Article

We evaluated the sensitivity of various rehabilitation and lung
function scales to detect differences between people with Charcot-Marie-Tooth
(CMT) disease and healthy controls. We also studied whether these measurements
are sensitive to disclose changes in patients with CMT disease after
rehabilitative treatment.
DESIGN : Eight patients with different types of CMT
participated in the study. Data were gathered at baseline;
at the end of the
treadmill training, stretching, respiratory, and proprioceptive exercise (TreSPE)
treatment period; and after a washout period of 6 mos. The following instruments
were used for data collection: Medical Research Council scale for lower limb
strength; Tinetti Balance Scale ; Physical Performance Battery; ankle angle,
oxygen consumption, and lung function tests; peak treadmill velocity and slope;
time to walk 6 m; and CMT Neuropathy Score. The participants underwent TreSPE
treatment twice every week for 8 wks. RESULTS: : All rehabilitative measures were
significantly worse in subjects with CMT disease than in healthy controls. Lung
function was always normal except for the maximum expiratory pressure and maximum
inspiratory pressure. No dropouts or worsening in any of the different outcome
measures were observed after TreSPE. The ankle angle and the time to walk 6 m
were the only measures that significantly improved after treatment. CONCLUSIONS : The rehabilitative outcome measures used in this protocol are useful in
detecting clinical impairment in people with CMT disease. Lung function tests
were confirmed to be minimally abnormal in patients with CMT disease. The TreSPE
treatment, besides being safe and well tolerated, induced some training effects
in CMT neuropathy, in the absence of lung function amelioration and effort
tolerance. Follow-up showed that CMT patients should be treated at least twice
every year because a regression of all outcome measures to the baseline state was
found after a 6-mo washout period.

Langue : ANGLAIS