A review of neuropsychological and motor studies in Turner Syndrome

Abstract

We reviewed the literature on Turner Syndrome (TS) from 1962 until March 2003 with respect to the following questions: Is there a consistent pattern of cognitive and/or motor dysfunction in TS girls and if so, is there an explanation for the disturbance? Many studies indicate that girls with TS have a disharmonic IQ profile: a verbal IQ that seems to be at a (nearly) normal level and a decreased PIQ. This profile remains into adulthood. Visual-spatial problems are mentioned most frequently and there is some evidence for a relationship to particular neuro-anatomical structures, hormonal dysfunction, and genotype. Although much less research has been done on motor performance in TS, there is clear evidence that it is disturbed too in TS. Many authors emphasize the interaction between somatic, psychological and social factors, but we did not find a clear theoretical framework explaining this relationship. We argue that there may be two independent problems: a visuospatial and a motor deficit possible related to specific genotypes and both have implications for functioning in daily life.