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Updated: Aug 5, 2026
07:03
Evaluation of Hemisphere Lateralization with Bilateral Local Field Potential Recording in Secondary Motor Cortex of Mice
Published on: July 31, 2019
Early Cognitive and Behavioral Changes in Primary Lateral Sclerosis: A Population-Based Study
Andrea Calvo1,2, Cristina Moglia1,2, Antonio Canosa1,2,3
1'Rita Levi Montalcini' Department of Neuroscience, ALS Center, University of Torino, Turin, Italy.
European Journal of Neurology
|August 1, 2026
Summary
Cognitive and behavioral issues are common in primary lateral sclerosis (PLS) at diagnosis, affecting executive function and memory. These findings highlight PLS as a neurodegenerative disorder with early extramotor involvement.
Area of Science:
- Neuroscience
- Neurology
Background:
- Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder affecting upper motor neurons.
- The cognitive and behavioral profile of PLS, especially in early stages, requires further definition.
Purpose of the Study:
- To characterize the cognitive and behavioral features of PLS at diagnosis.
- To compare these features with predominant upper motor neuron amyotrophic lateral sclerosis (PUMN-ALS) and healthy controls (HCs).
Main Methods:
- Patients diagnosed with PLS between 2007 and 2021 were identified from a population-based ALS register.
- Comprehensive neuropsychological and behavioral assessments were performed within 3 months of the first ALS center visit.
- Cognitive-behavioral status was classified using ALS-frontotemporal dementia (FTD) consensus criteria.
Main Results:
- Twenty-nine percent of PLS patients (including 21.1% in early stages) showed cognitive/behavioral impairment, primarily affecting executive function, memory, and social cognition.
- PLS patients performed worse across cognitive domains and had higher anxiety/depression scores than HCs.
- PLS patients had slightly worse executive performance than PUMN-ALS patients, with similar overall impairment frequency but different behavioral profiles (apathy more frequent in PUMN-ALS).
Conclusions:
- Cognitive and behavioral impairments are detectable at diagnosis in a significant proportion of PLS patients, including early-stage cases.
- These findings support the classification of PLS as a multidimensional neurodegenerative disorder with early extramotor system involvement.
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