Clinical features of autoimmune glial fibrillary acidic protein astrocytopathy in children: an analysis of 34 cases.
10.7499/j.issn.1008-8830.2208105
- Author:
Wen-Wen WANG
1
;
Mei LI
1
Author Information
1. Department of Neurology, Children's Hospital of Chongqing Medical University/National Clinical Research Center for Child Health and Disorders/Ministry of Education Key Laboratory of Child Development and Disorders/Chongqing Key Laboratory of Pedatrics, Chongqing 400014, China.
- Publication Type:Journal Article
- Keywords:
Autoimmune glial fibrillary acidic protein astrocytopathy;
Child;
Encephalitis;
Glial fibrillary acidic protein
- MeSH:
Adolescent;
Child;
Child, Preschool;
Humans;
Infant;
Astrocytes/metabolism*;
Autoantibodies/metabolism*;
Glial Fibrillary Acidic Protein/metabolism*;
Prognosis;
Retrospective Studies;
Autoimmune Diseases/metabolism*
- From:
Chinese Journal of Contemporary Pediatrics
2023;25(1):67-72
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVES:To study the clinical features of children with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A).
METHODS:A retrospective analysis was performed on the medical data of 34 children with GFAP-A who attended the Department of Neurology, Children's Hospital of Chongqing Medical University, from January 2020 to February 2022. The medical data included clinical manifestations, cerebrospinal fluid features, imaging examination results, treatment, and prognosis.
RESULTS:The median age of onset was 8.4 (range 1.9-14.9) years for the 34 children with GFAP-A. The main clinical manifestations included headache (50%, 17/34), fever (47%, 16/34), visual impairment (47%, 16/34), and disturbance of consciousness (44%, 15/34). Abnormal cerebrospinal fluid results were observed in 19 children (56%, 19/34), among whom 8 children had positive autoantibody. The children with overlap syndrome had significantly higher recurrence rate and rate of use of immunosuppressant than those without overlap syndrome (P<0.05). About 77% (24/31) of the children had good response to immunotherapy, and only 1 child had poor prognosis.
CONCLUSIONS:Children with GFAP-A often have non-specific clinical symptoms and show good response to immunotherapy. Children with overlap syndrome have a high recurrence rate, and early application of immunosuppressants may help to prevent recurrence and alleviate symptoms.
