Factors Influencing the Severity and Progression of Respiratory Muscle Dysfunction in Myotonic Dystrophy Type 1

We examined various parameters of respiratory functional status, and found FVC (% predicted) correlated best with other measures of disease severity. Annual change in FVC was −1.42 (std error = 0.381). Greater CTG repeat size, higher MIRS rating, and longer disease duration were all correlated with lower baseline FVC but not with annual rate of change. Wide variability in clinical phenotype made determination of disease measures directly related to respiratory functional decline challenging.
Source: Frontiers in Neurology - Category: Neurology Source Type: research