Electrophysiological findings including single fibre EMG in a family with mitochondrial myopathy

P.R.W. Fawcett; F.L. Mastaglia; F. Mechler

doi:10.1016/0022-510X(82)90021-1

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Electrophysiological findings including single fibre EMG in a family with mitochondrial myopathy

Journal article

Peer reviewed

Electrophysiological findings including single fibre EMG in a family with mitochondrial myopathy

P.R.W. Fawcett, F.L. Mastaglia and F. Mechler

Journal of the Neurological Sciences, Vol.53(2), pp.397-410

1982

DOI: https://doi.org/10.1016/0022-510X(82)90021-1

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Abstract

Nerve conduction studies, conventional and quantitative concentric needle EMG and single fibre EMG were performed on 5 clinically affected and 7 clinically asymptomatic membres of a family with a mitochondrial myopathy manifesting as a facioscapulohumeral syndrome. Abnormalities of nerve conduction present in 3 clinically affected cases were attributed to co-existent diabetes mellitus. Quantitative CNEMG showed a reduction of the mean motor unit potential duration and increased incidence of polyphasic potentials in all 5 clinically manifest cases consistent with a primary myopathic disorder. Similar but less marked changes were found in 6 of the clinically asymptomatic individuals revealing the presence of a subclinical myopathy. Abnormalities on SFEMG consisting of increases in fibre density and/or jitter were present in all the clinically affected and in 5 clinically normal cases. These changes indicate local reorganization of the spatial arrangement of muscle fibres of the motor unit and a disturbance of neuromuscular transmission. The CNEMG and SFEMG findings are discussed in relation to the histopathological changes in 4 cases.

Details

Title: Electrophysiological findings including single fibre EMG in a family with mitochondrial myopathy
Authors/Creators: P.R.W. Fawcett (Author/Creator)
F.L. Mastaglia (Author/Creator)
F. Mechler (Author/Creator)
Publication Details: Journal of the Neurological Sciences, Vol.53(2), pp.397-410
Publisher: Elsevier BV
Identifiers: 991005543441607891
Copyright: © 1982 Elsevier B.V.
Murdoch Affiliation: Murdoch University
Language: English
Resource Type: Journal article

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Citation topics: 1 Clinical & Life Sciences; 1.197 Molecular & Cell Biology - Mitochondria; 1.197.564 Mitochondrial Function
Web Of Science research areas: Clinical Neurology; Neurosciences
ESI research areas: Neuroscience & Behavior