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Background: Spina bifida (SB) is a rare congenital disease characterized by not only physical but also neuropsychological disturbances. Among these neuropsychological impairments, memory deficits are a significant concern, as they substantially hinder aspects of crucial importance in the lives of individuals with SB such as medical needs or daily life activities. The main objective is to conduct a systematic review of the current evidence on the memory deficits in the SB population, including children, adolescents, and adults. Methods: Four databases (PubMed, SCOPUS, Web of Science, and ProQuest) were systematically screened for eligible studies. Results: The present review reveals cognitive difficulties in different memory types among individuals with SB. These deficits, identified in childhood, seem to persist into adulthood. Specifically, impairments are evident in short-term memory, working memory, and long-term memory. The neuropsychological instruments applied in the studies that were included in this systematic review vary, however, most reach the same conclusions. Conclusions: The present findings underscore the importance of incorporating cognitive assessments, particularly those focused on the memory domain, into routine childhood evaluations for individuals with SB. Early identification of these cognitive difficulties allows for the timely implementation of cognitive interventions that could leverage the inherent plasticity of the developing brain, and prevent or delay the onset of these deficits in later adulthood for people with SB, ultimately improving their functionality and quality of life.

Chiari Malformation type I (CM-I) is a neurological disorder characterized by cerebellar tonsillar herniation. Chronic pain, particularly headaches, is a prevalent symptom in CM-I patients, significantly impacting their quality of life. The objective of this study was to evaluate the perceived quality of life in adults with CM-I and examine the influence of chronic pain and comorbid symptoms on their well-being. 26 CM-I patients (8 with decompressive surgery) and 26 matched healthy controls were recruited. Participants completed the following questionnaires: WHOQOL-BREF, HDI, NDI, OLBPDQ and HADS. CM-I patients exhibited significantly lower scores across all domains of quality of life when compared to healthy controls. Chronic pain, including headache, neck pain, and low back pain, was more pronounced among CM-I patients and demonstrated a significant correlation with depressive symptoms. Notably, after controlling for chronic pain, the differences in quality of life between CM-I patients and controls diminished. The results suggest that chronic pain, especially headaches, and comorbid depressive symptoms exert a substantial impact on the quality of life of CM-I patients. Surgical intervention alone may not fully address these issues, highlighting the importance of considering psychological interventions as part of the comprehensive treatment. Further research with larger samples and pre-post-surgery assessments is needed to validate these findings and explore the potential benefits of psychological therapies in enhancing the quality of life for CM-I patients., Competing Interests: The authors have no conflicts of interest to disclose., (2024, International Research and Cooperation Association for Bio & Socio - Sciences Advancement.)

Myasthenia gravis (MG) is a neuromuscular disease of autoimmune etiology and chronic evolution. In addition to the muscle weakness and fatigue that characterize MG, in some studies patients show an inferior performance in cognitive tasks and difficulties in recognizing basic emotions from facial expressions. However, it remains unclear if these difficulties are due to anxious-depressive symptoms that these patients present or related to cognitive abilities, such as facial recognition. This study had a descriptive cross-sectional design with a sample of 92 participants, 52 patients with MG and 40 healthy controls. The data collection protocol included measures to assess recognition of facial expressions (BRFT), facial emotional expression (FEEL), and levels of anxiety and depression (HADS). The MG group had worse performance than the control group in recognizing "fear" ( p = 0.001; r = 0.344), "happiness" ( p = 0.000; r = 0.580), "disgust" ( p = 0.000; r = 0.399), "surprise" ( p = 0.000; r = 0.602), and "anger" ( p = 0.007; r = 0.284). Likewise, the MG group also underperformed in facial recognition ( p = 0.001; r = 0.338). These difficulties were not related to their levels of anxiety and depression. Alterations were observed both in the recognition of facial emotions and in facial recognition, without being mediated by emotional variables. These difficulties can influence the interpersonal interaction of patients with MG.

Objective: This study aimed to determine the effects of a neuropsychosocial teleassistance group-based intervention on improving social cognitive functioning and health-related quality of life (HRQoL) in pediatric neuromuscular diseases (NMD)., Methods: Thirty-five pediatric patients with NMD were assigned to the neuropsychosocial intervention program (n = 20) or waiting list control condition (n = 15). The intervention group received an integrative approach that combines training in social cognition with cognitive behavioral therapy. All participants completed a neuropsychological and clinical assessment at baseline and follow-up, which included tests of social cognition, both for emotion recognition and theory of mind, and HRQoL. Repeated-measures multivariate analysis of covariance was used to determine the effects of the teleassistance program., Results: Group × Time interactions revealed significant improvements in the intervention group as compared with the control group for different social cognition's indicators (AR NEPSY-II: p = .003, η2p = .24; TM NEPSY: p < .001, η2p = .35; Verbal task: p < .001, η2p = .35; Happé's Strange Stories: p = .049, η2p = .11) and HRQoL (Psychosocial health: p = .012, η2p = .18; Emotional functioning: p = .037, η2p = 0.13; Social functioning: p = .006, η2p = .21; Total: p = .013, η2p = .17), showing medium to large effects., Conclusions: Patients receiving the neuropsychosocial intervention showed improvements in their social cognition performance and psychosocial HRQoL, providing evidence about the positive effects of the program in pediatric patients with NMD. This should be considered in further research and interventions in this field., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Introduction: Informal caregivers of elderly people with dependency (EPD) provide intensive care that can affect their quality of life (QoL). Psychosocial interventions such as music therapy are important to work on their self-care. The aim of this study is to analyze, with a mixed method approach, the experience of participating in a Songwriting Group Music Therapy (SGMT) intervention on informal caregivers of EPD., Methods: A total of 11 groups, with a convenience sample of 61 caregivers, received 10 SGMT sessions. Quantitative information related to QoL variables (anxiety, depression, spirituality, burden, and coping) was collected before and after the intervention and at 3 months of follow-up. Regarding qualitative data, an open-ended question about the experience of participating was asked., Results: Significant changes were shown, sustained over time, in trait anxiety and depression and subscales including inner peace, social functioning, and mental health. Three themes were generated from the thematic analysis, including that SGMT participation can enhance personal growth, bring out and enable work on emotions, and promote helpful interpersonal dynamics., Discussion: The findings indicate that SGMT is a useful intervention for informal caregivers of EPD, promoting psychological adjustment, enhanced coping, emotional regulation, and social support. This study reinforces the findings with caregivers of other populations, providing new results and highlighting the benefits of SGMT for caregivers of EPD., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Pérez-Núñez, O’Callaghan, López-Paz, Ruiz de Lazcano, Rodríguez and Amayra.)

Introduction: Pediatric muscular dystrophies (MDs) are a heterogeneous group of rare neuromuscular diseases characterized by progressive muscle degeneration. A neuropsychosocial approach is crucial for these patients due to associated cognitive, behavioral, and psychiatric comorbidities; however, the social cognitive domain has not been adequately addressed., Methods: This study aimed to analyze on social cognition performance in a pediatric MD patient cohort. This cross-sectional study included 32 pediatric patients with MD and 32 matched-healthy controls. The Social Perception Domain of the NEPSY-II, the Reading the Mind in the Eyes Test-Child and Happé's Strange Stories Test were administered. General intelligence and behavioral and emotional symptoms were controlled for to eliminate covariables' possible influence. The assessments were performed remotely., Results: Children with MDs performed significantly worse on most of the social cognition tasks. The differences found between the groups could be explained by the level of general intelligence for some aspects more related to theory of mind (ToM) (TM NEPSY-II: F  = 1.703, p  = .197; Verbal task: F  = 2.411, p  = .125; RMET-C: F  = 2.899, p  = .094), but not for emotion recognition. Furthermore, these differences were also independent of behavioral and emotional symptoms., Discussion: In conclusion, social cognition is apparently impaired in pediatric patients with MD, both for emotion recognition and ToM. Screening assessment in social cognition should be considered to promote early interventions aimed at improving these patient's quality of life., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 García, Martínez, López-Paz, García, Rodríguez and Amayra.)

