Cerebral proliferative angiopathy
Synonyms: CPA · Diffuse nidus arteriovenous malformation · Holohemispheric giant cerebral arteriovenous malformation · Proliferative angiopathy
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare arteriovenous malformation characterized by seizures, disabling headaches, and stroke-like symptoms with progressive neurological deficits due to a proliferative response to chronic parenchymal ischaemia. Normal brain parenchyma is interspersed throughout the vascular malformation. Hemorrhagic presentations are uncommon; however, when present, the risk of reccurence is higher in such patients than in those with classic cerebral arteriovenous malformation.
Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Cerebral proliferative angiopathy | CPA · Diffuse nidus arteriovenous malformation · Holohemispheric giant cerebral arteriovenous malformation · Proliferative angiopathy |
| fr | Angiopathie proliférative cérébrale | APC |
| de | Zerebrale proliferative Angiopathie | CPA · Diffuse arteriovenöse Fehlbildung des Nidus · Holohemisphärische zerebrale arteriovenöse Fehlbildung · Proliferative Angiopathie |
| es | Angiopatía proliferativa cerebral | APC · Angiopatía proliferativa · Malformación arteriovenosa cerebral holohemisférica gigante · Malformación arteriovenosa del nido difuso |
| it | Angiopatia proliferativa cerebrale | Malformazione arterovenosa cerebrale gigante oloemisferica · Angiopatia proliferativa · CPA · Malformazione arterovenosa diffusa a nido |
| nl | Cerebrale proliferatieve angiopathie | Arterioveneuze malformatie met diffuse nidus · CPA · Holohemisferische grote cerebrale arterioveneuze malformatie · Proliferatieve angiopathie |
| pt | Angiopatia cerebral proliferativa | Angiopatia proliferativa · CPA · Malformação arteriovenosa cerebral gigante holohemisférica · Malformação arteriovenosa nidus difuso |
| pl | Angiopatia proliferacyjna mózgu | = en |
| cs | Cerebrální proliferativní angiopatie | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q28.2 | NTBT | attributed | validated |
| ICD-11 | 8B22.40 | NTBT | attributed | 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 (3):
- ORPHA:166487Cerebral diseases of vascular origin with epilepsy
rare neurological diseases - ORPHA:715750Intracranial fast-flow vascular malformation
rare developmental anomalies during embryogenesis; rare neurological diseases; rare circulatory system diseases - ORPHA:98022Rare headache
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 | 1-9 / 1 000 000 | 0.6 / 100 000 | Worldwide | Value and class | Validated | 11571210[PMID]_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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/692271
- This record as JSON: /data/d/692271.json