[Rigid spine syndrome and its nosological borders. 2 cases]

Abstract

The rigid spine syndrome is characterized clinically by rigidity due to deficient extensor muscles of the spine (chiefly cervical) and radiologically, by a normal spinal image. Electromyography demonstrates abnormalities suggestive of a myogenic process, and histology shows alterations of muscular fibres. From the case-records of two male patients aged 14 and 15 years respectively, a relationship could be established between various diseases having in common a rigid spine syndrome. These include congenital muscular dystrophias , sequelae of arthrogryposis, Emery - Dreifuss myopathy and myosclerosis .