Frontotemporal dementia (FTD)

Published Date : Oct 2023
Category : Neurodegenerative Disorders
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Catalyze Change in FTD Diagnostic & Treatment Market with DiseaseLandscape Insights

Frontotemporal Disorder (FTD) stands out for its distinct difficulties and features in the field of neurodegenerative diseases, which continues to interest medical researchers, doctors, and caregivers. These mysterious neurological conditions offer a complicated tapestry of cognitive, behavioral, and emotional changes that can be mystifying and disappointing. They frequently affect individuals in their prime years.

The term "Frontotemporal Disorder" refers to a group of disorders where the frontal and temporal lobes of the brain have degenerated, significantly altering personality, behavior, and language skills. Symptoms typically occur between the ages of 40 to 65. FTD mainly affects regions involved in social behavior, decision-making, and language comprehension, as opposed to more well-known neurological disorders like Alzheimer's.

Recent developments in neurology and neuroimaging have helped to clarify the complicated complexities of FTD, illuminating its myriad symptoms, subgroups, and underlying genetic causes. However, because FTD is less common than other neurological disorders, it is frequently misdiagnosed or misunderstood, which highlights the urgent need for more education and study. Frontotemporal dementia has been linked to specific genetic alterations. However, there is no family history of dementia in more than half of those who acquire frontotemporal dementia. Recent studies have demonstrated that Amyotrophic Lateral Sclerosis (ALS) and frontotemporal dementia share similar genetic makeup and molecular mechanisms.

The most prominent types of FTD include –

  • Behavioral Variant Frontotemporal Dementia (bvFTD) – This form of FTD has an impact on personality, behavior & cognition
  • Primary Progressive Aphasia (PPA) – Aphasia involves difficulty in communicating and speaking. This form includes two subtypes –
  1. Semantic PPA (svPPA) - A person gradually loses the capacity to comprehend simple sentences and occasionally to identify the faces of well-known individuals and everyday things
  2. Agrammatic PPA - As speech becomes more complex, terms that connect nouns and verbs are often omitted. The person might ultimately lose the ability to talk at all. Eventually, the person experiences movement symptoms resembling those of Corticobasal Syndrome
  3. Lopogenic PPA - Though the patient understands words and sentences, the patient has problems finding the appropriate words during a conversation. No grammatical issues appear with this individual's words.

Furthermore, movement is impacted by a less prevalent type of FTD that indicates symptoms resembling those of Lou Gehrig's disease or Parkinson's disease. An FTD's fundamental cause is typically unknown. A family history of FTD increases a person's risk of developing the disease. Specific genetic factors account for ~ 10 to 30 percent of bvFTD cases.

Variants, or long-lasting mutations in specific genes, are frequently linked to FTD that runs in families. Genes are the fundamental building blocks of heredity that instruct cells to produce the proteins required for bodily function. An abnormal protein is produced by even minor alterations in a gene, which damages the brain and eventually results in disease.

Based on the report of the Alzheimer's Association in people over 65, bvFTD and PPA are both far less frequent than Alzheimer's disease. However, bvFTD and PPA are almost as prevalent in people aged 45 to 65 as in younger-onset Alzheimer's disease. In the United States, there probably are 50,000 to 60,000 people who have bvFTD or PPA, with a majority of them being between the ages of 45 and 65. Outside of this age range, these diseases are quite uncommon. More than 30,000 Americans suffer from ALS, but just about 20,000 suffer from PSP.

FTD is a rare condition, although it occurs frequently enough to be identified. Cleaveland clinic experts estimate that between 15 - 22 people out of every 100,000 suffer from FTD. Accordingly, it is present in between 1.2 to 1.8 million people worldwide. Men are more likely to be diagnosed with behavior variations (bvFTD) and semantic variants (svPPA), whereas women are more likely to suffer from Nonfluent Variant Primary Progressive Aphasia (nfvPPA). The average survival time is 7.5 years. To fully comprehend the relationship between these disorders, more study is required.

