Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology

doi:10.1212/WNL.0b013e3181bc01a4

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Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (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, Ontario, Canada.

Address correspondence and reprint requests to 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 breaking the news, multidisciplinary clinics, symptommanagement, cognitive and behavioral impairment, communication,and palliative care for patients with ALS.

Results: The authors identified 2 Class I studies, 8 Class IIstudies, and 30 Class III studies in ALS, but many importantareas have been little studied. More high-quality, controlledstudies of symptomatic therapies and palliative care are neededto guide management and assess outcomes in patients with ALS.

Recommendations: Multidisciplinary clinic referral should beconsidered for managing patients with ALS to optimize healthcare delivery and prolong survival (Level B) and may be consideredto enhance quality of life (Level C). For the treatment of refractorysialorrhea, botulinum toxin B should be considered (Level B)and low-dose radiation therapy to the salivary glands may beconsidered (Level C). For treatment of pseudobulbar affect,dextromethorphan and quinidine should be considered if approvedby the US Food and Drug Administration (Level B). For patientswho develop fatigue while taking riluzole, withholding the drugmay be considered (Level C). Because many patients with ALSdemonstrate cognitive impairment, which in some cases meetscriteria for dementia, screening for cognitive and behavioralimpairment should be considered in patients with ALS (LevelB). Other management strategies all lack strong evidence.

Abbreviations: ALS = amyotrophic lateral sclerosis; ALS-FTD = amyotrophic lateral sclerosis with a dementia meeting the Neary criteria for frontotemporal dementia; ALSbi = amyotrophic lateral sclerosis with behavioral impairment; ALSci = amyotrophic lateral sclerosis with cognitive impairment; BTxA = botulinum toxin type A; BTxB = botulinum toxin type B; DM = dextromethorphan; FDA = Food and Drug Administration; FTD = frontotemporal dementia; NIV = noninvasive ventilation; PEG = percutaneous endoscopic gastrostomy; Q = quinidine.