epilepsiaEpilepsy and paroxysmal conditionsЭпилепсия и пароксизмальные состояния2077-83332311-4088IRBIS LLC10.17749/2077-8333/epi.par.con.2022.124epilepsia-823Research ArticleSCIENTIFIC SURVEYSНАУЧНЫЕ ОБЗОРЫDiagnostics and age-related evolution of Lennox–Gastaut syndrome. Management in diverse patient age periodsДиагностика и возрастная эволюция синдрома Леннокса–Гасто. Ведение пациентов в разных возрастных периодахБелоусоваЕ. Д.BelousovaE. D.

Белоусова Елена Дмитриевна – д.м.н., профессор, заведующая отделом психоневрологии и эпилептологии Научно-исследовательского клинического института педиатрии им. академика Ю.Е. Вельтищева

РИНЦ SPIN-код: 6594-3049

Elena D. Belousova – Dr. Med. Sc., Professor, Head of Department of Neuropsychiatry and Epileptology, Veltishchev ResearchClinical Institute of Pediatrics

RSCI SPIN-code: 6594-3049

1 Ostrovityanov Str., Moscow 117513

edbelous56@gmail.comhttps://orcid.org/0000-0003-0804-7076БурдС. Г.BurdS. G.

Бурд Сергей Георгиевич – д.м.н., профессор кафедры неврологии, нейрохирургии и медицинской генетики лечебного факультета ; руководитель отдела пароксизмальных заболеваний  

РИНЦ SPIN-код: 1484-0178

ул. Островитянова, д. 1, Москва 117513; ул. Островитянова, д. 1, стр. 10, Москва 117997

Sergey G. Burd – Dr. Med. Sc., Professor, Chair of Neurology, Neurosurgery and Medical Genetics, Faculty of Medicine; Head of Department of Paroxysmal Diseases, Federal Center of Brain andNeurotechnologies

 RSCI SPIN-code: 1484-0178

1 Ostrovityanov Str., Moscow 117513; 1 bldg 10 Ostrovityanov Str., Moscow 117997

https://orcid.org/0000-0001-7197-6009ЕрмоленкоН. А.ErmolenkoN. A.

Ермоленко Наталия Александровна – д.м.н., заведующая кафедрой неврологии 

РИНЦ SPIN-код: 8604-1145

ул. Студенческая, д. 10, Воронеж 394036

Natalia A. Ermolenko – Dr. Med. Sc., Chief of Chair of Neurology

RSCI SPIN-code: 8604-1145

10 Studencheskaya Str., Voronezh 394036

https://orcid.org/0000-0001-8855-7740МухинК. Ю.MukhinK. Yu.

Мухин Константин Юрьевич – д.м.н., профессор, руководитель клиники

ул. Академика Анохина, д. 9, Москва 119571

Konstantin Yu. Mukhin – Dr. Med. Sc., Professor, Head of Clinic

9 Academician Anokhin Str., Moscow 119571

Федеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияPirogov Russian National Research Medical UniversityRussian FederationФедеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Министерства здравоохранения Российской Федерации; Федеральное государственное бюджетное учреждение «Федеральный центр мозга и нейротехнологий» Федерального медико-биологического агентства РоссииРоссияPirogov Russian National Research Medical University; Federal Center of Brain and Neurotechnologies, Federal Medical and Biological Agency of RussiaRussian FederationФедеральное государственное бюджетное образовательное учреждение высшего образования «Воронежский государственный медицинский университет им. Н.Н. Бурденко» Министерства здравоохранения Российской ФедерацииРоссияBurdenko Voronezh State Medical UniversityRussian FederationОбщество с ограниченной ответственностью «Институт детской неврологии и эпилепсии им. Святителя Луки»РоссияSt. Luke Institute of Pediatric Neurology and EpilepsyRussian Federation202216102022143276293Copyright © Belousova E.D., Burd S.G., Ermolenko N.A., Mukhin K.Y., 20222022Белоусова Е.Д., Бурд С.Г., Ермоленко Н.А., Мухин К.Ю.Belousova E.D., Burd S.G., Ermolenko N.A., Mukhin K.Y.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/823

Lennox–Gastaut syndrome is an epileptic encephalopathy with onset in childhood. The classical triad of diagnostic criteria is well known: 1) presence of various types of refractory epileptic seizures (tonic, atypical absences, myoclonic, tonic/atonic drop attacks, generalized tonic-clonic, focal); 2) cognitive disorders with frequent behavioral disorders (not always evident by the beginning of epileptic seizures); 3) generalized, slow (≤2.5 Hz) spike-wave activity of wakefulness and generalized paroxysmal fast activity on sleep electroencephalogram. Despite the seizure onset usually occurring before the age of 8 (peak at 3–5) years old, the Lennox–Gastaut syndrome is often featured with a lifelong course. Many patients with this syndrome suffer from refractory epilepsy in adulthood, however, not always being provided a proper syndromological diagnosis. Expanding the criteria to diagnose the Lennox–Gastaut syndrome discussed here would allow to choose a proper treatment algorithm. Rufinamide is the drug of the second choice in the adjunctive therapy of epileptic seizures associated with Lennox–Gastaut syndrome. However, a pediatric-to-adult clinic transition of patients with Lennox–Gastaut syndrome may pose some obstacles. Herein, an effective patient management requires not only seizure control, but also improvement of patient's quality of life by influencing cognitive and behavioral issues, sleep disorders, disability (both physical and social), educational problems and employment.

Синдром Леннокса–Гасто является эпилептической энцефалопатией с началом в детском возрасте. Хорошо известна классическая триада критериев диагноза: 1) наличие разнообразных типов рефрактерных эпилептических приступов (тонических, атипичных абсансов, миоклонических, тонических/атонических приступов падений, генерализованных тонико-клонических, фокальных); 2) когнитивные нарушения и часто поведенческие нарушения (не всегда очевидные к началу эпилептических приступов); 3) генерализованная медленная (≤2,5 Гц) спайк-волновая активность и генерализованная пароксизмальная быстрая активность сна на межприступной электроэнцефалограмме. Несмотря на обычное начало приступов в возрасте до 8 лет (пик в 3–5 лет), синдром часто имеет пожизненное течение. Многие пациенты с синдромом Леннокса–Гасто во взрослом возрасте страдают рефрактерной эпилепсией, тем не менее адекватный синдромологический диагноз им ставится далеко не всегда. Расширение критериев диагноза синдрома, обсуждаемых в статье, позволит выбрать правильный алгоритм лечения. Препаратом второй очереди выбора в дополнительной терапии эпилептических приступов, ассоциированных с синдромом Леннокса–Гасто, является руфинамид. Существует проблема передачи пациентов с синдромом из детской клиники во взрослую. Эффективное ведение больного требует не только контроля над приступами, но и улучшения качества жизни пациента за счет воздействия на его когнитивные и поведенческие проблемы, нарушения сна, инвалидность (как физическую, так и социальную), образовательные проблемы и трудоустройство.

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