Case Report: A Case of Epstein Barr Virus-related Glial Fibrillary Acidic Protein Astrocytopathy Refractory to Conventional Immunotherapy

Abstract

Background Epstein-Barr virus (EBV) infection has been associated with the development of glial fibrillary acidic protein astrocytopathy (GFAP-A), with most cases responding favorably to steroids and/or intravenous immunoglobulin (IVIG). We report a case of EBV-related GFAP-A exhibiting a poor response to conventional immunotherapy. Case presentation A 62-year-old Chinese male presented with dysuria, fever, and headache, rapidly progressing to paraparesis. Initial investigations indicated pulmonary infection and viral encephalitis. Despite intensive care and antiviral treatment, his condition deteriorated, necessitating tracheostomy and intubation. Upon transfer to a tertiary neurology center, he had reduced tone, power, and areflexia in the lower limbs, and a loss of sensation below the T6 level. Cerebrospinal fluid (CSF) analysis revealed markedly elevated cell counts and protein levels. MRI showed leptomeningeal enhancement in the left frontal lobe, cervical and thoracic spines. EBV DNA and GFAP-IgG were detected in the CSF, suggesting a diagnosis of EBV-related GFAP-A. Treatment with IV acyclovir, high-dose steroids, and IVIG was administered with no clinical improvement. Conclusions This report presents a case of EBV-related GFAP-A refractory to treatments with steroids and IVIG. It suggests that higher levels of CSF protein and cell count may predict worse outcomes and a poorer prognosis.