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GAD65抗体边缘性脑炎的九年随访:尽管影像学和血清学持续异常,但临床已缓解

Nine-Year Follow-Up of GAD65 Antibody Limbic Encephalitis With Clinical Remission Despite Persistent Imaging and Serological Abnormalities.

作者信息

Aung Thanda, Plotkin Benjamin E

机构信息

Rheumatology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, USA.

Radiology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, USA.

出版信息

Cureus. 2025 Jun 10;17(6):e85726. doi: 10.7759/cureus.85726. eCollection 2025 Jun.

DOI:10.7759/cureus.85726
PMID:40642695
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12245210/
Abstract

Glutamic acid decarboxylase 65 (GAD65) antibody-associated autoimmune encephalitis is a rare neurological disorder characterized by cognitive impairment, seizures, and psychiatric manifestations. Long-term outcomes and management strategies for this condition remain poorly documented in the medical literature. We present a nine-year follow-up of a young woman with GAD65 antibody-positive autoimmune limbic encephalitis who achieved complete clinical remission following aggressive immunomodulatory therapy. The patient, initially treated with rituximab, intravenous immunoglobulin (IVIG), and mycophenolate, successfully discontinued all immunosuppressive medications except hydroxychloroquine. She remains clinically asymptomatic with no recurrence of olfactory hallucinations or other neurological deficits, despite persistently elevated serum GAD65 antibody levels and persistent signal abnormalities on brain imaging. This case highlights the potential for favorable long-term outcomes in GAD65 antibody-associated encephalitis with appropriate immunotherapy and provides insights into maintenance therapy and medication withdrawal strategies for patients planning pregnancy.

摘要

谷氨酸脱羧酶65(GAD65)抗体相关的自身免疫性脑炎是一种罕见的神经系统疾病,其特征为认知障碍、癫痫发作和精神症状。关于这种疾病的长期预后和管理策略在医学文献中仍记载不足。我们报告了一名患有GAD65抗体阳性自身免疫性边缘叶脑炎的年轻女性的九年随访情况,该患者在积极的免疫调节治疗后实现了完全临床缓解。该患者最初接受了利妥昔单抗、静脉注射免疫球蛋白(IVIG)和霉酚酸酯治疗,除羟氯喹外成功停用了所有免疫抑制药物。尽管血清GAD65抗体水平持续升高且脑成像显示持续的信号异常,但她仍无临床症状,嗅觉幻觉或其他神经功能缺损也未复发。该病例突出了通过适当免疫治疗,GAD65抗体相关脑炎获得良好长期预后的可能性,并为计划怀孕的患者的维持治疗和停药策略提供了见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/2c6dfd794c86/cureus-0017-00000085726-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/48975f3c9477/cureus-0017-00000085726-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/9faabbd57697/cureus-0017-00000085726-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/2c6dfd794c86/cureus-0017-00000085726-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/48975f3c9477/cureus-0017-00000085726-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/9faabbd57697/cureus-0017-00000085726-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c0d/12245210/2c6dfd794c86/cureus-0017-00000085726-i03.jpg

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本文引用的文献

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Meningoencephalitis associated with GAD65 autoimmunity.伴谷氨酸脱羧酶 65 自身免疫性脑炎的脑膜脑炎。
Front Immunol. 2023 Mar 9;14:1120894. doi: 10.3389/fimmu.2023.1120894. eCollection 2023.
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Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response.谷氨酸脱羧酶抗体相关神经综合征:临床、抗体特征及治疗反应
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与谷氨酸脱羧酶65抗体相关的神经系统疾病:来自中国的队列研究的临床谱和预后
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Clinical characteristics of GAD 65-associated autoimmune encephalitis.GAD65 相关性自身免疫性脑炎的临床特征。
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