Question:
What is known about the nature and diagnosis of Tourette's syndrome?
2006-05-24 00:08:53 UTC
Do we know what causes it, and can it ever be cured?
Seven answers:
Juliart
2006-05-26 13:39:30 UTC
Since the others answers are packed with information, I'll address the last part of your question. There was a fascinating breakthrough at Harvard last October, by Dr. Matthew State. You can read about it here:



http://www.tsa-usa.org/



and click on the link "Genetic Breakthrough Yields Clue to Tourettes"
SuzieQ92
2006-05-30 22:58:54 UTC
I have Tourette Syndrome, and I found a great website yesterday. http://au.geocities.com/jones_kacm/contents.htm



It has the best information I have seen that a lay person can understand. No biology degree needed! :) The site is done by someone with Tourette Syndrome.



I am interested in talking with other people with Tourette Syndrome. Please e-mail me at momma2david@yahoo.com if you'd like to talk to someone about how to cope with Tourette's. ( I tried to get the e-mail address for Bull Dog who posted that he wanted to talk to others, but I didn't see it under his profile. If you read this Bull Dog, please e-mail me.)
Barbara
2017-03-01 06:26:24 UTC
1
2006-05-24 00:11:22 UTC
Don't effin know





HaHaha
2006-05-24 00:13:16 UTC
conditions:

http://www.tourettesyndrome.net/conditions.htm

Behavior:

http://www.tourettesyndrome.net/behavior.htm

anything else:

http://www.tourettesyndrome.net/
jarforjesus
2006-05-25 09:47:56 UTC
What is Tourette syndrome?



Tourette syndrome (TS) is a neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics. The disorder is named for Dr. Georges Gilles de la Tourette, the pioneering French neurologist who in 1885 first described the condition in an 86-year-old French noblewoman.



The early symptoms of TS are almost always noticed first in childhood, with the average onset between the ages of 7 and 10 years. TS occurs in people from all ethnic groups; males are affected about three to four times more often than females. It is estimated that 200,000 Americans have the most severe form of TS, and as many as one in 100 exhibit milder and less complex symptoms such as chronic motor or vocal tics or transient tics of childhood. Although TS can be a chronic condition with symptoms lasting a lifetime, most people with the condition experience their worst symptoms in their early teens, with improvement occurring in the late teens and continuing into adulthood.



What are the symptoms?



Tics are classified as either simple or complex. Simple motor tics are sudden, brief, repetitive movements that involve a limited number of muscle groups. Some of the more common simple tics include eye blinking and other vision irregularities, facial grimacing, shoulder shrugging, and head or shoulder jerking. Simple vocalizations might include repetitive throat-clearing, sniffing, or grunting sounds. Complex tics are distinct, coordinated patterns of movements involving several muscle groups. Complex motor tics might include facial grimacing combined with a head twist and a shoulder shrug. Other complex motor tics may actually appear purposeful, including sniffing or touching objects, hopping, jumping, bending, or twisting. Simple vocal tics may include throat-clearing, sniffing/snorting, grunting, or barking. More complex vocal tics include words or phrases. Perhaps the most dramatic and disabling tics include motor movements that result in self-harm such as punching oneself in the face or vocal tics including coprolalia (uttering swear words) or echolalia (repeating the words or phrases of others). Some tics are preceded by an urge or sensation in the affected muscle group, commonly called a premonitory urge. Some with TS will describe a need to complete a tic in a certain way or a certain number of times in order to relieve the urge or decrease the sensation.



Tics are often worse with excitement or anxiety and better during calm, focused activities. Certain physical experiences can trigger or worsen tics, for example tight collars may trigger neck tics, or hearing another person sniff or throat-clear may trigger similar sounds. Tics do not go away during sleep but are often significantly diminished.



What is the course of TS?



Tics come and go over time, varying in type, frequency, location, and severity. The first symptoms usually occur in the head and neck area and may progress to include muscles of the trunk and extremities. Motor tics generally precede the development of vocal tics and simple tics often precede complex tics. Most patients experience peak tic severity before the mid-teen years with improvement for the majority of patients in the late teen years and early adulthood. Approximately 10 percent of those affected have a progressive or disabling course that lasts into adulthood.



Can people with TS control their tics?



Although the symptoms of TS are involuntary, some people can sometimes suppress, camouflage, or otherwise manage their tics in an effort to minimize their impact on functioning. However, people with TS often report a substantial buildup in tension when suppressing their tics to the point where they feel that the tic must be expressed. Tics in response to an environmental trigger can appear to be voluntary or purposeful but are not.



What causes TS?



Although the cause of TS is unknown, current research points to abnormalities in certain brain regions (including the basal ganglia, frontal lobes, and cortex), the circuits that interconnect these regions, and the neurotransmitters (dopamine, serotonin, and norepinephrine) responsible for communication among nerve cells. Given the often complex presentation of TS, the cause of the disorder is likely to be equally complex.



What disorders are associated with TS?



Many with TS experience additional neurobehavioral problems including inattention; hyperactivity and impulsivity (attention deficit hyperactivity disorder—ADHD) and related problems with reading, writing, and arithmetic; and obsessive-compulsive symptoms such as intrusive thoughts/worries and repetitive behaviors. For example, worries about dirt and germs may be associated with repetitive hand-washing, and concerns about bad things happening may be associated with ritualistic behaviors such as counting, repeating, or ordering and arranging. People with TS have also reported problems with depression or anxiety disorders, as well as other difficulties with living, that may or may not be directly related to TS. Given the range of potential complications, people with TS are best served by receiving medical care that provides a comprehensive treatment plan.



How is TS diagnosed?



TS is a diagnosis that doctors make after verifying that the patient has had both motor and vocal tics for at least 1 year. The existence of other neurological or psychiatric conditions[1] can also help doctors arrive at a diagnosis. Common tics are not often misdiagnosed by knowledgeable clinicians. But atypical symptoms or atypical presentation (for example, onset of symptoms in adulthood) may require specific specialty expertise for diagnosis. There are no blood or laboratory tests needed for diagnosis, but neuroimaging studies, such as magnetic resonance imaging (MRI), computerized tomography (CT), and electroencephalogram (EEG) scans, or certain blood tests may be used to rule out other conditions that might be confused with TS.



It is not uncommon for patients to obtain a formal diagnosis of TS only after symptoms have been present for some time. The reasons for this are many. For families and physicians unfamiliar with TS, mild and even moderate tic symptoms may be considered inconsequential, part of a developmental phase, or the result of another condition. For example, parents may think that eye blinking is related to vision problems or that sniffing is related to seasonal allergies. Many patients are self-diagnosed after they, their parents, other relatives, or friends read or hear about TS from others.



[1] These include childhood-onset involuntary movement disorders such as dystonia, or psychiatric disorders characterized by repetitive behaviors/movements (for example, stereotypic behaviors in autism and compulsive behaviors in obsessive-compulsive disorder — OCD).



How is TS treated?



Because tic symptoms do not often cause impairment, the majority of people with TS require no medication for tic suppression. However, effective medications are available for those whose symptoms interfere with functioning. Neuroleptics are the most consistently useful medications for tic suppression; a number are available but some are more effective than others (for example, haloperidol and pimozide). Unfortunately, there is no one medication that is helpful to all people with TS, nor does any medication completely eliminate symptoms. In addition, all medications have side effects. Most neuroleptic side effects can be managed by initiating treatment slowly and reducing the dose when side effects occur. The most common side effects of neuroleptics include sedation, weight gain, and cognitive dulling. Neurological side effects such as tremor, dystonic reactions (twisting movements or postures), parkinsonian-like symptoms, and other dyskinetic (involuntary) movements are less common and are readily managed with dose reduction. Discontinuing neuroleptics after long-term use must be done slowly to avoid rebound increases in tics and withdrawal dyskinesias. One form of withdrawal dyskinesia called tardive dykinesia is a movement disorder distinct from TS that may result from the chronic use of neuroleptics. The risk of this side effect can be reduced by using lower doses of neuroleptics for shorter periods of time.



Other medications may also be useful for reducing tic severity, but most have not been as extensively studied or shown to be as consistently useful as neuroleptics. Additional medications with demonstrated efficacy include alpha-adrenergic agonists such as clonidine and guanfacine. These medications are used primarily for hypertension but are also used in the treatment of tics. The most common side effect from these medications that precludes their use is sedation.



Effective medications are also available to treat some of the associated neurobehavioral disorders that can occur in patients with TS. Recent research shows that stimulant medications such as methylphenidate and dextroamphetamine can lessen ADHD symptoms in people with TS without causing tics to become more severe. However, the product labeling for stimulants currently contraindicates the use of these drugs in children with tics/TS and those with a family history of tics. Scientists hope that future studies will include a thorough discussion of the risks and benefits of stimulants in those with TS or a family history of TS and will clarify this issue. For obsessive-compulsive symptoms that significantly disrupt daily functioning, the serotonin reuptake inhibitors (clomipramine, fluoxetine, fluvoxamine, paroxetine, and sertraline) have been proven effective in some patients.



Psychotherapy may also be helpful. Although psychological problems do not cause TS, such problems may result from TS. Psychotherapy can help the person with TS better cope with the disorder and deal with the secondary social and emotional problems that sometimes occur. More recently, specific behavioral treatments that include awareness training and competing response training, such as voluntarily moving in response to a premonitory urge, have shown effectiveness in small controlled trials. Larger and more definitive NIH-funded studies are underway.



Is TS inherited?



Evidence from twin and family studies suggests that TS is an inherited disorder. Although early family studies suggested an autosomal dominant mode of inheritance (an autosomal dominant disorder is one in which only one copy of the defective gene, inherited from one parent, is necessary to produce the disorder), more recent studies suggest that the pattern of inheritance is much more complex. Although there may be a few genes with substantial effects, it is also possible that many genes with smaller effects and environmental factors may play a role in the development of TS. Genetic studies also suggest that some forms of ADHD and OCD are genetically related to TS, but there is less evidence for a genetic relationship between TS and other neurobehavioral problems that commonly co-occur with TS. It is important for families to understand that genetic predisposition may not necessarily result in full-blown TS; instead, it may express itself as a milder tic disorder or as obsessive-compulsive behaviors. It is also possible that the gene-carrying offspring will not develop any TS symptoms.



The sex of the person also plays an important role in TS gene expression. At-risk males are more likely to have tics and at-risk females are more likely to have obsessive-compulsive symptoms.



People with TS may have genetic risks for other neurobehavioral disorders such as depression or substance abuse. Genetic counseling of individuals with TS should include a full review of all potentially hereditary conditions in the family.



What is the prognosis?



Although there is no cure for TS, the condition in many individuals improves in the late teens and early 20s. As a result, some may actually become symptom-free or no longer need medication for tic suppression. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. Individuals with TS have a normal life expectancy. TS does not impair intelligence. Although tic symptoms tend to decrease with age, it is possible that neurobehavioral disorders such as depression, panic attacks, mood swings, and antisocial behaviors can persist and cause impairment in adult life.



What is the best educational setting for children with TS?



Although students with TS often function well in the regular classroom, ADHD, learning disabilities, obsessive-compulsive symptoms, and frequent tics can greatly interfere with academic performance or social adjustment. After a comprehensive assessment, students should be placed in an educational setting that meets their individual needs. Students may require tutoring, smaller or special classes, and in some cases special schools.



All students with TS need a tolerant and compassionate setting that both encourages them to work to their full potential and is flexible enough to accommodate their special needs. This setting may include a private study area, exams outside the regular classroom, or even oral exams when the child's symptoms interfere with his or her ability to write. Untimed testing reduces stress for students with TS.



What research is being done?



Within the Federal government, the leading supporter of research on TS and other neurological disorders is the National Institute of Neurological Disorders and Stroke (NINDS). The NINDS, a part of the National Institutes of Health (NIH), is responsible for supporting and conducting research on the brain and central nervous system.



NINDS sponsors research on TS both in its laboratories at the NIH and through grants to major medical institutions across the country. The National Institute of Mental Health, the National Center for Research Resources, the National Institute of Child Health and Human Development, the National Institute on Drug Abuse, and the National Institute on Deafness and Other Communication Disorders also support research of relevance to TS. And another component of the Department of Health and Human Services, the Centers for Disease Control and Prevention, funds professional education programs as well as TS research.



Knowledge about TS comes from studies across a number of medical and scientific disciplines, including genetics, neuroimaging, neuropathology, clinical trials (medication and non-medication), epidemiology, neurophysiology, neuroimmunology, and descriptive/diagnostic clinical science.



Genetic studies. Currently, NIH-funded investigators are conducting a variety of large-scale genetic studies. Rapid advances in the technology of gene finding will allow for genome-wide screening approaches in TS, and finding a gene or genes for TS would be a major step toward understanding genetic risk factors. In addition, understanding the genetics of TS genes will strengthen clinical diagnosis, improve genetic counseling, lead to the clarification of pathophysiology, and provide clues for more effective therapies.



Neuroimaging studies. Within the past 5 years, advances in imaging technology and an increase in trained investigators have led to an increasing use of novel and powerful techniques to identify brain regions, circuitry, and neurochemical factors important in TS and related conditions.



Neuropathology. Within the past 5 years, there has been an increase in the number and quality of donated postmortem brains from TS patients available for research purposes. This increase, coupled with advances in neuropathological techniques, has led to initial findings with implications for neuroimaging studies and animal models of TS.



Clinical trials. A number of clinical trials in TS have recently been completed or are currently underway. These include studies of stimulant treatment of ADHD in TS and behavioral treatments for reducing tic severity in children and adults. Smaller trials of novel approaches to treatment such as dopamine agonist and GABAergic medications also show promise.



Epidemiology and clinical science. Careful epidemiological studies now estimate the prevalence of TS to be substantially higher than previously thought with a wider range of clinical severity. Furthermore, clinical studies are providing new findings regarding TS and co-existing conditions. These include subtyping studies of TS and OCD, an examination of the link between ADHD and learning problems in children with TS, a new appreciation of sensory tics, and the role of co-existing disorders in rage attacks. One of the most important and controversial areas of TS science involves the relationship between TS and autoimmune brain injury associated with group A beta-hemolytic streptococcal infections or other infectious processes. There are a number of epidemiological and clinical investigations currently underway in this intriguing area.



Where can I get more information?



For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network (BRAIN) at:
Torrealta
2006-05-24 00:34:53 UTC
Tourette syndrome (also called Tourette's syndrome, Tourette's disorder, Gilles de la Tourette syndrome, GTS or TS) is an inherited neurological disorder with onset in childhood, characterized by the presence of multiple motor tics and at least one phonic tic, which characteristically wax and wane. Tourette's syndrome occurs along a spectrum of tic disorders, which includes transient tics and chronic tics.



Tourette syndrome was once considered a rare and bizarre syndrome. It is no longer considered rare, but is often undetected because of the wide range of severity, with most cases classified as mild.



The eponym was bestowed by Jean-Martin Charcot after and 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. Another 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.



