scholarly journals Electrophysiological evidence for impaired auditory sensory memory in Cystinosis despite typical sensory processing: An MMN investigation

2019 ◽  
Author(s):  
Ana A. Francisco ◽  
John J. Foxe ◽  
Douwe J. Horsthuis ◽  
Sophie Molholm

AbstractCystinosis, a genetic rare disease characterized by cystine accumulation and crystallization, results in significant damage in a multitude of tissues and organs, such as the kidney, thyroid, eye, and brain. While Cystinosis’ impact on brain function is relatively mild compared to its effects on other organs, the increased lifespan of this population and thus potential for productive societal contributions have led to increased interest on the effects on brain function. Nevertheless, and despite some evidence of structural brain differences, the neural impact of the mutation is still not well characterized.Here, using a passive duration oddball paradigm (with different stimulus onset asynchronies (SOAs), representing different levels of demand on memory) and high-density electrophysiology, we tested basic auditory processing in a group of 22 children and adolescents diagnosed with Cystinosis (age range: 6-17 years old) and in neurotypical age-matched controls (N=24). We examined whether the N1 and mismatch negativity (MMN) significantly differed between the groups and if those neural measures correlated with verbal and non-verbal IQ. Individuals diagnosed with Cystinosis presented similar N1 responses to their age-matched peers, indicating typical basic auditory processing in this population. However, whereas both groups showed similar MMN responses for the shortest (450ms) SOA, suggesting intact change detection and sensory memory, individuals diagnosed with Cystinosis presented clearly reduced responses for the longer (900ms and 1800ms) SOAs. This could indicate reduced duration auditory sensory memory traces, and thus sensory memory impairment, in children and adolescents diagnosed with Cystinosis. Future work addressing other aspects of sensory and working memory is needed to understand the underlying bases of the differences described here, and their implication for higher order processing.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Ana A. Francisco ◽  
Alaina S. Berruti ◽  
Frederick J. Kaskel ◽  
John J. Foxe ◽  
Sophie Molholm

Abstract Background Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues and organs, including the kidneys and brain. Its impact on neural function appears mild relative to its effects on other organs, but therapeutic advances have led to substantially increased life expectancy, necessitating deeper understanding of its impact on neurocognitive function in adulthood. We previously demonstrated intact auditory sensory processing, accompanied by mild sensory memory difficulties, in children and adolescents with cystinosis. Methods We investigated whether further progressive decrements in these processes would be observed in adults with cystinosis, comparing high-density auditory-evoked potential (AEP) recordings from adults with cystinosis (N = 15; ages: 19–38 years) to those of age-matched controls (N = 17). We employed a duration oddball paradigm with different stimulation rates, in which participants passively listened to regularly occurring standard tones interspersed with infrequently occurring deviant tones. Analyses focused on AEP components reflecting auditory sensory-perceptual processing (N1 and P2), sensory memory (mismatch negativity, MMN), and attentional orienting (P3a). Results Overall, adults with cystinosis produced highly similar sensory-perceptual AEP responses to those observed in controls suggesting intact early auditory cortical processing. However, significantly increased P2 and P3a amplitudes and reduced MMN at slower stimulation rates were observed, suggesting mild-to-moderate changes in auditory sensory memory and attentional processing. While cognitive testing revealed lower scores on verbal IQ and perceptual reasoning in cystinosis, these did not correlate with the AEP measures. Conclusions These neurophysiological data point to the emergence of subtle auditory processing deficits in early adulthood in cystinosis, warranting further investigation of memory and attentional processes in this population, and of their consequences for perceptual and cognitive function.



2021 ◽  
Author(s):  
Ana A. Francisco ◽  
Alaina S. Berruti ◽  
Frederick J. Kaskel ◽  
John J. Foxe ◽  
Sophie Molholm

Abstract Background Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues and organs, including the kidneys and brain. Its impact on neural function appears mild relative to its effects on other organs, but therapeutic advances have led to substantially increased life expectancy, necessitating deeper understanding of its impact on neurocognitive function in adulthood. We previously demonstrated intact auditory sensory processing, accompanied by mild sensory memory difficulties, in children and adolescents with cystinosis. Methods We investigated whether further progressive decrements in these processes would be observed in adults with cystinosis, comparing high-density auditory-evoked potential (AEP) recordings from adults with cystinosis (N = 15; ages: 19–38 years) to those of age-matched controls (N = 17). We employed a duration oddball paradigm with different stimulation rates, in which participants passively listened to regularly occurring standard tones interspersed with infrequently occurring deviant tones. Analyses focused on AEP components reflecting auditory sensory-perceptual processing (N1 and P2), sensory memory (mismatch negativity, MMN), and attentional orienting (P3a). Results Overall, adults with cystinosis produced highly similar sensory-perceptual AEP responses to those observed in controls suggesting intact early auditory cortical processing. However, significantly increased P2 and P3a amplitudes and reduced MMN at slower stimulation rates were observed, suggesting mild-to-moderate changes in auditory sensory memory and attentional processing. While cognitive testing revealed lower scores on verbal IQ and perceptual reasoning in cystinosis, these did not correlate with the AEP measures. Conclusions These neurophysiological data point to the emergence of subtle auditory processing deficits in early adulthood in cystinosis, warranting further investigation of memory and attentional processes in this population, and of their consequences for perceptual and cognitive function.



2020 ◽  
Vol 14 ◽  
Author(s):  
Xin He ◽  
Jian Zhang ◽  
Zhilin Zhang ◽  
Ritsu Go ◽  
Jinglong Wu ◽  
...  

Auditory sensory memory indexed by mismatch negativity has been broadly studied over the past century, but far less attention has been directed to tactile sensory memory. To investigate whether tactile sensory memory is affected by attention, we recorded somatosensory mismatch negativity (sMMN) from 24 healthy adults in two experiments to distinguish sustained attention from non-sustained attention. Using the roving somatosensory oddball paradigm, we analyzed the average dynamic changes in the amplitude and latency of sMMN amplitude and found a clear sMMN component at the central region at a 100–300 ms interval. The sMMN amplitude, which indexes the early detection of tactile stimuli with the sensory memory trace, was larger in the tactile attentional task. Additionally, the sMMN latency increased with the increasing visual attentional load, which indicates a decay of tactile sensory memory. Our results indicate that the more attention resources are allocated for a tactile sensation, the more favorable it is to the generation of tactile sensory memory.



2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Liliana Ramona Demenescu ◽  
Yutaka Kato ◽  
Klaus Mathiak

Emotion recognition deficits emerge with the increasing age, in particular, a decline in the identification of sadness. However, little is known about the age-related changes of emotion processing in sensory, affective, and executive brain areas. This functional magnetic resonance imaging (fMRI) study investigated neural correlates of auditory processing of prosody across adult lifespan. Unattended detection of emotional prosody changes was assessed in 21 young (age range: 18–35 years), 19 middle-aged (age range: 36–55 years), and 15 older (age range: 56–75 years) adults. Pseudowords uttered with neutral prosody were standards in an oddball paradigm with angry, sad, happy, and gender deviants (total 20% deviants). Changes in emotional prosody and voice gender elicited bilateral superior temporal gyri (STG) responses reflecting automatic encoding of prosody. At the right STG, responses to sad deviants decreased linearly with age, whereas happy events exhibited a nonlinear relationship. In contrast to behavioral data, no age by sex interaction emerged on the neural networks. The aging decline of emotion processing of prosodic cues emerges already at an early automatic stage of information processing at the level of the auditory cortex. However, top-down modulation may lead to an additional perceptional bias, for example, towards positive stimuli, and may depend on context factors such as the listener’s sex.



1998 ◽  
Vol 793 (1-2) ◽  
pp. 95-102 ◽  
Author(s):  
Elyse Sussman ◽  
Hilary Gomes ◽  
Jo Manette K Nousak ◽  
Walter Ritter ◽  
Herbert G Vaughan


Autism ◽  
2021 ◽  
pp. 136236132098795
Author(s):  
Eleanor R Palser ◽  
Alejandro Galvez-Pol ◽  
Clare E Palmer ◽  
Ricci Hannah ◽  
Aikaterini Fotopoulou ◽  
...  

Differences in understanding emotion in autism are well-documented, although far more research has considered how being autistic impacts an understanding of other people’s emotions, compared to their own. In neurotypical adults and children, many emotions are associated with distinct bodily maps of experienced sensation, and the ability to report these maps is significantly related to the awareness of interoceptive signals. Here, in 100 children who either carry a clinical diagnosis of autism ( n = 45) or who have no history of autism ( n = 55), we investigated potential differences in differentiation across autistic children’s bodily maps of emotion, as well as how such differentiation relates to the processing of interoceptive signals. As such, we measured objective interoceptive performance using the heartbeat-counting task, and participants’ subjective experience of interoceptive signals using the child version of the Body Perception Questionnaire. We found less differentiation in the bodily maps of emotion in autistic children, but no association with either objective or subjective interoceptive processing. These findings suggest that, in addition to previously reported differences in detecting others’ emotional states, autistic children have a less differentiated bodily experience of emotion. This does not, however, relate to differences in interoceptive perception as measured here. Lay abstract More research has been conducted on how autistic people understand and interpret other people’s emotions, than on how autistic people experience their own emotions. The experience of emotion is important however, because it can relate to difficulties like anxiety and depression, which are common in autism. In neurotypical adults and children, different emotions have been associated with unique maps of activity patterns in the body. Whether these maps of emotion are comparable in autism is currently unknown. Here, we asked 100 children and adolescents, 45 of whom were autistic, to color in outlines of the body to indicate how they experienced seven emotions. Autistic adults and children sometimes report differences in how they experience their internal bodily states, termed interoception, and so we also investigated how this related to the bodily maps of emotion. In this study, the autistic children and adolescents had comparable interoception to the non-autistic children and adolescents, but there was less variability in their maps of emotion. In other words, they showed more similar patterns of activity across the different emotions. This was not related to interoception, however. This work suggests that there are differences in how autistic people experience emotion that are not explained by differences in interoception. In neurotypical people, less variability in emotional experiences is linked to anxiety and depression, and future work should seek to understand if this is a contributing factor to the increased prevalence of these difficulties in autism.



