Intended for healthcare professionals

Practice Guidelines

Cerebral palsy in adults: summary of NICE guidance

BMJ 2019; 364 doi: https://doi.org/10.1136/bmj.l806 (Published 19 March 2019) Cite this as: BMJ 2019;364:l806
  1. Nathan Bromham, senior systematic reviewer1,
  2. Katharina Dworzynski, guideline lead1,
  3. Paul Eunson, chair of Guideline Committee and consultant paediatric neurologist2,
  4. Charlie Fairhurst, clinical advisor1 3
  5. on behalf of the Guideline Committee
  1. 1National Guideline Alliance, Royal College of Obstetricians and Gynaecologists, London NW1 4RG, UK
  2. 2Royal Hospital for Sick Children, Edinburgh EH9 1LF, UK
  3. 3Evelina London Children’s Hospital, London SE1 7EF
  1. Correspondence to: P Eunson

What you need to know

  • Offer an annual review by a healthcare professional with expertise in neurodisabilities (who might be an advanced nurse practitioner, rehabilitation physician, physiotherapist, or other) to people with cerebral palsy and complex needs

  • Consider referral to speech and language therapy services and/or occupational therapy services for an assessment of functional needs and individualised support

  • Discuss factors that may exacerbate spasticity or dystonia, such as bladder problems, constipation, emotional distress, pain, and medication changes

  • Assess for risk of fractures secondary to osteoporosis in adults with cerebral palsy and specific risk factors, such as a history of falls or low body mass index (BMI)

  • Offer a regular weight check and BMI measurement due to risk of both obesity and undernutrition

Cerebral palsy is a non-progressive (though not unchanging) disorder of movement and posture pathology associated with an impairment of the developing fetal or infant brain (up to 2 years old)—when the maturation of the brain is most rapid and therefore most at risk from damage from, for example, prematurity, hypoxia, or infection. The prevalence remains at around 2-3 per 1000 live births, and most affected children live well into adult life.1 It is described in terms of topography (unilateral or bilateral), predominant motor subtype (usually spasticity and/or dyskinesis/dystonia), and severity (such as the Gross Motor Functional Classification System (GMFCS) see box 1).

Box 1

Gross Motor Function Classification System (GMFCS)2

This five-level clinical classification system describes the gross motor function of people with cerebral palsy based on their self-initiated movement abilities. People assessed as level I are the most able, and people assessed as level V are dependent on others for all their mobility needs:

  • Level I—Walks without restrictions; limitations in more advanced gross motor skills

  • Level II—Walks without assistive devices; limitations walking outdoors and in the community

  • Level III—Walks with assistive devices; limitations walking outdoors and in the community

  • Level IV—Self-mobility with limitations; the person is transported or uses power mobility outdoors and in the community

  • Level V—Self-mobility is severely limited even with the use of assistive technology.

  • The GMFCS is not validated for use in adults, but the GMFCS level at 12 years old is a good predictor of mobility into adulthood,3 especially at the milder and most severe levels. It is used in this guideline in the absence of a validated system for use in adults because it is readily understood by people with cerebral palsy, their families and carers, and health professionals involved in the care of adults with cerebral palsy.

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Adults with cerebral palsy have a wide range of abilities, from full independence in everyday life to needing 24 hour care. Their care and support needs depend on the severity of impairment and any comorbidities. They may continue to experience new symptoms and difficulties in adulthood because of progression of their musculoskeletal disorder, or the comorbidities associated with cerebral palsy. Adults with cerebral palsy want to participate in life as fully as possible, and the recommendations from this guideline encompass daily living skills, education, work, and leisure activities.

There is variability in how services for adults with cerebral palsy are provided. In many regions of the UK the management of cerebral palsy falls to specialists, but the management of that person’s comorbidities is up to primary care services. There are few specialists in adult neurology or rehabilitation who focus specifically on the long term needs of adults with cerebral palsy. There are, however, examples where a dedicated multidisciplinary team is in place to coordinate annual reviews for those with more complex neurological, physical, cognitive, or psychological comorbidity.

