Titre
Functional compensation of motor function in pre-symptomatic Huntington's disease.
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Klöppel, S.
Auteure/Auteur
Draganski, B.
Auteure/Auteur
Siebner, H.R.
Auteure/Auteur
Tabrizi, S.J.
Auteure/Auteur
Weiller, C.
Auteure/Auteur
Frackowiak, R.S.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
1460-2156[electronic]
Statut éditorial
Publié
Date de publication
2009
Volume
132
Numéro
Pt 6
Première page
1624
Dernière page/numéro d’article
1632
Peer-reviewed
Oui
Langue
anglais
Résumé
Involuntary choreiform movements are a clinical hallmark of Huntington's disease. Studies in clinically affected patients suggest a shift of motor activations to parietal cortices in response to progressive neurodegeneration. Here, we studied pre-symptomatic gene carriers to examine the compensatory mechanisms that underlie the phenomenon of retained motor function in the presence of degenerative change. Fifteen pre-symptomatic gene carriers and 12 matched controls performed button presses paced by a metronome at either 0.5 or 2 Hz with four fingers of the right hand whilst being scanned with functional magnetic resonance imaging. Subjects pressed buttons either in the order of a previously learnt 10-item finger sequence, from left to right, or kept still. Error rates ranged from 2% to 7% in the pre-symptomatic gene carriers and from 0.5% to 4% in controls, depending on the condition. No significant difference in task performance was found between groups for any of the conditions. Activations in the supplementary motor area (SMA) and superior parietal lobe differed with gene status. Compared with healthy controls, gene carriers showed greater activations of left caudal SMA with all movement conditions. Activations correlated with increasing speed of movement were greater the closer the gene carriers were to estimated clinical diagnosis, defined by the onset of unequivocal motor signs. Activations associated with increased movement complexity (i.e. with the pre-learnt 10-item sequence) decreased in the rostral SMA with nearing diagnostic onset. The left superior parietal lobe showed reduced activation with increased movement complexity in gene carriers compared with controls, and in the right superior parietal lobe showed greater activations with all but the most demanding movements. We identified a complex pattern of motor compensation in pre-symptomatic gene carriers. The results show that preclinical compensation goes beyond a simple shift of activity from premotor to parietal regions involving multiple compensatory mechanisms in executive and cognitive motor areas. Critically, the pattern of motor compensation is flexible depending on the actual task demands on motor control.
Sujets
PID Serval
serval:BIB_C91619330B3C
PMID
Date de création
2010-01-11T12:11:50.682Z
Date de création dans IRIS
2025-05-21T03:58:56Z
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Nom
BIB_C91619330B3C.P001.pdf
Version du manuscrit
preprint
Taille
433.76 KB
Format
Adobe PDF
PID Serval
serval:BIB_C91619330B3C.P001
URN
urn:nbn:ch:serval-BIB_C91619330B3C9
Somme de contrôle
(MD5):e0e9c1a68b21bd75ba62ed25e7d6fdc2