A 5-year-old previously normal boy presents with progressive cognitive decline, behavioral deterioration with autistic features, and occasional focal seizures over 3 months. His parents report he was learning normally until recently. Sleep-deprived EEG shows the pattern marked **C** in the diagram—continuous bilateral synchronous spike-and-wave discharges occupying 85% of non-REM sleep, while the waking EEG shows only scattered centrotemporal spikes. Brain MRI is normal. Which of the following is the MOST APPROPRIATE first-line treatment for this condition?

Question

A 5-year-old previously normal boy presents with progressive cognitive decline, behavioral deterioration with autistic features, and occasional focal seizures over 3 months. His parents report he was learning normally until recently. Sleep-deprived EEG shows the pattern marked **C** in the diagram—continuous bilateral synchronous spike-and-wave discharges occupying >85% of non-REM sleep, while the waking EEG shows only scattered centrotemporal spikes. Brain MRI is normal. Which of the following is the MOST APPROPRIATE first-line treatment for this condition?

Accepted Answer

B. High-dose corticosteroids (prednisolone) or ACTH. ## Why High-dose corticosteroids (prednisolone) or ACTH is right

The EEG pattern marked **C**—continuous spike-wave of sleep (CSWS), also called electrical status epilepticus in sleep (ESES)—is a rare age-related epileptic encephalopathy with onset typically between 3–10 years. The hallmark is a spike-wave index (SWI) >85% during non-REM sleep. The clinical presentation of global cognitive regression, behavioral deterioration, and seizures in a previously normal child with this EEG pattern is pathognomonic for CSWS. The mechanism involves disruption of sleep-dependent memory consolidation and synaptic homeostasis by near-continuous nocturnal epileptiform activity. According to Nelson Pediatrics 21e and the Epilepsia 2009 CSWS consensus, high-dose corticosteroids (prednisolone or ACTH) or valproate are the established first-line treatments. Early aggressive therapy is essential because cognitive sequelae often persist if treatment is delayed.

## Why each distractor is wrong

- **Carbamazepine monotherapy**: Carbamazepine is explicitly contraindicated and can worsen CSWS; it should be avoided in this condition despite its efficacy in other childhood epilepsies.
- **Phenytoin with valproate combination**: Phenytoin, like carbamazepine, can worsen CSWS and is contraindicated. Although valproate is a first-line option, combining it with phenytoin is inappropriate and potentially harmful.
- **Levetiracetam as monotherapy**: Levetiracetam is not established as first-line therapy for CSWS. The evidence base for corticosteroids and valproate is much stronger, and early aggressive treatment with proven agents is critical to prevent permanent cognitive damage.

**High-Yield:** CSWS (ESES) is an epileptic encephalopathy where the nocturnal spike-wave burden (>85% SWI) causes daytime cognitive regression—treat aggressively with corticosteroids or valproate; avoid carbamazepine and phenytoin.

[cite: Nelson Pediatrics 21e; Epilepsia 2009 CSWS consensus]

Answer

A. Carbamazepine monotherapy

Answer

C. Phenytoin with valproate combination

Answer

D. Levetiracetam as monotherapy

Explanation

Why High-dose corticosteroids (prednisolone) or ACTH is right

Why each distractor is wrong

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