Rigid spine syndrome with respiratory failure.
Open Access
- 1 September 1990
- journal article
- research article
- Published by BMJ in Journal of Neurology, Neurosurgery & Psychiatry
- Vol. 53 (9) , 782-784
- https://doi.org/10.1136/jnnp.53.9.782
Abstract
The pathogenesis and therapy of respiratory failure in the rigid spine syndrome are discussed in two cases who improved with respiratory assistance. In both cases, the partial pressures of oxygen and carbon dioxide were reversed in arterial blood gas analysis and %VC was less than 30%. Remission from respiratory failure has been obtained by the use of a ventilator during the night. The cause of the respiratory failure in both cases was severe restrictive respiratory dysfunction due to extreme flattening of the chest and fixation of the thorax during respiration as a result of contracture of costovertebral joints. All the previously reported cases of the rigid spine syndrome with respiratory failure died. Appropriate use of the ventilator can improve the prognosis.Keywords
This publication has 9 references indexed in Scilit:
- The rigid spine syndrome and Emery-Dreifuss muscular dystrophyClinical Neurology and Neurosurgery, 1986
- The rigid spine syndrome.Journal of Neurology, Neurosurgery & Psychiatry, 1986
- The rigid spine syndrome--a myopathy of uncertain nosological position.Journal of Neurology, Neurosurgery & Psychiatry, 1985
- Rigid spine syndrome. Some evidence of varying pathological patterns.1983
- [Case of rigid spine syndrome].1983
- Rigid spine syndrome in a girlZeitschrift für Neurologie, 1982
- Rigid spine syndrome and fatal cardiomyopathy.Archives of Disease in Childhood, 1981
- Rigid Spine Syndrome: Histological Examinations of Male and Female CasesPublished by Springer Nature ,1981
- Rigid Spine SyndromeArchives of Neurology, 1977