Abstract Details

Title A Case of Whipple Disease with Cognitive Impairment and Leptomeningeal Enhancement on Brain MRI

Topic Infectious Disease

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 082

Objective NA

Background

Whipple disease is a rare systemic disorder caused by the gram positive bacteria Tropheryma whipplei. It commonly presents with gastrointestinal symptoms, joint pains, fever and central nervous system (CNS) manifestations that mimic many autoimmune and inflammatory conditions. CNS involvement (CNS Whipple disease) is perhaps more well-known for oculomasticatory myorrhythmias; other common manifestations include cognitive impairment, myoclonus, ataxia and sleep disorders. Brain MRI typically shows lesions of high signal intensity with minimal enhancement in the hypothalamus, midbrain or medial temporal lobes. The presence of T. whipplei in the CSF confirms the diagnosis.

Design/Methods NA

Results

A 51-year-old previously healthy male was admitted for one-month history of watery diarrhea and profound weight loss. He had severe duodenitis on EGD and the biopsy confirmed infection with Tropheryma whipplei. During this time, he also developed headache and cognitive impairment. His brain MRI showed leptomeningeal enhancement over the bilateral superior parietal lobules, left pars marginalis and right superior frontal gyri. There were a few punctate juxtacortical T2 hyperintensities in the right frontal lobe but no other signal abnormalities or enhancement in the parenchyma were noted. His CSF showed normal cell count, protein and glucose with negative bacterial cultures but PCR testing for T. whipplei was positive. Extensive workup for other infections, immunodeficiency and malignancy were negative. He was given IV ceftriaxone for two weeks followed by oral trimetophrim-sulfamethoxazole and hydroxychloroquine for with good clinical outcome. Repeat MRI showed resolution of leptomeningeal enhancement and repeat CSF testing for T. whipple was negative after 3 months.

Conclusions

To our knowledge, this is the first reported case of CNS Whipple disease presenting with a meningitis-like syndrome with leptomeningeal enhancement on the MRI. The best way to diagnose CNS Whipple disease is by histopathologic identification of T. whipplei in the CSF (preferably by PCR).

Authors/Disclosures
Samantha Anne Gutierrez, MD PRESENTER	Dr. Gutierrez has nothing to disclose.
Ronak K. Kapadia, MD	Dr. Kapadia has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics/Amgen.

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