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Calcium-mediated excitotoxicity and defective energy metabolism have been implicated in the pathogenesis of Huntington disease (HD).1 In R6/2 HD transgenic mice, administration of 2% dietary creatine presymptomatically at 6 and 8 weeks improved survival, delayed motor progression, and reduced weight loss and brain atrophy.2,3 Creatine buffers intracellular energy reserves and has several neuroprotective effects demonstrated both in vivo and in vitro.4
A placebo-controlled pilot trial of 5 g/day of creatine for 1 year appeared ineffective in 26 patients with HD,5 although this dose was lower than the equivalent dose given to R6/2 HD mice. We previously reported clinical and MR spectroscopy (MRS) findings in a 1-year open-label pilot study of high-dose creatine (10 g/day) supplementation in 13 patients with HD.6 Serial 31P-MRS of muscle monitored changes in energy metabolism in vivo, and 1H-MRS was used to determine creatine levels in brain tissue. High-dose creatine was well tolerated, and total motor score (TMS), functional capacity, and neuropsychology testing remained stable at 12 months. We now report …
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