raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:71211

Neuromyelitis optica spectrum disorder

Synonyms: Devic disease · NMOSD

Type
Disease
Group
Disorder
Inheritance
Multigenic/multifactorial
Age of onset
Adolescent, Adult, Childhood, Elderly
Status
active

A rare inflammatory disease of the central nervous system characterized mainly by attacks of uni- or bilateral optic neuritis (ON) and acute myelitis.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNeuromyelitis optica spectrum disorderDevic disease · NMOSD
frNeuromyélite optiqueMaladie de Devic · Trouble du spectre de la neuromyélite optique
deNeuromyelitis-optica-Spektrum-StörungDevic-Krankheit · NMO-Spektrum-Erkrankungen · Neuromyelitis-optica-Spektrum-Krankheit
esTrastorno del espectro de la neuromielitis ópticaEnfermedad de Devic
itSpettro della neuromielite ottica
nlNeuromyelitis opticaZiekte van Devic
ptEspectro clínico de neuromielite ópticaDoença de Devic · Neuromielite óptica
plZapalenie rdzenia i nerwów wzrokowychChoroba Devica
csSpektrum onemocnění neuromyelitis opticaDevicova nemoc
ukРозлади спектру оптиконейромієлітуХвороба Девіча · NMOSD
zh视神经脊髓炎Devic病
trNöromiyelitis optikaDevic hastalığı

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10G36.0Especificvalidated
ICD-118A43Especificvalidated
MONDO0019100Evalidated
MONDO19100Evalidated
MeSHD009471Evalidated
UMLSC0027873Evalidated
GARD6267Evalidated
MedDRA10077875Evalidated

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

31 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 1 000 0000.73 / 100 000MartiniqueValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.054 / 100 000AustriaValue and classValidated33310876[PMID]
Annual incidence1-9 / 1 000 0000.1877 / 100 000WorldwideValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0002.071 / 100 000WorldwideValue and classValidated33310876[PMID]
Point prevalence1-9 / 1 000 0000.7 / 100 000AustriaValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.08 / 100 000United KingdomValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0001.43 / 100 000United KingdomValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.09 / 100 000NetherlandsValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.063 / 100 000SpainValue and classValidated33310876[PMID]
Point prevalence1-9 / 1 000 0000.89 / 100 000SpainValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.07 / 100 000DenmarkValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0001.09 / 100 000DenmarkValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.079 / 100 000SwedenValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0001.04 / 100 000SwedenValue and classValidated33310876[PMID]
Annual incidence1-9 / 1 000 0000.132 / 100 000HungaryValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0001.91 / 100 000HungaryValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.037 / 100 000AustraliaValue and classValidated33310876[PMID]
Point prevalence1-9 / 1 000 0000.7 / 100 000AustraliaValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0003.9 / 100 000United StatesValue and classValidated33310876[PMID]
Annual incidence1-9 / 1 000 0000.495 / 100 000IndiaValue and classValidated33310876[PMID]
Annual incidence1-9 / 1 000 0000.495 / 100 000CanadaValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0002.6 / 100 000IndiaValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0003.42 / 100 000JapanValue and classValidated33310876[PMID]
Annual incidence1-9 / 1 000 0000.39 / 100 000MalaysiaValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0001.94 / 100 000MalaysiaValue and classValidated33310876[PMID]
Point prevalence1-9 / 100 0001.51 / 100 000Iran, Islamic Republic ofValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.053 / 100 000CubaValue and classValidated33310876[PMID]
Point prevalence1-9 / 1 000 0000.52 / 100 000CubaValue and classValidated33310876[PMID]
Point prevalence1-5 / 10 00010 / 100 000MartiniqueValue and classValidated33310876[PMID]
Annual incidence1-9 / 1 000 0000.285 / 100 000Costa ricaValue and classValidated33310876[PMID]
Annual incidence<1 / 1 000 0000.07 / 100 000United StatesValue and classValidated33310876[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

17 annotations · validated 2016-12-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0030057Autoimmune antibody positivity
HP:0000009Functional abnormality of the bladder
HP:0012486Myelitis
HP:0002529Neuronal loss in central nervous system
HP:0200026Ocular pain
HP:0100653Optic neuritis
HP:0010550Paraplegia
HP:0011096Peripheral demyelination
HP:0003474Somatic sensory dysfunction
HP:0000572Visual loss
Frequent (79-30%)
HP:0012443Abnormality of brain morphology
HP:0033342Anti-aquaporin 4 antibody positivity
Occasional (29-5%)
HP:0032492Anti-myelin oligodendrocyte glycoprotein antibody positivity
HP:0012229CSF pleocytosis
HP:0002018Nausea
HP:0100247Recurrent singultus
HP:0002878Respiratory failure

Run the differential on the 10 obligate / very frequent terms

Annotation source: 3895189[PMID]

7

Sources for this card