What is frontotemporal dementia?
Frontotemporal dementia is a rare type of dementia that tends to run in families.
The term 'dementia' describes a loss of mental ability that's more than would be expected with ageing. It's sufficient to affect day-to-day activities and gets progressively worse.
Frontotemporal dementia is similar to Alzheimer's disease in that it's caused by a build-up of abnormal proteins in the brain, leading to a progressive loss of brain cells. The affected areas of the brain gradually function less well and shrink.
It tends to affect the frontal and temporal lobes of the brain (the front and sides) in particular, and often starts at a younger age than usually seen in Alzheimer's disease.
The frontal lobes are at the front of the brain, behind the forehead – they regulate our ability to plan and organise ourselves and are important in regulating our behaviour. The temporal lobes are on either side of the brain above and behind the ears, and they organise memory and language.
Several different conditions can affect these lobes and 'frontotemporal dementia' is the broad term to describe them all (read about the types of frontotemporal dementia). Symptoms vary depending on the exact areas of the brain affected.
What are the warning signs?
Generally, frontotemporal dementia causes slowly progressive problems with language, thinking, emotions and behaviour. It can profoundly alter a person's character and social conduct – for example, they may either become impulsive and much more outgoing, or uninterested and lacking in energy, depending on the type of disease they have.
Other typical symptoms include inappropriate behaviour in social situations and difficulty with speech.
Frontotemporal dementia typically starts between the ages of 45 and 65, although it can sometimes affect younger people as well as the elderly.
Learn more about the symptoms of frontotemporal dementia.
How does the disease progress?
The disease worsens over a period of time – typically 3 to 10 years – although it varies widely between patients (some cases can be more rapid; in others it can be much slower).
Over time, new symptoms may develop that can be quite different to those already present. Past problems might disappear and new ones emerge.
People with frontotemporal dementia can become isolated and socially withdrawn as the illness progresses. They may not want to spend time in the company of others, or when in company, may lack empathy and concern for others. They may behave in rude or insulting ways.
Some lack the motivation to care for themselves well and may need supervision and prompting. Home-based help is often needed, and some people will eventually need residential care in a nursing home.
What are the causes?
Frontotemporal dementia is caused by a build-up of abnormal proteins in the brain.
The abnormal proteins clump together and become toxic to the brain cells, causing them to die. The frontal and temporal lobes of the brain are typically most affected and as cells are lost, the brain areas shrink over time.
It's not fully understood why these abnormal proteins build up, but there's often a genetic cause. About a third to a half of cases of frontotemporal dementia are inherited and run in families.
Learn more about the causes of frontotemporal dementia.
How is frontotemporal dementia diagnosed?
If your doctor thinks you may have dementia, you may be referred to a memory clinic or another specialist clinic, where you'll be asked about your symptoms and have a physical check-up, memory test and cognitive test. You may also have blood tests and brain scans.
Learn more about the diagnosis of frontotemporal dementia.
How is it managed?
There's currently no cure for frontotemporal dementia, nor any drugs that will slow the progress of the disease.
Behaviour modification may help control challenging or dangerous behaviours, and antidepressants can be effective in improving some of the symptoms. Learn more about the management of frontotemporal dementia.
Rehabilitative support such as physiotherapy, occupational therapy and speech therapy aim to help with adapting to lost functions, and dementia activities such as 'memory cafes' and some psychological therapies can help with managing the symptoms.
If you have dementia, your future health and social care needs will need to be assessed and a care plan will be drawn up.
For more information, read about the management of frontotemporal dementia.
Signs of frontotemporal dementia
People with frontotemporal dementia tend to have problems with emotions, communication and behaviour. They will not usually be aware of these changes.
The early symptoms depend on which area of the brain is affected.
Generally, symptoms gradually worsen over a period of 3 to 10 years.
Behavioural and mood changes
Behavioural and emotional changes vary widely from person to person – for example, some may be uninterested and seem to have no drive, while others may be impulsive and more outgoing, with no inhibitions.
Typical signs are:
- inappropriate behaviour in public
- loss of inhibitions
- overeating, a change in food preferences (such as suddenly liking sweet foods), poor table manners
- neglect of personal hygiene
- repetitive or obsessional behaviours, such as humming, hand-rubbing and foot-tapping, or complex routines such as walking exactly the same route repetitively
- seeming more selfish
- inability to empathise with others, seeming cold and uncaring
- being tactless or rude
- being less or more outgoing than in the past
- being lethargic, lacking enthusiasm
As the disease progresses, people with frontotemporal dementia may become socially isolated and withdrawn.
Problems with language
People with frontotemporal dementia tend to gradually lose the ability to speak, eventually becoming mute.
- reduced speech and conversation
- lack of spontaneous speech
- difficulty finding words
- using lots of words but little content
- less fluent speech or problems with articulation
Problems with thinking
Many people with frontotemporal dementia lose the ability to think for themselves. Signs are:
- needing to be told what to do
- poor planning and problem solving
- becoming easily distractible
- thinking in a rigid and inflexible way
- losing the ability to understand abstract ideas
- memory difficulties (although not common in the early stages)
- difficulty in communicating
In the later stages, many people with frontotemporal dementia may develop problems with movement. They may no longer initiate movements and may become rigid.
Frontotemporal dementia causes
Frontotemporal dementia is caused by a build-up of abnormal proteins affecting the frontal and temporal areas of the brain (behind the forehead and above and behind the ears).
The abnormal proteins clump together and become toxic to the brain cells, causing them to die. The frontal and temporal lobes are most affected and shrink over time.
It's not yet fully understood why these abnormal proteins build up, but there's often a strong genetic cause. About a third to a half of cases of frontotemporal dementia are inherited and run in families.
Mutations (alterations) in 3 genes account for the majority of these inherited cases:
- the tau gene
- the progranulin gene
- a recently discovered gene called C9 or f72 that can cause motor neurone disease or frontotemporal dementia
Tau protein is involved in some cases of frontotemporal dementia. This protein is present in all nerve cells and has an important role in transporting other essential proteins between nerve cells in the brain.
A mutation in the gene that codes for tau protein causes abnormal tau to build up. This particular mutation is found on chromosome 17.
Without a usable form of tau protein, and with an abnormal form of the protein building up, the brain cells cannot function properly and so they die.
Other types of frontotemporal dementia will be associated with different genes, proteins and chromosomes. In other words, there's no single common cause of frontotemporal dementia.
Frontotemporal dementia diagnosis
Confirming a diagnosis of dementia can be difficult, particularly when the condition is in its early stages. This is because many of the symptoms of dementia can be caused by other conditions.
When you visit your doctor, it can be helpful if you and a close relative or friend are seen together, because they can help you remember what's happening.
For frontotemporal dementia to be diagnosed correctly, you'll have a number of tests and assessments, including:
- an assessment of your symptoms – for example, how long you've had problems with thinking or language
- a full assessment of your mental abilities
- a physical examination
- a review of the medication you're taking in relation to your symptoms
- a range of tests, including blood tests, to rule out other possible causes of your symptoms, such as a vitamin B deficiency
- brain scans such as a magnetic resonance imaging (MRI) scan can confirm a diagnosis of frontotemporal dementia by showing loss of brain cells in the frontal and temporal regions of the brain (behind the forehead and near the ears)
Some of these tests can be carried out by your doctor. Some will be carried out by specialists, such as a neurologist (an expert in treating conditions that affect the brain and nervous system), an elderly care physician, or a psychiatrist with experience in treating dementia.
Assessing your mental abilities
Questionnaires are sometimes used to help test your mental abilities and how severe your symptoms are. One widely used questionnaire is the Mini Mental State Examination (MMSE).
The MMSE can be used to assess a number of different mental abilities, including:
- short and long-term memory
- attention span
- language and communication skills
- ability to plan
- ability to understand instructions
The MMSE is a series of questions, each carrying a score with a maximum of 30 points. Example questions include:
- memorising a short list of objects and then repeating the list back
- writing a short sentence that's grammatically correct, such as ‘the dog sat on the floor’
- correctly identifying the current day of the week, followed by the date, the month, the season and the year
The MMSE is not a test to diagnose dementia. However, it's useful for assessing the level of mental impairment that a person with dementia may have.
Scores on these and other tests only help to assess the problem and must be taken into context. For example, someone who cannot read or write very well may score less than someone else, but they may not have dementia. Similarly, someone with a higher level of education may achieve a higher score but still have dementia.
More detailed psychological tests (including tests of frontal lobe function, language, memory and thinking) may also be used by a specialist psychologist.
Frontotemporal dementia treatment
Once you've been diagnosed with frontotemporal dementia, your future health and social care needs will need to be assessed and a care plan drawn up.
A care plan is a way of ensuring you receive the right treatment for your needs.
There's currently no cure for frontotemporal dementia, but certain medication and psychological therapy can help in managing the symptoms. These are detailed below.
Unfortunately, acetylcholinesterase inhibitors such as donepezil, which are normally prescribed for Alzheimer's disease and vascular dementia, are not effective in frontotemporal dementia.
Rehabilitative support such as physiotherapy, occupational therapy and speech therapy will help you to regain your lost functions as best as possible.
There's some evidence that selective serotonin reuptake inhibitors (SSRIs), a type of antidepressant, may help control the loss of inhibitions, overeating and compulsive behaviour seen in some people with frontotemporal dementia.
Learn more about SSRIs, including how they work and the side effects.
Antipsychotics, such as haloperidol, are only really used for treating severely challenging behaviour that's putting you or others at risk of harm.
This is because the drugs can cause a range of serious side effects, including rigidity and immobility.
If antipsychotics are needed, they'll be prescribed at the lowest possible dose and for the shortest possible time, and your health will be carefully monitored.
The medication may cause drowsiness and/or shaking.
Psychological treatments do not slow down the progression of dementia, but they can help you to cope with the symptoms.
Some psychological treatments are outlined below.
Cognitive stimulation involves taking part in activities and exercises designed to improve your memory, problem-solving skills and language ability.
Reality orientation therapy is a type of therapy that reduces feelings of mental disorientation, memory loss and confusion, while improving feelings of self-esteem.
The therapy may involve group work in a classroom, in which a board prominently displays information such as:
- the current day and date
- the location of the classroom
- the names of the people in the group
The group members repeat a series of tasks designed to give mental stimulation. The tasks also reinforce information regarding the time, the place and the people involved in the group. You'll be involved in discussions about a variety of topics, as well as taking part in word and memory games.
Reality orientation may be beneficial in some cases, but the benefits can be small and are often only apparent with continued effort. The technique is also often considered unsuitable for use in care home settings.
Behavioural therapy uses a problem-solving approach where possible motivations and reasons for troublesome behaviour are identified. Different strategies are adopted to try to change that behaviour.
For example, a person with dementia may have a history of wandering out of their home or care centre because they feel restless. Therefore, encouraging them to take part in regular physical exercise may help to decrease their restlessness.
Behavioural therapy can be used to treat many of the behavioural problems associated with dementia, such as depression, aggression, and delusional thinking. It's usually given by a carer, such as a trained friend or relative, or by an employed carer and always supervised by a healthcare professional.