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
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
Diagnostic and therapeutic biomarkers of dementia
Dementia affects 18 million people worldwide. Dementia is a syndrome of symptoms caused by brain disease, usually chronic or progressive, clinically characterized by multiple impairments of higher cortical functions such as memory, thinking, orientation, and learning. In addition, in the course of dementia, cognitive deficits are observed, which often hinder ...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
-
Neuroprotective Effects of Citrus reticulata in Scopolamine-Induced Dementia Oxidative Stress in Rats
CNS & Neurological Disorders - Drug Targets CYP-Mediated Clozapine Interactions: How Predictable Are They?
Current Drug Metabolism Neurotrophic Factors in Combination: A Possible new Therapeutic Strategy to Influence Pathophysiology of Spinal Cord Injury and Repair Mechanisms
Current Pharmaceutical Design Sundowning Syndrome: A Possible Marker of Frailty in Alzheimer’s Disease?
CNS & Neurological Disorders - Drug Targets Vascular Disease and Insulin-Like Growth Factor-1
Vascular Disease Prevention (Discontinued) Neuroprotection & Mechanism of Ethanol in Stroke and Traumatic Brain Injury Therapy: New Prospects for an Ancient Drug
Current Drug Targets The Essential Mechanisms of Aging: What Have We Learnt in Ten Years?
Current Topics in Medicinal Chemistry Guide of Hypertensive Crisis Pharmacotherapy
Cardiovascular & Hematological Disorders-Drug Targets Biological Basis of Ageing, Age-related Diseases and Interventions
Current Pharmacogenomics and Personalized Medicine Applications of Nanotechnology in Diagnostics and Therapeutics of Alzheimer’s and Parkinson’s Disease
Current Drug Metabolism Current Therapeutic Strategies and Future Perspectives for the Prevention of Arterial Thromboembolism: Focus on Atrial Fibrillation
Current Pharmaceutical Design Retinoic Acid and the Gut Microbiota in Alzheimer’s Disease: Fighting Back-to-Back?
Current Alzheimer Research (±)-3,5-Bis(substitutedmethyl)pyrrolidines: Application to the Synthesis of Analogues of glycine-L-proline-L-glutamic Acid (GPE)
Current Organic Synthesis A Hyperlipidemic Diet Induces Structural Changes in Cerebral Blood Vessels
Current Neurovascular Research Involvement of the Mitochondrial Benzodiazepine Receptor in Traumatic Brain Injury: Therapeutic Implications
CNS & Neurological Disorders - Drug Targets Phytocannabinoids: General Aspects and Pharmacological Potential in Neurodegenerative Diseases
Current Neuropharmacology Editorial : Novel Therapeutic Targets for Cardiovascular Disease
Current Pharmaceutical Design The Hypothesis of the Catalytic Action of Nucleic Acid on the Conversion of Prion Protein
Protein & Peptide Letters Recent Advancements in Nanodiamond Mediated Brain Targeted Drug Delivery and Bioimaging of Brain Ailments: A Holistic Review
Pharmaceutical Nanotechnology Potential Targets for the Development of Novel Antidepressants: Future Perspectives
CNS & Neurological Disorders - Drug Targets