Ventilatory response in myotonic dystrophy

James E. Carroll, Clifford W. Zwillich, John V. Weil

Research output: Contribution to journalArticle

55 Scopus citations


Patients with myotonic dystrophy often develop respiratory failure caused by alveolar hypoventilation. Abnormalities in the ventilatory response to hypoxia and hypercapnia may explain this phenomenon. Accordingly, hypoxic and hypercapnic responses were measured in seven patients with myotonic dystrophy who had only mild respiratory muscle weakness. Hypoxic response was significantly reduced, while hypercapnic response was affected more irregularly. It is possible that the high incidence of respiratory failure in such patients is related to decreased hypoxic ventilatory response, occurring because of an underlying neurogenic deficit.

Original languageEnglish (US)
Pages (from-to)1125-1128
Number of pages4
Issue number12
StatePublished - Dec 1977
Externally publishedYes


ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Carroll, J. E., Zwillich, C. W., & Weil, J. V. (1977). Ventilatory response in myotonic dystrophy. Neurology, 27(12), 1125-1128.