ÏÌÓÊÂÒÚ‚Ó ÔÓ‰ÍÓÓ‚Ë Ë ÍÓÓ‚Ë ÌÂ‚ÓÌ‡ÎÌË ÒËÒÚÂÏË ‚ ıÓ‰‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ (17). Ç˙ÁÏÓÊÌÓ Â Ô˙‚Ë˜Ì‡Ú‡ ‰Â„ÂÌÂ‡ˆËﬂ Ì‡ ÌÂ‚Ó‡Ì‡ÚÓÏË˜ÌËÚÂ ÒÚÛÍÚÛË Ì‡ ‰ÓÔ‡ÏËÌÂ„Ë˜Ì‡Ú‡ Ë ÌÂ‰ÓÔ‡ÏËÌÂ„Ë˜Ì‡Ú‡ ÒËÒÚÂÏ‡ ‰‡ Ò‡ ‰‚‡ ÌÂÁ‡‚ËÒËÏË ÔÓˆÂÒ‡, Í‡ÚÓ ‚ÚÓËﬂÚ ‰‡ ÒÂ ‡Á‚Ë‚‡ Ò ÔÓ-ÌËÒÍ‡ ÒÍÓÓÒÚ ÔË Ë‰ËÓÔ‡ÚË˜Ì‡Ú‡ èÅ. ÄÍÓ ÔËÎÓÊËÏ ÚÓ‚‡ ‰ÓÔÛÒÍ‡ÌÂ Í˙Ï Ì‡¯ËÚÂ ÂÁÛÎÚ‡ÚË, ÚÓ ÛÒÚ‡ÌÓ‚ÂÌËﬂÚ ÒÔ‡‰ ‚ „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ ·Ë ÏÓ„˙Î ‰‡ ÒÂ ‰˙ÎÊË ÓÒÌÓ‚ÌÓ Ì‡ ÔÓÏÂÌË ‚ ‰ÓÔ‡ÏËÌÂ„Ë˜Ì‡Ú‡ ÌÂ‚ÓÏÂ‰Ë‡ˆËﬂ, ‰ÓÍ‡ÚÓ ÂÁÛÎÚ‡ÚËÚÂ Ì‡ Vingerhoets Ë Ò˙‡‚ÚÓË (16) ‰‡ Ò‡ ÂÁÛÎÚ‡Ú Ì‡ ·‡‚ÌÓ ‡Á‚Ë‚‡˘ ÒÂ ÔÓˆÂÒ, ˜ËËÚÓ ÍÎËÌË˜ÂÌ ÂÙÂÍÚ ÒÂ ËÁﬂ‚‡‚ﬂ‚‡ ‚ ÔÓ-Í˙ÒÌËÚÂ ÂÚ‡ÔË ÓÚ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë Â ‚ÂÓﬂÚÌÓ ÒÎÂ‰ÒÚ‚ËÂ ÔÂ‰ËÏÌÓ Ì‡ ÒÏÛ˘ÂÌËﬂ ‚ ÌÂ‰ÓÔ‡ÏËÌÂ„Ë˜ÌËÚÂ ÒËÒÚÂÏË. ëÔÓÂ‰ ÌﬂÍÓË ‡‚ÚÓË, Á‡ ‚˙ÁÍ‡Ú‡ ÏÂÊ‰Û ÏÓÚÓÌËﬂ ÒËÏÔÚÓÏ ÔË ‰Â·˛Ú‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ë ÍÓ„ÌËÚË‚ÌËﬂ ‰ÂÙËˆËÚ ËÏ‡ ÁÌ‡˜ÂÌËÂ Î‡ÚÂ‡ÎËÁ‡ˆËﬂÚ‡ Ì‡ ÒËÏÔÚÓÏËÚÂ ÔË Ô˙‚ËÚÂ ÔÓﬂ‚Ë Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. Katzen Ë Ò˙‡‚ÚÓË ÛÒÚÓÌÓ‚ﬂ‚‡Ú ˜Â Ô‡ˆËÂÌÚË Ò ·‡‰ËÍËÌÂÁËﬂ ËÎË Ë„Ë‰ÌÓÒÚ Í‡ÚÓ Ô˙‚Ë ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÔË ÚÂÒÚÛ‚‡ÌÂ ËÏ‡Ú ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ ÌÂÁ‡‚ËÒËÏÓ ÓÚ ÒÚ‡Ì‡Ú‡ (Îﬂ‚Ó ËÎË ‰ﬂÒÌÓ) Ì‡ ÚﬂıÌ‡Ú‡ ÔÓﬂ‚‡, ‰ÓÍ‡ÚÓ Ô‡ˆËÂÌÚË Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ - ÔÓÍ‡Á‚‡Ú ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÂÌ ‰ÂÙËˆËÚ Ò‡ÏÓ ‚ ÒÎÛ˜‡ËÚÂ Ò ÎÂ‚ÓÒÚ‡ÌÌÓ Á‡Òﬂ„‡ÌÂ (18). Ç Ò˙˘ÓÚÓ ÔÓÛ˜‚‡ÌÂ Â‰ËÌÒÚ‚ÂÌÓ ÔË Ô‡ˆËÂÌÚËÚÂ Ò ‰ÂÒÌÓÒÚ‡ÌÌÓ Ì‡˜‡ÎÓ Ì‡ ÚÂÏÓ‡ ÎËÔÒ‚‡ ÍÓ„ÌËÚË‚ÂÌ ÒÔ‡‰. èÓ‡‰Ë Ï‡ÎÍËﬂ ·ÓÈ Ì‡ ËÌ‰Ë‚Ë‰ËÚÂ ‚ „ÛÔËÚÂ ÓÚ Ì‡ÒÚÓﬂ˘ÂÚÓ ÔÓÒÔÂÍÚË‚ÌÓ Ì‡·Î˛‰ÂÌËÂ Â ÌÂ‚˙ÁÏÓÊÌÓ ‰‡ ÒÂ Ì‡Ô‡‚Ë ÒÚ‡ÚËÒÚË˜ÂÒÍË ‰ÓÒÚÓ‚ÂÂÌ ‡Ì‡ÎËÁ ÔÓ·Ë‡˘ ‚˙ÁÏÓÊÌÓÒÚÚ‡ Á‡ ‚ÎËﬂÌËÂ Ì‡ Î‡ÚÂ‡ÎËÁ‡ˆËﬂÚ‡ Ì‡ ÚÂÏÓ‡ ÔË Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ‚˙ıÛ ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰. äÓ„‡ÚÓ Ó·Ò˙Ê‰‡ÏÂ ÁÌ‡˜ÂÌËÂÚÓ Ì‡ Ì‡˜‡ÎÌËﬂ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Í‡ÚÓ ‚˙ÁÏÓÊÂÌ ÔÂ‰ËÍÚÓ ‚ ÔÓ-ÓÚ‰‡ÎÂ˜ÂÌ ‚ÂÏÂ‚Ë ÔÎ‡Ì, Úﬂ·‚‡ ‰‡ Ó·˙ÌÂÏ ‚ÌËÏ‡ÌËÂ Ë Ì‡ Ù‡ÍÚ‡, ˜Â ‰‚Ë„‡ÚÂÎÌËÚÂ ÒËÏÔÚÓÏË ‚ ıÓ‰‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ Ú˙ÔﬂÚ ‰ËÌ‡ÏËÍ‡ ÔË ÌﬂÍÓË ·ÓÎÌË. í‡Í‡ Ì‡ÔËÏÂ Hershey Ë Ò˙‡‚ÚÓË ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ˜Â ÓÚ 27 ·ÓÎÌË Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ, ÔË ÔÓÒÎÂ‰ﬂ‚‡ÌÂ Á‡ 7 „Ó‰ËÌË Ò‡ÏÓ 16 ÔÓ‰˙ÎÊ‡‚‡Ú ‰‡ ËÏ‡Ú ‰ÓÏËÌË‡Ì‡ ÓÚ ÚÂÏÓ‡ ÒËÏÔÚÓÏ‡ÚËÍ‡ (19). Ç Ò˙˘‡Ú‡ ‡Á‡·ÓÚÍ‡ ËÁÒÎÂ‰Ó‚‡ÚÂÎËÚÂ Ô‡‚ﬂÚ Á‡ÍÎ˛˜ÂÌËÂ, ˜Â ÌÂ Ò‡ÏÓ ÚÂÏÓ˙Ú ÔË Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, ‡ Ë ÌÂ„Ó‚ÓÚÓ ‰ÓÏËÌË‡ÌÂ ‚ ÒÎÂ‰‚‡˘ËÚÂ ÌﬂÍÓÎÍÓ „Ó‰ËÌË ËÏ‡ ÁÌ‡˜ÂÌËÂ ‰Ó ËÁ‚ÂÒÚÌ‡ ÒÚÂÔÂÌ Á‡ ‰Ó·ÓÍ‡˜ÂÒÚ‚ÂÌÓÚÓ ÍÓ„ÌËÚË‚ÌÓ ÔÓÚË˜‡ÌÂ. Ç Ì‡¯ÂÚÓ ÔÓÛ˜‚‡ÌÂ ÚÓ‚‡ Â Ò˙Ó·‡ÁÂÌÓ ˜ÂÁ ÔËÎÓÊÂÌ‡ ÒÂÎÂÍˆËﬂ Ì‡ ·ÓÎÌËÚÂ. äÓÌÍÂÚÌÓ ÌËÂ ËÁÍÎ˛˜‚‡ÏÂ ÓÚ ‡Ì‡ÎËÁ‡ ÎËˆ‡, Á‡ ÍÓËÚÓ ËÏ‡ ‰‡ÌÌË, ˜Â ÚÂÏÓ˙Ú Â ·ËÎ ÔÓÒÎÂ‰‚‡Ì ÓÚ Ë„Ë‰ÌÓÒÚ ËÎË Ì‡Û¯ÂÌËﬂ ‚ ÔÓıÓ‰Í‡Ú‡ ‚ ‡ÏÍËÚÂ Ì‡ Ô˙‚‡Ú‡ „Ó‰ËÌ‡ ÓÚ ÌÂ„Ó‚ÓÚÓ Ì‡˜‡ÎÓ. èÓ ÚÓÁË Ì‡˜ËÌ Â Ì‡Ô‡‚ÂÌ ÓÔËÚ ‰‡ ÒÂ Ì‡Ï‡ÎË ‚ÂÓﬂÚÌÓÒÚÚ‡ Á‡ ÍÓÌ‚ÂÁËﬂ Ì‡ ÚÂ- Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ÏÓ ‰Â·˛ÚË‡˘‡Ú‡, ‚ ‰Û„ ÚËÔ ‰ÓÏËÌË‡˘‡ ÒËÏÔÚÓÏ‡ÚËÍ‡ ‚ ÒÎÂ‰‚‡˘ËÚÂ ÌﬂÍÓÎÍÓ „Ó‰ËÌË. Ö‰Ì‡ ÓÚ Ì‡È-˜ÂÒÚÓ Ò˙Ó·˘‡‚‡ÌËÚÂ ÓÒÓ·ÂÌÓÒÚË Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ÒÔ‡‰ ÔË ‡ÌÌ‡ èÅ Â ÂÍÁÂÍÛÚË‚ÌËﬂ ‰ÂÙËˆËÚ (20). Ç Ì‡ÒÚÓﬂ˘ÂÚÓ ÔÓÛ˜‚‡ÌÂ Ì‡ÎË˜ËÂÚÓ Ì‡ ÒÔ‡‰ ‚ Ú‡ÁË ÍÓ„ÌËÚË‚Ì‡ Ó·Î‡ÒÚ Â Ó·‚˙Á‡ÌÓ Ò‡ÏÓ Ò „ÛÔ‡Ú‡ Ì‡ Ô‡ˆËÂÌÚËÚÂ, ˜ËËÚÓ Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ Â ·‡‰ËÍËÌÂÁËﬂ/Ë„Ë‰ÌÓÒÚ. çÂÓ·ıÓ‰ËÏÓ Â ‰‡ ÒÂ ÛÚÓ˜ÌË, ˜Â ÔÓ‚Â‰ÂÌËﬂ ‡Ì‡ÎËÁ ÓÚ˜ËÚ‡ Â‰ËÌÒÚ‚ÂÌÓ Ì‡ÎË˜ËÂÚÓ ËÎË ÎËÔÒ‡Ú‡ Ì‡ ÁÌ‡˜ËÏ ÒÔ‡‰ ‚ ÂÁÛÎÚ‡ÚËÚÂ ÓÚ ÓÚ‰ÂÎÌËÚÂ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÌË ÚÂÒÚÓ‚Â, Ú.Â. ÌÂ ÒÂ ËÁÍÎ˛˜‚‡ Ì‡ÎË˜ËÂÚÓ Ì‡ ÂÍÁÂÍÛÚË‚Ì‡ ‰ËÒÙÛÌÍˆËﬂ ÔË Ô‡ˆËÂÌÚËÚÂ Ò Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ, ÌÓ ÚÓÁË ‰ÂÙËˆËÚ Â ÓÚÌÓÒËÚÂÎÌÓ ÒÚ‡ˆËÓÌË‡Ì ‚˙‚ ‚ÂÏÂ‚Ëﬂ ÓÚﬂÁ˙Í, ‚˙ıÛ ÍÓÈÚÓ Â ËÁ‚˙¯ÂÌÓ Ì‡·Î˛‰ÂÌËÂÚÓ. ê‡Á„ÎÂÊ‰‡ÈÍË ÔÓ ÚÓÁË Ì‡˜ËÌ ÂÁÛÎÚ‡ÚËÚÂ ·ËıÏÂ ÏÓ„ÎË ‰‡ ÔÂ‰ÔÓÎÓÊËÏ, ˜Â ‚ ‰˙Î„ÓÒÓ˜ÂÌ ÔÎ‡Ì „ÛÔ‡Ú‡ Ì‡ èÅ-Åê ˘Â ‰ÂÏÓÌÒÚË‡ ÔÓ-„ÓÎﬂÏ‡ ÒÚÂÔÂÌ Ì‡ ÍÓ„ÌËÚË‚Ì‡ ‰Â„‡‰‡ˆËﬂ ÓÚÍÓÎÍÓÚÓ „ÛÔ‡Ú‡ ËÏ‡˘‡ Ì‡˜‡ÎÂÌ ÏÓÚÓÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ. Ç Á‡ÍÎ˛˜ÂÌËÂ, Ì‡¯ËÚÂ ÂÁÛÎÚ‡ÚË ÔÓÍ‡Á‚‡Ú Ï‡Î˙Í, ÌÓ ÁÌ‡˜ËÏ ÒÔ‡‰ ‚ ‰‚‡ ÓÚ ÍÓ„ÌËÚË‚ÌËÚÂ ÚÂÒÚÓ‚Â Ò‡ÏÓ Á‡ „ÛÔ‡Ú‡ Ò Ì‡˜‡ÎÂÌ ÒËÏÔÚÓÏ Ë„Ë‰ÌÓÒÚ Ë/ËÎË ·‡‰ËÍËÌÂÁËﬂ. íÓÁË ÒÔ‡‰ Â ‚ÂÓﬂÚÌ‡ Ó·‚˙Á‡Ì ÔÂ‰ËÏÌÓ Ò ‰ÂÙËˆËÚ ‚ ‰ÓÔ‡ÏËÌÂ„Ë˜Ì‡Ú‡ ÏÂ‰Ë‡ˆËﬂ. è‡ˆËÂÌÚË Ò ‰Â·˛ÚÂÌ ÒËÏÔÚÓÏ ÚÂÏÓ ËÏ‡Ú ÔÓ-‰Ó·‡ ÔÓ„ÌÓÁ‡ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÍÓ„ÌËÚË‚ÌÓÚÓ ÙÛÌÍˆËÓÌË‡ÌÂ ÔÓÌÂ ‚ Ô˙‚ËÚÂ ÌﬂÍÓÎÍÓ „Ó‰ËÌË ÒÎÂ‰ Ì‡˜‡ÎÓÚÓ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. ê‡Á‰ÂÎﬂÌÂÚÓ Ì‡ Ô‡ˆËÂÌÚËÚÂ Ò èÅ Ì‡ ÔÓ‰„ÛÔË ÒÔÓÂ‰ ‰Â·˛ÚÌËﬂ ÒËÏÔÚÓÏ Â Û‰Ó·ÂÌ ‚ Ô‡ÍÚËÍ‡Ú‡ ÔÓ‰ıÓ‰ Ò Ì‡‰ÂÊ‰ÌÓ ÔÂ‰ËÍÚË‚ÌÓ ÁÌ‡˜ÂÌËÂ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÍÓ„ÌËÚË‚ÌÓÚÓ ÙÛÌÍˆËÓÌË‡ÌÂ. ãàíÖêÄíìêÄ 1. 2. 3. 4. Iwasaki, Y., et al., Cognitive function in Parkinson's disease: in relation to motor symptoms. Int J Neurosci, 1989. 47(3-4): p. 295-300. Roos, R.A., J.C. Jongen, and E.A. van der Velde, Clinical course of patients with idiopathic Parkinson's disease. Mov Disord, 1996. 11(3): p. 236-42. Zetusky, W.J., J. Jankovic, and F.J. 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Kulisevsky, J., et al., Chronic effects of dopaminergic replacement on cognitive function in Parkinson's disease: a two-year follow-up study of previously untreated patients. Mov Disord, 2000. 