CNS Whipple Disease Follow-up
- Author: George C Bobustuc, MD; Chief Editor: Niranjan N Singh, MD, DM more...
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.
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.
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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- 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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- 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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- 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%).
See the list below:
- 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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