Although cognitive impairment has been reported in adults with moyamoya disease (MMD), its relationship with cerebral hemodynamic disturbances has not been investigated. The aims of the present study were to confirm the presence of dysexecutive cognitive syndrome (DCS) in adults with MMD and to explore the relationship of DCS with frontal lobe perfusion as measured by perfusion MR imaging.
Cerebral blood volume (CBV) ratio and mean transit time delay were measured in frontal and temporoparietal regions using the cerebellum as a reference region in 10 European adults with MMD. In addition, the authors calculated the cerebrovascular reserve (CVR) using the CBV ratio and the acetazolamide challenge. All patients underwent a standardized neuropsychological assessment test battery. The authors defined DCS as an impairment shown on 3 tests or more of executive function.
The authors found DCS in 6 patients. The frontal CVR was lower in patients with DCS than in patients without DCS (mean ± SD: −13.5 ± 13.2% and 20.3 ± 21.3%; p = 0.019, Mann-Whitney U-test). Other parameters of frontal perfusion and temporoparietal CVR were not correlated with DCS.
The authors' findings suggest that DCS is common in European adults with MMD and may be related to frontal perfusion impairment.