Chorea misinterpreted as agitation in a 15-month-old girl with semilobar holoprosencephaly: a case report

We report the case of a 15-month-old girl with semilobar holoprosencephaly, microcephaly, central diabetes insipidus, failure to thrive, and severe global developmental delay who was admitted to the pediatric intensive care unit for aspiration pneumonia requiring mechanical ventilation.Caregivers reported long-standing severe irritability and poor sleep, for which she had been taking melatonin and diazepam before admission.During ventilator weaning, persistent irritability, irregular breathing, and excessive nonrhythmic movements were repeatedly interpreted as agitation, leading to increased analgesic and sedative requirements, including sufentanil and midazolam infusions and intermittent rescue chloral hydrate, and contributing to a prolonged ventilatory course.The movements were involuntary and irregular, worsened during wakefulness, and resolved during sleep.Electroencephalography revealed no electrographic seizures or epileptiform discharges and showed a poorly organized background consistent with the underlying structural brain disease.After chorea was suspected, pediatric neurology was consulted.Additional caregiver history suggested that similar involuntary movements had occurred before admission and may have contributed to her long-standing irritability and poor sleep.Treatment with clonazepam and dopamine receptor antagonists, including haloperidol and risperidone, reduced the movements and improved clinical stability, enabling transfer to the ward and subsequent discharge.This case illustrates that chorea may be mistaken for agitation in neurologically impaired infants or toddlers and that careful observation of the movement pattern, including suppression during sleep, may facilitate recognition and limit unnecessary escalation of sedation.