MCID: PST086
MIFTS: 37

Posterior Cortical Atrophy malady

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Posterior Cortical Atrophy

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Sources:
51Orphanet, 28ICD10 via Orphanet
See all MalaCards sources

Aliases & Descriptions for Posterior Cortical Atrophy:

Name: Posterior Cortical Atrophy 51
Biparietal Alzheimer Disease 51
 
Benson Syndrome 51
Pca 51

Characteristics:

Orphanet epidemiological data:

51
biparietal alzheimer disease:
Age of onset: Adult; Age of death: adult

Classifications:



External Ids:

Orphanet51 54247
ICD10 via Orphanet28 G31.1

Summaries for Posterior Cortical Atrophy

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Wikipedia:68 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 cerebritis and amyloidosis, primary localized cutaneous, 1, 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 Alzheimers disease and Reelin Pathway (Cajal-Retzius cells). Affiliated tissues include cortex, brain and heart, and related mouse phenotype taste/olfaction.

Related Diseases for Posterior Cortical Atrophy

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Diseases related to Posterior Cortical Atrophy via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 48)
idRelated DiseaseScoreTop Affiliating Genes
1cerebritis29.4APOE, MAPT
2amyloidosis, primary localized cutaneous, 110.9
3primary cutaneous amyloidosis10.9
4choanal atresia10.9
5prostate cancer10.4
6prostatitis10.4
7dementia10.3
8alzheimer disease10.1
9alexia10.1
10corticobasal syndrome10.0
11moyamoya disease10.0
12simultanagnosia9.9
13agraphia9.9
14agnosia9.9
15focal myositis9.9APOE, MAPT
16isolated tracheo-esophageal fistula9.9APOE, MAPT
17semmekrot haraldsson weemaes syndrome9.9APOE, MAPT
18spermatogenic failure 109.9APOE, MAPT
19birdshot chorioretinopathy9.9APOE, MAPT
20communicating hydrocephalus9.9APOE, MAPT
21lipoprotein glomerulopathy9.9APOE, MAPT
22encephalitozoonosis9.8APOE, MAPT
23supranuclear palsy, progressive atypical9.8APOE, MAPT
24writing disorder9.8APOE, MAPT
25milker's nodule9.8APOE, MAPT
26parkinson disease 19.8APOE, MAPT
27pick disease9.8APOE, MAPT
28temporal lobe epilepsy9.8
29aphasia9.8
30visual agnosia9.8
31parametritis9.8
32anxiety disorder9.8
33corneal dystrophy9.8
34dysgraphia9.8
35tauopathy9.8
36corticobasal degeneration9.8
37endotheliitis9.8
38candidiasis9.8APOE, MAPT
39cardiomyopathy, dilated, 1u9.8APOE, MAPT
40peripheral vascular disease9.7APOE, MAPT
41gerstmann-straussler disease9.7APOE, MAPT
42upper lip cancer9.7APOE, MAPT
43colon kaposi sarcoma9.6APOE, MAPT
44mitochondrial metabolism disease9.6APOE, MAPT
45atopic dermatitis9.5APOE, MAPT
46hypersensitivity syndrome, carbamazepine-induced9.5APOE, MAPT
47acquired hyperkeratosis9.4APOE, MAPT
48parkinson disease, late-onset9.3APOE, MAPT

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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Symptoms:

 51 (show all 8)
  • oculomotor apraxia/dyspraxia
  • alexia/agraphia/writing/reading troubles
  • visual/auditory/tactile agnosia/propopagnosia/simultagnosia
  • humour troubles/anxiety/depression/apathy/euphoria/irritability
  • extrapyramidal syndrome
  • troubles of memory/amnesia/hypermnesia
  • diplopia/double vision
  • psychic/psychomotor regression/dementia/intellectual decline

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):

(show all 10)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
AcetylcysteineapprovedPhase 4293616-91-112035
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
Cysteamineapproved, investigationalPhase 42360-23-16058
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]
Cystagon
Cystaran
Cystavision
Cysteamide
Cysteamin
Cysteamine
Cysteamine (USAN)
Cysteamine Bitartrate
Cysteamine Hydrochloride
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
3Protective AgentsPhase 45651
4Respiratory System AgentsPhase 43931
5N-monoacetylcystinePhase 4293
6ExpectorantsPhase 4337
7Antiviral AgentsPhase 48071
8Anti-Infective AgentsPhase 417220
9AntioxidantsPhase 42442
10
CorticosteronePhase 1950-22-65753
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
5Emotional Communication Disorders in Cerebellar DiseaseRecruitingNCT02106819
6Tau Imaging in Young Onset DementiaActive, not recruitingNCT02289118
7Molecular and Structural Imaging in Atypical Alzheimer's Disease: A Longitudinal StudyNot yet recruitingNCT02740634
8PET Imaging in Chronic Traumatic EncephalopathySuspendedNCT02211820

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:

33
Cortex, Brain, Heart, Lung, Prostate, Breast, Liver

Animal Models for Posterior Cortical Atrophy or affiliated genes

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MGI Mouse Phenotypes related to Posterior Cortical Atrophy:

38
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 158)
idTitleAuthorsYear
1
Memory Impairment at Initial Clinical Presentation in Posterior Cortical Atrophy. (27128371)
2016
2
Posterior cortical atrophy as a primary clinical phenotype of corticobasal syndrome with a progranulin gene rs5848 TT genotype. (26852323)
2016
3
Functional Connectivity of Ventral and Dorsal Visual Streams in Posterior Cortical Atrophy. (26923019)
2016
4
Posterior Cortical Atrophy: A Case Report of a 6-Year Natural Progression. (26704219)
2015
5
Differential hippocampal shapes in posterior cortical atrophy patients: A comparison with control and typical AD subjects. (26461053)
2015
6
Functional neural substrates of posterior cortical atrophy patients. (25976028)
2015
7
Derailed dementia: Alzheimer's disease versus posterior cortical atrophy. (25688631)
2015
8
3D Shape Perception in Posterior Cortical Atrophy: A Visual Neuroscience Perspective. (26377458)
2015
9
Neuropsychiatric Symptoms in Posterior Cortical Atrophy and Alzheimer Disease. (26404166)
2015
10
Facilitating text reading in posterior cortical atrophy. (26138948)
2015
11
(Con)text-specific effects of visual dysfunction on reading in posterior cortical atrophy. (24841985)
2014
12
Brain Changes within the Visuo-Spatial Attentional Network in Posterior Cortical Atrophy. (25114070)
2014
13
The Pattern of Brain Amyloid Load in Posterior Cortical Atrophy Using (18)F-AV45: Is Amyloid the Principal Actor in the Disease? (25538727)
2014
14
Late-onset Alzheimer disease genetic variants in posterior cortical atrophy and posterior AD. (24670887)
2014
15
The progression of posterior cortical atrophy to corticobasal syndrome: lumping or splitting neurodegenerative diseases? (25009764)
2014
16
Hiding in plain sight: a closer look at posterior cortical atrophy. (25216669)
2014
17
Upside-down Drawing in Posterior Cortical Atrophy. (25373818)
2014
18
Shining a light on posterior cortical atrophy. (23274153)
2013
19
Olfactory impairment in posterior cortical atrophy. (23435114)
2013
20
Visual impairment and posterior cortical atrophy preceding rapid progressive dementia. (23283613)
2013
21
Posterior cortical atrophy: an investigation of scan paths generated during face matching tasks. (23825453)
2013
22
Logopenic syndrome in posterior cortical atrophy. (23007194)
2013
23
Ophthalmic impairment or higher-order visual deficit? Posterior cortical atrophy: a case report. (23373584)
2012
24
Visuospatial deficits in posterior cortical atrophy: structural and functional correlates. (22645257)
2012
25
Brain networks in posterior cortical atrophy: a single case tractography study and literature review. (22099855)
2012
26
Long-standing prion dementia manifesting as posterior cortical atrophy. (21959360)
2012
27
Huntington's disease presenting as posterior cortical atrophy. (21625777)
2011
28
Posterior cortical atrophy: an exemplar for renovating diagnostic formulation in neuropsychiatry. (21497228)
2011
29
The evolution of alexia in two cases of posterior cortical atrophy. (21876262)
2011
30
Abnormal visual phenomena in posterior cortical atrophy. (20818540)
2011
31
Similar amyloid-I^ burden in posterior cortical atrophy and Alzheimer's disease. (21705422)
2011
32
Posterior cortical atrophy with prominent alexia without agraphia in a Tourette syndrome. (21912890)
2011
33
Presenile dementia diagnosed as posterior cortical atrophy. (21951958)
2011
34
Basic visual function and cortical thickness patterns in posterior cortical atrophy. (21310781)
2011
35
Cognitive rehabilitation in posterior cortical atrophy. (20306369)
2010
36
Visual impairments in dementia with Lewy bodies and posterior cortical atrophy. (20063945)
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
The role of neuropsychology in distinguishing the posterior cortical atrophy syndrome and Alzheimer's disease. (19542605)
2009
40
Use of the Visual Object and Space Perception (VOSP) test battery in two cases of posterior cortical atrophy. (19031338)
2008
41
Atrophy and decreased activation of fronto-parietal attention areas contribute to higher visual dysfunction in posterior cortical atrophy. (18945600)
2008
42
Foveal crowding in posterior cortical atrophy: a specific early-visual-processing deficit affecting word reading. (18161498)
2007
43
Exploded drawing in posterior cortical atrophy. (17130426)
2006
44
Visual hallucinations in posterior cortical atrophy. (17030659)
2006
45
What are we seeing? Is posterior cortical atrophy just Alzheimer disease? (16476924)
2006
46
Posterior cortical atrophy: a review of the literature. (15788276)
2004
47
Cortical sensory loss in a patient with posterior cortical atrophy: a case report. (15849159)
2004
48
Dementia following posterior cortical atrophy--a descriptive clinical case report. (15374033)
2001
49
Alexia and agraphia in posterior cortical atrophy. (9118197)
1997
50
Posterior cortical atrophy: neuropathological correlations. (8859056)
1996

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

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

Sources for Posterior Cortical Atrophy

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet