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Clinical management of complex motor stereotypies
  1. Raj Seraya Bhatoa1,
  2. Osman Malik1,
  3. Sally Robinson1,
  4. Tammy Hedderly1,2
  1. 1Children’s Neurosciences, Tics and Neurodevelopmental Movements Service (TANDeM), Evelina London Children’s Hospital, London, UK
  2. 2Child Health Children's Academic Group, King's College London, London, UK
  1. Correspondence to Dr Tammy Hedderly, Children’s Neurosciences, Tics and Neurodevelopmental Movements Service (TANDeM), Evelina London Children’s Hospital, London SE1 7EH, UK; Tammy.Hedderly{at}gstt.nhs.uk

Abstract

This paper will review complex motor stereotypies and provide a summary of the current proposed treatment pathway.

  • child health
  • mental health
  • neurology
  • paediatrics
  • psychology

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What this study adds?

  • To our knowledge, there is no similar paper aimed at helping the paediatrician and neurologist to support families and children with complex motor stereotypies, despite this being a common movement condition in childhood.

Introduction

This clinical practice guideline has been developed to describe how complex motor stereotypies (CMS) can be assessed in the clinic and then managed by a multidisciplinary team (MDT). The purpose of this paper is to serve as a resource for paediatricians and clinicians, to provide a treatment protocol and therefore support the management of CMS in children and young people (CYP) in local services.

We have limited this paper to describe assessment and management approaches, rather than including a wide discussion around the multiple aetiologies of CMS, as this is discussed elsewhere.1 2 The suggested management tips and approaches are likely to be most useful for children with CMS that are considered to be primary genetic, rather than stereotypies that are seen as secondary phenomena with seizures, autoimmune encephalitides or with exposure to toxins/drugs, as management of secondary stereotypies will usually involve treatment of the underlying cause. The suggested management strategies can, however, help in CYP whose CMS occur in association with autism spectrum, generalised anxiety disorders or genetic syndromes, providing the intellectual and cognitive abilities are of a level that will allow them to engage with the proposed approaches.

Definition, nature and course of CMS

Stereotypies are defined as involuntary, repetitive, rhythmic, patterned, predictable, but non-goal directed sounds and movements.3 They include ‘simple’ motor stereotypies such as body rocking, head nodding, finger tapping, and more CMS such as unusual upper limb twisting and hand flapping, waving or pacing. CMS can last from a few seconds to a few hours and can appear many times a day.4 5 Triggers include excitement, being engrossed in activities, thinking about things they like, being bored, anxious, stressed or tired.6 Common places to see stereotypies are while seated in the car, sitting in a high chair/at mealtimes, watching television and/or playing computer games.7 CMS may also be accompanied by intense imagery movements (IIMs),7 8 whereby CYP report conscious engagement in deep, enjoyable thoughts, visual images or imaginary play.4 The term ‘stereotypic movement disorder’ is sometimes used; specifically, when stereotypies interfere with social, academic or other activities and/or result in injury.9

CMS of the likely primary genetic type, focused on in this paper, usually appear during the first 3 years of life and often before the age of 2 years.3 5 Children with primary stereotypies have been reported to present to paediatric clinics at an earlier age (average age of 4 years 8 months) than children with secondary stereotypies (average age 6 years 8 months), which has been proposed to reflect the worsening progression of CMS from onset for children with primary stereotypies.10 CMS occur more commonly in males than females, with studies reporting a 3:2 ratio.3 6 11 CMS are reported to occur in 3%–8% of the general population12 13 with prevalence of motor stereotypies reported to range between 22% and 98% for individuals with autism spectrum disorder (ASD), and up to 80% for individuals with intellectual disability or developmental delay without ASD.14 Up to 60% of typically developing children reportedly show some stereotypic movements or behaviours between 2 and 5 years of age.15 This indicates that stereotypies form a normal part of early development, so conservative management is recommended. However, for children where the movements persist in later childhood and the teenage years, then the movements may be perceived as odd or unusual by peers, which for some can contribute to social difficulties or bullying.

