Tourette Syndrome

Overview

Tics are very common – about 1 in every 4 children will have a short-lived tic or set of tics during their childhood. Tics are best thought of as repeated (“stereotyped”) patterns and non-rhythmic, non-soothing movements or noises that resemble intentional ones but serve no functional purpose. However, for some children, tics endure for long periods of time.

Tourette syndrome (TS) is a type of tic disorder characterized by waxing and waning motor and phonic tics lasting over a year (although not necessarily at the same time). Chronic tic disorders – chronic motor tic disorder, chronic vocal tic disorder, and TS – are differentiated by the types of tics (motor, phonic, or both). Because the distinctions among TS and the other two are generally accepted as artificial, the term TS will be used to refer to all of them in the following discussion. Chronicity (lasting a year or more) distinguishes TS from provisional tic disorder, the duration of which is less than 1 year (determined retrospectively or currently).

In TS, motor and phonic tics range in their expression from simple to complex. Simple motor tics are brief and involve an isolated muscle group, such as an eye blink, while complex motor tics appear more purposeful, such as the holding of a posture or moving multiple muscle groups in sequence to jump or twirl. Complex motor tics may include copropraxia, involving vulgar gestures or other socially inappropriate actions, such as suddenly reaching for or touching another person inappropriately.

Simple phonic tics involve sudden meaningless noises, such as sniffing or grunting, while complex phonic tics sound language-based, such as echolalia (repeating vocalizations made by another person) or coprolalia (uttering socially inappropriate language). Despite frequent media portrayal, copropraxia and coprolalia are uncommon among people with TS.

By adolescence, most people with TS can describe an uncomfortable physical sensation or urge that precedes a tic. The performance of the tic or tics temporarily reduces this urge. Although most individuals with TS are able to suppress their tics temporarily, the urges usually persist and build, becoming so intolerable that the tics are ultimately expressed. Stress, anxiety, excitement, and fatigue may increase tic frequency and intensity.

Tic disorders reflect a complex underlying ‘leaky filtering’ of sensory inputs or ‘disinhibition,’ characterized by inconsistent ability to inhibit certain thoughts, emotions, or behaviors. Most people diagnosed with TS will also have one or more non-tic associated conditions, including attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), anxiety, learning problems, sleep disorders, depression, or sudden excessive outbursts of anger. Signs and symptoms of these comorbid conditions can precede, co-occur with, or follow the onset of tics. These conditions usually pose a greater risk to the neuro-developmental, psychosocial, and behavioral well-being of the child than the tics alone pose. Adverse consequences of TS on self-esteem, academic performance, and social flexibility are common and can be profound.

Many lines of evidence link TS with pathways between the prefrontal cortex, the thalamus, and the basal ganglia and changes in the distribution and activity of the neurotransmitter dopamine. For example, medications that decrease tics (including the antipsychotics haloperidol and pimozide) are known to alter traffic through these pathways, and data from functional imaging of individuals with TS support these neurophysiological mechanisms. [Butler: 2006] Also, deep brain stimulation to specific suspected brain regions appears helpful in some people with refractory TS. [Bajwa: 2007]

Other Names & Coding

Chronic motor tic disorder
Chronic vocal tic disorder
Gilles de la Tourettes syndrome
Tourette disorder
Tourette's disorder
TS
ICD-10 coding

F95.0, Transient tic disorder

F95.1, Chronic motor or vocal tic disorder

F95.2, Tourette's disorder

F95.8, Other tic disorder

F95.9, Tic disorder, unspecified

For differentiation among the codes, see ICD-10 for Tic Disorders.

Prevalence

The prevalence of TS in children is approximately 0.5 percent (1:200). [Scharf: 2015] Males are 3 to 4 times more likely than females to have TS. [Knight: 2012] Related chronic tic disorders (either motor or vocal, but not combined) are 3 to 4 times more common than TS. Short-lived tics lasting less than 1 year (provisional tic disorders) occur in up to 1:4 children, usually during the first decade of life.

Genetics

The causes of TS are poorly understood; genetic and environmental factors influence variations in the expression of tics and associated conditions.

Prognosis

Many people with TS adapt well to tics and neither seek nor require treatment. Tics are likely to decrease or dramatically improve in late adolescence. [Pappert: 2003] Associated conditions, such as ADHD and OCD, may be lifelong; symptoms vary with developmental stages.

Practice Guidelines

Pringsheim T, Okun MS, Müller-Vahl K, Martino D, Jankovic J, Cavanna AE, Woods DW, Robinson M, Jarvie E, Roessner V, Oskoui M, Holler-Managan Y, Piacentini J.
Practice guideline recommendations summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders.
Neurology. 2019;92(19):896-906. PubMed abstract / Full Text

Roles of the Medical Home

Most children with TS can be followed within their medical home with periodic surveillance and targeted screening for problems related to tics and associated conditions. Depending on the provider’s comfort with the diagnosis, the severity of TS and associated conditions, and if a medication trial is being considered, evaluation by a TS specialist may be appropriate. When indicated, the medical home coordinates care and ensures communication among a team that could include a psychologist and other school-based professionals, and depending on local availability, a neurologist, psychiatrist, or developmental-behavioral pediatrician. The Portal's Education & Schools section for clinicians can help guide communications with teachers and school personnel to ensure that patients receive appropriate education-related services.

