CEREBRAL PALSY (CP)

DEFINITION OF CP
A disorder primarily of movement control and posture
Resulting from a nonprogressive lesion to an immature brain
Occurring in utero, near time of delivery, or within the first three years of life
CP is the leading cause of childhood disability with an incidence of 23 per 1,000 births
The brain injury resulting in CP is limited to prenatal, perinatal, or postnatal periods

RISK FACTORS OF CP: PRENATAL, PERINATAL, AND POSTNATAL

Prenatal Risk Factors

The majority of CP
cases
(70%80%) occur
during the
prenatal period Risk
factors
include the following:
Prenatal
Intracranial
Hemorrhage
outcome
depends on degree of
associated
parenchymal injury
Placental
Complications
Gestational Toxins

Iodine deficiency can

lead
to Diplegia
Organic mercury
intoxication can lead to
quadriplegia
Gestational
Teratogenic
Agents
Congenital
malformations of
the Brain and
Cerebral
vascular occlusions
during

fetal life
Acquired
Congenital
Infections
Torch
Toxoplasmosis
Rubella
CMV
Herpes
Maternal Causes
Seizures
Hyperthyroidism
Mental retardation
Socioeconomic
Factors
Reproductive
Inefficiency
Prenatal HypoxicIschemic
injury as a result of:
Multiple pregnancies
Maternal bleeding
Maternal drug use
Idiopathic (most
common)
Note: Prenatal factors
may lead
to premature birth or
intrauterine growth
retardation
of both term and
preterm
infants

Perinatal Risk
Factors
Complications of
prematurity:
Births at < 32 weeks
gestation
Low birth weight <
2500 gms
Prematurity remains
the most
common antecedent of
CP due to
combination of:
Immaturity
Fragile brain
vasculation
Physical stresses of
immaturity,
combine to predispose
these
children to compromise
of cerebral
blood flow
Blood vessels are
particularly
vulnerable in the
watershed zone
next to the lateral
ventricles in the
capillaries of the
germinal matrix
Complications of
full term infant
Placenta previa

 Abruptio placenta
Meconium aspiration
resulting
in neonatal asphyxia
Hyperbilirubinemia
Rh incompatibility
G6PD
ABO incompatibility
Results in Kernicterus
with
disposition of bilirubin
in the cranial
nerve nuclei and basal
ganglia with
subsequent Athetoid CP
Difficult Traumatic
Delivery
Mechanical trauma to
the brain at
birth can result in CP
(i.e., spastic
hemiplegia)
Infection

Viral
Bacterial
Seizures
Bradycardia and
Hypoxia
Perinatal
Intracranial
Hemorrhage
Outcome depends
on the degree of
associated
parenchymal injury
Postnatal Risk Factor
Trauma
Fall
Child abuse (i.e.,
shaken baby
syndromelook for
retinal hemorrhage)
MVA
Toxins
Especially lead
(heavy metals)

organophosphates
Stroke syndromes
with
hemiplegia which
can
result from
Sickle cell anemia
AVM rupture
Congenital heart
disease (especially
tetralogy of Fallot)
Infection
Bacterial
Viral
Meningitis (especially
in first 6 months)
Neoplastic Disease
Anoxia
i.e., near drowning can
cause anoxic
encephalopathy
Intracranial
hemorrhage

CLASSIFICATION OF CP (see Tables 1024 and 1025)

Current methods try to incorporate a functional basis for classification. CP may manifest
itself differently as the child ages
The modified neurologic classification system divides patients into the following
categories:
1. Spastic (pyramidal) cerebral palsy} 75%
2. Dyskinetic (extrapyramidal) cerebral palsy
3. Mixed types
SPASTIC TYPE (75%)manifest signs of upper motor neuron involvement
Hyperreflexia
Clonus
Extensor Babinski response (abnormal at > 2 years).
Persistent primitive reflexes.
Overflow reflexes such as crossed adductor reflex
The spastic group can be further subdivided by the part of the body that is involved
Spastic monoplegia
Rarely seen, however, has isolated upper or lower extremity involvement and usually a
mild clinical presentation
Spastic diplegia
Primarily lower extremity (LE) involvement with history of prematurity common
Of premature infants that develop CP, 80% are spastic diplegics (most common lesion)
History of intraventricular hemorrhage is typical especially at 2832 weeks of gestation.
MRI imaging in the spastic diplegic may show periventricular leukomalacia or post
hemorrhagic porencephaly
History of early hypotonia followed by spasticity is typical
Developmental delays, commonly in the area of gross motor, result
Lower extremity spasticity is caused by damage to pyramidal fibers within the internal
capsule
Mild incoordination problems result in the upper extremities (UE) with upper motor
neuron findings in the lower extremities
Diplegic gait pattern includes spastic adductors, gastrocnemius muscles, and hip flexors.
Contractures result secondary to spasticity
Ocular findings include strabismus in 50% and visual deficits in 63%
Seizures occur in 20%25% and cognitive impairment in 30%
Spastic triplegia
Involves 3 extremities, classically both lower extremities and one upper extremity
Spasticity results in the involved limbs with mild coordination deficits in the uninvolved
limb
Upper motor neuron signs result with characteristic scissoring and toe walking
Spastic quadriplegia
All extremities are involved with quadriplegics, patterns of truncal hypotonia with
appendicular hypertonia or total body hypertonia exist
Often a history of difficult delivery with evidence of perinatal asphyxia
Approximately 50% have a prenatal origin, 30% perinatal, and 20% postnatal
MRI in the preterm child shows a periventricular leukomalacia
Opisthotonic posturing may begin in infancy, often persisting in the severely involved.
Oromotor dysfunction, pseudobulbar involvement and risk of aspiration with feeding
difficulties occur and may require feeding tube placement
Cognitive involvement results in a large percentage

