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Tourette syndrome

2007 Schools Wikipedia Selection. Related subjects: Health and medicine

   CAPTION: Tourette syndrome
   Classifications and external resources

   Georges Gilles de la Tourette (1859 – 1904)
     ICD- 10   F 95.2
     ICD- 9    307.23
      OMIM     137580
   DiseasesDB  5220
   MedlinePlus 000733
    eMedicine  med/3107  neuro/664

   Tourette syndrome (also called Tourette's syndrome, Tourette's
   disorder, Gilles de la Tourette syndrome, GTS or the more common
   Tourette's or TS) is an inherited neurological disorder with onset in
   childhood, characterized by the presence of multiple physical (motor)
   tics and at least one vocal (phonic) tic; these tics characteristically
   wax and wane. Tourette's is defined as part of a spectrum of tic
   disorders, which includes transient and chronic tics.

   Tourette's was once considered a rare and bizarre syndrome, most often
   associated with the exclamation of obscene words or socially
   inappropriate and derogatory remarks ( coprolalia). However, this
   symptom is present in only a small minority of people with Tourette's.
   Tourette's is no longer considered a rare condition, but it may not
   always be correctly identified because most cases are classified as
   mild. Since the incidence may be as high as one in a hundred people, up
   to 530,000 U.S. school-age children may have Tourette's, with the more
   common tics of eye blinking, coughing, throat clearing, sniffing, and
   facial movements. People with Tourette's have normal life expectancy
   and intelligence. The severity of the tics decreases for most children
   as they pass through adolescence, and extreme Tourette's in adulthood
   is a rarity. Notable individuals with Tourette's are found in all walks
   of life.

   Genetic and environmental factors each play a role in the etiology of
   Tourette's, but the exact causes are unknown. In most cases, medication
   is unnecessary. There is no effective medication for every case of
   tics, but there are medications and therapies that can help when their
   use is warranted. Explanation and reassurance alone are often
   sufficient treatment; education is an important part of any treatment
   plan.

   The eponym was bestowed by Jean-Martin Charcot (1825–93) on behalf of
   his resident, Georges Albert Édouard Brutus Gilles de la Tourette
   (1859–1904), a French physician and neurologist, who published an
   account of nine patients with Tourette's in 1885.

Classification

   Tics are sudden, repetitive, stereotyped, nonrhythmic, involuntary
   movements (motor tics) and utterances (phonic tics) that involve
   discrete muscle groups. Motor tics are movement-based tics, while
   phonic tics are involuntary sounds produced by moving air through the
   nose, mouth, or throat.

   Tourette's is one of several tic disorders, which are classified by the
   Diagnostic and Statistical Manual of Mental Disorders (DSM) according
   to type (motor or phonic tics) and duration (transient or chronic).
   Transient tic disorder consists of multiple motor tics, phonic tics or
   both, with a duration of between four weeks and twelve months. Chronic
   tic disorder is either single or multiple, motor or phonic tics (but
   not both), which are present for more than a year. Tourette's is
   diagnosed when multiple motor tics, and at least one phonic tic, are
   present for more than a year. Tic disorders are defined similarly by
   the World Health Organization (ICD-10 codes).

   Although Tourette's is the more severe expression of the spectrum of
   tic disorders, most cases are mild. The severity of symptoms varies
   widely among people with Tourette's, and mild cases may be undetected.

Characteristics

   Tics are movements or sounds "that occur intermittently and
   unpredictably out of a background of normal motor activity", having the
   appearance of "normal behaviors gone wrong." The tics associated with
   Tourette's constantly change in number, frequency, severity and
   anatomical location. Waxing and waning—the ongoing increase and
   decrease in severity and frequency of tics—occurs differently in each
   individual. Tics also occur in "bouts of bouts", which vary for each
   person.

   Coprolalia (the spontaneous utterance of socially objectionable or
   taboo words or phrases) is the most publicized symptom of Tourette's,
   but it is not required for a diagnosis of Tourette's. According to the
   Tourette Syndrome Association, fewer than 15% of Tourette's patients
   exhibit coprolalia. Echolalia (repeating the words of others) and
   palilalia (repeating one's own words) occur in a minority of cases,
   while the most common initial motor and vocal tics are, respectively,
   eye blinking and throat clearing.

                                                       Video clips of tics
                                                HBO documentary video clip
                                                   CBS News video clip
                                                    News10 video clip

   In contrast to the stereotyped movements of other movement disorders
   (e.g. choreas, dystonias, myoclonus, and dyskinesias), the tics of
   Tourette's are temporarily suppressible and preceded by a premonitory
   urge. Immediately preceding tic onset, most individuals with Tourette's
   are aware of an urge that is similar to the need to sneeze or scratch
   an itch. Individuals describe the need to tic as a buildup of tension
   which they consciously choose to release, as if they "had to do it".
   Examples of the premonitory urge are the feeling of having something in
   one's throat, or a localized discomfort in the shoulders, leading to
   the need to clear one's throat or shrug the shoulders. The actual tic
   may be felt as relieving this tension or sensation, similar to
   scratching an itch. Another example is blinking to relieve an
   uncomfortable sensation in the eye. These urges and sensations,
   preceding the expression of the movement or vocalization as a tic, are
   referred to as "premonitory sensory phenomena". Published descriptions
   of the tics of Tourette's identify sensory phenomena as the core
   symptom of the syndrome, even though they are not included in the
   diagnostic criteria.

   Tics are described as semi-voluntary or "unvoluntary", because they are
   not strictly involuntary—they may be experienced as a voluntary
   response to the unwanted, premonitory urge. A unique aspect of tics,
   relative to other movement disorders, is that they are suppressible yet
   irresistible; they are experienced as an irresistible urge that must
   eventually be expressed. People with Tourette's are sometimes able to
   suppress their tics to some extent for limited periods of time, but
   doing so often results in an explosion of tics afterward. People with
   Tourette's may seek a secluded spot to release their symptoms, or there
   may be a marked increase in tics, after a period of suppression at
   school or at work.

   Some people with Tourette's may not be aware of the premonitory urge.
   Children may be less aware of the premonitory urge associated with tics
   than are adults, but their awareness tends to increase with maturity.
   They may have tics for several years before becoming aware of
   premonitory urges. Children may suppress tics while in the doctor's
   office, so they may need to be observed while they are not aware they
   are being watched. The ability to suppress tics varies among
   individuals, and may be more developed in adults than children.

   Although there is no such thing as a "typical" case of Tourette
   syndrome, the condition follows a fairly reliable course in terms of
   the age of onset and the history of the severity of symptoms. Tics may
   appear up to the age of eighteen, but the most typical age of onset is
   from five to seven. The ages of highest tic severity are eight to
   twelve (average ten), with tics steadily declining for most patients as
   they pass through adolescence. The most common, first-presenting tics
   are eye blinking, facial movements, sniffing and throat clearing.
   Initial tics present most frequently in midline body regions where
   there are many muscles, usually the head, neck and facial region. This
   can be contrasted with the stereotyped movements of other disorders
   (such as stims and stereotypies of the autism spectrum disorders),
   which typically have an earlier age of onset, are more symmetrical,
   rhythmical and bilateral, and involve the extremities (e.g., flapping
   the hands). Tics that appear early in the course of the condition are
   frequently confused with other conditions, such as allergies, asthma,
   and vision problems: pediatricians, allergists and ophthalmologists are
   typically the first to see a child with tics.

   Among patients whose symptoms are severe enough to warrant referral to
   clinics, obsessive-compulsive disorder (OCD) and attention-deficit
   hyperactivity disorder (ADHD) are often associated with Tourette's. Not
   all persons with Tourette's have ADHD or OCD or other comorbid
   conditions (co-occurring diagnoses other than Tourette's), although in
   clinical populations, a high percentage of patients presenting for care
   do have ADHD. One author reports that a ten-year overview of patient
   records revealed about 40% of patients with Tourette's have "TS-only"
   or "pure TS", referring to Tourette syndrome in the absence of ADHD,
   OCD and other disorders. Another author reports that 57% of 656
   patients presenting with tic disorders had uncomplicated tics, while
   43% had tics plus comorbid conditions. "Full-blown Tourette's" is a
   term used to describe patients who have significant comorbid conditions
   in addition to tics.

Causes

   The exact cause of Tourette's is unknown, but it is well established
   that both genetic and environmental factors are involved. Genetic
   studies have proved that the overwhelming majority of cases of
   Tourette's are inherited, although the exact mode of inheritance is not
   yet known, and no gene has been identified. In some cases, tics may not
   be inherited; these cases are identified as "sporadic" Tourette
   syndrome (also known as tourettism) because a genetic link is missing.
   Brain structures implicated in Tourette's syndrome
   Enlarge
   Brain structures implicated in Tourette's syndrome

   A person with Tourette's has about a 50% chance of passing the gene(s)
   to one of his or her children, but Tourette's is a condition of
   variable expression and incomplete penetrance. Thus, not everyone who
   inherits the genetic vulnerability will show symptoms; even close
   family members may show different severities of symptoms, or no
   symptoms at all. The gene(s) may express as Tourette's, as a milder tic
   disorder (transient or chronic tics), or as obsessive compulsive
   symptoms without tics. Only a minority of the children who inherit the
   gene(s) have symptoms severe enough to require medical attention.
   Gender appears to have a role in the expression of the genetic
   vulnerability; males are more likely than females to express tics.

   Non-genetic, environmental, infectious, or psychosocial factors—while
   not causing Tourette's—can influence its severity. Autoimmune processes
   may affect tic onset and exacerbation in some cases. The unproven and
   contentious hypothesis that Pediatric Autoimmune Neuropsychiatric
   Disorders Associated with Streptococcal infections ( PANDAS) plays a
   role in the onset of tic disorders and OCD is a current focus of
   research.

   The exact mechanism affecting the inherited vulnerability to Tourette's
   has not been established, and the precise etiology is unknown. Tics are
   believed to result from dysfunction in cortical and subcortical
   regions, the thalamus, basal ganglia and frontal cortex. Neuroanatomic
   models implicate failures in circuits connecting the brain's cortex and
   subcortex, and imaging techniques implicate the basal ganglia and
   frontal cortex.

   Some forms of OCD may be genetically linked to Tourette's. A subset of
   OCD is thought to be etiologically related to Tourette's and may be a
   different expression of the same factors that are important for the
   expression of tics. The genetic relationship of ADHD to Tourette
   syndrome, however, has not been fully established.

Diagnosis

   According to the fourth edition of the Diagnostic and Statistical
   Manual of Mental Disorders ( DSM-IV-TR), Tourette’s Disorder may be
   diagnosed when a person exhibits both multiple motor and one or more
   vocal tics (although these do not need to be concurrent) over the
   period of a year, with no more than three consecutive tic-free months.
   Previous editions of the DSM included a requirement for "marked
   distress or significant impairment in social, occupational or other
   important areas of functioning", but this requirement was removed in
   the most recent update of the manual, in recognition that clinicians
   see patients who meet all the other criterion for Tourette's, but do
   not have distress or impairment. The onset must have occurred before
   the age of 18, and cannot be attributed to the "direct physiological
   effects of a substance or a general medical condition". Hence, other
   medical conditions that include tics or tic-like movements—such as
   autism or other causes of tourettism—must be ruled out before
   conferring a Tourette's diagnosis.

   There are no specific medical or screening tests that can be used in
   diagnosing Tourette's. The diagnosis is made based on observation of
   the individual's symptoms and family history, and after ruling out
   secondary causes of tic disorders. In patients with a typical onset and
   a family history of tics or obsessive–compulsive disorder, a basic
   physical and neurological examination may be sufficient.

   If a physician believes that there may be another condition present
   that could explain tics, tests may be ordered as necessary to rule out
   that condition. An example of this is when diagnostic confusion between
   tics and seizure activity exists, which would call for an EEG, or if
   there are symptoms that indicate an MRI to rule out brain
   abnormalities. TSH levels can be measured to rule out hypothyroidism,
   which can be a cause of tics. Brain imaging studies are not usually
   warranted. In teenagers and adults presenting with a sudden onset of
   tics and other behavioural symptoms, a urine drug screen for cocaine
   and stimulants might be necessary. If a family history of liver disease
   is present, serum copper and ceruloplasmin levels can rule out Wilson's
   disease. However, most cases are diagnosed by merely observing a
   history of tics.

   Secondary causes of tics (not related to inherited Tourette syndrome)
   are commonly referred to as tourettism. Dystonias, choreas, other
   genetic conditions, and secondary causes of tics should be ruled out in
   the differential diagnosis for Tourette syndrome. Other conditions that
   may manifest tics or stereotyped movements include developmental
   disorders, autism spectrum disorders, and stereotypic movement
   disorder; other genetic conditions such as Huntington's disease,
   neuroacanthocytosis, Hallervorden-Spatz syndrome, idiopathic dystonia,
   Duchenne muscular dystrophy, Wilson's disease, Sydenham's chorea and
   tuberous sclerosis. Other possibilities include chromosomal disorders
   such as Down syndrome, Klinefelter's syndrome, XYY syndrome and fragile
   X syndrome. Acquired causes of tics include drug-induced tics, head
   trauma, encephalitis, stroke, and carbon monoxide poisoning. The
   symptoms of Lesch-Nyhan syndrome may also be confused with Tourette
   syndrome. Most of these conditions are rarer than tic disorders, and a
   thorough history and examination may be enough to rule them out,
   without medical or screening tests.

