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In this study, we examined the effects of a dietary supplement containing carnosine and an arginine/lysine/carnosine mixture (ALC) on endurance exercise performance and muscle damage in patients with DM1. ALC has three primary components, arginine, L-carnosine and H-carnosine, which are metabolised in the body to enhance wakefulness and promote muscular recovery and repair. Arginine and carnosine have been shown to increase ATP generation, and carnosine has been found to increase myocardial contractility [247, 248]. Carnosine may also reduce muscular fatigue, muscle stiffness, pain due to inflammation and the production of free radicals [249]. Given these benefits, ALC could be useful for DM1 patients where exercise can be difficult due to fatigue. This is the first study of ALC supplementation in patients with DM1. Given previous research in NDM [3, 275], this is the only study to date that has examined the acute effects of carnosine supplementation on neuromuscular performance. This study showed no difference in maximal exercise testing capacity.
The most common drug-based neuromuscular treatment for DM1 is baclofen, which is used to reduce muscle spasticity. A combination of baclofen and botulinum toxin has been reported as therapeutic in some severe cases of myotonia [250]. An important negative effect of baclofen is the drug-induced muscle weakness and the related difficulty in getting out of chairs. Baclofen has been reported to cause severe weakness, making wheelchair use impossible and increasing the risk of falls [251]. Muscle weakness due to baclofen has been reported to begin at 5 mg and extend to 10 mg, and cannot be reversed by cessation of baclofen [252]. This study has shown no difference in acute muscle performance after baclofen washout. The reasons for this could be the use of a balanced amino acidic solution for the washout, or the washout period may not have been long enough. The study also showed no difference in performance with botulinum toxin, which is used to relax the muscle.] d2c66b5586
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