 |
|
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.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati  Twitter
What's this?
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
|
The association of CAG repeat length with clinical progression in Huntington disease
Rosenblatt et al.
Neurology 2006;66:1016-1020.
ABSTRACT
| FULL TEXT
Huntington's disease: Clinical correlates of disability and progression
Mahant et al.
Neurology 2003;61:1085-1092.
ABSTRACT
| FULL TEXT
Cognitive and Motor Functioning in Gene Carriers for Huntington's Disease: A Baseline Study
Witjes-Ane et al.
J. Neuropsychiatry Clin. Neurosi. 2003;15:7-16.
ABSTRACT
| FULL TEXT
PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
Modregger et al.
Hum Mol Genet 2002;11:2547-2558.
ABSTRACT
| FULL TEXT
Evidence for Genetic Mediation of Executive Control: A Study of Aging Male Twins
Swan and Carmelli
Journals of Gerontology Series B: Psychological Sciences and Social Science 2002;57:P133-143.
ABSTRACT
| FULL TEXT
Clinical markers of early disease in persons near onset of Huntington's disease
Paulsen et al.
Neurology 2001;57:658-662.
ABSTRACT
| FULL TEXT
The genetic basis of cognition
Flint
Brain 1999;122:2015-2032.
ABSTRACT
| FULL TEXT
Neuropsychological abnormalities in first degree relatives of patients with familial Parkinson's disease
Dujardin et al.
J. Neurol. Neurosurg. Psychiatry 1999;67:323-328.
ABSTRACT
| FULL TEXT
Genetic Structure and Cognitive Function in Huntington Disease
JWatch Psychiatry 1998;1998:13-13.
FULL TEXT
|
