In most cases, brain injury occurs during pregnancy. Except in its mildest forms, CP can become evident in the first 12-18 months. Approximately 40% of people with CP present with spastic hemiplegia.

In January, the National Institute for Health and Care Excellence (NICE), published their first guideline on the assessment and management of patients with CP. The new guideline focuses on patients under the age of 25.

Risk factors

According to the NICE guideline, the following are independent risk factors for CP:

Antenatal factors: Preterm birth (risk increases with decreasing gestational age), chorioamnionitis, maternal respiratory tract or genito-urinary infection treated in hospital.

Perinatal factors: Low birth weight, chorioamnionitis, neonatal encephalopathy,
neonatal sepsis (particularly with a birth weight below 1.5 kg), maternal respiratory tract or genito-urinary infection treated in hospital.

Postnatal factors: Meningitis.

Causes of cerebral palsy

The following brain abnormalities have been seen in children with CP based on magnetic resonance imaging:

45% caused by white matter damage: More common in children born preterm than in those born at term. May occur in children with any functional level or motor subtype, but is more common in spastic than in dyskinetic CP.

10% caused by vasal ganglia or deep grey matter damage: Mostly associated with dyskinetic CP.

10% caused by congenital malformation: More common in children born at term than in those born preterm. Associated with higher levels of functional impairment than other causes.

7% caused by focal infarcts: Neonatal encephalopathy can result from various pathological events, such as a hypoxic–ischaemic brain injury or sepsis. If there has been more than one such event, they may interact to cause damage to the developing brain.

Symptoms and signs of CP

According to the Australian Cerebral Palsy Alliance, signs and symptoms of CP include:

Babies

  • Low muscle tone
  • Unable to hold up his/her own head while lying on his/her stomach or in a supported sitting position
  • Muscle spasms or feeling stiff
  • Poor muscle control, reflexes and posture
  • Delayed development (can’t sit up or independently roll over by six months)
  • Feeding or swallowing difficulties
  • Prefers to use one side of their body.

Toddlers/children

Although the brain of toddlers/children with CP remains injured, the injury does not get worse as they develop. Depending on the level of severity of CP, toddlers/children may experience difficulties with physical development such as:

  • Not walking by 12-18 months
  • Not speaking simple sentences by 24 months.

Management

Although there is no cure, problems associated with CP such as swallowing and communication difficulties, saliva control, pain, as well as psychiatric conditions can be managed effectively.

NICE recommends the following:

Eating, drinking and swallowing difficulties

Do not use videofluoroscopy or fibroscopic endoscopy for the initial assessment of eating, drinking and swallowing difficulties in children and young people with CP. Consider videofluoroscopy only if any of the following apply:

  • Uncertainty about the safety of eating, drinking and swallowing
  • The patient has recurrent chest infection without overt clinical signs of aspiration
  • Deterioration in eating, drinking and swallowing ability with increasing age (particularly after adolescence)
  • Uncertainty about the impact of modifying food textures (for example, use of thickeners or pureeing).

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
  • Training needs of the people who care for the patient, particularly outside the home
  • Advise parents or carers that intra-oral devices have not been shown to improve eating, drinking and swallowing.

Speech, language and communication

Communication difficulties occur in around one in two people with CP. In addition, at least one in 10 need augmentative and alternative communication (signs, symbols and speech generating devices) and another one 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 CP. Communication difficulties do not necessarily correlate with learning/intellectual disability.

Interventions to improve speech include posture, breath control, voice production and rate of speech in patients who have a motor speech disorder and some intelligible speech and for whom speech is the primary means of communication, as well as those who can take an active part in interventions.

Augmentative and alternative communication systems should be considered for those 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.

Optimising nutritional status

Nutritional status should be monitored regularly and include measuring the patient’s height and weight. If height and weight cannot be measured, consider alternative anthropometric measurements. Provide timely access to assessment and nutritional interventional support from a dietitian if there are concerns about oral intake, growth or nutritional status.

If oral intake is still insufficient to provide adequate nutrition after assessment and nutritional interventions, refer the child or young person to be assessed for enteral tube feeding.

Managing saliva control

Assess factors that may affect drooling such as positioning, medication history, reflux and dental issues, before starting drug therapy. To reduce the severity and frequency of drooling in children and young people with cerebral palsy, consider the use of anticholinergic medication:

  • Glycopyrronium bromide (oral or by enteral tube)
  • Transdermal hyoscine hydrobromide
  • Trihexyphenidyl hydrochloride for children with dyskinetic CP.

Consider the use of botulinum toxin A injections to the salivary glands with ultrasound guidance to reduce the severity and frequency of drooling if anticholinergic drugs provide insufficient benefit or are not tolerated.

