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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. |
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