Advertisement

Whipple’s Disease

  • John Lynch
  • Tim Lynch
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Patient 1: A 55-year-old-man developed right facial twitching followed 6 months later by somnolence, blurred vision, and imbalance. He noticed that the facial twitching spread to his neck and tongue, and his family noticed that it persisted in sleep. He then developed dysarthria and complained of poor memory, change in personality, malaise, intermittent fevers, increased sweating, and impotence over the ensuing 6 months. On initial assessment 1 year after the onset of facial twitching, orientation, memory, and language were normal. He was intermittently inattentive and had marked dysarthria resulting from rhythmic lingual retraction and masticatory myorhythmia coinciding with rhythmic contractions of the right side of the face, neck, chest, and the right arm. The contractions spread irregularly to the left side of the face, chest, arm, and leg. Vertical gaze was limited, but improved with the oculocephalic maneuver. Saccades were slow in all directions. Pendular vergence oscillations of the right more than the left eye (frequency = 1 Hz) occurred synchronously with masticatory and skeletal myorhythmia (i.e., oculofacial-skeletal myorhythmia). Muscle tone, strength, sensation, deep tendon reflexes, plantar responses, and postural stability were normal. His gait was mildly ataxic.

Keywords

Cerebellar Ataxia Duodenal Biopsy Central Nervous System Tissue Facial Twitching Bull Johns Hopkins Hosp 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

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. Bull Johns Hopkins Hosp 1907;18:382–391.Google Scholar
  2. 2.
    Louis ED, Lynch T, Kaufmann P, Fahn S, Odel J. Diagnostic guidelines in central nervous system Whipple’s disease. Ann Neurol 1996;40:561–568.PubMedCrossRefGoogle Scholar
  3. 3.
    Lynch T, Fahn S, Louis E, Odel JG. Oculofacial-skeletal myorhythmia in Whipple’s disease. Mov Disord 1997;12:624–625.CrossRefGoogle Scholar
  4. 4.
    Wilson KH Blitchington R, Frothingham R, Wilson JAP. Phylogeny of the Whipple’s-disease-associated bacterium. Lancet 1991;338:474–475.PubMedCrossRefGoogle Scholar
  5. 5.
    Dutly F, Hinrikson HP, Seidel T, Morgenegg S, Altwegg M, Bauereind P. Tropheryma whippelii DNA in saliva of patients without Whipple’s disease. Infection 2000;28:219–222.PubMedCrossRefGoogle Scholar
  6. 6.
    Relman DA, Schmidt TM, Mac Dermott RP, Falkow S. Identification of the uncultured bacillus of Whipple’s disease. N Engl J Med 1992;327:293–301.PubMedCrossRefGoogle Scholar
  7. 7.
    La Scola B, Fenollar F, Fournier PE, Altwegg M, Mallet MN, Raoult D. Description of Tropheryma whippelii Int J Syst Evol Microbiol 2001;51:1471–1479.Google Scholar
  8. 8.
    Schoedon G, Goldenberger D, Forrer R, et al. Deactivation of macrophages with interleukin-4 is the key to the isolation of Tropheryma whippelii. J Infect Dis 1997;176:672–677.PubMedGoogle Scholar
  9. 9.
    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
  10. 10.
    Liang Z, La Scola B, Rao HD. Monoclonal antibodies to immunodominant epitope of Tropheryma whippellii. Clin Diagn Lab Immunol 2002;9:156–159.PubMedCrossRefGoogle Scholar
  11. 11.
    Tarroch X, Vives P, Salas A, More J. Subcutanoeus nodules in Whipple’s disease. J Cutan Pathol 2001;28:369–370.CrossRefGoogle Scholar
  12. 12.
    Walter R, Bachmann SP, Schaffner A, Ruegg R, Schoeden G. Bone marrow involvement in Whipple’s disease: rarely reported, but really rare? Br J Haematol 2001;112:677–679.PubMedCrossRefGoogle Scholar
  13. 13.
    James TN. On the wide spectrum of abnormalities in the coronary arteries of Whipple’s disease. Cor Artery Dis 2001;2:115–125.CrossRefGoogle Scholar
  14. 14.
    Lynch T, Odel J, Fredericks DN, et al. Polymerase chain reaction based detection of Tropheryma whippelii in central nervous system Whipple’s disease. Ann Neurol 1997;42:120–124.PubMedCrossRefGoogle Scholar
  15. 15.
    Levy S, Degott C, Redondo A, Benhamou JP, Bernuau J. Acute intracranial hypertension and anicteric cholestasis revealing Whipple’s disease without digestive involvement. Gastroenterol Clin Biol 2001;25:100–102.PubMedGoogle Scholar
  16. 16.
    Quinn N. Rhythmic tremor of the palate and other cranial limb muscles, with cerebellar ataxia: consider Whipple’s disease. Mov Disord 2001;16:787.PubMedCrossRefGoogle Scholar
  17. 17.
    Helliwell TR, Appleton RE, Mapstone NC, Davidson J, Walsh KP. Dermatomyositis and Whipple’s disease. Neuromuscul Disord 2000;10:46–51.PubMedCrossRefGoogle Scholar
  18. 18.
    Schwartz MA Selhorst SB, Ochs AL, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple’s disease. Ann Neurol 1986;20:677–683.PubMedCrossRefGoogle Scholar
  19. 19.
    Wroe SJ, Pires M, Harding B, Youl BD, Shorvon S. Whipple’s disease presenting with multiple intracerebral mass lesions. J Neurol Neurosurg Psychiatry 1991;54:989–992.PubMedCrossRefGoogle Scholar
  20. 20.
    Kremer S, Besson G, Bonaz B, Pasquier B, Le Bas J-F, Grand S. Diffuse lesions in the CNS revealed by MR imaging in a case of Whipple disease. AJNR 2001;22:493.PubMedGoogle Scholar
  21. 21.
    Clarke CE, Falope ZF, Abdelhadi HA, Franks AJ. Cervical myelopathy caused by Whipple’s disease. Neurology 1998;50:1505–1506.PubMedGoogle Scholar
  22. 21a.
    Messori A, Bella PD, Polonara G, et al. An unusual spinal presentation of Whipple disease. AJNR 2001;22:1004–1008.PubMedGoogle Scholar
  23. 22.
    Schnider P, Trattnig S, Kolleger H, Auff E. MR of cerebral Whipple disease. AJNR 1995;16:1328–1329.PubMedGoogle Scholar
  24. 23.
    Maiwald M, von Herbay A, Persing DH, et al. Tropheryma whippelii DNA is rare in the intestinal mucosa of patients without other evidence of Whipple disease. Ann Intern Med 2001;134:115–119.PubMedGoogle Scholar
  25. 24.
    Chan RY, Yannuzzi LA, Foster CS. Ocular Whipple’s disease: earlier definitive diagnosis. Ophthalmology 2001;108(12):2225–2231.PubMedCrossRefGoogle Scholar
  26. 25.
    Ramzan NN, Loftus E, Burgart LJ, et al. Diagnosis and monitoring of Whipple’s disease by polymerase chain reaction. Ann Intern Med 1997;126:520–527.PubMedGoogle Scholar
  27. 26.
    Coria F, Cuadrado N, Velasco C, et al. Whipple’s disease with isolated central nervous system symptomatology diagnosed by molecular identification of Tropheryma whippelii in peripheral blood. Neuologia 2000;15:173–176.Google Scholar
  28. 27.
    Rickman LS, Freman WR, Gren WR, Feldman ST, Sullivan J, Russack V, Relman DA. Uveitis caused by Tropheryma whippelii (Whipple’s disease). N Engl J Med 1995;332:363–366.PubMedCrossRefGoogle Scholar
  29. 28.
    Von Herbay A. Whipple’s disease. Histologic diagnosis after the discovery of Tropheryyma whippelii. Pathologe 2001;22:82–88.CrossRefGoogle Scholar

Copyright information

© Humana Press Inc. 2005

Authors and Affiliations

  • John Lynch
    • 1
  • Tim Lynch
    • 2
  1. 1.Beaumont HospitalIreland
  2. 2.The Mater Misericordiae HospitalDublinIreland

Personalised recommendations