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Research on the multiple aspects of cognitive impairment in Down syndrome (DS), from genes to behavior to treatment, has made tremendous progress in the last decade. The study of congenital intellectual disabilities such as DS is challenging since they originate from the earliest stages of development and both the acquisition of cognitive skills and neurodegenerative pathologies are cumulative. Comorbidities such as cardiac malformations, sleep apnea, diabetes and dementia are frequent in the DS population, as well, and their increased risk provides a means of assessing early stages of these pathologies that is relevant to the general population. Notably, persons with DS will develop the histopathology of Alzheimer’s disease (formation of neuritic plaques and tangles) and are at high risk for dementia, something that cannot be predicted in the population at large. Identification of the gene encoding the amyloid precursor protein, its localization to chromosome 21 in the 90’s and realization that all persons with DS develop pathology identified this as an important piece of the amyloid cascade hypothesis in Alzheimer’s disease. Awareness of the potential role of people with DS in understanding progression and treatment as well as identification of genetic risk factors and also protective factors for AD is reawakening. For the first time since DS was recognized, major pharmaceutical companies have entered the search for ameliorative treatments, and phase II clinical trials to improve learning and memory are in progress. Enriched environment, brain stimulation and alternative therapies are being tested while clinical assessment is improving, thus increasing the chances of success for therapeutic interventions. Researchers and clinicians are actively pursuing the possibility of prenatal treatments for many conditions, an area with a huge potential impact for developmental disorders such as DS. Our goal here is to present an overview of recent advances with an emphasis on behavioral and cognitive deficits and how these issues change through life in DS. The relevance of comorbidities to the end phenotypes described and relevance of pharmacological targets and possible treatments will be considerations throughout.
Children living in poverty are more likely to have mental health problems, and their conditions are more likely to be severe. Of the approximately 1.3 million children who were recipients of Supplemental Security Income (SSI) disability benefits in 2013, about 50% were disabled primarily due to a mental disorder. An increase in the number of children who are recipients of SSI benefits due to mental disorders has been observed through several decades of the program beginning in 1985 and continuing through 2010. Nevertheless, less than 1% of children in the United States are recipients of SSI disability benefits for a mental disorder. At the request of the Social Security Administration, Mental Disorders and Disability Among Low-Income Children compares national trends in the number of children with mental disorders with the trends in the number of children receiving benefits from the SSI program, and describes the possible factors that may contribute to any differences between the two groups. This report provides an overview of the current status of the diagnosis and treatment of mental disorders, and the levels of impairment in the U.S. population under age 18. The report focuses on 6 mental disorders, chosen due to their prevalence and the severity of disability attributed to those disorders within the SSI disability program: attention-deficit/hyperactivity disorder, oppositional defiant disorder/conduct disorder, autism spectrum disorder, intellectual disability, learning disabilities, and mood disorders. While this report is not a comprehensive discussion of these disorders, Mental Disorders and Disability Among Low-Income Children provides the best currently available information regarding demographics, diagnosis, treatment, and expectations for the disorder time course - both the natural course and under treatment.
The Neurobiology of Aging and Alzheimer Disease in Down Syndrome provides a multidisciplinary approach to the understanding of aging and Alzheimer disease in Down syndrome that is synergistic and focused on efforts to understand the neurobiology as it pertains to interventions that will slow or prevent disease. The book provides detailed knowledge of key molecular aspects of aging and neurodegeneration in Down Syndrome by bringing together different models of the diseases and highlighting multiple techniques. Additionally, it includes case studies and coverage of neuroimaging, neuropathological and biomarker changes associated with these cohorts. This is a must-have resource for researchers who work with or study aging and Alzheimer disease either in the general population or in people with Down syndrome, for academic and general physicians who interact with sporadic dementia patients and need more information about Down syndrome, and for new investigators to the aging and Alzheimer/Down syndrome arena. - Discusses the complexities involved with aging and Alzheimer's disease in Down syndrome - Summarizes the neurobiology of aging that requires management in adults with DS and leads to healthier aging and better quality of life into old age - Serves as learning tool to orient researchers to the key challenges and offers insights to help establish critical areas of need for further research
Brain disordersâ€"neurological, psychiatric, and developmentalâ€"now affect at least 250 million people in the developing world, and this number is expected to rise as life expectancy increases. Yet public and private health systems in developing countries have paid relatively little attention to brain disorders. The negative attitudes, prejudice, and stigma that often surround many of these disorders have contributed to this neglect. Lacking proper diagnosis and treatment, millions of individual lives are lost to disability and death. Such conditions exact both personal and economic costs on families, communities, and nations. The report describes the causes and risk factors associated with brain disorders. It focuses on six representative brain disorders that are prevalent in developing countries: developmental disabilities, epilepsy, schizophrenia, bipolar disorder, depression, and stroke. The report makes detailed recommendations of ways to reduce the toll exacted by these six disorders. In broader strokes, the report also proposes six major strategies toward reducing the overall burden of brain disorders in the developing world.
CAMDEX-DS is a comprehensive assessment tool for diagnosing dementia in people with Down's syndrome (a group known to be at particular risk of dementia). Based upon CAMDEX-R, CAMDEX-DS has been modified for use with intellectual disability. In order to differentiate decline due to dementia or other mental disorders from pre-existing impairment, particular emphasis has been placed on establishing change from the individual's best level of functioning. The pack is comprised of a structured informant interview, a direct assessment of the patient/participant (CAMCOG), and guidance for diagnosis and post-diagnosis intervention, providing a reliable way to identify dementia (and to differentiate it from other common disorders). It may be used in community settings by mental health professionals as part of the diagnostic process, or to formalize diagnosis in the context of research. Use of the CAMCOG provides the means for ongoing neuropsychological evaluation and informs future support and intervention strategies.
This book reviews important neuropsychological measures currently used in the assessment of dementia by the principal clinicians and researchers associated with the test, offering practical guidance on each test along with an analysis of its limitations.
This handbook offers a comprehensive review of intellectual disabilities (ID). It examines historical perspectives and foundational principles in the field. The handbook addresses philosophy of care for individuals with ID, as well as parent and professional issues and organizations, staffing, and working on multidisciplinary teams. Chapters explore issues of client protection, risk factors of ID, basic research issues, and legal concerns. In addition, chapters include information on evidence-based assessments and innovative treatments to address a variety of behaviors associated with ID. The handbook provides an in-depth analysis of comorbid physical disorders, such as cerebral palsy, epilepsy and seizures, and developmental coordination disorders (DCD), in relation to ID. Topics featured in this handbook include: Informed consent and the enablement of persons with ID. The responsible use of restraint and seclusion as a protective measure. Vocational training and job preparation programs that assist individuals with ID. Psychological and educational approaches to the treatment of aggression and tantrums. Emerging technologies that support learning for students with ID. Key sexuality and relationship issues that are faced by individuals with ID. Effective approaches to weight management for individuals with intellectual and developmental disabilities. The Handbook of Intellectual Disabilities is an essential reference for researchers, graduate students, clinicians and related therapists and professionals in clinical child and school psychology, pediatrics, social work, developmental psychology, behavioral therapy/rehabilitation, child and adolescent psychiatry, and special education.