Felicidad, bienestar y puntos fuertes del carácter: resultados para familias y hermanos/as de personas con retraso mental

¿Qué se sabe de la felicidad y el bienestar y cómo promocionar del mejor modo estos estados positivos en el planeta? No suficiente, pero más que hace una década. Rompiendo la tradición con el enfoque habitual sobre lo que es malo para las personas, el creciente campo de la psicología positiva se pregunta en vez de eso qué contribuye a que la gente haga el bien y sea feliz, más allá y por encima de la mejora del sufrimiento psicológico. La psicología positiva pretende comprender más completamente estados positivos como la esperanza, la gratitud, satisfacción, fluidez, compromiso, virtudes, voluntad y significado. Aunque la psicología positiva es aún un campo en construcción, proporciona un andamiaje sobre el que comenzar a construir una psicología positiva para las personas con retraso mental (Dykens, en prensa). Se sabe muy poco sobre los estados internos positivos de aquellos con retraso mental. Dykens describió recientemente las estrategias para conceptuar y medir la felicidad, bienestar y sentido de la vida y propositividad en las personas con retraso mental, así como en sus familias y cuidadores. En este artículo, se encuentra la necesidad de ampliar el espectro de la investigación tradicional sobre familias y hermanos/as de personas con retraso mental

Social and emotional processing in Prader-Willi syndrome: genetic subtype differences

BACKGROUND: People with Prader-Willi syndrome (PWS) demonstrate social dysfunction and increased risk of autism spectrum disorder, especially those with the maternal uniparental disomy (mUPD) versus paternal deletion genetic subtype. This study compared the neural processing of social (faces) and nonsocial stimuli, varying in emotional valence, across genetic subtypes in 24 adolescents and adults with PWS. METHODS: Upright and inverted faces, and nonsocial objects with positive and negative emotional valence were presented to participants with PWS in an oddball paradigm with smiling faces serving as targets. Behavioral and event-related potential (ERP) data were recorded. RESULTS: There were no genetic subtype group differences in accuracy, and all participants performed above chance level. ERP responses revealed genetic subtype differences in face versus object processing. In those with deletions, the face-specific posterior N170 response varied in size for face stimuli versus inverted faces versus nonsocial objects. Persons with mUPD generated N170 of smaller amplitude and showed no stimulus differentiation. Brain responses to emotional content did not vary by subtype. All participants elicited larger posterior and anterior late positive potential responses to positive objects than to negative objects. Emotion-related differences in response to faces were limited to inverted faces only in the form of larger anterior late positive potential amplitudes to negative emotions over the right hemisphere. Detection of the target smiling faces was evident in the increased amplitude of the frontal and central P3 responses but only for inverted smiling faces. CONCLUSION: Persons with the mUPD subtype of PWS may show atypical face versus object processes, yet both subtypes demonstrated potentially altered processing, attention to and/or recognition of faces and their expressions

Genética y fenotipo conductual en la discapacidad intelectual: su aplicación a la cognición y a la conducta problemática (2ªParte)

El artículo examina en primer lugar las conexiones entre trastornos genéticos y conducta. Después de describir cómo los trastornos genéticos predisponen a pensar que una conducta concreta guarda relación con la etiología, revisa brevemente el estado de la cuestión en el campo de la investigación sobre la conducta en diversas formas de discapacidad intelectual y ofrece algunos principios generales sobre cómo los genes afectan a la conducta. Después expone dos ejemplos, sobre la psicología y conductas maladaptativas, y sobre los puntos fuertes y débiles de la cognición, que ilustrarán los enfoques que, basados en la etiología, daremos para entender la conducta de las personas con discapacidad intelectual

The effect of intellectual ability on functional activation in a neurodevelopmental disorder: preliminary evidence from multiple fMRI studies in Williams syndrome

BACKGROUND: Williams syndrome (WS) is a rare genetic disorder caused by the deletion of approximately 25 genes at 7q11.23 that involves mild to moderate intellectual disability (ID). When using functional magnetic resonance imaging (fMRI) to compare individuals with ID to typically developing individuals, there is a possibility that differences in IQ contribute to between-group differences in BOLD signal. If IQ is correlated with BOLD signal, then group-level analyses should adjust for IQ, or else IQ should be matched between groups. If, however, IQ is not correlated with BOLD signal, no such adjustment or criteria for matching (and exclusion) based on IQ is necessary. METHODS: In this study, we aimed to test this hypothesis systematically using four extant fMRI datasets in WS. Participants included 29 adult subjects with WS (17 men) demonstrating a wide range of standardized IQ scores (composite IQ mean = 67, SD = 17.2). We extracted average BOLD activation for both cognitive and task-specific anatomically defined regions of interest (ROIs) in each individual and correlated BOLD with composite IQ scores, verbal IQ scores and non-verbal IQ scores in Spearman rank correlation tests. RESULTS: Of the 312 correlations performed, only six correlations (2%) in four ROIs reached statistical significance at a P value < 0.01, but none survived correction for multiple testing. All six correlations were positive. Therefore, none supports the hypothesis that IQ is negatively correlated with BOLD response. CONCLUSIONS: These data suggest that the inclusion of subjects with below normal IQ does not introduce a confounding factor, at least for some types of fMRI studies with low cognitive load. By including subjects who are representative of IQ range for the targeted disorder, findings are more likely to generalize to that population

