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Background: Individuals with Down syndrome (DS) exhibit higher risk for celiac disease (CD) than general population. Although literature suggests CD could be associated with behavioural problems in both paediatric and adult age, such association has been poorly explored in children and adolescents DS. Therefore, the current study aimed to investigate differences in emotional/behavioural difficulties, adaptive skills and sleep problems between children with DS with and without CD. Methods: Data were retrospectively collected from a database including data from 381 individuals with DS (3–18 years). The final sample included 65 participants, 27 with co‐occurring CD and 38 age, IQ, sex and body mass index‐matched controls without CD. Emotional/behavioural difficulties, adaptive skills and sleep problems were assessed through parent report questionnaires. Results: No group differences emerged in emotional/behavioural difficulties, whereas participants in the CD group showed better adaptive skills in the practical domain than control group. Weak differences emerged in sleep problems. Conclusions: Youth with DS and co‐occurring CD do not exhibit more emotional and behavioural problems than youth with DS without co‐occurring CD but exhibit better adaptive skills in the practical domain. [ABSTRACT FROM AUTHOR]

Background: Individuals with Williams syndrome (WS) show a disharmonic linguistic profile with a clear pattern of strengths and weaknesses. Despite their sociable nature, atypical socio-communicative abilities and deficits in communication and relationship with others have been found. Aim: The aim of the present study was to investigate whether linguistic skills (LS) were in line with the pragmatic and social use of language and the cognitive development of 32 individuals with WS (18 boys and 14 girls) with a mean chronological age of 12.3 (±4.4) years. To examine the relationship between language and mental age (MA) at different ages, the issue was investigated in children and adolescents/young adults with WS. Method: Measures of LS, including lexical and morphosyntactic competences, and adaptive socio-communicative abilities (ASCA), pertaining to the use of language in daily living social context, were compared with the MA of participants. In a second step, participants with WS were split into two subgroups based on age, and the relationship between LS, ASCA and MA was studied. Results: Although expressive and receptive LS were generally found to be in line with or better than would be expected for MA, specific deficits in receptive ASCA were documented. LS and ASCA appeared to have a different evolution during the different time windows considered. Conclusions: Our results underlined the importance of assessing linguistic abilities in the context of adaptive functioning, to guide educational and rehabilitative strategies for individuals with WS.

This brief report is a partial replication of the study by Jackson and Atance (J Dev Disabil 14:40-45, 2008 (See CR12)) assessing nonverbal Self-based and Mechanical-based future thinking (FT) in children with Autism Spectrum Disorder (ASD). In a first step, these tasks were administered to 30 children with ASD. The two Self-based tasks were then modified as a verbal component could not be completely ruled out. Consequently, 77 children with ASD and 77 children with typical development received the modified Self-based FT tasks and the Mechanical-based FT tasks. We partially replicated the previous findings. Participants with ASD had impaired FT in both kinds of tasks and both groups performed better on tasks assessing Mechanical-based FT than Self-based FT. These results suggest that impairments of FT in ASD are not limited to Self-Projection., Author(s): A. Marini[sup.1] [sup.2] , F. Ferretti[sup.3] , A. Chiera[sup.3] , R. Magni[sup.4] , I. Adornetti[sup.3] , S. Nicchiarelli[sup.3] , S. Vicari[sup.4] , G. Valeri[sup.4] Author Affiliations: (1) Department of [...]

Background: In the last few years, experimental data have been reported on differences in implicit memory processes of genetically distinct groups of individuals with Intellectual Disability (ID). These evidences are relevant for the more general debate on supposed asynchrony of cognitive maturation in children with abnormal brain development. This study, comparing implicit memory processes in individuals with Williams syndrome (WS) and Down syndrome (DS), was planned to verify the "etiological specificity" hypotheses pertaining to the skill learning abilities of individuals with ID. Method: A modified version of Nissen and Bullemer's (1987) Serial Reaction Time (SRT) task was used. The performances of three group were evaluated. The first group consisted of thirty-two people with WS (18 males and 14 females). The second group was comprised of twenty-six individuals with DS (14 males and 12 females). The two groups of individuals with ID were selected so that the groups were comparable as for mental age and chronological age. The third group consisted of forty-nine typically developed children with a mental age similar to that of the groups with WS and DS. Results: The two groups of individuals with ID demonstrated different patterns of procedural learning. WS individuals revealed poor implicit learning of the temporal sequence of events characterizing the ordered blocks in the SRT task. Indeed, differently from normal controls, WS participants showed no reaction time (RT) speeding through ordered blocks. Most importantly, the rebound effect, which so dramatically affected normal children's RTs passing from the last ordered to the last block, had only a marginal influence on WS children's RTs. Differently from the WS group, the rate of procedural learning of the participants with DS was comparable to that of their controls. Indeed, DS and typically developed individuals showed parallel RT variations in the series of ordered blocks and, more importantly, passing from the last ordered to the last block. Therefore, a substantial preservation of skill learning abilities in this genetic syndrome is confirmed. Conclusions: The results of the present study document that procedural learning in individuals with ID depends on the aetiology of the syndrome, thus supporting the etiological specificity account of their cognitive development. These results are relevant for our knowledge about the qualitative aspects and the underlying neurobiological substrate of the anomalous cognitive development in mentally retarded people.

