Clinical features of patients with autoimmune glial fibrillary acidic protein astrocytopathy
10.19845/j.cnki.zfysjjbzz.2021.0134
- VernacularTitle: 胶质纤维酸性蛋白星型细胞病的病例特点
- Author:
Quan YUAN
1
;
Shuhui TAN
1
Author Information
1. Innovation Center for Neurological Disorders,Department of Neurology，Xuanwu Hospital,Capital Medical University，Beijing 100053,China
- Publication Type:Journal Article
- Keywords:
GFAP astrocytosis;
Cerebrospinal fluid;
Electroencephalogram;
Treatment
- From:
Journal of Apoplexy and Nervous Diseases
2021;38(6):501-504
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical features through analyzing the clinical data of 5 patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy and reviewing the literatures.Methods We retrospectively analyzed the general data,clinical symptoms,cerebrospinal fluid results,electroencephalogram results,imaging data and treatment results of 5 patients with GFAP astrocytopathy at our hospital and reviewed the literatures.Result The average age of onset was 57 in five patients.The main clinical symptoms were limb fatigue,memory loss,increased sleep,urine and stool disorders,fever and so on,and 1 patient was accompanied by epileptic seizure in addition to the above manifestations.Cerebrospinal fluid examination showed that protein and cell number were increased in different degrees,4 patients showed positive oligoclonal bands and specific oligoclonal bands in cerebrospinal fluid,and 24 hours intrathecal synthesis rate increased in different degrees.The electroencephalogram showed varying degrees of slow waves.Abnormal signals in the temporal lobe,lateral ventricle,basal ganglia and corpus callosum were observed by craniocerebral MRI.Abnormal signals in the medulla oblongata,cervical medulla and thoracic medulla were observed in 3 patients.All the 5 patients received hormone therapy,and their symptoms were improved to varying degrees.Conclusions The clinical symptoms of patients with GFAP astrocytosis were mainly encephalopathy,meningitis and encephalomyelitis,and the cerebrospinal fluid had different degrees of immune-mediated inflammatory reaction.Imaging examination showed that the brain,meninges,spinal cord and optic nerve were involved,and most of patients respond well to hormones.
- Full text:2024072921584735725Clinical features of patients with autoimmune glial fibrillary acidic protein astrocytopathy.pdf