Screening

   Although not all people with Tourette's have comorbid conditions, most
   Tourette's patients presenting for clinical care at specialty referral
   centers may exhibit symptoms of other conditions along with their motor
   and phonic tics. Associated conditions include attention-deficit
   hyperactivity disorder (ADD or ADHD), obsessive–compulsive disorder
   (OCD), learning disabilities and sleep disorders. Disruptive behaviors,
   impaired functioning, or cognitive impairment in patients with comorbid
   Tourette's and ADHD may be accounted for by the comorbid ADHD,
   highlighting the importance of identifying and treating comorbid
   conditions. Disruption from tics is commonly overshadowed by comorbid
   conditions that present greater interference to the child. Tic
   disorders in the absence of ADHD do not appear to be associated with
   disruptive behaviour or functional impairment, while impairment in
   school, family, or peer relations is greater in patients who have more
   comorbid conditions and often determines whether therapy is needed.

   Because comorbid conditions such as OCD and ADHD can be more impairing
   than tics, these conditions are included in an evaluation of patients
   presenting with tics. "It is critical to note that the comorbid
   conditions may determine functional status more strongly than the tic
   disorder." The initial assessment of a patient referred for a tic
   disorder should include a thorough evaluation, including a family
   history of tics, ADHD, obsessive–compulsive symptoms, and other chronic
   medical, psychiatric and neurological conditions. Children and
   adolescents with TS who have learning difficulties are candidates for
   psychoeducational testing, particularly if the child also has ADHD.
   Undiagnosed comorbid conditions may result in functional impairment,
   and it is necessary to identify and treat these conditions to improve
   functioning. Complications may include depression, sleep problems,
   social discomfort and self-injury.

Management

   Clonidine (or the clonidine patch) is one of the medications typically
   tried first when medication is needed for Tourette's.
   Enlarge
   Clonidine (or the clonidine patch) is one of the medications typically
   tried first when medication is needed for Tourette's.

   The treatment of Tourette's focuses on identifying and helping the
   individual manage the most troubling or impairing symptoms. Most cases
   of Tourette's are mild, and do not require pharmacological treatment;
   instead, psychobehavioral therapy, education, and reassurance may be
   sufficient. Treatments, where warranted, can be divided into those that
   target tics and comorbid conditions, which, when present, are often a
   larger source of impairment than the tics themselves. Not all people
   with tics have comorbid conditions, but when those conditions are
   present, they often take treatment priority.

   There is no cure for Tourette's and no medication that works
   universally for all individuals without significant adverse effects.
   Knowledge, education and understanding are uppermost in management
   plans for tic disorders. The management of the symptoms of Tourette's
   may include pharmacological, behavioural and psychological therapies.
   While pharmacological intervention is reserved for more severe
   symptoms, other treatments (such as supportive psychotherapy or
   cognitive behavioural therapy) may help to avoid or ameliorate
   depression and social isolation, and to improve family support.
   Educating a patient, family, and surrounding community (such as
   friends, school, and church) is a key treatment strategy.
   Space-filling representation of a haloperidol molecule. Haloperidol is
   an antipsychotic medication sometimes used to treat severe cases of
   Tourette's.
   Enlarge
   Space-filling representation of a haloperidol molecule. Haloperidol is
   an antipsychotic medication sometimes used to treat severe cases of
   Tourette's.

   Medication is available to help when symptoms interfere with
   functioning. The classes of medication with the most proven efficacy in
   treating tics— typical and atypical neuroleptics including risperidone
   (trade name Risperdal), ziprasidone (Geodon), haloperidol (Haldol),
   pimozide (Orap) and fluphenazine (Prolixin)—can have long-term and
   short-term adverse effects. The antihypertensive agents clonidine
   (trade name Catapres) and guanfacine (Tenex) are also used to treat
   tics; studies show variable efficacy, but a lower side effect profile
   than the neuroleptics. Stimulants and other medications may be useful
   in treating ADHD when it co-occurs with tic disorders. Drugs from
   several other classes of medications can be used when stimulant trials
   fail, including guanfacine (trade name Tenex), atomoxetine (Strattera)
   and tricyclics. Clomipramine (Anafranil), a tricyclic antidepressant,
   and SSRIs—a class of antidepressants including fluoxetine (Prozac),
   sertraline (Zoloft), and fluvoxamine (Luvox)—may be prescribed when a
   Tourette's patient also has symptoms of obsessive–compulsive disorder.

   Because children with tics often present to physicians when their tics
   are most severe, and because of the waxing and waning nature of tics,
   it is recommended that medication not be started immediately or changed
   often. Frequently, the tics subside with explanation, reassurance,
   understanding of the condition and a supportive environment. When
   medication is used, the goal is not to eliminate symptoms: it should be
   used at the lowest possible dose that manages symptoms without adverse
   effects, given that these may be more disturbing than the symptoms for
   which they were prescribed.

