Publications by authors named "Inga-Sophia Knoth"

Quantifying cognitive potential relies on psychometric measures that do not directly reflect cortical activity. While the relationship between cognitive ability and resting state EEG signal dynamics has been extensively studied in children with below-average cognitive performances, there remains a paucity of research focusing on individuals with normal to above-average cognitive functioning. This study aimed to elucidate the resting EEG dynamics in children aged four to 12 years across normal to above-average cognitive potential.

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Background: Fragile X syndrome (FXS) and autism spectrum disorder (ASD) are neurodevelopmental conditions that often have a substantial impact on daily functioning and quality of life. FXS is the most common cause of inherited intellectual disability (ID) and the most common monogenetic cause of ASD. Previous literature has shown that electrophysiological activity measured by electroencephalogram (EEG) during resting state is perturbated in FXS and ASD.

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Children with neurofibromatosis type 1 (NF1) are at increased risk of developing cognitive problems, including attention deficits and learning difficulties. Alterations in brain response to repetition and change have been evidenced in other genetic conditions associated with cognitive dysfunctions. Whether the integrity of these fundamental neural responses is compromised in school-aged children with NF1 is still unknown.

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Neurodevelopmental disorders (NDDs) are mostly diagnosed around the age of 4-5 years, which is too late considering that the brain is most susceptive to interventions during the first two years of life. Currently, diagnosis of NDDs is based on observed behaviors and symptoms, but identification of objective biomarkers would allow for earlier screening. In this longitudinal study, we investigated the relationship between repetition and change detection responses measured using an EEG oddball task during the first year of life and at two years of age, and cognitive abilities and adaptive functioning during preschool years (4 years old).

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Background: Neurofibromatosis type 1 (NF1) is a genetic disorder often associated with cognitive dysfunctions, including a high occurrence of deficits in visuoperceptual skills. The neural underpinnings of these visuoperceptual deficits are not fully understood. We used steady-state visual evoked potentials (SSVEPs) to investigate possible alterations in the synchronization of neural activity in the occipital cortex of children with NF1.

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Neuronal repetition effect (repetition suppression and repetition enhancement) and change detection responses are fundamental brain responses that have implications in learning and cognitive development in infants and children. Studies have shown altered neuronal repetition and change detection responses in various clinical populations. However, the developmental course of these neuronal responses from infancy through childhood is still unknown.

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Article Synopsis
  • Fragile X syndrome (FXS) is a genetic disorder linked to neurophysiological changes, such as increased cortical excitability and specific EEG alterations that relate to developmental delays.
  • This study compared EEG power measures and signal complexity between FXS participants and neurotypical controls, revealing increased gamma and decreased alpha power in FXS, along with reduced signal complexity, particularly at higher scales.
  • The findings highlight that altered signal complexity is a key indicator of brain changes in FXS, helping to improve electrophysiological biomarkers for brain maturation in this population.
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Objective: Our goal was to assess development, cognition and behaviour following an initial complex febrile seizure (FS), at onset and school age, in the context of known risk factors for cognitive development.

Methods: Two cohorts were recruited. Thirty-five infants with an initial complex FS were assessed within the first year post-seizure and compared to 30 controls (simple FS) based on measures of cognitive, motor and language development, behaviour and emotions.

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Article Synopsis
  • Genomic copy number variants (CNVs), particularly deletions and duplications, have been linked to cognitive ability, but their specific effects on intelligence are still not fully understood, especially for duplications.
  • The study involved analyzing CNVs from over 24,000 individuals and used statistical models to show that deletions decrease intelligence more significantly than duplications, with certain genes being intolerant to haploinsufficiency playing a key role.
  • The findings indicate that while a small fraction of genes has a significant negative impact on intelligence, the overall effects on cognition may stem from complex interactions within the genome rather than just a few specific pathways.
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Learning abilities are present in infancy, as they are critical for adaptation. From simple habituation and novelty responses to stimuli, learning capacities evolve throughout the lifespan. During development, learning abilities become more flexible and integrated across sensory modalities, allowing the encoding of more complex information, and in larger amounts.

