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. 2022 Sep 16;8(1):75.
doi: 10.1186/s40981-022-00564-x.

General anesthesia with remimazolam for a pediatric patient with MELAS and recurrent epilepsy: a case report

Affiliations

General anesthesia with remimazolam for a pediatric patient with MELAS and recurrent epilepsy: a case report

Yusuke Yamadori et al. JA Clin Rep. .

Abstract

Background: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a mitochondrial disease. We report here the safe use of remimazolam in a pediatric MELAS patient.

Case presentation: A 10-year-old girl (118 cm, 16 kg) was scheduled for an open gastrostomy to improve nutrition and epileptic seizure control. We induced and maintained general anesthesia with remimazolam, remifentanil, fentanyl, and rocuronium. We also performed a bilateral subcostal transversus abdominis plane block before the surgery. The surgery finished uneventfully. After we discontinued remimazolam administration, the patient woke up immediately but calmly without flumazenil. Epileptic seizures did not occur during intra- and early post-operative periods.

Conclusion: Remimazolam enabled us to provide a pediatric MELAS patient with general anesthesia without causing delayed emergence or epileptic seizures.

Keywords: Epilepsy; Epileptic seizure; General anesthesia; MELAS; Mitochondrial Encephalomyopathy; Open gastrostomy; Remimazolam.

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Conflict of interest statement

The authors declare that they have no competing interests.

References

    1. Majamaa K, Moilanen JS, Uimonen S, Remes AM, Salmela PI, Kärppä M, et al. Epidemiology of A3243G, the mutation for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes: prevalence of the mutation in an adult population. Am J Hum Genet. 1998;63:447–454. doi: 10.1086/301959. - DOI - PMC - PubMed
    1. Uusimaa J, Moilanen JS, Vainionpää L, Tapanainen P, Lindholm P, Nuutinen M, et al. Prevalence, segregation, and phenotype of the mitochondrial DNA 3243A>G mutation in children. Ann Neurol. 2007;62:278–287. doi: 10.1002/ana.21196. - DOI - PubMed
    1. Fricker RM, Raffelsberger T, Rauch-Shorny S, Finsterer J, Müller-Reible C, Gilly H, et al. Positive malignant hyperthermia susceptibility in vitro test in a patient with mitochondrial myopathy and myoadenylate deaminase deficiency. Anesthesiology. 2002;97:1635–1637. doi: 10.1097/00000542-200212000-00044. - DOI - PubMed
    1. Nelson JH, Kaplan RF. Anesthetic management of two pediatric patients with concurrent diagnoses of mitochondrial disease and malignant hyperthermia susceptibility: A case report. A A Case Rep. 2017;9:204–206. doi: 10.1213/XAA.0000000000000565. - DOI - PubMed
    1. Kam PC, Cardone D. Propofol infusion syndrome. Anaesthesia. 2007;62:690–701. doi: 10.1111/j.1365-2044.2007.05055.x. - DOI - PubMed

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