Management of propofol-related infusion syndrome and discussion of POLG mitochondrial mutation: a case report

Background: Propofol-related infusion syndrome (PRIS) is a known complication of long-term propofol infusion. Providers should be aware of PRIS risk, as early recognition is key to avoiding mortality, which can range from 30% to 60%. The underlying mechanism of PRIS is unknown, but some studies suggest that underlying mitochondrial dysfunction may predispose patients to developing PRIS. Case Report: We present a case of refractory adult-onset epilepsy that was challenging due to a paradoxical response to propofol with worsening brief ictal/interictal rhythmic discharges and complicated by development of PRIS. We aimed to discuss the clinical presentations of PRIS, along with a review of the mitochondrial POLG mutation found in our patient, which has also been described in other case reports of refractory adult-onset epilepsy. Conclusion: We discuss the treatment strategy utilized in hopes of raising awareness of the risks in managing patients with epilepsy who have a potential underlying mitochondrial disorder.