CNS drugs and antiepileptics: Dravet syndrome: practice question

A Pharmacology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Pharmacology · CNS drugs and antiepileptics: Dravet syndrome

Sample item

An 8-month-old develops prolonged febrile hemiclonic seizures, later myoclonic seizures and ataxia, and genetic testing shows an SCN1A mutation. Seizures worsened after carbamazepine. Which add-on agent is appropriate?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

B. Stiripentol

SCN1A loss of function in Dravet syndrome means sodium channel blockers such as carbamazepine, phenytoin, lamotrigine and oxcarbazepine can worsen seizures. Stiripentol, used with valproate and clobazam, and cannabidiol are effective add-on agents. Valproate and clobazam form the usual base.

Option by option

  • A. Phenytoin

    Phenytoin blocks sodium channels and can worsen seizures in this condition.

  • B. Stiripentol (correct)

    Correct. Stiripentol is an effective add-on to valproate and clobazam in this syndrome.

  • C. Lamotrigine

    Lamotrigine blocks sodium channels and can aggravate seizures in Dravet syndrome.

  • D. Oxcarbazepine

    Oxcarbazepine is a sodium channel blocker and is expected to aggravate seizures.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this Pharmacology question

What is the answer to this Pharmacology question?

The best answer is B, Stiripentol. SCN1A loss of function in Dravet syndrome means sodium channel blockers such as carbamazepine, phenytoin, lamotrigine and oxcarbazepine can worsen seizures.

Why is "Phenytoin" not the answer?

Phenytoin blocks sodium channels and can worsen seizures in this condition.

Why is "Lamotrigine" not the answer?

Lamotrigine blocks sodium channels and can aggravate seizures in Dravet syndrome.

Why is "Oxcarbazepine" not the answer?

Oxcarbazepine is a sodium channel blocker and is expected to aggravate seizures.

What concept does this question test?

It tests CNS drugs and antiepileptics: Dravet syndrome in Pharmacology. Stiripentol, used with valproate and clobazam, and cannabidiol are effective add-on agents. Valproate and clobazam form the usual base.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study CNS drugs and antiepileptics: Dravet syndrome?

Learn drugs as classes, then the exception in each class that an examiner would pick. Then use the flashcards and the other Pharmacology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.