Dravet Syndrome and Juvenile Myoclonic Epilepsy Essentials

This session reviews Dravet Syndrome and Juvenile Myoclonic Epilepsy Essentials and its most clinically relevant points for exam preparation and bedside decision-making.

Duration

00:03:06

File size

1.40 MB

Practitioner-Guided Note

For Dravet Syndrome and Juvenile Myoclonic Epilepsy Essentials, use the highest-yield facts to drive concrete treatment decisions. Pay particular attention to Dravet syndrome: onset first year, prolonged febrile seizures (often unilateral), progresses to afebrile seizures + developmental delay, 70-80% caused by de novo SCN1A mutations, and Avoid sodium channel blockers (phenytoin, carbamazepine, lamotrigine) when choosing therapy, counseling about risk, planning monitoring, and deciding when closer follow-up or escalation is needed.

Key Takeaways

Dravet syndrome: onset first year, prolonged febrile seizures (often unilateral), progresses to afebrile seizures + developmental delay70-80% caused by de novo SCN1A mutationsAvoid sodium channel blockers (phenytoin, carbamazepine, lamotrigine)JME: adolescent onset, morning myoclonic jerks + GTCS + absences; sleep deprivation triggersValproate most effective JME treatment; lamotrigine can worsen myoclonus