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

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.

journal_name

Neurology

journal_title

Neurology

authors

al-Lozi MT,Hemelt VB,Pestronk A

doi

10.1212/wnl.50.2.526

subject

Has Abstract

pub_date

1998-02-01 00:00:00

pages

526-9

issue

2

eissn

0028-3878

issn

1526-632X

journal_volume

50

pub_type

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