PCA
MCID: PST086
MIFTS: 32

Posterior Cortical Atrophy (PCA) malady

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Posterior Cortical Atrophy

Aliases & Descriptions for Posterior Cortical Atrophy:

Name: Posterior Cortical Atrophy 56
Biparietal Alzheimer Disease 56
Benson Syndrome 56
Pca 56

Characteristics:

Orphanet epidemiological data:

56
posterior cortical atrophy
Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Orphanet 56 ORPHA54247
ICD10 via Orphanet 34 G31.1

Summaries for Posterior Cortical Atrophy

MalaCards based summary : Posterior Cortical Atrophy, also known as biparietal alzheimer disease, is related to amyloidosis, primary localized cutaneous, 1 and choanal atresia, posterior. An important gene associated with Posterior Cortical Atrophy is APOE (Apolipoprotein E), and among its related pathways/superpathways are Neuroscience and Reelin Pathway (Cajal-Retzius cells). The drugs Cysteamine and Acetylcysteine have been mentioned in the context of this disorder. Affiliated tissues include cortex, brain and temporal lobe, and related phenotype is taste/olfaction.

Wikipedia : 71 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 all 43)
id Related Disease Score Top Affiliating Genes
1 amyloidosis, primary localized cutaneous, 1 10.8
2 choanal atresia, posterior 10.8
3 primary cutaneous amyloidosis 10.8
4 prostatitis 10.3
5 prostate cancer 10.3
6 dementia 10.2
7 alzheimer disease 10.0
8 alexia 10.0
9 keratosis palmoplantaris striata 10.0 APOE MAPT
10 severe congenital nemaline myopathy 10.0 APOE MAPT
11 pulmonary systemic sclerosis 10.0 APOE MAPT
12 bone dysplasia azouz type 10.0 APOE MAPT
13 meckel syndrome 10 10.0 APOE MAPT
14 autoimmune disease of urogenital tract 10.0 APOE MAPT
15 meier-gorlin syndrome 5 9.9 APOE MAPT
16 autotopagnosia 9.9 APOE MAPT
17 contagious pustular dermatitis 9.9 APOE MAPT
18 coenzyme q10 deficiency, primary, 1 9.9 APOE MAPT
19 acne inversa, familial, 3 9.9 APOE MAPT
20 prion disease with protracted course 9.9 APOE MAPT
21 angina pectoris 9.9 APOE MAPT
22 simultanagnosia 9.9
23 ichthyosis lamellar 2 9.9 APOE MAPT
24 cardiomyopathy, dilated, 1u 9.9 APOE MAPT
25 moyamoya disease 9.9
26 allergic bronchopulmonary aspergillosis, familial 9.9 APOE MAPT
27 personality disorder 9.8 APOE MAPT
28 epithelioid type angiomyolipoma 9.8 APOE MAPT
29 agnosia 9.8
30 agraphia 9.8
31 eumycotic mycetoma 9.8 APOE MAPT
32 substance-induced psychosis 9.7 APOE MAPT
33 corneal dystrophy 9.7
34 cerebritis 9.7
35 dysgraphia 9.7
36 temporal lobe epilepsy 9.7
37 corticobasal degeneration 9.7
38 endotheliitis 9.7
39 aphasia 9.7
40 visual agnosia 9.7
41 parametritis 9.7
42 epilepsy 9.7
43 anxiety disorder 9.7

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



Diseases related to Posterior Cortical Atrophy

Symptoms & Phenotypes for Posterior Cortical Atrophy

MGI Mouse Phenotypes related to Posterior Cortical Atrophy:

44
id 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):

(show all 14)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cysteamine Approved, Investigational Phase 4 60-23-1 6058
2
Acetylcysteine Approved, Investigational Phase 4 616-91-1 12035
3
Choline Approved, Nutraceutical Phase 4 62-49-7 305
4 N-monoacetylcystine Phase 4
5 Expectorants Phase 4
6 Anti-Infective Agents Phase 4
7 Protective Agents Phase 4
8 Antidotes Phase 4
9 Respiratory System Agents Phase 4
10 Antiviral Agents Phase 4
11 Antioxidants Phase 4
12 Cytidine Diphosphate Choline Phase 4
13 Nootropic Agents Phase 4
14
Corticosterone Phase 1 50-22-6 5753

Interventional clinical trials:

(show all 12)
id Name Status NCT ID Phase
1 Cystagon to Treat Infantile Neuronal Ceroid Lipofuscinosis Completed NCT00028262 Phase 4
2 Citicoline Effect on Non-arteritic Anterior Ischemic Optic Neuropathy (NAION) Recruiting NCT03046693 Phase 4
3 Tau Brain Imaging in Typical and Atypical Alzheimer's Disease (AD) Recruiting NCT03022968 Phase 2
4 Amyloid-related Imaging Abnormalities (Microbleeds) in Atypical AD Completed NCT01723553 Phase 1
5 Buccal Fat Pad Derived Stem Cells With Cortical Tenting in Posterior Mandible Reconstruction Recruiting NCT02745366 Phase 1
6 Influence of Age on Amyloid Load in Alzheimer's Disease and in Atypical Focal Cortical Alzheimer's Disease Completed NCT01095744
7 PET Imaging in Chronic Traumatic Encephalopathy Completed NCT02211820
8 Emotional Communication Disorders in Cerebellar Disease Completed NCT02106819
9 Molecular and Structural Imaging in Atypical Alzheimer's Disease: A Longitudinal Study Recruiting NCT02740634
10 Early-onset Alzheimer's Disease Phenotypes: Neuropsychology and Neural Networks Recruiting NCT03153371
11 Tau Imaging in Young Onset Dementia Active, not recruiting NCT02289118
12 Plasticity of Grey and White Matter in Response to Motor Skill Training in Healthy Individuals and Those With Spinal Cord Injury Active, not recruiting NCT02148887

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:

39
Cortex, Brain, Temporal Lobe, Testes, Endothelial

Publications for Posterior Cortical Atrophy

Articles related to Posterior Cortical Atrophy:

(show top 50) (show all 168)
id Title Authors Year
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
A novel use of arterial spin labelling MRI to demonstrate focal hypoperfusion in individuals with posterior cortical atrophy: a multimodal imaging study. ( 26733599 )
2016
4
Posterior cortical atrophy as a primary clinical phenotype of corticobasal syndrome with a progranulin gene rs5848 TT genotype. ( 26852323 )
2016
5
Neuro-cognitive mechanisms of simultanagnosia in patients with posterior cortical atrophy. ( 27702740 )
2016
6
Memory Impairment at Initial Clinical Presentation in Posterior Cortical Atrophy. ( 27128371 )
2016
7
Functional Connectivity of Ventral and Dorsal Visual Streams in Posterior Cortical Atrophy. ( 26923019 )
2016
8
18F-FDG PET in Posterior Cortical Atrophy and Dementia with Lewy Bodies. ( 27688479 )
2016
9
Can patients without early, prominent visual deficits still be diagnosed of posterior cortical atrophy? ( 27423559 )
2016
10
Clinical and neuroimaging differences between posterior cortical atrophy and typical amnestic Alzheimer's disease patients at an early disease stage. ( 27377199 )
2016
11
Genetic risk factors for the posterior cortical atrophy variant of Alzheimer's disease. ( 26993346 )
2016
12
Saccades abnormalities in posterior cortical atrophy - A case report. ( 28063320 )
2016
13
Non-pharmacological intervention for posterior cortical atrophy. ( 27574605 )
2016
14
Distinct patterns of increased translocator protein in posterior cortical atrophy and amnestic Alzheimer's disease. ( 28068564 )
2016
15
Effect of age at onset on cortical thickness and cognition in posterior cortical atrophy. ( 27318138 )
2016
16
Neuropsychiatric Symptoms in Posterior Cortical Atrophy and Alzheimer Disease. ( 26404166 )
2015
17
Evaluating pathogenic dementia variants in posterior cortical atrophy. ( 26507310 )
2015
18
Posterior cortical atrophy: an atypical variant of Alzheimer disease. ( 25998111 )
2015
19
3D Shape Perception in Posterior Cortical Atrophy: A Visual Neuroscience Perspective. ( 26377458 )
2015
20
Usefulness of Multi-Parametric MRI for the Investigation of Posterior Cortical Atrophy. ( 26480294 )
2015
21
Functional neural substrates of posterior cortical atrophy patients. ( 25976028 )
2015
22
Abnormalities of fixation, saccade and pursuit in posterior cortical atrophy. ( 25895507 )
2015
23
Derailed dementia: Alzheimer's disease versus posterior cortical atrophy. ( 25688631 )
2015
24
Reduced modulation of scanpaths in response to task demands in posterior cortical atrophy. ( 25596316 )
2015
25
Posterior Cortical Atrophy: A Case Report of a 6-Year Natural Progression. ( 26704219 )
2015
26
A Pilot Study on Clinical and Neuroimaging Characteristics of Chinese Posterior Cortical Atrophy: Comparison with Typical Alzheimer's Disease. ( 26267071 )
2015
27
Left temporal lobe epilepsy revealing left posterior cortical atrophy due to Alzheimer's disease. ( 25589715 )
2015
28
Facilitating text reading in posterior cortical atrophy. ( 26138948 )
2015
29
Clinical, FDG and amyloid PET imaging in posterior cortical atrophy. ( 25862483 )
2015
30
Differential hippocampal shapes in posterior cortical atrophy patients: A comparison with control and typical AD subjects. ( 26461053 )
2015
31
Posterior cortical atrophy as an extreme phenotype of GRN mutations. ( 25546130 )
2015
32
Tau, amyloid, and hypometabolism in a patient with posterior cortical atrophy. ( 25448043 )
2015
33
Oculomotor abnormalities in posterior cortical atrophy: are they different from those in Alzheimer's disease after all? ( 26106096 )
2015
34
Posterior Cortical Atrophy Presenting with Superior Arcuate Field Defect. ( 26417467 )
2015
35
Treatment of a patient with posterior cortical atrophy (PCA) with chiropractic manipulation and Dynamic Neuromuscular Stabilization (DNS): A case report. ( 25729084 )
2015
36
Visual field defect as an uncommon presentation of posterior cortical atrophy. ( 25327905 )
2014
37
(Con)text-specific effects of visual dysfunction on reading in posterior cortical atrophy. ( 24841985 )
2014
38
Hiding in plain sight: a closer look at posterior cortical atrophy. ( 25216669 )
2014
39
Pronounced Impairment of Everyday Skills and Self-Care in Posterior Cortical Atrophy. ( 25096622 )
2014
40
Counting or chunking? Mathematical and heuristic abilities in patients with corticobasal syndrome and posterior cortical atrophy. ( 25278132 )
2014
41
Late-onset Alzheimer disease genetic variants in posterior cortical atrophy and posterior AD. ( 24670887 )
2014
42
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
43
Quantitative regional validation of the visual rating scale for posterior cortical atrophy. ( 24092044 )
2014
44
First Report of PSEN2 Mutation Presenting as Posterior Cortical Atrophy. ( 25010293 )
2014
45
Further evidence about the crucial role of CSF biomarkers in diagnosis of posterior cortical atrophy. ( 24487699 )
2014
46
Differential processing of natural scenes in posterior cortical atrophy and in Alzheimer's disease, as measured with a saccade choice task. ( 25120440 )
2014
47
Diagnosis of posterior cortical atrophy delayed by coexisting Fuchs' Endothelial Corneal Dystrophy. ( 24667904 )
2014
48
Phenotypical variation in Alzheimer's disease: insights from posterior cortical atrophy. ( 25239627 )
2014
49
Brain Changes within the Visuo-Spatial Attentional Network in Posterior Cortical Atrophy. ( 25114070 )
2014
50
The Neuropsychiatric Profile of Posterior Cortical Atrophy. ( 25330926 )
2014

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:

id Super pathways Score Top Affiliating Genes
1 11.67 APOE MAPT
2 10.96 APOE MAPT
3 10.28 APOE MAPT

GO Terms for Posterior Cortical Atrophy

Cellular components related to Posterior Cortical Atrophy according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 dendrite GO:0030425 8.96 APOE MAPT
2 neuronal cell body GO:0043025 8.62 APOE MAPT

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

id 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
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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