The age of onset is typically in the 50s or 60s but can be as young as 30. The disease is sometimes called frontotemporal lobar degeneration and was first described 100 years ago by Arnold Pick and was previously referred to as Pick’s disease.
Damage to brain cells begins in the frontal and/or temporal lobes of the brain. When the initial damage is in the frontal lobe (called behavioural-variant FTD) the main changes are in personality and behaviour. Individuals with damage predominantly in the temporal lobe (either progressive non-fluent aphasia or semantic dementia) lose the ability to speak or understand language.
Our research is investigating the cognitive, behavioural, psychological and physical brain changes associated with frontotemporal dementia, as well as the impact of the disease on the lives of patients and their families. We are also looking at the genetics of frontotemporal dementia, and are currently conducting animal studies and designing a clinical trial of a potential treatment to slow the disease.
The brains of people with frontotemporal dementia (FTD) show a severe loss of brain cells (neurons). In some individuals protein known as 'tau' collects in neurons known as ‘Pick bodies’. More commonly, the brains of people with FTD shows an accumulation of another cell protein called ubiquitin. Very recent research has suggested that the accumulation of ubiquitin is attached to another protein called TDP-43, which has a fundamental role in the nuclei of brain cells.
In frontotemporal dementia, as the name suggests, these changes may be present in the frontal lobes, the temporal lobes or both. As the disease progresses these brain regions show shrinkage.
Almost a third of patients with FTD have a family history of dementia, if you include relatives with any sort of dementia coming on at any age. True familial FTD, however, is much rarer, accounting for about 10 -15% of cases. The genetic basis of FTD is not fully understood and is a topic of active research.
The members of the Frontotemporal dementia research group (FRONTIER) are investigating the cognitive, behavioural, psychological, and brain changes associated with frontotemporal dementia and the impact on patients and their families. We are also studying how changes in FTD differ from those seen in other progressive neurodegenerative brain disorders, and in healthy ageing.
The Frontier Group conducted one of the first comprehensive descriptions of semantic dementia (1992), one of the principal forms of FTD. In further studies of semantic dementia we have discovered a specific reading deficit which has been important for theories related to how the brain processes spoken and written words, while our studies of object usage helped to understand the organisation of knowledge in the brain. More recently our work in FTD has been showing deficits in emotion processing, decision-making and Theory of Mind, which have been a crucial first step in understanding the emotional and behavioural disturbances in FTD.
The Kwok and Schofield Groups have recently published their findings on a gene that causes frontotemporal dementia, called SIGMAR1. This gene was discovered by scanning the DNA of a large Australian family with hereditary FTD. In this family, SIGMAR1 is responsible for the abnormal clumping of protein in brain which causes the symptoms of FTD.
The next stage of this research is to test drugs that we believe may act on this clumping process. You can read more about this discovery in our news story ('Common psychiatric drugs could slow dementia') and here: 'The SIGMAR1 gene – further information for families, caregivers and patients with FTD'.
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