Department of Neurology, Institute of Surgery Research, Daping Hospital, Third Military Medical University (Army Medical University), 10 Changjiangzhilu, Yuzhong District, Chongqing, China.
Medicine (Baltimore). 2021 Jan 29;100(4):e24384. doi: 10.1097/MD.0000000000024384.
Anti-IgLON5 disease was first described as a progressive antibody-associated encephalopathy, with multiple non-specific clinical symptoms including sleep dysfunction, bulbar symptoms, progressive supranuclear palsy-like syndrome, cognitive impairment, and a variety of movement disorders. This newly discovered disease presents with unremarkable or unspecific brain magnetic resonance imagings (MRI), and have poor responsiveness to immunotherapy.
In this case, a 37-year-old man presented with 4-day history of gait instability, dysarthria, and oculomotor abnormalities. The initial neurologic examination revealed mild unsteady gait, subtle dysarthria, and left abducent paralysis.
The patient was diagnosed with anti-IgLON5 disease, based on clinical features and positive anti-IgLON5 antibodies in serum.
Initially, the patient was treated with high dosages of methylprednisolone and immunoglobulins.Outcomes: The symptoms of patient rapidly improved after high-dose intravenous methylprednisolone and immunoglobulins.
In this paper, we report a new case of anti-IgLON5 disease with major symptoms of gait instability, dysarthria, and oculomotor abnormalities, with distinctive brain MRI findings, and responsive to immunotherapy.
抗 IgLON5 病最初被描述为一种进行性抗体相关脑病，具有多种非特异性临床症状，包括睡眠功能障碍、球部症状、进行性核上性麻痹样综合征、认知障碍和多种运动障碍。这种新发现的疾病表现为无明显或无特异性的脑磁共振成像（MRI），且对免疫疗法反应不佳。
本例患者为 37 岁男性，以步态不稳、构音障碍和眼球运动异常 4 天为主诉就诊。初始神经系统检查显示轻度步态不稳、轻微构音障碍和左侧外展神经麻痹。
根据临床表现和血清中抗 IgLON5 抗体阳性，患者被诊断为抗 IgLON5 病。
患者最初接受大剂量甲基强的松龙和免疫球蛋白治疗。
患者在接受大剂量静脉注射甲基强的松龙和免疫球蛋白后，症状迅速改善。
本文报道了一例新的抗 IgLON5 病病例，主要症状为步态不稳、构音障碍和眼球运动异常，具有独特的脑 MRI 表现，对免疫疗法有反应。
