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Disability and Rehabilitation, February 2006; 28(4): 183 – 191

REVIEW

The epidemiology of cerebral palsy: Incidence, impairments


and risk factors

ELSE ODDING, MARIJ E. ROEBROECK, & HENDRIK J. STAM

Department of Rehabilitation Medicine, Erasmus MC – University Medical Centre Rotterdam, Rotterdam, The Netherlands
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Accepted April 2005.

Abstract
Purpose. Describing the epidemiology of cerebral palsy (CP), its impairments and risk factors.
Method. Literature review 1965 – 2004. Search terms: Cerebral palsy, incidence, prevalence, impairments, risk factors.
Results. In the last 40 years the prevalence of CP has risen to well above 2.0 per 1000 life births. In this time span the
proportion of low-birthweight infants rose, the proportion of diplegia decreased, while the proportion of hemiplegia
increased. CP is more prevalent in more deprived socio-economic populations. The majority of people with CP have the
spastic syndrome of which the diplegic group is the smallest. Dependent on the subgroup of CP, 25 – 80% have
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additional impairments. A large proportion has some kind of cognitive impairment; the prevalence varies with the type
of CP and especially increases when epilepsy is present. Epilepsy is present in 20 – 40%; it is most common among the
hemi- and tetraplegics. Sensibility of the hands is impaired in about half. Chronic pain is reported by more than a
quarter of the adults. Up to 80% have at least some impairment of speech. Low visual acuity is reported in almost
three-quarters of all children. Half of all children have gastrointestinal and feeding problems. Stunted growth occurs in a
quarter, while under- or overweight problems are present in half of the children. Almost 70% of people with spastic CP
have abnormal brain CT findings; abnormal cranial ultrasounds is most strongly associated with hemiplegia, normal
cranial ultrasounds with diplegia. The most important risk factors for CP are low birthweight, intrauterine infections and
multiple gestation.

Keywords: Cerebral palsy, epidemiology, incidence, impairment, risk factors

introducing or reviewing measurement instruments,


Introduction
and describing specific impairments.
In The Netherlands as well as in many other western Combining the search term cerebral palsy with
countries children diagnosed with cerebral palsy the terms epidemiology, and incidence and pre-
(CP) are referred for rehabilitation care. They are valence, respectively, revealed 757 manuscripts
the largest diagnostic group treated in pediatric published between 1990 and 2002. Of these, the
rehabilitation. Research on the subject of CP is, vast majority is published in pediatric (411),
however, predominantly published in journals other obstetric (76), and neurological (70) journals. Only
than the 49 rehabilitation journals listed in PubMed. 13 manuscripts with the terms incidence and/or
In PubMed and Medline the search term CP was prevalence were published in rehabilitation journals.
found in 8566 articles, of which only 309 were in Of the 75 review articles about the epidemiology of
rehabilitation journals. Of those 309 articles, 93 were CP, published between 1990 and 2004, none were
published before 1980, 66 between 1980 and 1990, published in the rehabilitation journals. It must be
leaving 150 articles of more recent date. The concluded that an important aspect of the scientific
topics in the rehabilitation journals predominantly knowledge about CP is missing in the rehabilitation
are: (new) treatment policies, motion analysis, literature.

Correspondence: M.E. Roebroeck, Department of Rehabilitation Medicine, Erasmus MC – University Medical Centre Rotterdam, P.O. Box 2040, 3000 CA
Rotterdam, The Netherlands. Tel: 31 10 463 4769. Fax: 31 10 463 3843. E-mail: m.roebroeck@erasmusmc.ul
ISSN 0963-8288 print/ISSN 1464-5165 online ª 2006 Taylor & Francis
DOI: 10.1080/09638280500158422
184 E. Odding et al.

The Department of Rehabilitation Medicine of the 1000 neonatal survivors weighing less than 1500 g,
Erasmus Medical Centre Rotterdam investigates, in and from 3.9 to 11.5 for those weighing 1500 – 2499 g
co-operation with the rehabilitation centers in the [10]. More detailed study of the period between 1983
southwest region of The Netherlands, the long-term and 1995 revealed an increase for those weighing less
consequences of childhood disability. In order to than 1000 g to 90 per 1000 in 1987 – 1989 and a
design proper studies, two issues have to be resolved, decrease to 57 in 1993 – 1995. Among infants weigh-
the first being: which outcome measures and ing 1000 – 1500 g the rate rose to 77 in 1987 – 1989
potential determinants should be assessed. Or, to and then fell to 40 in 1993 – 1995 [13,14]. The
put it differently, what is known about the epide- proportion of low-birthweight infants among all
miology of CP? The second issue involves the way in children with CP is rising: 32% of all cases in 1966
which those outcome measures and determinants and 50% in 1989 [15]. Newborns weighing less than
should be assessed, or: do we have valid and reliable 2500 g now contribute half of all cases of CP and just
measurement instruments? over half of the most severe cases, whereas in the 1960s
This paper presents the results of a literature review they contributed one-third of all cases, and only one-
on the epidemiology of CP, and its impairments and sixth of the most severe [9].
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risk factors. The second issue – about measurement There is a clear social class gradient in the
instruments – will be addressed in another paper. prevalence of CP as a whole, and in the subgroups
hemiplegia and diplegia. Among normal birthweight
cases there is a strong association with social class [16].
Methods
In the United Kingdom the prevalence of CP at birth is
The literature search was performed on the Medline 3.33 per 1000 births in the most deprived quintile, and
database for the period 1966 – 2002, complemented 2.08 in the most affluent quintile of the Carstairs area
with PubMed for the period 1965 – 2004. Search deprivation index. This socio-economic gradient was
terms were: cerebral palsy, epidemiology, preva- similar at age five [17]. There is also an association
lence, incidence, impairment, assessment, adoles- between birthweight and socio-economic status.
For personal use only.

cents, hemiplegic, diplegic, tetraplegic, spasticity, Within the normal birthweight category the preva-
cognitive, behavior, speech, dysarthria, risk factors. lence of CP is 2.42 per 1000 in the most deprived
quintile, and 1.29 in the most affluent quintile. A
greater proportion of CP births in the more deprived
Prevalence and incidence
quintiles is of low or very low birthweight.
In 1998, fourteen centers in eight European countries The excess of summer births among children born
started a network called Surveillance of Cerebral with CP might be explained by the effect of infectious
Palsy in Europe (SCPE). After reaching consensus agents, many of which vary seasonally [18].
about the criteria to classify CP, they presented the
prevalence rates in six countries, and more detailed
Impairments
prevalence estimates of 13 areas [1,2]. In Table I a
summary of the prevalence estimates of CP of the Table II gives a summary of the data about the most
SCPE, as well as from some other northwestern common impairments associated with CP. In the
Europe countries, is given. As can be concluded from first column crude prevalences are given. The second
this table the prevalence and the trends in time of CP presents the prevalence of impairments in various
are comparable in these countries. The prevalence of subgroups. In the last columns the prevalence of the
CP rises in time from well below 2.0 per 1000 life various impairments within the diagnostic subgroups
births in the 1970s to well above 2.0 in the 1990s [3 – of CP is given, in so far as they could be traced from
9]. Boys form a small majority (58%) [5]. the literature. It must be noted that, because data are
It seems fair to assume that these European data derived from several publications, the totals of the
are not very different from findings in other parts of subgroups do not necessarily add up to 100.
the world. For example the prevalence of CP in
China is reported to be 1.6 per 1000 children under
Motor impairments
age 7 [10]. In Mississippi (USA) 2.12 per 1000
inhabitants were diagnosed with CP with a higher Motor impairment is obligatory for the diagnosis CP.
prevalence for males, and a, non-significant, higher Table II shows the distribution of these motor
prevalence in black people [11]. The prevalence of impairments. In summary: the majority is consti-
CP in Australia is 2.0 to 2.5 per 1000 live births [12]. tuted of the spastic syndromes, of which the diplegic
The prevalence of CP among low-birthweight group is the smallest [19 – 21]. With the rising
children is higher than among normal birthweight incidence of CP in time, the distribution over the
children [3 – 5,10]. From 1964 to 1993 the incidence subtypes changed: fewer cases with diplegia and
rose in low-birthweight children from 29.8 to 74.2 per more with hemiplegia [15]. The motor impairments
The epidemiology of cerebral palsy 185

Table I. Prevalence of cerebral palsy in Northwest Europe.

Birthweight specific
per 1000 lifebirths
Prevalence
Birthyear per 1000 lifebirths 51000 1000 – 1499 1500 – 2499 42500

Northeast England 1964 – 1968 1.68 59.7 6.55 1.19


1969 – 1973 1.39 22.9 6.40 0.96
1974 – 1978 1.71 27.6 8.90 1.20
1979 – 1983 2.00 76.6 8.22 1.26
1984 – 1988 2.27 75.9 9.65 1.31
1989 – 1993 2.45 80.0 11.80 1.26
Scotland, England 1984 – 1989 2.10 78.1 65.7 10.20 1.10
Northern Ireland 1981 – 1993 2.24
Norway 1977 – 1981 1.91 88.9
1982 – 1986 1.98 28.8
1987 – 1991 2.05
Denmark 1979 – 1986 2.80
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1987 – 1990 2.40


Sweden 1979 – 1982 2.17
1990 – 1993 2.20
1991 – 1994 2.12
The Netherlands 1977 – 1979 0.77
1980 – 1982 1.00
1983 – 1985 1.84
1986 – 1988 2.44

of CP, in especially the spastic types, lead to other impairment [1,29]. The prevalence varies with the
For personal use only.

impairments of the musculoskeletal system; for type of CP and especially increases when epilepsy is
example, among tetraplegic children and adoles- present. In severely disabled CP children, 97.7% are
cents, 75% have hip luxations, 73% contractures, profoundly mentally impaired [30]. But, since 1976
and 72% scoliosis [22]. the prevalence of severe mental retardation has
Children and adolescents with CP have a lower decreased significantly [31].
physical fitness compared with their able-bodied peers About 40% of children with hemiplegic CP have
[23]. They also have subnormal values for peak normal cognitive abilities, while children and ado-
anaerobic power and muscular endurance of the lescents with tetraplegic CP are generally severely
upper and lower limbs. They score between 2 and 4 intellectually impaired. [22,32]. There is no associa-
standard deviations below the expected mean value for tion between IQ level and memory scores and
power. Gait abnormalities increase submaximal walk- location of brain damage (left or right) [33]. Non-
ing energy expenditure almost 3-fold compared with verbal learning impairments, characterized by good
healthy children [24]. Diplegic children have a higher language abilities and week visual-spatial abilities
fat percentage and are hypoactive compared with with fear of new situations and stepwise develop-
healthy children [25]. Wheelchair-dependent adoles- ment, are common [34].
cents with CP are hypoactive, which is not the case for Behavior problems are five times more likely in
ambulatory adolescents with CP [26]. Physical, but children with CP (25.5%) compared with children
not mental, fatigue is more common in adults with CP, with no known health problem. The odds ratio for
than in the general population. The strongest pre- behavior problems of children with CP without
dictors for fatigue are bodily pain, deterioration of mental retardation is 4.9. The attention deficit
functional skills, limitations in emotional and physical hyperactivity disorder (ADHD) is more common
role function and life satisfaction [27]. among children with CP [35]. Other specific behavior
In people with hemiplegic CP the prevalence of problems in children with CP are dependency, being
additional impairments is 42% [28]. Common addi- headstrong, and hyperactivity in general [36].
tional impairments are cognitive impairments,
epilepsy, sensory, endocrine and urogenital impair-
Epilepsy
ments.
As can be seen in Table II, a large minority of
people with CP has epilepsy, and the prevalence
Cognitive impairments
varies with the type of motor impairment [37]. It is
As can be seen in Table II, a large proportion of most common among the hemi- and tetraplegics.
people with CP have some kind of cognitive Children with tetraplegic CP tend to have an earlier
186 E. Odding et al.

Table II. Prevalence of impairments among people with cerebral palsy.

