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  • Title: [Peripheral nerve changes in Tangier disease. Comparative light-, electron-microscopic and cytometric studies (author's transl)].
    Author: Hager H, Zimmermann P.
    Journal: Acta Neuropathol; 1979 Jan 12; 45(1):53-9. PubMed ID: 216226.
    Abstract:
    A sural nerve biopsy was performed in a 55-year-old male patient with Tangier disease (familial-lipoprotein deficiency). Light-microscopy showed an increase in the endoneural connective tissue and a loss of nerve fibers indicating a chronic peripheral neuropathy. Electron-microscopy revealed an accumulation of lipid droplets within Schwann cells of myelinated and unmyelinated nerve fibers. When compared with age-matched controls the myelinated fiber density was reduced with a relative preponderance of small myelinated fibers. In addition, distributional cytometric studies of nerve fibers in relation to the perineurium and endoneurial capillaries showed: Contrary to 4.6-7.5 micron thick nerve fibers, which accumulated in the center of the nerve fascicle, small (0.5-4.5 micron) and large (7.6-10.0 micron) fibers lay nearby the perineurium. The measured increase in small myelinated nerve fibers around endoneurial capillaries may be explained as a sign of regeneration.
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