Cerebral Palsy Flashcards
What is cerebral palsy?
A permanent disorder of movement and/or posture and of motor function due to a non-progressive abnormality in the developing brain
Does cerebral palsy get worse over time?
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.
What is the cause of cerebral palsy?
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
What are post-natal causes?
Meningitis/encephalitis/encephalopathy, head trauma from accidental or non-accidental injury, symptomatic hypoglycaemia, hydrocephalus and hyperbilirubinemia
What are early features of CP?
- 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.
What are normal motor development milestones?
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
Describe abrnomal motor development in CP
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
How is gross motor function classified?
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
What are the types of CP?
Spastic: bilateral, unilateral
Dyskinetic
Ataxic
Where is the damage in spastic cerebral palsy?
upper motor neurone (pyramidal or corticospinal tract) pathway
Describe neurological findings in spastic cerebral palsy.
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.
What is dynamic catch?
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.
What are the main types of spastic CP?
Unilateral (hemiplegia)
Bilateral (quadriplegia)
Bilateral (Diplegia
Describe unilateral spastic CP. How and when does ti present? Clinical features?
Risks?
GMFCS?
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
Describe bilateral spastic CP. Clinical features?
Risks?
GMFCS?
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
Describe bilateral diplegia spastic CP. Clinical features?
Risks?
GMFCS?
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
Describe dyskinetic cerebral palsy. define chorea, athetosis and dystonia
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.
How does dyskinetic cerebral palsy present? Clinical features?
Risks? GMFCS?
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
Where is the damage in dyskinetic CP? Causes?
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
Describe ataxic cerebral palsy
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.
Describe management of CP
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.