CNS Whipple’s Disease Heralded by Retinal Vasculiti

Background: Whipple's disease is a rare, chronic, multi-organ, bacterial infection. The most common presenting manifestation of Whipple's disease is gastrointestinal symptoms such as abdominal pain, weight loss, diarrhea, and migratory non-deforming sero negative polyarthralgias. Although neuro-ophthalmologic symptoms are common in CNS Whipple's disease, uveitis as a presenting sign is rare. Case Report: A 36-year-old black male presented with the initial complaint of left eye vision loss but left against medical advice, only to return nine weeks later with additional symptoms of right eye visual field loss. Ocular examination revealed panuveitis, vasculitis, optic nerve atrophy left eye, and right homonymous hemianopsia of both eyes. Extensive laboratory testings were all negative. A MRI imaging showed large enhancing lesions in the thalamus, temporal and parietal lobes. A brain biopsy revealed periodic acid-Schiff reagent (PAS)-positive intracytoplasmic organisms within multiple macrophages, consistent with Whipple's disease. After two months of antibiotic therapy, the patient's symptoms and MRI findings were markedly improved. Conclusion: Although rare, Whipple's disease should be considered in any unexplained chronic uveitis especially if accompanied by CNS, gastrointestinal or migratory polyarthralgia symptoms.