ABSTRACT

This chapter provides a detailed review of the literature on the cognitive and neuropsychological aspects of the Turner Syndrome (TS) phenotype. It is well known that females with TS are at risk for cognitive impairment. In reviewing the literature, the investigation of the neurological/neuropsychological concomitants of TS appears to follows four lines of pursuit: neurological studies, neuropathological studies, neuropsychological studies, and hemispheric lateralization studies. A. L. Christensen and J. Nielsen conducted a neuropsychological investigation of 17 TS women using Luria’s evaluation procedures. The analysis of the published research shows that the relative impairment in nonverbal visuo-spatial processing among TS individuals is robust and pervasive. However, results within the various studies appear to be far less consistent, showing considerable variability among the TS females sampled. The research falls into two major classifications: studies comparing TS individuals to patients having known localized brain lesions, and studies examining the performance of TS patients on clinical measures of brain dysfunction.