CEREBRAL PALSY

1. DISEASE/DISORDER:
Definition
Cerebral Palsy (CP) is a group of disorders of the development of movement and posture, causing
activity limitations that are attributed to nonprogressive disturbances that occurred in the developing
fetal or infant brain.1

Etiology
1. Defect or lesion in the developing brain during prenatal, perinatal, or postnatal period. 2
2. Major contributing factors include prematurity, coagulopathy with intrauterine stroke, and
intrauterine or fetal infection and inflammation.2
3. However, the majority of cases in term infants have no identifiable etiology.2

Epidemiology including risk factors3

1. Cerebral palsy is the most common motor disability in childhood. Overall incidence of CP is
1.5 to 4/1000 live births, with prevalence being the highest among African-Americans
children and in boys. This rate remained constant for nearly 50 years. 3
2. Incidence in Sweden data in term infants is 1.4/1000 live births; in 32-36 weeks gestation it is
6.1/1000 live births, and in premature infants (less than 28 weeks 43.7/1000 live births.
Prematurity is the single most important risk factor for CP.3
3. A study in Atlanta found that prevalence of CP based on weight showed to be 6.2/1000 births
for weight 1500 to 2499 grams, 59.5/1000 livebirths for children born weight less than 1500g,
and 1.1/1000 children born weight 2500 g or more. 3,4
4. A study in Denmark found that children born after in vitro fertilization were 1.6 times as
likely to have CP.3
5. Children born as part of a multiple birth pregnancy were found to be almost 5 times more
likely to have CP than children born as singletons.3
6. Maternal genito-urinary tract infections are associated with CP in all birth. Congenital
TORCH infections are also risk factors.3
7. Spasticity is the most frequent motor event (60%).3

Patho-anatomy/physiology
1. CP has possible multiple causes: Congenital, prenatal, perinatal brain injury and postnatal
causes.2
2. Spastic diplegia is commonly seen in premature infants and is associated with intraventricular
hemorrhage leading to periventricular leukomalacia (PVL).
3. Spastic hemiparesis is commonly secondary to focal cortical infarcts (middle cerebral artery). 5
4. Dystonic CP is associated with deep gray matter or subcortical infarctions affecting the basal
ganglia or thalamus.6
5. Developmental brain malformations are a factor in the development of CP. In general, insults
during the first trimesters are associated with cerebral maldevelopment such as
schizencephaly; in the second trimester, with periventricular white matter damage; and in the
third trimester, with cortical and deep grey matter damage. 7
6. Postneonatal cause is about 10% of all causes of CP. Majority are attributed to CNS infections
such as meningoencephalitis and brain injury. Traumatic brain injury or stroke in the young
child can lead to hemiparetic or tetraplegic CP.7
7. Hypoxic brain injury can also cause CP in in 10-20% due to encephalopathy.4 In order to
diagnose CP due to hypoxic event, a metabolic acidosis, early moderate to severe neonatal
 encephalopathy, CP of spastic quadriplegic or dyskinetic type and exclusion of other
identifiable causes of CP must be met.8
8. Infections including chorioamnionitis has been found to account for 12% to 28% of CP.9
9. Direct genetic causes or genetic variant causing susceptibility to insults causing CP is also
being investigated. Mutation of KANK1, AP4MI, and GAD1 gene mutations have shown to
cause CP.10

Disease progression including natural history, disease phases or stages,

disease trajectory (clinical features and presentation over time)
Although the underlying disorder leading to cerebral palsy is nonprogressive by definition, secondary
or associated conditions lead to functional loss and clinical progression over time. 11

Specific secondary or associated conditions and complications

1. Secondary complications associated with CP can include:11
o Joint/muscle contractures
o Foot deformity (equinus foot is the most common musculoskeletal deformity in CP)
o Hip dysplasia (chronic hip subluxation and progressive dislocation)
o Neuromuscular scoliosis
o Osteopenia
o Visual impairment
o Dysphagia
o Speech impairment
o Failure to thrive
o Impaired airway clearance
o Chronic pain
o GI problems: GERD, constipation
2. Co-Occuring developmental disabilities
o Seizure disorder (more common in tetraplegic and hemiparetic CP)
o Autism spectrum disorders (higher in hypotonic CP)
o Cognitive impairment (risk increases with greater degree of neuromuscular
impairment)

