title: Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis.
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.
authors.affiliation: Unité des rickettsies, URMITE CNRS-IRD UMR 6236, IFR 48, Faculté de médecine, Université de la Méditerranée, Marseille, France.
authors.forename: Florence
authors.initials: F
authors.lastname: Fenollar
authors.forename: François
authors.initials: F
authors.lastname: Nicoli
authors.forename: Claire
authors.initials: C
authors.lastname: Paquet
authors.forename: Hubert
authors.initials: H
authors.lastname: Lepidi
authors.forename: Patrick
authors.initials: P
authors.lastname: Cozzone
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Antoine
authors.forename: Jean
authors.initials: J
authors.lastname: Pouget
authors.forename: Didier
authors.initials: D
authors.lastname: Raoult
mesh_terms: D000328:Adult; D001259:Ataxia / Q000150:complications / Q000382:microbiology*; D001921:Brain / Q000382:microbiology; D001923:Brain Chemistry; D003704:Dementia / Q000150:complications / Q000382:microbiology*; D018450:Disease Progression; D004660:Encephalitis / Q000134:cerebrospinal fluid / Q000150:complications / Q000382:microbiology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009765:Obesity / Q000150:complications / Q000382:microbiology*; D016133:Polymerase Chain Reaction; D012189:Retrospective Studies; D054851:Tropheryma / Q000302:isolation & purification*; D008061:Whipple Disease / Q000134:cerebrospinal fluid / Q000150:complications* / Q000523:psychology