Keeping a Care Notebook can help families maintain continuity of care. Ongoing education and support for the child, family, peers, and educators are critical. The Tourette Association of America has a Youth Ambassador Program (Tourette Association) and provides explanations of protections under the Individuals with Disability Education Act (IDEA) and "Section 504" of the Americans with Disabilities Act. Parents may also find helpful information about school services and laws in the Portal's Education and Schools family's section. Effective child advocacy and self-advocacy depend on positive regard and solid self-esteem.

Clinical Assessment

Overview

Assessment of an individual with a suspected tic disorder should include a thorough medical history and physical exam. These will ideally include assessment of tics and their impact on academic, social, organizational, family, and health. Careful questioning for frequently co-existing conditions is critical. The relative impact of co-existing factors on self-esteem and quality of life should be defined.

Pearls & Alerts for Assessment

Tic occurrence

Tics wax and wane over time and emotional states can influence tics.

Severity

‘Tic severity’ is not a single thing but is determined by several features (number, intensity, frequency, complexity, functional interference, and distress).

Co-existing conditions

Expect 1 or more co-existing non-tic conditions (e.g., ADHD, OCD, academic difficulties, anger outbursts, anxiety). Their presence is the rule rather than the exception.

Screening

For Complications

Surveillance/screening should be conducted for associated behavioral problems, such as: Co-existing conditions can emerge at any time, so surveillance and, if indicated, further screening or evaluation is appropriate.

Presentations

The tics in TS typically emerge when a child is 4 -7 years of age. The tics may evolve from simple to complex and from just motor to motor and/or phonic. Associated behavioral or neurodevelopmental conditions may precede, occur with, or begin after the tics have emerged.

Diagnostic Criteria

No test confirms the diagnosis of TS. Note that tic disorders less than 1 year in duration are provisional, as the diagnosis cannot be made until the tics have been present for 1 year. The following criteria from the DSM-5 [American: 2013] must be met to make a diagnosis of TS (referred to as Tourette's Disorder in DSM-5):
  • Both multiple motor and 1 or more vocal tics have been present at some time, although not necessarily concurrently (a tic is a sudden rapid, recurrent, nonrhythmic motor movement or vocalization)
  • Tic frequency may wax and wane but have persisted for more than 1 year since first tic onset
  • Onset is before age 18 years
  • Disturbance is not attributable to the physiological effects of a substance (e.g., cocaine) or another medical condition (e.g., Huntington's disease, post-viral encephalitis).

Differential Diagnosis

Other tic disorders: See introductory discussion regarding lack of relevant clinical distinction among the chronic tic disorders in childhood.

The following are relatively common co-occurring conditions:
  • Stereotypies are repetitive, rhythmic, soothing movements, postures, or utterances. Although they are often seen in children with autism spectrum disorder, they are also very common in typically developing children. Stereotypies usually emerge prior to 3 years of age. Examples include hand-flapping, rocking, and twirling. Among children with communication limitations, stereotypies and complex tics may be indistinguishable.
  • Habits share features with stereotypies but typically are not rhythmic or soothing and may be quick behaviors that can be reinforced through repetition. Examples include nail-biting and lip-licking.
  • Compulsions are very similar to tics and sometimes difficult to distinguish from complex tics. Compulsions usually are responses to obsessive thoughts (i.e., irrational fears of an adverse outcome), whereas tics usually are responses to sensory experiences.
The following considerations are far less common:
  • Seizures: Generalized seizures are accompanied by a loss of consciousness, while complex partial seizures are associated with alterations in consciousness and responsiveness. Some partial seizures could be mistaken for tics, but these are generally not suppressible and are not preceded by a premonitory urge. Eyelid flickering (myoclonus) seen with absence seizures could resemble tics, but in absence seizures, there is loss of consciousness, no ability to suppress the seizure, and no premonitory urge. For clarification, truly involuntary myoclonic jerks are not stereotyped and can be seen in association with juvenile myoclonic epilepsy.
  • Substance exposure: Neuropsychiatric medications are unlikely to cause tics, but they can be associated with other movement problems, such as restlessness. Stimulant medications do not cause tics; in a fraction of patients, the medication exacerbates them. Tics have been reported with carbamazepine and rarely other medications. Prolonged use of some neuroleptics may result in tardive dyskinesia, a neurologic syndrome with a variety of repetitive purposeless movements that may appear tic-like. Movements may occur in the extremities, as well as the face and could include lip-smacking, grimacing, and rapid eye-blinking. Medication adjustments are usually necessary when tardive dyskinesia is noted.
  • Muscle spasms, such as blepharospasms or spasmodic torticollis, may be confused with TS, but the characteristics of tics in TS should allow differentiation.
  • Sydenham chorea is a neurological disorder of childhood resulting from an autoimmune reaction to infection group A beta-hemolytic Streptococcus (GABHS), which also causes rheumatic fever. This condition is characterized by involuntary movements of the face, arms, and legs, clumsiness, emotional volatility, difficulty concentrating, and no premonitory urge. Sydenham chorea is typically seen in children 5-15 years old. Of note, tics may also be present. It affects girls more than boys and may appear from several weeks to many months after strep infection. There is no treatment; the chorea usually resolves after weeks to months. Symptomatic management of chorea is rarely necessary.
  • Post-viral encephalitis: This brain-based inflammation is differentiated from tics by medical history and examination. As is the case for Syndenham chorea, tics may also be present.
  • Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a controversial condition postulated as either 1) a dramatic increase in symptoms in children already known to have tics or OCD or 2) an appearance of such symptoms in children without prior history, following a GABHS infection, other infection, or non-infectious suspected causes. [Blackburn: 2018] PANS – or pediatric acute-onset neuropsychiatric syndrome – has been coined as a broader term to include other infectious and non-infectious suspected causes. [Wilbur: 2019] No diagnostic criteria are uniformly accepted as definitive for this hotly debated condition. There is quite a bit of intrigue and controversy, and research is ongoing. [Chang: 2015]
  • Stroke, although very uncommon in children, can precede and presumably cause onset of tics.
  • Wilson disease is an autosomal recessive genetic disorder that prevents the body from disposing of extra copper. As copper levels build in the brain, tremor and tic-like movements, including dystonia, chorea, or ataxia may emerge. Incoordination of speech, swallowing, and gait, and behavioral changes can also occur. If the liver is also involved, jaundice, ascites, and other systemic symptoms may be present. Physical exam findings also include Kayser-Fleischer rings due to copper accumulation in the eye. Laboratory findings show decreased copper and ceruloplasmin levels in the blood, increased urinary excretion of copper, and copper accumulation in the liver. Since Wilson disease is treatable, there should be a low threshold for medical screening for this disorder.

