MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
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...
| Authors: | , , , |
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| Format: | article |
| Status: | Published version |
| Publication Date: | 2010 |
| Country: | España |
| Institution: | Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau) |
| Repository: | r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau |
| OAI Identifier: | oai:iibsantpau.fundanetsuite.com:p12834 |
| Online Access: | https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12834 |
| Access Level: | Open access |
| Keyword: | spinal muscular atrophy muscle strength weakness dynamometry SMN2 copies |
| Summary: | 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. |
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