Cerebral Palsy

Question 1

What is cerebral palsy?

Answer 1

A permanent disorder of movement and/or posture and of motor function due to a non-progressive abnormality in the developing brain

Question 2

Does cerebral palsy get worse over time?

Answer 2

Although the causative lesion is non-progressive and damage to the brain is static, clinical manifestations emerge over time, reflecting the balance between normal and abnormal cerebral maturation.

Question 3

What is the cause of cerebral palsy?

Answer 3

Antenatal in origin due to cerebrovascular haemorrhage or ischaemia, cortical migration disorders or structural maldevelopment of the brain during gestation.
- Linked to genital deletions, congenital infections, genetic syndromes.

10% of cases due to hypoxic-ischaemic injury before or during delivery.

10% post-natal

Question 4

What are post-natal causes?

Answer 4

Meningitis/encephalitis/encephalopathy, head trauma from accidental or non-accidental injury, symptomatic hypoglycaemia, hydrocephalus and hyperbilirubinemia

Question 5

What are early features of CP?

Answer 5

• Abnormal limb and/or trunk posture and tone in infancy with delayed motor milestones; this may be accompanied by slowing of head growth
• Feeding difficulties, with oromotor incoordination, slow feeding, gagging and vomiting
• Abnormal gait once walking is achieved
• Asymmetric hand function before 12 months of age.

Question 6

What are normal motor development milestones?

Answer 6

Median/limit
1.5 months/3m - pushes up on arms, holds head up

3m/6m - sits with support, holds head up, rounded back

6m/9m - sits without support, arms free to reach and grasp

9m/13m - pulls to stand

12m/18m - independently standing or walking

Question 7

Describe abrnomal motor development in CP

Answer 7

1.5m/3m
unable to lift head or push up on arms, stiff extended legs, difficulty moving out of position

3m/6m
Unable to lift head
Floppy trunk
Stiff limbs
Arms flexed and held back
Stiff crossed legs

6m/9m
Rounded back
Poor use of arms
Poor head control
Difficult getting arms forward
Will not take weight on legs

9m/13m
not interested in weigh bearing
Difficulty in pulling to stand
Stiff legs
Pointed toes
Cannot crawl on hands na knees
May only use one side of body to move

12m/18m
Holds arm or both arms stiffly bent
Excessive tiptoe gait
Sits with weight to one side
Uses on hand for play

Question 8

How is gross motor function classified?

Answer 8

Level I Walks without limitations
Level II Walks with limitations
Level III Walks using a handheld mobility device
Level IV Self-mobility with limitations; may use powered mobility
Level V Transported in a manual wheelchair

Question 9

What are the types of CP?

Answer 9

Spastic: bilateral, unilateral
Dyskinetic
Ataxic

Question 10

Where is the damage in spastic cerebral palsy?

Answer 10

upper motor neurone (pyramidal or corticospinal tract) pathway

Question 11

Describe neurological findings in spastic cerebral palsy.

Answer 11

Limb tone is persistently increased (spasticity) with associated brisk deep tendon reflexes and extensor plantar responses.
The tone in spasticity is velocity dependent, so the faster the muscle is stretched the greater the resistance it will have.

Question 12

What is dynamic catch?

Answer 12

The tone in spasticity may elicit a dynamic catch, which is the hallmark of spasticity. The increased limb tone may suddenly yield under pressure in a ‘clasp knife’ fashion.

Question 13

What are the main types of spastic CP?

Answer 13

Unilateral (hemiplegia)
Bilateral (quadriplegia)
Bilateral (Diplegia

Question 14

Describe unilateral spastic CP. How and when does ti present? Clinical features?
Risks?
GMFCS?

Answer 14

Unilateral involvement of the arm and leg.
The arm is usually affected more than the leg, with the face spared.
Affected children often present at 4–12 months of age with fisting of the affected hand, a flexed arm, a pronated forearm, asymmetric reaching, hand function or toe pointing when lifting the child.
Subsequently, a tiptoe walk (toe–heel gait) on the affected side may become evident.
Affected limbs may initially be flaccid and hypotonic, but increased tone soon emerges as the predominant sign.

May have visual feild defect on side of hemiplegia
Risk of learning difficulties and seizures
GMFCS 1 and 2

Question 15

Describe bilateral spastic CP. Clinical features?
Risks?
GMFCS?

Answer 15

All four limbs are affected, often severely.
The trunk is involved with a tendency to opisthotonus (extensor posturing), poor head control and low central tone
Associated with seizures, microcephaly and moderate or severe intellectual impairment.

Associated with learning difficulties, feeding difficulties, problems with speech, vision and hearing
Seizures common
Risk of hip sublimation, dislocation and scoliosis.
Dependent on others
GMFCS 4/5

Question 16

Describe bilateral diplegia spastic CP. Clinical features?
Risks?
GMFCS?

Answer 16

All four limbs, but the legs are affected to a much greater degree than the arms, so that hand function may appear to be relatively normal.

Motor difficulties are apparent in arms with functional use of hands.
Walking is abnormal
Associated with preterm birth due to periventricular brain damage

Usually no feeding or communication difficulties and good cognition
Often associated with squints

GMFCS 1-3

Question 17

Describe dyskinetic cerebral palsy. define chorea, athetosis and dystonia

Answer 17

Dyskinesia refers to movements that are involuntary, uncontrolled, occasionally stereotyped and often more evident with active movement or stress.

• chorea – irregular, sudden and brief non-repetitive movements
• athetosis – slow writhing movements occurring more distally such as fanning of the fingers
• dystonia – simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles often giving a twisting appearance.

Question 18

How does dyskinetic cerebral palsy present? Clinical features?
Risks? GMFCS?

Answer 18

Affected children often present with floppiness, poor trunk control and delayed motor development in infancy. Abnormal movements may only appear towards the end of the first year of life.

Mixture of motor patterns including dystonia, athetosis and chorea.
Cognition may be preserved but feeding difficulties common.
Risk of hip deformity and scoliosis.
Dependent on others due to severe movement difficulties even if cognition normal

GMFCS 4-5

Question 19

Where is the damage in dyskinetic CP? Causes?

Answer 19

basal ganglia or their associated pathways (extra-pyramidal)

the most common cause was hyperbilirubinemia (kernicterus) due to rhesus disease of the newborn but it is now hypoxic-ischaemic encephalopathy at term

Question 20

Describe ataxic cerebral palsy

Answer 20

When due to acquired brain injury (cerebellum or its connections), the signs occur on the same side as the lesion but are usually relatively symmetrical.

There is early trunk and limb hypotonia, poor balance and delayed motor development.

Incoordinate movements, intention tremor and an ataxic gait may be evident later.

Question 21

Describe management of CP

Answer 21

Parents informed of diagnosis ASAP
Prognosis is difficult during infancy until the severity and pattern of evolving signs and child’s developmental progress have become clearer over several month or years.
Children are likely to have a range of medical, psychological and social problems so MDT apporach is essential.

Novel treatments for treating hypertonia such as botulinum toxin injections, selective dorsal rhizotomy, intrathecal baclofen (muscle relaxant) and DBS of basal ganglia considered.