15(4): p. 613-26. Vingerhoets, G., et al., Predictors of cognitive impairment in advanced Parkinson's disease. J Neurol Neurosurg Psychiatry, 2003. 74(6): p. 793-6. Braak, H., et al., Staging of brain pathology related to sporadic Parkinson's disease. Neurobiol Aging, 2003. 24(2): p. 197-211. Katzen, H.L., B.E. Levin, and W. Weiner, Side and type of motor symptom influence cognition in Parkinson's disease. Mov Disord, 2006. 21(11): p. 1947-53. Hershey, L.A., et al., Tremor at onset. Predictor of cognitive and motor outcome in Parkinson's disease Arch Neurol, 1991. 48(10): p. 1049-51. Levin, B.E. and H.L. Katzen, Early cognitive changes and nondementing behavioral abnormalities in Parkinson's disease. Adv Neurol, 2005. 96: p. 84-94. Ä‰ÂÒ Á‡ ÍÓÂÒÔÓÌ‰ÂÌˆËﬂ: Ñ- ü‚Ó ÜÂÎÂ‚, ìåÅÄã “ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, äÎËÌËÍ‡ ÔÓ ÌÂ‚ÓÎÓ„Ëﬂ, ·ÛÎ. “ÉÂÓ„Ë ëÓÙËÈÒÍË” 1, ëÓÙËﬂ 1431 ÚÂÎ: 02/92 30 544 Â-mail: [email protected] Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 187 éË„ËÌ‡ÎÌË ÒÚ‡ÚËË äéÉçàíàÇçà çÄêìòÖçàü èêà åàéíéçàóçà Ñàëíêéîàà íàè STEINERT à PROMM. ÉÖçéíàè-îÖçéíàè äéêÖãÄñàà í. ó‡ÏÓ‚‡1, å ê‡È˜Â‚‡1, à. í˙ÌÂ‚1,2 1çÂ‚ÓÎÓ„Ë˜Ì‡ 2çÓ‚ ÍÎËÌËÍ‡, ìåÅÄã ◊ÄÎÂÍÒ‡Ì‰Ó‚ÒÍ‡”, åî –ëÓÙËﬂ Å˙Î„‡ÒÍË ìÌË‚ÂÒËÚÂÚ ◊ ä‡ÚÂ‰‡ ÔÓ ÍÓ„ÌËÚË‚ÌË Ì‡ÛÍË Ë ÔÒËıÓÎÓ„Ëﬂ” SUMMARY The myotonic dystrophies (myotonic dystrophy type 1, DM-1 and myotonic dystrophy type 2, PROMM/DM-2) are highly variable autosomal dominant, multisystem disorders characterised by myotonic phenomenons, involvement of the neuromuscular, cardiovascular and the endocrine systems as well as by abnormalities in cognition and personality. Indeed, CNS involvement in DM1 had been observed since the first descriptions of the disease, ranging from a condition of mental retardation, characteristic of congenital cases, to some behavioral and cognitive changes frequently seen in adult classical forms. The occurrence of different patterns of cognitive impairment in patients with DM1 is probably due to: altered expression of genes regulating later stages of brain development in congenital forms and affected correct splicing of tau proteins in neurons of frontotemporal cortex in classical forms. The visual-spatial abilities are affected in patients with PROMM in a milder extend compared to those with Steinert disease. KEY WORDS: myotonic dystrophies, cognitive and behavioral disorders, tau-proteins, frontotemporal cortex. êÖáûåÖ åËÓÚÓÌË˜ÌËÚÂ ‰ËÒÚÓÙËË ÚËÔ I (·ÓÎÂÒÚ Ì‡ Steinert) Ë ÚËÔ II (PROMM) Ò‡ „ÂÌÂÚË˜ÌË ÏÛÎÚËÒËÒÚÂÏÌË Á‡·ÓÎﬂ‚‡ÌËﬂ Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÂÌ ÚËÔ Ì‡ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ Ë ÌÂÔ˙ÎÌ‡ ÔÂÌÂÚ‡ÌÚÌÓÒÚ, ÍÓËÚÓ ÒÂ ı‡‡ÍÚÂËÁË‡Ú ÍÎËÌË˜ÌÓ Ò ÏËÓÚÓÌË˜ÌË ÙÂÌÓÏÂÌË (Á‡ÚÛ‰ÌÂÌ‡ ÂÎ‡ÍÒ‡ˆËﬂ ÒÎÂ‰ ÒËÎÌÓ ÏÛÒÍÛÎÌÓ Ò˙Í‡˘ÂÌËÂ), ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ Ë ‡ÚÓÙËﬂ, Í‡Ú‡‡ÍÚ‡, Í‡‰ËÓÏËÓÔ‡ÚËﬂ, „ÓÌ‡‰Ì‡ ‡ÚÓÙËﬂ Ë ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ. èÓﬂ‚Ë ÓÚ ÒÚ‡Ì‡ Ì‡ ñçë ÔË ÚÂÁË Ô‡ˆËÂÌÚË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ó˘Â ÔË Ô˙‚ËÚÂ ÓÔËÒ‡ÌËﬂ Ì‡ ÏËÓÚÓÌË˜ÌË ‰ËÒÚÓÙËË, Í‡ÚÓ ÚÂ ÏÓ„‡Ú ‰‡ ‚‡Ë‡Ú ÓÚ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ ÔË ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ ‰Ó ÒÔÂˆËÙË˜ÌË ÍÓ„ÌËÚË‚ÌË Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ, ˜ÂÒÚË ÔË ÍÎ‡ÒË˜ÂÒÍËÚÂ ÙÓÏË. Ç‡Ë‡ˆËËÚÂ ‚ ÍÓ„ÌËÚË‚ÌËﬂ ÔÓÙËÎ ÔË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ Ì‡ Steinert Ì‡È-‚ÂÓﬂÚÌÓ Ò‡ ÒÎÂ‰ÒÚ‚ËÂ ÓÚ ÒÏÛ˘ÂÌËÂ ‚ ÂÍÒÔÂÒËﬂÚ‡ Ì‡ „ÂÌË, Â„ÛÎË‡˘Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÌËÚÂ ÒÚÛÍÚÛË ÔË ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ Ë Ì‡Û¯ÂÌ ÒÔÎ‡ÈÒËÌ„ Ì‡ Ú‡Û-ÔÓÚÂËÌËÚÂ ‚ ÌÂ‚ÓÌËÚÂ Ì‡ ÙÓÌÚÓÚÂÏÔÓ‡ÎÌËﬂ ÍÓÚÂÍÒ ÔË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡. èË PROMM Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ì‡Û¯ÂÌËﬂ ‚ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÛÏÂÌËﬂ ‚ ÔÓ-ÎÂÍ‡ ÒÚÂÔÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò ÚËÔ I. äãûóéÇà Ñìåà: ÏËÓÚÓÌË˜ÌË ‰ËÒÚÓÙËË, ÍÓ„ÌËÚË‚ÌË Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ, Ú‡Û-ÔÓÚÂËÌË, ÙÓÌÚÓÚÂÏÔÓ‡ÎÂÌ ÍÓÚÂÍÒ. åËÓÚÓÌË˜ÌËÚÂ ‰ËÒÚÓÙËË ÚËÔ I (·ÓÎÂÒÚ Ì‡ Steinert) Ë ÚËÔ II (PROMM) Ò‡ „ÂÌÂÚË˜ÌË ÏÛÎÚËÒËÒÚÂÏÌË Á‡·ÓÎﬂ‚‡ÌËﬂ Ò ‡‚ÚÓÁÓÏÌÓ-‰ÓÏËÌ‡ÌÚÂÌ ÚËÔ Ì‡ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ Ë ÌÂÔ˙ÎÌ‡ ÔÂÌÂÚ‡ÌÚÌÓÒÚ, ÍÓËÚÓ ÒÂ ı‡‡ÍÚÂËÁË‡Ú ÍÎË- ÌË˜ÌÓ Ò ÏËÓÚÓÌË˜ÌË ÙÂÌÓÏÂÌË (Á‡ÚÛ‰ÌÂÌ‡ ÂÎ‡ÍÒ‡ˆËﬂ ÒÎÂ‰ ÒËÎÌÓ ÏÛÒÍÛÎÌÓ Ò˙Í‡˘ÂÌËÂ), ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ Ë ‡ÚÓÙËﬂ, Í‡Ú‡‡ÍÚ‡, Í‡‰ËÓÏËÓÔ‡ÚËﬂ, „ÓÌ‡‰Ì‡ ‡ÚÓÙËﬂ Ë ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ. èË ·ÓÎÂÒÚÚ‡ Ì‡ Steinert (åÑ 1) ÔÓ-ÚËÔË˜Ì‡ Â ÒÎ‡·ÓÒÚÚ‡ Ì‡ ‰ËÒÚ‡ÎÌËÚÂ ÏÛÒÍÛÎË, ‰ÓÍ‡ÚÓ ÔË PROMM (åÑ 2) ÍÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡ Â ÔÓ-ÎÂÍ‡ Ë ÒÂ Á‡Òﬂ„‡Ú ÔÂÓ·Î‡‰‡‚‡˘Ó ÔÓÍÒËÏ‡ÎÌËÚÂ ÏÛÒÍÛÎË. èÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ‚˙Á‡ÒÚÚ‡ Ì‡ Ì‡˜‡ÎÓ Ë ÚÂÊÂÒÚÚ‡ Ì‡ ÍÎËÌË˜ÌËÚÂ ÒËÏÔÚÓÏË ÒÂ Ì‡·Î˛‰‡‚‡Ú ËÁ‡ÁÂÌË ËÌÚÂ- Ë ËÌÚ‡Ù‡ÏËÎÌË ‚‡Ë‡ˆËË. àëíéêàü åËÓÚÓÌË˜Ì‡Ú‡ ‰ËÒÚÓÙËﬂ ÚËÔ I Â ÓÔËÒ‡Ì‡ Á‡ Ô˙‚Ë Ô˙Ú ÓÚ Steinert ÔÂÁ 1909 (64) Í‡ÚÓ Á‡·ÓÎﬂ‚‡ÌÂ Ò Ì‡˜‡ÎÓ ÔÂÁ ‚ÚÓÓÚÓ ËÎË ÚÂÚÓÚÓ ‰ÂÒÂÚËÎÂÚËÂ. Vanier Ë Ò˙‡‚Ú. ÔÂÁ 1960 „. ÓÔËÒ‚‡Ú ÍÓÌ„ÂÌËÚ‡ÎÌ‡ ÙÓÏ‡, ÍÓﬂÚÓ ÒÂ ÔÂ‰‡‚‡ ÓÚ ·ÓÎÌË Ï‡ÈÍË Ò ÚÓ‚‡ Á‡·ÓÎﬂ‚‡ÌÂ (72). èÂÁ 1992 ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡, ˜Â Á‡·ÓÎﬂ‚‡ÌÂÚÓ Â Ó·ÛÒÎÓ‚ÂÌÓ ÓÚ ÚËÌÛÍÎÂÓÚË‰Ì‡ ÂÍÒÔ‡ÌÁËﬂ ‚ ÌÂÚ‡ÌÒÎË‡Ì Â„ËÓÌ Ì‡ DMPK-„ÂÌ‡ (40). äÎËÌË˜ÌËÚÂ ı‡‡ÍÚÂËÒÚËÍË Ì‡ PROMM Ò‡ ÓÔËÒ‡ÌË ÔÂÁ 1994 „, ‡ ÚÓ˜Ì‡Ú‡ ÏÛÚ‡ˆËﬂ, Á‡Òﬂ„‡˘a ZNF9-„ÂÌ‡, Â ÓÚÍËÚ‡ ÔÂÁ 2001 „ (39). ÖèàÑÖåàéãéÉàü ÅÓÎÂÒÚÚ‡ Ì‡ Steinert Â Ì‡È-˜ÂÒÚÓ ÒÂ˘‡Ì‡Ú‡ ÙÓÏ‡ Ì‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÔË ‚˙Á‡ÒÚÌË. óÂÒÚÓÚ‡Ú‡ È ‚ Ó·˘‡Ú‡ ÔÓÔÛÎ‡ˆËﬂ ‚‡Ë‡ ÓÚ 1/100 000 ‚ ÌﬂÍÓË Ó·Î‡ÒÚË Ì‡ üÔÓÌËﬂ ‰Ó 1/10 000 ‚ àÎ‡Ì‰Ëﬂ, ‡ ÔÓ‡‰Ë ÂÙÂÍÚ‡ Ì‡ Ó‰ÓÌ‡˜‡ÎÌËÍ‡ ‚ ä‡Ì‡‰‡ (ä‚Â·ÂÍ) ˜ÂÒÚÓÚ‡Ú‡ È ‰ÓÒÚË„‡ ‰Ó 1/530. ëÂ‰Ì‡Ú‡ ˜ÂÒÚÓÚ‡ Â 1/20 000. åÑ 2 ÒÂ ÒÂ˘‡ ÔÂÓ·Î‡‰‡‚‡˘Ó ‚ ÒÚ‡ÌËÚÂ ÓÚ ÒÂ‚ÂÌ‡ Ö‚ÓÔ‡ Ë ÉÂÏ‡ÌËﬂ, Í˙‰ÂÚÓ ·ÓÎÂÒÚÌÓÒÚÚ‡ Â ·ÎËÁÍ‡ ‰Ó Ú‡ÁË ÔË åÑ 1 (40). äãÄëàîàäÄñàü ë˙˘ÂÒÚ‚Û‚‡Ú ˜ÂÚËË ÙÓÏË Ì‡ ·ÓÎÂÒÚÚ‡ Ì‡ Steinert, ‡Á„‡ÌË˜ÂÌË ÒÔÓÂ‰ Ì‡˜‡ÎÓÚÓ Ë ÚÂÊÂÒÚÚ‡ Ì‡ ÍÎËÌË˜Ì‡Ú‡ ÒËÏÔÚÓÏ‡ÚËÍ‡ ( 28): ● äÓÌ„ÂÌËÚ‡ÎÌ‡- ÓÍÓÎÓ 14% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò åÑ ÚËÔ 1 (29) ● ÑÂÚÒÍ‡ ● äÎ‡ÒË˜ÂÒÍ‡ ● ãÂÍ‡ éÒÌÓ‚Ì‡ ÓÒÓ·ÂÌÓÒÚ, ÓÚÎË˜‡‚‡˘‡ PROMM ÓÚ åÑ ÚËÔ 1 Â ÎËÔÒ‡Ú‡ Ì‡ ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡. ÖíàéãéÉàü åÑ 1 ÒÂ Ó·ÛÒÎ‡‚ﬂ ÓÚ ‰ËÌ‡ÏË˜ÌË ÏÛÚ‡ˆËË, Á‡Òﬂ„‡˘Ë DMPK-„ÂÌ‡, ÎÓÍ‡ÎËÁË‡Ì ‚˙ıÛ 19q13.3 ıÓÏÓÁÓÏ‡. ìÒÚ‡ÌÓ‚ﬂ‚‡ ÒÂ ‡ÏÔÎËÙËÍ‡ˆËﬂ Ì‡ ÚËÌÛÍÎÂÓÚË‰ÌË CTGÔÓ‚ÚÓÂÌËﬂ ‚ 3’ ÌÂÚ‡ÌÒÎËÛÂÏ Â„ËÓÌ Ì‡ DMPK-„ÂÌ‡ (12). çÓÏ‡ÎÌËﬂÚ ·ÓÈ Ì‡ ÚÂÁË ÔÓ‚ÚÓË ‚‡Ë‡ ÏÂÊ‰Û 5 Ë 37 (57). äÎËÌË˜Ì‡ ËÁﬂ‚‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡, ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÍÓ„‡ÚÓ ·ÓﬂÚ Ì‡ ÔÓ‚ÚÓËÚÂ Ì‡‰ı‚˙ÎË 50. èË ËÌ‰Ë‚Ë‰Ë Ò CTG-ÔÓ‚ÚÓÂÌËﬂ ÏÂÊ‰Û 35 Ë 49 ÌﬂÏ‡ ÍÎËÌË˜Ì‡ ÒËÏÔÚÓÏ‡ÚËÍ‡, ÌÓ ÚÂıÌËÚÂ ‰Âˆ‡ Ò‡ Ò ÔÓ‚Ë¯ÂÌ ËÒÍ ÓÚ ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ Ì‡ ÔÓ-„ÓÎﬂÏ ·ÓÈ ÔÓ‚ÚÓË Ë Ò˙ÓÚ‚ÂÚÌÓ ËÁﬂ‚ﬂ‚‡ÌÂ Ì‡ ·ÓÎÂÒÚÚ‡ (33). ç‡˜‡ÎÓÚÓ Ë ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ ÍÓÂÎË‡Ú Ò ·Óﬂ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ ÔÓ‚ÚÓË Ë ÔÓËÁıÓ‰‡ Ì‡ ÏÛÚ‡ÌÚÌËﬂ „ÂÌ- Ï‡È˜ËÌ ËÎË ·‡˘ËÌ. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ÍÓÎÍÓÚÓ Â ÔÓ-„ÓÎﬂÏ Â ÚÓÁË ·ÓÈ, ÚÓÎÍÓ‚‡ ÔÓ-‡ÌÓ Á‡ÔÓ˜‚‡ Ë ÔÓÚÂÊÍÓ ÔÓÚË˜‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. í˙È Í‡ÚÓ ÚËÌÛÍÎÂÓÚË‰ÌËÚÂ CTG ÔÓ‚ÚÓË ‚ DMPK „ÂÌ‡ Ò‡ ÌÂÒÚ‡·ËÎÌË ÔÓ ‚ÂÏÂ Ì‡ ÏËÚÓÁ‡Ú‡, ˜ÂÒÚÓ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÒÓÏ‡ÚË˜ÂÌ ÏÓÁ‡ËˆËÁ˙Ï ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ‡ÁÏÂ‡ Ì‡ CTG ÂÍÒÔ‡ÌÁËﬂÚ‡ (28). á‡ ÚÓ‚‡ ÌÂ ‚ËÌ‡„Ë Â ‚˙ÁÏÓÊÌ‡ ÍÓÂÎ‡ˆËﬂ ÏÂÊ‰Û ‡ÁÏÂ‡ Ì‡ ÔÓ‚ÚÓËÚÂ, Ì‡·Î˛‰‡‚‡ÌË ‚ Â‰Ì‡ Ú˙Í‡Ì, Ë ÚÂÊÂÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ. åÌÓ„Ó ÚËÔË˜ÌÓ ﬂ‚ÎÂÌËÂ Â ‡ÌÚËˆËÔ‡ˆËﬂÚ‡- ÔÓ-‡ÌÌÓ Ì‡˜‡ÎÓ Ë ÔÓ-ÚÂÊÍ‡ ÍÎËÌË˜Ì‡ ËÁﬂ‚‡ ‚˙‚ ‚ÒﬂÍÓ ÒÎÂ‰‚‡˘Ó ÔÓÍÓÎÂÌËÂ. íﬂ ÒÂ ‰˙ÎÊË Ì‡ Û‚ÂÎË˜‡‚‡ÌÂÚÓ Ì‡ CTG-ÔÓ‚ÚÓËÚÂ ‚ ÔÓÍÓÎÂÌËﬂÚ‡ (28; 48). ìÒÚ‡ÌÓ‚ﬂ‚‡ ÒÂ ÔÓÎÓ‚Ó-‰ËÙÂÂÌˆË‡Ì‡ ÂÍÒÔÂÒËﬂ Ì‡ „ÂÌ‡ Ò ÔÓÚÂÊÍ‡ ÍÎËÌË˜Ì‡ ËÁﬂ‚‡ ‡ÍÓ ÒÂ ÛÌ‡ÒÎÂ‰Ë ÓÚ Ï‡ÈÍ‡Ú‡. åÑ 2 ÒÂ Ó·ÛÒÎ‡‚ﬂ ÓÚ Ú‡ÌÒÍË·Ë‡Ì‡, ÌÓ ÌÂÚ‡ÌÒÎË‡Ì‡ ÂÍÒÔ‡ÌÁËﬂ Ì‡ CCTG- ÌÛÍÎÂÓÚË‰ÌË ÔÓ‚ÚÓË ‚ ËÌÚÓÌ 1 Ì‡ ZNF9 (Zinc Finger protein 9) „ÂÌ‡ (39; 48). Ç˙ÔÂÍË ˜Â ÍÎËÌË˜Ì‡Ú‡ Í‡ÚËÌ‡ ÔË PROMM Â ÔÓ-ÎÂÍ‡, ·ÓﬂÚ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ CCTG- ÔÓ‚ÚÓË ÏÓÊÂ ‰‡ ‰ÓÒÚË„ÌÂ ÓÚ 75 ‰Ó 11 000 (56). èÄíéÉÖçÖáÄ ÉÂÌ˙Ú, ‚ ÍÓÈÚÓ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ‰ËÌ‡ÏË˜Ì‡ ÏÛÚ‡ˆËﬂ ÔË åÑ 1, ÍÓ‰Ë‡ ÒÂËÌ-ÚÂÓÌËÌ ÔÓÚÂËÌ ÍËÌ‡Á‡ (DMPK –dystrophy myotonic protein kinase), ÎÓÍ‡ÎËÁË‡Ì‡ ‚ ÒÔÂˆËÙË˜ÌË ÍÎÂÚ˙˜ÌË ÒÚÛÍÚÛË Ì‡ ÒÍÂÎÂÚÌ‡Ú‡, Ò˙‰Â˜Ì‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ Ë ÌÂ‚ÓÌËÚÂ, ‡ÒÓˆËË‡ÌË Ò ÏÂÊ‰ÛÍÎÂÚ˙˜Ì‡Ú‡ ÔÓ‚Ó‰ËÏÓÒÚ Ë ÔÂ‰‡‚‡ÌÂÚÓ Ì‡ ËÏÔÛÎÒËÚÂ (6; 12;). ÉÂÌ˙Ú, ‚ ÍÓÈÚÓ ÒÂ ÓÚÍË‚‡ ‰ËÌ‡ÏË˜Ì‡ ÏÛÚ‡ˆËﬂ ÔË åÑ 2 Â ZNF9. íÓÈ ÍÓ‰Ë‡ ÔÓÚÂËÌ, Ò˙ÒÚ‡‚ÂÌ ÓÚ ÒÂ‰ÂÏ ÂÎÂÏÂÌÚ‡, ËÁ‚ÂÒÚÌË Í‡ÚÓ zinc finger ‰ÓÏÂÌË, ˜ËﬂÚÓ ÙÛÌÍˆËﬂ Â ‰‡ ÒÂ Ò‚˙Á‚‡Ú Ò ÚÓ˜ÌÓ ÓÔÂ‰ÂÎÂÌË Ó·Î‡ÒÚË Ì‡ Ëêçä. Zinc finger ÔÓÚÂËÌ 9 Û˜‡ÒÚ‚‡ ‚ Â„ÛÎ‡ˆËﬂÚ‡ Ì‡ „ÂÌËÚÂ ÓÚ„Ó‚ÓÌË Á‡ ÒËÌÚÂÁ‡Ú‡ Ë ÏÂÚ‡·ÓÎËÁÏ‡ Ì‡ ıÓÎÂÒÚÂÓÎ‡. íÓÁË ÔÓÚÂËÌ ÒÂ ÓÚÍË‚‡ ‚ ÏÌÓ„Ó Ú˙Í‡ÌË, ÌÓ ÓÒÌÓ‚ÌÓ ‚ Ò˙‰Â˜Ì‡Ú‡ Ë ÒÍÂÎÂÚÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡. ÖÙÂÍÚ˙Ú Ì‡ CTG, ÂÒÔ. Ì‡ CëTG - ÔÓ‚ÚÓËÚÂ Â ÍÓÏÔÎÂÍÒÂÌ Ë ÌÂÌ‡Ô˙ÎÌÓ ËÁﬂÒÌÂÌ. ìÒÚ‡ÌÓ‚ÂÌÓ Â, ˜Â ‡ÏÔÎËÙËÍ‡ˆËﬂÚ‡ ÌÂ Á‡Òﬂ„‡ ÍÓ‰Ë‡˘Ëﬂ ÒÂ„ÏÂÌÚ Ì‡ Ò˙ÓÚ‚ÂÚÌËﬂ „ÂÌ, Ú˙È Í‡ÚÓ Úﬂ ÒÂ Ú‡ÌÒÍË·Ë‡ ‚ êçä, ÌÓ ÌÂ ÒÂ Ú‡ÌÒÎË‡. åËÓÚÓÌË˜ÌËÚÂ ‰ËÒÚÓÙËË Ò‡ Ô˙‚ËﬂÚ ÔËÏÂ Á‡ êçä ÏÂ‰ËË‡ÌÓ Á‡·ÓÎﬂ‚‡ÌÂ. ÖÍÒÔÂÒËﬂÚ‡ Ì‡ ÏÛÚ‡ÌÚÌËﬂ „ÂÌ Ó·ÛÒÎ‡‚ﬂ ÔÓﬂ‚‡Ú‡ Ì‡ ÏÛÚ‡ÌÚÌ‡ Ëêçä Ò ‰ËÂÍÚÂÌ ÚÓÍÒË˜ÂÌ ÂÙÂÍÚ ‚˙ıÛ ÍÎÂÚÍËÚÂ ( 51). íﬂ ÒÂ ÓÚÎ‡„‡ ‚ ÍÎÂÚ˙˜ÌËÚÂ ﬂ‰‡ Ë ÙÓÏË‡ Ë·ÓÌÛÍÎÂ‡ÌË ‚ÍÎ˛˜‚‡ÌËﬂ ‚ Á‡ÒÂ„Ì‡ÚËÚÂ Ú˙Í‡ÌË- ÓÒÌÓ‚ÌÓ ‚ ÏÛÒÍÛÎËÚÂ Ë ÏÓÁ˙Í‡, ÛÒÚ‡ÌÓ‚ÂÌË ˜ÂÁ FISH (Fluorescence in situ hybridization). ä‡ÚÓ Ô˙‚‡ ÔÓÒÎÂ‰Ëˆ‡ Ì‡ êçä ÚÓÍÒË˜ÌÓÒÚÚ‡ ÔË ·ÓÎÂÒÚÚ‡ Ì‡ Steinert Â ‰ËÒÙÛÌÍˆËﬂ Ì‡ ‰‚‡ ÍÎ‡Ò‡ êçä Ò‚˙Á‚‡˘Ë ÔÓÚÂËÌË, ÍÓÂÚÓ ‚Ó‰Ë ‰Ó ‡·ÌÓÏÌ‡ Â„ÛÎ‡ˆËﬂ Ì‡ ‡ÎÚÂÌ‡ÚË‚ÌËﬂ ÒÔÎ‡ÈÒËÌ„ ( ÔË Ú‡ÌÒÍË·ˆËﬂÚ‡ Ë ÒËÌÚÂÁ‡Ú‡ Ì‡ Ò˙‰Â˜ÌËﬂ ÚÓÔÓÌËÌ í, ËÌÒÛÎËÌÓ‚Ëﬂ ÂˆÂÔÚÓ, ıÎÓË‰ÌËﬂ Í‡Ì‡Î 1- ÓÒÌÓ‚ÂÌ ‚ ÏÛÒÍÛÎËÚÂ) Ë ÒÔÎ‡ÈÒÂÓÔ‡ÚËﬂ, ÂÁÛÎÚ‡Ú, ÓÚ ÍÓÂÚÓ Ò‡ ÏËÓÚÓÌËﬂÚ‡ Ë ËÌÒÛÎËÌÓ‚‡Ú‡ ÂÁËÒÚÂÌÚÌÓÒÚ. èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â ÚÂÁË Ë·ÓÌÛÍÎÂ‡ÌË ‚ÍÎ˛˜‚‡ÌËﬂ ÏÓ„‡Ú ‰‡ Ì‡Û¯‡Ú Ë ‰Û„Ë ÍÎÂÚ˙˜ÌË ÙÛÌÍˆËË. DMPK ÒÂ ÔÓ‰ÛˆË‡ ‚ „ÓÎÂÏË ÍÓÎË˜ÂÒÚ‚‡ ‚ ÒÍÂÎÂÚÌËÚÂ ÏÛÒÍÛÎÌË ÍÎÂÚÍË Ë Í‡‰ËÓÏËÓˆËÚËÚÂ, ‰ÓÍ‡Á‡ÚÂÎÒÚ‚Ó Á‡ ÍÓÂÚÓ Â ÏÛÒÍÛÎÌÓÚÓ Ë Ò˙‰Â˜ÌÓÚÓ Á‡Òﬂ„‡ÌÂ 188 ÔË Ô‡ˆËÂÌÚËÚÂ Ò ·ÓÎÂÒÚÚ‡ Ì‡ Steinert. Ç ÏÛÒÍÛÎËÚÂ ÚÓÁË ÔÓÚÂËÌ ÒÂ ÓÚÍË‚‡ ÔÓÒÚÒËÌ‡ÔÚË˜ÌÓ ‚ ÌÂ‚ÓÏÛÒÍÛÎÌËÚÂ ÒËÌ‡ÔÒË, ‡ ‚ Í‡‰ËÓÏËÓˆËÚËÚÂ ‚ Ó·Î‡ÒÚÚ‡ Ì‡ ËÌÚÂÍ‡Î‡ÚÌËÚÂ ‰ËÒÍÓ‚Â (18). Ç ñçë ÏËÓÚÓÌËÌ ÔÓÚÂËÌ ÍËÌ‡Á‡Ú‡ ÒÂ ÎÓÍ‡ÎËÁË‡ ‚ Ó·Î‡ÒÚÚ‡ Ì‡ ÒËÌ‡ÔÒËÚÂ ‚ Ï‡ÎÍËﬂ ÏÓÁ˙Í, ÒÂ‰ÌËﬂ ÏÓÁ˙Í, ıËÔÓÍ‡ÏÔ‡, ÔÓ‰˙Î„Ó‚‡ÚËﬂ ÏÓÁ˙Í Ë Ó·Î‡ÒÚÚ‡ Ì‡ ‡ÔËÍ‡ÎÌ‡Ú‡ ÏÂÏ·‡Ì‡ Ì‡ ÂÔÂÌ‰ËÏÌËÚÂ ÍÎÂÚÍË Ë ÍÎÂÚÍËÚÂ Ì‡ ıÓËÓË‰ÌËﬂ ÔÎÂÍÒÛÒ. ÇÂÓﬂÚÌÓ Ò˙˘ÂÒÚ‚Û‚‡Ú ‰‚‡ ÓÒÌÓ‚ÌË Ô‡ÚÓ„ÂÌÂÚË˜ÌË ÏÂı‡ÌËÁ˙Ï‡, ÍÓËÚÓ Ó·ÛÒÎ‡‚ﬂÚ ÔÓﬂ‚ËÚÂ ÓÚ ÒÚ‡Ì‡ Ì‡ ñçë ÔË Ô‡ˆËÂÌÚË Ò åÑ ÚËÔ I. ● ç‡ÎË˜ËÂÚÓ Ì‡ Ì‡ ‡ÎÂÎË Ò „ÓÎﬂÏ ·ÓÈ CTG-ÔÓ‚ÚÓË Ë Ò˙ÓÚ‚ÂÚÒ‚‡˘ËÚÂ ËÏ CUG Ëêçä ÏÓÊÂ ‰‡ Ì‡Û¯Ë ÂÍÒÔÂÒËﬂÚ‡ Ì‡ „ÂÌË, Â„ÛÎË‡˘Ë ‡ÌÌËÚÂ ÂÚ‡ÔË ÓÚ ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙Í‡, ÍÓÂÚÓ ÒÂ ÓÚÍË‚‡ ÔË ÏË¯ÍË-ÏÓ‰ÂÎË (18; 48; 74) . íÓ‚‡ ÏÓÊÂ ‰‡ ·˙‰Â ÔË˜ËÌ‡Ú‡ ÔË Ô‡ˆËÂÌÚË Ò „ÓÎﬂÏ‡ ÚËÔÎÂÚÌ‡ ÂÍÒÔ‡ÌÁËﬂ (>1000), ÍÓÌ„ÂÌËÚ‡ÎÌË ÙÓÏË Ë ÒÎÛ˜‡Ë Ò ÚÂÊ˙Í ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ, Á‡Òﬂ„‡˘ ‚ÒË˜ÍË ÂÎÂÏÂÌÚË Ì‡ Ó·˘ÓÚÓ ÍÓ„ÌËÚË‚ÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ, ‚Â·‡ÎÌËÚÂ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË (51). ● ÇËÒÓÍËÚÂ ÍÓÎË˜ÂÒÚ‚‡ Ì‡ DMPK êçä Ò CUG-ÂÍÒÔ‡ÌÁËﬂ, ÂÍÒÔÂÒË‡Ì‡ ‚ ÍÓÚËÍ‡ÎÌËÚÂ Ë ÔÓ‰ÍÓÓ‚ËÚÂ ÌÂ‚ÓÌË, ÏÓÊÂ ‰‡ Ì‡Û¯Ë Ô‡‚ËÎÌËﬂ ÒÔÎ‡ÈÒËÌ„ Ì‡ Ú‡Û ÔÓÚÂËÌËÚÂ ‚˙‚ ÙÓÌÚÓÚÂÏÔÓ‡ÎÌËﬂ ÍÓÚÂÍÒ, Ì‡ N-ÏÂÚËÎ-D-‡ÒÔ‡Ú‡Ú ÂˆÂÔÚÓ 1 (NMDAR 1) Ë ‡ÏËÎÓË‰ ÔÂÍÛÒÓÌËﬂ ÔÓÚÂËÌ (APP). íÓ‚‡ ÏÓÊÂ ‰‡ ‰Ó‚Â‰Â ‰Ó ÔÓ„ÂÒË‡˘ Ò ‚˙Á‡ÒÚÚ‡ ÛÔ‡‰˙Í Ì‡ ÙÓÌÚ‡ÎÌËÚÂ Ë ÚÂÏÔÓ‡ÎÌËÚÂ ÍÓ„ÌËÚË‚ÌË ÙÛÌÍˆËË (‰˙Î„ÓÒÓ˜Ì‡ Ë ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌ‡ Ô‡ÏÂÚ) ÔË Ô‡ˆËÂÌÚË Ò Ï‡ÎÍ‡ CTG-ÂÍÒÔ‡ÌÁËﬂ. íÂÁË ‰‡ÌÌË Ó·ÛÒÎ‡‚ﬂÚ ‚ÍÎ˛˜‚‡ÌÂÚÓ Ì‡ åÑ ÚËÔ 1 Í˙Ï „ÂÌÂÚË˜ÌÓ ‰ÂÚÂÏËÌË‡ÌËÚÂ Ú‡ÛÔ‡ÚËË (13; 38; 61). ● èË ÏË¯Ë ÏÓ‰ÂÎË DMK ÒÂ ÎÓÍ‡ÎËÁË‡ ‚ ÒËÌ‡ÔÒËÚÂ Ì‡ Ï‡ÎÍËﬂ ÏÓÁ˙Í, ıËÔÓÍ‡ÏÔ‡ Ë ÏÓÁ˙˜ÌËﬂ ÒÚ‚ÓÎ, ÍÓÂÚÓ ‚ÂÓﬂÚÌÓ Ò˙˘Ó ËÏ‡ ÁÌ‡˜ÂÌËÂ Á‡ ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ (19; 62). Ç˙ÔÂÍË ˜Â ‡ÁÎË˜ÌË „ÂÌÌË ÎÓÍÛÒË Ò‡ ÓÚ„Ó‚ÓÌË Á‡ ‰‚ÂÚÂ ÙÓÏË Ì‡ ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ, ÚÂ ÒÂ Ó·ÛÒÎ‡‚ﬂÚ ÓÚ ÒıÓ‰ÌË Ô‡ÚÓÙËÁËÓÎÓ„Ë˜ÌË ÏÂı‡ÌËÁÏË Ë ËÏ‡Ú ÒıÓ‰ÂÌ ÙÂÌÓÚËÔ, Ú.Â. ‚ Ô‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ PROMM ÒÂ ÔÂ‰ÔÓÎ‡„‡Ú ‡Ì‡ÎÓ„Ë˜ÌË Ì‡ åÑ 1 ÏÂı‡ÌËÁÏË. èÄíéãéÉéÄçÄíéåàü ë‚ÂÚÎËÌÌÓÏËÍÓÒÍÓÔÒÍÓÚÓ ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ÏÛÒÍÛÎË ÔË ÚÂÁË Ô‡ˆËÂÌÚË ÓÚÍË‚‡ ÌÂÒÔÂˆËÙË˜ÌË ÔÓÏÂÌË, ÒıÓ‰ÌË Ò ÚÂÁË ÔË ‰Û„Ë ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË Í‡ÚÓ ÌÂÍÓÁ‡ Ì‡ ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡. èË ÚÂÁË Ô‡ˆËÂÌÚË ÌÂ Â ÚËÔË˜Ì‡ ËÎË Â ÒÎ‡·Ó ËÁ‡ÁÂÌÓ ‡Á‡ÒÚ‚‡ÌÂÚÓ Ì‡ Ò˙Â‰ËÌËÚÂÎÌ‡ Ú˙Í‡Ì. á‡ åÑ 1 Â ı‡‡ÍÚÂÌ‡ ‡ÚÓÙËﬂÚ‡ Ì‡ Ì‡ ÚËÔ 1 ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡, ÍÓÂÚÓ ÒÂ ÒÂ˘‡ Ë ÔË Emery-Dreifuss ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ. èË ÌÂÓÌ‡Ú‡ÎÌ‡Ú‡ ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ Ò‡ ‚ Ò˙ÒÚÓﬂÌËÂ Ì‡ ◊Ï‡ÚÛ‡ˆËÓÌÂÌ ‡ÂÒÚ” Ì‡ ‡ÁÎË˜ÌË ÂÚ‡ÔË ÓÚ ‡Á‚ËÚËÂÚÓ: ÏËÓ·Î‡ÒÚË, ÏËÓÚÛ·ÛÎË, ÌÂÔ˙ÎÌ‡ ıËÒÚÓıËÏË˜Ì‡ ‰ËÙÂÂÌˆË‡ˆËﬂ Ì‡ ÚËÔ 1 Ë ÚËÔ 2 ‚Î‡ÍÌ‡, ÂÍÒÔÂÒËﬂ Ì‡ ËÌÚÂÏÂ‰ËÂÌË ÙÂÚ‡ÎÌË ÙËÎ‡ÏÂÌÚË Í‡ÚÓ ‰ÂÁÏËÌ Ë ‚ËÏÂÌÚËÌ (59). èË ÌﬂÍÓË ÒÎÛ˜‡Ë Ì‡ ÍÓÌ„ÂÌËÚ‡ÎÌ‡ ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ ÒÂ ÓÚÍË‚‡ ‰ËÒÔÓÔÓˆËﬂ Ì‡ ÏËÓÙË·ËÎËÚÂ, ÚËÔË˜Ì‡ Á‡ ÌﬂÍÓË ÍÓÌ„ÂÌËÚ‡ÎÌË ÏËÓÔ‡ÚËË.èË åÑ 2 ‡ÚÓÙË˜ÌËÚÂ ËÁÏÂÌÂÌËﬂ Á‡Òﬂ„‡Ú ÔÂ‰ËÏÌÓ ÏÛÒÍÛÎÌË ‚Î‡ÍÌ‡ ÚËÔ 2. ëÔÂˆËÙË˜ÌËÚÂ ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ Ò‡ Ò‚˙Á‡ÌË Ò ‡ÁÌÓÓ·‡ÁÌË ÏÓÙÓÎÓ„Ë˜ÌË ‡·ÌÓÏÌÓÒÚË. ç‡È-ÚËÔË˜ÌË Ò‡ ÌÂ‚ÓÙË·ËÎÂÌËÚÂ ‰Â„ÂÌÂ‡ˆËË, ‚˙ÚÂÍÎÂÚ˙˜ÌËÚÂ ˆËÚÓÔÎ‡ÁÏÂÌË ‚ÍÎ˛˜‚‡ÌËﬂ, Ï‡ÒÚÌËÚÂ „‡ÌÛÎË‡ÌË ÍÎÂÚÍË, ÎÂÁËËÚÂ Ì‡ ÔÓ‰ÍÓÓ‚ÓÚÓ ·ﬂÎÓ ÏÓÁ˙˜ÌÓ ‚Â˘ÂÒÚ‚Ó, Ò Û‚ÂÊ‰‡ÌÂ Ì‡ ÏËÂÎËÌÓ‚ËÚÂ Ó·‚Ë‚ÍË ÔË ÓÚÌÓÒËÚÂÎÌÓ Á‡Ô‡ÁÂÌË ‡ÍÒÓÌË, Í‡ÍÚÓ Ë ÍÓÓ‚‡Ú‡ ‡ÚÓÙËﬂ (16; 17; 23; 52; 76 ). á‡ Ô‡ˆËÂÌÚË Ò ÍÓÌ„ÂÌËÚ‡ÎÌ‡ ÙÓÏ‡ Ì‡ åÑ 1 ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÔÓ-ÚËÔË˜ÌË Ò‡ ÍÓÓ‚‡Ú‡ ‡ÚÓÙËﬂ, ıËÔÓÔÎ‡ÁËﬂ Ì‡ corpus callosum Ë ‚ÂÌÚËÍÛÎÓÏÂ„‡ÎËﬂÚ‡ (16).íÂÌ‰ÂÌˆËﬂÚ‡ Á‡ ÒËÏÂÚË˜ÌÓ ÒÎË‚‡ÌÂ Ì‡ ÎÂÁËËÚÂ Ì‡ ·ﬂÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚Â˘ÂÒÚ‚Ó Ë ÔÓ„ÂÒË‡˘‡ ‚ÂÌÚËÍÛÎÓÏÂ„‡ÎËﬂ, ÍÓﬂÚÓ ÔÓ Ò‚ÓﬂÚ‡ ÚÂÊÂÒÚ ÍÓÂÎË‡ Ò ÔÓ‰˙ÎÊËÚÂÎÌÓÒÚÚ‡ Ì‡ Á‡·ÓÎﬂ‚‡ÌÂÚÓ, ÔÂ‰ÔÓÎ‡„‡Ú ·‡‚ÌÓ ÔÓ„ÂÒË‡˘ ÔÓˆÂÒ Ì‡ ‰ÂÏËÂÎËÌËÁ‡ˆËﬂ (16; 18; 35). íÓÈ Â ‚˙ÁÏÓÊÌÓ ‰‡ Â ‚ÒÎÂ‰ÒÚ‚ËÂ Ì‡ Ì‡Û¯ÂÌ ÔÓˆÂÒ Ì‡ Ó·‡ÁÛ‚‡ÌÂ Ì‡ ÏËÂÎËÌÓ‚ËÚÂ Ó·‚Ë‚ÍË ÔÓ ‚ÂÏÂ Ì‡ ÙÂÚ‡ÎÌÓÚÓ ‡Á‚ËÚËÂ. Ç˙ÚÂÌÂ‚ÓÌÌËÚÂ ‡„Â„‡ÚË Ì‡ ıËÔÂÙÓÒÙÓËÎË‡Ì Ô‡ÚÓÎÓ„Ë˜ÂÌ Ú‡Û-ÔÓÚÂËÌ ÒÂ ÓÚÍË‚‡Ú ‚ ÏÓÁ˙ˆËÚÂ Ì‡ Ô‡ˆËÂÌÚË Ò ‡ÁÎË˜ÌË ÌÂ‚Ó‰Â„ÂÌÂ‡ÚË‚ÌË Á‡·ÓÎﬂ‚‡ÌËﬂ, ‚ÍÎ. Ë åËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ I ÚËÔ (61; 62). çÂ‚ÓÙË·ËÎÂÌËÚÂ ‰Â„ÂÌÂ‡ˆËË ÒÂ ÓÚÍË‚‡Ú ‚ ÎËÏ·Ë˜Ì‡Ú‡ ÒËÒÚÂÏ‡, ÏÓÁ˙˜ÌËﬂ ÒÚ‚ÓÎ, ıËÔÓÍ‡ÏÔ‡, ÂÌÚÓËÌ‡ÎÌËﬂ ÍÓÚÂÍÒ Ë Ë ÚÂÏÔÓ‡ÎÌËÚÂ ÍÓÓ‚Ë ÁÓÌË (49; 73). çÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒÂÌËÎÌË ÔÎ‡ÍË Ë ‡ÏËÎÓË‰ÌË ‰ÂÔÓÁËÚË. ëÔÂˆËÙË˜ÌËÚÂ ÌÂ‚ÓÔ‡ÚÓÎÓ„Ë˜ÌË ·ÂÎÂÁË ÔË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ Ì‡ Steinert Ò‡ Ò‚˙Á‡ÌË Ò ÛÔ‡‰˙Í Ì‡ ÂÁËÍÓ‚ËÚÂ Ë ÂÍÁÂÍÛÚË‚ÌËÚÂ ÙÛÌÍˆËË, ÒıÓ‰ÂÌ Ò ÚÓÁË ÔË ÙÓÌÚÓ-ÚÂÏÔÓ‡ÎÌ‡ ‰ÂÏÂÌˆËﬂ. äãàçàóçÄ äÄêíàçÄ èêà êÄáãàóçàíÖ îéêåà çÄ ÅéãÖëííÄ çÄ STEINERT ãÂÍ‡Ú‡ ÙÓÏ‡ ÒÂ ı‡‡ÍÚÂËÁË‡ ÍÎËÌË˜ÂÒÍË Ò Í‡Ú‡‡ÍÚ‡, ÓÔÎÂ¯Ë‚ﬂ‚‡ÌÂ, ÏËÌËÏ‡ÎÂÌ ‰Ó ÎËÔÒ‚‡˘ ÏÛÒÍÛÎÂÌ ‰ÂÙËˆËÚ ËÎË Á‡ı‡ÂÌ ‰Ë‡·ÂÚ. ÅÓﬂÚ Ì‡ CTG ÔÓ‚ÚÓËÚÂ Â ÏÂÊ‰Û 50 Ë 150 (40) . èË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡ Ì‡˜‡ÎÓÚÓ Â Í˙Ï 20-30, ÔÓﬂ‰ÍÓ ÒÎÂ‰ 40 „Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ. ÅÓﬂÚ Ì‡ ÚËÌÛÍÎÂÓÚË‰ÌËÚÂ ÔÓ‚ÚÓË Â 300-1000. èÓ„ÂÒËﬂÚ‡ È Â Ò‡‚ÌËÚÂÎÌÓ ·‡‚Ì‡. ï‡‡ÍÚÂËÁË‡ ÒÂ Ò ÏËÓÚÓÌË˜ÌË ÔÓﬂ‚Ë, ÔÂ‰¯ÂÒÚ‚‡˘Ë ËÎË ‡Á‚Ë‚‡˘Ë ÒÂ Â‰ÌÓ‚ÂÏÂÌÌÓ Ò ÏÛÒÍÛÎÌ‡Ú‡ ÒÎ‡·ÓÒÚ Ë ‡ÚÓÙËﬂ ‚ ‰ËÒÚ‡ÎÌËÚÂ ÏÛÒÍÛÎË Ì‡ Í‡ÈÌËˆËÚÂ (1). á‡Òﬂ„‡Ú ÒÂ Ë m. sternocleidomastoideus, ÏÛÒÍÛÎËÚÂ Ì‡ ¯ËﬂÚ‡ ËÁÚ˙Ìﬂ‚‡Ú, ¯ËÈÌ‡Ú‡ ÎÓ‰ÓÁ‡ ÒÂ Û‚ÂÎË˜‡‚‡, ÔÓﬂ‚ﬂ‚‡ ÒÂ Ú. Ì‡. ◊ÎÂ·Â‰Ó‚‡ ¯Ëﬂ”. ìÒÚ‡ÌÓ‚ﬂ‚‡Ú ÒÂ ıËÔÓÚÓÙËË Ì‡ ‰˙‚Í‡ÚÂÎÌËÚÂ ÏÛÒÍÛÎË (ıÎ˙Ú‚‡ÌÂ Ì‡ ·ÛÁËÚÂ Ë ÒÎÂÔÓÓ˜ËﬂÚ‡), ÎÂ‚‡ÚÓËÚÂ Ì‡ ÍÎÂÔ‡˜ËÚÂ (ÔÚÓÁ‡) Ë ÎËˆÂ‚ËÚÂ ÏÛÒÍÛÎË. íÂÁË ·ÂÎÂÁË, Á‡Â‰ÌÓ Ò ÙÓÌÚ‡ÎÌÓÚÓ ÓÔÎÂ¯Ë‚ﬂ‚‡ÌÂ Ë „ÓÎÂÏËÚÂ ÓÚÎÂÔÂÌË Û¯Ë ÔË‰‡‚‡Ú ı‡‡ÍÚÂÂÌ ‚Ë‰ Ì‡ ·ÓÎÌËÚÂ. ê‡Á‚Ë‚‡ ÒÂ ‡ÚÓÙËﬂ Ì‡ ÔÓ‰·Â‰ËˆËÚÂ Ò˙Ò ÒÚÂÔ‡ÊÌ‡ ÔÓıÓ‰Í‡, ‡ÚÓÙËﬂ Ì‡ ÔÂ‰ÏË¯ÌËˆËÚÂ Ë ÍËÚÍ‡Ú‡. ÄÚÓÙËËÚÂ Ò‡ ‰‚ÛÒÚ‡ÌÌË, ÒËÏÂÚË˜ÌË (1). èÓÍÒËÏ‡ÎÌËÚÂ ÏÛÒÍÛÎË Ò‡ Ò‡‚ÌËÚÂÎÌÓ ÒÎ‡·Ó Á‡ÒÂ„Ì‡ÚË. ë Ì‡ÔÂ‰‚‡ÌÂ Ì‡ ·ÓÎÂÒÚÚ‡ ÏËÓÚÓÌË˜ÌËÚÂ ÙÂÌÓÏÂÌË ËÁ˜ÂÁ‚‡Ú Ë ÔÂÓ·Î‡‰‡‚‡˘Ë Ò‡ ÏÛÒÍÛÎÌ‡Ú‡ ÒÎ‡·ÓÒÚ Ë ‡ÚÓÙËﬂÚ‡. èË ÌﬂÍÓË Ô‡ˆËÂÌÚË ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÔÓÎËÌÂ‚ÓÔ‡ÚËﬂ ÓÚ ‰ÂÏËÂÎËÌËÁË‡˘ ÚËÔ Ò ÔÂÓ·Î‡‰‡‚‡˘‡ Á‡„Û·‡ Ì‡ ‰Â·ÂÎÓÏËÂÎËÌËÁË‡ÌË ‚Î‡ÍÌ‡. èÓﬂ‚Ë ÓÚ ÒÚ‡Ì‡ Ì‡ Ò˙‰Â˜ÌÓÒ˙‰Ó‚‡Ú‡ ÒËÒÚÂÏ‡ Ò‡ ÏÌÓ„Ó ÚËÔË˜ÌË Ë ‚ÍÎ˛˜‚‡Ú ‡ÒËÏÔÚÓÏÌË ÖäÉ ÔÓÏÂÌË ÔË 90% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÔÓ‚Ó‰ÌËÚÂ (Û‰˙ÎÊ‡‚‡ÌÂ Ì‡ PR- ËÌÚÂ‚‡Î‡, AV·ÎÓÍ) Ë ËÚ˙ÏÌËÚÂ (Í‡ÏÂÌË Ë Ì‡‰Í‡ÏÂÌË ‡ËÚÏËË) Ì‡Û¯ÂÌËﬂ, Í‡‰ËÓÏËÓÔ‡ÚËﬂ (ÔÓ-ﬂ‰ÍÓ ‚ Ò‡‚ÌÂÌËÂ Ò ‰Û„Ë ÏÛÒÍÛÎÌË ‰ËÒÚÓÙËË). íÂÁË Ô‡ˆËÂÌÚË Ò‡ Ò ‚ËÒÓÍ ËÒÍ ÓÚ ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú. (29) á‡Òﬂ„‡ÌÂÚÓ Ì‡ ‰Ë‡Ù‡„Ï‡Ú‡ Ë ‰Ëı‡ÚÂÎÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡ ‚Ó‰Ë ‰Ó ıËÔÓ‚ÂÌÚËÎ‡ˆËﬂ, ÍÓÂÚÓ Ì‡Î‡„‡ ÔÂËÓ‰Ë˜ÌÓ ÔÓ‚ÂÊ‰‡ÌÂ Ì‡ îàÑ Ë Í˙‚ÌÓ-„‡ÁÓ‚ ‡Ì‡ÎËÁ. èË ÓÍÓÎÓ 70% ÓÚ Ï˙ÊÂÚÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ıËÔÂ„ÓÌ‡‰ÓÚÓÔÂÌ ıËÔÓ„ÓÌ‡‰ËÁ˙Ï (‚ËÒÓÍË ÌË‚‡ Ì‡ ãï Ë îëï ÔË ÌÓÏ‡ÎÂÌ ËÎË ÔÓÌËÊÂÌ ÚÂÒÚÓÒÚÂÓÌ) Ò ‡ÚÓÙËﬂ Ì‡ ÚÂÒÚËÒËÚÂ Ë ‡ÁÓÓÒÔÂÏËﬂ. èË 15-20% ÓÚ ÊÂÌËÚÂ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ‰ËÒÏÂÌÓÂﬂ Ë ‡ÌÌ‡ ÏÂÌÓÔ‡ÛÁ‡. èÓ‚Ë¯ÂÌ Â ËÒÍ˙Ú ÓÚ ÒÔÓÌÚ‡ÌÌË ‡·ÓÚË. óÂÒÚÓ Â Ì‡ÎËˆÂ ËÌÒÛÎËÌÓ‚‡ ÂÁËÒÚÂÌÚÌÓÒÚ, Í‡ÍÚÓ Ë Ì‡Û¯ÂÌ ÎËÔË‰ÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï (29). åÌÓ„Ó ÚËÔË˜ÌË Ó˜ÌË ÔÓﬂ‚Ë Ò‡ ‰‚ÛÒÚ- 189 ‡ÌÌËÚÂ Á‡‰ÌÓÍÓÚËÍ‡ÎÌË Í‡Ú‡‡ÍÚË Ë ÚÂÁË ÚËÔ Christmas tree. èË„ÏÂÌÚÌ‡Ú‡ ‰Â„ÂÌÂ‡ˆËﬂ Ì‡ ÂÚËÌ‡Ú‡, ÛÒÚ‡ÌÓ‚ÂÌ‡ ˜ÂÁ ÂÎÂÍÚÓÂÚËÌÓ„‡ÙËﬂ, ÏÓÊÂ ‰‡ ÔÂ‰¯ÂÒÚ‚‡ ÏÛÒÍÛÎÌËÚÂ ÔÓﬂ‚Ë. éÚ ÒÚ‡Ì‡ Ì‡ ı‡ÌÓÒÏËÎ‡ÚÂÎÌ‡Ú‡ ÒËÒÚÂÏ‡ ÒÂ ÓÚÍË‚‡Ú ‰ËÒÙ‡„Ëﬂ ÔÓ‡‰Ë ÒÎ‡·ÓÒÚ Ì‡ Ù‡ËÌ„Â‡ÎÌ‡Ú‡ ÏÛÒÍÛÎ‡ÚÛ‡, „‡ÒÚÓÂÁÓÙ‡„Â‡ÎÂÌ ÂÙÎÛÍÒ, Ì‡Ï‡ÎÂÌ ˜Â‚ÂÌ ÏÓÚËÎËÚÂÚ, ÍÓÌÒÚËÔ‡ˆËﬂ, ıÓÎÂÎËÚË‡Á‡. èË ÓÍÓÎÓ 50% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò‡ ÔÓ‚Ë¯ÂÌË Äî Ë ÉÉí ÔÓ ÌÂﬂÒÂÌ ÏÂı‡ÌËÁ˙Ï ÔË ÎËÔÒ‡ Ì‡ ˜ÂÌÓ‰Ó·ÌÓ Û‚ÂÊ‰‡ÌÂ (30). ÇÒÎÂ‰ÒÚ‚ËÂ Ì‡ ÛÒÍÓÂÌ IgG- ÏÂÚ‡·ÓÎËÁ˙Ï ÔË ÓÍÓÎÓ 1/3 ÓÚ Ô‡ˆËÂÌÚËÚÂ ÌË‚‡Ú‡ ÏÛ Ò‡ ÔÓÌËÊÂÌË. èË ÏËÓÚÓÌË˜Ì‡Ì‡ ‰ËÒÚÓÙËﬂ Ì‡ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó Ì‡˜‡ÎÓÚÓ Â ÏÂÊ‰Û 1 Ë 10 „Ó‰ËÌË. ç‡È- ˜ÂÒÚÓ ÚÓ‚‡ Ò‡ ‰Âˆ‡ Ò ÌÓÏ‡ÎÌÓ ÔÂ- Ë ÔÓÒÚÌ‡Ú‡ÎÌÓ ‡Á‚ËÚËÂ ÔÂÁ Ô˙‚‡Ú‡ „Ó‰ËÌ‡ ÓÚ ÊË‚ÓÚ‡, ˜ËËÚÓ Ô‡ÚÓÎÓ„Ë˜ÌË ÓÚÍÎÓÌÂÌËﬂ („ÂÌÂ‡ÎËÁË‡Ì‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ, Á‡Òﬂ„‡˘‡ ÓÒÌÓ‚ÌÓ ÎËˆÂÚÓ Ë ‰ËÒÚ‡ÎÌËÚÂ ˜‡ÒÚË Ì‡ Í‡ÈÌËˆËÚÂ) ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÓÍÓÎÓ ÔÓıÓÊ‰‡ÌÂÚÓ (21). èÓÌﬂÍÓ„‡ ÏÛÒÍÛÎÌ‡Ú‡ ÒÎ‡·ÓÒÚ ÏÓÊÂ ‰‡ ·˙‰Â ‰ËÒÍÂÚÌÓ ÔÓﬂ‚ÂÌ‡. ÇÓ‰Â˘ Â ÍÓ„ÌËÚË‚ÌËﬂ ‰ÂÙËˆËÚ Ò ÚÛ‰ÌÓÒÚË ‚ Á‡Û˜‡‚‡ÌÂÚÓ, ‰ËÁ‡ÚËﬂ, ËÁÓÒÚ‡‚‡ÌÂ ‚ Û˜ËÎË˘Â Ë Á‡ÚÛ‰ÌÂÌ‡ ÒÓˆË‡ÎÌ‡ ËÌÚÂ„‡ˆËﬂ. ç‡ÎËˆÂ Â Ô‡ÚÓÎÓ„Ë˜Ì‡ Ò˙ÌÎË‚ÓÒÚ. óÂÒÚË Ò‡ ÔÓﬂ‚ËÚÂ ÓÚ ÒÚ‡Ì‡ Ì‡ ëëë Ò ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËﬂ, ÍÓÂÚÓ Ó·ÛÒÎ‡‚ﬂ ÔÓ‚Ë¯ÂÌËﬂ ËÒÍ ÓÚ ‚ÌÂÁ‡ÔÌ‡ Ò˙‰Â˜Ì‡ ÒÏ˙Ú (40). äÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ ÒÂ Ì‡·Î˛‰‡‚‡ ÔÓ-˜ÂÒÚÓ ÔË ÌÓ‚ÓÓ‰ÂÌËÚÂ Ì‡ Ï‡ÈÍË Ò ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ. èË ÚÂÁË ‰Âˆ‡ ·ÓﬂÚ Ì‡ CTG- ÔÓ‚ÚÓËÚÂ Ì‡‰ı‚˙Îﬂ 1500 (7). íﬂ Â ÎÂÚ‡ÎÌ‡ ‚ ÓÍÓÎÓ 16% ÓÚ ÒÎÛ˜‡ËÚÂ. èÂÌ‡Ú‡ÎÌÓ ÒÂ ÔÓﬂ‚ﬂ‚‡ Ò ıË‰‡ÏÌËÓÌ, Ò ÓÚÒÎ‡·ÂÌË ‰‚ËÊÂÌËﬂ Ì‡ ÔÎÓ‰‡, ËÒÍ ÓÚ ‡ÚÓ„ËÔÓÁ‡ (29). èË ‡Ê‰‡ÌÂÚÓ ÓÒÌÓ‚ÌËÚÂ ÒËÏÔÚÓÏË Ò‡ ÎËˆÂ‚‡ ‰ËÔÎÂ„Ëﬂ (tented upper lip), „ÂÌÂ‡ÎËÁË‡Ì‡ ÒÎ‡·ÓÒÚ, ıËÔÓÚÓÌËﬂ Ë ÂÒÔË‡ÚÓÂÌ ‰ËÒÚÂÒ (3; 29). äÎËÌË˜ÌÓ ÎËÔÒ‚‡ ÏËÓÚÓÌËﬂ, ÍÓﬂÚÓ ÏÓÊÂ ‰‡ ÒÂ Ó·ÂÍÚË‚ËÁË‡ ˜ÂÁ ÖåÉ (67). çË‚‡Ú‡ Ì‡ ÍÂ‡ÚËÌ ÍËÌ‡Á‡ ‚ Í˙‚Ú‡ Ò‡ ÌÓÏ‡ÎÌË, ÎËÔÒ‚‡Ú ÒÔÂˆËÙË˜ÌË ÔÓÏÂÌË ‚ ÏÛÒÍÛÎÌËÚÂ ·ËÓÔÒËË (68). åÓÚÓÌÓÚÓ ‡Á‚ËÚËÂ Ì‡ ÚÂÁË ‰Âˆ‡ Â Á‡·‡‚ÂÌÓ, ÌÓ ÚÂ ÒÂ Ì‡Û˜‡‚‡Ú ‰‡ ıÓ‰ﬂÚ. åÛÒÍÛÎÌ‡Ú‡ ıËÔÓÚÓÌËﬂ Ë ‰‚Ë„‡ÚÂÎÌ‡Ú‡ ÒÎ‡·ÓÒÚ ÏÓ„‡Ú ‰‡ ÒÂ ÔÓ‰Ó·ﬂÚ Ò „Ó‰ËÌËÚÂ, ÌÓ ÔÓ-Í˙ÒÌÓ ÒÂ ‡Á‚Ë‚‡Ú ÒËÏÔÚÓÏËÚÂ Ì‡ ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡. ÑÓ 11-„Ó‰Ë¯Ì‡ ‚˙Á‡ÒÚ ÒÂ ÔÓﬂ‚ﬂ‚‡Ú ÍÎËÌË˜ÂÒÍË Ë ÏËÓÚÓÌË˜ÌËÚÂ ÙÂÌÓÏÂÌË. 50-60% ÓÚ ‰Âˆ‡Ú‡ Ò‡ Ò ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ. èË˜ËÌ‡Ú‡ Á‡ ÚÓ‚‡ ‚ÒÂ Ó˘Â ÌÂ Â ﬂÒÌ‡, ‚˙ÔÂÍË ˜Â ÔË ‡Ê‰‡ÌÂÚÓ ˜ÂÒÚÓ Ò‡ Ì‡ÎËˆÂ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËﬂ, ıËÔÓÚÓÙËﬂ Ì‡ corpus callosum Ë ‚ÂÌÚËÍÛÎÌ‡ ‰ËÎ‡Ú‡ˆËﬂ. é·Ò˙Ê‰‡ ÒÂ ÍÓÏÔÎÂÍÒÌËﬂ ÂÙÂÍÚ Ì‡ DMPK-ÏÛÚ‡ˆËﬂÚ‡ Ë ‡ÌÌ‡Ú‡ ‰Ëı‡ÚÂÎÌ‡ ÌÂ‰ÓÒÚ‡Ú˙˜ÌÓÒÚ. äãàçàóçÄ äÄêíàçÄ èêà PROMM íﬂ Â ÒıÓ‰Ì‡, ÌÓ ÔÓ- ÎÂÍ‡ ÓÚ Ú‡ÁË ÔË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡ Ì‡ åÑ 1, Ò ÏËÓÚÓÌËﬂ, ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ, Í‡Ú‡‡ÍÚ‡, ‰Ë‡·ÂÚ, ËÚ˙ÏÌË Ë ÔÓ‚Ó‰ÌË Ì‡Û¯ÂÌËﬂ. ÑËÒÚÓÙËﬂÚ‡ Á‡Òﬂ„‡ ÓÒÌÓ‚ÌÓ ÔÓÍÒËÏ‡ÎÌËÚÂ ÏÛÒÍÛÎË (ÙÎÂÍÒÓËÚÂ Ë ÂÍÒÚÂÌÁÓËÚÂ Ì‡ ·Â‰‡Ú‡, ÙÎÂÍÒÓËÚÂ Ì‡ ¯ËﬂÚ‡, ÂÍÒÚÂÌÁÓËÚÂ Ì‡ ÏË¯ÌËˆ‡Ú‡), ·ÂÁ ‰‡ ÒÂ ‡ÒÓˆËË‡ Ò ÚÂÊÍË ‡ÚÓÙËË Ì‡ ÏÛÒÍÛÎËÚÂ Ì‡ ÎËˆÂÚÓ Ë ÔÂ‰ÏË¯ÌËˆËÚÂ. ÄÌÚËˆËÔ‡ˆËﬂÚ‡ Â ÏÌÓ„Ó ÔÓ-ÒÎ‡·Ó ËÁ‡ÁÂÌ‡ ‚ Ò‡‚ÌÂÌËÂ Ò ·ÓÎÂÒÚÚ‡ Ì‡ Steinert (40). äéÉçàíàÇçà çÄêìòÖçàü, ëèÖñàîàóçà èêà êÄáãàóçàíÖ îéêåà çÄ åàéíéçàóçÄ Ñàëíêéîàü é˘Â ÔË Ô˙‚ËÚÂ ÓÔËÒ‡ÌËﬂ Ì‡ Ô‡ˆËÂÌÚË Ò ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ ÒÂ ÓÚÍË‚‡Ú Ë ÍÓ„ÌËÚË‚ÌË Ì‡Û¯ÂÌËﬂ, ÍÓËÚÓ ‚‡Ë‡Ú ÓÚ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ ÔË ÍÓÌ„ÂÌËÚ‡Î- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology Ì‡Ú‡ ÙÓÏ‡ (8) ‰Ó ÒÔÂˆËÙË˜ÌË ÍÓ„ÌËÚË‚ÌË, ÂÍÁÂÍÛÚË‚ÌË, ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌË, ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ë ÔÒËıË˜ÂÒÍË Ì‡Û¯ÂÌËﬂ ÔË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡ (4; 48). ë˙˘ÂÒÚ‚Û‚‡Ú ‡ÁÎË˜ÌË ÚÂÓËË ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÚÓ‚‡ ‰‡ÎË ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ, ˜Â Ò‡ Ò ÔÓ„ÂÒË‡˘ ËÎË ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ. Ç Á‡˘ËÚ‡ Ì‡ ÒÚ‡ÌÓ‚Ë˘ÂÚÓ Á‡ ÎËÔÒ‡Ú‡ Ì‡ ÔÓ„ÂÒËﬂ Â ÚÂÓËﬂÚ‡, ˜Â ÍÓ„ÌËÚË‚ÌËﬂ ÛÔ‡‰˙Í ÔÓ-ÒÍÓÓ Â ÂÁÛÎÚ‡Ú ÓÚ Ì‡Û¯ÂÌËÂ ‚ ‡Á‚ËÚËÂÚÓ Ì‡ ñçë, ‡ ÌÂ ÒÎÂ‰ÒÚ‚ËÂ ÓÚ ‰Â„ÂÌÂ‡ÚË‚ÂÌ ÔÓˆÂÒ (25; 37; 41). èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂÚÓ ÓÚ Ï‡ÈÍ‡Ú‡, ÍÓÂÚÓ ‚ ÔÓ‚Â˜ÂÚÓ ÒÎÛ˜‡Ë ÍÓÂÎË‡ Ò ÔÓ-„ÓÎﬂÏ ·ÓÈ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ ÚËÌÛÍÎÂÓÚË‰ÌË ÔÓ‚ÚÓË, Â Ò ÏÌÓ„Ó ÔÓ-‚ËÒÓÍ ËÒÍ ÓÚ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ ‚ Ò‡‚ÌÂÌËÂ Ò ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂÚÓ ÓÚ ·‡˘‡Ú‡. éÔËÒ‡ÌË Ò‡ Ë ÒÎÛ˜‡Ë Ò „ÓÎﬂÏ ·ÓÈ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ ÔÓ‚ÚÓË, Ò ÚÂÊÍÓ ÒËÒÚÂÏÌÓ Á‡Òﬂ„‡ÌÂ, ÌÓ ·ÂÁ Ò˙˘ÂÒÚ‚ÂÌ ÍÓ„ÌËÚË‚ÂÌ ‰ÂÙËˆËÚ (11; 53). íÓÁË Ù‡ÍÚ ÏÓÊÂ ‰‡ ·˙‰Â Ó·ﬂÒÌÂÌ Ò˙Ò ÒÓÏ‡ÚË˜ÂÌ ÏÓÁ‡ËˆËÁ˙Ï. ÉÓÎÂÏËﬂÚ ·ÓÈ Ì‡ ÔÓ‚ÚÓËÚÂ, ËÁÓÎË‡Ì ÓÚ ÎÂ‚ÍÓˆËÚËÚÂ ÍÓÂÎË‡ Ò ·Óﬂ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ ÔÓ‚ÚÓË ‚ ÏËÓˆËÚËÚÂ, ÔÓ‡‰Ë Ó·˘Ëﬂ ËÏ ÂÏ·ËÓÌ‡ÎÂÌ ÔÓËÁıÓ‰ ÓÚ ÏÂÁÓ‰ÂÏ‡Ú‡, ÌÓ ÌÂ ‚ËÌ‡„Ë Ò˙ÓÚ‚ÂÚÒÚ‚‡ Ì‡ ÂÍÒÔ‡ÌÁËﬂÚ‡ Ì‡ ÔÓ‚ÚÓËÚÂ ‚ ÌÂ‚ÓÌËÚÂ. èÓÚË‚ÓÂ˜Ë‚Ë Ò‡ ‰‡ÌÌËÚÂ ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ Ù‡ÍÚ‡ ‰‡ÎË ÍÓ„ÌËÚË‚ÌËﬂÚ ‰ÂÙËˆËÚ ÔË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡ Â ÂÁÛÎÚ‡Ú Ì‡ ‰Â„ÂÌÂ‡ÚË‚ÂÌ ÔÓˆÂÒ (5; 8). ìÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡ÌÂ Ò ÌÂÔÓ„ÂÒË‡˘ ı‡‡ÍÚÂ Â ÏÌÓ„Ó ı‡‡ÍÚÂÌÓ Á‡ ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡. á‡Òﬂ„‡Ú ÒÂ Ó·˘ÓÚÓ ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ (Ò ËÁﬂ‚‡ Ì‡ ÎÂÍ‡ ‰Ó ÛÏÂÂÌÓ ÚÂÊÍ‡ ÒÚÂÔÂÌ Ì‡ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ), ‚Â·‡ÎÌËÚÂ ÒÔÓÒÓ·ÌÓÒÚË, ÙÓÌÚ‡ÎÌËÚÂ Ë ÂÍÁÂÍÛÚË‚ÌË ÙÛÌÍˆËË ( 31; 48; 71). Harper Ë Ò˙‡‚Ú. ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú, ˜Â ÒÂ‰ÌËﬂÚ IQ (Intelligence Quotient- ÍÓÂÙËˆËÂÌÚ Ì‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ) ÔË ÚÂÁË Ô‡ˆËÂÌÚË Â ÓÍÓÎÓ 66 (29; 57). àÌÚÂÂÒÌÓ Â, ˜Â ÔË ÌﬂÍÓË ·ÓÎÌË ÛÏÒÚ‚ÂÌÓÚÓ ËÁÓÒÚ‡‚‡ÌÂ ÏÓÊÂ ‰‡ ÓÒÚ‡ÌÂ Â‰ËÌÒÚ‚ÂÌ‡ ÍÎËÌË˜Ì‡ ËÁﬂ‚‡ Á‡ ÌﬂÍÓÎÍÓ „Ó‰ËÌË ÒÎÂ‰ ‡Ê‰‡ÌÂÚÓ (64). èË ‰Âˆ‡ Ò Ú‡ÁË ÙÓÏ‡ ÒÂ ÓÚÍË‚‡Ú ÏÓÁ˙˜Ì‡ ‡ÚÓÙËﬂ, ıËÔÓÔÎ‡ÁËﬂ Ì‡ corpus callosum Ë ‚ÂÌÚËÍÛÎÌ‡ ‰ËÎ‡Ú‡ˆËﬂ Ó˘Â ÒÎÂ‰ ‡Ê‰‡ÌÂÚÓ, ÍÓËÚÓ ÍÓÂÎË‡Ú Ò ÚÂÊÂÒÚÚ‡ Ì‡ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ (37). ë˙‚ÍÛÔÌÓÒÚÚ‡ ÓÚ ÏÛÒÍÛÎÌÓ Á‡Òﬂ„‡ÌÂ, ÏÂÌÚ‡ÎÌ‡ ÂÚ‡‰‡ˆËﬂ Ë åêí-Ì‡ıÓ‰Í‡Ú‡ Ó·ÛÒÎ‡‚ﬂÚ ‚˙ÁÏÓÊÌÓÒÚÚ‡ ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ Ì‡ åÑ 1 ‰‡ ÒÂ ÓÚÌÂÒÂ Í˙Ï ÏËÓÂÌˆÂÙ‡ÎÓÔ‡ÚËËÚÂ (41). èË ÏËÓÚÓÌË˜Ì‡Ú‡ ‰ËÒÚÓÙËﬂ Ì‡ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó ÚËÔË˜Ì‡ ÓÒÓ·ÂÌÓÒÚ Ò‡ Ó·Û˜ËÚÂÎÌËÚÂ Á‡ÚÛ‰ÌÂÌËﬂ, ÍÓËÚÓ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÔË 69-86,7% ÓÚ Ô‡ˆËÂÌÚËÚÂ, Í‡ÚÓ ÓÒÌÓ‚ÌËÚÂ ÚÛ‰ÌÓÒÚË Ò‡ ÔË ˜ÂÚÂÌÂÚÓ (15; 26; 22). çÂ‚ÓÔÒËıÓÎÓ„Ë˜ÂÒÍËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ Ì‡ Ó·˘ÓÚÓ ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ ÔÓÍ‡Á‚‡Ú IQ ÔÓ-ÌËÒ˙Í ÓÚ Â‰ÌÓ ÒÚ‡Ì‰‡ÚÌÓ ÓÚÍÎÓÌÂÌËÂ ÔÓ‰ ÌÓÏ‡Ú‡, Í‡ÚÓ ÏÂÊ‰Û 20% Ë 80% ÓÚ Ô‡ˆËÂÌÚËÚÂ, ÒÔÓÂ‰ ‡ÁÎË˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ, Ò‡ Ò ‡ÁÎË˜Ì‡ ÒÚÂÔÂÌ Ì‡ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ, Ú.Â. IQ ÔÓ‰ 70% (4; 43; 44 65; 66). íÂÁË ‰Âˆ‡ Ò‡ Ò ÔÓ- ‚ËÒÓÍÓ IQ ‚ Ò‡‚ÌÂÌËÂ Ò Ô‡ˆËÂÌÚËÚÂ Ò ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ (65). èË ÔÓ‚ÂÊ‰‡ÌÂ Ì‡ ÌÂ‚ÓÔÒËıÓÎÓ„Ë˜ÂÒÍË ÚÂÒÚÓ‚Â ÒÂ Ë‰ÂÌÚËÙËˆË‡Ú ÌﬂÍÓË ÒÔÂˆËÙË˜ÌË ‰ÂÙËˆËÚË: ‚˙‚ ‚ÌËÏ‡ÌËÂÚÓ, ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ, ÍÓÌÒÚÛÍÚË‚ÌËÚÂ ÛÏÂÌËﬂ Ë ‚Â·‡ÎÌ‡Ú‡ Ô‡ÏÂÚ. íÂÁË ‰Âˆ‡ Ò‡ Ò ÔÓÚÂÌˆË‡ÎÂÌ ‰ÂÙËˆËÚ Ì‡ ÂÍÁÂÍÛÚË‚ÌËÚÂ Ë ÔÂˆÂÔÚË‚ÌÓ-ÏÓÚÓÌËÚÂ ÙÛÌÍˆËË ‚ ÏÂÊ‰Û 50 Ë 90%. ëÔÓÂ‰ ÏÓ‰ÂÎ‡ Ì‡ Baddeley ‡·ÓÚÌ‡Ú‡ Ô‡ÏÂÚ ÒÂ Ò˙ÒÚÓË ÓÚ ˆÂÌÚ‡ÎÌ‡ ÂÍÁÂÍÛÚË‚Ì‡ ÒËÒÚÂÏ‡, ‚ÍÎ˛˜ÂÌ‡ ‚ ËÁ·Ó‡ Ë ÛÔ‡‚ÎÂÌËÂÚÓ Ì‡ ‰ÂÈÌÓÒÚËÚÂ ÔÓ ‡ÁÔÂ‰ÂÎÂÌËÂ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ (10). Ç˙ÁÏÓÊÌÓ Â ËÌÚÂÎÂÍÚÛ‡ÎÌÓÚÓ Á‡Òﬂ„‡ÌÂ ÔË ‰ÂÚÒÍ‡Ú‡ ÙÓÏ‡ Ì‡ DM1 ‰‡ ÒÂ ‰˙ÎÊË Í‡ÍÚÓ Ì‡ ÙÓÌÓÎÓ„Ë˜Ì‡ ÔË˜ËÌ‡, ‚ ÒÏËÒ˙Î Ì‡ Ò˙ı‡ÌÂÌËÂ Ì‡ ËÌÙÓÏ‡ˆËﬂ Ë ‰ÂÙËˆËÚ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ, Ú‡Í‡ Ë Ì‡ Ì‡Û¯ÂÌËÂ ‚ ÔÎ‡ÌË‡ÌÂÚÓ Ë Ó„‡ÌËÁ‡ˆËﬂÚ‡ Ì‡ ‰ÂÈÌÓÒÚËÚÂ. àÁ‚ÂÒÚÌÓ Â, ˜Â ÚÂÁË ÍÓ„ÌË- 190 ÚË‚ÌË ÛÏÂÌËﬂ Ò‡ ÙÛÌÍˆËﬂ Ì‡ ÍÓÚËÍ‡ÎÌË ÏÂÊË ‚˙‚ ÙÓÌÚÓ-ÚÂÏÔÓ‡ÎÌËÚÂ Â„ËÓÌË, ÓÒÌÓ‚ÌÓ ‰ÓÁÓÎ‡ÚÂ‡ÎÌËﬂ ÔÂÙÓÌÚ‡ÎÂÌ ÍÓÚÂÍÒ, ÍÓÈÚÓ ÔÂÚ˙Ôﬂ‚‡ ÁÌ‡˜ËÚÂÎÌÓ ‡Á‚ËÚËÂ ÔÂÁ ÔÂËÓ‰‡ Ì‡ ‰ÂÚÒÚ‚ÓÚÓ (4). èË ÓÍÓÎÓ 26% ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò åÑ Ì‡ ‡ÌÌÓÚÓ ‰ÂÚÒÚ‚Ó ÒÂ ÓÚÍË‚‡Ú ıËÔÂ‡ÍÚË‚ÌÓ ÔÓ‚Â‰ÂÌËÂ Ò ‰ÂÙËˆËÚ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ, ‡ ÔË 40 %- ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ (4). ê‡ÁÎË˜ÌË ÙÓÏË Ì‡ ÚÂ‚ÓÊÌÓÒÚ (Ó·˘‡, ÓÚ ‡Á‰ﬂÎ‡ Ë Ú.