perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2020-65-2-92-98perinatology-1114Research ArticleКЛИНИЧЕСКИЕ СЛУЧАИCLINICAL CASESХирургическое лечение эпилепсии у подростка с туберозным склерозомSurgical treatment of epilepsy in a teenager with tuberous sclerosishttps://orcid.org/0000-0003-4468-7660ПолянскаяМ. В.PolyanskayaM. V.

Полянская Майя Владимировна – врач-рентгенолог отделения лучевой диагностики Российской детской клинической больницы, старший лаборант кафедры неврологии, нейрохирургиии медицинской генетики им. акад. Л.О. Бадаляна педиатрического факультета РНИМУ им Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

m.polyan@gmail.comВасильевИ. Г.VasilyevI. G.

Васильев Игорь Германович – врач-нейрохирург отделения нейрохирургии Российской детской клинической больницы РНИМУ им. Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

ЧадаевВ. А.ChadaevV. A.

Чадаев Виктор Алексеевич – к.м.н., врач-невролог отделения нейрохирургии Российской детской клинической больницы РНИМУ им. Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

ДемушкинаА. А.DemushkinaA. A

Демушкина Алиса Анатольевна – к.м.н., врач-рентгенолог отделения лучевой диагностики Российской детской клинической больницы РНИМУ им. Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

ХондкарянГ. Ш.KhondkaryanG. Sh.

Хондкарян Гарегин Шаенович - д.м.н., профессор кафедры неврологии, нейрохирургии и медицинской генетики имени акад. Л.О. Бадаляна педиатрического факультета РНИМУ им. Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

https://orcid.org/0000-0003-0103-7422ЗаваденкоН. Н.ZavadenkoN. N.

Заваденко Николай Николаевич – д.м.н., проф., зав. кафедрой неврологии, нейрохирургии и медицинской генетики им. акад. Л.О. Бадаляна педиатрического факультета РНИМУ им Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

https://orcid.org/0000-0001-8097-7919АлихановА. А.AlikhanovA. A.

Алиханов Алихан Амруллахович – д.м.н., проф., зав. отделением лучевой диагностики Российской детской клинической больницы РНИМУ им. Н.И. Пирогова

119571 Москва, Ленинский пр-т, д. 117

ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииРоссияPirogov Russian National Research Medical UniversityRussian Federation2020140520206529298Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20202020Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/1114

Туберозный склероз – один из генетически детерминированных нейрокожных синдромов, основным клиническим проявлением которого является фокальная эпилепсия, в большом проценте случаев резистентная к медикаментозной терапии. Хирургическая тактика лечения долгое время не рассматривалась применительно к туберозному склерозу в связи с мультифокальностью поражения головного мозга. Однако новые инструментальные методы позволили выделить туберы – «водители», непосредственно ответственные за эпилептическую индукцию, которые, следовательно, могут являться мишенью для хирургического удаления. Представлен клинический случай лечения фармакорезистентной эпилепсии у девушки 17 лет с туберозным склерозом, сохранным психоневрологическим и когнитивным статусом, у которой удалось добиться полного регресса приступов после хирургического удаления одного из туберов. При помощи мультимодального комплекса обследований, включавшего магнитно-резонансную томографию высокого разрешения и инвазивный электроэнцефалографический мониторинг, среди множественных кортикальных туберов удалось выявить тубер-«индуктор», сопряженный с зоной эпилептической инициации. Данный участок коры головного мозга был подвергнут хирургической резекции. За весь период после операции (16 мес) не было отмечено ни одного приступа, прот ивоэпилептические препараты отменены, улучшились когнитивные процессы, память. Таким образом, более чем у 50% пациентов с туберозным склерозом и фармакорезистентной эпилепсией может быть эффективной активная хирургическая тактика. Описанный случай подтверждает необходимость мультидисциплинарного обследования перед хирургической операцией больных с туберозным склерозом.

Tuberous sclerosis is one of the genetically determined neurodermal syndromes, characterized by focal epilepsy, which is resistant to drug therapy in most cases. Tuberous sclerosis has not been surgically treated for a long time due to the multifocal nature of brain damage. However, using new instrumental methods the surgeon can isolate tuber – “drivers”, responsible for epileptic induction, which, therefore, can be a target for surgical removal. The article presents a clinical case of the treatment of pharmacoresistant epilepsy in a 17-year-old girl with tuberous sclerosis and normal neuropsychiatric and cognitive status; the surgeon managed to achieve a complete regression of seizures after the surgical removal of one of the tubers. Using a multimodal complex of examinations, including high-resolution magnetic resonance imaging and invasive electroencephalographic monitoring, the surgeon managed to identify the “inductor” tuber associated with the epileptic initiation zone among multiple cortical tubers. This section of the cerebral cortex was subjected to surgical resection. For the entire postoperative period (16 months) there were no seizures and antiepileptic drugs were canceled; cognitive processes, memory were improved. Thus, active surgical tactics may be effective in more than 50% of patients with tuberous sclerosis and pharmacoresistant epilepsy. This case confirms the need for a multidisciplinary examination prior to the surgery in patients with tuberous sclerosis.

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