Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-Like Episodes (MELAS): A Case Report and Critical Reappraisal of Treatment Options

Pediatr Neurol. 2016 Mar:56:59-61. doi: 10.1016/j.pediatrneurol.2015.12.010. Epub 2015 Dec 19.

Abstract

Importance: Stroke-like episodes signal progression and significant disability in the mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes syndrome. Arginine is widely used as a treatment for stroke-like episode, although there is little evidence for this intervention. We discuss the management of a patient with mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes who presented with a stroke-like episode.

Observation: During a seizure, which triggers the stroke-like episode, neurons are forced to utilize glycolysis as a source of adenosine triphosphate. Glycolytic by-products are damaging to the neuron. Breakdown of the blood-brain barrier leads to vasogenic edema.

Conclusion: Treatment of stroke-like episode should include anticonvulsants interictally to prevent seizures and dexamethasone ictally to help repair the blood-brain barrier.

Keywords: MELAS; arginine; aspirin; glycolysis; lactic acidosis; mitochondria; seizures; stroke-like episode.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Anticonvulsants / therapeutic use
  • Child
  • Dexamethasone / therapeutic use
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • MELAS Syndrome / diagnosis*
  • MELAS Syndrome / therapy*

Substances

  • Anticonvulsants
  • Glucocorticoids
  • Dexamethasone