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

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

Robert H Fryer  1 , Jennifer M Bain  2 , Darryl C De Vivo  2
Affiliations
Case Reports

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

Robert H Fryer et al. Pediatr Neurol. 2016 Mar.

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.

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Conflict of interest statement

Potential conflicts of interest: None (RF, JB and DD).

References

    1. Hirano M, Pavlakis SG. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS): current concepts. J Child Neurol. 1994;9(1):4–13. - PubMed
    1. Pavlakis SG, Phillips PC, DiMauro S, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndrome. Annals of Neurology. 1984;16(4):481–488. - PubMed
    1. Goto Y, Nonaka I, Horai S. A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature. 1990;348(6302):651–653. - PubMed
    1. DiMauro S, Hirano M. Melas. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews(R) Seattle (WA): 1993.
    1. Gold DR, Cohen BH. Treatment of mitochondrial cytopathies. Seminars in Neurology. 2001;21(3):309–325. - PubMed

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