Dravet syndrome

Description

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.

Coding note from above levels

Use additional code, if desired, to identify the type of seizure.

Index terms

Dravet syndrome
Severe myoclonic epilepsy in infancy

Exclusions from above levels

MG45 – Syncope
05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth and the puerperium

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19 – Certain conditions originating in the perinatal period
22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

8A61.10 – Benign familial infantile epilepsy
8A61.12 – Epilepsy of infancy with migrating focal seizures
8A61.1Y – Other specified genetic epileptic syndromes with onset in infancy
8A61.1Z – Genetic epileptic syndromes with onset in infancy, unspecified

Foundation entity

Dravet syndrome – http://id.who.int/icd/entity/1255654700

Allowed postcoordination axes

hasManifestation

Part of: 8A61.1: Genetic epileptic syndromes with onset in infancy › 8A61: Genetic or presumed genetic syndromes primarily expressed as epilepsy › 08: Diseases of the nervous system