Neuromuscular junction diseases

Invasive versus non-invasive ventilation for acute respiratory failure in neuromuscular disease and chest wall disorders

Abstract Background Acute respiratory failure is a common life-threatening complication of acute onset neuromuscular diseases, and may exacerbate chronic hypoventilation in patients with neuromuscular disease or chest wall disorders. Standard management includes oxygen supplementation, physiotherapy, cough assistance, and, whenever needed, antibiotics and intermittent positive pressure ventilation. Non-invasive mechanical ventilation (NIV) via nasal, buccal or full-face […]

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Acupuncture for neuropathic pain in adults

Abstract Background Neuropathic pain may be caused by nerve damage, and is often followed by changes to the central nervous system. Uncertainty remains regarding the effectiveness and safety of acupuncture treatments for neuropathic pain, despite a number of clinical trials being undertaken. Objectives To assess the analgesic efficacy and adverse events of acupuncture treatments for […]

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Acetylcholinesterase inhibitor treatment for myasthenia gravis

Abstract Background In myasthenia gravis, antibody-mediated blockade of acetylcholine receptors at the neuromuscular junction abolishes the naturally occurring ‘safety factor’ of synaptic transmission. Acetylcholinesterase inhibitors provide temporary symptomatic treatment of muscle weakness but there is controversy about their long-term efficacy, dosage and side effects. This is the second update of a review published in The […]

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Medical treatment for botulism

Abstract Background Botulism is an acute paralytic illness caused by a neurotoxin produced by Clostridium botulinum. Supportive care, including intensive care, is key but the role of other medical treatments is unclear. This is an update of a review first published in 2011. Objectives To assess the effects of medical treatments on mortality, duration of […]

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Thymectomy for non-thymomatous myasthenia gravis

Abstract Background Treatments currently used for patients with myasthenia gravis (MG) include steroids, non-steroid immune suppressive agents, plasma exchange, intravenous immunoglobulin and thymectomy. Data from randomized controlled trials (RCTs) support the use of some of these therapeutic modalities and the evidence for non-surgical therapies are the subject of other Cochrane reviews. Significant uncertainty and variation […]

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Intravenous immunoglobulin for myasthenia gravis

Abstract Background Myasthenia gravis is an autoimmune disease in which autoantibodies interfere with neuromuscular transmission. As with other autoimmune diseases, people with myasthenia gravis would be expected to benefit from intravenous immunoglobulin (IVIg). This is an update of a review first published in 2003 and last updated in 2007. Objectives To examine the efficacy of […]

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Medical and surgical treatment for ocular myasthenia

Abstract Background Approximately 50% of people with myasthenia gravis present with purely ocular symptoms, so called ocular myasthenia. Of these between 50% to 60% develop generalized disease, most within two years. Their management is controversial. This is an update of a review first published in 2006 and previously updated in 2008 and 2010. Objectives To […]

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Drug treatment for spinal muscular atrophy types II and III

Abstract Background Spinal muscular atrophy (SMA) is caused by degeneration of anterior horn cells, which leads to progressive muscle weakness. Children with SMA type II do not develop the ability to walk without support and have a shortened life expectancy, whereas children with SMA type III develop the ability to walk and have a normal […]

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Plasma exchange for generalised myasthenia gravis

Abstract Background Myasthenia gravis is an autoimmune disease mediated by auto-antibodies most often directed against the nicotinic acetylcholine receptor. Less than five per cent of patients have auto-antibodies to a muscle tyrosine kinase. Patients would be expected to benefit from plasma exchange. Objectives To examine the efficacy of plasma exchange in the short- and long-term treatment […]

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Corticosteroids for myasthenia gravis

Abstract Background Although widely accepted as an appropriate immunosuppressive therapy, the efficacy of glucocorticosteroid treatment has only rarely been tested in controlled studies. This is an update of a Cochrane review first published in 2005 and previously updated in 2006 and 2007. Objectives To assess the efficacy of glucocorticosteroids or adrenocorticotrophic hormone (ACTH) medication in […]

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