Spasticity in children and young people with non-progressive brain disorders: summary of NICE guidanceBMJ 2012; 345 doi: http://dx.doi.org/10.1136/bmj.e4845 (Published 26 July 2012) Cite this as: BMJ 2012;345:e4845
- Moira A Mugglestone, director of guideline development1,
- Paul Eunson, consultant paediatric neurologist2,
- M Stephen Murphy, clinical co-director (children’s health)1, senior lecturer in paediatrics and child health3, consultant paediatric gastroenterologist4
- on behalf of the Guideline Development Group
- 1National Collaborating Centre for Women’s and Children’s Health, London W1T 2QA, UK
- 2Royal Hospital for Sick Children, Edinburgh EH9 1LF, UK
- 3College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UK
- 4Birmingham Children’s Hospital NHS Foundation Trust, Birmingham B4 6NH
- Correspondence to: M A Mugglestone
Spasticity is a form of hypertonia1 and is associated with conditions such as cerebral palsy, which affects 110 000 people in the United Kingdom.2 More than 2000 children born this year in the UK will develop spasticity, which, if unmanaged, will cause pain, affect quality of life, and may lead to complications requiring major surgery. Children and young people with spasticity need early referral to local services that will meet their individual needs and allow them access to the range of interventions that will encourage their motor development. This article summarises the most recent recommendations from the National Institute for Health and Clinical Excellence (NICE) on the management of spasticity in children and young people with non-progressive brain disorders, including those with cerebral palsy.3
NICE recommendations are based on systematic reviews of best available evidence and explicit consideration of cost effectiveness. When minimal evidence is available, recommendations are based on the Guideline Development Group’s experience and opinion of what constitutes good practice. Evidence levels for the recommendations are given in italic in square brackets.
Principles of care
Children and young people with spasticity should have access to a network of care that uses agreed care pathways supported by effective communication and integrated team working, and provides access to healthcare professionals experienced in the care of such people. The network team should provide local expertise in paediatrics, nursing, physiotherapy, and occupational therapy. Access to other expertise, including orthotics, orthopaedic surgery (and/or neurosurgery), and paediatric neurology, may be provided locally or regionally.
If a person receives treatment for spasticity from healthcare professionals outside the network team, this should be planned and undertaken in discussion with the network team to ensure integrated care and effective subsequent management.
[Both points based on the experience and opinion of the Guideline Development Group (GDG)]
After diagnosis, ensure that all children and young people with spasticity are referred without delay to an appropriate member of the network team.
Offer a management programme that is (a) developed and implemented in partnership with the individual and his or her parents or carers, tailored to the individual, and goal focused; and (b) takes into account the programme’s possible impact on the person and family. [Based on the experience and opinion of the GDG]
Carefully assess the impact of spasticity in people with cognitive impairments: be aware that any benefit of treatments may be more difficult to assess in those with limited communication; and ensure that they have access to all appropriate services.
Record the individualised goals and share these goals with the network team and, where appropriate, other people involved in their care.
Offer relevant, and age and developmentally appropriate information, and educational materials; regular opportunities for discussion; and advice on individuals’ developmental potential and how treatment options may affect this. [Based on the experience and opinion of the GDG]
Monitor for response to treatments; worsening of spasticity; secondary consequences of spasticity (for example, pain or contractures); and the need to change individualised goals. [Based on the experience and opinion of the GDG]
Recognise clinical findings that are possible indicators of hip displacement (hip migration greater than 30%) (box 1).
Offer hip radiography to assess for hip displacement if there are clinical concerns about possible hip displacement or at 24 months in children with bilateral cerebral palsy.
Box 1 Possible indicators of hip displacement
Pain arising from the hip
Clinically important difference in leg length
Deterioration in hip abduction or range of hip movement
Increasing muscle tone in the hip
Deterioration in sitting or standing
Increasing difficulty with perineal care or hygiene
Physical therapy (physiotherapy and/or occupational therapy)
Everyone referred to the network team should be promptly assessed by a physiotherapist and, where necessary, an occupational therapist. [Based on the experience and opinion of the GDG]
Offer a programme of physical therapy (physiotherapy and/or occupational therapy) that is tailored to the individual’s needs and goals, such as enhancing skill development, function, and ability to participate in everyday activities; and preventing consequences such as pain or contractures.
