An Anthropologist on Mars (1995) (10 page)

A hospital for the chronically ill, where patients and staff live together for years, is a little like a village or a small town: everybody gets to meet, to know, everybody else. I often saw Greg in the corridors, being wheeled to different programs or out to the patio, in his wheelchair, with the same odd, blind yet searching look on his face. And he gradually got to know me, at least sufficiently to know my name, to ask each time we met, “How’re you doing, Dr. Sacks? When’s the next book coming out?” (a question that rather distressed me in the seemingly endless eleven-year interim between the publication of Awakenings and A Leg to Stand On).

Names, then, he might learn, with frequent contact, and in relation to them he would recollect a few details about each new person. Thus he came to know Connie Tomaino, the music therapist—he would recognize her voice, her footfalls, immediately—but he could never remember where or how he had met her. One day Greg began talking about “another Connie”, a girl called Connie whom he’d known in high school.

This other Connie, he told us, was also, remarkably, very musical—“How come all you Connies are so musical?” he teased. The other Connie would conduct music groups, he said, would give out song sheets, play the piano-accordion at singsongs at school. At this point, it started to dawn on us that this “other” Connie was in fact Connie herself, and this was clinched when he added, “You know, she played the trumpet, too.” (Connie Tomaino is a professional trumpet player.) This sort of thing often happened with Greg when he put things into the wrong context or failed to connect them with the present.

His sense of there being two Connies, his segmenting Connie into two, was characteristic of the bewilderments he sometimes found himself in, his need to hypothesize additional figures because he could not retain or conceive of an identity in time. With consistent repetition Greg might learn a few facts, and these would be retained. But the facts were isolated, denuded of context. A person, a voice, a place, would slowly become “familiar”, but he remained unable to remember where he had met the person, heard the voice, seen the place. Specifically, it was context-bound (or “episodic”) memory that was so grossly disturbed in Greg—as is the case with most amnesiacs.

Other sorts of memory were intact,—thus Greg had no difficulty remembering or applying geometric truths that he had learned in school. He saw instantly, for example, that the hypotenuse of a triangle was shorter than the sum of the two sides—thus his semantic memory, so-called, was fairly intact. Again, he not only retained his power to play the guitar, but actually enlarged his musical repertoire, learning new techniques and fingering with Connie; he also learned to type while at Williamsbridge—so his procedural memory was also unimpaired.

Finally, there seemed to be some sort of slow habituation or familiarization—so that he became able, within three months, to find his way about the hospital, to go to the coffee shop, the cinema, the auditorium, the patio, his favorite places. This sort of learning was exceedingly slow, but once it had been achieved, it was tenaciously retained.

It was clear that Greg’s tumor had caused damage that was complex and curious. First, it had compressed or destroyed structures of the inner, or medial, side of both the temporal lobes—in particular, the hippocampus and its adjacent cortex, areas crucial for the capacity to form new memories. With such damage, the ability to acquire information about new facts and events is devastated—there ceases to be any explicit or conscious remembrance of these. But while Greg was so often unable to recall events or encounters or facts to consciousness, he might nonetheless have an unconscious or implicit memory of them, a memory expressed in performance or behavior. Such implicit ability to remember allowed him to become slowly familiar with the physical layout and routines of the hospital and with some of the staff, and to make judgments on whether certain persons (or situations) were pleasant or unpleasant.
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36. That implicit memory (especially if emotionally charged) may exist in amnesiacs was shown, somewhat cruelly, in 1911, by Edouard Claparède, who, when shaking hands with such a patient whom he was presenting to his students, stuck a pin in his hand. Although the patient had no explicit memory of this, he refused, thereafter, to shake hands with him.

