Dystonia in Huntington's disease: Prevalence and clinical characteristics
Article first published online: 22 JAN 2001
Copyright © 1999 Movement Disorder Society
Volume 14, Issue 1, pages 95–101, January 1999
How to Cite
Louis, E. D., Lee, P., Quinn, L. and Marder, K. (1999), Dystonia in Huntington's disease: Prevalence and clinical characteristics. Mov. Disord., 14: 95–101. doi: 10.1002/1531-8257(199901)14:1<95::AID-MDS1016>3.0.CO;2-8
- Issue published online: 22 JAN 2001
- Article first published online: 22 JAN 2001
- Manuscript Accepted: 4 SEP 1998
- Manuscript Received: 3 JUL 1998
- NIH. Grant Numbers: NS01863, AG10963, NS36519, CUR410773
- Paul Beeson Physician Faculty Scholars in Aging Research Award
- Huntington's disease;
- Clinical characteristics
The prevalence and clinical characteristics of dystonia in Huntington's disease (HD) have not been formally assessed.
To study (1) the prevalence of dystonia in HD in a clinic population, (2) the clinical features of dystonia, and (3) clinical correlates of dystonia (for example, age, disease duration).
Patients with HD attending the HD Center at the New York State Psychiatric Center were administered the Unified HD Rating Scale and underwent a standardized 5.5-minute videotaped examination. Two neurologists reviewed the videotaped examination and rated the severity and constancy of dystonia, calculating a total dystonia score for each patient.
Prevalence of dystonia of any severity was 95.2%. Twenty-four of 42 (57.1%) had dystonia in at least one body region that was moderate and present more than half of the time, and seven of 42 (16.7%) had dystonia that was severe and constant. The most prevalent types of dystonia were internal shoulder rotation (64.3%), sustained fist clenching (47.1%), excessive knee flexion (42.9%), and foot inversion (42.9%). In 37 of 42 (88.1%) patients, there were more than two types of dystonia, and in the average patient, three to four types of dystonia. The mean severity was between 1 (mild) and 2 (moderate), and the mean constancy was between 2 (present less than half of the time) and 3 (present more than half of the time). Multivariate linear regression revealed that disease duration (p = 0.0005) and taking an antidopaminergic agent (p = 0.03) were positively associated with the total dystonia score.
The majority of patients in this HD clinic exhibited some dystonia. The dystonia was present in several body regions and manifested by a variety of movements and postures not typical of idiopathic torsion dystonia. The dystonia was not bothersome to most patients, and its severity was a function of disease duration and use of an antidopaminergic agent.