Inclusion body myositis: clinical, morphological, physiological and laboratory findings in 18 cases

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.

[1]  W. Litchy,et al.  Inclusion body myositis with cricopharyngeus muscle involvement and severe dysphagia. , 1992, Muscle and Nerve.

[2]  A. Eisen,et al.  Inclusion body myositis , 1978, Neurology.

[3]  M. Chevallay,et al.  Inclusion body myositis. , 1981, Acta neuropathologica. Supplementum.

[4]  Kucharz Ej.  [Inclusion body myositis]. , 1998, Polskie Archiwum Medycyny Wewnetrznej.

[5]  P. Behan,et al.  The inflammatory process in polymyositis: monoclonal antibody analysis of muscle and peripheral blood immunoregulatory lymphocytes. , 1987, Journal of neurology, neurosurgery, and psychiatry.

[6]  H. Mitsumoto,et al.  Inclusion body myositis presenting as treatment-resistant polymyositis. , 1987, Arthritis & Rheumatism.

[7]  Brain.  Aids to the Examination of the Peripheral Nervous System , 1987 .

[8]  P. Trend,et al.  Cyclosporin in the management of polymyositis and dermatomyositis. , 1991, Journal of neurology, neurosurgery, and psychiatry.

[9]  A. Engel,et al.  Azathioprine with prednisone for polymyositis. A controlled, clinical trial. , 1980, Annals of internal medicine.

[10]  A. Hedström,et al.  Inclusion body myositis and welander distal myopathy: a clinical, neurophysiological and morphological comparison , 1991, Journal of the Neurological Sciences.

[11]  J. Fulthorpe,et al.  Inclusion body myositis: a case with associated collagen vascular disease responding to treatment. , 1985, Journal of neurology, neurosurgery, and psychiatry.

[12]  P. Tsairis.  Muscle Biopsy: A Modern Approach , 1974 .

[13]  H. Neville,et al.  Spectrum of inclusion body myositis. , 1987, Archives of neurology.

[14]  B. Bake,et al.  Primary hypogammaglobulinaemia: impaired lung function and body growth with delayed diagnosis and inadequate treatment. , 1984, European journal of respiratory diseases.

[15]  M. Dalakas.  Polymyositis, dermatomyositis and inclusion-body myositis. , 1991, The New England journal of medicine.

[16]  C. Laurell,et al.  Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. , 1966, Analytical biochemistry.

[17]  G. Gibson,et al.  Inclusion body myositis (IBM) , 1983, Neurology.

[18]  W. Bradley,et al.  Inclusion body myositis with cricopharyngeus muscle involvement and severe dysphagia , 1991, Muscle & nerve.

[19]  R. Kuzniecky,et al.  Familial myopathy with changes resembling inclusion body myositis and periventricular leucoencephalopathy. A new syndrome. , 1988, Brain : a journal of neurology.

[20]  P. Phillips,et al.  Increased toxoplasma antibodies in idiopathic inflammatory muscle disease. A case-controlled study. , 1979, Arthritis and rheumatism.

[21]  Chou Sm,et al.  Inclusion body myositis: analysis of 32 cases. , 1992, The Journal of rheumatology.

[22]  W J Litchy,et al.  Inclusion body myositis. Observations in 40 patients. , 1989, Brain : a journal of neurology.

[23]  R. Wortmann,et al.  Clinical heterogeneity and treatment response in inclusion body myositis. , 1989, Arthritis and rheumatism.

[24]  S. Ohman,et al.  Principles of albumin and IgG analyses in neurological disorders. I. Establishment of reference values. , 1977, Scandinavian journal of clinical and laboratory investigation.

[25]  A. Hedström,et al.  Inclusion body myositis: peripheral nerve involvement Combined morphological and electrophysiological studies on peripheral nerves , 1990, Journal of the Neurological Sciences.

[26]  J. Masdeu,et al.  Inclusion body myositis. A corticosteroid-resistant idiopathic inflammatory myopathy. , 1982, Archives of neurology.

[27]  M. Dalakas,et al.  Clinical, immunopathologic, and therapeutic considerations of inflammatory myopathies. , 1992, Clinical neuropharmacology.

[28]  A. Engel,et al.  Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells , 1984, Annals of neurology.

[29]  J. Julien,et al.  Presence of inclusion body myositis‐like filaments in oculopharyngeal muscular dystrophy. Ultrastructural study of 10 cases , 1990, Neuropathology and applied neurobiology.

[30]  O. Borges.  Isometric and isokinetic knee extension and flexion torque in men and women aged 20-70. , 1989, Scandinavian journal of rehabilitation medicine.

[31]  C. Wiles,et al.  The measurement of muscle strength in patients with peripheral neuromuscular disorders. , 1983, Journal of neurology, neurosurgery, and psychiatry.

[32]  J. Vallat,et al.  Inclusion body myositis Clinical, biological and ultrastructural study , 1982, Journal of the Neurological Sciences.