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.

Advances in Tropheryma Whipplei Research: The Rush to Find Biomarkers for Whipple‘s Disease

“…Endocarditis, myocarditis and pericarditis have all been reported [3]. Symptomatic WD of the CNS can occur at the time of initial diagnosis and can accompany intestinal manifestations, but it is more commonly reported as the cause of disease relapse during or after antibiotic treatment [4]. Cases of primary neurological disease without detectable intestinal involvement, also known as pure neurological infection, have been reported and recently revisited [2].…”

Uveo-meningeal Syndromes

“…Neurologic manifestations are varied and thought to be due to parenchymal involvement and include cognitive impairment, psychiatric changes, upper motor neuron signs, and hypothalamic dysfunction. 59 MRI reveals areas of hyperintensity in the basal portions of the brain. 60 Ocular involvement occurs in less than 5% of patients 61 and CNS pathology causing nystagmus, oculomasticatory myorhythmia, and supranuclear ophthalmoplegia are the typical ocular findings.…”

“…[66][67][68] Antibiotic treatment is essential and prolonged treatment for up to 1 year is recommended to prevent relapses and neurologic involvement. 59,65 Drugs that cross the blood-brain barrier are preferred and double-strength oral trimethoprim-sulfamethoxazole is the usual firstline therapy.…”