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Tourette's syndrome is a neuropsychiatric disease that begins in childhood and manifests itself in multiple motor and vocal tics, as well as a combination of behavioral disorders that often dominate the clinical picture. The latter include symptoms of OCD and attention deficit hyperactivity disorder (ADHD). Tourette's syndrome is named after the French neurologist Georges Gilles de la Tourette, a disciple of Charcot, who in 1885 described 9 cases that correspond to the current definition of the syndrome (Tourette, 1885). However, the first medical observation of the case, which can be attributed to Tourette's syndrome, was presented by the French doctor Itard (Itard, 1825). He described the French aristocrat, who was forced to spend her life in solitude because of involuntary shouting of swear words. But the earliest in the history of mankind, the mention of Tourette's syndrome, appears to be contained in the treatise on witchcraft Malleus Maleficaram ("The Hammer of Witches"). It tells about a man who lived in the XV century:
"When he entered any church and knelt before the Virgin Mary, the devil forced him to put out his tongue. When he was asked why he could not restrain himself, he replied: "I can not help myself, because he commands all my limbs and organs, my neck, my tongue, my lungs, as he pleases, forcing me to speak or shout; I hear the words as if I were uttering them myself, but absolutely unable to resist them; when I try to pray, he controls me even more imperiously, pushing my tongue out. "
In accordance with modern terminology, the involuntary exposure of the tongue can be defined as copropraxia, a kind of complex motor tick (see below). One can argue that blasphemous thoughts are obsessions (obsessive, disturbing a sick idea), but the actions of the patient arising under their influence, unlike compulsions, do not neutralize this discomfort, but rather give rise to a new one. Although patients with OCD may experience anxiety due to the actions caused by unwanted impulses, in practice this is rarely observed.
During the course of life, Tourette's syndrome and close to it chronic tics are revealed in 3.4% of people and almost in 20% of children engaged in special schools. The males suffer more often than the females. Manifestations of Tourette's syndrome can persist throughout the life of the patient and significantly disrupt his social adaptation. Unfortunately, there have been no significant advances in the treatment of Tourette's syndrome lately.
Pathogenesis of Tourette's Syndrome
It is believed that Turetg's syndrome is inherited as a monogenic autosomal dominant disease with high (but not complete) penetrance and variable expressiveness of the pathological gene, manifested in the development of not only Tourette's syndrome, but also OCD, chronic ticks-XT and transient tics-TT. Genetic analysis shows that XT (and possibly TT) can be a manifestation of the same genetic defect as Tourette's syndrome. In a study of twins, it was found that in monozygotic pairs the level of concordance is higher (77-100% for all ticks) than in dizygotic vapors - 23%. At the same time, identical twins have pronounced discordance in terms of the severity of tics. A genetic linkage analysis is currently underway to identify the chromosomal location of a possible Tourette syndrome gene.
Symptoms of Tourette's Syndrome
Ticks include a wide repertoire of motor or vocal acts, which the patient feels as violent. Nevertheless, they can be delayed by willpower for a while. The extent to which tics can be delayed varies depending on their severity, type and timing characteristics. Many simple and fast-performing ticks (for example, quick flashing movements or twitching of one's head) can not be controlled, while other ticks that more closely resemble targeted movements, as they arise in response to an inner imperative call, can be delayed. Some patients try to disguise tics. For example, a teenager scratching the perineum can substitute socially more acceptable touches to the abdomen. Over time, the localization of tics and their severity changes - some tics can suddenly disappear or be replaced by others. Such changes sometimes cause the erroneous impression that patients are able to arbitrarily eliminate some tics and perform others. A survey of patients showed that about 90% of them have an unpleasant feeling before the teaches, which forces the patients to perform an action or produce a sound and can be designated as an imperative urge.
Diagnostic criteria and methods for evaluating Tourette's syndrome
Transit ticks are common - about a quarter of schoolchildren. The diagnosis is established with the preservation of ticks for at least 4 weeks, but not more than 12 months. The development of chronic tics or Tourette's syndrome may be preceded by several episodes of transient tics. Chronic ticks (XT) include motor or vocal tics (but not their combination), which persist for more than 1 year. The diagnostic criteria for Tourette's syndrome require a combination of multiple motor tics and at least one vocal tic, not necessarily at the same time. For example, in a 16-year-old youngster with multiple motor tics, but without vocal tics at the time of the examination, Tourette's syndrome should be diagnosed if vocal ticks were observed at the age of 12 years. Many consider the differences between Tourette's syndrome and chronic multiple motor tics artificial, especially considering the similar nature of inheritance according to genealogical research. Symptoms of Tourette syndrome should last more than 1 year, while the duration of remission should not exceed 3 months. According to DSM-TV, the disease should appear before the age of 18, although this criterion has varied in the past. If the tics appear later than 18 years, they should be qualified as "ticks without additional clarifications".
Drugs used in Tourette's syndrome
First of all, the doctor must decide whether pharmacotherapy for a given severity of symptoms is indicated. Tests of drugs with Tourette's syndrome are complicated by a wave-like course with exacerbations and remissions that do not necessarily occur under the influence of drugs. For short-term fluctuations in the severity of symptoms, it is not necessary to respond with an immediate change in the treatment regimen. The overall goal of treatment is to partially relieve the symptoms: complete drug suppression of tics is unlikely and is associated with the occurrence of side effects.
Special educational programs are needed for the patient, his family and school personnel, contributing to an understanding of the characteristics of the disease and the development of tolerance for symptoms. Comorbid disorders can be the main cause of discomfort and impaired social adaptation. Adequate therapy of comorbid DVG, OCD, anxiety and depression sometimes reduces the severity of tics, probably due to the improvement of the patient's psychological state and the easing of stress.