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Peripheral neuropathy in myotonic dystrophy: a nerve biopsy study.
Ann Neurol. 1988 May; 23(5):470-6.AN

Abstract

Sural nerve biopsies from 13 unselected myotonic dystrophy patients and 6 normal controls were studied morphometrically. The myelinated fiber density was reduced in 11 of the 13 myotonic dystrophy patients, with preferential loss of large myelinated fibers. Unmyelinated fiber densities and diameters were normal. Teased fiber studies commonly revealed focal areas of remyelination and abnormal wrinkling of the myelin sheath. Measurement of internodal length disclosed features of both axonal regeneration and focal demyelination-remyelination. These findings are consistent with a chronic axonopathy of moderate severity, possibly due to axonal atrophy.

Authors+Show Affiliations

Department of Psychiatry and Neurology, Tulane Medical School, New Orleans, LA.No affiliation info availableNo affiliation info availableNo 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

2839105

Citation

Cros, D, et al. "Peripheral Neuropathy in Myotonic Dystrophy: a Nerve Biopsy Study." Annals of Neurology, vol. 23, no. 5, 1988, pp. 470-6.
Cros D, Harnden P, Pouget J, et al. Peripheral neuropathy in myotonic dystrophy: a nerve biopsy study. Ann Neurol. 1988;23(5):470-6.
Cros, D., Harnden, P., Pouget, J., Pellissier, J. F., Gastaut, J. L., & Serratrice, G. (1988). Peripheral neuropathy in myotonic dystrophy: a nerve biopsy study. Annals of Neurology, 23(5), 470-6.
Cros D, et al. Peripheral Neuropathy in Myotonic Dystrophy: a Nerve Biopsy Study. Ann Neurol. 1988;23(5):470-6. PubMed PMID: 2839105.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral neuropathy in myotonic dystrophy: a nerve biopsy study. AU - Cros,D, AU - Harnden,P, AU - Pouget,J, AU - Pellissier,J F, AU - Gastaut,J L, AU - Serratrice,G, PY - 1988/5/1/pubmed PY - 1988/5/1/medline PY - 1988/5/1/entrez SP - 470 EP - 6 JF - Annals of neurology JO - Ann Neurol VL - 23 IS - 5 N2 - Sural nerve biopsies from 13 unselected myotonic dystrophy patients and 6 normal controls were studied morphometrically. The myelinated fiber density was reduced in 11 of the 13 myotonic dystrophy patients, with preferential loss of large myelinated fibers. Unmyelinated fiber densities and diameters were normal. Teased fiber studies commonly revealed focal areas of remyelination and abnormal wrinkling of the myelin sheath. Measurement of internodal length disclosed features of both axonal regeneration and focal demyelination-remyelination. These findings are consistent with a chronic axonopathy of moderate severity, possibly due to axonal atrophy. SN - 0364-5134 UR - https://www.unboundmedicine.com/medline/citation/2839105/Peripheral_neuropathy_in_myotonic_dystrophy:_a_nerve_biopsy_study_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0364-5134&date=1988&volume=23&issue=5&spage=470 DB - PRIME DP - Unbound Medicine ER -