THUR 159 Radiological findings in two patients with autoimmune GFAP astrocytopathy


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Abstract

BackgroundAutoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or subacute onset of cognitive impairment, seizures, encephalopathy, meningeal symptoms to more complex forms of encephalomyelitis.Materials and MethodsWe present a radiological review of two cases which were recently diagnosed in our institution. They presented with fever, meningoencephalitis and bilateral papilloedema. CSF antibody analysis (GFAPα-IgG) from Mayo Clinic confirmed the diagnosis of GFAP in both cases.ResultsThe typical radiological findings in both of the cases were a radial pattern of enhancement in brain and longitudinally extensive myelitic lesions in the spinal cord. The first patient improved with immunosupression treatment. The second patient had a significantly more severe clinical presentation with drug-refractory progression, who later died.ConclusionPatients presenting with subacute onset of cognitive impairment, meningoencephalomyelitis and papilloedema should raise the suspicion of autoimmune GFAP astrocytopathy. Though it is a relatively new disease entity, the radial pattern of enhancement and long spinal cord lesions on imaging are striking and CSF and serum antibodies are highly specific.

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