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Structural manifestations of leukodystrophies.
Neuropediatrics. 1984 Sep; 15 Suppl:53-61.N

Abstract

Following the definition of the leukodystrophies as degenerative demyelinating processes caused by metabolic disorders a pathogenetical classification of the leukodystrophies is given. Metabolic processes in the narrower sense (enzymopathies) and dysgenetic processes are distinguished. In the special part according to this classification the neuropathological characters of the representative leukodystrophies are described. Different types of loss of myelin sheaths, structural features of stored products, localisation patterns etc. are demonstrated. Etiogenetic, nosological and diagnostic aspects are discussed. Probable conditions of the inflammatory demyelination syndrome of adrenoleukodystrophy are considered. The possible coincidence of dysmyelination and demyelination in early manifested leukodystrophies in the frame of the Pelizaeus-Merzbacher-syndrome is emphasized. The findings in a connatal case of Pelizaeus-Merzbacher-syndrome are described.

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Publisher Full Text

Authors

Seitelberger F
No affiliation info available

MeSH

AtrophyAxonsBrainCerebroside-SulfataseDiffuse Cerebral Sclerosis of SchilderFemaleGalactosylceramidaseHumansInfantLeukodystrophy, Globoid CellLeukodystrophy, MetachromaticMyelin SheathNecrosisNerve DegenerationNerve Fibers, MyelinatedOligodendroglia

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

6152813

Citation

Seitelberger, F. "Structural Manifestations of Leukodystrophies." Neuropediatrics, vol. 15 Suppl, 1984, pp. 53-61.
Seitelberger F. Structural manifestations of leukodystrophies. Neuropediatrics. 1984;15 Suppl:53-61.
Seitelberger, F. (1984). Structural manifestations of leukodystrophies. Neuropediatrics, 15 Suppl, 53-61.
Seitelberger F. Structural Manifestations of Leukodystrophies. Neuropediatrics. 1984;15 Suppl:53-61. PubMed PMID: 6152813.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Structural manifestations of leukodystrophies. A1 - Seitelberger,F, PY - 1984/9/1/pubmed PY - 1984/9/1/medline PY - 1984/9/1/entrez SP - 53 EP - 61 JF - Neuropediatrics JO - Neuropediatrics VL - 15 Suppl N2 - Following the definition of the leukodystrophies as degenerative demyelinating processes caused by metabolic disorders a pathogenetical classification of the leukodystrophies is given. Metabolic processes in the narrower sense (enzymopathies) and dysgenetic processes are distinguished. In the special part according to this classification the neuropathological characters of the representative leukodystrophies are described. Different types of loss of myelin sheaths, structural features of stored products, localisation patterns etc. are demonstrated. Etiogenetic, nosological and diagnostic aspects are discussed. Probable conditions of the inflammatory demyelination syndrome of adrenoleukodystrophy are considered. The possible coincidence of dysmyelination and demyelination in early manifested leukodystrophies in the frame of the Pelizaeus-Merzbacher-syndrome is emphasized. The findings in a connatal case of Pelizaeus-Merzbacher-syndrome are described. SN - 0174-304X UR - https://www.unboundmedicine.com/medline/citation/6152813/Structural_manifestations_of_leukodystrophies_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1052382 DB - PRIME DP - Unbound Medicine ER -