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Muscle disease characterized by slowly progressive weakness of the muscles of the face, upper-arm, and shoulder.

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Facioscapulohumeral muscular dystrophy is a progressive muscle disease. Muscle weakness is often relatively mild and progression slow but around one fifth of affected people eventually become wheelchair‐bound. The muscles of the face, shoulder blades and upper arms are most severely effected, but weakness occurs in other muscles. There is no agreed treatment. Only two randomised controlled trials have been published. One small trial of albuterol (also known as salbutamol)and another small trial of creatine (a dietary supplement for building muscle) were inadequate to confirm or refute a significant effect. Further trials of albuterol, creatine and other agents are needed.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2008

Winging of the scapula (shoulder blade) in muscular dystrophy is caused by weakness of the muscles which attach the scapula to the chest and help raise the arms. Non‐surgical interventions involve casts or slings to support the shoulders. Surgical operations involve fixing the scapulae to the ribs using screws or wire. No randomised controlled trials were found. Small case series suggest that surgical operations might produce significant benefits in some people. These need to be balanced against loss of mobility, need for physiotherapy and possible complications including failure of the fixing material or reduced breathing capacity.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2011

Strength training, which is performed to improve muscle strength and muscle endurance, or aerobic exercise programmes, which are designed to improve cardiorespiratory endurance, might optimise physical fitness and prevent additional muscle wasting in people with muscle disease. However, people with muscle disease and some clinicians are still afraid of overuse and have a cautious approach to training. This updated review (most recent date of search 2 July 2012) included two eligible trials of strength training in people with facioscapulohumeral muscular dystrophy (FSHD) and myotonic dystrophy (101 participants), two trials of strength training combined with aerobic exercise in people with mitochondrial myopathy (18 participants) and myotonic dystrophy type I (35 participants) and one trial of aerobic exercise in people with polymyositis and dermatomyositis (14 participants). These trials showed that moderate‐intensity strength training in people with myotonic dystrophy or with FSHD, and aerobic exercise training in people with dermatomyositis or polymyositis appear not to harm muscles. Strength training combined with aerobic exercise appears to be safe in myotonic dystrophy type I and may be effective in increasing endurance in people with mitochondrial myopathy. Evidence suggests that strength training is not harmful in people in FSHD, myotonic dystrophy, mitochondrial disorders and dermatomyositis and polymyositis, but further research is needed to determine potential benefit.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

The study found limited evidence on the effectiveness of the various types of orthoses used within the NHS to manage patients with neuromuscular and central nervous system conditions, especially in relation to the outcomes that are important to patients.

Health Technology Assessment - NIHR Journals Library.

Version: July 2016

Systematic Reviews in PubMed

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