The hallmarks of Tourette's syndrome are repetitive, involuntary movements (motor tics) and utterances (phonic tics) that constantly change in number, frequency, severity, and anatomical location. The Tourette Syndrome Association describes tics as movements or sounds "that occur intermittently and unpredictably out of a background of normal motor activity". The tics of Tourette's characteristically come and go. Waxing and waning — a natural increase and decrease in severity and frequency of tics — occurs differently in each individual. Tics are described as occurring 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 syndrome, but it is not required for a diagnosis of Tourette's. Fewer than 15% of TS patients exhibit coprolalia. More common tics are eye blinking, throat clearing, coughing, neck stretching, and shoulder shrugging.



In contrast to the stereotyped movements of some 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 TS are aware of a premonitory urge, which is similar to the need to sneeze or scratch an itch. Individuals describe the need to tic as the buildup of tension in a particular anatomical location, which they consciously choose to release, as if the subject "had to do it". Some examples of this 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 in Tourette's subjects as "premonitory sensory phenomena". Some published descriptions of the tics of Tourette's identify sensory phenomena as the core symptom of TS, even though they are not included in the diagnostic criteria.

Due to the sensory nature of tics, they can be described as semi-voluntary or "unvoluntary", because they may be experienced as a voluntary response to an unwanted, premonitory urge. Tics are experienced as irresistible and must eventually be expressed. People with TS 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. The control which can be exerted (from seconds to hours at a time) may merely postpone and exacerbate the ultimate expression of the tic. People with TS may seek a secluded spot to release their symptoms after suppressing them in school or at work. Some people with TS 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.



Tourette syndrome patients 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, overall functioning, and cognitive function in patients with co-occurring Tourette's syndrome and ADHD may be accounted for by the comorbid ADHD, highlighting the importance of identifying and treating co-occurring conditions when they are present.



DIAGNOSIS:



According to the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders, DSM-IV-TR, TS 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 1 year, with no more than 3 consecutive tic-free months. Previous editions of the DSM included a requirement for distress or 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 not everyone with the diagnosis has distress or impairment to functioning. The onset must have been before the age of 18, and cannot be attributed to the use of a substance or another medical condition. Hence, other medical conditions which include tics or tic-like movements (such as autism) must be ruled out before conferring a Tourette's diagnosis.



Although there is no such thing as a "typical" case of Tourette's syndrome, the condition follows a fairly reliable course in terms of age of onset and the natural time course of severity of symptoms. Tics may appear up to the age of 18, but the most typical age of onset is six or seven. The ages of statistical highest tic severity are 8 to 12 (average 10), 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. Tics most frequently present initially in midline body regions where there are many muscles: the head, neck and facial region. This can be contrasted with the stereotyped movements of other disorders (e.g.; stims and stereotypies) which tend to have an earlier age of onset, are more symmetrical, and involve extremities (e.g.; flapping hands). The most common tics to appear early in the course of the condition are frequently confused with other conditions: examples are allergies, asthma, and vision problems.



There are no medical or screening tests which can be used in diagnosing Tourette's disorder. The diagnosis is made based on a history of symptoms, and after ruling out other conditions which can include tics. If a physician believes that there may be another condition present which could explain tics, some tests may be ordered as necessary to rule out those other conditions. An example of this would be when diagnostic confusion between tics and seizure activity exists, which would call for an EEG, or if there are indications that an MRI is warranted to rule out brain abnormalities. However, most cases are diagnosed by merely observing a history of tics, and medical tests are not always called for.



Because co-occurring conditions like OCD or attention-deficit hyperactivity disorder can be more impairing than tics, these conditions should be 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."



DIFFERENTIAL DIAGNOSIS:



Dystonias, choreas, other genetic conditions, and secondary causes of tics should be ruled out in the differential diagnosis for Tourette's syndrome. Other conditions which 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 disease, Idiopathic dystonia, Duchenne's disease, Tuberous sclerosis, Chromosomal disorders, Down syndrome, Klinefelter's syndrome, XYY karyotype, and Fragile X syndrome; Wilson's disease or Syndenham's chorea; and secondary or acquired causes of tics, such as drug-induced tics, head trauma, encephalitis, stroke, and carbon monoxide poisoning. Secondary causes of tics (not related to inherited Tourette's syndrome) are commonly referred to as tourettism. 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.



PROGNOSIS:



Tourette's syndrome is a spectrum disorder, which means that the severity of the condition can range along a continuum from mild to severe. However, it should be emphasized that "the majority of cases can be categorized as mild." Those with mild cases may be minimally impacted by symptoms, to the extent that casual observers might not know of their condition. Severe cases (which are the rare minority in adulthood) can inhibit or prevent the individual from engaging in common activities such as holding a job or having a fulfilling social life.



Regardless of symptom severity, individuals with TS can expect to live a normal life span. Although TS symptoms may be lifelong and chronic for some, it is not a degenerative condition and is not life-threatening. Life span and intelligence are normal, although learning disabilities may be present.



Multiple 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 brought to diagnostic awareness, and often improve with understanding of the condition. The statistical age of highest tic severity is typically between 8 and 12, 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 to the popular belief that tics increase at puberty. In many cases, complete remission of tic symptoms occurs after adolescence.



It is not uncommon for parents of affected children to be unaware that they, too, may have had tics as children. Because Tourette's syndrome tends to remit or subside in severity as one matures, 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 are 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 come to the realization that, "I did that, too, 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 which interfere with social or academic functioning, supportive psychotherapy or school accommodations can be helpful. Because co-occurring conditions (such as ADHD or OCD) can cause more impact on overall functioning than tics, a thorough evaluation for co-occurring conditions is called for when symptoms and impairment warrant.



A supportive environment and family generally gives one skills to manage the disorder. Persons with Tourette's syndrome may learn to camouflage socially inappropriate tics or channel the energy of their tics into some other endeavor, to their advantage. Accomplished musicians, athletes, public speakers, and professionals from all walks of life are found among people with Tourette's syndrome.



There is currently no reliable means of predicting the outcome for any individual. The gene or genes for Tourette's syndrome have not been identified, and there is no potential "cure".



Discussions with adults who have Tourette syndrome reveal that not everyone wants treatment or a "cure", especially if that means they may "lose" something else in the process. Some believe that there may even be latent advantages associated with the genetic vulnerability.



PREVALENCE:



Tourette's syndrome has historically been described as a rare disorder, with about 5 to 10 people in 10,000 having TS. However, multiple studies published since 2000 demonstrate that the prevalence is much higher than previously thought, and that Tourette's syndrome can no longer be considered rare. Contemporary prevalence estimates range from 1 to 3 per 1,000 to 10 per 1,000. 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's 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's Syndrome. Males are affected 3 to 4 times more often than females. The disorder 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's 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.



Tourette syndrome is found among all social, racial and ethnic groups.



CAUSES AND ORIGINS: GENETIC AND EPIGENETIC FACTORS:



Genetic studies have proven that the overwhelming majority of cases of Tourette's syndrome are inherited, although the exact mode of inheritance is not yet known. Tic disorders have long been thought to be inherited as an autosomal dominant gene. Recent research challenges the autosomal dominant hypothesis, and suggests an additive model involving multiple genes. In some cases, tics may not be inherited; these cases are identified as "sporadic" TS (also known as tourettism) because a genetic link is missing.



Tourette's is a condition of incomplete penetrance, meaning not everyone who inherits the genetic vulnerability will show symptoms; Tourette's also shows variable expression — even family members with the same genetic makeup may show different levels of symptom severity. The gene(s) may express as TS, as a milder tic disorder (transient or chronic tics), or as obsessive compulsive symptoms with no tics at all. Only a minority of the children who inherit the gene(s) will have symptoms severe enough to ever require medical attention. There is currently no way to predict the symptoms a child may display, even if the gene(s) are inherited.



A person with TS has about a 50% chance of passing the gene(s) to one of his/her children. Gender appears to have a role in the expression of the genetic vulnerability, with males more likely to express tics than females. The exact mechanism affecting the inherited vulnerability has not been established. Research presents considerable evidence that abnormal activity of the brain chemical, or neurotransmitter, dopamine, is involved. Other neurotransmitters may also be involved. Neuroanatomic models implicate failures in circuits connecting the brain's cortex and subcortex.



Studies suggest that non-genetic, environmental, infectious, or psychosocial factors — while not causing Tourette's — can influence the severity of the disorder. For example, twin studies have shown that the lower birth weight is more likely to have more noticeable symptoms. Other perinatal events, such as maternal stress and obstetric complications may impact upon the severity of the expression of the inherited genetic vulnerability. 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, P.A.N.D.A.S., plays a role in the onset of tic disorders and OCD is a current focus of research.



In clinical (referred) samples, OCD and ADHD are often associated with Tourette's. Some forms of OCD may be genetically linked to Tourette's. "...at least some forms of OCD are etiologically related to TS, and may, therefore, be a variant expression of the same etiologic factors that are important for the expression of tics." The genetic relationship of ADHD to Tourette's syndrome has not been fully established. Not all persons with Tourette's syndrome will have ADHD or OCD, although in clinical populations, a high percentage of patients presenting for care do have ADHD.



TREATMENT:



Treatment of Tourette syndrome can be divided into treatment of tics, and treatment of co-occurring conditions, which, when present, are often a larger source of impairment than the tics themselves. Not all persons with tics will also have co-occurring conditions, but when comorbid disorders are present, they often take treatment priority. Knowledge and understanding are the best treatments available for tics. The majority of people with TS require no medication, but medication is available to help when symptoms interfere with functioning. Because children with tics often present to physicians when their tics are at their highest severity, 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 understanding of the condition and a supportive environment.



The classes of medications with the most proven efficacy in treating tics — typical and atypical neuroleptics — can have long-term and short-term adverse effects. The antihypertensive agents, clonidine (Catapres®) and guanfacine (Tenex®), are also used to treat tics. 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 as alternatives when stimulant trials fail. Clomipramine (Anafranil®), a tricyclic antidepressant, and SSRIs, a class of antidepressants including fluoxetine, sertraline, and fluvoxamine, may be prescribed when a TS patient also has symptoms of Obsessive-compulsive disorder.



Cognitive Behavioral 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 also be useful in relieving stress that may aggravate tics.



RESEARCH DIRECTIONS AND CONTROVERSIES:



The direction of current and future research in Tourette's syndrome was delineated 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 do we study it, and how do we treat it"?



