AUTHOR=Svedung Wettervik Teodor , Fahlström Markus , Wikström Johan , Lewén Anders , Enblad Per 

TITLE=Cerebrovascular reserve in moyamoya disease: relation to cerebral blood flow, capillary dysfunction, oxygenation, and energy metabolism

JOURNAL=Frontiers in Neurology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2023.1190309

DOI=10.3389/fneur.2023.1190309

ISSN=1664-2295

ABSTRACT=Background: Cerebral hemodynamics in Moyamoya disease (MMD) is complex and needs further elucidation. The primary aim was to determine the association of the cerebrovascular reserve (CVR) with cerebral blood flow (CBF) disturbances, oxygen extraction fraction (OEFmax), and energy metabolism (CMRO2max) in MMD, using arterial spin label magnetic resonance imaging (ASL-MRI) before and after acetazolamide administration. 
Methods: Thirty-nine ASL-MRI scans with a concurrent acetazolamide challenge from 16 MMD patients at the Uppsala University Hospital, Sweden, 2016-2021, were retrospectively analyzed. CBF was assessed before and 5, 15, and 25 minutes after acetazolamide administration and the maximal response CVRmax was used for further analyses. Dynamic susceptibility contrast (DSC) MRI was performed 30 minutes after acetazolamide injection and the data were analysed using the Cercare Medical Neurosuite to assess capillary transit time heterogeneity (CTTH; indicating microvascular function), OEFmax, and CMRO2max. 
Results: In the ACA-territory, lower CVRmax was associated with lower baseline CBF, higher CTTH, and higher OEFmax, but not with CMRO2max in generalized estimating equation models. In the MCA-territory, lower CVRmax was associated with lower baseline CBF and higher CMRO2max, but not with CTTH and OEFmax. 
Conclusions: Altogether, a compromised CVR in MMD patients reflected disturbances in macro-/microvascular blood flow, oxygenation, and CMRO2. ASL-MRI with acetazolamide challenge is a feasible and radiation-free alternative to positron emission tomography (PET)-imaging in MMD.