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2014 Fiscal Year Final Research Report

Breakdown of Endoneurial Endothelial Cell Tight Junctions at the Site of Conduction Block in Mutifocal Motor Neuropathy

Research Project

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Project/Area Number 25870472
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Human pathology
Neurology
Research InstitutionYamaguchi University

Principal Investigator

OMOTO Masatoshi  山口大学, 医学部附属病院, 助教 (40464333)

Project Period (FY) 2013-04-01 – 2015-03-31
Keywords多巣性運動ニューロパチー / 血液神経関門
Outline of Final Research Achievements

To define whether peripheral nerve of multifocal motor neuropathy (MMN) has blood-nerve barrier (BNB) breakdown, and whether BNB breakdown is present adjacent to the site of motor conduction block (CB) in MMN, we investigated ultrastructural changes in endoneurial endothelial cells in biopsy specimens of peripheral nerve obtained from patients of MMN. In one patient, biopsy of peripheral nerve was performed after detection of a focal CB, and sural nerve biopsy specimens were obtained. The percentage of endothelial cell junctions (that make up the BNB) that showed evidence of disruption were evaluated.
Percentage of BNB breakdown was increased in the peripheral nerve adjacent to the site of CB, whereas there was no BNB breakdown in sural nerve specimens. We conclude that focal insufficiency of BNB may evoke local leakage of humoral factors in the motor nerves of MMN patients, and this would be the background pathology of scattered CB and clinical phenotype in this rare disorder.

Free Research Field

末梢神経

URL: 

Published: 2016-06-03  

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