Neurology of Whipple's disease
- Queen Elizabeth Neuroscience Centre, Edgbaston
- Birmingham B15 2TH, UK
- email Milne.Anderson@btinternet.com
In 1907, George Hoyt Whipple, then an instructor in pathology at Johns Hopkins University, published a case report of a 36 year old physician who had been domiciled in Constantinople.1 He developed recurring arthropathy, weight loss, and steatorrhoea, became worse, and died. At necropsy the intestine and mesenteric lymph glands were infiltrated by mononuclear and polynuclear giant cells and deposits of fat and fatty acids. Whipple described the large “foamy” mononuclear cells in the intestinal mucosa which were later shown to contain periodic acid Schiff (PAS) positive material. The aetiology of the condition was not established and he wrote “Gland tissue treated by the Levaditi method shows great numbers of a peculiar rod-shaped organism (?) which does not stain by the aniline dyes . . .” Whether this is the aetiological factor in this disease cannot be determined from this case”. A prescient observation. He suggested intestinal lipodystrophy as a name for the disease.
General features
Whipple's disease is rare—since its first description less than 1000 cases have been reported in the literature which is certainly an underestimate of all cases. Men are affected much more often than women (over 80%) and the mean age of onset approaches 50 although the age range extends from childhood to senility. Cases which have been reported have originated from Europe and North America in the main. Whether this is a true reflection of its aetiology or relates to technical difficulties in reaching a diagnosis is not clear. The natural habitat of the organism is not known, nor are the mechanisms by which infection takes place. The number of cases described in reports is too low to determine whether there is a racial susceptibility. The most common clinical presentation is a malabsorption syndrome with diarrhoea, abdominal pain, weight loss, generalised wasting, variable fever, and lymphadenopathy. …
