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Mov Disord. 2019 Oct 14. doi: 10.1002/mds.27866. [Epub ahead of print]

How different aspects of motor dysfunction influence day-to-day function in huntington's disease.

Author information

1
Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, Michigan, USA.
2
Institute for Social Research, University of Michigan, Ann Arbor, Michigan, USA.
3
Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA.
4
Department of Neurology, Radiology, Neuroscience, Physical Therapy and Occupational Therapy, Washington University in St. Louis, St. Louis, Missouri, USA.
5
Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
6
Department of Psychiatry, Rutgers-Robert Wood Johnson Medical School, Piscataway, New Jersey, USA.
7
Department of Pathology, Rowan-SOM (School of Medicine), Stratford, New Jersey, USA.
8
School of Psychological Sciences, Monash University, Clayton, Victoria, Australia.
9
Department of Psychiatry, Neurology, and Psychological and Brain Sciences, The University of Iowa, Iowa City, Iowa, USA.
10
Department of Medical Social Sciences, Northwestern University, Chicago, Illinois, USA.

Abstract

PURPOSE:

This study examined the relationships between different aspects of motor dysfunction (chorea, dystonia, rigidity, incoordination, oculomotor dysfunction, dysarthria, and gait difficulties) and functional status in persons with Huntington's disease.

METHODS:

A total of 527 persons with Huntington's disease completed the Unified Huntington's Disease Rating Scale motor, total functional capacity, and functional assessments.

RESULTS:

Confirmatory factor analysis indicated that a 4-factor model provided a better model fit than the existing 5-factor model. Exploratory factor analysis identified the following 4 factors from the motor scale: dystonia, chorea, rigidity, and a general motor factor. Regression indicated that dystonia (β = -0.47 and -0.79) and rigidity (β = -0.28 and -0.59) had strong associations with function, whereas chorea had modest correlations (β = -0.16 and -0.15).

CONCLUSIONS:

Dystonia and rigidity have stronger relationships with functional status than chorea in persons with Huntington's disease. The findings underscore the need for further research regarding the effects of dystonia and rigidity on functioning. © 2019 International Parkinson and Movement Disorder Society.

KEYWORDS:

HDQLIFE; Health-related quality of life; Huntington's disease; chorea; dystonia; motor function

PMID:
31609508
DOI:
10.1002/mds.27866

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