RISK FACTORS FOR DEVELOPING ALZHEIMER’S DISEASE: DOWN’S SYNDROME

April 2nd, 2009

It has been mentioned already that there are similar abnormal changes in the brains of older people with Down’s syndrome and sufferers from Alzheimer’s disease. In addition to the structural changes there are also similar biochemical abnormalities. Whether or not older people with Down’s syndrome are particularly likely to develop dementia is, however, a matter of contention, as with so many other aspects of Alzheimer’s disease. In one study, less than half of the group of Down’s syndrome sufferers over the age of fifty demonstrated significant intellectual decline; in others, psychological impairments characteristic of Alzheimer’s have been found in the majority if not all.

Part of the problem lies in the fact that we don’t have adequate tests to measure dementia in people who have a pre-existing intellectual problem. The existing tests on previously normal people are difficult enough to interpret, so results for people with

Down’s syndrome can be very misleading. This is particularly true if all the Down’s syndrome subjects are studied only once and a judgement made as to whether, at say the age of fifty, they are suffering from a dementia-like illness. The best way of deciding whether or not dementia is an inevitable accompaniment to increasing age in Down’s syndrome is to undertake a longitudinal study. This means regular assessment over a period of time, providing an opportunity to compare change in intellectual ability over a number of years. Further research of this type is needed, but if it is confirmed that subjects with Down’s syndrome can exhibit the abnormal changes in the brain that occur in Alzheimer’s disease, yet do not all develop dementia or significant intellectual decline, then this would have important implications for the significance of the presence of senile plaques and neurofibrillary tangles, etc. in the diagnosis of Alzheimer’s disease.

The presence of plaques and tangles in the brains of people with Down’s syndrome stimulated examination of chromosome 2 I. The chromosomes are the structures on which all genes lie, and in Down’s syndrome there is extra chromosome 2 I material. At one time it was postulated that people with Alzheimer’s disease also had extra chromosome 2 I material. It was therefore possible to conjecture that an excess of genetic material on chromosome 2 I may cause the abnormalities in the brain in both Alzheimer’s disease and Down’s syndrome. This view appeared particularly attractive when it was discovered that the gene responsible for the formation of the substance in the centre of the senile plaque was also on chromosome 2 I. It seemed as if at least part of the cause of Alzheimer’s disease had been found. We now know that it is not as simple as this; although chromosome 2 I is implicated in the development of the senile plaque protein, it seems that the earlier hypothesis that this particular gene was the Alzheimer gene is unlikely to be true.

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SYMPTOMS OF ALZHEIMER’S DISEASE: TEMPORAL LOBE CHANGES

April 2nd, 2009

This is the part of the brain that appears to bear the brunt of the disease process and in many ways it often seems as if it is the seat of the abnormal changes which eventually spread out from here to involve the other structures. The earliest symptom of Alzheimer’s disease is usually memory loss and it is the temporal lobe and in particular a special structure within it called the hippocampus that are particularly associated with memory.

Memory is often divided into two types, classified as short-term and long-term memory by most people working in this field. It may be more accurate to use a slightly different concept, that of recent and remote memory. In this discussion recent or short-term memory will be used to refer to memory processes relating to the preceding hours or days, occasionally a little longer, while long-term or remote memory can go back as far as childhood.

The way in which memory is affected in Alzheimer’s disease is, in trade union manner, ‘last in, first out’. A quite severely demented person, particularly with Alzheimer’s, can often remember in detail activities that happened in childhood while having forgotten what he or she had for lunch, even though that may have been only half an hour earlier. Some people use short-term memory to refer to the type of memory that only lasts a few seconds and this also is severely affected.

It would seem as if memory normally works by transferring information from very short-term memory processes to longer-term memory processes and the major defect in Alzheimer’s disease may be an inability to store any new information. Not only is it more difficult to put it into the long-term memory store, but once there it doesn’t seem to last as long as information that was put there years before.

The consequence of this memory problem is the familiar picture of a sufferer from Alzheimer’s disease being unable to grasp what is said to him or her, needing to have items repeated, needing to write lists, forgetting to keep appointments, and so on. This can often lead to hazards in the home and as the dementing process advances it is not uncommon for relatives to discover that saucepans are put on the stove and allowed to boil dry, or gas taps turned on but not lit.

