MCID: PST086
MIFTS: 33

Posterior Cortical Atrophy malady

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Posterior Cortical Atrophy

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Sources:
52Orphanet, 29ICD10 via Orphanet
See all MalaCards sources

Aliases & Descriptions for Posterior Cortical Atrophy:

Name: Posterior Cortical Atrophy 52
Biparietal Alzheimer Disease 52
 
Benson Syndrome 52
Pca 52

Characteristics:

Orphanet epidemiological data:

52
biparietal alzheimer disease:
Age of onset: Adult; Age of death: adult

Classifications:



External Ids:

Orphanet52 ORPHA54247
ICD10 via Orphanet29 G31.1

Summaries for Posterior Cortical Atrophy

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Wikipedia:69 Posterior cortical atrophy (PCA), also called Benson\'s syndrome, is a form of dementia which is usually... more...

MalaCards based summary: Posterior Cortical Atrophy, also known as biparietal alzheimer disease, is related to amyloidosis, primary localized cutaneous, 1 and choanal atresia, posterior, and has symptoms including oculomotor apraxia, behavioral abnormality and agnosia. An important gene associated with Posterior Cortical Atrophy is APOE (Apolipoprotein E), and among its related pathways are Alzheimer's disease and Neuroscience. Affiliated tissues include cortex, brain and temporal lobe, and related mouse phenotype taste/olfaction.

Related Diseases for Posterior Cortical Atrophy

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Graphical network of the top 20 diseases related to Posterior Cortical Atrophy:



Diseases related to posterior cortical atrophy

Symptoms for Posterior Cortical Atrophy

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HPO human phenotypes related to Posterior Cortical Atrophy:

(show all 7)
id Description Frequency HPO Source Accession
1 oculomotor apraxia hallmark (90%) HP:0000657
2 behavioral abnormality hallmark (90%) HP:0000708
3 agnosia hallmark (90%) HP:0010524
4 abnormality of extrapyramidal motor function typical (50%) HP:0002071
5 memory impairment typical (50%) HP:0002354
6 diplopia occasional (7.5%) HP:0000651
7 developmental regression occasional (7.5%) HP:0002376

Drugs & Therapeutics for Posterior Cortical Atrophy

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Drugs for Posterior Cortical Atrophy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
AcetylcysteinePhase 4304616-91-112035
Synonyms:
(2R)-2-acetylamino-3-Sulfanylpropanoic acid
(R)-2-acetylamino-3-Mercaptopropanoic acid
(R)-Mercapturic acid
2-Acetylamino-3-mercapto-propionate
2-Acetylamino-3-mercapto-propionic acid
ACC
Acetadote
Acetilcisteina
Acetylcysteine
Acetylcysteinum
Fluimicil Infantil
Fluimucetin
Fluimucil
Flumucetin
 
Fluprowit
L-Acetylcysteine
L-alpha-acetamido-beta-Mercaptopropionic acid
Lysox
Mercapturic acid
Mucolysin
Mucomyst
N-ACETYL-L-cysteine
N-Acety-L-Cysteine
N-Acetyl-3-mercaptoalanine
N-Acetyl-L-(+)-cysteine
N-Acetylcysteine
N-acetylcysteine
NAC
Parvolex
Sodium 2-acetamido-3-mercaptopropionate
2
CysteaminePhase 42660-23-16058
Synonyms:
(2-Mercaptoethyl)amine
(Mercaptoethyl)ammonium toluene-p-sulphonate
.beta.-Mercaptoethylamine
1-Amino-2-mercaptoethylamine
139720-70-0
156-57-0 (hydrochloride)
16904-32-8 (di-hydrochloride)
2-AMINO-ETHANETHIOL
2-AMINO-ethanethiol
2-Amino-1-ethanethiol
2-Aminoethanethiol
2-Aminoethyl mercaptan
2-Mercaptoethanamine
2-Mercaptoethylamine
2-Mercaptoethylamine, polymer-bound
27761-19-9 (tartrate (1:1))
2DFDA1F8-7010-4225-8280-AB1C4C43F546
30070_FLUKA
30070_SIGMA
3037-04-5
3037-04-5 (tosylate)
42954-15-4 (hydrobromide)
60-23-1
60-23-1 (Parent)
641022_ALDRICH
93965-19-6 (maleate (1:1))
A0648
AC1L1LPL
AC1Q54NL
AKOS003793343
Aminoethyl mercaptan
Becaptan
C-9500
C01678
C2H7NS
CASH
CCRIS 3083
CHEBI:17141
CHEMBL602
CID6058
CYSTEAMINE
Cisteamina
Cisteamina [Italian]
Cystavision
Cysteamide
Cysteamin
Cysteamine
Cysteamine (USAN)
Cysteamine [USAN:BAN]
Cysteamine bitartate
Cysteamine hydrochloride
Cysteaminium
Cysteinamine
D03634
DB00847
Decarboxycysteine
DivK1c_006750
 
