MCID: SMN008
MIFTS: 41

Semantic Dementia malady

Neuronal category

Summaries for Semantic Dementia

Sources:
44NINDS, 64Wikipedia, 33MalaCards
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NINDS:44 Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick?s disease, the name and classification of FTD has been a topic of discussion for over a century.

MalaCards: Semantic Dementia, also known as semantic primary progressive aphasia, is related to alzheimer's disease and frontotemporal dementia. An important gene associated with Semantic Dementia is GRN (granulin), and among its related pathways are Endosomal Sorting Complex Required For Transport (ESCRT) and Alzheimers Disease Pathway. The compounds tacrine and donepezil have been mentioned in the context of this disorder. Affiliated tissues include brain, cortex and temporal lobe, and related mouse phenotypes are other and cellular.

Wikipedia:64 Semantic dementia (SD) is a progressive neurodegenerative disorder characterized by loss of semantic... more...

Aliases & Classifications for Semantic Dementia

Sources:
61UMLS, 43NIH Rare Diseases, 44NINDS, 45Novoseek, 49Orphanet, 26ICD10 via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal


Characteristics (Orphanet epidemiological data):

49
semantic dementia:
Inheritance: Multigenic/multifactorial,Sporadic; Age of onset: Adulthood


Aliases & Descriptions:

semantic dementia 43 44 45 49 61
semantic primary progressive aphasia 43 49
semantic variant ppa 43 49
primary progressive aphasia 61
dementia - semantic 44


External Ids:

ICD10 via Orphanet26 G31.0

Related Diseases for Semantic Dementia

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Semantic Dementia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 58)
idRelated DiseaseScoreTop Affiliating Genes
1alzheimer's disease31.3RPS27A, APOE, PSEN1, GRN, MAPT, TARDBP
2frontotemporal dementia31.2MAPT, PSEN1
3aphasia31.1C9orf72, GRN, MAPT, TARDBP
4primary progressive aphasia30.7RPS27A, GRN, MAPT
5memory impairment30.7MAPT, GRN, PSEN1, APOE
6frontotemporal lobar degeneration with ubiquitin-positive inclusions30.5GRN
7progressive supranuclear palsy30.5RPS27A, APOE, MAPT
8apraxia30.1MAPT, GRN
9pick's disease30.1MAPT, PSEN1
10tauopathy30.1RPS27A, PSEN1, GRN, MAPT
11logopenic progressive aphasia10.8
12dyslexia10.6
13corticobasal degeneration10.4
14alexia10.3
15progressive non-fluent aphasia10.3
16transient global amnesia10.2
17agnosia10.2
18prosopagnosia10.1
19dysgraphia10.1
20frontotemporal dementia, ubiquitin-positive10.1
21herpes simplex encephalitis10.1
22hyperacusis10.1
23tau syndrome10.1
24n syndrome10.1
25ideomotor apraxia10.1
26foix chavany marie syndrome10.1
27variant creutzfeldt-jakob disease10.1
28cerebral atrophy10.1
29lateral sclerosis10.0TARDBP
30vascular dementia10.0APOE
31neuronal intranuclear inclusion disease10.0RPS27A
32myotonic dystrophy10.0MAPT
33pancreatic cancer10.0RPS27A
34scrapie10.0MAPT
35inclusion body myositis10.0MAPT, RPS27A
36spinocerebellar ataxia type 310.0RPS27A, VCP
37myopathy10.0RPS27A, VCP
38multiple system atrophy10.0MAPT, RPS27A
39spinocerebellar ataxia10.0RPS27A, MAPT
40schizophrenia10.0PSEN1
41ischemia10.0PSEN1, MAPT
42movement disease10.0MAPT, RPS27A
43prion disease10.0MAPT, PSEN1
44myositis10.0MAPT, PSEN1, RPS27A
45lewy body dementia10.0MAPT, APOE, RPS27A
46creutzfeldt-jakob syndrome10.0MAPT, APOE, RPS27A
47hemorrhage, intracerebral10.0RPS27A, APOE, PSEN1
48huntington's disease10.0MAPT, PSEN1, RPS27A
49cerebral amyloid angiopathy10.0APOE, PSEN1, MAPT
50alzheimer disease type 210.0MAPT, PSEN1, APOE

Graphical network of the top 20 diseases related to Semantic Dementia:



Diseases related to semantic dementia

Clinical Features for Semantic Dementia

Drugs & Therapeutics for Semantic Dementia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Semantic Dementia

Drug clinical trials:

Search ClinicalTrials for Semantic Dementia

Search NIH Clinical Center for Semantic Dementia

Search CenterWatch for Semantic Dementia

Genetic Tests for Semantic Dementia

Anatomical Context for Semantic Dementia

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Semantic Dementia:

