Dystonia musculorum deformans

Dystonia musculorum deformans is a disease of muscle torsion and posturing that affects children between the ages of 5 and 15. The disease first affects the feet and legs. In adolescents the symptoms tend to start in the hands and arms, sometimes resembling writer’s cramp. At early onset children may gaitor prance. This is a common occurrence among children in the early stages of this disorder, and it may be one of the first signs that is noticed. Some children are more skillful at walking backward than forward during the early onset of the disease. As the symptoms progress the torsion posturing becomes more rigid and severe. Because of the nature of the torsions, the name dystonia has recently been supplanted by idiopathic torsion dystonia. Dystonic posturing may also occur in Wilson’s disease, Huntington’s disease, and Parkinsonism, so differential diagnosis is essential.


 


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