Cerebral Palsy: Aetiology,

Associated Problems and

Management
Lecture for FRACP candidates
May 2014
 Cerebral palsy
 Definitions and
prevalence

 Risk factors and

aetiology

 Associated problems

 Management options
What is cerebral palsy?
 Definition (Bax 1964)
• Cerebral palsy is a
disorder of
movement and
posture due to a
defect or lesion of
the immature
brain
 Cerebral palsy – an umbrella term
• All children are different
• The associated
problems may be more
significant than the
motor disorder
• Permanent, non-
progressive but not
unchanging
 New definition of cerebral palsy
• Cerebral palsy describes a group of
developmental disorders of movement and
posture, causing activity restriction or disability,
that are attributed to disturbances occurring in
the fetal or infant brain. The motor impairment
may be accompanied by a seizure disorder and
by impairment of sensation, cognition,
communication and/or behaviour and by
secondary musculoskeletal problems
(Rosenbaum 2007)
Storm’s words
 Classification systems
• Nature of movement
disorder eg
spasticity, dystonia,
ataxia
• Distribution eg
diplegia,
quadriplegia
• Severity
 Why does classification matter?
• Descriptors for parents
• Type of treatment needed
• Prognosis eg for hip
displacement
• Associated problems
• Information for service
providers
• Research studies – drawing
together similar groups
 Type of motor disorder
• Spasticity

• Dyskinesia
• chorea

• dystonia

• athetosis

• Ataxia

• Hypotonia

• Mixed
 Type of motor disorder
• Dyskinetic CP is characterized by involuntary,
uncontrolled, recurring, occasionally
stereotyped movements, in which muscle tone
varies.
• Dystonia is characterised by abnormal postures
due to sustained muscle contractions.
• Chorea refers to rapid, involuntary, jerky, often
fragmented movements.
• Athetosis means slower, constantly changing,
writhing, or contorting movements.
 Distribution
• Quadriplegia
• arms
• legs
• trunk
• head and neck
• Diplegia
• Hemiplegia
 Severity
• Mild
• walks independently

• Moderate
• walks with sticks / frame

• Severe
• wheelchair dependent
 Classification of severity

Gross Motor
Function
Classification
System (The
“GMFCS”)
 The GMFCS
• Level 1 – walk without
restrictions
• Level 2 – walk independently
but more limitations
• Level 3 – need mobility
devices eg frames
• Level 4 – sit on a regular
chair, but use wheelchair
• Level 5 – no means of
independent mobility
The GMFCS
The GMFCS
 Measuring motor progress
The Motor Growth
Curves:
describe the pattern of
motor development of
children with varying
severities of cerebral
palsy using the GMFCS
The Growth Motor Curves
What causes cerebral palsy?
 Case history of Lisa
• PMH • At 8 years
– Pregnancy and birth – Presented to RCH
normal – Falling more
– Crawled 10 months – Decline in motor abilities
– Sat 12 months • Examination
– Well grown
• At 24 months – Increased tone / reflexes
– Diagnosis of spastic lower limbs
diplegia – Normal upper limb
– Cause unknown function
 History
Little: poor obstetric care associated with
birth asphyxia responsible for most cases
Freud: adverse fetal events early in
development may cause both birth
complications and spasticity
 Changing views
1960’s: mechanism for the prevention of
kernicterus
1970’s: increased resources for obstetric and
neonatal care, for example, increased
Caesarean Section rate and use of electronic
fetal monitoring
No major change in cerebral palsy rates
Prevalence of cerebral palsy
2 per thousand
live births

130 new cases in Victoria

700 new cases in Australia
each year
Rates of CP and neonatal deaths
Cerebral palsy and neonatal death
rates in Victoria, 1973 - 2005
12
Neonatal deaths
10
Rate per 1000 births

