Atypical Parkinsonian Syndromes: Tauopathies
Abstract
Atypical parkinsonian disorders, which include two common proteinopathies, tauopathies and α-synucleinopathies, are clinically characterized by a progressive parkinsonism that typically does not respond to levodopa therapy and usually associates with early postural instability, falls, and other atypical features not observed in Parkinson disease. Tauopathies refer to neurodegenerative diseases in which there is an abnormal accumulation of hyperphosphorylated tau. The most frequent tauopathies are progressive supranuclear palsy and corticobasal degeneration. Better recognition of the expanding phenotypes of these disorders has led to the development of new diagnostic criteria. Furthermore, better knowledge about the pathogenesis (cell-to-cell transmission of pathologic tau) has resulted in advances in novel disease-modifying therapies that target tau. This review addresses the basic concepts of and recent issues in tauopathies, including their clinical phenotypes, genetic features, biomarkers, and novel experimental therapies. Key words: atypical parkinsonian disorders, corticobasal degeneration, progressive supranuclear palsy, proteinopathies, tauopathies
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