INTRODUCTION TO

CEREBRAL PALSY
5th year
Paediatrics
Mrs Simpamba MM
 Introduction
• Cerebral palsy is one of the leading cause of childhood disability
affecting function and development.

• The term ‘cerebral palsy’ excludes progressive disorders of motor

function, defined as loss of previously acquired skills in the first 5
years of life.

• There are several definitions of Cerebral Palsy within the literature.

 Definition of cerebral palsy

• Cerebral palsy is “a persistent but not unchanging disorder of posture and

movement, caused by damage to the developing nervous system, before
or during birth or in the early months of infancy” (Griffiths & Clegg, 1988).

• “Cerebral Palsy is a group of permanent, but not unchanging, disorders of

movement and/or posture and of motor function, which are due to a non-
progressive interference, lesion, or abnormality of the
developing/immature brain” (Cans, 2000).
 ICF definition
• “Cerebral palsy describes a group of permanent disorders of the
development of movement and posture, causing activity
limitations that are attributed to non-progressive disturbances that
occurred in the developing foetal or infant brain”.

• The motor disorders of cerebral palsy are often accompanied by

disturbances of sensation, perception, cognition, communication,
behaviour, by epilepsy, and by secondary musculoskeletal
problems” (Rosenbaum et al., 2007).
Although the definitions may all vary slightly in the way they are
worded, they all have similar core components which are:

1. An umbrella term or group of disorders;

2. Disorder of movement and posture;

3. Non-progressive interference/ lesion or abnormality of the brain;

4. This affects the developing/immature brain;

5. A permanent but not unchanging disorder.

 AETIOLOGY
• Aetiology of cerebral palsy is important for developing preventive strategies as
well as communicating with parents and children themselves.

• The aetiology of Cerebral Palsy is very diverse and multifactorial.

Concept of cause and risk factors

- A cause is an event, characteristic or condition that precedes the disease and
without which the disease could not have occurred.

- A risk factor is any attribute, characteristic or exposure of an individual that

increases the likelihood of developing a disease or injury.
 Causes of cerebral palsy
• It is important to view causes of CP in three broad categories namely:
- Brain damage,
- Brain malformation and
- disorders of brain function without evidence of structural
malformation (Eunson, 2012).

• These causes are not mutually exclusive.

• Specific causes could be congenital, genetic, inflammatory, infectious,

anoxic, traumatic or metabolic disorders.
• The following types of brain damage can result in CP
symptoms:

- Damage to the white matter of the brain (periventricular

leukomalacia, or PVL)

- Abnormal development of the brain (cerebral dysgenesis

- Bleeding in the brain (intracranial hemorrhage)

- Severe lack of oxygen in the brain

• The motor impairments in CP are strongly associated with the
cerebral pathology.

- E.g. Spastic CP primarily arise from damage to the cerebral cortex

and subcortical white matter;

- Basal ganglia damage is associated with dyskinesia;

- and cerebellar lesions are associated with ataxic CP.

 Causes of spastic cp and outcomes
Cause Lesion Outcome

1. Infection in utero 1. Bilateral PVWMI; Diplegia

2. Widespread cortical lesions Quadriplegia

2. Prematurity 1. Bilateral PVWMI Diplegia

2. Unilateral PVWMI Hemiplegia

3. Focal cortical dysplasia or Unilateral PVWMI Hemiplegia

unilateral malformation or unilateral cortical lesion

4. Unilateral stroke Unilateral cortical lesion Hemiplegia

5. Birth asphyxia Widespread cortical lesions Quadriplegia

 Pathophysiology of cerebral palsy
• Cerebral palsy is a heterogeneous condition with multiple causes,
multiple clinical types, multiple pathologies and multiple
associated developmental pathologies.

• Despite the heterogeneity of CP, the primary pathology is in the

brain.

• Cerebral palsy may result from interaction of many causes/risk

factors, and in some cases, no identifiable cause may be found.