This guidance should help development of regional, equitable, cost effective services, with a clear network of referral from primary care to more specialised services as appropriate. This article summarises the most recent recommendations from the National Institute for Health and Care Excellence (NICE) on cerebral palsy in adults.4

What’s new in this guidance

  • For people with cerebral palsy and complex needs, offering an annual review of clinical and functional needs by a healthcare professional with expertise in neurodisabilities. For people with complex needs this would usually be a specialist rather than a general practitioner (GP). However, GPs will be involved in regular reviews (see below) for people with cerebral palsy

  • Emphasis on the changing health needs of older people with cerebral palsy, such as problems with bones and joints or respiratory disorders, anxiety and depression

  • Advice on how to address modifiable factors that may exacerbate spasticity and dystonia before further treatments are considered

Recommendations

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 Committee’s experience and opinion of what constitutes good practice. Evidence levels for the recommendations are given in italic in square brackets.

Access to services

Dependent on local pathways of care, it may be that support can be adequately provided in primary care settings, with regular review, but specialist evaluation will be necessary if the clinical impact of the movement disorder or comorbidity becomes more challenging to the individual.

  • Recognise and address any personal barriers to accessing primary and secondary care for adults with cerebral palsy. For example, this may involve:

    • Ensuring healthcare professionals have the skills and training to overcome communication difficulties

    • Providing treatment and support for mental health problems

    • Ensuring that the person has an advocate if needed

    • Providing support to help with social and emotional factors, such as fear of stigma, lack of motivation, and exhaustion.

      [Based on moderate quality qualitative evidence and the experience and opinion of the Guideline Committee (GC)]

  • Recognise and address any physical and organisational barriers to accessing primary and secondary care for adults with cerebral palsy. For example, this may involve:

    • Improving physical access to buildings

    • Providing advice and information on accessible transport services, such as local community transport services

    • Ensuring that appropriate equipment (for example, hoists and wheelchair weighing scales) and adequate changing and toilet facilities are available

    • Extending appointment times if needed.

      [Based on moderate quality qualitative evidence and the experience and opinion of the GC]

  • Give information to people with cerebral palsy about national screening services (for example, breast, colon, and cervical cancer screening) and encourage them to attend screening appointments. Explain that screening services will have to make arrangements to provide screening services that are accessible to people with cerebral palsy. [Based the experience and opinion of the GC]

  • Explain to the person with cerebral palsy and their family members and carers their right to a care and support needs assessment, in line with the Care Act 2014 and discuss with them the type of support available. [Based the experience and opinion of the GC]

  • Refer adults with cerebral palsy to a multidisciplinary team experienced in the management of neurological impairments if:

    • Their ability to carry out their usual daily activities deteriorates or

    • A neurosurgical or orthopaedic procedure is being considered that may affect their ability to carry out their usual daily activities.

      [Based the experience and opinion of the GC]

  • Recognise that re-assessment by this multidisciplinary team may be needed by adults with cerebral palsy at different points in their lives to ensure that their changing needs are met (for example, pregnancy and parenting, decreased mobility due to hip arthritis, and loss of care and support from a parent). [Based the experience and opinion of the GC]

The guideline states that commissioners and service providers should develop local pathways for the specialist care needed by adults with cerebral palsy and share this information.

  • Ensure that adults with cerebral palsy, their families and carers, and their primary care teams are provided with information about their local network of specialist services. [Based the experience and opinion of the GC]

Ongoing care

  • Consider regular reviews for adults with cerebral palsy, tailored to their needs and preferences. Agree with the person the frequency of review and which services should be involved based on their needs and preferences. [Based on very low quality evidence from a quantitative study, a health economic model, and the experience and opinion of the GC]

  • Offer an annual review of the person’s clinical and functional needs carried out by a healthcare professional with expertise in neurodisabilities for people with cerebral palsy who have complex needs (such as the Gross Motor Function Classification System (GMFCS) levels IV and V (see box 1 for definition)) and any of the following:

    • Communication difficulties

    • Learning disabilities

    • Living in long term care settings

    • Living in the community without sufficient practical and social support (for example, being cared for by elderly, frail parents)

    • Multiple comorbidities.

