Booklet - 2003

Pick's Disease (Frontotemporal lobar degeneration) by Dr Alison Godbolt, Dementia Research Group

What is it?
A progressive dementia occurring in middle life characterised by slowly developing changes in character and social behaviour, or impairment of language, due to degeneration of the frontal and temporal lobes of the brain.

Where does the name come from?

Arnold Pick was the doctor who was the first to describe the disease in 1892.

Other names for the disease

Many doctors now prefer to reserve the name “Pick’s” for just one of the types of changes in the brain tissue (histology) that may be seen with the disease. You may come across several different names which are explained below: Frontotemporal lobar degeneration, frontotemporal dementia, semantic dementia and primary progressive aphasia.

Frontotemporal lobar degeneration

Many doctors now prefer this name for the disease. Patients who would have previously been told they had Pick’s Disease may now be told they have frontotemporal lobar degeneration. The name refers to the parts of the brain that are affected: the frontal and temporal lobes (at the front and side of the brain respectively).

Frontotemporal lobar degeneration can present with three different patterns of symptoms: frontotemporal dementia, semantic dementia or primary progressive aphasia. In frontotemporal dementia the frontal lobes (controlling behaviour, organisation and planning) are affected first, whilst in semantic dementia it is the temporal lobes (controlling language) that are affected first. In primary progressive aphasia, the disease starts in an area at the back of the frontal lobes and front of the temporal lobes.

What are the Symptoms?

Frontotemporal dementia
Personality change is an early feature. The person may lose their inhibitions and become extrovert, or alternatively may become apathetic and withdrawn. They may talk to strangers, make inappropriate remarks in public and be rude or impatient. They may become aggressive which may be quite out of character, and may develop fixed routines. Some people begin to hoard things and become obsessive. Behaviour may be sexually suggestive, though a loss of interest in sexual acts themselves is also common. Often the person with dementia will be unaware of the problems. People may also develop a sweet tooth and overeat leading to gain in weight. Excessive alcohol intake may occur. Spending money and losing cash often causes problems. In the later stages people with the illness may compulsively put objects in their mouths. In the early stages memory is not usually affected. However sometimes difficulties in organisation and concentration may lead to an apparent memory problem. People may be very distractible. Later in the disease a more generalised dementia can develop.

Semantic Dementia
This begins with loss of knowledge about the world, which often presents as problems with language. Although people can still speak fluently they lose the words for certain items and also lose the knowledge of the meaning of the word. For example, someone may not only forget the word “hippopotamus” when shown a picture, but also loses all the knowledge they once had about this (e.g. that it is an African animal that lives in rivers). However, unlike Alzheimer’s disease, memory for day to day events may be good. People may also have difficulty recognising what things are. At later stages, personality is often affected.

Primary Progressive Aphasia
People gradually develop difficulties with their speech (aphasia), which becomes slow and laboured and contains errors. This can be an isolated problem, and personality, memory and understanding may be almost normal for a long period. Eventually other areas do become involved and at a late stage a more generalised dementia becomes apparent.

What is the life span of the disease?

This varies quite a lot between individuals. It may last from 2 to more than 10 years, and a long duration of illness is quite common.

Links to other diseases

A small number of people who have motor neuron disease may go on to develop frontotemporal lobar degeneration, with symptoms like those described above. Occasionally, the change in behaviour develops before any weakness or wasting, and so before motor neuron disease is diagnosed. This is not common however.

Who can get Pick’s Disease?

It usually affects men or women in their 40s and 50s. However a few patients are affected as young as in their 20s, and older people do sometimes develop the disease.

What is known about the brain changes in the disease?

Loss of cells from the frontal and temporal lobes of the brain leads to shrinkage (atrophy) of these areas that may be visible on a brain scan. The brain changes have also been studied in post mortem examinations when the brain can be examined under the microscope (“histology”). Several different types of brain changes can cause the same symptoms. One example is deposition of a protein called tau.

Is it inherited?

In less than a half of cases a person with the disease may have a family history of the disease in one of their parents, brothers or sisters. In these cases the cause may be genetic. Mutations (faults) in the tau gene on chromosome 17 are the cause in some of these cases. In other families with several members affected and no tau mutation is found, it is likely that other genes, which are yet to be discovered, can cause the disease.

How is it diagnosed?

The diagnosis is based on a clinical assessment and further tests are often suggested to investigate the problems further. These may include brain scans (MRI or CT), neuropsychology assessments, blood tests and EEG (electroencephalography). Not all tests are suitable for every person. There is no one test that proves or disproves the diagnosis, but they all provide information that overall aids the doctor in reaching a diagnosis.

Can it be treated?

There is no cure for the disease at present. Sometimes antidepressants or tranquillisers may be suggested to control particular problems. Knowing more about the disease and why the person is behaving as they are can in itself be an effective means of helping people cope with the disease.

Dr. Alison Godbolt,
Dementia Research Group

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