Cerebral Palsy

Author(s): Mi Ran Shin, MD and Heakyung Kim, MD

Originally published:11/10/2011

Last updated:04/17/2017

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
    • Joint/muscle contractures
    • Foot deformity (equinus foot is the most common musculoskeletal deformity in CP)
    • Hip dysplasia (chronic hip subluxation and progressive dislocation)
    • Neuromuscular scoliosis
    • Osteopenia
    • Visual impairment
    • Dysphagia
    • Speech impairment
    • Failure to thrive
    • Impaired airway clearance
    • Chronic pain
    • GI problems: GERD, constipation
  2. Co-Occuring developmental disabilities
    • Seizure disorder (more common in tetraplegic and hemiparetic CP)
    • Autism spectrum disorders (higher in hypotonic CP)
    • 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

  1. Although the mainstays of treatment for CP continue to be physical therapy, occupational therapy and speech therapy, some newer interventions are emerging.
  2. Stem cell therapy for treatment of CP; to replace the damaged tissue is being investigated.20,21
  3. Protective magnesium sulfate to be given before anticipated early preterm birth to reduce risk of CP.22
  4. Genomic diagnosis for cerebral palsy is being investigated.10
  5. Studies showing that higher prepregnancy maternal BMI associated with increased risk of CP23

5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE

Gaps in the evidence-based knowledge

  1. Effectiveness and safety of stem cell therapy
  2. Prevalence of CP in Zika virus transmitted children
  3. A knowledge and translational research gap between basic and clinical research

REFERENCES

  1. Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society, Delgado MR, Hirtz D, et al. 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. Neurology. 2010;74(4):336-343.
  2. Reddihough DS, Collins KJ. The epidemiology and causes of cerebral palsy. Australian Journal of physiotherapy. 2003;49(1):7-12.
  3. Maenner MJ, Blumberg SJ, Kogan MD, Christensen D, Yeargin-Allsopp M, Schieve LA. Prevalence of cerebral palsy and intellectual disability among children identified in two U.S. national surveys, 2011-2013. Ann Epidemiol. 2016;26(3):222-226.
  4. Van Naarden Braun K, Doernberg N, Schieve L, Christensen D, Goodman A, Yeargin-Allsopp M. Birth prevalence of cerebral palsy: A population-based study. Pediatrics. 2016;137(1):10.1542/peds.2015-2872. Epub 2015 Dec 9.
  5. Scher MS, Belfar H, Martin J, Painter MJ. Destructive brain lesions of presumed fetal onset: Antepartum causes of cerebral palsy. Pediatrics. 1991;88(5):898-906.
  6. MacLennan A. A template for defining a causal relation between acute intrapartum events and cerebral palsy: International consensus statement. BMJ. 1999;319(7216):1054-1059.
  7. Colver A, Fairhurst C, Pharoah PO. Cerebral palsy. Lancet. 2014;383(9924):1240-1249.
  8. Executive summary: Neonatal encephalopathy and neurologic outcome, second edition. report of the american college of obstetricians and gynecologists’ task force on neonatal encephalopathy. Obstet Gynecol. 2014;123(4):896-901.
  9. Wu YW, Colford JM,Jr. Chorioamnionitis as a risk factor for cerebral palsy: A meta-analysis. JAMA. 2000;284(11):1417-1424.
  10. Lim WH. Cerebral palsy: Causes, pathways, and the role of genetic variants. Am J Obstet Gynecol. 2016;214(5):670-671.
  11. Liptak GS, Murphy NA, Council on Children With Disabilities. Providing a primary care medical home for children and youth with cerebral palsy. Pediatrics. 2011;128(5):e1321-9.
  12. Ashwal S, Russman BS, Blasco PA, et al. 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 society. Neurology. 2004;62(6):851-863.
  13. Begnoche DM, Chiarello LA, Palisano RJ, Gracely EJ, McCoy SW, Orlin MN. Predictors of independent walking in young children with cerebral palsy. Phys Ther. 2016;96(2):183-192.
  14. The americans with disabilities act of 1990 and revised ADA regulations implementing title II and title III. https://www.ada.gov/2010_regs.htm. Updated 2016. Accessed 1/15, 2017.
  15. Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society, Delgado MR, Hirtz D, et al. 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. Neurology. 2010;74(4):336-343.
  16. Pediatric modified Constraint Induced Movement Therapy (mCIMT/BIT) Team. Cincinnati children’s hospital medical center: Evidence-based clinical care guideline pediatric modified constraint induced movement therapy (mCIMT) plus bimanual training (BIT). guideline 34, pages 1-21, http://www.cincinnatichildrens.org/svc/alpha/h/health-policy/ev-based / Pediatric modified Constraint Induced Movement Therapy (mCIMT) plus Bimanual Training (BIT).htm,. Updated December , 2014. Accessed November/18, 2016.
  17. Bianchi ML, Leonard MB, Bechtold S, et al. Bone health in children and adolescents with chronic diseases that may affect the skeleton: The 2013 ISCD pediatric official positions. J Clin Densitom. 2014;17(2):281-294.
  18. Whelan MA, Delgado MR. 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. Neurology. 2010;75(7):669.
  19. AACPDM database of evidence reports. http://www.aacpdm.org/education/reviews-reports. Updated 2016. Accessed 1/15, 2017.
  20. Abi Chahine NH, Wehbe TW, Hilal RA, Zoghbi VV, Melki AE, Habib EB. Treatment of cerebral palsy with stem cells: A report of 17 cases. Int J Stem Cells. 2016;9(1):90-95.
  21. Kiasatdolatabadi A, Lotfibakhshaiesh N, Yazdankhah M, et al. The role of stem cells in the treatment of cerebral palsy: A review. Mol Neurobiol. 2016.
  22. Jelin AC, Salmeen K, Gano D, Burd I, Thiet MP. Perinatal neuroprotection update. F1000Res. 2016;5:10.12688/f1000research.8546.1. eCollection 2016.
  23. Forthun I, Wilcox AJ, Strandberg-Larsen K, et al. Maternal prepregnancy BMI and risk of cerebral palsy in offspring. Pediatrics. 2016.
  24. Lungu C, Hirtz D, Damiano D, Gross P, Mink JW. Report of a workshop on research gaps in the treatment of cerebral palsy. Neurology. 2016.

Suggested Readings and Resources

Alexander MA, Matthews DJ. Pediatric Rehabilitation: Principles and Practice. 4th ed. New York, NY: Demos Medical; 2010.

Ment LR, Bada HS, Barnes P, et al. Practice parameter: neuroimaging of the neonate. Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2002;58(12):1726-1738.

Ashwal S, Russman BS, Blasco PA, et al. 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 Society. Neurology. 2004; 62(6):851-863.

Strauss D, Shavelle R, Reynolds R, Rosenbloom L, Day S. Survival in cerebral palsy in the last 20 years: signs of improvement? Dev Med Child Neurol. 2007;49:86-92.

Sala DA, Grant AD. Prognosis for ambulation in cerebral palsy. Dev Med Child Neurol. 1995;37(11):1020-1026.

Tosi L, Maher N, Winslow Moore D, Goldstein M, Aisen ML. Adults with cerebral palsy: a workshop to define the challenges of treating and preventing secondary musculoskeletal and neuromuscular complications in this rapidly growing population. Dev Med Child Neurol. 2009;51(suppl 4):2-11.

Yeargin-Allsopp M, Van Naarden Braun K, Doernberg NS, Benedict RE, Kirby RS, Durkin MS. Prevalence of cerebral palsy in 8-year-old children in three areas of the United States in 2002: a multisite collaboration. Pediatrics. 2008;121(3):547-554.

Original Version of the Topic

Robert Rinaldi, MD. Cerebral Palsy. 12/27/2012.

Author Disclosures

Mi Ran Shin, MD
Nothing to Disclose

Heakyung Kim, MD
Nothing to Disclose

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