Comorbid & Secondary Conditions

More than 90% of individuals with TS have 1 or more associated behavioral or neurodevelopmental conditions. Onset of the signs and symptoms of these co-existing conditions can precede, co-occur with, or follow the onset of the tics. These conditions may include:
ADHD (~50%) Fine motor coordination difficulty
Obsessions and Compulsions (>50%) Specific learning disability (~ 20%)
Anxiety (~30%) Autism spectrum disorder
Depression Sleep problems
Anger control difficulty Executive function difficulties

History & Examination

A child with new-onset tics should undergo a comprehensive evaluation that includes history and physical examination. Most people with tic behaviors find them tolerable but often have non-tic problems that cause more difficulties. The severity of tics and their impact on the individual’s life may be affected by a variety of environmental and social factors. Each visit offers an opportunity to explore these factors and what is working well and not-so-well for the patient.

Current & Past Medical History

Phonic tics such as sniffing or coughing may be confused with allergies. Likewise, eye-blinking is often attributed to allergies and eye irritation. For patients with known TS, tic severity (which may be determined by frequency, complexity, intensity, number, interference with social experience and functions in life, and discomfort or pain) should be monitored in ongoing visits. For patients taking medications to reduce tics, track effectiveness, side effects, and the need for ongoing treatment.

Family History

A family history of TS, OCD, ADHD, or learning problems is common; however, 10% of children with TS have no identifiable contributory family history.

Pregnancy/Perinatal History

Maternal cigarette smoking during pregnancy may increase tic severity in children predisposed to developing TS.

Developmental & Educational Progress

As with all children, social, academic, mood-based, and other functional symptoms should be a part of periodic routine screening. If concerns arise, a comprehensive psycho-educational assessment is usually indicated. Open-ended questions about academic, social, organizational skills, sleep, and family functioning may uncover new concerns that warrant investigation.

Social & Family Functioning

TS is commonly associated with ADHD, learning problems, and other developmental challenges. TS may first emerge or be identified after 1 of these other entities is discovered. Open-ended questions about academic, social, organizational skills, and family functioning may uncover concerns that warrant investigation.

Physical Exam

TS is rarely associated with specific physical examination findings; however, in certain cases, repetitive behaviors can result in findings specific to the tic as detailed below.

General

Watch for subtle signs of tics during the history-taking and physical examination. Remember that tics are suppressible and often embarrassing, and individuals will hide their tics in a variety of ways, such as with hair smoothing or stretching. Additionally, tics wax and wane naturally and have a variable frequency so will not necessarily be observed in the clinical setting

Vital Signs

Check HR | BP if medication is being considered or taken, or for other specific medical indications. Hypotension may occur in children on antipsychotic medications.

Growth Parameters

Note baseline and subsequent weights since medications used for TS or co-occurring conditions may be associated with weight change.

Skin

Chapping around lips, scabbing associated with repetitive behaviors; “moth-eaten” hair loss due to associated trichotillomania

HEENT/Oral

Dental trauma (enamel wear, flattening of occlusal surfaces) associated with tooth-chomping

Extremities/Musculoskeletal

Rarely, evidence of cervical spine injury secondary to severe neck-snapping tics

Neurologic Exam

Soft signs, such as impaired fine motor skills, may reflect neuro-maturational delay.

Testing

Sensory Testing

Even subtle problems with vision and hearing may compound learning and behavioral difficulties in children with TS, so screening for these sensory impairments should be performed with the initial diagnosis.

Laboratory Testing

Children taking medications should be tested as clinically indicated.

Imaging

Neither neuroimaging nor EEG is indicated for the diagnosis of TS.

Genetic Testing

Though TS is genetically influenced, no genetic tests are currently available to help with making the diagnosis.

Other Testing

If targeted history and physical exam are suggestive of possible cardiac disease and medications are being considered, cardiovascular workup may be indicated. Children with a family history of sudden death in children or young adults, hypertrophic cardiomyopathy, or long QT syndrome should have a screening EKG [Perrin: 2008]

Specialty Collaborations & Other Services

When history and physical examination suggest co-existing conditions outside the scope or competence of the primary care clinician, referral to and coordination with appropriate specialists is indicated. For example, a psychologist or education specialist may evaluate the child's behavior or learning problems, or an occupational therapist might best evaluate fine motor coordination problems. Pediatric specialists with the most expertise in TS and associated conditions may vary by community. Selection among the following specialists may depend on referring provider custom, availability, and/or specialist’s specific expertise.

