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Relative risk of spread of symptoms among the focal onset primary dystonias

Authors

  • Elliott M. Weiss BA,

    1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA
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  • Tamara Hershey PhD,

    1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA
    2. Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, USA
    3. Department of Radiology, Washington University School of Medicine, St. Louis, Missouri, USA
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  • Morvarid Karimi MD,

    1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA
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  • Brad Racette MD,

    1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA
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  • Samer D. Tabbal MD,

    1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA
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  • Jonathan W. Mink MD, PhD,

    1. Department of Neurology, University of Rochester Medical Center, Rochester, New York, USA
    2. Department of Neurobiology and Anatomy, University of Rochester Medical Center, Rochester, New York, USA
    3. Department of Pediatrics, University of Rochester Medical Center, Rochester, New York, USA
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  • Randal C. Paniello MD,

    1. Department of Otolaryngology, Washington University School of Medicine, St. Louis, Missouri, USA
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  • Joel S. Perlmutter MD

    Corresponding author
    1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA
    2. Department of Radiology, Washington University School of Medicine, St. Louis, Missouri, USA
    3. Department of Anatomy and Neurobiology, Washington University School of Medicine, St. Louis, Missouri, USA
    4. Program of Physical Therapy, Washington University School of Medicine, St. Louis, Missouri, USA
    • Campus Box 8225, Washington University School of Medicine, 4525 Scott Avenue, St. Louis, MO 63110
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Abstract

Adult-onset primary torsion dystonia (PTD) may spread to multiple body parts, but the relative risk of spread by site of onset of dystonia has not been well characterized. We retrospectively identified 602 patients with PTD out of 1,500 dystonia patients in our electronic database and extracted age at onset, site of onset, family history, and spread. Survival analyses were performed for groups based on site of onset, and hazard ratios compared relative risk of spread across groups. Patients with adult-onset blepharospasm were more likely to spread (31% past the head) than those with dystonia starting in the neck (9%), larynx (12%), or upper extremities (16%). Hazard ratios proved that the blepharospasm group had the greatest relative risk of spread. The rate of spread after onset varied significantly between the different groups. Most spread occurred in the first 1 to 2 years after onset of blepharospasm, whereas the risk of spread was relatively constant over time in cervical and laryngeal dystonia. Different sites of onset of PTD confer different risks of spread, important for clinical prognosis. Different risks of spread may provide clues about underlying pathogenesis of adult-onset primary dystonias. © 2006 Movement Disorder Society

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