Bibliographic Details
| Title: |
Examining the language and behavioural profile in FTD and ALS-FTD. |
| Authors: |
Saxon, Jennifer A., Thompson, Jennifer c., Jones, Matthew, harris, Jennifer M., Richardson, Anna M. T., Langheinrich, Tobias, Neary, David, Mann, David M. A., Snowden, Julie S., Richardson, Anna Mt, Mann, David Ma |
| Source: |
Journal of Neurology, Neurosurgery & Psychiatry; Aug2017, Vol. 88 Issue 8, p675-680, 6p, 4 Charts |
| Subject Terms: |
FRONTOTEMPORAL dementia, AMYOTROPHIC lateral sclerosis, COGNITION disorders, NEUROPSYCHOLOGICAL tests, BEHAVIOR, AFFECTIVE disorders, LANGUAGE disorder diagnosis, BEHAVIOR disorders, SOCIAL disabilities, AGRAMMATISM, WORD deafness, COMPARATIVE studies, DIFFERENTIAL diagnosis, LONGITUDINAL method, RESEARCH methodology, MEDICAL cooperation, RESEARCH, EVALUATION research, RETROSPECTIVE studies, DISEASE progression, EARLY diagnosis, DIAGNOSIS |
| Abstract: |
Background: A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone.Methods: A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms. Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment.Results: A number of behavioural and affective changes were reported more frequently in bvFTD than ALS-FTD: social disinhibition (p<0.001), inertia (p<0.001), loss of sympathy and empathy (p=0.008), repetitive behaviours (p<0.001) and dietary changes (p<0.001). Warmth of affect demonstrated in the clinic setting was reported more often in ALS-FTD than bvFTD (p<0.001). Executive impairments occurred equally in both groups. Language impairments were more common in ALS-FTD than bvFTD: agrammatism (p<0.017) and impaired sentence comprehension (p<0.036). Psychotic features were relatively rare and did not distinguish the groups.Conclusions: Our findings suggest differences between bvFTD and ALS-FTD. In particular, while changes in social behaviour are prominent in bvFTD alone, there may be a comparatively greater degree of language impairment in ALS-FTD. Prospective exploration of the pattern of differences between these groups will be essential. Identification of a distinct neuropsychological phenotype in ALS-FTD may have clinical implications for early diagnosis, disease management and care planning and theoretical implications for our understanding of the relationship between ALS and FTD. [ABSTRACT FROM AUTHOR] |
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| Database: |
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