• Seon Woo Yoo, Seon Ju Baek, Dong-Chan Kim, and A Ram Doo.
• Department of Anesthesiology and Pain Medicine, Jeonbuk National University Medical School.
• Medicine (Baltimore). 2021 Jun 11; 100 (23): e25859e25859.

RationaleSeveral hereditary myopathies that can predispose to malignant hyperthermia (MH) are reported. However, the risk of MH in myotonic dystrophy type I (DM1) has been suggested equal to general population, although the evidence is limited to only a few case reports.Patient ConcernsWe encountered a rare case of MH during anesthesia induction with sevoflurane in a male adolescent with previously undiagnosed DM1.DiagnosesAfter the event, genetic testing revealed the presence of a previously unknown heterozygous missense mutation in ryanodine receptor 1 (RYR1) associated with MH (c.6898T > C; p.ser2300Pro). Concomitantly, the patient was diagnosed with DM1 with abnormal cytosine-thymine-guanine triplet expansion in the DMPK gene.InterventionsDantrolene was administered to treat the hypermetabolic manifestations in 20 minutes after the identification of MH.OutcomesThe patient was successfully treated and discharged without any complications. Laboratory abnormalities were recovered to baseline at postoperative 4 days.LessonsThe authors suggest that possible MH susceptibility in DM1 patients may be refocused. Genetic testing can be a screening tool for MH susceptibility in these population, prior to receiving general anesthesia.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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