Department of Neurosurgery, Fu Xing Hospital, Capital Medical University, Beijing, China.
Epilepsy Behav. 2011 Feb;20(2):398-403. doi: 10.1016/j.yebeh.2010.12.004. Epub 2011 Jan 8.
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.
拉森姆森脑炎(RE)是一种罕见的慢性炎症性神经系统疾病,通常影响大脑的一个半球。RE 的特征是频繁和严重的癫痫发作、进行性神经功能障碍和单侧脑萎缩。本文记录了一例 2 岁时发生双侧受累的 RE 病例。患者的癫痫发作包括局灶性运动性发作(右手手指部分持续性癫痫);右臂和头部强直抽搐,向右侧转头,意识障碍;继发全面强直阵挛发作。抗癫痫药物未能有效控制癫痫发作。左侧额顶叶活检异常 MRI 信号的病理学显示典型的神经元丢失、血管周围空间大量淋巴细胞浸润和大量小胶质细胞结节。功能性皮质双极电凝未能改变病情进程,随后给予大剂量泼尼松(约 2 周剂量),但无改善。功能性大脑半球切除术和解剖性大脑半球切除术均未能阻止病情进展,手术后出现对侧癫痫发作且变得明显,提示双侧 RE。