epilepsiaEpilepsy and paroxysmal conditionsЭпилепсия и пароксизмальные состояния2077-83332311-4088IRBIS LLCepilepsia-232Research ArticleSCIENTIFIC SURVEYSНАУЧНЫЕ ОБЗОРЫTREATMENT OF EPILEPSY IN TUBEROUS SCLEROSISЛЕЧЕНИЕ ЭПИЛЕПСИИ ПРИ ТУБЕРОЗНОМ СКЛЕРОЗЕБелоусоваЕ. Д.BelousovaE. D.

д.м.н., профессор, Заслуженный врач Российской Федерации, заведующая отделом психоневрологии и эпилептологии Научно-исследовательского клинического института педиатрии,

ул. Островитянова, д. 1, Москва, 117997

MD, Professor, Honored Doctor of the Russian Federation. Department psychoneurology Epileptology, Research Clinical Institute,

ul. Ostrovityanova, 1, Moscow, 117997

ebelousova@inbox.ruДорофееваМ. Ю.DorofeevaM. Y.

к.м.н., ведущий научный сотрудник отдела психоневрологии и эпилептологии Научно-исследовательского клинического института педиатрии педиатрии им. Ю. Е. Вельтищева,

ул. Островитянова, д. 1, Москва, 117997

PhD, a leading researcher at the Department psychoneurology and Epileptology, Research Clinical Institute,

ul. Ostrovityanova, 1, Moscow, 117997

mdorofeeva@inbox.ruОхапкинаТ. Г.OhapkinaT. G.

врач-ординатор кафедры неврологии, нейрохирургии и медицинской генетики Педиатрического факультета,

ул. Островитянова, д. 1, Москва, 117997

doctor-intern of the Department of Neurology, Neurosurgery and Medical Genetics, Faculty of Pediatrics,

ul. Ostrovityanova, 1, Moscow, 117997

stranger.2688@mail.ruГБОУ ВПО «РНИМУ им. Н.Н. Пирогова» Минздрава РоссииРоссияThe Russian National Research Medical University named after N. I. Pirogov of the Ministry of Health of the Russian FederationRussian Federation201631082016823742Copyright © Belousova E.D., Dorofeeva M.Y., Ohapkina T.G., 20162016Белоусова Е.Д., Дорофеева М.Ю., Охапкина Т.Г.Belousova E.D., Dorofeeva M.Y., Ohapkina T.G.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/232

Epilepsy is the most frequent neurological sign of tuberous sclerosis complex (TSC) and it’s registered in 83.5% of patients. In majority of cases epilepsy begins during the first year of life (63%) and in 38.8% it’s manifesting as infantile spasms. The presence of epilepsy is closely connected with development of mental retardation in TSC. Early treatment of epilepsy is a key to prevention of mental retardation, and preventive treatment with vigabatrin is now advised by some specialists. Possibly high efficasy of vigabatrin in treatment of seizures associated with TSC is partly due to its m-TOR inhibiting property. M-TOR way is widely discussed now as one of the perspective targets for epilepsy treatment, associated or not associated with TSC.

Эпилепсия — наиболее частое неврологическое проявление туберозного склероза и, по данным Международного регистра пациентов с туберозным склерозом, встречается в 83,5% всех случаев. У 63% пациентов приступы начинаются на первом году жизни и в 38,8% всех случаев протекают как инфантильные спазмы. Эпилепсия с ранним началом негативно сказывается на когнитивных функциях и поведении пациентов. Существует сложная причинно-следственная взаимосвязь между туберозным склерозом, эпилепсией, лечением эпилепсии и прогнозом психического развития. Чем короче интервал между началом приступов и началом лечения, тем меньше риск развития эпилептической энцефалопатии. Вероятно, что профилактическое лечение вигабатрином может пре- дотвратить и развитие эпилепсии, и ее негативное влияние на интеллектуальные функции и поведение. Накапливаются данные о том, что путь m-TOR играет важную роль в эпилептогенезе, который запускается самыми разными факторами. Активация m-TOR — одна из перспективных мишеней для воздействия лекарственных средств при фармакорезистетной эпилепсии.

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The authors declare that there are no conflicts of interest present.