Department NEUROFARBA, Section Neurosciences, University of Florence, Viale Pieraccini 16, 50139 Florence, Italy.
Department NEUROFARBA, Section Neurosciences, University of Florence, Viale Pieraccini 16, 50139 Florence, Italy.
Mult Scler Relat Disord. 2019 Aug;33:51-54. doi: 10.1016/j.msard.2019.05.006. Epub 2019 May 16.
The spectrum of differential diagnosis of acquired demyelinating disorders of the central nervous system has been recently broadened. There is now growing evidence that supports anti-myelin oligodendrocyte antibodies associated demyelination as a distinct disease entity, with some clinical characteristics that somehow overlap those of Multiple Sclerosis (MS) and anti-AQP4+ Neuromyelitis Optica Spectrum Disorders (AQP4+NMOSD) but different pathogenesis and treatment strategies.
We hereby present 3 cases of anti-MOG+ patients with different disease courses - ranging from mild to severe - all presenting with Optic neuritis (ON) at the onset. Optic neuritis (ON) is a common manifestation of different central nervous system (CNS) inflammatory disorders and can represent the first clinical event of MS and NMOSD. ON is also the most common presentation of antiMOG demyelinating disorders, followed by - and sometimes associated with - myelitis, most commonly extended over more than 2 spinal cord segments and defined as longitudinally extended transverse myelitis (LETM). All the three patients tested negative for oligoclonal bands in CSF and anti-AQP4 Ab in serum, had a relapsing disease course characterized by prominent involvement of the optic nerve and spinal cord, with good recovery after treatment with high-dose corticosteroids. However, they had a different disease course at follow-up and underwent different treatment approaches.
Since anti-MOG+ patients can have a multiphasic disease course and accumulate disability over time, a high degree of suspicion and early diagnosis are of critical importance for treatment decision-making in clinical practice.
The aim of this case report is to enhance focus on an emerging disease spectrum among acquired CNS demyelinating disorders.
中枢神经系统获得性脱髓鞘疾病的鉴别诊断范围近年来已扩大。现在有越来越多的证据支持抗髓鞘少突胶质细胞抗体相关脱髓鞘是一种独特的疾病实体,其一些临床特征与多发性硬化症(MS)和抗 AQP4+视神经脊髓炎谱系障碍(AQP4+NMOSD)重叠,但发病机制和治疗策略不同。
我们在此介绍了 3 例具有不同病程的抗 MOG+患者 - 从轻度到重度不等 - 所有患者在发病时均表现为视神经炎(ON)。视神经炎(ON)是不同中枢神经系统(CNS)炎症性疾病的常见表现,可代表 MS 和 NMOSD 的首发临床事件。ON 也是抗 MOG 脱髓鞘疾病的最常见表现,其次是 - 有时与 - 脊髓炎相关,最常见的是超过 2 个脊髓节段的扩展,定义为纵向扩展的横贯性脊髓炎(LETM)。所有 3 例患者的 CSF 寡克隆带和血清抗 AQP4 Ab 均为阴性,疾病呈复发病程,视神经和脊髓受累明显,经大剂量皮质类固醇治疗后恢复良好。然而,他们在随访时有不同的病程,并接受了不同的治疗方法。
由于抗 MOG+患者可能有阶段性疾病病程,并随着时间的推移累积残疾,因此在临床实践中,高度怀疑和早期诊断对于治疗决策至关重要。
本病例报告的目的是提高对中枢神经系统获得性脱髓鞘疾病中新兴疾病谱的关注。