Posterior cortical atrophy
Synonyms: Benson syndrome · Biparietal Alzheimer disease · PCA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
A rare neurologic disease characterized by impairment of higher visual processing skills and other posterior cortical functions without any evidence of ocular abnormalities, relatively intact memory and language in the early stages, and atrophy of posterior brain regions.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Posterior cortical atrophy | Benson syndrome · Biparietal Alzheimer disease · PCA |
| fr | Atrophie corticale postérieure | PCA · Syndrome de Benson |
| de | Atrophie, kortikale posteriore | Alzheimer-Krankheit, biparietale · Benson-Syndrom · PCA |
| es | Atrofia cortical posterior | Enfermedad de Alzheimer biparietal · PCA · Síndrome de Benson |
| it | Atrofia corticale posteriore | Malattia di Alzheimer biparietale · PCA · Sindrome di Benson |
| nl | Posterieure corticale atrofie | Bipariëtale ziekte van Alzheimer · PCA · Syndroom van Benson |
| pt | Atrofia cortical posterior | Síndrome Benson · PCA |
| pl | Tylna atrofia kory | Dwuciemieniowa choroba Alzheimera · PCA · Zespół Bensona |
| cs | Posteriorní kortikální atrofie | Biparietální Alzheimerova nemoc · PCA · Bensonův syndrom |
| uk | Задня кортикальна атрофія | Синдром Бенсона · Біпарієтальна хвороба Альцгеймера · PCA |
| zh | 后皮质萎缩 | 二顶骨阿尔茨海默病 |
| tr | Posterior kortikal atrofi | PCA · Benson sendromu · Biparietal Alzheimer hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G31.1 | NTBT | attributed | validated |
| ICD-11 | 8A21.0 | E | specific | validated |
| MONDO | 0018899 | E | validated | |
| UMLS | C4275079 | E | validated | |
| MedDRA | 10078207 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:276058Genetic neurodegenerative disease with dementia
rare genetic diseases - ORPHA:98534Neurodegenerative disease with dementia
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
21 annotations · validated 2018-01-16| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010524 | Agnosia | |
| HP:0010523 | Alexia | |
| HP:0000739 | Anxiety | |
| HP:0001251 | Ataxia | |
| HP:0100704 | Cerebral visual impairment | |
| HP:0001289 | Confusion | |
| HP:0002442 | Dyscalculia | |
| HP:0010526 | Dysgraphia | |
| HP:0010525 | Finger agnosia | |
| HP:0000657 | Oculomotor apraxia | |
| Frequent (79-30%) | ||
| HP:0000504 | Abnormality of vision | |
| HP:0000551 | Color vision defect | |
| HP:0010522 | Dyslexia | |
| HP:0030217 | Limb apraxia | |
| HP:0000613 | Photophobia | |
| Occasional (29-5%) | ||
| HP:0002494 | Abnormal rapid eye movement sleep | |
| HP:0030216 | Inertia | |
| HP:0002463 | Language impairment | |
| HP:0002354 | Memory impairment | |
| HP:0011098 | Speech apraxia | |
| HP:0002367 | Visual hallucinations | |
Run the differential on the 10 obligate / very frequent terms
Annotation source: 3740301[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/54247
- This record as JSON: /data/d/54247.json