The cognitive profile of Sotos syndrome. Issue 2 (15th January 2018)

• Record Type: Journal Article
• Authors: Lane, Chloe; Milne, Elizabeth; Freeth, Megan
• Is Part Of: Journal of neuropsychology. Volume 13:Issue 2(2019)
• Page Start: 240
• Page End: 252
• Publication Date: 2018-01-15
• Subjects: cognitive profile -- intellectual disability -- sotos syndrome; Neuropsychology -- Periodicals; Brain -- Diseases -- Psychological aspects -- Periodicals; 612.82305
• DOI: 10.1111/jnp.12146
• Languages: English
• ISSNs: 1748-6645

Abstract

Abstract : Sotos syndrome is a congenital overgrowth disorder, associated with intellectual disability. Previous research suggests that Sotos syndrome may be associated with relative strength in verbal ability and relative weakness in non‐verbal reasoning ability but this has not been explicitly assessed. To date, the cognitive profile of Sotos syndrome is unknown. Cognitive abilities of a large and representative sample of individuals with Sotos syndrome ( N = 52) were assessed using the British Ability Scales (BAS3). The majority of participants had intellectual disability or borderline intellectual functioning. The cluster score profile analysis revealed a consistent verbal ability > non‐verbal reasoning ability profile. Four specific criteria were proposed as the Sotos syndrome cognitive profile (SSCP): verbal ability > non‐verbal reasoning ability; quantitative reasoning T‐score or matrices T‐score <20th percentile; quantitative reasoning T‐score < mean T‐score; recognition of designs T‐score or recognition of pictures T‐score > mean T‐score. Of the 35 participants included in the profile analysis, 28 met all four SSCP criteria, yielding a sensitivity of 0.8. The sensitivity of each of the SSCP criteria was >0.9. Individuals with Sotos syndrome display a clear and consistent cognitive profile, characterized by relative strength in verbal ability and visuospatial memory but relative weakness in non‐verbal reasoning ability and quantitative reasoning. This has important implications for the education of individuals with Sotos syndrome.