General anesthetic use in fragile X spectrum disorders

Andrew Ligsay, Marwa El-Deeb, Maria J. Salcedo-Arellano, Nina Schloemerkemper, Jeremy S. Grayson, Randi Hagerman

Research output: Contribution to journalReview articlepeer-review

4 Scopus citations

Abstract

The fragile X premutation is characterized by a repeat expansion mutation (between 55 to 200 CGG repeats) in the fragile X mental retardation 1 (FMR1) gene, which leads to RNA toxicity at the cellular level. This may cause patients with the premutation to be particularly susceptible to environmental toxins, which could manifest clinically as new or worsening ataxia and memory loss. Multiple published case reports have also suggested general anesthetics as a potential toxin leading to negative side effects when used in patients with fragile X-associated disorders. However, at this time, there have been no formal research studies regarding cellular changes or long-term clinical manifestations after general anesthetic use in this population. This review aims to highlight previous case reports regarding sequelae related to general anesthetic use in fragile X-associated disorders. New case reports related to this phenomenon are also included.

Original languageEnglish (US)
Pages (from-to)285-290
Number of pages6
JournalJournal of Neurosurgical Anesthesiology
Volume31
Issue number3
DOIs
StatePublished - Jul 1 2019

Keywords

  • FMR1 mRNA
  • fragile X syndrome
  • FXTAS
  • general anesthesia
  • POCD
  • postoperative cognitive dysfunction

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology
  • Anesthesiology and Pain Medicine

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