Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press6810.17650/2073-8803-2013-8-1-25-38REVIEWS AND LECTURESОБЗОРЫ И ЛЕКЦИИMYOCLONIC ASTATIC EPILEPSY (DOOSE SYNDROME)ЭПИЛЕПСИЯ С МИОКЛОНИЧЕСКИ-АСТАТИЧЕСКИМИ ПРИСТУПАМИ (СИНДРОМ ДОЗЕ)MukhinK. Yu.МухинК. Ю.
Russian Federation

Москва

MironovM. B.МироновМ. Б.
Russian Federation

Москва

PylaevaO. A.ПылаеваО. А.
Russian Federation

Москва

Институт детской неврологии и эпилепсии им. Святителя Луки150120138125382306201523062015Copyright ©; 2013, ABV-PressCopyright ©; 2013, АБВ-пресс2013ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/68

The authors proposed a detailed review of literature devoted to myoclonic astatic epilepsy (Doose syndrome). The authors describe the history of study of myoclonic astatic epilepsy, its prevalence, etiology and the role of genetic factors, clinical pictures, including the main type of seizures, diagnostic criteria and nosology. The characteristics of EEG and neuroimaging are prescribed, and also differential diagnosis, approaches to treatment and prognosis.

Представлен подробный обзор литературы и собственных данных, посвященный эпилепсии с миоклонически-астатическими приступами (синдром Дозе). Авторы рассматривают историю изучения заболевания, его распространенность, этиологию и роль генетических факторов, клинические проявления, включая основные типы приступов, диагностические критерии и нозологическая специфичность синдрома. Представлены особенности ЭЭГ и изменения при нейровизуализации. Описаны также подходы к дифференциальному диагнозу, лечение и прогноз заболевания.

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