Glial Fibrillary Acidic Protein (GFAP) astrocytopathy is an immune mediated inflammatory disorder, occasionally associated with malignancy, with manifestations of meningitis, optic neuritis, encephalitis or myelitis.

A 44-year-old woman presented with progressive weakness and numbness in the legs with no clear sensory level, areflexia in the legs, urinary retention and constipation over two weeks. These symptoms were preceded by a prodrome of fever and gastroenteritis.

MRI of the spine was notable for a T2 hyper-intense lesion at C4 level with enhancement of the cauda equina. CSF analysis showed lymphocytic pleocytosis (114 cells/mm³) with elevated protein (68 mg/dL) and normal glucose (65 mg/dL) levels. The patient received solumedrol 1gm daily for five days with intravenous immunoglobulins. However, her weakness progressed to involve her arms. Repeat MRIs of spine in one week and three weeks showed enhancing lesions in the T1-T8 and C4-C6 levels, respectively. MRI brain showed enhancing lesion in the dorsal medulla extending to the cervicomedullary junction. CSF showed three oligoclonal bands with the meningitis panel, with negative Lyme antibody, aquaporin-4 antibody, CSF culture and cytology. CSF autoimmune panel revealed antibodies to GFAP. There was no evidence of malignancy in CT-chest/abdomen/pelvis. She received two cycles of plasmapheresis with gradual improvement of symptoms over six weeks. She was nearly back to baseline in the follow up visit in four months and there was improvement in the spinal cord lesions on MRI with no post-gadolinium enhancement.

This case is interesting as the patient with autoimmune GFAP astrocytopathy did not have characteristic contrast enhancement in the brain MRI. She also did not have improvement of symptoms with steroids and required plasma exchange. Autoimmune GFAP astrocytopathy should always be considered in the differential diagnosis while evaluating patients with idiopathic LETM.