Everything in Its Place

by Oliver Sacks

  In the last decade or two, there has been increasingly active research in the field of “spiritual neurosciences.” There are special difficulties in this research, for religious experiences cannot be summoned at will; they come, if at all, in their own time and way—the religious would say in God’s time and way. Nonetheless, researchers have been able to demonstrate physiological changes not only in pathological states like seizures, OBEs, and NDEs, but also in positive states like prayer and meditation. Typically these changes are quite widespread, involving not only primary sensory areas in the brain but limbic (emotional) systems, hippocampal (memory) systems, and the prefrontal cortex, where intentionality and judgment reside.

  Hallucinations, whether revelatory or banal, are not of supernatural origin; they are part of the normal range of human consciousness and experience. This is not to say that they cannot play a part in the spiritual life or have great meaning for an individual. Yet while it is understandable that one might attribute value, ground beliefs, or construct narratives from them, hallucinations cannot provide evidence for the existence of any metaphysical beings or places. They provide evidence only of the brain’s power to create them.

  * I have described ecstatic seizures, as well as near-death experiences, at greater length in Hallucinations.

  Hiccups and Other Curious Behaviors

  In On the Move, I recounted the story of a man I met in 1960, when I worked as a research assistant in San Francisco for Grant Levin and Bertram Feinstein, two neurosurgeons whose specialty was operating on patients with parkinsonism.

  One of their patients, Mr. B., was a coffee merchant who had survived an attack of encephalitis lethargica during the great epidemic of the 1920s but was now very disabled by postencephalitic parkinsonism. Mr. B. was a little frail and had emphysema, but otherwise seemed an excellent candidate for a cryosurgery that had been developed to reduce parkinsonian tremor and stiffness.

  Immediately after the procedure, though, he developed hiccups, a symptom which at first we took to be trivial and transient. But his hiccups did not go away; they grew stronger and stronger, spreading to muscles in his back and abdomen, jolting his entire trunk. They were so violent as to interfere with eating, and they made sleep almost impossible. We tried the usual remedies—breathing into a paper bag, and so on—but none of these worked.

  After six days and nights of continued hiccupping, Mr. B. was exhausted and frightened—the more so since he had heard that hiccups, or their debilitating effects, could be fatal.

  Hiccupping involves a sudden jerk of the diaphragm, and sometimes, as a last resort for intractable hiccups, surgeons may block the phrenic nerves that supply the diaphragm. But this means that diaphragmatic breathing is no longer possible—one can only breathe shallowly, using the intercostal muscles in the chest. This was not an option with Mr. B., for he had emphysema and could not have survived without the use of his diaphragm.

  Hesitantly, I suggested hypnosis, and Levin and Feinstein, though skeptical, agreed that we had nothing to lose. We found a hypnotist and were astounded when he managed to induce a hypnotic state in Mr. B.—this in itself seemed little short of miraculous, given his constant hiccupping. The hypnotist planted a posthypnotic suggestion: “When I snap my fingers, you will wake up and no longer have hiccups.” He let the exhausted man sleep for ten more minutes and then snapped his fingers. Mr. B. came to, looking slightly confused—but free of hiccups. There were no relapses, and Mr. B., much helped by the cryosurgery, lived for several more years.

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  MR. B. WAS AMONG the hundreds of thousands who survived the worldwide epidemic of “sleepy sickness”—the encephalitis lethargica that raged between 1917 and 1927—only to suffer, sometimes years later, from postencephalitic syndromes of various sorts. Encephalitis lethargica could produce a wide array of lesions affecting the hypothalamus, the basal ganglia, the midbrain, and the brainstem, while sparing the cerebral cortex for the most part. Thus it particularly affected control mechanisms in the subcortex—systems involved with the regulation of sleep, sexuality, appetite; of posture, balance, and movement; and, at the brainstem level, autonomic functions like the regulation of breathing. These control systems are of great phylogenetic antiquity—occurring in most vertebrates.*1

  Many postencephalitics went on to develop an extreme form of parkinsonism, and they were also apt to develop various odd respiratory behaviors. These were especially severe in the immediate aftermath of the epidemic, although they tended to diminish with the passage of years. There were even “epidemics” of postencephalitic hiccup in several places.

