All about Frontotemporal Dementia

In the last few weeks, frontotemporal dementia has hit the headlines when it was announced that Hollywood actor Bruce Willis had been diagnosed with the condition. In a statement issued by the family, they stated that, “As Bruce’s condition advances, we hope that any media attention can be focused on shining a light on this disease that needs far more awareness and research.”

According to Dementia UK, frontotemporal dementia is a rare form of dementia affecting around one in 20 people with a dementia diagnosis. As there are approximately 944,000 people with dementia in the UK, frontotemporal dementia possibly affects just under 50,000 people in the UK.

What is frontotemporal dementia?

Frontotemporal dementia refers to a diverse group of conditions that collectively are a major cause of young-onset dementia. It is an umbrella term for a group of dementias that mainly affect the frontal and temporal lobes of the brain, which are responsible for personality, behaviour, language and speech.

Unlike some other types of dementia, frontotemporal dementia, which is also known as Pick’s disease, seems to affect men and women about equally.

Frontotemporal dementia is mostly diagnosed between the ages of 45 and 65, although 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 years. Because of this, frontotemporal dementia has a substantially greater impact on a person’s work, family and finances than other forms of dementia such as Alzheimer’s.

What causes frontotemporal dementia?

There is not enough evidence to know if certain lifestyle factors, such as smoking, drinking alcohol, or not being physically active can increase a person’s risk of getting frontotemporal dementia or whether other long-term health conditions, such as diabetes or high blood pressure, can make someone more likely to develop the condition.

There are types of genes, sometimes known as susceptibility genes, that can increase a person’s chances of getting frontotemporal dementia, although this doesn’t always mean that someone with this gene will go on to develop frontotemporal dementia.

However, frontotemporal dementia is much more likely to run in families than more common forms of dementia. There is a single faulty gene, called a familial gene, that runs in some families that will definitely cause frontotemporal dementia if it is passed down from a parent to a child. About 10 to 15 in every 100 people with frontotemporal dementia have this type and any child of a person with familial frontotemporal dementia has a 1 in 2 chance of having the same gene.

A person with a close family member with frontotemporal dementia may want to find out whether or not the condition has been caused by the familial gene, especially if they plan to have children. If you are concerned that you may have the familial gene, you can ask your GP to refer you to a local NHS genetics service for testing.

This service provides pre-test genetic counselling, which can help a person to consider all the possible effects of finding out if they have a familial frontotemporal dementia gene because test results can have a serious impact on a person’s mental wellbeing and that of their family.

A small but significant minority of people with Motor Neurone Disease (MND) experience severe changes that will be diagnosed as frontotemporal dementia (FTD). These changes are most often in behaviour and executive skills, but sometimes they are in language or conceptual understanding.

According to the MND Association, up to 15% of people develop frontotemporal dementia (FTD), either at the same time or after diagnosis of MND, and up to 15% of people diagnosed with FTD go on to develop MND. Symptoms of dementia may lead to FTD being diagnosed before movement is affected and MND is diagnosed.

What are the types of frontotemporal dementia?

There are two main types of frontotemporal dementia:

• Behavioural variant frontotemporal dementia (BvFTD) – This is the most common form of frontotemporal dementia, and is responsible for about half of all cases of this disease and primarily affects behaviours.
• Primary progressive aphasia (PPA) – This type has three variants based on the specific language skills that are most affected:
  – Non-fluent/Agrammatic variant – People with this variant find it increasingly difficult to speak yet can still recall the meanings of individual words.
  – Semantic variant – This is the progressive loss of the meanings of words.
  – Logopenic variant – People with this variant have difficulty finding words when they are speaking.

and three other subtypes:

• ALS and Frontotemporal Degeneration – Amyotrophic lateral sclerosis (ALS) is the commonest form of motor neuron disease (MND) and both conditions can coexist.
• Corticobasal syndrome – This belongs to the category of frontotemporal dementia disorders that primarily affect movement.
• Progressive Supranuclear Palsy – This belongs to the category of frontotemporal dementia disorders that primarily affect movement.

