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Tourette Syndrome

Description

Other Names

TS, Tourette disorder, Tourette's disorder, Gilles de la Tourettes syndrome, chronic motor tic disorder, chronic vocal tic disorder

Diagnosis Coding

Insurance compensation for patients with Tourette syndrome (TS) is complicated by the diagnosis being in the ICD-9 series for mental disorders (290-319). This may trigger use of mental health benefit rules rather than those for medical benefits despite the mounting evidence that TS is a neurological disorder associated with basal ganglia dysfunction. In addition, many insurance companies mistakenly interpret TS exclusively as a psychiatric disorder and initially deny reimbursement to non-psychiatrists.

If coding for a child with tics before knowing if there is a chronic tic disorder such as Tourette syndrome (TS), use 307.2. For TS, the code for static encephalopathy (348.3) or unspecified nervous system disorder (349.9), along with the code for TS (307.23) may result in appropriate compensation. If payment is denied, an appeal letter to the insurance company specifying that TS is a neurologic disorder and attaching the TS Fact Sheet (NIH) by the NIH National Institute of Neurological Disorders and Stroke (adapted from Zinner, 2004) may help.

ICD-9

307.23, Tourette syndrome

See Tourette ICD9(PDF Document 79 KB) for more detail and for coding of associated conditions.

ICD-10

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

Five codes describe tic disorder. For differentiation among the codes, see Tic Disorder Codes (ICD10).

Description

Tourette syndrome and other chronic tic disorders are childhood-onset neurological conditions characterized by chronicity (lasting over a year). In TS, the condition features the presence of both motor (movement) and phonic (often vocal) tics, although not necessarily at the same time. In contrast, in chronic motor or chronic vocal tic disorder, the condition features the presence of either motor or phonic tics, but not both. The distinction between TS and the other two chronic tic disorders is generally accepted as artificial, and for the purpose of this discussion, the term TS will be used to refer to Tourette syndrome, chronic motor tic disorder and chronic vocal tic disorder. The chronicity (tics lasting a year or more) distinguishes TS from provisional tic disorder (tic duration lasting less than one year, either because a full year has not passed since tic onset or because tic onset and resolution retrospectively lasted less than a full year).

Both 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, for example. Complex motor tics may also involve copropraxia (vulgar gestures or other socially inappropriate actions, such as suddenly reaching for or touching another person inappropriately). Simple phonic tics involve meaningless sudden noises such as sniffing or grunting, while complex phonic tics sound more 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 tics are ultimately expressed. Stress, anxiety, excitement, and fatigue may increase tic frequency and intensity.

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. Onset of the signs and symptoms of these co-morbid conditions can precede, co-occur with, or follow the onset of tics. These conditions usually pose greater risk to the neuro-developmental, psycho-social, and behavioral well-being of the child than do the tics. Adverse consequences of TS on self-esteem, academic performance, and social flexibility are common and can be profound.

Prevalence

Prevalence estimates vary, but nearly 1% of school children may have TS. Males are 3-4 times more likely than females to have TS. [Knight: 2012]

Genetics

The causes of TS are poorly understood, although both genetic and environmental factors influence variations in the expression of tics and of associated conditions. See Tourette Syndrome Mechanisms for more information.

Prognosis

Many people with TS adapt well to tics and neither seek, nor require, treatment. Tics are likely to decrease somewhat or even resolve in late adolescence. [Hassan: 2012] Associated conditions such as ADHD and OCD may be lifelong, although symptoms vary with developmental stages.

Roles Of The Medical Home

Most children with TS can be followed over time within their Medical Home, with periodic surveillance and targeted screening for problems related to tics and associated conditions. Evaluation with a TS specialist may be appropriate, depending on provider comfort with the diagnosis, severity of TS and associated conditions, and if medication trial is being considered. 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 medical clinician such as a neurologist, psychiatrist, or developmental-behavioral pediatrician. Care Notebook, and the Care Notebook (UT Dept. of Health)(PDF Document 467 KB) can help families maintain continuity of care. Ongoing education and support for the child, family, peers, and educators is critical to management of TS. The Tourette Syndrome Association features educational supports, Youth Ambassador Program, and explanations of protections under the Individuals with Disability Education Act (IDEA) and "Section 504" of the Americans with Disabilities Act . Effective child advocacy and, for older youth, self-advocacy depend on positive regard and solid self-esteem.

