MCID: BHV002
MIFTS: 42

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 59
Bv-Ftd 59

Characteristics:

Orphanet epidemiological data:

59
behavioral variant of frontotemporal dementia
Inheritance: Autosomal dominant; Age of onset: Adult; Age of death: elderly;

Classifications:

Orphanet: 59  
Rare neurological diseases


Summaries for Behavioral Variant of Frontotemporal Dementia

MalaCards based summary : Behavioral Variant of Frontotemporal Dementia, also known as bv-ftd, is related to alzheimer disease and lateral sclerosis. 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. Affiliated tissues include brain, skin and testes, and related phenotypes are gait disturbance and hyperreflexia

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 all 47)
# Related Disease Score Top Affiliating Genes
1 alzheimer disease 30.5 GRN MAPT PSEN1 SQSTM1
2 lateral sclerosis 30.1 C9orf72 SQSTM1 VCP
3 frontotemporal dementia 29.5 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1
4 dementia 29.5 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1
5 aphasia 29.4 C9orf72 CHMP2B GRN MAPT PSEN1
6 progressive non-fluent aphasia 28.5 C9orf72 CHMP2B GRN MAPT PSEN1 TMEM106B
7 amyotrophic lateral sclerosis 1 28.3 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1
8 frontotemporal dementia with parkinsonism-17 10.8
9 cognitive function 1, social 10.3
10 depression 10.2
11 phonagnosia 10.1 GRN PSEN1
12 associative agnosia 10.1 GRN PSEN1
13 writing disorder 10.1 GRN PSEN1
14 prosopagnosia 10.1 GRN PSEN1
15 gait apraxia 10.1 GRN PSEN1
16 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 10.1 GRN MAPT
17 ideomotor apraxia 10.1 GRN MAPT
18 familial idiopathic basal ganglia calcification 10.1 MAPT PSEN1
19 kohlschutter-tonz syndrome 10.1 MAPT PSEN1
20 visual agnosia 10.1 GRN PSEN1
21 kluver-bucy syndrome 10.1 MAPT PSEN1
22 perry syndrome 10.1 C9orf72 GRN
23 amyotrophic lateral sclerosis 21 10.1 C9orf72 VCP
24 intermittent claudication 10.1
25 patulous eustachian tube 10.1
26 alzheimer disease 2 10.0 MAPT PSEN1
27 early-onset, autosomal dominant alzheimer disease 10.0 PSEN1 TREM2
28 mammary paget's disease 10.0 SQSTM1 VCP
29 multiple system atrophy 1 10.0 MAPT SQSTM1
30 nervous system disease 10.0 C9orf72 MAPT PSEN1
31 cerebral amyloid angiopathy, cst3-related 10.0 MAPT PSEN1
32 echolalia 10.0 GRN MAPT PSEN1
33 agraphia 10.0 GRN MAPT PSEN1
34 dementia, lewy body 10.0 GRN MAPT PSEN1
35 vascular dementia 9.9 MAPT PSEN1
36 mutism 9.9 CHMP2B GRN
37 inclusion body myositis 9.9 MAPT SQSTM1 VCP
38 supranuclear palsy, progressive, 1 9.9 C9orf72 GRN MAPT PSEN1
39 paget's disease of bone 9.9 SQSTM1 VCP
40 apraxia 9.9 C9orf72 GRN MAPT PSEN1
41 speech and communication disorders 9.9 C9orf72 GRN MAPT PSEN1
42 disease of mental health 9.9 C9orf72 GRN MAPT PSEN1
43 pick disease of brain 9.9 GRN MAPT PSEN1 SQSTM1
44 motor neuron disease 9.7 C9orf72 GRN MAPT SQSTM1 VCP
45 nominal aphasia 9.7 CHMP2B GRN MAPT PSEN1
46 semantic dementia 9.1 C9orf72 CHMP2B GRN MAPT PSEN1 TMEM106B
47 frontotemporal dementia and/or amyotrophic lateral sclerosis 1 9.0 C9orf72 CHMP2B GRN MAPT PSEN1 SQSTM1

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:

59 32 (show all 40)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 gait disturbance 59 32 occasional (7.5%) Occasional (29-5%) HP:0001288
2 hyperreflexia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001347
3 dysphasia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002357
4 stereotypy 59 32 hallmark (90%) Very frequent (99-80%) HP:0000733
5 irritability 59 32 hallmark (90%) Very frequent (99-80%) HP:0000737
6 thickened nuchal skin fold 59 32 hallmark (90%) Very frequent (99-80%) HP:0000474
7 generalized tonic-clonic seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0002069
8 memory impairment 59 32 hallmark (90%) Very frequent (99-80%) HP:0002354
9 psychosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000709
10 aggressive behavior 59 32 hallmark (90%) Very frequent (99-80%) HP:0000718
11 fasciculations 59 32 occasional (7.5%) Occasional (29-5%) HP:0002380
12 dyslexia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010522
13 dysgraphia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010526
14 abnormality of extrapyramidal motor function 59 32 occasional (7.5%) Occasional (29-5%) HP:0002071
15 astrocytosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0002446
16 frontotemporal cerebral atrophy 59 32 hallmark (90%) Very frequent (99-80%) HP:0006892
17 restlessness 59 32 hallmark (90%) Very frequent (99-80%) HP:0000711
18 disinhibition 59 32 hallmark (90%) Very frequent (99-80%) HP:0000734
19 apathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0000741
20 personality changes 59 32 hallmark (90%) Very frequent (99-80%) HP:0000751
21 frontotemporal dementia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002145
22 abnormal brain fdg positron emission tomography 59 32 frequent (33%) Frequent (79-30%) HP:0012658
23 inappropriate behavior 59 32 hallmark (90%) Very frequent (99-80%) HP:0000719
24 echolalia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010529
25 loss of speech 59 32 hallmark (90%) Very frequent (99-80%) HP:0002371
26 dyscalculia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002442
27 poor speech 59 32 hallmark (90%) Very frequent (99-80%) HP:0002465
28 mutism 59 32 occasional (7.5%) Occasional (29-5%) HP:0002300
29 abnormality of the cerebral white matter 59 32 frequent (33%) Frequent (79-30%) HP:0002500
30 perseveration 59 32 hallmark (90%) Very frequent (99-80%) HP:0030223
31 hyperorality 59 32 hallmark (90%) Very frequent (99-80%) HP:0000710
32 restrictive behavior 59 32 hallmark (90%) Very frequent (99-80%) HP:0000723
33 lack of insight 59 32 hallmark (90%) Very frequent (99-80%) HP:0000757
34 upper motor neuron dysfunction 59 32 occasional (7.5%) Occasional (29-5%) HP:0002493
35 eeg with continuous slow activity 59 32 frequent (33%) Frequent (79-30%) HP:0011204
36 collectionism 59 32 frequent (33%) Frequent (79-30%) HP:0030212
37 emotional blunting 59 32 hallmark (90%) Very frequent (99-80%) HP:0030213
38 abulia 59 32 occasional (7.5%) Occasional (29-5%) HP:0012671
39 behavioral abnormality 59 Very frequent (99-80%)
40 mental deterioration 59 Very frequent (99-80%)

MGI Mouse Phenotypes related to Behavioral Variant of Frontotemporal Dementia:

46
# 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

Search Clinical Trials , 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:

41
Brain, Skin, Testes, Bone

Publications for Behavioral Variant of Frontotemporal Dementia

Articles related to Behavioral Variant of Frontotemporal Dementia:

(show all 38)
# Title Authors Year
1
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
2
Bifrontal Hypermetabolism on Brain FDG-PET in a Case of C9orf72-Related Behavioral Variant of Frontotemporal Dementia. ( 30404533 )
2018
3
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 )
2018
4
An autopsy case of dementia with Lewy bodies clinically diagnosed to have a behavioral variant of frontotemporal dementia. ( 27737532 )
2017
5
Mistakes, Too Few to Mention? Impaired Self-conscious Emotional Processing of Errors in the Behavioral Variant of Frontotemporal Dementia. ( 29089874 )
2017
6
Uncovering the Neural Bases of Cognitive and Affective Empathy Deficits inA Alzheimer's Disease and the Behavioral-Variant of Frontotemporal Dementia. ( 27258418 )
2016
7
Different functional connectivity and network topology in behavioral variant of frontotemporal dementia and Alzheimer's disease: an EEG study. ( 27143432 )
2016
8
The behavioral variant of frontotemporal dementia: An analysis of the literature and a case report. ( 27143117 )
2016
9
Behavioral variant of frontotemporal dementia: Fundamental clinical issues associated with prediction of pathological bases. ( 26969837 )
2016
10
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
11
DAPHNE: A New Tool for the Assessment of the Behavioral Variant of Frontotemporal Dementia. ( 26955383 )
2015
12
Impaired recognition of body expressions in the behavioral variant of frontotemporal dementia. ( 26162615 )
2015
13
The Peripheral Sympathetic Neuron is Intact in Alzheimer's Disease and Behavioral Variant of Frontotemporal Dementia. ( 25280792 )
2015
14
Evaluating Behavior in Mouse Models of the Behavioral Variant of Frontotemporal Dementia: Which Test for Which Symptom? ( 26517704 )
2015
15
Right Limbic FDG-PET Hypometabolism Correlates with Emotion Recognition and Attribution in Probable Behavioral Variant of Frontotemporal Dementia Patients. ( 26513651 )
2015
16
Drawing perseveration in the behavioral variant of frontotemporal dementia. ( 25548236 )
2015
17
Affective mentalizing and brain activity at rest in the behavioral variant of frontotemporal dementia. ( 26594631 )
2015
18
Theory of mind impairment in patients with behavioural variant fronto-temporal dementia (bv-FTD) increases caregiver burden. ( 26025914 )
2015
19
The phoenix from the ashes: cardiovascular autonomic dysfunction in behavioral variant of frontotemporal dementia. ( 25024313 )
2014
20
Metacognition in the behavioral variant of frontotemporal dementia and Alzheimer's disease. ( 24548124 )
2014
21
Neural correlates of empathic impairment in the behavioral variant of frontotemporal dementia. ( 24589435 )
2014
22
The added value of 18-fluorodeoxyglucose-positron emission tomography in the diagnosis of the behavioral variant of frontotemporal dementia. ( 24576796 )
2014
23
GroupICA dual regression analysis of resting state networks in a behavioral variant of frontotemporal dementia. ( 23986673 )
2013
24
Comparison of 2 diagnostic criteria for the behavioral variant of frontotemporal dementia. ( 23695224 )
2013
25
The behavioral variant of frontotemporal dementia: linking neuropathology to social cognition. ( 23377232 )
2013
26
Structural neuroimaging of social cognition in progressive non-fluent aphasia and behavioral variant of frontotemporal dementia. ( 23966929 )
2013
27
Functional network connectivity in the behavioral variant of frontotemporal dementia. ( 23164495 )
2013
28
The INECO Frontal Screening tool differentiates behavioral variant - frontotemporal dementia (bv-FTD) from major depression. ( 29213817 )
2013
29
Different apathy profile in behavioral variant of frontotemporal dementia and Alzheimer's disease: a preliminary investigation. ( 22719755 )
2012
30
Frontal and posterior cingulate metabolic impairment in the behavioral variant of frontotemporal dementia with impaired autonoetic consciousness. ( 21520350 )
2012
31
Contextual social cognition and the behavioral variant of frontotemporal dementia. ( 22529204 )
2012
32
Prospective evaluation of behavioral scales in the behavioral variant of frontotemporal dementia. ( 22922703 )
2012
33
Definite behavioral variant of frontotemporal dementia with C9ORF72 expansions despite positive Alzheimer's disease cerebrospinal fluid biomarkers. ( 22766732 )
2012
34
The experience of caregiving: differences between behavioral variant of frontotemporal dementia and Alzheimer disease. ( 21941168 )
2012
35
Cognitive decline in the behavioral variant of frontotemporal dementia. ( 20836915 )
2011
36
Behavioral variant of frontotemporal dementia mimicking Huntington's disease. ( 20170589 )
2010
37
Zarit burden inventory and activities of daily living in the behavioral variant of frontotemporal dementia. ( 18285675 )
2008
38
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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
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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