Tourette Syndrome

Tourette syndrome (TS) is a chronic, familial, neurobiological disorder with its

 onset in childhood and is marked by presence of involuntary motor movements and sounds, known as motor and vocal tics respectively (Bagheri, Kerbeshian & Burd, 1999). According to Lingui-Systems (1999, cited in Prestia, 2003), approximately 1 in every 2,500 individuals suffer from TS, with boys being diagnosed more frequently than girls. Tourette's syndrome also known as Gilles de la Tourette syndrome (after its discoverer Georges Gilles de la Tourette, a French neurologist), can be considered to be the most complex and severe manifestation of the spectrum of tic disorders (Kwak & Jankovic, 2002).

Etiology and Pathogenesis

The exact cause of TS is not known. TS is supposed to have a multifactorial

etiology with, genetic and environmental factors playing an important role in its development. Concordance rate of about 60% in monozygotic twins and 10% in dizygotic twins suggest genetic basis behind TS (Bagheri et al, 1999). The mode of transmission of Tourette's syndrome is controversial. Though the exact patho-physiological mechanism underlying the disease has yet not been discovered, abnormalities in basal ganglia (shown in figure 1) and frontal cortex have been implicated in the pathogenesis of Tourette's syndrome (Bagheri et al).

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Abnormality in secretion of neuro-transmitters especially dopamine is also thought to be associated with TS (Bagheri et al).

Figure1. Brain Structures Involved in Tourette Syndrome

Source: John Henkel, J. (2006). Food and Drug Administration. Retrieved on 21 April 2007 from http://en.wikipedia.org/wiki/Image:Brain_structure.gif

Symptoms

Tourette's syndrome follows a fluctuating course in most children. Symptoms

 usually appear at about 6-7 years of age.

 Many factors like anxiety, stress, substance abuse etc can intensify tics. Presence of tics (both motor and vocal) is characteristic of TS. According to American Psychiatric Association’s Diagnostic and Statistical manual of mental disorders, fourth edition- text revision. (DSM-IV-TR, 2000), tics are defined as sudden, rapid, purposeless, repetitive, non-rhythmic, stereotyped movements or vocalizations.

Tics in TS are not constantly present (except in extremely severe cases) and occur on a background of normal motor activity (Kwak & Jankovic, 2002). This disorder usually begins with simple tics which progress over time into more complex ones.  Different types of simple and complex tics as described by Bagheri et al (1999) which can be seen in patients with TS have been enumerated in table 1. Besides TS, tics can be seen associated with other neurologic disorders like (encephalitis, Huntington’s disease etc). These secondary tics, not associated with Tourette syndrome are commonly referred to as tourettism ((Bagheri et al, 1999).

Table 1.Common Types of Tics Seen in Patients with Tourette Syndrome.

Source: Bagheri, M.M. Kerbeshian, J., &Burd, L. (1999). Recognition and management of Tourette’s Syndrome and tic disorders. American Family Physician, 59 (8). Retrieved on 21 April from http://www.aafp.org/afp/990415ap/2263.html

Motor tics

Motor tics can be described as brief movements which occur due to  involuntary muscle contraction. Motor tics can be simple or complex in nature (see table 1). Tics usually occur as an involuntary movement or as a response to an involuntary urge to perform the movement (Kwak & Jankovic, 2002). These involuntary movements are often perceived as voluntary by the patient and he tries to suppress them. Suppression of tics results in building up of an inner tension or a feeling of discomfort. This leads a further burst of tics which is relieved after performance of the movement (Kwak & Jankovic, 2002).

Vocal tics

Vocal or phonic tics represent involuntary sounds produced due to contraction of

 nasal or oro-pharyngeal muscles (Kwak & Jankovic, 2002). Like somatic motor tics, vocal tics can also be divided into simple and complex tics (shown in table 1). Coprolalia once thought to be characteristic of TS is present in about 8-25% cases (Bagheri et al, 1999). Another type of speech disturbance present in some patients is ‘blocking tics’. They are manifested by either hesitancy in starting the speech or sudden interruption in the flow of normal speech (Kwak & Jankovic, 2002) Developmental stuttering is also sometimes seen in patients with TS (Kwak & Jankovic, 2002).

Tourette’s syndrome is often associated with other co-morbid conditions like obsessive compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), behavior problems and learning disabilities (Prestia, 2003).

Diagnosis

Diagnosis of TS is usually made clinically based on recognition of tics. The Diagnostic criteria for TS as defined by American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, fourth edition- text revision. (DSM-IV-TR), 2000 is shown in table 1.

Table 1. DSM-IV-TR Diagnostic Criteria for Tourette's Disorder

Treatment

     Treatment of TS may require a multi-disciplinary team approach involving

 neurologists, psychiatrists, psychologists, social workers, occupational therapists, educators etc. (Kwak & Jankovic, 2002).An algorithm for management of TS as described by Bagheri et al (1999) is shown in figure 2.

Pharmacological therapy

           Medical therapy should aim at the treatment of most disabling symptom. Tics

 (both vocal and motor) respond best to dopamine blocking drugs (Kwak & Jankovic, 2002). These can include conventional neuroleptics like haloperidol and pimozide and the atypical neuroleptic agents like risperidone. Neuroleptic agents are associated with numerous side effects like sedation, weight gain, impaired academic performance, in children and extra pyramidal movement symptoms (like tardive dyskinesia).

Since TS is frequently associated with other co-morbid conditions like OCD, ADHD etc, pharmacotherapy should be tailored to treat these conditions as well (Kwak & Jankovic, 2002). For e.g. Central nervous system stimulants (amphetamines and dextroamphetamine) can be used for treatment of ADHD and anti-depressants (selective serotonin reuptake inhibitors) for management of OCD, if present. Injections of botulinum toxin can be used for tics isolated to one body region such as the eyes, neck, or vocal cords.

Non-Pharmacologic interventions

Since TS most commonly affects children in the age when they are attending school, symptoms of TS can affect their academic performance, cause inference with their social-emotional development, and impose limitations due to physical disabilities produced as a result of tics (Prestia, 2003).

It is the duty of educational providers, school personnel and parents to keep these limitations in mind and to draw out an adequate plan incorporating carefully thought out interventions and adaptations, in order to enhance the success and performance of children suffering with TS, at school (Prestia, 2003). Behavior treatment and counseling can be used to promote development of social and academic skills in children and to prevent exacerbation of symptoms related to TS, by modifying their behavior (Bagheri et al, 1999).

Source: Bagheri, M.M. Kerbeshian, J., & Burd, L. (1999). Recognition and management of Tourette’s Syndrome and tic disorders. American Family Physician, 59 (8). Retrieved on 21 April from http://www.aafp.org/afp/990415ap/2263.html

Prognosis

TS is a lifelong disorder with an unpredictable course (Prestia, K. (2003). The

 clinical course of TS as described by Bagheri et al (1999) is shown in figure 3.  As shown in figure 3, symptoms of TS diminish during and after adolescence in about 85% of patients. About 5 to 10 % of patients may show no improvement. Most patients with TS require medication for up to one to two years. About 15 % of patients may require long-term medication for tic control.

Figure 3. Clinical Course of Tourette's Syndrome

Source: Bagheri, M.M. Kerbeshian, J., &Burd, L. (1999). Recognition and management of Tourette’s Syndrome and tic disorders. American Family Physician, 59 (8). Retrieved on 21 April from  http://www.aafp.org/afp/990415ap/2263.html

Conclusion

TS is not an uncommon problem and can be often encountered in school children. TS is a multifactorial neurological disorder requiring an integrated treatment approach targeting at the other co-morbid conditions associated with TS, as well. Treatment must also incorporate an effective multi-disciplinary team approach aiming at treatment and recognition of underlying complex mechanisms, solving speech and language problems and promoting socio-emotional and academic development to substantially improve the quality of life and performance of individuals with TS.

Article Review

The article by Prestia, 2003 focuses on need of educators to understand the special academic, social-emotional, and physical needs of the children suffering from TS. Educators need to make use of simple interventions to help these children overcome their illness and achieve best possible performance at school. TS can cause significant academic, social-emotional, and physical challenges for the child as described below:

Academic Challenges

TS does not directly affect intelligence, and many students with TS have average or above average IQs. Still approximately 40% of individuals with TS can have learning problems. This is so as many individuals with TS have associated comorbid disorders like learning disabilities, OCD, ADHD, Asperger syndrome etc. Assistance can be provided through special education services or individualized education program (IEP) or a 504 Plan.

If the student does not qualify for either, classroom teachers can make use of simple interventions and plans by using available resources and their own knowledge .For e.g. if the child is having trouble with writing, teachers can make use of alternatives like using oral assessment tests instead of written assignments, using multiple-choice questions etc. Since stress can precipitate tics, teachers should take steps to eliminate any source of stress for the student.

Social-emotional Challenges

Children with TS tend to have problems in interacting with and being accepted by their peers due to abnormal behavior produced as a result of motor and vocal tics. Such children may become isolated and depressed and are at risk for developing poor self-esteem and self-confidence. They are also easy targets to bullying. Educators should take steps to encourage acceptance and support of such students by their peers.

Physical Challenges

Underlying physical problems accompanying the disorder often go unaddressed. Educators should properly assess these problems by consulting a multi-disciplinary team (MDT) involving a physical therapist, occupational therapist, and adapted physical education teacher in order to determine the student's motor strengths and areas of need. For e.g.  Impairments in sensory processing or motor planning may interfere with normal day to day non-academic activities like toileting, eating lunch, etc. Simple solutions can be offered by the MDT to facilitate independence and to prevent self-harm during the period of tic release in such children.

        In my view this article provides helpful suggestions that can be utilized in school settings. In order to achieve optimal development and performance of these students, educators must make use of simple and carefully thought out interventions. They should find out ways to promote social-emotional development and also help them in coping with learning and physical disabilities, imposed by the tics.

References

American Psychiatric Association’s Diagnostic and Statistical manual of mental

 disorders, fourth edition- text revision.  (2000). Retrieved on 21 April from

Bagheri, M.M., Kerbeshian, J., & Burd, L. (1999). Recognition and management of

 Tourette’s Syndrome and tic disorders. American Family Physician, 59 (8). Retrieved on 21 April from http://www.aafp.org/afp/990415ap/2263.html

Kwak, C. & Jankovic, J. (2002). The Neurology of a Tic: From the Point of View

 of the Scientist. Retrieved on 21 April 2007 from http://www.asha.org/about/publications/leader-online/archives/2002/q3/020806a.htm

Prestia, K. (2003).Tourette's syndrome: Characteristics and interventions. Intervention

 in school and clinic, 39(2), 67. Retrieved on 21 April 2007 from http://proquest.umi.com/pqdweb?index=37&did=443394391&SrchMode=1&sid=2&Fmt=4&VInst=PROD&VType=PQD&RQT=309&VName=PQD&TS=1176684625&clientId=18091