Dravet syndrome

ICD-11 code : 8A61.11;

Preferred Label : Dravet syndrome;

ICD-11 definition : A refractory epileptic encephalopathy occurring in otherwise healthy infants during the first year of life with clonic/tonic-clonic, generalised and unilateral seizures, hemiclonic or generalised status epilepticus. The interictal EEG may initially be normal but with time background activity deteriorates and bilateral asymmetric, focal or multifocal paroxysms of polyspike and slow-waves appear. Mutations in the voltage-gated sodium channel gene SCN1A are commonly found.;

ICD-11 synonym : Severe myoclonic epilepsy in infancy;

Details

Origin ID

1255654700;

Automatic exact mappings (from CISMeF team)
- Developmental and Epileptic Encephalopathy 2 [NCIt concept]
- epilepsies, myoclonic [MeSH Descriptor]
Currated CISMeF NLP mapping
- Developmental and Epileptic Encephalopathy 6A [NCIt concept]
- Dravet Syndrome [NCIt concept]
- Dravet syndrome [OMIM Phenotype]
- Dravet syndrome [DO Concept]
- Dravet syndrome [ORDO Disease]
- Dravet syndrome [PASCAL descriptor]
- Dravet syndrome [MedDRA LLT]
- Severe myoclonic epilepsy in infancy (disorder) [SNOMED CT concept]
- Severe myoclonic epilepsy of infancy [MedDRA Preferred Term]
- epilepsy, myoclonic, infantile, severe [MeSH concept]
ICD-10 Mapping
- Generalized idiopathic epilepsy and epileptic syndromes [ICD-10 Sub-category]

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