Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Epidemiology and etiology of cerebral palsy

INTRODUCTION

Cerebral palsy (CP) consists of a heterogeneous group of nonprogressive clinical syndromes that are characterized by motor and postural dysfunction. These conditions, which range in severity, are due to abnormalities of the developing brain resulting from a variety of causes. Although the disorder itself is not progressive, the appearance of neuropathologic lesions and their clinical expression may change over time as the brain matures.

The epidemiology and etiology of CP are reviewed here. The clinical manifestations, diagnosis, management, and prognosis are discussed separately. (See "Clinical features of cerebral palsy" and "Diagnosis and classification of cerebral palsy" and "Management and prognosis of cerebral palsy".)

EPIDEMIOLOGY

The precise prevalence of cerebral palsy (CP) in the United States is uncertain because consistent information is lacking on follow-up of an entire population, especially for term and late preterm infants, which comprise the majority of births. A population-based surveillance study using data from three regions in the United States estimated a prevalence of 3.6 cases per 1000 children at eight years of age, but the study did not distinguish between children with and without a history of prematurity [1]. No data are available on CP prevalence in North America for term infants born since the mid-1980s [2]. In one report that used standard definitions and included 6000 children with CP from 13 geographically defined populations in Europe born from 1980 to 1990, the overall rate was 2.08/1000 live births [3].

The rate of CP is much higher in preterm than term infants, and increases with decreasing birth weight and gestational age. This is illustrated by the following studies:

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 [3].

               

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Mar 2014. | This topic last updated: Nov 11, 2013.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Yeargin-Allsopp M, Van Naarden Braun K, Doernberg NS, et al. Prevalence of cerebral palsy in 8-year-old children in three areas of the United States in 2002: a multisite collaboration. Pediatrics 2008; 121:547.
  2. Nelson KB. The epidemiology of cerebral palsy in term infants. Ment Retard Dev Disabil Res Rev 2002; 8:146.
  3. Prevalence and characteristics of children with cerebral palsy in Europe. Dev Med Child Neurol 2002; 44:633.
  4. Hagberg B, Hagberg G, Olow I, von Wendt L. The changing panorama of cerebral palsy in Sweden. VII. Prevalence and origin in the birth year period 1987-90. Acta Paediatr 1996; 85:954.
  5. Wood NS, Marlow N, Costeloe K, et al. Neurologic and developmental disability after extremely preterm birth. EPICure Study Group. N Engl J Med 2000; 343:378.
  6. Lorenz JM, Wooliever DE, Jetton JR, Paneth N. A quantitative review of mortality and developmental disability in extremely premature newborns. Arch Pediatr Adolesc Med 1998; 152:425.
  7. Moster D, Wilcox AJ, Vollset SE, et al. Cerebral palsy among term and postterm births. JAMA 2010; 304:976.
  8. Jarvis S, Glinianaia SV, Torrioli MG, et al. Cerebral palsy and intrauterine growth in single births: European collaborative study. Lancet 2003; 362:1106.
  9. Petersen MC, Palmer FB. Birthweight and risk for cerebral palsy. Lancet 2003; 362:1089.
  10. Pharoah PO, Platt MJ, Cooke T. The changing epidemiology of cerebral palsy. Arch Dis Child Fetal Neonatal Ed 1996; 75:F169.
  11. Platt MJ, Cans C, Johnson A, et al. Trends in cerebral palsy among infants of very low birthweight (<1500 g) or born prematurely (<32 weeks) in 16 European centres: a database study. Lancet 2007; 369:43.
  12. Robertson CM, Watt MJ, Yasui Y. Changes in the prevalence of cerebral palsy for children born very prematurely within a population-based program over 30 years. JAMA 2007; 297:2733.
  13. Nelson KB. Can we prevent cerebral palsy? N Engl J Med 2003; 349:1765.
  14. Strijbis EM, Oudman I, van Essen P, MacLennan AH. Cerebral palsy and the application of the international criteria for acute intrapartum hypoxia. Obstet Gynecol 2006; 107:1357.
  15. Olegård R, Sabel KG, Aronsson M, et al. Effects on the child of alcohol abuse during pregnancy. Retrospective and prospective studies. Acta Paediatr Scand Suppl 1979; 275:112.
  16. O'Leary CM, Watson L, D'Antoine H, et al. Heavy maternal alcohol consumption and cerebral palsy in the offspring. Dev Med Child Neurol 2012; 54:224.
  17. Abel EL. Cerebral palsy and alcohol consumption during pregnancy: is there a connection? Alcohol Alcohol 2010; 45:592.
  18. Streja E, Miller JE, Bech BH, et al. Congenital cerebral palsy and prenatal exposure to self-reported maternal infections, fever, or smoking. Am J Obstet Gynecol 2013; 209:332.e1.
  19. Perlman JM. Intrapartum hypoxic-ischemic cerebral injury and subsequent cerebral palsy: medicolegal issues. Pediatrics 1997; 99:851.
  20. Nelson KB. What proportion of cerebral palsy is related to birth asphyxia? J Pediatr 1988; 112:572.
  21. Hankins GD, Speer M. Defining the pathogenesis and pathophysiology of neonatal encephalopathy and cerebral palsy. Obstet Gynecol 2003; 102:628.
  22. Ellenberg JH, Nelson KB. The association of cerebral palsy with birth asphyxia: a definitional quagmire. Dev Med Child Neurol 2013; 55:210.
  23. Nelson KB, Ellenberg JH. Apgar scores as predictors of chronic neurologic disability. Pediatrics 1981; 68:36.
  24. Paneth N. Cerebral palsy in term infants--birth or before birth? J Pediatr 2001; 138:791.
  25. Nelson KB, Grether JK. Potentially asphyxiating conditions and spastic cerebral palsy in infants of normal birth weight. Am J Obstet Gynecol 1998; 179:507.
  26. Grether JK, Nelson KB. Maternal infection and cerebral palsy in infants of normal birth weight. JAMA 1997; 278:207.
  27. Nelson KB, Grether JK. Selection of neonates for neuroprotective therapies: one set of criteria applied to a population. Arch Pediatr Adolesc Med 1999; 153:393.
  28. American College of Obstetricians and Gynecologists and American Academy of Pediatrics. Neonatal encephalopathy and cerebral palsy: defining the pathogenesis and pathophysiology, ACOG, Washington, DC 2003.
  29. American Collge of Obstetricians and Gynecologists (ACOG). Neonatal encephalopathy and cerebral palsy: executive summary. Obstet Gynecol 2004; 103:780.
  30. Clancy RR, Sladky JT, Rorke LB. Hypoxic-ischemic spinal cord injury following perinatal asphyxia. Ann Neurol 1989; 25:185.
  31. Nelson KB, Ellenberg JH. Antecedents of cerebral palsy. Multivariate analysis of risk. N Engl J Med 1986; 315:81.
  32. Gaffney G, Sellers S, Flavell V, et al. Case-control study of intrapartum care, cerebral palsy, and perinatal death. BMJ 1994; 308:743.
  33. Croen LA, Grether JK, Curry CJ, Nelson KB. Congenital abnormalities among children with cerebral palsy: More evidence for prenatal antecedents. J Pediatr 2001; 138:804.
  34. Miller G. Minor congenital anomalies and ataxic cerebral palsy. Arch Dis Child 1989; 64:557.
  35. Pharoah PO. Prevalence and pathogenesis of congenital anomalies in cerebral palsy. Arch Dis Child Fetal Neonatal Ed 2007; 92:F489.
  36. Freeman JM, Nelson KB. Intrapartum asphyxia and cerebral palsy. Pediatrics 1988; 82:240.
  37. Brann AW. Factors during neonatal life that influence brain disorder. In: Prenatal and Perinatal Factors Associated with Brain Disorders (NIH Pub. No. 85-1149), Freeman JM (Ed), US Government Printing Office, Washington, DC 1998. p.263.
  38. Felix JF, Badawi N, Kurinczuk JJ, et al. Birth defects in children with newborn encephalopathy. Dev Med Child Neurol 2000; 42:803.
  39. Naeye RL, Peters EC, Bartholomew M, Landis JR. Origins of cerebral palsy. Am J Dis Child 1989; 143:1154.
  40. Andersen GL, Irgens LM, Skranes J, et al. Is breech presentation a risk factor for cerebral palsy? A Norwegian birth cohort study. Dev Med Child Neurol 2009; 51:860.
  41. Wood JW, Johnson KG, Omori Y. In utero exposure to the Hiroshima atomic bomb. An evaluation of head size and mental retardation: twenty years later. Pediatrics 1967; 39:385.
  42. Dekaban AS. Abnormalities in children exposed to x-radiation during various stages of gestation: tentative timetable of radiation injury to the human fetus. I. J Nucl Med 1968; 9:471.
  43. Brent RL, Beckman DA. Environmental teratogens. Bull N Y Acad Med 1990; 66:123.
  44. Rorke LB. A perspective: the role of disordered genetic control of neurogenesis in the pathogenesis of migration disorders. J Neuropathol Exp Neurol 1994; 53:105.
  45. Hilburger AC, Willis JK, Bouldin E, Henderson-Tilton A. Familial schizencephaly. Brain Dev 1993; 15:234.
  46. Haverkamp F, Zerres K, Ostertun B, et al. Familial schizencephaly: further delineation of a rare disorder. J Med Genet 1995; 32:242.
  47. Roessler E, Belloni E, Gaudenz K, et al. Mutations in the human Sonic Hedgehog gene cause holoprosencephaly. Nat Genet 1996; 14:357.
  48. Dobyns WB, Andermann E, Andermann F, et al. X-linked malformations of neuronal migration. Neurology 1996; 47:331.
  49. Barkovich AJ, Kuzniecky RI, Dobyns WB, et al. A classification scheme for malformations of cortical development. Neuropediatrics 1996; 27:59.
  50. Dodge NN, Dobyns WB. Agenesis of the corpus callosum and Dandy-Walker malformation associated with hemimegalencephaly in the sebaceous nevus syndrome. Am J Med Genet 1995; 56:147.
  51. Bundey S, Griffiths MI. Recurrence risks in families of children with symmetrical spasticity. Dev Med Child Neurol 1977; 19:179.
  52. Hemminki K, Sundquist K, Li X. Familial risks for main neurological diseases in siblings based on hospitalizations in Sweden. Twin Res Hum Genet 2006; 9:580.
  53. Kuroda MM, Weck ME, Sarwark JF, et al. Association of apolipoprotein E genotype and cerebral palsy in children. Pediatrics 2007; 119:306.
  54. Gibson CS, MacLennan AH, Hague WM, et al. Associations between inherited thrombophilias, gestational age, and cerebral palsy. Am J Obstet Gynecol 2005; 193:1437.
  55. O'Callaghan ME, Maclennan AH, Gibson CS, et al. Fetal and maternal candidate single nucleotide polymorphism associations with cerebral palsy: a case-control study. Pediatrics 2012; 129:e414.
