Pharmahealthblog. Health and medical information

A debilitated person, one suffering from a chronic disease, is more likely to suffer from infections. Some of these have already been mentioned, such as the skin rashes that develop in hot, sweaty places that are not kept clean and the infection that can occur in the urinary tract, causing cystitis and frequently incontinence of urine. There are many other infections, the most important of which is probably pneumonia. There may well be little outward sign of pneumonia because many old people don’t have a rise in temperature or produce phlegm as younger people do when they develop the infection. It can only be diagnosed by a doctor’s careful examination. Often the only clue to the presence of any infection may be a worsening of the level of confusion. If this happens without there being an obvious cause, it is important to ask the doctor to come and assess the situation.

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Some people with dementia are very unpleasant to those around them. They may accuse a spouse of trying to harm them, of stealing their belongings, of plotting against them, and so on. This type of behaviour is really very upsetting and often causes deep hurt. There is little one can do about it, as no amount of reasoning will make any long-term difference. In the short term, however, reassurance can sometimes be helpful, as the outburst may really be a means of saying ‘I don’t feel loved’ or ‘I am angry because I am frightened.’ If this kind of emotional insecurity is responsible, loving reassurance and a hug may well be the answer, but don’t feel rejected if this doesn’t work. There must be many other reasons for a reaction of this nature and the underlying cause is often not apparent.

Very often all that one can do is to try to ignore such comments and remember that they are not really the expression of a considered thought, but the results of brain damage. The sufferer is probably as upset and distressed about the situation as the person to whom the remarks are directed.

Above all, don’t try to react by justifying yourself or arguing. This could well result in a catastrophic reaction. If the situation has arisen because the sufferer has forgotten who a person is, even though they should know him quite well, and mistakes him for a stranger who, for instance, could be a thief, try to explain to the person or persons involved and at the same time reassure the sufferer.

If things get lost, it is very likely that they have either been put down in a strange place or that they have been deliberately hidden. You will soon learn about the favourite hiding-places. This will help you to recover things quickly when the sufferer begins to complain that they have disappeared and has forgotten that he or she has hidden them. If a lockable drawer or cupboard is the hiding-place have duplicate keys made or remove the existing key in case objects are locked away and the key in turn is hidden elsewhere or lost. It is also a good idea to keep objects such as jewellery, cash, legal documents, and so on, safely away from a demented person in case they too disappear.

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There have been many studies of the inheritance patterns of Alzheimer’s disease in families. Some of these studies are more reliable than others; in many, the criteria upon which the diagnosis has been made have been rather loosely defined. The most important studies are those whose data is based on post-mortem confirmation of Alzheimer’s disease. Studies that rely on the correct diagnosis being made during life are less satisfactory, not being backed up by pathological confirmation. In the best centres the diagnosis is probably only made with an accuracy of around 80 per cent, and in some it is no better than 50 per cent.

The results of many of the studies that have been undertaken are conflicting. There is, however, little doubt that in some families, where the disease strikes predominantly younger people, there is a very strong hereditary factor. Whether or not a person develops the disease is dependent upon the genetic material that is present within the cells of the body. This genetic material is divided up into small sub-units called genes; the more powerful genes are described as dominant and the less powerful ones as recessive. Even if the Alzheimer’s disease is controlled by a dominant gene in a family with a strong pattern of the illness, the risk of a child of an affected individual developing this type of dementia is less than 50 per cent. In many cases it is considerably less than this and once the gene becomes diluted even further, to grandchildren, nephews and nieces, etc., the risks are even smaller.

In most instances the disease is not caused by a single dominant gene. In practical terms this means that someone who is a member of a family in which several people have suffered from Alzheimer’s disease, even if the onset was before the age of sixty-five, faces a risk of developing the condition almost certainly less than 50 per cent, maybe very much less.

In most cases of Alzheimer’s disease it is very probable that heredity doesn’t play any part at all. If the family history contains only a single older person who has had this diagnosis made, there is virtually no increased risk for others in the family.

The risk is further diminished if it turns out to be true that it is not the inheritance of the gene itself that matters, but whether the gene makes us more sensitive to outside agents. An example of this is the relationship between smoking and lung cancer. We all know of people who smoke sixty cigarettes a day and yet manage to survive into their nineties without developing cancer. On the other hand we know also of other smokers who succumbed to lung cancer in their fifties or sixties. It is possible that within the genetic make-up of all of us, our genes determine how sensitive we are to the action of the chemicals in tobacco smoke. If we inherit a gene that makes us very sensitive, then we are more likely to develop lung cancer than another person who has inherited a gene that makes him resistant to the chemicals in cigarettes.

It could well be the same with Alzheimer’s disease, and for that matter the other dementias. Whether we do or do not develop the condition may depend not just upon the inheritance of a particular gene, but whether we are also exposed to something which that gene makes us particularly sensitive to. This external agent could be anything from the chemicals that we put in our food or spray on our crops to a particular virus or the way in which we react to a particular virus.

My own feeling is that the increased risk of a person developing Alzheimer’s disease is very small if the only member of the family with this diagnosis is an elderly grandparent. If two grandparents have been affected the risk rises but is not of undue concern. On the other hand, if three or four members of the family have developed the disease before the age of sixty-five there is indeed a significant risk of other members of the family being affected. The odds are, however, still likely to be better than 50 per cent in favour of any individual not contracting the condition.

