Article

Myotonic dystrophy (DM1) is a multisystemic disorder characterized by myotonic muscular weakness, and numerous organ impairments, especially cardiac
and respiratory disorders. The goal of this study was to evaluate in DM1 patients
the relationships between a new muscular disability scale, the motor function
measure (MFM), and functional measurements of organ involvements. The MFM and MDRS, as well as spirometry, blood gases, echocardiography,
electrocardiogram, and ophthalomological examination were performed in 69
consecutive DM1 patients. RESULTS: Significant relationships were found between
MDRS and MFM (p < 0.001) as well as between both and age and BMI of the patients.
Patients with total MFM below 76 had a higher risk of respiratory insufficiency,
conduction disorders, left ventricular dysfunction, hypoxemia and cataract than
the other DM1 patients. CONCLUSION: The MFM provides an effective evaluation of
muscular disability in DM1 patients. Cardiac and respiratory involvements are
correlated with lower MFM.
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Langue : FRANCAIS