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Gilles de la Tourette's syndrome

Tourette syndrome — also called Tourette's syndrome, Tourette's disorder, or Gilles de la Tourette syndrome — is a neurological or neurochemical disorder characterized by tics: involuntary, rapid, sudden movements or vocalizations that occur repeatedly in the same way. more...

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The eponym was bestowed by Jean-Martin Charcot after and on behalf of his resident, Georges Gilles de la Tourette, (1859 - 1904), French physician and neurologist.


Symptoms include multiple motor and one or more vocal tics present at some time during the disorder although not necessarily simultaneously; the occurrence of tics many times a day (usually in bouts) nearly every day or intermittently throughout a span of more than one year; the periodic change in the number, frequency, type and location of the tics, and in the waxing and waning of their severity; symptoms disappearing for weeks or months at a time; and onset before the age of 18.

Vocal tics may fall into various categories, including echolalia (the urge to repeat words spoken by someone else after being heard by the person with the disorder), palilalia (the urge to repeat one's own previously spoken words), lexilalia (the urge to repeat words after reading them) and, most controversially, coprolalia (the spontaneous utterance of socially objectionable or taboo words or phrases, such as obscenities and racial or ethnic slurs). However, according to the Tourette Syndrome Association, Inc., only about 10% of TS patients suffer from this aspect of the condition. There are many other vocal tics besides those categorized by word repetition: in fact, a TS tic can be almost any possible short vocalization, with common vocal tics being throat clearing, coughing, sniffing, grunts, or moans. Motor tics can be of an endless variety and may include hand-clapping, neck stretching, shoulder shrugging, eye blinking, and facial grimacing.

The term "involuntary" has been used to describe TS tics, since it is known that most people with TS do have limited control over the expression of symptoms. Immediately preceding tic onset, individuals with TS experience what is called a "premonitory urge," similar to the feeling that precedes yawning. The control which can be exerted (from seconds to hours at a time) may merely postpone and exacerbate the ultimate expression of the tic. Children may be less aware of the premonitory urge associated with tics than are adults, but their awareness tends to increase with maturity. Tics are experienced as irresistible (like a yawn or sneeze or itch) and must eventually be expressed. People with TS often seek a secluded spot to release their symptoms after delaying them in school or at work. It is not uncommon for children to suppress tics during a visit to the doctor or while at school. Typically, tics increase as a result of tension or stress (but are not solely caused by stress) and decrease with relaxation or concentration on an absorbing task. In fact, neurologist and writer Oliver Sacks has described a man with severe TS who is both a pilot and a surgeon.


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A Cursing Brain? The Histories of Tourette Syndrome. - Review - book review
From Journal of Social History, 3/22/00 by Gerald N. Grob

A Cursing Brain? The Histories of Tourette Syndrome. By Howard I. Kushner (Cambridge, Massachusetts: Harvard University Press, 1999. xii plus 3O3pp. $29.95).

How are diseases identified and defined? Although seemingly simple and straightforward, this question defies easy answers. Before the emergence of the specific germ theory, for example, diseases were defined by their symptoms. Such an approach, however, created insuperable difficulties. In the mid-nineteenth century medical writers generally identified specific diseases by distinguishing between different forms of fever; their nosologies (i.e., classification systems) included more than a hundred such categories (scarlet fever, yellow fever, rheumatic fever, etc.). The result was chaos, if only because fever is virtually a universal symptom and cannot be employed as a means of differentiating specific diseases.

The emergence of the specific germ theory of disease in the late nineteenth century led to a classification system based on etiology rather than symptomatology; infectious diseases were now defined by the presence of an invading organism. The apparent simplicity and rationality of the specific germ theory led to comparable efforts to identify noninfectious diseases in etiological terms. During the past century medical figures have defined numerous disease categories that incorporated descriptions and etiological claims even though supportive data were largely absent.

