Perfusion characteristics of Moyamoya disease: an anatomically and clinically oriented analysis and comparison

Gerrit Alexander Schubert1, Marcus Czabanka, Marcel Seiz

  • 1From 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.).

Stroke
|November 7, 2013
PubMed
Abstract

Insights

Moyamoya disease (MMD) shows unique brain blood flow patterns, with preserved central perfusion unlike cerebrovascular atherosclerotic disease (CAD). Hemodynamic stress and reserve capacity are key indicators of MMD severity.

Area of Science:

  • Neuroscience
  • Radiology
  • Cerebrovascular Diseases

Background:

  • Moyamoya disease (MMD) presents distinct angiographic collateralization patterns.
  • Quantitative analysis comparing MMD to cerebrovascular atherosclerotic disease (CAD) and controls is lacking.

Purpose of the Study:

  • To quantitatively analyze and compare cerebral blood flow (CBF), cerebrovascular reserve capacity (CVRC), and hemodynamic stress distribution in MMD versus CAD and controls.
  • To investigate the relationship between these hemodynamic parameters and age in MMD and CAD.
  • To correlate hemodynamic findings with the characteristic collateralization patterns in MMD.

Main Methods:

  • Retrospective review of 67 MMD patients, 108 CAD patients, and 5 controls using Xenon-enhanced computed tomography.
  • Calculation of CBF, CVRC, and hemodynamic stress distribution in cortical, central, infratentorial, pericallosal, and basal ganglia regions.
  • Comparative analysis of hemodynamic parameters across patient groups and with respect to age.

Main Results:

  • MMD exhibits a global decrease in CVRC and cortical CBF reduction compared to controls, with preserved basal ganglia perfusion.
  • Age significantly impacts cortical and central CBF in both MMD and CAD, but not CVRC or hemodynamic stress distribution.
  • MMD and CAD show similar CVRC, but MMD has higher hemodynamic stress, reflecting its unique collateral network (rete mirabilis).

Conclusions:

  • MMD demonstrates a unique territory-specific perfusion pattern, notably preserved central CBF, correlating with proximal collateralization.
  • CVRC and hemodynamic stress distribution are more sensitive indicators of disease severity than CBF alone in MMD.
  • Quantitative hemodynamic analysis aids in understanding MMD pathophysiology and differentiating it from CAD.