Assessing the integrity of auditory sensory memory processing in CLN3 disease (Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease)): an auditory evoked potential study of the duration-evoked mismatch negativity (MMN) / Tufikameni BRIMA in Journal of Neurodevelopmental Disorders, 16 (2024)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 16 (2024) Titre : Assessing the integrity of auditory sensory memory processing in CLN3 disease (Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease)): an auditory evoked potential study of the duration-evoked mismatch negativity (MMN) Type de document : texte imprimé Auteurs : Tufikameni BRIMA, Auteur ; Edward G. FREEDMAN, Auteur ; Kevin D. PRINSLOO, Auteur ; Erika F. AUGUSTINE, Auteur ; Heather R. ADAMS, Auteur ; Kuan Hong WANG, Auteur ; Jonathan W. MINK, Auteur ; Luke H. SHAW, Auteur ; Emma P. MANTEL, Auteur ; John J. FOXE, Auteur Langues : Anglais (eng) Mots-clés : Humans  Neuronal Ceroid-Lipofuscinoses/complications  Auditory Perception  Evoked Potentials, Auditory  Memory  Brain  Membrane Glycoproteins  Molecular Chaperones  Eeg  Erp  Event-related potential  Jncl  Lysosomal storage disorder  Neurodegenerative disease  Neurodevelopmental disorder  would bias the work reported herein. Index. décimale : PER Périodiques Résumé : BACKGROUND: We interrogated auditory sensory memory capabilities in individuals with CLN3 disease (juvenile neuronal ceroid lipofuscinosis), specifically for the feature of "duration" processing. Given decrements in auditory processing abilities associated with later-stage CLN3 disease, we hypothesized that the duration-evoked mismatch negativity (MMN) of the event related potential (ERP) would be a marker of progressively atypical cortical processing in this population, with potential applicability as a brain-based biomarker in clinical trials. METHODS: We employed three stimulation rates (fast: 450 ms, medium: 900 ms, slow: 1800 ms), allowing for assessment of the sustainability of the auditory sensory memory trace. The robustness of MMN directly relates to the rate at which the regularly occurring stimulus stream is presented. As presentation rate slows, robustness of the sensory memory trace diminishes. By manipulating presentation rate, the strength of the sensory memory trace is parametrically varied, providing greater sensitivity to detect auditory cortical dysfunction. A secondary hypothesis was that duration-evoked MMN abnormalities in CLN3 disease would be more severe at slower presentation rates, resulting from greater demand on the sensory memory system. RESULTS: Data from individuals with CLN3 disease (N = 21; range 6-28 years of age) showed robust MMN responses (i.e., intact auditory sensory memory processes) at the medium stimulation rate. However, at the fastest rate, MMN was significantly reduced, and at the slowest rate, MMN was not detectable in CLN3 disease relative to neurotypical controls (N = 41; ages 6-26 years). CONCLUSIONS: Results reveal emerging insufficiencies in this critical auditory perceptual system in individuals with CLN3 disease. En ligne : https://dx.doi.org/10.1186/s11689-023-09515-8 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575 [article] Assessing the integrity of auditory sensory memory processing in CLN3 disease (Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease)): an auditory evoked potential study of the duration-evoked mismatch negativity (MMN) [texte imprimé] / Tufikameni BRIMA, Auteur ; Edward G. FREEDMAN, Auteur ; Kevin D. PRINSLOO, Auteur ; Erika F. AUGUSTINE, Auteur ; Heather R. ADAMS, Auteur ; Kuan Hong WANG, Auteur ; Jonathan W. MINK, Auteur ; Luke H. SHAW, Auteur ; Emma P. MANTEL, Auteur ; John J. FOXE, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 16 (2024) Mots-clés : Humans  Neuronal Ceroid-Lipofuscinoses/complications  Auditory Perception  Evoked Potentials, Auditory  Memory  Brain  Membrane Glycoproteins  Molecular Chaperones  Eeg  Erp  Event-related potential  Jncl  Lysosomal storage disorder  Neurodegenerative disease  Neurodevelopmental disorder  would bias the work reported herein. Index. décimale : PER Périodiques Résumé : BACKGROUND: We interrogated auditory sensory memory capabilities in individuals with CLN3 disease (juvenile neuronal ceroid lipofuscinosis), specifically for the feature of "duration" processing. Given decrements in auditory processing abilities associated with later-stage CLN3 disease, we hypothesized that the duration-evoked mismatch negativity (MMN) of the event related potential (ERP) would be a marker of progressively atypical cortical processing in this population, with potential applicability as a brain-based biomarker in clinical trials. METHODS: We employed three stimulation rates (fast: 450 ms, medium: 900 ms, slow: 1800 ms), allowing for assessment of the sustainability of the auditory sensory memory trace. The robustness of MMN directly relates to the rate at which the regularly occurring stimulus stream is presented. As presentation rate slows, robustness of the sensory memory trace diminishes. By manipulating presentation rate, the strength of the sensory memory trace is parametrically varied, providing greater sensitivity to detect auditory cortical dysfunction. A secondary hypothesis was that duration-evoked MMN abnormalities in CLN3 disease would be more severe at slower presentation rates, resulting from greater demand on the sensory memory system. RESULTS: Data from individuals with CLN3 disease (N = 21; range 6-28 years of age) showed robust MMN responses (i.e., intact auditory sensory memory processes) at the medium stimulation rate. However, at the fastest rate, MMN was significantly reduced, and at the slowest rate, MMN was not detectable in CLN3 disease relative to neurotypical controls (N = 41; ages 6-26 years). CONCLUSIONS: Results reveal emerging insufficiencies in this critical auditory perceptual system in individuals with CLN3 disease. En ligne : https://dx.doi.org/10.1186/s11689-023-09515-8 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575

Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome / Tufikameni BRIMA in Journal of Neurodevelopmental Disorders, 16 (2024)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 16 (2024) Titre : Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome Type de document : texte imprimé Auteurs : Tufikameni BRIMA, Auteur ; Shlomit BEKER, Auteur ; Kevin D. PRINSLOO, Auteur ; John S. BUTLER, Auteur ; Aleksandra DJUKIC, Auteur ; Edward G. FREEDMAN, Auteur ; Sophie MOLHOLM, Auteur ; John J. FOXE, Auteur Langues : Anglais (eng) Mots-clés : Humans  Rett Syndrome/physiopathology/complications  Adolescent  Female  Evoked Potentials, Auditory/physiology  Child  Young Adult  Electroencephalography  Auditory Perception/physiology  Reproducibility of Results  Acoustic Stimulation  Male  Signal-To-Noise Ratio  Adult  Auditory Evoked Potential  AEP  Auditory discrimination  Denoising Source Separation (DSS)  Eeg  Event-related potential  ERP  Females  High-density electrical mapping  Inter-Trial Phase Coherence (ITPC)  Inter-trial variability (ITV)  Neurodevelopmental disorder  Rett Syndrome Severity Scale (RSSS)  Signal-noise ratio (SNR)  X-linked mutation  MECP2  to the results of this study. Index. décimale : PER Périodiques Résumé : 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. En ligne : https://dx.doi.org/10.1186/s11689-024-09544-x Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575 [article] Probing a neural unreliability account of auditory sensory processing atypicalities in Rett Syndrome [texte imprimé] / Tufikameni BRIMA, Auteur ; Shlomit BEKER, Auteur ; Kevin D. PRINSLOO, Auteur ; John S. BUTLER, Auteur ; Aleksandra DJUKIC, Auteur ; Edward G. FREEDMAN, Auteur ; Sophie MOLHOLM, Auteur ; John J. FOXE, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 16 (2024) Mots-clés : Humans  Rett Syndrome/physiopathology/complications  Adolescent  Female  Evoked Potentials, Auditory/physiology  Child  Young Adult  Electroencephalography  Auditory Perception/physiology  Reproducibility of Results  Acoustic Stimulation  Male  Signal-To-Noise Ratio  Adult  Auditory Evoked Potential  AEP  Auditory discrimination  Denoising Source Separation (DSS)  Eeg  Event-related potential  ERP  Females  High-density electrical mapping  Inter-Trial Phase Coherence (ITPC)  Inter-trial variability (ITV)  Neurodevelopmental disorder  Rett Syndrome Severity Scale (RSSS)  Signal-noise ratio (SNR)  X-linked mutation  MECP2  to the results of this study. Index. décimale : PER Périodiques Résumé : 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. En ligne : https://dx.doi.org/10.1186/s11689-024-09544-x Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575

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