Moyamoya Disease and Syndrome: A National Inpatient Study of Ischemic Stroke Predictors

Objectives: Ischemic stroke and hemorrhagic stroke are the most common sequelae of the Moyamoya variants [Moyamoya disease (MMD) and syndrome (MMS)]. We sought to determine the rates of stroke subtypes and the predictive factors of arterial ischemic stroke (AIS) utilizing a large data sample of MMD and MMS patients in the US. Materials and methods: We queried the 2016 and 2017 National Inpatient Sample database for Moyamoya diagnosis plus any of the following associated conditions; sickle cell disease, neurofibromatosis type 1, cranial radiation therapy or Down Syndrome. Multivariate regression determined the risk factors for AIS onset in MMD and MMS. Results: 2323 patients with a diagnosis of Moyamoya were included; 668 (28.8%) patients were classified as MMS and 1655 (71.2%) as MMD. AIS was the most common presentation in both cohorts; however, MMD patients had higher rates of AIS (20.4 vs 6%, p < 0.001), hemorrhagic stroke (7.4vs 2.5%, p < 0.001), and TIA (3.3vs 0.9%, p = 0.001) compared to MMS patients. Multivariate analysis showed that increasing age [OR = 1.017 95%CI: 1.008–1.03, p < 0.001], lipidemia [OR = 1.32 95%CI: 1.02–1.74, p = 0.049], and current smoking status [OR = 1.43 95%CI: 1.04–1.97, p = 0.026] were independent risk factors for AIS in MMD patients, whereas hypertension [OR = 2.61 95%CI: 1.29–5.25, p = 0.007] and African-American race [OR = 0.274, 95%CI: .117–.64, p = 0.003] were independent predictors in the MMS cohort. Conclusion: AIS is the most common presentation in both, MMD and MMS. However, MMD patients had higher rates of stroke events compared to MMS. Risk factors for AIS in MMD included increasing age, lipidemia and smoking status, whereas in MMS hypertension was the only independent risk factor.