Sampson, Elizabeth L.; (2003) Longitudinal studies in frontotemporal dementia. Doctoral thesis (Ph.D.), University College London (United Kingdom).

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Abstract

Background Frontotemporal dementia (FTD) is a common cause of young onset dementia. The behavioural, language and personality changes cause considerable distress to patients and their carers. The current diagnostic criteria for FTD describe two distinct clinical subtypes, one mainly affecting language and one causing a predominantly behavioural syndrome. These are thought to be associated with particular patterns of underlying neuropathological change affecting the frontal or temporal lobe. Aims The aim was to investigate the longitudinal evolution of FTD and compare the subtypes of FTD in terms of cognition, function, behavioural problems and patterns of cerebral atrophy as demonstrated by neuroimaging. The relationship of these patient factors with distress and burden in carers was also evaluated. Method Patients were recruited from the cognitive disorders clinic at the National Hospital for Neurology and Neurosurgery, London. They underwent a range of assessments of their cognitive function, functional ability, psychiatric symptoms and behavioural problems at baseline and after one year. The carers completed measures of carer distress, mental health, burden and service utilisation at baseline and after one year. Volumetric neuroimaging was used to establish the predominant areas of cerebral atrophy and calculate tissue loss over 1 year. The behavioural and cognitive profiles of patients were compared by clinical presentation (behavioural or language form) and according to the pattern of cerebral atrophy. Results The cohort comprised of 42 patients with frontotemporal dementia and their carers. The majority of patients, regardless of the subtype of FTD, displayed behavioural disturbances. Patients who presented with language problems or with temporal lobe atrophy showed a more severe course with greater decline in cognitive measures, functional ability, behaviour and brain volume lost. Most carers showed significant levels of burden and distress which did not decrease during the study. Conclusions There are no clear behavioural syndromes that differentiate the subtypes of frontotemporal dementia. Patients presenting with predominant language problems appear to experience a more rapid disease course.

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