Ethical Foundations of Palliative Care for Alzheimer Disease

Introduction: Historical Overview of a Current Global Challenge

m Introduction Historical Overview of a Current Global Challenge Henk A.M.J. ten Have, M.D., Ph.D., and Ruth B. Purtilo, Ph.D. Alzheimer disease (AD) was identified as a particular disease in 1910. The construct was rooted in the concepts and methods of psychiatry and neuropathology in nineteenth-century Germany. However, its existence was disputed from the time of its identification; in particular, it was controversial whether it was distinct from senile dementia and the normal processes of aging (Dillmann, 1990). The first case was reported by Alois Alzheimer (1864–1915) at a scientific meeting in 1906 in Tübingen, a famous university town in southern Germany.A fiftyone -year-old woman had unusual clinical symptoms that could not be classified within the existing nosological system. Starting with memory failures and an intense jealousy toward her husband, she soon became helpless and disoriented to time and place, with perception disorders and deliria. Her mental state deteriorated rapidly until she was fully bedridden, apathetic, and severely demented. Furthermore, the anatomical patterns found during autopsy were different from those of the usual disease processes. Specific changes in the brain cells were discovered , with tangled bundles of fibrils subsequently replacing the normal neurons , and multiple foci of deposits of a peculiar substance in the cerebral cortex were noted (later known as plaques). These typical clinical and anatomical observations were possible because of the particular conditions in which Alzheimer worked. He was director of the research laboratories of the newly built psychiatric clinic in Munich. This clinic was established (in 1904) and headed by Emil Kraepelin (1856–1926), one of the leaders of German psychiatry and a professor of psychiatry at the University of Munich. Alzheimer, after working with Kraepelin in Heidelberg, in 1902 moved with his teacher to Munich. Under the direction of Alzheimer, the laboratories developed into the leading research facilities for the neuropathological study of mental disorders. A school of psychiatric researchers was trained there, with Fritz Lewy, Alfons Jakob, and Hans Creutzfeld as disciples of Alzheimer. The focus of the laboratories was on neuropathology, using innovative techniques and instruments such as chemical staining and powerful microscopes to investigate the brains of deceased patients. At the same time, due to the efforts of Kraepelin , psychiatric patients were hospitalized in academic centers for diagnosis and research. This growth of institutional psychiatric care not only was a reaction against the dehumanizing conditions of Irrenanstalten (“lunatic asylums”), where patients were merely boarded, but it also was motivated by the need for care and possible cures. For Kraepelin, psychiatry as a medical discipline could emerge only when patients were observed and examined, in order to delineate particular diseases, combining clinical data (Kraepelin introduced a system of patient records) with autopsy findings (van Bakel, 2000). The interconnection of psychiatric care and neuropathological research was characteristic of German psychiatry for a long time (until 1930). Alzheimer was the first (in 1904) to describe the histopathological changes in the brains of patients with dementia paralytica. In 1907, Alzheimer published his case of the unusual brain disease. Three years later, G. Perusini, an assistant to Alzheimer, published a more extensive report, with four cases (including Alzheimer’s earlier one). Perusini did extensive pathological investigations of the brain. He concluded that the findings indicated a disease picture of a characteristic type: the clinical symptoms as well as neuropathology were similar to the changes in senile dementia, but progressed further and at an earlier stage, which he termed “the presenile age period.” Both publications motivated Kraepelin to proclaim the existence of a new disease named after Alzheimer. There was a particular set of clinical symptoms that could not be classified under a known clinical pattern. There was also a particular anatomical pattern, made visible with the latest staining techniques and microscopes, indicating particular chemical transformations . For Kraepelin, the clinical symptoms were distinctive and, therefore, 2 Introduction determinative in delineating this new disease. However, in Alzheimer’s point of view, specific neuropathological changes were considered basic and primary, the clinical symptoms secondary. This scientific view made it rather problematic to demarcate the clinical picture; since the disease basically could exist only when a particular neuropathology (especially neurofibrillary tangles and plaques) had been confirmed, the clinical diagnosis could be made only retrospectively. Finally, in the eighth edition of his widely read textbook, Kraepelin introduced the concept...