We have developed a protocol to evaluate, prospectively, patients with spinal muscular atrophy (SMA) so that the natural course may be defined and treatment efficacy measured. A fixed myometry system with a sensitivity of 0.5 kgs. of force was used to eliminate the subjectivity experienced with manual muscle testing and handheld dynamometry. Thirteen patients were evaluated weekly for twelve weeks by the clinical evaluators (CEs). Intrarater reliability was established by demonstrating that repeated measurements by the same examiner were consistent. The CEs examined patients at each of the participating institutions on three separate occasions. Interrater reliability was demonstrated by showing that the strength evaluations made on the same subject by different evaluators did not differ significantly. The fixed myometry system was found to be a highly reliable method for evaluating very weak muscle groups in patients with SMA.
- Quantitative muscle strength assessment
- Spinal muscular atrophy
ASJC Scopus subject areas
- Clinical Neurology