Practice Guidelines

No U.S. guidelines exist for the management of TS; however, the Scahill reference below is a guide for clinical practice based on a literature review on the assessment and pharmacologic treatment of TS, ADHD, and OCD. Also included are several clinical guidelines from the European Society for the Study of TS.

Scahill L, Erenberg G, Berlin CM Jr, Budman C, Coffey BJ, Jankovic J, Kiessling L, King RA, Kurlan R, Lang A, Mink J, Murphy T, Zinner S, Walkup J.
Contemporary assessment and pharmacotherapy of Tourette syndrome.
NeuroRx. 2006;3(2):192-206. PubMed abstract

Cath DC, Hedderly T, Ludolph AG, Stern JS, Murphy T, Hartmann A, Czernecki V, Robertson MM, Martino D, Munchau A, Rizzo R.
European clinical guidelines for Tourette syndrome and other tic disorders. Part I: assessment.
Eur Child Adolesc Psychiatry. 2011;20(4):155-71. PubMed abstract / Full Text

Roessner V, Plessen KJ, Rothenberger A, Ludolph AG, Rizzo R, Skov L, Strand G, Stern JS, Termine C, Hoekstra PJ.
European clinical guidelines for Tourette syndrome and other tic disorders. Part II: pharmacological treatment.
Eur Child Adolesc Psychiatry. 2011;20(4):173-96. PubMed abstract / Full Text

Verdellen C, van de Griendt J, Hartmann A, Murphy T.
European clinical guidelines for Tourette syndrome and other tic disorders. Part III: behavioural and psychosocial interventions.
Eur Child Adolesc Psychiatry. 2011;20(4):197-207. PubMed abstract

Müller-Vahl KR, Cath DC, Cavanna AE, Dehning S, Porta M, Robertson MM, Visser-Vandewalle V.
European clinical guidelines for Tourette syndrome and other tic disorders. Part IV: deep brain stimulation.
Eur Child Adolesc Psychiatry. 2011;20(4):209-17. PubMed abstract

Müller-Vahl KR, Roessner V.
Treatment of tics in patients with Tourette syndrome: recommendations according to the European Society for the Study of Tourette Syndrome.
Mov Disord. 2011;26(13):2447; author reply 2448. PubMed abstract

Helpful Articles

PubMed search on Tourette syndrome: review articles over

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

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

Bagheri, MM, Kerbeshian, J, and Burd, L.
Recognition and Management of Tourette's Syndrome and Tic Disorders.
American Family Physician. 1999;59(8):2263-2272, 2274. PubMed abstract
Excellent medical information regarding TS for the medical home.

Dooley JM.
Tic disorders in childhood.
Semin Pediatr Neurol. 2006;13(4):231-42. PubMed abstract

Tourette syndrome association.
TS: Basic Information.
Tourette syndrome association; (1997) http://tsa-usa.org/.
Comprehensive source of family-focused information.

Clinical Assessment

Overview

No clinical test is available for TS. Mild TS is possibly as common as 1 in 100 people; related chronic motor or vocal tic disorders (that don't meet all the clinical criteria for TS) are even more common (and commonly overlooked). Short-lived tics lasting less than 1 year occur in up to 1 in 4 children and are classified as “provisional tic disorders.” A medical history and physical exam should include assessment of tics and their impact on academic, social, organizational, family, and health functions. Careful questioning for frequently co-occurring conditions is critical. The relative impact of co-occurring factors on self-esteem and quality of life should be defined.

Screening

No screening test exists for TS. Consider asking about symptoms in children presenting with conditions commonly associated with TS, such as ADHD and OCD.

For Complications

Consider screening for associated behavioral and neurodevelopmental disorders since more than 90% of patients with TS are affected with conditions such as:
  • ADHD (in approximately 50%)
  • OCD (in approximately 30%)
  • Learning problems (in approximately 20%)
  • Anxiety (in approximately 30%)
  • Sleep problems
  • Depression
  • Anger control difficulty
  • Autistic disorder

Presentations

The tics in TS typically first emerge between 4 and 7 years of age; they 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 one 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.)
  • Tics may wax and wane in frequency, 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: Chronic non-TS disorders have either motor or vocal tics, but not both. They may be just as disturbing or disabling as TS. Further, it is difficult to differentiate these tic disorders without benefit of hindsight, as chronic motor tics without phonic tics may later evolve into bona fide TS, and phonic tics are motor tics because muscle movements produce phonic tics. Increasingly, the distinction between TS and the chronic non-TS tic disorders is generally accepted as artificial. TS and chronic motor or vocal tics may remit for weeks to months and, in some affected individuals, may disappear completely. Tics occur along a spectrum of symptoms and severity.
  • 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.
  • 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, although in a minority of patients the medication may exacerbate 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.
  • Spasms: Muscle spasms, such as blepharospasms or spasmodic torticollis, may be confused with TS, but the characteristics of tics in TS should allow differentiation.
  • Stereotypies: Stereotypies are repetitive, rhythmic movements, postures, or utterances and are frequently seen in children with autistic disorder, but can also be observed 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.
  • Postviral encephalitis: This brain-based inflammation is differentiated from tics by medical history and examination.
  • Sydenham chorea : Sydenham chorea is a neurological disorder of childhood resulting from an auto-immune reaction to infection with 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 from about 5 to 15 years old. 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.
  • Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): Pandas is a controversial condition postulated as either 1) a dramatic increase in symptoms in children already known to have tics or obsessive compulsive symptoms or 2) as an appearance of such symptoms in children without prior history, following a Group A beta-hemolytic streptococcal infection, other infection, or non-infectious suspected causes. Because the PANDAS are no longer considered only related to the Streptococcus variety of bacteria, as well as other limitations, the name CANS (Childhood Acute Neuropsychiatric Symptoms) has been proposed. [Singer: 2012] No diagnostic criteria are uniformly accepted as definitive for this putative condition.
  • Wilson disease: 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, incoordination of speech, swallowing, and gait, and behavioral changes can 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.
  • The Diagnostic Decision Tree (European Society for the Study of TS), figure 1, may be helpful for the differential diagnosis of tic disorders.

Comorbid Conditions

More than 90% of patients with TS have one or more associated behavioral or neurodevelopmental conditions. Onset of the signs and symptoms of these co-morbid conditions can precede, co-occur with, or follow the onset of the tics. These conditions may include:
  • ADHD (in approximately 50%)
  • OCD (in approximately 30%)
  • Learning problems (in approximately 20%)
  • Anxiety (in approximately 30%)
  • Sleep problems
  • Depression
  • Anger control difficulty
  • Autistic disorder

History & Examination

TS is not associated with any physical findings other than tics. Since tics are fleeting and suppressible, they are often difficult to see in clinical settings. Individuals with tics may deliberately, or unconsciously, develop techniques to minimize their appearance, so some tics may appear as hair smoothing, stretching, etc.

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 Or Perinatal History

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

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. A child with new-onset tics should undergo a comprehensive evaluation that includes history and physical examination. For patients with known TS, tic severity (which may be determined by frequency, complexity, intensity, interference with social experience and functions in life, and discomfort or pain)) should be monitored in ongoing visits. If the patient is taking medications to reduce tics, track effectiveness, side effects, and the need for ongoing treatment.

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, 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 one 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

General

Watch for subtle signs of tics during the history-taking and physical examination.

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.

Neurologic Exam

Neurological exam may reveal "soft" neuromaturational delay, such as impairment in the quality of fine motor skills.

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

No specific test exists to diagnose TS. Children taking medications should be tested as clinically indicated.

Imaging

Neuroimaging and EEG are not helpful in the diagnosis of TS; however, consultants may order these tests if they suspect or want to rule out another diagnosis.

Genetic Testing

No specific test exists for TS.

Other Testing

Consider a screening EKG if medication is being considered, especially antipsychotics that may increase Q-T interval. Children with a family history of sudden death in children or young adults, hypertrophic cardiomyopathy or long QT syndrome, should also undergo EKG. Other indications, and specifically, indications when considering use of stimulant medications, are determined by using targeted cardiac history and a physical exam. [Perrin: 2008]

Subspecialist Collaborations & Other Resources

Children with TS often benefit from evaluation for associated conditions by specialists. For example, a psychologist or education specialist may evaluation 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.

