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一名自身免疫性精神病患者中具有体树突状染色模式的新型抗神经元自身抗体

Novel Antineuronal Autoantibodies With Somatodendritic Staining Pattern in a Patient With Autoimmune Psychosis.

作者信息

Endres Dominique, Rauer Sebastian, Pschibul Alexander, Süß Patrick, Venhoff Nils, Runge Kimon, Feige Bernd, Denzel Dominik, Nickel Kathrin, Schweizer Tina, Maier Simon, Egger Karl, Domschke Katharina, Meyer Philipp T, Prüss Harald, Tebartz van Elst Ludger

机构信息

Section for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Department of Psychiatry and Psychotherapy, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

出版信息

Front Psychiatry. 2020 Aug 6;11:627. doi: 10.3389/fpsyt.2020.00627. eCollection 2020.

Abstract

BACKGROUND

Autoimmune encephalitis, such as anti-NMDA-receptor encephalitis, typically presenting with subacute onset of neuropsychiatric symptoms, can be detected by antineuronal autoantibodies or inflammatory changes in the cerebrospinal fluid (CSF), as well as pathological alterations in electroencephalography (EEG), magnetic resonance imaging (MRI), or [18F]fluorodeoxyglucose positron emission tomography (FDG PET). For patients with predominant psychotic symptoms, the term autoimmune psychosis was proposed. Here, the authors present the case of a patient with probable autoimmune psychosis associated with unknown antineuronal antibodies.

CASE PRESENTATION

A 18-year-old male patient with preexisting autism spectrum disorder developed a severe catatonic syndrome over 2.5 years. The MRI showed normal findings, the EEG depicted intermittent slowing, and the independent component analyses showed additional sharp spikes. However, FDG PET, the basic laboratory analysis and testing of the serum/CSF for well-characterized antineuronal autoantibodies were unsuspicious. The serum and CSF "tissue-based assay" using indirect immunofluorescence on unfixed murine brain tissue revealed antineuronal autoantibodies against an unknown epitope in granule cells in the cerebellum and to neurites of hippocampal interneurons with a somatodendritic staining pattern. The immunosuppressive treatment with high-dose glucocorticoids, plasma exchange, and rituximab led to partial improvement.

CONCLUSION

The patient probably suffered from autoantibody-associated autoimmune psychosis. The special features of the case were that the patient (1) presented with mostly inconspicuous basic diagnostics, except for the altered EEG in combination with the detection of CSF autoantibodies directed against a currently unknown epitope, (2) experienced an isolated and long-lasting psychotic course, and (3) had pre-existing autism spectrum disorder. The detection of a probable autoimmune pathophysiology in such cases seems important, as it offers new and more causal immunosuppressive treatment alternatives.

摘要

背景

自身免疫性脑炎,如抗 N-甲基-D-天冬氨酸受体(NMDA)脑炎,通常以神经精神症状亚急性起病,可通过抗神经元自身抗体、脑脊液(CSF)中的炎症变化以及脑电图(EEG)、磁共振成像(MRI)或[18F]氟脱氧葡萄糖正电子发射断层扫描(FDG PET)的病理改变来检测。对于以精神病性症状为主的患者,提出了自身免疫性精神病这一术语。在此,作者报告一例可能与未知抗神经元抗体相关的自身免疫性精神病患者的病例。

病例介绍

一名 18 岁男性患者,既往患有自闭症谱系障碍,在 2.5 年的时间里发展为严重的紧张症综合征。MRI 显示结果正常,EEG 显示间歇性减慢,独立成分分析显示有额外的尖峰。然而,FDG PET、血清/CSF 针对特征明确的抗神经元自身抗体的基础实验室分析和检测均未发现异常。使用未固定的鼠脑组织进行间接免疫荧光的血清和 CSF“基于组织的检测”显示,存在针对小脑颗粒细胞中未知表位以及海马中间神经元神经突的抗神经元自身抗体,呈现躯体树突状染色模式。大剂量糖皮质激素、血浆置换和利妥昔单抗的免疫抑制治疗导致部分改善。

结论

该患者可能患有自身抗体相关的自身免疫性精神病。该病例的特殊之处在于患者(1)除 EEG 改变并检测到针对目前未知表位的 CSF 自身抗体外,基本诊断大多不明显,(2)经历了孤立且持久的精神病病程,(3)既往患有自闭症谱系障碍。在此类病例中检测到可能的自身免疫病理生理学似乎很重要,因为它提供了新的、更具因果关系的免疫抑制治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac6f/7424063/872c4f8ee531/fpsyt-11-00627-g001.jpg

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