Vintage Sacks (5 page)

I hesitated very greatly in regard to the original publication of our patients' “story” and their lives. But they themselves encouraged me, and said to me from the first, “Tell our story—or it will never be known.”

A few of the patients are still alive—we have known each other for twenty-four years now. But those who have died are in some sense not dead—their unclosed charts, their letters, still face me as I write. They still live, for me, in some very personal way. They were not only patients but teachers and friends, and the years I spent with them were the most significant of my life. I want something of their lives, their presence, to be preserved and live for others, as exemplars of human predicament and survival. This is the testimony, the only testimony, of a unique event—but one which may become an allegory for us all.

ROSE R.

Miss R. was born in New York City in 1905, the youngest child of a large, wealthy, and talented family. Her childhood and school days were free of serious illness, and were marked, from their earliest days, by love of merriment, games, and jokes. High-spirited, talented, full of interests and hobbies, sustained by deep family affection and love, and a sure sense of who and what and why she was, Miss R. steered clear of significant neurotic problems or “identity-crises” in her growing-up period.

On leaving school, Miss R. threw herself ardently into a social and peripatetic life. Airplanes, above all, appealed to her eager, volant, and irrepressible spirit; she flew to Pittsburgh and Denver, New Orleans and Chicago, and twice to the California of Hearst and Hollywood (no mean feat in the planes of those days). She went to innumerable parties and shows, was toasted and fêted, and rolled home drunk at night. And between parties and flights she dashed off sketches of the bridges and water-fronts with which New York abounded. Between 1922 and 1926, Miss R. lived in the blaze of her own vitality, and lived more than most other people in the whole of their lives. And this was as well, for at the age of twenty-one she was suddenly struck down by a virulent form of
encephalitis lethargica
—one of its last victims before the epidemic vanished. Nineteen twenty-six, then, was the last year in which Miss R. really
lived.

The night of the sleeping sickness, and the days which followed it, can be reconstructed in great detail from Miss R.'s relatives, and Miss R. herself. The acute phase announced itself (as sometimes happened: compare Maria G.) by nightmares of a grotesque and terrifying and premonitory nature. Miss R. had a series of dreams about one central theme: she dreamed she was imprisoned in an inaccessible castle, but the castle had the form and shape of herself; she dreamed of enchantments, bewitchments, entrancements; she dreamed that she had become a living, sentient statue of stone; she dreamed that the world had come to a stop; she dreamed that she had fallen into a sleep so deep that nothing could wake her; she dreamed of a death which was different from death. Her family had difficulty waking her the next morning, and when she awoke there was intense consternation: “Rose,” they cried, “wake up! What's the matter? Your expression, your position . . . You're so still and so strange.” Miss R. could not answer, but turned her eyes to the wardrobe mirror, and there she saw that her dreams had come true. The local doctor was brisk and unhelpful: “Catatonia,” he said;
“Flexibilitas cerea.
What can you expect with the life she's been leading? She's broken her heart over one of these bums. Keep her quiet and feed her—she'll be fine in a week.”

But Miss R. was not to recover for a week, or a year, or forty-three years. She recovered the ability to speak in short sentences, or to make sudden movements before she froze up again. She showed, increasingly, a forced retraction of her neck and her eyes—a state of almost continuous oculogyric crisis, broken only by sleep, meals, and occasional “releases.” She was alert, and seemed to notice what went on around her; she lost none of her affection for her numerous family—and they lost none of their affection for her; but she seemed absorbed and preoccupied in some unimaginable state. For the most part, she showed no sign of distress, and no sign of anything save intense
concentration:
“She looked,” said one of her sisters, “as if she were trying her hardest to remember something—or, maybe, doing her damnedest to forget something. Whatever it was, it took all her attention.” In her years at home, and subsequently in hospital, her family did their utmost to penetrate this absorption, to learn what was going on with their beloved “kid” sister. With them—and, much later, with me—Miss R. was exceedingly candid, but whatever she said seemed cryptic and gnomic, and yet at the same time disquietingly clear.
⁸

When there was only this state, and no other problems, Miss R.'s family could keep her at home: she was no trouble, they loved her, she was simply—elsewhere (or nowhere). But three or four years after her trance-state had started, she started to become rigid on the left side of her body, to lose her balance when walking, and to develop other signs of Parkinsonism. Gradually these symptoms grew worse and worse, until full-time nursing became a necessity. Her siblings left home, and her parents were aging, and it was increasingly difficult to keep her at home. Finally, in 1935, she was admitted to Mount Carmel.

Her state changed little after the age of thirty, and when I first saw her in 1966, my findings coincided with the original notes from her admission. Indeed, the old staff-nurse on her ward, who had known her throughout, said: “It's uncanny, that woman hasn't aged a day in the thirty years I've known her. The rest of us get older—but Rosie's the same.” It was true: Miss R. at sixty-one looked thirty years younger; she had raven-black hair, and her face was unlined, as if she had been magically preserved by her trance or her stupor.