2. ESSENTIALS OF ASSESSMENT
History11
1. Important historical information includes understanding
1. Prenatal risk factors (infections, drug/toxin exposures, thyroid disease, maternal body
mass index)
2. Family history of similar conditions
3. Perinatal history (as indicated by APGAR scores, birth weight and history of
asphyxia)
4. Comprehensive developmental history including ages at which developmental
milestones were met or if any were lost
2. Loss of achieved milestones suggests a neurodegenerative condition,

Physical examination11
1. Key neurologic findings may include:
1. Elevated tone (may be hypotonic for the first 6 months of life);
2. Retention of primitive reflexes;
3. Abnormal postural control;
4. Abnormal motor control, including:
1. Co-contraction of antagonist and agonist muscles
 2. Synergy patterning
1. Flexion synergy upper extremitie
2. Extension synergy lower extremities
3. Extrampyramidal findings
1. dystonia
2. athetosis
2. Key musculoskeletal findings in the examination may include
1. Contractures
2. Hip subluxation/postural asymmetries of the pelvis
3. Scoliosis
4. Foot changes: equinus foot, varus or valgust foot or foot drop
5. Abnormal gait pattern
6. Toe walking: can be physiologic up to 2 years
7. Jumpers gait: Equinus foot, genu flexum and coxa flecta
8. Crouched gait: Excessive dorsiflexion of the ankle and genu flexum with coxa flecta
9. Hemiparetic gait

Functional assessment
Commonly used assessment tools include

1. Gross Motor Functional Classification System (age-based classification of mobility functions)

2. Gross Motor Function Measure (evaluates 5 dimensions of function, including lying/rolling,
sitting, crawling/kneeling, standing, walking, running, jumping)
3. Pediatric Evaluation of Disability Inventory (assesses realms of activity and participation)

Laboratory studies12
Metabolic and genetic studies should be considered if neuroimaging does not demonstrate a specific
structural abnormality, or if additional atypical history and clinical findings are noted.

Testing for coagulopathies should be considered if evidence of cerebral infarction is seen on

neuroimaging.

Imaging12
Neuroimaging is beneficial in the evaluation of CP in order to establish a 12diagnosis and prognosis,
although 13% have normal scans.

When available, MRI of the brain is preferred to CT scanning because of its higher yield (89% vs
77%). The yield on imagings depends on the type of CP and MRI is more likely to show abnormalities
associated with premature CP such as PVL.12

Supplemental assessment tools

Supplemental testing is patient specific and based on associated secondary conditions and
complications. Considerations include

1. Electroencephalography
2. Oropharyngeal motility studies (Oromotor impairment and subsequent dysphagia are most
common intetraplegia.)
3. Hearing screening (Sensorineural hearing loss is associated with CP secondary to TORCH
infections, meningitis.)
4. Vision screening (40%-100% prevalence of visual dysfunction)
5. Nutritional screening (risk of malnutrition secondary to oromotorimpairment, communication
impairment and poor socialsupport)
 6. Bone density studies (Risk of osteopenia increases with severity of CP and nonambulatory
status.)
1. DEXA studies are recommended following first fracture.

Early predictions of outcomes

1. Possible predictors of eventual ambulation include persistence of primitive reflexes, type of
cerebral palsy and gross motor skills.
2. Achieving independent sitting by age 2 years is directly related to achieving ambulation.
3. A measure of functional strength and dynamic postural control in a sit-to-stand activity was
significant predictor of taking ≥3 steps independently at age 2. 13
4. Nearly 100% of children with spastic hemiparesis, and 85% with spastic diplegia, will
ambulate.
5. Adverse factors affecting survival include severe/profound mental retardation, epilepsy and
type of CP.

