Abstract
De Wel et al. conducted a prospective study to evaluate prognosis in 24 patients with limb-girdle muscular dystrophy autosomal recessive type 12 (LGMDR12).1 They prepared 24 healthy controls by way of an age- and sex-matching procedure. Biodex(R) isometric dynamometry showed a significant decrease of muscle strength in the right quadricep muscles for all patients after 1 year. In contrast, outcomes from the 6-minute walk distance, 10-meter walk test, and Medical Research Council sum scores did not change significantly, even after 2 years. The authors recognized that thigh muscle proton density fat fraction imaging was a sensitive indicator to track progressive muscle fat replacement.
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