Dementia and Aging Adults with Intellectual Disabilities

A Handbook

Matthew P. Janicki, Arthur J. Dalton, Matthew P. Janicki, Arthur J. Dalton

• Publisher: Routledge
• Year: 2014
• Print ISBN: 9780876309162
• eBook ISBN: 9781317823810
• Topic: Psychology
• Subtopic: Mental Health in Psychology
• Index: Psychology

This definitive handbook assembles the most recent advances in knowledge about dementia, Alzheimer Disease, and related disorders as they affect persons with intellectual disabilities. Diagnosis, assessment, treatment, and management and care practices are detailed in a practical manner making this a useful tool to both students and trained professionals. After an introduction to the subject, the book begins with persoanl accounts of three affected individuals whose signs of dementia are described from clinical, family member, and care-provider perspectives, respectively. The biology and physiology of dementia, as well as the neurological and medical complications associated with it, are then provided in Parts Two, three, and Four. The application and practical perspectives of this handbook are enhanced in Part Five which details the best practices available to meet the needs and challenges involved in care and quality of life issues. The challenge raised by the rapidly growing number of aging individuals with intellectual disabilities forms the basis for the final part of the volume, an analysis and presentation of rarely addressed policy issues. Extensive resource information and a comprehensive glossary contribute to the useful nature of this handbook. Practitioners, service providers, educators and students will benefit from the accessability and practicality if this text as well as the breadth and depth of knowledge of the editors and contributors.

Part 1
Introduction

The three chapters in this section provide varying perspectives on the changes in behavior, personality, and physical and mental health that occur in individuals with Down syndrome affected by Alzheimer's disease. Each serves as a dramatic anchor linking the rest of the chapters in this volume. First, after briefly defining the terms aging, dementia, and Alzheimer's disease, the authors of the opening chapter review alternative current research hypotheses, which have implications for the potential treatment of Alzheimer's disease, namely, the role of beta amyloid, oxidative damage, and inflammatory mechanisms. The authors then introduce the notions of staging of the onset and progression of the disease, and the problem of diagnosis with a particular emphasis on medical and other conditions that may accompany Alzheimer's disease as an associated or comorbid condition. Since these notions appear and reappear throughout the volume in different guises, this chapter sets the tone for the analyses and commentaries that follow and emphasizes their central importance in meeting the challenges of dementia and aging in persons with intellectual disabilities. The bulk of the chapter is then devoted to a detailed presentation of RM, a woman with Down syndrome and dementia, that provides a vivid clinical picture of the broad array of complex issues that must be addressed on a case-by-case basis when offering services to meet the needs of affected individuals.

The authors of the second chapter were professionally involved on a daily basis with the care and management of Jan, a woman with Down syndrome, whose onset of dementia was first noted at the age of 47 and who quickly deteriorated over a period of two years, dying at the age of 49 of complications arising from respiratory disease. It is a gripping chronology of deterioration and suffering, which the authors observed in a woman whom they knew as a happy, sociable, nearly independent person prior to her illness. The narrative only indirectly reveals the intensity of the feelings of anxiety and affection which were felt by the care provider staff who was involved with Jan on a daily basis. One year before Jan's death, they were helpless to stop the disease and could only provide palliative care, a situation that is presently inescapable because of the absence of any known effective treatment for Alzheimer's disease. The heartbreaking story brings all of us closer to the human drama of Alzheimer's disease.

The third chapter is authored by a father who, along with his wife, spent a lifetime of love, devotion, and attention to their son, David, who had Down syndrome. Their story only begins to touch on some of the milestones that are particularly memorable to the family. Yet, it is possible to see, between the lines, a family's quiet resolution and determination to raise a child with Down syndrome from his infancy, childhood, and adolescence to his adulthood and older age. Their story will no doubt sound a familiar note for many, if not all, families of this generation who are raising an adult child with Down syndrome who was born 40 to 50 years ago. The problems and frustrations that parents faced and successfully solved in the early fifties to obtain and provide schooling for children and then find appropriate activities, jobs, and other occupations for them as adults, can be traced in the story of David. Similarly, the almost ubiquitous problems of finding good care from a medical community which was mostly ignorant of the unique problems of aging individuals with Down syndrome are evident when David reaches middle age. The struggles were renewed again when the parents had to deal with a medical community which was mostly ignorant of the connection between Alzheimer's disease and Down syndrome. The parents were left with very little support in coping with the declining functions of their son. They offer us much to contemplate with regard to how we can begin to provide aging-associated services to this generation of parents. Their story is emotionally compelling even though it is presented in an understated fashion.