Visuo-spatial cognition in Williams syndrome: Reviewing and accounting for the strengths and weaknesses in performance

Individuals with Williams syndrome typically show relatively poor visuo-spatial abilities in comparison to stronger verbal skills. However, individuals' level of performance is not consistent across all visuo-spatial tasks. The studies assessing visuo-spatial functioning in Williams syndrome are critically reviewed, in order to provide a clear pattern of the relative difficulty of these tasks. This prompts a possible explanation of the variability in performance seen which focuses on the processing demands of some of these tasks. Individuals with Williams syndrome show an atypical processing style on tests of construction, which does not affect tests of perception

Prader–Willi syndrome and autism spectrum disorders: an evolving story

Prader–Willi syndrome (PWS) is well-known for its genetic and phenotypic complexities. Caused by a lack of paternally derived imprinted material on chromosome 15q11–q13, individuals with PWS have mild to moderate intellectual disabilities, repetitive and compulsive behaviors, skin picking, tantrums, irritability, hyperphagia, and increased risks of obesity. Many individuals also have co-occurring autism spectrum disorders (ASDs), psychosis, and mood disorders. Although the PWS 15q11–q13 region confers risks for autism, relatively few studies have assessed autism symptoms in PWS or directly compared social, behavioral, and cognitive functioning across groups with autism or PWS. This article identifies areas of phenotypic overlap and difference between PWS and ASD in core autism symptoms and in such comorbidities as psychiatric disorders, and dysregulated sleep and eating. Though future studies are needed, PWS provides a promising alternative lens into specific symptoms and comorbidities of autism

Electrophysiological study of local/global processing in Williams syndrome

Persons with Williams syndrome (WS) demonstrate pronounced deficits in visuo-spatial processing. The purpose of the current study was to examine the preferred level of perceptual analysis in young adults with WS (n = 21) and the role of attention in the processing of hierarchical stimuli. Navon-like letter stimuli were presented to adults with WS and age-matched typical controls in an oddball paradigm where local and global targets could appear with equal probability. Participants received no explicit instruction to direct their attention toward a particular stimulus level. Behavioral and event-related potential (ERP) data were recorded. Behavioral data indicated presence of a global precedence effect in persons with WS. However, their ERP responses revealed atypical brain mechanisms underlying attention to local information. During the early perceptual analysis, global targets resulted in reduced P1 and enhanced N150 responses in both participant groups. However, only the typical comparison group demonstrated a larger N150 to local targets. At the more advanced stages of cognitive processing, a larger P3b response to global and local targets was observed in the typical group but not in persons with WS, who instead demonstrated an enhanced P3a to global targets only. The results indicate that in a perceptual task, adults with WS may experience greater than typical global-to-local interference and not allocate sufficient attentional resources to local information

Felicidad, bienestar y puntos fuertes del carácter: resultados para familias y hermanos/as de personas con retraso mental

¿Qué se sabe de la felicidad y el bienestar y cómo promocionar del mejor modo estos estados positivos en el planeta? No suficiente, pero más que hace una década. Rompiendo la tradición con el enfoque habitual sobre lo que es malo para las personas, el creciente campo de la psicología positiva se pregunta en vez de eso qué contribuye a que la gente haga el bien y sea feliz, más allá y por encima de la mejora del sufrimiento psicológico. La psicología positiva pretende comprender más completamente estados positivos como la esperanza, la gratitud, satisfacción, fluidez, compromiso, virtudes, voluntad y significado. Aunque la psicología positiva es aún un campo en construcción, proporciona un andamiaje sobre el que comenzar a construir una psicología positiva para las personas con retraso mental (Dykens, en prensa). Se sabe muy poco sobre los estados internos positivos de aquellos con retraso mental. Dykens describió recientemente las estrategias para conceptuar y medir la felicidad, bienestar y sentido de la vida y propositividad en las personas con retraso mental, así como en sus familias y cuidadores. En este artículo, se encuentra la necesidad de ampliar el espectro de la investigación tradicional sobre familias y hermanos/as de personas con retraso mental