Research progress of autoimmune glial fibrillary acidic protein astrocytopathy
10.3760/cma.j.cn101070-20231019-00292
- VernacularTitle:自身免疫性胶质纤维酸性蛋白星形胶质细胞病研究进展
- Author:
Yanmei TIAN
1
;
Rong LUO
;
Qianyun CAI
Author Information
1. 四川大学华西第二医院小儿神经内科，成都 610041
- Keywords:
Glial fibrillary acidic protein;
Encephalitis;
Myelitis;
Autoimmune glial fibrillary acidic protein astrocytopathy;
Research progress
- From:
Chinese Journal of Applied Clinical Pediatrics
2024;39(6):473-476
- CountryChina
- Language:Chinese
-
Abstract:
Autoimmune glial fibrillary acidic protein astrocytopathy(GFAP-A) is a novel autoimmune disease of the central nervous system.The disease can affect any region of the cerebrum, cerebellum, optic nerve, and spinal cord, with encephalitis, meningoencephalitis and encephalomyelitis being the most common.Patients usually present with headache, fever, disturbance of consciousness, autonomic dysfunction, and visual impairment, and the diagnosis is mainly based on positive anti-glial fibrillary acidic protein antibodies in cerebrospinal fluid and serum.The disease is sensitive to glucocorticoids, with 70% of patients responding well to high-dose glucocorticoid pulse therapy and having a good prognosis.About one in five patients have residual nervous system sequelae, requiring long-term immunosuppressive therapy.This paper reviews the etiology, pathogenesis, clinical features, diagnosis and differential diagnosis, treatment and prognosis of GFAP-A by analyzing relevant studies at home and abroad, in order to deepen the understanding of this disease and provide references for its clinical diagnosis and treatment.
