In August 1884 Parisian newspapers reported the death of the eighty-ﬁve-year-old Marquise de Dampierre, notorious for publicly shouting out, in the middle of conversations, inappropriate or obscene words...
The history of “la maladie des tics de Gilles de la Tourette,” now commonly called Tourette syndrome, is also a history of medical practice and clinical science in Western Europe and North America from the end of the 19th century to the present. It is also a tale of medical empiricism, professional rivalries, national pride, medical authoritarianism, and the growing revolt of patients and their families against them all.
At the same time, it is a reminder of the uncharted waters and treacherous reefs that clinicians dealing with the nervous system navigated without the basic neurosciences, instruments of brain imaging, or information from controlled clinical trials to help guide them.
Encased in bone, isolated by societal and medical suppositions, until very recently the brain was fortiﬁed against meaningful study. During the 19th and the ﬁrst half of the 20th centuries, the biology of mind and brain reﬂected reports of clinical signs and symptoms, from which supposed patterns of pathology were extrapolated. Howard A. Kushner, a medical historian, skillfully documents an era in which the scientiﬁc method was being introduced into medicine. With one of the most puzzling manifestations of brain dysfunction as his protagonist, Kushner chronicles the evolution of a better understanding of the intricate relationship of the physical and behavioral manifestations of brain function.
A “MALADIE DES TICS”
History is rich in descriptions of involuntary body movements. Many were thought to have religious signiﬁcance. On other occasions, they were considered chicanery or hysteria. By the 18th century, the Age of Enlightenment, discussion of strange body posturings moved from the pulpit to the medical clinic. In the mid 19th century, the premier neurologist of France and probably Western Europe was Dr. Jean-Martin Charcot, chief physician at the Salpetriere Hospital in Paris. Dr. Charcot’s clinic was famous for presentations of unexplained cases of neurological and mental disorders, among which were disorders of movement.
Charcot assigned a young medical neurologist to organize his experiences with an unusual disorder. It was characterized by “motor tics” (involuntary movements of the face, head, limbs, or trunk) and “verbal tics” (vocalizations that included grunting, barking, and sometimes shouting profanities). The young neurologist, George Gilles de la Tourette, deﬁned these motor and verbal manifestations of brain dysfunction as a “convulsive tic”—a “maladie des tics.” Following common practice, Tourette presented what was essentially a description of a few clinical cases, extrapolating from these to a hypothesis about causes and treatment. Charcot honored his young colleague for his contribution by renaming the illness “la maladie des tics de Gilles de la Tourette.”
Tourette’s work was of general importance because it also suggested that there were different classes of movement disorders, each possibly with a separate cause and each requiring a speciﬁc approach to therapy. Kushner describes at length the unwelcome impact of Tourette’s presentation on the French medical community. The presentation threatened the established philosophies of several “schools” of neurology and psychiatry, especially psychoanalytic theory. Each of these schools, including that of Charcot and Tourette, had based its conceptualization of tics on an evaluation of a clinical experience, usually involving only a few patients and often another physician’s patient. Thus, the individual physician’s interpretation of a case report, his own case or someone else’s, was the foundation for discussion of causation and treatment. This often sparked lively professional disagreements as did Tourette’s presentation. This speculative and argumentative approach was de rigeur for academic medical discussions of the period, so the hostile reaction to Tourette’s presentation was to be expected.
A CENTURY’S SEARCH FOR THERAPY
Kushner characterizes the last 100 years as devoted to professional debates over the syndrome (collection of symptoms) described by Tourette. Was it a discrete clinical disorder, different from other movement disorders such as chorea? If so, was the motor (movement) manifestation its primary characteristic or secondary to vocalizations? This latter question was pivotal because, depending upon the answer, the disorder would be considered principally psychiatric or principally neurological in origin. With the rising strength of psychoanalytic movements in Europe and North America in the ﬁrst half of the 20th century, the behavioral manifestation position prevailed. Motor manifestations were explained as the expression of a disturbed mind and therapy was directed at treating the psychiatric bases for “immature behavior”: personal guilt over masturbation in the patient and reaction to emotional rigidity in the parent.
