Failure of IVIG in steroid-responsive autoimmune glial fibrillary acidic protein astrocytopathy: A case report.

ABSTRACT

OBJECTIVE: To advocate for high-dose steroids, not intravenous immunoglobulins (IVIG), as first-line treatment for Anti-glial fibrillary acidic protein (GFAP) associated meningoencephalomyelitis. BACKGROUND: A novel IgG antibody against GFAP was associated with relapsing autoimmune meningoencephalomyelitis. DESIGN/METHODS: Here, we present an investigational case report to highlight continuing challenges in diagnosing and managing Anti-GFAP associated meningoencephalomyelitis. RESULTS: Our 45-year-old Asian female presented to the emergency department with an acute onset low-grade fever and back pain associated with headaches, intermittent confusion, vision changes, and hand tremors. A review of systems identified no inciting factors. Past medical history was significant only for chronic Hepatitis B without significant viral load. Neurological exam was significant for decreased visual acuity, high-frequency hand tremor, and gait imbalance. Serum labs were within normal limits. Video electroencephalogram captured tremors without electrographical correlates. Cerebrospinal fluid 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 were negative. Patient continued to decline clinically, working diagnosis was possible paraneoplastic syndrome with pending laboratory results. She was given a five-day course of intravenous immunoglobulin as a therapeutic trial,hh however, her symptoms did not improve. A broader investigation with repeat lumbar puncture, imaging and serum laboratory failed to provide any additional information. She was treated symptomatically with minimal benefit. A trial of steroids was given with clinical improvement and continued stability. Paraneoplastic panels returned positive for high levels of Anti-GFAP antibody for confirmation of diagnosis. CONCLUSIONS: Autoimmune GFAP astrocytopathy is a rare cause of meningoencephalomyelitis 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.