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.

Full Abstract

OBJECTIVE: To systematically review evidence bearing on the management of patients with amyotrophic lateral sclerosis (ALS). METHODS: The authors analyzed studies from 1998 to 2007 to update the 1999 practice parameter. Topics covered in this section include breaking the news, multidisciplinary clinics, symptom management, cognitive and behavioral impairment, communication, and palliative care for patients with ALS. RESULTS: The authors identified 2 Class I studies, 8 Class II studies, and 30 Class III studies in ALS, but many important areas have been little studied. More high-quality, controlled studies of symptomatic therapies and palliative care are needed to guide management and assess outcomes in patients with ALS. Recommendations: Multidisciplinary clinic referral should be considered for managing patients with ALS to optimize health care delivery and prolong survival (Level B) and may be considered to enhance quality of life (Level C). For the treatment of refractory sialorrhea, botulinum toxin B should be considered (Level B) and low-dose radiation therapy to the salivary glands may be considered (Level C). For treatment of pseudobulbar affect, dextromethorphan and quinidine should be considered if approved by the US Food and Drug Administration (Level B). For patients who develop fatigue while taking riluzole, withholding the drug may be considered (Level C). Because many patients with ALS demonstrate cognitive impairment, which in some cases meets criteria for dementia, screening for cognitive and behavioral impairment should be considered in patients with ALS (Level B). Other management strategies all lack strong evidence.

Author information

Author/s: Miller, R G (RG); Jackson, C E (CE); Kasarskis, E J (EJ); England, J D (JD); Forshew, D (D); Johnston, W (W); Kalra, S (S); Katz, J S (JS); Mitsumoto, H (H); Rosenfeld, J (J); Shoesmith, C (C); Strong, M J (MJ); Woolley, S C (SC); Quality Standards Subcommittee of the American Academy of Neurology;

Affiliation: Department of Neurology, California Pacific Medical Center, San Francisco, California, USA.

Grants: 1U01 NS049640 (Agency:NINDS NIH HHS) ; N01-AR-2250 (Agency:NIAMS NIH HHS) ; R01 NS 44887 (Agency:NINDS NIH HHS) ; R01-NS045087 (Agency:NINDS NIH HHS) ; R01NS045087-01A2 (Agency:NINDS NIH HHS) ; U01NS042685-0 (Agency:NINDS NIH HHS)

Journal and publication information

Publication Type: Journal Article; Practice Guideline; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review

Journal: Neurology (Neurology), published in United States. (Language: eng)

Reference: 2009-Oct; vol 73 (issue 15) : pp 1227-33

Dates: Created 2009/10/13; Completed 2009/11/04;

PMID: 19822873, status: MEDLINE (last retrieval date: 11/4/2009, IMS Date: )

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

External Links for this article
(including full text providers, if available):

Click Electronic Full-text Provider Links to see options for finding the electronic full text links to this article. Note there may be a subscription or fee required for access to the full text. See our FAQ for information on finding FREE full text articles.

This article may also be located in paper journal collections available in many libraries. Use the Journal and Publication Information above to find the full article.