Tourette Syndrome

Alternative Names: Tourette’s syndrome, Tourette’s disorder, Gilles de la Tourette syndrome, GTS or, more commonly, simply Tourette’s or TS

Definition:
Tourette syndrome  is an inherited neuropsychiatric disorder with onset in childhood, characterized by 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.

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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), but 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 10 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. Tourette’s does not adversely affect intelligence or life expectancy. 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.

Tourette syndrome 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.

Clacification
Tics are sudden, repetitive, stereotyped, nonrhythmic movements (motor tics) and utterances (phonic tics) that involve discrete muscle groups.[8] 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. 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 (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

Symptoms:
Tics — sudden, brief, intermittent movements or sounds — are the hallmark sign of Tourette syndrome. Symptoms range from mild to severe and debilitating.

Tics are classified as either:
*Simple tics, which are sudden, brief and repetitive and involve a limited number of muscle groups

*Complex tics, which are distinct, coordinated patterns of movements involving several muscle groups

Tics involving movement (motor tics) — often facial tics, such as blinking — usually begin before vocal tics do. But the spectrum of tics that people experience is diverse, and there’s no typical case.

Some of the more common tics seen in Tourette syndrome
Motor tics:-

Simple tics:
*Eye blinking
*Head jerking
*Shoulder shrugging
*Eye darting
*Finger flexing
*Sticking the tongue out

Complex tics :
*Touching the nose
*Touching other people
*Smelling objects
*Obscene gestures
*Flapping the arms
*Hopping

Vocal tics:-

Simple tics :
*Hiccuping
*Yelling
*Throat clearing
*Barking

Complex tics :
*Using different voice intonations
*Repeating one’s own words or phrases
*Repeating others’ words or phrases
*Using expletives

Tics can vary in type, frequency and severity over time. They may worsen during periods of stress and anxiety, fatigue, illness, or excitement. They can occur during sleep. You’ll likely experience an urge, called a premonitory urge, before the onset of motor or vocal tics. A premonitory urge is an uncomfortable bodily sensation, such as an itch, a tingle or tension. Expression of the tic brings relief.

Different tics may develop over time. Tourette symptoms are usually at their worst during the teenage years and sometimes improve during the transition to adulthood.

With great effort, some people with Tourette syndrome can sometimes temporarily stop a tic or hold back tics until they find a place where it’s less disruptive to express them.

 

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 shown 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, Tourette’s is sporadic, that is, it is not inherited from parents. In other cases, tics are associated with disorders other than Tourette’s, a phenomenon known as tourettism.

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. In 1998, a team at the US National Institute of Mental Health proposed a hypothesis that both obsessive–compulsive disorder (OCD) and tic disorders may arise in a subset of children as a result of a poststreptococcal autoimmune process. 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 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

Risk factors
Having a family history of Tourette syndrome or other tic disorders may increase the risk of developing Tourette syndrome.

Complication:
People with Tourette syndrome have a normal life span and often lead a healthy, active life. However, having Tourette syndrome may increase the risk of learning, behavioral and social challenges, which can mar self-image.

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In addition, having Tourette syndrome means you’re likely to have other related conditions, such as:

*Attention-deficit/hyperactivity disorder (ADHD)

*Obsessive-compulsive disorder

*Learning disabilities

*Sleep disorders

*Depression

*Anxiety disorders

Diagnosis:
According to the revised fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR), Tourette’s 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. The previous DSM-IV 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 criteria for Tourette’s, but do not have distress or impairment.[44] 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; it 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). Coughing, eye blinking and tics that mimic asthma are commonly misdiagnosed.

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.

There is no requirement that other comorbid conditions (such as ADHD or OCD) be present, but 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 behavioral 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. 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;  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. 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
Treatment:
There’s no cure for Tourette syndrome. Treatment is intended to help control tics that interfere with everyday activities and functioning. When tics aren’t severe, treatment may be unnecessary.

Medications:
No medication is helpful to everyone with Tourette syndrome, none completely eliminates symptoms, and they all have side effects to be weighed against the benefits. However, some medications can be used to help control or minimize tics or to control symptoms of related conditions, such as attention-deficit/hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD). These may include:

*Drugs that block or deplete the neurotransmitter dopamine in the brain, such as fluphenazine or pimozide (Orap). Used to control tics, these medications may have side effects such as weight gain and a dulling of the mind.

*Botulinum Toxin Type A (Botox) injections. For simple or vocal tics, an injection into the affected muscle may help relieve the tic.

*Stimulant medications, such as methylphenidate (Concerta, Ritalin, others) and dextroamphetamine (Dexedrine, others). These are used to help increase attention and concentration for people with ADHD.

*Central adrenergic inhibitors, such as clonidine (Catapres) or guanfacine (Tenex). Typically prescribed for high blood pressure, these drugs may help control behavioral symptoms, such as impulse control problems and rage attacks. Side effects may include sleepiness.

*Antidepressants, such as fluoxetine (Prozac, Sarafem, others). These may help control the symptoms of OCD.

Therapies
*Psychotherapy. Psychotherapy can be helpful for two reasons. It can help with accompanying problems, such as ADHD, obsessions, depression and anxiety. Therapy can also help people cope with Tourette syndrome.

*Deep brain stimulation. For debilitating tics that don’t respond to other treatment, deep brain stimulation (DBS) may help. DBS consists of implanting a battery-operated medical device (neurostimulator) in the brain to deliver electrical stimulation to targeted areas that control movement. Further research is needed to determine whether DBS is beneficial for people with Tourette syndrome.

 

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. 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 have 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. Severity of tics early in life does not predict tic severity in later life, and prognosis is generally favorable, although 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”. A higher rate of migraines than the general population and sleep disturbances are reported.

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

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources;
http://en.wikipedia.org/wiki/Tourette_syndrome
http://www.mayoclinic.com/health/tourette-syndrome/DS00541

http://www.sfn.org/index.aspx?pagename=brainBriefings_tourette#full

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