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Practice Guideline
. 2020 Nov 16;192(46):E1453-E1468.
doi: 10.1503/cmaj.191721.

Canadian best practice recommendations for the management of amyotrophic lateral sclerosis

Affiliations
Practice Guideline

Canadian best practice recommendations for the management of amyotrophic lateral sclerosis

Christen Shoesmith et al. CMAJ. .
No abstract available

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Conflict of interest statement

Competing interests: Christen Shoesmith, Aaron Izenberg, Wendy Johnston, Colleen O’Connell, Kerri Schellenberg and Lorne Zinman were members of a scientific advisory committee for Radicava (edaravone; Mitsubishi Tanabe Pharma Canada). Christen Shoesmith reports being a site principal investigator for several multicentre amyotrophic lateral sclerosis (ALS) clinical trials. In the last 36 months, Dr. Shoesmith has participated in clinical trials sponsored by Biogen, Cytokinetics, ALS Pharma and Orphazyme. Marvin Chum reports receiving a grant from Bernice Ramsay ALS Clinical Research Fellowship, outside the submitted work. Aaron Izenberg reports receiving personal fees from Biogen, Roche, Alnylam, Genzyme, Takeda and Mitsubishi Tanabe Pharma, outside the submitted work. No other competing interests were declared.

Figures

Figure 1:
Figure 1:
Respiratory decision tree: Summary of recommendations for respiratory management in patients with amyotrophic lateral sclerosis (ALS), including ventilation (A) and airway clearance (B). Note: FVC = forced vital capacity, H2O = water, LVR = lung volume recruitment, MIE = mechanical insufflation–exsufflation, MIP = maximal inspiratory pressure, NIV = noninvasive ventilation, PCF = peak cough flow, pCO2 = partial pressure of carbon dioxide, SNIP = sniff nasal inspiratory pressure, SVC = slow vital capacity.
Figure 2:
Figure 2:
Nutrition decision tree: Summary of recommendations for nutritional management in patients with amyotrophic lateral sclerosis (ALS). Note: BMI = body mass index, FEES = fibre-optic endoscopic evaluation of swallowing, FVC = forced vital capacity, MBS = modified barium swallow, NG = nasogastric tube, PEG = percutaneous endoscopy gastrostomy, RIG = radiologically inserted gastrostomy, TDEE = total daily energy expenditure.

References

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    1. van Es MA, Hardiman O, Chio A, et al. Amyotrophic lateral sclerosis. Lancet 2017;390:2084–98. - PubMed
    1. Strong MJ, Abrahams S, Goldstein LH, et al. Amyotrophic lateral sclerosis — frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener 2017;18:153–74. - PMC - PubMed
    1. Shoesmith C. A recipe for ALS. Can J Neurol Sci 2008;35:125–126. - PubMed
    1. Health Canada approves new drug to treat patients with amyotrophic lateral sclerosis (ALS) [press release]. Ottawa: Health Canada; 2018. October 4.

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