Rigid spine syndrome. Report of 4 cases.

ABSTRACT

Four cases of rigid spine syndrome are reported for the first time in China. They are 2 males and 2 females. One girl has a positive family history. Limitation of flexion of the cervical and dorsolumbar spine, contractures of joints, especially the elbow joints, extreme flattening of the chest and scoliosis are the prominent symptoms of this syndrome. The clinical criteria for this rigid spine syndrome tally with those described by Dubowitz (1965). Symptomatology, diagnosis, differential diagnosis and genetics are discussed. However, the question whether rigid spine syndrome is a single, well defined disease entity remains to be explored.