Moyamoya Disease and Syndrome: A National Inpatient Study of Ischemic Stroke Predictors
Santiago R Unda1, Aldana M Antoniazzi1, Raphael Miller1
1Leo M. Davidoff Department of Neurosurgery, Montefiore Medical Center, Albert Einstein College of Medicine, 3316 Rochambeau Ave. Bronx, NY 10467 USA.
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.
Insights
Arterial ischemic stroke (AIS) is the most common presentation for both Moyamoya disease (MMD) and Moyamoya syndrome (MMS). MMD patients experience higher stroke rates, with age, lipidemia, and smoking as key risk factors, while hypertension is a predictor in MMS.
Area of Science:
- Neurology
- Vascular Neurology
- Epidemiology
Background:
- Moyamoya disease (MMD) and Moyamoya syndrome (MMS) are rare cerebrovascular disorders characterized by progressive stenosis of intracranial arteries.
- Stroke, both ischemic and hemorrhagic, is a primary complication of MMD and MMS.
Purpose of the Study:
- To determine the incidence of stroke subtypes in patients with MMD and MMS.
- To identify predictive factors for arterial ischemic stroke (AIS) in a large US-based cohort of MMD and MMS patients.
Main Methods:
- Utilized the 2016-2017 National Inpatient Sample database.
- Included patients diagnosed with Moyamoya and associated conditions (sickle cell disease, neurofibromatosis type 1, cranial radiation, Down Syndrome).
- Employed multivariate regression analysis to identify risk factors for AIS.
Main Results:
- A total of 2323 patients were analyzed; 71.2% had MMD and 28.8% had MMS.
- AIS was the most frequent stroke type in both MMD and MMS cohorts.
- MMD patients exhibited significantly higher rates of AIS, hemorrhagic stroke, and transient ischemic attack (TIA) compared to MMS patients.
- Independent risk factors for AIS in MMD included advanced age, lipidemia, and current smoking.
- In MMS, hypertension and African-American race were independent predictors of AIS.
Conclusions:
- Arterial ischemic stroke is the predominant stroke presentation in both Moyamoya disease and Moyamoya syndrome.
- Moyamoya disease patients face a higher burden of stroke events compared to Moyamoya syndrome patients.
- Distinct risk factor profiles for AIS exist between MMD (age, lipidemia, smoking) and MMS (hypertension).
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