Partial Klüver-Bucy syndrome in a Paediatric patient: A post-neurosurgical and neuropsychological cases.

A variety of cognitive, behavioural, and emotional impairments have been reported in the literature that are associated with the resection of the temporal cortex. Klüver-Bucy syndrome is one infrequently reported disorder in the paediatric population. This paper describes the neuropsychological findings of a female paediatric patient at 7 and 10 years of age with a diagnosis of partial Klüver-Bucy syndrome (pKBS) following total resection of the amygdala and right hippocampus to resect a glioma. The patient presented emotional problems, aggressiveness, hypermetamorphosis, social indifference, and behavioural dysexecutive syndrome, which was found at both 7 and 10 years, but with a decrease in the severity of alterations in attention, impulsivity, hyperactivity, and aggressive behaviour in a second evaluation after she had a neuropsychological intervention. These findings describe the neuropsychological profile of paediatric case with resection of the amygdala and right temporal lobe.

Exploratory study of the prevalence of food addiction and its relationship with executive functioning, depression, and reinforcement sensitivity in a sample of Mexican adults.

Introduction: The study of food addiction (FA) has become relevant due to its high prevalence, the negative impact on quality of life, and its association with neuropsychological and psychiatric symptoms. Several studies have provided scientific support for these associations, however, the results are contradictory. Additionally, studies have unsuccessfully elucidated the true nature of the failures in executive functioning in people with FA symptomatology, particularly when it comes to executive deficits. Therefore, the purpose of this research was to establish whether the presence of executive dysfunction, depressive symptoms and binge eating problems, as well as high reward sensitivity entails a greater severity in FA traits and high body mass index (BMI) in a sample of Mexican adults. Methods: The sample consisted of Mexican men and women between 21-59 years (n = 36); who completed self-report questionnaires and performance tests to measure the study variables. Additionally, BMI was estimated with self-reported height and weight. Results: Our results showed that a high number of FA symptoms were associated with higher executive dysfunction scores, greater reward sensitivity, and more severe depressive and binge eating problems. Furthermore, factors that are more strongly associated with higher scores of FA include severe executive deficits, greater activation of the punishment avoidance system, and persistence in the search for reward when the depressive symptoms increased. The factors that best explained changes in the estimated BMI of women were a decreased crystallized intellectual capacity and the inability to control food intake as the number of FA symptoms increased. Discussion: In summary, the cognitive functioning profile characterized by general failure of the executive functioning, as well as a greater activation of the Punishment Avoidance System and persistence in the search for reward, were associated with greater severity of FA symptoms, especially when the depressive symptomatology was severe. In parallel, the psychopathology in participants associated with FA confirms the contribution of anxious and depressive symptomatology and borderline personality traits which could facilitate the expression of clinically relevant FA symptoms in women. Finally, we found that decreased crystallized intellectual capacity and inability to control food intake were linked to higher BMI when the number of FA symptoms increased.

Teleneuropsychology during the COVID-19 pandemic in Mexico: the perspective from a middle-income country.

INTRODUCTION: The use of teleneuropsychology (TeleNP) increased as a result of the COVID-19 pandemic; however, there have been no studies of the benefits and difficulties with this modality in middle-income countries. This study aimed to assess the current use of TeleNP in Mexico. METHOD: Mexican neuropsychologists were invited to participate in an online survey regarding the use of TeleNP during the COVID-19 pandemic. The survey was based on issues from a literature review and consisted of 36 questions requiring yes/no, multiple choice, or ordinal answers. The survey was created using Google Forms and asked respondents to provide informed consent. A total of 107 clinical neuropsychologists completed the survey. RESULTS: 82% of participants currently use TeleNP, and most reported learning about TeleNP through personal experience, literature research, and colleagues. Brief evaluations, delivery of results, and intervention were the principal services provided, most frequently on a home-to-home basis. Almost 30% of clinicians reported not requiring informed consent for use of the modality. Consultations included children, adolescents, and adults in similar numbers; older adults were less frequent. Technological limitations were the most frequent reason for ruling out the modality with particular patients. Perceived benefits included the ability to continue consultations despite social distancing measures, lesser risk of COVID-19 infection, and the possibility of seeing patients with limited access to neuropsychological services. Reasons for not using TeleNP included a lack of standardized instruments, not feeling comfortable with the modality, and lack of technological resources and skills. CONCLUSIONS: Despite the socioeconomic differences between Mexico and high-income countries, most of our findings were similar to reports from those countries. However, technological limitations were common, and smartphones were commonly used, contrary to recommendations in the literature. The future use of TeleNP in Mexico should include formal training and ethical guidelines.

Neuropsychological intervention in attention and visuospatial skills in two patients with Williams syndrome with different types of genetic deletion.

Williams Syndrome (WS) is a neurodevelopmental disorder with a distinctive physical, cognitive, and behavioral profile caused by a microdeletion in the q11.23 region of chromosome 7. The neuropsychological profile of WS is characterized by intellectual disability, hypersociability, and deficits, especially in attention and visuospatial skills. Our objective was to assess the effectiveness of a neuropsychological intervention program in attention and visuospatial skills in two patients with WS (aged 7 and 13 years old) with different types of deletion (1.5 and 1.8 Mb). Cognitive, behavioral, and adaptive abilities were evaluated through various neuropsychological tests and scales; the neuropsychological intervention program was subsequently applied, and we assessed its effectiveness. Both patients initially presented significant deficits in attention and visuospatial skills. After the program, we found improvements in attention and visuospatial skills. In addition, both patients had significant clinical advances and changes in adaptive behaviors (social and self-care). These findings suggest that this intervention program could improve attention processes, visuospatial skills, and some aspects of adaptive behavior in patients with WS, regardless of deletion size. Although the sample was small, limiting the generalizability of the results, we believe this program could be a helpful resource for professionals working with individuals with WS.

