Uncommon genetic syndromes and narrative production - Case Studies with Williams, Smith-Magenis and Prader-Willi Syndromes?

Abstract

This study compares narrative production among three syndromes with genetic microdeletions: Williams syndrome (WS), Smith-Magenis syndrome (SMS), and Prader-Willi syndrome (PWS), characterized by intellectual disabilities and relatively spared language abilities. Our objective is to study the quality of narrative production in the context of a common intellectual disability. To elicit a narrative production, the task Frog! Where Are You was used. Then, structure, process, and content of the narrative process were analysed in the three genetic disorders: WS (n52), SMS (n52), and PWS (n52). Data show evidence of an overall low narrative quality in these syndromes, despite a high variability within different measures of narrative production. Results support the hypothesis that narrative is a highly complex cognitive process and that, in a context of intellectual disability, there is no evidence of particular 'hypernarrativity' in these syndromes.

Neurodevelopmental features of Smith–Magenis syndrome: strengths and weaknesses

“…However, they acknowledged that these individuals also tended to have difficulties in communicating clearly. This is in keeping with previous results from our team (Garayzá bal-Heinze et al, 2011;Garayzá bal-Heinze et al 2012) that showed important delays of individuals with SMS in psycholinguistic and narrative tasks.…”

The Narrative Abilities of an English-Spanish Bilingual with Prader-Willi Syndrome

“…These results showed that Yves' narrative abilities, unlike Olivia's, were lower than what is considered typical development. Thus, this outcome is in line with previous findings from studies focused on the narrative abilities of monolinguals with PWS, which consistently demonstrated low narrative quality (Lewis et al 2002;Garayzábal-Heinze et al 2012).…”

“…Due to the fact that this is a case study involving two participants, a descriptive data analysis was performed. This type of data analysis is the most extended practice within the field due to the recurrent limited number of participants (Garayzábal-Heinze et al 2012;Feltmate and Bird 2008). I first present an overview of the characteristics of the narratives and then an analysis of the participants' narrative abilities.…”

“…Due to the fact that this is a case study, results should be considered as a first approximation to the analysis of narrative abilities in bilingual speakers with PWS and should, therefore, be interpreted cautiously. However, interestingly enough, Garayzábal-Heinze et al (2012) also obtained the gradation scale presented above when analyzing the narrative's abilities of two Spanish monolingual adolescents with PWS (even though they do not discuss this in their paper). These similar outcomes seem to suggest a tendency that should be confirmed or disproved in future studies including larger samples of monolinguals and bilinguals with PWS.…”

“…This methodology was originally conceived to evaluate the narrative abilities of individuals with psychological disorders and its reliability was satisfactorily tested in patients with agoraphobia (Gonçalves et al 2002). Subsequently, it was extended to the assessment of the narrative abilities in individuals with neurodevelopmental disorders such as Williams syndrome, Smith-Magenis syndrome, and PWS (Gonçalves et al 2011;Garayzábal-Heinze et al 2012). Table 1.…”