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

Pediatric Neurology
Robert H FryerD C De Vivo

Abstract

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

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Citations

Apr 12, 2016·International Journal of Cardiology·Josef Finsterer, Sinda Zarrouk-Mahjoub
Jul 12, 2016·Annals of Neurology·Josef Finsterer, Sinda Zarrouk-Mahjoub
Feb 3, 2018·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Josef Finsterer, Elmano Henrique Torres de Carvalho
Nov 8, 2017·Journal of Inherited Metabolic Disease·Han Som ChoiYoung-Mock Lee
Nov 7, 2019·Current Opinion in Clinical Nutrition and Metabolic Care·Masamichi IkawaYasutoshi Koga
Jul 30, 2018·Acta neurologica Belgica·Josef Finsterer, Sinda Zarrouk-Mahjoub
Nov 29, 2016·Case Reports in Cardiology·Sara SeitunAlberto Valbusa
Jan 20, 2021·The Journal of Clinical Investigation·Rohit SharmaVamsi K Mootha
Feb 11, 2021·Nutrients·Camila D S BarrosCelia Harumi Tengan
Nov 4, 2021·BMJ Case Reports·Dearbhail Ni CathainKarl Boyle

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