Tourette's Syndrome - Symptoms.

Symptoms of Tourette Syndrome

Tics include a wide repertoire of motor or vocal acts that the patient experiences as forced. However, they can be inhibited by an effort of will for some time. The degree to which tics can be inhibited varies depending on their severity, type, and temporal characteristics. Many simple and rapidly performed tics (for example, rapidly following one another blinking movements or head jerks) are not amenable to control, whereas other tics, which are more reminiscent of purposeful movements, since they occur in response to an internal imperative urge, can be inhibited. Some patients try to disguise tics. For example, an adolescent may replace scratching the perineum with more socially acceptable touching of the abdomen. Over time, the location of tics and their severity change - some tics may suddenly disappear or be replaced by others. Such changes sometimes create the erroneous impression that patients are able to voluntarily eliminate some tics and perform others. A survey of patients showed that approximately 90% of them experience tics preceded by an unpleasant sensation that forces patients to perform an action or make a sound and can be described as an imperative urge.

The intensity of tics can be influenced by a number of factors. During sleep, tics decrease, but do not disappear completely. Tics often become more noticeable in a state of relaxation (for example, if the patient is watching TV at home), as well as during stress. Tics can significantly decrease and even disappear if the patient is concentrating on some activity. For example, here is a description of a surgeon (before and during the operation) given by the famous English neurologist and writer Oliver Sacks (1995): "... his hands were constantly in motion. Every now and then he almost touched (but never quite) his unsterile shoulder, assistant, mirror, made sudden movements of the body, touched colleagues with his foot. A flurry of vocalizations was heard - "Uh-uh" - as if a huge owl was somewhere nearby. Having treated the surgical field, Bennett took a knife, made a neat, even incision - there was not a hint of any tic excessive movement. The hands moved strictly in accordance with the rhythm of the operation. Twenty minutes passed, fifty, seventy, a hundred. The operation was complicated: it was necessary to tie off vessels, find nerves - but the surgeon's actions were skillful, precise, and there was not the slightest hint of Tourette's syndrome..."

Associated disorders

Patients with Tourette syndrome often have comorbid disorders, which can be a significant factor in the maladjustment of patients. However, despite numerous obstacles, many patients achieve success in life. A great example is Samuel Johnson, one of the most outstanding personalities in English literature of the 18th century. He suffered from severe Tourette syndrome with pronounced obsessive-compulsive symptoms. He also had autoaggressive actions and symptoms of depression.

It remains debatable whether concomitant disorders should be considered an integral part of the clinical picture of Tourette syndrome or just comorbid conditions. Data on the genetic link between OCD and Tourette syndrome indicate that obsessive-compulsive symptoms are an integral component of the disease. There is reason to believe that autoaggressive actions and some cases of ADHD should also be included in the spectrum of clinical manifestations of Tourette syndrome. Patients with Tourette syndrome often also have personality disorders, affective disorders, anxiety disorders not associated with OCD, sleep disorders, learning disabilities, phoniatric disorders.

Recent studies using standardized assessment methods and specific diagnostic criteria have shown that approximately 40-60% of patients with Tourette syndrome have obsessive-compulsive symptoms. According to epidemiological data, OCD occurs in 2-3% of individuals in the population, so such a high prevalence of these symptoms in patients with Tourette syndrome cannot be explained by a simple random combination of the two diseases. Studies have shown that OCD is more often detected in cases where mothers of patients with Tourette syndrome experienced stress during pregnancy, as well as in male patients with complications during childbirth. Obsessive-compulsive symptoms in Tourette syndrome are an age-dependent phenomenon: symptoms intensify in adolescence and young adulthood, when tics tend to weaken. The most common compulsions in patients with Tourette syndrome include obsessive counting, tidying up or lining up objects in a certain sequence, rubbing with hands, touching, and attempts to achieve absolute symmetry. The fear of contamination and cleansing rituals characteristic of OCD are less common.

As already mentioned, differentiating between some compulsions and tics can be difficult. It is common to classify an action as a compulsion if it is performed to neutralize discomfort caused by a previous thought (obsession). However, it should be taken into account that some patients with tics invent an "obsession" "retroactively" to explain their uncontrollable actions. On the other hand, tic movements may be later included by the patient in the repertoire of compulsions. For example, we observed a 21-year-old patient who had had blinking tics since the age of eight, who stated that he had to blink exactly six times to rid himself of a terrifying image of death. Sometimes a tic can be recognized by context - if a movement is accompanied by other movements, the belonging of which to tics is beyond doubt, then the movement itself is probably of a tic nature. In any case, tic-like compulsions (e.g., blinking, touching, tapping) and some complex motor tics are located at the “intersection” of OCD and Tourette syndrome, which makes it extremely difficult to try to separate them at the clinical level.

