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

doi:10.1212/WNL.0b013e3181bc0141

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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, MD, FAAN, C. E. Jackson, MD, FAAN, E. J. Kasarskis, MD, PhD, FAAN, J. D. England, MD, FAAN, D. Forshew, RN, W. Johnston, MD, S. Kalra, MD, J. S. Katz, MD, H. Mitsumoto, MD, FAAN, J. Rosenfeld, MD, PhD, FAAN, C. Shoesmith, MD, BSc, M. J. Strong, MD and S. C. Woolley, PhD

From the Department of Neurology (R.G.M., D.F., J.S.K., S.C.W.), California Pacific Medical Center, San Francisco; University of Texas Health Science Center of San Antonio (C.E.J.); University of Kentucky (E.J.K.), Lexington; Louisiana State University Health Sciences Center (J.D.E.), New Orleans; Department of Neurology (W.J., S.K.), University of Alberta, Canada; Neurological Institute (NI-9) (H.M.), New York, NY; Division of Neurology (J.R.), UCSF, Fresno, CA; and London Health Sciences Center (C.S., M.J.S.), London, Canada.

Address correspondence and reprint requests to the American Academy of Neurology, 1080 Montreal Avenue, St. Paul, MN 55116 guidelines{at}aan.com

Objective: To systematically review evidence bearing on themanagement of patients with amyotrophic lateral sclerosis (ALS).

Methods: The authors analyzed studies from 1998 to 2007 to updatethe 1999 practice parameter. Topics covered in this sectioninclude slowing disease progression, nutrition, and respiratorymanagement for patients with ALS.

Results: The authors identified 8 Class I studies, 5 Class IIstudies, and 43 Class III studies in ALS. Important treatmentsare available for patients with ALS that are underutilized.Noninvasive ventilation (NIV), percutaneous endoscopic gastrostomy(PEG), and riluzole are particularly important and have thebest evidence. More studies are needed to examine the best testsof respiratory function in ALS, as well as the optimal timefor starting PEG, the impact of PEG on quality of life and survival,and the effect of vitamins and supplements on ALS.

Recommendations: Riluzole should be offered to slow diseaseprogression (Level A). PEG should be considered to stabilizeweight and to prolong survival in patients with ALS (Level B).NIV should be considered to treat respiratory insufficiencyin order to lengthen survival (Level B), and may be consideredto slow the decline of forced vital capacity (Level C) and improvequality of life (Level C). Early initiation of NIV may increasecompliance (Level C), and insufflation/exsufflation may be consideredto help clear secretions (Level C).

Abbreviations: AAN = American Academy of Neurology; ALS = amyotrophic lateral sclerosis; FVC = forced vital capacity; HFCWO = high frequency chest wall oscillation; MIE = mechanical insufflation/exsufflation; MIP = maximal inspiratory pressure; NIV = noninvasive ventilation; PCEF = peak cough expiratory flow; Pdi = transdiaphragmatic pressure; PEG = percutaneous endoscopic gastrostomy; QOL = quality of life; RIG = radiologically inserted device; SNP = sniff nasal pressure; TIV = tracheostomy invasive ventilation.