Table 1.
Description of the qualitative muscle grading scale (21).
Table 2.
Median qualitative grades for selected muscles at baseline and at 12 months.
Figure 1.
Quantitative fat fraction images of the thigh in LGMD2I patients showing the wide range of severity in fat replacement at baseline, from minimal involvement (a) to sparing of only gracilis and sartorius (e).
Table 3.
Median values of the fat fractions at baseline and follow up.
Figure 2.
Quantitative fat fraction differences from baseline to 12 month follow-up.
The box indicates the lower and upper quartiles, with the median change with time represented as the bar within the box. The bars enclose the outliers.
Figure 3.
Quantitative fat fraction images at baseline in an individual patient (a – mid lower leg level, c – mid thigh level) and at 12 months follow-up (b – mid lower leg level, d – mid thigh level).
Analysis reveals an increase in fat fraction of the medial and lateral gastrocnemius, peroneus longus, vastus lateralis and medialis, semimembranosus, semitendinosus, sartorius and gracilis.
Figure 4.
Quantitative fat fraction images at baseline in an individual patient (a – mid lower leg level, c – mid thigh level) and at 12 months follow-up (b – mid lower leg level, d – mid thigh level).
Analysis reveals an increase in fat fraction of the lateral and medial gastrocnemius, sartorius, gracilis, semitendinosus, rectus femoris and vastus lateralis.
Table 4.
Minimum, maximum and median values of myometry, timed tests and FVC at baseline and follow-up in 32 paired individuals.