Department of Clinical Neurosciences, and Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada.
Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada.
Can J Neurol Sci. 2018 Nov;45(6):652-659. doi: 10.1017/cjn.2018.311.
Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease resulting in muscle weakness, dysarthria and dysphagia, and ultimately respiratory failure leading to death. Half of the ALS patients survive less than 3 years, and 80% of the patients survive less than 5 years. Riluzole is the only approved medication in Canada with randomized controlled clinical trial evidence to slow the progression of ALS, albeit only to a modest degree. The Canadian Neuromuscular Disease Registry (CNDR) collects data on over 140 different neuromuscular diseases including ALS across ten academic institutions and 28 clinics including ten multidisciplinary ALS clinics.
In this study, CNDR registry data were analyzed to examine potential differences in ALS care among provinces in time to diagnosis, riluzole and feeding tube use.
Significant differences were found among provinces, in time to diagnosis from symptom onset, in the use of riluzole and in feeding tube use.
Future investigations should be undertaken to identify factors contributing to such differences, and to propose potential interventions to address the provincial differences reported.
肌萎缩侧索硬化症（ALS）是一种进行性运动神经元疾病，导致肌肉无力、构音障碍和吞咽困难，最终导致呼吸衰竭而死亡。一半的 ALS 患者存活时间不到 3 年，80%的患者存活时间不到 5 年。利鲁唑是加拿大唯一一种经随机对照临床试验证实可减缓 ALS 进展的药物，尽管只是适度减缓。加拿大神经肌肉疾病登记处（CNDR）收集了来自十个学术机构和 28 个诊所的超过 140 种不同神经肌肉疾病的数据，包括十个多学科 ALS 诊所。
本研究分析了 CNDR 登记处的数据，以检查在诊断时间、利鲁唑和饲管使用方面，各省 ALS 治疗的潜在差异。
在从症状出现到诊断的时间、利鲁唑的使用和饲管使用方面，各省之间存在显著差异。
应进行进一步研究，以确定造成这种差异的因素，并提出解决报告的省级差异的潜在干预措施。
