Anesthetic Implications in Alternating Hemiplegia of Childhood: A Case Report

AANA J. 2022 Aug;90(4):297-302.

Abstract

The following case report describes a 13-year-old child with alternating hemiplegia of childhood (AHC) who underwent magnetic resonance imaging MRI with general anesthesia and experienced a hemiplegic spell, seizure, apnea, and sudden cardiac arrest with successful resuscitation. AHC is a rare neurodevelopmental disorder characterized by repeated episodes of weakness or paralysis affecting one or both sides of the body and multiple other neurologic problems. The challenges associated with this disorder include management of developmental delay, dystonia, hemiplegia, cerebrovascular dysfunction, apnea, and autonomic dysfunction. The current literature is extremely limited in describing the effects of general anesthesia for a patient with AHC. While the neurologic manifestations of AHC are well described, autonomic dysfunction and the potential for sudden cardiac arrest have not been widely reported. To our knowledge, this is the first case report to emphasize anesthetic considerations in a pediatric patient with AHC, specifically the unrecognized potential for cardiac arrhythmia and sudden cardiac arrest.

Keywords: ATP1A3; Alternating hemiplegia of childhood; anesthesia; pediatrics; short QTc.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anesthetics*
  • Apnea
  • Child
  • Death, Sudden, Cardiac
  • Hemiplegia*
  • Humans
  • Mutation
  • Sodium-Potassium-Exchanging ATPase / genetics

Substances

  • Anesthetics
  • Sodium-Potassium-Exchanging ATPase

Supplementary concepts

  • Alternating hemiplegia of childhood