Background: Although reading abilities play a fundamental role in the acquisition of personal autonomy, up until now studies investigating these abilities in Down syndrome (DS) are aimed at defining educational or rehability acquisition. However, studies describing the relationship between reading and phonological awareness in individuals with DS by comparing them to typically developing children often report contradictory results. The aim of this study is to explore reading and phonological awareness skills in a group of participants with DS. Methods: We administered reading and phonological processing ability tests to 17 DS individuals and to 17 reading-age-matched typically developing children. Results: Concerning reading abilities, participants with DS were impaired on non-word reading and on interpreting accuracy of non-homographic homophones. Their passage comprehension was also limited. Comparable ability was reported in the two groups on irregular word reading and passage reading tasks. Regarding phonological awareness ability, individuals with DS showed lower performances on several tasks, such as rhyming, deletion and syllable segmentation. Conclusions: People with DS show particular failure on non-word reading, a task where correct decoding is only partially influenced by lexical access or semantic context. Correct non-word reading mainly requires the use of the grapheme--phoneme conversion process. This process is based on the efficiency of phonological awareness abilities, which are partly impaired in people with DS. The rehabilitative implications of these findings are discussed.

Verbal short-term memory, as measured by digit or word span, is generally impaired in individuals with Down's syndrome (DS) compared to mental age-matched controls. Moving from the working memory model, the present authors investigated the hypothesis that impairment in some of the articulatory loop sub-components is at the base of the deficient maintenance and recall of phonological representations in individuals with DS. Two experiments were carried out in a group of adolescents with DS and in typically developing children matched for mental age. In the first experiment, the authors explored the reliance of these subjects on the subvocal rehearsal mechanism during a word-span task and the effects produced by varying the frequency of occurrence of the words on the extension of the word span. In the second experiment, they investigated the functioning of the phonological store component of the articulatory loop in more detail. A reduced verbal span in DS was confirmed. Neither individuals with DS nor controls engaged in spontaneous subvocal rehearsal. Moreover, the data provide little support for defective functioning of the phonological store in DS. No evidence was found suggesting that a dysfunction of the articulatory loop and lexical-semantic competence significantly contributed to verbal span reduction in subjects with DS. Alternative explanations of defective verbal short-term memory in DS, such as a central executive system impairment, must be considered.

Background Method Results Conclusions Individuals with intellectual disability, including people with Down syndrome (DS), often exhibit restricted and repetitive behaviours (RRBs). However, RRBs have not been deeply characterised in children and adolescents with DS.The study encompassed a cohort of 151 participants aged 4 to 18 years with DS. RRBs were assessed utilising the Repetitive Behaviour Scale‐Revised. Additionally, data pertaining to cognitive and adaptive functioning, linguistic abilities, sleep patterns and emotional/behavioural issues were gathered.Self‐injurious behaviours were reported less frequently whereas parents most commonly endorsed items related to behaviours associated with the need for sameness and ritualistic behaviours. We observed very few gender differences, whereas some age‐related differences emerged, with adolescents exhibiting higher scores in items related with higher‐level RRBs. The analysis of the association between RRBs and clinical features revealed that RRBs were associated with parent‐reported sleep difficulties, as well as with internalising and externalising problems. We also observed a negative correlation with IQ whereas associations with adaptive skills emerged mainly for lower‐level RRBs, such as motor stereotypies. Finally, RRBs were negatively associated with linguistic abilities, both expressive and receptive.RRBs in children and adolescents with DS are of significant clinical interest due to their associations with various clinical dimensions. Therefore, psychological and neuropsychiatric assessment should include an accurate evaluation of RRBs for young people with DS. [ABSTRACT FROM AUTHOR]