To describe the characteristics of patients with moyamoya disease (MMD) and analyze its associated risk factors in the United States population.

MMD is a rare cause of stroke, primarily prevalent in Asian populations but routinely encountered by neurologists and neurosurgeons in the United States.  MMD usually presents with strokes and intracerebral hemorrhages and is diagnosed radiographically with intracranial vessel imaging.

We performed a cross-sectional study using the National Inpatient Sampling (NIS) database for year 2014. Moyamoya disease and other risk factors were identified using ICD-9 codes. Multiple logistic regression for complex design was used to evaluate the odds of MMD for a set of risk factors. All statistical analyses were performed using the SAS system (version 9.4, Cary, NC, USA).

A total of 907 patients with MMD were identified from 2014 NIS database. The mean age of patients with MMD was 34.4 years with 67.9% being females, 38.3% are Whites, 36.7% are African Americans, 11.7% are Hispanics and 8.2% are Asians. In MMD, the prevalence of ischemic stroke (IS) was 17.5%, while hemorrhagic stroke (HS) was found in 4.4% of the patients. Patients with thyroid disorders, type I diabetes, and Down’s syndrome, were 5.2 (95% CI: 3.0-9.0), 1.8 (95% CI: 1.1-3.0), and 21.2 (95% CI: 13.7-32.6) times more likely to have MMD. Gender, age, race, hypertension, congestive heart failure (CHF), IS, HS, and transient ischemic attack were also associated with MMD. 

There is increased prevalence of ischemic and hemorrhagic stroke in MMD. Our study showed higher number of females in MMD patients and strong association of MMD with other autoimmune diseases.