FRONTAL LOBE DEMENTIA AND PICK'S DISEASE

Like Alzheimer's disease, Pick's disease and frontal lobe dementias cause a progressive and irreversible decline in a person's abilities over a number of years. Frontal lobe dementia and Pick's disease are the cause of less than 10% of all dementias and may usually be distinguished from Alzheimer's disease early in the course of the illness. Arnold Pick first described Pick's disease in a 71 year old man in 1892. Pick's disease affects the temporal lobes of the brain in 25% , frontal lobes in 25% and both frontal and temporal lobes in 50% of cases. Frontal lobe dementia affects the frontal lobes initially. Damage to the frontal lobes leads to alterations in personality and behaviour, changes in the way a person feels and expresses emotion, and loss of judgement. The following information about Pick's disease is also appropriate for other frontal lobe dementias.

THE MORE COMMON SYMPTOMS INCLUDE:

Personality and Behavioural Problems The first symptoms of Pick's disease are typically psychological and behavioural problems. Initially, the diagnosis may be suspected to be a mental illness. People may show symptoms of a change in their character and in their social behaviour. They may have a diminished drive and often their expression is vacant. A person with Pick's disease may show insensitivity, which is especially noticeable in a person who previously showed consideration to others. There may be a lack of restraint and the person may be caught up in the criminal justice system because of stealing or behaviour which lands him/her in trouble. Another sign of Pick's disease is that the person often becomes quite obsessional during the early stages, insisting that everything is absolutely neat and in order, or repeatedly washing his hands or observing little rituals each time a certain task is carried out. There may be sexual misadventures, social graces may deteriorate, the person may talk to strangers, make inappropriate comments in public and indulge in practical jokes. Unfortunately, people with Pick's disease often suffer from significant absence of insight into the effects of their behaviour. Language Problems Language problems also occur early in the disease: limited speech output, lack of speech spontaneity, stereotyping of phrases (ie., use of pat phrases repeatedly and excessively), perseveration (a meaningless persistence of verbal activity), a decreased vocabulary, a considerable amount of repetition, especially of brief words and phrases. Often there is jargon and instead of being able to find the word to describe an object, the person with this disease will give a description of it instead (ie., a "watch" referred to as "something you tell the time with"). This means that the person may not be able to name objects early in the disease. Eventually the person becomes mute for periods and then completely mute by the end of the disease.

Kluver Bucy Syndrome

This refers to a group of problems which are relatively common in Pick's disease. These are hypersexuality, gluttony, and an obsession to touch and seize any objects in the person's field of vision. Overeating may lead to considerable weight gain.

WHO CAN GET THE DISEASE?

Anybody can get the disease, although there may be geographical differences in the incidence of Pick's disease. Some studies suggest the disease to be more common in women while others suggest a greater risk in men. The most severe cases of Pick's disease occur before the age of 60 years. The highest incidence is between 50 and 60, but people may develop the disease earlier or up to 80 years.

WHAT IS THE CAUSE?

As with Alzheimer's disease, in most cases, the cause cannot yet be determined. However, there are strong genetic components in certain families. When there is a genetic element, it is autosomal dominant, (on average, half of the children of an affected parent will develop the disease, but half will not) but is clearly modified by a number of environmental factors as yet to be discovered. The genetic component has been variously described as affecting 20 to 50% of people with Pick's disease.

DIAGNOSIS:

Although Pick's disease can only be conclusively diagnosed after a person's death by a post mortem examination of the brain, there are several techniques, such as brain scans and EEGs, which can be used during the person's lifetime to give a probable diagnosis. These techniques can help in determining whether the dementia is likely to be Pick's disease or a closely related disorder, for example, Alzheimer's disease.

PROGNOSIS and TREATMENT:

As yet, there is no cure for Pick's disease, neither can the progression be slowed down with any drug treatment. Probably because Pick's disease is much less common than Alzheimer's disease, there is less research into Pick's, and there are currently no drug trials taking place in relation to treating Pick's disease. The course of Pick's disease is an inevitable progressive deterioration. From the onset of the disease, life expectancy is 2-15 years, with an average of 6-12 years. Death is usually caused by infection. Some of the symptoms of the disease can be treated effectively. For example, certain medications can reduce some of the behavioural problems. Also knowing more about the disease and why the person is behaving as they are can in itself be an effective means of helping people to cope with the disease. (The Alzheimer's Association NSW has an extensive dementia library, education and counselling services).

DIFFERENCES BETWEEN PICK'S DISEASE AND ALZHEIMER'S DISEASE:

The main difference between Pick's disease and Alzheimer's disease is that the damage occurs in different areas of the brain, at least in the early stages. In most cases of Pick's disease, the frontal and temporal lobes of the brain are the areas affected and with Alzheimer's disease, the temporal and parietal lobes are affected. Mendez and co-workers (1993) found, even in the absence of temporal or frontal lobe atrophy on CT brain scans, that Pick's disease can be distinguished from Alzheimer's disease if three of the following five characteristics are present early in the disease: presenile onset (under 65 years old); initial personality change; hyperorality (loss of normal controls, ie., excessive eating, indiscriminate putting things in one's mouth); disinhibition; roaming behaviour. Compared with Alzheimer's disease, impairment of intellect and memory occur later. As well, epilepsy is uncommon, delusions and hallucinations are rare, and apraxia (inability to perform, command, or imitate a familiar action) and agnosia (inability to recognise objects) are less common. Gait and muscle tone are less affected. In the late stages, Parkinsonism symptoms, immobility, incontinence and mutism occur. In the terminal stage, the different dementias are clinically undistinguishable.

CARERS:

Being a family carer of a person with dementia, regardless of whether it's Alzheimer's disease, Pick's disease or another type, can be physically and emotionally exhausting. However, Pick's disease can often be even harder on families because: of the personality changes and behavioural quirks which are very distressing; often there is a delay in diagnosis of the disease; it receives less publicity than Alzheimer's disease and therefore, is even less understood by the public, friends, etc. often, persons affected with Pick's disease are younger; language problems develop earlier. Families of people with Pick's disease and other frontal lobe dementias need support in their caring role. The Alzheimer's Association NSW offers a variety of support services which can be accessed by ringing their HelpLine on 1800 639 331.

The majority of this HelpNote was written by Dr Keith Fleming, Department of Geriatric Medicine, ACT. It has been edited by Professor Henry Brodaty, Department of Psychogeriatrics, Prince Henry Hospital and Honorary Medical Adviser to the Alzheimer's Association NSW. REFERENCES: Berrios G.E.; Freeman H.L. (1991) Alzheimer and the Dementias: Eponymists in Medicine. Royal Society of Medicine Series. - London. Brun A. (1987) Frontal Lobe Degeneration of Non-Alzheimer Type 1 Neuropathology. Arch. Gerontol. Geriatr., 6, p. 193-208. Mendez M.F., et al (1993) Pick's Disease Versus Alzheimer's Disease: A Comparison of Clinical Characteristics. Neurology, 43(2), p. 289-292. Brown, Jeremy; Rossor, Martin (1996) Pick's Disease and Frontal Lobe Dementia. - Alzheimer's Disease Society UK

This information is one in a series of HelpNotes produced by the Alzheimer's Association NSW. The Alzheimer's Association NSW offers a variety of statewide services to people with dementia and their family carers. These include a free carer HELPLINE (1800 639 331), face-to-face counselling, education programs, support groups, a dementia library and books and videos for purchase. If you would like more information on this HelpNote topic, want other HelpNotes, or to access any of the above services, please ring 1800 639 331.

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