Diaphragm weakness in Charcot-Marie-Tooth disease

Two patients are described with Charcot-Marie-Tooth disease and chronic peripheral neuropathy. Both had dyspnoea, orthopnoea, and evidence of severe diaphragm weakness. Expiratory muscle function was well preserved and abnormalities of gas exchange during sleep were only minor.

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Bibliographic Details
Published inThorax Vol. 43; no. 6; pp. 478 - 479
Main Authors Laroche, C M, Carroll, N, Moxham, J, Stanley, N N, Evans, R J, Green, M
Format Journal Article
LanguageEnglish
Published London BMJ Publishing Group Ltd and British Thoracic Society 01.06.1988
BMJ
Subjects
Biological and medical sciences
Charcot-Marie-Tooth Disease - physiopathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Diaphragm - physiopathology
Humans
Male
Medical sciences
Middle Aged
Muscular Atrophy, Spinal - physiopathology
Neurology
Respiratory Function Tests
Human
Nervous system diseases
Respiratory muscle
Charcot Marie Tooth disease
Respiratory disease
Mechanic of breathing
Diaphragm
Paralysis
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Summary:Two patients are described with Charcot-Marie-Tooth disease and chronic peripheral neuropathy. Both had dyspnoea, orthopnoea, and evidence of severe diaphragm weakness. Expiratory muscle function was well preserved and abnormalities of gas exchange during sleep were only minor.
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PMID:3420560
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ISSN:0040-6376
1468-3296
DOI:10.1136/thx.43.6.478