Long-term mortality in patients with moyamoya angiopathy according to stroke presentation type in South Korea

Background Incidence, prevalence, and long-term survival outcomes in patients with moyamoya angiopathy (MMA) according to stroke presentation type and age group have not been clearly elucidated. Methods We investigated mortality in patients with MMA (moyamoya disease, probable moyamoya disease, moyamoya syndrome) of whose International Classification Disease 10 code was I67.5 from 2006 to 2015 using the Korean National Health Insurance database. MMA at diagnosis was classified into 3 types (ischemic, hemorrhagic, and asymptomatic or else) according to stroke presentation. Survival analysis was performed according to stroke presentation type and age group (< 15 years and >= 15 years) using the Kaplan-Meier method. Results There were 12,146 newly diagnosed moyamoya cases, with a female-to-male ratio of 1.81; the ischemic type was identified in 3671 (30.2%) patients, the hemorrhagic type in 2449 (20.2%) patients, and the asymptomatic or else type in 6026 (49.6%) patients. The mean age at diagnosis according to stroke presentation was 33.1 (+/- 14.8) years in asymptomatic or else type, 41.2 (+/- 17.3) years in ischemic type, and 45.4 (+/- 14.3) years in hemorrhagic type (P < 0.001). The 10-year survival rates in ischemic-, hemorrhagic-, and asymptomatic or else-type patients were 88.9%, 76.3%, and 94.3%, respectively (log-rank test; P < 0.001). Pediatric MMA (< 15 years) and adult MMA (>= 15 years) showed different survival curves according to stroke presentation type (log-rank test; P = 0.017, P < 0.001, respectively). Conclusions Our study showed that moyamoya patients had different diagnosis ages and distinct survival courses according to stroke presentation type. Adult moyamoya patients with hemorrhagic presentation had the worst survival outcomes.