Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, 13-1 Takara-machi, Kanazawa, 920-8640, Japan.
Department of Neurosurgery, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan.
Neurol Sci. 2021 Nov;42(11):4731-4735. doi: 10.1007/s10072-021-05419-4. Epub 2021 Jun 25.
Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ADEM-ON) is characterized by the following features: early onset, monophasic or multiphasic ADEM followed by one or more episodes of ON, and the presence of serum anti-myelin oligodendrocyte glycoprotein (MOG) antibodies.
We report a case of ADEM-ON without anti-MOG antibodies in a 78-year-old woman. The patient developed acute-onset neurological findings and was diagnosed with ADEM. She was treated with intravenous methylprednisolone (IVMP), and oral corticosteroids. Her clinical symptoms and MRI findings subsequently improved. Left optic neuritis emerged 6 months later, and we made a diagnosis of ADEM-ON. A brain biopsy performed during the acute phase of ADEM showed perivascular infiltration of macrophages with demyelination.
The majority of the reported ADEM-ON cases are pediatric cases with serum anti-MOG antibodies, but our patient was the elderly, without anti-MOG antibodies. Moreover, the pathological features of our case were similar to those observed in patients with typical ADEM and in patients with anti-MOG antibody-positive ADEM. Although ADEM-ON is related to the presence of anti-MOG antibodies, factors other than anti-MOG antibodies could contribute to the development of ADEM-ON.
急性播散性脑脊髓炎(ADEM)继视神经炎(ADEM-ON)的特征如下:早发、单相或多相 ADEM 后出现一次或多次 ON 发作,以及血清抗髓鞘少突胶质细胞糖蛋白(MOG)抗体存在。
我们报告一例 78 岁女性 ADEM-ON 而无抗 MOG 抗体。患者出现急性发作性神经表现,诊断为 ADEM。她接受了静脉注射甲基泼尼松龙(IVMP)和口服皮质类固醇治疗。她的临床症状和 MRI 发现随后得到改善。6 个月后出现左侧视神经炎,我们诊断为 ADEM-ON。在 ADEM 的急性期进行的脑活检显示巨噬细胞围绕血管浸润伴脱髓鞘。
报告的大多数 ADEM-ON 病例为伴有血清抗 MOG 抗体的儿科病例,但我们的患者为老年人,无抗 MOG 抗体。此外,我们病例的病理特征与典型 ADEM 患者和抗 MOG 抗体阳性 ADEM 患者观察到的特征相似。尽管 ADEM-ON 与抗 MOG 抗体的存在有关,但除抗 MOG 抗体以外的因素也可能导致 ADEM-ON 的发生。