• The pathogenesis of the brain damage varies depending on the

cause.
• The major pathophysiology include the following:

- Brain injury or abnormal brain development. E.g. Periventricular white matter injury
in preterm (due to cerebral hemorrhage or ischemia)

- Perinatal infarcts of middle cerebral artery,

- Generalised anoxia at birth (asphyxia),

- Intrauterine infections,

- Genetic predisposition to infarction,

- CNS malformations,

- Hyper-bilirubinaemia (or neonatal jaundice/kernicterus).

 RISK FACTORS OF CEREBRAL PALSY
• The damage to the brain may occur during prenatal, perinatal or
postnatal period.

• Over 75%-80% of the CP cases are due to prenatal injury with less
than 10% being due to significant birth trauma or asphyxia (Sankar
& Mundkar, 2005).
 PRENATAL RISK FACTORS
• Maternal poor nutritional status

• Multiple pregnancies.

• Infertility treatments.

• Maternal high blood pressure

• Maternal intrauterine infections such as TORCH infections and chorioamnionitis. (TORCH:

Toxoplasmosis, others (syphilis, hepatitis B), rubella, cytomegalovirus, herpes simplex)

• Less common ones include metabolic disorders, congenital defects, maternal ingestion of
toxins, drug abuse and trauma.
 PERINATAL RISK FACTORS
• Obstructed/prolonged labour (Birth asphyxia/fetal distress)
• Antepartum haemorrhage
• Pre-eclampsia (Due to maternal hypertension)
• Neonatal hyperbilirubinemia (Severe jaundice) due to prematurity or RH
incompatibility
• Breech presentation
• Placental Insufficiency/premature detachment or rupture of placenta
(Placenta praevia)
• Cord prolapse
• Multiple births
• Birth trauma (Forceps delivery)
 POST NATAL RISK FACTORS

• Premature birth and low birth weight • Cerebral vascular accidents (Stroke,

• Central Nervous System infection e.g. SCD)

(bacterial, viral or fungal infections) • Postnatal trauma (head injury)

• Encephalopathies • Hypoxia (Seizures, drowning)

• Intracranial hemorrhages • Coagulopathies (Bleeding disorders)

• Toxicities
 Timing of brain damage
• It is usually difficult to determine the nature and the exact timing of
the damaging event.

• In the absence of clear evidence for perinatal or postnatal cause,

the current trend is to assume a prenatal cause.

• Methods used to determine time when the brain damage occurred

include:
- Obstetric history of adverse events.

- Monitoring infant behaviour using parameters such as Apgar

scores or cord blood gases.
• Ultrasound of the brain in the neonatal period and
magnetic resonance imaging (MRI) of the brain.

NB: Apgar scores and blood gases are usually poor predictors
of cerebral palsy.
 DIAGNOSIS OF CEREBRAL PALSY
1. Neuro-imaging:

• Cranial ultrasound: Captures brain images of infants but can’t successfully capture
details of the damage

• Computed Tomography (CT): Shows brain structure and areas of damage but less is
precise.

• Magnetic Resonance Imaging (MRI): Shows location and type of brain damage in more
detail

2. Electroencephalogram (EEG): Detects electric activity in the brain

 DIAGNOSIS OF CEREBRAL PALSY
4. Physical dentification of clues and risk factors

• Initially the parents may complain of failure or delayed achievement motor

milestones.

• Clinical diagnosis of CP can be made by an awareness of risk factors,

developmental screening and neurological examination.

• Carrying out a systematic assessment focusing on maternal, obstetric and

perinatal histories and review of developmental milestones.

• Thorough neurological examination and observation of the child in various

positions such as supine, prone, sitting, standing, walking and running.
 EARLY SIGNS OF CEREBRAL PALSY
Early signs of CP include:
• Hand preference in the first year
• Prominent fisting of hand
• Tone abnormalities (Spasticity or hypotonia)
• Persistence of neonatal reflexes
• Delay in emergence of protective and postural reflexes
• Asymmetrical movements and hyperreflexia
 Differential diagnosis of cerebral palsy
1. Early infancy neuromuscular myopathies may be mistaken for
cerebral palsy, especially when child is in hypotonic phase, e.g
spinal muscular atrophy.

