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Cornelia de Lange Syndrome (CdLS) is a genetic disorder characterized by different somatic impairments and retarded psychomotor development. 8-yearold male diagnosed with CdLS comes to Children' Psychiatry assesment presenting behavioural disorder as well as delayed motor and cognitive development. He cannot talk and only pictograms are used for communication. Severe cognitive rigidity, specially to manage changes and new contexts. In these cases, he shows aggressiveness against objects and self-injurious behaviour. A trial test of risperidone is started based on similarities betweeen these behavioral disorders and those present in autism spectrum. He shows a remarkable improvement, decreasing three points in the Clinical Global Impression. Scientific evidence mostly derives from case reports studies. That implies the quality of scientific evidence is low. Though the treatment with risperidone may be effective and well tolerated, more studies are required to move forward on the pharmacological treatment of behavioral disorders in CdLS. [ABSTRACT FROM AUTHOR]

Huntington's disease is the most frequent neurodegenerative disease with a prevalence of fewer than 10 cases per 10,000 inhabitants; the juvenile form is responsible for less than 10% of all cases. Huntington's disease belongs to the group known as "triad syndromes," which evolve with cognitive, motor and neuropsychiatric manifestations. Around 30% of patients debut with behavioral symptoms, which are a major challenge for management by patients, families, and caregivers. Huntington's disease (HD) is reviewed and a case of juvenile onset is reported in this article. The characteristics of juvenile-onset Huntington's disease (HD) differ from those of adult-onset HD, as chorea does not occur, although bradykinesia, dystonia, and signs of cerebellar disorder, such as rigidity, are present, frequently in association with convulsive episodes and psychotic manifestations. [ABSTRACT FROM AUTHOR]

En los últimos años ha aumentado la preocupación por mejorar la calidad de vida de las personas con discapacidad intelectual. Esta inquietud ha impulsado desde el año 2005 la aparición de centros específicos que atienden a personas con discapacidad intelectual ligera o inteligencia límite y trastornos de conducta y/o enfermedad mental. Sin embargo, apenas se contaba con datos sobre el perfil de personas atendidas, más allá de las amplias etiquetas de discapacidad intelectual ligera/límite y alteraciones de conducta. Diez años después de la creación de estos recursos, y con la intención de conocer el perfil del usuario y mejorar las estrategias de atención, se ha realizado una recogida de datos sistemática de numerosas variables en tres dispositivos: dos centros de día y un centro residencial, obteniéndose datos sociodemográficos de las historias clínicas de los usuarios atendidos, así como medidas pre y post de varias pruebas (ICAP, Escala de Calidad de Vida, Escala de Intensidad de Apoyos) que formaban parte del protocolo de evaluación de los centros. Se creó una base de datos intercentros para su posterior análisis estadístico, tanto a nivel descriptivo como en interacciones entre distintas variables. En total se ha analizado información de 122 personas de entre 18 y 40 años que han ocupado plaza en estos dispositivos entre los años 2006 y 2013. Los resultados ofrecen una visión global del perfil de usuarios atendidos, así como las características psicopatológicas y necesidades de apoyo que presentan y la relación entre estas, y se analizan las diferencias de perfiles atendidos en los distintos recursos. Los resultados apoyan la idoneidad de este tipo de servicios para atender a esta esta población, al ofrecer una atención más especializada, pero también muestran carencias existentes en este campo. Por último, se plantean otras líneas de investigación y trabajo de cara al futuro. [ABSTRACT FROM AUTHOR]

