Pick's Disease

Pick's Disease - Frontal Lobe Dementia/Memory Loss

Pick's disease is a cause of dementia similar to Alzheimer's disease involving a progressive decline in a person's mental powers over a number of years. It occurs far less frequently than Alzheimer's disease, and usually starts to take effect between the ages of 40 and 65.    Damage to brain cells is more localised than in Alzheimer's disease, and usually begins in the front part of the brain or frontal lobe. The initial symptoms affect personality and behaviour more and memory rather less than those of Alzheimer's.    In later stages the person becomes totally dependent on others and the condition is very similar to late stage Alzheimer's.    After death Pick's disease can be distinguished from Alzheimer's because there are no 'senile plaques' and characteristic changes in affected areas, known as Pick's bodies and Pick's cells, are found.    The condition was first described by a German neurologist, Arnold Pick, in 1892.

What is frontal lobe dementia?

Frontal lobe dementia is the name given to any dementia caused by damage to this part of the brain. It includes Pick's disease but can also be caused by other diseases. They all have similar symptoms and prognoses. Find more on Frontal lobe dementia

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 are the symptoms of Pick's Disease?

Early symptoms can affect behaviour, and sometimes language. People may show a change in their character and in their social behaviour. For example, they may show insensitivity, which is especially noticeable in a person who may have previously been very considerate of others. A person with frontal lobe dementia may become obsessive and repeat the same action over and over again.

Language problems often occur early in the disease and may range from limited speech to total loss of speech. Repeating phrases over and over, or echoing what others have said are also common symptoms. Instead of being able to find the right word to describe an object, the person with frontal lobe dementia may give a description of it instead. For instance, instead of
naming a watch, the person may refer to something you tell the time with.

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.

Prognosis and treatment of Pick's Disease

As yet, there is no cure for Pick's disease and neither can the progression be slowed down with any medication 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).

Niemann Pick disease

In 1914, German pediatrician Albert Niemann described a young child with brain and nervous system impairment. Later, in the 1920's, Luddwick Pick studied tissues after the death of such children and provided evidence of a new disorder, distinct from those storage disorders previously described.

Today, there are three separate diseases that carry the name Nieman Pick: Type A is the acute infantile form, Type B is a less common, chronic, non-neurological form, while Type C is a biochemically and genetically distinct form of the disease. Recently, the major locus responsible for NiemannPick type C (NP-C) was cloned from chromosome 18, and found to be similar to proteins that play a role in cholesterol homeostasis.

Usually, cellular cholesterol is imported into lysosomes'bags of enzymes' in the cellfor processing, after which it is released. Cells taken from NP-C patients have been shown to be defective in releasing cholesterol from lysosomes. This leads to an excessive build-up of cholesterol inside lysosomes, causing processing errors. NPC1 was found to have known sterol-sensing regions similar to those in other proteins, which suggests it plays a role in regulating cholesterol traffic. Find more information on Niemann-Pick Disease

Dementia Overview
Multi infarct Dementia
Fronto Temporal dementia
Lewy Body dementia
Frontal Lobe dementia
Parkinsons-Dementia
Pick's disease
Niemann-Pick Disease

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