Original Title: Eficacia, efectividad y seguridad de las terapias físicas en las enfermedades neuromusculares. Revisión sistemática

Authors' objectives: The aim of this review has been to identify and assess the evidence on the efficiency, effectiveness and safety of strength physical training and aerobic exercise, as well as other physical therapies prescribed and performed in the healthcare setting.

Authors' results and conclusions: Evidence indicates that strength training alone could have little or no effect, and that training based only on aerobic exercises, can produce a possible improvement in the aerobic capacity, though only in participants suffering from Facioscapulohumeral dystrophy (FSHD). The combination of aerobic exercise and strength training could produce slight increase in muscle strength gain and aerobic capacity improvement in patients suffering from dermatomyositis and polymyositis, as well as a slight decrease in aerobic capacity and increase in muscular strength gain for patients suffering from juvenile dermatomyositis. Evidence on the effects of physical training on walking ability, cardiopulmonary capacity, fatigue, quality of life, functional performance or muscle strength in a combined program consisting of strength and aerobic exercise on bicycle ergometer in patients suffering from type 3 spinal muscular atrophy which are between 10 and 48 years old, was too uncertain to draw conclusions. There were no study-related serious adverse events. In patients diagnosed with Duchenne muscular dystrophy (DMD), respiratory muscular training could be beneficial to improve endurance and / or respiratory muscle strength. Positive effects can be observed, but no statistical significance was reached. Currently, therefore, there is no clear evidence in favour or against the use of inspiratory muscle training in children and adolescents suffering from DMD. The effect of joint mobility and contraction exercise programs analyzed in patients with DMD, myotonic dystrophy and FSHA, in the review of Gianola S et al., 2013 (critically low quality) generates a positive trend, though not significant, in muscle strength. Allthough the effects were consistent, the benefits were limited, and the possibility of the detrimental effect remains. Some training programs in patients with type 1 myotonic dystrophy, have reported improvements on muscle strength or functionality of isolated muscles or muscle groups, although the variability of the used therapies limits the result synthesis. Inspiratory muscle training with threshold in patients with Pompe Disease increase pressures generated during the inspiration (MIP). Higher quality studies and a larger sample size studies are needed to confirm the effectiveness of the programs.

Authors' methods: A literature review was carried out in order to find outcomes identified in a panel developed with Delphi technique.A critical appraisal of the systematic reviews has been performed, aiming to identify methodological problems which could affect its validity, as well as a descriptive synthesis of primary research, without a critical appraisal, to respond to patients’ demands and the association that requested this report.

Authors' identified further research: Further research is needed to establish whether the different therapies used could benefit all forms of neuromuscular disorders and thus be able to define the optimal physical activity protocols for these people.

Project Status: Completed

Year Published: 2020

URL for published report: https://www.aetsa.org/publicacion/eficacia-efectividad-y-seguridad-de-las-terapias-fisicas-en-las-enfermedades-neuromusculares-revision-sistematica/

English language abstract: An English language summary is available

Publication Type: Mini HTA

Country: Spain

Organisation Name: Andalusian Health Technology Assessment Area

Contact Address: Area de Evaluacion de Tecnologias Sanitarias Sanitarias de Andalucia (AETSA) Avda. Innovación, s/n Edificio Arena 1. Sevilla (Spain) Tel. +34 955 006 309

Contact Name: aetsa.csalud@juntadeandalucia.es

Contact Email: aetsa.csalud@juntadeandalucia.es

Copyright: Andalusian Agency for Health Technology Assessment (AETSA)