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

© Georg Thieme Verlag KG Stuttgart · New York

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
Weitere Informationen

Publikationsverlauf

received 10.02.2010

accepted 10.06.2010

Publikationsdatum:
26. August 2010 (online)

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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

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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

Telefon: +81/3/3353 8111

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