Cerebral Palsy

What is Cerebral Palsy

Cerebral palsy is a group of movement disorders that affect the body’s motor function, coordination, muscle tone and posture. It is a permanent, non-progressive condition that often develops before birth or early childhood. It is caused by abnormal development or damage to the brain.

How common is it?

● Cerebral palsy is the most common physical disability in childhood
● 1 in 700 babies are diagnosed with cerebral palsy in Australia
● Approximately 34,000 Australians are currently living with cerebral palsy

What are the risk factors?

● Premature birth – 43% of people with cerebral palsy were preterm
● Multiple birth e.g. twins, triplets
● Traumatic birth
● Infection during pregnancy
● Exposure of toxins during pregnancy

What causes cerebral palsy?

Cerebral palsy can be congenital (caused before or during birth) or acquired (caused at least 28 days after birth). Congenital cases of cerebral palsy are more common.

Congenital causes:
● Damage to the brain’s white matter during foetal development
● Abnormal brain development
● Stroke
● Impaired oxygen to the brain due to:
○ Low blood pressure in the mother
○ Torn uterus
○ Detachment of the placenta
○ Defects with the umbilical cords
○ Severe trauma to the infant’s head during labour or delivery

Acquired causes:
● Brain damage in the first few months or years of life
● Childhood infections e.g. meningitis, encephalitis
● Impaired blood flow to the brain due to:
○ Stroke
○ Blood clotting issues
○ Heart defect
○ Sickle cell disease
● Head injury from a car incident, fall or child abuse e.g. shaken baby syndrome

How is cerebral palsy diagnosed?

Cerebral palsy is usually diagnosed at 1 to 2 years old but may be diagnosed later if symptoms are mild. The diagnosis is made by a GP or specialist such as a paediatrician or child neurologist.

During the consultation, the doctor will take a comprehensive obstetric history and look for any risk factors such as adverse antenatal or perinatal events. During the physical examination, they will take note of early indicators including motor milestone delays, persistent primitive reflexes and upper motor neuron signs. Further genetic, metabolic and brain imaging tests (CT or MRI) may be conducted to confirm the cause of the cerebral palsy.

What does cerebral palsy look like?

There are 4 main types of cerebral palsy – spastic, dyskinetic, ataxic and mixed:

Spastic cerebral palsy is characterised by stiff and tight muscles. It is caused by damage to the upper motor neurons in the brain, resulting in upper motor neuron signs such as spasticity, hypertonia, clonus and a positive babinski sign. It is the most common type of cerebral palsy, making up 60% to 80% of all cases. Spastic cerebral palsy can be categorised by the area of the body affected:

Spastic hemiplegia – Affects one side of the body
○ Circumduction gait – they may swing their affected leg outwards to clear the ground due to tightness in their hip flexors
○ Early handedness – they may exhibit early preference of one hand due to difficulty using the other side
○ Toe walking, ankle clonus and other upper motor neuron signs are observable on the affected side
○ Majority of cases exhibit normal intellectual development and can ambulate independently

Spastic diplegia – Affects the lower limbs
○ Commando crawling – they may crawl with their hands and drag their feet behind due to lower limb weakness
○ Scissor gait – they may walk with their knees crossing over like scissors due to tightness in their hip adductors
○ Toe walking, ankle clonus and other upper motor neuron signs are observable in both legs
○ Majority of cases exhibit normal intellectual development and can ambulate independently

Spastic quadriplegia – Affects both upper and lower limbs
○ Often the face and torso muscles are also affected
○ If swallowing is impaired, there is often a risk of aspiration pneumonia
○ It is the most severe type of cerebral palsy and is associated with significant functional limitations, cognitive deficits, epilepsy and visual impairment
○ People with spastic quadriplegia usually require a manual wheelchair for mobility

Spastic Cerebral Palsy

Figure 1. Types of spastic cerebral palsy

Dyskinetic cerebral palsy is characterised by random involuntary movements. These movements may decrease with rest and increase with stress. It is caused by damage to the basal ganglia in the brain, which is responsible for automatic and voluntary movements. It is a rare type of cerebral palsy, making up 6% of all cases. The random involuntary movements can be categorised into three types:

● Dystonia – Twisting, repetitive movements and abnormal postures caused by frequent changes in muscle tone
● Athetosis – Slow, circular and writhing movements
● Chorea – Unpredictable, dance-like movements

Figure 2. Man with dystonia

Figure 3. Musculoskeletal impairments in cerebral palsy

Ataxic cerebral palsy is caused by damage to the cerebellum, resulting in poor balance and coordination. It is a rare type of cerebral palsy, making up 6% of all cases. People with ataxic cerebral palsy tend to:

● Have bent knees and a wide base of support to help maintain their balance
● Take irregular steps, similar to someone who is intoxicated
● Have poor depth perception e.g. undershoot or overshoot targets
● Fall and stumble

Some people have mixed cerebral palsy, which is a combination of the 3 types.

What other conditions are associated with cerebral palsy?

People with cerebral palsy are likely to have other associated conditions in addition to their motor impairments:

● Pain
● Intellectual disability
● Spinal and hip deformities
● Speech impairment
● Epilepsy
● Continence and constipation issues
● Vision impairment
● Hearing loss

As you can see, cerebral palsy has a variety of presentations depending on the neurological symptoms, the area of the body affected and the presence of associated conditions. No two cases are the same.

