Semantic dementia
disease diseaseOn this page
Also known as dementia, frontotemporaldementia, frontotemporal, with or without parkinsonismfrontotemporal dementiaFTDsemantic primary progressive aphasiasemantic variant PPA
Summary
Semantic dementia (MONDO:0010857) is a disease caused by variants in MAPT and PSEN1, with 2 cohort genes and 161 clinical trials. Top therapeutic interventions include citalopram, florbetapir, and memantine.
At a glance
- Prevalence: <1 / 1 000 000 (Worldwide) [Orphanet-validated]
- Causal genes: MAPT (GenCC Strong), PSEN1 (GenCC Strong)
- Cohort genes: 2
- ClinVar variants: 1
- Phenotypes (HPO): 10
- Clinical trials: 161
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Cases/families | 49 | Worldwide | Validated | |
| Point prevalence | <1 / 1 000 000 | Worldwide | Validated |
Signs & symptoms
Clinical features (HPO)
10 HPO clinical features (Orphanet curated; top 10 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0002381 | Aphasia | Very frequent (80-99%) |
| HP:0012444 | Brain atrophy | Very frequent (80-99%) |
| HP:0030222 | Visual agnosia | Very frequent (80-99%) |
| HP:0030784 | Anomia | Very frequent (80-99%) |
| HP:0000726 | Dementia | Frequent (30-79%) |
| HP:0002167 | Abnormality of speech or vocalization | Frequent (30-79%) |
| HP:0010522 | Dyslexia | Frequent (30-79%) |
| HP:0010523 | Alexia | Frequent (30-79%) |
| HP:0010526 | Dysgraphia | Frequent (30-79%) |
| HP:0012671 | Abulia | Frequent (30-79%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | semantic dementia |
| Mondo ID | MONDO:0010857 |
| OMIM | 600274 |
| Orphanet | 100069 |
| DOID | DOID:0051060, DOID:0081391 |
| UMLS | C0338462 |
| MedGen | 83268 |
| GARD | 0010792 |
| Is cancer (heuristic) | no |
Also known as: dementia, frontotemporal · dementia, frontotemporal, with or without parkinsonism · frontotemporal dementia · FTD · semantic primary progressive aphasia · semantic variant PPA
Data availability: 1 ClinVar variant · 3 GenCC gene-disease records · 143 cell lines.
Disease family
An umbrella term covering 1 Mondo subtype.
Classification path: disease › human disease › disease by body system or component › nervous system disorder › movement disorder › progressive non-fluent aphasia › semantic dementia
Subtypes (1): frontotemporal dementia, right temporal atrophy variant
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
ClinVar germline variants
1 retrieved; paginated sample, class counts are floors:
1 conflicting classifications of pathogenicity
| ClinVar | Variant (HGVS) | Gene | Classification | Review |
|---|---|---|---|---|
| 3036349 | NM_001377265.1(MAPT):c.1568G>A (p.Arg523Gln) | MAPT | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
Genes & proteins
Mendelian disease overlap and somatic drivers
GenCC: 20 · Orphanet: 13 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0
GenCC gene–disease validity (cohort genes)
the Disease column is the GenCC-asserted condition — a cohort gene’s strongest validity may be for a related predisposition syndrome.
