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

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• 作者：Robert H. Fryer ; MD ; PhD ; ^{rf203@cumc.columbia.edu" class="auth_mail" title="E-mail the corresponding author} ; Jennifer M. Bain ; MD ; PhD ; Darryl C. De Vivo ; MD
• 关键词：stroke-like episode ; MELAS ; aspirin ; seizures ; glycolysis ; arginine ; mitochondria ; lactic acidosis
• 刊名：Pediatric Neurology
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：56
• 期：Complete
• 页码：59-61
• 全文大小：187 K

文摘

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.