33
Brain, Cortex, Temporal lobe

Animal Models for Semantic Dementia or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Semantic Dementia:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053958.8APOE, PSEN1, MAPT
2MP:00053847.6VCP, TARDBP, MAPT, GRN, PSEN1, APOE
3MP:00053867.5VCP, TARDBP, MAPT, GRN, PSEN1, APOE

Publications for Semantic Dementia

Sources:
51PubMed
See all sources

Articles related to Semantic Dementia:

(show top 50)    (show all 291)
idTitleAuthorsYear
1
Semantic Dementia Diagnosed by F-18 FDG PET/MRI: Co-registered Images. (24083072)
2013
2
Fluorodeoxyglucose positron emission tomography in semantic dementia after 6a88months of memantine: an open-label pilot study. (22674572)
2013
3
The degraded concept representation system in semantic dementia: damage to pan-modal hub, then visual spoke. (23250888)
2012
4
Semantic dementia versus nonfluent progressive aphasia: neuropsychological characterization and differentiation. (21552095)
2012
5
Semantic dementia associated with corticobasal syndrome: a further variant of frontotemporal lobe degeneration? (22231872)
2012
6
Verb production during action naming in semantic dementia. (21237467)
2011
7
Magnetoencephalography of frontotemporal dementia: spatiotemporally localized changes during semantic decisions. (21840892)
2011
8
Semantic dementia: a specific network-opathy. (21710360)
2011
9
Motor knowledge is one dimension for concept organization: further evidence from a Chinese semantic dementia case. (20701959)
2011
10
Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. (19805492)
2010
11
Flavour processing in semantic dementia. (19656505)
2010
12
Hanja (Ideogram) alexia and agraphia in patients with semantic dementia. (19967597)
2010
13
The Cambridge Semantic Memory Test Battery: detection of semantic deficits in semantic dementia and Alzheimer's disease. (20408046)
2010
14
The ventral and inferolateral aspects of the anterior temporal lobe are crucial in semantic memory: evidence from a novel direct comparison of distortion-corrected fMRI, rTMS, and semantic dementia. (20190005)
2010
15
Alzheimer's disease neuropathologic changes in semantic dementia. (19866390)
2010
16
Lost and found: bespoke memory testing for Alzheimer's disease and semantic dementia. (21504124)
2010
17
Semantic dementia associated with mutation V363I in the tau gene. (20598713)
2010
18
Errorless learning of computer-generated words in a patient with semantic dementia. (19504403)
2010
19
'The quicksand of forgetfulness': semantic dementia in One hundred years of solitude. (19447824)
2009
20
Emergence and progression of 'non-semantic' deficits in semantic dementia. (19231477)
2009
21
Patterns of autobiographical memory impairment according to disease severity in semantic dementia. (19231476)
2009
22
The relation between content and structure in language production: an analysis of speech errors in semantic dementia. (19477502)
2009
23
The neural basis of surface dyslexia in semantic dementia. (19022856)
2009
24
Semantic dementia combined with motor neuron disease. (19800241)
2009
25
Memory complaints in mild cognitive impairment, worried well, and semantic dementia patients. (18580592)
2008
26
Differentiation of semantic dementia and Alzheimer's disease using the Addenbrooke's Cognitive Examination (ACE). (17768772)
2008
27
'Non-semantic' aspects of language in semantic dementia: as normal as they're said to be? (16517512)
2006
28
How Berlusconi keeps his face: a neuropsychological study in a case of semantic dementia. (16771038)
2006
29
Frontotemporal lobar degeneration with ubiquitin pathology: an autopsy case presenting with semantic dementia and upper motor neuron signs with a clinical course of 19 years. (17016698)
2006
30
Treating anomia in semantic dementia: improvement, maintenance, or both? (16835150)
2006
31
The role of semantic distance in category-specific impairments for living things: evidence from a case of semantic dementia. (16352319)
2006
32
The natural history of late-stage "pure" semantic dementia. (16517511)
2006
33
Failing to get the gist: reduced false recognition of semantic associates in semantic dementia. (15910121)
2005
34
Sentence comprehension in semantic dementia: a longitudinal case study. (21038208)
2004
35
Neuropsychological and functional measures of severity in Alzheimer disease, frontotemporal dementia, and semantic dementia. (15592131)
2004
36
Simulating episodic memory deficits in semantic dementia with the TraceLink model. (15279432)
2004
37
A category-specific advantage for numbers in verbal short-term memory: evidence from semantic dementia. (14725802)
2004
38
Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and Alzheimer disease. (14665820)
2003
39
Lost for words or loss of memories? Autobiographical memory in semantic dementia. (20957590)
2003
40
Why semantic dementia drives you to the dogs (but not to the horses): A theoretical account. (20957550)
2002
41
Preserved calculation skills in a case of semantic dementia. (12146663)
2002
42
Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. (11468305)
2001
43
Patterns of temporal lobe atrophy in semantic dementia and Alzheimer's disease. (11310620)
2001
44
Semantic dementia: clinical, radiological and pathological perspectives. (10929269)
2000
45
Tau protein in cerebrospinal fluid from semantic dementia patients. (11072138)
2000
46
Semantic dementia: a challenge to the multiple-trace theory? (10322458)
1999
47
The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: a comparative neuropsychological study. (10067773)
1999
48
Interaction of cortex and hippocampus in a model of amnesia and semantic dementia. (10526891)
1999
49
Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimer's disease. (9055272)
1997
50
Response biases in oral reading: an account of the co-occurrence of surface dyslexia and semantic dementia. (8685392)
1996

