Abstract
The primary progressive aphasias (PPA) are a group of clinically, genetically and pathologically heterogeneous neurodegenerative disorders caused by FTLD-tau, FTLD-TDP or Alzheimers disease pathology. Clinically, three subtypes are recognized, the semantic, logopenic and nonfluent variants but there remains ongoing discussions over how the clinical subtypes should be dissected. This review looks at the genetic and pathological basis of PPA and argues that with the advent of clinical trials in PPA, establishing the underlying pathology accurately during life will become increasingly important. Current and future biomarkers that may help make a pathological diagnosis in life, i.e. PPA-tau, PPA-TDP and PPA-AD, are reviewed including clinical and neuropsychological data, neuroimaging, blood and CSF markers.
Keywords: Primary progressive aphasia, progressive nonfluent aphasia, ntic dementia, logopenic aphasia, frontotemporal dementia, frontotemporal lobar degeneration, tau, TDP-43, progranulin, Mesulam, corticobasal syndrome, progressive supranuclear palsy, semantic variant, logopenic variant, nonfluent variant
Current Alzheimer Research
Title: Primary Progressive Aphasia-Defining Genetic and Pathological Subtypes
Volume: 8 Issue: 3
Author(s): J. D. Rohrer and J. M. Schott
Affiliation:
Keywords: Primary progressive aphasia, progressive nonfluent aphasia, ntic dementia, logopenic aphasia, frontotemporal dementia, frontotemporal lobar degeneration, tau, TDP-43, progranulin, Mesulam, corticobasal syndrome, progressive supranuclear palsy, semantic variant, logopenic variant, nonfluent variant
Abstract: The primary progressive aphasias (PPA) are a group of clinically, genetically and pathologically heterogeneous neurodegenerative disorders caused by FTLD-tau, FTLD-TDP or Alzheimers disease pathology. Clinically, three subtypes are recognized, the semantic, logopenic and nonfluent variants but there remains ongoing discussions over how the clinical subtypes should be dissected. This review looks at the genetic and pathological basis of PPA and argues that with the advent of clinical trials in PPA, establishing the underlying pathology accurately during life will become increasingly important. Current and future biomarkers that may help make a pathological diagnosis in life, i.e. PPA-tau, PPA-TDP and PPA-AD, are reviewed including clinical and neuropsychological data, neuroimaging, blood and CSF markers.
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Cite this article as:
D. Rohrer J. and M. Schott J., Primary Progressive Aphasia-Defining Genetic and Pathological Subtypes, Current Alzheimer Research 2011; 8 (3) . https://dx.doi.org/10.2174/156720511795563728
DOI https://dx.doi.org/10.2174/156720511795563728 |
Print ISSN 1567-2050 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5828 |
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