Management of Tourette syndrome

Managing symptoms of Tourette syndrome
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;

There is no cure for Tourette's. Treatment is focused on identifying the most troubling or impairing symptoms and helping the individual manage them. The management of Tourette's is individualized and involves shared decision making between the clinician, patient, family and caregivers. Education, reassurance and psychobehavioral therapy are often sufficient for the majority of cases. 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.

Knowledge, education and understanding are uppermost in management plans for tic disorders, A child's parents are typically the first to notice their tics; they may feel worried, imagine that they are somehow responsible, or feel burdened by misinformation about Tourette's. or have seen media portrayals of severe cases. Parents may be overly concerned, 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 Psychoeducation that encourages a "matter of fact" attitude, and helps dispel misconceptions and stigma, is most effective when provided to parents. 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. 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, because "tic suppression can be exhausting, unpleasant, and attention demanding and can result in a subsequent rebound bout of tics". 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.

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. and finding practitioners outside of specialty clinics can be difficult. When disruptive behaviors related to comorbid conditions exist, anger control training and parent management training can be effective. CBT is a useful treatment when OCD is present.

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. 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. Because of lower side effects, aripiprazole is preferred over other antipsychotics. The α2 adrenergic receptor agonists (antihypertensive agents) show some efficacy in reducing tics, as well as other comorbid features of some people with Tourette's. Originally developed to treat high blood pressure, these medications are a safer alternative to neuroleptic medications for the people with TS that respond to them. This class of medication is often the first tried for tics, as the antihypertensives have a lower side effect profile than some of the medications with more proven efficacy. The evidence for their safety and efficacy is not as strong as the evidence for some of the standard and atypical neuroleptics, but there is fair supportive evidence for their use, nonetheless. This class of medication takes about six weeks to begin to work on tics, so sustained trials are warranted. Because of the blood pressure effects, antihypertensive agents should not be discontinued suddenly. Clonidine (brand name Catapres) works on tics for about half of people with TS. Maximal benefit may not be achieved for 4–6 months. A small number of patients may worsen on clonidine. Guanfacine (brand name Tenex) is another antihypertensive that is used in treating TS. Side effects can include sedation, dry mouth, fatigue, headaches and dizziness. Sedation can be problematic when treatment is first initiated, but may wear off as the patient adjusts to the medication.

Patients with Tourette's who are referred to specialty clinics have a high rate of comorbid attention deficit hyperactivity disorder (ADHD), so the treatment of ADHD co occurring with tics is often part of the clinical treatment of Tourette's. Patients who have ADHD along with Tourette's may also have problems with disruptive behaviors, overall functioning, and cognitive function, accounted for by the comorbid ADHD, highlighting the importance of identifying and treating other conditions when present. Stimulants and other medications may be useful in treating ADHD when it co occurs with tic disorders. Drugs from several other classes of medications can be used when stimulants fail. however, multiple lines of research have shown that stimulants can be cautiously used in the presence of tic disorders. Several studies have shown that stimulants do not exacerbate tics any more than placebo does, and suggest that stimulants may even reduce tic severity. Controversy remains, and the PDR continues to carry a warning that stimulants should not be used in the presence of tic disorders, so physicians may be reluctant to use them. Others are comfortable using them and even advocate for a stimulant trial when ADHD co occurs with tics, because the symptoms of ADHD can be more impairing than tics.

Complementary and alternative medicine approaches, such as dietary modification, neurofeedback and allergy testing and control have popular appeal, but they have no proven benefit in the management of Tourette syndrome. Despite this lack of evidence, up to two thirds of parents, caregivers and individuals with TS use dietary approaches and alternative treatments and do not always inform their physicians. According to Müller Vahl (2013), medical professionals "should feel obliged to inform their patients about not only effective but also ineffective treatments". no particular diet or alternative therapy (vitamin or diet) is supported by scientific evidence. although it is an experimental treatment. There is low quality, limited evidence that DBS is safe, well tolerated, and yields a reduction of symptoms ranging from no change to complete remission. The ideal brain location to target has not been identified as of 2019. DBS has been used to treat adults with severe Tourette's that does not respond to conventional treatment. Viswanathan A et al (2012) say that DBS should be used in patients with "severe functional impairment that can not be managed medically".

A quarter of women report that their tics increase before menstruation, however studies have not shown consistent evidence of a change in frequency or severity of tics related to pregnancy. Overall, symptoms in women respond better to haloperidol than they do for men, Most women find they can withdraw from medication during pregnancy without much trouble.

In 2019, the American Academy of Neurology (AAN) published practice guidelines, "Treatment of tics in people with Tourette syndrome and chronic tic disorders", including 46 recommendations based on a systematic review by nine physicians, two psychologists, and two patient representatives. The panel assigned three levels of recommendations corresponding to the strength of the evidence supporting the recommendation:
A: "rare because they are based on high confidence in the evidence and require both a high magnitude of benefit and low risk". B: "common because the requirements are less stringent but still based on the evidence and benefit–risk profile". C: "lowest allowable recommendation level that the AAN considers useful within the scope of clinical practice and accommodates the highest degree of practice variation". The panel attached a helping verb to each level of recommendation: A = must; B = should, and C = may. DescriptionRecommendation per American Academy of Neurology 2019 practice guidelinesClinicians A: Must B: Should C: May Counseling Inform individuals and caregivers about the natural course of tic disorders Counseling Evaluate tic related impairment in functioning Counseling Inform about watchful waiting for those who do not experience impairment Counseling Initially prescribe Comprehensive Behavioral Intervention for Tics (CBIT) for those who are motivated and without functional impairment Counseling Periodically re evaluate need for any prescribed medications for tics Psychoeducation Refer teachers and peers to resources for education about TS ADHD assessment and management Assess for comorbid ADHD ADHD assessment and management Evaluate impairment from symptoms of ADHD ADHD assessment and management Ensure ADHD is treated when it is causing impairment OCD assessment and management Assess for comorbid OCD OCD assessment and management Ensure OCD is treated when it is present Other comorbid disorders Screen for comorbid anxiety, mood, and disruptive behavior disorders Other comorbid disorders Inquire about suicidal ideation and recommend resources if present Tic severity assessmentMeasure severity of tics using a validated assessment scale Treatment expectations Inform that treating tics rarely leads to complete cessation of tics Behavioral treatmentsFor those who have access to it, prescribe CBIT initially relative to other behavioral interventions Behavioral treatmentsOffer CBIT initially relative to medication Behavioral treatments If face to face CBIT is not available, prescribe CBIT via Internet, or prescribe other behavioral interventions α agonist treatment Inform individuals with comorbid ADHD that α2 agonists may treat both tics and ADHD α agonist treatment Prescribe α2 agonists when benefits outweigh risks α agonist treatment Inform individuals treated about side effects of α2 agonists α agonist treatmentIn those treated with α2 agonists, monitor heart rate and blood pressure α agonist treatment For those taking extended release guanfacine, monitor QTc interval as indicated α agonist treatment Gradually taper α2 agonists when discontinuing them Antipsychotic treatmentPrescribe antipsychotics when benefit outweighs risks Antipsychotic treatmentInform patients about adverse effects (extrapyramidal, hormonal, and metabolic) of antipsychotics Antipsychotic treatmentPrescribe lowest effective dosage of antipsychotics when using them Antipsychotic treatment When using antipsychotics, use evidence based monitoring for drug induced movement disorders and adverse effects Antipsychotic treatment When prescribing certain antipsychotics, monitor QTc interval and perform elecrocardiography Antipsychotic treatment Gradually taper (over weeks to months) antipsychotics when discontinuing Botulinum toxin injections Prescribe botulinum toxin injections for localized simple motor tics to adolescents and adults when benefits outweigh risks Botulinum toxin injections Prescribe botulinum toxin injections for aggressive or disabling vocal tics to adolescents and adults when benefits outweigh risks Botulinum toxin injections Inform individuals that temporary effects of botulinum toxin injections of hypophonia and weakness may occur Topiramate treatmentPrescribe topiramate when benefits outweigh risks Topiramate treatment Inform individuals when prescribing topiramate of adverse effects Cannabis based treatmentWhen individuals are using cannabis as self medication for tics, direct them to appropriate medical supervision Cannabis based treatmentFor "treatment resistant adults with clinically relevant tics", consider cannabis based products where legislation permits it. Cannabis based treatmentFor adults who already self medicate tics with cannabis based products, consider cannabis based medication where legislation permits it. Cannabis based treatmentWhen prescribing cannabis products where legislation permits it, use lowest effective dose Cannabis based treatmentWhen prescribing, inform individuals that cannabis based products can affect driving Cannabis based treatmentWhen prescribing, provide ongoing re evaluation of need Deep brain stimulation treatmentEmploy multidisciplinary evaluation of benefits versus risks Deep brain stimulation treatmentExclude secondary causes of tic like movements and confirm TS diagnosis when considering deep brain stimulation Deep brain stimulation treatmentScreen for psychiatric disorders and follow deep brain stimulation subjects post operatively Deep brain stimulation treatmentBefore prescribing, assure that multiple classes of medications have been tried Deep brain stimulation treatmentConsider deep brain stimulation for "severe, self injurious tics"