Hemiconvulsion-hemiplegia-epilepsy syndrome
Synonyms: IHHS · HHE · HHE syndrome
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare childhood-onset epilepsy syndrome characterized by hemiplegia occurring in children (typically under the age of 4) following prolonged focal seizures, often febrile in nature. The condition progresses through three stages: initial prolonged seizures, subsequent hemiplegia (weakness or paralysis on one side of the body), and eventual epilepsy, following a latent period. The condition may be idiopathic or associated with structural, infectious, traumatic, or degenerative brain abnormalities. Patients present with acute unilateral brain swelling, followed by cerebral hemiatrophy. Inflammation, genetic predisposition, and prolonged seizure activity may play a role in the disease development/progression.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hemiconvulsion-hemiplegia-epilepsy syndrome | IHHS · HHE · HHE syndrome |
| fr | Syndrome d'hémiconvulsion-hémiplégie-épilepsie | HHE · SHHEI · Syndrome HHE |
| de | Hemikonvulsion-Hemiplegie-Epilepsie-Syndrom | HHE-Syndrom · IHHS |
| es | Síndrome de hemiconvulsión-hemiplejia-epilepsia | HHE · IHHS · Síndrome HHE |
| it | Sindrome emiconvulsione-emiplegia-epilessia | IHHS · Sindrome HHE · HHE |
| nl | Hemiconvulsie - hemiplegie - epilepsie-syndroom | HHE · HHE-syndroom · IHHS |
| pt | Síndrome de hemiconvulsão-hemiplegia-epilepsia | HHE · IHHS · Síndrome HHE · Síndrome de hemiconvulsão-hemiplegia-epilepsia |
| pl | Idiopatyczny zespół z drgawkami połowiczymi i porażeniem połowiczym | IHHS · Zespół HHE · Zespół padaczkowy z drgawkami połowiczymi i porażeniem połowiczym |
| cs | Idiopatický syndrom hemikonvulze a hemiplegie | HHE syndrom · Syndrom zahrnující hemikonvulzi, hemiplegii a epilepsii · IHHS |
| uk | Синдром ідіопатичної геміконвульсії-геміплегії | Синдром геміконвульсій-геміплегії-епілепсії · Синдром HHE · IHHS |
| zh | 特发性偏侧惊厥-偏瘫综合征 | 偏侧惊厥-偏瘫-癫痫综合征 |
| tr | İdiyopatik hemikonvülsiyon-hemipleji sendromu | IHHS · HHE sendromu · Hemikonvülsiyon-hemipleji-epilepsi sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G40.4 | NTBT | attributed | validated |
| ICD-11 | 8A62.Y | NTBT | index | validated |
| MONDO | 0019485 | E | validated | |
| UMLS | C0549118 | E | validated | |
| MedDRA | 10085010 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98259Childhood-onset epilepsy syndrome
rare genetic diseases; rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86908
- This record as JSON: /data/d/86908.json