Auditory Attraction: Activation of Visual Cortex by Music and Sound in Williams Syndrome

Abstract Williams syndrome is a genetic neurodevelopmental disorder with a distinctive phenotype, including cognitive–linguistic features, nonsocial anxiety, and a strong attraction to music. We performed functional MRI studies examining brain responses to musical and other types of auditory stimuli in young adults with Williams syndrome and typically developing controls. In Study 1, the Williams syndrome group exhibited unforeseen activations of the visual cortex to musical stimuli, and it was this novel finding that became the focus of two subsequent studies. Using retinotopy, color localizers, and additional sound conditions, we identified specific visual areas in subjects with Williams syndrome that were activated by both musical and nonmusical auditory stimuli. The results, similar to synesthetic-like experiences, have implications for cross-modal sensory processing in typical and atypical neurodevelopment.

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Attention allocation to facial expressions of emotion among persons with Williams and Down syndromes

Development and Psychopathology ◽

10.1017/s0954579416001231 ◽

2016 ◽

Vol 29 (4) ◽

pp. 1189-1197 ◽

Cited By ~ 3

Author(s):

Karen J. Goldman ◽

Cory Shulman ◽

Yair Bar-Haim ◽

Rany Abend ◽

Jacob A. Burack

Keyword(s):

Down Syndrome ◽

Facial Expressions ◽

Williams Syndrome ◽

Attention Capture ◽

Typically Developing ◽

Typically Developing Children ◽

Social Approach ◽

Mental Age ◽

Williams Syndrome Group ◽

Facial Expressions Of Emotion

AbstractIndividuals with Williams syndrome and those with Down syndrome are both characterized by heightened social interest, although the manifestation is not always similar. Using a dot-probe task, we examined one possible source of difference: allocation of attention to facial expressions of emotion. Thirteen individuals with Williams syndrome (mean age = 19.2 years, range = 10–28.6), 20 with Down syndrome (mean age = 18.8 years, range = 12.1–26.3), and 19 typically developing children participated. The groups were matched for mental age (mean = 5.8 years). None of the groups displayed a bias to angry faces. The participants with Williams syndrome showed a selective bias toward happy faces, whereas the participants with Down syndrome behaved similarly to the typically developing participants with no such bias. Homogeneity in the direction of bias was markedly highest in the Williams syndrome group whose bias appeared to result from enhanced attention capture. They appeared to rapidly and selectively allocate attention toward positive facial expressions. The complexity of social approach behavior and the need to explore other aspects of cognition that may be implicated in this behavior in both syndromes is discussed.

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Cortisol Reactivity and Performance Abilities in Social Situations in Adults with Williams Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-118.5.381 ◽

2013 ◽

Vol 118 (5) ◽

pp. 381-393 ◽

Cited By ~ 7

Author(s):

Miriam D. Lense ◽

Elisabeth M. Dykens

Keyword(s):

Williams Syndrome ◽

Psychosocial Stress ◽

Neurodevelopmental Disorder ◽

Typically Developing ◽

Social Situations ◽

Cortisol Reactivity ◽

Baseline Cortisol ◽

Performance Abilities ◽

Cortisol Levels ◽

And Performance

Abstract Williams syndrome (WS) is a neurodevelopmental disorder associated with hypersociability and anxiety. However, little is known about how these salient aspects of the phenotype are related or their underlying physiology. We examined cortisol reactivity in WS because cortisol is responsive to psychosocial stress. Compared to typically developing adults, adults with WS had a significant cortisol decrease in response to a challenging cognitive battery. In contrast, cortisol levels in WS stayed stable in response to a solo musical performance, and baseline cortisol levels were significantly associated with musical skill. Results indicate that people with WS respond differentially to different socially-loaded situations. Implications for salience and arousal in cognitive and social situations are discussed.

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Decreased Neuron Density and Increased Glia Density in the Ventromedial Prefrontal Cortex (Brodmann Area 25) in Williams Syndrome

Brain Sciences ◽

10.3390/brainsci8120209 ◽

2018 ◽

Vol 8 (12) ◽

pp. 209 ◽

Cited By ~ 5

Author(s):

Linnea Wilder ◽

Kari Hanson ◽

Caroline Lew ◽

Ursula Bellugi ◽

Katerina Semendeferi

Keyword(s):