Objective : The dystrophinopathies called Duchenne and Becker muscular dystrophies (DMD/BMD) are rare, progressive, incurable, and life-limiting paediatric-onset neuromuscular diseases. These diseases have long been associated with specific neuropsychological deficits. However, the performance of these patients in the social cognition domain has not been properly investigated. Thus, the main objective of this study was to compare the performance on social cognition between DMD/BMD patients and healthy age-matched boys. Method : This cross-sectional study included 20 DMD/BMD children and adolescents and 20 healthy controls. The protocol included the Social Perception Domain of the NEPSY-II, the Reading the Mind in the Eyes Test - Child and Happé's Strange Stories test. General intelligence was controlled to eliminate the possible influence of covariables. All the assessments were performed remotely. Results: Most social cognition tasks were worse in patients with DMD/BMD than in matched healthy controls. These differences remained even after controlling for the general intelligence variable, with the exception of Total Disgust Errors ( F  = 1.462, p = .234, η 2 p = .038) and Verbal task ( F  = 1.820, p = .185, η 2 p = .047) scores from the NEPSY-II. Conclusions : This is the first study to demonstrate that the neuropsychological domain of social cognition is impaired in DMD/BMD patients, independent of the level of general intelligence. Screening assessments in DMD/BMD patients should be promoted to allow social cognition difficulties to be detected at an early stage to enhance patients' quality of life and social development.

Chiari malformation has been classified as a group of posterior cranial fossa disorders characterized by hindbrain herniation. Chiari malformation type I (CM-I) is the most common subtype, ranging from asymptomatic patients to those with severe disorders. Research about clinical manifestations or medical treatments is still growing, but cognitive functioning has been less explored. The aim of this systematic review is to update the literature search about cognitive deficits in CM-I patients. A literature search was performed through the following electronic databases: MEDLINE, PsychINFO, Pubmed, Cochrane Library, Scopus, and Web of Science. The date last searched was February 1, 2023. The inclusion criteria were as follows: (a) include pediatric or adult participants with a CM-I diagnosis, (b) include cognitive or neuropsychological assessment with standardized tests, (c) be published in English or Spanish, and (d) be empirical studies. Articles that did not report empirical data, textbooks and conference abstracts were excluded. After the screening, twenty-eight articles were included in this systematic review. From those, twenty-one articles were focused on adult samples and seven included pediatric patients. There is a great heterogeneity in the recruited samples, followed methodology and administered neurocognitive protocols. Cognitive functioning appears to be affected in CM-I patients, at least some aspects of attention, executive functions, visuospatial abilities, episodic memory, or processing speed. However, these results require careful interpretation due to the methodological limitations of the studies. Although it is difficult to draw a clear profile of cognitive deficits related to CM-I, the literature suggests that cognitive dysfunction may be a symptom of CM-I. This suggest that clinicians should include cognitive assessment in their diagnostic procedures used for CM-I. In summary, further research is needed to determine a well-defined cognitive profile related to CM-I, favoring a multidisciplinary approach of this disorder., (© 2023. The Author(s).)

The aim of this study was to compare the effects of the pandemic on health-related quality of life (HRQoL), anxious-depressive symptoms, feelings of loneliness, and fear of COVID-19 between people with myasthenia gravis (MG) and healthy controls. We also wanted to know in which group the variable fear of COVID-19 interfered the most with the results. This cross-sectional study involved 60 people with MG and 60 healthy controls. Participants using an online platform completed a sociodemographic questionnaire, the Short Form-36 Health Survey (SF-36), the Hospital Anxiety and Depression Scale (HADS), the revised UCLA Loneliness Scale and the Fear of COVID19 Scale (FCV- 19S). The MG group reported worse levels in HRQoL indicators ( p = 0.043- <.001), more severe anxiety-depressive symptoms ( p = 0.002), and greater fear of COVID-19 ( p < 0.001), but there were no differences in feelings of loneliness ( p = 0.002). Furthermore, after controlling for the effect of the fear of COVID-19 variable, the differences remained for physical health indicators, but not for the most of psychosocial indicators (Social Functioning p = 0.102, η 2 p = 0.023; Role Emotional p = 0.250, η 2 p = 0.011; and HADS Total p = 0.161, η 2 p = 0.017). The harmful effect of the COVID-19 pandemic was greater in the MG group, and the perceived fear of COVID-19 had also a greater impact among this group, which has increased its negative effect on their psychosocial health., Competing Interests: The authors have no conflicts of interest to disclose., (2023, International Research and Cooperation Association for Bio & Socio - Sciences Advancement.)

Caregivers' emotions and finances are affected by the deterioration of functional capacity of patients with Duchenne muscular dystrophy (DMD), both in Mexico and Spain. Patient associations may reduce this impact on caregivers. This study aims to study the role of two models of associations, inspired by two different cultural models, in how the services they provide can help decrease the emotional and financial impact on the caregivers of children with DMD. The sample consisted of 34 caregivers from Mexico and 40 from Spain recruited from Spanish hospitals and rare disease organizations in Spain and Mexico. The instruments used consisted of a sociodemographic and socioeconomic questionnaire, the CarerQol-7D, the PHQ-15, the Zarit Caregiver's Burden Scale and the SWLS. The results showed that caregivers in Mexico are in better physical and psychological health than caregivers in Spain. They also receive more subsidies than those in Spain. Caregivers in Mexico have a greater well-being and are less affected by the economic impact of the disease due to the associations' day-to-day work and the fact that they generate a network of health services that they make available to the patient free of charge. These differences may also be attributable to cultural issues and to the fact that Mexico has a deeply established culture of support.

Hereditary ataxias are a heterogeneous group of disorders characterized by degeneration of the cerebellum and its connections. It is known that patients with ataxia can manifest a broad spectrum of motor symptoms; however, current research has emphasized the relevance of cognitive disturbances. The aim of this study is to analyze the presence of cognitive impairment in a heterogeneous cohort of patients with hereditary ataxia (HA). A group of 38 patients with HA of different etiologies and a matched group of 38 healthy controls were recruited and evaluated through a comprehensive battery of neuropsychological tests. The findings show a worse performance in ataxic patients on planning, visuospatial skills, naming, and Theory of Mind tasks, regardless their physical and psychological symptomatology. The influence of clinical status as well as functional ability-related variables on their performance were analyzed, showing that the level of disability and motor disturbances have a significant effect on verbal memory, verbal fluency, and working memory. These findings suggest that patients with HA can manifest cognitive and neuropsychiatric symptoms as part of their clinical features, which demands its inclusion for the diagnosis and management of the disease., (© The Author(s) 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

About 30% of patients with amyotrophic lateral sclerosis (ALS) suffer from cognitive impairment and 10-15% suffer from frontotemporal dementia (FTD). Due to the patients' reduced motor function, a neuropsychological assessment with a low motor demand can be an advantage when evaluating patients, aiding its application. The present work has studied the usefulness of the Faces subtest of the Wechsler Memory Scale in 42 patients with ALS and 42 healthy volunteers applying Holdnack's Two-High Threshold model. The ALS group performed significantly worse in immediate and delayed discriminability. With respect to the presence of cognitive impairment, it seemed to be independent of the indexes proposed by Holdnack and Delis. On the other hand, motor problems were associated with delayed recognition while behavior alterations were linked to problems with delayed discriminability. ALS patients do not express differences in the bias index, in line with other types of previously studied pathologies.

This study aims to evaluate the costs of informal care for children with neuromuscular disease and evaluate how physical and psychological health is associated with socio-demographic variables. A cross sectional design was used with a convenience sample of 110 carers that participated in this study. Participants were recruited from Spanish hospitals and rare diseases organizations. Economic costs and sociodemographic aspects were assessed using the economic costs questionnaire and the sociodemographic questionnaire. Physical and psychological health was evaluated using the CarerQol-7D, PHQ-15, Barthel Index, Zarit Overload Scale and Satisfaction with Life Scale. Carers of children with neuromuscular disease spent a large percentage of their annual income in physical therapy, psychological care and speech therapy. Informal costs differed according to the degree of dependency of the child. These were higher in those caregivers whose child under their care presented low functional independence. The loss of work productivity was related to marital status, use of professional services and the child's dependency. Finally, carers who were female, single or separated and without a job showed worse physical and psychological health. The results highlighted that carers have to face a number of high costs because of the non-existence of social protection and due to the child's diagnosis.

Rare neuromuscular diseases (RNMDs) are a group of pathologies characterized by a progressive loss of muscular strength, atrophy, fatigue, and other muscle-related symptoms, which affect quality of life (QoL) levels. The low prevalence, high geographical dispersion and disability of these individuals involve difficulties in accessing health and social care services. Teleassistance is presented as a useful tool to perform psychosocial interventions in these situations. The main aim of this research is to assess the effects of a teleassistance psychosocial program on the QoL levels of people with RNMDs who have different levels of disability. A sample of 73 participants was divided into an experimental group ( n = 40), which participated in the intervention, and a control wait list group ( n = 33). QoL was evaluated through the SIP and the SF-36, and disability through the WHO-DAS II. The participants with a moderate to severe level of disability were those who most benefited from the intervention. The results also revealed that the psychosocial teleassistance program was suitable to improve physical and psychosocial aspects of people suffering from a rare neuromuscular disease with a moderate level of disability, but just psychosocial aspects in those with a severe level of disability., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Martínez, Amayra, López-Paz, Lázaro, Caballero, García, Rodríguez, García, Luna, Pérez-Núñez, Barrera, Passi, Berrocoso, Pérez and Al-Rashaida.)

