Cognition in Huntington's disease : the influence of motor behaviour and time

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Cognition in Huntington's disease : the influence of motor behaviour and time

Title: Cognition in Huntington's disease : the influence of motor behaviour and time
Author: Hart, Ellen Patricia 't
Publisher: Department Neurology, Faculty of Medicine, Leiden University Medical Center (LUMC), Leiden University
Issue Date: 2013-11-14
Keywords: Huntington's disease
Cognition
Movement disorder
Executive functioning
Memory
P300
Motor behaviour
Abstract: Huntington's disease (HD) is a genetic neurodegenerative disease. Carriers of the HD gene without clinical symptoms of the disease can be identified and studied. The study of these premanifest subjects is of importance for the understanding of preclinical disease progression and for the design of future clinical trials. HD is characterized by progressive decline in motor functioning, cognition and behaviour. The unwanted motor disturbances that patients experience are likely to be of influence on cognitive functioning, as cognitive tests almost always require a motoric response. In this thesis we investigated cognitive functioning in both premanifest HD gene carriers and HD patients by taking into account the motor disturbances that have been reported in both pre- and manifest phases of the disease. We also reports on the influence of time on cognition in HD by means of several longitudinal reports with follow-up periods as long as ten years. We found both carriers and patients deteriorate most on memory and executive functioning domains, with the latter being most sensitive in the premanifest phase. We have also found evidence for the presence of premanifest (cognitive) compensatory mechanisms. As expected, there is a substantial (negative) effect of motor functioning on cognition in HD.
Description: Promotor: R.A.C. Roos
Supervisor: With Summary in Dutch
Faculty: LUMC
Citation: Hart, E.P. 't, 2013, Doctoral Thesis, Leiden University
Sponsor: de Vereniging van Huntington - Topaz - Lundbeck B.V. - Stichting Alkemade-Keuls
Handle: http://hdl.handle.net/1887/22209
 

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application/pdf Acknowledgements_Publications_Curriculum Vitae 126.5Kb View/Open
application/pdf Summary in Dutch 112.6Kb View/Open
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