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Last Update: 04/01/2024 02:31 AM

Current Deck: Neurology::Prior Rite Questions

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A 2-year-old boy has had seizures since age 9 months. Initially, most episodes were characterized by prolonged hemiclonic seizures that coincided with fever. The parents now report seizures can be triggered by hot or warm baths. "Staring off" spells and myoclonic jerks have also emerged lately. For the past couple months, he has missed his development milestones. Which of the following antiseizure medications is contraindicated?
  • cannabidiol
  • topiramate
  • Lamotrigine
  • clobiazam
  • valproic acid
Answer: {{c1::Lamotrigine}}
Notes
his case is suggestive of Dravet syndrome, a severe form of epilepsy that begins in infancy. Lamotrigine is contraindicated in patients with Dravet syndrome because it can exacerbate seizures in these individuals.

Analysis of Incorrect Answers:
  • Cannabidiol: This is incorrect because cannabidiol is actually one of the few effective treatments for Dravet syndrome and has been approved by the FDA for this indication.
  • Topiramate: This is also incorrect because topiramate can be used in the management of Dravet syndrome and does not exacerbate seizures.
  • Clobiazam: This is incorrect because clobiazam is often used as an adjunctive treatment in Dravet syndrome and has been shown to reduce seizure frequency.
  • Valproic acid: This is incorrect because valproic acid is a commonly used antiseizure medication in Dravet syndrome and can be effective in controlling seizures.
Pathophysiology Outline: Dravet syndrome, also known as severe myoclonic epilepsy of infancy (SMEI), is a rare and catastrophic form of intractable epilepsy that begins in infancy. It is often caused by a mutation in the SCN1A gene, which affects the function of sodium channels in the brain. The disease is characterized by frequent, prolonged seizures often triggered by fever, hot baths, or warm temperatures. Over time, other types of seizures such as myoclonic seizures and absence seizures may also appear. Developmental delay and other neurological symptoms are also common.


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