Skip to main content

Advertisement

SpringerLink
Log in

Neurologic manifestations of Whipple’s disease

  • Published:
Current Infectious Disease Reports Aims and scope Submit manuscript

Abstract

Whipple’s disease is a rare, multisystem, infectious disorder caused by the bacterium Tropheryma whippelii. Patients with central nervous system (CNS) involvement may present in a variety of ways, including cognitive impairment, psychiatric manifestations, gaze palsies, upper motor neuron signs, and hypothalamic dysfunction. A tissue-based diagnosis of Whipple’s disease is made based on the presence of periodic acid-Schiff-stained material or by the identification of bacterial RNA using polymerase chain reaction. To avoid relapse, patients should be treated with antimicrobial agents that penetrate the CNS, and therapy should be continued for at least 1 year.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Whipple GH: A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. Johns Hopkins Hosp Bull 1907, 18:382–391.

    CAS  Google Scholar 

  2. Relman DA, Schmidt TM, MacDermott RP, Falkow S: Identification of the uncultured bacillus of Whipple’s disease. N Engl J Med 1992, 30:293–301.

    Article  Google Scholar 

  3. Raoult D, Birg ML, La Scola B, et al.: Cultivation of the bacillus of Whipple’s disease. N Engl J Med 2000, 342:620–625.

    Article  PubMed  CAS  Google Scholar 

  4. Bentley SD, Maiwald M, Murphy LD, et al.: Sequencing and analysis of the genome of the Whipple’s disease bacterium Tropheryma whipplei. Lancet 2003, 361:637–644.

    Article  PubMed  CAS  Google Scholar 

  5. Louis ED, Lynch T, Kaufmann P, et al.: Diagnostic guidelines in central nervous system whipple’s disease. Ann Neurol 1996, 40:561–568.

    Article  PubMed  CAS  Google Scholar 

  6. Louis ED: Whipple disease. Curr Neurol Neurosci Rep 2003, 3:470–475.

    PubMed  Google Scholar 

  7. Franca MC, de Castro R, Balthazar ML, et al.: Whipple’s disease with neurological manifestations. Arq Neuropsiquiatr 2004, 62:342–346.

    PubMed  Google Scholar 

  8. Mahnel R, Marth T: Progress, problems, and perspectives in diagnosis and treatment of Whipple’s disease. Clin Exp Med 2004, 3:39–43. This is a review of current recommendations for diagnosis and treatment of Whipple’s disease.

    Article  Google Scholar 

  9. Gerard A, Sarrot-Reynauld F, Liozon E, et al.: Neurologic presentation of Whipple disease: Report of 12 cases and review of the literature. Medicine (Baltimore) 2002, 81:443–457. This is a series of 12 patients with CNS Whipple’s disease, along with an analysis of 122 CNS Whipple’s disease cases reported in the literature.

    Article  Google Scholar 

  10. Manzel K, Tranel D, Cooper G: Cognitive and behavioral abnormalities in a case of central nervous system Whipple disease. Arch Neurol 2000, 57:399–403.

    Article  PubMed  CAS  Google Scholar 

  11. Rossi T, Haghighipour R, Haghighi M, et al.: Cerebral Whipple’s disease as a cause of reversible dementia. Clin Neurol Neurosurg 2005, 107:258–261.

    Article  PubMed  CAS  Google Scholar 

  12. Anderson M: Neurology of Whipples disease. J Neurol Neurosurg Psychiatr 2000, 68:2–5.

    Article  PubMed  CAS  Google Scholar 

  13. Averbuch-Heller L, Paulson GW, Daroff RB, Leight RJ: Whipple’s disease mimicking progressive supranuclear palsy: the diagnostic value of eye movement recording. J Neurol Neurosurg Psychiatry 1999, 66:532–535.

    PubMed  CAS  Google Scholar 

  14. Simpson DA, Wishnow R, Gargulinski RB, Pawlak AM: Oculofacial-skeletal myorhythmia in central nervous system Whipple’s disease: additional case and review of the literature. Mov Disord 1995, 10:195–200.

    Article  PubMed  CAS  Google Scholar 

  15. Lynch T, Fahn S, Louis ED, Odel JG: Oculofacial-skeletal myorhythmia in Whipple’s disease. Letter to the Editor. Mov Disord 1997, 12:625–626.

    PubMed  CAS  Google Scholar 

  16. Ford FR, Walsh FB: Clinical observations upon the importance of the vestibular reflexes in ocular movements. Bull Johns Hopkins Hosp 1936, 58:80–88.

    Google Scholar 

  17. Knox DL, Green WR, Tronco JC, et al.: Cerebral ocular Whipple’s disease: a 62-year odyssey from death to diagnosis. Neurology 1995, 45:617–625.

    PubMed  CAS  Google Scholar 

  18. Matthews BR, Jones LK, Saad DA, et al.: Cerebellar ataxia and central nervous system Whipple disease. Arch Neurol 2005, 62:618–620.

    Article  PubMed  Google Scholar 

  19. Naegeli B, Bannwart F, Bertel O: An uncommon cause of recurrent strokes: tropheryma whippleii endocarditis. Stroke 2000, 31:2002–2003.

    PubMed  CAS  Google Scholar 

  20. Famularo G, Minisola G, De Simone C: A patient with cerebral Whipple’s disease and a stroke-like syndrome. Scand J Gastroenterol 2005, 40:607–609.

    PubMed  Google Scholar 

  21. Peters G, Du Plessis DG, Humphrey PR: Cerebral Whipple’s disease with a stroke-like presentation and cerebrovascular pathology. J Neurol Neurosurg Psychiatry 2002, 73:336–339.

    Article  PubMed  CAS  Google Scholar 

  22. Voderholzer U, Riemann D, Gann H, et al.: Transient total sleep loss in cerebral Whipple’s disease: a longitudinal study. J Sleep Res 2002, 11:321–329.

    Article  PubMed  Google Scholar 

  23. Himmelmann B, Brander S, Jung HH, et al.: Severe hypothermia in a patient with cerebral relapse of Whipple’s disease. Infection 2004, 32:119–121.

    Article  PubMed  CAS  Google Scholar 

  24. Papadopoulou M, Rentzos M, Vagiakis E, et al.: An unusual case of insomnia associated with Whipple encephalopathy: first case reported from Greece. Neurol Sci 2005, 26:174–177.

    Article  PubMed  CAS  Google Scholar 

  25. Schroter A, Brinkhoff J, Gunthner-Lengsfeld, et al.: Whipple’s disease presenting as an isolated lesion of the cervical spinal cord. Eur J Neurol 2005, 12:276–279.

    Article  PubMed  CAS  Google Scholar 

  26. Messori A, Di Bella P, Polonara G, et al.: An unusual spinal presentation of Whipple disease. Am J Neuroradiol 2001, 22:1004–1008.

    PubMed  CAS  Google Scholar 

  27. Clarke CE, Falope ZF, Abdelhadi HA, Franks AJ: Cervical myeolpathy caused by Whipple’s disease. Neurology 1998, 50:1505–1506.

    PubMed  CAS  Google Scholar 

  28. Galldiks N, Burghaus L, Vollmar S, et al.: Novel neuroimaging findings in a patient with cerebral Whipple’s disease: a magnetic resonance imaging and positron emission tomography study. J Neuroimaging 2004, 14:372–376.

    Article  PubMed  CAS  Google Scholar 

  29. Lohr M, Stenzel W, Plum G, et al.: Whipple disease confined to the central nervous system presenting as a solitary frontal tumor. Case report. J Neurosurg 2004, 101:336–339.

    Article  PubMed  Google Scholar 

  30. Nelson JW, White ML, Zhang Y, Moritani T: Proton magnetic resonance spectrosopy and diffusion-weighted imaging of central nervous system Whipple disease. J Comput Assist Tomogr 2005, 29:320–322.

    Article  PubMed  Google Scholar 

  31. Misbah SA, Mapston NP: Whipple’s disease revisited. J Clin Pathol 2000, 53:750–755.

    Article  PubMed  CAS  Google Scholar 

  32. Muller SA, Vogt P, Altwegg M, Seebach JD: Deadly carousel or difficult interpretation of new diagnostic tools for Whipple’s disease: case report and review of the literature. Infection 2005, 33:39–42.

    Article  PubMed  CAS  Google Scholar 

  33. Misbah SA, Aamir A, Costello C: Whipple’s disease. Lancet 2004, 363:654–656.

    Article  PubMed  Google Scholar 

  34. Mendel E, Khoo LT, Go JL, et al.: Intracerebral Whipple’s disease diagnosed by stereotactic biopsy: a care report and review of the literature. Neurosurgery 1999, 44:203–209.

    Article  PubMed  CAS  Google Scholar 

  35. Von Herbay A, Ditton HJ, Shuhmacher F, Maiwald M: Whipple’s disease: staging and monitoring by cytology and polymerase chain reaction analysis of cerebrospinal fluid. Gastroenterology 1997, 113:434–441.

    Article  Google Scholar 

  36. Feurle GE, Marth T: An evaluation of antimicrobial treatment for Whipple’s Disease. Tetracycline versus trimethoprimsulfamethoxazole. Dig Dis Sci 1994, 39:1642–1648.

    Article  PubMed  CAS  Google Scholar 

  37. Schnider PJ, Reisinger EC, Gerschlager W, et al.: Long-term follow-up in cerebral Whipple’s disease. Eur J Gastroenterol Hepatol 1996, 8:899–903.

    PubMed  CAS  Google Scholar 

  38. Cooper GS, Blades EW, Remler BF, et al.: Central nervous system Whipple’s disease: relapse during therapy with trimethoprim-sulfamethoxazole and remission with cefixime. Gastroenterology 1994, 106:782–786.

    PubMed  CAS  Google Scholar 

  39. Schnider T, Reisinger EC, Berger, et al.: Treatment guidelines in central nervous system Whipple’s disease. Ann Neurol 1997, 41:561–562.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Unit 198, Neurological Institute, 710 West 168th Street, 10032, New York, NY, USA

    Elan D. Louis MD, MS

Authors
  1. Mara M. Lugassy MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Elan D. Louis MD, MS
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Elan D. Louis MD, MS.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lugassy, M.M., Louis, E.D. Neurologic manifestations of Whipple’s disease. Curr Infect Dis Rep 8, 301–306 (2006). https://doi.org/10.1007/s11908-006-0075-8

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11908-006-0075-8

Keywords

Search

Navigation