Encephalopathy in Whipple's disease

Neurology of Whipple's disease

“…Precedence in describing changes in the CNS in Whipple's disease is given to Sieracki22 and Sieracki et al 23 who were closely followed by Lampert et al 24 and Badenoch et al 25. Since then numerous descriptions of pathological changes in the CNS have appeared26-28 and in 1977 Romanul et al 29 published details of a case in which the disease seemed to be confined to the CNS.…”

Whipple's disease confined to the brain: a case studied clinically and pathologically

“…The picture was that of progressive dementia with rather inconspicuous pyramidal or extrapyramidal signs, ending in an akinetic mute state and eventual coma over a period of a few months to seven years (Krucke and Stochdorph, 1962;Lampert et al, 1962;Badenoch et al, 1963;Smith et al, 1965;Schwartzova et al, 1967;Minauf and Stochdorph, 1969;Silbert et al, 1976). Several patients had disorders of eye movements (Lampert et al, 1962;Badenoch et al, 1963;Smith et al, 1965;Minauf and Stochdorph 1969;Stoupel et al, 1969) and seizures or myoclonus (Lampert et al, 1962;Smith et al, 1965;Minauf and Stochdorph, 1969;Stoupel et al, 1969). One patien had papilloedema (Lampert et al, 1962).…”

“…The lesions in the central nervous system are identical to those in the small intestine both with the light (Sieracki et al, 1960) and the electron microscope (Groodt-Lassell and Schochet and Lampert, 1969). There are 21 cases in the literature in which the brain was found to contain the lesions at necropsy (Rutishauser and Borer, 1960;Sieracki et al, 1960;Krucke and Stochdorph, 1962;Lampert et al, 1962;Badenoch et al, 1963;Smith et al, 1965;Schwartzova et al, 1967;Groodt-Lassell and Martin, 1969;Minauf and Stochdorph, 1969;Schochet and Lampert, 1969;Stoupel et al, 1969;Switz et al, 1969;Kodousek and Kojecki, 1971;Janota, 1974;Kitamura, 1975;Silbert et al, 1976). In two of these (Rutishauser and Borer, 1960;Janota, 1974) the examination of the important organs was not available but the patients had clinical evidence of systemic involvement.…”

Whipple's disease with minimal intestinal involvement.

“…When Whipple's disease involves the central nervous system, the intestine does not appear to be either unusually spared or particularly severely involved (Sieracki et al, 1960;Smith et al, 1965). Lampert et al (1962) reported a fatal case of Whipple's encephalopathy in which the patient had no diarrhoea but had typical intestinal and mesenteric nodal lesions at necropsy. In a report by Knox et al (1968), a positive peroral intestinal biopsy established the diagnosis of Whipple's disease in a patient with ocular inflammation and minimal intestinal involvement, and it is possible that early treatment prevented more serious CNS disease.…”

“…However, in some cases signs and symptoms referable to the gastrointestinal tract are either absent or appear late in the course of the disease, and involvement of the central nervous system, joints or lymphatic system, or simply fever, may be the major clinical manifestations (Hargrove et al, 1960;Lampert et al, 1962). We are reporting the gastroenterological aspects of an unusual case of Whipple's disease, in which, despite the absence of overt intestinal involvement, and with only a patchy histological lesion, the diagnosis was confirmed by electron-microscopic examination of peroral intestinal biopsies.…”