Tourette Syndrome Programs (see UT providers [1])

Tourette-focused clinical programs, including the 15 Tourette Syndrome Centers of Excellence (Tourette Association) designated by the Tourette Association of America, will offer multi-disciplinary approaches and are likely to have the most ongoing clinical experience in assessing difficult or complex TS.

Mental Health/Counseling > … (see UT providers [1078])

Referral and/or co-management for ADHD, OCD, learning problems, anxiety, sleep problems, depression, anger dysregulation, or autism spectrum disorder may improve functional outcomes.

Educational Testing/Assessment (see UT providers [12])

If problems in school exist, a psycho-educational evaluation that includes IQ and other cognitive components, academic achievement skills, learning strengths and weaknesses, attention problems, and social function may help educational planning. Public schools must complete indicated evaluations at no cost to the family; private assessment is also available. Testing is time-consuming and expensive, so it should be performed with specific goals in mind. Insurance is more likely to cover private testing if results lead to changes in treatment. See Preauthorization Letter for Psychological Testing (PDF Document 58 KB).

Developmental - Behavioral Pediatrics (see UT providers [8])

These clinicians bring a "whole-child" perspective, with emphasis on neurodevelopmental and psychosocial influences on behavior, learning, and growth. They may have clinical experience with TS and co-existing conditions, including ADHD and learning disabilities.

Psychiatry/Medication Management (see UT providers [62])

Their approach to diagnosis and management of TS may have particular emphasis on the inclusion of psychotropic medication management. Consider referral when such treatment is anticipated or if psychiatry offers the best local expertise with TS.

Neuropsychiatry/Neuropsychology (see UT providers [11])

May assist with neuro-psychological evaluation when TS is complicated by associated disorders and learning problems, and where brain-based impairment in cognitive function/behavior is suspected (e.g., in a child with TS who also has seizures.

Pediatric Neurology (see UT providers [5])

May have considerable clinical experience with TS; particularly helpful if differentiating TS from other movement disorders or seizures

Treatment & Management

Pearls & Alerts for Treatment & Management

Co-existing conditions

The vast majority of patients with Tourette syndrome and other chronic disorders also have one or more non-tic co-existing conditions, about which concerns usually outweigh those related to problems and interferences associated with tics.

Need for ongoing surveillance/screening

Surveillance and periodic screening for tics and associated co-existing conditions (for example, learning disabilities, anxiety disorders, substance abuse disorders) is an ongoing process, as symptoms emerge over time.

Understand patient/family priorities

Clinicians and families often have different opinions about the importance of tic or related symptoms. Clear and effective communication will help to prioritize problems and guide management.

Systems

Neurology

Treatment of tics should be guided by the patient’s priorities, tolerance of the tics, family expectations, and the impact on their lives. Co-occurring conditions often pose greater challenges to function or quality of life than the tics and may be a higher priority for treatment. Also, effective treatment of co-conditions such as anxiety disorders, sleep disturbances, and ADHD can result in improvement in tic frequency/severity.

When tics are mild and not disabling, education about the condition and supportive counseling may suffice. Since tics wax and wane in TS, watchful waiting can be the best approach for some. When tics are disabling due to their impact on social, educational, or other domains, treatment options include comprehensive behavioral intervention for tics and/or pharmacotherapy. Medication remains the treatment of choice for tics of greater severity.

Comprehensive behavioral intervention for tics (CBIT) – not to be confused with cognitive behavioral therapy (CBT) – has been shown to be as effective as pharmacotherapy in reducing tic behaviors in both children and adults. [Pringsheim: 2019] The best candidates are those who recognize their premonitory sensations and are motivated to reduce their tics. CBIT combines Habit Reversal Training and Functional Analysis and can be used alone or with medication. Other behavioral approaches have either not been adequately assessed or have not shown efficacy. Families may be interested in learning more at CBIT: Information for Patients (Tourette Association).

Deep brain stimulation (DBS) is a neurosurgical approach typically reserved for adult patients with severe tics that pose substantial impairment to quality of life or threaten personal safety and who have not responded adequately to comprehensive medication, psychological, and behavioral interventions. In rare cases, DBS may be considered for select pediatric and adolescent patients after thorough investigation with a local ethics committee and/or institutional review board. [Deeb: 2019] [Schrock: 2015]

Transcranial magnetic stimulation (TMS) has also been explored as a treatment option. [Grados: 2018] A decision tree for management of TS can be found at Management Decision Tree (European Society for the Study of TS).

Specialty Collaborations & Other Services

Specialists within child neurology, child psychiatry, or developmental pediatrics likely have experience managing TS; the degree for each will vary by individual. For the child with severe TS and/or associated problems, co-management with a TS expert may be helpful.

Tourette Syndrome Programs (see UT providers [1])

Tourette-focused clinical programs, including the 15 Tourette Syndrome Centers of Excellence (Tourette Association) designated by the Tourette Association of America, will offer multi-disciplinary approaches and are likely to have the most ongoing clinical experience in managing TS.

Developmental - Behavioral Pediatrics (see UT providers [8])

Focused on the "whole-child" evaluation, with emphases on neurodevelopmental and psychosocial influences on behavior, learning, and growth. Likely to have clinical experience with TS and with common co-occurring conditions, including ADHD and learning disabilities.

Psychiatry/Medication Management (see UT providers [62])

For the child with additional behavioral, learning, or mental health problems likely to benefit from psychotropic medications - may have clinical experience with TS.

Pediatric Neurology (see UT providers [5])

For the child with additional neurologic conditions - may have clinical experience with TS.

Pharmacy & Medications

As medication treatment is contemplated, it is essential to consider the impact of co-existing conditions, present in the majority of children with TS. Management should focus on areas with the greatest impact on social, academic, or other areas of function, and co-existing conditions usually warrant first consideration. With appropriate information, most children and families will choose not to use medication to treat tics. Since tics tend to wax and wane, baseline severity should be assessed for several months before starting medication. The gold-standard approach uses the Yale Global Tic Severity Scale [Leckman: 1989], but it requires a skilled administrator. A variety of other tic-rating scales, such as the Tic Severity Checklist (PDF Document 16 KB), are easier to use in the medical home setting. Each has pros and cons that are described in [Martino: 2017].

Most medications used for tic reduction are prescribed "off-label." The only ones approved by the US Food and Drug Administration for treatment of tics in TS are the typical neuroleptics haloperidol and pimozide and aripiprazole, an atypical neuroleptic. [Silay: 2005] [Sallee: 1997] [Sallee: 2017] Those, however, are generally not used as first-line therapy because of potential adverse side effects, including tardive dyskinesia – a rare but permanent movement disorder that particularly involves the mouth.

Treatment with medications requires a trial and error approach with frequent clinical visits to assess response and titrate dosage. A provider with expertise in tic management is preferable. Medication trials should be conducted over weeks to months to account for waxing and waning tics. In general, start medications at small doses and increase gradually. Any benefits gained with medications should be weighed against current and potential side effects. When medications for tics are to be discontinued, most should be weaned over time, and only under provider guidance.

Alpha-2 adrenergic agonists (clonidine and guanfacine) are often the first choice because their side effects are more tolerable than those of other drug classes and do not include tardive dyskinesia. However, these agents may take weeks or longer to decrease tics, and the reduction is less certain and often less robust than with other agents. Sedation is the most likely side effect of these medications, especially the short-acting forms.

The short-acting forms, which may need to be given 3-4 times a day, can be used initially to assess tolerability and then switched to a long-acting preparation. Oral long-acting forms are available for clonidine and guanfacine; clonidine is available as a transdermal patch. The latter needs to be changed only once a week and produces less sleepiness than the oral form, but local allergic reactions are common. Guanfacine has a longer half-life and is reportedly less sedating than clonidine.

Beneficial side effects of these agents may include the reduction of hyperactive and impulsive behaviors associated with ADHD and reduced latency to sleep onset in patients with difficulty getting to sleep.


Neuroleptics are usually reserved for more severe tics. Although they work well, no medication is successful in all people at doses that don't cause some side effects. Medications should be started at small doses that are increased gradually as needed while watching for side effects. The most common side effects are sedation and weight gain, but neurologic side effects, such as Parkinson-like symptoms and tremor, are also possible. Although dystonic reactions are rare, families should be warned about them. Atypical neuroleptics pose a risk for development of insulin resistance and diabetes – periodic laboratory monitoring is required.

The typical neuroleptics haloperidol and pimozide and the atypical aripiprazole are approved for use by the FDA. [Silay: 2005] [Sallee: 1997] Among atypicals, risperidone and olanzapine have been shown to be effective. [Sandor: 2003] [Budman: 2001] [Bruun: 1996] After long-term use, neuroleptics need to be discontinued slowly to avoid withdrawal problems that may include a rebound in tic frequency and tardive dyskinesia.

Botulinum toxin (Botox) is most helpful when the patient has a severe, localized tic, such as in the face or neck. It is injected by an experienced practitioner into the affected muscle groups. The therapeutic effect lasts for several months.

Anti-epileptic drugs (topiramate in particular) may be useful in reducing tic severity; however, potential side effects are important to consider, particularly at higher doses. [Yang: 2013]

Benzodiazepines, antispasmodic agents (baclofen), dopamine agonists (pergolide), and tetrabenazine are also considered options for treatment of tics. The research on each of these agents is variable; until more thorough research is undertaken, available evidence for the use of these agents is limited.

Genetics

Specialty Collaborations & Other Services

Tourette Syndrome Programs (see UT providers [1])

Tourette-focused clinical programs, including the 15 Tourette Syndrome Centers of Excellence (Tourette Association) designated by the Tourette Association of America, will offer multi-disciplinary approaches and are likely to have the most ongoing clinical experience in managing TS.

Pediatric Neurology (see UT providers [5])

For a child with additional neurologic conditions; may have clinical experience with TS.

Mental Health/Behavior

Diagnosis and management of TS-associated conditions (ADHD, OCD, learning problems, anxiety, sleep problems, depression, anger dysregulation, and autism spectrum disorder) can greatly improve social and educational functioning and may reduce tic severity. The medical home provider should monitor for signs of these conditions and provide treatment with co-management and referral to counseling as necessary.

Attention-Deficit/Hyperactivity Disorder (ADHD) is the most commonly occurring comorbid condition. The presence of tics should not affect a choice among standard treatment modalities – stimulant medications are unlikely to influence tic behavior. Families should know that the course of tics is unpredictable and a waxing of tics while on stimulant medication could falsely suggest they are the cause. [Tourette's: 2002] [Erenberg: 2005] Although the alpha-2 adrenergic agonists clonidine and, less so, guanfacine may be helpful for the treatment of ADHD symptoms in those with TS, these medications are usually not as effective as stimulants and take weeks to months to show beneficial effects. The alpha-2 agonists may, however, help decrease the frequency and intensity of tics in some patients, reduce hyperactive and impulsive behaviors in children with co-occurring ADHD, and, when taken in the evening, assist with reducing sleep onset latency.

Children with obsessive-compulsive disorder (OCD) and tics may not respond as well as those without tics to selective serotonin reuptake inhibitors, but both groups respond similarly to cognitive behavioral therapy (CBT) for OCD symptoms. CBT is the recommended first-line treatment of OCD in individuals with tic disorders. [Pringsheim: 2019]

Specialty Collaborations & Other Services

Psychiatry/Medication Management (see UT providers [62])

May be helpful in managing TS with psychotropic medications. Consider referral if response to first-line therapy is inadequate or if psychiatry offers the best local expertise with TS.

General Counseling Services (see UT providers [389])

Helps with management of associated disorders and the provisions of counseling and interventions, including cognitive behavioral therapy (CBT).

Learning/Education/Schools

The reactions of peers and teachers can adversely impact the school experience and educational outcomes of children with TS. Education of peers about TS can result in more positive attitudes toward affected children, and education of teachers can improve knowledge about the condition and their ability to support the child with TS. Improving peers' attitudes about and teachers' knowledge of TS may positively affect children with TS. [Pringsheim: 2019]

Complementary & Alternative Medicine

Dietary modifications and allergen control are popular complementary/alternative healthcare approaches for treating TS. High-quality research is limited and none has shown benefit in reducing tics. Because parents or youth with TS may try unproven approaches (such as restrictive diets), these and alternative approaches should be discussed. Guidance should balance respect for a family's interests and values with evidence regarding safety and efficacy. Complementary/alternative healthcare options that have been explored include acupuncture [Yu: 2016], herbal formulations (in particular, the 5-Ling granule and Ningdong granule formulations) [Kim: 2014], and dental orthotics. [Mathews: 2016] No formal recommendations have been published for the use of these agents and further research is indicated.
No Related Issues were found for this diagnosis.

Ask the Specialist

Why do children with TS sometimes shout obscenities and ethnic slurs?

Coprolalia occurs in fewer than 15% of people with TS. These symptoms appear to be due to an uncontrollable urge to "voice the forbidden," even when the person with TS does not feel anger.

What do tics feel like?

There is often a premonitory sensation that individuals feel prior to a tic behavior. Though this sensation differs across individuals (and even across different tic types), it is often described as a pressure-like sensation or an ‘itch’ in the muscle group where the tic occurs that may build over time. A specific example everyone is familiar with is the sensation that builds up in the eyelids when holding back a blink, and the subsequent relief after blinking is similar to the relief that occurs after performing a tic.

What is the difference between a tic and a motor stereotypy?

Both tics and motor stereotypies are technically “stereotyped,” which is to say that the behavior is performed in a similar way again and again. Motor stereotypies are sustained and repetitive movements that are rhythmic, generally emerge in the toddler years, do not have a premonitory urge, are pleasurable, and typically do not change over time. Tics, in contrast, occur in bouts but are not sustained, are not rhythmic, generally emerge later in childhood, start with a premonitory sensation that drives a tic, are unpleasant (but are followed by relief), and tend to change in type over time.

Resources for Clinicians

On the Web

Tourette Disorder (OMIM)
Extensive review of literature that provides technical information on genetic disorders; Online Mendelian Inheritance in Man site, hosted by Johns Hopkins University.

Gilles de la Tourette Syndrome (NIH)
Database search that gives links to abstracts, full-text journals, books, and reports about TS; National Institutes of Health.

Helpful Articles

PubMed search for Tourette syndrome in children, last 2 years.

Deng H, Gao K, Jankovic J.
The genetics of Tourette syndrome.
Nat Rev Neurol. 2012;8(4):203-13. PubMed abstract

Pringsheim T, Holler-Managan Y, Okun MS, Jankovic J, Piacentini J, Cavanna AE, Martino D, Müller-Vahl K, Woods DW, Robinson M, Jarvie E, Roessner V, Oskoui M.
Comprehensive systematic review summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders.
Neurology. 2019;92(19):907-915. PubMed abstract / Full Text

Zinner SH, Mink JW.
Movement disorders I: tics and stereotypies.
Pediatr Rev. 2010;31(6):223-33. PubMed abstract

Clinical Tools

Assessment Tools/Scales

Tic Severity Checklist (PDF Document 16 KB)
An easy-to-use tic severity scale for keeping an accurate record of the type and frequency of tics. Useful for assessing the need for and response to medications. This checklist can be downloaded for family use.

Letters of Medical Necessity

Preauthorization Letter for Psychological Testing (PDF Document 58 KB)
A sample letter requesting preauthorization for neuropsychological testing to help identify strengths, weaknesses, and possibly mood disorders in a child with Tourette syndrome.

