Motor neuron diseases

Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders

Abstract Background Chronic alveolar hypoventilation is a common complication of many neuromuscular and chest wall disorders. Long-term nocturnal mechanical ventilation is commonly used to treat it. This is a 2014 update of a review first published in 2000 and previously updated in 2007. Objectives To examine the effects on mortality of nocturnal mechanical ventilation in […]

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Mechanical insufflation-exsufflation for people with neuromuscular disorders

Abstract Background People with neuromuscular disorders (NMDs) may have weak respiratory (breathing) muscles which makes it difficult for them to effectively cough and clear mucus from the lungs. This places them at risk of recurrent chest infections and chronic lung disease. Mechanical insufflation-exsufflation (MI-E) is one of a number of techniques available to improve cough […]

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Passive movements for the treatment and prevention of contractures

Abstract Background Contractures, a common complication following immobility, lead to restricted joint range of motion. Passive movements (PMs) are widely used for the treatment and prevention of contractures; however, it is not clear whether they are effective. Objectives The aim of this review was to determine the effects of PMs on persons with contractures or […]

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Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease

Abstract Background Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is the most common neurodegenerative disorder of the motor system in adults. Pain in ALS is a frequent symptom especially in the later stages of disease and can have a pronounced influence on quality of life and suffering. Treatment of pain therefore […]

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Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease

Abstract Background Despite the high incidence of muscle weakness in individuals with amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND), the effects of exercise in this population are not well understood. This is an update of a review first published in 2008. Objectives To systematically review randomised and quasi-randomised studies of exercise for people […]

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Repetitive transcranial magnetic stimulation for the treatment of amyotrophic lateral sclerosis or motor neuron disease

Abstract Background Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a progressive neurodegenerative disease without effective therapies. Several studies have suggested that repetitive transcranial magnetic stimulation (rTMS) may have positive benefit in ALS. However, the efficacy and safety of this therapy remain uncertain. This is the first update of a review […]

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Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease

Abstract Background Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common cause of death, which usually occurs within two to five years of the disease onset. Supporting respiratory function with mechanical ventilation may improve survival and quality of life. This is the […]

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Creatine for amyotrophic lateral sclerosis /motor neuron disease

Abstract Background Creatine, a naturally-occurring nitrogenous organic acid involved in adenosine triphosphate (ATP) production, has been shown to increase survival in mouse models of amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND). Results from human trials, however, have been mixed. Given conflicting results regarding the efficacy of creatine, we conducted a systematic review, which […]

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Recombinant human insulin-like growth factor I (rhIGF-I) for the treatment of amyotrophic lateral sclerosis/motor neuron disease

Abstract Background Recombinant human insulin-like growth factor I (rhIGF-I) is a possible disease modifying therapy for amyotrophic lateral sclerosis (ALS, which is also known as motor neuron disease (MND)). Objectives To examine the efficacy of rhIGF-I in affecting disease progression, impact on measures of functional health status, prolonging survival and delaying the use of surrogates […]

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Treatment for cramps in amyotrophic lateral sclerosis /motor neuron disease

Abstract Background Cramps are painful, involuntary muscle contractions. They commonly affect people with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) at all stages of the disease. To date, the treatment of muscle cramps in ALS has been largely empirical without any evidence from randomised controlled trials. Objectives To systematically assess the effect of interventions on muscle […]

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