Department of Pediatric Neurology, Osaka City General Hospital, Japan; Department of Pediatric Emergency Medicine, Osaka City General Hospital, Japan.
Department of Pediatric Neurology, Osaka City General Hospital, Japan.
Brain Dev. 2022 Mar;44(3):254-258. doi: 10.1016/j.braindev.2021.10.011. Epub 2021 Nov 18.
Anti-myelin oligodendrocyte glycoprotein (MOG) antibody can be detected not only in acute disseminated encephalomyelitis or optic neuritis but also in limbic or cortical encephalitis. However, no previous reports have demonstrated a relapsing case of these two types of encephalitis.
An 11-year-old girl presented with fever, headache, abnormal behavior, focal impaired awareness seizures (FIAS) on the left side, and MRI hyperintensities in the bilateral amygdala, hippocampus, and right posterior temporal cortex. The symptoms were alleviated with two courses of intravenous methylprednisolone (IVMP) and one course of immunoglobulin. At 16 years of age, the patient returned with left-sided headache and MRI hyperintensities in the left temporal, parietal, and insular cortices, which improved after 3 courses of IVMP. Oral prednisolone (PSL) was tapered over 6 months, when FIAS reappeared on the right side of the body. MRI showed recurrence in the same regions as in the second episode. She received 3 courses of IVMP, followed by gradually tapered PSL without relapse for 1.5 year. Anti-MOG antibodies were positive in both serum and the cerebrospinal fluid prior to treatment in all three episodes.
Our results revealed that anti-MOG antibody-related bilateral limbic and unilateral cortical encephalitis can manifest with a variety of phenotypes over time in the same patient.
抗髓鞘少突胶质细胞糖蛋白 (MOG) 抗体不仅可在急性播散性脑脊髓炎或视神经炎中检测到,也可在边缘性或皮质性脑炎中检测到。然而,以前的报告没有显示这两种类型的脑炎存在复发性病例。
一名 11 岁女孩出现发热、头痛、异常行为、左侧局灶性意识障碍性癫痫发作 (FIAS) 和双侧杏仁核、海马体及右侧颞叶后皮质的 MRI 高信号。两疗程静脉注射甲基泼尼松龙 (IVMP) 和一疗程免疫球蛋白治疗后症状缓解。16 岁时,患者因左侧头痛和左颞叶、顶叶和岛叶皮质的 MRI 高信号再次就诊,3 疗程 IVMP 治疗后改善。6 个月内逐渐减少口服泼尼松龙 (PSL),右侧身体出现 FIAS。MRI 显示与第二次发作相同的区域复发。她接受了 3 个疗程的 IVMP 治疗,随后逐渐减少 PSL,1.5 年内未复发。在所有三个发作中,在治疗前患者的血清和脑脊液中均检测到抗 MOG 抗体阳性。
我们的结果表明,抗 MOG 抗体相关的双侧边缘性和单侧皮质性脑炎可在同一患者中随时间表现出多种表型。