2. Intellectual disability: Some children with mental retardation may

have hypotonia but without abnormal patterns.

3. Neurodegenerative/genetic disorders: E.g. Idiopathic degenerative

encephalopathy; hereditary spastic paraplegia.

4. CNS tumours or injuries which delay development and may be

outgrown.
 EPIDEMIOLOGY
• Cerebral palsy is an important epidemiological problem because
of:
- its severity,

- the consequent burden that it places on affected children, their

families, and societies,

- And its high frequency as a cause of disability in childhood.

• Understanding the epidemiology of CP is important for planning
services as well as informing preventive strategies.

• Previously, the causes of CP were mainly attributed to birth trauma

due to poor maternal and neonatal care.

• This led to improvements in maternal and neonatal care with

subsequent decline in infant mortality and increased survival of
premature babies.
• This increased survival rate has subsequently resulted in increase in
incidence/prevalence of severe cases of children with CP.

• Cerebral Palsy still remains a common and significant disorder of motor

development, with a global incidence of 2-2.5 per 1000 live births
(Eunson,2012).

• The risk of Cerebral Palsy increases with decreasing gestational age

at birth.
• Recent studies on CP showed that CP is more common among the
following:
- boys than among girls;
- black children than white;

• Most CP children had spastic type; over half could walk; many had
at least one associated impairment with epilepsy present in about
41% (Christensen, et al. 2014).

• WHO estimates show that majority of the world’s disabled

population live in developing countries, hence it is expected that
rates of CP in these settings are high (Gladstone, 2010).
 ASSOCIATED IMPAIRMENTS
• Respiratory difficulties: These may be due to difficulties in swallowing, low
activity levels, excessive drooling, blocked airways, and the inability to cough.

• Speech impairment: Speech problems may be caused by inability to control and

coordinate muscles used for speech such as lips, tongue, vocal cords and
diaphragm.

• Feeding disorders: Difficulties controlling muscles of the mouth, tongue and

throat may lead to problems with chewing, dysphagia (Difficulty swallowing) and
drooling.

• Hearing impairment: Hearing impairments are common in children who had

kernicterus, premature birth or those exposed to toxic drugs.
• Epilepsy/Seizures: Over half of children with CP have one or more
seizures. Children with seizures are more likely to have intellectual
disabilities

• Learning/ Intellectual/cognitive disabilities: CP children may

experience learning difficulties in areas involving basic learning
skills, organisation, abstract reasoning, attention, motor planning,
perception and language.

• A child with intellectual disabilities may have limitations in both

cognitive functioning and adaptive behaviour.
• Vision impairment: Visual impairments in CP children include cerebral
visual impairment, strabismus (squint) and hyperopia (Long sightedness).

• In CVI, the eyes will look normal but affected children can not make
sense of what they see.

• Perceptual difficulties: Perception is the making sense of information

gained from the senses. Children with perceptual problems have
difficulties making sense of the information they take in through their
eyes and/or ears, which can impact many areas of learning.

• Communication / Language difficulties: Children with cerebral palsy may

have difficulties with understanding and expressing ideas.
• Delayed growth and development: Children with CP especially those with spastic
quadriplegia often lag behind in growth and development.

• Musculoskeletal disorders: Contractures and deformities, spinal deformities and

osteoarthritis are common.

• Infections and long term illnesses: Many children and adults with CP have higher risk of
heart and lung diseases and pneumonia than the general population.

• Malnutrition: Feeding difficulties makes it difficult to get proper nutrition and weight gain.

• Dental problems: Children with CP have higher risk of developing dental problems due to
poor dental hygiene and medications (Seizure drugs)

• Inactivity: Movement impairments make it difficult for children to participate in sports

and other physical activities.
 CLASSIFICATION OF CEREBRAL PALSY
• The importance of using a classification system for children with
Cerebral Palsy is that it provides a common language and enables
clinicians to develop a treatment plan.

• Classification is also useful for description, prediction, comparison

and evaluation of change in children with CP (Rosenbaum et al.,
2007).

