Randomized placebo-controlled crossover trial of memantine in children with epileptic encephalopathy.

Memantine is an N-methyl-D-aspartate receptor antagonist, approved for dementia treatment. There is limited evidence of memantine showing benefit for paediatric neurodevelopmental phenotypes, but no randomized placebo-controlled trials in children with developmental and epileptic encephalopathy. In this randomized double-blind placebo-controlled crossover trial (Trial registration: https://clinicaltrials.gov/ct2/show/NCT03779672), patients with developmental and epileptic encephalopathy received memantine and placebo, each for a 6-week period separated by a 2-week washout phase. Electroencephalography, seizure diary, patient caregivers' global impression, serum inflammatory markers and neuropsychological evaluation were performed at baseline and after each treatment phase. The primary outcome measure was classification as a 'responder', defined as ≥2 of: >50% seizure frequency reduction, electroencephalography improvement, caregiver clinical impression improvement or clear neuropsychological testing improvement. Thirty-one patients (13 females) enrolled. Two patients withdrew prior to initiating medication and two (twins) had to be removed from analysis. Of the remaining 27 patients, nine (33%) were classified as responders to memantine versus two (7%) in the placebo group (P < 0.02). Electroencephalography improvement was seen in eight patients on memantine compared to two on placebo (P < 0.04). Seizure improvement was observed in eight patients on memantine and two on placebo (P < 0.04). Caregivers reported overall clinical improvement in 10 patients on memantine compared to seven on placebo (not significant). Statistical analysis of neuropsychological evaluation suggested improvements in symptoms of attention-deficit hyperactivity disorder and autism. Memantine is a safe and effective treatment for children with developmental and epileptic encephalopathy, having the potential to improve both seizure control and cognitive function.

Physicians' attitudes towards ethical issues and end-of-life decision-making for pediatric patients with unresponsive wakefulness syndrome: An international survey.

AIM: We examined physicians' perspectives on the mental capabilities of pediatric patients with unresponsive wakefulness syndrome (UWS) and their attitudes towards limiting life-sustaining treatment (LST) in an international context. METHOD: A questionnaire survey was conducted among 267 neuropediatricians, practicing in 65 countries. Comparisons were made according to the Human Development Index (HDI) of the countries. The Idler Index of Religiosity was applied to determine religiosity. RESULTS: Participants from countries with a very high HDI were generally more favorable to limiting LST (p < 0.001), specifically cardiopulmonary resuscitation (p = 0.021), intubation/ventilation (p = 0.014), hemodialysis/hemofiltration (p < 0.001), and antibiotic therapy (p < 0.001). Treatment costs that were too high had a weaker influence on their decisions (p < 0.001). Participants who found it never ethically justifiable to limit LST had a higher mean Idler Index of private (p = 0.001) and general (p = 0.020) religiosity and were less satisfied with treatment decisions (p < 0.001) and the communication during the process (p = 0.016). INTERPRETATION: The perspectives towards limiting LST for pediatric patients with UWS are markedly different between physicians from countries with very high and lower HDIs.

Perinatal arterial ischemic stroke and periventricular venous infarction in infants with unilateral cerebral palsy.

AIM: To explore clinical factors associated with perinatal arterial ischemic stroke (AIS) and periventricular venous infarction (PVI) in infants who develop unilateral cerebral palsy (CP). METHOD: This was a case-control study. Data current to 2019 was extracted from the Canadian Cerebral Palsy Registry (CCPR). Cases were infants born at term with confirmed unilateral CP. Magnetic resonance images were stratified by expert review of reports as definitive perinatal stroke (AIS or PVI). Controls with common data elements were recruited from a population-based study in Alberta. Multivariable regression analyses were performed to estimate associations expressed as odds ratios with 95% confidence intervals. RESULTS: Of 2093 cases from the CCPR, 662 had unilateral CP, of whom 299 (45%) had perinatal stroke: AIS 169 (57%) and PVI 130 (43%). Median age at diagnosis for AIS was 11.9 months (interquartile range: 6.2-25.7mo; range 0.17-104.1mo), and 58.6% were male. Median age at diagnosis for PVI was 25.3 months (interquartile range: 14.5-38mo, range 0.7-114.7mo) and 57.7% were male. Independent associations for both AIS and PVI on multivariable analysis were chorioamnionitis, illicit drug exposure, diabetes, gestational age, and maternal age. Variables associated with AIS alone were low Apgar score and prolonged rupture of membranes. Variables associated with PVI alone were small for gestational age and primigravida. INTERPRETATION: Controlled analysis of disease-specific unilateral CP may offer unique perspectives on its pathophysiology. Acute intrapartum factors are mainly associated with AIS, while in utero factors are associated with PVI.

