Rehabilitation management of Friedreich ataxia: lower extremity force-control variability and gait performance

Neurorehabil Neural Repair. 2004 Jun;18(2):117-24. doi: 10.1177/0888439004267241.

Abstract

We describe the rehabilitation management during a 12-month period of a 14-year-old female with Friedreich ataxia. Interventions included task-oriented bimanual reaching activities, functional strengthening, and gait training using a walker featuring tension-controlled wheels and a reverse-braking system. Her physical status was assessed with the Nine-Hole Peg Test, single limb stance time, manual muscle testing, self-reported falls, isometric force control testing, and 3-dimensional gait analysis in a motion-capture laboratory. Although measures of the patient's Nine-Hole Peg Test, single limb stance time, and manual muscle testing reflected minimal changes, her gait speed decreased by 69.4%. However, the force-control targeting of her dominant knee extensors showed a 43.7% increase in force variability that was concomitant with her decline in gait performance. The decrement of her initial gait speed was reduced to 42.9% on replacing the wheeled walker with the U-Step Walking Stabilizer at the end of the intervention period. Although the patient's gait remained significantly impaired, extended use of the U-Step Walking Stabilizer modestly improved her gait performance, and her rate of falls decreased from 10 to 3 per month. Our observations suggest that use of force-control testing as proxy measures of ataxia and tension-controlled gait aids show promise in the management of Friedreich ataxia and merit further investigation.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Exercise Therapy / methods*
  • Female
  • Friedreich Ataxia / rehabilitation*
  • Gait / physiology*
  • Humans
  • Isometric Contraction / physiology
  • Leg / physiopathology*
  • Motor Activity / physiology*
  • Task Performance and Analysis
  • Walkers
  • Weight-Bearing / physiology*