Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press42510.17650/2073-8803-2022-17-4-63-70REVIEWS AND LECTURESОБЗОРЫ И ЛЕКЦИИEpilepsy in mitochondrial diseases. Clinical lectureЭпилепсия при митохондриальных заболеваниях. Клиническая лекцияhttps://orcid.org/0000-0001-6260-6989KotovA. S.КотовА. С.
Russian Federation

Aleksey Sergeevich Kotov

129110

61/2 Shchepkina St.

Moscow

Алексей Сергеевич Котов

129110

ул. Щепкина, 61/2

Москва

alexeykotov1980@gmail.comhttps://orcid.org/0000-0002-3291-7994FirsovK. V.ФирсовК. В.
Russian Federation

129110

61/2 Shchepkina St.

Moscow

129110

ул. Щепкина, 61/2

Москва

M. F. Vladimirskiy Moscow Regional Research Clinical InstituteГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М. Ф. Владимирского»1512202217463701805202318052023Copyright ©; 2022, ABV-PressCopyright ©; 2022, АБВ-пресс2022ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/425

   Mitochondrial dysfunction mainly affects organs with high metabolic demand, primarily the brain. Epilepsy is a common phenotypic sign of both syndromic and non-syndromic mitochondrial diseases. Epilepsy in mitochondrial diseases is poorly treatable and is often an unfavorable prognostic sign. Patients with mitochondrial epilepsy are prescribed various anti-seizure medications. Mitochondriotoxic drugs should be avoided. Descriptions of MELAS-syndrome, MERRF-syndrome, Leigh-syndrome, NARP-syndrome are given.

   При дисфункции митохондрий преимущественно поражаются органы с высокой метаболической потребностью, в первую очередь – головной мозг. Эпилепсия является распространенным фенотипическим признаком как синдромных, так и несиндромных митохондриальных заболеваний. Эпилепсия при митохондриальных заболеваниях плохо поддается лечению и часто является неблагоприятным прогностическим признаком. Пациентам с митохондриальной эпилепсией назначают различные антиэпилептические препараты, однако следует избегать митохондриотоксичных препаратов. Представлены описания синдромов MELAS, MERRF, Leigh и NARP.

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