Epilepsy in children with cerebral palsy: a data linkage study.

AIM: To compare the prevalence of epilepsy in children with cerebral palsy (CP) to peer controls and their differences in healthcare utilization. METHOD: The Quebec CP registry was linked to the provincial administrative health database. Two CP cohorts were identified from the registry (n=302, 168 males, 1y 2mo-14y) and administrative data (n=370, 221 males, 2y 2mo-14y). A control cohort (n=6040, 3340 males, 10-14y) was matched by age, sex, and region to the CP registry cohort. Administrative data algorithms were used to define epilepsy cases. Data on hospitalizations and emergency department presentations were obtained. RESULTS: Using the most sensitive epilepsy definition, prevalence was 42.05% in the CP registry, 43.24% in the CP administrative data, and 1.39% in controls. Prevalence rose with increasing Gross Motor Function Classification System level. Children with CP and epilepsy had increased number and length of hospitalizations and emergency department presentations compared to children with CP or epilepsy alone. Epilepsy accounted for approximately 5% of emergency department presentations and 10% of hospitalizations in children with epilepsy, with and without CP. INTERPRETATION: Children with CP have an increased risk of epilepsy compared to their peers. Children with CP and coexisting epilepsy represent a unique subset with complex developmental disability and increased healthcare service utilization.

Stability of the Gross Motor Function Classification System over time in children with cerebral palsy.

AIM: To assess the stability of the Gross Motor Functional Classification System (GMFCS) in children with cerebral palsy (CP) from time of preliminary diagnosis (~2 years of age) to time of diagnosis (~5 years of age), and to examine factors associated with reclassification. METHOD: We conducted a longitudinal study using a sample from the Canadian CP Registry. Stability was analysed by using the percentage of agreement between timepoints and a weighted prevalence and bias adjusted kappa statistic. Univariate and multivariate logistic regressions were performed to identify variables associated with reclassification. RESULTS: The study included 1670 children (857 males, 713 females) with a mean age of 11 years 4 months (SD 4 years, range 3 years 5 months-20 years 1 month) at time of data extraction (3rd September 2019), of which 1435 (85.9%) maintained a stable GMFCS, with a weighted kappa of 0.91 (95% confidence interval 0.89-0.92). Univariate logistic regression showed that initial GMFCS level, CP subtype, and the presence of cognitive impairment were associated with the likelihood of change in the GMFCS level (p < 0.1). In the multivariate analysis, however, the likelihood was associated with initial GMFCS level only (odds ratio 7.10-8.88, p < 0.00). INTERPRETATION: The GMFCS has good stability in early childhood. For the majority of children, it is predictive of their long-term motor function. WHAT THIS PAPER ADDS: The Gross Motor Function Classification System (GMFCS) rating in early childhood is stable over time. There is no directionality in the reclassification of the GMFCS. The initial GMFCS level was related to the likelihood of change in follow-up GMFCS level.

Cerebral palsy in Canadian Indigenous children.

AIM: To determine whether inequities in health outcomes for Indigenous Canadians are also present in cerebral palsy (CP) by comparing CP profiles between Indigenous and non-Indigenous children. METHOD: Using the Canadian Cerebral Palsy Registry, we conducted a cross-sectional study. CP motor subtype, gross motor severity, comorbidities, perinatal adversity, preterm birth, and parental education were compared between 94 Indigenous (53 males, 41 females) and 1555 non-Indigenous (891 males, 664 females) children (all >5y). Multivariate analysis was done to analyze adverse CP factors, defined as CP gross motor severity and comorbidities. CP etiologies, either prenatal/perinatal or postnatal, were also compared. RESULTS: Indigenous children with CP have higher odds of having low parental education (odds ratio [OR] 6.15, 95% confidence interval [CI] 3.36-11.3) and comorbidities (OR 4.46, 95% CI 1.62-12.3), especially cognitive (OR 4.52, 95% CI 2.27-9.05), communication (OR 2.66, 95% CI 1.54-4.61), and feeding (OR 2.25, 95% CI 1.33-3.83) impairment. Indigenous children also have higher CP gross motor severity (p=0.03). Indigenous children are also more likely to have non-accidental head injury (n=4; OR 8.18, 95% CI 1.86-36.0) as the cause of their postnatal CP. INTERPRETATION: Indigenous populations have worse health outcomes as a result of intergenerational impacts of colonization. Our study shows that Indigenous children with CP have increased comorbidities and higher CP gross motor severity, reinforcing the need for a multidisciplinary approach to management. Furthermore, targeted prevention programs against preventable causes of CP, such as non-accidental head injury, may be beneficial. WHAT THIS PAPER ADDS: Indigenous children with cerebral palsy (CP) have more severe motor impairment and more comorbidities. Non-accidental head injury is a significant cause of postnatal CP.

