Canadian best practice recommendations for the management of amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a debilitating, progressive disease with degeneration of motor neurons in the brain and spinal cord causing weakness, muscle atrophy, fasciculations and spasticity. Onset in the limbs, with extremity weakness and impairment in mobility, is the most common prese...

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Published in:Canadian Medical Association journal (CMAJ) 2020-11, Vol.192 (46), p.E1453-E1468
Main Authors: Shoesmith, Christen, Abrahao, Agessandro, Benstead, Tim, Chum, Marvin, Dupre, Nicolas, Izenberg, Aaron, Johnston, Wendy, Kalra, Sanjay, Leddin, Desmond, O'Connell, Colleen, Schellenberg, Kerri, Tandon, Anu, Zinman, Lorne
Format: Article
Language:eng
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Summary:Amyotrophic lateral sclerosis (ALS) is a debilitating, progressive disease with degeneration of motor neurons in the brain and spinal cord causing weakness, muscle atrophy, fasciculations and spasticity. Onset in the limbs, with extremity weakness and impairment in mobility, is the most common presentation, occurring in about 70% of patients. Bulbar onset with oropharyngeal muscle involvement affecting swallowing and speech occurs in about 25% of cases. In addition to motor impairment, degeneration in the frontal and temporal lobes, resulting in cognitive or behavioral impairments, occurs in up to 50% of patients. Here, Shoesmith et al discuss the Canadian best practice recommendations for the management of ALS.
ISSN:0820-3946
1488-2329