Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

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

Hiroshi Adachi, M.D.1), Yuma Shiomi, M.D.1), Akio Kimura, M.D.2), Takayoshi Shimohata, M.D.2), Yukihiro Yoneda, M.D.1) and Yasufumi Kageyama, M.D.1)

1) Department of Neurology, Hyogo Prefectural Amagasaki General Medical Center
2) Department of Neurology, Gifu University Graduate School of Medicine

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.
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(CLINICA NEUROL, 61: 401|404, 2021)
key words: glial fibrillary acidic protein (GFAP), meningo-encephalitis, autoimmune, astrocytopathy, hiccups

(Received: 18-Dec-20)