{
  "abstract": "Introduction Glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune inflammatory central nervous system disease that is typically steroid-responsive. The most common phenotypical syndromes are meningitis, encephalitis and myelitis. The hallmark MRI feature of linear perivascular enhancement extending radially outward from the ventricles is present in approximately 50% of cases.Case A previously well 61-year-old male presented with a 3-week history of fevers, bilateral leg oedema and urinary retention. Over the preceding 2-months he experienced fatigue, night sweats and weight loss. In the first 72-hours of admission, the patient’s neurological function rapidly deteriorated with progressive encephalopathy and quadriplegia, requiring intubation and sedation. Extensive serum investigations were unremarkable. Lumbar puncture yielded a high opening pressure (32cmH2O), elevated protein (1.73g/L), reduced glucose (1.1mmol/L), elevated mononuclear cells (242x10^6/L) and negative cultures/PCRs. MRI brain demonstrated bilateral asymmetrical mesial temporal lobe T2 hyperintensity and swelling as well as striking leptomeningeal enhancement around the brainstem and multiple cranial nerves. MRI spine demonstrated extensive patchy anterior/central grey T2 hyperintensity and leptomeningeal enhancement. A brain biopsy suggested autoimmune encephalitis. CSF autoantibodies to GFAP were eventually confirmed by both immunofluorescence and antigen-specific cell-based assay. After minimal improvement with empirical IVIG and plasma exchange he was treated with high dose steroids followed by cyclophosphamide, with stabilisation followed by slow neurological improvement. Despite this, he remains significantly disabled.Conclusion This case illustrates a rare presentation of GFAP astrocytopathy involving rapid neurological deterioration with poor recovery despite immunotherapy and highlights the diagnostic difficulty in cases presenting without well-recognised characteristic MRI features.",
  "authors": [
    {
      "affiliations": [
        "Department of Neurology, St George Hospital, Sydney, NSW, Australia"
      ],
      "name": "Spiro Menounos"
    },
    {
      "affiliations": [
        "Department of Neurology, St George Hospital, Sydney, NSW, Australia"
      ],
      "name": "Mahtab Ghadiri"
    }
  ],
  "title": "3464 Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy with rapid neurological deterioration and atypical MRI findings",
  "uid": "80b92ca2-cb70-5ebf-bbdb-a7052637b245"
}