      [Based on very low quality evidence from a quantitative study, a health economic model, and the experience and opinion of the GC]

  • If an adult with cerebral palsy chooses not to have regular reviews, offer the person (and their family and carers, if agreed) information on when to contact a healthcare professional and how to access the specialist services that they may need. Ensure that the person’s GP and multidisciplinary team are aware that they do not want to be reviewed regularly (with the person’s permission). [Based on the experience and opinion of the GC]

Function and participation

Speech and communication needs may change over time and with social circumstances or care arrangements. Those with new difficulties may need referral to speech and language therapy services.

Adults with cerebral palsy may benefit from referral to occupational therapy services for an assessment of functional needs and individualised support and referral to services providing information, assessment, and provision of electronic assistive technology. This might be prompted, for example, by a change in the home or work environment.

Physical activity to maintain general fitness and physical and mental health is important to those with cerebral palsy as it is to the general population.

Managing abnormal muscle tone

Adults with cerebral palsy may experience decreased mobility because of a number of factors, including muscle tone, weakness, and pain.

  • At every review discuss with the person with cerebral palsy (and their family and carers, if agreed) factors that may exacerbate their spasticity or dystonia, such as:

    • Bladder problems (for example, urinary tract infection or bladder stones)

    • Constipation

    • Emotional distress

    • Pain

    • Posture

    • Pressure sores

    • Changes in home or work environments, including seating

    • Medication changes and side effects.

      [Based on the experience and opinion of the GC]

Beyond considering exacerbating factors and the use of baclofen to treat spasticity, most medication to manage abnormal muscle tone will be initiated and optimised by specialist movement disorder teams. However, the supervision and longer term prescribing of these medicines will generally be managed under shared care arrangements with local primary care services. Figure 1 summarises the guidance on managing spasticity or dystonia causing functional impairment, pain, or spasms.

Fig 1
Fig 1

Managing spasticity or dystonia causing functional impairment, pain, or spasms

Monitoring and assessing complications and comorbidities

Complications and comorbid symptoms can affect participation and quality of life of people with cerebral palsy and are a high priority for management.

Osteoporosis and fracture risk

Low bone mineral density is common in adults with cerebral palsy, with a prevalence of 10% in 31-40 year olds.5 Risk factors include reduced mobility or reduced weight bearing, those taking anticonvulsants or proton pump inhibitors, and those who have had a previous low-impact fracture. The guideline refers readers to NICE guideline on osteoporosis6 for more advice.

  • Consider assessing for risk of fractures secondary to osteoporosis in adults with cerebral palsy. Risk factors to assess include:

    • Needing help with moving or having to be moved, such as hoisting

    • History of falls

    • Low body mass index (BMI)

    • History of low-impact fractures

    • Other medical factors, such as steroid use, that may adversely affect bone health.

      [Based on very low quality evidence from quantitative studies and the experience and opinion of the GC]

Bone and joint disorders

Adults with cerebral palsy are more likely to have bone and joint disorders due to abnormal musculoskeletal development. For example, the prevalence of osteoarthritis is 13% in 31-40 year olds.5

  • Refer adults with cerebral palsy to a specialist orthopaedic or musculoskeletal service if a bone or joint disorder is suspected and is causing pain or affecting posture or function. These may include:

    • Osteoarthritis

    • Cervical instability or spondylosis (including athetosis)

    • Spinal deformity (including scoliosis, kyphosis, and lordosis)

    • Subluxation of the hips, wrist, and shoulders

    • Biomechanical knee problems

    • Abnormalities of the foot structure.