She sat upright and motionless in her wheelchair, with little or no spontaneous movement for hours on end. There was no spontaneous blinking, and her eyes stared straight ahead, seemingly indifferent to her environment but completely absorbed. Her gaze, when requested to look in different directions, was full, save for complete inability to converge the eyes. Fixation of gaze lacked smooth and subtle modulation, and was accomplished by sudden, gross movements which seemed to cost her considerable effort. Her face was completely masked and expressionless. The tongue could not be protruded beyond the lip-margins, and its movements, on request, were exceedingly slow and small. Her voice was virtually inaudible, though Miss R. could whisper quite well with considerable effort. Drooling was profuse, saturating a cloth bib within an hour, and the entire skin was oily, seborrhoeic, and sweating intensely. Akinesia was global, although rigidity and dystonia were strikingly unilateral in distribution. There was intense axial rigidity, no movement of the neck or trunk muscles being possible. There was equally intense rigidity in the left arm, and a very severe dystonic contracture of the left hand. No voluntary movement of this limb was possible. The right arm was much less rigid, but showed great akinesia, all movements being minimal, and decaying to zero after two or three repetitions. Both legs were hypertonic, the left much more so. The left foot was bent inward in dystonic inversion. Miss R. could not rise to her feet unaided, but when assisted to do so could maintain her balance and take a few small, shuffling, precarious steps, although the tendency to backward-falling and pulsion was very great.

She was in a state of near-continuous oculogyric crisis, although this varied a good deal in severity. When it became more severe, her Parkinsonian “background” was increased in intensity, and an intermittent coarse tremor appeared in her right arm. Prominent tremor of the head, lips, and tongue also became evident at these times, and rhythmic movement of buccinators and corrugators. Her breathing would become somewhat stertorous at such times, and would be accompanied by a guttural phonation reminiscent of a pig grunting. Severe crises would always be accompanied by tachycardia and hypertension. Her neck would be thrown back in an intense and sometimes agonizing opisthotonic posture. Her eyes would generally stare directly ahead, and could not be moved by voluntary effort: in the severest crises they were forced upward and fixed on the ceiling.

Miss R.'s capacity to speak or move, minimal at the best of times, would disappear almost entirely during her severer crises, although in her greatest extremity she would sometimes call out, in a strange high-pitched voice, perseverative and palilalic, utterly unlike her husky “normal” whisper: “Doctor, doctor, doctor, doctor . . . help me, help, help, h'lp, h'lp. . . . I am in terrible pain, I'm so frightened, so frightened, so frightened. . . . I'm going to die, I know it, I know it, I know it, I know it. . . .” And at other times, if nobody was near, she would whimper softly to herself, like some small animal caught in a trap. The nature of Miss R.'s pain during her crises was only elucidated later, when speech had become easy: some of it was a local pain associated with extreme opisthotonos, but a large component seemed to be central—diffuse, unlocalizable, of sudden onset and offset, and inseparably coalesced with feelings of dread and threat, in the severest crises a true
angor animi.
During exceptionally severe attacks, Miss R.'s face would become flushed, her eyes reddened and protruding, and she would repeat, “It'll kill me, it'll kill me, it'll kill me . . .” hundreds of times in succession.
⁹

Miss R's state scarcely changed between 1966 and 1969, and when L-DOPA became available I was in two minds about using it. She was, it was true, intensely disabled, and had been virtually helpless for over forty years. It was her
strangeness
above all which made me hesitate and wonder—fearing what might happen if I gave her L-DOPA. I had never seen a patient whose regard was so turned away from the world, and so immured in a private, inaccessible world of her own.

I kept thinking of something Joyce wrote about his mad daughter: “. . . fervently as I desire her cure, I ask myself what then will happen when and if she finally withdraws her regard from the lightning-lit reverie of her clairvoyance and turns it upon that battered cabman's face, the world. . . .”

Course on L-DOPA

But I started her on L-DOPA, despite my misgivings, on June 18, 1969. The following is an extract from my diary.

25 June.
The first therapeutic responses have already occurred, even though the dosage has only been raised to 1.5 gm. a day. Miss R. has experienced two entire days unprecedentedly free of oculogyric crises, and her eyes, so still and preoccupied before, are brighter and more mobile and attentive to her surroundings.

1 July.
Very real improvements are evident by this date: Miss R. is able to walk unaided down the passage, shows a distinct reduction of rigidity in the left arm and elsewhere, and has become able to speak at a normal conversational volume. Her mood is cheerful, and she has had no oculogyric crises for three days. In view of this propitious response, and the absence of any adverse effects, I am increasing the dosage of L-DOPA to 4 gm. daily.

6 July. Now receiving 4 gm. L-DOPA. Miss R. has continued to improve in almost every way. When I saw her at lunchtime, she was delighted with everything: “Dr. Sacks!” she called out, “I walked to and from the new building today” (this is a distance of about six hundred yards). “It's fabulous, it's gorgeous!” Miss R. has now been free from oculogyric crises for eight days, and has shown no akathisia or undue excitement. I too feel delighted at her progress, but for some reason am conscious of obscure forebodings.