Toolkits

Managing Tourette & Tic Disorders (PDF Document 5.8 MB)
Comprehensive, 16-page guide to treatment for providers; from the Tourette Association of America.

Other

VISIT-TS (Video-Integrated Screening Instrument for Tics and Tourette Syndrome)
Multimedia tool intended to demonstrate tics to a lay audience.

Patient Education & Instructions

Living with Tourette & Tic Disorders (Tourette Association) (PDF Document 7.5 MB)
An 18-page, free booklet that can serve as a starting point for patients and families affected by a new diagnosis of TS; from the Tourette Association of America.

Newly Diagnosed with Tourette Seminar
Presented by Dr. John Walkup, MD, Division of Adolescent and Child Psychiatry at Cornell Weill Medical College (4 part series).

‘I Have Tourette’s, But Tourette’s Doesn’t Have Me’
Documentary produced in collaboration with the Tourette Syndrome Association

Managing Tourette Syndrome: Behavioral Interventions, Parents Workbook
A 96-page, behavioral intervention workbook for children and adults with tic disorders; written by Douglas W. Woods: link to the publisher's web site, available from local and online bookstores.

Resources for Patients & Families

Information on the Web

Tourette Syndrome (Genetics Home Reference)
Excellent, detailed review of condition for patients and families; sponsored by the U.S. National Library of Medicine.

CBIT: Information for Patients (Tourette Association)
Information about the effectiveness and application of CBIT; from the Tourette Association of America.

Tourette Syndrome - Getting Help at School (Tourette Association)
Advice on how to help your child at school and help your school best serve your child, includes a sample letter requesting evaluation; from the Tourette Association of America.

National & Local Support

Tourette Association of America
Information regarding the diagnosis and treatment of TS, as well as research, educational concerns, and much more.

Studies/Registries

Children with Tourette Syndrome (clinicaltrials.gov)
Lists numerous studies focusing on children with TS, includes etiology, manifestations, and treatment studies.

Services for Patients & Families in Utah (UT)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: November 2013; last update/revision: February 2020
Current Authors and Reviewers:
Authors: Julia Mattson, MD, PhD
Angela Armen, MD
Senior Author: Samuel H. Zinner, MD
Reviewer: David R. Shprecher, DO, MSCI
Authoring history
2017: update: Francis Filloux, MDR
2016: update: Meghan Candee, MDCA
2013: first version: Lynne M. Kerr, MD, PhDA; Samuel H. Zinner, MDSA
AAuthor; CAContributing Author; SASenior Author; RReviewer

Bibliography

American Psychiatric Association.
Diagnostic and Statistical Manual of Mental Disorders, DSM-5.
Fifth ed. Arlington, VA: American Psychiatric Association; 2013. 978-0-89042-554-1

Bajwa RJ, de Lotbinière AJ, King RA, Jabbari B, Quatrano S, Kunze K, Scahill L, Leckman JF.
Deep brain stimulation in Tourette's syndrome.
Mov Disord. 2007;22(9):1346-50. PubMed abstract

Blackburn JS.
Tic Disorders and PANDAS.
Semin Pediatr Neurol. 2018;25:25-33. PubMed abstract

Bruun RD, Budman CL.
Risperidone as a treatment for Tourette's syndrome.
J Clin Psychiatry. 1996;57(1):29-31. PubMed abstract

Budman CL, Gayer A, Lesser M, Shi Q, Bruun RD.
An open-label study of the treatment efficacy of olanzapine for Tourette's disorder.
J Clin Psychiatry. 2001;62(4):290-4. PubMed abstract

Butler MG.
Management of obesity in Prader-Willi syndrome.
Nat Clin Pract Endocrinol Metab. 2006;2(11):592-3. PubMed abstract
Discusses pros and cons of various ways to treat obesity in Prader-Willi syndrome.

Chang K, Frankovich J, Cooperstock M, Cunningham MW, Latimer ME, Murphy TK, Pasternack M, Thienemann M, Williams K, Walter J, Swedo SE.
Clinical evaluation of youth with pediatric acute-onset neuropsychiatric syndrome (PANS): recommendations from the 2013 PANS Consensus Conference.
J Child Adolesc Psychopharmacol. 2015;25(1):3-13. PubMed abstract / Full Text

Deeb W, Malaty IA, Mathews CA.
Tourette disorder and other tic disorders.
Handb Clin Neurol. 2019;165:123-153. PubMed abstract

Deng H, Gao K, Jankovic J.
The genetics of Tourette syndrome.
Nat Rev Neurol. 2012;8(4):203-13. PubMed abstract

Erenberg G.
The relationship between Tourette syndrome, attention deficit hyperactivity disorder, and stimulant medication: a critical review.
Semin Pediatr Neurol. 2005;12(4):217-21. PubMed abstract

Grados M, Huselid R, Duque-Serrano L.
Transcranial Magnetic Stimulation in Tourette Syndrome: A Historical Perspective, Its Current Use and the Influence of Comorbidities in Treatment Response.
Brain Sci. 2018;8(7). PubMed abstract / Full Text

Kim YH, Son CG, Ku BC, Lee HW, Lim HS, Lee MS.
Herbal medicines for treating tic disorders: a systematic review of randomised controlled trials.
Chin Med. 2014;9(1):6. PubMed abstract / Full Text

Knight T, Steeves T, Day L, Lowerison M, Jette N, Pringsheim T.
Prevalence of tic disorders: a systematic review and meta-analysis.
Pediatr Neurol. 2012;47(2):77-90. PubMed abstract

