Autoimmune glial fibrillary acidic protein astrocytopathy - The benefit of early diagnosis and initiation of therapy: A case report.

We describe a teenage lady who presented with headache, fever, neck stiffness, torticollis, progressive disturbance of consciousness as well as hemodynamic instability and subsequently developed quadriparesis. CSF revealed lymphocytic pleocytosis, elevated protein and low glucose with negative microbial studies. Her clinical course was complicated by acute encephalopathy requiring ITU admission and intubation. Full neuroaxis MRI revealed focal cerebritis with perivenular radial enhancement, and a non-enhancing longitudinally extensive myelitis. Positive Autoimmune glial fibrillary acidic protein (GFAP) IgG was subsequently identified in the CSF via immunofluorescence (IF) assay. CT thorax, abdomen and pelvis was negative for neoplasms. She was initially treated with pulsed intravenous corticosteroids with minimal improvement; however, following plasmapheresis she made a complete recovery of her neurological function. Long-term treatment consisted of oral prednisolone taper and azathioprine to lower the risk of early relapse. GFAP astrocytopathy is a novel autoimmune disease of the central nervous system. It is especially easy to misdiagnose and as such it is imperative to recognize the clinical spectrum of this disorder as early detection and treatment should improve prognosis.