Service de Neurologie, Sclérose en Plaques, Pathologies de la Myéline et Neuro-Inflammation, Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon.
Centre de Reférence des Maladies Inflammatoires Rares du Cerveau et de la Moelle, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon.
Curr Opin Neurol. 2019 Jun;32(3):459-466. doi: 10.1097/WCO.0000000000000681.
The clinical interest for auto-antibodies against myelin oligodendrocyte glycoprotein (MOG) has recently reemerged, with the use of more specific detection methods. Large national cohorts have allowed characterizing a more precise clinical spectrum delineated by the presence of human MOG-antibodies.
In adults with MOG-antibodies, optic neuritis is the most frequent clinical presentation, with features different from multiple sclerosis (MS), including bilateral involvement and predilection for the anterior part of the optic nerve. Myelitis and brainstem syndrome are also frequent, and may clinically mimic neuromyelitis optica spectrum disorders (NMOSD). Despite the frequently severe clinical presentation, most of patients recover quickly after steroids initiation. Other less typical presentations include encephalitis with seizures, cranial nerve involvement, and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids-like. Although the majority of adult patients follow a relapsing course, long-term prognosis differs from aquaporin-4-antibodies NMOSD, with only a small proportion of patients with a poor outcome.
MOG-antibodies-associated disease is a new entity in the spectrum of inflammatory demyelinating diseases, distinct from both MS and NMOSD. There is a crucial need to identify factors associated to the risk of relapse or poor outcome, to seek patient subgroups in which immunoactive treatments could be beneficial.
近期,随着更特异的检测方法的应用,自身抗体抗髓鞘少突胶质细胞糖蛋白(MOG)的临床意义再次受到关注。大样本的国家队列研究使得更精确的临床谱得以描绘,该谱与人类 MOG 抗体的存在相关。
在成人 MOG 抗体患者中,视神经炎是最常见的临床表现,其特征与多发性硬化(MS)不同,包括双侧受累和视神经前部偏好性。脊髓炎和脑干综合征也很常见,可能在临床上类似于视神经脊髓炎谱系疾病(NMOSD)。尽管临床表现通常严重,但大多数患者在开始类固醇治疗后很快恢复。其他不太典型的表现包括伴有癫痫发作的脑炎、颅神经受累和慢性淋巴细胞性炎症伴桥脑血管周围强化,对类固醇样药物有反应。尽管大多数成年患者呈复发病程,但长期预后与水通道蛋白 4 抗体 NMOSD 不同,仅有一小部分患者预后不良。
MOG 抗体相关疾病是炎症性脱髓鞘疾病谱中的一种新实体,与 MS 和 NMOSD 均不同。迫切需要识别与复发风险或不良预后相关的因素,以寻找免疫活性治疗可能有益的患者亚组。