      [Based on the experience and opinion of the GC]

Mental health problems

Healthcare services for adults with cerebral palsy tend to focus on physical rather than mental health, but physical or communication problems can affect emotional wellbeing and negatively affect mental health. There is evidence of increased risk of developing depression and anxiety in this group.7

  • Identify and address mental health problems alongside physical health problems. Recognise that the impact of mental health problems and emotional difficulties can be as important as physical health problems for adults with cerebral palsy. [Based on the experience and opinion of the GC]

Difficulties with eating and nutritional problems

Obesity rates range from 5% to 41% in adults with cerebral palsy8 and will have an impact on mobility and independence. The risks of undernutrition in dyskinetic cerebral palsy and those severely impaired is well recognised: 58% of institutionalised adults were undernourished in one study.9 This has been partly addressed by more access to gastrostomy feeds and supplementing diets.

  • Offer adults with cerebral palsy a regular weight check and BMI or another anthropometric measurement, and identify people who may be at risk of undernutrition or obesity (see also the NICE guideline on obesity identification, assessment, and management10). [Based on moderate to low quality evidence from quantitative studies and the experience and opinion of the GC]

Respiratory disorders

Adults with cerebral palsy are at increased risk of respiratory failure.

  • Recognise that some risk factors for respiratory impairment are more common in adults with severe cerebral palsy (GMFCS level IV or V), such as:

    • Aspiration pneumonia

    • Chronic cardiorespiratory disorders (for example, cor pulmonale or pulmonary circulation hypertension)

    • Chronic suppurative lung disease

    • Kyphoscoliosis

    • Poor saliva control

    • Recurrent chest infections.

      [Based on the experience and opinion of the GC]

  • Offer vaccinations to adults with cerebral palsy and their carers, in line with the national immunisation programme (see the Green Book for further information). [Based on the experience and opinion of the GC]

  • Do not offer prophylactic antibiotics for lower respiratory tract infections in adults with cerebral palsy unless:

    • The person is at high risk of respiratory impairment and

    • They are offered on the advice of a respiratory specialist with expertise in neurodisability management.

      [Based on the experience and opinion of the GC]

Dysphagia is a common cause of recurrent chest infections in adults with cerebral palsy.

  • Refer adults with cerebral palsy and recurrent chest infections, if dysphagia is suspected, to a speech and language therapist with training in dysphagia to assess swallowing. [Based on the experience and opinion of the GC]

Pain

  • Be aware that some adults with cerebral palsy have difficulty communicating, or are unable to communicate, that they are in pain. [Based on the experience and opinion of the GC]

  • Assess for the presence, severity, and location of pain in adults with cerebral palsy using pain assessment tools such as:

    • Numerical rating scales

    • Visual analogue scales

    • Faces pain scales

    • Body maps.

      [Based on moderate to low quality evidence from quantitative studies and the experience and opinion of the GC]

Challenges to implementation

The main challenges to the implementation of this guidance for specialist services are

  • Service organisation for networks of care is variable: changes to practice will depend on the availability of services within a particular local area.

  • The limited number of healthcare professionals with expertise in neurological disorders. Training will be needed to ensure healthcare professionals can manage referrals rapidly.

  • Annual reviews for adults with less severe impairment, who are not currently receiving them, will increase costs. However, the GC (supported by a cost effectiveness analysis) thought this will be offset by the benefits of more frequent checks.

Future research

The Guideline Committee prioritised the following research recommendations:

  1. Is guided botulinum toxin type A injection using electrical localisation of muscles more clinically and cost effective than ultrasound guided injections or clinical positioning for localisation of injections in treating focal spasticity in adults with cerebral palsy?

  2. What is the clinical and cost effectiveness of selective dorsal rhizotomy compared with intrathecal baclofen to reduce spasticity in adults with cerebral palsy?

  3. Can detection and management of respiratory disorders in adults with cerebral palsy be improved in primary and community care?

