PCA
MCID: PST086
MIFTS: 37

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 58
Biparietal Alzheimer Disease 58
Benson Syndrome 58
Pca 58

Characteristics:

Orphanet epidemiological data:

58
posterior cortical atrophy
Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 58  
Rare neurological diseases


Summaries for Posterior Cortical Atrophy

MalaCards based summary : Posterior Cortical Atrophy, also known as biparietal alzheimer disease, is related to visual agnosia and alexia. 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 Alzheimers Disease. Affiliated tissues include cortex, brain and eye, and related phenotypes are ataxia and anxiety

Wikipedia : 74 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 237)
# Related Disease Score Top Affiliating Genes
1 visual agnosia 30.8 MAPT APOE
2 alexia 30.7 MAPT APOE
3 apraxia 30.7 MAPT APOE
4 aphasia 30.6 MAPT APOE
5 simultanagnosia 30.6 MAPT APOE
6 gerstmann syndrome 30.6 MAPT APOE
7 prion disease 30.2 MAPT APOE
8 nominal aphasia 30.2 MAPT APOE
9 dementia, lewy body 30.0 MAPT APOE
10 semantic dementia 30.0 MAPT APOE
11 pick disease of brain 29.7 MAPT APOE
12 amnestic disorder 29.7 MAPT APOE
13 sleep disorder 29.6 MAPT APOE
14 creutzfeldt-jakob disease 29.6 MAPT APOE
15 supranuclear palsy, progressive, 1 29.5 MAPT APOE
16 speech and communication disorders 29.3 MAPT APOE
17 amyloidosis, primary localized cutaneous, 1 11.4
18 choanal atresia, posterior 11.4
19 primary cutaneous amyloidosis 11.2
20 pain agnosia 10.9
21 alzheimer disease 10.8
22 agnosia 10.6
23 dementia 10.5
24 dysgraphia 10.5
25 corticobasal degeneration 10.5
26 agraphia 10.4
27 prostate cancer 10.4
28 dyslexia 10.4
29 ataxia and polyneuropathy, adult-onset 10.3
30 prosopagnosia 10.3
31 48,xyyy 10.3
32 cerebral atrophy 10.2
33 oculomotor apraxia 10.2
34 scoliosis 10.2
35 aneurysm 10.2
36 frontotemporal dementia 10.2
37 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 10.2
38 dyscalculia 10.2
39 ideomotor apraxia 10.2
40 allergic hypersensitivity disease 10.2
41 prostatic hyperplasia, benign 10.1
42 mucositis 10.1
43 prostatic hypertrophy 10.1
44 prostatic adenoma 10.1
45 adenocarcinoma 10.1
46 yemenite deaf-blind hypopigmentation syndrome 10.1
47 myoclonus 10.1
48 balint syndrome 10.1
49 pectus excavatum 10.1
50 moyamoya disease 1 10.1

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:

58 31 (show all 21)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ataxia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001251
2 anxiety 58 31 hallmark (90%) Very frequent (99-80%) HP:0000739
3 dysgraphia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010526
4 oculomotor apraxia 58 31 hallmark (90%) Very frequent (99-80%) HP:0000657
5 confusion 58 31 hallmark (90%) Very frequent (99-80%) HP:0001289
6 cerebral visual impairment 58 31 hallmark (90%) Very frequent (99-80%) HP:0100704
7 dyscalculia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002442
8 alexia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010523
9 finger agnosia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010525
10 photophobia 58 31 frequent (33%) Frequent (79-30%) HP:0000613
11 dyslexia 58 31 frequent (33%) Frequent (79-30%) HP:0010522
12 color vision defect 58 31 frequent (33%) Frequent (79-30%) HP:0000551
13 limb apraxia 58 31 frequent (33%) Frequent (79-30%) HP:0030217
14 memory impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0002354
15 speech apraxia 58 31 occasional (7.5%) Occasional (29-5%) HP:0011098
16 visual hallucinations 58 31 occasional (7.5%) Occasional (29-5%) HP:0002367
17 abnormal rapid eye movement sleep 58 31 occasional (7.5%) Occasional (29-5%) HP:0002494
18 inertia 58 31 occasional (7.5%) Occasional (29-5%) HP:0030216
19 language impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0002463
20 abnormality of vision 58 Frequent (79-30%)
21 agnosia 58 Very frequent (99-80%)

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) Active, not recruiting 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
5 MECHANISMS OF NEURONAL RESILIENCE IN ALZHEIMER'S DISEASE AND ITS FOCAL VARIANTS: A PET/MR STUDY Not yet recruiting NCT04150198

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:

40
Cortex, Brain, Eye, Testes, Temporal Lobe, Thyroid, Endothelial

Publications for Posterior Cortical Atrophy

Articles related to Posterior Cortical Atrophy:

(show top 50) (show all 419)
# Title Authors PMID Year
1
18F-FDG PET/CT in Sporadic Creutzfeldt-Jakob Disease. 61
32433176 2020
2
Longitudinal neuroimaging biomarkers differ across Alzheimer's disease phenotypes. 61
32572464 2020
3
Posterior cortical atrophy initially presenting as dementia with Lewy bodies. 61
31897937 2020
4
When posterior cortical atrophy invests art: a case report. 61
32378454 2020
5
Pure allocentric unilateral spatial neglect in a case of Alzheimer's disease presenting with posterior cortical atrophy syndrome. 61
31909449 2020
6
Sequences of cognitive decline in typical Alzheimer's disease and posterior cortical atrophy estimated using a novel event-based model of disease progression. 61
32489019 2020
7
Posterior Cortical Atrophy with Right Lower Egocentric Quadrantic Neglect and Lower Vertical Allocentric Neglect. 61
31942921 2020
8
Prodromal Dementia With Lewy Bodies: Clinical Characterization and Predictors of Progression. 61
32048343 2020
9
Normative brain volume reports may improve differential diagnosis of dementing neurodegenerative diseases in clinical practice. 61
32002640 2020
10
'I see colors when I touch them'. Color agnosia with visuo-tactile facilitation in a patient with posterior cortical atrophy. 61
32171092 2020
11
Pronounced Impairment of Activities of Daily Living in Posterior Cortical Atrophy. 61
32320973 2020
12
Impaired perception of simultaneous stimuli in a patient with posterior cortical atrophy: an attentional account. 61
32070200 2020
13
Word retrieval across the biomarker-confirmed Alzheimer's disease syndromic spectrum. 61
32057937 2020
14
Update on posterior cortical atrophy. 61
31688098 2020
15
Functional brain connectome in posterior cortical atrophy. 61
31865020 2020
16
Difficulty differentiating a case of posterior cortical atrophy from a psychogenic disturbance of vision. 61
30997705 2020
17
The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman. 61
31203704 2020
18
Capgras Delusion in Posterior Cortical Atrophy-A Quantitative Multimodal Imaging Single Case Study. 61
32547387 2020
19
Seeing though the clutter: A case report of Posterior Cortical Atrophy and patient-centered care. 61
31855068 2019
20
Metabolic imaging patterns in posterior cortical atrophy and Lewy body dementia. 61
31633646 2019
21
A case of ALS with posterior cortical atrophy. 61
31284766 2019
22
[Progressive supranuclear palsy-Richardson syndrome with visual attention disturbance (Holmes and Horrax) and ataxie optique (Garcin): a case report]. 61
31656263 2019
23
Disease Knowledge Transfer across Neurodegenerative Diseases. 61
32432230 2019
24
Longitudinal progression of posterior cortical atrophy over 11 years: Relationship between lesion topology and clinical deficits. 61
31322045 2019
25
[Posterior cortical atrophy-a heterogeneous syndrome : A case series]. 61
30903200 2019
26
Homonymous Hemianopia as the Initial Sign of Posterior Cortical Atrophy. 61
31612661 2019
27
The impact of localized grey matter damage on neighboring connectivity: posterior cortical atrophy and the visual network. 61
30145717 2019
28
Looking beyond the eyes: visual impairment in posterior cortical atrophy. 61
31544747 2019
29
Where words meet numbers: Comprehension of measurement unit terms in posterior cortical atrophy. 61
31095931 2019
30
Diagnostic evaluation and monitoring of patients with posterior cortical atrophy. 61
31392920 2019
31
Working memory in posterior cortical atrophy. 61
30963338 2019
32
Role of Population Receptive Field Size in Complex Visual Dysfunctions: A Posterior Cortical Atrophy Model. 61
31403655 2019
33
Clinical utility of 18F-FDG-PET/MRI brain in dementia: Preliminary experience from a geriatric clinic in South India. 61
31336358 2019
34
Retinal thickness as potential biomarker in posterior cortical atrophy and typical Alzheimer's disease. 61
31319885 2019
35
Longitudinal neuroanatomical and cognitive progression of posterior cortical atrophy. 61
31219516 2019
36
Health and social care practitioners' understanding of the problems of people with dementia-related visual processing impairment. 61
30737853 2019
37
Longitudinal progression of grey matter atrophy in non-amnestic Alzheimer's disease. 61
31135048 2019
38
The functional neuroanatomy of musical memory in Alzheimer's disease. 61
30846199 2019
39
Unusual Pattern of Reading Errors in a Patient with Posterior Cortical Atrophy. 61
31543797 2019
40
DIVE: A spatiotemporal progression model of brain pathology in neurodegenerative disorders. 61
30844504 2019
41
Reply to letter: Basis of cingulate island sign may differ in dementia with lewy bodies and posterior cortical atrophy. 61
31091363 2019
42
Dopaminergic degeneration induces early posterior cortical thinning in Parkinson's disease. 61
30408592 2019
43
Structural imaging findings on non-enhanced computed tomography are severely underreported in the primary care diagnostic work-up of subjective cognitive decline. 61
30656357 2019
44
Alzheimer's Disease Including Focal Presentations. 61
30925614 2019
45
Detection and localisation of hesitant steps in people with Alzheimer's disease navigating routes of varying complexity. 61
31119037 2019
46
A Case of Overlap Posterior Cortical Atrophy and Logopenic Variant Primary Progressive Aphasia. 61
30817493 2019
47
Relative preservation of facial expression recognition in posterior cortical atrophy. 61
30760634 2019
48
18 F-AV-1451 uptake differs between dementia with lewy bodies and posterior cortical atrophy. 61
30615804 2019
49
Qualitative, exploratory pilot study to investigate how people living with posterior cortical atrophy, their carers and clinicians experience tests used to assess vision. 61
30898793 2019
50
Review: Clinical, genetic and neuroimaging features of frontotemporal dementia. 61
30582889 2019

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 11.13 MAPT APOE
3 10.28 MAPT APOE

GO Terms for Posterior Cortical Atrophy

Biological processes related to Posterior Cortical Atrophy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neuron projection development GO:0031175 8.62 MAPT APOE

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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