CMS can occur in typically developing CYP, children who appear to be neurotypical but with traits or features of neurodevelopmental difficulties, or in association with established neurodevelopmental difficulties such as ASD, attention-deficit–hyperactivity disorder (ADHD), tic disorders, Fragile X syndrome, Down syndrome and Prader-Willi syndrome.16 17 CYP may also have higher rates of developmental coordination disorder than the general population.4 There is likely to be a spectrum of primary CMS in which stereotypies exist as part of a wider neurodevelopmental presentation or personality type, with some children with CMS presenting with traits or features of other neurodevelopmental difficulties without meeting threshold for a diagnosis on formal evaluation. Consideration of broader developmental, social and emotional needs is therefore recommended as part of clinical management to ensure the CYP can access the full range of support required to meet their presenting needs.

Parents often ask what causes primary CMS. Currently, the aetiology of stereotypies remains unknown, but it is useful for clinicians to share with families that it is thought that stereotypies may reflect differences in the structure and chemistry of the brain,18–25 possible genetic differences,3 26 and/or a child’s method for self-soothing or self-stimulating.21 27–29

Differential diagnoses

CYP may present with a range of movements, with CMS often being misunderstood or misdiagnosed as ‘tics’. Tics are very important to diagnose as the management is different, with non-suggestibility being key in tic treatments. CMS typically occur ‘continuously for a period of time in the same form and on multiple occasions’ and are typically distracting, which would exclude brief intermittent movements that are typical of tics.1 It is also important to note that CYP with tics usually report them as being bothersome or annoying to experience, with the tic providing temporary relief from the preceding premonitory urges that are described as unpleasant. Conversely, CMS usually lack an urge and are typically reported as being enjoyable, fun or soothing to perform, with CYP (and adults) often expressing a desire to engage with the movements due to their enjoyable nature.4 5

It is often helpful to share with families that while CMS and tics may occur in the same individual, they have important differences.12 In particular, CMS have an earlier age of onset (before the age of 3 years), whereas tics usually occur later in childhood (mean onset 5–7 years).5 CMS tend to cluster and last for a longer period compared with tics, which are briefer and more sudden in nature.12 CMS also comprise fixed, rhythmic movements which are predictable in pattern, amplitude and location across development in each child.13 They are usually represented in the distal upper extremities and trunk and often include a mouth stretching phenomenon.12 In contrast, tics wax and wane over time and typically follow a rostral–caudal pattern over development.12 These differences are summarised in table 1.

Table 1

Differentiations between tics and stereotypies

There are some movements that can look like CMS or neurological conditions that produce CMS as part of the neurological process, such as some forms of seizure disorder.30 These are more likely to have atypical features such as sudden onset at a later age and be associated with other changes in behaviour. Some of the autoimmune encephalopathies experienced in childhood such as N-methyl-D-aspartate receptor (a glutamate receptor and ion channel found in neurons) antibody-mediated encephalitis can also include stereotypies within the clinical phenomena.31 If perioral dyskinesias or severe behavioural changes are present in association with CMS, a full neurological assessment is advised. Drug-induced stereotypies with certain medications (such as stimulants) could also be apparent,32 and it is important to consider recreational drug use if there are atypical features or a later age of presentation. In children with ASD and learning disability stereotypies or an increase in stereotypies is a feature of catatonia,9 hence if stereotypies become problematic or very prominent with other features of catatonia (such as freezing or being stuck in a posture), then this needs to be assessed and managed.

Multidisciplinary assessment

The Tics and Neurodevelopmental Movements (TANDeM) clinic is a National and Specialist Service in the National Health Service that provides services for CYP with movement difficulties. The TANDeM clinic includes an MDT comprising neurology, psychology, psychiatry and nursing. In the TANDeM clinic, CYP are initially offered a comprehensive MDT appointment, which includes examination of their current needs and history of presenting concerns. A clinical review will include an assessment of probable causes to ensure that the movements are not markers of an identifiable neurological disorder/structural brain lesion, neurogenetic diagnosis or medication side effects. Points to guide clinical assessment are included in figure 1.

