Glial fibrillary acidic protein astrocytopathy presenting as progressive encephalomyelitis with rigidity and myoclonus: 1 case report
10.3760/cma.j.cn113694-20241130-00774
- VernacularTitle:以伴强直及肌阵挛的进行性脑脊髓炎为突出表现的自身免疫性胶质纤维酸性蛋白星形胶质细胞病1例
- Author:
Sheng ZHUANG
1
;
Hanying GU
1
;
Jinru ZHANG
1
;
Huihui LIU
1
;
Weidong HU
1
Author Information
1. 苏州大学附属第二医院神经内科，苏州215004
- Publication Type:Journal Article
- Keywords:
Autoimmune diseases;
Glial fibrillary acidic protein;
Astrocytes;
Encephalomyelitis
- From:
Chinese Journal of Neurology
2025;58(8):873-876
- CountryChina
- Language:Chinese
-
Abstract:
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an immune-mediated inflammatory disease of the central nervous system. However, GFAP-A characterized by progressive encephalomyelitis with rigidity and myoclonus as its predominant clinical phenotype is rare. This article described a case, supplemented with clinical video, of a young male patient who developed fever and neck pain at disease onset. Over the course of the illness, the patient experienced progressively worsening painful muscle spasms, muscle rigidity, and stimulus-sensitive myoclonus, accompanied by autonomic dysfunction. Cerebrospinal fluid analysis revealed positive GFAP-IgG antibodies. The patient showed significant improvement with treatment of high-dose corticosteroids, intravenous immunoglobulin, and mycophenolate mofetil.
