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Assessment of Sensorimotor Function in Mouse Models of Parkinson's Disease
10:32

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Published on: June 17, 2013

Motor disorder in Huntington's disease begins as a dysfunction in error feedback control.

M A Smith1, J Brandt, R Shadmehr

  • 1Department of Biomedical Engineering, Behavioral Sciences, Johns Hopkins University, Baltimore, Maryland 21205-2195, USA. msmith@bme.jhu.edu

Nature
|February 17, 2000
PubMed
Summary

Huntington's disease (HD) causes motor dysfunction. Early signs include jerky movements and poor error correction, detectable years before clinical symptoms manifest in gene carriers.

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Area of Science:

  • Neuroscience
  • Motor Control
  • Human Movement Analysis

Background:

  • Huntington's disease (HD) is characterized by progressive motor dysfunction.
  • The precise origins of motor control disturbances in HD remain unclear.
  • Understanding early motor deficits is crucial for timely intervention.

Purpose of the Study:

  • To investigate motor control deficits in asymptomatic HD gene-carriers (AGCs) and manifest HD patients.
  • To identify early indicators of HD progression in reaching movements.
  • To elucidate the role of error correction in HD-related motor dysfunction.

Main Methods:

  • Analysis of reaching movements in AGCs, manifest HD patients, and healthy controls.
  • Quantification of movement jerkiness, variability, and corrective responses.
  • Assessment of sensitivity to self-generated and externally-generated movement errors.

Main Results:

  • Elevated movement jerkiness, a marker of motor dysfunction, was observed in a significant proportion of AGCs, even years before predicted disease onset.
  • Movement termination was more impaired and variable than initiation in HD subjects.
  • HD subjects exhibited heightened sensitivity to early movement errors and disturbed corrective responses to external perturbations.

Conclusions:

  • A dysfunction in motor error correction is a key characteristic of early Huntington's disease, detectable presymptomatically.
  • This error correction deficit contributes to the observed jerkiness and impaired movement termination in HD.
  • Motor control abnormalities in HD can be identified years before clinical diagnosis and worsen with disease progression.