Ultrastructure of peripheral nerve in metachromatic leucodystrophy

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ID: 112986
1970
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Abstract
An electron microscopical study of two consecutive nerve biopsies from a patient with metachromatic leucodystrophy (sulphatide lipidosis) was made. The ultrastructural changes observed consisted of: a) irregular whorls of myelin. The myelin in the whorls showed a thickened, sometimes doubled, intraperiod line, which was barely visible in compact myelin; b) “inclusion bodies” up to 1 μ in diameter in the cytoplasm of Schwann cells. These had a lamellar structure, with stacked membranes 60 Å apart; c) a “loose” pattern of the myelin in some nerve fibers, with loss of the intraperiod line, and d) presence of abnormally dense mitochondria with thickened cristae in Schwann cells. It is suggested that: a) the whorl formation and the ultrastructural abnormalities of the myelin in the whorls may be due to impaired myelin synthesis, and b) that the “inclusion bodies” may represent the accumulation of cerebroside sulfate in micellar aggregates. The “loose” pattern of myelin is considered artifactural until proven otherwise.
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cravioto1970actaultrastructure Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Humberto Cravioto;John S. O'Brien;Benjamin H. Landing;Barbara Finck;Humberto Cravioto;John S. O'Brien;Benjamin H. Landing;Barbara Finck;
Journal acta neuropathologica
Year 1970
DOI
doi:10.1007/BF00686778
URL
https://link.springer.com/article/10.1007/BF00686778
Keywords
pathology neurosciences

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