MELAS and Reversible Vasoconstriction of the Major Cerebral Arteries

Takeshi Yoshida; Atsushi Ouchi; Daisuke Miura; Kunihiro Shimoji; Kazushi Kinjo; Takeshi Sueyoshi; Manabu Jonosono; Vijay Rajput

doi:10.2169/internalmedicine.52.0188

CASE REPORTS

MELAS and Reversible Vasoconstriction of the Major Cerebral Arteries

Takeshi Yoshida, Atsushi Ouchi, Daisuke Miura, Kunihiro Shimoji, Kazushi Kinjo, Takeshi Sueyoshi, Manabu Jonosono, Vijay Rajput

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Keywords: stroke-like episode, mitochondrial encephalopathy, lactic acidosis, L-arginine, reversible cerebral vasoconstriction syndrome

JOURNAL OPEN ACCESS

2013 Volume 52 Issue 12 Pages 1389-1392

DOI https://doi.org/10.2169/internalmedicine.52.0188

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Abstract

A 26-year-old woman was admitted due to an altered mental status and generalized tonic-clonic seizures. She had experienced chronic migraine-like headaches, progressive bilateral hearing loss, a short stature and nephrotic syndrome. Laboratory data showed elevated lactate and pyruvate levels. Brain MRI using diffusion-weighted imaging revealed a hyperintense lesion in the left temporal lobe. MR angiography revealed segmental stenosis at the C1 and M1-2 junction. A genetic study revealed a mitochondrial DNA A3243G point mutation. The patient's clinical symptoms and MRI/MR angiography (MRA) findings improved within four weeks. We herein discuss the possible pathophysiology involving both stroke-like episodes and reversible vasoconstriction.

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