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Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome.

A V Vanlander¹, P G Jorens, J Smet

¹Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Ghent University Hospital, Ghent, Belgium.

Acta Anaesthesiologica Scandinavica

|January 21, 2012

Summary

Propofol infusion syndrome (PRIS) can be fatal, especially in patients with underlying mitochondrial defects. This case highlights the risk of PRIS in an adult with Leber hereditary optic neuropathy (LHON) and impaired oxidative phosphorylation.

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Area of Science:

Mitochondrial Medicine
Neuro-ophthalmology
Critical Care Medicine

Background:

Propofol is a common anesthetic agent, but its use can lead to Propofol Infusion Syndrome (PRIS), a serious complication.
Mitochondrial dysfunction is a suspected cause of PRIS.
Leber hereditary optic neuropathy (LHON) is an inherited mitochondrial disease.

Observation:

This report details an adult patient with LHON, a known mitochondrial disorder, who developed PRIS.
The patient carried the m.3460G>A mutation, a common cause of LHON.
The patient received propofol for sedation following a head injury and subsequently died.

Findings:

The patient exhibited severe deficiency in Complex I activity of the oxidative phosphorylation (OXPHOS) system in skeletal muscle.
This finding suggests an inborn error of metabolism affecting mitochondrial function.

Implications:

This case demonstrates that severe PRIS can occur in adults with pre-existing OXPHOS defects.
The findings support the hypothesis that PRIS results from the inhibition of the OXPHOS system.
Clinicians should consider underlying mitochondrial disorders in patients developing PRIS, particularly those with neurological or metabolic symptoms.