Pick`s Disease & FT Dementia

What is fronto-temporal dementia (including Pick's disease)?

This sheet provides some general information about one of the rarer forms of dementia, fronto-temporal dementia. It outlines who is likely to be affected and what symptoms they may experience.
What is fronto-temporal lobe 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 is affected?
Fronto-temporal dementia is a rare form of dementia, occurring far less frequently than Alzheimer’s disease, for example.
Younger people, specifically those under the age of 65, are more likely to be affected.
Men and women are equally likely to develop the condition.
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.
Personality and behaviour changeTypically, during the initial stages of fronto-temporal dementia, memory is still intact, but the personality and behaviour of the person changes.
People with fronto-temporal dementia may:
Lack insight and lose the ability to empathise with others – they may 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Become extrovert when they were previously introverted, or withdrawn when they were previously outgoing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 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.
Language problemsThe person with fronto-temporal dementia may experience language problems, including:
Difficulties finding the right words
A lack of spontaneous conversation
Circumlocution, or using many words with little content
A reduction in or lack of speech.
Changes in eating habitsThe person may overeat and/or develop a liking for sweet foods.
Later stagesThe 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. People affected may no longer recognise friends and family and may need nursing care.
How is this form of dementia 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.
CT (computerised axial tomography) and MRI (magnetic resonance imaging) scans may also be used to determine the extent of damage to the brain.
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 it a genetic disease?
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.
The causes of non-inherited fronto-temporal dementia are so far unknown.
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, Exelon and Reminyl, may make symptoms worse and increase aggression.
However, much can be done to ease symptoms. Knowing more about the disease and understanding why the person is behaving in certain ways can help the person to cope with the disease. Carers may be able to develop coping strategies, such as avoiding confrontation and working round obsessions, rather than trying to change the behaviour of those affected.
Speech therapists may be helpful for language problems.
Further information
Books:
Kertesz, Andrew and Munoz, David G (eds). Pick’s disease and Pick complex, Chichester: Wiley-Liss, 1998
Harvey, Richard; Fox, Nick and Rossor, Martin. Dementia handbook, Martin Dunitz, 1999
Cummings, Jeffrey L. The neuropsychiatry of Alzheimer’s disease and related dementias, Martin Dunitz, 2003