Progressive Supranuclear Palsy and Corticobasal Degeneration
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are commonly referred to as “atypical parkinsonian syndromes” or “frontotemporal dementias.” Clinically, they are characterized by an akinetic-rigid syndrome and cognitive impairment
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Progressive Supranuclear Palsy and Corticobasal Degeneration
F. Geser . S. W. Scholz . G. K. Wenning
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Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124
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Neuropathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125
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Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 Environmental Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 Tau Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128 Oxidative Stress . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 Neuroinflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131
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Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132
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Springer-Verlag Berlin Heidelberg 2007
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Progressive supranuclear palsy and corticobasal degeneration
Abstract: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are commonly referred to as ‘‘atypical parkinsonian syndromes’’ or ‘‘frontotemporal dementias.’’ Clinically, they are characterized by an akinetic‐rigid syndrome and cognitive impairment combined with a variety of so‐called ‘‘plus features.’’ On pathological grounds, their hallmarks are certain neurodegenerative lesion patterns coupled with tau protein‐positive intracellular protein aggregates in both neuronal and glial cells bodies or their processes. The pathogenesis of these disorders is not fully elucidated yet. Both genetic and environmental factors may be contributory. Here we review briefly the clinical presentation, comment on the neuropathology, and discuss in more detail the current state of pathogenetic concepts of these disorders with a special focus on recent genetic findings. List of Abbreviations: AD, Alzheimer’s disease; CBD, corticobasal degeneration; FTDP‐17, frontotemporal dementia with parkinsonism linked to chromosome 17; 3R‐tau, 3 repeat tau; 4R‐tau, 4 repeat tau; PD, Parkinson’s disease; PiD, Pick’s disease; PSP, progressive supranuclear palsy; TIQs, tetrahydroisoquinolines
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Clinical Presentation
In 1964, John Steele, Clifford Richardson, and Jerzy Olszewski were the first to describe progressive s
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