Brain, Vol 120, Issue 1 39-45, Copyright © 1997 by Oxford University Press
G Blume, A Pestronk, B Frank and DR Johns
We evaluated 10 patients with histologically typical polymyositis except
for an excess of muscle fibres with absent cytochrome oxidase (COX)
staining. No biopsies had vacuoles or congophilic material in muscle
fibres. All patients presented with a history of slowly progressive
weakness. The average age of onset was 9 years older than a group of
polymyositis patients with normal COX staining of muscle fibres. Selective
weakness of knee extension was a prominent and disabling feature in most
patients. Serum creatine kinase was usually mildly elevated (363 +/- 115
U/l) but at levels lower than those in other patients with polymyositis.
Mitochondrial DNA analysis showed multiple deletions in 90% of muscles from
patients with excessive numbers of COX-negative muscle fibres, a prevalence
significantly greater than the 22% figure for controls (P = 0.005). As a
group, the patients responded poorly to immunosuppressive therapy. We
conclude that patients with polymyositis and an excess of COX-negative
muscle fibres, but no inclusion bodies, have common features including
selective quadriceps weakness, mitochondrial pathology by histochemical and
DNA analysis and a poor response to immunosuppressive therapy. Some of
these features are shared with inclusion body myositis (IBM) and this
entity cannot be entirely excluded as vacuoles may not be present in all
muscle tissue in IBM patients. Evaluation of the COX activity in muscle
fibres of patients with inflammatory myopathies provides useful prognostic
information regarding the likelihood of improved strength after
immunosuppressive treatment.
ARTICLES
Polymyositis with cytochrome oxidase negative muscle fibres. Early quadriceps weakness and poor response to immunosuppressive therapy
Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA.
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