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.
Export Options
About this article
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 |
Call for Papers in Thematic Issues
New Advances in the Prevention, Diagnosis, Treatment, and Rehabilitation of Alzheimer's Disease
Aims and Scope: Introduction: Alzheimer's disease (AD) poses a significant global health challenge, with an increasing prevalence that demands concerted efforts to advance our understanding and strategies for prevention, diagnosis, treatment, and rehabilitation. This thematic issue aims to bring together cutting-edge research and innovative approaches from multidisciplinary perspectives to address ...read more
Alzheimer's Disease Drug Development
Alzheimer's disease is a progressive neurodegenerative disorder that affects millions of people worldwide. Despite decades of research, no cure or disease-modifying treatment is available yet. Therefore, the need for developing effective therapies to treat Alzheimer's disease is an urgent matter. This special issue aims to provide a comprehensive overview of ...read more
Current updates on the Role of Neuroinflammation in Neurodegenerative Disorders
Neuroinflammation is an invariable hallmark of chronic and acute neurodegenerative disorders and has long been considered a potential drug target for Alzheimer?s disease (AD) and dementia. Significant evidence of inflammatory processes as a feature of AD is provided by the presence of inflammatory markers in plasma, CSF and postmortem brain ...read more
Deep Learning for Advancing Alzheimer's Disease Research
Alzheimer's disease (AD) poses a significant global health challenge, with an increasing number of individuals affected yearly. Deep learning, a subfield of artificial intelligence, has shown immense potential in various domains, including healthcare. This thematic issue of Current Alzheimer Research explores the application of deep learning techniques in advancing our ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
5-HT1A Receptor, an Old Target for New Therapeutic Agents
Current Topics in Medicinal Chemistry Energy Metabolism, Adult Neurogenesis and their Possible Roles in Alzheimer’s Disease: A Brief Overview
Current Topics in Medicinal Chemistry Inflammatory Cyclooxygenase Activity and PGE<sub>2</sub> Signaling in Models of Alzheimer’s Disease
Current Immunology Reviews (Discontinued) Biological Function and Medicinal Research Significance of G-Quadruplex Interactive Proteins
Current Topics in Medicinal Chemistry Patterns of Self-Medication with Medicinal Plants and Related Adverse Events - A South American Survey
Current Drug Safety Gastric Inhibitory Polypeptide and its Receptor are Expressed in the Central Nervous System and Support Neuronal Survival
Central Nervous System Agents in Medicinal Chemistry Neuropeptides in Alzheimer’s Disease: An Update
Current Alzheimer Research Essential Roles of Intracellular Calcium Release Channels in Muscle, Brain, Metabolism, and Aging
Current Molecular Pharmacology A Review for Lithium: Pharmacokinetics, Drug Design, and Toxicity
CNS & Neurological Disorders - Drug Targets Molecular Biology, Pharmacology and Functional Role of the Plasma Membrane Dopamine Transporter
CNS & Neurological Disorders - Drug Targets The Endocrine Regulation of Stem Cells: Physiological Importance and Pharmacological Potentials for Cell-Based Therapy
Current Stem Cell Research & Therapy Nanoparticles, Neurotoxicity and Neurodegenerative Diseases
Current Drug Metabolism Lipid-Based Nanocarriers for CNS-Targeted Drug Delivery
Recent Patents on CNS Drug Discovery (Discontinued) Vascular Endothelial Growth Factor in Central Nervous System Injuries – A Vascular Growth Factor Getting Nervous?
Current Neurovascular Research Biophysics of Parkinsons Disease: Structure and Aggregation of α- Synuclein
Current Protein & Peptide Science Novel Non-Steroidal Anti-Inflammatory Drugs: What we have Learned from Animal Studies
Current Drug Targets - Inflammation & Allergy On the Search for Skin Gene Therapy Strategies of Xeroderma Pigmentosum Disease
Current Gene Therapy Neuroimaging of the Serotonin Transporter: Possibilities and Pitfalls
Current Psychiatry Reviews A Hyperlipidemic Diet Induces Structural Changes in Cerebral Blood Vessels
Current Neurovascular Research Models of Parkinson’s Disease with Special Emphasis on Drosophila melanogaster
CNS & Neurological Disorders - Drug Targets