Epidemiological and Clinical Features of Moyamoya Disease in the USA

Abstract

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.

Cerebrovascular Collaterals Correlate with Disease Severity in Adult North American Patients with Moyamoya Disease

“…1,6–8,29,30 In contrast, Asian studies reported higher rates of hemorrhage, ranging from 25% to 62% (Japan, China, and Korea) compared with 10%–29% (Iowa and Hawaii). 3,15,29,31,32 Hemorrhage has been shown to be the most significant factor affecting poor outcome in Asian cohorts, and ruptured thin-walled basal perforators have been cited as the source of high rates of hemorrhage.…”

Research Progresses in Understanding the Pathophysiology of Moyamoya Disease

“…Several studies highlighted the differences between MMD presentation in Japanese and American patients. In comparison to Asian cases, Caucasian MMD patients are characterized by an older average age of presentation with lack of bimodal age distribution, prevalence of ischemic stroke type at all ages, more benign presenting symptoms and less common familial occurrence [7][8][9][10][11][12][13][14] . However, the basis of geographical differences in disease occurrence and presentation are still unknown, although environmental and genetic factors have been invoked as possibly influencing factors.…”

Moyamoya in Hispanics: not only in Japanese

“…15 Typically moyamoya disease and moyamoya syndrome present during childhood, however the incidence of both forms in North America occurs in the mid-40s. 3 …”

“…In general, Moyamoya disease is observed among the various races based on their proportions of the total US population. 3 …”