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The association of CAG repeat length with clinical progression in Huntington disease

The association of CAG repeat length with clinical progression in Huntington disease The association of CAG repeat length with clinical progression in Huntington disease A. Rosenblatt, MD; K.-Y. Liang, PhD; H. Zhou, MS; M.H. Abbott, MPH, RN; L.M. Gourley, MA; R.L. Margolis, MD; J. Brandt, PhD; and C.A. Ross, MD, PhD Abstract—Objective: To determine whether the rate of clinical progression in Huntington disease (HD) is influenced by the size of the CAG expansion. Methods: The dataset consisted of 3,402 examinations of 512 subjects seen through the Baltimore Huntington’s Disease Center. Subjects were seen for a mean of 6.64 visits, with mean follow-up of 6.74 years. Subjects were administered the Quantified Neurological Examination, with its subsets the Motor Impairment and Chorea Scores, the Mini-Mental State Examination, and the HD Activities of Daily Living (ADL) Scale. Results: In an analysis based on the Random Effects Model, CAG length was significantly associated with the rate of progression of all measures except chorea and ADL. There was a significant interaction term between CAG length and disease duration for all measures except chorea. Further graphical exploration of the data supported these linear models and suggested that subjects at the low end of the expanded CAG repeat range may experience a more benign late course. Conclusions: CAG http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Neurology Wolters Kluwer Health

The association of CAG repeat length with clinical progression in Huntington disease

Neurology , Volume 66 (7) – Apr 1, 2006

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ISSN
0028-3878
eISSN
1526-632X
DOI
10.1212/01.wnl.0000204230.16619.d9
pmid
16606912
Publisher site
See Article on Publisher Site

Abstract

The association of CAG repeat length with clinical progression in Huntington disease A. Rosenblatt, MD; K.-Y. Liang, PhD; H. Zhou, MS; M.H. Abbott, MPH, RN; L.M. Gourley, MA; R.L. Margolis, MD; J. Brandt, PhD; and C.A. Ross, MD, PhD Abstract—Objective: To determine whether the rate of clinical progression in Huntington disease (HD) is influenced by the size of the CAG expansion. Methods: The dataset consisted of 3,402 examinations of 512 subjects seen through the Baltimore Huntington’s Disease Center. Subjects were seen for a mean of 6.64 visits, with mean follow-up of 6.74 years. Subjects were administered the Quantified Neurological Examination, with its subsets the Motor Impairment and Chorea Scores, the Mini-Mental State Examination, and the HD Activities of Daily Living (ADL) Scale. Results: In an analysis based on the Random Effects Model, CAG length was significantly associated with the rate of progression of all measures except chorea and ADL. There was a significant interaction term between CAG length and disease duration for all measures except chorea. Further graphical exploration of the data supported these linear models and suggested that subjects at the low end of the expanded CAG repeat range may experience a more benign late course. Conclusions: CAG

Journal

NeurologyWolters Kluwer Health

Published: Apr 1, 2006

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