A Systematic Review and a Latin American Clinical Model for Teleneuropsychological Assessment.

OBJECTIVE: The objective of this study is to propose a TeleNP model for remote assessment and offer practical recommendations for clinical practice with patients in Mexico and Latin America, based on a systematic literature review and clinical experience. METHOD: A systematic review of studies from 2011 to 2021 in English and Spanish used TeleNP, teleneuropsychology, telepsychology, online, assessment, teleneuropsicología, and evaluación for the search; the databases examined included PubMed, BiDi UNAM, ScienceDirect, Google Scholar, and Wiley One Library; the Oxford Centre for Evidence-Based Medicine system was used to grade the levels of evidence. The experience of the last two years of students and faculty in the Master's and Doctoral Programs in Psychology, Clinical Neuropsychology Residency Program, was also used as a basis for this guide. RESULTS: We propose a clinical model for TeleNP assessment in Mexico and Latin America based on the review of 31 articles and the practice of professors and students of clinical neuropsychology. CONCLUSION: The proposed model describes a procedure and adaptations for home-to-home clinical practice in the neuropsychological assessment of Mexican patients that could also be used in other Latin American countries. Its reliability remains to be assessed, but this model and the suggestions proposed could be used in future studies and clinical trials for Mexican and Latin American populations.

Neuropsychological Genotype-Phenotype in Patients with Williams Syndrome with Atypical Deletions: A Systematic Review.

Williams syndrome (WS) is a neurodevelopmental disorder caused by a microdeletion in the q11.23 region of chromosome 7. Recent case series reports and clinical case studies have suggested that the cognitive, behavioral, emotional, and social profile in WS could depend on the genes involved in the deletion. The objective of this systematic review was to analyze and synthesize the variability of the cognitive and behavioral profile of WS with atypical deletion and its probable relationship with the affected genes. The medical subject headings searched were "Williams syndrome," "genotype," "phenotype," "cognitive profile," and "atypical deletion." The studies included were in English or Spanish, with children and adults, and published between January 2000 and October 2022. Twenty-three studies are reported. The characteristics of the participants, the genes involved, the neuropsychological domains and instruments, and the prevalence of the WS cognitive profile criteria were used for the genotype-phenotype analysis. The genes with a major impact on the cognitive profile of WS were (a) LIMK1 and those belonging to the GTF2I family, the former with a greater influence on visuospatial abilities; (b) GTF2IRD1 and GTF2I, which have an impact on intellectual capacity as well as on visuospatial and social skills; (c) FZD9, BAZ1B, STX1A, and CLIP2, which influence the cognitive profile if other genes are also effected; and (d) GTF2IRD2, which is related to the severity of the effect on visuospatial and social skills, producing a behavioral phenotype like that of the autism spectrum. The review revealed four neuropsychological phenotypes, depending on the genes involved, and established the need for more comprehensive study of the neuropsychological profile of these patients. Based on the results found, we propose a model for the investigation of and clinical approach to the WS neuropsychological phenotype.

An Exploration of Social Cognition in Children with Different Degrees of Genetic Deletion in Williams Syndrome.

An explanation for the social dysfunction observed in Williams syndrome may be deficits in social cognition. This study explored aspects of social cognition in children with Williams syndrome with different genotypes. The 12 participants included one with a 1.1 Mb deletion that retained the GTF2IRD1, GTF2I, and GTF2IRD2 genes, seven with a 1.5 Mb deletion that preserved the GTF2IRD2 gene, and four with a 1.8 Mb deletion with loss of all three genes. The participant retaining all three genes was found to have better performance on social judgment and first-order theory of mind tasks than the group with loss of all three genes. These results may reflect the influence of the GTF2I gene family on social cognition in Williams syndrome.

Cognitive, Behavioral, and Adaptive Profiles in Williams Syndrome With and Without Loss of GTF2IRD2.

Williams syndrome (WS) is a neurodevelopmental disorder that results from a heterozygous microdeletion on chromosome 7q11.23. Most of the time, the affected region contains ~1.5 Mb of sequence encoding approximately 24 genes. Some 5-8% of patients with WS have a deletion exceeding 1.8 Mb, thereby affecting two additional genes, including GTF2IRD2. Currently, there is no consensus regarding the implications of GTF2IRD2 loss for the neuropsychological phenotype of WS patients. OBJECTIVES: The present study aimed to identify the role of GTF2IRD2 in the cognitive, behavioral, and adaptive profile of WS patients. METHODS: Twelve patients diagnosed with WS participated, four with GTF2IRD2 deletion (atypical WS group), and eight without this deletion (typical WS group). The age range of both groups was 7-18 years old. Each patient's 7q11.23 deletion scope was determined by chromosomal microarray analysis. Cognitive, behavioral, and adaptive abilities were assessed with a battery of neuropsychological tests. RESULTS: Compared with the typical WS group, the atypical WS patients with GTF2IRD2 deletion had more impaired visuospatial abilities and more significant behavioral problems, mainly related to the construct of social cognition. CONCLUSIONS: These findings provide new evidence regarding the influence of the GTF2IRD2 gene on the severity of behavioral symptoms of WS related to social cognition and certain visuospatial abilities. (JINS, 2018, 24, 896-904).