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Provincial Differences in the Diagnosis and Care of Amyotrophic Lateral Sclerosis

  • Victoria L. Hodgkinson (a1), Josh Lounsberry (a1), Ario Mirian (a1), Angela Genge (a2), Timothy Benstead (a3), Hannah Briemberg (a4), Ian Grant (a3), Walter Hader (a5), Wendy S. Johnston (a6), Sanjay Kalra (a6), Gary Linassi (a5), Rami Massie (a2), Michel Melanson (a7), Colleen O’Connell (a8) (a9), Kerri Schellenberg (a10), Christen Shoesmith (a11), Sean Taylor (a7), Scott Worley (a8) (a9), Lorne Zinman (a12) and Lawrence Korngut (a1)...
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.

Différences entres les provinces canadiennes en ce qui concerne le diagnostic de la sclérose latérale amyotrophique et les soins destinés aux patients.Contexte: La sclérose latérale amyotrophique (SLA) est une pathologie progressive des neurones moteurs qui entraîne l’affaiblissement des muscles, des manifestations de dysarthrie et de dysphagie et à terme une insuffisance respiratoire causant la mort. La moitié des patients atteints de SLA survive moins de 3 ans; 80 % d’entre eux, moins de 5 ans. Au Canada, le riluzole demeure le seul médicament autorisé permettant, bien que seulement à un faible degré, de ralentir la progression de la SLA. À noter que l’autorisation donnée à ce médicament repose sur des essais cliniques randomisés et contrôlés par placebo. En collaboration avec 10 établissements d’enseignement et 28 cliniques, dont 10 sont des cliniques multidisciplinaires spécialisées dans la SLA, le Registre canadien des maladies neuromusculaires (RCMN) recueille des données sur plus de 140 maladies neuromusculaires, ce qui inclut la SLA. Méthodes: Dans cette étude, le registre de données du RCMN a été analysé afin de détecter, parmi les provinces canadiennes, d’éventuelles différences en matière de soins pour la SLA, par exemple le moment où un diagnostic a été établi ou encore l’utilisation de riluzole et d’une sonde d’alimentation. Résultats: Des différences notables ont été observées entre les provinces, et ce, qu’il s’agisse du moment, à partir de l’apparition des premiers symptômes, où un diagnostic a été établi ou de l’utilisation de riluzole et d’une sonde alimentaire. Conclusions: Des travaux de recherche devraient être menés à l’avenir afin que l’on puisse identifier les facteurs expliquant ces différences et recommander de possibles interventions visant à y remédier.

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This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution, and reproduction in any medium, provided the original work is properly cited.
Corresponding author
Correspondence to: Lawrence Korngut, Associate Professor (Neurology), Director, Calgary ALS and Motor Neuron Disease Clinic, 480060, 4th Floor Administration, Clinical Neurosciences, South Health Campus, 4448 Front Street SE, Calgary, AB, Canada T3M 1M4. Email: lwkorngu@ucalgary.ca
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