It was also demonstrated that motor unit number estimation was performed with acceptable reproducibility and tolerability, and may be a useful outcome measure in future clinical trials.Ĭonclusion: Any beneficial effect of creatine at 5 g per day in ALS must be small. CI analysis showed that the study, although powered to detect a 50% or greater change in rate of decline of muscle strength, actually made an effect size of greater than 23% unlikely. Results: Creatine was tolerated well, but no benefit of creatine could be demonstrated in any outcome measure. Patients were treated for 6 months, and evaluated monthly. The primary outcome measure was maximum voluntary isometric contraction of eight upper extremity muscles, with secondary outcomes including grip strength, ALS Functional Rating Scale–Revised, and motor unit number estimates. Methods: The authors conducted a randomized double-blind, placebo controlled trial on 104 patients with ALS from 14 sites to evaluate the efficacy of creatine supplementation in ALS. In a transgenic mouse model of ALS, administration of creatine prolongs survival and preserves motor function and motor neurons. Creatine stabilizes the mitochondrial transition pore, and is important in mitochondrial ATP production. Background: Mitochondrial dysfunction occurs early in the course of ALS, and the mitochondria may be an important site for therapeutic intervention.
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