Tourette syndrome (abbreviated as Tourette's or TS) is an inherited neurodevelopmental disorder that begins in childhood or adolescence, characterized by the presence of motor and phonic tics. The management of Tourette syndrome has the goal of managing symptoms to achieve optimum functioning, rather than eliminating symptoms; not all persons with Tourette's require treatment, and there is no cure or universally effective medication. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.
Tourette syndrome patients may exhibit symptoms of other comorbid conditions along with their motor and phonic tics. Associated conditions include attention-deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), learning disabilities and sleep disorders. Patients who have ADHD along with Tourette's may also have problems with disruptive behaviors, overall functioning, and cognitive function. Co-occurring OCD can also be a source of impairment, necessitating treatment. Not all persons with tics will also have other conditions and not all persons with tics require treatment, but when comorbid disorders are present, they often require treatment.
Management priority Management of Tourette syndrome can be divided into treatment of tics, and treatment of co-occurring conditions, which, when present, are often a larger source of functional impairment than the tics themselves.
There is no cure for Tourette's. No one medication effectively treats all symptoms, most medications prescribed for tics have not been approved for that use, and no medication is without the risk of significant adverse effects. Treatment is focused on identifying the most troubling or impairing symptoms and helping the individual manage them. Because comorbid conditions are often a larger source of impairment than tics, they are a priority in treatment. The management of Tourette's is individualized and involves shared decision-making between the clinician, patient, family and caregivers.Müller-Vahl KR (2013) in Martino D, Leckman JF, eds, p. 628.
Education, reassurance and psychobehavioral therapy are often sufficient for the majority of cases. Full text, archived May 25, 1998. Also see In particular, psychoeducation targeting the patient and their family and surrounding community is a key management strategy. Watchful waiting "is an acceptable approach" for those who are not functionally impaired. Symptom management may include behavioral, psychological and pharmacological therapies. Pharmacological intervention is reserved for more severe symptoms, while psychotherapy or cognitive behavioral therapy (CBT) may ameliorate depression and social isolation, and improve family support. The decision to use behavioral or pharmacological treatment is "usually made after the educational and supportive interventions have been in place for a period of months, and it is clear that the tic symptoms are persistently severe and are themselves a source of impairment in terms of self-esteem, relationships with the family or peers, or school performance".Sukhodolsky, et al (2017), p. 248.
Psychoeducation and social support Knowledge, education and understanding are uppermost in management plans for tic disorders, and psychoeducation is the first step.Müller-Vahl KR (2013) in Martino D, Leckman JF, eds, pp. 623–24. A child's parents are typically the first to notice their tics;Müller-Vahl KR (2013) in Martino D, Leckman JF, eds, p. 625. they may feel worried, imagine that they are somehow responsible, or feel burdened by misinformation about Tourette's. Before seeing a doctor, many parents research TS on the internet,Müller-Vahl KR (2013) in Martino D, Leckman JF, eds, p. 626. "Quite often, the unimpaired child receives medical treatment to reduce tics, when instead the parents should more appropriately receive psychoeducation and social support to better cope with the condition." or have seen media portrayals of severe cases.Müller-Vahl KR (2013) in Martino D, Leckman JF, eds, p. 627. Parents may be overly concerned, and not realize that they, too, have tics, while the child may not be bothered by the tics. In some cases, neither the parents nor their child know they are ticcing; pointing out tics in this case will unnecessarily draw attention to them. In such a case, informing a parent who is unaware of their own tics can be disturbing, and they have a "right not to be informed". When their child receives a diagnosis of Tourette syndrome, both parent and child usually feel relieved, although the diagnosis can also cause distress for the parents as they confront a chronic condition that can be difficult to treat. They may also react with disbelief, denial, anger or resentment, or be exhausted and discouraged by previous encounters with medical professionals and the daily challenges faced by their child and family.Abi-Jaoude E, Kidecekl D, Stephens R, et al (2009), in Carlstedt RA (ed). p. 564. Effectively educating parents about the diagnosis and providing social support can ease their anxiety. This support can also lower the chance that their child will be unnecessarily medicated or experience an exacerbation of tics due to their parents' emotional state. Psychoeducation that encourages a "matter-of-fact" attitude, and helps dispel misconceptions and stigma, is most effective when provided to parents.
People with Tourette's may suffer socially if their tics are viewed as "bizarre". If a child has disabling tics, or tics that interfere with social or academic functioning, supportive psychotherapy or school accommodations can be helpful. Even children with milder tics may be angry, depressed or have low self-esteem as a result of increased teasing, bullying, rejection by peers or social stigmatization, and this can lead to social withdrawal. Some children feel empowered by presenting a peer awareness program to their classmates.Pruitt SK & Packer LE (2013) in Martino D, Leckman JF, eds, pp. 646–47. It can be helpful to educate teachers and school staff about typical tics, how they fluctuate during the day, how they impact the child, and how to distinguish tics from naughty behavior. By learning to identify tics, adults can refrain from asking or expecting a child to stop ticcing,Muller-Vahl KR (2013) in Martino D, Leckman JF, eds, p. 629. because "tic suppression can be exhausting, unpleasant, and attention-demanding and can result in a subsequent rebound bout of tics". The presence of ADHD is associated with functional impairment, disruptive behavior, and tic severity. Strategies to help the child at school can be established; these include allowing the child to chew gum to help reduce vocal tics, to use a laptop instead of writing by hand, and to take breaks from the classroom when tics are high. Providing additional test time can also be helpful, as can using oral tests when needed.
Adults with TS may withdraw socially to avoid stigmatization and discrimination because of their tics. Depending on their country's healthcare system, they may receive social services or help from support groups.Müller-Vahl KR (2013) in Martino D, Leckman JF, eds, p. 633.
Behavioral Behavioral therapies using habit reversal training (HRT) and exposure and response prevention (ERP) are first-line interventions, and have been shown to be effective. Because tics are somewhat suppressible, when people with TS are aware of the premonitory urge that precedes a tic, they can be trained to develop a response to the urge that competes with the tic.
Comprehensive behavioral intervention for tics (CBIT) is based on HRT, the best researched behavioral therapy for tics. CBIT has been shown with a high level of confidence to be more likely to lead to a reduction in tics than other behavioral therapies or psychoeducation. CBIT has some limitations. Children under ten may not understand the treatment, and people with severe tics or ADHD may not be able to suppress their tics or sustain the focus required to benefit from behavioral treatments. There is a lack of therapists trained in behavioral interventions, and finding practitioners outside of specialty clinics can be difficult. Costs may also limit accessibility. TS experts debate whether increasing a child's awareness of tics with HRT/CBIT (as opposed to ignoring tics) can lead to more tics later in life.
There is little evidence supporting massed negative practice in the management of tics.
In the UK, where there is a shortage of therapists trained to deliver behavioral treatments such that behavioral therapy is not available to most for whom it is recommended, the National Institute for Health and Care Research funded a study of behavioral therapy delivered online to individuals with moderate to severe tics and states that it might be effective in reducing tics and could help remedy the lack of professionals.
When disruptive behaviors related to comorbid conditions exist, anger control training and parent management training can be effective.Sudhodolsky, et al (2017), p. 250. CBT is a useful treatment when OCD is present. Relaxation techniques, such as exercise, yoga and meditation may be useful in relieving the stress that can aggravate tics. Beyond HRT, the majority of behavioral interventions for Tourette's (for example, relaxation training and biofeedback) have not been systematically evaluated and are not empirically supported.
Medication
Children with tics typically present when their tics are most severe, but because the condition waxes and wanes, medication is not started immediately or changed often. Tics may subside with education, reassurance and a supportive environment. When medication is used, the goal is not to eliminate symptoms. Instead, the lowest dose that manages symptoms without adverse effects is used, because adverse effects may be more disturbing than the symptoms being treated with medication.
There are no medications specifically designed to target tics, although some antipsychotics (for example, pimozide) have been FDA-approved for treating Tourette's. Medications which are used as primary treatment in other conditions are used with some success in treating tics. Neuroleptic medications (antipsychotics), such as haloperidol (brand name Haldol) or pimozide (brand name Orap), have historically been and continue to be the medications with the most proven efficacy in controlling tics. These medications work by blocking dopamine receptors, and are associated with a high side effect profile. The traditional antipsychotic drugs are associated with tardive dyskinesia when used long-term; and parkinsonism, dystonia, dyskinesia, and akathisia when used short-term. Additional side effects can be school phobia (a form of separation anxiety), depression, weight gain, and cognitive blunting (dulling of cognitive ability). Another traditional antipsychotic used in treating Tourette's is fluphenazine (brand name Prolixin), although the evidence supporting its use is less than that of haloperidol and pimozide.
The classes of medication with proven efficacy in treating tics—typical and atypical neuroleptics—can have long-term and short-term adverse effects. The antihypertensive agents are also used to treat tics; studies show variable efficacy, but a lower side effect profile than the neuroleptics. See also There is moderate evidence that the antihypertensive clonidine, along with aripiprazole, haloperidol, risperidone, and tiapride, reduce tics more than placebo. Aripiprazole and risperidone are likely to lead to weight gain and sedation or fatigue; tiapride may produce sleep disturbances and tiredness; and clonidine may produce sedation. Risperidone and haloperidol may produce extrapyramidal symptoms, and increase prolactin levels. Because of lower side effects, aripiprazole is preferred over other antipsychotics.