What is fronto-temporal dementia (including Pick's disease)? from the Alzheimer's Society, U.K.
What is fronto-temporal dementia (including Pick's disease)?

This factsheet provides some general information about one of the rarer forms of dementia: fronto-temporal dementia. It explains who is likely to be affected, what symptoms they may experience, how it is diagnosed, and what can be done to help.

What is fronto-temporal dementia?

The term 'fronto-temporal dementia' covers a range of conditions, including Pick's disease, frontal lobe degeneration, and dementia associated with motor neurone disease. All are caused by damage to the frontal lobe and/or the temporal parts of the brain. These areas are responsible for our behaviour, emotional responses and language skills.

Who does it affect?

Fronto-temporal dementia is a rare form of dementia. It occurs far less frequently than other conditions such as Alzheimer's disease. It is more likely to affect younger people - specifically those under the age of 65 - and it is slightly more common in men.

What are the symptoms?

Damage to the frontal and temporal lobe areas of the brain causes a variety of different symptoms. Each person will experience the condition in his or her own individual way, but there are some symptoms commonly experienced by people with the condition.

Personality and behaviour change

Typically, during the initial stages of fronto-temporal dementia, the person's memory is still intact but their personality and behaviour changes. People with fronto-temporal dementia may:

* lack insight, and lose the ability to empathise with others. This can make them appear selfish and unfeeling
* become extrovert when they were previously introverted, or withdrawn when they were previously outgoing
* behave inappropriately - for example, making tactless comments, joking at the 'wrong' moments, or being rude.
* lose their inhibitions - for example, exhibiting sexual behaviour in public
* become aggressive
* be easily distracted
* develop routines - for example, compulsive rituals.

It is important to recognise that these symptoms have a physical cause, and cannot usually be controlled or contained by the person. (See Factsheet 525, Unusual behaviour.)

Language difficulties

The person with fronto-temporal dementia may experience language difficulties, including:

* problems finding the right words
* a lack of spontaneous conversation
* circumlocution, using many words to describe something simple
* a reduction in or lack of speech.

Changes in eating habits

The person may overeat and/or develop a liking for sweet foods. (See Factsheet 511, Eating.)

Later stages

The rate of progression of fronto-temporal dementia varies enormously, ranging from less than two years to over ten years.

In the later stages, the damage to the brain is usually more generalised, and symptoms usually appear to be similar to those of Alzheimer's disease. People affected may no longer recognise friends and family, and may need nursing care.

What causes it?

There is a family history in about half of all cases of fronto-temporal dementia. In these families, the course of the disease usually has a specific pattern across the generations. Some of these inherited forms have been linked to abnormalities on chromosomes 3 and 17.

So far, the causes of non-inherited fronto-temporal dementia are unknown.

How is it diagnosed?

Fronto-temporal dementia is commonly misdiagnosed as Alzheimer's disease. A specialist may be able to make a diagnosis of fronto-temporal dementia by questioning the person affected and taking a detailed history of their symptoms. They may also ask for information from family, friends and carers, to gain a wider picture of the person's behaviour.

Computerised axial tomography (CT) and magnetic resonance imaging (MRI) scans may also be used to determine the extent of damage to the brain. However, a firm diagnosis may only be possible after death, when changes in the structure of the brain can be directly observed at post mortem.

Is treatment possible?

As yet, there is no cure for fronto-temporal dementia, and the progression of the condition cannot be slowed. Drugs that are designed for the treatment of Alzheimer's disease, such as Aricept (trade name for donepezil hydrochloride), Exelon (memantine) and Reminyl (galantamine), may actually make symptoms worse and increase aggression.

However, much can be done to ease symptoms. Knowing more about the disease, and understanding that it is the cause of behaviour changes, can help the person - and those around them - to cope with the disease.

People who are close to someone with fronto-temporal dementia need to try to develop coping strategies, such as avoiding confrontation and working round obsessions, rather than trying to change the person's behaviour.

Speech therapists may be helpful for language problems.

Your local Alzheimer's Society branch will always be willing to talk to you and offer advice and information to support your needs.

For more information, Dementia Catalogue, our specialist dementia information resource, is available on the website at alzheimers.org.uk/dementiacatalogue

Further reading

Cummings JL (2003) The neuropsychiatry of Alzheimer's disease and related dementias, London: Martin Dunitz.

Harvey R, Fox N and Rossor M (1999) Dementia handbook, London: Martin Dunitz.

Kertesz A and Munoz DG eds (1998) Pick's disease and Pick complex, Chichester: Wiley-Liss.

Useful organisations

Alzheimer's Society

Devon House
58 St Katharine's Way
London E1W 1JX
T 020 7423 3500
0845 300 0336 (helpline open 8.30am-6.30pm weekdays)
E info@alzheimers.org.uk (general information)
helpline@alzheimers.org.uk (helpline)
W alzheimers.org.uk

The UK's leading care and research charity for people with dementia and those who care for them. The helpline provides information, support, guidance and referrals to other appropriate organisations.

Pick's Disease Support Group

8 Brooksby Close
Oadby
Leicester LE2 5AB
T 0116 271 1414
E info@pdsg.org.uk
W http://www.pdsg.org.uk/
Factsheet 404

Last updated: September 2008
Last reviewed: September 2008

Reviewed by: Professor Robert Baldwin, Consultant Psychiatrist and Professor, University of Manchester