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
-
Targeting Transcription Factors for Cancer Therapy
Current Pharmaceutical Design Atypical GTPases as Drug Targets
Anti-Cancer Agents in Medicinal Chemistry Editorial (Hot Topics: Naturally Occurring Molecules and Anticancer Combination Therapies in the Era of Personalized Medicine and Economic Crisis)
Current Pharmaceutical Design Novel Colchicine Derivatives and their Anti-cancer Activity
Current Topics in Medicinal Chemistry SRC: Regulation, Role in Human Carcinogenesis and Pharmacological Inhibitors
Current Pharmaceutical Design Association of Gag Multimers with Filamentous Actin During Equine Infectious Anemia Virus Assembly
Current HIV Research Development and Engineering of Lymphatic Endothelial Cells: Clinical Implications
Current Pharmaceutical Design Recent Progress in Molecular Mechanisms of Angiotensin II Type 1 and 2 Receptors
Current Pharmaceutical Design Involvement of Cysteine Proteases in Cancer
Current Medicinal Chemistry Thymic Immunosuppressive Pentapeptide (TIPP) Shown Anticancer Activity in Breast Cancer and Chronic Myeloid Leukemia Both <i>In Vitro</i> and <i>In Vivo</i>
Protein & Peptide Letters Contrast Echocardiography: An Update on Clinical Applications
Current Pharmaceutical Design Namitecan: a Hydrophilic Camptothecin with a Promising Preclinical Profile
Current Medicinal Chemistry Discussion on the Structural Modification and Anti-tumor Activity of Flavonoids
Current Topics in Medicinal Chemistry Translational Research of Photodynamic Therapy with Acridine Orange which Targets Cancer Acidity
Current Pharmaceutical Design Can the Use of HIV-1 Derived Gene Transfer Vectors for Clinical Application be Justified?
Current Gene Therapy Influence of Tumor Microenvironment on the Distribution and Elimination of Nano-formulations
Current Drug Metabolism Synthesis and In Vitro Antiproliferative Activity of Thiazole-Based Nitrogen Mustards: The Hydrogen Bonding Interaction between Model Systems and Nucleobases
Anti-Cancer Agents in Medicinal Chemistry Glycoconjugates in Cancer Therapy
Anti-Cancer Agents in Medicinal Chemistry Pharmacogenetic Applications of the Post Genomic Era
Current Pharmaceutical Biotechnology Cardiovascular Complications of HIV Infection and Treatment
Cardiovascular & Hematological Agents in Medicinal Chemistry