Autor: |
Saxon JA; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK., Thompson JC; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK., Harris JM; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK., Ealing J; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK.; Motor Neurone Disease Care Centre, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK., Hamdalla H; Motor Neurone Disease Care Centre, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK., Chaouch A; Motor Neurone Disease Care Centre, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK., Young C; The Walton Centre NHS Foundation Trust, Liverpool, UK.; Institute of Translational Medicine, University of Liverpool, Liverpool, UK., Blackburn D; Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK, and., Majeed T; Lancashire Teaching Hospital NHS Foundation Trust, Preston, UK., Gall C; Lancashire Teaching Hospital NHS Foundation Trust, Preston, UK., Richardson AMT; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK., Langheinrich T; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK., Jones M; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK., Snowden JS; Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.; Division of Neuroscience and Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, UK. |
Abstrakt: |
Objectives: To examine the usefulness of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis (ALS) Screen (ECAS) as a cognitive screening tool for the detection of behavioral variant frontotemporal dementia (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 behavioral 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. |