Effect of thalamotomy on focal hand dystonia in a family with DYT1 mutation

Authors

  • Mi J. Kim MD,

    1. Center for Parkinsonism and Other Movement Disorders, Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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  • Sang R. Jeon MD,

    1. Center for Parkinsonism and Other Movement Disorders, Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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  • Han-Wook Yoo MD,

    1. Genome Research Center for Birth Defects & Genetic Disorders, Medical Genetics Clinic & Laboratory, Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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  • Gu-Hwan Kim PhD,

    1. Genome Research Center for Birth Defects & Genetic Disorders, Medical Genetics Clinic & Laboratory, Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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  • Myoung C. Lee MD,

    1. Center for Parkinsonism and Other Movement Disorders, Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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  • Sun J. Chung MD

    Corresponding author
    1. Center for Parkinsonism and Other Movement Disorders, Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
    • Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, 388-1, Poongnap-dong, Songpa-gu, Seoul 138-736, South Korea
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  • Potential conflict of interest: None reported.

Abstract

We report the clinical and molecular features of a family with focal hand dystonia caused by DYT1 mutation. Four members of a family who underwent thalamotomy showed a marked and sustained therapeutic benefit that lasted for up to 12 years without recurrence of dystonia or any significant surgical complication. The hand dystonia caused by DYT1 mutation may be successfully managed by thalamotomy. © 2008 Movement Disorder Society

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