Visual Evoked Potentials in Rett Syndrome

Jg, Millichap

doi:10.15844/pedneurbriefs-29-10-7

Cited by 4 publications

(2 citation statements)

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“…To date, most ERP studies in Rett have shown highly disordered sensory responses, both in audition (Foxe, Burke et al 2016) (Key, Jones et al 2019) (Saby, Benke et al 2021) (Sysoeva, Molholm et al 2020) (Brima, Molholm et al 2019) (Peters, Katzenstein et al 2017) (Badr, Witt-Engerstrom et al 1987) and vision (Millichap 2015) (LeBlanc, DeGregorio et al 2015) (Stauder, Smeets et al 2006) (Saunders, McCulloch et al 1995) (Kalmanchey 1990) (Bader, Witt-Engerstrom et al 1989), but to our knowledge, all of these previous studies, including those from our own research group, rely on the standard signal-averaging approach. Here, we are interested in determining whether a higher degree of variability, both at the individual participant and at the group level, might be a factor in the reduced and delayed ERP responses typically reported in RTT.…”

Section: Introductionmentioning

confidence: 99%

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome

Brima,

Beker,

Prinsloo

et al. 2024

Preprint

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Background: In the search for objective tools to quantify neural function in Rett Syndrome (RTT), which are crucial in the evaluation of therapeutic efficacy in clinical trials, recordings of sensory-perceptual functioning using event-related potential (ERP) approaches have emerged as potentially powerful tools. Considerable work points to highly anomalous auditory evoked potentials (AEPs) in RTT. However, an assumption of the typical signal-averaging method used to derive these measures is "stationarity" of the underlying responses - i.e. neural responses to each input are highly stereotyped. An alternate possibility is that responses to repeated stimuli are highly variable in RTT. If so, this will significantly impact the validity of assumptions about underlying neural dysfunction, and likely lead to overestimation of underlying neuropathology. To assess this possibility, analyses at the single-trial level assessing signal-to-noise ratios (SNR), inter-trial variability (ITV) and inter-trial phase coherence (ITPC) are necessary. Methods: AEPs were recorded to simple 100Hz tones from 18 RTT and 27 age-matched controls (Ages: 6-22 years). We applied standard AEP averaging, as well as measures of neuronal reliability at the single-trial level (i.e. SNR, ITV, ITPC). To separate signal-carrying components from non-neural noise sources, we also applied a denoising source separation (DSS) algorithm and then repeated the reliability measures. Results: Substantially increased ITV, lower SNRs, and reduced ITPC were observed in auditory responses of RTT participants, supporting a "neural unreliability" account. Application of the DSS technique made it clear that non-neural noise sources contribute to overestimation of the extent of processing deficits in RTT. Post-DSS, ITV measures were substantially reduced, so much so that pre-DSS ITV differences between RTT and TD populations were no longer detected. In the case of SNR and ITPC, DSS substantially improved these estimates in the RTT population, but robust differences between RTT and TD were still fully evident. Conclusions: To accurately represent the degree of neural dysfunction in RTT using the ERP technique, a consideration of response reliability at the single-trial level is highly advised. Non-neural sources of noise lead to overestimation of the degree of pathological processing in RTT, and denoising source separation techniques during signal processing substantially ameliorate this issue.

show abstract

Section: Introductionmentioning

confidence: 99%

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome

Brima,

Beker,

Prinsloo

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

“…To date, most ERP studies in Rett have shown highly disordered sensory responses, both in audition [ 3 , 12 , 20 , 33 , 53 , 57 , 67 ] and vision [ 2 , 31 , 38 , 45 , 60 , 63 ], but to our knowledge, all of these previous studies, including those from our own research group, rely on the standard signal-averaging approach. Here, we are interested in determining whether a higher degree of variability, both at the individual participant and at the group level, might be a factor in the reduced and delayed ERP responses typically reported in RTT.…”

Section: Introductionmentioning

confidence: 99%

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome

Brima,

Beker,

Prinsloo

et al. 2024

J Neurodevelop Disord

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Background In the search for objective tools to quantify neural function in Rett Syndrome (RTT), which are crucial in the evaluation of therapeutic efficacy in clinical trials, recordings of sensory-perceptual functioning using event-related potential (ERP) approaches have emerged as potentially powerful tools. Considerable work points to highly anomalous auditory evoked potentials (AEPs) in RTT. However, an assumption of the typical signal-averaging method used to derive these measures is “stationarity” of the underlying responses – i.e. neural responses to each input are highly stereotyped. An alternate possibility is that responses to repeated stimuli are highly variable in RTT. If so, this will significantly impact the validity of assumptions about underlying neural dysfunction, and likely lead to overestimation of underlying neuropathology. To assess this possibility, analyses at the single-trial level assessing signal-to-noise ratios (SNR), inter-trial variability (ITV) and inter-trial phase coherence (ITPC) are necessary. Methods AEPs were recorded to simple 100 Hz tones from 18 RTT and 27 age-matched controls (Ages: 6–22 years). We applied standard AEP averaging, as well as measures of neuronal reliability at the single-trial level (i.e. SNR, ITV, ITPC). To separate signal-carrying components from non-neural noise sources, we also applied a denoising source separation (DSS) algorithm and then repeated the reliability measures. Results Substantially increased ITV, lower SNRs, and reduced ITPC were observed in auditory responses of RTT participants, supporting a “neural unreliability” account. Application of the DSS technique made it clear that non-neural noise sources contribute to overestimation of the extent of processing deficits in RTT. Post-DSS, ITV measures were substantially reduced, so much so that pre-DSS ITV differences between RTT and TD populations were no longer detected. In the case of SNR and ITPC, DSS substantially improved these estimates in the RTT population, but robust differences between RTT and TD were still fully evident. Conclusions To accurately represent the degree of neural dysfunction in RTT using the ERP technique, a consideration of response reliability at the single-trial level is highly advised. Non-neural sources of noise lead to overestimation of the degree of pathological processing in RTT, and denoising source separation techniques during signal processing substantially ameliorate this issue.

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Sensory evoked potentials in patients with Rett syndrome through the lens of animal studies: Systematic review

Sysoeva

Smirnov

Stroganova

2020

Clinical Neurophysiology

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Visual Evoked Potentials in Rett Syndrome

Abstract: Investigators from the Boston Children's Hospital recorded pattern-reversal visual evoked potentials (VEPs) in Mecp2 heterozygous female mice and in 34 girls with Rett syndrome (RTT).

Cited by 4 publications

References 5 publications

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome

Sensory evoked potentials in patients with Rett syndrome through the lens of animal studies: Systematic review

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