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Clinical Pediatrics
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Idiopathic Torsion Dystonia Associated with Lesions of the Basal Ganglia

Rhandy PeBenito

Stanley S. Lamm Institute for Developments) Disabilities, Department of Pediatrics and the Department of Radiology, the Long Island College Hospital, Department of Neurology (Pediatric Neurology), State University of New York, Downstate Medical Center, Brook- lyn, New York

Chantal Farretti

Stanley S. Lamm Institute for Developments) Disabilities, Department of Pediatrics and the Department of Radiology, the Long Island College Hospital, Department of Neurology (Pediatric Neurology), State University of New York, Downstate Medical Center, Brook- lyn, New York

Riffat R. Chaudary

Stanley S. Lamm Institute for Developments) Disabilities, Department of Pediatrics and the Department of Radiology, the Long Island College Hospital, Department of Neurology (Pediatric Neurology), State University of New York, Downstate Medical Center, Brook- lyn, New York

Paul K. Woodrow

Stanley S. Lamm Institute for Developments) Disabilities, Department of Pediatrics and the Department of Radiology, the Long Island College Hospital, Department of Neurology (Pediatric Neurology), State University of New York, Downstate Medical Center, Brook- lyn, New York

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.

Clinical Pediatrics, Vol. 23, No. 4, 232-235 (1984)
DOI: 10.1177/000992288402300410
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 J Child NeurolHome page
R.D. Craver, M.C. Duncan, and J.S. Nelson
Familial Dystonia and Choreoathetosis in Three Generations Associated With Bilateral Striatal Necrosis
J Child Neurol, May 1, 1996; 11(3): 185 - 188.
[Abstract] [PDF]