Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients

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 P Nations, Andrea Swenson, Agnes Koczon-Jaremko, Georgia ChristodoulouHiroshi Mitsumoto

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish (US)
Number of pages1
JournalBehavioural Neurology
Volume2018
DOIs
StatePublished - Jan 1 2018
Externally publishedYes

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Vital Capacity
Equipment and Supplies
Frustration
Multicenter Studies
Oxidative Stress
Cohort Studies
Education
Problem Behavior

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Neurology
  • Clinical Neurology

Cite this

Woolley, S., Goetz, R., Factor-Litvak, P., Murphy, J., Hupf, J., Lomen-Hoerth, C., ... Mitsumoto, H. (2018). Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients. Behavioural Neurology, 2018. https://doi.org/10.1155/2018/5969137

Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients. / Woolley, Susan; Goetz, Ray; Factor-Litvak, Pam; Murphy, Jennifer; Hupf, Jonathan; Lomen-Hoerth, Catherine; Andrews, Howard; Heitzman, Daragh; Bedlack, Richard; Katz, Jonathan; Barohn, Richard; Sorenson, Eric; Oskarsson, Bjorn; Filho, Americo Fernandes; Kasarskis, Edward; Mozaffar, Tahseen; Nations, Sharon P; Swenson, Andrea; Koczon-Jaremko, Agnes; Christodoulou, Georgia; Mitsumoto, Hiroshi.

In: Behavioural Neurology, Vol. 2018, 01.01.2018.

Research output: Contribution to journalArticle

Woolley, S, Goetz, R, Factor-Litvak, P, Murphy, J, Hupf, J, Lomen-Hoerth, C, Andrews, H, Heitzman, D, Bedlack, R, Katz, J, Barohn, R, Sorenson, E, Oskarsson, B, Filho, AF, Kasarskis, E, Mozaffar, T, Nations, SP, Swenson, A, Koczon-Jaremko, A, Christodoulou, G & Mitsumoto, H 2018, 'Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients', Behavioural Neurology, vol. 2018. https://doi.org/10.1155/2018/5969137
Woolley, Susan ; Goetz, Ray ; Factor-Litvak, Pam ; Murphy, Jennifer ; Hupf, Jonathan ; Lomen-Hoerth, Catherine ; Andrews, Howard ; Heitzman, Daragh ; Bedlack, Richard ; Katz, Jonathan ; Barohn, Richard ; Sorenson, Eric ; Oskarsson, Bjorn ; Filho, Americo Fernandes ; Kasarskis, Edward ; Mozaffar, Tahseen ; Nations, Sharon P ; Swenson, Andrea ; Koczon-Jaremko, Agnes ; Christodoulou, Georgia ; Mitsumoto, Hiroshi. / Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients. In: Behavioural Neurology. 2018 ; Vol. 2018.
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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.",
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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 P

AU - Swenson, Andrea

AU - Koczon-Jaremko, Agnes

AU - Christodoulou, Georgia

AU - Mitsumoto, Hiroshi

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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.

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