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Autoimmune glial fibrillary acidic protein astrocytopathy in children: a retrospective study
Xiamei Zhuang1, Ke Jin2, Xiaoming Li1
1Department of Radiology, Hunan Children's Hospital, 86 Ziyuan Road, Yuhua District, Changsha, China.
Insights
Pediatric autoimmune GFAP astrocytopathy presents with encephalitis and myelitis. Clinical features and neuroimaging findings in children differ from prior adult studies, highlighting unique pediatric manifestations.
Area of Science:
- Neuroimmunology
- Pediatric Neurology
- Autoimmune Diseases
Background:
- Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an emerging neurological disorder.
- Understanding its presentation in children is crucial for diagnosis and management.
Purpose of the Study:
- To delineate the clinical characteristics of autoimmune GFAP astrocytopathy in pediatric patients.
- To compare pediatric findings with existing literature.
Main Methods:
- Retrospective analysis of clinical data from 11 pediatric patients diagnosed with autoimmune GFAP astrocytopathy.
- Review of clinical symptoms, cerebrospinal fluid (CSF) analysis, and neuroimaging findings (MRI).
Main Results:
- All patients exhibited encephalitis/meningoencephalitis/meningoencephalomyelitis, with or without myelitis.
- Common symptoms included fever, headache, and altered mental status. CSF analysis revealed elevated white blood cell counts and protein.
- Neuroimaging showed brain abnormalities in 90%, including characteristic lesions in the basal ganglia and white matter, and leptomeningeal enhancement.
Conclusions:
- Pediatric autoimmune GFAP astrocytopathy presents with distinct clinical and radiological features compared to adult cases.
- These findings underscore the need for specific diagnostic criteria and treatment strategies for children.
Objective:
To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.
Method:
Data from 11 pediatric patients with autoimmune GFAP astrocytopathy were retrospectively analyzed.
Results:
All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis. 45.4% of the patients had fever, 27.3% headaches, 18.2% dizziness, 18.2% drowsiness, and 18.2% mental disorders. Cerebrospinal fluid (CSF) was detected in all patients. The white blood cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, with the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions.
Conclusion:
The findings of pediatric patients with autoimmune GFAP astrocytopathy are different from previous reports.
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