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Steroid-responsive myopathy with deficient chondroitin sulfate C in skeletal muscle connective tissue
M T al-Lozi1, V B Hemelt, A Pestronk
1Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Neurology
|March 4, 1998
Abstract:
A 71-year-old man developed severe limb, bulbar, and respiratory weakness over 18 months. A muscle biopsy showed only a moderate degree of type 2 atrophy, but immunocytochemistry showed absence of chondroitin sulfate C glycosaminoglycan in the endomysium. Prednisone produced a marked increase in strength. Diffuse loss of endomysial chondroitin sulfate C was a feature of this treatable myopathy with severe weakness, but few pathologic changes.