Summary
Neuropathological examination of the gut in a patient with chronic idiopathic intestinal pseudoobstruction and temporal lobe epilepsy showed a degeneration of Auerbach's and of Meissner's plexus. The extent of degenerative changes increased in an aboral to oral direction. Neuronal degeneration was characterized by ballooning of the cytoplasm of the ganglial cells, by a hyperargyrophilia, a shortening and dilation of cell processes, and a progressive fragmentation and loss of axoris. In rectal biopsies, a PAS-positive granular material was detected in the cytoplasm of ballooned ganglial cells. A severe peripheral neuropathy with fiber degeneration in the posterior columns and axonal lesions within ventral nerve roots and a more recent fiber degeneration in the lateral columns corresponding to the picture of subacute combined degeneration of the spinal cord and a symmetrical Ammon's horn sclerosis was present. This case of progressive intestinal neuronal degeneration of unknown origin is a newly described condition leading to chronic idiopathic intestinal pseudoobstruction.
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References
Faulk DL, Anuras S, Christensen J: Chronic intestinal pseudoobstruction. Gastroenterology 74:922–931, 1978
Anuras S, Crane SA, Faulk DL, Hubel KA: Intestinal pseudoobstruction. Gastroenterology 74:1318–1324, 1978
Bachulis BL, Smith PE: Pseudoobstruction of the colon. Am J Sur 136:66–72, 1978
Maldonado JE, Gregg JA, Green PA, Brown AL: Chronic idiopathic intestinal pseudoobstruction. Am J Med 49:203–212, 1970
Shuffler MD, Rohrmann CA, Chaffee RG, Brand DL, Delaney JH, Young JH: Chronic intestinal pseudoobstruction: A report of 27 cases and review of the literature. Medicine 60:173–196, 1981
Shuffler MD, Lowe MC, Bill AH: Studies of idiopathic intestinal pseudoobstruction. I. Hereditary hollow visceral myopathy: Clinical and pathological studies. Gastroenterology 73:327–338, 1977
Faulk DL, Anuras S, Gardner GD, Mitros FA, Summers RW, Christensen J: A familial visceral myopathy. Ann Intern Med 89:600–606, 1978
Shuffler MD, Beegle RG: Progressive systemic scle rosis of the gastrointestinal tract and hollow visceral myopathy: Two distinguishable disorders of intestinal smooth muscle. Gastroenterology 77:664–671, 1979
Shuffler MD, Bird TD, Sumi SM, Cook A: A familial neuronal disease presenting as intestinal pseudoobstruction. Gastroenterology 75:889–898, 1978
Cockel R, Hill EE, Rushton DI, Smith B, Hawkins CF: Familial steatorrhea with calcification of the basal ganglia and mental retardation Q J Med 42:771–783, 1973
Dyer NH, Dawson AM, Smith BF, Todd IP: Obstruction of bowel due to lesion in the myenteric plexus. Br Med J 1:686–689, 1969
Shuffler MD, Jonak Z: Chronic idiopathic intestinal pseudoobstruction caused by a degenerative disorder of the myenteric plexus: The use of Smith's method to define the neuropathology. Gastroenterology 82:476–486, 1982
Krishnamurthy S, Shuffler MD, Rohrmann CA, Pope CE: Severe idiopathic constipation is associated with a distinctive abnormality of the colonic myenteric plexus. Gastroenterology 88:26–34, 1985
Karnovsky MU, Roots L: A direct-coloring thiocholine method for cholinesterases. J Histochem Cytochem 12:211–219, 1964
Lindvall O, Björklund A: The glyoxylic acid fluorescence histochemical method: A detailed account of the methodology for the visualization of central catecholamine neurons. Histochemistry 36:97–127, 1974
Bodian M, Lake BA: The rectal approach to neuro pathology. Br J Surg 50:702–714, 1962
Romeis B: Mikroskopische Technik, 15th ed. München, Leibnitz Verlag, 1948
Pearse AGE: Histochemistry, Theoretical and Applied. London, J and A Churchill, 1953
Cauna N: The mode of termination of sensory nerves and its significance. J Comp Neurol 113:169–210, 1959
O'Brien JS, Okada S, Chen A, Fillerup DL: Tay-Sachs disease: Detection of heterozygotes and homozygotes by serum hexosaminidase assay. N Engl J Med 283:115, 1970
Suzuki J, Hawakawa T, Yazaki M, Hiratani Y: GM-gangliosidosis. A variant with high activity of hepatic neutral β-galactosidase Eur J Pediatr 122:177–186, 1976
Wenger DA, Clark C, Sattler M, Wharton C: Synthetic substrate β-glucosidase activity in leucozytes: A reproductible method for the identification of patients and carriers of Gaucher's disease. Clin Genet 13:143–153, 1978
Kampine JP, Brady RO, Yankee RA, Kanfer JN, Shapiro D, Gal AE: Sphingolipid metabolism in leucemic leucocytes. Cancer Res 27:1312–1315, 1976
Folch J, Lees M, Sloane-Stanley GH: A simple method for the isolation and purification of total lipids from animal tissues. J Biol Chem 226:497–509, 1957
Jatzkewitz H: Eine neue Methode zur quantitativen Ultramikrobestimmung der Spingolipide aus dem Gehirn. Hoppe-Seylers Z Phys Chem 326:61–64, 1961
Suzuki K: The pattern of mammalian brain gangliosides II. J Neurochem 12:629–638, 1965
Ueno K, Ando S, Yu RK: Gangliosides of human cat and rabbit spinal cords and myelins. J Lipid Res 19:863–871, 1978
Rose M: Der Allocortex bei Tier und Mensch I. und II. J Psychol 34:1–30; 35:42–76, 1926/7
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Schuster, R., Ferenci, P., Schmidbauer, M. et al. Intestinal neuronal degeneration in a patient with chronic idiopathic intestinal pseudoobstruction. Digest Dis Sci 34, 123–128 (1989). https://doi.org/10.1007/BF01536166
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DOI: https://doi.org/10.1007/BF01536166