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免疫治疗反应性非副肿瘤性脑炎伴抗谷氨酸脱羧酶、LGI1 和 GABA 受体抗体。

Immunotherapy-responsive Non-paraneoplastic Encephalitis with Antibodies against GAD, LGI1, and GABA Receptor.

机构信息

Division of Neurology, Kobe University Graduate School of Medicine, Japan.

Department of Neurology, Hyogo Rehabilitation Center Hospital, Japan.

出版信息

Intern Med. 2022 Feb 1;61(3):419-423. doi: 10.2169/internalmedicine.7846-21. Epub 2021 Jul 30.

Abstract

A 62-year-old man showed abnormal behavior. Brain magnetic resonance imaging revealed multifocal lesions on T2-weighted images. Initial screening revealed that he was seropositive for antibodies against glutamate decarboxylase, which usually indicates treatment resistance to autoimmune encephalitis (AE). Intensive immunosuppressive therapies, however, improved the neurological symptoms. In line with this, we also detected seropositivity for antibodies against leucine-rich glioma-inactivated 1 and gamma-aminobutyric acid A receptor (GABAR). Brain imaging and treatment responsiveness suggested that antibodies against GABAR were the main cause of symptoms. Furthermore, the patient showed the presence of triple anti-neural antibodies in the absence of malignancy and had a favorable clinical course.

摘要

一位 62 岁男性出现异常行为。脑部磁共振成像显示 T2 加权图像上有多发性病变。初步筛查显示他谷氨酸脱羧酶抗体呈阳性,这通常表明自身免疫性脑炎(AE)对治疗有抗性。然而,强化免疫抑制疗法改善了神经症状。与此一致,我们还检测到针对亮氨酸丰富型胶质瘤失活 1 和γ-氨基丁酸 A 受体(GABAR)的抗体呈阳性。脑部影像学和治疗反应性表明,针对 GABAR 的抗体是症状的主要原因。此外,该患者在无恶性肿瘤的情况下表现出三重抗神经抗体,且具有良好的临床过程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0192/8866773/8ae30d379a7a/1349-7235-61-0419-g001.jpg

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