El bienestar mental es un componente fundamental de la definición de salud según la OMS que, unido al bienestar físico y social, facilita que la persona participe en la comunidad. Plena Inclusión en Castilla-La Mancha quiere generar, junto a la administración, un plan de acción que incida en la mejora de la calidad de vida de las personas con discapacidad intelectual que presentan alteraciones de conducta y/o problemas de salud mental. Se ha realizado un estudio en colaboración con la Universidad de Salamanca para conocer la prevalencia y la situación general de las personas que reciben apoyos en recursos de entidades federadas en Plena Inclusión C- LM. Este estudio ha servido de base para una posterior investigación acción a través del debate entre expertos del ámbito sanitario, educativo y social. La comunicación tiene como objetivo presentar las conclusiones de dicho debate formuladas en propuestas de mejora tanto para la prevención como la intervención con personas con discapacidad intelectual o del desarrollo, con el fin de obtener ideas para avanzar en un futuro plan de acción regional que permita sistematizar el trabajo de los diferentes profesionales en tres ámbitos: la prevención, la coordinación entre los servicios sanitarios y los sociales, y la intervención con personas con graves alteraciones de conducta. Este plan, ya realizado en otras comunidades autónomas, resultará clave para mejorar la calidad de vida de estas personas. Así mismo, se pretende iniciar un proyecto de transformación de servicios residenciales que incidirá en las alteraciones de conducta teniendo como base las buenas prácticas en el apoyo conductual positivo y el apoyo activo, junto a la coordinación con los servicios especializados en salud mental. [ABSTRACT FROM AUTHOR]

Introduction: Adenotonsillectomy is an effective treatment for sleep-disordered breathing in children, but its ability to resolve neurocognitive issues, is not clear., Objective: To analyze the outcomes of cognitive and behavioral disorders after one year of adenotonsillectomy., Method: We studied the behavioral and cognitive abnormalities in 45 children with obstructive sleep apnea and 30 healthy controls, aged 3 to 13 years. Psychological tests were performed in both groups at baseline and at 12 months., Results: Preoperatively, all cognitive and behavioral disturbances were higher in the study group than in the control group: attention in 46.7% of cases in the study group versus 20% in the control group (P=.016), anxiety 60.9% versus 40.9% (not significant); memory 55.6% versus 36.7% (P=.019), spatial structuring 64.4% versus 36.7% (P=.017), hyperactivity 42.9% versus 12.5% (P=.016), and attention deficit 46.4% versus 8.3% (P=.003). After one year we observed more disturbances in all variables in the study group. However, significant differences remained only in spatial structure (31.3% versus 3.3%, P=.017), and attention deficit (40.5% versus 16.7%, P=.031). The percentages of patients that improved in one year were not significantly different between both groups., Conclusion: Behavioral and cognitive disturbances in children with sleep apnea were partially resolved following adenotonsillectomy. Improvements in the cognitive and behavioral variables did not differ significantly from those the normal evolution of the individual, and were independent of the resolution of respiratory disorders., (Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

Introduction: The number of adoptions has increased in the Western world in recent decades. An over-representation in mental health services and an increased risk of displaying behavioural disorders has been reported among adopted adolescents. In this study, we aim to assess the proportions of adopted and non-adopted adolescents and the presence of externalizing disorders in an inpatient setting., Methods: Prospective data were collected over 56 months (n=431) from all adolescent admissions (12-17 years of age) to the Child and Adolescent's Inpatient Psychiatric Unit, which serves the Biscay province (Basque Country, Spain). The sample was divided into adopted (2.6%) and non-adopted adolescents (97.4%), and the following variables were compared: age, gender, cause of admission, prior admissions, main diagnosis, drug use and Overt Aggression Scale score at admission., Results: Adopted adolescents were over-represented in our sample (2.84% vs. 0.6% in the general population of Biscay, P<.001). Compared to the non-adopted, age at admission was significantly lower (14.09 vs. 15.21 years old, P=.017) and they showed a statistical tendency to be admitted more frequently for behavioural disorders (63.6% vs. 38.1%, P=.086)., Conclusions: The over-representation and the earlier age at admission suggest that the conditions of adopted adolescents are more serious and-or adoptive families are less able to manage them. The data also show that they tend to be admitted more for behavioural disorders, whilst no significant differences in the diagnosis on discharge were found, which suggests that they tend to externalise their symptoms more., (Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)