Spontaneous brain activity in adult patients with moyamoya disease: a resting-state fMRI study

• We studied spatial patterns of functional brain activity of adult moyamoya disease.
• Amplitude of low-frequency fluctuations (ALFF) of BOLD-fMRI at rest was examined.
• Differences in ALFF were mainly observed in frontal, parietal and temporal gyri.
• Moyamoya disease exhibits a specific intrinsic pattern of ALFF.
• ALFF patterns change with the progression of vascular cognitive decline.

Adult patients with moyamoya disease (MMD) are reported to suffer from vascular cognitive impairment (VCI), including considerable impairment of executive function/attention. The spatial pattern of functional brain activity in adult MMD patients with VCI has not been studied before and can be measured by examining the amplitude of low-frequency fluctuations (ALFF) of blood oxygen level-dependent functional magnetic resonance imaging (BOLD fMRI) during rest. Twenty-three adult patients with MMD were recruited to participate in this study, including 11 with VCI and 12 without VCI (NonVCI), as well as 22 healthy young adults (normal control, NC). Widespread differences in ALFF were observed between the VCI/NonVCI and NC groups in such regions as the frontal, parietal and temporal gyri, with parts of the frontal gyrus, such as the anterior cingulate cortex (ACC) and the right supplemental motor area (SMA), showing significant differences in ALFF. It is worth to note that regions such as the parietal gyrus, the right superior frontal gyrus (SFG), the right superior temporal gyrus (STG) and the left caudate nucleus (CN) exhibited significant changes in ALFF during the progressive cognitive decline of MMD. Taken together, our results demonstrate that MMD exhibits a specific intrinsic pattern of ALFF and that this pattern changes with the progression of cognitive decline, providing insight into the pathophysiological nature of this disease.