CNS involvement in myotonic dystrophy type 1: does sex play a role?

Introduction: Myotonic dystrophy type 1 (DM1) is a hereditary neuromuscular disorder affecting the central nervous system (CNS). Although sex differences have been explored in other neuromuscular disorders, research on this topic in DM1 remains limited. The present study aims to analyze sex differences (both the patient's and disease-transmitting parent's sex) with a focus on CNS outcomes. Methods: Retrospective data from 146 non-congenital DM1 patients were analyzed, including clinical, molecular, neuropsychological, and neuroradiological data. Sex and inheritance pattern differences were analyzed using t-tests, and ANOVA analyses were conducted to address the interactions. Results: Overall, no significant sex differences were observed except in certain cognitive domains. However, individuals with maternal inheritance showed larger CTG expansion size, lower estimated IQs, and poorer performance on visual memory, executive functions, and language domains than those with paternal inheritance. Notably, IQ performance was independently influenced by inheritance pattern and CTG expansion. Discussion: This study is the first to delve into sex differences in DM1 with a focus on CNS outcomes. While the results revealed the absence of a sex-specific clinic-molecular profile, more substantial CNS differences were observed between patients with maternal and paternal inheritance patterns. The hypothetical existence of genomic imprinting and its potential mechanism are discussed. These findings hold potential implications for aiding clinical management by improving genetic counseling and predicting disease severity and prognosis.

Cross-cultural adaptation and psychometric validation of a Spanish version of the Maryland Assessment of Recovery Scale (MARS-12).

The aim of this study was to adapt and validate a Spanish version of the Maryland Assessment of Recovery Scale (MARS-12). It was carried out in strict accordance with internationally recognized guidelines for test adaptation. A preliminary Spanish version of the MARS-12 was first produced through a standardized translation/back-translation process, ensuring semantic, linguistic, and contextual equivalence with respect to the original scale. Its psychometric properties were then examined in a sample of 325 people with serious mental illness recruited from six different provinces in the Basque Country (northern Spain) and Catalonia (north-eastern Spain). They were users of a total of 20 community rehabilitation and psychiatry services. Confirmatory factor analysis supported a unidimensional structure, consistent with the original scale. Scores on the MARS-12 were positively correlated (.83) with scores on the Questionnaire about the Process of Recovery, supporting convergent validity, while validity evidence based on relationships with other variables was provided by positive correlations between MARS-12 scores and scores on the Dispositional Hope Scale (.82) and on the three dimensions of the Multidimensional Scale of Perceived Social Support (range .30 to .41). Reliability of MARS-12 scores was high (McDonald's ω = .97), as was temporal stability across a one-week interval (.89). The Spanish version of the MARS-12 is a valid and reliable scale that may be used by mental health professionals to assess recovery among Spanish people with serious mental illness.

Psychometric Properties and Factorial Invariance of the Educational-Clinical Questionnaire: Anxiety and Depression (CECAD) in Basque Population.

BACKGROUND: Anxiety and depression are the most common current mental health problems. Due to their comorbidity, there is a need for instruments that measure them simultaneously. Moreover, given that their prevalence varies by gender and age, it is important to examine the factorial invariance of such instruments. The present study aimed to analyze the dimensionality and factorial invariance of the Basque version of the Educational-Clinical Questionnaire: Anxiety and Depression (CECAD) as a function of gender and age, and to gather additional evidence of its validity. METHOD: The sample comprised 2131 participants (54.2% female) between 7 and 24 years old ( M = 13.2; SD = 3.52). RESULTS: The CECAD was found to have a two-dimensional structure invariant to gender and age, with higher latent means for girls in both dimensions, and for those aged 14 and over in depression, but with small effect sizes. Both reliability and convergent validity values were good. CONCLUSIONS: The Basque version of the CECAD has good evidence of validity and reliability for assessing anxiety and depression in Basque-speaking children and adolescents.

Psychometric Properties and Factorial Invariance of the Educational-Clinical Questionnaire: Anxiety and Depression (CECAD) in Basque Population / Propiedades Psicométricas e Invarianza Factorial del Cuestionario Educativo-Clínico: Ansiedad y Depresión (CECAD) en Población Vasca

Background: Anxiety and depression are the most common current mental health problems. Due to their comorbidity, there is a need for instruments that measure them simultaneously. Moreover, given that their prevalence varies by gender and age, it is important to examine the factorial invariance of such instruments. The present study aimed to analyze the dimensionality and factorial invariance of the Basque version of the Educational-Clinical Questionnaire: Anxiety and Depression (CECAD) as a function of gender and age, and to gather additional evidence of its validity. Method: The sample comprised 2131 participants (54.2% female) between 7 and 24 years old (M = 13.2; SD = 3.52). Results: The CECAD was found to have a two-dimensional structure invariant to gender and age, with higher latent means for girls in both dimensions, and for those aged 14 and over in depression, but with small effect sizes. Both reliability and convergent validity values were good. Conclusions: The Basque version of the CECAD has good evidence of validity and reliability for assessing anxiety and depression in Basque-speaking children and adolescents.(AU)

Antecedentes: En la actualidad, la ansiedad y la depresión son los problemas de salud mental más comunes. Debido a su comorbilidad, resulta necesario disponer de instrumentos que los midan simultáneamente. Además, dado que su prevalencia varía en función del género y la edad, es importante examinar la invarianza factorial de tales instrumentos. El objetivo del presente estudio fue analizar la dimensionalidad y la invarianza factorial de la versión en euskera del Cuestionario Educativo-Clínico: Ansiedad y Depresión (CECAD) en función del género y la edad, y recabar evidencias adicionales de validez. Método: La muestra estuvo compuesta por 2131 participantes (54.2% mujeres) con edades comprendidas entre los 7 y 24 años (M = 13.2; DT = 3.52). Resultados: El CECAD mostró una estructura de dos dimensiones invariantes al género y la edad, con medias latentes más altas para las chicas en ambas dimensiones, y para mayores de 14 años en depresión, pero con tamaños del efecto pequeños. Tanto las estimaciones de los índices de fiabilidad como las evidencias de validez convergente fueron buenas. Conclusiones: La versión en euskera del CECAD posee adecuadas evidencias de validez y fiabilidad para evaluar la ansiedad y la depresión en niños y adolescentes vascoparlantes.(AU)

Executive functions and daily functioning in myotonic dystrophy type 1 ecological assessment with virtual reality.

Central nervous system dysfunction is characteristic of patients with myotonic dystrophy type 1 (DM1). Although no consensus exists regarding the exact cognitive profile of these patients, executive dysfunction has been suggested to play a role. Due to the impact of executive functions on daily performance, this study aimed to describe executive functioning in an ecological manner and to analyze its impact - and that of other clinical variables - on the functional performance of DM1 patients. A Virtual Reality executive functioning test (Nesplora Ice Cream), the Wechsler Adult Intelligence Scale-Fourth Edition, and self-report questionnaires (AES, FSS, ESS and LIFE-H) were administered to 20 patients. Statistical analyses included correlation and multiple regression analyses to analyze the best predictors of daily performance. DM1 patients did not show major difficulties in the executive functioning tasks or in their overall performance on daily habits. However, both cold and hot executive functions still seem necessary for the correct accomplishment of life habits, since planning and level of apathy explained 47.6% of the total variance of daily functioning. This was the first study to assess executive functions in DM1 using Virtual Reality, and our findings open a debate about their actual impairment in this population.

Assessment of Attachment Representations in Basque Adolescents and their Relationship with Internalizing and Externalizing Problems.

