Aim The aim is to study access to intrathecal baclofen (ITB) for children with cerebral palsy (CP... more Aim The aim is to study access to intrathecal baclofen (ITB) for children with cerebral palsy (CP) in Europe, as an indicator of access to advanced care. Methods Surveys were sent to CP registers, clinical networks, and pump manufacturers. Enquiries were made about ITB treatment in children born in 1990 to 2005 by sex, CP type, level of gross motor function classification system (GMFCS) and age at the start of treatment. Access to ITB was related to the country's gross domestic product (GDP) and % GDP spent on health. Results In 2011 population-based data from Sweden, Norway, England, Portugal, Slovenia, and Denmark showed that 114 (3.4%) of 3,398 children with CP were treated with ITB, varying from 0.4 to 4.7% between centers. The majority of the children were at GMFCS levels IV-V and had bilateral spastic CP. In Sweden, dyskinetic CP was the most commonly treated subtype. Boys were more often treated with ITB than girls (p = 0.014). ITB was reported to be available for childre...
Surveillance registers monitor the prevalence of cerebral palsy and the severity of resulting imp... more Surveillance registers monitor the prevalence of cerebral palsy and the severity of resulting impairments across time and place. The motor disorders of cerebral palsy can affect children's speech production and limit their intelligibility. We describe the development of a scale to classify children's speech performance for use in cerebral palsy surveillance registers, and its reliability across raters and across time. Speech and language therapists, other healthcare professionals and parents classified the speech of 139 children with cerebral palsy (85 boys, 54 girls; mean age 6.03 years, SD 1.09) from observation and previous knowledge of the children. Another group of health professionals rated children's speech from information in their medical notes. With the exception of parents, raters reclassified children's speech at least four weeks after their initial classification. Raters were asked to rate how easy the scale was to use and how well the scale described the child's speech production using Likert scales. Inter-rater reliability was moderate to substantial (k >.58 for all comparisons). Test-retest reliability was substantial to almost perfect for all groups (k >.68). Over 74% of raters found the scale easy or very easy to use; 66% of parents 2 and over 70% of health care professionals judged the scale to describe children's speech well or very well. We conclude that the Viking Speech Scale is a reliable tool to describe the speech performance of children with cerebral palsy, which can be applied through direct observation of children or through case note review.
Developmental Medicine & Child Neurology, 2011
The aim of this study was to describe trends in prevalence, subtypes, and severity among children... more The aim of this study was to describe trends in prevalence, subtypes, and severity among children with cerebral palsy (CP) born moderately preterm (MPT; (gestational age 32-36wks) or at moderately low birthweight (MLBW; 1500-2499g) in Europe. METHOD We conducted trend analyses of data from 903 children with CP born between 1980 and 1998 who were MPT (gestational age 32-36wks), taken from 11 registers in the Surveillance of Cerebral Palsy in Europe database and from 1835 children with CP who were born at moderately low birthweight (1500-2499g), taken from 14 registers in the Surveillance of Cerebral Palsy in Europe database. RESULTS The overall annual prevalence of CP in children born MPT varied between 12.2 (95% confidence interval [CI] 8.5-17.1) per 1000 live births in 1983 and 4.5 (95% CI 3.2-6.3) per 1000 in 1997. There was a significant decrease in the prevalence over time adjusted for register, with an annual change in prevalence of)3% (95% CI)5 to)2%). This was due to a decrease in the prevalence of bilateral spastic CP (annual change)5%; 95% CI)7 to)3%). INTERPRETATION There was a trend towards a decrease in the prevalence of CP among children born MPT, but no difference in prevalence among children born at MLBW. Both results may represent an improvement in perinatal and neonatal care.
