Seminar

Cerebral palsy
Allan Colver, Charles Fairhurst, Peter O D Pharoah
Lancet 2014; 383: 124049
Published Online
November 20, 2013
http://dx.doi.org/10.1016/
S0140-6736(13)61835-8
Institute of Health and Society,
Newcastle University, Royal
Victoria Inrmary, Newcastle
upon Tyne, UK
(Prof A Colver FRCPCH);
Department of Paediatric
Neurosciences, Evelina
Childrens Hospital, Guys and
Saint Thomas NHS Foundation
Trust, London, UK
(C Fairhurst FRCPCH); and
University of Liverpool,
Liverpool, UK
(Prof P O D Pharoah FRCP)
Correspondence to:
Prof Peter O D Pharoah,
University of Liverpool,
Liverpool L69 3GB, UK
[email protected]

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 235 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 benet 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 inuence brain plasticity could yield signicant 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 palsys milder forms, individuals present
with mild spasticity and contracture in one arm and leg
on one side of the body, which interferes with uid
movement and ne manual dexterity. The individual
might have some sensory inattention to that side of the
body and to that visual eld, 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 diculties, 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 dene and classify the syndrome have been
made. Recently, the International Executive Committee
for the Denition of Cerebral Palsy, proposed the
following denition: 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 denition is supplemented by a full
explanation of the terms used in the denition.2

Search strategy and selection criteria

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

1240

The complexity of the syndrome is clear from its various

classications; cerebral palsy can be dened 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 classication of degree
of muscle tone (isotonic, hypotonic, or hypertonic).3
Standard classications are essential for research and
transfer of knowledge. The 9th and 10th International
Classications of Disease include many categories of
cerebral palsy and substantial inconsistency exists in
how clinicians interpret these guidelines.4 A
straightforward classication is needed that can be
applied reliably by clinicians and used in registers. Such
a classication (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

Epidemiology
Development of registers for cerebral palsy, with an
emphasis on a shared denition of the syndrome and
eorts to ensure complete identication of cases, has
shown a cerebral palsy prevalence of 2035 per 1000 livebirths.69 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
weighing less than 1000 g to 15 cases per 1000 for those
born weighing 2500 g or more.1214 The upper age limit
used for denition of postneonatal cerebral palsy is
arbitrary, but in most studies it is considered to be about
5 years. About 10% of all cases of cerebral palsy are
classied as postneonatal,15 which are largely attributable
to CNS infections such as meningoencephalitis and head
www.thelancet.com Vol 383 April 5, 2014

Seminar

Pathogenesis
Although many mechanisms have been proposed to
explain the cause, nature, and timing of the denitive
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 (gure 2).27 This optimum birthweight
eect is especially pronounced when fetal assessment of
weights in the womb are used to generate weight
standards rather than actual weights of delivered babies.
Most cases of cerebral palsy result from an interference
in brain development in utero and MRI scanning has
helped understanding of these processes. In general,
insults during the rst trimester are associated with
www.thelancet.com Vol 383 April 5, 2014

cerebral maldevelopments such as schizencephaly; in the

second trimester, with periventricular white matter
damage; and in the third trimester, with cortical and deep
grey matter damage.28 Neonatal asphyxia was thought to
be a key cause of brain damage in preterm or term
babies. However, the evidence for asphyxia was applied
loosely, and often referred only to the need for
administration of oxygen after birth. Asphyxia is now
thought to account for 1020% of cerebral palsy cases29
and attribution of causation now requires evidence of
encephalopathy.30
Monochorionicity can have an important role in the
pathogenesis of cerebral palsy because the vascular
anastomoses in the placenta serve both fetuses and
transfusion potentially could occur between fetuses.
Embolic theory proposes that the transference of
thromboplastin or thromboemboli from the dead fetus to
the cotwin leads to cerebral damage;31,32 ischaemic theory
suggests that some exsanguination occurs from the
surviving fetus into the low resistance dead fetus.33
Although these theories do not seem to help to explain
cerebral palsy in singletons or dichorionic multiple
pregnancies, obstetric ultrasound has shown that one or
more embryos from a multiple conception can be lost
early in pregnancy as a vanishing twin or triplet;34,35
therefore, this twin could be the cause of cerebral palsy in
some singleton births.36
In developed countries, causes of cerebral palsy are
routinely prevented and can go unnoticed. For example,
marriage between close family members is not common, mothers receive prompt treatment for rhesus
isoimmunisation (in which severe jaundice is an
established cause of dyskinetic cerebral palsy), and many
immunisations are directed at prevention of infant
infections such as meningitis. In some developing
countries, iodine deciency causes a specic type of
cerebral palsy.37 A controlled trial of iodinated oil
100
90
80
Prevelence per 1000 livebirths

injuries (accidental and non-accidental). Prevalence of

cerebral palsy in infants of normal birthweight (2500 g)
seems not to have changed over time,14 but a decreasing
prevalence in low birthweight infants has been noted in
Europe (gure 1),16 and a decrease, which then levelled
out, was conrmed in an Australian cohort.17
As noted more than a century ago by Sigmund
Freud,18 multiple pregnancy is also a risk factor for
cerebral palsy. However, comparisons of the risk of
cerebral palsy in singleton and multiple births are
confounded by the eect of birthweight and gestational
age. This problem can be split into two parts: rst, the
inverse association of the prevalence of cerebral palsy
with birthweight and, second, the increasing proportion
of multiple births of decreasing birthweights. Compared with singletons, the relative risk of cerebral palsy
in twins is 56 and in triplets is 126.19 When both twins
are livebirths, there is a one in 56 probability that one
infant has cerebral palsy and a one in 430 probability
that both have cerebral palsy.20,21 Most pregnancies are
monochorionic (where twins share the same amniotic
sac), which is a known risk factor for cerebral palsy22
even in very preterm infants. Population studies
suggest a 50100 times increase in the prevalence of
cerebral palsy in a live cotwin of a stillbirth2023 compared with singleton pregnancies. When both twins are
livebirths, infant death of one twin is associated with a
signicant increase in risk of cerebral palsy in the
survivor. The cerebral palsy risk in a same-sex survivor
with infant death of the cotwin is 167 per 1000 compared
with 21 per 1000 in an unlike pair.24 A risk factor is not
necessarily causal and other recently identied risk
factors have yielded little in the way of preventive
strategies.25 A systematic review26 done in 2013 reported
ten risk factors as signicantly associated with cerebral
palsy: placental abnormalities, major and minor
birth defects, low birthweight, meconium aspiration,
emergency caesarean section, birth asphyxia, neonatal
seizures, respiratory distress syndrome, hypoglycaemia,
and neonatal infections.

70
60
50
40
30
20
10
0
1981 1982 1983 1984 1985 1986 1987 1988 1989 1990 1991 1992 1993 1994 1995
Midpoint birth year

Figure 1: Prevalence of cerebral palsy in infants with birthweights of 10001499 g from nine European
countries in birth years 198096 (3 year moving average)
Countries are Denmark, France, Germany, Ireland, Italy, The Netherlands, Norway, Sweden, and the UK. Reproduced
from Platt and colleagues.16

1241

Seminar

Conventional standard

attention decit disorder, intellectual impairment,

and epilepsy.51

Fetal standard

Number per 1000 livebirths

1000

MRI
100

10

01
3

<28 weeks
2831 weeks

3
3
Z score
3236 weeks
3738 weeks

3941 weeks
>42 weeks

Figure 2: Prevalence of cerebral palsy by Z score of weight for gestation

Reproduced from Jarvis and colleagues.27

established that the disease could be prevented, provided

supplementation was given before conception.38 In
another study, withdrawal of a rich source of dietary
iodine precipitated an epidemic of cerebral palsy.39
Maternal infections such as rubella and cytomegalovirus
can result in cerebral palsy. Several studies have reported
chorioamnionitis to be a risk factor for cerebral palsy,
especially in preterm babies.40 To date, however, enhanced
identication and treatment of such infections has not
been eective. A signicant increase in the prevalence of
non-cerebral congenital anomalies coexistent with
cerebral palsy has been reported.4143 Undoubtedly, some
cases of cerebral palsy that have a fetal origin associated
with non-cerebral congenital anomalies4246 have a genetic
or teratogenic cause, but transfusion problems between
fetuses might also provide a unied pathogenic mechanism in some cases.
Articial reproductive therapies could play a part in
the genesis of cerebral palsy, although these associations
are dicult to study because of strict condentiality
regulations in most countries. When studies linking
anonymised national datasets have been possible, an
increased risk was reported, but was largely attributable
to increased risk of multiple pregnancies and preterm
births.47,48
Genetic factors are increasingly implicated in our
understanding of cerebral palsy. Several single gene
Mendelian disorders cause cerebral palsy, including
isolated bilateral polymicrogyria and spastic types in
closely related families. Single nucleotide polymorphisms, especially clotting abnormalities such as factor V
Leiden, are reported to not be predictive of cerebral
palsy.49,50 However, complete genome and exome
sequencing will probably identify genes and combinations of genes that are predictive of cerebral palsy, and
many of these genes could be shared with predictors of
other neurodevelopmental disorders such as autism,
1242

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 nding.52 An
abnormal scan nding 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
childs cerebral palsy or could show lesions such as
bilateral polymicrogyria with implications for genetic
counselling. Finally, a normal scan can suggest the need
for a more detailed investigation of some genetic conditions such as hereditary spastic paraplegias, doparesponsive dystonias, and metabolic disorders that can
mimic cerebral palsy.

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 signicant factors in survival. If all impairment domains are
not severe, survival is only marginally less than that of
individuals without cerebral palsy.5456 If severe
impairments are present, then life expectancy is reduced
approximately in proportion to the number and severity
of associated impairments.54 Of individuals with cerebral
palsy in the UK who were alive at age 2 years with four
severe impairments (intelligence quotient <50, nonambulant, partially sighted, and poor manual function),
72% lived to 10 years, 44% to 20 years, 34% to 30 years,
and 27% to 40 years (JL Hutton, University of Warwick,
Coventry, UK, personal communication). Life expectancy
does not seem to be improving, although this observation
might be because more children with severe impairments
(who used to die before diagnosis of cerebral palsy could
be made) are now more likely to live at least into their
early years. However, quality of care can also be relevant.
A UK report57 noted that in adults with severe intellectual
impairment, features of severe physical illness might not
have been noticed; which is a particular problem in those
with additional severe cerebral palsy. The most recent
study to report cause of death in individuals with cerebral
palsy in Australia58 noted that, as in previous studies,59,60
interpretation of information in death certicates is
dicult, especially when cerebral palsy is stated as the
only cause of death. The most common cause in
individuals with severe cerebral palsy was cerebral palsy
in 50% of cases. Next was pneumonia at 23% and
aspiration at 11%. In individuals with mild to moderate
impairment, cerebral palsy was cited in 28%, accidents
in 18%, cardiac causes in 15%, and pneumonia in 12% of
www.thelancet.com Vol 383 April 5, 2014

Seminar

reported cases. Inclusion of cerebral palsy on death

certicates as a secondary rather than the sole cause of
death would benet knowledge about cause of death and
how to consider prevention.

Quality of life
In the past decade, studies of individuals with cerebral
palsy have been done within the framework of the
International Classication of Functioning, Disability
and Health (ICF).61 The ICF recognises three constructs:
body structure and function, activity limitation, and
participation. Each construct can inuence the others,
there is not a causative progression from an impairment
of structure to participation. Furthermore, their
interactions are inuenced by the context in which an
individual lives, and the ICF recognises two further
constructs: environmental and personal factors. Means
to measure ICF concepts have been developed or revised;
for children and young people, a recent book has set out
the dierent questionnaires that are available to measure
the constructs.62 Increasingly, children and young people
are asked to complete questionnaires as well as parents.
This shift is in line with the United Nations Declaration
on the Rights of the Child63 that emphasises the need to
listen to and take into account childrens views.
Although cerebral palsy is a lifelong disorder, most
research regards it as a paediatric illness. Recognition
that outcomes in adulthood have been less than positive
has shown the need for clinical practice to adopt a lifelong perspective on the disorder.64 Adults with cerebral
palsy have disadvantages in social life and
employment.65,66 Fatigue, pain, and depressive symptoms
are also common in adults with cerebral palsy,67,68 and
some evidence suggests that physical ageing might
occur more rapidly than in adults without the
disorder.69,70 A life-course perspective also highlights the
transition phase, when a young persons health care
transfers from child to adult services at the same time
as they progress from adolescence to adulthood.
Outcomes are also poor in this period.7173 Paediatric
services often fail to prepare young people for adult
health care. Moreover, an adult is able to balance choices
between therapy, education, pain relief, and
employment, whereas children usually have less control
over such choices. However, as children enter
adolescence, such independence should be encouraged
rather than restricted by parents and clinicians.
Pain in children and adults with cerebral palsy is much
more common than previously thought,67,7476 and is either
not recognised or poorly managed by clinicians. Pain in
individuals with cerebral palsy is caused by many
reasons, including spasms, contractures, hip dislocation,
gastrostomy tubes, gastric reux, and hypersensitivity
around operative scars. Furthermore, therapy is painful
for many children.76 Assisted stretching has been identied as the daily activity most frequently associated with
pain;77 which is of concern because, in individuals with
www.thelancet.com Vol 383 April 5, 2014

neurological disorders such as cerebral palsy, stretching

does not produce clinically signicant changes in contractures or function.78 Moreover, little evidence exists in
favour of the benet of postural management but substantial evidence of its disadvantages, including pain.79
The ICF denes participation as involvement in life
situations and is captured across nine domains including
self-care, interpersonal relationships, schooling, and
employment. Participation is consistently reduced in
children and adults with cerebral palsy compared with
the general population,80,81 and in proportion to the
severity and number of impairments.8284
Health-related quality of life of individuals with cerebral
palsy is lower than that of the general population.64,85,86
However, the constructs captured such as services needed,
frequency of treatment, and activity restrictions inevitably
score lower in individuals with a health disorder such as
cerebral palsy. Such factors could have little inuence on
an individuals overall subjective wellbeing. In those who
are able to self-report, subjective wellbeing is broadly
similar to that of the general population according to
quantitative (gure 3)8789 and qualitative studies.90,91
Children with cerebral palsy have more psychological
diculties than do children of the general population.92,93
Such diculties can be due to disruption of neural
pathways or networks that regulate emotions and
behaviour, thereby rendering the brain less adaptive.
Alternatively, parents can manage and set boundaries
dierently for a disabled child. Societal views and
preconceptions of the parents can mix with parental
feelings of guilt and sadness to fundamentally change
how parents deal with a child with cerebral palsy, thereby
allowing child behaviour that parents would not allow in
a typically developing child.94

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 oers
benet, at least in the short term. Seeking normalisation
of physical impairment in childhood only achieves gains
in the direction of normalisation; even these might not
be sustained beyond the few months of a trial or are lost
as an individual grows heavier. Adults with cerebral palsy
state that their participation in life does not depend on
being able to walk but on communication and being able
to manage and control their environment. Rather than
seeking small improvements in physical function during
childhood which are then lost, concentration on communication and technical skills needed for the workplace
might be more important, as well as the adoption of a
more realistic approach to what physical rehabilitation
might achieve.95
1243

Seminar

Physical wellbeing
Psychological wellbeing
Moods and emotions
Self-perception
Autonomy
Relationships with parents
Social support and peers
School environment
Financial resources
Social acceptance
10

50
Score
Children in general population

90

Children with cerebral palsy

Figure 3: Self-reported quality of life scores by domain for children aged 812 years with cerebral palsy, and
children in the general population of the same age
Median, interquartile range, and adjacent values are shown. Quality of life was assessed with the KIDSCREEN
questionnaire. Reproduced from Dickinson and colleagues.87
For more on KIDSCREEN see
http://www.kidscreen.org/
english

After controlling for severity of cerebral palsy, striking

dierences exist in participation between individuals
from dierent countries or dierent regions of the same
country.82,96 These dierences suggest that environmental
adjustments might be possible, some of which will need
legislation or regulation to raise a country to the standards
of those that best facilitate participation. However, many
environmental adjustments can also be delivered locally.
The importance of family-centred services for disabled
children, which aim to help a family feel in control, be
free of stress, and better able to be responsive to their
child, has been emphasised.97 Additionally, family lifestyle
eects child participation as much for disabled as for
able-bodied children (eg, families with a high participation
in sports).98 The rst randomised controlled trial in this
specialty suggested that environmental adjustments for
children with physical impairment were at least as
eective (as judged by self-help skills and mobility) as
conventional therapeutic interventions that aimed to
change the child.99

Clinical management
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, diculties encountered are not
restricted to an individuals 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
1244

and adapt to the needs of the individual, has its limits.

Successful management balances the social and clinical
models of care; it minimises the eect of medical
diculties while maximising physical ability, environmental adjustment, child and family choice, and social
support.
Individuals with cerebral palsy usually have comorbidities, especially if they have more severe forms of
cerebral palsy, including epilepsy, problems with feeding,
swallowing, and bowel motility, poor nutrition and
growth, high rates of infection, and poor hearing and
vision. In individuals with unilateral spastic cerebral palsy,
partial epilepsy is common.101 In patients with severe
bilateral involvement, up to 50% have generalised epilepsy
for which seizure control can be dicult to achieve.
Moreover, diagnostic diculties can also arise if the
underlying movement disorder mimics epileptic events.
Problems with saliva control are managed by drugs,
botulinum toxin to the salivary glands, or resiting of
salivary ducts.102 Unsafe swallowing and aspiration can
require gastrostomy.103 Gastro-oesophageal reux, delay in
gastric emptying, and constipation are frequently reported
and contribute to poor nutrition. Various factors, including
posture, intake, absorption, and endocrine and gastrointestinal problems can contribute to poor growth and
nutrition in individuals with cerebral palsy.103,104 However,
health and wellbeing, not growth itself, are the objectives,
and a balance needs to be reached for each individual
between growth and the complexity and invasiveness of
any intervention.104 Osteopenia and osteoporosis are
recognised in non-ambulant adults with cerebral palsy,
especially when an individual is on anticonvulsants and
has nutritional diculties.105 Poor nutrition can lead to
immune dysfunction106 and immobility, and deformity can
lead to skin and urinary tract infections; aspiration of
saliva or gastric contents greatly increases the risk of chest
infections.103 Postoperative infection rates, especially after
surgery to correct severe scoliosis, can be as high as 10%.
About 35% of children with cerebral palsy have a visual
problem17 and therefore, all children should have an
ophthalmological assessment. The most common
disorders are strabismus, visual eld defect, myopia, or
hypermetropia. About 8% of children with cerebral palsy
have severe visual impairment, mostly attributable to
cortical visual impairment,107 and noted most often in
those with severe cerebral palsy and associated severe
learning diculties. Permanent sensorineural deafness
is less common and, if present, is usually associated with
neonatal gentamicin therapy or neonatal hyperbilirubinemia.

Management of motor problems

Medical management of motor disorders has changed
substantially in the past 30 years. This change is due, in
part, to introduction of new interventions or modication
of others, but also because of development of appropriate
measures of functional ability that allow goals for clinical
www.thelancet.com Vol 383 April 5, 2014

Seminar

management to be set and outcomes of trials to be

compared. Ordinal classications and scales used include
the Gross Motor Function Classication System,108 the
Manual Ability Classication System,109 the Drooling
Impact Scale,110 the Viking Speech Scale111, and two
classication systems for communication.112,113 Validated
scales also exist for pain, health-related quality of life, and
specic oromotor, upper, and lower limb abilities.
Appropriate interventions for cerebral palsy are dictated
by the patients functional ability, severity, pattern of
motor disorder, associated pain and discomfort, and age.
Paediatric practice aims to reduce secondary musculoskeletal deformity rather than treat the primary central
neurological decit. An adaptive approach is needed to
facilitate all developmental domains and reduce the
eect of medical problems. Physical, occupational, and
speech and language therapy approaches are essential;114
they also work in combination with medical management.
Dierent centres use slightly dierent approaches but
they share core principles. For example, most physical
therapies are based on the principles of neuroplasticity,
patterning, postural balance, muscle strengthening, or
stretching. One integrated educational and therapeutic
programme is conductive education;115 however, a
randomised clinical trial of conductive education did not
show any greater benets in gross motor function than
were noted with traditional physiotherapy approaches.116
Motor interventions aim to change the overactive
elements of the upper motor neurone syndrome by
reducing the eect of increased muscle tone or improving
the uidity of motor control. Their eects might be
temporary, as with oral medication, or permanent as in
most surgical interventions. The evidence for most of
these interventions is weak, partly because of the paucity
of randomised controlled trials but also because of the
many confounders to treatment such as the eect of
comorbidities. Clinical practice has often been based on
reports from case series and expert guidelines. Recently,
the UKs National Institute for Health and Care
Excellence (NICE) panel provided guidelines on spasticity
management in children117 and a review of the use of
selective dorsal rhizotomy.118

Temporary medical interventions

Trihexyphenidyl, tetrabenazine, and carbidopa-levodopa
are antidystonic drugs with anticholinergic or dopamine
analogue properties used to reduce dystonia by
adjusting neurotransmitter bioavailability to the deep
cranial nuclei.119,120
Muscle relaxants work at the spinal and muscle levels to
reduce muscle activation by the spinal reex arc and
spasticity occurring because of lack of descending
inhibition.121 The most commonly used muscle relaxant is
baclofen, a -aminobutyric acid (GABA) B agonist that
depresses the release of excitatory neurotransmitters at
the spinal level. Because baclofen is very lipophilic, it does
not cross the bloodbrain barrier easily. To obtain useful
www.thelancet.com Vol 383 April 5, 2014

CNS concentrations, patients have a dose-dependent risk

of side-eects including sedation, hypoventilation, and
increased frequency of seizures. Therefore, patients
increasingly have baclofen administered by an intrathecal
route with an implantable pump. Evidence of benet is
strongest in the most severely disabled individuals with
gross motor function classication system levels IV and
V.122 Botulinum toxin A injections work by blocking the
release of acetylcholine at the neuromuscular junction,
thereby reducing focal spasticity. The drug has a limited
licence in the UK, but international guidelines for its use
for upper and lower limb spasticity are widely accepted.123,124
Botulinum toxin A has a particular benet in children
with pain secondary to muscle spasm at the hip.125

Neurosurgical interventions
In selective dorsal rhizotomy, some dorsal spinal nerve
rootlets are resected, thereby downregulating the
overactive spinal reex. The procedure is done under
electrophysiological guidance and is predominantly used
in ambulant individuals with bilateral involvement. A
meta-analysis126 of a series of controlled trials conrmed
reduction in spasticity; but whether this reduction led to
improved long-term functional goals was contentious.
Comparative analysis between dierent treatment
modalities showed little variation in outcomes.127129 For
patients whose motor disorder is purely dyskinetic,
researchers are assessing the value of deep brain stimulation, in which quadripolar electrodes are implanted into
the basal ganglia.130 Theoretically, low-voltage stimulation
should lead to better organisation of neuro-modulated
messages, particularly within the globus pallidus;
however, insucient studies have been done to date to
determine long-term clinical and functional ecacy.131

Neuroprotection
Neuroprotective treatments focus on minimising the
brain damage itself. To reduce initial injury, recent
advances in neonatal neuroprotection target babies at
high risk of a perinatal hypoxic-ischaemic event. An
acute event precedes secondary cell death and metabolic
responses, which in turn are followed by ongoing
inammation and epigenetic changes that lead to further
damage in the next few months.132 Therapeutic cerebral
hypothermia after delivery133 and high-dose maternal
magnesium sulfate before delivery134 are given to highrisk term and preterm neonates to reduce the excitooxidative cascade that mediates hypoxic-ischaemic
damage. Several anticonvulsant, anti-excitatory, and antiinammatory agents under investigation increase the
neuroprotective eects of these therapeutic approaches,
including phenobarbital, topiramate, inhaled xenon,
sodium cromoglicate, allopurinol, and melatonin.135
Erythropoietin is also being used to reduce inammation
and apoptosis and directly stimulate neurogenesis.136
Stem cell therapy also aims to reduce these acute
and delayed inammatory responses and stimulate
1245

Seminar

neurogenesis. In cerebral palsy, choice of cell type, dose,

timing, and route of administration are not established,
and the type and distribution of brain damage such therapy
should be directed at is not known. Several international
clinical trials in progress are assessing safety and ecacy
of autologous, embryonic, or induced pluripotential stem
cells. At present, there is no evidence for clinical application
of such therapies in cerebral palsy.137,138

Orthopaedic interventions
The hip joints of individuals with cerebral palsy are at
particular risk of displacement.139 Prevalence of actual
dislocation is highest in the non-ambulant population. In
Sweden and Australia, hip surveillance approaches for
children with cerebral palsy involve serial radiographs
and examinations. When hip subluxation is reported,
early soft and bony tissue surgery has ensured very low
rates of subsequent dislocation.140,141
Monitoring for scoliosis is essential because the
disorder can develop rapidly from a young age in children
with severe bilateral spasticity, and eventually restrict
respiratory function. Interventions such as instrumented
fusion of the spine to the pelvis by insertion of rods can
be undertaken with excellent results in terms of
deformity correction but at the expense of signicant
morbidity in this high-risk group.142,143
In an ambulant individual, contracture formation can
lead to reduced mobility. There is some evidence for the
benets of single-event multilevel orthopaedic surgery
guided by three-dimensional gait analysis followed by
intensive rehabilitation.143 However, recent results of a
5-year follow-up study showed that substantial
improvement in gait was not matched by improvements
in motor function.144 Surgery to lengthen or move
contracted muscle-tendon complexes can improve
biomechanics and provide stability of the supporting base.

Management in developing countries

Few studies have assessed adults with cerebral palsy in
developing countries. In developing countries, proportionately more cerebral palsy in children is of
postneonatal origin than in developed countries; with
accompanying opportunities for prevention and
treatment of infection and trauma. Children with severe
cerebral palsy are more likely to die young because of
malnutrition and infection.145 Access to clinical care is
restricted, so most treatments are provided by families.
Carefully considered low-cost interventions can be very
successful, such as guidance on how to feed a child with
posture and swallowing diculties146 or multidisciplinary
assessment in rural clinics.147,148

Conclusions
In the next decade, the brain lesions described by the
umbrella term cerebral palsy might be classied by their
causal factors coupled with a full description of impairments to body structure and functions they produce.
1246

