Abstract
Objectives To highlight a rare mimic of an infective meningomyeloencephalitis.
Methods A case report.
Results A 44-year-old male presented with intractable fevers and headache. There is no significant past medical or drug history or regular medication use. He reported nausea, fevers and malaise over 2 days. Severe bifrontal headaches persisted for 5 days, whilst urinary hesitancy and difficulty urinating coupled with progressive lower limb weakness developed over the proceeding days of admission. He remained febrile for multiple days despite regular antipyretic use and subsequently became confused and somnolent followed by developing hallucinations, acute urinary retention and increasing pyramidal lower limb signs. Investigations revealed a CRP of 4 with a normal urinalysis and normal CT Brain. CSF studies showed a raised protein of 1.94g/L and a lymphocytic predominant pleocytosis of 144 x109/L with normal cultures and negative pan-viral PCR multiplex. Broad anti-infective treatment was continued. MRI Brain and spine revealed intracranial and conus medullaris leptomeningeal enhancement. Standard anti-neuronal antibody and limbic encephalitis panel testing was negative. Due to persistent fever, lower limb weakness and neurogenic bladder a PET study confirmed on-going meningomyeloencephalitis despite broad anti-infective therapy. At further request, extended anti-neuronal antibody testing by immunopathology supported a diagnosis of glial fibrillary acidic protein (GFAP) astrocytopathy. Immunotherapy with IVIG, mycophenolate and initial pulse steroids with a slow oral taper were able to induce clinical remission after 4 months.
Conclusion GFAP astrocytopathy can mimic an infective meningomyeloencephalitis. Early broader immunologpathological consideration is key to diagnosis.