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posterior cortical atrophy - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

posterior cortical atrophy

MONDO:0018899

Also known as:Benson syndromePCAbiparietal Alzheimer disease

Find Clinical TrialsTrials

Overview

Posterior Cortical Atrophy (PCA) is a rare progressive neurodegenerative disorder with a typical onset between 50-65 years of age characterized by progressive impairment of higher visual processing skills and other posterior cortical functions without any evidence of ocular abnormalities.

Clinical Trials7 registered · 4 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Oculomotor apraxia, Anxiety, Ataxia, and Confusion and others; and common findings: Abnormality of vision, Color vision defect, Photophobia, and Dyslexia and others. 21 total HPO annotations.

Organ System	Phenotype Count	Example Features
Brain and nerves	6	Anxiety, Ataxia, Cerebral visual impairment

Diagnosis

Biomarker and diagnostic research for posterior cortical atrophy has been reported in the published literature.

Prognosis

Phenotype severity distribution: 10 very common features, 5 common features.

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

7 clinical trials registered, 4 recruiting. Interventions under study include medical devices, drug therapy, other interventions, and procedural interventions. Pipeline includes 1 PHASE2, 5 NA. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT02740634](https://clinicaltrials.gov/study/NCT02740634)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 11:53 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:54247

GARD

Genetic and Rare Diseases Info Center

GARD:18846

Questions for Your Care Team

→Are any of the 7 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Laboratory research	9	33%
Research summaries	6	22%
Patient case studies	5	19%
Disease patterns and progression	5	19%
Testing and diagnosis research	2	7%

Kisho

posterior cortical atrophy

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Multi-case review of posterior cortical atrophy in a neuropsychology clinic.

Neurofibrillary tangle distribution in posterior cortical atrophy and typical early-onset Alzheimer's Disease.

posterior cortical atrophy

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Multi-case review of posterior cortical atrophy in a neuropsychology clinic.

Neurofibrillary tangle distribution in posterior cortical atrophy and typical early-onset Alzheimer's Disease.