Research has shown a relationship between attachment style and psychosocial adjustment in adolescents. Whereas secure attachment is related to fewer internalizing and externalizing symptoms, the opposite is the case for the various insecure attachment styles. The aim of the two studies reported in this paper was to adapt and validate the CaMir-R (a self-report measure of attachment that has shown adequate psychometric properties) for use among Basque adolescents, and to analyse the relationship between attachment and internalizing and externalizing symptoms. In Study 1, the instrument was adapted using the back translation method and applied to a sample of 203 adolescents and young adults. Confirmatory factor analysis supported the theoretical dimensions of the scale, and its psychometric properties were found to be adequate. In Study 2 we obtained additional validity evidence by applying, in a sample of 786 adolescents and young adults, the attachment representations section of the CaMir-R alongside other measures of attachment and clinical symptoms. The results once again supported the dimensional structure of the instrument, and evidence of convergent validity was obtained based on correlations between CaMir-R scores and scores on the Inventory of Parent and Peer Attachment (IPPA). In addition, scores on the five dimensions of attachment representations (Security, Family concerns, Parental interference, Self-sufficiency and resentment of parents and Childhood trauma) were correlated with scores on other measures of internalizing and externalizing symptoms. Based on these results, we conclude that the Basque version of the CaMir-R is a valid instrument for assessing the quality of attachment representations among adolescents, and also that internalizing and externalizing problems are related to attachment style. We discuss the importance of attachment in relation to behaviour problems and clinical symptoms.

Gender awareness is also nurses' business: Measuring sensitivity and role ideology towards patients.

AIM: This study aims to validate the Nijmegen Gender Awareness in Medicine Scale, which assesses gender sensitivity and gender-role ideology towards patients in the Spanish language for use among physicians and nurses. BACKGROUND: Women are more likely to suffer pain, delays and health consequences related to low therapeutic effort. Health professionals' gender awareness may minimize such bias; however, the only instrument to assess such awareness is limited to physicians and lacks a Spanish version. METHODS: After using the back-translation method, a sample of 167 Spanish nurses and nursing students completed the instrument. In order to obtain additional validity evidence, 98 health professionals filled in gender sensitivity and gender-role ideology towards patients' subscales and the short versions of the Ambivalent Sexism Inventory. RESULTS: Gender-role ideology towards patients correlated strongly with sexist attitudes, demonstrating convergent validity, and Cronbach's alpha coefficients showed an adequate internal consistency. CONCLUSIONS: Nijmegen Gender Awareness in Medicine Scale perfectly applies to nurse population, and this adaptation also broadens its use for Spanish professionals. IMPLICATIONS FOR NURSING MANAGEMENT: Nurse managers and educators can use this applicable tool to treat low gender awareness levels as a modifiable risk factor and promote a gender-sensitive caring culture.

A validated WAIS-IV short-form to estimate intellectual functioning in myotonic dystrophy type 1.

Currently, no rapid and specific instrument is available to briefly estimate intelligence in patients with myotonic dystrophy type 1 (DM1), a multisystemic disease that involves the CNS and is associated with cognitive deficits and low intellectual functioning. This study aimed to develop a DM1-specific and valid short-form of the Wechsler Adult Intelligence Scale-Fourth Edition (WAIS-IV) to estimate intellectual functioning in this population. Thirty non-congenital DM1 patients (10 female; mean age=46.77; SD= 9.76) were assessed with the WAIS-IV. Data were analyzed following two independent strategies: A) multiple linear regression with the aim of maintaining the scale's factorial structure; and B) correlational analyses between scores on all WAIS-IV subtests and Full-Scale IQ (FSIQ). Validity of the resulting short-forms was also analyzed. Three short-forms were developed: Proposal A from strategy A (Vocabulary, Block Design, Arithmetic and Symbol Search), Proposal B1 (Vocabulary, Block Design, Digit Span and Visual Puzzles) and Proposal B2 (Vocabulary and Block Design), from strategy B. All three short-forms showed a strong and significant correlation with the FSIQ and were considered psychometrically acceptable. Arguments in favor of Proposal B1 are discussed. Assessing FSIQ with these short-forms will be useful for avoiding long assessment procedures in a population characterized by high fatigability.

The Impact of Sexism and Gender Stereotypes on the Legitimization of Women's Low Back Pain.

