Canadian Journal of Neurological Sciences: Volume 15 -

The Dystonias
Edith G. McGeer, Patrick L. McGeer
Published online by Cambridge University Press:

18 September 2015, pp. 447-483
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Dystonia is a difficult problem for both the clinician and the scientist. It is sufficiently common to be seen by almost all physicians, yet uncommon enough to prevent any physician from gaining broad experience in its diagnosis and treatment. Each case represents a difficult challenge even to the specialist. The basic scientist is faced with investigating a disorder that is without relevant animal models and which is so rare that obtaining suitable tissue for study is a major obstacle. Dystonia may be idiopathic, or associated with lesions from many sources, including a variety of rare diseases. If idiopathic, it may be genetically transmitted or sporadic. If genetically transmitted, it may be generalized or focal, with symptoms varying in different members of the same family. It may be refractory to treatment, or it may respond to any one of a number of individual drugs that have very different mechanisms of action. For idiopathic dystonias, no clear method of genetic transmission has been established and no consistent pathology identified.