Interhemispheric Communication in Down Syndrome.

BACKGROUND: Within the domain of intellectual disability, evidence suggests a potential association with changes in the developmental pathways governing the maturation of interhemispheric communication mechanisms. SUBJECTS AND METHOD: Through this study, our objective was to identify potential anomalies in interhemispheric transfer among individuals with Down syndrome (DS). Specifically, we investigated whether a significant difference exists in interhemispheric communication efficiency between DS and control participants of similar chronological age. For our analysis, 11 participants with DS and 13 from the control group were evaluated. A fingertip cross-localization test was used to quantify the efficiency of interhemispheric information transfer. RESULTS: The data revealed a significantly reduced efficiency of interhemispheric communication in participants diagnosed with DS compared to the control group. CONCLUSIONS: These findings suggest a potential dysfunction in the corpus callosum of DS individuals. It is plausible to suggest that this functional disconnection might influence the development of cognitive anomalies.

The Relationship Between Math Anxiety and Math Achievement: Differentiating the Role of Learning Math Anxiety and Math Evaluation Anxiety.

BACKGROUND: Math anxiety (MA) is considered one of the variables that cause poor performance in contexts where mathematical competence is required. SUBJECTS AND METHODS: The survey involved 28 Italian students in the first year of secondary school (corresponding to grade nine of English-speaking schools and of the Italian INVALSI evaluation system). The students, examined at the beginning of the school year, scored below the threshold of sufficiency in the calculation skills assessment test. This data analysis takes into consideration the development of their math performance; the hypothesis is that the level of MA may differentiate students who are able or not able to achieve the competencies expected by the course of study. RESULTS: Students who transitioned from an inadequate score to a sufficient one over the school year, exhibited significantly lower levels of MA compared to students who did not score sufficient on the math test at the end of the year. However, the significant difference primarily pertained to the "learning math" subscale, while the "math evaluation" subscale showed no significant differences between the two groups. CONCLUSIONS: Our results suggest the possibility of using the assessment of MA levels to identify the presence and type of emotional difficulties associated with the study of mathematics. Specifically, in individuals with difficulties in learning mathematics, the presence of a dissociation between the two AMAS subscales could suggest different approaches to educational intervention.

Italian translation and cross-cultural adaptation of the Progressive Aphasia Severity Scale.

Primary progressive aphasia (PPA) is a neurodegenerative disease characterised by a progressive decline in language and speech as the first clinical manifestation, which mostly spares other cognitive functions. However, the linguistic impairment of PPA shows different features than that resulting from cerebrovascular diseases. The major difference between the linguistic manifestations of PPA and the traditional classification of aphasias has led to the development of new, more specific methods of language assessment. Among the currently available tools, there has been great interest in the Progressive Aphasia Severity Scale (PASS). This quick and easy-to-use clinical tool allows to collect significant information from caregivers about the communicative, linguistic, and functional difficulties of patients affected by PPA. In addition to monitoring the severity and progression of deficits in 13 different language domains, this scale integrates the classic "clinically reported" assessment with a "caregiver-reported" analysis of the daily experience of the patient, which provides a better understanding of how the disease affects the quality of life of both the patient and the caregiver. In the present contribution, the PASS was translated and adapted into Italian according to the international guidelines for the cross-cultural adaptation of self-report measures. This version of the PASS can help clinicians and researchers in the diagnosis of PPA in Italian clinical populations. Furthermore, it could be particularly useful for the long-term evaluation of the disease, in order to monitor its evolution, and might represent an optimal means to verify the efficacy of speech/language therapy in delaying the progression of the disease.

Music Playing and Interhemispheric Communication: Older Professional Musicians Outperform Age-Matched Non-Musicians in Fingertip Cross-Localization Test.

OBJECTIVE: Numerous investigations have documented that age-related changes in the integrity of the corpus callosum are associated with age-related decline in the interhemispheric transfer of information. Conversely, there is accumulating evidence for more efficient white matter organization of the corpus callosum in individuals with extensive musical training. However, the relationship between making music and accuracy in interhemispheric transfer remains poorly explored. METHODS: To test the hypothesis that musicians show enhanced functional connectivity between the two hemispheres, 65 professional musicians (aged 56-90 years) and 65 age- and sex-matched non-musicians performed the fingertip cross-localization test. In this task, subjects must respond to a tactile stimulus presented to one hand using the ipsilateral (intra-hemispheric test) or contralateral (inter-hemispheric test) hand. Because the transfer of information from one hemisphere to another may imply a loss of accuracy, the value of the difference between the intrahemispheric and interhemispheric tests can be utilized as a reliable measure of the effectiveness of hemispheric interactions. RESULTS: Older professional musicians show significantly greater accuracy in tactile interhemispheric transfer than non-musicians who suffer from age-related decline. CONCLUSIONS: Musicians have more efficient interhemispheric communication than age-matched non-musicians. This finding is in keeping with studies showing that individuals with extensive musical training have a larger corpus callosum. The results are discussed in relation to relevant data suggesting that music positively influences aging brain plasticity.

Music Training as a Potential Neuroprotective Agent.

A substantial amount of evidence suggest that music training is a powerful means of plastic reorganization of brain structures. Musical training is accompanied by plastic rearrangement of the central nervous system and numerous studies converge in disclosing that the brain differs significantly between musicians and non-musicians. Music-dependent brain changes concern both the grey and the white matter so that musicians possess a neuronal network shaped by type and degree of individual expertise as well as a different connectome than non-musicians. It is reasonable to assume that these plastic changes can provide a more efficient configuration of the neural network and justify an impact on cognition and behaviour in all ages of life. Furthermore, a number of studies suggest the effectiveness of "neurological music therapy" in clinical practice. Based on available literature, music should be considered one of the main activity that can preserve the brain efficiency and can be proposed as a primary non pharmachological agent in promoting a neuroprotective lifestyle. Understanding the variables of musical training that can positively influence brain plasticity might be one of the most exciting and promising areas of future research.

Mathematics Anxiety and Cognitive Performance in Adolescent Students.

Several studies highlight that many students feel negative feelings about mathematical learning and that the mathematics anxiety seems to play a central role in mathematical performance. More specifically students with higher level of maths anxiety are less efficient in mathematical tasks. The aim of this study was to investigate the relationship between specific mathematics anxiety as assessed by AMAS, trait and state anxiety as assessed by STAI-Y, and mathematical skills assessed through the ABCA tests in a sample of 83 adolescent students (78.3% males) without diagnosis of dyscalculia and cognitive disorder attending their first year of secondary school. Results showed that 38% of the students referred high level of maths anxiety. Independent T-test revealed that female students referred a higher level of maths anxiety as well as of trait and state anxiety than male ones, while there were no differences in the mathematics performance. The simultaneous multivariate linear regression analysis showed that maths anxiety was influenced by trait anxiety and in its turn has an impact on the high level mathematics performances (i.e. arithmetic facts). Understanding the relationships between maths anxiety and maths learning and performance may have relevant implications in clinical, educational and didactic practice.

Evaluation of the Quality of Rehabilitation Treatment in Neurodevelopmental Disorder.

Complex disability is very difficult to manage. It usually subtends very serious clinical pictures, because it affect several body systems, or because it is associated with intellectual disability and behavioral disorders. Often affected patients are unable to communicate their basic needs. All these factors combine to make the management of these patients very complex, and those who care for them realize how important it is to find a way to detect their state and to identify their potential capabilities. Developing appropriate rehabilitation programs for these patients requires additional effort and an assessment capacity that is as objective as possible. Few scales cited in the literature are capable of evaluating these aspects in patients with complex disabilities, among them the Barthel Index (Mahoney & Barthel 1965) and the Vineland Adaptive Behavior scale II (Sparrow et al. 2005). The majority of these scales often tend to depict the data regarding the disease to a degree of severity that precludes adequate individual rehabilitation program development. There is a dire need for a more appropriate instrument, an observational grid that is capable of identifying the potential of this patient population and evaluate the effectiveness of rehabilitation interventions provided. The aim of the study is to evaluate the efficacy of rehabilitation interventions in a group of patients with IQ <32 (determined by the Vineland II scale) using an evaluation tool created ad hoc called D-Rubrics, designed with the intent to identify "micro-differences" between baseline (T0) and post-rehabilitation (T1). The goal is part of a more long term-term objective which involves developing an effective assessment tool for patients with complex disabilities. Such an assessment tool should be practical, easy to administer and useful in both clinical and research settings.

The Effect of Mozart's Music in Severe Epilepsy: Functional and Morphological Features.

Music is a very important factor in everyday life, involving mood, emotions and memories. The effect of music on the brain is very debated. Certainly, music activates a complex network of neurones in auditory areas, mesolimbic areas, cerebellum and multisensory areas. In particular, music exerts its effects on the brain of patients with epilepsy, having a dichotomous influence: it can either be seizure-promoting in musicogenic epilepsy or antiepileptic. Several studies have shown that seizure-prone neural networks may be stimulated by certain periodicities while other frequencies may prevent seizure activity. There are a lot of data in the literature about the so-called "Mozart effect" (Rauscher et al. 1993). In previous studies we observed that in institutionalized subjects with severe/profound intellectual disability and drug-resistant epilepsy, a systematic music listening protocol reduced the frequency of seizures in about 50% of the cases. In this study we are conducting a survey on the observation of what happens to the brain of patients suffering from drug-resistant epilepsy through electroencephalographic investigations, brain MRI and behavioural analysis before and after six months of listening to Mozart music (Sonata K.448). The first step is to present the data of the first patient under investigation.

Effectiveness of serotonergic drugs in the management of problem behaviors in patients with neurodevelopmental disorders.

Neurodevelopmental disorders often result in disabilities associated with auto- and / or hetero-aggressive behaviors, that can be defined as "problem behaviors" (Lacy 2007). Therapeutic interventions are mainly directed towards the use of neuroleptic drugs or benzodiazepines, to ensure a rapid and significant sedation in most of cases. These pharmacological devices exposes the patient to clinical risks and/or long-term management difficulties. The main problem of the chronic use of benzodiazepines is the development of tolerance and dependence; furthermore benzodiazepine withdrawal or their abrupt reduction may lead to rebound effect. Regarding the long-term effects of neuroleptics, it is necessary to focus on extrapyramidal effects, motor restlessness and akathisia, anticholinergic effects, as well as endocrine and metabolic alterations. Several studies have shown that the reduction of serotonergic receptor activity is associated with the appearance of aggressive behavior (Farnam et al. 2017), especially impulsive behaviors (Manchia et al. 2017, Takahashi et al. 2012). The dynamics that subtend these data are still not fully clarified, however there are evidences that the use of selective serotonin reuptake inhibitors (SSRI) is helpful in the treatment of aggressive behavior in mental disabilities (Sterke et al. 2012, Janowsky et al. 2015). In this study we observe the behavioral response to sertraline, for minors, and to vortioxetine, for adults, considering that the literature shows significant evidence of modulation of synaptic neuroplasticity (Waller et al. 2017). To support the observation we used behavioural scales to collect the data, before the administration of the drug, during the course of treatment, at 3 months from the start of the administration. We detected the improve of behavioral disorders with the less use neuroleptic drugs and benzodiazepines.

Mozart's music and multidrug-resistant epilepsy: a potential EEG index of therapeutic effectiveness.

Multidrug-resistant epilepsy is a pathological condition that affects approximately one-third of patients with epilepsy, especially those with associated intellectual disabilities. Several non-pharmacological interventions have been proposed to improve quality of life of these patients. In particular, Mozart's sonata for two pianos in D major, K448, has been shown to decrease interictal electroencephalography (EEG) discharges and recurrence of clinical seizures in these patients. In a previous study we observed that in institutionalized subjects with severe/profound intellectual disability and drug-resistant epilepsy, a systematic music listening protocol reduced the frequency of seizures in about 50% of cases. This study aims to assess electroencephalography as a quantitative (qEEG) predictive biomarker of effectiveness of listening to music on the frequency of epileptic discharges and on background rhythm frequency (BRF).

Effects of music on seizure frequency in institutionalized subjects with severe/profound intellectual disability and drug-resistant epilepsy.

BACKGROUND: Approximately one-third of patients with epilepsy continue to experience seizures despite adequate therapy with antiepileptic drugs. Drug-resistant epilepsy is even more frequent in subjects with intellectual disability. As a result, several non-pharmacological interventions have been proposed to improve quality of life in patients with intellectual disability and drug-resistant epilepsy. A number of studies have demonstrated that music can be effective at reducing seizures and epileptiform discharges. In particular, Mozart's sonata for two pianos in D major, K448, has been shown to decrease interictal EEG discharges and recurrence of clinical seizures in patients with intellectual disability and drug-resistant epilepsy as well. The aim of this study is to investigate the influence of Mozart's music on seizure frequency in institutionalized epileptic subjects with profound/severe intellectual disability. SUBJECTS AND METHODS: Twelve patients (10 males and 2 females) with a mean age of 21.6 years were randomly assigned to two groups in a cross-over design; they listened to Mozart K448 once a day for six months. RESULTS: A statistically significant difference was observed between the listening period and both baseline and control periods. During the music period, none of the patients worsened in seizure frequency; one patient was seizure-free, five had a greater than 50% reduction in seizure frequency and the remaining showed minimal (N=2) or no difference (N=4). The average seizure reduction compared to the baseline was 20.5%. Our results are discussed in relation to data in the literature considering differences in protocol investigation. CONCLUSIONS: Music may be considered a useful approach as add-on therapy in some subjects with profound intellectual disability and drug-resistant epilepsy and can provide a new option for clinicians to consider, but further large sample, multicenter studies are needed to better understand the characteristics of responders and non-responders to this type of non-pharmacological intervention.

Impairment of homonymous processing in Alzheimer's disease.

An important issue in research on language is how concepts are represented and associated with each other in the brain. Many investigations have focused on language ambiguity and the phenomenon of homonymy in which a single lexical item, presenting the same form, is related to different meanings. Our study aims to test the hypothesis that weak association of meaning characterizing homonyms may be especially prone to brain damage. To verify this hypothesis a test of attribution of the meaning of homonymous words, the Humpty Dumpty (HD) test, was applied to 50 patients with Alzheimer's disease (AD) and 50 healthy subjects. Results show that AD patients are impaired in the HD test in an early phase of disease and that performance correlates with naming ability and phonological fluency. The data are in keeping with a growing body of literature that supports dual impairment to the semantic system in AD, i.e., to semantic knowledge and active processing and access to the semantic field. The evaluation of the ability to resolve homonymous ambiguity, using the HD test, may provide a useful and quick clinical tool to detect the anomalies of the semantic network linked to either a loss of the core system where meaning of words is stored or an impairment of the access to an intact semantic representation.

Assessment of math anxiety as a potential tool to identify students at risk of poor acquisition of new math skills: longitudinal study of grade 9 Italian students.

Introduction: Numerous international educational institutions have sounded the alarm about the gradual increase in the number of students failing to achieve a sufficient level of proficiency in mathematical abilities. Thus, the growing interest in identifying possible solutions and factors interfering with learning seems justified. In recent years, special attention has accrued to the possible role played by emotional factors. Methods: In the present investigation, students in the first grade of a technical vocational secondary school are followed to assess the influence of math anxiety (MA) on the development of skill acquisition in calculus. A math skills assessment test is administered on two occasions, at the beginning and end of the school year. Results: Results highlighted that the score on the anxiety scale, administered at the beginning of the year, negatively correlated with the score obtained on the mathematics test, administered at the end of the school year: the higher the level of anxiety, the worse the performance. Furthermore, the score obtained in the second administration makes it possible to divide the students tested into two groups: students who improved their performance and students who did not benefit at all from repeating the test. In these two groups, an analysis of the relationships between the outcome of the end-of-year mathematics test and the level of MA at the beginning of the year showed that MA correlates negatively with performance only in students who will fail to acquire new expertise in mathematics over the course of the school year. Discussion: The results suggest that MA may interfere with the smooth development of math skills. Assessing the level of MA at the beginning of the school year could prove to be a useful tool in identifying which and how many students are at risk of failing to achieve the skills expected from the usual course of instruction. A consideration of anxiety as one of the variables at play in the genesis of learning difficulties may prompt educators to modify teaching methodology and strategies by increasing focus on the impact of the emotional dimension on learning.

Functional neuroimaging in specific phobia.

Specific phobias (SPs) are common, with lifetime prevalence estimates of 10%. Our current understanding of their pathophysiology owes much to neuroimaging studies, which enabled us to construct increasingly efficient models of the underlying neurocircuitry. We provide an updated, comprehensive review and analyze the relevant literature of functional neuroimaging studies in specific phobias. Findings are presented according to the functional neuroanatomy of patients with SPs. We performed a careful search of the major medical and psychological databases by crossing SP with each neuroimaging technique. Functional neuroimaging, mostly using symptom provocation paradigms, showed abnormal activations in brain areas involved in emotional perception and early amplification, mainly the amygdala, anterior cingulate cortex, thalamus, and insula. The insula, thalamus and other limbic/paralimbic structures are particularly involved in SPs with prominent autonomic arousal. Emotional modulation is also impaired after exposure to phobic stimuli, with abnormal activations reported for the prefrontal, orbitofrontal and visual cortices. Other cortices and the cerebellum also appear to be involved in the pathophysiology of this disorder. Functional neuroimaging identified neural substrates that differentiate SPs from other anxiety disorders and separate SP subtypes from one another; the results support current Diagnostic and Statistical Manual of Mental Disorders, 4th edition-Text Revision (DSM-IV-TR) diagnostic subtyping of SPs. Functional neuroimaging shows promise as a means of identifying treatment-response predictors. Improvement in these techniques may help in clarifying the neurocircuitry underlying SP, for both research and clinical-therapeutic purposes.

Non-verbal memory measured by Rey-Osterrieth Complex Figure B: normative data.

One of the major problems that clinical neuropsychology has had in memory clinics is to apply ecological, easily administrable and sensitive tests that can make the diagnosis of dementia both precocious and reliable. Often the choice of the best neuropsychological test is hard because of a number of variables that can influence a subject's performance. In this regard, tests originally devised to investigate cognitive functions in healthy adults are not often appropriate to analyze cognitive performance in old subjects with low education because of their intrinsically complex nature. In the present paper, we present normative values for the Rey-Osterrieth Complex Figure B Test (ROCF-B) a simple test that explores constructional praxis and visuospatial memory. We collected normative data of copy, immediate and delayed recall of the ROCF-B in a group of 346 normal Italian subjects above 40 years. A multiple regression analysis was performed to evaluate the potential effect of age, sex, and education on the three tasks administered to the subjects. Age and education had a significant effect on copying, immediate recall, and delayed recall as well as on the rate of forgetting. Correction grids and equivalent scores with cut-off values relative to each task are available. The availability of normative values can make the ROCF-B a valid instrument to assess non-verbal memory in adults and in the elderly for whom the commonly used ROCF-A is too demanding.

Age-related decline in interhemispheric transfer of tactile information: The fingertip cross-localization task.

According to the disconnection hypothesis of cognitive aging, cognitive deficits associated with brain aging could be a result of damage to connective fibres. It has been suggested that the age-related decline in cognitive abilities is accompanied by age-related changes in interhemispheric communication ensured by commissural fibres. This study aimed to contribute to this topic by investigating the effects of aging on the efficiency of interhemispheric transfer of tactile information. A total of 168 right-handed subjects, aged 20-90 years, have been tested using the fingertip cross-localization task: the subject must respond to a tactile stimulus presented to one hand using the ipsilateral (uncrossed condition) or contralateral hand (crossed condition). Because the crossed task requires interhemispheric transfer of information, the value of the difference between the uncrossed and crossed conditions (CUD) can be deemed to be a reliable measure of the efficiency of the interhemispheric interactions. The uncrossed condition was more accurate than the crossed condition for all ages. However, the degree of the CUD was significantly age-dependent. The effectiveness of the interhemispheric transfer of tactile information decreased significantly with age and may indicate the occurrence of age-related changes of the corpus callosum. Considerably, performance appears to decline around the seventh decade of life with the fastest decline in the subsequent decades. The results suggest a relationship between brain aging and the efficiency of the interhemispheric transfer of tactile information. The findings are discussed in relation to the strategic role of white matter integrity in preserving behavioural performances.

Improvement in cognitive performance and mood in healthy older adults: a multimodal approach.

The aim of this research was to evaluate if being involved in a programme that integrates physical, mental, and social activities could help to reduce the impacts of cerebral ageing on cognitive functions. Fifty healthy adults over 65 years of age and without cognitive impairment were randomly assigned to either an experimental or a control group; subjects were equally divided by age, sex, schooling, physical health, mood, and social integration. For 6 months, the experimental group had biweekly meetings, participating in a multimodal approach based on a combination of simultaneous physical, mental, and social activities. At pre-test and post-test, both experimental and control subjects underwent a neuropsychological assessment, including tests to measure attention, verbal and spatial memory, language, constructional praxis, executive functions, processing speed, and intelligence. Furthermore, a visual analogue scale was used to examine well-being and mood states. Compared to the pre-test levels and the control subjects, the performance of subjects in the experimental group significantly improved in several neuropsychological tests, including attention, processing speed, memory, and executive functions, as well as mood state. Even in older subjects without cognitive impairments, a multimodal approach based on simultaneous physical, mental, and social activity can be a useful strategy that has beneficial effects on mood and cognition. The results suggest that an active lifestyle may protect against cognitive decline in ageing.

Slowly progressive pure dysgraphia with late apraxia of speech: a further variant of the focal cerebral degeneration.

We report a longitudinal neuropsychological investigation of a patient with slowly progressive pure dysgraphia. Cognitive analysis of writing errors suggested a selective impairment of the graphemic buffer. After about seven years, the patient developed an apraxia of speech. No other linguistic or generalized cognitive impairment occurred subsequently, so that, twelve years after the beginning of the disease, the patient showed complete independence in daily life and still remained professionally active. Functional neuroimaging revealed hypoperfusion confined to left fronto-temporal lobe. This well-recognizable syndrome does not fit any of the cases described previously in the literature. This report therefore, adds another variant to heterogeneous clinical spectrum of focal neurodegenerative disorders, further suggesting the opportunity of their distinction from pathological processes leading to dementia.

Brief report: functional MRI of a patient with 7q11.23 duplication syndrome and autism spectrum disorder.

The duplication of the Williams-Beuren syndrome (WBS) region (7q11.23) is a copy number variant associated with autism spectrum disorder (ASD). One of the most intriguing aspects is that the reciprocal microdeletion causes WBS, characterized by hypersociability, marked empathy, and a relative capacity in verbal short-term memory and language. Herein, we studied, by using functional morphological and volumetric magnetic resonance, a 17-year-old male patient who displays a de novo 7q11.23 duplication and ASD. The limbic system of the patient appeared hypo-functional, while the total brain volume was increased, thus contrasting, in an opposite and intriguing manner, with the global brain volume reduction reported in WBS. Even if these findings come from the analysis of a single patient and, therefore, have to be considered preliminary results, they encourage carrying on further functional and volumetric studies in patients with 7q11.23 duplication, to fully elucidate the role of this gene-dosage alteration on brain development and limbic system function.