Tourette Syndrome, named after the neurologist Gilles de la Tourette, is a disorder characterized primarily by involuntary repetitive movements and described as “exhausting” by the sufferer. According to the National Consensus on Tourette Syndrome, the prevalence of the syndrome is stated to be 0.3-0.8% of the pediatric population and more common in boys than in girls.
In this article we talk to Dr. Javier Pagonabarraga, specialist in Neurology, and the coordinator of the Consensus, about what the syndrome is, what causes it and what its prognosis is.
What is Tourette syndrome and how does it manifest itself?
Tourette syndrome is a disease that starts in childhood, before the age of 21, and usually begins to manifest itself around the age of seven. There are infants who, with three or four years already can manifest their first tics, which are the neurological symptom guide in the diagnosis, but most of them, they begin to manifest the first tics between the six or seven years of life.
Which are the symptoms that define the Tourette syndrome?
The main symptoms are motor tics and phonic tics. The motor tics can be sudden, accelerated, of short duration and involuntary movements that appear predominantly in the region of the face, in the neck and in the shoulders. The most characteristic ones are the blinking, the involuntary blinking of the eyes, grimaces with the mouth, movements with the shoulders or the neck, that sometimes are abrupt and generate discomfort to the boys or girls who have it. On the other hand, the phonic tics that, many times are necessary for the diagnosis, are noises that are emitted in an involuntary way. The most frequent are: throat clearing sounds, coughing sounds, sniffing noises, mucus throwing inwards, involuntary noises, etc. In more severe cases, which occurs in only 10-15% of patients, they may emit involuntary words, either the name of objects they see (vocalizations) or, in the most severe case, they may emit insults (coprolalia). But coprolalia, as famous as it may seem, is not usually the most common symptom, only this imperious need to swear appears in this small percentage of patients.
Other symptoms
Apart from the tics, which can already be very serious and tiring for the infant because they have an impact on their social relationships with friends or family, approximately 30-40% of children with Tourette syndrome with motor and phonic tics can also have attention deficit hyperactivity disorder (ADHD), which is more frequent in Tourette’s than in the general population, and which can give problems of academic performance with a tendency to have a difficulty to organize tasks, pay attention, to do homework or to keep school material tidy, and also some children with Tourette’s may have obsessive compulsive traits, which if they are very severe, end up meeting the criteria for obsessive compulsive disorder and, basically, what happens is that they end up having recurrent thoughts in their mind based on negative events that have happened in their day to day life and also have ideas of what is going on in their lives. of doubt and verification, for example: they have doubts if they have done well what they had to do, doubts if they have closed the window of their room, doubts if they have said something to someone they wanted to say something to. These ideas, which are called intrusive or obsessive, can cause problems for these children.
What are the possible causes of this syndrome?
Fundamentally, it is a very genetic syndrome, it has a high heritability in such a way, that when we visit children with Tourette’s, with motor and phonic tics, many times some relative also has tics. Therefore, there is a very important genetic base in this disease. Then, on a genetic base that confers a state of vulnerability, there are certain environments with many stressors that can favor that the tics manifest themselves in a clearer way. But the cause of the disease is genetic.
What prognosis or day to day life can have the people who suffer from it?
Not all the people who have tics fulfill the diagnosis of the Syndrome of Tourette. The tics of the syndrome are frequent, changeable and that persist in the time (they do not last a few months and then disappear) and tend to be present during their life, especially during the childhood, towards the 10-14 years they usually intensify and then they can diminish, from the 16 to the 25 years. But they are boys and girls who spend many years with tics that generate an important emotional impact. It is true that many boys or girls with Tourette’s, like the singer Billie Eilish, recognize that it is something exhausting because these tics are involuntary movements against which they have to fight. It is part of their life to learn to recognize them, to learn to try to look for a way out or an alternative that allows them to live a more normalized life in the family or social environment.
I would like to transmit the message that an infant with tics is a patient who deserves a very personalized medical attention, trying to explain what happens to him and trying to look for solutions, so much with drugs, if it is necessary, as with different psychological therapies that nowadays are being developed so that the infant knows his tics and tries to look for alternative solutions to that involuntary movements or sound that he or she does not want to have.