Longitudinal Screening Detects Cognitive Stability and Behavioral Deterioration in ALS Patients

Area of Science:

• Neuroscience
• Neurology
• Psychiatry

Background:

• Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
• Cognitive and behavioral changes are increasingly recognized in ALS, impacting patient quality of life.
• Longitudinal studies are crucial for understanding the trajectory of these changes.

Purpose of the Study:

• To assess longitudinal cognitive and behavioral changes over 12 months in participants of the ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS).
• To identify correlations between cognitive/behavioral changes and disease progression markers like ALSFRS-R and FVC.

Main Methods:

• Analysis of data from 294 ALS participants, with 134 studied serially over 12 months.
• Statistical methods included multiple regression and one-way analyses of covariance, controlling for demographic and disease factors.
• Evaluated changes in cognitive/behavioral scales (ALS CBS, FBI-ALS) and their association with ALSFRS-R and FVC.

Main Results:

• Significant worsening of behavioral symptoms, including disinhibition, was observed (p < 0.001 for ALS CBS, p = 0.044 for FBI-ALS).
• Specific behavioral changes noted in frustration tolerance, insight, mental rigidity, and interests.
• Changes in ALSFRS-R scores correlated with ALS CBS, but worsening disinhibition did not correlate with ALSFRS-R, FVC, or disease duration.

Conclusions:

• No significant cognitive decline was detected over the 12-month study period.
• Behavioral changes, especially increased disinhibition, were evident, particularly in patients with pre-existing behavioral issues.
• Baseline behavioral problems were linked to advanced, rapidly progressing ALS and increased study attrition, suggesting their role in disease monitoring and patient retention.