Objective:

To highlight multiple chronic neurological manifestations of Whipple disease.

Background:

Whipple’s disease (WD)  is a rare multi-systemic disorder caused by Tropheryma whipplei. Typical manifestations are gastrointestinal, causing weight loss, diarrhea, and malabsorption; however, broad spectrum presentations of uveitis, endocarditis, and, rarely, nervous system disorders are possible. Neurological manifestations of WD include dementia, cerebellar ataxia, psychiatric dysfunction, and seizures.  

Design/Methods:

Case report and literature review using Pub Med and Google Scholar, Search terms utilized: “Whipple’s disease,” and “neurologic,” and “long term.” Inclusion criteria included articles in English discussing long term neurologic manifestations of WD.

Results:

42-year-old woman with a history of hypertension, pericarditis, and presumed WD presented with new-onset refractory status epilepticus after cessation of TMP/SMX regimen. She had continuous right temporal central region subclinical seizures for 11 days despite three anti-seizure medications (levetiracetam, valproic acid, lacosamide and midazolam drip). MRI brain showed multiple supra and infratentorial lesions with patchy enhancement located in bilateral frontal and occipital lobes, right parietotemporal, midbrain, pons, and superior cerebellar peduncle. She completed 21 days of ceftriaxone, with improvement of seizure frequency and severity after initiating antibiotics therapy. Whipple serum PCR negative. Brain biopsy showed showed foamy macrophages. She was diagnosed with CNS Whipple Disease and discharged on lacosamide, valproic acid, and lifelong TMP/SMX. Per literature review, the most common CSN symptoms include dementia, abnormal movements, extrapyramidal symptoms, and ophthalmoplegia. Status epilepticus as an uncommon presentation of WD.

Conclusions:

This patient had negative immunohistochemistry and neuropathology consistent with WD. She was on chronic antibiotics and presented with status epilepticus after antibiotic cessation.  Her seizures were refractory to three anti-seizure medications and resolved with antibiotic therapy for T. whipplei. Her advanced uncontrolled WD caused this new seizure disorder. This case aims to highlight long-term manifestations of CNS WD in a patient with a previous history of GI and cardiac involvement.