PCA
MCID: PST086
MIFTS: 36

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 Pathway. Affiliated tissues include brain, cortex and prostate, 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 240)
# Related Disease Score Top Affiliating Genes
1 visual agnosia 30.8 MAPT APOE
2 alexia 30.8 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 30.3 MAPT APOE
8 nominal aphasia 30.2 MAPT APOE
9 dementia, lewy body 30.1 MAPT APOE
10 semantic dementia 30.0 MAPT APOE
11 pick disease of brain 29.8 MAPT APOE
12 creutzfeldt-jakob disease 29.8 MAPT APOE
13 supranuclear palsy, progressive, 1 29.5 MAPT APOE
14 speech and communication disorders 29.4 MAPT APOE
15 amyloidosis, primary localized cutaneous, 1 11.4
16 choanal atresia, posterior 11.4
17 primary cutaneous amyloidosis 11.2
18 pain agnosia 10.9
19 alzheimer disease 10.8
20 agnosia 10.6
21 dementia 10.5
22 dysgraphia 10.5
23 corticobasal degeneration 10.5
24 agraphia 10.4
25 prostate cancer 10.4
26 dyslexia 10.4
27 ataxia and polyneuropathy, adult-onset 10.3
28 prosopagnosia 10.3
29 48,xyyy 10.3
30 aneurysm 10.2
31 cerebral atrophy 10.2
32 oculomotor apraxia 10.2
33 scoliosis 10.2
34 frontotemporal dementia 10.2
35 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 10.2
36 dyscalculia 10.2
37 ideomotor apraxia 10.2
38 allergic hypersensitivity disease 10.2
39 yemenite deaf-blind hypopigmentation syndrome 10.1
40 myoclonus 10.1
41 balint syndrome 10.1
42 prostatic hyperplasia, benign 10.1
43 mucositis 10.1
44 prostatic hypertrophy 10.1
45 prostatic adenoma 10.1
46 adenocarcinoma 10.1
47 pectus excavatum 10.0
48 moyamoya disease 1 10.0
49 constipation 10.0
50 osteoarthritis 10.0

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 22)
# 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 confusion 58 31 hallmark (90%) Very frequent (99-80%) HP:0001289
4 dysgraphia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010526
5 oculomotor apraxia 58 31 hallmark (90%) Very frequent (99-80%) HP:0000657
6 finger agnosia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010525
7 cerebral visual impairment 58 31 hallmark (90%) Very frequent (99-80%) HP:0100704
8 dyscalculia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002442
9 alexia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010523
10 photophobia 58 31 frequent (33%) Frequent (79-30%) HP:0000613
11 dyslexia 58 31 frequent (33%) Frequent (79-30%) HP:0010522
12 limb apraxia 58 31 frequent (33%) Frequent (79-30%) HP:0030217
13 color vision defect 31 frequent (33%) HP:0000551
14 memory impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0002354
15 visual hallucinations 58 31 occasional (7.5%) Occasional (29-5%) HP:0002367
16 abnormal rapid eye movement sleep 58 31 occasional (7.5%) Occasional (29-5%) HP:0002494
17 speech apraxia 58 31 occasional (7.5%) Occasional (29-5%) HP:0011098
18 language impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0002463
19 inertia 58 31 occasional (7.5%) Occasional (29-5%) HP:0030216
20 abnormality of vision 58 Frequent (79-30%)
21 abnormality of color vision 58 Frequent (79-30%)
22 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
Brain, Cortex, Prostate, Eye, Bone, Temporal Lobe, Endothelial

Publications for Posterior Cortical Atrophy

Articles related to Posterior Cortical Atrophy:

(show top 50) (show all 408)
# Title Authors PMID Year
1
Update on posterior cortical atrophy. 61
31688098 2020
2
Posterior cortical atrophy initially presenting as dementia with Lewy bodies. 61
31897937 2020
3
Normative brain volume reports may improve differential diagnosis of dementing neurodegenerative diseases in clinical practice. 61
32002640 2020
4
Difficulty differentiating a case of posterior cortical atrophy from a psychogenic disturbance of vision. 61
30997705 2020
5
Posterior Cortical Atrophy with Right Lower Egocentric Quadrantic Neglect and Lower Vertical Allocentric Neglect. 61
31942921 2020
6
The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman. 61
31203704 2020
7
Pure allocentric unilateral spatial neglect in a case of Alzheimer's disease presenting with posterior cortical atrophy syndrome. 61
31909449 2020
8
Seeing though the clutter: A case report of Posterior Cortical Atrophy and patient-centered care. 61
31855068 2019
9
Metabolic imaging patterns in posterior cortical atrophy and Lewy body dementia. 61
31633646 2019
10
A case of ALS with posterior cortical atrophy. 61
31284766 2019
11
[Progressive supranuclear palsy-Richardson syndrome with visual attention disturbance (Holmes and Horrax) and ataxie optique (Garcin): a case report]. 61
31656263 2019
12
Functional brain connectome in posterior cortical atrophy. 61
31865020 2019
13
The impact of localized grey matter damage on neighboring connectivity: posterior cortical atrophy and the visual network. 61
30145717 2019
14
Longitudinal progression of posterior cortical atrophy over 11 years: Relationship between lesion topology and clinical deficits. 61
31322045 2019
15
[Posterior cortical atrophy-a heterogeneous syndrome : A case series]. 61
30903200 2019
16
Homonymous Hemianopia as the Initial Sign of Posterior Cortical Atrophy. 61
31612661 2019
17
Looking beyond the eyes: visual impairment in posterior cortical atrophy. 61
31544747 2019
18
Diagnostic evaluation and monitoring of patients with posterior cortical atrophy. 61
31392920 2019
19
Where words meet numbers: Comprehension of measurement unit terms in posterior cortical atrophy. 61
31095931 2019
20
Working memory in posterior cortical atrophy. 61
30963338 2019
21
Role of Population Receptive Field Size in Complex Visual Dysfunctions: A Posterior Cortical Atrophy Model. 61
31403655 2019
22
Clinical utility of 18F-FDG-PET/MRI brain in dementia: Preliminary experience from a geriatric clinic in South India. 61
31336358 2019
23
Retinal thickness as potential biomarker in posterior cortical atrophy and typical Alzheimer's disease. 61
31319885 2019
24
Longitudinal neuroanatomical and cognitive progression of posterior cortical atrophy. 61
31219516 2019
25
Health and social care practitioners' understanding of the problems of people with dementia-related visual processing impairment. 61
30737853 2019
26
The functional neuroanatomy of musical memory in Alzheimer's disease. 61
30846199 2019
27
Longitudinal progression of grey matter atrophy in non-amnestic Alzheimer's disease. 61
31135048 2019
28
Unusual Pattern of Reading Errors in a Patient with Posterior Cortical Atrophy. 61
31543797 2019
29
DIVE: A spatiotemporal progression model of brain pathology in neurodegenerative disorders. 61
30844504 2019
30
Reply to letter: Basis of cingulate island sign may differ in dementia with lewy bodies and posterior cortical atrophy. 61
31091363 2019
31
Dopaminergic degeneration induces early posterior cortical thinning in Parkinson's disease. 61
30408592 2019
32
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
33
Detection and localisation of hesitant steps in people with Alzheimer's disease navigating routes of varying complexity. 61
31119037 2019
34
Alzheimer's Disease Including Focal Presentations. 61
30925614 2019
35
A Case of Overlap Posterior Cortical Atrophy and Logopenic Variant Primary Progressive Aphasia. 61
30817493 2019
36
Relative preservation of facial expression recognition in posterior cortical atrophy. 61
30760634 2019
37
18 F-AV-1451 uptake differs between dementia with lewy bodies and posterior cortical atrophy. 61
30615804 2019
38
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
39
Review: Clinical, genetic and neuroimaging features of frontotemporal dementia. 61
30582889 2019
40
Posterior Cortical Atrophy. 61
30707187 2019
41
Visual cognition in non-amnestic Alzheimer's disease: Relations to tau, amyloid, and cortical atrophy. 61
31200149 2019
42
Differences in hippocampal subfield volume are seen in phenotypic variants of early onset Alzheimer's disease. 61
30558867 2019
43
Does the Right Focal Variant of Alzheimer's Disease Really Exist? A Literature Analysis. 61
31381515 2019
44
Neuropsychological Similarities and Differences Between Amnestic Alzheimer's Disease and its Non-Amnestic Variants. 61
31156165 2019
45
Neuroimaging in aging and neurologic diseases. 61
31753134 2019
46
Patterns of Grey Matter Atrophy at Different Stages of Parkinson's and Alzheimer's Diseases and Relation to Cognition. 61
30206799 2019
47
ReadClear: An Assistive Reading Tool for People Living with Posterior Cortical Atrophy. 61
31524161 2019
48
Intrinsic connectivity networks in posterior cortical atrophy: A role for the pulvinar? 61
30528957 2019
49
Posterior Cortical Atrophy: Does Complaint Match the Impairment? A Neuropsychological and FDG-PET Study. 61
31616363 2019
50
Attention network dysfunction underlies memory impairment in posterior cortical atrophy. 61
30991615 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 10.96 MAPT APOE
2 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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