CEREBRAL PALSY

Definition
Disability primarily of movement control and posture resulting from a non progressive lesion to an
immature brain occurring in utero, near the time of delivery or within the first 3 years of life. (DeLisa)
A heterogeneous spectrum of clinical syndromes characterized by alteration in muscle tone, deep tendon
reflexes, primitive reflexes and postural reactions. (Molnar)
Cerebral palsy is a disorder of movement, muscle tone or posture that is caused by injury or abnormal
development in the immature brain, most often before birth.
Epidemiology
Leading cause of childhood disability
Approximately 2-3 per 1000 live births
More prevalent on deprived socio-economic population
In a study from Europe, the rate was more than 70 times higher in infants with birth weight <1500 g than
in those with birth weight >2500 g
Despite marked improvement in maternal and perinatal care, the prevalence has remained relatively
constant
Etiology
The origin of the brain injury resulting in cerebral palsy may occur during the prenatal, perinatal or
postnatal period.
Risk Factors:
Prenatal Risk Factors
o Majority of CP cases (70-80%) occur during prenatal period
o Prenatal Intracranial Hemorrhage
- outcome depends on degree of associated parenchymal injury
o Placental Complications
o Gestational Toxins
- Iodine deficiency can lead to diplegia
- Organic mercury intoxication can lead to quadriplegia
o Gestational Teralogenic Agents
o Congenital malformation of the brain and cerebral vascular occlusion during fetal life
o Acquired congenital infections:
- TORCH (Toxoplasmosis, Rubella, CMV, Herpes)
o Maternal Causes
- Seizure
- Hyperthyroidism
- Mental Retardation
o Socioeconomic Factors
o Reproduction inefficiency
o Prenatal Hypoxic-Ischemic injury as a result of:
- Multiple pregnancies
- Maternal bleeding
- Maternal drug use
- Idiopathic (most common)
Perinatal Risk Factors
o Complications of prematurity: Births at <32 weeks gestation
o Low birth weight <2500 gms
o 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
o Complications of full term infant
- Placenta previa
- Abruptio placenta
- Meconium aspiration resulting in neonatal asphyxia
o Hyperbilirubinemia
- Rh incompatibility
- G6PD
- ABO incompatibility
o Difficult Traumatic Delivery
- Mechanical trauma to the brain at birth can result into CP
o Infection
- Viral
- Bacterial
o Seizures
o Bradycardia and Hypoxia
o Perinatal Intracranial Hemorrhage
- Outcome depends on the degree of associated parenchymal injury
Postnatal Risk Factor
o Trauma
- Fall
- Child abuse (shaken baby syndrome look for retinal hemorrhage)
- MVA
o Toxins
- Lead (heavy metals)
- Organophosphates
o Stroke syndrome with hemiplegia which can result from
- Sickle-cell anemia
- AVM rapture
- Congenital heart dse (esp. Tetralogy of fallot)
o Infection
- Bacterial
- Viral
- Meningitis
o Neoplastic Dse
o Anoxia
o Intacranial Hemorrhage

CLASSIFICATION
Neurologic Classification
Current classification methods try to incorporate a functional basis for classification. CP may manifest
itself differently as the child ages
Modified neurologic classification system divides patients into ff. categories:
o Spastic (pyramidal) CP 75%
o Dyskinetic (extrapyramidal) CP
o Mixed type
Spastic Type most commonly occurring, manifest upper motor neuron involvement.
Includes the following:
o Hyperreflexia
o Clonus
o Extensor Babinski response
o Persistent primitive reflexes
o Overflow reflexes such as crossed adductor
Further subdivided into:
o Monoplegia one limb, either an arm or leg
o Diplegia primarily lower extremities are involved
o Triplegia Three extremities
o Quadriplegia all four extremities and trunk involved
o Hemiplegia one side of the body involved, including arm and leg
Description of each types:
o Monoplegia
Isolated upper and lower extremity involvement
Rarely seen
Usually mild clinical presentation
Often the presentation of misdiagnosed hemiplegia

o Diplegia (Littles Disease)
Etiology:
History of prematurity common; in this group of preterm infants who eventually
develop CP, 80% evolve into spastic diplegia
History of intraventricular hemorrhage, particularly 28-32 weeks of gestation
MRI imaging may show periventricular leukomalacia or posthemorrhagic
porencephaly
More complex multifactorial cause in the term infant, although in 28% no
identifiable risk factor present
History of early hypotonia followed by spasticity
Developmental delays most pronounced in the gross motor sector
Lower extremity spasticity caused by damage to pyramidal tracts within the
internal capsule
Characteristics:
- Upper motor neuron signs in the lower extremity
- Diplegic gait pattern, including spastic adductors, gastrocnemius and hip flexors
- Disproportionate involvement of the legs,
- Developmental delay in gross motor skills
- Ocular findings: strabismus in 50% of the cases (visual deficits in 63%)
- Mild or no mental retardation
- Seizures occur in 25%
- Cognitive impairment in 30% (mild retardation)
- Initially there is a period of hypotonicity, later these patients manifest spasticity in
the LE.