Developmental Pediatrics (see Services below for relevant providers)

Often focused on the "whole-child" evaluation, with emphases on neurodevelopmental and psychosocial influences on behavior, learning, and growth. May have clinical experience with TS and will have clinical experience with co-occurring conditions, including ADHD and learning disabilities.

Psychiatrist, Child-18 (see Services below for relevant providers)

May have considerable clinical experience with TS; helpful with additional behavioral, learning, or mental health problems.

Pediatric Neurology (see Services below for relevant providers)

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

Treatment & Management

Systems

Neurology

Treatment of co-occurring conditions is critical in patients with TS because they often pose greater challenges to function or quality of life than tics. In addition, effective treatment of co-conditions such as anxiety disorders, sleep disturbances, and ADHD can secondarily result in tic symptom improvement.

To reduce tic severity, a minority of patients will require direct intervention; others will benefit from a "wait and see" approach since tics in TS wax and wane. Treatment options should be discussed with families who may base treatment thresholds on personal priorities and tolerance for the tics, family expectations, and the presence or absence of associated conditions.

Both behavioral approaches and pharmacotherapy have shown some, although incomplete, success in achieving tic reduction, and the combination of both behavioral and pharmacologic approaches may be considered for tics of mild to moderate severity. Medication remains the treatment of choice for tics of greater severity.

Deep brain stimulation (DBS) is an emerging neurosurgical approach reserved for adult patients with very severe tics that pose substantial impairment to quality of life or threaten personal safety. DBS is applied under strict guidelines for select patients who have not responded adequately to comprehensive medication, psychological, and behavioral interventions.

Dietary modifications and allergen control are popular complementary/alternative healthcare approaches for treating TS. Good-quality research is currently very limited, none has shown benefit in reducing tics. Because parents or youth with TS may try scientifically unproven approaches (such as restrictive diets), this interest should be discussed and appropriate guidance should balance respect for a family's interests and values with evidence regarding safety and efficacy.

A decision tree for Tourette syndrome and other tic disorders is available in the European clinical guidelines for Tourette syndrome and may be helpful. Management Decision Tree (European Society for the Study of TS).

General guidelines of pharmacotherapy:
  • Because the majority of children diagnosed with TS have co-morbid conditions, it is essential to consider their potential impact. Management should focus on areas of greatest consequence on social, academic, or other areas of function. Most often, comorbid conditions warrant first consideration.
  • With appropriate information, most children and families will choose not to treat tics.
  • Any benefits of medications need to be weighed against potential side effects.
  • Most medications used for tic reduction are prescribed "off-label."
  • Tics tend to wax and wane over time; before treatment is started, baseline severity should be assessed for several months. The Yale Global Tic Severity Scale ([Leckman: 1989]) is available to document tics according to type, severity and impairment, but is designed for use by a trained observer. Other tic-rating scales (for example, see the Tic Severity Checklist(PDF Document 16 KB)) are easier to use in the Medical Home setting.
  • The treatment of tics depends on trial and error with frequent clinical visits to assess response and adjust dosage. A provider with expertise in tic management is preferable. Because tics wax and wane, medication trials should be conducted over prolonged time (weeks to months). As a rule of thumb, medications should be started at small doses and increased gradually.
  • If medications for tics are to be discontinued, most should be weaned over time, and only under provider guidance.
  • When ADHD is co-morbid with TS, the treatment for ADHD should be the same as that for ADHD in a child without tics. Stimulant medications are unlikely to influence tic behavior. As tics will wax and wane naturally, it might appear in some cases that stimulant medication is exacerbating tics. Families should be alerted to the possibility that the course of tics is unpredictable and that tics may intensify, irrespective of ADHD treatment. [Tourette's: 2002] [Erenberg: 2005]
  • Although the alpha-2 adrenergic agonists clonidine and guanfacine (see below) may be helpful for the treatment of ADHD symptoms in this population, these medications are not generally as successful as treatment with stimulants and takes weeks to months to show beneficial effects. The alpha-2 agonists may, however, decrease the frequency and intensity of tics in some patients, help reduce hyperactive and impulsive behaviors in children with co-occurring ADHD, and when taken in the evening, assist with reducing sleep onset latency.

