Definition and Overview

Tourette syndrome is a neurological condition that causes a person to have involuntary and repetitive movements as well as vocalizations that are generally known as “tics.” This condition is believed to involve specific parts of the brain, their circuits, and the neurotransmitters that allow nerve communication, although the primary cause is not yet known.

Named after Dr. Georges Gilles de la Tourette, this disorder was first observed in an 86-year-old French woman, but is known at present as a disease that can begin even in childhood (as early as 3 years old). The severity of symptoms, however, can worsen or improve at different stages of the patient’s life.

Patients with Tourette syndrome are encouraged to seek medical attention for their symptoms, as certain treatment methods are currently available.

Causes of Condition

The main cause of Tourette syndrome is yet unknown, although research has managed to narrow down its origins to a number of brain regions, namely the frontal lobes, the cortex, and the basal ganglia. Research shows that abnormalities in these brain regions may be responsible for causing the tics, although what causes these abnormalities or triggers the disease in the first place are yet to be discovered.

Studies also show that aside from abnormalities in the brain, it is possible that the tics result from problems affecting the brain circuits that connect the three affected regions as well as the neurotransmitters that communicate with the nerves in the said regions. These neurotransmitters include serotonin, dopamine, and norepinephrine.

As for the risk factors, the disorder does not seem to be limited to a certain race and can thus affect anybody. It is found to be around 3 to 4 times more common among men, and can cause mild and almost unnoticeable symptoms as well as more severe and complicated ones. The symptoms can also be temporary or chronic, lasting throughout the patient’s entire lifetime.

Despite the lack of conclusive information on what causes the disorder, researchers have observed a link between Tourette syndrome and other neurobehavioral problems, such as ADHD (attention deficit hyperactivity disorder) and OCD (obsessive-compulsive disorder). It has also been linked with anxiety disorders and depression, or problems leading to these mental disorders.

Due to the wide range of possible and unpredictable complications that may arise from this disorder, patients suffering from it should seek medical care.

Key Symptoms

The main symptoms of Tourette syndrome are tics, which can manifest in many forms. They can be either motor or vocal in nature, and are generally classified into two categories: simple and complex.

Simple motor tics – These are brief but repetitive movements that come on suddenly and involuntarily. Simple motor tics affect only small muscle groups. Some examples are: * Eye blinking * Grimacing * Shrugging * Jerking the head


Simple vocal tics – These are repetitive sounds that the patient involuntarily makes. Some examples include:

  • Sniffing
  • Snorting
  • Grunting
  • Clearing the throat


Complex motor tics – These are more distinct and easily noticeable symptoms because they involve several muscle groups working at the same time, and thus cause larger, more distinct, and sometimes even coordinated movements. Some examples are:

  • Hopping
  • Jumping
  • Touching certain things
  • Bending
  • Twisting
  • Combination of two distinct movements


Complex vocal tics – These refer to the tendency to utter words or phrases uncontrollably.

In some cases, these tics can have a more serious effect on the patient, such as:

  • Motor tics leading to self-harm, such as punching oneself
  • Vocal tics leading to socially inappropriate utterances, two examples of which are:
  • Echolalia – repeating other people’s words
  • Coprolalia – involuntarily swearing


Before the tics occur, the patient usually feels an urge in the specific muscle group involved in the movement. This urge will lead to the actual movement, and to satisfy the urge, the patient sometimes need to complete a series of movements or to complete a single movement repetitively for a specific number of times. They can also be triggered or worsened by external factors. For example, hearing similar sounds may trigger vocal tics. The tics also become more prevalent when the patient is anxious or excited, but tend to decrease when the patient is calmer.

When the tics are milder, patients can still sometimes suppress or minimize them and thus are able to manage their symptoms on their own. However, studies show that when tics are suppressed, they tend to cause some tension in the affected muscle group.

Additionally, the tics can sometimes vary in intensity, and can even disappear or change to some other movement or vocalization. However, it has been noticed that the earliest tics involve the head and neck region and that motor tics usually develop before the vocal ones.

Who to See and Types of Treatments Available

A patient suspected of having Tourette syndrome can seek the help of a knowledgeable neurologist.

Tourette syndrome is diagnosed when the patient has had the tics for a minimum of 1 year. If the patient is also suffering from other neurological disorders, it is easier to confirm the diagnosis. But in patients showing no signs of other psychiatric issues, diagnosis can be more challenging, especially since no tests or imaging scans can be used. At the very least, blood tests, MRI scans, EEG, and CT scans can be used to rule out other conditions, but Tourette syndrome does not generally cause physical signs that show up on any of these tests and scans. Another hindrance to diagnosing TS is that some simple tics can be dismissed by some physicians as passing or inconsequential. Thus, for a successful diagnosis, it is crucial to see a doctor who is knowledgeable about the condition and is familiar with its various manifestations.

As for treatment, if the TS is associated with another present neurological condition, doctors may first attempt to treat the said condition and observe whether doing so also improves the tics.

If no other neurological condition is observed, the following treatment method will be used:


Medications

The medications used to help patients suppress their tics and improve their normal functioning include:

  • Neuroleptics – Known as the most effective medication for TS, these are drugs commonly used in the treatment of psychotic disorders. Some examples are pimozide and haloperidol. However, due to potential side effects, the drugs should be started slowly and the patient’s response to them should be closely monitored. If side effects begin to occur, the dosage needs to be reduced. Some of the possible side effects linked with these drugs include weight gain, tremors, and sedation, among others.

  • Alpha-adrenergic agonists – Although primarily prescribed for hypertension, these drugs have also shown some benefits for tic suppression. Since they cause fewer and less serious side effects, doctors usually prescribe these first to see if they help before resorting to neuroleptics.

Behavioral therapy

More recent developments show that patients suffering from Tourette syndrome can be trained to voluntarily respond to the urge that precedes the tics. By doing so, they can cancel out the urge to perform the tics involuntarily. These therapies are called competing response and awareness training. These are also sometimes done in conjunction with supportive therapy, such as counseling, to help patients with severe complex tic symptoms to cope with the embarrassment and social problems caused by their condition.

References:

  • Cohen S, Leckman JF, Bloch MH. Clinical assessment of Tourette syndrome and tic disorders. Neurosci Biobehav Rev. http://dx.doi.org/10.1016/j.neubiorev.2012.11.013.

  • Kurlan R. Clinical practice Tourette's Syndrome. N Engl J Med. 2010;363:2332-2338.

  • Ryan CA, Gosselin GJ, DeMaso DR. Habit and tic disorders. In: Kliegman RM, Stanton BF, St. Geme JW III, et al., eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 22.

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