title: Rehabilitation management of Friedreich ataxia: lower extremity force-control variability and gait performance.
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.
authors.affiliation: Physical Therapy Section, Rehabilitation Medicine Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland, USA. mlove@nih.gov
authors.forename: Michael O
authors.initials: MO
authors.lastname: Harris-Love
authors.forename: Karen Lohmann
authors.initials: KL
authors.lastname: Siegel
authors.forename: Scott M
authors.initials: SM
authors.lastname: Paul
authors.forename: Kimberly
authors.initials: K
authors.lastname: Benson
mesh_terms: D000293:Adolescent; D005081:Exercise Therapy / Q000379:methods*; D005260:Female; D005621:Friedreich Ataxia / Q000534:rehabilitation*; D005684:Gait / Q000502:physiology*; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology; D007866:Leg / Q000503:physiopathology*; D009043:Motor Activity / Q000502:physiology*; D013647:Task Performance and Analysis; D014853:Walkers; D016474:Weight-Bearing / Q000502:physiology*