Diabetes mellitus in Huntington disease

Abstract

There were conflicting reports that individuals with Huntington disease (HD) are prone to abnormalities of carbohydrate metabolism. Information about the incidence and control of diabetes mellitus in 620 probands (278 living, 332 deceased) with HD and in their 1st and 2nd degree relatives was obtained by questionnaire method from participants of the National HD Research Roster. Among the probands, 65 individuals (10.5%) were identified by the informant or vertified by examination of Roster family records as diabetic. The prevalence of diabetes, particularly among those less than 50 yr of age, is significantly greater than corresponding figures among the general USA Caucasian population. Incidence rates were not calculated because of ascertainment and other biases in the data. Analysis of family data indicates that HD affected relatives of an HD proband with diabetes are 7 times as likely to have diabetes over the proband''s non-HD relatives. A non-diabetic HD proband is equally likely to have an HD or non-HD relative with diabetes.