CNS Whipple Disease Follow-up

Updated: Dec 11, 2014
  • Author: George C Bobustuc, MD; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM  more...
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Follow-up

Further Outpatient Care

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  • 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.

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Further Inpatient Care

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  • 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.

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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.

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Deterrence/Prevention

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  • 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.

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Complications

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  • 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.

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Prognosis

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  • 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%).

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Patient Education

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  • 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, see eMedicineHealth's patient education article Stroke-Related Dementia.

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