Tic disorders are neurodevelopmental conditions characterised by fast, irregular and repetitive muscle movements that can manifest in any part of the body. Tics that affect body movements (e.g. blinking, grimacing, head jerking, head banging, finger clicking) are known as motor tics, while involuntary repetitive sounds, such as grunting, sniffing or throat clearing, are known as vocal or phonic tics. Tic disorders manifest more often in boys than girls with a ratio between 3 : 1 and 4 : 1.1–4 There are several types of tic disorders according to their manifestation and frequency. Transient or provisional tic disorders refer to single or multiple motor and/or vocal tics that have been present for < 12 months since the first tic onset. Persistent or chronic tic disorder (CTD) refer to single or multiple motor or vocal tics (but not both) that have persisted for more than 12 months since the first tic onset. Tourette syndrome (TS) refers to multiple motor tics and one or more vocal tics that have been present at the same time (but not necessarily concurrently) during the course of the disease and have persisted for more than 12 months since the first tic onset. In all cases, onset is before the age of 18 years, and the tics are not attributable to the physiological effects of a substance (e.g. cocaine) or other medical conditions (e.g. Huntington’s disease, post-viral encephalitis).5
The mean age of onset for tic disorders is approximately 5 years, although it can be lower in up to 40% of patients.4,6 Typically, the severity of tic disorders worsens between 10 and 12 years of age and improves naturally during adolescence and early adulthood.7,8 In children and young people, tics tend to come and go, while in adults, they show a more persistent pattern.9 Psychiatric comorbidities are common among people who suffer from CTDs.10 People with TS or CTDs often experience associated psychiatric conditions, such as attention deficit hyperactivity disorder (ADHD) (30–54% of people) and obsessive–compulsive disorder (OCD) (10–50% of people).11 Other common comorbidities which are highly associated with comorbid OCD and ADHD in people with CTDs include mood disorders, disruptive behaviour and anxiety (30% of people).6,11 Comorbid mood disorders tend to be observed more frequently in adolescents and adults than in children.12 Independent of ADHD and OCD comorbidities, TS has also been reported to be associated with an increased risk of anxiety.6
Most prevalence studies of TS have focused specifically on children and young people, with few or less reliable data on adults. Internationally, the prevalence of TS in young people in the community has been reported to be between 0.4% and 3.8%.2 In the UK, TS is identified in 1 per 100 school children.13 A meta-analysis published in 2012 reported a pooled prevalence rate of 0.77% [95% confidence interval (CI) 0.39% to 1.51%] in children (13 studies) and of 0.05% (95% CI 0.03% to 0.08%) in adults (2 studies).14 A more recent meta-analysis suggested that the adulthood prevalence of TS is around 118 cases per million adults (95% CI 19 to 751 cases per million adults) but with considerable heterogeneity between prevalence studies.15
Tic disorders can vary in severity and impact various aspects of people’s lives, contributing to a reduced quality of life (QoL). It is not uncommon for people with tic disorders, particularly when the illness is more severe, to experience serious social issues, such as extensive stigma, public avoidance and discrimination.10,16 Severe long-lasting tic disorders are also associated with a fourfold increased risk of suicide.17
The clinical pathway, management and treatment options are the same for all tic disorders.
