A 5-year-old boy with developmental delay has daily multiple seizure types: tonic seizures during sleep, atypical absences, and drop attacks causing frequent falls. EEG shows slow spike-and-wave discharges at less than 2.5 Hz with background slowing. What is this electroclinical syndrome called?
- A Childhood absence epilepsy
- B West syndrome
- C Lennox-Gastaut syndrome ✓
- D Benign epilepsy with centrotemporal spikes
Explanation
Lennox-Gastaut syndrome is defined by the triad of multiple seizure types including tonic seizures and drop attacks, an EEG showing slow spike-and-wave below 2.5 Hz over a slowed background, and cognitive impairment. Childhood absence epilepsy has regular 3 Hz spike-wave with preserved cognition, West syndrome features infantile spasms with hypsarrhythmia at a younger age, and rolandic epilepsy is a benign focal syndrome. Treatment is difficult; valproate, lamotrigine, clobazam, rufinamide, and the ketogenic diet are options.
Reference: Nelson Textbook of Pediatrics, 21st ed.
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