Data_Sheet_1_Investigating the association between neoplasms and MOG antibody-associated disease.DOCX

IntroductionThe 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.MethodsWe 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).ResultsTwo 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 (n = 4;23.5%), encephalomyelitis (n = 3;17.6%), and monolateral optic neuritis (n = 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 (n = 4), CNS (n = 3), melanoma (n = 2), lung (n = 2), hematological (n = 2), ovary (n = 1), breast (n = 1), gastrointestinal (n = 1), and thymic (n = 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.”DiscussionOur 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.

引言：髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）与肿瘤的关联报道极为罕见。本研究旨在调查MOGAD患者群体中肿瘤的发生情况，并描述其临床特征，此外，还包括之前报道的病例。方法：我们对2015年1月1日至2023年1月1日期间被诊断为MOGAD（即，符合临床表型且经活细胞检测的MOG抗体呈阳性）且在MOGAD发病后2年内确诊为肿瘤的患者进行了回顾性分析。此外，我们还对文献进行了系统回顾，以识别之前报道的病例。收集并报告了临床、实验室和肿瘤学发现，以中位数（范围）或数量（百分比）表示。结果：在我们的150名MOGAD患者中，有2例（1%）伴有并发肿瘤。从文献中检索到另外15个病例。中位年龄为39岁（16-73岁），其中12位患者为女性。最常见的是急性 disseminated encephalomyelitis（ADEM）（n = 4；23.5%）、脑脊髓炎（n = 3；17.6%）和单侧视神经炎（n = 2；11.8%）。中位治疗次数为1次（范围1-4），17例中有14例（82.4%）报告了改善。肿瘤伴随症状包括畸胎瘤（n = 4）、中枢神经系统（n = 3）、黑色素瘤（n = 2）、肺癌（n = 2）、血液学（n = 2）、卵巢（n = 1）、乳腺癌（n = 1）、胃肠道（n = 1）和胸腺（n = 1）肿瘤。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）。在2/4名患者中报告了肿瘤组织中MOG的表达。中位PNS-CARE评分为3（范围0-7）：11位患者被分类为“非PNS”，5位为“可能PNS”，1位为“可能PNS”。讨论：我们的研究证实，MOG是导致副肿瘤性神经综合征的低风险抗体，其临床表现和肿瘤伴随症状极其多变。大多数患者被归类为非PNS，而只有少数被诊断为可能/可能的PNS，通常与卵巢畸胎瘤相关。这些发现支持了MOGAD并非副肿瘤性疾病的观点。