Anesth Pain Med.  2019 Oct;14(4):416-422. 10.17085/apm.2019.14.4.416.

Dexmedetomidine as a non-triggering anesthetic agent in a patient with MELAS syndrome and systemic sepsis: A case report

  • 1Department of Anesthesiology and Pain Medicine, Chosun University Hospital, Gwangju, Korea.


The selection of anesthetic agents is important in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome patient because serious and unexpected complications can occur after anesthetic exposure. CASE: A 30-year-old man with MELAS syndrome and sepsis underwent colectomy. Propofol was administered by step-wise until target effect-site concentration (Ce) 1.0 µg/ml and stopped for the loss of consciousness and to avoid hemodynamic instability. After the loss of consciousness, total intravenous anesthesia (TIVA) using dexmedetomidine (1.0 µg/ml/h) and remifentanil (1-4 ng/ml of Ce) was performed for the maintenance of anesthesia to avoid malignant hyperthermia and mitochondrial dysfunction. During the surgery, the bispectral index score stayed between 26 and 44, and increased to 97 after the end of anesthesia.
TIVA with dexmedetomidine and remifentanil as non-triggering anesthetic agents in patients with MELAS syndrome and systemic sepsis may have advantages to decrease damages associated with mitochondrial stress and metabolic burden.


Dexmedetomidine; Intravenous anesthesia; MELAS syndrome; Remifentanil; Sepsis

MeSH Terms

Anesthesia, Intravenous
Malignant Hyperthermia
MELAS Syndrome*


  • Fig. 1 Timeline of anesthesia during the surgery. Propofol was administered by target-controlled infusion by step-wise escalation of the effect-site concentration (0.2 μg/ml per 1 min). After five minutes, the patient lost consciousness and his BIS score became 44 when the Ce of propofol was 1.0 μg/ml. During the infusion of propofol, dexmedetomidine infusion (1.0 μg/kg/h) without a loading dose was started simultaneously. After the loss of consciousness, the Ce of the propofol was adjusted to 0 μg/ml and anesthesia was maintained with dexmedetomidine (1.0 μg/ml/h) and remifentanil (1–4 ng/ml of Ce). The blue line shows the change in the BIS score. The black line shows the change in the estimated plasma concentration of dexmedetomidine. The blue box shows the effective dexmedetomidine concentration in clinically ill patients (0.22–2.50 ng/ml) [4]. Ce: effect site concentration, BIS: bispectral index.

  • Fig. 2 Changes in SpO2, temperature, ETCO2, and the results of ABGA during the surgery. Metabolic acidosis and hypoglycemia were managed during anesthesia. The patient’s temperature was managed carefully to avoid hypothermia or hyperthermia during anesthesia. SPO2: oxygen saturation, ETCO2: end-tidal CO2, ABGA: arterial blood gas analysis.

  • Fig. 3 Hemodynamic data during the surgery. Dobutamine and norepinephrine were administered from the start of anesthesia, and MAP and CO were maintained above 50 mmHg and 6 L/min, respectively, guided by SVV during anesthesia. MAP: mean arterial pressure, SVV: stroke volume variation, CO: cardiac output.


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