  56. Gibson CS, MacLennan AH, Goldwater PN, et al. The association between inherited cytokine polymorphisms and cerebral palsy. Am J Obstet Gynecol 2006; 194:674.e1.
  57. Gibson CS, Maclennan AH, Dekker GA, et al. Candidate genes and cerebral palsy: a population-based study. Pediatrics 2008; 122:1079.
  58. Wells J, Rosenberg M, Hoffman G, et al. A decision-tree approach to cost comparison of newborn screening strategies for cystic fibrosis. Pediatrics 2012; 129:e339.
  59. Petterson B, Nelson KB, Watson L, Stanley F. Twins, triplets, and cerebral palsy in births in Western Australia in the 1980s. BMJ 1993; 307:1239.
  60. Pharoah PO, Cooke T. Cerebral palsy and multiple births. Arch Dis Child Fetal Neonatal Ed 1996; 75:F174.
  61. Larroche JC, Droullé P, Delezoide AL, et al. Brain damage in monozygous twins. Biol Neonate 1990; 57:261.
  62. Scheller JM, Nelson KB. Twinning and neurologic morbidity. Am J Dis Child 1992; 146:1110.
  63. Weig SG, Marshall PC, Abroms IF, Gauthier NS. Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins. Pediatr Neurol 1995; 13:279.
  64. Bonellie SR, Currie D, Chalmers J. Comparison of risk factors for cerebral palsy in twins and singletons. Dev Med Child Neurol 2005; 47:587.
  65. Bejar R, Vigliocco G, Gramajo H, et al. Antenatal origin of neurologic damage in newborn infants. II. Multiple gestations. Am J Obstet Gynecol 1990; 162:1230.
  66. Pharoah PO. Twins and cerebral palsy. Acta Paediatr Suppl 2001; 90:6.
  67. Pharoah PO. Cerebral palsy in the surviving twin associated with infant death of the co-twin. Arch Dis Child Fetal Neonatal Ed 2001; 84:F111.
  68. Hanigan WC, Powell FC, Miller TC, Wright RM. Symptomatic intracranial hemorrhage in full-term infants. Childs Nerv Syst 1995; 11:698.
  69. Gradnitzer E, Urlesberger B, Maurer U, et al. [Cerebral hemorrhage in term newborn infants--an analysis of 10 years (1989-1999)]. Wien Med Wochenschr 2002; 152:9.
  70. Roland EH, Flodmark O, Hill A. Thalamic hemorrhage with intraventricular hemorrhage in the full-term newborn. Pediatrics 1990; 85:737.
  71. Nelson KB, Ellenberg JH. Antecedents of cerebral palsy. I. Univariate analysis of risks. Am J Dis Child 1985; 139:1031.
  72. Wu YW, Escobar GJ, Grether JK, et al. Chorioamnionitis and cerebral palsy in term and near-term infants. JAMA 2003; 290:2677.
  73. Ahlin K, Himmelmann K, Hagberg G, et al. Cerebral palsy and perinatal infection in children born at term. Obstet Gynecol 2013; 122:41.
  74. Nelson KB, Dambrosia JM, Grether JK, Phillips TM. Neonatal cytokines and coagulation factors in children with cerebral palsy. Ann Neurol 1998; 44:665.
  75. O'Shea TM. Cerebral palsy in very preterm infants: new epidemiological insights. Ment Retard Dev Disabil Res Rev 2002; 8:135.
  76. Wu YW, Colford JM Jr. Chorioamnionitis as a risk factor for cerebral palsy: A meta-analysis. JAMA 2000; 284:1417.
  77. Yoon BH, Jun JK, Romero R, et al. Amniotic fluid inflammatory cytokines (interleukin-6, interleukin-1beta, and tumor necrosis factor-alpha), neonatal brain white matter lesions, and cerebral palsy. Am J Obstet Gynecol 1997; 177:19.
  78. Yoon BH, Romero R, Yang SH, et al. Interleukin-6 concentrations in umbilical cord plasma are elevated in neonates with white matter lesions associated with periventricular leukomalacia. Am J Obstet Gynecol 1996; 174:1433.
  79. Dykes FD, Dunbar B, Lazarra A, Ahmann PA. Posthemorrhagic hydrocephalus in high-risk preterm infants: natural history, management, and long-term outcome. J Pediatr 1989; 114:611.
  80. Perlman JM, Volpe JJ. Movement disorder of premature infants with severe bronchopulmonary dysplasia: a new syndrome. Pediatrics 1989; 84:215.
  81. Wical BS, Tomasi LG. A distinctive neurologic syndrome after induced profound hypothermia. Pediatr Neurol 1990; 6:202.
  82. Kupsky WJ, Drozd MA, Barlow CF. Selective injury of the globus pallidus in children with post-cardiac surgery choreic syndrome. Dev Med Child Neurol 1995; 37:135.
  83. Pharoah PO, Cooke T, Rosenbloom L. Acquired cerebral palsy. Arch Dis Child 1989; 64:1013.