PCA
MCID: PST086
MIFTS: 35

Posterior Cortical Atrophy (PCA)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Posterior Cortical Atrophy

MalaCards integrated aliases for Posterior Cortical Atrophy:

Name: Posterior Cortical Atrophy 59
Biparietal Alzheimer Disease 59
Benson Syndrome 59
Pca 59

Characteristics:

Orphanet epidemiological data:

59
posterior cortical atrophy
Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 34 G31.1
Orphanet 59 ORPHA54247

Summaries for Posterior Cortical Atrophy

MalaCards based summary : Posterior Cortical Atrophy, also known as biparietal alzheimer disease, is related to simultanagnosia and aphasia. An important gene associated with Posterior Cortical Atrophy is APOE (Apolipoprotein E), and among its related pathways/superpathways are Reelin Pathway (Cajal-Retzius cells) and G-protein signaling_Regulation of RAC1 activity. Affiliated tissues include cortex, brain and eye, and related phenotypes are ataxia and anxiety

Wikipedia : 75 Posterior cortical atrophy (PCA), also called Benson's syndrome, is a form of dementia which is usually... more...

Related Diseases for Posterior Cortical Atrophy

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

(show top 50) (show all 231)
# Related Disease Score Top Affiliating Genes
1 simultanagnosia 30.7 MAPT APOE
2 aphasia 30.7 MAPT APOE
3 creutzfeldt-jakob disease 30.1 MAPT APOE
4 pick disease of brain 30.1 MAPT APOE
5 semantic dementia 30.1 MAPT APOE
6 dementia, lewy body 30.1 MAPT APOE
7 supranuclear palsy, progressive, 1 29.7 MAPT APOE
8 speech and communication disorders 29.7 MAPT APOE
9 frontotemporal dementia 29.4 MAPT APOE
10 cerebrovascular disease 29.4 MAPT APOE
11 amyloidosis, primary localized cutaneous, 1 11.4
12 choanal atresia, posterior 11.4
13 primary cutaneous amyloidosis 11.2
14 pain agnosia 10.9
15 alzheimer disease 10.7
16 agnosia 10.6
17 apraxia 10.6
18 visual agnosia 10.6
19 alexia 10.5
20 dysgraphia 10.5
21 dementia 10.5
22 corticobasal degeneration 10.5
23 agraphia 10.4
24 prostate cancer 10.4
25 gerstmann syndrome 10.4
26 ataxia and polyneuropathy, adult-onset 10.3
27 dyslexia 10.3
28 prosopagnosia 10.3
29 prion disease 10.3
30 48,xyyy 10.3
31 cerebral atrophy 10.2
32 oculomotor apraxia 10.2
33 scoliosis 10.2
34 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 10.2
35 dyscalculia 10.2
36 nominal aphasia 10.2
37 ideomotor apraxia 10.2
38 allergic hypersensitivity disease 10.2
39 aneurysm 10.2
40 prostatic hyperplasia, benign 10.1
41 mucositis 10.1
42 prostatic hypertrophy 10.1
43 prostatic adenoma 10.1
44 adenocarcinoma 10.1
45 osteoarthritis 10.1
46 yemenite deaf-blind hypopigmentation syndrome 10.1
47 behavioral variant of frontotemporal dementia 10.1
48 myoclonus 10.1
49 balint syndrome 10.1
50 kohlschutter-tonz syndrome 10.1 MAPT APOE

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



Diseases related to Posterior Cortical Atrophy

Symptoms & Phenotypes for Posterior Cortical Atrophy

Human phenotypes related to Posterior Cortical Atrophy:

59 32 (show all 22)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ataxia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001251
2 anxiety 59 32 hallmark (90%) Very frequent (99-80%) HP:0000739
3 confusion 59 32 hallmark (90%) Very frequent (99-80%) HP:0001289
4 dysgraphia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010526
5 oculomotor apraxia 59 32 hallmark (90%) Very frequent (99-80%) HP:0000657
6 finger agnosia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010525
7 cerebral visual impairment 59 32 hallmark (90%) Very frequent (99-80%) HP:0100704
8 dyscalculia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002442
9 alexia 59 32 hallmark (90%) Very frequent (99-80%) HP:0010523
10 photophobia 59 32 frequent (33%) Frequent (79-30%) HP:0000613
11 dyslexia 59 32 frequent (33%) Frequent (79-30%) HP:0010522
12 limb apraxia 59 32 frequent (33%) Frequent (79-30%) HP:0030217
13 color vision defect 32 frequent (33%) HP:0000551
14 memory impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0002354
15 visual hallucinations 59 32 occasional (7.5%) Occasional (29-5%) HP:0002367
16 speech apraxia 59 32 occasional (7.5%) Occasional (29-5%) HP:0011098
17 language impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0002463
18 inertia 59 32 occasional (7.5%) Occasional (29-5%) HP:0030216
19 abnormal rapid eye movement sleep 59 32 occasional (7.5%) Occasional (29-5%) HP:0002494
20 abnormality of vision 59 Frequent (79-30%)
21 abnormality of color vision 59 Frequent (79-30%)
22 agnosia 59 Very frequent (99-80%)

MGI Mouse Phenotypes related to Posterior Cortical Atrophy:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 taste/olfaction MP:0005394 8.62 APOE MAPT

Drugs & Therapeutics for Posterior Cortical Atrophy

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Tau Brain Imaging in Typical and Atypical Alzheimer's Disease (AD) Unknown status NCT03022968 Phase 2 [18F]T807 PET
2 [18F]-T807 PET/CT Imaging of Tau Pathology in Young Onset Focal Dementia Completed NCT02289118 [18F]-T807 imaging tracer
3 Influence of Age on amyloïd Load in Alzheimer's Disease and in Atypical Focal Cortical Alzheimer's Disease Like Posterior Cortical Atrophy (PCA) and Logopenic Progressive Aphasia (LPA)Using Positron Emitting Tomography (PET) Imaging Completed NCT01095744
4 Development and Evaluation of the Effectiveness of Rehabilitation Tools for Neurovisual Disorders in Patients With Posterior Cortical Atrophy (PCA) Not yet recruiting NCT04041089

Search NIH Clinical Center for Posterior Cortical Atrophy

Genetic Tests for Posterior Cortical Atrophy

Anatomical Context for Posterior Cortical Atrophy

MalaCards organs/tissues related to Posterior Cortical Atrophy:

41
Cortex, Brain, Eye, Testes, Temporal Lobe, Thyroid, Endothelial

Publications for Posterior Cortical Atrophy

Articles related to Posterior Cortical Atrophy:

(show top 50) (show all 392)
# Title Authors PMID Year
1
Longitudinal progression of posterior cortical atrophy over 11 years: Relationship between lesion topology and clinical deficits. 38
31322045 2019
2
Where words meet numbers: Comprehension of measurement unit terms in posterior cortical atrophy. 38
31095931 2019
3
Role of Population Receptive Field Size in Complex Visual Dysfunctions: A Posterior Cortical Atrophy Model. 38
31403655 2019
4
Working memory in posterior cortical atrophy. 38
30963338 2019
5
Diagnostic evaluation and monitoring of patients with posterior cortical atrophy. 38
31392920 2019
6
Does the Right Focal Variant of Alzheimer's Disease Really Exist? A Literature Analysis. 38
31381515 2019
7
Retinal thickness as potential biomarker in posterior cortical atrophy and typical Alzheimer's disease. 38
31319885 2019
8
A case of ALS with posterior cortical atrophy. 38
31284766 2019
9
Clinical utility of 18F-FDG-PET/MRI brain in dementia: Preliminary experience from a geriatric clinic in South India. 38
31336358 2019
10
Health and social care practitioners' understanding of the problems of people with dementia-related visual processing impairment. 38
30737853 2019
11
The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman. 38
31203704 2019
12
Longitudinal progression of grey matter atrophy in non-amnestic Alzheimer's disease. 38
31135048 2019
13
Visual cognition in non-amnestic Alzheimer's disease: Relations to tau, amyloid, and cortical atrophy. 38
31200149 2019
14
The functional neuroanatomy of musical memory in Alzheimer's disease. 38
30846199 2019
15
Longitudinal neuroanatomical and cognitive progression of posterior cortical atrophy. 38
31219516 2019
16
Reply to letter: Basis of cingulate island sign may differ in dementia with lewy bodies and posterior cortical atrophy. 38
31091363 2019
17
DIVE: A spatiotemporal progression model of brain pathology in neurodegenerative disorders. 38
30844504 2019
18
Dopaminergic degeneration induces early posterior cortical thinning in Parkinson's disease. 38
30408592 2019
19
Structural imaging findings on non-enhanced computed tomography are severely underreported in the primary care diagnostic work-up of subjective cognitive decline. 38
30656357 2019
20
Difficulty differentiating a case of posterior cortical atrophy from a psychogenic disturbance of vision. 38
30997705 2019
21
Alzheimer's Disease Including Focal Presentations. 38
30925614 2019
22
Detection and localisation of hesitant steps in people with Alzheimer's disease navigating routes of varying complexity. 38
31119037 2019
23
A Case of Overlap Posterior Cortical Atrophy and Logopenic Variant Primary Progressive Aphasia. 38
30817493 2019
24
Relative preservation of facial expression recognition in posterior cortical atrophy. 38
30760634 2019
25
18 F-AV-1451 uptake differs between dementia with lewy bodies and posterior cortical atrophy. 38
30615804 2019
26
Qualitative, exploratory pilot study to investigate how people living with posterior cortical atrophy, their carers and clinicians experience tests used to assess vision. 38
30898793 2019
27
[Posterior cortical atrophy-a heterogeneous syndrome : A case series]. 38
30903200 2019
28
Review: Clinical, genetic and neuroimaging features of frontotemporal dementia. 38
30582889 2019
29
Posterior Cortical Atrophy. 38
30707187 2019
30
Neuropsychological Similarities and Differences Between Amnestic Alzheimer's Disease and its Non-Amnestic Variants. 38
31156165 2019
31
Differences in hippocampal subfield volume are seen in phenotypic variants of early onset Alzheimer's disease. 38
30558867 2019
32
Neural Mechanisms of Visual Dysfunction in Posterior Cortical Atrophy. 38
31293507 2019
33
Attention network dysfunction underlies memory impairment in posterior cortical atrophy. 38
30991615 2019
34
Intrinsic connectivity networks in posterior cortical atrophy: A role for the pulvinar? 38
30528957 2019
35
Patterns of Grey Matter Atrophy at Different Stages of Parkinson's and Alzheimer's Diseases and Relation to Cognition. 38
30206799 2019
36
Posterior Cortical Atrophy. 38
30476997 2018
37
The rat model of placental ischemia as a model of postpartum posterior cortical atrophy? 38
30323132 2018
38
Tau filaments from multiple cases of sporadic and inherited Alzheimer's disease adopt a common fold. 38
30276465 2018
39
Thinner retinal layers are associated with changes in the visual pathway: A population-based study. 38
29935103 2018
40
Contributions of patient and citizen researchers to 'Am I the right way up?' study of balance in posterior cortical atrophy and typical Alzheimer's disease. 38
30373456 2018
41
Electroencephalogram findings in patients with posterior cortical atrophy. 38
30297099 2018
42
Executive dysfunction contributes to verbal encoding and retrieval deficits in posterior cortical atrophy. 38
29864594 2018
43
Exploring the contribution of visual imagery to scene construction - Evidence from Posterior Cortical Atrophy. 38
30059847 2018
44
["I can't complete my payment form anymore" : Visual disorders in posterior cortical atrophy]. 38
30083812 2018
45
Neurophysiological signatures of Alzheimer's disease and frontotemporal lobar degeneration: pathology versus phenotype. 38
30060017 2018
46
Do all visual deficits cause pure alexia? Dissociations between visual processing and reading suggest "no". 38
29902728 2018
47
18F-FIBT may expand PET for β-amyloid imaging in neurodegenerative diseases. 38
30120417 2018
48
The impact of localized grey matter damage on neighboring connectivity: posterior cortical atrophy and the visual network. 38
30145717 2018
49
[Organic Anxiety Disorder in Posterior Cortical Atrophy]. 38
29665606 2018
50
A patient with posterior cortical atrophy due to Alzheimer's disease. 38
30425798 2018

Variations for Posterior Cortical Atrophy

Expression for Posterior Cortical Atrophy

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

Pathways for Posterior Cortical Atrophy

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

# Super pathways Score Top Affiliating Genes
1 11.16 MAPT APOE
2 10.64 MAPT APOE
3 10.28 MAPT APOE

GO Terms for Posterior Cortical Atrophy

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

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

Sources for Posterior Cortical Atrophy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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