  There could also be spontaneous sneezing, coughing, or yawning among the victims of the sleepy sickness, as well as paroxysmal laughter or crying. These are normal, if curious, behaviors, as Robert Provine emphasizes in his book Curious Behavior: Yawning, Laughing, Hiccupping, and Beyond. But they are rendered abnormal by their severity, their incessancy, and their occurrence in the absence of any demonstrable cause—such patients did not have irritation of the esophagus, the diaphragm, the throat or nostrils; they had nothing to laugh or cry about. Yet they were overcome by hiccups, coughs, sneezes, yawns, laughter, or crying, presumably due to lesions in the brain stimulating or releasing such behaviors so that they occurred in an autonomous and inappropriate fashion.*2

  By 1935, most of these postencephalitic patients were submerged in an all-embracing catatonia or deep parkinsonism, and their odd respiratory behaviors had all but disappeared.

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  THIRTY YEARS LATER, I was working at Beth Abraham Hospital in the Bronx with eighty-odd postencephalitic patients; and while most had parkinsonism and sleep disorders, none of them had the overt respiratory disorders described in the earlier literature. But this changed when I gave them L-dopa in 1969 and many subsequently developed respiratory and phonatory tics, including sudden deep breaths, yawns, coughs, sighs, grunts, and sniffs.

  I asked each of these patients whether they had ever experienced such respiratory symptoms in the past. Most could not give me a clear answer, but Frances D., an intelligent and articulate woman, said that she had indeed had respiratory crises from 1919 (when she came down with encephalitis lethargica) to 1924, but not thereafter. It seemed probable, in her case, that L-dopa had activated or released a preexisting sensitivity or proclivity to respiratory disorders, and I had to wonder whether this could have been the case with the other patients who developed respiratory symptoms.

  I was reminded of Mr. B., the postencephalitic coffee merchant with hiccups. Could he, too, have had damaged and hypersensitive respiratory controls, which, in his case, were released by a surgical lesion to the basal ganglia?

  There tended to be, with the continued use of L-dopa, an elaboration of these respiratory or phonatory behaviors—not only grunting and coughing but hooting and snorting, hissing and whistling, barking, bleating, lowing and mooing, humming and buzzing. Rolando O., as I wrote in Awakenings, would make a sort of “murmuring-purring sound emitted with each expiration, rather pleasing to the ear, like the sound of a distant sawmill, or bees swarming, or a contented lion after a satisfactory meal.” (Smith Ely Jelliffe, writing in the 1920s at the height of the epidemic, spoke of “menagerie noises” in such postencephalitic patients. With an entire ward of such patients at Beth Abraham now activated by L-dopa, startled visitors to the hospital sometimes wondered if there was indeed a menagerie up on the fifth floor, where my patients resided.)

  Further elaboration occurred in several patients—for Frank G., a humming noise became a verbigeration of the phrase “keep cool, keep cool,” which he uttered hundreds of times a day. Other patients developed chanting tics—tics given a rhythmic, melodic form, with a word or phrase embedded in them.*3

  Once, doing late-night rounds among my postencephalitic patients, I heard a singular sound, a sort of chorus, from a four-bedded room.
When I looked in, I found that all four patients were asleep but singing in their sleep—a rather dreary, repetitive singsong melody, but one in which the four voices were synchronized and attuned with each other. Sleepwalking, sleep talking, and sleep singing were not uncommon in these sleepy-sickness patients, but it was the coordination of the four sleeping singers that amazed me. I wondered whether it had started with Rosalie B., a very musical woman, and spread by a sort of contagion to the other sleepers.

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  A VAST NUMBER of other involuntary behaviors were activated or released with L-dopa, virtually every subcortical function taking on a life of its own, occurring autonomously and spontaneously but amplified by involuntary imitations and mimicries as the patients saw and heard each other.

  Frances D. showed a disintegration of the normal automatic controls of breathing within ten days of starting L-dopa. Her breathing became rapid, shallow, and irregular, broken up by sudden violent inspirations. Within a few days these differentiated into clear respiratory crises that would start without any warning, with a sudden inspiratory gasp followed by forced breath-holding for ten or fifteen seconds, then a violent expiration. These attacks become more and more intense, lasting almost a minute, during which Frances would struggle to expel air through a closed glottis, in so doing becoming purple and congested from the futile effort; finally the breath would be expelled with tremendous force, making a noise like the boom of a gun.

  I observed similar propensities in Frances’s roommate Martha, who had rapid breathing and difficulty in catching her breath, moving towards full-blown respiratory crises. These women’s symptoms were so similar that I had to wonder whether one of them was “imitating” the other, a thought that was reinforced when Miriam, a third patient in their four-bedded room, also started to get progressively more severe respiratory disorders:

  The first such effect [I noted] was hiccup, which would come in hour-long attacks at 6:30 every morning….A “nervous” cough and throat-clearing started, associated with a recurrent tic-like feeling of something blocking or scratching her throat…[then] a tendency to gasping and breath-holding, which in turn “replaced” the throat-clearing and coughing…finally full-blown respiratory crises that closely resembled those of Miss D.

  Another patient, Lillian W., had at least a hundred clearly different forms of crises: hiccups, panting attacks, oculogyric attacks, sniffing, sweating, chattering of the teeth, attacks in which her left shoulder would grow warm, and paroxysmal ticcing. She had ritualized iterative attacks, in which she would tap one foot in three different positions or dab her forehead in four set places; counting attacks; verbigerative attacks, in which certain phrases were said a certain number of times; fear attacks, giggling attacks, and so on. Any mention of a particular attack to Lillian would invariably bring it on. She was deeply suggestible, especially during her oculogyric crises.

  It was common for all of these curious behaviors not only to persist but to build up in intensity and spread, as if the brain was becoming sensitized and conditioned, learning or becoming overwhelmed by these perverse behaviors. These behaviors have a life of their own and once started may have to run their course; they can be difficult to stop by an act of will. They connect us to the origins of vertebrate behavior, and the ancient core of the vertebrate brain—the brainstem.

  *1 . Hiccups can appear in fetuses as early as eight weeks after gestation but diminish in the later stages of pregnancy. Though hiccups have no obvious function after birth, they may be a vestigial behavior, perhaps a vestige of the gill movements of our fishy ancestors. A similar thought can arise when one sees, in patients with certain brainstem lesions, synchronous movements affecting muscles in the neck, the palate, and the middle ear. These muscles seem to have little to do with each other until one realizes that they are all vestiges of the branchial or gill muscles of fishes—neurologists speak, therefore, of branchial myoclonus. (Many similar examples, both anatomical and functional, are discussed by Neil Shubin in Your Inner Fish.)

  *2 . This may be analogous to the occurrence of the “forced” laughter or crying sometimes seen in multiple sclerosis, ALS, Alzheimer’s disease, after some strokes, or in some patients with epilepsy who suffer so-called dacrystic (crying) or gelastic (laughing) seizures.

  *3 . In Musicophilia I described a similar evolution of an expiratory/phonatory tic to full-blown incantations, in a man with tardive dyskinesia (“Accidental Davening”).

  Travels with Lowell

  In 1986, I met a young photojournalist, Lowell Handler, who told me he had Tourette’s syndrome and that he had been experimenting with strobe photography to take pictures of other people with Tourette’s. He could often, he said, catch his subjects in mid-tic. I very much liked his photos, and we decided to travel together, meeting his fellow Touretters around the world and documenting their lives and adaptations to this strange neurological condition.

