What is it and what causes it?
Frontotemporal dementia (FTD) is an umbrella term for a group of brain diseases that affect the frontal and temporal lobes of the brain, the areas of the brain that are associated with personality, behaviour and language. It is one of the less common types of dementia and is mostly diagnosed in people under 65.
As nerve cells are damaged and die, the brain tissue in the frontal and temporal lobes shrink, also known as atrophy.
Lady Helen Stewart was diagnosed with FTD in 2014. This inspired Sir Jackie to found Race Against Dementia, to fund research into preventative treatments and cures.
The subtypes of frontotemporal dementia include :
- Behavioural variant FTD: The most common form, also known as Pick’s Disease, where personality and behaviour
change dramatically. People may become impulsive, distracted easily, withdrawn or behave in socially inappropriate ways.
- Primary progressive aphasia (PPA): Initially causes difficulties with language, with some having trouble remembering words and names of objects or struggling to use words in the right order.
In clinical terms there is also a lot of overlap between these main variants of frontotemporal dementia and moto neuron disease, meaning the conditions can be similar in many ways.
As with most forms of dementia, FTD is progressive, meaning symptoms may be mild to begin with, but will get worse over time. Many people with FTD don’t have a significant problem with their memory during the early stages.
Inside the labs researching Frontotemporal dementia
At Race Against Dementia, we support a number of projects that are investigating frontotemporal dementia, here are just a few of them.
Team FTD – University of Cambridge
Team FTD are running trials to test new treatments for FTD, primary progressive aphasia, progressive supranuclear palsy and corticobasal syndrome.
Each have their own root causes, but they share some distressing symptoms. Behavioural change, impulsivity, personality change, apathy and obsessional habits can be experienced by people living with these conditions. These challenging behaviours have no proven effective treatments currently and can cause considerable distress to individuals and their loved ones.
Research has suggested that a lack of a substance called serotonin in the brain contributes to the challenging behaviours. The team have proposed a clinical trial that will test a widely used drug, citalopram, that increases serotonin levels compared to a placebo.
FTD has been largely excluded from clinical trials, leaving individuals with FTD conditions without treatment. The team propose to address this urgent unmet need.
It will be the first FTD trial to recruit by symptom rather than diagnosis (a basket trial), overcoming barriers of disease complexity. By repurposing existing medicines, the team hope to show that basket trials can deliver urgently needed symptomatic relief, with potential to extend to disease-modifying therapies.
Dr Aitana Sogorb-Esteve – Center for Research on Neurological Diseases (CIEN), Madrid
Dr Aitana’s Race Against Dementia fellowship focuses on identifying new biomarkers in blood that could help diagnose FTD.
There are several different ways in which scientists can go about looking for signals in the blood to tell them what is going on in the brain and Aitana is focusing on synapses in the brain. The method she is developing measures signs of damage to synapses, the connections between brain cells, using blood samples from individuals with genetic FTD. The work is part of the GENetic FTD Initiative, a large international study that follows people with a family history of FTD.
Aitana is working to improve the extraction of extracellular vesicles, tiny particles that carry biological information, from blood samples. Funding from her Race Against Dementia fellowship enabled Aitana to acquire advanced automated equipment, transforming the efficiency of her research process and enhancing the precision of her work.
Dr Yazead Buhidma – University College London
Yazead is investigating how the brain’s immune cells are impacted in frontotemporal lobar degeneration (FTLD), which refers to the underlying brain changes that drive the development of FTD. Yazead’s research focuses on microglia which are crucial brain cells that help fight infections, and support nerve cell health.
Yazead and his team have discovered that microglia in FTLD have a reduced ability to combat viral infections. Viruses, already linked to other neurodegenerative diseases like multiple sclerosis, may contribute to FTLD by activating genes typically silent in the brain. These genes, once activated, accelerate cell aging. In this project, Yazead will further investigate this by analysing post-mortem human tissue to detect viral infections in the brains of FTLD patients. This research could pave the way for new treatment targets for FTLD.
By funding researchers like Team FTD, Aitana and Yazead, Race Against Dementia is helping accelerate breakthroughs that could change lives.