Clinical rating, caudate atrophy, disturbed movement performance, neuropsychological testing, and age-related genetic disease load (CAG index) are tools that reflect impairment after onset of Huntington's disease (HD). Objectives were to compare scored HD symptoms, results of neuropsychological testing and of instrumental measurement of simple motion sequences, assess caudate atrophy and CAG index, and investigate their relation to each other in 131 subjects of various HD stages. Caudate atrophy and CAG index significantly increased in advanced HD patients. Motor test results significantly differed between HD patients and 49 controls, but not between HD gene carriers and controls. Instrumental test outcomes, scored HD intensity, caudate atrophy, and CAG index significantly correlated to each other. Neuropsychological testing, which we only performed in the HD gene carriers and the previously untreated HD patients, reflected the early appearance of HD symptoms and correlated with the motor test results. Results of our applied instrumental tool measure impaired movement performance, which is not specific for HD, but reflects the various methods assessed and the slowly evolving symptoms of the degenerative process in HD.
(c) 2006 Movement Disorder Society