MCID: PST086
MIFTS: 41

Posterior Cortical Atrophy

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


Summaries for Posterior Cortical Atrophy

MalaCards based summary : Posterior Cortical Atrophy, also known as biparietal alzheimer disease, is related to simultanagnosia and semantic dementia. An important gene associated with Posterior Cortical Atrophy is APOE (Apolipoprotein E), and among its related pathways/superpathways are G-protein signaling_Regulation of RAC1 activity and Alzheimers Disease Pathway. The drug Corticosterone has been mentioned in the context of this disorder. Affiliated tissues include cortex, brain and eye, and related phenotypes are oculomotor apraxia and anxiety

Wikipedia : 76 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 51)
# Related Disease Score Top Affiliating Genes
1 simultanagnosia 30.1 APOE MAPT
2 semantic dementia 29.7 APOE MAPT
3 alzheimer disease 2 29.7 APOE MAPT
4 aphasia 29.6 APOE MAPT
5 frontotemporal dementia 28.6 APOE MAPT
6 amyloidosis, primary localized cutaneous, 1 11.0
7 choanal atresia, posterior 10.9
8 primary cutaneous amyloidosis 10.9
9 alzheimer disease 10.5
10 prostatitis 10.4
11 prostate cancer 10.3
12 dementia 10.3
13 alexia 10.1
14 familial idiopathic basal ganglia calcification 10.1 APOE MAPT
15 communicating hydrocephalus 10.1 APOE MAPT
16 hydrocephalus, normal-pressure 10.0 APOE MAPT
17 binswanger's disease 10.0 APOE MAPT
18 brain injury 10.0 APOE MAPT
19 cerebral amyloid angiopathy, cst3-related 10.0 APOE MAPT
20 pick disease of brain 10.0 APOE MAPT
21 supranuclear palsy, progressive, 1 10.0 APOE MAPT
22 inclusion body myositis 9.9 APOE MAPT
23 vascular dementia 9.9 APOE MAPT
24 dementia, lewy body 9.9 APOE MAPT
25 aging 9.9
26 agnosia 9.9
27 agraphia 9.9
28 speech and communication disorders 9.9 APOE MAPT
29 disease of mental health 9.8 APOE MAPT
30 creutzfeldt-jakob disease 9.8 APOE MAPT
31 parametritis 9.7
32 gilles de la tourette syndrome 9.7
33 down syndrome 9.7
34 epilepsy 9.7
35 corneal dystrophy 9.7
36 anxiety 9.7
37 cerebritis 9.7
38 alzheimer disease 19 9.7
39 dysgraphia 9.7
40 temporal lobe epilepsy 9.7
41 thyroiditis 9.7
42 learning disability 9.7
43 corticobasal degeneration 9.7
44 visual agnosia 9.7
45 depression 9.7
46 frontotemporal dementia with parkinsonism-17 9.7
47 fuchs' endothelial dystrophy 9.7
48 endotheliitis 9.7
49 central nervous system disease 9.7 APOE MAPT
50 nervous system disease 9.7 APOE MAPT

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

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

Drugs for Posterior Cortical Atrophy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Corticosterone Experimental Phase 1 50-22-6 5753

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Tau Brain Imaging in Typical and Atypical Alzheimer's Disease (AD) Recruiting NCT03022968 Phase 2 [18F]T807 PET
2 Amyloid-related Imaging Abnormalities (Microbleeds) in Atypical AD Completed NCT01723553 Phase 1 C-11 PiB
3 Influence of Age on Amyloid Load in Alzheimer's Disease and in Atypical Focal Cortical Alzheimer's Disease Completed NCT01095744
4 Molecular and Structural Imaging in Atypical Alzheimer's Disease: A Longitudinal Study Recruiting NCT02740634 Not Applicable F-18 AV 1451;C-11 PiB
5 Early-onset Alzheimer's Disease Phenotypes: Neuropsychology and Neural Networks Recruiting NCT03153371
6 Tau Imaging in Young Onset Dementia Active, not recruiting NCT02289118 Not Applicable [18F]-T807 imaging tracer

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, Temporal Lobe, Endothelial, Thyroid, Testes

Publications for Posterior Cortical Atrophy

Articles related to Posterior Cortical Atrophy:

(show top 50) (show all 198)
# Title Authors Year
1
Case of posterior cortical atrophy (PCA) evolved to PCA-CBS. ( 29914902 )
2018
2
Prevalence of Mathematical and Visuospatial Learning Disabilities in Patients With Posterior Cortical Atrophy. ( 29630699 )
2018
3
Posterior cortical atrophy: a single case cognitive and radiological follow-up. ( 29308699 )
2018
4
Navigational cue effects in Alzheimer's disease and posterior cortical atrophy. ( 29928653 )
2018
5
Cognitive Rehabilitation and Transcranial Direct Current Stimulation in a Patient with Posterior Cortical Atrophy: An fMRI Study. ( 29925828 )
2018
6
Clinical significance of cognitive rehabilitation and psychoeducational intervention for family caregivers of patients with posterior cortical atrophy: a longitudinal study. ( 29372599 )
2018
7
Methylphenidate Treatment for Patients With Posterior Cortical Atrophy. ( 29685063 )
2018
8
Executive dysfunction contributes to verbal encoding and retrieval deficits in posterior cortical atrophy. ( 29864594 )
2018
9
Differentiation of neuropsychological features between posterior cortical atrophy and early onset Alzheimer's disease. ( 29747584 )
2018
10
Association between precuneus volume and autobiographical memory impairment in posterior cortical atrophy: Beyond the visual syndrome. ( 29876268 )
2018
11
Down syndrome with posterior cortical atrophy. ( 29437810 )
2018
12
Posterior cortical atrophy variant of Alzheimer's dementia-A case report. ( 29864740 )
2018
13
Posterior cortical atrophy: from vision to emotion. ( 29569567 )
2018
14
'Because my brain isn't as active as it should be, my eyes don't always see': a qualitative exploration of the stress process for those living with posterior cortical atrophy. ( 29439072 )
2018
15
A double-blind placebo-controlled cross-over clinical trial of DONepezil In Posterior cortical atrophy due to underlying Alzheimer's Disease: DONIPAD study. ( 29716655 )
2018
16
The Nature and Natural History of Posterior Cortical Atrophy Syndrome: A Variant of Early-onset Alzheimer Disease. ( 28796011 )
2017
17
Homonymous Hemianopsia Due to Posterior Cortical Atrophy. ( 28512507 )
2017
18
A case of posterior cortical atrophy with vertical neglect. ( 28387593 )
2017
19
Lateralized occipital degeneration in posterior cortical atrophy predicts visual field deficits. ( 28180083 )
2017
20
Consensus classification of posterior cortical atrophy. ( 28259709 )
2017
21
Progression of oculomotor deficit in a patient with posterior cortical atrophy. ( 27816183 )
2017
22
Statokinetic Dissociation (Riddoch Phenomenon) in a Patient with Homonymous Hemianopsia as the First Sign of Posterior Cortical Atrophy. ( 29422846 )
2017
23
Tau-PET Binding Distinguishes Patients With Early-stage Posterior Cortical Atrophy From Amnestic Alzheimer Disease Dementia. ( 28394771 )
2017
24
Semantic word category processing in semantic dementia and posterior cortical atrophy. ( 28624681 )
2017
25
Association between low thyroid-stimulating hormone, posterior cortical atrophy and nitro-oxidative stress in elderly patients with cognitive dysfunction. ( 28883858 )
2017
26
Posterior cortical atrophy: the value of neuroimaging in assessing memory loss. ( 29312879 )
2017
27
Posterior cortical atrophy of a suspected non-Alzheimer type: a case report. ( 29072103 )
2017
28
Homonymous hemianopia as the first sign of posterior cortical atrophy. ( 28131221 )
2017
29
The oral spelling profile of posterior cortical atrophy and the nature of the graphemic representation. ( 27913156 )
2017
30
Visual Dysfunction in Posterior Cortical Atrophy. ( 28861031 )
2017
31
F-18 FDG and F-18 Tau PET in posterior cortical atrophy. ( 28584971 )
2017
32
Posterior cortical atrophy as a primary clinical phenotype of corticobasal syndrome with a progranulin gene rs5848 TT genotype. ( 26852323 )
2016
33
Genetic risk factors for the posterior cortical atrophy variant of Alzheimer's disease. ( 26993346 )
2016
34
18F-FDG PET in Posterior Cortical Atrophy and Dementia with Lewy Bodies. ( 27688479 )
2016
35
Clinical and neuroimaging differences between posterior cortical atrophy and typical amnestic Alzheimer's disease patients at an early disease stage. ( 27377199 )
2016
36
Effect of age at onset on cortical thickness and cognition in posterior cortical atrophy. ( 27318138 )
2016
37
Non-pharmacological intervention for posterior cortical atrophy. ( 27574605 )
2016
38
Saccades abnormalities in posterior cortical atrophy - A case report. ( 28063320 )
2016
39
Neuro-cognitive mechanisms of simultanagnosia in patients with posterior cortical atrophy. ( 27702740 )
2016
40
Memory Impairment at Initial Clinical Presentation in Posterior Cortical Atrophy. ( 27128371 )
2016
41
Distinct patterns of increased translocator protein in posterior cortical atrophy and amnestic Alzheimer's disease. ( 28068564 )
2016
42
Functional Connectivity of Ventral and Dorsal Visual Streams in Posterior Cortical Atrophy. ( 26923019 )
2016
43
A novel use of arterial spin labelling MRI to demonstrate focal hypoperfusion in individuals with posterior cortical atrophy: a multimodal imaging study. ( 26733599 )
2016
44
Can patients without early, prominent visual deficits still be diagnosed of posterior cortical atrophy? ( 27423559 )
2016
45
Posterior Cortical Atrophy: A Case Report of a 6-Year Natural Progression. ( 26704219 )
2015
46
Neuropsychiatric Symptoms in Posterior Cortical Atrophy and Alzheimer Disease. ( 26404166 )
2015
47
Usefulness of Multi-Parametric MRI for the Investigation of Posterior Cortical Atrophy. ( 26480294 )
2015
48
Posterior cortical atrophy: an atypical variant of Alzheimer disease. ( 25998111 )
2015
49
Posterior cortical atrophy as an extreme phenotype of GRN mutations. ( 25546130 )
2015
50
Facilitating text reading in posterior cortical atrophy. ( 26138948 )
2015

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 10.64 APOE MAPT
2 10.28 APOE MAPT

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 APOE MAPT

Sources for Posterior Cortical Atrophy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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