Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy
Progressive Supranuclear Palsy (PSP) is a neurodegenerative disorder characterized clinically by the appearance of a supranuclear gaze palsy and extrapyramidal features which include bradykinesia and axial dystonia. Postural instability and falls and frontal-type behavioral and cognitive disturbances complete the clinical picture of this progressive disorder. Although it is not an uncommon disorder in movement disorder clinics, due to its rarity and its protean clinical manifestations it is frequently diagnosed correctly years after the onset of symptoms and not infrequently confused with other diseases in which dementia or parkinsonism are prominent.
PSP was defined as a separate nosological disorder by Steele et al. in 1964 and subsequent publications on the subject have confirmed the essential clinical and pathological features outlined by these authors and made important contributions towards the characterization of the disorder. These new publications have emphasized the clinical heterogeneity of PSP, not all patients, for example, develop supranuclear gaze palsy and others show profound dementia, and also the not infrequent difficulties pathologists also have in diagnosing the disorder. Also, new information has emerged on the natural history and epidemiology of PSP and on its neuropathology, biochemistry, neurophysiology, and therapeutics. All authors in this volume are experts in different fields, basic or clinical, in the area of Movement Disorders, and their contributions review and add new information on all of the areas outlined above. This book will therefore update the reader on modern concepts regarding PSP.
This book is intended primarily for a clinical audience. Neurologists in general and, in particular, Movement Disorder specialists, who are faced with the difficult task of diagnosing and treating patients with PSP will hopefully find in the text information that will be of help in managing their patients. Neuropsychologists, pathologists, radiologists, and other professionals interested in new developments in the field of neurodegenerative disorders will also find in this book, we believe, valuable information.
We wish to thank each of the authors of this volume for the valuable personal time they have to spend writing their outstanding and timely contributions. We also wish to extend our thanks to the Fondo de Investigaciones Sanitarias de la Seguridad Social of the Spanish Ministry of Health for their help in organizing in Barcelona, in October of 1992, the symposium "Advances in Progressive Supranuclear Palsy". Many of the contributors to this book participated in this VIII Preface symposium, the first ever to be organized solely on PSP-related issues, reflecting the growing interest of the scientific community on this disorder.
Despite new developments PSP remains a progressively debilitating disorder for which we have no cure. We hope that this book will help early recognition and proper management of PSP and stimulate successful research that will soon enable us to dispose of an effective, curative treatment that we can offer our patients.
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