MCID: BHV002
MIFTS: 53

Behavioral Variant of Frontotemporal Dementia

Categories: Mental diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Behavioral Variant of Frontotemporal Dementia

MalaCards integrated aliases for Behavioral Variant of Frontotemporal Dementia:

Name: Behavioral Variant of Frontotemporal Dementia 54 60
Dementia with Lobar Atrophy and Neuronal Cytoplasmic Inclusions 54
Lobar Atrophy of the Brain 54
Pick Disease of the Brain 54
Pick's Disease 54
Bv-Ftd 60
Bvftd 54

Characteristics:

Orphanet epidemiological data:

60
behavioral variant of frontotemporal dementia
Inheritance: Autosomal dominant; Age of onset: Adult; Age of death: elderly;

Classifications:

Orphanet: 60  
Rare neurological diseases


Summaries for Behavioral Variant of Frontotemporal Dementia

NIH Rare Diseases : 54 Pick�??s disease is a neurological condition characterized by a slowly progressive deterioration of behavior, personality, or language. People with Pick's disease have abnormal substances (called Pick bodies) inside nerve cells in the damaged areas of the brain. Pick bodies contain an abnormal form of a protein called tau. This protein is found in all nerve cells, but people with Pick's disease have an abnormal amount or type of this protein. Symptoms often present sometime in the 50s, though it can occur as early as age 20 or as late as age 80. The course of the disease varies from person to person. The underlying cause of Pick's disease is unknown. In some cases, the disease runs in families. While there is no treatment to slow the progression of the disease, medications can be used to treat individual symptoms.

MalaCards based summary : Behavioral Variant of Frontotemporal Dementia, also known as dementia with lobar atrophy and neuronal cytoplasmic inclusions, is related to pick disease of brain and supranuclear palsy, progressive, 1. An important gene associated with Behavioral Variant of Frontotemporal Dementia is SQSTM1 (Sequestosome 1), and among its related pathways/superpathways are Neuroscience and Cytoskeletal Signaling. The drugs Dopamine and Citalopram have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and testes, and related phenotypes are dysphasia and stereotypy

Related Diseases for Behavioral Variant of Frontotemporal Dementia

Diseases related to Behavioral Variant of Frontotemporal Dementia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 65)
# Related Disease Score Top Affiliating Genes
1 pick disease of brain 32.5 GRN MAPT PSEN1 SQSTM1
2 supranuclear palsy, progressive, 1 30.7 GRN MAPT
3 alzheimer disease 30.4 GRN MAPT PSEN1 SQSTM1
4 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 30.3 GRN MAPT
5 lateral sclerosis 30.1 C9orf72 SQSTM1 VCP
6 kluver-bucy syndrome 30.1 MAPT PSEN1
7 multiple system atrophy 1 29.9 MAPT SQSTM1
8 apraxia 29.8 C9orf72 GRN MAPT PSEN1
9 motor neuron disease 29.1 C9orf72 GRN MAPT SQSTM1 VCP
10 aphasia 29.0 C9orf72 CHMP2B GRN MAPT PSEN1
11 frontotemporal dementia 28.5 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1
12 dementia 28.5 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1
13 progressive non-fluent aphasia 27.6 C9orf72 CHMP2B GRN MAPT PSEN1 TMEM106B
14 amyotrophic lateral sclerosis 1 27.3 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1
15 frontotemporal dementia with parkinsonism-17 11.2
16 corticobasal degeneration 10.5
17 cognitive function 1, social 10.3
18 phonagnosia 10.2 GRN PSEN1
19 associative agnosia 10.2 GRN PSEN1
20 patulous eustachian tube 10.2
21 writing disorder 10.2 GRN PSEN1
22 prosopagnosia 10.2 GRN PSEN1
23 gait apraxia 10.2 GRN PSEN1
24 ideomotor apraxia 10.2 GRN MAPT
25 down syndrome 10.1
26 choreatic disease 10.1
27 visual agnosia 10.1 GRN PSEN1
28 kohlschutter-tonz syndrome 10.1 MAPT PSEN1
29 perry syndrome 10.1 C9orf72 GRN
30 amyotrophic lateral sclerosis 21 10.1 C9orf72 VCP
31 alzheimer disease 2 10.1 MAPT PSEN1
32 mammary paget's disease 10.1 SQSTM1 VCP
33 schizophrenia 10.1
34 tay-sachs disease 10.1
35 aging 10.1
36 constrictive pericarditis 10.1
37 pericarditis 10.1
38 early-onset, autosomal dominant alzheimer disease 10.1 PSEN1 TREM2
39 huntington disease 10.0
40 intermittent claudication 10.0
41 cerebral amyloid angiopathy, cst3-related 10.0 MAPT PSEN1
42 nervous system disease 10.0 C9orf72 MAPT PSEN1
43 echolalia 10.0 GRN MAPT PSEN1
44 agraphia 10.0 GRN MAPT PSEN1
45 paget's disease of bone 10.0 SQSTM1 VCP
46 dementia, lewy body 9.9 GRN MAPT PSEN1
47 mutism 9.9 CHMP2B GRN
48 vascular dementia 9.9 MAPT PSEN1
49 creutzfeldt-jakob disease 9.9
50 hydrocephalus, normal-pressure 9.9

