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[Charcot-Marie-Tooth disease. Study of sural nerve biopsy in 41 patients].
Arq Neuropsiquiatr. 1995 Sep; 53(3-B):560-9.AN

Abstract

We studied the pathological findings of sural nerve biopsy in 41 patients with Charcot-Marie-Tooth (CMT) disease. They were previously classified by the median motor conduction velocity (MCV) in two types. Type I (demyelinating) with 9 patients and type II (axonal) with 32 cases. In type I we found loss of myelinated fibers (unimodal histogram), demyelinated and remyelinated axons with numerous onion bulb formations. In one case there was thickness of myelin with thin axons (axonal atrophy). In type II there were seven patients with normal sural nerve biopsy. In 25 cases there were mild to severe loss of myelinated fibers. In 5 patients the number of myelinated fibers was increased due to the great regeneration of the axons. The electron microscopic studies in type II showed in a few cases small onion bulbs and in one case axonal atrophy. In type I there was no correlation between clinical severity and the loss of myelinated fibers, but there was relationship between the low MCV and the intensity of myelinated fibers. In type II we did not found any correlation between clinical course, MCV and pathological findings.

Authors+Show Affiliations

Serviço de Neurologia, Hospital Universitário Antônio Pedro, Brasil.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Comparative Study
English Abstract
Journal Article

Language

por

PubMed ID

8585811

Citation

Freitas, M R., et al. "[Charcot-Marie-Tooth Disease. Study of Sural Nerve Biopsy in 41 Patients]." Arquivos De Neuro-psiquiatria, vol. 53, no. 3-B, 1995, pp. 560-9.
Freitas MR, Nascimento OJ, Chimelli L, et al. [Charcot-Marie-Tooth disease. Study of sural nerve biopsy in 41 patients]. Arq Neuropsiquiatr. 1995;53(3-B):560-9.
Freitas, M. R., Nascimento, O. J., Chimelli, L., & de Freitas, G. R. (1995). [Charcot-Marie-Tooth disease. Study of sural nerve biopsy in 41 patients]. Arquivos De Neuro-psiquiatria, 53(3-B), 560-9.
Freitas MR, et al. [Charcot-Marie-Tooth Disease. Study of Sural Nerve Biopsy in 41 Patients]. Arq Neuropsiquiatr. 1995;53(3-B):560-9. PubMed PMID: 8585811.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Charcot-Marie-Tooth disease. Study of sural nerve biopsy in 41 patients]. AU - Freitas,M R, AU - Nascimento,O J, AU - Chimelli,L, AU - de Freitas,G R, PY - 1995/9/1/pubmed PY - 1995/9/1/medline PY - 1995/9/1/entrez SP - 560 EP - 9 JF - Arquivos de neuro-psiquiatria JO - Arq Neuropsiquiatr VL - 53 IS - 3-B N2 - We studied the pathological findings of sural nerve biopsy in 41 patients with Charcot-Marie-Tooth (CMT) disease. They were previously classified by the median motor conduction velocity (MCV) in two types. Type I (demyelinating) with 9 patients and type II (axonal) with 32 cases. In type I we found loss of myelinated fibers (unimodal histogram), demyelinated and remyelinated axons with numerous onion bulb formations. In one case there was thickness of myelin with thin axons (axonal atrophy). In type II there were seven patients with normal sural nerve biopsy. In 25 cases there were mild to severe loss of myelinated fibers. In 5 patients the number of myelinated fibers was increased due to the great regeneration of the axons. The electron microscopic studies in type II showed in a few cases small onion bulbs and in one case axonal atrophy. In type I there was no correlation between clinical severity and the loss of myelinated fibers, but there was relationship between the low MCV and the intensity of myelinated fibers. In type II we did not found any correlation between clinical course, MCV and pathological findings. SN - 0004-282X UR - https://www.unboundmedicine.com/medline/citation/8585811/[Charcot_Marie_Tooth_disease__Study_of_sural_nerve_biopsy_in_41_patients]_ L2 - http://www.diseaseinfosearch.org/result/1276 DB - PRIME DP - Unbound Medicine ER -