A systematic review of speech, language and communication interventions for children with Down syndrome from 0 to 6 years.

BACKGROUND: Speech and language acquisition can be a challenge for young children with Down syndrome (DS), and while early intervention is important, we do not know what early interventions exist and how effective they may be. AIMS: To systematically review existing early speech, language and communication interventions for young children with DS from birth up to 6 years, and to investigate their effectiveness in improving speech, language and communication outcomes in children with DS. Other outcomes are changes in parental behaviour and their responsiveness METHODS & PROCEDURES: We conducted a systematic search of relevant electronic databases to identify early intervention studies targeting speech, language and communication outcomes in children with DS published up to May 2020. A total of 11 studies that met the inclusion criteria were synthesized and appraised for quality using the PEDro-P scale. There were a total of 242 children. We identified three types of intervention: communication training and responsive teaching, early stimulation programme, and dialectic-didactic approach. MAIN CONTRIBUTION: The findings from nine out of the 11 studies reported positive outcomes for children's language and communication up to 18 months following the intervention. All nine studies reported interventions that were co-delivered by parents and clinicians. However, there was also a de-accelerated growth in requesting behaviours in the intervention group reported by one study as well as a case of no improvement for the intervention group. Three studies provided some evidence of improvements to parent outcomes, such as increased parental language input and increased responsiveness. However, there was a moderate to high risk of bias for all studies included. CONCLUSIONS: The findings from this review suggest that interventions that have high dosage, focus on language and communication training within a naturalistic setting, and are co-delivered by parents and clinicians/researchers may have the potential to provide positive outcomes for children with DS between 0 and 6 years of age. Due to the limited number of studies, limited heterogeneous data and the moderate to high risk of bias across studies, there is an urgent need for higher quality intervention studies in the field to build the evidence base. WHAT THIS PAPER ADDS: What is already known on the subject Speech and language acquisition is usually delayed in children with DS, yet there are currently no standard interventions for children under 6. A number of research-based interventions exist in the literature, yet it is unknown how effective these are. What this study adds to existing knowledge This is the first systematic review that specifically and exclusively focuses on parent- and non-parent-mediated speech, language and communication interventions for children with DS between 0 and 6 years of age. It complements three existing recent reviews, each of which has a slightly different focus. The previously published reviews have covered only parent-mediated interventions, excluding interventions not mediated by parents, have reviewed interventions including children and adults, without any mention of what early interventions may be like or how effective these may be for young children with DS, have not always assessed risk of bias or have focused specifically on language interventions excluding those focusing on speech articulation or pre-linguistic skills. The findings from the current review suggest that interventions that have high dosage focus on language and communication training within a naturalistic setting and are co-delivered by parents and clinicians/researchers may have the potential to provide positive outcomes for children with Diwn syndrome from 0 to 6. We acknowledge that the current evidence base comes from studies with moderate to high risk of bias, hence our conclusions are not definitive. What are the potential or actual clinical implications of this work? Speech and language therapists will have synthesized information and a quick reference point on what type of interventions exist for children with DS under the age of 6, and evidence of which intervention approaches may be promising in terms of providing positive outcomes. However, it is acknowledged that, due to the limited number of studies and the moderate to high risk of bias inherent in the evidence, there is an urgent need for higher quality intervention studies in the field to build the evidence base

Autistic Disorder in Patients with Williams-Beuren Syndrome: A Reconsideration of the Williams-Beuren Syndrome Phenotype

International audienceBackground: Williams-Beuren syndrome (WBS), a rare developmental disorder caused by deletion of contiguous genes at 7q11.23, has been characterized by strengths in socialization (overfriendliness) and communication (excessive talkativeness). WBS has been often considered as the polar opposite behavioral phenotype to autism. Our objective was to better understand the range of phenotypic expression in WBS and the relationship between WBS and autistic disorder. Methodology: The study was conducted on 9 French individuals aged from 4 to 37 years old with autistic disorder associated with WBS. Behavioral assessments were performed using Autism Diagnostic Interview-Revised (ADI-R) and Autism Diagnostic Observation Schedule (ADOS) scales. Molecular characterization of the WBS critical region was performed by FISH. Findings: FISH analysis indicated that all 9 patients displayed the common WBS deletion. All 9 patients met ADI-R and ADOS diagnostic criteria for autism, displaying stereotypies and severe impairments in social interaction and communication (including the absence of expressive language). Additionally, patients showed improvement in social communication over time. Conclusions: The results indicate that comorbid autism and WBS is more frequent than expected and suggest that the common WBS deletion can result in a continuum of social communication impairment, ranging from excessive talkativeness and overfriendliness to absence of verbal language and poor social relationships. Appreciation of the possible co-occurrence of WBS and autism challenges the common view that WBS represents the opposite behavioral phenotype of autism, and might lead to improved recognition of WBS in individuals diagnosed with autism

Social interaction deficits and conversational inadequacy in Williams syndrome

Research on social communication skills in individuals with Williams syndrome has been inconclusive, with some arguing that these skills are a relative strength and others that they are a weakness. The aim of the present study was to investigate social interaction abilities in a group of children with WS, and to compare them to a group of children with specific language impairment and a group of typically developing children. Semi-structured conversations were conducted and 100-150 utterances were selected for analysis in terms of exchange structure, turn taking, information transfer and conversational inadequacy. The statistical analyses showed that the children with WS had difficulties with exchange structure and responding appropriately to the interlocutor's requests for information and clarification. They also had significant difficulties with interpreting meaning and providing enough information for the conversational partner. Despite similar language abilities with a group of children with specific language impairment, the children with WS had different social interaction skills, which suggests that they follow an atypical trajectory of development and their neurolinguistic profile does not directly support innate modularity. (c) 2005 Elsevier Ltd. All rights reserved

The co-occurrence of autism and Williams syndrome: a case study report

This paper describes a longitudinal case study detailing the communication profile of one child with both Williams syndrome (WS) and autism. The participant was administered two standardized assessments of language and general cognitive abilities. His parents completed the Pre-Verbal Communication Schedule; and a sample of the child's spontaneous interaction was analyzed. The results show that this child presents with markedly delayed language and communication skills and that his communication profile is the opposite of the assumed 'typical' WS profile. The conclusion is that clinicians need to be aware of the co-occurrence of genetic disorders, such as WS and autism in order to facilitate accurate diagnosis and effective treatment

Expressive versus receptive language skills in specific reading disorder

Despite ample research into the language skills of children with specific reading disorder no studies so far have investigated whether there may be a difference between expressive and receptive language skills in this population. Yet, neuro-anatomical models would predict that children who have specific reading disorder which is not associated with movement or attention difficulties, would have lower receptive language skills than expressive. This study investigates the difference between expressive and receptive language skills in a sample of 17 children with specific reading difficulty aged between 7 and 12 years. They were administered a battery of two receptive and two expressive language measures. The results showed that as the neuro-anatomical model would predict, the children scored significantly lower on tests of receptive than on tests of expressive language skills

Do children with Williams syndrome have unusual vocabularies?

Aims: The present study investigated whether children with Williams syndrome (WS) produced a higher number of different word roots and low-frequency words in spontaneous speech in a topic controlled setting. Method: A group of children with WS was compared to a group of typically developing children matched for chronological age (CA), and a group of typically developing children matched for receptive language abilities (LA). A further comparison was made between the WS group and a group of children matched for non-verbal abilities (NA). Spontaneous speech was elicited using a narrative task. The data were analysed using three different measures of lexical diversity. The results revealed that the children with WS neither produce a higher number of different word roots nor significantly more low-frequency items in comparison to the CA, LA and NA matched participants. Furthermore, language and non-verbal abilities did not predict the number of different and low frequency words used by the typically developing children, however in the WS group non-verbal abilities predicted the number of low-frequency words and receptive language skills predicted the number of different words produced. It is concluded that individuals with WS do not have unusual vocabularies and that the subdomain of language, lexical semantics, does not seem to be an independent cognitive skill. (c) 2007 Elsevier Ltd. All rights reserved