Sporadic visceral myopathy with inclusion bodies. A light-microscopic and ultrastructural study

We report the clinical and pathologic findings of two patients with sporadic visceral myopathy. Both presented with chronic intestinal pseudo-obstruction that necessitated colectomy. Microscopically, typical changes of primary visceral myopathy were present, including variable fibrous replacement of the muscularis externa and vacuolar degeneration of myocytes. In addition, the muscle cells contained cytoplasmic inclusions that have only been recently reported in visceral myopathy. These inclusions were numerous and easily visible in routine hematoxylin-eosin-stained sections but greatly enhanced by periodic acid-Schiff staining. They were reactive immunohistochemically at their periphery and were seen to be myofibrils at various stages of degeneration on electron microscopy. Inclusions were present in both muscularis externae and muscularis mucosae and were identified in mucosal biopsy specimens, providing a means of diagnosing this type of myopathic change on endoscopic biopsies.