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General Anesthesia for a Patient With Pelizaeus-Merzbacher Disease

Nobuhito Kamekura1, Yukie Nitta2, Shigeru Takuma2

  • 1Associate Professor.

Anesthesia Progress
|June 9, 2016
PubMed

Insights

General anesthesia was successfully administered to a patient with Pelizaeus-Merzbacher disease (PMD), a rare white matter disorder. This case highlights safe anesthetic management strategies for patients with PMD, minimizing risks like seizures and spasticity.

Area of Science:

  • Neurology
  • Anesthesiology

Background:

  • Pelizaeus-Merzbacher disease (PMD) is a rare, progressive, degenerative disorder of the cerebral white matter.
  • Clinical manifestations include psychomotor retardation, nystagmus, abnormal muscle tone, seizures, and cognitive impairment.
  • General anesthesia in PMD patients presents challenges due to potential seizures, airway complications from poor pharyngeal muscle control, and exacerbation of spasticity.

Observation:

  • A 20-year-old male patient with PMD underwent impacted wisdom teeth removal under general anesthesia.
  • Anesthesia was induced with thiamylal, fentanyl, and desflurane, and maintained with desflurane and continuous intravenous remifentanil.
  • Monitoring included bispectral index and train-of-4, with anesthesia lasting 1 hour and 20 minutes.

Findings:

  • The general anesthesia procedure was completed uneventfully.
  • The patient experienced no postoperative airway complications, seizures, or exacerbation of spasticity.
  • Preoperative assessment revealed no history of epilepsy, aspiration pneumonia, or gastroesophageal reflux disease.

Implications:

  • This case demonstrates the feasibility of safe general anesthesia management in patients with Pelizaeus-Merzbacher disease.
  • Careful preoperative evaluation of associated conditions and risk factors is crucial for successful anesthetic outcomes.
  • The findings underscore the importance of vigilance for potential complications such as spasticity exacerbation during perioperative care.
Keywords:
BISBody temperatureDesfluraneGeneral anesthesiaPelizaeus-Merzbacher disease

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