What is Tourette Syndrome?

Tourette Syndrome (TS) is a neurological condition that is estimated to effect over 300,000 children and adults in the UK; and one school child in every hundred. As with other neurological conditions, TS is more prevalent in boys – it has been estimated that there is a prevalence of 0.7% from community—based research (Scharf et al, 2012). The main symptoms are physical and vocal tics, which typically develop in childhood.  Tics can be simple such as eye blinking, sniffing and throat clearing; through to complex movements and vocalisations (such as shouting phrases, skipping and throwing) that can, at the extreme end, be incredibly debilitating. Symptoms fluctuate; this could be across a period of hours, days, weeks or months. Some people find they have periods when they do not tic at all. Environmental factors such as stress and excitement can also affect frequency and severity of tics, and it is noticed that tics tend to peak between 10-13 years of age with a reduction for most individuals as they mature towards early adulthood (Groth et al, 2017).

Up to 85 percent of people with TS will have additional symptoms to tics. Co-occurring conditions may include ADHD, OCD, Anxiety, as well as depression, sleep problems and sensory processing difficulties. The co-morbidities rather than the tics themselves mean that children with TS and co-occurring conditions may be treated in CAMHS.  These additional features and conditions can, for some people, be more challenging to live with than tics.

About two-thirds of children with tic disorders will meet the criteria for ADHD, and about 20-30 per cent of children with ADHD also have a tic disorder.  About one-third of individuals with Tourette Syndrome experience recurrent obsessive compulsive symptoms (Groth et al, 2017).

Studies have shown higher anxiety symptoms in young people with Tourette Syndrome than those without TS. (Lewin et al. 2011)

Despite the prevalence of TS (1 in 100 school children) and its relationship to other neurodevelopmental conditions, there is currently – unlike ADHD, OCD and anxiety disorder – no NICE guidance on Tourette Syndrome.

A myth about TS is that it involves swearing (Coprolalia).  Coprolalia is found in a minority of people with TS and is not one of the diagnostic factors required for someone to be diagnosed with TS (Cavanna and Rickards, 2013). Other symptoms which can be present but are rare include – copropraxia (obscene gestures), echolalia (repeating the words said by other people) and echopraxia (repeating other people’s gestures).

It is thought that approximately half of children with a TS diagnosis will see a significant reduction in their symptoms as they approach adulthood. Some research findings back this up, where adults who had TS as children reported that most of them had far fewer if any tics in adulthood (Groth et al, 2017). For a significant minority of people, tics will continue throughout their life, but symptoms are likely to wax and wane and for many will not impact on their daily function.

Many people experience a physical sensation – a premonitory urge/tic signal – before they express a tic. It has been compared to other physical sensations such as the need to itch or sneeze (Kurlan, Lichter and Hewitt, 1989). Suppressing a tic can increase the premonitory urge, and once a tic has been performed the premonitory urge often reduces. For some people, multiple attempts of a tic are necessary until it ‘feels just right’ and the premonitory urge goes away.

Although tics are involuntary, many people are able to suppress their tics for a short time. A helpful way of understanding this is to compare it to blinking. For a short period of time it is possible to keep your eyes wide open and avoid blinking – and with practice you will get better at doing it for longer – but eventually you will have to blink as the urge is too strong to control. Suppressing tics works in the same way. It can take a great measure of concentration – especially to begin with – to resist the urge to tic, but with practice a certain level of control can be applied. Some people will be able to suppress their tics more easily than others.

Impact of living with TS

The impact of living with TS is variable as the condition is unique to each individual and ranges across a spectrum.  Aspects of life likely to be affected are:

Physical and Mental Health

Tics can be very painful and in some cases can cause serious harm or injury.  In a recent survey conducted by Tourettes Action in 2018, out of 118 participants 96% reported that they experienced pain due to their tics.

Out of 208 respondents, to another 2018 Tourettes Action survey about living with TS, 78% reported that TS has had an effect on their mental health.  With 35% saying that they have considered suicide and self-harming behaviour.  Research form the Karolinska institute in Sweden suggests a higher risk of suicide in people with TS (Fernandez de la Cruz, et al 2017)

Education

TS can affect concentration levels, and certain tics can have a direct impact on reading and writing.  Standing out from peers and being ‘different’ can be a source of anxiety, and young people with TS may fear being or be directly bullied because of their overt symptoms.  What has been found is that giving some sort of psychoeducation in schools where a pupil has TS can be very beneficial (Nussey, Pistrang and Murphy, 2013). Better understanding and more tolerance and teachers reporting they felt more confident were some of the results from this primary school study. Tourettes Action has many resources about supporting a child with TS in the classroom.

Socially

Perhaps one of the biggest challenges for people living with TS is managing the reactions of others. There is a lot of stigma attached to the condition, and it is still misunderstood as being ‘that thing where people swear’, and is often spoken of in derogatory terms.  Because of the unpredictable nature of TS, people may resist socialising; and therefore can feel ostracized from their community, and wider society. With loud or physically noticeable tics it can be difficult to sit in an audience at a concert or cinema, or in a library without attracting attention.

Causes

Although the exact cause has not been established, tic disorders appear to be an inherited developmental disorder affecting the central nervous system’ (Chowdhury and Murphy, 2017). There appear to be structural brain differences and changes in the way the brain functions in the circuits of the brain (Felling and Singer, 2011).

Although it is understood that TS is a genetic condition, the genetic cause of TS is complex as not one single gene has been identified to be the cause. Twin studies have suggested a shared genetic etiology (cause) between TS and OCD but the relationship between TS and ADHD and/or ASD is less clear.

Brain studies show that certain structures in the brain and spinal cord are associated with tics.  However, due to the variability of the severity and range of symptoms, and the way behaviour and brain activity and structure are measured, it is hard to make sense of research studies. (Chowdhury and Murphy, 2017)

Different environmental factors may, in some cases, contribute to the onset or affect the severity of TS. These include pregnancy and birth related problems such as complications during pregnancy, and premature low birth weight children.

Diagnosis and assessment

As it stands there is no NICE guidance on Tourette Syndrome.

For TS to be diagnosed, multiple motor tics and at least one vocal tic must be present for at least 12 months.  Symptoms usually present in children around the age of six years old, although formal diagnosis is not always immediate as the symptoms can go unidentified by both families and GPs and considered to be the result of allergies (e.g. sniffing) or a need for glasses (e.g. eye blinking).

TS can only be diagnosed by observing and evaluating symptoms and there are rating scales to help with the assessment of tic severity. Some doctors may use an EEG, MRI, CT scan, or certain blood tests to rule out other conditions that might be confused with TS such as epilepsy, autism, dystonia and Sydenham’s chorea.  However, these assessments are not needed in all diagnostic evaluation and not indicated in best practice guidelines

Medication

There is no specific drug that can stop all tics permanently. There are European Guidelines based on systematic reviews and an NIHR Health Technology Assessment which looked at a whole group of studies (Hollis, et al 2016). Medication might be able to reduce the intensity of frequency for some length of time. For children medication is helpful in reducing tics to a level that allows them to function in their everyday lives. The lowest effective dose should be used and medication should be carefully monitored, especially in relation to the waxing and waning of tics, frequency, severity and well-being and possible side effects (Chowdhury and Murphy, 2017).

Neuroleptic medication

Neuroleptics are a type of medication known as antipsychotics. They have been primarily used to manage psychosis, principally in schizophrenia and bipolar disorder. However, they have also been used in the management of non-psychotic disorders such as Tourette Syndrome. They block dopamine receptors in the basal ganglia in the brain, which reduces tics. Two such medications are Haloperidol and Pimozide, however Pimozide has been associated with cardiac effects and is rarely used in the UK for children. Side effects for Neuroleptic medication can include unpleasant side effects including sudden stiffness and prolonged muscle contraction (acute dystonia), shaking, restlessness and more commonly drowsiness. Other side effects include ‘weight gain, occasional worsened depression or anxiety’ (Stern, 2018).

Another class of medications are Benzamides which include Sulpride and Amisuplride. This medication results in fewer side effects than Haloperidol, but patients can still experience sedation, increased appetite and sometimes depression (Chowdhury and Murphy, 2017).

Atypical neuroleptics

This type of medication blocks the serotonin and dopamine receptors and has reduced side effects. These medications include Risperidone and Aripiprazole. Risperidone has been found to result in fewer side effects than Haloperidol, and useful in reducing aggressive behaviour and improving sleep but weight gain remains a problem side-effect.

Nonadrenergic medication

These include medications such as Clonodine and Guanfacine. These have often been used to reduce tics in children with tics and ADHD symptoms, but there are side effects including sedation, headache and blood pressure needs monitoring.

Psychological interventions

Recently, research has turned towards evaluating more broadly focused psychological treatments that attempt to equip children with the tools to live with Tourette Syndrome rather than just control their tics, although naturally, this may also be an important part of any treatment (Chowdhury and Murphy, 2017).  An intervention designed to take a wider focus to include tics, self-esteem, anxiety management, management of attention and hyperactivity challenges has been highlighted as being effective. (McGuire et al. 2015)

The strongest evidence base to date for psychological interventions uses behavioural treatments which primarily focus on tic management.

Habit Reversal Therapy (HRT)

The best scientifically validated behavioural treatment is Habit Reversal Therapy (HRT), which has been researched since the 1970s.

Comprehensive Behavioural Intervention for Tics (CBiT)

More recently, HRT has been shown to be effective as part of a package alongside other elements of therapy, known as Comprehensive Behavioural Intervention for Tics (CBIT). CBIT is made up of different elements which include: Psychoeducation, Functional Analysis, HRT, Social Support and Reward System and Relaxation Training. HRT is a major component of CBITS, CBITS training includes HRT. In the treatment the patient is made more aware of the premonitory urge and helped to implement a behaviour before the tics occur, to stop the tic -a competing response to block a tic.

Exposure and Response Prevention (ERP)

A fairly similar approach is exposure with response prevention (ERP). Patients practice suppressing their tics and don’t try and block them with a physical gesture, as is the technique used in ERP. The individual gets used to the uncomfortable sensation and discovers it goes away or they get used to it after a while. There is evidence to suggest that this approach is as effective as HRT. ERP may be best suited to people who have a range of very annoying tics or are of a young age.

Myths

There are some myths surrounding behavioural therapy interventions, which can be dispelled. ‘Holding tics in’ will not cause them ‘to all burst out’ later on; research has found holding tics in decreases tics generally. ‘Stopping one tic does not mean another one will get worse’ – research has found learning to control some tics makes all tics better (even the ones you have not worked on). ‘Will my child ‘catch’ other children’s tics’ – children may briefly pick up other tics during contact with other children with tics but they usually only last a couple of days (Chowdhury and Murphy, 2017).  Even with this short-term issue, parents and children report it is definitely worth picking up a few temporary tics when meeting other families affected by TS – the benefits are very much worth it!

In the ‘European clinical guidelines for Tourette Syndrome. Part III: behavioural and psychosocial interventions’, both HRT and ERP interventions are considered first line behavioural treatments for tics for both children and adults (Verdellen, van de Griendt, Hartmann, et al. 2011).

Deep Brain Stimulation (DBS)

There have been trials in the UK and internationally for treating very severe TS – which has been resistant to all other means of treatment and management – with Deep Brain Stimulation (DBS), a form of neurosurgery involving the implantation of electrodes at selected targets in the brain. The use of DBS in patients with very severe TS is still in its research stages, and although results have been hopeful, it is still not clear whether this will become an available treatment option. Due to both different legality and practical facilities in different European countries these guidelines, therefore, have to be understood as recommendations of experts. However, there seems to be general agreement that, at present time, DBS should only be used in adult, treatment resistant, and severely affected patients.  DBS is not available for children or teenagers in the UK aside from when the symptoms are life threatening.

PANDAS

Paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) has been suggested as relating to a subset of children who present with sudden-onset with OCD and or tics. This is a controversial condition with little research evidence to back up a link to a streptococcal infection but ‘it is recognised that there is a subgroup of children who present with sudden-onset acute OCD with or without tics (Chowdhury and Murphy, 2017).

PANDAS has been partially superseded by the term PANS (also known as CANS) which stands for Paediatric Acute-Onset Neuropsychiatric Syndrome. PANS potentially includes cases that would have previously fitted the definition of PANDAS, but PANS does not require the presence of tics or streptococcal infection, although the majority of PANS cases do have tics.  PANS is defined by the sudden start of OCD, eating problems and other neuropsychiatric problems.

As PANS and PANDAS is surrounded by scientific uncertainty and controversy, clinical opinions from different doctors can vary. Assessment should be by a doctor with experience in TS.

Cannabinoids

Medical cannabinoids are not yet a proven treatment for Tourette syndrome, although there is ongoing research on this in Germany and the US. In the NHS they can be prescribed only with a special application in the case of Tourettes. Whilst the available medications vary in how effective they are in each person, there is no evidence to suggest that cannibinoids are better, so there is not current justification for first line use.

In the Cannabis Scheduling Review Part 1, reviewing recent evidence of the therapeutic and medicinal benefits of cannabis based product (Department of Health and Social Care, 2018), there is limited evidence (supportive findings from fair-quality studies or mixed findings with most favouring one conclusion) that cannabis or cannabinoids are effective for improving symptoms of Tourette syndrome (THC capsules).

Research into genetics, brain imaging, behavioural science, neuroscience and neuropathology is helping us to make progress towards understanding the basis of TS. One of the main aims of Tourettes Action (TA) is to support research and to seek better treatment and management of TS (via research into new therapies and potential cures) with a view to improving the lives of those living with TS.

TMS

TMS stands for Transcranial magnetic stimulation. ‘This is a non-invasive method of brain stimulation in which an electric coil is used to induce a magnetic field over the scalp. This induces circular electrical (eddy) currents in the brain that in turn cause stimulation of neurons and muscle activity. Repeated stimulation with short bursts of high-frequency stimulation has been applied for the treatment of movement disorders in humans’ (Hollis et al, 2016).

TMS is one particular type of non-invasive brain stimulation (NIBS); research findings suggest that NIBS may form the basis for new non-drug treatments for Tourette syndrome that are safe and effective in reducing tics. They have been used with some success in other movement disorders such as Parkinson’s Disease. NIBS is being used in the UK to treat a number of conditions such as depression, chronic pain, and to promote recovery of function after stroke, however NIBS is not yet being used to treat Tourette syndrome. More research evidence would be needed before this could be considered as a potential treatment.

Emerging treatments

In addition to the pharmacological treatment presented, the EU clinical guidelines (Roessner et al 2011) also put forward the case that there is increasing evidence ‘for the efficacy of botulinum toxin injections to treat persistent well-localized (non-complex) motor and, sometimes, vocal tics by temporarily weakening the associated muscles’. There can be adverse reactions including temporary soreness and mild muscle weakness.

There are some research evidence that there can be beneficial effects of exercise on TS symptomatology as well as beneficial impacts on self-reported anxiety and mood levels (Nixon, Glazebrook, Hollis and Jackson, 2014).

There are some not-yet validated treatments which do not have an established research evidence base, but which some people report having found including neurofeedback, acupuncture, dental splints, music, nutritional supplements.

Murphy, T., Chowdhury, U. (2017) Tic Disorders: A Guide for Parents and Professionals. Jessica Kingsley Publishers

Robertson, M.M. AND Baron-Cohen, S. (1998) Tourette Syndrome: The Facts (2^nd ed). Oxford University Press

Verdellen, C., van de Griendt, J., Kriens, S. and van Oostrum, I. (2011) Tics: Therapist Manual. Amsterdam: Boom Publishers.

Woods. D., Piacentini, J., Chang, S.W., Deckersbach, T. et al. (2008) Managing Tourette Syndrome: A Behavioural Intervention for Children and Adults.  Therapist Guide (Treatments That Work). Oxford University Press.

Websites

Tourettes Action
Supports people with Tourette Syndrome, and those who work with them, and funds research into treatment and diagnosis.

OCD Action
Provide support and information to anybody affected by OCD, work to raise awareness of the disorder amongst the public and front-line healthcare workers, and strive to secure a better deal for people with OCD.

ADHD Foundation
The ADHD Foundation works in partnership with individuals, families, and professionals to improve the well-being, and life chances of people living with ADHD, ASD and related learning difficulties.

Anxiety UK
Works to relieve and support those living with anxiety and anxiety-based depression by providing information, support and understanding via an extensive range of services.

Tourette Scotland
A charity working exclusively in Scotland to support people living with TS

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