Am. J. Respir. Crit. Care Med., Vol 150, No. 2, Aug 1994, 540-546.
Respiratory manifestations of rigid spine syndrome
GJ Ras, M van Staden, C Schultz, JP Stubgen, BP Lotz and C van der Merwe
Department of Internal Medicine, University of Pretoria, Republic of South Africa.
Thoracic abnormalities and respiratory muscle function were investigated in
nine patients with rigid spine syndrome. A severe restrictive chest wall
defect and limited mobility of the spine associated with clinically
significant respiratory muscle weakness were present in all patients.
Respiratory muscle strength and endurance were less than 60% of control
values. Slight to moderate scoliosis was present in five patients and
absent in four. Scoliosis appeared to have only a minor additional effect
on respiratory muscle function. Six patients were emaciated, and one
patient was underweight, but no relationship was seen between body mass
index and respiratory muscle strength. Respiratory muscle function was more
impaired in patients with hypoventilation than in normocapnic patients.
Respiratory muscle involvement appears to be a significant feature of rigid
spine syndrome, terminating in hypercapnic ventilatory failure in some
patients.