| Gene | Classification | Inheritance | Disease | Records |
|---|---|---|---|---|
| MAPT | Strong | Autosomal dominant | semantic dementia | 8 |
| PSEN1 | Strong | Autosomal dominant | semantic dementia | 12 |
Orphanet rare-disease linkage (cohort genes)
| Gene | Orphanet ID | Rare disease |
|---|---|---|
| MAPT | Orphanet:100069 | Semantic dementia |
| MAPT | Orphanet:100070 | Progressive non-fluent aphasia |
| MAPT | Orphanet:240071 | Classic progressive supranuclear palsy syndrome |
| MAPT | Orphanet:240085 | Progressive supranuclear palsy-predominant parkinsonism syndrome |
| MAPT | Orphanet:240094 | Progressive supranuclear palsy-pure akinesia with gait freezing syndrome |
| MAPT | Orphanet:240103 | Progressive supranuclear palsy-corticobasal syndrome |
| MAPT | Orphanet:240112 | Progressive supranuclear palsy-progressive non-fluent aphasia syndrome |
| MAPT | Orphanet:275864 | Behavioral variant of frontotemporal dementia |
| PSEN1 | Orphanet:100069 | Semantic dementia |
| PSEN1 | Orphanet:100070 | Progressive non-fluent aphasia |
| PSEN1 | Orphanet:1020 | Early-onset autosomal dominant Alzheimer disease |
| PSEN1 | Orphanet:154 | Familial isolated dilated cardiomyopathy |
| PSEN1 | Orphanet:275864 | Behavioral variant of frontotemporal dementia |
Cohort genes → proteins
2 cohort genes, 2 distinct canonical proteins.
Evidence partition
| Subset | Genes |
|---|---|
| multi_evidence | 2 |
Cohort genes (full)
| Symbol | HGNC | Ensembl | UniProt | Name | Evidence |
|---|---|---|---|---|---|
| MAPT | HGNC:6893 | ENSG00000186868 | P10636 | Microtubule-associated protein tau | gencc,clinvar |
| PSEN1 | HGNC:9508 | ENSG00000080815 | P49768 | Presenilin-1 | gencc |
Cohort function summary
Lead sentence per gene, UniProt-curated.
| Symbol | Protein name | Function (lead sentence) |
|---|---|---|
| MAPT | Microtubule-associated protein tau | Promotes microtubule assembly and stability, and might be involved in the establishment and maintenance of neuronal polarity. |
| PSEN1 | Presenilin-1 | Catalytic subunit of the gamma-secretase complex, an endoprotease complex that catalyzes the intramembrane cleavage of integral membrane proteins such as Notch receptors and APP (amyloid-beta precursor protein). |
Protein-family classification
Druggable: 1 · Difficult: 0 · Unknown: 1 · Druggable fraction: 0.5
Family distribution
Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.
| Family | Genes | Fold | FDR |
|---|---|---|---|
| Protease | 1 | 18.3× | 0.108 |
| Other/Unknown | 1 | 0.9× | 0.805 |
Per-gene assignment
| Symbol | Family | Druggable? | EC | InterPro (top 3) |
|---|---|---|---|---|
| MAPT | Other/Unknown | no | MAP_tubulin-bd_rpt, Tau, MAP2/MAP4/Tau | |
| PSEN1 | Protease | yes | Peptidase_A22A, Pept_A22A_PS1, Preselin/SPP |
Expression context
Cohort genes with no expression data: 0.
2 cohort genes are a single-cell marker in ≥1 SCXA experiment.
Breadth distribution (Bgee present_calls)
| Bucket | Genes |
|---|---|
| narrow (1-5 tissues) | 0 |
| moderate (6-20) | 0 |
| broad (>20) | 2 |
| unknown | 0 |
Top tissues across cohort
| Tissue | Cohort genes |
|---|---|
| cortical plate | 1 |
| prefrontal cortex | 1 |
| superior frontal gyrus | 1 |
| C1 segment of cervical spinal cord | 1 |
| corpus callosum | 1 |
| middle frontal gyrus | 1 |
Per-gene tissue summary (top 30)
| Symbol | Bgee breadth | FANTOM5 breadth | SCXA | Top tissues |
|---|---|---|---|---|
| MAPT | 141 | broad | marker | cortical plate, superior frontal gyrus, prefrontal cortex |
| PSEN1 | 287 | ubiquitous | marker | middle frontal gyrus, corpus callosum, C1 segment of cervical spinal cord |
Protein interactions among cohort
Intra-cohort edges: 1.
Hub genes (top 10 by interactor count)
| Symbol | Interactor count |
|---|---|
| MAPT | 7,289 |
| PSEN1 | 3,732 |
Intra-cohort edges
| A | B | Sources |
|---|---|---|
| MAPT | PSEN1 | string_interaction |
Structural data
PDB: 2 · AlphaFold-only: 0 · No structure: 0
Cohort genes with PDB structures (top 30)
| Symbol | UniProt | PDB entries |
|---|---|---|
| MAPT | P10636 | 293 |
| PSEN1 | P49768 | 27 |
Function
Pathway analysis
Distinct Reactome pathways touched by cohort: 21. Enrichment computed across 2 evidence-associated genes (2 with Reactome annotation).
Pathways by enrichment
Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| Pathway | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| Noncanonical activation of NOTCH3 | 1 | 713.8× | 0.009 | PSEN1 |
| Regulated proteolysis of p75NTR | 1 | 519.1× | 0.009 | PSEN1 |
| NOTCH4 Activation and Transmission of Signal to the Nucleus | 1 | 519.1× | 0.009 | PSEN1 |
| Caspase-mediated cleavage of cytoskeletal proteins | 1 | 475.8× | 0.009 | MAPT |
| TGFBR3 PTM regulation | 1 | 475.8× | 0.009 | PSEN1 |
| NRIF signals cell death from the nucleus | 1 | 356.9× | 0.009 | PSEN1 |
| Apoptotic cleavage of cellular proteins | 1 | 237.9× | 0.009 | MAPT |
| Apoptotic execution phase | 1 | 237.9× | 0.009 | MAPT |
| NOTCH3 Activation and Transmission of Signal to the Nucleus | 1 | 237.9× | 0.009 | PSEN1 |
| NOTCH2 Activation and Transmission of Signal to the Nucleus | 1 | 219.6× | 0.009 | PSEN1 |
| Activation of AMPK downstream of NMDARs | 1 | 190.3× | 0.009 | MAPT |
| Activated NOTCH1 Transmits Signal to the Nucleus | 1 | 178.4× | 0.009 | PSEN1 |
| Nuclear signaling by ERBB4 | 1 | 173.0× | 0.009 | PSEN1 |
| EPH-ephrin mediated repulsion of cells | 1 | 109.8× | 0.013 | PSEN1 |
| Constitutive Signaling by NOTCH1 PEST Domain Mutants | 1 | 98.5× | 0.013 | PSEN1 |
| Constitutive Signaling by NOTCH1 HD+PEST Domain Mutants | 1 | 98.5× | 0.013 | PSEN1 |
| Apoptosis | 1 | 84.0× | 0.015 | MAPT |
| Programmed Cell Death | 1 | 73.2× | 0.016 | MAPT |
| PKR-mediated signaling | 1 | 70.5× | 0.016 | MAPT |
| Degradation of the extracellular matrix | 1 | 58.9× | 0.018 | PSEN1 |
| Neutrophil degranulation | 1 | 11.5× | 0.085 | PSEN1 |
GO biological processes by enrichment
Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| GO term | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| astrocyte activation | 2 | 991.3× | 1e-04 | MAPT, PSEN1 |
| synapse organization | 2 | 280.9× | 5e-04 | MAPT, PSEN1 |
| regulation of synaptic plasticity | 2 | 259.3× | 5e-04 | MAPT, PSEN1 |
| learning or memory | 2 | 240.7× | 5e-04 | MAPT, PSEN1 |
| memory | 2 | 183.2× | 7e-04 | MAPT, PSEN1 |
| plus-end-directed organelle transport along microtubule | 1 | 8426.0× | 0.002 | MAPT |
| positive regulation of L-glutamate import across plasma membrane | 1 | 4213.0× | 0.002 | PSEN1 |
| Cajal-Retzius cell differentiation | 1 | 4213.0× | 0.002 | PSEN1 |
| smooth endoplasmic reticulum calcium ion homeostasis | 1 | 4213.0× | 0.002 | PSEN1 |
| neurofibrillary tangle assembly | 1 | 4213.0× | 0.002 | MAPT |
