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October 13, 2009; 73 (15) Special Article

Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review)

Report of the Quality Standards Subcommittee of the American Academy of Neurology

R. G. Miller, C. E. Jackson, E. J. Kasarskis, J. D. England, D. Forshew, W. Johnston, S. Kalra, J. S. Katz, H. Mitsumoto, J. Rosenfeld, C. Shoesmith, M. J. Strong, S. C. Woolley
First published October 12, 2009, DOI: https://doi.org/10.1212/WNL.0b013e3181bc0141
R. G. Miller
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C. E. Jackson
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E. J. Kasarskis
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J. D. England
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D. Forshew
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S. Kalra
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J. S. Katz
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S. C. Woolley
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Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review)
Report of the Quality Standards Subcommittee of the American Academy of Neurology
R. G. Miller, C. E. Jackson, E. J. Kasarskis, J. D. England, D. Forshew, W. Johnston, S. Kalra, J. S. Katz, H. Mitsumoto, J. Rosenfeld, C. Shoesmith, M. J. Strong, S. C. Woolley
Neurology Oct 2009, 73 (15) 1218-1226; DOI: 10.1212/WNL.0b013e3181bc0141

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This article has a correction. Please see:

  • Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology - December 15, 2009
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    Figure 1 Nutrition management algorithm

    *e.g., Bulbar questions in the Amyotrophic Lateral Sclerosis Functional Rating Scale, or other instrument. †Prolonged meal time; ending meal prematurely because of fatigue; accelerated weight loss due to poor caloric intake; family concern about feeding difficulties. ‡Percutaneous endoscopic gastrostomy: rule out contraindications.

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    Figure 2 Respiratory management algorithm

    PFT = pulmonary function tests; PCEF = peak cough expiratory flow; NIV = noninvasive ventilation; SNP = sniff nasal pressure; MIP = maximal inspiratory pressure; FVC = forced vital capacity (supine or erect); Abnl.nocturnal oximetry = pO2 <4% from baseline. *Symptoms suggestive of nocturnal hypoventilation: frequent arousals, morning headaches, excessive daytime sleepiness, vivid dreams. †If NIV is not tolerated or accepted in the setting of advancing respiratory compromise, consider invasive ventilation or referral to hospice.

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