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MCID: SMN008
MIFTS: 54

Semantic Dementia

Categories: Rare diseases, Neuronal diseases, Mental diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Semantic Dementia

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MalaCards integrated aliases for Semantic Dementia:

Name: Semantic Dementia 53 54 59 55 73
Semantic Primary Progressive Aphasia 53 59
Semantic Variant Ppa 53 59
Dementia - Semantic 54

Characteristics:

Orphanet epidemiological data:

59
semantic dementia
Inheritance: Multigenic/multifactorial,Not applicable; Age of onset: Adult;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Neuronal diseases Mental diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA100069
ICD10 via Orphanet 34 G31.0
UMLS via Orphanet 74 C0338462
UMLS 73 C0338462
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Summaries for Semantic Dementia

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NINDS : 54 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.  The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD.  Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex.  These designations will continue to be debated.  As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language.  The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic) and includes inappropriate social behavior; lack of social tact; lack of empathy; distractability; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation.  The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms.  Spatial skills and memory remain intact.  There is a strong genetic component to the disease; FTD often runs in families.

MalaCards based summary : Semantic Dementia, also known as semantic primary progressive aphasia, is related to frontotemporal lobar degeneration with tdp43 inclusions, grn-related and prosopagnosia. An important gene associated with Semantic Dementia is PSEN1 (Presenilin 1), and among its related pathways/superpathways are Neuroscience and Alzheimers Disease Pathway. The drugs Dopamine and Memantine have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and testes, and related phenotypes are dementia and aphasia

NIH Rare Diseases : 53 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 100069Disease definitionSemantic dementia (SD) is a form of frontotemporal dementia (FTD; see this term), characterized by the progressive, amodal and profound loss of semantic knowledge (combination of visual associative agnosia, anomia, surface dyslexia or dysgraphia and disrupted comprehension of word meaning) and behavioral abnormalities, attributable to the degeneration of the anterior temporal lobes.Visit the Orphanet disease page for more resources.

Wikipedia : 76 Semantic dementia (SD), also known as semantic variant primary progressive aphasia (svPPA), is a... more...

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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 top 50) (show all 69)
# Related Disease Score Top Affiliating Genes
1 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 32.3 GRN MAPT RPS27A
2 prosopagnosia 30.7 GRN PSEN1
3 posterior cortical atrophy 30.7 APOE MAPT
4 alexia 30.4 APOE PSEN1 TARDBP
5 corticobasal degeneration 30.2 MAPT RPS27A TARDBP
6 pick disease of brain 30.1 APOE GRN MAPT PSEN1 RPS27A TARDBP
7 alzheimer disease 29.9 APOE GRN MAPT PSEN1 TARDBP
8 agraphia 29.4 GRN MAPT PSEN1 TARDBP
9 apraxia 29.1 C9orf72 GRN MAPT PSEN1
10 motor neuron disease 28.6 C9orf72 GRN MAPT RPS27A TARDBP
11 aphasia 28.2 APOE C9orf72 CHMP2B GRN MAPT PSEN1
12 supranuclear palsy, progressive, 1 27.5 APOE C9orf72 GRN MAPT PSEN1 RPS27A
13 dementia 26.5 APOE C9orf72 CHMP2B GRN MAPT PSEN1
14 frontotemporal dementia 26.5 APOE C9orf72 CHMP2B GRN MAPT PSEN1
15 progressive non-fluent aphasia 25.9 C9orf72 CHMP2B GRN MAPT PSEN1 TMEM106B
16 amyotrophic lateral sclerosis 1 24.2 C9orf72 CHMP2B GRN MAPT PSEN1 RPS27A
17 simultanagnosia 10.6 APOE MAPT
18 postencephalitic parkinson disease 10.6 MAPT TARDBP
19 expressive language disorder 10.5 PSEN1 TARDBP
20 alzheimer disease 4 10.5 APOE PSEN1
21 kluver-bucy syndrome 10.5 MAPT PSEN1
22 frontotemporal dementia with parkinsonism-17 10.5
23 amyotrophic lateral sclerosis 18 10.4 C9orf72 TARDBP
24 communicating hydrocephalus 10.4 APOE MAPT
25 hydrocephalus, normal-pressure 10.4 APOE MAPT
26 progressive muscular atrophy 10.3 C9orf72 TARDBP
27 amyotrophic lateral sclerosis 21 10.3 C9orf72 TARDBP
28 binswanger's disease 10.3 APOE MAPT
29 dyslexia 10.3
30 leukoencephalopathy, hereditary diffuse, with spheroids 10.3 MAPT RPS27A
31 early-onset, autosomal dominant alzheimer disease 10.3 PSEN1 TREM2
32 visual agnosia 10.2 GRN PSEN1
33 spinocerebellar ataxia 11 10.2 MAPT TARDBP
34 familial idiopathic basal ganglia calcification 10.2 APOE MAPT PSEN1
35 alzheimer disease 2 10.2 APOE MAPT PSEN1
36 cerebral amyloid angiopathy, cst3-related 10.2 APOE MAPT PSEN1
37 vascular dementia 10.2 APOE MAPT PSEN1
38 neuronal intranuclear inclusion disease 10.2 MAPT RPS27A
39 phonagnosia 10.2 GRN PSEN1 TARDBP
40 associative agnosia 10.2 GRN PSEN1 TARDBP
41 writing disorder 10.1 GRN PSEN1 TARDBP
42 echolalia 10.1 GRN MAPT PSEN1
43 cerebritis 10.1
44 neuronitis 10.1
45 gait apraxia 10.1 APOE GRN PSEN1
46 classic progressive supranuclear palsy syndrome 10.0
47 perry syndrome 10.0 C9orf72 GRN TARDBP
48 dysgraphia 10.0
49 aging 10.0
50 herpes simplex 10.0

