The Evolving Natural History of Neurologic Involvement in Whipple Disease: A Hypothesis

To the Editor. —George Hoyt Whipple, Nobel Laureate and founding Dean of the University of Rochester (NY) School of Medicine and Dentistry, reported the initial description of intestinal lipodystrophy in 1907.1Chronic diarrhea with steatorrhea, weight loss, abdominal pain, lymphadenopathy, arthritis, and constitutional symptoms such as fever and weakness are frequent clinical features. The pathologic hallmark of the disorder is the presence of periodic acid-Schiff-positive rods and sickle-shaped inclusions in macrophages, which, by electron microscopy, are bacilliform particles. Neurologic manifestations occur in at least 10% of patients and include alterations of mental function with dementia, abnormalities of eye movement reflecting midbrain involvement, sleep disturbance, impotence, polydipsia and polyphagia reflecting hypothalamic involvement, seizures, meningoencephalitis, myelopathy, ataxia, and myoclonus. The etiopathogenesis of Whipple's disease remains incompletely defined, although an infectious process is implicated in part because bacilliform structures have been seen by electron microscopy and the response to antibiotics.2