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Whipple’s Disease

  • Eoin MulroyEmail author
  • John Lynch
  • Tim Lynch
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Whipple’s disease is a rare multi-system bacterial infection caused by the bacilliform bacterium, Tropheryma whipplei. Neurological involvement occurs in up to 50 % of cases. As illustrated by two clinical cases, certain findings can be suggestive of Whipple’s disease of the central nervous system (CNS). In particular, pendular vergence nystagmus, oculomasticatory myorhythmia and oculo-facio-skeletal myorhythmia are pathognomonic of the condition. Nearly all patients also exhibit a supranuclear gaze palsy.

Diagnosing the condition remains exceedingly difficult and often relies on identification of the bacterium within cerebrospinal fluid, usually through polymerase chain reaction. In this publication we propose new diagnostic criteria for Whipple’s disease of the CNS based on both clinical findings and identification of the bacterium.

Imaging of CNS Whipple’s disease has evolved and in particular magnetic resonance spectroscopy and diffusion-weighted MRI have an important diagnostic role and may have a role in monitoring response to treatment.

There are no agreed treatment schedules for Whipple’s disease of the CNS but in addition to new diagnostic criteria, we also propose a treatment algorithm based on the most recent clinical evidence.

Keywords

Cerebellar Ataxia Immune Reconstitution Inflammatory Syndrome Hemifacial Spasm Venereal Disease Research Laboratory Tropheryma Whipplei 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Movement_Disorders_Emergencies_C25.mov (28,691 KB)

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of NeurologyDublin Neurological Institute at the Mater Misericordiae University HospitalDublin 7Ireland
  2. 2.Department of NeurologyUniversity Hospital GalwayGalwayIreland

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