J Neurocrit Care.  2022 Dec;15(2):122-125. 10.18700/jnc.220071.

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

• Nguyen B
• Figueroa S

• KMID: 2538769
• DOI: http://doi.org/10.18700/jnc.220071

Abstract

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.
Conclusions
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.

Keyword

Epilepsy; Propofol infusion syndrome; Mitochondrial diseases; Mutation; Propofol; Adult

Figure

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  Fig. 1. Electroencephalogram demonstrating the refractory brief ictal/interictal rhythmic discharges pattern.

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  Fig. 2. Electrocardiogram changes showing deep T wave inversions after lab abnormalities were discovered that were not present previously.

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