Cerebral Whipple’s Disease: Diagnosis by Brain Biopsy

Whipple’s disease is a rare chronic systemic bacterial infectious disease which can affect multiple organs, with a wide clinical spectrum encompassing many symptoms presenting in various forms and combinations. In the cases where the gastrointestinal tract is implicated, the more frequent localizations involve the small bowel, especially the duodenum. A case of a 67-year-old man who underwent clinical investigation after presenting with a progressive weight loss and showing a hypercapting right paracoeliac adenopathy at PET-CT scan is reported herein. A gastroscopy and a colonoscopy were done. The biopsies of the endoscopically normal ileal mucosa encompassed some submucosal Peyer’s patches. Histological examination of this lymphoid tissue revealed several foamy macrophages which turned out positive on periodic acid-Schiff special staining. Polymerase chain reaction of the microdissected lymph follicles allowed for confirming Whipple’s disease diagnosis. A targeted antibiotic treatment administrated to the patient led to a rapid clinical improvement. This finding of a previously unreported localization of infected macrophages in Whipple’s disease suggests that sampling the organized mucosal-submucosal lymphoid tissue may increase the diagnostic yield in endoscopic biopsies.

Download Full-text

Whipple's disease: rare disorder and late diagnosis

Revista do Instituto de Medicina Tropical de São Paulo ◽

10.1590/s0036-46652012000500010 ◽

2012 ◽

Vol 54 (5) ◽

pp. 293-297 ◽

Cited By ~ 5

Author(s):

Viviane Plasse Renon ◽

Marcelo Campos Appel-da-Silva ◽

Rafael Bergesch D'Incao ◽

Rodrigo Mayer Lul ◽

Luciana Schmidt Kirschnick ◽

...

Keyword(s):

Gastrointestinal Symptoms ◽

Disease Diagnosis ◽

Endoscopic Biopsy ◽

Upper Gastrointestinal Endoscopy ◽

Whipple’S Disease ◽

Whipple's Disease ◽

Polymerase Chain ◽

One Year ◽

Infectious Disorder

Whipple's disease is a rare systemic infectious disorder caused by the bacterium Tropheryma whipplei. We report the case of a 61-year-old male patient who presented to emergency room complaining of asthenia, arthralgia, anorexia, articular complaints intermittent diarrhea, and a 10-kg weight loss in one year. Laboratory tests showed the following results: Hb = 7.5 g/dL, albumin = 2.5 mg/dL, weight = 50.3 kg (BMI 17.4 kg/m²). Upper gastrointestinal endoscopy revealed areas of focal enanthema in the duodenum. An endoscopic biopsy was suggestive of Whipple's disease. Diagnosis was confirmed based on a positive serum polymerase chain reaction. Treatment was initiated with intravenous ceftriaxone followed by oral trimethoprim-sulfamethoxazole. After one year of treatment, the patient was asymptomatic, with Hb = 13.5 g/dL, serum albumin = 5.3 mg/dL, and weight = 70 kg (BMI 24.2 kg/m²). Whipple's disease should be considered a differential diagnosis in patients with prolonged constitutional and/or gastrointestinal symptoms. Appropriate antibiotic treatment improves the quality of life of patients.

Download Full-text

Whipple's disease presenting with isolated neurological symptoms

Journal of Neurosurgery ◽

10.3171/jns.1990.73.4.0623 ◽

1990 ◽

Vol 73 (4) ◽

pp. 623-627 ◽

Cited By ~ 33

Author(s):

Adam P. Brown ◽

Judy C. Lane ◽

Shigeo Murayama ◽

Dennis G. Vollmer

Keyword(s):

Differential Diagnosis ◽

Brain Biopsy ◽

Neurological Symptoms ◽

Neurological Deterioration ◽

Whipple’S Disease ◽

Progressive Dementia ◽

Whipple's Disease ◽

Content Type ◽

The Central Nervous System ◽

Relative Rarity

✓ Whipple's disease is infrequently considered in the differential diagnosis of patients presenting with progressive neurological deterioration. This is in part a result of the relative rarity of this entity and in part due to the more frequent initial presentation of the disease with gastrointestinal, musculoskeletal, or cardiovascular symptoms. A case is described in which the neurological symptoms of progressive dementia and weakness were seen in the relative absence of non-neurological symptomatology. The diagnosis of Whipple's disease was made from a brain biopsy. The neuropathology of Whipple's disease of the central nervous system is described and the importance of considering it as a treatable entity in the differential diagnosis of progressive neurological deterioration, despite the absence of systemic symptomatology, is stressed.