 Seizures occur in 50% of affected children

Spasticity and persistent primitive reflexes contribute to making these children the most
severely involved of those with cerebral palsy
Spastic hemiplegia
One side of the body is involved, usually the arm more than the leg
The majority are congenital, 70%90%
10%30% are acquired secondary to vascular, inflammatory or traumatic causes
MRI reveals evidence of unilateral lesions in 66% of cases
In term infants, the cause is usually secondary to prenatal events
In premature infants, asymmetric periventricular leukomalacia is a common cause.
Hemiparesis is usually evident by 46 months of age with hypotonia usually being the first
indicator; other indicators include preferential hand use
There is a slightly higher incidence of right-sided involvement
Cranial nerves may be involved, generally presenting as facial weakness
Often growth retardation of the affected side with associated spasticity
Sensory deficits on the ipsilateral side occur in 68%
Visual deficits occur in 25% of hemiplegics, cognitive impairment in 28%, seizures in 33%
Perceptual motor deficits are common, causing learning disabilities
DYSKINETIC TYPES
Dyskinesias are characterized by extrapyramidal movement patterns secondary to abnormal
regulation of tone, defects in postural control and coordination deficits
Athetosis or slow writhing involuntary movements, particularly in the distal extremities
ChoreaAbrupt, irregular jerky movements, usually occurring in the head, neck, and
extremities
ChoreoathetoidCombination of athetosis and choreiform movements. Generally are
large-amplitude involuntary movements. The dominating pattern is the athetoid movement
DystoniaA slow rhythmic movement with tone changes generally found in the trunk
and extremities, associated with abnormal posturing
AtaxiaUnsteadiness with uncoordinated movements, often associated with nystagmus,
dysmetria, and a wide-based gait
Classic movement patterns emerge sometime between 1 and 3 years of age. Severely
affected
children have persistent hypotonia. Movement patterns typically increase with stress or
purposeful activity. During sleep, muscle tone is normal and involuntary movement stops.
Pseudobulbar involvement presents with dysarthria, dysphasia, drooling, and oromotor
dyskinesias. These children have normal intelligence 78% of the time.
There is a high incidence of sensorineural hearing loss which has been associated with
hyperbilirubinemia and neonatal jaundice
MIXED TYPE
This includes descriptions from both spastic and dyskinetic classifications. e.g., spastic
athetoid (predominant dyskinetic movement pattern with underlying component of
spasticity

The modified neurologic classification system divides patients into the following categories:

GROSS MOTOR FUNCTION CLASSIFICATION SYSTEM

This is a functionally based system to standardize gross motor function in the CP child:
Level 1
Walks without restrictions: limitations in more advanced gross motor skills
Level 2
Walks without assistive devices: limitations walking outdoors and in the community
Level 3
Walks with assistive mobility devices: limitations walking outdoors and in the community
Level 4
Self-mobility with limitations: transported or use power mobility outdoors and in the