Psychotherapy often focused exclusively on the parent because analysts believed that the patient was reacting to parental misdeeds. Similarly, clinicians who championed a brain degenerative or infectious cause of tics also accepted the premise that therapy had to include substantial attention to a disturbed personality, seeing Tourette syndrome as a psychiatric manifestation of an injured brain.
The concept of Tourette’s as primarily a disorder of behavior persisted until the 1980s, undeterred by evidence in the 1950s that intervention with psychoactive drugs brought dramatic relief for the ﬁrst time from both its motor and behavioral manifestations.
The concept of Tourette’s as primarily a disorder of behavior persisted until the 1980s, undeterred by evidence in the 1950s that intervention with psychoactive drugs brought dramatic relief for the ﬁrst time from both its motor and behavioral manifestations. If the psychoanalytic approach to therapy was ﬁxed, so too was the therapeutic approach of the medical communities. Sinus surgery or tonsillectomy to remove sites of supposed lingering infection, antibiotic therapy for streptococcal infection, and prefrontal lobotomy (removal of part of the brain) were claimed to be effective, each based on observational results in a few patients.
In the 1990s, the signiﬁcance of brain-active drugs to control both the physical tics and verbal outbursts of Tourette syndrome patients was recognized. Careful studies demonstrated the value of these drugs so that by the mid 1990s, drug therapy came to dominate treatment; pychotherapy was relegated to a supporting role. This strategy became still more common when Tourette syndrome was linked to obsessive-compulsive behavior and the latter responded more successfully to drug therapy than to psychotherapy.
Kushner also documents complications arising from chauvinistic nationalism. With resources for European medical research nearly absent during and just after World War II, American science became dominant internationally. Leaders of European medical science often felt belittled by this “Americanization” of science, resenting what they saw as domination by American thinking and methodologies. The growing insistence in America of hypothesis-testing as the basis of neurologocal and behavioral science research was not greeted with universal enthusiasm in Europe, particularly in France.
Findings from genetic research done in England and the United States, and the linking of Tourette syndrome with a genetic form of obsessive-convulsive disorder, added fuel to French concerns about Anglo-American scientiﬁc domination. It did not help that English and American scientists tended to assign the methodology of the case report and the case series— still utilized by French academicians as an important basis for understanding and treating Tourette syndrome—to the belittling category of preliminary evidence. Fortunately, these differences in research approach have lost their signiﬁcance as younger clinical scientists and academic leaders, well-trained in laboratory research and controlled clinical trials, have assumed responsibility for brain research internationally. But for many years national pride and professional rivalries complicated and even interfered with the development of needed knowledge about the cause and improved treatment of Tourette syndrome.
ENFRANCHISING THE PATIENT AND FAMILY
The art and science of medicine have long been almost universally the exclusive domain of the physician. Individuals and disciplines might engage in ﬁerce professional rivalries, but only the physician’s opinion mattered in diagnosis, methods of medical care, and establishment of directions and priorities in research. Neither government, society, nor patients were invited to participate. In the Platonic sense, the physician was a benevolent monarch; he knew what was best for patients individually and as a community.
The years after World War II saw a fundamental change in the physician-patient relationship. It is too simple to credit this change solely to reaction to the brutality of the medical procedures fostered by the Nazi regime, although the Helsinki Declaration and other international manifestos calling on all parties to protect the rights of the individual in a clinical or research setting clearly resulted from reaction to the Nazi atrocities. The unveiling of previous abuses of research subjects even in democratic societies, and public anger at the self-serving autocracy of some medical scientists, also contributed to the public demand for more information about, and active participation in, medical and scientiﬁc decision making.
In the United States there was another factor: a cultural pattern of people coming together to further mutual causes and serve the public. Brotherhoods and sisterhoods ﬂourished; and America had long been known as a nation of joiners. The organization of the American Cancer Society and the American Heart Association are examples of how these ways of seeking change were brought to bear on the issues of health care and medical research. Both organizations were started as professional groups, then leadership was taken over by spirited and concerned public leaders with interests in speciﬁc medical areas. In medical research, the March of Dimes provided a dramatic demonstration of leadership on poliomyelitis, combining concern for health care services with success in setting the national agenda for medical research. Establishment of these disease-oriented public organizations became a template for patient-dominated organizations, including the American Tourette Syndrome Association.
