A 7-month-old boy develops clusters of flexor spasms on waking, developmental regression, and EEG shows hypsarrhythmia. MRI shows a large right hemispheric malformation of cortical development. What is the most appropriate initial treatment?

• A Oral levetiracetam monotherapy
• B Corpus callosotomy
• C Ketogenic diet as first step
• D Intramuscular adrenocorticotropic hormone or oral vigabatrin ✓

Explanation

West syndrome consists of infantile spasms, hypsarrhythmia, and developmental arrest. First-line therapy is hormonal treatment with ACTH or high-dose oral corticosteroids, or vigabatrin, which is especially effective when a focal cortical lesion is present. Levetiracetam alone is inadequate for spasms, the ketogenic diet is reserved for refractory cases, and callosotomy is used for refractory drop attacks in Lennox-Gastaut syndrome rather than focal lesion-related spasms.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.