Paediatric moyamoya in mainland France: a comprehensive survey of academic neuropaediatric centres

Cerebrovasc Dis. 2012;33(1):76-9. doi: 10.1159/000333424. Epub 2011 Dec 1.

Abstract

Background: Epidemiological data of paediatric moyamoya disease/syndrome (MMD/MMS) in non-Asian populations are scarce.

Methods: A questionnaire was sent to every French neuropaediatric academic centre to estimate the prevalence, incidence, familial form rate and location of paediatric MMD/MMS cases. Specific paediatric data were also retrieved from the most recent nationwide Japanese study.

Results: A 100% response rate was obtained. The prevalence of paediatric MMD/MMS was estimated at 0.39/100,000 children (95% CI: 0.28-0.49), and the incidence was estimated at 0.065/100,000 children/year (95% CI: 0.025-0.12), with 7.5% familial cases. The prevalence was homogenous within the different administrative areas.

Conclusions: This comprehensive survey of MMD/MMS in academic neuropaediatric centres suggests that the prevalence of the disease in children in France is approximately 1/20th of that estimated in Asia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Academic Medical Centers / statistics & numerical data*
  • Adolescent
  • Age Distribution
  • Age Factors
  • Asian People / statistics & numerical data
  • Child
  • Child, Preschool
  • France / epidemiology
  • Genetic Predisposition to Disease
  • Health Surveys
  • Humans
  • Incidence
  • Japan / epidemiology
  • Moyamoya Disease / epidemiology*
  • Moyamoya Disease / ethnology
  • Moyamoya Disease / genetics
  • Prevalence
  • Residence Characteristics
  • Surveys and Questionnaires