Idiopathic Torsion Dystonia Associated with Lesions of the Basal Ganglia

Abstract

We report two siblings who are suffering from a dystonic syndrome, clinically indistinguishable from idiopathic torsion dystonia (dystonia musculorum defor mans) but with cranial computerized tomographic scan findings of basal ganglia lesions, similar to that reported in Wilson's disease.

The occurrence of the disorder in the same sibship suggests an autosomal recessive mode of inheritance and may represent another variety of the syndrome of idiopathic torsion dystonia.