Muscle strength and motor function in children and adolescents with spinal muscular atrophy II and III

The aim of this study was to quantify isometric muscle strength and motor function in children and adolescents with spinal muscular atrophy (SMA) and to analyse the impact of reduced muscle strength on motor function. Six children and adolescents with SMA II and eight with SMA IlI were assessed regarding isometric muscle strength and motor function. Isometric muscle strength was tested with a myometer and the values obtained were compared with normative data. Motor function was videotaped and 20 movements were scored according to a three-point scale. All of the assessed children and adolescents with SMA II and SMA III showed reduced muscle strength, but there were great differences within the group. The typical pattern of muscle weakness in SMA, with proximal weakness greater than distal and the lower limbs more affected than the upper, was also seen in these children. The muscle weakness affected motor function in all assessed children. Walking, transfer from lying or sitting to the standing position and stair-climbing were possible in some of the children, despite marked reduction of muscle strength. The study increases our knowledge concerning the degree of muscle weakness in children with SMA and the impact of muscle weakness on motor function. The results increase our possibilities of understanding the prerequisites for everyday life in these children and planning therapeutic interventions. Repeated assessments with the methods used in this study may be used to monitor the course of the disease and to evaluate the efficacy of treatment.