High throughput sequencing is discovering many likely causative genetic variants in individuals w... more High throughput sequencing is discovering many likely causative genetic variants in individuals with cerebral palsy. Some investigators have suggested that this changes the clinical diagnosis of cerebral palsy and that these individuals should be removed from this diagnostic category. Cerebral palsy is a neurodevelopmental disorder diagnosed on clinical signs, not etiology. All
Developmental medicine and child neurology, Apr 1, 2018
To systematically review evidence for pharmacological/neurosurgical interventions for managing dy... more To systematically review evidence for pharmacological/neurosurgical interventions for managing dystonia in individuals with cerebral palsy (CP) to inform a care pathway. Searches included studies with a minimum of five participants with dystonia in CP receiving oral baclofen, benzodiazepines (clonazepam, diazepam, lorazepam), clonidine, gabapentin, levodopa, trihexyphenidyl, botulinum toxin, intrathecal baclofen (ITB), or deep brain stimulation (DBS). Evidence was classified according to American Academy of Neurology guidelines. Twenty-eight articles underwent data extraction: one levodopa, five trihexyphenidyl, three botulinum toxin, six ITB, and 13 DBS studies. No articles for oral baclofen, benzodiazepines, clonidine, or gabapentin met the inclusion criteria. Evidence for reducing dystonia was level C (possibly effective) for ITB and DBS; level C (possibly ineffective) for trihexyphenidyl; and level U (inadequate data) for botulinum toxin. For dystonia reduction, ITB and DBS are ...
To describe the birth prevalence, temporal trends, and clinical outcomes of twins, triplets, or q... more To describe the birth prevalence, temporal trends, and clinical outcomes of twins, triplets, or quadruplets with cerebral palsy (CP).
Time trends for cerebral palsy (CP) prevalence in children born ≥ 2500 g vary across studies and ... more Time trends for cerebral palsy (CP) prevalence in children born ≥ 2500 g vary across studies and scarce data exist on trends by subtype of CP. The objective of this study was to describe changes in prevalence of CP in infants born ≥ 2500 g between 1980 and 1998 in Europe. Data were collated from the SCPE (Surveillance of Cerebral Palsy in Europe collaboration) common database. Poisson regression was used to test for change in prevalence over time. Birth year and register effects were explored and trends in prevalence were estimated by CP subtype and severity. Four thousand and two children with CP and birthweight ≥ 2500 g were recorded in 15 population based-registers. The overall prevalence of CP was 1.16 per 1000 live births (0.88−1.48) in 1980 and 0.99 (CI, 0.80−1.20) in 1998. The trend was not significant (P=.14), except in two registers. However, there were significant changes in the prevalence of spastic CP subtypes, with a decrease in the bilateral spastic form (P <.001), and an increase in the unilateral spastic form (P=.004). There was a concurrent reduction in neonatal mortality from 1.7 (CI, 1.4−2.1) to 0.9 (CI, 0.7−1.1) per 1000 live births with birthweight ≥ 2500 g. In conclusion, for children born with birthweight ≥ 2500 g, the prevalence of CP in Europe was stable in spite of changes by subtype and a significant decrease in neonatal mortality.
Developmental Medicine & Child Neurology, 2013
Determining inclusion/exclusion criteria for cerebral palsy (CP) surveillance is challenging. The... more Determining inclusion/exclusion criteria for cerebral palsy (CP) surveillance is challenging. The aims of this paper were to (1) define inclusion/exclusion criteria that have been adopted uniformly by surveillance programmes and identify where consensus is still elusive, and (2) provide an updated list of the consensus concerning CP inclusion/exclusion when a syndrome/disorder is diagnosed.
Low birth weight and high placental weight are associated with increased cardiovascular morbidity... more Low birth weight and high placental weight are associated with increased cardiovascular morbidity and mortality. Children born after hypertensive pregnancies have higher blood pressure than children born after normotensive pregnancies. Hypertension in pregnancy is considered a major risk factor for intrauterine growth retardation. The present study describes size at birth and perinatal characteristics in children born after hypertensive pregnancies. During five consecutive years 17,000 deliveries took place in the well defined geographic area of eastern Göteborg. Hypertension in pregnancy complicated 261 pregnancies. For comparison 260 normotensive pregnancies, matched for maternal age and time of delivery, were used. Standard deviation score for birth weight and length were calculated according to reference standards for birth weight (BWSDS) and length (BLSDS) based on all Swedish births 1977-1981 (n = 475,588). Children born after hypertensive pregnancies had lower birth weight, were shorter and had a shorter gestational period as compared with children born after normotensive pregnancies. Head circumference and placental weight did not differ and there were no significant differences in BWSDS. It is concluded that hypertensive pregnancies are characterized by lower birth weight and shorter gestational period. However, intrauterine growth retardation is not a general characteristic of hypertension in pregnancy.
To describe speech ability in a population-based study of children with cerebral palsy (CP), in r... more To describe speech ability in a population-based study of children with cerebral palsy (CP), in relation to CP subtype, motor function, cognitive level and neuroimaging findings. Methods: A retrospective chart review of 129 children (66 girls, 63 boys) with CP, born in 1999-2002, was carried out. Speech ability and background information, such as type of CP, motor function, cognitive level and neuroimaging data, were collected and analysed. Results: Speech disorders were found in 21% of the children and were present in all types of CP. Forty-one per cent of the children with speech disorders also had mental retardation, and 42% were able to walk independently. A further 32% of the children were nonverbal, and maldevelopment and basal ganglia lesions were most common in this group. The remaining 47% had no speech disorders, and this group was most likely to display white matter lesions of immaturity. Conclusion: More than half of the children in this CP cohort had a speech disorder (21%) or were nonverbal (32%). Speech ability was related to the type of CP, gross motor function, the presence of mental retardation and the localization of brain maldevelopment and lesions. Neuroimaging results differed between the three speech ability groups.
Aim: This is the ninth report from the western-Swedish study of the prevalence and origin of cere... more 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 postneonatally 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%.
The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is t... more The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is the tenth report from the western Swedish study. A population-based study covering 85,737 live births in the area in 1999-2002. Birth characteristics and neuroimaging findings were recorded, prevalence of CP was calculated and aetiology was analysed. CP was found in 186 children. The crude prevalence was 2.18 per 1000 live births. The gestational age-specific prevalence for &lt;28 gestational weeks was 55.6 per 1000 live births, whereas it was 43.7 for 28-31 weeks, 6.1 for 32-36 weeks and 1.43 per 1000 for &gt;36 weeks. There was a female majority among children born at term and a male predominance in children born preterm. Hemiplegia accounted for 38%, diplegia for 32%, tetraplegia for 7%, whereas 17% had dyskinetic CP and 5% ataxia. Neuroimaging showed white-matter lesions in 31% and cortical/subcortical lesions in 29%. The aetiology was considered to be prenatal in 36%, peri/neonatal in 42%, whereas it remained unclassified in 21%. The decrease in CP prevalence observed since the 1980s had ceased. An increase in children born at term and in dyskinetic CP was found. In children born before 28 weeks of gestation, the prevalence decreased significantly. White-matter and cortical/subcortical lesions dominated on neuroimaging.
Acta Obstetricia et Gynecologica Scandinavica, 2011
Objective. To provide an overview of current research on risk factors for cerebral palsy (CP) in ... more Objective. To provide an overview of current research on risk factors for cerebral palsy (CP) in children born at term and hypothesize how new findings can affect the content of the CP registers worldwide. Design. A systematic search in PubMed for original articles, published from 2000 to 2010, regarding risk factors for CP in children born at term was conducted. Methods. Full text review was made of 266 articles. Main Outcome Measures. Factors from the prenatal, perinatal and neonatal period considered as possible contributors to the causal pathway to CP in children born at term were regarded as risk factors. Results. Sixty-two articles met the criteria for an original report on risk factors for CP in children born at term. Perinatal adverse events, including stroke, were the focus of most publications, followed by genetic studies. Malformations, infections, perinatal adverse events and multiple gestation were risk factors associated with CP. The evidence regarding, for example, thrombophilic factors and non-CNS abnormalities was inconsistent. Conclusions. Information on maternal and neonatal infections, umbilical cord blood gases at birth, mode of delivery and placental status should be collected in a standardized way in CP registers. Information on social factors, such as education level, family income and area of residence, is also of importance. More research is needed to understand the risk factors of CP and specifically how they relate to causal pathways of cerebral palsy.
Cans C; SCPE collaboration. Archives of disease in childhood. 2009 Dec;94(12):921-6 Background: D... more Cans C; SCPE collaboration. Archives of disease in childhood. 2009 Dec;94(12):921-6 Background: Dyskinetic CP is characterized by involuntary changes in muscle tone, and involuntary positions and movements.
To investigate the link between infection-related risk factors for cerebral palsy subtypes in chi... more To investigate the link between infection-related risk factors for cerebral palsy subtypes in children born at term. 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. 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. 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. II.
