Provincial Differences in the Diagnosis and Care of Amyotrophic Lateral Sclerosis

Abstract

Background: 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. Methods: 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. Results: Significant differences were found among provinces, in time to diagnosis from symptom onset, in the use of riluzole and in feeding tube use. Conclusions: Future investigations should be undertaken to identify factors contributing to such differences, and to propose potential interventions to address the provincial differences reported.

Rural Residence and Diagnostic Delay for Amyotrophic Lateral Sclerosis in Saskatchewan

“…This may also partly explain why geospatial factors in Saskatchewan were inconsequential to patient life span after first symptom onseta finding consistent with the 2016 study in Ireland that found that distance from dwelling to the ALS multidisciplinary clinic had no bearing on survival. 9 Lastly, while a recent study on provincial differences in ALS care in Canada found Saskatchewan to have the longest diagnostic delay at 27 months, 6 our study shows Saskatchewan's diagnostic delay to be an average of 16.5 monthsa timeline more consistent with the previously reported provincial averages (mean time to diagnosis 21 months; range 15.1-27.0 months). It has been previously noted that slower progressors may be more likely to be referred to multidisciplinary clinics, 26,27 and slower progression is also associated with diagnostic delay.…”

“…1,5 In a recently published Canadian study, mean time of diagnostic delay was significantly different between provinces. 6 Saskatchewan was found to have the longest delay at 27 months, almost double that of some other provinces, but the reasons for this delay were not elucidated. As Saskatchewan has a very large rural and remote population, we sought to explore the possibility of a link between diagnostic delay and distance from tertiary health centers.…”

Epidemiology of amyotrophic lateral sclerosis: an update of recent literature

“…Recent studies have shown that the mean or median age of ALS onset is between 51 and 66 years [1 ▪ ,3,4 ▪ ,5,6,7 ▪▪ ,18 ▪ ,19,20,23,26,28–31,32 ▪▪ ]. Patients in Europe have usually a later age at ALS onset compared with patients from China, Cuba, and Uruguay.…”

“…The mean or median age at ALS diagnosis was reported as between 54 and 69 years in recent studies [1 ▪ ,2,3,4 ▪ ,5,6,7 ▪▪ ,16,19,20,26,30,32 ▪▪ ]. Predictors of higher age at diagnosis are cognitive impairment and sex.…”

Antioxidant Alternatives in the Treatment of Amyotrophic Lateral Sclerosis: A Comprehensive Review

“…There are currently no effective treatments for ALS and therapy is limited to symptomatic and palliative treatment (Bhattacharya et al, 2019). Riluzole and edaravone (Rothstein, 2017) are the only approved pharmacological therapies that increase the survival rate by 2-3 months (Dorst et al, 2017;Hodgkinson et al, 2018), but edaravone is only beneficial to a subset of people with early stage ALS (Abe et al, 2017). Other therapeutic routes indicate multidisciplinary treatment involving: nurses, dieticians, nutritionists, occupational therapists, physical therapists, psychologists, social workers and speech therapists (Gordon, 2013).…”