Reversible cerebral vasoconstriction syndrome
Synonyms: RCVS
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare cerebrovascular disorder characterized by severe headaches with or without focal neurological deficits or seizures, and a reversible segmental and multifocal vasoconstriction of cerebral arteries. It may occur spontaneously or be provoked by various precipitating factors, the most common being postpartum and exposure to various vasoactive substances such as illicit drugs and selective serotonin-reuptake inhibitors. The major complication is ischemic or hemorrhagic stroke.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Reversible cerebral vasoconstriction syndrome | RCVS |
| fr | Syndrome de vasoconstriction cérébrale réversible | RCVS · SVCR |
| de | Reversibles zerebrales Vasokonstriktionssyndrom | Call-Fleming-Syndrom · RCVS |
| es | Síndrome de vasoconstricción cerebral reversible | SVCR |
| it | Sindrome da vasocostrizione cerebrale reversibile | = en |
| nl | Reversibele cerebrale vasoconstrictie-syndroom | = en |
| pt | Síndrome de vasoconstrição cerebral reversível | |
| pl | Zespół odwracalnego kurczu naczyń mózgowych | = en |
| cs | Syndrom reverzibilní cerebrální vazokonstrikce | RCVS · Call-Flemmingův syndrom |
| uk | Синдром зворотного звуження судин головного мозку | = en |
| zh | 可逆性脑血管收缩性综合征 | = en |
| tr | Tersinir serebral vazokonstriksiyon sendromu | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I67.8 | NTBT | attributed | validated |
| ICD-11 | 8B22.2 | NTBT | index | validated |
| MONDO | 0017291 | E | validated | |
| UMLS | C3544214 | E | validated | |
| GARD | 12768 | E | validated | |
| MedDRA | 10073240 | 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 (1):
- 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 | 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
24 annotations · validated 2021-03-31| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0030907 | Thunderclap headache | |
| HP:0025637 | Vasospasm | |
| Frequent (79-30%) | ||
| HP:0410263 | Brain imaging abnormality | |
| HP:0002076 | Migraine | |
| HP:0002331 | Recurrent paroxysmal headache | |
| Occasional (29-5%) | ||
| HP:0001892 | Abnormal bleeding | |
| HP:0009145 | Abnormal cerebral artery morphology | |
| HP:0000622 | Blurred vision | |
| HP:0001342 | Cerebral hemorrhage | |
| HP:0001289 | Confusion | |
| HP:0004944 | Dilatation of the cerebral artery | |
| HP:0004302 | Functional motor deficit | |
| HP:0002140 | Ischemic stroke | |
| HP:0002352 | Leukoencephalopathy | |
| HP:0002183 | Phonophobia | |
| HP:0000613 | Photophobia | |
| HP:0002138 | Subarachnoid hemorrhage | |
| HP:0002326 | Transient ischemic attack | |
| HP:0002013 | Vomiting | |
| Very rare (<4-1%) | ||
| HP:0012158 | Carotid artery dissection | |
| HP:0002181 | Cerebral edema | |
| HP:0030746 | Intraventricular hemorrhage | |
| HP:0001250 | Seizure | |
| HP:0100309 | Subdural hemorrhage | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 31582040[PMID]_31869187[PMID]_27015869[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/284388
- This record as JSON: /data/d/284388.json