Background: Wolf-Hirschhorn Syndrome (WHS) is a rare, congenital disease characterized by a distinctive facial phenotype, seizures, intellectual disability and developmental delay, and pre and postnatal growth requiring lifelong care. The psychosocial status of the family caregivers of children diagnosed with WHS is unknown. This study aims to characterize the sociodemographic and psychosocial profile of WHS caregivers and analyze how these variables impact their quality of life (QoL) and well-being., Results: The sociodemographic and clinical profile of 22 Spanish caregivers of children with WHS and the characteristics of those affected have been described. Significant relationships were found between sociodemographic and psychosocial characteristics among caregivers. The impact on the parents' QoL and negative relationship with the symptomatology were assessed. The use of engagement strategies such as problem focused coping was associated with improved psychological QoL and social support., Conclusions: WHS caregivers share similarities in their profile and needs with caregivers of children with other rare diseases. Pychosocial support groups involving parents caring for children with the same disease could improve caregivers' well-being and QoL by strengthening their social support network and using positive coping styles.

Chiari malformation type I (CM-I) is a neurological disorder in which cerebellar tonsils are herniated through the foramen magnum into the spinal canal. A wide spectrum of cognitive deficits underlying this pathology has been reported, but the literature about social cognition is insufficient. Clinical research has pointed out the cerebellar role in Theory of Mind (ToM), indicating that there are several disorders with cerebellar pathology that reveal a poorer performance in social cognition tasks. The main purpose of this study is to compare the performance on ToM tasks between CM-I patients and healthy controls. The protocol includes Faux Pas test, Happé's Strange Stories test, Ice-Cream Van task, the FEEL test, and the Word Accentuation Test. In order to eliminate the possible influence of covariables, physical pain and anxious-depressive symptomatology have been controlled for. According to the results, CM-I patients performed worse than matched healthy controls on ToM tasks, except for facial emotion recognition. These differences remained even after controlling for the neuropsychiatric variables and physical pain. Thus, it can be suggested that patients with CM-I are impaired in their social skills related to their performance on ToM tasks. These findings can be considered to be a preliminary approach to the specific study of social cognition in relation to CM-I since it is similar to other cerebellar pathologies and to previous literature on the cerebellum's role in social cognition.

Background: Numerous studies have noted the presence of a dysexecutive component of the ALS-FTD. The most widely replicated result refers to the significantly reduced verbal fluency of ALS patients when compared to healthy people. As ALS patients have motor alterations that interfere with production, qualitative studies have the advantage of being independent of the degree of motor disability and revealing patients' cognitive state. This study examined the production differences between 42 ALS patients who presented with different degrees of dementia and motor impairment and 42 healthy people. Production processes were studied by extending the administration time of a letter fluency task to 2 minutes for the phonemic verbal fluency (PVF) and semantic verbal fluency (SVF) categories. This ensured that the qualitative aspects of verbal fluency were addressed, paying special attention to the new perseverations and intrusions, as well as any clinical correlates that may exist., Results: The ALS patients produced a significantly lower number of responses in PVF (p = .017) and SVF (p = .008). The rest of the indicators for frontal lobe alteration also suggested the existence of a dysfunction. The most remarkable results were the number of intrusions on the PVF task, which was much higher in the ALS group (p = .002). However, the number of perseverations did not differ significantly., Conclusions: This study highlights the value of intrusions in addressing cognitive deterioration in ALS patients. This deterioration seems to be independent of the degree of motor impairment and of behavioural alterations. Therefore, the value of the intromissions on the verbal fluency task was highlighted as an indicator of a new cognitive alteration, which can be easily evaluated, even retrospectively., Competing Interests: The authors have declared that no competing interests exist.

