In popular culture, certain repetitive and “nervous” movements that some people display under various situations are portrayed with a lot of humor. However, few are aware that they manifest them perhaps because they suffer from some type of disorder, as is the case of Tourette syndrome. Therefore, in the following article we present a complete definition of it, together with details about its symptoms, causes, comorbidity, diagnosis and treatment.
What is Tourette syndrome?
Gilles de la Tourette syndrome, more commonly known as Tourette syndrome, consists of a neuropsychiatric disorder in which the individual manifests verbal and motor tics involuntarily. These symptoms may appear during the first 10 years, but remain intermittent for the rest of your life. It was described by the neurologist Georges Gilles de la Tourette of French nationality, in the year 1885, and it is precisely in honor of him that this name has been given.
The particularity of this syndrome lies, as already said, in the intermittency of its signs despite its chronic nature. These can manifest at any time between 2 and 18 years of age, remaining constant for a period of around 10 years. After that, these may disappear for a period of time ranging from days to months, and then manifest again. In addition, symptoms manifest with greater intensity during childhood.
Regarding its prevalence, statistics indicate that 30 children out of every 10.000 develop Tourette syndrome; In addition, it is observed more frequently in males than in females. On the other hand, it is estimated that around 70% of patients who suffer from it have a immediate family member with tics or associated disorders to this, like the obsessive-compulsive.
At this point, it doesn't hurt to explain what a tic is. A tic It is a quick movement, which is performed repeatedly and involuntarily, which is generally stereotyped. The tics that manifest in Tourette syndrome are classified as simple and complex; depending on the number of muscles involved and the repetition time between each movement. They are defined below:
- The simple tics They include those brief and unexpected movements, the development of which depends on a small number of muscles. They tend to repeat themselves, but over relatively long periods of time.
- The complex tics They are those coordinated movements in which several muscle groups intervene. Likewise, they have a repetitive and successive nature, that is, they are executed between very short periods.
This is a rare disorder. However, history indicates that famous personalities such as Napoleon and Mozart suffered from it. Especially the latter, in whose biographies he is described as a person with a tendency to make a lot of faces, he played excessively with his hands and with unexpected gestures.
Symptoms of Tourette Syndrome
Regarding the signs of this syndrome, it is important to explain the two different types of tics that appear: vocal and motor, which in turn can be simple or complex. These are all involuntary, and although the patient may repress them for certain periods of time, they inevitably reappear.
vocal tics:
Simple:
- Flash continuously.
- Shake your head.
- Shrug.
- Grimaces and facial gestures.
Complexes:
- Jump, touch people or things.
- Turn around.
- Sniff.
- Echopraxia, a tic that consists of the involuntary repetition of the gestures and movements of another person.
- Copropraxia, a tic characterized by the tendency to make rude or obscene gestures.
Motor tics:
Simple:
- Cough.
- Hawk.
- Breathe in through your nose loudly.
- Blow.
- Click with your tongue.
Complexes:
- Expression of words or phrases outside the context of a conversation.
- Coprolalia, which occurs between 10% and 30% of cases, and consists of the use of phrases or words considered inappropriate in certain contexts. It is usually maintained during a single stage of life.
- Palilalia, which consists of the repetition of one's thoughts.
- Echolalia, which consists of the continuous repetition of words or phrases that have just been heard.
- In general, symptoms usually appear between 7 and 10 years of age. Among the first to manifest are simple ones at the level of the head and face, such as blinking, making recurring facial gestures and shaking the head. However, it is normal for the syndrome to appear suddenly, and for the individual to exhibit various motor and vocal symptoms.
- It is important to highlight that with regard to complex motor tics, coprolalia, echolalia and palilalia represent the most representative signs of the syndrome. Furthermore, they are the ones that make the patient's integration into different social environments the most difficult, since these behaviors are unpleasant or offensive for most people.
Comorbidity
Tourette syndrome presents a high comorbidity rate; that is, its coexistence with other unrelated pathologies. In fact, patients who suffer from this syndrome may simultaneously present obsessive compulsive disorder (60% of cases), attention deficit disorder, with or without hyperactivity (50% of cases), in conjunction with learning disorders, and even anxiety and humor.
Causes of Tourette syndrome
a) Genetic causes:
The main cause of TS is heredity. There is a dominant gene that is transmitted with a 50% probability, from parents to descendants, causing the tics that can trigger the diagnosis of the syndrome, or other related disorders. However, the conclusion is that the probability of developing it is greater in those people belonging to families in which there have already been cases.
b) Neurological causes:
Currently, it has been suggested that causes of Tourette syndrome They originate from the malfunction of the basal ganglia in the brain; These are the mechanisms responsible for motor control. In addition, it is also attributed to alterations in the systems dopaminergic, serotonergic and adrenergic.
c) Gender:
It is a syndrome with a higher incidence in males than in females. Thus, the probability of a boy developing TS is four times greater than that of a female from the same family.
d) Other diseases:
First, there are cases in which this disease originates simultaneously with the cortical-striatal-pallidal-thalamo-cortical disorder, which results from an autoimmune lesion in the putamen. In this case, this injury may be hereditary. Second, it is likely to develop due to an autoimmune disorder as a result of pharyngitis caused by bacteria such as group A beta-hemolytic streptococci.
Diagnosis of Tourette syndrome
The diagnosis of Tourette syndrome is based primarily on careful observation of the behavior of the affected person; In conjunction with an investigation into their history, it takes into consideration the age at which the tics began to manifest.
The main criteria for the specific diagnosis of this disorder are:
- Manifestation of several motor tics, and some vocal tics.
- Onset of symptoms at an age less than 20 years.
- The patient must present true motor and vocal tics, which must be differentiated from other abnormal movements, such as dystonia, chorea, ballismus, athetosis, hemifacial spasm, myokymias or myoclonus. Tics are sudden and repetitive movements, while these others go from one part of the body to another, at high speed but not repetitively. The exception here is hemifacial spasms, which do recur, but in Tourette syndrome grimaces do not.
- The intensity, frequency and form of the tics have varied throughout the patient's life.
- Tics should not have their origin in the administration of medications, and another similar disorder.
It is important to note that this condition cannot be diagnosed through blood tests or other laboratory studies. However, in view of the nature of the symptoms presented, the specialist could suggest neurological studies; These may include an electroencephalogram or MRI, to rule out other disorders such as hyperactivity, obsessive-compulsive disorder, or attention deficit disorder.
Once these other diseases have been ruled out through examinations and careful study of the signs, we then proceed to rule out the disorders most related to tics. Patients who develop vocal or motor tics for a year are diagnosed with transient nervous tic disorder. However, if their duration exceeds one year, then the diagnosis may be Tourette syndrome or chronic tic disorder.
Tourette syndrome treatment
The treatment of TS is based on the use of a variety of drugs that will help inhibit the most prominent symptoms.
There are different medications used in the treatment of Tourette syndrome. However, there is still no specific criterion based on which to prescribe them, in addition to comorbidity with other disorders. The most common compounds are antipsychotics, alpha-2 autoreceptor blockers, cyclic antidepressants and/or benzoadiazepines.
Antipsychotics
Generally, the group of most recommended antipsychotics are: olanzapine, risperidone, ziprasidone, haloperidol and pimozide. For its administration, it is generally started with small doses, which will then be gradually increased until the maximum doses or average effective doses are reached.
Antipsychotics are the most used in treatment of this disease, since they have shown greater effectiveness in the treatment of signs, however, there are side effects associated with their administration. In fact, their selection is somewhat complex, since it involves an evaluation of the patient's tolerance to reduce, to the extent possible, side effects.
For example, treatment is generally started with olanzapine and risperidone, which have a lower risk of causing akathisia and tardive dyskinesia in the patient, but could also cause weight gain, risk of diabetes, as well as dysphoria and depression. , in the exclusive case of the administration of risperidone.
Alpha-2 autoreceptor blockers
In this group are guanfacine and clonidine, the latter being the most used. Clonidine, compared to antipsychotics, has a much slower response that can even be seen months after starting its administration. That is why its doses tend to be increased gradually; However, this is done by stopping from the beginning, in order to verify its effects, and once they are stabilized, continue the treatment with a higher dose, repeating this method. The average dose comprises 0,5 mg.
Tricyclic antidepressant
As for cyclic antidepressants, desipramine is used, highly recommended in adult patients, but not for children, since for them it results in cardiotoxicity. Its doses start with 25 mg, and are increased over the days until reaching approximately 100 mg, which is the average dose. At this point special attention is paid to the evolution of the individual.
Benzoadiazepine
The benzodiazepine used for Tourette syndrome treatment is clonazepam, whose initial dose is usually 0,5 mg. This increases over the days, until the desired effects or, on the contrary, side effects are observed. Its average dose is 2 mg, and the maximum is 4 mg.
We hope that this article about this syndrome has been detailed enough to leave no doubt. However, you are free to leave any questions, opinions or experiences in the comment box; We will try to respond to you as soon as possible.