Electrophysiological study of face inversion effects in Williams syndrome.
OBJECTIVE
In order to evaluate whether face perception is intact or not in Williams syndrome (WS), the face inversion effects (FIE) in the event-related potential (ERP) or magnetoencephalography (MEG) were investigated in three teenaged patients with WS.


METHODS
Responses to the inverted faces and upright faces were compared using MEG for one 13year old girl with WS (subject A) and ERP for boys with WS at 16 and 14years of age (subjects B and C, respectively).


RESULTS
Although age-matched control children showed FIE in both MEG and ERP studies, two subjects (A and B) with WS showed no FIE at all. The neurophysiological data of ERP in subject B was significantly different from those of the age-matched controls. On the other hand, a boy with WS (subject C) showed typical FIE in the same manner as the age-matched controls.


CONCLUSIONS
The difference between those with or without FIE was not explained merely by the chronological age, a simple delay in mental age or in the ability to discriminate among upright faces. The absence of FIE may be related to the severity of a deficit in the dorsal pathway function that is characteristic to the syndrome.
Department of Integrative Physiology, National Institute for Physiological Sciences, Japan; Institute for Developmental Research, Aichi Human Service Center, Japan. mihon@inst-hsc.jp
Miho
M
Nakamura
Shoko
S
Watanabe
Masumi
M
Inagaki
Masahiro
M
Hirai
Kensaku
K
Miki
Yukiko
Y
Honda
Ryusuke
R
Kakigi
D000293:Adolescent; D002648:Child; D004569:Electroencephalography; D005071:Evoked Potentials / Q000502:physiology*; D005145:Face; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography; D008297:Male; D010364:Pattern Recognition, Visual / Q000502:physiology*; D010468:Perceptual Disorders / Q000209:etiology*; D010775:Photic Stimulation; D011930:Reaction Time; D018980:Williams Syndrome / Q000150:complications*