Health status of children with cerebral palsy living in Europe: a multi-centre study.

AIM: The aim of this report is to describe the health status of 8-12-year-old children with cerebral palsy (CP) of all severities in Europe using the Child Health Questionnaire (CHQ). METHOD: A total of 818 children with CP from nine centres in defined geographical areas participated. CP type, gross and fine motor function, additional impairments were classified and family data were obtained. The CHQ was used to measure the parent's perception of their child's physical (PHY) and psychosocial (PSY) health. RESULTS: PHY scores were lower than the reference samples with a median of 46. The severity of gross motor function influenced the CHQ scores significantly in the PHY scale with the lowest scores for children with least gross motor function. There were significant differences between the CP types in PHY with the higher scores for children with unilateral spastic and the lowest scores for children with bilateral spastic and dyskinetic CP type. Fine motor function severity significantly affected both the PHY and PSY scales. The severity of intellectual impairment was significantly associated with CHQ scores in most dimensions with higher scores for higher IQ level in PHY and PSY. Children with seizures during the last year had a significantly lower health compared with children without seizures. The results of the multivariate regression analyses (forward stepwise regression) of CHQ scores on CP subtype, gross and fine motor function, cognitive function, additional impairments, seizures, parental education and employment revealed gross motor function, cognitive level and type of school attended were significant prognostic factors. CONCLUSION: This report is based on the largest sample to date of children with CP. Health status as measured using the CHQ was affected in all children and was highly variable. Gross motor function level correlates with health from the PHY well-being perspective but the PSY and emotional aspects do not appear to follow the same pattern.

The natural history of gross motor development in children with cerebral palsy aged 1 to 15 years.

The aim of this study was to explore motor development in children with cerebral palsy (CP) using developmental curves for CP, subtypes, and the five severity levels of the Gross Motor Function Classification System (GMFCS). The Gross Motor Function Measure (GMFM) and the GMFCS were applied to 317 children (145 females, 172 males) with CP, aged between 1 and 15 years. The CP type distribution was spastic diplegia in 157 (49%), spastic hemiplegia in 101 (33%), spastic tetraplegia in 11 (3%), dyskinesia in 38 (12%), and ataxia in 10 (3%). Forty-five physiotherapists were trained in the GMFM and intra- and interrater reliability was tested. The GMFM was measured prospectively every 6 months up to the age of 4 years and once a year thereafter. Developmental curves were constructed for 258 children with spastic CP. About three-quarters of the children at GMFCS Level I reached 90% of the maximum GMFM score at 5 years of age. The performance peaked at 7 years of age. Children at GMFCS Level II reached 90% at a median age of 5 years, which was also the upper limit, reached by about three-quarters at 7 years of age. The majority of children at GMFCS Level III reached 80% of the GMFM by 7 years of age and most of the children at GMFCS Level IV reached 30% at 5 years and remained there. The median score for children at GMFCS Level V was 20%. The intra- and interrater reliability for the GMFM 88 among physiotherapists were Spearman's rank correlation coefficient 0.91 and 0.99 respectively. There were 931 measurements with a median of 2 (1-11) per child. The gross motor development was demonstrated for the five GMFCS levels in children with spastic CP. These kind of curves may be useful for monitoring and predicting motor development, for planning treatment, and for evaluating outcome after interventions.

Gross and fine motor function and accompanying impairments in cerebral palsy.

The aim of this study was to describe and analyze gross and fine motor function and accompanying neurological impairments in children with cerebral palsy (CP) born between 1991 and 1998 in western Sweden. A population-based study comprised 411 children with a diagnosis of CP ascertained at 4 to 8 years of age. Gross Motor Function Classification System (GMFCS) levels were documented in 367 children (205 males, 162 females). Bimanual Fine Motor Function (BFMF) classification levels of 345 of the children and information on learning disability, epilepsy, visual and hearing impairments, and hydrocephalus from 353 children were obtained. For spastic CP, a new classification according to the Surveillance of Cerebral Palsy in Europe of uni- and bilateral spastic CP was applied. GMFCS was distributed at Level I in 32%, Level II in 29%, Level III in 8%, Level IV in 15%, and Level V in 16%. The corresponding percentages for BFMF were 30.7%, 31.6%, 12.2%, 11.9%, and 13.6% respectively. Learning disability was present in 40%, epilepsy in 33%, and severe visual impairment in 19% of the children. Motor function differed between CP types. More severe GMFCS levels correlated with larger proportions of accompanying impairments and, in children born at term, to the presence of adverse peri/neonatal events in the form of intracranial haemorrhage/stroke, cerebral infection, and hypoxic-ischaemic encephalopathy. GMFCS Level I correlated positively to increasing gestational age. We conclude that the classification of CP should be based on CP type and motor function, as the two combine to produce an indicator of total impairment load.

Discussion groups with parents of children with cerebral palsy in Europe designed to assist development of a relevant measure of environment.

BACKGROUND: An instrument to measure environmental factors relevant to physically impaired children is being developed in a European context. Preliminary work in England had identified some potentially important themes. Further inquiry was needed to identify issues important in other European countries. OBJECTIVE: To inform the content of a questionnaire relevant to the environment of children with cerebral palsy (CP) living in Europe. DESIGN: A qualitative study using discussion groups. PARTICIPANTS: Parents of 28 children with CP from five countries; Denmark, France, Italy, Ireland and Sweden. One discussion group was held in each country with an average of seven parents per group. RESULTS: The four themes identified in the preliminary work done in England were strongly confirmed across Europe - namely: Mobility, Transport, Support by and to parents, and Attitudes of individuals and institutions towards children. Two new themes identified in the discussion groups were Bureaucracy and Access to information about rights and entitlements. CONCLUSIONS: The environmental factors that cause concern to parents of children with CP are similar across Europe. A prototype environmental questionnaire has been developed based on these findings. The environmental questionnaire is in use in a study in nine European centres.

The changing panorama of cerebral palsy in Sweden. IX. Prevalence and origin in the birth-year period 1995-1998.

AIM: This is the ninth report from the western-Swedish study of the prevalence and origin of cerebral palsy. METHODS: A population-based study covering the 88 371 live births in the area in 1995-1998. Birth characteristics, neuroimaging findings and risk factors in children with cerebral palsy were recorded, prevalence was calculated, and aetiology was analysed. RESULTS: The study comprised 170 children with cerebral palsy, i.e. a prevalence of 1.92 per 1000 live births. Excluding eight post-neonatally derived cases, the gestational age-specific prevalences were 77 per 1000 for children born before 28 wk of gestation, 40 for children born at 28-31 wk, 7 for children born at 32-36 wk and 1.1 for children born after 36 wk of gestation. Spastic hemiplegia, diplegia and tetraplegia accounted for 38%, 35% and 6%, respectively, dyskinetic cerebral palsy for 15%, and ataxia for 6%. For the first time, hemiplegia was now most common, due to the decline in preterm diplegia. There was a further increase in full-term dyskinetic cerebral palsy. The origin of cerebral palsy in children born at term was considered to be prenatal in 38%, peri/neonatal in 35% and unclassifiable in 27%, while in children born preterm it was 17%, 49% and 33%, respectively. CONCLUSION: The decreasing trend from the period 1991-1994 continued, both in children born at term and especially in those born preterm. However, the increase in dyskinetic cerebral palsy in children born at term was a matter of concern. In this group, a perinatal hypoxic ischaemic encephalopathy had been present in 71%.

Muscle strength and motor function in children and adolescents with spinal muscular atrophy II and III.

The aim of this study was to quantify isometric muscle strength and motor function in children and adolescents with spinal muscular atrophy (SMA) and to analyse the impact of reduced muscle strength on motor function. Six children and adolescents with SMA II and eight with SMA IlI were assessed regarding isometric muscle strength and motor function. Isometric muscle strength was tested with a myometer and the values obtained were compared with normative data. Motor function was videotaped and 20 movements were scored according to a three-point scale. All of the assessed children and adolescents with SMA II and SMA III showed reduced muscle strength, but there were great differences within the group. The typical pattern of muscle weakness in SMA, with proximal weakness greater than distal and the lower limbs more affected than the upper, was also seen in these children. The muscle weakness affected motor function in all assessed children. Walking, transfer from lying or sitting to the standing position and stair-climbing were possible in some of the children, despite marked reduction of muscle strength. The study increases our knowledge concerning the degree of muscle weakness in children with SMA and the impact of muscle weakness on motor function. The results increase our possibilities of understanding the prerequisites for everyday life in these children and planning therapeutic interventions. Repeated assessments with the methods used in this study may be used to monitor the course of the disease and to evaluate the efficacy of treatment.

Changing panorama of cerebral palsy in Sweden. VIII. Prevalence and origin in the birth year period 1991-94.

UNLABELLED: This 8th Swedish population-based cerebral palsy (CP) report comprises 241 children born 1991-94. The live birth prevalence was 2.12 per 1000. Excluding 7 postnatally-derived cases, the gestational age-specific prevalences were 86 for extremely preterm children, 60 for very preterm and 6 for moderately preterm, and 1.3 for term children per 1000. Spastic hemiplegic, diplegic and tetraplegic subtypes accounted for 33%, 44% and 6%, dyskinetic CP for 12% and simple ataxia for 4%. Neuroimaging had been performed in 90%. Probable aetiology was identified in 73% of preterm and 86% of term children. Among preterm children it was considered prenatal in 12%, peri/neonatal in 61% and unclassifiable in 27%, while it was 51%, 36% and 14% among term children. CONCLUSION: The live birth prevalence for CP in the birth year period 1991-94 continued to decrease slightly. Gestational age-specific prevalences increased marginally in extremely and very preterm births, continued to decrease in moderately preterm births and decreased slightly in term births. Probable aetiology and timing of the brain insult could be revealed in 81%, birth asphyxia being the likely cause in 28% of term children.

Correlation between ICIDH handicap code and Gross Motor Function Classification System in children with cerebral palsy.

The aim of this study was to apply the International Classification of Impairments, Disabilities and Handicap (ICIDH; WHO 1980) parallel to the Gross Motor Function Classification System (GMFCS; Palisano et al. 1997) in a population-based series of children with cerebral palsy (CP). Of the 116 children studied, birth characteristics, data on gross motor function, and level of handicap at 5 to 6 years of age, were retrospectively collected from medical records and documentation made by rehabilitation team members. Low handicap scores and mild levels of gross motor disability were present in children with hemiplegic CP, moderate scores in children with diplegic CP, simple ataxia, and athetotic CP, and high scores in children with dystonic CP and tetraplegic CP. A significant correlation was found between high handicap scores as well as high levels on the GMFCS and the presence of learning disability, epilepsy, and obvious aetiology of CP. A strong correlation was found between the handicap code and the GMFCS, the strongest concerning the dimension of mobility (r = 0.95,p<0.0001). A striking similarity in the grading of disability was present between the ICIDH handicap code and the GMFCS. The GMFCS is considerably less time-consuming and can be evaluated retrospectively. The handicap code requires more detailed information and is more useful for a comprehensive profile of the child.

Self-assessment of well-being in a group of children with epilepsy.

Epilepsy is common in childhood, the prevalence being about five per 1000 children. The purpose of this study was to assess well-being in children with controlled epilepsy (but did not include those with obvious neurodeficits such as mental retardation or cerebral palsy) and compare them with age-matched healthy children. The patient group comprised of 31 children, 12 boys and 19 girls, whereas the control population group consisted of 342 children, 176 boys and 166 girls who were all in good health. All children involved in the study were aged between 9-13 years. A questionnaire was distributed to the children to complete. It consisted of 39 bipolar adjectives and a visual analogue scale was employed. The results show that the group of children with controlled epilepsy did not differ significantly from the age-matched control group. There was no significant difference between the sexes except for the dimension of vitality, where the boys scored better than the girls. Thus the well-being of children with controlled epilepsy seems to be similar to that of children from a control population. The psychometric properties of the instrument were also assessed. An assessment of well-being in children with intractable epilepsy, using a similar approach, is in progress.

Late outcome after severe traumatic brain injury in children and adolescents.

OBJECTIVES: Eighteen surviving adolescents with severe traumatic brain injury were re-examined a mean period of 7.1 years after their trauma in order to determine their life situation, motor, cognitive functions and pattern of handicap. METHODS: A structured interview, the EB test of motor function, Ravens's progressive matrices, Peabody's neuropsychological test, SPIQ and the WHO classification of handicap were used. RESULTS: The group had a mean WHO Classification of Handicap score of 1.61 (SD 1.60) revealing mild handicap, and performed as a group significantly subnormal (p < 0.0001) in gross motor, fine motor, sensibility and perception sub-tests. The EB test revealed a mean value of 2.23 (SD 0.89) corresponding to mild disability. The mean non-verbal IQ score of 93.1 (SD 13.9) and the verbal score of 93.4 (SD 14.8) were within normal limits. CONCLUSIONS: Only 28% of the group of surviving adolescent TBI victims functioned within normal limits. The most crucial disabling component was poor social integration, which was clearly demonstrated in the WHO score.

Impairments, disabilities and handicaps in children and adolescents with epilepsy.

The patterns of motor and sensory impairments, disabilities and handicap were assessed in 217 children and adolescents with epilepsy. Motor and sensory dysfunctions were found to be common even in children without major additional neurodisorders such as cerebral palsy and mental retardation. Handicap was most severe in the dimensions of physical independence and orientation and increased significantly with duration of epilepsy. It was more severe when the onset of seizures was early and when secondary generalized seizures were present. Handicap was significantly reduced after epilepsy surgery.

Motor and sensory dysfunctions in children with mental retardation and epilepsy.

The aim of this study was to assess motor and sensory functions in a population-based series of 88 mentally retarded children with epilepsy. A new standardized physiotherapy protocol was developed for the heterogeneous population of children with epilepsy; the Cailler-Azusa scale was also found to be useful. For children with cerebral palsy, the gross motor function measure was used. Sensorimotor impairments, resulting in disabilities and handicap, were found to be very common but often overlooked and neglected. Sensorimotor dysfunctions need to be identified in order to provide rational training, understanding and care to children with epilepsy and mental retardation.

Hidden dysfunction in childhood epilepsy.

Sensory and motor function were evaluated in 84 children and adolescents with epilepsy but not additional neuroimpairments such as mental retardation or cerebral palsy. The influence of sex, age, age at onset of epilepsy, duration, seizure type and frequency and medication was analysed. The Bruininks-Oseretsky Test of Motor Proficiency and the EB (Eva Beckung) test were used. We found significant dysfunction in gross as well as in fine motor functions. The most obvious deviation from normal was noted in gross motor function, mainly in the areas of running speed, balance, response speed and bilateral coordination, but also in upper-limb speed, dexterity and coordination. Boys performed better than girls. Motor function scores improved with age but sensory and motor problems persisted in the teenage years. Early onset and a long duration of the epilepsy were associated with significantly more sensory and motor difficulties. The children on monotherapy performed significantly better than the children on polytherapy and, surprisingly, also better than those without antiepileptic medication. Sensory and motor function should be carefully investigated in the context of a comprehensive rehabilitation programme for children with epilepsy.

The effects of epilepsy surgery on the sensorimotor function of children.

The motor and sensory functions of 50 children were investigated before and six months after epilepsy surgery; 34 infants were assessed 24 months after surgery. Postoperatively, 20 children were seizure-free and 22 had a significant reduction of seizures. Epilepsy surgery was found to be an effective mode of treatment for intractable seizures in childhood, even in multiply handicapped individuals. Motor and sensory functions did not deteriorate after surgery; in fact, significant improvements were found in more than half of the children, including those with multiple handicaps. Improvements were most obvious in the seizure-free group, but were also noted in those with reduced seizure frequency. The younger children benefited more from surgery as regards sensorimotor function than did older children and adolescents.

Motor and sensory impairments in children with intractable epilepsy.

During a 3-year period (1988-1991), 72 children with severe intractable epilepsy were studied. A standardized protocol for assessment of motor and sensory function was designed for school age children. Function was quantified on a 4-point scale on 47 items, including gross motor function, balance, coordination, strength, range of motion (ROM), velocity, fine motor function, sensation, perception, and neurologic tests. Classification of handicaps according to World Health Organization (WHO) definitions was performed. Videotape documentation completed the assessment. Evaluation of treatment services showed that provision of rehabilitation services had been insufficient and provided only for children with additional major movement disorders, mainly cerebral palsy (CP) cases. To minimize the handicap in children with severe epilepsy, it is essential to clarify the total sensorimotor impairment pattern, including balance, coordination, and perceptual capacity. Impairments in these functions are, as shown in this study, frequent and exist independent of major disabilities such as mental retardation or cerebral palsy. When several neuroimpairments were identified, a multiplicative rather than an additive effect on the total handicap was evident.