Prefrontal Cortex ◽

Williams Syndrome ◽

Statistical Significance ◽

Neurodevelopmental Disorder ◽

Brodmann Area ◽

Ventromedial Prefrontal Cortex ◽

Typically Developing ◽

Frontal Pole ◽

Neuron Density ◽

Human Brains

Williams Syndrome (WS) is a neurodevelopmental disorder caused by a deletion of 25–28 genes on chromosome 7 and characterized by a specific behavioral phenotype, which includes hypersociability and anxiety. Here, we examined the density of neurons and glia in fourteen human brains in Brodmann area 25 (BA 25), in the ventromedial prefrontal cortex (vmPFC), using a postmortem sample of five adult and two infant WS brains and seven age-, sex- and hemisphere-matched typically developing control (TD) brains. We found decreased neuron density, which reached statistical significance in the supragranular layers, and increased glia density and glia to neuron ratio, which reached statistical significance in both supra- and infragranular layers. Combined with our previous findings in the amygdala, caudate nucleus and frontal pole (BA 10), these results in the vmPFC suggest that abnormalities in frontostriatal and frontoamygdala circuitry may contribute to the anxiety and atypical social behavior observed in WS.

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Myelin densities in retinotopically defined dorsal visual areas of the macaque

Brain Structure and Function ◽

10.1007/s00429-021-02363-z ◽

2021 ◽

Author(s):

Xiaolian Li ◽

Qi Zhu ◽

Wim Vanduffel

Keyword(s):

Visual Cortex ◽

Visual Field ◽

Cortical Thickness ◽

New World Monkeys ◽

Isotropic Resolution ◽

Density Mapping ◽

Area V2 ◽

Visual Areas ◽

Density Results

AbstractThe visuotopic organization of dorsal visual cortex rostral to area V2 in primates has been a longstanding source of controversy. Using sub-millimeter phase-encoded retinotopic fMRI mapping, we recently provided evidence for a surprisingly similar visuotopic organization in dorsal visual cortex of macaques compared to previously published maps in New world monkeys (Zhu and Vanduffel, Proc Natl Acad Sci USA 116:2306–2311, 2019). Although individual quadrant representations could be robustly delineated in that study, their grouping into hemifield representations remains a major challenge. Here, we combined in-vivo high-resolution myelin density mapping based on MR imaging (400 µm isotropic resolution) with fine-grained retinotopic fMRI to quantitatively compare myelin densities across retinotopically defined visual areas in macaques. Complementing previously documented differences in populational receptive-field (pRF) size and visual field signs, myelin densities of both quadrants of the dorsolateral posterior area (DLP) and area V3A are significantly different compared to dorsal and ventral area V3. Moreover, no differences in myelin density were observed between the two matching quadrants belonging to areas DLP, V3A, V1, V2 and V4, respectively. This was not the case, however, for the dorsal and ventral quadrants of area V3, which showed significant differences in MR-defined myelin densities, corroborating evidence of previous myelin staining studies. Interestingly, the pRF sizes and visual field signs of both quadrant representations in V3 are not different. Although myelin density correlates with curvature and anticorrelates with cortical thickness when measured across the entire cortex, exactly as in humans, the myelin density results in the visual areas cannot be explained by variability in cortical thickness and curvature between these areas. The present myelin density results largely support our previous model to group the two quadrants of DLP and V3A, rather than grouping DLP- with V3v into a single area VLP, or V3d with V3A+ into DM.

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Pathways to language: a naturalistic study of children with Williams syndrome and children with Down syndrome

Journal of Child Language ◽

10.1017/s0305000912000475 ◽

2012 ◽

Vol 40 (1) ◽

pp. 106-138 ◽

Cited By ~ 12

Author(s):

YONATA LEVY ◽

ARIELA EILAM

Keyword(s):

Down Syndrome ◽

Data Collection ◽

Williams Syndrome ◽

Language Delay ◽

Typically Developing ◽

Naturalistic Study ◽

Children With Down Syndrome ◽

Network Properties ◽

Development Of Language ◽

Language Onset

ABSTRACTThis is a naturalistic study of the development of language in Hebrew-speaking children with Williams syndrome (WS) and children with Down syndrome (DS), whose MLU extended from 1·0 to 4·4. Developmental curves over the entire span of data collection revealed minor differences between children with WS, children with DS, and typically developing (TD) controls of similar MLU. Development within one calendar year showed remarkable synchrony among the variables. However, age of language onset and pace of acquisition departed significantly from normal timing. It is argued that in view of the centrality of genetic timing and the network properties of cognition, normal schedules are crucial determinants of intact development. Consequently, with respect to neurodevelopmental syndromes, the so-called ‘language delay’ is indicative of deviance that is likely to impact development in critical ways.

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Top-down control of visual cortex by the frontal eye fields through oscillatory realignment

Nature Communications ◽

10.1038/s41467-021-21979-7 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Domenica Veniero ◽

Joachim Gross ◽

Stephanie Morand ◽

Felix Duecker ◽

Alexander T. Sack ◽

...

