Teaching NeuroImages: Autoimmune glial fibrillary acidic protein meningoencephalomyelitis

A 73-year-old woman with myelodysplastic syndrome presented with 2 months of decline in cognition and mobility, accompanied by headache and weight loss. Examination revealed a somnolent woman with symmetric cogwheel rigidity, bradykinesia, arm myoclonus, and hyperreflexia. Brain MRI showed leptomeningeal, linear/stippled parenchymal, and ependymal enhancement (figure 1). Spine MRI demonstrated nonenhancing cervicothoracic cord signal change and circumferential enhancement of conus medullaris with central canal enhancement (figure 2). CSF was inflammatory with glial fibrillary acidic protein (GFAP)–immunoglobulin G (IgG). The patient responded promptly to IV methylprednisolone. GFAP-IgG identifies a severe but highly corticosteroid-responsive autoimmune meningoencephalomyelitis with distinctive MRI findings.^1,2

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