According to Swerdlow, "we still lack consensus on the definition of TS." He calls this "the 'core' TS conundrum". 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. Because "individuals with tics alone may not be functionally impaired", should TS, as currently defined, be a DSM diagnosis? Swerdlow highlights the importance of studies in new areas, such as behavioral techniques, and that "the whole-cloth dismissal of psychologic forces in the pathobiology of TS was a strategic error". Questions remain about whether co-occurring 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 prevalence estimates for TS (the question of "who has it?"). With TS prevalence estimates at 1% to 2%, the condition is cast in an entirely new light.



Discovering "what causes TS" may resolve the questions of what it is and who has it. Research has been affected by the problem of referred samples, which may not reflect broader populations of persons with Tourette's. Probabilistic models may yield better results in finding the cause, as the autosomal dominant inheritance model has not been validated. The P.A.N.D.A.S. controversy remains contentious.



Expanding criteria for the diagnosis, and increasing awareness of the impact of co-occurring diagnoses result in further questions of how to study Tourette's. Developing and applying standardized instruments, along with awareness of ascertainment bias in recruitment sources, will be important in genetic studies. We don't know if "we lose both signals and are just adding noise to the experimental outcome" when co-occurring 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 heterogeous condition, with waxing and waning symptoms. The inherent changing nature of its core symptoms complicates research design. Results from case studies may not be borne out by controlled studies, stimulants may be under utilized, and behavioral therapies are understudied. High profile media coverage focuses on treatments that do not have established safety or efficacy e.g.; deep brain stimulation.



SOCIOLOGICAL AND CULTURAL ASPECTS:



With the increased knowledge of the full range of symptomatology of Tourette's syndrome, it has shifted from a condition only recognized in its most severe and impairing forms, to being recognized as a condition which is often mild, and which may be associated with some advantages and disadvantages. There are many individuals with Tourette's, living and deceased, recognized in their fields, or for whom obsessive-compulsive tendencies associated with Tourette's may have helped fuel their success. The best known example of a person who may have used obsessive-compulsive traits to advantage is Dr Samuel Johnson, lexicographer, who certainly had Tourette syndrome as 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. There are numerous recognized athletes, musicians, and authors with Tourette's.



Some authors have speculated that Mozart may have had Tourette syndrome. However no Tourette's syndrome expert or organization has voiced concurrence that there is credible evidence to conclude that Mozart had Tourette's.



The entertainment industry often depicts those with TS as being social misfits whose only tic is coprolalia, which has furthered stigmatization and the general public's misunderstanding of persons with Tourette's. The symptoms of Tourette syndrome are fodder for radio and television talk shows. Many television shows have addressed the topic of Tourette's, but often, television and film productions are not accurate representations of the condition, and many of them have used misconceptions about coprolalia as a plot device, or portrayed people with Tourette's as being dangerously out of control.



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Causes and origins of Tourette syndrome have not been fully elucidated. Tourette syndrome (also called "Tourette's syndrome", "Tourette's disorder", "Gilles de la Tourette syndrome", "GTS" or "TS") is an inherited neurological disorder with onset in childhood, characterized by the presence of multiple motor tics and at least one phonic tic, which characteristically wax and wane. Tourette's syndrome occurs along a spectrum of tic disorders, which includes transient tics and chronic tics. Contemporary prevalence estimates of the condition range from 1 to 3 per 1,000 to 10 per 1,000.



Genetic factors

Genetic studies have proven that the overwhelming majority of cases of Tourette's syndrome are inherited, although the exact mode of inheritance is not yet known. Tic disorders have long been thought to be inherited as an autosomal dominant gene. Recent research challenges the autosomal dominant hypothesis, and suggests an additive model involving multiple genes. According to Roger Freeman, M.D., "Genes that cause tics have not yet been identified; it's very unlikely there's just one. Tics are very familial, but not in a Mendelian pattern (dominant, recessive, etc.). You can't 'inherit' the committee decision to define a problem in a specific way, so TS can't itself be inherited. It's a tic disorder that is inherited."



The inherited vulnerability to tic disorders may produce varying symptoms in different family members: Tourette's is a condition of incomplete penetrance, meaning not everyone who inherits the genetic vulnerability will show symptoms; Tourette's also shows variable expression — even family members with the same genetic makeup may show different levels of symptom severity. Inheriting the gene or genes does not necessarily mean that symptoms will be displayed. The gene(s) may express as TS, as a milder tic disorder (transient or chronic tics), or as obsessive compulsive symptoms with no tics at all. Only a minority of the children who inherit the gene(s) will have symptoms severe enough to ever require medical attention.



A person with TS has about a 50% chance of passing the gene(s) to one of his/her children. Gender appears to have a role in the expression of the genetic vulnerability, with males more likely to express tics than females. There is currently no way to predict the symptoms a child may display, even if the gene(s) are inherited.



Recent research suggests that a small number of Tourette syndrome cases may be caused by a defect on chromosome 13 of gene SLITRK1. Some cases of tourettism (tics due to reasons other than inherited Tourette's syndrome) can be caused by mutation. The finding of a chromosomal abnormality appears to apply to a very small minority of cases (1 - 2%). Studies to locate all of the genes implicated in Tourette's syndrome are ongoing.



