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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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, η 2p =.24; TM NEPSY: p  <  .001, η 2p =.35; Verbal task: p  <  .001, η 2p =.35; Happé's Strange Stories: p  =  .049, η 2p =.11) and HRQoL (Psychosocial health: p  =  .012, η 2p =.18; Emotional functioning: p  =  .037, η 2p = 0.13; Social functioning: p  =  .006, η 2p =.21; Total: p  =  .013, η 2p =.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. [ABSTRACT FROM AUTHOR]

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 < 0.005). 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 > 0.005). 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.

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, η2p=.038) and Verbal task (F = 1.820, p =.185, η2p=.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. [ABSTRACT FROM AUTHOR]

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 (CM) is a rare disorder that implies a congenital anomaly in the craniocervical junction. It is considered one of 7,000 different rare disorders affecting 7% of the whole [...]

The diagnosis of a pediatric neuromuscular disease has a psychological impact on parents. The study aimed to describe mental health and psychological adaptation in parents of children with neuromuscular diseases. The evaluation of parents (n = 35) through the Psychological Adaptation Scale and Adult Self-Report Questionnaire showed that (a) 82.4% of the participants had an adequate level of psychological adaptation; (b) parents presented high levels of mental health problems, (c) significant correlations were found between the mental health problems and psychological adaptation. This study highlights the need for psychological interventions which aim to promote psychological adaptation to NMD diagnoses and protect parental mental health. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]