When deciding who should deliver physical therapy, take into account whether the person and parents or carers can deliver this therapy, what training they might need, and what their wishes are.
Encourage individuals and their parents or carers to incorporate physical therapy into daily activities—for example, standing while brushing their teeth (to stretch leg muscles).
Consider including 24 hour postural management strategies (a) to prevent or delay the development of contractures or skeletal deformities in those at risk of developing these; and (b) to enable participation in activities appropriate to their stage of development.
Consider task focused, “active use” therapy, such as “constraint induced movement therapy” (temporary restraint of an unaffected arm to encourage use of the other arm) followed by bimanual therapy (unrestrained use of both arms) to enhance manual skills.
Consider muscle strengthening therapy when the assessment indicates that muscle weakness is contributing to loss of function or postural difficulties.
Provision of an adapted physical therapy programme is essential after treatment with botulinum toxin type A, continuous pump administered intrathecal baclofen, orthopaedic surgery, or selective dorsal rhizotomy. [Based on the experience and opinion of the GDG]
Consider orthoses based on individual needs and goals, such as improving posture, upper limb function, or walking efficiency; preventing or slowing hip migration or development of contractures; relieving discomfort, pain, or pressure points. [Based on low to high quality evidence from randomised controlled trials]
Consider using elbow gaiters to maintain extension and improve function; rigid wrist orthoses to prevent contractures and limit flexion deformity; and dynamic orthoses to improve hand function (for example, a non-rigid thumb abduction splint to allow some movement with a “thumb in palm” deformity).
Consider ankle-foot orthoses for those with serious functional limitations (level IV or V in the gross motor function classification system; box 2) to aid sitting, transfers between sitting and standing, and assisted standing.
If an orthosis is used overnight, check that it is acceptable to the child or young person and does not cause injury or disturb sleep.
Box 2 Gross motor function classification system
Level I—Walks without restrictions
Level II—Walks without assistive devices
Level III—Walks with assistive devices
Level IV—Has limited self mobility
Level V—Has severely limited self mobility even with assistive devices
The network team should review the use of orthoses at every contact with the person. Ensure the orthosis remains acceptable to the person and parents or carers; remains appropriate to treatment goals; is being used as advised; remains well fitting and in good repair; and is not causing adverse effects such as discomfort, pain, sleep disturbance, injury, or excessive muscle wasting.
Consider oral diazepam or oral baclofen if spasticity is contributing to discomfort or pain; muscle spasms (for example, at night); or functional disability. Diazepam is particularly useful if a rapid effect is desirable (for example, in a pain crisis), and baclofen for a sustained long term effect (for example, to relieve continuous discomfort or improve motor function). [Based on low to moderate quality evidence from randomised controlled trials]
Give oral diazepam treatment as a bedtime dose. If the response is unsatisfactory consider increasing the dose or adding a daytime dose.
Start oral baclofen treatment with a low dose and increase the dose stepwise over about four weeks to achieve the optimum therapeutic effect.
Continue using oral diazepam or oral baclofen if they have a clinical benefit and are well tolerated, but consider stopping the treatment whenever the child’s or young person’s management programme is reviewed and at least every six months.
If the response to oral diazepam and oral baclofen used individually for four to six weeks is unsatisfactory, consider a trial of combined treatment using both drugs.
If stopping oral diazepam and/or baclofen after several weeks of use, ensure that the dose is reduced in stages to avoid withdrawal symptoms.
If dystonia is considered to seriously impair posture or function or cause pain, consider a trial of oral drug treatment—for example, with trihexyphenidyl, levodopa, or baclofen.
