Inclusion body myositis coexisting with hypertrophic cardiomyopathy: an autopsy study

Neuromuscular Disorders : NMD
Yukie InamoriHiroshi Takashima

Abstract

Inclusion body myositis is an inflammatory myopathy characterized pathologically by rimmed vacuoles and the accumulation of amyloid-related proteins. Autopsy studies in these patients, including histochemical examinations of multiple skeletal muscles, have not yet been published. In this paper, we describe the autopsy findings of a patient with inclusion body myositis and hypertrophic cardiomyopathy. A 69-year-old man, who was a human T lymphotropic virus type 1 carrier, exhibited slowly progressive muscle weakness and atrophy, predominantly affecting the scapular, quadriceps femoris, and forearm flexor muscles. His disease course was more rapidly progressive than that typically observed; the patient died suddenly of arrhythmia 5 years after diagnosis. Autopsy findings revealed that multiple muscles, including the respiratory muscles, were involved. Longitudinal studies revealed an increased frequency of rimmed vacuoles and p62/sequestosome 1- and/or TAR DNA-binding protein 43-positive deposits in autopsied muscles, although the amount of inflammatory infiltrate appeared to be decreased. We speculated that muscle degeneration may be more closely involved in disease progression compared with autoimmunity. Genetic analysis reveal...Continue Reading

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Citations

Feb 22, 2014·Age·Alessandro BittoChristian Sell
Jan 19, 2016·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Merrilee Needham, Frank L Mastaglia
Dec 3, 2014·International Journal of Cardiology·Claudia StöllbergerJosef Finsterer

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