o Triplegia
Characteristics:
Three extremities involved, classically bilateral lower extremity and one upper
extremity
Spasticity in involved extremities
Mild coordination problems in non-involved limbs
Upper neuron signs in involve limbs
Scissoring and toe walking
Similar features to the spastic quadriplegia

o Quadriplegia
Etiology:
History of difficult delivery with evidence of perinatal asphyxia common
Approximately 50% prenatal in origin; 30% perinatal, 20% postnatal
MRI imaging in preterm children showed periventricular leukomalacia;full term
babies had term-type lesion and brain anomalies
Characteristics:
- All extremity involved; patterns include trunk hypotonia with appendicular
hypertonia or total body hypertonia
- Individuals with more involvement in the upper extremity than the lower
extremities categorized into double hemiplegia
- Opisthotonic posturing may begin in infancy, often persisting in the severely
involved
- Oromotor dysfunction
- Pseudobulbar involvement
- Risk for aspiration
- Strabismus
- Mental retardation can be significant
- UMNL signs in all limbs
- Must be monitored for hip dislocation and scoliosis

o Hemiplegia
Etiology:
Majority are congenital, 70-90%
10-30% are acquired secondary to vascular, inflammatory or traumatic causes
MRI reveals evidence of unilateral lesion of 66%
In term infants, the cause is usually secondary to prenatal events
In premature infants, asymmetric periventricular leukomalacia is a common
cause.
Characteristics
- One side of the body involved, usually the arm more than the leg
- 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 Type
Dyskinesias are characterized by extrapyramidal movement patterns secondary to abnormal
regulation of tone, defects in postural control and coordination deficits
o Athetosis or slow writhing involuntary movements, particularly in the distal extremities
o ChoreaAbrupt, irregular jerky movements, usually occurring in the head, neck, and
o extremities
o ChoreoathetoidCombination of athetosis and choreiform movements. Generally are
o large-amplitude involuntary movements. The dominating pattern is the athetoid
movement
o DystoniaA slow rhythmic movement with tone changes generally found in the trunk
o and extremities, associated with abnormal posturing
o AtaxiaUnsteadiness with uncoordinated movements, often associated with nystagmus,
o 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)

Gross Motor Function Classification System

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

Spastic diplegia
o Scissoring gait pattern
o Hips flexed and adducted
o Knees flexed with valgus
o Ankles in equinus

Spastic hemiplegia
o Weak hip flexion and ankle dorsiflexion
o Overactive posterior tibialis
o Hip hiking or hip circumduction
o Supinated foot in stance phase
o Upper extremity posturing

Crouch
o Tight hip flexors
o Tight hamstrings
o Weak quadriceps
o Excessive dorsiflexion in both diplegic and quadriplegics

Associated Deficits
Mental retardation 50% incidence, most common in rigid atonic, and severely spastic quadriplegia
Seizures 50% incidence, most frequent in hemiplegia and spastic quadriplegia
Oromotor
Difficulty sucking, swallowing, and chewing; poor lip closure, tongue thrust,
drooling, dysarthria; most common in spastic quadriplegia, dyskinetic
Gastrointestinal Reflux Constipation
Dental Enamel dysgenesis, malocclusion, caries, gingival hyperplasia
Visual Strabismus, refractory errors; hemianopsia in hemiplegia
Hearing Impairment Infection (TORCH), medications, bilirubin encephalopathy
Cortical Sensory Deficit Hemiplegia
Pulmonary
Deficient ventilation, bronchopulmonary dysplasia in premature infants;
microaspirations with oromotor dysfunction
Bowel and Bladder
Dysfunction of central neuromotor control and cognitive developmental status of the
child
Behavior
Attention deficit disorder, distractibility, impulse control, overt hyperkinesis, organic
pseudobulbar palsy
Language Disorders
Developmental, pseudobulbar supranuclear spastic paralysis, incoordination of
muscles innervated by the lower cranial nerves

