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成人及青少年慢性脑炎与癫痫:拉斯穆森综合征的一种变异型?

Chronic encephalitis and epilepsy in adults and adolescents: a variant of Rasmussen's syndrome?

作者信息

Hart Y M, Andermann F, Fish D R, Dubeau F, Robitaille Y, Rasmussen T, Berkovic S, Marino R, Yakoubian E M, Spillane K, Scaravilli F

机构信息

Montreal Neurologica Hospital and Institute, Quebec, Canada.

出版信息

Neurology. 1997 Feb;48(2):418-24. doi: 10.1212/wnl.48.2.418.

Abstract

Chronic encephalitis and epilepsy (Rasmussen's encephalitis) is a rare progressive disorder of uncertain etiology that usually occurs in children, producing focal epilepsy, hemiparesis, and intellectual deterioration. We identified 13 patients in whom seizures developed in adolescence or adulthood with a pathologic picture of chronic encephalitis. The clinical characteristics were more variable than those occurring in children, with the patients falling into three groups: five patients developed seizures in adulthood, but otherwise showed many resemblances to the childhood form; five developed seizures in adolescence, with similar presentation but rather more benign course than in the younger patients; and three presented with clinical features initially suggestive of a tumor. Occipital onset to the seizures appeared to be more common than in the childhood form, and bilateral disease also occurred.

摘要

慢性脑炎与癫痫(拉斯穆森脑炎)是一种病因不明的罕见进行性疾病,通常发生于儿童,可导致局灶性癫痫、偏瘫和智力衰退。我们确定了13例在青少年期或成年期出现癫痫发作且具有慢性脑炎病理表现的患者。其临床特征比发生于儿童时的情况更具多样性,这些患者分为三组:5例在成年期出现癫痫发作,但在其他方面与儿童型有许多相似之处;5例在青春期出现癫痫发作,表现相似,但病程比年幼患者更为良性;3例最初的临床特征提示为肿瘤。癫痫发作从枕叶开始似乎比儿童型更为常见,并且也会出现双侧病变。

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