TY - JOUR
T1 - Longitudinal screening detects cognitive stability and behavioral deterioration in ALS patients
AU - Woolley, Susan
AU - Goetz, Ray
AU - Factor-Litvak, Pam
AU - Murphy, Jennifer
AU - Hupf, Jonathan
AU - Lomen-Hoerth, Catherine
AU - Andrews, Howard
AU - Heitzman, Daragh
AU - Bedlack, Richard
AU - Katz, Jonathan
AU - Barohn, Richard
AU - Sorenson, Eric
AU - Oskarsson, Bjorn
AU - Filho, Americo Fernandes
AU - Kasarskis, Edward
AU - Mozaffar, Tahseen
AU - Nations, Sharon
AU - Swenson, Andrea
AU - Koczon-Jaremko, Agnes
AU - Christodoulou, Georgia
AU - Mitsumoto, Hiroshi
N1 - Funding Information:
The authors acknowledged B. A. Jessica Singleton who was a research assistant during part of the ALS COSMOS study. We thank the many patients and families who participated in this study and the ALS COSMOS Study Group site investigators. The authors also acknowledged Ray Goetz, PhD, Department of Psychiatry, New York State Psychiatric Institute, Columbia University Medical Center, who died in December 2017, for conducting the statistical analysis. 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, GlaxoS-mithKline, 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.
Publisher Copyright:
© 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.
PY - 2018
Y1 - 2018
N2 - 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.
AB - 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.
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U2 - 10.1155/2018/5969137
DO - 10.1155/2018/5969137
M3 - Article
C2 - 30515252
AN - SCOPUS:85060544675
SN - 0953-4180
VL - 2018
JO - Behavioural neurology
JF - Behavioural neurology
M1 - 5969137
ER -