Motor Disorder in Huntington's Disease Begins as a Dysfunction in Error Feedback Control
Access StatusFull text of the requested work is not available in DASH at this time ("dark deposit"). For more information on dark deposits, see our FAQ.
MetadataShow full item record
CitationSmith, Maurice A., Jason Brandt, and Reza Shadmehr. 2000. Motor disorder in Huntington's disease begins as a dysfunction in error feedback control. Nature 403(6769): 544-549.
AbstractA steady progression of motor dysfunction takes place in Huntington's disease1 (HD). The origin of this disturbance with relation to the motor control process is not understood. Here we studied reaching movements in asymptomatic HD gene-carriers (AGCs) and subjects with manifest HD. We found that movement jerkiness, which characterizes the smoothness and efficiency of motion, was a sensitive indicator of presymptomatic HD progression. A large fraction of AGCs displayed elevated jerk even when more than seven years remained until predicted disease onset. Movement termination was disturbed much more than initiation and was highly variable from trial to trial. Analysis of this variability revealed that the sensitivity of end-movement jerk to subtle, self-generated early-movement errors was greater in HD subjects than in controls. Additionally, we found that HD corrective responses to externally-generated force pulses were greatly disturbed, indicating that HD subjects display aberrant responses to both external and self-generated errors. Because feedback corrections are driven by error and are delayed such that they predominantly affect movement termination, these findings suggest that a dysfunction in error correction characterizes the motor control deficit in early HD. This dysfunction may be observed years before clinical disease onset and grows worse as the disease progresses.
Citable link to this pagehttp://nrs.harvard.edu/urn-3:HUL.InstRepos:32116864
- FAS Scholarly Articles