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



Diseases related to Semantic Dementia
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Symptoms & Phenotypes for Semantic Dementia

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Human phenotypes related to Semantic Dementia:

59 32 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dementia 59 32 frequent (33%) Frequent (79-30%) HP:0000726
2 aphasia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002381
3 brain atrophy 59 32 hallmark (90%) Very frequent (99-80%) HP:0012444
4 visual agnosia 59 32 hallmark (90%) Very frequent (99-80%) HP:0030222
5 anomia 59 32 hallmark (90%) Very frequent (99-80%) HP:0030784
6 abnormal test result 59 32 hallmark (90%) Very frequent (99-80%) HP:0500014
7 dyslexia 59 32 frequent (33%) Frequent (79-30%) HP:0010522
8 alexia 59 32 frequent (33%) Frequent (79-30%) HP:0010523
9 dysgraphia 59 32 frequent (33%) Frequent (79-30%) HP:0010526
10 abulia 59 32 frequent (33%) Frequent (79-30%) HP:0012671
11 neurological speech impairment 59 Frequent (79-30%)

MGI Mouse Phenotypes related to Semantic Dementia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.92 GRN MAPT PCBP2 PSEN1 TARDBP APOE
2 homeostasis/metabolism MP:0005376 9.86 GRN MAPT PCBP2 PSEN1 TARDBP TMEM106B
3 immune system MP:0005387 9.7 C9orf72 GRN MAPT PSEN1 TARDBP APOE
4 nervous system MP:0003631 9.56 CHMP2B GRN MAPT PSEN1 TARDBP APOE
5 no phenotypic analysis MP:0003012 9.1 GRN MAPT TARDBP APOE TREM2 C9orf72
Sources

Drugs & Therapeutics for Semantic Dementia

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Drugs for Semantic Dementia (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 Semantic Dementia

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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:

41
Brain, Temporal Lobe, Testes, Liver, Cortex, Bone, Prefrontal Cortex
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Publications for Semantic Dementia

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Articles related to Semantic Dementia:

(show top 50) (show all 358)
# Title Authors Year
1
Posterior Associative and Cingulate Cortex Involvement of Brain Single-Photon Emission Computed Tomography (SPECT) Imaging in Semantic Dementia with Probable Alzheimer Disease Pathology: A Case Report. ( 29429985 )
2018
2
Why do herpes simplex encephalitis and semantic dementia show a different pattern of semantic impairment in spite of their main common involvement within the anterior temporal lobes? ( 29573378 )
2018
3
Distinct Interplay Between Atrophy and Hypometabolism in Alzheimer's Versus Semantic Dementia. ( 29668866 )
2018
4
Caregiver Burden in Semantic Dementia with Right- and Left-Sided Predominant Cerebral Atrophy and in Behavioral-Variant Frontotemporal Dementia. ( 29706989 )
2018
5
Aide-mAcmoires in semantic dementia. ( 29875167 )
2018
6
Lexical and Grammatical Factors in Sentence Production in Semantic Dementia: Insights From Greek. ( 29635463 )
2018
7
Reading and visual word recognition ability in semantic dementia is not predicted by semantic performance. ( 29432768 )
2018
8
Association between semantic dementia and progressive supranuclear palsy. ( 29661923 )
2018
9
What semantic dementia teaches us about the functional organization of the left posterior fusiform gyrus. ( 28951166 )
2017
10
Diverging Progression of Network Disruption and Atrophy in Alzheimer's Disease and Semantic Dementia. ( 27802229 )
2017
11
Brain Network for the Core Deficits of Semantic Dementia: A Neural Network Connectivity-Behavior Mapping Study. ( 28579952 )
2017
12
Rethinking the Cognitive Mechanisms Underlying Pantomime of Tool Use: Evidence from Alzheimer's Disease and Semantic Dementia. ( 28205493 )
2017
13
Gustatory Dysfunction as an Early Symptom of Semantic Dementia. ( 29430242 )
2017
14
Semantic dementia and the left and right temporal lobes. ( 28947063 )
2017
15
Famous faces and voices: Differential profiles in early right and left semantic dementia and in Alzheimer's disease. ( 27916672 )
2017
16
A novel prion protein variant in a patient with semantic dementia. ( 28572272 )
2017
17
Semantic word category processing in semantic dementia and posterior cortical atrophy. ( 28624681 )
2017
18
Cognitive consequences of the left-right asymmetry of atrophy in semantic dementia. ( 29289335 )
2017
19
A SEMantic and EPisodic Memory Test (SEMEP) Developed within the Embodied Cognition Framework: Application to Normal Aging, Alzheimer's Disease and Semantic Dementia. ( 28955261 )
2017
20
Semantic dementia, progressive non-fluent aphasia and their association with amyotrophic lateral sclerosis. ( 28554960 )
2017
21
Topological Alterations and Symptom-Relevant Modules in the Whole-Brain Structural Network in Semantic Dementia. ( 28731453 )
2017
22
Heterogeneous ribonuclear protein E2 (hnRNP E2) is associated with TDP-43-immunoreactive neurites in Semantic Dementia but not with other TDP-43 pathological subtypes of Frontotemporal Lobar Degeneration. ( 28666471 )
2017
23
Sensitivity of the Wechsler Abbreviated Scale of Intelligence-Second Edition (WASI-II) to the Neurocognitive Deficits Associated with the Semantic Dementia Variant of Frontotemporal Lobar Degeneration: A Case Study. ( 27101084 )
2016
24
An update on semantic dementia: genetics, imaging, and pathology. ( 27915998 )
2016
25
Semantic Dementia: A Mini-Review. ( 28019640 )
2016
26
Two cases of food aversion with semantic dementia. ( 26963051 )
2016
27
Preservation of episodic memory in semantic dementia: The importance of regions beyond the medial temporal lobes. ( 26683384 )
2016
28
Somatic Symptom Disorder in Semantic Dementia: The Role of Alexisomia. ( 27647568 )
2016
29
Distinct white matter injury associated with medial temporal lobe atrophy in Alzheimer's versus semantic dementia. ( 27981671 )
2016
30
Amyloid deposition in semantic dementia: a positron emission tomography study. ( 26807731 )
2016
31
Discovering EEG resting state alterations of semantic dementia. ( 27072087 )
2016
32
Regularity and beyond: Impaired production and comprehension of inflectional morphology in semantic dementia. ( 26994740 )
2016
33
Atrophy in Alzheimer's Disease andA Semantic Dementia: An ALE Meta-Analysis of Voxel-Based Morphometry Studies. ( 27567843 )
2016
34
Distinct neural substrates of affective and cognitive theory of mind impairment in semantic dementia. ( 26998659 )
2016
35
Integrated PET/MRI imaging of semantic dementia. ( 27381147 )
2016
36
Neuroeconomic dissociation of semantic dementia and behavioural variant frontotemporal dementia. ( 26667277 )
2016
37
Prosopagnosia as unusual presentation of semantic dementia: a case study. ( 27489094 )
2016
38
On the right side? A longitudinal study of left- versus right-lateralized semantic dementia. ( 26811253 )
2016
39
Successful short-term re-learning and generalisation of concepts in semantic dementia. ( 27677318 )
2016
40
The Left Fusiform Gyrus is a Critical Region Contributing to the Core Behavioral Profile of Semantic Dementia. ( 27242479 )
2016
41
Relying on procedural memory to enhance independence in daily living activities: Smartphone use in a case of semantic dementia. ( 25558769 )
2015
42
Vocabulary relearning in semantic dementia: Positive and negative consequences of increasing variability in the learning experience. ( 25585251 )
2015
43
The Brain Connectivity Basis of Semantic Dementia: A Selective Review. ( 26336932 )
2015
44
Impairments in the Face-Processing Network in Developmental Prosopagnosia and Semantic Dementia. ( 26705265 )
2015
45
Dissociated word production and comprehension in semantic dementia. ( 25987438 )
2015
46
&amp;quot;Knowing What You Don't Know&amp;quot;: Language Insight in Semantic Dementia. ( 25720396 )
2015
47
For richer or poorer? Imageability effects in semantic dementia patients' reading aloud. ( 25804665 )
2015
48
Preservation of Person-Specific Semantic Knowledge in Semantic Dementia: Does Direct Personal Experience Have a Specific Role? ( 26635578 )
2015
49
Mechanical Problem-Solving Strategies in Alzheimer's Disease and Semantic Dementia. ( 26523522 )
2015
50
Declining object recognition performance in semantic dementia: A case for stored visual object representations. ( 27355607 )
2015
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Variations for Semantic Dementia

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Expression for Semantic Dementia

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Search GEO for disease gene expression data for Semantic Dementia.
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Pathways for Semantic Dementia

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

# Super pathways Score Top Affiliating Genes
1
Neuroscience 4
11.63 APOE MAPT PSEN1 RPS27A TARDBP TREM2
2
Alzheimers Disease Pathway 64
10.79 APOE MAPT PSEN1
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GO Terms for Semantic Dementia

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Cellular components related to Semantic Dementia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endosome GO:0005768 9.62 C9orf72 CHMP2B GRN TMEM106B
2 dendrite GO:0030425 9.56 APOE C9orf72 MAPT PSEN1
3 growth cone GO:0030426 9.33 C9orf72 MAPT PSEN1
4 lysosome GO:0005764 9.26 C9orf72 CHMP2B GRN TMEM106B
5 main axon GO:0044304 8.62 C9orf72 MAPT

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

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.67 APOE MAPT TARDBP
2 cellular protein metabolic process GO:0044267 9.65 APOE PSEN1 RPS27A
3 autophagy GO:0006914 9.58 C9orf72 CHMP2B PSEN1
4 regulation of synaptic plasticity GO:0048167 9.54 MAPT PSEN1
5 synapse organization GO:0050808 9.52 MAPT PSEN1
6 negative regulation of type I interferon production GO:0032480 9.51 PCBP2 RPS27A
7 viral life cycle GO:0019058 9.46 CHMP2B RPS27A
8 positive regulation of dendritic spine development GO:0060999 9.43 APOE PSEN1
9 stress granule assembly GO:0034063 9.37 C9orf72 MAPT
10 amyloid precursor protein metabolic process GO:0042982 9.32 APOE PSEN1
11 virion assembly GO:0019068 9.26 APOE RPS27A
12 astrocyte activation GO:0048143 9.16 MAPT PSEN1
13 negative regulation of protein phosphorylation GO:0001933 9.13 C9orf72 PSEN1 TARDBP
14 positive regulation of amyloid fibril formation GO:1905908 8.62 APOE PSEN1

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

# Name GO ID Score Top Affiliating Genes
1 lipoprotein particle binding GO:0071813 8.62 APOE MAPT
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Sources for Semantic Dementia

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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