Specific Medications:

  • Alpha-2 adrenergic agonists. Clonidine and guanfacine. Although these agents may take weeks or longer to decrease tics and their impact in reducing tics is less certain and usually less robust than with other agents, the alpha-2 adrenergic agonists are often the drug class of first choice because they have a more tolerable side effect profile than other drug class options, including no risk of tardive dyskinesia. Sedation is the most likely side effect of these medications, especially when the short-acting forms are used. The short-acting forms, which may need to be given 3-4 times a day, may be selected initially to assess for tolerability and then switched to one of the long-acting preparations.
    Long-acting preparations are available for clonidine and guanfacine and a transdermal patch for clonidine. The clonidine patch, which needs to be changed only once a week, produces less sleepiness than the oral form but local allergic reactions to the patch are common. Guanfacine has a longer half-life and is reportedly less sedating than clonidine.
    Additionally, these agents may reduce hyperactive and impulsive behaviors associated with ADHD and reduce latency to sleep onset in patients with difficult in sleep initiation.
  • Neuroleptics. These medications are usually reserved for more severe tics. Although neuroleptics work well, no single medication is successful in all people with TS at doses that don't cause some side effects. Medications should be started at small doses, gradually increasing 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 in advance. Atypical neuroleptics pose a risk for development of insulin resistance and diabetes – periodic laboratory monitoring is required.

    Atypical neuroleptics are generally preferred over typical neuroleptics for treatment in tic reduction. To date however, only the typical neuroleptics haloperidol and pimozide are approved by the FDA for the management of tics in TS. [Silay: 2005] [Sallee: 1997] Among atypicals, risperidone and olanzapine have been shown to be effective. [Sandor: 2003] [Budman: 2001] [Bruun: 1996] Families should be told that, after long-term use, neuroleptics need to be discontinued slowly to avoid withdrawal problems, that include a rebound in tic frequency, and tardive dyskinesia, a rare but permanent movement disorder particularly involving the mouth.
  • Other Medications. Benzodiazepines, antispasmodic agents (baclofen), dopamine agonists (permax),anti-epileptic drugs (topiramate in particular), tetrabenazine and botulinum injections are also considered options for treatment of tics. Botulinum toxin (Botox) is most helpful when the patient is experiencing a severe, localized tic, such as in the face or neck. The botulinum toxin is injected by an experienced practitioner into the affected muscle groups. The therapeutic effect lasts for several months.

Comprehensive Behavioral Intervention for Tics (CBIT) may be as effective as pharmacotherapy in reducing tic behaviors in both children and adults. This approach combines Habit Reversal Training and Functional Analysis. Other behavioral approaches either have not been adequately assessed or do not show efficacy. For more information, see Comprehensive Behavioral Intervention for Tics (CBIT), figure 1.

Subspecialist Collaborations & Other Resources

For the child with severe TS and/or associated problems, co-management with a TS expert may be helpful. Specialists within child neurology, child psychiatry, or developmental pediatrics will have varying degrees of experience with TS, which will vary by community. . In addition, children with TS often need assessment by occupational therapists and psychologists.For the child with severe TS and/or associated problems, co-management with a TS expert may be helpful. Specialists within child neurology, child psychiatry, or developmental pediatrics will have varying degrees of experience with TS – which group has the most expertise will vary by community. In addition, children with TS often need assessment by occupational therapists and psychologists.

Developmental Pediatrics (see Services below for relevant providers)

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

Psychiatrist, Child-18 (see Services below for relevant providers)

For child with additional behavioral, learning, or mental health problems; may have clinical experience with TS..

Pediatric Neurology (see Services below for relevant providers)

For 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 autistic spectrum disorder) often improve functioning of the child with TS as an adult. The Medical Home provider should monitor for signs of these conditions and provide treatment with co-management and referral to councilor as necessary.

Subspecialist Collaborations & Other Resources

Psychiatrist, Child-18 (see Services below for relevant providers)

Help with management of TS and associated disorders.

Psychologist, Child-18 (see Services below for relevant providers)

Helps with evaluation of associated disorders, provides psychometric assessment to include IQ and academic achievement testing, assessment of executive functions, and counseling for the child and/or family as needed—should have experience with TS.

Learning/Education/Schools

A full psycho-educational evaluation that includes evaluation of IQ and other cognitive components, academic achievement skills, and learning strengths and weaknesses, as well as assessment of attention problems and social function, might be helpful in educational planning if problems in school are occurring. A public school district must complete such evaluations when indicated at no cost to the family. Private assessment is also available and may or may not duplicate components evaluated via the public school district. A Medical Home provider for a child with TS may serve as a helpful advocate to children for whom qualification for public school district testing is uncertain. Testing is time-consuming and expensive, so should be performed with specific goals in mind. Private testing is often covered or partially covered by insurance benefits. Insurance is more likely partially to pay if results lead to changes in treatment of the condition(s). See a sample of an Insurance Letter for Preauthorization (psych testing, TS)(PDF Document 58 KB).

Subspecialist Collaborations & Other Resources

Psychologist, Child-18 (see Services below for relevant providers)

Performs psychological profile to guide educational planning when TS is complicated by associated disorders and learning problems.

Neuropsychology (see Services below for relevant providers)

Performs neuro-psychological profile 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).

Frequently Asked Questions

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. [Tourette: 2007].

Will my child with TS need special education?

Children with TS have the same IQ distribution as the entire population; however, special education support may be helpful for children with tics and/or the problems associated with TS, such as attention deficits and learning disabilities. See Educational information (TSA) for more information.

Will my child with TS always have problems?

Tic symptoms usually decrease as children mature; some adults show no symptoms of TS as adults. Co-occurring conditions can still pose challenges and must be considered individually.

Issues Related to Tourette Syndrome

Resources

Information for Clinicians

Tourette syndrome (Mayo Clinic)

Helpful Articles

PubMed search on Tourette syndrome: review articles over

Bagheri, MM, Kerbeshian, J, and Burd, L.
Recognition and Management of Tourette's Syndrome and Tic Disorders.
American Family Physician. 1999;59(8):2263-2272, 2274. PubMed abstract
Excellent medical information regarding TS for the medical home.

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

Dooley JM.
Tic disorders in childhood.
Semin Pediatr Neurol. 2006;13(4):231-42. PubMed abstract

Tourette syndrome association.
TS: Basic Information.
Tourette syndrome association; (1997) http://tsa-usa.org/.
Comprehensive source of family-focused information.

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 originally from dbpeds.org. An accurate record of the type and frequency of tics is helpful to assess the need for and response to medications. This checklist can be downloaded for family use.

Patient Education & Instructions

Tourette Syndrome "Plus"
Information for parents and patients about TS plus related disorders from Leslie Packer, Ph.D., a psychologist, spouse and a mother of children with TS. Includes sections on behavior and education

Information & Support for Families

The Tourette Syndrome Association published a book for families that can be purchased from their website, A Family's Guide to Tourette Syndrome [Various: 2013].

Family Diagnosis Page

Information on the Web

Tourette syndrome (Genetics Home Reference)
Information for patients/families about TS; National Library of Medicine.

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

Ask the expert newsletters (TSA)
Articles written by professionals that address questions about treatment, coping, and medical issues affecting people with TS and their families.

Support National & Local

Tourette Syndrome Association
The only national organization serving children, individuals, and families affected by Tourette Syndrome (TS). Includes six online CME/CNE programs, recognition, diagnosis and treatment programs, training for allied professionals and educators, and resources for patients and families.

Studies/Registries

Ongoing Studies on Tourette Syndrome (clinicaltrials.gov)
List of numerous studies focusing on TS; includes etiology, manifestations, and treatment studies.

Services for Patients & Families

In addition to diagnostic services, children with TS often benefit from evaluation by specialists for associated conditions. For example, a psychologist, neuropsychologist, or education specialist may evaluate the child's behavior or learning problems, or an occupational therapist might best evaluate fine motor coordination problems. If you have chosen a particular state, local experts will be shown below; otherwise, all experts in our database will be shown.

Developmental Pediatrics

See all Developmental Pediatrics services providers (5) in our database.

Neuropsychology

See all Neuropsychology services providers (10) in our database.

Occupational Therapy

See all Occupational Therapy services providers (33) in our database.

Pediatric Neurology

See all Pediatric Neurology services providers (4) in our database.

Psychiatrist, Child-18

See all Psychiatrist, Child-18 services providers (15) in our database.

Psychologist, Child-18

See all Psychologist, Child-18 services providers (77) in our database.

For other services related to this condition, browse our Services categories or search our database.

Authors

Authors: Samuel H. Zinner, M.D. - 11/2013
Lynne M Kerr, MD, PhD - 11/2013
Content Last Updated: 12/2013

Bibliography

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

Bagheri, MM, Kerbeshian, J, and Burd, L.
Recognition and Management of Tourette's Syndrome and Tic Disorders.
American Family Physician. 1999;59(8):2263-2272, 2274. PubMed abstract
Excellent medical information regarding TS for the medical home.

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

Cath DC, Hedderly T, Ludolph AG, Stern JS, Murphy T, Hartmann A, Czernecki V, Robertson MM, Martino D, Munchau A, Rizzo R.
European clinical guidelines for Tourette syndrome and other tic disorders. Part I: assessment.
Eur Child Adolesc Psychiatry. 2011;20(4):155-71. PubMed abstract / Full Text

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

Dooley JM.
Tic disorders in childhood.
Semin Pediatr Neurol. 2006;13(4):231-42. 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

Hassan N, Cavanna AE.
The prognosis of Tourette syndrome: implications for clinical practice.
Funct Neurol. 2012;27(1):23-7. PubMed abstract

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

Müller-Vahl KR, Cath DC, Cavanna AE, Dehning S, Porta M, Robertson MM, Visser-Vandewalle V.
European clinical guidelines for Tourette syndrome and other tic disorders. Part IV: deep brain stimulation.
Eur Child Adolesc Psychiatry. 2011;20(4):209-17. PubMed abstract

Müller-Vahl KR, Roessner V.
Treatment of tics in patients with Tourette syndrome: recommendations according to the European Society for the Study of Tourette Syndrome.
Mov Disord. 2011;26(13):2447; author reply 2448. 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

Roessner V, Plessen KJ, Rothenberger A, Ludolph AG, Rizzo R, Skov L, Strand G, Stern JS, Termine C, Hoekstra PJ.
European clinical guidelines for Tourette syndrome and other tic disorders. Part II: pharmacological treatment.
Eur Child Adolesc Psychiatry. 2011;20(4):173-96. 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

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

Scahill L, Erenberg G, Berlin CM Jr, Budman C, Coffey BJ, Jankovic J, Kiessling L, King RA, Kurlan R, Lang A, Mink J, Murphy T, Zinner S, Walkup J.
Contemporary assessment and pharmacotherapy of Tourette syndrome.
NeuroRx. 2006;3(2):192-206. PubMed abstract
Guidelines from the Tourette Syndrome Association Medical Advisory Board: Practice Committee.

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

Tourette Syndrome Association.
What is Tourette syndrome? and TS: Frequently Asked Questions.
Tourette syndrome association; (2007) http://www.tsa-usa.org/People/LivingWithTS/Images/Fact_Sheet.pdf.
Basic information regarding the diagnosis of TS as well as information about the TS Association for individuals and families with TS

Tourette syndrome association.
TS: Basic Information.
Tourette syndrome association; (1997) http://tsa-usa.org/.
Comprehensive source of family-focused information.

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

Various.
A Family's Guide to Tourette Syndrome.
First ed. Bayside, NY: Tourette Syndrome Association; 2013. http://www.tsa-usa.org/news/FamilyGuide_book0612.html
Written by 20 of the world’s leaders in understanding Tourette syndrome in a non-technical manner for people affected by TS or caring for those with TS.

Verdellen C, van de Griendt J, Hartmann A, Murphy T.
European clinical guidelines for Tourette syndrome and other tic disorders. Part III: behavioural and psychosocial interventions.
Eur Child Adolesc Psychiatry. 2011;20(4):197-207. PubMed abstract

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