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PLoS One. 2013;8(2):e57584. doi: 10.1371/journal.pone.0057584. Epub 2013 Feb 27.

Behavior matters--cognitive predictors of survival in amyotrophic lateral sclerosis.

Author information

1
Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, United States of America. william.hu@emory.edu

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.

PMID:
23460879
PMCID:
PMC3583832
DOI:
10.1371/journal.pone.0057584
[Indexed for MEDLINE]
Free PMC Article

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