BACKGROUND: Low back pain is the worldwide leading cause of disability and, even though women's pain experience is more severe, frequent, and enduring, female patients are often underdiagnosed and undertreated. Health professionals' gender stereotypes and social norms may underlie the downgrading of pain. AIM: This pilot study aimed to examine the legitimation of low back pain by health professionals in relation to the sex of the patient as well as their gender awareness and the relationship between them. METHOD: This study had a cross-sectional design. Eighty health professionals and students selected by convenience answered a 4-part online questionnaire. The eligibility criteria for participants were: aged >18 years, students in the last course of nursing/medicine or a physician/nurse, and Spanish-speaking. The questionnaire comprises: (1) a between-subjects virtual clinical low back pain case with four random versions (female/male patient and evidence/non-evidence of pathology); (2) the Spanish version of Nijmegen Gender Awareness Scale (S-NGAMS); (3) Ambivalent Sexism Inventory (ASI); and (4) Ambivalence toward Men Inventory (AMI). RESULTS: The total score of legitimation of low back pain correlated negatively with gender role ideology and sexism scales (when the virtual patient was female), as well as the subscales of willingness to offer support and credibility. CONCLUSIONS: Both sexism and gender role ideology could undermine the legitimation of low back pain, the willingness to offer support, and credibility only in female patients. The results showed a possible gender bias in low back pain assessment in health professionals. Low gender sensitivity and high sexism must be treated as modifiable risk factors for health inequities in pain care.

Assessment of Personality in Basque Public Sector Employees and Its Role in Predicting Organizational Citizenship Behaviors in Selection Processes.

Organizational citizenship behaviors (OCBs) are an important aspect of job performance as they enhance the effectiveness of organizations. Research has shown that personality is a moderate predictor of job performance. This study, involving a sample of 678 public sector employees in the Basque Country (northern Spain), pursued two aims: First, to develop and validate a Basque-language version of the Overall Personality Assessment Scale (OPERAS), a scale designed to assess the Big Five personality factors in a wide range of settings; and second, to examine whether person-organization fit (PO fit) and adaptive performance improve the capacity of personality to predict OCBs. The results indicated that the adapted scale was a suitable instrument for assessing personality in the Basque-speaking population. Furthermore, PO fit and adaptive performance improved the capacity of personality to predict OCBs. Based on these results, we propose a new predictive model that may enhance the efficiency of personnel selection processes.

Small for Gestational Age Moderate to Late Preterm Children: A Neuropsychological Follow-up.

Determine whether SGA constitutes a neurodevelopmental risk-factor of MLP, exploring if potential developmental difficulties at toddlerhood persist and are related to school-age performance. 109 SGA and 109 adequate for gestational age MLP children were evaluated at 2 and at 6.5 y.o. SGA children obtained poorer results in several areas at both timepoints; and their development at toddlerhood strongly correlated with only some results at school-age. SGA confers vulnerability to MLP, evolving from global/unspecific difficulties in toddlerhood to a domain-specific profile (attentional/dysexecutive) at 6.5. Findings claim the need for neuropsychological follow-up in MLP to identify emerging difficulties.

Initial validation of the Basque version of the Parental Burnout Assessment (B-PBA).

The aim of the present study was to validate the Parental Burnout Assessment in a Basque sample of parents. The Basque version of the PBA (B-PBA) was administered to 250 parents, with at least one child living at home. We investigated whether the four-dimensional structure of the PBA held in a sample of male and female parents. Furthermore, we examined the relationships between PBA and several sociodemographic variables such as participants' age, gender, number of children, family type, level of education, socioeconomic level, being in paid employment, and time spent with children. The results confirm the four-factor structure of the B-PBA including exhaustion in one's parental role, contrast with previous parental self, feelings of being fed up, and emotional distancing from one's children. The B-PBA shows good psychometric properties. Regarding sociodemographic variables, mothers present higher levels of parental burnout in comparison with fathers. In the rest of the sociodemographic variables there are weak relationships regarding parental burnout. In the discussion, we provide feedback on the hypotheses and results obtained and we interpret the results; we highlight the relevance of the parental burnout construct in the Basque Country; and we present practical implications as well as future perspectives.

Promoting adherence to psychopharmacological treatment among prisoners with mental health problems: Follow-up of a randomized controlled trial.

