Figure 1

Muscle function patterns of 15 assessed patients with SMA types 2–4. Individual values are presented as the mean of both sides (with the exception of neck flexion und extension) and sorted by maximum total MRC % at baseline (a) or month 14 (b). Patients are represented by columns on the x-axis. Patients with minor muscle strength grouped on the far left. Columns on the y-axis represent the impairment pattern of specific muscle functions through the study population. Green (100%) corresponds to the absence of muscle weakness and red (0%) to plegia. Lines underneath the heatmaps indicate the corresponding distribution of SMA subtypes, ambulatory status and SMN2 copies. (a) The impairment pattern at baseline shows a main involvement of proximal muscles, while distal muscle weakness was only visible in more progressed patients. (b) After 14 months on treatment, a trend towards regaining proximal muscle strength was seen, predominantly in the upper limbs.