A Japanese case of steroid responsive myopathy with deficient chondroitin sulphate

In 1998, Al-Lozi et al described a case of steroid responsive myopathy with deficient chondroitin sulphate C that had not been reported before.1 The patient developed diffuse bulbar and systemic weakness with respiratory failure. While muscle biopsy showed only a moderate degree of type 2 atrophy with one small perimysial, perivascular mononuclear cell infiltration, immunocytochemistry showed an absence of chondroitin sulphate C in the endomysium. Prednisone treatment resulted in a marked increase in muscle strength. Here we report another case of this interesting and treatable muscle disorder. A Japanese woman first developed neck muscle weakness, dysphasia, and weight loss at the age of 42 years. She was diagnosed as having anorexia nervosa and was placed on antidepressive drug treatment. Her symptoms worsened and after a few months she developed weakness in all of her limbs, sluggish speech, and diplopia, and began having difficulty with breathing. She presented in our outpatient clinic in 1993 at the age of 43. Her personal and family histories were negative for neuromuscular disorders. On physical examination, she was very thin (height 162 cm; weight 32 kg), and her temperature was 38.1°C. Coarse crackles were heard in the right lower lung. Neurological examination …