Robert H Fryer1, Jennifer M Bain2, Darryl C De Vivo2. 1. Department of Neurology, Columbia University Medical Center, New York, New York. Electronic address: rf203@cumc.columbia.edu. 2. Department of Neurology, Columbia University Medical Center, New York, New York.
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.
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.
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