neurosurgeryНейрохирургияRussian journal of neurosurgery1683-32952587-7569Издательский дом "МедИНК"10.17650/1683-3295-2024-26-3-112-125neurosurgery-1577Research ArticleНАБЛЮДЕНИЕ ИЗ ПРАКТИКИFROM PRACTICEХирургическое лечение эпилепсии у детей с туберозным склерозом: обзор литературы и клиническое наблюдениеEpilepsy surgery for tuberous sclerosis complex in children: literature review and clinical casehttps://orcid.org/0000-0001-5314-4436БердиновФ. Б.BerdinovF. B.

Фархад Бахрамджанович Бердинов

117198 Москва, ул. Миклухо-Маклая, 6;

119049 Москва, 4-й Добрынинский пер., 1/9

Farkhad Bakhramdzhanovich Berdinov

6 Miklukho-Maklaya St., Moscow 117198;

1/9 4th Dobrynynsky Ln., Moscow 119049

Berdinovns@gmail.comhttps://orcid.org/0000-0002-0344-9765КузнецоваА. А.KuznetsovaA. A.

119049 Москва, 4-й Добрынинский пер., 1/9;

115419 Москва, ул. Донская, 43

1/9 4th Dobrynynsky Ln., Moscow 119049;

43 Donskaya St., Moscow 115419

https://orcid.org/0000-0003-3035-9375ЛевовА. В.LevovA. V.

119049 Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobrynynsky Ln., Moscow 119049

https://orcid.org/0000-0003-0024-4630СоловьевВ. Б.SolovevV. B.

119049 Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobrynynsky Ln., Moscow 119049

https://orcid.org/0000-0003-4743-5522РусскинВ. О.RusskinV. O.

119049 Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobrynynsky Ln., Moscow 119049

https://orcid.org/0000-0002-2302-1205ЩедеркинаИ. О.ShchederkinaI. O.

119049 Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobrynynsky Ln., Moscow 119049

https://orcid.org/0000-0001-7010-0101ЛившицМ. И.LivshitsM. I.

117198 Москва, ул. Миклухо-Маклая, 6;

119049 Москва, 4-й Добрынинский пер., 1/9

6 Miklukho-Maklaya St., Moscow 117198;

1/9 4th Dobrynynsky Ln., Moscow 119049

https://orcid.org/0000-0002-3323-508XЧмутинГ. Е.ChmutinG. E.

117198 Москва, ул. Миклухо-Маклая, 6;

119049 Москва, 4-й Добрынинский пер., 1/9

6 Miklukho-Maklaya St., Moscow 117198;

1/9 4th Dobrynynsky Ln., Moscow 119049

ФГАОУ ВО «Российский университет дружбы народов им. Патриса Лумумбы»; ГБУЗ г. Москвы «Морозовская детская городская клиническая больница Департамента здравоохранения г. Москвы»РоссияPeoples’ Friendship University of Russia n. a. Patrice Lumumba; Morozov Children’s City Clinical Hospital, Moscow Healthcare DepartmentRussian FederationГБУЗ г. Москвы «Морозовская детская городская клиническая больница Департамента здравоохранения г. Москвы»; ГБУЗ г. Москвы «Научно-практический психоневрологический центр им. З.П. Соловьева Департамента здравоохранения г. Москвы»РоссияMorozov Children’s City Clinical Hospital, Moscow Healthcare Department; Z.P. Solovyev Research and Clinical Center for Neuropsychiatry, Moscow Healthcare DepartmentRussian FederationГБУЗ г. Москвы «Морозовская детская городская клиническая больница Департамента здравоохранения г. Москвы»РоссияMorozov Children’s City Clinical Hospital, Moscow Healthcare DepartmentRussian Federation202407092024263112125Copyright © Бердинов Ф.Б., Кузнецова А.А., Левов А.В., Соловьев В.Б., Русскин В.О., Щедеркина И.О., Лившиц М.И., Чмутин Г.Е., 20242024Бердинов Ф.Б., Кузнецова А.А., Левов А.В., Соловьев В.Б., Русскин В.О., Щедеркина И.О., Лившиц М.И., Чмутин Г.Е.Berdinov F.B., Kuznetsova A.A., Levov A.V., Solovev V.B., Russkin V.O., Shchederkina I.O., Livshits M.I., Chmutin G.E.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.therjn.com/jour/article/view/1577

