Abstract

Objective: Cognitive functioning in Turner Syndrome has been characterized by strengths in verbal functioning, relative weaknesses in visuospatial functioning and in executive functioning, specific learning disorders and limitations in social cognition. Ongoing parental education about potential cognitive manifestations of Turner Syndrome would facilitate early educational intervention enabling better academic and occupational outcome. Method: An 18-year-old college student diagnosed with Turner Syndrome was referred for neuropsychological assessment to rule out a mathematics disorder. She had received mathematics tutoring in junior high school and high school, but continued to have difficulty with simple arithmetic operations, associated with errors such as inversion of numbers (e.g., 6 and 9). Errors in telling time occurred due to numerical inversions. Teachers noted essay writing weakness but formal assessment or intervention was not provided. She reported being bullied in middle school. While aware of the physical impact of Turner Syndrome, the family was not aware of its potential cognitive impact. Results: Neuropsychological findings included Verbal Comprehension and Perceptual Reasoning Index discrepancy, with weaknesses in visuospatial perception and organization, integration, and construction. Facial recognition and judgment of line orientation were at the borderline level. A mathematics disorder and a disorder of written expression were evident. Conclusion(s): The cognitive profile obtained was highly consistent with that reported in the literature for Turner Syndrome. Evidence based feedback enabled acquisition of educational accommodations. Earlier cognitive assessment and provision of additional educational services may have enabled compensation of some areas of weakness, leading to better educational, social and psychological functioning.