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Rehabilitation Mnagement of Friedreich Ataxia: Lower Extremity Force-Control Variability and Gait Performance

Physical Therapy Section mlove{at}nih.gov

Karen Lohmann Siegel

Rehabilitation Medicine Department, Warren G. Magnuson Clinical Center, and the Physical Disabilities Branch

Scott M. Paul

Physical Therapy Section and Medical Section

Kimberly Benson

National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland, and the Program in Physical Therapy, Bouvé College of Health Sciences, Northeastern University, Boston, Massachusetts

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.

Key Words: Friedreich ataxia • Force control • Rehabilitation

Neurorehabilitation and Neural Repair, Vol. 18, No. 2, 117-124 (2004)
DOI: 10.1177/0888439004267241


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