Chapter 1
Aging and Dementia

Arthur J. Dalton
Matthew P. Janicki

Aging, dementia, and Alzheimer's disease are defined carefully to ease the task of identifying their relationships to older individuals with intellectual disabilities. Known risk factors, current mechanisms underlying the development of Alzheimer's disease, and a three-stage model of the progression of dementia of the Alzheimer type are briefly described. The three-stage model is used as the framework for understanding the detailed clinical presentation and management practices utilized for RM, a woman with Down's syndrome who was systematically followed for a period of 20 years while she was alive with a confirmed postmortem neuropathological diagnosis of Alzheimer's disease. References to relevant chapters in this volume are also liberally interspersed throughout the account of RM. These citations provide the reader with current knowledge bearing on many aspects of the management and care of affected individuals with intellectual disabilities.

Aging is a progressive, predictable process that involves the evolution and maturation of living organisms; it is evitable but varies greatly in terms of rate among individuals (Williams, 1995). From the perspective of the biological sciences, aging is defined as a cumulative, universal, progressive, internal, and deleterious set of processes that begin at the moment of conception and that are active until the death of the individual (Machemer, 1993). The processes of aging are unique for each species and they are genetically determined. It is essential to distinguish universal aging processes from age-associated diseases and disorders such as dementia and Alzheimer's disease, which appear in some, but not all individuals, in late life. Dementia represents an acquired loss of intellectual ability that occurs over a long period of time and affects many areas of cognitive functioning (Williams, 1995). Prior to the early 20th century the term dementia was used interchangeably with the word senility. Senility was then considered a normal consequence of aging, which included all the physical and mental infirmities that can affect the elderly (Hendrie & Hingtgen, 1990). Gradually, the growing realization became more widespread that the degenerative changes were due to disease processes rather than to normal aging. This changing view was finally accepted by the medical community by the mid-20th century. A detailed analysis of the term applied to individuals with intellectual disabilities is provided by Burt and Aylward (this volume) and some of the complexities of using the term in the context of psychiatric disorders is illustrated by Burt (this volume). A glossary at the end of the volume provides six other definitions of the term that are currently in use among professionals involved in the field of intellectual disabilities.

For the first half of this century, Alzheimer's disease was a term reserved to describe "senility" in the presenium, that is, the severe mental and personality deterioration that occurred at ages younger than 60 years. The term, like the word dementia, has also undergone a number of transformations since then. It is now applied to older adults of any age who show a pattern of progressive mental deterioration coupled with personality changes in a state of clear consciousness associated with a specific configuration of neuropathological changes in the brain (Reisberg, Ferris, & DeLeon, 1989; Berg, Karlinsky, & Holland, 1993). A glossary at the end of this text provides additional definitions which distinguish familial from sporadic forms of the disease. The cause(s), treatment, and prevention of this disease are presently not known. It affects about 10% of the general aging population over 65 years of age and represents a significant risk factor for all persons with Down's syndrome over the age of 40 years or so.

□ Prevalence and Incidence of Alzheimer's Disease

The exact prevalence of Alzheimer's disease among adults with intellectual disability is unknown. For adults with Down's syndrome, recent research suggests that the neuropathological features of Alzheimer's disease may be much more common in this group than previously suspected (Wisniewski, Silverman, & Wegiel, 1994) and, consequently, dementia may also be more common. Zigman, Schupf, Haveman and Silverman (1995; 1977) in a comprehensive review of the epidemiological literature concluded that the age-specific prevalence rate of Alzheimer's dementia among adults with Down syndrome was consistently high, even though estimates of age-specific prevalence varied widely across studies. They attributed much of this variability to divergent sampling techniques and subject populations, varying assessment instruments, and nonstandardized diagnostic criteria. For those adults with intellectual disabilities other than Down's syndrome, the prevalence rates are not yet determined and studies show varying rates. For example, Cooper (1997) noted that dementia occurs at a much higher rate among older adults with intellectual disability than it does in the general population. In a population survey. Cooper found 22% of persons aged 65 and older with dementia. In contrast, others have noted rates equivalent to those of the general population. Haveman, Maaskant and Sturmans (1989) found dementia occurring in 10% of older adults with intellectual disability. Janicki and Dalton (1997; 1998) found dementia occurring in 6% of adults aged 60 and older and 12% of adults aged 80 and older.

