小児虚血型もやもや病の長期転帰および発症様式に関与する因子の解析

Abstract

To clarify the important factors contributing to outcome of moyamoya disease patients showing ischemia onset, we analyzed the long-term outcomes of 59 (24 males, 35 females) young moyamoya patients. The mean follow-up period and mean age was 11.1±7.8 years and 7.0±3.9 years old, respectively. Forty-three cases received bypass operations and 4 cases refused. The distribution of Glasgow Outcome Scale of all cases at onset and at follow-up showed no statistical difference. Eight cases (2 females, 6 males) showed neurological deterioration during the follow-up period, and the causes of worsened outcomes were 4 infarctions, 1 hemorrhage and 3 other complications. No cerebral hemisphere that received bypass surgery suffered infarction. Thus, it is concluded that the most important factor contributing to poor neurological outcome is infarction and that bypass surgery (direct or indirect bypass) can effectively prevent infarction over long terms. To clarify the important angiographic findings contributing to infarction occurrence, we analyzed the relationship between angiographical findings and cerebral infarction. In 143 bilateral hemispheres, we identified the presence of infarction (n=57) and transient ischemic attack (TIA, n=43). Forty-three hemispheres were asymptomatic. We analyzed the relationship between the occurrence of infarction and the status of stenosis/occlusion in the internal carotid artery (ICA) and PCA in each hemisphere of moyamoya disease patients. The incidence of PCA stenotic/occlusive lesions was significantly higher in infarcted (78.9%) than non-infarcted hemispheres (TIA: 14.0%; asymptomatic: 9.4%; p<0.01). No other factors exhibited a statistical correlation. Our data suggest that stenotic/occlusive PCA lesions were the most important independent factor contributing to the occurrence of cerebral infarction and that inhibition of the progression of PCA stenosis/occlusion may help to avoid broad cerebral infarction in moyamoya disease patients.