A brain scan or psychological test cannot yet reliably tell doctors which specific disease is causing a person’s dementia before they die. Frontotemporal lobar degeneration (FTLD) is a common dementia in younger people, caused by several different diseases that are not Alzheimer’s, and no treatments exist to slow or reverse them. This project will test whether repeated MRI scans and cognitive tests, combined with genetic results, can accurately measure how FTLD first develops and spreads in the brain, and distinguish its subtypes from one another and from Alzheimer’s. The researchers will study a large group of patients with FTLD and at-risk family members already enrolled at their centre, tracking changes over time. If successful, the work will provide the measurement tools needed to run future treatment trials—giving drug developers a way to detect whether an experimental therapy is actually slowing disease progression in the brain. It could also lead to earlier, more accurate diagnoses for younger people with dementia, helping clinicians and families plan care based on the specific disease, not just a generic label.
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Frontotemporal lobar degeneration (FTLD) is a common form of dementia, particularly in younger people, and places a heavy burden on sufferers, families, carers and society at large. FTLD is not usually caused by Alzheimer?s disease, and it may be caused by a number of other diseases. Treatments that could slow or reverse these diseases are urgently needed, but before such treatments can become a reality, we need much more accurate and reliable ways of diagnosing the different types of FTLD and in particular, methods of measuring how these diseases first develop and progress in the brain. Brain scan technology (in particular, MRI) and psychological tests, alone and in combination, have promise as measurement tools but have not been adequately tested in people with FTLD. We plan a large study of patients with FTLD and people from FTLD families that are at risk of developing the disease, to test possible methods of measuring disease as it begins and spreads in the brain, and to decide with measurement methods could best be used in future treatment trials. We will use repeated MRI brain scans and psychology testing to measure disease changes and we will combine this information with other information, including the results of genetic tests, to build up a picture of the variation within the FTLD population. We will also compare these with the same measurements in patients with Alzheimer?s disease. This will tell us how to diagnose FTLD early and accurately, how we should measure the ways that the disease affects the brain over time, and which kinds of measurement would best allow us to assess a treatment in future. In carrying out this work we will draw upon a large number of volunteer patients with FTLD and members of FTLD families who are already involved in research at our Centre, and we will capitalise on our Centre?s long experience in developing new methods of assessing the onset and effects of dementia diseases. We hope that this work will help us find methods of measuring disease effects that will help with diagnosis and treatment trials in the large population of FTLD sufferers, and help guide future health care delivery in younger people with dementia.
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