Genetic Variations for Semantic Dementia

Expression for genes affiliated with Semantic Dementia

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Semantic Dementia

Search GEO for disease gene expression data for Semantic Dementia.

Pathways for genes affiliated with Semantic Dementia

Sources:
54Reactome, 52QIAGEN, 4Cell Signaling Technology
See all sources

Pathways related to Semantic Dementia according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
9.4CHMP2B, RPS27A
29.1MAPT, PSEN1, APOE
38.3TARDBP, MAPT, PSEN1, APOE

Compounds for genes affiliated with Semantic Dementia

Sources:
45Novoseek, 11DrugBank, 24HMDB, 50PharmGKB, 29IUPHAR, 2BitterDB
See all sources

Compounds related to Semantic Dementia according to GeneCards/GeneDecks:

(show all 36)
idCompoundScoreTop Affiliating Genes
1tacrine45 1111.0MAPT, APOE
2donepezil45 11 2411.9MAPT, APOE
3galantamine45 50 1111.9MAPT, APOE
4fluorochrome459.8MAPT, RPS27A
5guanidine45 11 2411.7MAPT, APOE
6cysteamine45 11 2411.7APOE, RPS27A
7spec-t459.5MAPT, PSEN1, APOE
8vitamin b12459.5APOE, PSEN1, MAPT
9kainate45 2910.5MAPT, PSEN1, APOE
10thioflavine s459.4MAPT, APOE, RPS27A
11folate459.4MAPT, PSEN1, APOE
12formate459.4RPS27A, APOE, MAPT
13chloroquine45 2 50 29 1113.4MAPT, APOE, RPS27A
14sodium dodecylsulfate459.4RPS27A, APOE, MAPT
15glycerol45 11 2411.3RPS27A, PSEN1, MAPT
16n acetylcysteine459.2RPS27A, APOE, MAPT
17biotin45 11 2411.2PSEN1, APOE, RPS27A
18tamoxifen45 50 29 1112.1RPS27A, APOE, GRN
19streptozotocin459.0APOE, RPS27A
20acetylcholine45 50 29 11 2413.0MAPT, APOE, RPS27A
21glycogen45 2410.0MAPT, PSEN1, RPS27A
224-hydroxynonenal45 249.9RPS27A, APOE, PSEN1, MAPT
23lactacystin458.9MAPT, PSEN1, APOE, RPS27A
24valine458.9MAPT, PSEN1, APOE, RPS27A
25nmda45 299.9MAPT, PSEN1, APOE, RPS27A
26threonine458.8VCP, MAPT, PSEN1, RPS27A
27aspartate458.8RPS27A, APOE, PSEN1, MAPT
28cycloheximide458.8RPS27A, APOE, PSEN1, MAPT
29h2o2458.8RPS27A, APOE, PSEN1, MAPT
30arginine458.7RPS27A, APOE, PSEN1, MAPT
31glutamate458.6RPS27A, APOE, PSEN1, MAPT
32alanine458.6MAPT, PSEN1, APOE, RPS27A
33estrogen458.6RPS27A, APOE, GRN, MAPT
34cysteine458.5RPS27A, APOE, PSEN1, GRN, MAPT
35glutamine458.5RPS27A, APOE, PSEN1, MAPT, VCP
36serine458.3VCP, MAPT, PSEN1, APOE, RPS27A

GO Terms for genes affiliated with Semantic Dementia

Sources:
16Gene Ontology
See all sources

Biological processes related to Semantic Dementia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of proteasomal ubiquitin-dependent protein catabolic processGO:0324369.6VCP, PSEN1
2positive regulation of axon extensionGO:0457739.5APOE, MAPT
3Notch receptor processingGO:0072209.0PSEN1, RPS27A
4cell deathGO:0082197.9TARDBP, GRN, CHMP2B, C9orf72, APOE

Molecular functions related to Semantic Dementia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lipoprotein particle bindingGO:0718139.6MAPT, APOE

Products for genes affiliated with Semantic Dementia

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Semantic Dementia

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet