ORPHA:624166
Non-specific autoimmune supratentorial encephalitis with characteristic antibodies
Synonyms: Non-specific supratentorial AE with characteristic antibodies
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Orphadata ships no English definition for this code.
1
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Non-specific autoimmune supratentorial encephalitis with characteristic antibodies | Non-specific supratentorial AE with characteristic antibodies |
| fr | Encéphalite auto-immune supratentoriale non-spécifique avec anticorps caractéristiques | EA supratentoriale non-spécifique avec anticorps caractéristiques |
| de | Unspezifische autoimmune supratentorielle Enzephalitis mit charakteristischen Antikörpern | Unspezifische supratentorielle AE mit charakteristischen Antikörpern |
| es | Encefalitis autoinmune supratentorial inespecífica con anticuerpos característicos | EA supratentorial inespecífica con anticuerpos característicos |
| it | Encefalite autoimmune sopratentoriale aspecifica con anticorpi caratteristici | EA sopratentoriale aspecifica con anticorpi caratteristici |
| nl | Aspecifieke auto-immune supratentoriële encefalitis met typische antilichamen | Aspecifieke supratentoriële AE met typische antilichamen |
| pt | Encefalite supratentorial autoimune não-específica com anticorpos característicos | AE supratentorial não-específica com anticorpos característicos |
| pl | Niespecyficzne autoimmunologiczne nadnamiotowe zapalenie mózgu z charakterystycznymi przeciwciałami | = en |
| cs | Nespecifická autoimunitní supratentoriální encefalitida s charakteristickými protilátkami | = en |
| uk | Неспецифічний аутоімунний супратенторіальний енцефаліт з характерними антитілами | Неспецифічний супратенторіальний АЕ з характерними антитілами |
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G04.8 | NTBT | attributed | validated |
| MONDO | 0850102 | E | validated | |
| UMLS | C5680408 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:622014Autoimmune encephalitis
rare neurological diseases - ORPHA:166484Inflammatory and autoimmune disease with epilepsy
rare neurological diseases - ORPHA:36388Paraneoplastic neurologic syndrome
rare neurological diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
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.
6
HPO phenotypes
3 annotations · validated 2025-08-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:6001059 | Anti-DREBRIN antibody positivity | |
| HP:6000877 | Anti-dachshund family transcription factor 1 antibody positivity | |
| HP:6000878 | Anti-potassium channel tetramerization domain containing 16 antibody positivity | |
Annotation source: 35150165[PMID]_31009048[PMID]_32196746[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/624166
- This record as JSON: /data/d/624166.json