Cerebral Palsy
Definition
Cerebral palsy is an umbrella term for non-progressive, permanent motor disorders caused by: [Ref]
- Acquired brain injury (~90% of cases), or
- Abnormal brain development (~10-15% of cases)
- That occurred in the developing fetal / infant brain (i.e. occurred during the antenatal / perinatal / early postnatal period)
Cerebral palsy is usually multifactorial, with no single identifiable cause in most cases [Ref]
Cerebral palsy is strictly defined by non-progressive insults occurring during early fetal or infant brain development. [Ref]
Brain injuries sustained after this early developmental window (e.g. head injury or stroke in later childhood or adulthood) are classified as acquired brain injuries, not cerebral palsy. [Ref]
Causes and Risk Factors
| Period | Important causes / risk factors |
|---|---|
| Antenatal period (affecting the fetal brain) |
|
| Perinatal / neonatal period (around birth and early neonatal life) |
|
| Postnatal period (affecting the developing infant brain) |
NB Postnatally acquired cerebral palsy refers to brain injury occurring during early brain development, rather than neurological injury occurring later outside this developmental period. Epidemiological definitions commonly use <2 y/o as the upper limit for post-neonatally acquired cerebral palsy [Ref] |
Clinical Features
Cerebral palsy is primarily a motor disorder, characterised by abnormalities in the development of movement and posture, resulting in activity limitation [Ref1][Ref2]
Motor Features
Common motor features include: [Ref1][Ref2]
- Early features [NICE NG62]
- Delayed motor development / milestones
- Absent or abnormal fidgety movements
- Movement abnormalities (e.g. asymmetry or lack of movement)
- Abnormal muscle tone (e.g. spasticity, dystonia, fluctuating tone)
- Feeding difficulties
- Other motor features that become more apparent with development
- Abnormal gait
- Muscle weakness
- Impaired coordination / selective motor control
- +/- Involuntary movements (depending on the motor phenotype – see below)
Key milestone red flags for cerebral palsy: [NICE NG62]
- Not sitting by 8 months
- Not walking by 18 months
- Hand preference (early asymmetry of hand function) before 1 year of age (due to possible weakness / spasticity of the contralateral limb)
Use corrected age for those born preterm
Key MSK complications arise from chronic abnormal muscle tone, muscle imbalance and abnormal postures: [Ref]
- Muscle shortening and fixed contractures
- Hip dislocation
- Scoliosis
- Joint deformity
- Pain and reduced mobility
Classification by Predominant Motor Phenotype
| Cerebral palsy phenotype | Predominant motor features | Classic associations / causes |
|---|---|---|
| Spastic – most common | Primarily affects: upper motor neuron (corticospinal pathways)
Causes typical upper motor neuron lesion findings:
Gait changes:
|
Prematurity / PVL → bilateral spastic cerebral palsy
Hypoxic-ischaemic encephalopathy (can cause both spastic or dyskinetic cerebral palsy, depending on the brain injury pattern) Perinatal stroke / unilateral cerebral injury → unilateral spastic cerebral palsy |
| Dyskinetic | Primarily affects: basal ganglia / extrapyramidal motor pathways
Primary features: involuntary movements + fluctuating muscle tone There are 2 main subtypes:
|
Hypoxic-ischaemic encephalopathy (can cause both spastic or dyskinetic cerebral palsy, depending on the brain injury pattern)
Kernicterus classically associated with choreoathetotic type |
| Ataxic | Primarily affects: cerebellar system
Causes typical cerebellar features (DANISH):
|
Cerebellar injury
Maldevelopment |
Cerebral palsy can also be a mixed subtype where there are features of >1 phenotype (e.g. spasticity + involuntary movements)
Associated Non-Motor Features and Comorbidities
| Body system | Associated features / comorbidities |
|---|---|
| Neurological / neurodevelopment |
|
| Sensory |
|
| Gastrointestinal | Bulbar muscle weakness can result in:
Other GI features:
|
| Systemic |
|
Investigation and Diagnosis
Cerebral palsy is a clinical diagnosis based on: [NICE NG62]
- Detailed history including
- Antenatal, perinatal, postnatal history
- Developmental progress (esp. motor milestones)
- Neurological examination
Consider general movement assessment in high-risk infants (0-3 months) [NICE NG62]
MRI may be required: [NICE NG62]
- MRI is NOT required to confirm the diagnosis; its main role is to investigate the underlying cause
- MR is indicated when there is no clear cause from the history, developmental progress, examination, and previous cranial ultrasound findings
Red flags suggesting an alternative neurological diagnosis: [NICE NG62]
- Absence of known causes / risk factors
- Family history of a progressive neurological disorder (e.g. hereditary spastic paraplegia, mitochondrial disorder)
- Regression of developmental milestones / attained cognitive function (suggests a progressive neurological / neurodegenerative disorder)
- Development of unexpected focal neurological signs
- MRI findings suggestive of a progressive neurological disorder or not in keeping with those of cerebral palsy
If ANY of the above are present, refer to paediatric neurology for further assessment
Management
All children with suspected cerebral palsy should be referred to a child development service for urgent multidisciplinary assessment
Management is individualised and multidisciplinary
| Problem / manifestation | Management |
|---|---|
| Problems with movement or posture (spasticity-related) [NICE CG145] | Mainstay: physiotherapy +/- occupational therapy
Consider orthoses to improve posture / function and reduce risk of contracture and hip dislocation Pharmacological adjuncts:
In severe refractory cases, seek specialist opinion regarding options like intrathecal baclofen, orthopaedic surgery, selective dorsal rhizotomy |
| Drooling / saliva control [NICE NG62] | First, assess reversible contributors (e.g. positioning, reflux, dental problems, medications)
Choice of therapy:
|
| Eating, drinking, or swallowing difficulties | Involve speech and language therapy
If there are concerns regarding poor nutrition / impaired growth:
|
| Speech, language, or communication difficulties | Involve speech and language therapy |
| Low bone mineral density | Optimise nutrition, calcium and vitamin D
Encourage appropriate active movement / weight-bearing where possible Also see the Osteoporosis article |
| Sleep disturbances | Optimise sleep hygiene
Assess for / manage contributors to sleep disturbances (e.g. pain, seizures, positioning problems, OSA, medication effects) Consider melatonin, esp. for sleep-onset problems |
| Pain, discomfort, and distress | Assess for and manage any underlying causes, such as spasticity / dystonia, hip displacement, scoliosis, constipation, GORD |
| Visual and hearing impairment | ALL children with cerebral palsy should have a baseline ophthalmological + orthoptic assessment upon diagnosis
Arrange ongoing regular hearing assessment |
| Epilepsy | Manage as per the Seizures and Epilepsy in Adults and Childhood Onset Epilepsy articles |
| Constipation | Assess for constipation regularly and manage accordingly |
| GORD | Manage as per the Gastro-Oesophageal Reflux Disease (GORD) and Gastro-Oesophageal Reflux Disease (GORD) in Children articles |
| Mental health or behavioural problems | Identify and manage problems, such as: |
Prevention
In preterm labour, antenatal IV magnesium sulfate (to the mother) can be given for fetal neuroprotection (reduces risk of cerebral palsy) [NICE NG25]
- Consider if 30+0 – 33+6 weeks
- Offer if 24+0 – 29+6 weeks
See the PROM, P-PROM, and Preterm Labour article for more information