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Paranodal pathology in Tangier disease with remitting-relapsing multifocal neuropathy.

Authors :
Cai Z
Blumbergs PC
Cash K
Rice PJ
Manavis J
Swift J
Ghabriel MN
Thompson PD
Source :
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia [J Clin Neurosci] 2006 May; Vol. 13 (4), pp. 492-7.
Publication Year :
2006

Abstract

Pathological studies of a sural nerve biopsy in a man with Tangier disease presenting as a remitting-relapsing multifocal neuropathy showed abnormalities in the paranodal regions, including lipid deposition (65%) and redundant myelin foldings, with various degrees of myelin splitting and vesiculation (43%) forming small tomacula and abnormal myelin terminal loops (4%). The internodal regions were normal in the majority of myelinated fibres. Abnormal lipid storage was also present in the Schwann cells of the majority of unmyelinated fibres (67%). The evidence suggests that the noncompacted myelin region of the paranode is a preferential site for lipid storage in the myelinated Schwann cell, and that the space-occupying effects of the cholesterol esters leads to paranodal malfunction and tomacula formation as the pathological basis for the multifocal relapsing-remitting clinical course.

Details

Language :
English
ISSN :
0967-5868
Volume :
13
Issue :
4
Database :
MEDLINE
Journal :
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
Publication Type :
Academic Journal
Accession number :
16678735
Full Text :
https://doi.org/10.1016/j.jocn.2005.07.009