Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press31310.17650/2073-8803-2019-14-4-8-16ORIGINAL REPORTSОРИГИНАЛЬНЫЕ СТАТЬИOutcomes of West syndrome: literature review and own dataИсходы синдрома Веста: обзор литературы и собственные данныеhttps://orcid.org/0000-0001-8009-6177PrygunovaT. M.ПрыгуноваТ. М.
Russian Federation

Neurological Department for Children with Disorders of the Central Nervous System and Mental Disorders, 211 Vaneeva St., Nizhniy Novgorod 603136, Russia

Татьяна Михайловна Прыгунова, Неврологическое отделение для детей с поражением центральной нервной системы и нарушением психики, Россия, 603136 Нижний Новгород, ул. Ванеева, 211

p-tanchita@yandex.ruNizhniy Novgorod Regional Children’s Clinical HospitalГБУЗ НО «Нижегородская областная детская клиническая больница»151220191448161605202016052020Copyright ©; 2019, ABV-PressCopyright ©; 2019, АБВ-пресс2019ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/313

The article is devoted to the treatment and prediction of outcomes of West syndrome. The analysis of literary and own data was carried out. Was estimated the effectiveness of treatment and predicted the outcomes of the disease by data of anamnesis, seizures and the possibilities of therapy. The article presents a stepwise scheme for the treatment of West syndrome and evaluates the results of the application of various drugs, including valproic acid, vigabatrin*, levetiracetam, phenobarbital, clonazepam and topiramate. Special attention is paid to the role of hormone therapy, demonstrated its high efficiency. The most significant prognostic criteria for the outcome of this disease are identified. These criteria are based on literature and our own data. Predictors of an adverse outcome of West syndrome include: symptomatic forms of disease (structural, genetic, infectious), impaired neuropsychological development before onset of epileptic spasms, age of spasms onset more than 8 months, the presence of other seizures types in addition to epileptic spasms, the need for polytherapy of antiepileptic drugs. The pathology of pregnancy, the period of adaptation, neonatal seizures, starting therapy and the response to it are not always important in predicting remission.

Статья посвящена лечению и прогнозированию исходов синдрома Веста. На основании анализа данных литературы и собственных данных выполнены оценка эффективности лечения и попытка прогнозирования исходов заболевания с учетом анамнеза, семиологии приступов и возможностей терапии. Изложена ступенчатая схема лечения синдрома Веста и оценены результаты применения различных препаратов, включая вальпроат, вигабатрин*, леветирацетам, фенобарбитал, клоназепам и топирамат. Отдельное внимание уделено роли гормонотерапии, продемонстрирована ее высокая эффективность. Выделены наиболее значимые прогностические критерии исхода данной патологии. На основании данных литературы и собственных данных установлено, что предикторами неблагоприятного исхода синдрома Веста являются симптоматические формы заболевания (структурная, генетическая, инфекционная), признаки нарушения нервно-психического развития до дебюта спазмов, возраст дебюта спазмов >8 мес, наличие других приступов кроме эпилептических спазмов, необходимость политерапии антиэпилептическими препаратами. Патология беременности, период адаптации, неонатальные судороги, стартовая терапия и ответ на нее не всегда имеют значение при прогнозировании ремиссии.

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