Since the original classic description of Parkinson's disease, there have been swings in concept from a unitary disease with characteristic clinical features and unique neuropathologic changes to that of a more variable disorder with multiple etiologies owing to a spectrum of pathological processes. Originally, the terms paralysis agitans and Parkinson's disease first used in the 19th century implied a unitary disease. The concept of parkinsonism as a special disease entity was supported by the stereotyped features of akinesia, rigidity, tremor, and postural instability. The appearance of postencephalitic parkinsonism and later the recognition of "arteriosclerotic parkinsonism" led to the realization that there must be multiple forms of the disease. Idiopathic Parkinson's disease finally became anchored by identification of the Lewy body, which provided the necessary objective marker for what, at least temporarily, quite remarkably came to be called "Lewy body disease." However, the later discovery that parkinsonism and dementia arise from a more diffuse distribution of Lewy bodies led to the designation of dementia with Lewy bodies, one of the first of the new generation of atypical parkinsonian disorders to be recognized. Striatonigral degeneration, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration soon followed and rapidly evolved from relatively exotic disorders to household words, at least among the rapidly growing community of movement disorder neurologists. Finally, more recently, even the unitary concept of idiopathic Parkinson's disease has been shaken by the discovery of multiple genetic types of Parkinson's disease that may occur with or without Lewy bodies!
Currently, patients are increasingly aware of the possibility of atypical parkinsonism. Many ask about it at early visits and understand their dismal prognosis and treatment prospects if they should have one of these disorders. Dr. Irene Litvan has been at the front line for many years in the effort to make sense of this bewildering array of atypical parkinsonian syndromes. In this volume she has brought together an impressive group of experts in the field. All aspects of these disorders are covered by highly knowledgeable and thoughtful investigators. Some of the clinical and scientific disciplines which are reviewed are clearly more mature than others, but it would be safe to say that our understanding of these disorders remains very much in its infancy. A particularly unique and very useful aspect of the book is that each chapter concludes with a section on future directions in research. As Dr. Litvan states in her preface, an important goal of this book is to enlist new researchers to further our knowledge about the cause and treatment of these devastating disorders. Atypical Parkinsonian Disorders: Clinical and Research Aspects will serve as the comprehensive reference to current state-of-the-art scientific developments in the field and will hopefully provide a launching pad for future fundamental discoveries in the pathophysiology and treatment of these currently hopeless disorders.
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