Department of Neurology, Dr. D.Y. Patil Medical College, Dr. D.Y. Patil Vidyapeeth Pune, Maharashtra, India.
Neurol India. 2020 Sep-Oct;68(5):1235-1237. doi: 10.4103/0028-3886.299157.
We report a case of a young female who had magnetic resonance imaging (MRI) brain lesions typical of multiple sclerosis (MS) with cerebrospinal fluid (CSF) oligoclonal bands (OCBs) and definite multiple sclerosis based on revised McDonald criteria; however, she also had atypical features of mild pleocytosis, brainstem and cerebellar peduncle involvement apart from opticospinal (OS) involvement. She also turned out to be positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody; hence, she was diagnosed with atypical multiple sclerosis. This case highlights when to suspect atypical MS and its management approach.
Typical MS cases are largely anti-MOG-negative. In a study of 50 Japanese cases, with anti-aquaporin 4 (AQP4)-immunoglobulin (IgG)-negative OSMS, just 2 were MOG-IgG-positive, but they had some features atypical for MS, such as bilateral optic neuritis, longitudinally extensive transverse myelitis, or moderate pleocytosis. In another study, antibodies to MOG were found in about 5% (5/104) of preselected adult patients with MS. Patients with MS with antibodies to MOG showed typical MS lesions on brain MRI with concomitant severe brainstem and spinal cord involvement and had a severe disease course with high relapse rates.
In conclusion, any patient showing typical MS lesions on brain MRI with OCB present in CSF but has atypical features like mild pleocytosis with brainstem, cerebellar, or OS involvement should also be tested for autoantibodies to MOG, and if positive, then he/she would require aggressive treatment approach in the form of plasma exchange, if resistant to pulse steroid therapy, followed by either rituximab or natalizumab rather than trying other disease modifying therapies (DMTs).
我们报告了一例年轻女性患者,其脑部磁共振成像(MRI)存在多发性硬化症(MS)的典型病变,脑脊液(CSF)寡克隆带(OCBs)阳性,且符合修订后的 McDonald 标准,明确诊断为多发性硬化症;然而,她还伴有轻度白细胞增多、脑干和小脑脚受累等不典型特征,此外还存在视神经脊髓炎(OS)受累。她还被检测出抗髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性,因此被诊断为不典型多发性硬化症。该病例强调了何时怀疑不典型 MS 及其管理方法。
典型 MS 病例大多为抗-MOG 阴性。在一项对 50 例日本病例的研究中,抗水通道蛋白 4(AQP4)-免疫球蛋白(IgG)阴性的 OSMS 中,仅有 2 例 MOG-IgG 阳性,但它们具有一些不符合 MS 的特征,如双侧视神经炎、长节段横贯性脊髓炎或中度白细胞增多。在另一项研究中,在约 5%(5/104)的经预选的成人 MS 患者中发现了针对 MOG 的抗体。患有 MOG 抗体的 MS 患者在脑 MRI 上显示出典型的 MS 病变,同时伴有严重的脑干和脊髓受累,疾病病程严重,复发率高。
总之,任何在脑 MRI 上显示出典型 MS 病变且 CSF 中存在 OCB,但具有轻度白细胞增多、脑干、小脑或 OS 受累等不典型特征的患者,也应进行 MOG 自身抗体检测,如果阳性,则需要采用血浆置换进行积极治疗,如果对脉冲类固醇治疗无反应,则应选择利妥昔单抗或那他珠单抗,而不是尝试其他疾病修正疗法(DMTs)。