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Adolescent idiopathic scoliosis (AIS) is a multifactorial disorder characterized by a tridimensional deformation of the spine. AIS pathophysiology is still unclear and its aetiology is unknown. Results from several studies revealed balance control alterations in adolescents with AIS suggesting cortical sensorimotor processing impairments.

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Objective: Studies have identified mild but persistent cognitive and functional deficits, which could be linked to each other, in children with complex febrile seizures (FS). Our aim was to investigate differences in brain activity in children with a history of complex FS, through a study paradigm notably associated with the development of learning capacities and using electroencephalographic (EEG) signal. To further increase our understanding of these differences, complex FS were studied separately depending on their type.

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Nutritional deficiencies often precede the diagnosis of cystic fibrosis (CF) in infants, and occur at a stage where the rapidly developing brain is more vulnerable to insult. We aim to compare fat-soluble nutrient status of newly diagnosed non-screened infants with CF to that of healthy infants, and explore the association with neurodevelopment evaluated by electroencephalography (EEG). Our results show that CF infants had lower levels of all fat-soluble vitamins and docosahexaenoic acid (DHA) compared to controls.

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Objective: This study aims at examining the cortical dynamics of sensorimotor information processing related to balance control in participants with adolescent idiopathic scoliosis (AIS) and in age-matched controls (CTL).

Methods: Cortical dynamics during standing balance control were assessed in 13 girls with AIS and 13 age-matched controls using electroencephalography. Time-frequency analysis were used to determine frequency power during ankle proprioception alteration (ankle tendons co-vibration interval) or reintegration of ankle proprioception (post-vibration interval) with or without vision.

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Background: Studies suggest that the relationship between seizures and stress starts early in life. However, evidence of long-term altered stress reactivity following early-life seizures is lacking. Our objectives were to assess alterations in stress hormone reactivity in children with past febrile seizures (FS) and investigate how these alterations relate to clinical characteristics.

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Over activation of the hypothalamo-pituitary-adrenal (HPA) axis in stress situations is known to influence learning and memory. In adults, an inverted-U shape relationship between acute stress, and learning and memory has been demonstrated. Whether this model fits learning performances in infants is unknown.

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For newborns and neonates, ultrasound (US) is the most common imaging modality used for examinations due to its accessibility and ease of use. However, precise volume measurements remain limited in 2D, while MRI in newborns is typically avoided because of immobilization issues which may require sedation. The objective of this study is to assess and validate the lateral ventricular and total brain volumes obtained with an automatic segmentation method using cerebral trans-fontanelle 3D US.

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Background: Fragile X syndrome (FXS) is a neurodevelopmental genetic disorder causing cognitive and behavioural deficits. Repetition suppression (RS), a learning phenomenon in which stimulus repetitions result in diminished brain activity, has been found to be impaired in FXS. Alterations in RS have been associated with behavioural problems in FXS; however, relations between RS and intellectual functioning have not yet been elucidated.

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Background: Fragile X Syndrome (FXS) is the most common monogenic form of intellectual disability and one of the few known monogenic causes of autism. It is caused by a trinucleotide repeat expansion in the FMR1 ('Fragile X Mental Retardation 1') gene, which prevents expression of the 'Fragile X Mental Retardation Protein' (FMRP). In FXS, the absence of FMRP leads to altered structural and functional development of the synapse, while preventing activity-based synapse maturation and synaptic pruning, which are essential for normal brain development and cognitive development.

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Based on the continuity hypothesis of dreaming, a study was designed to examine whether time of day within the dream was related to dream emotions. A sample of 1,612 dreams reported by 444 participants was analyzed. As predicted, dream scenarios set at nighttime were associated with less positive and more negative emotions compared to dream scenarios set at other times of the day.

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