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Sung VW, Lyerly MJ, Fallon KB, et al. Isolated CNS Whipple disease with normal brain MRI and false-positive CSF 14-3-3 protein: a case report and review of the literature. Brain Behav. 2012;2(6):838-43doi: 10.1002/brb3.97.
Sung, V. W., Lyerly, M. J., Fallon, K. B., & Bashir, K. (2012). Isolated CNS Whipple disease with normal brain MRI and false-positive CSF 14-3-3 protein: a case report and review of the literature. Brain and behavior, 2(6), 838-43. https://doi.org/10.1002/brb3.97
Sung, Victor W, et al. "Isolated CNS Whipple disease with normal brain MRI and false-positive CSF 14-3-3 protein: a case report and review of the literature." Brain and behavior vol. 2,6 (2012): 838-43. doi: https://doi.org/10.1002/brb3.97
Sung VW, Lyerly MJ, Fallon KB, Bashir K. Isolated CNS Whipple disease with normal brain MRI and false-positive CSF 14-3-3 protein: a case report and review of the literature. Brain Behav. 2012 Nov;2(6):838-43. doi: 10.1002/brb3.97. Epub 2012 Oct 01. PMID: 23170246; PMCID: PMC3500470.
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Brain Behav. 2012 Nov;2(6):838-43. doi: 10.1002/brb3.97. Epub 2012 Oct 01.

Isolated CNS Whipple disease with normal brain MRI and false-positive CSF 14-3-3 protein: a case report and review of the literature.

Brain and behavior

Victor W Sung, Michael J Lyerly, Kenneth B Fallon, Khurram Bashir

Affiliations

  1. Department of Neurology, University of Alabama at Birmingham Birmingham, Alabama.

PMID: 23170246 PMCID: PMC3500470 DOI: 10.1002/brb3.97

Abstract

Whipple disease (WD) is usually a systemic infectious disease that can have central nervous system (CNS) involvement. WD confined to the CNS is extremely rare and difficult to diagnose, but can be fatal if not treated in a timely fashion. We present the case of a 42-year-old man with a subacute dementia accompanied by a movement disorder consisting of progressive supranuclear gaze palsy, myoclonus, and ataxia. Our patient lacked the typical magnetic resonance imaging (MRI) findings reported with isolated CNS WD and had a false-positive cerebrospinal fluid (CSF) 14-3-3 protein. The patient expired, and definitive diagnosis of isolated CNS WD was made by autopsy with characteristic macrophage accumulations found in the brain but not in the gastrointestinal tract. We examine the literature on isolated CNS WD and discuss how these previously unreported findings make a rare diagnosis even more challenging. The reported patient is the first in the literature with tissue diagnosis of isolated CNS WD in the setting of normal brain MRI and positive CSF 14-3-3 protein. Isolated CNS WD should be added to the list of considerations for a false-positive CSF 14-3-3 protein. Even in the absence of typical MRI lesions, a patient with subacute progressive dementia, supranuclear gaze palsy, and other various neurologic abnormalities should have the diagnosis of isolated CNS WD considered.

Keywords: Infectious disease; MRI; Whipple disease; neuroimaging; neurology

References

  1. Clin Neurol Neurosurg. 2008 Jul;110(7):747-9 - PubMed
  2. Neurology. 2008 Feb 5;70(6):e25 - PubMed
  3. Neurology. 2001 Jun 12;56(11):1528-33 - PubMed
  4. Ann Neurol. 2000 Sep;48(3):395-8 - PubMed
  5. Curr Allergy Asthma Rep. 2001 Nov;1(6):566-71 - PubMed
  6. Scand J Gastroenterol. 2005 May;40(5):607-9 - PubMed
  7. Neurology. 2001 May 8;56(9):1143-53 - PubMed
  8. J Comput Assist Tomogr. 1981 Apr;5(2):246-8 - PubMed
  9. Int J Syst Evol Microbiol. 2001 Jul;51(Pt 4):1471-1479 - PubMed
  10. N Engl J Med. 2007 Jan 4;356(1):55-66 - PubMed
  11. Arch Neurol. 2005 Apr;62(4):618-20 - PubMed
  12. Neurology. 2007 Sep 11;69(11):E12 - PubMed
  13. AJNR Am J Neuroradiol. 2010 Sep;31(8):1493-7 - PubMed
  14. Medicine (Baltimore). 1997 May;76(3):170-84 - PubMed
  15. Ann Neurol. 1999 Nov;46(5):774-7 - PubMed
  16. Neuroradiology. 1996 May;38(4):325-7 - PubMed
  17. Ann Neurol. 1996 Oct;40(4):561-8 - PubMed
  18. J Neuropathol Exp Neurol. 1960 Jan;19:70-5 - PubMed
  19. J Infect Dis. 2003 Sep 15;188(6):797-800 - PubMed
  20. Eur J Gastroenterol Hepatol. 1996 Sep;8(9):899-903 - PubMed
  21. Mov Disord. 1988;3(2):179-84 - PubMed
  22. J Neurol Neurosurg Psychiatry. 2000 Jan;68(1):2-5 - PubMed
  23. J Neurosurg. 2002 Oct;97(4):988-91 - PubMed
  24. Ann Neurol. 1997 Apr;41(4):560-1 - PubMed
  25. Neurosci Lett. 2001 Apr 6;301(3):167-70 - PubMed
  26. Neurology. 2000 Aug 22;55(4):514-6 - PubMed
  27. Mayo Clin Proc. 1988 Jun;63(6):539-51 - PubMed
  28. Lancet Infect Dis. 2008 Mar;8(3):179-90 - PubMed
  29. Medicine (Baltimore). 2002 Nov;81(6):443-57 - PubMed
  30. Dig Dis Sci. 1994 Aug;39(8):1642-8 - PubMed
  31. Am J Pathol. 1967 Aug;51(2):225-42 - PubMed
  32. Neurologia. 1998 Aug-Sep;13(7):324-8 - PubMed
  33. Dig Dis. 2009;27(4):494-501 - PubMed
  34. Neurology. 1982 Jun;32(6):612-7 - PubMed
  35. Mov Disord. 1995 Mar;10(2):195-200 - PubMed
  36. Neurology. 2000 Sep 26;55(6):811-5 - PubMed
  37. Gastroenterology. 1985 Jun;88(6):1867-73 - PubMed
  38. QJM. 2006 Sep;99(9):609-23 - PubMed
  39. Ann Neurol. 1986 Dec;20(6):677-83 - PubMed
  40. Scand J Infect Dis. 2007;39(6-7):623-5 - PubMed
  41. J Neurol Neurosurg Psychiatry. 2002 Sep;73(3):336-9 - PubMed
  42. Clin Neurol Neurosurg. 1996 Nov;98(4):299-304 - PubMed
  43. AJNR Am J Neuroradiol. 1995 Jun-Jul;16(6):1328-9 - PubMed

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