title: CD4(+) T cells predominate in cerebrospinal fluid and leptomeningeal and parenchymal infiltrates in cerebral amyloid β-related angiitis.
abstract: BACKGROUND
  In amyloid (Aβ)-related angiitis (ABRA)of the central nervous system (CNS), cerebral amyloid angiopathy occurs in association with primary vasculitis of small- and medium-sized leptomeningeal and cortical arteries. It has been suggested that ABRA is triggered by vascular deposition of A followed by an Aβ-directed (auto)immune response.
  
  
  OBJECTIVE
  To provide a detailed description of the cellular composition of the inflammatory infiltrates in the cerebrospinal fluid (CSF) and CNS and their response to immunotherapy in a typical case of ABRA.
  
  
  METHODS
  Report of a single case.
  
  
  METHODS
  Neurologic referral center.
  
  
  METHODS
  67-year-old white woman.
  
  
  METHODS
  Neurologic examination,magnetic resonance imaging, lumbar puncture, flow cytometry,leptomeningeal biopsy, and histopathologic analysis.
  
  
  RESULTS
  In a typical case of ABRA, we demonstrate for the first time the presence of a vast majority of partially activated CD4(+) T cells in CSF and leptomeningeal and parenchymal (peri)vascular infiltrates, which were frequently found in close proximity to major histocompatibility complex (MHC) class II-expressing microglia, epithelioid macrophages, and multinucleated giant cells containing intracellular deposits of Aβ.
  
  
  CONCLUSIONS
  Our findings support the notion of adaptive Aß-directed autoimmunity as the underlying pathogenic mechanism in ABRA.
authors.affiliation: Departments of Neurology, Inflammatory Disorders of the Nervous System, and Neurooncology, University of Münster, Albert-Schweitzer-Campus 1, Münster, 48149 Germany. nico.melzer@ukmuenster.de
authors.forename: Nico
authors.initials: N
authors.lastname: Melzer
authors.forename: Anja
authors.initials: A
authors.lastname: Harder
authors.forename: Catharina C
authors.initials: CC
authors.lastname: Gross
authors.forename: Johannes
authors.initials: J
authors.lastname: Wölfer
authors.forename: Walter
authors.initials: W
authors.lastname: Stummer
authors.forename: Thomas
authors.initials: T
authors.lastname: Niederstadt
authors.forename: Sven G
authors.initials: SG
authors.lastname: Meuth
authors.forename: Martin
authors.initials: M
authors.lastname: Marziniak
authors.forename: Oliver Martin
authors.initials: OM
authors.lastname: Grauer
authors.forename: Heinz
authors.initials: H
authors.lastname: Wiendl
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000134:cerebrospinal fluid*; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology*; D016657:Cerebral Amyloid Angiopathy / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007167:Immunotherapy; D008279:Magnetic Resonance Imaging; D020293:Vasculitis, Central Nervous System / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology
chemical_list: D016229:Amyloid beta-Peptides