Frontotemporal dementia (FTD) is one of the less common types of dementia.

It covers a range of different conditions that can affect personality, behaviour and language. FTD is mostly diagnosed in people under 65. It is sometimes called Pick’s disease or frontal lobe dementia.

What is frontotemporal dementia (FTD)?

Dementia describes a group of symptoms that can include problems with memory, thinking or language, and changes in mood, emotions and behaviour. It is caused when the brain is damaged by disease.

The word ‘frontotemporal’ refers to the two sets of lobes (frontal and temporal) in the brain that are damaged in this type of dementia. FTD occurs when disease damages nerve cells in these lobes. This causes the connections between them and other parts of the brain to break down. The levels of chemical messengers in the brain also get lower over time. These messengers allow nerve cells to send signals to each other and the rest of the body. As more and more nerve cells are damaged and die, the brain tissue in the frontal and temporal lobes starts to get smaller.

 

There are two broad types of FTD:

Behavioural variant FTD – where damage to the frontal lobes of the brain mainly causes problems with behaviour and personality. These lobes are found behind the forehead and process information that affects how we behave and the control of our emotions. They also help us to plan, solve problems and focus for long enough to finish a task.

 

Primary progressive aphasia (PPA) occurs when damage to the temporal lobes – on either side of the head nearest the ears – causes language problems. This part of the brain has many roles. A key function of the
left temporal lobe is to store the meanings of words and the names of objects. The right temporal lobe helps most people recognise familiar faces and objects. 

The first noticeable symptoms for a person with FTD will be changes to their personality and behaviour and/or difficulties with language. 
These are very different from the early symptoms of more common types of dementia. For example, in Alzheimer’s disease, early changes are often problems with day-to-day memory. In the early stages of FTD, many people can still remember recent events.

 

Symptoms

Frontotemporal dementia affects everyone differently. Its symptoms vary a lot and depend on which areas of the frontal and temporal lobes are damaged – and so the type of FTD the person has.

As with most forms of dementia, FTD is progressive. This means its symptoms may be mild at first, but they will get worse over time. 

Behavioural variant FTD

This is the most common type of FTD. In the early stages it mainly causes changes in someone’s personality and behaviour.

A person with behavioural variant FTD may:

 

lose motivation to do things that they used to enjoy

struggle to focus on tasks and become distracted easily

find it difficult to plan, organise and make decisions – these problems may first appear at work or with managing money

lose their inhibitions – behaving in socially inappropriate ways and acting impulsively or without thinking. For example, making insensitive or rude comments about someone’s appearance, making sexual gestures in public, staring at strangers, 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. They may also have less of a sense of humour or laugh at other people’s problems. This can make the person appear cold and selfish

show repetitive or obsessive behaviours – for example, repeating phrases or gestures, hoarding or being obsessed with timekeeping. They may also take up new interests – for example, music or spirituality. These might give the person a positive focus but can become obsessive in nature

crave sweet, fatty foods or carbohydrates and forget their table manners. They may also no longer know when to stop eating, drinking alcohol or smoking.

 

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

 

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 close to them. As a result, people with this type of FTD rarely think they need to seek medical help for their condition. They may also 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. Safe driving will also be difficult. 

This can all be very stressful for the family and carers of a person with behavioural variant FTD. It can also affect the person’s relationships. It may be helpful to call Alzheimer’s Society support line on 0333 150 3456 or visit the online community, Talking Point, at alzheimers.org.uk/talkingpoint

Primary progressive aphasia (language variants of FTD)

The other main type of FTD is primary progressive aphasia (PPA) where the early symptoms are mostly problems with language. Other aspects of thinking, perception and behaviour are not affected as much during the early stages. However, as the disease progresses, there may start to be changes in these areas.

The two main subtypes of PPA are:

semantic variant PPA

non-fluent variant PPA.

Semantic variant PPA

This condition causes a person to forget the meaning of words. About 1 in 5 people with FTD will have this type. A person with semantic variant PPA is likely to:

nlose 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 ‘crinoline’). 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.