While explicit learning requires the integrity of the medial temporal lobe systems, implicit learning may employ more primitive and diffuse paths, as do the simple processes of conditioning and habituation. Explicit learning, however, involves the construction of complex percepts—syntheses of representations from every part of the cerebral cortex—brought together into a contextual unity, or “scene.” Such syntheses can be held in mind for only a minute or two—the limit of short-term memory—and after this will be lost unless they can be shunted into long-term memory. Thus higher-order memorization is a multistage process, involving the transfer of perceptions, or perceptual syntheses, from short-term to long-term memory. It is just such a transfer that fails to occur in people with temporal lobe damage. Thus Greg can repeat a complicated sentence with complete accuracy and understanding the moment he hears it, but within three minutes, or sooner if he is distracted for an instant, he will retain not a trace of it, or any idea of its sense, or any memory that it ever existed.

Larry Squire, a neuropsychologist at the University of California, San Diego, who has been a central figure in elucidating this shunting function of the temporal lobe memory system, speaks of the brevity, the precariousness, of short-term memory in us all; all of us, on occasion, suddenly lose a perception or an image or a thought we had vividly in mind (“Damn it”, we may say, “I’ve forgotten what I wanted to say!”), but only in amnesiacs is this precariousness realized to the full.

Yet while Greg, no longer capable of transforming his perceptions or immediate memories into permanent ones, remains stuck in the sixties, when his ability to learn new information broke down, he has nevertheless adapted somehow and absorbed some of his surroundings, albeit very slowly and incompletely.
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37. A.R. Luria, in The Neuropsychology of Memory, remarks that all his amnesiac patients, if hospitalized for any length of time, acquired “a sense of familiarity” with their surroundings.

Some amnesiacs (like Jimmie, the patient with Korsakov’s syndrome whom I described in “The Lost Mariner”) have brain damage largely confined to the memory systems of the diencephalon and medial temporal lobe; others (like Mr. Thompson, described in “A Matter of Identity”) are not only amnesiac but have frontal lobe syndromes, too; yet others—like Greg, with immense tumors—tend to have a third area of damage as well, deep below the cerebral cortex, in the fore-brain, or diencephalon. In Greg, this widespread damage had created a very complicated clinical picture, with sometimes overlapping or even contradictory symptoms and syndromes.

Thus though his amnesia was chiefly caused by damage to the temporal lobe systems, damage to the diencephalon and frontal lobes also played a part. Similarly there were multiple origins for his blandness and indifference, for which damage to the frontal lobes, diencephalon, and pituitary gland was in varying degrees responsible. In fact, Greg’s tumor first caused damage to his pituitary gland; this was responsible not only for his gain in weight and loss of body hair but also for undermining his hormonally driven aggressiveness and assertiveness, and hence for his abnormal submissiveness and placidity.

The diencephalon is especially a regulator of basic functions—of sleep, of appetite, of libido. And all of these were at a low ebb with Greg—he had (or expressed) no sexual interest; he did not think of eating, or express any desire to eat, unless food was brought to him. He seemed to exist only in the present, only in response to the immediacy of stimuli around him. If he was not stimulated, he fell into a sort of daze.

Left alone, Greg would spend hours in the ward without spontaneous activity. This inert state was at first described by the nurses as “brooding”; it had been seen in the temple as “meditating”; my own feeling was that it was a profoundly pathological mental “idling”, almost devoid of mental content or affect. It was difficult to give a name to this state, so different from alert, attentive wakefulness, but also, clearly, quite different from sleep—it had a blankness resembling no normal state. It reminded me somewhat of the vacant states I had seen with some of my postencephalitic patients and, as with them, went with profound damage to the diencephalon. As soon as one talked to him, or if he was stimulated by sounds (especially music) near him, he would “come to”, “awaken”, in an astonishing way.

Once Greg was “awakened”, once his cortex came to life, one saw that his animation itself had a strange quality—an uninhibited and quirky quality of the sort one tends to see when the orbital portions of the frontal lobes (that is, the portions adjacent to the eyes) are damaged, a so-called orbito-frontal syndrome. The frontal lobes are the most complex part of the brain, concerned not with the “lower” functions of movement and sensation, but the highest ones of integrating all judgment and behavior, all imagination and emotion, into that unique identity that we like to speak of as “personality” or “self.” Damage to other parts of the brain may produce specific disturbances of sensation or movement, of language, or of specific perceptual, cognitive, or memory functions. Damage to the frontal lobes, in contrast, does not affect these, but produces a subtler and profounder disturbance of identity.

