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A Corticosteroid-Resistant Idiopathic Inflammatory Myopathy

Moris J. Danon, MD; Marcelino G. Reyes, MD; Osvaldo H. Perurena, MD; Joseph C. Masdeu, MD; Jose R. Manaligod, MD, PhD

Arch Neurol. 1982;39(12):760-764.

Abstract
• In seven patients with slowly progressive muscle weakness, inclusion body myositis (IBM) was diagnosed on biopsy. None had stigmata of collagenvascular disease or malignancy. Serum creatine kinase levels were mildly or moderately increased. The six patients treated with prednisone did not improve. Needle electromyography showed a "myopathic" pattern in all patients, but four also had diffuse neurogenic changes with normal nerve conductions. Histologic study of muscle showed a mixture of small rounded fibers varying in size, atrophic angulated fibers forming small groups, and hypertrophic fibers. Variable amounts of inflammation, necrosis, and regeneration were seen in all specimens. All showed numerous intracytoplasmic vacuoles lined with purple-blue granules. Electron microscopy showed membranous whorls and masses of abnormal filaments measuring 14 to 18 nm in diameter. Although IBM seems to be a distinct type of inflammatory myopathy, its etiology and pathogenesis are not clear.

Author Affiliations
From the Departments of Neurology (Drs Danon and Perurena) and Pathology (Drs Danon and Manaligod), University of Illinois Medical Center, Chicago, the Department of Pathology, Mount Sinai Medical Center, Chicago (Dr Reyes), and the Department of Neurology, Hines (Ill) Veterans Administration Hospital (Dr Masdeu).

Footnotes
Accepted for publication Feb 18, 1982.

Reprint requests to Department of Neurology, University of Illinois at the Medical Center, 912 S Wood St, Chicago, IL 60612 (Dr Danon).

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