Dravet Syndrome – Board-Style Questions

What this deck covers

Dravet syndrome in 18 board-style clinical cases with worked explanations. Each case gives you a clinical vignette, four options, the correct answer, and an explanation of why each of the other three is wrong, with the guideline or textbook it follows named on the answer.

Board-style teaching questions for paediatricians and trainees preparing for paediatric board, MRCPCH and licensing examinations. Written from published guidelines and standard paediatric textbooks, with the source named on every answer.

The 18 cases, in order

1. The first-year trace
2. Diary of triggers
3. New kinds of event
4. What to expect
5. The next pregnancy
6. A paradox
7. Only the women
8. A family pattern
9. The injection day
10. Worse on a new drug
11. A first regular drug
12. Still not settled
13. Before the new medicine
14. Sleepy after the oil
15. The hardest question
16. A change in walking
17. A summer abroad
18. Into adult life

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: Generalised spike and wave at three cycles per second, A rise in body temperature, as in a hot bath, Clusters of epileptic spasms shortly after waking up, Loss of hand use with repetitive wringing movements, It is low, with a small residual risk from mosaicism, Excitatory neurones gain channel function and fire repeatedly, CDKL5, causing refractory seizures from the first weeks, Nocturnal frontal lobe epilepsy of dominant type, Continue immunising, as that day only brought onset forward, Natural progression of the disease, unrelated to any medicine, Oral phenytoin, given as long-term maintenance, Add vigabatrin as the next agent in the ladder, Echocardiography for valve disease and lung hypertension, A new infection of the brain causing encephalitis.

Clinical pearls from this deck

• Dravet in year one: the trace is often entirely normal.
• Dravet: a hot bath can trigger a seizure without fever.
• Alternating hemiclonic seizures in infancy suggest Dravet.
• Dravet: normal infant, slowing only from the second year.
• De novo SCN1A: low recurrence, but mosaicism is possible.
• SCN1A loss silences the brake cells, the interneurons.

Work through the deck once for recognition, then again a week later to test yourself — each question gives the answer and explains why every other option fails.

More from this system: all Neurology case decks · pediatric reference values.