EBV-associated autoimmune glial fibrillary acidic protein astrocytopathy with lumbosacral leptomeningeal enhancement: a case report and literature review.
Yueying Liu, Huimin Chen, Miaoxian Xie et al.
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Case in brief
A 58-year-old male presented with fever, low back pain, progressive limb numbness, weakness, and urinary retention. Diagnostic tests revealed positive GFAP-IgG in serum and cerebrospinal fluid, and metagenomic sequencing identified Epstein-Barr virus in the CSF. MRI showed isolated lumbosacral leptomeningeal enhancement. The patient improved significantly after treatment with methylprednisolone, antiviral agents, and rituximab.
What made this case unusual
The case is notable for the isolated lumbosacral leptomeningeal enhancement, which is extremely rare in autoimmune glial fibrillary acidic protein astrocytopathy.
Diagnostic / clinical pearl
Clinicians should consider EBV-associated GFAP-A in patients presenting with neurological symptoms and isolated lumbosacral leptomeningeal enhancement, even in the absence of classic MRI findings.
Why it matters
This case highlights the need for awareness of atypical presentations of GFAP-A, which can lead to timely diagnosis and treatment, improving patient outcomes.