Williams syndrome (WS) is a neurodevelopmental disorder characterized by severe visuospatial deficits, particularly affecting spatial navigation and wayfinding. Creating egocentric (viewer-dependent) and allocentric (viewer-independent) representations of space is essential for the development of these abilities. However, it remains unclear whether egocentric and allocentric representations are impaired in WS. In this study, we investigate egocentric and allocentric frames of reference in this disorder. A WS group (n = 18), as well as a chronological age-matched control group (n = 20), a non-verbal mental age-matched control group (n = 20) and a control group with intellectual disability (n = 17), was tested with a computerized and a 3D spatial judgment task. The results showed that WS participants are impaired when performing both egocentric and allocentric spatial judgments even when compared with mental age-matched control participants. This indicates that a substantial deficit affecting both spatial representations is present in WS. The egocentric impairment is in line with the dorsal visual pathway deficit previously reported in WS. Interestingly, the difficulties found in performing allocentric spatial judgments give important cues to better understand the ventral visual functioning in WS. (JINS, 2013, 19, 1–9)

Among children born at extremely low birthweight (ELBW: <1000 g at birth) there is an association between the presence of periventricular brain injury (PVBI) and lowered performance on tests of reading and spelling ability. The present study was designed to determine if this association might be related to underlying dysfunction in the subcortical magnocellular visual pathway or its cortical targets in the dorsal stream, a prediction motivated by the magnocellular theory of dyslexia. Thirty-five ELBW children were divided into two groups based upon the presence or absence of PVBI (no PVBI, n = 11; PVBI, n = 24). The performance of these two groups was compared to that of a group of healthy full term children (n = 12) on a motion-defined form recognition task believed to tap into the functioning of the magnocellular pathway and/or the dorsal stream. ELBW children did, in fact, show a striking impairment on this task, with 71% of the sample performing at a level more than three standard deviations below the mean of full term controls. Surprisingly, their difficulties were not found to be related to either the presence of brain injury (verified by neonatal cranial ultrasound) or to problems with reading or spelling. An association was documented, however, between difficulties with motion processing and performance on several subtests of the Performance IQ scale of the Wechsler Intelligence Scale for Children–Third Edition. This latter finding is consistent with our earlier suggestion that magnocellular pathway/dorsal stream dysfunction may underlie problems with visuospatial and visuomotor performance in this population. (JINS, 2003, 9, 440–449.)