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JAMA Neurol. 2016 May 1;73(5):591-4. doi: 10.1001/jamaneurol.2015.5072.

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

Author information

1
Departments of Pediatrics and Neurology, The University of Texas Medical School at Houston, Houston.
2
Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston.
3
Department of Genetics and Metabolism, Massachusetts General Hospital, Harvard Medical School, Boston.
4
Genetic Metabolic Center for Education, Salem, Massachusetts.
5
Department of Molecular and Human Genetics, Baylor College of Medicine and Texas Children's Hospital, Houston.
6
Center for Pediatric Neurology, Neurosciences Institute, Cleveland Clinic, Cleveland, Ohio.
7
Department of Pediatrics, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

Abstract

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.

PMID:
26954033
DOI:
10.1001/jamaneurol.2015.5072
[Indexed for MEDLINE]

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