1 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. Scientifi cAmerican.com UNCOMMON GENIUS exclusive online issue no. 31
Millions of years of evolution have endowed Homo sapiens with remarkable intellect. But not all human brains are created equal. From the great powers of memory seen in savants to the skills of chess grandmasters, unusual talents can offer a unique window on how the mind works. This exclusive online issue examines genius in some of its most intriguing forms. Meet Kim Peek, whose abilities provided the inspiration for the character Raymond Babbit in the movie Rain Man. Peek’s severe developmental disabilities prevent him from managing the chores of daily life, but he has learned 9,000 books by heart so far, among other astonishing feats of memory. Other savants have musical or artistic talents. Less well known than savant syndrome is Williams syndrome, a disorder in which affected individuals generally score below average on standard IQ tests, but often possess startling language and music skills, as another article in this issue describes. Mood disorders, too, have been linked to genius: it seems that manic-depressive illness and major depression can enhance creativity in some people. Other articles focus on gifted children. These youngsters fascinate with their precocious intellect, but they often suffer ridicule and neglect. They also tend to be keenly aware of the potential risk of failure, which can prove emotionally paralyzing for them. Studies of such children have provided key insights into brain development—and revealed how best to nurture their extraordinary minds. Our fi nal article in the issue considers whether some geniuses are made, not born. Dissections of the mental processes of chess grandmasters have shown that their skills arise from years of “effortful study”—continually tackling challenges that lie just beyond their competence. Could comparable training turn any one of us into such an expert? Food for thought.--The Editors
TABLE OF CONTENTS 2 Islands of Genius BY DAROLD A. TREFFERT AND GREGORY L. WALLACE; SCIENTIFIC AMERICAN MIND JANUARY 2004 Artistic brilliance and a dazzling memory can sometimes accompany autism and other developmental disorders
7 Inside the Mind of a Savant BY DAROLD A. TREFFERT AND DANIEL D. CHRISTENSEN SCIENTIFIC AMERICAN MAGAZINE; DECEMBER 2005 Kim Peek possesses one of the most extraordinary memories ever recorded. Until we can explain his abilities, we cannot pretend to under- stand human cognition
11 Williams Syndrome and the Brain BY HOWARD M. LENHOFF, PAUL P. WANG, FRANK GREENBERG AND URSULA BELLUGI; SCIENTIFIC AMERICAN MAGAZINE DECEMBER 1997 To gain fresh insights into how the brain is organized, investigators are turning to a little known disorder
16 Manic-Depressive Illness and Creativity BY KAY REDFIELD JAMISON; SCIENTIFIC AMERICAN PRESENTS: MYSTERIES OF THE MIND Does some fi ne madness plague great artists? Several studies show that creativity and mood disorders are linked
21 Uncommon Talents: Gifted Children, Prodigies and Savants BY ELLEN WINNER; SCIENTIFIC AMERICAN PRESENTS: EXPLORING INTELLIGENCE Possessing abilities well beyond their years, gifted children inspire admiration, but they also suffer ridicule, neglect and misunderstanding
25 Watching Prodigies for the Dark Side BY MARIE-NOËLLE GANRY-TARDY; SCIENTIFIC AMERICAN MIND APRIL 2005 Gifted children who are not challenged can quickly grow bored with school, but a hidden fear of failure can lead to far greater problems
27 The Expert Mind BY PHILIP E. ROSS; SCIENTIFIC AMERICAN MAGAZINE AUGUST 2006 Studies of the mental processes of chess grandmasters have revealed clues to how people become experts in other fi elds as well
1 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. Islands OF GENIUS Artistic brilliance and a dazzling memory can sometimes accompany autism and other developmental disorders
By Darold A. Treffert and Gregory L. Wallace
ORIGINALLY PUBLISHED IN JANUARY 2004
eslie Lemke is a musical virtuo- renowned, collected by Margaret Thatcher and so. At the age of 14 he played, Pope John Paul II, among others. A London art flawlessly and without hesi- professor was “thunderstruck” by the oil crayon tation, Tchaikovsky’s Piano drawings that Wawro did as a child, describing Concerto No. 1 after hearing them as an “incredible phenomenon rendered it for the fi rst time while listen- with the precision of a mechanic and the vision ing to a television movie sev- of a poet.” Wawro, who lives in Scotland, is au- eral hours earlier. Lemke had never had a piano tistic. lessonL—and he still has not had one. He is blind Kim Peek is a walking encyclopedia. He has and developmentally disabled, and he has cere- memorized more than 7,600 books. He can re- bral palsy. Lemke plays and sings thousands of cite the highways that go to each American city, pieces at concerts in the U.S. and abroad, and he town or county, along with the area and zip codes, improvises and composes as well. television stations and telephone networks that Richard Wawro’s artwork is internationally serve them. If you tell him your date of birth, he
2 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. can tell you what day of the week it fell on and using the then accepted classifi cation of an idiot what day of the week it will be when you turn 65 as someone with an IQ of less than 25, combined “and can retire.” Peek can identify most classical with a derivative of the French word savoir, which compositions and knows the date the music was means “to know.” published or fi rst performed as well as the com- More than a century has passed since Down’s poser’s birthplace and dates of birth and death. description. Today we know much more about He is also developmentally disabled and depends this perplexing set of abilities from the 100 or on his father for many of his basic daily needs. His so cases described in the scientifi c literature. Sa- abilities provided the inspiration for the character vant syndrome generally occurs in people with Raymond Babbitt, whom Dustin Hoffman played IQs between 40 and 70—although it can occur in in the 1988 movie Rain Man. some with IQs up to 114 or even higher. It dispro- Lemke, Wawro and Peek all have savant syn- portionately affects males, with four to six male drome, an uncommon but spectacular condition savants for every one female. And it can be con- in which people with various developmental dis- genital or acquired later in life following disease abilities, including autism, possess astonishing is- (such as encephalitis) or brain injury. lands of ability and brilliance that stand in jarring juxtaposition to their overall mental handicap. Narrow Repertoire Savant syndrome is seen in about one in 10 people The skills that savant syndrome gives rise to with autism and in approximately one in 2,000 are limited for the most part, and they tend to people with brain damage or mental retardation. be based in the right hemisphere. That is, they Of the known savants, at least half are autistic are predominantly nonsymbolic, artistic, visual and the remainder have some other kind of de- and motor. They include music, art, mathematics, velopmental disorder. forms of calculating, and an assortment of other Much remains mysterious about savant syn- abilities, such as mechanical aptitude or spatial drome. Nevertheless, advances in brain imaging skills. In contrast, left hemisphere skills are more are permitting a more complete view of the con- sequential, logical and symbolic; they include lan- dition, and a long-standing theory of left hemi- guage and speech specialization [see “The Split spheric damage has found support in these imag- Brain Revisited,” by Michael S. Gazzaniga; Sci- ing studies. In addition, new reports of the sudden entifi c American, July 1998]. appearance of savant syndrome in people with Most musical savants have perfect pitch and certain forms of dementia have raised the intrigu- perform with amazing ease, most often on the ing possibility that some aspects of such genius lie piano. Some are able to create complex composi- dormant in all of us. tions. And for some reason, musical genius often seems to accompany blindness and mental retar- Down’s Defi nition dation, as it does for Lemke. One of the most Descriptions of savant syndrome appear in famous savants was “Blind Tom” Bethune, who the scientifi c literature as early as 1789. Benja- lived from 1849 to 1908. In his time, he was re- min Rush, the “father of American psychiatry,” ferred to as “the eighth wonder of the world.” described the lightning-quick calculating ability Although he could speak fewer than 100 words, of Thomas Fuller, who understood little math he could play beautifully more than 7,000 pieces more complex than counting. When Fuller was on the piano, including many of his own works. asked how many seconds a man had lived by the (Some of his compositions were recorded by mu- time he was 70 years, 17 days and 12 hours old, sician John Davis and released in 2000.) he gave the correct answer of 2,210,500,800 a For their part, savant visual artists use a variety minute and a half later—and he had taken into of media, although they most frequently express account 17 leap years. themselves through drawing and sculpture. Artis- It was not until 1887, however, that the re- tic savant Alonzo Clemons, for example, can see a markable coexistence of defi ciency and superior- fl eeting image of an animal on a television screen ity was more completely laid out. That year J. and in less than 20 minutes sculpt a perfect replica Langdon Down, who is best known for having of that animal. His wax model will be correct in identifi ed Down syndrome, described 10 people every detail, every fi ber and muscle and propor- with savant syndrome. He had met these fasci- tion. nating individuals during his 30 years as superin- Mathematical savants calculate incredibly tendent of the Earlswood Asylum in London. He rapidly and often have a particular facility with coined the now discarded term “idiot savant,” prime numbers. Curiously, the obscure skill of
3 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. calendar calculating that Peek demonstrates is to establish some kind of normal routine or way not confi ned to mathematical savants; it seems to of life [see box on page 6]. coexist with many different skills. Several other abilities appear less frequently. A Looking to the Left Hemisphere rare savant may have extensive language ability— Although specialists today are better able to that is, the capacity to memorize many languages characterize the talents of savants, no overarching but not to understand them. Other unusual traits theory can describe exactly how or why savants include heightened olfactory, tactile and visual do what they do. The most powerful explana- sensitivity; outstanding knowledge in fi elds such tion suggests that some injury to the left brain as history, neurophysiology, statistics or naviga- causes the right brain to compensate for the loss. tion; and spatial ability. For instance, a musical The evidence for this idea has been building for and blind savant named Ellen can navigate in several decades. A 1975 pneumoencephalogram thick forests or other unfamiliar spaces without study found left hemispheric damage in 15 of 17 running into objects. Ellen also has a perfect ap- autistic patients; four of them had savant skills. preciation of passing time despite the fact that she (A pneumoencephalogram was an early and pain- doesn’t have access to a watch or clock, even in ful imaging technique during which a physician Braille. This ability was discovered one day when would inject air into a patient’s spinal fl uid and her mother let her listen to the “time lady” on then x-ray the brain to determine where the air the telephone. After listening for a short while to traveled. It is no longer used.) the recorded voice intone the hour and seconds, A dramatic study published by T. L. Brink Ellen apparently set her own internal clock. Since in 1980 lent further credence to the possibility then, she has been able to tell what time it is to the that changes to the left hemisphere were impor- second, no matter the season. tant to savant syndrome. Brink, a psychologist at Savant skills are always linked to a remark- Crafton Hills College in California, described a able memory. This memory is deep, focused and normal nine-year-old boy who had become mute, based on habitual recitation. But it entails little deaf and paralyzed on the right side when a bul- understanding of what is being described. Some let damaged his left hemisphere. After the acci- early observers aptly called this “memory with- dent, unusual savant mechanical skills emerged. out reckoning.” Down himself used the phrase He was able to repair multigeared bicycles and to “verbal adhesion” to characterize it. One of his design contraptions, such as a punching bag that patients was a boy who had read the six-volume would weave and bob like a real opponent. History of the Decline and Fall of the Roman Em- The fi ndings of Bernard Rimland of the Au- pire, by Edward Gibbon, and could recite it back tism Research Institute in San Diego support this word for word, although he did so without any idea as well. Rimland maintains the largest data- comprehension. base in the world on people with autism; he has Although they share many talents, including information on more than 34,000 individuals. memory, savants vary enormously in their levels He has observed that the savant skills most of- of ability. So-called splinter-skill savants have a ten present in autistic people are those associated preoccupation and mild expertise with, say, the with right hemisphere functions and the most de- memorization of sports trivia and license plate fi cient abilities are associated with left hemisphere numbers. Talented savants have musical or artis- functions. tic gifts that are conspicuously above what would In the late 1980s Norman Geschwind and be expected of someone with their handicaps. Albert M. Galaburda of Harvard University of- And prodigious savants are those very uncom- fered an explanation for some causes of left hemi- mon people whose abilities are so advanced that spheric damage—and for the higher number of they would be distinctive even if they were to oc- male savants. In their book Cerebral Lateraliza- cur in a normal person. Probably fewer than 50 tion, the two neurologists point out that the left prodigious savants are alive at the moment. hemisphere of the brain normally completes its Whatever their talents, savants usually main- development later than the right and is therefore tain them over the course of their life. With contin- subject to prenatal infl uences—some of them det- ued use, the abilities are sustained and sometimes rimental—for a longer period. In the male fetus, even improve. And in almost all cases, there is no circulating testosterone can act as one of these dreaded trade-off of these wonderful abilities with detrimental infl uences by slowing growth and im- the acquisition of language, socialization or daily pairing neuronal function in the more vulnerable living skills. Instead the talents often help savants left hemisphere. As a result, the right brain often
4 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. compensates, becoming larger and more domi- ticolimbic circuit for what is generally referred to nant in males. The greater male-to-female ratio as explicit, semantic or cognitive memory, and is seen not just in savant syndrome but in other a lower-level corticostriatal circuit for the more forms of central nervous system dysfunction, such primitive habit memory referred to as implicit as dyslexia, delayed speech, stuttering, hyperac- or procedural memory. The memory of savants tivity and autism. seems to be the noncognitive habit form. The same factors that produce left hemispheric Newly Savant damage may be instrumental in producing dam- In recent years, more data have emerged to age to higher-level memory circuits. As a result, support the left hemisphere hypothesis. In 1998 savants may be forced to rely on more primitive, Bruce L. Miller of the University of California at but spared, habit memory circuits. Perhaps brain San Francisco examined fi ve elderly patients with injuries—whether they result from hormones, dis- frontotemporal dementia (FTD), one form of pre- ease, or prenatal or subsequent injury—produce senile dementia. These patients had developed in some instances certain right-brain skills linked artistic skills with the onset and progression of with habit memory function. In those situations, their dementia. They were able to make meticu- savant syndrome may appear. lous copies of artworks and to paint beautifully. Consistent with that in savants, the creativity in Rain Man in Us All? these fi ve individuals was visual, not verbal. Single- The emergence of savantlike skills in people photon-emission computed tomography (SPECT) with dementia raises profound questions about showed that injury was predominantly on the left the buried potential in all of us. Accordingly, sev- side of the brain. Miller examined seven other eral researchers are seeking to unlock what has patients who had developed musical or artistic been called the “little Rain Man in each of us.” ability after the appearance of FTD. He found One group has used a technique called repetitive damage on the left as well. transcranial magnetic stimulation (rTMS) in 17 Miller, Craig Hou, then at Washington Uni- normal individuals, eight male and nine female. versity, and others then compared these images Tracy Morrell of the University of South Austra- with those of a nine-year-old artistic autistic sa- lia, Robyn L. Young of Flinders University in Ad- vant named DB. SPECT scans of DB revealed elaide and Michael C. Ridding of Adelaide Uni- a higher-than-normal blood fl ow in part of his versity applied magnetic stimulation to the area neocortex but decreased fl ow in his left temporal in the left temporal lobe that Miller identifi ed as lobe. (The neocortex is involved with high-level damaged in his FTD patients. cognitive function; the temporal lobe is respon- In its study, the team reports that only two sible for some aspects of memory and emotion.) of the participants experienced a series of short- Miller is hoping to study other artistic savants lived skills, such as calendar calculating, artistic to see if the fi ndings hold true for them as well. ability and enhanced habit memory. Other sub- But the fact that DB and older FTD patients with jects discovered a new skill here and there, also newfound savant skills have the same pathology lasting just a few hours. The researchers suggest is quite striking and suggests that researchers will that savant skills may be limited to a small per- soon be able to identify precisely the neurological centage of the normal population, much as they features associated with savant syndrome. are limited to a small percentage of the disabled The seemingly limitless memory of savants population. will most likely be harder to pinpoint physiologi- Nevertheless, many experts believe that real cally. Mortimer Mishkin of the National Institute potential exists to tap into islands of savant intelli- of Mental Health has proposed different neural gence. Allan Snyder and John Mitchell of the Aus- circuits for memory, including a higher-level cor- tralian National University in Canberra argue that savant brain processes occur in each of us but are overwhelmed by more sophisticated conceptual (The Authors) cognition. Autistic savants, they conclude, “have DAROLD A. TREFFERT and GREGORY L. WALLACE share a long-standing privileged access to lower levels of information not interest in savant syndrome. Treffert ([email protected]) is a clinical professor normally available through intro spection.” of psychiatry at the University of Wisconsin–Madison and has done research Our view is also that all of us have some of the on autism and savant syndrome since 1962, the year he met his fi rst savant. same circuitry and pathways intrinsic to savant Wallace ([email protected]) is a research fellow in the Child Psychiatry Branch of the National Institute of Mental Health. He is conducting studies on functioning but that these are less accessible—in why individuals with autism are more likely to develop savant skills. part because we tend to be a left-brain society.
5 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. (Living with Savant Syndrome) Richard Wawro feels delight and excitement when he fi n- few reports in the literature suggest that when sa- ishes a work, and he seeks out celebration. And memory vants are encouraged to acquire better language wizard Kim Peek has emerged from the social isolation Askills they lose their special artistic talents. Per- that characterized him before the movie Rain Man was haps the most famous of these cases is that of Nadia, made; he now travels the country talking to hundreds of a girl with autism who by the age of three was producing school groups. astounding drawings. When she turned seven, Nadia en- Fortunately, simultaneously encouraging savant tered a school for autistic children that focused on verbal abilities and normalization is now the generally accepted abilities; by the time she was a teenager, Nadia was more approach to such individuals’ care. Savants are being verbal but could no longer create brilliant and intricate placed in some classes for the gifted and talented, an drawings. opportunity that promotes social growth for both them This trade-off between talent and language or so- and their classmates. Some new programs, such as the cialization is not something we have witnessed. Instead one at Hope University in Anaheim, Calif., cater entirely the exceptional abilities of savants have proved to be to these exceptional individuals. Others include people strengths that are built on and used as a conduit toward with similar disorders as well; for example, music and art normalization; these skills have helped individuals de- camps have been established for those with Williams syn- velop improved social skills, better language acquisition drome, many of whom have savantlike musical skills [see and greater independence. Savants gain a sense of ac- “Williams Syndrome and the Brain,” by Howard M. Len- complishment because of their talent; that sense, in turn, hoff, Paul P. Wang, Frank Greenberg and Ursula Bellugi; allows them to participate more fully in the world. Musical SCIENTIFIC AMERICAN, December 1997]. Nurturing the tal- prodigy Leslie Lemke has become more animated, per- ent of these people is the most fulfi lling approach. forming concerts and interacting with audiences. Painter —D.A.T. and G.L.W.
Sometimes, though, we can fi nd elements of the and repair—areas of research that are vital in un- savant in ourselves. At certain moments, we just derstanding and treating such diverse conditions “get” something or discover a new ability. And as stroke, paralysis and Alzheimer’s disease. some procedures—including hypnosis; interviews But savant syndrome has relevance outside of subjects under the infl uence of the barbiturate the scientifi c realm. Many lessons can be learned sodium amytal, which induces relaxation; and from these remarkable people and their equally brain stimulation during neurosurgery—provide remarkable families, caretakers, therapists and evidence that a huge reservoir of memories lies teachers. One of the greatest lessons is that they dormant in every individual. Dreams can also re- have been shaped by far more than neural cir- vive those memories or trigger new abilities. cuitry. The savants thrive because of the reinforce- No model of brain function will be complete ment provided by the unconditional love, belief until it can explain this rare condition. Now that and determination of those who care for them. we have the tools to examine brain structure and Savant syndrome promises to take us further than function, such studies can be correlated with de- we have ever been toward understanding both the tailed neuropsychological testing of savants. We brain and human potential. hope the anecdotal case reports that have charac- terized the literature on this topic for the past cen- tury will soon be replaced by data comparing and contrasting groups of normal and disabled people, including prodigies, geniuses and savants. (Further Reading) A Window into the Brain Savant syndrome provides a unique window ◆ Emergence of Artistic Talent in Frontotemporal Dementia. B. Miller, J. Cummings and F. Mishkin et al. in Neurology, Vol. 51, No. 4, into the brain with regard to questions of general pages 978–982; October 1, 1998. intelligence versus multiple forms of intelligence. ◆ Extraordinary People: Understanding Savant Syndrome. It may also shed light on brain plasticity and cen- Darold A. Treffert. iUniverse.com, Inc., 2000. tral nervous system compensation, recruitment ◆ www.savantsyndrome.com
6 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. Inside the MMindind of a SSavantavant Kim Peek possesses one of the most extraordinary memories ever recorded. Until we can explain his abilities, we cannot pretend to understand human cognition
By Darold A. Treffert and Daniel D. Christensen Originally published in December 2005
When J. Langdon Down fi rst described savant syndrome in 1887, coining its name and noting its association with astounding powers of memory, he cited a patient who could recite Edward Gibbon’s The Decline and Fall of the Roman Empire verbatim. Since then, in almost all cases, savant memory has been linked to a specifi c domain, such as music, art or mathematics. But phenomenal memory is itself the skill in a 54-year-old man named Kim Peek. His friends call him “Kim-puter.”
7 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. He can, indeed, pull a fact from his mental library as fast the ordinarily obscure skill of calendar calculating (always as a search engine can mine the Internet. He read Tom Clan- associated with massive memory), occur with such regularity cy’s The Hunt for Red October in one hour and 25 minutes. among savants. Recently, when an interviewer offered that he Four months later, when asked, he gave the name of the Rus- had been born on March 31, 1956, Kim noted, in less than a sian radio operator in the book, referring to the page describ- second, that it was a Saturday on Easter weekend. ing the character and quoting several passages verbatim. Kim Imaging studies of Kim’s brain thus far show considerable began memorizing books at the age of 18 months, as they structural abnormality [see box on page 10]. These fi ndings were read to him. He has learned 9,000 books by heart so far. cannot yet be linked directly to any of his skills; that quest is He reads a page in eight to 10 seconds and places the memo- just beginning. Newer imaging techniques that plot the brain’s rized book upside down on the shelf to signify that it is now functions—rather than just its structure—should provide more on his mental “hard drive.” insight, though. In the meantime, we believe it is worthwhile Kim’s memory extends to at least 15 interests—among to document the remarkable things that Kim can do. People them, world and American history, sports, movies, geogra- like him are not easily found, and it is useful to record their phy, space programs, actors and actresses, the Bible, church characteristics for future research. Savantism offers a unique history, literature, Shakespeare and classical music. He knows window into the mind. If we cannot explain it, we cannot all the area codes and zip codes in the U.S., together with the claim full understanding of how the brain functions. television stations serving those locales. He learns the maps in the front of phone books and can provide Yahoo-like trav- An Unusual Brain el directions within any major U.S. city or between any pair kim was born on November 11, 1951 (a Sunday, he will of them. He can identify hundreds of classical compositions, tell you). He had an enlarged head, on the back of which was tell when and where each was composed and fi rst performed, an encephalocele, or baseball-size “blister,” which spontane- give the name of the composer and many biographical details, ously resolved. But there were also other brain abnormalities, and even discuss the formal and tonal components of the mu- including a malformed cerebellum. One of us (Christensen) sic. Most intriguing of all, he appears to be developing a new did the initial MRI brain scans on Kim in 1988 and has fol- skill in middle life. Whereas before he could merely talk about lowed his progress ever since. music, for the past two years he has been learning to play it. The cerebellar fi ndings may account for Kim’s problems It is an amazing feat in light of his severe developmental with coordination and mobility. But more striking still is the problems—characteristics shared, in varying extents, by all absence of a corpus callosum, the sizable stalk of nerve tissue savants. He walks with a sidelong gait, cannot button his that normally connects the left and right halves of the brain. clothes, cannot manage the chores of daily life and has great We do not know what to make of this defect, because al- diffi culties with abstraction. Against these disabilities, his tal- though it is rare, it is not always accompanied by functional ents—which would be extraordinary in any person—shine all disorders. Some people have been found to lack the structure the brighter. An explanation of how Kim does what he does without suffering any detectable problems at all. Yet in people would provide better insight into why certain skills, including whose corpus callosum has been severed in adulthood, gener- ally in an effort to prevent epileptic seizures from spreading Peek’s Peaks from one hemisphere to the other, a characteristic “split- Overview/ brain” syndrome arises in which the estranged hemispheres ■ Great powers of memory run through every known begin to work almost independently of each other. manifestation of savant skill. In the case of Kim Peek, It would seem that those born without a corpus callosum memory is itself the skill. somehow develop back channels of communication between ■ Kim’s brain exhibits many abnormalities, including an the hemispheres. Perhaps the resulting structures allow the absent corpus callosum. The role of that particular two hemispheres to function, in certain respects, as one giant abnormality in Kim’s case remains to be explained, but it hemisphere, putting functions normally rather separate under evokes a question raised by the skills of all savants: the same roof, as it were. If so, then Kim may owe some of his Does brain damage stimulate compensatory talents to this particular abnormality. In any case, the fact development in some other area of the brain, or does it that some people lacking a corpus callosum suffer no dis- simply allow otherwise latent abilities to emerge? abilities, whereas others have savant abilities, makes its pur- ■ Kim’s rote learning later developed into a form of pose less clear than formerly thought. Neurologists joke that associative thinking, with clear evidence of creativity. its only two certain functions are to propagate seizures and His success then helped him engage the wider world. hold the brain together. The authors conclude that savant skills should never be Theory guides us in one respect. Kim’s brain shows abnor- dismissed but should be cultivated for the patient’s malities in the left hemisphere, a pattern found in many sa- intellectual and social development. vants. What is more, left hemisphere damage has been in- voked as an explanation of why males are much more likely
8 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. than females to display not only savantism but also dyslexia, taurant to “lower his voice,” Kim merely slid lower into his stuttering, delayed speech, and autism. The proposed mecha- chair, thus lowering his voice box. In other cases, his answers nism has two parts: male fetuses have a higher level of circu- can seem quite ingenious. In one of his talks he answered a lating testosterone, which can be toxic to developing brain question about Abraham Lincoln’s Gettysburg Address by tissue; and the left hemisphere develops more slowly than the responding, “Will’s house, 227 North West Front Street. But right and therefore remains vulnerable for a longer period. he stayed there only one night—he gave the speech the next Also supporting the role of left hemisphere damage are the day.” Kim intended no joke, but when his questioner laughed, many reported cases of “acquired savant syndrome,” in which he saw the point; since then, he has purposely recycled the older children and adults suddenly develop savant skills after story with humorous intent and effect. damage to the left hemisphere. Yet Kim does have an undeniable power to make clever What does all this evidence imply? One possibility is that connections. He once attended a Shakespeare festival spon- when the left hemisphere cannot function properly, the right sored by a philanthropist known by the initials O.C., whose hemisphere compensates by developing new skills, perhaps by laryngitis threatened to keep him from acknowledging a tes- recruiting brain tissue normally earmarked for other purpos- timonial. Kim—a fan of Shakespeare, and like him, an incor- es. Another possibility is that injury to the left hemisphere rigible punster—quipped, “O.C., can you say?” merely unveils skills that had been latent in the right hemi- Such creative use of material that had originally been sphere all along, a phenomenon some have called a release memorized by rote can be seen as the verbal equivalent of a from the “tyranny” of the dominant left hemisphere. musician’s improvisation. Like the musician, Kim thinks Kim underwent psychological testing in 1988. His overall quickly, so quickly that it can be diffi cult to keep up with his IQ score was 87, but the verbal and performance subtests intricate associations. Often he seems two or three steps varied greatly, with some scores falling in the superior range ahead of his audiences in his responses. of intelligence and others in the mentally retarded range. The A rather startling new dimension to Kim’s savant skills has psychological report concluded, therefore, that “Kim’s IQ recently surfaced. In 2002 he met April Greenan, director of classifi cation is not a valid description of his intellectual abil- the McKay Music Library and professor of music at the Uni- ity.” The “general intelligence” versus “multiple intelligences” versity of Utah. With her help, he soon began to play the piano debate rages on in psychology. We believe that Kim’s case and to enhance his discussion of compositions by playing pas- argues for the latter point of view. sages from them, demonstrating on the keyboard many of the Kim’s overall diagnosis was “developmental disorder not pieces he recalled from his massive mental library. Kim also otherwise specifi ed,” with no diagnosis of autistic disorder. has remarkable long-term memory of pitch, remembering the Indeed, although autism is more commonly linked with sa- original pitch level of each composition. vantism than is any other single disorder, only about half of He possesses complete knowledge of the instruments in all savants are autistic. In contrast with autistic people, Kim the traditional symphony orchestra and readily identifi es the is outgoing and quite personable. One thing that does seem timbre of any instrumental passage. For example, he present- necessary for the full development of savant skills is a strong ed the opening of Bedrich Smetana’s orchestral tone poem interest in the subject matter in question. The Moldau, by reducing the fl ute and clarinet parts to an arpeggiated fi gure in his left hand and explaining that the Memory and Music oboes and bassoons enter with the primary theme, which he in kim’s case, all the interests began in rote memorization then reduced to pitches played singly and then in thirds by his but later progressed to something more. Although Kim gener- right hand (the left-hand fi gure continuing as it does in the ally has a limited capacity for abstract or conceptual think- score). His comprehension of musical styles is demonstrated ing—he cannot, for example, explain many commonplace in his ability to identify composers of pieces he had not previ- proverbs—he does comprehend much of the material he has ously heard by assessing the piece’s musical style and deduc- committed to memory. This degree of comprehension is un- ing who that composer might be. usual among savants. Down himself coined the interesting phrase “verbal adhesion” to describe the savant’s ability to DAROLD A. TREFFERT and DANIEL D. CHRISTENSEN have long remember huge quantities of words without comprehension. been fascinated by savantism. Treffert, a psychiatrist in Wis- Sarah Parker, a graduate student in psychology at the Univer- consin, has done research on autism and savant syndrome sity of Pennsylvania, in a description of a savant named Gor- since 1962, the year he fi rst met a savant. He was consultant don stated it more colorfully when she noted that “owning a to the movie Rain Man and is author of Extraordinary People: kiln of bricks does not make one a mason.” Kim not only Understanding Savant Syndrome. Christensen is clinical profes- THE AUTHORS owns a large kiln of bricks, he has also become a strikingly sor of psychiatry, clinical professor of neurology and adjunct creative and versatile word mason within his chosen areas of professor of pharmacology at the University of Utah Medical expertise. School. His work focuses on Alzheimer’s disease, but following Sometimes his answers to questions or directions are quite Kim Peek for more than two decades has given him an ongoing concrete and literal. Once when asked by his father in a res- interest in savant syndrome.
9 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. A MISSING CONNECTION? Rain Man, whose main character, Raymond Babbitt, is a sa- Kim Peek’s brain differs from typical brains in several vant played by Dustin Hoffman. The movie is purely fi ctional ways. Kim’s brain and head are very large, each in the 99th and does not tell Kim’s life story, even in outline. But in one percentile. Most striking is the complete absence of the remarkably prescient scene, Raymond instantly computes corpus callosum, which normally connects the left and right hemispheres. Missing, too, are the anterior and posterior square roots in his head, and his brother, Charlie, remarks, commissures, which also usually link the hemispheres. “He ought to work for NASA or something.” For Kim, such a The cerebellum, responsible for certain motor functions, collaboration might well happen. is smaller than usual and malformed, with fl uid occupying NASA has proposed to make a high-resolution 3-D ana- much of the surrounding space; this may explain some tomical model of Kim’s brain architecture. Richard Boyle, of Kim’s diffi culties with coordination. What role these director of the NASA BioVIS Technology Center, describes the abnormalities play in his mental abilities is the subject of project as part of a larger effort to overlay and fuse image data investigation. from as wide a range of brains as possible—and that is why Kim’s unusual brain is of particular value. The data, both static and functional, should enable investigators to locate and identify changes in the brain that accompany thought and behavior. NASA hopes that this detailed model will enable physicians to improve their ability to interpret output from far less capable ultrasound imaging systems, which are the only kind that can now be carried into space and used to Corpus callosum monitor astronauts. The fi lming of Rain Man and the movie’s subsequent suc- cess proved to be a turning point in Kim’s life. Before then, he had been reclusive, retreating to his room when company came; afterward, the confi dence he gained from his contacts with the fi lmmakers, together with the celebrity provided by the movie’s success, inspired him and his father, Fran Peek, to share Kim’s talents with many audiences. They became en- thusiastic emissaries for people with disabilities, and over the years they have shared their story with more than 2.6 million people. We believe that Kim’s transformation has general appli- cability. Much of what scientists know about health comes Though Kim is still physically awkward, his manual dex- out of the study of pathologies, and certainly much of what terity is increasing. When seated at the piano, he may play the will be learned about normal memory will come from the piece he wishes to discuss, sing the passage of interest or de- study of unique or unusual memory. In the meantime, we scribe the music verbally, shifting seamlessly from one mode draw some practical conclusions for the care of other persons to another. Kim pays attention to rhythm as well, lightly tap- with special needs who have some savant skill. We recom- ping the beat on his chest with his right hand or, when play- mend that family and other caregivers “train the talent,” ing, tapping his right foot. rather than dismissing such skills as frivolous, as a means for Greenan, a Mozart scholar, makes these observations: the savant to connect with other people and mitigate the ef- “Kim’s knowledge of music is considerable. His ability to re- fects of the disability. It is not an easy path, because disability call every detail of a composition he has heard—in many cas- and limitations still require a great deal of dedication, pa- es only once and more than 40 years ago —is astonishing. The tience and hard work—as Kim’s father, by his example, so connections he draws between and weaves through composi- convincingly demonstrates. tions, composer’s lives, historical events, movie soundtracks Further exploration of savant syndrome will provide both and thousands of facts stored in his database reveal enormous scientifi c insights and stories of immense human interest. Kim intellectual capacity.” She even compares him to Mozart, who Peek provides ample evidence of both. also had an enlarged head, a fascination with numbers and uneven social skills. She wonders whether Kim might even MORE TO EXPLORE learn to compose. The Real Rain Man. Fran Peek. Harkness Publishing Consultants, 1996. Extraordinary People: Understanding Savant Syndrome. Reprint Life after Rain Man edition. Darold A. Treffert. iUniverse, Inc., 2000. Islands of Genius. Darold A. Treffert and Gregory L. Wallace in it is not surprising that Kim’s prodigious memory Scientifi c American, Vol. 286, No. 6, pages 76–85; June 2002. caught the attention of writer Barry Morrow at a chance www.savantsyndrome.com, a Web site maintained by the Wisconsin
meeting in 1984 and inspired him to write the screenplay for Medical Society. CHEN SARA
10 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. Williams Syndrome and the Brain To gain fresh insights into how the brain is organized, investigators are turning to a little known disorder
by Howard M. Lenhoff, Paul P. Wang, Frank Greenberg and Ursula Bellugi
originally published in December 1997
hen a teenager with an Such anecdotes have recently led to live up to their fullest potential; lack of IQ of just 49 was asked the first systematic study of musical abil- familiarity with Williams syndrome in Wto draw an elephant and ity in Williams children. The results in- medical circles and the absence of reli- tell what she knew about the animal, dicate that the youngsters discriminate able tests have hindered prompt diag- her sketch was almost indecipherable. melodies well; they also show signifi- nosis in the past. But her description was impressively cantly more interest in and emotional rich, even lyrical. As part of that de- responsivity to music than do subjects Understanding Grew Slowly scription, she noted, “It has long, gray from the general population. As one ears, fan ears, ears that can blow in the Williams child said, “Music is my favo- lthough Williams syndrome, which wind....” rite way of thinking.” A occurs in an estimated one in In her verbal ability, that young wom- Investigators are attracted to Wil- 20,000 births worldwide, has gained an is fairly typical of people who have liams syndrome in part because they increased attention lately, it is not by Williams syndrome, a rare condition suspect the dramatic peaks and valleys any means new. An investigation by that has recently started to draw the at- in the abilities of affected individuals one of us (Lenhoff) suggests that Wil- tention of a range of scientists. Affected will provide a new window to the or- liams people were the inspiration for individuals, sometimes called Williams ganization and adaptability of the nor- some age-old folktales about elves, pix- people, are not all alike but often are mal brain. Some groups are attempting ies and other “wee people” [see box on similar to one another. They are fre- to pinpoint characteristic properties of page 15]. quently diagnosed as mildly to mod- the Williams brain and to determine The medical community became erately “retarded” and generally score how those properties influence perfor- aware of the syndrome fairly recently, below average on standard IQ tests. mance in intellectual and other realms. however—only about 40 years ago. They usually read and write poorly and At the same time, researchers are trying In 1961 J.C.P. Williams, a heart spe- struggle with simple arithmetic. Yet to uncover the genetic abnormalities re- cialist in New Zealand, noted that a they display striking strengths in some sponsible for Wil liams syndrome. subset of his pediatric patients shared realms. They generally demonstrate a In 1993 they learned that the disorder many characteristics. Beyond having facility not only for spoken language is caused by loss of a tiny piece from related cardiovascular problems, they but also for recognizing faces. And, as one of the two copies of chromosome also had elfin facial features (such as a a group, they tend to be empathetic, lo- 7 present in every cell of the body. The turned-up nose and a small chin) and quacious and sociable. delet ed piece can contain 15 or more seemed to be mentally retarded. The What is more, anecdotal evidence genes. As the lost genes are identified, cardiac problems Williams observed implies that some Williams people scientists can begin to determine how often included heart murmurs and nar- possess extraordinary musical talent. their absence leads to the neuro ana tom- rowing of major blood vessels. In par- Even though their attention span for ical and behavioral features already ticular, Williams people frequently suf- most tasks is short, many will listen to observed. This integrated approach to fer from supraval vular aortic stenosis music, sing and play instruments with the study of Williams syndrome—con- (SVAS), a mild to severe constriction of astonishing persistence. Most can- necting genes to neurobiology and, ul- the aorta. not read musical notes, yet some have timately, to behavior—may become a Since that time, physicians have perfect or nearly perfect pitch and an model for exploring how genes affect noted other traits, some of which can uncanny sense of rhythm. One boy brain development and function. be seen quite early in life. In infancy, quickly learned to play an extremely Medical scientists are interested in babies may have difficulty feeding and complex drumbeat in 7/4 time with Williams syndrome in its own right may suffer from stomach pains, consti- one hand while drumming in 4/4 time as well. Analysis of the genes in the pation and hernias. They may also sleep with the other hand. A number of indi- deleted region has already explained poorly and can be irritable and colicky, viduals retain complex music for years, why Williams people commonly suffer behavior sometimes caused by anoth- remembering melodies and verses of from certain physical ailments. It has er frequent sign: elevated amounts of long ballads; one even sings songs in 25 also provided a means of prenatal test- calcium in the blood. As the children languages. Experienced Wil liams musi- ing and is helping to diagnose the dis- get older, they reveal hoarse voices and cians also sing harmonies, improvise order earlier, so that children who are show delayed physical and mental de- and compose lyrics readily. affected can be helped from infancy to velopment. They begin walking at an
11 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. Williams Syndrome: An Inspiration for Some Pixie Legends?
olktales from many cultures feature magical “little people”—pixies, THE DEPICTION OF an elf at the left is Felves, trolls and other fairies. A number of physical and behavioral the 19th-century work of Richard Doyle, similarities suggest that at least some of the fairies in the early yarns were an uncle of the Sherlock Holmes creator. modeled on people who have Williams syndrome. Such a view is in keep- ing with the contention of historians that a good deal of folklore and my- thology is based on real life. The facial traits of Williams people are often described as pixielike. In represent the sensitivity of those mythical individuals—and of Williams common with pixies in folklore and art, many with Wil liams syndrome have people—to music and to sound in general.) small, upturned noses, a depressed nasal bridge, “puffy” eyes, oval ears and As a group, Williams people are loving, trusting, caring and extremely broad mouths with full lips accented by a small chin. Indeed, those features sensitive to the feelings of others. Similarly, fairies are frequently referred to are so common that Williams children tend to look more like one another as the “good people” or as kind and gentle-hearted souls. Finally, Williams than their relatives, especially as children. The syndrome also is accompa- individuals, much like the fairies of leg end, require order and pre dict ability. nied by slow growth and development, which leads most Williams indi- In Williams people this need shows up as rigid adherence to daily routines viduals to be relatively short. and a constant need to keep abreast of future plans. The “wee, magical people” of assorted folktales often are musicians and In the past, storytellers created folktales about imaginary beings to help storytellers. Fairies are said to “repeat the songs they have heard” and can explain phenomena that they did not understand—perhaps including “enchant” humans with their melo dies. Much the same can be said of peo- the distinguishing physical and behavioral traits of Williams syndrome. ple with Williams syndrome, who in spite of typically having subnormal Today researchers turn to Williams people in a quest to understand the IQs, usually display vivid narrative skills and often show talent for music. unknown, hoping to decipher some of the secrets of how the brain func- (The large pointed ears so often associated with fairies may symbolically tions.—H.M.L.
ILLUSTRATION FROM In Fairyland, by William Allingham, 1870 William Allingham, by FROM In Fairyland, ILLUSTRATION average of 21 months, often on the tors discovered that for a segment of for elastin. Sure enough, they found balls of their feet and usually with an this population, SVAS stemmed from the gene was deleted from one of the awkwardness that persists throughout an inherited mutation in one copy of two copies of chromosome 7 in cells. life. Fine motor control is disturbed as the gene that gives rise to elastin—a Today it is evident that the deletion of well. In addition, Williams people are protein that provides elasticity to many the gene occurs in approximately 95 extremely sensitive to noise, are often organs and tissues, such as the arteries, percent of patients with Williams syn- short compared with their peers and lungs, intestines and skin. drome. The loss is harmful presumably seem to age prematurely (for instance, because both gene copies are needed to their hair grays and their skin wrinkles Missing Genes Are Identified make adequate amounts of the elastin relatively early). protein. Description began to give way to ge- ware that SVAS is common in Wil- The investigators knew that a re- netic understanding about four years A liams people and that individu- duction in the elastin supply could ago, thanks in part to a study of SVAS als with familial SVAS alone and indi- contrib ute to various physical features in people who did not have Williams viduals with Williams syndrome both of Williams syndrome (such as SVAS, syndrome. In 1993 Amanda K. Ewart suffer disturbances in organs that re- hernias and premature wrinkling), but and Mark T. Keating of the University quire elasticity, the workers wondered it could not by itself account for the of Utah, Colleen A. Morris of the Uni- whether Williams syndrome, too, in- cognitive and behavioral signatures. versity of Nevada and other collabora- volved some kind of change in the gene After all, their first subjects, who had
Drawing and Description of an Elephant by a Teen with Williams Syndrome
“What an elephant is, it is one of the animals. And what an elephant does, it lives in the jun- gle. It can also live in the zoo. And what it has, it has long, gray ears, fan ears, ears that can HEAD BODY blow in the wind. It has a long trunk that can EAR pick up grass or pick up hay. If they’re in a bad EYE mood, it can be terrible. If the elephant gets mad, it could stomp; it could charge. Some- TRUNK times elephants can charge. They have big long tusks. They can damage a car. It could MOUTH be dangerous. When they’re in a pinch, when they’re in a bad mood, it can be terrible. You don’t want an elephant as a pet. You want a cat or a dog or a bird.” COURTESY OF URSULA BELLUGI COURTESY
12 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. BAND 7q11.23 TINY DELETION from one of the two copies of chromosome 7 in cells NORMAL COPY OF is the cause of Williams syndrome CHROMOSOME 7 (drawing). The excised region can contain 15 or more genes, only DAMAGED COPY OF some of which have been identified. CHROMOSOME 7 A diagnostic test is based on the dis- covery that the gene for elastin is APPROXIMATE POSITION usually among those lost. The test Known Genes in Deleted Segment OF DELETION flags copies of chromosome 7 with a fluorescent green tag and flags the ELASTIN FZD3 gene for elastin with a fluorescent LIM-KINASE 1 WSCR1 TOMO NARASHIMA TOMO red tag.
SVAS alone without cognitive impair- more likely than other children to ac- guishing that population from others. ment, would also have had low IQs if a quire Williams syndrome. Finally, the She and her colleagues therefore began dim inution of elastin could unilaterally microscopic technique that originally to devise tests of specific abilities and to produce all the symptoms of Williams revealed the deletion of the gene for compare the scores of Williams people syndrome. This awareness led them to elastin—fluorescent in situ hybridiza- with those of the general population suspect that more genes were affected. tion, or FISH—has now been adapted and of another cognitively impaired In support of that idea, direct examina- for use as a diagnostic tool. group: people with Down syndrome. tions of chromosomes from Williams The investigations, which continue, patients indicated that the region de- A Cognitive Profile Emerges examine populations of adolescents leted from chromosome 7 extended be- matched for sex, age and IQ level. (Wil- yond the bound aries of the gene for elas- ork on the genetics of Williams liams people range in IQ from 40 to tin and probably encompassed many Wsyndrome is complementing 100, but their mean score is about 60.) genes. efforts to specify the neurobiologi- Early on, the team saw that Williams Several of those other genes are now cal hallmarks of the disorder. That subjects, in contrast to their generally being uncovered. Among them are three research, which today involves sev- weak performance on overall tests of (LIM-kinase 1, FZD3 and WSCR1) eral laboratories, began about 15 years cognitive ability, commonly used well- that are active in the brain—a sign that ago, when one of us (Bel lugi) answered formed grammar in their spontaneous they could influence brain develop- a late-night telephone call in her labo- speech. On the whole, they also per- ment and function. The exact activities ratory at the Salk Institute for Biologi- formed significantly better than the carried out by the encoded proteins are cal Studies in La Jolla, Calif. The caller group with Down syndrome did on all not known, although Ewart and her knew that Bel lugi investigated the neu- tasks of grammatical comprehension colleagues have proposed that LIM-ki- robiological underpinnings of language and production. nase 1 (which is invariably deleted with and believed her daughter, who had Many also did well at the rather the gene for elastin) may be involved Williams syndrome, would interest the complex task of constructing tag ques- in the ability to grasp spatial relation- Salk group. The girl, then 13, had an IQ tions, such as adding “does n’t she?” to ships. This role could help explain why near 50 and was considered mentally the statement “Leslie likes fish.” The Williams people have diffi culty draw- retarded. Consistent with that profile, person being tested must first take the ing simple com mon objects accurately she read and wrote at the level of a first original statement (“Leslie likes fish”) from memory. Another gene from the grader. Yet she spoke beautifully. and substitute a matching pronoun deleted area, RFC2, specifies a protein Then, as now, scientists had difficulty for the subject (“She likes fish”). Then involved in replication of DNA, but its distinguishing the brain processes con- the individual must add a conjugated contribution to Williams syndrome has trolling language from those control- auxiliary verb, negate it and contract it not been established. ling reasoning, because in the general (“She doesn’t like fish”), omit the origi- The genetic understanding of Wil- population, adeptness at language and nal verb and object (leaving only “She liams syndrome is far from complete. cognition usually go hand in hand. The doesn’t”) and invert the word order to Still, discovery of the deletion in chro- dichotomy in the caller’s daughter sug- form a question (“..., doesn’t she?”). mosome 7 has yielded some practical gested that study of Williams people The Salk researchers further found, rewards. That the deletion occurs in all might help tease apart those processes. as others did later, that the Williams cells of the body in Williams people tells Fascinated, Bellugi agreed to meet subjects frequently had vocabularies mothers nothing they did or failed to do the girl and then continued to see her larger than would be expected for their during pregnancy caused their child’s regularly. She also sought literature mental age. When asked to list some condition. The disorder stems from a detailing the cognitive strengths and animals, they often did not stick to easy sperm or egg that, by chance, suffers weaknesses of Williams people but words but chose such exotic examples a loss of genes from chromosome 7 be- found little beyond general assertions. as yak, Chihuahua, ibex, condor and fore donating its chromosomes to the Before Bellugi could hope to uncover unicorn. crea tion of an embryo. That knowledge the areas of the brain and the neuro- Beyond possessing richer vocabular- also tells healthy siblings of Williams logical processes that accounted for ies, subjects with Williams syndrome people that their copies of chromo- the unique cognitive characteristics tended to be more expressive than even some 7 are free of the deletion; there- of Williams people, she would need a normal children were. This animation fore, any children they bear are no finer-grained profile of the traits distin- was demonstrated amusingly when Wil-
13 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. liams children were asked to provide a In some ways, the general profile age-matched normal subjects. But the story for a series of wordless pictures. revealed by the various cognitive tests volumes of individual regions differ be- As they told their tale, they often al- implies that the chromosomal defect in tween the two groups. For instance, the tered their pitch, volume, length of Williams syndrome essentially spares frontal lobes and the limbic region of words or rhythm to enhance the emo- the left hemisphere (the region most the temporal lobes are better preserved tional tone of the story. Similarly, they important to language in the large ma- in Williams people. The limbic system, added more drama to engage their au- jority of people) and disrupts the right which also includes other structures, is dience (“And suddenly, splash!”; “And (the more visual-spatial hemisphere). important for brain activities involving BOOM!”; “Gadzooks!”) than subjects But the emotional expressiveness of memory and emotions; sparing of the with Down syndrome did. (Sadly, the Williams people (also thought to be a limbic region may help explain why Wil- gift of gab and sociability of Wil liams right-sided function) and at least one liams people are quite expressive and children can mislead teachers into other finding cast doubt on that sim- empathetic. thinking the children have better rea- plistic view. Williams people recognize Analyses of the cerebellum uncov- soning skills than they actually possess; and discriminate among pictures of un- ered further differences between the in those cases, the children may not get familiar faces (a skill that requires the Williams and Down groups. Whereas the academic support they need.) right hemisphere) remarkably well. its volume in Down subjects was small, One possible explanation for the In fact, they perform as well as adults that in Wil liams subjects was normal. strong verbal performance of Williams from the general population. And in Williams subjects the neocere- individuals is that their chromosomal bellum (considered to be the evolution- defect, in contrast to that of Down Neurological Studies Add Clarity arily young est region of the cerebel- subjects, may not significantly disrupt lum) was equal to or larger than that in certain faculties that support language he Salk group’s examination of age-matched individuals in the general processing. Other researchers, for in- Tbrains by magnetic resonance imag- population but was reduced in Down stance, have reported that short-term ing and by autopsy supports the prob- subjects. memory for speech sounds, or “phono- ability that the chromosomal deletion The finding that the neocerebellum logical working memory”—a form that responsible for Williams syndrome is preserved in Williams people is par- seems to assist in language learning and alters the brain in a more complicated ticularly intriguing when placed in the comprehension—is relatively preserved way. The deletion seems to produce context of other research. Until recent- in the Williams population. anatomical changes (such as abnormal ly, the cerebellum was thought to be Interestingly, recent studies of French clustering of neurons in visual areas) concerned primarily with movement. and Italian Williams subjects suggest that yield deficits in visual-spatial abili- Yet Steven E. Petersen and his col- that one aspect of language known as ties. But the chromosomal defect ap- leagues at Washington University have morphology—the facet of grammar pears to spare a network that includes shown that the neo cerebellum becomes that deals with verb conjugation, gen- structures in the frontal lobes, the tem- active when subjects try to think of a der assignment and pluralization—may poral lobe and the cerebellum. This verb that fits with a given noun (such not be completely preserved in Wil- preserved network, then, may serve as as “sit” for “chair”). Further, tests of liams people. (These languages are a neuroanatomical scaffolding for the patients with cerebellar injuries reveal much richer in morphology than Eng- unexpectedly strong language abilities deficits in cognitive function, not just in lish is.) This discovery implies that the of Williams people. motor abilities. And anatomists report brain regions preserved in Williams To be more specific, the neuroana- that the neocere bel lum communicates syndrome and the presence of an intact tomical studies indicate that the overall extensively with a part of the frontal short-term memory for speech sounds cortical volume in both Williams and cortex that, in common with the neo- support many verbal aptitudes but may Down people is smaller than that of cerebellum, is larger in humans than in not suffice for full mastery of language. In contrast to their generally good showing on verbal tests, Williams sub- The Making of a Cognitive Profile jects typically do poorly on tasks in- volving visual processing, such as copy- s part of an effort to pinpoint cognitive features that are characteristic of Williams people, in- ing drawings. But they often fail on Avestigators have compared subjects with Williams and with Down syndrome on tests of spe- such tasks in different ways than Down cific abilities. One test (top)—which asked adolescents to copy from memory a letter D that was built subjects do, suggesting that the deficits from a collection of small Y’s—revealed impairment in integrating details into a larger configuration. in the two groups may stem from dif- The Williams group tended to draw only Y’s, whereas the Down group tended to maintain the overall ferences in brain anatomy. For exam- configuration but omit local details. Another test (bottom)—in which subjects had to invent a story ple, Williams people, in common with for a series of wordless pictures—revealed that Williams people can often generate well-structured patients who have suffered a stroke in narratives. the right hemisphere of the brain, may Task: REPRODUCE attend to components of images but Williams subjects Down subjects fail to appreciate the overall pattern IMAGE (the gestalt). Down people, however, are more likely to perceive the global organization but to overlook many de-
tails, just as individuals do who have OF URSULA BELLUGI COURTESY suffered left-hemisphere strokes.
14 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. BASIC ANATOMY OF BRAIN in a SIDE OF BRAIN PARIETAL tor who carried out the first studies of people with Williams syndrome is nor- LOBE musical ability in Williams people. She mal, but the total volume is somewhat FRONTAL OCCIPITAL reduced. The areas that seem to be best concludes that intact perception of au- LOBE LOBE preserved include the frontal lobes and a ditory patterns may account for much part of the cerebellum called the neocer- of the strength in music and language ebellum (a), as well as parts of the tem- seen in Williams subjects—a result that poral lobes known as the limbic area (b), implies the related brain structures the primary auditory area and the pla- should also be intact. num temporale (c). Physiological probes comparing elec- tri cal activity in the brains of Williams apes. people and others during specific tasks SYLVIAN Given that humans have language offer more insights into how the brain FISSURE and apes do not, some observers have develops. In response to grammatical TEMPORAL CEREBELLUM proposed that the neocerebellum and stimuli, for example, normal subjects LOBE the connected region of the frontal cor- show greater activity from the left hemi- NEOCEREBELLUM tex evolved together to support the flu- sphere than from the right, as would be ent processing of speech and may fall expected for language tasks. But Wil- under the control of the same genes. liams people show symmetrical respons- b UNDERSIDE The relative preservation of the fron- es in the two hemispheres, a sign that LEFT HEMISPHERE tal cortex and the enlargement of the the typical language specialization of neocerebellum in Williams people, to- the left hemisphere has not occurred. gether with the rather spared fluency Further, whereas normal adults gener- LIMBIC AREAS OF in language, lend some credence to this ally show greater activity from the right TEMPORAL last notion and to the idea that the cer- hemisphere than the left when process- LOBES ebellum plays a part in language pro- ing images of faces, Williams people cessing. show the opposite pattern. Such work Recent anatomical analyses have ad- favors the possibility that when normal ditionally identified features that could developmental processes go awry, the help explain the apparent musical tal- brain often redistributes responsibili- c CUT IN PLANE OF SYLVIAN FISSURE ent of Williams people. The primary au- ties, forming new circuits to carry out ditory cortex (located in the temporal the functions of the disrupted ones. lobe) and an adjacent auditory region, Research into Williams syndrome is the planum temporale (thought to be just now taking off, but it is already important to language as well as mu- helping to clarify how the brain is or- CUT LINE sicality), are proportionately enlarged ganized. It is also making investigators in the few Williams brains examined see “mentally retarded” individuals so far. In addition, the planum tempo- in a new light. Close study of Wil- rale is normally more extensive in the liams syndrome has shown that low IQ PRIMARY LEFT HEMISPHERE AUDITORY left hemisphere than in the right, but in scores can mask the existence of excit- CORTEX some Williams people the left region is ing capacities. And it warns that other unusually big, to an extent character- so-called mentally retarded individuals TOMO NARASHIMA TOMO PLANUM TEMPORALE istic of professional musicians. These could have untapped potentials wait- findings mesh well with observations ing to be uncovered—if only research- by Audrey Don of the University of ers, and society, will take the trouble to Windsor in Ontario, the investiga- look for and cultivate them.
The Authors Further Reading
HOWARD M. LENHOFF, PAUL P. WANG, FRANK GREENBERG Hemizygosity at the Elastin Locus in a Developmen- and URSULA BELLUGI offer several perspectives on Williams syndrome. tal Disorder: Williams Syndrome. A. K. Ewart et al. Lenhoff is professor emeritus of biological sciences at the University of in Nature Genetics, Vol. 5, No. 1, pages 11–16; September California, Irvine, the father of a 42-year-old Williams syndrome musi- 1993. cian and co-organizer of the Williams Syndrome Music and Arts Camp, Cognitive and Neural Development: Clues from Ge- held in Massachusetts. He is also principal investigator of a team com- netically Based Syndromes. U. Bellugi, E. S. Klima and paring music cognition in Williams people with other populations. Wang, P. P. Wang in The Lifespan Development of Individuals: assistant professor of pediatrics at the University of Pennsylvania School Behavioral, Neurobiological, and Psychosocial Perspec- of Medicine, studies the neurobehavioral manifestations of Williams syn- tives: A Synthesis. Nobel Symposium. Edited by D. Mag- drome and other genetic disorders. Greenberg, clinical consultant with the nusson. Cambridge University Press, 1996. National Center for Human Genome Research at the National Institutes Real-World Source for the “Little People”: The Re- of Health, has worked with Williams syndrome individuals for 20 years. lationship of Fairies to Individuals with Wil liams Bellugi is director of the Laboratory for Cognitive Neurosciences at the Syndrome. Howard M. Lenhoff in Nursery Realms: Chil- Salk Institute for Biological Studies. She heads a multidisciplinary team dren in the Worlds of Science Fiction, Fantasy and Horror. that has been examining the cognitive, neuroanatomical and neurophysi- Edited by Gary Westfahl and George Slusser. University of ological characteristics of Williams syndrome for more than a decade. Georgia (in press).
15 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. Manic-Depressive Illness and Creativity Does some fine madness plague great artists? Several studies now show that creativity and mood disorders are linked
by Kay Redfield Jamison Originally published in February 1995
en have called me mad,” wrote Edgar Allan Poe, “but the question is not yet settled, whether madness is or is not the loftiest intelligence— whether much that is glorious—whether all that is profound—does The Author not spring from disease of thought—from moods of mind exalted at theM expense of the general intellect.” KAY REDFIELD JAMISON is professor of psychiatry at Many people have long shared Poe’s suspicion that genius and insanity are entwined. the Johns Hopkins Univer- Indeed, history holds countless examples of “that fine madness.” Scores of influential sity School of Medicine. She 18th- and 19th-century poets, notably William Blake, Lord Byron and Alfred, Lord Ten- wrote Touched with Fire: nyson, wrote about the extreme mood swings they endured. Modern American poets Manic-Depressive Illness and John Berryman, Randall Jarrell, Robert Lowell, Sylvia Plath, Theodore Roethke, Delmore the Artistic Temperament Schwartz and Anne Sexton were all hospitalized for either mania or depression during and co-authored the medi- their lives. And many painters and composers, among them Vincent van Gogh, Georgia cal text Manic-Depressive Illness. Jamison is a member O’Keeffe, Charles Min gus and Robert Schumann, have been similarly afflicted. of the National Advisory Judging by current diagnostic criteria, it seems that most of these artists—and many Council for Hu man Genome others besides—suffered from one of the major mood disorders, namely, manic-depressive Research and clinical di rector illness or major depression. Both are fairly common, very treatable and yet frequently of the Dana Consortium on lethal diseases. Major depression induces intense melancholic spells, whereas manic-de- the Genetic Basis of Manic- pression, a strongly genetic disease, pitches patients repeatedly from depressed to hyper- Depressive Illness. She has also written and produced a active and euphoric, or intensely irritable, states. In its milder form, termed cyclothymia, series of public television spe- manic-depression causes pronounced but not totally debilitating changes in mood, behav- cials about manic-depressive ior, sleep, thought patterns and energy levels. Advanced cases are marked by dramatic, illness and the arts. cyclic shifts. Could such disruptive diseases convey certain creative advantages? Many people find that proposition counterintuitive. Most manic-depressives do not possess extraordinary imagination, and most accomplished artists do not suffer from recurring mood swings. To assume, then, that such diseases usually promote artistic talent wrongly reinforces simplis- tic notions of the “mad genius.” Worse yet, such a generalization trivializes a very serious medical condition and, to some degree, discredits individuality in the arts as well. It would be wrong to label anyone who is unusually accomplished, energetic, intense, moody or eccentric as manic-depressive. All the same, recent studies indicate that a high number of established art ists—far more than could be expected by chance—meet the diagnostic crite- ria for manic-depression or major depression given in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV). In fact, it seems that these dis eases can sometimes enhance or otherwise contribute to creativity in some people. By virtue of their prevalence alone, it is clear that mood disorders do not necessarily breed genius. Indeed, 1 percent of the general population suffer from manic-depression, also called bipo lar disorder, and 5 percent from a major depression, or unipolar disorder, during their lifetime. Depression affects twice as many women as men and most often, but not always, strikes later in life. Bipolar disorder afflicts equal num bers of women and men, and more than a third of all cases surface before age 20. Some 60 to 80 percent of all adolescents and adults who commit suicide have a history of bipolar or uni polar illness. Before the late 1970s, when the drug lithium first became widely available, one person in five with manic-depression com mit ted suicide.
16 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. The Case of Vincent van Gogh
any clinicians have reviewed the medical Mand psychiatric problems of the painter Vin- cent van Gogh posthumously, diagnosing him with a range of disorders, including epilepsy, schizophrenia, digitalis and absinthe poisoning, manic-depressive psychosis, acute intermittent porphyria and Ménière’s disease. Richard Jed Wyatt of the National Institute of Men- tal Health and I have argued in detail that van Gogh’s symptoms, the natural course of his illness and his family psychiatric history strongly indicate manic- depressive illness. The extent of the artist’s purported use of absinthe and convulsive behavior remains unclear; in any event, his psychiatric symptoms long predate any possible history of seizures. It is possible that he suffered from both an epileptic disorder and manic-depressive illness. —K. R. J. 1958 LEVY, R. ADELE OF GIFT ART, OF MUSEUM METROPOLITAN Irises, 1889
Major depression in both unipolar have chaotic personal and professional dinarily high occurrence of mood dis- and bipolar disorders manifests itself relationships. They may spend large orders and alcohol i sm among them. through apathy, lethargy, hopeless- sums of money, drive recklessly or pur- Eighty percent had experienced at least ness, sleep disturbances, slowed physi- sue questionable busi ness ventures or one epi sode of major depression, hypo- cal movements and thinking, impaired sexual liaisons. In some cases, manics mania or mania; 43 percent reported a memory and concentration, and a suffer from violent agitation and de- history of hypomania or mania. Also, loss of pleasure in typically enjoyable lusional thoughts as well as visual and the relatives of these writers, compared events. The diagnostic criteria also in- auditory hallucinations. with the relatives of the control sub- clude suicidal thinking, self-blame and jects, generally performed more creative inappropriate guilt. To distinguish clin- Rates of Mood Disorders work and more often had a mood dis- ical depression from normal periods of order. un hap piness, the common guidelines or years, scientists have documented A few years later, while on sabbatical further require that these symptoms Fsome kind of connection between in England from the University of Cali- persist for a minimum of two to four mania, depression and creative output. fornia at Los Angeles, I began a study weeks and also that they significantly In the late 19th and early 20th centuries, of 47 distinguished British writers and interfere with a person’s everyday func- researchers turned to accounts of mood visual artists. To select the group as tioning. disorders written by prominent artists, best I could for creativity, I purpose- their physicians and friends. Although fully chose painters and sculptors who Mood Elevation largely anecdotal, this work strongly were Royal Academicians or Associates suggested that renowned writers, art- of the Royal Academy. All the play- uring episodes of mania or hypo- ists and composers—and their first- wrights had won the New York Drama Dma nia (mild mania), bipolar pa- degree relatives—were far more likely Critics Award or the Evening Standard tients experience symptoms that are in to experience mood disorders and to Drama (London Critics) Award, or many ways the opposite of those as- commit suicide than was the general both. Half of the poets were already sociated with depression. Their mood population. During the past 20 years, represented in the Oxford Book of and self-esteem are elevated. They more systematic studies of artistic pop- Twentieth Century English Verse. I sleep less and have abundant energy; ulations have confirmed these findings found that 38 percent of these artists their productivity increases. Manics [see illustration on page 19]. Diagnos- and writers had in fact been previous- frequently become paranoid and irri- tic and psychological analyses of living ly treated for a mood disorder; three table. Moreover, their speech is often writers and artists can give quite mean- fourths of those treated had required rapid, excit able and intrusive, and their ingful estimates of the rates and types medication or hospitalization, or both. thoughts move quickly and fluidly from of psychopathology they experience. And half of the poets—the largest frac- one topic to another. They usually hold In the 1970s Nancy C. Andreasen of tion from any one group—had needed tre men dous conviction about the cor- the University of Iowa completed the such extensive care. rectness and importance of their own first of these rigorous studies, which Hagop S. Akiskal of the University of ideas as well. This grandi osity can con- made use of structured interviews, California at San Diego, also affiliated tribute to poor judg ment and impulsive matched control groups and strict di- with the University of Tennessee at behavior. agnostic criteria. She examined 30 Memphis, and his wife, Kareen Akis- Hypomanics and manics generally creative writers and found an extraor- kal, subsequently interviewed 20 award-
17 SCIENTIFIC AMERICAN EXCLUSIVE ONLINE ISSUE AUGUST 2006 COPYRIGHT 2006 SCIENTIFIC AMERICAN, INC. The Tainted Blood of the Tennysons Alfred, Lord Tennyson, who experienced recurrent, debilitating depressions and probable hypomanic spells, often ex- pressed fear that he might inherit the madness, or “taint of blood,” in his family. His father, grandfather, two of his great- grandfathers as well as five of his seven brothers suffered from insanity, mel ancholia, uncontrollable rage or what is today known as manic-depressive illness. His brother Edward was confined to an asylum for nearly 60 years before he died from manic exhaustion. Lionel Tennyson, one of Alfred’s two sons, displayed a mercurial temperament, as did one of his three grandsons. Modern medicine has confirmed that manic-depression and creativity tend to run in cer tain families. Studies of twins pro- vide strong evidence for the heritability of manic-depres sive illness. If an identical twin has manic-depressive illness, the other twin typically has a 70 to 100 percent chance of also having the disease; if the other twin is fraternal, the chances are considerably lower (approximately 20 percent). A review of pairs of identical twins reared apart from birth—in which at least one had been diagnosed as manic-depressive—found that in two thirds or more of the sets, the illness was present in both twins. —K. R. J.
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