Pick's Disease

An Explanation by Penelope Roques

What is it?

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”. Pick’s disease is thought to be due to a build up in the brain of an abnormal form of the protein tau..

What are the Symptoms?

The frontal lobes of the brain are where we plan and monitor performance and where we control our behavioural and emotional responses.  Damage to the frontal lobes may cause a variety of symptoms that differ greatly from one individual to another.  There are however a common “core” of symptoms and some or all of these may be present at different stages of the illness. The temporal lobes are responsible for our speech and comprehension of language.  Damage to these areas of the brain may cause speech loss.

Personality Change: the person may lose their inhibitions and becomes extrovert or may become withdrawn.   They may talk to strangers, particularly children, make inappropriate remarks in public, and be rude or impatient.  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 which may be quite out of character. The development of routines and behaviour - for example the person may develop compulsory walking routines or become obsession in certain behaviours. Their behaviour is often described as childlike. Changes in sexual behaviour may be linked to general loss of inhibition or to apathy.

Speech problems: these are common and range from reduction in the quantity or quality of speech in some to total loss of speech in others. Understanding speech and writing is also affected.

Changes in eating habits:  overeating, changes in dietary preferences, particularly for sweet foods, often cause weight gain. Excessive alcohol intake may occur.  In the later stages sufferers may compulsively put objects in their mouths.  Swallowing may become difficult with patients choking on food.

Attention:   difficulty sustaining a line of thought or maintaining a conversation for any length of time often occurs.  People with Pick’s disease are often easily distracted.

In the early stages of the disease the person usually remains aware of time and is able to recognise people and faces 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.

What is the Life Span of the Illness?

Pick’s Disease may be a rapidly progressive illness, it may last between 2 to 10 years the mean length of illness being 7 years.

Who can get Pick's Disease?

The cause of this form of neurodegenerative disease is uncertain. There are overlaps between Alzheimer's disease and Parkinson's disease. Genetic studies are making some progress in revealing a matrix of different genes, which may contribute to development of DLB. This appears to be complex but may explain firstly the relationship of DLB to the other primary Lewy body disorders including Parkinson's disease and secondly the association with Alzheimer's disease. It remains to be seen whether genetic testing will be sufficiently simple to help in clinical diagnosis.

Management

There are no drugs so far which affect the progression of the disease.  The drugs that are designed for the treatment of Alzheimer’s disease are contraindicated in frontotemporal dementia as they may increase aggression.  Management lies in coping strategies such as side stepping issues rather than being confrontational and working round obsessions rather than trying to change them.  Professionals such as Speech Therapists and Occupational Therapists may be helpful.  A serious problem is boredom and carers have found such diverse new hobbies as art, music, rug making, walking and jigsaw puzzles helpful.

Why is it called Pick's Disease?

In 1892 Arnold Pick described a man who had presented in life with progressive loss of speech and dementia. After death the patient’s brain was found to be atrophied   (shrunken, caused by brain cells dying) in localised areas as opposed to Alzheimer’s disease where the atrophy is more general. In Pick’s disease the frontal and temporal lobes are most affected.  In addition swollen brain cells (Pick cells) together with the presence of abnormal staining within cells (Pick bodies) are the hallmark of this disease.