Cerebral palsy (CP) is a clinical description for a group of motor disorders that are heterogeneo... more Cerebral palsy (CP) is a clinical description for a group of motor disorders that are heterogeneous with respect to causes, symptoms and severity. A diagnosis of CP cannot usually be made at birth and in some cases may be delayed until 2-3 years of age. This limits opportunities for early intervention that could otherwise improve long-term outcomes. CP has been recorded in monozygotic twins discordant for the disorder, indicating a potential role of non-genetic factors such as intrauterine infection, hypoxia-ischaemia, haemorrhage and thrombosis. The aim of this exploratory study was to utilise the discordant monozygotic twin model to understand and measure epigenetic changes associated with the development of CP. We performed a genome-wide analysis of DNA methylation using the Illumina Infinium Human Methylation 450 BeadChip array with DNA from newborn blood spots of 15 monozygotic twin pairs who later became discordant for CP. Quality control and data preprocessing were undertaken...
Developmental medicine and child neurology, Nov 8, 2017
The aims of this study were to estimate the proportion of emergency department presentations attr... more The aims of this study were to estimate the proportion of emergency department presentations attributable to children with cerebral palsy (CP), investigate the frequency of emergency department presentations in a CP cohort, and compare emergency department presentations among children with CP with those of other children. This was a retrospective cohort study. The Victorian Cerebral Palsy Register was linked to the Victorian Emergency Minimum Dataset. Data on emergency department presentations for the CP cohort occurring between 2007 and 2014 and population control data were obtained. The CP cohort (n=1748) had 7015 emergency department presentations during the 7-year period, accounting for 0.4% of the 1.69 million age-specific presentations during that time. The number of annual presentations per 1000 children rose with increasing CP severity. Compared with presentations among the general population, higher proportions of presentations among the CP cohort were preceded by ambulance...
The goal of this study was to describe the prevalence, syndromes, and evolution of seizure disord... more The goal of this study was to describe the prevalence, syndromes, and evolution of seizure disorders in children with cerebral palsy (CP) due to white matter injury (WMI). For this population-based cohort study, brain MRI scans and medical records were reviewed in children in the Victorian Cerebral Palsy Register born between 1999 and 2006 recorded as having WMI. Children were excluded if they had features of an undiagnosed syndrome, associated cortical malformation or injury, or no medical contact in the preceding year. Included were 166 children with CP and isolated WMI due to presumed vascular insufficiency or hemorrhage; 87 were born preterm. Seizure and CP details were obtained from medical records and interviews, and EEG recordings were reviewed. Forty-one children (25%) had seizures beyond the neonatal period. Four children had West syndrome, which resolved with treatment. Thirteen children had febrile seizures that they outgrew. Thirty children had focal epilepsy with seizur...
Developmental medicine and child neurology, May 30, 2016
The overall aim was to investigate the feasibility and utility of linking a cerebral palsy (CP) r... more The overall aim was to investigate the feasibility and utility of linking a cerebral palsy (CP) register to an administrative data set for health services research purposes. We sought to compare CP hospital admissions to general childhood population admissions, and identify factors associated with type and frequency of admissions in a CP cohort. The CP register for Victoria, Australia was linked to the state's hospital admissions database. Data pertaining to the admissions of a CP cohort (n=1748) that took place between 2007 and 2014 were extracted. Population data were also obtained. Overall, 80% of the CP cohort (n=1401) had at least admission between 2007 and 2014, accounting for 11 012 admissions or 1.5% of all admissions in their age group. Compared to general population admissions, CP admissions were more costly and more likely to be elective (66% vs 57%; p<0.001), medical (71% vs 57%; p<0.001), and to take place in metropolitan hospitals (92% vs 78%; p<0.001). In...
Physical & Occupational Therapy In Pediatrics, 2012
To examine the inter-rater reliability of the Communication Function Classification System (CFCS)... more To examine the inter-rater reliability of the Communication Function Classification System (CFCS), Bimanual Fine Motor Function (BFMF), Surveillance of Cerebral Palsy in Europe (SCPE) classification tree, and Gross Motor Function Classification System (GMFCS) in children with cerebral palsy (CP) and periventricular white matter injury (PWMI) aged 4-11 years. Twenty children were assessed by two raters using the four tools, in addition parents undertook ratings on the Manual Ability Classification System (MACS). Kappa statistics were used to calculate the level of agreement between raters&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; classifications. Participants comprised 12 males and 8 females with CP and PWMI, mean age 8 years 1 month (standard deviation 2 years 3 months). Inter-rater reliability across the four tools was 0.98 (CFCS, BFMF, and GMFCS) and 0.84 (SCPE). These findings suggest that these four tools are reasonably robust to inter-rater variability supporting their routine use along with the MACS in clinical and research applications.
