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Published on: March 11, 2020
Tapping linked to function and structure in premanifest and symptomatic Huntington disease
N Bechtel1, R I Scahill, H D Rosas
1Department of Neurology, University of Münster, Münster, Germany. r.reilmann@uni-muenster.de
Neurology
|November 12, 2010
Summary
Force-transducer-based tapping objectively quantifies Huntington
Area of Science:
- Neuroscience
- Neurology
- Biomedical Engineering
Background:
- Huntington disease (HD) motor signs are disabling, with current assessment scales lacking objectivity and sensitivity, especially in premanifest stages.
- There is a critical need for precise, reliable measures to facilitate clinical trials in premanifest Huntington disease populations.
- The Total Motor Score (TMS) of the Unified Huntington's Disease Rating Scale (UHDRS) is limited by interrater variability and insensitivity in early disease stages.
Purpose of the Study:
- To hypothesize that force-transducer-based tapping can objectively quantify motor deficits in Huntington disease.
- To investigate the correlation between tapping performance, disease burden, and brain atrophy in gene carriers.
- To assess the potential of tapping tasks for detecting early motor changes in premanifest Huntington disease.
Main Methods:
- A multicenter study (TRACK-HD) involving 123 controls, 120 premanifest, and 123 early symptomatic Huntington disease gene carriers.
- Participants performed speeded and metronome tapping tasks using force transducers.
- Data collected included Total Motor Score (TMS), CAG repeat length, and MRI scans; analyses were performed blinded.
Main Results:
- Tapping variability successfully distinguished between all groups and subgroups in both tasks.
- Tapping deficits correlated significantly with disease burden, clinical motor phenotype, gray and white matter atrophy, and cortical thinning.
- Speeded tapping demonstrated higher sensitivity in detecting early motor changes associated with Huntington disease.
Conclusions:
- Motor deficits, measurable by tapping variability, are present throughout premanifest and manifest stages of Huntington disease.
- These deficits worsen with disease progression and correlate with clinical severity and regional brain atrophy, linking structure and function.
- Force-transducer-based tapping shows promise for prospectively tracking motor phenotype progression in Huntington disease.
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