Article

Friedreich's Ataxia (FA) is a devastating, progressive,
neurodegenerative disease. Objective measures that detect changes in neurological
function in FA patients are needed to facilitate therapeutic clinical trials. The
purpose of this pilot study was to analyze longitudinal changes in gait and
balance in subjects with FA using the GAITRite Walkway System(R) and Biodex
Balance System, respectively, and to test the ability of these measures to detect
change over time compared to the Friedreich's Ataxia Rating Scale (FARS).
METHODS: This was a 24-month longitudinal study comparing ambulatory FA subjects
with age- and gender-matched, healthy controls. Eight FA subjects and 8 controls
were tested at regular intervals using the GAITRite and Biodex Balance systems
and the FARS. RESULTS: In the FA group, comfortable and fast gait velocity
declined 8.0% and 13.9% after 12 months and 24.1% and 30.3% after 24 months,
respectively. Postural stability indices increased in FA subjects an average of
41% from baseline to 24 months, representing a decline in balance. Subjects with
FA also demonstrated a 17.7% increase in FARS neurological exam scores over 24
months. There were no changes in gait or balance variables in controls. In the FA
group, multiple gait and balance measures correlated significantly with FARS
neurological exam scores. CONCLUSIONS: The GAITRite and Biodex Balance systems
provided objective and clinically relevant measures of functional decline in
subjects with FA that correlated significantly with performance measures in the
FARS. Gait velocity may be an important objective measure to identify disease
progression in adults with FA.
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Langue : ANGLAIS