7 July.
Today Miss R. has shown her first signs of unstable and abrupt responses to L-DOPA. Seeing her 3½ hours after her early-morning dose, I was shocked to find her very “down”—hypophonic, somewhat depressed, rigid and akinetic, with extremely small pupils and profuse salivation. Fifteen minutes after receiving her medication she was “up” again—her voice and walking fully restored, cheerful, smiling, talkative, her eyes alert and shining, and her pupils somewhat dilated. I was further disquieted by observing an occasional impulsion to run, although this was easily checked by her.

8 July.
Following an insomniac night (“I didn't feel in the least sleepy: thoughts just kept rushing through my head”), Miss R. is extremely active, cheerful, and affectionate. She seems to be very busy, constantly flying from one place to another, and all her thoughts too are concerned with movement; “Dr. Sacks,” she exclaimed breathlessly, “I feel great today. I feel I want to fly. I love you, Dr. Sacks, I love you, I love you. You know, you're the kindest doctor in the world. . . . You know I always liked to travel around: I used to fly to Pittsburgh, Chicago, Miami, California. . . .” etc. Her skin is warm and flushed, her pupils are again very widely dilated, and her eyes constantly glancing to and fro. Her energy seems limitless and untiring, although I get the impression of exhaustion somewhere beneath the pressured surface. An entirely new symptom has also appeared today, a sudden quick movement of the right hand to the chin, which is repeated two or three times an hour. When I questioned Miss R. about this she said: “It's new, it's odd, it's strange, I never did it before. God knows why I do it. I just suddenly get an
urge,
like you suddenly got to sneeze or scratch yourself.” Fearing the onset of akathisia or excessive emotional excitement, I have reduced the dosage of L-DOPA to 3 gm. daily.

9 July.
Today Miss R.'s energy and excitement are unabated, but her mood has veered from elation to anxiety. She is impatient, touchy, and extremely demanding. She became much agitated in the middle of the day, asserting that seven dresses had been stolen from her closet, and that her purse had been stolen. She entertained dark suspicions of various fellow patients: no doubt they had been plotting this for weeks before. Later in the day, she discovered that her dresses were in fact in her closet in their usual position. Her paranoid recriminations instantly vanished: “Wow!” she said, “I must have imagined it all. I guess I better take myself in hand.”

14 July.
Following the excitements and changing moods of July 9, Miss R.'s state has become less pressured and hyperactive. She has been able to sleep, and has lost the ticlike “wiping” movements of her right hand. Unfortunately, after a two-week remission, her old enemy has reemerged, and she has experienced two severe oculogyric crises. I observed in these not only the usual staring, but a more bizarre symptom—captivation or enthrallment of gaze: in one of these crises she had been forced to stare at one of her fellow patients, and had felt her eyes “drawn” this way and that, following the movements of this patient around the ward. “It was uncanny,” Miss R. said later. “My eyes were spellbound. I felt like I was bewitched or something, like a rabbit with a snake.” During the periods of “bewitchment” or fascination, Miss R. had the feeling that her “thoughts had stopped,” and that she could only think of one thing, the object of her gaze. If, on the other hand, her attention was distracted, the quality of thinking would suddenly change, the motionless fascination would be broken up, and she would experience instead “an absolute torrent of thoughts,” rushing through her mind: these thoughts did not seem to be “her” thoughts, they were not what she wanted to think, they were “peculiar thoughts” which appeared “by themselves.” Miss R. could not or would not specify the nature of these intrusive thoughts, but she was greatly frightened by the whole business: “These crises are different from the ones I used to get,” she said. “They are worse. They are completely mad!”
¹⁰
25 July.
Miss R. has had an astonishing ten days, and has shown phenomena I never thought possible. Her mood has been joyous and elated, and very salacious. Her social behavior has remained impeccable, but she has developed an insatiable urge to sing songs and tell jokes, and has made very full use of our portable tape recorder. In the past few days, she has recorded innumerable songs of an astonishing lewdness, and reams of “light” verse all dating from the twenties. She is also full of anecdotes and allusions to “current” figures—to figures who were current in the mid-1920s. We have been forced to do some archival research, looking at old newspaper files in the New York Library. We have found that almost all of Miss R.'s allusions date to 1926, her last year of real life before her illness closed round her. Her memory is uncanny, considering she is speaking of so long ago. Miss R. wants the tape recorder, and nobody around; she stays in her room, alone with the tape recorder; she is looking at everyone as if they didn't exist. She is completely engrossed in her memories of the twenties, and is doing her best to not notice anything later. I suppose one calls this “forced reminiscence,” or incontinent nostalgia.
¹¹
But I also have the feeling that she feels her “past” as present, and that, perhaps it has never felt “past” for her. Is it possible that Miss R. has never, in fact, moved on from the “past”?
Could she still be “in” 1926 forty-three
years later? Is 1926 “now”?
¹²