Whipple disease confined to the central nervous system presenting as a solitary frontal tumor. Case report.

Abstract

Whipple disease is a rare infection caused by the bacterium Tropheryma whippelii. Patients usually present with gastrointestinal symptoms or migratory arthralgias. Although symptomatic central nervous system (CNS) involvement frequently occurs, Whipple disease confined to the CNS is rare. The authors present the case of a 40-year-old man who was surgically treated for a symptomatic left frontal tumor that had the neuroimaging features of a low-grade glioma (LGG). A histopathological investigation revealed a perivascular accentuated inflammation with macrophages harboring PAS-positive diastase-resistant rods, which are distinctive features of cerebral Whipple disease. The patient received cotrimoxazole for 1 year postoperatively and recovered well. This case is exceptional because it represents an isolated cerebral manifestation of Whipple disease that presented as a solitary frontal tumor, thus raising the differential diagnosis of LGG. A review of diagnostic and therapeutic options in suspected cases is presented.