MCID: FRN030
MIFTS: 31

Frontotemporal Dementia with Parkinsonism-17

Categories: Genetic diseases, Mental 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 73
Disinhibition-Dementia-Parkinsonism-Amytrophy Complex 25
Wilhelmsen-Lynch Disease 25
Frontotemporal Dementia 73
Familial Pick's Disease 25
Ftdp-17 25
Ddpac 25

Classifications:



External Ids:

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.

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 Dopamine and Memantine have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and testes.

Related Diseases for Frontotemporal Dementia with Parkinsonism-17

Diseases related to Frontotemporal Dementia with Parkinsonism-17 via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 frontotemporal dementia 11.3
2 grn-related frontotemporal dementia 10.9
3 dementia 10.4

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 121)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4,Phase 3,Phase 2,Phase 1 51-61-6, 62-31-7 681
2
Memantine Approved, Investigational Phase 4,Phase 3,Phase 2 19982-08-2 4054
3
Citalopram Approved Phase 4 59729-33-8 2771
4
Iodine Approved, Investigational Phase 4 7553-56-2 807
5
Corticosterone Experimental Phase 4,Phase 1,Early Phase 1 50-22-6 5753
6 Fluorodeoxyglucose F18 Phase 4,Phase 2,Phase 1,Not Applicable
7 Neurotransmitter Agents Phase 4,Phase 3,Phase 2
8 Dopamine Agents Phase 4,Phase 3,Phase 2,Phase 1
9 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 1
10 Antiparkinson Agents Phase 4,Phase 3,Phase 2
11 Radiopharmaceuticals Phase 4,Phase 2,Not Applicable
12 Serotonin Agents Phase 4,Phase 3
13 Serotonin Uptake Inhibitors Phase 4
14 Neurotransmitter Uptake Inhibitors Phase 4
15 Excitatory Amino Acid Antagonists Phase 4,Phase 3,Phase 2
16 Excitatory Amino Acids Phase 4,Phase 3,Phase 2
17 Antidepressive Agents Phase 4,Phase 2,Phase 1
18 Antidepressive Agents, Second-Generation Phase 4
19 Anti-Infective Agents Phase 4,Phase 3,Phase 1,Phase 2
20 Psychotropic Drugs Phase 4,Phase 3,Phase 2,Phase 1
21 Antiviral Agents Phase 4,Phase 3,Phase 1,Phase 2
22 Analgesics Phase 4,Phase 1
23 Analgesics, Non-Narcotic Phase 4,Phase 1
24 Pharmaceutical Solutions Phase 4,Phase 2,Phase 3,Phase 1
25 Anti-Inflammatory Agents Phase 4,Phase 1
26 cadexomer iodine Phase 4
27
Serotonin Investigational, Nutraceutical Phase 4,Phase 3 50-67-9 5202
28 Calamus Nutraceutical Phase 4
29
Methylene blue Approved, Investigational Phase 3 61-73-4
30
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
31
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
32
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
33
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
34
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
35
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
36
Pimavanserin Approved, Investigational Phase 3 706779-91-1 16058810
37
Deferiprone Approved Phase 2, Phase 3 30652-11-0 2972
38
Iron Approved Phase 2, Phase 3 7439-89-6 23925
39 tannic acid Approved, Nutraceutical Phase 3
40
1-Deoxynojirimycin Experimental Phase 3,Phase 2 19130-96-2 1374
41 Prednisolone acetate Phase 2, Phase 3
42 Alkylating Agents Phase 2, Phase 3
43 Methylprednisolone acetate Phase 2, Phase 3
44 Methylprednisolone Hemisuccinate Phase 2, Phase 3
45 Glycoside Hydrolase Inhibitors Phase 3,Phase 2,Phase 1
46 Hypoglycemic Agents Phase 3,Phase 2,Phase 1
47 Immunosuppressive Agents Phase 2, Phase 3
48 Prednisolone hemisuccinate Phase 2, Phase 3
49 Prednisolone phosphate Phase 2, Phase 3
50 Anti-HIV Agents Phase 3,Phase 2,Phase 1

Interventional clinical trials:

(show top 50) (show all 161)
# Name Status NCT ID Phase Drugs
1 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 Imaging of Brain Amyloid Plaques in the Aging Population Enrolling by invitation NCT00950430 Phase 4 Pittsburgh Compound B (C-11 PiB);F-18 FDG;Tau (18-F-AV-1451)
5 DaTSCAN Imaging in Aging and Neurodegenerative Disease Enrolling by invitation NCT01453127 Phase 4 I-123 Ioflupane solution injection prior to SPECT scan (DaTscan)
6 Amantadine for the Treatment of Behavioral Disturbance in Frontotemporal Dementia (FTD) Withdrawn NCT00127114 Phase 4 Amantadine;Placebo
7 Safety and Efficacy Study Evaluating TRx0237 in Subjects With Behavioral Variant Frontotemporal Dementia (bvFTD) Completed NCT01626378 Phase 3 TRx0237;Placebo
8 Open Label Pilot Study of the Effects of Memantine 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 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3 Busulfan, Cyclophosphamide, Antithymocyte Globulin
11 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Recruiting NCT02004691 Phase 2, Phase 3 placebo (saline);GZ402665
12 Relapse Prevention Study of Pimavanserin in Dementia-related Psychosis Recruiting NCT03325556 Phase 3 Placebo;Pimavanserin 34 mg;Pimavanserin 20 mg
13 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3 arimoclomol;Placebo
14 Study of VTS-270 (2-hydroxypropyl-β-cyclodextrin) to Treat Niemann-Pick Type C1 (NPC1) Disease Active, not recruiting NCT02534844 Phase 2, Phase 3 VTS-270;Sham Procedure Control
15 SurVival of Lysosomal Acid Lipase Deficiency (LAL-D) Infants Treated With SebelipAse aLfa Active, not recruiting NCT01371825 Phase 2, Phase 3 Sebelipase alfa (SBC-102)
16 Conservative Iron Chelation as a Disease-modifying Strategy in Amyotrophic Lateral Sclerosis Not yet recruiting NCT03293069 Phase 2, Phase 3 Deferiprone;Placebo Oral Tablet
17 Open-Label Study of TRx0237 in Subjects With Alzheimer's Disease or Behavioral Variant Frontotemporal Dementia (bvFTD) Terminated NCT02245568 Phase 3 TRx0237
18 ALD-101 Adjuvant Therapy of Unrelated Umbilical Cord Blood Transfusion (UCBT) in Patients With Inherited Metabolic Diseases Terminated NCT00654433 Phase 3
19 Children With Lysosomal Acid Lipase Deficiency Who Previously Received Treatment With SBC-102 Terminated NCT01473875 Phase 2, Phase 3 SBC-102
20 Study to Assess the Safety, Tolerability, and Pharmacodynamic (PD) Effects of FRM-0334 in Subjects With Prodromal to Moderate Frontotemporal Dementia With Granulin Mutation Unknown status NCT02149160 Phase 2 FRM-0334;Placebo
21 Effects of Tolcapone on Frontotemporal Dementia Unknown status NCT00604591 Phase 2 Tolcapone;Placebo
22 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2 Vorinostat
23 A Pilot Study to Explore the Safety and Tolerability of Galantamine HBr in the Treatment of Pick Complex/Frontotemporal Dementia Completed NCT00416169 Phase 2 galantamine hydrobromide
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 Efficacy and Tolerability of Memantine in Frontotemporal Dementia (FTD) Patients Completed NCT00200538 Phase 2 memantine
26 Imaging Characteristics of Florbetapir 18F in Patients With Frontotemporal Dementia, Alzheimer's Disease and Normal Controls. Completed NCT01890343 Phase 2 florbetapir 18F;18F-FDG
27 Double Blind Trial of DC Polarization in FTD Completed NCT00117858 Phase 2
28 Davunetide (AL-108) in Predicted Tauopathies - Pilot Study Completed NCT01056965 Phase 2 davunetide (AL-108, NAP);Placebo nasal spray
29 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
30 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2 miglustat
31 Intranasal Oxytocin for Frontotemporal Dementia Recruiting NCT03260920 Phase 2 Syntocinon
32 Low-Dose Lithium for the Treatment of Behavioral Symptoms in Frontotemporal Dementia Recruiting NCT02862210 Phase 2 Lithium Carbonate;Placebo
33 Study of IV VTS-270 for Infantile Liver Disease Associated With Niemann-Pick Disease, Type C Recruiting NCT03471143 Phase 1, Phase 2 2-Hydroxypropyl-Beta-Cyclodextrin
34 Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia Recruiting NCT01937013 Phase 2 Intranasal oxytocin;Saline Nasal Mist
35 Study of Pharmacokinetics and Preliminary Efficacy in Patients With Niemann-Pick C1 Recruiting NCT02912793 Phase 1, Phase 2 Hydroxypropyl-beta-cyclodextrin
36 Study of Lithium Carbonate to Treat Niemann-Pick Type C1 Disease Recruiting NCT03201627 Phase 1, Phase 2 Lithium Carbonate
37 Safety, Tolerability, PK, and Efficacy Evaluation of Repeat Ascending Doses of Olipudase Alfa in Pediatric Patients <18 Years of Age With Acid Sphingomyelinase Deficiency Recruiting NCT02292654 Phase 1, Phase 2 Olipudase alfa
38 F 18 T807 Tau PET Imaging in Familial Amyotrophic Lateral Sclerosis Active, not recruiting NCT02414230 Phase 2 Drug: F 18 T807
39 Phase I/II Pilot Study of Mixed Chimerism to Treat Inherited Metabolic Disorders Active, not recruiting NCT01372228 Phase 1, Phase 2
40 Clinical Trial in Infants With Rapidly Progressive Lysosomal Acid Lipase Deficiency Active, not recruiting NCT02193867 Phase 2 sebelipase alfa
41 F 18 T807 Tau PET Imaging of Progressive Posterior Cortical Dysfunction (IND 123119, Protocol E) Enrolling by invitation NCT02414282 Phase 2 F 18 T807
42 Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations Enrolling by invitation NCT02676843 Phase 2 18F-AV-1451
43 A Long-Term Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Enrolling by invitation NCT02004704 Phase 2 GZ402665
44 Hematopoietic Stem Cell Transplantation (HCT) for Inborn Errors of Metabolism Terminated NCT00668564 Phase 2 Cyclophosphamide;Campath-1H;Busulfan
45 F 18 T807 Tau PET Imaging of Frontotemporal Dementia (FTD) Withdrawn NCT02707978 Phase 2 F 18 T807
46 Far Infrared Irradiation for the Management, Control and Treatment of Frontotemporal Dementia Unknown status NCT00674960 Phase 1
47 Far Infrared Radiation Treatment of Dementia and Other Mental Illness Unknown status NCT00574054 Phase 1
48 Safety Study of Intranasal Oxytocin in Frontotemporal Dementia Completed NCT01386333 Phase 1 oxytocin;Saline Nasal Mist
49 Direct Current Brain Polarization in Frontotemporal Dementia Completed NCT00077896 Phase 1
50 Hydroxypropyl Beta Cyclodextrin for Niemann-Pick Type C1 Disease Completed NCT01747135 Phase 1 VTS-270