Figure 4. Levels of Gross Motor Function Classification System for children with cerebral palsy

Medical treatments for cerebral palsy

Common medications used to treat cerebral palsy include:

● Anticholinergics for dyskinesia e.g. robinul
● Anticonvulsants for epilepsy e.g. benzodiazepine
● Muscle relaxants for spasticity and contractures e.g. botulinum A, botox
● Anti-inflammatories for pain relief e.g. NSAIDS
● Antidepressants for behavioural issues e.g. lexapro

Surgical treatments for cerebral palsy

Surgery can help correct deformities, increase independent motor function and improve gait and range of motion in people with cerebral palsy. The goal of surgery differs depending on the ambulatory status of the patient – surgery can help ambulatory children improve their ability to walk independently and can provide comfort and pain relief to non-ambulatory children.
Physiotherapy and medical treatment are recommended before considering surgery.

Neurosurgeries
● Selective Dorsal Rhizotomy – Problematic nerve root of the spinal cord is cut to reduce spasticity
● Intrathecal Baclofen – A pump and tube is permanently inserted into the abdomen and spinal cord so that baclofen (muscle relaxant medication) can be administered directly into spinal fluid. It can be used to relieve severe spasticity when oral medications are ineffective.

Figure 5. Selective Dorsal Rhizotomy

Figure 6. Intrathecal Baclofen

Orthopaedic surgeries

● Muscle lengthening and tendon transfer – muscles and tendons are surgically lengthened to relieve tightness (contractures)
● Tenotomy/myotomy – muscle and tendon are cut to relieve contractures
● Osteotomy – bones are cut and repositioned for better joint alignment
● Arthrodesis – bones are fused together to relieve contractures and provide stability
● Single-event multilevel surgery (SEMLS) – corrects all bone and soft tissue issues in one surgery

Figure 6. Before and after SEMLS

Figure 8. SEMLS example

Other types of surgeries may be undertaken in conjunction with neurosurgery and orthopaedic surgery to address other non-motor impairments.

Physiotherapy treatments for cerebral palsy

Physiotherapy plays an important role in the management of cerebral palsy by:
● Increasing fitness, flexibility, balance and posture
● Helping manage pain and discomfort
● Increasing range of motion and muscular strength
● Improving quality of life and reducing physical impairments to maximise independence
● Providing advice on mobility aids and assistive equipment

Clinical practice guidelines recommend the following physiotherapy interventions to improve physical function for children and young people (2 to 18 years old) with cerebral palsy:

Hand Function
● Constraint Induced Movement Therapy (CIMT) and Bimanual Therapy is strongly recommended
● CIMT can be done first to improve hand function on the affected side and can be progressed to bimanual therapy later
● Exercises should be task-specific and use assistive devices to encourage independence

Improving Strength
● Resistance training is strongly recommended
● Hydrotherapy and electrical stimulation has limited evidence

Mobility
● Gait training is strongly recommended
● Overground or treadmill walking can help increase walking speed and endurance

Range of Motion
● Serial casting and active hip surveillance is strongly recommended
● Stretching is ineffective long-term but can help the patient feel more flexible short-term

Cardiovascular Fitness
● Aerobic training is strongly recommended

Functional Capacity
● Context focused, goal oriented training is strongly recommended
● Home programs are strongly recommended

Figure 9. CIMT

Figure 10. Bimanual therapy

Figure 11. Partial weight body support with treadmill training

Figure 12. Serial Casting

Figure 13. Progressive dislocation of hip in cerebral palsy

References

Das, S. P., & Ganesh, G. S. (2019). Evidence-based Approach to Physical Therapy in Cerebral Palsy. Indian journal of orthopaedics, 53(1), 20–34. https://doi.org/10.4103/ortho.IJOrtho_241_17

Cerebral Palsy Alliance. (2023). What is cerebral palsy? https://cerebralpalsy.org.au/cerebral-palsy/

McIntyre, S., Morgan, C., Walker, K., & Novak, I. (2011). Cerebral palsy–don’t delay. Developmental disabilities research reviews, 17(2), 114–129. https://doi.org/10.1002/ddrr.1106

National Institute of Neurological Disorders and Stroke. (2023, January 27). Cerebral Palsy. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/cerebral-palsy

Novak I. Evidence-Based Diagnosis, Health Care, and Rehabilitation for Children With Cerebral Palsy. Journal of Child Neurology. 2014;29(8):1141-1156. doi:10.1177/0883073814535503

Novak, I., Hines, M., Goldsmith, S., & Barclay, R. (2012). Clinical prognostic messages from a systematic review on cerebral palsy. Pediatrics, 130(5), e1285–e1312. https://doi.org/10.1542/peds.2012-0924

Ostensjø, S., Carlberg, E. B., & Vøllestad, N. K. (2004). Motor impairments in young children with cerebral palsy: relationship to gross motor function and everyday activities. Developmental medicine and child neurology, 46(9), 580–589. https://doi.org/10.1017/s0012162204000994

Patel, D. R., Neelakantan, M., Pandher, K., & Merrick, J. (2020). Cerebral palsy in children: a clinical overview. Translational pediatrics, 9(Suppl 1), S125–S135. https://doi.org/10.21037/tp.2020.01.01

Stanton, M. (2012). Understanding cerebral palsy : a guide for parents and professionals. London: Jessica Kingsley Publishers.

Wallis M. (2007). Cerebral palsy: a complete guide for caregiving, 2nd edn. Archives of Disease in Childhood, 92(1), 92.