Tourettes Syndrome

What is a Tic?

Tics are involuntary, rapid, repetitive, and stereotyped movements of individual muscle groups.

Motor Tics

Simple motor tics: fast, darting, and meaningless.
Examples include: Eye blinking, grimacing, nose twitching, lip pouting, shoulder shrugging, arm jerking, head jerking, abdominal tensing, kicking, finger movements, jaw snapping, tooth clicking, frowning, tensing parts of the body, and rapid jerking of any part of the body. Complex motor tics: slower, may appear purposeful
Examples include: Hopping, clapping, touching objects (or others or self), throwing, arranging, gyrating, bending, “dystonic” postures, biting the mouth, the lip, or the arm, headbanging, arm thrusting, striking out, picking scabs, writhing movements, rolling eyes upwards or side-to-side, making funny expressions, sticking out the tongue, kissing, pinching, and tearing paper or books. Copropraxia: “Giving the finger” and other obscene gestures. Echopraxia: Imitating gestures or movements of other people.

Vocal Tics

Simple vocal tics: meaningless sounds and noises.
Examples include: Coughing, spitting, screeching, barking, grunting, gurgling, clacking, whistling, hissing, sucking sounds, and syllable sounds.

Complex vocal tics: linguistically meaningful utterances such as words and phrases. Examples include: “Oh boy,” “you know,” “shut up,” “you’re fat,” “all right,” and “what’s that.” Rituals:Repeating a phrase until it sounds “just right” and saying something over 3 times. Speech atypicalities: Unusual rhythms, tone, accents, loudness, and very rapid speech. Coprolalia :Obscene, aggressive, or otherwise socially unacceptable words or phrases. Palilalia: Repeating one’s own words or parts of words. Echolalia:Repeating sounds, words, or parts of words of others.

 Types of Tic Disorders

Transient tic disorders often begin during the early school years and can occur in up to 15% of all children. Common tics include eye blinking, nose puckering, grimacing, and squinting. Transient vocalizations are less common and include various throat sounds, humming, or other noises. Transient tics last only weeks or a few months and usually are not associated with specific behavioral or school problems. They are especially noticeable with heightened excitement or fatigue. As with all tic syndromes, boys are three to four times more often afflicted than girls.

Chronic tic disorders are differentiated from those that are transient not only by their duration over many years, but by their relatively unchanging character. While transient tics come and go, chronic tics may persist unchanged for years.

Tourette Syndrome (TS), first described by Gilles de la Tourette, can be the most debilitating tic disorder, and is characterized by multiform, frequently changing motor and phonic tics.

 

Diagnostic Criteria for Tourette’s Disorder

  • Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently.
  • The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than 1 year, and during this period there was never a tic-free period of more than 3 consecutive months.
  • The disturbance causes marked distress or significant impairment in social, occupational, or other important areas of functioning.
  • The onset is before age 18 years.
  • The disturbance is not due to the direct physiological effects of a substance (e. g., stimulants) or a general medical condition (e.g., Huntington’s disease or postviral encephalitis).

 

Prevalence

The incidence of TS in adults is about 0.1–1%. Estimates of TS in children have been as high as 3%. The lifetime prevalence of TD is not known but estimates vary between 5% and 10% of the population, with estimates of 18% in child populations. Tics develop in childhood and simple tics usually precede more complex tics, with phonic tics usually developing which will often begin as breathing or sniffing noises subsequent to motor and sensory tics. The most notorious TS vocal tic, coprolalia (swearing), is rare.

Cognitive, Behavioural and Psychological Problems Associated with Tourette’s Syndrome

As well as tics, there are a variety of behavioral and psychological difficulties that are experienced by many, though not all, patients with TS. The most frequently reported behavioral problems are attentional deficits, obsessions, compulsions, impulsivity, irritability, aggressivity, immaturity, self-injurious behaviors, and depression. Some of the behaviors (e.g., obsessive compulsive behavior) may be an integral part of TS, while others may be more common in TS patients because of certain biological vulnerabilities (e.g., ADHD). Still others may represent responses to the social stresses associated with a multiple tic disorder or a combination of biological and psychological reactions.

 Causes of Tourette’s Syndrome 

Research shows that, in Tourette syndrome, something is wrong with the way in which the brain produces or uses important substances called neurotransmitters, which control how signals are sent along the nerve cells. The neurotransmitters dopamine and serotonin have been implicated in Tourette syndrome; noradrenaline is thought to be the most important stimulant. (Medications that mimic noradrenaline may cause tics in susceptible patients.) Whatever the exact defect, it is handed down through the genes from parents to children. If one parent has Tourette syndrome, each child has a 50% chance of getting the abnormal gene.

Treatment of Tourette’s Syndrome

Treatment of Tourette syndrome can be divided into treatment of tics, and treatment of co-occurring conditions, which, when present, are often a larger source of functional impairment than the tics themselves. There is no cure for Tourette’s and no medication which works universally for all individuals without significant adverse effects; knowledge and understanding are the best treatments available for tics. Management of the symptoms of Tourette’s may include pharmacological, behavioral and psychological therapies. While pharmacological treatment is reserved for more severe symptoms, other types of treatments may help avoid or improve symptoms of depression or social isolation, and improve supportive family functioning.

The majority of people with TS require no medication, but medication is available to help when symptoms interfere with functioning. Because children with tics often present to physicians when their tics are at their highest severity, and because of the waxing and waning nature of tics, medication is not usually started immediately or changed often. Frequently, the tics subside with understanding of the condition and a supportive environment. When medication is necessary, pharmaceutical intervention should be targeted at the most impairing symptoms, taking into account co-occurring conditions such as ADHD or OCD, which when present, may warrant treatment even when tics are mild.

Cognitive Behavioral Therapy (CBT) is a useful treatment when Obsessive Compulsive Diisorder (OCD) is present, and there is good evidence supporting the use of habit reversal in the treatment of tics.  Habit reversal training is a set of procedures that have been used very successfully with hair pulling (trichotillomania), nail-biting, thumb-sucking, chronic motor tics, and the tics associated with Tourette’s disorder. Habit reversal is a therapy composed primarily of awareness training and competing response training. If anxiety is an issue, CBT addressing these issues should be used alongside habit reversal, as anxiety often makes the tics not only worse but harder to control. Relaxation techniques, such as exercise, yoga or meditation, may also be useful in relieving stress that may aggravate tics, but the majority of behavioral interventions (such as relaxation training and biofeedback, with the exception of habit reversal) have not been systematically evaluated and are not proven therapies for Tourette’s.