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General anesthesia for an adolescent with Pelizaeus-erzbacher disease - A case report -
Anesth Pain Med 2019;14(1):44-7
Published online January 31, 2019
© 2019 Korean Society of Neuroscience in Anesthesiology and Critical Care.

Hyuckgoo Kim , and Chaeseok Lim
Department of Anesthesiology and Pain Medicine, Yeungnam University College of Medicine, Daegu, Korea
Correspondence to: Hyuckgoo Kim, M.D. Department of Anesthesiology and Pain Medicine, Yeungnam University College of Medicine, 170 Hyeonchung-ro, Nam-gu, Daegu 42415, Korea Tel: 82-53-620-3357 Fax: 82-53-626-5275 E-mail:
Received May 4, 2018; Revised June 18, 2018; Accepted June 22, 2018.
cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Pelizaeus-Merzbacher disease (PMD) is a progressive and degenerative chromosomal disorder of the central nervous system caused by defective myelin production. Few case reports have been issued on the anesthetic management of PMD, because of its extremely low incidence. We anesthetized a 13-year-old female patient diagnosed with PMD for ophthalmic surgery because of intermittent exotropia. General anesthesia was induced and maintained with propofol and sevoflurane in air and oxygen. Rocuronium was administered to facilitate orotracheal intubation, and residual neuromuscular blockage was reversed with pyridostigmine. Between emergence to 24 hours postoperatively, her muscle power completely recovered and no unpredictable events occurred. Summarizing, anesthesiologists should be concerned about the high possibility of aspiration, spasticity, and seizure during the perioperative period in patients with even mild PMD. Appropriate preoperative evaluation, intraoperative monitoring, and choice of proper anesthetic drugs enable safe anesthesia in patients with PMD.

Key Words : General anesthesia, Genetic disease, Leukodystropy, Pelizaeus–Merzbacher disease

July 2019, 14 (3)
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