DeustoTeka Examinando por Autor "Amayra Caro, Imanol"

Examinando por Autor "Amayra Caro, Imanol"

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Clinical and psychological disturbances of myasthenia gravis: how anxiety, sleep quality, and thymectomy interact
(Multidisciplinary Digital Publishing Institute (MDPI), 2024-12) García Sanchoyerto, Maddalen; Amayra Caro, Imanol; Ruiz de Lazcano Sánchez, Aitana; Rodríguez Bermejo, Alicia Aurora; Anguiano, Samuel; Salgueiro, Monika
Background: Myasthenia gravis (MG) is an autoimmune neuromuscular disease characterized by fatigue and muscle weakness. Previous research has noted a high incidence of psychological problems, particularly anxiety and depression, as well as alterations in sleep quality among patients with MG. The aim of this study was to assess depression, anxiety, sleep problems, and fatigue in individuals with MG and to analyze the relationships among these variables, as well as to determine the influence of corticosteroid use and thymectomy. Methods: The sample consisted of 72 participants, 42 belonging to the clinical group with MG and 30 healthy participants to the control group. The instruments used were Hospital Anxiety and Depression Scale (HADS), Pittsburgh Sleep Quality Index (PSQI), and Fatigue Severity Scale (FSS). Results: The results showed that MG patients exhibited higher levels of fatigue, anxiety, depression, and poorer sleep quality compared to the control group (p < 0.05). Significant differences in sleep quality and anxiety levels were observed between thymectomized and non-thymectomized patients. Additionally, a positive correlation was found between anxiety symptoms and sleep quality. Conclusions: The study demonstrated the presence of anxious and depressive symptoms in patients with MG, as well as poorer sleep quality and increased fatigue, suggesting that MG affects not only neuromuscular function but also the psychological well-being of patients.
Cognition in Chiari malformation type I: an update of a systematic review
(Springer, 2024-09) García Martín, Maitane; Amayra Caro, Imanol; Pérez Álvarez, Manuel; Salgueiro, Monika; Martínez, Óscar; López Paz, Juan Francisco; Allen, Philip A.
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.
Cognitive functioning in adults with phenylketonuria in a cohort of Spanish patients
(Hindawi Limited, 2023) Luna Ovalle, Paula; López Paz, Juan Francisco; García Martín, Maitane; Amayra Caro, Imanol; Martínez, Óscar; Pérez Álvarez, Manuel; Rodríguez Bermejo, Alicia Aurora; Pérez Núñez, Paula; Ceberio, Imanol; Mansilla, Noelia; Soria, Cristina
The early introduction of a low phenylalanine (Phe) diet has been demonstrated to be the most successful treatment in subjects with phenylketonuria (PKU), especially for preventing severe cognitive and neurological damages. However, it still concerns that even if treated in the first months of life with supplements and following a diet, they can show slight scores below people without PKU in neuropsychological assignments. We investigated 20 adults with classical PKU aged 19-48 years (mean age 29 years) and 20 heathy controls matched by age, gender, and years of education. Patients and controls were assessed with an extended neuropsychological battery, as well as psychological aspects and quality of life, also the last Phe level result was obtained. Results showed that the most affected cognitive domains are processing speed, executive functioning, memory, and also theory of mind, but very well-preserved verbal fluency, language, and visuospatial functioning. In quality of life, some significant results were seen specially in anxiety of Phe levels, anxiety of Phe levels during pregnancy, guilt if poor adherence to supplements, and if dietary protein restriction not followed. No significant results were obtained for the psychological variables. In conclusion, it has been shown that a combination of a low Phe diet, supplement intake, and keeping Phe levels in a low range seems appropriate to have the most normal and alike cognitive performance to persons without PKU.
