Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId
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DOI：
10.3389/fneur.2019.01233
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发表时间：
2019-11-20
影响因子：
3.4
通讯作者：
Fujimoto, Ayataka
中科院分区：
文献类型：
--
作者：
Itamura, Shinji;Okanishi, Tohru;Fujimoto, Ayataka
半痉挛-偏瘫-癫痫综合征（HHES）是急性脑病的一个亚型，其特征是婴儿期发病，伴有急性半痉挛性发热状态，随后出现单侧脑萎缩和偏瘫。在慢性期，HHES患者发展为癫痫，典型表现为难治性局灶性癫痫发作。抗癫痫药物治疗往往难以治愈，需要手术治疗。虽然病毒性脑炎和遗传异常被认为是潜在的病因，但发病机制仍不清楚。我们描述了三例成功的功能性半球切开术治疗顽固性癫痫的HHES。患者在17-30个月的病毒感染后出现急性不对称抽搐状态。他们的癫痫发作是顽固性的抗癫痫药物，需要半球切除术。根据病理结果，所有病例均诊断为局灶性皮质发育不良（FCD） IIId型。癫痫引起的轻度皮质畸形可能是HHES的病因。
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHES) is a subset of acute encephalopathy characterized by infantile-onset with acute hemiconvulsive febrile status and subsequent unilateral cerebral atrophy and hemiparesis. In the chronic phase, patients with HHES develop epilepsy, typically displayed as intractable focal seizures. The patients are often intractable with antiepileptic drugs and need surgical treatment. Although viral encephalitis and genetic abnormalities are presumed to be the underlying etiology, the pathogenesis remains mostly unknown. We describe three cases of successful functional hemispherotomy for intractable epilepsy in HHES. Patients developed acute asymmetrical convulsive status following viral infections during the ages of 17-30 months. Their seizures were intractable with antiepileptic drugs and required hemispherotomy. On the basis of the pathological findings, all cases were diagnosed as focal cortical dysplasia (FCD) type IIId. The epileptogenic mild cortical malformations may be the cause of HHES.