Behavior Matters-Cognitive Predictors of Survival in Amyotrophic Lateral Sclerosis

William T. Hu, Matthew Shelnutt, Ashley Wilson, Nicole Yarab, Crystal Kelly, Murray Grossman, David J. Libon, Jaffar Khan, James J. Lah, Allan I. Levey, Jonathan Glass

Research output: Contribution to journalArticlepeer-review

52 Scopus citations

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. Conclusion: 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.

Original languageEnglish (US)
Article numbere57584
JournalPloS one
Volume8
Issue number2
DOIs
StatePublished - Feb 27 2013
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

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