Key result
Distal vacuolar myopathy featuring membranous whorls without filamentous inclusions can present with complete heart block and life-threatening cardiac disease.
Patients with distal vacuolar myopathy may present with life-threatening cardiac disease such as complete heart block.
May prompt cardiac screening in distal vacuolar myopathy; leaves open whether this represents a distinct entity needing prospective validation.
We describe a 26-year-old man with a predominantly distal myopathy and complete heart block. A muscle biopsy specimen demonstrated numerous vacuoles, which, by electron microscopy, contained membranous whorls. Filamentous inclusions characteristic of inclusion body myositis were not seen. It is important to be aware that life-threatening cardiac disease may occur in this setting.
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Krendel et al. (1988) studied this question. Distal vacuolar myopathy featuring membranous whorls without filamentous inclusions can present with complete heart block and life-threatening cardiac disease.
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