关键词: Aicardi syndrome Corpus callosotomy Epilepsy

Mesh : Aicardi Syndrome / complications Child Corpus Callosum / surgery Drug Resistant Epilepsy / etiology surgery Female Humans Neurosurgical Procedures / methods

来  源:   DOI:10.1016/j.wneu.2020.06.230   PDF(Sci-hub)

Abstract:
Aicardi syndrome is a severe neurodevelopmental disorder that occurs primarily in females and is characterized by seizures, agenesis of the corpus callosum, and chorioretinal lacunae, which occur together in the majority of affected individuals. Seizures begin in infancy and tend to progress in intensity and are often refractory to standard multimodal medication treatments.
We present here a unique case of a 12-year-old girl with partial agenesis of the corpus callosum who underwent a corpus callosotomy for treatment of medically refractory epilepsy. In so doing, we also review the literature with regard to the neurosurgical management of these unique patients.
For the subset of children who present with partial, rather than complete, agenesis of the corpus callosum, corpus callosotomy should be considered as a treatment option to reduce seizure burden.
摘要:
Aicardi综合征是一种严重的神经发育障碍,主要发生在女性,以癫痫发作为特征,call体的发育不全,和脉络膜视网膜腔隙,在大多数受影响的个体中一起发生。癫痫发作始于婴儿期,并倾向于强度发展,并且通常对标准的多模式药物治疗难以治疗。
我们在这里介绍一个独特的案例,一个12岁的女孩,她患有call体部分发育不全,接受了call体切开术治疗药物难治性癫痫。这样做,我们还回顾了有关这些独特患者的神经外科治疗的文献.
对于部分存在的孩子的子集,而不是完整的,call体的发育不全,骨体切开术应被视为减少癫痫发作负担的治疗选择.
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