Frontotemporal dementia, also known as FTD, is one of the less common types of dementia. As part of our campaign for World Alzheimer’s Month we caught up with Professor James Rowe, based in the Cambridge Centre for Frontotemporal Dementia and Related Disorders, at the University of Cambridge, to find out more about FTD and current research into the condition.

What is frontotemporal dementia and how common is it?

Frontotemporal dementia, or FTD, affects 10 people out of every 100,000 in the UK. As the name suggests, FTD refers to progressive damage to the front of the brain (which controls behaviour, impulses, motivation, empathy and eating habits) and the temporal part of the brain (that stores words, meaning and language).

How does it differ from other types of dementia?

It is very different from the dementia that many readers will be familiar with. For example, it changes personality and behaviour rather than memory, and it causes language and communication to breakdown, rather than disorientation. It is also more common in younger people, often developing in the 50’s or 60’s. Unlike other forms of dementia, FTD often runs in a family.

Many people associate memory loss with dementia, is it correct that with FTD you can have dementia but not have concerns with your memory?

Yes. Memory is a much smaller problem in FTD than other forms of dementia. Many people with the behavioural type of FTD do not know that they are ill, and may even deny that anything is wrong, even when they are clearly different from before. People with language types of FTD are generally very aware of their illness, and can stay active and independent for many years.

What treatments are there currently for FTD?

At present, treatments are supportive, of patients and families. There is a lot that needs to be done to limit the distress and harm from FTD. This includes medical treatment of aggression, agitation, distress, anxiety, psychosis, hallucinations, bladder problems, sleep disruption, rampant appetite. It also includes getting people off unnecessary medications or drugs that can make things worse.

It is important to coordinate physio- and occupational therapists, and speech and language therapists, together with social services and community based NHS support. Helping families manage the legal and financial aspects of FTD is essential, especially as many healthcare and social services professionals will not know much about FTD and its consequences.

There’s often a lot of in the news about dementia research, particularly Alzheimer’s disease. Will all this benefit people with FTD too?

Very much so. The Dementia Research Institute, the Drug Discovery Institutes, the Dementias Platform UK and the NIHR Biomedical Research Centres are working together in a very joined up way to bring the benefits of laboratory science through to clinical utility as fast as possible. FTD gains hugely from this investment, and the attraction of bright scientists and doctors into the race for a cure for Dementia.

What are specific research priorities and most promising research opportunities for FTD?

To accelerate FTD research, towards a cure, one needs to know FTD of course, but one also needs to know what is happening in other fields, like motor neuron disease and progressive supranuclear palsy. These ‘cousins’ of FTD have a lot to teach us about the causes of FTD and potential treatments.

The priorities, for me, are:

  1. Being able to identify FTD earlier in the course of illness, and with research tools available (like PET and MRI scanning or specialist cognitive tests) that can quantify the illness.
  2. To understand why patients vary so much, a problem we call heterogeneity. Without better knowledge – and prediction – of patient variability, new drugs may fail in clinical trials even if they could help some patients.
  3. Opening up the bottleneck in drug development, especially at the point where most new treatments fail – the transition between positive results in animal models of FTD and failures in clinical trials with people with dementia. To open this bottleneck, we see great potential with methods like MEG, PET and 7T advanced brain imaging, with specialist computer models of the brain circuitry.

If FTD is less common than other dementias, such as Alzheimer’s disease, does that make it more challenging to deliver robust research?

The lack of investment and training in ‘minority’ dementias is certainly a problem, and it is essential to raise awareness of the illness throughout the public and NHS. Many patients with the behavioural form of FTD, spend years with the wrong diagnosis (depression, stress etc), or with no diagnosis, before it is recognised. This wastes a critical window of opportunity for treatment and planning.

On the other hand, the high rate of genetic causes of FTD, called Familial FTD, provides the opportunity to develop truly preventive treatments – by treating someone who carries a faulty gene, decades before the illness comes on. Such preventative treatment is not possible yet but it is in sight, for some of the genetic causes at least.

How did you get involved in FTD research?

I must be the luckiest doctor in England, to be able to work both in the NHS clinics for FTD and in the laboratory, studying the brain and ways to protect it. For me, integrating FTD research and care calls for many different roles to play – physician, psychiatrist, scientist, social worker, mathematician, philosopher and artist.

I wanted to be a doctor from the age of four, but physics and artificial intelligence were my teenage interests. It was in studying Experimental Psychology for my first degree in Cambridge that I saw how these could be brought together, which I continued in my later medical studies in Oxford and PhD at UCL. I then trained on the boundary between behavioural neurology and psychiatry, both of which are needed for good FTD care.

I have been very lucky in my career, in the opportunities given to me in psychology, neuroscience and neurology, by Trevor Robbins, Dick Passingham and Richard Frackowiak, and my introduction to FTD by Martin Rossor and John Hodges. Between them, I learned how to use neuroscience to bring together advances in animal models and clinical disorders; how to scientifically study uniquely human problems of personality, empathy, language, etc; and how important it is to be interested in what makes us individual – to see the person, not the diagnosis.

Can you tell me about some of the research you are delivering at the Cambridge Centre for FTD?

We are working on three main areas. First, to develop new ways to reduce the intensity of symptoms, like apathy and impulsivity. This is not only an immediate need, but it will grow as we find ways to help people live longer with FTD. We have a unique program of pharmacology and MEG-scanning to investigate thinking, language and behaviour.

Secondly, we want to understand why people with the same diagnosis differ so much from one another, including how fast the illness progresses. FTD is really a ‘family’ of very different illnesses, and knowing which variety of FTD someone has and what their outlook is, from the outset would help guide management, especially as we anticipate a new generation of drugs to slow the illnesses down. This theme draws on genetics, our unique PET program and ultrahigh-field MRI to study brain pathology, early in the illness.

Third, we want to be able to treat FTD even before it starts, in other words to prevent it. This means finding ways (including genetics and MRI) to predict FTD, and ways to show that a drug is working in someone who looks and feels entirely well. This is a very collaborative program, for example working with the GENFI consortium and Dementia Research Institute.

What do you see the role of patients and the public in delivering research? How do you encourage people to get involved?

Patients and families are essential partners in research, for shaping the program, setting priorities, and for delivering the successful progress that we strive for. Full engagement of patients not only makes for better research, but is also much more rewarding and motivating for our research team.

Taking part in research is not necessary for people coming to our NHS clinics. But 90% of our patients choose to take part in research of one kind or another, and I am immensely grateful for their hard work and commitment. This inclusivity is really important to me, as it means that the result of research can speak to every patient and family.