Descriptors Found: 1
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Descriptor English:   Dystonia Musculorum Deformans 
Descriptor Spanish:   Distonía Muscular Deformante 
Descriptor Portuguese:   Distonia Muscular Deformante 
Synonyms English:   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 of Childhood
Torsion Disease, Childhood
Torsion Dystonia
Torsion Dystonia, Idiopathic
Torsion Spasm, Progressive  
Tree Number:   C10.
Definition English:   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) 
See Related English:   Dystonia
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications CN congenital
DI diagnosis DG diagnostic imaging
DH diet therapy DT drug therapy
EC economics EM embryology
EN enzymology EP epidemiology
EH ethnology ET etiology
GE genetics HI history
IM immunology ME metabolism
MI microbiology MO mortality
NU nursing PS parasitology
PA pathology PP physiopathology
PC prevention & control PX psychology
RT radiotherapy RH rehabilitation
SU surgery TH therapy
UR urine VE veterinary
VI virology  
Record Number:   4485 
Unique Identifier:   D004422 

Occurrence in VHL: