Disruption of Endothelial Tight Junctions in a Patient with Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes (MELAS)

M. Matsuzaki; R. Takahashi; T. Nakayama; K. Shishikura; H. Suzuki; Y. Hirayama; M. Osawa; H. Oda

doi:10.1055/s-0030-1261886

Neuropediatrics, Table of Contents

Neuropediatrics 2010; 41(2): 72-74
DOI: 10.1055/s-0030-1261886

Short Communication

Disruption of Endothelial Tight Junctions in a Patient with Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes (MELAS)

M. Matsuzaki¹ ^, ³ , R. Takahashi¹ , T. Nakayama¹ , K. Shishikura¹ , H. Suzuki¹ , Y. Hirayama¹ , M. Osawa¹ , H. Oda²

¹Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan
²Department of Pathology, Tokyo Women's Medical University, Tokyo, Japan
³Nakagawa-No-Sato, Hospital for the Disabled, Saitama, Japan

Abstract

An electron microscopic study revealed disruption of capillary endothelial tight junctions (TJs) in both biopsied muscle, taken at 5 years and 1 month of age, and the autopsied brain, taken at 13 years and 6 months of age, in a patient with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) and mitochondrial DNA (mtDNA) point mutation A3243G. This endothelial barrier disruption might result in vasogenic edema and systemic lactic acidosis, possibly the critical pathology of MELAS.

Key words

mitochondrial encephalomyopathy - lactic acidosis and stroke-like episodes (MELAS) - tight junctions - disruption of endothelial tight junctions

Full Text

References

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Correspondence

Mihoko Matsuzaki

Department of Pediatrics

Tokyo Women's Medical University

8-1 Kawada-cho

Shinjuku-ku

162-8666 Tokyo

Japan

Phone: +81/3/3353 8111

Email: mi.matsu@ped.twmu.ac.jp