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副肿瘤性神经系统综合征患者的免疫表型特征:一项多中心研究。

Immunophenotypical characterization of paraneoplastic neurological syndrome patients: a multicentric study.

机构信息

UOC Neurology and Stroke Unit, ASST Lecco, Merate, LC, Italy.

出版信息

J Biosci. 2021;46.

PMID:33709965
Abstract

Paraneoplastic neurological syndromes (PNS) are a group of rare and severe immune-mediated disorders that affect the nervous system in patients with cancer. The best way to diagnose a paraneoplastic neurological disorder is to identify anti-onconeural protein antibodies that are specifically associated with various cancers. The aim of this multicentric study was to clinically and immunologically characterize patients with PNS and study their association with cancer. Patients suspected to have PNS were enrolled from various clinical centres and were characterized immunologically. This study population consisted of 112 patients. Onset of PNS was mainly subacute (76 %). PNS patients had various neurological disorders and symptoms. PNS developed before the diagnosis of cancer in 28 definite PNS patients and in six suspected PNS patients. The most frequent autoantibodies detected in PNS patients were anti-Hu and anti-Yo. One definite PNS patient with cerebellar syndrome had anti-Tr antibody and seven patients had atypical antibodies. The literature associates these antibodies with various neurological disorders and cancers. Our observations confirm the important role of autoantibodies in PNS and their importance for the early diagnosis of cancer in PNS patients.

摘要

副肿瘤性神经系统疾病(PNS)是一组罕见且严重的免疫介导性疾病,影响癌症患者的神经系统。诊断副肿瘤性神经疾病的最佳方法是识别与各种癌症特异性相关的抗神经原性蛋白抗体。本多中心研究的目的是临床和免疫上描述 PNS 患者,并研究其与癌症的关联。从各个临床中心招募疑似患有 PNS 的患者,并进行免疫学特征分析。该研究人群包括 112 名患者。PNS 的发病主要为亚急性(76%)。PNS 患者有各种神经系统疾病和症状。在 28 例明确的 PNS 患者和 6 例疑似 PNS 患者中,PNS 在癌症诊断前发生。在 PNS 患者中检测到的最常见的自身抗体是抗 Hu 和抗 Yo。1 例小脑综合征的明确 PNS 患者存在抗 Tr 抗体,7 例患者存在非典型抗体。文献将这些抗体与各种神经疾病和癌症相关联。我们的观察结果证实了自身抗体在 PNS 中的重要作用及其对 PNS 患者癌症早期诊断的重要性。

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Medicine (Baltimore). 2024 May 10;103(19):e38148. doi: 10.1097/MD.0000000000038148.
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Nat Rev Neurol. 2023 Mar;19(3):172-190. doi: 10.1038/s41582-023-00776-4. Epub 2023 Feb 14.
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神经眼科学中的副肿瘤综合征。
J Neurol. 2022 Oct;269(10):5272-5282. doi: 10.1007/s00415-022-11247-z. Epub 2022 Jul 2.
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Paraneoplastic Neurological Syndromes: Transitioning Between the Old and the New.副肿瘤性神经系统疾病:新旧之间的转变。
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