Reversible cerebral vasoconstriction syndrome

Summary

Reversible cerebral vasoconstriction syndrome (MONDO:0017291) is a disease and 7 clinical trials. Top therapeutic interventions include nimodipine. A subtype of headache disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 24
• Clinical trials: 7

Clinical features

Signs & symptoms

Clinical features (HPO)

24 HPO clinical features (Orphanet curated; top 24 by frequency):

HPO ID	Term	Frequency
HP:0025637	Vasospasm	Very frequent (80-99%)
HP:0030907	Thunderclap headache	Very frequent (80-99%)
HP:0002076	Migraine	Frequent (30-79%)
HP:0002331	Recurrent paroxysmal headache	Frequent (30-79%)
HP:0410263	Brain imaging abnormality	Frequent (30-79%)
HP:0000613	Photophobia	Occasional (5-29%)
HP:0000622	Blurred vision	Occasional (5-29%)
HP:0001289	Confusion	Occasional (5-29%)
HP:0001342	Cerebral hemorrhage	Occasional (5-29%)
HP:0001892	Abnormal bleeding	Occasional (5-29%)
HP:0002013	Vomiting	Occasional (5-29%)
HP:0002138	Subarachnoid hemorrhage	Occasional (5-29%)
HP:0002140	Ischemic stroke	Occasional (5-29%)
HP:0002183	Phonophobia	Occasional (5-29%)
HP:0002326	Transient ischemic attack	Occasional (5-29%)
HP:0002352	Leukoencephalopathy	Occasional (5-29%)
HP:0004302	Functional motor deficit	Occasional (5-29%)
HP:0004944	Dilatation of the cerebral artery	Occasional (5-29%)
HP:0009145	Abnormal cerebral artery morphology	Occasional (5-29%)
HP:0001250	Seizure	Very rare (<1-4%)
HP:0002181	Cerebral edema	Very rare (<1-4%)
HP:0012158	Carotid artery dissection	Very rare (<1-4%)
HP:0030746	Intraventricular hemorrhage	Very rare (<1-4%)
HP:0100309	Subdural hemorrhage	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	reversible cerebral vasoconstriction syndrome
Mondo ID	MONDO:0017291
Orphanet	284388
ICD-11	1474881372
SNOMED CT	700467001
UMLS	C3544214
MedGen	811291
GARD	0012768
Is cancer (heuristic)	no

Also known as: RCVS

Disease family

This is a subtype of headache disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › nervous system disorder › neurological pain disorder › headache disorder › reversible cerebral vasoconstriction syndrome

Related subtypes (3): hypnic headache, spontaneous intracranial hypotension, exertional headache

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 7.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	5
PHASE4	1
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03150524	PHASE4	WITHDRAWN	RCVS: The Rational Approach to Diagnosis and Treatment
NCT04580238	PHASE1	WITHDRAWN	Onabotulinum Toxin A (Botox) for the Treatment of Persistent Post-Stroke and Vascular Headache
NCT04179383	Not specified	RECRUITING	Triggers And Risk Factors to Develop a Reversible Cerebral Vasoconstriction Syndrome
NCT05491980	Not specified	ACTIVE_NOT_RECRUITING	Florida Cerebrovascular Disease Biorepository and Genomics Center
NCT02756416	Not specified	UNKNOWN	Perfusion MRI in Reversible Cerebral Vasoconstriction Syndrome
NCT03509701	Not specified	UNKNOWN	Reversible Cerebral Vasoconstriction Syndrome and Varicella Zoster Virus
NCT04463212	Not specified	COMPLETED	Endothelial Dysfunction in Reversible Cerebral Vasoconstriction Syndrome

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
NIMODIPINE	4	1
CHEMBL1514440	0	1