MCID: PST086
MIFTS: 35

Posterior Cortical Atrophy malady

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Posterior Cortical Atrophy

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Sources:
31ICD10 via Orphanet, 54Orphanet
See all MalaCards sources

Aliases & Descriptions for Posterior Cortical Atrophy:

Name: Posterior Cortical Atrophy 54
Biparietal Alzheimer Disease 54
 
Benson Syndrome 54
Pca 54

Characteristics:

Orphanet epidemiological data:

54
biparietal alzheimer disease:
Age of onset: Adult; Age of death: adult

Classifications:



External Ids:

Orphanet54 ORPHA54247
ICD10 via Orphanet31 G31.1

Summaries for Posterior Cortical Atrophy

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Wikipedia:71 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 Neuroscience and Reelin Pathway (Cajal-Retzius cells). Affiliated tissues include cortex, brain and spinal cord, 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 & Phenotypes for Posterior Cortical Atrophy

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Human phenotypes related to Posterior Cortical Atrophy:

 64 (show all 7)
id Description HPO Frequency HPO Source Accession
1 oculomotor apraxia64 hallmark (90%) HP:0000657
2 behavioral abnormality64 hallmark (90%) HP:0000708
3 agnosia64 hallmark (90%) HP:0010524
4 abnormality of extrapyramidal motor function64 typical (50%) HP:0002071
5 memory impairment64 typical (50%) HP:0002354
6 diplopia64 occasional (7.5%) HP:0000651
7 developmental regression64 occasional (7.5%) HP:0002376

MGI Mouse Phenotypes related to Posterior Cortical Atrophy according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053949.1APOE, MAPT

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 11)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
AcetylcysteineapprovedPhase 4316616-91-112035
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 42760-23-16058
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
3N-monoacetylcystinePhase 4316
4Protective AgentsPhase 47190
5Respiratory System AgentsPhase 44818
6Anti-Infective AgentsPhase 421402
7ExpectorantsPhase 4388
8AntioxidantsPhase 42928
9Antiviral AgentsPhase 49732
10AntidotesPhase 41038
11
CorticosteronePhase 11150-22-65753
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
2Tau Brain Imaging in Typical and Atypical Alzheimer's Disease (AD)Not yet recruitingNCT03022968Phase 2
3Amyloid-related Imaging Abnormalities (Microbleeds) in Atypical ADCompletedNCT01723553Phase 1
4Buccal Fat Pad Derived Stem Cells With Cortical Tenting in Posterior Mandible ReconstructionRecruitingNCT02745366Phase 1
5Influence of Age on Amyloid Load in Alzheimer's Disease and in Atypical Focal Cortical Alzheimer's DiseaseCompletedNCT01095744
6PET Imaging in Chronic Traumatic EncephalopathyCompletedNCT02211820
7Molecular and Structural Imaging in Atypical Alzheimer's Disease: A Longitudinal StudyRecruitingNCT02740634
8Emotional Communication Disorders in Cerebellar DiseaseRecruitingNCT02106819
9Plasticity of Grey and White Matter in Response to Motor Skill Training in Healthy Individuals and Those With Spinal Cord InjuryRecruitingNCT02148887
10Tau Imaging in Young Onset DementiaActive, not recruitingNCT02289118

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:

36
Cortex, Brain, Spinal cord, Temporal lobe, Endothelial, Testes

Publications for Posterior Cortical Atrophy

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Articles related to Posterior Cortical Atrophy:

(show top 50)    (show all 168)
idTitleAuthorsYear
1
The oral spelling profile of posterior cortical atrophy and the nature of the graphemic representation. (27913156)
2017
2
Progression of oculomotor deficit in a patient with posterior cortical atrophy. (27816183)
2017
3
Posterior cortical atrophy as a primary clinical phenotype of corticobasal syndrome with a progranulin gene rs5848 TT genotype. (26852323)
2016
4
Functional Connectivity of Ventral and Dorsal Visual Streams in Posterior Cortical Atrophy. (26923019)
2016
5
A novel use of arterial spin labelling MRI to demonstrate focal hypoperfusion in individuals with posterior cortical atrophy: a multimodal imaging study. (26733599)
2016
6
Saccades abnormalities in posterior cortical atrophy - A case report. (28063320)
2016
7
Memory Impairment at Initial Clinical Presentation in Posterior Cortical Atrophy. (27128371)
2016
8
Effect of age at onset on cortical thickness and cognition in posterior cortical atrophy. (27318138)
2016
9
Clinical and neuroimaging differences between posterior cortical atrophy and typical amnestic Alzheimer's disease patients at an early disease stage. (27377199)
2016
10
Neuro-cognitive mechanisms of simultanagnosia in patients with posterior cortical atrophy. (27702740)
2016
11
Distinct patterns of increased translocator protein in posterior cortical atrophy and amnestic Alzheimer's disease. (28068564)
2016
12
18F-FDG PET in Posterior Cortical Atrophy and Dementia with Lewy Bodies. (27688479)
2016
13
Can patients without early, prominent visual deficits still be diagnosed of posterior cortical atrophy? (27423559)
2016
14
Non-pharmacological intervention for posterior cortical atrophy. (27574605)
2016
15
Genetic risk factors for the posterior cortical atrophy variant of Alzheimer's disease. (26993346)
2016
16
Functional neural substrates of posterior cortical atrophy patients. (25976028)
2015
17
3D Shape Perception in Posterior Cortical Atrophy: A Visual Neuroscience Perspective. (26377458)
2015
18
Facilitating text reading in posterior cortical atrophy. (26138948)
2015
19
Posterior cortical atrophy as an extreme phenotype of GRN mutations. (25546130)
2015
20
Posterior Cortical Atrophy Presenting with Superior Arcuate Field Defect. (26417467)
2015
21
Oculomotor abnormalities in posterior cortical atrophy: are they different from those in Alzheimer's disease after all? (26106096)
2015
22
Tau, amyloid, and hypometabolism in a patient with posterior cortical atrophy. (25448043)
2015
23
Evaluating pathogenic dementia variants in posterior cortical atrophy. (26507310)
2015
24
Left temporal lobe epilepsy revealing left posterior cortical atrophy due to Alzheimer's disease. (25589715)
2015
25
Treatment of a patient with posterior cortical atrophy (PCA) with chiropractic manipulation and Dynamic Neuromuscular Stabilization (DNS): A case report. (25729084)
2015
26
A Pilot Study on Clinical and Neuroimaging Characteristics of Chinese Posterior Cortical Atrophy: Comparison with Typical Alzheimer's Disease. (26267071)
2015
27
Posterior Cortical Atrophy: A Case Report of a 6-Year Natural Progression. (26704219)
2015
28
Differential hippocampal shapes in posterior cortical atrophy patients: A comparison with control and typical AD subjects. (26461053)
2015
29
Derailed dementia: Alzheimer's disease versus posterior cortical atrophy. (25688631)
2015
30
Neuropsychiatric Symptoms in Posterior Cortical Atrophy and Alzheimer Disease. (26404166)
2015
31
Clinical, FDG and amyloid PET imaging in posterior cortical atrophy. (25862483)
2015
32
Abnormalities of fixation, saccade and pursuit in posterior cortical atrophy. (25895507)
2015
33
Reduced modulation of scanpaths in response to task demands in posterior cortical atrophy. (25596316)
2015
34
Posterior cortical atrophy: an atypical variant of Alzheimer disease. (25998111)
2015
35
Usefulness of Multi-Parametric MRI for the Investigation of Posterior Cortical Atrophy. (26480294)
2015
36
Brain Changes within the Visuo-Spatial Attentional Network in Posterior Cortical Atrophy. (25114070)
2014
37
The Pattern of Brain Amyloid Load in Posterior Cortical Atrophy Using (18)F-AV45: Is Amyloid the Principal Actor in the Disease? (25538727)
2014
38
Late-onset Alzheimer disease genetic variants in posterior cortical atrophy and posterior AD. (24670887)
2014
39
Hiding in plain sight: a closer look at posterior cortical atrophy. (25216669)
2014
40
Upside-down Drawing in Posterior Cortical Atrophy. (25373818)
2014
41
Visual field defect as an uncommon presentation of posterior cortical atrophy. (25327905)
2014
42
Motor features in posterior cortical atrophy and their imaging correlates. (25086839)
2014
43
Pronounced Impairment of Everyday Skills and Self-Care in Posterior Cortical Atrophy. (25096622)
2014
44
Phenotypical variation in Alzheimer's disease: insights from posterior cortical atrophy. (25239627)
2014
45
(Con)text-specific effects of visual dysfunction on reading in posterior cortical atrophy. (24841985)
2014
46
The progression of posterior cortical atrophy to corticobasal syndrome: lumping or splitting neurodegenerative diseases? (25009764)
2014
47
The Neuropsychiatric Profile of Posterior Cortical Atrophy. (25330926)
2014
48
Further evidence about the crucial role of CSF biomarkers in diagnosis of posterior cortical atrophy. (24487699)
2014
49
Different levels of implicit emotional recognition in posterior cortical atrophy (PCA). (24874521)
2014
50
Syndrome-Specific Patterns of Regional Cerebral Glucose Metabolism in Posterior Cortical Atrophy in Comparison to Dementia with Lewy Bodies and Alzheimer's Disease-A [F-18]-Fdg Pet Study. (24593796)
2014

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
19.1APOE, 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

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
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet