Weaker number sense accounts for impaired numerosity perception in dyscalculia: Behavioral and computational evidence.

Impaired numerosity perception in developmental dyscalculia (low "number acuity") has been interpreted as evidence of reduced representational precision in the neurocognitive system supporting non-symbolic number sense. However, recent studies suggest that poor numerosity judgments might stem from stronger interference from non-numerical visual information, in line with alternative accounts that highlight impairments in executive functions and visuospatial abilities in the etiology of dyscalculia. To resolve this debate, we used a psychophysical method designed to disentangle the contribution of numerical and non-numerical features to explicit numerosity judgments in a dot comparison task and we assessed the relative saliency of numerosity in a spontaneous categorization task. Children with dyscalculia were compared to control children with average mathematical skills matched for age, IQ, and visuospatial memory. In the comparison task, the lower accuracy of dyscalculics compared to controls was linked to weaker encoding of numerosity, but not to the strength of non-numerical biases. Similarly, in the spontaneous categorization task, children with dyscalculia showed a weaker number-based categorization compared to the control group, with no evidence of a stronger influence of non-numerical information on category choice. Simulations with a neurocomputational model of numerosity perception showed that the reduction of representational resources affected the progressive refinement of number acuity, with little effect on non-numerical bias in numerosity judgments. Together, these results suggest that impaired numerosity perception in dyscalculia cannot be explained by increased interference from non-numerical visual cues, thereby supporting the hypothesis of a core number sense deficit. RESEARCH HIGHLIGHTS: A strongly debated issue is whether impaired numerosity perception in dyscalculia stems from a deficit in number sense or from poor executive and visuospatial functions. Dyscalculic children show reduced precision in visual numerosity judgments and weaker number-based spontaneous categorization, but no increasing reliance on continuous visual properties. Simulations with deep neural networks demonstrate that reduced neural/computational resources affect the developmental trajectory of number acuity and account for impaired numerosity judgments. Our findings show that weaker number acuity in developmental dyscalculia is not necessarily related to increased interference from non-numerical visual cues.

Low discriminative power of WISC cognitive profile in developmental dyscalculia.

BACKGROUND: The role of domain-general cognitive abilities in the etiology of Developmental Dyscalculia (DD) is a hotly debated issue. AIMS: In the present study, we tested whether WISC-IV cognitive profiles can be useful to single out DD. METHODS AND PROCEDURES: Using a stringent 2-SD cutoff in a standardized numeracy battery, we identified children with DD (N = 43) within a clinical sample referred for assessment of learning disability and compared them in terms of WISC cognitive indexes to the remaining children without DD (N = 100) employing cross-validated logistic regression. OUTCOMES AND RESULTS: Both groups showed higher Verbal Comprehension and Perceptual Reasoning than Working Memory and Processing Speed, and DD scores were generally lower. Predictive accuracy of WISC indexes in identifying DD individuals was low (AUC = 0.67) and it dropped to chance level in discriminating DD from selected controls (N = 43) with average math performance but matched on global IQ. The inclusion of a visuospatial memory score as an additional predictor did not improve classification accuracy. CONCLUSIONS AND IMPLICATIONS: These results demonstrate that cognitive profiles do not reliably discriminate DD from non-DD children, thereby weakening the appeal of domain-general accounts.

Severe Developmental Dyscalculia Is Characterized by Core Deficits in Both Symbolic and Nonsymbolic Number Sense.

A long-standing debate concerns whether developmental dyscalculia is characterized by core deficits in processing nonsymbolic or symbolic numerical information as well as the role of domain-general difficulties. Heterogeneity in recruitment and diagnostic criteria make it difficult to disentangle this issue. Here, we selected children (n = 58) with severely compromised mathematical skills (2 SD below average) but average domain-general skills from a large sample referred for clinical assessment of learning disabilities. From the same sample, we selected a control group of children (n = 42) matched for IQ, age, and visuospatial memory but with average mathematical skills. Children with dyscalculia showed deficits in both symbolic and nonsymbolic number sense assessed with simple computerized tasks. Performance in the digit-comparison task and the numerosity match-to-sample task reliably separated children with developmental dyscalculia from controls in cross-validated logistic regression (area under the curve = .84). These results support a number-sense-deficit theory and highlight basic numerical abilities that could be targeted for early identification of at-risk children as well as for intervention.

Morpho-Syntactic Deficit in Children with Cochlear Implant: Consequence of Hearing Loss or Concomitant Impairment to the Language System?

