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Paranodal pathology in Tangier disease with remitting-relapsing multifocal neuropathy.
- 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.
- Subjects :
- Adolescent
Humans
Immunohistochemistry methods
Lipid Metabolism
Male
Microscopy, Electron, Transmission methods
Myelin Proteins metabolism
Myelin Sheath pathology
Myelin Sheath ultrastructure
Neurofilament Proteins metabolism
Peripheral Nervous System Diseases metabolism
Ranvier's Nodes diagnostic imaging
Sural Nerve pathology
Sural Nerve ultrastructure
Tangier Disease metabolism
Ultrasonography
Peripheral Nervous System Diseases complications
Peripheral Nervous System Diseases pathology
Ranvier's Nodes pathology
Tangier Disease complications
Tangier Disease pathology
Subjects
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