Steroid-responsive myopathy with deficient chondroitin sulfate C in skeletal muscle connective tissue.

al-Lozi, M T; Hemelt, V B; Pestronk, A · Neurology · 1998

case_report · Level V

Where this comes from

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.

Medical subject headings

Anatomy