Comparative study of emotional facial expression recognition among Prader–Willi syndrome subtypes
(John Wiley and Sons Inc, 2024) Perosanz Hidalgo, Ane; López Paz, Juan Francisco; Amayra Caro, Imanol; García Martín, Maitane; Martínez, Óscar
Background: Prader–Willi syndrome (PWS) is a congenital disease caused by a rare and generally non-inherited genetic disorder. The inability to recognise facial expressions of emotion is an apparent social cognition deficit in people diagnosed with PWS. The main objective of the present study is to compare the ability to recognise emotional facial expression, in both non-contextualised and contextualised scenarios, among the main subtypes of PWS and a control group. Methods: The sample consisted of 46 children divided into three groups: deletion (n = 10), maternal uniparental disomy (mUPD) (n = 13) and control (n = 23). The protocol included the Facially Expressed Emotion Labeling and the Deusto-e-Motion 1.0. Results: The control group recognised facial emotions more accurately and quickly in both non-contextualised and contextualised scenarios than children with PWS, regardless of genetic subtype. Despite no differences being detected between PWS subtypes when non-contextualised scenarios were analysed, in contextualised situations, a longer reaction time was observed in children with the mUPD subtype. Conclusions: This is the first study to assess the ability to recognise emotional facial expressions in contextualised situations among PWS subtypes and a control group. The findings suggest that some of the social cognitive deficits evidenced in children with mUPD PWS may be similar to those in autism spectrum disorder.
Content and face validity in virtual reality with children: a validation in five steps+1 of a wheelchair basketball game
(Frontiers Media SA, 2024) Ceberio, Imanol; Al-Rashaida, Mohammad Ali Hussein; García Martín, Maitane; López Paz, Juan Francisco; Salgueiro, Monika; Passi, Nicole; Pavel, Humberto; Amayra Caro, Imanol
Objective: The present study is aimed to elaborate and determinate the content and face validity of a virtual reality program attending the perspective of children. This simulation is designed to promote empathy and understanding towards children with motor disabilities through adapted sport scenes. This study proposed a validation approach with six phases to assess technical and aesthetic aspects. Method: Sample: a) Content validity study, 20 children (11–18 years old) were recruited as lay and content experts, who assessed the properties of grade of realism and physical fidelity of wheelchair basketball scenarios. b) Face validity study, 395 children were recruited as lay experts and divided into two groups (7–9 years old and 10–12 years old), or into ten subgroups according to Age × Gender interaction. The face validity sample assessed the psychological fidelity and the presence of wheelchair basketball scenarios. Instruments: Virtual Reality Content Validity Questionnaire, GAMEX questionnaire and Simulator Sickness Questionnaire (SSQ). Results: The content validity study showed preference for technical aspects (music, colors and degree of realism). Therefore, modifications in the design were made. In the content validity study, the high agreement level was influenced by previous sport experiences. In the face validity study, the cognitive development of children determined the differences in agreement levels in some virtual properties (absorption and cybersickness). In this sixth step, the ages 7–8 years versus 10–11 years showed significant differences in validity. The study also criticized the face validity cut-offs often used in adult-focused research, emphasizing the need to adapt them for children´s developmental stages. Conclusion: This study proposes a sixth step not traditionally included in content and face validity processes, specially focusing on the child user. The suitability of content and scenes should follow the same principles of standardization as other methodologies, such as psychometric tests, considering age and gender.
Coping with Wolf-Hirschhorn syndrome: quality of life and psychosocial features of family carers
(BioMed Central Ltd, 2020-10-19) Berrocoso Cascallana, Sarah; Amayra Caro, Imanol; Lázaro Pérez, Esther; Martínez, Óscar; López Paz, Juan Francisco; García Martín, Maitane; Pérez Álvarez, Manuel; Al-Rashaida, Mohammad Ali Hussein; Rodríguez Bermejo, Alicia Aurora; Luna Ovalle, Paula; Pérez Núñez, Paula; Blanco, Raquel; Nevado, Julián
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.
Difficulties in social cognitive functioning among pediatric patients with muscular dystrophies
(Frontiers Media SA, 2023) García Urquiza, Irune; Martínez, Óscar; López Paz, Juan Francisco; García Martín, Maitane; Rodríguez Bermejo, Alicia Aurora; Amayra Caro, Imanol
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.
Diseases costs and impact of the caring role on informal carers of children with neuromuscular disease
(MDPI AG, 2021-03-15) Rodríguez Bermejo, Alicia Aurora ; Martinez Gutierrez, Oscar ; Amayra Caro, Imanol ; López Paz, Juan Francisco; Al-Rashaida, Mohammad Ali Hussein ; Lázaro Pérez, Esther; Caballero, Patricia ; Pérez Álvarez, Manuel ; Berrocoso Cascallana, Sarah; García Martín, Maitane; Luna Ovalle, Paula ; Pérez Núñez, Paula ; Passi, Nicole
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.