Graphical network of the top 20 diseases related to Behavioral Variant of Frontotemporal Dementia:



Diseases related to Behavioral Variant of Frontotemporal Dementia

Symptoms & Phenotypes for Behavioral Variant of Frontotemporal Dementia

Human phenotypes related to Behavioral Variant of Frontotemporal Dementia:

60 33 (show all 40)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dysphasia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002357
2 stereotypy 60 33 hallmark (90%) Very frequent (99-80%) HP:0000733
3 irritability 60 33 hallmark (90%) Very frequent (99-80%) HP:0000737
4 thickened nuchal skin fold 60 33 hallmark (90%) Very frequent (99-80%) HP:0000474
5 memory impairment 60 33 hallmark (90%) Very frequent (99-80%) HP:0002354
6 aggressive behavior 60 33 hallmark (90%) Very frequent (99-80%) HP:0000718
7 dyslexia 60 33 hallmark (90%) Very frequent (99-80%) HP:0010522
8 dysgraphia 60 33 hallmark (90%) Very frequent (99-80%) HP:0010526
9 frontotemporal cerebral atrophy 60 33 hallmark (90%) Very frequent (99-80%) HP:0006892
10 restlessness 60 33 hallmark (90%) Very frequent (99-80%) HP:0000711
11 disinhibition 60 33 hallmark (90%) Very frequent (99-80%) HP:0000734
12 personality changes 60 33 hallmark (90%) Very frequent (99-80%) HP:0000751
13 frontotemporal dementia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002145
14 inappropriate behavior 60 33 hallmark (90%) Very frequent (99-80%) HP:0000719
15 echolalia 60 33 hallmark (90%) Very frequent (99-80%) HP:0010529
16 loss of speech 60 33 hallmark (90%) Very frequent (99-80%) HP:0002371
17 dyscalculia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002442
18 poor speech 60 33 hallmark (90%) Very frequent (99-80%) HP:0002465
19 perseveration 60 33 hallmark (90%) Very frequent (99-80%) HP:0030223
20 hyperorality 60 33 hallmark (90%) Very frequent (99-80%) HP:0000710
21 restrictive behavior 60 33 hallmark (90%) Very frequent (99-80%) HP:0000723
22 lack of insight 60 33 hallmark (90%) Very frequent (99-80%) HP:0000757
23 emotional blunting 60 33 hallmark (90%) Very frequent (99-80%) HP:0030213
24 abnormal brain fdg positron emission tomography 60 33 frequent (33%) Frequent (79-30%) HP:0012658
25 abnormality of the cerebral white matter 60 33 frequent (33%) Frequent (79-30%) HP:0002500
26 eeg with continuous slow activity 60 33 frequent (33%) Frequent (79-30%) HP:0011204
27 collectionism 60 33 frequent (33%) Frequent (79-30%) HP:0030212
28 gait disturbance 60 33 occasional (7.5%) Occasional (29-5%) HP:0001288
29 hyperreflexia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001347
30 generalized tonic-clonic seizures 60 33 occasional (7.5%) Occasional (29-5%) HP:0002069
31 psychosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000709
32 fasciculations 60 33 occasional (7.5%) Occasional (29-5%) HP:0002380
33 abnormality of extrapyramidal motor function 60 33 occasional (7.5%) Occasional (29-5%) HP:0002071
34 astrocytosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0002446
35 upper motor neuron dysfunction 60 33 occasional (7.5%) Occasional (29-5%) HP:0002493
36 apathy 60 33 occasional (7.5%) Occasional (29-5%) HP:0000741
37 mutism 60 33 occasional (7.5%) Occasional (29-5%) HP:0002300
38 abulia 60 33 occasional (7.5%) Occasional (29-5%) HP:0012671
39 behavioral abnormality 60 Very frequent (99-80%)
40 mental deterioration 60 Very frequent (99-80%)

MGI Mouse Phenotypes related to Behavioral Variant of Frontotemporal Dementia:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.87 C9orf72 GRN MAPT PSEN1 SQSTM1 TREM2
2 growth/size/body region MP:0005378 9.8 C9orf72 GRN MAPT PSEN1 SQSTM1 TMEM106B
3 hematopoietic system MP:0005397 9.7 C9orf72 GRN MAPT PSEN1 SQSTM1 TREM2
4 immune system MP:0005387 9.5 C9orf72 GRN MAPT PSEN1 SQSTM1 TREM2
5 nervous system MP:0003631 9.23 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1

Drugs & Therapeutics for Behavioral Variant of Frontotemporal Dementia

Drugs for Behavioral Variant of Frontotemporal Dementia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 141)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 62-31-7, 51-61-6 681
2
Citalopram Approved Phase 4 59729-33-8 2771
3
Memantine Approved, Investigational Phase 4,Phase 3,Phase 2 19982-08-2 4054
4
Miglustat Approved Phase 4,Phase 3,Phase 2,Phase 1 72599-27-0 51634
5
Iodine Approved, Investigational Phase 4 7553-56-2 807
6
Corticosterone Experimental Phase 4,Phase 1,Early Phase 1 50-22-6 5753
7
1-Deoxynojirimycin Investigational Phase 4,Phase 3,Phase 2 19130-96-2 1374
8 Neurotransmitter Agents Phase 4,Phase 3,Phase 2
9 Dopamine Agents Phase 4,Phase 3,Phase 2
10 Antiparkinson Agents Phase 4,Phase 3,Phase 2
11 Fluorodeoxyglucose F18 Phase 4,Phase 2,Phase 1,Not Applicable
12 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 1
13 Antidepressive Agents Phase 4,Phase 1,Phase 2
14 Neurotransmitter Uptake Inhibitors Phase 4
15 Serotonin Uptake Inhibitors Phase 4
16 Psychotropic Drugs Phase 4,Phase 3,Phase 1,Phase 2
17 Serotonin Agents Phase 4,Phase 3
18 Antidepressive Agents, Second-Generation Phase 4
19 Excitatory Amino Acid Antagonists Phase 4,Phase 3,Phase 2
20 Excitatory Amino Acids Phase 4,Phase 3,Phase 2
21 Cardiac Glycosides Phase 4,Phase 3,Phase 2,Phase 1
22 Anti-Infective Agents Phase 4,Phase 3,Phase 1,Phase 2
23 Antiviral Agents Phase 4,Phase 3,Phase 1,Phase 2
24 Anti-HIV Agents Phase 4,Phase 3,Phase 2,Phase 1
25 Anti-Retroviral Agents Phase 4,Phase 3,Phase 2,Phase 1
26 Hypoglycemic Agents Phase 4,Phase 3,Phase 2,Phase 1
27 Glycoside Hydrolase Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
28 Anti-Inflammatory Agents Phase 4,Phase 1
29 Pharmaceutical Solutions Phase 4,Phase 2,Phase 3,Phase 1
30 Analgesics, Non-Narcotic Phase 4,Phase 1
31 Analgesics Phase 4,Phase 1
32 Calamus Phase 4
33 cadexomer iodine Phase 4
34
Serotonin Investigational, Nutraceutical Phase 4,Phase 3 50-67-9 5202
35
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
36
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
37
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
38
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
39
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
40
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
41
tannic acid Approved Phase 3 1401-55-4
42
Methylene blue Approved, Investigational Phase 3 61-73-4
43
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
44
Deferiprone Approved Phase 2, Phase 3 30652-11-0 2972
45