Figure 1

Clinical assessment points in children with suspected CMS. With permission from RSB, OM, SR and TH, who have created this original figure for the sole purpose of this manuscript (and has not been published elsewhere). CMS, complex motor stereotypies; IIM, intense imagery movement.

Examination

A full neurological examination is recommended as part of the initial assessment. This should include an assessment of subtle unusual features and neurocutaneous stigmata. It can be helpful to assess carefully for other movement patterns such as myoclonus, tremor, chorea, dystonia and tics, as well as to perform a gait examination. Growth parameters to include head circumference should be recorded and a developmental assessment is advised. It is helpful to include some assessments of social communication and a mental state examination where appropriate.

Functional analysis

CMS may form a part of normal early development; therefore, in many cases, no treatment may be required. However, if the movements are more persistent across development or impairing to daily functioning, then a functional behavioural analysis (also known as an ‘ABC’ analysis) may be helpful. This would involve interviewing CYP and families to identify the ‘Antecedents’ or triggers to the movements (eg, extreme excitement, anxiety-provoking situations, environmental demands), the ‘Behaviours’ (ie, a clear description of the CMS movement patterns), and the ‘Consequences’ or responses following the movements (eg, others’ reactions, task avoidance, increased excitement, gratification). This may help clarify situations where the movements occur, if the movements serve a functional purpose and whether further support is required (eg, unmet learning needs, attention problems, anxiety).

Giving the diagnosis

In our clinical opinion, it is important to present the view to CYP and families/carers that a diagnosis of CMS is a positive diagnosis (given the typical developmental trajectory), with concerns raised only if they are interfering significantly with daily functioning (eg, engaging in learning, social interactions) or have become compulsive and/or distressing in nature.12 Some CYP learn to privatise CMS with age and will modify or control them in public with little support or guidance.5 This appears to be related to increased awareness of how the movements may be perceived by others, as well as increased maturation of neural networks that support inhibition and motor control.

Clinical management

A ‘stepped care’ approach that is matched to the CYP needs is advised, with support that primarily includes psychoeducation and behavioural management strategies. Pharmacological support may be helpful in some cases, though this would not be considered the first-line approach.

Psychoeducation

Psychoeducation, as a first-line intervention for all families, provides CYP and parents/carers with information about the prevalence, characteristics, nature and course of CMS (as discussed in the introduction) with parents/carers often being reassured about the typical usual outcomes, especially since CMS may be a new term to most families. This may be held with individual CYP and parents/carers following the clinical assessment, or as part of a group intervention with several families.33 Suggested psychoeducation tips are explored below:

Self-help strategies

Psychoeducation can help reduce distress and support CYP and parents/carers to better manage the movements at home and school. In our experience, it is helpful to provide the following self-help strategies at the clinical assessment:

  • Talk to your child about the CMS to help reassure them that the movements are nothing to worry about and encourage them to come and tell you if they have any problems.

  • Name the CMS to help your child realise that they and the stereotypies are not the same thing (‘externalise’ the problem).

  • Monitor/observe when your child performs the CMS to help identify when they are happening in case they need extra support at these times (for example, at school when bored, not understanding the work or trying something new).

  • Minimise the CMS by helping your child to develop a movement/sound that is ‘smaller’ and ‘less socially noticeable’ than the CMS (for example, standing up/down/on tiptoes, rather than jumping).

  • Practise CMS suppression by selecting a time/situation when your child will try to ‘hold in’ the CMS, with short practice sessions to begin with (perhaps 3–5 min) that are slowly increased as your child successfully holds them in.

  • Praise and rewards should be given to help keep your child motivated to practise controlling CMS and at any other time when you notice they have stopped.

CYP may not be motivated to engage in strategies to reduce or manage the CMS, since they are often enjoyable, soothing and may provide some form of sensory gratification. It is therefore important to establish with families if the CMS are indeed causing any dysfunction, as this helps families to work towards a balance between children enjoying the CMS, while ensuring the movements are not intrusive to their daily functioning or quality of life.

