ILEUS IN MITOCHONDRIAL ENCEPHALOMYOPATHY, LACTIC-ACIDOSIS AND STROKE-LIKE EPISODES

Patient: A 39-year-old woman with mitochondrial encephalomyopathy, lac tic acidosis and stroke-like episodes (MELAS) who developed paralytic ileus and died of irreversible shock. Methods: Abdominal X-ray, autops y using light microscopy, electron microscopy and mitochondrial DNA an alysis. Results: Paralytic ileus was diagnosed. Several hours after ad mission the patient died from irreversible shock. At autopsy, ultrastr uctural examination of the small intestine revealed abnormal accumulat ion of mitochondria in smooth muscle cells. DNA analysis of the intest inal tissue showed a tRNA(Leu(UUR)) A-->G transition at nucleotide pos ition 3243 of the mitochondrial DNA. The amount of mutated mitochondri al DNA was markedly higher in the lamina muscularis than in the mucosa : 30% vs. 8%, Conclusions: Paralytic ileus may be due to mutated mitoc hondrial DNA which ultimately leads to smooth muscle dysfunction in th e small intestine. Recognizing mitochondrial DNA abnormalities as a ne w etiopathogenetic factor of paralytic ileus may become more important in clinical medicine in the near future. (C) 1998 Published by Elsevi er Science B.V, All rights reserved.