Keyword(s):

Visual Cortex ◽

Transcranial Magnetic Stimulation ◽

Visual Perception ◽

Magnetic Stimulation ◽

Single Pulse ◽

Frontal Eye Fields ◽

Phase Reset ◽

Top Down ◽

Visual Areas ◽

Beta Frequency

AbstractVoluntary allocation of visual attention is controlled by top-down signals generated within the Frontal Eye Fields (FEFs) that can change the excitability of lower-level visual areas. However, the mechanism through which this control is achieved remains elusive. Here, we emulated the generation of an attentional signal using single-pulse transcranial magnetic stimulation to activate the FEFs and tracked its consequences over the visual cortex. First, we documented changes to brain oscillations using electroencephalography and found evidence for a phase reset over occipital sites at beta frequency. We then probed for perceptual consequences of this top-down triggered phase reset and assessed its anatomical specificity. We show that FEF activation leads to cyclic modulation of visual perception and extrastriate but not primary visual cortex excitability, again at beta frequency. We conclude that top-down signals originating in FEF causally shape visual cortex activity and perception through mechanisms of oscillatory realignment.

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Adaptive Integration in the Visual Cortex by Depressing Recurrent Cortical Circuits

Neural Computation ◽

10.1162/neco.2008.06-07-546 ◽

2008 ◽

Vol 20 (7) ◽

pp. 1847-1872 ◽

Cited By ~ 24

Author(s):

Mark C. W. van Rossum ◽

Matthijs A. A. van der Meer ◽

Dengke Xiao ◽

Mike W. Oram

Keyword(s):

Visual Cortex ◽

Physiological Data ◽

High Contrast ◽

Cortical Circuits ◽

Response Latencies ◽

Low Contrast ◽

Visual Areas ◽

Higher Visual Areas ◽

Recurrent Connections

Neurons in the visual cortex receive a large amount of input from recurrent connections, yet the functional role of these connections remains unclear. Here we explore networks with strong recurrence in a computational model and show that short-term depression of the synapses in the recurrent loops implements an adaptive filter. This allows the visual system to respond reliably to deteriorated stimuli yet quickly to high-quality stimuli. For low-contrast stimuli, the model predicts long response latencies, whereas latencies are short for high-contrast stimuli. This is consistent with physiological data showing that in higher visual areas, latencies can increase more than 100 ms at low contrast compared to high contrast. Moreover, when presented with briefly flashed stimuli, the model predicts stereotypical responses that outlast the stimulus, again consistent with physiological findings. The adaptive properties of the model suggest that the abundant recurrent connections found in visual cortex serve to adapt the network's time constant in accordance with the stimulus and normalizes neuronal signals such that processing is as fast as possible while maintaining reliability.

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Word learning in a special population: do individuals with Williams syndrome obey lexical constraints?

Journal of Child Language ◽

10.1017/s0305000997003279 ◽

1997 ◽

Vol 24 (3) ◽

pp. 737-765 ◽

Cited By ~ 43

Author(s):

TASSOS STEVENS ◽

ANNETTE KARMILOFF-SMITH

Keyword(s):

Williams Syndrome ◽

Word Learning ◽

Neurodevelopmental Disorder ◽

Fast Mapping ◽

Mutual Exclusivity ◽

Vocabulary Size ◽

Normal Children ◽

New Words ◽

Adolescents And Adults ◽

Lexical Constraints

Williams syndrome (WS), a rare neurodevelopmental disorder, is of special interest to developmental psycholinguists because of its uneven linguistico-cognitive profile of abilities and deficits. One proficiency manifest in WS adolescents and adults is an unusually large vocabulary despite serious deficits in other domains. In this paper, rather than focus on vocabulary size, we explore the processes underlying vocabulary acquisition, i.e. how new words are learned. A WS group was compared to groups of normal MA-matched controls in the range 3–9 years in four different experiments testing for constraints on word learning. We show that in construing the meaning of new words, normal children at all ages display fast mapping and abide by the constraints tested: mutual exclusivity, whole object and taxonomic. By contrast, while the WS group showed fast mapping and the mutual exclusivity constraint, they did not abide by the whole object or taxonomic constraints. This suggests that measuring only the size of WS vocabulary can distort conclusions about the normalcy of WS language. Our study shows that despite equivalent behaviour (i.e. vocabulary test age), the processes underlying how vocabulary is acquired in WS follow a somewhat different path from that of normal children and that the atypically developing brain is not necessarily a window on normal development.

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