Neuromuscular disorders

Treatment for familial amyotrophic lateral sclerosis/motor neuron disease

Abstract Background Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a rare neurodegenerative disease. Approximately 5% to 7% of ALS/MND patients report a family history of a similarly affected relative. Superoxide dismutase-1 gene mutations are the cause in about 20% of familial cases. In those with non-familial (sporadic) ALS/MND the cause […]

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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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Antioxidant treatment for amyotrophic lateral sclerosis or motor neuron disease

Abstract Background Free radical accumulation and oxidative stress have been proposed as contributing to the progression of amyotrophic lateral sclerosis (or motor neuron disease). A range of antioxidant medications have been studied. This is an updated review. Objectives To examine the effects of antioxidant medication in the treatment of people with amyotrophic lateral sclerosis. Search […]

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Interventions for increasing ankle range of motion in patients with neuromuscular disease

Abstract Background Reduced ankle dorsiflexion range of motion, or ankle equinus, is a common and disabling problem for patients with neuromuscular disease. Clinicians devote considerable time and resources implementing interventions to correct this problem although few of these interventions have been subject to rigorous empirical investigation. Objectives To assess the effect of interventions to reduce […]

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Scapular fixation in muscular dystrophy

Abstract Background Winging of the scapula is caused by weakness of the thoracoscapular muscles, which allows the scapula to lift off the chest wall during shoulder movements. In facioscapulohumeral muscular dystrophy (and occasionally in other muscular dystrophies) there is selective weakness of the thoracoscapular muscles which may spare other shoulder muscles such as the deltoid […]

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Exercise for people with peripheral neuropathy

Abstract Background Peripheral neuropathies are a wide range of diseases affecting the peripheral nerves. Demyelination or axonal degeneration gives rise to a variety of symptoms including reduced or altered sensation, pain, muscle weakness and fatigue. Secondary disability arises and this may result in adjustments to psychological and social function. Exercise therapy, with a view to […]

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Drug treatment for myotonia

Abstract Background Abnormal delayed relaxation of skeletal muscles, known as myotonia, can cause disability in myotonic disorders. Sodium channel blockers, tricyclic antidepressive drugs, benzodiazepines, calcium-antagonists, taurine and prednisone may be of use in reducing myotonia. Objectives To consider the evidence from randomised controlled trials on the efficacy and tolerability of drug treatment in myotonia . […]

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Treatment for Fisher syndrome, Bickerstaff’s brainstem encephalitis and related disorders

Abstract Background Fisher syndrome is one of the regional variants of Guillain-Barré syndrome, characterised by impairment of eye movements (ophthalmoplegia), incoordination (ataxia) and loss of tendon reflexes (areflexia). It can occur in more limited forms, and may overlap with Guillain-Barré syndrome. A further variant is associated with upper motor neuron signs and disturbance of consciousness […]

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Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults

Abstract Background Herpes zoster oticus (HZO) is a viral infection of the ear and when associated with acute facial paralysis is known as Ramsay Hunt syndrome. Antiviral agents are the standard first-line treatment for herpes zoster infections at other body sites and are thought to reduce or minimise nerve damage, thereby improving outcomes. It has […]

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Surgical versus non-surgical treatment for carpal tunnel syndrome

Abstract Background Carpal tunnel syndrome results from entrapment of the median nerve in the wrist. Common symptoms are tingling, numbness, and pain in the hand that may radiate to the forearm or shoulder. Most symptomatic cases are treated non-surgically. Objectives The objective is to compare the efficacy of surgical treatment of carpal tunnel syndrome with […]

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