2016 Volume 44 Issue 1 Pages 1-7
This study included 42 patients with moyamoya disease hospitalized at our institution between August 1995 and September 2012. We retrospectively examined their medical records for the disease course, patients' baseline characteristics, and outcomes of the surgical intervention.
Twenty-five patients were female (59.5%) and 17 were male (40.5%). Their age at the time of diagnosis ranged from 1 to 70 years (mean age, 37 years). Fourteen (33.3%) patients showed hemorrhage, 11 (26.2%) patients experienced transient ischemic attack, 6 (14.3%) patients showed cerebral infarction, 2 (4.8%) patients showed epilepsy, and 3 (7.1%) patients experienced headache at the time of diagnosis. In the case of 4 patients, the disease was incidentally detected, and in the case of 2 patients, the cause was unknown. Thirteen (76.5%) patients with ischemic symptoms, 2 patients (14.3%) with hemorrhage, 2 patients (100%) with epilepsy, and 2 patients who were asymptomatic underwent surgical intervention. In 3 of 18 (16.7%) patients who underwent surgical intervention and in 8 of 24 (33.3%) patients who did not undergo surgical intervention, the outcome was poor (modified Rankin Scale [mRS] score, 5 or 6). According to the classification of the patients by the disease onset, 4 of 17 (23.5%) patients with ischemic onset and 7 of 14 (50%) patients with hemorrhagic onset showed poor outcomes (mRS 5 or 6). Patients with hemorrhagic onset showed poorer outcomes than did those with ischemic onset, and the poorer outcomes were the consequence of bleeding. A method for the prevention of bleeding in patients with moyamoya disease needs to be established.
