To advocate for high-dose steroids, not IVIG, as first-line treatment for Anti-GFAP associated meningoencephalomyelitis.

Astrocytic glial fibrillary acidic protein (GFAP) is being explored as a biomarker for ischemic stroke and potential prognostic marker for stroke recovery. More recently, a novel IgG antibody against GFAP was associated with relapsing autoimmune meningoencephalomyelitis.

Here, we present an investigational case report to highlight continuing challenges in diagnosing and managing Anti-GFAP associated meningoencephalomyelitis.

Our 45-year-old Asian female presented to ED with acute onset of low-grade fever and back pain associated with headaches, intermittent confusion, vision changes and hand tremors. ROS identified no inciting factors. PMH was significant only for chronic Hepatitis B without significant viral load. Neurological exam was significant only for decreased visual acuity, high-frequency hand and gait imbalance. Serum labs were within normal limits. Video EEG captured tremors without electrographical correlates. CSF analysis revealed lymphocytic leukocytosis, elevated protein and reduced glucose. A wide range of infectious studies including bacterial, viral, and fungal cultures were negative. MRI brain and spine showed leptomeningeal enhancement. CT chest abdomen pelvis did not show any lesions. Differential diagnosis included paraneoplastic syndromes and she underwent 5-day course of IVIG. However, her symptoms did not improve. A broader investigation with several repeat LPs and numerous MRIs yielded similar findings as those described above. She was trialed on several medications for symptomatic management with minimal benefit. Eventually paraneoplastic panels returned positive for high levels of Anti-GFAP antibody. Patient was then started on steroids and is now demonstrating good recovery.

Autoimmune GFAP astrocytopathy is a rare cause of meningoencephalitis that remains difficult to diagnose despite emerging laboratory studies. Our case adds to the limited literature by proposing that high-dose steroids, not IVIG, should be the first-line treatment. Further investigations are underway to assess implications of this finding in disease pathophysiology and management.