[A neuropsychological study of Huntington's disease in families in Antioquia, Colombia].

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.