Rigid spine syndrome. A type I fiber myopathy

Arch Neurol. 1977 Feb;34(2):119-22. doi: 10.1001/archneur.1977.00500140073016.


The rigid spine syndrome is an unusual disorder characterized by nonprogressive mild axial and proximal muscle weakness, flexion contractures of the neck and spine, scoliosis, and joint contractures. The biopsy specimen from the patient described in this report shows type I fiber hypotrophy and predominance. This contrasts with previous reports that describe connective tissue proliferation as the major histologic abnormality.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Contracture / pathology*
  • Contracture / physiopathology
  • Humans
  • Male
  • Muscles / pathology*
  • Muscular Diseases / pathology*
  • Muscular Diseases / physiopathology
  • Spinal Diseases / pathology*
  • Spinal Diseases / physiopathology
  • Syndrome