Phenotypic expression of the DYT1 mutation: A family with writer's cramp of juvenile onset

Recently, the mutation causing early‐onset generalized torsion dystonia has been identified as a GAG deletion in the gene for an adenosine triphosphate–binding protein named torsinA. We describe a German family with 5 clinically affected individuals carrying this mutation. In at least 4 of the 5 pat...

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Veröffentlicht in:Annals of neurology 1998-07, Vol.44 (1), p.126-128
Hauptverfasser: Gasser, T., Windgassen, K., Bereznai, B., Kabus, C., Ludolph, A. C.
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Sprache:eng
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Zusammenfassung:Recently, the mutation causing early‐onset generalized torsion dystonia has been identified as a GAG deletion in the gene for an adenosine triphosphate–binding protein named torsinA. We describe a German family with 5 clinically affected individuals carrying this mutation. In at least 4 of the 5 patients, the disease presented as a dystonic writer's cramp during late childhood or adolescence, which affected sequentially both sides but did not progress to a generalized form of dystonia. We conclude that familial writer's cramp may be a manifestation of the DYT1 mutation.
ISSN:0364-5134
1531-8249
DOI:10.1002/ana.410440119