In the study conducted in 2021 by Alzheimer's Research & Therapy patients with the last clinical diagnosis was degenerative or vascular dementia were included in the study. During the 7 years of follow-up, 690 incident cases of FTD were associated, giving a frequency rate of 2.42 per 100,000 person-years. The FTD incidence across age groups at diagnosis reached the highest point in the 75-to-79-year-old group, with an incidence rate of 14.95 per 100,000 person-years. The age-standardized incidence rate was 2.90 per 100,000 person-years.

DiseaseLandscape Insights (DLI) Navigates the Healthcare Horizon and Empowers Informed Decision-Making in the Diagnostic, Treatment, Medical Device, and Clinical Trial Landscape.

DiseaseLandscape Insights (DLI) explores the different types of frontotemporal disorders, and diagnostic difficulties, and encourages advancements in research and treatment. Renowned authorities on neurology and neurodegenerative diseases offer their perspectives on how a greater comprehension of FTD contributes to improvements in patient care, support for caregivers, and developments in the broader area of neuroscience.

FTD Diagnostic Analysis –

Enrich Healthcare Diagnostic Choices by Gaining a Perception of the Latest Developments and Market Insights with DLI!!

The medical history, physical examination, and neurological examination (during which a healthcare professional searches for signs and symptoms of a problem) are all used to diagnose FTD by a healthcare professional, typically a Neurologist. Imaging studies are crucial because they reveal regions of the brain where deterioration appears to be occurring. Healthcare professionals frequently ask patients to complete a neurocognitive assessment in addition to a neurological check.

There is currently no single diagnostic technique that confirms or disproves the diagnosis of FTD. Changes in cognition, behavior, and motor abilities result from a variety of possible causes. In order to get a more precise diagnosis, more information about the patient is learned with each test that looks at these criteria. The doctor makes therapy recommendations using this information.

The tests that might be performed as part of a diagnostic work-up are listed below, along with a brief description of each test.

The following are the most likely diagnostic tests performed for FTD:

  • Medical History & Detailed Neurological Exam - A comprehensive study of the complete nerve system, including how it affects both physical and mental abilities. An initial assessment typically lasts one hour and includes:
  • Obtaining medical previous information.
  • Physical examination: assessing walking, balance, coordination, reflexes, strength, vision, and hearing as well as motor function.
  • Cognitive Examination - Memory, language, thinking, planning and organizational skills, visuospatial ability, behavior, and mood cognitively.
  • Neurophysiological Testing - Early diagnosis of the type of dementia is made possible with the use of this test. Frontotemporal dementia is distinguished from other types of dementia by the pattern of testing anomalies.
  • Magnetic Resonance Imaging (MRI scan) - A non-invasive technique that creates images of the brain and other organs using magnets and radio waves. For the majority of brain problems, MRI is preferred to a CT scan because it produces images from various angles and offers a complete examination of many brain regions that a CT scan is unable to reveal. MRI identifies atrophy or shrinking of particular brain regions that are indicative of FTD.
  • Positron Emission Tomography (PET scan) - The brain's activity is captured via PET scanning. An image of the quantity of glucose absorbed by neurons in certain brain regions is captured by a glucose PET scan. This is beneficial since glucose is the primary fuel for neurons, and decreased glucose absorption in a particular brain region indicates that the neurons in that region aren't operating as well. The amount of amyloid in the brain is visualized with an amyloid PET scan. Amyloid is a protein that has been improperly folded, builds up with aging, and is heavily involved in Alzheimer's disease.
  • Spinal Tap (Lumbar Puncture) –

Cerebrospinal fluid (CSF), the fluid that surrounds the brain and spinal cord, is collected and studied using this test. Each day, our body produces roughly 500 ml (about 16 ounces) of CSF, yet there is only adequate space for about 175 ml (6 ounces). Regular CSF analysis detects diseases like cancer, inflammatory processes, and uncommon infections that resemble FTD. Some of the misfolded proteins that are building up in the brain and leading to neurodegenerative diseases like FTD are assessed as the CSF moves about and surrounds the brain. A little needle is inserted during a lumbar puncture operation into the lower region of the back, just below the spinal cord.