□ Neuropathology of Alzheimer's Disease

The loss of millions of brain cells is the most dramatic physical evidence of Alzheimer's disease. The processes whereby these nerve cells die in such numbers and the significance of the selective losses in particular regions (such as the nucleus basalis) and the death of specific types of nerve cells are not known. The search for infectious or transmissible agents, genetic or inherited abnormalities, and environmental toxins has yielded a large and important body of knowledge, but no specific cause(s) for Alzheimer's disease has been established. No infectious or viral agent has yet been identified and there have been no reported instances of one affected person transmitting the disease to any other. The neurofibrillary tangles and the senile plaques which are found in widespread regions of the brains of affected individuals have been the subject of intense research over many years (see recent reviews by Iqbal et al„ 1993; Wisniewski & Wisniewski, 1992) and data from such studies hold promise for the development of potential biomarkers useful in diagnosis and rational approaches to drug therapies. Unfortunately, it is not clear whether the neurofibrillary tangles or the senile plaques or both are causes of Alzheimer's disease or whether they simply represent the debris or consequences of some as yet undetermined process or unidentified causative event.

Neurofibrillary tangles are bundles of filaments in the cytoplasm of the neuron that displace or encircle the neuron. Immunocytochemical studies suggest that when the neuron is still intact, a major surface antigen of the tangles is phosphorylated tau protein. They are stained strongly by silver (e.g., Bielchowsky methods). They are a characteristic feature of Alzheimer's disease, but tangles in relatively small numbers appear in the brain tissues of elderly individuals with no apparent dementia. Senile plaques are areas of incomplete degeneration in the brains of elderly individuals or person with Alzheimer's disease. These are focal collections of tortuous silver-staining processes. Microglial cells and reactive astrocytes can be seen around the periphery. Early plaques have only neuritic processes (remnants of axons and/or dendrites), but later in evolution they develop a central amyloid core around which a clear halo tends to form. These are often present in the brains of aging individuals without signs of dementia while alive.

The possibility of a genetic basis for Alzheimer's disease has received a significant boost since the discovery of a location on chromosome 21 for the genetic blueprint for an important protein associated with deposits of beta-amyloid in senile plaques and blood vessels of the brain (Robakis et al., 1987). While this gene may lead to the over-production of betaamyloid, there is no proof that it causes Alzheimer's disease. Understanding of the molecular events which are involved in the production, breakdown, modification, and regulation of beta amyloid may yield important new treatment strategies.

□ Risk Factors for Alzheimer's disease

Besides age, the presence of trisomy 21 is the only well-established risk factor for Alzheimer's disease. For more than 60 years since Struwe's first report (Struwe, 1929), all reports of postmortem examinations of brain tissue specimens from virtually all individuals with Down's syndrome who have died after the age of 40 years show the characteristic lesions of Alzheimer's disease. Other genetically based risk factors have been suggested. Variants of some genes located on chromosome 19, 14, and 1 and specific point mutations in two mitochondrial genes raise the possibility that they contribute to the pathology of Alzheimer's disease. Laboratory, clinical, and epidemiological studies have also identified a number of environmental risk factors that may predispose persons to the development of Alzheimer's disease, including head injury, depression, hypothyroidism, poor nutrition, and neurotoxic environmental agents.

A review of these possibilities and a critical examination of the literature are provided elsewhere (Percy, this volume). Two excellent reviews with extensive references to the literature dealing with the long-standing controversy about the role of the salts of "trace metals/' such as aluminum and iron, in Alzheimer's disease have been recently published (Markesbery & Ehmann, 1994; McLachlan, 1995).

Amyloid Precursor Protein (APP). Several observations firmly link Alzheimer's disease to the gene for APP. One of the most characteristic changes in the Alzheimer's disease brain is the accumulation of amyloid plaques. Amyloid (3 peptide (A(3), a product of the metabolism of APP, is the major component of the plaques. A(3 is a 39-43 amino acid peptide resulting from cleavage of APP that forms the core of the amyloid plaques in the Alzheimer's disease hippocampus and neocortex. An inflammatory reaction, including activated microglia and astrocytes, with increased levels of inflammatory cytokines, acute phase proteins, complement proteins, and cyclooxygenase, accompanies the dystrophic neurites around mature plaques.

In certain families with ...

Table of contents

• Cover
• Half Title
• Title
• Copyright
• Dedication
• Contents
• Contributors
• Foreword
• Preface
• Acknowledgments
• PART 1. INTRODUCTION
• PART 2. BIOMEDICAL CONSIDERATIONS
• PART 3. ASSESSMENT CONSIDERATIONS
• PART 4. CLINICAL CONSIDERATIONS
• PART 5. PROGRAM CONSIDERATIONS
• PART 6. EDUCATION AND POLICY CONSIDERATIONS
• Appendix 1: AAMR/IASSID Practice Guidelines for Diagnosis and Care Management of Adults with Intellectual Disability and Dementia
• Appendix 2: Guidelines for Coping with Alzheimer's Disease in Persons with Down's Syndrome
• Appendix 3: Instruments and Tests
• Glossary
• Index