Everything about Tourette Syndrome totally explained
Tourette syndrome (also called
Tourette's syndrome,
Tourette's disorder,
Gilles de la Tourette syndrome,
GTS or, more commonly, simply
Tourette's or
TS) is an
inherited neuropsychiatric 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. Between 1 and 11 children per 1,000 have Tourette's; as many as 10 per 1,000 people may have tic disorders, 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 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 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. Tic disorders are defined similarly by the
World Health Organization (International Statistical Classification of Diseases and Related Health Problems,
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. 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.
Echolalia (repeating the words of others) and
palilalia (repeating one's own words) occur in a minority of cases,
In contrast to the abnormal movements of other
movement disorders (for example,
choreas,
dystonias,
myoclonus, and
dyskinesias), the tics of Tourette's are stereotypic, temporarily suppressible, nonrhythmic, and often preceded by a premonitory urge. Immediately preceding tic onset, most individuals with Tourette's are aware of an urge that's 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're not included in the diagnostic criteria.
Tics are described as semi-voluntary or
"unvoluntary", The ability to suppress tics varies among individuals, and may be more developed in adults than children.
Although there's no such thing as a "typical" case of Tourette syndrome, 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. 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. 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. Genetic studies have shown that the overwhelming majority of cases of Tourette's are inherited, although the exact mode of inheritance isn't yet known, and no gene has been identified.
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. Children who meet five diagnostic criteria are classified, according to the hypothesis, as having Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections (
PANDAS). This contentious hypothesis is the focus of clinical and laboratory research, but remains unproven.
The exact mechanism affecting the inherited vulnerability to Tourette's hasn't 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.
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, hasn't been fully established. The onset must have occurred before the age of 18, and can't be attributed to the "direct physiological effects of a substance or a general medical condition".
TSH levels can be measured to rule out
hypothyroidism, which can be a cause of tics.
Brain imaging studies are not usually warranted. and
stereotypic movement disorder;
Sydenham's chorea;
idiopathic dystonia; and genetic conditions such as
Huntington's disease,
neuroacanthocytosis,
Hallervorden-Spatz syndrome,
Duchenne muscular dystrophy, Wilson's disease, 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. 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. 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.
Medication is available to help when symptoms interfere with functioning. and there's 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 behavioral 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
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. 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.
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 isn't
degenerative or life-threatening.
Intelligence is normal in those with Tourette's, although there may be learning disabilities. 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.]]
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 subsequent study confirmed and extended the paradoxical result that individuals with Tourette's exhibit greater levels of cognitive control than age-matched healthy peers. A study of eight children, age 8–17, found that children with Tourette syndrome were quicker at processing certain mental grammar skills than are children without the condition. The abnormalities that lead to tics may also lead to "other rapid behaviors, including the cognitive processing of rule-governed forms in language and other types of procedural knowledge".
Epidemiology
Tourette syndrome is found among all social, racial and ethnic groups, has been reported in all parts of the world, and is three to four times more frequent among males than among females. The tics of Tourette syndrome begin in childhood and tend to remit or subside with maturity; thus, a diagnosis may no longer be warranted for many adults, and
prevalence is much higher among children than adults. as many as 1 in 100 people experience tic disorders, including chronic tics and transient tics in childhood. There were few broad-based community studies published before 2000 and until the 1980s, most
epidemiological studies of Tourette syndrome were based on individuals referred to
tertiary care or specialty clinics. Children with milder symptoms are unlikely to be referred to specialty clinics, so these studies have an inherent
bias towards more severe cases. Studies of Tourette syndrome are vulnerable to error because tics vary in intensity and
expression, are often intermittent, and are not always recognized by clinicians, patients, family members, friends or teachers; approximately 20% of persons with Tourette syndrome don't recognize that they've tics. and a 1973 registry reported only 485 cases worldwide. However, multiple studies published since 2000 have consistently demonstrated that the prevalence is much higher than previously thought. The emerging consensus is that 1–11 children per 1,000 have Tourette's, with several studies supporting a tighter range of 6–8 children per 1,000. describing Marquise de Dampierre, an important woman of nobility in her time. 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, and a psychogenic view prevailed well into the 20th century.
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 turning point came in 1965, when
Arthur K. Shapiro—described as "the father of modern tic disorder research"—treated a Tourette’s patient with haloperidol, and published a paper criticizing the psychoanalytic approach.
Findings since 1999 have advanced TS science in the areas of genetics,
neuroimaging,
neurophysiology, and
neuropathology. 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. High-profile media coverage focuses on treatments that don't have established safety or efficacy, such as
deep brain stimulation, and alternative therapies involving unstudied efficacy and side effects are pursued by many parents. Some people believe that there may be latent advantages associated with genetic vulnerability to the syndrome. One study found that children with TS-only are faster than the average for their age group on timed
motor coordination.
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.
Although it has been
speculated that Mozart had Tourette's, no Tourette's expert or organization has presented credible evidence to show that this was the case,
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 in the US and in the British media.
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