Leckman JF, Riddle MA, Hardin MT, Ort SI, Swartz KL, Stevenson J, Cohen DJ.
The Yale Global Tic Severity Scale: initial testing of a clinician-rated scale of tic severity.
J Am Acad Child Adolesc Psychiatry. 1989;28(4):566-73. PubMed abstract

Martino D, Pringsheim TM, Cavanna AE, Colosimo C, Hartmann A, Leckman JF, Luo S, Munchau A, Goetz CG, Stebbins GT, Martinez-Martin P.
Systematic review of severity scales and screening instruments for tics: Critique and recommendations.
Mov Disord. 2017;32(3):467-473. PubMed abstract / Full Text

Mathews CA, Stern JS.
The First World Congress on Tourette Syndrome and Tic Disorders: Controversies and Hot Topics in Etiology and Treatment.
Front Neurosci. 2016;10:246. PubMed abstract / Full Text

Pappert EJ, Goetz CG, Louis ED, Blasucci L, Leurgans S.
Objective assessments of longitudinal outcome in Gilles de la Tourette's syndrome.
Neurology. 2003;61(7):936-40. PubMed abstract

Perrin JM, Friedman RA, Knilans TK.
Cardiovascular monitoring and stimulant drugs for attention-deficit/hyperactivity disorder.
Pediatrics. 2008;122(2):451-3. PubMed abstract / Full Text

Pringsheim T, Holler-Managan Y, Okun MS, Jankovic J, Piacentini J, Cavanna AE, Martino D, Müller-Vahl K, Woods DW, Robinson M, Jarvie E, Roessner V, Oskoui M.
Comprehensive systematic review summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders.
Neurology. 2019;92(19):907-915. PubMed abstract / Full Text

Pringsheim T, Okun MS, Müller-Vahl K, Martino D, Jankovic J, Cavanna AE, Woods DW, Robinson M, Jarvie E, Roessner V, Oskoui M, Holler-Managan Y, Piacentini J.
Practice guideline recommendations summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders.
Neurology. 2019;92(19):896-906. PubMed abstract / Full Text

Sallee F, Kohegyi E, Zhao J, McQuade R, Cox K, Sanchez R, van Beek A, Nyilas M, Carson W, Kurlan R.
Randomized, Double-Blind, Placebo-Controlled Trial Demonstrates the Efficacy and Safety of Oral Aripiprazole for the Treatment of Tourette's Disorder in Children and Adolescents.
J Child Adolesc Psychopharmacol. 2017;27(9):771-781. PubMed abstract / Full Text

Sallee FR, Nesbitt L, Jackson C, Sine L, Sethuraman G.
Relative efficacy of haloperidol and pimozide in children and adolescents with Tourette's disorder.
Am J Psychiatry. 1997;154(8):1057-62. PubMed abstract / Full Text

Sandor P.
Pharmacological management of tics in patients with TS.
J Psychosom Res. 2003;55(1):41-8. PubMed abstract

Scharf JM, Miller LL, Gauvin CA, Alabiso J, Mathews CA, Ben-Shlomo Y.
Population prevalence of Tourette syndrome: a systematic review and meta-analysis.
Mov Disord. 2015;30(2):221-8. PubMed abstract

Schrock LE, Mink JW, Woods DW, Porta M, Servello D, Visser-Vandewalle V, Silburn PA, Foltynie T, Walker HC, Shahed-Jimenez J, Savica R, Klassen BT, Machado AG, Foote KD, Zhang JG, Hu W, Ackermans L, Temel Y, Mari Z, Changizi BK, Lozano A, Auyeung M, Kaido T, Agid Y, Welter ML, Khandhar SM, Mogilner AY, Pourfar MH, Walter BL, Juncos JL, Gross RE, Kuhn J, Leckman JF, Neimat JA, Okun MS.
Tourette syndrome deep brain stimulation: a review and updated recommendations.
Mov Disord. 2015;30(4):448-71. PubMed abstract

Silay YS, Jankovic J.
Emerging drugs in Tourette syndrome.
Expert Opin Emerg Drugs. 2005;10(2):365-80. PubMed abstract

Tourette's Syndrome Study Group.
Treatment of ADHD in children with tics: a randomized controlled trial.
Neurology. 2002;58(4):527-36. PubMed abstract / Full Text
This study offers support for using methylphenidate and/or the combination of methylphenidate/clonidine in the treatment of ADHD with tic disorder.

Wilbur C, Bitnun A, Kronenberg S, Laxer RM, Levy DM, Logan WJ, Shouldice M, Yeh EA.
PANDAS/PANS in childhood: Controversies and evidence.
Paediatr Child Health. 2019;24(2):85-91. PubMed abstract / Full Text

Yang CS, Zhang LL, Zeng LN, Huang L, Liu YT.
Topiramate for Tourette's syndrome in children: a meta-analysis.
Pediatr Neurol. 2013;49(5):344-50. PubMed abstract

Yu J, Ye Y, Liu J, Wang Y, Peng W, Liu Z.
Acupuncture for Tourette Syndrome: A Systematic Review.
Evid Based Complement Alternat Med. 2016;2016:1834646. PubMed abstract / Full Text

Zinner SH, Mink JW.
Movement disorders I: tics and stereotypies.
Pediatr Rev. 2010;31(6):223-33. PubMed abstract