NIHR Signal Drug and behavioural therapy are both effective for tics in people with Tourette syndrome

Published on 15 March 2016

There are effective drug and behavioural treatments for tics in children and young people with Tourette syndrome. Although most of the studies included in this review were small with risk of bias, the effects found were moderate to large and likely to be clinically meaningful.

This NIHR funded systematic review and qualitative study found that habit reversal therapy, where people with Tourette’s are taught to replace their tic with a less noticeable alternative, was effective. The drugs with the best balance of benefits and harms – risperidone, clonidine and aripiprazole – are those most commonly used in the UK.

The qualitative review suggested that there is room for improvement in the management of Tourette syndrome in the NHS. Access to care and behavioural treatments are patchy, there are long delays in diagnosis, care pathways are often absent, and there is a lack of integration in the commissioning and provision of services. This comprehensive study offers suggestions on how care could be improved.

Share your views on the research.

Why was this study needed?

Tourette syndrome is a neurological condition characterised by a combination of involuntary noises and movements called tics. It usually starts during childhood and continues into adulthood. Vocal tics are sounds and motor tics are body movements. Children and adolescents with Tourette syndrome may also have one or more other behavioural conditions such as obsessive-compulsive disorder, autistic spectrum disorder or attention deficit hyperactivity disorder.

Tourette syndrome is common, affecting about one in every 100 children and young people. Treatment can be with drugs and/or behavioural therapies. Service provision in the NHS is variable, depending on the configuration of local services.

This study reviewed the effectiveness of treatments for tics and the experience of young people with Tourette’s and their parents when being cared for in the NHS.

What did this study do?

The study had two main components: 1) a systematic review of tic treatment trials; and 2) a qualitative study of patient and parent experience.

Systematic review: 70 studies, including randomised controlled trials and controlled before-and-after studies, of drug and behavioural interventions in children and young people (up to 18 years of age) with Tourette syndrome or chronic tic disorder were included. Meta-analyses were performed where possible. Most of the studies were relatively small and the quality of the evidence on the whole was low.

Qualitative study: Four qualitative studies were reviewed. In addition 295 parents or carers contributed to a survey via the Tourette’s Action website, and 40 people with Tourette syndrome, recruited via a national survey and specialist UK Tourette’s clinics, were interviewed.

What did it find?

  • Antipsychotic and noradrenergic drugs were both effective in treating tics in children and young people with Tourette syndrome. The effect sizes were moderate to large, and probably clinically meaningful. Compared to placebo antipsychotic drugs reduced tics (standardised mean difference [SMD] -0.74, 95% confidence interval [CI] -1.08 to -0.41) amongst 75 people studied. Noradrenergic drugs reduced tics (SMD -0.72, 95% CI -1.03 to -0.40) amongst 164 people studied.
  • There is no evidence that the effectiveness of the drugs is modified by either tic severity or other existing illness. The authors judged that the balance of benefits and harms favoured the most commonly used medications: antipsychotics risperidone and aripiprazole, and the noradrenergic drug clonidine. In surveys, parents perceived aripiprazole as being the most helpful drug with the least troublesome adverse effects.
  • Habit reversal therapy was similarly effective (SMD -0.64, 95% CI -0.99 to -0.29) amongst 133 people studied. This behavioural therapy teaches people to be aware of tic urges and replace the tic with another, less noticeable response. There is some preliminary evidence that delivering the therapy via video consultation may be as effective as face-to-face.
  • Key themes that emerged from the qualitative review were: difficulties in accessing specialist care and behavioural interventions; delay in diagnosis; importance of anxiety and emotional symptoms; lack of provision of information to schools, and inadequate information regarding medication and adverse effects.

What does current guidance say on this issue?

NICE have not published guidelines on Tourette syndrome. Many of the treatments are used off-label, meaning that tics are not their licensed indication.

The European clinical guidelines for Tourette syndrome recommend behavioural therapy as the first-line treatment option for most people, with habit reversal training being the recommended first choice behavioural therapy. This may be combined with drug treatment. Risperidone is recommended as first-line drug treatment, though they note that its use is limited by adverse reactions. The guidelines suggest that aripiprazole is useful in treatment-resistant cases, and that clonidine can be given especially when coexisting attention deficit hyperactivity disorder is present.

What are the implications?

This was a very comprehensive review, covering a wide range of interventions. There are some limitations. The number and quality of clinical trials was low, and the qualitative study cannot provide reliable evidence of benefit or lack of harm because there was no dosing information and no control group.

Nevertheless, the review’s findings contain some important messages.

First, access to behavioural interventions is limited. The survey found that only 25% of young people had a behavioural intervention. Future work could look into widening access through the use of mobile phone apps and video consultation. In practice, it appears that the primary intervention from paediatricians is often a trial of medication for tics – the choice of drug used is highly variable.

Second, there is a long delay in diagnosis and access to treatment. The average length of delay in diagnosis of Tourette syndrome was three years from symptom onset.

Third, clear care pathways are often absent. Care pathways for suspected Tourette’s need to be established to improve access to assessment and diagnosis.

Fourth, there is a lack of integration in the commissioning and provision of physical and mental health services. The authors suggest that Local Child and Adolescent Mental Health Services should provide assessment and treatment, and offer referral and support for patients who need to access specialist centres.

Citation and Funding

Hollis C, Pennant M, Cuenca J, et al. Clinical effectiveness and patient perspectives of different treatment strategies for tics in children and adolescents with Tourette syndrome: a systematic review and qualitative analysis. Health Technol Assess. 2016;20(4):1-450.

This project was funded by the National Institute for Health Research HTA programme (project number 10/142/01).

Bibliography

NHS Choices. Tourette’s syndrome. London: NHS Choices; 2015.

Roessner V, Plessen KJ, Rothenberger A, et al. European clinical guidelines for Tourette syndrome and other tic disorders. Part II: pharmacological treatment. Eur Child Adolesc Psychiatry. 2011;20(4):173-96.

Verdellen C, van de Griendt J, Hartmann A, et al. 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.

Why was this study needed?

Tourette syndrome is a neurological condition characterised by a combination of involuntary noises and movements called tics. It usually starts during childhood and continues into adulthood. Vocal tics are sounds and motor tics are body movements. Children and adolescents with Tourette syndrome may also have one or more other behavioural conditions such as obsessive-compulsive disorder, autistic spectrum disorder or attention deficit hyperactivity disorder.

Tourette syndrome is common, affecting about one in every 100 children and young people. Treatment can be with drugs and/or behavioural therapies. Service provision in the NHS is variable, depending on the configuration of local services.

This study reviewed the effectiveness of treatments for tics and the experience of young people with Tourette’s and their parents when being cared for in the NHS.

What did this study do?

The study had two main components: 1) a systematic review of tic treatment trials; and 2) a qualitative study of patient and parent experience.

Systematic review: 70 studies, including randomised controlled trials and controlled before-and-after studies, of drug and behavioural interventions in children and young people (up to 18 years of age) with Tourette syndrome or chronic tic disorder were included. Meta-analyses were performed where possible. Most of the studies were relatively small and the quality of the evidence on the whole was low.

Qualitative study: Four qualitative studies were reviewed. In addition 295 parents or carers contributed to a survey via the Tourette’s Action website, and 40 people with Tourette syndrome, recruited via a national survey and specialist UK Tourette’s clinics, were interviewed.

What did it find?

  • Antipsychotic and noradrenergic drugs were both effective in treating tics in children and young people with Tourette syndrome. The effect sizes were moderate to large, and probably clinically meaningful. Compared to placebo antipsychotic drugs reduced tics (standardised mean difference [SMD] -0.74, 95% confidence interval [CI] -1.08 to -0.41) amongst 75 people studied. Noradrenergic drugs reduced tics (SMD -0.72, 95% CI -1.03 to -0.40) amongst 164 people studied.
  • There is no evidence that the effectiveness of the drugs is modified by either tic severity or other existing illness. The authors judged that the balance of benefits and harms favoured the most commonly used medications: antipsychotics risperidone and aripiprazole, and the noradrenergic drug clonidine. In surveys, parents perceived aripiprazole as being the most helpful drug with the least troublesome adverse effects.
  • Habit reversal therapy was similarly effective (SMD -0.64, 95% CI -0.99 to -0.29) amongst 133 people studied. This behavioural therapy teaches people to be aware of tic urges and replace the tic with another, less noticeable response. There is some preliminary evidence that delivering the therapy via video consultation may be as effective as face-to-face.
  • Key themes that emerged from the qualitative review were: difficulties in accessing specialist care and behavioural interventions; delay in diagnosis; importance of anxiety and emotional symptoms; lack of provision of information to schools, and inadequate information regarding medication and adverse effects.

What does current guidance say on this issue?

NICE have not published guidelines on Tourette syndrome. Many of the treatments are used off-label, meaning that tics are not their licensed indication.

The European clinical guidelines for Tourette syndrome recommend behavioural therapy as the first-line treatment option for most people, with habit reversal training being the recommended first choice behavioural therapy. This may be combined with drug treatment. Risperidone is recommended as first-line drug treatment, though they note that its use is limited by adverse reactions. The guidelines suggest that aripiprazole is useful in treatment-resistant cases, and that clonidine can be given especially when coexisting attention deficit hyperactivity disorder is present.

What are the implications?

This was a very comprehensive review, covering a wide range of interventions. There are some limitations. The number and quality of clinical trials was low, and the qualitative study cannot provide reliable evidence of benefit or lack of harm because there was no dosing information and no control group.

Nevertheless, the review’s findings contain some important messages.

First, access to behavioural interventions is limited. The survey found that only 25% of young people had a behavioural intervention. Future work could look into widening access through the use of mobile phone apps and video consultation. In practice, it appears that the primary intervention from paediatricians is often a trial of medication for tics – the choice of drug used is highly variable.

Second, there is a long delay in diagnosis and access to treatment. The average length of delay in diagnosis of Tourette syndrome was three years from symptom onset.

Third, clear care pathways are often absent. Care pathways for suspected Tourette’s need to be established to improve access to assessment and diagnosis.

Fourth, there is a lack of integration in the commissioning and provision of physical and mental health services. The authors suggest that Local Child and Adolescent Mental Health Services should provide assessment and treatment, and offer referral and support for patients who need to access specialist centres.

Citation and Funding

Hollis C, Pennant M, Cuenca J, et al. Clinical effectiveness and patient perspectives of different treatment strategies for tics in children and adolescents with Tourette syndrome: a systematic review and qualitative analysis. Health Technol Assess. 2016;20(4):1-450.

This project was funded by the National Institute for Health Research HTA programme (project number 10/142/01).

Bibliography

NHS Choices. Tourette’s syndrome. London: NHS Choices; 2015.

Roessner V, Plessen KJ, Rothenberger A, et al. European clinical guidelines for Tourette syndrome and other tic disorders. Part II: pharmacological treatment. Eur Child Adolesc Psychiatry. 2011;20(4):173-96.

Verdellen C, van de Griendt J, Hartmann A, et al. 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.

Clinical effectiveness and patient perspectives of different treatment strategies for tics in children and adolescents with Tourette syndrome: a systematic review and qualitative analysis

Published on 21 January 2016

Hollis, C.,Pennant, M.,Cuenca, J.,Glazebrook, C.,Kendall, T.,Whittington, C.,Stockton, S.,Larsson, L.,Bunton, P.,Dobson, S.,Groom, M.,Hedderly, T.,Heyman, I.,Jackson, G. M.,Jackson, S.,Murphy, T.,Rickards, H.,Robertson, M.,Stern, J.