Botulinum toxin type A
Consider this treatment for focal spasticity of the upper limb that is impeding fine motor function; compromising care and hygiene; causing pain; impeding tolerance of other treatments, such as orthoses; or causing cosmetic concerns to the person. [Based on low to high quality evidence from a meta-analysis of randomised controlled trials and a further randomised controlled trial]
Consider this treatment where focal spasticity of the lower limb is impeding gross motor function; compromising care and hygiene; causing pain; disturbing sleep; impeding tolerance of other treatments, such as orthoses and equipment to support posture; or causing cosmetic concerns to the person. [Based on very low to moderate quality evidence from randomised controlled trials]
Consider this treatment after an acquired non-progressive brain injury if rapid onset spasticity is impairing posture or function.
Consider a trial of this treatment for focal dystonia that is seriously impairing posture or function or causing pain.
Before starting this treatment, advise people and their parents or carers of its rare but serious complications (swallowing and breathing difficulties) and how to recognise signs suggesting these complications. Advise that these complications may occur at any time during the first week after the treatment and that if these complications occur they should return to hospital immediately.
Consider treatment with continuous pump administered intrathecal baclofen if, despite the use of non-invasive treatments, spasticity or dystonia is causing pain or muscle spasms, or difficulties with posture, function, self care, or care by parents or carers. [Based on very low to moderate quality evidence from a randomised controlled trial and observational studies and on a health economic model developed for the guideline]
Be aware that those who benefit from this treatment typically have moderate or severe motor function problems (level III, IV, or V; box 2) or bilateral spasticity affecting upper and lower limbs.
Support those receiving this treatment and their parents or carers by offering regular follow-up with the network team and a consistent point of contact with the specialist neurosurgical centre.
Consider orthopaedic surgery as an important adjunct to other interventions. Timely surgery can prevent deterioration and improve function.
If clinical or radiological findings indicate possible spinal deformity or hip displacement, an orthopaedic surgeon in the network team should do an assessment. [Based on the experience and opinion of the GDG]
Consider an assessment by an orthopaedic surgeon in the network team if limb function is limited by unfavourable posture or pain as a result of muscle shortening, contractures, or bony deformities; any upper limb contractures cause difficulty with skin hygiene; or the cosmetic appearance of the upper limb causes major concern for the person.
Selective dorsal rhizotomy
Consider selective dorsal rhizotomy to improve walking ability in children and young people who are at level II or III in the gross motor function classification system (box 2). Patient selection and treatment should be carried out by a multidisciplinary team with specialist training and expertise in the care of spasticity, and with access to the full range of treatment options. Discuss the irreversibility of the treatment, the known complications and the uncertainties over long term outcomes with children and young people, and their parents and/or carers (and see also NICE’s interventional procedure guidance4). Teams offering selective dorsal rhizotomy should participate in a coordinated, national, agreed programme to collect information on short and long term outcomes on all patients assessed for selective dorsal rhizotomy, regardless of whether selective dorsal rhizotomy is performed. These recorded outcomes should include measures of muscle tone, gross motor function, neurological impairment, spinal deformity, quality of life, and need for additional operations, with nationally agreed consistent definitions. [Based on very low to high quality evidence from randomised controlled trials and observational studies]
Delivering services for the individual needs of children and young people with spasticity may be difficult if they cannot express their views and preferences clearly. The aim of the recommendation to carefully assess the impact of spasticity in those with cognitive impairments is to help formulate appropriate management programmes. Delivering integrated services that encompass the entire range of recommended interventions may be difficult to achieve in some localities. The definition of the network team is deliberately flexible to facilitate commissioning of services unrestricted by institutional or regional boundaries.
Further information on the guidance
This is the first clinical guideline to be published by NICE on the management of spasticity in children and young people with non-progressive brain disorders. It includes some recommendations on the management of coexisting motor disorders (such as dystonia) and early musculoskeletal complications (for example, pain and contractures). Although the guideline covers the management of spasticity associated with cerebral palsy, it does not cover all aspects of the management of cerebral palsy.
