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New classification of tauopathies
Authors:G.U. Höglinger  G. Respondek  G.G. Kovacs
Affiliation:1. German Center for Neurodegenerative Diseases (DZNE), Feodor-Lynen Str. 17, D-81377 Munich, Germany;2. Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany;3. Klinisches Institut für Neurologie, Medizinische Universität Wien, Vienna, Austria
Abstract:Tauopathies are a group of neurodegenerative diseases characterized by pathological intracellular deposits of the protein tau. Isoform composition, morphology and anatomical distribution of cellular tau-immunoreactivities are defining distinct tauopathies as molecular pathological disease entities. The clinical spectrum of tauopathies includes syndromes with primary motor symptoms and with primary cognitive dysfunction. The traditional syndrome-based classification is currently being complemented by a molecular-pathological classification. While the syndrome-based classification is helpful to select symptomatic therapies, and to generate clinical working hypotheses about underlying etiologies, the molecular-pathological classification is most important for the development and application of molecularly tailored disease-modifying therapies.
Keywords:Tauopathies  Corticobasal degeneration  Corticobasal syndrome  Progressive supranuclear palsy  Richardson's syndrome  AGD  argyrophilic grain disease  ARTAG  aging-related tau astrogliopathy  bvFTD  behavioral variant of frontotemporal dementia  CBD  corticobasal degeneration  CBS  corticobasal syndrome  CTE  chronic traumatic encephalopathy  FTD  Frontotemporal dementia  FTLD  Frontotemporal lobar degeneration  FTLD-MAPT  Frontotemporal lobar degeneration associated with mutations in MAPT  GGT  Globular glial tauopathy  nfavPPA  Non-fluid agrammatic variant of primary progressive aphasia  NFT  Neurofibrillary tangles  PART  Primary age-related tauopathy  PGF  Primary gait freezing  PiD  Pick's disease  PPA  Primary progressive aphasia  PSP  Progressive supranuclear palsy  PSP-P  PSP with predominant Parkinsonism  svPPA  Semantic variant of primary-progressive aphasia  FTDP-17  frontotemporal dementia with Parkinsonism associated with chromosome-17
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