Abstract

Introduction

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.

中文翻译：

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利鲁唑对肌萎缩侧索硬化短期和长期幸存者体重的影响

摘要

介绍

利鲁唑是美国食品和药物管理局于 1995 年批准的第一种治疗肌萎缩侧索硬化症 (ALS) 的疾病改善疗法，现在可在全球范围内使用。它延迟了气管切开术或死亡的时间并延长了生存期。生存延长效应的确切机制尚不清楚。营养不良和随之而来的体重减轻与 ALS 的生存期缩短有关。鉴于利鲁唑和营养维持的延长寿命作用，我们检查了利鲁唑与 ALS 患者体重之间的关系。材料和方法：使用来自佛蒙特大学医学中心国家 ALS 卓越中心诊所数据库的数据，我们根据利鲁唑的使用情况和从基线访问到短期（≤3 年）和长期的生存持续时间，将 244 名患者分层为队列长期（> 3 年）幸存者。我们检查了基线访视后第一年和死亡前最后一年患者的平均每月体重变化。结果和讨论：在服用利鲁唑的 156 名短期幸存者中，与未服用利鲁唑的患者相比，基线后第一年体重减轻了 37%，死亡前最后一年体重减轻了 46%。74 名长期服用利鲁唑的幸存者在基线访视后的第一年体重下降减少。我们推测利鲁唑可能影响生存的一种机制是减轻体重减轻并可能维持营养状况和身体成分，尽管这值得前瞻性研究。