Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
NMO Clinical Service, The Walton Centre, Liverpool, UK.
Brain. 2017 Dec 1;140(12):3128-3138. doi: 10.1093/brain/awx276.
See de Seze (doi:10.1093/brain/awx292) for a scientific commentary on this article. A condition associated with an autoantibody against MOG has been recently recognized as a new inflammatory disease of the central nervous system, but the disease course and disability outcomes are largely unknown. In this study we investigated clinical characteristics of MOG-antibody disease on a large cohort of patients from the UK. We obtained demographic and clinical data on 252 UK patients positive for serum immunoglobulin G1 MOG antibodies as tested by the Autoimmune Neurology Group in Oxford. Disability outcomes and disease course were analysed in more detail in a cohort followed in the Neuromyelitis Optica Oxford Service (n = 75), and this included an incident cohort who were diagnosed at disease onset (n = 44). MOG-antibody disease affects females (57%) slightly more often than males, shows no ethnic bias and typically presents with isolated optic neuritis (55%, bilateral in almost half), transverse myelitis (18%) or acute disseminated encephalomyelitis-like presentations (18%). In the total Oxford cohort after a median disease duration of 28 months, 47% of patients were left with permanent disability in at least one of the following: 16% patients had visual acuity ≤6/36 in at least one eye, mobility was limited in 7% (i.e. Expanded Disability Status Scale ≥ 4.0), 5% had Expanded Disability Status Scale ≥ 6.0, 28% had permanent bladder issues, 20% had bowel dysfunction, and 21% of males had erectile dysfunction. Transverse myelitis at onset was a significant predictor of long-term disability. In the incident cohort 36% relapsed after median disease duration of 16 months. The annualized relapse rate was 0.2. Immunosuppression longer than 3 months following the onset attack was associated with a lower risk of a second relapse. MOG-antibody disease has a moderate relapse risk, which might be mitigated by medium term immunosuppression at onset. Permanent disability occurs in about half of patients and more often involves sphincter and erectile functions than vision or mobility.
请参阅 de Seze 的文章(doi:10.1093/brain/awx292),该文对本文进行了科学评论。最近,人们认识到一种与抗 MO G 自身抗体相关的疾病是一种新的中枢神经系统炎症性疾病,但疾病的病程和残疾结局在很大程度上仍不清楚。在这项研究中,我们对来自英国的大量 MO G 抗体疾病患者进行了临床特征研究。我们获得了牛津大学自身免疫神经病学组检测到的 252 名英国血清 IgG1 MO G 抗体阳性患者的人口统计学和临床数据。在神经脱髓鞘疾病牛津服务处(n = 75)随访的队列中,更详细地分析了残疾结局和疾病病程,这包括在疾病发病时诊断的发病队列(n = 44)。MO G 抗体疾病女性(57%)略多于男性,无种族偏见,通常表现为孤立性视神经炎(55%,近一半为双侧)、横贯性脊髓炎(18%)或急性播散性脑脊髓炎样表现(18%)。在牛津大学的总队列中,中位病程为 28 个月后,47%的患者至少在以下一项中存在永久性残疾:16%的患者至少一只眼视力≤6/36,7%的患者活动受限(即扩展残疾状态量表≥4.0),5%的患者扩展残疾状态量表≥6.0,28%的患者有永久性膀胱问题,20%的患者有肠道功能障碍,21%的男性有勃起功能障碍。发病时的横贯性脊髓炎是长期残疾的显著预测因素。在发病队列中,中位疾病持续时间为 16 个月后,36%的患者复发。年复发率为 0.2。发病后免疫抑制治疗时间超过 3 个月与第二次复发风险降低相关。MO G 抗体疾病的复发风险中等,发病时采用中期免疫抑制治疗可能会降低复发风险。大约一半的患者会发生永久性残疾,且更常涉及括约肌和勃起功能,而非视力或活动能力。