Behaviour in children with cerebral palsy with and without epilepsy.

The aim of the study was to describe behavioural problems in children with cerebral palsy (CP) with and without epilepsy. The children were sampled from the Western Sweden CP register and were part of a European Union project. The Strength and Difficulties Questionnaire and questions on epilepsy were answered by one parent of each child. Medical records were reviewed. Parents of 83 children (44 males, 39 females) age range participated: 30 at Gross Motor Function Classification System levels I and II, and 53 at levels III to V; 60 had spastic age range 8 to 12 years (bilateral 42, unilateral 18) and 23 dyskinetic CP; 34 children had active epilepsy. The proportion of children with normal behaviour on the total difficulties score (TDS) of the Strength and Difficulties Questionnaire was significantly lower than normative data (57% vs 80%, p<0.001). Parents of 21 children (25%) considered their child's behaviour to be abnormal. Children with CP and epilepsy had a significantly higher median TDS (p=0.03) than seizure-free children. In children with aided or no walking ability, the TDS was significantly higher in those with epilepsy (p=0.04). Parents of 32 children (39%) considered their children's behaviour to have an impact on themselves and others. We conclude that behavioural problems are common in children with CP, and even more when epilepsy is present. Parents identify these problems, and professionals need to address them.

Bilateral spastic cerebral palsy--prevalence through four decades, motor function and growth.

UNLABELLED: The aim was to depict changes in the prevalence and severity of bilateral spastic cerebral palsy (CP) over a 40-year period. Another objective was to characterise the group born in 1991-1998 with respect to gross motor function, spasticity and growth. Data were obtained from the CP register of western Sweden and rehabilitation records. RESULTS: After a rise to 1.27 per 1000 live births in 1983-1986, the prevalence decreased significantly, in children born both preterm and at term, to 0.69 in 1995-1998. After 1975, more children were born preterm than at term. There was a significant decrease in severe bilateral spastic CP during the same period, mainly in children born at term. In all, 46% of the children born at term and 33% of those born preterm had a severe motor impairment, i.e. no walking ability. In the 167 children born in 1991-1998, the gross motor function classification system (GMFCS) level was I in 14%, II in 34%, III in 10%, IV in 25% and V in 17%. The GMFCS level correlated with the gross motor function measure (GMFM) and the Ashworth spasticity scores, as well as with the deviation in postnatal weight and height. We conclude that the prevalence of bilateral spastic CP has decreased since the mid-1980s, parallel to a reduction in the severity of the motor impairment. Children born preterm have predominated since the mid-1970s. The severity of the motor impairment correlated with the degree of spasticity, GMFM and growth. The percentage of children who were underweight was substantial.

Cerebral palsy and intrauterine growth in single births: European collaborative study.

BACKGROUND: Cerebral palsy seems to be more common in term babies whose birthweight is low for their gestational age at delivery, but past analyses have been hampered by small datasets and Z-score calculation methods. METHODS: We compared data from ten European registers for 4503 singleton children with cerebral palsy born between 1976 and 1990 with the number of births in each study population. Weight and gestation of these children were compared with reference standards for the normal spread of gestation and weight-for-gestational age at birth. FINDINGS: Babies of 32-42 weeks' gestation with a birthweight for gestational age below the 10th percentile (using fetal growth standards) were 4-6 times more likely to have cerebral palsy than were children in a reference band between the 25th and 75th percentiles. In children with a weight above the 97th percentile, the increased risk was smaller (from 1.6 to 3.1), but still significant. Those with a birthweight about 1 SD above average always had the lowest risk of cerebral palsy. A similar pattern was seen in those with unilateral or bilateral spasticity, as in those with a dyskinetic or ataxic disability. In babies of less than 32 weeks' gestation, the relation between weight and risk was less clear. INTERPRETATION: The risk of cerebral palsy, like the risk of perinatal death, is lowest in babies who are of above average weight-for-gestation at birth, but risk rises when weight is well above normal as well as when it is well below normal. Whether deviant growth is the cause or a consequence of the disability remains to be determined.

Long-term follow-up, neurological outcome and survival rate in 28 Nordic patients with glutaric aciduria type 1.

All 28 patients, 13 females and 15 males, with glutaric aciduria type 1 diagnosed between 1975 and 2001 in Denmark, Finland, Norway and Sweden were identified and studied retrospectively until 2001. Mass screening was not performed. Three were sibling cases. Prenatal enzymatic diagnosis performed in 11 pregnancies led to termination in one. The median follow-up time was 14 years. Six patients had died. At 10 years of age the cumulative survival rate was 89% and at 35 years 44%. The dominating neurological sign was dystonia in 20 and dyskinesia in 4. Three had only slight spastic signs and information was missing in one. The head circumference at birth was significantly larger than normal and increased significantly until 6 months of age. The onset was acute encephalopathic in 24 patients and insidious in 3. From the time of diagnosis, all patients but one were prescribed protein restriction and/or a diet low in lysine and tryptophan. Riboflavine and/or carnitine supplementation were given to 25. Neurological deficits did not improve on the offered treatment. Deterioration may have been averted by intense acute metabolic treatment in a few patients. Dystonia correlated significantly to absence of speech but not to cognitive function. Severe disability, including motor, cognitive and speech functions, correlated significantly with acute onset, dystonia and mortality, and weakly with a deteriorating course, but not with age at onset, diagnosis, or follow-up, nor to head size. Results from future population studies derived from mass screening will have to relate to clinical diagnostic series of the kind presented here.

Antenatal risk factors for cerebral palsy.

Two of every 1000 live-born children develop cerebral palsy (CP). The aetiology of CP is often unclear and because CP is a symptom complex rather than a disease, clinically defined at 4-5 years of age, it is not surprising that there are considerable problems associated with epidemiological studies of its aetiology. The only reason for the CP concept is that it emanates from an insult to a growing, developing brain and a dynamic clinical picture from static pathology. Evidence suggests that 70-80% of CP cases are due to prenatal factors and that birth asphyxia plays a relatively minor role (<10%). Some antenatal risk factors are repeatedly observed to be related to CP: low gestational age, male gender, multiple gestation, intrauterine viral infections and maternal thyroid abnormalities. Recently, intrauterine infection/inflammation with a maternal response (consisting of chorioamnionitis) and a fetal inflammatory response (consisting of funicitis or elevated interleukin-6 in fetal plasma) has been found to be related to white matter injury and CP. Some risk factors are associated with CP at all gestational ages whereas others mostly affect term or preterm infants, e.g. intrauterine growth restriction seems to be a risk factor in term infants. There also seems to be an association between autoimmune and coagulation disorders and CP.

Cerebral palsy and perinatal infection in children born at term.

OBJECTIVE: To investigate the link between infection-related risk factors for cerebral palsy subtypes in children born at term. METHODS: A case-control study was performed in a population-based series of children with cerebral palsy born at term (n=309) matched with a control group (n=618). The cases were divided into cerebral palsy subtypes: spastic hemiplegia, spastic diplegia, spastic tetraplegia, and dyskinetic cerebral palsy. All forms of spastic cerebral palsy were also analyzed together. All records were examined for maternal and neonatal signs of infection. Univariate and adjusted analyses were performed. RESULTS: Infection-related risk factors were shown to be independent risk factors for spastic cerebral palsy in the adjusted analyses. This was especially pronounced in the subgroup with spastic hemiplegia in which bacterial growth in urine during pregnancy (n=11 [7.5%], odds ratio [OR] 4.7, 95% confidence interval [CI] 1.5-15.2), any infectious disease during pregnancy (n=57 [39.0%], OR 2.9, 95% CI 1.7-4.8), severe infection during pregnancy (n=12 [8.2%], OR 15.4, 95% CI 3.0-78.1), antibiotic therapy once during pregnancy (n=33 [22.6%], OR 6.3, 95% CI 3.0-15.2) as well as several times during pregnancy (n=9 [6.2%], OR 15.6, 95% CI 1.8-134.2) constituted strong independent risk factors. However, only neonatal infection (n=11 [9.1%], OR 14.7, 95% CI 1.7-126.5) was independently significantly associated with an increased risk of spastic diplegia and tetraplegia. CONCLUSIONS: Infection-related factors are strong independent risk factors for the subgroup with spastic hemiplegia in children with cerebral palsy born at term. The finding is less pronounced in the subgroups with spastic diplegia or tetraplegia. LEVEL OF EVIDENCE: II.

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.

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 child's participation in society.

Clinical and aetiological aspects of epilepsy in children with cerebral palsy.

The aims of this retrospective and population-based study were to describe the frequency and characteristics of epilepsy in 146 children (82 males and 64 females) with cerebral palsy (CP) born from 1987 to 1994 in the Göteborg area of Sweden. The frequency of epilepsy was found to be 38% (55 children). All children with tetraplegic CP and about one-third of the children with other CP types developed epilepsy. Age at onset of epilepsy varied with type of CP: children with tetraplegic CP tended to have an earlier onset of epilepsy than children with other CP types. Partial seizures were the most common seizure type; all children with hemiplegic CP had partial seizures. Children with cognitive impairment had a higher frequency of epilepsy than those without cognitive impairment. CP aetiology may predict the development and outcome of epilepsy, as children with CP caused by CNS malformation, CNS infection, and grey matter damage all showed a higher frequency of epilepsy than children with CP of other aetiology, and also had less chance of becoming seizure-free.