title: Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology.
abstract: BACKGROUND
  The American Academy of Neurology (AAN) ALS Practice Parameter was published in April 1999. The ALS CARE Database has been collecting data on the management of patients with ALS in North America since 1996.
  
  
  OBJECTIVE
  To compare the management of patients with ALS in North America as recorded in the ALS CARE Database with the recommendations of the AAN ALS Practice Parameter.
  
  
  METHODS
  Data were analyzed from 2018 patients at enrollment and from 373 of these patients who died between enrollment and May 1999.
  
  
  RESULTS
  Eighty-two percent of the enrolled patients reported that they had been given enough information about ALS. Only 54% of patients with drooling were receiving medication for this problem. Only 41% of those who reported being depressed most of the time were receiving antidepressant medications. Only 28% of those with dyspnea and only 9.2% of those with a forced vital capacity <40% predicted were receiving noninvasive positive pressure ventilator support. Only 30% of those with moderate to severe dysphagia had a gastrostomy tube. Half of the patients who died did so at home, but only 47% of them received residential hospice services. Although 89% of patients who died were recorded as having done so peacefully, 17% were reported to have had breathing difficulties (i.e., respiratory distress), 8% anxiety, 3.3% pain, and 2.5% choking. Advance directives were in place for 90% of the patients who died, and in 97% of cases these directives were followed.
  
  
  CONCLUSIONS
  These findings indicate that in the 3-year period prior to the publication of the AAN Practice Parameter, many but not all patients received the care that is recommended in that parameter; there were deficiencies, particularly in the key areas of gastrostomy and noninvasive positive pressure ventilation.
authors.affiliation: Department of Neurology, University of Miami School of Medicine, FL 33101, USA. wbradley@med.miami.edu
authors.forename: W G
authors.initials: WG
authors.lastname: Bradley
authors.forename: F
authors.initials: F
authors.lastname: Anderson
authors.forename: M
authors.initials: M
authors.lastname: Bromberg
authors.forename: L
authors.initials: L
authors.lastname: Gutmann
authors.forename: Y
authors.initials: Y
authors.lastname: Harati
authors.forename: M
authors.initials: M
authors.lastname: Ross
authors.forename: R G
authors.initials: RG
authors.lastname: Miller
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000628:therapy*; D016208:Databases, Factual; D006801:Humans; D005791:Patient Care; D010818:Practice Patterns, Physicians'; D014481:United States