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Introduction: Intellectual disability (ID) is a neurodevelopmental disorder of etiologically diverse conditions starting in childhood characterised by impairments in intellectual and adaptive functioning. Although life expectancy is still lower compared with the general population, the number of people with ID growing old is increasing. Consequently, age-related mental health (MH) disorders, such as dementia, are becoming a common comorbidity of ID. Objectives: Description of the challenges related to the provision of appropriate healthcare for elderly persons with ID and MH disorders. Method: Review of the recent literature based upon a comprehensive search of medical databases using the keywords: intellectual disability, mental health, dementia, Alzheimer's disease, Down's syndrome and age. Results: ID is associated with a higher prevalence of physical and MH disorders across all ages, while clinical diagnostics and management of dementia are made complicated by the comorbidity rate. Elderly people with ID, especially those with Down's syndrome (DS), more commonly develop dementia than their age-matched counterparts without ID. For peoplewithDS and Alzheimer's disease (AD) the survival time was related to the age at diagnosis of AD, severity of ID, anti-dementia medication, history of epilepsy and living status. Conclusion: Elderly people with ID have greater early-onset multimorbidity and a prevalence of dementia which is up to five times higher compared to the elderly non-ID population. Their care is more challenging, and their diagnostics requires a comprehensive approach, including ID-appropriate neuropsychological evaluation. Specialist services for elderly persons with ID and MH disorders are, however, sparsely available and should be established. [ABSTRACT FROM AUTHOR]

Introduction: The 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome associated with multiple health manifestations such as intellectual disability (ID) or psychotic disorders. Delays in emotional functioning that are associated with ID may lead to difficulties with affect regulation and coping skills. There is scarce literature concerning the relevance of adapted behavioral interventions (ABI) for patients with ID and a comorbid psychotic disorder. Objectives: Relevance of ABI, evolved from the learning theory and cognitive behavioral therapy, in the treatment of a patient with 22q11.2DS focusing on the level of emotional functioning (LEF). Methods: Description of a three-year clinical course of a patient with 22q11.2DS, moderate ID, schizoaffective disorder and acute psychotic symptoms. In addition to a standard pharmacological treatment she received a long-term ABI, psychoeducation and systemic interventions in terms of skills-training, focusing on her affect regulation and emotional perception. The LEF was assessed at the beginning of the treatment and repeated annually using the scale of emotional development (SED), consisting of eight specific domains divided into six levels each. Results: The long-term ABI increased the LEF from Level 4 to 5 and smoothened the differences between the different aspects of emotional development as measured in the different domains of the SED, which led to sustainable benefits in her social participation. Conclusions: For this patient, ABI together with a standard pharmacological treatment improved the LEF considerably. Furthermore, the SED led to an effective therapeutic strategy and might also be helpful in the treatment of other patients with ID. [ABSTRACT FROM AUTHOR]

Introduction: The International Classification of Functioning, Disability and Health (ICF) provides a framework rooted in patientcentered care and the biopsychosocial model that facilitates a comprehensive description of a person's health and their level of societal participation. The importance of the ICF for assessing the needs of individuals with mental health problems (MHP) and intellectual disabilities (ID) is growing, especially in the social medicine. Objective: To describe the benefits and limitations of the ICF in clinical practice, pertaining to the assessment of healthcare needs and societal participation in persons with MHP and ID. Method: Comprehensive literature search in medical databases using the Keywords: ICF, mental health, intellectual disabilities, social and occupational participation. Results: ICF-based instruments such as the Mini-ICF-APP, with which impairments and competencies in social and occupational participation can be described, are playing an increasingly important role in healthcare and rehabilitation. In Germany, for example, in accordance with the Federal Participation Act, the entitlement to disability support benefits is assessed using ICF-based instruments, which therefore play a decisive role in social medical care. Conclusion: The functional descriptions of the ICF provide the opportunity for a standardized, yet individualized assessment of medical needs, general health and societal participation, thus facilitating the provision of a comprehensive package of care and support for people with disabilities. ICF-Core Sets and the Mini-ICF-APP are effective tools to describe level of function. It would be clinically valuable to further develop these instruments for use in persons with ID and MHP in the field of social medicine. [ABSTRACT FROM AUTHOR]

