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Progressive encephalomyelitis with rigidity and myoclonus: anesthesia and glycine receptor antibodies

Aikaterini Papadopoulou1, Theophilus Luke Samuels, Asitha Dassanayake

  • 1From the Department of Anesthesia, Worthing Hospital, Worthing, United Kingdom.

A & a Case Reports
|January 23, 2015
PubMed

Insights

In patients with glycine receptor (GlyR) antibody-associated Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM), sevoflurane and nitrous oxide anesthesia did not show altered effects. This study suggests anesthetic management may be standard for PERM patients with GlyR antibodies.

Area of Science:

  • Neurology
  • Immunology
  • Anesthesiology

Background:

  • Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare autoimmune neurological disorder.
  • Antibodies targeting glycine receptors (GlyR) are implicated in the pathophysiology of PERM.
  • GlyR activity is modulated by inhaled anesthetics, raising theoretical concerns about anesthetic efficacy in PERM patients.

Purpose of the Study:

  • To report on the anesthetic management of a patient with PERM and GlyR antibodies.
  • To investigate the potential impact of GlyR antibodies on the efficacy of general anesthesia.

Main Methods:

  • Case report of general anesthesia in a patient diagnosed with PERM and confirmed GlyR antibodies.
  • Anesthesia was induced with sevoflurane and maintained with sevoflurane and nitrous oxide.
  • Clinical observation of the patient's response to anesthetic agents.

Main Results:

  • The patient with PERM and GlyR antibodies did not exhibit clinically significant alterations in anesthetic requirements or response.
  • Induction and maintenance of anesthesia with sevoflurane and nitrous oxide were uneventful.
  • No unexpected or exaggerated anesthetic effects were observed.

Conclusions:

  • General anesthesia with sevoflurane and nitrous oxide appears safe and effective in patients with PERM and GlyR antibodies.
  • The theoretical concern regarding altered anesthetic efficacy due to GlyR antibodies may not be clinically significant.
  • Further studies are warranted to confirm these findings in a larger cohort.

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