Consider referring young people for a surgical opinion, after an assessment confirming clinically safe swallow, if there is:

  • A potential need for lifelong drug treatment
  • Insufficient benefit or non-tolerance of anticholinergic drugs and botulinum toxin A injections.

Low bone mineral density

The following are independent risk factors for low bone mineral density:

  • Non-ambulant (Gross Motor Function Classification System 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 second centile)
  • History of low-impact fracture
  • Use of anticonvulsant medication.

There is an increased risk of low-impact fractures in patients who are non-ambulant or have low bone mineral density. Inform the patient and their parents or carers if they are at an increased risk of low-impact fractures.

Pain, discomfort and distress

Pain is common in people with CP, especially those with more severe motor impairment. The following common conditions cause pain, discomfort and distress:

  • 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.

Common types of pain in children and young people with CP include:

  • Non-specific back pain
  • Headache
  • Non-specific abdominal pain
  • Dental pain
  • Dysmenorhea

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.

Identify pain or assess severity using for e.g. the paediatric pain profile, the non-communicating Children’s Pain Checklist – the postoperative version or the numeric pain rating scale. For reversible causes of pain, discomfort and distress, treat the cause as appropriate using targeted interventions.

In the absence of an identifiable cause of pain, discomfort or distress 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 physician specialising in pain management.

Sleep disturbances

Sleep disturbances such as difficulties falling or staying asleep, or daytime sleepiness are common in patients with CP and may be caused by factors such as the environment, hunger and thirst. The most common condition-specific causes of sleep 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.

Interventions should be aimed at optimising sleep hygiene and managing treatable causes of sleep disturbances. If no treatable cause is found, consider a trial of melatonin to manage sleep disturbances, particularly for problems with falling asleep.

Regular sedative medication should only be prescribed following specialist consultation. Do not offer sleep positioning systems solely to manage primary sleep. Refer the patient to specialist sleep services for multidisciplinary team assessment and management if there are ongoing sleep disturbances.

Mental health problems

People with CP have an increased prevalence of mental health and psychological problems, including depression, anxiety and conduct disorders, behaviours that challenge, triggered by pain, discomfort or sleep disturbances, neurodevelopmental disorders, including autism spectrum disorder and attention deficit hyperactivity disorder.

Refer the patient for specialist psychological assessment and ongoing management if emotional and behavioural difficulties persist or there are concerns about their mental health. When developing an individual management plan to address the mental health needs of a patient, 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.

Registering and processing sensory information

Learning and movement problems may be exacerbated by difficulties with registering or processing sensory information, which can affect function and participation. Sensory difficulties may include:

  • Primary sensory disorders in any of the sensory systems, such as processing of visual or auditory information (for example, difficulties with depth perception may affect the ability to walk on stairs)
  • Disorders of sensory processing and perception, such as planning movements or being able to concentrate and pay attention

Develop a functional, goal-orientated, individualised programme in partnership with parents or carers to address these problems and explain that there is a lack of evidence to support specific interventions.

Visual impairment

About one in two patients with CP will have some form of visual impairment. Visual impairment may occur with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment. Refer patients for regular ophthalmological and orthoptic assessment at the time of diagnosis.

Different types of visual impairment are associated with CP. These include one or more of the following:

  • Problems controlling eye movements
  • Strabismus
  • Refractive errors.
  • Eye function problems, including retinopathy of prematurity
  • Cerebral visual information processing impairment
  • Visual field defects.

Hearing impairment

Hearing impairment occurs in around one in 10 people with CP with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment. Hearing impairment is more common in people with dyskinetic or ataxic cerebral palsy than in those with spastic CP. Regular ongoing hearing assessment is necessary.

Learning disability

Learning disability (IQ below 70) occurs in around one in two patients, while severe learning disability (IQ below 50) occurs in around one in four. Example of learning disabilities include problems with knowledge acquisition, memory, and understanding and use of language. Learning disability can be associated with any functional level, but prevalence increases with increasing severity of motor impairment.

Behavioural difficulties

Around two to three in 10 children and young people with CP have one or more of the following:

  • Emotional and behavioural difficulties that affect the patients function
  • Problems with peer relationships
  • Difficulties with attention, concentration and hyperactivity
  • Conduct behavioural difficulties.

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

Vomiting, regurgitation and reflux

Vomiting, regurgitation and gastro-oesophageal reflux are common in people with CP. If there is a marked change in the pattern of vomiting, assess for a clinical cause.

Constipation

Around three in five children and young people with CP have chronic constipation. Carry out regular clinical assessments for constipation.

Epilepsy

Around one in three children with CP may have epilepsy and it may occur with any functional level or motor subtype, but prevalence increases with increasing severity of motor impairment. Make sure that dyskinetic movements are not misinterpreted as epilepsy.

Reference:
NICE guideline. Cerebral palsy in under 25s: assessment and management. https://www.nice.org.uk/guidance/NG62.