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Neurobiol Dis. 2011 May;42(2):177-84. doi: 10.1016/j.nbd.2010.08.025. Epub 2010 Sep 15.

Neurophysiology of dystonia: The role of inhibition.

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Human Motor Control Section, National Institute of Neurological Disorders and Stroke, NIH, Building 10, Room 7D37, 10 Center Dr MSC 1428, Bethesda, MD 20892-1428, USA.


The pathophysiology of dystonia has been best studied in patients with focal hand dystonia. A loss of inhibitory function has been demonstrated at spinal, brainstem and cortical levels. Many cortical circuits seem to be involved. One consequence of the loss of inhibition is a failure of surround inhibition, and this appears to directly lead to overflow and unwanted muscle spasms. There are mild sensory abnormalities and deficits in sensorimotor integration; these also might be explained by a loss of inhibition. Increasing inhibition may be therapeutic. A possible hypothesis is that there is a genetic loss of inhibitory interneurons in dystonia and that this deficit is a substrate on which other factors can act to produce dystonia. This article is part of a Special Issue entitled "Advances in dystonia".

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