CASPR2抗体疾病谱系中的神经性疼痛：一项系统综述

IF 2.5 4区医学 Q3 IMMUNOLOGY

Journal of neuroimmunology Pub Date : 2025-08-24 DOI:10.1016/j.jneuroim.2025.578734

Sara Cornacchini , Massimiliano Ugo Verza , Mattia Schiavolin , Gregorio Spagni , Ebe Schiavo , Antonio Lotti , Antonio Farina , Deborah Leccese , Alessandro Barilaro , Luca Massacesi , Valentina Damato

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引用次数: 0

摘要

背景：接触蛋白相关蛋白样2 （CASPR2）抗体主要与获得性神经肌强直、边缘脑炎和Morvan综合征有关。尽管如此，神经性疼痛的报道越来越多，尽管其特征的数据仍然有限。在这里，我们描述了一位与CASPR2抗体相关的孤立神经性疼痛患者，我们进行了系统回顾以更好地定义这一相关表现。方法按照CARE指南报告index病例。根据最新的PRISMA声明指南，在MEDLINE、Embase和Cochrane上进行系统评价。结果文献综述确定了216例CASPR2抗体疾病谱系中的神经性疼痛患者，仅16/216（7.4%）病例描述了孤立性疼痛。疼痛通常是严重的，表现为远端烧灼痛（74/147,50.3%），广泛的疼痛伴肌痛和痉挛（31/147,21.1%），或严重的背部疼痛放射到腿部（13/147,8.8%）。50/76（65.8%）的患者表现为周围神经兴奋性亢进，16/19（84.2%）的患者表现为小伤害纤维功能障碍。免疫治疗，特别是二线治疗，85.4%（76/89）的患者疼痛得到改善，34/89（38.2%）的患者症状完全缓解，而对症治疗帮助55.1%（27/49）的患者，10/49（20.4%）的患者完全缓解。经常需要长期治疗，停药后复发的发生率为5.2%（5/96）。结论神经性疼痛是CASPR2疾病的一种提示性相关症状。虽然罕见，但它可能是该病唯一的临床特征。CASPR2抗体检测应包括在神经性疼痛患者的筛查中，因为在这些病例中，快速启动免疫治疗和升级到二线治疗可能非常有效。注册信息系统评审协议已在PROSPERO网站注册[协议号CRD42024582950]。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

Neuropathic pain in CASPR2 antibody disease spectrum: A systematic review

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Neuropathic pain in CASPR2 antibody disease spectrum: A systematic review

Background

Contactin-associated protein-like 2 (CASPR2) antibodies are mainly linked to acquired neuromyotonia, limbic encephalitis and Morvan syndrome. Nevertheless, neuropathic pain is increasingly reported, though data on its characteristics remain limited. Here, we describe a patient with isolated neuropathic pain associated with CASPR2 antibodies and we conducted a systematic review to better define this relevant presentation.

Methods

Index case was reported following CARE guidelines. The systematic review was performed on MEDLINE, Embase, and Cochrane, according to the updated PRISMA statement guidelines.

Results

The literature review identified 216 patients with neuropathic pain in the context CASPR2 antibody disease spectrum, with isolated pain described in only 16/216 (7.4 %) cases. Pain was typically severe and presented as distal burning pain (74/147, 50.3 %), widespread pain with myalgia and cramps (31/147, 21.1 %), or severe back pain radiating to the legs (13/147, 8.8 %). Peripheral nerve hyperexcitability was found in 50/76 (65.8 %) of patients, while small nociceptive fiber dysfunction was identified in 16/19 (84.2 %). Immunotherapy, especially second line treatments, improved pain in 85.4 % (76/89) of cases, with complete remission of symptoms in 34/89 (38.2 %), while symptomatic treatment helped 55.1 % (27/49) of patients, with complete response in 10/49 (20.4 %). Long-term therapy was often needed, and relapses occurred in 5.2 % (5/96) after discontinuation.

Conclusions

Neuropathic pain appears to be a suggestive and relevant symptom in CASPR2 disease. Although rare, it can be the solely clinical feature of the disease. Testing for CASPR2 antibodies should be included in the screening of patients with neuropathic pain as in these cases rapid initiation of immunotherapy and escalation to second-line treatments can be highly effective.

Registration information

The systematic review protocol was registered in the PROSPERO website [Protocol number CRD42024582950].

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来源期刊

Journal of neuroimmunology 医学-免疫学

CiteScore

6.10

自引率

3.00%

发文量

154

审稿时长

37 days

期刊介绍： The Journal of Neuroimmunology affords a forum for the publication of works applying immunologic methodology to the furtherance of the neurological sciences. Studies on all branches of the neurosciences, particularly fundamental and applied neurobiology, neurology, neuropathology, neurochemistry, neurovirology, neuroendocrinology, neuromuscular research, neuropharmacology and psychology, which involve either immunologic methodology (e.g. immunocytochemistry) or fundamental immunology (e.g. antibody and lymphocyte assays), are considered for publication.