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Inclusion body myositis: clinical, morphological, physiological and laboratory findings in 18 cases
Author(s) -
Lindberg C.,
Persson Ll.,
Björkander J.,
Oldfors A.
Publication year - 1994
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1994.tb01647.x
Subject(s) - inclusion body myositis , prednisone , medicine , myositis , muscle weakness , weakness , dysphagia , polymyositis , proximal muscle weakness , dermatomyositis , pediatrics , physical therapy , surgery , muscle biopsy , biopsy
Eighteen consecutive patients with inclusion body myositis (IBM) were studied. The mean age of onset of symptoms was 60 years. A typical clinical pattern with insidious onset of muscle weakness in knee extensors and finger flexors combined with dysphagia was observed. Serial measurements of the maximal voluntary muscle strength revealed a mean loss of muscle strength of 1.4% per month. Two of the cases had common variable immunodeficiency, and three cases had reduced levels of the IgG3 subclass. Treatment with prednisone resulted in a temporary improvement of muscle function in three patients. No positive effect of azathioprine or cyclosporine A could be documented. The results show that IBM may be associated with immunodeficiency, and that prednisone treatment may temporarily improve the clinical signs. The results from our studies on the progression of the muscle weakness may provide basis for future studies on treatment of IBM.