Presentation
The patient has presented with progressive weakness, numbness, confusion, dysphagia, and urinary retention over the past 3–4 weeks.
Patient Data
Contrast-enhanced MRI of the brain displays punctate and linear perivascular enhancement radiating from the ventricles into the adjacent white matter. There is also leptomeningeal enhancement, most pronounced at the inferior brainstem and upper cervical spine. Radiating linear and leptomeningeal enhancement is complemented by a subtle signal abnormality on FLAIR. There are no corresponding abnormalities on DWI/ADC or GRE to suggest complications from infarction or hemorrhage.
Evaluation of the spine cord shows diffuse cord hyperintensity on T2 and STIR with associated cord expansion. Findings are most pronounced in the cervical spine, though they involve the entire spinal cord. Following contrast administration, there is diffuse patchy cord enhancement as well as leptomeningeal enhancement with relative sparing of the cauda equina nerve roots.
Case Discussion
This case demonstrates the typical appearance of autoimmune GFAP astrocytopathy with radiating perivascular enhancement and marked longitudinally extensive transverse myelitis. On CSF analysis, there was a marked elevation in lymphocytes and protein with normal glucose; for autoimmune encephalitis, GFAP titer was positive at 1:512.
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