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Author Notes:

Correspondence: William T. Hu; Email: william.hu@emory.edu

Authors' Contributions: Conceived and designed the experiments: WH, MG, DL and JG.

Performed the experiments: WH, MS, AW, NY, CK, JK, JL, AL and JG.

Analyzed the data: WH, MS, CK and JG. .

Contributed reagents/materials/analysis tools: WH, MG, DL and JG.

Wrote the paper: WH and JG

Disclosures: The authors have declared that no competing interests exist.


Research Funding:

This study was supported by the Viretta Brady Discovery Fund at Emory University School of Medicine, and NIH AG025688.

Behavior Matters—Cognitive Predictors of Survival in Amyotrophic Lateral Sclerosis

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Journal Title:



Volume 8, Number 2


, Pages e57584-e57584

Type of Work:

Article | Final Publisher PDF


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.

Copyright information:

© 2013 Hu et al.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/).
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