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Aim: This paper introduces the Surveillance of Cerebral Palsy in Europe (SCPE) classification of events contributing to postneonatally acquired cerebral palsy, presents its interrater reliability, and describes the cases identified in the SCPE database. Method: The development of the classification, based on literature review and expert discussions, resulted in six main categories and 19 subcategories. The first chronological event designated as the primary event was mainly reported. Interrater reliability was assessed through online exercise providing 24 clinical vignettes representing single/complex pathways. Percent agreement and Gwet's AC1 index of reliability were estimated. Primary events were described using data of 221 children born between 2008 and 2012. Results: Thirty‐nine professionals (21 registries) participated in the reliability exercise. Substantial overall agreement was reached (0.75), with some contrast between complex (0.48, moderate agreement) and single events involved (0.89, almost perfect). The distribution of primary events showed that 32.1% were infections (category A), 23.1% head injuries (B), 15.4% related to surgery or medical interventions (C), 13.1% cerebrovascular accidents (D), 9.1% hypoxic brain damaging events of other origins (E), and 7.2% miscellaneous (F). Interpretation: This classification allows all the events involved to be recorded while consistently reporting the primary event, and may be used in different settings. What this paper adds: A standardized classification enables the description of the events contributing to postneonatal cerebral palsy (CP).The first chronological event in complex pathway leading to CP is coded.Category choice and coding of the primary event identify preventable situations.The detailed 2‐level classification is easy to use in various settings.Substantial overall interrater reliability shows that main categories can be consistently differentiated. [ABSTRACT FROM AUTHOR]

Aim: To study long‐term sequelae in children with Guillain–Barré syndrome (GBS). Method: This was a prospective observational study with children from two French tertiary centres. Data were from clinical and several standardized scales or questionnaires. Results: Fifty‐one patients were included with a median follow‐up of 6 years 4 months (range 3–20 years) after the acute phase. The sequelae rate was 67% (95% confidence interval [CI] 53–78) and did not vary with time. Most children had minor sequelae (Guillain–Barré Syndrome Disability Score [GBSDS] = 1); only one was unable to run (GBSDS = 2). The most frequent complaints were paraesthesia (43%), pain (35%), and fatigue (31%). The neurological examination was abnormal in 18% of children, autonomy was compromised in 14%, and symptoms of depression occurred in 34%. The factors associated with late‐onset sequelae were correlated with severity during the initial phase (i.e. initial GBSDS >4, odds ratio 6.6, 95% CI 1.8–33; p = 0.009). The predictive factors of more severe late‐onset conditions were initial severity (p = 0.002) and sex (female patients; p = 0.01). Interpretation: Two‐thirds of children with GBS had late‐onset sequelae following an episode, often minor, but sometimes with continuing effects on their everyday lives. Particularly affected were those who had severe GBS during the acute phase and who lost the ability to walk. What this paper adds: Two‐thirds of children with Guillain–Barré syndrome (GBS) had persistent sequelae.Sequelae were often minor, but daily repercussions of them were sometimes serious.Sequelae were significantly associated with severe GBS during the acute phase. What this paper adds: Two‐thirds of children with Guillain–Barré syndrome (GBS) had persistent sequelae.Sequelae were often minor, but daily repercussions of them were sometimes serious.Sequelae were significantly associated with severe GBS during the acute phase. [ABSTRACT FROM AUTHOR]

Duchenne Muscular Dystrophy (DMD) is a neuromuscular disease that inevitably leads to total loss of autonomy. The new therapeutic strategies aim to both improve survival and optimise quality of life. Evaluating quality of life is nevertheless a major challenge. No DMD-specific quality of life scale to exists in French. We therefore produced a French translation of the English Duchenne Muscular Dystrophy module of the Pediatric Quality of Life Inventory (PedsQLTMDMD) following international recommendations. The study objective was to carry out a confirmatory validation of the French version of the PedsQLTMDMD for paediatric patients with DMD, using French multicentre descriptive cross-sectional data. The sample consisted of 107 patients. Internal consistency was acceptable for proxy-assessments, with Cronbach's alpha coefficients above 0.70, except for the Treatment dimension. For self-assessments, internal consistency was acceptable only for the Daily Activities dimension. Our results showed poor metric qualities for the French version of the PedsQLTMDMD based on a sample of about 100 children, but these results remained consistent with those of the original validation. This confirms the interest of its use in clinical practice. [ABSTRACT FROM AUTHOR]

Abstract Cyanobacterial blooms pose serious threats to aquatic organisms and strongly impact the functioning of aquatic ecosystems. Due to their ability to produce a wide range of potentially bioactive secondary metabolites, so called cyanotoxins, cyanobacteria have been extensively studied in the past decades. Proteomic and metabolomic analyses provide a unique opportunity to evaluate the global response of hundreds of proteins and metabolites at a glance. In this study, we provide the first combined utilization of these methods targeted to identify the response of fish to bloom-forming cyanobacteria. Medaka fish (Oryzias latipes) were exposed for 96 hours either to a MC-producing or to a non-MC-producing strain of Microcystis aeruginosa and cellular, proteome and metabolome changes following exposure to cyanobacteria were characterized in the fish livers. The results suggest that a short-term exposure to cyanobacteria, producing or not MCs, induces sex-dependent molecular changes in medaka fish, without causing any cellular alterations. Globally, molecular entities involved in stress response, lipid metabolism and developmental processes exhibit the most contrasted changes following a cyanobacterial exposure. Moreover, it appears that proteomic and metabolomic analyses are useful tools to verify previous information and to additionally bring new horizons concerning molecular effects of cyanobacteria on fish.

Coring of a medication vial’s rubber stopper has been reported as a major cause of visible particle presence in injectable preparations. In this study, we investigated and quantified visible particle formation caused by coring associated with four potential causal factors.

Cyanobacteria are photosynthetic prokaryotes capable of synthesizing a large variety of secondary metabolites that exhibit significant bioactivity or toxicity. Microcystis constitutes one of the most common cyanobacterial genera, forming the intensive blooms that nowadays arise in freshwater ecosystems worldwide. Species in this genus can produce numerous cyanotoxins (i.e., toxic cyanobacterial metabolites), which can be harmful to human health and aquatic organisms. To better understand variations in cyanotoxin production between clones of Microcystis species, we investigated the diversity of 24 strains isolated from the same blooms or from different populations in various geographical areas. Strains were compared by genotyping with 16S-ITS fragment sequencing and metabolite chemotyping using LC ESI-qTOF mass spectrometry. While genotyping can help to discriminate among different species, the global metabolome analysis revealed clearly discriminating molecular profiles among strains. These profiles could be clustered primarily according to their global metabolite content, then according to their genotype, and finally according to their sampling location. A global molecular network of all metabolites produced by Microcystis species highlights the production of a wide set of chemically diverse metabolites, including a few microcystins, many aeruginosins, microginins, cyanopeptolins, and anabaenopeptins, together with a large set of unknown molecules. These components, which constitute the molecular biodiversity of Microcystis species, still need to be investigated in terms of their structure and potential bioactivites (e.g., toxicity).

Register-based prevalence rates of childhood autism (CA), Asperger's syndrome (AS) and other autism spectrum disorders (ASD) were calculated among children aged 7 years old of the 1997-2003 birth cohorts, living in four counties in France. The proportion of children presenting comorbidities was reported. 1123 children with ASD were recorded (M/F ratio: 4.1), representing an overall prevalence rate of 36.5/10,000 children (95% CI 34.4-38.7): 8.8/10,000 for CA (95% CI 7.8-9.9), 1.7/10,000 for AS (95 % CI 1.3-2.3) and 25.9/10,000 for other ASD (95% CI 24.2-27.8). ASD prevalence significantly increased (p < 0.0001) during the period under study. The proportion of children with an intellectual disability was 47.3 %, all other comorbidities were present in less than 5% of the cases.

