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Tangier disease. A case with sensorimotor distal polyneuropathy and lipid accumulation in striated muscle and vasa nervorum.
- Source :
-
Acta neuropathologica [Acta Neuropathol] 1985; Vol. 67 (1-2), pp. 121-7. - Publication Year :
- 1985
-
Abstract
- A 65-year-old man with Tangier disease (analphalipoproteinemia) had had a progressive sensorimotor distal neuropathy with sensory ataxia for 1 year. Muscle biopsy demonstrated excess lipid vacuoles on histochemical and electron-microscopic techniques. Sural nerve biopsy showed a marked loss of large fibers and an increase in small myelinated fibers, with presence of remyelinating fibers and clusters of regeneration; a few aspects of active demyelination and some onion-like formations were also present. Lipid accumulation chiefly affected the Schwann cells of unmyelinated fibers and, to a lesser degree, of myelinated fibers, endoneurial fibroblast, and vasa nervorum. Teased fibers showed prevalent aspects of de-/remyelination and, often in association, marked myelin wrinkling suggesting axonal atrophy. This Tangier patient differs from known cases for the presence of a distal symmetrical sensorimotor polyneuropathy (not previously reported in Tangier disease) and because of the morphological findings of de-/remyelination coexisting with aspects of axonal atrophy and previous degeneration, and of lipid accumulation within striated muscle and vasa nervorum. This latter finding contrasts with the assumption that in Tangier disease vessel walls are not a site of lipid storage: probably the vasa nervorum are different, in this respect, from other vessels, because of the intense lipid metabolism of the nervous tissue. Thus we suggest that involvement of vasa nervorum in Tangier disease may be more important than previously suspected, possibly playing a role in the causation of neuropathy.
- Subjects :
- Aged
Biopsy
Humans
Male
Microscopy, Electron
Muscles pathology
Peripheral Nervous System Diseases pathology
Sural Nerve pathology
Sural Nerve ultrastructure
Tangier Disease metabolism
Tangier Disease pathology
Blood Vessels metabolism
Hypolipoproteinemias complications
Lipid Metabolism
Muscles metabolism
Peripheral Nervous System Diseases complications
Tangier Disease complications
Vasa Nervorum metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0001-6322
- Volume :
- 67
- Issue :
- 1-2
- Database :
- MEDLINE
- Journal :
- Acta neuropathologica
- Publication Type :
- Academic Journal
- Accession number :
- 2992211
- Full Text :
- https://doi.org/10.1007/BF00688132