Focal encephalopathy with recurrent episodes of epileptic status and cluster mimicking hemiconvulsion-hemiplegia-epilepsy syndrome

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• 作者：Rie Miyata^a ; Naoyuki Tanuma^b ; Masaharu Hayashi^b ; ^{hayashi-ms@igakuken.or.jp} ; Yukitoshi Takahashi^c
• 关键词：Status epilepticus ; Cluster ; Hemiconvulsion&ndash ; hemiplegia&ndash ; epilepsy syndrome ; Immunohistochemistry
• 刊名：Brain and Development
• 出版年：2012
• 期刊代码：40_03877604
• 类别：med
• 出版时间：May, 2012
• 卷：34
• 期：5
• 页码：360-363
• 文件大小：435 K

摘要

Hemiconvulsion-hemiplegia-epilepsy syndrome is characterized by unilateral convulsions during fever, transient hemiplegia, and subsequent partial epilepsy with atrophy in the cerebrum. A 9-year-old boy with a history of West syndrome and hypoglycemic attacks had three episodes of epileptic status and clusters mimicking HHE syndrome over a 2-year period. Magnetic resonance imaging revealed the involvement of the right and left cerebrums. Because no abnormalities were detected in an endocrine examination, screening tests for metabolic errors, or magnetic resonance spectroscopy, a diagnosis of metabolic errors was not supported. Immunohistochemistry using the patient鈥檚 sera showed binding of the serum immunoglobulin with neurons in the temporal and occipital cerebral cortices, indicating the possible involvement of autoimmune mechanisms in this case. Focal encephalopathy should be considered in children showing convulsions, psychiatric disorders, and/or involuntary movements for several months in a row. In such cases, immunohistochemistry using the patient鈥檚 sera may be useful for the investigation of the pathogenesis of the illness.