title: Amyloid β-related angiitis of the central nervous system: report of 3 cases.
abstract: OBJECTIVE
  Amyloid-β (Aβ) related angiitis (ABRA) is a recently described clinicopathological entity characterized by cerebrovascular Aβ deposition and arteritis. Cerebral Aβ deposition is commonly present in cerebal amyloid angiopathy (CAA) and Alzheimer's disease (AD) but is rarely associated with inflammatory infiltration of vessel walls. Our objective is to help clarify the clinical spectrum, radiographic findings, response to treatment, and outcomes of ABRA. The neuropathological relationship between ABRA, cerebral amyloid angiopathy, and Alzheimer's disease is discussed.
  
  
  METHODS
  We present three cases of ABRA managed at a tertiary care centre.
  
  
  RESULTS
  All three patients presented with seizures and cognitive dysfunction; one had multifocal neurologic findings. Brain biopsies revealed inflammatory arteritis with Aβ deposits in the vessel walls. All were treated with steroids and cyclophosphamide. Two had favorable outcomes and one stabilized but with severe residual neurologic disability.
  
  
  CONCLUSIONS
  ABRA is an unusual but likely under-recognized and potentially treatable disorder. As in other reported cases, our findings suggest that many patients respond favorably to immunosuppressive therapy. We believe that all biopsy specimens consistent with primary angiitis of the central nervous system (CNS) should be further examined for vascular Aβ deposition.
authors.affiliation: Dalhousie University, Halifax, Nova Scotia, Canada QEII Health Sciences Centre, Halifax, Nova Scotia, Canada.
authors.forename: Heather
authors.initials: H
authors.lastname: Rigby
authors.forename: Alexander
authors.initials: A
authors.lastname: Easton
authors.forename: Virender
authors.initials: V
authors.lastname: Bhan
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000378:metabolism*; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D017252:CD3 Complex / Q000378:metabolism; D002490:Central Nervous System / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D014657:Vasculitis / Q000473:pathology*; D016875:tau Proteins / Q000378:metabolism; D000097382:CD68 Molecule
chemical_list: D016229:Amyloid beta-Peptides; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D017252:CD3 Complex; D016875:tau Proteins; C067980:CD68 antigen, human; D000097382:CD68 Molecule