© 2003 BMJ Publishing Group & Association of Clinical Pathologists
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Myopathy and an unusual serum antibody point to a defined syndrome
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A study of patients presenting over three years with syndromes affecting skeletal muscles has found that those with serum antibody to the signal recognition particle (SRP) represent a defined syndrome. Empirical evidence suggests that early corticosteroid treatment may improve muscle strength.
Seven patients had antibodies to SRP but no antibodies linked with other myopathies. Their age range was 32–70 years and five were women. In most patients the disease started in the autumn (August-January), and all had similar signs and symptoms—severe, rapidly increasing weakness of proximal muscles, leading to disability. Muscle strength was recorded in some proximal muscles as MRC grade 0/5 in five patients and 3/5 in two. The weakness was always greatest in the deltoid and psoas muscles. Sensation was unaffected. Creatine kinase was greatly increased, up to 3000–25 000 U/l, and active myopathy was confirmed by electromyography.
Biopsy specimens of affected muscles showed active degeneration and regeneration
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