Clinical, imaging, and immunological features of GFAP astrocytopathy: a systematic review with regional comparison

This systematic review aims to analyze the clinical, laboratory, and imaging characteristics of GFAP astrocytopathy, with attention to differences between Asian and non-Asian populations.

We searched PubMed, Embase, and Scopus from inception through June 2025 for case reports, case series, and observational studies involving adult patients with GFAP astrocytopathy. Fifteen studies, including 538 patients, were analyzed. Data on clinical presentations, MRI findings, and laboratory results were extracted and compared across regions.

We identified a total of 2,272 studies, of which 15 were included in our final analysis, comprising 538 patients. Our analysis identified three main clinical phenotypes: meningoencephalitis (164/371, 44.2%), meningoencephalomyelitis (94/371, 25.3%), and myelitis (26/371, 7.0%). The most common clinical symptoms were headache (186/470, 39.6%), abnormal movements (160/473, 33.8%), fever (172/538, 32.0%), and psychiatric symptoms (111/471, 23.6%). MRI findings frequently showed lesions in the periventricular (85/434, 19.6%) and subcortical regions (80/432, 18.5%). Perivascular and meningeal enhancements represented the most common radiological findings. Laboratory findings revealed positive GFAP antibodies in either serum or CSF. Co-existence of other antibodies in CSF and serum was also observed, most notably anti-AQP4, anti-MOG, and anti-NMDAR.

GFAP astrocytopathy is characterized by a wide range of clinical and radiological manifestations, most commonly presenting as meningoencephalitis, meningoencephalomyelitis, or myelitis, with meningoencephalitis being the most prevalent clinical syndrome observed in this study. Commonly reported symptoms include headache, fever, abnormal movements, and psychiatric symptoms. The disease exhibits diverse neurological and imaging features. Regional differences suggest that genetics, diagnostic practices, and cultural factors may influence symptom profiles. These findings highlight the need for standardized diagnostic criteria and further research across diverse populations.