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Antiepileptic (orphan)

Stiripentol

Brand names: Diacomit

Adult dose

Dose: 50 mg/kg/day PO in 2-3 divided doses (with clobazam ± valproate)
Route: PO
Frequency: divided

Clinical pearls

  • Dravet syndrome (SCN1A) refractory generalised tonic-clonic seizures — orphan
  • Specialist neurology

Contraindications

  • Severe hepatic impairment
  • Severe renal impairment
  • Hypersensitivity

Side effects

  • Sedation
  • Anorexia
  • Weight loss
  • Ataxia
  • Cytopenias
  • Hepatic enzyme elevation

Interactions

  • Strong CYP inhibitor — many
  • Clobazam (raises norclobazam — usually intentional)
  • Valproate

Monitoring

  • FBC
  • LFTs
  • Drug levels (concomitant)

Reference: BNF; NICE; SmPC; https://bnf.nice.org.uk/drugs/stiripentol/. Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.