{
  "abstract": "A previously well 63-year-old male presented with acute encephalopathy and fevers after being seen well one week earlier. Cerebrospinal fluid (CSF) analysis demonstrated markedly elevated protein concentration and mononuclear pleocytosis. Other than a newly identified active hepatitis C infection, comprehensive infective screening was negative. Magnetic resonance imaging (MRI) demonstrated diffuse leptomeningeal enhancement, several punctate foci of restricted diffusion and microhaemorrhages in both cerebral hemispheres. Further extensive investigations did not identify an inflammatory, malignant or paraneoplastic aetiology. The patient subsequently underwent a brain biopsy. Histopathology demonstrated transmural granulomatous vasculitis predominantly involving the leptomeningeal vessels. Immunohistochemistry confirmed the presence of amyloid-beta, consistent with a diagnosis of amyloid-beta related angiitis (ABRA). The patient had significant neurological improvement after treatment with corticosteroids and was able to be discharged home. Repeat MRI demonstrated resolution of the leptomeningeal enhancement.Discussion This case highlights the need for a broad differential diagnosis when approaching a patient with acute encephalopathy. ABRA more commonly presents with subacute cognitive dysfunction, 1 whereas this patient presented with acute symptoms over days. The confounding bystanders of fevers and active hepatitis C infection led to infective causes being prioritised in the initial work-up. The MRI findings were difficult to interpret given it is uncharacteristic for ABRA to lack the T2/FLAIR subcortical white matter changes. While clinical-radiologic criteria have been suggested,2 this case demonstrates the importance of proceeding with brain biopsy for pathological diagnosis. While rare, ABRA should be considered as a differential diagnosis as it is a treatable cause of progressive cognitive dysfunction.References Danve A, Grafe M, Deodhar A. Amyloid beta-related angiitis—a case report and comprehensive review of literature of 94 cases. InSeminars in arthritis and rheumatism 2014 Aug 1 (Vol. 44, No. 1, pp. 86–92). WB Saunders.Auriel E, Charidimou A, Gurol ME, Ni J, Van Etten ES, Martinez-Ramirez S, Boulouis G, Piazza F, DiFrancesco JC, Frosch MP, Pontes-Neto OM. Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation. JAMA Neurology 2016 Feb 1;73(2):197–202.",
  "authors": [
    {
      "affiliations": [
        "Department of General Medicine, Princess Alexandra Hospital, Brisbane, QLD, Australia",
        "School of Medicine and Dentistry, Griffith University, Gold Coast, QLD, Australia"
      ],
      "name": "Alexandra Radke"
    },
    {
      "affiliations": [
        "Department of Neurology, Princess Alexandra Hospital, Brisbane, QLD, Australia"
      ],
      "name": "Jane McAuliffe"
    },
    {
      "affiliations": [
        "Department of Neurology, Princess Alexandra Hospital, Brisbane, QLD, Australia"
      ],
      "name": "Tamara Canento Sanchez"
    },
    {
      "affiliations": [
        "Department of Neurology, Princess Alexandra Hospital, Brisbane, QLD, Australia"
      ],
      "name": "Laurie McLaughlin"
    },
    {
      "affiliations": [
        "Department of Neurology, Princess Alexandra Hospital, Brisbane, QLD, Australia"
      ],
      "name": "Lori Mackay"
    },
    {
      "affiliations": [
        "Anatomical Pathology, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia"
      ],
      "name": "Thomas Robertson"
    },
    {
      "affiliations": [
        "Department of General Medicine, Princess Alexandra Hospital, Brisbane, QLD, Australia"
      ],
      "name": "Rajesh Gupta"
    }
  ],
  "title": "3609 A case of amyloid-beta related angiitis (ABRA) presenting with acute encephalopathy",
  "uid": "8794a575-8600-5e1a-94d5-48460c6c731e"
}