Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy

When Steele, Richardson and Olszewski described Progressive Supranuclear Palsy in Archives of Neurology in 1964, it was thought to be a rare disease. Recent pathological studies of large numbers of patients diagnosed as having Parkinson's disease in life have highlighted the fact that at least one in ten of such cases have some other condition. Progressive Supranuclear Palsy is one of the commonest alternative diagnoses. This book is therefore a timely review of present understanding of Progressive Supranuclear Palsy. Much has been learnt about this sporadic illness of middle and late life although its cause remains unknown, and its treatment continues to be difficult. The Editors have selected a team of authors who review the clinical aspects, neuro-imaging find ings, neuropathology, neurochemistry, epidemiology, and therapy of Progres sive Supranuclear Palsy. All are to congratulated on producing an excellent and detailed picture of contemporary knowledge of the condition. Anyone interested in Progressive Supranuclear Palsy cannot do better than to start by reading this book. It is to be hoped that it will prompt further investigation to establish its cause and cure. C. D. MARSDEN, London Preface Progressive Supranuclear Palsy (PSP) is a neurodegenerative disorder charac terized clinically by the appearance of a supranuclear gaze palsy and extra pyramidal features which include bradykinesia and axial dystonia. Postural instability and falls and frontal-type behavioural and cognitive disturbances complete the clinical picture of this progressive disorder.

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This monograph summarizes recent developments in clinical and basic research in progressive supranuclear palsy (PSP). This disorder is the second most common cause of parkinsonism seen in practice. Only recently we have been able to diagnose it regularly in living patients. There is an outporing of new interest in PSP in many research centers around the world and this volume is an expression of this research interest. Different clinical and basic subjects on PSP are covered in this monograph by individual experts in the field, incorporating their latest advances in research. With this book the reader will be able to have on hand the most recent advances on PSP covering areas such as epidemiology, neuropathology and neurophysiological, and neuroimaging aspects, as well as recent advances in neurochemistry, neuropathology and treatment of this disorder. (Special Edition of "Journal of Neural Transmission", Supplementum 42, 1994)

Contenu
Clinical aspects.- Historical notes.- Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).- Eyelid motor abnormalities in progressive supranuclear palsy.- he auditory startle response in progressive supranuclear palsy.- Differential diagnosis of PSP.- Cognitive disturbances in progressive supranuclear palsy.- Progressive supranuclear palsy and corticobasal ganglionic degeneration: differentiation by clinical features and neuroimaging techniques.- Neuroimage analysis, cerebral blood flow, and metabolism.- Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders.- Clinical progressive supranuclear palsy: differential diagnosis by IBZM-SPECT and MRI.- PET studies in progressive supranuclear palsy.- Morphological aspects.- The neuropathology of progressive supranuclear palsy.- Neurofibrillary pathology in progressive supranuclear palsy (PSP).- Antigenic determinant properties of neurofibrillary tangles. Relevance to progressive supranuclear palsy.- Cortical tangles in progressive supranuclear palsy.- Vascular progressive supranuclear palsy.- Biochemical aspects.- Cholinergic and peptidergic systems in PSP.- Brain monoamines in progressive supranuclear palsycomparison with idiopathic Parkinson's disease.- Alterations of neurotransmitter receptors and neurotransmitter transporters in progressive supranuclear palsy.- Changes in aminergic receptors in a PSP postmortem brain: correlation with pathological findings.- Epidemiology and treatment.- The epidemiology of PSP.- Cholinergic approaches to the treatment of progressive supranuclear palsy.- Therapy for progressive supranuclear palsy: past and future.