Abstract:This study aimed at comparing implicit sequence learning in individuals affected by Duchenne Muscular Dystrophy without intellectual disability and age-matched typically developing children. A modified version of the Serial Reaction Time task was administered to 32 Duchenne children and 37 controls of comparable chronological age. The Duchenne group showed a reduced rate of implicit learning even if in the absence of global intellectual disability. This finding provides further evidence of the involvement of s… Show more
“…Saturation in the learning performance also indicates poor availability of neural resources to support higher level cognitive processing consistent with the neural integrity as suggested by Dixon et al 37 . The distal mutation location in DMD has previously been associated to severe intellectual deficits 5,19,35,38 . www.nature.com/scientificreports/ Poor performance in recalling list B indicates susceptibility to interference from previously acquired memory.…”
Duchenne Muscular Dystrophy has emerged as a model to assess cognitive domains. the DMD gene variant location and its association with variable degrees of cognitive impairment necessitate identification of a common denominator. Computer architectures provide a framework to delineate the mechanisms involved in the cognitive functioning of the human brain. copy number variations in the 79 exons of DMD gene were screened in 84 DMD subjects by Multiplex Ligation-dependent Probe Amplification (MLPA). DMD subjects were categorized based on the presence or absence of DP140 isoform. the cognitive and neuropsychological assessments were carried out as per inclusion criteria using standard scales. Instance-based learning theory (IBLT) based on the partial matching process was developed to mimic Stroop Color and Word Task (SCWT) performance on Adaptive Control of Thought-Rational (ACT-R) cognitive architecture based on IBLT. Genotype-phenotype correlation was conducted based on the mutation location in DMD gene. Assessment of specific cognitive domains in DP140 − ve group corresponded to the involvement of multiple brain lobes including temporal (verbal and visual learning and memory), parietal (visuo-conceptual and visuo-constructive abilities) and frontal (sustained and focused attention, verbal fluency, cognitive control). Working memory axis was found to be the central domain through tasks including RAVLT trial 1, recency effect, digit span backward, working memory index, arithmetic subtests in the Dp140 − ve group. IBLT validated the non-reliance of DMD subjects on recency indicating affected working memory domain. Modeling strategy revealed altered working memory processes in DMD cases with affected Dp140 isoform. DMD brain was observed to rely on primacy than the recency suggesting alterations in working memory capacity. Modeling revealed lowered activation of DMD brain with Dp140 − ve in order to retrieve the instances.
“…Saturation in the learning performance also indicates poor availability of neural resources to support higher level cognitive processing consistent with the neural integrity as suggested by Dixon et al 37 . The distal mutation location in DMD has previously been associated to severe intellectual deficits 5,19,35,38 . www.nature.com/scientificreports/ Poor performance in recalling list B indicates susceptibility to interference from previously acquired memory.…”
Duchenne Muscular Dystrophy has emerged as a model to assess cognitive domains. the DMD gene variant location and its association with variable degrees of cognitive impairment necessitate identification of a common denominator. Computer architectures provide a framework to delineate the mechanisms involved in the cognitive functioning of the human brain. copy number variations in the 79 exons of DMD gene were screened in 84 DMD subjects by Multiplex Ligation-dependent Probe Amplification (MLPA). DMD subjects were categorized based on the presence or absence of DP140 isoform. the cognitive and neuropsychological assessments were carried out as per inclusion criteria using standard scales. Instance-based learning theory (IBLT) based on the partial matching process was developed to mimic Stroop Color and Word Task (SCWT) performance on Adaptive Control of Thought-Rational (ACT-R) cognitive architecture based on IBLT. Genotype-phenotype correlation was conducted based on the mutation location in DMD gene. Assessment of specific cognitive domains in DP140 − ve group corresponded to the involvement of multiple brain lobes including temporal (verbal and visual learning and memory), parietal (visuo-conceptual and visuo-constructive abilities) and frontal (sustained and focused attention, verbal fluency, cognitive control). Working memory axis was found to be the central domain through tasks including RAVLT trial 1, recency effect, digit span backward, working memory index, arithmetic subtests in the Dp140 − ve group. IBLT validated the non-reliance of DMD subjects on recency indicating affected working memory domain. Modeling strategy revealed altered working memory processes in DMD cases with affected Dp140 isoform. DMD brain was observed to rely on primacy than the recency suggesting alterations in working memory capacity. Modeling revealed lowered activation of DMD brain with Dp140 − ve in order to retrieve the instances.
“…People with DMD mainly experience motor impairments (primarily muscle weakness), but a large number of people with DMD also experience cognitive impairments and impaired ability in processing visual information [28]. A recent study showed that even individuals with DMD without intellectual disability have a deficit in implicit learning [29]. Therefore, we cannot assume that individuals with DMD will also improve due to implicit learning that will lead to lower cognitive effort.…”
Duchenne muscular Dystrophy (DMD) is a progressive degenerative muscle disease, affecting, among others, the upper extremities. Effective hand rehabilitation can improve the hand function of people with DMD. To reach this goal, we first need to gain more insight into the hand cognitive-motor performance of people with DMD. This is the first study employing a systematic analysis on multi-finger, cognitive-motor performance of people with DMD. For this purpose, we propose an active dynamic visuo-motor task. The task employed six visual stimuli, a subset of which was activated at each trial. The stimuli were activated with a frequency of 1, 2, 3 and 4 Hz. Eight healthy participants and three participants with DMD performed the task. Additionally, the healthy participants performed seven sessions, and we assessed the training effects. Task-related cognitive-motor performance was evaluated using information transfer rate (ITR) and perceived workload. Regarding ITR, healthy participants performed significantly better than DMD participants; however, this was more evident for trials involving more than three fingers. Workload showed no difference between the healthy and the DMD groups. Healthy participants significantly improved their performance during training. Our results suggest that hand rehabilitation of people with DMD should consider multi-finger dynamic training. However, additional research with more people with DMD is needed for further generalization of our conclusions.
“…Le cervelet jouerait un rôle important dans la détection des séquences d'événements, ainsi que dans l'acquisition et l'automatisation de procédures cognitives. L'objectif de l'étude [1] est de comparer, à l'aide d'un test d'apprentissage implicite (Serial Reaction Time Task -SRTT, version modifiée), les capacités d'apprentissage implicite chez des enfants atteints de DMD sans retard intellectuel (n = 32) versus un groupe contrôle d'enfants se présentant avec un développement normal et appariés pour l'âge (n = 37). Le SRTT évalue l'apprentissage d'un rythme d'apparition d'un stimulus, sans que le sujet n'ait conscience de réaliser une tâche de mémoire.…”
Section: Claire-cécile Michon Christian Réveillèreunclassified
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