Bengt Hagberg

The changing panorama of cerebral palsy in Sweden. IV. Epidemiological trends 1959-78.

From a population‐based series of 773 patients with cerebral palsy (CP) born in 1959–78, an analysis was made of the epidemiological trends over this period of 20 years, divided into five 4‐year periods with emphasis on the last two. After a significantly decreasing incidence of CP in the first three periods (1959–70) from 1.9 to 1.4 per thousand, there was a significant increase in the last two periods, reaching 2.0 per thousand in the period 1975–78. Both the decreasing trend in the earlier periods and the increasing trend in the more recent ones were mainly referrable to spastic/ataxic diplegia in preterm CP, and to dyskinetic syndromes in CP infants born at term. With respect to pathogenesis, the corresponding changes in CP incidence were mainly accounted for by the group with potential perinatal risk factors. When analysed on the basis of surviving babies in birth‐weight‐specific groups, the incidence of CP in 1971–78 was found to have increased in all groups, but this was only statistically significant in the low birth weight group of 2 000–2 500 g. Changing trends in incidences ran parallel with a steadily progressive decline in perinatal mortality through all five periods. A considerable and cumulative net gain of surviving non‐CP children was continuously achieved; this was also true for 1970–78, in spite of an increasing CP morbidity during these last two 4‐year periods.

THE CHANGING PANORAMA OF CEREBRAL PALSY IN SWEDEN 1954–1970

ABSTRACT: Hagberg, B., Hagberg, G. and Olow, I. (Department of Paediatrics II, Childrens Hospital and the Habilitation Unit of Bräcke‐Östergård, Gothenburg, Sweden). The changing panorama of cerebral palsy in Sweden 1954–1970. I. Analysis of the general changes. Acta Paediatr Scand 64:187, 1975 From an unselected series of 560 cases of cerebral palsy in Sweden, born 1954‐70, the changes in incidence through the four periods 1954‐58, 1959‐62, 1963‐66 and 1967‐70 were analysed. The total incidence successively and significantly decreased from 2·2 %0 in the first period to 1·3 %0 in the last. This decrease was mainly related to (1) the syndromes of spastic and ataxic diplegia, (2) the low birth weight babies, and (3) the group with perinatal causes. The decrease was not related to any special intelligence quotient or geographical region.

The changing panorama of cerebral palsy in Sweden. VI. Prevalence and origin during the birth year period 1983-1986.

The prevalence and origin of cerebral palsy in children born between 1983 and 1986 are reported. The crude live‐birth prevalence was 2.49 per 1000; 1.56 for term births, 0.93 for preterm births. The increasing trend from 1970 persisted and was statistically significant for both groups. The most pronounced increase during 1983–1986 occurred in term cerebral palsy. The increasing use of neuroimaging techniques made the timing of damaging events more reliable. In term cerebral palsy, the origin was convincingly prenatal in 28% of cases and perinatal in 25%. The particular critical period for the brain damage underlying cerebral palsy was considered to be weeks 26–34 of gestation, i.e. when periventricular structures are extraordinarily vulnerable. In term infants, this occurs during late intrauterine life, while in the majority of preterms it occurs in early neonatal life. In total, two‐thirds of cerebral palsy lesions might have been acquired during these decisive months of brain development.

Epilepsy in a representative series of Rett syndrome

In a representative series of 53 females with Rett syndrome (RS), aged 5–55 y, a history of epilepsy was present in 50 (94%), 45 of whom had 5‐y active epilepsy. Compared with severe mental retardation in general, the median age of seizure onset was significantly later (4 vs 0.8 y) and partial complex seizures were more frequent (54% vs 23%). Neonatal seizures had occurred in only one and infantile spasms in none compared with 26% and 12%. After teenage, the severity of epilepsy tended to decrease, i.e. lower seizure frequency and relatively more partial seizures. The rate of being seizure‐free for 1 y was 8% after 10 y and 40% after 27 y of epilepsy duration. Frequent seizures were associated with smaller head circumference.

Cerebral palsy in preterm infants: a population-based case-control study of antenatal and intrapartal risk factors

Previous studies have indicated that foetomaternal infection increases the risk of spastic cerebral palsy (CP) in term infants, whereas this association appears to be less evident in preterm infants. The aim of this study was to analyse infection‐related risk factors for spastic CP in preterm infants. A population‐based series of preterm infants with spastic CP, 91 very preterm (>32wk) and 57 moderately preterm (32–36 wk), born in 1983–90, were included and matched with a control group (n= 296). In total, 154 maternal, antenatal and intrapartal variables were retrieved from obstetric records. In the entire group, histological chorioamnionitis/pyelonephritis, long interval between rupture of membranes and birth, admission‐delivery interval >4 h and Apgar scores of >7 at 1 min just significantly increased the risk of CP, and Apgar scores of >7 at 5 and 10 min were strongly associated with an increased risk. Abruptio placentae, Apgar scores >7 at 1 min and pathological non‐stress test (reason for delivery) were significant risk factors of CP only in the moderately preterm and hemiplegic groups, whereas fever before delivery was a significant risk factor in the very preterm and spastic diplegic groups. Antibiotics during pregnancy was associated with CP only in the spastic diplegic CP group.

The changing panorama of cerebral palsy in Sweden 1954-1970. III. The importance of foetal deprivation of supply.

Abstract. From record data derived from an unselected series of 560 Swedish cases of cerebral palsy, born in 1954‐70, the significance of prenatal factors associated with materno‐foetal vascular insufficiency causing foetal deprivation of supply (FDS) was investigated. Cerebral palsied children were found to have, on an average, retarded intrauterine growth when compared with a normal population. The incidence of bleeding during pregnancy, maternal toxaemia and multiple pregnancies were significantly increased (p<0.0001). The incidence of the prenatal factors in question had remained unchanged through the years 1954‐70. An additive interplay between factors of FDS and asphyxia/cerebral haemorrhage was revealed. As a whole the findings support the conclusion that FDS plays a major role in the pathogenesis of cerebral palsy today, particularly among the multihandkapped.

Three decades of sociomedical experiences from West Swedish Rett females 4-60 years of age.

Rett syndrome, today known as a worldwide important cause behind severe mental retardation in females, has been seen in Sweden since the 1960s (the senior author). This study gives population representative clinical and sociomedical follow-up data from West Sweden (1/4 of the population in Sweden). The series comprises 54 females diagnosed in 1971-1998, 5-60 years old, median age 20. Mortality in 1971-2000 was 18% (10/54), median age at death 20. Death usually had been sudden and unexpected. The sociomedical follow-up emphasized the adult group. The large majority had usually moved to small group homes, a good and well-accepted environment once the staff had learned the particularities of these so uniquely neurodevelopmentally disabled women.

DECREASING INCIDENCE OF LOW BIRTH WEIGHT DIPLEGIA—AN ACHIEVEMENT OF MODERN NEONATAL CARE?

The clinical syndromes of 429 cerebral palsied children born in 1959–68 and constituting a representative and unselected Swedish series were analysed with respect to the changing panorama of the various syndromes. The main preliminary finding was a significantly decreased total incidence due to a likewise significantly lowered number of diplegic babies with a birth weight less than 2 500 g.

'Anonymous' - an organization for families of children with undiagnosed brain diseases

As a consultant child neurologist with opportunities over many years to travel widely throughout Sweden, I have time and again seen severely brain impaired children, without any reasonable explanation for their condition and with a disturbing lack of precise diagnosis, despite vigorous investigations. Not even a name for the disastrous condition could be provided for the frustrated parents. Whilst understanding that an effective treatment was impossible to find, these families repeatedly asked for new consultations in the hope of at least a precise diagnosis and a label. Indeed, it is a hard burden for any family to have a child with a hopelessly progressive brain disorder, and ignorance as to the cause makes it even worse. There are no experiences to learn from, no straws to clutch for help, no hope for meaningful treatment, no special interest group to join. Encouraged by the Swedish Social Council in their 1994-97 campaign for ‘Small and less well known handicap groups’, I took the initiative to start a family network group for support and information to families with situations as described. ‘Anonymous’ was rapidly established through this official support and with financial starting grants. From being a pioneering project in 1994, the activity has since 1997 become established as an independent special parents’ interest organization. The project immediately created wide interest and became an instant success among families with these problems. Their relief to meet other parents in the same situation was great. The medical

Guillain‐Barré's Syndrome Associated with Poliomyelitis in Newborn

Two cases of Guillain‐Barrés syndrome in newborns with, probable poliomyelitis are reported. The infants were 10 and 8 days old. Both were afebrile, had general flaecidity with tetraplegia and initially the cerebrospinal fluid showed marked albu‐minocytologic dissociation. The mother of the first had typical poliomyelitis, starting on the day of delivery. This boy recovered completely. The other developed residual paralysis characteristic of poliomyelitis. A survey of the well documented cases of poliomyelitis in infants under two weeks of age reveals that general flaecidity and elevated cerebrospinal protein levels seem to be the rule rather than an exception. Different possibilities for this phenomenon are discussed.