Hayley Smithers-Sheedy

What constitutes cerebral palsy in the twenty-first century?

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.

A special supplement: findings from the Australian Cerebral Palsy Register, birth years 1993 to 2006

To briefly outline the strengths and limitations of cerebral palsy (CP) registers, and to report on findings of the Australian Cerebral Palsy Register (ACPR) pertaining to a population cohort of children with CP.

Congenital cytomegalovirus is associated with severe forms of cerebral palsy and female sex in a retrospective population-based study

Congenital cytomegalovirus (cCMV) infection can result in poor outcomes including cerebral palsy (CP). The aim of this study was to describe the incidence and comorbidities of CP reported to the Australian Cerebral Palsy Register (ACPR) as attributed to cCMV infection.

An international survey of cerebral palsy registers and surveillance systems

To describe cerebral palsy (CP) surveillance programmes and identify similarities and differences in governance and funding, aims and scope, definition, inclusion/exclusion criteria, ascertainment and data collection, to enhance the potential for research collaboration.

Congenital Cytomegalovirus among Children with Cerebral Palsy

Objectives To determine the proportion of children with cerebral palsy (CP) and cytomegalovirus (CMV) DNA detected retrospectively in their newborn screening cards (NBSC), to compare the proportion of children with CMV DNA in their NBSC across spastic subtypes of CP, and to compare the sex and other characteristics of children with CP and CMV detected on their NSBC with those in whom CMV DNA was not detected. Study design Retrospective observational study. Data were extracted from patient records on children with CP (birth years 1996‐2014) from 2 Australian state CP registers and state‐wide paediatric rehabilitation services with consent. NBSCs were retrospectively analyzed for CMV DNA by nested polymerase chain reaction (PCR) using primers against gB. Positive samples were validated using real time PCR for CMV UL83. Results Of 401 children recruited, 323 (80.5%) had an available NBSC. Of these, 31 (9.6%; 95% CI, 6.8‐13.3) tested positive for CMV DNA by nested PCR for CMV gB, of whom 28 (8.7%; 95% CI, 6.1‐12.2) also had CMV DNA detected by real‐time PCR for CMV UL83. Detection of CMV DNA was significantly associated with epilepsy, but not with clinical or epidemiologic characteristics, including sex and pattern of spasticity. Conclusions CMV viremia in the newborn period, indicating congenital CMV infection, is highly prevalent among children with CP. Further research is needed to investigate the mechanisms and contribution of congenital CMV to the causal pathways to CP.

Cytomegalovirus-related childhood mortality in Australia 1999-2011.

Cytomegalovirus (CMV) is an important cause of congenital infection, which can result in neonatal deaths or contribute to deaths in later childhood. Post‐natally acquired CMV is a less common cause of disease and mortality, and only in preterm infants or immunocompromised children. Here we sought to describe CMV as a direct or secondary contributor to childhood mortality in Australia.

Congenital anomalies in cerebral palsy: where to from here?

Proportions of cases of cerebral palsy (CP) with congenital anomalies recorded in Australian CP registers range from 15% to 40%. The anomalies seen in CP are extremely variable. We have identified that CP registers often do not have quality data on congenital anomalies, necessitating linkage with congenital anomaly registers. However, a lack of unified processes and definitions in congenital anomaly registers and data collections means that linkages are complex, need to be carefully planned, and limitations acknowledged. Historically in CP research, congenital anomalies have been classified by International Classification of Disease codes, then combined into brain and other major and minor anomalies. Systems have been developed to classify congenital anomalies into aetiologically related groups, but such a classification has yet to be trialled in CP. It is anticipated that primary prevention of a small proportion of cases of CP is possible through the primary prevention of congenital anomalies, especially those due to teratogens. Owing to the anticipated low prevalence of each subgroup, global collaboration will be required to further these lines of enquiry.

Risk factors for dental caries among children with cerebral palsy in a low-resource setting.

To describe the oral health status and investigate factors affecting dental caries experience among children with cerebral palsy (CP) in rural Bangladesh.

Change in residential remoteness during the first 5 years of life in an Australian cerebral palsy cohort

To determine if families of children with cerebral palsy living in Australia move to less remote areas between birth and 5 years.

Declining prevalence of cerebral palsy in Europe: good news?

This outstanding paper from Sellier et al. is proof positive (if any were needed) that cerebral palsy (CP) registers have particular research utility as they allow analysis of trends over multiple time points both within and across geographical regions. Furthermore, these data sets (which are often substantial in size) are particularly useful when a research question requires stratification of a population to consider specific subgroups of children. To increase the power of these studies further, pooling multiple registers’ data using a standardized methodology is both costeffective and efficient. The Surveillance of Cerebral Palsy in Europe (SCPE) network is an excellent example of such a partnership between registers. Rates of CP in developed countries have been reported as remaining relatively stable over recent decades, with small variations noted during the 2000s. These were postulated to reflect a stabilization or a slight reduction in prevalence of CP following increases observed during the 1970s and 1980s that accompanied increased survival amongst preterm infants. Where reductions in prevalence amongst subgroups of CP have been observed (e.g. children born extremely preterm), the small sub-group frequencies have been insufficient to alter the overall rate of CP. In this context, this well-executed and thoughtful study from researchers from the SCPE network is of particular interest. Sellier et al. used records from 20 CP registers to examine the prevalence of CP by birthweight over time (1980–2003). Their observations include a reduction in the prevalence of CP amongst children in both moderate and very low birthweight subgroups, a reduction in severity, and a reduction in CP prevalence overall. It is interesting to speculate as to whether these findings might herald the beginning of further observed reductions in the prevalence and severity of CP in other developed regions of the world. Certainly this study provides support for cautious optimism. Whilst live births rather than neonatal survivors were used as denominators, the decrease in CP was noted despite a sizeable increase in neonatal survival. Additionally, similar findings in relation to the reduced prevalence of moderate-to-severe CP reported here have been observed in cohorts from the Netherlands and Victoria, Australia over similar time periods. Some caution should however be maintained. In recent reports, research from Australia and Western Sweden noted the rates of CP amongst children born extremely preterm, and found reductions in prevalence rates over the latter years of the 1990s and early to mid2000s. However, in the most recent data from the western Sweden 2003 to 2006 cohort, the prevalence of CP within this group increased once more. The next cohort of data from SCPE and other CP registers in developed regions will assist in determining whether these observed trends in declining CP prevalence and moderateto-severe CP amongst specific subgroups and in the population overall are sustained. In the interim we remain cautiously optimistic. The authors have also discussed a number of practice changes in neonatal care and emerging developments in the neuroimaging findings amongst preterm infants over time. This should encourage the continuing search for further neuroprotective strategies and is a timely reminder that CP registers have an important part to play in evaluating the impact of practice changes at the population level. Clinicians, researchers, and CP register groups have the opportunity to collaborate and to ensure population measures are incorporated into the long-term evaluation of new practice changes. In this way we will have better means of establishing the impact of these therapies on the prevalence and severity of CP into the future.