Cerebral palsy in under 25s: assessment and management

Offer MRI to investigate aetiology in a child or young person with suspected or known cerebral palsy if this is not clear from:

• antenatal, perinatal and postnatal history

• their developmental progress

• findings on clinical examination

• results of cranial ultrasound examinations.

Recognise that MRI will not accurately establish the timing of a hypoxic–ischaemic brain injury in a child with cerebral palsy.

When deciding the best age to perform an MRI scan for a child with cerebral palsy, take account of the following:

• Subtle neuro-anatomical changes that could explain the aetiology of cerebral palsy may not be apparent until 2 years of age.

• The presence of any red flags for a progressive neurological disorder (see the section on red flags for other neurological disorders).

• That general anaesthesia or sedation is usually needed for young children having MRI.

• The views of the child or young person and their parents or carers.

Explain to parents or carers and the child or young person with cerebral palsy that it is not always possible to identify a cause for cerebral palsy.

Consider repeating the MRI scan if:

• there is a change in the expected clinical and developmental profile or

• any red flags for a progressive neurological disorder (see the section on red flags for other neurological disorders).

Discuss with the child or young person and their parents or carers the reasons for performing MRI in each individual circumstance.

For guidance on the safe and effective use of medicines, see the NICE guideline on medicines optimisation.

For guidance on managing problems with movement and posture in children and young people with cerebral palsy, see the NICE guideline on spasticity in under 19s.

Do not rely on MRI alone for predicting prognosis in children with cerebral palsy.

Take account of the likely cause of cerebral palsy and the findings from MRI (if performed) when discussing prognosis with the child or young person and their parents or carers.

If eating, drinking and swallowing difficulties are suspected in a child or young person with cerebral palsy, carry out a clinical assessment as first-line investigation to determine the safety, efficiency and enjoyment of eating and drinking. This should include:

• taking a relevant clinical history, including asking about any previous chest infections

• observation of eating and drinking in a normal mealtime environment by a speech and language therapist with training in assessing and treating dysphagia.

Refer the child or young person to a local specialist multidisciplinary team with training in assessing and treating dysphagia if there are clinical concerns about eating, drinking and swallowing, such as:

• coughing, choking, gagging, altered breathing pattern or change in colour while eating or drinking

• recurrent chest infection

• mealtimes regularly being stressful or distressing for the child or young person or their parents or carers

• prolonged meal duration.

Do not use videofluoroscopy or fibroscopic endoscopy for the initial assessment of eating, drinking and swallowing difficulties in children and young people with cerebral palsy.

The specialist multidisciplinary team should consider videofluoroscopy if any of the following apply:

• There is uncertainty about the safety of eating, drinking and swallowing after specialist clinical assessment.

• The child or young person has recurrent chest infection without overt clinical signs of aspiration.

• There is deterioration in eating, drinking and swallowing ability with increasing age (particularly after adolescence).

• There is uncertainty about the impact of modifying food textures (for example, use of thickeners or pureeing).

• Parents or carers need support to understand eating, drinking and swallowing difficulties, to help with decision making.

Videofluoroscopy should only be performed in a centre with a specialist multidisciplinary team who have experience and competence in using it with children and young people with cerebral palsy.

Do not routinely perform videofluoroscopy when considering starting enteral tube feeding in children and young people with cerebral palsy.

Ensure that children and young people with ongoing eating, drinking and swallowing difficulties have access to tertiary specialist assessment, including advice from other services (such as paediatric surgery and respiratory paediatrics).

Develop strategies and goals in partnership with the child or young person with cerebral palsy and their parents, carers and other family members for interventions to improve eating, drinking and swallowing.

Create an individualised plan for managing eating, drinking and swallowing difficulties in children and young people with cerebral palsy, taking into account the understanding, knowledge and skills of parents, carers and any other people involved in feeding the child or young person. Assess the role of the following:

• postural management and positioning when eating

• modifying fluid and food textures and flavours

• feeding techniques, such as pacing and spoon placement

• equipment, such as specialised feeding utensils

• optimising the mealtime environment

• strategies for managing behavioural difficulties associated with eating and drinking

• strategies for developing oral motor skills

• communication strategies

• modifications to accommodate visual or other sensory impairments that affect eating, drinking and swallowing

• the training needs of the people who care for the child or young person, particularly outside the home.

