AUTHOR=Shosha Eslam , Connolly Colleen , Budhram Adrian 

TITLE=Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy

JOURNAL=Frontiers in Neurology

VOLUME=Volume 15 - 2024

YEAR=2024

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1366263

DOI=10.3389/fneur.2024.1366263

ISSN=1664-2295

ABSTRACT=Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); characteristic of GFAP astrocytopathy is optic neuritis, meningoencephalo-myelitis. We report a 55-year-old male, otherwise healthy, presented with isolated headache for three months, without other features of meningo-encephalitis or myelitis. His neurological examination and fundoscopy were unremarkable. Gadolinium-enhanced brain MRI demonstrated increased T2-hyperintensity within the right sublenticular basal ganglia, with additional leptomeningeal enhancement along the bilateral perisylvian regions and mesial temporal lobes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, elevated protein, matching oligoclonal bands, and negative infectious and cytology workup. Cell-based assays for anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, autoimmune encephalitis panel, and vasculitis workup were all negative, except for CSF positivity for glial fibrillary acidic protein (GFAP) α antibody.Oncological screening was unremarkable, including CT chest, abdomen, pelvis, and scrotal US. Immunotherapy with intravenous high-dose steroids for five days and subsequent single four weekly doses resulted in resolution of both clinical and radiographic picture, with maintained status 24 months after onset. This case identifies isolated headache and basal ganglia, mesial temporal lobes involvement as a rare presentation of autoimmune GFAP astrocytopathy.