Frontotemporal dementia | UniteRare Disease Library

Overview

Frontotemporal dementia (FTD), also known as frontotemporal lobar degeneration (FTLD), Pick disease, or frontotemporal neurocognitive disorder, is a group of progressive neurodegenerative disorders primarily affecting the frontal and temporal lobes of the brain. It is one of the most common causes of early-onset dementia, typically presenting before age 65. The disease leads to progressive deterioration of behavior, personality, language, and executive function due to selective atrophy of the frontal and temporal cortical regions. FTD encompasses several clinical subtypes. The behavioral variant (bvFTD) is characterized by progressive changes in personality, social conduct, and behavior, including disinhibition, apathy, loss of empathy, compulsive or ritualistic behaviors, hyperorality, and impaired executive function. The language variants include progressive nonfluent aphasia (characterized by effortful, halting speech and grammatical errors) and semantic dementia (characterized by loss of word meaning and object knowledge). As the disease progresses, patients may develop motor symptoms overlapping with amyotrophic lateral sclerosis (ALS) or parkinsonism. Memory is often relatively preserved in early stages, distinguishing FTD from Alzheimer disease. Approximately 30-50% of FTD cases are familial, with autosomal dominant inheritance identified in a significant proportion. Major causative genes include MAPT (microtubule-associated protein tau), GRN (progranulin), and C9orf72 (chromosome 9 open reading frame 72 hexanucleotide repeat expansion), the latter also being the most common genetic cause of ALS. Other rarer genes include VCP, CHMP2B, TBK1, and TARDBP. Currently, there is no disease-modifying treatment for FTD. Management is symptomatic and supportive, involving behavioral interventions, speech and language therapy, and pharmacological management of specific symptoms such as agitation or depression with selective serotonin reuptake inhibitors (SSRIs). Antipsychotics may be used cautiously for severe behavioral disturbances. Multidisciplinary care and caregiver support are essential components of management.

Also known as:

FTD

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events

Feb 2026Social-cognitive Functioning: Validation of a New Neuropsychological Test

Assistance Publique - Hôpitaux de Paris — NA

TrialRECRUITING

Jan 2026Prospective Observational Study of the Relationship Between Sociodemographic Factors, Blood-based Biomarkers and Psychiatric Symptoms in Neurodegenerative Diseases and Mental Disorders

Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau

TrialENROLLING BY INVITATION

Nov 2025A Phase Ib/III Study of Suvemcitug Plus FTD/TPI in Participants With Refractory Metastatic Colorectal Cancer

Jiangsu Simcere Pharmaceutical Co., Ltd. — PHASE3

TrialRECRUITING

Oct 2025Investigator Initiated Study for the Safety and Efficacy in Frontotemporal Dementia

Hee-Jin Kim — PHASE2

TrialNOT YET RECRUITING

Oct 2025Metabolic Characterization of Alzheimer's Disease and Frontotemporal Dementia by 23Na-MRI and FDG-PET

Centre Hospitalier St Anne — NA

TrialNOT YET RECRUITING

Aug 2025Single-centre Descriptive Study of Taste in Frontotemporal Lobar Degeneration Through the Analysis of Taste Evoked Potentials

Centre Hospitalier Universitaire Dijon — NA

TrialRECRUITING

Aug 2025Evaluation of the Safety and Efficacy of Treatment w/High Dose Melphalan Given Directly Into the Liver Followed by Treatment w/Approved Cancer Treatment or Approved Cancer Treatment Alone in Patients w/ Metastatic Colorectal Cancer w/Liver Dominant Disease

Delcath Systems Inc. — PHASE2

TrialRECRUITING

Jun 2025A Study to Evaluate the Safety and Efficacy of Oral Nizubaglustat (AZ-3102) in Late-infantile and Juvenile Forms of Niemann-Pick Type C Disease (NPC)

Azafaros A.G. — PHASE3

TrialRECRUITING

Jun 2025A Study to Evaluate the Safety and Efficacy of Oral Nizubaglustat (AZ-3102) in Late-infantile and Juvenile Forms of Niemann-Pick Type C Disease, GM1 Gangliosidosis or GM2 Gangliosidosis

Azafaros A.G. — PHASE3

TrialRECRUITING

May 2025Deep Cervical Lymphaticovenous Anastomosis Surgery for Moderate-to-Advanced Dementia Patients

RenJi Hospital — NA

TrialNOT YET RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Frontotemporal dementia.

