Case of autoimmune glial fibrillary acidic protein astrocytopathy associated with Epstein–Barr virus reactivation

Abstract

AbstractBackgroundIn autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, brain magnetic resonance imaging (MRI) shows no abnormal findings in many cases, which attributes to misdiagnoses. To our knowledge, magnetic resonance spectroscopy (MRS) findings have never been reported for autoimmune GFAP astrocytopathy.Case presentationA 17‐year‐old girl presented with impaired consciousness and delirium; her cerebrospinal fluid was positive for Epstein–Barr virus. Reduced diffusion in the splenium of the corpus callosum on brain MRI led to a tentative diagnosis of mild encephalitis/encephalopathy with a reversible splenial lesion. On repeatedly carrying out brain MRI, new lesions characteristic of autoimmune GFAP astrocytopathy were identified. MRS showed a low concentration of myo‐inositol, an astrocyte‐specific marker. Intravenous methylprednisolone gradually resolved the patient’s symptoms, while the MRS findings were normalized as her symptoms resolved. Anti‐GFAP antibody was positive in the patient’s cerebrospinal fluid.ConclusionsThe MRI findings characteristic of autoimmune GFAP astrocytopathy might not be present at the illness onset when triggered by Epstein–Barr virus reactivation. Our results suggest that MRS might show astrocytic dysfunction and be useful in evaluating treatment progress.