Symptoms of frontotemporal dementia

The signs and symptoms of frontotemporal dementia can be different from one individual to the next. Clusters of symptom types tend to occur together, and people may have more than one cluster of symptom types.

The most common signs of frontotemporal dementia involve extreme changes in behaviour and personality.

These include:

• Increasingly inappropriate social behaviour.
• Loss of empathy and other interpersonal skills, such as having sensitivity to another’s feelings.
• Lack of judgement.
• Loss of inhibition.
• Lack of interest (apathy), which can be mistaken for depression.
• Repetitive compulsive behaviour, such as tapping, clapping or smacking lips.
• A decline in personal hygiene.
• Changes in eating habits, usually overeating or developing a preference for sweets and carbohydrates.
• Eating inedible objects.
• Compulsively wanting to put things in the mouth.

Some subtypes of frontotemporal dementia lead to language problems or impairment or loss of speech. Primary progressive aphasia, semantic dementia and progressive agrammatic (non-fluent) aphasia are all considered to be frontotemporal dementia.

 Problems caused by these conditions include:

• Increasing difficulty in using and understanding written and spoken language, such as having trouble finding the right word to use in speech or naming objects.
• Trouble naming things, possibly replacing a specific word with a more general word such as “it” for “car”.
• No longer knowing word meanings.
• Having hesitant speech that may sound compressed.
• Making mistakes in sentence construction.

Rarer subtypes of frontotemporal dementia are characterised by problems with movement similar to those associated with Parkinson’s disease or amyotrophic lateral sclerosis (ALS).

Motor-related problems may include:

• Tremor.
• Rigidity.
• Muscle spasms or twitches.
• Poor coordination.
• Difficulty swallowing.
• Muscle weakness.
• Inappropriate laughing or crying.
• Falls or walking problems.

The signs and symptoms of frontotemporal dementia get progressively worse over time, usually over years. The rate at which frontotemporal dementia progresses varies greatly, with life expectancy ranging from less than two years to 10 years or more. Research shows that, on average, people live for about six to eight years after the start of symptoms but this varies widely.

As the condition progresses people with the behavioural variant tend to develop language problems as their condition progresses. They may eventually lose all speech, like a person with one of the language variants. Similarly, over several years a person with a language variant, especially semantic dementia, will tend to develop the behavioural problems typical of the behavioural variant.

In the later stages of all types of frontotemporal dementia, more of the brain becomes damaged. As a result, the symptoms are often similar to those of the later stages of Alzheimer’s disease.

The person may become less interested in people and things and will have limited communication. They may become restless or agitated, or behave aggressively. At this late stage, they may no longer recognise friends and family, and are likely to need full-time care to meet their needs.

What parts of the brain are affected?

Frontotemporal dementia causes a build-up of proteins in the frontal and temporal lobes, the parts of the brain that control language and behaviour. Frontotemporal dementia occurs when nerve cells in the frontal and/or temporal lobes of the brain die, and the pathways that connect the lobes change. Some of the chemical messengers that transmit signals between nerve cells are also lost. Over time, as more and more nerve cells die, the brain tissue in the frontal and temporal lobes shrinks.

The frontal lobes are the largest of the four lobes responsible for many different functions. These include motor skills such as voluntary movement, speech, and intellectual and behavioural functions. The areas that produce movement in parts of the body are found in the primary motor cortex or precentral gyrus.

The prefrontal cortex plays an important part in memory, intelligence, concentration, temper and personality. The premotor cortex is a region found beside the primary motor cortex. It guides eye and head movements and a person’s sense of orientation. Broca’s area, important in language production, is found in the frontal lobe, usually on the left side.

The temporal lobes are located on each side of the brain at about ear level, and can be divided into two parts. One part is on the bottom (ventral) of each hemisphere, and the other part is on the side (lateral) of each hemisphere. An area on the right side is involved in visual memory and helps humans recognise objects and people’s faces. An area on the left side is involved in verbal memory and helps humans remember and understand language. The rear of the temporal lobe enables humans to interpret other people’s emotions and reactions.

How is frontotemporal dementia diagnosed?

