Riluzole is the first disease-modifying therapy for amyotrophic lateral sclerosis (ALS) approved in 1995 by the Food and Drug Administration in the USA, and is now available worldwide. It delays time to tracheostomy or death and prolongs survival. The precise mechanism of the survival prolonging effect is unknown. Malnutrition and ensuing weight loss are associated with shorter survival in ALS. Given the life-prolonging effects of riluzole and nutritional maintenance, we examined the relationship between riluzole and weight in ALS patients. Materials and Methods: Using data from the National ALS Center of Excellence clinic database at the University of Vermont Medical Center, we stratified 244 patients into cohorts based on riluzole use, and duration of survival from the baseline visit into short-term (≤3 years) and long-term (>3 years) survivors. We examined average monthly weight change in patients during the first year after the baseline visit, and the last year before death. Results and Discussion: In 156 short-term survivors taking riluzole compared to those not taking riluzole, there was a 37% attenuation of weight loss in the first year after baseline, and 46% attenuation of weight loss in the last year before death. Seventy-four n long-term survivors on riluzole showed reduced weight decline in the first year after the baseline visit. We speculate that one mechanism by which riluzole may affect survival is by attenuating weight loss and possibly maintaining nutritional status and body composition, although this warrants prospective study.
Keywords: Survival; nutrition; therapy.