Herman Levente, Zsigmond Ildiko Reka, Peter Laszlo, Rethelyi Janos M
Semmelweis University, Department of Psychiatry and Psychotherapy, Budapest, Hungary.
Neuropsychopharmacol Hung. 2016 Dec;18(4):199-208.
Anti-NMDAR (N-methyl-D-aspartic acid receptor) encephalitis, first described in 2007, is a rare, autoimmune limbic encephalitis. In half of the cases anti-NMDAR antibodies are paraneoplastic manifestations of an underlying tumor (mostly ovarian teratoma). In the early stage of the disease psychiatric symptoms are prominent, therefore 60-70% of the patients are first treated in a psychiatric department. In most of the cases, typical neurological symptoms appear later. Besides the clinical picture and typical symptoms, verifying presence of IgG antibodies in the serum or CSF is necessary to set up the diagnosis. Other diagnostic tools, including laboratory tests, MRI, lumbar puncture or EEG are neither specific, nor sensitive enough. Therapy is based on supportive care, plasma exchange and immune suppression, intensive care administration can be necessary. If there is an underlying tumor, tumor removal is the first-line treatment. The disease can cause fatal complications in the acute phase but with adequate therapy long-term prognosis is good, although rehabilitation can last for months. In the past few years besides the typical clinical picture and illness course an increasing number of case reports described no typical neurological symptoms, only psychiatric symptoms, including psychosis, disorganized behavior, and catatonic symptoms. Immune suppressive treatment was still effective in most of these cases. Such cases present a difficult diagnostic challenge. These patients may receive unnecessary antipsychotic treatment because of the suspected schizophrenia, although they often suffer from serious extrapyramidal side effects. A few years ago there was a hypothesis that a small part of the patients who are treated with therapy-resistant schizophrenia may suffer from anti-NMDAR encephalitis, so they require a different kind of medication. Evidence from the latest publications did not confirm this hypothesis, although the connection between anti-NMDAR antibodies and disorders with psychotic symptoms is still not clear. After reviewing the most important studies regarding the psychiatric aspects of anti-NMDAR encephalitis, we present a case report of a patient with a pure psychiatric manifestation of this disease. We conclude with a short outline of the design and plan of our future study.
抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎于 2007 年首次被描述，是一种罕见的自身免疫性边缘叶脑炎。在半数病例中，抗 NMDAR 抗体是潜在肿瘤（大多为卵巢畸胎瘤）的副肿瘤表现。在疾病早期，精神症状较为突出，因此 60%至 70%的患者最初在精神科接受治疗。在大多数病例中，典型的神经症状随后出现。除了临床表现和典型症状外，血清或脑脊液中 IgG 抗体的检测对于确诊至关重要。其他诊断工具，包括实验室检查、磁共振成像（MRI）、腰椎穿刺或脑电图，既不具有特异性，也不够敏感。治疗基于支持治疗、血浆置换和免疫抑制，可能需要重症监护。如果存在潜在肿瘤，肿瘤切除是一线治疗方法。该疾病在急性期可导致致命并发症，但经过适当治疗，长期预后良好，尽管康复可能持续数月。在过去几年中，除了典型的临床表现和病程外，越来越多的病例报告描述了没有典型神经症状，仅有精神症状，包括精神病、行为紊乱和紧张症症状。免疫抑制治疗在大多数此类病例中仍然有效。这些病例带来了诊断难题。由于怀疑患有精神分裂症，这些患者可能会接受不必要的抗精神病药物治疗，尽管他们经常遭受严重的锥体外系副作用。几年前有一个假说，即一小部分接受难治性精神分裂症治疗的患者可能患有抗 NMDAR 脑炎，因此他们需要不同类型的药物治疗。尽管抗 NMDAR 抗体与伴有精神病性症状的疾病之间的联系仍不清楚，但最新出版物的证据并未证实这一假说。在回顾了关于抗 NMDAR 脑炎精神方面的最重要研究后，我们报告了一例该疾病仅有精神症状表现的患者。我们最后简要概述了我们未来研究的设计和计划。
