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Peroneal muscular atrophy with autosomal dominant inheritance.
Clin Exp Neurol. 1977; 14:142-53.CE

Abstract

Nerve conduction studies were performed on 45 patients in 17 kinships with autosomal dominant Charcot-Marie-Tooth disease. Sural nerve biopsies were performed on 20 patients. The mean motor conduction velocities in median, ulnar and lateral popliteal nerves were 27.5m/sec, 25.9m/sec and 26.3m/sec respectively. Sensory conduction was impaired in all cases. The fastest motor conduction velocities were found in 5 members of 1 kinship who were found on sural nerve biopsy to have the neuronal form of the disease in which there was a reduction in density of large diameter myelinated fibres. In this group segmental demyelination was not a prominent feature in teased fibre preparations and onion bulb formations were not seen on electron microscopy. In the other patients conduction velocities were more severely slowed and hypertrophic changes and segmental demyelination were found in sural nerve biopsies.

Authors

McLeod JG
No affiliation info available
Low PA
No affiliation info available

MeSH

AdolescentAdultAgedCharcot-Marie-Tooth DiseaseChildChild, PreschoolChromosome AberrationsChromosome DisordersFemaleGenes, DominantHumansHypertrophyInfantMaleMiddle AgedMotor NeuronsMuscular AtrophyNeural ConductionPeripheral NervesSural Nerve

Pub Type(s)

Journal Article

Language

eng

PubMed ID

616594

Citation

McLeod, J G., and P A. Low. "Peroneal Muscular Atrophy With Autosomal Dominant Inheritance." Clinical and Experimental Neurology, vol. 14, 1977, pp. 142-53.
McLeod JG, Low PA. Peroneal muscular atrophy with autosomal dominant inheritance. Clin Exp Neurol. 1977;14:142-53.
McLeod, J. G., & Low, P. A. (1977). Peroneal muscular atrophy with autosomal dominant inheritance. Clinical and Experimental Neurology, 14, 142-53.
McLeod JG, Low PA. Peroneal Muscular Atrophy With Autosomal Dominant Inheritance. Clin Exp Neurol. 1977;14:142-53. PubMed PMID: 616594.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peroneal muscular atrophy with autosomal dominant inheritance. AU - McLeod,J G, AU - Low,P A, PY - 1977/1/1/pubmed PY - 1977/1/1/medline PY - 1977/1/1/entrez SP - 142 EP - 53 JF - Clinical and experimental neurology JO - Clin Exp Neurol VL - 14 N2 - Nerve conduction studies were performed on 45 patients in 17 kinships with autosomal dominant Charcot-Marie-Tooth disease. Sural nerve biopsies were performed on 20 patients. The mean motor conduction velocities in median, ulnar and lateral popliteal nerves were 27.5m/sec, 25.9m/sec and 26.3m/sec respectively. Sensory conduction was impaired in all cases. The fastest motor conduction velocities were found in 5 members of 1 kinship who were found on sural nerve biopsy to have the neuronal form of the disease in which there was a reduction in density of large diameter myelinated fibres. In this group segmental demyelination was not a prominent feature in teased fibre preparations and onion bulb formations were not seen on electron microscopy. In the other patients conduction velocities were more severely slowed and hypertrophic changes and segmental demyelination were found in sural nerve biopsies. SN - 0196-6383 UR - https://www.unboundmedicine.com/medline/citation/616594/Peroneal_muscular_atrophy_with_autosomal_dominant_inheritance_ L2 - http://www.diseaseinfosearch.org/result/1285 DB - PRIME DP - Unbound Medicine ER -