Reading the Windows to the Soul: Evidence of Domain-Specific Sparing in Williams Syndrome

Abstract

This study tested the hypothesis that Williams syndrome, a rare genetic neurodevelopmental disorder with an unusual cognitive phenotype, involves spared abilities in the domain of understanding other minds. A group of retarded adults with Williams syndrome was compared to an age-, IQ-, and language-matched group of adults with Prader-Willi syndrome, another genetic disorder without the cognitive characteristics of Williams syndrome, and a group of age-matched normal adults, on a task that taps mentalizing ability. The task involved selecting the correct labels to match photographs of complex mental state expressions in the eye region of the face. The adults with Williams syndrome performed significantly better than the adults with Prader-Willi on this task, and about half the group performed in the same range as the normal adults. These findings are consistent with anecdotal evidence about Williams syndrome and provide evidence that mentalizing is a distinct cognitive domain. This spared cognitive capacity may be linked to the relative sparing of limbic-cerebellar neural substrate in Williams syndrome, which is also connected to cortico-frontal regions that are known to be involved in understanding complex mental states.