Pediatrics · CNS Disorders in Children (Seizures, Hydrocephalus, Meningitis)

A 4-year-old boy has daily axial tonic seizures causing sudden falls, atypical absences lasting up to 30 seconds, and generalized tonic-clonic seizures. He has moderate intellectual disability and behavior problems. EEG shows slow spike-and-wave discharges below 2.5 Hz with bursts of fast rhythms during sleep. What is the most likely diagnosis?

  • A Childhood absence epilepsy
  • B Benign epilepsy with centrotemporal spikes
  • C Lennox-Gastaut syndrome
  • D Dravet syndrome
Correct answer: C. Lennox-Gastaut syndrome

Explanation

The combination of multiple refractory seizure types including tonic drop attacks, cognitive impairment, and EEG showing slow spike-and-wave under 2.5 Hz with generalized paroxysmal fast activity defines Lennox-Gastaut syndrome. Childhood absence epilepsy has regular 3 Hz spike-wave, normal cognition and absence seizures alone, and lacks tonic seizures. Dravet syndrome begins earlier with fever-triggered hemiclonic seizures and SCN1A mutations.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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