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Ultrastructural and cytochemical observations in a case of dominantly inherited hypertrophic (Charcot-Marie-Tooth) neuropathy.
J Neuropathol Exp Neurol. 1979 Nov; 38(6):586-95.JN

Abstract

Ultrastructural and cytochemical studies were carried out on the sural nerve of a 6 1/2 year old girl with dominantly inherited hypertrophic (Charcot-Marie-Tooth) neuropathy. Electron microscopy revealed a paucity of myelinated fibers, with inappropriately thin myelin sheaths and onion-bulb formations associated with those fibers that were myelinated. In some cases the nodal axolemma was folded so as to form irregular excrescences. At other nodes, the non-myelinated gap was enlarged. Following staining with ferric ion and ferrocyanide, dense precipitates were observed on the cytoplasmic surface of the axolemma at some nodes of Ranvier, as in normal peripheral axons. At other nodes, staining was attenuated or absent. The latter result is similar to our findings in the dy/dy dystrophic mouse. These results are consistent with the hypothesis that, in dominantly inherited hypertrophic neuropathy, there are abnormalities of structure of the axolemma, in addition to an abnormality of the myelin sheath.

Authors

No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

533859

Citation

Waxman, S G., and E M. Ouelette. "Ultrastructural and Cytochemical Observations in a Case of Dominantly Inherited Hypertrophic (Charcot-Marie-Tooth) Neuropathy." Journal of Neuropathology and Experimental Neurology, vol. 38, no. 6, 1979, pp. 586-95.
Waxman SG, Ouelette EM. Ultrastructural and cytochemical observations in a case of dominantly inherited hypertrophic (Charcot-Marie-Tooth) neuropathy. J Neuropathol Exp Neurol. 1979;38(6):586-95.
Waxman, S. G., & Ouelette, E. M. (1979). Ultrastructural and cytochemical observations in a case of dominantly inherited hypertrophic (Charcot-Marie-Tooth) neuropathy. Journal of Neuropathology and Experimental Neurology, 38(6), 586-95.
Waxman SG, Ouelette EM. Ultrastructural and Cytochemical Observations in a Case of Dominantly Inherited Hypertrophic (Charcot-Marie-Tooth) Neuropathy. J Neuropathol Exp Neurol. 1979;38(6):586-95. PubMed PMID: 533859.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ultrastructural and cytochemical observations in a case of dominantly inherited hypertrophic (Charcot-Marie-Tooth) neuropathy. AU - Waxman,S G, AU - Ouelette,E M, PY - 1979/11/1/pubmed PY - 1979/11/1/medline PY - 1979/11/1/entrez SP - 586 EP - 95 JF - Journal of neuropathology and experimental neurology JO - J. Neuropathol. Exp. Neurol. VL - 38 IS - 6 N2 - Ultrastructural and cytochemical studies were carried out on the sural nerve of a 6 1/2 year old girl with dominantly inherited hypertrophic (Charcot-Marie-Tooth) neuropathy. Electron microscopy revealed a paucity of myelinated fibers, with inappropriately thin myelin sheaths and onion-bulb formations associated with those fibers that were myelinated. In some cases the nodal axolemma was folded so as to form irregular excrescences. At other nodes, the non-myelinated gap was enlarged. Following staining with ferric ion and ferrocyanide, dense precipitates were observed on the cytoplasmic surface of the axolemma at some nodes of Ranvier, as in normal peripheral axons. At other nodes, staining was attenuated or absent. The latter result is similar to our findings in the dy/dy dystrophic mouse. These results are consistent with the hypothesis that, in dominantly inherited hypertrophic neuropathy, there are abnormalities of structure of the axolemma, in addition to an abnormality of the myelin sheath. SN - 0022-3069 UR - https://www.unboundmedicine.com/medline/citation/533859/Ultrastructural_and_cytochemical_observations_in_a_case_of_dominantly_inherited_hypertrophic__Charcot_Marie_Tooth__neuropathy_ L2 - https://academic.oup.com/jnen/article-lookup/doi/10.1097/00005072-197911000-00003 DB - PRIME DP - Unbound Medicine ER -