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首页> 外文期刊>Journal, Indian Academy of Clinical Medicine >Adult-onset Rasmussen's encephalitis
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Adult-onset Rasmussen's encephalitis

机译:成人发病的拉斯穆森脑炎

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Rasmussen's encephalitis (RE) is charac terised by refractory focal seizures, unilateral cortical deficits, and progressiveunihemispheric focal cortical atrophy of undetermined aetiology. A majority of these cases present in childhood, and three diseasestages have been recognised: a 'prodromal stage', an 'acute stage' and a 'residual stage'. Adult-onset and variant forms have alsobeen described but are rare, and we report one such case with frequent refractory focal seizures, right-sided hemiparesis, aphasia,and marked atrophy of the left perisylvian cortex with a slow disease progression over 17 years. Immunotherapy withcor ticosteroids, IVIG, plasma exchange, and tacrolimus has been tried in RE with variable results. Hemispherectomy anddisconnective techniques like functional hemispherectomy and hemispherotomy are effective in achieving seizure freedom in62.5% to 85% cases, but carry a risk of motor and language deficits
机译:拉斯穆森脑炎(RE)的特征是难治性局灶性癫痫发作,单侧皮质功能不全和病因尚未明确的进行性单侧半球局灶性皮质萎缩。这些病例大多数出现在儿童时期,并且已经认识到三个疾病阶段:“前驱阶段”,“急性阶段”和“残留阶段”。成人发作和变异形式也已被描述,但很少见,我们报道了一种这样的病例,其伴有难治性局灶性癫痫发作,右侧偏瘫,失语症和左侧肩周皮层明显萎缩,疾病进展缓慢,历时17年。 RE中尝试了使用类固醇,IVIG,血浆置换和他克莫司的免疫治疗,结果不一。半球切除术和功能性半球切除术和半球切开术之类的断开连接技术可有效实现62.5%至85%的癫痫发作自由度,但存在运动和语言缺陷的风险

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