   Cognitive behavioural therapy (CBT) is a useful treatment when OCD is
   present, and there is increasing evidence supporting the use of habit
   reversal in the treatment of tics. Relaxation techniques, such as
   exercise, yoga or meditation, may be useful in relieving the stress
   that may aggravate tics, but the majority of behavioural interventions
   (such as relaxation training and biofeedback, with the exception of
   habit reversal) have not been systematically evaluated and are not
   empirically supported therapies for Tourette's.

Prognosis

   Samuel Johnson (1709 – 1784) circa 1772. Johnson wrote A Dictionary of
   the English Language in 1747, and was a prolific writer, poet, and
   critic who had Tourette syndrome.
   Enlarge
   Samuel Johnson (1709 – 1784) circa 1772. Johnson wrote A Dictionary of
   the English Language in 1747, and was a prolific writer, poet, and
   critic who had Tourette syndrome.

   Tourette syndrome is a spectrum disorder—its severity ranges over a
   spectrum from mild to severe. The majority of cases are mild and
   require no treatment. In these cases, the impact of symptoms on the
   individual may be mild, to the extent that casual observers might not
   know of their condition. The overall prognosis is positive, but a
   minority of children with Tourette syndrome have severe symptoms that
   persist into adulthood. A study of 46 subjects at 19 years of age found
   that the symptoms of 80% had minimum to mild impact on their overall
   functioning, and that the other 20% experienced at least a moderate
   impact on their overall functioning. The rare minority of severe cases
   can inhibit or prevent individuals from holding a job or having a
   fulfilling social life. In a follow-up study of thirty-one adults with
   Tourette's, all patients completed high school, 52% finished at least
   two years of college, and 71% were full-time employed or were pursuing
   higher education.

   Regardless of symptom severity, individuals with Tourette's can expect
   to live a normal life span. Although the symptoms may be lifelong and
   chronic for some, the condition is not degenerative or
   life-threatening. Intelligence is normal in those with Tourette's,
   although there may be learning disabilities. There is no reliable means
   of predicting the outcome for a particular individual. The gene or
   genes associated with Tourette's have not been identified, and there is
   no potential "cure".

   Several studies have demonstrated that the condition in most children
   improves with maturity. Tics may be at their highest severity at the
   time that they are diagnosed, and often improve with understanding of
   the condition by individuals and their families and friends. The
   statistical age of highest tic severity is typically between eight and
   twelve, with most individuals experiencing steadily declining tic
   severity as they pass through adolescence. One study showed no
   correlation with tic severity and the onset of puberty, in contrast
   with the popular belief that tics increase at puberty. In many cases, a
   complete remission of tic symptoms occurs after adolescence. However, a
   study using videotape to record tics in adults found that, although
   tics diminished in comparison with childhood, and all measures of tic
   severity improved by adulthood, 90% of adults still had tics. Half of
   the adults who considered themselves tic-free still displayed evidence
   of tics.

   It is not uncommon for the parents of affected children to be unaware
   that they, too, may have had tics as children. Because Tourette's tends
   to subside with maturity, and because milder cases of Tourette's are
   now more likely to be recognized, the first realization that a parent
   had tics as a child may not come until their offspring is diagnosed. It
   is not uncommon for several members of a family to be diagnosed
   together, as parents bringing children to a physician for an evaluation
   of tics become aware that they, too, had tics as a child.

   Children with Tourette's may suffer socially if their tics are viewed
   as "bizarre". If a child has disabling tics, or tics that interfere
   with social or academic functioning, supportive psychotherapy or school
   accommodations can be helpful. Because comorbid conditions (such as
   ADHD or OCD) can cause greater impact on overall functioning than tics,
   a thorough evaluation for comorbidity is called for when symptoms and
   impairment warrant.

   A supportive environment and family generally gives those with
   Tourette's the skills to manage the disorder. People with Tourette's
   may learn to camouflage socially inappropriate tics or to channel the
   energy of their tics into a functional endeavor. Accomplished
   musicians, athletes, public speakers, and professionals from all walks
   of life are found among people with Tourette's. Outcomes in adulthood
   are associated more with the perceived significance of having severe
   tics as a child than with the actual severity of the tics. A person who
   was misunderstood, punished, or teased at home or at school will fare
   worse than children who enjoyed an understanding and supportive
   environment.

   A controlled study on a small group of individuals with Tourette's
   found that young people with the syndrome have greater cognitive
   control over movements than their age-matched controls, possibly
   because the need to suppress tics results in a more efficient control
   over inhibitory movements, a skill that confers an advantage when
   switching between tasks. Children with Tourette's (in the absence of
   comorbid ADHD) performed more accurately on a goal-oriented
   eye-movement task, requiring active inhibition of automatic eye
   movements, and they made fewer errors than their unaffected peers,
   suggesting compensatory changes in the brain resulting in greater
   cognitive control.

Epidemiology

   Tourette syndrome has historically been described as a rare disorder,
   with about 5 to 10 people in 10,000 having the condition. However,
   multiple studies published since 2000 demonstrate that the prevalence
   is much higher than previously thought, and that Tourette syndrome can
   no longer be considered rare. Contemporary prevalence estimates range
   from 1 to 3 per 1,000 to 10 per 1,000; the latter yields an estimate of
   530,000 school-age children with Tourette's in the United States, based
   on 2000 US census data.

   A large, community-based study suggested that over 19% of school-age
   children have tics, with almost 4% of children in regular education
   fulfilling the diagnostic criteria for Tourette syndrome. The children
   with tic disorders in that study were usually undiagnosed. As many as 1
   in 100 people may experience some form of tic disorder, which includes
   transient tics, chronic tics, or Tourette syndrome. Tourette syndrome
   is found among all social, racial and ethnic groups, and males are
   affected 3 to 4 times more often than females.

   Tourette syndrome is frequently misdiagnosed or underdiagnosed, partly
   because of the wide expression of severity, ranging from mild (the
   majority of cases) or moderate, to severe (the rare, but more
   widely-recognized and publicized cases). Because the tics of Tourette
   syndrome tend to remit or subside with maturity, such that a diagnosis
   may no longer be warranted for many adults, prevalence rates in
   pediatric populations are higher than those in adult populations.

   Mild cases of Tourette syndrome are less likely to come to tertiary or
   clinical attention, raising the possibility of ascertainment bias in
   referred populations. There are few broad-based population studies, and
   most older epidemiological studies of Tourette's were based on
   individuals referred to specialty clinics. Children with milder
   symptoms are not likely to be referred to specialty clinics, so most
   studies of Tourette's patients have an inherent bias towards more
   severe cases. Epidemiologic methods are vulnerable to errors, because
   of the variance in symptom severity and because symptoms are not always
   recognized by the patient or family.

History

   Jean-Martin Charcot (1825 - 1893) was a French neurologist and
   professor who bestowed the eponym for Tourette syndrome on behalf of
   his resident, Georges Albert Édouard Brutus Gilles de la Tourette.
   Charcot is shown here during a lesson with a "hysterical" woman patient
   at the Salpêtrière hospital.
   Enlarge
   Jean-Martin Charcot (1825 - 1893) was a French neurologist and
   professor who bestowed the eponym for Tourette syndrome on behalf of
   his resident, Georges Albert Édouard Brutus Gilles de la Tourette.
   Charcot is shown here during a lesson with a "hysterical" woman patient
   at the Salpêtrière hospital.

   A thorough history of Tourette syndrome is published in Kushner's A
   Cursing Brain? : The Histories of Tourette Syndrome. A French doctor,
   Jean Marc Gaspard Itard, reported the first case of Tourette syndrome
   in 1825, describing Marquise de Dampierre, an important woman of
   nobility in her time. Jean-Martin Charcot, an influential French
   physician, assigned his resident Georges Albert Édouard Brutus Gilles
   de la Tourette, a French physician and neurologist, to study patients
   at the Salpêtrière Hospital, with the goal of defining an illness
   distinct from hysteria and from chorea. Charcot and Tourette believed
   that the "tic illness" they had observed was an untreatable, chronic,
   and progressive hereditary condition.

   In 1885, Gilles de la Tourette published an account of nine patients,
   Study of a Nervous Affliction, concluding that a new clinical category
   should be defined. His description included accounts of Marquise de
   Dampierre, previously described by Itard, as a reclusive aristocratic
   lady who "ticked and blasphemed" from the age of seven until her death
   at the age of 80 years. Gilles de la Tourette describes the common
   feature of involuntary movements or tics in all nine patients. The
   eponym was later bestowed by Charcot after and on behalf of Gilles de
   la Tourette.

   Little progress was made over the next century in explaining or
   treating tics. With limited clinical experience, involving typically
   one or two patients, authors advanced different ideas, including brain
   lesions similar to those resulting from rheumatic chorea or
   encephalitis lethargica as a cause of tics, faulty mechanisms of normal
   habit formation, and treatment with Freudian psychoanalysis. The
   psychogenic view prevailed well into the 20th century.

