Behavior matters--cognitive predictors of survival in amyotrophic lateral sclerosis.
PLoS One, 2013;8(2):e57584.
Hu WT[1], Shelnutt M, Wilson A, Yarab N, Kelly C, Grossman M, Libon DJ, Khan J, Lah JJ, Levey AI, Glass J
Affiliations
PMID: 23460879DOI: 10.1371/journal.pone.0057584
Impact factor: 3.752
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.
MeSH terms
Aged; Amyotrophic Lateral Sclerosis; Behavior; Cognition; Dementia; Female; Humans; Kaplan-Meier Estimate; Longitudinal Studies; Male; Middle Aged; Proportional Hazards Models; ROC Curve; Reproducibility of Results
More resources
Full text:
Europe PubMed Central; PubMed Central
EndNote: Download