Abstract
Objectives
To examine the usefulness of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis (ALS) Screen (ECAS) as a cognitive screening tool for the detection of behavioural variant FTD (bvFTD). A secondary aim was to determine whether people with FTD combined with ALS (ALS-FTD) exhibit a similar ECAS profile to that of people with bvFTD alone.
Methods
Patients with ALS-FTD and bvFTD and healthy controls were recruited. Participants were administered the ECAS, which comprises tests of language, verbal fluency, executive functions, memory and visual spatial functions. They also carried out analogous, full-length cognitive tests that examine naming, spelling, sentence completion and social cognition
skills.
Results
The study cohort comprised 20 ALS-FTD patients, 23 with bvFTD and 30 controls. Highly significant group differences were elicited for all cognitive domains, reflecting poorer performance in patients compared to controls. No significant differences in overall test scores were found between ALS-FTD and bvFTD patients, although ALS-FTD patients showed a higher frequency of impairment on verbal fluency. Correlative analyses revealed inter-relationships in patients (but not controls) between scores in different domains, most
marked in bvFTD. There were strong correlations between performance on ECAS subtests and analogous cognitive tasks.
Conclusion
The ECAS is a sensitive and valuable tool for the assessment of FTD. Executive, language and behavioural breakdown may, however, compromise performance in other cognitive domains, reducing the specificity of the ‘frontotemporal’ cognitive profile. Subtle differences observed between ALS-FTD and bvFTD raise questions regarding the precise relationship between bvFTD with and without ALS.
To examine the usefulness of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis (ALS) Screen (ECAS) as a cognitive screening tool for the detection of behavioural variant FTD (bvFTD). A secondary aim was to determine whether people with FTD combined with ALS (ALS-FTD) exhibit a similar ECAS profile to that of people with bvFTD alone.
Methods
Patients with ALS-FTD and bvFTD and healthy controls were recruited. Participants were administered the ECAS, which comprises tests of language, verbal fluency, executive functions, memory and visual spatial functions. They also carried out analogous, full-length cognitive tests that examine naming, spelling, sentence completion and social cognition
skills.
Results
The study cohort comprised 20 ALS-FTD patients, 23 with bvFTD and 30 controls. Highly significant group differences were elicited for all cognitive domains, reflecting poorer performance in patients compared to controls. No significant differences in overall test scores were found between ALS-FTD and bvFTD patients, although ALS-FTD patients showed a higher frequency of impairment on verbal fluency. Correlative analyses revealed inter-relationships in patients (but not controls) between scores in different domains, most
marked in bvFTD. There were strong correlations between performance on ECAS subtests and analogous cognitive tasks.
Conclusion
The ECAS is a sensitive and valuable tool for the assessment of FTD. Executive, language and behavioural breakdown may, however, compromise performance in other cognitive domains, reducing the specificity of the ‘frontotemporal’ cognitive profile. Subtle differences observed between ALS-FTD and bvFTD raise questions regarding the precise relationship between bvFTD with and without ALS.
| Original language | English |
|---|---|
| Pages (from-to) | 606-613 |
| Number of pages | 8 |
| Journal | Amyotrophic lateral sclerosis & frontotemporal degeneration |
| Volume | 21 |
| Issue number | 7 |
| Publication status | Published - Nov 2020 |
Keywords
- motor neurone disease
- frontotemporal dementia
- congnition