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Front. Neurol. | doi: 10.3389/fneur.2018.00012

How many types of dystonia? Pathophysiological considerations

  • 1Department of Biomedical, Dental Sciences and Morphological and Functional Images, University of Messina, Italy
  • 2IRCCS Centro Neurolesi Bonino Pulejo, Italy
  • 3Leibniz Research Centre for Working Environment and Human Factors, at Technical University, Germany

Dystonia can be seen in a number of different phenotypes that may arise from different aetiologies. The pathophysiological substrate of dystonia is related to three lines of research.
The first postulate a loss of inhibition which may account for the excess of movement and for the overflow phenomena. A second abnormality is sensory dysfunction which is related to the mild sensory complaints in patients with focal dystonias and may be responsible for some of the motor dysfunction. Finally there are strong evidences form animal and human studies suggesting that alterations of synaptic plasticity characterized by a disruption of homeostatic plasticity, with a prevailing facilitation of synaptic potentiation may play a pivotal role in primary dystonia.
These working hypotheses have been generalized in all form of dystonia. On the other hand, several evidences now suggest that the pathophysiology may be slightly different in the different types of dystonia. Therefore in the present review we would like to discuss the neural mechanisms underlying the different forms of dystonia to disentangle the different weight and role of environmental and predisposing factors.

Keywords: Dystonia, Basal Ganglia Diseases, Transcranial Magnetic Stimulation, neuroplasticity, tractography

Received: 12 Apr 2017; Accepted: 08 Jan 2018.

Edited by:

Antonio Pisani, Università degli Studi di Roma Tor Vergata, Italy

Reviewed by:

Graziella Madeo, National Institutes of Health (NIH), United States
Francesca Morgante, Università degli Studi di Messina, Italy
Kathrin Grundmann, Universität Tübingen, Germany  

Copyright: © 2018 Quartarone and Ruge. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Angelo Quartarone, University of Messina, Department of Biomedical, Dental Sciences and Morphological and Functional Images, Messina, Italy, angelo.quartarone@unime.it