Abstract
Progressive Supranuclear Palsy (PSP) has been used to denote a unifying disorder with progressive parkinsonism with early falls, vertical supranuclear gaze palsy, pseudobulbar dysfunction and cognitive decline. Over the last decade, heterogeneity of the disease into different clinical subtypes has been recognized in clinicopathological studies. Although neuroimaging features and laboratory findings may support the diagnosis, true biomarkers are still lacking in the clinical setting. Neuronal and glial tau positive aggregates are predominantly found in basal ganglia and brainstem, and the significant association of PSP with the common H1 tau haplotype likely points to a pathophysiological role of the tau protein in the disease process. Future genetic studies of familial cases and an ongoing genome-wide association study of large series of pathological-proven cases may reveal additional genetic factors in the near future.
Keywords: Progressive supranuclear palsy, review, diagnosis, neuropathology, genetics, Parkinson's disease, MAPT, hallucinations, tremor, dysautonomia, GABAergic cell loss, striatum, cholinergic, dopaminergic cell loss
Current Alzheimer Research
Title: Recent Advances in Progressive Supranuclear Palsy: A Review
Volume: 8 Issue: 3
Author(s): L. D. Kaat, W. Z. Chiu, A. J.W. Boon and J. C. van Swieten
Affiliation:
Keywords: Progressive supranuclear palsy, review, diagnosis, neuropathology, genetics, Parkinson's disease, MAPT, hallucinations, tremor, dysautonomia, GABAergic cell loss, striatum, cholinergic, dopaminergic cell loss
Abstract: Progressive Supranuclear Palsy (PSP) has been used to denote a unifying disorder with progressive parkinsonism with early falls, vertical supranuclear gaze palsy, pseudobulbar dysfunction and cognitive decline. Over the last decade, heterogeneity of the disease into different clinical subtypes has been recognized in clinicopathological studies. Although neuroimaging features and laboratory findings may support the diagnosis, true biomarkers are still lacking in the clinical setting. Neuronal and glial tau positive aggregates are predominantly found in basal ganglia and brainstem, and the significant association of PSP with the common H1 tau haplotype likely points to a pathophysiological role of the tau protein in the disease process. Future genetic studies of familial cases and an ongoing genome-wide association study of large series of pathological-proven cases may reveal additional genetic factors in the near future.
Export Options
About this article
Cite this article as:
D. Kaat L., Z. Chiu W., J.W. Boon A. and C. van Swieten J., Recent Advances in Progressive Supranuclear Palsy: A Review, Current Alzheimer Research 2011; 8 (3) . https://dx.doi.org/10.2174/156720511795563809
DOI https://dx.doi.org/10.2174/156720511795563809 |
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
-
Multi-Target Compounds Acting in the Central Nervous System Designed From Natural Products
Current Medicinal Chemistry Beneficial Effects of Herbs, Spices and Medicinal Plants on the Metabolic Syndrome, Brain and Cognitive Function
Central Nervous System Agents in Medicinal Chemistry Current Options and Future Possibilities for the Treatment of Dyskinesia and Motor Fluctuations in Parkinson's Disease
CNS & Neurological Disorders - Drug Targets Cognition and Neuropathology in Aging: Multidimensional Perspectives from the Rush Religious Orders Study and Rush Memory and Aging Project
Current Alzheimer Research Hyperglycemia-induced Oxidative Stress and its Role in Diabetes Mellitus Related Cardiovascular Diseases
Current Pharmaceutical Design Drugs to Treat Neuroinflammation in Neurodegenerative Disorders
Current Medicinal Chemistry Cholinesterase Inhibitory Activities of N-Phenylthiazol-2-Amine Derivatives and their Molecular Docking Studies
Medicinal Chemistry Misfolded Proteins and Human Diseases
Protein & Peptide Letters (Pro)renin Receptor as a New Drug Target
Current Pharmaceutical Design Ceramide Involvement in Apoptosis and Apoptotic Diseases
Mini-Reviews in Medicinal Chemistry The prediction of response to Galantamine treatment in Patients with mild to moderate Alzheimer’s Disease
Current Alzheimer Research A Systematic Approach to Hypertensive Urgencies and Emergencies
Current Hypertension Reviews Oxidative/Nitrosative Brain Damage in Stress: Possible Target for Neuropsychopharmacological Drugs
Current Medicinal Chemistry - Central Nervous System Agents Drugs as Possible Triggers of Takotsubo Cardiomyopathy: A Comprehensive Literature Search - Update 2015
Current Clinical Pharmacology Biological Evaluation of Azomethine-dihydroquinazolinone Conjugates as Cancer and Cholinesterase Inhibitors
Medicinal Chemistry Promising Therapies in Sickle Cell Disease
Cardiovascular & Hematological Disorders-Drug Targets Therapeutic Application of Histone Deacetylase Inhibitors for Stroke
Central Nervous System Agents in Medicinal Chemistry Raf Inhibitors as Therapeutic Agents Against Neurodegenerative Diseases
CNS & Neurological Disorders - Drug Targets Selected PET Radioligands for Ion Channel Linked Neuroreceptor Imaging: Focus on GABA, NMDA and nACh Receptors
Current Topics in Medicinal Chemistry Natural Proteinaceous Inhibitors of Serine Proteases
Current Pharmaceutical Design