Promoting ‘privatisation’

One way to support the management of CMS is to encourage CYP to perform the movements at home, in their own bedroom or in the bathroom. This strategy is called promotion of privatisation, as this encourages CYP to do the CMS in private only.12 CYP naturally adopt this strategy as they get older and are more aware of social norms. Increase awareness to help your child learn when they are performing the CMS by discretely pointing it out to them (for example, special signal/name, a gentle tap on the shoulder) and re-engaging them with their activity.

To encourage or ‘promote’ privatisation of the CMS, clear goal–setting is encouraged with the CYP. The goals should include which setting(s) the CYP are expected to reduce the CMS in, as well as time(s) when the CYP are able to freely engage in the CMS (eg, privately at home or in their bedroom). To support privatisation, a short achievable time frame (eg, 5/10 min) is encouraged to begin with, with the length of time the CYP are encouraged to engage in privatisation being increased with practise. This helps the CYP to develop more control. If CYP are non-verbal, strategies such as visual schedules, countdown, communication tools (Picture Exchange Communication System, Makaton) or social stories can help.

If the CMS serve a soothing function, then reducing them may result in increased anxiety in the CYP. It is therefore vital to incorporate alternative, soothing strategies into the treatment plan (which can be a personalised plan), as well as the use of evidence-based strategies (eg, cognitive–behavioural therapy (CBT)) to provide the CYP with the tools to manage underlying anxieties, so that the need to engage in CMS to self-soothe is reduced. It should be noted that in these cases, that support for CYP to privatise or reduce engagement with CMS may be most challenging, with the CYP often being reluctant to engage in these strategies due to the functional nature served by the CMS.

Distraction

Distraction techniques often help to minimise the amount of time CYP spend engaging in CMS, with distraction exerting a powerful and immediate suppressive effect on them.5 12 This can include substituting the movements with other enjoyable activities such as drawing or playing indoors/outdoors. For older CYP, helping them to identify the added benefits/pros of engaging in alternative behaviours can be helpful to support motivation and engagement with distraction techniques.

Rewards

Incentivising efforts with rewards is another useful method for supporting engagement in strategies to support reduction of the movements. It is helpful to encourage parents/carers to develop reward charts with the CYP, as this can provide a clear structure to help monitor a child’s progress and incentivise them for their hard work. The use of ‘protected time’ when the CYP can freely engage in the CMS is also encouraged as a form of reward, as this can help sustain a child’s motivation to reduce the CMS in other settings when the movements may be more functionally impairing.

Behavioural therapies

The majority of CYP and parents/carers respond well to psychoeducation about CMS with self-help strategies, and they often do not require further psychological or pharmacological treatments. However, clinicians should inform CYP and parents/carers that behavioural strategies such as habit reversal therapy and differential reinforcement have been shown to be effective for the management of CMS.34 35 These therapies involve helping CYP gain greater control over the CMS by increasing awareness of when they are performing the movements/sounds and teaching CYP to perform a new response when they feel the urge to perform the CMS. Over time, CYP get used to not performing the CMS, which helps to reduce them. This technique is more likely to be successful if practised regularly and if the CYP are aware of their inner bodily sensations and want to stop performing the CMS.

CBT may also be an option for families. Clinicians should inform families that CBT is a talking therapy that involves helping children gain an understanding of how thoughts, feelings and behaviours are connected. CBT can help children who perform CMS when they are worried or upset, by teaching them other ways to cope with their emotions. CBT is often more suitable for older children who are experiencing problems controlling the CMS due to feelings of anxiety or low mood affecting their daily life. CYP and parents/carers should be made aware that CBT requires the active participation and the cognitive capabilities to understand and follow advice from a therapist, which may exclude younger children or those with moderate/severe learning disabilities—who often experience more severe and frequent CMS symptoms.21 34

School liaison by clinicians can support the consistent use of behavioural strategies across settings, which is helpful to achieve the best possible outcomes for supporting CYP to manage CMS. In some cases, CYP may present with unmet learning needs and/or neurodevelopmental needs, with the identification and management of these difficulties often having a positive impact on the movements, which can help support children’s academic development and emotional well-being.