| negative regulation of protein localization to mitochondrion | 1 | 4213.0× | 0.002 | MAPT |
| negative regulation of gene expression | 2 | 69.1× | 0.002 | MAPT, PSEN1 |
| protein catabolic process at postsynapse | 1 | 2808.7× | 0.003 | PSEN1 |
| astrocyte activation involved in immune response | 1 | 2106.5× | 0.003 | PSEN1 |
| rRNA metabolic process | 1 | 2106.5× | 0.003 | MAPT |
| obsolete synaptic vesicle targeting | 1 | 2106.5× | 0.003 | PSEN1 |
| axonal transport | 1 | 2106.5× | 0.003 | MAPT |
| positive regulation of protein localization to synapse | 1 | 2106.5× | 0.003 | MAPT |
| DNA damage response | 2 | 53.5× | 0.003 | MAPT, PSEN1 |
| obsolete sequestering of calcium ion | 1 | 1685.2× | 0.003 | PSEN1 |
| negative regulation of mitochondrial fission | 1 | 1685.2× | 0.003 | MAPT |
| regulation of long-term synaptic depression | 1 | 1685.2× | 0.003 | MAPT |
| positive regulation of amyloid fibril formation | 1 | 1685.2× | 0.003 | PSEN1 |
| positive regulation of coagulation | 1 | 1404.3× | 0.004 | PSEN1 |
| regulation of microtubule-based movement | 1 | 1404.3× | 0.004 | MAPT |
| intracellular distribution of mitochondria | 1 | 1203.7× | 0.004 | MAPT |
| regulation of mitochondrial fission | 1 | 1053.2× | 0.004 | MAPT |
| Notch receptor processing | 1 | 936.2× | 0.004 | PSEN1 |
| amyloid-beta formation | 1 | 936.2× | 0.004 | PSEN1 |
| L-glutamate import across plasma membrane | 1 | 936.2× | 0.004 | PSEN1 |
Therapeutics
Drug target analysis
Approved (phase 4): 2 · Phase ≥3: 2 · Phased (≥1): 2 · Undrugged: 0
Druggability breadth: 2 of 2 evidence-associated genes (100%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).
Genes with an approved drug
The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.
| Symbol | Example approved molecule |
|---|---|
| MAPT | BEPRIDIL |
| PSEN1 | NIROGACESTAT |
Top cohort targets by molecule count
| Symbol | Molecules | Max phase |
|---|---|---|
| MAPT | 449 | 4 |
| PSEN1 | 8 | 4 |
Drugs targeting cohort genes (top 30)
| Molecule | Max phase | Targets in cohort |
|---|---|---|
| BEPRIDIL | 4 | MAPT |
| PHENYLBUTAZONE | 4 | MAPT |
| CEFOTAXIME SODIUM | 4 | MAPT |
| DIENESTROL | 4 | MAPT |
| PROGESTERONE | 4 | MAPT |
| CLOTRIMAZOLE | 4 | MAPT |
| CHOLECALCIFEROL | 4 | MAPT |
| LATANOPROST | 4 | MAPT |
| CHLORTHALIDONE | 4 | MAPT |
| FLUORESCEIN | 4 | MAPT |
| OXCARBAZEPINE | 4 | MAPT |
| NABUMETONE | 4 | MAPT |
| GLIPIZIDE | 4 | MAPT |
| AMIODARONE HYDROCHLORIDE | 4 | MAPT |
| TRICLABENDAZOLE | 4 | MAPT |
| MESORIDAZINE | 4 | MAPT |
| INDIGOTINDISULFONATE | 4 | MAPT |
| TRIHEXYPHENIDYL HYDROCHLORIDE | 4 | MAPT |
| IMIPRAMINE | 4 | MAPT |
| FURAZOLIDONE | 4 | MAPT |
| DROPERIDOL | 4 | MAPT |
| ARIPIPRAZOLE | 4 | MAPT |
| RALOXIFENE HYDROCHLORIDE | 4 | MAPT |
| IDARUBICIN | 4 | MAPT |
| ACETAMINOPHEN | 4 | MAPT |
| ACITRETIN | 4 | MAPT |
| CISPLATIN | 4 | MAPT |
| CLOBETASOL PROPIONATE | 4 | MAPT |
| AMINOSALICYLIC ACID | 4 | MAPT |
| TETRABENAZINE | 4 | MAPT |
Bioactivity and enzyme data
Enzyme cohort genes (≥1 EC): 0.
