The effect of motor learning in facioscapulohumeral muscular dystrophy patients.

Two models of motor learning (ML) practice (complex and simple) were used to investigate upper limb function in individuals with facioscapulohumeral dystrophy (FSHD). The effect of ML practice was studied by examining changes in the ability to undertake a simulated drinking task. In the complex model, seven FSHD patients and seven age- and gender-matched healthy volunteers were studied. Performance was assessed by measurement of the electromyographic (EMG) activity of the biceps brachii and brachioradialis, elbow joint flexion, shoulder joint flexion and abduction, maximum acceleration at the onset of movement, movement time and reaction time, both before and after a 30-min complex ML task. In the simple model, a second group of six FSHD patients and six age- and gender-matched healthy subjects were studied. The same parameters were measured as for the complex ML task, except that the EMG activity of the triceps and deltoid muscles (anterior part) were measured instead of that of the brachioradialis. In both studies, the FSHD patients showed significantly larger values for all parameters except the flexion of shoulder joint and reaction time, compared with controls before the ML task. In the FSHD group, while the complex ML resulted in decreases in the brachioradialis EMG activity (P<0.005) and reaction time (P<0.0001), the simple ML model resulted in significant changes towards the normal value in all parameters measured except shoulder flexion. The change in the measured variables towards normal values indicates that ML may help to improve performance in FSHD.