FACTORS OF VULNERABILITY:



While it is known that Tourette's is genetic, the exact mechanism affecting the inherited vulnerability has not been established. Research presents considerable evidence that abnormal activity of the brain chemical, or neurotransmitter, dopamine, is involved. Other neurotransmitters may also be involved. Neuroanatomic models implicate failures in circuits connecting the brain's cortex and subcortex.



Again, in some cases, tics may not be inherited; these cases are identified as "sporadic" TS (also known as tourettism) because a genetic link is missing.



NON-GENETIC INFLUENCES:



Studies suggest that non-genetic, environmental, infectious, or psychosocial factors — while not causing Tourette's — can impact upon the expression of the severity of the disorder. As one example of non-genetic factors, twin studies have shown that the lower birth weight twin is the one more likely to display more symptoms. Other perinatal events, such as maternal stress and obstetric complications, may also impact upon the severity of the expression of the inherited genetic vulnerability. 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, P.A.N.D.A.S., plays a role in the onset of tic disorders and OCD is a current focus of research.



RELATION WITH OCD AND ADHD:



Some forms of obsessive-compulsive disorder (OCD) may be genetically linked to Tourette's. Genetic studies show an increased rate of tics and obsessive-compulsive behaviors or OCD in relatives of patients with Tourette's, and "reinforce the idea that at least some forms of OCD are etiologically related to TS, and may, therefore, be a variant expression of the same etiologic factors that are important for the expression of tics." Further evidence supporting that OCD and Tourette's are alternative expressions of a common genetic vulnerability is that males inheriting the genetic vulnerability are more likely to display tics, while females are more likely to display obsessive-compulsive traits.



The genetic relationship of attention-deficit hyperactivity disorder (ADHD) to Tourette's syndrome is less clear, with some evidence to suggest no genetic linkage, and some evidence to suggest that some forms of Tourette's syndrome may be genetically related to ADHD. Not all persons with Tourette's syndrome will have ADHD or OCD, although in clinical populations, a high percentage of patients presenting for care do have ADHD. The high co-occurrence of ADHD observed in tertiary, referred populations may be an artifact of clinical ascertainment bias. Further study is needed to elucidate the genetic relationship between ADHD and Tourette's.



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Tourette syndrome (also Tourette's syndrome or TS) is an inherited neurological disorder with onset in childhood, characterized by the presence of motor and phonic tics. Tourette syndrome was once considered a rare and bizarre syndrome. It is no longer considered rare, but is often undetected because of the wide range of severity, with most cases classified as mild. Tourette syndrome occurs along a spectrum of tic disorders, which includes transient tics and chronic tics.



Recognized individuals with Tourette syndrome

Tourette syndrome has shifted from being a condition only recognized in its most severe and impairing forms, to being recognized as a condition which is often mild, and which may be associated with some advantages and disadvantages. There are many individuals with Tourette's, living and deceased, recognized in their fields, or for whom obsessive-compulsive tendencies associated with Tourette's may have helped fuel their success. An example of a person who may have used obsessive-compulsive traits to advantage is Dr Samuel Johnson, lexicographer, who certainly had Tourette syndrome as 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.



Andre Malraux, the French author, adventurer and statesman, also had Tourette syndrome. Howard Ahmanson, Jr, an American millionaire philanthropist who funds the causes of Christian fundamentalism, has Tourette's.



Prominent athletes diagnosed with Tourette syndrome include Mahmoud Abdul-Rauf (formerly Chris Jackson), a former NBA player; Eric Bernotas, a three-time U.S. skeleton champion who made his Olympics debut in 2006; Jim Eisenreich, a former major league baseball player; Tim Howard, a goalkeeper for Manchester United Football Club (currently on loan to Everton F.C); Mike Johnston, a relief pitcher formerly on the roster for the Pittsburgh Pirates; and Jeremy Stenberg, a motocross rider nicknamed "Twitch."



Recognized musicians with Tourette syndrome include Tobias Picker, a composer; Nick Tatham, a singer/songwriter; and Michael Wolff, a jazz musician. Author and neurologist Oliver Sacks describes the case of a drummer with TS, who uses his tics to give him a certain 'flair' or 'special sound' to his drumming.



Neurologist Oliver Sacks uses the pseudonym Carl Bennett to describe real-life Canadian Mort Doran, M.D., a pilot and surgeon with severe TS, whose tics remit almost completely while he is performing surgery.



SPECULATION ABOUT RECOGNIZED INDIVIDUALS WITH TOURETTE SYNDROME:



Some authors have speculated that Mozart may have had Tourette syndrome. However no Tourette's syndrome expert or organization has voiced concurrence that there is credible evidence to conclude that Mozart had Tourette's. One TS specialist stated that, "although some web sites list Mozart as an individual who had Tourette's and/or OCD, it's not clear from the descriptions of his behavior that he actually had either."



