Background

A 50-year-old man with no prior medical history presented with three months of persistent daily headaches unresponsive to over-the-counter analgesics. He also developed asymmetric upper extremity action tremor and clumsiness, predominantly affecting the right hand and interfering with writing. In the preceding month, his wife noted personality changes, anxiety, and depression, and he reported a flu-like illness several weeks before symptom onset. Neurological examination showed reduced digit span, pyramidal signs with asymmetric hyperreflexia, mild lower-extremity spasticity, cerebellar ataxia, and postural/action tremor. Ophthalmologic examination revealed mild bilateral optic disc edema with preserved visual acuity.

Brain MRI demonstrated linear radial perivascular gadolinium enhancement in both cerebral hemispheres, and spinal MRI showed hazy thoracic cord T2 hyperintensity with subtle enhancement. Cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis (63 WBC/μL) and elevated protein (130 mg/dL) without oligoclonal bands. Extensive infectious, metabolic, and malignancy evaluations were negative. CSF testing was positive for GFAP-IgG, confirming autoimmune GFAP meningoencephalomyelitis. The patient improved with high-dose intravenous steroids followed by oral prednisone but experienced an early relapse after rapid tapering. Prolonged steroid therapy led to full recovery, and he remained relapse-free at three years of follow-up.

Learn how our autoimmune/paraneoplastic evaluation for encephalopathy uses CSF testing to confirm an autoimmune GFAP meningoencephalomyelitis diagnosis.