EINECS 200-463-0
EINECS 221-235-7
Ethanethiolamine
HSDB 7353
KBio1_001694
KBio2_002235
KBio2_004803
KBio2_007371
KBioSS_002235
L 1573
L-1573
LS-65761
Lambraten
Lambratene
Lopac-M-6500
M9768_ALDRICH
M9768_SIGMA
MEA
MEA (mercaptan)
Mecramine
Mercamin
Mercamine
Mercaptamin
Mercaptamina
Mercaptamina [INN-Spanish]
Mercaptamine
Mercaptamine (INN)
Mercaptaminum
Mercaptaminum [INN-Latin]
Mercaptoethylamine
Merkamin
MolPort-001-662-635
NCGC00015691-01
NCGC00015691-04
NCGC00162236-01
NCI60_002000
NSC 647528
NSC647528
Riacon
STK315355
SpecPlus_000654
Spectrum_001755
Thioethanolamine
UNII-5UX2SD1KE2
WR 347
b-Aminoethanethiol
b-Aminoethylthiol
b-Mercaptoethylamine
beta-Aminoethanethiol
beta-Aminoethylthiol
beta-MEA
beta-Mercaptoethylamine
bmse000388
cysteamine bitartrate
nchembio.315-comp1
nchembio.316-comp1
╬▓-MEA
╬▓-aminoethylthiol
3
CorticosteronePhase 11050-22-65753
Synonyms:
11,21-Dihydroxypregn-4-ene-3,20-dione
11,21-Dihydroxyprogesterone
11-Hydroxycorticoaldosterone
 
17-Deoxycortisol
4-Pregnene-11 Corticosteron
Corticosterone
Kendall'S compound B
Reichstein'S substance H

Interventional clinical trials:

idNameStatusNCT IDPhase
1Cystagon to Treat Infantile Neuronal Ceroid LipofuscinosisCompletedNCT00028262Phase 4
2Amyloid-related Imaging Abnormalities (Microbleeds) in Atypical ADCompletedNCT01723553Phase 1
3Buccal Fat Pad Derived Stem Cells With Cortical Tenting in Posterior Mandible ReconstructionRecruitingNCT02745366Phase 1
4Influence of Age on Amyloid Load in Alzheimer's Disease and in Atypical Focal Cortical Alzheimer's DiseaseCompletedNCT01095744
5PET Imaging in Chronic Traumatic EncephalopathyCompletedNCT02211820
6Emotional Communication Disorders in Cerebellar DiseaseRecruitingNCT02106819
7Tau Imaging in Young Onset DementiaActive, not recruitingNCT02289118
8Molecular and Structural Imaging in Atypical Alzheimer's Disease: A Longitudinal StudyNot yet recruitingNCT02740634

Search NIH Clinical Center for Posterior Cortical Atrophy

Genetic Tests for Posterior Cortical Atrophy

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Anatomical Context for Posterior Cortical Atrophy

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MalaCards organs/tissues related to Posterior Cortical Atrophy:

34
Cortex, Brain, Temporal lobe, Testes, Endothelial

Animal Models for Posterior Cortical Atrophy or affiliated genes

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MGI Mouse Phenotypes related to Posterior Cortical Atrophy:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053949.1APOE, MAPT

Publications for Posterior Cortical Atrophy

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Articles related to Posterior Cortical Atrophy:

(show top 50)    (show all 162)
idTitleAuthorsYear
1
Posterior cortical atrophy as a primary clinical phenotype of corticobasal syndrome with a progranulin gene rs5848 TT genotype. (26852323)
2016
2
Functional Connectivity of Ventral and Dorsal Visual Streams in Posterior Cortical Atrophy. (26923019)
2016
3
Functional neural substrates of posterior cortical atrophy patients. (25976028)
2015
4
3D Shape Perception in Posterior Cortical Atrophy: A Visual Neuroscience Perspective. (26377458)
2015
5
Facilitating text reading in posterior cortical atrophy. (26138948)
2015
6
Posterior cortical atrophy as an extreme phenotype of GRN mutations. (25546130)
2015
7
Posterior Cortical Atrophy Presenting with Superior Arcuate Field Defect. (26417467)
2015
8
Oculomotor abnormalities in posterior cortical atrophy: are they different from those in Alzheimer's disease after all? (26106096)
2015
9
Tau, amyloid, and hypometabolism in a patient with posterior cortical atrophy. (25448043)
2015
10
Brain Changes within the Visuo-Spatial Attentional Network in Posterior Cortical Atrophy. (25114070)
2014
11
The Pattern of Brain Amyloid Load in Posterior Cortical Atrophy Using (18)F-AV45: Is Amyloid the Principal Actor in the Disease? (25538727)
2014
12
Late-onset Alzheimer disease genetic variants in posterior cortical atrophy and posterior AD. (24670887)
2014
13
Hiding in plain sight: a closer look at posterior cortical atrophy. (25216669)
2014
14
Upside-down Drawing in Posterior Cortical Atrophy. (25373818)
2014
15
Visual field defect as an uncommon presentation of posterior cortical atrophy. (25327905)
2014
16
Shining a light on posterior cortical atrophy. (23274153)
2013
17
Olfactory impairment in posterior cortical atrophy. (23435114)
2013
18
Posterior cortical atrophy: an investigation of scan paths generated during face matching tasks. (23825453)
2013
19
Logopenic syndrome in posterior cortical atrophy. (23007194)
2013
20
Aphasia with left occipitotemporal hypometabolism: a novel presentation of posterior cortical atrophy? (23850398)
2013
21
Posterior cortical atrophy: visuomotor deficits in reaching and grasping. (23801956)
2013
22
Posterior cortical atrophy: review of the recent literature. (24136454)
2013
23
Visuospatial deficits in posterior cortical atrophy: structural and functional correlates. (22645257)
2012
24
Long-standing prion dementia manifesting as posterior cortical atrophy. (21959360)
2012
25
Posterior cortical atrophy: a case study of Benson's syndrome that initially presented as anxiety disorder. (23373609)
2012
26
Physical medicine and rehabilitation multidisciplinary approach in a case of posterior cortical atrophy. (22771215)
2012
27
Ventral and dorsal visual streams in posterior cortical atrophy: a DT MRI study. (22277261)
2012
28
The evolution of alexia in two cases of posterior cortical atrophy. (21876262)
2011
29
Abnormal visual phenomena in posterior cortical atrophy. (20818540)
2011
30
Similar amyloid-I^ burden in posterior cortical atrophy and Alzheimer's disease. (21705422)
2011
31
Presenile dementia diagnosed as posterior cortical atrophy. (21951958)
2011
32
Basic visual function and cortical thickness patterns in posterior cortical atrophy. (21310781)
2011
33
Visuo-spatial imagery impairment in posterior cortical atrophy: a cognitive and SPECT study. (21606573)
2011
34
Neural correlates of cognitive impairment in posterior cortical atrophy. (21478188)
2011
35
Visual impairments in dementia with Lewy bodies and posterior cortical atrophy. (20063945)
2010
36
Action-intentional spatial bias in a patient with posterior cortical atrophy. (20672222)
2010
37
Processing of wh-questions in a case of posterior cortical atrophy. (21271923)
2009
38
Pattern and progression of white-matter changes in a case of posterior cortical atrophy using diffusion tensor imaging. (19289480)
2009
39
Use of the Visual Object and Space Perception (VOSP) test battery in two cases of posterior cortical atrophy. (19031338)
2008
40
Atrophy and decreased activation of fronto-parietal attention areas contribute to higher visual dysfunction in posterior cortical atrophy. (18945600)
2008
41
Posterior cortical atrophy: a rare form of dementia with in vivo evidence of amyloid-beta accumulation. (18997288)
2008
42
Risperidone treatment of complex hallucinations in a patient with posterior cortical atrophy. (18806250)
2008
43
What are we seeing? Is posterior cortical atrophy just Alzheimer disease? (16476924)
2006
44
A case of progressive posterior cortical atrophy (PCA) with vivid hallucination: are some ghost tales vivid hallucinations in normal people? (16484663)
2006
45
Posterior cortical atrophy: a review of the literature. (15788276)
2004
46
Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy. (15477533)
2004
47
Simultanagnosia in posterior cortical atrophy. (11796782)
2002
48
Progressive visual agnosia with posterior cortical atrophy. (8836594)
1996
49
Posterior cortical atrophy in Alzheimer's disease: analysis of a new case and re-evaluation of a historical report. (8213079)
1993
50
Alzheimer's disease in a patient with posterior cortical atrophy. (1783928)
1991

Variations for Posterior Cortical Atrophy

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Expression for genes affiliated with Posterior Cortical Atrophy

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Search GEO for disease gene expression data for Posterior Cortical Atrophy.

Pathways for genes affiliated with Posterior Cortical Atrophy

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Pathways related to Posterior Cortical Atrophy according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
1
Show member pathways
9.1APOE, MAPT
29.1APOE, MAPT
39.1APOE, MAPT
49.1APOE, MAPT

GO Terms for genes affiliated with Posterior Cortical Atrophy

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Cellular components related to Posterior Cortical Atrophy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1dendriteGO:00304259.1APOE, MAPT

Molecular functions related to Posterior Cortical Atrophy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1lipoprotein particle bindingGO:00718139.1APOE, MAPT

Sources for Posterior Cortical Atrophy

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet