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Primary segmental demyelination in the sural nerve in Cockayne's syndrome.
Muscle Nerve. 1987 Feb; 10(2):163-7.MN

Abstract

In a typical case of Cockayne's syndrome, morphometric histopathologic findings of the sural nerve were described and compared with data on two controls. The main pathologic changes of myelinated fibers were segmental demyelination and remyelination with onion bulb formation and a moderate decrease of myelinated fibers. There was no evidence of axonal atrophy in relation to myelin spiral length. In addition, no evidence of the clustered occurrence of demyelinated and remyelinated internodes was obtained on the statistical evaluation of the distribution of such internodes. Although the presence or absence of hypomyelination of a mild degree and maldevelopment of axons to be myelinated has not been clarified, our morphometric data do suggest the presence of primary segmental demyelination of the biopsied sural nerve.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

3821788

Citation

Ohnishi, A, et al. "Primary Segmental Demyelination in the Sural Nerve in Cockayne's Syndrome." Muscle & Nerve, vol. 10, no. 2, 1987, pp. 163-7.
Ohnishi A, Mitsudome A, Murai Y. Primary segmental demyelination in the sural nerve in Cockayne's syndrome. Muscle Nerve. 1987;10(2):163-7.
Ohnishi, A., Mitsudome, A., & Murai, Y. (1987). Primary segmental demyelination in the sural nerve in Cockayne's syndrome. Muscle & Nerve, 10(2), 163-7.
Ohnishi A, Mitsudome A, Murai Y. Primary Segmental Demyelination in the Sural Nerve in Cockayne's Syndrome. Muscle Nerve. 1987;10(2):163-7. PubMed PMID: 3821788.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary segmental demyelination in the sural nerve in Cockayne's syndrome. AU - Ohnishi,A, AU - Mitsudome,A, AU - Murai,Y, PY - 1987/2/1/pubmed PY - 1987/2/1/medline PY - 1987/2/1/entrez SP - 163 EP - 7 JF - Muscle & nerve JO - Muscle Nerve VL - 10 IS - 2 N2 - In a typical case of Cockayne's syndrome, morphometric histopathologic findings of the sural nerve were described and compared with data on two controls. The main pathologic changes of myelinated fibers were segmental demyelination and remyelination with onion bulb formation and a moderate decrease of myelinated fibers. There was no evidence of axonal atrophy in relation to myelin spiral length. In addition, no evidence of the clustered occurrence of demyelinated and remyelinated internodes was obtained on the statistical evaluation of the distribution of such internodes. Although the presence or absence of hypomyelination of a mild degree and maldevelopment of axons to be myelinated has not been clarified, our morphometric data do suggest the presence of primary segmental demyelination of the biopsied sural nerve. SN - 0148-639X UR - https://www.unboundmedicine.com/medline/citation/3821788/Primary_segmental_demyelination_in_the_sural_nerve_in_Cockayne's_syndrome_ L2 - https://doi.org/10.1002/mus.880100210 DB - PRIME DP - Unbound Medicine ER -