Auditory mismatch negativity in pre-manifest and manifest Huntington's disease

Marianna Delussi; Christian Valt; Adelchi Silvestri; Katia Ricci; Emanuella Ladisa; Elena Ammendola; Antonio Rampino; Giulio Pergola; Marina de Tommaso

doi:10.1016/j.clinph.2024.03.020

Auditory mismatch negativity in pre-manifest and manifest Huntington's disease

Clin Neurophysiol. 2024 Mar 26:162:121-128. doi: 10.1016/j.clinph.2024.03.020. Online ahead of print.

Authors

Marianna Delussi¹, Christian Valt², Adelchi Silvestri², Katia Ricci³, Emanuella Ladisa², Elena Ammendola³, Antonio Rampino², Giulio Pergola⁴, Marina de Tommaso⁵

Affiliations

¹ Department of Education, Psychology and Communication, University of Bari Aldo Moro, Bari, Italy.
² Department of Translational Biomedicine and Neuroscience, University of Bari Aldo Moro, Bari, Italy.
³ Neurophysiopathology Unit, Policlinico General Hospital, Bari, Italy.
⁴ Department of Translational Biomedicine and Neuroscience, University of Bari Aldo Moro, Bari, Italy; Lieber Institute for Brain Development, Johns Hopkins Medical Campus, Baltimore, MD, United States; Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, MD, United States.
⁵ Department of Translational Biomedicine and Neuroscience, University of Bari Aldo Moro, Bari, Italy. Electronic address: marina.detommaso@uniba.it.

PMID: 38603947
DOI: 10.1016/j.clinph.2024.03.020

Abstract

Aim: The aim of this study was to investigate the characteristics of the electrophysiological brain response elicited in a passive acoustic oddball paradigm, i.e. mismatch negativity (MMN), in patients with Huntington's disease (HD) in the premanifest (pHD) and manifest (mHD) phases. In this regard, we correlated the results of event-related potentials (ERP) with disease characteristics.

Methods: This was an observational cross-sectional MMN study. In addition to the MMN recording of the passive oddball task, all subjects with first-degree inheritance for HD underwent genetic testing for mutant HTT, the Huntington's Disease Rating Scale, the Total Functional Capacity Scale, the Problem Behaviors Assessment short form, and the Mini-Mental State Examination.

Results: We found that global field power (GFP) was reduced in the MMN time window in mHD patients compared to pHD and normal controls (NC). In the pHD group, MMN amplitude was only slightly and not significantly increased compared to mHD, while pHD patients showed increased theta coherence between trials compared to mHD. In the entire sample of HD gene carriers, the main MMN traits were not correlated with motor performance, cognitive impairment and functional disability.

Conclusion: These results suggest an initial and subtle deterioration of pre-attentive mechanisms in the presymptomatic phase of HD, with an increasing phase shift in the MMN time frame. This result could indicate initial functional changes with a possible compensatory effect.

Significance: An initial and slight decrease in MMN associated with increased phase coherence in the corresponding EEG frequencies could indicate an early functional involvement of pre-attentive resources that could precede the clinical expression of HD.

Keywords: Auditory mismatch negativity; Cognitive decline; Huntington’s Disease; Motor impairment; Presymptomatic phase; Sensory processing.