Stiripentol

DIACOMIT

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Pronunciation: stir-i-PEN-tol (dye-a-KA-mit)

Stiripentol ballicule: receptor binding, kinetics and half-life diagram — Mechanism of action: Stiripentol ballicule

Stiripentol DIACOMIT mascot mnemonic cartoon — Visual mnemonic: Stiripentol mascot
**“Die or commit, Stirrup pen doll”**
Approved as an add-on to clobazam (ONFI, the mascot on the left) for seizures in Dravet syndrome

What it isGABA-A positive allosteric modulator used with clobazam for Dravet syndrome.

Why this oneIts Dravet-specific role includes deliberately increasing clobazam’s active metabolite through CYP inhibition.

What limits itSomnolence, appetite and weight loss, neutropenia, and interactions are common; clobazam often needs reduction.

Stiripentol molecular structure (skeletal formula) — Molecular structure: Stiripentol

FDA-approved for

Dravet syndrome (2018)

FDA label

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FDA dosing	Population	Start	Target	Max
Seizures with Dravet syndrome (adjunct with clobazam)	≥6 months, ≥7 kg	50 mg/kg/day divided BID or TID	50 mg/kg/day	3000 mg/day

Renal Undefined

Hepatic Undefined

Max 3000 mg/day; do not exceed BID frequency in patients <10 kg (limit free water/overexposure).

Instructions

Capsules: swallow whole with water during a meal; do not break or open.
Oral suspension: mix in water (100 mL) and take immediately during a meal.
Withdraw gradually to minimize risk of increased seizures/status epilepticus.
If somnolence with concomitant clobazam, reduce clobazam by 25%.

Available strengths

cap 250, 500 mg
susp 250, 500 mg/packet

Cautions

Somnolence, esp. with clobazam/other CNS depressants
Decreased appetite and weight — monitor growth in pediatrics
Neutropenia and thrombocytopenia — CBC before treatment and every 6 months
Withdrawal seizures/status epilepticus on abrupt discontinuation
PKU: oral suspension contains phenylalanine
Suicidal behavior and ideation

Adverse reactions

Somnolence
Decreased appetite
Agitation
Ataxia
Weight decreased
Hypotonia
Nausea
Tremor
Dysarthria
Insomnia

Pregnancy No adequate human data; animal developmental toxicity (malformations, mortality) below clinical dose. Enroll in NAAED registry.

Lactation No data on presence in milk or infant effects; weigh benefits/risks.

Cost

Stiripentol (DIACOMIT)

Strength		#/mo	CostPlus
250 mg	cap	60 BID	not offered
500 mg	cap	60 BID	not offered
250 mg/packet	susp	per unit	not offered
500 mg/packet	susp	per unit	not offered

NADAC is a voluntary CMS survey of what retail community pharmacies pay for the drugs they stock. Stiripentol is dispensed outside retail pharmacies, so it rarely appears on surveyed invoices.

Generic entry2029 est.

NO BOXED WARNINGS

Principal riskmarked sedation from increased clobazam and norclobazam exposure

Classes and tags

Clinical profile

Therapeutic safety burden2 / 4

Overdose danger2 / 4

Weight-gain liability1 / 4

Sedation liability3 / 4

QT / Torsades0 / 4

Direct muscarinic antagonism0 / 4

Embryofetal risk2 / 4

Legacy pregnancy categoryNot assigned — PLLR-era drug

Defining liabilitySedation, dizziness and agent-specific rash, behavioral, hepatic, hematologic or teratogenic toxicity.

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Memory aids: interaction avatars

CYP2C19 inhibitor (moderate)

CYP3A4 inhibitor (moderate)

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