Aerobic training in patients with anoctamin 5 myopathy and hyperckemia. Issue 1 (5th May 2014)

• Record Type: Journal Article
• Authors: Vissing, Christoffer R.; Preisler, Nicolai; Husu, Edith; Prahm, Kira P.; Vissing, John
• Is Part Of: Muscle & nerve. Volume 50:Issue 1(2014:Jul.)
• Page Start: 119
• Page End: 123
• Publication Date: 2014-05-05
• Subjects: Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.24112
• Languages: English
• ISSNs: 0148-639X

Abstract

<abstract abstract-type="main"> <title>ABSTRACT</title> <p> <italic>Introduction</italic>: Anoctamin 5 deficiency has recently been defined to cause limb‐girdle muscular dystrophy type 2L (LGMD2L) with pronounced hyperCKemia. No treatment interventions have been made so far in this condition. <italic>Methods</italic>: In 6 patients with LGMD2L, we studied the effect of home‐based, pulse‐watch monitored, moderate‐intensity exercise on a cycle ergometer for 30 minutes, 3 times weekly, for 10 weeks. Plasma creatine kinase (CK) was assessed before, during, and after the program as a marker of muscle damage. Primary outcome measures were maximum oxygen uptake (VO_2max) and time in the 5‐repetitions‐sit‐to‐stand test (FRSTST). <italic>Results</italic>: Training resulted in improvements in VO_2max (27 ± 7%; <italic>P</italic> = 0.0001) and FRSTST time (35 ± 12%; <italic>P</italic> = 0.007). Improvements in physiologic and functional muscle testing were accompanied by stable CK levels and no reports of adverse effects. <italic>Conclusions</italic>: These findings suggest that supervised aerobic exercise training is safe and effective in improving oxidative capacity and muscle function in patients with anoctamin 5 deficiency. <italic>Muscle Nerve</italic><bold>50</bold>: 119–123, 2014</p> </abstract>