Creatine supplementation in Huntington's disease: a placebo-controlled pilot trial.

OBJECTIVE: To evaluate the effect of creatine (Cr) supplementation (5 g/day) in Huntington's disease (HD). METHODS: A 1-year double-blind placebo-controlled study was performed in 41 patients with HD (stage I through III). At baseline and after 6 and 12 months, the functional, neuromuscular, and cognitive status of the patients was assessed by a test battery that consisted of 1) the Unified Huntington's Disease Rating Scale (UHDRS), 2) an exercise test on an isokinetic dynamometer to assess strength of the elbow flexor muscles, 3) a maximal exercise test on a bicycle ergometer to evaluate cardiorespiratory fitness, and 4) a test to assess bimanual coordination ability. Following the baseline measurements, the subjects were assigned to either a creatine (n = 26) or a placebo group (n = 15). RESULTS: Scores on the functional checklist of the UHDRS (p < 0.05), maximal static torque (p < 0.05), and peak oxygen uptake (p < 0.05) decreased from the start to the end of the study, independent of the treatment received. Cognitive functioning, bimanual coordination ability, and general motor function (total motor scale, UHDRS) did not change from baseline to 1 year in either group. CONCLUSION: One year of Cr intake, at a rate that can improve muscle functional capacity in healthy subjects and patients with neuromuscular disease (5 g/day), did not improve functional, neuromuscular, and cognitive status in patients with stage I to III HD.

Unimanual and bimanual voluntary movement in Huntington's disease.

Unimanual and bimanual cyclical forearm movements were studied in 15 Huntington's disease (HD) patients and 15 healthy, gender- and age-matched controls. Whereas the unimanual task was only performed at maximal speed, the bimanual movements were performed according to the in-phase and anti-phase mode at different cycling frequencies. The HD patients also performed the tasks after 12 months of follow-up. Findings revealed that maximal cycling frequency during unimanual movement was significantly lower in HD patients as compared with controls. In addition, measures of relative phasing established that bimanual cyclical movements were performed with lower accuracy and higher variability in HD patients. The differential variability between both groups was magnified by increasing the cycling frequency and coordinative complexity whereas only coordinative complexity differentially affected the accuracy of relative phasing. The obtained performance measures were found to be significantly correlated with disease duration (unimanual) and with the score on the total motor scale, the Mini-Mental State Examination and the Stroop Interference Test (uni- and bimanual). After 12 months, maximal cycling frequency of unimanual elbow flexion-extension was significantly decreased in HD patients whereas the quality of the in-phase and anti-phase movement patterns remained stable.

No effects of oral ribose supplementation on repeated maximal exercise and de novo ATP resynthesis.

A double-blind randomized study was performed to evaluate the effect of oral ribose supplementation on repeated maximal exercise and ATP recovery after intermittent maximal muscle contractions. Muscle power output was measured during dynamic knee extensions with the right leg on an isokinetic dynamometer before (pretest) and after (posttest) a 6-day training period in conjunction with ribose (R, 4 doses/day at 4 g/dose, n = 10) or placebo (P, n = 9) intake. The exercise protocol consisted of two bouts (A and B) of maximal contractions, separated by 15 s of rest. Bouts A and B consisted of 15 series of 12 contractions each, separated by a 60-min rest period. During the training period, the subjects performed the same exercise protocol twice per day, with 3-5 h of rest between exercise sessions. Blood samples were collected before and after bouts A and B and 24 h after bout B. Knee-extension power outputs were approximately 10% higher in the posttest than in the pretest but were similar between P and R for all contraction series. The exercise increased blood lactate and plasma ammonia concentrations (P < 0.05), with no significant differences between P and R at any time. After a 6-wk washout period, in a subgroup of subjects (n = 8), needle-biopsy samples were taken from the vastus lateralis before, immediately after, and 24 h after an exercise bout similar to the pretest. ATP and total adenine nucleotide content were decreased by approximately 25 and 20% immediately after and 24 h after exercise in P and R. Oral ribose supplementation with 4-g doses four times a day does not beneficially impact on postexercise muscle ATP recovery and maximal intermittent exercise performance.