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Nosology of the leukodystrophies: ultrastructural contributions.
Birth Defects Orig Artic Ser. 1971 Feb; 7(1):113-6.BD

Abstract

Ultrastructural aspects of some of the conditions usually included as "leukodystrophies" are described. In metachromatic leukodystrophy, the most striking ultrastructural features include a variety of inclusions within cells some of which can be identified as oligodendroglia. Some inclusions were relatively amorphous while others possess a well-defined lamellar pattern. The fine structure of the globoid-epithelioid cells of Krabbe's disease differs from that of the usual macrophage and contains membrane-bound areas of relative translucency as well as abundant amorphous moderately osmiophiclic deposits. Epithelioid cells are seen in the perivascular spaces which favors their origin from mesodermal elements and is against their being derived from macroglia. In sudanophilic leukodystrophy there are macrophages which are not distinguishable from macrophages seen in areas of myelin destruction from whatever cause. In the spongy degeneration of infants, there is a peculiar myelin abnormality with splitting of the intraperiod line of the myelin lamellae and the existance of large abnormal mitochondria in astrocyte cytoplasm. There may also be vaculolization of significance within oliogodendroglia cytoplasm. The Rosenthal fibers seen in Alexander's type of leukodystrophy appear as osmiophilic masses in astroyte cytoplasm and probably consist of altered glial filaments.

Authors

No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

5173356

Citation

Nelson, E. "Nosology of the Leukodystrophies: Ultrastructural Contributions." Birth Defects Original Article Series, vol. 7, no. 1, 1971, pp. 113-6.
Nelson E. Nosology of the leukodystrophies: ultrastructural contributions. Birth Defects Orig Artic Ser. 1971;7(1):113-6.
Nelson, E. (1971). Nosology of the leukodystrophies: ultrastructural contributions. Birth Defects Original Article Series, 7(1), 113-6.
Nelson E. Nosology of the Leukodystrophies: Ultrastructural Contributions. Birth Defects Orig Artic Ser. 1971;7(1):113-6. PubMed PMID: 5173356.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Nosology of the leukodystrophies: ultrastructural contributions. A1 - Nelson,E, PY - 1971/2/1/pubmed PY - 1971/2/1/medline PY - 1971/2/1/entrez SP - 113 EP - 6 JF - Birth defects original article series JO - Birth Defects Orig Artic Ser VL - 7 IS - 1 N2 - Ultrastructural aspects of some of the conditions usually included as "leukodystrophies" are described. In metachromatic leukodystrophy, the most striking ultrastructural features include a variety of inclusions within cells some of which can be identified as oligodendroglia. Some inclusions were relatively amorphous while others possess a well-defined lamellar pattern. The fine structure of the globoid-epithelioid cells of Krabbe's disease differs from that of the usual macrophage and contains membrane-bound areas of relative translucency as well as abundant amorphous moderately osmiophiclic deposits. Epithelioid cells are seen in the perivascular spaces which favors their origin from mesodermal elements and is against their being derived from macroglia. In sudanophilic leukodystrophy there are macrophages which are not distinguishable from macrophages seen in areas of myelin destruction from whatever cause. In the spongy degeneration of infants, there is a peculiar myelin abnormality with splitting of the intraperiod line of the myelin lamellae and the existance of large abnormal mitochondria in astrocyte cytoplasm. There may also be vaculolization of significance within oliogodendroglia cytoplasm. The Rosenthal fibers seen in Alexander's type of leukodystrophy appear as osmiophilic masses in astroyte cytoplasm and probably consist of altered glial filaments. SN - 0547-6844 UR - https://www.unboundmedicine.com/medline/citation/5173356/Nosology_of_the_leukodystrophies:_ultrastructural_contributions_ DB - PRIME DP - Unbound Medicine ER -