Spatial Cognition in Males With Fragile-X Syndrome: Evidence for a Neuropsychological Phenotype

doi:10.1016/S0010-9452(08)70799-8

Cortex

Volume 35, Issue 2, 1999, Pages 263-271

https://doi.org/10.1016/S0010-9452(08)70799-8 Get rights and content

Abstract

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.

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Citation Excerpt :
WCC posits that localised processing results from a failure to attend to the global or meaningful items of a stimulus, whereas EPF argues that global and local processing operate independently of each other. Impairments among FXS populations have been shown on drawing, pegboard and block design tasks (Pegoraro et al., 2014; Crowe and Hay, 1990; Cornish et al., 1998, 1999). Cornish and colleagues highlighted dissociations between visuo-spatial perception and visuo-spatial construction tasks, finding a deficit among the FXS group in the latter and a relatively strong performance in the former.
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Navon-type tasks were used to assess visuo-spatial construction ability across 5 groups of children: typically developing, FXS, AFXS, ASD children who scored low–moderate (HFA) and ASD children that scored severe (LFA) on the Childhood Autism Rating Scale (CARS). Analyses of their developmental trajectories compared the performance of these groups.
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Spatial Cognition in Males With Fragile-X Syndrome: Evidence for a Neuropsychological Phenotype

Abstract

Neuropsychologia

Cell

Psychiatry Research

Cortex

Cell

A classification of hand preference by association analysis

British Journal of Psychology

Specifications of the neurobehavioural associations in males with Fragile-X syndrome

Pediatrics

Fragile-X syndrome: A study of the psychological profile in 23 prepubertal patients

Clinical Genetics

The nature of the spatial deficit in young females with Fragile-X syndrome: A neuropsychological and molecular perspective

Neuropsychologia

Disorders of Space Exploration and Cognition

Strengths and weaknesses in the intellectual functioning of males with Fragile-X syndrome

American Journal of Mental Deficiency

British Picture Vocabulary Scale

Lack of association between mutation size and cognitive/behaviour in fragile-X males: A brief report

American Journal of Medical Genetics

Cognitive profiles associated with the Fra(X) syndrome in males and females

American Journal of Medical Genetics

The right hemisphere: Neuropsychological functions

Journal of Neurosurgery