J Neurol Neurosurg Psychiatry 83:531-536 doi:10.1136/jnnp-2011-301387
  • Cerebrovascular disease
  • Research paper

Regional differences in incidence and patient characteristics of moyamoya disease: a systematic review

  1. Catharina J M Klijn
  1. Utrecht Stroke Centre, Rudolf Magnus Institute of Neuroscience, Department of Neurology and Neurosurgery, University Medical Centre Utrecht, The Netherlands
  1. Correspondence to R Kleinloog, Utrecht Stroke Centre, Rudolf Magnus Institute of Neuroscience, Department of Neurology and Neurosurgery, G.03.232, University Medical Centre Utrecht, Heidelberglaan 100, Utrecht 3584 CX, The Netherlands; r.kleinloog{at}
  1. Contributors CJMK and GJER had the idea for the article. RK performed the literature search and wrote the article with CJMK. All authors extracted data from the selected articles. GJER, CJMK and LR reviewed the article and gave supervision. CJMK is the guarantor.

  • Received 8 September 2011
  • Revised 9 December 2011
  • Accepted 25 January 2012
  • Published Online First 29 February 2012


Background and purpose Moyamoya disease (MMD) is a rare cause of stroke, initially described in Japan. In other countries, incidences and presenting symptoms may differ from those in Japan. The literature on regional differences in incidence and patient characteristics of MMD was systematically reviewed.

Methods Medline, EMBASE and CINAHL were searched for population based studies on MMD published between January 1969 and January 2011. From studies that met predefined inclusion criteria, information was extracted on incidence and patient characteristics. Incidences with corresponding 95% CIs if possible were calculated and descriptive statistics for patient characteristics were used.

Results 8 studies were included: three from Japan, one each from Taiwan and China and three from the USA. Incidences per 100 000 patient years ranged in Japan from 0.35 to 0.94 (95% CI 0.69 to 1.19), in the USA from 0.05 (−0.04 to 0.12) in Iowa to 0.17 (−0.06 to 0.40) in Hawaii and were 0.41 (0.28 to 0.54) in Nanjing, China and 0.02 (0.003 to 0.04) in Taiwan. Female to male ratio ranged from 1.1 (0.9 to 1.5) in Nanjing to 2.8 (1.2 to 6.1) in Iowa. Proportions with intracerebral haemorrhage as the initial presentation were 56% in China, 52% in Taiwan, 29% in Hawaii, 21% in Japan and 10% in Iowa. Patients with childhood onset presented most often with ischaemia (>75%) in all regions.

Conclusions MMD incidence was higher in Japan and China than in Taiwan and North America and presenting symptoms showed regional differences, which are thus far unexplained. Population based data on MMD in Europe are lacking.


  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Articles citing this article

Visit the full archive of podcasts for JNNP here >>

Free sample
This recent issue is free to all users to allow everyone the opportunity to see the full scope and typical content of JNNP.
View free sample issue >>

Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.

Navigate This Article