This study presents follow-up results regarding a treatment adherence programme (TAP) for prisoners, the initial effectiveness of which we previously evaluated in a randomized controlled trial. Here we used an experimental design with two randomized groups and assessment at four time points: baseline (pre-intervention), at 3 months (post-intervention), and at 6 and 9 months after baseline. Participants were 151 prisoners with mental health problems (Mage = 41.85, SD = 10.31) who were randomly assigned to either the TAP or treatment as usual (TAU). Prisoners who completed the TAP showed a greater improvement in treatment adherence at 3 and 9 months, compared with those who received TAU. There were no significant differences between the groups in subjective well-being under medication. The availability of an easy-to-apply, universal programme that is able to promote treatment adherence in the prison context could make a positive contribution to the general health of inmates.

A Validated WISC-V Short-Form to Estimate Intellectual Functioning in Very Preterm Children at Early School Age.

Very preterm children (gestational age < 32 weeks) frequently show neurodevelopmental difficulties (Inattention/dysexecutiveness) throughout their life-stages. A scarcity of resources, along with this population's cognitive vulnerability, makes the neuropsychological evaluation of these children both complicated and time-consuming. This study aimed to develop a specific and valid Wechsler Intelligence Scale for Children-Fifth Edition (WISC-V) short-form to estimate intellectual functioning in this population. Eighty-four very preterm children (39 female; mean age = 6.50; SD: 0.06) were assessed with the WISC-V. Short-forms were developed following two independent strategies: a) multiple linear regressions for each index; b) correlational analyses between scores on all administered subtests and Full-Scale IQ. Validity of short-forms was analyzed. A short-form (Vocabulary, Matrix Reasoning, Picture Span, and Symbol Search) that satisfied 2/3 validation criteria was proposed. This validated short-form could facilitate the identification of cognitive difficulties in very preterm children, so that they could benefit from early care and support services, avoiding long assessment procedures.

Team Emotional Intelligence in Working Contexts: Development and Validation of the Team-Trait Meta Mood Scale (T-TMMS).

The collective construct of Team Emotional Intelligence (TEI) has been widely used and discussed. However, although several studies have examined the relationship between individual emotional intelligence and transformational leadership, few reports have explored the TEI of leadership teams. The aim of this study was to develop a scale to measure TEI, developing and validating the T-TMMS in a sample of 1,746 participants grouped into 152 leadership teams. The research design of the study was cross-sectional, and, in order to observe reliability as well as the construct, convergent, and predictive validity of the scale, we conducted an internal consistency analysis, confirmatory factor analysis, as well as a correlation and hierarchical linear regression analysis. The T-TMMS showed a three-factor structure (Attention, Clarity, and Repair), with adequate internal consistency, temporal stability, and convergent validity. We also examined the relationship between TEI and organizational performance. The limitations and implications of this new scale for organizational contexts are discussed.

Randomised controlled trial of a treatment adherence programme for prisoners with mental health problems in Spain.

The prevalence of psychiatric disorders is high among prisoners, who often need psychotropic medication, but compliance with treatment is often poor. This combination leads to poorer prisoner-patient health and increased health costs.The aim of this study was to test the impact of a treatment adherence programme (TAP) on medication adherence and subjective well-being among prisoners with mental health problems. We conducted a randomised controlled trial of a treatment adherence programme compared with treatment as usual (TAU) among 183 prisoners with mental health problems. Those in the TAP group showed greater treatment adherence and reported better subjective well-being with medication than those in the TAU group. We conclude that the treatment adherence of prisoners who require medication for mental health problems may be improved through specific intervention programmes. This could make a positive contribution to individual health and well-being.

The Emotional Divergent-Convergent Thinking Program (EDICOP): Design, Implementation, and Results.

In a social environment that requires young people to adapt to increasingly demanding situations, emotional education and creativity training may be key for both personal development and academic performance. Given that there are currently no known interventions that develop emotional and creative skills simultaneously in a youth population, the main objective of this study was to design, implement, and evaluate the Emotional Divergent-Convergent Thinking Program (EDICOP). The study design was quasi-experimental with a non-equivalent control group and pretest-posttest measures. The participants included 196 students between 16 and 24 years of age belonging to two centers of higher education. Our results showed that the EDICOP contributed to the improvement of the participants' divergent-proactive style, positive affectivity, emotional predisposition, and attention, as well as to their preference for cognition. Overall, the EDICOP is, therefore, both relevant and useful, and further research on the mood-creativity link is merited to generate new contexts in higher education for the promotion of both the emotional and creativity dispositions and self-awareness, by combining three basic psychological processes (emotion, cognition, and motivation).