MANGEMENT
Therapeutic Management:
PhelpsUses extensive bracing, withdrawing support as motion is performed with a minimum of tension,
overflow and substitution
DeaverUses extensive bracing, limiting all but two motions of an extremity
Doman and DelacatoA series of set patterns repeated many times during the day, attempting to train
cerebral dominance and normalization of function
RoodEmphasizes sensory and motor systems equally, activating muscles through sensory receptors
Bobath (most widely used)Neurodevelopmental treatment to normalize tone, inhibit abnormal
primitive reflex patterns and facilitate automatic reactions and subsequent normal development
Vojta-European methodActivates postural development and equilibrium reactions to guide normal
development
Conductive educationBased on theory that difficulties with motor dysfunction are problems of learning
Spasticity Management:
The mainstay of treatment is through the application of modalities, primarily therapeutic exercise, range of
motion, hot and cold application, as well as casting and splinting

Nerve/Motor Point Blocks
o Decreased injection site pain and discomfort.
o Indicated for spasticity affecting specific muscle groups
o They are commonly done to decrease scissoring due to adductor spasticity and equinovarus foot
deformity during gait and hamstring tightness.
Botulinum Toxin
o Botulinum toxin affects the neuromuscular junction (NMJ) with essentially the same results
o Advantages over nerve blocks include:
Less technically demanding on clinicians
Does not cause dysesthesia

Surgical Procedures
Neurosurgical Procedures
o Neurosurgical procedures have been employed for tone and management.
o Selective Posterior Rhizotomy (SPR)
SPR is a neurosurgical procedure designed to decrease the excitatory input to the motor
neuron, thereby decreasing spasticity.
This technique allows for decreased tone without significant sensation loss.
Patient selection criteria include:
- Lack of dystonia and/or athetosis
- Preservation of functional strength independent of spasticity
- Presence of selective motor control Younger age (3-8 yrs.)
- Lack of significant joint contractures and few previous orthopaedic procedures
- Cognitive preservation, motivation and positive family supports are important.
Poor Candidates for SPR Include children with poor head and trunk control and children
who use spasticity for functional purposes, (i.e. extensor spasms to stand).
Negative effects of SPR include:
- Hypotonia (usually transitory immediately post-op, but occasionally lasting 6 months)
- Weakness (unmasked by reduction of tone)
- Sensory changes and bladder dysfunction (both usually of brief duration)
- Hip dislocation (thought to be exacerbated by sparing of L1 root leading to
unbalanced hip flexor spasticity)
- Spinal deformity (lordosis possibly secondary to sparing of L1)
- After surgery, the children require an extensive physical and occupational therapy
program to recover from post-op weakness to maximize functional gains.

Baclofen Pump
o The baclofen pump delivers baclofen directly to the spinal cord performing a chemically
adjustable rhizotomy and minimizing side effects.
o Of course, implanted devices have their own problems and they need to be filled on a regular
basis.

Ankle Foot Orthoses (AFO)
o AFOs aid in gait by controlling the equinus or equino varus deformity.
Tone Reducing AFOs (TRAFOs)
- TRAFOs have certain features designed to decrease abnormal reflexes,
including a foot plate that extends past the toe to discourage toe flexion and a
metatarsal support to discourage stimulation to a particularly reflexogenic area of
the foot.
- They are most effective during gait, but use during rest helps prevent
contractures.
Knee Ankle Foot Orthoses (KAFO)
- KAFOs add direct control over knee flexion and extension as well as varus and
valgus, but add bulk and weight.
Hip Knee Ankle Foot Orthoses (HKAFO)
- HKAFOs add direct control over hip position. Neither of the two (KAFO or
HKAFO) braces significantly improve gait but they do prevent deformity.

PROGNOSIS

Positive factors for independent living
- Regular schooling
- Completion of secondary school
- Independent mobility and ability to travel beyond the house
- Good hand skills, living in a small community, and having spasticity as the motor dysfunction.
- Mental retardation, seizures, and wheelchair dependency are factors that reduce the likelihood of
independent living.
Positive prognostic indicators for employment
- Mild physical involvement
- Good family support
- Vocational training
- Good employment contracts
- Immobility and severe or profound retardation reduce life expectancy
- About 90% of children with CP survive to adulthood


AGING WITH CEREBRAL PALSY
- One of the most common complaints is neck pain, 50% in spastic patients, and 75% in the dyskinetic
group.
- Scoliosis has a much higher incidence in nonambulatory individuals.

VOCATIONAL ASPECTS

Competitive/able to work
- IQ > 80
- Ambulation with or without assistive device
- Speech hard to understand to normal
- Hand use normal to requiring assistance

Sheltered employment
- IQ between 50 and 79
- Ambulation with or without assistive devices
- Speech hard to understand to normal
- Hand use normal to requiring assistance

Unemployable/unable to work
- IQ < 50
- Nonambulatory and nonoral
- Requires assistance using hand