J Korean Neurol Assoc.  2023 Nov;41(4):306-309. 10.17340/jkna.2023.0030.

An Acute Metabolic Encephalopathy, Lactic Acidosis and Stroke-like Episodes Syndrome Patients with Hyperperfusion Responsive to Steroid Treatment

Affiliations
  • 1Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul Korea
  • 2Department of Neurology, Gachon University Gil Medical Center, Incheon, Korea

Abstract

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a genetic disorder caused by mutation in mitochondrial DNA. Patients with stroke-like episodes show restrictive lesions in diffusion weighted image, whereas magnetic resonance angiography images show both vasodilation or vasoconstriction. Vasodilation may lead to hyperperfusion and cerebral edema, which may worsen clinical outcome. Here, we report a 25-year-old male patient diagnosed as MELAS, who presented with stroke-like episodes and seizures and showed cerebral edema with vasodilation which was normalized after steroid treatment.

Keyword

MELAS syndrome; Cerebral blood flow; Steroids
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