Propofol infusion syndrome complicated with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes: a case report

Shimizu, Junji; Tabata, Toshihide; Tsujita, Yasuyuki; Yamane, Tetsunobu; Yamamoto, Yutaka; Tsukamoto, Takahito; Ogawa, Norio; Kim, Hyou; Urushitani, Makoto; Eguchi, Yawara

doi:10.1002/ams2.473

Cited by 9 publications

(13 citation statements)

References 10 publications

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“…PRIS patient with the genetically proven mitochondrial disease[ 23 ] and MELAS patient who developed PRIS were also reported. [ 25 ] MELAS could not be ruled out, but laboratory tests, radiological findings of our patient did not meet the diagnostic criteria,[ 10 ] and we did not perform pathological or genetic tests. We should have tested these tests and proceeded with a differential diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Unilateral posterior reversible encephalopathy syndrome characterized with a long and gradually exacerbating course over 3 years and that presented propofol infusion syndrome – A case report

Katsuki

Ozaki

Narita

et al. 2021

Surgical Neurology International

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Background: Posterior reversible encephalopathy syndrome (PRES) is characterized by acute neurological symptoms and vasogenic edema, and most patients wholly recover. We report a unilateral PRES patient characterized by a gradual onset followed by propofol infusion syndrome (PRIS) due to general anesthesia therapy. Case Description: A 32-year-old woman had ovarian dysfunction treated by Kaufmann’s treatment for 17 years. Three years ago, she developed seizures, and photophobia and myoclonus sometimes occurred. This time, she had strong photophobia and nausea for 3 months and then developed tonic-clonic seizures for 3 min. Her blood pressure and laboratory test on admission were all within normal limits. She presented no neurological deficits at admission, but the T2-weighted image (T2WI) showed a high-intensity area (HIA), and arterial spin labeling (ASL) image described cerebral blood flow (CBF) increase in the left parieto-occipital region. We diagnosed PRES and started anticonvulsants, antihypertensive, and steroid pulse therapy. However, her aphasia and neuroimaging findings worsened, so we started general anesthesia treatment with propofol on day 29. On day 32, she suddenly developed multiple organ dysfunctions due to PRIS. After intensive care with other sedatives over 2 months, the systemic status and neurological symptoms gradually improved almost as before the onset. On day 90, HIA in the T2WI in the lesion became small, and CBF was severely downregulated in the ASL image. Conclusion: Unilateral PRES’s pathophysiology and the association with the female hormone remain unknown. Some patients undergo gradual onset and long-term courses, and we should care for PRIS during PRES treatment.

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Section: Discussionmentioning

confidence: 99%

Unilateral posterior reversible encephalopathy syndrome characterized with a long and gradually exacerbating course over 3 years and that presented propofol infusion syndrome – A case report

Katsuki

Ozaki

Narita

et al. 2021

Surgical Neurology International

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“…Hence, anaesthesiologists are advised against the prolonged use of volatile anaesthetics in patients with NMDs. Although often recommended as an alternative, total intravenous anaesthesia (TIVA; most frequently based on a propofol, short‐acting benzodiazepines, or dexmedetomidine‐based technique in combination with short‐acting potent opioids) has other risks [ 53 , 54 , 55 , 56 ]. This is an important consideration as part of a comprehensive risk–benefit assessment between TIVA and volatile anaesthetic‐based techniques.…”

Section: Level 1: General Recommendations For Patients With ...mentioning

confidence: 99%

“…Mitochondrial disorders are considered to increase the risk of propofol infusion syndrome [ 54 , 55 , 56 ], although there are case series reporting no complications [ 93 , 94 ]. The cases of propofol infusion syndrome in patients with mitochondrial disorders occurred after 3–5 days of propofol infusion [ 54 , 55 , 56 ]. However, propofol infusion syndrome has also been reported after <1 h of infusion in healthy children and after 2 h in healthy adults [ 53 ].…”

Section: Level 2: Group‐specific Recommendationsmentioning

confidence: 99%

European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

Bersselaar

Heytens

Silva

et al. 2022

Euro J of Neurology

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Background and purpose Patients with neuromuscular conditions are at increased risk of suffering perioperative complications related to anaesthesia. There is currently little specific anaesthetic guidance concerning these patients. Here, we present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with neuromuscular disorders as formulated during the 259th ENMC Workshop on Anaesthesia in Neuromuscular Disorders. Methods International experts in the field of (paediatric) anaesthesia, neurology, and genetics were invited to participate in the ENMC workshop. A literature search was conducted in PubMed and Embase, the main findings of which were disseminated to the participants and presented during the workshop. Depending on specific expertise, participants presented the existing evidence and their expert opinion concerning anaesthetic management in six specific groups of myopathies and neuromuscular junction disorders. The consensus statement was prepared according to the AGREE II (Appraisal of Guidelines for Research & Evaluation) reporting checklist. The level of evidence has been adapted according to the SIGN (Scottish Intercollegiate Guidelines Network) grading system. The final consensus statement was subjected to a modified Delphi process. Results A set of general recommendations valid for the anaesthetic management of patients with neuromuscular disorders in general have been formulated. Specific recommendations were formulated for (i) neuromuscular junction disorders, (ii) muscle channelopathies (nondystrophic myotonia and periodic paralysis), (iii) myotonic dystrophy (types 1 and 2), (iv) muscular dystrophies, (v) congenital myopathies and congenital dystrophies, and (vi) mitochondrial and metabolic myopathies. Conclusions This ENMC consensus statement summarizes the most important considerations for planning and performing anaesthesia in patients with neuromuscular disorders.

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“…(6 of 15) who had an improvement were also treated concomitantly with AEDs (eTable 5, links.lww.com/WNL/ B836). Six patients deteriorated clinically, with worsening seizures, despite acute treatment with L-arginine, [12][13][14][15][16][17] requiring escalation of AED therapy, including admission to the intensive care unit. 16 Radiologic changes after L-arginine treatment were reported as improvements in magnetic resonance spectroscopy parameters and partial or full resolution of stroke-like lesions on brain MRI (Table 3).…”

Section: Efficacy: Acute L-argininementioning

confidence: 99%

l -Arginine in Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes

Stefanetti

Errington

et al. 2022

Neurology

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Objective:Stroke management in the context of primary mitochondrial disease is clinically challenging and the best treatment options for patients with stroke-like episodes remain uncertain. We sought to perform a systematic review on the safety and efficacy of L-arginine use in the acute and prophylactic management of stroke-like episodes in patients with mitochondrial disease.Methods:The systematic review was registered in PROSPERO (CRD42020181230). We searched six databases from inception − 15/01/2021: MEDLINE, Embase, Scopus, Web of Science, CINAHL and ClinicalTrials.gov. Original articles and registered trials available, in English, reporting L-arginine use in the acute or prophylactic management of stroke-like episodes in patients with genetically confirmed mitochondrial disease were eligible for inclusion.Data on safety and treatment response were extracted and summarized by multiple observers. Risk of bias was assessed by the methodological quality of case reports, case series and a risk-of-bias checklist for non-randomized studies. Quality of evidence was synthesized using the Oxford Centre for Evidence-Based Medicine Levels of Evidence and Grade of Recommendations. The predetermined main outcome measures were clinical response to L-arginine treatment, adverse events, withdrawals, and deaths (on treatment and/or during follow up), as defined by the author.Results:Thirty-seven articles met inclusion [0=randomised controlled trials (RCTs); 3 open-label; 1 retrospective cohort; 33 case reports/case series] (N = 91 patients; 86% m.3243A>G). In the case reports, 54% of patients reported a positive clinical response to acute L-arginine, of which 40% were concomitantly treated with AEDs. Improved headache at 24-hours was the greatest reported benefit in response to IV L-arginine in the open-label trials (31/39, 79%). Of 15/48 patients (31%) who positively responded to prophylactic L-arginine, AEDs were either used (7/15) or unreported (8/15). Moderate adverse events were reported in the follow-up of both IV and oral L-arginine treatment and 11 patients (12%) died during follow up or while on prophylactic treatment.Conclusion:The available evidence is of poor methodological quality and classified as Level 5. IV and/or oral L-arginine confers no demonstrable clinical benefit in either the acute or prophylactic treatment of MELAS, with more robust controlled trials required to assess its efficacy and safety profile.

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Propofol infusion syndrome complicated with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes: a case report

Cited by 9 publications

References 10 publications

Unilateral posterior reversible encephalopathy syndrome characterized with a long and gradually exacerbating course over 3 years and that presented propofol infusion syndrome – A case report

Unilateral posterior reversible encephalopathy syndrome characterized with a long and gradually exacerbating course over 3 years and that presented propofol infusion syndrome – A case report

European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

l -Arginine in Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes

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