raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:488239

Acute macular neuroretinopathy

Synonyms: AMNR

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

A rare, acquired retinal disorder characterised by transient or permanent visual impairment accompanied by the presence of reddish-brown, wedge-shaped lesions in the macula, the apices of which tend to point towards the fovea. The lesions usually appear in a petaloid or tear-drop configuration. Patients tend to be young, Caucasian, and female.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcute macular neuroretinopathyAMNR
frNeurorétinopathie aiguë maculaire= en
deNeuroretinopathie, akute makuläre= en
esNeurorretinopatía macular aguda= en
itNeuroretinopatia maculare acuta
nlAcute maculaire neuroretinopathie= en
ptNeurorretinopatia macular aguda
plOstra neuroretinopatia plamki żółtej= en
csAkutní makulární neuroretinopatie= en
ukГостра макулярна нейроретинопатія= en
zh急性黄斑神经视网膜病变= en
trAkut maküler nöroretinopati= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H35.3NTBTattributedvalidated
ICD-119B65.0NTBTindexvalidated
MONDO0044627Evalidated
UMLSC5200735Evalidated
MedDRA10086444Evalidated

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 (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families101 casesWorldwideCase(s)Validated26973287[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidatedORPHANET_26973287[PMID]

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