Background and Purpose: The cause of moyamoya disease (MMD) remains unknown. We aimed to investigate the association between modifiable risk factors and MMD in a prospective, case-control study. Methods: Clinical and laboratory characteristics were evaluated in consecutively recruited adult patients with MMD and age-matched healthy control individuals. The potential risk factors for MMD were estimated by logistic regression analysis. Results: Our prospective study included 138 adult patients and 138 healthy control subjects. Logistic regression analyses showed that increased body mass index (odds ratio [OR], 1.121 [95% CI, 1.018–1.234]; P =0.020) and homocysteine (OR, 1.201 [95% CI, 1.081–1.334]; P =0.001) were associated with higher risk of MMD. Whereas increased albumin (OR, 1.043 [95% CI, 1.004–1.082]; P =0.028) and high-density lipoprotein cholesterol (OR, 1.043 [95% CI, 1.004–1.082]; P =0.028) were correlated with a lower risk of MMD. Furthermore, homocysteine (OR, 1.070 [95% CI, 1.010–1.134]; P =0.023) was significantly related to unilateral lesions. Conclusions: Increased body mass index and homocysteine were associated with a higher risk of MMD. In contrast, increased albumin and high-density lipoprotein cholesterol were correlated with a lower risk of MMD. Furthermore, increased homocysteine was related to a higher prevalence of unilateral MMD. More attention should be paid to the modifiable risk factors of MMD, as these might help us finding its cause and new therapeutic regimen. Registration: URL: http://www.chictr.org . Unique identifier: ChiCTR2000031412.

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