Vintage Sacks by Oliver Sacks

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