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Open Access Research article

Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis

Florence Fenollar1, François Nicoli23, Claire Paquet4, Hubert Lepidi1, Patrick Cozzone3, Jean-Christophe Antoine5, Jean Pouget2 and Didier Raoult1*

Author Affiliations

1 Unité des rickettsies, URMITE CNRS-IRD UMR 6236, IFR 48, Faculté de médecine, Université de la Méditerranée, Marseille, France

2 Service de Neurologie, Hôpital de La Timone, Marseille, France

3 Centre de Résonance Magnétique Biologique et Médicale and Centre d'Exploration Métabolique par Résonance Magnétique, UMR CNRS 6612, Faculté de Médecine, Université de la Méditerranée, and Hôpital de La Timone, Marseille, France

4 Memory Center, Department of Pathology, Lariboisière Fernand Widal Hospital University of Paris 7 and INSERM UMRS 839, France

5 Service de Neurologie, CHU de Saint-Etienne, France

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BMC Infectious Diseases 2011, 11:171  doi:10.1186/1471-2334-11-171

Published: 15 June 2011

Abstract

Background

Tropheryma whipplei, the agent of Whipple's disease, causes localised infections in the absence of histological digestive involvement. Our objective is to describe T. whipplei encephalitis.

Methods

We first diagnosed a patient presenting dementia and obesity whose brain biopsy and cerebrospinal fluid specimens contained T. whipplei DNA and who responded dramatically to antibiotic treatment. We subsequently tested cerebrospinal fluid specimens and brain biopsies sent to our laboratory using T. whipplei PCR assays. PAS-staining and T. whipplei immunohistochemistry were also performed on brain biopsies. Analysis was conducted for 824 cerebrospinal fluid specimens and 16 brain biopsies.

Results

We diagnosed seven patients with T. whipplei encephalitis who demonstrated no digestive involvement. Detailed clinical histories were available for 5 of them. Regular PCR that targeted a monocopy sequence, PAS-staining and immunohistochemistry were negative; however, several highly sensitive and specific PCR assays targeting a repeated sequence were positive. Cognitive impairments and ataxia were the most common neurologic manifestations. Weight gain was paradoxically observed for 2 patients. The patients' responses to the antibiotic treatment were dramatic and included weight loss in the obese patients.

Conclusions

We describe a new clinical condition in patients with dementia and obesity or ataxia linked to T. whipplei that may be cured with antibiotics.