Where is the 'subjective straight ahead' in Williams syndrome?

Abstract

Individuals with Williams Syndrome (WS) are known to have particular difficulties when performing visuo-spatial tasks, which could be related to their difficulties in using a specific reference system to determine spatial relations. The aim of the present study was to assess the internal representation of the body's sagittal plane, which is an important benchmark for an egocentric frame of reference. The results of 18 WS individuals (mean age=20.5±9.2years) on the subjective straight ahead (SSA) task were compared with those of two healthy control groups composed of 36 participants matched on chronological age matched on chronological age (CA) and 30 young children matched on non-verbal intellectual ability (YC). Individuals with WS showed a significant left deviation on the SSA body's sagittal plane representation compared with the chronological age control group and a marginal left deviation compared with the young children control group. A comparison with the objective SA (0°) showed a significant leftward deviation in the WS group but not in the two control groups. Individuals with WS showed a significant leftward deviation in the SSA task. This bias of the body's longitudinal axe representation could have a negative impact on the use of an egocentric reference system, which could be the cause for their difficulties in defining spatial relations (e.g. location and orientation) necessary for performing spatial tasks.