Dystonia Musculorum Deformans

**Definition:** 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)

**Tree numbers:** - C10.228.140.079.357 - C10.228.662.300.200 - C10.574.500.393 - C16.320.400.330

**Synonyms:** - Childhood Torsion Disease - Dystonia Deformans Musculorum - Dystonia Deformans Progressiva - Oppenheim-Ziehen Disease - Progressive Torsion Spasm - Torsion Disease of Childhood - Torsion Dystonia