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Updated: Aug 5, 2026
08:59
Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
Published on: July 16, 2021
Neuropsychological Sub-Phenotypes in Amyotrophic Lateral Sclerosis
Barbara Poletti1,2, Edoardo Nicolò Aiello1, Monica Consonni3
1Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milano, Italy.
European Journal of Neurology
|August 3, 2026
Summary
This study identified distinct cognitive and behavioral subtypes in amyotrophic lateral sclerosis (ALS) patients using the ECAS tool. These findings offer a new framework for understanding neuropsychological profiles in non-demented ALS.
Area of Science:
- Neuroscience
- Neurology
- Cognitive Science
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
- Neuropsychological impairments, including mild cognitive impairment (MCI) and mild behavioral impairment (MBI), are common in ALS.
- Identifying distinct neuropsychological sub-phenotypes is crucial for understanding disease heterogeneity and progression.
Purpose of the Study:
- To identify neuropsychological sub-phenotypes in non-demented ALS patients.
- To apply frameworks of mild cognitive impairment (MCI) and mild behavioral impairment (MBI).
- To utilize an expanded version of Strong's criteria incorporating memory and visuo-spatial measures.
Main Methods:
- Utilized individual task/item-level data from the Edinburgh Cognitive and Behavioral ALS Screen (ECAS).
- Analyzed data from 901 non-demented ALS patients.
- Derived sub-phenotypes based on classical MCI and MBI frameworks and expanded criteria.
Main Results:
- Prevalence of MCI was 39% and MBI was 37%.
- Identified dysexecutive (dMCI) and non-dysexecutive (ndMCI) MCI sub-phenotypes.
- Classified MBI into categories including apathetic (aMBI), apathetic-disinhibited/perseverative (ad/pMBI), disinhibited/perseverative (d/pMBI), and psychotic (psyMBI).
- 16% of patients exhibited mild cognitive and behavioral impairment (MCBI).
Conclusions:
- Developed a provisional ECAS-based classification for neuropsychological sub-phenotyping in non-demented ALS.
- This classification may aid research and clinical practice.
- Further validation is needed to confirm the utility of this sub-phenotyping approach.
Keywords:
amyotrophic lateral sclerosisedinburgh cognitive and behavioral ALS screenfrontotemporal‐spectrum disorderneuropsychologyphenotyperesearch criteriaMore Related Videos
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