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 the
individual (such as voice synthesisers and robotic
assistance) and to the environment (such as intelligent
household appliances). Individual and societal attitudes
will continue to change to recognise that individuals with
cerebral palsy have a right not only to inclusion but also
to full participation in society and pursuit of their hopes
and aspirations.
Contributors
PODP is the guarantor of the article and the corresponding author.
PODP, AC, and CF participated in the planning of the review, wrote and
revised the report, and approved the nal version.
Conicts of interest
PODP receives payment for legal reports on the probability of survival in
cerebral palsy from several solicitors pursuing litigation in cases of
cerebral palsy in the past 3 years. CF is an unpaid adviser to the
European Medicines Agency on treatment of spasticity. AC declares that
he has no conicts of interest.
References
1
Little W. On the inuence of abnormal parturition, dicult labours
and asphyxia neonatorum on the mental and physical condition of
the child, especially in relation to deformities. In: Phillips J, Boulton
P, eds. Transactions of the Obstetric Society of London. London:
Obstetrical Society of London, 1862; 3: 293.
2
Rosenbaum P, Paneth N, Leviton A, et al. A report: the denition
and classication of cerebral palsy April 2006.
Dev Med Child Neurol Suppl 2007; 109: 814.
3
Sanger TD, Delgado MR, Gaebler-Spira D, Hallett M, Mink JW, and
the Task Force on Childhood Motor Disorders. Classication and
denition of disorders causing hypertonia in childhood. Pediatrics
2003; 111: e8997.
4
Colver AF, Sethumadhavan T. The term diplegia should be
abandoned. Arch Dis Child 2003; 88: 28690.
5
Surveillance of Cerebral Palsy in Europe. Surveillance of cerebral
palsy in Europe: a collaboration of cerebral palsy surveys and
registers. Dev Med Child Neurol 2000; 42: 81624.
6
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: 54754.
7
Stanley FJ, Watson L. Trends in perinatal mortality and cerebral
palsy in Western Australia, 1967 to 1985. BMJ 1992; 304: 165863.
8
Hagberg B, Hagberg G, Beckung E, Uvebrant P. Changing
panorama of cerebral palsy in Sweden. VIII. Prevalence and
origin in the birth year period 199194. Acta Paediatr 2001;
90: 27177.
9
Pharoah PO, Cooke T, Johnson MA, King R, Mutch L. Epidemiology
of cerebral palsy in England and Scotland, 19849.
Arch Dis Child Fetal Neonatal Ed 1998; 79: F2125.
10 Gladstone M. A review of the incidence and prevalence, types and
aetiology of childhood cerebral palsy in resource-poor settings.
Ann Trop Paediatr 2010; 30: 18196.
11 Ibrahim SH, Bhutta ZA. Prevalence of early childhood disability in
a rural district of Sind, Pakistan. Dev Med Child Neurol 2013;
55: 35763.
12 Paneth N, Kiely J. The frequency of cerebral palsy: a review of
population studies in industrialised nations since 1950.
Clin Dev Med 1984; 87: 4656.
13 Stanley F, Alberman E. Birthweight, gestational age and the
cerebral palsies. Clin Dev Med 1984; 87: 5768.
14 Sellier E, Surman G, Himmelmann K, 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;
25: 63542.

www.thelancet.com Vol 383 April 5, 2014

Seminar

15
16

17
18
19

20
21

22

23

24

25
26

27

28
29
30

31

32

33

34
35
36

37

38

39
40

Maudsley G, Hutton JL, Pharoah PO. Cause of death in cerebral

palsy: a descriptive study. Arch Dis Child 1999; 81: 39094.
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: 4350.
Report of the Australian cerebral palsy register, birth years 19932006.
Sydney, NSW, Australia: Cerebral Palsy Research Institute, 2013.
Freud S. Infantile cerebralhmung, 1897 [Infantile cerebral
paralysis]. Florida: University of Miami Press, 1968.
National Perinatal Epidemiology Unit. Oxford register of early
childhood impairments. Oxford, UK: National Perinatal
Epidemiology Unit, 2002.
Pharoah PO, Cooke T. Cerebral palsy and multiple births.
Arch Dis Child Fetal Neonatal Ed 1996; 75: F17477.
Burguet A, Monnet E, Pauchard JY, 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; 75: 17786.
Grether JK, Nelson KB, Cummins SK. Twinning and cerebral palsy:
experience in four northern California counties, births 1983
through 1985. Pediatrics 1993; 92: 85458.
Petterson B, Nelson KB, Watson L, Stanley F. Twins, triplets, and
cerebral palsy in births in Western Australia in the 1980s. BMJ 1993;
307: 123943.
Pharoah PO. Cerebral palsy in the surviving twin associated with
infant death of the co-twin. Arch Dis Child Fet Neonatal Ed 2001;
84: F11116.
Blair E, Watson L. Epidemiology of cerebral palsy.
Semin Fetal Neonatal Med 2006; 11: 11725.
McIntyre S, Taitz D, Keogh J, Goldsmith S, Badawi N, Blair E.
A systematic review of risk factors for cerebral palsy in children
born at term in developed countries. Dev Med Child Neurol 2013;
55: 499508.
Jarvis S, Glinianaia S, Torrioli M, et al, for the Surveillance of
Cerebral Palsy in Europe (SCPE) collaboration of European Cerebral
Palsy Registers. Cerebral palsy and intrauterine growth in single
births: European collaborative study. Lancet 2003; 62: 110611.
Krgeloh-Mann I, Cans C. Cerebral palsy update. Brain Dev 2009;
31: 53744.
Blair E, Stanley FJ. Issues in the classication and epidemiology of
cerebral palsy. Ment Retard Dev Disabil Res Rev 1997; 3: 18493.
MacLennan A. A template for dening a causal relation between
acute intrapartum events and cerebral palsy: international
consensus statement. BMJ 1999; 319: 105459.
Moore CM, McAdams AJ, Sutherland J. Intrauterine disseminated
intravascular coagulation: a syndrome of multiple pregnancy with a
dead twin fetus. J Pediatr 1969; 74: 52328.
Hoyme HE, Higginbottom MC, Jones KL. Vascular etiology of
disruptive structural defects in monozygotic twins. Pediatrics 1981;
67: 28891.
Fusi L, McParland P, Fisk N, Nicolini U, Wigglesworth J. Acute
twin-twin transfusion: a possible mechanism for brain-damaged
survivors after intrauterine death of a monochorionic twin.
Obstet Gynecol 1991; 78: 51720.
Landy H, Nies B. The vanishing twin. New York, NY, USA:
Parthenon Publishing Group, 1995.
Landy HJ, Keith LG. The vanishing twin: a review.
Hum Reprod Update 1998; 4: 17783.
Pharoah PO, Cooke RW. A hypothesis for the aetiology of spastic
cerebral palsythe vanishing twin. Dev Med Child Neurol 1997;
39: 29296.
Dunn J, Pretell E, Daza C, Viteri F, eds. Towards the eradication of
endemic goitre, cretinism and iodine deciency. Austin, TX, USA:
Pan American Health Organization, 1986.
Pharoah PO, Butteld IH, Hetzel BS. Neurological damage to the
fetus resulting from severe iodine deciency during pregnancy.
Lancet 1971; 1: 30810.
Pharoah PO, Hornabrook RW. Endemic cretinism of recent onset
in New Guinea. Lancet 1974; 2: 103840.
Shatrov JG, Birch SC, Lam LT, Quinlivan JA, McIntyre S,
Mendz GL. Chorioamnionitis and cerebral palsy: a meta-analysis.
Obstet Gynecol 2010; 116: 38792.

www.thelancet.com Vol 383 April 5, 2014

41

42
43

44

45
46
47

48

49

50

51

52

53

54
55
56

57

58

59
60
61
62
63
64

65

Croen LA, Grether JK, Curry CJ, Nelson KB. Congenital

abnormalities among children with cerebral palsy: More evidence
for prenatal antecedents. J Pediatr 2001; 138: 80410.
Pharoah PO. Prevalence and pathogenesis of congenital anomalies
in cerebral palsy. Arch Dis Child Fetal Neonatal Ed 2007; 92: F48993.
Rankin J, Cans C, Garne E, et al. Congenital anomalies in children
with cerebral palsy: a population-based record linkage study.
Dev Med Child Neurol 2010; 52: 34551.
Bahtiyar MO, Dulay AT, Weeks BP, Friedman AH, Copel JA.
Prevalence of congenital heart defects in monochorionic/diamniotic
twin gestations: a systematic literature review. J Ultrasound Med
2007; 26: 149198.
Pharoah POD, Dundar Y. Monozygotic twinning, cerebral palsy and
congenital anomalies. Hum Reprod Update 2009; 15: 63948.
Pharoah POD. Causal hypothesis for some congenital anomalies.
Twin Res Hum Genet 2005; 8: 54350.
Hvidtjrn D, Grove J, Schendel DE, et al. Cerebral palsy among
children born after in vitro fertilization: the role of preterm
deliverya population-based, cohort study. Pediatrics 2006;
118: 47582.
Hvidtjrn D, Grove J, Schendel D, et al. Multiplicity and early
gestational age contribute to an increased risk of cerebral palsy
from assisted conception: a population-based cohort study.
Hum Reprod 2010; 25: 211523.
OCallaghan ME, Maclennan AH, Gibson CS, et al, and the
Australian Collaborative Cerebral Palsy Research Group. Fetal and
maternal candidate single nucleotide polymorphism associations
with cerebral palsy: a case-control study. Pediatrics 2012;
129: e41423.
Wu YW, Croen LA, Vanderwerf A, Gelfand AA, Torres AR.
Candidate genes and risk for CP: a population-based study.
Pediatr Res 2011; 70: 64246.
Moreno-De-Luca A, Ledbetter DH, Martin CL. Genetic insights into
the causes and classication of cerebral palsies. Lancet Neurol 2012;
11: 28392.
Krgeloh-Mann I, Horber V. The role of magnetic resonance
imaging in elucidating the pathogenesis of cerebral palsy:
a systematic review. Dev Med Child Neurol 2007; 49: 14451.
Ashwal S, Russman BS, Blasco PA, et al, and the Quality Standards
Subcommittee of the American Academy of Neurology, and the
Practice Committee of the Child Neurology Society. 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: 85163.
Hutton JL, Pharoah PO. Life expectancy in severe cerebral palsy.
Arch Dis Child 2006; 91: 25458.
Strauss D, Brooks J, Rosenbloom L, Shavelle R. Life expectancy in
cerebral palsy: an update. Dev Med Child Neurol 2008; 50: 48793.
Blair E, Watson L, Badawi N, Stanley FJ. Life expectancy among
people with cerebral palsy in Western Australia.
Dev Med Child Neurol 2001; 43: 50815.
Michael J. Healthcare for all: report of the independent inquiry into
access to healthcare for people with learning disabilities London,
UK: Department of Health, 2008.
Reid SM, Carlin JB, Reddihough DS. Survival of individuals with
cerebral palsy born in Victoria, Australia, between 1970 and 2004.
Dev Med Child Neurol 2012; 54: 35360.
Evans PM, Alberman E. Certied cause of death in children and
young adults with cerebral palsy. Arch Dis Child 1991; 66: 32529.
Strauss D, Cable W, Shavelle R. Causes of excess mortality in
cerebral palsy. Dev Med Child Neurol 1999; 41: 58085.
WHO. International classication of functioning, disability and
health. Geneva, Switzerland: World Health Organisation, 2001.
Majnemer A. Measures for children with developmental disabilities:
an ICF-CY approach, 1st edn. London, UK: Mac Keith Press, 2012.
United Nations. Convention on the rights of the child. New York,
NY, USA: United Nations, 1989.
Roebroeck ME, Jahnsen R, Carona C, Kent RM, Chamberlain MA.
Adult outcomes and lifespan issues for people with childhood-onset
physical disability. Dev Med Child Neurol 2009; 51: 67078.
Michelsen SI, Uldall P, Hansen T, Madsen M. Social integration of
adults with cerebral palsy. Dev Med Child Neurol 2006; 48: 64349.

1247

Seminar

66

67

68

69
70
71
72

73

74

75

76

77
78

79

80

81
82

83
84

85

86
87

88

89

90

1248

Michelsen SI, Uldall P, Kejs AMT, Madsen M. Education and

employment prospects in cerebral palsy. Dev Med Child Neurol 2005;
47: 51117.
Opheim A, Jahnsen R, Olsson E, Stanghelle JK. Walking function,
pain, and fatigue in adults with cerebral palsy: a 7-year follow-up
study. Dev Med Child Neurol 2009; 51: 38188.
Van Der Slot WM, Nieuwenhuijsen C, Van Den Berg-Emons RJ, et al.
Chronic pain, fatigue, and depressive symptoms in adults with spastic
bilateral cerebral palsy. Dev Med Child Neurol 2012; 54: 83642.
Strax TE, Luciano L, Dunn AM, Quevedo JP. Aging and
developmental disability. Phys Med Rehabil Clin N Am 2010; 21: 41927.
Haak P, Lenski M, Hidecker MJ, Li M, Paneth N. Cerebral palsy and
aging. Dev Med Child Neurol 2009; 51 (suppl 4): 1623.
Kirk S. Transitions in the lives of young people with complex
healthcare needs. Child Care Health Dev 2008; 34: 56775.
Nieuwenhuijsen C, Donkervoort M, Nieuwstraten W, Stam HJ,
Roebroeck ME, and the Transition Research Group South West
Netherlands. Experienced problems of young adults with cerebral
palsy: targets for rehabilitation care. Arch Phys Med Rehabil 2009;
90: 189197.
Ko B, McEnery G. The needs of physically disabled young people
during transition to adult services. Child Care Health Dev 2004;
30: 31723.
Parkinson KN, Gibson L, Dickinson HO, Colver AF. Pain in
children with cerebral palsy: a cross-sectional multicentre European
study. Acta Paediatr 2010; 99: 44651.
Ramstad K, Jahnsen R, Skjeldal OH, Diseth TH. Characteristics of
recurrent musculoskeletal pain in children with cerebral palsy aged
8 to 18 years. Dev Med Child Neurol 2011; 53: 101318.
Parkinson KN, Dickinson HO, Arnaud C, Lyons A, Colver A, and
the SPARCLE group. Pain in young people aged 13 to 17 years with
cerebral palsy: cross-sectional, multicentre European study.
Arch Dis Child 2013; 98: 43440.
Hadden KL, von Baeyer CL. Pain in children with cerebral palsy:
common triggers and expressive behaviors. Pain 2002; 99: 28188.
Katalinic OM, Harvey LA, Herbert RD. Eectiveness of stretch for the
treatment and prevention of contractures in people with neurological
conditions: a systematic review. Phys Ther 2011; 91: 1124.
Gough M. Continuous postural management and the prevention of
deformity in children with cerebral palsy: an appraisal.
Dev Med Child Neurol 2009; 51: 10510.
Michelsen SI, Flachs EM, Uldall P, et al. Frequency of participation
of 812-year-old children with cerebral palsy: a multi-centre
cross-sectional European study. Eur J Paediatr Neurol 2009; 13: 16577.
Imms C, Reilly S, Carlin J, Dodd K. Diversity of participation in
children with cerebral palsy. Dev Med Child Neurol 2008; 50: 36369.
Fauconnier J, Dickinson HO, Beckung E, et al. Participation in life
situations of 812 year old children with cerebral palsy: cross
sectional European study. BMJ 2009; 338: b1458.
Imms C. Children with cerebral palsy participate: a review of the
literature. Disabil Rehabil 2008; 30: 186784.
Majnemer A, Shevell M, Law M, et al. Participation and enjoyment
of leisure activities in school-aged children with cerebral palsy.
Dev Med Child Neurol 2008; 50: 75158.
Roebroeck ME, Jahnsen R, Carona C, Kent RM, Chamberlain MA.
Adult outcomes and lifespan issues for people with childhood-onset
physical disability. Dev Med Child Neurol 2009; 51: 67078.
Vargus-Adams J. Health-related quality of life in childhood cerebral
palsy. Arch Phys Med Rehabil 2005; 86: 94045.
Dickinson HO, Parkinson KN, Ravens-Sieberer U, et al.
Self-reported quality of life of 812-year-old children with cerebral
palsy: a cross-sectional European study. Lancet 2007; 369: 217178.
Bjornson KF, Belza B, Kartin D, Logsdon RG, McLaughlin J.
Self-reported health status and quality of life in youth with cerebral
palsy and typically developing youth. Arch Phys Med Rehabil 2008;
89: 12127.
Shields N, Loy Y, Murdoch A, Taylor NF, Dodd KJ. Self-concept of
children with cerebral palsy compared with that of children without
impairment. Dev Med Child Neurol 2007; 49: 35054.
Watson N, Shakespeare T, Cunningham-Burley S, Barnes C. Life as
a disabled child: a qualitative study of young peoples experience
and perspectives. Edinburgh, UK: Department of Nursing Studies,
University of Edinburgh, Economic and Social Science Research
Council, 1999.

91

92

93

94

95

96

97

98

99

100
101
102
103

104
105

106
107

108

109

110

111

112

113

Shikako-Thomas K, Lach L, Majnemer A, Nimigon J, Cameron K,

Shevell M. Quality of life from the perspective of adolescents with
cerebral palsy: I just think Im a normal kid, I just happen to have
a disability. Qual Life Res 2009; 18: 82532.
Parkes J, White-Koning M, Dickinson HO, et al. Psychological
problems in children with cerebral palsy: a cross-sectional European
study. J Child Psychol Psychiatry 2008; 49: 40513.
Sigurdardottir S, Indredavik MS, Eiriksdottir A, Einarsdottir K,
Gudmundsson HS, Vik T. Behavioural and emotional symptoms of
preschool children with cerebral palsy: a population-based study.
Dev Med Child Neurol 2010; 52: 105661.
Woolfson L. Family well-being and disabled children: a psychosocial
model of disability-related child behaviour problems.
Br J Health Psychol 2004; 9: 113.
Moll LR, Cott CA. The paradox of normalization through
rehabilitation: growing up and growing older with cerebral palsy.
Disabil Rehabil 2013; 35: 127683.
Hammal D, Jarvis SN, Colver AF. Participation of children with
cerebral palsy is inuenced by where they live. Dev Med Child Neurol
2004; 46: 29298.
Rosenbaum P. Family and quality of life: key elements in
intervention in children with cerebral palsy. Dev Med Child Neurol
2011; 53 (suppl 4): 6870.
King G, Law M, Hanna S, et al. Predictors of the leisure and
recreation participation of children with physical disabilities:
a structural equation modeling analysis. Child Health Care 2006;
35: 20934.
Law MC, Darrah J, Pollock N, et al. Focus on function: a cluster,
randomized controlled trial comparing child- versus
context-focused intervention for young children with cerebral palsy.
Dev Med Child Neurol 2011; 53: 62129.
Oliver M. Theories in health care and research: theories of disability
in health practice and research. BMJ 1998; 317: 144649.
Hadjipanayis A, Hadjichristodoulou C, Youroukos S. Epilepsy in
patients with cerebral palsy. Dev Med Child Neurol 1997; 39: 65963.
Fairhurst C, Cockerill H. Management of drooling in children.
Arch Dis Child Pract Ed 2011; 96: 2530.
Sullivan P, Morrice J, Vernon-Roberts A, Grant H, Eltumi M,
Thomas A. Does gastrostomy tube feeding increase the risk of
respiratory morbidity? Arch Dis Child 2006; 91: 47882.
Kuperminc M, Stevenson R. Growth and nutritional disorders in
children with cerebral palsy. Dev Dis Res Rev 2008; 14: 13746.
Fehlings D, Switzer L, Agarwal P, et al. Informing evidence-based
clinical practice guidelines for children with cerebral palsy at risk of
osteoporosis: a systematic review. Dev Med Child Neurol 2012;
54: 10616.
Chandra RK, Kumari S. Nutrition and immunity: an overview.
J Nutr 1994; 124 (suppl): S143335.
Dutton G, Bax M, eds. Visual impairment in children due to
damage to the brain. Clinics in developmental medicine no. 186.
London: Mac Keith Press, Wiley-Blackwell, 2010.
Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B.
Development and reliability of a system to classify gross motor
function in children with cerebral palsy. Dev Med Child Neurol 1997;
39: 21423.
Eliasson AC, Krumlinde-Sundholm L, Rsblad B, et al. The Manual
Ability Classication System (MACS) for children with cerebral
palsy: scale development and evidence of validity and reliability.
Dev Med Child Neurol 2006; 48: 54954.
Reid SM, Johnson HM, Reddihough DS. The Drooling Impact
Scale: a measure of the impact of drooling in children with
developmental disabilities. Dev Med Child Neurol 2010; 52: e2328.
Pennington L, Virella D, Mjen T, et al. Development of The Viking
Speech Scale to classify the speech of children with cerebral palsy.
Res Dev Disabil 2013; 34: 320210.
Hidecker MJ, Paneth N, Rosenbaum PL, et al. Developing and
validating the Communication Function Classication System for
individuals with cerebral palsy. Dev Med Child Neurol 2011;
53: 70410.
The Centre for Cerebral Palsy and Cerebral Palsy League of
Queensland. Functional Communication Classication System
http://www.tccp.com.au/docs/documents/FCCS%20and%20
Prompt%20Questions.pdf (accessed Oct 11, 2013).

www.thelancet.com Vol 383 April 5, 2014

Seminar

114 Damiano DL. Rehabilitative therapies in cerebral palsy: the good,

the not as good, and the possible. J Child Neurol 2009; 24: 120004.
115 Anttila H, Suoranta J, Malmivaara A, Mkel M, Autti-Rm I.
Eectiveness of physiotherapy and conductive education
interventions in children with cerebral palsy: a focused review.
Am J Phys Med Rehabil 2008; 87: 478501.
116 Reddihough DS, King J, Coleman G, Catanese T. Ecacy of
programmes based on Conductive Education for young children
with cerebral palsy. Dev Med Child Neurol 1998; 40: 76370.
117 NICE. Guideline 145. Spasticity in children and young people with
non-progressive brain disorders. Manchester, UK: National
Institute for Health and Clinical Excellence, 2012.
118 NICE. Report IP318-2. Interventional procedure overview of
selective dorsal rhizotomy for spasticity in cerebral palsy. London,
UK: National Institute for Health and Clinical Excellence, 2009.
119 Cloud LJ, Jinnah HA. Treatment strategies for dystonia.
Expert Opin Pharmacother 2010; 11: 515.
120 Carranza-del Rio J, Clegg NJ, Moore A, Delgado MR. Use of
trihexyphenidyl in children with cerebral palsy. Pediatr Neurol 2011;
44: 20206.
121 Delgardo M, Hirtz D, Aisen M, Ashwal S. Practice parameter:
pharmacologic treatment of spasticity in children and adolescents
with cerebral palsy (an evidence based review). Report of the
Quality Standards Sub-Committee of the American Academy of
Neurology and Practice Committee of the Child Neurology Society.
Neurology 2010; 24: 33643.
122 Dan B, Motta F, Vles JS, et al. Consensus on the appropriate use of
intrathecal baclofen (ITB) therapy in paediatric spasticity.
Eur J Paediatr Neurol 2010; 14: 1928.
123 Simpson DM, Gracies JM, Graham HK, et al, and the Therapeutics
and Technology Assessment Subcommittee of the American
Academy of Neurology. Assessment: Botulinum neurotoxin for the
treatment of spasticity (an evidence-based review): report of the
Therapeutics and Technology Assessment Subcommittee of the
American Academy of Neurology. Neurology 2008; 70: 169198.
124 Heinen F, Desloovere K, Schroeder AS, et al. The updated
European Consensus 2009 on the use of Botulinum toxin for
children with cerebral palsy. Eur J Paediatr Neurol 2010; 14: 4566.
125 Lundy CT, Doherty GM, Fairhurst CB. Botulinum toxin type A
injections can be an eective treatment for pain in children with hip
spasms and cerebral palsy. Dev Med Child Neurol 2009; 51: 70510.
126 Grunt S, Becher JG, Vermeulen RJ, Becher JG, Vermuelen RJ.
Long-term outcome and adverse eects of selective dorsal
rhizotomy in children with cerebral palsy: a systematic review.
Dev Med Child Neurol 2011; 53: 49098.
127 Kan P, Gooch J, Amini A, et al. Surgical treatment of spasticity in
children: comparison of selective dorsal rhizotomy and intrathecal
baclofen pump implantation. Childs Nerv Syst 2008; 24: 23943.
128 Buckon CE, Thomas SS, Piatt JH Jr, Aiona MD, Sussman MD.
Selective dorsal rhizotomy versus orthopedic surgery:
a multidimensional assessment of outcome ecacy.
Arch Phys Med Rehabil 2004; 85: 45765.
129 Wong A, Pei Y-C, Lui T. Comparison between botulinum toxin type
A injections and selective posterior rhizotomy in improving gait
performance in children with cerebral palsy. J Neurosurg Pediatr
2005; 102: 38589.
130 Vidailhet M, Yelnik J, Lagrange C, et al, and the French SPIDY-2
Study Group. Bilateral pallidal deep brain stimulation for the
treatment of patients with dystonia-choreoathetosis cerebral palsy:
a prospective pilot study. Lancet Neurol 2009; 8: 70917.

www.thelancet.com Vol 383 April 5, 2014

131 Koy A, Hellmich M, Pauls KA, et al. Eects of deep brain

stimulation in dyskinetic cerebral palsy: a meta-analysis. Mov Disord
2013; 28: 64754.
132 Fleiss B, Gressens P. Tertiary mechanisms of brain damage: a new
hope for treatment of cerebral palsy? Lancet Neurol 2012; 11: 55666.
133 Edwards AD, Brocklehurst P, Gunn AJ, et al. Neurological
outcomes at 18 months of age after moderate hypothermia for
perinatal hypoxic ischaemic encephalopathy: synthesis and
meta-analysis of trial data. BMJ 2010; 340: c363.
134 Doyle LW, Crowther CA, Middleton P, Marret S, Rouse D.
Magnesium sulphate for women at risk of preterm birth for
neuroprotection of the fetus. Cochrane Database Syst Rev 2009;
1: CD004661.
135 Johnston MV, Fatemi A, Wilson MA, Northington F. Treatment
advances in neonatal neuroprotection and neurointensive care.
Lancet Neurol 2011; 10: 37282.
136 McPherson RJ, Juul SE. Erythropoietin for infants with
hypoxic-ischemic encephalopathy. Curr Opin Pediatr 2010; 22: 13945.
137 Gaspard N, Vanderhaeghen P. From stem cells to neural networks:
recent advances and perspectives for neurodevelopmental
disorders. Dev Med Child Neurol 2011; 53: 1317.
138 Bennet L, Tan S, Van den Heuij L, et al. Cell therapy for neonatal
hypoxia-ischaemia and cerebral palsy. Ann Neurol 2012; 7: 586600.
139 Scrutton D, Baird G, Smeeton N. Hip dysplasia in bilateral cerebral
palsy: incidence and natural history in children aged 18 months to
5 years. Dev Med Child Neurol 2001; 43: 586600.
140 Wynter M, Gibson N, Kentish M, Love S, Thomason P,
Kerr Graham H. The consensus statement on hip surveillance for
children with cerebral palsy: Australian standards of care.
J Pediatr Rehabil Med 2011; 4: 18395.
141 Jones-Quaidoo SM, Yang S, Arlet V. Surgical management of spinal
deformities in cerebral palsy. A review. J Neurosurg Spine 2010;
13: 67285.
142 Sponseller PD, Shah SA, Abel MF, Newton PO, Letko L, Marks M.
Infection rate after spine surgery in cerebral palsy is high and
impairs results: multicenter analysis of risk factors and treatment.
Clin Orthop Relat Res 2010; 468: 71116.
143 Robb J, Brunner R. Management of cerebral palsy. Childrens
orthopaedics and fractures. London, UK: Springer Verlag,
2010: 30725.
144 Thomason P, Selber P, Graham HK. Single event multilevel surgery
in children with bilateral spastic diplegia: a 5 year prospective study.
Gait Posture 2013; 37: 2328.
145 Khan NZ, Ferdous S, Munir S, Huq S, McConachie H. Mortality of
urban and rural young children with cerebral palsy in Bangladesh.
Dev Med Child Neurol 1998; 40: 74953.
146 Adams MS, Khan NZ, Begum SA, Wirz SL, Hesketh T, Pring TR.
Feeding diculties in children with cerebral palsy: low-cost
caregiver training in Dhaka, Bangladesh. Child Care Health Dev
2012; 38: 87888.
147 Morgan F, Tan B-K. Rehabilitation for children with cerebral palsy
in rural Cambodia: parental perceptions of family-centred practices.
Child Care Health Dev 2011; 37: 16167.
148 McConachie H, Huq S, Munir S, Ferdous S, Zaman S, Khan NZ.
A randomized controlled trial of alternative modes of service
provision to young children with cerebral palsy in Bangladesh.
J Pediatr 2000; 137: 76976.

1249