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Clinical and Morphofunctional Features of Idiopathic Myenteric Ganglionitis Underlying Severe Intestinal Motor Dysfunction: A Study of Three Cases Roberto De Giorgio, M.D., Ph.D., Giovanni Barbara, M.D., Vincenzo Stanghellini, M.D., Fabrizio De Ponti, M.D., Ph.D., Beatrice Salvioli, M.D., Marcello Tonini, Ph.D., Pietro Velio, M.D., Gabrio Bassotti, M.D., Ph.D., and Roberto Corinaldesi, M.D. Departments of Internal Medicine and Gastroenterology and Pharmacology, University of Bologna, Bologna, Italy; Department of Physiological and Pharmacological Sciences, University of Pavia, Pavia, Italy; Department of Medical Sciences, University of Milan, Milan, Italy; and Department of Clinical and Experimental Medicine, Division of Gastroenterology and Hepatology, University of Perugia, Perugia, Italy ABSTRACT Ganglionitis, i.e., the inflammatory neuropathy character- ized by a marked lymphoplasmacellular infiltrate in the myenteric plexus, may underlie a variety of paraneoplastic, infectious, or neurological disorders, although occasional cases are idiopathic in origin. We report clinical, manomet- ric, morphofunctional, and immunological features of three cases of idiopathic ganglionitis. All patients had megacolon and underwent surgery for repeated episodes of intestinal subocclusion. Esophageal, GI, and colonic manometry per- formed in one patient showed dysmotility of the whole gut. Histological examination of colonic and ileum specimens identified a prominent lymphoplasmacellular infiltrate within the myenteric plexus along with a marked decrease of a wide array of neuronal peptides/transmitters. In one pa- tient, tissue analysis revealed progressive neuronal changes up to marked myenteric neuron damage. The inflammatory infiltrate in all patients comprised CD4
and CD8
T lymphocytes. Abundance of both subclasses of lymphocytes suggests that immune-mediated mechanisms were respon- sible for neuronal degeneration. In one patient, systemic steroid therapy brought a signif