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25 - Focal hand dystonia

Published online by Cambridge University Press:  23 December 2009

By
Sarah Pirio Richardson  and
Mark Hallett
Edited by
Dennis A. Nowak  and
Joachim Hermsdörfer
Dennis A. Nowak
Affiliation:
Klinik Kipfenberg, Kipfenberg, Germany
Joachim Hermsdörfer
Affiliation:
Technical University of Munich
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Summary

Summary

Imprecise and unwanted movements characterize the clinical presentation of focal hand dystonia. It is often task-specific, manifesting as writer's cramp or musician's dystonia (e.g. guitarist's cramp). Repetitive, stereotyped movements play a role in the development of the dystonia, but clearly, a pathophysiological substrate must be present for the disorder to manifest. This substrate is likely due to genetics; however, the exact genetic abnormality is not yet known. Although presenting as a motor disorder, dystonia is also a sensory disorder with subtle abnormalities found in spatial and temporal discrimination and with disordered sensory cortical maps. Abnormal cortical plasticity and a failure of homeostatic mechanisms also are seen in dystonia. Finally, a loss of inhibition from excessive muscle discharge to alterations in cortical circuits has been identified in dystonia. As a result, abnormal sensorimotor integration, abnormal plasticity and a loss of inhibition all are implicated in the pathophysiology of focal hand dystonia. Currently, it is not known which of these pathophysiological abnormalities is primary or secondary to the disorder development. Treatment strategies are aimed at ameliorating these physiological changes by improving the sensory deficit, normalizing plasticity and restoring inhibition.

Focal hand dystonia

Dystonia, a neurological disorder, is characterized by abnormal posturing due to sustained muscle contractions, which interferes with the normal performance of motor tasks (Hallett, 2004). Dystonia can be classified by age at onset, by distribution and by cause (Tarsy & Simon, 2006).

Type
Chapter
Information
Sensorimotor Control of Grasping
Physiology and Pathophysiology
 , pp. 348 - 360
Publisher: Cambridge University Press
Print publication year: 2009

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