Axonal Dystrophy Presenting as the Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome
- 1 January 1992
- journal article
- case report
- Published by Taylor & Francis in Pediatric Pathology
- Vol. 12 (5) , 743-750
- https://doi.org/10.3109/15513819209024229
Abstract
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a neonatal intestinal syndrome, characterized by defective peristalsis and bladder dilatation, refractory to pharmacological treatment. Examinations of bowel and bladder have failed to demonstrate a pathological explanation for this syndrome. We describe a 7-month-old female infant with MMIHS who had generalized axonal dystrophy of her central, peripheral, and autonomic nervous systems, which may provide a neuropathological explanation for some cases of MMIHS.Keywords
This publication has 12 references indexed in Scilit:
- Neuroaxonal Dystrophy Due to Lysosomal α-N-Acetylgalactosaminidase DeficiencyNew England Journal of Medicine, 1989
- The megacystis-microcolon-intestinal hypoperistalsis syndrome: a fatal autosomal recessive condition.Journal of Medical Genetics, 1989
- The association of infantile osteopetrosis and neuronal storage disease in two brothersActa Neuropathologica, 1988
- Neuroaxonal dystrophy presenting with neonatal dysmorphic features, early onset of peripheral gangrene, and a rapidly lethal courseAmerican Journal of Medical Genetics, 1987
- Megacystic, microcolon, intestinal hypoperistalsis syndrome: Possible pathogenesisJournal of Pediatric Surgery, 1984
- Megacystis-microcolon-intestinal hypoperistalsis syndrome: A visceral myopathyJournal of Pediatric Surgery, 1983
- Megacystis-microcolon-intestinal hypoperistalsis syndrome: additional clinical, radiologic, surgical, and histopathologic aspectsAmerican Journal of Roentgenology, 1981
- Neuroaxonal dystrophy in the neonateActa Neuropathologica, 1979
- Megacystis-microcolon-intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girlsAmerican Journal of Roentgenology, 1976
- AMERICAN ASSOCIATION OF NEUROPATHOLOGISTSJournal of Neuropathology and Experimental Neurology, 1963