european journal of paediatric neurology 14 (2010) 214–218

Official Journal of the European Paediatric Neurology Society

Original article

Cerebral palsy in eastern Denmark: Declining birth prevalence

but increasing numbers of unilateral cerebral palsy in birth
year period 1986–1998

Susanne Holst Ravn*, Esben Meulengracht Flachs, Peter Uldall

The Danish Cerebral Registry, National Institute of Public Health, Copenhagen, Denmark

article info abstract

Article history: The Cerebral Palsy Registry in eastern Denmark has been collecting cases using a uniform
Received 6 May 2008 data sampling procedure since birth year 1979. Children are included by two child
Received in revised form neurologists and an obstetrician. Information on pregnancy, birth, neonatal period,
3 February 2009 impairments and demographic data are registered.
Accepted 2 June 2009 The total cerebral palsy birth prevalence has been significantly decreasing since the birth
period 1983–1986 with 3.0 per 1000 live births until the period 1995–1998 with 2.1 per 1000
Keywords: live births. The overall decrease was seen in preterm infants (<31 weeks) as well as in term
Cerebral palsy infants and despite a simultaneous fall in perinatal and early neonatal mortality in the
Unilateral cerebral palsy preterm group.
Bilateral cerebral palsy Analysing the subtypes of CP we found a significant increase in the numbers as well as the
Hemiplegia rate of unilateral CP with a simultaneous fall in the numbers as well as the rate of bilateral
Diplegia CP. The explanation of this rise is not obvious. A change from bilateral periventricular
lesions to unilateral is a possibility, but no major change in the neonatal handling could be
documented.
Regarding associated impairments, developmental delay/learning disabilities as well as
motor function assessed by ability to walk (unassisted/assisted), both have changed toward
higher percentage of children with unassisted walking and in need of special education.
ª 2009 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights
reserved.

1. Introduction
Cerebral palsy is one of the most severe disabilities in child-
hood, making heavy demands on health, educational and
social services as well as on families and children themselves.
The Danish Cerebral Palsy Registry (DCPR) has existed
since 1967, collecting cases of cerebral palsy (CP) from birth
years 1925–1953. CP-rates have been recorded since 1940 but
birth prevalence is not presumed to be reliable until 1950.1
Until recently DCPR has only covered the eastern part of
Denmark (about 50% of the Danish population) but is now
a national registry with start of registration from birth year
1995. The DCPR is placed at the National Institute of Public
Health and has since 1997 been an active participant in the
Surveillance of Cerebral Palsy in Europe (SCPE).2
According to SCPE definition, cerebral palsy is ‘a group of
permanent but not unchanging disorders of movement
and/or posture and of motor function, which are due to

* Corresponding author. Ved Fortunen 25a, 2800 Lyngby, Denmark. Tel.: þ45 45888248; fax: þ45 45938248.
E-mail address: s.h.ravn@dadlnet.dk (S.H. Ravn).
1090-3798/$ – see front matter ª 2009 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.ejpn.2009.06.001
 european journal of paediatric neurology 14 (2010) 214–218
a non-progressive interference, lesion, or abnormality of
the developing/immature brain’. In practice this definition
has been used by the DCPR since birth year 1950. Cerebral
palsy is an umbrella term covering a wide range of cerebral
disorders, which result in childhood motor impairment and
a syndrome with a wide spectrum of causal pathways.
Possible risk factors are decreasing birth weight and
gestational age,3 chorioamnionitis,4,5 and inflammation
with activation of coagulation and the inflammatory
response,6–9 and in the neonatal period hypoxia, respiratory
and circulatory problems.10 The risk factors are different for
the different CP syndromes e.g. unilateral and bilateral
spastic, ataxic and dyskinetic cases.11
Neuropathogenic events affecting the developing brain
cause abnormalities or lesions, the patterns of which depend
on the stage of brain development, resulting in maldevelop-
ments in the first and second trimester and lesions in the third
trimester.12 The two lesions of cerebral white matter, that are
principally/especially involved, are the periventricular hae-
morrhagic infarction and the periventricular leucomalacia,
which might be diminished by prevention of impaired cere-
bral blood flow and cerebral vascular autoregulation and
inflammation.13
The DCPR has earlier reported the trend in CP-birth
prevalence up to 1990.1,14,15 In this epidemiologic survey we
report the cerebral palsy rate in relation to gestational age in
the birth years 1991–1998 in eastern Denmark compared to
the rate in preceding years including neonatal mortality, the
distribution in the clinical subgroups and possible changes in
the associated impairments/handicaps.
2. Methods
Since birth year 1979 a uniform data sampling procedure has
been used to identify the cerebral palsy cases in East Denmark.
Data are gathered either from specific contacts in the paedi-
atric departments who then refer when a diagnosis of CP is
suspected, or by a cross cheque examination with the Danish
National Patient Registry, using any type of CP-diagnosis,
making it possible to get data on every child in any department
anywhere in Denmark who has had a diagnosis of CP.
The full case report including obstetric data is gathered and
the diagnosis is evaluated by the same two neuro-
paediatricians (PU, SHR) before inclusion or inclusion when
the child is 5–6 years old. Afterwards a data abstraction form
(specific information on pregnancy, birth, neonatal period,
impairments and demographic data) is filled in for every
included child and later the form is transferred for statistical
analyses. If needed supplementary demographic and obstetric
data are collected from the Danish Medical Birth Registry.
A validation of the registry was performed in 1997 to
determine the completeness of the DCPR.14 The validation of
the data was done by a linkage between the National Patient
Registry (identifying the diagnosis of CP) and the cases in the
Danish Cerebral Palsy Registry diagnosis. The DCPR was
assessed to be 85% complete for the birth years 1979–1982, and
the National Patient registry has therefore been used as
a supplementary source of case ascertainment from the birth
year 1979.16
215
Inclusion criteria for the CP register (in the described
periods) are as follows:
- Born in (eastern) Denmark and still living at age 4–5 years. If
the child dies between age 1 and 4–5 years, it is included
anyway provided the CP-diagnosis is unquestionable.
- Pre- or perinatal aetiology (before 28th day of life).
- Fulfilment of diagnostic criteria according to SCPE.2
Excluded from the register are progressive disorders of motor
function (defined as loss of previously acquired skills in the first
5 years of life), spinal diseases and disorders of short duration.
Parameters used to characterise the degree of handicap for
the CP group are developmental quotient, motor function and
whether the child is suffering of epilepsy.
The developmental quotient, which is mainly due to clin-
ical assessment upon the medical records, is divided into
normal for children without learning disabilities and expected
to start normal school, and abnormal for children with
moderate learning disabilities and need of special education/
attending special schools (including children with a severe
mental handicap).
Motor function is divided into normal reflecting unassisted
walking and for children from birth year 1997, where use of
the Gross Motor Function Classification System (GMFCS)
started, as in the level 1. All others are classified as having
abnormal function.
Registration of epilepsy is done according to whether the
children have or has had epilepsy after the neonatal period
(e.g. after the 28th day of birth), regardless of ongoing medi-
cation or not.
3. Results
Overall from 1983 to 1998 a significant decline in the birth
prevalence of cerebral palsy was seen ( p < 0.001) with a prev-
alence of 2.1 per 1000 live births (Fig. 1). Concerning preterms
the decline was seen during all three 4-year periods, whereas
a slight increase was seen in the term group of CP children in
the last period (Table 1).
This decrease in CP-rate is seen despite an overall stable
neo- and perinatal mortality (reflecting a decline in mortality
regarding preterms and a rather stable mortality among terms
30
Total
25
Term
Preterm
20
15
10
5
0
1983-86 1987-90 1991-94 1995-98
Fig. 1 – CP-rates according to GA in eastern Denmark.
216 european journal of paediatric neurology 14 (2010) 214–218

Table 1 – CP-rates per 1000 live births according to Unilateral

gestational age in eastern Denmark. Bilateral
Total
1983– 1987– 1991– 1995–
1986 1990 1994 1998 3,5
3
<32 weeks 112.4 79.8 56.7 47.9
32–36 weeks 14.0 8.7 7.9 6.2 2,5
Total  36 weeks 29.1 19.5 15.6 13.0 2
Total 37þ 1.5 1.5 1.1 1.4 1,5
Total 3.0 2.4 2.0 2.1 1
0,5

except for a rise in the last 4-year period) and a continuing rise
in the overall birth rate (Table 2).
As seen in Fig. 2 and Table 3 changes of the total CP-rate
reflect different development in the birth prevalence of
unilateral and bilateral spastic CP children. A significant
increase in unilateral term and preterm CP children (Fig. 3) and
a significant decrease of bilateral CP, especially in the preterm
group (Fig. 4), were seen over the four 4-year periods (Table 4a
and b). The proportions of dystonic, choreatic and ataxic
children did not show any significant changes (Table 4a and b).
In order to analyze the increased birth prevalence of
unilateral CP, we compared the unilateral cases from the birth
year period 1983–1990 with the cases from the period
1991–1998. No significant changes were found concerning
birth, perinatal events (Apgar scores, neonatal seizures and
neurological symptoms, use of CPAP or mechanical ventila-
tion) (figures not shown) or associated impairments (Table 5a
and b). The increased numbers of unilateral cases were seen
equally distributed over the different paediatric departments
of eastern Denmark. The classification from the case notes
has been done by the same person over the four periods (PU),
though in the last period with supplement of another neuro-
paediatrician (SHR). The same percentage was classified as
unilateral by the two neuropaediatricians in the latter period.
The associated impairments did not show any changes
except for a tendency to more children being able to walk
unassisted (Table 5a and b).
4. Discussion
An important observation is the trend with a significantly
falling CP-rate over the last 16 years. The fall is continuous for
the preterms despite a falling perinatal mortality in eastern
0
1983-86 1987-90 1991-94 1995-98
Fig. 2 – CP-rates according to subtype in eastern Denmark.
Denmark. This trend is seen at other centres in Europe as
well.3,17 Improved perinatal care is thought to be an important
factor for this fall.
Specific causative factors are difficult to pinpoint even
though our previous study suggested that the decline in use of
mechanical ventilation could be one of the factors.15 Surfac-
tant was introduced in Denmark in 1989 and used in 19% of the
preterm born CP children in the birth years 1991–1994 and 27%
in the birth years 1995–1998 (no use in term babies) resulting in
an expected diminished need of ventilatory assistance. This
could, however, not be found in our CP-population. The rise in
term CP children during the last period is difficult to explain
but has been observed in Sweden as well.17,18 Whatever the
reason is, it is important that the overall CP-birth prevalence is
falling, especial since the numbers of premature births in
Denmark seem to be steadily increasing (Table 2). The rate of
epilepsy and inability to walk in our registry are rather similar
to a recent Norwegian study.19
The most surprising observation is the increasing numbers
of unilateral CP cases in proportions as well as absolute
numbers. These marked observations have not been reported
by any other registers. There might to be a tendency in the
Swedish register in the same birth period when examining the
figures in the latest trend article from Sweden.17
The cause(s) of the changes in subgroups are not obvious
but the decline in bilaterals, which is most pronounced in the
preterm group, is best explained by a better perinatal care,
especially by avoiding inflammation and impairment of as
well cerebral blood flow as cerebral vascular autoregulation.

Table 2 – Stillbirths and mortality in Denmark 1883–1998.

1983–1986 1987–1990 1991–1994 1995–1998

Stillbirth (28–36 week) 40.5 41.1 37.3 36.8

Neonatal mortality 36 weeks 41.6 41.4 32.2 28.7

37 weeksþ 1.4 1.4 1.2 1.3
Total 3.6 3.5 3.0 2.9

Perinatal mortality 36 weeks 80.5 84.5 68.4 64.4

37 weeksþ 3.3 3.8 3.3 4.1
Total 7.7 8.2 7.2 7.8

Liveborns 36 weeks 5844 6552 8085 8614

Total 107,319 123,584 140,531 143,962
 european journal of paediatric neurology 14 (2010) 214–218 217

Table 3 – Unilateral and bilateral CP-rates (preterm and Table 4 – Proportions and numbers of CP-children in the
term babies) in eastern Denmark. clinical subgroups, divided into preterm (a) and term (b)
1983–1986 1987–1990 1991–1994 1995–1998 infants in eastern Denmark.
Table 4a Preterm % 1983– 1987– 1991– 1995– p-value
Bilateral rate 2.03 1.59 1.05 0.96
(numbers) 1986 1990 1994 1998 for trend
Unilateral rate 0.53 0.49 0.63 0.87
Bilateral spastic 74(126) 77(99) 63(79) 54(60)
Unilateral spastic 12(21) 13(17) 25(31) 38(43) 0.001
Dystonic 11(18) 9.4(12) 8.7(11) 4.5(5)
Choreatic 5(3) 0 3(4) 0
Regarding the increase in unilateral CP, in the term as well
Ataxic 0 0 0.8(1) 3.6(4)
as the preterm group, one simple explanation is probably not
to be found. Unilateral CP is often due to vascular events. Table 4b Term % 1983– 1987– 1991– 1995– p-value for
(numbers) 1986 1990 1994 1998 trend
Perinatal strokes, defined as a cerebrovascular event that
occur between 28 weeks of gestation and 28 days of postnatal Bilateral spastic 60(92) 57(98) 44(66) 41(78)
age, are recognized in approximately 1 per 4000 live births per Unilateral spastic 23(36) 25(43) 38(57) 44(82) 0.001
year,20 but the causal pathways are not understood. In case Dystonic 13(20) 8(13) 7(11) 10(18)
Choreatic 2(3) 5(9) 7(11) 2(4)
series trauma and alloimmune thrombocytopenia are the
Ataxic 1(2) 3.5(6) 3.3(5) 3.2(6)
major recognized causes of antenatal strokes, but more than
50% do not have any recognized risk factors.21 Nearer to term
embolic or trombotic causes appear important, maybe due to
the genetically determined protrombotic factors leading to an
increased risk.22–24 Furthermore synergistic factors may very
140 well play an important role: preeclampsia, intrauterine
growth retardation, placental infarction, maternal dehydra-
Total
120 tion and stress during labour.25,26 An American Japanese
Term group has proposed clinically silent periventricular venous
100 Preterm infarction in uteri as a cause of hemiplegia in term babies.27 It
is, however, unlikely to assume that trauma, alloimmune
Numbers

80 trombocytopenia or genetic factors should have determined

the rise in unilateral cases of CP.
60

40
Table 5 – Associated impairments (developmental
quotient DQ, motor function and epilepsy) in preterm (a)
20
and term (b) in % in eastern Denmark.
Table 5a Preterm 1983– 1987– 1991– 1995–
0
(%) 1986 1990 1994 1998
1983-86 1987-90 1991-94 1995-98
DQ Normal 44 42 41 41
Fig. 3 – Unilateral CP according to GA in eastern Denmark. Subnormal 56 58 59 59

Motor Walks 48 50 56 63
function alone
Assisted 52 50 44 37
250 walk
Total or
immobile
Term
200
Epilepsy No 77 73 74 80
Preterm
Yes 23 27 25 20

150 Table 5b Term 1983– 1987– 1991– 1995–

Numbers

(%) 1986 1990 1994 1998

DQ Normal 44 37 31 38
100 Subnormal 56 63 69 62

Motor Walks 56 56 60 61
function alone
50 Assisted 44 44 40 39
walk
or
0 immobile
1983-86 1987-90 1991-94 1995-98 Epilepsy No 68 66 68 80
Yes 32 34 32 20
Fig. 4 – Bilateral CP according to GA in eastern Denmark.
218 european journal of paediatric neurology 14 (2010) 214–218
In a review of MRI of CP cases by Krägeloh-Mann28 20% of
term and 71% of preterm cases of unilateral CP were found to
be due to periventricular lesions. Another study found similar
figures.29 One explanation of the increase in unilateral CP
might be that children, who earlier developed a bilateral per-
iventricular leucomalacia, now is recognized as unilateral as
a result of a minor cerebral lesion due to a better neonatal
care. Unfortunately we are not able to confirm this hypothesis
since very few children in our register had an MRI. An alter-
ation in the classification diplegia–hemiplegia (Hagberg et al.,
2001) might also explain the change but in our registry the
process concerning registration and classification has not
changed over the four periods.
Another more simple explanation could be a better ascer-
tainment, meaning that those with a very discrete unilateral
CP now are registered in the Danish National Patient Registry
with the correct diagnose. A less severe grade of impairments
among hemiplegia would then be expected. This was,
however, not the case in our population.
5. Conclusion
The overall CP-rate in the birth year period 1986–1998 has
declined to a level similar to other European countries. A rise
in unilateral CP is seen with no obvious explanation to be
found. A change from bilateral periventricular lesions to
unilateral might play a role. Other more simple explanations
could not be ruled out. Clarification will have to await the
trends in other CP-registries as well as a better follow up with
MRI on these children.
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