Cerebral blood flow, transit time, and apparent diffusion coefficient in moyamoya disease before and after acetazolamide



The goal of this study was to assess the changes in arterial spin labeling (ASL) cerebral blood flow (CBF) and arterial transit time (ATT), and in apparent diffusion coefficient (ADC), before and after an acetazolamide challenge in moyamoya patients, as function of arterial stenosis severity.


Pre-operative patients diagnosed with moyamoya disease who could undergo MRI at 3.0T were recruited for this study. A multi-delay pseudo-continuous ASL and a diffusion-weighted sequence were acquired before and 15 min after acetazolamide injection. The severity of anterior, middle, and posterior cerebral artery pathology was graded on time-of-flight MR angiographic images. CBF, ATT, and ADC were measured on standardized regions of interest as function of the vessel stenosis severity.


Thirty patients were included. Fifty-four percent of all vessels were normal, 28% mildly/moderately stenosed, and 18% severely stenosed/occluded. Post-acetazolamide, a significantly larger CBF (ml/100 g/min) increase was observed in territories of normal (+19.6 ± 14.9) compared to mildly/moderately stenosed (+14.2 ± 27.2, p = 0.007), and severely stenosed/occluded arteries (+9.9 ± 24.2, p < 0.0001). ATT was longer in territories of vessel anomalies compared with normal regions at baseline. ATT decreases were observed in all territories post-acetazolamide. ADC did not decrease after acetazolamide in any regions, and no correlation was found between ADC changes and baseline ATT, change in ATT, or CVR.


The hemodynamic response in moyamoya disease, as measured with ASL CBF, is impaired mostly in territories with severe arterial stenosis/occlusion, while ATT was prolonged in all non-normal regions. No significant changes in ADC were observed after acetazolamide.

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CF was supported by the Swiss National Science Foundation.

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Corresponding author

Correspondence to Christian Federau.

Ethics declarations

We declare that all human and animal studies have been approved by the Stanford University Ethics Committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. We declare that all patients gave informed consent prior to inclusion in this study.

Conflict of interest

SC consults for iSchemaView and GZ receives research support from GE Healthcare.

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Federau, C., Christensen, S., Zun, Z. et al. Cerebral blood flow, transit time, and apparent diffusion coefficient in moyamoya disease before and after acetazolamide. Neuroradiology 59, 5–12 (2017). https://doi.org/10.1007/s00234-016-1766-y

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  • Arterial spin labeling
  • Moyamoya disease
  • Acetazolamide challenge
  • Cerebral blood flow
  • Apparent diffusion coefficient