CEREBRAL PALSY

Question 1

What is cerebral palsy?

Answer 1

Group of conditions affecting motor function and posture due to a non-progressive lesion of the developing brain.

Question 2

What proportions of babies are affected by cerebral palsy?

Answer 2

2 in 1,000

Question 3

What are the possible motor features of cerebral palsy?

Answer 3

Abnormal tone early infancy

Delayed motor milestones

Abnormal gait

Feeding difficulties

Persisting primitive reflexes

Question 4

What are the associated non-motor features of cerebral palsy?

Answer 4

Learning difficulties (60%)

Epilepsy (30%)

Squints (30%)

Hearing impairment (20%)

Question 5

What are the antenatal causes of cerebral palsy?

Answer 5

Antenatal (80%):

Cerebral malformation

Congenital infection (rubella, toxoplasmosis, CMV)

Question 6

What are the intrapartum causes of cerebral palsy?

Answer 6

Intrapartum (10%):

Birth asphyxia

Trauma

Question 7

What are the post-partum causes of cerebral palsy?

Answer 7

Postnatal (10%):

Intraventricular haemorrhage

Meningitis

Head-trauma

Hypoglycaemia

Hyperbilirubinaemia

Question 8

What are the congenital infections that are known to be a risk factor for cerebral palsy?

Answer 8

Rubella

Toxoplasmosis

CMV

Question 9

What are the types of cerebral palsy?

Answer 9

Spastic (70%): hemiplegia, diplegia (all four limbs but mostly arms) or quadriplegia (all four limbs but mostly legs)

Dyskinetic (10%)

Ataxic (10%)

Mixed (10%)

Question 10

What are the features of spastic cerebral palsy?

Answer 10

Damage to pyramidal pathways causes increased limb tone, often due to intraventricular haemorrhage

Brisk deep tendon reflexes

Velocity dependent increase in tone - dynamic catch

Extensor palmar responses

May be preceded by period of hypotonia

Question 11

What are the three main types of spastic cerebral palsy?

Answer 11

Hemiplegic

Quadriplegic

Diplegic

Question 12

What are the features of hemiplegic spastic cerebral palsy?

Answer 12

Unilateral involvement of the arm and leg. The arm will often be more affected than the leg, with the face being spared.

Fisting of the hand

Flexed arm

Pronated forearm

Tiptoe walk - toe-heel gait

Often no history of hypoxic-ischaemic encephalopathy

Question 13

What are the features of quadriplegic spastic cerebral palsy?

Answer 13

All four limbs affected.

Extensor posturing of the trunk

Poor head control

Low central tone

Associated with seizures, microcephaly and moderate/severe intellectual impairment

There may be a history of perinatal hypoxic-ischaemic encephalopathy

Question 14

What are the features of diplegic spastic cerebral palsy?

Answer 14

All four limbs affected but legs a lot more than arms.

Hand function can be normal

Associated with preterm birth due to periventriculaar brain damage.

Question 15

What are the features of dyskinetic cerebral palsy?

Answer 15

Damage to the basal ganglia or extrapyramidal pathways causes decrease in tone

Abnormal involuntary movements - chorea, athetosis and dystonia

Intellect often relatively unimpaired.

Question 16

What is chorea?

Answer 16

Irregular, sudden and brief non-repetitive movements

Question 17

What is athetosis?

Answer 17

Slow writhing movements occuring more distally such as fanning of fingers

Question 18

What is dystonia?

Answer 18

Simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles giving a twisting appearance

Question 19

What are the features of ataxic cerebral palsy?

Answer 19

Damage to the cerebellum or its pathways. Associated with genetic factors

Hypotonia

Poor balance

Uncoordinated movements

Delayed motor development

Question 20

What are the management options for someone diagnosed with cerebral palsy?

Answer 20

MDT - physiotherapy is key

Treatments for spasticity include:

Oral diazepam

Oral and intrathecal baclofen

Botulinum toxin type A

Orthopaedic surgery

Selective dorsal rhizotom

Anticonvulsants and analgesia as required

Question 21

You review a 7-year-old girl who has cerebral palsy. She is having ongoing problems with spasticity in her legs which is causing pain and contractures. On speaking to her mother you ascertain that she is having regular physiotherapy, using the appropriate orthoses and has tried oral diazepam in the past. Which one of the following treatments may she be offered to try and improve her symptoms?

Baclofen

Clozapine

Clonidine

Dantrolene

Glyceryl trinitrate

Answer 21

Baclofen