Cerebral palsy
8

0
19 1 1 1 1 1 1 1 1 2 2
73 976 979 982 985 988 991 994 997 000 003

Year of birth
 Gender

• Males are over

represented in all

case series of

cerebral palsy
Risk factors and causes: challenges
• Many risk factors, for example, prematurity, multiple
births, but few definite causes
• Rates 25-30 x higher in infants weighing less than
1500 g
• Babies who weigh <2500g account for 1/3 of children
with cerebral palsy
• Causal pathways – a series of factors leading to the
damaging event
 Why do premature infants
develop cerebral palsy?
1. Are they damaged before
birth and then survive with
good neonatal care?
2. Do they develop
complications of
prematurity such as IVH?
When does cerebral palsy occur?
• Antenatally 75%
• Perinatal 10 - 15%
• Postneonatal 10%
• The cause remains unknown in a substantial
proportion of cases, and is therefore attributed
to antenatal events
 Prenatal events
• Prenatal (75%)
• Brain malformations eg
cortical dysplasias 10-15%

• Intrauterine infections eg
TORCH (toxoplasmosis,
rubella, CMV, herpes simplex)
5% or less
 Prenatal events
• Vascular eg infarct in area of
middle cerebral artery

• Metabolic eg iodine deficiency

associated with hypothyroidism,
intellectual disability and spastic
diplegia

• Toxins eg lead, mercury

 Perinatal events 10-15%
Neonatal encephalopathy:
disturbed neurological function
early in life with difficulty in
initiating and maintaining
respiration, depression of tone and
reflexes, depressed consciousness
and frequently seizures – can be
mild, moderate or severe
 “Birth asphyxia”

Even in the group where there appears

to be evidence of asphyxia,

it may not have been preventable

 Post neonatal causes
• Postneonatal 10%
• injury
accidental
non-accidental
• infections
• meningitis
• encephalitis
 New directions
• The Australian
Cerebral Palsy
Register
– A collaboration
between all the
States and the
AIHW
 New directions
• The Victorian Cerebral
Palsy Register

• Collects information
about all children and
young adults with
cerebral palsy born in
Victoria since 1970
 New directions
• Role of thrombophilic mutations
– Factor V Leiden mutation and
other coagulopathies
• Genetic and epigenetic factors
• Maternal infection – urinary
tract infection, chorioamnionitis
• Multiple pregnancy / low birth
weight
• Interventions at birth “rescue
therapies” such as infant cooling
• More information from MRI
 Prevention of cerebral palsy
This will only be achieved

when more information is

available about the

multiple causes
 Diagnosis
• Follow up of “at risk” infants, e.g, those of
low birth weight

• Delayed motor development, eg, delay in

learning to sit and stand

• Abnormalities of behaviour
 Early signs of cerebral palsy
• Assymmetric motor development
• fisting of one hand
• early favouring of one hand for reaching
and grasping
• assymmetrical position in crawling
• “limp” when starts to walk
 Early signs of cerebral palsy
• abnormalities of muscle tone

• persistence of primitive reflexes

• feeding problems

• abnormalities of behaviour
 Difficulties in diagnosis
• Different grades of severity

• Wide range of intellectual ability

• Signs may be late in appearance

• Prematurity
 Collaborative perinatal project
• 229 children with abnormal signs during

the first year of life

• At 7 years of age, 118 were free of motor

handicap
 Diagnostic approach
• MRI brain recommended
(American Academy of
Neurology Practice
Parameter)
– Genetic counselling
– Establishing timing
• The majority of children will
have abnormalities
– 80-90 % with MRI
– 75% with CT
 Diagnostic approach
Genetic and metabolic causes are unusual but
should be considered in the presence of a
normal MRI
– the true prevalence of these disorders in CP is
not known
– Sometimes another treatable cause may be
found eg dopa responsive dystonia, biotidinase
deficiency
– Some may have associated problems that need
treatment eg Leisch-Nyhan Disease
What can MRI tell us about timing?
• Brain malformations: 12 – 20
weeks
• Periventricular white matter
injury: 26 – 34 weeks
• Cortical and subcortical gliosis:
36 – 44 weeks
 Confirming the diagnosis

• A period of observation
may be necessary

• Multidisciplinary
assessment may be
helpful
 Issues in management
• Associated disabilities

• Health problems

• Consequences of the

motor disorder
 Associated disabilities
Epilepsy
• Occurs in about 40% of all children with cerebral
palsy
• Most common in spastic quadriplegia (50% - 94%)
and hemiplegia (33% - 50%)
• Higher incidence of refractory seizures and
admissions for status epilepticus (Gururaj et al.
Seizure 12:2;2003)
• Few population based studies
 Associated disabilities
 Hearing problems
• Severe/profound 3-4%

