Tourette Syndrome: Facts, Symptoms & Treatment
Author: Disabled World : Contact: Disabled World
Published: 2009-07-16 : (Rev. 2016-03-26)
Synopsis and Key Points:
Symptoms of Tourette syndrome nearly always appear during childhood, with an average onset between the ages of seven and ten.
Tourette syndrome occurs in persons of all ethnic groups, with males being affected approximately three to four times as often as females.
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 neurological disorder with onset in childhood, characterized by the presence of multiple physical (motor) tics and at least one vocal (phonic) tic; these tics characteristically wax and wane. The exact cause of Tourette's is unknown, but it is well established that both genetic and environmental factors are involved.
Defining Tourette Syndrome
Tourette syndrome is a form of neurological disorder which is marked by repetitive, involuntary vocalizations and movements referred to as, 'tics,' that is named for Dr. Georges Gilles de la Tourette, a French neurologist who described the condition in an 86 year old French noblewoman in 1885. Symptoms of Tourette syndrome nearly always appear during childhood, with an average onset between the ages of seven and ten years of age. Tourette syndrome occurs in persons of all ethnic groups, with males being affected approximately three to four times as often as females. An estimated two-hundred thousand Americans experience the most severe form of Tourette syndrome, with as many as one in one-hundred exhibiting milder and less complex symptoms that can include vocal tics and chronic motor or transient tics. While Tourette syndrome may be a chronic condition that presents symptoms lasting throughout the person's life, many people experience the worst of the symptoms during their adolescent years and improvement during their late teens and into adulthood.
Symptoms of Tourette Syndrome
An estimated two-hundred thousand Americans experience the most severe form of Tourette syndrome, with as many as one in one-hundred exhibiting milder and less complex symptoms that can include vocal tics and chronic motor or transient tics. Symptoms of Tourette syndrome nearly always appear during childhood, with an average onset between the ages of seven and ten years of age.
The tics associated with Tourette syndrome are classified as either simple or complex. Tics that are simple are brief, sudden and repetitive motions which involve limited numbers of muscle groups. Common simple tics can include blinking of the eyes or other vision irregularities, shoulder shrugging, facial grimacing, and should or head jerking. Vocalizations involved can include sniffing, grunting sounds, or repetitive throat-clearing. Vocal tics that are simple can include sniffing or snorting, throat-clearing, or barking.
Complex tics involve coordinated and distinct patterns of movements that include several muscle groups in the person's body. These tics may include facial grimacing in combination with shoulder shrugging or head twisting. Additional complex tics can appear to be purposeful, such as touching objects or sniffing; jumping, twisting, hopping, or bending. Complex vocal tics can include words or phrases. Dramatic and disabling tics may include movements resulting in self-harm such as punching oneself in the face, or vocal tics that include repeating the words or phrases of others or swear words. At times tics persons with Tourette syndrome experience are preceded by a sensation or urge in the muscle group that is affected, referred to as, 'Premonitory Urge.' Some persons with Tourette describe a need to complete a tic in a specific way, or a specific number of times, in order to relieve this urge or to decrease the sensation.
The tics persons with Tourette syndrome experience are often worse when they are excited or anxious, and better while they are calm and focused on activities. Specific physical experiences may either trigger or worsen the tics they experience. Examples of this can be hearing another person clear their throat or sniff, or having a tight collar that can trigger neck tics. The tics the person experiences do not go away while they sleep, although they are many times diminished significantly.
Tics that persons with Tourette syndrome experience may come and go over time, as well as varying in frequency, type, severity, and location. The first symptoms the person experiences commonly occur in their neck and head, progressing to include the muscles of their trunk and extremities. Motor tics usually precede development of tics which are vocal, with simple tics preceding complex ones. Many persons with Tourette syndrome experience their most severe tics prior to their mid-teenage years, improving in their late teens and into early adulthood. Around ten-percent of persons affected present with a progressive or disabling course lasting into adulthood.
While the symptoms of Tourette syndrome are involuntary, some persons may at times camouflage, suppress, or otherwise manage the tics they experience through an effort to minimize their impact. Persons with Tourette syndrome many times report a buildup of tension when they suppress their tics, to the point where they feel that the tic must be expressed. Tics in response to environmental triggers may seem to be either purposeful or voluntary; they are not.
A lot of people with Tourette syndrome have other neuro-behavioral problems involving inattention. These problems can include ADHD or OCD. Worried about germs or dirt, for example, can be associated with repetitious hand washing; concerns over bad things happening can be associated with ritualistic behaviors like repeating or counting; arranging or ordering. Persons with Tourette syndrome have reported problems with anxiety and depression, and difficulties with living that might be directly related to their disability.
Causes of Tourette Syndrome
The causes of Tourette syndrome are currently unknown, although research points to abnormalities in specific brain regions that include the frontal lobes, basal ganglia, and cortex, as well as the circuits which connect these regions and the neurotransmitters responsible for communication among nerve cells. With the complexity of Tourette syndrome, the cause is likely to be just as complex.
Diagnosing Tourette Syndrome
A diagnosis of Tourette syndrome is made after a doctor verifies that the person has had both vocal and motor tics over a period of a year. The presence of additional psychiatric or neurological conditions may assist a doctor in arriving at a diagnosis. Common tics are not often misdiagnosed by knowledgeable doctors. Atypical presentation or symptoms such as the onset of symptoms in adulthood can require specific specialty expertise for diagnosis. There are no laboratory or blood tests for Tourette syndrome, although neuroimaging studies such as MRI's, CT scans, and EEG's, or some blood tests, can be used in order to rule out conditions that may be confused with Tourette syndrome.
People commonly obtain a formal diagnosis of Tourette syndrome after experiencing symptoms for some time. For both doctors and families that are unfamiliar with the syndrome, mild and moderate tics might be viewed as inconsequential, a part of a development stage, or as the result of another condition. Parents might believe that eye blinking could be related to vision issues, or that sniffing is related to allergies, for example. Many persons with Tourette syndrome are self-diagnosed after either they have themselves, or their parents, or perhaps a relative or friend, have either read or heard about Tourette syndrome.
Treatment of Tourette Syndrome
Many times tic symptoms do not cause impairment, so the majority of persons with Tourette syndrome do not need medication for tic suppression. There are effective medications available for persons whose symptoms do interfere with their ability to function. Neuroleptics are administered for tic suppression, with a number of forms available; some are more effective than others, such as Pimozide and Haloperidol. There is no one, specific medication that is helpful to everyone with Tourette syndrome, and there is no single medication which completely alleviates symptoms. All medications have side effects.
The majority of the side effects associated with neuroleptic medications can be managed by initiating treatment slowly, reducing the dose when effects occur. Common side effects of neuroleptics include weight gain, sedation, and cognitive dulling. Side effects such as dystonic reactions to include twisting movements or postures, parkinsonian-like symptoms, tremor, or other involuntary movements are less common and can be managed through reduction of the dosage. Discontinuing the use of neuroleptic medications involves a slow reduction in use to avoid rebound increases in tics, as well as withdrawal dyskinesias. A form of withdrawal dyskinesia is referred to as, 'Tardive Dyskinesia,' and involves a movement disorder which is distinct from Tourette syndrome, and may result from the chronic use of neuroleptics. Risk of this particular side effect may be reduced through using lower amounts of neuroleptics over a shorter period of time.
There are other medications that can be used to reduce the severity of tics, although most of them have not been as extensively studied, or shown to be as useful as neuroleptics. These medications include alpha-adrenergic agonists like Clonidine and Guanfacine. These kinds of medications are use mostly to control hypertension. The most common side effect of these medications is sedation. Medications are also available to treat associated neuro-behavioral disorders which may occur in persons with Tourette syndrome. Medications such as Detroamephetamine and Methylphenidate may lessen symptoms of ADHD in persons with Tourette syndrome without increasing the severity of the tics they experience. Persons with OCD may benefit from medications such as Clomipramine, Fluvoxamine, Fluoxetine, Sertraline, or Paroxetine.
Psychotherapy may benefit persons with Tourette syndrome, even though psychological problems do not cause the syndrome. Psychotherapy may assist persons with the syndrome to cope with the disorder and deal with either emotional or social issues that can occur. Behavioral treatments that include awareness training and competing response training, such as voluntarily moving in response to premonitory urge, have shown effectiveness in controlled trials.
Tourette Syndrome Information
Studies based on twins and families has suggested that Tourette syndrome is an inherited disorder. While early family studies have suggested an autosomal dominant mode of inheritance, recent studies suggest a pattern of inheritance that is more complex. There may be a few genes with substantial effects, although it is possible that a number of genes with smaller effects and environmental factors could play a part in development of Tourette syndrome. Genetic studies have also suggested that there are some forms of both OCD and ADHD which are genetically related to Tourette syndrome, although there is less evidence for a genetic relationship between Tourette syndrome and other neuro-behavioral problems which commonly occur with the disorder. Families need to understand that genetic predisposition may not necessarily result in Tourette syndrome, it may instead result in a milder tic disorder or as OCD behaviors. A child may also not develop any Tourette syndrome symptoms at all.
Gender plays an important role in the genetic expression of Tourette syndrome. At-risk males are more likely to experience tics, while at-risk females are more likely to experience OCD symptoms. Persons with Tourette syndrome may also have genetic risks for additional neuro-behavioral disorders, such as either depression or substance abuse. Genetic counseling of persons with Tourette syndrome needs to include a review of potentially hereditary conditions in the persons family.
While there is currently no cure for Tourette syndrome, the condition in a number of persons with the disorder does improve in their late teens and early twenties. Because of this, some persons with the disorder may become symptom-free, or may no longer require medications for the suppression of tics. Although Tourette syndrome is usually chronic, it is not degenerative; persons with the disorder have a normal life expectancy. Tourette syndrome does not impair intelligence.
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