Hemodynamic stress distribution identified by SPECT reflects ischemic symptoms of Moyamoya disease patients

This study aimed to validate the hypothesis that the ratio of cerebral blood flow (CBF) at rest in the lenticular nucleus (LN) territory to that in the middle cerebral artery (MCA) territory is higher in symptomatic Moyamoya disease (MMD) patients than in asymptomatic MMD patients. This was a retrospective observational study of adult patients with documented MMD who underwent single-photon emission computed tomography (SPECT) and had been examined at the Department of Neurosurgery of Keio University Hospital during a 10-year period (2006–2016). The diagnosis was made on the basis of typical imaging findings. We classified unoperated MMD patients into three groups: class I, no evidence of stenosis or occlusion hemispheres and without symptoms in unilateral MMD patients; class II, hemispheres with stenosis or occlusion but without ischemic symptoms; and class III, hemispheres with evidence of stenosis or occlusion associated with ischemic symptoms. Hemodynamic stress distribution (hdSD) was defined as the ratio of CBF in one LN to the CBF in the peripheral MCA; this was obtained by SPECT at rest. We compared the values of CBF and hdSD among the groups. A total of 173 adult patients were diagnosed with MMD from January 1, 2006, to January 1, 2016. Among them, 85 MMD patients underwent SPECT studies. After excluding inappropriate cases, 144 hemispheres were included in our analysis. hdSD was significantly higher ( p < 0.001) in hemispheres with ischemic symptoms (class III, mean hdSD = 1.1; 36 sides) than in those without symptoms (class II, mean hdSD = 1.03; 82 sides). However, CBF at rest in the MCA or LN was not significantly associated with ischemic symptoms. The optimal threshold for hdSD to have ischemic symptoms was 1.040 (area under the curve; 74% sensitivity 91.7% and specificity 54.9%). We used SPECT to investigate cerebral blood from MMD patients and found that high hdSD values were predictive of ischemic symptom development in these patients.