Introduction: Congruent with the UN Convention on the Rights of Persons with Disabilities, a new, specialized health care offer for persons with intellectual disability (ID) called "Medical treatment centres for adults with intellectual and severe multiple disabilities" (MZEB) has been established in Germany since 2015. MZEB adopt a multidisciplinary and holistic approach to patient care, focusing on comprehensive clinical diagnosis, treatment-planning and acute treatment. The MZEB evaluated has a psychiatric focus, specialising in the treatment of patients with ID and comorbid mental health issues. Objectives: The evaluation of effectiveness and relevance of MZEB treatment and its implications on parameters of quality of life (QoL) of persons with ID treated in MZEB. Methods: Over a period of nine weeks, 135 questionnaires were randomly assigned to persons with ID, who underwent treatment in the MZEB. The QoL was measured using a shortened version of the German adaptation of the World Health Organisation Quality of Life Questionnaire for People with ID (WHOQOL-Bref-ID). Results: A 60% response rate was attained. The MZEB was perceived as an important and necessary complement in the treatment of persons with ID. The QoL improved, especially regarding patients social participation. Moreover, patients health status improved substantially, leading to a better mental stability and reduced medication, particularly the use of psychopharmacological medication. Conclusions: The results show that MZEB provide an important contribution to improving patients QoL. Therefore, MZEB play with their specialized multidisciplinary and holistic approach to patient care a significant role in the health care of persons with ID and comorbid mental health issues. [ABSTRACT FROM AUTHOR]

Matrix metalloproteinases (MMPs) are involved in tissue repair, cell death and morphogenesis. We investigated the role of the gelatinases MMP-2 and MMP-9 in the pathogenesis of neuronal death induced by prolonged seizures in the developing brain. Seven-day-old rats, MMP-9 knockout mice and transgenic rats overexpressing MMP-9 received intraperitoneal injections of pilocarpine, 250 mg/kg, to induce seizures. After 6-72 h pups were sacrificed, tissue from different brain regions was isolated and expression of MMP-9 mRNA and protein was analyzed by real-time PCR or Western blot. Additionally, brains were fixed and processed for TUNEL-staining, immunohistochemistry and in situ zymography. We found increased numbers of TUNEL-positive cells 24 h after pilocarpine-induced seizures, most pronounced in cortical areas and the dentate gyrus, and less pronounced in thalamus. At 6-24 h, MMP-9 mRNA levels showed significant elevation compared to sham-treated controls; this effect resolved by 48 h, whereas MMP-2 mRNA levels remained stable. Cortical gelatinolytic activity, monitored by in situ zymography, was enhanced following pilocarpine-induced seizures. The MMP inhibitor GM 6001 ameliorated cell death following pilocarpine-induced seizures in infant rats. MMP-9 knockout mice were less susceptible to seizure-induced brain injury. Transgenic rats overexpressing MMP-9 were equally susceptible to seizure-induced brain injury as wild type rats. Our results suggest a significant contribution of MMP-9 to cell death after pilocarpine-induced seizures in the developing brain. As indicated by Western blot analysis, MMP-9 activation may be linked to activation of the Erk/CREB-pathway. The findings implicate involvement of MMP-9 in the pathophysiology of brain injury following seizures in the developing brain., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Matrix metalloproteinases (MMPs) play an essential role in tissue repair, cell death, and morphogenesis. The aim of the present study was to investigate potential involvement of selected MMPs in the pathogenesis of neuronal apoptosis induced by the NMDA antagonist MK-801 (dizocilpine) or the GABA(A) agonist phenobarbital in infant rats, transgenic rats overexpressing MMP-9 and MMP-9 knockout mice. Seven-day-old rats or knockout mice received intraperitoneal injections of MK-801, 1 mg/kg, or phenobarbital, 50 mg/kg. At different survival intervals following administration of the compounds (1-72 h), pups were sacrificed, tissue from different brain regions was isolated, and the expression and activity of MMP-2 and MMP-9 were analyzed by real-time PCR, western blot, and zymography. In addition, brains were fixed and processed for TUNEL staining. In all the brain regions analyzed, we found an increased number of TUNEL-positive cells 24 h after administration of MK-801. After treatment, we detected no significant increase in MMP-2 or MMP-9 mRNA expression in cortical areas. No changes in the MMP-9 protein expression or gelatinolytic activity of MMP-2 were observed in conjunction with MK-801 or phenobarbital-induced neuroapoptosis in any brain region analyzed. The extent of neurodegeneration induced by MK-801 or phenobarbital was not altered in MMP-9 transgenic rats and was increased in MMP-9 knockout mice compared to wild-type rats and mice. Treatment with the panmetalloproteinase inhibitor GM6001 did not confer protection against MK-801-induced apoptotic cell death in the developing rat brain. Our results suggest that activation of MMP-9 and MMP-2 does not contribute to pathogenesis of neuronal apoptosis caused by NMDA antagonists or GABA(A) agonists in the developing rat and mouse brain.