BACKGROUND: Among implanted children with similar duration of auditory deprivation and clinical history, the morpho-syntactic skills remain highly variable, suggesting that other fundamental factors may determine the linguistic outcomes of these children, beyond their auditory recovery. The present study analyzed the morpho-syntactic discrepancies among three children with cochlear implant (CI), with the aim of understanding if morpho-syntactic deficits may be characterized as a domain-specific language disorder. METHOD: The three children (mean age = 7.2; SD = 0.4) received their CI at 2.7, 3.7, and 5.9 years of age. Their morpho-syntactic skills were evaluated in both comprehension and production and compared with 15 age-matched normal-hearing children (mean age = 6.6; SD = 0.3). RESULTS: Cases 1 and 2 displayed a marked impairment across morphology and syntax, whereas Case 3, the late-implanted child, showed a morpho-syntactic profile well within the normal boundaries. A qualitative analysis showed, in Cases 1 and 2, language deficits similar to those of normal hearing children with Developmental Language Disorder (DLD). CONCLUSIONS: We suggest that a severe grammatical deficit may be, in some implanted children, the final outcome of a concomitant impairment to the language system. Clinical implications for assessment and intervention are discussed.

Diverse Linguistic Development in Prelingually Deaf Children with Cochlear Implants.

The advent of cochlear implants has enormously improved the quality of sensory perception in deaf children. Notwithstanding these advantages, the current literature shows a substantial variability in language proficiency among implanted children. This case series explores the variability of language acquisition in congenitally deaf children with cochlear implants. We report 4 prelingually deaf children (mean age = 10.5; SD = 1.08), affected by a genetically determined bilateral deafness, due to GJB2 gene mutation Cx26. Each implanted child underwent a systematic assessment of speech perception and production, as well as of lexical, morphologic, and syntactic skills in both comprehension and production. Notwithstanding similar clinical histories and similarly good postimplant pure-tone audiometry, two of the four children fared very poorly in speech audiometry, whereas the other two children gained very good results. We suggest that the language impairment detected in (some) implanted children may not be fully accounted for by pure auditory thresholds and that may be the outcome of concomitant damage to core components of the child's linguistic brain.

Facial emotion recognition in childhood: the effects of febrile seizures in the developing brain.

It has been documented that anteromedial temporal lobe dysfunction can cause impairment in emotional intelligence. In particular, medial temporal lobe epilepsy (MTLE) is associated with disorders in emotion recognition from facial expressions. About one-third of patients with MTLE experienced febrile seizures (FSs) during childhood. In the present study, we investigated facial emotion recognition ability in a group of 38 school-aged children with antecedent FSs and in an age- and sex-matched control group. Children with abnormal general visuoperceptual abilities were excluded. Children with FSs showed lower recognition scores versus controls in both matching (28.64 vs 33.47; p<.0001) and labeling (21.25 vs 23.03; p=.001) facial emotions. Our findings support the hypothesis that FSs can be associated during childhood with a dysfunction within the neural network subserving the processing of facial expressions of the basic emotions.

Randomized trial of observation and execution of upper extremity actions versus action alone in children with unilateral cerebral palsy.

BACKGROUND: The properties of the mirror neuron system suggest a new type of upper limb (UL) rehabilitation in children with unilateral cerebral palsy (UCP), based on observation of action therapy followed by execution of a variety of observed movements (AOT). OBJECTIVE: We tested the effects of AOT in the Upper Limb Children Action Observation Training (UP-CAT) trial. METHODS: In a randomized, evaluator-blinded, block-designed trial, 24 UCP children with mild to moderate hand impairment were assigned to 2 groups. The experimental group observed, 1 hour per day for 3 consecutive weeks, video sequences of unimanual or bimanual goal-directed actions and subsequently executed observed actions with the hemiparetic UL or both ULs. The control group performed the same actions in the same order as the experimental sample, but had watched computer games. The Assisting Hand Assessment (AHA) scale was the primary outcome measure; the Melbourne assessment and ABILHAND-Kids were secondary ones. Outcomes were assessed at 1 week (T1), 8 weeks (T2), and 24 weeks (T3) after the end of the training. RESULTS: The experimental group improved more (P = .008) in score changes for the AHA at the primary endpoints T1 (P = .008), T2 (P = .019), and T3 (P = .049). No between-group significant changes were found for ABILHAND-Kids or Melbourne assessment. CONCLUSIONS: UP-CAT improved daily UL activities in UCP children, suggesting a new rehabilitation approach based on a neurophysiological model of motor learning.

Is a lone right hemisphere enough? Neurolinguistic architecture in a case with a very early left hemispherectomy.

We studied the linguistic profile and neurolinguistic organization of a 14-year-old adolescent (EB) who underwent a left hemispherectomy at the age of 2.5 years. After initial aphasia, his language skills recovered within 2 years, with the exception of some word finding problems. Over the years, the neuropsychological assessments showed that EB's language was near-to-normal, with the exception of lexical competence, which lagged slightly behind for both auditory and written language. Moreover, EB's accuracy and speed in both reading and writing words and non-words were within the normal range, whereas difficulties emerged in reading loan words and in tasks with homophones. EB's functional magnetic resonance imaging (fMRI) patterns for several linguistic and metalinguistic tasks were similar to those observed in the dominant hemisphere of controls, suggesting that his language network conforms to a left-like linguistic neural blueprint. However, a stronger frontal recruitment suggests that linguistic tasks are more demanding for him. Finally, no specific reading activation was found in EB's occipitotemporal region, a finding consistent with the surface dyslexia-like behavioral pattern of the patient. While a lone right hemisphere may not be sufficient to guarantee full blown linguistic competences after early hemispherectomy, EB's behavioral and fMRI patterns suggest that his lone right hemisphere followed a left-like blueprint of the linguistic network.

Centrotemporal spikes during NREM sleep: The promoting action of thalamus revealed by simultaneous EEG and fMRI coregistration.

Benign childhood epilepsy with centrotemporal spikes (BECTS) has been investigated through EEG-fMRI with the aim of localizing the generators of the epileptic activity, revealing, in most cases, the activation of the sensory-motor cortex ipsilateral to the centrotemporal spikes (CTS). In this case report, we investigated the brain circuits hemodynamically involved by CTS recorded during wakefulness and sleep in one boy with CTS and a language disorder but without epilepsy. For this purpose, the patient underwent EEG-fMRI coregistration. During the "awake session", fMRI analysis of right-sided CTS showed increments of BOLD signal in the bilateral sensory-motor cortex. During the "sleep session", BOLD increments related to right-sided CTS were observed in a widespread bilateral cortical-subcortical network involving the thalamus, basal ganglia, sensory-motor cortex, perisylvian cortex, and cerebellum. In this patient, who fulfilled neither the diagnostic criteria for BECTS nor that for electrical status epilepticus in sleep (ESES), the transition from wakefulness to sleep was related to the involvement of a widespread cortical-subcortical network related to CTS. In particular, the involvement of a thalamic-perisylvian neural network similar to the one previously observed in patients with ESES suggests a common sleep-related network dysfunction even in cases with milder phenotypes without seizures. This finding, if confirmed in a larger cohort of patients, could have relevant therapeutic implication.

Motor representation of actions in children with autism.

BACKGROUND: Children with Autistic Spectrum Disorders (ASD) are frequently hampered by motor impairment, with difficulties ranging from imitation of actions to recognition of motor intentions. Such a widespread inefficiency of the motor system is likely to interfere on the ontogeny of both motor planning and understanding of the goals of actions, thus delivering its ultimate effects on the emergence of social cognition. METHODOLOGY/PRINCIPAL FINDINGS: We investigate the organization of action representation in 15 high functioning ASD (mean age: 8.11) and in two control samples of typically developing (TD) children: the first one, from a primary school, was matched for chronological age (CA), the second one, from a kindergarten, comprised children of much younger age (CY). We used nine newly designed behavioural motor tasks, aiming at exploring three domains of motor cognition: 1) imitation of actions, 2) production of pantomimes, and 3) comprehension of pantomimes. The findings reveal that ASD children fare significantly worse than the two control samples in each of the inspected components of the motor representation of actions, be it the imitation of gestures, the self-planning of pantomimes, or the (verbal) comprehension of observed pantomimes. In the latter task, owing to its cognitive complexity, ASD children come close to the younger TD children's level of performance; yet they fare significantly worse with respect to their age-mate controls. Overall, ASD children reveal a profound damage to the mechanisms that control both production and pre-cognitive "comprehension" of the motor representation of actions. CONCLUSIONS/SIGNIFICANCE: Our findings suggest that many of the social cognitive impairments manifested by ASD individuals are likely rooted in their incapacity to assemble and directly grasp the intrinsic goal-related organization of motor behaviour. Such impairment of motor cognition might be partly due to an early damage of the Mirror Neuron Mechanism (MNM).

Mortality risk after neonatal seizures in very preterm newborns.

We analyzed clinical and instrumental data of 403 consecutive newborns with gestational age from 24 to 32 weeks, admitted to the University-Hospital of Parma between January 2000 and December 2007, to evaluate the possible relationship between neonatal mortality and occurrence of neonatal seizures in very preterm newborns. Seventy-four subjects died during hospital stay. Seizures were present in 35 neonates, in whom the mortality rate was 37.1%. Multivariate analysis revealed that birth-weight <1000 g (odds ratio: 4.48; 95% confidence interval: 1.47-13.68; P < .01), cardiopulmonary resuscitation (odds ratio: 5.35; 95% confidence interval: 1.19-23.98; P = .02), and moderately and severely abnormal cerebral ultrasound scan findings (odds ratio: 2.48; 95% confidence interval: 1.02-6.05; P < .04; odds ratio: 9.56; 95% confidence interval: 3.45-26.51; P < .01, respectively) were related to the in-hospital mortality but not the presence of neonatal seizures. Our study suggests that neonatal seizures alone are not an independent risk factor for early death in very preterm newborns.

Hemimegalencephalic variant of epidermal nevus syndrome: case report and literature review.

The epidermal nevus syndrome (ENS) is an uncommon neurocutaneous disorder in which epidermal nevi are found in association with congenital abnormalities of the brain, eye, and/or skeleton. The association of epidermal nevi and neurologic abnormalities was comprehensively described by Schimmelpenning in 1957. Pavone et al. (1991) identified a homogeneous variant of ENS with hemimegalencephaly, gyral malformation, mental retardation, seizures and facial hemihypertrophy. A 13-year-old boy with the neurologic variant of ENS with hemimegalencephaly, facial asymmetry, febrile seizures and mental retardation is reported. Additionally, we performed a literature review using the search terms "epidermal nevus syndrome" and "hemimegalencephaly", including secondary sources of data such as reference lists of articles reviewed. We found 57 previously reported cases with the hemimegalencephalic variant of epidermal nevus syndrome, in which the most frequent associated features are severe epilepsy, in about half of cases with neonatal onset, mental retardation/developmental delay, ocular/visual involvement, and facial abnormalities.

Upper limb children action-observation training (UP-CAT): a randomised controlled trial in hemiplegic cerebral palsy.

BACKGROUND: Rehabilitation for children with hemiplegic cerebral palsy (HCP) aimed to improve function of the impaired upper limb (UL) uses a wide range of intervention programs. A new rehabilitative approach, called Action-Observation Therapy, based on the recent discovery of mirror neurons, has been used in adult stroke but not in children. The purpose of the present study is to design a randomised controlled trial (RCT) for evaluating the efficacy of Action-Observation Therapy in improving UL activity in children with HCP. METHODS/DESIGN: The trial is designed according to CONSORT Statement. It is a randomised, evaluator-blinded, match-pair group trial. Children with HCP will be randomised within pairs to either experimental or control group. The experimental group will perform an Action-Observation Therapy, called UP-CAT (Upper Limb-Children Action-Observation Training) in which they will watch video sequences showing goal-directed actions, chosen according to children UL functional level, combined with motor training with their hemiplegic UL. The control group will perform the same tailored actions after watching computer games. A careful revision of psychometric properties of UL outcome measures for children with hemiplegia was performed. Assisting Hand Assessment was chosen as primary measure and, based on its calculation power, a sample size of 12 matched pairs was established. Moreover, Melbourne and ABILHAND-Kids were included as secondary measures. The time line of assessments will be T0 (in the week preceding the onset of the treatment), T1 and T2 (in the week after the end of the treatment and 8 weeks later, respectively). A further assessment will be performed at T3 (24 weeks after T1), to evaluate the retention of effects. In a subgroup of children enrolled in both groups functional Magnetic Resonance Imaging, exploring the mirror system and sensory-motor function, will be performed at T0, T1 and T2. DISCUSSION: The paper aims to describe the methodology of a RCT for evaluating the efficacy of Action-Observation Therapy in improving UL activity in children with hemiplegia. This study will be the first to test this new type of treatment in childhood. The paper presents the theoretical background, study hypotheses, outcome measures and trial methodology. TRIAL REGISTRATION: NCT01016496.

Intention understanding in autism.

When we observe a motor act (e.g. grasping a cup) done by another individual, we extract, according to how the motor act is performed and its context, two types of information: the goal (grasping) and the intention underlying it (e.g. grasping for drinking). Here we examined whether children with autistic spectrum disorder (ASD) are able to understand these two aspects of motor acts. Two experiments were carried out. In the first, one group of high-functioning children with ASD and one of typically developing (TD) children were presented with pictures showing hand-object interactions and asked what the individual was doing and why. In half of the "why" trials the observed grip was congruent with the function of the object ("why-use" trials), in the other half it corresponded to the grip typically used to move that object ("why-place" trials). The results showed that children with ASD have no difficulties in reporting the goals of individual motor acts. In contrast they made several errors in the why task with all errors occurring in the "why-place" trials. In the second experiment the same two groups of children saw pictures showing a hand-grip congruent with the object use, but within a context suggesting either the use of the object or its placement into a container. Here children with ASD performed as TD children, correctly indicating the agent's intention. In conclusion, our data show that understanding others' intentions can occur in two ways: by relying on motor information derived from the hand-object interaction, and by using functional information derived from the object's standard use. Children with ASD have no deficit in the second type of understanding, while they have difficulties in understanding others' intentions when they have to rely exclusively on motor cues.

Motor cognition and its role in the phylogeny and ontogeny of action understanding.

Social life rests in large part on the capacity to understand the intentions behind the behavior of others. What are the origins of this capacity? How is one to construe its development in ontogenesis? By assuming that action understanding can be explained only in terms of the ability to read the minds of others--that is, to represent mental states--the traditional view claims that a sharp discontinuity occurs in both phylogeny and ontogeny. Over the last few years this view has been challenged by a number of ethological and psychological studies, as well as by several neurophysiological findings. In particular, the functional properties of the mirror neuron system and its direct matching mechanism indicate that action understanding may be primarily based on the motor cognition that underpins one's own capacity to act. This article aims to elaborate and motivate the pivotal role of such motor cognition, providing a biologically plausible and theoretically unitary account for the phylogeny and ontogeny of action understanding and also its impairment, as in the case of autistic spectrum disorder.

Is there a distinct form of developmental dyslexia in children with specific language impairment? Findings from an orthographically regular language.

OBJECTIVES: The aim of this study was to identify quantitative and qualitative differences between the reading and writing skills of children with developmental dyslexia and those of dyslexic children with a specific language impairment (SLI). BACKGROUND: It is suggested that although the etiology of developmental dyslexia and SLI may be diverse, dyslexic children with SLI and their language-intact peers are comparable on a behavioral level. METHODS: Three groups of second-grade children were compared on reading and writing tests with single words and nonwords: 15 dyslexic children with a history of SLI (SLI group), 15 dyslexic children with a typical pattern of language development (non-SLI group), and a control group of 30 children with no clinical history of learning disabilities or communication disorders. RESULTS: Analysis of the results revealed the performances of both SLI and non-SLI dyslexic groups to be comparable in terms of speed, accuracy, and error typology. CONCLUSIONS: This study confirms that there are parallels between dyslexic children with language disorders and their dyslexic peers with intact language skills, at least in terms of their performance on reading and writing tests.

Impairment of actions chains in autism and its possible role in intention understanding.

Experiments in monkeys demonstrated that many parietal and premotor neurons coding a specific motor act (e.g., grasping) show a markedly different activation when this act is part of actions that have different goals (e.g., grasping for eating vs. grasping for placing). Many of these "action-constrained" neurons have mirror properties firing selectively to the observation of the initial motor act of the actions to which they belong motorically. By activating a specific action chain from its very outset, this mechanism allows the observers to have an internal copy of the whole action before its execution, thus enabling them to understand directly the agent's intention. Using electromyographic recordings, we show that a similar chained organization exists in typically developing children, whereas it is impaired in children with autism. We propose that, as a consequence of this functional impairment, high-functioning autistic children may understand the intentions of others cognitively but lack the mechanism for understanding them experientially.

Introductory remarks on musical beginnings: ten years later.

Over the last 10 years, the neurosciences have witnessed an exponential increase of research in the neuropsychology of music. Consequently, old and new questions on the ontogeny of musical skills can be refined and reformulated with greater accuracy. Three interrelated issues are discussed.