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研究肿瘤与MOG抗体相关疾病之间的关联。

Investigating the association between neoplasms and MOG antibody-associated disease.

作者信息

Trentinaglia Milena, Dinoto Alessandro, Carta Sara, Chiodega Vanessa, Ferrari Sergio, Andreone Vincenzo, Maniscalco Giorgia Teresa, Mariotto Sara

机构信息

Neurology Unit, Department of Neurosciences, Biomedicine, and Movement Sciences, University of Verona, Verona, Italy.

Neurological Clinic and Stroke Unit, Naples, Italy.

出版信息

Front Neurol. 2023 Jun 9;14:1193211. doi: 10.3389/fneur.2023.1193211. eCollection 2023.

Abstract

INTRODUCTION

The association of myelin oligodendrocyte glycoprotein (MOG) antibody associated disease (MOGAD) and tumors has seldom been reported. We aim to investigate the occurrence of tumors in a cohort of patients with MOGAD and to describe their clinical features, in addition to previously reported cases.

METHODS

We retrospectively identified patients with MOGAD (i.e., compatible clinical phenotype and positive MOG antibodies analysed with a live cell-based assay) from 1/1/2015 to 1/1/2023 who had a neoplasm diagnosed within 2  years from MOGAD onset. Furthermore, we performed systematic review of literature to identify previously reported cases. Clinical, paraclinical and oncological findings were collected and reported as median (range) or number (percentage).

RESULTS

Two of 150 MOGAD patients (1%) had a concomitant neoplasm in our cohort. Fifteen additional cases were retrieved from literature. Median age was 39 (16-73) years-old, 12 patients were female. ADEM ( = 4;23.5%), encephalomyelitis ( = 3;17.6%), and monolateral optic neuritis ( = 2;11.8%) were the most frequent phenotypes. Median number of treatments was 1 (range 1-4), improvement was reported in 14/17 cases (82.4%). Oncological accompaniments were teratoma ( = 4), CNS ( = 3), melanoma ( = 2), lung ( = 2), hematological ( = 2), ovary ( = 1), breast ( = 1), gastrointestinal ( = 1), and thymic ( = 1) neoplasms. Median time from tumor diagnosis to MOGAD onset was 0 (range - 60 to 20) months. MOG expression in neoplastic tissue was reported in 2/4 patients. Median PNS-CARE score was 3 (range 0-7): 11 patients were classified as "non-PNS," 5 as "possible PNS," and 1 as "probable PNS."

DISCUSSION

Our study confirms that MOG is a low-risk antibody for paraneoplastic neurological syndromes and that the clinical presentation and oncological accompaniments are extremely variable. Most of these patients were classified as non-PNS, whereas only a minority was diagnosed with possible/probable PNS, frequently in association with ovarian teratoma. These findings support the notion that MOGAD is not a paraneoplastic disease.

摘要

引言

髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)与肿瘤的关联鲜有报道。除先前报道的病例外,我们旨在调查一组MOGAD患者中肿瘤的发生情况,并描述其临床特征。

方法

我们回顾性确定了2015年1月1日至2023年1月1日期间患有MOGAD(即具有符合的临床表型且基于活细胞检测分析的MOG抗体呈阳性)且在MOGAD发病后2年内诊断出肿瘤的患者。此外,我们对文献进行了系统回顾以确定先前报道的病例。收集临床、副临床和肿瘤学检查结果,并报告为中位数(范围)或数量(百分比)。

结果

在我们的队列中,150例MOGAD患者中有2例(1%)患有伴发肿瘤。从文献中检索到另外15例病例。中位年龄为39岁(16 - 73岁),12例患者为女性。急性播散性脑脊髓炎(n = 4;23.5%)、脑脊髓炎(n = 3;17.6%)和单侧视神经炎(n = 2;11.8%)是最常见的表型。中位治疗次数为1次(范围1 - 4次),14/17例(82.4%)报告有改善。肿瘤伴随情况包括畸胎瘤(n = 4)、中枢神经系统肿瘤(n = 3)、黑色素瘤(n = 2)、肺癌(n = 2)、血液系统肿瘤(n = 2)、卵巢肿瘤(n = 1)、乳腺肿瘤(n = 1)、胃肠道肿瘤(n = 1)和胸腺肿瘤(n = 1)。从肿瘤诊断到MOGAD发病的中位时间为0个月(范围 - 60至20个月)。4例患者中有2例报告了肿瘤组织中的MOG表达。中位PNS - CARE评分为3分(范围0 - 7分):11例患者被分类为“非副肿瘤性神经系统综合征(non - PNS)”,5例为“可能的PNS”,1例为“很可能的PNS”。

讨论

我们的研究证实,MOG是副肿瘤性神经系统综合征的低风险抗体,其临床表现和肿瘤伴随情况极为多样。这些患者大多数被分类为非PNS,而只有少数被诊断为可能/很可能的PNS,且常与卵巢畸胎瘤相关。这些发现支持MOGAD不是副肿瘤性疾病这一观点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/070d/10289293/e1e347fdb2a6/fneur-14-1193211-g001.jpg

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