raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:363567

Acute encephalopathy with inflammation-mediated status epilepticus

Type
Clinical group
Group
Group of disorders
Age of onset
All ages
Status
active

This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcute encephalopathy with inflammation-mediated status epilepticus
frEncéphalopathie épileptique aiguë fébrileAEIMSE
deAkute Enzephalopathie mit entzündungsvermitteltem Status Epilepticus
esEncefalopatía aguda con estado epiléptico mediado por una inflamación
itEncefalopatia acuta con stato epilettico mediato da un'infiammazione
nlAcute encefalopathie met inflammatiegemedieerde status epilepticus
ptEncefalopatia aguda com estado epiléptico mediado por inflamação
plOstra encefalopatia ze stanem padaczkowym wywołana stanem zapalnym
csAkutní encefalopatie se status epilepticus na podkladě zánětu
ukГостра енцефалопатія з епілептичним статусом, повязана з запаленням
zh急性脑病伴炎症介导的癫痫持续状态
trİnflamasyon aracılı status epileptikus ile seyreden akut ensefalopati
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO18200Evalidated
UMLSC5680984Evalidated

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 (2):

Children (3):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

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

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card