Exploring the approximate number system in Sotos syndrome: insights from a dot comparison task

Abstract

Background Sotos syndrome is a congenital overgrowth condition associated with intellectual disability and an uneven cognitive profile. Previous research has established that individuals with Sotos syndrome have relatively poor mathematical ability, but domain‐specific numeracy skills have not been explored within this population. This study investigated the approximate number system (ANS) in Sotos syndrome. Method A dot comparison task was administered to 20 participants with Sotos syndrome (mean age in years = 18.43, SD = 9.29). Performance was compared to a chronological agematched typically developing control group (n = 25) and a mental age‐matched Williams syndrome group (n = 24). Results The Sotos group did not display an ANS deficit overall when compared to chronological agematched control participants. However, for trials where the size of the individual dots and the envelope area were negatively correlated with the total number of dots (incongruent trials), the Sotos group were less accurate than the typically developing group but more accurate than the Williams syndrome group, suggesting an inhibitory control deficit. Better accuracy on incongruent trials, but not congruent trials, was associated with higher quantitative reasoning ability for participants with Sotos syndrome. Conclusion Overall, the findings suggest that ANS acuity is not impaired in Sotos syndrome but that numerical difficulties may be associated with an inhibitory control deficit for individuals with Sotos syndrome.

Online and Face-to-Face Performance on Two Cognitive Tasks in Children With Williams Syndrome

“…This study can also serve as an example for the development of future online assessment for children with other neurodevelopmental conditions, especially those with mild to moderate ID and uneven cognitive profiles who experience attention difficulties. Relatedly, online testing could also benefit research in other rare neurodevelopmental conditions that suffer from similar recruitment issues such as Cri du Chat syndrome, Cornelia de Lange syndrome, and Sotos syndrome that occur 1:15,000, 1:10,000, and 1:14,000, respectively (Mainardi, 2006;Kline et al, 2007;Lane et al, 2019). The current study also provides evidence that despite their ID, children with WS were able to engage with RCPM and BPVS tasks online.…”

„Ohne Geld kann man ja nicht überleben oder nichts machen oder sich entfalten“

“…B. Lane, Herwegen & Freeth, 2019;Garrote, Moser Opitz & Ratz, 2015). Tan, Lambert, Padilla und Wieman (2019) kritisieren zudem in ihrer Metastudie über USamerikanische Forschungs arbeiten (2006 -2017) zu Schulmathematik und geistiger Behinderung die Dominanz kognitiver Testverfahren und eines behavioristischen Paradigmas.…”

Abilities in the blind spot of testing regimes: Eliciting the benefits and the limitations of participatory research approaches for numeracy in adult basic education

“…Despite this growing attention, the extant body of scholarship on adult numeracy and disability, particularly on the subcomponent of learning difficulties, is still very limited (see for example Prendergast et al 2017;Lane et al 2019). This dearth echoes data collection omissions in large-scale assessment surveys.…”