 Visual deficits
• squints
• refractive errors
• field defects
• cortical visual
impairment
 Associated disabilities
Cognitive deficits

• Children may have intellectual, learning and

perceptual problems. Assessment can be
difficult in the presence of severe physical
disability
 Health problems
Nutritional problems
• Under nutrition and failure to
thrive
• Difficulties with sucking and
swallowing
• Tongue thrusting
• Decreased tongue movements
and lip closure
• Hypo or hypersensitive gag
response
• Meal times become long and
carers anxious
 Health problems - undernutrition
• 235 participants with GMFCS 111, 1V and V,
average age 9.7 years
– Indicators of malnutrition common
• 47% of children had weight < 5% for age/gender
• 68% had short stature
– Correlated with increased health care utilisation
and decreased participation in normal activities
(Samson-Fang et al J Pediatr 141;5:2002)
 Gastrostomy
• Avoids aspiration
• Often improves
nutrition
• Saves many hours of
meal time assistance
which can be devoted
to other activities
 Impact of quality of life on carers
At 12 months after gastrostomy
placement, carers reported
• Reduction in feeding times,
increased ease of drug
administration
• Reduced concern about their
child’s nutritional status
• Significant measurable
improvement in quality of life
of carers
(Sullivan et al Dev Med Child
Neurol 46:2004)
 Health problems
• Obesity
• Chronic constipation
– Diet
– Fluid intake
– Laxatives
• Movicol
• Osmolax
• Senokot
• Lactulose
– Enemas
 Health problems
• Gastro-esophageal reflux
• can result in oesophagitis /
gastritis, causing pain and
poor appetite. If severe,
aspiration can result
 Health problems – gastroesophageal
reflux
• Conservative measures
– propping upright after a meal /
thickening feeds / slowing the
rate of feeding
• Medications
– anti-acid medications eg
ranitidine, omeprazole reduce
acidity of stomach and limit
damage produced by acid reflux
– prokinetic agents such as
domperidone may reduce the
amount of reflux
• Fundoplication
 Health problems – lung disease
Lung disease
• caused by aspiration from oromotor dysfunction or
severe gastro-esophageal reflux.
• Coughing or choking during meal times or wheeze
during or after meals may signal the presence of
aspiration
• Videofluoroscopy may be helpful
 Health problems – bone disease
• Increased skeletal fragility
• Increased risk of pathological fractures
– Strategies include
• Education concerning consumption of calcium
• Adequate exposure to sunlight
• Vitamin D preparations
• Use of newer drugs such as bisphosphonates
when indicated
 Health problems
• Dental disease

• Higher frequency of
undescended testes

• Puberty / menstrual issues

• Emotional problems
 Consequences of the motor disorder
• Saliva control problems
• Speech pathology techniques

• Orthodontic appliances

• Medication

Benzhexol hydrochloride

Glycopyrrolate
Consequences of the motor disorder
• Botulinum toxin

• Surgery

Relocation of
submandibular ducts
and excision of
sublingual glands
 Monitoring of adverse effects

• 19 children with cerebral palsy following surgery

• 75 other children with cerebral palsy

• Surgical group had significantly more caries than the

comparison group (P<0.0001)
Consequences of the motor disorder
Incontinence
• cognitive deficits
• lack of opportunity
• inability to communicate
• detrusor overactivity causing urgency and
frequency
 How does one manage equinus?
Tom – aged 3 years
• Walked independently at 27 months
• His calves are now tightening
• He walks on his toes
• Parents are concerned that he is
falling over more and hurting himself
 Assessment of spasticity
1. Is spasticity interfering with
• function
• care
• quality of life
2. Is the spasticity
• localised?
• generalised?
 Spasticity management
• Localised spasticity
• Botulinum toxin A injections
• Orthotics / splinting
• Serial casting
 Botulinum toxin
• Neurotoxin type A
produced by
C.Botulinum
• Injection of BTX-A
results in a reversible
blockade of ACh release
at neuro-muscular
junction
 Generalised spasticity
Kathryn aged 13 years
• Severe spastic quadriplegia
• Can be walked but getting increasingly difficult
• Legs are stiff
• Problems with bathing, dressing and toiletting
 Spasticity management
• Generalised spasticity
– medications
• Diazepam
• Baclofen
• Dantrolene
– intrathecal baclofen
– selective dorsal
rhizotomy
 Role of intrathecal baclofen
• Used for children with

severe spasticity

interfering with care,

comfort and function

 Consequences of the motor disorder
• Orthopaedic problems
• equinus foot deformity
• hamstring overactivity
• subluxed / dislocated hips
• upper limb problems
• scoliosis
 Orthopaedic issues
• In the young child,
mainly issues relating to
subluxed / dislocating
hips
(GMFCS 3,4,5)
• Older child –
lengthening / tendon
transfers, SEMLS
(GMFCS 1,2,3)
 Deterioration in cerebral palsy
• Emotional stress
• Uncontrolled epilepsy
• Worsening contractures
• Intercurrent infection
• Wrong diagnosis
• Inappropriate anticonvulsants
 How do we evaluate what we do?

• Few, or no,
measurement tools
• No means of
evaluation of programs
 Measurement tools

Gross Motor
Function
Measure
 Other tools developed
• PEDI
• WeeFIM
• The Tardieu Scale
• Modified Ashworth
Scale and range of
motion at specific joints
• Goal Attainment Scale
 Assessment of upper limb function

• QUEST
• Melbourne
Assessment of Upper
Limb Function
• MACS
Assessment of upper limb function: The Manual
Abilities Classification Scale
• How children use their
hands when handling
objects in daily activities.
• To reflect the child’s typical
manual performance, not
maximal capacity.
• It classifies what children do
when using one or both of
their hands for activities,
rather than classifying each
hand separately.
Assessment of upper limb function: The Manual
Abilities Classification Scale
• Used for children 4-18
years
• When defining the five
levels of the MACS,
primary criterion was
that the distinctions in
manual ability should
be clinically meaningful.
Assessment of upper limb function: The Manual
Abilities Classification Scale
Level 1: Handles objects easily and successfully
Level 2: Handles most objects but with somewhat
reduced quality and / or speed of achievement
Level 3: Handles objects with difficulty: needs
help to prepare or modify activities
Level 4: Handles a limited selection of easily
managed objects in adapted situations
Level 5: Does not handle objects and has severely
limited ability to perform even simple actions
Communication Function Classification System
• The purpose of the CFCS is to classify the
everyday communication performance of an
individual with cerebral palsy into one of five
levels.
• All methods of communication performance
are considered in determining the CFCS level.
These include the use of speech, gestures,
behaviors, eye gaze, facial expressions, and
augmentative and alternative communication
(AAC).
 Communication
• Signing
– Makaton
– Auslan
• Communication boards
– Pictures
– Compics
– Words
• Electronic communication
devices
 Measurement tools
• Development of a
quality of life
measures for
children with
cerebral palsy CP
 Conventional therapies
• Physiotherapy
– NDT (Bobath)
– Conductive
Education
(Peto)
• Occupational
therapy
• Speech pathology
 Alternative therapies
1. Yearning to “do something”
2. More optimistic approach from alternative
sources, for example, questions are
answered without hesitation
3. Publicity in the media
4. Failure to provide parents with facts about
traditional and acceptable treatment
modalities
 Working with families
• Child is part of a family unit

• A supportive environment
builds self-esteem and
confidence

• Parents need practical help

 Working with families
• Provision of information
is essential

• Parents as partners

• Family centred approach

 Other resources
• Parent support groups
• Association for Children
with a Disability
• Cerebral Palsy Support
Network
• Noah’s Ark
• DHS
• Scope / Yooralla/ CPEC
 Working with families
Health and well-being of
caregivers (468 families)
• Child behaviour
More problems associated with
lower levels of both
psychological and physical
health of the caregivers
(Pediatrics 115:2005)
 Working with families
Health and well-being of
caregivers (468 families)
– Caregiving demands
Less caregiving demands
associated with better physical
and psychological well-being
of caregivers
– Family function
Higher functioning associated
with similar outcomes
Thank you

Thank you