• It has not been easy to come up with a reliable and valid

classification of CP due to its heterogeneous nature which makes
classification a complex process.
 Methods of classification

The many ways of classifying CP include:

1. Severity

2. Anatomical distribution of motor disorders

3. Nature of motor disorders

4. Functional classification
 1. SEVERITY
• Cerebral Palsy is often classified by severity level as Mild, Moderate, Severe.

• This type of classification provides little specific information, especially when

compared to other means of Classification.

1. Mild: The child can move without assistance; his or her daily activities are not
limited.

2. Moderate: The child will need braces, medications, and adaptive technology to
accomplish daily activities.

3. Severe: The child will require a wheelchair and will have significant challenges in
accomplishing daily activities and will need important support.
 2. Anatomic (Topographic)
• Anatomically, the classification of CP refers to body parts affected.

• Because most non-spastic CPs involves all 4 limbs, the anatomic classification
is restricted to spastic CPs.

1. Diplegia: Lower limbs more affected than upper limbs.

2. Hemiplegia: Unilateral upper and lower limb involvement.

3. Quadriplegia/double hemiplegia: All 4 limbs are involved with either upper

limbs or one side more involved.
Anatomic (Topographic) cont.
 3. Nature of motor disorders
• This type of classification refers to the type of abnormal muscle tone and abnormalities
of movements.

• These abnormalities are usually related to underlying pathophysiology.

• For example, spasticity is associated with pyramidal disorders while involuntary

movements are associated with extrapyramidal disorders.

• The major types under this category are spastic, dyskinetic (athetoid), ataxia and mixed.

• Dyskinesia is further classified into dystonia and choreoathetosis.

• Hypotonia is rare because children usually grow up to be dyskinetic, spastic or ataxic.

 3 a. Spastic cerebral palsy
- Spastic CP is characterised by at least two of the following symptoms:

- Abnormal pattern of posture and/or movement

- Increased tone (not necessarily constantly)

- Pathological reflexes

• Spasticity can be classified using tools such as Modified Ashworth scale on a 0-5
scale.

• Spasticity can further be classified into severe and moderate

 Features of severe spasticity
• Exaggerated co-contraction

• Tone unchanging with changing conditions

• Tone increased proximally more than distally

• Little or no movement

• Whatever movement is available occurs only in middle range

• Difficulty in initiating movement

• Difficulty in adjusting to being moved or handled

• No balance or protective reactions

• Poor righting reactions

• Associated reactions causing increased spasticity

 Features of moderate spasticity
• Changeable hypertonus rising from relatively normal at rest to high or very high with
stimulation, effort, speech or emotion (particularly fear).

• Poor balance and protective responses

• Spasticity more distal than proximal

• Associated reactions, seen as movements, likely to increase spasticity as child uses effort to
function

• Child likely to move and function using stereotyped abnormal patterns

• Total patterns of flexion or extension which are likely to be compensatory i.e. flexion in
lower limbs with extension in upper and vice versa.
 3 b. Dyskinetic cerebral palsy
• Characterised by abnormal patterns of posture and/or involuntary
movements and occasionally stereotyped movements of the
affected body parts

• Dyskinetic Cerebral Palsy may be dystonic or choreoathetosis.

• Dyskinetic Cerebral Palsy may be due to Hyperbilirubinemia or

severe anoxia resulting in damage to the basal ganglia.
1. Features of choreoathetosis:

• Constant fluctuations in tone between abnormally high and abnormally low

• Involuntary movements

• Lack of adequate co-contraction leading to difficulty in sustaining postural control against

gravity and poor proximal fixation

• Inadequate balance and protective responses

• Marked Asymmetry

• Lack of grading of movement

• Child dislikes being held still.

2. Features of dystonia

• Tone fluctuates between fairly low and very high, staying high for longer than in
choreoathetosis.

• Lack of proximal fixation

• Dystonic spasms causing twisting non-purposeful patterns that are sustained

sometimes for minutes at a time

• Very marked asymmetry

• Danger of contractures and deformities such as scoliosis

• Older children show more spasticity and their picture can look like spastic quadriplegia
 3 c. Ataxic cerebral palsy
Features of ataxic CP:

• Postural tone is fairly low to normal and the child can move, and hold some postures
against gravity.

