Use of hand-held dynamometry in the evaluation of lower limb muscle strength in people with Huntington’s disease

Purpose Sub-clinical muscle involvement, including myopathic changes and mitochondrial dysfunction of skeletal muscle, has been reported in people with Huntington’s disease (HD). Muscle strength was evaluated using a hand-held dynamometer. Reliability and validity in people with HD were determined....

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Bibliographic Details
Published in:Journal of neurology 2008-10, Vol.255 (10), p.1534-1540
Main Authors: Busse, M. E., Hughes, G., Wiles, C. M., Rosser, A. E.
Format: Article
Language:English
Subjects:
Biological and medical sciences
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Humans
Huntington Disease - physiopathology
Huntingtons disease
Isometric Contraction
Leg
Male
Medical sciences
Medicine
Medicine & Public Health
Middle Aged
Muscle Strength
Muscle Strength Dynamometer
Neurological disorders
Neurology
Neuroradiology
Neurosciences
Original Communication
Validation studies
Validity
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Summary:Purpose Sub-clinical muscle involvement, including myopathic changes and mitochondrial dysfunction of skeletal muscle, has been reported in people with Huntington’s disease (HD). Muscle strength was evaluated using a hand-held dynamometer. Reliability and validity in people with HD were determined. Method Isometric muscle strength of 6 lower limb muscle groups was measured in 20 people with HD and matched healthy controls. People with HD were evaluated with the Unified Huntington’s Disease Rating Scales (UHDRS). Within session reliability using intra-class correlation coefficients (ICC) was calculated. Discriminant and convergent validity was also evaluated. Results UHDRS motor scores of people with HD ranged from 28 to 80. Reliability of strength testing was excellent (ICC 0.86 to 0.98). People with HD had on average about half the strength of healthy matched controls. UHDRS motor scores and strength scores were significantly correlated (convergent) providing a further indication of validity of strength testing. Conclusions The hand-held dynamometer is a reliable and valid measurement tool to detect strength differences between people with HD and a matched control group. There is significant reduction in lower limb muscle strength in HD which does not appear to have been described previously.
ISSN:0340-5354
1432-1459
DOI:10.1007/s00415-008-0964-x