Evidence-Based Neurology
CATEGORY: Neuromuscular Diseases

The incidence of statin induced myopathy is very low
Date appraised: May 2006
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Dichlorphenamide reduced the severity and frequency of attacks in the primary periodic paralyses
Date appraised: April 2006
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In patients with amyotrophic lateral sclerosis, age and forced vital capacity progression rate predicted survival
Date appraised: February 2005
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In the short-term, IVIg treatment increased muscle strength and resulted in the subjective improvement of patients with multifocal motor neuropathy
Date appraised: July 2004
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Weak evidence showed improved survival with the use of BiPAP in patients with respiratory insufficiency due to ALS.
Date appraised: January 2003
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IVIg improved disability scores at 6 weeks in patients with CIDP compared with placebo (NNT = 3), but with a slightly higher risk of side effects. IVIg appeared equally efficacious to prednisolone.
Date appraised: October 2002
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In patients with steroid-resistant polymyositis and dermatomyositis, weak evidence suggested that Methotrexate or Cyclosporine A, in addition to corticosteroids, may produce clinical and laboratory improvement.
Date appraised: July 2002
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There is no difference in the functional outcome of patients with a myasthenic exacerbation treated with either IVIg or plasma exhange
Date appraised: December 2001
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In patients with GBS, the following factors predicted poor outcome (inability to walk independently) at 8 weeks: age & 50 yo., preceding gastrointestinal illness, and MRC sum score <40.
Date appraised: October 2001
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For patients with Bell's palsy, weak evidence suggested benefit from steroids and/or acyclovir. There was no evidence that surgical nerve decompression is beneficial.
Date appraised: September 2001
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Prognostic factors in Duchenne Muscular Dystrophy include: location of mutation, the amount and evenness of distribution among muscle fibres of dystrophin, and possible compensatory mechanisms (eg. Utrophin). However, at present, the evidence for clinical prognostic features is lacking.
Date appraised: January 2000
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Daily prednisone at 0.75 mg/kg/d slowed progression of DMD (+36%muscle mass, +1 myometer score, +0.13 kg lifted) over 18 months but had significant side-effects.
Date appraised: November 1999
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Weak evidence suggested that surgical decompression was effective for all levels of severity of ulnar nerve compression at the elbow (77-89% chance of improvement).
Date appraised: October 1999
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With conservative management alone, 42% of mild cases of ulnar neuropathies and 20% of severe cases became symptom free;79% improved and 21% worsened.
Date appraised: October 1999
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Riluzole appears to minimally improve survival in ALS.
Date appraised: April 1999
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The NMJ "enhancers" physostigmine, neostigmine, and 3,4-DAP do not improve motor function in ALS
Date appraised: April 1999
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Intramuscular injections of gangliosides do not alter progressive deterioration of ALS.
Date appraised: April 1999
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Overall, immune suppressants and modulating agents do not alter disease progression in ALS.
Date appraised: April 1999
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Treatment of ALS with selegiline or L-Dopa does not alter the rate of clinical deterioration
Date appraised: April 1999
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Treatment of ALS with Modified Snake Venom did not alter clinical progression.
Date appraised: April 1999
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rhIGF-I may slow progression of functional impairment and the decline in health-related quality of life in ALS patients while rhCNTF and TRH do not alter clinical progression of ALS.
Date appraised: April 1999
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Extremely weak evidence indicates that patients with myasthenia gravis may improve with trans-sternal thymectomy in addition to medical management.
Date appraised: February 1999
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IVIg is at least as effective and perhaps superior to Plasma Exchange in the treatment of patients with acute non-bulbar Guillain-Barre Syndrome.
Date appraised: August 1998
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