• Co-contraction is poor causing difficulty in holding steady postures.

• Proximal fixation is not effective for carrying out selective movements.

• There may be an intention tremor and jerky quality of movement especially with effort
and up against gravity.

• Inadequate balance reactions and slow or delayed protective responses

 3 d. Features of hypotonia
• Child takes up all available support.

• Poor head and trunk control.

• Child doesn’t move much.

• Joints are hypermobile (wide range of movement).

• Child doesn’t respond even to quite strong stimulation.

• Associated problems such as poor vision, hearing, speech and feeding are very
common
 4. Functional classification
Types of functional classifications include the following:

1. Gross motor functional classification

2. Manual Ability Classification System (MACS)

3. Communication Function Classification System (CFCS)

4 a) Gross motor functional classification system (GMFCS)

•The GMFCS was developed to provide a standardised system for

classifying the severity of movement disability in children with CP.

•Consists of a five-level system, ranking severity of motor involvement

based on age, motor ability and use of assistive technology.

•Two versions: Initial version and the expanded and revised version
• The initial GMFCS provided descriptions for prescribed age bands
such as, less than 2 years, between 2-4 years, between 4-6 years,
between and 6-12 years.

• The expanded and revised (E & R) GMFCS includes an age band for
youths between 12-18 years and emphasizes on the WHO’s
concepts of international classification of functioning, disability and
health (ICF).
 Functional classification
GMFCS Level Description

Level I Walks without limitations

Level II Walks with limitations

Level III Walks using a hand held mobility device

Self-mobility with great limitations and

Level IV
may use powered mobility
Has no means of independent mobility.
Level V
Transported in a manual wheel chair
 Manual Ability Classification System (MACS)
• The MACS describes how children with cerebral palsy use their
hands to handle objects in daily activities.

• It is a five level scale based on the children's self-initiated ability to

handle objects and their need for assistance or adaptation to
perform manual activities in everyday life.

• The children are assessed for age appropriate skills in activities such
as eating, dressing, playing, drawing.

• MACS can be used for children aged 4-18 years.

Manual Ability Classification System (MAC)
Level Description
I Handles objects easily and successfully.
II Handles most objects but with somewhat reduced
quality and/or speed of achievement.
III Handles objects with difficulty; needs help to prepare
and /or modify activities.
IV Handles a limited selection of easily managed objects
in adapted situations.
V Does not handle objects and has severely limited
ability to perform even simple actions.
Communication Function Classification System (CFCS)
• The CFCS provides 5 levels (CFCS I, II, III, IV, V) to describe everyday
communication performance.
 
• It was originally developed for individuals with CP but can now be used for
individuals with any disabilities.

• Communication occurs when a sender transmits a message and a receiver

understands the message.

• An effective communicator alternates as a sender and receiver regardless of

the demands of conversation.
Communication Function Classification System for
individuals with cerebral palsy
Level Description
I Effective Sender and Receiver with unfamiliar and familiar
partners.

II Effective but slower paced Sender and/or Receiver with

unfamiliar and/or familiar partners.

III Effective Sender and Receiver with familiar partners

IV Inconsistent Sender and/or Receiver with familiar partners
V Seldom Effective Sender and Receiver even with familiar
partners.
 REFERENCES
• Bax, M., Goldstein, M., Rosenbaum, P., Leviton, A., Paneth, N., Dan, B., Jacobsson,
B. and Damiano, D., 2005. Proposed definition and classification of cerebral palsy,
April 2005. Developmental Medicine & Child Neurology, 47(08), pp.571-576.
• Blair, E., 2010. Epidemiology of the cerebral palsies. Orthopedic Clinics of North
America, 41(4), pp.441-455.
• Cans, C., 2000. Surveillance of cerebral palsy in Europe: a collaboration of cerebral
palsy surveys and registers. Developmental Medicine & Child Neurology, 42(12),
pp.816-824.
• Clowry, G.J., Basuodan, R. and Chan, F., 2014. What are the best animal models
for testing early intervention in cerebral palsy?.