Glial fibrillary acidic protein astrocytopathy misdiagnosed as viral encephalitis： A case report and literature review
10.19845/j.cnki.zfysjjbzz.2026.0063
- VernacularTitle:Glial fibrillary acidic protein astrocytopathy misdiagnosed as viral encephalitis： A case report and literature review
- Author:
Weihong CHEN
1
;
Peiyuan LV
1
Author Information
1. Department of Neurology， Hebei General Hospital， Shijiazhuang 050051， Chin
- Publication Type:Journal Article
- Keywords:
Autoimmune glial fibrillary acidic protein astrocytopathy;
Glial fibrillary acidic protein;
Meningoencephalomyelitis
- From:
Journal of Apoplexy and Nervous Diseases
2026;43(4):369-372
- CountryChina
- Language:Chinese
-
Abstract:
A retrospective analysis was performed for the clinical data of a patient with GFAP astrocytopathy that was initially misdiagnosed as viral encephalitis， and the diagnostic and therapeutic experience of this disease was summarized through a literature review.The female patient， aged 39 years， presented with headache and fever after diarrhea for 1 day， with the main manifestations of distending pain in the brain， nausea， vomiting， low‑grade fever， and right‑sided ataxia. Physical examination showed papilledema in both eyes and neck stiffness； contrast-enhanced cranial MRI showed extensive leptomeningeal enhancement； cerebrospinal fluid （CSF） analysis showed a significant increase in opening pressure and lymphocytic‑predominant pleocytosis. The patient was tested negative for common infectious pathogens and autoimmune encephalitis antibodies， and therefore， she was once misdiagnosed with viral encephalitis. After unsuccessful antiviral and antibiotic therapies， CSF and serum samples were tested positive for GFAP‑IgG antibodies， and the patient was finally diagnosed with GFAP astrocytopathy. The patient symptoms were rapidly relieved after pulse therapy with methylprednisolone （1 g/day）， and imaging reexamination showed the disappearance of leptomeningeal enhancement， with significant improvements in both intracranial pressure and CSF parameters. The patient remained asymptomatic till the last follow‑up in July 2025， and negative conversion was observed for CSF GFAP antibodies.GFAP astrocytopathy lacks specific clinical manifestations， with leptomeningeal enhancement and lymphocytic predominance in CSF as crucial clues for early diagnosis， and detection of GFAP IgG antibodies in CSF and serum is the key to a confirmed diagnosis. This disease is highly sensitive to glucocorticoid therapy， and early identification followed by standardized treatment can help to achieve a good prognosis. This disease should be differentiated from common central nervous system infections such as viral encephalitis in clinical practice to avoid treatment delay due to missed diagnosis or misdiagnosis.