The evolution of Tourette syndrome is a case in point. Its symptoms--at least in terms of current thinking--include a broad range of vocal and physical tics. In A Cursing Brain Howard I. Kushner imaginatively traces its troubled history. He explores three distinct but related themes: the claims of medical knowledge; the experiences of afflicted individuals; and cultural expectations and assumptions. His book serves as a warning to those who confidently make assertions about the nature and causes of diseases that rest overwhelmingly on speculation or faith.

Kushner begins with the celebrated case of the Marquise de Dampierre, a woman who gained notoriety in the early nineteenth century because of her penchant to introduce obscene words into ordinary conversation, particularly "shit and fucking pig." Her case had been reported in 1825 by Jean Marc Gaspard Itard, the physician who had educated the so-called Wild Child of Aveyron. More than half a century later a young Parisian neurologist, Georges Gilles de la Tourette, selected this case as an example of the illness that he classified as "maladie des tics." The marquise's symptoms became, as Kushner notes, "a vehicle for validating particular medical and philosophical theories of human behavior," (p. 13): free will vs. biological reductionism, psychogenic vs. organic. What is especially noteworthy was the degree to which successive clinicians drew selectively from their predecessors and interpreted or reinterpreted symptoms within their own ideological framework, often ignoring patient narratives.

Tourette's "maladie des tics" was by no means accepted either at the time of its publication in 1885 or for much of the twentieth century. Indeed, the disease virtually disappeared from the medical map for many decades. Some insisted that it was but a manifestation of hysteria; others suggested that multiple tics and cursing (coprolalia) were a subset of movement disorders known as choreas and which were caused by a prior attack of rheumatic fever. Psychogenic explanations also abounded. In their influential Les Tics et leur Traitment (1902), Henry Meige and E. Feindel rejected claims that specific localized cortical lesions caused ticcing behaviors and instead argued that they resulted from bad habits formed during childhood. In the 1920's Sandor Ferenczi developed a purely psychoanalytical explanation, namely, that tics represented a reaction against a repressed libido. Two decades later Margaret S. Mahler, a pediatrician and psychoanalyst who had been influenced by Ferenczi before migrating to the United States from Hungary in 1938, developed an elaborate and influential theory that convulsive tics (which admittedly had an organic substrate) developed only among susceptible children who had experienced repressed familial psychological conflicts. Virtually none of these interpretations of ticcing behavior grew out of the study of individual case histories; rather the latter were selected to prove a prior theoretical commitment.

Despite studies that indicated that ticcing behavior had a somatic etiology, psychogenic explanations remained dominant. Nor did the introduction of haloperidol in the 1960's--a drug that appeared effective in reducing symptoms--diminish faith in the psychoanalytic paradigm. Not until the creation of the Tourette Syndrome Association, a patient support group founded in 1971, did the balance shift toward an organic perspective. The transformation, however, was by no means universal. In France psychoanalytic interpretations continue to dominate, In the United States, by contrast, Tourette syndrome is now regarded as an organic disorder whose origins may be found in genetic, neurochemical, or even immune malfunctions.

Kushner's richly textured and nuanced study is an important contribution and deserves a wide readership among historians, clinicians, and medical researchers. This is not to suggest that he is invulnerable to criticism. His personal commitment to an underlying somaticism, after all, rests in part on faith. There is also little doubt that his own beliefs shape the manner in which he traces the thinking of those physicians who dealt with ticcing disorders; he is partial to those who posited a somatic etiology. At present the jury is still out; the evidence to resolve the mystery of such behaviors is not yet available. Indeed, I remain skeptical of the claim that it is possible to arrive at a full and complete understanding of the nature of human beings and their behavior. Such an understanding, after all, would have to rest on a purely reductionist and deterministic foundation, and thus exclude a variety of moral concerns. These comments notwithstanding, we are all in Kushner's debt for producing a major work of scholarship.

COPYRIGHT 2000 Journal of Social History
COPYRIGHT 2003 Gale Group

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