  The word “tic,” in the context of Tourette’s syndrome, covers a multitude of odd, repetitive, stereotyped, irrepressible behaviors. The simplest tics may consist of twitches or jerks, blinking, grimacing, shrugging, or sniffing. Other tics may be much more elaborate and complex. Lowell, for instance, fascinated by my old-fashioned pocket watch, developed an irresistible urge to tap it gently three times on the glass. (Once I teased him by moving the watch as he reached for it, then hiding it in a pocket. He became quite frantic at the frustration of his compulsion, and I had to produce the watch so he could satisfy his need.)

  Most tics do not have any “meaning” to begin with but are more akin to involuntary muscle (so-called myoclonic) jerks, though some tics may be elaborated or given meaning subsequently. Despite this, many of the tics and compulsions of Tourette’s seem to be aimed at testing the boundaries of what is socially acceptable or, indeed, physically possible.

  A person with Tourette’s has a certain degree of voluntary control of an otherwise involuntary or compulsive behavior, so that, with a punching tic, for example, the fist will stop millimeters from someone’s face. But Touretters may be less careful with themselves—I know two who have compulsions to fling themselves facedown onto the ground, and others who have broken bones or concussed themselves from violent blows to their own chests or heads.

  Verbal tics, especially blurted-out obscenities or curses, are relatively rare in Tourette’s, but they can cause deep offense—and here consciousness may step in to defuse the offending words. For example, Steve B., who feels compelled to shout “Nigger!,” will at the last moment turn this into “Nickels and dimes!”

  Tourettic behaviors are often at complete odds with the “real” person. Thus, when I first met Andy J., who has an irrepressible spitting tic, he struck the clipboard out of my hand and pointed to his wife, shouting, “She’s a whore, and I’m a pimp”—but he is a sweet and even-tempered young man, with the most tender feelings for his wife.

  And yet sometimes one feels that Tourette’s may contribute a special creative energy. Samuel Johnson, the great eighteenth-century man of letters, almost certainly had Tourette’s. He had many compulsions or rituals, especially upon entering a house, when he would twirl about or gesticulate in the doorway, then give a sudden spring, followed by a vast stride over the doorsill. He also exhibited strange vocalizations, litanic muttering, and involuntary mimicry of others. One cannot avoid thinking that his enormous spontaneity, antics, and lightning-quick wit had an organic connection with his accelerated, motor-impulsive state.

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  TOGETHER LOWELL AND I went to Toronto to visit Shane F., an artist who manages to make beautiful, compelling paintings and sculptures despite tics and compulsions so severe that his daily life is full of challenges and vicissitudes.

  It was obvious at first glance that Shane had a different form of Tourette’s than Lowell’s. He was constantly in movement, constantly exploring. Everything and everyone around
him would be looked at, palpated, turned over, prodded, scrutinized, smelled—a convulsive but at the same time playful investigation of the world around him. His senses seemed hyperacute; he noticed everything, and he could hear a whisper fifty yards away. He would run thirty or forty yards and then loop back—on the way, he might, with amazing agility, duck and run between someone’s legs. And he had an anarchic sense of humor, often making multilayered, instant puns and jokes.

  Shane has a particularly intense form of Tourette’s, but he avoids the medications available to dampen down his tics and vocalizations. For him, they come at too great a price, since he feels they also dampen down his creativity.

  One day the three of us strolled along a boulevard in Toronto—a sauntering broken by Shane’s sudden dashes and occasional kneeling on the ground to smell or taste the asphalt. It was a perfect, sunny day, and we passed an open-air café, where at one sidewalk table, we saw a young woman bringing a delicious-looking hamburger to her mouth. Lowell and I felt our mouths watering, but Shane leapt into action and, with a lightning-quick lunge, took a large bite out of her hamburger before it reached her mouth.

  The woman was stunned, as were her companions—but then she broke out into laughter. She saw the comedic aspect of Shane’s bizarre act, and a potentially provocative episode was defused. There are not always such happy endings to Shane’s sudden acts, which frequently go beyond the limits of social tolerance. Often he is regarded with suspicion; a number of times his unusual behavior has aroused the aggression of police or passersby. And his constant tics and compulsions can be exhausting to him and to those around him.