title: 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.
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 slowing disease progression, nutrition, and respiratory management for patients with ALS.
  
  
  RESULTS
  The authors identified 8 Class I studies, 5 Class II studies, and 43 Class III studies in ALS. Important treatments are available for patients with ALS that are underutilized. Noninvasive ventilation (NIV), percutaneous endoscopic gastrostomy (PEG), and riluzole are particularly important and have the best evidence. More studies are needed to examine the best tests of respiratory function in ALS, as well as the optimal time for starting PEG, the impact of PEG on quality of life and survival, and the effect of vitamins and supplements on ALS.
  
  
  CONCLUSIONS
  Riluzole should be offered to slow disease progression (Level A). PEG should be considered to stabilize weight and to prolong survival in patients with ALS (Level B). NIV should be considered to treat respiratory insufficiency in order to lengthen survival (Level B) and to slow the decline of forced vital capacity (Level B). NIV may be considered to improve quality of life (Level C) [corrected].Early initiation of NIV may increase compliance (Level C), and insufflation/exsufflation may be considered to help clear secretions (Level C).
authors.affiliation: Department of Neurology, California Pacific Medical Center, San Francisco, California, USA.
authors.forename: R G
authors.initials: RG
authors.lastname: Miller
authors.forename: C E
authors.initials: CE
authors.lastname: Jackson
authors.forename: E J
authors.initials: EJ
authors.lastname: Kasarskis
authors.forename: J D
authors.initials: JD
authors.lastname: England
authors.forename: D
authors.initials: D
authors.lastname: Forshew
authors.forename: W
authors.initials: W
authors.lastname: Johnston
authors.forename: S
authors.initials: S
authors.lastname: Kalra
authors.forename: J S
authors.initials: JS
authors.lastname: Katz
authors.forename: H
authors.initials: H
authors.lastname: Mitsumoto
authors.forename: J
authors.initials: J
authors.lastname: Rosenfeld
authors.forename: C
authors.initials: C
authors.lastname: Shoesmith
authors.forename: M J
authors.initials: MJ
authors.lastname: Strong
authors.forename: S C
authors.initials: SC
authors.lastname: Woolley
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000178:diet therapy / Q000188:drug therapy / Q000628:therapy*; D004750:Enteral Nutrition / Q000379:methods; D019317:Evidence-Based Medicine; D006801:Humans; D016651:Lithium Carbonate / Q000627:therapeutic use; D011788:Quality of Life; D012138:Respiratory Therapy / Q000379:methods*; D019782:Riluzole / Q000627:therapeutic use
chemical_list: D016651:Lithium Carbonate; D019782:Riluzole