Tourette syndrome (TS) is an inherited disorder of the nervous system, characterized by a variable expression ofunwanted movements and noises (tics).
The first references in the literature to what might today be classified as Tourette syndrome largely describe individualswho were wrongly believed to be possessed by the devil. In 1885 Gilles de la Tourette, a French neurologist, provided thefirst formal description of this syndrome, which he described as an inherited neurological condition characterized by motorand vocal tics.
Although vocal and motor tics are the hallmark of Tourette syndrome, such other symptoms as the expression of sociallyinappropriate comments or behaviors, obsessive compulsive disorder, attention deficit disorder, self-injuring behavior,depression, and anxiety also appear to be associated with Tourette syndrome. Most research suggests that Tourettesyndrome is an autosomal dominant disorder, although a gene responsible for Tourette syndrome has not yet been discovered.
Tourette syndrome is found in all populations and all ethnic groups, but is three to four times more common in males thanfemales and is more common in children than adults. The exact frequency of Tourette syndrome is unknown, butestimates range from 0.05% to 3%.
The cause of Tourette syndrome is unknown, although some studies suggest that the tics in Tourette syndrome arecaused by an increased amount of a neurotransmitter called dopamine. A neurotransmitter is a chemical found in thebrain that helps to transmit information from one brain cell to another. Other studies suggest that the defect in Tourettesyndrome involves another neurotransmitter called serotonin; or involves other chemicals required for normal functioningof the brain.
Most studies suggest that Tourette syndrome is an autosomal dominant disorder with decreased penetrance, althoughthis hypothesis has not been proven and may not be true in all families. An autosomal dominant disorder results from achange in one copy of a pair of genes. Individuals with an autosomal dominant disorder have a 50% chance of passing onthe changed gene to their children. Decreased penetrance means that not all people who inherit the changed gene willdevelop symptoms. There is some evidence that females who inherit the Tourette syndrome gene have a 70% chance ofexhibiting symptoms and males have a 99% chance of having symptoms. It has been suggested that other genetic andenvironmental factors may play a role in the development of symptoms in people who inherit the changed gene, but nonehave been discovered. Some researchers believe that Tourette syndrome has different causes in different individuals or iscaused by changes in more than one gene, although these theories are less substantiated. Further research is needed toestablish the cause of Tourette syndrome.
The principal symptoms of Tourette syndrome include simple and complex motor and vocal tics. Simple motor tics arecharacterized by brief muscle contractions of one or more limited muscle groups. An eye twitch is an example of a simplemotor tic. Complex motor tics tend to appear more complicated and purposeful than simple tics, and involve coordinatedcontractions of several muscle groups. Some examples of complex motor tics include the act of hitting oneself andjumping. Copropraxia, the involuntary display of unacceptable/obscene gestures; and echopraxia, the imitation of themovement of another individual, are other examples of complex motor tics.
Vocal tics are actually manifestations of motor tics that involve the muscles required for vocalization. Simple vocal ticsinclude stuttering, stammering, abnormal emphasis of part of a word or phrase, and inarticulate noises such as throatclearing, grunts, and high-pitched sounds. Complex vocal tics typically involve the involuntary expression of words.Perhaps the most striking example of this is coprolalia, the involuntary expression of obscene words or phrases, whichoccurs in fewer than one-third of people with Tourette syndrome. The involuntary echoing of the last word, phrase,sentence or sound vocalized by oneself (phalilalia) or of another person or sound in the environment (echolalia) are alsoclassified as complex tics.
The type, frequency, and severity of tics exhibited varies tremendously between individuals with Tourette syndrome.Tourette syndrome has a variable age of onset and tics can start anytime between infancy and age 18. Initial symptomsusually occur before the early teens; the mean age of onset for both males and females is approximately seven years ofage. Most individuals with symptoms initially experience simple muscle tics involving the eyes and the head. Thesesymptoms can progress to tics involving the upper torso, neck, arms, hands, and occasionally the legs and feet. Complexmotor tics are usually the latest-onset muscle tics. Vocal tics usually have a later onset then motor tics. In some rarecases, people with Tourette syndrome suddenly present with multiple, severe, or bizarre symptoms.