Frontotemporal dementia is much less common than other types of dementia and often has different early symptoms. This means that frontotemporal dementia can be hard for doctors to diagnose as they may not recognise its symptoms as dementia.

If you are concerned that you or someone you know may have frontotemporal dementia, it is important to see your GP, or encourage them to see their GP. It is normally helpful to have somebody who knows the person well to give an account of their symptoms to the GP, especially as someone with frontotemporal dementia may not be aware of changes in their behaviour.

There is no single test for frontotemporal dementia, and the GP should:

• Take a full history including:
  – The person’s symptoms.
  – When they started.
  – How they affect the person’s life.
  – Any family history of dementia.
• Perform or arrange blood and urine tests and physical examinations to rule out other physical or mental health issues.
• Conduct a brief assessment of memory and cognitive skills – this may be unreliable for people with frontotemporal dementia as memory problems are less common than in some other types of dementia.
• Arrange for brain scans, such as an MRI scan, a CT scan or a PET scan. These can detect signs of dementia and help identify which parts of the brain are most affected, or help rule out other problems with the brain.
• Arrange for a lumbar puncture to test the spinal fluid – the fluid that surrounds and supports the brain and spine. A lumbar puncture can be useful to rule out Alzheimer’s disease as the cause of symptoms.

The GP may refer the person to a specialist who may recommend that because of frontotemporal dementia symptoms they have a genetic test. This can show if the person’s condition is caused by a specific faulty gene. Knowing this can help the specialist to make a more precise diagnosis and to better understand the changes that are happening in the person’s brain.

Treatments for frontotemporal dementia

There is no cure for frontotemporal dementia, and no treatments available to slow or stop the progression of the disease. There are, however, treatments and interventions that can help to manage the symptoms, and to preserve and maximise quality of life for people with frontotemporal dementia.

Drugs that are commonly used to treat other types of dementia are not recommended for people with frontotemporal dementia. These drugs, known as cholinesterase inhibitors, for example donepezil, rivastigmine and galantamine, can actually make the symptoms of frontotemporal dementia worse. Some types of antidepressants, such as trazodone, may reduce the behavioural problems associated with frontotemporal dementia.

People experiencing language difficulties may benefit from speech therapy to learn alternate strategies for communication, and regular cardiovascular exercise may help improve mood and thinking skills.

Some practical strategies and interventions can help the person live as well as possible with the diagnosis, such as:

• Encourage the person to carry a form of identity with details of their diagnosis. This can help in public situations where there may be misunderstandings or a need for extra support. The hidden disabilities sunflower scheme provides a range of cards and lanyards.
• Follow a daily routine to help the person feel more relaxed.
• Try to avoid triggers such as noisy or crowded places, changes in routine, misunderstanding, boredom and physical inactivity.
• Encourage the person to continue activities and hobbies they enjoy.
• If the person overeats or craves sweet foods, offer food at set times, try to control portion size, and provide healthier options. 
• Remember that the person is not intentionally causing upset or offence. Rather than challenging or correcting them, try to stay patient, calm and empathetic to reduce distress.

People with frontotemporal dementia can become socially isolated as the illness progresses. They may not want to spend time in the company of others, or may behave in rude or insulting ways which may limit their social contact.

At some stage the person may need care in their own home or may need residential care in, for example, a nursing home. Dementia has a huge impact on someone’s life, and they may need to think about legal and financial matters and seek advice on the best approach for them. They can arrange for a loved one to make financial, legal and health decisions on their behalf. This is called Lasting Power of Attorney (LPA).

Final thoughts

Caring for someone with frontotemporal dementia can be challenging and stressful because of the extreme personality changes and behavioural problems that often develop.

To speak to a specialist dementia nurse known as an Admiral Nurse about frontotemporal dementia or any other aspect of dementia, call the free helpline on 0800 888 6678

There are other sources of advice and support including:

• Dementia UK 0800 888 6678.
• Rare Dementia Support contact@raredementiasupport.org
• Young Dementia Network.
• Dementia Voices 07720 538851
• Motor Neurone Disease (MND) Association 0808 802 6262.
• Dementia Carers Count 0800 652 1102.
• Together In Dementia Everyday (tide).