  • Blood Test - Tests are conducted to check for diseases including thyroid disease, B12 deficiency, infections like syphilis or HIV, dehydration, or cancer that resemble FTD. These tests look for specific substances, proteins, hormones, and antibodies. Some of these disorders are cured, and they are all handled very differently.
  • Electroencephalography This method of diagnosis is less frequently used. Electroencephalography (EEG) is one potential biomarker for dementia with young onset. EEG is an inexpensive method of investigating brain activity and offers superior temporal resolution. Current studies on the EEG as a dementia biomarker do reveal recognizable abnormalities. The FDA (Food and Drug Administration) in the US has suggested production guidelines for EEG machine makers. The various components of an EEG machine are made independently, and the primary manufacturer assembles them before shipping. These parts, such as the electrodes, amplifier, storage, and output devices, can be purchased from outside suppliers or produced on-site.

The complete resource that highlights manufacturers and their Frontotemporal Dementia (FTD)-related goods is described in the table below. DLI emphasizes that there is a developing market for FTD diagnoses and therapies by highlighting the necessity of listing these producers and their products. For investors, business owners, and healthcare industry stakeholders with an interest in this area, this represents a potential opportunity. The underlying business opportunities within the FTD domain are highlighted by an understanding of the market landscape, which aids in the identification of possible areas for collaboration, investment, product development, or strategic partnerships.

                                                                                          Diagnostic Market Players

Magnetic Resonance Imaging

Positron Emission Tomography


Siemens Healthineers

Siemens Healthineers

Natus Medical Incorporated

General Electric (GE) Healthcare

Neusoft Medical Systems


Philips Healthcare

Canon Medical Systems

NeuroWave Systems Inc

Hitachi Healthcare Americas

Advanced Accelerator Applications (a Novartis company)

Cadwell Industries, Inc.

Neusoft Medical Systems

Mediso Medical Imaging Systems


Canon Medical Systems

Raycan Technology (Shanghai) Inc.

Compumedics Limited

Bruker Corporation

Toshiba Medical Systems Corporation


Aspect Imaging

Naviscan, Inc.



NeuroLogica Corporation

ANT Neuro


                                                                                             Diagnostic Market Products

Magnetic Resonance Imaging

Positron Emission Tomography




NeuroWave™ FTD EEG Diagnostic System

NeuroScan FTDMRI™


CogniScan™ EEG FTD Analyzer



MindScribe™ FTD-EEG Profiler

CogniScan FTD™


NeuraScan™ FTD Electrograph

CortexVision FTDMRI™


BrainWaveDetect™ FTD EEG Monitor

Temporal Insight MRI™

NeuroScan FTDPET™

CerebroSync™ FTD EEG Profiler

FTDetect MRI™

NeuroPET FTDetect™

MindWave™ FTD EEG Analytics Platform

NeuraScan FTDMRI™


NeuraWave™ FTD Brainwave Profiler


FTD Treatment Analysis -

The symptoms of FTD are easily managed with medication, but there is no cure or way to slow down the disease's course. Antidepressants are used to alleviate anxiety, as well as to manage other symptoms like compulsive behaviors. Insomnia and other sleep disorders are helped with prescription sleeping pills. Antipsychotic medication lessens obsessive and illogical conduct. Controlling undesirable or dangerous habits is possible with behavior modification. Physical, occupational, and speech therapists aid in the adjustment to some of the changes brought on by FTD.

Hence creating opportunities for the healthcare industries, and the development of a permanent cure for FTD.

Below are the tables indicating the Treatment market players as well as their products in the pursuit of improved health outcomes -

                                                                                           Treatment Market Players



Eli Lilly and Company

Otsuka Pharmaceutical Co., Ltd

Pfizer, Inc.

Janssen Pharmaceuticals




Sun Pharmaceutical Industries Ltd.

Johnson & Johnson


Takeda Pharmaceutical Company

Zydus Cadila

Otsuka Pharmaceutical Co.

Torrent Pharmaceuticals Ltd

Novartis AG

Apotex Inc.


