Document Overview

Document Title

The Care of the Patient with Amyotrophic Lateral Sclerosis: Drug, Nutritional, And Respiratory Therapies

Authoring Society

American Academy of Neurology

Document Publication Date

Oct 1, 2009

Page Last Reviewed/Updated

May 6, 2026

Document Type

Guideline

Country of Publication

United States

Full Text Freely Available

Yes

Full Text Guideline

n.neurology.org/content/73/15/1218.full

Source Citation

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

Supplemental Implementation Resources

• Quick-Reference Guide
• Quick-Reference Guide
• Slide Set
• Patient Information

Document Scope, Criteria, and Use Cases

Document Objectives

This guideline was retired on February 21, 2026, by the Guidelines Subcommittee.

To systematically review evidence bearing on the management of patients with amyotrophic lateral sclerosis (ALS).

Scope

Assessment and Screening, Management, Treatment

Diseases/Conditions (MeSH)

D000690 - Amyotrophic Lateral Sclerosis

D019636 - Neurodegenerative Diseases

D000690 - Amyotrophic Lateral Sclerosis

D019636 - Neurodegenerative Diseases

Keywords

amyotrophic lateral sclerosis, neurodegenerative disease

Target Patient Population

patients with amyotrophic lateral sclerosis

Inclusion Criteria

Male, Female, Adult

Health Care Settings

Ambulatory, Hospital, Outpatient

Intended Users

Dietician/Nutritionist, Nurse, Nurse Practitioner, Occupational Therapist, Physician, Physician Assistant, Respiratory Therapist

Recommendation Development Processes & Methodology

PICO Questions

1. Does lithium carbonate prolong survival or slow disease progression in amyotrophic lateral sclerosis?
2. What is the effect of riluzole on slowing the disease process or prolonging survival in amyotrophic lateral sclerosis?
3. What is the effect of enteral nutrition administered via Percutaneous Endoscopic Gastrostomy on weight stability?
4. When is Percutaneous Endoscopic Gastrostomy indicated in amyotrophic lateral sclerosis?
5. What is the efficacy of nutritional support via Percutaneous Endoscopic Gastrostomy in prolonging survival?
6. What is the effect of enteral nutrition delivered via Percutaneous Endoscopic Gastrostomy on quality of life?
7. What is the efficacy of vitamin and nutritional supplements on prolonging survival or quality of life?
8. What are the optimal pulmonary tests to detect respiratory insufficiency?
9. Does Non-invasive ventilation improve respiratory function or increase survival?
10. How do invasive and noninvasive ventilation affect quality of life?
11. What factors influence acceptance of invasive and noninvasive ventilation?
12. What is the efficacy of targeted respiratory interventions for clearing secretions?

Number of Source Documents

40

Literature Search End Date

Saturday, September 1, 2007

Includes peer/external review process?

Yes

Includes public comment process?

No

Methodologist involvement?

Yes

Patient involvement?

No

Includes multi-disciplinary group?

Yes

Includes systematic review?

Yes

Grades quality of strength of evidence?

Yes

Grades quality of strength of recommendation?

Yes

Discloses funding source?

Yes

Discloses conflicts of interest?

Yes

Includes benefits/harms analysis with recommendations?

No