The Neuropsychological Phenotype in Turner Syndrome

Numerous studies have demonstrated a significant depression in performance IQ (PIQ) in Turner Syndrome (TS) females, but the neuropsychological interpretation of this finding remains unclear. The present study addressed the following questions regarding the neurospychological phenotype in TS: (1) Are TS women neuropsychologically impaired? (2) Is the impairment lateralized and (3) How consistent is the neuropsychological phenotype across TS individuals? Unlike previous studies, the present study utilized both normal and brain damaged female controls. All subjects were given an extended Halstead-Reitan neuropsychological battery. The TS females were significantly worse than normals but not significantly different from brain damaged females in their overall level of neuropsychological functioning. However, their impairment was not lateralized. Their pattern of lateralizing findings was similar to that found in the Diffuse and Normal groups, but significantly different from either the right or left unilateral lesion groups. Fairly consistent deficits were found on tests of visuospatial skills and long term memory, but there was considerable variability in all the other test findings among TS individuals. The results are discussed in relation to the recent findings (Inglis and Lawson, 1981) that verbal-performance IQ discrepancies may be unreliable indicators of lateralized cerebral dysfunction in females. Hence the depressed PIQ in TS appears not to indicate predominantly right hemisphere dysfunction and may not even indicate a consistent underlying neuropsychological phenotype.