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Cognitive Manifestations of Huntington Disease in Relation to Genetic Structure and Clinical Onset
Gregor W. Jason, D Phil;
Oksana Suchowersky, MD;
Eva M. Pajurkova, PhD;
Lisa Graham;
Mary Lou Klimek, MA;
Anthony T. Garber, PhD;
Danielle Poirier-Heine
Arch Neurol. 1997;54(9):1081-1088.
Abstract
Objective
To examine the cognitive manifestations of Huntington disease (HD) with respect to age, clinical onset, progression, and genetic analyses.
Design
Case series of people with HD or at risk (AR) for HD.
Setting
Movement disorders and medical genetics clinics.
Participants
Volunteer sample of 50 patients with HD and 127 AR adults.
Measures
Neuropsychological evaluation was conducted with multiple measures of cognitive function (intelligence, memory, attention, executive, spatial, language), strength, manual speed/dexterity, somato-sensory function, and mood. Quantitative molecular genetic analysis by means of polymerase chain reaction was conducted on 31 patients with HD and 86 AR subjects.
Results
In clinical HD, cognitive impairment correlated with number of years affected but not age at onset. The linear regression had a negative intercept, suggesting impaired cognitive function by the time of onset. In AR gene carriers, lower cognitive performance correlated with more trinucleotide repeats. In clinical HD, trinucleotide repeats interacted with disease chronicity such that more repeats were associated with worse performance over time; the overall effect of this was small compared with the effect of disease chronicity alone. Except for one AR subject, mood state was not associated with cognitive performance in either patients with HD or AR subjects.
Conclusions
Cognitive decline appears to start before clinical onset of HD and is correlated with the number of trinucleotide repeats. Subsequent cognitive decline is primarily a function of number of years affected, although there is evidence that the presence of more trinucleotide repeats is associated with faster deterioration.
Author Affiliations
From the Department of Psychology, Foothills Hospital (Drs Jason and Pajurkova), and Departments of Clinical Neurosciences (Drs Jason, Suchowersky, and Pajurkova and Ms Poirier-Heine) and Medical Genetics (Drs Suchowersky and Garber and Mss Graham and Klimek), University of Calgary, Calgary, Alberta.
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