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NEUROLOGY 1975;25:114
© 1975 American Academy of Neurology

Myopathy associated with linear scleroderma

A histochemical and electron microscopic study

LAWRENCE Z. STERN, M.D., CLAIRE M. PAYNE, Ph.D., JULIAN T. ALVAREZ, M.D. and LINDA K. HANNAPEL, A.B.

Departments of Neurology (Dr. Stern, Dr. Alvarez, Ms. Hannapel) and Pathology (Dr. Payne), The University of Arizona College of Medicine, Tucson.

Pathologic findings in biopsied rectus femoris muscle underlying an area of linear scleroderma are described. The affected muscle was weak and atrophic. On electromyography it showed motor unit potentials of decreased amplitude and duration. Light microscopic changes were minimal. There was atrophy of some histochemical type I fibers. More prominent changes were found at the ultrastructural level, where many of the capillary basal laminae were thickened and reduplicated. Most striking was the presence of two types of electron-dense, rounded inclusions within the mitochondria, one ranging from 29 to 47 nm in diameter and the other from 54 to 131 nm in diameter.

This study was supported in part by a grant from the Muscular Dystrophy Associations of America (to Dr. Stern and Dr. Raphael Gruener), by NIH grant NS 10417, and by a gift from Mr, and Mrs. Edward Watz.

Received for publication July 29, 1974.

Dr. Stern's address is Department of Neurology, University of Arizona Medical Center, Tucson, AZ 85724.




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