Hospitalizations in School-Aged Children with Cerebral Palsy and Population-Based Controls.

OBJECTIVE: To compare hospitalizations among children with cerebral palsy (CP) and healthy controls and to identify factors associated with hospitalizations in children with CP. METHODS: This retrospective cohort study linked data from a provincial CP Registry and administrative health databases. The CP cohort was comprised of children born from 1999 to 2002. Age, sex, and region-matched controls were identified from administrative health databases. Mean differences, relative risk (RR), and 95% confidence intervals (CIs) were calculated. RESULTS: A total of 301 children with CP were linked to administrative health data and matched to 6040 controls. Mean hospitalizations per child during the study period were higher in children with CP compared to controls (raw mean difference (RMD) 5.0 95% CI 4.7 to 5.2) with longer length of stay (RMD 2.8 95% CI 1.8 to 3.8) and number of diagnoses per hospitalization (RMD 1.6 95% CI 1.4 to 1.8). Increased risk of hospitalization was observed in non-ambulant children with CP (RR 1.12 95% CI 1.01 to 1.22) compared to ambulant children and among those with spastic tri/quadriplegic CP compared to other CP subtypes (RR 1.15, 95% CI 1.05 to 1.27). Feeding difficulties (RR 1.20 95% CI 1.13 to 1.27), cortical visual (RR 1.22 95% CI 1.13 to 1.32), cognitive (RR 1.16 95% CI 1.04 to 1.30), and communication impairment (RR 1.26 95% CI 1.10 to 1.44) were associated with increased hospitalizations. CONCLUSIONS: Children with CP face more frequent, longer hospital stays than peers, especially those with a more severe CP profile. Coordinated interdisciplinary care is needed in school-aged children with CP and medical complexity.

Emergency Department Use in Children with Cerebral Palsy: A Data Linkage Study.

OBJECTIVE: To describe the pattern of emergency department (ED) consultations in children with cerebral palsy (CP) compared to controls and factors predictive of ED consultations. METHODS: This retrospective cohort study linked data from the Registre de la paralysie cérébrale du Québec (REPACQ) and provincial administrative health databases. The CP cohort was comprised of children enrolled in REPACQ born between 1999 and 2002. REPACQ covers 6 of 17 Quebec health administrative regions. Region-, age-, and gender-matched controls were identified from administrative health databases in a 20:1 ratio. The primary outcome was high use of ED services (≥4 ED visits during the study period). Relative risk (RR) and 95% confidence interval (CI) were calculated. RESULTS: In total, 301 children with CP were linked to administrative data and 6040 peer controls were selected. Ninety-two percent (92%) of the CP cohort had at least one ED visit in the study period, compared to 74% among controls (RR 1.24, 95% CI 1.19-1.28). Children with CP were more likely than their peers to have high ED use (RR 1.40; 95% CI 1.30-1.52). Factors predictive of high ED use were comorbid epilepsy (RR 1.23; 95% CI 1.04-1.46) and severity of motor impairment (RR 1.14; 95% CI 0.95-1.37). CONCLUSION: Children with CP are more likely to present to the ED than their peers, resulting in increased use of ED services. Coordinated care with improved access to same-day evaluations could decrease ED use. Health system factors and barriers should be investigated to ensure optimal and appropriate use of ED services.

Complementary and Alternative Therapy Use in Children with Cerebral Palsy.

OBJECTIVE: To describe complementary and alternative medicine (CAM) use amongst children with cerebral palsy (CP) in Canada and to identify factors associated with CAM use. METHODS: We conducted a cross-sectional study, utilising data from the Canadian CP Registry. We explored the association between CAM use and regional, socioeconomic and CP phenotypic variables, and parental perception of the family-centredness of clinical care using the Measures of Process of Care-56 (MPOC-56). Chi-square analyses were performed, and odds ratios (OR) and 95% confidence intervals (CI) were obtained. Mann-Whitney U tests were used to compare MPOC-56 scores between CAM users and non-CAM users. RESULTS: The study sample consisted of 313 families of which 27% reported CAM use in the past year. Children with CP using CAM were more likely to reside in Western Canada (OR 3.3, 95% CI 1.6-6.7), live in a two-parent household (OR 3.5, 95% CI 1.5-8.4), have an ataxic/hypotonic or dyskinetic CP subtype (OR 3.0, 95% CI 1.5-6.1) and have a greater motor impairment (OR 2.8, 95% CI 1.7-4.9). MPOC-56 subscale scores were not significantly associated with CAM use. CONCLUSION: Physicians need to be aware of existing CAM therapies, the level of evidence supporting their efficacy (beneficence), their associated risks of adverse events (non-maleficence) and enable fair access to care that may be of benefit to each child.

Look Around Me: Environmental and Socio-Economic Factors Related to Community Participation for Children with Cerebral Palsy in Québec.

Aims: This study aimed at gaining a deeper understanding of the environmental and socio-economic factors affecting participation outcomes in community and leisure activities for children with disabilities, as well as the trajectories of participation for these children to promote their health and guarantee their rights are respected.Methods: A participatory action research (PAR) approach and linear regression analysis were employed to identify contextual factors associated with the community participation of children with cerebral palsy (CP) living in Quebec, Canada. Stakeholders engaged through the entire research process supported the development of questionnaires, data collection, analysis and interpretation of results.Results: Neighborhood outings were ranked among the most practiced activities by children with CP. Only in a few cases (9%) did children participate in more than two types of activities outside of school. Factors limiting children's participation were predominantly extrinsic in origin, including financial burden and lack of information about existing opportunities.Conclusions: There is a serious need for communities and local governments to inform parents about available resources, programs and policies that can support their child's participation. Rehabilitation professionals could partner with stakeholders to inform the development of public policies that target the identified barriers and promote children's integration and fulfillment.

Death after Birth Asphyxia in the Cooling Era.

In asphyxiated newborn infants treated with hypothermia, 31 of 50 (62%) deaths occurred in unstable infants electively extubated before completing hypothermia treatment. Later deaths occurred after consultation with palliative care (13/19) or clinical ethics (6/19) services, suggesting these decisions were challenging and required support, particularly if nutrition and hydration were withdrawn (n = 4).

Current Referral Practices for Diagnosis and Intervention for Children with Cerebral Palsy: A National Environmental Scan.

OBJECTIVES: To describe current physician referral practices with respect to age at referral to medical specialists for initial diagnosis of cerebral palsy (CP) and rehabilitation specialists for intervention and to identify factors associated with delayed referral. STUDY DESIGN: National environmental scan of 455 children diagnosed with CP who were born in Canada between 2008 and 2011, selected from 4 sites within the Canadian CP Registry (Edmonton, Calgary, Toronto, and Montreal). Two sources of information were used-children's medical charts and the population-based registry, which provided corresponding data for each child. Primary outcomes extracted from the charts were age at referral for diagnostic assessment, age at diagnosis, age at referral for rehabilitation services, and age at initial rehabilitation intervention. Twelve variables were explored as potential predictors. Descriptive statistics, bivariate analyses, and multiple linear regressions were conducted. RESULTS: Median age (in months) at referral for diagnostic assessment was 8 (mean: 12.7 ± 14.3), diagnosis 16 (mean: 18.9 ± 12.8), referral for rehabilitation services 10 (mean: 13.4 ± 13.5), and rehabilitation initiation 12 (mean: 15.9 ± 12.9). Lower maternal education, mild severity of motor dysfunction, type of CP, early discharge after birth, and region of residence explained between 20% and 32% of the variance in age at referral for assessment, diagnosis, referral for rehabilitation, and rehabilitation initiation. CONCLUSIONS: Findings suggest wide variability exists in the age at which young children with CP are referred to specialists for diagnosis and intervention. User-friendly tools are therefore needed to enhance early detection and referral strategies by primary care practitioners, to ensure early interventions to optimize developmental outcomes and enhance opportunities for neural repair at a younger age.

Losing a diagnosis of cerebral palsy: a comparison of variables at 2 and 5 years.

AIM: This study aims to identify characteristics at 2 years of age that differ between children with confirmed cerebral palsy (CP) and a non-CP diagnosis by 5 years of age. METHOD: This was a retrospective cohort analysis. A CP diagnosis may be considered a 'probable' diagnosis at 2 years, which is often 'confirmed' at 4 or 5 years, particularly in the context of CP registries. A total of 1683 children with a diagnosis of CP or probable CP at 2 years of age were identified from the Canadian Cerebral Palsy Registry, of whom 48 received a non-CP diagnosis at 5 years ('non-confirmed CP'). Perinatal adversity, preterm birth status, Gross Motor Function Classification System (GMFCS) level, presence of comorbidities, magnetic resonance imaging (MRI) findings, and initial CP motor type were compared between the two groups by univariate and logistic regression analyses. RESULTS: χ2 analysis and multivariate analysis both confirmed that children with a non-CP diagnosis by 5 years of age were more likely to have a normal MRI (χ2 odds ratio [OR]=7.8, 95% confidence interval [CI]=3.8-16.1; OR=5.4, 95% CI=2.4-12.5), ataxic-hypotonic (χ2 OR=10.1, 95% CI=4.9-21.2; OR=6.1, 95% CI=2.2-16.2) or dyskinetic CP (χ2 OR=2.7, 95% CI=1.2-5.9; OR=2.9, 95% CI=1.0-7.6), born at term (χ2 OR=3.7, 95% CI=1.7-8.0; OR=3.6, 95% CI=1.0-12.1), and lack perinatal adversity (χ2 OR=4.1, 95% CI=1.6-10.7; OR=3.4, 95% CI=1.0-11.7). INTERPRETATION: Normal MRI, ataxic-hypotonic or dyskinetic CP, lack of perinatal adversity, and term birth are associated with a higher odds of non-CP diagnosis by 5 years of age, thus potentially enhancing diagnostic work-up. WHAT THIS PAPER ADDS: Normal magnetic resonance imaging (MRI) at 2 years was associated with a non-cerebral palsy (CP) diagnosis by 5 years. Diagnosis of ataxic-hypotonic or dyskinetic CP motor subtype at 2 years was associated with a non-CP diagnosis by 5 years. Perinatal adversity and preterm birth were rarer with a non-CP diagnosis by 5 years.

OPORTUNIDADES DIAGNOSTICAS PERDIDAS DE PARÁLISIS CEREBRAL: UNA COMPARACIÓN DE VARIABLES A LOS 2 Y 5 AÑOS: OBJETIVO: Este estudio tiene como objetivo identificar características a los 2 años de edad que diferencian a los niños con parálisis cerebral (PC) confirmada y niños sin diagnóstico de PC a los 5 años de edad. MÉTODO: Se realizó un estudio de cohorte retrospectivo. Un diagnóstico de PC puede considerarse como "probable" a los 2 años, lo que es con frecuencia "confirmado" a los 4 o 5 años de edad, particularmente en el contexto de los registros de PC. Se identificaron un total de 1.683 niños con diagnóstico de PC o PC probable a los 2 años de edad en el Registro Canadiense de PC, de los cuales 48 recibieron un diagnóstico de no PC a los 5 años (PC no confirmado). Se compararon entre los 2 grupos: la adversidad perinatal, prematuridad, nivel de función motora en el sistema de Clasificación Motora Gruesa (GMFCS), la presencia de comorbilidades, hallazgos en imágenes de resonancia magnética nuclear (RMN), y tipo motor inicial de PC; usando un análisis univariado y de regresión logística. RESULTADOS: Tanto el análisis X2 como el multivariado confirmaron que los niños sin diagnóstico de PC a los 5 años de vida tenían más probabilidades de tener una RMN normal (X2 odds ratio [OR]= 7.8, intervalo de confianza 95 % [IC]= 3.8-16.1; OR=5.4, IC 95%= 2.4-12.5), ataxia (X2 OR=10.1, IC95%=4.9-21.2; OR=6.1, IC 95%=2.2-16.2) o PC disquinesia (X2 OR =2.7, IC 95%= 1.2-5.9; OR= 2.9, IC 95%= 1.0-7.6), nacidos a término (X2 OR=3.7, IC 95%= 1.7-8.0; OR=3.6, IC 95%=1.0-12.1), y niños sin adversidad perinatal (X2 OR=4.1, IC 95%=1.6-10.7; OR=3.4, IC 95% 1.0-11.7). INTERPRETACIÓN: La resonancia magnética normal, la PC atáxica o disquinesica, la falta de adversidad perinatal y el parto a término se asocian con una mayor probabilidad de no diagnóstico de PC a los 5 años de edad; esto refuerza el trabajo diagnóstico.

