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May 2016

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

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

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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