Symptoms of ADHD - hyperactivity, inattention, impulsivity - are detected in about 50% of patients with Tourette syndrome and often appear before the onset of tics. A child with moderate or severe Tourette syndrome, as a rule, gives the impression of inattentive, fidgety, impulsive, so it can be difficult to identify symptoms of ADHD in such a patient. It is still unclear whether ADHD is one of the manifestations of Tourette syndrome or just a comorbid disorder. Scientists have identified two types of Tourette syndrome with comorbid ADHD: in one of them, ADHD is independent of Tourette syndrome, and in the other, ADHD is secondary to Tourette syndrome. Some researchers reported that the presence of ADHD predicts a high risk of severe tics and the presence of other comorbid disorders. Children with ADHD and Tourette syndrome often experience more significant difficulties in controlling their own impulses, including aggressive ones. Aggression may be accompanied by unpredictable episodes of affective discharge, which are provoked by frustration or ridicule from peers or relatives. According to one study, attacks of rage are more common in people with a combination of OCD and ADHD.

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Course of Tourette syndrome

The average age of onset of motor tics is 7 years. As the disease progresses, tics often spread in a rostrocaudal direction. The average age of onset of vocal tics is 11 years. The type and severity of tics typically changes in a wave-like pattern, with symptoms tending to become more severe until mid-adolescence. In adolescence, partial remission or stabilization of symptoms is often observed. In most adults with Tourette syndrome, tics continue to interfere with the patient's daily activities, and in one-third of cases, this interference is significant.

Tourette syndrome classification

Motor and vocal tics are divided into simple and complex. Simple motor tics are fast or lightning-fast movements involving a single muscle group. Unlike tremor, tics are not rhythmic. Examples of simple motor tics include blinking, head twitching, and shoulder shrugging. Complex motor tics are slower and more coordinated movements that resemble normal, purposeful movements or gestures, but are untimely or differ in timing and amplitude. Examples include grimacing, touching, twisting objects, copropraxia (indecent gestures), and echopraxia (imitation of other people's movements). Motor tics are most often clonic movements, but can also be dystonic. Clonic tics are sudden, short-term, and usually repetitive movements, such as blinking or tapping. Dystonic tics also begin suddenly, but involve a more persistent change in posture - for example, prolonged opening of the mouth, forced forward bending of the trunk, accompanied by clenching of the jaw. Tics often occur in bursts, including several different movements or sounds, quickly performed or emitted one after another.

Simple vocal tics are quick, inarticulate sounds such as snorting, wheezing, coughing, which can be mistakenly assessed as a manifestation of "allergy". Complex vocal tics involve processes of higher nervous activity: these are linguistically meaningful, but inappropriately timed utterances of interjections, words or phrases. Complex vocal tics include echolalia (repetition of someone else's speech), palilalia (repetition of one's own speech), coprolalia (shouting out obscene words or expressions). Some authors believe that vocal tics should be considered a type of motor tics, which are characterized by contraction of the respiratory tract muscles.

Classification of tics

	Motor	Vocal
Simple	Rapid, lightning-fast, meaningless (eg, blinking, nodding, shrugging, sticking out tongue, tensing stomach, moving toes)	Rapid, inarticulate sounds (e.g. coughing, grunting, snorting, mooing, "uh, uh, uh")
Complex	Slower, seemingly purposeful (eg, gestures, dystonic postures, copropraxia, repetitive touching, hair smoothing, jumping, spinning, finger snapping, spitting)	Linguistically meaningful speech elements (e.g. coprolalia, echolalia, palilalia, "eh. eh", "wow")

Many doctors mistakenly believe that the presence of coprolalia is necessary for establishing the diagnosis of Tourette syndrome, but in fact it is observed only in a small proportion of cases (in 2-27% of patients with Tourette syndrome) and, as a rule, appears only in adolescence. The more severe the disease, the higher the probability of detecting coprolalia. Some researchers consider copropraxia and coprolalia as part of the spectrum of socially unacceptable actions or vocalizations, designated as coprophilia. In a large series of patients with Tourette syndrome, coprolalia was noted in 32% of cases, copropraxia - in 13% of cases, some variant of coprophilia - in 38% of cases. Another study of socially unacceptable actions and expressions found that 22% of patients with Tourette syndrome constantly offend other people, 30% feel the desire to offend others, 40% try to suppress this desire, 24% try to hide their impulses by replacing the aggressive remark with something else that is not offensive to the other person. In an effort to offend others, patients most often say: "You are fat, ugly, stupid..." etc. Aggressive actions and remarks are most often observed in young men with ADHD, behavioral disorder, coprolalia, copropraxia, internal ("mental") coprolalia.

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