好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Frequency and Predictors of Cognitive Deterioration in Amyotrophic Lateral Sclerosis: A Population-based Longitudinal Study
Neuromuscular and Clinical Neurophysiology (EMG)
S5 - Updates on Amyotrophic Lateral Sclerosis (4:30 PM-4:42 PM)
006
Despite advances in understanding cognitive and behavioral impairments in ALS, how these functions change over time is still uncertain. Existing longitudinal studies have yielded inconsistent results
This study aimed to assess the progression of cognitive and behavioral changes and identify early predictors of these alterations in ALS patients.
A total of 161 ALS patients were evaluated within one month of diagnosis (T0) and re-assessed after one year (T1) using a comprehensive set of cognitive and behavioral tests. Whole genome sequencing was performed on all participants, and 26 patients with normal cognitive function (ALS-CN) underwent 18F-FDG PET imaging at T0.
Of the 161 patients, 107 completed the follow-up at T1. The remaining patients included 10 with frontotemporal dementia and 44 who were either unable to participate or had passed away. At T0, 67 patients (62.6%) were cognitively normal (ALS-CN), while 40 (38.4%) exhibited some level of cognitive or behavioral impairment. By T1, 18 ALS-CN patients (26.9%) showed cognitive decline. Those who experienced phenoconversion had lower baseline scores in Letter Fluency (FAS) (p<0.001) and ECAS Verbal Fluency (p=0.017). Both measures independently predicted phenoconversion in logistic regression models, with cut-off values of 28.75 and 14.2, providing good sensitivity and specificity. Additional predictors included older age, lower education, and specific ALS-related genetic mutations. Phenoconverting patients also exhibited hypometabolism in the left temporal lobe. Among the 40 patients with cognitive impairment at T0, 13 (32.5%) showed further deterioration by T1, with FAS (p=0.02) and ECAS Verbal Fluency (p=0.023) scores predicting their decline.

Around 30% of ALS patients in this cohort experienced cognitive or behavioral decline within one year of diagnosis. FAS and ECAS Verbal Fluency emerged as reliable predictors of cognitive phenoconversion. These findings underscore the importance of identifying at-risk individuals early and conducting regular cognitive assessments to monitor disease progression.
Authors/Disclosures
Maurizio Grassano, MD (Dept. of Neuroscience, University of Turin)
PRESENTER 		Dr. Grassano has received research support from American Brain Foundation, ALS Association and 好色先生.
Adriano Chio, MD, FAAN (Dept. of Neuroscience, University of Turin) Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cytokinetics. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Corcept.
Francesca Palumbo (University of Turin Department of Neurosciences: Universita degli Studi di Torin) Dr. Palumbo has nothing to disclose.
Cristina Moglia (University of Torino) Cristina Moglia has nothing to disclose.
Umberto Manera, MD (Department of Neuroscience "Rita Levi Montalcini" - University of Torino) Dr. Manera has nothing to disclose.
Rosario Vasta, MD (University of Turin, Department of Neurosciences) Dr. Vasta has nothing to disclose.
Gabriele Mora, MD Dr. Mora has nothing to disclose.
Antonio Canosa Antonio Canosa has nothing to disclose.
Barbara Iazzolino Barbara Iazzolino has nothing to disclose.
Andrea Calvo, MD, PhD, FAAN (Dept. of Neuroscience, University of Turin) Dr. Calvo has nothing to disclose.