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A Brief Note from the Chairman

Dear Friends

It is my unfortunate task to inform everyone that our daughter Hilary died on 15th December at the age of 31, after a long 8 year struggle against Pick's Disease.

Jean and I would like to thank everyone in the Pick's Disease Support Group without whose support during this period the task of coping would have been much harder.

During this controversial time of organ retention we decided to donate Hilary's brain to Professor Neary's team at Manchester Royal Infirmary and hopefully this action will go a long way to help others in the future and lead to a greater understanding of this awful debilitating illness.

On the positive side you will receive or will be able to obtain from Carol Jennings a sponsor form. Ian Fisher has kindly offered to run in the London Marathon in Hilary's memory to raise funds for the PDSG, and we would ask you to support this generous effort. Ian finished 34th in last years Marathon and hopes to better his performance this year.

Jean and I send all our best wishes and hope to see as many as possible at our AGM in Nottingham this year, when we can all discuss and debate how to take our group forward in the future.

Kind Regards,

David Hunter

There are several issues in talking about staging:

1) More so than in Alzheimers, FTD/Picks patients can vary enormously in the duration and course of their illness. Some have been described as lasting 30 years from initial behavioural change (although one wonders whether this early change was just a coincidence and not a symptom of their later disease), while others progress very rapidly. It is possible that we are talking about a range of different diseases which we can’t tell the difference between yet. Certainly one cause of FTD is Motor Neurone Disease (also called Amyotrophic Lateral Sclerosis, and Lou Gehrig’s disease in the US); this causes progressive muscle weakness and results in death due to breathing or swallowing difficulties, often within 18 months of diagnosis.

2) The important thing to remember is that the symptoms simply reflect the brain region which is being affected. Although in general some regions might typically be involved later than other regions, the disease process can spread differently in different brains. For example, the left frontal lobe (at least in right handed people) is important in language production. Loss of expressive language leading to muteness is a universal feature by the end stages of FTD/Picks, but some people with the disease mainly on the right side, or in the temporal lobes, won't lose language 'til late, while others (in whom the disease seems to start in the left frontal lobe) lose expressive language very early, and are said to have Primary Progressive Aphasia. It’s the same disease (as far as we know) but the symptom purely depends on which part of the brain is affected. So it’s difficult to say what the loss of expressive language (just as one example) means in terms of staging.

3) It can be useful to differentiate cognitive/behavioural symptoms from neurological ones, even though the distinction is not always clear. For example swallowing problems could be due to a failure to initiate swallowing (because too busy stuffing one’s mouth, for example; a behavioural symptom), but might be due to muscular weakness or loss of throat reflexes (a neurological symptom). Incontinence similarly can be behavioural (ignoring bladder signals, inertia, lack of concern about ‘accidents’), or neurological (loss of sensation or control). Problems with walking, falls, muscular rigidity, and major voice changes tend to be neurological. FTD/Picks (like Alzheimers) is usually a ‘cortical’ disease, which is to say that it primarily affects the cerebral cortex (Latin for bark) which is the grey matter on the outside surface of the brain (like the bark of a tree). This is where most cognitive/behavioural processes are controlled. Regions in the subcortex, below the surface of the brain, are more involved in neurological functions.

Typically the disease starts in cortical regions and the later emergence of neurological signs signals that subcortical regions are being affected (the frontal lobes have major subcortical connections). So neurological features tend to occur with further progression. BUT, subcortical regions can be affected early, and there are a number of conditions (not strictly speaking FTD/ Picks) which START in subcortical regions and which also affect the frontal lobes (giving the classic symptoms of a frontal dementia).

Neurological symptoms tend to be more incapacitating, and will generally be associated with shorter survival. So in that sense you would say that they reflect a later stage of the disease (given that the patient is closer to death). But if neurological signs appear early it doesn’t mean that the disease has had a rapid course; it may simply mean that subcortical regions were affected early. It is quite possible to run the full course of FTD/Picks (like Alzheimers) and exhibit no neurological features at all. Thus it’s difficult to stage people on the basis of the appearance of symptoms, except in general terms of increasing incapacity as an indication of probable survival time.

Barney Casey is an Australian neuropsychologist who has been researching FTD/Picks Disease for the past decade. He works at The Centre for Education and Research on Ageing, at Concord Hopsital in Sydney. The above is edited from a recent post of his to the Pick’s Disease Support Group.

Short Breaks

Val Bywater Tel: 0148 356 2233 and Lynn Goodfellow Tel: 020 8788 7861 are sometimes able to negotiate very competitive rates for short breaks for affected, carers and ex-carers at holiday centres. If you wish to participate in the future please call Val or Lynn and they will keep you informed of any future special offers. These breaks are usually for 3 or 4 nights, self-catering and represent very good value for money. Planned outing to Wetland Centre, Barnes, London SW13. Carers go free but charge of £5.00 for people with dementia. Any newcomers please phone Lynn after 1st March. Date to be advised.