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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).
Int J Tissue React. 1984; 6(3):267-74.IJ

Abstract

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.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

6090332

Citation

Tredici, G, et al. "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)." International Journal of Tissue Reactions, vol. 6, no. 3, 1984, pp. 267-74.
Tredici G, Petruccioli-Pizzini MG, Gergely A, et al. 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). Int J Tissue React. 1984;6(3):267-74.
Tredici, G., Petruccioli-Pizzini, M. G., Gergely, A., & Coletti, A. (1984). 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). International Journal of Tissue Reactions, 6(3), 267-74.
Tredici G, et al. 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). Int J Tissue React. 1984;6(3):267-74. PubMed PMID: 6090332.
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TY - JOUR T1 - 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). AU - Tredici,G, AU - Petruccioli-Pizzini,M G, AU - Gergely,A, AU - Coletti,A, PY - 1984/1/1/pubmed PY - 1984/1/1/medline PY - 1984/1/1/entrez SP - 267 EP - 74 JF - International journal of tissue reactions JO - Int J Tissue React VL - 6 IS - 3 N2 - 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. SN - 0250-0868 UR - https://www.unboundmedicine.com/medline/citation/6090332/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__ L2 - http://www.diseaseinfosearch.org/result/3622 DB - PRIME DP - Unbound Medicine ER -