Idiopathic intracranial hypertension
Synonyms: Benign intracranial hypertension · IIH · Pseudotumor cerebri
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
Idiopathic intracranial hypertension is a neurological disorder characterized by isolated increased intracranial pressure manifesting with recurrent and persistent headaches, nausea, vomiting, progressive and transient obstruction of the visual field, papilledema. Visual loss can be irreversible.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic intracranial hypertension | Benign intracranial hypertension · IIH · Pseudotumor cerebri |
| fr | Hypertension intracrânienne idiopathique | HII · Hypertension intracrânienne bénigne · IIH · Pseudotumeur cérébrale · Pseudotumor cerebri |
| de | Hypertension, idiopathische intrakranielle | IIH · Intrakranielle Hypertension, benigne · Pseudotumor cerebri |
| es | Hipertensión intracraneal idiopática | IIH · Pseudotumor cerebri · HII · Hipertensión intracraneal benigna |
| it | Ipertensione intracranica idiopatica | IIH · Ipertensione intracranica benigna · Pseudotumor cerebri |
| nl | Idiopathische intracraniële hypertensie | Goedaardige intracraniële hypertensie · IIH · Pseudotumor cerebri |
| pt | Hipertensão intracraniana idiopática | |
| pl | Idiopatyczne nadciśnienie wewnątrzczaszkowe | IIH · Łagodne nadciśnienie wewnątrzczaszkowe · Rzekomy guz mózgu |
| cs | Idiopatická intrakraniální hypertenze | IIH · Pseudotumor cerebri · Benigní intrakraniální hypertenze |
| uk | Ідіопатична внутрішньочерепна гіпертензія | Доброякісна внутрішньочерепна гіпертензія · IIH · Псевдопухлина головного мозку |
| zh | 自发性颅内高压 | 良性颅内高压 |
| tr | İdiyopatik intrakraniyal hipertansiyon | IIH · Psödotümör serebri · Selim intrakraniyal hipertansiyon |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 243200 | E | validated | |
| ICD-10 | G93.2 | E | specific | validated |
| ICD-11 | 8D60.Y | NTBT | index | validated |
| MONDO | 0009468 | E | validated | |
| MeSH | D011559 | E | validated | |
| UMLS | C0033845 | E | validated | |
| GARD | 4561 | E | validated | |
| MedDRA | 10078904 | 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:98006Rare neurologic disease
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 | 1-5 / 10 000 | 14 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2016[OTHER] |
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 2019-07-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002315 | Headache | |
| HP:0002516 | Increased intracranial pressure | |
| Frequent (79-30%) | ||
| HP:0012393 | Allergy | |
| HP:0001513 | Obesity | |
| HP:0001085 | Papilledema | |
| Occasional (29-5%) | ||
| HP:0100851 | Abnormal emotion/affect behavior | |
| HP:0000622 | Blurred vision | |
| HP:0000651 | Diplopia | |
| HP:0002018 | Nausea | |
| HP:0000613 | Photophobia | |
| HP:0010822 | Scintillating scotoma | |
| HP:0002360 | Sleep abnormality | |
| HP:0000572 | Visual loss | |
| HP:0002013 | Vomiting | |
| Very rare (<4-1%) | ||
| HP:0003418 | Back pain | |
| HP:0000716 | Depression | |
| HP:0001254 | Lethargy | |
| HP:0002076 | Migraine | |
| HP:0011161 | Olfactory auras | |
| HP:0008629 | Pulsatile tinnitus | |
| HP:0002321 | Vertigo | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 31213758[PMID]_28013373[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/238624
- This record as JSON: /data/d/238624.json