Abstract
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.
Highlights
Туберозный склероз – один из генетически детерминированных нейрокожных синдромов, основным клиническим проявлением которого является фокальная эпилепсия, в большом проценте случаев резистентная к медикаментозной терапии
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
Summary
Туберозный склероз – один из генетически детерминированных нейрокожных синдромов, основным клиническим проявлением которого является фокальная эпилепсия, в большом проценте случаев резистентная к медикаментозной терапии. Представляем клинический случай лечения фармакорезистентной эпилепсии у девушки 17 лет с туберозным склерозом, у которой удалось добиться полной ремиссии приступов после хирургического удаления одного из туберов. Всем детям с неврологическими проявлениями туберозного склероза, даже в отсутствие приступов, показаны видео-ЭЭГ-мониторинг и МРТ головного мозга каждые 1–3 года до достижения 20–25 лет с целью динамического наблюдения [16,17].
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