BILATERAL SPASTIC CEREBRAL PALSY-A
COMPARATIVE STUDY BETWEEN SOUTH-
WEST GERMANY AND WESTEPN SWEDEN.
1 1 : EPIDEMIOLOGY
~ : & : y & , , + @ : - ~ $ , ~ ~ . +&i:.$Ty";.::z
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~. . -.
Ingeborg Krageloh-Manri Kariri Edehol Eeg-Olofssoti
Girdrun Hagberg l h n s h'onrud SelDrnann
Ciiris!oph Meisner Beng! Hugberg
Birgi! Schelp Richard Miciiuelis
Gerhard Haas
There have been many reports from
around the world of advances in perinatal
care, resulting in a progressive decrease in
perinatal mortality rates. During the
1970s and early 1980s, however, there has
been a parallel increase in the prevalence
of cerebral palsy (CP) among low-
birthweight (I.wv)-children, especially
those of very low birthweight-whereas
morbidity trends in children of normal
birthweight have been inconsistent
(Hagberg ef a/. 1984, 1989a, 1993;
Pharoah er al. 1987, 1990; Dowding and
Barry 1988; Finne el al. 1988; Stanley and
Watson 1988, 1992; Riikonen et al. 1989;
Takeshita e f al. 1989).
Changes in the prevalence of CP may
give new clues about aetiology. Among
normal-birthweight children with CP,
there is general agreement that around 80
per cent of cases originate from the period
before birth, while around 10 to 20 per
cent originate from the perinatal or
neonatal period (Blair and Stanley 1988;
Ellenberg and Nelson 1988; Hagberg et al.
19896, 1993). Among L B w children,
however, the timing of brain-damaging
events is controversial (Pharoah ef al.
1990, Stanley and Watson 1992, Hagberg
and Hagberg 1993). Is the neurological
deficit caused by prenatal brain-damage
or genetically determined maldevelop-
ment, and does the reduced mortality now
allow more children with CP to survive,
''3 y'#"?*:.. '.:&g-:+
so that the prevalence (though not the
incidence) of CP is affected? Or are
immature infants, born with intact but
vulnerable brains, liable to a variety of
perinatal insults? The last implies that
progress in clinical knowledge of
SpeciaVintensive care may lead to a
decrease in the numbers of affected
children.
Although C P is the second most
common neuro-impairment in childhood M
(after mental retardation), with a pre- m
Tr
valence of 2 to 2 . 5 per 1000, statistically M
r-
7
significant changes over time will only be
seen in large, long-term studies. Even
among LBW children, w h o have a
considerably higher risk of CP (Stanley and
Alberman 1984, Takeshita et al. 1989,
Hagberg el al. 19896), the combination of
LBW and CP accounts for no more than
around 0.7 per 1000 total live births.
As bilateral spastic CP (BSCP) is the
most common form of the CP syndromes
and has also shqwn the most significant
changes in prevalence during recent
decades (Hagberg and Hagberg 1989), we
undertook a comparative study of BSCP
between two geographic regions, south-
west Germany and western Sweden. This
paper is the second report from that
collaborative study; the first showed that
the two series were comparable and could
profitably be used for a collaborative
study (Krageloh-Mann el al. 1993). The 4 73
 aim of this second paper was to compare
and analyse prevalence rates.
The level of perinatal care was con-
sidered to be the same in the two regions.
Thus we hypothesized that birthweight-
specific prevalence should also be similar,
in which case we would be entitled to pool
the t w o series to obtain a larger sample
with more statistical power.
Definitions and method
CP was defined as ‘a disorder of move-
ment and posture due to a defect o r lesion
of the immature brain’ (Bax 1964). RSCP
was defined as spasticity of the extremities
on both sides, with flexor hypertonicity,
increased tendon reflexes and charac-
teristic posture (hip adduction and
c
internal rotation, with equinus of the feet
d
L
0 or its secondary malposition). We used
u)
u the following clinical subtypes of BSCP
3
U (Michaelis and Hege 1982, Michaelis and
C
CJ Edebol-Tysk 1989): ‘leg dominated’ if,
m
C.
C according to functional grading, legs were
E more affected than arms; ‘three-limb
G dominated’ if one arm and both legs were
affected equally and more than the other
arm; ‘four-limb dominated’ if arms were
equally or more affected than legs;
and ‘dyskinetic-spastic’ if lower-limb
c
spasticity was present in the legs, but
L
;
u dyskinetic posturing was the dominant
n
h
feature. ‘Very low birthweight’ ( V L R W )
-
U
3
cn
was defined as a birthweight of < ISOOg,
and ‘moderately low birthweight’ (MI.BW)
-0x
u)
as between 1500 and 24993. ‘Neonatal’
n. was defined as referring to the first four
weeks of life.
The study period covered the birth
years 1975 to 1986. The study area in
Germany was the district of Tubingen, a
rural area in the south-west of Germany,
with 204,434 live births during the study
period. The area is covered by two
university hospitals and three local
hospitals, all with neonatology units. The
study area in Sweden was the western
Swedish health-care region, with 229,853
live births during the study period. The
area is covered by one regional university
hospital and seven county hospitals with
neonatology units.
The search procedures included mul-
tiple sources: schools and institutions for
those with motor disabilities and mental
4 74 retardation, and diagnostic registers of
hospitals and units in charge of children
with CP. In Germany, children were
looked for first in schools and institutions
for those with motor disabilities and
mental retardation, and then in the
medical records of three hospitals in the
area (including the University Hospital of
Tubingen). The families were contacted
through teachers or family doctors,
because of the law regarding data
protection and medical confidentiality. In
Sweden all children with abnormal motor
development ( I to 2 per 300,000 inhabi-
tants) are referred to special habilitation
centres. Local and regional habilitation
registers and diagnostic hospital registers
were the main sources of case findings.
Data on vital statistics were kindly
provided by the regional office for popu-
lation statistics (Statist isches Landesamt
Baden Wiirttemberg) in south-west
Germany, and the medical birth
registration office in Sweden (Statistics
Sweden).
Statistical methods
Conventional xz tests and Armitage’s
(1974) ‘trend in proportion’ xz test were
used for statistical calculations. 95 per
cent confidence intervals (binomial
approximation) were calculated according
to the methods of Boyle and Parkin
(1991). All the statistics and p values
have been interpreted in a descriptive
manner.
Results
Comparison of vital statisrics
The German newborn population had a
higher LBW rate: 0 . 7 7 per cent were VLBW
and 4.35 per cent were MLBW, compared
with 0.56 per cent and 3 . 5 8 per cent,
respectively, in the Swedish newborn
population (P<C)*OOl). I n both series,
neonatal mortality was steadily decreasing
(Fig. 1); it was generally lower in Sweden
than in Germany, but the difference was
decreasing. Neonatal mortality was lower
in south-west Germany, than in western
Sweden during the last birth-year period
(1983-86), among both m B W and
normal-birthweight infants.
Comparisoti of BSCP rates
We found a total of 249 children with
BSCP in south-west Germany and 264 in
western Sweden (Tables Ia and b, Figs. 2 1000,
Neonala' modal ly m
m
ct
and 3). The prevalences per live births or M
t-
per neonatal survivors did not differ CI

significantly between the two series. This 2

was rrue both for crude prevalences and e-
rn
for birthweight-specific ones, and when 0
Q'
subgrouped into three-year birth cohorts.
With a few exceptions, both series had a 13i
peak prevalence, calculated per live births .753cg

or per neonatal survivors, in the birth- 11- c -7-71

year period 1978-80 ,and valid for the 75 77 78 ao a1 83 a4 a6
crude prevalence as well as for the Blnh year per od
different birthweight groups. Exceptions Fig. 1. Neonarol tnorralrry per 1000 live birihs in
were the Swedish MLBW group and the souih- wesi Cernrany (open circles) and u'estern
German normal-birthweight group, both Sweden (closed circles) by birihwerghi groups ond
birth-year periods.
of which showed a peak in the 1981-83
birth cohort (although changes in the
group of normal-birthweight children
were very small).
The risk of BSCP increased sharply with
decreasing birthweight (Tables l a and b).
Liveborn VLBW children had an 86-fold
higher risk in the German series, and a
68-fold higher risk in the Swedish series,
than those of normal birthweight; and
VLBW survivors had a 147-fold and a
100-fold higher risk, respectively. The
corresponding figures in the ML.BW group I
were 24 and 18, respectively, for liveborn 01 - * -
7- 1

75 77 713 ao 81 a3 M a6 75 7 7 78 80 a1 a3
1,. - T
a4
l
a6
infants, and 25 and 19, respectively, for B nh year per od
survivors. Fig. 2. Crude prevalence of BSCP per 1000 live
births (left) and neonatal survoors (righr) i n
Secular trends in the pooled series south-west Germany (open circles). wesrern
Sweden (closed ctrcles) and pooled series (solid
The pooled crude prevalence of BSCP bar)
showed a marked increase between the
two birth-year periods 1975-77 and
1978-80 (p = 0-001 for livebirths and
neonatal survivors), and thereafter a
slightly dccreasing trend for rates based
both on livebirths and neonatal survivors
LNC births h'wnatal s u r v w m
@ = 0.07) (Table 11, Figs. 2 and 3). When
analysed with respect to birthweight
groups the initial increase was found in all
groups, being most important in the
V m W group, when calculated both per
livebirths (p = 0.002) and per neonatal
survivors (p =0.014). The subsequent
decrease in BSCP prevalence applied
mainly to the LBW groups, and was most
significant in the M L B W group, both per
live births @ = 0.033) and per neonatal
31--
75 7;
- r-- 1 J ' 7-- 1
78 80 a1 a3 ad 86 75 77 78 ao a1 03 go 86
Ridh year period
survivors @=0-027), and in the VLBW
group for neonatal survivors @ = 0.046). Fig. 3. Birth weight-specific prevalence o j BSCP
per 1000 live births (left) and neonatal survivors
The normar-birthweight group showed (right) in south-west Germany (open circles),
only minor changes in BSCP prevalence western Sweden (closed circles) and pooled series
after the initial increase. (solid bar). 4 7s
i
TAB1.F la
-. Proalence of BSCP per lire births and per neonatal surtitors b j birthweight groups and birth-)ear period5 in
south-west German)

1 l i e births h'eonatol Sur~ivol BSCP BSCf' 9 5 % CI BSC? 9so-0 ('I

sitrvivors rate raw rate'
(per 1000)
~ ____
< lS00g
P I915-77 392 166 423.5 7 17.0 4.7-31 . O 12.2 I I .6-72.7
0 1978-80 383 206 537.9 23 60.1 36.3-83 .8 111.7 68.6-1 5 4 .
3Qa 1981-83 423 272 643.0 I2 2X.4 12.5-44.2 44. I 19.7-68.5
1984-86 376 277 736.7 20 53.2 30.5--75.9 72.2 4 I .7- 102.
I
1975-86 I574 92 I 585. I 62 39.4 29.8 49.0 67.3 5 1 . 1 83.5

1500-2499~
1975-77 2358 2230 945.7 24 10.2 6.1-14.2 10.8 6.5- 1 5 . 0
1978-80 2132 205 1 962.0 33 15.5 10.2-20.7 16.1 10'6-21 ' 5
1981-83 2 I49 2104 979. I 25 I I .6 7.1-16.2 11.9 7.3- 16.5
1984-86 2258 2219 982.7 IS 6.6 3.3-10.0 6.R 3.4- 10.2
1975-86 R897 R60J 967. I 97 10.9 8.7-1 3 . I 11.3 9.0-13.5

C z 250U.g
u 1975-77 47.452 47.324 997.3 20 0'4 0.2 0 . 6 0.4 0 . 2 0.6
B
v)
1978-80
1981-83
47.181
50,254
47.065
50.161
997.5
998.2
999.0
21
29
20
0.4
0.6
0.3-0.6
0.4-0.8
0.2-0.6
0.4
0.6
0.3-0.6
0.4-0.8
1983-86 49.076 49,029 0.4 0.4 0.2-0.6
1975-86 193.963 193.579 998.0 90 0.5 0.4-0.6 0.5 0.4-0.6
Totol
U
I975--77 50,202 . 49,720 990.4 51 I .0 0.7-1.3 I .o 0.7-1.3
1978-80 49.696 49,322 992'5 17 1.5 I .2-I . 9 I .6 I .2-I .9
0 0.9-1.6
I98 1-83 52,826 52.537 994.5 66 1.2 I .3 I .O-I . 6
h
C 1984-86 51,710 51.525 996.4 55 1.1 0.8-I . 3 1.1 0.8-1.3
0
E 197546 204.434 203.104 993.5 249 I .z 1.1-1.4 1.2 1.1-1.4
L

8
'Per 1000 live births.
'Per 1000 neonatal survivors.

TABLE Ib
C Preralence of BSCP per lire births and per neonatal survivors by birthHeight groups snd birth-year periods in
u
0 western SHeden
-3u
D I ibe births Neonoral Siirvrval BSCP BSCf' 95% CI BSCP 9STo CI
x wrvibors rate rate rare'
m
(per I 000)
rii --
-
h
VI
0
< fS0Og
1915-77 317 I72 54 2 . 6 9 28.4 10.1-46.7 52.3 19.0-85.6
3 1978-80 309 I99 644.0 15 48.5 24.6-72.5 75.4 38.7-112.1
1981-83 34 I 257 753.7 16 46.9 24.5-69.4 62.3 32.7-91 ' 8
1984-Rh 319 250 783.7 II 34.5 14.5 54.5 44.0 18.6-69.4
1975-86 I 2R6 878 682.7 51 39.7 29.0-50.3 SR. I 42.6-73.6
.-'a 1500-2499.g
d
vi 1975-77 2102 201 1 956.7 18 8.6 4.6- 12.5 9.0 4.8-1 3. I
0
P 1978-80 I974 1913 969. I 21 10.6 6. I - 1 5 . 2 11.0 6'3-1 5 '6
v) 1981-R3 1991 1952 980.4 25 12.6 7.7 -17.4 12.8 7.8-17.8
1984- 86 2173 2131 980.7 22 10. I 5.9- 14.3 10.3 6.0- 14.6
-
L
u
--
m
1975-86 8240 8007 971.7 86 10.4 8.2- 12.6 10.7 8 . 5 - 13.0
m 2 25 00s
1975-77 56.714 56,605 998. I 25 0.4 0-3-0.6 0.4 0 3-0.6
1978-80 54,064 53,978 998.4 37 0.7 0.5-0.9 0.7 0 . 5 -0.9
1981-83 53.106 53.017 998.3 27 0.5 0.3-0.7 0.5 0.3-0.7
1984-86 56.443 56.377 998.8 38 0.7 0 . 5-0,9 0.7 0.5-0.9
1975-86 220.327 219.977 998.4 127 0.6 0.5-0.7 0.6 0.5-0.7
Toral
1975-77 59.133 58,788 994.2 52 0.9 0.6-1.1 0.9 0.6- 1 . I
1978-80 56.347 56.090 995.4 73 1.3 1.0-1.6 1.3 1.0-1.6
1981-83 55.438 55.226 996.2 68 1.2 0.9-1.5 1.2 0.9-1.5
1984-86 58.935 58.758 997.0 71 1.2 0.9- I ' 5 1.2 0.9-1.5
1975-86 229.853 228,862 995.7 264 1.1 1-0-1.3 1.1 I .O-I . 3

'Per lo00 live births.

4 76 'Per 1000 neonatal survivors.
 0
r m L E II 01
cl
Pooled prevalence of B S C P b! birthneinht group and birth-Jear period in m
south-nest German) and western Sweden r-
7

a-
h
BSCP BSC? 9S% ('I BSCP 95% CI
7-
rote role- 01
-~-- --- Q'
< 1500~
1975-77 16 22.57 1 I .63-33'50 47.34 24.10-69.98
1978-80 38 54.91 37.94-71.89 93.83 65'43-122.23
I981- x3 28 36.65 23.33-49.97 52.93 33.85-72.01
1984-86 31 44.60
1975 -86 I13 39.51
29-26-59.95
32.37-46-65
58.82
62.81
38.73-78'91
51 -60-74.02
2-
5
1500-249Y~ p
1975-77 42 9.42 6.58-12-25 9.90 6.92-12.88 D
1978-80 54 13.15 9.67-16.64 13.62 lo~o1-17.23
1981-83
1984-86
50
37
12.05
8.35
8.75-15 '40 12.33
5'67-11.03 8 . 5 1
n .93-I 5.72
5.78-11.23
z
3
b

1975-86 I 83 10.68 9.14-12.22 11-02 9'43-12'60 .

2
Q
z
2 2SOOg
1975-77 45 0.43 0'31-0.56 0.43 0.31-0.56
$
1978-80
1981-83
58
56
0.57
0.54
0'43-0.72
0.40-0.68
0.57
0.54
0.43-0.72
0.40-0.68
s2
.L

1984-86 58 0.55 0.4 1-0.69 0.55 0 4 1-0.69 b

Q
1975-86 217 0.52 0.45-0.59 0.52 0.45-0.59
Total
1975-77 103 0.94 0'76-1.12 0.95 0.77-1.13
1978 -80 I50 1.41 1.19-1.64. 1.42 1.20-1.65
I981-83 I34 I *24 1.03-1'45 I .24 1.03-1'45
1984-86 126 1.14 0.94-1.34 1.14 0.94-1.34
1975-86 513 1.18 1.08-1.28 1.19 1.08-1.29

'Per lo00 live births.

'Per 1000 neonatal survivors.

The trends in prevalence for neonatal leg-dominated BSCP. When comparing

survivors within the different subtypes are
shown in Table 111. Although the two
series were pooled, the numbers were still
so small that the clinical subtypes had to
be limited to two groups: those with
kg-dominated BSCP, and others. Cor-
respondingIy, the birthweight groups were
limited to children with LBW and with
normal birthweight. In LBW children with
leg-dominated BSCP, there was an
increase between the periods 1975-77
and 1978-80 @=0*025), but then this
changed to a slightly decreasing trend
@=0.085). In 1.BW infants with other
forms of BSCP, there was an increasing
trend up to the period 1981-83 @ =
0.01 I ) , and thereafter a decreasing trend
@=0*007). The trends for the normal-
birthweight groups were less conclusive,
although we found a similar pattern with
a peak prevalence in the birth-year period
1978-80 among sub-types other than
the German and Swedish series, very
similar patterns of trends in the LBW
groups were found (Fig. 4). Among
children with normal birthweight,
however, the pattern between the two
countries was more inconsistent, the
Swedish series having a higher prevalence
of leg-dominated BSCP than the German
series @=0-019).
Control of recruitment
As in the German series, recruitment
facilities were not entirely similar
throughout the region of investigation.
The area with best recruitment facilities
(the catchment area of the University
Hospital of Tubingen, covering about 30
per cent of live births) was compared with
the remaining area with regard to crude
prevalence and birthweight-specific pre-
valences. Crude live-birt h prevalence for
the catchment area was 1.76 per 1000, 4 77
TABLE I l l
Pooled precafence per neonatal surtivors of BSCP by birthweight group, birth-year period and
clinical subope in Touth-west Germany and Hestern Sweden

Neonaral 1.eg-dominurerl BSCP Orher subtypes of BSCP

survivors - ~
- _- -. _-
N Rare per 95% CI N Rare per 95% CI
1000 I000

< 25001:
1975-77 457Y 46 10.05 7.16-12.93 I2 2.62 I . 14-4.10
1978-80 4369 67 1.34 11.69-18.98 25 5.72 3-49-7.96
1980-83 4585 49 10.69 7-71-13*66 29 6-32 4.03-8.62
1984-R6 4877 55 11.28 8.31-14'24 13 2.67 1.22-4. I I
Total IX.JI0 217 11.79 10.23-13.35 79 4.29 3.35-5.24

2 2500g
1975-77 103,929 23 0.22 0.13-0.31 22 0.21 0.12-0.30
1978-80 101.013 24 0.24 0.14-0.33 34 0.34 0.22-0'45
1980-83 103,178 29 0.28 0-18-0.38 30 0.29 0.19-0.39
1984-86 105.406 31 0.29 0-19-0.40 24 0.23 0.14-0'32
Iota1 413.556 107 0.26 0.21-0.31 110 0.27 0.22-0.32

Leg dorrinaled Orper than leg dom naiea
1 period (52 per cent compared with 39 per
O'-r----r 70 1-- .-
75 i 7 78 83 81 83 84 86 15 77 78 80 8' 83 84 86
aim year per od
Fig. 4. Prevalence of BSCP per neonalal survivors
in soitrh-wesr Germony (open circles), western
Sweden (closed circles) and pooled series (solid
bar) by cliniral strbiype, brrfh~ceighrgroup and
Dirlli-year period
and for the remaining area 0.95 per 1000
(crude prevalence for the total area
being 1.22 per 1000). Birthweight-specific
prevalences were 66.5 and 26.6 per 1000,
respectively, for VLBW children (total
area 39-4 per 1000); 16.4 and 7 - 9 per
1000 for MLBW children (total area 10.9
per 1000); and 0.57 and 0.41 per 1000 for
normal-birthweight children (total area
0-46 per 1000). These differences most
probably reflect the different recruitment
facilities. For VLBW children, whose
higher prevalence in the catchment area
was the most remarkable, a higher
survival rate in the catchment area-
especially at the beginning of the study
cent)-explains part of the difference.
However, the different recruitment
facilities did not influence the distribution
of disabilities in the different sub-areas
(Krageloh-Mann ei a/. 1993), nor did trends
over time concerning crude prevalence and
birthweight-specific prevalences in the two
1
sub-areas differ from trends in the total
region as presented above. In the
catchment area, rates were as follows (per
neonatal sqvivors): for VLBW children, a
prevalence increase followed by a decrease
during the four three-year periods was
found, with rates of 37,232.1, 69 and 93-3
per 1000 neonatal survivors; while for
MLBW children, the decreasing trend was
already seen from the beginning, the rates
being 20.8, 16.5, 18.4 and 12.6 per 1000
neonatal survivors; and for children with
normal birthweight, a slight increase and
decrease was found, the rates being 1.4,
2.4, 1.8 and 1.5 per 1000 neonatal
survivors. These findings fed us to believe
that the data of the total area were reliable
in the description of prevalence trends.
Discussion
Comparative prevalence studies of CP
between countries are desirable, and
should .allow us to approach potential
aetiologies more systematically. However,
various factors must be considered before
constructive analyses and comparisons
can be made.
First, demographic factors are im-
portant. Differences in the proportion
of 1 . r ~ infants between populations
constitute one such confounding factor.
A higher rate of LFWwill itself increase
the crude CP prevalence, since the risk of
CP increases sharply with decreasing
birthweight (Hagberg ef al. 1984, 1989b;
Stanley and Alberman 1984; Pharoah er
a/. 1987, 1990; Dowding and Barry 1988;
Finne ef a/. 1988; Riikonen ef a/. 1989;
Takeshita et a/. 1989; Stanley and Watson
1992). In this study, the pooled risk for
BSCP among Vl.BM' survivors was
121-fold, and among %lLB\\' survivors
76-fold, higher than among children of
normal birthweight. Throughout the
study period, south-west Germany had a
higher proportion 'of I HM' births than
western Sweden. We allowed for this
difference by calculating birthweight-
specific prevalences. Another demo-
graphic factor to consider is the neonatal
survival rate. A higher survival rate means
more infants at risk for CP; consequently,
a higher survival rate will itself give a
higher live birth prevalence of CP. The
Swedish population had a higher neonatal
survival rate throughout than the German
population, but the differences were
mainly small and adjusting for them made
only marginal differences.
Second, compatibility in clinical
classifications between series is essential.
As we agreed not only on clinical
categorization of cases, but also on the
grading of neuro-impairments/disabilities,
and demonstrated similarity between the
German and Swedish series, we concluded
that they were clinically comparable
(Krageloh-Mann et al. 1993). Since our
hypothesis of similar birthweight-specific
CP prevalences also turned out to be true,
we found it appropriate to pool the two
series in order to look for changes over
time.
In south-west Germany, recruitment
facilities were not entirely similar all over
the region. A certain under-reporting in
sub-areas with non-optimal facilities,
therefore, had to be taken into account.
Taken to extremes, the impact of under-
reporting could be greater than all
possible factors influencing time trends of
prevalences between the two countries. M
m
v
However, this seems highly improbable, M
r-
since trends over time in the sub-area *
with the best recruitment facilities and %-
the remaining area turned out to be
similar, both in comparison within the
region and with the Swedish series. All
given statistics, however, have to be
interpreted exclusively in a descriptive
sense.
The differences between the two series
were small and occurred mainly in the
normal-birthweight group. The German p
series had a lower prevalence of leg-
dominated RSCP with normal birthweight,
which is difficult to explain. The less
complete ascertainment of cases in the
outer areas of the Tubingen region does
not seem to be responsible, because the
lower prevalence rate in these areas was
distributed across all clinical subtypes as
well as all birthweight groups.
The prevalence of BSCP in children of
normal birthweight in the pooled series
showed no significant changes during the
study period. A stable prevalence of CP
children of normal birthweight and/or
normal gestational age has been the
predominant finding in various studies
(Hagberg et a/. 1984, 1989a; Pharoah
ef al. 1987, 1990; Dowding and Barry
1988; Finric ef ul. 1988; Stanley and
Watson 1988, 1992; Riikonen et a/. 1989;
Takeshita ef al. 1989). Recently, however,
Hagberg ef al. (1993). revealed a signifi-
cantly increasing trend from the late 1960s
to the mid-1980s for term CP children.
The prevalence of RSCP among LBW
children showed first an increasing, and
then a decreasing, trend. An increase in
prevalence of CP among LBW infants does
not give clues about the timing of adverse
events leading to the CP damage. How-
ever, decreasing prevalence combined
with increasing survival rates strongly
supports perinatal factors as the major
causative role, sudden changes towards
fewer adverse prenatal events being
unlikely. In both the German and the
Swedish series of BSCP there was a peak
prevalence in 1978-80 (except for MLBW
children in Sweden, who had a peak
in 1981-83), followed by a decrease,
calculated per live births as well as per
neonatal survivors for VLBW and MLBW
children. In the pooled series, the 4 79
-
i
a
b
C
decrease was remarkable per neonatal
survivors for VLBW and MLBW children,
and per live births for MLBW children
@<0.01 or ~ 0 . 0 5 ) We. believe that the
-
i
eq
association shown between a continued
decrease in mortality and increase in
e
X morbidity followed by a decrease in
morbidity is not accidental, and supports
the predominance of a perinatal origin of
BSCP in LBW infants.
The assumption of a perinatal origin is
strengthened by the time connection
between new perinatal and neonatal care-
practices and the reported changes in CP
trends. Parallel with increasing survival
-
I
rates for LBW newborn infants, there was
C
a marked decrease in the number of
B LBW/preterm children with CP (mainly
3
rn diplegia) in the Swedish CP panorama
study for the birth-year period 1954-70
(Hagberg et al. 1984). Both the increase in
0 survival and the decrease in CP was
m interpreted as a result of the introduction
x
t
m
and organization of good basic neonatal
E care for all newborn infants, as neonatal
3 intensive care had not then been
d
5 introduced. A subsequent increase in CP
3 among LBW children, combined with still-
5
¶ increasing survival rates, appeared during
0
vl the 1970s in Sweden (Hagberg et al.
u
C
19890) as well as in other countries,
-E
0
concurrent with the successive intro-
L
l
duction of neonatal intensive care. In
Sweden, that trend now seems to have
levelled off for VLBW children, and for
MLBW children there is even a slow
decrease (Hagberg et al. 1993), like that
for BSCP in the present study. This may
agree with Davies’ (1976) theory that
intensive neonatal care first leads to
increased rates of disability, but that these
later will decrease. A decline in live-birth
prevalence of CP among VLBW children
has been shown also in four other studies
(Haas et al. 1986, Robertson and Etches
1988, Saigal et al. 1989, Grogaard et al.
1990). and among MLBW children in a
study from Western Australia, concerning
specifically the spastic forms (Stanley and
Watson 1992).
Furthermore, the theory that BSCP in
LBW children has a perinatal origin is
supported by the biology of brain
development, and also by recent advances
in neuro-imaging techniques. The vulner-
480 ability of the periventricular and sur-
rounding areas in the immature brain
of 26 to 34 gestational weeks, and the
underlying periventricular leukomalacia
and inrracerebral haemorrhage which
mainly result in spastic diplegia, is well
documented (Volpe 1987, 1990, 1992;
Rorke 1992). Refined neuro-imaging
techniques nowadays visualize lesional
patterns of the brain which can be
correlated to the time of the lesional event
(Dubowitz et al. 1985; de Vries et al.
1987, 1988, 1989; Flodmark et al. 1989;
Barkovich and Truwit 1990; Keeney et al.
1991). Recent data from ongoing M R I
studies from the German series (Krageloh-
Mann et al. 1992) and others (Koeda et al.
1990, Yokochi et al. 1991) convincingly
indicate the perinatal timing of brain
parenchyma lesions in the majority of
LBW children with BSCP.
The incidence of the more severely
disabling forms of BSCP (the three-limb-
dominated, the four-limb-dominated and
the dyskinetic-spastic subtypes) increased
significantly from the 1970s to the early
1980s. There was also a higher proportion
of children with severe motor disabilities
and a higher proportion of the additional
severe impairments/disabilities associated
with BSCP during the same period of time
(Krageloh-Mann et al. 1993). A similar
finding was reported by Stanley et al.
(1993) for spastic quadriplegia in Western
Australia, spastic quadriplegia increasing
significantly during the birth-year period
1976-85. However, one encouraging sign
for the future was the break in this trend
in our series during the last birth-year
period, 1983-86. Progress in intensive
neonatal care ultimately might result in
fewer affected children and lesser degrees
of severity. Further studies are necessary
to confirm these trends.
Accepted j o r publication 19th October 1993.
Ackno wledgernents
These comparative investigations between south-
west Germany and western Sweden were supported
by rants from the Bundesministerium fur
Forsciung und Technologie (Germany), the Vera
and Hans Albrechtson. the First of May Flower and
the Torbjorn Jebner Foundations (Sweden). Most
valuable support was given by the Little Foundation,
by arranging important international research
workshops. It wodd not have been possible to
perform the study without the extensive help of a
large number of parents and professionals, to whom
the authors are especially grateful.
 rn
Authors’ Appointmettls Gudrun Hagberg. B.M.. B.A.. Ph.D.h.c.; m
Olngeborg Krageloh-Mann, M.D.; Karin Edebol Eeg-Olofsson, M.D., Ph.D.; -7
m
Cerhard Haas, M.D., Ph.D.; Bengt Hagberg, M.D., Ph.D.; I-
d
Richard Michaelis, M.D., Ph.D.; Department of Pediatrics, University of Goteborg.
Department of Child Neurology; Sweden.
Christoph Meisner. M.A.; d
Birgit Schelp; Torres ondence to first author at Eberhard-
Hans Konrad Selbmann, B.M.,Ph.D.; Karls-diversitat Tubifigen. Abteilung Entwick- E
Uepartment of Medical Data Processing, Uniwrsiiy lungsneurologie. Frondsbergstr. 23. D 72070
of Tubingen, Germany. Tubingen I. Germany.

SUMMARY
Epidemiological data of a collaborative study on children with bilateral spastic cerebral palsy (BSCP)
between south-west Germany and western Sweden are reported. The study period covered the birth
years 1975 to 1986. Overall, the rate of BSCP increased during the birth year periods 1975-77 and
1978-80, but decreased thereafter. The rise was due to an increase of BSCP in low-birthweight 2P
(LBW) children, especially very LBW (VLBW) children. Mortality rates in LBW, and particularly
VLBW, children decreased significantly during the whole study period in both countries. The BSCP
rate, after the initial increase, showed a decrease during the second half of the study period in LBW
children. Results are interpreted in favour of a predominantly prenatal aetiology in normal-
birthweight and of a predominantly peri- and neonatal aetiology in LBW children.

RESUMC
IMC Spastique bilarerale: etude comparative de population entre I’Allemagne du sud-ouest et la
Suede orriden tale. 1I: Epidemiologie
Les donnees epidemiologiques d’une etude en collaboration, des enfants presentant une forme
spastique bilaterale d’1.M.C. (BSCP) entre I’Allemagne du Sud-ouest et la Suede occidentale sont
rapportees. La periode d’etude couvrait une naissance entre 1975 et 1986. Globalement, le taux de
BSCP augmentait durant les periodes de naissance 1975-77 et 1978-80 puis diminuait ensuite.
L’augmentation etait liee a un accroissement de BSCP chez les enfants de faible poids de naissance
(LBW) et tout specialement de trks faible poids de naissance (VLBW). Les taux de mortalite pour les
enfants LBW, et particulierement VLBW. diminuaient significativement durant la periode totale
d’observation dans les deux etudes. 1.e taux de BSCP chez les enfants LBW, apres I’augmentation
initiale, presentait une diminution durant la seconde moitie de la periode d’etude. La repartition
entre soustypes de BSCP pour les enfants LBW indiquait une dimunition plus precoce pour les
forrnes predominant aux membres inferieurs que pour les autres formes; pour les enfants avec un
poids de naissance normal, les variations de taux n’etait pas significative.

ZUSAMMENFASSUNG
Bilaterale spastische Cerebralparese: eine vergleichende Populationsstudie z wischen
Sudwestdeulschland und West-sch weden. 11: Epidemiologie
Es werden epidemiologische Daten einer kollaborativen Studie an Kindern mit bilateraler spastischer
Cerebralparese (BSCP) zwischen Sudwestdeutschland und Westschweden vorgestellt. Die
Studiendauer erstreckte sich uber die Geburtsjahre 1975 bis 1986. Allgemein nahm die BSCP in den
Zeiten von 1975-77 und 1978-80 zu, danach jedoch ab. Der Anstieg war durch eine Zunahrne der
BSCP bei Kindern rnit niedrigem Geburtsgewicht (LBW) und besonders bei Kindern mit sehr
niedrigem Geburtsgewicht (VLBW) bedingt. Die Mortalitatsrate bei LBW-und besonders bei VLBW-
Kindern ist im Verlauf der Studie in beiden Landern signifikant gesunken. Die BSCP-Rate zeigte
nach dem anfanglichen Anstieg bei den LBW-Kindern eine Abnahme in der zweiten Halfte der
Studie. Bei den BSCP Untergruppen fur LBW-Kinder zeigte sich eine Verminderung der Bein-
betonten Formen eher als in den anderen Untergruppen; Fur Kinder mit normalem Geburtsgewicht
gab es keinen eindeutigen Trend.

RESUMEN
Paralisis cerebral espastica bilateral: estudio comparativo de poblacion entre el sud-oeste de
Alemania y la Suecia occidental. 11: Epidemiologia
Se aportan 10s datos epidemiol6gicos recogidos en un estudio en colaboraci6n entre niAos con
paralisis cerebral espastica (PCEB) del sud-este de Alemania y del oeste de Suecia. El periodo
estudiado cubria 10s nacimientos de 10s allos 1975 a 1986. En conjunto, el porcentaje de PCEB
aumentaba en 10s periodos 1975-77 y 1978-80 per0 disminuia despuks. El aurnento se debio a un
aumento de las PCEB en 10s nifios con bajo peso al nacer (BPN) especialrnente en 10s de muy bajo
peso (MBPN). La mortalidad en 10s BPN y en particular en 10s MBPN disrninuyb significativamente
a lo largo de todo el periodo de estudio en ambos paises. El porcentaje de PCEB, despuks del
aumento inicial, rnostro una disminucibn en la segunda mitad del periodo en estudio, sobre todo en
ninos con BPN. Las tendencias de 10s diversos tipos de PCE en 10s BPN mostraron una disminucibn
en las formas en que dominaba la efectaci6n de las piernas, rn& que en las otras formas. En 10s
ninos con peso normal al nacer. las tendencias eran inconsistentes. 481
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483