کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
3063660 | 1187527 | 2006 | 6 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Paranodal pathology in Tangier disease with remitting-relapsing multifocal neuropathy Paranodal pathology in Tangier disease with remitting-relapsing multifocal neuropathy](/preview/png/3063660.png)
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.
Journal: Journal of Clinical Neuroscience - Volume 13, Issue 4, May 2006, Pages 492–497