THUR 159 Radiological findings in two patients with autoimmune GFAP astrocytopathy

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Abstract

Background

Autoimmune 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 Methods

We 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.

Results

The 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.

Conclusion

Patients 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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