Summary
Whipple's disease presenting as a neurological disease without gastrointestinal symptoms is an unusual occurrence. A 40 year old man suffered hypersomnia, memory loss and progressive ophthalmoplegia for 6 months prior to death. The nature of his disease was not established during life. Extensive granulomatous inflammation affecting the hypothalamus, hippocampus and periaqueductal gray matter of the brain was found to represent Whipple's disease by electron microscopy. Characteristic lesions were also present in spleen, mesenteric lymph nodes, small intestine and myocardium. Bacillary bodies and membranous inclusions similar to those seen in visceral lesions of Whipple's disease were present in macrophages. The findings supported the theory of direct involvement of the central nervous system by bacilli rather than a metabolic origin for the lesions.
Similar content being viewed by others
References
Badenoch, J., Richards, W. C. D., Oppenheimer, D. R.: Encephalopathy in a case of Whipple's disease. J. Neurol. Neurosurg. Psychiat.26, 203–210 (1963)
Chears, W. C., Jr., Ashworth, C. T.: Electron microscopic study of the intestinal mucosa in Whipple's disease. Demonstration of encapsulated bacilliform bodies in the lesion. Gastroenterology41, 129–138 (1961)
DeGroodt-Lasseel, M., Martin, J. J.: Étude ultrastructurale des lesions du systeme nerveux central dans la maladie de Whipple. Path. et Biol.17, 121–131 (1969)
Enzinger, F. M., Helwig, E. B.: Whipple's disease. A review of the literature and report of fifteen patients. Virchows Arch. path. Anat.336, 238–269 (1963)
Kitamura, T.: Brain involvement in Whipple's disease. A case report. Acta neuropath. (Berl.)33, 275–278 (1975)
Kodousek, R., Kojecky, Z.: On the basis of cerebral lesions in Whipple's disease. Rev. Czech. Med.17, 137–143 (1971)
Koudouris, S. D., Stern, T. N., Utterback, R. A.: Involvement of central nervous system in Whipple's disease. Neurology (Minneap.)13, 397–404 (1963)
Krücke, W., Stochdorph, O.: Über Veränderungen im Zentralnervensystem bei Wipplescher Krankheit. Verh. dtsch. Ges. Path.46, 198–202 (1962)
Lamberty, J., Varela, P. Y., Font, R. G., Jarvis, B. W., Coover, J.: Whipple disease. Light and electron microscopy study. Arch. Path.98, 325–330 (1974)
Lampert, P., Tom, M. I., Cummings, J. N.: Encephalopathy in Whipple's disease. A histochemical study. Neurology (Minneap.)12, 65–71 (1962)
Maizel, H., Ruffin, J. M., Dobbins, W. O.: Whipple's disease: a review of 19 patients from one hospital and a review of the literature since 1950 Medicine (Baltimore)49, 175–205 (1970)
Minauf, M., Stochdorph, O.: Das ZNS bei Morbus Whipple. Arch. Psychiat. Nervenkr.212, 180–199 (1969)
Roberts, D. M., Themann, H., Knust, F.-J., Preston F. E., Donaldson, J. R.: An electron-microscope study of bacteria in two cases of Whipple's disease. J. Path.100, 249–255 (1970)
Schochet, S. S., Jr., Lampert, P. W.: Granulomatous encephalitis in Whipple's disease. Electron microscopic observations. Acta neuropath. (Berl.)13, 1–11 (1969)
Sieracki, J. C., Fine, G., Horn, R. C., Bebin, J.: Central nervous system involvement in Whipple's disease. J. Neuropath. exp. Neurol.19, 70–75 (1960)
Smith, W. T., French, J. M., Gottsman, M., Smith, A. J., Wakes-Miller, J. A.: Cerebral complications of Whipple's disease. Brain88, 137–150 (1965)
Stoupel, N., Monseu, G., Pardoe, A., Heimann, R., Martin, J. J.: Encephalitis with myoclous in Whipple's disease. J. Neurol. Neurosurg. Psychiat.32, 338–343 (1969)
Sun, C. N., White, H. J., Hall, W. H.: Fission of Whipple's disease rod bodies. Amer. J. dig. Dis.18, 532–535 (1973)
Switz, D. M., Casey, T. R., Bogaty, G. V.: Whipple's disease and papilledema. An unreported presentation. Arch. intern. Med.123, 74–77 (1969)
Trier, J. S., Phelps, P. C., Eidelman, S., Rubin, C. E.: Whipple's disease: light and electron microscope correlation of jejunal mucosal histology with antibiotic treatment and clinical status. Gastroenterology48, 684–707 (1965)
Yardley, J. H., Hendrix, T. R.: Combined electron and light microscopy in Whipple's disease. Demonstration of “bacillary bodies” in the intestine. Bull. Johns Hopk. Hosp.109, 80–98 (1961)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Silbert, S.W., Parker, E. & Horenstein, S. Whipple's disease of the central nervous system. Acta Neuropathol 36, 31–38 (1976). https://doi.org/10.1007/BF00685145
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00685145