Current Infectious Disease Reports

, Volume 8, Issue 4, pp 301–306 | Cite as

Neurologic manifestations of Whipple’s disease

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.

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References

  1. 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.Google Scholar
  2. 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.CrossRefGoogle Scholar
  3. 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.PubMedCrossRefGoogle Scholar
  4. 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.PubMedCrossRefGoogle Scholar
  5. 5.
    Louis ED, Lynch T, Kaufmann P, et al.: Diagnostic guidelines in central nervous system whipple’s disease. Ann Neurol 1996, 40:561–568.PubMedCrossRefGoogle Scholar
  6. 6.
    Louis ED: Whipple disease. Curr Neurol Neurosci Rep 2003, 3:470–475.PubMedGoogle Scholar
  7. 7.
    Franca MC, de Castro R, Balthazar ML, et al.: Whipple’s disease with neurological manifestations. Arq Neuropsiquiatr 2004, 62:342–346.PubMedGoogle Scholar
  8. 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.CrossRefGoogle Scholar
  9. 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.CrossRefGoogle Scholar
  10. 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.PubMedCrossRefGoogle Scholar
  11. 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.PubMedCrossRefGoogle Scholar
  12. 12.
    Anderson M: Neurology of Whipples disease. J Neurol Neurosurg Psychiatr 2000, 68:2–5.PubMedCrossRefGoogle Scholar
  13. 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.PubMedGoogle Scholar
  14. 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.PubMedCrossRefGoogle Scholar
  15. 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.PubMedGoogle Scholar
  16. 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. 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.PubMedGoogle Scholar
  18. 18.
    Matthews BR, Jones LK, Saad DA, et al.: Cerebellar ataxia and central nervous system Whipple disease. Arch Neurol 2005, 62:618–620.PubMedCrossRefGoogle Scholar
  19. 19.
    Naegeli B, Bannwart F, Bertel O: An uncommon cause of recurrent strokes: tropheryma whippleii endocarditis. Stroke 2000, 31:2002–2003.PubMedGoogle Scholar
  20. 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.PubMedGoogle Scholar
  21. 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.PubMedCrossRefGoogle Scholar
  22. 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.PubMedCrossRefGoogle Scholar
  23. 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.PubMedCrossRefGoogle Scholar
  24. 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.PubMedCrossRefGoogle Scholar
  25. 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.PubMedCrossRefGoogle Scholar
  26. 26.
    Messori A, Di Bella P, Polonara G, et al.: An unusual spinal presentation of Whipple disease. Am J Neuroradiol 2001, 22:1004–1008.PubMedGoogle Scholar
  27. 27.
    Clarke CE, Falope ZF, Abdelhadi HA, Franks AJ: Cervical myeolpathy caused by Whipple’s disease. Neurology 1998, 50:1505–1506.PubMedGoogle Scholar
  28. 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.PubMedCrossRefGoogle Scholar
  29. 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.PubMedCrossRefGoogle Scholar
  30. 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.PubMedCrossRefGoogle Scholar
  31. 31.
    Misbah SA, Mapston NP: Whipple’s disease revisited. J Clin Pathol 2000, 53:750–755.PubMedCrossRefGoogle Scholar
  32. 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.PubMedCrossRefGoogle Scholar
  33. 33.
    Misbah SA, Aamir A, Costello C: Whipple’s disease. Lancet 2004, 363:654–656.PubMedCrossRefGoogle Scholar
  34. 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.PubMedCrossRefGoogle Scholar
  35. 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.CrossRefGoogle Scholar
  36. 36.
    Feurle GE, Marth T: An evaluation of antimicrobial treatment for Whipple’s Disease. Tetracycline versus trimethoprimsulfamethoxazole. Dig Dis Sci 1994, 39:1642–1648.PubMedCrossRefGoogle Scholar
  37. 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.PubMedGoogle Scholar
  38. 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.PubMedGoogle Scholar
  39. 39.
    Schnider T, Reisinger EC, Berger, et al.: Treatment guidelines in central nervous system Whipple’s disease. Ann Neurol 1997, 41:561–562.PubMedCrossRefGoogle Scholar

Copyright information

© Current science inc 2006

Authors and Affiliations

  1. 1.Unit 198Neurological InstituteNew YorkUSA

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