Summary
Among the 21 autopsied cases of Whipple's disease on file at the Armed Forces Institute of Pathology, 4 had neurological symptoms and prominent involvement of the central nervous system, By light microscopy the lesions were composed of nodular aggregates of macrophages having cytoplasm that stained a distinctive pale blue with hematoxylin-eosin and bright red with the periodic acid-Schiff technique. By electron microscopy the cerebral lesions revealed bacilli morphologically identical to and undergoing the same sequence of degenerative changes as those observed in the intestine. The periodic acid-Schiff reaction stains the walls and the capsular material that persists even after intact organisms disappear. These observations further support the infectious nature of Whipple's disease.
Zusammenfassung
Von 21 Fällen von Whipplescher Krankheit in der Sammlung des Armed Forces Institute of Pathology zeigten 4 Fälle neurologische Symptome mit besonders ausgeprägten Hirnläsionen. Lichtmikroskopisch bestanden die Herde aus knötchenförmigen Anhäufungen von Makrophagen, deren Cytoplasma mit Hämatoxylin-Eosin eine charakteristische schwachblaue und nach Durchführung der Perjodsäure-Schiff-Reaktion eine stark rote Färbung zeigte. Elektronenmikroskopisch fanden sich in den Hirnläsionen Bacillen, die morphologisch den im Darm beschriebenen glichen und die auch dieselben Degenerationserscheinungen aufwiesen. Die Perjodsäure-Schiff-Reaktion färbt Kapselmaterial und Zellwände,die auch nach Untergang der Bacillen noch nachweisbar sind. Diese Beobachtungen werden als weiterer Beweis für die infektiöse Ätiologie der Whippleschen Krankheit angeführt.
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References
Adams, W. R., A. W. Wolfsohn, andH. M. Spiro: Some morphologic characteristics of Whipple's disease. Amer. J. Path.42, 415–429 (1963).
Badenoch, J., W. C. D. Richards, andD. R. Oppenheimer: Encephalopathy in a case of Whipple's disease. J. Neurol. Neurosurg. Psychiat.26, 203–210 (1963).
Black-Schaffer, B.: Tinctoral demonstration of a glycoprotein in Whipple's disease. Proc. Soc. exp. Biol. (N. Y.)72, 225–227 (1949).
Caroli, J., A. R. Prevot, C. Julien, L. Gueritat etH. Straline: L'étiologie bactérienne de la maladie de Whipple. III. A propos d'une nouvelle observation. Isolement de Corynebacterium anaerobium. Arch. Mal. Appar. dig.52, 177–193 (1963).
Chears, W. C., Jr., andC. T. Ashworth: Electron microscopic study of the intestinal mucosa in Whipple's disease. Demonstration of encapsulated bacilliform bodies in the lesion. Gastroenterology41, 129–138 (1961).
Cohen, A. S., E. M. Schemel, P. R. Holt, andK. J. Isselbacher: Ultrastructural abnormalities in Whipple's disease. Proc. Soc. exp. Biol. (N.Y.)105, 411 (1960).
Davis, T. D., Jr., J. W. McBee, J. L. Borland, Jr., S. M. Kurtz,andJ. M. Ruffin: The effect of antibiotic and steriod therapy in Whipple's disease. Gastroenterology44, 112–116 (1963).
Dobbins, W. O., andJ. M. Ruffin: A light and electron microsocpic study of bacterial invasion in Whipple's disease. Amer. J. Path.51, 225–242 (1967).
England, M. T., J. M. French, andA. B. Rawson: Antibiotic control of diarrhea in Whipple's disease. Gastroenterology39, 219–231 (1960).
Enzinger, F. M., andE. B. Helwig: Whipple's disease. A review of the literature and report of fifteen patients. Virchows Arch. path. Anat.336, 238–269 (1963).
Farnan, P.: The systemic lesions of Whipple's disease. J. clin. Path.11, 382–390 (1958).
Fisher, E. R.: Whipple's disease: Pathogenetic considerations: Electron microscopic and histochemical observations. J. Amer. med. Ass.181, 396–403 (1962).
Haubrich, W. S., J. H. L. Watson, andJ. C. Sieracki: Unique morphologic features of Whipple's disease. A study by light and electron microscopy. Gastroenterology39, 454 to 468 (1960).
Hollenberg, M.: Whipple's disease. A case report with enzyme histochemical and electron microscopic findings. Amer. J. Med.32, 448–459 (1962).
Kent, T. J., J. M. Layton, J. A. Clifton, andH. P. Schede: Whipple's disease: Light and electron microscopic studies combined with clinical studies suggesting an infective nature. Lab. Invest.12, 1163–1178 (1963).
Kjaerheim, A., T. Midtredt, S. Skrede, andE. Gjone: Bacteria in Whipple's disease. Isolation of a Haemophilus strain from the jejunal propria. Acta path. microbiol. scand.66, 135–143 (1966).
Kok, N., R. Dybkaer, andJ. Rostgaard: Bacteria in Whipple's disease. Acta path. microbiol. scand.60, 431–449 (1964).
Krücke, W., u.O. Stochdorph: Über Veränderungen im Zentralnervensystem bei Whipplescher Krankheit. Verh. dtsch. Ges. Path.46, 198–202 (1962).
Kurtz, S. M., T. D. Davis, Jr., andJ. M. Ruffin: Light and electron microscopic studies of Whipple's disease. Lab. Invest.11, 653–655 (1962).
Lampert, P., M. I. Tom, andJ. N. Cumings: Encephalopathy in Whipple's disease. Neurology (Minneap.)12, 65–71 (1962).
Perkins, H. R.: Chemical structure and biosynthesis of bacterial cell walls. Bact. Rev.27, 18–55 (1963).
Phillips, M. J., andJ. M. Finlay: Bacilli-lipid associations in Whipple's disease. J. Path. Bact.94, 131–137 (1967).
Sherris, J. C., C. E. Roberts,andR. L. Porus: Microbiological studies of intestinal biopsies taken during active Whipple's disease. Gastroenterology48, 708–710 (1965).
Sieracki, J. C.: Whipple's disease: Observations on systemic involvement.I. Cytologic observations. Arch. Path.66, 464–467 (1958).
—,G. Fine, R. C. Horn, andJ. Bebin: Central nervous system involvement in Whipple's disease. J. Neuropath. exp. Neurol.19, 70–75 (1960).
Smith, W. T., J. M. French, M. Gottsman, A. J. Smith, andJ. A. Wakes-Miller: Cerebral complications of Whipple's disease. Brain88, 137–150 (1965).
Trier, J. S., andP. C. Phelps: Serial observations of the structure of the small intestinal mucosa in Whipple's disease. J. Lab. clin. Med.62, 1017 (1963).
——,S. Eidelman, andC. E. Rubin: Whipple's disease: Light and electron microscopic correlation of jejunal mucosal histology with antibiotic treatment and clinical status. Gastroenterology48, 684–707 (1965).
Upton, A. C.: Histochemical investigation of mesenchymal lesions in Whipple's disease. Amer. J. clin. Path.22, 755–764 (1952).
Whipple, G. H.: A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues (intestinal lipodistrophy). Bull. Johns Hopk. Hosp.18, 382–391 (1907).
Yardley, J. H., andT. R. Hendrix: Combined electron and light microscopy in Whipple's disease. Demonstration of “bacillary bodies” in the intestine. Bull. Johns Hopk. Hosp.109, 80–98 (1961).
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Schochet, S.S., Lampert, P.W. Granulomatous encephalitis in Whipple's disease. Acta Neuropathol 13, 1–11 (1969). https://doi.org/10.1007/BF00686137
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DOI: https://doi.org/10.1007/BF00686137