Abstract

Objective. To evaluate longitudinal cognitive/behavioral change over 12 months in participants enrolled in the ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS). Methods. We analyzed data from 294 ALS participants, 134 of whom were studied serially. Change over time was evaluated controlling for age, sex, symptom duration, education, race, and ethnicity. Using multiple regression, we evaluated associations among decline in ALS Functional Rating Scale-Revised (ALSFRS-R) scores, forced vital capacity (FVC), and cognitive/behavioral changes. Change in cognitive/behavioral subgroups was assessed using one-way analyses of covariance. Results. Participants with follow-up data had fewer baseline behavior problems compared to patients without follow-up data. We found significant worsening of behavior (ALS Cognitive Behavioral Screen (ALS CBS) behavioral scale, p < 0.001; Frontal Behavioral Inventory-ALS (FBI-ALS) disinhibition subscale, p = 0.044). Item analysis suggested change in frustration tolerance, insight, mental rigidity, and interests (p < 0.05). Changes in ALSFRS-R correlated with the ALS CBS. Worsening disinhibition (FBI-ALS) did not correlate with ALSFRS-R, FVC, or disease duration. Conclusion. We did not detect cognitive change. Behavioral change was detected, and increased disinhibition was found among patients with abnormal baseline behavioral scores. Disinhibition changes did not correlate with disease duration or progression. Baseline behavioral problems were associated with advanced, rapidly progressive disease and study attrition.

Document Type

Article

Publication Date

10-31-2018

Notes/Citation Information

Published in Behavioural Neurology, v. 2018, article ID 5969137, p. 1-7.

© 2018 Susan Woolley et al.

This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Digital Object Identifier (DOI)

https://doi.org/10.1155/2018/5969137

Funding Information

Pam Factor-Litvak received travel funds from the Environment and Health Fund and travel funds and honorarium to serve as a reviewer for the European Union 2020 grant initiative. Daragh Heitzman received financial support from the Muscular Dystrophy Association and research support from National Institute of Health (via Columbia University Subcontract), Biogen Idec, Questcor Pharmaceuticals, Merz Pharmaceuticals, and Cytokinetics for research trials as a site investigator. Richard Bedlack received consulting support from ALSA, Neuraltus, Ultragenyx, Pinnacle Research Group, Lighthouse Research Group, and Guidepoint Global and research support from Amyotrophic Lateral Sclerosis Association, Motor Neurone Disease Association, Iron Horse Diagnostics, Cytokinetics, Neuraltus, and GlaxoSmithKline. Bjorn Oskarsson received research support from NIH, Amyotrophic Lateral Sclerosis Association, Novartis, BMS, and Cytokinetics. Tahseen Mozaffar received personal compensation from consulting activities to Baxter, Biogen Idec, BioMarin, California Stem Cell Inc., Crescent (a Walgreens company) CSL Behring, Genzyme, Grifols, Idera, NuFACTOR, and Ultragenyx. Dr. Mozaffar received funding from NIH (#NS049203) and received clinical research support from ALS Therapy Development Institute, Alexion Pharmaceuticals, Alnylam Pharmaceuticals, Amicus, Biogen Idec, BioMarin Pharmaceutical, CSL Behring, Cytokinetics, Food and Drug Administration, Grifols, Genzyme, GlaxoSmithKline, ISIS, Neuraltus, Novartis, and Ultragenyx Pharmaceutical. Hiroshi Mitsumoto received government research support from NIEHS (R01ES016348) and research support from MDA Wings Over Wall Street for the publication. This study was funded by NIEHS (R01ES016348), MDA Wings Over Wall Street, and Anthony Senerchia Jr. ALS Charitable Foundation.

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Data Availability

This multicenter trial does not currently have IRB permission to share data. Permission was requested by the lead authors but the IRB had not granted approval by the time of publication.

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