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 relatively uncommon form of dementia. As a result, many people (including health professionals) may not have heard of it. Overall, it occurs less frequently than other conditions such as Alzheimer's disease. However, it is a significant cause in younger people - specifically those under the age of 65 - and is the second or third most common cause of dementia in this age group.

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• become extrovert when they were previously introverted, or withdrawn when they were previously outgoing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 and drinking.)

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 one third to one 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 specific genes - the most important discovered so far are two genes called tau and progranulin. In a number of families the responsible gene defect has not been identified.

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

How is it diagnosed?

Fronto-temporal dementia can be misdiagnosed as Alzheimer's disease or mistaken for a mood or other psychiatric problem. A specialist may be able to make a diagnosis of fronto-temporal dementia by questioning the person affected, taking a detailed history of their symptoms and organising some tests. They may also ask for information from family, friends and carers, to gain a wider picture of the person's behaviour.

There are no blood tests that can diagnose fronto-temporal dementia but blood tests can help exclude other causes of problems.

Cognitive tests (often with a neuropsychologist) can assess which brain functions are particularly affected.
Computerised axial tomography (CT) and magnetic resonance imaging (MRI) scans may also be used to determine the extent and location of damage to the brain. Scans that look at the activity of the brain such as positron emission tomography (PET) and single photon emission computerised tomography (SPECT) may identify areas which are not functioning normally. However, a definite diagnosis may only be possible after death, when changes in the cells 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 (rivastigmine) and Reminyl (galantamine), may occasionally 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.

The Pick's Disease Support Group is another source of information and support, see 'Useful organisations' for details.

For details of Alzheimer's Society services in your area, visit alzheimers.org.uk/localinfo
For information about a wide range of dementia-related topics, visit alzheimers.org.uk/factsheets

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 1LB

T 020 7423 3500

Helpline 0300 222 11 22 info@alzheimers.org.uk  (general information)

helpline@alzheimers.org.uk (helpline)

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.

Fronto-temporal Dementia Support Group

Miss Penelope Roques
3 Fairfield Park
Lyme Regis  DT7 3DS
T 01297445488
E frontotemp@aol.com
W www.ftdsg.org

Provides information and support for people affected by fronto-temporal dementia (formerly the Pick's Disease Support Group).

Factsheet 404

Last updated: September 2010, updated: June 2012
Last reviewed: September 2010


Reviewed by: Professor Nick Fox, Professor of Neurology and MRC Senior Clinical Fellow at the Institute of Neurology, University College London in the Dementia Research Centre