A Cochrane review [Abstract] 1 included 4 trials with 1477 patients with motoneuron disease. The trials examined tracheostomy-free survival. One trial (n=169) included older patients in more advanced stages of ALS. Riluzole 100 mg daily provided a benefit for the homogeneous group of patients in the first two trials (HR 0.80, 95% CI 0.64 to 0.99; 2 trials, n=323). When the third trial (including older and more seriously affected patients) was added, the overall treatment effect was reduced but still significant (HR 0.84, 95% CI 0.698 to 0.997; 3 trials, n=1282). This represented a 9% gain in the probability of surviving one year (49% in the placebo vs. 58% in the riluzole group), and increased median survival from 11.8 to 14.8 months. There was a small beneficial effect on both bulbar and limb function, but not on muscle strength. A three-fold increase in serum alanine transferase was more frequent in riluzole-treated patients than controls in the combined data (MD 2.62, 95% CI 1.59 to 4.31).
Comment: The quality of evidence is downgraded by inconsistency (heterogeneity in patients and results).
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