Author/Authors :
Woolley, Susan Department of Neurosciences - Forbes Norris ALS Research Center - Sutter Pacific Medical Foundation, USA , Goetz, Ray Department of Psychiatry - New York State Psychiatric Institute - Columbia University Medical Center (CUMC), USA , Factor-Litvak, Pam Department of Epidemiology - CUMC - Mailman School of Public Health, USA , Murphy, Jennifer Department of Neurology - University of California - San Francisco (UCSF), USA , Hupf, Jonathan Department of Neurology - Eleanor and Lou Gehrig MD/ALS Research Center, USA , Lomen-Hoerth, Catherine Department of Neurology, UCSF, USA , Andrews, Howard Departments of Biostatistics and Psychiatry - CUMC - Mailman School of Medicine, USA , Heitzman, Daragh Texas Neurology, PA, USA , Bedlack, Richard Duke University, USA , Katz, Jonathan Department of Neurosciences - Forbes Norris ALS Research Center - Sutter Pacific Medical Foundation, USA , Barohn, Richard Department of Neurology - University of Kansas, USA , Sorenson, Eric Mayo Clinic, Rochester, USA , Oskarsson, Bjorn University of California - Davis, Mayo Clinic, Jacksonville, USA , Fernandes Filho, Americo University of Nebraska Medical Center, USA , Kasarskis, Edward University of Kentucky, Lexington, USA , Mozaffar, Tahseen University of California, Irvine, USA , Nations, Sharon University of Texas, Southwestern, USA , Swenson, Andrea University of Iowa, USA , Koczon-Jaremko, Agnes Hospital for Special Care, USA , Christodoulou, Georgia Department of Neurology - Eleanor and Lou Gehrig MD/ALS Research Center, USA , Mitsumoto, Hiroshi Department of Neurology - Eleanor and Lou Gehrig MD/ALS Research Center, USA
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.
