DDPAC
MCID: FRN030
MIFTS: 24

Frontotemporal Dementia with Parkinsonism-17 (DDPAC)

Categories: Genetic diseases, Mental diseases, Neuronal diseases

Aliases & Classifications for Frontotemporal Dementia with Parkinsonism-17

MalaCards integrated aliases for Frontotemporal Dementia with Parkinsonism-17:

Name: Frontotemporal Dementia with Parkinsonism-17 25 71
Disinhibition-Dementia-Parkinsonism-Amytrophy Complex 25
Wilhelmsen-Lynch Disease 25
Frontotemporal Dementia 71
Familial Pick's Disease 25
Ftdp-17 25
Ddpac 25

Classifications:



External Ids:

UMLS 71 C0338451 C3811924

Summaries for Frontotemporal Dementia with Parkinsonism-17

Genetics Home Reference : 25 Frontotemporal dementia with parkinsonism-17 (FTDP-17) is a brain disorder. It is part of a group of conditions, called frontotemporal dementia or frontotemporal degeneration, that are characterized by a loss of nerve cells (neurons) in areas of the brain called the frontal and temporal lobes. Over time, a loss of these cells can affect personality, behavior, language, and movement. The signs and symptoms of FTDP-17 usually become noticeable in a person's forties or fifties. Most affected people survive 5 to 10 years after the appearance of symptoms, although a few have survived for two decades or more. Changes in personality and behavior are often early signs of FTDP-17. These changes include a loss of inhibition, inappropriate emotional responses, restlessness, neglect of personal hygiene, and a general loss of interest in activities and events. The disease also leads to deterioration of cognitive functions (dementia), including problems with judgment, planning, and concentration. Some people with FTDP-17 develop psychiatric symptoms, including obsessive-compulsive behaviors, strongly held false beliefs (delusions), and false perceptions (hallucinations). It may become difficult for affected individuals to interact with others in a socially appropriate manner. They increasingly require help with personal care and other activities of daily living. Many people with FTDP-17 develop problems with speech and language. They may have trouble finding words, confuse one word with another (semantic paraphasias), and repeat words spoken by others (echolalia). Difficulties with speech and language worsen over time, and most affected individuals eventually lose the ability to communicate. FTDP-17 is also characterized by problems with movement that worsen over time. Many affected individuals develop features of parkinsonism, including tremors, rigidity, and unusually slow movement (bradykinesia). As the disease progresses, most affected individuals become unable to walk. Some people with FTDP-17 also have restricted up-and-down eye movement (vertical gaze palsy) and rapid abnormal movements of both eyes (saccades).

MalaCards based summary : Frontotemporal Dementia with Parkinsonism-17, also known as disinhibition-dementia-parkinsonism-amytrophy complex, is related to frontotemporal dementia and grn-related frontotemporal dementia, and has symptoms including personality changes The drugs Citalopram and Corticosterone have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and testes.

Related Diseases for Frontotemporal Dementia with Parkinsonism-17

Graphical network of the top 20 diseases related to Frontotemporal Dementia with Parkinsonism-17:



Diseases related to Frontotemporal Dementia with Parkinsonism-17

Symptoms & Phenotypes for Frontotemporal Dementia with Parkinsonism-17

UMLS symptoms related to Frontotemporal Dementia with Parkinsonism-17:


