by Oliver Sacks
He had his job: checking the windows and doors to make sure they were securely locked at night, inspecting the laundry and boiler room to make sure all was functioning smoothly. The sisters who ran the home, though perceiving his confusion and delusion, respected and even reinforced the identity of this somewhat demented resident, who, they felt, might fall apart if it were taken away. So they encouraged him in his janitorial role, giving him keys to certain closets and encouraging him to lock up at night before he retired. He wore a bunch of keys jangling at his waist—the insignia of his office, his official identity. He would check the kitchen to make sure all of the gas rings and stoves were turned off and no perishable food had been left unrefrigerated. And though he slowly became more and more demented over the years, he seemed to be organized and held together in a remarkable way by his role, the varied tasks of checking, cleaning, and maintenance that he performed throughout the day. When Mr. Q. died of a sudden heart attack, he did so without perhaps ever realizing that he had been anything but a janitor with a lifetime of loyal work behind him.
Should we have told Mr. Q. that he was no longer a janitor but a declining and demented patient in a nursing home? Should we have taken away his accustomed and well-rehearsed identity and replaced it with a “reality” that, though real to us, would have been meaningless to him? It seemed not only pointless but cruel to do so—and might well have hastened his decline.
The Aging Brain
Having worked as a neurologist in old-age homes and chronic hospitals for almost fifty years, I have seen thousands of older patients with Alzheimer’s disease or other dementias, and what strikes me most is the immense diversity of the clinical presentation, despite the fact that most of these patients suffer from disease processes that are pathologically similar. One sees a kaleidoscopic array of symptoms and dysfunctions, never exactly the same in any two people. The neurological dysfunctions interact with all that is particular and unique in an individual—their preexisting strengths and weaknesses, their intellectual powers, their skills, their life experience, their character, their habitual styles, as well as their particular life situations.
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 tumor; it is only later that the generalized nature of the disease becomes evident (hence the frequent failure to diagnose Alzheimer’s at the start). The early symptoms, whether they appear singly or in clusters, are usually subtle. There may be subtle language or memory problems, such as difficulty recalling proper names; subtle perceptual problems, such as momentary illusions or misperceptions; or subtle intellectual problems, such as difficulties getting jokes or following arguments. But in general it is the most recently evolved functions, the complex associational functions, that are the first to be affected.
In these very early stages, the dysfunctions tend to be fugitive and momentary (as is true of the electroencephalographic changes at this time—sometimes one must look through an hour of EEG recordings to find a second of abnormality). But soon there are grosser disturbances of cognition, memory, behavior, judgment, and disorientation in space and time, all finally coalescing as profound global dementia. As the disease advances, sensory and motor disturbances often appear, along with spasticity and rigidity, myoclonus, sometimes seizures, and sometimes parkinsonism. It may bring distressing personality changes and even violent behavior in some people. Finally, there may be almost no responses above a brainstem reflex level. Every possible cortical disorder (and a good many subcortical ones) 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 insofar 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.*1
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GIVEN THE MULTIFORMITY of symptoms in dementia, one can see why standardized tests, although useful for screening patients and for delineating populations for genetic studies or drug trials, give little idea of what the disease is actually like, the ways in which the beleaguered patient may adapt and react, and the ways in which such people can, on occasion, be helped—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. These brief visual agnosias rapidly worsened: 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 organize 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 humor 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.
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IT IS SOMETIMES SAID that people with Alzheimer’s do not realize that they are impaired, that insight is lost from the start. Although this may sometimes be so (if, for example, there is a frontal-lobe type of onset), 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 sixty-nine. But 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 (although one has to beware of this kind of formulation) to have regressed intellectually, so that they are once again like children, restricted to a narrative mode of thought. Kurt Goldstein, a neurologist and psychiatrist, would say of such patients that they had lost not only their abstract capacities but also their abstract “attitude”—that they were now in a lower, more concrete form of consciousness or being.
