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...

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Autores: Febrer, A, Rodriguez, N, Alias, L, Tizzano, E
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2010
País:España
Recursos:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repositorio:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:oai:iibsantpau.fundanetsuite.com:p12834
Acesso em linha:https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12834
Access Level:acceso abierto
Palavra-chave:spinal muscular atrophy
muscle strength
weakness
dynamometry
SMN2 copies
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spelling MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHYFebrer, ARodriguez, NAlias, LTizzano, Espinal muscular atrophymuscle strengthweaknessdynamometrySMN2 copiesObjective: 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.FOUNDATION REHABILITATION INFORMATION2010info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12834JOURNAL OF REHABILITATION MEDICINEISSN: 16501977ISSNe: 16512081reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pauinstname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)Inglésinfo:eu-repo/semantics/openAccessoai:iibsantpau.fundanetsuite.com:p128342026-06-14T12:41:47Z
dc.title.none.fl_str_mv MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
title MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
spellingShingle MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
Febrer, A
spinal muscular atrophy
muscle strength
weakness
dynamometry
SMN2 copies
title_short MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
title_full MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
title_fullStr MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
title_full_unstemmed MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
title_sort MEASUREMENT OF MUSCLE STRENGTH WITH A HANDHELD DYNAMOMETER IN PATIENTS WITH CHRONIC SPINAL MUSCULAR ATROPHY
dc.creator.none.fl_str_mv Febrer, A
Rodriguez, N
Alias, L
Tizzano, E
author Febrer, A
author_facet Febrer, A
Rodriguez, N
Alias, L
Tizzano, E
author_role author
author2 Rodriguez, N
Alias, L
Tizzano, E
author2_role author
author
author
dc.subject.none.fl_str_mv spinal muscular atrophy
muscle strength
weakness
dynamometry
SMN2 copies
topic spinal muscular atrophy
muscle strength
weakness
dynamometry
SMN2 copies
description 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.
publishDate 2010
dc.date.none.fl_str_mv 2010
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12834
url https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12834
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv FOUNDATION REHABILITATION INFORMATION
publisher.none.fl_str_mv FOUNDATION REHABILITATION INFORMATION
dc.source.none.fl_str_mv JOURNAL OF REHABILITATION MEDICINE
ISSN: 16501977
ISSNe: 16512081
reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
instname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
instname_str Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
reponame_str r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
collection r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
repository.name.fl_str_mv
repository.mail.fl_str_mv
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