Abstract 22q11.2 Deletion Syndrome (22q11.2DS) is the strongest known molecular risk factor for schizophrenia. Brain responses to auditory stimuli have been studied extensively in schizophrenia and described as potential biomarkers of vulnerability psychosis. We sought understand whether these might aid differentiating individuals with 22q11.2DS a function psychotic symptoms, ultimately serve signals A duration oddball paradigm high-density electrophysiology were used test processing 26...

• Children and adolescents with Cystinosis show similar N1 responses to their age-matched peers. reduced MMNs for longer SOAs. Our results suggest typical auditory processing, but impaired sensory memory in Cystinosis. Cystinosis, a genetic rare disease characterized by cystine accumulation crystallization, significant damage multitude of tissues organs, such as the kidney, thyroid, eye, brain. While Cystinosis' impact on brain function is relatively mild compared its effects other increased...

22q11.2 deletion syndrome (also known as DiGeorge or velo-cardio-facial syndrome) is characterized by increased vulnerability to neuropsychiatric symptoms, with approximately 30% of individuals the going on develop schizophrenia. Clinically, deficits in executive function have been noted this population, but underlying neural processes are not well understood. Using a Go/No-Go response inhibition task conjunction high-density electrophysiological recordings (EEG), we sought investigate...

We investigated visual processing and adaptation in 22q11.2 deletion syndrome (22q11.2DS), a condition characterized by an increased risk for schizophrenia. Visual differences have been described schizophrenia but remain understudied early the disease course. Electrophysiology was recorded during task with different interstimulus intervals to investigate 22q11.2DS (with (22q+) without (22q-) psychotic symptoms), compared control idiopathic groups. Analyses focused on windows of processing....

ABSTRACT Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues organs, including the kidneys brain. Its impact on neural function appears mild relative to its effects other but therapeutic advances have led substantially increased life expectancy, necessitating deeper understanding of neurocognitive function. Behaviorally, some deficits in executive been noted this population, underlying processes are not understood. Using...

Abstract Background Cystinosis, a rare lysosomal storage disease caused by mutations in the CTNS gene, is characterized cystine crystallization and accumulation within multiple tissues, including kidney brain. Its impact on neural function appears mild relative to its effects other organs during early disease, but since therapeutic advances have led substantially increased life expectancy, neurological implications are of increasing interest, necessitating deeper understanding cystinosis...

Abstract Assessment of everyday activities are central to the diagnosis pre-dementia and dementia. Yet, little is known about brain substrates processes that contribute functional impairment, particularly during early stages cognitive decline. We investigated function using a complex gait task in normal older adults stratified by risk impairment. applied novel EEG approach, which combines electroencephalographic with 3D-body tracking technology measure brain-gait dynamics millisecond...

Abstract Background 22q11.2 Deletion Syndrome (22q11.2DS) is the strongest known molecular risk factor for schizophrenia. Brain responses to auditory stimuli have been studied extensively in schizophrenia and described as potential biomarkers of vulnerability psychosis. We sought understand whether these might aid differentiating individuals with 22q11.2DS a function psychotic symptoms, ultimately serve signals Methods A duration oddball paradigm high-density electrophysiology were used test...

ABSTRACT 22q11.2 deletion syndrome (also known as DiGeorge or velo-cardio-facial syndrome) is characterized by increased vulnerability to neuropsychiatric symptoms, with approximately 30% of individuals the going on develop schizophrenia. Clinically, deficits in executive function have been noted this population, but underlying neural processes are not well understood. Using a Go/No-Go response inhibition task conjunction high-density electrophysiological recordings (EEG), we sought...

Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues organs, including the kidneys brain. Its impact on neural function appears mild relative to its effects other but therapeutic advances have led substantially increased life expectancy, necessitating deeper understanding of neurocognitive function. Behavioral difficulties been reported in cystinosis visual visual-processing domain. Very little known, however, about how...

ABSTRACT Background Altered patterns of eye-movements during scene exploration, and atypical gaze preferences in social settings, have long been noted as features the Autism phenotype. While these are typically attributed to differences engagement interests (e.g., for inanimate objects over face stimuli), there also reports differential saccade measures non-social stimuli, raising possibility that fundamental visuo-sensorimotor processing may be at play. Here, we tested plasticity...

Cystinosis, a rare lysosomal storage disease caused by mutations in the CTNS gene, is characterized cystine crystallization and accumulation within multiple tissues, including kidney brain. Its impact on neural function appears mild relative to its effects other organs during early disease, but since therapeutic advances have led substantially increased life expectancy, neurological implications are of increasing interest, necessitating deeper understanding cystinosis neurocognitive...

Background Altered patterns of eye-movements during scene exploration, and atypical gaze preferences in social settings, have long been noted as features the Autism phenotype. While these are typically attributed to differences engagement interests (e.g., for inanimate objects over face stimuli), there also reports differential saccade measures non-social stimuli, raising possibility that fundamental visuo-sensorimotor processing may be at play. Here, we tested plasticity eye-movement system...

22q11.2DS, the most common chromosomal microdeletion disorder, is characterized by greatly increased vulnerability for developmental delays and cognitive deficits, neuropsychiatric symptoms beginning in adolescence/early adulthood, with up to 30% of these individuals developing schizophrenia. Though there have been numerous studies on deletion’s neuroanatomical signatures, its impact brain activity less well understood. In schizophrenia, neural circuits auditory system shown be impaired,...

Abstract Cystinosis, a genetic rare disease characterized by cystine accumulation and crystallization, results in significant damage multitude of tissues organs, such as the kidney, thyroid, eye, brain. While Cystinosis’ impact on brain function is relatively mild compared to its effects other increased lifespan this population thus potential for productive societal contributions have led interest function. Nevertheless, despite some evidence structural differences, neural mutation still not...

Abstract Background 22q11.2 deletion syndrome (22q11.2DS; also known as DiGeorge or velo-cardio-facial syndrome) is characterized by increased vulnerability for neuropsychiatric symptoms, with approximately 30% of the individuals developing schizophrenia. Clinically, deficits in executive function have been noted this population, but underlying neural processes are not well understood. Methods Using high-density electrophysiology (EEG), we investigated dynamics inhibition a prepotent...

Abstract We investigated visual processing in 22q11.2 deletion syndrome (22q11.2DS), a condition characterized by an increased risk for schizophrenia. Visual differences have been described schizophrenia but remain understudied early the disease course. Electrophysiology was recorded during adaptation task with different interstimulus intervals to investigate and 22q11.2DS (with (22q+) without (22q-) psychotic symptoms), compared control idiopathic groups. Analyses focused on windows of...