raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:637051

Borna virus encephalitis

Synonyms: Bornavirus encephalitis

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Elderly
Status
active

A rare infectious disease characterized by severe non-purulent meningoencephalitis caused by Borna disease virus 1 (BoDV-1). Patients present with fever, headache, malaise, and fatigue, followed after a few days by nausea, confusion and disorientation, and psychomotor slowing. Ataxia, aphasia, dyspnea, multifocal myoclonic jerks, and paraplegia have been described. The condition is very often fatal after progress to lethargy, deteriorating brain stem reflexes, and coma. Examination of the cerebrospinal fluid reveals moderate lympho-monocytic pleocytosis and presence of BoDV-1 RNA.

1

Names in Orphadata languages

10 of 12 languages
LangNameSynonyms
enBorna virus encephalitisBornavirus encephalitis
frEncéphalite à virus Borna
deBorna-Virus-Enzephalitis
esEncefalitis por virus BornaEncefalitis por Bornavirus
itEncefalite da virus BornaEncefalite da Bornavirus
nlEncefalitis door bornavirusBorna-virus-encefalitis
ptEncefalite Borna virusEncefalite por Bornavirus
plZapalenie mózgu wywołane wirusem Borna= en
csBornavirová encefalitida= en
ukБорнавірусний енцефалітБорнавірусний енцефаліт

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10A85.8NTBTattributedvalidated
ICD-111C80NTBTindexvalidated
MONDO0957421Evalidated
UMLSC5816783Evalidated

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):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families18 casesWorldwideCase(s)Validated31924550[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidated31924550[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.

6

HPO phenotypes

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

7

Sources for this card