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抗γ-氨基丁酸(GABA)受体抗体阳性脑炎合并广泛多灶性脑病变的成功联合免疫治疗

[Successful combination immunotherapy of anti-gamma aminobutyric acid (GABA) receptor antibody-positive encephalitis with extensive multifocal brain lesions].

作者信息

Fukami Yuki, Okada Hiroaki, Yoshida Mari, Yamaguchi Keiji

机构信息

Department of Neurology, Ichinomiya-Nishi Hospital.

Institute for Medical Science of Aging, Aichi Medical University.

出版信息

Rinsho Shinkeigaku. 2017 Aug 31;57(8):436-440. doi: 10.5692/clinicalneurol.cn-001038. Epub 2017 Jul 22.

Abstract

A 78-year old woman who presented with akinetic mutism was admitted to our hospital. Brain MRI showed multifocal increased T/FLAIR signal with extensive cortical-subcortical involvement. We suspected autoimmune encephalitis and the patient received methylprednisolone pulse. Her conscious level gradually recovered, but later relapsed again and presented with refractory status epilepticus. We treated her with intravenous immunoglobulin, plasma exchange and pulsed cyclophosphamide, with satisfactory response. A brain biopsy showed perivascular lymphocytic infiltrates and reactive gliosis. Anti-gamma aminobutyric acid (GABA) receptor antibodies test came back to be positive after her recovery, and the diagnosis of anti-GABA receptor antibody-positive encephalitis was made. This is a very rare case where brain biopsies were performed in a patient with anti-GABA receptor antibody-positive encephalitis.

摘要

一名表现为运动不能性缄默症的78岁女性被收治入院。脑部磁共振成像(MRI)显示多灶性T/液体衰减反转恢复序列(FLAIR)信号增强,广泛累及皮质-皮质下区域。我们怀疑是自身免疫性脑炎,患者接受了甲泼尼龙冲击治疗。她的意识水平逐渐恢复,但后来再次复发并出现难治性癫痫持续状态。我们用静脉注射免疫球蛋白、血浆置换和脉冲式环磷酰胺对她进行治疗,反应良好。脑活检显示血管周围淋巴细胞浸润和反应性胶质增生。患者康复后,抗γ-氨基丁酸(GABA)受体抗体检测呈阳性,确诊为抗GABA受体抗体阳性脑炎。这是一例非常罕见的在抗GABA受体抗体阳性脑炎患者中进行脑活检的病例。

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