Paraneoplastic Neurologic Syndromes Associated With Merkel Cell Carcinoma.

IF 7.8 1区医学 Q1 CLINICAL NEUROLOGY

Neurology® Neuroimmunology & Neuroinflammation Pub Date : 2024-11-01 Epub Date: 2024-10-07 DOI:10.1212/NXI.0000000000200260

Nicolás Lundahl Ciano-Petersen, Sergio Muñiz-Castrillo, Macarena Villagrán-García, Antonio Farina, Alberto Vogrig, Valentin Wucher, Le Duy, Cristina Birzu, David Goncalves, Olivier Flabeau, Coline Duwicquet, Adrien Benard, Fabien Nicole, Veronique Rogemond, Geraldine Picard, Bastien Joubert, Jerome Honnorat

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引用次数: 0

Abstract

Background and objectives: To define the clinical and immunologic profile of patients with paraneoplastic neurologic syndromes (PNSs) associated with Merkel cell carcinoma (MCC).

Methods: Retrospective analysis was conducted on patients with suspected MCC-related PNS assessed at the French Reference Center, and cases were identified by a systematic review of the literature (MEDLINE, Embase) following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

Results: A total of 17 patients were identified in our center and 30 in the systematic review, resulting in an overall cohort of 47 patients. The median age was 65 years (range 41-90), and 30 of 46 (65%) were men. Lambert-Eaton myasthenic syndrome (LEMS) (14/47, 29%), rapidly progressive cerebellar syndrome (11/47, 23%), and encephalomyelitis (EM) (8/47, 17%) were the most common associated clinical phenotypes. The most frequently associated neural antibodies (Abs) were voltage-gated calcium channel (VGCC)-Abs (14/45, 31%), followed by Hu-Abs (8/45, 17%) and neurofilament (NF)-Abs (8/45, 17%). Patients with NF-Abs only exhibited CNS disorders (8/8, 100%) and often had antibodies against >1 NF subunit (6/8, 75%). At onset, 26 of 43 patients (60%) had no identifiable primary skin tumor but had lymph node metastasis; these patients were more frequently men (21/26, 80%, vs 7/17, 41%; p = 0.007), had more frequently VGCC-Abs (12/26, 46%, vs 2/17, 11%, p = 0.02) predominantly among those with LEMS, and presented reduced mortality than patients with a known primary tumor (5/25, 20%, vs 8/15, 53%; p = 0.02).

Discussion: MCC-related PNSs present as a heterogeneous clinical spectrum including central and/or peripheral nervous system disorders such as LEMS, RCPS, and EM, mainly associated with VGCC-Abs, NF-Abs, and Hu-Abs. NF-Abs were only seen among patients with CNS disorders. At onset, the absence of a primary skin tumor but presence of lymph node metastasis is frequently observed, and this particular clinical presentation is linked to reduced mortality, highlighting distinctive clinical and immunologic features of MCC-related PNS.

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与梅克尔细胞癌相关的副肿瘤性神经系统综合征。

背景和目的：确定与梅克尔细胞癌（MCC）相关的副肿瘤性神经综合征（PNS）患者的临床和免疫特征：界定与梅克尔细胞癌（MCC）相关的副肿瘤性神经综合征（PNS）患者的临床和免疫学特征：对法国参考资料中心评估的疑似梅克尔细胞癌相关副肿瘤性神经综合征患者进行回顾性分析，并根据《系统综述和荟萃分析首选报告项目》指南，通过系统性文献综述（MEDLINE、Embase）确定病例：结果：我们中心共发现了 17 名患者，系统性回顾中发现了 30 名患者，总计 47 名患者。中位年龄为 65 岁（41-90 岁不等），46 人中有 30 人（65%）为男性。兰伯特-伊顿肌萎缩综合征（LEMS）（14/47，29%）、快速进展性小脑综合征（11/47，23%）和脑脊髓炎（EM）（8/47，17%）是最常见的相关临床表型。最常见的相关神经抗体（Abs）是电压门控钙通道（VGCC）抗体（14/45，31%），其次是Hu-Abs（8/45，17%）和神经丝（NF）抗体（8/45，17%）。NF-Abs患者仅表现为中枢神经系统疾病（8/8，100%），通常有针对>1个NF亚基的抗体（6/8，75%）。发病时，43 名患者中有 26 名（60%）没有可确定的原发性皮肤肿瘤，但有淋巴结转移；这些患者中男性较多（21/26，80%，vs 7/17，41%；P = 0.007），VGCC-Abs 较多（12/26，46%，vs 2/17，11%，P = 0.02），主要是在 LEMS 患者中，死亡率低于已知原发性肿瘤患者（5/25，20%，vs 8/15，53%；P = 0.02）：讨论：与MCC相关的PNS表现为异质性临床谱系，包括中枢和/或周围神经系统疾病，如LEMS、RCPS和EM，主要与VGCC-Abs、NF-Abs和Hu-Abs相关。NF-Abs仅见于中枢神经系统疾病患者。在发病时，经常观察到没有原发性皮肤肿瘤但存在淋巴结转移的情况，这种特殊的临床表现与死亡率降低有关，突出了 MCC 相关 PNS 的独特临床和免疫学特征。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurology® Neuroimmunology & Neuroinflammation Neuroscience-Neurology

CiteScore

15.60

自引率

2.30%

发文量

219

审稿时长

8 weeks

期刊介绍： Neurology Neuroimmunology & Neuroinflammation is an official journal of the American Academy of Neurology. Neurology: Neuroimmunology & Neuroinflammation will be the premier peer-reviewed journal in neuroimmunology and neuroinflammation. This journal publishes rigorously peer-reviewed open-access reports of original research and in-depth reviews of topics in neuroimmunology & neuroinflammation, affecting the full range of neurologic diseases including (but not limited to) Alzheimer's disease, Parkinson's disease, ALS, tauopathy, and stroke; multiple sclerosis and NMO; inflammatory peripheral nerve and muscle disease, Guillain-Barré and myasthenia gravis; nervous system infection; paraneoplastic syndromes, noninfectious encephalitides and other antibody-mediated disorders; and psychiatric and neurodevelopmental disorders. Clinical trials, instructive case reports, and small case series will also be featured.