Distinguishing response to names in Rett and MECP2 Duplication syndrome: An ERP study of auditory social information processing

Peters, Sarika U.; Katzenstein, Ashley; Jones, Dorita; Key, Alexandra P.

doi:10.1016/j.brainres.2017.08.028

Cited by 25 publications

(44 citation statements)

References 41 publications

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“…; Peters et al . ) and confirmed by visual inspection of the grand‐averaged waveforms. To account for potential variability in scalp topography previously observed in some individuals with developmental disabilities (Peters et al .…”

Section: Methodsmentioning

confidence: 54%

“…) and in those with developmental disabilities (Peters et al . ), the social–emotional processing assessed by the ERPs was unrelated to cognitive abilities. This pattern of correlations between the ERP markers of name differentiation and the behavioural characteristics in typical and atypical populations further supports the idea that ERPs can be a valid direct measure of social–emotional processes previously assessed primarily using indirect, informant‐based tools.…”

Section: Introductionmentioning

confidence: 91%

“…; Peters et al . ), we hypothesised that participants with AS whose caregivers report higher levels of social and communicative functioning will demonstrate greater parietal amplitudes in response to known than unknown names within 450–750 ms.…”

Section: Introductionmentioning

confidence: 99%

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Social–emotional processing in nonverbal individuals with Angelman syndrome: evidence from brain responses to known and novel names

Key

Jones

2018

J intellect Disabil Res

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Background The combination of intellectual, communicative and motor deficits limits the use of standardised behavioural assessments in individuals with Angelman syndrome (AS). The current study aimed to objectively evaluate the extent of social–emotional processing in AS using auditory event‐related potentials (ERPs) during passive exposure to spoken stimuli. Methods Auditory ERP responses were recorded in 13 nonverbal individuals with the deletion subtype of AS, age 4–45 years, during the name recognition paradigm, in which their own names and names of close others (relative or friend) were presented among novel names. No behavioural responses were required. Results Contrary to findings in typical children and adults, there was no significant evidence of differential neural response to known vs. novel names in participants with AS. Nevertheless, greater amplitude differences between known and unknown names demonstrated the predicted association with better interpersonal relationships and receptive communication abilities. Conclusions These findings indicate good tolerability of ERP procedures (85% success rate). The lack of own name differentiation is consistent with increased incidence of the autism‐related symptoms in AS. Strong associations between the caregiver reports of adaptive functioning and neural indices of known name recognition support the utility of brain‐based measures for objectively evaluating cognitive and affective processes in nonverbal persons with neurodevelopmental disorders.

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Section: Methodsmentioning

confidence: 54%

Section: Introductionmentioning

confidence: 91%

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Social–emotional processing in nonverbal individuals with Angelman syndrome: evidence from brain responses to known and novel names

Key

Jones

2018

J intellect Disabil Res

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“…Additionally, our participants represented a wide age range, from young children to adults. Previously published studies suggest that passive measures of incidental memory may be age-independent in children and young adults (Key & Corbett, 2014; Peters et al, 2017), however, spontaneous stimulus repetition detection is sensitive to aging-related cognitive decline in older participants (Key & Dykens, 2014). Little is known about the developmental trajectory over the lifespan in AS, and future studies in this population will need to examine possible age-related differences in auditory learning and memory.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, we successfully used auditory ERPs in response to known or familiarized through repetition stimuli compared to novel distracters to document learning and memory in nonverbal individuals with other neurogenetic disorders, such as MECP2 duplication and Rett syndromes (Peters et al, 2015, 2017, 2018), the latter sharing several phenotypic features with AS. In particular, our auditory incidental memory paradigm allows to evaluate the extent of participant’s spontaneous engagement with auditory information.…”

Section: Introductionmentioning

confidence: 99%

Feasibility of using auditory event-related potentials to investigate learning and memory in nonverbal individuals with Angelman syndrome

Key

Jones

Peters

et al. 2018

Brain and Cognition

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The combination of intellectual, communicative, and motor deficits limit the use of standardized behavioral assessments of cognition in individuals with Angelman syndrome (AS). The current study is the first to objectively evaluate learning and memory in AS using auditory event-related potentials (ERP) during passive exposure to spoken stimuli. Fifteen nonverbal individuals with the deletion subtype of AS (age 4–45 years) completed the auditory incidental memory paradigm. Auditory ERPs were recorded in response to a sequence of unfamiliar nonwords, in which one randomly selected stimulus was repeated multiple times and the rest were presented once. Larger parietal responses within 200–500ms for the repeated nonword compared to novel distracters were associated with caregiver reports of more adaptive communication skills. These findings demonstrate good tolerability of ERP procedures (94% success rate) and indicate that persons with AS can acquire new information following repeated auditory exposure, even in the absence of explicit memorization instructions. Strong associations between the caregiver reports of adaptive functioning and neural indices of auditory learning and memory support the utility of brain-based measures for objectively evaluating higher-order information processing in nonverbal persons with neurodevelopmental disorders.

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Comparison of Core Features in Four Developmental Encephalopathies in the Rett Natural History Study

Cutri‐French

Armstrong

Saby

et al. 2020

Annals of Neurology

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Objective Rett syndrome, CDKL5‐deficiency disorder, FOXG1 disorder, and MECP2 duplication disorder are developmental encephalopathies with shared and distinct features. Although they are historically linked, no direct comparison has been performed. The first head‐to‐head comparison of clinical features in these conditions is presented. Methods Comprehensive clinical information was collected from 793 individuals enrolled in the Rett and Rett‐Related Disorders Natural History Study. Clinical features including clinical severity, regression, and seizures were cross‐sectionally compared between diagnoses to test the hypothesis that these are 4 distinct disorders. Results Distinct patterns of clinical severity, seizure onset age, and regression were present. Individuals with CDKL5‐deficency disorder were the most severely affected and had the youngest age at seizure onset (2 months), whereas children with MECP2 duplication syndrome had the oldest median age at seizure onset (64 months) and lowest severity scores. Rett syndrome and FOGX1 were intermediate in both features. Smaller head circumference correlates with increased severity in all disorders and earlier age at seizure onset in MECP2 duplication syndrome. Developmental regression occurred in all Rett syndrome participants (median = 18 months) but only 23 to 34% of the other disorders. Seizure incidence prior to the baseline visit was highest for CDKL5 deficiency disorder (96.2%) and lowest for Rett syndrome (47.5%). Other clinical features including seizure types and frequency differed among groups. Interpretation Although these developmental encephalopathies share many clinical features, clear differences in severity, regression, and seizures warrant considering them as unique disorders. These results will aid in the development of disease‐specific severity scales, precise therapeutics, and future clinical trials. ANN NEUROL 2020;88:396–406.

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Distinguishing response to names in Rett and MECP2 Duplication syndrome: An ERP study of auditory social information processing

Cited by 25 publications

References 41 publications

Social–emotional processing in nonverbal individuals with Angelman syndrome: evidence from brain responses to known and novel names

Social–emotional processing in nonverbal individuals with Angelman syndrome: evidence from brain responses to known and novel names

Feasibility of using auditory event-related potentials to investigate learning and memory in nonverbal individuals with Angelman syndrome

Comparison of Core Features in Four Developmental Encephalopathies in the Rett Natural History Study

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