And it was this—rather than his blindness, or his weakness, or his disorientation, or his amnesia—that so horrified his parents when they finally saw Greg in 1975. It was not just that he was damaged, but that he was changed beyond recognition, had been “dispossessed”, in his father’s words, by a sort of simulacrum, or changeling, which had Greg’s voice and manner and humor and intelligence but not his “spirit” or “realness” or “depth”—a changeling whose wisecracking and levity formed a shocking counterpoint to the fearful gravity of what had happened.

This sort of wisecracking, indeed, is quite characteristic of such orbito-frontal syndromes—and is so striking that it has been given a name unto itself: witzelsucht, or “joking disease.” Some restraint, some caution, some inhibition, is destroyed, and patients with such syndromes tend to react immediately and incontinently to everything around them and everything within them—to virtually every object, every person, every sensation, every word, every thought, every emotion, every nuance and tone.

There is an overwhelming tendency, in such states, to wordplay and puns. Once when I was in Greg’s room another patient walked past. “That’s Bernie”, I said. “Bernie the Hernie”, quipped Greg. Another day when I visited him, he was in the dining room, awaiting lunch. When a nurse announced, “Lunch is here”, he immediately responded, “It’s time for cheer”; when she said, “Shall I take the skin off your chicken?” he instantly responded, “Yeah, why don’t you slip me some skin.” “Oh, you want the skin?” she asked, puzzled.

“Nah”, he replied, “it’s just a saying.” He was, in a sense, preternaturally sensitive—but it was a sensitivity that was passive, without selectivity or focus. There is no differentiation in such a sensitivity—the grand, the trivial, the sublime, the ridiculous, are all mixed up and treated as equal.
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38. Luria provides immensely detailed, at times almost novelistic, descriptions of frontal lobe syndromes—in Human Brain and Psychological Processes—and sees this “equalization” as the heart of such syndromes.

There may be a childlike spontaneity and transparency about such patients in their immediate and unpremeditated (and often playful) reactions. And yet there is something ultimately disquieting, and bizarre, because the reacting mind (which may still be highly intelligent and inventive) loses its coherence, its inwardness, its autonomy, its “self”, and becomes the slave of every passing sensation. The French neurologist François Lhermitte speaks of an “environmental dependency syndrome” in such patients, a lack of psychological distance between them and their environment. So it was with Greg: he seized his environment, he was seized by it, he could not distinguish himself from it.
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39. A similar indiscriminate reactivity is sometimes seen in people with Tourette’s syndrome—sometimes in the automatic form of echoing others’ words or actions, sometimes in the more complex forms of mimicry, parodying or impersonating others’ behavior, or in incontinent verbal associations (rhymings, punnings, clangings).

Dreaming and waking, for us, are usually distinct—dreaming is enclosed in sleep and enjoys a special license because it is cut off from external perception and action; while waking perception is constrained by reality.
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40. Rodolfo Llinâs and his colleagues at New York University, comparing the electrophysiological properties of the brain in waking and dreaming, postulate a single fundamental mechanism for both—a ceaseless inner talking between cerebral cortex and thalamus, a ceaseless interplay of image and feeling, irrespective of whether there is sensory input or not. When there is sensory input, this interplay integrates it to generate waking consciousness, but in the absence of sensory input it continues to generate brain states, those brain states we call fantasy, hallucination, or dreams. Thus waking consciousness is dreaming—but dreaming constrained by external reality.

But in Greg the boundary between waking and sleep seemed to break down, and what emerged was a sort of waking or public dream, in which dreamlike fancies and associations and symbols would proliferate and weave themselves into the waking perceptions of the mind.
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41. Dreamlike or oneiric states have been described, by Luria and others, with lesions of the thalamus and diencephalon. J.-J. Moreau, in a famous early study, Hashish and Mental Illness (1845), described both madness and hashish trances as “waking dreams.” A particularly striking form of waking dream may be seen with the severer forms of Tourette’s syndrome, where the external and the internal, the perceptual and the instinctual, burst forth in a sort of public phantasmagoria or dream.