Iron Approved, Experimental Phase 2, Phase 3 7439-89-6, 15438-31-0 27284 23925
46
Pimavanserin Approved, Investigational Phase 3 706779-91-1 16058810
47
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7
48 Prednisolone acetate Phase 2, Phase 3
49 Antineoplastic Agents, Alkylating Phase 2, Phase 3
50 Methylprednisolone Acetate Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 188)
# Name Status NCT ID Phase Drugs
1 Serotonergic Function and Behavioural and Psychological Symptoms of Frontotemporal Dementia Completed NCT00376051 Phase 4 Citalopram
2 Memantine (10mg BID) for the Frontal and Temporal Subtypes of Frontotemporal Dementia Completed NCT00545974 Phase 4 memantine;Placebo pill
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 Safety and Efficacy of Miglustat in Chinese NPC Patients Not yet recruiting NCT03910621 Phase 4 Miglustat
7 Amantadine for the Treatment of Behavioral Disturbance in Frontotemporal Dementia (FTD) Withdrawn NCT00127114 Phase 4 Amantadine;Placebo
8 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3 Miglustat
9 Open Label Pilot Study of the Effects of Memantine on FDG-PET in Frontotemporal Dementia Completed NCT00594737 Phase 3 memantine hydrochloride
10 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3 Busulfan, Cyclophosphamide, Antithymocyte Globulin
11 Safety and Efficacy Study Evaluating TRx0237 in Subjects With Behavioral Variant Frontotemporal Dementia (bvFTD) Completed NCT01626378 Phase 3 TRx0237;Placebo
12 Safety, Tolerability, Efficacy, Pharmacokinetics, and Pharmacodynamics of Sebelipase Alfa in Children With Growth Failure Due to Lysosomal Acid Lipase Deficiency Completed NCT01371825 Phase 2, Phase 3 Sebelipase alfa (SBC-102)
13 Effects of Miglustat Therapy on Infantile Type of Sandhoff and Taysachs Diseases (EMTISTD) Recruiting NCT03822013 Phase 3 Miglustat
14 The Healthy Patterns Study Recruiting NCT03682185 Phase 3
15 Conservative Iron Chelation as a Disease-modifying Strategy in Amyotrophic Lateral Sclerosis Recruiting NCT03293069 Phase 2, Phase 3 Deferiprone;Placebo Oral Tablet
16 Relapse Prevention Study of Pimavanserin in Dementia-related Psychosis Recruiting NCT03325556 Phase 3 Placebo;Pimavanserin 34 mg;Pimavanserin 20 mg
17 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3 arimoclomol;Placebo
18 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
19 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Active, not recruiting NCT02004691 Phase 2, Phase 3 placebo (saline);GZ402665
20 Open-label Study of VTS-270 in Participants With Neurologic Manifestations of Niemann-Pick Type C1 Not yet recruiting NCT03879655 Phase 2, Phase 3 VTS-270
21 ALD-101 Adjuvant Therapy of Unrelated Umbilical Cord Blood Transfusion (UCBT) in Patients With Inherited Metabolic Diseases Terminated NCT00654433 Phase 3
22 Children With Lysosomal Acid Lipase Deficiency Who Previously Received Treatment With SBC-102 Terminated NCT01473875 Phase 2, Phase 3 SBC-102
23 Open-Label Study of TRx0237 in Subjects With Alzheimer's Disease or Behavioral Variant Frontotemporal Dementia (bvFTD) Terminated NCT02245568 Phase 3 TRx0237
