The cognitive profile of Sotos syndrome.

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.