Physical & Occupational Therapy In Pediatrics, 2014
The Bimanual Fine Motor Function (BFMF) is currently the principal classification of hand functio... more The Bimanual Fine Motor Function (BFMF) is currently the principal classification of hand function recorded by the Surveillance of Cerebral Palsy in Europe (SCPE) register. The BFMF is used in a number of epidemiological studies, but has not yet been validated. Aims: To examine aspects of construct and content validity of the BFMF. Methods and Results: Construct validity of the BFMF was assessed by comparison with the Manual Ability Classification System (MACS) using register-based data from 539 children born 1999-2003 (304 boys; 4-12 years). The high correlation with the MACS (Spearman's rho = 0.89, CI: 0.86-0.91, p<.001) supports construct validity of the BFMF. The content of the BFMF was appraised through literature review, and by using the ICF-CY as a framework to compare the BFMF and MACS. The items hold, grasp and manipulate were found to be relevant to describe increasingly advanced fine motor abilities in children with CP, but the description of the BFMF does not state whether it is a classification of fine motor capacity or performance. Conclusion: Our results suggest that the BFMF may provide complementary information to the MACS regarding fine motor function and actual use of the hands, particularly if used as a classification of fine motor capacity.
A girl with Hurler disease (MPS IH) underwent allogeneic stem cell transplantation at 13 months o... more A girl with Hurler disease (MPS IH) underwent allogeneic stem cell transplantation at 13 months of age with her one HLA-B antigen mismatch mother as donor. The procedure was complicated by cerebral hemorrhage and a ventricular-peritoneal shunt device was inserted. Mild GVH reactions were rapidly reversed. One year after transplantation ventriculitis was suspected and the shunt was replaced by a ventricular drainage catheter. Antibiotics had no effect and graft-versus-host disease (GVHD) was diagnosed. All symptoms were reversed by prednisolone and cyclosporine. Increased albumin and pleocytosis in the cerebrospinal fluid (CSF) normalized concomitantly. Electron microscopy of the CSF sediment showed debris consisting of numerous complex aggregates of thin lamellae and electron dense fragments with a tight lamellar texture. Biochemical analysis of the CSF sediment proved that the debris contained galactosylceramide and sulfatide. The electron microscopic and biochemical findings were interpreted to represent stripping of central myelin as a result of subacute GVHD in the central nervous system and its desquamation from the brain parenchyma into the ventricular CSF through the post-hemorrhage defect. From reversal of the GVHD at 2 years of age until follow-up at 10 years of age the clinical condition remained stable with no recurrence or deterioration.
Aim The aim is to study access to intrathecal baclofen (ITB) for children with cerebral palsy (CP... more Aim The aim is to study access to intrathecal baclofen (ITB) for children with cerebral palsy (CP) in Europe, as an indicator of access to advanced care. Methods Surveys were sent to CP registers, clinical networks, and pump manufacturers. Enquiries were made about ITB treatment in children born in 1990 to 2005 by sex, CP type, level of gross motor function classification system (GMFCS) and age at the start of treatment. Access to ITB was related to the country's gross domestic product (GDP) and % GDP spent on health. Results In 2011 population-based data from Sweden, Norway, England, Portugal, Slovenia, and Denmark showed that 114 (3.4%) of 3,398 children with CP were treated with ITB, varying from 0.4 to 4.7% between centers. The majority of the children were at GMFCS levels IV-V and had bilateral spastic CP. In Sweden, dyskinetic CP was the most commonly treated subtype. Boys were more often treated with ITB than girls (p = 0.014). ITB was reported to be available for childre...
Surveillance registers monitor the prevalence of cerebral palsy and the severity of resulting imp... more Surveillance registers monitor the prevalence of cerebral palsy and the severity of resulting impairments across time and place. The motor disorders of cerebral palsy can affect children's speech production and limit their intelligibility. We describe the development of a scale to classify children's speech performance for use in cerebral palsy surveillance registers, and its reliability across raters and across time. Speech and language therapists, other healthcare professionals and parents classified the speech of 139 children with cerebral palsy (85 boys, 54 girls; mean age 6.03 years, SD 1.09) from observation and previous knowledge of the children. Another group of health professionals rated children's speech from information in their medical notes. With the exception of parents, raters reclassified children's speech at least four weeks after their initial classification. Raters were asked to rate how easy the scale was to use and how well the scale described the child's speech production using Likert scales. Inter-rater reliability was moderate to substantial (k >.58 for all comparisons). Test-retest reliability was substantial to almost perfect for all groups (k >.68). Over 74% of raters found the scale easy or very easy to use; 66% of parents 2 and over 70% of health care professionals judged the scale to describe children's speech well or very well. We conclude that the Viking Speech Scale is a reliable tool to describe the speech performance of children with cerebral palsy, which can be applied through direct observation of children or through case note review.
Developmental Medicine & Child Neurology, 2011
The aim of this study was to describe trends in prevalence, subtypes, and severity among children... more The aim of this study was to describe trends in prevalence, subtypes, and severity among children with cerebral palsy (CP) born moderately preterm (MPT; (gestational age 32-36wks) or at moderately low birthweight (MLBW; 1500-2499g) in Europe. METHOD We conducted trend analyses of data from 903 children with CP born between 1980 and 1998 who were MPT (gestational age 32-36wks), taken from 11 registers in the Surveillance of Cerebral Palsy in Europe database and from 1835 children with CP who were born at moderately low birthweight (1500-2499g), taken from 14 registers in the Surveillance of Cerebral Palsy in Europe database. RESULTS The overall annual prevalence of CP in children born MPT varied between 12.2 (95% confidence interval [CI] 8.5-17.1) per 1000 live births in 1983 and 4.5 (95% CI 3.2-6.3) per 1000 in 1997. There was a significant decrease in the prevalence over time adjusted for register, with an annual change in prevalence of)3% (95% CI)5 to)2%). This was due to a decrease in the prevalence of bilateral spastic CP (annual change)5%; 95% CI)7 to)3%). INTERPRETATION There was a trend towards a decrease in the prevalence of CP among children born MPT, but no difference in prevalence among children born at MLBW. Both results may represent an improvement in perinatal and neonatal care.
High throughput sequencing is discovering many likely causative genetic variants in individuals w... more High throughput sequencing is discovering many likely causative genetic variants in individuals with cerebral palsy. Some investigators have suggested that this changes the clinical diagnosis of cerebral palsy and that these individuals should be removed from this diagnostic category. Cerebral palsy is a neurodevelopmental disorder diagnosed on clinical signs, not etiology. All
Developmental medicine and child neurology, Apr 1, 2018
To systematically review evidence for pharmacological/neurosurgical interventions for managing dy... more To systematically review evidence for pharmacological/neurosurgical interventions for managing dystonia in individuals with cerebral palsy (CP) to inform a care pathway. Searches included studies with a minimum of five participants with dystonia in CP receiving oral baclofen, benzodiazepines (clonazepam, diazepam, lorazepam), clonidine, gabapentin, levodopa, trihexyphenidyl, botulinum toxin, intrathecal baclofen (ITB), or deep brain stimulation (DBS). Evidence was classified according to American Academy of Neurology guidelines. Twenty-eight articles underwent data extraction: one levodopa, five trihexyphenidyl, three botulinum toxin, six ITB, and 13 DBS studies. No articles for oral baclofen, benzodiazepines, clonidine, or gabapentin met the inclusion criteria. Evidence for reducing dystonia was level C (possibly effective) for ITB and DBS; level C (possibly ineffective) for trihexyphenidyl; and level U (inadequate data) for botulinum toxin. For dystonia reduction, ITB and DBS are ...
To describe the birth prevalence, temporal trends, and clinical outcomes of twins, triplets, or q... more To describe the birth prevalence, temporal trends, and clinical outcomes of twins, triplets, or quadruplets with cerebral palsy (CP).
Time trends for cerebral palsy (CP) prevalence in children born ≥ 2500 g vary across studies and ... more Time trends for cerebral palsy (CP) prevalence in children born ≥ 2500 g vary across studies and scarce data exist on trends by subtype of CP. The objective of this study was to describe changes in prevalence of CP in infants born ≥ 2500 g between 1980 and 1998 in Europe. Data were collated from the SCPE (Surveillance of Cerebral Palsy in Europe collaboration) common database. Poisson regression was used to test for change in prevalence over time. Birth year and register effects were explored and trends in prevalence were estimated by CP subtype and severity. Four thousand and two children with CP and birthweight ≥ 2500 g were recorded in 15 population based-registers. The overall prevalence of CP was 1.16 per 1000 live births (0.88−1.48) in 1980 and 0.99 (CI, 0.80−1.20) in 1998. The trend was not significant (P=.14), except in two registers. However, there were significant changes in the prevalence of spastic CP subtypes, with a decrease in the bilateral spastic form (P <.001), and an increase in the unilateral spastic form (P=.004). There was a concurrent reduction in neonatal mortality from 1.7 (CI, 1.4−2.1) to 0.9 (CI, 0.7−1.1) per 1000 live births with birthweight ≥ 2500 g. In conclusion, for children born with birthweight ≥ 2500 g, the prevalence of CP in Europe was stable in spite of changes by subtype and a significant decrease in neonatal mortality.
