Hunar Kainth

Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease.

BACKGROUND An association between moyamoya disease and Down syndrome appears to exist on the basis of reported anecdotal cases in the literature. OBJECTIVE To determine the prevalence of Down syndrome associated with moyamoya disease in inpatients and to identify the demographic and clinical features of moyamoya disease that may be unique when associated with Down syndrome. METHODS In this observational study, we analyzed data from the Nationwide Inpatient Sample between 2002 and 2009 using International Classification of Diseases codes for moyamoya disease and Down syndrome for patient identification. Data including patient age, sex, race/ethnicity, secondary diagnosis, procedures, hospital costs, and patient outcomes were obtained. RESULTS From 2002 to 2009, an estimated 518 patients (mean ± SD age, 16.2 ± 1.68 years) with coexisting moyamoya disease and Down syndrome were admitted. The estimated prevalence was 3.8% (3760 per 100,000) among patients admitted with moyamoya disease and 9.5% (9540 per 100,000) among moyamoya patients < 15 years of age. Patients admitted with moyamoya disease and Down syndrome were most frequently white and Hispanic (P = .02). They were more likely to present with ischemic stroke and less commonly with hemorrhagic stroke (15.3% and 2.7%, respectively; P < .05). CONCLUSION This is the first study to estimate the prevalence of Down syndrome in patients with moyamoya disease. The 26-fold-greater prevalence of Down syndrome in patients with coexisting moyamoya disease compared with the prevalence of Down syndrome among live births (145 per 100,000) highlights the need for a better understanding of the common pathophysiology of the 2 conditions.

Epidemiological and clinical features of moyamoya disease in the USA

Background: An increasing number of cases of Moyamoya disease have been reported in the Japanese and US literature. We performed this study to quantify the rise in the prevalence of Moyamoya disease and to study the unique epidemiological and clinical features in the USA that may explain a change in incidence. Methods: We analyzed data derived from patients entered in the Nationwide Inpatient Sample between 2005 and 2008, using ICD-9 codes for Moyamoya disease. Data including patient age, gender, ethnicity, secondary diagnosis, medical complications, and hospital costs were obtained. Results: From 2005 to 2008 in the USA, there were an estimated 7,473 patients admitted with a primary or secondary diagnosis of Moyamoya disease. Patients admitted with Moyamoya disease were most frequently women and Caucasian. Overall, ischemic stroke was the most common reason for admission. Hemorrhagic stroke was more frequent in adults compared with children, 18.1 versus 1.5% (p < 0.05). Conclusion: The number of patients identified and admitted with Moyamoya disease has risen dramatically in the last decade. This study can lead to a better understanding of the disease pattern and healthcare consequences in the USA and suggests that pathophysiologic differences in Moyamoya disease may exist.

Contents Vol. 40, 2013

296 Regional North American Annual Meeting of the World Federation of Neurology – Research Group on Neuroepidemiology University of California at San Diego, San Diego, Calif., March 22, 2013 Guest Editors: Weisskopf, M.G. (Boston, Mass.); Leimpeter, A. (Oakland, Calif.); Van Den Eeden, S.K. (Oakland, Calif.) (available online only)

Front & Back Matter

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