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成人拉斯穆森脑炎的功能性大脑半球切除术:病例报告及文献综述

Functional Hemispherectomy for Adult Rasmussen Encephalitis: A Case Report and Literature Review.

作者信息

Wang Qun, Zhu Zhanpeng, Wang Guangming, Sun Lichao, Qiu Jiqing

机构信息

Hiser Hospital, Department of Neurology, Qingdao, China.

出版信息

Turk Neurosurg. 2019;29(6):945-949. doi: 10.5137/1019-5149.JTN.21188-17.1.

Abstract

Rasmussen encephalitis (RE) is a rare and severe brain disorder that is associated with unilateral hemispheric atrophy and manifests as severe refractory epilepsy, hemiplegia, defects of motor and speech functions, and cognitive impairment. Treatment of RE, especially in adult patients, is extremely challenging. Herein, we report the case of an adult patient with RE who was treated with a functional hemispherectomy and achieved a favorable prognosis. A 29-year-old woman presented with a 24-year history of epileptic seizures. Neurological examination showed hemiplegia, homonymous hemianopsia, and right muscular atrophy. Neuropsychological examination demonstrated cognitive disorders. Serial magnetic resonance imaging (MRI) and computed tomography (CT) scans showed progressive encephalatrophy in the left hemisphere and ventriculomegaly in the left lateral ventricle. The Wada test showed that the right hemisphere was dominant for language. A functional hemispherectomy was performed. Postoperatively, no antiepileptic drugs were administered, and the patient remained seizure-free without aggravation of hemiplegia. Over a 2-year follow-up, cognitive functions improved. In an adult patient with RE, a favorable prognosis was achieved after functional hemispherectomy. The safety and efficacy of functional hemispherectomy in patients with RE is highlighted.

摘要

拉斯穆森脑炎(RE)是一种罕见且严重的脑部疾病,与单侧半球萎缩相关,表现为严重的难治性癫痫、偏瘫、运动和言语功能缺陷以及认知障碍。RE的治疗,尤其是成年患者的治疗极具挑战性。在此,我们报告一例成年RE患者,其接受了功能性半球切除术并获得了良好的预后。一名29岁女性有24年癫痫发作史。神经系统检查显示偏瘫、同向性偏盲和右侧肌肉萎缩。神经心理学检查显示认知障碍。系列磁共振成像(MRI)和计算机断层扫描(CT)显示左半球进行性脑萎缩和左侧侧脑室脑室扩大。Wada试验显示右侧半球为语言优势半球。进行了功能性半球切除术。术后未给予抗癫痫药物,患者无癫痫发作且偏瘫未加重。经过2年随访,认知功能有所改善。对于成年RE患者,功能性半球切除术后获得了良好的预后。突出了功能性半球切除术在RE患者中的安全性和有效性。

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