Clinical Features of Multicenter Autoimmune Glial Fibrillary Acidic Protein Astrocytosis
10.12376/j.issn.2097-0501.2022.02.007
- VernacularTitle:自身免疫性胶质纤维酸性蛋白星形胶质细胞病临床特征分析
- Author:
Xiujun ZHENG
1
;
Qi LIN
1
;
Yaqing SHU
2
;
Xiaobo SUN
2
;
Xiaonan ZHONG
2
;
Rui LI
2
;
Yanyu CHANG
2
;
Wei QIU
2
;
Yuge WANG
2
Author Information
1. Department of Neurology, Shantou Central Hospital, Shangtou 515031, China.
2. Department of Neurology, the Third Affiliated Hospital, SUN Yat-sen University, Guangzhou 510630, China.
- Publication Type:Journal Article
- Keywords:
autoimmune glial fibrillary acidic protein astrocytopathy;
anti-glial fibrillary acidic protein;
progressive loss of consciousness;
tubercular meningitis
- From:
JOURNAL OF RARE DISEASES
2022;1(2):137-141
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Methods We collected and analyzed the clinical and laboratory data and obtained the clinical characteristics of diagnosis and treatment from fifteen patients with positive GFAP antibody tested by cerebrospinal fluid and diagnosed autoimmune GFAP astrocytopathy by the multi-centers. Results The mean age of the first onset of autoimmune GFAP astrocytopathy was 39.73 years old (range 4-65 years), with no significant gender difference. In terms of clinical manifestations, we found the whole brain symptoms including abnormal mental behavior, disturbance of consciousness, epileptic attack accounting for more than 50, , meningitis accounting for 66.7%, myelitis (53.3%), limb tremor (53.3%), vision loss (33.3%); systemic symptoms including fever(100%) and fatigue(86.7%). 46.7% of patients were initially diagnosed with tuberculous meningoencephalitis and were treated with diagnostic antituberculous therapy. The MRI showed 46.7% of patients showed brain linear perivascular radial gadolinium enhancement in the white matter perpendicular to the ventricle. Conclusions Autoimmune GFAP astrocytopathy are acute or subacute dieases and the main clinical features include encephalitis, meningitis, myelitis and optic neuritis. They are likely to be misdiagnosed as tuberculous meningoencephalitis and can manifest progressive loss of consciousness in early phase, which is even life threatening.
