Nervous System Disease

Clinical analysis of autoimmune glial fibrillary acidic protein astrocytopathy in children

  • Chi HOU ,
  • Wenxiong CHEN ,
  • Yinting LIAO ,
  • Wenxiao WU ,
  • Yang TIAN ,
  • Haixia ZHU ,
  • Bingwei PENG ,
  • Yiru ZENG ,
  • Wenlin WU ,
  • Zongzong CHEN ,
  • Xiaojing LI
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  • Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou 510120, Guangdong, China

Received date: 2023-01-04

  Online published: 2023-09-05

Abstract

Objective To investigate the clinical characteristics, treatment and prognosis of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in children. Methods The clinical data of children diagnosed with GFAP-A from June 2018 to July 2022 were retrospectively analyzed. Results Five patients were diagnosed with GFAP-A, including 3 boys and 2 girls, and the onset age was 11.0 (5.0-13.5) years. The commonest initial symptom was fever (4 cases), and the commonest neurological symptoms and signs were headache (5 cases) and neck rigidity with positive Kerning sign (5 cases). Cerebrospinal fluid examination (CSF) in acute stage showed elevated white blood cell count and protein level in 4 cases and protein-cell separation in 1 case. The median CSF GFAP antibody titer was 1:10 (1:10-1:100), and 1 patient had combined N-methyl-D-aspartate receptor (NMDAR) antibody. The commonest magnetic resonance imaging (MRI) findings were cerebral leptomeningeal enhancement (4 cases). In acute phase, all children received first-line immunotherapy (high-dose intravenous methylprednisolone combined with intravenous immunoglobulin), and 4 children had a good response. One patient with positive CSF NMDAR antibody had no response to first-line treatment, but improved after rituximab treatment. During a follow-up of 8.0 (4.0-36.0) months, no neurological sequelae associated with the disease were detected. Conclusions The commonest symptoms and signs of GFAP-A in children are fever, headache and meningeal irritation, and leptomeningeal enhancement is the commonest MRI change. Most children have a good response to first-line immunotherapy and have a good prognosis during follow-up.

Cite this article

Chi HOU , Wenxiong CHEN , Yinting LIAO , Wenxiao WU , Yang TIAN , Haixia ZHU , Bingwei PENG , Yiru ZENG , Wenlin WU , Zongzong CHEN , Xiaojing LI . Clinical analysis of autoimmune glial fibrillary acidic protein astrocytopathy in children[J]. Journal of Clinical Pediatrics, 2023 , 41(9) : 656 -660 . DOI: 10.12372/jcp.2023.22e1735

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