Ì.) Ë ÙÓ·ËË ÒÂ ÓÔËÒ‚‡Ú ÔË ÚÂÁË Ô‡ˆËÂÌÚË (26; 65). èË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡ Ì‡ ·ÓÎÂÒÚÚ‡ Ì‡ Steinert ÔÓÌËÒÍË ÒÚÓÈÌÓÒÚË Ì‡ IQ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÔË Ô‡ˆËÂÌÚË Ò ÔÓ-„ÓÎﬂÏ‡ CTG-ÂÍÒÔ‡ÌÁËﬂ Ë ÛÌ‡ÒÎÂ‰ﬂ‚‡ÌÂ ÓÚ Ï‡ÈÍ‡Ú‡. ÑÓË Ô‡ˆËÂÌÚË Ò IQ ·ÎËÁ˙Í ‰Ó ÌÓÏ‡ÎÌËﬂ ÔÓÍ‡Á‚‡Ú Ì‡Û¯ÂÌËÂ ‚ ÂÍÁÂÍÛÚË‚ÌËÚÂ Ë ÂÁËÍÓ‚ËÚÂ ÙÛÌÍˆËË, ‚ÌËÏ‡ÌËÂÚÓ, ÁËÚÂÎÌ‡Ú‡ ÔÂˆÂÔˆËﬂ (14) Ë Ô‡ÏÂÚ, Í‡ÍÚÓ Ë ‚ ÍÓÌÒÚÛÍÚË‚ÌËÚÂ ÒÔÓÒÓ·ÌÓÒÚË (44; 49, 58). èÓÍÓÌÍÂÚÌÓ ‚ ÚÂÊÍ‡ ÒÚÂÔÂÌ Â Á‡ÒÂ„Ì‡ÚÓ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌÓÚÓ ÔËÔÓÏÌﬂÌÂ Ë ‚ ÔÓ-ÎÂÍ‡ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌ‡Ú‡ ÍÓÌÒÚÛÍÚË‚ÌÓÒÚ, ‚Â·‡ÎÌ‡Ú‡ Í‡ÚÍÓÒÓ˜Ì‡ Ë ‰˙Î„ÓÒÓ˜Ì‡ Ô‡ÏÂÚ ( 43). ç‡Û¯ÂÌËﬂÚ‡ ‚ ÔÓÒÚ‡ÌÒÚ‚ÂÌ‡Ú‡ ÓËÂÌÚ‡ˆËﬂ Ë ÁËÚÂÎÌ‡Ú‡ Ô‡ÏÂÚ ÍÓÂÎË‡Ú Ò ÛÒÚ‡ÌÓ‚ÂÌ‡Ú‡ ıËÔÓÔÂÙÛÁËﬂ ‚ Á‡‰ÌËÚÂ ÍÓÚËÍ‡ÎÌË Â„ËÓÌË Í‡ÚÓ cuneus Ë ÂÚÓÒÔÎÂÌË‡ÎÌËﬂ ÍÓÚÂÍÒ, Í‡ÍÚÓ Ë ‰ÂÒÌËﬂ Ô‡ËÂÚ‡ÎÂÌ ÎÓ· (43). IQ ÌÂ ÍÓÂÎË‡ Ò ÚÂÊÂÒÚÚ‡ Ì‡ ÏÛÒÍÛÎÌÓÚÓ Á‡Òﬂ„‡ÌÂ (49). Rubinstein Ë Ò˙‡‚Ú. 1997 ÔÂ‰ÔÓÎ‡„‡Ú, ˜Â Ô‡ÏÂÚÓ‚ËÚÂ ÙÛÌÍˆËË ÏÓ„‡Ú ‰‡ Ò‡ ÔÓ-˜Û‚ÒÚ‚ËÚÂÎÌË Ì‡ Ï‡Î˙Í ·ÓÈ Ì‡ CTG-ÔÓ‚ÚÓËÚÂ ‚ Ò‡‚ÌÂÌËÂ Ò Ó·˘ÓÚÓ ËÌÚÂÎÂÍÚÛ‡ÎÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ ÔË ÎÂÍËÚÂ ÙÓÏË Ì‡ ÏËÓÚÓÌË˜ÌËÚÂ ‰ËÒÚÓÙËË (43). èË Ô‡ˆËÂÌÚË Ò Ï‡Î˙Í ·ÓÈ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ ÔÓ‚ÚÓË Ì‡‰ ÌÓÏ‡Ú‡, ÍÓËÚÓ ÏÓÊÂ ‰‡ Ò‡ ÓÎË„Ó- ËÎË ‡ÒËÏÔÚÓÏÌË ÔÓ ÓÚÌÓ¯ÂÌËÂ Ì‡ ÏÛÒÍÛÎÌÓÚÓ Á‡Òﬂ„‡ÌÂ, Ò Ì‡ÔÂ‰‚‡ÌÂ Ì‡ ‚˙Á‡ÒÚÚ‡ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ÁÌ‡˜ËÏÓ Á‡Òﬂ„‡ÌÂ Ì‡ ‰˙Î„ÓÒÓ˜Ì‡Ú‡ Ë ÁËÚÂÎÌ‡Ú‡ Ô‡ÏÂÚ, ÍÓÂÚÓ Ò‚Ë‰ÂÚÂÎÒÚ‚‡ Á‡ ‰ËÒÙÛÌÍˆËﬂ Ì‡ ÚÂÏÔÓ‡ÎÌËﬂ ‰ﬂÎ (48). èË „ÓÎﬂÏ‡ ˜‡ÒÚ ÓÚ Ô‡ˆËÂÌÚËÚÂ Ò‡ Á‡ÒÂ„Ì‡ÚË ÙÛÌÍˆËËÚÂ Ì‡ ÙÓÌÚ‡ÎÌËﬂ ‰ﬂÎ, Í‡ÚÓ ÔÎ‡ÌË‡ÌÂ, ÍÓÌÚÓÎ Ì‡ ‚ÌËÏ‡ÌËÂÚÓ, ÍÓÂÚÓ ÏÓÊÂ ‰‡ ‚ÎËﬂÂ ‚˙ıÛ ÍÓ„ÌËˆËﬂÚ‡. çﬂÍÓË ÔÓÛ˜‚‡ÌËﬂ ÔÂ‰ÔÓÎ‡„‡Ú Ò˙˘ÂÒÚ‚Û‚‡ÌÂÚÓ Ì‡ ÚÂÒÌË ‚˙ÁÍË Á‡‰‡˜ËÚÂ Á‡ Â¯‡‚‡ÌÂ Ì‡ ÔÓ·ÎÂÏË, Î‡ÚÂ‡ÎÌËﬂ ÔÂÙÓÌÚ‡ÎÂÌ ÍÓÚÂÍÒ Ë Ó·˘‡Ú‡ ËÌÚÂÎË„ÂÌÚÌÓÒÚ. èÂ‰ÔÓÎ‡„‡ ÒÂ, ˜Â ÍÓ„ÌËÚË‚ÌËﬂÚ ÛÔ‡‰˙Í, ‚ ˜‡ÒÚÌÓÒÚ ÒÏÛ˘ÂÌËÂÚÓ Ì‡ ÂÍÁÂÍÛÚË‚ÌËÚÂ Ë ÂÁËÍÓ‚ËÚÂ ÙÛÌÍˆËË ÏÓÊÂ ÔÓ„ÂÒË‡ Ò ‚˙Á‡ÒÚÚ‡, ÌÓ ÌÂ Â ÛÒÚ‡ÌÓ‚ÂÌ‡ ÔÓ-„ÓÎﬂÏ‡ ÚÂÊÂÒÚ Ë ÔÓ-·˙Á‡ ÔÓ„ÂÒËﬂ ÔË Ô‡ˆËÂÌÚË Ò ÔÓ-„ÓÎﬂÏ‡ CTG-ÂÍÒÔ‡ÌÁËﬂ (49; 58). èÓ„ÂÒËﬂÚ‡ ÒÂ Ó·ﬂÒÌﬂ‚‡ Ò Ì‡ÚÛÔ‚‡ÌÂÚÓ Ì‡ ÏÛÚ‡ÌÚÌË DMPK-Ú‡ÌÒÍËÔÚË ‚ ﬂ‰‡Ú‡ Ì‡ ÌÂ‚ÓÌËÚÂ, ÍÓËÚÓ ÔÓ ÚÓÁË Ì‡˜ËÌ Ì‡Û¯‡‚‡Ú ‡ÎÚÂÌ‡ÚË‚ÌËﬂ ÒÔÎ‡ÈÒËÌ„ Ë Â„ÛÎ‡ˆËﬂÚ‡ Ì‡ Ú‡ÌÒÍËÔˆËﬂÚ‡. èË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚ Ì‡ Steinert Ò‡ ÓÔËÒ‡ÌË Ì‡Ï‡ÎÂÌ‡ ÒÔÓÒÓ·ÌÓÒÚ Á‡ ‡ÁÔÓÁÌ‡‚‡ÌÂ Ì‡ ÎËˆÂ‚‡Ú‡ ÂÍÒÔÂÒËﬂ Ë ÂÏÓˆËÓÌ‡ÎÌÓ ÙÛÌÍˆËÓÌË‡ÌÂ (69;75), ÍÓÂÚÓ ‚ÂÓﬂÚÌÓ Â Ò‚˙Á‡ÌÓ Ò˙Ò Á‡Òﬂ„‡ÌÂ Ì‡ ÎËÏ·Ë˜ÌËÚÂ ÒÚÛÍÚÛË- ‰‚ÛÒÚ‡ÌÌË ÒÛ·ÍÓÚËÍ‡ÎÌË ÎÂÁËË ‚ ÔÂ‰ÌËÚÂ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â, ‚ÍÎ. ‡ÏË„‰‡Î‡Ú‡ Ë ÂÌÚÓËÌ‡ÎÌËﬂ ÍÓÚÂÍÒ ËÎË Ó·‡·ÓÚÍ‡Ú‡ Ì‡ ÔÓÎÛ˜ÂÌ‡Ú‡ ËÌÙÓÏ‡ˆËﬂ ÓÚ ÚÂÏÓ‡ÎÌËÚÂ ËÎË ÙÓÌÚ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â. ÄÏË„‰‡Î‡Ú‡ ÒÂ ‡ÒÓˆËË‡ Ò ‡ÁÔÓÁÌ‡‚‡ÌÂ Ì‡ ÎËˆÂ‚‡Ú‡ ÂÍÒÔÂÒËﬂ Ì‡ ÓÚËˆ‡ÚÂÎÌË ÂÏÓˆËË Í‡ÚÓ ÒÚ‡ı Ë „Ìﬂ‚. ÇÂÓﬂÚÌÓ ËÌÒÛÎ‡Ú‡ ËÏ‡ ÔÓ‰Ó·ÌË ÙÛÌÍˆËË (2). ç‡Ï‡ÎÂÌ‡Ú‡ ˜Û‚ÒÚ‚ËÚÂÎÌÓÒÚ Í˙Ï ÌÂ„‡ÚË‚ÌË ÂÏÓˆËË ÏÓÊÂ ‰‡ Â ÔË˜ËÌ‡ ÚÂÁË Ô‡ˆËÂÌÚË ‰‡ Ò‡ ÔÓ-ÒÎ‡·Ó ÍÓÓÔÂ‡ÚË‚ÌË Ë ÒÍÎÓÌÌË Í˙Ï ÂÏÔ‡ÚËﬂ (75). ãË˜ÌÓÒÚÓ‚Ë Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË (Í‡ÚÓ ÔÓ‰ÓÁËÚÂÎÌÓ ÔÓ‚Â‰ÂÌËÂ, Â„ÓˆÂÌÚË˜ÌÓÒÚ Ë ·ÂÁ‡ÁÎË˜ËÂ) Ì‡Û¯ÂÌËﬂ ÔË ÚÂÁË ·ÓÎÌË Ò‡ ÓÔËÒ‡ÌË Ë ÓÚ Ò‡ÏËﬂ Steinert. ÅÓÎÌËÚÂ Ò‡ Ò ıÓÏÓ„ÂÌÂÌ ÔÓ‚Â‰ÂÌ˜ÂÒÍË ÔÓÙËÎ Ò˙Ò ÒÚ‡ÚËÒÚË˜ÂÒÍË ÁÌ‡˜ËÏË ‡ÁÎË˜Ëﬂ Á‡ ÓÚ·ﬂ„‚‡ÌÂ, Ó·ÒÂÒË‚ÌÓ-ÍÓÏÔÛÎÒË‚ÌË, Ô‡- ÑÂÍÂÏ‚Ë 2009 Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÒË‚ÌÓ-‡„ÂÒË‚ÌË Ë ¯ËÁÓË‰ÌË ˜ÂÚË (20) èÓ‚Â‰ÂÌËÂÚÓ Ì‡ ÓÚ·ﬂ„‚‡ÌÂ ÒÂ ı‡‡ÍÚÂËÁË‡ Ò˙Ò ÒÚ‡ı ÓÚ ÚÓ‚‡ ‰‡ ·˙‰‡Ú ÓˆÂÌﬂ‚‡ÌË, ÍËÚËÍÛ‚‡ÌË ËÎË ÓÚıÎ˙ÎÂÌË ÓÚ ‰Û„ËÚÂ. íÂÁË Ô‡ˆËÂÌÚË ÚÛ‰ÌÓ ÒÂ ÒÔËﬂÚÂÎﬂ‚‡Ú ËÎË Á‡ÔÓ˜‚‡Ú ÌÓ‚‡ ‡·ÓÚ‡. ÑÂÔÂÒËﬂÚ‡ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ ˜ÂÒÚÓ, ÌÓ ÌÂ Â ﬂÒÌÓ ‰‡ÎË Â Ô˙‚Ë˜Ì‡ ËÎË ‚ÚÓË˜Ì‡, ÔÓ‡‰Ë ÙËÁË˜ÂÒÍËÚÂ Ó„‡ÌË˜ÂÌËﬂ, ‰Ó ÍÓËÚÓ ‚Ó‰Ë Á‡·ÓÎﬂ‚‡ÌÂÚÓ. ÇÒ˙˘ÌÓÒÚ Ô‡ˆËÂÌÚËÚÂ ÌﬂÏ‡Ú ÚÂÊÍË ÒËÏÔÚÓÏË Ì‡ ÚÂ‚ÓÊÌÓÒÚ ËÎË ‰ÂÔÂÒËﬂ, ‡ ÔÓ-ÒÍÓÓ- ÂÏÓˆËÓÌ‡ÎÂÌ ‰ÂÙËˆËÚ (43). èË Ô‡ˆËÂÌÚË Ò åÑ ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡ Ë ÔÓ‚Ë¯ÂÌ‡ ‰ÌÂ‚Ì‡ Ò˙ÌÎË‚ÓÒÚ, ÍÓﬂÚÓ ‚ÂÓﬂÚÌÓ Â Ò ÍÓÏÔÎÂÍÒÂÌ ı‡‡ÍÚÂ, Ó·ÛÒÎÓ‚ÂÌ‡ Â ÓÚ ÔÓ„ÂÒË‡˘‡Ú‡ ÂÒÔË‡ÚÓÌ‡ ‰ËÒÙÛÌÍˆËﬂ Ë ˜ÂÒÚËÚÂ ÔÓﬂ‚Ë Ì‡ ‰ÂÔÂÒËﬂ (54; 55). äÓ„ÌËÚ‚ÌË Ì‡Û¯ÂÌËﬂ ÔË Ô‡ˆËÂÌÚË Ò PROMM ÇÒÂ Ó˘Â ÌÂ Â ﬂÒÌÓ ‰‡ÎË ÒÔÂÍÚ˙˙Ú Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ ÔË PROMM e ÒıÓ‰eÌ Ò ÚÂÁË ÔË ·ÓÎÂÒÚÚ‡ Ì‡ Steinert (43). ìÒÚ‡ÌÓ‚ÂÌË Ò‡ Ì‡Û¯ÂÌËﬂ ‚ ÁËÚÂÎÌÓÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÛÏÂÌËﬂ ‚ ÔÓ-ÎÂÍ‡ ÒÚÂÔÂÌ ‚ Ò‡‚ÌÂÌËÂ Ò ÚËÔ I. äÓ„ÌËÚË‚ÌËÚÂ ‡·ÌÓÏÌÓÒÚË ÌÂ ÍÓÂÎË‡Ú Ò ÔÓÏÂÌËÚÂ ‚ åêí Ì‡ıÓ‰Í‡Ú‡ (18). ÑË‡„ÌÓÒÚË˜ÌËﬂÚ ‡Î„ÓËÚ˙Ï ‚ÍÎ˛˜‚‡: ÄÌ‡ÏÌÂÁ‡, ‚ÍÎ. Ù‡ÏËÎÌ‡; çÂ‚ÓÎÓ„Ë˜ÂÌ ÒÚ‡ÚÛÒ; ÅËÓıËÏË˜ÌË ËÁÒÎÂ‰‚‡ÌËﬂ (ëêä Â ÌÓÏ‡ÎÌ‡; ïÓÏÓÌ‡ÎÌË ËÁÒÎÂ‰‚‡ÌËﬂ- Á‡ ÓÔÂ‰ÂÎﬂÌÂ Ì‡ ÙÛÌÍˆËﬂÚ‡ Ì‡ Ô‡ÌÍÂ‡Ò‡ Ë „ÓÌ‡‰Ì‡Ú‡ ÓÒ); ÖåÉ Ò ‰‡ÌÌË Á‡ ÏËÓÚÓÌË˜ÌË Á‡ÎÔÓ‚Â Ë ÏËÓ„ÂÌÌ‡ Û‚Â‰‡; ÖäÉ, ïÓÎÚÂ-ÖäÉ Ë ÖıÓäÉ ● Ö‚ÓÍË‡ÌË ÔÓÚÂÌˆË‡ÎË á‡Òﬂ„‡ÌÂÚÓ Ì‡ ñçë ÔË Ô‡ˆËÂÌÚË Ò ÏËÓÚÓÌË˜Ì‡ ‰ËÒÚÓÙËﬂ ÒÂ Ó·ÂÍÚË‚ËÁË‡ Ë ˜ÂÁ ËÁÒÎÂ‰‚‡ÌÂÚÓ Ì‡ ÒÎÛıÓ‚ËÚÂ (70) Ë ÁËÚÂÎÌËÚÂ (24) Â‚ÓÍË‡ÌË ÔÓÚÂÌˆË‡ÎË. çﬂÍÓË ËÁÒÎÂ‰‚‡ÌËﬂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú Û‰˙ÎÊÂÌË ˆÂÌÚ‡ÎÌË ‚ÂÏÂÌ‡ Ì‡ ÔÓ‚ÂÊ‰‡ÌÂ ÔÓ ÒÓÏ‡ÚÓÒÂÌÁÓÌËÚÂ Ô˙ÚË˘‡ (24; 27;60; 72) Ë ÔÓ‚Ë¯ÂÌ‡ ÌÂ‚Ì‡ ‚˙Á·Û‰ËÏÓÒÚ Ì‡ Ô˙‚Ë˜ÌËﬂ ÒÂÚË‚ÂÌ ÍÓÚÂÍÒ (47), ÍÓÂÚÓ ÔÂ‰ÔÓÎ‡„‡ ÒÛ·ÍÎËÌË˜ÌÓ Á‡Òﬂ„‡ÌÂ Ì‡ ÒÓÏ‡ÚÓÒÂÌÁÓÌËÚÂ Ô˙ÚË˘‡ Ë Ô˙‚Ë˜Ì‡Ú‡ ÒÂÚË‚Ì‡ ÍÓ‡. ë‡‚ÌËÚÂÎÌÓ Ï‡ÎÍÓ Ò‡ ÔÓ‚Â‰ÂÌËÚÂ ÔÓÛ˜‚‡ÌËﬂ, ËÁÛ˜‡‚‡˘Ë ÙÛÌÍˆËÓÌË‡ÌÂÚÓ Ì‡ ˆÂÌÚ‡ÎÌ‡Ú‡ ÒËÒÚÂÏ‡ Ì‡ ÏÓÚÓÂÌ ÍÓÌÚÓÎ. Oliveri Ë Ò˙‡‚Ú. ÔÂÁ 1997 ˜ÂÁ Ú‡ÌÒÍ‡ÌË‡ÎÌ‡ Ï‡„ÌËÚÌ‡ ÒÚËÏÛÎ‡ˆËﬂ Ì‡ ÏÓÚÓÌ‡Ú‡ ÍÓ‡ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ÁÌ‡˜ËÚÂÎÌÓ Û‰˙ÎÊÂÌÓ ˆÂÌÚ‡ÎÌÓ ÏÓÚÓÌÓ ‚ÂÏÂ Ì‡ ÔÓ‚ÂÊ‰‡ÌÂ (50). ë‚˙Á‡ÌËÚÂ Ò ‰‚ËÊÂÌËÂ ÍÓÓ‚Ë ÔÓÚÂÌˆË‡ÎË Ò‡ ÂÁÛÎÚ‡Ú ÓÚ ËÁ‚˙¯‚‡ÌÂ Ì‡ ‚ÓÎÂ‚Ë ‰‚ËÊÂÌËﬂ Ë Ò‡ ‚‡ÊÌË Á‡ ÓˆÂÌÍ‡Ú‡ Ì‡ ÔÓ‰„ÓÚÓ‚Í‡Ú‡ Ë ËÁ‚˙¯‚‡ÌÂÚÓ Ì‡ ‰‚ËÊÂÌËﬂÚ‡ Ì‡ ˆÂÌÚ‡ÎÌÓÌÂ‚ÌÓ ÌË‚Ó (63). ÄÍÓ ÒÂ Ò‡‚ÌﬂÚ NS’ (negative slope) Ë BP (Bereitshaftpotential) ÔË Ô‡ˆËÂÌÚËÚÂ Ò åÑ, NS’ Â ÁÌ‡˜ËÏÓ ÒÌËÊÂÌ Í‡ÚÓ ‡ÏÔÎËÚÛ‰‡ ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË Ë Ô‡ˆËÂÌÚË Ò ‰Û„Ë ÌÂ‚ÓÏÛÒÍÛÎÌË Á‡·ÓÎﬂ‚‡ÌËﬂ Í‡ÚÓ ëÔËÌ‡ÎÌ‡ ÏÛÒÍÛÎÌ‡ ‡ÚÓÙËﬂ, ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ ÚËÔ ÔÓﬂÒ-Í‡ÈÌËÍ Ë Ù‡ˆËÓÒÍ‡ÔÛÎÓıÛÏÂ‡ÎÌ‡ ÏÛÒÍÛÎÌ‡ ‰ËÒÚÓÙËﬂ. íÂÁË ÓÚÍËÚËﬂ ÔÂ‰ÔÓ„‡Ú ‰ËÒÙÛÌÍˆËﬂ Ì‡ ÒËÒÚÂÏ‡Ú‡ Ì‡ ˆÂÌÚ‡ÎÂÌ ÏÓÚÓÂÌ ÍÓÌÚÓÎ, Ò‚˙Á‡Ì‡ Ò ÔÓ‰„ÓÚÓ‚Í‡Ú‡ Ë ËÁÔ˙ÎÌÂÌËÂÚÓ Ì‡ ‰‚ËÊÂÌËﬂÚ‡, Ó·ÛÒÎ‡‚ﬂ˘ËÚÂ ÏÂı‡ÌËÁÏË, Á‡ ÍÓÂÚÓ ‚ÒÂ Ó˘Â ÌÂ Ò‡ ËÁ‚ÂÒÚÌË (46). ● çÂ‚ÓÔÒËıÓÎÓ„Ë˜ÂÒÍË ËÁÒÎÂ‰‚‡ÌËﬂ óÂÁ Úﬂı ÒÂ ËÁÛ˜‡‚‡Ú ‡ÁÎË˜ÌËÚÂ ‡ÒÔÂÍÚË Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ‰ÂÙËˆËÚ ÔË ÚÂÁË Ô‡ˆËÂÌÚË. ● é·‡ÁÌË ËÁÒÎÂ‰‚‡ÌËﬂ Ì‡ ñçë: åêí ÔË Ô‡ˆËÂÌÚË Ò åÑ ÚËÔ I ÔÓÍ‡Á‚‡Ú ÍÓÓ‚‡ ‡ÚÓÙËﬂ, ÔÓÏÂÌË ‚ ÔÓ‰ÍÓÓ‚ÓÚÓ ·ﬂÎÓ ÏÓÁ˙˜ÌÓ ‚Â˘ÂÒÚ‚Ó ÔÓ ÚËÔ‡ Ì‡ ‰ÂÏËÂÎËÌËÁ‡ˆËË, ÓÒÌÓ‚ÌÓ ‚˙‚ ÙÓÌÚ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â Ë ÔÂ‰ÌËÚÂ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â, Í‡ÎˆËÙËÍ‡ÚË ‚ ·‡Á‡ÎÌËÚÂ „‡Ì„ÎËË (16; 34). Ç˙ÔÂÍË ˜Â ÚÂÊÂÒÚÚ‡ Ì‡ ÏÓÁ˙˜Ì‡ ‡ÚÓÙËﬂ ÌÂ ÍÓÂÎË‡ Ì‡Ô˙ÎÌÓ Ò ÚÂÊÂÒÚÚ‡ Ì‡ ÍÓ„ÌËÚË‚ÌËﬂ ‰ÂÙËˆËÚ, ÎÂÁËËÚÂ ‚ ·ﬂÎÓÚÓ ÏÓÁ˙˜ÌÓ ‚Â˘ÂÒÚ‚Ó, ‡·ÌÓÏÌÓÒÚËÚÂ ‚ ÔÂ‰ÌËÚÂ ÚÂÏÔÓ‡ÎÌË ‰ﬂÎÓ‚Â Ë ‚ÂÌÚËÍÛÎÓÏÂ„‡ÎËﬂÚ‡ Ò‡ ÏÌÓ„Ó ÔÓ-ÚËÔË˜ÌË ÔË Ô‡ˆËÂÌÚË Ò ÚÂÊÍÓ Á‡ÒÂ„Ì‡Ú ËÌ- àÁÒÎÂ‰‚‡ÌË ÍÓ„ÌËÚË‚ÌË ÙÛÌÍˆËË é·˘Ó ËÁÒÎÂ‰‚‡ÌÂ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ ÙÛÌÍˆËË è‡ÏÂÚ ÇÂ·‡ÎÌ‡-ÒÎÛıÓ‚‡ áËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌ‡ Rey complex figure test EÁËÍÓ‚‡ ÙÛÌÍˆËË Controlled association letters test ÖÍÁÂÍÛÚË‚ÌË ÙÛÌÍˆËË Ë ‚ÌËÏ‡ÌËÂ ÇÂ·‡ÎÌ‡ ÙÎÛÂÌÚÌÓÒÚ óÂÚÂÌÂ 191 çÂ‚ÓÔÒËıÓÎÓ-„Ë˜ÂÌ ÚÂÒÚ Wechsler intelligence scale for children Stanford-Binet scale Raven progressive matrices Digit span Rey auditory verbal learning Story recall Block span Rey-Ostereich figure recall Spatial learning test Token test Controlled association category test Stroop test Trial making test Wisconsin card sorting test Tower of London Alertness without auditory warning signal Alertness with auditory warning signal Divided attention Verbal fluency test (semantic and phonetic) National adult reading test ÚÂÎÂÍÚ (32; 45). á‡ Ô‡ˆËÂÌÚË Ò ÍÓÌ„ÂÌËÚ‡ÎÌ‡ ÙÓÏ‡ Ì‡ åÑ 1 ÔÓ-ÚËÔË˜ÌË Ò‡ ÍÓÓ‚‡Ú‡ ‡ÚÓÙËﬂ, ıËÔÓÔÎ‡ÁËﬂ Ì‡ corpus callosum Ë ‚ÂÌÚËÍÛÎÓÏÂ„‡ÎËﬂÚ‡ (16). ãÂÁËË ‚ ÔÓ‰ÍÓÓ‚ÓÚÓ ·ﬂÎÓ ÏÓÁ˙˜ÌÓ ‚Â˘ÂÒÚ‚Ó ÔË ÚÂÁË Ô‡ˆËÂÌÚË ÒÂ ÓÚÍË‚‡Ú ‚ ÔÓ-Ì‡ÔÂ‰Ì‡Î‡ ‚˙Á‡ÒÚ. ëÔÂˆËÙË˜Ì‡ ÓÒÓ·ÂÌÓÒÚ Ò‡ ıËÔÂËÌÚÂÌÁÌËÚÂ ÎÂÁËË ‚ „ÓÌËﬂ Á‡‰ÂÌ ÚË˙„˙ÎÌËÍ – trigone (37). èË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡ Ì‡ ·ÓÎÂÒÚÚ‡ Ì‡ Steinert ÔÓı‡‡ÍÚÂÌË Ò‡ ıËÔÂËÌÚÂÌÁÌËÚÂ Ì‡ í2 ÁÓÌË, ‡ÁÔÓÎÓÊÂÌË ÔÓ‰ÍÓÓ‚Ó ‚ ÚÂÏÔÓ‡ÎÌËÚÂ, ÙÓÌÚ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â Ë ÔÂË‚ÂÌÚËÍÛÎ‡ÌÓ ( 9; 36). ç‡ 3-D åêí ËÁÒÎÂ‰‚‡ÌËﬂ, ÔË ÍÓËÚÓ ÏÓÁ˙˜ÌÓ-Ô‡ÂÌıËÏÌ‡Ú‡ Ù‡ÍˆËﬂ ÔË åÑ 1 Ë åÑ 2 ÒÂ Ò‡‚Ìﬂ‚‡ Ò˙Ò Á‰‡‚Ë ÍÓÌÚÓÎË, ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú, ˜Â ÔË ·ÓÎÂÒÚÚ‡ Ì‡ Steinert ÏÓÁ˙˜Ì‡Ú‡ ‡ÚÓÙËﬂ Â ÏÌÓ„Ó ÔÓ- ÚÂÊÍÓ ËÁ‡ÁÂÌ‡ ‚ Ò‡‚ÌÂÌËÂ Ò˙Ò Ò˙ÓÚ‚ÂÚÌË ÔÓ ‚˙Á‡ÒÚ ËÌ‰Ë‚Ë‰Ë. èË ·ÓÎÌË Ò PROMM ÚÂÁË ËÁÏÂÌÂÌËﬂ Ò‡ ÔÓ- ÒÎ‡·Ó ËÁ‡ÁÂÌË Ë Ò˙˘ÂÒÚ‚Û‚‡ ÚﬂÒÌ‡ ÍÓÂÎ‡ˆËﬂ Ò ‚˙Á‡ÒÚÚ‡ Ì‡ Ô‡ˆËÂÌÚËÚÂ, Ú. Â. ‡ÚÓÙËﬂÚ‡ ÔÓ„ÂÒË‡ Ò ‚˙Á‡ÒÚÚ‡, ÍÓÂÚÓ Â ÚËÔË˜ÌÓ Ë Á‡ Á‰‡‚ËÚÂ ÍÓÌÚÓÎË. îÛÌÍˆËÓÌ‡ÎÌË Ó·‡ÁÌË ËÁÒÎÂ‰‚‡ÌËﬂ: ç‡ SPECT (Single Photon Emission Computed Tomography) Ë PET (Positron Emission Tomography) ÒÂ ÛÒÚ‡ÌÓ‚ﬂ‚‡Ú ·ËÎ‡ÚÂ‡ÎÌÓ Ì‡Ï‡ÎÂÌ‡ ÔÂÙÛÁËﬂ Ë Â‰ÛˆË‡Ì „Î˛ÍÓÁÂÌ ÏÂÚ‡·ÓÎËÁ˙Ï ‚ „ÓÌËﬂ ÏÂÁË‡ÎÂÌ ÙÓÌÚ‡ÎÂÌ ÍÓÚÂÍÒ, ÔÂ‰ÌËﬂ ˆËÌ„ÛÎ‡ÂÌ, Ó·ËÚÓÙÓÌÚ‡ÎÌËﬂ, ÂÚÓÒÔÎÂÌË‡ÎÌËﬂ ÍÓÚÂÍÒ, ÓÔ‡¯‡ÚÓÚÓ ﬂ‰Ó, Ú‡Î‡ÏÛÒ‡ Ë ÚÂÏÔÓÓÔ‡ËÂÚ‡ÌËÚÂ ‰ﬂÎÓ‚Â (42). íËÔÎÂÚÌ‡Ú‡ ÂÍÒÔ‡ÌÁËﬂ Ì‡Û¯‡‚‡ Ì‡‚ÎËÁ‡ÌÂÚÓ Ì‡ „Î˛ÍÓÁ‡ ‚ ÍÎÂÚÍËÚÂ. ● ÉÂÌÂÚË˜ÌÓ ËÁÒÎÂ‰‚‡ÌÂ: ÅÓﬂÚ Ì‡ ‡ÏÔÎËÙËˆË‡ÌËÚÂ ÔÓ‚ÚÓË CTG ‚ DMPK-„ÂÌ‡ ÔË ·ÓÎÂÒÚÚ‡ Ì‡ Steinert Ë CCTG ‚ ZNF9-„ÂÌ‡ ÔË PROMM ÒÂ ÓÔÂ‰ÂÎﬂ ˜ÂÁ Southern blot ‡Ì‡ÎËÁ Ë PCR. áÄäãûóÖçàÖ åËÓÚÓÌË˜ÌËÚÂ ‰ËÒÚÓÙËË ÚËÔ I (·ÓÎÂÒÚ Ì‡ Steinert) Ë ÚËÔ II (PROMM) Ò‡ „ÂÌÂÚË˜ÌË ÏÛÎÚËÒËÒÚÂÏÌË Á‡·ÓÎﬂ‚‡ÌËﬂ, ÍÓËÚÓ ÍÎËÌË˜ÌÓ ÒÂ ı‡‡ÍÚÂËÁË‡Ú Ò ÔÓ„ÂÒË‡˘‡ ÏÛÒÍÛÎÌ‡ ÒÎ‡·ÓÒÚ Ë ÏËÓÚÓÌËﬂ. èÓﬂ‚ËÚÂ ÓÚ ÒÚ‡Ì‡ Ì‡ ñçë ÔË Ô‡ˆËÂÌÚË Ò ·ÓÎÂÒÚÚ‡ Ì‡ Steinert ÏÓ„‡Ú ‰‡ ‚‡Ë‡Ú ÓÚ ÛÏÒÚ‚ÂÌÓ ËÁÓÒÚ‡‚‡ÌÂ ÔË ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ ‰Ó ÒÔÂˆËÙË˜ÌË ÍÓ„ÌËÚË‚ÌË (ÓÒÌÓ‚ÌÓ Ì‡Û¯ÂÌËﬂ ‚ Å˙Î„‡ÒÍ‡ çÂ‚ÓÎÓ„Ëﬂ Bulgarian Neurology ÑÂÍÂÏ‚Ë 2009 ÂÍÁÂÍÛÚË‚ÌËÚÂ, Ô‡ÏÂÚÓ‚ËÚÂ Ë ÂÁËÍÓ‚ËÚÂ ÙÛÌÍˆËË) Ë ÔÓ‚Â‰ÂÌ˜ÂÒÍË Ì‡Û¯ÂÌËﬂ, ˜ÂÒÚË ÔË ÍÎ‡ÒË˜ÂÒÍËÚÂ ÙÓÏË. è‡ÚÓ„ÂÌÂÁ‡Ú‡ Ì‡ ÍÓ„ÌËÚË‚ÌËÚÂ Ì‡Û¯ÂÌËﬂ Â ÍÓÏÔÎÂÍÒÌ‡ Ë Â Ò‚˙Á‡Ì‡ Ò˙Ò ÒÏÛ˘ÂÌËÂ ‚ ÂÍÒÔÂÒËﬂÚ‡ Ì‡ „ÂÌË, Â„ÛÎË‡˘Ë ‡Á‚ËÚËÂÚÓ Ì‡ ÏÓÁ˙˜ÌËÚÂ ÒÚÛÍÚÛË ÔË ÍÓÌ„ÂÌËÚ‡ÎÌ‡Ú‡ ÙÓÏ‡ Ë Ì‡Û¯ÂÌ ÒÔÎ‡ÈÒËÌ„ Ì‡ Ú‡Û-ÔÓÚÂËÌËÚÂ ‚ ÌÂ‚ÓÌËÚÂ Ì‡ ÙÓÌÚÓÚÂÏÔÓ‡ÎÌËﬂ ÍÓÚÂÍÒ ÔË ÍÎ‡ÒË˜ÂÒÍ‡Ú‡ ÙÓÏ‡. èË PROMM ÔÓﬂ‚ËÚÂ ÓÚ ÒÚ‡Ì‡ Ì‡ ñçë Ò‡ ÔÓ-ÒÎ‡·Ó ÔÓÛ˜ÂÌË Ë ‚ÍÎ˛˜‚‡Ú ÎÂÍÓ Ì‡Û¯ÂÌËÂ Ì‡ ÁËÚÂÎÌÓ-ÔÓÒÚ‡ÌÒÚ‚ÂÌËÚÂ ÛÏÂÌËﬂ. èË Ô‡ˆËÂÌÚË Ò ÏËÚÓÌË˜ÌË ‰ËÒÚÓÙËË Ò‡ ÛÒÚ‡ÌÓ‚ÂÌË Ë ÚËÔË˜ÌË ÔÓÏÂÌË ‚ Ó·‡ÁÌËÚÂ ËÁÒÎÂ‰‚‡ÌËﬂ Í‡ÚÓ ÍÓÓ‚‡ ‡ÚÓÙËﬂ, ÔÓÏÂÌË ‚ ÔÓ‰ÍÓÓ‚ÓÚÓ ·ﬂÎÓ ÏÓÁ˙˜ÌÓ ‚Â˘ÂÒÚ‚Ó ÔÓ ÚËÔ‡ Ì‡ ‰ÂÏËÂÎËÌËÁ‡ˆËË, ÓÒÌÓ‚ÌÓ ‚˙‚ ÙÓÌÚ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â Ë ÔÂ‰ÌËÚÂ ˜‡ÒÚË Ì‡ ÚÂÏÔÓ‡ÎÌËÚÂ ‰ﬂÎÓ‚Â, Í‡ÎˆËÙËÍ‡ÚË ‚ ·‡Á‡ÎÌËÚÂ „‡Ì„ÎËË. 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