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Older onset essential tremor: More rapid progression and more degenerative pathology

Authors

  • Elan D. Louis MD, MSc,

    Corresponding author
    1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    3. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    4. Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, New York, USA
    • Unit 198, Neurological Institute, 710 West 168th Street, New York, NY 10032, USA
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  • Phyllis L. Faust MD, PhD,

    1. Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Jean-Paul G. Vonsattel MD,

    1. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Lawrence S. Honig MD, PhD,

    1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    3. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Claire Henchcliffe MD, DPhil,

    1. Weill Medical College of Cornell University, New York, New York, USA
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  • Rajesh Pahwa MD,

    1. Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA
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  • Kelly E. Lyons PhD,

    1. Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA
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  • Eileen Rios BS,

    1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Cordelia Erickson-Davis BA,

    1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Carol B. Moskowitz MS,

    1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Arlene Lawton RN

    1. GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, New York, USA
    2. Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, New York, USA
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  • Potential conflict of interest: Nothing to report.

Abstract

There are few data on rate of progression in essential tremor (ET). To quantify the rate of tremor progression in a cross-sectional sample of 348 ET cases in an epidemiological study; characterize the relationship between age of tremor onset and rate of tremor progression in that sample; and characterize the relationship between age of tremor onset, rate of tremor progression, and severity of underlying brain changes in 9 cases from a brain repository. Rate of tremor progression was defined as tremor severity ÷ duration. The degeneration index = number of torpedoes per section ÷ Purkinje cell linear density. In the epidemiological study, older age of tremor onset was associated with faster rate of tremor progression (P < 0.001). In the brain repository, older age of tremor onset was associated with higher degeneration index (P = 0.037), and higher degeneration index was associated with faster rate of tremor progression (P = 0.018). In a large clinical sample, older age of onset was associated with more rapid tremor progression. In a brain bank, older age of onset was associated with more degenerative pathology in the cerebellum. As in several neurodegenerative disorders, in older onset cases, it is possible that the disease advances more rapidly. © 2009 Movement Disorder Society

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