-
Part I: The Cases of Auguste D. and Johann F.: Origins of the Modern Concept of Alzheimer Disease
- Johns Hopkins University Press
- Chapter
- Additional Information
PART I The Cases of Auguste D. and Johann F. Origins of the Modern Concept of Alzheimer Disease As we enter a new millennium, neuroscience seems the most modern , forward-looking of endeavors in biomedicine, generating stunning insights into the molecular basis of brain function, which are then applied to problems of human behavior that have vexed humanity throughout history—problems such as age-associated progressive dementia. Over the past decade, research on Alzheimer disease (AD) has been on the cutting edge of the revolution in our understanding of the basic biologic mechanisms of the brain. Yet, however promising its prospects appear, neuroscience and the particular field of AD research cannot afford to neglect the study of its origins. As master historian of medicine and psychiatry Erwin Ackerknecht argued, the question of origins is not merely of academic interest . If any science is to chart new and reliable paths, it must continually reexamine its essential premises, which can best be understood by studying its origins (Ackerknecht and Vallois 1956). The two chapters in this section make an important contribution to our understanding of the origins of the concept of AD by providing newly uncovered historical evidence concerning the first two cases of the disease that Alzheimer described. Konrad Maurer’s chapter presents evidence from the clinical records and recently recovered brain tissue of Auguste D., the 51-year-old woman whose case Alzheimer first de- 2 Concepts of Alzheimer Disease scribed in 1907 (and which Gaetano Perusini described in greater detail in 1910). The clinical records were recovered by Maurer and his colleagues in Frankfurt in 1995, and the original histologic slides of Auguste D.’s brain that Alzheimer prepared were found by Hans-Jurgen Möller and his colleagues in Munich in 1998. In Chapter 2, Möller presents similar evidence concerning Johann F., the second case of the disease studied by Alzheimer and a crucial part of his important 1911 publication. Presenting this material in the context of the broader clinical and pathological work of Alzheimer, Perusini, Kraepelin, and others of this generation of pioneers, Maurer and Möller provide the reader with a thorough understanding of the clinical and pathological work that produced the modern concept of AD. These two cases remain interesting and informative puzzles even from the perspective of contemporary neuroscience. Although, as later chapters in this volume will document, tremendous progress has been made in the understanding of AD, the basic pathological features of the disease, which were identified almost a century ago, remain mysterious enough to be open to differing interpretations. Although the specific proteins of which both the tangle (tau) and the plaque (beta amyloid) are composed have been identified, it remains a point of contention among researchers as to which of these is more important in the pathogenesis of dementia—a debate that has been caricatured as a fight between two groups of religious zealots, the ‘‘Baptists’’ (proponents of the beta amyloid protein of neuritic plaques) and ‘‘Taoists’’ (proponents of the tau protein of neurofibrillary tangles). Although researchers are perhaps not ready to burn each other at the stake—as the amicable coexistence of both points of view in this volume demonstrates—the issue remains beyond the ability of current neuroscientific knowledge to resolve. The case of Johann F. is particularly interesting in connection with this issue because no neurofibrillary tangles were found in his brain, making it a case of ‘‘plaque-only AD.’’ Plaques had been described in the brains of demented individuals even before Alzheimer by Fischer and Redlich. Alzheimer is credited with the discovery of neurofibrillary tangles and with providing, in the case of Auguste D., an account that associated the clinical signs of dementia with both plaques and tangles. In this light, it might even seem appropriate to refer to plaque-only dementia as Fischer’s or Redlich’s disease, rather than as AD. And in more recent times, there has been considerable controversy about whether we should call plaque-only progressive degenerative dementia AD or restrict the term Alzheimer disease to those cases in which both pathologic signatures are found. Most neuropathologists believe it is possible to [34.228.7.237] Project MUSE (2024-03-19 02:58 GMT) Origins of the Modern Concept 3 label plaque-only and plaque-and-tangle dementia as the same entity, because other than this difference their pathological features are similar (i.e., neuronal loss and neurochemical changes). However, it is possible that...