Impairment % Subgroups % CP-subgroups %

Motor 100 Spasticity 72 – 91 Hemiplegic 21 – 40


Diplegic 13 – 25
Tetraplegic 20 – 43
Other 9 – 28 Dyskinetic 12 – 14
Ataxic 4 – 13
Cognitive (*) 23 – 44 Hemiplegic 60
With epilepsy 59 – 77
Without epilepsy 18 – 50
Severe (IQ 5 50) 30 – 41 Tetraplegic 100
Sensibility 44 – 51 Hemiplegic 90
Speach 42 – 81 Hemiplegic 30
Diplegic 20
Tetraplegic 85
Dyskinetic 95
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Severe 24
Visual (**) 62 – 71 Strabismus 50
Hemianopsy 15 – 25
Moderate 16
Severe 10 Tetraplegic 47
Hearing (#) 25 Moderate 1
Severe 2
Epilepsy 22 – 40 Hemiplegic 28 – 35
Diplegic 14
Tetraplegic 19 – 36
Dyskinetic 8 – 13
Ataxic 13 – 16
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Feeding Choking 56
Long feeding time 28
Nonorally 80
Gastrointestinal Constipation 59
Vomiting 22
Growth 23
Weight 52 Undernourished 30
Overweight 14
Obesity 8
Urinary 23.5 Hemiplegic 20
incontinence Diplegic 20
Tetraplegic 46

*Cognitive impairment: IQ570.


**Visual impairment: moderate = 6/18–6/60 D, severe = 56/60 D.
#
Hearing impairment: moderate = 45–70 dB loss, severe = 70 dB loss.

onset than children with other types of CP [38]. (astereognosia in 20%). Term children tend to be
Epilepsy is present in 79.5% of severely disabled more severely affected [41]. Sensory impairments are
children. [30]. Of the severely mentally disabled most common among hemiplegic CP people. Nine
tetraplegics, 94% have epilepsy [22]. All types of out of 10 hemiplegic children have significant bilateral
epilepsy occur; but generalized and partial epilepsy sensory deficits. Stereognosis and proprioception are
are the predominant types [39]. Children with the chief modalities affected bilaterally. The extent of
tetraplegic CP are more likely to have generalized sensory loss does not mirror the severity of the motor
epilepsy, and more than half of them require two or deficit [42]. Chronic pain is reported by 28% of the
more anti-epileptic drugs. In hemiplegic children adults with CP, versus 15% of the adults in the general
the predominant type is localization related epilepsy population. Back pain is the most common in all types
(83%) [38,40]. The frequency of seizures often of CP, with the lowest prevalence in persons with
decreases after age 16. hemiplegia. Foot and ankle pain is most prevalent in
persons with diplegia, pain in the knee in tetraplegia,
and neck and shoulder pain and headache in persons
Impairments in sensibility and senses
with dyskinesia. Poor physical role functioning, low
Stereognosis and two-point discrimination of the life satisfaction and deterioration of functional skills
hands is impaired in 44 – 51% of all children with CP are associated with chronic pain [43].
The epidemiology of cerebral palsy 187

Impairment of speech is common (Table II) and difficulty feeding the child, use of anticonvulsants
strongly associated with the type and severity of the and lower triceps skinfold scores are all indepen-
motor impairment (dyskinetic the most, tetraplegic dently associated with low BMD [55,56].
more than diplegic) [44]. The most common
impairment is dysarthria but aphasia occurs also.
Urogenital impairments
As a rule mentally retarded tetraplegic people do not
speak [22]. Almost a quarter of children and adolescents with CP
Ophthalmic abnormalities are present in 62% of have primary urinary incontinence. Tetraplegia and
CP children [5,21,45]. Low visual acuity is reported low intellectual capacity are the most important
in 71% of children with CP. Because ophthalmolo- determinants. At age six, 54% of the tetraplegic,
gical examination cannot explain the low visual and 80% of the hemiplegic and diplegic children gain
acuity of the vast majority, there is a high probability urinary continence spontaneously [57]. Among CP
of cerebral visual disturbance [46]. Among mentally children referred with daytime urinary incontinence
disabled tetraplegic children, 47% have severe visual at age 10 years, 85% have abnormal videourodynamic
impairments [22]. In prematurely born children (less patterns. Treatment leads to improvement in all [58].
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than 32 weeks) with CP there are significantly more


cases with cicatricial retinopathy, cortical visual
Radiological features
impairment and concomitant strabismus. In preterm
children the prevalence of refractive error without Sixty-eight percent of children with tetraplegic CP are
other ocular abnormalities is similar for children with microcephalic [22]. Almost 70% of children with
and without CP [47]. spastic CP have abnormal brain CT findings; this
Impairments in hearing do occur but less often group has a greater possibility of having develop-
than the other impairments; however, data are very mental delay [59]. MRI brain abnormalities can be
scarce [4,45]. classified into four groups: i.e. group 1: brain
malformations; group 2: cortical-subcortical lesions;
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group 3: abnormalities of the periventricular white


