A psychometric study of children at-risk for Huntington disease

Huntington disease (HD) is an autosomal dominant disorder of the central nervous system with an average age of onset between 35 and 45 years and symptoms progressing slowly over the next 10 to 20 years. Research in the past few years has focused on the hypothesis that a presymptomatic or prodromal phase for HD is detectable at least 10 years prior to chronic symptoms. This study attempts to identify possible signs of a prodromal phase for HD in children who are at primary (50%) and secondary (25%) risk for HD using a screening battery of psychometric tests. The children tested were between the ages of 5 1/2 and 15 years and the tests used were the WISC-R, the PPVT-R, and the VMI. Results from this study indicated performance on the WISC-R Digit Span and to a lesser extent Coding subtests might be useful in assessing a possible memory dysfunction in children at-risk for HD.