Cohort genes with ChEMBL bioactivity (full, sorted by assay count)
| Symbol | Assays | Type breakdown |
|---|---|---|
| PSEN1 | 557 | Binding:538, Functional:12, ADMET:6, Unclassified:1 |
| MAPT | 184 | Binding:180, Functional:4 |
Cohort genes with high screening signal
≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.
| Symbol | ChEMBL assays |
|---|---|
| MAPT | 184 |
| PSEN1 | 557 |
Pharmacogenomics
Cohort genes with a PharmGKB record: 2; with CPIC/DPWG dosing guidelines: 0.
No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).
Chemical tractability of cohort targets
30 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.
| Compound | Max phase | Cohort target (bioactivity) |
|---|---|---|
| BEPRIDIL | 4 | MAPT |
| PHENYLBUTAZONE | 4 | MAPT |
| CEFOTAXIME SODIUM | 4 | MAPT |
| DIENESTROL | 4 | MAPT |
| PROGESTERONE | 4 | MAPT |
| CLOTRIMAZOLE | 4 | MAPT |
| CHOLECALCIFEROL | 4 | MAPT |
| LATANOPROST | 4 | MAPT |
| CHLORTHALIDONE | 4 | MAPT |
| FLUORESCEIN | 4 | MAPT |
| OXCARBAZEPINE | 4 | MAPT |
| NABUMETONE | 4 | MAPT |
| GLIPIZIDE | 4 | MAPT |
| AMIODARONE HYDROCHLORIDE | 4 | MAPT |
| TRICLABENDAZOLE | 4 | MAPT |
| MESORIDAZINE | 4 | MAPT |
| INDIGOTINDISULFONATE | 4 | MAPT |
| TRIHEXYPHENIDYL HYDROCHLORIDE | 4 | MAPT |
| IMIPRAMINE | 4 | MAPT |
| FURAZOLIDONE | 4 | MAPT |
| DROPERIDOL | 4 | MAPT |
| ARIPIPRAZOLE | 4 | MAPT |
| RALOXIFENE HYDROCHLORIDE | 4 | MAPT |
| IDARUBICIN | 4 | MAPT |
| ACETAMINOPHEN | 4 | MAPT |
| ACITRETIN | 4 | MAPT |
| CISPLATIN | 4 | MAPT |
| CLOBETASOL PROPIONATE | 4 | MAPT |
| AMINOSALICYLIC ACID | 4 | MAPT |
| TETRABENAZINE | 4 | MAPT |
Druggability pyramid
Cohort genes binned by druggability tier (high → low):
| Tier | Definition | Genes | Symbols |
|---|---|---|---|
| A | Approved (phase 4 drug) | 2 | MAPT, PSEN1 |
| B | Phased (≥1) drug, not yet approved | 0 | |
| C | Druggable family + PDB, no drug | 0 | |
| D | Druggable family + AlphaFold only, no drug | 0 | |
| E | Difficult family or no structure, no drug | 0 |
Undrugged target profiles
0 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).
