Reader Response: Prospective Natural History Study in 24 Adult Patients With LGMDR12 Over 2 Years of Follow-up: Quantitative MRI and Clinical Outcome Measures

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.

Automated summary

De Wel et al. conducted a prospective study to evaluate the prognosis in patients with LGMDR12. Muscle strength decreased significantly in the right quadricep muscles after 1 year, as shown by Biodex(R) isometric dynamometry. However, measures such as 6-minute walk distance, 10-meter walk test, and Medical Research Council sum scores did not show significant changes, even after 2 years. Thigh muscle proton density fat fraction imaging was identified as a sensitive indicator for tracking progressive muscle fat replacement.