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
28 July. Miss R. sought me out this morning—the first time she had done so in almost two weeks. Her face has lost its jubilant look, and she looks anxious and shadowed and slightly bewildered: “Things can’t last,” she said. “Something awful is coming. God knows what it is, but it’s bad as they come.” I tried to find out more, but Miss R. shook her head: “It’s just a feeling, I can’t tell you more. . . .”
1 August. A few hours after stating her prediction, Miss R. ran straight into a barrage of difficulties. Suddenly she was ticcing, jammed, and blocked; the beautiful smooth flow which had borne her along seemed to break up, and dam, and crash back on itself. Her walking and talking are gravely affected. She is
impelled to rush forward for five or six steps, and then suddenly freezes or jams without warning; she continually gets more excited and frustrated, and with increasing excitement the jamming grows worse. If she can moderate her excitement or her impulsion to run, she can still walk the corridor without freezing or jamming. Analogous problems are affecting her speech: she can only speak softly, if she is to speak at all, for with increased vocal impetus she stutters and stops. I have the feeling that Miss R.’s “motor space” is becoming confined, so that she rebounds internally if she moves with too much speed or force. Reducing her L-DOPA to 3 gm. a day reduced the dangerous hurry and block, but led to an intensely severe oculogyric crisis—the worst Miss R. has had since starting L-DOPA. Moreover, her “wiping” tic—which reappeared on the 28th—has grown more severe and more complex with each passing hour. From a harmless feather-light brush of the chin, the movement has become a deep circular gouging, her right index finger scratching incessantly in tight little circles, abrading the skin and making it bleed. Miss R. has been quite unable to stop this compulsion directly, but she can override it by thrusting her tic-hand deep in her pocket and clutching its lining with all of her force. The moment she forgets to do this, the hand flies up and scratches her face.
AUGUST 1969
During the first week of August,13 Miss R. continued to have oculogyric crises every day of extreme severity, during which she would be intensely rigid and opisthotonic, anguished, whimpering, and bathed in sweat. Her tics of the right hand became almost too fast for the eye to follow, their rate having increased to almost three hundred per minute (an estimate confirmed by a slow-motion film). On August 6, Miss R. showed very obvious palilalia, repeating entire sentences and strings of words again and again: “I’m going round like a record,” she said, “which gets stuck in the groove. . . .” During the second week of August, her tics became more complex, and were conflated with defensive maneuvers, counter-tics, and elaborate rituals. Thus Miss R. would clutch someone’s hand, release her grip, touch something nearby, put her hand in her pocket, withdraw it, slap the pocket three times, put it back in the pocket, wipe her chin five times, clutch someone’s hand . . . and move again and again through this stereotyped sequence.
The evening of August 15 provided the only pleasant interlude in a month otherwise full of disability and suffering. On this evening, quite unexpectedly, Miss R. emerged from her crises and blocking and ticcing, and had a brief return of joyous salacity, accompanied with free-flowing singing and movement. For an hour this evening, she improvised a variety of coprolalic limericks to the tune of “The Sheikh of Araby,” accompanying herself on the piano with her uncontractured right hand.
Later this week, her motor and vocal block became absolute. She would suddenly call out to Miss Kohl: “Margie, I . . . Margie, I want . . . Margie! . . . ,” completely unable to proceed beyond the first word or two of what she so desperately wanted to say. When she tried to write, similarly, her hand (and thoughts) suddenly stopped after a couple of words. If one asked her to try and say what she wanted, softly and slowly, her face would go blank, and her eyes would shift in a tantalized manner, indicating, perhaps, her frantic inner search for the dislimning thought. Walking became impossible at this time, for Miss R. would find her feet completely stuck to the ground, but the impulse to move would throw her flat on her face. During the last ten days of August, Miss R. seemed to be totally blocked in all spheres of activity; everything about her showed an extremity of tension, which was entirely prevented from finding any outlet. Her face at this time was continually clenched in a horrified, tortured, and anguished expression. Her prediction of a month earlier was completely fulfilled: something awful had come, and it was as bad as they came.
1969–72
Miss R.’s reactions to L-DOPA since the summer of 1969 have been almost nonexistent compared with her dramatic initial reaction. She has been placed on L-DOPA five further times, each with an increase of dose by degrees to about 3.0 gm. per day. Each time the L-DOPA has procured some reduction in her rigidity, oculogyria, and general entrancement, but less and less on each succeeding occasion. It has never called forth anything resembling the amazing mobility and mood change of July 1969, and in particular has never recalled the extraordinary sense of 1926-ness which she had at that time. When Miss R. has been on L-DOPA for several weeks its advantages invariably become overweighed by its disadvantages, and she returns to a state of intense “block,” crises, and tic-like impulsions. The form of her tics has varied a good deal on different occasions: in one of her periods on L-DOPA her crises were always accompanied by a palilalic verbigeration of the word “Honeybunch!” which she would repeat twenty or thirty times a minute for the entire day.
