成年人抗接触蛋白相关蛋白2抗体自身免疫性脑炎的临床特征研究

doi:10.12114/j.issn.1007-9572.2021.02.023

摘要/Abstract

摘要： 背景　抗接触蛋白相关蛋白2（Caspr 2）抗体相关脑炎临床罕见，临床表现多变，诊断困难，目前国内外针对该病的研究少见。目的　总结并分析成年人抗Caspr 2抗体自身免疫性脑炎（AE）患者的临床表现、辅助检查、治疗及预后，以提高临床医生对该病的认识。方法　2016年4月至2020年12月于南昌大学第二附属医院神经内科就诊的表现为急性或亚急性脑炎症状的成年患者中被确诊为AE者共198例，回顾性选取其中血清或脑脊液抗Caspr 2抗体阳性患者14例为研究对象。回顾性收集14例抗Caspr 2抗体AE患者的临床特征、实验室检查结果、影像学检查结果、脑电图表现、治疗和随访。结果　14例患者中男11例，女3例；平均年龄（47.9±19.6）岁。精神行为异常是最常见首发症状；病程中11例出现边缘性脑炎症状（精神行为异常9例、认知功能下降7例、癫痫发作6例），10例出现自主神经功能障碍（合并多汗症8例、窦性心动过速6例、便秘2例、小便困难1例），6例出现失眠，5例出现周围神经高兴奋性症状，4例出现神经性疼痛，3例出现小脑症状。血清抗Caspr 2抗体阳性13例，脑脊液抗Caspr 2抗体阳性8例，其中5例患者同时合并抗N-甲基-D-天冬氨酸受体（NMDAR）抗体阳性。所有患者行肿瘤筛查未发现肿瘤。患者均接受一线免疫治疗，10例预后良好，1例死亡，1例复发，1例仍在治疗中，1例并发左侧股骨头坏死。结论　抗Caspr 2抗体AE临床表现多样，可累及中枢和周围神经肌肉系统及自主神经系统等，发生心律失常时应警惕猝死的可能，可出现其他类型自身抗体阳性，免疫治疗有效，可有复发。

Abstract: Background　Anti-contact protein related protein-2 （Caspr 2） antibody autoimmune-associated encephalitis （AE） is a seldom encountered disease with diverse clinical manifestations，which is difficult to diagnose，and has been rarely studied. Objective　This study was aimed to summarize and analyze the clinical features，auxiliary examinations，treatments，and prognosis of anti-Caspr 2 antibody AE in adults，to improve clinicians' understanding of this disease. Methods　Fourteen patients who were positive for anti-Caspr 2 antibody in the serum or cerebrospinal fluid （CSF） were retrospectively selected from 198 adults with acute or subacute encephalitis symptoms and a definite diagnosis of AE who hospitalized in Department of Neurology，the Second Affiliated Hospital of Nanchang University from April 2016 to December 2020. The clinical features，laboratory test results，imaging results，EEG results，treatments，and follow-up data of these patients were collected. Results　Among the 14 patients〔including 11 men and three women，with a mean age of （47.9±19.6） years〕，mental and behavioral disorders were the most common initial symptoms；during the course of disease，11 showed limbic encephalitis symptoms （nine with mental and behavioral disorders，seven with declined cognitive function，and six with epilepsy），10 showed autonomic dysfunction （eight with hyperhidrosis，six with sinus tachycardia，two with constipation，and one with dysuresia），six had insomnia，and three showed cerebellar symptoms，five showed peripheral nerve hyperexcitability symptoms，four showed neuropathic pain. Laboratory tests found that 13 were positive for serum anti-Caspr 2 antibody，eight were positive for CSF anti-Caspr 2 antibody，and five were concurrently positive for anti-NMDAR antibody. None of the patients were identified with cancer by cancer screening. All patients received first-line immunotherapy，10 of them showed good prognosis，one died，one had recurrence，one was still undergoing treatment（by the end of the follow-up），and one had comlicated with head necrosis. Conclusion　Anti-Caspr 2 antibody AE has diverse clinical manifestations that may involve the central and peripheral neuromuscular systems and autonomic nervous system. Sudden death is possible when arrhythmia occurs，and the patients may be positive for other types of autoantibodies. Immunotherapy is effective for this disease，but recurrence may still occur.

Key words: Encephalitis, Contact protein related protein-2 antibody-related encephalitis, Anti-N-methyl-D-aspartate receptor encephalitis, Syringomyelia, Antibodies

吴兰香,田胜,郑合情,等. 成年人抗接触蛋白相关蛋白2抗体自身免疫性脑炎的临床特征研究[J]. 中国全科医学, 2021, 24(30): 3870-3877. DOI: 10.12114/j.issn.1007-9572.2021.02.023.
WU Lanxiang,TIAN Sheng,ZHENG Heqing, et al. Clinical Characteristic of Anti-Caspr 2 Antibody Autoimmune-associated Encephalitis in Adults　[J]. Chinese General Practice, 2021, 24(30): 3870-3877. DOI: 10.12114/j.issn.1007-9572.2021.02.023.

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