Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain

Deficits in several cognitive domains are prevalent in men with Klinefelter Syndrome (KS). Verbal deficits are among the most characteristic cognitive impairments of KS, yet other cognitive domains also exhibit deficits. Executive functions, especially working memory capacity and inhibitory control,...

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Main Authors: René J. Huster, Christina Thunberg, Anne-Kristin Solbakk, Claus H. Gravholt, Krister Fjermestad
Format: Article
Language:English
Published: Elsevier 2024-01-01
Series:NeuroImage: Clinical
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Online Access:http://www.sciencedirect.com/science/article/pii/S2213158224001153
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author René J. Huster
Christina Thunberg
Anne-Kristin Solbakk
Claus H. Gravholt
Krister Fjermestad
author_facet René J. Huster
Christina Thunberg
Anne-Kristin Solbakk
Claus H. Gravholt
Krister Fjermestad
author_sort René J. Huster
collection DOAJ
description Deficits in several cognitive domains are prevalent in men with Klinefelter Syndrome (KS). Verbal deficits are among the most characteristic cognitive impairments of KS, yet other cognitive domains also exhibit deficits. Executive functions, especially working memory capacity and inhibitory control, are frequently affected as well. A common limitation of previous studies addressing potential deficits in inhibitory control is their potential conflation with language-related capabilities, as for example is the case with the Stroop task. Importantly, none of the prior studies utilized the best-accepted approach to study inhibition, namely the Stop Signal Task (SST). This study therefore tested for deficits in inhibitory control in individuals with KS and controls (HC) using a version of the SST with non-semantic auditory stimuli. In addition to the classic behavioral performance measures (e.g., the go reaction time, stopping accuracy, or stop signal reaction time), we also derived parameters of inhibition and attention from electromyography (EMG), electroencephalography (EEG), and Bayesian modeling. Men with KS exhibited prolonged stopping latencies (i.e., stop signal reaction times) and reduced stopping accuracies. Yet, whereas these model-based indices were indicative of attenuated inhibitory control, neither event-related brain potentials nor an EMG-measure of the stopping latency confirmed such deficits. Behavioral and EEG indices, however, provided evidence for deficits in motor response preparation and generation, as well as the early processing of auditory stimuli. In sum, the overall pattern of results does not support the existence of inhibitory deficits in KS per se, but rather suggests that behavioral indices of impaired inhibition may result from early low-level deficits in the auditory and motor domains, as well as a differential weighting in the processing of different aspects of the task.
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spelling doaj-art-25761494509146dba1d2899bc92588212025-08-20T02:49:00ZengElsevierNeuroImage: Clinical2213-15822024-01-014410367410.1016/j.nicl.2024.103674Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domainRené J. Huster0Christina Thunberg1Anne-Kristin Solbakk2Claus H. Gravholt3Krister Fjermestad4Multimodal Imaging and Cognitive Control Lab, Department of Psychology, University of Oslo, Oslo, Norway; Cognitive and Translational Neuroscience Cluster, Department of Psychology, University of Oslo, Oslo, Norway; Corresponding author at: Multimodal Imaging and Cognitive Control Lab, Department of Psychology, University of Oslo, Oslo, Norway.Multimodal Imaging and Cognitive Control Lab, Department of Psychology, University of Oslo, Oslo, NorwayCognitive and Translational Neuroscience Cluster, Department of Psychology, University of Oslo, Oslo, Norway; RITMO Centre for Interdisciplinary Studies in Rhythm, Time and Motion, Norway; Department of Psychology, University of Oslo, Norway; Department of Neuropsychology, Helgeland Hospital, Mosjøen, Norway; Department of Neurosurgery, Oslo University Hospital, NorwayDepartment of Molecular Medicine, Aarhus University Hospital, Denmark; Department of Clinical Medicine, Aarhus University, Denmark; Department of Endocrinology, Aarhus University Hospital, DenmarkSection for Clinical Psychology, Department of Psychology, University of Oslo, Oslo, Norway; Frambu Resource Centre for Rare Disorders, Siggerud, NorwayDeficits in several cognitive domains are prevalent in men with Klinefelter Syndrome (KS). Verbal deficits are among the most characteristic cognitive impairments of KS, yet other cognitive domains also exhibit deficits. Executive functions, especially working memory capacity and inhibitory control, are frequently affected as well. A common limitation of previous studies addressing potential deficits in inhibitory control is their potential conflation with language-related capabilities, as for example is the case with the Stroop task. Importantly, none of the prior studies utilized the best-accepted approach to study inhibition, namely the Stop Signal Task (SST). This study therefore tested for deficits in inhibitory control in individuals with KS and controls (HC) using a version of the SST with non-semantic auditory stimuli. In addition to the classic behavioral performance measures (e.g., the go reaction time, stopping accuracy, or stop signal reaction time), we also derived parameters of inhibition and attention from electromyography (EMG), electroencephalography (EEG), and Bayesian modeling. Men with KS exhibited prolonged stopping latencies (i.e., stop signal reaction times) and reduced stopping accuracies. Yet, whereas these model-based indices were indicative of attenuated inhibitory control, neither event-related brain potentials nor an EMG-measure of the stopping latency confirmed such deficits. Behavioral and EEG indices, however, provided evidence for deficits in motor response preparation and generation, as well as the early processing of auditory stimuli. In sum, the overall pattern of results does not support the existence of inhibitory deficits in KS per se, but rather suggests that behavioral indices of impaired inhibition may result from early low-level deficits in the auditory and motor domains, as well as a differential weighting in the processing of different aspects of the task.http://www.sciencedirect.com/science/article/pii/S2213158224001153KlinefelterXXYResponse InhibitionattentionCognitionEEG
spellingShingle René J. Huster
Christina Thunberg
Anne-Kristin Solbakk
Claus H. Gravholt
Krister Fjermestad
Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain
NeuroImage: Clinical
Klinefelter
XXY
Response Inhibition
attention
Cognition
EEG
title Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain
title_full Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain
title_fullStr Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain
title_full_unstemmed Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain
title_short Inhibitory deficits in Klinefelter syndrome are secondary to deficits in the auditory and motor domain
title_sort inhibitory deficits in klinefelter syndrome are secondary to deficits in the auditory and motor domain
topic Klinefelter
XXY
Response Inhibition
attention
Cognition
EEG
url http://www.sciencedirect.com/science/article/pii/S2213158224001153
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