Cerebral palsy

Question 1

What is cerebral palsy?

Answer 1

Cerebral palsy (CP) is the name given to the permanent neurological problems resulting from damage to the brain around the time of birth. It is not a progressive condition, however the nature of the symptoms and problems may change over time during growth and development. There is huge variation in the severity and type of symptoms, ranging from completely wheelchair bound and dependent on others for all activities of daily living, to para-olympic athletes with only subtle problems with coordination or mobility.

Question 2

Causes of CP?

Answer 2

Antenatal:

Maternal infections
Trauma during pregnancy

Perinatal:

Birth asphyxia
Pre-term birth

Postnatal:

Meningitis
Severe neonatal jaundice
Head injury

Question 3

Types of CP?

Answer 3

Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones

Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.

Ataxic: problems with coordinated movement resulting from damage to the cerebellum

Mixed: a mix of spastic, dyskinetic and/or ataxic features

Spastic CP is also known as pyramidal CP. Dyskinetic CP is also known as athetoid CP and extrapyramidal CP.

Question 4

Patterns of spastic cerebral palsy?

Answer 4

Monoplegia: one limb affected
Hemiplegia: one side of the body affected
Diplegia: four limbs are affects, but mostly the legs
Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments

Question 5

Presentation of CP?

Answer 5

Children at risk of developing cerebral palsy, such as those with hypoxic-ischaemic encephalopathy, need to be followed up to identify any signs and symptoms that develop.

Signs and symptoms of cerebral palsy will become more evident during development:

Failure to meet milestones
Increased or decreased tone, generally or in specific limbs
Hand preference below 18 months is a key sign to remember for exams
Problems with coordination, speech or walking
Feeding or swallowing problems
Learning difficulties

Question 6

Neurological exam of CP?

Answer 6

You can gain a lot of information about a child from their gait:

Hemiplegic / diplegic gait: indicates an upper motor neurone lesion
Broad based gait / ataxic gait: indicates a cerebellar lesion
High stepping gait: indicates foot drop or a lower motor neurone lesion
Waddling gait: indicates pelvic muscle weakness due to myopathy
Antalgic gait (limp): indicates localised pain

Patients with cerebral palsy may have a hemiplegic or diplegic gait. This gait is caused by increased muscle tone and spasticity in the legs. The leg will be extended with plantar flexion of the feet and toes. This means they have to swing the leg around in a large semicircle when moving their leg from behind them to in front. There is not enough space to swing the extended leg in a straight line below them.

They will have signs of an upper motor neurone lesion, with good muscle bulk, increased tone, brisk reflexes and slightly reduced power. Power may be normal. Look for athetoid movements that indicate extrapyramidal (basal ganglia) involvement. Test for coordination to look for cerebellar involvement.

TOM TIP: Get used to assessing and recognising the patterns of upper and lower motor neurone lesions. Cerebral palsy is a perfect condition for examiners to bring to OSCEs, because signs are reliable and patients are stable. The differential diagnosis of an upper motor neurone lesion is acquired brain injury or a tumour.

Question 7

Complications + associated conditions CP?

Answer 7

Learning disability
Epilepsy
Kyphoscoliosis
Muscle contractures
Hearing and visual impairment
Gastro-oesophageal reflux

Question 8

Management CP?

Answer 8

Cerebral palsy is a permanent condition with problems that are life-long. It cannot be cured, but there are many ways to manage the symptoms and maximise function. The focus is on supporting the patient to achieve the most fulfilled and independent life. When asked how you would manage complex conditions such as cerebral palsy the answer should always start with “management will involve a multi-disciplinary team approach”.

Physiotherapy is used to stretch and strengthen muscles, maximise function and prevent muscle contractures.

Occupational therapy is used to help patients manage their everyday activities, such as getting dressed and using the bathroom. That can involve techniques to perform tasks despite disability. They can also make adaptations and supply equipment, such as rails for assistance or fitting a hoist for a patient who is entirely wheelchair bound.

Speech and language therapy can help with speech and swallowing. When swallowing difficultly prevents them meeting their nutritional requirements they may require an NG tube or PEG tube to be fitted.

Dieticians can help ensure they meet nutritional requirements. Some children may require PEG feeding through a port on their abdomen that gives direct access to the stomach.

Orthopaedic surgeons can perform procedures to release contractures or lengthen tendons (tenotomy).

Paediatricians will regularly see the child to optimise their medications. This may involve:

Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures
Anti-epileptic drugs for seizures
Glycopyrronium bromide for excessive drooling
Social workers to help with benefits and support.

Charities and support groups provide opportunities to connect with others affected by cerebral palsy and learn and share information on the condition.