36 The name of an eminent researcher on Tourette’s syndrome—Dr. Abuzzahab—has an almost diagnostic power, provoking grotesque, perseverative elaborations in Tourette’s (Abuzzahuzzahab, etc.). The power of the unusual to excite and impress is not, of course, confined to Touretters. The anonymous author of the ancient mnemotechnic text Ad Herennium described it, two thousand years ago, as a natural bent of the mind and one to be exploited for fixing images more firmly in the mind:
When we see in everyday life things that are petty, ordinary, and banal, we generally fail to remember them, because the mind is not being stirred by anything novel or marvellous. But if we see or hear something exceptionally base, dishonourable, unusual, great, unbelievable, or ridiculous, that we are likely to remember for a long time. . . . Ordinary things easily slip from the memory while the striking and the novel stay longer in the mind. . . . Let art, then, imitate nature.
37 This was long presumed to have been the condition that afflicted the famous Elephant Man, John Merrick, although there is some suggestion that he may have had Proteus syndrome instead.
38 What most of us call a startling or “abnormal” speed of movement appears perfectly normal to Touretters when they show it. This was very clear in a recent experiment of target pointing with Shane F., an artist with Tourette’s. Shane showed markedly reduced reaction times, and reaching rates of almost six times normal, combined with great smoothness and accuracy of movement and aim. Such speeds were achieved quite effortlessly and naturally; normal subjects, by contrast, could achieve them, if at all, only by violent effort and with obvious compromise of accuracy and control.
On the other hand, when Shane was asked to stick to (our) normal speeds, his movements became constrained, awkward, inaccurate, and tic filled. It was clear that his normal and our normal were very different, that the Tourettic nervous system, in this sense, is more highly tuned (though, by the same token, given to precipitancy and reaction).
A similar speed and precipitancy were to be seen in many post-encephalitic patients, especially when they were activated by L-DOPA. Thus, as I remarked of Hester Y., in Awakenings, “If Mrs. Y., before L-DOPA was the most impeded person I have ever seen, she became, on L-DOPA, the most accelerated person I have ever seen. I have known a number of Olympic athletes, but Mrs. Y. could have beaten them all in terms of reaction time; under other circumstances she could have been the fastest gun in the West.”
39 The matter is especially complex, for some Touretters are given to mimicry, imitation, and impersonation of a more convulsive kind. (I describe an example of this in “The Possessed.”) This sort of imitation has no transformative effect; on the contrary, it thrusts the person deeper into Tourette’s. The Tourettic character actor was very given to convulsive impersonations and other Tourettisms offstage, but these were quite different from the deep and healing role-playing that he was able to do onstage. The superficially imitative or impersonative impulse comes from, and stimulates, a superficial part of the person (and his neural organization)—it is only a deep, total identification, as with Bennett, that can work the transformation.
40 Driving cross-country with another friend with Tourette’s was also a memorable experience, for he would twitch the steering wheel violently from side to side, stamp on the brake or the accelerator suddenly, or pull out the ignition key at speed. But he always checked that these Tourettisms were safe, and never had an accident in ten years of driving.
41 This was very clear with another Tourettic physician, an obstetrician, who had not only tics but panics and rages that, with a great effort, he could contain. When he was put on Prozac, this precarious control broke down, and he got into a violent fight with the police and spent a night in jail.
42 Canoeing with Shane F. one summer on Lake Huron was a remarkable human and clinical experience, for the canoe became an extension of his body, would pitch and plunge with each of his Tourettisms, giving me an unforgettably direct sense of what it must be like to be him. We were constantly flung around, as in a storm, constantly on the point of overturning, and I longed for the canoe to founder, and sink once and for all, so that I could escape and swim back to the shore.
43 “There are moments [Dostoevski writes of such auras], and it is only a matter of five or six seconds, when you feel the presence of the eternal harmony . . . a terrible thing is the frightful clearness with which it manifests itself and the rapture with which it fills you. If this state were to last more than five seconds, the soul could not endure it and would have to disappear. During these five seconds I live a whole human existence, and for that I would give my whole life and not think that I was paying too dearly. . . .”
44 A similar feeling of kinship may occur for a deaf traveler, who has crossed the sea or the world, if he lights upon other deaf people on his arrival. In 1814, the deaf French educator Laurent Clerc came to visit a deaf school in London, and this was described by a contemporary:
As soon as Clerc beheld this sight [of the children at dinner] his face became animated: he was as agitated as a traveller of sensibility would be on meeting all of a sudden in distant regions, a colony of his countrymen. . . . Clerc approached them. He made signs and they answered him by signs. This unexpected communication caused a most delicious sensation in them and for us was a scene of expression and sensibility that gave us the most heartfelt satisfaction. (cont.)
And it was similar when I went with Lowell Handler, a friend with Tourette’s syndrome, to a remote Mennonite community in northern Alberta where a genetic form of Tourette’s had become remarkably common. At first a bit tense, and on his best behavior, Lowell was able to suppress his tics; but after a few minutes he let out a loud Tourettic shriek. Everyone turned to look at him, as always happens. But then everybody smiled—they understood—and some even answered Lowell with their own tics and noises. Surrounded by other Touretters, his Tourettic brethren, Lowell felt, in many ways, that he had “come home” at last—he dubbed the village “Tourettesville,” and mused about marrying a beautiful Mennonite woman with Tourette’s, and living there happily ever after.
45 It was striking how green everything was in Pingelap, not only the foliage of trees, but their fruits as well—breadfruit and pandanus are both green, as were many varieties of bananas on the island. The brightly colored red and yellow fruits—papaya, mango, guava—are not native to these islands, but were only introduced by the Europeans in the 1820s.
J. D. Mollon, a preeminent researcher on the mechanisms of color vision, notes that Old World monkeys “are particularly attracted to orange or yellow fruit (as opposed to birds, which go predominantly for red or purple fruit).” Most mammals (indeed, most vertebrates) have evolved a system of dichromatic vision, based on the correlation of short- and medium-wavelength information, which helps them to recognize their environments, their foods, their friends and enemies, and to live in a world of color, albeit of a very limited and muted type. Only certain primates have evolved full trichromatic vision, and this is what enables them to detect yellow and orange fruits against a dappled green background; Mollon suggests that the coloration of these fruits may indeed have coevolved with such a trichromatic system in monkeys. Trichromatic vision enables them too to recognize the most delicate facial shades of emotional and biological states, and to use these (as monkeys do, no less than humans) to signal aggression or sexual display.
Achromatopes, or rod-monochromats (as they are also called), lack even the primordial dichromatic system, considered to have developed far back in the Paleozoic. If “human dichromats,” in Mollon’s words, “have especial difficulty in detecting colored fruit against dappled foliage that varies randomly in luminosity,” one would expect that monochromats would be even more profoundly disabled, scarcely able to survive in a world geared, at the least, for dichromats. But it is here that adaptation and compensation can play a crucial part. This quite different mode of perception is well brought out by Frances Futterman, who writes:
When a new objec
t would come into my life, I would have a very thorough sensory experience of it. I would savor the feel of it, the smell of it, and the appearance of it (all the visible aspects except color, of course). I would even stroke it or tap it or do whatever created an auditory experience. All objects have unique qualities which can be savored. All can be looked at in different lights and in different kinds of shadows. Dull finishes, shiny finishes, textures, prints, transparent qualities—I scrutinized them all, up close, in my accustomed way (which occurred because of my visual impairment but which, I think, provided me with more multi-sensory impressions of things). How might this have been different if I were seeing in color? Might the colors of things have dominated my experience, preventing me from knowing so intimately the other qualities of things?
46 In a small, isolated or recently founded population, accurate geneologies—as preserved orally among the Pingelapese and in written records by many other communities—may make it possible to delineate a single ancestral individual, or a small number of ancestors, as responsible for the spread of a genetic trait; such a situation is referred to by geneticists as “the founder effect.” Carefully kept records on Martha’s Vineyard show the “founders” of hereditary deafness there to have been two brothers carrying a recessive gene for the trait, who arrived in the 1690s. Similarly, in the little Mennonite community of LaCrete, in Alberta, where there is a high incidence of Tourette’s syndrome, all known cases of the disorder can be traced to one Gerhard Jantzen, who arrived from the Ukraine in the 1880s and founded the LaCrete community—with three successive wives, he fathered twenty-four children. And Huntington’s chorea in this country can be traced to two (very fertile) brothers arriving on Long Island in the 1630s.
Such genetically (but not necessarily clinically) abnormal individuals would not have so disproportionate an effect in a larger community—but in a small or isolated community, or one proscribing marriage outside the community (as with Mennonites, Amish, Ashkenazi Jews, etc.) there will necessarily be a great deal of intermarriage between blood relatives, many of them (in subsequent generations) now carriers of the gene. A marriage between two such carriers of a recessive gene will be likely to produce some children with manifest disease, in a Mendelian ratio.
Jared Diamond has discussed the founder effect in relation to the changing genetic profile of the world, which has moved, over the last few thousand years, to mixed and genetically homogenized populations—first with the spread of agriculture, then with the establishment of political states, and now with the extreme facility, the incontinence, of world travel. “Human genetic diversity,” he writes (in a review of this book): must have been much higher in the past than at present, as new populations were constantly being founded and expanding to carry their private genes over small local areas. Pingelap and New Guinea [it was in New Guinea that Diamond’s own evolutionary work was first done] are thus far more important to geneticists than their tiny fraction of the world’s population would suggest, because they show us our genetic landscape as it used to be.
47 There are two kerosene generators on Pingelap: one for lighting the administration building and dispensary and three or four other buildings, and one for running the island’s videotape recorders. But the first has been out of action for years, and nobody has made much effort to repair or replace it—candles or kerosene lamps are more reliable. The other dynamo, however, is carefully tended, because the viewing of action films from the States exerts a compulsive force.
48 Many holothurians have very sharp, microscopic spicules in their body walls; these spicules take all sorts of shapes—one sees button granules, ellipsoids, bars, racquets, wheel forms with spokes, and anchors. If the spicules (especially the anchor-shaped ones, which are as perfect and sharp as any boat anchor) are not dissolved or destroyed (many hours, or even days, of boiling may be needed), they may lodge in the gut lining of the unfortunate eater, causing serious but invisible bleeding. This has been used to murderous effect for many centuries in China, where trepang is regarded as a great delicacy.
49 Irene Maumenee Hussels and her colleagues at Johns Hopkins have taken samples of blood from the entire population of Pingelap and from many Pingelapese in Pohnpei and Mokil. Using DNA analysis, they hope it will be possible to locate the genetic abnormality which causes the maskun. If this is achieved, it will then be possible to identify carriers of the disease—but this, Maumenee Hussels points out, will raise complex ethical and cultural questions. It may be, for example, that such identification would militate against chances of marriage or employment for the thirty percent of the population that carries the gene.
50 In 1970 Maumenee Hussels and Morton came to Pingelap with a team of geneticists from the University of Hawaii. They came on the MS Microglory , bringing sophisticated equipment, including an electroretinogram for measuring the retina’s response to flashes of light. The retinas of those with the maskun, they found, showed normal responses from the rods, but no response whatsoever from the cones—but it was not until 1994 that Donald Miller and David Williams at the University of Rochester described the first direct observation of retinal cones in living subjects. Since then, they have used techniques from astronomy, adaptive optics, to allow routine imaging of the moving eye. This equipment has not yet been used to examine any congenital achromatopes, but it would be interesting to do so, to see whether the absence or defect of cones can be visualized directly.
Intriguingly, as Gustavo Aguirre and his colleagues at Cornell University have been investigating, there is a strain of Alaskan malamute dogs who exhibit severe day-blindness (hemeralopia) by the age of eight to ten weeks; in these dogs the retinal cones degenerate early and disappear. Like human beings with maskun, such dogs show day-blindness and total colorblindness, but—unlike achromatopes—do not have a profound loss of visual acuity, because canines lack a foveomacular region in the retina and thus do not encounter problems with foveal fixation. Like human maskun, this canine hemeralopia is inherited as an autosomal recessive, and preliminary work seems to pinpoint a specific gene in the disease process. “It will be interesting,” Aguirre notes, “once we identify the gene and the mutation in the dog, to establish whether or not the Pingelap islanders and other human achromatopes have a mutation in the same gene.”
51 There may be as many as thirty thousand of these tiny bioluminescent creatures in a cubic foot of seawater, and many observers have attested to the extraordinary brilliance of seas filled with Noctiluca. Charles Frederick Holder, in his 1887 Living Lights: A Popular Account of Phosphorescent Animals and Vegetables , relates how M. de Tessan described the phosphorescent waves as “appearing like the vivid flashes of lightning,” giving enough illumination to read by:
It lighted up the chamber that I and my companions occupied [de Tessan wrote] . . . though it was situated more than fifty yards distant from the breakers. I even attempted to write by the light, but the flashes were of too short duration.
Holder continues his account of these “living asteroids”:
When a vessel is ploughing through masses of these animals, the effect is extremely brilliant. An American captain states that when his ship traversed a zone of these animals in the Indian Ocean, nearly thirty miles in extent, the light emitted by these myriads of fire-bodies . . . eclipsed the brightest stars; the milky way was but dimly seen; and as far as the eye could reach the water presented the appearance of a vast, gleaming sea of molten metal, of purest white. The sails, masts, and rigging cast weird shadows all about; flames sprang from the bow as the ship surged along, and great waves of living light spread out ahead—a fascinating and appalling sight. . . .
The light of Noctilucae in full vigor is a clear blue; but, if the water is agitated, it becames nearly, if not quite white, producing rich silvery gleams sprinkled with greenish and bluish spangles.
Humboldt also describes this phenomenon, in his Views of Nature:
In the ocean, gelatinous sea-worms, living and dead, shine like luminous stars, conver
ting by their phosphorescent light the green surface of the ocean into one vast sheet of fire. Indelible is the impression left on my mind by those calm tropical nights in the Pacific, where the constellation of Argo in its zenith, and the setting Southern Cross, pour their mild planetary light through the ethereal azure of the sky, while dolphins mark the foaming waves with their luminous furrows.
VINTAGE BOOKS BY OLIVER SACKS
An Anthropologist on Mars
In these seven paradoxical tales of neurological disorder and creativity, Sacks transports us into the uncanny worlds of his subjects, including an artist who loses his ability to see (or even imagine) color, and a surgeon who performs delicate operations in spite of the compulsive tics and outbursts of his Tourette’s syndrome.
Psychology/Literature/0-679-75697-3
Awakenings
In the spring of 1969, Oliver Sacks initiated L-DOPA drug treatment for the post-encephalitic residents of Mount Carmel hospital, many of whom had been “frozen” and catatonic for decades. The drug had an astonishing, explosive, “awakening” effect, but the challenges of awakening to a changed world raised profound questions about medical care and what gives meaning to a life.
Psychology/Literature/0-375-70405-1
The Island of the Colorblind
Part travelogue, part medical mystery story, this is an account in which Sacks’s journeys to a tiny Pacific atoll and the island of Guam become explorations of the meaning of islands, the genesis of disease, the wonders of botany, the nature of deep geological time, and the complexities of being human.