A Cursing Brain?

The Histories of Tourette Syndrome

Howard I. Kushner

• Publisher: Harvard University Press
• Year: 2000
• Print ISBN: 9780674003866, 9780674180222
• eBook ISBN: 9780674255050
• Topic: Biowissenschaften
• Subtopic: Wissenschaftsgeschichte

Over a century and a half ago, a French physician reported the bizarre behavior of a young aristocratic woman who would suddenly, without warning, erupt in a startling fit of obscene shouts and curses. The image of the afflicted Marquise de Dampierre echoes through the decades as the emblematic example of an illness that today represents one of the fastest-growing diagnoses in North America. Tourette syndrome is a set of behaviors, including recurrent ticcing and involuntary shouting (sometimes cursing) as well as obsessive-compulsive actions. The fascinating history of this syndrome reveals how cultural and medical assumptions have determined and radically altered its characterization and treatment from the early nineteenth century to the present.

A Cursing Brain? traces the problematic classification of Tourette syndrome through three distinct but overlapping stories: that of the claims of medical knowledge, that of patients' experiences, and that of cultural expectations and assumptions. Earlier researchers asserted that the bizarre ticcing and impromptu vocalizations were psychological--resulting from sustained bad habits or lack of self-control. Today, patients exhibiting these behaviors are seen as suffering from a neurological disease and generally are treated with drug therapy. Although current clinical research indicates that Tourette's is an organic disorder, this pioneering history of the syndrome reminds us to be skeptical of medical orthodoxies so that we may stay open to fresh understandings and more effective interventions.

An Elusive Syndrome

1

Seated toward the back of a meeting room in a local hospital I listen to a speaker, a published writer in his early thirties. He periodically turns his head almost rhythmically, first to the left and then to the right, each time forming his lips as if to spit without actually expectorating. Behind me, a young man shouts out “Fuck you! I love you.” A half minute later he bellows, “Sit down, shut up!” Neither the speaker nor any of the fifty or so persons in the audience reacts to any of this behavior. During the question period following the talk a woman sitting in the front row asks the speaker a question, which he answers; but, in the middle of his response he blurts out “Douche bag!” and then continues speaking to the woman as if the sounds had come from somewhere and someone else. She appears neither offended nor surprised by this outburst. Another hand is raised in the audience and for half an hour the speaker deftly handles the questions, always punctuating his responses with parenthetical foul language or remarks that seem so particularly inappropriate as to be purposeful. Thus, responding to a question from a noticeably obese woman, he interjects “Fat pig!” into his remarks. Meanwhile, the man in the back of the room continues to shout obscenities, as if competing for attention. Still, no one in the audience seems offended or even appears to notice the bizarre and offensive behavior of the speaker or his interlocutor.

The speaker who had been introduced at the outset as having a disorder called Gilles de la Tourette syndrome (TS) was addressing a support group comprised mainly of parents of diagnosed children.¹ The man in the audience shouting obscenities has been afflicted since his early teens, and as his neighbor for fifteen years, I had watched his symptoms develop from uncontrolled eye blinking to more pronounced facial and body tics accompanied by vocalizations that at first sounded like a person muttering to himself. When “Michael,” as I will call him, approached his late teens, he began to curse and blurt out uncontrollable inappropriate remarks that made it difficult for him to socialize with peers or with anyone unaware of the nature of his offensive behavior. Even ordinary tasks became dangerous. Once, phoning an airline to make a reservation, Michael blurted out, “There’s a bomb on the plane!” The next day the FBI appeared at his door to question a “suspected terrorist.” Sometimes, when Michael is introduced to an African-American, he cannot stop himself from exclaiming, “Nigger!”

The speaker recounts similar experiences, one of which resulted in his removal from an airplane after he failed to abide a flight attendant’s request that he cease cursing. His explanation that he suffered from a neurological disorder known as Tourette syndrome did not win sympathy from the attendant or other passengers. Michael, likewise, has been evicted from two apartments, has been served restraining orders barring him from local fast-food restaurants, and has been involved in litigation as a result of a landlord’s complaint that Michael allegedly threatened to “drop a bowling ball from an airplane” on the man. Like the speaker, Michael attempts to explain to those with whom he comes into contact that his cursing and strange vocalizations are the result of Tourette syndrome. Nevertheless, the content and timing of these outbursts can be discomforting to anyone who is the target or for those who witness them. On the one hand, they are apt but painful characterizations, such as blurting out what may be obvious, but tasteless or tactless; for instance, exclaiming to an obese person that they are “fat.” On the other hand, these unrestrained words and phrases can be threatening, as when Michael interjects “I want to rape you” when talking to a woman.

Such reactions influenced a local municipal court judge’s decision to uphold an order restraining Michael from visiting a neighboring apartment complex. The judge refused to believe that a disease could account for Michael’s cursing and threatening outbursts. At a subsequent hearing only the presence of a television news crew persuaded the judge to moderate his order. The judge’s good will was, no doubt, stretched thin by Michael’s periodic outburst of “I’ll kill you, I love you!”

