Brief overview
- Symptoms: Involuntary, uncontrollable movements and vocalizations (tics) such as eye blinking, jumping, twisting, stomping, clearing the throat, grunting or uttering words
- Causes: Disturbance of the neurotransmitter metabolism in the brain due to hereditary factors and environmental triggers (for example, smoking or stress during pregnancy)
- Diagnosis: Based on medical history and typical symptoms, which can be evaluated with the help of questionnaires.
- Course and prognosis: Onset usually in elementary school age, often decreasing symptoms from adolescence into adulthood.
What is Tourette’s syndrome?
Tourette syndrome is not a mental disorder, but a neuropsychiatric disorder. In tic disorder, the filtering functions of motor control fail. Tourette’s usually begins in childhood, more rarely in adolescence. Younger children in particular often go through a phase with tics, but these disappear on their own after a few months.
Experts estimate that around one percent of people develop Tourette syndrome. However, only a small proportion are affected to such an extent that the condition requires treatment. Boys are affected four times as often as girls. The reasons for this are still unknown.
The French physician Gille de la Tourette described the disorder for the first time in 1885; he is the eponym for the disorder, whose full name is “Gilles-de-la-Tourette syndrome”.
The Tourette Syndrome Severity Scale (TSSS) can be used to determine the severity of the tic disorder:
- Low impairment: The tics do not interfere with behavior at school or work. Outsiders hardly notice the disorder. The affected person perceives them as unproblematic.
- Moderate impairment: The tics are noticeable to outsiders, so there is always irritation. They also make it difficult to perform certain tasks at school or at work.
What are the symptoms of Tourette syndrome?
Tourette syndrome manifests itself in so-called tics. These are involuntary movements or vocalizations. The term tic comes from the French and means something like “twitching”. Doctors distinguish between motor and vocal tics as well as simple and complex tics.
Motor tics
Motor tics are abrupt, often violent movements that serve no purpose and always occur in the same way.
Complex motor tics are tics that involve multiple muscle groups. These include, for example, hopping, turning, or touching objects or people. Obscene gestures also appear (copropraxia). Sometimes self-injurious acts occur – sufferers bang their head against the wall, pinch themselves or stab themselves with a pen.
Vocal tics
Complex vocal tics are words or sentences that affected persons literally hurl out and that have no logical connection to the situation.
Tourette’s syndrome has become known in the media in particular for the fact that affected persons involuntarily utter obscenities or swear words (coprolalia). In fact, this tic occurs in only about ten to 20 percent of those affected.
Variable clinical picture
Sometimes the tics announce themselves by sensorimotor signs, for example tingling or feelings of tension. These unpleasant sensations disappear when the tic is performed. As a rule, however, those affected also only notice the tic when it appears. Simple, mild tics such as eye blinking are often not even noticed by the sufferers themselves until they are made aware of them.
During emotional excitement such as joy, anger or fear, the symptoms intensify. The same applies to stress, but also to some extent to phases of relaxation. If the affected person is strongly focused on one thing, the tics decrease.
The tics do not disappear during sleep and occur in all stages of sleep. However, they are then attenuated. As a rule, the affected person has forgotten the occurrence of the tics by the next morning.
Other disorders
Very many people with Tourette syndrome develop other disorders. These include:
- attention deficit hyperactivity disorder (ADHD)
- Obsessive-compulsive disorder
- Sleep disorders
- Depression
- Anxiety disorders
- Social phobias
What are the causes of Tourette syndrome?
However, for it to develop, additional triggers in the environment must be added. These include, for example, negative factors during pregnancy and birth such as smoking, alcohol consumption, medication use, drugs, psychosocial stress, prematurity, and lack of oxygen during birth. In addition, bacterial infections with certain streptococci are considered possible triggers of Tourette syndrome.
Disturbed neurotransmitter metabolism
A disturbed household of other neurotransmitter systems, such as serotonin, norepinephrine, glutamine, histamine and opioids, as well as the interactions between these substances also seem to play a role.
The disorders primarily affect the so-called basal ganglia. These brain areas are located in the deeper structures of both cerebral hemispheres and fulfill a kind of filtering function. They regulate which impulses a person translates into actions and which do not.
Tourette’s syndrome is often diagnosed years after the first symptoms appear. Since the disorder causes misunderstandings and annoys fellow people, this is problematic. The children may be seen as cheeky and stiff-necked, and the parents worry because their upbringing does not seem to be bearing fruit. In such cases, the diagnosis is a relief for all concerned.
Important questions for the attending physician are:
- How do the tics manifest themselves?
- Where, how often and how strongly do they occur?
- Does stress have an exacerbating influence on the symptoms?
- Can the tics be suppressed?
- Do they announce themselves by some kind of premonitory sensation?
- At what age did the tics first appear?
- Do the symptoms change in terms of type, intensity and frequency?
- Have there been any cases of Tourette syndrome within the family?
Since the tics do not always occur, it may be helpful for the doctor’s visit to record them in a video beforehand.
Exclusion of other diseases
To date, there are no laboratory tests or neurological and psychiatric examinations for Tourette syndrome that can be used to make a diagnosis. Therefore, examinations are primarily used to rule out other causes of tics or tic-like symptoms. These are, for example:
- Brain tumors
- Epilepsy
- Inflammation of the brain (encephalitis)
- Chorea (various malfunctions of the basal ganglia that result in involuntary movements)
- Ballismus (neurological disorder in which affected individuals make abrupt slingshot-like movements)
- Myoclonus (involuntary, sudden short muscle twitches of various origins)
- Streptococcal infections
Treatment
There is currently no cure for Tourette syndrome. Existing therapies improve the symptoms, but have no influence on the course of the disease. Nevertheless, there is a whole range of offers that make life with Tourette syndrome easier.
It is crucial to treat concomitant illnesses in addition to Tourette’s syndrome, such as ADHD, obsessive-compulsive disorder, and sleep disorders. Often, this also improves the tics.
Psychoeducational counseling
If the feeling of strain diminishes, the stress caused by the disease also decreases. In this case, it may be sufficient to just observe the disease and only take further action if it worsens.
Behavioral therapy treatment
In HRT, those affected train their self-awareness. As a result, they become more aware of the tics and learn to interrupt the automated behavioral chains with alternative actions.
In addition, the psychological consequences of the disease can be addressed with behavioral therapy measures. These include damaged self-esteem, insecurity in dealing with other people, social phobias, anxiety disorders and depression. Learning a relaxation technique complements behavioral therapy. It can help relieve stress that would otherwise exacerbate symptoms.
Medication
- Suffers from pain due to tics (e.g., neck, back pain) or self-injures.
- is socially excluded, teased or bullied because of his or her tics. This is especially the case with vocal tics and with strong motor tics.
- Has emotional problems such as anxiety, depression, social phobias, or low self-esteem due to his or her disorder.
Most medications used to treat Tourette’s syndrome target dopamine metabolism in the brain. The so-called dopamine receptor antagonists dock onto the various dopamine receptors and block them for the brain messenger. These include, in particular, the various representatives of antipsychotic drugs (neuroleptics), such as haloperidol and risperidone. They are considered the drugs of first choice for the treatment of Tourette’s syndrome.
- Tetrabenazine, a dopamine memory depleter
- Topiramate, an antiepileptic drug
- Noradrenergic agents such as clonidine, guanfacine, and atomoxetine (especially if concomitant ADHD is present)
- Cannabis-based agents (cannabinoids) such as tetrahydrocannabinol
- Botulinum toxin for tics that are permanent and limited to readily accessible muscles
Operations: Deep brain stimulation
For adults whose quality of life is severely limited by Tourette’s syndrome and who are not sufficiently helped by other therapies, deep brain stimulation is an option. For this purpose, the doctor implants a brain pacemaker under the abdominal skin, which electronically stimulates the brain via electrodes.
Course of the disease and prognosis
In general, the prognosis is favorable. In two-thirds of children, the symptoms improve significantly over time or even disappear completely. From the age of 18, the tics have diminished in most of them to the point where they are no longer a nuisance.
For the remaining third, however, the prognosis is less favorable. In some of them, the symptoms are even more pronounced in adulthood. The loss of quality of life is particularly great for them.
Living with Tourette syndrome
For some sufferers, these misunderstandings and rejection by the environment understandably make them reluctant to go out among people. It is also difficult for people with severe Tourette’s to pursue certain professions, especially those with a lot of social contact.