Dystonia Musculorum Deformans, Sprague-Dawley Rat with the Dystonic (dt) Mutation

  • Henry J. Baker
Part of the Monographs on Pathology of Laboratory Animals book series (LABORATORY)


The dystonias are a heterogeneous group of motor disorders characterized by sustained, involuntary twisting movements (Fahn and Eldridge 1976). Although dystonia can be a Symptom of a variety of neuromuscular diseases, it is the primary clinicopathologic hallmark of the family of human inherited torsion dystonias known as dystonia musculorum deformans. The pathogenetic basis of these diseases is unknown and no characteristic light or electron microscopic lesions have been observed (Zeman 1976). In 1980, Baker et al. and Lorden et al. (1984) described Sprague-Dawley rats with clinical and biochemical characteristics similar to human inherited torsion dystonias, and named the mutation Dystonie (dt).


Glutamic Acid Decarboxylase Deep Cerebellar Nucleus Dystonic Movement Affected Mouse Torsion Dystonia 
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© Springer-Verlag Berlin Heidelberg 1991

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  • Henry J. Baker

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