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:

41
Brain, Temporal Lobe, Testes, Bone, Cortex, Liver, Eye

Publications for Frontotemporal Dementia with Parkinsonism-17

Articles related to Frontotemporal Dementia with Parkinsonism-17:

(show top 50) (show all 1127)
# Title Authors Year
1
Enhanced Positive Emotional Reactivity Undermines Empathy in Behavioral Variant Frontotemporal Dementia. ( 29915557 )
2018
2
Executive and social-cognitive determinants of environmental dependency syndrome in behavioral frontotemporal dementia. ( 29809029 )
2018
3
Meaning of psychiatric symptoms in frontotemporal dementia. ( 29439161 )
2018
4
Strategic value-directed learning and memory in Alzheimer's disease and behavioural-variant frontotemporal dementia. ( 29431279 )
2018
5
Molecular Genetics of Frontotemporal Dementia Elucidated by <i>Drosophila</i> Models-Defects in Endosomala8>Lysosomal Pathway. ( 29890743 )
2018
6
Correction: Immune-related genetic enrichment in frontotemporal dementia: An analysis of genome-wide association studies. ( 29377952 )
2018
7
Diagnosing pre-clinical dementia: the NZ Genetic Frontotemporal Dementia Study (FTDGeNZ). ( 29879731 )
2018
8
Trichotillomania Ranging from &amp;quot;Ritual to Illness&amp;quot; and as a Rare Clinical Manifestation of Frontotemporal Dementia: Review of Literature and Case Report. ( 29769783 )
2018
9
Affective Empathy in Behavioral Variant Frontotemporal Dementia: A Meta-Analysis. ( 29946291 )
2018
10
Frontotemporal dementia with Parkinsonism linked to chromosome-17 mutations enhance tau oligomer formation. ( 29852407 )
2018
11
Lipid Metabolism and Survival Across the Frontotemporal Dementia-Amyotrophic Lateral Sclerosis Spectrum: Relationships to Eating Behavior and Cognition. ( 29254092 )
2018
12
Plasma tau is increased in frontotemporal dementia. ( 29440230 )
2018
13
Combined Socio-Behavioral Evaluation Improves the Differential Diagnosis Between the Behavioral Variant of Frontotemporal Dementia and Alzheimer's Disease: In Search of Neuropsychological Markers. ( 29254091 )
2018
14
Regional structural hypo- and hyperconnectivity of frontal-striatal and frontal-thalamic pathways in behavioral variant frontotemporal dementia. ( 29923666 )
2018
15
Autoimmunity and Frontotemporal Dementia. ( 29357796 )
2018
16
Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis. ( 29942091 )
2018
17
Progranulin Gene Therapy Improves Lysosomal Dysfunction and Microglial Pathology Associated with Frontotemporal Dementia and Neuronal Ceroid Lipofuscinosis. ( 29378861 )
2018
18
Caregiver Burden in Semantic Dementia with Right- and Left-Sided Predominant Cerebral Atrophy and in Behavioral-Variant Frontotemporal Dementia. ( 29706989 )
2018
19
Lacosamide in the Management of Behavioral Symptoms in Frontotemporal Dementia: A 2-Case Report. ( 29389672 )
2018
20
Thalamic atrophy in frontotemporal dementia - Not just a <i>C9orf72</i> problem. ( 29876259 )
2018
21
Functional Connectivity Changes in Behavioral, Semantic, and Nonfluent Variants of Frontotemporal Dementia. ( 29808100 )
2018
22
Dysregulation and Dislocation of SFPQ Disturbed DNA Organization in Alzheimer's Disease and Frontotemporal Dementia. ( 29376859 )
2018
23
Association between TBK1 mutations and risk of amyotrophic lateral sclerosis/frontotemporal dementia spectrum: a meta-analysis. ( 29349657 )
2018
24
Validity and Reliability of the Frontotemporal Dementia Rating Scale (FTD-FRS) for the Progression and Staging of Dementia in Brazilian Patients. ( 29438114 )
2018
25
Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum. ( 29868980 )
2018
26
The Pro-Apoptotic JNK Scaffold POSH/SH3RF1 Mediates CHMP2BIntron5-Associated Toxicity in Animal Models of Frontotemporal Dementia. ( 29432529 )
2018
27
Relevance of raised cerebrospinal fluid monocyte levels in patients with frontotemporal dementia. ( 29107846 )
2018
28