An example of this kind of behaviour is the person suffering from Alzheimer’s disease who went shopping in a nearby town, having driven ten miles from the village in which he lived. After completing as many of the purchases as he could remember and having got into difficulty in working out the change, he then set out to find his car to make the return journey home. Unfortunately, he could not find his car anywhere, decided it had been stolen, and managed to walk all the way home, turning up several hours late, having caused much distress to his wife. This example illustrates two aspects of memory. On the one hand he was unable to remember where he had parked his car, which was in fact only about thirty seconds’ walk from the store in which he had done most of his shopping; on the other hand the long journey home was sufficiently familiar to enable him to arrive back safely, although rather late. When questioned he said that he hadn’t been able to telephone his wife as he couldn’t remember their telephone number and he hadn’t been able to take a bus because he couldn’t remember the name of the village in which he lived; being unfamiliar with the bus service, he was unable to ask someone which bus he should take.

The pattern of memory loss, particularly the differentiation between very short-term (lasting only a few seconds) and longer but still relatively recent memory function, changes as the disease progresses, with the very short-term memory traces becoming more disturbed as the disease advances, whereas early on in the condition it is memory loss over a period of a few minutes or so that is most marked.

Doctors often try to test memory by asking questions that test the subjects’ ability to place themselves in the correct environment — space or time. The former is particularly affected by the context in which the question is asked. For instance questioning an Alzheimer’s disease sufferer after he or she has been moved to a hospital, clinic, or ward will frequently reveal disorientation in space, while the same question asked of him or her at home may elicit the correct answer.

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LIVING WITH ALZHEIMER’S DISEASE: THE SIZE OF THE PROBLEM

April 2nd, 2009

Dementia probably afflicts in the region of a half to three-quarters of a million people in the UK. It is therefore a problem that already surpasses Aids in its magnitude and, unlike Aids, many of the causes of dementia, especially Alzheimer’s disease, are conditions for which there are no known preventative or therapeutic measures currently available. It is often estimated that approximately 7-10 per cent of the population aged sixty-five and over suffer from one of the dementias. This of course means that most people are not so afflicted and are, therefore, in a position to help those less fortunate than themselves. Clearly, those suffering from dementia are unable to improve their lot on their own, and cannot lobby for better facilities and care for themselves, or greater support for those looking after them. It is a great scandal that even in such a civilized society as ours, it is those who can make the biggest noise about their plight or who command a significant proportion of the vote, that are most likely to advance their position.

The size of the problem is going to escalate rapidly, especially in the next twenty years. Of those aged sixty-five to seventy, it appears that approximately one person in twenty has dementia, but the proportion of sufferers rises with increasing age, and of those over the age of seventy-five, one person in five will probably have some degree of dementia. The number of older people in our population is expected to increase greatly between now and the early years of the next century, and by far the largest proportional increase will be in those aged seventy-five and over. This means, of course, that the growing number of elderly people suffering with dementia is going to make greater and greater demands upon the rest of society, unless effective treatment or preventative measures become available in the near future.

Alzheimer’s disease is probably responsible for about 60 per cent, just a little under two-thirds, of all the cases of dementia. A further 15 to 20 per cent are probably caused by multiple infarct dementia and approximately another 15 per cent have both Alzheimer’s disease and multiple infarct dementia. This means that the other conditions, some of which are treatable, make up only a small proportion of the causes.

In approximately 10 per cent of people suffering from dementia up to the age of sixty-five, the underlying cause will be treatable. In older people, especially those over the age of seventy, this figure probably falls to 5 per cent, one person in twenty. This is nevertheless a significant and important number of people for whom treatment can alleviate an otherwise bleak future. Treatment of some of the underlying conditions will not necessarily reverse the dementia, but may only stop it from getting worse, but everybody who develops dementia should be screened for the underlying illness that is causing it as early as possible to limit the damage that is done before treatment is started.

Even for those in whom the dementia proves to be untreatable, once a diagnosis has been made it is easier to plan the future, advise relatives or other carers of what the future may hold and how they can best prepare themselves for what lies ahead. A lot of support is usually available, far more than is often realized, and access to this is facilitated once the diagnosis is known and accepted. In those parts of the country where sadly there is little support or none, knowledge of the diagnosis may allow relatives to get together to try to help each other and to lobby for more resources.

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ROUTINE PERIODIC MEDICAL EXAMINATIONS

March 12th, 2009

Here I would like to speak out loud and clear: I advocate compulsory periodic medical examinations for everyone.

Already there is the compulsory X-ray examination for tuberculosis. At schools, doctors and dentists give our children regular ‘physicals.’ We accept the fact that our community water and milk supply and the food we eat should be subject to inspection. We take it for granted that our children should be vaccinated against polio. We agree that individuals have a social obligation not to transmit illnesses to others. Nurses and individuals in certain other professions and jobs are examined regularly.

It seems obvious to me that this practice should be extended. Why not include everybody, in these days when we come into close contact with total strangers every day in trains, stores, movies, swimming pools, and so on? In addition to the examinations and tests for communicable diseases, optional examinations for other potential killers and disablers should become a national habit. These would include tests for diabetes, cancer, heart and kidney diseases, and other illnesses, according to the age or condition of the individual being examined. Naturally, I realize that this programme will not be introduced today or even tomorrow. I hope some of you will help to speed its realization.

When people come to accept such examinations and tests as a part of their regular lives, diseases can be prevented or cured without arousing alarm.

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HOW TO AVOID BEING A HYPOCHONDRIAC: CONTINUOUS HEALTH EDUCATION

March 12th, 2009

Most of the harm done by campaigns against the killing and disabling diseases stems from the fact that such campaigns are conducted over relatively short periods of time. Organizations that do excellent work every day of the year are limited to a week or so in which to educate the public and publicize their work. Whether they wish to or not, they must in a sense compete with other valuable organizations. If they want to put across their message, they must use advertising methods and publicity techniques similar to those intended to sell a commercial product.

This situation would be greatly improved if these organizations could obtain the money they need without having to engage in intensive fund-raising campaigns—for example, by increased donations from individuals and foundations and by aid from the Government. It would also help if the campaigns placed even more emphasis on the fact that the health hazards they are publicizing can be cured or controlled if they are detected early enough. As an example, this positive approach to the dangers of childbirth has done a great deal to eliminate fear and anxiety in prospective mothers.

But most important of all, health education should be a continuous process, so that organizations would not have to crowd their warnings and recommendations into a single intensive week.

Health education should begin in childhood. Some elementary schools train young children in many of the essentials of health and hygiene. Some high schools continue this excellent work, teaching first aid, human biology, and the prevention of disease. How about your school? By working through parent-teacher associations or by organizing interesting after-school programmes, parents can see to it that their children’s health education is not neglected.

This education should not end when young people leave school. Churches, adult education centres, clubs, trade unions, and other organizations should keep health on their agenda, working out ways of interesting their members by means of lectures, exhibits, and so on, and of bringing these projects to the entire community.

The public health department in your community can be of great assistance. Many of us tend to overlook this valuable agency. The effectiveness of your local health department depends to a great extent on the interest of the local citizenry. Public health departments and health inspectors have learnt how to get excellent results without resorting to scare techniques.

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HOW TO AVOID BEING A HYPOCHONDRIAC: THE VALUE OF HEALTH CAMPAIGNS

March 12th, 2009

I am by no means opposed to health campaigns. On the contrary, I feel that they and the voluntary or governmental agencies sponsoring them do wonderful work. They alert and educate the public. They raise money to carry out vital research and to assist the victims of various diseases. They deserve great credit for helping to eliminate or control many illnesses that once took a toll of disability and death.

It is impossible even to estimate the number of lives that have been saved and the amount of suffering that has been prevented by the efforts of national health campaigns. The endless, tireless—and unpublicized— work of our local public health departments protects us against typhoid fever, dysentery, undulant fever, and other milk-, water-, and food-borne diseases. Public health agencies have all but eliminated malaria, smallpox, and yellow fever.

Not so very long ago, a man who was even suspected of having leprosy might be stoned to death, and people who had touched him were apt to consider suicide. We know now that the danger of catching leprosy by touching a leper is, practically speaking, non-existent.

Having eliminated the ignorance responsible for such panics, must we now pay for our knowledge by becoming a nation of chronic hypochondriacs?

My answer is No. We can have the tremendous advantages that come with enlightenment and at the same time avoid the danger of being frightened into becoming hypochondriacs by (1) continuous health education; (2) routine periodic medical examinations; and (3) a sensible attitude towards the calculated risk in health

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HOW TO AVOID BEING A HYPOCHONDRIAC

March 12th, 2009

When one of my non-medical friends learnt that 1 was writing the Reader’s Digest Family Health Guide and Medical Encyclopaedia, he remarked, ‘But don’t you think there’s a danger that it will make some people worry about themselves—that they’ll get to be hypochondriacs who think they have every disease they read about?’

Doctors know that patients do not need to read about actual diseases in order to suffer from imaginary ones. We have all had patients who could invent more ailments than medical science ever dreamt of.

After each health campaign or drive to raise funds for a health organization, doctors are besieged by people who are afraid they have the disease that has been publicized. Campaigns concerned with cancer, tuberculosis, heart disease and high blood pressure, diabetes, obesity, rheumatism and arthritis, multiple sclerosis, cerebral palsy, muscular dystrophy, and venereal disease give suggestible people something to worry about all year long.

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