Neurologic manifestations of Whipple's disease

Abstract

Whipple's disease is a rare, multisystem, infectious disorder caused by the bacterium Tropheryma whippelii. Patients with central nervous system (CNS) involvement may present in a variety of ways, including cognitive impairment, psychiatric manifestations, gaze palsies, upper motor neuron signs, and hypothalamic dysfunction. A tissue-based diagnosis of Whipple's disease is made based on the presence of periodic acid-Schiff-stained material or by the identification of bacterial RNA using polymerase chain reaction. To avoid relapse, patients should be treated with antimicrobial agents that penetrate the CNS, and therapy should be continued for at least 1 year.