Comedian Dan Aykroyd described himself (in a radio interview with Terry Gross) as having mild Tourette syndrome that was successfully treated with therapy when he was a preteen, as well as mild Asperger syndrome. The diagnosis of Asperger syndrome did not exist in the 1960s, when Aykroyd was a preteen. The term was coined in 1981, and the diagnosis began to be more widely recognized in the 1990s. Tics can be caused by other disorders, including austistic spectrum disorders such as Asperger's. It is unclear if Aykroyd received the diagnoses of TS or AS from a medical source, whether he was speaking in his role as a comic, or whether the diagnoses were self-made. It was an audio interview, so the audience could not see Aykroyd's facial expressions, but the interviewer indicated uncertainty about whether Aykroyd was kidding.



REFERENCES TO TOURETTE'S IN THE ENTERTAINMENT INDUSTRY:



The entertainment industry often depicts those with TS as being social misfits whose only tic is coprolalia, which has furthered stigmatization and the general public's misunderstanding of persons with Tourette's.



Several documentaries have attempted, with varying levels of success, to portray Tourette's syndrome accurately and to advocate for greater understanding of persons with Tourette's. I Have Tourette's but Tourette's Doesn't Have Me, was produced by HBO in conjunction with the Tourette Syndrome Association, featuring children between the ages of six and thirteen. John's not Mad is a 1989 documentary about a Scottish teenager who has severe Tourette's and coprolalia. Twitch and Shout examines a society that is quick to judge a person who strays outside the limits of conventional behavior, won numerous awards, and was nominated for an Emmy.



A movie released on video, The Tic Code, stars Gregory Hines as a saxophone player with TS who befriends a 10 year old boy with TS. It was written by Polly Draper, and produced with her husband, jazz musician Michael Wolff, who has Tourette's and on whose life the script was loosely based. The UK movie, Dirty Filthy Love, tells the story of Mark Furness (Michael Sheen) with Obsessive Compulsive Disorder (OCD) and Tourette's negotiating his way through divorce, matchmaking efforts, and a woman who introduces him to therapy and unconditional love. A protaganist with Tourette's is presented in Jonathan Lethem's award-winning detective novel, Motherless Brooklyn.



The symptoms of Tourette syndrome are fodder for radio and television talk shows. Some talk shows (e.g.; Oprah) have focused on accurate portrayals of people with Tourette's, while others (e.g.; Dr. Phil) have furthered stigmatization, focusing on rare and sensational aspects of the condition. An incident of disinformation about coprolalia and Tourette's involved Dr. Laura Schlessinger. Garrison Keillor, radio show host of NPR's A Prairie Home Companion, produced a segment in 2006, titled "Broadway Tourette's", about segregating people with stereotypical Tourette's from other passengers on a cruise ship, prompting a press release from the Tourette Syndrome Association.



Many television shows have addressed the topic of Tourette's, but few have advanced understanding of Tourette's. A 1981 episode of the television show, Quincy, M.E., "Seldom Silent, Never Heard", was a seminal moment in the history of Tourette's. It was perhaps the first television representation of Tourette's syndrome, and its portrayal led to many undiagnosed people with tics recognizing their symptoms and getting a correct diagnosis. Other television shows which helped advance accurate information about Tourette's include L.A. Law, The Practice and 7th Heaven.



However, even more television and film productions are not accurate representations of persons with Tourette's, and many of them have used misconceptions about coprolalia as a plot device, or portrayed people with Tourette's as being dangerously out of control. For example, in an episode of Ally McBeal, Anne Heche portrays a woman with Tourette's who gets a sudden leg tic that causes her to run over and kill her boyfriend. An episode of Touched by an Angel, "An Angel on my Tree", was about a father who committed manslaughter in an angry "rage" reaction to an event that involved his son, both with Tourette's. In an episode of The Simpsons, Mrs Edna Krabappel is recounting all the diseases and illnesses Bart has claimed to have to excuse himself for a test. She says, "and that unfortunate case of Tourette's Syndrome", and Bart tries to pretend he still might have it by cursing and rambling. The British comedic drama Shameless features a character with Tourette's syndrome - Marty Fisher, who is also an arsonist. Singer, Pete Bennett, from Brighton, a contestent on the 2006 edition of British TV reality show, Big Brother 7, has Tourette syndrome. The show has been accused of exploiting Pete's Tourette's syndrome. The TSA UK "claimed the broadcaster had deliberately cast someone with relatively strong symptoms in order to make him a'figure of fun'." His condition was reported to have been aggravated by drug use. Other examples are The Big White, Deuce Bigalow: Male Gigolo, Niagara, Niagara, Not Another Teen Movie, Wedding Crashers, The Wedding Singer, The West Wing and What About Bob. In Matchstick Men, the protagonist (Nicolas Cage) is a neurotic con artist with Tourette's and OCD.



TOURETTE'S IN THE MUSIC INDUSTRY:



The UK Britpop band Manic Street Preachers recorded a song on the Gold Against The Soul album titled "Symphony Of Tourette", the lyric being sung from the perspective of a person with Tourette's. The album sleeve also contains a brief description of the condition and its symptoms following the lyrics for the song. Nirvana recorded a song on the In Utero album titled "Tourette's", sung with the lyrics intensely shouted, perhaps to mimic the syndrome. A musical about Tourette's, In My Life, was a flop on Broadway, opening in October, 2005, and closing quickly to poor reviews.


This content was originally posted on Y! Answers, a Q&A website that shut down in 2021.
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