Anesth Pain Med.  2019 Jan;14(1):44-47. 10.17085/apm.2019.14.1.44.

General anesthesia for an adolescent with Pelizaeus-Merzbacher disease: A case report

• Kim H
• Lim C

• KMID: 2434198
• DOI: http://doi.org/10.17085/apm.2019.14.1.44

Abstract

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.

Keyword

General anesthesia; Genetic disease; Leukodystropy; Pelizaeus-Merzbacher disease

MeSH Terms

Adolescent*
Anesthesia
Anesthesia, General*
Anesthetics
Central Nervous System
Chromosome Disorders
Exotropia
Female
Humans
Incidence
Intubation
Monitoring, Intraoperative
Muscle Spasticity
Myelin Sheath
Oxygen
Pelizaeus-Merzbacher Disease*
Perioperative Period
Propofol
Pyridostigmine Bromide
Seizures
Anesthetics
Oxygen
Propofol
Pyridostigmine Bromide

Reference

1. Kamekura N, Nitta Y, Takuma S, Fujisawa T. General anesthesia for a patient with pelizaeus-merzbacher disease. Anesth Prog. 2016; 63:91–4. DOI: 10.2344/15-00022.1. PMID: 27269667. PMCID: PMC4896048.

2. Numata Y, Gotoh L, Iwaki A, Kurosawa K, Takanashi J, Deguchi K, et al. Epidemiological, clinical, and genetic landscapes of hypoymelinating leukodystrophies. J Neurol. 2014; 261:752–8. DOI: 10.1007/s00415-014-7263-5. PMID: 24532200.

3. Hobson GM, Garbern JY. Pelizaeus-merzbacher disease, pelizaeus-merzbacher-like disease 1, and related hypomyelinating disorders. Semin Neurol. 2012; 32:62–7. DOI: 10.1055/s-0032-1306388. PMID: 22422208.

4. Hernández-Palazón J. Anaesthetic management in children with metachromatic leukodystrophy. Paediatr Anaesth. 2003; 13:733–4. DOI: 10.1046/j.1460-9592.2003.01133.x. PMID: 14535916.

5. Torii T, Miyamoto Y, Yamauchi J, Tanoue A. Pelizaeus-merzbacher disease: cellular pathogenesis and pharmacologic therapy. Pediatr Int. 2014; 56:659–66. DOI: 10.1111/ped.12450. PMID: 25040584.

6. Garbern JY. Pelizaeus-merzbacher disease: genetic and cellular pathogenesis. Cell Mol Life Sci. 2007; 64:50–65. DOI: 10.1007/s00018-006-6182-8. PMID: 17115121.

7. American Society of Anesthesiologists Committee. Practice guidelines for preoperative fasting and the use of pharmacologic agents to reduce the risk of pulmonary aspiration: application to healthy patients undergoing elective procedures: an updated report by the American Society of Anesthesiologists task force on preoperative fasting and the use of pharmacologic agents to reduce the risk of pulmonary aspiration. Anesthesiology. 2017; 126:376–93. DOI: 10.1097/ALN.0000000000001452. PMID: 28045707.

8. Tobias JD. Anaesthetic considerations for the child with leukodystrophy. Can J Anaesth. 1992; 39:394–7. DOI: 10.1007/BF03009053. PMID: 1563064.

9. Mariotti P, Fasano A, Contarino MF, Della Marca G, Piastra M, Genovese O, et al. Management of status dystonicus: our experience and review of the literature. Mov Disord. 2007; 22:963–8. DOI: 10.1002/mds.21471. PMID: 17427939.

10. Wang PJ, Hwu WL, Shen YZ. Epileptic seizures and electroencephalographic evolution in genetic leukodystrophies. J Clin Neurophysiol. 2001; 18:25–32. DOI: 10.1097/00004691-200101000-00006. PMID: 11290936.

11. Cooperman LH, Strobel GE Jr, Kennell EM. Massive hyperkalemia after administration of succinylcholine. Anesthesiology. 1970; 32:161–4. DOI: 10.1097/00000542-197002000-00017. PMID: 5414294.

12. Renier WO, Gabreëls FJ, Hustinx TW, Jaspar HH, Geelen JA, Van Haelst UJ, et al. Connatal pelizaeus-merzbacher disease with congenital stridor in two maternal cousins. Acta Neuropathol. 1981; 54:11–7. DOI: 10.1007/BF00691328. PMID: 7234326.

13. Saugier-Veber P, Munnich A, Bonneau D, Rozet JM, Le Merrer M, Gil R, et al. X-linked spastic paraplegia and pelizaeus-merzbacher disease are allelic disorders at the proteolipid protein locus. Nat Genet. 1994; 6:257–62. DOI: 10.1038/ng0394-257. PMID: 8012387.

14. Arroyo EJ, Xu T, Grinspan J, Lambert S, Levinson SR, Brophy PJ, et al. Genetic dysmyelination alters the molecular architecture of the nodal region. J Neurosci. 2002; 22:1726–37. DOI: 10.1523/JNEUROSCI.22-05-01726.2002. PMID: 11880502.

15. Yamamoto T, Shimojima K. Pelizaeus-merzbacher disease as a chromosomal disorder. Congenit Anom (Kyoto). 2013; 53:3–8. DOI: 10.1111/cga.12005. PMID: 23480352.