title: Behavior matters--cognitive predictors of survival in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  It is difficult to longitudinally characterize cognitive impairment in amyotrophic lateral sclerosis (ALS) due to motor deficits, and existing instruments aren't comparable with assessments in other dementias.
  
  
  METHODS
  The ALS Brief Cognitive Assessment (ALS-BCA) was validated in 70 subjects (37 with ALS) who also underwent detailed neuropsychological analysis. Cognitive predictors for poor survival were then analyzed in a longitudinal cohort of 171 ALS patients.
  
  
  RESULTS
  The ALS-BCA was highly sensitive (90%) and specific (85%) for ALS-dementia (ALS-D). ALS-D patients had shorter overall survival, primarily due to the poor survival among ALS-D patients with disinhibited or apathetic behaviors after adjusting for demographic variables, ALS site of onset, medications, and supportive measures. ALS-D without behavioral changes was not a predictor of poor survival.
  
  
  CONCLUSIONS
  ALS-D can present with or without prominent behavioral changes. Cognitive screening in ALS patients should focus on behavioral changes for prognosis, while non-behavioral cognitive impairments may impact quality of life without impacting survival.
authors: Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, United States of America. william.hu@emory.edu
authors: William T
authors: WT
authors: Hu
authors: Matthew
authors: M
authors: Shelnutt
authors: Ashley
authors: A
authors: Wilson
authors: Nicole
authors: N
authors: Yarab
authors: Crystal
authors: C
authors: Kelly
authors: Murray
authors: M
authors: Grossman
authors: David J
authors: DJ
authors: Libon
authors: Jaffar
authors: J
authors: Khan
authors: James J
authors: JJ
authors: Lah
authors: Allan I
authors: AI
authors: Levey
authors: Jonathan
authors: J
authors: Glass
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000401:mortality* / Q000503:physiopathology*; D001519:Behavior / Q000502:physiology*; D003071:Cognition / Q000502:physiology*; D003704:Dementia / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012372:ROC Curve; D015203:Reproducibility of Results