PERDENDO UM DIAGNÓSTICO DE PARALISIA CEREBRAL: UMA COMPARAÇÃO DE VARIÁVEIS AOS 2 E 5 ANOS: OBJETIVO: Este estudo visa identificar características aos 2 anos de idade que discriminem crianças com paralisia cerebral (PC) confirmada e um diagnóstico de não PC aos 5 anos de idade. MÉTODO: Esta foi uma análise de coorte retrospectiva. Um diagnóstico de PC pode ser considerado "provável" aos 2 anos, e é frequentemente confirmado aos 4 ou 5 anos, particularmente no contexto dos registros de PC. Um total de 1.683 crianças com diagnóstico de PC ou provável aos 2 anos de idade foram identificadas no Registro Canadense de Paralisia, dos quais 48 receberam um diagnóstico de não PC aos 5 anos (PC não confirmada). Adversidade perinatal, nascimento prematuro, nível I no sistema de classificação da função motora grossa (GMFCS), presença de comorbidades, achados de imagem por ressonância magnética (IRM), e tipo motor inicial de PC foram comparados entre os dois grupos por meio de análises de regressão univariada e logística. RESULTADOS: Tanto a análise de χ2 quanto a multivariada confirmaram que crianças com diagnóstico de não PC aos 5 anos de idade tinham maior probabilidade de uma IRM normal (χ2 odds ratio [OR]=7,8, intervalo de confiança [IC] 95%=3,8-16,1; OR=5,4, IC 95% =2,4-12,5), PC tipo atáxica (χ2 OR=10,1, IC 95%=4,9-21,2; OR=6,1, IC 95% =2,2-16,2) ou discinética (χ2 OR=2,7, IC 95%=1,2-5,9; OR=2,9, IC 95% =1,0-7,6), serem nascidas a termo (χ2 OR=3,7, IC 95% =1,7-8,0; OR=3,6, IC 95% =1,0-12,1), e não terem histórico de adversidade perinatal (χ2 OR=4,1, IC95% =1,6-10,7; OR=3,4, IC 95% =1,0-11,7). INTERPRETAÇÃO: IRM normal, PC tipo atáxica ou discinética, falta de adversidade perinatal, e nascimento a termo são associados com maior chance de um diagnóstico de PC não confirmado aos 5 anos de idade, o que potencialmente favorece o processo de diagnóstico.

International expert recommendations of clinical features to prompt referral for diagnostic assessment of cerebral palsy.

AIM: To establish international expert recommendations on clinical features to prompt referral for diagnostic assessment of cerebral palsy (CP). METHOD: An online Delphi survey was conducted with international experts in early identification and intervention for children with CP, to validate the results obtained in two previous consensus groups with Canadian content experts and knowledge users. We sent two rounds of questionnaires by e-mail. Participants rated their agreement using a 4-point Likert scale, along with optional open-ended questions for additional feedback. Additionally, a panel of experts and knowledge-users reviewed the results of each round and determined the content of subsequent surveys. RESULTS: Overall, there was high-level of agreement on: (1) six clinical features that should prompt referral for diagnosis; (2) two 'warning sign' features that warrant monitoring rather than immediate referral for diagnosis; and (3) five referral recommendations to other healthcare professionals to occur simultaneously with referral for diagnosis. INTERPRETATION: There was high agreement among international experts, suggesting that the features and referral recommendations proposed for primary care physicians for early detection of CP were broadly generalizable. These results will inform the content of educational tools to improve the early detection of CP in the primary care context. WHAT THIS PAPER ADDS: International experts provide strong agreement on clinical features to detect cerebral palsy. Consensus on clinical 'warning signs' to monitor over time. Referral recommendations from primary care to specialized health services are identified.

