Cerebral palsy in Norway: prevalence, subtypes and severity.

BACKGROUND/AIM: To describe prevalence, subtypes and severity of cerebral palsy (CP) in Norway using criteria proposed by the Surveillance of Cerebral Palsy in Europe (SCPE) network. MATERIAL: All children in Norway with CP born in January 1996-December 1998 were registered in the Cerebral Palsy Registry of Norway. The Medical Birth Registry of Norway provided the perinatal data. RESULTS: A total of 374 children with CP were identified with a prevalence of 2.1 per 1000 live births. Detailed information was obtained from 294 (79%) children. Median age at clinical assessment was 6.9 years (range: 1.9-10.2 years). Thirty-three percent of the children had spastic unilateral CP, 49% spastic bilateral, 6% dyskinetic, 5% ataxic CP and 7% were not classified. Severely impaired vision and hearing were present in 5% and 4% of the children, respectively. Active epilepsy was present in 28%, mental retardation in 31% and severely impaired or no speech in 28% children. The most severe impairments in gross motor function were observed in children with low Apgar scores, and the most severe impairments in fine motor function in children born at term, with normal birth weight and low Apgar scores. CONCLUSION: Compared with other populations, the prevalence of CP as well as the proportions of subtypes and gross motor impairments were similar, whereas fine motor impairments and associated impairments were more common. The classification of children with mixed forms of CP is still a challenge. Children were more severely affected if Apgar scores were low, and if they were born at term.     

A non-handicapped cohort of low-birthweight children: growth and general health status at 11 years of age

Aims: To describe and compare physical growth, current health status, functional limitations and neurodevelopmental impairments (defined as low IQ, school problems or psychiatric disorder) at 11 y of age in a population of non-handicapped low-birthweight (LBW) children with that of normal-birthweight (NBW) children. Methods: A population-based sample of 130 LBW children (weighing less than 2000 g at birth) without major handicaps, and a random sample of 131 NBW children born at term. Somatic and mental health and cognitive abilities were assessed through questionnaires to parents, a physical examination, standardized tests of cognitive function (WISC-R) and a semi-structured interview (Children Assessment Schedule). Results: General somatic health status was similar for the LBW and NBW children. The LBW children were shorter (mean difference -2.5 cm; 95% CI -0.9 to -4.2) and had a smaller head circumference (mean difference -0.8 cm; 95% CI -0.4 to -1.1) but similar weights and body mass indices. Differences and similarities in anthropometric measures were the same at 5 and 11 y of age. The LBW children had higher systolic (mean difference 3.2 mmHg; 95% CI -0.6 to -0.3) but similar diastolic blood pressure. A higher proportion of LBW children had decreased visual acuity and hearing impairment. Forty per cent of LBW children had neurodevelopmental impairments, compared to 20% of NBW children (OR 2.6; 95% CI 1.5 to 4.5).

Conclusion: At 11 y of age, survivors of moderately low birthweight without major handicaps may have generally good health, but are at risk of neurodevelopmental impairments.     

The relationship of child functioning to parental physical assault: linear and curvilinear models

Previous research suggests a curvilinear relationship between child disability and physical abuse, with children with mild impairments at greater risk than both children with severe impairments and superior functioning. Using a national probability sample of families investigated for maltreatment (N = 1675), this study tested for both linear and curvilinear relationships of child functioning to parental physical assault. Linear relationships were found between problem behaviors and minor and severe assault, and between social skills and minor assault: the more impaired the level of child functioning, the greater the risk. Curvilinear relationships were found in which children with mildly impaired or average language skills were at greater risk for minor assault than both children with severe impairment or above average and superior skills. Children with superior daily-living skills were at lower risk for severe assault than all other children. Implications for understanding processes underlying parental physical assault of children with impairments are discussed.     

Implications for estimates of disability in children: a comparison of definitional components

OBJECTIVE: To test how prevalence estimates and characteristics of children vary by the way that disability is defined. Specifically, to determine 1) the proportions of children identified as disabled by one particular operationalization of disability based on parental reports of three types of consequences (i.e., functional limitations [FL]), dependence on compensatory mechanisms (CD), and service use or need beyond routine [SU/N]), and 2) whether children identified as disabled by these three types of consequences differ by type of disorder or condition, age, socioeconomic status, or race. METHOD: We analyzed a national dataset representing a random sample of 712 households with 1388 children. The Questionnaire for Identifying Children with Chronic Conditions (QuICCC) was used to identify children with disabling conditions. We divided the QuICCC items into three discrete sets, reflecting three definitional components of disability, and compared the proportions and characteristics of children fitting these components separately and in combination. RESULTS: Using the QuICCC definition of disability, SU/N identified the largest proportion of children (72%), followed by CD (55%) and FLs (49%). Forty-four percent of children were identified by only one component, 36% by two components in any combination, and 20% by all three components. The type of disorder or condition generally did not vary by the three definitional components, although the FL component may be more effective at identifying children with sensory impairments. Children identified by two or more components were more likely to have multiple conditions and had more pervasive disorders than those identified by only one component. The youngest children (0 to 3 years old) may be less likely to be identified as disabled than children of other ages, especially by FLs. FLs also were more likely to identify children from the poorest and least educated families. CONCLUSIONS: Although the specific findings reported here pertain to a single definitional approach (the QuICCC), the data highlight that who will be classified as disabled (and who will not) may be dependent on how disability is defined. The implications of using different definitions and definitional components on both the prevalence and the characteristics of children with disabilities need to be considered before data can be applied responsibly and appropriately.     

Parental perception of cold extremities and other accompanying symptoms in children with cerebral palsy.

Cold extremities have been noted in non-walking children with cerebral damage compared with healthy controls. Whether this is a general problem in children with cerebral palsy (CP) and associated with other symptoms is unknown. This study describes accompanying symptoms such as cold extremities, constipation, pain, sleeping disorders and impaired well-being in children with CP as well as treatment the children have undergone. Associations between cold extremities and other symptoms borne by the children were analysed and discussed. From information in postal surveys received from parents of children with CP, 107 children (60 boys and 47 girls) aged 5-13 years, mean 11 years 8 months (SD 2 years 11 months), were described and analysed. Besides neurological impairments, many children had cold extremities and pain, sleeping disorders, constipation, and impaired well-being. Most children had had one or more of these symptoms for over 1 year but the symptoms were largely untreated. Non-walkers generally had more symptoms than walkers. Although pain, constipation, and sleeping disorders may have different underlying causes in children with CP, these symptoms might also be mediated or aggravated by dysfunction in the autonomic nervous system. To improve the child's well-being, early recognition and treatment of accompanying symptoms is important.     

Utility of brainstem auditory evoked potentials in children with spastic cerebral palsy

In a retrospective study of 75 children with spastic cerebral palsy (CP), brainstem auditory evoked potentials (BAEP) were recorded and subsequently correlated with birthweight, gestational age, aetiology and type of CP, neuroradiological findings, additional impairments and disabilities (including the inability to walk independently). Seventeen patients (22.7%) had abnormal BAEP recordings. Thirteen of these 17 patients (76.5%) had spastic tetraplegia, 16 patients (94.1%) were full-term infants, 12 patients (70.6%) had myoskeletal problems, 9 (52.9%) had epilepsy, 16 (94.1%) had visual impairment, 13 patients (76.5%) were unable to walk independently, while all 17 patients (100%) had speech impairment and mental retardation. The aetiology of CP was prenatal in 2 of these 17 patients (11.8%) and perinatal in 15 patients (88.2%). Thirteen patients (76.5%) had cortical atrophy determined by either computed tomography or magnetic resonance imaging, two patients (11.8%) had an infarct picture and two patients (11.8%) had maldevelopment of the central nervous system. There was a definite statistically significant association between abnormal BAEP recordings and full-term delivery, perinatal aetiology of CP, spastic tetraplegia, speech, visual and myoskeletal impairments, epilepsy, mental retardation, inability to walk independently and cortical atrophy on neuroimaging (p < 0.001). We conclude that abnormal BAEP recordings in children with spastic CP are indicative of poor prognosis and associated with a "multihandicap state". BAEP testing should be incorporated into the diagnostic plan of all children with spastic CP newly referred to neurodevelopmental centres.     

Outcome in infants of birth weight 500 to 999 g: a continuing regional study of 5-year-old survivors

During 1979 and 1980, 351 infants of birth weight 500 to 999 g were born in the State of Victoria: 89 (25.4%) survived to the age of 2 years corrected for prematurity, and 83 were fully assessed by a multidisciplinary team; partial data were obtained on the remainder. At the age of 5 years, corrected for prematurity, 85/89 (96%) were evaluated by a multidisciplinary team, although not all children could be fully evaluated by the psychologists. Reports were available for another three children; one child was untraced. Of the survivors able to be classified at 5 years, 59/82 (72%) had no functional handicap. Functional handicaps was severe in 16 (19%), moderate in four (5%), and mild in three (4%). Functional handicaps were present in 50% (8/16) of outborn survivors compared with the 23% (15/66) for the inborn survivors (P = 0.02). Cerebral palsy was diagnosed in eight children at 5 years and in 12 children at 2 years. The diagnosis was stable for the children not ambulant at 2 years; five of seven 2-year-old children with mild cerebral palsy had "outgrown" the diagnosis by 5 years, but ataxic cerebral palsy was not identified in one child until 5 years. Six children were blind; four had severe sensorineural or mixed deafness, one more than at 2 years. Of 82 children assessed according to identical criteria for functional handicap at both 2 and 5 years, 52 (63%) remained in the same category at 5 years, three (4%) were judged to be more severely handicapped, and 27 (33%) were less severely handicapped. The 2-year evaluation of extremely low birth weight children often proved to be unduly pessimistic, for many showed improvement or recovery from functional handicaps and impairments by 5 years of age.     

Comparison of impact on mood,health, and daily living experiences of primary caregivers of walking and non-walking children with cerebral palsy and provided community services support

Many children with cerebral palsy (CP), especially non-walkers, were previously reported to have cold extremities, pain, sleeping disorders, constipation, and impaired well-being – besides accompanying impairments. Most children had had one or more of these symptoms for more than 1 year, and the symptoms were largely untreated. This study (1) describes mood, health, and daily life experiences of the children's parents; (2) explains impact that the child's impairments and symptoms have on the family; and (3) investigates community services support. Information in this study was gathered from parents of 106 children, ages 5–16, with CP, who lived in northern Sweden. Compared with parents of walkers, parents with non-walking children and several disorders were more frequently anxious for their children's physical and psychological health; often experienced restricted time for themselves; reported frequent daily living interferences; and stated that their health was affected due to the child's health. 10 percent of all families reported that their need of community services support was unmet. To improve health and to provide good community services support for the entire family, regular follow-up and evaluation of the child's treatment and family support are important.     

Employment in adults with congenital heart disease

OBJECTIVE: To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. DESIGN: Cross-sectional study. SETTING: Patients were randomly selected from the archives of the Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. PATIENTS AND MAIN OUTCOME MEASURES: In total, 76 patients with complex CHD and 80 with mild CHD (age range, 17-32 years) completed a self-reported questionnaire on employment and handicaps, with reference data available (response rate, 70%). RESULTS: In the study groups, 45 (59%) of 76 patients with complex CHD had a paid job compared with 61 (76%) of 80 patients with mild CHD. Patients older than 25 years with complex CHD had significantly lower job participation (64%) than the general population (83%). Multiple logistic regression showed that type of CHD and level of education were significantly and independently related to job participation (odds ratio, 4.8; 99% confidence interval, 1.2-19.6; and odds ratio, 4.7; 99% confidence interval, 1.3-17.2, respectively). Of the 76 patients with complex CHD, 42 (55%) experienced disease-related career problems, in contrast to only 1 patient with mild CHD. Both CHD groups had more job-related mobility handicaps than did the reference group. However, in the mild CHD group, handicaps could be attributed to additional noncardiac diseases. CONCLUSIONS: Patients with complex CHD have reduced job participation compared with patients with mild CHD and the general population. Many receive disability benefits or experience career problems or job handicaps. Career counseling focusing on physical abilities and level of education may help prevent or reduce these job-related problems.     

How Does the Functional Mobility Scale Relate to Capacity-Based Measures of Walking Ability in Children and Youth with Cerebral Palsy?

ABSTRACT

This study examined the relationship between walking performance rated on the Functional Mobility Scale (FMS) and measures of walking capacity in children with cerebral palsy (CP). A total of 143 participants with spastic CP (GMFCS levels I to III) were rated on the FMS and had assessment of self-selected walking speed (WS), fast 1 minute walk test (1MWT) and six minute walk test (6MWT). For each FMS distance, children rated 6 had significantly better 6MWT than children scored 5; children rated FMS 2, 3, or 4 had lower walking capacity measures but were not clearly distinguishable from each other. The 6MWT was an independent predictor of variation in FMS score, accounting for 20% to 27% of the variance across the three FMS distances. While walking capacity impacts on community mobility in children with CP much of the variance remains unexplained, suggesting that other factors play an important role.     

Disability in children with myelomeningocele. A Nordic study

This study was carried out to evaluate the medical impairments and disability among 527 children with myelomeningocele living in four Nordic countries. The information was extracted form questionnaires and data based on case records. The level of intellectual functioning was fairly high with 76% within normal range (IQ greater than 90). Shunt operations were performed in 68% of the children and 1/3 had had four or more shunt revisions. Twenty-three per cent walked without support. Bladder incontinence was found in 60% of the children whereas 4% were entirely continent. Intermittent catheterisation was used in 36% with higher frequency among girls (48%) than boys (24%). We found a higher incidence of bacteriuria among those who used catheterisation than among those who used manual compression. Bowel control was experienced by 112 (21%) of the children. The overall disability is classified according to Lorbber's 5-graded handicap scale and Lagergren's 3-graded scale. According to Lagergren's scale, 70% suffered form severe overall disability.     

The Carter Neurocognitive Assessment for children with severely compromised expressive language and motor skills

In this paper, different means of assessing cognitive development in children with severe impairments in both their expressive language and their motor skills are reviewed. A range of techniques are considered, including traditional cognitive tests and behavioral and physiological measures, but these techniques are generally impractical and minimally informative when it comes to assessing children with both motor and speech impairments. Electrophysiological measures show some promise for the future, but are currently inadequate for wide-ranging cognitive assessment. Development of the Carter Neurocognitive Assessment (CNA) is described. The CNA is appropriate for use in clinical and research settings and was designed to minimalize the impact of severely impaired motor skills and expressive language on performance. The CNA is intended to itemize and quantify a range of skills reflecting a cognitive level up to approximately 18 to 24 months in four areas: Social Awareness, Visual Attention, Auditory Comprehension and Vocal Communication. The use of the CNA to assess the performance and developmental growth of eight children with Holoprosencephaly (HPE), a midline developmental brain malformation, is described. The CNA is a useful tool for the assessment of children with severely compromised motor and verbal skills and has provided a more positive view of the cognitive potential of children with severe handicaps, such as the sample of children with HPE, than that presented in the past.     

Disability in Children with Myelomeningocele A Nordic Study

ABSTRACT. This study was carried out to evaluate the medical impairments and disability among 527 children with myelomeningocele living in four Nordic countries. The information was extracted from questionnaires and data based on case records. The level of intellectual functioning was fairly high with 76% within normal range (IQ>90). Shunt operations were performed in 68% of the children and 1/3 had had four or more shunt revisions. Twenty-three per cent walked without support. Bladder incontinence was found in 60% of the children whereas 4% were entirely continent. Intermittent catheterisation was used in 36% with higher frequency among girls (48%) than boys (24%). We found a higher incidence of bacteriuria among those who used catheterisation than among those who used manual compression. Bowel control was experienced by 112 (21%) of the children. The overall disability is classified according to Lorber's 5-graded handicap scale and Lagergren's 3-graded scale. According to Lagergren's scale, 70% suffered from severe overall disability.     

Meeting the Information Needs of Families of Children with Chronic Health Conditions

ABSTRACT

The aim of this study was to examine which child and family characteristics at the child's age of 2 years are determinants of development of self-care and mobility activities over a period of 2 years in young children with cerebral palsy (CP). Longitudinal data of 92 children, representing all levels of the Gross Motor Function Classification System (GMFCS), were analyzed. Children's self-care and mobility activities were assessed with the Functional Skills Scale of the Pediatric Evaluation of Disability Inventory. Development of self-care and mobility activities was related to several child determinants but no family determinants. GMFCS, type of CP, intellectual capacity, and epilepsy were related to the development of self-care and mobility activities, while manual ability and spasticity were related to development of mobility activities. Multivariate analysis indicated that GMFCS and intellectual capacity were the strongest determinants of development of self-care activities, and GMFCS was the strongest determinant of development of mobility activities. The change in self-care and mobility activities was less favorable in severely affected children with severe disability. Knowledge of GMFCS level and intellectual capacity is important in anticipating change over time and goal setting in young children with CP.     

Psychological problems and quality of life in children with thalassemia

Objective The study is aimed to assess psychological problems and quality of life (QOL) in children with thalassemia. Methods Thirty-nine children (8–16 yr) with transfusion dependent thalassemia attending day care services for blood transfusion were assessed for psychological problems using the Childhood Psychopathology Measurement Schedule and QOL was assessed using the EQ-5D. Results Forty-four percent of the children had psychological problems and 74% had a poor QOL. Anxiety-related symptoms (67%), emotional problems, particularly depression (62%), and conduct problems (49%) were the main findings. The children were most likely to report impaired QOL due to severe difficulties in pain/discomfort (64%) dimension, followed by depression and mobility problems of equal severity (33%). The side effects of chelation were an independent predictor of psychological problems and impaired QOL. Also psychological problems were a significant predictor of impaired QOL. Conclusion The recognition and management of the psychological problems that accompany chronic physical illnesses including thalassemia would optimize treatment outcomes and QOL.     

Psychological distress in mothers of disabled children

We examined the impact of child disability on psychological distress in mothers, by comparing scores on two indexes of psychological distress of 369 mothers of children with cystic fibrosis, cerebral palsy, myelodysplasia, or multiple physical handicaps, with those of 456 mothers from a randomly selected sample of families ("control" subjects). Mothers of disabled children scored significantly higher than control subjects on both indexes of psychological distress. This finding persisted when the mothers' education, family income, and racial composition were controlled for. Type of disability, ie, the diagnostic classification of the disabled children, was unrelated to the mother's level of psychological distress. In contrast, the disabled child's dependence on others in daily activities had a significant effect on both measures of psychological distress; the more dependent the child, the greater was the mother's distress.     

Causes of visual impairment in children: a study of 3,210 cases

PURPOSE: To determine causes of visual impairment in children at the Low Vision Service of the Ophthalmic Clinic at the University of S?o Paulo and at the Brazilian Association for the Visually Impaired People (Laramara), located in S?o Paulo, Brazil. PATIENTS AND METHODS: This study evaluated 3,210 visually impaired children (49% female, 51% male; average age, 5.9 years). Visual impairment was present in 57% (visually impaired group) and 43% presented another associated disability (multiple disability group). RESULTS: The main causes of visual impairment in the visually impaired group were toxoplasmic macular retinochoroiditis (20.7%), retinal dystrophies (12.2%), retinopathy of prematurity (11.8%), ocular malformation (11.6%), congenital glaucoma (10.8%), optic atrophy (9.7%), and congenital cataracts (7.1%). The main causes of visual impairment in the multiple disability group were optic atrophy (37.7%), cortical visual impairment (19.7%), toxoplasmic macular retinochoroiditis (8.6%), retinopathy of prematurity (7.6%), ocular malformation (6.8%), congenital cataracts (6.1%), and degenerative disorders of the retina and macula (4.8%). The retina was the most frequently affected anatomic site in the visually impaired group (49.2%) and the optic nerve in the multiple disability group (39%). CONCLUSION: Primary, secondary, and tertiary prevention efforts for childhood blindness and visual rehabilitation must be considered in Latin America.     

Rehabilitation of cerebral palsy in a developing country: the need for comprehensive assessment

Records of 100 children with cerebral palsy from rural India attending a cerebral palsy clinic were analysed to determine the frequency of associated handicaps and to evaluate whether appropriate intervention had been instituted for these handicaps. All the children underwent speech, hearing, psychological, ophthalmologic, neurological, physical, functional and orthopaedic evaluations on their visit to the clinic. History was elicited from the parents of the patients to identify which of the disabilities were recognized either by them or their primary care physician, and whether any intervention had been instituted. The mean age of the patients was 6.9 years. Eighty-two per cent of the children had one or more disabilities apart from locomotor disabilities, visual defects being the commonest (54%). Fifty-four per cent of the children had more than one associated disability. Although 43% of the patients had been referred by a primary care physician, one or more associated disabilities had not been recognized and epilepsy was the only associated problem that had been treated prior to the evaluation. The study emphasizes the need for comprehensive evaluation of all children with cerebral palsy to enable proper rehabilitation.