raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:166427

Startle epilepsy

Type
Disease
Group
Disorder
Status
active

Startle epilepsy is a rare neurologic disease characterized by frequent and spontaneous epileptic seizures (frequently with symmetrical or asymmetrical tonic features) triggered by a normal startle in response to a sudden and unexpected somatosensory (most frequently auditory) stimulus. Falls are common and can be traumatic. In most cases, the disease is associated with spastic hemi-, di-, or tetraplegia and intellectual disability.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enStartle epilepsy
frEpilepsie-sursaut
deSchreckepilepsieStartle-Epilepsie
esEpilepsia del sobresalto
itEpilessia indotta da sussulti
nlSchrikepilepsieEpilepsie door schrikken
ptEpilepsia do susto
plPadaczka z zaskoczenia
csÚleková epilepsie
ukЕпілепсія з рефлекторними судомами, повязаними з відчуттям переляку
zh惊吓性癫痫
trStartle epilepsisi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10G40.5NTBTattributedvalidated
ICD-118A61.40NTBTindexvalidated
MONDO0015648Evalidated
UMLSC4706527Evalidated

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

0 records

No prevalence record in this release.

6

HPO phenotypes

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

7

Sources for this card