Gudrun 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.

Neuroimpairments, activity limitations, and participation restrictions in children with cerebral palsy.

In a representative series of 176 children with cerebral palsy (CP), aged 5 to 8 years, associations were studied between additional neuroimpairments, activity limitations, and participation restrictions in the domains of mobility, education, and social relations as proposed in the International Classification of Functioning Disability and Health (ICF). Learning disability occurred in 40%, epilepsy in 35%, visual impairment in 20%, and infantile hydrocephalus in 9% of the children. Additional neuroimpairments were most frequently seen in children with tetraplegia and dystonic CP and in those with antecedents of brain malformations or severe perinatal compromise. Activity limitations were studied with the Gross Motor Function Classification System (GMFCS) and a system for grading bimanual fine motor function (BFMF) was developed. There was a strong correlation of 0.74 between the GMFCS and BFMF (p<0.001). Learning disability, activity limitations, and participation restrictions were all clinically strongly associated with each other (p<0.001). Restriction in mobility was best predicted by the GMFCS, learning disability, and the BFMF; in education by learning disability and the GFMCS; and in social relations by learning disability, the GMFCS, and BFMF. Motor function and learning disability were important predictors for participation restrictions in children with CP. The ICF has the capacity to be a model to help plan interventions for specific functional goals and to ascertain the childs participation in society.

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 and restricted growth status at birth: population-based case-control study

Objective  To evaluate the association between growth status at birth and subsequent development of cerebral palsy in preterm and term infants.

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.

Probability of Walking in Children With Cerebral Palsy in Europe

OBJECTIVES. The purpose of this work was to describe walking ability in children with cerebral palsy from the Surveillance of Cerebral Palsy in Europe common database through 21 years and to examine the association between walking ability and predicting factors. PATIENTS AND METHODS. Anonymous data on 10042 children with cerebral palsy born between 1976 and 1996 were gathered from 14 European centers; 9012 patients were eligible for the analyses. RESULTS. Unaided walking as the primary way of walking at 5 years of age was reported for 54%, walking with assistive devices was reported for 16%, and no walking ability was reported for 30%. The proportion of children who were unable to walk was rather stable over time in all of the centers, with a mean proportion of 28%. Walking ability related significantly to cerebral palsy types, that is, spastic unilateral, spastic bilateral, dyskinetic, and ataxic cerebral palsy, as well as to IQ level, active epilepsy, and severe visual and hearing impairment. Severe cerebral palsy, defined as both the inability to walk and an IQ of <50, was present in 20% of the subjects. Logistic regression revealed that intellectual capacity was the variable most associated with walking ability in all 4 of the cerebral palsy types. The presence of a severe intellectual impairment increased the risk of being unable to walk 56 times if the child had unilateral spastic cerebral palsy type and 9 times if the child had bilateral spastic cerebral palsy type. CONCLUSIONS. The collaboration Surveillance of Cerebral Palsy in Europe provides a powerful means of monitoring trends in cerebral palsy and its functional consequences. The proportion of nonwalking in children with cerebral palsy seems to be rather stable over years and across centers despite the changes that have occurred in neonatal care across Europe. As is well known and also shown in this study, walking ability varied strongly with cerebral palsy type. Additional impairments, as well as the presence of epilepsy, correlated significantly with walking ability and, thus, the walking ability can be an indicator of total disability load.

Hydrocephalus prevalence and outcome in a population-based cohort of children born in 1989-1998

AIM To determine the prevalence, aetiology and clinical outcome in children with surgically treated hydrocephalus. METHODS A population-based study of all 208 liveborn children with hydrocephalus, 124 with infantile hydrocephalus and 84 with hydrocephalus associated with myelomeningocoele, born during 1989-1998 in western Sweden. Aetiological and clinical information was collected from records. RESULTS The prevalence of hydrocephalus was 0.82 per 1000 live births, 0.49 for children with infantile hydrocephalus and 0.33 for children with myelomeningocoele. The prevalence of infantile hydrocephalus decreased during the period from 0.55 to 0.43 per 1000. In this group, the aetiology was prenatal in 55% and peri-postnatal in 44% of the children. The origin was perinatal haemorrhage in all cases born very preterm. The mortality rate was 5% for children with either infantile hydrocephalus or myelomeningocoele. Mental retardation, cerebral palsy and epilepsy were significantly more frequent in the group with infantile hydrocephalus: 46% vs 16%, 31% vs 4% and 31% vs 10%, respectively. All children with infantile hydrocephalus born very preterm had at least one of these impairments, as did 80% of those with overt hydrocephalus at birth. CONCLUSION A slightly decreasing trend for infantile hydrocephalus was observed during the 10-y period. Children with infantile hydrocephalus had a worse outcome than those with myelomeningocoele. The need for neurosurgical revisions for two-thirds of the children indicates the need for further development of prevention and treatment strategies.Aims: The main objective of this thesis was to explore the cognitive and behavioural consequences of hydrocephalus in children born at term and preterm, with or without myelomeningocele (MMC) and with or without concomitant neurological impairments, such as cerebral palsy (CP), epilepsy or learning disabilities. Material and methods: From a population-based cohort of all 107 children with hydrocephalus born in 1989-1993, 73 of the surviving children were assessed with intelligence tests and most of them also using behavioural and autism rating scales. Thirty-six of 47 (77%) children with an IQ of ≥ 70 and eight children with MMC but no hydrocephalus were assessed with a neuropsychological test battery (NIMES) and compared with ageand gender-matched controls. Results: One-third of the children were normally gifted (IQ > 85), another 30% had a low-average IQ of 70-84 and 37% had learning disabilities (IQ < 70). An IQ of < 70 was found in 42% of children without MMC and in 29% of those with MMC. Children born preterm had a lower IQ than those born at term. Children with CP and/or epilepsy had significantly lower IQ scores than those without these impairments. Parents rated 67% and teachers 39% of the children as having behavioural problems. Learning disabilities increased the risk significantly. Almost all the children with CP and/or epilepsy had behavioural problems. Learning disabilities, CP and epilepsy significantly increased the risk of autistic symptoms, which were present in 13 %, in 4 % of those with MMC and in 20 % of those without MMC. Children with hydrocephalus both with and without MMC and with an IQ of > 70 performed significantly less well than controls on learning, memory and executive functions but not on registration skills. There were no differences between children with hydrocephalus in combination with MMC and those without MMC, whereas children with MMC but no hydrocephalus and normal intelligence performed as well as controls on all the neuropsychological functions. Conclusions: The majority of children with hydrocephalus had learning disabilities or a low-average IQ, as well as behavioural problems, and some had autistic symptoms. Despite average or slightly below average intelligence, children with hydrocephalus had major difficulties with learning and memory and with executive functions, regardless of the aetiology of the hydrocephalus. Only MMC did not appear to influence cognitive and neuropsychological outcome as much as the brain lesion causing or caused by the hydrocephalus. ISBN 978-91-628-7051-5 Göteborg 2007

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.