What Is Picks Disease?
Pick's disease can be defined as:
A progressive dementia commencing in middle life (usually between 50 and 60 years) characterised by slowly progressing changes in character and social deterioration leading to impairment of intellect, memory and language.
Why Is It Called Pick's Disease?
In 1892 Arnold Pick, a German neurologist, described a man who had presented in life with dementia and loss of speech. After death the patient's brain was found to be atrophied (shrunken, with brain cells having died) in localised areas as opposed to Alzheimer's disease where the degeneration generally affects most of the brain. In Pick's disease areas of the brain that are most affected are the frontal and temporal lobes. (Medical students often use the mnemonic that "Pick's disease picks off the frontal or temporal lobes but leaves the rest".) In addition, brain cells in these areas sometimes are found to be abnormal and swollen. These abnormal cells are the hallmark of Pick's disease and are called Pick's cells.
When such typical cells are not seen on post-mortem examination but the same areas of the brain are affected by cell death the case may be described as Pick's syndrome. The symptoms of the illness are very similar.
Pick's disease varies greatly in the way it affects individuals. There are however a common 'core' of symptoms. Some or all of these symptoms may be present at different stages of the disease.
The More Common Symptoms Include:
Personality change: the person may lose their inhibitions and become extrovert or may become withdrawn. They may talk to strangers, make inappropriate comments in public, be rude or impatient etc. They sometimes appear selfish which is caused by a loss of mental flexibility and being unable to see someone else's point of view. The person may become aggressive in a way which is quite out of character. Failure to recognise faces and using objects wrongly sometimes occurs. The development of routines and behaviour - for example the person may develop compulsory walking routines or become obsessional in certain behaviours. Their behaviour is often described as childlike.
Speech problems are common and range from a reduction in the quantity or quality of speech in some people to total loss of speech in others. Echoing what has just been said is a fairly common symptom.
Overeating, changes in dietary preference, obsessional cravings for certain types of food (usually sweet foods) - these symptoms are often accompanied by weight gain. Excessive alcohol intake may also occur. In the later stages sufferers may reach out for any available object and compulsively put objects in their mouth.
Attention problems lead to difficulty sustaining a line of thought or maintaining a conversation for any length of time.
Changes in sexual behaviour may be linked to general loss of inhibition or to apathy. Failure to consider partners' needs is not selfishness but caused by the changes in the frontal lobes of the brain. These changes in behaviour can be extremely distressing.
In the early stages of the disease the person usually remains aware of time and is able to recognise people and places unlike the usual presentation of Alzheimer's disease. However, failure to recognise faces and using objects wrongly sometimes occurs. Mathematical skills are usually relatively well preserved.
There have been some attempts to divide the development of Pick's disease into 3 stages:
Stage 1: disturbances of judgement and behaviour Stage 2: the development of other symptoms e.g. loss of speech and obsessional behaviour becomes more marked. Stage 3: generalised dementia
Who Gets Pick's Disease?
Anybody can develop Pick's disease. It affects men and women alike. Although it typically affects people in their 50s and 60s it has been diagnosed in people from the ages of 20 to 80.
Because so little is known at present about the causes of the disease it is impossible to identify risk factors for developing the disease.
Is It Genetic?
Research has not been wholly conclusive about the proportion of 'familial' cases of Pick's disease, but it does exist as an inherited disease in some families. The majority of cases of Pick's disease are sporadic with no other family members being affected.
In the familial cases it is known as an 'autosomal dominant' disease and tends to affect people by about 40 years of age. 'Autosomal dominant' means that if you carry the gene you eventually develop the disease, and if you carry the gene your children have a 50% chance of inheriting the illness.
Can It Be Treated?
As yet there is no cure for Pick's disease neither can the progression be slowed down. The rate of progression varies enormously between people ranging from a duration of less than 2 years in some to well over 10 years in others.
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 disease.
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