Specific Neurocognitive Deficits in Cornelia de Lange Syndrome

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ABSTRACT.Cornelia de Lange Syndrome (DLS) is a dysmorphogenic disorder typically associated with severe mental retardation. This report describes a rare case with normal-range verbal intelligence and specific cognitive deficits suggestive of a developmental visuospatial disorder. This was apparent in selective deficits in the ability to integrate visual percepts, copy spatial configurations, and manipulate representations of objects in space. Visual memory and psychomotor skills were also impaired. These deficits occurred within the context of normal language abilities and language-related academic achievement. The observed pattern of neuropsychologic impairment, in addition to aspects of socioemotional development, was similar to that seen in children with developmental right-hemisphere dysfunction. These findings suggest that the factors underlying DLS can be associated with fairly specific aberrations of cortical functioning.J Dev Behav Pediatr 15:39-43, 1994

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