However deep and strange her pathological state, Miss R. can invariably be “awakened” for a few seconds or minutes by external stimuli, although she is obviously quite unable to generate any such stimuli or calls-to-action for herself. If Miss A.—a fellow patient with dipsomania—drinks more than twenty times an hour at the water fountain, Miss R. cries, “Get away from that fountain, Margaret, or I’ll clobber you!” or “Stop sucking that spout, Margaret, we all know what you really want to suck!” Whenever she hears my name being paged she yells out, “Dr. Sacks! Dr. Sacks!! They’re after you again!” and continues to yell this until I have answered the page.
Miss R. is at her best when she is visited—as she frequently is—by any of her devoted family who fly in from all over the country to see her. At such time she is all agog with excitement, her blank masked face cracks into a smile, and she shows a great hunger for family gossip, though no interest at all in political events or other current “news”; at such times she is able to say a certain amount quite intelligibly, and in particular shows her fondness for jokes and mildly salacious indiscretions. Seeing Miss R. at this time one realizes what a “normal” and charming and alive personality is imprisoned or suspended by her ridiculous disease.
On a number of occasions I have asked Miss R. about the strange “nostalgia” which she showed in July 1969, and how she experiences the world generally. She usually becomes distressed and “blocked” when I ask such questions, but on a few occasions she has given me enough information for me to perceive the almost incredible truth about her. She indicates that in her “nostalgic” state she knew perfectly well that it was 1969 and that she was sixty-four years old, but that she felt that it was 1926 and she was twenty-one; she adds that she can’t really imagine what it’s like being older than twenty-one, because she has never really experienced it. For most of the time, however, there is “nothing, absolutely nothing, no thoughts at all” in her head, as if she is forced to block off an intolerable and insoluble anachronism—the almost half-century gap between her age as felt and experienced (her ontological age) and her actual or official age. It seems, in retrospect, as if the L-DOPA must have “de-blocked” her for a few days, and revealed to her a time-gap beyond comprehension or bearing, and that she has subsequently been forced to “re-block” herself and the possibility of any similar reaction to L-DOPA ever happening again. She continues to look much younger than her years; indeed, in a fundamental sense, she is much younger than her age. But she is a Sleeping Beauty whose “awakening” was unbearable to her, and who will never be awoken again.
A DEAF WORLD
We are remarkably ignorant about deafness, which Dr. Johnson called “one of the most desperate of human calamities”—much more ignorant than an educated man would have been in 1886, or 1786. Ignorant and indifferent. During the last few months I have raised the subject with countless people and nearly always met with responses like: “Deafness? Don’t know any deaf people. Never thought much about it. There’s nothing interesting about deafness, is there?” This would have been my own response a few months ago.
Things changed for me when I was sent a fat book by Harlan Lane called When the Mind Hears: A History of the Deaf, which I opened with indifference, soon to be changed to astonishment,
and then to something approaching incredulity. I discussed the subject with my friend and colleague Dr. Isabelle Rapin, who has worked closely with the deaf for twenty-five years. I got to know better a congenitally deaf colleague, a remarkable and highly gifted woman, whom I had previously taken for granted.14 I started seeing, or exploring for the first time, a number of deaf patients under my care. My reading rapidly spread from Harlan Lane’s history to The Deaf Experience, a collection of memoirs by and about the first literate deaf, edited by Lane, and then to Nora Ellen Groce’s Everyone Here Spoke Sign Language, and to a great many other books. Now I have an entire bookshelf on a subject that I had not thought of even as existing six months ago, and have seen some of the remarkable films that have been produced on the subject.
The text of this selection has been edited slightly from its original form in Seeing Voices.
One more acknowledgment by way of preamble. In 1969 W. H. Auden gave me a copy, his own copy, of Deafness,a remarkable autobiographical memoir by the South African poet and novelist David Wright, who became deaf at the age of seven. “You’ll find it fascinating,” he said. “It’s a wonderful book.” It was dotted with his own annotations (though I do not know whether he ever reviewed it). I skimmed it, without paying more attention, in 1969. But now I was to rediscover it for myself. David Wright is a writer who writes from the depths of his own experience—and not as a historian or scholar writes about a subject. Moreover, he is not alien to us. We can easily imagine, more or less, what it would be like to be him (whereas we cannot without difficulty imagine what it would be like to be someone born deaf, like the famous deaf teacher Laurent Clerc). Thus he can serve as a bridge for us, conveying us through his own experiences into the realm of the unimaginable. Since Wright is easier to read than the great mutes of the eighteenth century, he should if possible be read first—for he prepares us for them. Toward the close of the book he writes: