Tourette syndrome

Tourette syndrome is a neuropsychiatric disorder with onset in childhood or adolescence (before the age of 21). It is characterized by multiple motor tics (movements) and at least one tic. vocal or phonic (words or sounds), lasting for more than a year from the onset of the first tic. These tics characteristically wax or wane in number, intensity, and type, with periods of exacerbation or remission. They are involuntary, although they can be temporarily suppressed, and are preceded by a premonitory impulse, comparable to the sensation that occurs before a sneeze. Tourette's syndrome is defined as part of a spectrum of tic disorders, including tics transitory and chronic.

Tourette's syndrome was once considered a rare and bizarre syndrome, often associated with the exclamation of obscene words or socially inappropriate and derogatory comments (coprolalia), but this symptom is only present in a small minority of sufferers. Tourette syndrome is no longer considered a rare syndrome, but it is not always correctly diagnosed because most cases are mild and the severity of tics decreases in most children by the time they reach adolescence. Between 0.4% and 3.8% of children ages 5 to 18 may have Tourette syndrome; The prevalence of transient and chronic tics in school-age children is high, with the most common tics being eye blinking, coughing, throat clearing, sniffing, and facial movements. A severe Tourette's in adulthood is a rarity, and Tourette's syndrome does not adversely affect intelligence or life expectancy.

It can affect people of any ethnic group and any gender, although men have it about three times more often than women. The natural course of the disease varies among patients. Although the symptoms range from mild to very severe, in most cases they are moderate.

There is no known cure for Tourette syndrome. Treatment consists of relieving symptoms when they interfere with daily tasks, using certain drugs such as haloperidol or pimozide, although they can cause adverse side effects. Preliminary evidence suggests a relationship with the consumption of gluten and the improvement of symptoms, both in tics and associated disorders, after its strict withdrawal from the diet.

History

A French physician, Jean Itard, first described in 1825 the case of an 86-year-old French noblewoman with the disease, the Marquise de Dampierre.

From 1890 a neurologist, also French, Georges Gilles de la Tourette, following the instructions of Professor Jean Martin Charcot, deepened studies on movement disorders, and found an article in the Journal of Nervous and Mental Disease, whose author was the American doctor George Beard and described a group of French Canadians, who suffered from contractions, abnormal movements, echolalia, echopraxia, and difficulty controlling "tics". Tourette translates the article into French and publishes it in the journal Archives de Neurologie.

Tourette continues the search for scientific papers that describe symptoms of the same type and analyzes the cases called Latah or Miryachit.

Then he set out to review the medical records of the Salpêtrière hospital in Paris and there he found the case of a 15-year-old boy who presented episodes of hyperexcitability, tics, shaking his head, trunk, pronouncing obscene words and in dialogue with other people used to repeat the last words spoken by the interlocutor.

Gilles de la Tourette finds coincidences between the cases described by his colleagues and the one he had found in the Parisian hospital. And in 1885 he published in the magazine Archives de Neurologie an article entitled “Étude sur une affection nerveuse caractérisée par de l'incoordination motrice accompagnée d'écholalie et de coprolalie (jumping, latah, and myriachit)” ("Study on a nervous condition characterized by motor incoordination accompanied by echolalia and coprolalia (jumping, latah and myriachit)").

His research leads him to distinguish this disease as a neurological disorder, although the cognitive conditions are normal and, in agreement with Charcot, he determines that it was degenerative and incurable. The latter, being impressed by the work of his disciple, decides to name the new entity "Tourette syndrome".

Epidemiology

The prevalence of Tourette syndrome in school-age children worldwide is around 1%, with a clear predominance in males compared to females, with a ratio of 3:1.

Symptoms

Tourette syndrome begins in childhood or adolescence, before the age of 21.

This is a condition that causes a person to make rapid, repetitive movements or sounds that they cannot control.

It is characterized by the presence of multiple motor tics and at least one vocal or phonic tic. They are generally intermittent, although they do not necessarily occur simultaneously. Its frequency is variable, with periods of intensification and remission, which persist for more than a year from the appearance of the first tic.

There is a characteristic of tics that can cause a lot of suffering and feelings of guilt, especially in children. It is about the fact that, although in reality the tics are involuntary, a premonitory feeling is usually produced, which can lead to confusion for believing that they make the movements or sounds voluntarily. A good comparison is the sensation that is experiences before a sneeze, in which the urge to sneeze may be felt, but cannot be avoided.

The severity of tics worsens throughout childhood, and for most people, the worst period of tics occurs between 8 and 12 years of age.

Up to 80% of people with Tourette syndrome experience a significant decrease in tics during adolescence. By the age of 18, the intensity and frequency of these tics usually decreases to such an extent that the person no longer experiences impaired quality of life due to their tics.

However, objective assessments indicate that up to 90% of adults continue to display mild tics, although they sometimes go unnoticed.

Associated disorders

In up to 90% of cases, associated disorders appear. These include mainly obsessive-compulsive disorder (OCD) and attention deficit hyperactivity disorder (ADHD).

Associated OCD can appear during childhood, adolescence, or early adulthood. When its onset is in childhood, it only remits in adulthood in 40% of cases. It can also develop during adolescence or early adulthood.

Causes

Although the cause of Tourette syndrome is unknown, current research reveals abnormalities in certain brain regions (including the basal ganglia, frontal lobes, and cerebral cortex), the circuitry that interconnects those regions, and neurotransmitters (dopamine, serotonin, and norepinephrine) that carry out communication between nerve cells. Given the often complex presentation of Tourette syndrome, the cause of the disorder is surely equally complex.

A new possibility is the existence of a relationship with non-celiac gluten sensitivity. Preliminary evidence suggests that children with Tourette syndrome react to dietary gluten, causing new or worsening symptoms. In 2015, Rodrigo et al. documented the first case of complete remission of Tourette syndrome symptoms, both tics and the associated obsessive-compulsive disorder (OCD), solely through a gluten-free diet and without drug treatment. The patient did not show appreciable digestive symptoms nor did she meet the criteria for the diagnosis of celiac disease.

In 2018, this same researcher published the first series of patients, including children and adults, in whom there was a clear improvement in both tics and OCD after a year of a gluten-free diet and found that the symptoms reappeared or worsened as a consequence of accidental contamination with gluten, subsiding after several days or even weeks once the strict gluten-free diet was resumed. to the subgroup of people whose disorder does not improve or even worsens with age, show that the improvements cannot be justified by the passage of time or by a hypothetical spontaneous remission.

Associated disorders

There is a close relationship between tic disorders (TTs) and Tourette syndrome. Not all people with Tourette syndrome have disorders other than tics. However, many people experience additional problems (comorbidity) such as obsessive-compulsive disorder (OCD), in which the person feels that something "must" be done repeatedly; attention deficit disorder (ADHD), in which the person has difficulty concentrating and is easily distracted; various developmental learning disorders, which include difficulties with reading, writing, arithmetic, and perceptual problems; and sleep disturbances, including frequent waking or sleep talking.

The wide variety of symptoms that often accompany tics can cause more limitations than the tics themselves. Patients, families and physicians need to determine which symptoms cause the most limitations in order to choose the most appropriate medications and therapies.

Diagnosis

Tourette syndrome is usually diagnosed by looking at symptoms and evaluating family history. There are no blood tests or other laboratory tests that can diagnose the disorder. According to the DSM-V, the following criteria must be met:

• Having presented in some period of the disease multiple motor tics and at least one vocal, they don't have to be given all at once. Although in some cases it may occur 2 at a time.

• For more than a year, tics appear several times a day, often in the form of waves. This happens almost daily or intermittently, with periods free of tics that never exceed three consecutive months.

• The disorder interferes with the normal performance of the person, affecting his/her family life and his/her school, social or work activity.

• The first episodes of tics occur before the age of 18.

• The disorder is never due to the direct effects of any medication, such as stimulants, or a medical condition, including Huntington's disease, Sydenham's chore, Parkinson's disease, Wilson's disease, post-viral encephalitis, dystonies, dyskines, choreas, atetosis, myocylonies, hemi-balism, compulsions, manierism. The tics are distinguished from stereotyped movements because the latter are volunteers and do not cause subjective discomfort, unlike the tics, which are involuntary and cause discomfort.

Neuroimaging studies, such as magnetic resonance imaging (MRI), computed tomography (CT), and electroencephalogram (EEG), or various blood tests, may be used to exclude other conditions that may be confused with TS.^{[ citation required]}

Treatment

Drugs

Because the symptoms are not limiting in most patients and their development proceeds normally, most people with Tourette syndrome do not require medication. However, medications are available to help patients when symptoms interfere with daily tasks.

There is no single medication that works for everyone with Tourette syndrome. Also, there is no medicine that eliminates all symptoms and all medicines have side effects. Furthermore, the available medications can only reduce specific symptoms.

Some patients who need medication to reduce the frequency and intensity of tics can be treated with neuroleptic drugs such as haloperidol and pimocide. These drugs are usually given in very small doses that are slowly increased until the best possible balance between symptoms and side effects is achieved.

Long-term use of neuroleptic drugs can cause an involuntary movement disorder called tardive dyskinesia. However, this disease usually goes away when you stop taking the medicine. Short-term side effects of haloperidol and pimocide include muscle stiffness, drooling, tremors, lack of facial expression, slow movement, and restlessness. These side effects can be reduced by drugs commonly used to treat Parkinson's disease. Other side effects such as fatigue, depression, anxiety, weight gain, and difficulty thinking clearly may be more bothersome.

Clonidine, an antihypertensive drug, is also used to treat tics. Common side effects associated with the use of clonidine are fatigue, dry mouth, irritability, dizziness, headaches, and insomnia. Fluphenazine and clonazepam may be prescribed to help control tic symptoms.

Medications are also available to treat some of the disorders associated with TS. Stimulants such as methylphenidate, pemoline, and dextroamphetamine, usually prescribed for attention deficit disorder, are somewhat effective, but their use is controversial because they have been reported to increase tics. For obsessive-compulsive behaviors that significantly interfere with daily functioning, fluoxetine, clomipramine, sertraline, and paroxetine may be prescribed. Additionally, another of the medications for Tourette syndrome that is also used to treat ADD (attention deficit disorder) is Strattera (atomoxetine).

Behavioral Therapy

Behavioral therapy is a treatment that teaches people with TS different ways to control tics. Behavior therapy is not a cure for tics. However, it can help reduce the number, severity, and impact of tics, or a combination of all of these factors. It is important to understand that while behavioral therapies can reduce the severity of tics, this does not mean that tics are merely psychological or that anyone with tics will be able to control them.

Diet

To date (2017), very few studies have been carried out on the effect of dietary interventions on Tourette's symptoms and the few studies carried out are methodologically poor. Preliminary evidence shows that gluten-free diets and those low in refined sugars can alleviate Tourette's symptoms.

Hereditary factor

Evidence from genetic research suggests that it is dominantly inherited and that the gene(s) involved can cause a variable range of symptoms in different family members. A person with this syndrome has a 10% chance of passing the gene or genes to one of her children. However, this genetic predisposition does not necessarily result in the full syndrome. Instead, the syndrome is expressed in a milder tic disorder, obsessive-compulsive behavior, or attention deficit disorder, with few or no tics. It is also possible that offspring carrying the gene will not develop any TS symptoms. A higher than normal incidence of mild tic disorders and obsessive-compulsive behaviors has been found in families of individuals.

Sex plays an important role in the gene expression of ST. If a patient's offspring carrying the gene is male, the risk of developing symptoms is 3 to 4 times higher. However, most people who inherit the genes do not develop symptoms severe enough to warrant medical treatment. In some cases inheritance cannot be established. These cases are called sporadic and their cause is unknown.

Forecast

Although there is no cure for Tourette syndrome, many patients improve as they mature. Individuals do not see their life expectancy reduced. Although the disorder is chronic and lifelong, it is not a degenerative disease. Tourette's syndrome does not impair intelligence. Tics tend to decrease as the patient ages, allowing some patients to stop using medications. In some cases, a complete remission occurs after adolescence.

School difficulties linked to Tourette syndrome

Students affected by Tourette syndrome need specific school support. To do this, both parents and education professionals (teachers, educators, psychologists, educational psychologists, among others) must be duly informed about how tics and other symptoms of the syndrome (TS) can affect performance and behavior of a student.

School alternatives for students with Tourette syndrome

Depending on the severity of the tics and the attitude towards them of teachers, students and even himself, social, emotional and academic relationships may be affected.

Tourette syndrome can affect school learning in many ways, both positively (increased creativity) and negatively (increased inattention).

If the learning problems are mild, they can be overcome through in-class support or small adaptations, but if we are dealing with a severe case of tics or other disorders associated with tics, specially adapted curricular programs and interventions may be required.

From adolescence (where the most critical levels are reached) learning problems usually decrease thanks to the help provided and the adaptations achieved.

The incidence of associated disorders and tics themselves on school performance

Children's efforts to control their tics can cause their attention span and attention span to decrease markedly.

Tics affect almost all areas of learning such as: Calculation, problem solving, writing, reading, handling utensils, etc.

Tourette syndrome may be associated with ADD and hyperactivity; in many cases, this disorder appears before the tics. It can also be associated with obsessive-compulsive disorder, which also greatly influences the ability to concentrate, because it generates stress and anxiety.

Another disorder that is associated with Tourette syndrome is depression and, in general, mood disorders, since it influences both concentration and self-esteem, motivation, security, etc.

Tourette syndrome and intelligence

Intelligence is not affected in this entity; most have average or above average intelligence. But students with Tourette syndrome can have learning problems, especially because of attention and behavioral disturbances.

The intervention of teachers and teaching teams (EOPs)

Both teachers and EOP should have a good knowledge and understanding of ST. This is necessary so that they know how to minimize the attention and importance of the student's tics, contributing to the reduction of stress in the student, with which the tics can manifest with less intensity and on fewer occasions, in addition to avoiding greater interpersonal conflicts. This attitude will help the children's peers to do the same and learn to tolerate these symptoms. This favors the integration of the child.

Teachers must also cover emotional needs, such as empathy and understanding, to reduce negative emotions and to prevent fights, teasing, isolation and rejection, among others.

Measures to help students with Tourette syndrome in the school setting

• Maintenance of school habits and routine: this helps the student to calm down and perform structured, orderly and predictable space-time situations. Sit in the same place, tasks in the same order, etc. Any change, such as excursions, new teachers, constant group or school mute, can increase stress and nervousness, so it will increase hyperactivity, tics and symptoms in general. But it is desirable that constancy and structuring do not become isolation or rigidity, because as with any person, new experiences need to appear in your life that will allow you to expand your interests and learnings. This will be done with the necessary support.

• Guidelines and habits should be followed to prevent or improve attention and concentration difficulties. To do this, it is advisable to use different notebooks for each subject, archivalators, make a list of the tasks to perform, among other measures that allow it to carry and maintain better control of its activities. The use of computers (computers) involves improving your attention capacity and maintaining a better calligraphy.

• Trust the student with Tourette syndrome to perform certain tasks that involve a motor activity or the possibility of getting out of the classroom, such as deleting the board, inmates to other teachers, etc. This gives him the opportunity to release his tics and to foster his responsibility.

• Students may have rare or inappropriate behaviors; it is appropriate to act on these behaviors only to the extent that they distort or interfere notoriously with the normal development of the class, with relationships with their peers or with their own learning.

• Its proper conduct and behaviour must be strengthened and encouraged. They need to be praised and to recognize their efforts, creativity, spontaneity, control their impulses, etc. In the event that any punishment is imposed, the child must be told of the motive that originated him or her and the conduct that he or she should have had, being especially careful not to punish him or her as a result of his or her suffering.

• Integrating students with ST helps them with their self-esteem and social skills.

• Avoid labeling it, as "vago", "caprichoso", "dispisted", etc.

• Physical exercise is important to eliminate stress and develop psychomotor skills. In this area you need to be careful to integrate the student well into the team games, since otherwise they would increase the nervousness and tics in the student. The activities must also be adapted so that they can be done without problems (supply effects of some medications, etc.).

• It is of the utmost importance that he is not prevented from performing any activity or treated as incapable of actions that any other child of his or her age may perform. This only leads to psychomotor or intellectual delays that are not the consequence of the suffering itself. In activities where potential danger is perceived, you should only be aware of the importance of caution, as well as an adult staying closer and attentive in case of requiring any help.