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Cent Eur Neurosurg 2011; 72(3): 144-148
DOI: 10.1055/s-0031-1271730
Review

© Georg Thieme Verlag KG Stuttgart · New York

Current Concepts on the Mechanisms of Dystonia and the Beneficial Effects of Deep Brain Stimulation

L. C. Stavrinou1 , 2 , E. J. Boviatsis1 , 2 , A. Leonardos2 , I. G. Panourias1 , 2 , D. E. Sakas1 , 2
  • 1Department of Neurosurgery, Evangelismos General Hospital, University of Athens, Greece
  • 2“Petros Kokkalis” Hellenic Centre for Neurosurgical Research, Athens, Greece
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Publication History

Publication Date:
20 May 2011 (online)

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Abstract

The application of lesioning procedures in the basal ganglia and, more recently, of deep brain stimulation (DBS) has revolutionalized dystonia treatment. However, our understanding of the mechanism of action of DBS is only minimal. This is largely due to a rudimentary understanding of dystonia pathophysiology itself, which in turn reflects an insufficient understanding of the functional significance of the cortico-striato-pallido-thalamocortical loops. The initial dystonia pathophysiology concept was one of changes in oscillation rate. Soon, it was realized that not only rate but also the pattern of basal ganglia activity is crucial in the etiology of the disease. The observations of altered somatosensory responsiveness and cortical neuroplasticity, along with the vast array of clinical phenotypes, imply the need for a wholistic neuronal pathophysiology model; one in which an underlying defect of basal ganglia function results in increased cortical excitability, misprocessing of sensory feedback, aberrant cortical plasticity, and ultimately clinical dystonia. This unified dystonia pathophysiology model, although simplistic, may provide the scaffold on which all incoming research and clinical data becomes united in a meaningful and practical way. In light of this model, the dramatic response of some forms of dystonia to pallidal stimulation, the time latency for the beneficial effect and even the presence of non-responders may be explained. Additionally, it may help in developing a rationale for more efficacious DBS programming, better selection of the timing of surgery, and more successful identification of those candidates that are most likely to respond to DBS.

Key words

dystonia - deep brain stimulation - pathophysiology - DBS mechanism

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Correspondence

Dr. L. C. Stavrinou

Department of Neurosurgery

Evangelismos General Hospital

University of Athens

Ipsilantou str. 45–47

10676 Athens

Greece

Phone: + 30/210/7201 742

Fax: + 30/210/7201 704

Email: mplam@hotmail.com

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