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Concept information

Preferred term

Dystonia Musculorum Deformans  

Type

  • Topical Descriptor

Entry terms

  • Childhood Torsion Disease
  • Dystonia Deformans Musculorum
  • Dystonia Deformans Progressiva
  • Dystonia, Idiopathic Torsion
  • Dystonias, Idiopathic Torsion
  • Dystonias, Torsion
  • Idiopathic Torsion Dystonia
  • Idiopathic Torsion Dystonias
  • Oppenheim Ziehen Disease
  • Oppenheim-Ziehen Disease
  • Progressive Torsion Spasm
  • Spasm, Progressive Torsion
  • Torsion Disease, Childhood
  • Torsion Disease of Childhood
  • Torsion Dystonia
  • Torsion Dystonia, Idiopathic
  • Torsion Spasm, Progressive

Scope note

  • A condition characterized by focal DYSTONIA that progresses to involuntary spasmodic contractions of the muscles of the legs, trunk, arms, and face. The hands are often spared, however, sustained axial and limb contractions may lead to a state where the body is grossly contorted. Onset is usually in the first or second decade. Familial patterns of inheritance, primarily autosomal dominant with incomplete penetrance, have been identified. (Adams et al., Principles of Neurology, 6th ed, p1078)

In other languages

URI

http://www.yso.fi/onto/mesh/D004422

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