Abstract
The first reports of disorders that in terms of cognitive and behavioral symptoms resemble frontotemporal dementia (FTD) and in terms of motor symptoms resemble amyotrophic lateral sclerosis (ALS) bring us back to the second half of the 1800s. Over the last 150 years, and especially in the last two decades, there has been growing evidence that FTD signs can be seen in patients primarily diagnosed with ALS, implying clinical overlap among these two disorders. In the last decade pathological investigations and genetic screening have contributed tremendously in elucidating the pathology and genetic variability associated with FTD and ALS. TAR DNA binding protein [TARDBP or TDP-43] and the fused in sarcoma gene [FUS] and their implication in these disorders belong to the most important recent discoveries. FTD and ALS are the focus of this review which aims to: 1. summarize clinical features by describing the diagnostic criteria and specific symptomatology, 2. describe the morphological aspects and related pathology, 3. describe the genetic factors associated with the diseases and 4. summarize the current status of clinical trials and treatment options. A better understanding of the clinical, pathological and genetic features characterizing FTD and ALS will shed light into overlaps among these two disorders and the underpinning mechanisms that contribute to their onset and development. Advancements in the knowledge of the biology of these two disorders will help developing novel and, hopefully, more effective diagnostic and treatment options.
Keywords: ALS, FTD, FUS, motor disease, proteinopathies, TAU, TDP-43, ubiquitin inclusions, FTLD, MAPT, MDD, cognitive dysfunctions, ALSbi, affective symptoms, DLPFC
Current Alzheimer Research
Title: FTD and ALS: A Tale of Two Diseases
Volume: 8 Issue: 3
Author(s): R. Ferrari, D. Kapogiannis, E. D. Huey and P. Momeni
Affiliation:
Keywords: ALS, FTD, FUS, motor disease, proteinopathies, TAU, TDP-43, ubiquitin inclusions, FTLD, MAPT, MDD, cognitive dysfunctions, ALSbi, affective symptoms, DLPFC
Abstract: The first reports of disorders that in terms of cognitive and behavioral symptoms resemble frontotemporal dementia (FTD) and in terms of motor symptoms resemble amyotrophic lateral sclerosis (ALS) bring us back to the second half of the 1800s. Over the last 150 years, and especially in the last two decades, there has been growing evidence that FTD signs can be seen in patients primarily diagnosed with ALS, implying clinical overlap among these two disorders. In the last decade pathological investigations and genetic screening have contributed tremendously in elucidating the pathology and genetic variability associated with FTD and ALS. TAR DNA binding protein [TARDBP or TDP-43] and the fused in sarcoma gene [FUS] and their implication in these disorders belong to the most important recent discoveries. FTD and ALS are the focus of this review which aims to: 1. summarize clinical features by describing the diagnostic criteria and specific symptomatology, 2. describe the morphological aspects and related pathology, 3. describe the genetic factors associated with the diseases and 4. summarize the current status of clinical trials and treatment options. A better understanding of the clinical, pathological and genetic features characterizing FTD and ALS will shed light into overlaps among these two disorders and the underpinning mechanisms that contribute to their onset and development. Advancements in the knowledge of the biology of these two disorders will help developing novel and, hopefully, more effective diagnostic and treatment options.
Export Options
About this article
Cite this article as:
Ferrari R., Kapogiannis D., D. Huey E. and Momeni P., FTD and ALS: A Tale of Two Diseases, Current Alzheimer Research 2011; 8 (3) . https://dx.doi.org/10.2174/156720511795563700
DOI https://dx.doi.org/10.2174/156720511795563700 |
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
-
Oxidative Stress and Neurodegenerative Diseases: A Neurotrophic Approach
Current Drug Targets Occurrence and Biological Activities of Eremophilane-type Sesquiterpenes
Mini-Reviews in Medicinal Chemistry Influence of Tumor Microenvironment on the Distribution and Elimination of Nano-formulations
Current Drug Metabolism The Therapeutic Potential of RNA Interference: Novel Approaches for Cancer Treatment
Current Pharmaceutical Biotechnology Prolyl Peptidases Related to Dipeptidyl Peptidase IV: Potential of Specific Inhibitors in Drug Discovery.
Current Topics in Medicinal Chemistry Current Management of Neonatal Liver Tumors
Current Pediatric Reviews NF-κB Down–regulation and PARP Cleavage by novel 3-α-butyryloxy-β-boswellic Acid Results in Cancer Cell Specific Apoptosis and in vivo Tumor Regression
Anti-Cancer Agents in Medicinal Chemistry Focus on MicroRNAs as Biomarker in Pediatric Diseases
Current Pharmaceutical Design Scorpion Extracts Inhibit Tumor Growth in Esophageal Cancer and Lung Adenocarcinoma
Current Signal Transduction Therapy Interleukin-6 In Rheumatoid Arthritis - From The Laboratory To The Bedside
Current Pharmaceutical Design Targeting Chk2 Kinase: Molecular Interaction Maps and Therapeutic Rationale
Current Pharmaceutical Design Vascular Targeting: A New Antitumor Activity
Drug Design Reviews - Online (Discontinued) Radiotracers in Oncology
Current Radiopharmaceuticals Modulation of Gene Transcription by Natural Products - A Viable Anticancer Strategy
Current Pharmaceutical Design Chemotherapeutic Agents That Induce Mitochondrial Apoptosis
Current Cancer Drug Targets Activated Cancer Therapy Using Light and Ultrasound - A Case Series of Sonodynamic Photodynamic Therapy in 115 Patients Over a 4 Year Period
Current Drug Therapy Neuropathic Pain and Lung Delivery of Nanoparticulate Drugs: An Emerging Novel Therapeutic Strategy
CNS & Neurological Disorders - Drug Targets The MYC Oncogene as a Cancer Drug Target
Current Cancer Drug Targets Olefin Metathesis Route to Antiviral Nucleosides
Current Topics in Medicinal Chemistry Hijacking the Hedgehog Pathway in Cancer Therapy
Anti-Cancer Agents in Medicinal Chemistry