EBV-associated autoimmune glial fibrillary acidic protein astrocytopathy with lumbosacral leptomeningeal enhancement: a case report and literature review.

BACKGROUND: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a rare inflammatory central nervous system autoimmune disease. Viral pathogens, most notably Epstein-Barr virus (EBV), represent a major disease trigger. Classic spinal magnetic resonance imaging (MRI) manifestations of GFAP-A are characterized by long-segment T2-weighted hyperintensities surrounding the central canal and patchy intramedullary enhancement, whereas lumbosacral leptomeningeal involvement is extremely rare. METHODS: We report a 58-year-old male patient with EBV-associated GFAP-A featuring isolated lumbosacral leptomeningeal enhancement. We consequently conducted a literature review restricted to EBV-associated GFAP-A cases to characterize their clinical manifestations, laboratory examinations, therapeutic regimens, and prognoses. RESULTS: The patient presented with fever, low back pain, progressive limb numbness and weakness, and urinary retention. Serum and cerebrospinal fluid (CSF) GFAP-immunoglobulin G (IgG) tests were positive. Concurrently, metagenomic next-generation sequencing detected EBV reads in the CSF, whereas plasma EBV DNA was negative. Contrast-enhanced lumbar MRI demonstrated smooth linear leptomeningeal enhancement along the spinal cord. The patient showed substantial neurological recovery after receiving methylprednisolone pulse therapy, antiviral agents, and rituximab. A literature analysis of 24 cases of EBV-associated GFAP-A indicated a predominance among young and middle-aged males. Prominent clinical features included fever, urinary retention, headache, limb weakness, and tremor, with CSF GFAP-IgG positivity reaching 87.5%. Glucocorticoids, intravenous immunoglobulin, and antiviral agents constituted the first-line therapies. CONCLUSIONS: EBV-associated GFAP-A can manifest as isolated lumbosacral leptomeningeal enhancement in the absence of classic intracranial radial perivascular enhancement. Combined antiviral and immunotherapy demonstrates favorable short-term efficacy, whereas long-term, B-cell-targeted maintenance therapy may reduce the risk of relapse.