What is Whipple’s disease?

Diseases & Conditions > Public Health > What is Whipple’s disease?
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Whipple’s disease is a rare bacterial infection primarily affecting the small intestine. It can also affect the heart, lungs, brain, joints, and eyes. Left untreated, Whipple’s disease is fatal.

Introduction

Background
Whipple disease constitutes a rare, relapsing, slowly progressive, infectious, systemic illness characterized by fever of unknown origin, polyarthralgias, and chronic diarrhea.
Other manifestations include skin and ocular involvement (ie, uveitis, retinitis, optic neuritis); generalized lymphadenopathy; afebrile, blood culture-negative endocarditis which, reportedly, can be complicated with cardioembolic strokes; and a sarcoidosis-like syndrome with mediastinal lymph nodes and central nervous system (CNS) involvement (ie, dementia, sensory and motor deficits, ophthalmoplegia, myoclonus, stroke and hypothalamic damage with dysautonomia, emotional impairment, endocrinopathy).
Fewer than 1000 cases have been reported, and less than one half (6-43%) of these patients presented with neurological manifestations. This likely represents an underestimate due to both a low index of suspicion in some cases and difficulties in reaching a diagnosis in others.
This article, besides being a general presentation of Whipple disease, focuses on both the neurologic manifestations and specifics of diagnosis and treatment of Whipple disease with symptomatic CNS involvement (CNS-WD).
Despite the slowly progressive course of most cases of Whipple disease, CNS-WD may have a fulminant course, and manifest isolated CNS-WD cases have been reported in the literature. Prompt diagnosis is imperative, as very effective therapies are easy to employ with typically rapid limitation of CNS progression and even partial reversal of CNS symptoms. If left untreated, progression to death may come as quickly as 1 month after CNS involvement begins.
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What is Whipple’s disease?