III. Electrophysiological Studies of Face Processing in Williams Syndrome

Williams Syndrome (WMS) is a genetically based disorder characterized by pronounced variability in performance across different domains of cognitive functioning. This study examined brain activity linked to face-processing abilities, which are typically spared in individuals with WMS. Subjects watched photographic pairs of upright or inverted faces and indicated if the second face matched or did not match the first face. Results from a previous study with normal adults showed dramatic differences in the timing and distribution of ERP effects linked to recognition of upright and inverted faces. In normal adults, upright faces elicited ERP differences to matched vs. mismatched faces at approximately 320 msec (N320) after the onset of the second stimulus. This N320 effect was largest over anterior regions of the right hemisphere. In contrast, the mismatch/match effect for inverted faces consisted of a large positive component between 400 and 1000 msec (P500) that was largest over parietal regions and was symmetrical. In contrast to normal adults, WMS subjects showed an N320-mismatch effect for both upright and inverted faces. Additionally, the WMS subjects did not display the N320 right-hemisphere asymmetry observed in the normal adults. WMS subjects also displayed an abnormally small negativity at 100 msec (N100) and an abnormally large negativity at 200 msec (N200) to both upright and inverted faces. This ERP pattern was observed in all subjects with WMS but was not observed in the normal controls. These results may be linked to increased attention to faces in subjects with WMS and might be specific to the disorder. These results were consistent with our ERP studies of language processing in WMS, which suggested abnormal cerebral specialization for spared cognitive functions in individuals with WMS.

[1]  O. Braddick,et al.  A specific deficit of dorsal stream function in Williams' syndrome , 1997, Neuroreport.

[2]  Susan Carey,et al.  Upright and Inverted Faces: The Right Hemisphere Knows the Difference , 1978, Cortex.

[3]  A. Damasio,et al.  Face agnosia and the neural substrates of memory. , 1990, Annual review of neuroscience.

[4]  Helen J. Neville,et al.  Effects of altered auditory sensitivity and age of language acquisition on the development of language-relevant neural systems: Preliminary studies of Williams syndrome. , 1994 .

[5]  T. Allison,et al.  Face-sensitive regions in human extrastriate cortex studied by functional MRI. , 1995, Journal of neurophysiology.

[6]  John W. Tukey,et al.  Exploratory Data Analysis. , 1979 .

[7]  Ursula Bellugi,et al.  Bridging cognition, the brain and molecular genetics: evidence from Williams syndrome , 1999, Trends in Neurosciences.

[8]  T. Allison,et al.  Electrophysiological studies of human face perception. III: Effects of top-down processing on face-specific potentials. , 1999, Cerebral cortex.

[9]  T. Valentine.  Upside-down faces: a review of the effect of inversion upon face recognition. , 1988, British journal of psychology.

[10]  G. Winocur,et al.  What Is Special about Face Recognition? Nineteen Experiments on a Person with Visual Object Agnosia and Dyslexia but Normal Face Recognition , 1997, Journal of Cognitive Neuroscience.

[11]  S. Hillyard,et al.  Modulations of sensory-evoked brain potentials indicate changes in perceptual processing during visual-spatial priming. , 1991, Journal of experimental psychology. Human perception and performance.

[12]  Werner Sommer,et al.  Event-related potentials and models of performance asymmetries in face and word recognition , 1994, Neuropsychologia.

[13]  H. Neville,et al.  The Neurobiology of Sensory and Language Processing in Language-Impaired Children , 1993, Journal of Cognitive Neuroscience.

[14]  T. Allison,et al.  Face-Specific Processing in the Human Fusiform Gyrus , 1997, Journal of Cognitive Neuroscience.

[15]  M. Farah,et al.  What causes the face inversion effect? , 1995, Journal of experimental psychology. Human perception and performance.

[16]  Leslie G. Ungerleider,et al.  The functional organization of human extrastriate cortex: a PET-rCBF study of selective attention to faces and locations , 1994, The Journal of neuroscience : the official journal of the Society for Neuroscience.

[17]  R W Sperry,et al.  Reception of bilateral chimeric figures following hemispheric deconnexion. , 1972, Brain : a journal of neurology.

[18]  M. Farah,et al.  What is "special" about face perception? , 1998, Psychological review.

[19]  N. Kanwisher,et al.  The Fusiform Face Area: A Module in Human Extrastriate Cortex Specialized for Face Perception , 1997, The Journal of Neuroscience.

[20]  M J Farah,et al.  Second-order relational properties and the inversion effect: Testing a theory of face perception , 1991, Perception & psychophysics.

[21]  R. Yin,et al.  Face recognition by brain-injured patients: a dissociable ability? , 1970, Neuropsychologia.

[22]  Ursula Bellugi,et al.  Seeing either the forest or the trees: Dissociation in visuospatial processing , 1989, Brain and Cognition.

[23]  Joseph M. Petrosko,et al.  Wechsler Intelligence Scale for Children—Revised, 1974. David Wechsler , 1975 .

[24]  S. Carey,et al.  Development of face recognition: A maturational component? , 1980 .

[25]  S. Luck,et al.  Electrocortical substrates of visual selective attention , 1993 .

[26]  T. Allison,et al.  Electrophysiological Studies of Face Perception in Humans , 1996, Journal of Cognitive Neuroscience.

[27]  G. Rhodes.  Lateralized processes in face recognition. , 1985, British journal of psychology.

[28]  K. Nakayama,et al.  The effect of face inversion on the human fusiform face area , 1998, Cognition.

[29]  C. C. Wood,et al.  Scalp distributions of event-related potentials: an ambiguity associated with analysis of variance models. , 1985, Electroencephalography and clinical neurophysiology.

[30]  T. Allison,et al.  Electrophysiological studies of human face perception. I: Potentials generated in occipitotemporal cortex by face and non-face stimuli. , 1999, Cerebral cortex.

[31]  J. Haxby,et al.  Functional Magnetic Resonance Imaging of Human Visual Cortex during Face Matching: A Comparison with Positron Emission Tomography , 1996, NeuroImage.

[32]  N. Kanwisher,et al.  Covert visual attention modulates face-specific activity in the human fusiform gyrus: fMRI study. , 1998, Journal of neurophysiology.

[33]  E. Renzi,et al.  Prosopagnosia in two patients with CT scan evidence of damage confined to the right hemisphere , 1986, Neuropsychologia.

[34]  T. Allison,et al.  Electrophysiological studies of human face perception. II: Response properties of face-specific potentials generated in occipitotemporal cortex. , 1999, Cerebral cortex.

[35]  J. Sergent,et al.  Functional neuroanatomy of face and object processing. A positron emission tomography study. , 1992, Brain : a journal of neurology.

[36]  Stephan Eliez,et al.  IV. Neuroanatomy of Williams Syndrome: A High-Resolution MRI Study , 2000, Journal of Cognitive Neuroscience.

[37]  E. De Renzi,et al.  Prosopagnosia in two patients with CT scan evidence of damage confined to the right hemisphere. , 1986, Neuropsychologia.

[38]  David E. Meyer,et al.  Electrocortical Substrates of Visual Selective Attention , 1993 .

[39]  Electrophysiological correlations of face processing by adults and 6-month-old infants , 1998 .

[40]  M. Kutas,et al.  Electrophysiology of cognitive processing. , 1983, Annual review of psychology.

[41]  T. Allison,et al.  Face recognition in human extrastriate cortex. , 1994, Journal of neurophysiology.

[42]  Svein Magnussen,et al.  Patterns of Perceptual Asymmetry in Processing Facial Expression , 1994, Cortex.

[43]  O. Grüsser,et al.  The Search for Face-Specific Evoked Potentials , 1989 .

[44]  M. Kutas,et al.  Event-related brain potentials to semantically inappropriate and surprisingly large words , 1980, Biological Psychology.

[45]  R. Yin.  Looking at Upside-down Faces , 1969 .

[46]  M R Harter,et al.  Hemispheric Differences in Event‐Related Potential Measures of Selective Attention a , 1984, Annals of the New York Academy of Sciences.

[47]  Ursula Bellugi,et al.  V. Multi-Level Analysis of Cortical Neuroanatomy in Williams Syndrome , 2000, Journal of Cognitive Neuroscience.

[48]  S. Carey,et al.  Why faces are and are not special: an effect of expertise. , 1986, Journal of experimental psychology. General.

[49]  G. V. Van Hoesen,et al.  Prosopagnosia , 1982, Neurology.

[50]  C. M. Mooney.  Age in the development of closure ability in children. , 1957, Canadian journal of psychology.

[51]  T. Allison,et al.  Human extrastriate visual cortex and the perception of faces, words, numbers, and colors. , 1994, Cerebral cortex.

[52]  J. Cohen,et al.  P300, stimulus intensity, modality, and probability. , 1996, International journal of psychophysiology : official journal of the International Organization of Psychophysiology.

[53]  Werner Sommer,et al.  Contributions of stimulus encoding and memory search to right hemisphere superiority in face recognition: Behavioural and electrophysiological evidence , 1991, Neuropsychologia.

[54]  David I. Perrett,et al.  Event-related potentials and the matching of familiar and unfamiliar faces , 1988, Neuropsychologia.

[55]  Ursula Bellugi,et al.  I. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses , 2000, Journal of Cognitive Neuroscience.

[56]  S. Carey,et al.  From piecemeal to configurational representation of faces. , 1977, Science.

[57]  S. Hillyard,et al.  Modulations of sensory-evoked brain potentials indicate changes in perceptual processing during visual-spatial priming. , 1991, Journal of experimental psychology. Human perception and performance.

[58]  S. Geisser,et al.  On methods in the analysis of profile data , 1959 .

[59]  Leslie G. Ungerleider,et al.  Dissociation of object and spatial visual processing pathways in human extrastriate cortex. , 1991, Proceedings of the National Academy of Sciences of the United States of America.

[60]  M. Masure,et al.  Une nouvelle observation d'agnosie d'objet. deficit de l'association ou de la categorisation, specifique de la modalite visuelle? ☆ , 1974 .