Mesial temporal lobe epilepsy with hippocampal sclerosis
Synonyms: HS-MTLE · Hippocampal sclerosis-related mesial temporal lobe epilepsy · MTLE-HS
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
Mesial temporal lobe epilepsy with hippocampal sclerosis is a rare epilepsy syndrome defined by seizures originating in limbic areas of the mesial temporal lobe, particularly in the hippocampus, amygdala, and in the parahippocampal gyrus and its connections, and hippocampal sclerosis, usually unilateral or assymetric. It is frequently associated with an initial precipitating event, such as febrile seizures, hypoxia, intracranial infection or head trauma, most often occurring in the first five years of life, followed by a latent period without seizures. Typical seizures consist of a characteristic aura that is frequently a rising epigastric sensation associated with emotional disturbances, illusions, and autonomic symptoms (widened pupils, palpitations), progressive impairment of consciousness, oro-alimentary automatisms (lip smacking, chewing, licking, tooth grinding), behavioral arrest, head deviation, dystonic postures, hand and verbal automatisms. Seizures are followed by postictal dysfunction. Initially, seizures are easily controlled with antiepileptic drugs, later they frequently become refractory and associated with progressive behavioral changes and memory deficits.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mesial temporal lobe epilepsy with hippocampal sclerosis | HS-MTLE · Hippocampal sclerosis-related mesial temporal lobe epilepsy · MTLE-HS |
| fr | Epilepsie du lobe mésio-temporal avec sclérose de l'hippocampe | Epilepsie mésiotemporale avec sclérose hippocampique · MTLE-HS |
| de | Mesiale Temporallappenepilepsie mit Hippocampussklerose | HS-MTLE · Hippokampussklerose-assoziierte mesiale Temporallappenepilepsie · MTLE-HS |
| es | Epilepsia del lóbulo temporal mesial con esclerosis del hipocampo | Epilepsia del lóbulo temporal mesial asociada a esclerosis del hipocampo · HS-MTLE · MTLE-HS |
| it | Epilessia del lobo temporale mesiale con sclerosi ippocampale | Epilessia del lobo temporale mesiale da sclerosi ippocampale · HS-MTLE · MTLE-HS |
| nl | Mesiale temporaalkwabepilepsie met sclerose van hippocampus | HS-MTLE · MTLE-HS · Mesiale temporaalkwabepilepsie gerelateerd aan hippocampale sclerose |
| pt | Epilepsia do lobo temporal mesial com esclerose do hipocampo | Epilepsia do lobo temporal mesial relacionada com esclerose do hipocampo · HS-MTLE · MTLE-HS |
| pl | Padaczka mezjalna skroniowa skroniowego ze stwardnieniem hipokampa | Padaczka przyśrodkowej częśći płata skroniowego ze stwardnieniem hipokampa |
| cs | Meziální temporální epilepsie s hipokampální sklerózou | = en |
| uk | Мезіальна скронева епілепсія з гіпокампальним склерозом | Мезіальна скронева епілепсія, повязана зі склерозом гіпокампу · HS-MTLE · MTLE-HS |
| zh | 颞叶内侧癫痫伴海马硬化 | MTLE-HS |
| tr | Hipokampal skleroz ile seyreden mezial temporal lob epilepsisi | MTLE-HS |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G40.0 | NTBT | attributed | validated |
| MONDO | 0020476 | E | validated | |
| UMLS | C4749367 | 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/99701
- This record as JSON: /data/d/99701.json