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Muscle tone in Huntington's disease
1Neurological Therapy Center, Heinrich Heine University, Düsseldorf, Germany.
Journal of the Neurological Sciences
|February 1, 1994
Summary
Huntington's disease (HD) can cause increased muscle tone, particularly in elbow flexion, which worsens with disease duration. This hypertonic pattern is common in advanced HD stages and independent of medication or cognitive decline.
Area of Science:
- Neurology
- Movement Disorders
- Muscle Physiology
Background:
- Huntington's disease (HD) is a progressive neurodegenerative disorder.
- Muscle tone abnormalities are reported in HD, but quantitative data are limited.
Purpose of the Study:
- To quantitatively assess muscle tone in patients with Huntington's disease.
- To characterize the pattern of tone changes and its relationship to disease progression and other clinical features.
Main Methods:
- Quantitative evaluation of elbow flexion and extension resistance using a hydraulic testing device in 39 HD patients.
- Analysis of tone changes in relation to disease duration, age, chorea severity, cognitive impairment, and medication.
Main Results:
- A velocity-dependent increase in resistance was observed, predominantly in elbow flexion.
- Increased muscle tone correlated with disease duration but not with age, chorea, or cognitive impairment.
- Hypertonicity was present even in unmedicated patients and unrelated to neuroleptic treatment.
Conclusions:
- Increased muscle tone, characterized by velocity-dependent resistance to flexion, is a frequent feature in advanced Huntington's disease.
- This pattern of hypertonia differs from hemispasticity and Parkinson's disease.
- Muscle tone changes in HD are linked to disease progression and are not solely medication-induced.