Advise parents or carers that intra-oral devices have not been shown to improve eating, drinking and swallowing in children and young people with cerebral palsy.

Use outcome measures important to the child or young person and their parents or carers to review:

• whether individualised goals have been achieved

• the clinical and functional impact of interventions to improve eating, drinking and swallowing.

Talk to children and young people and their parents or carers about communication difficulties that can be associated with cerebral palsy. Information that may be useful to discuss includes the following:

• communication difficulties occur in around 1 in 2 children and young people with cerebral palsy

• at least 1 in 10 need augmentative and alternative communication (signs, symbols and speech generating devices)

• around 1 in 10 cannot use formal methods of augmentative and alternative communication because of cognitive and sensory impairments and communication difficulties

• communication difficulties may occur with any functional level or motor subtype, but are more common in children and young people with dyskinetic or severe bilateral spastic cerebral palsy

• communication difficulties do not necessarily correlate with learning disability (intellectual disability).

Regularly assess children and young people with cerebral palsy during routine reviews to identify concerns about speech, language and communication, including speech intelligibility.

Refer children and young people with cerebral palsy for specialist assessment if there are concerns about speech, language and communication, including speech intelligibility.

Specialist assessment of the communication skills, including speech intelligibility, of children and young people with cerebral palsy should be conducted by a multidisciplinary team that includes a speech and language therapist.

Recognise the importance of early intervention to improve the communication skills of children and young people with cerebral palsy.

Offer interventions to improve speech intelligibility, for example targeting posture, breath control, voice production and rate of speech, to children and young people with cerebral palsy:

• who have a motor speech disorder and some intelligible speech and

• for whom speech is the primary means of communication and

• who can engage with the intervention.

Consider augmentative and alternative communication systems for children and young people with cerebral palsy who need support in understanding and producing speech. These may include pictures, objects, symbols and signs, and speech generating devices.

If there are ongoing problems with using augmentative and alternative communication systems, refer the child or young person to a specialist service in order to tailor interventions to their individual needs, taking account of their cognitive, linguistic, motor, hearing and visual abilities.

Regularly review children and young people who are using augmentative and alternative communication systems, to monitor their progress and ensure that interventions continue to be appropriate for their needs.

Provide individualised training in communication techniques for families, carers, preschool and school staff and other people involved in the care of a child or young person with cerebral palsy.

Recognise that in children and young people with cerebral palsy the following are independent risk factors for low bone mineral density:

• non-ambulant (GMFCS level IV or V)

• vitamin D deficiency

• presence of eating, drinking and swallowing difficulties or concerns about nutritional status

• low weight for age (below the 2nd centile)

• history of low-impact fracture

• use of anticonvulsant medication.

Recognise that there is an increased risk of low-impact fractures in children and young people with cerebral palsy who are non-ambulant or have low bone mineral density.

Inform children and young people with cerebral palsy and their parents or carers if they are at an increased risk of low-impact fractures.

If a child and young person with cerebral palsy has 1 or more risk factors for low bone mineral density (see recommendation 1.12.1):

• assess their dietary intake of calcium and vitamin D and

• consider the following laboratory investigations of calcium and vitamin D status:

  • serum calcium, phosphate and alkaline phosphatase

  • serum vitamin D

  • urinary calcium/creatinine ratio.

Create an individualised care plan for children and young people with cerebral palsy who have 1 or more risk factors for low bone mineral density (see recommendation 1.12.1).

Consider the following as possible interventions to reduce the risk of reduced bone mineral density and low-impact fractures:

• an active movement programme

• active weight bearing

• dietetic interventions as appropriate, including nutritional support and calcium and vitamin D supplementation

• minimising risks associated with movement and handling.

Consider a DEXA scan under specialist guidance for children and young people with cerebral palsy who have had a low-impact fracture.

Refer children and young people with cerebral palsy with reduced bone density and a history of low-impact fracture to a specialist centre for consideration of bisphosphonate therapy.

Do not offer standing frames solely to prevent low bone mineral density in children and young people with cerebral palsy.

Do not offer vibration therapy solely to prevent low bone mineral density in children and young people with cerebral palsy.

Explain to children and young people with cerebral palsy and their parents or carers that pain is common in people with cerebral palsy, especially those with more severe motor impairment, and this should be recognised and addressed.

Recognise that common condition-specific causes of pain, discomfort and distress in children and young people with cerebral palsy include:

• musculoskeletal problems (for example, scoliosis, hip subluxation and dislocation)

• increased muscle tone (including dystonia and spasticity)

• muscle fatigue and immobility

• constipation

• vomiting

• gastro-oesophageal reflux disease.

Recognise that usual causes of pain, discomfort and distress that affect children and young people generally also occur in those with cerebral palsy, and that difficulties with communication and perception may make identifying the cause more challenging. Common types of pain in children and young people include:

• non-specific back pain

• headache

• non-specific abdominal pain

• dental pain

• dysmenorrhea.

Take into account that parents and familiar carers have a key role in recognising and assessing pain, discomfort and distress in children and young people with cerebral palsy.

When assessing pain in children and young people with cerebral palsy:

• recognise that assessing the presence and degree of pain can be challenging, especially if:

  • there are communication difficulties or learning disability (intellectual disability)

  • there are difficulties with registering or processing sensory information (see the section on registering and processing sensory information)

• ask about signs of pain, discomfort, distress and sleep disturbances (see the section on sleep disturbances) at every contact

• recognise that pain-related behaviour can present differently compared with that in the wider population.

Assess for other possible causes of distress in the absence of identifiable physical causes of pain and discomfort, such as:

• psychological and emotional distress

• increased sensitivity to environmental triggers

• thirst or hunger.

Consider using tools to identify pain or assess severity of pain in children and young people with cerebral palsy, for example:

• For children and young people with communication difficulties:

  • Paediatric Pain Profile

  • Non-communicating Children's Pain Checklist – postoperative version.

• For children and young people without communication difficulties:

  • Numeric pain rating scale.

Refer the child or young person for a specialist multidisciplinary team assessment of pain, discomfort, distress and sleep if the cause of these is not clear after routine assessment.

For reversible causes of pain, discomfort and distress identified in children and young people with cerebral palsy, treat the cause as appropriate using targeted interventions in line with the following NICE guidelines:

• spasticity in under 19s

• constipation in children and young people

• gastro-oesophageal reflux disease in children and young people

• gastro-oesophageal reflux disease and dyspepsia in adults

• headaches in over 12s

• low back pain and sciatica in over 16s

• urinary incontinence in neurological disease

• urinary tract infection in under 16s.

For common interventions used in the management of cerebral palsy (such as physical therapies, botulinum toxin A injections and surgery) that can cause acute pain:

• advise the child or young person and their parents or carers that these interventions may reduce discomfort in the long term

• minimise discomfort during these procedures.

In the absence of an identifiable cause of pain, discomfort or distress in a child or young person with cerebral palsy:

• take into account the impact of anxiety, depression or other possible mental health problems

• consider a 'stepped approach' trial of simple analgesia (such as paracetamol and/or ibuprofen) for mild to moderate pain

• monitor the duration, pattern and severity of symptoms.

If a trial of analgesia is unsuccessful, refer the child or young person to a specialist pain multidisciplinary team, which may be a palliative care service, for a more detailed assessment.

Explain to parents or carers that, in children and young people with cerebral palsy, sleep disturbances (for example, difficulties with falling asleep, staying asleep or daytime sleepiness):

• are common

• may be caused by factors such as environment, hunger and thirst.

Recognise that the most common condition-specific causes of sleep disturbances in children and young people with cerebral palsy include:

• sleep-induced breathing disorders, such as obstructive sleep apnoea

• seizures

• pain and discomfort

• need for repositioning because of immobility

• poor sleep hygiene (poor night-time routine and environment)

• night-time interventions, including overnight tube feeding or the use of orthoses

• comorbidities, including adverse effects of medication.

When identifying and assessing sleep disturbances in children and young people with cerebral palsy:

• recognise that parents and familiar carers have the primary role in this

• consider using sleep questionnaires or diaries.

Always ask about pain, sleep and distress as part of any clinical consultation.

Optimise sleep hygiene for children and young people with cerebral palsy.

