Primate models of dystonia.

Dominique Guehl, Emmanuel Cuny, Imad Ghorayeb, Thomas Michelet, Bernard Bioulac, Pierre Burbaud
Progress in Neurobiology. 2009-02-01; 87(2): 118-131
DOI: 10.1016/j.pneurobio.2008.10.003

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1. Prog Neurobiol. 2009 Feb;87(2):118-31. doi: 10.1016/j.pneurobio.2008.10.003. Epub
2008 Nov 6.

Primate models of dystonia.

Guehl D(1), Cuny E, Ghorayeb I, Michelet T, Bioulac B, Burbaud P.

Author information:
(1)Service de Neurophysiologie clinique, CHU de Bordeaux, Hôpital Pellegrin, Rue
Amélie-Rabat Léon, 33076 Bordeaux, France.

Several models of dystonia have emerged from clinical studies providing a
comprehensive explanation for the pathophysiology of this movement disorder.
However, several points remain unclear notably concerning the specific role of
brainstem, basal ganglia nuclei and premotor cortex. We review data collected in
sub-human primate to see whether they might provide new insights into the
pathophysiology of dystonia. As in human patients, lesions of the putamen induce
dystonia, as well as pharmacological manipulations of the dopaminergic system. In
addition, primate studies revealed that lesions in brain stem areas involved in
the control of muscular tone and GABAergic manipulations in various basal ganglia
nuclei or thalamus also lead to dystonia. Moreover, there is a dramatic
disruption in the processing of proprioceptive information with abnormal large
receptive fields in the basal ganglia, thalamus, primary somesthetic cortex and
premotor cortex of dystonic monkeys. These data highlight the idea that dystonia
is associated with aberrant sensory representations interfering with motor
control. Considering that the supplementary motor area (SMAp) is the target of
basal ganglia projections within the motor loop, we propose a model of dystonia
in which abnormal excitability, associated with alteration in sensory receptive
fields within the SMAp, leads to an abnormal synchronization between primary
motor cortex columns. Such a phenomenon might account for the co-contractions of
antagonist muscles favored by action and the abnormal postures observed in

DOI: 10.1016/j.pneurobio.2008.10.003
PMID: 19022333 [Indexed for MEDLINE]

Auteurs Bordeaux Neurocampus