Measurement of muscle strength with a handheld dynamometer in patients with chronic spinal muscular atrophy.
Por:
Febrer A, Rodriguez N, Alias L and Tizzano E
Publicada:
1 mar 2010
Resumen:
OBJECTIVE: To measure muscle strength in patients with spinal muscular atrophy using a handheld dynamometer as an objective tool to evaluate the progression of disease and the outcome of therapeutic trials. DESIGN: Maximum voluntary isometric contraction was measured in a group of 24 patients aged 5-38 years with types II and III spinal muscular atrophy. Four muscle groups were examined. Data were grouped according to age and sex. Comparison was made between spinal muscular atrophy types; ambulatory vs non-ambulatory, and survival motor neuron (SMN)2 copies. The results were compared with those of a healthy reference population. RESULTS: Muscle strength was much lower in patients with spinal muscular atrophy than in the healthy population. The walkers group yielded higher values than patients who were non-walkers. Knee extensors were the weakest muscles in both groups, regardless of the ability to walk. The greatest differences were found between ambulatory and non-ambulatory patients. Non-walkers type III patients showed lower values, similar to those for type II patients. Patients with 3 and 4 SMN2 copies showed higher strength with respect to those with 2 SMN2 copies, although not statistically significant. CONCLUSION: The handheld dynamometer is a valid tool for measuring muscle strength in patients with spinal muscular atrophy. It can be used to measure disease progression and to evaluate changes in therapeutic trials.
Filiaciones:
Febrer A:
Service of Physical Medicine and Rehabilitation, University Hospital Sant Joan de Deu, Barcelona, Spain
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