Developmental Medicine & Child Neurology, 2013
Determining inclusion/exclusion criteria for cerebral palsy (CP) surveillance is challenging. The... more Determining inclusion/exclusion criteria for cerebral palsy (CP) surveillance is challenging. The aims of this paper were to (1) define inclusion/exclusion criteria that have been adopted uniformly by surveillance programmes and identify where consensus is still elusive, and (2) provide an updated list of the consensus concerning CP inclusion/exclusion when a syndrome/disorder is diagnosed.
Low birth weight and high placental weight are associated with increased cardiovascular morbidity... more Low birth weight and high placental weight are associated with increased cardiovascular morbidity and mortality. Children born after hypertensive pregnancies have higher blood pressure than children born after normotensive pregnancies. Hypertension in pregnancy is considered a major risk factor for intrauterine growth retardation. The present study describes size at birth and perinatal characteristics in children born after hypertensive pregnancies. During five consecutive years 17,000 deliveries took place in the well defined geographic area of eastern Göteborg. Hypertension in pregnancy complicated 261 pregnancies. For comparison 260 normotensive pregnancies, matched for maternal age and time of delivery, were used. Standard deviation score for birth weight and length were calculated according to reference standards for birth weight (BWSDS) and length (BLSDS) based on all Swedish births 1977-1981 (n = 475,588). Children born after hypertensive pregnancies had lower birth weight, were shorter and had a shorter gestational period as compared with children born after normotensive pregnancies. Head circumference and placental weight did not differ and there were no significant differences in BWSDS. It is concluded that hypertensive pregnancies are characterized by lower birth weight and shorter gestational period. However, intrauterine growth retardation is not a general characteristic of hypertension in pregnancy.
To describe speech ability in a population-based study of children with cerebral palsy (CP), in r... more To describe speech ability in a population-based study of children with cerebral palsy (CP), in relation to CP subtype, motor function, cognitive level and neuroimaging findings. Methods: A retrospective chart review of 129 children (66 girls, 63 boys) with CP, born in 1999-2002, was carried out. Speech ability and background information, such as type of CP, motor function, cognitive level and neuroimaging data, were collected and analysed. Results: Speech disorders were found in 21% of the children and were present in all types of CP. Forty-one per cent of the children with speech disorders also had mental retardation, and 42% were able to walk independently. A further 32% of the children were nonverbal, and maldevelopment and basal ganglia lesions were most common in this group. The remaining 47% had no speech disorders, and this group was most likely to display white matter lesions of immaturity. Conclusion: More than half of the children in this CP cohort had a speech disorder (21%) or were nonverbal (32%). Speech ability was related to the type of CP, gross motor function, the presence of mental retardation and the localization of brain maldevelopment and lesions. Neuroimaging results differed between the three speech ability groups.
Aim: This is the ninth report from the western-Swedish study of the prevalence and origin of cere... more 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 postneonatally 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%.
The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is t... more The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is the tenth report from the western Swedish study. A population-based study covering 85,737 live births in the area in 1999-2002. Birth characteristics and neuroimaging findings were recorded, prevalence of CP was calculated and aetiology was analysed. CP was found in 186 children. The crude prevalence was 2.18 per 1000 live births. The gestational age-specific prevalence for &lt;28 gestational weeks was 55.6 per 1000 live births, whereas it was 43.7 for 28-31 weeks, 6.1 for 32-36 weeks and 1.43 per 1000 for &gt;36 weeks. There was a female majority among children born at term and a male predominance in children born preterm. Hemiplegia accounted for 38%, diplegia for 32%, tetraplegia for 7%, whereas 17% had dyskinetic CP and 5% ataxia. Neuroimaging showed white-matter lesions in 31% and cortical/subcortical lesions in 29%. The aetiology was considered to be prenatal in 36%, peri/neonatal in 42%, whereas it remained unclassified in 21%. The decrease in CP prevalence observed since the 1980s had ceased. An increase in children born at term and in dyskinetic CP was found. In children born before 28 weeks of gestation, the prevalence decreased significantly. White-matter and cortical/subcortical lesions dominated on neuroimaging.
