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Infantile epileptic spasms syndrome — RACP Paediatrics MCQ

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HardNeurology and neurodevelopmentInfantile epileptic spasms syndromeRACP Paediatrics

A 5-month-old has clusters of brief flexor spasms on waking and has stopped rolling. EEG shows high-amplitude chaotic activity with multifocal spikes. What is the most appropriate management?

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Correct answer: C — Urgent paediatric neurology treatment with hormonal therapy or vigabatrin pathway

Clusters of developmental-regression-associated spasms with hypsarrhythmia indicate infantile epileptic spasms syndrome, requiring urgent treatment to improve developmental outcomes. Reflux and benign shuddering do not cause developmental regression or hypsarrhythmia. The pearl is that infantile spasms are a neurological emergency even when each event is brief.

Reference: RCH Clinical Practice Guidelines: Seizures; Therapeutic Guidelines (eTG)