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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
3
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pubmed:dateCreated |
1984-11-9
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pubmed:abstractText |
The electron microscopic features and quantitative morphometric data of the sural nerve in Déjérine-Sottas disease (HMSN III) and in the hypertrophic form of Charcot-Marie-Tooth disease (HMSN I) are reported. Both forms are characterized by onion bulb formations, but they differ in: a) increased incidence of mucoid connective tissue in DS disease; b) higher incidence of demyelination in DS disease; c) uniformly small size of the remaining myelinated fibers in DS disease; d) larger and more developed onion bulbs in DS disease; e) clusters of 2-4 myelinated fibers in the core of onion bulbs of CMT disease; f) peripheral concentric lamellae with the typical aspects of the denervation bands of the unmyelinated fiber type in the onion bulbs of the CMT disease; g) non-involvement of unmyelinated fibers in DS disease. These differences permit two types of onion bulbs to be distinguished, and suggest a different pathogenesis. Electron microscopy, coupled with quantitative determinations, permits a deeper insight into the formation modalities of onion bulbs and may help in the diagnosis of the different forms of hypertrophic neuropathies.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:issn |
0250-0868
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
6
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
267-74
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:6090332-Adolescent,
pubmed-meshheading:6090332-Adult,
pubmed-meshheading:6090332-Axons,
pubmed-meshheading:6090332-Charcot-Marie-Tooth Disease,
pubmed-meshheading:6090332-Connective Tissue,
pubmed-meshheading:6090332-Humans,
pubmed-meshheading:6090332-Male,
pubmed-meshheading:6090332-Microscopy, Electron,
pubmed-meshheading:6090332-Muscular Atrophy,
pubmed-meshheading:6090332-Myelin Sheath,
pubmed-meshheading:6090332-Peripheral Nervous System Diseases,
pubmed-meshheading:6090332-Schwann Cells
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pubmed:year |
1984
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pubmed:articleTitle |
The importance of quantitative electron microscopy in studying hypertrophic neuropathies. A comparison between a case of Déjérine Sottas disease (HMSN III) and a case of the hypertrophic form of Charcot-Marie-Tooth disease (HMSN I).
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pubmed:publicationType |
Journal Article,
Comparative Study,
Case Reports,
Research Support, Non-U.S. Gov't
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