These organizations eventually began to work together to demand greater government support of research, particularly in areas of their own primary interest. The logorithmic growth of the National Institutes of Health and expansion of the research agenda at the Centers for Disease Control are direct results of these efforts. But with more government participation inevitably came more public oversight and agenda-setting. The Federal government established operating guidelines in areas previously the exclusive responsibility of physicians and their professional organizations. Today, both medical care and medical research are rapidly being redeﬁned as “public utilities,” with comprehensive regulatory legislation to govern them and public oversight to monitor them.
Patients also gained new leverage as informed consent and individual participation in health care and related research decisions became requirements of public funding. Patients now have a legal as well as moral right to know what clinical intervention the physician is offering, why it is being prescribed, the dangers involved, and available alternatives. The patient thus has become a partner in medical decision making and patients as a group have become a force in setting research priorities and direction. Kushner discusses how these forces shaped the organization and mission of the American Tourette Syndrome Association.
Although the pattern of a concerned group of people organizing to participate in and provide leadership for research and medical services is an American phenomenon, it is now becoming an international movement. In Europe, the general pattern in the past has been that the meeting of a public need was the nearly exclusive responsibility of government. On occasion, a patron—sometimes a royal patron—lent titular support to a cause; however, disease speciﬁc organizations of patients assuming responsibility and exercising authority for their own well being was a rarity. National and sometimes international organizations of patients and their friends are now also being established in Europe to inﬂuence government and professional groups and to provide private resources to meet their own objectives. This “patient empowerment” is a steadily increasing force in the decision making process in both health care and research. The Tourette syndrome public movement in both the United States and Europe is one example of this international development.
Kushner’s book would have beneﬁted from more attention to this compelling force in shaping the future in medicine, research, and society—and to another problem now arising in both the United States and Europe. With so many health disorders and such great need in each, limitations in available personal, patronage, and public and private funding are beginning to foster competition among causes. The earlier pattern of at times destructive rivalries between medical groups is now beginning to reappear in the competition for attention (and authority) in disease-oriented, patient-led organizations. Nevertheless, the progress is real. From the role of passive recipient of the good intentions of the medical establishment, the patient and family have become active partners in making health care decisions and setting research priorities.
A WORD TO READERS
Historians often try to document every available detail of an event, person, or era. They enrich these details by recreating the historical setting that might have inﬂuenced the speciﬁc event. Kushner’s description of historical occurrences surrounding the evolution of concepts about Tourette syndrome accomplishes just that, but only for the medical aspects of Tourette syndrome. He provides only modest amounts of information about government and public forces and their impact on the changes now occurring.
Kushner’s presentation is encyclopedic in medical detail (60 pages of notes and references) and description of the medical environment. To the scholar, this is informative, readable, and useful; to the casual reader, interested but not immersed in the subject, it becomes laborious when events repeat themselves. To the former, I commend this history both for the information it provides and as a model of documenting the history of the medical aspects of a disease. For others, I recommend the last two chapters, where Kushner summarizes medical developments related to Tourette syndrome and lessons to be learned from them. I look forward to a second edition of Prof. Kushner’s book, when medical science will have answered his question: A Cursing Brain?
From A Cursing Brain? The Histories of Tourette Syndrome by Howard I. Kushner. © 1999 by Howard I. Kushner. Reprinted by permission of Harvard University Press.
The Triumph Of Organic Narrative
In 1975, a worried mother thumbed through the October 1975 issue of Today’s Health, a magazine published by the American Medical Association for its member physicians to place in their waiting rooms. Her nine-year-old son, Tommy (a pseudonym), was being treated by a Freudian psychiatrist. Six years previously, when Tommy had continuous eye blinking, his doctor had assured the parents that “nothing was wrong,” that Tommy merely had acquired a “habit.”