Туберозный склероз (ТС) (tuberous sclerosis complex, TSC) – мультисистемный, аутосомно-доминантный, нейрокожный синдром, для которого характерно формирование доброкачественных новообразований (туберов, гамартом) в различных органах, включая головной мозг. Эпилепсия является наиболее распространенным симптомом у пациентов с ТС, при этом в 62,5 % случаев развивается ее фармакорезистентная форма. Трудность хирургического лечения заключается в том, что для эпилепсии при ТС характерно мультифокальное течение. Несмотря на это, имеющиеся данные свидетельствуют в пользу хирургического вмешательства как наиболее вероятного метода для достижения долговременного разрешения приступов.Цель работы – проанализировать актуальные данные по исследуемой теме, аспекты хирургического лечения эпилепсии у детей с ТС.Поиск публикаций осуществляли на научных платформах PubMed, Google Scholar, eLIBRARY. RU за период с 2000 по 2022 г. с использованием следующих запросов: TSC-associated epilepsy in children, epilepsy surgery in children with TSC, epilepsy surgery for TSC, хирургическое лечение эпилепсии у детей с туберозным склерозом, хирургия эпилепсии при туберозном склерозе.Источником эпилептической активности являются не только туберы, но и мозговая ткань вокруг тубера. В настоящее время имеет место тенденция раннего проведения оперативного лечения: при неэффективной терапии двумя противоэпилептическими препаратами пациенты должны рассматриваться как кандидаты на хирургическое лечение. С учетом множественных поражений головного мозга, мультифокального течения эпилепсии при предхирургическом обследовании пациентов требуется применение инвазивных методов электроэнцефалографии. Эффективность резективной хирургии составляет 65–75 %. Со временем доля пациентов с полной ремиссией приступов уменьшается. Лобэктомия и туберэктомия плюс являются благоприятными прогностическими факторами. Хирургическое лечение значительно повышает шансы пациентов на полную ремиссию эпилептических приступов, в свою очередь прекращение эпилептогенеза развивающегося головного мозга оказывает положительное влияние на когнитивное развитие детей.В настоящий момент нет определенного алгоритма предхирургического обследования пациентов и критериев отбора детей на хирургическое лечение, часть обследований не входит в систему обязательного медицинского страхования, что затрудняет диагностику и своевременное лечение и приводит к повышению доли пациентов с инвалидностью и низким качеством жизни.

Tuberous sclerosis complex (TSC) is a multisystem, autosomal-dominant, neurocutaneous syndrome that is characterized by the presence of hamartomas involving multiple organs, including the brain. Epilepsy is the most common neurological manifestation and the main cause of disability in children. Drug-resistant epilepsy is seen in 62.5 % of cases. The challenge of surgical treatment in these patients is the multifocal nature of epilepsy. Nonetheless, there is available data to suggest that surgical intervention is most likely to achieve long-term seizure freedom.The aim of the work – to analyze current data and aspects of surgical treatment of epilepsy associated with tuberous sclerosis in children.A literature search for was done on PubMed, Google Scholar, and eLIBRARY. RU for the period from 2000 to 2022. Search phrases included: TSC-associated epilepsy in children, epilepsy surgery in children with TSC, epilepsy surgery for TSC. The tubers are not the only source of epileptic activity; the perituberal brain tissue is also a proven focus. Currently, there is a tendency towards early pre-surgical evaluation and surgical treatment, which is recommended after the failure of two antiepileptic drugs. Considering the multiple brain lesions and multifocal epilepsy, the use of invasive electroencephalography is invaluable in the preoperative assessment of these patients. The effectiveness of resection surgery is 65–75 %. Over time, the proportion of patients in complete remission from seizures decreases. Lobectomy and tuberectomy plus procedures are favorable prognostic factors. Surgical treatmentsignificantly increasesthe chances of seizure freedom. Eliminating seizures in children has been shown to improve cognitive development.There is no algorithm for pre-surgical patient evaluation or selection criteria for surgical treatment. Some methods of presurgical evaluation are not included in the compulsory health insurance system, making early diagnosis and treatment very difficult. This leads to an increase in the number of patients with disabilities and a poor quality of life.

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