Partially Reversible FLAIR Hyperintensity Along the Brainstem Surface in Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

A 41-year-old woman presented with headache, fever, irritability, and confusion. She developed a coma after admission. MRI of the brain revealed periventricular and deep white matter lesions. Fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging hyperintensity along the brainstem surface was observed (Figure 1), considered a rare but characteristic finding in leptomeningeal carcinomatosis from lung cancer.1,2 However, serum tumor markers, CSF cytopathology, contrast-enhanced high-resolution chest CT, and whole-body PET were negative. Antiglial fibrillary acidic protein antibodies (cell-based assay) were positive in serum and CSF. After receiving intravenous methylprednisolone and immunoglobulin, the symptoms improved significantly. Repeated MRI of the brain showed partial resolution of the lesions (Figure 2). The lesions were possibly induced by immune-mediated intramyelinic edema.