References

epilepsia

Epilepsy and paroxysmal conditions

Эпилепсия и пароксизмальные состояния

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epilepsia-42

Research Article

ORIGINAL ARTICLES

ОРИГИНАЛЬНЫЕ СТАТЬИ

GELASTIC SEIZURES

ГЕЛАСТИЧЕСКИЕ ПРИСТУПЫ

Миронов

М. Б.

Mironov

M. B.

Иванова

И. В.

Ivanova

I. V.

Мухин

К. Ю.

Mukhin

K. Yu.

Институт детской неврологии и эпилепсии имени Святителя Луки, МоскваРоссияSvt. Lukas Institute of Child Neurology and Epilepsy, MoscowRussian Federation

2014

02062016

632029

2016

Миронов М.Б., Иванова И.В., Мухин К.Ю.

Mironov M.B., Ivanova I.V., 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/42

Abstract: gelastic seizures are focal epileptic seizures that involve sudden outbursts of forced laughter. In our study Gelastic seizures were revealed in 25 patients that comprised 1,98% of all epilepsy events with onset under 18 years of age (n=1261). Male patients prevailed in the group: 16 male patients (64.0%) against 9 female patients (36.0%). Symptomatic focal epilepsy was identified most frequently - in 80% of patients. Cryptogenic focal epilepsy (probably symptomatic focal epilepsy) was defined in 20% of cases. Epilepsy onset varied in a broad age interval: from the first day after birth till 16 years, 4.53+4.52 years on average. Most often (82%) onset was observed in the first years of life - from the first days of life till 6 years old. Clinical findings may disclose 12 different types of seizures. Most frequently were observed: focal motor seizures with typical automatisms - 64% of cases, secondarily generalized tonic-clonic seizures - 48% of cases and tonic seizures - in 32% of all cases. MRI changes: hypothalamic hamartoma - 32% of cases, temporal lobe atrophy - 12% of cases, DNET - 8% of cases, focal cortical dysplasia (insular-opercular region) - 4%, local bifrontal atrophy - 8%, Tuberous Sclerosis - 8% of cases, diffuse cerebral atrophy presented in the form of ventriculomegalia was diagnosed in 2 patients - 8% of cases. Remission was achieved only in 24% of cases. Whereas, in 12% of cases seizures were blocked only after surgical intervention. Reduction of seizures frequency >50% was observed in 20% of patients. In 56% of cases AED therapy produced no significant effect.

Геластические приступы − фокальные эпилептические приступы, проявляющиеся внезапными эпизодами насильственного смеха. В нашем исследовании геластические приступы были выявлены у 25 пациентов, что составило 1,98% от всех случаев эпилепсии с дебютом приступов до 18 лет (n=1261). Отмечалось преобладание в группе пациентов мужского пола: 16 мужского пола (64,0%) против 9 женского (36,0%). Наиболее часто констатировалась симптоматическая фокальная эпилепсия, выявленная у 80% пациентов. Криптогенная фокальная эпилепсия отмечена в 20% случаев. Дебют эпилепсии варьировал в широком возрастном интервале: от первых суток жизни до 16 лет, в среднем 4,53+4,52 лет. В большинстве случаев (82%) дебют отмечался в первые годы жизни в интервале от первых суток жизни до шести лет. В клинической картине могут регистрироваться 12 различных типов приступов. Наиболее часто выявлялись: фокальные аутомоторные приступы − 64% случаев, вторично-генерализованные судорожные приступы − 48% случаев, тонические приступы констатировались в 32% случаев. Изменения на МРТ: гамартома гипоталамуса − 32% случаев, локальная атрофия височной доли − 12% случаев, ДНЭТ − 8% случаев, фокальная корковая дисплазия оперкулярноинсулярной зоны − 4%, очаговая бифронтальная атрофия − 8%, туберозный склероз − 8% случаев, диффузные атрофические изменения − 8% случаев. Ремиссия была достигнута лишь в 24% случаев. При этом в 12% случаев блокирование приступов стало возможным только после хирургического вмешательства. Снижение частоты приступов на 50% и более наблюдалосьу 20% пациентов. В 56% случаев отсутствовал значимый эффект на фоне АЭП.

геластические приступыприступы смехагамартоматуберозный склерозДНЭТэпилепсияфеномен вторичной билатеральной синхронизации

gelastic seizuresseizures of laughterhamartomatuberous sclerosisDNETepilepsysecondary bilateral synchrony

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