Objective: To compare the neurodevelopmental outcomes of preterm twins at 5½ years by chorionicity of pregnancy. Design: Prospective nationwide population‐based EPIPAGE2 (Etude Epidémiologique sur les Petits Âges Gestationnels) cohort study. Setting: A total of 546 maternity units in France, between March and December 2011. Population: A total of 1126 twins eligible for follow‐up at 5½ years. Methods: The association of chorionicity with outcomes was analysed using multivariate regression models. Main outcome measures: Survival at 5½ years with or without neurodevelopmental disabilities (comprising cerebral palsy, visual, hearing, cognitive deficiency, behavioural difficulties or developmental coordination disorders) were described and compared by chorionicity. Results: Among the 1126 twins eligible for follow‐up at 5½ years, 926 (82.2%) could be evaluated: 228 monochorionic (MC) and 698 dichorionic (DC). Based on chronicity and gestational age of birth, we found no significant differences for severe neonatal morbidity. The rates of moderate/severe neurobehavioral disabilities were similar in infants from DC pregnancies versus infants from MC pregnancies (OR 1.22, 95% CI 0.65–2.28). By gestational age and without twin–twin transfusion syndrome (TTTS), no difference according to chorionicity was found for all neurodevelopmental outcome measures. Conclusions: The neurodevelopmental outcomes among preterm twins at 5½ years is similar, irrespective of chorionicity. Linked article: This article is commented on by R. N. Brown, pp. 1059 in this issue. To view this mini commentary visit https://doi.org/10.1111/1471‐0528.17527. [ABSTRACT FROM AUTHOR]

The present paper aims to analyze trends over time in prevalence of cerebral palsy of post-neonatal origin, to investigate whether changes are similar according to severity and to describe the disability profile by etiology. Post-neonatal cases, birth years 1976 to 1998, were identified from the Surveillance of Cerebral Palsy in Europe collaboration (19 population-based registries). A recognized causal event occurring between 28 days and 24 months of age was considered to define the cases. Trends in prevalence were explored using graphical methods (Lowess and Cusum control chart) and modeled with negative binomial regressions. Over the study period, 404 cases were identified as post-neonatal cases (5.5% of the total). Mean prevalence rate was 1.20 per 10,000 live births (95% CI [1.08-1.31]). A significant downward trend was observed (p = 0.001), with an accentuated decrease in the 1990s. The prevalence of severe cases which account for around one third of the total also significantly decreased over time (p less than 0.001). In 46% of cases, an infectious aetiology was reported; the corresponding prevalence significantly decreased since 1989. No significant decrease was observed for the rate of cases due to a vascular episode or of traumatic origin. Our results emphasize the need of large population-based surveillance systems to reliably monitor trends in prevalence in rare subgroups of children like those with acquired cerebral palsy. The decrease of the overall prevalence as well as those of the most severe cases may be partly due to public health actions targeted to prevent such events. (Contains 2 figures and 2 tables.)

The principle of inclusive education has been increasingly recognised over recent decades and most countries officially support schooling of children with disabilities in mainstream settings. The SPARCLE study offers the opportunity to report on the schooling practices for children with cerebral palsy according to the nature and severity of their impairments and the schooling policy in European regions. The aim of this paper is to describe the type of schooling of children with cerebral palsy in various European regions after controlling for relevant individual factors. Children aged 8-12 years with cerebral palsy from 9 European regions and their families were interviewed. Our findings support the hypothesis that between-region variations in the type of schooling are still significant after adjustment for individual factors; and that motor function and intellectual ability have different effects on inclusion in mainstream school, depending on the region. (Contains 3 tables and 1 figure.)

Living with a child with a disability can affect family life in various domains. Impacts on time, expenses, work, relationships within the family, social relationships and physical and psychological health can be observed. The Family Impact of Childhood Disability (FICD) is a specific instrument designed to assess this situation. Used in a cross-sectional survey, this questionnaire was extended to consider two missing aspects: impact on work and health (FICD + 4). This paper addresses the psychometric qualities of the FICD in Europe among parents living with an adolescent with cerebral palsy. Expecting the FICD + 4 could assess detailed impact dimensions, an exploratory analysis was conducted. We interviewed 242 families of 13- to 17-year-old adolescents with cerebral palsy living in Europe. Good psychometric properties were found in negative and positive FICD scales and in six underlying factors extracted from exploratory factor analysis on FICD + 4. These results support the psychometric validity of the FICD in the assessment of the impact of disability in European families who live with an adolescent with cerebral palsy. They also highlight the multifaceted aspects of the impact of childhood disability on the family and suggest that the FICD + 4 is a good tool for assessing specific negative impacts on time, finances, work, social relationships and positive impacts on parental feeling and family attitude. This scale needs further validation and could be helpful for research and clinical interventions. (Contains 1 figure and 5 tables.)