The blinking spontaneously disappeared, but a few years later Tommy displayed “a tiny” eye and lip twitch, “a nervous cough,” and “a shrug of the shoulder.” When these too went away, Tommy’s mother, Claire Gold (also a pseudonym), assumed these also were habits similar to his earlier eye blinking. Tommy was an excessively shy child; soon after his mother forced him to attend a schoolmate’s birthday party, he “started to go through a grotesque pattern of movements. He jerked his right arm and leg out at awkward angles to his body.” When ordered to stop, he “kept right on.” Claire was “alarmed and then angry. There’s obviously nothing wrong with him,” she remembered thinking. “He’s just getting back at me, in his childish way, for making him go to the party.” The situation escalated, with Claire demanding that her son control himself and Tommy insisting he could-n’t. Although she had never spanked her son, Claire Gold lost control and slapped Tommy. The gyrations continued. A call to Tommy’s pediatrician led to an examination by a neurologist, who concluded that “the boy’s symptom is most likely an emotional reaction to stress or anxiety.” He told Claire that she shouldn’t “nag and discipline” Tommy “so much about making friends.” Tommy’s father, Jack (also a pseudonym), endorsed the neurologist’s suggestion, increasing Claire’s guilt about having initiated Tommy’s habits.” “Was I actually the cause of my child’s nervous tics?” Claire wondered.
However, within the two weeks, “Tommy the Tic,” as other children now referred to him, “began making noises-grunts, whistles, throat-clearings.” When these increased, Tommy’s pediatrician sent him to a psychiatrist with whom Tommy met “three times a week to talk about his ‘emotional problems.’” But Tommy seemed to get worse. He developed what the psychiatrist called a “patterned tic.” Every few minutes he would lick his lips, wipe his mouth on his sleeve, then his forehead with his hand.” He would also repeat the same word several times. By now neighbors kept their children from playing with Tommy; Claire and Jack’s marriage was becoming severely strained. “Jack and I had no sex life and,” Claire recalled, “absolutely no social life. We couldn’t have company because Tommy’s actions were so bizarre. And, we couldn’t ask a babysitter to stay with him while we went out. And we couldn’t take him out with us because we never knew when he would start to shriek or shout.” Also, the ninety dollars per week for therapy was depleting the young couple’s savings.
As she absent-mindedly stared at the front cover of Today’s Health, Claire saw the headline, “A Nightmarish Disease: Tourette’s Syndrome—Literally—Can Make a Person Scream.” She read the story of an eighteen-year-old boy, Orrin Palmer, whose symptoms and case history were similar to Tommy’s. The condition, called Tourette Syndrome, wrote the article’s author Sally Wendkos Olds, “is physiological rather than psychological in origin...Many researchers feel that a chemical imbalance in the brain, most probably an excess of dopamine, may be at the root of Tourette’s syndrome.” Orrin, reported Olds, was under the care of Dr. Arthur K. Shapiro of Cornell University Medical College in New York City. With his wife, psychologist Elaine Shapiro, Olds continued, he had successfully treated 250 patients with “haloperidol, a drug that often eliminates Tourette’s syndrome.” That was because haloperidol [Haldol] was “known to block the effects of the brain chemical, dopamine...in the basal ganglia, an area associated with the coordination of movement.”
Two decades later Claire would remember how she “sat there stunned” and then “burst into the doctor’s office waving the magazine. ‘Look at this,’ I shouted. ‘It describes all Tommy’s symptoms ...and it says they come from a disease which can be treated with medicine! Why didn’t you know this? Why didn’t you tell me?’” Unfazed, Tommy’s psychiatrist replied, “your son may indeed have Tourette’s syndrome, I considered it but even if that’s the case, I still think it is the result of an emotional disturbance. I think therapy is the answer rather than pills.”
The article in Today’s Health also mentioned the Tourette Syndrome Association that had been founded in 1972 and was located in Bayside, New York. That afternoon, Claire phoned the Association’s office and within a few weeks Tommy was examined by Dr. Shapiro, who “had no doubt that my son was suffering from Tourette’s Syndrome.” And, Claire, reported, “after a week on haloperidol, most of Tommy’s symptoms disappeared.”
Claire Gold’s story, which was published in Good Housekeeping magazine in September 1976, is emblematic, one might even say formulaic, of the numerous newspapers and magazine articles that appeared in the American press from 1971 to 1985. All related a similar story of children with bizarre movements and vocalizations, shunted from pediatrician to psychiatrist, whose parents were told that the behaviors were a result of their parenting. Then, after years of failed psychotherapeutic and behavioral modification treatments, each family learned, as if through some deus ex machina, the truth that these odd behaviors were caused by a chemical imbalance or neurotransmission malfunction, which was most likely amenable to treatment with haloperidol.