This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on Web of Science (5)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Possible Source of Human Myopathy

Jack B. Shumate, MD; Michael H. Brooke, MD; James E. Carroll, MD; Rati M. Choksi, MS

Arch Neurol. 1982;39(9):561-564.

Abstract
• A study of palmitate oxidation in 200 consecutive human muscle biopsy specimens showed 14 patients in whom there was abnormal, incomplete palmitate oxidation when the rate of oxidation of palmitate¹⁴C (ul) was compared with that of palmitate¹⁴C (at carbon 1). Five patients had denervation as a primary diagnosis, and the remaining nine had a primary muscle disease. Of this latter group, six had clinical similarities, including proximal weakness, necrotic fibers on muscle biopsy, and extreme elevations of serum creatine kinase. With one exception, lipid storage was not part of the syndrome. The possibility of incomplete palmitate oxidation due to a defect in β-oxidation producing a human myopathy is discussed.

Author Affiliations
From the Department of Neurology and Neurosurgery (Neurology) (Drs Shumate, Brooke, and Carroll and Ms Choksi) and the Department of Pediatrics (Dr Carroll), Washington University School of Medicine, St Louis. Dr Shumate is now with Case Western Reserve University, Cleveland.

Footnotes
Accepted for publication Oct 18, 1981.

Reprint requests to Department of Neurology, University Hospitals, Case Western Reserve University, Cleveland, OH 44106 (Dr Shumate).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?