Serratrice G, Pellissier J F, Pouget J, Gastaut J L
Presse Med. 1984 Apr 28;13(18):1129-32.
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 .
僵硬脊柱综合征的临床特征是由于脊柱(主要是颈椎)伸肌不足导致的僵硬,影像学上脊柱影像正常。肌电图显示提示肌源性过程的异常,组织学显示肌纤维改变。从两名分别为14岁和15岁男性患者的病例记录中,可以确定各种具有共同僵硬脊柱综合征的疾病之间的关系。这些疾病包括先天性肌营养不良、关节挛缩后遗症、埃默里-德赖富斯肌病和肌硬化症。