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成人拉斯穆森慢性脑炎

Rasmussen's chronic encephalitis in adults.

作者信息

McLachlan R S, Girvin J P, Blume W T, Reichman H

机构信息

Department of Clinical Neurological Sciences, University of Western Ontario, London.

出版信息

Arch Neurol. 1993 Mar;50(3):269-74. doi: 10.1001/archneur.1993.00540030035011.

DOI:10.1001/archneur.1993.00540030035011
PMID:8382920
Abstract

OBJECTIVE

Rasmussen's chronic encephalitis, a cause of intractable epilepsy in childhood, is described in three adults.

SETTING

Inpatient epilepsy unit.

PATIENTS

Of 11 patients with pathological confirmation of Rasmussen's encephalitis, three were adults with intractable seizures, progressive sensorimotor deficits, and cognitive decline beginning at the ages of 36, 24, and 16 years.

RESULTS

Clinical, electroencephalographic, and magnetic resonance imaging findings indicated patchy, multifocal involvement of primarily one hemisphere, but the adults had more evidence of disease in the opposite hemisphere than occurs in children. The sensorimotor deficit that the adults developed was greater and the cognitive decline was less than in children. Seizure control following multilobe resection was proportionate to the amount of tissue removed. Cytomegalovirus genome was found in the resected cortical tissue of all three patients.

CONCLUSIONS

Rasmussen's encephalitis is a cause of intractable epilepsy with progressive neurological deficit in adults as well as children. Cytomegalovirus may be involved in the pathogenesis of the disease.

摘要

目的

报告3例成人患有的拉斯穆森慢性脑炎,这是儿童期顽固性癫痫的一个病因。

背景

住院癫痫科。

患者

在11例经病理证实为拉斯穆森脑炎的患者中,3例为成人,分别在36岁、24岁和16岁时开始出现顽固性癫痫发作、进行性感觉运动功能障碍和认知功能下降。

结果

临床、脑电图和磁共振成像结果表明主要为单侧半球的斑片状、多灶性受累,但成人对侧半球的病变证据比儿童更多。成人出现的感觉运动功能障碍更严重,认知功能下降程度小于儿童。多叶切除术后癫痫控制情况与切除组织量成正比。在所有3例患者的切除皮质组织中均发现巨细胞病毒基因组。

结论

拉斯穆森脑炎是成人和儿童顽固性癫痫伴进行性神经功能缺损的一个病因。巨细胞病毒可能参与了该疾病的发病机制。

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