Autoimmune Encephalitis With Multiple Autoantibodies: A Diagnostic and Therapeutic Challenge
Autoimmune Encephalitis With Multiple Autoantibodies: A Diagnostic and Therapeutic Challenge
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DOI：
10.1097/nrl.0000000000000171
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发表时间：
2018-03-01
期刊：
影响因子：
1.2
通讯作者：
Day, Gregory S.
中科院分区：
文献类型：
--
作者：
Kim, Albert E.;Kang, Peter;Day, Gregory S.
导读：随着疾病相关自身抗体和临床综合征数量的增加，快速发作性认知障碍患者自身抗体检测的适应症也在扩大。虽然自身抗体检测的增加扩大了我们对自身免疫性脑炎（AE）谱的理解，但它也带来了新的挑战，即在具有非典型临床特征和/或多种自身抗体的患者中，如何解读疾病相关自身抗体的作用。这些挑战是通过介绍AE患者与自身抗体对抗细胞内和细胞表面神经元抗原。在相关文献的背景下考虑多种自身抗体检测的含义，并用于构建诊断和治疗方法。病例报告：一名先前身体健康的67岁男性，表现为脑病和精神病，视力固定障碍和共济失调，出现时间超过3个月。脑脊液中检测到Hu、CRMP-5和NMDAR自身抗体。尽管进行了广泛的检查，但未发现恶性肿瘤。积极的免疫疗法暂时稳定了他的病情；然而，患者在症状出现10个月后死亡。缺乏对免疫治疗的持续反应和神经病理学结果表明，AE与Hu抗体相关是该患者进行性衰退的主要原因。结论：AE患者可检出多种自身抗体。当同时检测到针对细胞内和细胞表面抗原的抗体时，应优先考虑与细胞内抗体相关的综合征的调查和治疗，承认这些抗体与不可逆神经元损伤之间的联系。在副肿瘤病例中，预后可能与早期发现和治疗潜在的恶性肿瘤有关。
Introduction:Indications for autoantibody testing in patients with rapid-onset cognitive impairment have expanded in step with the growing number of disease-associated autoantibodies and clinical syndromes. Although increased access to autoantibody testing has broadened our understanding of the spectrum of autoimmune encephalitis (AE), it has also produced new challenges associated with deciphering the contributions of disease-associated autoantibodies in patients with atypical clinical features and/or multiple autoantibodies. These challenges are illustrated through presentation of a patient with AE associated with autoantibodies against intracellular and cell-surface neuronal antigens. The implications of detection of multiple autoantibodies are considered in the context of relevant literature, and used to frame a diagnostic and therapeutic approach.Case Report:A previously well 67-year-old man presented with encephalopathy and psychosis, impaired visual fixation, and ataxia, emerging over 3 months. Hu, CRMP-5, and NMDAR autoantibodies were identified in the cerebrospinal fluid. No malignancy was discovered despite extensive investigations. An aggressive course of immunotherapy temporarily stabilized his course; however, the patient succumbed to his illness 10 months after symptom onset. Lack of sustained response to immunotherapy and neuropathologic findings suggested that AE associated with Hu antibodies was primarily responsible for this patient's progressive decline.Conclusions:Multiple autoantibodies may be detected in patients with AE. When antibodies targeting intracellular and cell-surface antigens are detected together, investigation and treatment of syndromes associated with intracellular antibodies should be prioritized, acknowledging the link between these antibodies and irreversible neuronal injury. In paraneoplastic cases, prognosis may be tied to early detection and treatment of the underlying malignancy.