title: Recommendations for the Management of Strokelike Episodes in Patients With Mitochondrial Encephalomyopathy, Lactic Acidosis, and Strokelike Episodes.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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
  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.
authors.affiliation: Departments of Pediatrics and Neurology, The University of Texas Medical School at Houston, Houston.
authors.forename: Mary Kay
authors.initials: MK
authors.lastname: Koenig
authors.affiliation: Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston.
authors.forename: Lisa
authors.initials: L
authors.lastname: Emrick
authors.affiliation: Department of Genetics and Metabolism, Massachusetts General Hospital, Harvard Medical School, Boston.
authors.forename: Amel
authors.initials: A
authors.lastname: Karaa
authors.affiliation: Genetic Metabolic Center for Education, Salem, Massachusetts.
authors.forename: Mark
authors.initials: M
authors.lastname: Korson
authors.affiliation: Department of Molecular and Human Genetics, Baylor College of Medicine and Texas Children's Hospital, Houston.
authors.forename: Fernando
authors.initials: F
authors.lastname: Scaglia
authors.affiliation: Center for Pediatric Neurology, Neurosciences Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Sumit
authors.initials: S
authors.lastname: Parikh
authors.affiliation: Department of Pediatrics, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
authors.forename: Amy
authors.initials: A
authors.lastname: Goldstein
mesh_terms: D019468:Disease Management; D006801:Humans; D017241:MELAS Syndrome / Q000175:diagnosis* / Q000628:therapy*