RECOMENDACIONES DE EXPERTOS INTERNACIONALES SOBRE CARACTERÍSTICAS CLÍNICAS PARA UNA DERIVACIÓN RÁPIDA PARA LA EVALUACIÓN DIAGNÓSTICA DE LA PARÁLISIS CEREBRAL: OBJETIVO: Establecer recomendaciones de expertos internacionales sobre características clínicas para iniciar derivación para la evaluación diagnóstica de la parálisis cerebral (PC). MÉTODO: Se realizó una encuesta online tipo Delphi con expertos en identificación e intervención temprana de niños con PC a fin de validar los resultados obtenidos en dos grupos de consenso realizados previamente en Canadá con expertos en contenidos y usuarios. Se enviaron dos rondas de cuestionarios por correo electrónico. Los participantes calificaron su acuerdo con un puntaje de 4 puntos en una escala Likert y con preguntas opcionales de respuesta abierta para comentarios adicionales. Además, un panel de expertos y usuarios revisaron los resultados de cada ronda y determinaron el contenido de las encuestas subsiguientes. RESULTADOS: En general, hubo un alto nivel de acuerdo sobre: ​​(1) seis características clínicas que requieren derivación rápida para el diagnóstico, (2) dos características de "señales de advertencia" que requieren monitoreo en lugar de referencia inmediata para el diagnóstico, y (3) cinco recomendaciones de referencia a otros profesionales de la salud que deben realizarse simultáneamente con la derivación para el diagnóstico. INTERPRETACIÓN: Hubo gran acuerdo entre los expertos internacionales, sugiriendo que las características y recomendaciones de referencia propuestas para los médicos de atención primaria para la detección de PC fue ampliamente generalizable. Estos resultados informarán el contenido de herramientas educativas para mejorar la detección precoz de PC en el contexto de atención primaria.

RECOMENDAÇÕES DE ESPECIALISTAS INTERNACIONAIS SOBRE ASPECTOS CLÍNICOS DISPARADORES DE ENCAMINHAMENTO PARA AVALIAÇÃO DIAGNÓSTICA EM PARALISIA CEREBRAL: OBJETIVO: Estabelecer recomendações de especialistas internacionais sobre os aspectos clínicos disparadores de encaminhamento para avaliação diagnóstica em paralisia cerebral (PC). MÉTODO: Um levantamento online internacional do tipo Delphi foi realizado com especialistas em identificação e intervenção precoce para crianças com PC, para validar os resultados obtidos em dois consensos prévios com especialistas no conteúdo e usuários canadenses. Enviamos duas rodadas de questionários por email. Os participantes pontuaram sua concordância usando uma escala Likert de 4 pontos, junto com questões abertas opcionais para informações adicionais. Além disso, um painel de especialistas e usuários revisaram os resultados de cada rodada, e determinaram o conteúdo das pesquisas subsequentes. RESULTADOS: Em geral, houve alto nível de concordância em: 1) seis aspectos clínicos que devem disparar encaminhamento para diagnóstico. 2) dois 'sinais de alerta' que merecem monitoramento mas não encaminhamento imediato para diagnóstico, e 3) cinco recomendações de encaminhamento para outros profissionais da saúde simultaneamente ao encaminhamento para diagnóstico. INTERPRETAÇÃO: Houve alta concordância entre especialistas internacionais, sugerindo que os aspectos e recomendações para encaminhamento propostos para médicos na atenção básica para a identificação precoce da PC foram amplamente generalizáveis. Estes resultados informarão o conteúdo de ferramentas educacionais para melhorar a detecção precoce de PC no contexto da atenção básica.

Use of consensus methods to determine the early clinical signs of cerebral palsy.

OBJECTIVES: To develop expert-informed content regarding the early motor attributes of cerebral palsy (CP) that should prompt physician referral for diagnostic assessment of CP, as well as concurrent referral recommendations. This content will be used in the creation of knowledge translation (KT) tools for primary care practitioners and parents. METHODS: Two nominal group processes were conducted with relevant stakeholders, representing Canadian 'content experts' and 'knowledge-users', using an integrated KT approach. RESULTS: Six attributes were identified that should prompt referral for diagnosis. If the child demonstrates: Early handedness <12 months; stiffness or tightness in the legs between 6 and 12 months; persistent fisting of the hands >4 months; persistent head-lag >4 months; inability to sit without support >9 months; any asymmetry in posture or movement. Five referral recommendations were agreed upon: Motor intervention specialist (physical therapy and/or occupational therapy) for ALL; speech-language pathology IF there is a communication delay; audiology IF there is parental or healthcare professional concern regarding a communication delay; functional vision specialist (e.g., optometrist or occupational therapist) IF there is a vision concern (e.g., not fixating, following, or tracking); feeding specialist (e.g., occupational therapist, speech-language pathologist) IF there are feeding difficulties (e.g., poor sucking, poor swallowing, choking, and/or not gaining weight). CONCLUSION: Rigorous consensus methods provided the initial evidence necessary to inform the content of tools to assist primary care providers in the early detection of CP. Results will be validated through a Delphi process with international experts, and user-friendly formats of this KT tool will be developed collaboratively with stakeholders.

Developmental outcomes in children with congenital cerebellar malformations.

AIM: Neurodevelopmental outcomes in children with congenital cerebellar malformations (CCMs) remain poorly defined. We aimed to assess whether specific neuroimaging features in CCM patients correlate with neurodevelopmental outcomes. METHOD: Hospital records and neuroimaging of 67 children with CCMs were systematically reviewed. Logistic regression analyses were used to assess associations between specific imaging features and neurodevelopmental outcomes. RESULTS: CCM categories were distributed as follows: 28 percent isolated vermis hypoplasia (n=19), 28 percent global cerebellar hypoplasia (n=19), 15 percent Dandy-Walker malformation (n=10), 13 percent pontocerebellar hypoplasia (PCH, n=9), 9 percent molar tooth malformation (n=6), 3 percent rhombencephalosynapsis (n=2), and 3 percent unilateral cerebellar malformation (n=2). Overall, 85 percent (55/65) of the cohort had global developmental delay (GDD). Intellectual disability was present in 61 percent (27/43) and autism spectrum disorder (ASD) in 12 percent (6/52). Adjusting for supratentorial malformations and presence of genetic findings, severe GDD was associated with cerebellar hypoplasia (p=0.049) and PCH (p=0.030), whereas children with vermis hypoplasia were less likely to have severe GDD (p=0.003). Presence of supratentorial abnormalities was not significantly associated with worse neurodevelopmental outcome but was associated with epilepsy. INTERPRETATION: Children with CCMs have high prevalence of neurodevelopmental deficits. Specific features on imaging can aid prognostication and establish early intervention strategies. WHAT THIS PAPER ADDS: Atypical long-term neurodevelopmental outcome is very common in patients with congenital cerebellar malformations (CCMs). Involvement of the brainstem and cerebellar hemispheres predicts more severe neurodevelopmental disability. Most patients with vermis hypoplasia have language delay but are verbal. Supratentorial abnormalities are not significantly associated with worse neurodevelopmental outcome but are associated with epilepsy. Comorbidities are common in CCMs, especially ophthalmological issues in cerebellar hypoplasia and sensorineural hearing loss in pontocerebellar hypoplasia.

Family-centred health care for children with cerebral palsy.

AIM: To identify characteristics of young children with cerebral palsy (CP), and intrinsic and extrinsic factors, that may be associated with parental perceptions regarding family-centred health care services. METHOD: We conducted a cross-sectional study, drawing our sample from the Canadian Cerebral Palsy Registry (CCPR). Parents rated the extent of family-centred care provided by their child's health care teams using the 56-item Measures of Process of Care (MPOC) questionnaire. Environmental and CP phenotypic variables were extracted from the CCPR for group comparisons. Low and high MPOC-56 raters were also compared. RESULTS: Valid responses were obtained from 282 families (90%). All MPOC-56 subscales were highly rated (median ≥6.0), indicating satisfaction with health care services, with the exception of the Providing General Information subscale (median 4.8, interquartile range 3.2-6.0). Parents from Nova Scotia rated all subscales significantly higher than parents from other regions. CP subtype and severity were not significantly associated with MPOC-56 subscale scores. Higher socio-economic status was associated with lower MPOC-56 subscale scores. Higher paternal educational attainment and household income were significantly associated with lower scores on the Providing General Information and Providing Specific Information about the Child subscales respectively. INTERPRETATION: Participants affirmed the provision of family-centred services from Canadian pediatric rehabilitation centres. Sociodemographic factors were associated with parental perceptions of family-centred services. WHAT THIS PAPER ADDS: Sociodemographic factors were associated with parental perceptions of family-centred care. Factors intrinsic to the child's cerebral palsy were not associated with parental perceptions.

The 'ouR-HOPE' approach for ethics and communication about neonatal neurological injury.

Predicting neurological outcomes of neonates with acute brain injury is an essential component of shared decision-making, in order to guide the development of treatment goals and appropriate care plans. It can aid parents in imagining the child's future, and guide timely and ongoing treatment decisions, including shifting treatment goals and focusing on comfort care. However, numerous challenges have been reported with respect to evidence-based practices for prognostication such as biases about prognosis among clinicians. Additionally, the evaluation or appreciation of living with disability can differ, including the well-known disability paradox where patients self-report a good quality of life in spite of severe disability. Herein, we put forward a set of five practice principles captured in the "ouR-HOPE" approach (Reflection, Humility, Open-mindedness, Partnership, and Engagement) and related questions to encourage clinicians to self-assess their practice and engage with others in responding to these challenges. We hope that this proposal paves the way to greater discussion and attention to ethical aspects of communicating prognosis in the context of neonatal brain injury.