Health Technol Assess Volume 20 , 2016

BACKGROUND: Tourette syndrome (TS) is a neurodevelopmental condition characterised by chronic motor and vocal tics affecting up to 1% of school-age children and young people and is associated with significant distress and psychosocial impairment. OBJECTIVE: To conduct a systematic review of the benefits and risks of pharmacological, behavioural and physical interventions for tics in children and young people with TS (part 1) and to explore the experience of treatment and services from the perspective of young people with TS and their parents (part 2). DATA SOURCES: For the systematic reviews (parts 1 and 2), mainstream bibliographic databases, The Cochrane Library, education, social care and grey literature databases were searched using subject headings and text words for tic* and Tourette* from database inception to January 2013. REVIEW/RESEARCH METHODS: For part 1, randomised controlled trials and controlled before-and-after studies of pharmacological, behavioural or physical interventions in children or young people (aged < 18 years) with TS or chronic tic disorder were included. Mixed studies and studies in adults were considered as supporting evidence. Risk of bias associated with each study was evaluated using the Cochrane tool. When there was sufficient data, random-effects meta-analysis was used to synthesize the evidence and the quality of evidence for each outcome was assessed using the Grading of Recommendations Assessment, Development and Evaluation approach. For part 2, qualitative studies and survey literature conducted in populations of children/young people with TS or their carers or in health professionals with experience of treating TS were included in the qualitative review. Results were synthesized narratively. In addition, a national parent/carer survey was conducted via the Tourettes Action website. Participants included parents of children and young people with TS aged under 18 years. Participants (young people with TS aged 10-17 years) for the in-depth interviews were recruited via a national survey and specialist Tourettes clinics in the UK. RESULTS: For part 1, 70 studies were included in the quantitative systematic review. The evidence suggested that for treating tics in children and young people with TS, antipsychotic drugs [standardised mean difference (SMD) -0.74, 95% confidence interval (CI) -1.08 to -0.41; n = 75] and noradrenergic agents [clonidine (Dixarit((R)), Boehringer Ingelheim) and guanfacine: SMD -0.72, 95% CI -1.03 to -0.40; n = 164] are effective in the short term. There was little difference among antipsychotics in terms of benefits, but adverse effect profiles do differ. Habit reversal training (HRT)/comprehensive behavioural intervention for tics (CBIT) was also shown to be effective (SMD -0.64, 95% CI -0.99 to -0.29; n = 133). For part 2, 295 parents/carers of children and young people with TS contributed useable survey data. Forty young people with TS participated in in-depth interviews. Four studies were in the qualitative review. Key themes were difficulties in accessing specialist care and behavioural interventions, delay in diagnosis, importance of anxiety and emotional symptoms, lack of provision of information to schools and inadequate information regarding medication and adverse effects. LIMITATIONS: The number and quality of clinical trials is low and this downgrades the strength of the evidence and conclusions. CONCLUSIONS: Antipsychotics, noradrenergic agents and HRT/CBIT are effective in reducing tics in children and young people with TS. The balance of benefits and harms favours the most commonly used medications: risperidone (Risperdal((R)), Janssen), clonidine and aripiprazole (Abilify((R)), Otsuka). Larger and better-conducted trials addressing important clinical uncertainties are required. Further research is needed into widening access to behavioural interventions through use of technology including mobile applications ('apps') and video consultation. STUDY REGISTRATION: This study is registered as PROSPERO CRD42012002059. FUNDING: The National Institute for Health Research Health Technology Assessment programme.

Habit reversal is a type of behavioural therapy that is used in treating Tourette’s syndrome. It's based on the following two principles: 1) people with Tourette’s syndrome are often unaware of their tics, and 2) tics are used to relieve uncomfortable feelings, called premonitory sensations, that build up before tics.

The first stage of the therapy is to monitor the pattern and frequency of the tics, and identify any sensations that are triggering them. The second stage is to find an alternative, less noticeable method of relieving the premonitory sensations instead of a tic. This is known as a competing response.

Habit reversal therapy is sometimes combined with relaxation therapy.

Expert commentary

This review is an excellent portrait of the patient pathway and the difficulties children face in achieving first of all a diagnosis of Tourette syndrome; recognition of co-morbidities and then treatment and support to the child and family in primary care and secondary care in the UK.

I have been doing a Specialist Tics and Tourette Clinic for the last six years. I fully agree with the recommendations made by the qualitative part (part two) of the study which says that improved access to assessment and diagnosis is required. I hope that this review helps children with Tourette syndrome to get holistic care all over the UK.

Dr Santosh R Mordekar, Consultant Paediatric Neurologist, Sheffield Children's NHS Foundation Trust