It is true that Michael has difficulty controlling his temper; thus, when particularly frustrated by the effects of his behavior or others’ reactions to it, Michael sometimes will punch or kick a wall or damage fixtures in his apartment. Michael has never physically harmed others. However, many parents report that their afflicted children do sometimes hit, punch, and kick their siblings and their pets. While not apparent in either Michael or the speaker, other florid symptoms beyond cursing, which is clinically referred to as “coprolalia,” include “copropraxia,” the acting out of explicitly sexual gestures or displays; “echolalia,” repetition of one’s own or others’ words or phrases; and “echopraxia,” imitation of others’ behaviors or actions.

These are the florid symptoms. Most persons diagnosed with Gilles de la Tourette syndrome neither curse nor display inappropriate sexual behavior, although they often have difficulty controlling their tempers. Typically, sufferers develop involuntary motor movements, referred to as “tics,” during early childhood (ages seven to nine). The motor tics, which occur frequently throughout the day, generally involve head and neck jerking, eye blinking, tongue protrusions, shoulder shrugs, and various torso and limb movements. A diagnosis of Tourette syndrome requires one or more vocal tics to have been present for some time. These may include barks, grunts, yelps, and coughs. Tics and vocalizations appear suddenly and characteristically are rapid, recurrent, non-rhythmic, and stereotyped. Often these symptoms are coupled with obsessive and compulsive behaviors, such as a repeated series of actions that must be performed before entering or leaving a room. Tics wax and wane, often increasing in frequency and complexity, with later tics replacing earlier ones.² Motor movements, however, can be quite debilitating and painful. For instance, aside from the muscle strain caused by severe head-jerking, tics can make reading an arduous, if not impossible task.³ Sometimes the tics disappear completely and never recur. Often, however, they merely remit, returning later in slightly different form with renewed force.

Commonly, parents of children with TS report their long and difficult journey through a medical maze in search of a diagnosis for their children’s behavior. It is not at all unusual that the diagnosis is finally obtained when parents, armed with literature from newspapers, magazines, or from the Tourette Syndrome Association, educate their physicians about the syndrome. Resistance to labeling TS behavior as a disease, however, flows as much from the nature of its most florid symptoms as from a general lack of awareness. Like the municipal judge in Michael’s case, even physicians, not least of all psychiatrists, have been reluctant to diagnose seemingly obvious antisocial behavior, such as inappropriate cursing or explicitly sexual displays, as an organic disorder. The controversy over whether these behaviors constitute an illness has a long history. Exploration of that history reveals much about the continuing reluctance to validate inappropriate social behaviors as having organic causes.

Once thought to be a rare occurrence, Tourette syndrome represents one of the fastest growing diagnoses in North America, with a prevalence of from 2.9 to 5.2 per 10,000.⁴ A British team recently reported that 3 percent of thirteen- to fourteen-year-old secondary schoolchildren (299 per 10,000) fit the Diagnostic and Statistical Manual of Mental Disorders-III-R (DSM-III-R) classification of Tourette syndrome, which is four times higher than the previous highest rates reported in 1990.⁵ All studies find that males are four to five times more likely to become afflicted than females. One recent investigation reported that among boys the prevalence may be as high as 5 per 100.⁶ Even at the low end, estimates would make the prevalence of Tourette syndrome more than double that of teen suicide in the United States.⁷

Part of the reason for contradictory findings about prevalence is attributable to physicians’ and researchers’ disagreements about which symptoms to include and which to exclude in their definitions of Tourette’s. That is because TS is a syndrome rather than a disease. Although the term “disease” can mean many things, including simply “distress,” a disorder generally only graduates from its status as a syndrome when its underlying pathological causes or “etiologies,” as complex as they may be, are uncovered. Measles, polio, smallpox, and sickle-cell anemia are labeled as diseases and not as syndromes because a tentative diagnosis based on signs and symptoms is confirmed or rejected through a laboratory test that indicates infection by a pathogen or the presence of an enabling gene. In contrast, the cause of a syndrome remains unknown.⁸ With syndromes, like Tourette’s, schizophrenia, and chronic fatigue, diagnoses depend on identification of a list of possible combinations of signs and symptoms that a person must display over an assigned period of time.⁹ But as Terra Ziporyn notes, this list may vary from physician to physician: “One researcher, for example, may have defined chronic fatigue syndrome as including anyone who feels tired most of the time, while her colleague may have restricted it to persistent fatigue coupled with flu symptoms and perhaps even associated with a virus . . . Without an agreed-on definition, more sophisticated correlations and predictions become meaningless. If you don’t know what chronic fatigue syndrome is, for example, how can you say what happens to people with chronic fatigue syndrome?”¹⁰ The signs and symptoms that have been grouped together as Tourette’s often seem to fit a spectrum, in which a single cause can result in a series of different symptoms. Alternatively, because its underlying pathology remains unknown, these signs and symptoms could result from a variety of disparate causes.¹¹

Grouping certain symptoms, but not others, under a single “name” or category inevitably influences practitioners’ and patients’ understanding of the possible causes and course of a disorder. The purpose of syndrome construction is to focus a practitioner’s view of a patient’s illness by privileging certain symptoms while downplaying others. What physicians believe constitute the legitimate symptoms of a disorder can have a profound influence on a sufferer’s experience. Certainly it influences which symptoms become the focus for treatment and which are viewed as unrelated to the syndrome.