24 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
25 Effects of Tolcapone on Frontotemporal Dementia Unknown status NCT00604591 Phase 2 Tolcapone;Placebo
26 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2 Vorinostat
27 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2 N-Acetyl Cysteine
28 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
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 Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia Completed NCT01937013 Phase 2 Intranasal oxytocin;Saline Nasal Mist
32 Double Blind Trial of DC Polarization in FTD Completed NCT00117858 Phase 2
33 Efficacy and Tolerability of Memantine in Frontotemporal Dementia (FTD) Patients Completed NCT00200538 Phase 2 memantine
34 Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations Completed NCT02676843 Phase 2 18F-AV-1451
35 Imaging Characteristics of Florbetapir 18F in Patients With Frontotemporal Dementia, Alzheimer's Disease and Normal Controls. Completed NCT01890343 Phase 2 florbetapir 18F;18F-FDG
36 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
37 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
38 Safety and Efficacy of Intravenous Trappsol Cyclo (HPBCD) in Niemann-Pick Type C Patients Recruiting NCT02912793 Phase 1, Phase 2 Hydroxypropyl-beta-cyclodextrin
39 Study of Lithium Carbonate to Treat Niemann-Pick Type C1 Disease Recruiting NCT03201627 Phase 1, Phase 2 Lithium Carbonate
40 Intranasal Oxytocin for Frontotemporal Dementia Recruiting NCT03260920 Phase 2 Syntocinon
41 Low-Dose Lithium for the Treatment of Behavioral Symptoms in Frontotemporal Dementia Recruiting NCT02862210 Phase 2 Lithium Carbonate;Placebo
42 Phase I/II Pilot Study of Mixed Chimerism to Treat Inherited Metabolic Disorders Active, not recruiting NCT01372228 Phase 1, Phase 2
43 Safety, Tolerability, PK, and Efficacy Evaluation of Repeat Ascending Doses of Olipudase Alfa in Pediatric Patients <18 Years of Age With Acid Sphingomyelinase Deficiency Active, not recruiting NCT02292654 Phase 1, Phase 2 Olipudase alfa
44 Clinical Trial in Infants With Rapidly Progressive Lysosomal Acid Lipase Deficiency Active, not recruiting NCT02193867 Phase 2 sebelipase alfa
45 F 18 T807 Tau PET Imaging in Familial Amyotrophic Lateral Sclerosis Active, not recruiting NCT02414230 Phase 2 Drug: F 18 T807
46 A Long-Term Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Enrolling by invitation NCT02004704 Phase 2 GZ402665
47 F 18 T807 Tau PET Imaging of Progressive Posterior Cortical Dysfunction (IND 123119, Protocol E) Enrolling by invitation NCT02414282 Phase 2 F 18 T807
48 N-Acetyl-L-Leucine for Niemann-Pick Disease, Type C (NPC) Not yet recruiting NCT03759639 Phase 2 IB1001
49 Safety and Tolerability Study of VTS-270 in Pediatric Participants With Niemann-Pick Type C (NPC) Disease Not yet recruiting NCT03687476 Phase 2 VTS-270
50 Hematopoietic Stem Cell Transplantation (HCT) for Inborn Errors of Metabolism Terminated NCT00668564 Phase 2 Cyclophosphamide;Campath-1H;Busulfan

