Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome

Gombos, Ferenc; Bódizs, Róbert; Kovács, Ilona

doi:10.1038/s41598-017-06280-2

Cited by 5 publications

(7 citation statements)

References 37 publications

(64 reference statements)

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“…The firing rate was larger in WT animals, and the significant windows are indicated above with a black line (p < 0.05) high integration capacity (lower path length) and low segregation levels (modularity); alterations that are especially notorious at gamma-like frequency bands. These results are in agreement with previous findings in humans reporting increased overall cortical connectivity [11] and suggest that the cognitive deficits previously reported in this murine model of WBS [26] may be associated with the interruption of the equilibrium between integration and segregation at gamma frequencies. The abovementioned alterations in the morphology and number of dendritic spines at the level of the frontal cortex might explain, at least partially, the origin of these functional abnormalities, which may hamper the capacity of the cortical network to distribute and integrate information in order to execute specialized functions.…”

Section: Discussionsupporting

confidence: 93%

“…Alterations in specific patterns of neuronal synchronization are characteristic of different neuropsychiatric and neurodevelopmental conditions [45,46]. Indeed, previous studies in WBS patients have shown alterations in the overall level of cortical coherence [10,11]. Thus, in order to test whether the alterations in synchronization we observed at the level of the frontal areas were a general phenomenon that could be affecting the integrity of the cortical network in our mouse model, we next studied the overall functional connectivity of the cortical network.…”

Section: Resultsmentioning

confidence: 94%

“…Specifically, sleep cycles are fragmented and disorganized, REM sleep is decreased, and the frequency content of NREM sleep is unbalanced [7,8]. In addition, alterations in functional connectivity have been described during resting states and natural sleep in individuals suffering from WBS [9][10][11].…”

Section: Introductionmentioning

confidence: 99%

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Altered Neocortical Dynamics in a Mouse Model of Williams–Beuren Syndrome

et al. 2019

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Williams-Beuren syndrome (WBS) is a rare neurodevelopmental disorder characterized by moderate intellectual disability and learning difficulties alongside behavioral abnormalities such as hypersociability. Several structural and functional brain alterations are characteristic of this syndrome, as well as disturbed sleep and sleeping patterns. However, the detailed physiological mechanisms underlying WBS are mostly unknown. Here, we characterized the cortical dynamics in a mouse model of WBS previously reported to replicate most of the behavioral alterations described in humans. We recorded the laminar local field potential generated in the frontal cortex during deep anesthesia and characterized the properties of the emergent slow oscillation activity. Moreover, we performed micro-electrocorticogram recordings using multielectrode arrays covering the cortical surface of one hemisphere. We found significant differences between the cortical emergent activity and functional connectivity between wild-type mice and WBS model mice. Slow oscillations displayed Up states with diminished firing rate and lower high-frequency content in the gamma range. Lower firing rates were also recorded in the awake WBS animals while performing a marble burying task and could be associated with the decreased spine density and thus synaptic connectivity in this cortical area. We also found an overall increase in functional connectivity between brain areas, reflected in lower clustering and abnormally high integration, especially in the gamma range. These results expand previous findings in humans, suggesting that the cognitive deficits characterizing WBS might be associated with reduced excitability, plus an imbalance in the capacity to functionally integrate and segregate information.

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Section: Discussionsupporting

confidence: 93%

Section: Resultsmentioning

confidence: 94%

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Altered Neocortical Dynamics in a Mouse Model of Williams–Beuren Syndrome

et al. 2019

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“…The lack of statistical significance prevents interpretation of the observed attenuated alpha power in the WS group, however attenuated full- and low-alpha compared to the CA and MA groups was predicted based on the existing literature with other developmental disorders with attentional deficiencies including FXS [ 59 ], DS [ 56 ], and ADHD [ 57 , 82 , 83 ]. In WS, significantly attenuated alpha power has been observed in research using combined EC/EO data [ 67 ], and in sleep states [ 64 , 65 ]. In ADHD, attenuated alpha power during resting states is thought to reflect an ongoing state of cortical hyper-arousal even in the absence of cognitive processing [ 48 ].…”

Section: Discussionmentioning

confidence: 99%

An EEG investigation of alpha and beta activity during resting states in adults with Williams syndrome

et al. 2021

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Background Williams syndrome (WS) is neurodevelopmental disorder characterised by executive deficits of attention and inhibitory processing. The current study examined the neural mechanisms during resting states in adults with WS in order to investigate how this subserves the attention and inhibitory deficits associated with the syndrome. Method Adopting electroencephalography (EEG) methodology, cortical electrical activity was recorded from eleven adults with WS aged 35 + years during Eyes Closed (EC) and Eyes Open (EO) resting states, and compared to that of thirteen typically developing adults matched for chronological age (CA) and ten typically developing children matched for verbal mental ability (MA). Using mixed-design analyses of variance (ANOVA), analyses focused on the full alpha (8–12.5 Hz), low-alpha (8–10 Hz), upper-alpha (10–12.5 Hz), and beta (13–29.5 Hz) bands, as these are thought to have functional significance with attentional and inhibitory processes. Results No significant difference in alpha power were found between the WS and CA groups across all analyses, however a trend for numerically lower alpha power was observed in the WS group, consistent with other developmental disorders characterised by attentional/inhibitory deficits such as Attention Deficit Hyperactivity Disorder (ADHD). In contrast, comparable beta power between the WS and CA groups during both EC/EO conditions suggests that their baseline EEG signature is commensurate with successful attentional processing, though this needs to be interpreted with caution due to the small sample size. Analyses also revealed an unusual trend for low variability in the EEG signature of the WS group, which contradicts the heterogeneity typically observed behaviourally. Conclusions This novel finding of low variability in the EEG spectra in the WS group has been previously associated with poor behavioural performance in ADHD and is highly informative, highlighting future research needs to also consider how the role of low variability in the EEG profile of WS manifests in relation to their behavioural and cognitive profiles.

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“…While other types of motor skills certainly need to be also mapped in this regard, its applications may be useful in educational settings and during career planning before adolescence. Furthermore, the present phenomena should be examined in atypical development since there are neurodevelopmental disorders such as autism spectrum disorder (Oldehinkel et al 2019), attention deficit hyperactivity disorder (Bos et al 2017) and rare disorders (Gombos, Bódizs, and Kovács 2017) that involve both atypically developing long-range brain networks including motor subsystems and altered hand function (Berencsi, Gombos, and Kovács 2016;Rommelse et al 2007;Travers et al 2017).…”

Section: Discussionmentioning

confidence: 99%

Dissociating experience-dependent and maturational changes in fine motor function during adolescence

Berencsi

Gombos

Gerván

et al. 2021

Preprint

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Adolescence is a sensitive period in motor development but little is known about how long-term learning dependent processes shape hand function in tasks of different complexity. We mapped two fundamental aspects of hand function: simple repetitive and complex sequential finger movements, as a function of the length of musical instrumental training. We controlled maturational factors such as chronological and biological age of adolescent female participants (11 to 15 years of age, n=114). We demonstrated that experience improves performance as a function of task complexity, the more complex task being more susceptible for experience driven performance changes. Overall, these results suggest that fine motor skills involving cognitive control and relying on long-range functional brain networks are substantially shaped by experience. On the other hand, performance in a simple repetitive task that explains fine motor speed is primarily shaped by white matter development driven by maturational factors.

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Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome

Cited by 5 publications

References 37 publications

Altered Neocortical Dynamics in a Mouse Model of Williams–Beuren Syndrome

Altered Neocortical Dynamics in a Mouse Model of Williams–Beuren Syndrome

An EEG investigation of alpha and beta activity during resting states in adults with Williams syndrome

Dissociating experience-dependent and maturational changes in fine motor function during adolescence

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