Background: Given the interactive media characteristics and intrinsically motivating appeal, virtual serious games are often praised for their potential for assessment and treatment., Objective: This study aims to validate and develop normative data for a virtual serious game (Deusto-e-motion1.0) for the evaluation of emotional facial expression recognition and social skills, both of which are components of the theory of mind., Methods: A total of 1236 children took part in the study. The children were classified by age (8-12 years old), gender (males=639, females=597), and educational level (between the third and sixth years of Primary Education). A total of 10 schools from the Basque Country and 20 trained evaluators participated in this study., Results: Differences were found in Deusto-e-motion1.0 scores between groups of children depending on age and gender. Moreover, there was a moderately significant correlation between the emotional recognition scores of Deusto-e-motion1.0 and those of the Feel facial recognition test., Conclusions: Deusto-e-motion1.0 shows concurrent validity with instruments that assess emotional recognition. Results support the adequacy of Deusto-e-motion1.0 in assessing components of the theory of mind in children., (©Esther Lázaro, Imanol Amayra, Juan Francisco López-Paz, Oscar Martínez, Manuel Pérez Alvarez, Sarah Berrocoso, Mohammad Al-Rashaida, Maitane García, Paula Luna, Paula Pérez-Núñez, Alicia Aurora Rodriguez, Paula Fernández, Pamela Parada Fernández, Mireia Oliva-Macías. Originally published in JMIR Serious Games (http://games.jmir.org), 02.04.2020.)

The involvement of the cerebellum in visuospatial abilities has been evidenced in numerous studies, based on the cerebellar-cortical circuitry. This domain has been evaluated in several patients with cerebellar disorders, but the assessment of visuospatial processing in Chiari malformation type I (CM-I) is scarce. The aim of this study is to analyze the visuospatial performance between CM-I adult patients and healthy controls. Participants have been tested using Block Design and Visual Puzzles subtests of the Wechsler Adult Intelligence Scale (WAIS), the Benton Judgment of Line Orientation test, and the Rey-Osterrieth Complex Figure test. The anxious-depressive symptomatology, the physical pain, and the premorbid intelligence have been controlled for, as well. The CM-I patients showed a significantly lower performance; however, after analyzing and controlling for the effect of clinical variables and psychopathological symptomatology, the main effect was maintained for visual puzzles and line orientation tasks. The findings suggest that CM-I patients show a poorer performance in tasks that require an exercise of perceptual reasoning without motor demand, accompanied by visualization and mental imagery of the stimuli. This study contributes towards the reinforcement of the evidence on the cognitive alterations associated to CM-I.

This study aimed to create a valid and reliable instrument to measure people with disabilities' perceptions regarding the extent of application of international standards issued by the Council of Europe, International Labour Organization, and the International Classification of Functioning, Disability and Health in vocational rehabilitation centers in the Basque country, Spain. The instrument items were selected from international recommendations and conventions issued by the International Labour Organization, Council of Europe, International Classification of Functioning, Disability and Health, and a vocational rehabilitation literature review. The instrument was translated from English to Spanish, and the content validity index of an expert panel survey was used to assess content validity. For the Spanish version of the instrument, internal consistency reliability, confirmatory factor analysis and factor analysis were examined with 186 people with disabilities in 10 vocational rehabilitation centers in the Basque country. The coefficient alpha estimate was 0.945, indicating excellent internal consistency. Three factors were identified: job-related services, aims of vocational rehabilitation center, and vocational rehabilitation guidance. The instrument appears to have good validity and reliability but requires further validation. Support is provided for its use in both English and Spanish. Implications for vocational rehabilitation practice and suggestions for future research are provided.

Objective: This study presents the validation of a computerized assessment tool that studies the ability to recognize emotional facial expressions in children between 8 and 11 years of age: the Facially Expressed Emotion Labeling Test (FEEL Test)., Method: The two tests composing the protocol were applied using a laptop in the following order: the FEEL Test followed by the Deusto-e-Motion 1.0 Test.The sample consisted of a total of 1,189 schoolchildren aged between 8 and 11 years, 594 boys and 594 girls. A clinical sample of 47 children with ADHD also took part in this study., Results: The Cronbach's α coefficient for the total scale was .82, showing high levels of reliability. The difficulty index of the items ranged between .4 and .7. The statistical analyses showed a high rate of discrimination between those who obtained low scores compared with those who obtained high scores. The test results reflected differences according to age and gender of participants in many of the variables associated with both response accuracy and response speed. Regarding its predictive validity, the test is able to find statistically significant differences in the total test score among a group of children diagnosed with ADHD and a matched control group., Conclusion: This article presents the validation of an instrument that assesses the ability to recognize facial expressions in children between 8 and 11 years old and can discriminate and detect differences in gender, age, and possible deficits in social skills within the ADHD.

