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Familial essential tremor and idiopathic torsion dystonia are different genetic entities

INSERM U289 and Clinique de Neurologie, Hôpital de la Salpêtrière, Paris, France.

Familia1 essential tremor (ET) is an autosomal dominant disorder presenting as an isolated postural tremor. Its frequent association with dystonia suggests that the two disorders might be pathogenically related. We report the exclusion of the DYTl locus on chromosome 9q32–34, responsible for idiopathic torsion dystonia (ITD), in two large ET families. We conclude that ET and ITD are distinct genetic disorders.

Address correspondence and reprint requests to Dr. Alexandra Dürr, INSERM U289, Hôpital de la Salpêtrière, 47 boulevard de l'Hôpita1, 75651 Paris Cedex 13, France.

Supported by the Association Fransaise contre les Myopathies and the Association pour le Développement de la Recherche sur les Maladies Génétiques Neurologiques et Psychiatriques.

Received February 18, 1993. Accepted for publication in final form March 31, 1993.

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