Journal of Intellectual & Developmental Disability, 2013
Functional abilities and social outcomes of young adults with cerebral palsy (CP) are relatively ... more Functional abilities and social outcomes of young adults with cerebral palsy (CP) are relatively under-researched. Improvements in paediatric care have extended the expectation of achieving adulthood to 90%. Young adults aged 20-30 years with CP (n = 335) were compared to a population-based control group (n = 2,152) of the same age. Motor function, self-care abilities, educational level, and social outcomes were determined by questionnaire. Half the study group walked independently, but only 35.5% were independent in self-care. In comparison to their peers without disability, the study group&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s highest educational level was lower (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; .0001), as were rates of employment (36.3% compared with 80%), they were more likely to be living with parents (80% compared with 21%), to be single, and to have limited financial resources. Young adults with CP are functionally and socially disadvantaged in contrast with their peers without disability. Self-care dependence, intellectual disability, and communication impairments contribute to these outcomes but are not solely responsible.
The Journal of Bone and Joint Surgery (American), 2006
Hip displacement is considered to be common in children with cerebral palsy but the reported inci... more Hip displacement is considered to be common in children with cerebral palsy but the reported incidence and the proposed risk factors vary widely. Knowledge regarding its overall incidence and associated risk factors can facilitate treatment of these children. An inception cohort was generated from the Victorian Cerebral Palsy Register for the birth years 1990 through 1992, inclusive, and multiple data sources pertaining to the cohort were reviewed during 2004. Gross motor function was assessed for each child and was graded according to the Gross Motor Function Classification System (GMFCS), which is a valid, reliable, five-level ordinal grading system. Hip displacement, defined as a migration percentage of &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;30%, was measured on an anteroposterior radiograph of the pelvis with use of a reliable technique. A full data set was obtained for 323 (86%) of 374 children in the Register for the birth years 1990 through 1992. The mean duration of follow-up was eleven years and eight months. The incidence of hip displacement for the entire birth cohort was 35%, and it showed a linear relationship with the level of gross motor function. The incidence of hip displacement was 0% for children with GMFCS level I and 90% for those with GMFCS level V. Compared with children with GMFCS level II, those with levels III, IV, and V had significantly higher relative risks of hip displacement (2.7, 4.6, and 5.9, respectively). Hip displacement is common in children with cerebral palsy, with an overall incidence of 35% found in this study. The risk of hip displacement is directly related to gross motor function as graded with the Gross Motor Function Classification System. This information may be important when assessing the risk of hip displacement for an individual child who has cerebral palsy, for counseling parents, and in the design of screening programs and resource allocation.
Cerebral palsy (CP) is a clinical description for a group of motor disorders that are heterogeneo... more Cerebral palsy (CP) is a clinical description for a group of motor disorders that are heterogeneous with respect to causes, symptoms and severity. A diagnosis of CP cannot usually be made at birth and in some cases may be delayed until 2-3 years of age. This limits opportunities for early intervention that could otherwise improve long-term outcomes. CP has been recorded in monozygotic twins discordant for the disorder, indicating a potential role of non-genetic factors such as intrauterine infection, hypoxia-ischaemia, haemorrhage and thrombosis. The aim of this exploratory study was to utilise the discordant monozygotic twin model to understand and measure epigenetic changes associated with the development of CP. We performed a genome-wide analysis of DNA methylation using the Illumina Infinium Human Methylation 450 BeadChip array with DNA from newborn blood spots of 15 monozygotic twin pairs who later became discordant for CP. Quality control and data preprocessing were undertaken...
Developmental medicine and child neurology, Nov 8, 2017
The aims of this study were to estimate the proportion of emergency department presentations attr... more The aims of this study were to estimate the proportion of emergency department presentations attributable to children with cerebral palsy (CP), investigate the frequency of emergency department presentations in a CP cohort, and compare emergency department presentations among children with CP with those of other children. This was a retrospective cohort study. The Victorian Cerebral Palsy Register was linked to the Victorian Emergency Minimum Dataset. Data on emergency department presentations for the CP cohort occurring between 2007 and 2014 and population control data were obtained. The CP cohort (n=1748) had 7015 emergency department presentations during the 7-year period, accounting for 0.4% of the 1.69 million age-specific presentations during that time. The number of annual presentations per 1000 children rose with increasing CP severity. Compared with presentations among the general population, higher proportions of presentations among the CP cohort were preceded by ambulance...
The goal of this study was to describe the prevalence, syndromes, and evolution of seizure disord... more The goal of this study was to describe the prevalence, syndromes, and evolution of seizure disorders in children with cerebral palsy (CP) due to white matter injury (WMI). For this population-based cohort study, brain MRI scans and medical records were reviewed in children in the Victorian Cerebral Palsy Register born between 1999 and 2006 recorded as having WMI. Children were excluded if they had features of an undiagnosed syndrome, associated cortical malformation or injury, or no medical contact in the preceding year. Included were 166 children with CP and isolated WMI due to presumed vascular insufficiency or hemorrhage; 87 were born preterm. Seizure and CP details were obtained from medical records and interviews, and EEG recordings were reviewed. Forty-one children (25%) had seizures beyond the neonatal period. Four children had West syndrome, which resolved with treatment. Thirteen children had febrile seizures that they outgrew. Thirty children had focal epilepsy with seizur...
