al-Lozi M T, Hemelt V B, Pestronk A
Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Neurology. 1998 Feb;50(2):526-9. doi: 10.1212/wnl.50.2.526.
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.
一名71岁男性在18个月内出现严重的肢体、延髓和呼吸肌无力。肌肉活检仅显示中度2型萎缩，但免疫细胞化学显示肌内膜中硫酸软骨素C糖胺聚糖缺失。泼尼松治疗后肌力显著增强。肌内膜硫酸软骨素C弥漫性缺失是这种可治疗的、伴有严重肌无力但病理改变较少的肌病的一个特征。
