{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T03:52:52Z","timestamp":1775793172249,"version":"3.50.1"},"reference-count":16,"publisher":"American Dental Society of Anesthesiology (ADSA)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016]]},"abstract":"<jats:p>We report the successful management of general anesthesia for a patient with Pelizaeus-Merzbacher disease (PMD). PMD is one of a group of progressive, degenerative disorders of the cerebral white matter. The typical clinical manifestations of PMD include psychomotor retardation, nystagmus, abnormal muscle tone, seizures, and cognitive impairment. General anesthesia for a patient with PMD may be difficult mainly because of seizures and airway complications related to poor pharyngeal muscle control. In addition, the possibility of exacerbation of spasticity should be considered. A 20-year-old man with PMD required removal of impacted wisdom teeth under general anesthesia. General anesthesia was induced with thiamylal, fentanyl, and desflurane. Anesthesia was maintained with desflurane and continuous intravenous remifentanil under bispectral index and train-of-4 monitoring. Anesthesia lasted 1 hour 20 minutes and was completed uneventfully. Airway complications, seizures, and exacerbation of spasticity did not occur postoperatively. Preoperatively, our patient had no history of epilepsy attacks or aspiration pneumonia, and no clinical symptoms of gastroesophageal reflux disease. Therefore, exacerbation of spasticity was one of the most likely potential complications. Identification of these associated conditions and evaluation of risk factors during preoperative examination is important for performing safe anesthesia in these patients.<\/jats:p>","DOI":"10.2344\/15-00022.1","type":"journal-article","created":{"date-parts":[[2016,6,6]],"date-time":"2016-06-06T22:05:52Z","timestamp":1465250752000},"page":"91-94","source":"Crossref","is-referenced-by-count":5,"title":["General Anesthesia for a Patient With Pelizaeus-Merzbacher Disease"],"prefix":"10.2344","volume":"63","author":[{"given":"Nobuhito","family":"Kamekura","sequence":"first","affiliation":[{"name":"Associate Professor,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yukie","family":"Nitta","sequence":"additional","affiliation":[{"name":"Assistant Professor, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeru","family":"Takuma","sequence":"additional","affiliation":[{"name":"Assistant Professor, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiaki","family":"Fujisawa","sequence":"additional","affiliation":[{"name":"Professor, Department of Dental Anesthesiology, Graduate School of Dental Medicine, Hokkaido University, Sapporo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1191","reference":[{"key":"i0003-3006-63-2-91-b01","series-title":"Congenit Anom (Kyoto)","first-page":"3","article-title":"Pelizaeus-Merzbacher disease as a chromosomal disorder","volume":"53","author":"Yamamoto \n              T,","year":"2013","unstructured":"Yamamoto \n              T, \n            \n            \n              Shimojima \n              K. \n            \n          \n          Pelizaeus-Merzbacher disease as a chromosomal disorder. 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J Neurol. 2014;261:752\u2013758."},{"key":"i0003-3006-63-2-91-b03","series-title":"Semin Neurol","first-page":"62","article-title":"Pelizaeus-Merzbacher disease, Pelizaeus-Merzbacher-like disease 1, and related hypomyelinating disorders","volume":"32","author":"Hobson \n              GM,","year":"2012","unstructured":"Hobson \n              GM, \n            \n            \n              Garbern \n              JY. \n            \n          \n          Pelizaeus-Merzbacher disease, Pelizaeus-Merzbacher-like disease 1, and related hypomyelinating disorders. Semin Neurol. 2012;32:62\u201367."},{"key":"i0003-3006-63-2-91-b04","series-title":"Pagon RA, Adam MP, Ardinger HH, et al, eds. GeneReviews","article-title":"PLP1-related disorders","author":"Hobson \n              GM,","year":"2013","unstructured":"Hobson \n              GM, \n            \n            \n              Kamholz \n              J. \n            \n          \n          PLP1-related disorders. 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Brain. 1998;121:243\u2013252."},{"key":"i0003-3006-63-2-91-b15","series-title":"Mov Disord","first-page":"963","article-title":"Management of status dystonicus: our experience and review of the literature","volume":"22","author":"Mariotti \n              P,","year":"2007","unstructured":"Mariotti \n              P, \n            \n            \n              Fasano \n              A, \n            \n            \n              Contarino \n              MF, \n            \n            et al. \n          Management of status dystonicus: our experience and review of the literature. Mov Disord. 2007;22:963\u2013968."},{"key":"i0003-3006-63-2-91-b16","series-title":"J Clin Neurophysiol","first-page":"25","article-title":"Epileptic seizures and electroencephalographic evolution in genetic leukodystrophies","volume":"18","author":"Wang \n              PJ,","year":"2001","unstructured":"Wang \n              PJ, \n            \n            \n              Hwu \n              WL, \n            \n            \n              Shen \n              YZ. \n            \n          \n          Epileptic seizures and electroencephalographic evolution in genetic leukodystrophies. J Clin Neurophysiol. 2001;18:25\u201332."}],"container-title":["Anesthesia Progress"],"original-title":[],"link":[{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/view\/journals\/anpr\/63\/2\/article-p91.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/meridian.allenpress.com\/anesthesia-progress\/article-pdf\/63\/2\/91\/2100790\/15-00022_1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/downloadpdf\/journals\/anpr\/63\/2\/article-p91.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/downloadpdf\/journals\/anpr\/63\/2\/article-p91.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,7]],"date-time":"2024-10-07T12:35:02Z","timestamp":1728304502000},"score":1,"resource":{"primary":{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/view\/journals\/anpr\/63\/2\/article-p91.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016]]},"references-count":16,"journal-issue":{"issue":"2"},"URL":"https:\/\/doi.org\/10.2344\/15-00022.1","relation":{},"ISSN":["0003-3006","1878-7177"],"issn-type":[{"value":"0003-3006","type":"print"},{"value":"1878-7177","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016]]}}}