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A Type I Fiber Myopathy

Alan R. Seay, MD; Fred A. Ziter, MD; Jack H. Petajan, MD

Arch Neurol. 1977;34(2):119-122.

Abstract
• 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.

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

Footnotes
Accepted for publication Aug 25, 1976.

Reprint requests to Department of Neurology, University of Utah College of Medicine, 50 N Medical Dr, Salt Lake City, UT 84132 (Dr Seay).

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