Clinical trials & evidence
Clinical trials
Clinical trials: 161.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 122 |
| PHASE2 | 14 |
| PHASE1 | 8 |
| PHASE1/PHASE2 | 7 |
| PHASE4 | 4 |
| PHASE3 | 3 |
| EARLY_PHASE1 | 3 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00950430 | PHASE4 | ENROLLING_BY_INVITATION | Imaging of Brain Amyloid Plaques in the Aging Population |
| NCT06093126 | PHASE4 | RECRUITING | Lemborexant for Insomnia in a Patient With Dementia: An N-of-1 Trial |
| NCT00376051 | PHASE4 | COMPLETED | Serotonergic Function and Behavioural and Psychological Symptoms of Frontotemporal Dementia |
| NCT00545974 | PHASE4 | COMPLETED | Memantine (10mg BID) for the Frontal and Temporal Subtypes of Frontotemporal Dementia |
| NCT00594737 | PHASE3 | COMPLETED | Open Label Pilot Study of the Effects of Memantine on FDG-PET in Frontotemporal Dementia |
| NCT03682185 | PHASE3 | COMPLETED | The Healthy Patterns Sleep Study |
| NCT04374136 | PHASE3 | TERMINATED | A Phase 3 Study to Evaluate Efficacy and Safety of AL001 in Frontotemporal Dementia (INFRONT-3) |
| NCT04220021 | PHASE2 | ACTIVE_NOT_RECRUITING | Safety and Therapeutic Potential of the FDA-approved Drug Metformin for C9orf72 ALS/FTD |
| NCT04408625 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Phase 1/2 Clinical Trial of LY3884963 in Patients With Frontotemporal Dementia With Progranulin Mutations (FTD-GRN) |
| NCT04747431 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | A Study of PBFT02 in Participants With FTD and Mutations in the Granulin Precursor (GRN) or C9ORF72 Genes |
| NCT05262023 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | A Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of DNL593 in Healthy Participants and Participants With Frontotemporal Dementia (FTD-GRN) |
| NCT05374278 | PHASE1/PHASE2 | RECRUITING | First-in-Human Evaluation of an Astrocytic Glutamate Transporter (EAAT2) PET Tracer in Dementia |
| NCT05742698 | PHASE2 | RECRUITING | Nabilone for Agitation in Frontotemporal Dementia |
| NCT06064890 | PHASE1/PHASE2 | RECRUITING | A Study to Evaluate the Safety and Effect of AVB-101, a Gene Therapy Product, in Subjects With a Genetic Sub-type of Frontotemporal Dementia (FTD-GRN) |
| NCT06604520 | PHASE2 | RECRUITING | Vortioxetine for the Treatment of Mood and Cognitive Symptoms in Frontotemporal Dementia |
| NCT07154485 | PHASE2 | NOT_YET_RECRUITING | Investigator Initiated Study for the Safety and Efficacy in Frontotemporal Dementia |
| NCT00416169 | PHASE2 | COMPLETED | A Pilot Study to Explore the Safety and Tolerability of Galantamine HBr in the Treatment of Pick Complex/Frontotemporal Dementia |
| NCT01890343 | PHASE2 | COMPLETED | Imaging Characteristics of Florbetapir 18F in Patients With Frontotemporal Dementia, Alzheimer’s Disease and Normal Controls. |
| NCT01937013 | PHASE2 | COMPLETED | Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia |
| NCT02676843 | PHASE2 | COMPLETED | Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations |
| NCT02862210 | PHASE2 | COMPLETED | Low-Dose Lithium for the Treatment of Behavioral Symptoms in Frontotemporal Dementia |
| NCT03260920 | PHASE2 | UNKNOWN | Intranasal Oxytocin for Frontotemporal Dementia |
