Self-limited epilepsy with autonomic seizures
Synonyms: Benign childhood occipital epilepsy, Panayiotopoulos type · Early-onset benign childhood occipital epilepsy · Panayiotopoulos syndrome · SeLEAS
- Type
- Disease
- Group
- Disorder
- Status
- active
A form of rare self-limited childhood occipital epilepsy characterized by focal seizures with autonomic features, such as nausea, vomiting, pallor, and cardiorespiratory changes, in addition to visual disturbances. Patient shows a fluctuation in symptoms and awareness during the same seizure event. Seizures are typically long lasting. This syndrome is the most common cause of afebrile, nonconvulsive status epilepticus in childhood. Seizures typically begin between the ages of 3 and 6, with remission usually occurring within 2 or 3 years. They are usually infrequent, however in some patients they can be frequent or disruptive. There is no impact on neurodevelopment, unlike self-limited childhood focal epilepsies.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Self-limited epilepsy with autonomic seizures | Benign childhood occipital epilepsy, Panayiotopoulos type · Early-onset benign childhood occipital epilepsy · Panayiotopoulos syndrome · SeLEAS |
| fr | Epilepsie autolimitée avec crises autonomiques | Epilepsie occipitale bénigne à début précoce · SeLEAS · Syndrome de Panayiotopoulos · Epilepsie occipitale bénigne type Panayiotopoulos |
| de | Selbstlimitierende Epilepsie mit autonomen Anfällen | Früh beginnende benigne okzipitale Epilepsie des Kindesalters · Panayiotopoulos-Syndrom · SeLEAS |
| es | Epilepsia autolimitada con crisis autonómicas | Epilepsia occipital benigna de la infancia de inicio precoz · Epilepsia occipital benigna de la infancia tipo Panayiotopoulos · Síndrome de Panayiotopoulos |
| it | Epilessia autolimitante con convulsioni del sistema autonomo | Epilessia occipitale infantile benigna, tipo Panayiotopoulos · Epilessia occipitale infantile benigna a esordio precoce · Sindrome di Panayiotopoulos |
| nl | Zelflimiterende epilepsie met autonome insulten | Goedaardige occipitale epilepsie in de kindertijd, Panayiotopoulos-type · SeLEAS · Syndroom van Panayiotopoulos · Vroeg optredende goedaardige occipitale epilepsie in de kindertijd |
| pt | Epilepsia auto-limitada com convulsões autonómicas | Epilepsia ocipital benigna da infância de início precoce · Epilepsia ocipital benigna da infância, tipo Panayiotopoulos · SeLEAS · Síndrome Panayiotopoulos |
| pl | Łagodna dziecięca padaczka potyliczna typu Panayiotopoulosa | Łagodna dziecięca padaczka potyliczna, typ Panayiotopoulosa |
| cs | Benigní dětská okcipitální epilepsie, Panayiotopoulosův typ | Benigní dětská okcipitální epilepsie s časným počátkem · Panayiotopoulosův syndrom |
| uk | Доброякісна дитяча потилична епілепсія, тип Панайотопулоса | Рання доброякісна дитяча потилична епілепсія · Синдром Панайотопулоса |
| zh | 儿童良性枕叶癫痫型Panayiotopoulos型 | 早发性儿童良性枕叶癫痫 |
| tr | Selim çocukluk çağı oksipital epilepsi, Panayiotopoulos tipi | Panayiotopoulos sendromu · Erken başlangıçlı selim çocukluk çağı oksipital epilepsisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G40.0 | NTBT | attributed | validated |
| ICD-11 | 8A61.2Y | NTBT | index | validated |
| MONDO | 0020307 | E | validated | |
| UMLS | C0393676 | 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:726812Self limited focal childhood epilepsy
rare genetic diseases; rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.8 / 100 000 | United Kingdom | Value and class | Validated | DOI:10.1136/archdischild-2016-310863.98[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/98815
- This record as JSON: /data/d/98815.json