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Recommendations for the Management of Strokelike Episodes in Patients With Mitochondrial Encephalomyopathy, Lactic Acidosis, and Strokelike Episodes

Mary Kay Koenig, MD1; Lisa Emrick, MD2; Amel Karaa, MD3; Mark Korson, MD4; Fernando Scaglia, MD5; Sumit Parikh, MD6; Amy Goldstein, MD7
[+] Author Affiliations
1Departments of Pediatrics and Neurology, The University of Texas Medical School at Houston, Houston
2Department of Pediatrics, Baylor College of Medicine and Texas Children’s Hospital, Houston
3Department of Genetics and Metabolism, Massachusetts General Hospital, Harvard Medical School, Boston
4Genetic Metabolic Center for Education, Salem, Massachusetts
5Department of Molecular and Human Genetics, Baylor College of Medicine and Texas Children’s Hospital, Houston
6Center for Pediatric Neurology, Neurosciences Institute, Cleveland Clinic, Cleveland, Ohio
7Department of Pediatrics, Children’s Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
JAMA Neurol. 2016;73(5):591-594. doi:10.1001/jamaneurol.2015.5072.
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Importance  Strokelike episodes are a cardinal feature of several mitochondrial syndromes, including mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS). Recent advances in the understanding of the pathophysiologic mechanisms of strokelike episodes in MELAS have led to improved treatment options.

Observations  Current understanding of the cause of strokelike episodes in MELAS and present recommendations to assist in the identification and treatment of patients with MELAS who present with stroke are presented. Mounting evidence points toward a benefit of the nitric oxide precursors, arginine, to both prevent and reduce the severity of strokes in patients with MELAS.

Conclusions and Relevance  Although much information is still needed regarding the appropriate dosing and timing of arginine therapy in patients with MELAS, urgent administration of nitric oxide precursors in patients with MELAS ameliorates the clinical symptoms associated with strokelike episodes.

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Figure 1.
Magnetic Resonance Imaging in a Patient With Mitochondrial Encephalomyopathy, Lactic Acidosis, and Strokelike Episodes (MELAS) vs Ischemic Stroke

Top, Fluid-attenuated inversion recovery (left) and diffusion-weighted (right) magnetic resonance brain images of a patient with MELAS showing the patchy abnormalities in multiple regions of the cortical tissue. Bottom, Fluid-attenuated inversion recovery (left) and diffusion-weighted (right) magnetic resonance brain images of a patient with typical ischemic stroke showing abnormalities only in the right middle cerebral artery territory.

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Figure 2.
Nitric Oxide Metabolism in the Vascular Endothelium

Endothelium-dependent vascular relaxation is mediated by nitric oxide synthase (NOS), which converts l-arginine to nitric oxide (NO). The NO binds to soluble guanylate cyclase (sGC), converting guanosine triphosphate (GTP) to cyclic guanosine monophosphate (cGMP) and relaxing the smooth muscle cells and dilating the blood vessel. Cyt-C indicates cytochrome c.

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