| NCT03987295 | PHASE2 | COMPLETED | A Phase 2 Study to Evaluate Safety of Long-term AL001 Dosing in Frontotemporal Dementia (FTD) Patients (INFRONT-2) |
| NCT04489017 | PHASE2 | COMPLETED | Palmitoylethanolamide Combined With Luteoline in Frontotemporal Dementia Patients. A Randomized Controlled Trial |
| NCT04931862 | PHASE1/PHASE2 | TERMINATED | Study of WVE-004 in Patients With C9orf72-associated Amyotrophic Lateral Sclerosis (ALS) or Frontotemporal Dementia (FTD) |
| NCT04937452 | PHASE2 | COMPLETED | Dopaminergic Therapy for Frontotemporal Dementia Patients |
| NCT04993755 | PHASE2 | COMPLETED | A Phase 2a Study of TPN-101 in Patients With C9ORF72 ALS/FTD |
| NCT05683860 | PHASE1/PHASE2 | TERMINATED | Open-label Extension (OLE) Study of WVE-004 in Patients With C9orf72-associated Amyotrophic Lateral Sclerosis (ALS) and/or Frontotemporal Dementia (FTD) |
| NCT05184569 | PHASE1 | RECRUITING | Veri-T: A Trial of Verdiperstat in Patients With svPPA Due to TDP-43 Pathology |
| NCT05315661 | PHASE1 | ACTIVE_NOT_RECRUITING | The Safety and The Efficacy Evaluation of ET-STEM in Patients With Frontotemporal Dementia |
| NCT01386333 | PHASE1 | COMPLETED | Safety Study of Intranasal Oxytocin in Frontotemporal Dementia |
| NCT01623284 | PHASE1 | COMPLETED | PiB PET Scanning in Speech and Language Based Dementias |
| NCT03040713 | PHASE1 | COMPLETED | Flortaucipir PET Imaging in Subjects With FTD |
| NCT03636204 | PHASE1 | COMPLETED | A First in Human Study in Healthy Volunteers and in Participants With Frontotemporal Dementia With Granulin (GRN) Mutation |
| NCT06226064 | PHASE1 | COMPLETED | A Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of VES001 in Healthy Participants |
| NCT06705192 | PHASE1 | COMPLETED | Study in Asymptomatic GRN-FTD Patients to Investigate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of VES001 |
| NCT06891716 | EARLY_PHASE1 | RECRUITING | [18F]ACI-19626 PET in TDP-43 Proteinopathies |
| NCT03510572 | EARLY_PHASE1 | COMPLETED | Evaluation of [18F]PI-2620 as a Potential Positron Emission Computed Tomography Radioligand for Imaging Tau Protein in the Brain |
| NCT03625128 | EARLY_PHASE1 | COMPLETED | 18F-PM-PBB3 PET Study in Tauopathy Including Alzheimer’s Disease, Other Dementias and Normal Controls |
| NCT01353430 | Not specified | RECRUITING | Characterization of Inclusion Body Myopathy Associated With Paget’s Disease of Bone and Frontotemporal Dementia (IBMPFD) |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CITALOPRAM | 4 | 3 |
| FLORBETAPIR | 4 | 3 |
| MEMANTINE | 4 | 3 |
| FLORTAUCIPIR F 18 | 4 | 2 |
| ESCITALOPRAM | 4 | 1 |
| FLUDEOXYGLUCOSE F 18 | 4 | 1 |
| FLUTEMETAMOL | 4 | 1 |
| GALANTAMINE HYDROBROMIDE | 4 | 1 |
| LEMBOREXANT | 4 | 1 |
| LITHIUM CARBONATE | 4 | 1 |
| NABILONE | 4 | 1 |
| ROTIGOTINE | 4 | 1 |
| VORTIOXETINE | 4 | 1 |
| LATOZINEMAB | 3 | 3 |
| FLUORIDE ION F-18 | 3 | 1 |
| LEUCINE | 3 | 1 |
| VERDIPERSTAT | 3 | 1 |
| COSFROVIXIMAB | 2 | 1 |
| PITTSBURGH COMPOUND B | 2 | 1 |
| CHEMBL498563 | 0 | 1 |
| CHEMBL5189046 | 0 | 1 |
| CHEMBL104383 | 0 | 1 |
| F-18 | 0 | 1 |
Related Atlas pages
- Cohort genes: MAPT, PSEN1
- Drugs: Citalopram, Florbetapir, Memantine, FLORTAUCIPIR F 18, Escitalopram, FLUDEOXYGLUCOSE F 18, Flutemetamol, Galantamine, Lemborexant, Lithium Carbonate, Nabilone, Rotigotine, Vortioxetine, Latozinemab, FLUORIDE ION F-18, Verdiperstat