Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press10210.17650/2073-8803-2012-7-4-31-40REVIEWS AND LECTURESОБЗОРЫ И ЛЕКЦИИRESISTANT EPILEPSY FORMS, ASSOCIATED WITH FEVER-PROVOKED (FEBRILE) EPILEPTIC SEIZURES (DRAVET SYNDROME, DESC, HHE) (A LECTURE)РЕЗИСТЕНТНЫЕ ФОРМЫ ЭПИЛЕПСИИ, АССОЦИИРОВАННЫЕ С ФЕБРИЛЬНО- ПРОВОЦИРУЕМЫМИ ПРИСТУПАМИ (СИНДРОМЫ ДРАВЕ, DESC, HHE) (ЛЕКЦИЯ)BobylovaM. Yu.БобыловаМ. Ю.
Russian Federation
Институт, детской неврологии и эпилепсии им. Святителя Луки151220127431401510201515102015Copyright ©; 2012, ABV-PressCopyright ©; 2012, АБВ-пресс2012ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/102

The most important characteristics of resistant epilepsy forms, associated with fever-provoked (febrile) epileptic seizures (Dravet syndrome, DESC — Devastating epileptic encephalopathy in school-aged children, HHE — hemiconvul- sion-hemiplegia-epilepsy syndrome) are proposed. For every syndrome all the main aspects of disease are considered in detail: aetiology and pathogenesis, clinical picture, including of characteristics of seizures, EEG, neuroimaging, therapeu­tic approachers and prognosis.

 

 

Представлены основные характеристики резистентных форм эпилепсии, ассоциированных с фебрилъно-провоцируемыми приступами (синдромы Драве, DESC, HHE). Для каждого из синдромов подробно рассма­триваются все основные аспекты заболевания: этиология и патогенез, клиническая картина, включая ха­рактеристику эпилептических приступов, изменения на ЭЭГ, данные нейровизуализации, терапевтичес­кие подходы и прогноз.

 

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