Age-related cognitive decline in myotonic dystrophy type 1: An 11-year longitudinal follow-up study.

BACKGROUND: Myotonic dystrophy type 1 (DM1) is an inherited multi-systemic disease involving the central nervous system (CNS) and is consequently characterized by a range of cognitive impairments. However, whether this cognitive profile progresses over time is still a matter of debate. The aim of this study was to longitudinally assess a DM1 sample, in order to compare, for the first time, this progression with that of a control group. Clinical and socio-demographic predictive factors potentially implicated in this possible decline are analysed. METHOD: Seventy-five DM1 patients with childhood, juvenile, adult, and late-onset, and 54 control participants were re-assessed in an 11-year follow-up with a comprehensive neuropsychological battery. The analyses employed were mixed ANOVA for repeated measures to test intergroup comparisons over time and multiple linear regression for predictive variable analysis. RESULTS: Myotonic dystrophy type 1 patients significantly worsened in visuospatial/visuoconstructive abilities and visual memory compared with controls. Multiple linear regression revealed that progression of cognitive impairment measured by copy of the Rey-Osterrieth complex figure was predicted by muscular impairment, whilst on the block design test age predicted the change with a cut-off at 31 years of age. DISCUSSION: A domain-specific progressive cognitive decline was found in DM1, with visuospatial/visuoconstructive abilities showing the greatest vulnerability to the passage of time. In addition to important clinical implications, these results suggest the need for the scientific community to delve deeper into the potential mechanisms underlying early cognitive decline in this population.

Parenting Styles and Internalizing Symptoms in Adolescence: A Systematic Literature Review.

A growing body of recent research has identified associations between various parenting practices and styles and internalizing problems among adolescents. However, the reported findings are inconsistent and the studies in question have been conducted from different theoretical backgrounds. The aim of this systematic review was to synthesize the literature on the association of parental socialization styles with depression, anxiety, and suicidal ideation. To this end, we conducted a systematic search of the PsycInfo, Scopus, Pubmed, and Web of Science databases, covering literature published from 2010 to 2019. The search was restricted to peer-reviewed studies in English or Spanish. The results show that parental warmth, behavioural control, and autonomy granting are inversely related to internalizing symptoms in adolescents. Conversely, psychological control and harsh control by parents are positively associated with adolescent anxiety, depression, and suicidal ideation. Although the associated effect sizes are only small or moderate, the results suggest that these variables should be taken into account when designing programmes aimed at promoting parenting styles conducive to the wellbeing of adolescents.

Assessment of Entrepreneurial Orientation in Vocational Training Students: Development of a New Scale and Relationships With Self-Efficacy and Personal Initiative.

Having emerged as an important concept in the organizational field, entrepreneurial orientation has also become a key idea in the context of education. Indeed, entrepreneurial education is now one of the common objectives for education and training systems in the European Union. Despite its importance, however, there is a scarcity of valid and reliable measures for assessing entrepreneurial orientation in students. The present study aimed to address this by developing and examining the psychometric properties of the Entrepreneurial Orientation Scale (EOS). A second objective is to study the relationships between entrepreneurial orientation and gender, self-efficacy, and personal initiative. The sample comprised 411 vocational training students (50.36% male, 49.64% female). The final version of the instrument comprised 32 items assessing six dimensions: innovativeness, risk-taking, proactiveness, competitiveness, achievement orientation, and learning orientation. The EOS showed good psychometric properties and its dimensions demonstrated concurrent relationships with self-efficacy and personal initiative. The EOS may be used to measure entrepreneurial orientation in the educational context and to evaluate interventions designed to promote an entrepreneurial spirit in schools, colleges, and universities.