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

M. T. Al-Lozi; V. B. Hemelt; A. Pestronk

doi:10.1212/WNL.50.2.526

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

M. T. Al-Lozi, V. B. Hemelt, A. Pestronk

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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.

Original language	English
Pages (from-to)	526-529
Number of pages	4
Journal	Neurology
Volume	50
Issue number	2
DOIs	https://doi.org/10.1212/WNL.50.2.526
State	Published - Feb 1998

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title = "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.",

author = "Al-Lozi, {M. T.} and Hemelt, {V. B.} and A. Pestronk",

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AU - Hemelt, V. B.

AU - Pestronk, A.

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Y1 - 1998/2

N2 - 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.

AB - 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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VL - 50

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JO - Neurology

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ER -

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

Abstract

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