   The possibility that movement disorders, including Tourette syndrome,
   might have an organic origin was raised when an encephalitis epidemic
   from 1918-1926 led to a subsequent epidemic of tic disorders. The
   psychoanalytic theory was so dominant that it was claimed that an
   organic component alone would not be sufficient to produce Tourette
   syndrome. At the time, psychiatrists believed patients with tics must
   also be suffering from unresolved psychological disturbances or
   psychosexual conflicts, and psychiatric intervention was the preferred
   method of treatment. Patients and their families were told that their
   own psychological maladjustments were to blame for their symptoms,
   adding to the burden carried by the patients and their families. Until
   the early 1970s, psychoanalysis was the preferred intervention for
   Tourette syndrome.

   During the 1960s and 1970s, as the beneficial effects of haloperidol
   (Haldol) on tics became known, the psychoanalytic approach to Tourette
   syndrome was questioned. The first description of haloperidol in the
   treatment of Tourette's was published by Seignot in 1961. The turning
   point came in 1965, when Dr. Arthur K. Shapiro—described as "the father
   of modern tic disorder research"—treated a Tourette’s patient with
   haloperidol. Dr. Shapiro and his wife, Elaine Shapiro (Ph.D.), reported
   the treatment in a 1968 article, and severely criticized the
   psychoanalytic approach.

   The Shapiros, working with the patient families who founded the
   Tourette Syndrome Association (TSA) in 1972, advanced the argument that
   Tourette's is a neurological, rather than psychological, disorder, and
   worked to persuade the media to promote information about Tourette's.
   The U.S. National Institutes of Health (NIH) turned down a 1972 grant
   proposal from the TSA because "the reviewers believed there were
   probably no more than 100 cases of TS in the entire nation". Subsequent
   articles on Tourette's in Good Housekeeping, The New York Times and Ann
   Landers produced an "enormous response, proving that there were many
   undiagnosed cases of TS across the United States".

   Since the 1990s, a more neutral view of Tourette's has emerged, in
   which biological vulnerability and adverse environmental events are
   seen to interact. In 2000, the American Psychiatric Association
   published the DSM-IV-TR, revising the text of DSM-IV to no longer
   require that symptoms of tic disorders cause distress or impair
   functioning. As of 2006, the Tourette Syndrome Association has contacts
   in more than 50 countries. The Tourette syndrome International database
   Consortium (TIC) brought together data on clinical samples of patients
   with Tourette syndrome from twenty-two countries (Argentina, Australia,
   Austria, Belgium, Brazil, Canada, Denmark, Germany, Hungary, Iceland,
   Israel, Italy, Japan, The Netherlands, Norway, Peoples Republic of
   China, Poland, South Africa, Sweden, Turkey, the US and the UK);
   Tourette's has also been studied in Chile, Colombia, Costa Rica, India,
   Indonesia, Korea, and Spain.

Social impact and notable cases

   Discussions with adults who have Tourette's reveal that not everyone
   wants treatment or a "cure", especially if that means they may "lose"
   something else in the process. Some people believe that there may be
   latent advantages associated with genetic vulnerability to the
   syndrome. Cognitive control may be enhanced in young people with
   Tourette's because the need to suppress tics results in a more
   efficient control of inhibitions. There is evidence to support the
   clinical lore that children with "TS-only" (Tourette's in the absence
   of comorbid conditions) are unusually gifted: neuropsychological
   studies have identified advantages in children with TS-only. One study
   found that children with TS-only are faster than the average for their
   age group on timed motor coordination.
   Wolfgang Amadeus Mozart in 1777, aged twenty-one. Speculation that he
   may have had Tourette's is not based on reliable evidence.
   Enlarge
   Wolfgang Amadeus Mozart in 1777, aged twenty-one. Speculation that he
   may have had Tourette's is not based on reliable evidence.

   Notable individuals with Tourette syndrome are found in all walks of
   life, including musicians, athletes and authors. The best-known example
   of a person who may have used obsessive–compulsive traits to advantage
   is Dr Samuel Johnson, the 18th-century English man of letters, who had
   Tourette syndrome as clearly evidenced by the writings of James
   Boswell. Johnson wrote A Dictionary of the English Language in 1747,
   and was a prolific writer, poet, and critic.

   Some authors have speculated that Mozart may have had Tourette's.
   Benjamin Simkin, a medical doctor, argued that Mozart had Tourette
   syndrome. Simkin is an endocrinologist—not a psychiatrist or a
   neurologist, the medical fields which specialize in the neurological
   disorder. His claim was picked up by newspapers worldwide, causing an
   international sensation, and internet websites have fueled the
   speculation. However, no Tourette's expert or organization has
   presented credible evidence to show that this was the case, and noted
   neurologist and author Oliver Sacks published an editorial disputing
   Simkin's claim.

