MCID: SMN008
MIFTS: 43

Semantic Dementia malady

Rare diseases, Neuronal diseases, Mental diseases categories

Aliases & Classifications for Semantic Dementia

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Sources:
42NIH Rare Diseases, 43NINDS, 44Novoseek, 48Orphanet, 61UMLS, 26ICD10 via Orphanet, 62UMLS via Orphanet
See all sources

Aliases & Descriptions for Semantic Dementia:

Name: Semantic Dementia 42 43 44 48 61
Semantic Primary Progressive Aphasia 42 48
 
Semantic Variant Ppa 42 48
Dementia - Semantic 43


Classifications:



Characteristics (Orphanet epidemiological data):

48
semantic dementia:
Inheritance: Multigenic/multifactorial,Not applicable; Age of onset: Adult


External Ids:

Orphanet48 100069
ICD10 via Orphanet26 G31.0
UMLS via Orphanet62 C0338462

Summaries for Semantic Dementia

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NINDS:43 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 based summary: Semantic Dementia, also known as semantic primary progressive aphasia, is related to dementia and aphasia. An important gene associated with Semantic Dementia is GRN (granulin), and among its related pathways are Budding and maturation of HIV virion and Fanconi anemia pathway. The compounds tacrine and donepezil have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and testes, and related mouse phenotypes are other and no phenotypic analysis.

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

Related Diseases for Semantic Dementia

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

(show all 45)
idRelated DiseaseScoreTop Affiliating Genes
1dementia32.2PSEN1, MAPT, APOE
2aphasia31.2GRN, C9orf72, MAPT, TARDBP
3dementia, frontotemporal31.0PSEN1, MAPT
4apraxia30.6MAPT, GRN
5pick disease30.5PSEN1, MAPT
6frontotemporal lobar degeneration with ubiquitin-positive inclusions30.5RPS27A, MAPT, GRN
7tauopathy30.2GRN, MAPT, RPS27A, PSEN1
8motor neuron disease30.0TARDBP, GRN, MAPT, VCP, RPS27A
9alzheimer disease29.5PSEN1, VCP, APOE, TARDBP, RPS27A, GRN
10dyslexia10.6
11neuronitis10.4
12neuronal intranuclear inclusion disease10.4RPS27A
13alexia10.4
14cerebritis10.4
15corticobasal degeneration10.4
16lateral sclerosis10.3TARDBP
17inclusion body myositis10.3RPS27A, MAPT
18dysgraphia10.3
19spinocerebellar ataxia10.2RPS27A, MAPT
20dementia, lewy body10.2MAPT, APOE
21movement disease10.2MAPT, RPS27A
22prion disease10.2PSEN1, MAPT
23supranuclear palsy, progressive10.2RPS27A, MAPT, APOE
24creutzfeldt-jakob disease10.1RPS27A, MAPT, APOE
25prosopagnosia10.1
26agraphia10.1
27encephalitis10.1
28herpes simplex10.1
29hyperacusis10.1
30progressive non-fluent aphasia10.1
31multiple system atrophy10.1MAPT, RPS27A
32parkinson disease, late-onset10.1RPS27A, MAPT, APOE
33myositis10.1RPS27A, MAPT, PSEN1
34huntington disease10.1RPS27A, MAPT, PSEN1
35teratocarcinoma10.1MAPT, PSEN1
36hemorrhage, intracerebral10.1RPS27A, PSEN1, APOE
37alzheimer disease-210.1MAPT, PSEN1, APOE
38cerebral amyloid angiopathy10.1APOE, PSEN1, MAPT
39machado-joseph disease10.0VCP, RPS27A
40down syndrome10.0PSEN1, MAPT, APOE
41brain disease10.0PSEN1, MAPT
42vascular dementia9.9PSEN1, APOE, MAPT, RPS27A
43amyloidosis9.9RPS27A, PSEN1, APOE, MAPT
44stroke, ischemic9.9APOE, PSEN1, MAPT
45amyotrophic lateral sclerosis 19.0TARDBP, GRN, MAPT, VCP, CHMP2B, PSEN1

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



Diseases related to semantic dementia

Symptoms for Semantic Dementia

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Drugs & Therapeutics for Semantic Dementia

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Drug clinical trials:

Search ClinicalTrials for Semantic Dementia

Search NIH Clinical Center for Semantic Dementia

Genetic Tests for Semantic Dementia

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Anatomical Context for Semantic Dementia

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MalaCards organs/tissues related to Semantic Dementia:

31
Brain, Temporal lobe, Testes, Cortex

Animal Models for Semantic Dementia or affiliated genes

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MGI Mouse Phenotypes related to Semantic Dementia:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053959.0MAPT, PSEN1, APOE
2MP:00030128.2APOE, C9orf72, MAPT, GRN, TARDBP
3MP:00053698.1APOE, VCP, PSEN1, MAPT, TARDBP
4MP:00053977.8APOE, VCP, PSEN1, MAPT, GRN, TARDBP
5MP:00053867.7APOE, VCP, PSEN1, MAPT, GRN, TARDBP
6MP:00053847.7APOE, VCP, PSEN1, MAPT, GRN, TARDBP
7MP:00107687.7VCP, PSEN1, MAPT, GRN, TARDBP, APOE
8MP:00036317.6APOE, VCP, PSEN1, MAPT, GRN, TARDBP
9MP:00053877.5TREM2, TARDBP, GRN, MAPT, PSEN1, VCP

Publications for Semantic Dementia

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Articles related to Semantic Dementia:

(show top 50)    (show all 310)
idTitleAuthorsYear
1
Differentiating between right-lateralised semantic dementia and behavioural-variant frontotemporal dementia: an examination of clinical characteristics and emotion processing. (25511791)
2014
2
Cerebellar hyperperfusion in semantic dementia. (23414345)
2014
3
Semantic dementia shows both storage and access disorders of semantic memory. (24825958)
2014
4
Machine learning approaches to diagnosis and laterality effects in semantic dementia discourse. (23876449)
2013
5
Phonology without semantics? Good enough for verbal short-term memory. Evidence from a patient with semantic dementia. (22664140)
2013
6
Ever decreasing circles: Speech production in semantic dementia. (23561299)
2013
7
Late-onset OCD as presenting manifestation of semantic dementia. (21908052)
2012
8
Considering the role of semantic memory in episodic future thinking: evidence from semantic dementia. (22614246)
2012
9
Semantic dementia without surface dyslexia in Spanish: unimpaired reading with impaired semantics. (22713391)
2012
10
Reverse concreteness effects are not a typical feature of semantic dementia: evidence for the hub-and-spoke model of conceptual representation. (21285258)
2011
11
Clinical profiles of late-onset semantic dementia, compared with early-onset semantic dementia and late-onset Alzheimer's disease. (21447109)
2011
12
Verb production during action naming in semantic dementia. (21237467)
2011
13
Anomia as a marker of distinct semantic memory impairments in Alzheimer's disease and semantic dementia. (21443339)
2011
14
Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie). (21029204)
2011
15
Visual search patterns in semantic dementia show paradoxical facilitation of binding processes. (21215762)
2011
16
Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. (19805492)
2010
17
Flavour processing in semantic dementia. (19656505)
2010
18
Personal experience and arithmetic meaning in semantic dementia. (19766666)
2010
19
Language networks in semantic dementia. (19759202)
2010
20
Abnormal discourse in semantic dementia: a data-driven approach. (20614366)
2010
21
Atrophy patterns in Alzheimer's disease and semantic dementia: a comparison of FreeSurfer and manual volumetric measurements. (19874902)
2010
22
'The quicksand of forgetfulness': semantic dementia in One hundred years of solitude. (19447824)
2009
23
Effects of semantic impairment on language processing in semantic dementia. (18348090)
2008
24
What does a patient with semantic dementia remember in verbal short-term memory? Order and sound but not words. (18416485)
2007
25
Conceptual priming in semantic dementia: A window into the cognitive and neural basis of conceptual implicit memory. (21049346)
2006
26
'Non-semantic' aspects of language in semantic dementia: as normal as they're said to be? (16517512)
2006
27
How Berlusconi keeps his face: a neuropsychological study in a case of semantic dementia. (16771038)
2006
28
Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? (17071925)
2006
29
Autobiographical memory in semantic dementia: implication for theories of limbic-neocortical interaction in remote memory. (16765999)
2006
30
Semantic dementia, a many-worded disorder]. (17133330)
2006
31
The understanding of quantifiers in semantic dementia: a single-case study. (16801149)
2006
32
Jargon dyslexia in an individual with semantic dementia: further evidence for task-specificity in phonological output. (16251136)
2005
33
Frontotemporal dementia, semantic dementia, and Alzheimer's disease: the contribution of standard neuropsychological tests to differential diagnosis. (15681627)
2005
34
A semantic contribution to nonword recall? Evidence for intact phonological processes in semantic dementia. (21038246)
2005
35
The role of the temporal lobe semantic system in number knowledge: evidence from late-stage semantic dementia. (15716160)
2005
36
Semantic and syntactic aspects of the mass/count distinction: a case study of semantic dementia. (15780454)
2005
37
The contribution of autobiographical significance to semantic memory: evidence from Alzheimer's disease, semantic dementia, and amnesia. (14615074)
2004
38
Knowledge of famous faces and names in semantic dementia. (14985259)
2004
39
Semantic dementia in multilingual patients. (15377748)
2004
40
A duck with four legs: Investigating the structure of conceptual knowledge using picture drawing in semantic dementia. (20957563)
2003
41
Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. (14610120)
2003
42
Memory consolidation and the hippocampus: further evidence from studies of autobiographical memory in semantic dementia and frontal variant frontotemporal dementia. (11792404)
2002
43
The influence of personal familiarity and context on object use in semantic dementia. (11997491)
2002
44
Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. (11468305)
2001
45
Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. (10648435)
2000
46
Combined semantic dementia and apraxia in a patient with frontotemporal lobar degeneration. (11090722)
2000
47
The rise and fall of frequency and imageability: noun and verb production in semantic dementia. (10872636)
2000
48
What does semantic dementia reveal about the functional role of the perirhinal cortex? (10377536)
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
Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study. (9375155)
1996

Variations for Semantic Dementia

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Expression for genes affiliated with Semantic Dementia

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Search GEO for disease gene expression data for Semantic Dementia.

Pathways for genes affiliated with Semantic Dementia

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Pathways related to Semantic Dementia according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.4RPS27A, CHMP2B
2
Show member pathways
9.4RPS27A, VCP
3
Show member pathways
Alzheimers Disease36
9.3APOE, PSEN1, MAPT
49.3MAPT, PSEN1, APOE
58.4TARDBP, MAPT, PSEN1, APOE

Compounds for genes affiliated with Semantic Dementia

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Compounds related to Semantic Dementia according to GeneCards Suite gene sharing:

(show all 43)
idCompoundScoreTop Affiliating Genes
1tacrine44 1111.0APOE, MAPT
2donepezil44 50 24 1113.0MAPT, APOE
3galantamine44 50 1112.0APOE, MAPT
4guanidine44 24 1111.9APOE, MAPT
5fluorochrome449.9RPS27A, MAPT
6cysteamine44 24 1111.7APOE, RPS27A
7spec-t449.7PSEN1, APOE, MAPT
8vitamin b12449.7MAPT, PSEN1, APOE
9kainate44 2810.7PSEN1, MAPT, APOE
10folate449.6PSEN1, APOE, MAPT
11glycerol44 24 1111.5RPS27A, PSEN1, MAPT
12streptozotocin449.5APOE, RPS27A
13thioflavine s449.4APOE, RPS27A, MAPT
14formate449.4APOE, MAPT, RPS27A
15chloroquine44 1 50 28 1113.4APOE, RPS27A, MAPT
16sodium dodecylsulfate449.4APOE, RPS27A, MAPT
17glycogen44 2410.4RPS27A, MAPT, PSEN1
18biotin44 24 1111.3PSEN1, APOE, RPS27A
19tamoxifen44 50 28 1112.3RPS27A, APOE, GRN
20n acetylcysteine449.3MAPT, APOE, RPS27A
21acetylcholine44 50 28 24 1113.2MAPT, APOE, RPS27A
22proline449.2MAPT, PSEN1, RPS27A
23actinomycin d449.2MAPT, RPS27A, APOE
24paraffin449.1MAPT, RPS27A, APOE
254-hydroxynonenal44 2410.1APOE, PSEN1, RPS27A, MAPT
26lactacystin449.1MAPT, RPS27A, APOE, PSEN1
27valine449.1PSEN1, RPS27A, MAPT, APOE
28nmda44 2810.1MAPT, RPS27A, PSEN1, APOE
29guanidine hydrochloride449.0RPS27A, APOE
30aspartate449.0MAPT, RPS27A, PSEN1, APOE
31threonine449.0MAPT, PSEN1, RPS27A, VCP
32cycloheximide449.0APOE, PSEN1, RPS27A, MAPT
33cyclosporin a44 28 6011.0RPS27A, MAPT, APOE
34h2o2449.0APOE, PSEN1, MAPT, RPS27A
35arginine449.0APOE, PSEN1, RPS27A, MAPT
36glutamate448.9RPS27A, MAPT, PSEN1, APOE
37estrogen448.9MAPT, GRN, APOE, RPS27A
38alanine448.9MAPT, RPS27A, PSEN1, APOE
39dopamine44 28 24 1111.9MAPT, RPS27A, APOE
40superoxide44 249.8TARDBP, PSEN1, VCP
41cysteine448.7PSEN1, GRN, APOE, RPS27A, MAPT
42glutamine448.6RPS27A, MAPT, PSEN1, APOE, VCP
43serine448.5PSEN1, MAPT, RPS27A, APOE, VCP

GO Terms for genes affiliated with Semantic Dementia

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Biological processes related to Semantic Dementia according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of axon extensionGO:00457739.6APOE, MAPT
2endosomal transportGO:00161979.4RPS27A, CHMP2B
3Notch receptor processingGO:00072209.4RPS27A, PSEN1
4positive regulation of proteasomal ubiquitin-dependent protein catabolic processGO:00324369.3PSEN1, VCP
5cell deathGO:00082198.0TARDBP, GRN, CHMP2B, C9orf72, APOE

Molecular functions related to Semantic Dementia according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1lipoprotein particle bindingGO:00718139.5MAPT, APOE
2poly(A) RNA bindingGO:00448228.0TARDBP, GRN, RPS27A, VCP
3protein bindingGO:00055157.2C9orf72, VCP, PSEN1, MAPT, GRN, TARDBP

Sources for Semantic Dementia

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet