Alzheimer’s disease may first present as a full-blown syndrome, but more often it starts as isolated symptoms so focal that one may initially suspect a small stroke or tumour; it is only later that the generalised nature of the disease becomes evident (hence the frequent failure to diagnose Alz- heimer’s at the start). The early symptoms, whether they appear singly or in clusters, are usually subtle. There may be momentary illusions or misperceptions; or difficulties getting jokes or following arguments. But in general it is the most recently evolved functions that are the first to be affected.
In these very early stages, the dysfunctions tend to be momentary. But soon there are grosser disturbances of cognition, memory, behaviour, judgment, and disorientation in space and time, all finally coalescing as profound dementia. As the disease advances it may bring distressing personality changes and even violent behaviour in some people. Finally, every possible cortical disorder may be seen in this devastating disease, even though the paths by which the disease advances are so different in each patient.
Sooner or later patients lose the power to articulate their condition, to communicate in any way, except in so far as tone of voice, touch, or music can briefly reach them. Finally, even this is lost and there is indeed total loss of consciousness, of cortical function, of self-psychic death.
Caring for someone else, especially if that someone is already quite demented and is inexorably going downhill, can involve back-breaking physical exertion as well as a constant, almost telepathic sensitivity to what is going on in a mind now less and less able to communicate its thoughts, less and less able to have clear thoughts. People with dementia may get terrifyingly confused and disoriented. Such a burden can make the caretaker ill with stress. As a physician, I see this all too often.
And yet there are many ways the beleaguered patient may adapt and react, or even help themselves. One of my patients, very early in the course of her disease, suddenly found that she could no longer tell the time when she looked at her watch. She saw the position of the watch hands clearly, but she could not interpret them; for a split second, they made no sense, and then, equally suddenly, they did. The unintelligible periods lengthened to seconds, then minutes, and soon the watch hands were unintelligible all the time.
She was acutely and mortifyingly conscious of this deterioration; it gave her a sharp sense of horror, of the Alzheimer’s process behind it. But she herself was the one to make a crucial therapeutic suggestion: Why don’t I wear a digital watch, she asked, and have digital clocks everywhere? She acted on this, and although her agnosia and other problems continued to increase, she remained able to tell the time and organise her day for another three months.
Another of my patients, who was fond of cooking and whose overall cognitive powers were still very good, found that she could no longer compare the volume of liquids in different containers; an ounce of milk did not look the same if it was poured from a glass into a pan, and ludicrous errors started to occur. The patient herself, a former psychologist, ruefully recognized this as a Piagetian error, a loss of the sense of volumetric constancy that is acquired in early childhood. However, by using graduated vessels and measuring cups instead of trying to guess as she used to, she was able to compensate for the problem and to continue safely in the kitchen.
Such patients may perform badly on formal mental testing and yet be able to describe with clarity, vividness, correctness and humour precisely how one bakes an artichoke or a cake; they may be able to sing a song, tell a story, act a part, play a violin, or paint a painting with remarkably little impairment. It is as if they have lost certain modes of thought while retaining other modes perfectly.
It is sometimes said that people with Alzheimer’s do not realise that they are impaired, that insight is lost from the start. Although this may sometimes be so, it is more common, in my experience, for patients to realize their condition at first. Thomas DeBaggio, a writer and horticulturalist, was even able to publish two insightful memoirs about his own early-onset Alzheimer’s before the disease killed him at the age of 69. Most patients are frightened or mortified by the knowledge of what is befalling them.
Some continue to be severely terrified as they lose their intellectual competences and bearings and find themselves in a world increasingly fragmented and chaotic. But the majority, I think, become calmer with time as they perhaps start to lose the sense of what they have lost and find themselves shifted into a simpler, unreflective world. Such patients might seem to have regressed intellectually, so that they are once again like children. Kurt Goldstein, a neurologist and psychiatrist, would say of such patients that they were now in a lower, more concrete form of consciousness or being.
For John Hughlings Jackson, the great English neurologist, there were never just deficits with neurological damage, but “positive” symptoms, as he called them, “releases” or exaggerations of normally constrained neural functions. He spoke of “dissolution”, which was, for him, characterised by regression or reversion to more archaic levels of neural function — the reverse of evolution.
Although Jackson’s notion of dissolution in the nervous system as evolution in reverse can no longer be maintained in so simplistic a fashion, one does see some remarkable behavioural regressions or releases in a disease like Alzheimer’s. I have often seen patients with advanced dementia who show picking, hunting and brushing — a whole range of primitive grooming behaviours that are not seen in normal human development, but are suggestive, perhaps, of a reversion to a primate level. In the final stages of dementia, where no organised behaviours of any sort remain, one may see reflexes that are normally only seen in infancy, including grasping reflexes, snout and sucking reflexes.
Such dissolution was very clear, Jackson thought, in the processes of dreaming, delirium and insanity, and his long 1894 paper The Factors of Insanities is full of fascinating observations and insights in this regard.
One may see remarkable (and sometimes very poignant) behavioural regressions at a more human level too. I had one patient — a very demented woman of 100, who was incoherent, distracted and agitated much of the time — who, if given a doll, would immediately become focused, sharply attentive and take the doll to her breast as if to nurse it, rocking it in her arms, cuddling it, crooning to it. As long as she was occupied by this mothering behaviour, she was perfectly calm; but the moment she stopped, she became agitated and incoherent again.
The sense that everything is lost with a diagnosis of Alzheimer’s is all too common among neurologists, as well as among patients and their families. This may give rise to a premature sense of impotence and doom, whereas in fact all sorts of neurological functions seem remarkably able to persist relatively intact.
In the early part of the 20th century, neurologists started to pay more attention not just to the primary symptoms of neurological disease, but also to the compensations and adaptations to these. Goldstein, studying brain-damaged soldiers during the First World War, was moved from his original, deficit-based point of view to a more holistic one. There were never, he believed, just deficits; there were always reorganisations, and these he saw as strategies (albeit unconscious) by which the brain-damaged organism sought to survive.
People with Alzheimer’s disease may remain intensely human, very much themselves, and capable of normal emotion and relationships until quite late in their illness. (This preservation of self may, paradoxically, be a source of torment for the patient or their families, who see them so painfully eroded in other ways.)
The relative preservation of the personal allows a great range of supportive and therapeutic activities that have in common that they address or evoke the personal. Religious services, theatre, music and art, gardening, cooking, or other hobbies can anchor patients despite their disintegrations and temporarily restore a focus, an island of identity. Familiar melodies, poems, or stories may still be recognised and responded to despite advanced disease.
As Henry James was dying, with pneumonia and a high fever, he became delirious — and it is said, as I wrote in Hallucinations, that though the master was raving, his style was “pure James” and, indeed, “late James”. The neural embodiment of self, it seems, is extremely robust. Every perception, every action, every thought, every utterance seems to bear the mark of the individual’s experience, of his value system, of all that is peculiar to him. If individual experience and experiential selection so determine the developing brain, we should not perhaps be surprised that individuality, self, is preserved for so long even in the face of diffuse neuronal damage.
Aging, of course, does not necessarily entail neurological illness. Working in old-age homes, where people are admitted with a variety of problems (heart ailments, arthritis, blindness, or sometimes just loneliness and a desire to live in a community), I see numbers of old people who are, so far as I can judge, intellectually and neurologically wholly intact. Indeed, several of my patients are bright and intellectually active centenarians who have retained all their zest for life.
One woman, admitted at the age of 109 with diminishing vision, discharged herself once her cataracts had been taken care of and returned home to an independent life. (“Why should I stay here with all these old people?” she asked.) Even in a chronic hospital, there is a sizable proportion of people who can live out a century or more without significant intellectual decline, and this proportion must be considerably greater in the population at large.
So it is not just the absence of disease or preservation of function that we should be concerned with, but the potential for a continuing development throughout life. Cerebral function is not like cardiac or renal function, which proceeds autonomously, almost mechanically, in a fairly uniform way throughout life. The brain/mind, in contrast, is anything but automatic, for it is always seeking to categorise and recategorise the world, to give meaning to its own experience.
It is the nature of living a real life that experience is not uniform, but ever changing and ever challenging and requiring more and more comprehensive integration. It is not enough for the brain/mind simply to tick over (like the heart); it must adventure and advance throughout life. The very concept of health or wellness requires a special definition in relation to the brain.
A distinction must be made in the aging patient between longevity and vitality. A constitutional sturdiness and good luck may make for a long and healthy life. I think here of five siblings I know, all in their nineties or early hundreds, all looking far younger than their age, and all having the physiques, the sexual drives, the behaviours of much younger people. And yet, human beings may be physically and neurologically healthy, but psychically burned out at a relatively early age.
If the brain is to stay healthy, it must remain active, wondering, playing, exploring and experimenting right to the end. Such activities or dispositions may not show up on tests, but they are of the essence in defining the health of the brain and in allowing its development throughout life. This accords well with what Erik and Joan Erikson devoted a lifetime to studying: universal, age-related stages that seem to appear in all cultures. As the Eriksons advanced through their nineties, they added a further stage to the eight stages they originally described: the stage appropriate to old age — wisdom or integrity.
The achievement of this stage involves the synthesis of a long lifetime’s experience, coupled with the lengthening and enlargement of the individual’s perspectives and a sort of detachment or calm. Such a process is entirely individual. It cannot be prescribed or taught; nor is it directly dependent on education or intelligence or specific talents. “We cannot be taught wisdom,” as Proust remarks, “we have to discover it for ourselves by a journey which no one can undertake for us, an effort which no one can spare us.”
Are such stages purely existential or cultural — the behaviours, the perspectives appropriate to various ages and stages — or do they also have some specific neural basis? We know that learning is possible throughout life, even in the presence of cerebral aging or disease, and we can be sure that other processes, at a much deeper level, are continuing, too — a culmination of the ever wider and deeper generalisations and integrations that have been occurring in the brain/mind throughout life.
In the 19th century, when a powerful mind could still take all of nature for its subject, the great naturalist Alexander von Humboldt, after a lifetime of travel and scientific research, embarked in his mid-seventies on a grand synthetic view of the universe, bringing together everything he had seen and thought into a final work, Cosmos. He was well into its fifth volume when he died at 89.
If we are lucky enough to reach a healthy old age, this sense of wonder can keep us passionate and productive to the end of our lives.
Everything in Its Place: First Loves and Last Tales by Oliver Sacks, is published by Picador on May 2.