Background: Stroke is the most common neurological disease in the world. After the stroke, some people suffer a cognitive disability. Commercial videogames have been used after stroke for physical rehabilitation; however, their use in cognitive rehabilitation has hardly been studied. The objectives of this study were to analyze attention, processing speed, and working memory in patients with moderate stroke after an intervention with Wii Sports Resort and compared these results with a control group., Methods: A pre-post design study was conducted with 30 moderate stroke patients aged 65 ± 15. The study lasted eight weeks. 15 participated in the intervention group and 15 belong to the control group. They were assessed in attention and processing speed (TMT-A and B) and working memory (Digit Span of WAIS-III). Parametric and effect size tests were used to analyze the improvement of those outcomes and compared both groups., Results: At the baseline, there was no difference between TMT-A and B. A difference was found in the scalar score of TMT-B, as well as in Digit Backward Span and Total Digit Task. In TMT-A and B, the intervention group had better scores than the control group. The intervention group in the Digit Forward Span and the Total Digit obtained a moderate effect size and the control group also obtained a moderate effect size in Total Digit. In the Digit scalar scores, the control group achieved better results than the intervention group., Conclusions: The results on attention, processing speed and working memory improved in both groups. However, according to the effect sizes, the intervention group achieved better results than the control group. In addition, the attention and processing speed improved more than the working memory after the intervention. Although more studies are needed in this area, the results are encouraging for cognitive rehabilitation after stroke.

Background: Previous studies have suggested an association of Chiari Malformation type I (CM-I) and cognitive deficits. CM-I is a neurological disorder characterized by a descent of cerebellar tonsils into the foramen magnum, resulting in overcrowding of the upper cervical spine region. Posterior fossa decompression (PFD) is the surgical treatment of choice, however, the literature on the consequences for patients is mainly reduced to the assessment of physical symptoms., Methods: Data from a neuropsychological assessment of 76 patients with CM-I, both with PFD (n = 37) and without PFD (n = 39) surgery, and 76 healthy controls, matched by gender, age and years of education are reported., Results: CM-I patients show a generally lower cognitive performance in executive function, verbal fluency, spatial cognition, language (naming), verbal memory, processing speed, emotional facial recognition and theory of mind, compared to control group. The results are maintained even after statistically controlling for the influence of perceived physical pain and the presence of anxious-depressive symptomatology. Data also illustrate a similar cognitive profile between both groups with CM-I., Conclusion: These findings provide evidence of a deficient cognitive profile associated with CM-I, regardless of the PFD surgery. According to these results, both physical and cognitive consequences must be considered in the treatment of CM-I., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Purpose: Chiari malformation (CM) Type I is a rare disorder that implies an anomaly in the craniocervical junction, where one or both cerebellar tonsils are displaced below the foramen magnum into the cervical spinal channel. Research carried out regarding cognitive symptoms such as verbal fluency is scarce. The aim of this study was to investigate whether verbal fluency is impaired in a CM clinical group compared to a group of healthy control individuals while controlling for depression and anxiety symptomatology., Method: For this purpose, 101 individuals were enrolled to take part in the study (51 CM, 50 healthy controls). The Controlled Oral Word Association Test (Benton, de Hamsher, & Sivan, 1983) and the Hospital Anxiety and Depression Scale (Zigmond & Snaith, 1983) were administered., Results: Results showed significantly lower scores for the CM group in verbal fluency compared to the control group (p < .005). After performing an analysis of covariance to eliminate depression and anxiety symptomatology tendencies, it was observed that verbal fluency could not be predicted by this variable (p > .005)., Conclusions: From the results of this study, it can be concluded that people suffering from CM exhibit less verbal fluency than healthy control individuals and that this difference is not caused by depression or anxiety.

Chiari Malformation type I (CM-I) is a neurological disorder characterized by a displacement of the cerebellar tonsils through the foramen magnum into the spinal canal. Most research has focused on physical symptomatology but few studies include neuropsychological examinations. Moreover, although current research highlights the involvement of the cerebellum on higher cognitive functions, little is known about cognitive consequences associated with CM-I. The aim of this study is to analyze cognitive functioning between 39 CM-I patients and 39 healthy controls, matched by gender, age and years of education. Participants have been examined on a large battery of neuropsychological tests, including executive functioning, verbal fluency, spatial cognition, language, verbal memory, processing speed, facial recognition and theory of mind. Results show a poorer performance of the clinical group compared to the control group, even after controlling the effect of physical pain and anxious-depressive symptomatology. The findings suggest the presence of a generalized cognitive deficit associated with CM-I, which makes it necessary to focus attention not only on physical consequences, but also on cognitive ones.