community
Level 5
Self-mobility severely limited even with use of assistive devices
Typical Gait Abnormalities Include
Spastic diplegia
Scissoring gait pattern
Hips flexed and adducted
Knees flexed with valgus
Ankles in equinus
Spastic hemiplegia
Weak hip flexion and ankle dorsiflexion
Overactive posterior tibialis
Hip hiking or hip circumduction
Supinated foot in stance phase
Upper extremity posturing
Crouch
Tight hip flexors
Tight hamstrings
Weak quadriceps
Excessive dorsiflexion in both diplegic and quadriplegics
Will My Child Walk?
This is usually the most frequent question asked by the parent of a newly diagnosed CP
child.
Several factors are relevant. The best indicator of how the child is going to do is how the
child
is doing.
Sitting: Molnar has shown that if independent sitting occurs by age 2, prognosis for
ambulation is good
Crawl: Badell felt that ability to crawl on hands and knees by 1.52.5 years is a good
prognostic sign
Primitive Reflexes: Persistence of three or more primitive reflexes at 1824 months is a
poor prognostic sign (Table 1028)
Type of CP: See (Table 1025)
ASSOCIATED DEFICITS (Table 1026)
Mental Retardation
The incidence of associated disabilities in cerebral palsy varies. The overall incidence of
mental retardation is approximately 50%. Microcephaly, seizures, and severe neuromuscular
dysfunction are associated with increased risk of intellectual deficit. Spastic quadriparetics,
rigid and atonic types have the highest rate of mental retardation, and spastic hemiparetics
and diplegics the lowest.
Language development, especially speaking in twothree word sentences by age three, is
a good indication of intellectual potential.
Seizures
The overall incidence of seizure in children with CP is approximately 50%. Seizures are more
common in spastic quadriparetics (50%) and less frequent in diplegics and dyskinetics
(25%33%). Grand mal with tonic clonic convulsions is a frequent type.
Visual Deficits
Deficits of extraocular movements and vision are also common in CP. Strabismus is the most
frequent at 25%60% of all cases with the highest rate in spastic diplegics and
quadriparetics.

Esotropia is more frequent than exotropia. Paralysis of conjugate upward gaze is a clinical
manifestation of kernicterus. Nystagmus is present in the ataxic type. A homonymous
hemianopsia
occurs in hemiparetic CP in 25% of cases. Retinopathy of prematurity occurs in preterm
infants.
Hearing Impairment
The characteristic hearing loss in cerebral palsy is a sensory neural impairment, which
occurs
in 12% of CP children < age 15 with a fourfold increased prevalence in athetosis than in
spasticity. Kernicterus is the most common cause of sensory neural hearing loss in athetosis.
Other causes include intrauterine infections, especially rubella, cytomegalovirus,
toxoplasmosis and syphilis, as well as perinatal hypoxia, meningitis, encephalitis, and
ototoxic drugs.
Language Disorders
Developmental language disorders of verbal and written communication also occur. After
one and one-half to two years of age, insults to the dominant hemisphere lead to aphasia.
Although most children show significant recovery from aphasia acquired before 810 years,
they rarely regain premorbid levels. Defective speech results from pseudobulbar palsy and
supranuclear spastic paralysis or dyskinetic incoordination of the muscles innervated by the
lower cranial nerves. Most athetoids and 50% of bilateral spastics have some dysarthria.
Respiratory Impairment
Respiratory impairment may also occur in CP children. Decreased vital capacity and aerobic
working capacity is seen both in spastic and athetoid types. Restrictive pulmonary disease
accompanies scoliosis.
Behavioral Disorders
Disorders of behavior may present with attention deficit, distractibility, disturbances of
impulse control and overt hyperkinesis. Behavior disorders also include true emotional
lability as part of an organic pseudobulbar palsy consisting of dysarthria, drooling and poor
chewing. Poor peer acceptance leading to a negative self-image, school problems,
depression,
and anger may be exacerbated during normal periods of transition, i.e. preschool
kindergarten, early adolescence. The more mildly physically involved child may have more
difficulty and need more psychosocial support.
Gastrointestinal Problems
Symptoms are frequent. Gastroesophageal reflux often requires medical management.
Constipation is exaggerated by immobility and abnormal diet and fluid intake.
Bowel and Bladder Dysfunction
Management is usually related to dysfunction of central neuromotor control and cognitive
development status of the child.
Oromotor Problems
May lead to difficulty swallowing, sucking, and chewing. The motor incoordination is
manifested by poor lip closure, retraction or thrusting of the tongue, and decreased tongue
movements. Feeding difficulties can contribute to malnutrition and aspiration. Dysphagia
evaluation, modified barium swallow, fiber optic endoscopy evaluation may be needed.
Gastric tube may be necessary in certain cases.
Dental Problems
Include malocclusion, enamel dysgenesis secondary to palatal distortions and abnormal
oromotor reflexes. CP children are also at increased risk for cavities due to poor handling of
secretions and food as well as chronic drooling. (Meds, i.e. Scopolamine patches can be used
to address the problem of drooling).