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February 1977

Rigid Spine Syndrome: A Type I Fiber Myopathy

Author Affiliations

From the Division of Pediatric Neurology and the Departments of Neurology and Pediatrics, University of Utah College of Medicine, Salt Lake City.

Arch Neurol. 1977;34(2):119-122. doi:10.1001/archneur.1977.00500140073016

• The rigid spine syndrome is an unusual disorder characterized by non-progressive 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.

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