Introduction: Facial emotional expression constitutes a basic guide in the social interaction and, thus, the alterations in its expression or recognition imply an important limitation for the communication. On the other hand, cognitive impairment and the presence of depressive symptoms, which are commonly found in patients with multiple sclerosis, it is unknown how they influence cognitive function and depression on emotional recognition., Aims: To consider the evaluation of time reaction and response accuracy of facial expression recognition in people affected by multiple sclerosis, and to assess the possible variables that may be modulating the emotion recognition, such as depression and cognitive functions., Subjects and Methods: The study has a cross-sectional non-experimental design with a single measurement. The sample is compound by 85 participants, 45 diagnosed as multiple sclerosis and 40 control subjects., Results: Multiple sclerosis subjects reveal significant differences in both reaction time and response accuracy in neuropsychological tests in comparison to the control group. Explanatory models were identified in the emotional recognition., Conclusion: Multiple sclerosis subjects face difficulties at recognising facial emotions; and differences at attention memory, processing speed and depressive symptomatology were observed in regard to the control group.

Rare neuromuscular diseases (NDs) are a group of inherited or acquired neurological pathologies affecting the muscles and the nervous system. Their low prevalence and high geographical dispersion can cause isolation and difficulties in social interaction between affected equals. New technologies, such as videoconferencing, offer a complementary option for improving the health of this population. The purpose of this study was to assess the effectiveness of a teleassistance program at improving health-related quality of life (HRQoL) through social interaction in adults with NDs. The sample consisted of 45 participants affected by rare NDs. Twenty-four participants were assigned to the experimental group (EG), which participated in the videoconferencing sessions, and 21 to the control group. Three questionnaires were administered: WHO-DAS II, Sickness Impact Profile, and SF-36 Health Survey. Effectiveness was assessed by a pre-post design. An online psychosocial program was applied over three-month period. Data revealed an improvement of the EG in psychosocial variables, e.g. "Getting along with people" (z = -2.289, r = -.47, p ≤ .05) or "Psychosocial Domain" (z = -2.404, r = -.49, p ≤ .05), and in physical variables, e.g. "Life activities" (z = -2.844, r = -.58, p ≤ .05). Social interaction appeared as a relevant factor at improving HRQoL levels. High levels of satisfaction about the teleassistance program were reported.

The assessment of facial expression is an important aspect of a clinical neurological examination, both as an indicator of a mood disorder and as a sign of neurological damage. To date, although studies have been conducted on certain psychosocial aspects of myasthenia, such as quality of life and anxiety, and on neuropsychological aspects such as memory, no studies have directly assessed facial emotion recognition accuracy. The aim of this study was to assess the facial emotion recognition accuracy (fear, surprise, sadness, happiness, anger, and disgust), empathy, and reaction time of patients with myasthenia. Thirty-five patients with myasthenia and 36 healthy controls were tested for their ability to differentiate emotional facial expressions. Participants were matched with respect to age, gender, and education level. Their ability to differentiate emotional facial expressions was evaluated using the computer-based program Feel Test. The data showed that myasthenic patients scored significantly lower (p < 0.05) than healthy controls in the total Feel score, fear, surprise, and higher reaction time. The findings suggest that the ability to recognize facial affect may be reduced in individuals with myasthenia.

Cognitive dysfunction in Parkinson's Disease (PD) has been consistently reported, but little is known about cognitive impairment in PD patients without dementia, and its association with clinical characteristics, neuropsychiatric disturbance and functional activities. Therefore, we evaluated 52 non-demented PD patients, 22 of them with mild cognitive impairment (PD-MCI) who were matched with 52 healthy controls. Our results confirm the existence of dysfunction in information processing speed, executive function, verbal memory and visuo-perceptual processing in PD. On the other hand, PD-MCI was associated with advanced age at the onset of PD, more neuropsychiatric symptoms, caregiver stress and functional problems. The study supports the hypothesis that specific neuropsychological impairments may act as modulators of functional impairment in PD, for instance slowness of information processing.