2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Janaina R. Niehues ◽  
Ana Inês Gonzales ◽  
Robson R. Lemos ◽  
Poliana Penasso Bezerra ◽  
Patrícia Haas

Introduction. Infant juvenile obesity is currently a worldwide public health problem and it is increasing at alarming rate in the Brazilian population, showing its relevance in terms of public health.Objectives. Determine the prevalence of overweight and obesity in children and adolescents between 2 and 19 years old in different regions of Brazil.Methods. The following electronic databases were searched (from September through November 2013): Medline (PubMed), LILACS, and SciELO, using the descriptors and Boolean operators (obesity) and (overweight) and (child) and (prevalence) and (Brazil). Prospective and/or cross-sectional designs studies were found regarding the prevalence of overweight and obese children and adolescents in the five regions of Brazil.Results. A total of 191 scientific articles were found of which 17 met all inclusion criteria. A higher prevalence of overweight was found in the south (25.7%) and north (28.8%) of the country, and obesity in the southeast (15.4%) and south (10.4%).Conclusions. The scope of the studies was mostly based on municipal coverage, which resulted in limitations for conclusive analysis, showing the need for further studies of prevalence at the national level, with emphasis on public health in obese children and adolescents throughout the Brazilian territory.



2017 ◽  
Vol 38 (2) ◽  
pp. 262-265 ◽  
Author(s):  
Doug J. K. Barrett ◽  
Michael Pilling


2003 ◽  
Vol 33 (2) ◽  
pp. 289-297 ◽  
Author(s):  
L. RANGEL ◽  
M. E. GARRALDA ◽  
A. HALL ◽  
S. WOODHAM

Background. High rates of psychopathology and of personality problems have been reported in children and adolescents with chronic fatigue syndrome (CFS). It is not clear whether this is consequent on the experience of chronic physical ill health. We compare psychiatric adjustment in children with CFS and in children suffering from another chronic physical disorder (juvenile idiopathic arthritis or JIA).Method. Our sample consisted of 28 children with CFS and 30 with JIA attending tertiary paediatric centres (age range, 11 to 18 years, mean 15, S.D. 2·3). In order to assess psychiatric status and functioning, we used the K-SADS psychiatric interviews, CGAS and Harter Self-Esteem Questionnaire with child subjects; behavioural questionnaires (CBCL) and child personality assessment interviews (PAS) with parent informants.Results. Psychiatric disorders in the year prior to interview had been present significantly more commonly in the CFS group (72% v. 34% in JIA) and were more impairing to them (CGAS scores of 45 v. 77). Most common diagnoses in both groups were depressive and anxiety disorders. Personality problems were also significantly more frequent in CFS subjects (48% disorder and 26% difficulty v. 11% and 11% in JIA). There were few differences between the two groups in self-esteem.Conclusions. Psychopathology and personality problems are common in children and adolescents with severe forms of CFS and cannot be explained strictly through the experience of chronic physical illness.



2021 ◽  
Vol 6 (1) ◽  
pp. 9
Author(s):  
Ewan Thomas ◽  
Marianna Alesi ◽  
Garden Tabacchi ◽  
Carlos Marques da Silva ◽  
David J. Sturm ◽  
...  

The aim of this investigation was to identify possible related factors associated to the performance of the crunning test in European children and adolescents. A total number of 559 children and adolescents (age range 6–14 years) of which 308 boys (55.1%) and 251 girls (44.9%), from seven European countries, were screened. A questionnaire concerning demographic and personal life-related factors and a cognitive assessment were performed. A regression analysis was conducted with the performance measures of the crunning movement. T-tests and ANCOVA were used to analyze sub-group differences. Boys have greater crunning performance values compared to girls (5.55 s vs. 7.06 s, p < 0.001) and older children perform better than younger ones (R2 −0.23; p < 0.001). Children with healthy and active habits (exercising or spending time with family members vs. reading or surfing the internet) performed better in the test. Children engaged in team sports had better crunning performances compared to those engaged in individual sports (6.01 s vs. 6.66 s, p = 0.0166). No significant association was found regarding cognitive-related aspects in either children engaged in team or individual sports and the crunning performance. Older and male children performed better in the crunning test than younger and female children. Physical activity-related aspects of children’s life are associated with crunning movement performance. No association was found between higher cognitive performance and the crunning test results.



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