ReviewJournal of neurology2026
Clinical characteristics of autoimmune GFAP astrocytopathy with concomitant Epstein-Barr virus positivity: a case series of 12 patients and literature review.
Review in Journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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6 authors.
Funding
Abstract
backgroundAutoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel autoimmune central nervous system (CNS) disorder. Epstein-Barr virus (EBV) has been implicated in various neurological diseases, yet GFAP-A with concomitant Epstein-Barr virus positivity is poorly understood.
methodsWe retrospectively analyzed 12 patients with concomitant EBV and GFAP positivity admitted between January 2022 and September 2025, and performed a literature review. Clinical, imaging, laboratory features, treatments, and outcomes were evaluated.
resultsIn our cohort, the median age at onset was 52.5 years (58% male). The predominant phenotype was meningo-encephalomyelitis (33%), with headache (100%) and fever (92%) as common symptoms. Peripheral nervous system involvement was noted in 16%. CSF analysis showed pleocytosis (100%) and elevated protein levels. MRI revealed T2/FLAIR hyperintensities in 67%, mainly in white matter and brainstem, and spinal lesions in 73%, including longitudinally extensive transverse myelitis (27%). All received antiviral and immunotherapy, with most showing improvement after intravenous methylprednisolone (± IVIG). At a median 32-month follow-up, only one relapsed, two died, and 80% of survivors achieved good outcomes (mRS < 3). Literature review (32 cases) showed similar features and immunotherapy responsiveness. Overall recurrence and mortality rates were both 7%.
conclusionsIn this small series, cases with concomitant EBV and GFAP positivity showed overlapping clinical features with classical GFAP-A and generally favorable responses to immunotherapy. Early recognition and CSF-based GFAP antibody testing may help improve patient outcomes; however, further studies are needed to confirm these findings.
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