Evidence for a disorder of locomotor timing in Huntington's disease

Abstract

Disturbances of walking have been described in people with Huntington's disease (HD), although the nature of the deficits have not yet been well defined. The purpose of this investigation was to determine whether people with HD have a deficit in the regulation of footstep timing during walking. The footstep patterns of 30 people with HD and 30 matched comparisons were measured at self-selected slow, preferred, and fast speeds. Subjects were also instructed to match their footsteps to auditory metronome cues set at 80 and 120 beats per minute. Gait speed, cadence, stride length, and double limb support as a percentage of the gait cycle were measured using a computerized foot-switch system. People with HD demonstrated a disorder in their ability to regulate cadence, manifest as a reduced step frequency when walking at preferred speed and when required to increase their speed. For all walking conditions, people with HD had increased variability of footstep cadence. They also had difficulty synchronizing their footstep timing to an auditory cue. For all walking conditions, people with HD had reduced stride length. Thus, in HD, there is a disorder in the regulation of footstep timing, with increased variability, a restricted cadence range, difficulty synchronizing footsteps to an auditory cue and reduced stride length. The exact neural correlates of this timing disorder are yet to be determined.

Wearable Sensors in Huntington Disease: A Pilot Study

“…This study demonstrates that gait cadence is also decreased when measured at home. In addition, the values of at-home steps per minute for controls and participants with HD are very similar to values measured in clinic [16][17][18][19].…”

“…Previous studies have also shown that gait cadence is decreased in HD through in-clinic evaluations [16][17][18][19]29]. This study demonstrates that gait cadence is also decreased when measured at home.…”

“…Q-Motor assessments have demonstrated utility in tracking longitudinal progression of motor impairment [12,13] and as a supplemental measure in clinical trials [14]. Likewise, force-sensitive insoles [15,16], video motion analysis systems [17], and pressure-sensitive walking mats [18][19][20] have been utilized to objectively measure impaired gait in Huntington disease. However, these gait-measuring technologies and Q-Motor assessments must be administered in clinic by trained personnel.…”

First use of creatine hydrochloride in premanifest Huntington disease

“…Implementation of a targeted multidisciplinary rehabilitation program into the Huntington's disease population is anticipated to reduce dependence on the health system due to a reduction in the utilisation of health services as a result of a reduction in fall-related injuries, and may delay placement into residential and end-stage facilities by encouraging and maintaining patient independence. Huntington's disease is characterised by progressive cognitive, affective and motor deterioration over a 15-25 year period, together with a spectrum of associated physical deficits including postural instability [6][7][8][9][10] , gait impairments [11][12][13][14][15][16] and lean and fat tissue loss 17,18 .…”

“…Volumetric muscle strength changes in the upper and lower body showed highly significant, progressive increases in total group muscle strength, and significant increases for female (p<0.03) and male (p<0.02) subgroups, in all exercises after 9 months intervention (p<0.0005; Figure 40). [21], and separately for female [11] and male [10] sub-groups to indicate gender response). Highly significant increases in muscle strength were achieved for each of the above exercises after 9 months intervention (p<0.0005).…”