A 9-month-old infant has episodes of sudden flexion of the neck and trunk with arm extension lasting 2 seconds, occurring in clusters of 20–30 upon waking. EEG shows hypsarrhythmia (chaotic high-voltage disorganised pattern). MRI brain is normal. Which treatment has evidence for both seizure control AND improved developmental outcome?
- A Sodium valproate monotherapy
- B Vigabatrin as first-line in all cases
- C ACTH (adrenocorticotropic hormone) or oral prednisolone ✓
- D Phenobarbitone loading
Explanation
West syndrome (infantile spasms + hypsarrhythmia) in a cryptogenic/unknown cause infant is treated with ACTH or high-dose oral corticosteroids (prednisolone). The UKISS and ICISS trials demonstrated that hormonal therapy (ACTH/prednisolone) achieves faster cessation of infantile spasms and improves neurodevelopmental outcomes compared to vigabatrin alone in non-tuberous sclerosis cases. Vigabatrin is preferred in tuberous sclerosis complex (TSC). The combination of hormonal therapy + vigabatrin may be superior in some protocols. Valproate and phenobarbitone are ineffective for infantile spasms.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.