Inclusion Body Myositis? Distal Myopathy?

J Korean Neurol Assoc > Volume 11(3); 1993 > Article

Journal of the Korean Neurological Association 1993;11(3): 427-433.

봉입체 근염? 원위부 근질환?

김성민, 이현미,송홍기,김상윤,권기한,이병철,하창원,지제근

한림대학교 신경과. 서울대학교 병리학.

Inclusion Body Myositis? Distal Myopathy?

Sung-Min Kim, M.D., Hyeon-Mi Lee, M.D., Hong-Ki Song, M.D., Sang-Yoon Kim, M.D., Ki-Han Kwon, M.D., Byung-Chul Lee, M.D., Chang-Won Ha, M.D., Je-Geun Chi, M.D.

Department of Neurology, College of Medicine Hallym University, Department of Pathology, College of Medicine Seoul National University

Abstract

Inclusion body myositis (IBM) is a chronic inflammatory myopathy with characteristic rimmed vacuoles. Cytoplasmic and intranuclear filamentous inclusions in muscle speci men. The clinical features manifest male dominance with the onset between the second and eighth decade but usually after the age of 50 years. Slow progression of painless muscle weakness, normal or mildly elevated serum CK level, myopathic and neurogenic eIectromyographic patterns and refractory to steriod therapy. The presence of rimmed vacuoles and filamentous inclusions in myofiber gives an important clue in the diagnosis of IBM, however, it is very difficult to differentiated young age onset IBM with distally predominant muscle weakness from distal myopathy with rimmed vacuole formation. While we present a young female patient who had slow progression of painless distal muscle weakness in both upper and lower extremities for 1 year, previously published articles concerning of IBM and distal myopathy with rimmed vacuole formation are reviewed and our own differential points are discussed in the diagnosis of this case as IBM.

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