Hereditary ataxias: epidemiological aspects

Neuroepidemiology. 1990;9(6):321-31. doi: 10.1159/000110795.


Hereditary ataxias, hereditary spastic paraplegia and Charcot-Marie-Tooth syndrome (HA) are chronic progressive neurological diseases. Epidemiologic studies of these disorders are few. In a geographically well-defined Danish population, we present incidence rates, cumulated incidence rates and prevalence for patients with HA based on modern continuous-time survival analysis techniques. From these, prevalence has been estimated to be 6.06 per 10(5) in the 10 to 50-year-old population. Combined risk of HA was found to be 0.16% for women and 0.20% for men up to their 51st birthday.

MeSH terms

  • Adolescent
  • Adult
  • Cerebellar Ataxia / mortality
  • Charcot-Marie-Tooth Disease / mortality
  • Child
  • Cross-Sectional Studies
  • Denmark / epidemiology
  • Female
  • Follow-Up Studies
  • Friedreich Ataxia / mortality
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Spastic Paraplegia, Hereditary / mortality
  • Spinocerebellar Degenerations / mortality*
  • Survival Rate