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[关于拉斯穆森脑炎的最新见解]

[Recent insights into Rasmussen encephalitis].

作者信息

Bien C G, Elger C E

机构信息

Klinik für Epileptologie, Universität Bonn.

出版信息

Nervenarzt. 2005 Dec;76(12):1470, 1472-4, 1477-80, 1484-7. doi: 10.1007/s00115-005-1955-0.

Abstract

Rasmussen encephalitis (RE) is a severe and at the same time pathophysiologically fascinating condition. The chronic inflammation affects one of the two cerebral hemispheres and destroys it during the disease process that lasts from months to years. The patients -- mostly children -- suffer from frequent pharmacoresistant seizures, often in the form of epilepsia partialis continua. In parallel to the atrophy of the affected hemisphere, the neurological functions associated with it decline continuously. This results in a final stage with a usually high-grade sensorimotor hemisyndrome, hemianopia, cognitive impairment and -- if the language-dominant hemisphere is affected -- aphasia. Research results in the last 5 years have contributed to a deeper understanding of the pathogenesis of this condition. Formal diagnostic criteria have been proposed, and new therapeutic options have emerged by which the disease progression can be slowed or stopped. This article summarizes the current research results on the background of older data and gives recommendations regarding diagnostic and therapeutic procedures in RE patients.

摘要

拉斯穆森脑炎(RE)是一种严重且在病理生理学方面引人入胜的病症。慢性炎症影响双侧大脑半球中的一个,并在持续数月至数年的疾病过程中将其破坏。患者——大多为儿童——频繁出现药物难治性癫痫发作,通常表现为持续性部分性癫痫。与受影响半球萎缩并行的是,与之相关的神经功能持续衰退。这导致最终阶段通常出现高级别感觉运动性偏侧综合征、偏盲、认知障碍,以及——如果语言优势半球受到影响——失语。过去5年的研究成果有助于更深入地理解这种病症的发病机制。已提出了正式的诊断标准,并且出现了新的治疗选择,通过这些选择可以减缓或阻止疾病进展。本文在以往数据的背景下总结了当前的研究成果,并给出了关于RE患者诊断和治疗程序的建议。

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