Dance Improves Motor, Cognitive, and Social Skills in Children With Developmental Cerebellar Anomalies.

In this multiple single-cases study, we used dance to train sensorimotor synchronization (SMS), motor, and cognitive functions in children with developmental cerebellar anomalies (DCA). DCA are rare dysfunctions of the cerebellum that affect motor and cognitive skills. The cerebellum plays an important role in temporal cognition, including SMS, which is critical for motor and cognitive development. Dancing engages the SMS neuronal circuitry, composed of the cerebellum, the basal ganglia, and the motor cortices. Thus, we hypothesized that dance has a beneficial effect on SMS skills and associated motor and cognitive functions in children with DCA. Seven children (aged 7-11) with DCA participated in a 2-month dance training protocol (3 h/week). A test-retest design protocol with multiple baselines was used to assess children's SMS skills as well as motor, cognitive, and social abilities. SMS skills were impaired in DCA before the training. The training led to improvements in SMS (reduced variability in paced tapping), balance, and executive functioning (cognitive flexibility), as well as in social skills (social cognition). The beneficial effects of the dance training were visible in all participants. Notably, gains were maintained 2 months after the intervention. These effects are likely to be sustained by enhanced activity in SMS brain networks due to the dance training protocol.

An Intracranial EEG Study of the Neural Dynamics of Musical Valence Processing.

The processing of valence is known to recruit the amygdala, orbitofrontal cortex, and relevant sensory areas. However, how these regions interact remains unclear. We recorded cortical electrical activity from 7 epileptic patients implanted with depth electrodes for presurgical evaluation while they listened to positively and negatively valenced musical chords. Time-frequency analysis suggested a specific role of the orbitofrontal cortex in the processing of positively valenced stimuli while, most importantly, Granger causality analysis revealed that the amygdala tends to drive both the orbitofrontal cortex and the auditory cortex in theta and alpha frequency bands, during the processing of valenced stimuli. Results from the current study show the amygdala to be a critical hub in the emotion processing network: specifically one that influences not only the higher order areas involved in the evaluation of a stimulus's emotional value but also the sensory cortical areas involved in the processing of its low-level acoustic features.

TMEM240 mutations cause spinocerebellar ataxia 21 with mental retardation and severe cognitive impairment.

Autosomal dominant cerebellar ataxia corresponds to a clinically and genetically heterogeneous group of neurodegenerative disorders that primarily affect the cerebellum. Here, we report the identification of the causative gene in spinocerebellar ataxia 21, an autosomal-dominant disorder previously mapped to chromosome 7p21.3-p15.1. This ataxia was firstly characterized in a large French family with slowly progressive cerebellar ataxia, accompanied by severe cognitive impairment and mental retardation in two young children. Following the recruitment of 12 additional young family members, linkage analysis enabled us to definitively map the disease locus to chromosome 1p36.33-p36.32. The causative mutation, (c.509C>T/p.P170L) in the transmembrane protein gene TMEM240, was identified by whole exome sequencing and then was confirmed by Sanger sequencing and co-segregation analyses. Index cases from 368 French families with autosomal-dominant cerebellar ataxia were also screened for mutations. In seven cases, we identified a range of missense mutations (c.509C>T/p.P170L, c.239C>T/p.T80M, c.346C>T/p.R116C, c.445G>A/p.E149K, c.511C>T/p.R171W), and a stop mutation (c.489C>G/p.Y163*) in the same gene. TMEM240 is a small, strongly conserved transmembrane protein of unknown function present in cerebellum and brain. Spinocerebellar ataxia 21 may be a particular early-onset disease associated with severe cognitive impairment.

Rhythm as an independent determinant of developmental dyslexia.

Temporal accounts of Developmental Dyslexia (DD) postulate that a timing impairment plays an important role in this learning disorder. However, DD has been associated with timing disorders as well as other motor and cognitive dysfunctions. It is still unclear whether nonverbal timing skills per se may be considered as independent determinants of DD. In this study, we investigated the independent contribution of predictive timing to DD above and beyond the motor and cognitive dysfunctions typically associated with this disorder. Twenty-one children with DD (aged 8-12, nine females) and 27 controls (14 females) were evaluated on perceptual timing, finger tapping, fine motor control, as well as attention and executive tasks. Participants were native French speakers from various socioeconomic backgrounds. The performance of children with DD was poorer than that of controls in most of the tasks. Predictors of DD, as identified by logistic regression modeling, were beat perception and precision in tapping to the beat, which are both predictive timing variables, children's tapping rate, and cognitive flexibility. These data support temporal accounts of DD in which predictive timing impairments partially explain the core phonological deficit, independent from general motor and cognitive functioning, making predictive timing a valuable tool for early diagnosis and remediation of DD. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Musical abilities in children with developmental cerebellar anomalies.

Developmental Cerebellar Anomalies (DCA) are rare diseases (e.g., Joubert syndrome) that affect various motor and non-motor functions during childhood. The present study examined whether music perception and production are affected in children with DCA. Sixteen children with DCA and 37 healthy matched control children were tested with the Montreal Battery for Evaluation of Musical Abilities (MBEMA) to assess musical perception. Musical production was assessed using two singing tasks: a pitch-matching task and a melodic reproduction task. Mixed model analyses showed that children with DCA were impaired on the MBEMA rhythm perception subtest, whereas there was no difference between the two groups on the melodic perception subtest. Children with DCA were also impaired in the melodic reproduction task. In both groups, singing performance was positively correlated with rhythmic and melodic perception scores, and a strong correlation was found between singing ability and oro-bucco-facial praxis in children with DCA. Overall, children with DCA showed impairments in both music perception and production, although heterogeneity in cerebellar patient's profiles was highlighted by individual analyses. These results confirm the role of the cerebellum in rhythm processing as well as in the vocal sensorimotor loop in a developmental perspective. Rhythmic deficits in cerebellar patients are discussed in light of recent work on predictive timing networks including the cerebellum. Our results open innovative remediation perspectives aiming at improving perceptual and/or production musical abilities while considering the heterogeneity of patients' clinical profiles to design music-based therapies.

Corrigendum: Musical abilities in children with developmental cerebellar anomalies.

[This corrects the article DOI: 10.3389/fnsys.2022.886427.].

The Impact of Emotion on Musical Long-Term Memory.

The influence of emotional dimensions such as arousal and valence on memory has been a topic of particularly intense inquiry. As stimuli go, music is capable of provoking strong emotional responses from listeners, which can in turn influence memory. However, few studies have examined the effect of musical emotions on memory, and even fewer the effect of valence and arousal. In order to shed light on the ways in which emotional dimensions affect musical memory as study-test delay intervals increase, we tested recognition after a short delay and after a long delay. In line with the literature, we hypothesized an emotional enhancement of music memory induced by post-encoding processes leading to better recognition of musical excerpts in delayed condition, as compared to the immediate condition. The effects of arousal and valence were expected to become exaggerated after a long delay. We also predicted that the two emotional dimensions would be differently affected by the study-test intervals. Our results showed that the emotional enhancement of memory depends upon the valence, with remembering of positive and negative stimuli being differently affected by the duration of the study-test delay interval. Furthermore, our data demonstrated that musical excerpts were better recognized after a long delay than after a short delay, illustrating that memory consolidation for musical information is taking place during the long study-test interval. Moreover, musical memory consolidation is strongly related to the characteristics of the positive valence, which have been discussed in relation to its pleasantness. This original finding provides new insights into the modulatory effects of emotional valence on memory consolidation and could offer promising therapeutic possibilities for the rehabilitation of memory disorders.

Chapman-Cook' complex reading comprehension test: better performances for aged participants in comparison with youngers for level of schooling lower than baccalaureate.

We studied the comprehension abilities of healthy participants with a French version of the Chapman-Cook Speed of Reading Test. The objective was to assess the effect of gender, age and educational level on chronometric performances and errors. In this test, the task is to cross out an inappropriate word within short passages. In the original version, the participant is told to perform as quickly as possible during 150 seconds. The score is usually the number of passages correctly completed within this time limit. In the present study, we measured the time to achieve the first 10 passages, the first 14 passages corresponding to the first page and the total (29 passages) corresponding to the two pages. The number of errors was also considered. The normative sample included 150 participants (63 males; 87 females) with three educational level (47: superior to baccalaureate; 21: baccalaureate and 78: inferior to baccalaureate). Age was between 20 and 69 years old, divided in 5 age groups, without neurological or psychiatric disease, or cognitive abnormal development. All were French native speaking and have been schooling in France. For time completion, no effect of gender was found, but a significant and unexpected effect of age was shown according to educational level. Whereas the age groups obtained similar times for educational levels superior to baccalaureate, an age effect was demonstrated for the educational level inferior to baccalaureate. Participants over 40 years of age were faster than younger participants with the same educational level and similar than all age groups of higher educational level. On the contrary, young participants were slower compared to those with high educational levels and all older participants without baccalaureate. This surprising result is discussed.

Children and adults with Attention-Deficit/Hyperactivity Disorder cannot move to the beat.

Children and adults with Attention-Deficit Hyperactivity Disorder (ADHD) fail in simple tasks like telling whether two sounds have different durations, or in reproducing single durations. The deficit is linked to poor reading, attention, and language skills. Here we demonstrate that these timing distortions emerge also when tracking the beat of rhythmic sounds in perceptual and sensorimotor tasks. This contrasts with the common observation that durations are better perceived and produced when embedded in rhythmic stimuli. Children and adults with ADHD struggled when moving to the beat of rhythmic sounds, and when detecting deviations from the beat. Our findings point to failure in generating an internal beat in ADHD while listening to rhythmic sounds, a function typically associated with the basal ganglia. Rhythm-based interventions aimed at reinstating or compensating this malfunctioning circuitry may be particularly valuable in ADHD, as already shown for other neurodevelopmental disorders, such as dyslexia and Specific Language Impairment.

Expanding the phenotype of SCA19/22: Parkinsonism, cognitive impairment and epilepsy.

INTRODUCTION: Spinocerebellar ataxia types 19 and 22 (SCA19/22) are rare conditions in which relatively isolated cerebellar involvement is frequently associated with cognitive impairment. Here, we report on new clinical features and provide details of the cognitive profile in two SCA19/22 families. METHODS: Two families displaying an autosomal-dominant form of cerebellar ataxia underwent clinical examinations and genetic testing. RESULTS: In addition to the classical clinical features of SCA, a wide spectrum of cognitive disorders (including visuospatial impairments) was observed. Eight patients had mild Parkinsonism, and five had epilepsy. Genetic testing showed that the KCND3 mutation (c.679_681delTTC, p.F227del) was present in both families. CONCLUSIONS: Our findings broaden the phenotypic spectrum of SCA19/22, and suggest that KCND3 should be included in the list of candidate genes for epilepsy, Parkinsonism and cognitive impairment.

Emotional memory for musical excerpts in young and older adults.

The emotions evoked by music can enhance recognition of excerpts. It has been suggested that memory is better for high than for low arousing music (Eschrich et al., 2005; Samson et al., 2009), but it remains unclear whether positively (Eschrich et al., 2008) or negatively valenced music (Aubé et al., 2013; Vieillard and Gilet, 2013) may be better recognized. Moreover, we still know very little about the influence of age on emotional memory for music. To address these issues, we tested emotional memory for music in young and older adults using musical excerpts varying in terms of arousal and valence. Participants completed immediate and 24 h delayed recognition tests. We predicted highly arousing excerpts to be better recognized by both groups in immediate recognition. We hypothesized that arousal may compensate consolidation deficits in aging, thus showing more prominent benefit of high over low arousing stimuli in older than younger adults on delayed recognition. We also hypothesized worst retention of negative excerpts for the older group, resulting in a recognition benefit for positive over negative excerpts specific to older adults. Our results suggest that although older adults had worse recognition than young adults overall, effects of emotion on memory do not seem to be modified by aging. Results on immediate recognition suggest that recognition of low arousing excerpts can be affected by valence, with better memory for positive relative to negative low arousing music. However, 24 h delayed recognition results demonstrate effects of emotion on memory consolidation regardless of age, with a recognition benefit for high arousal and for negatively valenced music. The present study highlights the role of emotion on memory consolidation. Findings are examined in light of the literature on emotional memory for music and for other stimuli. We finally discuss the implication of the present results for potential music interventions in aging and dementia.

The feeling of familiarity for music in patients with a unilateral temporal lobe lesion: A gating study.

Previous research has indicated that the medial temporal lobe (MTL), and more specifically the perirhinal cortex, plays a role in the feeling of familiarity for non-musical stimuli. Here, we examined contribution of the MTL to the feeling of familiarity for music by testing patients with unilateral MTL lesions. We used a gating paradigm: segments of familiar and unfamiliar musical excerpts were played with increasing durations (250, 500, 1000, 2000, 4000 ms and complete excerpts), and participants provided familiarity judgments for each segment. Based on the hypothesis that patients might need longer segments than healthy controls (HC) to identify excerpts as familiar, we examined the onset of the emergence of familiarity in HC, patients with a right MTL resection (RTR), and patients with a left MTL resection (LTR). In contrast to our hypothesis, we found that the feeling of familiarity was relatively spared in patients with a right or left MTL lesion, even for short excerpts. All participants were able to differentiate familiar from unfamiliar excerpts as early as 500 ms, although the difference between familiar and unfamiliar judgements was greater in HC than in patients. These findings suggest that a unilateral MTL lesion does not impair the emergence of the feeling of familiarity. We also assessed whether the dynamics of the musical excerpt (linked to the type and amount of information contained in the excerpts) modulated the onset of the feeling of familiarity in the three groups. The difference between familiar and unfamiliar judgements was greater for high than for low-dynamic excerpts for HC and RTR patients, but not for LTR patients. This indicates that the LTR group did not benefit in the same way from dynamics. Overall, our results imply that the recognition of previously well-learned musical excerpts does not depend on the integrity of either right or the left MTL structures. Patients with a unilateral MTL resection may compensate for the effects of unilateral damage by using the intact contralateral temporal lobe. Moreover, we suggest that remote semantic memory for music might depend more strongly on neocortical structures rather than the MTL.

Intracranial markers of emotional valence processing and judgments in music.

The involvement of the amygdala and orbitofrontal cortex in the processing of valenced stimuli is well established. However, less is known about the extent to which activity in these regions reflects a stimulus' physical properties, the individual subjective experience it evokes, or both. We recorded cortical electrical activity from five epileptic patients implanted with depth electrodes for presurgical evaluation while they rated "consonant" and "dissonant" musical chords using a "pleasantness" scale. We compared the pattern of responses in the amygdala and orbitofrontal cortex when trials were sorted by pleasantness judgments relative to when they were sorted by the acoustic properties known to influence emotional reactions to musical chords. This revealed earlier differential activity in the amygdala in the physical properties-based, relative to in the judgment-based, analyses. Thus, our results demonstrate that the amygdala has, first and foremost, a high initial sensitivity to the physical properties of valenced stimuli. The finding that differentiations in the amygdala based on pleasantness ratings had a longer latency suggests that in this structure, mediation of emotional judgment follows accumulation of sensory information. This is in contrast to the orbitofrontal cortex where sensitivity to sensory information did not precede differentiation based on affective judgments.

The effect of musical experience on emotional self-reports and psychophysiological responses to dissonance.

To study the influence of musical education on emotional reactions to dissonance, we examined self-reports and physiological responses to dissonant and consonant musical excerpts in listeners with low (LE: n=15) and high (HE: n=13) musical experience. The results show that dissonance induces more unpleasant feelings and stronger physiological responses in HE than in LE participants, suggesting that musical education reinforces aversion to dissonance. Skin conductance (SCR) and electromyographic (EMG) signals were analyzed according to a defense cascade model, which takes into account two successive time windows corresponding to orienting and defense responses. These analyses suggest that musical experience can influence the defense response to dissonance and demonstrate a powerful role of musical experience not only in autonomic but also in expressive responses to music.

Emotional power of music in patients with memory disorders: clinical implications of cognitive neuroscience.

By adapting methods of cognitive psychology to neuropsychology, we examined memory and familiarity abilities in music in relation to emotion. First we present data illustrating how the emotional content of stimuli influences memory for music. Second, we discuss recent findings obtained in patients with two different brain disorders (medically intractable epilepsy and Alzheimer's disease) that show relatively spared memory performance for music, despite severe verbal memory disorders. Studies on musical memory and its relation to emotion open up paths for new strategies in cognitive rehabilitation and reinstate the importance of examining interactions between cognitive and clinical neurosciences.

The birth of musical emotion: a depth electrode case study in a human subject with epilepsy.

Intracranial electroencephalography was recorded in an epileptic patient when he was listening to dissonant and consonant chords and to minor and major chords. Changes in dissonance induced event-related potentials (ERPs) in the auditory areas from 200 ms onward, in the orbito-frontal cortex (500-1000 ms), and later in the amygdala and anterior cingulate gyrus (1200-1400 ms), suggesting the sequential involvement of these brain structures in implicit emotional judgment of musical dissonance. Changes in musical mode induced ERPs only in the orbito-frontal cortex (500-1000 ms), emphasizing the implication of this frontal region in emotional judgment of pleasant music.