Endocrine impairments
matter; and group 4: postnatal brain-injuries. In
As can be seen in Table II, in children with CP groups 1 and 2 the severity of hemiplegia is mainly
gastrointestinal as well as feeding problems are moderate, while it is mild in groups 3 and 4. The third
common [48]. Sucking and swallowing problems in group presents a large involvement of the lower limb,
the first 12 months of life are common: 57 and 38%, while the upper limb is more affected in the other
respectively. Eighty percent have been fed non-orally groups. Mental retardation occurs in one-third of
on at least one occasion. In 60% of the children, children in groups 1 and 4, less often in the other two.
severe feeding problems even precede the diagnosis Seizures occur in half of the children in groups 1 or 2,
of CP [49]. Significant silent aspiration is found in while the incidence was lower in the other two [60].
68.2% of those with severe spastic CP [50,51]. In very low-birthweight children (500 – 1499 g)
Almost a third of all children with CP have suffered periventricular leukomalacia, and secondly, intraven-
at least one pulmonary infection in the previous 6 tricular hemorrhage are predictive of cerebral palsy
months [48]. There is an increased prevalence of and of functional outcome [61 – 63]. Among all CP
malocclusion in children with CP; they are likely to categories, abnormal cranial ultrasound is most
have a significant increased overjet [52]. Children strongly associated with hemiplegia, normal cranial
with CP who have permanent dentition have more ultrasounds with diplegia [64].
dental caries than healthy children with the same age In children with bilateral spastic CP hypoperfusion
and socioeconomic background [53]. in the thalamus or cerebellar hemispheres is found.
Linear growth in diplegic and hemiplegic CP Mildly decreased perfusion is associated with mild
children is often significantly reduced. More than delays in gross motor development, while almost all
half of children with CP have problems with their children with severe hypoperfusion show severe
weight, either under- or overweight, and almost a developmental delay [65].
quarter have stunted growth [54].
Bone-mineral density (BMD) in children and
Risk factors
adolescents with spastic CP varies greatly, but
averages nearly one standard deviation below the It has been long known that there is an association
age-matched normal means. Osteopenia of the femur with birthweight [66]. Table I gives some prevalence
is present in three-quarters of all children with data. The prevalence of CP among low-birthweight
moderate to severe CP and in almost all who are children is higher than among normal birthweight
unable to stand. Decrease in BMD of the lumbar children [3 – 5]. Children of 32 – 42 weeks gestation
spine is less pronounced. The severity of disability, with a birthweight for gestational age below the 10th
188 E. Odding et al.

percentile are 4 – 6 times more likely to have CP then obvious perinatally derived tetraplegic CP have a high
are children between the 25th and 75th percentile. In load of complications in the partum and postpartum
children with a weight above the 97th percentile, the period [21]. Reported perinatal risk factors for CP are:
increased risk is smaller (2–3), but still significant neonatal convulsions, birth asphyxia, instrument-
[67]. assisted delivery, neonatal jaundice, antepartum
Studies on possible risk factors for CP are hemorrhage, and neonatal infection [83]. The role
abundant. Most studies trying to investigate causes of asphyxia in the etiology of CP is somewhat
of CP conclude that there are many pathways, many controversial. Some report that only a minority of
of which cannot be identified yet. Each contributes cases with asphyxia are associated with an increased
only a small proportion and many may be multi- risk of CP, while others state that in almost one-third
factorial. Risk factors can be categorized as of the term CP children birth asphyxia is the likely
prenatally, perinatally and postnatally acquired. cause [84]. Low Apgar scores at 5, 10 and 20 min are
Among congenital hemiplegics, 42% are classified strongly associated with CP [85 – 87]. In low-birth-
as prenatal, 16% as perinatal, 9% as combined pre- weight in nonvertex presentations only, active labor is
and perinatal, and 34% as untraceable [28]. Of associated with CP [88]. The higher risk among term
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children with tetraplegic CP some 50 – 55% are breech presentation infants is more likely linked to a
prenatally, around 30% perinatally and about 15 – higher rate of being small for gestational age in breech
20% postnatally derived [21]. In preterm children, infants than to the mode of delivery [89]. In children
12% have a prenatal etiology, 61% a peri/postnatal, with hemiplegic CP there is no association with
and 27% cannot be classified. In term children these thrombophilia [90]. In very low-birthweight children
figures are 51, 36 and 14%, respectively [68]. there is no association with multiple and complicated
Among the prenatal risk factors, intrauterine pregnancy, caesarean section, severe asphyxia and
infection (chorioamnionitis) is the best known. In respiratory distress syndrome [91].
full-term infants the relative risk of intrauterine Multiple gestation is a risk factor for CP: the
infections for CP is 4.7 (95% CI 1.3 – 16.2) and in prevalence of CP is 2.3 per 1000 in singletons, 12.6
For personal use only.

preterm infants 1.9 (1.4 – 2.5) [69 – 71]. It is the most in twins and 44.8 in triplets. Among infants weighing
important prenatal factor in the pathogenesis of CP in 2500 g or more, there is a significantly higher risk in
very low-birthweight infants. The relative risk of CP multiple than in singleton births; among those with
increases approximately 4-fold in very low-birth- birthweight less than 2500 g there is no such
weight infants with a neonatal history of sepsis [72]. difference [92]. From 1971 to 1997, the incidence
Perinatal infection and other risk factors, such as of twin births increased with 53%, of triplets with
death of a co-twin, placental abruption, and cerebral more than 400% and of quadruplets with more than
ischemia, are thought to trigger a cytokine cascade 1100%. In the same period there was a vast increase
resulting in damage to the developing brain [73]. in the rate of CP per 1000 live births in triplets
Periventricular leukomalacia is one of the under- compared with singletons (26.6 vs. 1.6) [93]. Apart
lying disorders accounting for much of the CP from the risk of CP by multiple gestation alone, the
among children who were very preterm, and for live-birth co-twin of a fetus that died in utero is also at
perhaps a fraction of CP in infants born at term [74]. an increased risk of CP. The CP prevalence is 95 per
The risk of periventricular leukomalacia is higher 1000 in same-sex life births and 29 per 1000 in
with delivery at a very low gestational age, and different-sex births [94]. In the surviving low-birth-
maternal or placental infection [75,76]. There is an weight twin (1000 – 1999 g) whose co-twin has died
association between increased cytokine levels in postnatally the prevalence of CP is much higher
amniotic fluid or umbilical cord blood and risk of in same sex twins (167 per 1000) than in those of
preterm labor and periventricular leukomalacia different sex (21 per 1000) [95].
[77,78]. The cytokine interleukin-18 (IL-18) is Finally, term infants with encephalopathy with
undetectable in the cord blood of normal full-term other birth defects are three times more likely to have
infants, but is present in high levels in the cord blood CP than term infants with encephalopathy without
samples obtained from pre-term infants who neona- other birth defects [96].
tally developed periventricular leukomalacia followed
by CP [79].
Preeclampsia has been shown to be strongly Conclusion
protective against CP in preterm infants [80,81].
Prevalence
The low frequency of intrauterine infections in
mothers with preeclampsia might explain the appar- The prevalence of CP has risen in time from about
ent protective effect of this disorder. 1.5 per 1000 life births in the 1960s to about 2.5
Intrapartum initiation of the etiological pathway in the 1990s. With the rising incidence of CP
is likely in only 9% of the cases [82]. Children with there were fewer cases with diplegia and more with
The epidemiology of cerebral palsy 189

hemiplegia. The proportion of low-birthweight higher than in singletons. Given that low birthweight
infants among all children with CP is rising. and multiple gestation often coincide, and that
Newborns weighing less than 2500 g now contribute multiple births of more than two infants are
half of all cases of CP and just over half of the most predominantly caused by in vitro fertilization pro-
severe cases, whereas in the 1960 they contributed cedures, we think that the Dutch policy to implant a
one-third of all cases, and only one-sixth of the most maximum of two embryos per procedure should be
severe. There is a clear social class gradient in the promoted.
prevalence of CP.

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