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This study aimed to determine the influence of atherosclerotic risk factors on initial and further cerebrovascular events in adult patients with moyamoya disease (MMD) by combined analysis of two prospective cohorts in which patients received pharmacotherapy alone and were prospectively followed-up for 5 years. In 71 patients, smoking status, home blood pressure, hemoglobin (Hb)A1c and low-density lipoprotein cholesterol (LDL-chol) were checked at inclusion and at further cerebrovascular event or at the end of 5-year follow-up. When a patient had daily smoking, increased HbA1c, increased LDL-chol, increased systolic blood pressure, or increased diastolic blood pressure, the patient was categorized as showing atherosclerotic burden. Angiographic disease progression was determined using changes on magnetic resonance angiography. Eleven patients showed angiographic disease progression and seven of these 11 patients experienced further cerebrovascular events during the follow-up period. The remaining 60 patients did not exhibit either condition. At inclusion, the incidence of atherosclerotic burden was significantly greater in patients without angiographic disease progression (80%) than in those with such progression (45%;  p  = 0.0249). For patients without angiographic disease progression, values or incidence of almost all variables showed significant interval decreases at the end of 5-year follow-up ( p  &lt; 0.05). Adult patients with ischemic MMD who do not exhibit angiographic disease progression appear more strongly affected by atherosclerotic burden at the initial onset of cerebrovascular events than those exhibiting angiographic disease progression. A reduction in atherosclerotic burden by medical treatments for the former patients prevents further cerebrovascular events.

Influence of atherosclerotic burden on adult patients with ischemic moyamoya disease: combined analysis of two prospective cohorts