20 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

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Clinical Trials

20 recruitingView all trials with filters →

Other17 trials

Proteinopathies Expression in Skin of Neurodegenerative Disorders

Actively Recruiting

· Sites: San Luis Potosí City · Age: 45–99 yrs

Longitudinal Cognitive Assessment by BoCA

Actively Recruiting

· Sites: Miami, Florida · Age: 50–99 yrs

ECOCAPTURE for the Assessment of Apathy Under Real-life Conditions

Actively Recruiting

PI: Richard Levy, MD, PhD (Institut National de la Santé Et de la Recherche M) · Sites: Paris · Age: 40–85 yrs

Understanding Mechanisms of Synaptic Degeneration Underlying Clinical Symptoms in Patients With MDs and NDs.

Enrolling by Invitation

· Sites: Barcelona · Age: 40–99 yrs

Comprehensive Assessment of Neurodegeneration and Dementia

Actively Recruiting

PI: Michael Borrie, MD (Western University) · Sites: Calgary, Alberta; Edmonton, Alberta +17 more · Age: 50–90 yrs

Prospective Observational Study of the Relationship Between Sociodemographic Factors, Blood-based Biomarkers and Psychiatric Symptoms in Neurodegenerative Diseases and Mental Disorders

Enrolling by Invitation

· Sites: Barcelona · Age: 18–99 yrs

Tanycytes in Alzheimer's Disease and Frontotemporal Dementia

Actively Recruiting

PI: Thibaud LEBOUVIER, MD,PhD (University Hospital, Lille) · Sites: Lille · Age: 40–85 yrs

BioFINDER-Brown: Examination of Alzheimer's Disease Biomarkers

Enrolling by Invitation

PI: Edward Huey, MD (Butler Hospital Memory and Aging Program) · Sites: Providence, Rhode Island · Age: 50–80 yrs

Alzheimer's Disease Neuroimaging Initiative 4

Enrolling by Invitation

PI: Michael Weiner, MD (University of California, San Francisco) · Sites: Birmingham, Alabama; Phoenix, Arizona +60 more · Age: 55–90 yrs

Neurofilament Surveillance Project (NSP)

Active

PI: Adam Boxer, MD, PhD (University of California, San Francisco) · Sites: San Francisco, California; Jacksonville, Florida +6 more · Age: 18–85 yrs

Explore Biomarkers of Motor Neuron Disease/Frontal Dementia Spectrum Disease in China

Enrolling by Invitation

· Sites: Beijing, Beijing Municipality · Age: 18–80 yrs

The Sunnybrook Dementia Study

Active

PI: Sandra E Black, MD (Sunnybrook Health Sciences Centre) · Sites: Toronto, Ontario · Age: 40–90 yrs

Discovery and Validation of Protein Structural Complexes in Circulating Biofluids As Novel Biomarkers for Early Diagnosis, Prognosis and Therapeutic Management of Patients Affected by Neurodegenerative Disorders

Actively Recruiting

PI: TERESA ESPOSITO, PhD (IRCCS INM Neuromed) · Sites: Pozzilli, Italy · Age: 20–99 yrs

Cerebro Spinal Fluid Collection (CSF)

Actively Recruiting

PI: Frédéric BLANC, MD/PhD (Hôpitaux Universitaires de Strasbourg) · Sites: Strasbourg

Disease Biosignatures in ALS/FTD Spectrum: New Impactful Biological Perspectives Beyond Clinical Approaches

Actively Recruiting

· Sites: Milan; Naples +2 more · Age: 18–99 yrs

Characterization of Inclusion Body Myopathy Associated With Paget's Disease of Bone and Frontotemporal Dementia (IBMPFD)

Actively Recruiting

PI: Virginia Kimonis, MD (University of California, Irvine) · Sites: Irvine, California · Age: 18–99 yrs

Genetic Frontotemporal Dementia Initiative for Neurodevelopment

Actively Recruiting

PI: Elizabeth Finger, MD (Western University) · Sites: London, Ontario · Age: 7–17 yrs

Specialists

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OF

Orla Hardiman, BSc MB BCh BAO MD FRCPI FAAN

Specialist

Postpoliomyelitis syndrome

PI on 3 active trials

Active trials Directions Travel grants

AY

Ailing Yue

Specialist

2 Frontotemporal dementia publications

Overview

FDA & Trial Timeline

Treatments

Clinical Trials

Specialists

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Travel Grants

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