raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:221091

Trigeminal neuralgia

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

A rare acquired peripheral neuropathy characterized by paroxysmal, sharp, stabbing, electric-shock-like orofacial pain, that is restricted to one or more of the trigeminal nerve divisions and mostly unilateral. Attacks are brief (few seconds to a maximum of two minutes), but typically occur repeatedly and periodically, can arise spontaneously or be triggered by innocuous stimuli, and are frequently accompanied by tic-like cramps of facial muscles. The condition affects women more often than men.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enTrigeminal neuralgia
frNévralgie trigéminaleNévralgie du trijumeau · Tic douloureux
deTrigeminusneuralgieTic douloureux
esNeuralgia trigeminalTic doloroso
itNevralgia del trigemino
nlTrigeminusneuralgieTic douloureux
ptNeuralgia do trigémio
plNeuralgia nerwu trójdzielnego
csNeuralgie trigeminu
ukНевралгія трійчастого нерва
zh三叉神经痛
trTrigeminal nevralji

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
OMIM190400Evalidated
ICD-10G50.0Especificvalidated
ICD-118B82.0Especificvalidated
MONDO0008599Evalidated
MeSHD014277Evalidated
UMLSC0040997Evalidated
GARD7805Evalidated
MedDRA10044652Evalidated

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

3 records
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET
Annual incidence1-9 / 100 0004.3 / 100 000United StatesValue and classValidated15062534[PMID]
Annual incidence1-9 / 100 0008 / 100 000United KingdomValue and classValidated10733998[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 2018-09-19
HPOTerm (as annotated by Orphanet)Diagnostic
Obligate (100%)
HP:0100661Trigeminal neuralgia
Very frequent (99-80%)
HP:0012533Allodynia
HP:0012199Cluster headache
HP:0000716Depression
Frequent (79-30%)
HP:0000740Episodic paroxysmal anxiety
HP:0011968Feeding difficulties
HP:0200025Mandibular pain
HP:0003401Paresthesia
HP:0002465Poor speech
HP:0003474Somatic sensory dysfunction
HP:0000183Tongue muscle weakness
HP:0004948Vascular tortuosity
Occasional (29-5%)
HP:0007305CNS demyelination
HP:0001293Cranial nerve compression
HP:0002664Neoplasm
HP:0200026Ocular pain
HP:0011096Peripheral demyelination

Run the differential on the 4 obligate / very frequent terms

Annotation source: 12940815[PMID]_11960159[PMID]_11565877[PMID]_1983882[PMID]_24534115[PMID]_27306631[PMID]_2716987[PMID]_5334334[PMID]_14315671[PMID]

7

Sources for this card