Effect of the Wii Sports Resort on the improvement in attention, processing speed and working memory in moderate stroke
(BioMed Central Ltd., 2019-02-28) Unibaso Markaida, Iratxe; Iraurgi Castillo, Ioseba; Ortiz Marqués, Nuria; Amayra Caro, Imanol; Martínez Rodríguez, Silvia
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
Effects of a neuropsychosocial teleassistance intervention on social cognition and health-related quality of life of pediatric patients with neuromuscular diseases
(Oxford University Press, 2024-08) García Urquiza, Irune; Martínez, Óscar; Amayra Caro, Imanol; Salgueiro, Monika; Rodríguez Bermejo, Alicia Aurora; López Paz, Juan Francisco
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; Happe'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.
Effects of teleassistance on the quality of life of people with rare neuromuscular diseases according to their degree of disability
(Frontiers Media S.A., 2021-03) Martínez, Óscar; Amayra Caro, Imanol; López Paz, Juan Francisco; Lázaro Pérez, Esther; Caballero, Patricia; García Urquiza, Irune; Rodríguez Bermejo, Alicia Aurora; García Martín, Maitane; Luna Ovalle, Paula; Pérez Núñez, Paula; Barrera, Jaume; Passi, Nicole; Berrocoso Cascallana, Sarah; Pérez Álvarez, Manuel; Al-Rashaida, Mohammad Ali Hussein
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.
Facial and emotion recognition deficits in Myasthenia Gravis
(Multidisciplinary Digital Publishing Institute (MDPI), 2024-08) García Sanchoyerto, Maddalen; Salgueiro, Monika; Ortega, Javiera; Rodríguez Bermejo, Alicia Aurora; Parada Fernández, Pamela; Amayra Caro, Imanol
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.
Health-related quality of life (HRQoL) and psychological impact of the COVID-19 pandemic on patients with myasthenia gravis
(International Advancement Center for Medicine and Health Research Co., Ltd., 2023) García Urquiza, Irune; Martínez, Óscar; López Paz, Juan Francisco; Salgueiro, Monika; Rodríguez Bermejo, Alicia Aurora; Zorita-Pérez, Janire; García Sanchoyerto, Maddalen; Amayra Caro, Imanol
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, η2p = 0.023; Role Emotional p = 0.250, η2p = 0.011; and HADS Total p = 0.161, η2p = 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.
Health-related quality of life in 153 children with neuromuscular disorders in Latin America: is it age, functional dependence or diagnosis?
(W.B. Saunders Ltd, 2024-09) Ortega, Javier; Vázquez, Natalia; Amayra Caro, Imanol; Muntadas, Javier; Squitín Tasende, Magalí; Rodríguez Bermejo, Alicia Aurora
Neuromuscular diseases impact on children's health related quality of life but there is a lack of studies in Latin America that measured this construct. To respond to this need, this study aimed to explore quality of life and its relationship with age, functional dependence and specific diagnosis in children and adolescents in Latin America. A cross-sectional correlation study was carried out with 133 caregivers on children (2–18 years old) with various neuromuscular disorders. Parents reported on their children's health related quality of life through the PedsQL GCS and the PedsQL NMM. Differences in quality of life were found when comparing children with high functional dependence with those with mild dependence (p = 0.05). No significant differences were found regarding the child diagnosis. Finally, quality of life was highly correlated with the child's age, even when controlling for functional dependence differences between ages. Children and adolescents with neuromuscular show a diminished health related quality of life, not only in physical functioning but in their psychosocial functioning. Health related quality of life may vary according to the child's age and functional dependence
Impact of chronic pain and depressive symptoms on the quality of life of adults with Chiari Malformation type I: a comparative study
(International Advancement Center for Medicine and Health Research Co., Ltd., 2024) García Martín, Maitane; Amayra Caro, Imanol; Pérez, Manuel; Rodríguez Bermejo, Alicia Aurora; Salgueiro, Monika; Infante, Jon
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
Intrusion errors during verbal fluency task in amyotrophic lateral sclerosis
(Public Library of Science, 2020-05-29) Pérez Álvarez, Manuel; Amayra Caro, Imanol; Lázaro Pérez, Esther; García Martín, Maitane; Martínez, Óscar; Caballero, Patricia; Berrocoso Cascallana, Sarah; López Paz, Juan Francisco; Al-Rashaida, Mohammad Ali Hussein; Rodríguez Bermejo, Alicia Aurora; Luna Ovalle, Paula; Varona, Luis
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.
