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Open Access 01-12-2024 | Electroencephalography | Research

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)

Authors: Tufikameni Brima, Edward G. Freedman, Kevin D. Prinsloo, Erika F. Augustine, Heather R. Adams, Kuan Hong Wang, Jonathan W. Mink, Luke H. Shaw, Emma P. Mantel, John J. Foxe

Published in: Journal of Neurodevelopmental Disorders | Issue 1/2024

Abstract

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.

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Title: 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)
Authors: Tufikameni Brima
Edward G. Freedman
Kevin D. Prinsloo
Erika F. Augustine
Heather R. Adams
Kuan Hong Wang
Jonathan W. Mink
Luke H. Shaw
Emma P. Mantel
John J. Foxe
Publication date: 01-12-2024
Publisher: BioMed Central
Keyword: Electroencephalography
Published in: Journal of Neurodevelopmental Disorders / Issue 1/2024
Print ISSN: 1866-1947
Electronic ISSN: 1866-1955
DOI: https://doi.org/10.1186/s11689-023-09515-8

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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)

Abstract

Background

Methods

Results

Conclusions

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Abstract

Background

Methods

Results

Conclusions

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