A case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy

A 46-year-old man developed acute meningo-encephalitis with confusion, headache, fever, intractable hiccups, dysuria, myoclonus/tremor, and ataxia. Analysis of cerebrospinal fluid (CSF) showed elevated levels of cell counts and protein. Brain MRI demonstrated multiple linear increased FLAIR signals in bilateral basal ganglia and corona radiata. Repeated MRI showed T2 hyperintensity areas in the lower brainstem, sparing the area postrema. Immunotherapy with intravenous high-dose steroid and subsequent oral steroid was successful, and the symptoms improved completely. Later MRI study showed a striking resolution. Glial fibrillary acidic protein (GFAP) α antibody was positive in the CSF, while anti-aquaporin-4 antibody, anti-myelin oligodendrocyte glycoprotein antibody, and N-methyl-D-aspartate receptor antibody were all negative. There were no relapses at final follow-up of 6 months after onset. Autoimmune GFAP astrocytopathy is not an uncommon immune-mediated inflammatory disorder in the central nervous system.