[Rigid spine syndrome in children and adolescents]

The syndrome of the rigid spine is described in two patients aged 15 and 12 years. A total of 8 patients with this syndrome were studied. The disease was characterized by the myopathic syndrome, scoliosis, failure to bend the head and body, cardiopathy and occasional contractures of the proximal joints. The clinical, biochemical, roentgenological and morphological findings are presented. A multimodality treatment suggested should be carried out by a team comprising an orthopedist, a neuropathologist, and a pediatrician.