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[结节性硬化症患儿癫痫的病程及治疗]

[The course and treatment of epilepsy in children with tuberose sclerosis].

作者信息

Dorofeeva M Iu, Belousova E D

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2012;112(6 Pt 2):27-31.

Abstract

Tuberous sclerosis complex is a multisystem genetic disorder. Epilepsy is very common in tuberous sclerosis and occurs in 75-92% of affected individuals during their life-time. Onset usually occurs during childhood and up to one third of children with tuberous sclerosis will develop infantile spasms. Of all the possible manifestations of this complex disorder the resistant epilepsy, the cognitive and behavioral problems represent the area of greatest concern to parents, caregivers and physicians. Treatment of epilepsy in tuberous sclerosis is similar to epilepsy resulting from other cases and includes anticonvulsant medications, the epilepsy surgery, the vagus nerve stimulation and the ketogenic diet. Vigabatrin has been shown to be particularly effective in treating infantile spasms in the setting of tuberous sclerosis.

摘要

结节性硬化症是一种多系统遗传性疾病。癫痫在结节性硬化症中非常常见,75% - 92%的患者在其一生中会发生癫痫。癫痫通常在儿童期发病,多达三分之一的结节性硬化症患儿会出现婴儿痉挛。在这种复杂疾病的所有可能表现中,难治性癫痫、认知和行为问题是家长、护理人员和医生最为关注的领域。结节性硬化症的癫痫治疗与其他病因导致的癫痫相似,包括抗惊厥药物、癫痫手术、迷走神经刺激和生酮饮食。已证明vigabatrin在治疗结节性硬化症所致婴儿痉挛方面特别有效。

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