Sign Up for a Free Account
  • Updated 07.12.2023
  • Released 12.09.2003
  • Expires For CME 07.12.2026

Whipple disease



Whipple disease is a treatable, multisystem disease caused by infection with Tropheryma whipplei. Primarily considered a gastrointestinal disorder, the organism also invades joints, heart, skin, eyes, endocrine, and nervous system. Rarely, Whipple disease of the brain may occur as a primary neurologic disorder without multisystem involvement. Cognitive changes, supranuclear gaze palsy, altered consciousness, myoclonus, seizures, and ataxia occur. Oculomasticatory myorhythmia and oculofacioskeletal myorhythmia are pathognomonic signs. Isolated Whipple disease also mimics glioma. PET studies are useful in evaluating atypical MRI; diffusion restriction might also be a useful marker of progression and response to treatment. The author of this article emphasizes the importance of molecular techniques, particularly polymerase chain reaction, to identify and confirm the diagnosis and evaluate therapy. Antibiotic treatment results in improvement with a sustained remission in the majority of patients.

Key points

• Whipple disease of the central nervous system may be primary (ie, without evidence of disease outside of the brain) or secondary, occurring in the context of a multi-system condition.

• The neurologic features of primary and secondary Whipple disease of the brain are diverse and overlap.

• Molecular techniques, in particular, PCR on CSF and blood are essential to the diagnosis.

• Antibiotics can successfully treat Whipple disease of the brain and prevent fatal infection.

Historical note and terminology

Whipple disease is caused by Tropheryma whipplei (T whipplei) and was first described in 1907.

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

  • Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660



ISSN: 2831-9125