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Although generally considered as reliable and safe, administration of propofol can occasionally induce a potentially fatal complication known as<jats:italic>propofol infusion syndrome<\/jats:italic>(<jats:styled-content style=\"fixed-case\">PRIS<\/jats:styled-content>). Mitochondrial dysfunction has been implicated in the pathogenesis of<jats:styled-content style=\"fixed-case\">PRIS<\/jats:styled-content>. We report on an adult patient with<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>eber hereditary optic neuropathy (<jats:styled-content style=\"fixed-case\">LHON<\/jats:styled-content>) who developed<jats:styled-content style=\"fixed-case\">PRIS<\/jats:styled-content>. He was a carrier of the m.3460<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>&gt;<jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>mutation, one of the major three pathogenic point mutations associated with<jats:styled-content style=\"fixed-case\">LHON<\/jats:styled-content>. The propositus was blind and underwent propofol sedation after severe head injury. Five days after start of propofol infusion, the patient died. The activity of complex<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>of the oxidative phosphorylation (<jats:styled-content style=\"fixed-case\">OXPHOS<\/jats:styled-content>) system was severely deficient in skeletal muscle. Our observation indicates that fulminate<jats:styled-content style=\"fixed-case\">PRIS<\/jats:styled-content>can occur in an adult patient with an inborn<jats:styled-content style=\"fixed-case\">OXPHOS<\/jats:styled-content>defect and corroborates the hypothesis that<jats:styled-content style=\"fixed-case\">PRIS<\/jats:styled-content>is caused by inhibition of the<jats:styled-content style=\"fixed-case\">OXPHOS<\/jats:styled-content>system.<\/jats:p>","DOI":"10.1111\/j.1399-6576.2011.02628.x","type":"journal-article","created":{"date-parts":[[2012,1,19]],"date-time":"2012-01-19T12:45:32Z","timestamp":1326977132000},"page":"520-525","source":"Crossref","is-referenced-by-count":37,"title":["Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome"],"prefix":"10.1111","volume":"56","author":[{"given":"A. 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