Manage treatable causes of sleep disturbances that are identified in children and young people with cerebral palsy.

If no treatable cause is found, consider a trial of melatonin to manage sleep disturbances for children and young people with cerebral palsy, particularly for problems with falling asleep.

At the time of publication (January 2017), melatonin did not have a UK marketing authorisation for use in children and young people under 18 for this indication. See NICE's information on prescribing medicines.

Do not offer regular sedative medication to manage primary sleep disorders in children with cerebral palsy without seeking specialist advice.

Do not offer sleep positioning systems solely to manage primary sleep disorders in children and young people with cerebral palsy.

Refer the child or young person to specialist sleep services for multidisciplinary team assessment and management if there are ongoing sleep disturbances.

Take into account that parents and familiar carers have a central role in recognising and assessing emotional difficulties and mental health problems in children and young people with cerebral palsy.

Recognise that children and young people with cerebral palsy have an increased prevalence of:

• mental health and psychological problems, including depression, anxiety and conduct disorders

• behaviours that challenge, which may be triggered by pain, discomfort or sleep disturbances

• neurodevelopmental disorders, including autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD).

Recognise that emotional and behavioural difficulties (for example, low self-esteem) are reported in up to 1 in 4 children and young people with cerebral palsy.

Any multidisciplinary team should:

• recognise that mental health problems and emotional difficulties can be as important as physical health problems for children and young people with cerebral palsy

• explore such difficulties during consultations

• recognise that assessing psychological problems can be challenging in children and young people with communication difficulties or learning disability (intellectual disability).

Think about and address the following contributory factors if a change in emotional state occurs in a child or young person with cerebral palsy:

• pain or discomfort (see the section on pain, discomfort and distress)

• frustration associated with communication difficulties

• social factors, such as a change in home circumstances or care provision.

Use validated tools, such as the Child Health Questionnaire and the Strengths and Difficulties Questionnaire, to assess mental health problems in children and young people with cerebral palsy.

Refer the child or young person with cerebral palsy for specialist psychological assessment and ongoing management if emotional and behavioural difficulties persist or there are concerns about their mental health.

Work in partnership with the child or young person with cerebral palsy, and their parents and primary carers, when assessing and managing mental health problems and setting goals.

When making an individual management plan to address the mental health needs of a child or young person with cerebral palsy, take into account ways of providing support to parents or carers.

Recognise that there are specific challenges in managing and minimising the impact of mental health problems in children and young people with cerebral palsy. These include:

• communication difficulties

• comorbidities, particularly epilepsy and pain

• side effects and drug interactions of multiple medicines (polypharmacy)

• adverse effects of medicines used for managing mental health problems on motor function

• adverse effects of medicines used for managing motor function on mental health

• specific social care needs.

Refer all children with cerebral palsy for an initial baseline ophthalmological and orthoptic assessment at the time of diagnosis.

Talk to children and young people and their parents or carers about visual impairment that can be associated with cerebral palsy. Information that may be useful to discuss includes the following:

• around 1 in 2 children and young people with cerebral palsy will have some form of visual impairment

• visual impairment may occur in children and young people with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment.

Talk to children and young people and their parents or carers about the different types of visual impairment that can be associated with cerebral palsy. Explain that these could include 1 or more of the following:

• problems with controlling eye movements

• strabismus (squint)

• refractive errors (short or long sighted or distorted image)

• problems of eye function, including retinopathy of prematurity

• impaired cerebral visual information processing (problems with seeing objects caused by damage to the parts of the brain that control vision)

• visual field defects (loss of the part of usual field of vision).

If concerns about visual impairment are raised by parents, carers or members of the care team, consider referring the child or young person with cerebral palsy to a specialist team for evaluation of the whole visual system (including eye health, eye movements, refraction, squint and visual acuity), especially if there are communication difficulties.

Regularly assess children and young people with cerebral palsy for signs of cerebral visual impairment, bearing in mind that this:

• occurs in around 1 in 5 children and young people with cerebral palsy

• may occur in children and young people with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment

• may be difficult to recognise in the early stages.

