Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients

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 control...

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Main Authors: Susan Woolley, Ray Goetz, Pam Factor-Litvak, Jennifer Murphy, Jonathan Hupf, Catherine Lomen-Hoerth, Howard Andrews, Daragh Heitzman, Richard Bedlack, Jonathan Katz, Richard Barohn, Eric Sorenson, Bjorn Oskarsson, Americo Fernandes Filho, Edward Kasarskis, Tahseen Mozaffar, Sharon Nations, Andrea Swenson, Agnes Koczon-Jaremko, Georgia Christodoulou, Hiroshi Mitsumoto
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Behavioural Neurology
Online Access:http://dx.doi.org/10.1155/2018/5969137
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author Susan Woolley
Ray Goetz
Pam Factor-Litvak
Jennifer Murphy
Jonathan Hupf
Catherine Lomen-Hoerth
Howard Andrews
Daragh Heitzman
Richard Bedlack
Jonathan Katz
Richard Barohn
Eric Sorenson
Bjorn Oskarsson
Americo Fernandes Filho
Edward Kasarskis
Tahseen Mozaffar
Sharon Nations
Andrea Swenson
Agnes Koczon-Jaremko
Georgia Christodoulou
Hiroshi Mitsumoto
author_facet Susan Woolley
Ray Goetz
Pam Factor-Litvak
Jennifer Murphy
Jonathan Hupf
Catherine Lomen-Hoerth
Howard Andrews
Daragh Heitzman
Richard Bedlack
Jonathan Katz
Richard Barohn
Eric Sorenson
Bjorn Oskarsson
Americo Fernandes Filho
Edward Kasarskis
Tahseen Mozaffar
Sharon Nations
Andrea Swenson
Agnes Koczon-Jaremko
Georgia Christodoulou
Hiroshi Mitsumoto
author_sort Susan Woolley
collection DOAJ
description 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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spelling doaj-art-35f6be91ed3046459fe8d22778be5b372025-02-03T05:47:54ZengWileyBehavioural Neurology0953-41801875-85842018-01-01201810.1155/2018/59691375969137Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS PatientsSusan Woolley0Ray Goetz1Pam Factor-Litvak2Jennifer Murphy3Jonathan Hupf4Catherine Lomen-Hoerth5Howard Andrews6Daragh Heitzman7Richard Bedlack8Jonathan Katz9Richard Barohn10Eric Sorenson11Bjorn Oskarsson12Americo Fernandes Filho13Edward Kasarskis14Tahseen Mozaffar15Sharon Nations16Andrea Swenson17Agnes Koczon-Jaremko18Georgia Christodoulou19Hiroshi Mitsumoto20Department of Neurosciences, Forbes Norris ALS Research Center, Sutter Pacific Medical Foundation, USADepartment of Psychiatry, New York State Psychiatric Institute, Columbia University Medical Center (CUMC), USADepartment of Epidemiology, CUMC, Mailman School of Public Health, USADepartment of Neurology, University of California, San Francisco (UCSF), USADepartment of Neurology, Eleanor and Lou Gehrig MD/ALS Research Center, CUMC, USADepartment of Neurology, UCSF, USADepartments of Biostatistics and Psychiatry, CUMC, Mailman School of Medicine, USATexas Neurology, PA, USADuke University, USADepartment of Neurosciences, Forbes Norris ALS Research Center, Sutter Pacific Medical Foundation, USADepartment of Neurology, University of Kansas, USAMayo Clinic, Rochester, USAUniversity of California, Davis, Mayo Clinic, Jacksonville, USAUniversity of Nebraska Medical Center, USAUniversity of Kentucky, Lexington, USAUniversity of California, Irvine, USAUniversity of Texas, Southwestern, USAUniversity of Iowa, USAHospital for Special Care, USADepartment of Neurology, Eleanor and Lou Gehrig MD/ALS Research Center, CUMC, USADepartment of Neurology, Eleanor and Lou Gehrig MD/ALS Research Center, CUMC, USAObjective. 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.http://dx.doi.org/10.1155/2018/5969137
spellingShingle Susan Woolley
Ray Goetz
Pam Factor-Litvak
Jennifer Murphy
Jonathan Hupf
Catherine Lomen-Hoerth
Howard Andrews
Daragh Heitzman
Richard Bedlack
Jonathan Katz
Richard Barohn
Eric Sorenson
Bjorn Oskarsson
Americo Fernandes Filho
Edward Kasarskis
Tahseen Mozaffar
Sharon Nations
Andrea Swenson
Agnes Koczon-Jaremko
Georgia Christodoulou
Hiroshi Mitsumoto
Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients
Behavioural Neurology
title Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients
title_full Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients
title_fullStr Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients
title_full_unstemmed Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients
title_short Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients
title_sort longitudinal screening detects cognitive stability and behavioral deterioration in als patients
url http://dx.doi.org/10.1155/2018/5969137
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