Elsevier

The Lancet

Volume 383, Issue 9924, 5–11 April 2014, Pages 1240-1249
The Lancet

Seminar
Cerebral palsy

https://doi.org/10.1016/S0140-6736(13)61835-8Get rights and content

Summary

The syndrome of cerebral palsy encompasses a large group of childhood movement and posture disorders. Severity, patterns of motor involvement, and associated impairments such as those of communication, intellectual ability, and epilepsy vary widely. Overall prevalence has remained stable in the past 40 years at 2–3·5 cases per 1000 livebirths, despite changes in antenatal and perinatal care. The few studies available from developing countries suggest prevalence of comparable magnitude. Cerebral palsy is a lifelong disorder; approaches to intervention, whether at an individual or environmental level, should recognise that quality of life and social participation throughout life are what individuals with cerebral palsy seek, not improved physical function for its own sake. In the past few years, the cerebral palsy community has learned that the evidence of benefit for the numerous drugs, surgery, and therapies used over previous decades is weak. Improved understanding of the role of multiple gestation in pathogenesis, of gene environment interaction, and how to influence brain plasticity could yield significant advances in treatment of the disorder. Reduction in the prevalence of post-neonatal cerebral palsy, especially in developing countries, should be possible through improved nutrition, infection control, and accident prevention.

Introduction

In cerebral palsy's milder forms, individuals present with mild spasticity and contracture in one arm and leg on one side of the body, which interferes with fluid movement and fine manual dexterity. The individual might have some sensory inattention to that side of the body and to that visual field, and might have focal epilepsy. At the other end of the spectrum, an individual can present with involvement of the four limbs, with a mixed picture of spasticity and dyskinesia. The individual can have substantial contractures and scoliosis, and therefore require a wheelchair for mobility. They might also have associated severe learning difficulties, cortical visual impairment, and be prone to chest infections.

Cerebral palsy is a syndrome of motor impairment that results from a lesion occurring in the developing brain; the disorder varies in the timing of the lesion, the clinical presentation, and the site and severity of the impairments. The earliest description of the disorder is attributed to the orthopaedic surgeon William Little in 1862.1 Several attempts to define and classify the syndrome have been made. Recently, the International Executive Committee for the Definition of Cerebral Palsy, proposed the following definition: “Cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication and behaviour, by epilepsy, and by secondary musculoskeletal problems”. This definition is supplemented by a full explanation of the terms used in the definition.2

The complexity of the syndrome is clear from its various classifications; cerebral palsy can be defined according to the anatomical site of the brain lesion (cerebral cortex, pyramidal tract, extrapyramidal system, or cerebellum); clinical symptoms and signs (spasticity, dyskinesia [dystonic and choreo-athetotic forms], or ataxia); topographical involvement of extremities (diplegia, quadriplegia, or hemiplegia); timing of presumed insult (prepartum, intrapartum, or postneonatal); and classification of degree of muscle tone (isotonic, hypotonic, or hypertonic).3

Standard classifications are essential for research and transfer of knowledge. The 9th and 10th International Classifications of Disease include many categories of cerebral palsy and substantial inconsistency exists in how clinicians interpret these guidelines.4 A straightforward classification is needed that can be applied reliably by clinicians and used in registers. Such a classification (with categories of unilateral spastic, bilateral spastic, dyskinetic, and ataxic) and an associated decision tree was developed by the Surveillance of Cerebral Palsy in Europe (European network SCPE) and is now widely adopted.5

Section snippets

Epidemiology

Development of registers for cerebral palsy, with an emphasis on a shared definition of the syndrome and efforts to ensure complete identification of cases, has shown a cerebral palsy prevalence of 2·0–3·5 per 1000 livebirths.6, 7, 8, 9 Prevalence in developing countries seems to be similar, but data sources are not well established.10, 11 Cerebral palsy prevalence is inversely associated with gestational age and birthweight, with a prevalence ranging from 90 cases per 1000 neonatal survivors

Pathogenesis

Although many mechanisms have been proposed to explain the cause, nature, and timing of the definitive cerebral insult, adverse factors might have been present for some time during pregnancy. For every gestation and for each type of cerebral palsy, an optimum birthweight exists; the high rates of cerebral palsy observed in preterm births occur when gestational birthweight deviates from this optimum (figure 2).27 This optimum birthweight effect is especially pronounced when fetal assessment of

MRI

About 85% of children with cerebral palsy have an abnormal MRI scan. An MRI scan can provide an estimate of the timing of the lesion and assist in determination of whether the lesion is responsible for the motor impairment or is an incidental finding.52 An abnormal scan finding is not a prerequisite for diagnosis of cerebral palsy but scans are recommended to assist clinical management.53 Findings could provide families with a more complete explanation of the cause of their child's cerebral

Life expectancy

To estimate life expectancy, a register of all cases is needed with dates of birth and regular updates of deaths to allow actuarial analysis. The severities of mental, manual, ambulatory, and visual impairments are significant factors in survival. If all impairment domains are not severe, survival is only marginally less than that of individuals without cerebral palsy.54, 55, 56 If severe impairments are present, then life expectancy is reduced approximately in proportion to the number and

Quality of life

In the past decade, studies of individuals with cerebral palsy have been done within the framework of the International Classification of Functioning, Disability and Health (ICF).61 The ICF recognises three constructs: body structure and function, activity limitation, and participation. Each construct can influence the others, there is not a causative progression from an impairment of structure to participation. Furthermore, their interactions are influenced by the context in which an

Improving outcomes

As the ICF and life-course frameworks predict, a divergence exists between trying to make the body of the individual to function more normally and accepting the person as they are and concentrating on environmental adjustments. Many physical therapies are available, but little evidence exists on which to choose. Therapy combined with medical and surgical interventions offers benefit, at least in the short term. Seeking normalisation of physical impairment in childhood only achieves gains in the

Overview

Two factors are key in management of individuals with cerebral palsy. First, all interventions must be planned, done, and validated by a multidisciplinary service with the choices of the child and family at the core of decision making. Second, difficulties encountered are not restricted to an individual's motor disorder but also to the variety of comorbidities.

The social model of disability,100 in which problems in participation are attributed to failure of society to accept and adapt to the

Conclusions

In the next decade, the brain lesions described by the umbrella term cerebral palsy might be classified by their causal factors coupled with a full description of impairments to body structure and functions they produce. Improved understanding of brain plasticity will probably lead to new treatments or at least better application of existing ones. Management will increasingly focus on promotion of participation and quality of life, with an expanding range of new technologies directed both to

Search strategy and selection criteria

References included in this Seminar were identified by the authors based on their respective areas of expertise and supplemented by unsystematic database searches.

References (148)

  • C Nieuwenhuijsen et al.

    Experienced problems of young adults with cerebral palsy: targets for rehabilitation care

    Arch Phys Med Rehabil

    (2009)
  • KL Hadden et al.

    Pain in children with cerebral palsy: common triggers and expressive behaviors

    Pain

    (2002)
  • SI Michelsen et al.

    Frequency of participation of 8–12-year-old children with cerebral palsy: a multi-centre cross-sectional European study

    Eur J Paediatr Neurol

    (2009)
  • J Vargus-Adams

    Health-related quality of life in childhood cerebral palsy

    Arch Phys Med Rehabil

    (2005)
  • HO Dickinson et al.

    Self-reported quality of life of 8–12-year-old children with cerebral palsy: a cross-sectional European study

    Lancet

    (2007)
  • KF Bjornson et al.

    Self-reported health status and quality of life in youth with cerebral palsy and typically developing youth

    Arch Phys Med Rehabil

    (2008)
  • W Little

    On the influence of abnormal parturition, difficult labours and asphyxia neonatorum on the mental and physical condition of the child, especially in relation to deformities

  • P Rosenbaum et al.

    A report: the definition and classification of cerebral palsy April 2006

    Dev Med Child Neurol Suppl

    (2007)
  • TD Sanger et al.

    Classification and definition of disorders causing hypertonia in childhood

    Pediatrics

    (2003)
  • AF Colver et al.

    The term diplegia should be abandoned

    Arch Dis Child

    (2003)
  • Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers

    Dev Med Child Neurol

    (2000)
  • M Yeargin-Allsopp 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)
  • FJ Stanley et al.

    Trends in perinatal mortality and cerebral palsy in Western Australia, 1967 to 1985

    BMJ

    (1992)
  • B Hagberg et al.

    Changing panorama of cerebral palsy in Sweden. VIII. Prevalence and origin in the birth year period 1991–94

    Acta Paediatr

    (2001)
  • PO Pharoah et al.

    Epidemiology of cerebral palsy in England and Scotland, 1984–9

    Arch Dis Child Fetal Neonatal Ed

    (1998)
  • M Gladstone

    A review of the incidence and prevalence, types and aetiology of childhood cerebral palsy in resource-poor settings

    Ann Trop Paediatr

    (2010)
  • SH Ibrahim et al.

    Prevalence of early childhood disability in a rural district of Sind, Pakistan

    Dev Med Child Neurol

    (2013)
  • N Paneth et al.

    The frequency of cerebral palsy: a review of population studies in industrialised nations since 1950

    Clin Dev Med

    (1984)
  • F Stanley et al.

    Birthweight, gestational age and the cerebral palsies

    Clin Dev Med

    (1984)
  • E Sellier et al.

    Trends in prevalence of cerebral palsy in children born with a birthweight of 2500 g or over in Europe from 1980 to 1998

    Eur J Epidemiol

    (2010)
  • G Maudsley et al.

    Cause of death in cerebral palsy: a descriptive study

    Arch Dis Child

    (1999)
  • Report of the Australian cerebral palsy register, birth years 1993–2006

    (2013)
  • S Freud

    Infantile cerebralähmung, 1897 [Infantile cerebral paralysis]

    (1968)
  • Oxford register of early childhood impairments

    (2002)
  • PO Pharoah et al.

    Cerebral palsy and multiple births

    Arch Dis Child Fetal Neonatal Ed

    (1996)
  • A Burguet et al.

    Some risk factors for cerebral palsy in very premature infants: importance of premature rupture of membranes and monochorionic twin placentation

    Biol Neonate

    (1999)
  • JK Grether et al.

    Twinning and cerebral palsy: experience in four northern California counties, births 1983 through 1985

    Pediatrics

    (1993)
  • B Petterson et al.

    Twins, triplets, and cerebral palsy in births in Western Australia in the 1980s

    BMJ

    (1993)
  • PO Pharoah

    Cerebral palsy in the surviving twin associated with infant death of the co-twin

    Arch Dis Child Fet Neonatal Ed

    (2001)
  • S McIntyre et al.

    A systematic review of risk factors for cerebral palsy in children born at term in developed countries

    Dev Med Child Neurol

    (2013)
  • E Blair et al.

    Issues in the classification and epidemiology of cerebral palsy

    Ment Retard Dev Disabil Res Rev

    (1997)
  • A MacLennan

    A template for defining a causal relation between acute intrapartum events and cerebral palsy: international consensus statement

    BMJ

    (1999)
  • HE Hoyme et al.

    Vascular etiology of disruptive structural defects in monozygotic twins

    Pediatrics

    (1981)
  • L Fusi et al.

    Acute twin-twin transfusion: a possible mechanism for brain-damaged survivors after intrauterine death of a monochorionic twin

    Obstet Gynecol

    (1991)
  • H Landy et al.

    The vanishing twin

    (1995)
  • HJ Landy et al.

    The vanishing twin: a review

    Hum Reprod Update

    (1998)
  • PO Pharoah et al.

    A hypothesis for the aetiology of spastic cerebral palsy—the vanishing twin

    Dev Med Child Neurol

    (1997)
  • JG Shatrov et al.

    Chorioamnionitis and cerebral palsy: a meta-analysis

    Obstet Gynecol

    (2010)
  • PO Pharoah

    Prevalence and pathogenesis of congenital anomalies in cerebral palsy

    Arch Dis Child Fetal Neonatal Ed

    (2007)
  • Cited by (419)

    View all citing articles on Scopus
    View full text