General Anesthesia for a Patient With Pelizaeus-Merzbacher Disease

Abstract

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.

Epidural Anesthesia and Continuous Epidural Analgesia in a Pediatric Patient With Pelizaeus-Merzbacher Disease: A Case Report

“…There are no restrictions on the use of anesthetic agents and analgesics in general anesthesia in patients with PMD [8]. However, suxamethonium can cause unpredictable reactions such as hyperkalemia and should be avoided in rapid induction [4][5][6][7][8]. Considerations in anesthesia should include efforts to prevent complications associated with abnormal muscle tone due to spasticity, airway complications due to decreased pharyngeal muscle strength, aspiration, and convulsions.…”

“…The frequency in Europe and the United States is reported to be one in 200,000 to 500,000 live births and the number of patients in Japan is estimated to be about 200 [3]. Because the incidence of PMD is extremely low, only a few case reports have been published regarding its anesthetic management [4][5][6][7][8]. In particular, epidural anesthesia has only been reported in one case of general anesthesia combined with caudal anesthesia [5].…”

Síndrome de Pelizaeus Merzbacher: breve revisión de la literatura

“…El retraso psicomotor es evidente a los 6 meses, la ataxia en extremidades puede ser aparente y agravarse durante los años, la hipotonía de extremidades es remplazada por espasticidad, las posturas distónicas y los movimientos coreicos pueden estar presentes, el compromiso cognitivo se manifiesta en la mayoría de los niños 5,12 . Existiendo también riesgo para la anestesia general que puede correlacionarse con complicaciones por crisis y dificultades de la vía aérea por pobre control de los músculos faríngeos, así como la exacerbación de la espasticidad 13 .…”

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

“…Thus, the clinical symptoms of PMD arise from widespread hypomyelination of the nervous system and include developmental motor disability, ataxia, choreoathetosis, and cognitive impairment. In addition, an anesthesiologist must consider the possibility of abnormal muscle tone, such as spasticity, airway complications due to pharyngeal muscle weakness, and the risks of aspiration and seizure.Only a few case reports have been published on the anesthetic management of patients with PMD because of its low prevalence [1], which is about 0.13 or 1.45 per 100,000 live births [2,3]. Here we present the case of a 13-year-old girl with PMD who underwent elective ophthalmic surgery under general anesthesia.…”

“…Only a few case reports have been published on the anesthetic management of patients with PMD because of its low prevalence [1], which is about 0.13 or 1.45 per 100,000 live births [2,3]. Here we present the case of a 13-year-old girl with PMD who underwent elective ophthalmic surgery under general anesthesia.…”