Neuromuscular disorders

Cough augmentation techniques for people with chronic neuromuscular disorders

Abstract Background People with neuromuscular disorders may have a weak, ineffective cough predisposing them to respiratory complications. Cough augmentation techniques aim to improve cough effectiveness and mucous clearance, reduce the frequency and duration of respiratory infections requiring hospital admission, and improve quality of life. Objectives To determine the efficacy and safety of cough augmentation techniques […]

Share

Magnesium for skeletal muscle cramps

Abstract Background Skeletal muscle cramps are common and often occur in association with pregnancy, advanced age, exercise or motor neuron disorders (such as amyotrophic lateral sclerosis). Typically, such cramps have no obvious underlying pathology, and so are termed idiopathic. Magnesium supplements are marketed for the prophylaxis of cramps but the efficacy of magnesium for this […]

Share

Pharmacological treatment for familial amyloid polyneuropathy

Abstract Background Disease‐modifying pharmacological agents for transthyretin (TTR)‐related familial amyloid polyneuropathy (FAP) have become available in the last decade, but evidence on their efficacy and safety is limited. This review focuses on disease‐modifying pharmacological treatment for TTR‐related and other FAPs, encompassing amyloid kinetic stabilisers, amyloid matrix solvents, and amyloid precursor inhibitors. Objectives To assess and […]

Share

Pharmacological treatment for familial amyloid polyneuropathy

Abstract Background Disease‐modifying pharmacological agents for transthyretin (TTR)‐related familial amyloid polyneuropathy (FAP) have become available in the last decade, but evidence on their efficacy and safety is limited. This review focuses on disease‐modifying pharmacological treatment for TTR‐related and other FAPs, encompassing amyloid kinetic stabilisers, amyloid matrix solvents, and amyloid precursor inhibitors. Objectives To assess and […]

Share

Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome

Abstract Background Plasma exchange and intravenous immunoglobulin, but not corticosteroids, are beneficial in Guillain‐Barré syndrome (GBS). The efficacy of other pharmacological agents is unknown. This review was first published in 2011 and previously updated in 2013, and 2016. Objectives To assess the effects of pharmacological agents other than plasma exchange, intravenous immunoglobulin and corticosteroids for […]

Share

Drug treatment for spinal muscular atrophy types II and III

Abstract Background Spinal muscular atrophy (SMA) is caused by a homozygous deletion of the survival motor neuron 1 (SMN1) gene on chromosome 5, or a heterozygous deletion in combination with a (point) mutation in the second SMN1 allele. This results in degeneration of anterior horn cells, which leads to progressive muscle weakness. Children with SMA type II do not […]

Share

Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease

Abstract Background Amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND), is a fatal disease associated with rapidly progressive disability, for which no definitive treatment exists. Current treatment approaches largely focus on relieving symptoms to improve the quality of life of those affected. The therapeutic potential of cell‐based therapies in ALS/MND […]

Share

Drug treatment for spinal muscular atrophy type I

Abstract Background Spinal muscular atrophy (SMA) is caused by a homozygous deletion of the survival motor neuron 1 (SMN1) gene on chromosome 5, or a heterozygous deletion in combination with a point mutation in the second SMN1 allele. This results in degeneration of anterior horn cells, which leads to progressive muscle weakness. By definition, children with SMA […]

Share

Strength training and aerobic exercise training for muscle disease

Abstract Background Strength training or aerobic exercise programmes, or both, might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease. This is an update of a review first published in 2004 and last updated in 2013. We undertook an update to incorporate new evidence in this […]

Share

Assisted standing for Duchenne muscular dystrophy

Abstract Background Duchenne muscular dystrophy (DMD) is the most common X‐linked neuromuscular disorder. When boys with DMD reach the second decade of life, they lose their ability to walk and become wheelchair dependent. Standing devices and orthoses are considered to be an essential component in the therapy management of DMD. Clinical opinion and research from […]

Share
  • The review abstracts published on this site are the property of John Wiley & Sons, Ltd., and of the Cochrane Review Groups that have produced the reviews.
Share
Share