1656 Gastrointestinal Whipple’s Disease Complicated With Meningoencephalitis, Ataxia, and Bilateral Uveitis

Abstract

INTRODUCTION: Whipple’s disease (WD) is a rare systemic infection caused by the gram-positive bacterium Tropheryma whipplei. It classically presents as a malabsorption syndrome, but can involve other organs. Incidence is estimated to be 1–3 persons for every one million people. We present a patient with WD complicated by meningoencephalitis, ataxia, and bilateral uveitis. CASE DESCRIPTION/METHODS: A 68-year-old man presented to clinic for nonbloody diarrhea, unintentional weight loss, and joint pains. EGD with duodenal biopsy revealed intracellular periodic-acid Schiff positive bacteria compatible with WD. He was began on a one year course of oral Bactrim twice daily. Eight years later, he presented with altered mental status and ataxia to the hospital. Head imaging was unremarkable. Polymerase chain reaction (PCR) for Tropheryma whipplei was positive in both cerebral spinal fluid (CSF) and vitreous fluid. Repeat EGD with duodenal biopsy was negative for WD. He received a three-week course of IV Meropenem and was switched to IV Ceftriaxone on discharge with plans to receive 2 weeks as outpatient. Afterwards, he once again received a one year course of oral Bactrim. On follow up, his diarrhea had resolved and mentation had improved. DISCUSSION: WD presents a diagnostic challenge due to nonspecific symptoms similar to those in our patient. Histology of duodenal mucosa is the most reliable diagnostic method, however, molecular–biological methods are often required. In one cohort, 99.4% of patients had positive duodenal biopsies. PCR of duodenal biopsies can also be performed. Central nervous system (CNS) involvement is found in 90% of post-mortem autopsies; yet, overt symptoms develop in only 10–40% of patients. Extended antibiotic courses are often required to prevent relapse; however, relapse can still occur years after proper treatment. Manifestation of relapse usually include mental status changes. Time can range from 1.5 to 12 years and the mean reported time is 4.2 years. Our patient falls within the latter portion in terms of relapse time. In a review of 696 patients with WD, one study noted that only 19 had eye involvement including uveitis, vitritis, retinitis, and retrobulbar neuritis. In another article, among 33 WD patients, 1 had eye involvement. Combination of ocular and CNS involvement in WD like in our patient is uncommon. Clinicians must remain vigilant when patients with history of WD exhibit acute mental status changes after adequate therapy as neurologic involvement can be irreversible.