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Tics and Tourette
Syndrome: A Clinical
Child and Adolescent Psychiatrist Imam Hossein Hospital
• Tics consist of patterned involuntary (or semi voluntary) movements and vocalizations and can present as either motor or phonic (vocal) tics, or both.
• One measure of tic severity is how much effort a person must exert in order to suppress a tic and how successfully he can inhibit them.
• Tics may be influenced by suggestions.
• It is common for someone with tics to experience more symptoms while describing them.
• Tics can mimic others' movements(echopraxia),words(echolalia) or sounds in the environment.
• It is common for a new tic to begin with a stimulus (a temporary physical irritation or a forceful emotional experience) and to continue long after that stimulus has ended.
• Tics often increase in association with emotionally stimulating events, whether exciting and pleasing or stressful or distressing events. • Tics are exacerbated during times of stress, anxiety, fatigue, excitement, or after the school day.
• Conversely, tics may improve during periods of intense concentration or while performing activities that require fine motor skills or in public, or during competitive athletes ,but are worse just before or afterward.
• During sleep, tics usually diminish in intensity but often do not completely abate.
• Although tics may occur in bursts, their inter-tic intervals are variable and range from seconds, to minutes, to hours or longer.
• Tics may be preceded by premonitory urges, a sense or feeling (tightening, tingling, or tension) that occurs prior to the actual tic. These urges are noted more in adults than in children.
• Tics can be briefly suppressed voluntarily but, during this time, often result in a build-up of "inner tension" that resolves when the tic is performed.
• Motor tics typically develop as sudden, rapid, recurrent, involuntary movements involving the head and facial area, e.g., repeated eye blinking, facial twitching, neck stretching, head jerking, or shoulder shrugging.
• Less commonly, motor tics are more "coordinated," with distinct movements involving several muscle groups, such as repetitive squatting, skipping, or hopping. These complex motor tics may also include repetitive touching of others, deep knee bending, jumping, smelling of objects, hand gesturing, head shaking, leg kicking, or turning in a circle.
• The anatomic locations of motor tics may change over time. They can appear in the face at one point and, later, the shoulder, neck, or extremity. • Rarely, motor tics, usually in combination with obsessive compulsive disorder (OCD), evolve to include behaviors that may result in self-injury, such as excessive scratching and lip biting.
Phonic (vocal) tics
• Phonic tics are sudden, involuntary, recurrent, often loud vocalizations. They usually begin as single simple sounds that may progress to involve more complex phrases and vocalizations. • For example, patients may initially have sounds and noises such as grunting, throat clearing, sighing, barking, hissing, sniffing, tongue clicking, or snorting.
• Complex vocal tics, in contrast, involve repeating certain phrases or words out of context, one's own words or sounds (palilalia), or words and phrases spoken by others (echolalia).
• Rarely, there may be involuntary, explosive utterances of obscene words or phrases (coprolalia).
and Age of Onset of Tics
• Simple or transient tics are very common, affecting between 5% and 18% of children. Tics rarely begin before the age of three years, usually manifesting between 5 to 15 years of age, with the majority of patients affected by age 8.
• Initially tics are typically simple, motor more often than vocal, but can become more complex over time.
• Tics typically reach their peak intensity between the ages of 8 to 14 years.
• Most patients (65%-80%) have a reduction or resolution of symptoms by early adulthood.
• No objective testing is available to confirm the diagnosis of tics; a careful history confirming classical tic characteristics should assist in making the diagnosis. • If patients do not exhibit tics during an office examination, obtaining a video of the movements can provide essential diagnostic information.
• Tics usually do not impair the performance of activities of daily living. This is in contrast with other movement disorders, such as chorea, dystonia, tremor, psychogenic movement disorders, or obsessive compulsive behaviors. • Stereotypies can be differentiated from tics by their onset before age three years, fixed and prolonged nature, and discontinuation with distraction.
• Vocal tics are uncommon in other neurological conditions. • Children with sniffling, throat-clearing, and eye-blinking tics are frequently misdiagnosed with allergies or visual problems. • Compulsions, manifestations of an OCD, may be mistakenly assumed to be tics.
• The primary characteristics associated with Tourette syndrome (TS) are multiple motor tics and one or more phonic tics.
• Motor and phonic tics may develop at about the same time or predominate at different times during the course of the disorder.
• Most TS clinical populations develop associated behavioral problems, particularly obsessive-compulsive behaviors(10-80%) and attention-deficit/hyperactivity disorder (ADHD).
• obsessive-compulsive behaviors include the performance of repetitive actions or rituals, e.g., touching particular objects in a predetermined sequence, repeatedly counting, or engaging in repetitive hand washing.
• In addition, as many as 60% of children with TS have symptoms of ADHD, possibly due to a common underlying neurobiological substrate. • Other issues seen frequently in patients with TS include anxiety (30%), depression(10-75%), episodic outbursts, and school difficulties. • These co morbidities can further impair social interactions, academic and occupational performance, and quality of life.
• In severe cases, patients may exhibit self- injurious behaviors, and, rarely, these behaviors may result in life-threatening situations.
Epidemiology and Age at Onset
• TS usually manifests in children between ages 2 to 15 years of age, with approximately 50% of patients affected by age 7. • TS occurs more frequently in males than in females: a ratio of about 3 or 4 to 1. • The disorder is thought to affect 0.1% to 1.0% of individuals in the general population.
• TS is more frequently in males than in females: a ratio of about 2 or 4 to 1. The disorder occurs worldwide and affects about 0.1% to 1.0% of individuals in the general population.
• Primary TS has a genetic basis, confirmed by its high prevalence in twins and families with one or more affected members.
• The precise gene and mechanism of inheritance remain undetermined. • A complex genetic mechanism is likely, perhaps one associated with multiple genes or an epigenetic effect, i.e., an environmental exposure influencing gene expression.
• The basic underlying neurobiological defect in TS is unknown. Most investigators concur that the disorder results from abnormalities within cortical-striatal-thalamo-cortical pathways. The precise localization, whether cortical or striatal, however, remains controversial.
• Recent neuro imaging studies suggest small volumes in the caudate and possibly compensatory increases in prefrontal cortices, the later contributing to persistence of symptoms.
• Other studies have shown larger volumes of the hippocampus and amygdala, regions that send projections to the ventromedial striatum(motor planning and execution).
• Abnormalities of neurotransmitters, which convey messages between brain neurons, are thought to have a prominent pathological role in TS.
• Abnormalities of dopamine markers in postmortem frontal lobe and PET studies showing excessive neurotransmitter release support a role for dopamine.
• Other investigators, however, emphasize the importance of serotonin.
Treatment of Tics and TS
• The goal of therapy in patients with tic disorders is to reduce motor and vocal tics to a point where they are no longer causing psychosocial or physical problems, as well as alleviating associated behavioral problems, such as OCD and ADHD. • Several non pharmacologic treatments have been shown to be beneficial -- including education interventions; increasing awareness among family members, peers, and school faculty; habit reversal; and exposure therapy.
Psycho educational • The cornerstone of treatment is • Clinicians, patients, parents , and teachers benefit from knowing -what symptoms are present and how they change over time, -how much a child struggles with his -what strategies he uses to reduce them.
• Detailed observations at specific periods• Observation alone can have a potent effect on reducing symptoms by raising awareness and increasing helpful coping responses.
• In some circumstances, observation can "backfire" by reminding the patients about tics, or by expanding parents anxiety, and leading the patient to feel greater pressure to contain and monitor his symptoms.
• The initial focus should be on providing accurate information to patients and parents and assuring they comprehend the problem by: -hearing the patients and families conceptions about the etiology and nature of symptoms -revising them as necessary -teaching about the course and outcome • Education aims to - reduce fears about the future, - decrease blame, - promote cohesion in the family's efforts to resolve problems that arise from the patient's symptoms.
Behavioral interventions • Habit reversal relies on a competing response procedure –an action that when carried out, makes it -impossible to produce the tic,-can be sustained for several minutes,-would not be readily visible to some one who was casually observing the patient Isometric tensing of muscles in opposition to a ticBreathing in a certain way to subvert a vocal tic • Relaxation training Respond to Behavioral
• Patients often report they tic in response to premonitory urges or sensations that are perceived as unpleasant, and are relieved by completion of the tic.
• The goal is not to eliminate tics, but to teach kids how to manage the urge to tic so they don't have to tic as often or intensely.
for tics (CBIT)
• CBIT is based primarily on habit reversal training. The child is taught to be aware of the urge to tic and to use a competing response; for vocal tics, for example, they might focus on diaphragmatic breathing until the urge to vocalize subsides Piacentini J et al( 2010).Behavior therapy for children with Tourette
disorder: a randomized controlled trial. JAMA;303(19):1929-37.
• Pharmacotherapy is not recommended unless the patient's tics are causing significant functional impairment (e.g., academic, occupational, or social performance) or physical discomfort. • Medications may also be necessary to address associated conditions, such as ADHD, OCD, anxiety, and depression.
• First-line therapy for mild to moderate tics includes clonidine and guanfacine -- alpha - • These medications have a fair record of tic suppression and a low incidence of serious adverse events.
• Low dose Clonidine down-regulates NE and leads to decreased serotonine in the median rapheÎdecreased dopamine in the substantia nigra • Clonidine 0.15-0.25 mg/day 3- • Guanfacine 0.5-4 mg/day twice • Haloperidol 0.25-8 mg/day • Pimozide 0.5-8 mg/day • Risperidone 0.25-4 mg/day • Dopamine receptor-blocking agents -- typical and atypical antipsychotic drugs --comprise second-line treatment of tics.
• These medications are generally more effective than first-line medications but should be prescribed only when needed, since serious side effects can be associated with their use. • Injection of botulinum toxin into involved muscles may reduce abnormal movements and the premonitory sensations or urges that precede the tics.
• Some adult patients, at least 25 years of age, who have chronic, severe, debilitating tics that are refractory to pharmacologic and behavioral interventions may be candidates for treatment with deep brain stimulation.
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