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Introduction. Various neuropsychological studies have been conducted to determine the cognitive functioning of patients with Huntington's disease and their results have shown that the pattern of cognitive decline is thought to be typical of a frontal-subcortical dementia. Objectives. To determine if significant differences exist between the cognitive performances of a group diagnosed with Huntington's disease and a group of healthy, at-risk relatives on a series of neuropsychological tests, and to examine the extent to which the Huntington group's cognitive performance profile corresponds to frontal-subcortical damage as reported in other studies. Patients and methods. The sample was comprised of a group of 18 subjects with a clinical diagnosis of initial stage Huntington's disease and a group of 15 healthy relatives at-risk for Huntington's disease. A battery of tests (the CERAD) and additional neuropsychological tests were administered to all subjects. Results. Compared to the at-risk group, the Huntington's disease group scored significantly lower on 85.41% of the tests. Conclusions. The neuropsychological tests utilized in this study were useful to discriminate between diagnosed and at-risk groups. The Huntington's disease group's global neuropsychological profile was similar to that of a frontal-subcortical dementia in which the predominant features include executive functioning deficits, memory problems, visuo-constructive alterations, attention deficits, verbal fluency problems and naming deficits.

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