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Definition

A X-linked dystonia-parkinsonism disorder. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. DYT3 is characterized by severe progressive torsion dystonia followed by parkinsonism. It has a well-defined pathology of extensive neuronal loss and mosaic gliosis in the striatum (caudate nucleus and putamen) which appears to resemble that in Huntington disease.

Acronym

DYT3

Synonyms

Dystonia-3
Lubag
Torsion dystonia-parkinsonism Filipino type
XDP
X-linked dystonia-parkinsonism
X-linked torsion dystonia 3

Keywords

Parkinsonism
Dystonia

 
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