Prevalence and clinicoepidemiological features of moyamoya disease in Japan: Findings from a nationwide epidemiological survey

Shinichi Kuriyama, Yasuko Kusaka, Miki Fujimura, Kenji Wakai, Akiko Tamakoshi, Shuji Hashimoto, Ichiro Tsuji, Yutaka Inaba, Takashi Yoshimoto

Research output: Contribution to journalArticle

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Abstract

BACKGROUND AND PURPOSE - The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease. METHODS - The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey. RESULTS - In 2003, the total number of patients treated in Japan was estimated at 7700 (95% confidence interval, 6300 to 9300). Sex ratio (women to men) of the patients was 1.8. For men, the peak of moyamoya disease was observed in patients aged 10 to 14 years and for women aged 20 to 24 years. Annual rate of newly diagnosed cases in 2003 was 0.54 per 100 000 population. Family history of moyamoya disease was found in 12.1% of the patients. The majority (77.9%) were treated as outpatients. CONCLUSIONS - Although the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones, the estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade (3900 in 1994 and 7700 in 2003). The increase could partly be explained by the increase in newly diagnosed cases (0.35 in 1994 and 0.54 in 2003 per 100 000 population).

Original languageEnglish
Pages (from-to)42-47
Number of pages6
JournalStroke
Volume39
Issue number1
DOIs
Publication statusPublished - 01-01-2008

Fingerprint

Moyamoya Disease
Japan
Pediatric Hospitals
Surveys and Questionnaires
Sex Ratio
Neurosurgery
Neurology
Internal Medicine
Population
Outpatients
Medicine
Confidence Intervals

All Science Journal Classification (ASJC) codes

  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine
  • Advanced and Specialised Nursing

Cite this

Kuriyama, Shinichi ; Kusaka, Yasuko ; Fujimura, Miki ; Wakai, Kenji ; Tamakoshi, Akiko ; Hashimoto, Shuji ; Tsuji, Ichiro ; Inaba, Yutaka ; Yoshimoto, Takashi. / Prevalence and clinicoepidemiological features of moyamoya disease in Japan : Findings from a nationwide epidemiological survey. In: Stroke. 2008 ; Vol. 39, No. 1. pp. 42-47.
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title = "Prevalence and clinicoepidemiological features of moyamoya disease in Japan: Findings from a nationwide epidemiological survey",
abstract = "BACKGROUND AND PURPOSE - The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease. METHODS - The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey. RESULTS - In 2003, the total number of patients treated in Japan was estimated at 7700 (95{\%} confidence interval, 6300 to 9300). Sex ratio (women to men) of the patients was 1.8. For men, the peak of moyamoya disease was observed in patients aged 10 to 14 years and for women aged 20 to 24 years. Annual rate of newly diagnosed cases in 2003 was 0.54 per 100 000 population. Family history of moyamoya disease was found in 12.1{\%} of the patients. The majority (77.9{\%}) were treated as outpatients. CONCLUSIONS - Although the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones, the estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade (3900 in 1994 and 7700 in 2003). The increase could partly be explained by the increase in newly diagnosed cases (0.35 in 1994 and 0.54 in 2003 per 100 000 population).",
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Kuriyama, S, Kusaka, Y, Fujimura, M, Wakai, K, Tamakoshi, A, Hashimoto, S, Tsuji, I, Inaba, Y & Yoshimoto, T 2008, 'Prevalence and clinicoepidemiological features of moyamoya disease in Japan: Findings from a nationwide epidemiological survey', Stroke, vol. 39, no. 1, pp. 42-47. https://doi.org/10.1161/STROKEAHA.107.490714

Prevalence and clinicoepidemiological features of moyamoya disease in Japan : Findings from a nationwide epidemiological survey. / Kuriyama, Shinichi; Kusaka, Yasuko; Fujimura, Miki; Wakai, Kenji; Tamakoshi, Akiko; Hashimoto, Shuji; Tsuji, Ichiro; Inaba, Yutaka; Yoshimoto, Takashi.

In: Stroke, Vol. 39, No. 1, 01.01.2008, p. 42-47.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Prevalence and clinicoepidemiological features of moyamoya disease in Japan

T2 - Findings from a nationwide epidemiological survey

AU - Kuriyama, Shinichi

AU - Kusaka, Yasuko

AU - Fujimura, Miki

AU - Wakai, Kenji

AU - Tamakoshi, Akiko

AU - Hashimoto, Shuji

AU - Tsuji, Ichiro

AU - Inaba, Yutaka

AU - Yoshimoto, Takashi

PY - 2008/1/1

Y1 - 2008/1/1

N2 - BACKGROUND AND PURPOSE - The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease. METHODS - The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey. RESULTS - In 2003, the total number of patients treated in Japan was estimated at 7700 (95% confidence interval, 6300 to 9300). Sex ratio (women to men) of the patients was 1.8. For men, the peak of moyamoya disease was observed in patients aged 10 to 14 years and for women aged 20 to 24 years. Annual rate of newly diagnosed cases in 2003 was 0.54 per 100 000 population. Family history of moyamoya disease was found in 12.1% of the patients. The majority (77.9%) were treated as outpatients. CONCLUSIONS - Although the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones, the estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade (3900 in 1994 and 7700 in 2003). The increase could partly be explained by the increase in newly diagnosed cases (0.35 in 1994 and 0.54 in 2003 per 100 000 population).

AB - BACKGROUND AND PURPOSE - The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease. METHODS - The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey. RESULTS - In 2003, the total number of patients treated in Japan was estimated at 7700 (95% confidence interval, 6300 to 9300). Sex ratio (women to men) of the patients was 1.8. For men, the peak of moyamoya disease was observed in patients aged 10 to 14 years and for women aged 20 to 24 years. Annual rate of newly diagnosed cases in 2003 was 0.54 per 100 000 population. Family history of moyamoya disease was found in 12.1% of the patients. The majority (77.9%) were treated as outpatients. CONCLUSIONS - Although the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones, the estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade (3900 in 1994 and 7700 in 2003). The increase could partly be explained by the increase in newly diagnosed cases (0.35 in 1994 and 0.54 in 2003 per 100 000 population).

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