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Cerebral Whipple's disease: clinical and cerebrospinal fluid findings
F Carella1, P Valla, G Bernardi
1Department of Neurology, National Neurological Institute C. Besta, Milano, Italy.
Abstract:
The case of a patient who had a relapse of cerebral Whipple's disease (WD) one year after discontinuation of a two-years' antibiotic treatment is reported. Neither the clinical course nor the results of magnetic resonance imaging (MRI) and routine examination of the cerebrospinal fluid (CSF) allowed the caring physician to predict the relapse. Retrospective analysis of serial specimens of CSF showed that slight CSF leucocytosis and intrathecal synthesis of IgA might have suggested persistence of infection. The decision to stop antibiotic therapy in cerebral WD is difficult, but evaluation of cell counts and of intrathecal synthesis of IgA may help in the decision. Some patients may need to take treatment indefinitely.
Insights
A cerebral Whipple's disease relapse occurred despite prior antibiotic treatment. Monitoring cerebrospinal fluid (CSF) for leucocytosis and IgA synthesis may help predict relapses in Whipple's disease patients.
Area of Science:
- Neurology
- Infectious Diseases
- Immunology
Background:
- Cerebral Whipple's disease (WD) is a rare neurodegenerative disorder caused by Tropheryma whipplei.
- Long-term antibiotic therapy is the standard treatment, but relapse can occur after treatment cessation.
- Predicting relapse after antibiotic treatment for cerebral WD remains challenging.
Observation:
- A patient relapsed with cerebral WD one year after completing a two-year antibiotic course.
- Clinical presentation, MRI, and routine CSF analysis did not predict the relapse.
- Serial CSF analysis revealed subtle leucocytosis and intrathecal IgA synthesis, potentially indicating persistent infection.
Findings:
- Slight CSF leucocytosis and intrathecal IgA synthesis in serial CSF samples may indicate persistent infection in cerebral WD.
- These markers might aid in predicting relapse risk after antibiotic therapy.
- Standard diagnostic methods were insufficient to foresee the patient's relapse.
Implications:
- The decision to discontinue antibiotic therapy in cerebral WD requires careful consideration.
- Monitoring CSF cell counts and intrathecal IgA synthesis could improve relapse prediction.
- Some patients with cerebral WD may require indefinite antibiotic treatment to prevent recurrence.