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[副肿瘤性神经系统综合征:实验室诊断及免疫学方面]

[Paraneoplastic neurologic syndromes: laboratory diagnostics and immunological aspects].

作者信息

Hayden Zsófia, Böröcz Katalin, Csizmadia Zsuzsanna, Kellermayer Zoltán, Balogh Péter, Berki Tímea

机构信息

Immunológiai és Biotechnológiai Intézet, Pécsi Tudományegyetem, Általános Orvostudományi Kar, Klinikai Központ, Pécs, Hungary.

出版信息

Magy Onkol. 2019 Sep 18;63(3):261-267. Epub 2019 Jul 18.

Abstract

Paraneoplastic neurologic syndromes (PNS) and autoimmune encephalitis (AE) are rare neurological disorders, which have similar symptoms, but vary in outcome and treatment strategy. In our retrospective statistical study we evaluated the autoantibody test results of serum and CSF from 2362 patients with suspected PNS and 1034 patients with suspected AE. For autoantibody testing, immunoblot assay (PNS) and cell-based indirect immunofluorescence assay (AE) were used. Autoantibodies were present in 8% of patients with suspected PNS: anti-Yo > anti-Hu > anti-Ma2 > anti-CV2 > anti-titin > anti-Zic4 > anti-amphiphysin > anti-Ri > anti-GAD65 > anti-Sox1 > anti-recoverin. Mostly elderly women were affected. Autoantibodies were present in 5.8% of patients with suspected AE: anti-NMDAR (young women) > anti-LGI1 (middle-aged men) > anti-GABABR (elderly men) > anti-Caspr2 (adult men). Our results correspond to the data described in the literature. The number of patients with suspected PNS and AE shows an increasing tendency, where the autoantibody testing with modern laboratory diagnostic methods helps in the early introduction of the appropriate therapy.

摘要

副肿瘤性神经系统综合征(PNS)和自身免疫性脑炎(AE)是罕见的神经系统疾病,它们症状相似,但预后和治疗策略有所不同。在我们的回顾性统计研究中,我们评估了2362例疑似PNS患者和1034例疑似AE患者的血清和脑脊液自身抗体检测结果。自身抗体检测采用免疫印迹法(用于PNS)和基于细胞的间接免疫荧光法(用于AE)。疑似PNS患者中有8%存在自身抗体:抗Yo>抗Hu>抗Ma2>抗CV2>抗肌联蛋白>抗Zic4>抗 amphiphysin>抗Ri>抗GAD65>抗Sox1>抗恢复蛋白。受影响的主要是老年女性。疑似AE患者中有5.8%存在自身抗体:抗NMDAR(年轻女性)>抗LGI1(中年男性)>抗GABABR(老年男性)>抗Caspr2(成年男性)。我们的结果与文献中描述的数据相符。疑似PNS和AE的患者数量呈上升趋势,采用现代实验室诊断方法进行自身抗体检测有助于尽早引入适当的治疗。

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