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Vol. 40, No. 4, 2013
Issue release date: May 2013
Neuroepidemiology 2013;40:282-287

Epidemiological and Clinical Features of Moyamoya Disease in the USA

Kainth D. · Chaudhry S.A. · Kainth H. · Suri F.K. · Qureshi A.I.
Zeenat Qureshi Stroke Research Center, Departments of Neurology, Neurosurgery, and Radiology, University of Minnesota, Minneapolis, Minn., USA

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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.

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  1. Smith E, Scott M: Surgical management of Moyamoya syndrome. Skull Base 2005;15:15-26.
  2. Suzuki J, Kodama N: Moyamoya disease: a review. Stroke 1983;14:104-109.
  3. Fukui M: Current state of study on Moya-moya disease in Japan. Surg Neurol 1997;47:138-143.
  4. Goto Y, Yonekawa Y: Worldwide distribution of moyamoya disease. Neurol Med Chir (Tokyo) 1992;32:883-886.
  5. Kuriyama S, Kusaka Y, Fujimura M, Wakai K, et al: Prevalence and clinicoepidemiological features of Moyamoya disease in Japan. Stroke 2008;39:42-47.
  6. Chui D, Shedden P, Bratina P, Grotta J: Clinical features of Moyamoya disease in the United States. Stroke 1998;29:1347-1351.
  7. Graham JF, Matoba A: A survey of Moyamoya disease in Hawaii. Clin Neurol Neurosurg 1997;99:S31-S35.
  8. Numaguchi Y, Gonzalez CF, Davis PC, et al: Moyamoya disease in the United States. Clin Neurol Neurosurg 1997;99(suppl 2):S26-S30.
  9. HCUP Nationwide Inpatient Sample: Design of the Nationwide Inpatient Sample, 2010.
  10. Ishimori M, Cohen S, Hallegua D, Moser F, Weisman M: Ischemic stroke in a postpartum patient: understanding the epidemiology, pathogenesis, and outcome of Moyamoya disease. Semin Arthritis Rheum 2006;35:250-259.
  11. Yoshida Y, Yoshimoto T, Shirane R, Sakurai Y: Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: an extensive follow-up study. Stroke 1999;30:2272-2276.
  12. Edwards-Brown MK, Quets J: Midwest experience with Moyamoya disease. Clin Neurol Neurosurg 1997;99:S36-S38.
  13. Scott RM, Smith JL, Robertson RL, et al: Long-term outcome in children with moya-moya syndrome after cranial revascularization by pial synangiosis. J Neurosurg 2004;100(suppl 2):142-149.
  14. Peerless SJ: Risk factors of Moyamoya disease in Canada and the USA. Clin Neurol Neurosurg 1997;99:S45-S48.
  15. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoiki R, et al: Epidemiologic features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 1997;99:S1-S5.
  16. United States Census Bureau 2010.
  17. Zipel G, Fox D, Rivet D: Moyamoya Disease in adults: the role of cerebral vascularization. Skull Base 2005;15:27-41.
  18. Lee D, Liebeskind D: Characterization of inpatient moyamoya in the United States: 1988-2004. Front Neurol 2011;2:1-5.
  19. Ikezaki K, Han DH, Kawano T, Kinuwaka N, Fukui M: A clinical comparison of definite moyamoya disease between South Korea and Japan. Stroke 1997;28:2513-2517.
  20. Yilmaz E, Pritz M, Bruno A, et al: Moyamoya: Indiana University Medical Center experience. Arch Neurol 2001;58:1274-1278.
  21. Yamashita M, Oka K, Tanaka K: Histopathology of the brain vascular network in moyamoya disease. Stroke 1983;14:50-58.

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