Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press50810.17650/2073-8803-2025-20-1-17-24REVIEWS AND LECTURESОБЗОРЫ И ЛЕКЦИИDefinition and new approaches to understanding the pathogenesis of Drave syndrome in childrenОпределение и новые подходы к пониманию патогенеза синдрома Драве у детейhttps://orcid.org/0000-0003-3054-7560UlyakovA. N.УляковА. Н.
Russian Federation

Artur N. Ulyakov.

5, 8 Nagornaya St., Troitsk, Moscow 108842; 117 Leninskiy Prospekt, Moscow 119571

Уляков Артур Николаевич.

108842 Москва, Троицк, ул. Нагорная, 5, 8; Россия, 119571 Москва, Ленинский проспект, 117

arturulyakov@mail.ruhttps://orcid.org/0000-0001-6125-0618BobylovaM. Yu.БобыловаМ. Ю.
Russian Federation

5, 8 Nagornaya St., Troitsk, Moscow 108842

108842 Москва, Троицк, ул. Нагорная, 5, 8

https://orcid.org/0000-0002-3764-2766LobanovaV. S.ЛобановаВ. С.
Russian Federation

5, 8 Nagornaya St., Troitsk, Moscow 108842; 1/9 4-yy Dobryninskiy Pereulok, Moscow 119049

108842 Москва, Троицк, ул. Нагорная, 5, 8; 119049 Москва, 4-й Добрынинский переулок, 1/9

https://orcid.org/0000-0001-8855-7740МukhinК. Yu.МухинК. Ю.
Russian Federation

5, 8 Nagornaya St., Troitsk, Moscow 108842

108842 Москва, Троицк, ул. Нагорная, 5, 8

https://orcid.org/0000-0001-9050-2036PylaevaO. A.ПылаеваО. А.
Russian Federation

5, 8 Nagornaya St., Troitsk, Moscow 108842

108842 Москва, Троицк, ул. Нагорная, 5, 8

https://orcid.org/0009-0001-4160-2644TishchenkoI. S.ТищенкоИ. С.
Russian Federation

5, 8 Nagornaya St., Troitsk, Moscow 108842; 1 Ostrovityanova St., Moscow 117513

108842 Москва, Троицк, ул. Нагорная, 5, 8; 117513 Москва, ул. Островитянова, 1

https://orcid.org/0009-0007-3232-6733KazakovS. O.КазаковС. О.
Russian Federation

5, 8 Nagornaya St., Troitsk, Moscow 108842; 1 Ostrovityanova St., Moscow 117513

108842 Москва, Троицк, ул. Нагорная, 5, 8; 117513 Москва, ул. Островитянова, 1

Svt. Luka’s Institute of Child Neurology and EpilepsyООО «Институт детской неврологии и эпилепсии им. Святителя Луки»Russian Children Clinical Hospital – branch of the Pirogov Russian National Research Medical University, Ministry of Health of RussiaРоссийская детская клиническая больница – филиал ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииMorozov Children’s City Clinical Hospital, Moscow Healthcare DepartmentГБУЗ «Морозовская детская городская клиническая больница Департамента здравоохранения г. Москвы»Pirogov Russian National Research Medical University, Ministry of Health of RussiaФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России1804202520117241704202517042025Copyright ©; 2025, ABV-PressCopyright ©; 2025, АБВ-пресс2025ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/508

Nowadays, Dravet syndrome is one of the most studied forms of genetic epilepsy. Despite a large number of studies and publications, Dravet syndrome remains a difficult neurological disease to treat. The relationship of epileptogenesis in Dravet syndrome with the activity of sodium channels has been studied, and data on the different effectiveness and ineffectiveness of anticonvulsant drugs with different mechanisms of action have been obtained. Drugs that modify neural transmission, enhance inhibitory transmission, and reduce excitatory transmission, as well as their combination, are more effective. Drugs that reduce the effect of inhibitory transmission (in particular, sodium channel blockers) should not be used. In our opinion, the correct choice of therapy for Dravet syndrome associated with a mutation in the SCN1A gene is possible only from the perspective of understanding the work of neural channels in the brain.

В наши дни синдром Драве является одной из самых изученных форм генетической эпилепсии. Несмотря на большое количество исследований и публикаций, синдром Драве остается труднокурабельным неврологическим заболеванием. Изучена связь эпилептогенеза при синдроме Драве с активностью натриевых каналов, получены данные о различной эффективности и неэффективности противосудорожных препаратов с разным механизмом действия. Наиболее эффективны препараты, модифицирующие нейронную передачу – усиливающие тормозную и уменьшающие возбуждающую передачу, – а также их комбинация. Не рекомендовано применение препаратов, уменьшающих влияние тормозной передачи (в частности блокаторов натриевых каналов). По нашему мнению, правильный подбор терапии синдрома Драве, ассоциированного с мутацией в гене SCN1A, возможен только с позиции понимания работы нейрональных каналов в головном мозге.

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