Connectivity-based characterisation of subcortical grey matter pathology in frontotemporal dementia and ALS: a multimodal neuroimaging study. ( 29423814 )
2018
29
Mutation Screening of the CHCHD2 Gene for Alzheimer's Disease and Frontotemporal Dementia in Chinese Mainland Population. ( 29376860 )
2018
30
A Modified Reading the Mind in the Eyes Test Predicts Behavioral Variant Frontotemporal Dementia Better Than Executive Function Tests. ( 29441012 )
2018
31
Parkinsonism is associated with altered primary motor cortex plasticity in frontotemporal dementia-primary progressive aphasia variant. ( 29909180 )
2018
32
Facial Emotion Recognition Performance Differentiates Between Behavioral Variant Frontotemporal Dementia and Major Depressive Disorder. ( 29360290 )
2018
33
Publisher Correction: Anti-AMPA GluA3 antibodies in Frontotemporal dementia: a new molecular target. ( 29305592 )
2018
34
Psychiatric symptoms in preclinical behavioural-variant frontotemporal dementia in<i>MAPT</i>mutation carriers. ( 29353234 )
2018
35
Comparative transcriptomics of choroid plexus in Alzheimer's disease, frontotemporal dementia and Huntington's disease: implications for CSF homeostasis. ( 29848382 )
2018
36
A Case of Rapidly Progressing Frontotemporal Dementia. ( 29403137 )
2018
37
Behavioral Variant Frontotemporal Dementia as a Serious Complication of Spontaneous Intracranial Hypotension. ( 29534203 )
2018
38
Asymmetry of post-mortem neuropathology in behavioural-variant frontotemporal dementia. ( 29228211 )
2018
39
Alzheimer's Disease and Frontotemporal Dementia: The Current State of Genetics and Genetic Testing Since the Advent of Next-Generation Sequencing. ( 29971646 )
2018
40
Slowly progressive behavioral frontotemporal dementia with C9orf72 mutation. Case report and review of the literature. ( 29355451 )
2018
41
Hepatitis B core VLP-based mis-disordered tau vaccine elicits strong immune response and alleviates cognitive deficits and neuropathology progression in Tau.P301S mouse model of Alzheimer's disease and frontotemporal dementia. ( 29914543 )
2018
42
Empathy in Frontotemporal Dementia. ( 29927802 )
2018
43
Early-stage right temporal lobe variant of frontotemporal dementia: 3 years of follow-up observations. ( 29960960 )
2018
44
Social and cognitive control skills in long-life occupation activities modulate the brain reserve in the behavioural variant of frontotemporal dementia. ( 29328983 )
2018
45
Hippocampal Subfield Volumetry: Differential Pattern of Atrophy in Different Forms of Genetic Frontotemporal Dementia. ( 29889066 )
2018
46
Beyond the face: how context modulates emotion processing in frontotemporal dementia subtypes. ( 29394332 )
2018
47
Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity. ( 29855382 )
2018
48
Distinct Clinical Features and Outcomes in Motor Neuron Disease Associated with Behavioural Variant Frontotemporal Dementia. ( 29886477 )
2018
49
Frontotemporal Dementia. ( 28410663 )
2017
50
Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder. ( 28916614 )
2017

Variations for Frontotemporal Dementia with Parkinsonism-17

Expression for Frontotemporal Dementia with Parkinsonism-17

Search GEO for disease gene expression data for Frontotemporal Dementia with Parkinsonism-17.

Pathways for Frontotemporal Dementia with Parkinsonism-17

GO Terms for Frontotemporal Dementia with Parkinsonism-17

Sources for Frontotemporal Dementia with Parkinsonism-17

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
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30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
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37 KEGG
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44 MeSH
45 MESH via Orphanet
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49 NCI
50 NCIt
51 NDF-RT
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55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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