In addition to the recommendations summarised here, the guideline advises on support for the person and their parents and carers (for example, offering contact details of patient organisations, ensuring timely access to postural management equipment, and explaining the central role of the network team in preparing young people and their parents or carers for the transfer to adult services). It also covers contraindications to treatment with botulinum toxin type A; the possible need for analgesia, anaesthesia, or sedation during the botulinum toxin injection; the use of ultrasound or electrical muscle stimulation to guide the injection; the use of orthoses after botulinum toxin type A; and assessments to evaluate the response to this treatment and inform decisions about further injections. Other recommendations in the guideline cover contraindications to treatment with intrathecal baclofen, intrathecal baclofen testing to evaluate therapeutic and adverse effects, and aspects of treatment with continuous pump administered intrathecal baclofen, including implanting the pump and pre-implantation and post-implantation assessments.
This guidance was developed by the National Collaborating Centre for Women’s and Children’s Health in accordance with NICE guideline development methods (www.nice.org.uk/guidelinesmanual). The Guideline Development Group (GDG) comprised healthcare professionals from the disciplines involved in caring for children and young people who have spasticity and coexisting motor disorders and their early musculoskeletal complications as a result of a non-progressive brain disorder (physiotherapists, an occupational therapist, a nurse, a community paediatrician, paediatric neurologists, an orthopaedic surgeon, and a general practitioner), lay members with experience of caring for such children or young people, and experts in guideline methodology. The GDG identified relevant clinical questions, collected and appraised clinical evidence, and where possible evaluated the cost effectiveness of proposed interventions. The draft guideline underwent a rigorous validation process in which the GDG invited stakeholder organisations to comment and took all comments into consideration when producing the final version of the guideline.
NICE has produced four different versions of the guideline: a full version containing all the evidence, the process undertaken to develop the recommendations, and all the recommendations; a version containing a list of all the recommendations, known as the “NICE guideline”; a version for patients and the public; and a web version of the care pathway (the “NICE pathway”). All these versions are available from the NICE website (www.nice.org.uk/CG145). The guideline will be reviewed for an update in three years’ time as part of the NICE guideline development programme.
What are the greatest inhibitors of functional ability in children and young people with upper motor neurone lesions?
What is the optimal postural management programme using a standing frame in children aged 1-3 years?
What is the clinical and cost effectiveness of botulinum toxin type A when used routinely or according to clinical need in children and young people who are at level I, II, or III in the gross motor function classification system (box 2)?
What is the effectiveness of continuous pump administered intrathecal baclofen compared with usual care in children and young people who are at level IV or V (box 2)?
Does selective dorsal rhizotomy followed by intensive rehabilitation performed between the ages of 3 and 9 years in children who are at level II or III (box 2) result in good community mobility as a young adult?
Cite this as: BMJ 2012;345:e4845
This is one of a series of BMJ summaries of new guidelines based on the best available evidence; they highlight important recommendations for clinical practice, especially where uncertainty or controversy exists.
The members of the Guideline Development Group were Gordon Allan, Lauren Bardisa-Ezcurra (until April 2011), Liz Barnes, Zosia Beckles, Shona Burman-Roy, Lucinda Carr, Stephanie Cawker, Katherine Cullen, Elspeth Dixon, Paul Eunson (chair), Christina Gericke, Juliet Kenny, Moira A Mugglestone (from June 2011), M Stephen Murphy, Alec Musson, James Robb, Trudy Ward, Jane Williams.
Contributors: All authors contributed to the initial draft and revisions and approved the final version for publication. MAM is the guarantor.
Competing interests: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare: MAM and MSM have support from the National Institute for Health and Clinical Excellence for the submitted work; PE was a member of a physicians advisory panel for Medtronic until he was offered the position of chair of the NICE Guideline Development Group; none of the authors has other relationships or activities that could have appeared to have influenced the submitted work.
Provenance and peer review: Commissioned; not externally peer reviewed.