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Background: Rubinstein-Taybi syndrome (RSTS) is a multiple congenital anomaly syndrome characterised by several typical somatic characteristics and by developmental disabilities with various degrees of severity. Focusing on children with RSTS, the aim of this study was to describe their psychomotor, cognitive, and socio-emotional developmental profiles. Method: Twenty-three children with RSTS (12 boys; 11 girls; mean chronological age: 4 years and 10 months) with severe intellectual disability (mean developmental quotient = 32.39) were recruited from an Expert Department of Medical Genetics. Developmental assessments were carried out with the Brunet-Lézine-Revised scale and the Social Cognitive Evaluation Battery. Results: The participants' developmental profiles were characterised by heterogeneous psychomotor development, homogeneous cognitive and socio-emotional development, by more severe delays in expressive language, vocal imitation, and symbolic play skills, and by better developmental levels in socio-emotional abilities. Conclusions: Based on these atypical developmental profiles, early interventions should target the three most delayed abilities.

Introduction: Intellectual disability (ID) is frequently associated as a comorbidity in autism spectrum disorders (ASD). This study investigated a) how similar the heterogeneity in the cognitive and socio-emotional developmental profiles was for children with ASD and ID, b) the difference between the subjects' profiles and those of typically developing children (TD) matched for developmental levels, c) the skills existing with the lowest and highest developmental levels, and d) the relationship between developmental profiles in ASD and the severity of autism, ID, and the overall developmental level. Participants: The sample was comprised of 119 children (101 boys and 18 girls) who ranged in chronological age (CA) from 21 months to 14 years (M = 5 years 2 months; SD = 2 years 6 months) with developmental levels lower than 24 months. They came from three countries (France = 40, Brazil = 40, and Algeria = 39). The control group was comprised of 40 TD children from these same countries who ranged in CA from 4 to 24 months (M = 1 year 3 months; SD = 5 months). The ASD diagnosis was carried out according to International Statistical Classification of Diseases and Related Health Problems-10th Edition (ICD-10), Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision (DSM-IV-TR), Diagnostic and Statistical Manual of Mental Disorders-5th ed (DSM-5) criteria and the Childhood Autism Rating Scale (CARS). Measures: Children were tested using the Social Cognitive Evaluation Battery (SCEB; Adrien, 2007) by trained psychologists from public and private institutions specialized in the diagnosis of autism and interventions in this field. The SCEB explores 16 functional abilities, in both cognitive and socio-emotional areas, and allows the calculation of domain and area developmental levels and heterogeneity indices for the global, cognitive, and socio-emotional areas. Results: Children with ASD developmental profiles show very high heterogeneity as opposed to TD children. Regardless of the country of origin, there are similarities between the heterogeneous cognitive and socio-emotional developmental profiles of the children with ASD, whose profiles are characterized by lower developmental levels of language and vocal imitation skills, and a relationship between these developmental heterogeneities and the degree of severity of autistic symptomatology, intellectual disability, and overall development level. The implications of this study are presented for clinical assessment and intervention purposes in ASD and ID. [ABSTRACT FROM AUTHOR]

Abstract: Haploops nirae is a gregarious tubiculous amphipod that creates dense tube mats in two ecosystems of South Brittany (i.e. the Bay of Vilaine and the Bay of Concarneau). To assess the ecological consequences of its recent expansion, the pattern of population dynamics of H. nirae was determined from January 2010 to March 2011 at a 29 m deep station in the Bay of Concarneau with a sampling interval of ca. 3 weeks (23 sampling dates). Modal analysis of size-frequency distribution at each date was used to describe the life cycle and the population dynamics of H. nirae, and to estimate the annual secondary production using the increment summation method. We showed that H. nirae is a semelparous species exhibiting a biannual life cycle with a lifespan ranging from 24 to 28 months. The recruitment was extremely high in 2010 and occurred between January and April but most recruits arrived at the end of March. Recruits are produced by two year old individuals, which die shortly after hatching. Female densities were low in winter. Females produced only one brood during their lifetime with an average brood size of 29. H. nirae secondary production was estimated at 9.66 gDW m−2 y−1 and the production to biomass ratio was 2.26 y−1. Results are compared with previous published data on other Ampeliscid amphipods. The biannual life cycle appears unusual for most of Ampeliscidae except for the genus Haploops where a biannual life cycle appears characteristic of this genus. The secondary production calculated for H. nirae was high compared to other biannual Ampeliscidae species and constituted one of the highest production values calculated for an Ampeliscid. [Copyright &y& Elsevier]