Bilateral Rasmussen encephalitis
Bilateral Rasmussen encephalitis
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DOI：
10.1016/j.yebeh.2010.12.004
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发表时间：
2011-02-01
影响因子：
2.6
通讯作者：
Liu, Xingzhou
中科院分区：
文献类型：
--
作者：
Guan, Yuguang;Luan, Guoming;Liu, Xingzhou
拉斯穆森脑炎(RE)是一种罕见的慢性炎症性神经疾病，通常影响一侧大脑。Re的特点是频繁而严重的癫痫发作，进行性神经功能障碍，以及单侧脑萎缩。本文报道一例2岁时双侧受累的RE患儿，其癫痫发作包括局灶性运动性癫痫(右手手指部分性连续癫痫)，右臂和头部强直抽搐伴意识障碍，以及继发性全身性强直阵挛发作。抗癫痫药物不能有效控制癫痫发作。MRI信号异常的左额顶叶活检病理显示典型的神经元丢失，大量淋巴细胞渗入血管周围间隙，并有大量小胶质细胞结节。功能性皮质双极电凝未能改变病程，随后是大剂量强的松(大约相当于2周)，没有改善。功能性半球切除和解剖半球切除均未能阻止病程，术后出现对侧癫痫发作，提示双侧RE。(C)2010 Elsevier Inc.保留所有权利。
Rasmussen encephalitis (RE) is a rare, chronic, inflammatory neurological disorder that usually affects one hemisphere of the brain. RE is characterized by frequent and severe seizures, progressive neurological dysfunction, and unilateral brain atrophy. This article documents a case of RE with bilateral involvement occurring at the age of 2. The patient's seizures consisted of focal motor seizures (epilepsia partialis continua in the fingers on the right hand); tonic convulsions of the right arm and head turning to the right with impairment of consciousness; and secondarily generalized tonic-clonic seizures. Antiepileptic drugs did not effectively control the seizures. The pathology of the left frontoparietal lobe biopsy of the abnormal MRI signal showed typical neuronal loss, abundant lymphocytic infiltration into the perivascular space, and numerous microglial nodules. Bipolar electrocoagulation on functional cortexes, which failed to alter the course of the illness, was followed by high-dose prednisone (about 2 weeks worth), without improvement. Both functional hemispherectomies and anatomical hemispherectomies failed to halt the course of the illness, and contralateral seizures developed and became apparent after the operation, suggesting bilateral RE. (C) 2010 Elsevier Inc. All rights reserved.