Spatial cognition in males with Fragile-X syndrome: evidence for a neuropsychological phenotype.
Spatial performance in a group of young Fragile-X syndrome males with FMR-1 full mutation was compared to a learning disabled control group comprising young Down's syndrome males and two control groups of mainstream schoolchildren. Performance was assessed across a wide range of spatial tasks including visuo-construction, visuo-spatial memory, visuo-motor, and visuo-perception. The findings indicate a task-specific rather than global deficit in spatial performance in Fragile-X males with visuo-constructive and visuo-motor skills most vulnerable. Molecular analysis of the lymphocyte DNA found minimal evidence for a correlation between CGG expansion size and spatial performance, although tasks with a visuo-perceptual component correlated negatively with expansion size indicating that the further away the number of repeats are from the 200 threshold the poorer the performance.
Cornish
K M
KM
Neuropsychology of Genetic Disorders Research Unit, Medical School, University of Nottingham, Queens Medical Centre, UK.
Munir
F
F
Cross
G
G
D000293:Adolescent; D002648:Child; D003071:Cognition / Q000502:physiology*; D004247:DNA / Q000032:analysis / Q000235:genetics; D004314:Down Syndrome / Q000523:psychology; D005600:Fragile X Syndrome / Q000235:genetics* / Q000523:psychology*; D005838:Genotype; D006801:Humans; D007360:Intelligence / Q000502:physiology; D008297:Male; D008568:Memory / Q000502:physiology; D009483:Neuropsychological Tests; D010641:Phenotype; D013028:Space Perception / Q000502:physiology*
D004247:DNA