Determinants of respiratory muscle weakness in stable chronic neuromuscular disorders |
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Authors: | W Vincken M G Elleker M G Cosio |
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Affiliation: | 1. Serviço de Endocrinologia, Hospital de Clínicas de Porto Alegre, UFRGS, Porto Alegre, RS, Brazil;2. CEDOES, Vitória, ES, Brazil;3. ICF - Instituto de Ciências Farmacêuticas de Estudos e Pesquisas Ltda, Aparecida de Goiânia, GO, Brazil;4. Hospital Alberto Rassi – HGG, Goiânia, GO, Brazil;5. Hospital Universitário João de Barros Barreto, Universidade Federal do Pará, Belém, PA, Brazil;6. Hospital Universitário da Universidade Federal do Maranhão/HU/UFMA, São Luis, MA, Brazil;7. Centro de Diabetes de Curitiba, Curitiba, PR, Brazil;8. CPQUALI Pesquisa Clínica Ltda, São Paulo, SP, Brazil;9. Instituto de Ensino e Pesquisa Clínica do Ceará, Fortaleza, CE, Brazil;10. Instituto de Medicina Integral Professor Fernando Figueira – IMIP, Recife, PE, Brazil;11. Unidade de Pesquisa Clínica de Marília Ltda (UpCliM), Marília, SP, Brazil;12. Universidade Federal de São Paulo - UNIFESP/EPM, São Paulo, SP, Brazil;13. CAEP - Centro Avançado de Estudos e Pesquisas Ltda, Campinas, SP, Brazil;14. Centro de Pesquisas Médicas Básica e Clínica Ltda, Recife, PE, Brazil;15. Cristália Produtos Químicos Farmacêuticos Ltda, Itapira, SP, Brazil;1. Child Neurology Unit, Pediatric Hospital Anna Meyer, Florence, Italy;2. Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy |
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Abstract: | In neuromuscular disease, the precise relationship between general and respiratory muscle weakness is at present unclear. That relationship and the influence on respiratory muscle strength of such factors as type and duration of neuromuscular disease, distribution of general muscle weakness, and nutritional status were studied in 30 patients with stable chronic neuromuscular disease not presenting with respiratory symptoms. The degree of general muscle weakness was assessed by clinical examination of the strength of 17 muscle groups, yielding a general muscle strength index. The degree of respiratory muscle weakness was assessed by measuring maximal static inspiratory and expiratory mouth pressures. Maximal inspiratory (mean +/- SD: 68 +/- 28 percent predicted) and expiratory (66 +/- 29 percent predicted) mouth pressures were frequently reduced, but did not correlate with general muscle strength. The ability to estimate the degree of respiratory muscle weakness improved to some extent when the type of neuromuscular disease and the distribution of general muscle weakness were taken into account: thus, maximal expiratory mouth pressure was significantly lower (p less than 0.05) in myopathy than in polyneuropathy, and in proximal than in distal muscle weakness. Duration of neuromuscular disease and nutritional status did not influence respiratory muscle strength. It is concluded that in stable chronic neuromuscular disease, respiratory muscle involvement depends on a complexity of factors, in particular the type of neuromuscular disease and the distribution, rather than the degree, of general muscle weakness. In the individual patient, however, only direct measurement of maximal inspiratory and expiratory mouth pressures allows accurate assessment of respiratory muscle strength. These tests ought to complement neurologic examination. |
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