[Efficacy of Dexmedetomidine in Monitored Anesthesia Care (MAC) for a Woman with Mitochondrial Encephalomyopathy Associated with Myoclonus].

In general, there is no high resistance to intravenous anesthetics for sedation or anesthetic induction in patients with mitochondrial encephalomyopathy. We experienced a female case of mitochondrial encephalomyopathy with myoclonus, who showed high resistance to midazolam and propofol in monitored anesthe- sia care (MAC) for drainage of subcutaneous abscess. However, dexmedetomidine (DEX) was very effective. First total of midazolam 100 mg was administrated in ten minutes and she was sedated. But her myoclo- nus continued. Secondly, propofol 30 mg was adminis- trated stopping her myoclonus. But it recurred in a few minutes and propofol 30 mg was re-administrated. Next DEX was administered at 1.0 μg · kg⁻¹ in ten minutes and 0.7-0.8 μg · kg⁻¹ · hr⁻¹ continuously. After that her sedation became stabilized. In conclusion, mitochondrial encephalomyopathy patients with myoclonus may show resistance to midazolam and propofol for sedation or MAC for surgical local treatment DEX may be effective in such cases.