Since the late nineteenth century, when Tourette’s was initially described, psychiatrists have disagreed, often vigorously, over which symptoms and behaviors should be included as part of TS. These controversies continue. Beginning in the 1980s a serious dispute arose among experts over whether obsessive-compulsive behaviors should be included within the Tourette’s typology.¹² More recently a division has emerged among those who believe that conduct disorders are part of TS and those who resist too wide a spectrum of symptoms.¹³ In addition, clinicians disagree over the length of time a symptom must be present to be considered for a diagnosis and whether or not onset must occur before the age of eighteen.

Although one of the purposes of the American Psychiatric Association’s diagnostic handbook, the DSM, is to provide uniform definitions of psychiatric syndromes, a comparison of the definitions and classifications of Tourette syndrome in the revisions of DSM (III-R) reveals that there has been no absolute agreement over time about what symptoms are necessary or how long they must persist for a diagnosis of Tourette syndrome.¹⁴ A new effort at a uniform definition was made in the construction of Tourette’s categories in the DSM-IV (1994),¹⁵ but dissension over the typology erupted among both members of the committee that had been commissioned to write this section, as well as among other experts as soon as the volume appeared.¹⁶ By 1997 a team of experienced Tourette’s researchers at Yale University Medical School’s Child Study Center admitted that continuing disagreements over what constitutes the symptoms that are part of TS (its phenotype) continue to frustrate attempts to locate its underlying pathogenesis (causes).¹⁷

Nevertheless, the vast majority of researchers are persuaded that, no matter what its constituents, Tourette’s is an organic disorder. The most persuasive evidence for a physiological basis of Tourette’s is that motor tics and involuntary vocalizations can often be controlled by drugs that act to suppress the transmission of the neurotransmitter dopamine in that part of the brain called the basal ganglia, which is responsible for certain motor movements. However, these drugs can only provide relief from the symptoms of Tourette’s and do not eliminate the causes of the tics and vocalizations. Researchers continue to search for the underlying causes, which may reside in a genetic malfunction or an autoimmune reaction to a previous infection, or a combination of these and other cofactors.

Even as persuasive evidence mounts tying specific ticcing and obsessive-compulsive symptoms to organic mechanisms, a number of clinicians have resisted a biologically reductionist explanation of Tourette’s. They point to the elusive quality of many symptoms that wax and wane for no apparent reason, sometimes disappearing completely. Because some of those afflicted are able to suppress their tics and vocalizations, releasing them minutes later away from the observation of others, skeptics are unpersuaded that TS behaviors are completely involuntary. Alternatively, these temporary suppressions could be understood as elaborate strategies, as Michael has attempted, substituting less offensive words when unable to resist the urge to curse. Oliver Sacks’s portrayal of a ticcing drummer, “Witty Ticcy Ray,” suggests that some are able to turn their symptoms into socially sanctioned behaviors.¹⁸ But Sacks’s description of a Canadian surgeon with florid TS symptoms, who is able to suspend his tics while performing operations or when flying his airplane, no doubt raises questions in many minds about the assertions that these behaviors are unintentional.¹⁹

Citing these and other examples of the seeming ability of some diagnosed with Tourette’s to control their behaviors, a few observers insist that psychological conflicts play a crucial role in the onset and perpetuation of ticcing symptoms. Claiming that psychological factors influence the way that biochemistry and brain circuitry operate, this minority continues to frame physiological symptoms as manifestations of psychological conflicts.²⁰ Most often those who resist organic explanations point to cursing as evidence in support of their conclusions.

Although cursing is not present in every case of TS, this symptom, more than any other, periodically has brought Gilles de la Tourette syndrome to medical and popular notice. In fact, this odd behavior had intrig...

Table of contents

• Cover
• Title
• Copyright
• Dedication
• Contents
• Preface
• Note on Terms
• 1 An Elusive Syndrome
• 2 The Case of the Cursing Marquise
• 3 A Disputed Illness
• 4 The Case of “O.” and the Emergence of Psychoanalysis
• 5 Competing Claims
• 6 The Disappearance of Tic Illness
• 7 Margaret Mahler and the Tic Syndrome
• 8 Haloperidol and the Persistence of the Psychogenic Frame
• 9 The French Resistance
• 10 The Triumph of the Organic Narrative
• 11 Clashing Cultural Conceptions
• 12 Clinical Lessons
• Glossary
• Notes
• Acknowledgments
• Index