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Early Cognitive and Motor Symptoms in Identified Carriers of the Gene for Huntington Disease
Gerly M. de Boo, MSc;
Aad Tibben, PhD;
Jan B. K. Lanser, PhD;
Aag Jennekens-Schinkel, PhD;
Jo Hermans, PhD;
Anneke Maat-Kievit, MD;
Raymund A. C. Roos, MD, PhD
Arch Neurol. 1997;54(11):1353-1357.
Abstract
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Objective
To study early motor and cognitive symptoms in Huntington disease.
Design
A follow-up cohort study after a DNA test procedure in which gene carriers and noncarriers were identified among people genetically at risk for Huntington disease.
Setting
Leiden University Medical Center, Department of Neurology, Leiden, the Netherlands, in cooperation with the Clinical Genetics Center Leiden and the Department of Medical Psychology and Psychotherapy, Erasmus University Rotterdam, Rotterdam, the Netherlands.
Participants
Thirty-three individuals: 9 unaffected gene carriers, 6 gene carriers with motor symptoms, and 18 noncarriers of the gene for Huntington disease.
Main Outcome Measures
A neuropsychologic examination covering a broad area of cognitive functioning, reaction time procedures, and motor tasks.
Results
The neuropsychologic assessment showed no significant differences between presymptomatic gene carriers and noncarriers. Three motor tasks differentiated between these 2 groups on a liberal.05 P level (analysis of variance followed by the Student t test). The affected gene carriers performed less well than the presymptomatic gene carriers and the noncarriers in 10 motor tasks and 7 cognitive tasks. These differences were significant at P<.05.
Conclusion
Motor symptoms play a more prominent and unequivocal role than cognitive symptoms in early stages of Huntington disease.
Author Affiliations
From the Departments of Neurology (Ms de Boo and Drs Lanser, Jennekens-Schinkel, and Roos), Medical Statistics (Dr Hermans), and Clinical Genetics (Dr Maat-Kievit), Leiden University Medical Centre, Leiden, the Netherlands; Departments of Medical Psychology and Psychotherapy (Ms de Boo and Dr Tibben) and Clinical Genetics (Dr Tibben), Erasmus University Rotterdam, Rotterdam, the Netherlands. Dr Jennekens-Schinkel is now with Wilhelmina Children's Hospital, Utrecht University Hospital, Utrecht, the Netherlands.
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