Social role and social support system14

1. Federal law mandates the provision of educational support for children with special needs.
The Individuals with Disabilities Education Act of 1997 (IDEA) ensures meaningful
educational opportunities for individuals with disabilities from birth to 21 years.
2. IDEA includes early intervention services (Part C: birth to 3 years), special education services
(Part B: 3y to 21y), and Individualized Education Program (IEP)
3. The most recent final rule was published in the Federal Register on November 21 2016 and
since initial publication from 2008, most recent version made a number of significant changes
including clarifying and broadening the definition of “disability”. Also it further clarified
public accommodation’s obligations to provide auxiallry aids and services. 14

3. REHABILITATION MANAGEMENT AND

TREATMENTS
Available or current treatment guidelines
Evidence-based treatment guidelines are limited in cerebral palsy. Applicable guidelines (listed in the
National Guidelines Clearinghouse website http://www.guideline.gov) include:

1. Botulinum neurotoxin for the treatment of spasticity15

2. Evidence-based care guidelines for pediatric constraint-induced movement therapy 16
3. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the
Quality Standards Subcommittee of the American Academy of Neurology and the Practice
Committee of the Child Neurology Society15
4. Bone health in children and adolescents with chronic diseases that may affect the skeleton: the
2013 ISCD Pediatric Official Positions 17
5. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with
cerebral palsy (an evidence-based review). Report of the Quality Standards Subcommittee of
the American Academy of Neurology and the Practice Committee of the Child Neurology
Society.18
6. AACPDM Database of Evidence Reports with subjects including: splinting, aerobic exercise,
casting, conductive education, intrathecal baclofen, NDT, gastrostomy feeding, and hip
subluxation. 19

At different disease stages11

1. During infancy and early childhood, the focus of management primarily is on motor and
cognitive development. Considerations during this time must include spasticity management
 (oral medications, botulinum toxin injections), contracture prevention (passive range of
motion, splinting, both static and dynamic), feeding and nutrition, motor education, adaptive
equipment (orthotics, postural control devices), and communication.
2. As the child grows and develops many of these considerations remain, but new factors may
emerge that affect management strategies. Options for spasticity management expand
(baclofen pump, selective dorsal rhizotomy, muscle-tendon lengthening). Mobility needs
increase and are addressed through adaptive mobility equipment (walkers, crutches,
wheelchairs) and orthoses. The initiation of routine skeletal surveillance is recommended in
early childhood and may include annual hip films (anterior-posterior and frog views) and
scoliosis series radiographs.
3. As the child enters school age, school entry programming should begin and includes formal
cognitive testing and IEP development.
4. Considerations for the adolescent and young adult must include social adaptation, including
disabled sports programs, vocational rehabilitation assessment, and impaired-drivers
evaluation.
5. Planning the transition to adulthood is also critical. It includes developing goals for education,
independent living, medical care and legal guardianship.

Coordination of care
1. The longitudinal management of the individual with CP should be carried out as a
multidisciplinary, integrated, or coordinated effort.
2. Specialists in pediatric orthopedic surgery, neurosurgery, physiatry, neurology, nutrition,
physical therapy, occupational therapy, speech therapy, social work and nursing all play
critical roles in addressing the myriad needs (physical, medical, social) of this population.
3. Providing services to adults is challenging due to the lack of specialists trained and interested
in adults with CP.

Patient & family education

1. Patient and family education are key to the successful management of the individual with CP.
2. Important topics to address include social participation, educational resources, legal resources,
school transitions, family centered care concepts, advocacy for the child, anticipatory
guidance and transition to adulthood (vocational, medical, social, legal).

Emerging/unique Interventions
1. Impairment-based measures are commonly used to clearly define an individual level of
physical impairment, and are not patient specific. Frequently used tools include:
1. Modified Ashworth Scale (assesses degree of spasticity across a joint)
2. Tardieu Scale (assesses degree of spasticity across a joint)
3. Berg Balance Test (measures balance in individuals with mild to moderate motor
impairment)
4. Range of motion/goniometry (angular measure of passive and active range of
motion)
5. Selective voluntary Motor Control Assessment of the Lower Extremities (SCALES)
(assesses isolated active motion across a joint)
6. Hypertonia Assessment Tools (to differentiate spasticity, dystonia, rigidity and mixed
tone)
2. Tools for measuring functional outcomes include:
1. Gross Motor Function Measure
2. Gross Motor Function Classification System
3. Manual Abilities Classification System
4. Jebsen-Taylor Hand Function Test
5. Assisting Hand Assessment
6. Melbourne Assessment of Unilateral Upper Limb Function
7. Functional Independence Measure for Children
8. Pediatric Evaluation of Disability Inventory
 9. Pediatric Outcomes Data Collection Instrument

4. CUTTING EDGE/EMERGING AND UNIQUE

CONCEPTS AND PRACTICE
Cutting edge concepts and practice