Search NIH Clinical Center for Behavioral Variant of Frontotemporal Dementia

Genetic Tests for Behavioral Variant of Frontotemporal Dementia

Anatomical Context for Behavioral Variant of Frontotemporal Dementia

MalaCards organs/tissues related to Behavioral Variant of Frontotemporal Dementia:

42
Brain, Liver, Testes, Skin, Bone, Eye, Cortex

Publications for Behavioral Variant of Frontotemporal Dementia

Articles related to Behavioral Variant of Frontotemporal Dementia:

(show all 37)
# Title Authors Year
1
Bifrontal Hypermetabolism on Brain FDG-PET in a Case of C9orf72-Related Behavioral Variant of Frontotemporal Dementia. ( 30404533 )
2019
2
Moral judgment in patients with behavioral variant of frontotemporal dementia and amyotrophic lateral sclerosis: no impairment of the moral position, but rather its execution. ( 30513214 )
2019
3
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
4
18F-THK5351 PET Imaging in the Behavioral Variant of Frontotemporal Dementia. ( 30906406 )
2018
5
An autopsy case of dementia with Lewy bodies clinically diagnosed to have a behavioral variant of frontotemporal dementia. ( 27737532 )
2017
6
Mistakes, Too Few to Mention? Impaired Self-conscious Emotional Processing of Errors in the Behavioral Variant of Frontotemporal Dementia. ( 29089874 )
2017
7
Evaluating Behavior in Mouse Models of the Behavioral Variant of Frontotemporal Dementia: Which Test for Which Symptom? ( 26517704 )
2016
8
Differential Impairment of Cognitive and Affective Mentalizing Abilities in Neurodegenerative Dementias: Evidence from Behavioral Variant of Frontotemporal Dementia, Alzheimer's Disease, and Mild Cognitive Impairment. ( 26836153 )
2016
9
Behavioral variant of frontotemporal dementia: Fundamental clinical issues associated with prediction of pathological bases. ( 26969837 )
2016
10
The behavioral variant of frontotemporal dementia: An analysis of the literature and a case report. ( 27143117 )
2016
11
Different functional connectivity and network topology in behavioral variant of frontotemporal dementia and Alzheimer's disease: an EEG study. ( 27143432 )
2016
12
Uncovering the Neural Bases of Cognitive and Affective Empathy Deficits in Alzheimer's Disease and the Behavioral-Variant of Frontotemporal Dementia. ( 27258418 )
2016
13
The Peripheral Sympathetic Neuron is Intact in Alzheimer's Disease and Behavioral Variant of Frontotemporal Dementia. ( 25280792 )
2015
14
Drawing perseveration in the behavioral variant of frontotemporal dementia. ( 25548236 )
2015
15
Impaired recognition of body expressions in the behavioral variant of frontotemporal dementia. ( 26162615 )
2015
16
Right Limbic FDG-PET Hypometabolism Correlates with Emotion Recognition and Attribution in Probable Behavioral Variant of Frontotemporal Dementia Patients. ( 26513651 )
2015
17
Affective mentalizing and brain activity at rest in the behavioral variant of frontotemporal dementia. ( 26594631 )
2015
18
DAPHNE: A New Tool for the Assessment of the Behavioral Variant of Frontotemporal Dementia. ( 26955383 )
2015
19
Metacognition in the behavioral variant of frontotemporal dementia and Alzheimer's disease. ( 24548124 )
2014
20
The added value of 18-fluorodeoxyglucose-positron emission tomography in the diagnosis of the behavioral variant of frontotemporal dementia. ( 24576796 )
2014
21
Neural correlates of empathic impairment in the behavioral variant of frontotemporal dementia. ( 24589435 )
2014
22
The phoenix from the ashes: cardiovascular autonomic dysfunction in behavioral variant of frontotemporal dementia. ( 25024313 )
2014
23
Structural neuroimaging of social cognition in progressive non-fluent aphasia and behavioral variant of frontotemporal dementia. ( 23966929 )
2013
24
Functional network connectivity in the behavioral variant of frontotemporal dementia. ( 23164495 )
2013
25
The behavioral variant of frontotemporal dementia: linking neuropathology to social cognition. ( 23377232 )
2013
26
Comparison of 2 diagnostic criteria for the behavioral variant of frontotemporal dementia. ( 23695224 )
2013
27
GroupICA dual regression analysis of resting state networks in a behavioral variant of frontotemporal dementia. ( 23986673 )
2013
28
The experience of caregiving: differences between behavioral variant of frontotemporal dementia and Alzheimer disease. ( 21941168 )
2012
29
Frontal and posterior cingulate metabolic impairment in the behavioral variant of frontotemporal dementia with impaired autonoetic consciousness. ( 21520350 )
2012
30
Contextual social cognition and the behavioral variant of frontotemporal dementia. ( 22529204 )
2012
31
Different apathy profile in behavioral variant of frontotemporal dementia and Alzheimer's disease: a preliminary investigation. ( 22719755 )
2012
32
Definite behavioral variant of frontotemporal dementia with C9ORF72 expansions despite positive Alzheimer's disease cerebrospinal fluid biomarkers. ( 22766732 )
2012
33
Prospective evaluation of behavioral scales in the behavioral variant of frontotemporal dementia. ( 22922703 )
2012
34
Cognitive decline in the behavioral variant of frontotemporal dementia. ( 20836915 )
2011
35
Behavioral variant of frontotemporal dementia mimicking Huntington's disease. ( 20170589 )
2010
36
Zarit burden inventory and activities of daily living in the behavioral variant of frontotemporal dementia. ( 18285675 )
2008
37
Diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD): current limitations and future directions. ( 18090417 )
2007

