Efficacy, safety and tolerability of high lipid supplementation in amyotrophic lateral sclerosis

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Amyotrophic lateral sclerosis (ALS) is characterized by the simultaneous degeneration of lower (spinal and bulbar) and upper (corticospinal) motor neurons, leading to progressive muscle atrophy and paralysis. ALS usually leads to death within 2 to 3 years after diagnosis, mostly from respiratory failure. Weight loss is a common phenomenon and an independent prognostic factor in ALS. Several potential causal mechanisms including intrinsic hypermetabolism and deficient food intake have been discussed. Very few clinical efforts focused on nutrition. It was shown in a retrospective data analysis that high serum levels of both fasting cholesterol and triglycerides had a significant positive effect on survival. Life expectancy of patients with high triglyceride levels was prolonged by 14 months. Therefore we will investigate in a multicentre, 1:1-randomised, stratified (onset of disease: spinal and bulbar form; BMI status: low and high BMI), double-blind, placebo-controlled study the effect of a high caloric fatty diet (39.3% lipids) for drinking additionally to daily food intake on survival of ALS patients. 324 patients will be recruited by 12 centres (neurological clinics with specialised motoneuron outpatient units) in Germany. The results of this study will provide an evidence-based evaluation of a nutritional impact, e.g. lipids, on survival of ALS patients. Furthermore the results could improve patient care.