Acta Obstetricia et Gynecologica Scandinavica, 2011
Objective. To provide an overview of current research on risk factors for cerebral palsy (CP) in ... more Objective. To provide an overview of current research on risk factors for cerebral palsy (CP) in children born at term and hypothesize how new findings can affect the content of the CP registers worldwide. Design. A systematic search in PubMed for original articles, published from 2000 to 2010, regarding risk factors for CP in children born at term was conducted. Methods. Full text review was made of 266 articles. Main Outcome Measures. Factors from the prenatal, perinatal and neonatal period considered as possible contributors to the causal pathway to CP in children born at term were regarded as risk factors. Results. Sixty-two articles met the criteria for an original report on risk factors for CP in children born at term. Perinatal adverse events, including stroke, were the focus of most publications, followed by genetic studies. Malformations, infections, perinatal adverse events and multiple gestation were risk factors associated with CP. The evidence regarding, for example, thrombophilic factors and non-CNS abnormalities was inconsistent. Conclusions. Information on maternal and neonatal infections, umbilical cord blood gases at birth, mode of delivery and placental status should be collected in a standardized way in CP registers. Information on social factors, such as education level, family income and area of residence, is also of importance. More research is needed to understand the risk factors of CP and specifically how they relate to causal pathways of cerebral palsy.
Cans C; SCPE collaboration. Archives of disease in childhood. 2009 Dec;94(12):921-6 Background: D... more Cans C; SCPE collaboration. Archives of disease in childhood. 2009 Dec;94(12):921-6 Background: Dyskinetic CP is characterized by involuntary changes in muscle tone, and involuntary positions and movements.
To investigate the link between infection-related risk factors for cerebral palsy subtypes in chi... more To investigate the link between infection-related risk factors for cerebral palsy subtypes in children born at term. 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. 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. 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. II.
Physical & Occupational Therapy In Pediatrics, 2014
The Bimanual Fine Motor Function (BFMF) is currently the principal classification of hand functio... more The Bimanual Fine Motor Function (BFMF) is currently the principal classification of hand function recorded by the Surveillance of Cerebral Palsy in Europe (SCPE) register. The BFMF is used in a number of epidemiological studies, but has not yet been validated. Aims: To examine aspects of construct and content validity of the BFMF. Methods and Results: Construct validity of the BFMF was assessed by comparison with the Manual Ability Classification System (MACS) using register-based data from 539 children born 1999-2003 (304 boys; 4-12 years). The high correlation with the MACS (Spearman's rho = 0.89, CI: 0.86-0.91, p<.001) supports construct validity of the BFMF. The content of the BFMF was appraised through literature review, and by using the ICF-CY as a framework to compare the BFMF and MACS. The items hold, grasp and manipulate were found to be relevant to describe increasingly advanced fine motor abilities in children with CP, but the description of the BFMF does not state whether it is a classification of fine motor capacity or performance. Conclusion: Our results suggest that the BFMF may provide complementary information to the MACS regarding fine motor function and actual use of the hands, particularly if used as a classification of fine motor capacity.
A girl with Hurler disease (MPS IH) underwent allogeneic stem cell transplantation at 13 months o... more A girl with Hurler disease (MPS IH) underwent allogeneic stem cell transplantation at 13 months of age with her one HLA-B antigen mismatch mother as donor. The procedure was complicated by cerebral hemorrhage and a ventricular-peritoneal shunt device was inserted. Mild GVH reactions were rapidly reversed. One year after transplantation ventriculitis was suspected and the shunt was replaced by a ventricular drainage catheter. Antibiotics had no effect and graft-versus-host disease (GVHD) was diagnosed. All symptoms were reversed by prednisolone and cyclosporine. Increased albumin and pleocytosis in the cerebrospinal fluid (CSF) normalized concomitantly. Electron microscopy of the CSF sediment showed debris consisting of numerous complex aggregates of thin lamellae and electron dense fragments with a tight lamellar texture. Biochemical analysis of the CSF sediment proved that the debris contained galactosylceramide and sulfatide. The electron microscopic and biochemical findings were interpreted to represent stripping of central myelin as a result of subacute GVHD in the central nervous system and its desquamation from the brain parenchyma into the ventricular CSF through the post-hemorrhage defect. From reversal of the GVHD at 2 years of age until follow-up at 10 years of age the clinical condition remained stable with no recurrence or deterioration.
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Papers by K. Himmelmann