Developmental medicine and child neurology, May 30, 2016
The overall aim was to investigate the feasibility and utility of linking a cerebral palsy (CP) r... more The overall aim was to investigate the feasibility and utility of linking a cerebral palsy (CP) register to an administrative data set for health services research purposes. We sought to compare CP hospital admissions to general childhood population admissions, and identify factors associated with type and frequency of admissions in a CP cohort. The CP register for Victoria, Australia was linked to the state's hospital admissions database. Data pertaining to the admissions of a CP cohort (n=1748) that took place between 2007 and 2014 were extracted. Population data were also obtained. Overall, 80% of the CP cohort (n=1401) had at least admission between 2007 and 2014, accounting for 11 012 admissions or 1.5% of all admissions in their age group. Compared to general population admissions, CP admissions were more costly and more likely to be elective (66% vs 57%; p<0.001), medical (71% vs 57%; p<0.001), and to take place in metropolitan hospitals (92% vs 78%; p<0.001). In...
Physical & Occupational Therapy In Pediatrics, 2012
To examine the inter-rater reliability of the Communication Function Classification System (CFCS)... more To examine the inter-rater reliability of the Communication Function Classification System (CFCS), Bimanual Fine Motor Function (BFMF), Surveillance of Cerebral Palsy in Europe (SCPE) classification tree, and Gross Motor Function Classification System (GMFCS) in children with cerebral palsy (CP) and periventricular white matter injury (PWMI) aged 4-11 years. Twenty children were assessed by two raters using the four tools, in addition parents undertook ratings on the Manual Ability Classification System (MACS). Kappa statistics were used to calculate the level of agreement between raters&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; classifications. Participants comprised 12 males and 8 females with CP and PWMI, mean age 8 years 1 month (standard deviation 2 years 3 months). Inter-rater reliability across the four tools was 0.98 (CFCS, BFMF, and GMFCS) and 0.84 (SCPE). These findings suggest that these four tools are reasonably robust to inter-rater variability supporting their routine use along with the MACS in clinical and research applications.
Journal of Intellectual & Developmental Disability, 2013
Functional abilities and social outcomes of young adults with cerebral palsy (CP) are relatively ... more Functional abilities and social outcomes of young adults with cerebral palsy (CP) are relatively under-researched. Improvements in paediatric care have extended the expectation of achieving adulthood to 90%. Young adults aged 20-30 years with CP (n = 335) were compared to a population-based control group (n = 2,152) of the same age. Motor function, self-care abilities, educational level, and social outcomes were determined by questionnaire. Half the study group walked independently, but only 35.5% were independent in self-care. In comparison to their peers without disability, the study group&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s highest educational level was lower (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; .0001), as were rates of employment (36.3% compared with 80%), they were more likely to be living with parents (80% compared with 21%), to be single, and to have limited financial resources. Young adults with CP are functionally and socially disadvantaged in contrast with their peers without disability. Self-care dependence, intellectual disability, and communication impairments contribute to these outcomes but are not solely responsible.
The Journal of Bone and Joint Surgery (American), 2006
Hip displacement is considered to be common in children with cerebral palsy but the reported inci... more Hip displacement is considered to be common in children with cerebral palsy but the reported incidence and the proposed risk factors vary widely. Knowledge regarding its overall incidence and associated risk factors can facilitate treatment of these children. An inception cohort was generated from the Victorian Cerebral Palsy Register for the birth years 1990 through 1992, inclusive, and multiple data sources pertaining to the cohort were reviewed during 2004. Gross motor function was assessed for each child and was graded according to the Gross Motor Function Classification System (GMFCS), which is a valid, reliable, five-level ordinal grading system. Hip displacement, defined as a migration percentage of &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;30%, was measured on an anteroposterior radiograph of the pelvis with use of a reliable technique. A full data set was obtained for 323 (86%) of 374 children in the Register for the birth years 1990 through 1992. The mean duration of follow-up was eleven years and eight months. The incidence of hip displacement for the entire birth cohort was 35%, and it showed a linear relationship with the level of gross motor function. The incidence of hip displacement was 0% for children with GMFCS level I and 90% for those with GMFCS level V. Compared with children with GMFCS level II, those with levels III, IV, and V had significantly higher relative risks of hip displacement (2.7, 4.6, and 5.9, respectively). Hip displacement is common in children with cerebral palsy, with an overall incidence of 35% found in this study. The risk of hip displacement is directly related to gross motor function as graded with the Gross Motor Function Classification System. This information may be important when assessing the risk of hip displacement for an individual child who has cerebral palsy, for counseling parents, and in the design of screening programs and resource allocation.
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