                                                                                        Treatment Products



Prozac (fluoxetine)

Quetiapine (Seroquel)

Cymbalta (duloxetine)

Olanzapine (Zyprexa)

Zoloft (sertraline)

Risperidone (Risperdal)

Paxil (paroxetine)

Aripiprazole (Abilify)

Lexapro (escitalopram)

Brexpiprazole (Rexulti)

Celexa (citalopram)

Paliperidone (Invega)

Remeron (mirtazapine)

Clozapine (Clozaril)

Brintellix (vortioxetine)

Lurasidone (Latuda)


Even though there is no treatment for this neurological disease, companies and researchers are working on projects that could benefit patients and their families.

Antisense Oligonucleotides (ASOs) and gene therapies, which are still in the early stages of clinical studies, are two different strategies being researched. Other lines of investigation focus on the genetic relationship between FTD and ALS, which is useful in creating therapies for both diseases.

Treatments that specifically target potential disease-causing mutations are being developed by multiple companies. Included in this is Denali Therapeutics, whose TAK-594/DNL593 therapy is undergoing a Phase I/II trial. The granulin gene, which codes for the protein progranulin (PGRN), has been specifically targeted for mutation in FTD patients. The lysosomal process is supported by this protein.

Emerging Treatment Options –

  • Tau Targeting Therapeutics - Precision medicine could be used to treat FTD by focusing on the underlying pathogenesis as a result of advances in our understanding of molecular biology, pathophysiology, and neuropathology. The inhibition of the transneuronal spreading of pathological abnormalities is possibly a useful treatment technique, given the understanding of the pathogenic tau protein spreading by prion-like propagation. Patients with tau pathology benefit from receiving tau vaccinations, microtubule-stabilizing medications, tau-targeted immunotherapy, tau aggregation inhibitors (such as methylthioninium chloride), and microtubule-stabilizing pharmaceuticals.

Relevant facts concerning tau-targeting therapies for FTD:

  • Deviant Tau Protein - The stability and structure of nerve cells are maintained by tau proteins. However, in FTD and other tauopathies, these proteins accumulate and form neurofibrillary tangles, which cause neuronal cells to malfunction and perish.
  • Mechanism of Action - Therapeutics that target aberrant tau proteins aim to prevent or halt tau aggregation. These medications have the ability to either prevent the development of aberrant tau or aid in the removal of already present tau clumps.

Therapeutic Approaches –

  • Tau Kinase Inhibitors - These medications prevent the aberrant aggregation of tau by inhibiting the kinases that phosphorylate tau. Examples include TRx0237, sometimes referred to as LMTX.
  • Tau Antibodies - Tau aggregates in the brain can be specifically targeted by monoclonal antibodies and cleared.
  • Microtubule Stabilizing Agent - Certain substances inhibit tau from aggregating by stabilizing microtubules, the cellular structures to which tau normally interacts. Consider TPI-287 with epothilone D.
  • Personalized Medicine – Precision medicine has the potential to help doctors find highly targeted, efficient treatments for patients with particular diseases that have few adverse effects. Precision medicine is currently used in the diagnosis and treatment of FTD. Precision medicine is now used in clinical trials and is based on advancements in neuroimaging and genomic research that have investigated underlying genetic risk variations and cerebral structure and functional change in order to discover particular molecular pathways and pathophysiological processes.

Clinical Trial Assessment –

DiseaseLandscape Insights assists in the advancement of clinical trials for new drugs, therapies & medical devices, patient recruitment, site feasibility regulatory compliance for successful outcomes, and patient benefit. The below table updates the ongoing clinical trials & the phase in which they are being conducted


Phase 1

Phase 2

Phase 3

Clinical Assessment on the Safety and Potential Efficacy of Mesenchymal Stem Cells Preconditioned with Ethionamide (ET-STEM) in Patients with Frontotemporal Dementia (FTD)

The Influence of Vascular Burden, Amyloid Plaque, and Tau Protein in Patients with Vascular Cognitive Impairment and Dementia with Tauopathy

Imaging Tau Accumulation in FTLD and Atypical Alzheimer's Disease Using the PET Ligand PI-2620

A Phase 1b Open-Label, Multicenter, Dose-Escalation Study to Assess the Safety, Tolerability, and Pharmacodynamic Effects of a Single Dose of PBFT02 Delivered into the Cisterna Magna of Adult Subjects with Frontotemporal Dementia and Mutations in the Progranulin Gene

A Single-Center, Open-Label Study to Assess the Safety and Tolerability of Metformin in Subjects with C9orf72 Amyotrophic Lateral Sclerosis Over 24 Weeks of Treatment

Phase 3, Douwithblind, Randomized, Placebo-controlled, Parallel-group, Multicenter Study to Evaluate the Safety, Tolerability, and Efficacy of 2000mg/kg of Trappsol®Cyclo™ (Hydroxypropyl-B-cyclodextrin) and Standard of Care Compared to Placebo and Standard of Care in Patients with Niemann-Pick Disease Type C1 (TransportNPC)


A Phase 1/2 Ascending Dose Study to Evaluate the Safety and Effects on Progranulin Levels of PR006A in Patients with Fronto-Temporal Dementia with Progranulin Mutations (FTD-GRN)

A Phase 1b Open-Label, Multicenter, Dose-Escalation Study to Assess the Safety, Tolerability, and Pharmacodynamic Effects of a Single Dose of PBFT02 Delivered into the Cisterna Magna of Adult Subjects with Frontotemporal Dementia and Mutations in the Progranulin Gene

A Phase 3, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of AL001 in Individuals at Risk for or With Frontotemporal Dementia Due to Heterozygous Mutations in the Progranulin Gene

A Phase 1/2, Multicenter, Randomized, Placebo-Controlled, Double-Blind Single Dose and Multiple Dose Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of DNL593 in Healthy Participants and Participants with Frontotemporal Dementia Followed by an Open-Label Extension


A Phase 1/2 Ascending Dose Study to Evaluate the Safety and Effects on Progranulin Levels of PR006A in Patients with Fronto-Temporal Dementia with Progranulin Mutations (FTD-GRN)


PET Imaging of Neuroinflammation in Neurodegenerative Diseases Via a Novel TSPO Radioligand

A Phase 1/2, Multicenter, Randomized, Placebo-Controlled, Double-Blind Single Dose and Multiple Dose Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of DNL593 in Healthy Participants and Participants with Frontotemporal Dementia Followed by an Open-Label Extension


Tau PET Imaging in Atypical Dementias

Randomized, Double-Blind, Placebo-Controlled, Multicenter, 12 Weeks Phase 2 Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Oral AZ-3102 in Patients with GM2 Gangliosidosis or Niemann-Pick Type C Disease


Augmentation of Umbilical Cord Blood Transplantation for Inherited Metabolic Diseases with Intrathecal Administration of Human Umbilical Cord Blood-Derived Oligodendrocyte-Like Cells

Treating Primary Progressive Aphasia (PPA) and Elucidating Neurodegeneration in the Language Network Using Transcranial Direct Current Stimulation (tDCS)



Conclusion –

Our goal as a committed consultancy firm specializing in the diagnosis and management of frontotemporal dementia (FTD) is to enlighten patients, families, and healthcare professionals as they confront the difficulties associated with this challenging neurological condition. We are prepared to work with you to understand, manage, and eventually improve the lives of people struck by FTD owing to our steady dedication to revolutionary studies, clinical insights, and creative solutions.

The most recent developments in FTD research, clinical practice, and patient-centered treatment are brought together by our multidisciplinary team of specialists. Since every case is different, we customize our approach to offer precise diagnostics, individualized treatment plans, and extensive support networks. We work to close the knowledge-to-action gap by promoting early detection and coordination among medical specialties.

Together, DLI services open the door to improved FTD diagnosis, care, and treatment. As our experts lead the way in frontotemporal dementia transformation and advancement, join us in welcoming the possibilities. We are here to help you every step of the way because your trip is also our journey.

Vishal SawantBusiness Development

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