Download Full-text

Tumor Necrosis Factor Inhibitors Exacerbate Whipple’s Disease by Reprogramming Macrophage and Inducing Apoptosis

Frontiers in Immunology ◽

10.3389/fimmu.2021.667357 ◽

2021 ◽

Vol 12 ◽

Author(s):

Asma Boumaza ◽

Soraya Mezouar ◽

Matthieu Bardou ◽

Didier Raoult ◽

Jean-Louis Mège ◽

...

Keyword(s):

Tumor Necrosis Factor ◽

Tumor Necrosis ◽

Systemic Disease ◽

Disease Diagnosis ◽

Whipple’S Disease ◽

Tumor Necrosis Factor Inhibitors ◽

Whipple's Disease ◽

Ifn Γ ◽

Necrosis Factor

Tropheryma whipplei is the agent of Whipple’s disease, a rare systemic disease characterized by macrophage infiltration of the intestinal mucosa. The disease first manifests as arthralgia and/or arthropathy that usually precede the diagnosis by years, and which may push clinicians to prescribe Tumor necrosis factor inhibitors (TNFI) to treat unexplained arthralgia. However, such therapies have been associated with exacerbation of subclinical undiagnosed Whipple’s disease. The objective of this study was to delineate the biological basis of disease exacerbation. We found that etanercept, adalimumab or certolizumab treatment of monocyte-derived macrophages from healthy subjects significantly increased bacterial replication in vitro without affecting uptake. Interestingly, this effect was associated with macrophage repolarization and increased rate of apoptosis. Further analysis revealed that in patients for whom Whipple’s disease diagnosis was made while under TNFI therapy, apoptosis was increased in duodenal tissue specimens as compared with control Whipple’s disease patients who never received TNFI prior diagnosis. In addition, IFN-γ expression was increased in duodenal biopsy specimen and circulating levels of IFN-γ were higher in patients for whom Whipple’s disease diagnosis was made while under TNFI therapy. Taken together, our findings establish that TNFI aggravate/exacerbate latent or subclinical undiagnosed Whipple’s disease by promoting a strong inflammatory response and apoptosis and confirm that patients may be screened for T. whipplei prior to introduction of TNFI therapy.

Download Full-text

Whipple's disease diagnosis following the use of TNF- blockade

Rheumatology ◽

10.1093/rheumatology/ker387 ◽

2011 ◽

Vol 51 (5) ◽

pp. 946-946 ◽

Cited By ~ 13

Author(s):

J. R. Gaddy ◽

Z. Z. Khan ◽

B. Chaser ◽

R. H. Scofield

Keyword(s):

Disease Diagnosis ◽

Whipple’S Disease ◽

Whipple's Disease

Download Full-text

Scanning Electron Microscopy of Human Jejunum in Whipple's Disease

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100079279 ◽

1977 ◽

Vol 35 ◽

pp. 354-355

Author(s):

John H. L. Watson ◽

C. N. Sun

Keyword(s):

Electron Microscopy ◽

Scanning Electron Microscopy ◽

Basement Membrane ◽

Lamina Propria ◽

Brush Border ◽

Whipple’S Disease ◽

Whipple's Disease ◽

Biopsy Specimens ◽

Brush Borders ◽

Scanning Electron

That the etiology of Whipple's disease could be bacterial was first suggested from electron micrographs in 1960. Evidence for binary fission of the bacteria, their phagocytosis by histiocytes in the lamina propria, their occurrence between and within the cells of the epithelium and on the brush border of the lumen were reported later. Scanning electron microscopy has been applied by us in an attempt to confirm the earlier observations by the new technique and to describe the bacterium further. Both transmission and scanning electron microscopy have been used concurrently to study the same biopsy specimens, and transmission observations have been used to confirm those made by scanning.The locations of the brush borders, the columnar epithelial cells, the basement membrane and the lamina propria beneath it were each easily identified by scanning electron microscopy. The lamina propria was completely filled with the wiener-shaped bacteria, Fig. 1.

Download Full-text