   The entertainment industry often depicts those with Tourette syndrome
   as social misfits whose only tic is coprolalia, which has furthered
   stigmatization and the public's misunderstanding of those with
   Tourette's. The coprolalic symptoms of Tourette's are also fodder for
   radio and television talk shows.

Research directions and controversies

   Findings reported in published studies of Tourette syndrome since 1999
   have advanced TS science in the areas of genetics, neuroimaging,
   neurophysiology, and neuropathology. The TSA supports a clinical
   database that may help identify genes involved in Tourette syndrome,
   and the TSA International Genetic Consortium has collected a database
   on large extended families for future studies. Novel neuroimaging
   studies are being employed to study tic expression and functional or
   cognitive deficits in TS patients. Studies of Tourette's
   neurophysiology and neuropathology are attempting to link deficits in
   Tourette's to specific brain mechanisms, and have taken advantage of a
   brain bank sponsored by the TSA. Clinical trials have focused on
   understanding tic suppression, comorbid conditions, novel treatment
   approaches such as botulinum toxin, and targeted behavioural therapies.
   Controversy remains in the areas of deep brain stimulation and PANDAS.

   Questions remain regarding how best to classify Tourette syndrome, and
   how closely Tourette's is related to other movement disorders or
   psychiatric disorders. Good epidemiologic data is still lacking, and
   available treatments are not risk free and not always well tolerated.

   The direction of current and future research in Tourette's was outlined
   in a 2005 journal article by the outgoing chairman of the TSA
   Scientific Advisory Board. Swerdlow divides the research landscape into
   five broad questions about Tourette's: what is it, who has it, what
   causes it, how it should be studied, and how it should be (medically)
   treated.

   According to Swerdlow, "the 'core' TS conundrum" is a lack of consensus
   about the definition of Tourette syndrome. Since vocal tics result from
   a "motor event (ie, a contracting diaphragm moving air through the
   upper airways)", TS could be defined as a disorder of motor tics,
   eliminating the distinction between TS and the other tic disorders.
   Individuals who have only tics may not be functionally impaired,
   raising the question of whether Tourette's as currently defined should
   be a DSM diagnosis. Swerdlow highlights the importance of studies in
   new areas such as behavioural techniques, and says that "the
   whole-cloth dismissal of psychologic forces in the pathobiology of TS
   was a strategic error". Questions remain about whether co-occurring
   (comorbid) conditions should be part of the core definition, and why
   sensory phenomena, which are a core part of Tourette's, are not part of
   the diagnostic criteria.

   Dropping the criteria for impairment from the diagnosis resulted in
   higher estimates of the prevalence of TS (the question of "who has
   it?"). Older estimates "came from tertiary referral samples, the
   sickest of the sick"; greater prevalence casts the condition in an
   entirely new light, and calls for new biological models of the
   condition and new approaches to addressing a more common disorder.
   Discovering the causes of Tourette's may help resolve the questions of
   what it is and who has it. The autosomal dominant inheritance model has
   not been validated, and past research has been affected by the problem
   of referred samples, which may not reflect broader populations of
   persons with Tourette's. Probabilistic genetic models may yield better
   clues than the "one gene equals one disorder" approach. One of the most
   controversial presumed causes, the PANDAS hypothesis, has sparked
   disagreement.

   Expanding criteria for the diagnosis, and increasing awareness of the
   impact of comorbid diagnoses, has resulted in further questions of how
   to study Tourette's. Tourette's patients are often recruited from
   sources that introduce ascertainment bias towards one 'type' of TS.
   Developing and applying standardized instruments, along with a greater
   awareness of ascertainment bias in recruitment sources, will be
   important in genetic studies. We do not know if "we lose both signals
   and are just adding noise to the experimental outcome" when comorbid
   conditions, such as OCD or ADHD, are included or excluded from study
   samples, or samples include/exclude children or adults, or patients
   with severe symptoms.

   Tourette's is a heterogeneous condition, with waxing and waning
   symptoms. The inherently changing nature of its core symptoms
   complicates research design, resulting in questions about medications
   in clinical practice. Results from case studies may not be borne out by
   controlled or prospective, longitudinal studies, stimulants may be
   underused, and behavioural therapies are understudied. High-profile
   media coverage focuses on treatments that do not have established
   safety or efficacy e.g., deep brain stimulation, and alternative
   therapies involving unstudied efficacy and side effects are pursued by
   many parents.

   In the USA, the NIH has ongoing clinical trials, and the TSA funds
   ongoing research through its Research Program and Research Grant
   Awards. Other worldwide ongoing trials can be found by contacting
   Tourette syndrome advocacy groups.

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