It is possible that in some families a single dominant gene may be responsible for Alzheimer’s, but in most cases, if there is a genetic basis at all, it will more likely result from the interplay of several genes or the interaction between a person’s genetic makeup and other factors in the environment.

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Most doctors think of Alzheimer’s disease as being characterized by abnormalities of cortical function which they call amnesia, aphasia, and agnosia. This is not as complicated as it sounds. The cortex is of course the outer layer of grey cells which lie on the surface of the brain and the three terms refer, respectively, to general memory failure, difficulty in remembering or understanding the names of objects, and failure to recognize people or objects. The symptoms are, however, very much more complicated than this and the disease often progresses along a different path in different individuals, although the later stages of the different courses of the disease tend to resemble each other. For this reason 1 am going to describe the symptoms caused by the malfunction of the three major lobes of the brain — the temporal lobe, the frontal lobe, and the parietal lobe – although there is some overlap in the relationship between structure and function.

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A lot of confusing terminology has arisen, which can lead to misunderstanding, not only in the minds of lay people but also among the medical and associated professions. The term dementia means simply a reduction in or impairment of mental powers. It does not mean that a person is mad — indeed often the only early sign is an exaggeration of the memory loss that so many of us suffer as we grow older, though most people who are forgetful are not suffering from, and will never develop, dementia. As is explained later, dementia eventually involves far more than memory loss; symptoms may include disorientation in time, a tendency to become lost in familiar surroundings, difficulty in recognizing objects or people, and eventually an inability to carry out previously familiar tasks. The fact that several mental abilities are affected is one of the hallmarks of dementia although of course, to begin with, the impairment of mental functioning is less.

It is important also to realize what is not dementia. Dementia is very definitely not a part of normal ageing, and can in fact occur in quite young people, although not as frequently as in those who are over the age of seventy-five. It is also not a diagnosis. The term dementia merely describes the state that a person’s mind is in; the way it is working or not working, a person’s behaviour pattern, and so on. It is rather akin to saying that one is short of breath. In the case of breathlessness something has usually affected the way in which the lungs or the heart work, and it is the underlying cause of this, for example pneumonia or a heart attack, that is the true diagnosis.

Many conditions cause dementia. The most common is Alzheimer’s disease and although it can occur in people who are old or young it very rarely occurs before the age of forty-five. The next most common cause of dementia is a series of small strokes, which cause the death of brain cells. Such small strokes may occur without any of the usual manifestations — the paralysis of an arm or a leg or part of the face that is so often the sign of a stroke. Some unfortunate people are affected both by Alzheimer’s disease and by dementia due to strokes.

Alzheimer’s disease is named after the German doctor Alois Alzheimer who first described it in detail in 1907. The changes in the brain, described in more detail in later chapters, are caused by a slowly progressive loss of the nerve cells lying in the cerebral cortex – the thin rim of grey matter on the outside of the brain — and in other collections of nerve cells lying more deeply within the brain. Dementia that is caused by small strokes usually results from a succession, often over many years, of blockages to small arteries affecting areas of the brain. When these are deprived of their blood the loss of oxygen and nutrient materials results in the death of the cells and their fibres. This type of dementia is often also called multiple infarct dementia – MID for short.

There are of course many other causes of dementia; some of them, albeit a minority, are treatable, which is why it is so important that a proper diagnosis of the underlying illness is always made. These causes will be described in later chapters.

Many other terms for describing the condition are in common use, among them chronic brain failure, organic brain failure, organic brain syndrome, hardening of the arteries, and senility. The latter is an unfortunate term and implies that the condition is associated with ageing, which in turn implies that it is inevitable and untreatable.

It is essential to realize that dementia is not the same as the more short-term episodes of confusion that are suffered by many old people as a result of infections such as pneumonia or urinary tract infection, the side-effects of drugs, or as a consequence of other medical conditions. These are more correctly called acute confusional states and don’t produce the gradual and relentlessly progressive deterioration in mental functioning that is a feature of dementia; on the contrary, the onset is usually fairly rapid and the sufferer is often in a state of semi-consciousness or subconsciousness. The most common example of this is the delirium associated with a high fever.

Finally, there is a lot of misunderstanding about the role of ‘hardening of the arteries’ or ‘arteriosclerosis’. These two terms essentially mean the same thing, and are often used to describe the condition that is causing a person’s dementia. This dates back to an extremely old-fashioned and incorrect belief that hardening of the arteries narrowed them to such an extent that the whole brain, or parts of it, had its blood supply slowly strangled and in consequence could not work properly. It has been known since the early seventies that this is not the case, yet it is still given as the diagnosis by many doctors. As mentioned above, the abnormality of the blood-vessel system that most commonly causes dementia is that a lot of small areas of brain suddenly die because their blood supply has been completely obstructed, thereby producing small strokes. ‘Hardening of the arteries’ and ‘arteriosclerosis’ are often incorrectly applied in a blanket manner to anyone with dementia without any thought about the real nature of the underlying condition. It is probable that the majority will have Alzheimer’s disease, and that the person applying the label is either not up to date in his or her knowledge or does not really understand the issues involved.

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