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Whipple's Disease - 11/19/12

posted Nov 19, 2012, 2:12 PM by Monique Tanna   [ updated Dec 27, 2012, 6:41 AM by Purnema Madahar ]
Today's Chief of Service was about a 35 year old male who presented with dizziness, diploplia, arthralgia, diarrhea and gait instability for 3 weeks.  Following the principles of Occam's Razor, Dr. Schuster encouraged the housestaff to come up with a differential diagnosis for these complaints.  Further history revealted symptoms of generalized fatigue, arthralgias in bilateral knees and ankles, watery diarrhea (3-4 episodes daily), abdominal pain, and subjective fevers and cold sweats.  The patient also reported vertigo, gait instability, and falls, as well as diplopia.

On exam, the patient was afebrile with stable vitals, had normal extraocular movements but vertical and horizontal nystagmus, tenderness in bilateral knee and ankle joints, normal muscle strength, with a neurological exam significant only for an unsteady gait and positive romberg.  Labs revealed an H/H of 12.4/37.8, wbc count 11.7, platelets 416, creatinine 0.8, low vitamin A and D 25-OH levels (34 and 9.2, respectively).  The patient underwent an LP which showed
169 wbc, 93% lymphs, 3 rbc, normal glucose (43), elevated protein (56).  CSF was positive for t. whipplei DNA PCR.  EGD showed duodenitis with a normal duodenal biopsy.  The patient had had similar symptoms in 2006 and at that time had had a duodenal biopsy with positive PAS staining consistent with Whipple's disease.  He had been maintained on Keflex for four years until 2010, and now presented with a recurrence with neurologic symptoms.  The patient was recommended to to continue cefixime for one year.

Attached are some excellent review articles on Whipple's disease, including the original article by Dr. Whipple himself!  (Courtesy of Jenn Batisti)
Original Article by Dr. Whipple, Johns Hopkins 1907
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;18:382–93.

Whipple's Review Article
Moos V, Schneider T. Changing paradigms in Whipple's disease and infection with Tropheryma whipplei. Eur J Clin Microbiol Infect Dis. 2011 Oct 30(10):1151-8.