Spontaneous intracranial hypotension
Synonyms: Spontaneous cerebrospinal fluid leak
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare headache resulting from a cerebrospinal fluid (CSF) leak with subsequent lowered CSF pressure, characterized clinically by severe headaches which typically worsen upon standing up and get better when lying down. Additional features may include neck stiffness, nausea, vomiting, vertigo, tinnitus, visual disturbances, and cognitive abnormalities, among others, as sagging and displacement of the brain can lead to a variety of lesions and symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Spontaneous intracranial hypotension | Spontaneous cerebrospinal fluid leak |
| fr | Hypotension intracrânienne spontanée | Fuite spontanée de liquide céphalo-rachidien |
| de | Spontane intrakranielle Hypotension | Spontanes Liquorunterdrucksyndrom |
| es | Hipotensión intracraneal espontánea | Pérdida espontánea de líquido cefalorraquídeo |
| it | Ipotensione intracranica spontanea | Perdita spontanea di liquido cefalorachidiano |
| nl | Spontane intracraniële hypotensie | Spontaan lek van cerebrospinaal vocht |
| pt | Hipotensão intracraniana espontânea | |
| pl | Spontaniczne niedociśnienie wewnątrzczaszkowe | Spontaniczny wyciek płynu mózgowo-rdzeniowego |
| cs | Spontánní intrakraniální hypotenze | Spontánní únik mozkomíšního moku |
| uk | Спонтанна внутрішньочерепна гіпотензія | Спонтанний витік спинномозкової рідини |
| zh | 自发性颅内低压 | 自发性脑脊液漏 |
| tr | Spontan intrakraniyal hipotansiyon | Spontan beyin omurilik sıvısı sızıntısı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G96.0 | E | specific | validated |
| ICD-11 | 8D61.0 | E | specific | validated |
| MONDO | 0018624 | E | validated | |
| UMLS | C0751731 | 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 |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 5 / 100 000 | Worldwide | Value and class | Validated | 32644492[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/443180
- This record as JSON: /data/d/443180.json