Dravet's syndrome

Generalized epilepsy with febrile seizures plus Dravet syndrome corresponds a phenotype severe myoclonic epilepsy of infancy (SMEI), a form of intractable epilepsy of childhood. Prompt and precise diagnosis of Dravet is critical, due to the high risk of status epilepticus and sudden unexplained death in epilepsy (SUDEP), and that commonly prescribed antiepileptic sodium channel blockers aggravate Dravet seizures The proportion of patients with SMEIB (i.e. borderline Dravet syndrome) who carry SCN1A mutations is the same as that observed in classical Dravet syndrome and, even within a same family, the same mutation can cause both typical and “borderline” forms After Guerrini & Oguni SMEIB is not more recommended nomenclature

Links

Lennox–Gastaut syndrome

Stiripentol

CBD

Dravet, Charlotte & Hirokazu Oguni DRAVET syndrome (severe myoclonic epilepsy in infancy)

Dravet, Charlotte Acute encephalopathy after febrile status epilepticus: an underdiagnosed, misunderstood complication of Dravet syndrome

The clinical outcome and neuroimaging of acute encephalopathy after status epilepticus in Dravet syndrome. Dev Med Child Neurol 2018; 60: 566–73

síndrome de Dravet Rev Clin Med fam 7(2): 134-136

Guerrini, R Oguni H Borderline Dravet syndrome: A useful diagnostic category? Epilepsia

Small changes in synaptic gain lead to seizure-like activity in neuronal network at criticality

Proposal for Revised Clinical and Electroencephalographic Classification of Epileptic Seizures

Augmented Reticular Thalamic Bursting and Seizures in Scn1a-Dravet Syndrome.

Disease classification WHO

G40.3 Dravet's syndrome

ICD-10 online

References

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