Pharmacological treatments

CYP and parents/carers should be informed that medication is normally only considered to manage primary stereotypies if behavioural strategies have been ineffective, not tolerated or difficult to engage with due to other factors (eg, moderate/severe learning disability). Medication should be considered if there is interference with function or if the movements are associated with distress. The choice of medication will therefore depend on: (1) the type of symptoms that are causing concern, (2) how severe the symptoms and functional impairment are, and (3) the possible risk of side effects.

The evidence for pharmacological treatments in CMS is variable. One study investigating the effect of sertraline found limited success.36 Fluoxetine is a selective serotonin reuptake inhibitor (SSRI) that has been shown to decrease stereotypic behaviours in vervet monkeys in captivity,37 though the mechanism by which the stereotypy decreases is unclear.17 Psychotropic drugs such as low-dose risperidone and aripiprazole have been shown to reduce CMS in children with ASD.38 39 A double-blind placebo-controlled study of low-dose buspirone in children with ASD has also been shown to reduce stereotyped repetitive behaviours.40 In our clinical experience, some children with ADHD and CMS have shown a significant reduction in CMS when the ADHD was treated with a stimulant, although caution is advised as stimulants can also produce or exaggerate stereotyped movements. If CMS have worsened with anxiety, then anxiolytic medications such as SSRIs may reduce CMS, but this is likely an indirect effect from lowering anxiety. Similarly, if IIM/CMS have become part of an obsessive compulsive disorder (OCD) then other treatment, for example, OCD medications (SSRIs) may reduce the IIM.

As with all prescriptions, the side effect profile is reviewed as part of usual paediatric care. We recommend regular reassessment in children on medication to monitor for any negative effects.

Recommendations for further referral

There are a range of factors that will influence whether a referral to a neurologist or tertiary movement disorder clinic is required to support CYP in managing CMS. In particular, consideration should be given to whether the CYP is presenting with atypical features (eg, later age of onset, lack of natural reduction with age), the extent to which the movements are impacting on daily functioning (eg, interference with learning, peer interactions) and response to psychoeducation or self-help strategies already shared (eg, non-suppression with distraction, difficulties with privatisation). Other indications for referral would include additional neurological features, focal neurological signs and abnormal systemic or neurological examination.

Conclusion

CMS can occur in typically developing CYP and can be associated with neurodevelopmental and/or neuropsychiatric conditions. Commonly co-occurring conditions should be screened for and appropriate support for any additional difficulties should be provided, or the CYP should be referred or signposted to relevant services. Whenever possible, an initial assessment should include a full medical and neurodevelopmental history, with analysis of presenting movements, impact on daily functioning and the CYP’s motivation to manage the movements. Identifying CYP and parent/carer beliefs about the nature of the movements is also helpful so that any misconceptions can be addressed and appropriate psychoeducation and advice to promote privatisation provided. The techniques are likely to be most effective in children with intellectual and cognitive abilities within the broad average ranges, though with appropriate adaptations these strategies can also be used to support CYP with lower levels of functioning. However, for children with severe learning difficulties and intrusive CMS, environmental modifications and/or medication is likely to be most helpful to manage the movements.

The overall prognosis for CYP with CMS is generally positive and this should be conveyed to CYP and parents/carers, with many young people learning to inhibit their CMS either completely or when required to minimise impact on daily functioning. However, it is acknowledged that this view and the guidance provided in this article is based primarily on clinical experience, with a need to evaluate the clinical efficacy of proposed strategies and longer term outcomes for individuals with CMS during childhood.

Ethics statements

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References

Footnotes

  • Contributors All authors contributed to this manuscript. RSB composed the main text, with contributing expert opinions from neuropsychiatry (OM), psychology (SR and RSB) and neurology (TH). Team agreement was made for RSB to be the first (and submitting) author, and for TH to be senior (and corresponding) author.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.