eMedicine Specialties > Neurology > Neurological Infections

Whipple Disease: Follow-up

Author: George C Bobustuc, MD, Consulting Staff, Department of Neuro-Oncology, MD Anderson Cancer Center Orlando
Contributor Information and Disclosures

Updated: Jul 15, 2009

Follow-up

Further Inpatient Care

  • Diagnosis and treatment of patients with Whipple disease should be based on a multiteam approach, targeting early involvement of gastroenterology, neurology, ophthalmology, cardiology, and rheumatology specialists.
  • No patients in whom multiple target-organ routine inventory status has been pursued, unless indicated by symptomatology or clinical examination (except for CNS), have been reported.
  • Some have speculated that by the time the CNS involvement becomes clinically relevant in patients with Whipple disease, they also might have disseminated pulmonary, cardiovascular, hepatic, and/or ocular disease. Furthermore, the choice and duration of antibiotic treatment might be influenced by the presence of disease in these organs.
  • The role of routine echocardiogram and chest and abdomen imaging (CT scan or MRI) remains to be established; these should be pursued on an individual case basis and on the clinician's need to know for significant management decisions and prognostic evaluation.

Further Outpatient Care

  • Regular visits in various specialty clinics targeting monitoring clinical response to treatment of significant symptoms and laboratory tests should be pursued.
  • Significant symptom inventory should be reviewed with the patient at each clinic visit.
  • Treatment adherence should be emphasized continuously.
  • Guidelines for response assessment should be reviewed consistently with other specialty teams involved in the care of the patient.
  • CSF PCR analysis should be used as the ultimate tool in monitoring response and treatment decision making.

Inpatient & Outpatient Medications

A long course of antibiotics (more than 1 y) that has good BBB penetrance represents the key in successful treatment of patients with Whipple disease.

Deterrence/Prevention

  • Limited information suggests that the WD bacillus is a saprobe.
  • A limited number of cases have been reported of patients diagnosed with WD several months after spending vacation time in lake regions.
  • In patients who might have a specific IL-12–gamma-interferon axis defect, swimming in lakes may be hazardous, especially in those where accidental drainage of sewage water took place.

Complications

  • Potentially irreversible CNS symptoms have been reported at relapse together with an increased likelihood of onset of resistance to previously efficacious antibiotics.
  • Aggressive CSF PCR monitoring of response to treatment at relapse is of utmost importance.

Prognosis

  • WD left untreated is uniformly fatal.
  • WD may represent a diagnostic dilemma in some cases.
  • For the astute clinician with a high index of suspicion, treating patients with WD could end up being a very rewarding experience. Timely diagnosis and rapid institution of efficacious treatments are paramount in obtaining a potential cure. A long course of antibiotics (over 1 y) which have good BBB penetrance and treatment decisions based on PCR studies of both significant organs (accountable for the symptoms encountered) and CSF are key for successful treatment of WD.
  • In patients treated for less than 1 year, with antibiotics with low BBB penetrance, or without PCR studies to guide treatment decisions, the likelihood of relapse and potentially irreversible neurological deficits is very high (approaching 40%).

Patient Education

  • WD may represent a diagnostic challenge, but treatment is readily available and potentially curative.
  • Patient adherence to a long course of antibiotics is paramount in obtaining a cure; the importance of this should be emphasized repeatedly to the patient.
  • The alternative to poor antibiotic treatment compliance, as a rule, is worsening or early relapse with new or worse and potentially irreversible CNS symptoms. Resistance to previously, clinically proven, sensitive antibiotics also has been reported at relapse.
  • For excellent patient education resources, visit eMedicine's Dementia Center. Also, see eMedicine's patient education article Stroke-Related Dementia.

Miscellaneous

Medicolegal Pitfalls

  • CNS-WD is a very rare condition. It is practically impossible to plan major randomized, prospective clinical trials to compare various management strategies.
  • At this time, no treatment guidelines have been established by any of the major medical entities relevant to this disease (eg, American Academy for Infectious Diseases, American Academy of Gastroenterology, American Academy of Neurology).
  • Most accepted management strategies are from retrospective reviews and consistently emphasize both the necessity of long-term antibiotics with good BBB penetrance and the usefulness of PCR as the best modality of assessing extension of disease, response to treatment, and early relapse. Documentation of PCR is the only way to objectify the use of criteria for success and relapse in terms of credibility for legal and medical purposes.

Special Concerns

Interpretation of some reported results of PCR testing for Whipple disease remains difficult owing to the limited amount of essential information with respect to amplicon analysis with Southern hybridization of a specific probe, restriction fragment length polymorphism analysis, or sequence determination.

 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Mark Gilbert, MD to the development and writing of this article.



More on Whipple Disease

Overview: Whipple Disease
Differential Diagnoses & Workup: Whipple Disease
Treatment & Medication: Whipple Disease
Follow-up: Whipple Disease
References
Further Reading
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Further Reading

Clinical guidelines

Surveillance and management of groups at increased risk of colorectal cancer.
New Zealand Guidelines Group - Private Nonprofit Organization.  2004 May.  84 pages.  NGC:003655

WGO practice guideline: acute diarrhea.
World Gastroenterology Organisation - Medical Specialty Society.  2008 Mar.  28 pages.  NGC:006567


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Neurosarcoidosis

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Keywords

intestinal lipodystrophy, Tropheryma whippelii, T whippelii, WD, CNS-WD, Whipple disease with symptomatic CNS involvement, fever of unknown origin, polyarthralgias, chronic diarrhea

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Contributor Information and Disclosures

Author

George C Bobustuc, MD, Consulting Staff, Department of Neuro-Oncology, MD Anderson Cancer Center Orlando
George C Bobustuc, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, Society for Neuro-Oncology, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Norman C Reynolds Jr, MD, Neurologist, Veterans Affairs Medical Center of Milwaukee; Professor Medical College of Wisconsin (retired)
Norman C Reynolds Jr, MD is a member of the following medical societies: American Academy of Neurology, Association of Military Surgeons of the US, Movement Disorders Society, Sigma Xi, and Society for Neuroscience
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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