title: Perfusion characteristics of Moyamoya disease: an anatomically and clinically oriented analysis and comparison.
abstract: OBJECTIVE
  Moyamoya disease (MMD) is characterized by unique angiographic features of collateralization. However, a detailed quantification as well as comparative analysis with cerebrovascular atherosclerotic disease (CAD) and healthy controls have not been performed to date.
  
  
  METHODS
  We reviewed 67 patients with MMD undergoing Xenon-enhanced computed tomography, as well as 108 patients with CAD and 5 controls. In addition to cortical, central, and infratentorial regions of interest, particular emphasis was put on regions that are typically involved in MMD (pericallosal territory, basal ganglia). Cerebral blood flow (CBF), cerebrovascular reserve capacity (CVRC), and hemodynamic stress distribution were calculated.
  
  
  RESULTS
  MMD is characterized by a significant, ubiquitous decrease in CVRC and a cortical but not pericallosal decrease in CBF when compared with controls. Baseline perfusion is maintained within the basal ganglia, and hemodynamic stress distribution confirmed a relative preservation of central regions of interest in MMD, indicative for its characteristic proximal collateralization pattern. In MMD and CAD, cortical and central CBF decreased significantly with age, whereas CVRC and hemodynamic stress distribution are relatively unaffected by age. No difference in CVRC of comparable regions of interest was seen between MMD and CAD, but stress distribution was significantly higher in MMD, illustrating the functionality of the characteristic rete mirabilis.
  
  
  CONCLUSIONS
  Our data provide quantitative support for a territory-specific perfusion pattern that is unique for MMD, including central preservation of CBF compared with controls and patients with CAD. This correlates well with its characteristic feature of proximal collateralization. CVRC and hemodynamic stress distribution seem to be more robust parameters than CBF alone for assessment of disease severity.
authors.affiliation: From the Department of Neurosurgery, Uniklinik RWTH Aachen, Aachen, Germany (G.A.S.); Department of Neurosurgery, Universitätsmedizin Mannheim, University of Heidelberg, Heidelberg, Germany (G.A.S., M.S.); Department of Neurosurgery, Charité Universitätsmedizin Berlin, Berlin, Germany (M.C., P.V.); Department of Neurosurgery, Dr. Horst Schmidt Klinik (HSK), Wiesbaden, Germany (P.H.); and Department of Neurosurgery, Medical University Innsbruck, Innsbruck, Austria (C.T.).
authors.forename: Gerrit Alexander
authors.initials: GA
authors.lastname: Schubert
authors.forename: Marcus
authors.initials: M
authors.lastname: Czabanka
authors.forename: Marcel
authors.initials: M
authors.lastname: Seiz
authors.forename: Peter
authors.initials: P
authors.lastname: Horn
authors.forename: Peter
authors.initials: P
authors.lastname: Vajkoczy
authors.forename: Claudius
authors.initials: C
authors.lastname: Thomé
mesh_terms: D000375:Aging / Q000502:physiology; D002533:Cerebral Angiography; D002560:Cerebrovascular Circulation / Q000502:physiology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D009072:Moyamoya Disease / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014978:Xenon
chemical_list: D014978:Xenon
keywords: Moyamoya disease; Xenon; computed tomography; perfusion