Daily use of buccal midazolam for refractory seizures in an infant with holoprosencephaly: A case report

We report the case of an infant with alobar holoprosencephaly with drug-resistant severe epilepsy. Due to a heavy seizure burden, frequent use of buccal midazolam was attempted, and the seizures and associated sleep disturbances were effectively controlled without major adverse events. The patient was a female infant with alobar holoprosencephaly. At 60 days of age, she experienced clinical seizures with eye deviation, oral automatism, and clonic limb movements that rapidly worsened to frequent and prolonged seizures. Intravenous phenobarbital temporarily suppressed the seizures but caused prolonged sedation and feeding difficulty. Several oral anti-seizure medications were ineffective. We introduced the frequent, nightly repeated use of buccal midazolam for status epilepticus, leading to seizure-free intervals, better nutrition, and the establishment of sleep-wake cycles. No significant adverse effects were observed, including respiratory suppression. Frequent use of buccal midazolam not only reduced the seizure burden but also improved sleep and nutrition related to seizures in a patient with holoprosencephaly.