Frontotemporal dementia (FTD)

This is the most common type of FTD. In the early stage, behavioural variant FTD mainly causes changes in someone’s personality and behaviour. It can also affect their mood and their ability to think things through properly.

A person with behavioural variant FTD may:

• struggle to focus and become distracted easily
• find it difficult to plan, organise and make decisions – these problems may first appear at work or with managing money 
• behave in socially inappropriate ways and act without thinking – for example, making insensitive or rude comments about someone’s appearance, making sexual gestures in public, or being verbally or physically aggressive
• lose the ability to understand what others might be thinking or feeling – they may be less considerate of the needs of others, lose interest in social activities or be less friendly
• lose motivation to do things they used to enjoy
• have less of a sense of humour, or laugh at other people’s problems – this personality change can appear cold and selfish, though it is just a symptom of disease 
• show repetitive or obsessive behaviours – for example, repeating phrases or gestures, hoarding or being obsessed with timekeeping
• take up new interests – for example, music or spirituality – these might give the person a positive focus, but can sometimes become obsessive in nature
• crave sweet, fatty foods or carbohydrates and forget their table manners
• no longer know when to stop eating, drinking alcohol or smoking, or may feel a strong urge to do these activities.

FTD can also affect how sensitive a person is to physical or environmental stimulation, such as temperature, sounds and even pain.

Symptom awareness

Most people with behavioural variant FTD are not fully aware of their symptoms. Instead, these changes are often first noticed by the people who are closest to them. 

As a result, people with this type of FTD rarely think they need to seek medical help for their condition. They may refuse to do so if others suggest it. This can cause long delays in getting an accurate diagnosis.

The person may also have problems taking medication or going to therapy sessions if they are not aware of their condition. Driving can be a difficult issue if they do not see a need to stop doing it.

This can all be very stressful for family members and carers – particularly when someone goes for several years without an accurate diagnosis. It can strain personal relationships. It may be helpful to call our Dementia Support Line on 0333 150 3456 or visit our Dementia Support Forum. 

The other main type of FTD is called primary progressive aphasia (PPA). This is when the early symptoms of dementia are dominated by problems using language. 

Problems with other aspects of thinking, mood and behaviour tend to be less noticeable at first. However, as the disease progresses, they may become more severe.

The two main types of language-led FTD are:

• semantic variant PPA
• non-fluent variant PPA.

There is also another type of PPA called logopenic aphasia. However, this is generally a type of Alzheimer’s disease, not FTD. 

Some people with PPA don’t fit neatly into any of these diagnoses. If so, they may have a diagnosis of mixed or ‘atypical’ PPA.

Semantic variant PPA

This condition causes a person to forget the meaning of words. It’s sometimes known as semantic dementia.

A person with semantic variant PPA is likely to:

• lose their vocabulary over time – at first mostly words they are less familiar with, such as technical words (for example ‘accelerator’) or less common words (for example ‘adhesive’). They may try to use more general words instead – for example, calling a spanner a tool or a ‘thingy’. As the condition develops, they will start to forget even basic words, such as ‘wet’ or ‘sugar’.
• forget what familiar objects are used for – for example, a person may forget what a toaster does and why it’s in the kitchen. This tends to happen later on.

These changes can cause a great loss of independence for the person with dementia. Along with losing the ability to communicate clearly, they may also eventually lose the skills to use everyday objects such as cutlery, toothbrushes or doors.

Although the main symptoms of semantic variant PPA involve language, the condition usually also causes changes in behaviour. It’s common to become obsessed about daily routines or have eating problems – as happens with behavioural variant FTD.

Non-fluent variant PPA

This condition causes a person to have difficulty speaking. As a result, they talk slowly with lots of gaps and errors. Even this can take a lot of effort.

Over time, a person with non-fluent variant PPA will find it more and more difficult to speak out loud. They may:

• speak differently – much more slowly, often using the wrong forms of words or putting them in the wrong order. For example, ‘I…walking…shop’ rather than ‘I’m going to walk to the shop’. For this reason, non-fluent PPA is also sometimes called agrammatic/non-fluent PPA, meaning ‘without grammar’.
• use shorter, simpler sentences – leaving out shorter words and sounding more like a text message – for example, ‘Tired. Going bed now.’
• say the opposite of what they mean – for example, saying ‘yes’ when they mean ‘no’.
• have difficulty physically speaking – forming words correctly requires a lot more effort and the sounds might not come out right. This is known as ‘apraxia of speech’.
• have difficulty swallowing or moving – some people may have symptoms similar to Parkinson’s disease, where they are only able to move slowly or stiffly. They may also lose their balance more easily.

People with non-fluent variant PPA are often still able to understand individual words. However, they increasingly struggle to understand how words are being used.

For example, a person may know what a spoon is, but they may not understand the meaning of ‘Can you pass me the spoon that’s over there on the table, please?’. This is because language relies on being able to understand the meaning behind the order of words, or the different forms they take.

These difficulties make it increasingly challenging to have a conversation, which can be frustrating and isolating.

At some point, most people with non-fluent variant PPA stop speaking completely.

As FTD progresses, the differences between the various types become less obvious. Many people with the behavioural variant develop language problems and may eventually lose all speech.

Similarly, many people with language-led dementia develop clear changes in their behaviour and personality.

Over time, the disease spreads into other parts of the brain, which leads to new symptoms. At the same time, most of the existing symptoms become more severe. The condition can start to look like the later stages of other types of dementia, such as Alzheimer’s disease.

The person may:

• become forgetful
• have delusions or hallucinations
• become increasingly withdrawn
• get restless or agitated easily, or behave aggressively
• become unlikely to attempt communication
• no longer recognise friends and family
• have trouble swallowing or moving (if not already)
• have poorer control of their bladder and bowels.

At this late stage, they are also likely to need full-time care to meet their daily needs, such as eating, drinking, washing, dressing and going to the toilet. At some point, they may need to be cared for in a residential care home. 

Finding a suitable care home when someone is younger and has an unusual type of dementia can be hard. You may need to look over a
wider area to find somewhere that’s right for them. 

People vary greatly in how long they live with FTD. This is very difficult to predict for individual people, although generally people tend to live longer if they:

• are younger when they are diagnosed
• are diagnosed quickly before their symptoms become severe
• don’t have other health conditions.

About 1 or 2 in every 10 people with FTD also develop one of a number of conditions that makes it harder for them to move.

This can happen either before or after dementia symptoms first appear. These are generally uncommon conditions, but there are three that are more likely to affect people with FTD:

• motor neurone disease
• corticobasal syndrome (CBS)
• progressive supranuclear palsy (PSP).

Motor neurone disease

Motor neurone disease (MND) is a condition that causes muscles to weaken and waste away. It is also sometimes known as amyotrophic lateral sclerosis (ALS). Eventually, a person with the condition becomes almost completely paralysed and struggles to breathe or swallow.

Some people with FTD start to have symptoms of MND as their condition progresses. This is more likely to happen to people with behavioural variant FTD, particularly if their condition is caused by a single gene. It’s less likely for people who have a language-led dementia and very rare for those with semantic variant PPA.

FTD and MND are both progressive conditions that get worse over time. Having the two conditions can reduce a person’s life expectancy more than having FTD on its own.

The Motor Neurone Disease Association provides support for people affected by this condition.  

Corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP)

CBS and PSP involve a combination of dementia symptoms and difficulties with movement. For many people, the first signs of these conditions are problems with thinking, language, mood or behaviour. This is similar to the early stages of FTD. 

For others, the problems with movement may come first. 

As well as dementia, CBS also causes:

• muscle twitching or jerking
• stiffness
• slow movements
• slurred speech
• loss of balance or coordination.

Sometimes an arm or leg can move by itself without the person controlling it. 

CBS can start off as a language-led dementia, such as non-fluent variant PPA. It can then progress over time to involve more severe problems with movement.

PSP is similar to CBS, but also causes problems with control of eye movements, difficulty walking, and being more prone to falling.

Ideally, these conditions need specialised support from people and organisations who understand both the dementia and the movement disorders. In the UK, this is the PSP Association. For more, see our information on rarer types of dementia.