Behavior Matters—Cognitive Predictors of Survival in Amyotrophic Lateral Sclerosis

• Type of Material: Article
• Authors: William Hu, Emory University; Matthew Shelnutt, Emory University; Ashley Wilson, Emory University; Nicole Yarab, Emory University; Crystal Kelly, Emory University; Murray Grossman, University of Pennsylvania; David J. Libon, Drexel University, Philadelphia, Pennsylvania; Jaffar Khan, Emory University; James J Lah, Emory University; Allan I Levey, Emory University; Jonathan D Glass, Emory University
• Language: English
• Date: 2013-02-27
• Publisher: Public Library of Science
• Publication Version: Final Published Version
• Copyright Statement: © 2013 Hu et al.
• License: Creative Commons Attribution 2.0 Generic
• Final Published Version (URL): http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0057584
• Title of Journal or Parent Work: PLoS ONE
• ISSN: 1932-6203
• Volume: 8
• Issue: 2
• Start Page: e57584
• End Page: e57584
• Grant/Funding Information: This study was supported by the Viretta Brady Discovery Fund at Emory University School of Medicine, and NIH AG025688. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
• Research Categories: Biology, Neuroscience

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.