Variations for Behavioral Variant of Frontotemporal Dementia

Expression for Behavioral Variant of Frontotemporal Dementia

Search GEO for disease gene expression data for Behavioral Variant of Frontotemporal Dementia.

Pathways for Behavioral Variant of Frontotemporal Dementia

Pathways related to Behavioral Variant of Frontotemporal Dementia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.97 MAPT PSEN1 TREM2
2 11.62 CHMP2B MAPT VCP
3
Show member pathways
11.14 PSEN1 SQSTM1
4 10.9 PSEN1 SQSTM1
5 10.28 MAPT PSEN1

GO Terms for Behavioral Variant of Frontotemporal Dementia

Cellular components related to Behavioral Variant of Frontotemporal Dementia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 axon GO:0030424 9.61 C9orf72 MAPT PSEN1
2 growth cone GO:0030426 9.43 C9orf72 MAPT PSEN1
3 autophagosome GO:0005776 9.4 C9orf72 SQSTM1
4 cytoplasmic stress granule GO:0010494 9.37 C9orf72 VCP
5 endosome GO:0005768 9.35 C9orf72 CHMP2B GRN SQSTM1 TMEM106B
6 aggresome GO:0016235 9.32 PSEN1 SQSTM1
7 main axon GO:0044304 9.16 C9orf72 MAPT
8 lysosome GO:0005764 9.02 C9orf72 CHMP2B GRN SQSTM1 TMEM106B

Biological processes related to Behavioral Variant of Frontotemporal Dementia according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of proteasomal ubiquitin-dependent protein catabolic process GO:0032436 9.49 PSEN1 VCP
2 cellular response to heat GO:0034605 9.48 MAPT VCP
3 regulation of autophagy GO:0010506 9.46 C9orf72 MAPT
4 positive regulation of protein localization to plasma membrane GO:1903078 9.43 SQSTM1 TREM2
5 regulation of synaptic plasticity GO:0048167 9.4 MAPT PSEN1
6 synapse organization GO:0050808 9.37 MAPT PSEN1
7 endosome organization GO:0007032 9.32 CHMP2B SQSTM1
8 stress granule assembly GO:0034063 9.26 C9orf72 MAPT
9 astrocyte activation GO:0048143 9.16 MAPT PSEN1
10 macroautophagy GO:0016236 9.13 CHMP2B SQSTM1 VCP
11 autophagy GO:0006914 9.02 C9orf72 CHMP2B PSEN1 SQSTM1 VCP

Sources for Behavioral Variant of Frontotemporal Dementia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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