Kim Seoyeon, Ahn Seon-Jae, Chu Kon
Department of Neurology, Seoul National University Hospital, Seoul, Korea.
Center for Hospital Medicine, Seoul National University Hospital, Seoul, Korea.
Encephalitis. 2021 Jul;1(3):85-88. doi: 10.47936/encephalitis.2021.00066. Epub 2021 Jun 7.
Acute disseminated encephalomyelitis (ADEM) is a monophasic central nervous system inflammatory demyelinating disorder clinically defined by multifocal neurologic symptoms with encephalopathy. Brain magnetic resonance imaging most often reveals multiple T2 hyperintense lesions consistent with demyelination. High-dose corticosteroids are the current treatment of choice, and intravenous immunoglobulin or plasmapheresis is considered in steroid-unresponsive cases. The majority of patients show favorable outcomes with full clinical recovery and complete or partial resolution of previous lesions. For patients who are refractory to multiple immunomodulatory agents, other treatments such as rituximab have been used. We present a patient who developed ADEM after Epstein-Barr virus infection who achieved full recovery with immunotherapy including rituximab.
急性播散性脑脊髓炎(ADEM)是一种单相性中枢神经系统炎性脱髓鞘疾病,临床上以伴有脑病的多灶性神经系统症状为特征。脑部磁共振成像最常显示与脱髓鞘相符的多个T2高信号病变。大剂量皮质类固醇是目前的首选治疗方法,对于类固醇无反应的病例,可考虑静脉注射免疫球蛋白或血浆置换。大多数患者预后良好,临床完全恢复,先前病变完全或部分消退。对于多种免疫调节药物治疗无效的患者,已使用诸如利妥昔单抗等其他治疗方法。我们报告一例在感染爱泼斯坦-巴尔病毒后发生ADEM的患者,该患者通过包括利妥昔单抗在内的免疫疗法实现了完全康复。