Cerebral Palsy

MBBS YEAR 3, Batch 8, 2019

DR.SWE ZIN AYE
zinaye.swe@qiup.edu.my
Learning Outcomes:
At the end of this session, the students are expected to:
• Discuss the common common differential diagnosis of
movement and posture disorders.
• Define and classify types of cerebral palsy.
• Establish a diagnosis of cerebral palsy based on the history
and physical examination.
• Understand the aetiology, pathophysiology and natural
history of cerebral palsy.
• Develop plans for investigations, treatment, monitoring and
follow-up for cerebral palsy.
Little's Disease
William John Little (1810–
1894)  
The first person to medically
record the condition.

English Orthopaedic surgeon

Little founded the 

Royal Orthopaedic Hospital
 of London.
Definition (Old)
A disorder of movement and/or posture as a result of
permanent non-progressive lesion of motor pathway in the
developing brain.

A group of permanent disorders of movement and posture

causing activity limitations that are attributed to non-
progressive disturbances that occurred in the developing fetal
or infant brain. (New)

First two years of life involved

Abnormal posture
Abnormal posture
Cerebral Palsy
• Abnormal motor behaviour is the core feature of CP.
• Disturbance resulting in CP is presumed to occur before the
affected function has developed (e.g. walking,
manipulation, etc.)
• Includes
• the cerebrum,
• the cerebellum,
• the brain stem.
• Excludes motor disorders of:
• spinal, peripheral nerve
• muscular or mechanical origin.
Epidemiology

• Most common cause of motor impairment in children

• The incidence is 3.6 per 1,000 children
• Male : female ratio of 1.4 : 1
• Elevated levels of in inflammatory cytokines have been
reported in heelstick blood collected at birth from children
who later were identifed with CP.
Aetiology
Not well understood!
Thought to be associated with:
1. Antenatal (80%)
• Vascular occlusion
• Congenital malformation of brain
• Genetic syndromes
• Congenital infection – TORCH,
maternal sepsis, chorioamnionitis
• Drugs (eg. Antiepileptics –
phenytoin)
• Radiation
• APH causing foetal anoxia
2. At birth (10%)
• Hypoxic-ischaemic injury (HIE)
• Low birth weight infants <1000g
(ICH and PVL)
3.Postnatal (10%)
• PVL
• Hyperbilirubinaemia
• Symptomatic hypoglycaemia
• Hydrocephalus
• Meningitis/ encephalitis/
encephalopathy
• Head trauma (accidental/
nonaccidental injury)
 Periventricular leukomalcia
• Reflects the enhanced vulnerability of immature oligodendroglia in
premature infants to oxidative stress

• Caused by ischemia or infectious/inflammatory insults.

• White matter abnormalities (loss of volume of periventricular white

matter, extent of cystic changes, ventricular dilation, thinning of the
corpus callosum)

• Present on MRI at 40 wk of gestational age of preterm infants are a

predictor of later CP.
Clinical presentation
Early features
1. Abnormal limb tone and
posture
2. Delayed motor mile stone
3. Feeding difficulties
4. Abnormal gait
5. Asymmetric hand function
before 12 months of age
6. Persistence of primitive
reflexes
Damage to the upper motor
neuron (Pyramidal tract)
1. Limb tone is increased
2. Brisk tendon reflexes
3. Extensor plantar responses
4. Spasticity tends to present
early/ neonatal period
5. Sometimes there is initial
hypotonia particulary of the
head and trunk
Gross Motor Function Classification
System (GMFCS)
LEVEL 1 - Walks without limitations

LEVEL 2 - Walks with limitations

LEVEL 3 - Walks using a handheld mobility device

LEVEL 4 - Self-mobility with limitations, may use powered mobility.

LEVEL 5 - Transported in a manual wheelchair

Physiological classifications

90% 6% 4%
Spastic Hemiplegia
Unilateral involvement of the arm and leg. Arm
is usually affected more.
1. Often present at 4-12 months of age with
fisting of affected hand, a flexed arm, a
pronated forearm.
2. Hand preference before the age of one year.
3. Walking is usually delayed until 18–24
months and a circumductive gait is apparent
4. Tiptoe walk (toe-heel gait) on the affected
side because of increased tone
5. Extremities - growth arrest (asymmetry)
Spastic Hemiplegia
Associated conditions: Investigations:

• About one-third of patients • CT scan/MRI - atrophic

have a seizure disorder cerebral hemisphere with a
dilated lateral ventricle
• 25% - mental retardation contralateral to the side of
the affected extremities

• MRI - for calcifications

associated with congenital
infections.
Spastic Diplegia
All 4 limbs are affected.
Legs are affected more. Hand function
appears to be relatively normal.
1. Spasticity in the legs with brisk reflexes,
ankle clonus, and a bilateral Babinski
sign
2. Scissoring posture on suspended by the
axilla and excessive hip adduction (hard
to put on a nappy)
3. Walking is delayed, and characteristic
gait: feet in equinovarus and walking on
tiptoe
Spastic Diplegia
Prognosis: Investigations:

• normal intellectual • MRI - the severity of white

development matter injury usually in
• Seizures is minimal preterm babies
(most commonly PVL, fibers
innervating the legs course
through the internal capsule)
Spastic Quadriplegia
Most severe form. All 4 limbs are
affected, arms may be more affected than
the legs.
1. Trunk is involved with extensor
posturing. Poor head control.
2. Increased tone and spasticity in all
extremities.
3. Decreased spontaneous movements,
brisk reflexes, and plantar extensor
responses.
Spastic Quadriplegia
Assoicated with: Investigations:

1. Seizures HIE
2. Microcephaly
Thinning of corpus callosum
3. moderate or severe
intellectual impairment  PVL

4. swallowing difficulty
(pseudobulbar palsy) ventriculomegaly
Dyskinetic cerebral palsy
Extrapyramidal tract (basal
ganglia)
Fluctuating muscle tone
leading to involuntary
movements:
1. athetoid – slow writhing
2. chorea – sudden, jerky,
non-repetitive
3. choreo-athetoid
4. dystonic
Associated with birth
asphyxia, kernicterus:
• Hypotonic with poor head
control and delayed motor
development in infancy
• Abnormal movement usually
not evident before 1 year of
age.
• Marked head lag and develop
variably increased tone with
rigidity and dystonia over
several years.
Dyskinetic cerebral palsy
Associated with:

• Intellect is relatively
unimpaired

• Seizures uncommon

• Speech problem due to

oropharyngeal muscle
involvement
Ataxic hypotonic cerebral palsy
Relatively symmetrical
Early trunk and limb hypotonia
1. Poor balance and delayed motor
development
2. Incoordinate movements, intention
tremor and ataxic gait
Ataxic hypotonic cerebral palsy
Associated problems
1. Learning difficulties
(60%)
2. Epilepsy (40%)
3. Squints
4. Visual impairment
(refractory error, cortical
damage)
5. Hearing impairment
6. Speech and language
disorders
7. Behavioural disorders
8. Feeding problems,
aspiration pneumonia
9. Bladder and bowel
problems –
incontinence,
constipation
10. Joint contractures, hip
subluxation, scoliosis
 Diagnosis of CP
Conventional methods!
1. History - milestones
2. Examination
3. Causes and risk factors

Exclude differential diagnosis- degenerative diseases,

metabolic disorders, spinal cord tumor, or muscular dystrophy
 Investigations
1. MRI is far more sensitive than CT for most lesions seen with
CP, although a CT scan may be useful for detecting
calcifications associated with congenital infections
2. TORCH screening
3. Metabolic screening
4. Genetic evaluation
Management (Multidisciplinary
Child Development Services)
Multidisciplinary: Health professionals:

1. Paediatrician, 1. identify the cause,

2. Physiotherapist, 2. prevent contractures and
deformities.
3. Occupational therapist, 3. eye hand coordination, activities of
daily living, adaptation of housing
4. Speech and language 4. language & speech development,
therapist, feeding
5. Clinical psychologist, 5. cognitive assessment, behavioural
management, educational advice
6. Dietician 6. advice on feeding and nutrition
7. Social worker 7. social environmental assessment
Management (Multidisciplinary
Child Development Services)
Multiagency: Orthoses:

1. health,
2. social services,
3. education,
4. volunteers, voluntary
agencies,
5. parent support groups
Management of spasticity
Medical management Surgical/Orthopaedic

1. dantrolene sodium, 1. operations for deformities

benzodiazepines, and and contractures
baclofen. 2. rhizotomy procedure in
2. Botulinum toxin injected which the roots of the
into specific muscle spinal nerves are divided
groups
3. Treatment of associated
conditions.
Prevention of CP
IV MgSO4 to mother in Cooling term infants with HIE
preterm labour <32weeks
• Preliminary results of • 33.3°C (91.9°F) for 3 days,
controlled trials: significant starting within 6 hr of birth,
reduction in the risk of CP
at 2 yr of age.
• reduces the risk of
dyskinetic or spastic
• One study: no benefit in quadriplegia form of CP.
terms of incidence of CP,
and abnormal motor,
cognitive, behavioural
function at school age.
Summary
• History of cerebral palsy
• Definition, classification and clinical presentations of
cerebral palsy.
• Aetiology, risk factor and pathophysiology of cerebral palsy.
• Investigations, management and prevention of cerebral
palsy.
References
• Nelson Essentials of Pediatrics 8th edition 2019 by K.
Marcdante MD,R. M. Kliegman MD,R. E. Behrman MD 
• Malaysia paediatrics protocol, 4th Edition.
• Illustrated Textbook of Paediatrics (5th Edition) International
Edition by Tom Lissauer, Will Carroll