Limb–girdle muscular dystrophy D2 TNPO3-Related: a quality of life study
(Multidisciplinary Digital Publishing Institute (MDPI), 2023-07-24) Rodríguez Bermejo, Alicia Aurora ; Amayra Caro, Imanol ; García Urquiza, Irune ; Angelini, Corrado
The present study is the first research that analyzes the quality of life (QoL) of people affected by a dominant form of limb–girdle muscular dystrophy, specifically limb–girdle muscular dystrophy D2 (LGMD-D2). Additionally, clinical forms of the individual cases of the six affected patients are presented. This study also aims to explore the differences between patients’ reports and caregivers’ reports, and between LGMD-D2 and recessive forms of LGMD. The instruments used were as follows: sociodemographic data, GSGC, and INQoL instrument. The sample consisted of six people affected by LGMD-D2: three caregivers of three affected people, and three patients with recessive LGMD. They came from associations of affected people and a hospital in Padua. Those affected have multiple symptoms that lead to disability, which ultimately leads to dependence on the assistance. The present study shows that LGMD-D2 has a greater impact on activities of daily living, fatigue, muscle pain, and independence than other LGMD pathologies or other neuromuscular diseases. It also appears that age could influence QoL, and that muscle weakness is a very disabling symptom in this variant. In the current context of constantly developing research for new treatments, it is essential to analyze which aspects are most affected. Finally, caregivers can play an essential role in symptom reporting, as certain psychological adjustment mechanisms in the patient may be interfering with the objectivity of the report.
Memory in spina bifida, from childhood to adulthood: a systematic review
(Multidisciplinary Digital Publishing Institute (MDPI), 2024-09) Amayra Caro, Imanol; Ruiz de Lazcano Sánchez, Aitana; Salgueiro, Monika; Anguiano, Samuel; Ureña, Malena; Martínez, Óscar
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
Music therapy and Sanfilippo syndrome: an analysis of psychological and physiological variables of three case studies
(BioMed Central Ltd, 2021-11-20) Pérez Núñez, Paula; Lázaro Pérez, Esther ; Amayra Caro, Imanol ; López Paz, Juan Francisco; Caballero, Patricia; Martinez Gutierrez, Oscar; Pérez, Manuel; Berrocoso Cascallana, Sarah; Al-Rashaida, Mohammad Ali Hussein; García Martín, Maitane; Rodríguez Bermejo, Alicia Aurora; Luna Ovalle, Paula
Introduction: Mucopolysaccharidosis type III (MPS III) or Sanfilippo syndrome is a neurodegenerative disease caused by the accumulation of mucopolysaccharides in the body. As the symptoms are wide ranging, it is a challenge to provide a diagnosis and psychological treatment for affected children. Method: The main objective of this study was to describe a form of music therapy treatment applied to three children diagnosed with MPS III. The psychological variables were evaluated by an ad hoc observation recording template, and the physiological variables were measured with a digital meter before and after each session. The perception of the parents was also considered through a semi-structured interview. Results: An improvement in the psychological variables was shown in all cases. Changes in the physiological variables were also noted, although they varied according to each child. The parents report some benefit of music therapy and they share difficulty in assessing the extent of benefits of the music therapy. Discussion: Findings indicate that music therapy can be a useful form of treatment with multiple benefits for children with conditions such as MPS III or similar conditions. However, further research is needed in this area and in the development of specific ways of evaluating music therapy.
Neuropsychological profile of hereditary ataxias: study of 38 patients
(Oxford University Press, 2022-08) García Martín, Maitane; Rouco Axpe, Idoia; Amayra Caro, Imanol; Rodríguez-Antigüedad Zarranz, Alfredo; Catalli, Claudio; Cabrera Zubizarreta, Alberto; Rodríguez Bermejo, Alicia Aurora; Pérez Álvarez, Manuel
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

Examinando por Autor "Amayra Caro, Imanol"

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