The autoimmune GFAP astrocytopathy has been associated with meningoencephalomyelitis that usually responds to glucocorticoids. We report a 20-year-old man that developed an acute and severe meningoencephalomyelitis with remarkable CNS hyperexcitability and oculogyric crises. CSF analysis showed hypoglycorrhachia, pleocytosis, elevated ADA, and CSF-immunofluorescence characteristic of autoimmune GFAP astrocytopathy. MRI showed lesions at thalamus, corpus-callosum, dorsal pons and dentate nucleus with associated myelitis. Immunotherapy led to a full recovery, although MRI activity was observed at follow-up. CNS hyperexcitability, typically seen in other immune-mediated syndromes, represents a novel presenting form to be included as part of the clinical spectrum of this entity.

自身免疫性 GFAP 星形细胞病与通常对糖皮质激素有反应的脑膜脑脊髓炎有关。我们报告了一名 20 岁的男子，他患有急性和严重的脑膜脑脊髓炎，伴有显着的中枢神经系统过度兴奋和眼科危象。CSF 分析显示自身免疫性 GFAP 星形细胞病的低血糖、细胞增多症、ADA 升高和 CSF 免疫荧光特征。MRI 显示丘脑、胼胝体、脑桥背侧和齿状核病变伴脊髓炎。尽管在随访中观察到 MRI 活动，但免疫治疗导致完全康复。CNS 过度兴奋，通常见于其他免疫介导的综合征，代表了一种新的表现形式，将作为该实体临床谱的一部分。