The prevalence of Huntington's disease in European populations is estimated at 4 to 8 per 100,000. In the US it is estimated at about 7 per 100,000. The duration of disease is approximately 20 years from time of diagnosis to time of death. As such, the incidence can be estimated at 3.5 cases per million per year or 1000 new cases in the US per year. Data from the English and Welsh Huntington’s Disease Association suggest that the prevalence may be higher than previously estimated. The disease affects men and women equally; typical onset is 35 to 45 years of age with a range of 2 to 80 years of age. It is 10 times more common in North Americans of European descent than in those of pure African or Asian descent or in Native Americans. Mixed populations have an intermediate incidence. Similar trends are seen globally, with a significantly lower incidence in Asia and Africa. In isolated areas, such as Tasmania or Lake Maracaibo in Venezuela, a founder's effect can be seen, where an affected early settler can significantly increase the prevalence of Huntington's disease in a concentrated population.
BMJ Best Practice is an evidence-based point of care tool for healthcare practitioners.
To continue reading and access all of BMJ Best Practice's pages you'll need to log in or start a free trial.
You can access through your institution if your hospital, university, trust or other institution provides access to BMJ Best Practice through either OpenAthens or Shibboleth.
Use of this content is subject to our disclaimer