epilepsiaEpilepsy and paroxysmal conditionsЭпилепсия и пароксизмальные состояния2077-83332311-4088IRBIS LLC10.17749/2077-8333.2017.9.2.074-090epilepsia-348Research ArticleLITERATURE SURVEYОБЗОР ЛИТЕРАТУРЫWEST SYNDROME REVISITEDСОВРЕМЕННЫЕ ПРЕДСТАВЛЕНИЯ О СИНДРОМЕ ВЕСТАОхапкинаТ. Г.OkhapkinaT. G.

младший научный сотрудник отдела психоневрологии и эпилептологии НИКИ педиатрии имени Ю. Е. Вельтищева ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» МЗ РФ

Адрес: ул. Островитянова, д. 1, Москва, Россия, 11799

Junior Researcher, the Department of Psychoneurology and Epileptology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997

stranger.2688@mail.ruГорчхановаЗ. К.GorchanovaZ. K.

к. м.н., врач-невролог отделения видео-ЭЭГ-мониторинга отделения психоневрологии ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» МЗ РФ

Адрес: ул. Островитянова, д. 1, Москва, Россия, 117997

neurologist, the Department of Psychoneurology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997

ШуляковаИ. В.ShulyakovaI. V.

к. м.н., врач-невролог отделения психоневрологии №2 НИКИ педиатрии имени Ю. Е. Вельтищева ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» МЗ РФ

Адрес: ул. Островитянова, д. 1, Москва, Россия, 117997

neurologist, the Department of Psychoneurology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997

irina-shulyakova@mail.ruИльинаЕ. С.IlyinaE. S.

к. м.н., зав. психоневрологическим отделением №2 ФГБУ «РДКБ» МЗ РФ

Адрес: Ленинский пр-т, 117, Москва, Россия, Р 119571

Head, the Department of Psychoneurology, Russian Children’s Clinical Hospital, Health Ministry of Russian Federation

Address: Leninskii pr-t, 117, Moscow, Russia, 119571

pno2.rdkb@gmail.ruМичуринаЕ. С.MichurinaE. S.

врач невролог отделения психоневрологии №2 ФГБУ «РДКБ» МЗ РФ

Адрес: Ленинский пр-т, 117, Москва, Россия, Р 119571

neurologist, the Department of Psychoneurology, Russian Children’s Clinical Hospital, Health Ministry of Russian Federation

Address: Leninskii pr-t, 117, Moscow, Russia, 119571

dr.michurina@gmail.comБелоусоваЕ. Д.BelousovaE. D.

д. м.н., профессор, руководитель отдела психоневрологии и эпилептологии НИКИ педиатрии имени Ю. Е. Вельтищева ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» МЗ РФ

Адрес: ул. Островитянова, д. 1, Москва, Россия, 117997

Head, the Department of Psychoneurology and Epileptology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997

ebelousova@inbox.ruФГБОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияRussian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian FederationRussian FederationФГБУ «Российская детская клиническая больница» Министерства здравоохранения Российской ФедерацииРоссияRussian Children’s Clinical Hospital, Health Ministry of Russian FederationRussian Federation201729082017927490Copyright © Okhapkina T.G., Gorchanova Z.K., Shulyakova I.V., Ilyina E.S., Michurina E.S., Belousova E.D., 20172017Охапкина Т.Г., Горчханова З.К., Шулякова И.В., Ильина Е.С., Мичурина Е.С., Белоусова Е.Д.Okhapkina T.G., Gorchanova Z.K., Shulyakova I.V., Ilyina E.S., Michurina E.S., Belousova E.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.epilepsia.su/jour/article/view/348

West syndrome is an epileptic syndrome, characterized by a triad of symptoms: infantile spasms, hypsarrhythmia on interictal EEG, and a regression or delay of psycho-motoric development. According to the current concept, two of these three criteria suffice for the diagnosis. The incidence of this syndrome is 1 per 2000 newborns. This article reviews recent publications on the etiology, pathogenesis, clinical course, diagnosis, and treatment options in West syndrome. The text is supplemented with illustrations of ictal EEG at infantile spasms and variants of hypsarrhythmia. Today, in addition to classical hypsarrhythmia, five versions of modified hypsarrhythmia are proposed: synchronized version, asymmetric version, version with stable focus of spikes or acute waves, version with rhythmic flattening episodes, version with high-amplitude and asynchronous slow activity. The hormonal therapy is recommended to begin the treatment; if not effective, vigabatrin is to be used as the second choice medication.

Синдром Веста – эпилептический синдром, характеризующийся триадой симптомов: инфантильными спазмами, гипсаритмией на межприступной ЭЭГ, регрессом или задержкой психомоторного развития. Согласно современным представлениям, для постановки диагноза достаточно наличие двух из трех критериев. Частота встречаемости составляет 1 на 2000 новорожденных. Cтатья представляет собой обзор современных представлений об этиологии, патогенезе, клинических проявлениях, диагностике и лечении синдрома Веста. Отдельный раздел статьи иллюстрирует сложности трактовки приступной и межприступной энцефалограммы при инфантильных спазмах. В настоящее время, кроме классической гипсаритмии, описывается пять вариантов модифицированной гипсаритмии: синхронизированный вариант, асимметричный вариант, с устойчивым фокусом спайков или острых волн, с эпизодами уплощения ритмики, с высокоамплитудной асинхронной медленной активностью. Лечение, вне зависимости от этиологии, рекомендовано начинать с гормональной терапии, при отсутствии эффекта препаратом выбора является вигабатрин; обсуждается тактика его применения.

Эпилепсиясиндром Вестаинфантильные спазмыприступная ЭЭГмежприступная ЭЭГмодифицированная гипсаритмиялечение.EpilepsyWest syndromeinfantile spasmsictal EEGinterictal EEGmodified hypsarrhythmiatreatment.ReferencesAuvin S., Cilio M. R., Vezzani A. Current understanding and neurobiology of epileptic encephalopathies. DOI: 10.1016/j.nbd.2016.03.007.Auvin S., Cilio M. R., Vezzani A. Current understanding and neurobiology of epileptic encephalopathies. DOI: 10.1016/j.nbd.2016.03.007.Cайт Международной Лиги по борьбе с эпилепсией. Раздел EpilepsyDiagnosis.org. Подраздел West syndrome. https://www.epilepsydiagnosis.org/seizure/epileptic-spasms- overview.html. Дата обращения: 11.04.2017.International League against Epilepsy (ILAE) official site; EpilepsyDiagnosis.org; West syndrome. https://www.epilepsydiagnosis.org/seizure/epileptic-spasms-overview.html. Accessed: 11.04.2017.Мухин К. Ю., Миронов М. Б. Эпилептические спазмы. Русский журнал детской неврологии. 2014; 9 (4): 20-29. 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The authors declare that there are no conflicts of interest present.