Talk to children and young people and their parents or carers about hearing impairment that can be associated with cerebral palsy. Information that may be useful to discuss includes the following:

• hearing impairment occurs in around 1 in 10 children and young people with cerebral palsy

• it may occur in children and young people with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment

• it is more common in people with dyskinetic or ataxic cerebral palsy than in those with spastic cerebral palsy

• regular ongoing hearing assessment is necessary.

Talk to children and young people and their parents or carers about learning disability (intellectual disability) that can be associated with cerebral palsy (for example, problems with knowledge acquisition, memory, and understanding and use of language). Information that may be useful to discuss includes the following:

• learning disability (IQ below 70) occurs in around 1 in 2 children and young people with cerebral palsy

• severe learning disability (IQ below 50) occurs in around 1 in 4 children and young people with cerebral palsy

• learning disability can be associated with any functional level, but prevalence increases with increasing severity of motor impairment:

  • GMFCS level I or II: around 1 in 3 have an IQ below 70

  • GMFCS level III, IV or V: around 2 in 3 have an IQ below 70.

Talk to children and young people and their parents or carers about behavioural difficulties that can be associated with cerebral palsy. Information that may be useful to discuss includes that around 2 to 3 in 10 children and young people with cerebral palsy have 1 or more of the following:

• emotional and behavioural difficulties that have an effect on the child or young person's function and participation

• problems with peer relationships

• difficulties with attention, concentration and hyperactivity

• conduct behavioural difficulties.

Recognise that difficulties with registering or processing sensory information (see the section on registering and processing sensory information) may present as behavioural difficulties.

Support children and young people with cerebral palsy and their families and carers to recognise behavioural difficulties.

Manage routine behavioural difficulties within the multidisciplinary team, and refer the child or young person to specialist services if difficulties persist.

Advise parents or carers that vomiting, regurgitation and gastro-oesophageal reflux are common in children and young people with cerebral palsy. If there is a marked change in the pattern of vomiting, assess for a clinical cause.

For guidance on identifying and managing gastro-oesophageal reflux disease, see the NICE guidelines on gastro-oesophageal reflux disease in children and young people and gastro-oesophageal reflux disease and dyspepsia in adults.

Recognise that around 3 in 5 children and young people with cerebral palsy have chronic constipation, and:

• discuss this with children and young people and their parents or carers

• carry out regular clinical assessments for constipation.

For guidance on identifying and managing constipation in under 18s, see the NICE guideline on constipation in children and young people.

Advise children and their parents or carers that epilepsy may be associated with cerebral palsy. Information that may be useful to discuss includes the following:

• epilepsy occurs in around 1 in 3 children with cerebral palsy

• it may occur in children and young people with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment

• it is reported in around 1 in 2 children with dyskinetic cerebral palsy.

Ensure that dyskinetic movements are not misinterpreted as epilepsy in children with cerebral palsy.

For guidance on identifying and managing epilepsy, see the NICE guideline on epilepsies in children, young people and adults.

Recognise that challenges for young people with cerebral palsy continue into adulthood, and ensure that their individual developmental, social and health needs, particularly those relating to learning and communication, are addressed when planning and delivering transition.

Recognise that for young people with cerebral palsy, there may be more than 1 transition period in health and social care settings; for example, college, resident educational and adult home settings.

Develop clear pathways for transition that involve:

• the young person's GP and

• named paediatricians and named clinicians in adults' services, both locally and regionally, who have an interest in the management of cerebral palsy.

Ensure that professionals involved in providing future care for young people with cerebral palsy have sufficient training in order to address all their health and social care needs.

As a minimum standard of care, ensure that the young person has access to adults' services both locally and regionally that include healthcare professionals with an understanding of managing cerebral palsy.

Ensure that all relevant information is communicated at each point of transition; for example, using a personal 'folder' containing relevant information as described in recommendation 1.6.5 in the section on information and support (see also recommendations about support before transfer in the NICE guideline on transition from children's to adults' services).

Recognise that functional challenges (including those involving eating, drinking and swallowing, communication and mobility) and physical problems (including pain and discomfort) may change over time for people with cerebral palsy, and take this into account in transition planning.

Provide a named worker to facilitate timely and effective transition, and recognise the importance of continuity of care (see also recommendations about transition planning in the NICE guideline on transition from children's to adults' services and about continuity of care and relationships in the NICE guideline on patient experience in adult NHS services).