For Hughlings Jackson, the great English neurologist, there were never just deficits with neurological damage, but “hyperphysiological” or “positive” symptoms, as he called them, “releases” or exaggerations of normally constrained or inhibited neural functions. He spoke of “dissolution,” which was, for him, characterized by regression or reversion to more archaic levels of neural function—the reverse of evolution.*2
Although J
ackson’s notion of dissolution in the nervous system as evolution in reverse can hardly be maintained now in so simplistic a fashion, one does see some remarkable behavioral regressions or releases in a diffuse cortical disease like Alzheimer’s. I have often seen patients with advanced dementia who show picking, hunting, and brushing—a whole range of primitive grooming behaviors that are not seen in normal human development but are suggestive, perhaps, of a phylogenetic reversion to a prehuman, primate level. In the final stages of dementia, where no organized behaviors of any sort remain, one may see reflexes that are normally only seen in infancy, including grasping reflexes, snout and sucking reflexes, and Moro reflexes.
One may see remarkable (and sometimes very poignant) behavioral regressions at a more human level, too. I had one patient—a very demented woman of one hundred 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 behavior, she was perfectly calm; but the moment she stopped, she became agitated and incoherent again.
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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 (including many that subserve the self) seem remarkably able to resist even widespread neuronal dysfunction.
In the early part of the twentieth 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. Kurt Goldstein, studying brain-damaged soldiers during World War I, was moved from his original, deficit-based point of view to a more holistic, organismal one. There were never, he believed, just deficits or releases; there were always reorganizations, and these he saw as strategies (albeit unconscious and almost automatic) by which the brain-damaged organism sought to survive, although perhaps in a more rigid and impoverished way.
Ivy Mackenzie, a Scottish physician working with postencephalitic patients, described the remote effects—“subversions,” compensations, and adaptations—that follow the primary insult. In the study of these, he wrote, we see “an organized chaos,” ways in which the organism, the brain, comes to terms with itself, reestablishes itself, at other levels. “The physician,” he wrote, “is concerned, unlike the naturalist, with a single organism, the human subject, striving to preserve its identity in adverse circumstances.”
This theme, the preservation of identity, is well brought out by Donna Cohen and Carl Eisdorfer in their fine book The Loss of Self, which is based on painstaking studies of a number of people with Alzheimer’s. The title of their book is perhaps misleading, for it is not loss (at least until very late) but surprising preservations and transformations that we see in Alzheimer’s, and this, indeed, is what Cohen and Eisdorfer show.*3
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, theater, 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 recognized and responded to despite advanced disease—a response that may be richly associative and bring back, for a while, some of the patient’s memories and feelings and their former powers and worlds. This can bring at least a temporary “awakening” and fullness of life to patients who may otherwise be dismissed or ignored, left in states of bewilderment and vacancy, prone at any moment to losing their bearings or to catastrophic reactions (as Goldstein called them) of unimaginable confusion and panic.
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. In Gerald Edelman’s theory of neuronal group selection (as in Esther Thelen’s work on the development of cognition and action in children), we find a rich account of how neuronal connectivity may be determined by, literally shaped by, the individual’s experience, thoughts, and actions no less than by all that is hardwired and biologically given. 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.
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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, all their interests and faculties, into their eleventh decade. 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, at every level from the perceptual to the philosophical, to categorize and recategorize the world, to comprehend and 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, maintaining uniform function (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 behaviors 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 functional brain imaging or, for that matter, on neuropsychological tests, but they are of the essence in defining the health of the brain and in allowing its development throughout life. This is clear in Edelman’s neurobiological model, where the brain/mind is conceived as incessantly active, categorizing and recategorizing its activities throughout life, constructing interpretations and meanings at ever higher levels.
Such a neurobiological model 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 themselves advanced through their nine
ties, they added a further stage to the eight stages they originally described. This last stage is well recognized and respected in many cultures (although sometimes forgotten in our own). This is the stage appropriate to old age; and the solution or strategy to be achieved at this stage is what the Eriksons call wisdom or integrity.
The achievement of this stage involves the integration of vast amounts of information, 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 behaviors, 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 generalizations and integrations that have been occurring in the brain/mind throughout life.