Current management and clinical pathway
At present, in the UK, there are specific national guidelines for the assessment, management and referral of neurodevelopmental conditions such as AHDH and autism.18–20 However, a comprehensive clinical guideline for the diagnosis and management of tic disorders in children and young people does not exist. The National Institute for Health and Care Excellence (NICE) guideline 127 on ‘Suspected neurological conditions: recognition and referral’ contains some information on tic disorders and indicates that (1) children or young people with tic disorders, that significantly interfere with their ability to function in their daily lives, should be referred to specialist mental health services, neurodevelopmental teams or for neurological assessment; (2) adults with tic disorders should be considered for neurological assessment if their symptoms are severe and the disorder continues to cause distress.21 Current international guidelines and recommendations include the European clinical guidelines for Tourette and other tic disorders, the Canadian guidelines for the evidence-based treatment of tic disorders, practice guideline recommendations summary for TS and CTDs from the American Academy of Neurology, and BMJ Best Practice Tic disorders.12,13,22–24
Symptoms of tic disorders may be reported by people themselves or for children or young people identified by their parents/carers or school educators. In the UK, people with tic disorders attend an initial appointment with a general practitioner working in primary care. When the presence of a tic disorder is recognised to have a significant impact on people’s QoL, a referral is usually made to appropriate secondary or tertiary care services (depending on the presentation, comorbidities, and local specialist clinics).17,24
For children and young people, referrals may be made to mental health services [including the Children and Young People’s Mental Health Services (CYPMHS)] neurodevelopmental teams, paediatric or neurology teams dependent on local services. For adults, referrals are usually made to neurological services.
As tics may improve with time, the NICE guideline 127 indicates that for individuals presenting in primary care a watch-and-wait approach is considered acceptable, especially for those who do not experience any functional impairment.21
Current practice varies between countries and according to the availability of local services but, in general, treatment options for CTDs include psychoeducation, behavioural therapy, pharmacological therapy, and deep brain stimulation (DBS).
Psychoeducation for patients, their families, teachers, and peers, which aims to reduce stigma and distress and increase awareness of the illness, is regarded as the initial approach to treating all tic disorders. This includes information on the natural waxing and waning course of the disorder, which is favourable in most cases, on what can worsen tics, such as stress, anxiety and excitement, and on the importance of avoiding focusing on the presence of tics. An assessment of concomitant psychiatric and mood disorders [e.g. ADHD, OCD, autism spectrum disorder (ASD), anxiety] should also be considered, as these may further aggravate the patients’ emotional, behavioural and social functioning.13 In many cases, people with tic disorders may not require further treatment aside from psychoeducation and observation (watch and wait approach).24
However, it has been reported that in the UK psychoeducation is rarely provided by general practitioners in the first appointment, and many people with tic disorders do not receive advice on how to manage their tics or information on treatment options.17,24
Current international guidelines recommend the use of behavioural therapy as the first-line intervention for tic disorders in both children and young people and adults.12,22,23,25 The behavioural approaches with more robust evidence of efficacy are habit reversal training (HRT), comprehensive behavioural intervention for tics (CBIT) and the efficacy of exposure and response prevention (ERP).12 With HRT the patient is trained to perform a voluntary movement, which is physically incompatible with the performance of the tic until the urge (unpleasant internal stimulus) to perform the tic goes away. The CBIT utilises the same components of HRT alongside relaxation training and functional interventions to tackle factors that may provoke or exacerbate tics.24 The ERP aims to break the association between the urge and the tic by asking the patient to suppress the tics for prolonged periods using various cognitive tools.23 However, due to a shortage of trained therapists, behavioural therapy is only available in a small number of specialist centres, and only about 20% of people with tic disorders have access to it.17 Therefore, digitally enabled interventions have the potential to improve access as well as equity of access to treatment for people with tic disorders.
Concerning pharmacological therapy, there is some evidence that α2-adrenergic receptor agonists (e.g. clonidine, guanfacine) and antipsychotic drugs (e.g. risperidone, haloperidol) are effective in the short term.26–28 Antipsychotic drugs, due to their adverse effect profile, are mostly considered for the treatment of severe tics when α2-adrenergic receptor agonists are not effective or not tolerated. The decision about the type and dosage of pharmacological therapy should be provided by a health professional with experience in the management of tic disorders after taking into consideration the presence of comorbidities, which may affect the patient’s treatment response.24
Deep brain stimulation in specialised centres has been proposed for patients with severe tics that are refractory to behavioural and pharmacological interventions.12,13 There is, however, little information on the effects of DBS in children and young people with CTDs to support its use in clinical practice.23,24 The largest DBS randomised cross-over trial published in 2015 indicated some possible benefits for adults with TS but also highlighted several methodological challenges in the design of brain stimulation studies.12,29,30 Similarly, the prospective International DBS Database and Registry published in 2018, which included 185 patients with medically refractory TS who underwent DBS implantation, showed that DBS was associated with improvement in patients’ symptoms but also with important adverse events (AEs).31
Alternative treatments such as dietary supplements, fish oils, acupuncture and antibiotics have also been proposed for tic disorders, but the rationale and evidence of their efficacy is still unclear or insufficient.24
Novel treatment options such as median nerve stimulation (MNS) are currently under investigation. Results from a recent open-label comparative study assessing 27 people (15–64 years of age) with CTDs suggest that MNS may improve the frequency and intensity of tics with minimal side effects.24,32
Description of the technologies of interest
The technologies considered for this appraisal are digital technologies that enable the remote/online delivery of therapeutic intervention to people with CTDs or TS. These interventions should only be considered provided the person (and parent or carer where appropriate) have had access to a form of psychoeducation. If the tic disorder continues to cause difficulties, a clinician may consider referring people for these proposed interventions. These technologies should have received or are likely to receive appropriate regulatory approval (e.g. Conformité Européenne or European Conformity [CE] mark/UK Conformity Assessed [UKCA] mark and Digital Technology Assessment Criteria [DTAC] compliance), should be available or likely to be soon available to the NHS and should have online guided contact with a practitioner as part of the programme, or clinician oversight with the intervention for user safety. We have identified two digitally enabled technologies (Table 1) for the treatment of people with CTDs: Online Remote Behavioural Treatment for Tics (ORBIT) and Neupulse.24
| Device name | ORBIT (MindTech) | Neupulse (neurotherapeutics) clinical trial | Neupulse (neurotherapeutics) current medical device |
|---|---|---|---|
| Platform | Delivered remotely via the BiP (Barninternetprokektet, Swedish for Child Internet Project; www.bup.se/BIP/) technical platform, a Swedish web-based platform specifically designed for use by children and their parents with an age-appropriate appearance, animations and interactive scripts. The platform can be accessed via the internet using a smartphone, desktop computer or laptop | Wrist-worn stimulation device designed and approved for a home use trial by the Medicines and Healthcare products Regulatory Agency | Wrist-worn wearable device with hydrogel pad to provide MNS. An accompanying mobile phone app is used to setup and adjust the device |
| Type of behavioural therapy | Exposure with response prevention (ERP). ERP aims to break the urge-tic-relief cycle of reinforcement while promoting tolerance of premonitory urges and tic suppression. The intervention is delivered in 10 chapters split into child intervention and parent/supporter intervention: Learn about tics/introductionMore about tics/thoughts and behaviours of supportersPractising stopping your tics/praiseMaking the practice more challenging/promptsContinued practice/situations and reactionsSchool/troubleshootingTalk about your tics/continued practiceContinued practice/continued practiceThe final sprint/continued practicePlan for the future/plan for the future | Neuromodulation of the brain’s sensorimotor networks using low intensity (1–19 mA) 10 Hz TENS delivered to the median nerve (MNS) | TENS to the MNS at a frequency of 10 Hz with intensity levels (1–14 mA) adjustable via a mobile phone app |
| Aim of therapy | ERP aims to break the urge-tic-relief cycle of reinforcement while promoting tolerance of premonitory urges and tic suppression | Immediate reduction in tic frequency and complexity during device use and reduction of tic severity after 4 weeks of use | Immediate reduction of urge to tic and tic intensity |
| Duration | 10 weeks | The therapy aims to address the imbalances in neural activity which are associated with tics and premonitory urges by modulating neural oscillations within the brain’s sensorimotor networks. The trial device was pre-programmed to deliver once a day rhythmic trains of low intensity electrical stimulation in bursts of 2 minutes of stimulation followed by 1 minute of no stimulation for 14 minutes. Users were instructed to use the device at home for 5 days a week for a month | Intermittent on demand (1 hour session) up to 8 hours depending on battery lifeStimulation can be activated “on demand” by users with a maximum individual session time of 1 hour. Session parameters can be adjusted via the app. The default session provides burst stimulation comprising 2 minutes of stimulation followed by 1 minute of no stimulation |
| Contact with therapist | Remote contact; at least once a week via messages sent inside the treatment platform (resembling an e-mail). The therapist’s role is to encourage uptake and adherence to the programme plus troubleshooting and technical support rather than delivering therapeutic content | Remote video: Weekly contact. The therapist completed weekly clinical assessments | Over the counter – no prescription required. No contact with the therapist is required.App can collate symptom monitoring data for review by an HCPTechnical support availableChange to the pathway is required – as people with TS find it hard to access HCPs |
The ORBIT (MindTech) is an online therapeutic intervention which aims to reduce tic severity in children and young people with tic disorders. The ORBIT treatment programme was developed from an existing research platform Barninternetprojektet (Child Internet Project; Swedish digital platform) in Sweden, which was designed to be age-appropriate in appearance for use by children and their parents and included animations and interactive scripts. The platform has been used to deliver internet-based therapy for conditions such as phobia, anxiety and OCD. ORBIT provides a form of behavioural therapy called exposure and response prevention, which is supported by an online therapist across a 10-week programme. It is delivered on a secure internet platform and includes 10 self-help guided chapters followed by ERP tasks. Through the ORBIT programme, patients practise controlling their tics for increasingly long periods and then deliberately provoke urges while not releasing any tics. Related interventions are delivered to the patient’s parent/supporter on the same time scale. The therapist has 10–20 minutes of contact time with the family each week and promotes engagement with the intervention as well as answering any questions rather than delivering therapeutic content.33–35 ORBIT has been studied as part of NIHR-funded UK-based trials which have reported it to be a clinically and cost-effective intervention at up to 18 months.34,35 ORBIT does not require CE marking as it is not considered a medical device. At present, the investigators are working towards DTAC compliance.24
Neupulse (developed by Neurotherapeutics Ltd) is a wearable wrist-worn neuromodulation device with a corresponding phone app which provides a novel approach to reduce tic frequency and severity. The device addresses the imbalances in neural activity which are associated with tics and premonitory urges by modulating neural oscillations within the brain’s sensorimotor networks. No active effort is required of the user to reduce these symptoms (besides turning on the device), which delivers low-intensity electrical pulses to the median nerve (MNS). Currently, the device has been assessed for children and young adults aged 12 and over with suspected or diagnosed TS or a chronic (motor or vocal) tic disorder.24
Median nerve stimulation is a type of transcutaneous electrical nerve stimulation (TENS) delivered to the wrist. TENS has a long history of therapeutic use and TENS machines are widely available over the counter for use within the community. The Neupulse device produces low-intensity electrical stimulation up to a maximum of 14 mA. It has been shown within a UK double-blind sham control trial to reduce tic frequency when the stimulation is turned on and to be clinically effective with 4 weeks of once-a-day 14-minute use.36 The rhythmic pattern of medial nerve stimulation used by Neupulse has been shown to increase brain activity associated with movement suppression, without impairing intentional movement or cognitive function.36,37 The device currently under development for over-the-counter sale will be supported by written and video-based guidance and a technical support helpline. Neupulse is working towards CE and UKCA marking, and it is estimated that the device will be available in 2026.24
Population and relevant subgroups
The population of interest is people with a diagnosed primary tic disorder who have had access to psychoeducation; however, their tics continue to be bothersome to them.
Where data permit, the following subgroups were considered:
- Children and young people with diagnosed comorbidities, including ADHD, OCD, ASD, mood disorders and anxiety.
- Adults with CTDs.24
Aim and objectives
This assessment aims to establish whether digitally enabled therapy for people with CTDs and TS represents a clinically and cost-effective use of NHS resources.
The specific objectives are:
- to evaluate the safety and effectiveness of digitally enabled non-pharmacological therapy for treating CTDs and TS in UK clinical practice
- to develop an economic model to assess the cost-effectiveness of digitally enabled technologies for the non-pharmacological treatment of CTDs that are available or likely to become available in UK clinical practice.24