  4. Are prophylactic antibiotics clinically and cost effective in the management of respiratory symptoms in adults with cerebral palsy with significant respiratory comorbidity?

  5. What is the optimum regime for splints applied to the upper limb in adults with cerebral palsy to improve or maintain posture or function?

  6. Are augmentative and alternative communication systems clinically and cost effective in promoting communication for adults with cerebral palsy who have communication difficulties?

How patients were involved in the creation of this article

Committee members involved in this guideline included two adults with cerebral palsy and a parent of a young adult with cerebral palsy who contributed to the formulation of the recommendations summarised here. They were not involved in preparing this summary article.

Further information on the guidance

Methods

This guidance was developed by the National Guideline Alliance in accordance with NICE guideline methodology (www.nice.org.uk/media/default/about/what-we-do/our-programmes/developing-nice-guidelines-the-manual.pdf). A guideline committee (GC) was established by the National Guideline Alliance, which incorporated healthcare and allied healthcare professionals (two consultants in rehabilitation medicine, two consultant neurologists, one nurse, one occupational therapist, one general practitioner, one speech and language therapist, one orthopaedic surgeon, one consultant in respiratory medicine, one consultant geriatrician) and three lay members.

The GC identified relevant review questions and collected and appraised clinical and cost effectiveness evidence. Quality ratings of the evidence were based on GRADE methodology (www.gradeworkinggroup.org). These relate to the quality of the available evidence for assessed outcomes or themes rather than the quality of the study. The GC agreed recommendations for clinical practice based on the available evidence or, when evidence was not found, based on their experience and opinion using informal consensus methods.

The scope and the draft of the guideline went through a rigorous reviewing process, in which stakeholder organisations were invited to comment; the GC took all comments into consideration when producing the final version of the guideline.

NICE will conduct regular reviews after publication of the guidance, to determine whether the evidence base has progressed significantly enough to alter the current guideline recommendations and require an update.

Acknowledgments

The members of the Guideline Committee were (shown alphabetically): Umesh Chauhan, Stephen Bianchi, Lloyd Bradley, Richard Bunn, Elspeth Dixon, Paul Eunson, Susan Hourihan, Mohammed Jeewa, Ruth Kent, Andrew Roberts, Jill Scarisbrick, John Starr, Valerie Stevenson, Duncan Walsh, Rosie Werner.

The members of the National Guideline Alliance technical team were (shown alphabetically): Offiong Ani, Sabine Berendse, Nathan Bromham, Katharina Dworzynski, Charlie Fairhurst, James Hawkins, Jose Marcano-Belisario, Gemma Marceniuk, Fionnuala O'Brien, Anuja Pandey, Ferruccio Pelone, Victoria Rowland, Gemma Simons.

Footnotes

  • Contributors: All authors contributed to the initial draft of this article, helped revise the manuscript, and approved the final version for the publication.

  • Funding: NB and KD are employees of the National Guideline Alliance, which is commissioned and funded by NICE to develop clinical guidelines and write this BMJ summary. No authors received special funding from any other source to write this summary.

  • Competing interests: We declared the following interests based on NICE’s policy on conflicts of interests (www.nice.org.uk/Media/Default/About/Who-we-are/Policies-and-procedures/declaration-of-interests-policy.pdf). PE: provides medico-legal reports (which may include giving an opinion on life expectancy with children with cerebral palsy); trustee of Castang Foundation, a charity that supports research and management of cerebral palsy; has published a paper on long term and social impact of hypoxic-ischaemic encephalopathy; and completed an unpaid interview with an American pharmaceutical company about improvements in outcomes for 0-6 year olds. CF: was the chair for the NICE clinical guideline “Cerebral palsy in under 25s: assessment and management”; trustee of Whizz Kidz, a charity that funds powered mobility aids for children.

  • Disclaimer: The views expressed in this publication are those of the authors and not necessarily those of NICE.

References