personality changes

Drugs & Therapeutics for Frontotemporal Dementia with Parkinsonism-17

Drugs for Frontotemporal Dementia with Parkinsonism-17 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 109)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Citalopram Approved Phase 4 59729-33-8 2771
2
Corticosterone Experimental Phase 4 50-22-6 5753
3 Psychotropic Drugs Phase 4
4 Antidepressive Agents Phase 4
5 Serotonin Agents Phase 4
6 Serotonin Uptake Inhibitors Phase 4
7 Fluorodeoxyglucose F18 Phase 4
8 Anti-Inflammatory Agents Phase 4
9
Serotonin Investigational, Nutraceutical Phase 4 50-67-9 5202
10
tannic acid Approved Phase 3 1401-55-4
11
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
12
Miglustat Approved Phase 3 72599-27-0 51634
13
Pimavanserin Approved, Investigational Phase 3 706779-91-1 16058810
14
Methylene blue Approved, Investigational Phase 3 61-73-4
15
1-Deoxynojirimycin Investigational Phase 3 19130-96-2 1374
16 Hypoglycemic Agents Phase 3
17 Tranquilizing Agents Phase 3
18 Central Nervous System Depressants Phase 3
19 Antipsychotic Agents Phase 3
20 Anti-Retroviral Agents Phase 3
21 Cardiac Glycosides Phase 3
22 Glycoside Hydrolase Inhibitors Phase 3
23 Anti-HIV Agents Phase 3
24 Serotonin 5-HT2 Receptor Antagonists Phase 3
25 Serotonin Antagonists Phase 3
26
Galantamine Approved Phase 2 357-70-0 9651
27
Acetylcysteine Approved, Investigational Phase 1, Phase 2 616-91-1 12035
28
Vorinostat Approved, Investigational Phase 1, Phase 2 149647-78-9 5311
29
Oxytocin Approved, Vet_approved Phase 2 50-56-6 439302 53477758
30
Memantine Approved, Investigational Phase 2 19982-08-2 4054
31
Zinc Approved, Investigational Phase 2 7440-66-6 32051
32
Lithium carbonate Approved Phase 2 554-13-2
33
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
34
Busulfan Approved, Investigational Phase 2 55-98-1 2478
35
alemtuzumab Approved, Investigational Phase 2 216503-57-0
36
Cysteine Approved, Nutraceutical Phase 1, Phase 2 52-90-4 5862
37
Glycine Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 56-40-6 750
38
Emodepside Investigational, Vet_approved Phase 1, Phase 2 155030-63-0
39
Betadex Experimental Phase 1, Phase 2 7585-39-9 320761
40 Histone Deacetylase Inhibitors Phase 1, Phase 2
41 Nootropic Agents Phase 2
42 Cholinergic Agents Phase 2
43 Autonomic Agents Phase 2
44 Cholinesterase Inhibitors Phase 2
45 Sympathomimetics Phase 2
46 Catechol O-Methyltransferase Inhibitors Phase 2
47 Catechol Phase 2
48 Protective Agents Phase 2
49 N-monoacetylcystine Phase 1, Phase 2
50 Free Radical Scavengers Phase 1, Phase 2

Interventional clinical trials:

(show top 50) (show all 173)
# Name Status NCT ID Phase Drugs
1 A Prospective, Randomized, Multi-Center, Double-Blind, 26 Week, Placebo-Controlled Trial of Memantine (10mg BID) for the Frontal and Temporal Subtypes of Frontotemporal Dementia Completed NCT00545974 Phase 4 memantine;Placebo pill
2 Serotonergic Function and Behavioural and Psychological Symptoms of Frontotemporal Dementia Completed NCT00376051 Phase 4 Citalopram
3 Longitudinal Multi-Modality Imaging in Progressive Apraxia of Speech Recruiting NCT01818661 Phase 4 AV-1451
4 Brain Amyloid Imaging With Pittsburgh Compound B in Normal Aging, Mild Cognitive Impairment, and Dementia Enrolling by invitation NCT00950430 Phase 4 Pittsburgh Compound B (C-11 PiB);F-18 FDG;Tau (18-F-AV-1451)
5 A Training and Fidelity Model to Move and Scale Evidence-based Dementia Care and Caregiver Support Programs Into Practice: The Case for COPE in PACE Service Settings Not yet recruiting NCT04165213 Phase 4
6 Amantadine for the Treatment of Behavioral Disturbance in Frontotemporal Dementia (FTD) Withdrawn NCT00127114 Phase 4 Amantadine;Placebo
7 A Double-Blind, Placebo-Controlled, Randomized, Parallel Group, 12-Month Safety and Efficacy Trial of TRx0237 in Subjects With Behavioral Variant Frontotemporal Dementia (bvFTD) Completed NCT01626378 Phase 3 TRx0237;Placebo
8 An Open Label Pilot Study of the Effects of Memantine Administration on FDG-PET in Frontotemporal Dementia Completed NCT00594737 Phase 3 memantine hydrochloride
9 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3 Miglustat
10 A Double-blind, Placebo-controlled, Relapse Prevention Study of Pimavanserin for the Treatment of Hallucinations and Delusions Associated With Dementia-related Psychosis Completed NCT03325556 Phase 3 Placebo;Pimavanserin 34 mg;Pimavanserin 20 mg
11 The Role of Palliative Care Interventions to Reduce Circadian Rhythm Disorders in Persons With Dementia: The Healthy Patterns Study Recruiting NCT03682185 Phase 3
12 An Open-Label, Extension Study of the Effects of TRx0237 in Subjects With Alzheimer's Disease or Behavioral Variant Frontotemporal Dementia (bvFTD) Terminated NCT02245568 Phase 3 TRx0237
13 A Randomized, Double-Blind, Placebo-Controlled, Dose-Escalating, Phase 2a Safety, Tolerability, and Pharmacodynamic Study of Two Doses of an Histone Deacetylase Inhibitor (FRM-0334) in Subjects With Prodromal to Moderate Frontotemporal Dementia With Granulin Mutation Unknown status NCT02149160 Phase 2 FRM-0334;Placebo
14 Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia Completed NCT01937013 Phase 2 Intranasal oxytocin;Saline Nasal Mist
15 A Study Evaluating the Imaging Characteristics of Florbetapir 18F (18F-AV-45) in Patients With Frontotemporal Dementia Compared to Patients With Alzheimer's Disease and Normal Controls. Completed NCT01890343 Phase 2 florbetapir 18F;18F-FDG
16 An Open Pilot Study to Evaluate the Safety and Efficacy of Galantamine in the Treatment of Pick's Disease/Frontotemporal Dementia /Pick Complex Completed NCT00416169 Phase 2 galantamine hydrobromide
17 Double Blind Trial of DC Polarization in FTD Completed NCT00117858 Phase 2
18 Investigation of the Dopamine System in Frontotemporal Dementia Completed NCT00604591 Phase 2 Tolcapone;Placebo
19 Double-blind, Parallel Group, Placebo-controlled Trial of the Efficacy and Tolerability of Memantine (20 mg) in Frontotemporal Dementia (FTD) Patients Completed NCT00200538 Phase 2 memantine
20 Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations Completed NCT02676843 Phase 2 18F-AV-1451
21 Direct Current Brain Polarization for Apraxia in Corticobasal Syndrome Completed NCT00273897 Phase 2
22 Phase I/II Trial Of Hematopoietic Stem Cell Transplant (HSCT) For Children With A Genetic Disease Of Blood Cells Without An HLA-Matched Sibling Donor Completed NCT00730314 Phase 1, Phase 2
23 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2 Vorinostat
24 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2 N-Acetyl Cysteine
25 A Phase 2 Clinical Trial of Intranasal Oxytocin for Frontotemporal Dementia Recruiting NCT03260920 Phase 2 Syntocinon
26 A Phase 2, Multicenter, Open-Label Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of AL001 in Heterozygous Carriers of Granulin or C9ORF72 Mutations Causative of Frontotemporal Dementia Recruiting NCT03987295 Phase 2 AL001
27 A Single Center Feasibility Study of Intranasal Insulin in Frontotemporal Dementia NIFT-D Recruiting NCT04115384 Phase 2 Novolin-R insulin
28 Low-Dose Lithium for the Treatment of Behavioral Symptoms in Frontotemporal Dementia Recruiting NCT02862210 Phase 2 Lithium Carbonate;Placebo
29 Effects of N-Acetyl-L-Leucine on Niemann Pick Type C Disease: A Multinational, Multicenter, Open-label, Rater-blinded Phase II Study. Recruiting NCT03759639 Phase 2 IB1001
30 Phase 1/2a Study of 2-Hydroxypropyl-Beta-Cyclodextrin Therapy for Infantile Liver Disease Associated With Niemann-Pick Disease, Type C Recruiting NCT03471143 Phase 1, Phase 2 2-Hydroxypropyl-Beta-Cyclodextrin
31 Combined Intrathecal and Intravenous VTS-270 Therapy for Liver and Neurological Disease Associated With Niemann-Pick Disease, Type C1 Recruiting NCT03887533 Phase 1, Phase 2 VTS-270
32 A Phase I/II Study to Evaluate the Safety and PK of iv Trappsol Cyclo (HP-β-CD) in Patients With Niemann-Pick Disease Type C NPC-1 and the Pharmacodynamic Effects of Treatment Upon Markers of Cholesterol Metabolism and Clinical Outcomes Recruiting NCT02912793 Phase 1, Phase 2 Hydroxypropyl-beta-cyclodextrin
33 Multi-centered Double Blind, Placebo Controlled Study Evaluating the Safety and Efficacy of Memantine at 20 mg BID in Patients With ALS Recruiting NCT02118727 Phase 2 Memantine;Placebo (for Memantine)
34 Treating Primary Progressive Aphasia (PPA) and Elucidating Neurodegeneration in the Language Network Using Transcranial Direct Current Stimulation (tDCS) Not yet recruiting NCT04046991 Phase 2
35 Treatment of Lysosomal and Peroxisomal Inborn Errors of Metabolism by Hematopoietic Cell Transplantation Terminated NCT00668564 Phase 2 Cyclophosphamide;Campath-1H;Busulfan
36 F 18 T807 Tau PET Imaging of Frontotemporal Dementia Withdrawn NCT02707978 Phase 2 F 18 T807
37 Phase 1 Study to Determine the Efficacy of Using Far Infrared Radiation for the Management, Control and Treatment of Frontotemporal Dementia (Pick's Disease) Unknown status NCT00674960 Phase 1
38 18F-AV-1451 PET Imaging in Subjects With Frontotemporal Dementia Completed NCT03040713 Phase 1 18F-AV-1451;18F-AV-45
39 A Phase I Dose Finding Study of Intranasal Oxytocin in Frontotemporal Dementia, Protocol # FTDOXY10EF Completed NCT01386333 Phase 1 oxytocin;Saline Nasal Mist
40 Direct Current Brain Polarization in Frontotemporal Dementia Completed NCT00077896 Phase 1
41 A 12 Week Randomized, Double Blind, Placebo-Controlled Pilot Study of Davunetide (NAP, AL-108) in Predicted Tauopathies Completed NCT01056965 Phase 1 davunetide (AL-108, NAP);Placebo nasal spray
42 Evaluation of [18F]MNI-777 PET as a Marker of Tau Pathology in Subjects With Clinically Diagnosed Tauopathies in Comparison to Healthy Subjects Completed NCT02103894 Phase 1 [18F]T807 ([18F]MNI-777)
43 An Open Label Dose Finding Study of Nimodipine for the Treatment of Progranulin Insufficiency From GRN Gene Mutations Completed NCT01835665 Phase 1 Nimodipine
44 Amyloid-related Imaging Abnormalities (Microbleeds) in Atypical AD Completed NCT01723553 Phase 1 C-11 PiB
45 Hydroxypropyl Beta Cyclodextrin for Niemann-Pick Type C1 Disease Completed NCT01747135 Phase 1 VTS-270
46 A Phase I Study to Evaluate the Single and Multiple-dose Pharmacokinetics of Intravenous Trappsol Cyclo (HP-Beta-CD) in Patients With Niemann-Pick Disease Type C (NPC-1) and the Effects of Dosing Upon Biomarkers of NPC Disease Completed NCT02939547 Phase 1 Hydroxypropyl-beta-cyclodextrin
47 PiB PET Scanning in Speech and Language Based Dementias Completed NCT01623284 Phase 1 C-11 PiB;F-18 FDG
48 A First in Human Phase 1 Study in Healthy Volunteers and in Participants With Frontotemporal Dementia (FTD) With Granulin Mutation Recruiting NCT03636204 Phase 1
49 Tau PET Imaging in Atypical Dementias Recruiting NCT03283449 Phase 1 18F-AV-1451
50 PET Imaging of Neuroinflammation in Neurodegenerative Diseases Via a Novel TSPO Radioligand Recruiting NCT03958630 Phase 1 11C-ER176;11C-PIB

Search NIH Clinical Center for Frontotemporal Dementia with Parkinsonism-17

Genetic Tests for Frontotemporal Dementia with Parkinsonism-17

Anatomical Context for Frontotemporal Dementia with Parkinsonism-17

MalaCards organs/tissues related to Frontotemporal Dementia with Parkinsonism-17:

40
Brain, Eye, Testes, Temporal Lobe, Liver, Cortex, Bone

Publications for Frontotemporal Dementia with Parkinsonism-17

Articles related to Frontotemporal Dementia with Parkinsonism-17:

(show all 12)
# Title Authors PMID Year
1
4',6-Diamidino-2-Phenylindole Distinctly Labels Tau Deposits. 61
30106598 2018
2
Tau in neurodegenerative disease. 61
29951497 2018
3
Profiling of Argonaute-2-loaded microRNAs in a mouse model of frontotemporal dementia with parkinsonism-17. 61
30697364 2018
4
Frontotemporal Dementia-Associated N279K Tau Mutation Localizes at the Nuclear Compartment. 61
30050413 2018
5
Characterization of tau positron emission tomography tracer [18F]AV-1451 binding to postmortem tissue in Alzheimer's disease, primary tauopathies, and other dementias. 61
26892233 2016
6
Accelerated neurodegeneration and neuroinflammation in transgenic mice expressing P301L tau mutant and tau-tubulin kinase 1. 61
24418258 2014
7
Cerebral hypometabolism and grey matter density in MAPT intron 10 +3 mutation carriers. 61
25628962 2014
8
Adult-onset focal expression of mutated human tau in the hippocampus impairs spatial working memory of rats. 61
22561128 2012
9
Familial semantic dementia with P301L mutation in the Tau gene. 61
21555888 2011
10
Differential effects of Tau on the integrity and function of neurons essential for learning in Drosophila. 61
20071510 2010
11
U-box protein carboxyl terminus of Hsc70-interacting protein (CHIP) mediates poly-ubiquitylation preferentially on four-repeat Tau and is involved in neurodegeneration of tauopathy. 61
15447663 2004
12
A new eponym: Wilhelmsen-Lynch disease. 61
7936262 1994

Variations for Frontotemporal Dementia with Parkinsonism-17

Expression for Frontotemporal Dementia with Parkinsonism-17

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Pathways for Frontotemporal Dementia with Parkinsonism-17

GO Terms for Frontotemporal Dementia with Parkinsonism-17

Sources for Frontotemporal Dementia with Parkinsonism-17

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7 CNVD
9 Cosmic
10 dbSNP
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35 IUPHAR
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37 LifeMap
39 LOVD
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43 MeSH
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48 NCI
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50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
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63 QIAGEN
68 SNOMED-CT via HPO
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