 

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. See the ‘Treatment and support’ section for ideas about how to manage these symptoms.

Although the main symptoms of semantic variant PPA involve language, the condition usually also causes changes in behaviour. Getting obsessed about daily routines or having eating problems are common – like in behavioural variant FTD.

Non-fluent variant PPA

This condition causes a person to have problems with speaking. About 1 in 4 people with FTD will have this condition. Over time, a person with non- fluent variant PPA will find it more and more difficult to get their words out. They may also:

start to speak differently – this includes speaking more slowly, using the wrong grammar and putting words in the wrong order. For example, ‘I gone to the shop.’

They may use shorter, simpler sentences that miss out shorter words (known as telegraphic speech)

– for example, ‘Tired. Going bed now.’

They may say the opposite of what they mean – for example, saying ‘yes’ when they mean ‘no’.

Unlike those with semantic variant PPA, many people with non-fluent variant PPA still understand individual words. However, over time they will struggle to understand full sentences that use these words. For example, a person with this condition may know what a ‘spoon’ is, but they may not know the meaning of ‘Can you pass me the spoon, please?’ This makes it difficult for the person to have a conversation, which can be both frustrating and isolating. At some point the person may stop speaking completely.

Later stages of FTD

As FTD progresses, differences between the three types described above become much less obvious. Many people with the behavioural variant develop language problems and may eventually lose all speech. Similarly, many people with a language variant of FTD (especially semantic variant dementia) develop clear changes in their behaviour and personality.

The later stages of all types of FTD bring a greater range of symptoms as the disease spreads to other areas of the brain. Any symptoms the person already has will also get worse. As a result, symptoms become similar to the later stages of other types of dementia, such as Alzheimer’s disease.

The person may:

become forgetful
have delusions or hallucinations
get restless or agitated easily, or behave aggressively nno longer recognise friends and family.

At this late stage they are also likely to need full-time care to meet their daily needs.

How quickly FTD progresses, and the life expectancy of someone with the condition varies from person to person.

Many factors can affect the speed and pattern of progression. This includes a person’s age when they were diagnosed and whether they have other health conditions. 

Related conditions that affect movement

About 1 or 2 in every 10 people with frontotemporal dementia 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. These are:

motor neurone disease nprogressive supranuclear palsy ncorticobasal syndrome.

Motor neurone disease (MND) is a progressive condition that causes muscles to weaken and waste away. Eventually a person with MND becomes almost completely paralysed and struggles to breathe or swallow. FTD and MND are both progressive and life-limiting conditions. Having the two conditions can reduce a person’s life expectancy more than FTD can on its own.

Corticobasal syndrome (CBS) and progressive supranuclear palsy
(PSP) are conditions with a combination of dementia symptoms and movement difficulties. These can include muscle twitching, stiffness, slow movements, slurred speech and loss of balance or co-ordination. PSP
also causes problems with the control of eye movements. Both conditions can also cause FTD-like symptoms and, in the later stages, difficulties with swallowing. 

Who gets FTD?

While researchers know a lot about how FTD develops in the brain, they still don’t fully understand why some people get FTD and others don’t. This is mainly because FTD is a less common type of dementia – so it is harder to study its causes. However, there are some things that are known to affect a person’s chances of getting FTD.

 

Age

Frontotemporal dementia is mostly diagnosed between the ages of 45 and 65 (though it can affect people younger or older than this). This is much younger than more common types of dementia such as Alzheimer’s disease, which mostly affects people over 65.

Being diagnosed at a younger age can bring someone a very different set of challenges. A person under 65 may still be working, have a mortgage and other bills to pay, or have children who still rely on them. This means they may need different services and support. For more information about these issues see factsheet 440, What is young-onset dementia?

 

Please see this factsheet for ore information:

 

https://www.alzheimers.org.uk/sites/default/files/2019-09/factsheet_what_is_frontotemporal_dementia.pdf