Not only is there extreme variability in clinical symptoms between individuals with Tourette syndrome, but individualscommonly experience a variability in type, frequency, and severity of symptoms over the course of their lifetime.Adolescents with Tourette syndrome often experience unpredictable and variable symptoms, which may be related tofluctuating hormone levels and decreased compliance in taking medications. Adults often experience a decrease insymptoms or a complete end to symptoms.
A number of factors appear to affect the severity and frequency of tics. Stress appears to increase the frequency andseverity of tics, while concentration on another part of the body that is not involved in a tic can result in the temporaryalleviation of symptoms. Relaxation, following attempts to suppress the occurrence of tics, may result in an increasedfrequency of tics. An increased frequency and severity of tics can also result from exposure to such drugs as steroids,cocaine, amphetamines, and caffeine. Hormonal changes such as those that occur prior to the menstrual cycle can alsoincrease the severity of symptoms.
People with Tourette syndrome are more likely to exhibit non-obscene, socially inappropriate behaviors such asexpressing insulting or socially unacceptable comments or socially unacceptable actions. It is not known whether thesesymptoms stem from a more general dysfunction of impulse control that might be part of Tourette syndrome.
Tourette syndrome appears to also be associated with attention deficit disorder (ADD). ADD is a disorder characterized bya short attention span and impulsivity, and in some cases hyperactivity. Researchers have found that 21-90% ofindividuals with Tourette syndrome also exhibit symptoms of ADD, whereas 2-15% of the general population exhibitsymptoms of ADD.
People with Tourette syndrome are also at higher risk for having symptoms of obsessive-compulsive disorder (OCD).OCD is a disorder characterized by persistent, intrusive, and senseless thoughts (obsessions) or compulsions to performrepetitive behaviors that interfere with normal functioning. A person with OCD, for example, may be obsessed with germsand may counteract this obsession with continual hand washing. Symptoms of OCD are present in 1.9-3% of the generalpopulation, whereas 28-50% of people with Tourette syndrome have symptoms of OCD.
Self-injurious behavior (SIB) is also seen more frequently in those with Tourette syndrome. Approximately 34-53% ofindividuals with Tourette syndrome exhibit some form of self-injuring behavior. The SIB is often related to OCD but canalso occur in those with Tourette syndrome who do not have OCD.
Symptoms of anxiety and depression are also found more commonly in people with Tourette syndrome. It is not clear,however, whether these symptoms are symptoms of Tourette syndrome or occur as a result of having to deal with thesymptoms of moderate to severe Tourette syndrome.
People with Tourette syndrome may also be at increased risk for having learning disabilities and personality disorders;and may be more predisposed to such behaviors as aggression, antisocial behaviors, severe temper outbursts, andinappropriate sexual behavior. Further controlled studies need to be performed, however, to ascertain whether thesebehaviors are symptoms of Tourette syndrome.
Tourette syndrome cannot be diagnosed through a blood test. The diagnosis is made through observation and interview ofthe patient and discussions with other family members. The diagnosis, of Tourette syndrome is complicated by a variety offactors. The extreme range of symptoms of this disorder makes it difficult to differentiate Tourette syndrome from otherdisorders with similar symptoms. Diagnosis is further complicated by the fact that some tics appear to be within the rangeof normal behavior. For example an individual who only exhibits such tics as throat clearing and sniffing may bemisdiagnosed with a medical problem such as allergies. In addition, such bizarre and complex tics as coprolalia may bemistaken for psychotic or “bad” behavior. Diagnosis is also confounded by individuals who attempt to control tics in publicand in front of health care professionals and deny the existence of symptoms. Although there is disagreement over whatcriteria should be used to diagnosis Tourette syndrome, one aid in the diagnosis is the DSMMD (DSM-IV). The DSM-IVoutlines suggested diagnostic criteria for a variety of conditions including Tourette syndrome.
• presence of both motor and vocal tics at some time during the course of the illness
• the occurrence of multiple tics nearly every day through a period of more than one year, without a remission of tics fora period of greater than three consecutive months
• the symptoms cause distress or impairment in functioning
• age of onset of prior to 18 years of age
• the symptoms are not due to medications or drugs and are not related to another medical condition
Some physicians critique the DSM-IV criteria, arguing that they do not include the full range of behaviors and symptomsseen in Tourette syndrome. Others criticize the criteria since they limit the diagnosis to those who experience a significantimpairment, which may not be true for individuals with milder symptoms. For this reason many physicians use their clinicaljudgment as well as the DSM-IV criteria as a guide to diagnosing Tourette syndrome.
There is no cure for Tourette syndrome. Treatment involves the control of symptoms through educational andpsychological interventions and/or medications. The treatment and management of Tourette syndrome varies from patientto patient and should focus on the alleviation of the symptoms that are most bothersome to the patient or that cause themost interference with daily functioning.
Psychological treatments such as counseling are not generally useful for the treatment of tics but can be beneficial in thetreatment of such associated symptoms as obsessive-compulsive behavior and attention deficit disorder. Counseling mayalso help individuals to cope better with the symptoms of this disorder and to have more positive social interactions.Psychological interventions may also help people cope better with stressors that can normally be triggers for tics andnegative behaviors. Relaxation therapies may, however, increase the occurrence of tics. The education of familymembers, teachers, and peers about Tourette
Many people with mild symptoms of Tourette syndrome never require medications. Those with severe symptoms mayrequire medications for all or part of their lifetime. The most effective treatment of tics associated with Tourette syndromeinvolves the use of drugs such as haloperidol, pimozide, sulpiride, and tiapride, which decrease the amount of dopaminein the body. Unfortunately, the incidence of side effects, even at low dosages, is quite high. The short-term side effectscan include sedation, dysphoria, weight gain, movement abnormalities, depression, and poor school performance. Long-term side effects can include phobias, memory difficulties, and personality changes. These drugs are therefore bettercandidates for short-term rather than long-term therapy.
Tourette syndrome can also be treated with such other drugs as clonidine, clonazepam, and risperidone, but the efficacyof these treatments is unknown. In many cases, treatment of such as associated conditions such as ADD and OCD isoften more of a concern than the tics themselves. Clonidine used in conjunction with such stimulants as Ritalin may beuseful for treating people with Tourette syndrome who also have symptoms of ADD. Stimulants should be used withcaution in individuals with Tourette syndrome since they can sometimes increase the frequency and severity of tics. OCDsymptoms in those with Tourette syndrome are often treated with such drugs as Prozac, Luvox, Paxil, and Zoloft.
In many cases the treatment of Tourette syndrome with medications can be discontinued after adolescence. Trials shouldbe performed through the gradual tapering off of medications and should always be done under a doctor’s supervision.
The prognosis for Tourette syndrome in individuals without associated psychological conditions is often quite good, andonly approximately 10% of Tourette syndrome individuals experience severe tic symptoms. Approximately 30% of peoplewith Tourette syndrome will experience a decrease in the frequency and severity of tics, and another 30-40% willexperience a complete end of symptoms by late adolescence. The other 30-40% will continue to exhibit moderate tosevere symptoms in adulthood. There does not appear to be a definite correlation between the type, frequency, andseverity of symptoms and the eventual prognosis. Patients with severe tics may experience social difficulties and mayisolate themselves from others in fear of shocking and embarrassing them. People with Tourette syndrome who havesuch other symptoms as obsessive compulsive disorder, attention deficit disorder, and self-injurious behavior usually havea poorer prognosis
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